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1 Tijdschrift van de Belgische Kinderarts Journal du Pédiatre Belge Belgische vereniging voor kindergeneeskunde société belge de pédiatrie 2012-volume 14-number 1- January-March Article Les pictogrammes au service de l information en pédiatrie Made in Belgium Pseudomonas aeruginosa in cystic fibrosis patients: acquisition, early diagnosis and efficacy of eradication treatment. Surgeon s Corner Single Incision Video- Assisted Thoracic Surgery for Parapneumonic Empyema in Children : a Safe Alternative for Conventional Multiport VATS. Activities Abstracts Belgische Vereniging voor Kindergeneeskunde Société Belge de Pédiatrie Driemaandelijks tijdschrift Publication trimestrielle V.U./ER. : S. Cadranel, ULB, P. Casaer, KUL Kindergeneeskunde, Gasthuisberg Herestraat 49, 3000 Leuven

2 Article Les pictogrammes au service de l information en pédiatrie Fr. André Tous les membres du personnel soignant du service de pédiatrie de l hôpital Princesse Paola, Marche-en-Famenne Keywords: pictogramme, communication, projet d équipe, compliance Abstract : Pictograms, a graphic way of communication and information, have been known for a long time. A team project in the pediatric ward of the Hospital Princesse Paola, has developed a unique way to use them. Indeed, pictograms allow them to communicate with parents and hospitalized children in order to increase their understanding and hence compliance between them and all medical personnel and paramedics. The implementation is explained and examples are given. A practical assessment is planned throughout the year2012. Introduction : Le service de pédiatrie de l hôpital Princesse Paola de Marche-en-Famenne accueille de plus en plus d enfants de nationalités différentes (nombreux enfants issus de centres de réfugiés) et de contextes socio-économiques différents avec des difficultés de compréhension liées aux limites de la langue. L ouverture à ces communautés multiculturelles et parfois défavorisées est pour le personnel soignant une priorité. En effet, quand l information est bien intégrée et comprise, la qualité des soins est plus grande et le suivi (compliance) par les petits patients et leurs parents également. Nous savons que le monde des images est certainement le premier que l enfant parvient à intégrer. L emploi de pictogrammes est un phénomène généralisé : dans les aéroports, sur les produits toxiques, avec des enfants présentant des troubles autistiques, etc, [1,2] Nous évaluons la douleur grâce à l échelle bien connue des visages. (fig.1) Fig. 1 : Exemple d échelle visuelle de la douleur employée dans le service. De même, la poupée Kiwanis (fig. 2) fait partie du quotidien de la pédiatrie puisqu elle est offerte à chaque enfant qui doit être admis à l hôpital et ce, dès les urgences. C est suite à ces deux constats et avec le souhait d améliorer la communication entre les parents, les enfants et le personnel soignant que l équipe de pédiatrie choisi comme projet d équipe pour 2011 de développer l emploi des pictogrammes. De plus, ce mode de communication élargi n est pas très courant dans les services de pédiatrie et il sera intéressant de le confronter à la pratique. Population et méthode Cadre philosophique : Nous nous sommes basés à l origine sur deux textes «fondamentaux» : la Charte européenne de l enfant hospitalisé, notamment en son art. 4 : «Les enfants et leurs parents ont le droit de recevoir une information sur la maladie et les soins, adaptée à leur âge et à leur compréhension» ainsi que la loi coordonnée sur les hôpitaux et autres établissements de soins qui stipule dans son art. lll (normes d organisation) que le protocole d organisation devra traiter en particulier : «des mesures particulières d information, de conseils et de soutien psychologique à destination des enfants, de leurs parents ou de leurs représentants légaux». Dans le cadre précité, des applications bien concrètes sont en usage dans le service (comme dans beaucoup d autres, d ailleurs). Voici deux exemples que nous citerons sans les développer car ils ne font pas l objet de cet article : Fig. 2 : Poupée Kiwanis. Nous proposons à l enfant de lui dessiner un visage, lui donner un prénom et, si l enfant le souhaite, lui «greffer une pathologie». Grâce à ce support, nous pouvons expliquer les examens, les traitements, les conséquences post-op. Nous disposons également de poupées appareillées qui peuvent accompagner une information (par ex drain abdo, ) (fig. 3) Fig. 3 : poupée kiwanis appareillée. Dans un souci de cohérence des différents outils, nous retrouverons tout naturellement la poupée kiwanis dans les pictogrammes. Tijdschrift van de Belgische Kinderarts Vol Nr.1 12

3 Article Définition : Qu est-ce qu un pictogramme? Plusieurs définitions sont proposées [3]. Nous avons choisi de retenir que c est un «symbole graphique destiné à transmettre un message. Il permet de signifier un objet ou transmettre certaines indications simples, sans l utilisation d un texte écrit, conçu pour fournir une information à un large public et dont la compréhension ne dépend pas normalement des connaissances relevant de spécialistes ou de professionnels» (d après l AFNOR) [1] Usage : Destinés au départ aux familles de réfugiés, nous avons rapidement étendu l emploi des pictogrammes à toute la population des parents et enfants hospitalisés. En effet, lors d une hospitalisation, la multiplication des sollicitations et des informations, d autant plus en période de stress, fait que les patients ne peuvent pas toujours tout enregistrer ou alors d une manière qui peut être sélective. Nous avons fréquemment constaté que les parents et les enfants comprennent mieux une information par le biais d une illustration. Fig. 7 : Pictogrammes originaux conçus par la société Pixiwooh. -intégrer tout ceci dans un grand tableau représentant la «durée du séjour», mis en évidence dans la chambre, pour chaque enfant. (Fig. 8) Nous pensons donc que les pictogrammes, soigneusement choisis, peuvent grandement aider les soignants, les enfants et leurs parents. L élaboration des pictogrammes se fera en deux temps : 1 er temps : déterminer les situations auxquelles l enfant et ses parents sont confrontés : soins et traitements, examens techniques, avis médicaux, régimes,.tout cela en fonction d un calendrier horaire et journalier. représenter ces situations : afin de couvrir un large éventail de situations, nous avons opté pour trois types de pictogrammes : les «vrais» pictogrammes pour lesquels l aide d une société d infographie [4] a été nécessaire, (fig. 4) Fig. 8 : tableau «durée du séjour» Ce premier temps est terminé depuis octobre ème temps : évaluation pratique : pictogrammes à adapter, retirer, modifier. mise en place de nouveaux pictogrammes en fonction de cette évaluation pratique Ce deuxième temps est prévu tout au long de l année Fig. 4 : Pictogramme original conçu par la société Pixiwooh. les dessins, élaborés par des personnes du service de pédiatrie ayant une grande expérience dans cette technique, (fig. 5) Conclusion Bien que remontant à la nuit des temps, les pictogrammes sont plus que jamais d actualité dans les relations entre les personnes. Communication symbolique graphique sans l utilisation de texte écrit, mais par dessins figuratifs ayant une signification, le pictogramme crée une association qui permet à l enfant sans langage et écriture, et à ses parents le plus souvent en situation de détresse, de comprendre le message qui, ainsi transmis, favorisera, nous le pensons, la relation entre les différents personnes intervenant pour les soins dans le cadre d un service de pédiatrie. REFERENCES Fig. 5 : dessin original, effectué au sein de l équipe. et les photos, notamment pour certains actes techniques, régimes. La poupée Kiwanis appareillée entre dans cette catégorie. (fig. 6) Fig. 6 : Photos originales Fr. André. élaboration des pictogrammes en tant que tels (outils informatiques, découpage, plastification, supports autocollants) des pictogrammes originaux ont été créés afin de gérer certaines données plus abstraites comme les mots «hier», «demain», «dans 3 jours», etc et la prise horaire de certains médicaments. (Fig. 7) 1. AFNOR : Agence française de Normalisation 2. ASH : Adaptation scolaire et scolarisation des enfants handicapés 3. Fruttiger A., L homme et ses signes. Signes, symboles, signaux. Atelier Perrousseaux, Société Pixiwooh : société infographiste, Rixensart Sites internets utiles Axelia est un logiciel qui a pour objectif la construction de textes en français à partir de pictogrammes (dessin, photos, ou dessins animés). Il a été conçu pour des personnes n ayant pas accès au langage (autisme, aphasie, surdité, troubles du développement du langage oral ou de l écrit.) pour leur permettre de développer une communication fonctionnelle. Il est également utilisé dans un cadre scolaire pour faciliter l accès à la communication et à la structuration de la langue Définition et exemples de pictogrammes : Guide d accessibilité Bruges 2002 : Journal du Pédiatre Belge Vol Nr.1 13

4 Made in Belgium Pseudomonas aeruginosa in cystic fibrosis patients : acquisition, early diagnosis and efficacy of eradication treatment. P. Schelstraete 1, M. Vaneeechoutte 2, F. Haerynck 1, S. Van Daele 1, P. Deschaght 2, F. De Baets 1 1. Department of Pediatric Pulmonology and Cystic Fibrosis Centre, Ghent University Hospital, Ghent 2 Laboratory for Bacteriology Research, Ghent University, Ghent INTRODUCTION Cystic fibrosis (CF) is a life-shortening genetic multi-organ disease, affecting more than people worldwide and about 1000 people in Belgium. The hallmark of cystic fibrosis is a chronic pulmonary disease, ultimately leading to respiratory insufficiency. The disease is caused by a defect in the CFTR protein, resulting in a defective transport of chloride across the cellular membrane. CF affects all racial and ethnic groups, but it is more common among Caucasians. Life expectancy of CF patients has increased considerably over the last decades, due to advances in treatment and better organised patient care. While in the fifties, most CF patients did not survive infancy, over the years the mean survival has steadily increased to a mean predicted survival age of 37.4 years in the US. Pseudomonas aeruginosa is one of the most common and clinically important pathogens in CF patients. First infection can occur very early on in life and by the age of 25, nearly 80% of the patients are intermittently or chronically colonised by P. aeruginosa. Such colonisation adversely affects lung function and survival. P. aeruginosa strains causing early colonisation or infection usually have a nonmucoid phenotype and are relatively antibiotic-sensitive. Aggressive treatment of these early colonising strains often successfully eradicates P. aeruginosa. Early treatment of initial P. aeruginosa colonisation can therefore postpone the onset of chronic colonisation and maintain or increase pulmonary function. Chronic mucoid P. aeruginosa colonisation is usually impossible to eradicate, and the goal of antibiotic treatment is then to suppress, rather than to eliminate the pathogen. We studied different unknown aspects of acquisition, results of eradication therapy and early diagnosis of P. aeruginosa in CF patients. For most CF patients the origin of P. aeruginosa is unclear. Patient-to-patient transmission has been described and mainly results from prolonged and close contact. As P. aeruginosa is widely present in the environment, the possibility of acquisition from the environment has been raised. The different studies on the prevalence of P. aeruginosa in CF health care settings demonstrate that particularly sinks are heavily contaminated. Genotypes of environmental P. aeruginosa sometimes matched patient strains and in one CF patient healthcare environment-to-patient transmission was strongly suspected. In contrast to the extensive studies on patient-to-patient transmission and on the presence of P. aeruginosa in the hospital environment, there are few reports on the prevalence of P. aeruginosa in the home environment of both CF patients and healthy individuals. None of these studies investigated the possibility of environmentto-patient transmission, neither did they compare genotypes of P. aeruginosa isolated from either patients or from their home environment. In the first study of this thesis, we investigated the possibility of CF home environment-to-patient transmission: Pseudomonas aeruginosa in the home environment of newly infected cystic fibrosis patients [1]. We demonstrated that P. aeruginosa is not widely present in the homes of CF patients: P. aeruginosa was detected in only 6% of the samples, originating from 36% of the houses. In nine patients (18%), the genotype of the environmental P. aeruginosa isolate was identical to that of the patient s isolate, suggesting possible environment-to-patient transmission. It remains debatable however, whether the environmental isolates were the source of infection or conversely, whether contamination of the environment was caused by the patient. In our study, 72% of the environmental P. aeruginosa isolates were detected in the drains of the bathroom. Therefore, bathroom drains should be targeted in preventive cleaning procedures. These results indicate that the home environment is not a major source of P. aeruginosa infection in CF patients and as a consequence, the exact source still remains unknown. However, the results of this study in combination with literature data suggest that probably no major source of acquisition of P. aeruginosa for CF patients exists. Some patients get infected from the environment, while others contract P. aeruginosa through patient contact. The latter can be avoided by patient segregation but as P. aeruginosa is ubiquitously present in nature, acquisition in most patients seems unavoidable. Avoiding all possible sources puts a high psychological and social burden on the patients. Therefore, too strict and poorly validated hygienic guidelines should be avoided, although drains should be targeted in preventive cleaning procedures. After the first isolation of P. aeruginosa, patients may go through different episodes of colonisation, preceding chronic colonisation by months to years, eventually resulting in chronic infection. Early treatment for P. aeruginosa colonisation/ infection episodes is now considered the standard of care in the CF centres. Although the effect of eradication treatment regimens on the deferral of chronic colonisation CF patients has been reported, longitudinal data on the genotype of P. aeruginosa isolates after initial colonisation and eradication treatment; i.e. the efficacy of the eradication therapy are limited. We analysed our data in Genotype based evaluation of Pseudomonas aeruginosa eradication treatment success in cystic fibrosis patients [2] and demonstrated that patients becoming chronically colonised have a significantly shorter P. aeruginosa free interval between the first and second isolation event and that the detection of an identical P. aeruginosa genotype in the second isolate is an ominous sign for impending chronic colonisation. In our study, a surprisingly high number of second P. aeruginosa isolates identical to the first isolate (i.e. 68%) was seen, whereas two other and smaller studies revealed identical genotypes of P. aeruginosa isolates compared to the first isolate in just about 25% of the episodes. Our results indicate treatment failure, with rather suppressing but not really eradicating P. aeruginosa after an eradication therapy. More studies are needed to determine the optimal treatment regimen and duration of eradication treatment. Ideally, each study on eradication therapy should include comparison of genotypes of initial and subsequent colonising P. aeruginosa strains. Early detection of P. aeruginosa in CF patients is of utmost importance, as early initiated therapy increases the chances of eradication and consequently postpones the evolution to chronic infection with P. aeruginosa. Culture of broncho-alveolar lavage fluid is considered the gold standard for diagnosis of lower airway infection by P. aeruginosa, but is too invasive for regular use. Sputum cultures have good concordance with cultures of the lower airways. Intermittent P. aeruginosa colonisation however mainly occurs in young children who are not able to expectorate sputum. Oropharyngeal cultures have a low sensitivity for detection of lower airway P. aeruginosa. The results of the studies on the value of P. aeruginosa serology in the early detection of P. aeruginosa indicate that positive serology can precede the isolation of P. aeruginosa by various time periods, but that this phenomenon is not observed in all patients. It is clear that more sensitive diagnostic techniques for early detection of P. aeruginosa in CF patients not chronically colonised by this pathogen are needed. Therefore, we were interested whether PCR based detection techniques can advance the diagnosis of P. aeruginosa colonisation, allowing earlier initiation of P. aeruginosa eradication regimens, which resulted in a third study: Comparison of culture and qpcr for the detection of P. aeruginosa in not chronically colonised CF patients [3]. Our results indicate that there is a good concordance between the results of conventional culture and qpcr for detection of P. aeruginosa in the Journal du Pédiatre Belge Vol Nr.1 15

5 Made in Belgium vast majority of the samples but that PCR positivity may have a predictive value for impending infection in only a limited number of patients. These results do not confirm the results of two other reports on the value of PCR for early detection of P. aeruginosa, with respectively 50% and 32.5% of the samples identified as PCR positive/culture negative for P. aeruginosa, a subsequent patient sample became positive for both culture and PCR, suggesting that PCR has the potential to advance the diagnosis of impending infection with P. aeruginosa. Based on our research and on current literature data, neither PCR nor P. aeruginosa serology alone can predict impending infection but any positive test can heighten the awareness. The ideal combination of diagnostic tests for early detection of P. aeruginosa in not chronically colonised CF patients is still to be determined. REFERENCES 1. Schelstraete P, Van Daele S, De Boeck K et al. Pseudomonas aeruginosa in the home environment of newly infected cystic fibrosis patients. Eur Respir J 2008;31: Schelstraete P, Deschaght P, Van Simaey L et al. Genotype based evaluation of Pseudomonas aeruginosa eradication treatment success in cystic fibrosis patients. J Cyst Fibros 2010;9: Deschaght P, Schelstraete P, Lopes dos Santos Santiago G et al. Comparison of culture and qpcr for the detection of Pseudomonas aeruginosa in not chronically infected cystic fibrosis patients. BMC microbiol 2010;10:245. Tijdschrift van de Belgische Kinderarts Vol Nr.1 16

