Thierry Lamireau

Liver Stiffness Measurement in Children Using FibroScan: Feasibility Study and Comparison With Fibrotest, Aspartate Transaminase to Platelets Ratio Index, and Liver Biopsy

Objective: Transient elastography (FibroScan) is a novel, noninvasive, rapid bedside method to assess liver fibrosis by measuring liver stiffness in adult patients. The usefulness of FibroScan in children with chronic liver diseases is unknown. The aim of this prospective study was to evaluate the feasibility of liver stiffness measurement and to compare FibroScan, Fibrotest, and aspartate transaminase to platelets ratio index (APRI) with liver biopsy for the diagnosis of cirrhosis in children with chronic liver diseases. Patients and Methods: Between February 2004 and October 2005, 116 consecutive children with chronic liver diseases were prospectively included. All except 1 child (58 boys, mean age 10.7 years) could have noninvasive tests for fibrosis: FibroScan, Fibrotest, and APRI, and, when necessary, a liver biopsy (n = 33). Results: FibroScan, Fibrotest, and APRI were correlated with clinical or biological parameters of chronic liver diseases, but the FibroScan marker correlated most with all parameters. By histology, the METAVIR fibrosis category score was F1 in 7 cases, F2 in 8 cases, F3 in 6 cases, and F4 in 12 cases. FibroScan, Fibrotest, and APRI were significantly correlated with the METAVIR fibrosis score. For the diagnosis of cirrhosis, the area under the receiver operating characteristic curve was 0.88, 0.73, and 0.73 for FibroScan, Fibrotest, and APRI, respectively. Conclusions: These results indicate that liver stiffness measurement is feasible in children and is related to liver fibrosis. A specific probe dedicated to children and slender patients has thus been developed and is currently under evaluation. The FibroScan equipped with this specific probe could become a useful tool for the management of chronic liver diseases in children.

Oxygen saturation during esophagogastroduodenoscopy in children : General anesthesia versus intravenous sedation

BACKGROUNDnHypoxia may occur in children undergoing upper digestive endoscopy under sedation. The purpose of this study was to compare the occurrence of desaturation during intravenous sedation with that which occurs during general anesthesia.nnnMETHODSnThirty-six patients between 3 months and 6 years old underwent a diagnostic esophagogastroduodenoscopy under sedation (n = 18) or general anesthesia (n = 18). Oxygen pulse oximetry, heart rate, and mean arterial pressure were monitored throughout the procedure. At the end of the procedure, the operator gave the value of the endoscopy satisfaction score on a scale of I (very good conditions) to IV (impossible procedure).nnnRESULTSnThe minimum oxygen pulse oximetry value was significantly lower in the sedation group compared with that in the general anesthesia group (89 +/- 5 vs. 97 +/- 1; p < 0.001). In the general anesthesia group, the oxygen pulse oximetry level declined to less than 95% in only one child; but in the sedation group, it declined to less than 95% in 16 patients (5.5% vs. 89%). Nine patients had a profound desaturation in sedation group (oxygen pulse oximetry < 90%); no patients in the general anesthesia group had desaturation (50% vs. 0%). In the general anesthesia group, heart rate and mean arterial pressure remained stable during the whole procedure, whereas in the sedation group, heart rate and mean arterial pressure increased significantly during the procedure. The endoscopy satisfaction score was I in all 18 patients in the general anesthesia group, whereas in the sedation group, it was I in only 2 patients, II in 8 patients, and III in 10 patients.nnnCONCLUSIONSnThese results confirm that hypoxia during upper digestive endoscopy in patients under sedation is a frequent occurrence in children. When compared with sedation, general anesthesia is a safer technique that prevents hypoxia and allows the gastroenterologist to perform the endoscopy under better conditions.