6 Surgeon s Corner Single Incision Video-Assisted Thoracic Surgery for Parapneumonic Empyema in Children : a Safe Alternative for Conventional Multiport VATS. E. Van Der Veken 1, E. Van Hoorde 2, F. Mergan 3, De Bont 4, P. Van Laer 5, P. Corouge 5, F. Otte 5, T. Khalil 5, Ph. Everarts 6, L. Van Obbergh 7, J. Papadopoulos 5 1. Department of Pediatric Surgery, Centre Hospitalier Jolimont-Lobbes 2. Department of Pediatric Surgery, CHU Charleroi 3. Department of Pediatric Surgery, CHU Tivoli 4. Department of Pediatrics, Centre Hospitalier Jolimont-Lobbes 5. Pediatric Intensive Care Unit, Centre Hospitalier Jolimont-Lobbes 6. Department of Radiology, Centre Hospitalier Jolimont-Lobbes 7. Department of Anesthesiology, Centre Hospitalier Jolimont-Lobbes Keywords: Empyema, children, VATS, parapneumonic effusion Abbreviations: SIVATS : Single Incision Video-Assisted Thoracic Surgery - ICU : Pediatric Intensive Care Unit - LOS : Length of Hospital Stay - VATS : Video-Assisted Thoracic Surgery - PCR :Polymerase Chain Reaction Abstract: Background: In this series of parapneumonic thoracic empyema we emphasize the interest of combining single incision with endobronchial blocker and gasless thoracoscopy (SIVATS) instead of conventional multiport VATS Patients and methods: A retrospective single center study of 50 consecutive cases between January 2003 and August 2010 was performed. Results: All 50 patients were admitted at our pediatric intensive care unit (PICU-middle care), 39 of them (78%) were transferred from other hospitals. Mean age was 60 months (range 8-180). General anesthesia with endobronchial blocking device (Arndt Pediatric Endobronchial Blocker, Cook or Fogarty 4F, Edwards Lifesciences ) was used in all except one case. A single incision of 2 cm was made on the mid-axillar line just below the inferior angle of the scapula. Complete pleural debridement was performed without port introduction and without CO2 insufflation, the instruments being inserted through the single incision. There were no technical difficulties.most patients (48/50) were extubated in the operating room. Mean PICU stay was 7 days (range 4-21). We noticed one recurrence which was treated by redo (SIVATS). Short term results were excellent. Conclusion: Trocarless SIVATS with endobronchial blocker and without carbon dioxide insufflation is a safe and effective method for treatment of parapneumonic empyema. Introduction The frequency of children who are hospitalized with pneumonia complicated by parapneumonic empyema seems to be increasing [1]. Empyema is classically divided into three stages [2]. Stage 1, the early exudative phase, involves a collection of clear reactive fluid in the pleural space (simple parapneumonic effusion). Stage 2 is the fibropurulent phase with deposition of fibrin in the pleural space leading to septation and the formation of loculations. The fluid is thickened and eventually becomes purulent (complicated parapneumonic effusion). Stage 3 is the organizing phase in which a thick and non-elastic peel encases the lung (pleural peel) preventing expansion (trapped lung).the management of empyema remains controversial. Possible therapeutic options include antibiotics, thoracocentesis, chest tube, fibrinolytics, video-assisted thoracic surgery and thoracotomy. With the development of minimal invasive surgery, thoracoscopy became a main option in the treatment of empyema. Most surgeons use two or three ports for the dissection and debridement. However, introduction of several ports can be hazardous especially in the presence of multiple adhesions. We reviewed our experience with SIVATS, emphasizing the interest of combining single incision with endobronchial blocker, gasless thoracoscopy and introduction of the instruments through the incision without port introduction. Patients and Methods A retrospective single center study (Centre Hospitalier Jolimont-Lobbes) of 50 consecutive cases between January 2003 and August 2010 was performed. All patients were admitted at our intensive care unit (PICU-middle care) 78% being transferred from other hospitals with a previous story of severe pneumonia and/or its complications.mean age was 60 months (range 8-180), mean WBC count was /microliter, mean CRP was 28 mg/dl. Diagnosis was made by chest X-ray, ultrasonography or CT scan. Indications for surgery were loculated pleural fluid on ultrasound examination (stage 2) or trapped lung (stage 3). General anesthesia with endobronchial blocking device (Arndt Pediatric Endobronchial Blocker, Cook or Fogarty 4F, Edwards Lifesciences ) was used in all except one case. A single incision of 2 cm on the mid-axillar line just below the inferior angle of the scapula was made.. Through the incision, we performed a mini-thoracotomy and we inserted the 5 mm videolaparoscope under direct control without port introduction. Then we progressively removed the adhesions using an endograsp instrument and an aspiration cannula (alternatively) and complete debridement of the pleural space was performed in all cases. As the affected lung was excluded and exsufflated by the means of an endobronchial blocking device, there was enough working space and no need for CO2 insufflation. Pleural fluid or pus was systematically aspirated and sent for cytological and microbiological analysis. Two chest tubes were left in place. Tijdschrift van de Belgische Kinderarts Vol Nr.1 18

7 Article Results There were no per-operative complications, there was no conversion to thoracotomy. Most patients (48/50) were extubated in the operating room. Mean duration of chest tube drainage was 5 days (range 2-21). Mean PICU stay was 7 days (range 4-21). We noticed one recurrence which was successfully treated by redo (SIVATS). There was one minor air leak which resolved after 48 hours of drainage. No other complications were seen. There were no deaths. After leaving our intensive care unit, patients were send back to the pediatric ward of the referring hospital. Streptococcus pneumoniae was identified in 32 patients. However over the period Streptococcus pneumoniae was identified in 22 cases on 25 mainly due to the introduction of PCR techniques. Antibiotics were maintained for 4 to 6 weeks, depending on the severity of the initial pneumonia and on bacteriological findings. Short term results were excellent. Discussion Management of empyema is still a matter of debate. Many studies are published but there is not yet a consensus concerning optimal treatment. Some authors mention excellent results with non-operative methods such as fibrinolytic therapy [3,4] but with the development of minimally invasive surgery thoracoscopy became a main option. Many authors recommend early VATS as it is associated with reduced morbidity and decreased hospital length of stay (LOS) and hospital charges [5-18]. In late stage empyema with multiple septations and adhesions technical difficulties may occur during VATS. Introduction of ports can be hazardous and parenchymal lung injury is to be avoided. Complications, even deaths, are described [8] but there are probably more problems than mentioned in literature. A few authors used single-port techniques [19-20] one of them with introduction of a balloon in the pleural cavity to create working space [19]. We use a similar technique making a single incision of 2 cm through which a mini-thoracotomy is performed. With the Arndt blocking device there is no need for CO2 insufflation, the affected lung is exsufflated and flat allowing a full debridement. Other authors have described the advantages of one-lung ventilation during video-assisted thoracic surgery [21-22].We found this technique very satisfactory giving less complications than conventional VATS. Conclusion Single incision video assisted thoracic surgery with endobronchial blocker, without carbon dioxide insufflation and without port introduction is an adequate technique for pleural cavity debridement. In late stage empyema were multiple adhesions can cause technical difficulties it seems to be a good and safe alternative for VATS. Possible complications due to carbon dioxide (over) insufflation are also avoided. REFERENCES 1. Proesmans M, De Boeck K. Clinical practice : treatment of childhood empyema. Eur J Pediatr. 2009;168: Balfour-Lynn IM, Abrahamson E, Cohen G et al. Paediatric Pleural Diseases Subcommittee of the BTS Standards of Care Committee. BTS guidelines for the management of pleural infection in children. Thorax. 2005;60: St Peter SD, Tsao K, Spilde TL et al. Thoracoscopic decortication vs tube thoracostomy with fibrinolysis for empyema in children : a prospective randomized trial. J Pediatr Surg. 2009;44: Sonnappa S, Cohen G, Owens CM et al. Comparison of urokinase and videoassisted thoracoscopic surgery for treatment of childhood empyema. Am J Respir Crit Care Med. 2006;174: Padman R, King KA, Iqbal S et al. Parapneumonic effusion and empyema in children : retrospective review of the dupont experience Clin Pediatr (Phila). 2007;46: Suchar AM, Zureikat AH, Glynn L et al. Ready for the frontline: is early thoracoscopic decortication the new standard of care for advanced pneumonia with empyema? Am Surg. 2006;72: Gates RL, Caniano DA, Hayes JR et al. Does VATS provide optimal treatment of empyema in children? A systematic review. J Pediatr Surg. 2004;39: Meier AH, Hess CB, Cilley RE. Complications and treatment failures of videoassisted thoracoscopic debridement for pediatric empyema. Pediatr Surg Int. 2010;26: Li ST, Gates RL. Primary operative management for pediatric empyema: decreases in hospital length of stay and charges in a national sample. Arch Pediatr Adolesc Med. 2008;162: Shah SS, Ten Have TR, Metlay JP. Costs of treating children with complicated pneumonia: a comparision of primary video-assisted thoracoscopic surgery and chest tube placement. Pediatr Pulmonol. 2010;45: Aziz A, Healey JM, Qureshi F et al. Comparative analysis of chest tube thoracostomy and video-assisted thoracoscopic surgery in empyema and parapneumonic effusion associated with pneumonia in children. Surg Infect (Larchmt). 2008;9: Avansino JR, Goldman B, Sawin RS et al. Primary operative versus nonoperative therapy for pediatric empyema: a meta-analysis. Pediatrics. 2005;115: Bishay M, Short M, Shah K et al. Efficacy of video-assisted thoracoscopic surgery in managing childhood empyema: a large single-centre study. J Pediatr Surg. 2009;44: Pappalardo E, Laungani A, Demarche M et al. Early thoracoscopy for the management of empyema in children.acta Chir Belg. 2009;109: Coote N, Kay ES. Surgical versus non-surgical management of pleural empyema. Cochrane Database Syst Rev. 2009;4:CD Liu HP, Hsieh MJ, Lu HI et al. Thoracoscopic-assisted management of postpneumonic empyema in children refractory to medical response. Surg Endosc. 2002;16: Cohen G, Hjortdal V, Ricci M et al. Primary thoracoscopic treatment of empyema in children. J Thorac Cardiovasc Surg. 2003;125: Scarci M, Zahid I, Billé A et al. Is video-assisted thoracoscopic surgery the best treatment for bpaediatric pleural empyema? Interact Cardiovasc Thorac Surg. 2011;13: tander B, Ustun L, Ariturk E et al. Balloon-assisted single-port thoracoscopic debridement in children with thoracic empyema. J Laparoendosc Adv Surg Tech A. 2007;17: Martínez-Ferro M, Duarte S, Laje P. Single-port thoracoscopy for the treatment of pleural empyema in children. J Pediatr Surg. 2004;39: Ho AC, Chung HS, Lu PP et al. Facilitation of alternative one-lung and two-lung ventilation by use of an endotracheal tube exchanger for pediatric empyema during video-assisted thoracoscopy. Surg Endosc. 2004;18: Ho AC, Chen CY, Yang MW et al. Use of the Arndt wire-guided endobronchial blocker to facilitate one-lung ventilation for pediatric empyema during video-assisted thoracoscopy. Chang Gung Med J. 2005;28: Journal du Pédiatre Belge Vol Nr.1 19

8 Activities of Paediatric Societes RESUME DES DOSSIERS ETUDIES en 2011 MEMBRES ACTUELS du Comité de Direction : Président : Georges Casimir; Vice-Président : Philippe Alliet; Secrétaire : Michel Pletincx; Trésorier : Yvan Vandenplas; Membres : Marc Alexander, Pierre Bauche, Dino Barillari, Herman Fivez, Dirk Mathys, Jean-Paul Misson, Jose Ramet, Hilde Van Hauthem, Christel Van Geet, Gaston Verellen, Pierre Vincke; Experts :Samy Cadranel, Yves Louis. Le Groupe de Direction de l Académie s est réuni les 18/01/11, 15/02/11, 21/03/11, 3/5, 15/6, 6/9, 4/10, 7/11 et 6/12/2011 et les principaux sujets abordés ont été : COLLEGE DE PEDIATRIE : 1. Compétences particulières : Le texte concernant les soins intensifs pédiatriques a été finalisé. Le Collège a reçu le 26/5 le Dr Marc Moens, président de l absym et secrétaire général du GBS concernant le dossier des compétences particulières qui doit être discuté au Conseil supérieur. Des corrections devront être apportées au texte envoyé par les pédiatres pour se mettre en accord avec les dispositions légales. Le Dr Moens promet d appuyer la position des pédiatres concernant la reconnaissance des hémato-oncologues pédiatriques par les commissions d agrément de pédiatrie. Le maintien d accès aux prestations connexes pour les pédiatres a été maintenu contrairement à ce qui s est fait pour les spécialistes d adultes. Les textes concernant la gastro, la pneumo, l endocrino, la cardio et la néphro ont été mis à l ordre de la réunion plénière de Conseil Supérieur du 8/09/11. Des points importants concernent la possibilité d exercice de la Pédiatrie Générale pour les pédiatres reconnus dans ces compétences ainsi que la reconnaissance des pédiatres compétents par les commissions actuelles d agrément en pédiatrie auxquelles sont adjointes des experts. On évoque aussi l obligation pour les futurs neuropédiatres de passer par la filière pédiatrique. 2. Projets : le Collège a retenu comme projet l étude des urgences pédiatriques : organisation actuelle, charge de travail, guidelines, qualité et efficacité du tri Un questionnaire destiné à tous les services de pédiatrie doit être rédigé. Il est suggéré de se pencher aussi sur l activité de garde pour la maternité et la salle d accouchement. 3. Conseil National des Etablissements Hospitaliers : L introduction de la norme des 4 pédiatres a une nouvelle fois été repoussée, cette fois sans date butoir. Un groupe de travail du CNEH, à la demande de la ministre, se penche sur la réactualisation du programme de soins pédiatrique qui date de Le document de Zorgnet Vlaanderen doit y être discuté. On reparle de fixer le nombre de pédiatres à 3 par programme et d en séparer l hôpital de jour chirurgical. Les membres du Collège ont été invités à plusieurs réunions de ce groupe de travail du CNEH dirigé par Rob Van Den Oever auquel participent les différentes organisations de gestionnaires hospitaliers. Chris Van Geet a présenté le point de vue du Collège. Un équilibre doit être trouvé entre une accessibilité suffisante et la rentabilité financière de programmes de soins pédiatriques avec un encadrement de qualité. HOSPITALISATION JOUR et PROVISOIRE : Les résultats de l étude BePASSTA ont été présentés ; il manque encore les détails de facturation et d emploi du temps. Il apparaît que dans les hôpitaux ayant participé à l étude, 71% des enfants se présentant aux urgences sont vus par un pédiatre ou un assistant en pédiatrie et pas par un urgentiste. Les visites des patients aux urgences seraient inappropriées dans 40% des cas selon les critères fixés par les chercheurs. Les patients de moins de 5 ans sont 46% à se présenter la nuit et le WE contre 30% après 10 ans ; 4 enfants sur 10 venus aux urgences sont vus en période difficile. Si l on veut développer l activité médicale de jour par rapport à d autres, il faudra encourager les hôpitaux par un bon financement des infrastructures, du personnel et des honoraires. DUREE DE TRAVAIL des ASSISTANTS : La réduction du temps de travail des assistants et un financement complémentaire pour les 12 heures du opting out impliquent une augmentation de charge de travail pour les autres et un surcoût. Il faudra trouver de nouveaux moyens de financement des candidats spécialistes et des maîtres de stage si l on veut garantir la pérennité des places de stage nécessaires. Une lettre a été envoyée à Laurette Onkelinx par tous les assistants des différentes spécialités pour demander des conditions de travail et de rémunération identiques dans tout le pays. Une intervention de l Etat pourrait alléger la charge des services de stage. Aux Pays-Bas, l Etat finance totalement les assistants qui reçoivent une somme déterminée et peuvent ensuite choisir leur endroit de stage. En Belgique, les maîtres de stage en médecine générale bénéficient déjà d un soutien financier sans que cela ne soit le cas pour la médecine spécialisée. AGREMENT des PEDIATRES, SERVICES et MAITRES de STAGE : Le Docteur Dirk Cuypers, Président du Conseil Supérieur des médecins spécialistes et généralistes, a rencontré les 2 commissions d agrément de Pédiatrie pour informer que le conseil Supérieur désirait revoir les critères de formation avec des membres de commissions d agrément de toutes les spécialités. Une réunion plénière puis une réunion en groupe de travail par spécialité sont prévues. Une réunion plénière regroupant les membres du Conseil Supérieur et 2 représentants de chaque Commission néerlandophone et francophone de médecins spécialistes s est tenue le 14/05/11. Tous les interlocuteurs étaient d accord sur la nécessité de revoir les critères des formations. Les échanges furent très intéressants mais sont restés malgré tout très généraux. La volonté semble Tijdschrift van de Belgische Kinderarts Vol Nr.1 20

9 Activities of paediatric Societes bien exister de la part du Conseil Supérieur d écouter les commissions d Agrément et d examiner chaque spécialité en particulier. Il faudra sans doute changer les critères d agrément, adapter le nombre des places de stage, examiner les critères minimaux à atteindre, surtout depuis la mise en place des 48h/ semaine. Il faudrait financer le travail du maître de stage à l instar de ce qui se fait en médecine générale. Une réunion s est tenue le 12/7/11 entre 4 représentants des commissions d agrément de pédiatrie et un groupe de travail du Conseil Supérieur concernant la réactualisation des critères de formation des candidats spécialistes et des services de stage. Le but est de trouver également une certaine harmonisation entre les textes proposés par les différentes spécialités. Un juriste présent veillait à la conformité des textes et à leur simplification. Le Conseil Supérieur demande que les commissions d agrément déterminent les objectifs à atteindre pendant la formation. Les carnets de stage devraient exister en version électronique. Il faut exiger qu un pédiatre ne soit reconnu qu après avoir presté au moins 3 ans de médecine pédiatrique clinique. Enfin, l attestation de compétence en néonatologie pour le N* devrait faire partie intégrante de la formation du pédiatre. PLAN ENFANT : 1. Forfait patients chroniques : s il est nécessaire d améliorer l encadrement paramédical dans la prise en charge des malades chroniques hors convention, il est tout aussi nécessaire de l améliorer pour les cas aigus. Ces problèmes sont aussi très présents dans les unités N*. Le Dr Haucotte de l INAMI a envoyé un mail à Georges Casimir concernant les pathologies non reprises actuellement dans les forfaits et la réflexion sur une prise en charge globale de ces pathologies. Plusieurs demandes ont été introduites à l INAMI pour des pathologies comme la drépanocytose ou le syndrome de Down. Georges Casimir et Philippe Alliet ont rencontré Madame Haucotte de l INAMI en charge des conventions. Madame Haucotte a prêté une oreille attentive aux propositions visant à améliorer l encadrement paramédical général des patients ne bénéficiant pas de conventions. Elle va rédiger un texte qu elle enverra à l Académie. 2. Planification de la Pédiatrie : Il faudrait analyser les forces et faiblesses du programme de soins pédiatriques en réfléchissant sur l encadrement nécessaire, par exemple pour la gestion des urgences, les soins intensifs pédiatriques, la cardiologie ou la chirurgie lourde. ACADEMIE ROYALE de MEDECINE : L Académie Royale de Médecine de Belgique désire se pencher sur les problèmes rencontrés dans les soins aux enfants et veut créer une commission Mère-Enfant en son sein. Des membres gynécologues et pédiatres devraient y être nommés. Le but de cette Commission serait de faire des propositions sur des sujets comme la protection de la mère et l entant, la vaccination ou des améliorations à apporter aux programmes de soins pédiatriques, l idée étant de proposer une réflexion intégrée vers un plan «Enfant». Il est cependant souligné qu il existe un risque de multiplication de commissions au détriment de la force de notre Académie qui est unifie toutes les forces de la Pédiatrie. Il ne faudrait pas perdre en particulier notre représentativité visà-vis du politique. Les sujets abordés au niveau de l Académie de Médecine devraient se limiter au niveau scientifique. Rapport Michel PLETINCX, Secrétaire Journal du Pédiatre Belge Vol Nr.1 21

10 Activities of Paediatric Societes SAMENVATTING VAN DE IN 2011 BEHANDELDE DOSSIERS HUIDIGE LEDEN van het Bestuurscomité : Voorzitter: Georges Casimir Ondervoorzitter: Philippe Alliet Secretaris: Michel Pletincx Penningmeester: Yvan Vandenplas leden: Marc Alexander, Pierre Bauche, Dino Barillari, Herman Fivez, Dirk Mathys, Jean-Paul Misson, Jose Ramet, Hilde Van Hauthem, Christel Van Geet, Gaston Verellen, Pierre Vincke. Experts: Samy Cadranel, Yves Louis. De Stuurgroep van de Academie is bijeengekomen op , , , , , , , en en de belangrijkste punten die aan bod kwamen, waren: COLLEGE PEDIATRIE : 1. Bijzondere bekwaamheden: De tekst over de pediatrische intensieve zorgen werd afgewerkt. Het College ontving op Dr. Marc Moens, voorzitter van BVAS en secretaris-generaal van het VBS, in verband met het dossier van de bijzondere bekwaamheden, dat moet worden besproken in de Hoge Raad. Er zullen correcties moeten worden aangebracht aan de tekst die de pediaters hebben gestuurd om te voldoen aan de wettelijke bepalingen. Dr. Moens belooft het standpunt van de pediaters m.b.t. de erkenning van de kinderhemato-oncologen door de erkenningscommissie kindergeneeskunde te steunen. De toegang tot de verwante verstrekkingen voor de pediaters werd behouden, in tegenstelling tot de specialisten voor volwassenen. De teksten over gastro, pneumo, endocrino, cardio en nefro werden op de dagorde van de plenaire vergadering van de Hoge Raad van gezet. Belangrijke punten zijn de mogelijkheid om Algemene Kindergeneeskunde te beoefenen voor de pediaters die in voormelde bekwaamheden erkend zijn, alsook de erkenning van de bevoegde pediaters door de huidige erkenningscommissie kindergeneeskunde waaraan experts zijn toegevoegd. Ook de verplichting voor de toekomstige kinderneurologen om een pediatrie-opleiding te volgen, zal worden besproken. 2. Projecten: het College heeft de studie van de pediatrische urgenties als project in aanmerking genomen: huidige organisatie, werklast, guidelines, kwaliteit en efficiëntie van triage Er moet een vragenlijst ter attentie van alle kinderafdelingen worden opgesteld. Er wordt gesuggereerd om zich ook te buigen over de wachtdiensten op de kraamafdeling en in het verloskwartier. 3. Nationale Raad voor Ziekenhuisvoorzieningen: De invoering van de norm van 4 pediaters werd nog maar eens uitgesteld, dit maal zonder limietdatum. Op vraag van de minister houdt een werkgroep van de NRZV zicht bezig met het updaten van het pediatrisch zorgprogramma, dat dateert van Het document van Zorgnet Vlaanderen zal er moeten worden besproken. Er gaan opnieuw stemmen op om het aantal pediaters vast te leggen op 3 per zorgprogramma en het chirurgisch dagziekenhuis ervan los te koppelen. De leden van het College werden uitgenodigd op verscheidene vergaderingen van deze werkgroep van de NRZV, die door Rob van den Oever wordt geleid en waaraan de diverse organisaties van ziekenhuisbeheerders deelnemen. Chris Van Geet heeft de visie van het College uiteengezet. Er moet een evenwicht worden gevonden tussen een voldoende toegankelijkheid en de financiële rentabiliteit van de pediatrische zorgprogramma s met een kwalitatief hoogstaande omkadering. DAGHOSPITALISATIE EN VOORLOPIGE HOSPITALISATIE: De resultaten van de BePASSTA-studie zijn voorgesteld; de details over facturering en tijdsgebruik ontbreken nog. In de ziekenhuizen die aan de studie hebben deelgenomen blijkt dat 71 % van de kinderen die zich op de spoedafdeling aandienen, worden gezien door een pediater of een assistent in de pediatrie en niet door een spoedarts. Van de patiëntbezoeken aan de spoedafdeling zou, op basis van de door de onderzoekers vastgestelde criteria, 40 % ongerechtvaardigd zijn. Kinderen onder 5 jaar maken 46 % uit van de patiënten die zich s nachts en tijdens het WE aandienen, tegen 30 % kinderen ouder dan 10 jaar; 4 op 10 kinderen op de spoedafdeling worden gezien in moeilijke periodes. Als men de medische dagactiviteit t.o.v. andere activiteiten wil ontwikkelen, zal men de ziekenhuizen moeten aanmoedigen door een goede financiering van infrastructuur, personeel en honoraria. WERKDUUR ASSISTENTEN: De beperking van de werkduur van de assistenten en een aanvullende financiering voor de 12 opting out-uren brengen een toename van de werklast voor de anderen en extra kosten met zich mee. Er moeten nieuwe middelen voor de financiering van de kandidaat-specialisten en de stagemeesters gevonden worden als men het duurzaam behoud van de noodzakelijke stageplaatsen wil garanderen. De assistenten van de diverse specialismen hebben een brief gestuurd naar Laurette Onkelinx om in het hele land identieke werk- en vergoedingsvoorwaarden te vragen. Door een tussenkomst vanwege de Staat zou de werklast van de stagediensten kunnen worden verlicht. In Nederland staat de Staat in voor de volledige financiering van de assistenten, die een bepaald bedrag ontvangen en vervolgens hun stageplaats kunnen kiezen. In België genieten de stagemeesters in de algemene geneeskunde al een financiële tegemoetkoming, maar dit geldt niet voor de specialistische geneeskunde. ERKENNING VAN PEDIATERS, STAGEDIENSTEN en -MEESTERS: Dokter Dirk Cuypers, voorzitter van de Hoge Raad van geneesheren-specialisten en huisartsen, heeft een onderhoud gehad met de 2 kamers van de erkenningscommissie Kindergeneeskunde om mee te delen dat de Hoge Raad de opleidingscriteria wenste te herzien in samenspraak met leden van de erkenningscommissies van alle specialismen. Er zijn een plenaire vergadering, en vervolgens een vergadering in een werkgroep per specialisme gepland. Tijdschrift van de Belgische Kinderarts Vol Nr.1 22

11 Activities of paediatric Societes Op vond een plenaire vergadering plaats met de leden van de Hoge Raad en 2 vertegenwoordigers van elke Nederlandstalige en Franstalige kamer van de erkenningscommissies van geneesheren-specialisten. Alle gesprekspartners waren het eens over de noodzaak om de opleidingscriteria te herzien. De gedachtewisselingen waren zeer interessant, maar bleven niettemin zeer algemeen. Vanwege de Hoge Raad lijkt wel degelijk de wil te bestaan om naar de erkenningscommissies te luisteren en elk specialisme individueel door te lichten. Ongetwijfeld zullen de opleidingscriteria moeten worden gewijzigd, het aantal stageplaatsen aangepast en de te bereiken minimumcriteria bestudeerd, vooral sinds de invoering van de 48-urenweek. Er zou een financiering voor het werk van de stagemeester moeten komen, zoals bij de algemene geneeskunde. Op heeft er een vergadering plaatsgevonden met 4 vertegenwoordigers van de twee kamers van de erkenningscommissie kindergeneeskunde en een werkgroep van de Hoge Raad voor de bijwerking van de opleidingscriteria voor de kandidaat-specialisten en de stagediensten. Het was de bedoeling een zekere harmonisatie te bereiken tussen de door de verschillende specialismen voorgestelde teksten. Een jurist was aanwezig om over de gelijkvormigheid en vereenvoudiging van de teksten te waken. De Hoge Raad vraagt dat de erkenningscommissies de tijdens de opleiding te bereiken doelstellingen vastleggen. Er dient echter op gewezen dat er een risico bestaat op een wildgroei van commissies, die nadelig zou zijn voor de kracht van de Académie, die alle spelers binnen de Pediatrie verenigt. We mogen vooral onze representativiteit t.o.v. de politiek niet kwijtspelen. De onderwerpen die worden behandeld in de Académie de Médecine moeten beperkt blijven tot het wetenschappelijke domein. Verslag Michel PLETINCX, Secretaris De stageboekjes zouden in elektronische versie moeten bestaan. De eis moet worden gesteld dat een pediater slechts kan worden erkend nadat hij minstens 3 jaar klinische kindergeneeskunde heeft verricht. Ten slotte zou de attestering voor bekwaamheid in de neonatologie voor de N* noodzakelijk deel moeten uitmaken van de opleiding van de pediater. KINDERPLAN: 1. Forfait chronische patiënten: als de paramedische omkadering bij de zorgverstrekking aan chronisch zieken buiten conventie moet verbeteren, is het net zo goed noodzakelijk dat dit ook voor de acute gevallen gebeurt. Deze problemen zijn ook erg aanwezig op de N*-afdelingen. Dr. Haucotte van het RIZIV heeft een mail gestuurd naar Georges Casimir over de aandoeningen die momenteel niet in de forfaits begrepen zijn, waarbij ze een bespiegeling maakt over een alomvattende aanpak van deze aandoeningen. Er zijn verscheidene aanvragen ingediend bij het RIZIV voor aandoeningen zoals sikkelcelanemie en het syndroom van Down. Georges Casimir en Philippe Alliet hebben een ontmoeting gehad met mevrouw Haucotte, die bij het RIZIV verantwoordelijk is voor de conventies. Mevrouw Haucotte heeft aandachtig geluisterd naar de voorstellen om de paramedische omkadering van de patiënten die niet van de conventies genieten te verbeteren. Ze gaat een tekst opstellen die ze naar de Academie zal sturen. 2. Planning van de Pediatrie: Er zou een analyse moeten worden gemaakt van de sterke en zwakke punten van het pediatrisch zorgprogramma met als uitgangspunt de omkadering die vereist is, bijvoorbeeld voor de afhandeling van de spoedgevallen, de pediatrische intensieve zorgen, de cardiologie en de zware heelkunde. ACADEMIE ROYALE de MEDECINE : De Académie Royale de Médecine de Belgique wenst de problemen waarmee men te maken krijgt bij de zorgverstrekking aan kinderen te bestuderen en wil in haar schoot een commissie Moeder-Kind oprichten. In die commissie zouden gynaecologen en pediaters als leden moeten worden aangesteld. Deze commissie zou voorstellen moeten uitwerken voor thema s als de bescherming van moeder en kind, vaccinatie en verbeteringen aan de pediatrische zorgprogramma s. De idee bestaat erin tot een geïntegreerde benadering te komen die de weg bereidt naar een Kinderplan. Journal du Pédiatre Belge Vol Nr.1 23

12 ABSTRACTS 40 ème Congrès Annuel de la Société Belge de Pédiatrie ste Jaarlijks Congres van de Belgische Vereniging voor Kindergeneeskunde 2012 INDEX N Subspeciality Pages Letter Abstracts 1 Cardiology P 28 to P 30 C C 01 to C 12 2 Endocrinology P 31 to P 33 E E 01 to E 12 3 Gastro-Entero- Hepatology P 34 to P 36 H H 01 to H 14 4 General Paediatrics P 39 to P 53 A A 01 to A 58 5 Genetics and Basis Sciences P 55 to P 57 G G 01 to G 10 6 Immunology and Infectious disease P 58 to P 64 L L 01 to L 20 7 Metabolic diseases P 64 to P 64 M M 01 8 Neonatology and Intensive care P 65 to P 70 K K 01 to K 17 9 Nephrology - Urology P 72 to P 73 U U 01 to U 6 10 Neurology P 74 to P 75 B B 02 to B Nutrition P 76 to P 76 N N Oncology - Hematology P 78 to P 82 O O 01 to O Others P 83 to P 84 X X01 14 Paediatric Surgery P 85 to P 85 D D Pharmacology P 85 to P 85 J J Pneumology P 86 to P89 P P 01 to P 08 Journal du Pédiatre Belge Vol Nr.1 27