Indications and Limitations of Bariatric Intervention in Severely Obese Children and Adolescents With and Without Nonalcoholic Steatohepatitis: ESPGHAN Hepatology Committee Position Statement

ABSTRACT Morbid obesity is strongly associated with nonalcoholic fatty liver disease (NAFLD), which is one of the most common causes of chronic liver disease worldwide. The present best treatment for NAFLD and nonalcoholic steatohepatitis (NASH) is weight reduction through lifestyle modification. Because of frustrating inefficiency of such a therapeutic approach, bariatric surgery is increasingly performed in adolescents as an alternative option for weight reduction. Standards of care and consensus for indications are, however, scarce. We explore the indications and limitations of bariatric surgery in children with severe obesity with and without NASH and aim to provide guidance for the exceptional indications for adolescents with extreme obesity with major comorbidity that may benefit from these controversial interventions. Present evidence suggests that bariatric surgery can decrease the grade of steatosis, hepatic inflammation, and fibrosis in NASH. Uncomplicated NAFLD is not an indication for bariatric surgery. Roux-en-Y gastric bypass is considered a safe and effective option for adolescents with extreme obesity, as long as an appropriate long-term follow-up is provided. Laparoscopic adjustable gastric banding has not been approved by the Food and Drug Administration for use in adolescents and therefore should be considered investigational. Finally, sleeve gastrectomy and other types of weight loss surgery that have grown increasingly common in adults, still need to be considered investigational. Temporary devices may be increasingly being used in pediatrics; however, future studies, including a long-term risk analysis of patients who undergo surgery, are much needed to clarify the exact indications for bariatric surgery in adolescents.

Cholestasis in the newborn and infant

Neonatal cholestasis occurs in approximately 1 in 2500 term infants, the most common underlying disease being biliary atresia, viral infections and α1-antitrypsin deficiency. The incidence of cholestasis is much higher in extremely premature newborns. The etiology of biliary atresia remains unclear, which in turn makes the search for additional treatments to surgery challenging. Reliable non-invasive tools to differentiate biliary atresia from other forms of neonatal cholestasis need to be further investigated. Despite important findings in the last decades, the pathogenesis of cholestatic liver disease in α1-antitrypsin deficiency remains to be clarified. Any such explanation would also need to explain why only a minority of individuals with PiZZ phenotype develop liver disease. For other genetic diseases causing neonatal cholestasis, such as Alagilles syndrome and progressive familial intrahepatic cholestasis the breakthrough within the field of molecular biology has definitely deepened our understanding of both etiology and pathogenesis. However, the correlation between genotype and phenotype is rarely obvious and for several patients with the seemingly correct phenotype no known genetic mutation is detected. A stepwise approach to the management of cholestasis in the newborn and infant is suggested, where percutaneous liver biopsy is of value to select patients with suspected biliary atresia for laparotomy.

Pancréatite récurrente révélant une maladie de Crohn

Resume Une atteinte pancreatique asymptomatique peut sobserver au cours de la maladie de Crohn. Exceptionnellement elle peut inaugurer les manifestations et reveler la maladie inflammatoire digestive. Observation.xa0– Un garcon de 12 ans a presente des poussees de pancreatite aigue recidivante dont le bilan etiologique etait negatif. Lors de la quatrieme poussee, des douleurs abdominales diffuses, un abces anal, dans un contexte d’alteration de l’etat general, ont conduit au diagnostic de maladie de Crohn. La corticotherapie a permis la disparition des manifestations pancreatiques et de la poussee intestinale. Conclusion. – Une pancreatite recidivante peut inaugurer une maladie de Crohn, et faire errer le diagnostic. Un examen endoscopique, œsogastroduodenal et colique, doit etre envisage en cas de pancreatite recidivante dont le bilan etiologique reste negatif.

Attempt to Determine the Prevalence of Two Inborn Errors of Primary Bile Acid Synthesis: Results of a European Survey

Objective: Inborn errors of primary bile acid (BA) synthesis are genetic cholestatic disorders leading to accumulation of atypical BA with deficiency of normal BA. Unless treated with primary BA, chronic liver disease usually progresses to cirrhosis and liver failure before adulthood. We sought to determine the prevalence of 2 common disorders, 3&bgr;-hydroxy-&Dgr;5-C27-steroid dehydrogenase (3&bgr;-HSD) and &Dgr;4–3-oxosteroid-5&bgr;-reductase (&Dgr;4–3-oxoR) deficiencies and to describe current diagnostic and treatment strategies among different European paediatric hepatology centres. Methods: A total of 52 clinical paediatric centres were approached and 39 centres in 21 countries agreed to participate in the Web-based survey. The survey comprised questions regarding general information, number of cases, diagnostic, and therapeutic management. Results: Seventeen centres located in 11 countries reported patients with inborn errors in primary BA synthesis, 22 centres never had cases diagnosed. In total, we could identify 63 patients; 55 with 3&bgr;-HSD and 8 with &Dgr;4–3-oxoR deficiency in 21 countries. The minimum estimated combined prevalence of these diseases was 1.13 cases per 10 million (0.99 and 0.14 for 3&bgr;-HSD and &Dgr;4–3-oxoR deficiencies, respectively). The surveyed colleagues indicated their main challenges to be the rarity of diseases and the lack of convenient laboratory facilities nearby. Conclusion: We have identified the largest cohort of patients with 3&bgr;-HSD or &Dgr;4–3-oxoR deficiency described so far. These diseases are likely underdiagnosed mainly due to unawareness of their existence and the lack of laboratory facilities.