13 01. CARDIOLOGY C 01 Percutaneous revalvulation of the RVOT in Belgium Cools Bjorn, UZ Leuven Boshoff Derize, UZ Leuven Budts Werner, UZ Leuven Sluysmans Thierry, UCL Saint Luc Dessy Hughes, HUDERF Reine Fabiola Dewolf Daniel, UZ Gent Heying Ruth, UZ Leuven Kefer J, UCL Saint Luc Milani S, HUDERF Reine Fabiola Gewillig Marc, UZ Leuven INTRODUCTION Percutaneous valve implantation of the RVOT is a feasible treatment option for important valvular dysfunction. A mandatory database has been requested for reimbursement in Belgium; the scientific data are available for analysis. METHOD Nationwide multicentre interim analysis of percutaneous valve implantation in the RVOT; prospective and ongoing study. Since 2006 percutaneous Melody valve implantations are performed in Belgium. Until 12/2011 a total of 77 valves have been implanted in 4 centres. RESULTS Median age at implantation of 20.2 years (range ). Male predominance (ratio 2:1). Primary lesions : Tetralogy of Fallot (37), pulmonary atresia with VSD (5), common arterial trunk (6), TGA with VSD and PS (13), aortic valve (Ross) (12) and critical pulmonary stenosis (4). The RVOT characteristics before revalvulation : native or patch extended RVOT (7) requiring open cell stents to anchor, homograft (51), contegra conduit (3), bioprosthesis (16). The mean diameter of the conduit was 19 mm (range 10 27). Pulmonary stenosis was the main reason for revalvulation (41), pulmonary regurgitation (23) and mixed PS/ PR (13). In 70 patients (90%) prestenting was done, with a clear trend to go for 100% prestenting. In 19 patients more than one stent was necessary (max 3). A total of 36 covered and 54 bare stents were used. RV systolic pressure dropped from mean 56 mmhg (range ) to 42 mmhg (range 23-91) after valve implantation. Complications early : (2) pulmonary artery bleeding solved by covered stent implantation, (1) ischemia due to LAD compression solved by surgical removal of the stent. Complications late : stent fractures (3). No deaths. CONCLUSION Percutaneous valve replacement in conduits and homografts in the RVOT is a safe and grateful treatment with futile morbidity. Adequate prestenting to create a nice landing zone is a key feature avoiding many complications such as embolisation, recompression and fracture. Covered stents allow to expand any breackable dilatable conduit beyond the nominal value at implantation or later during growth. Open cell stents allow to hook on the dilated native outflow tracts. C 03 Relation between red cell distribution width and clinical outcome after cardiac surgery Caroline Jacquemart, Pediatric cardiology, HUDERF (ULB), Brussels Martial MASSIN, Pediatric cardiology, HUDERF (ULB), Brussels INTRODUCTION Recent studies have reported a strong association between increased red cell distribution width (RDW), a measurement of size variability of the red blood cell population, and the risk of adverse outcomes in adults with heart failure or coronary disease. We studied the association between preoperative RDW and postoperative clinical outcomes in cardiac children. METHOD Preoperative RDW was determined in 688 consecutive children undergoing cardiac surgery for congenital heart disease (CHD). Patients with recent infection, previous transfusion, renal insufficiency or age less than 3 months were not included. The relation between RDW and length of postoperative stay was tested using regression analysis. RESULTS RDW was significantly higher in patients who died during the postoperative stay (mean ±SD: ±4.69 (n=14) vs ±2.84 (n=674 survivors); p=0.004). The risk of postoperative death was 5 times higher in patients with RDW >16% than among those with RDW <16% (3.8% vs. 0.8%). RDW correlated with intensive care unit stay in the general study population (n=674, p<0.0001), as well as with total hospital stay in the subpopulation of local patients (n=235, p<0.0001). The correlation between RDW and intensive care unit stay was stronger in patients with acyanotic CHD (n=398, p<0.0001) than in those with cyanotic CHD (n=276, p=0.0007), and it was stronger in the subpopulation of patients, aged 6 months or older, with acyanotic CHD and normal hemoglobin level (n=281, p<0.0001) than in anemic patients with acyanotic CHD (n=84, p=0.025). The same strong correlation was also confirmed in large subpopulations of nonanemic patients with specific CHD such as ventricular septal defect (n=51, p=0.014), complete atrioventricular septal defect (n=50, p=0.029) or atrial septal defect (n=44, p=0.0002). CONCLUSION It is very important to predict the risk of cardiac surgery in order to better evaluate perioperative difficulties, resource management in the intensive care unit and quality of health care. RDW, a biomarker routinely reported in automated complete blood counts, is an integrative correlate of ineffective red cell production, inflammation and undernutrition. In this study, preoperative RDW was a strong predictor of adverse outcome in children undergoing cardiac surgery for congenital heart disease, especially in nonanemic patients (normal hemoglobin value and no cyanotic CHD) where it only reflects an underlying inflammatory stress that is associated with adverse outcomes. RDW can be considered as a simple, inexpensive and objective tool for the detection of high-risk children after cardiac surgery for CHD. C 02 Males with 45,X/46,XY have similar cardiovascular problems as females with Turner syndrome De Groote Katya, Universitair Ziekenhuis Gent De Wolf Daniël, Universitair Ziekenhuis Gent Vandekerckhove Kristof, Universitair Ziekenhuis Gent Panzer Joseph, Universitair Ziekenhuis Gent Sluysmans Thierry, Hospitals universitaires Saint-Luc Gewillig Marc, Universitair Ziekenhuis Leuven De Schepper Jean, Universitair Ziekenhuis Gent en Brussel INTRODUCTION Cardiovascular pathology in Turner syndrome is well described and afflicts 25 to 45% of patients. AIM We hypothesised that males with mixed gonadal dysgenesis and 45,X/46,XY (MGD) have similar cardiovascular problems as female Turner patients with 45,X/46,XY (TS). METHOD In a multicenter Belgian study, we collected 16 patients with mosaicism 45,X/46,XY who were followed at an endocrinology department: 6 females with Turner syndrome and 10 males with mixed gonadal dysgenesis. Age ranged from 1 to 38 y with a median of 13 y. Data on sexual phenotype and growth were collected from the files. Patients underwent blood pressure measurement, ECG, echocardiography and MRI (in 10/16). RESULTS Turner patients with 45,X/46,XY (age y) had normal female genitals, short height and delayed pubertal induction. All received growth hormone and hormone replacement therapy. Males with 45,X/46,XY (age 1-24y) had abnormal genitals ranging from minor abnormality (EMS12) to ambiguous genitals (EMS5). Seven received growth hormone, the other 3 were still too young. All patients had normal blood pressure. Prolonged QTc was found in 5 (3/10 MGD, 2/6 TS). Structural heart abnormalities were equally found in TS (3/6) and MGD (5/10): VSD in 1 (1/10 MGD), bicuspid aortic valve in 7 (5/10 MGD, 2/6 TS), dilation of the ascending aorta in 2 (2/10 MGD), coarctation in 1 (1/6 TS) and right arteria lusoria in 1 (1/6 TS). Males with MGD had a high incidence of bicuspid aortic valve (50%) and dilation of the ascending aorta (20%). Both cases of dilated ascending aorta were in young boys (age 10 y) who were not followed at the cardiology department. Dilation was important (28 and 30 mm). Both boys also had a bicuspid aortic valve and one had an impaired ventricular function. CONCLUSION Males with 45,X/46,XY have the same cardiovascular defects as females with Turner syndrome. Dilation of the ascending aorta can be important and might become life threatening. We advise a cardiac screening in all males with 45,X/46,XY. As in Turner syndrome, life-long follow up may be recommended. C 04 Effects of flecainide therapy on catecholaminergic polymorphic ventricular tachycardia Caroline Jacquemart, pediatric cardiology, HUDERF (ULB), Brussels Martial MASSIN, pediatric cardiology, HUDERF (ULB), Brussels INTRODUCTION Current recommendations for therapy of catecholaminergic polymorphic ventricular tachycardia (CPVT) include ß-blockade and implantable cardioverter defibrillators (ICD). Patients may experience recurrent arrhythmias, ICD shocks and sudden death despite optimal medical therapy. It was recently shown that flecainide directly targets the molecular defect in CPVT by inhibiting premature Ca++ release. This study evaluated the efficacy and safety of flecainide in our CPVT children. METHOD We collected data from the genotype-positive CPVT children started on flecainide in our tertiary pediatric cardiac center. RESULTS The first case is a boy who was diagnosed with CPVT at 3 years of age. He was managed with nadolol (75 mg/m² BSA daily) with no further occurrence of syncope. At age 11, we noted bigeminal ventricular premature beats and couplets during exercise testing and one episode of sustained ventricular tachycardia on Holter recording despite nadolol titrated to maximum tolerable dose, so that an ICD was implanted. A few months later, inappropriate shocks were delivered due to episodes of sinus tachycardia. Holter monitor showed multiple episodes of sinus tachycardia and non-sustained ventricular tachycardia. Flecainide (100 mg = 4 mg/kg BW daily) was added to the ß-blocker regimen. At 18-months follow-up, no ICD shock occurred and repetitive Holter recordings and exercise testings excluded sinus tachycardia and arrhythmias. The second case is a girl who was diagnosed with CPVT at 8 years of age. At age 13, we noted bigeminal ventricular premature beats and couplets during exercise testing and on Holter recording despite nadolol titrated to maximum tolerable dose. Flecainide (100 mg = 2 mg/kg BW daily) was added to the ß-blocker regimen. At 6-months follow-up, the patient has complete suppression of exercise-induced ventricular arrhythmias and Holter recordings also excluded arrhythmias. CONCLUSION Recent experimental evidence suggested that flecainide can suppress CPVT. The presented cases support that novel strategy and show that flecainide is effective in preventing exercise-induced ventricular arrhythmias and also inappropriate shocks in children with CPVT. Tijdschrift van de Belgische Kinderarts Vol Nr.1 28

14 Abstracts C 05 Emerging MRI techniques in cardiovascular research Vanhoutte Laetitia, Esfahani Hrag, Feron Olivier, Balligand Jean-Luc, Gallez Bernard, Moniotte Stéphane, Cliniques Universitaires Saint-Luc (UCL), Brussels. INTRODUCTION In cardiovascular research, imaging of small animals with high-frequency cardiac ultrasound is considered as a standard approach. However, its limited accuracy and reproducibility requires large sample sizes. The recent development of high-field MRI offers the potential to better define structural and functional changes in murine models of cardiovascular diseases. AIM The purpose of our work was to evaluate left ventricular systolic function in mice using a 11.7 Tesla MRI scanner. METHOD A total of eight C57Bl/6 wild-type mice were studied, including 3 animals having undergone a surgical transverse aortic constriction (TAC). During the entire MRI scanning, mice were anaesthetized with Isoflurane 1-3%, in a temperature-controlled setting. Scans were prospectively gated for electrocardiogram (ECG) and respiration. Imaging was performed on a 11.7 Tesla Bruker MR scanner. Cardiac scout images were obtained in the conventional planes (2-chambers and 4-chambers) with a tripilot sequence. A FLASH-cine sequence was applied to produce a stack of short-axis images covering the entire ventricles, perpendicular to the LV long-axis. The left ventricular (LV) systolic function was assessed from the full stack of short-axis cine images using the Segment software (Medviso, Sweden) by tracing epicardial and endocardial borders, including papillary muscles. The following volumes (µl) were determined: end-diastolic (LVEDV), end-systolic (LVESV) and stroke volume (LVSV). LV ejection fraction (LVEF, in %) and LV mass (mcg) were subsequently deduced. RESULTS Accurate quantitative data were obtained in all animals. The mean LVEDV, masses and EF obtained in wild-type mice were 29.5±4.5 µl, 43.5±5.5mg and 68±12 % respectively. As expected, the mice with TAC presented significant LV hypertrophy, with a LV mass of 80.5mg±7.5mg (p<0.05), but unchanged LVEDV and EF. When compared to high-frequency ultrasound, preliminary data showed that inter- and intra-experiment reproducibility was likely better in MRI (e.g. almost identical LVEDV, LV masses and LV EFs (n=2-3)). Using light anesthesia with isoflurane, we also showed that mice survive the scanning procedure, and can be re-studied later on when appropriate. CONCLUSION High-field cardiac MRI is a very promising tool to assess LV systolic function and hypertrophy in mouse models of cardiovascular diseases. Although the development of other sequences already used in the clinical field (i.e. tagging, velocity mapping or contrast-enhanced sequences) is still under investigation, we are confident that this technique will soon allow a noninvasive and reproducible approach of diastolic function and myocardial viability, as needed in translational cardiovascular research. C 07 Self-expanding stent Sinus superflex Visual to create an unrestrictive atrial communicatio Cools Bjorn, UZ Leuven Heying Ruth, UZ Leuven Boshoff Derize, UZ Leuven Eyskens Benedicte, UZ Leuven Louw Jacoba, UZ Leuven Gewillig Marc, UZ Leuven INTRODUCTION In infants with complex CHD an unrestrictive atrial septal communication may be necessary. Balloon septostomy and dilation is however sometimes inefficient in creating an adequate atrial shunt; blade septostomy requires a big sheath and balloon expandable stents may slide and embolise. METHOD Procedure done in 6 infants requiring a stable atrial shunt after failed Rashkind or thick atrial septum predicting poor result. Access through a 5 Fr sheath in the femoral vein. A stiff coronary wire is preferably curled in left atrium; balloon interrogation of atrial septum with a soft balloon (10 mm Tyshak, Numed); optimizing the beamer angulation (LAO-cranial) perpendicular to the atrial septum; positioning of Optimed Sinus superflex Visual 5F 10/20 mm; opening the distal part in the LA, hand injection through sidearm of the sheath to delineate relationship with the septum; pull back if required; opening the stent completely; post dilation if required. Low dose acetylcalicylic acid is given at 2 mg/d. RESULTS 6 infants age median 3.7 months (range ); CHD: complex TGA (1), tricuspid atresia (1), small left heart (4). In all patients the stent was successfully deployed; the anti-jump technique and visual markers allowed perfect placement; the open cell design allows the stent to hook at the septum, preventing sliding or embolisation. In all patients the stent provided an adequate unrestrictive atrial communication until the next surgical step [switch (1) or Glenn (5)]. The timing of the next operation was elective and not imposed by inadequate atrial shunt. All stents could easily be removed by the surgeon. No evidence of any thombo-embolic event during a follow-up of 4.7 months (range ). The stent was firmly attached to the atrial wall. CONCLUSION Sinus superflex Visual stent delivered safely through a 5F sheath allows to obtain an adequate atrial connection for several months in infants with complex congenital heart disease. C 06 Intracardiac Tumors in Neonatal Period: Report of 2 Cases of Myxoma Hubrechts Jelena, Faculté de médecine, UCL, postulante pédiatrie Stéphane Moniotte, Cliniques universitaires St-Luc, département cardiopédiatrie Karlien Carbonez, Cliniques universitaires St-Luc, département cardiopédiatrie Cathérine Barrea, Cliniques universitaires St-Luc, département cardiopédiatrie INTRODUCTION Cardiac tumors are uncommon in children. Primary benign tumors are more frequent than malignant, and during perinatal period, rhabdomyomas are the most frequent tumors. Myxomas are exceedingly rare. METHOD We report 2 cases of myxoma, diagnosed during fetal life and early neonatal period. RESULTS A 29-year-old G1P0 woman was referred for evaluation of a heart mass detected at 21 weeks gestation. At fetal echocardiogram, the tumor appeared unique (7x5mm) and was localized at the apex of the right ventricle with a broad basis. The echogenicity was homogenous without calcifications and slightly hyperechogeneous compared to the neighbouring myocardium. According to power-doppler, the tumor wasn t highly vascularized. The rest of the cardiac anatomy was normal, with normal biventricular function and sinus rhythm. At 27.4 weeks, the tumor was lightly increased with as only repercussion a mild tricuspid valve regurgitation. A possible multi-organic damage was excluded by ultrasound and fetal MRI. Fetal karyotype was normal and there was no deletion or duplication in both genes for tuberous sclerosis. A 4.4kgs full-term boy was born uneventfully. Clinical examination and ECG were normal. Postnatal echocardiogram defined the tumor as a sessile multilobed mass in the apex of the right ventricle. At 1-month age, MRI sequences led to the diagnosis of an isolated myxoma (17x11x12mm) without hemodynamic repercussion. Our second case was diagnosed in a 2-week-old girl, born full term with 3.5kgs, without any personal or familial history. During a check-up for cyanotic breath-holding-spells, an hyperechogeneous, homogeneous, sessile cardiac mass was shown in the apex of the left ventricle. The biventricular function, cardiac anatomy and electrical activity were normal. MRI suggested the etiology of a myxoma of oval form, 8x9x3mm. In both cases, conservative approach was adopted because of the tumor stability and the absence of symptoms, arrhythmia and hemodynamic compromise. Our two patients are currently well at respectively 5-month and 3-year follow-up. CONCLUSION Myxomas are very rare tumors in the pediatric age. They may however already be present antenatally and should be part of the differential diagnosis of cardiac mass. MRI may confirm the diagnosis. Surgical abstention is conceivable, especially when the patient is asymptomatic. C 08 Sternotomy As a Safe Alternative Access for Interventions In Low Birth Weight infants Cools Bjorn, Boshoff Derize, Heying Ruth, Rega Filip, Meyns Bart, Gewillig Marc UZ Leuven INTRODUCTION Low birth weight infants with congenital heart defect remain a therapeutic challenge. Prolonged administration of prostaglandin has many disadvantages; the surgical options yield high morbidity and mortality; vascular access for percutaneous interventions may seriously damage vessels required for later procedures. We used sternotomy for hybrid direct cardiovascular access in low birth weight infants to perform a bail out stenting procedure. METHOD 4 patients: hybrid suite; sternotomy; A/ 3 patients (weight 1620, 2190 & 2630 g) extreme Fallot pulmonary atresia with hypoplasia pulmonary trunk (2-3 mm); purse string on the right ventricle; 2 vascular clips were placed as radio-opaque markers: one at the pulmonary valve annulus, one at the puncture site within the purse string. Double needle technique: 2 identical 21G needles: 1 needle as a reference adjacent on the surface of the RVOT to determine precise puncture depth and direction; the other needle was used to perform the punction. The punction was performed under direct vision in two motions: first access perpendicular to the surface for mm into the right ventricular cavity with free blood, second angulation of the needle towards and advancement through the atretic outflow tract into the pulmonary trunk. A 0,014 coronary wire into the pulmonary arterial branch; needle exchanged for a 4 Fr short sheath; angio by mini 1cc injections through side-arm; a premounted coronary stent was deployed into the RVOT 5/16 mm to obtain an intracardiac Sano shunt ; sheath and clips removed. B: 1 patient (900g) with critical aortic coarctation and open duct under PG; purse-string on the ascending aorta with radiomarker clip; puncture with 21 gauge needle; 0,014 wire into the descending aorta; needle exchanged for a 4 Fr short introducer sheath with the tip at the aortic cross; angios through side-arm. A 3/8 mm coronary stent deployed into the aortic isthmus (from LSA until beyond coarctation); sheath and clip removed, arterial duct clipped. RESULTS Fallot: adequate palliation with good anterograde flow to the pulmonary arteries (sat> 92%). After a median of 3 months additional transvenous stenting required in all patients because of progressive muscular infundibular stenosis. Two patients evolved to full repair at 5 months; one patient with multiple hilar stenoses requires additional percutaneous procedures through the stented RV outflow tract. Coarctation: good aortic flow, stent resected at 4 months. No associated morbidity as frequently seen in premature infants with CHD. CONCLUSION Medial sternotomy can be a safe alternative access for bail out transluminal cardiac interventions in low birth weight infants, allowing conventional repair at bigger weight. The technique with 2 identical needles and radio-opaque markers markedly simplifies the hybrid procedures. Journal du Pédiatre Belge Vol Nr.1 29

15 C 09 Off-label use of percutaneous pulmonary valved stents in the RVOT. Boshoff Derize, UZ Leuven Cools Bjorn, UZ Leuven Heying Ruth, UZ Leuven Troost Els, UZ Leuven Budts Werner, UZ Leuven Gewillig Marc, UZ Leuven INTRODUCTION Percutaneous pulmonary valve implantation is now considered feasible and safe in selected patients with dysfunctional RVOT conduits. The native RVOT, smaller conduits (less than 16mm) and the relatively large RVOT with dynamic outflow aneurysms, as commonly seen with trans-annular patch repair of the RVOT, are considered off-label use for percutaneous valve implantation. Modification of the device design to make it suitable for patients with aneurismal RVOT is being explored but clinical reports are so far limited. AIM To report the safety and feasibility of extended (off-label) application of percutaneous pulmonary valve implantation in patients with RVOT dysfunction. METHOD Design: Retrospective analysis of prospectively collected data. Setting: Tertiary pediatric and adult congenital heart cardiac centre. Patients and Methods: Off-label use was defined as valve implantation in patients with a native RVOT (pulmonary valve or patch), RVOT conduits smaller than 16mm or larger than 24mm or the final valve diameter ;110% of the nominal conduit diameter. Successful valve implantation was defined as sufficient relief of RVOT obstruction (if present) and valve competence. RESULTS Twenty Melody valves and 2 Sapien valves were successfully implanted in 22 patients at a mean age of 17 years (range years). Pre-stenting was performed in 21 patients 4.8 months (range ) before valve implantation (14 covered stents; 12 bare stents). In 10 patients valves were implanted in the native RVOT after transannular/ infundibular patch (n = 8) or pulmonary valvoplasty (n = 2). Mean diameter of the native RVOT was 18.8mm (range 14 24) and mean final valve diameter was 22mm (range 16 26). Twelve patients had their valves implanted in existing conduits ranging from 10 to 20mm in size (mean 16mm) with a mean final valve diameter of 20mm (range final size 18 22; increase of diameter from nominal 4mm (range 2-6 mm)). The implantations were uneventful and all patients were hemodynamically stable throughout the procedure. CONCLUSION Percutaneous pulmonary valve implantation is safe and feasible even in patients with unfavourable anatomy according to traditional protocol. Creating an adequate landing zone by pre-stenting is recommended. Larger studies are needed to identify the ideal timing for pre-stenting of the native RVOT. C 11 Interventional closure of ventricular septal defects using the Amplatze Duct Occluder II Heying R, Cools B, Boshoff D, Eyskens B, Louw J, Gewillig M, Department of Pediatric Cardiology, KU Leuven, Belgium INTRODUCTION Interventional closure of perimembranous ventricular septal defects (pmvsds) which are partially closed by an aneurysm remains controversial concerning AVblock. We report our experience using the soft Amplatzer Duct Occluder (ADO) II for pmvsd closure. METHOD In five patients a pmvsd was closed between 4/2010 and 12/2011; age: 6 months 8.6 years, pmvsd size 4-10 mm, formation of an aneurysm left an effective opening of mm. Distance of pmvsd to aortic valve 3-5 mm. No patient had aortic insufficiency. RESULTS Hemodynamic measurements: left to right shunt Qp/Qs /1; normal pulmonary artery pressures. Technical procedure of VSD closure was done via the classically described way forming a loop from the left ventricular side to the right side. Final closure was performed from the right ventricular side. The following devices were placed: ADO II 4-4 mm in 1 patient, 5-6 mm in 2 patients, 6-6 mm in 1 patient and ADO II AS 5-4 mm in 1 patient. 24 hours post closure we visualised no residual shunts in all patients. None of the patients had aortic insufficiency or a remarkable AV valve insufficiency. No AVblock occurred. In 1 patient the right disk was deployed at the right atrial side. No tricuspid valve dysfunction occurred, device was left in place. One patient who was known with an intermittent AV block I pre catheter presented with a transient AV dissociation post VSD closure and a left anterior hemiblock and right bundle branch block 1 month after closure without any clinical symptoms. Total FU time was 1 20 months. CONCLUSION In our experience of 5 patients, pmvsds with aneurysm formation can be closed with a good result using the Amplatzer Duct occluder II. A total closure rate of the pmvsds could be reached. To avoid interference of the device with the tricuspid valve or a position of the right disc towards the right atrium, we recommend choosing the minimal device length needed. We might speculate that the ADO II device seems softer and less traumatic to the His bundle. Long term follow-up has to verify patency of AV-conduction. C 10 Evaluation of performance of various QT correction formulae in infants and children Benatar, Abraham, De Wilde Hans, UZ Brussel Dewals Wendy, UZ Brussel Decraene Tine, UZ Brussel Feenstra Arjen, UZ Brussel Benatar Abraham, UZ Brussel INTRODUCTION Several formulae have been used to correct QT interval for heart rate. These are however known to over- or under-correct the QT interval. Bazett is not the ideal formula for children. AIM Study performed to ascertain which formula best corrected for heart rate in children of all ages METHOD We enrolled a cohort of 600 healthy children. While in a quiet state, a digital 12 lead electrocardiogram (50 mm/second) was recorded and stored. Subsequently, the QT and RR intervals were measured digitally from lead 2. The QT interval was corrected for RR interval by applying 6 formulae (Bazett, Fridericia, Framingham, van de Water, Hodges and Tabo). Linear regression equations for the corrected QT (QTc) intervals against RR intervals were obtained by the method of least squares and the slope and R square (R2) of equations were calculated. When both the slope and R2 were close to zero, the formula was judged to eliminate the effect of heart rate on QT interval. RESULTS Mean age: 2,1 years, SD + 3,5 (range 0-18 years). Mean QT ms, RR mean ms; QTc Bazett ms; QTc Fridericia ms, QTc Tabo ms; QTc Framingham ms; QTc Hodges ms, QTc Van De Water ms. Linear Regression plots of QTc against RR intervals: QTc Bazett slope 0.06, R2 0.16; QTc Fridericia slope 0.053, R2 0.15; QTc Hodges slope -0.07, R ; Qtc Tabo slope 0.017, R ; QTc Framingham slope 0.065, R2 0.3; QTc Van de Water slope 0.13, R CONCLUSION The many QT correction formulae available attests to the importance of adjusting QT interval for heart rate particularly in infants with increased basal heart rate. From linear regression analysis of the QTc interval against RR interval, this study illustrates that, of the published formulae, Tabo s formula best corrected the effect of RR on QT interval, even in infants, and showed superior dissociation of the QTc interval from RR interval (least slope and lowest R2). Further evaluation is in progress. C 12 Arteria lusoria and premature closure of the ductus arteriosus: a rare coincidence? Anneleen Dereymaeker 1, Sophie Van Haesebrouck 1, Christine Vanhole 1, Karel Allegaert 1, Gunnar Naulaers 1, Bjorn Cools 2, Marc Gewillig 2, 1-Neonatology Unit, University Hospitals Leuven - 2-Department of Pediatric Cardiology, University Hospitals INTRODUCTION Fetal dysfunction of the ductus arteriosus causes right heart failure due to elevation of the ventricular afterload and causes volume overload of the pulmonary circulation, leading to persistent pulmonary hypertension. The clinical outcome of this entity ranges from mild respiratory symptoms at birth to hydrops foetalis and neonatal death. Premature ductal constriction is often ascribed to maternal administration of prostaglandin synthetase inhibitors, such as indomethacin or other NSAIDs, but may also occur in the absence of maternal drug ingestion. RESULTS We report on a female fetus which was diagnosed at 38 weeks of gestation with severe right ventricular hypertrophy, and a hypocontractile, dilated right ventricle with tricuspid regurgitation on prenatal ultrasound. Urgent cesarean section was performed on the suspicion of complete prenatal closure of the ductus arteriosus. Maternal history was negative with respect to drug ingestion or congenital anomalies. At birth, complete closure of the ductus arteriosus and significant right ventricular hypertrophy (6 mm) were confirmed on cardiac ultrasound. Despite oxygen administration, the patient exhibited mild cyanosis related to pulmonary hypertension, temporarily requiring treatment with inotropics and vasopressors. Furthermore, inspiratory stridor was noted, when agitated or crying. With increasing oral feeding, she presented with excessive crying, regurgitation and a nutrition associated pain syndrome. Gastroesophageal reflux disease was suspected, but anti-reflux therapy was proven unsuccessful. Further investigation by means of a Barium esophagram and chest CT showed a posterior impression on the middle thoracic segment of the esophagus, which likely relates to an arteria lusoria (aberrant right subclavian artery), causing a mass effect and dysphagia. Therefore, nasogastric tube feeding was started, with partial relieve of the symptoms. Further investigations, including heart catheterization and gastroscopy, are pending. CONCLUSION Premature closure of the ductus arteriosus in the absence of maternal NSAID ingestion, has been reported in association with altered fetal hemodynamics, induced by congenital cardiovascular anomalies, such as tetralogy of Fallot or truncus arteriosus. Thus far, spontaneous foetal closure of the ductus arteriosus has not been reported in association with an aberrant right subclavian artery or arteria lusoria. Further investigations are undertaken to show a hemodynamic or anatomic link between these rare entities. Moreover, neonatal signs of an arteria lusoria are rare and usually involve respiratory distress or stridor, whereas neonatal dysphagia, as in the present case, is uncommon and requires further investigation. Tijdschrift van de Belgische Kinderarts Vol Nr.1 30

16 Abstracts 02. ENDOCRINOLOGY E 01 Bone geometry and density at the distal radius in female adolescents with anorexia nervosa Inge Roggen, UZ Brussel, Vrije Universiteit Brussel Jesse Vanbesien, UZ Brussel, Vrije Universiteit Brussel Inge Gies, UZ Brussel, Vrije Universiteit Brussel Ursula Van Den Eede, UZ Brussel, Vrije Universiteit Brussel Annick Lampo, UZ Brussel, Vrije Universiteit Brussel Olivia Louis, UZ Brussel, Vrije Universiteit Brussel Jean De Schepper, UZ Brussel, Vrije Universiteit Brussel INTRODUCTION Osteopenia is a well-known complication of anorexia nervosa (AN) in older adolescents and adults, especially in those with a long duration of the disease and a severe underweight. AIM We investigated whether young premenarchal girls with AN have similar risk factors for a disturbed bone growth and mineralization. METHOD Twenty-four female premenarchal AN patients as well as 24 age and height matched female controls underwent a peripheral quantitative computed tomography (pqct) scan at the distal radius of the non-dominant arm. RESULTS The age of the AN subjects ranged between 11.8 and 18.5 years. Mean (± SD) weight loss was 22.3 ± 9.3%, which occurred during the preceding 7.8 ± 3.9 months. Mean weight z-score (-2.19 ± 1.40 vs ± 0.90; p<0.0001) as well as mean BMI z-score (-2.28 ± 1.09 vs ± 0.83; p<0.0001) was significantly lower in AN patients, whereas height z-score (-0.36 ± 1.02 vs ± 1.02; p=0.1) was comparable at the moment of examination. AN patients had a significantly lower mean total bone crosssectional area (CSA) (294.9 ± 31.5 vs ± 54.4 mm²; p=0.004) and periosteal circumference (60.8 ± 3.2 vs ± 5.0 mm; p=0.003) in comparison with the control subjects. Trabecular bone mineral density (186.1 ± 32.2 vs ± 30.9 mg/cm³; p=0.06) and bone strength index (30.1 ± 9.0 vs ± 10.5 mg/mm4; p=0.1) were also lower, but at borderline significance. Total bone CSA z-score correlated with duration of the weight loss (r=0.43; p=0.03), but not with the age and BMI z-score at diagnosis and the severity of the weight loss was found. None of the other bone parameters correlated with any of the disease related factors. CONCLUSION In female premenarchal AN patients the rapidity but not the severity of the weight loss before diagnosis affects the periosteal bone apposition, which is more disturbed than the bone mineralization at the distal bone end of radius. E 02 Septo-optic dysplasia: retrospective review of HUDERF cohort E Boros, Pediatric Encocrinology Unit, HUDERF, ULB C Brachet, Pediatric Endocrinology Unit, HUDERF, ULB S Tenoutasse, Pediatric Endocrinology Unit, HUDERF, ULB C Heinrichs, Pediatric Endocrinology Unit, HUDERF, ULB INTRODUCTION Septo-optic dysplasia (SOD) is a rare congenital anomaly, more common in infants born to younger mothers. Classically the diagnosis can be made when two or more features of the classical triad (optic nerve hypoplasia, pituitary hormone abnormalities and midline brain defects) are present. Almost 40% of SOD patients do not present hormonal abnormalities. AIM Our aim was to assess the frequency and timing of hormonal deficiencies in patients with SOD. METHOD We retrospectively studied the patients with SOD followed in the pediatric endocrinology unit of HUDERF over the last 23 years. RESULTS Eleven SOD patients where diagnosed. The mean age of the mothers at the time of delivery was 25.6 year (range: years). The optic nerve hypoplasia diagnosed mostly in the first 9 months of life (72 %) was bilateral in nine patients and unilateral in two patients. At magnetic resonance (MRI) of the brain, hypoplasia of anterior pituitary was found in seven patients, ectopic posterior pituitary without associated diabetes insipidus in four patients, while posterior pituitary was not found in three patients with diabetes insipidus. Growth hormone deficiency (GHD) was the most frequently associated endocrine deficiency (8 of 11), followed by central hypothyroidism (6 of 11), diabetes insipidus (3 of 11) and ACTH deficiency (3 of 11). None of the patients had isolated GHD. Central hypogonadism was assessed in 6 patients and 2 were deficient. Three patients (27 %) had no hormonal anomalies. When present, central diabetes insipidus was diagnosed in the first year of life shortly after the optic nerve hypoplasia diagnosis. GHD was diagnosed at a mean age of 4.7 years and at the mean height SD of -2.8 DS. It was the first hormonal deficiency in 2 cases and it was diagnosed together with other deficiencies in 3 cases. In one case, ACTH deficiency appeared 6 years after GHD. CONCLUSION Every patient with SOD needs a continuous endocrine follow-up, because a progressive loss of endocrine function over time is observed. GHD is the most frequently observed endocrine deficit. Diabetes insipidus, when present, was diagnosed early in life. E 03 Quality improvement and Epidemiology in Paediatric Diabetes in Belgium Doggen K, Wetenschappelijk Instituut Volksgezondheid, Brussel Debacker N, Wetenschappelijk Instituut Volksgezondheid, Brussel Van Casteren V, Wetenschappelijk Instituut Volksgezondheid, Brussel On Behalf Of The IQECAD Study Group, INTRODUCTION The importance of quality of care monitoring, allowing identification of shortcomings in care, is increasingly recognized among health care practitioners. It is especially important among children with chronic illness, such as type 1 diabetes, as high care quality in childhood might prevent long-term complications. Several national and international systems of audit and feedback have been established, aimed at improving care quality in hospital-based multidisciplinary paediatric diabetes centres (HBMC). AIM To study and improve the processes and outcomes of care by implementing a system of audit and feedback in Belgian HBMCs. To identify risk factors of poor care delivery and outcomes. METHOD In 2009, twelve HBMCs transmitted data on a random sample (N=976) of type 1 diabetic patients aged 0-18 years (representing about one third of all Belgian patients) to the Scientific Institute of Public Health (IPH) using an on-line secured questionnaire. Data pertained to demographic and clinical characteristics, as well as process of care completion and outcomes of care in Outcomes were studied for patients with diabetes duration exceeding 1 year (N=861). Quality of care indicators were defined using international recommendations. RESULTS Following the audit, the IPH provided each HBMC with a quality indicator feedback report of the individual scores as well as caterpillar plots allowing the comparison of individual scores to anonymised scores of the other HBMCs (benchmarking). Next, a global description of care delivery and barriers to successful outcomes was undertaken (available at: Nearly all patients ( ;95%) underwent determination of glycated haemoglobin (HbA1c) and BMI. Screening for retinopathy (55%) and microalbuminuria (73%) was less frequent, but rates increased with age and diabetes duration, consistent with the observed absence of complications in young patients. Median HbA1c was 7.7% (interquartile range: %) and increased with age and insulin dose. HbA1c was higher among patients on insulin pump therapy. Median HbA1c significantly differed between centres (from 7.3% to 8.2%). Incidence of severe hypoglycemia was 30 per 100 patient-years. Admissions for ketoacidosis had a rate of 3.2 per 100 patient-years. Most patients lived with both biological or adoption parents (77%) and had at least one parent of Belgian origin (78%). Patients not living with both biological or adoption parents had higher HbA1c and more admissions for ketoacidosis. Parents country of origin was not associated with processes and outcomes of care. CONCLUSION The IQECAD Study Group successfully implemented a system of audit and feedback in Belgian HBMCs. Future cycles will indicate whether feedback leads to quality improvement. At baseline, outcomes of care ranked well compared to other European countries, while screening rates for complications were intermediate. Centre variation in HbA1c remained unexplained. Outcomes were associated with family structure, highlighting the continuing need for strategies to cope with this emerging challenge. E 04 A search for spermatogenesis by spermaturia and testicular biopsy in 47,XXY adolescents Gies Inge, UZ Brussel, Departement Pediatrie Van Saen Dorien, BITE, Vrije Universiteit Brussel Anckaert Ellen, UZ Brussel, Departement Klinische Chemie Goossens Ellen, BITE, Vrije Universiteit Brussel Tournaye Herman, UZ Brussel, Center for Reproductive Medecine De Schepper Jean, UZ Brussel, Departement Pediatrie en Endocrinologie INTRODUCTION Although pubertal development starts normally in boys with Klinefelter Syndrome (KS), a testicular arrest and fibrosis associated with a profound germ cell loss occurs in mid puberty. We investigated if it is possible to detect spermatogenesis by either spermaturia, sperm examination after electro-ejaculation and testicular biopsy in pubertal KS adolescents when clinical or hormonal signs of testicular fibrosis are observed. METHOD In 7 non-mosaic 47,XXY adolescents, aged between 10 and 16 years at start of the study, Tanner staging, testicular growth, serum inhibin B and FSH, and spermaturia were assessed every four months up to the moment of the biopsy. When testicular volume was 6 ml or more, spermaturia in 5 consecutive first-voided morning urine samples was investigated. When no further testicular growth was observed and a decreasing serum inhibin B concentration and/or an increasing FSH concentration (at least above 10 mu/l) was documented, and/or when initial serum inhibin B concentration was below the lower reference value, testicular biopsy was performed. RESULTS Despite an increasing testis volume in the youngest and a Tanner staging of more than 3 in the oldest patients, no spermaturia was observed. In only 2 patients, serum inhibin B increased gradually, while in all the others a rather rapid, but variable decline was observed, occurring at very different ages. In none of 7 KS adolescents, spermatozoa were observed after electroejaculation. While no spermatocytes or spermatids were found at microscopic examination of single biopsies, spermatogonia were identified in 4 subjects, of who 3 had a measurable inhibin B. Massive fibrosis and hyalinization was observed in all boys at testicular tissue sampling, except in the youngest boy (aged 13 yr). CONCLUSION No spermatogenesis was observed in non-mosaic 47,XXY adolescents either by spermaturia, electroejaculation or testicular biopsy. Serum inhibin B levels varied greatly between subjects and did not fully correlate with the presence of spermatogonia, which were found in half of the studied KS adolescents. More data are needed to elucidate the potential role of testicular tissue cryopreservation in KS adolescents. Journal du Pédiatre Belge Vol Nr.1 31

17 E 05 TRANSIENT HYPOGLYCEMIA INDUCED HEMIPARESIS IN PEDIATRIC DIABETIC PATIENTS: A CASE SERIES Toelen, Jaan, M. Gielen, KU Leuven W. Asscherickx, KU Leuven K. Casteels, KU Leuven INTRODUCTION Acute hemiplegia is a rare and infaust symptom in a pediatric population. It has a broad differential diagnosis, ranging from infectious, neurological, traumatic to metabolic origins. In children with diabetes mellitus (DM) it can be the presenting symptom of a common metabolic disturbance. We present three cases of transient hemiplegia in pediatric diabetes patients. RESULTS A 3y old boy, with a 2y history of type 1 DM, presented to the pediatric outpatient clinic with a Shigella gastro-enteritis. His mother also mentioned an episode of acute hemiplegia the night before. She found him in the bathroom with a left sided paresis of both arm and leg, at that time his glycemia was 48 mg/dl. She administered him a sugar containing beverage after which his symptoms subsided. He was admitted for his gastroenteritis and during hospitalization a CT scan of the brain was obtained which showed no abnormalities. A 9y old girl, with a 2m history of type 1 DM, was transferred to our unit because of an acute hemiplegia. When waking up after an afternoon sleep, a paresis of the right arm and leg was noticed. Her glycemia at that time was 54 mg/dl. She was administered sugar and glucagon by the emergency services. Upon arrival at our unit only a mild paresis in her right arm was still present. After normalization of her glycemia, all symptoms subsided. A 13y old boy, with a 6y history of type 1 DM, presented to the pediatric diabetes clinic with the history of two nocturnal episodes of paresis of the right arm and headache. On both occasions he woke up and noticed the paresis, at those times he was hypoglycemic. Upon normalization of his glycemia, his paresis subsided. CONCLUSION Acute hypoglycemia-induced hemiplegia is a rare clinical manifestation of diabetes patients described in sporadic case reports or case series. The paresis is often present on the right side, can be flaccid or spastic and occurs in the majority of cases when the patient wakes up. The pathogenesis of this paresis remains elusive, current theories include selective neuronal vulnerability to hypoglycemia and variability in the cerebrovascular circulation. Diagnostic tests, such as MRI or angiography, are universally normal; the EEG often reveals unilateral slow-wave activity. The symptoms normally subside within two hours of onset and after normalization of the blood glucose level. Long-term prognosis is invariably excellent. E 07 Rémission (temporaire?) d un diabète de type 1 chez une patiente présentant un syndrome néphrotique Messaaoui, Anissa, Clinique de diabétologie, HUDERF Sylvie Tenoutasse, Clinique de diabétologie, HUDERF Pernille Hansen, Hôpital Ambroise Paré Harry Dorchy, Clinique de diabétologie, HUDERF INTRODUCTION Diabète de type 1 et syndrome néphrotique peuvent coexister avec une probabilité d environ 1/ d enfants. Histoire Clinique Juin 2009, une patiente de 8 ans est hospitalisée pour diabète inaugural : hyperglycémie, glycosurie et HbA1c à 16 %. Antécédents Syndrome néphrotique cortico-sensible en janvier Corticothérapie durant 5 mois avec rémission. Récidive, en janvier 2009, retraitée par corticothérapie et rémission. En juin 2009, le sevrage des corticoïdes est en cours (prednisone 0,2 mg/kg/j). Le traitement associé consiste en calcium, vitamine D, régime sans sel et protection gastrique. Examens complémentaires le c-peptide est dosable à 0,4 µg/l témoignant d une faible sécrétion résiduelle d insuline; le profil lipidique est altéré à trois reprises : cholestérol total à 302 mg/dl, HDL cholestérol à 29 mg/dl, LDL cholestérol à 213 mg/dl et triglycérides à 1580 mg/dl; les anticorps β;-insulaires sont positifs (IAA 0,6 %, ICA 25 unités JDF, GADA 24,2 %, IA2A 13 %) ; le typage HLA est HLA DQA3-DQB3.2/DQA4-DQB2 (risque relatif de diabète de type 1 : 31). Traitement et évolution Diabète : sous une insulinothérapie d un mélange individualisé d insulines (0,7 unités/kg/j), les glycémies sont contrôlées et l HbA1c normalisée. Dès 2011, les doses diminuent parallèlement au sevrage des corticoïdes. L insuline est arrêtée en octobre L HbA1c reste normale. Syndrome néphrotique : lors de l hospitalisation de juin 2009, le syndrome néphrotique récidive. La prednisone est augmentée à 60 mg/jour. La patiente présente ensuite plusieurs rechutes lors de la décroissance du traitement. Echec du lévamisole. Ajout de la cyclophosphamide en décembre 2010, avec sevrage des corticoïdes. Tous les traitements sont arrêtés en juillet 2011, sans nouvelle rechute. La corticothérapie a entraîné stagnation staturale et aspect cushingoïde avec hirsutisme. Depuis son arrêt, la croissance a repris. Altération des lipides: malgré un régime pauvre en graisses, l hyperlipidémie persiste et un traitement par atorvastatine est instauré. A l arrêt des corticoïdes, le traitement est interrompu et le profil lipidique reste normal. CONCLUSION Jeune fille présentant un diabète de type 1 auto-immun, probablement déclenché par la prise de corticoïdes, sans insulinothérapie actuellement. Antécédents de syndrome néphrotique avec hyperlipidémie. Discussion 1) Le syndrome néphrotique est exceptionnellement présent chez les patients diabétiques. Cette association pourrait être due aux troubles immunitaires responsables de ces deux pathologies. A contrario, la néphropathie diabétique n apparaît qu après des années d un diabète très mal équilibré. 2) La corticothérapie, traitement du syndrome néphrotique chez les jeunes patients, n est pas dénuée d effets secondaires : non seulement stagnation staturale, aspect cushingoïde, altération du profil lipidique, gastrites mais aussi déclenchement d un diabète de type 1. 3) La présence d anticorps β;-insulaires confirme le diabète de type 1. Cette patiente doit être suivie régulièrement car la «rémission» du diabète risque malheureusement de n être que temporaire. E 06 Spiermassa en -kracht bij met groeihormoon behandelde kinderen: een transversale studie K. Huysentruyt, Dienst Pediatrie, UZ Brussel, Brussel I. Gies, Dienst Pediatrie, UZ Brussel, Brussel J. Vanbesien, Dienst Pediatrie, UZ Brussel, Brussel P. Jacobs, Faculteit Lichamelijke Opvoeding en Kinesitherapie, Vrije U P. Clarys, Faculteit Lichamelijke Opvoeding en Kinesitherapie, Vrije U C. Watthy, Dienst Kinesitherapie, UZ Brussel, Brussel J. De Schepper, Dienst Pediatrie, UZ Brussel, Brussel INTRODUCTION Groeihormoon (GH) wordt in een supra-fysiologische dosis toegediend bij kinderen met een kleine gestalte ten gevolge van het Turner syndroom en als gevolg van een afwezige postnatale inhaalgroei bij small for gestational age (SGA) geboren kinderen. In tegenstelling tot het groeibevorderend effect, is de invloed van de GH behandeling op de spierontwikkeling en spierkracht weinig bestudeerd bij kinderen. AIM De doelstelling van deze studie was het effect van zowel een fysiologische als een supra -fysiologische GH toediening op de spiermassa en spierkracht bij kinderen met verschillende groeistoornissen te evalueren door een vergelijking met een gepaarde controle groep, getest in dezelfde condities. METHOD 19 (7 jongens en 12 meisjes) met voor minimum 1 jaar GH behandelde patiënten (10 met GH deficiëntie in een fysiologische dosis en 4 SGA geboren kinderen en 5 met Turner syndroom in een suprafysiologische dosis), alsook 19 voor geslacht en leeftijd gepaarde gezonde controles werden bestudeerd. Standaard anthropometrische metingen (o.a. van bovenarm, bovenbeen en kuit), de lichaamssamenstelling met bio-elektrische impedantieanalyse (BIA), de grijpkracht (met een handdynamometer), de sprongkracht (d.m.v. staande wijdbeense sprong) en het aantal zit-staan bewegingen in 30 seconden werden bepaald door dezelfde onderzoeker. RESULTS De leeftijd van de bestudeerde kinderen varieerde tussen 8 en 17 jaar. De controlegroep had wel een significant grotere gestalte (p=0,015) en bovenarmomtrek (p=0,017), doch een vergelijkbare BMI, kuiten bovenbeenomtrek. De totale vetvrije lichaamsmassa bepaald door BIA was niet significant verschillend tussen beide groepen en tussen de kinderen behandeld met supra-fysiologische of fysiologische dosis GH. Bij de GH behandelde kinderen waren zowel de grijpkracht (p<0,09) als het aantal zit-staan bewegingen (p<0,001) en de lengte van de vertesprong (p<0,001) lager dan bij de controle groep. Na correctie voor totale spiermassa verdween het verschil in grijpkracht bij meisjes, maar niet bij de jongens. De andere geobserveerde verschillen in de functionele spiertesten tussen de controle en GH groep bleven bestaan, ook na normering voor de totale spiermassa, bovenbeenomtrek en gestalte. CONCLUSION Met groeihormoon behandelde kinderen vertonen een vergelijkbare totale spiermassa bij BIA bepaling ten opzichte van hun leeftijdsgenoten. Desondanks is hun statische kracht (handgrijpkracht), explosieve kracht (staande vertesprong) en spieruithouding (aantal herhalingen vanuit zit naar stand) lager, ook na normering voor het gestalteverschil. Deze persisterende verschillen kunnen mogelijks inherent aan de onderliggende groeistoornis zijn of aan een verminderde fitheid te wijten zijn en kunnen blijkbaar niet door GH toediening, zelfs in supra-fysiologische dosering, gecorrigeerd worden. Bevestiging van deze resultaten in een grotere groep en in een longitudinale studie zijn evenwel noodzakelijk. Tijdschrift van de Belgische Kinderarts Vol Nr.1 32 E 08 Neonatal hypoglycemia as an early manifestation of X-linked adrenal hypoplasia congenita Willekens Julie, Dept of Pediatrics, University Hospital Brussels, Brussels Gies Inge, Dept of Pediatrics, University Hospital Brussels, Brussels Vanbesien Jesse, Dept of Pediatrics, University Hospital Brussels, Brussels Van Putten Willemijn, Dept of Pediatrics, University Hospital Brussels, Brussels Anckaert Ellen, Dept of Clinical Chemistry, University Hospital Brussels, Br Marjan De Rademaeker, Dept of Medical Genetics, University Hospital Brussel, Bruss Bonduelle Maryse, Dept of Medical Genetics, University Hospital Brussel, Bruss Cools Filip, Dept of Neonatology, University Hospital Brussel, Brussels Moens Els, Dept of Neonatology, University Hospital Brussel, Brussels De Schepper Jean, Dept of Pediatrics, University Hospital Brussels, Brussels INTRODUCTION Introduction and objective X-linked adrenal hypoplasia congenita (AHC) is a rare disease, caused by a mutation or deletion in the NR0B1 gene (Xp21.2), which encodes an orphan nuclear receptor DAX-1. Most of the boys with an early (during infancy) manifestation present with salt wasting, resulting from aldosterone deficiency. We report a neonate with early hypoglycemia related to absent adrenals due to a proven NR0B1 gene mutation to highlight the potential pitfalls and potential fatal outcome of the disease. RESULTS Clinical case description The boy was born after an uneventful pregnancy by caesarean section at 39 3/7 wks. His birth weight was 2950 g, and APGAR score 10/10. He was the third child of unrelated parents. Their first son died at 4 days of life after peripartal asphyxia associated with hyponatremia. Severe hypoglycemia (16 mg/dl) was documented at 12 hours of life. Clinical examination was normal. Serum electrolytes, lactate and ammonia were normal. Urinary organic acids were normal; urinary ketone bodies were absent. Hormonal workup in this boy revealed an undetectable insulin and cortisol level and a high growth hormone level. He was treated with continuous glucose infusion for the first 12 hrs; and oral feeding was introduced the second day with normal capillary glycemia results in the following 24h. He presented with feeding difficulties, hypotonia and hypothermia at the 13th day of life, together with severe hyponatremia (106 meq/l) and hyperkalemia (8.6 meq/l). Serum ACTH was elevated (>2000 ng/l), cortisol was low (10 µg/l) as well as 17-OH progesterone (<0.1 µg/l). Serum renin was elevated (>900 ng/l) and serum aldosterone was undetectable. Adrenal glands were absent on ultrasound and MRI scan. The clinical diagnosis of adrenal hypoplasia congenita was suspected and a genetic workup revealed a missense mutation (c.1260_- 1261ins22, p.arg425aspfsx7) in the NROB1 gene. CONCLUSION Although seldom described in the literature, hypoglycemia might occur in the first days of life in X-linked AHC. Monitoring of glucose and electrolyte levels as well as ACTH determination is mandatory to prevent acute adrenal failure in case of low cortisol levels in neonates with hypoglycemia, even if it occurs transiently. Scrotal hyperpigmentation is not always present in newborns with acute primary adrenal failure. In case of a sibling with neonatal death, the finding of adrenal aplasia or hypoplasia in a boy with adrenal failure and a normal neonatal 17-OH progesterone screening should point to X-linked AHC.

18 Abstracts E 09 Clinical phenotype and hormonal findings in a young child with a monocarboxylate transport Maris Ellen, UZ Gent De Schepper Jean, UZ Gent - UZ Brussel Cools Martine, UZ Gent S Straatmans, UZ Gent Verloo P, UZ Gent Van Coster R, UZ Gent B Delle Chiaie, UZ Gent INTRODUCTION MCT8 has a pivotal role in neuronal triiodothyronine (T3) uptake. Mutations of this transporter determine a distinct X-linked psychomotor retardation syndrome ( Allan-Herndon-Dudley syndrome). We report a male infant with global hypotonia, visual impairment and severe mental retardation and a discordant growth pattern related to an inactivating MCT8 mutation to illustrate the particular clinical phenotype and thyroid function tests. METHOD CASE PRESENTATION The male patient was for the first time evaluated at the age of 16 months at our endocrine clinic for unusual thyroid function tests : repetitive testing from the age of 7 months disclosed consistently elevated ft3, low to normal ft4 and mildly elevated TSH serum levels. The mother had been treated during pregnancy for Graves disease and she delivered after an otherwise uneventful pregnancy at 37 weeks. She and the healthy father are non-consanguineous and both from Belgian origin. Birth parameters were normal. Global hypotonia was observed and visual impairment was already suspected by the mother at the age of 1 month. Persisting global axial hypotonia initiated a neurological work up at the age of 6 months : a metabolic screening, karyotyping and array-cgh analysis were normal, while MRI of the brain showed small frontal subdural collections. His weight gain worsened despite a normal food intake. Physical examination at 16 months showed craniofacial dysmorphism and muscular wasting, but no goiter was palpated and a normal heart rythm was noted. Body weight was at 5 SDS, height at -2.5 SDS and head circumference at -0.2 SDS. No thyroid auto-antibodies were found, but SHBG, a marker for hepatic T3 action, was elevated. Bone age was normal. These additional findings together with the absence of typical signs and symptoms of hypothyroidism, while the laboratory examinations disclosed elevated T3, with changing low to normal T4 and mildly elevated TSH serum levels, was very suspicious for a MCT8 defect. A known hemizygeous c.812g>a missense mutation in exon 3 was documented in the boy and his mother. CONCLUSION Male patients with severe failure to thrive, truncal hypotonia and mental disability should be investigated for a MCT8 gene mutation if they present unusual thyroid function tests. Thyroxine therapy is of no use in these children since it will further increase the high serum T3 concentration and thereby further increase bone age acceleration and muscle hypoplasia and limit further weight gain, while being ineffective in stimulating brain development. E 11 Peripheral artery tonometry in adolescents with type 1 diabetes. A. Pareyn, AZ Sint-Jan Brugge-Oostende AV K. Allegaert, Universitair ziekenhuis Leuven W. Asscherickx, Universitair ziekenhuis Leuven E. Peirsman, Universitair ziekenhuis Antwerpen P. Verhamme, Universitair ziekenhuis Leuven K. Casteels, Universitair ziekenhuis Leuven INTRODUCTION Type 1 diabetes (T1D) is a significant risk factor for atherosclerotic vascular change with clinical evidence for manifest coronary disease before 30 years of age. Therefore, measures to detect pediatric patients at risk for the development of premature cardiovascular disease (CVD) are needed. A well known surrogate marker of CVD is endothelial function. The reactive hyperemia-peripheral artery tonometry (RH-PAT) is a newly developed method for noninvasive endothelial function assessment. AIM The goal of this study is to determine whether a significant difference in RH-PAT score is found in adolescents with T1D as compared to controls. METHOD Thirty-four adolescents with T1D (16 male and 18 female) and 28 healthy age and sex matched control subjects (12 male and 16 female) were recruited from our University Diabetes Centre. Subjects presented in the morning for clinical examination and fasting blood analysis before RH-PAT testing. This technique uses fingertip plethysmography probes to measure the changes in pulse wave amplitude observed before and after a period of reactive hyperemia produced by occlusion of the brachial artery of one arm with a blood pressure cuff. Probes are placed on the index fingers of both hands, the non-occluded arm serving as control. The RH-PAT score is the ratio between the pulse amplitude during a 1 minute period beginning exactly after 90 seconds of reactive hyperemia compared to the pre-occlusion period. RESULTS Adolescents with T1D (n = 34) had significantly lower RH-PAT scores compared to healthy controls (median 1.6 vs 1.8, p < 0.05). As the BMI was significantly higher in children with T1D, we reanalyzed the data after exclusion of overweight and obese children. Five children were excluded in the diabetes group, none in the control group. The data of this analysis shows similar results. There remains a significantly lower RH-PAT score in the diabetic subjects as compared to control patients (median 1.5 vs 1.8, p < 0.01). CONCLUSION There s increasing evidence that vascular change occurs early in children with T1D what implicates a need for early intervention. Some risk factors cannot be changed but attention to identification and treatment of dyslipidemia, hypertension and maintenance of near normal glycemia are mandatory in children with T1D. E 10 Clinical and hormonal characteristics of premature pubarche in a mixed Brussels population Verheyden Stephanie, GIES Inge, VANBESIEN Jesse, ANCKAERT Ellen, De Schepper Jean INTRODUCTION Premature pubarche (PP), defined as the development of pubic hair before the age of 8 years in girls and 9 years in boys, occurs mainly in girls. Axillary hair, increased body odor and acne may also be present, but without other signs of pubertal development. PP is most frequently secondary to a premature or exaggerated adrenarche or it can be idiopathic. Smallness for gestation, prematurity and obesity are known risk factors for PP, whereas PP can be a forerunner of polycystic ovary syndrome in girls. AIM We wanted to analyze 1) whether Magreb girls are at higher risk for exaggerated adrenarche and higher serum AMH concentrations, given their known risk for increased adiposity and insulin resistance, and 2) whether the presence of acne was associated with an exaggerated form of adrenarche. METHOD 17 (4 boys and 13 girls) children evaluated for PP in 2011 at our hospital, in whom a non-classical form of congenital adrenal hyperplasia by ACTH testing and an adrenal tumor by ultrasound were excluded, were studied. Anthropometric data, basal and ACTH stimulated serum androgens, serum SHBG as marker of insulin resistance, serum AMH and bone age readings at the left hand and wrist were collected. RESULTS Chronological age at examination ranged between 5 and 8.8 years, height SDS between -0.8 and 3.2, BMI SDS between -1.0 and 1.9 and bone age advancement between -0.7 and 2.5 year. Seven subjects had mild acne and 9 had axillary hair at the moment of testing. Basal DHEAS was above 0.4 mg/l in all children and correlated with birth weight (r=-0.44, p=0.08), basal androstenedione (r=0.58, p<0.05) and stimulated androstenedione ( r=0.76,p<0.0001), but not with serum SHBG, BMI SDS and bone age advancement. Magreb girls (n=7) had similar BMI SDS and basal androgen, AMH and SHBG concentrations than the allochthonous girls (n=6). Children with associated acne had similar basal serum DHEAS and bone age advancement, but higher stimulated DHEA (7.6(3.3) vs 4.8(2.0)µg/L, p<0.05). CONCLUSION Magreb girls with PP are not at higher risk for associated insulin resistance and potential later risk for polycystic ovary syndrome in our population. Associated acne does not necessarily indicate an exaggerated form of PP. The known gender difference in PP and the association with lower birth weight was confirmed in our mixed population. E 12 Le déficit partiel en 21 hydroxylase Van Lier Zoé, Clinique St Pierre, Ottignies Benoit Brasseur, Clinique St Pierre, Ottignies INTRODUCTION Le déficit partiel en 21 hydroxylase est une cause d hyperplasie congénitale des surrénales de révélation tardive. Il s agit d une pathologie de transmission autosomique récessive dont l expression est variable. Le gène impliqué est le CYP21A2. Les symptômes sont une pubarche prématurée chez la fille ou un hirsutisme associé à d éventuels troubles de fertilité chez la femme adulte, une pilosité pubienne précoce chez le garçon. Une accélération de la vitesse de croissance en période pré-pubertaire est possible dans les 2 sexes. Sa sévérité dépend du type de mutation, un dépistage familial est proposé lorsqu on pose ce diagnostic afin de dépister les personnes porteuses de mutation pouvant entrainer des formes sévères de la pathologie. Cas clinique : Une fillette de 5 ans et 4 mois se présente à la consultation pour une apparition progressive de pilosité pubienne depuis 6 mois. A l examen clinique, on note une taille et un poids supérieurs au P95, un développement pubertaire au stade A1P3M1. Le bilan complémentaire montre un âge osseux de 7 ans et 10 mois soit une avance de 2 ans et 6 mois. La 17OH progestérone et la DHEA sulfate sont augmentées, le taux de cortisol est normal ainsi que celui des autres hormones sexuelles (LH, FSH, 17 ß œstradiol, testostérone). Le taux de 17 OH progestérone augmente lors du test de provocation à l ACTH. Une échographie abdominale ne montre pas de masse surrénalienne, montre un utérus et des ovaires de taille pré-pubère. Le bilan génétique a confirmé la présence d une mutation à l état homozygote du gène CYP21A2. La mère nous signale à l annonce du diagnostic génétique que le père officiel de l enfant n est pas son père biologique, et qu il n est pas au courant de cela. Vu qu il s agit d une mutation homozygote peu sévère un dépistage parental n a pas été effectué. Dans le cas d une mutation sévère un problème éthique aurait été inévitable. Un traitement par hydrocortisone est débuté. Il a permis après quelques mois une régression de la pilosité pubienne (stade P2) et une diminution de la vitesse de croissance. CONCLUSION En cas de pubarche prématurée il est primordial de réaliser un âge osseux, si celui ci est avancé il faut toujours rechercher un bloc enzymatique, celui en 21 hydroxylase est le plus fréquent. Les avancées de la génétique peuvent nous apporter de plus en plus de problèmes éthiques. Dans une situation de mutation peu sévère comme celle ci, le plus important à nos yeux est probablement de privilégier le bien du patient. Journal du Pédiatre Belge Vol Nr.1 33

19 03. Gastro-enterology H 01 Under-nutrition in hospitalized Belgian children. Ph. Alliet, Jessa Hospital, Hasselt M. Uyttendaele, CHU Tivoli, La Louvière Th. Devreker, UZ Brussel L. Muyshondt, CHU, Charleroi P. Bontems, CHU Tivoli, La Louvière Y. Vandenplas, UZ Brussel INTRODUCTION Under-nutrition has a prevalence of % among hospitalised children in western countries and is known to have an adverse effect on clinical outcome. In 2006, a Screening Tool for Risk Of Impaired Nutritional Status and Growth (STRONGkids) was developed (1). This simple assessment is based on four key items presence/absence of an underlying disease with increased risk for under-nutrition, subjective global assessment of nutritional status, decreased intake and weight loss during the last weeks/months. AIM Assessment of under-nutrition in hospitalized Belgian children by anthropometric measurements and by using the STRONGkids assessment (low/medium/high risk), and its effect on duration of hospitalisation. METHOD Prospective, multicenter study with enrolment of all patients aged 1 mth-16 years admitted to the paediatric department in the second trimester 2011 until 100 patients/centre were included after informed consent. Acute under-nutrition was defined as ; -2 SD Wt-for Ht and chronic under-nutrition as ; -2 SD Ht-for age. The STRONGkids assessment divided the children in a low (score 0), moderate (score 1-3) and high risk (score 4-5) group. The study was approved by the ethics committee. RESULTS 416 children (47.8 % F) were included % were admitted for surgery. The mean age was 3.6 years (range 0-16 y) with a mean hospital stay of 5.2 days (range 1-64 days). 37 % of the children stayed > 4 days in the hospital. The prevalence of acute and chronic under-nutrition in the whole group was 9.8 % and 8.1% respectively. In the group staying > 4 days in the hospital, the prevalence of acute and chronic under-nutrition was 10.7% and 11.8 % respectively. The STRONGkids assessment was successfully applied in 96.6 %. Low, moderate and high risk for under-nutrition was observed in 26.1 %, 58 % and 15.9 %. The proportion of children with acute and chronic undernutrition in the high risk group was significantly higher than in the low risk group, 26.8 % vs 5.4 % (p=0.0003) and 17.5 % vs 5.4 % (p=0.0074). The mean hospital stay in the high vs low risk group was 7.1 vs 4.3 days with a higher percentage of children staying more than 4 days (60.7% vs 25.5 %) in the former group. CONCLUSION Acute and chronic under-nutrition has a prevalence of respectively 10.7% and 11.8 % (in the group with a hospital stay > 4 days) in hospitalized Belgian children. The STRONG-kids assessment is an easy screening tool. A high score correlated well with an increased risk of acute or chronic under-nutrition and with a longer hospital stay. (1) Joosten KF, Zwart H, Hop WC, Hulst JM. Three national screening days on malnutrition in children admitted to the hospital in the Netherlands. Arch Dis Child 2010; 95: H 03 Celiac disease in childhood: a 20-year single-centre experience Coolen Claudia, Jessa Hospital Hasselt Gillis Philippe, Jessa Hospital Hasselt Janssens Elke, Jessa Hospital Hasselt Alliet Philippe, Jessa Hospital Hasselt INTRODUCTION Celiac disease (CD) is a genetic, immune-mediated, chronic enteropathy, characterized by a permanent sensitivity to gluten, causing atrophy and inflammation of the small bowel mucosa. The serologic prevalence differs between 0.5 to 1.26% in the general population. CD can present as a wide variety of gastro-intestinal and extra-intestinal symptoms. A lifelong gluten-free diet is the only safe and efficient treatment. AIM Retrospective analysis of patient characteristics, presenting symptoms and follow-up of all children diagnosed with CD in our centre during the period METHOD The diagnosis of CD was made by the combination of positive antibody tests (endomysium antibodies (EmA IgA), tissue-transglutaminase IgA (t-tg IgA) and/or deaminated gliadine IgG in case of IgA deficiency) and a positive biopsy according to the Marsh-Oberhuber classification. After professional dietary counseling and introduction of a gluten-free diet, patients came for follow-up every 2 months until normalisation of the CD-specific antibodies (EmA IgA negative or t-tg IgA < 7 U/L). Afterwards a yearly follow-up was proposed. RESULTS 71 children were included in the analysis. 40 of them were diagnosed during the last 5 years. The median age of diagnosis was 5.5 years (range 11 months - 16 years). Most common presenting symptoms were abdominal pain (30/71 (42.25%)), diarrhea (31/71 (43.66%)), weight loss/failure to thrive (14/71 (19.72%)) and behavior alterations (12/71 (16.9%)). Constipation was a presenting symptom in 11 children (15.49%). 21% of our patient group had an associated autoimmune disorder (type-1 diabetes mellitus (8/15), thyroid disorders (3/15) and alopecia areata (3/15)). In those patients, the diagnosis was made by systematic serological screening. 7% (5/71) had an IgA-deficiency. Histology showed partial or total villus atrophy (Marsh 3a/3b) in 60,42% (50/71). After starting a strict gluten-free diet, the median time until negativation of the celiac antibody titers was 6.5 months (range 2 23 months). Patients stayed in follow up for a median duration of 2 years (range 0 14 years). 8.5% of the population was lost for follow-up. In the patients following a gluten-free diet for more than 6 months, 84% had a strict negative celiac serology at the last evaluation. CONCLUSION Celiac disease is a multisystemic disorder with an apparent rising prevalence during the last years. This was confirmed by our findings (40/71 diagnoses in the last 5 years). A high degree of diagnostic suspicion is needed since less than half presented with the classical symptomatic triad (diarrhea, abdominal pain, weight loss). More than 20% of the children were asymptomatic and had positive CD-specific antibodies at the diagnosis of another auto-immune disorder. The median time for negativation of the serology after starting a gluten-free diet was 6.5 months. In our population, 84% adhered well to the strict gluten-free diet. H 02 Safety and cost of infliximab for the treatment of belgian pediatric patients with crohn s de Greef E, UZ Brussel John J Mahachie, ULG, Montefiore Institute Van Steen Kristel, ULG, Montefiore Institute Veereman G, UZ Brussel IBD Working Group, Bespghan IBD Working Group, Bird INTRODUCTION Biologicals have become an important component in the treatment of Crohn s disease in children. Their increased and long term use raises safety concerns. AIM To describe safety and cost of infliximab in a Belgian cohort of pediatric Crohn s disease patients. METHOD All patients on infliximab as part of the present or past treatment for Crohn s Disease until January 1st 2011 were selected from an existing database. Information on disease phenotype, medication and adverse events were extracted. RESULTS Adverse events occurred in 25.9% of patients exposed to infliximab of which 29.6% were severe. In total 31.7 % of patients stopped infliximab therapy. The main reasons for discontinuation were adverse events in 45.4% and loss of response in 30.3%. No malignancies or lethal complications occurred over this 241 patient year observation period. Immunomodulators were concomitant medication in 75.9% of patients and were discontinued subsequently in 35.4% of them. The cost of infliximab infusions per treated patient per year in the Belgian health care setting is approximately euro, including only medication and hospital related costs. CONCLUSION Even though infliximab is relatively safe in pediatric CD on the short term, close follow-up and an increased awareness of the possible adverse reactions is highly recommended. Adverse reactions appeared in 25.9% of all patients and were the main reason for discontinuation. Treatment cost has to be balanced against efficacy and modifications in disease course. In the Belgian health care system, the medication is available to all patients with moderate to severe CD. H 04 A rare case of protein-loosing enteropathy and congenital lymphedema? Saskia Vande Velde, UZ Gent, dienst kindergastro-enterologie Bert Calluwaert, UZ Gent, dienst medische genetica Jelle Degraeuwe, Maria Middelares Gent Olivier Van Akker, UZ Gent, dienst medische genetica Ruth De Bruyne, UZ Gent, dienst kindergastro-enterologie Stephanie Van Biervliet, UZ Gent, dienst kindergastro-enterologie Myriam Van Winckel, UZ Gent, dienst kindergastro-enterologie INTRODUCTION Protein-loosing enteropathy (PLE), is a rare condition in infants and mostly due to primary intestinal lymphangiectasia (PIL). This case report illustrates PLE as a complication of a more generalized congenital anomaly of the lymphatic system. METHOD Case report: A 3 week old male baby, the first child of healthy unrelated parents, is referred with lymphedema of the lower limbs. At birth, edema of the feet was noted and became more generalized. Laboratory findings include hypoalbuminemia, hypogammaglobulinemia, and normal liver function. Albuminuria is absent. α;1-antitrypsin in faeces is elevated. Abdominal ultrasound shows limited ascites with normal duplex sonography. Gastroduodenoscopy indicates white spots. Although these findings suggest intestinal lymphangiectasia, a biopsy of duodenal mucosa did not visualize lymphatic vessels and a transmural duodenal biopsy after 5 weeks of treatment showed a slightly dilated lymphatic system. The protein loss was resistant to a median chain triglyceride (MCT) based diet and to basic -F, an almost completely fat free milk, necessitating total parenteral nutrition (TPN), which stopped intestinal protein loss. Reintroduction of a MCT diet resulted in relapse. Fat-free solid foods were introduced at 4 months. Hypoproteinemia was compensated by regular infusions of albumin (initially twice weekly, now at the age of 9 months, every two weeks) and immunoglobulins (every 4 weeks). Growth and psychomotor development is normal, but lower limb lymphedema persists. RESULTS Discussion: The early presentation of PLE and the presence of congenital lower limb lymphedema, suggests a primary congenital lymphedema with intestinal manifestations rather than PIL. PLE is present in only a minority of these patients. Both Milroy disease and Emberger syndrome should be considered. These autosomal dominant disorders display reduced penetrance and are characterized by edema of the lower limbs, which can be asymmetric. In Milroy disease, lymphedema is congenital and affects primarily the feet in neonates. Mutations have been found in FOXC2 and FLT4 (VEGFR3). Emberger syndrome associates lymfedema, sometimes congenital with increased risk for acute myeloid leukemia, immunological impairment and sensorineural deafness. The syndrome is caused by mutations in GATA2. Mutation analysis is ongoing in our patient. CONCLUSION We present a case of primary congenital lymphedema complicated with severe PLE that only partially responded to LCT free nutrition. Related cases are sparse and the prognosis remains unclear. Tijdschrift van de Belgische Kinderarts Vol Nr.1 34

20 Abstracts H 06 Rectal prolaps in a 12-year old girl N. Segers, Prinses Elisabeth Kinderziekenhuis Gent S. Vande Velde, Prinses Elisabeth Kinderziekenhuis Gent L. Delrue, UZ Gent, radiologie M. Van Winckel, Prinses Elisabeth Kinderziekenhuis Gent D. Vande Putte, UZ Gent, kinderheelkunde K. Van Renterghem, UZ Gent, kinderheelkunde S. Van Biervliet, Prinses Elisabeth Kinderziekenhuis Gent INTRODUCTION Rectal prolaps in children is most often caused by either cystic fibrosis, by chronic constipation and straining, or induced by a rectal mass (eg juvenile polyp). We present a patient with rectal prolaps and longstanding defaecation problems as the consequence of an anatomic variant. RESULTS This report describes a 12-year old girl who presented with chronic lower abdominal pain improving after defaecation, sometimes associated with diarrhea and occasionally vomiting since the age of 7. There is also daily involuntary stool loss. There is no history of weight loss. She grows on the 17th centile and has a BMI on the 50th centile. She has a normal development and no gait abnormalities. Since six months, there is a rectal prolaps after every evacuation, which reduces spontaneously. She had several examinations (abdominal ultrasound, sweat test, hydrogen breath test, anorectal manometry and colonoscopy) in another centre demonstrating only faecal contamination in the colon. She was treated with laxatives and toilet training without effect on the prolaps frequency or abdominal pain. When consulting at our centre, she also complaints of false defaecation urge and looses small amounts of blood after defaecation. As a photography revealed a prolaps without leading point, a defaecography was planned. This revealed laxity of the pelvic floor muscles with a descending proximal rectosigmoid during defaecation, leading to pressure on the distal part of the rectosigmoid. This caused an incomplete emptying of the rectum with persisting defaecation need and rectal prolaps as result. A barium enema study showed also a dolichocolon. It was decided to perform a rectopexy with resection of the abundant sigmoid. There were no post-operative complications and she was discharged after 7 days with normal stools without prolaps. Six months after surgery she remains without complaints. CONCLUSION This case report illustrates the value of defecography in patients with unexplained defaecation disorders. H 08 Is it worthwile looking for Hirschprungs disease in children ; 1 year with constipation? Hauser Bruno, Universitair Kinderziekenhuis Brussel Devreker Thierry, Universitair Kinderziekenhuis Brussel Veereman Gigi, Universitair Kinderziekenhuis Brussel De Greef Elisabeth, Universitair Kinderziekenhuis Brussel Vandenplas Yvan, Universitair Kinderziekenhuis Brussel AIM Constipation is frequent in children. Hirschprungs disease is mainly diagnosed in infants. We performed a retrospective study to look for Hirschprungs disease in children older than 1 year of age with constipation. METHOD One hundred and one children (49 boys) with an age of 4.3 ± 2.96 years ( years) presenting with constipation for 1.86 ± 2.32 years ( years) underwent a rectoscopy with forceps biopsies taken at 3 cm of the anal margin under intrarectal Midazolam between January and December The biopsies were examined for the presence of muscularis mucosa, submucosa, ganglion cells and hypertrophic fibers. RESULTS Muscularis mucosa was present in 58/101 (57.4 %), absent in 4/101 (4.0 %) and presence unknown in 39/101 (38.6 %) of the biopsies. Submucosa was present in 17/101 (16.8 %), absent in 74/101 (73.3 %) and presence unknown in 10 (9.9 %) of the biopsies. Ganglion cells were present in 30/101 (30.3 %) and absent in 71/101 (69.7 %) of the biopsies. Ganglion cells were present in the muscularis mucosa and submucosa in 13/30 (43.3 %), only in the muscularis mucosa because there was no submucosa in 16/30 (53.3 %) and it was unknown in 1/30 (3.4 %) of the biopsies. Few hypertrophic fibers were present in 8/101 (7.9 %), absent in 86/101 (85.2 %) and presence unknown in 7/101 (6.9 %) of the biopsies. In the group of 71 patients without ganglion cells, there were no hypertrophic fibers in 64/71 (90.1 %) and only a few hypertrophic fibers but not enough for a diagnosis of Hirschprungs disease in 7/101 (9.9 %) of the biopsies. CONCLUSION In a group of children older than 1 year of age with constipation, Hirschprungs disease was excluded with certainty in 30 % of the patients and was very unlikely in the other 70 % of patients using rectal biopsies obtained by rectoscopy. H 07 Thrombosis as extra-intestinal manifestation of inflammatory bowel disease in children Gysemans Willem, Jessa Ziekenhuis, Hasselt Van Geet Christel, UZ Gasthuisberg, Leuven Alliet Philippe, Jessa Ziekenhuis, Hasselt INTRODUCTION Population-based studies show an incidence rate of venous thromboembolism of 20-30/ person-years in IBD. Compared to control groups, the incidence is twice that of the general population. RESULTS A thirteen year old boy with ulcerative colitis (UC) in clinical and biochemical remission since 2.5 years, presented with of a ten day course of right-sided neck pain. Complaints had started two days after patient had fallen on his back. Active movement of the neck was especially painful. Algipan, a local NSAID, was being administered without bringing any relief. Patient had no fever, no abdominal pain, diarrhoea or bloody stools. He was on therapy with Colitofalk (mesalazine) 500 mg three times daily. Further personal history was negative apart from relapsing urticaria/itching for which in the past cetrizine was used. Family history was negative for bleeding disorders and thrombotic disease. There was no history of IBD or other auto-immune diseases. Clinical examination was unremarkable. Weight and height were situated at the tenth percentile. There were no bruises, petechiae or ecchymoses. Mobilisation of the neck was painful. An ultrasound of the neck showed a partial thrombosis of the right jugular vein over a distance of approximately 5 cm. CT angiography confirmed the diagnosis. Laboratory investigation showed normal hematocrit and haemoglobin. White blood cell count was /mm³ and showed 64 % eosinophilic granulocytes. Inflammatory markers were elevated with CRP 1.52 mg/dl and sedimentation rate 33 mm/h. Prothrombotic screening revealed heterozygousity for Factor V Leiden mutation. Factor II G20210A mutation was negative. apc resistance was withheld as diagnosis. Treatment was promptly started with low molecular weight heparins (LMWH) 1 mg/kg twice daily for two weeks. Therapy with LMWH once daily was administered for a total duration of four months, until no more thrombus was visualised on ultrasound and fibrinogen degradation products in blood had disappeared. CONCLUSION IBD is a thrombotic risk factor. Together with other risk factors as in our case apc resistance and trauma, even young patients can present with venous thromboembolism. When thrombosis occurs, the treatment is equal to that of thromboembolic events in the general population. H 09 Présence inattendue d histiocytes bleu-mer dans une ponction de moëlle... Bénédicte Van Grambezen, UCL Laurence Pain, UCL Xavier Stéphenne, UCL Françoise Smets, UCL Etienne Sokal, UCL AIM Un garçon de 14 ans nous est référé pour transplantation hépatique dans le cadre d une cirrhose avec syndrome hépato-pulmonaire. Ses antécédents néonataux et de la petite enfance sont sans particularité. A l âge de 4 ans, il présente un épisode fébrile associé à des adénopathies et à une splénomégalie. Les enzymes hépatiques sont légèrement perturbées et sa bilirubine est légèrement majorée. Les recherches infectieuses, métaboliques et hématologiques sont négatives. La splénomégalie persiste, et à l âge de 11 ans, il développe une importante cyanose et un syndrome hépato-pulmonaire est diagnostiqué. Vu le contexte initial et la splénomégalie persistante, une ponction de moëlle est effectuée et met en évidence des histiocytes bleu-mer. RESULTS Les histiocytes bleu-mer sont des histiocytes surchargés en lipide dont le cytoplasme comprend des granules qui se colorent en bleu en coloration de May Giemsa. La présence des histiocytes bleu-mer oriente le diagnostic vers une maladie de Niemann Pick de type B ou un syndrome primaire des histiocytes bleu-mer. Le syndrome des histiocytes bleu-mer a été décrit pour la première fois dans les années 70. Cette pathologie débute habituellement de manière brutale : le patient est fébrile et présente de nombreuses adénopathies ainsi qu une splénomégalie. L atteinte hépatique est souvent peu importante. La thrombopénie est quasi constante. Le diagnostic repose sur la présence d histiocytes bleu-mer dans la moëlle, le foie, la rate ou les ganglions. L évolution de cette pathologie est souvent bénigne. Il arrive dans de rares cas que le patient évolue vers une insuffisance hépatique nécessitant une greffe. Un syndrome hépato-pulmonaire n a pas été rapporté chez ces patients. Ces histiocytes peuvent également être vus dans la maladie de Niemann Pick B qui se caractérise par une déficience en sphingomyélinase. Cette entité associe une thrombopénie et une splénomégalie; une atteinte hépatique ou pulmonaire de type pneumopathie interstitielle est également possible. Il n y a pas d atteinte neurologique. CONCLUSION Le dosage de la sphingomyélinase est actuellement en cours chez notre patient. Ce dosage permettra de faire le diagnostic différentiel entre une maladie de Niemann Pick B ou un syndrome primaire des histiocytes bleu-mer. Journal du Pédiatre Belge Vol Nr.1 35

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