Non-surgical management of idiopathic fibrosing pancreatitis.

Introduction: Surgical exploration, including pancreatic biopsy and bilioenteric bypass, is still considered to be the gold standard treatment for children presenting with idiopathic fi brosing pancreatitis. Patients and method: We report two cases of IFP in a 4-year-old and an 11-year-old girl, respectively. Both patients underwent a CT- guided biopsy, which showed severe lesions of active fi brosing pancreatitis. All symptoms of obstructive jaundice resolved spontaneously within two months without the need for any invasive surgery. Discussion: We believe surgery should no longer be considered the gold standard treat- ment for children with obstructive jaundice and a pancreatic mass and that these children should be investigated by percutaneous radiologically guided biopsy and initially treated conserva- tively.

Differential Diagnosis of Inflammatory Bowel Disease

Numerous diseases can present with symptoms similar to IBD. In case of acute-onset diarrhea, infectious or allergic diseases and acute appendicitis should be ruled out before considering the diagnosis of IBD. Chronic or recurrent intestinal symptoms are more suggestive of IBD. Infection, celiac disease, or food allergy should all be entertained in the differential diagnosis in this situation. Neoplasm and vasculitic disorders are more rarely involved. Disorders of the immune system should be sought after, especially in cases of early-onset (<6 years) symptoms, as numerous monogenic disorders are currently being discovered with specific therapeutic options available. Less classic presentations of IBD can be encountered, leading to consideration of other diagnoses such as tumors or tuberculosis in cases of an abdominal mass, peptic disease in cases of isolated esophagogastroduodenal involvement, immunodeficiency syndromes, or trauma in cases of isolated perianal/perineal disease.

A practical approach to the child with abnormal liver tests.

The presence of elevated aminotransferases on routine blood tests can reveal liver diseases of various severities. In children, etiologies are more numerous and complex than those usually considered in adults. Information derived from family and personal history, physical examination and basic laboratory data are necessary to reach a timely and correct diagnosis. A stepwise approach is proposed to guide the timing of more specific investigations that are often required.

SFP-P159 – Hépatologie, gastro-entérologie et nutrition – Résultats de la chirurgie transanale dans la maladie de Hirschsprung

Objectifs Durant la derniere decennie, une technique par voie transanale s’est developpee pour le traitement des formes rectales et sigmoidiennes distales qui representent 75 % des maladies de Hirschsprung. Le but de ce travail est d’evaluer les resultats immediats et a moyen terme. Materiel et Methodes Les dossiers des enfants presentant une maladie de Hirschsprung, confirmee par l’absence de cellules ganglionnaires sur les biopsies rectales profondes, et operes par voie transanale entre 2000 et 2006 ont ete analyses. La technique operatoire consiste a dilater le canal anal puis everser et exterioriser le colon a travers l’anus, resequer la zone aganglionnaire, la zone de transition et la zone dilatee immediatement sus-jacente, et anastomoser le colon sain et la ligne pectinee. Les caracteristiques des enfants, le devenir a court et moyen terme ont ete analyses. Resultats Quinze enfants, 13 G et 2 F, ont ete operes a un âge median de 4 mois (2 a 84 mois). La longueur mediane du segment reseque etait de 12 cm (3 a 22 cm). Il n’y a pas eu de complications post-operatoires immediates et la duree mediane d’hospitalisation a ete de 7 jours (5 a 30 j). Une enterocolite est survenue chez 2 enfants a J7 et J21 post-operatoires. Un enfant a presente a J30 post-operatoire une peritonite appendiculaire, puis 2 mois plus tard un abces de la marge anale. Le pronostic fonctionnel a ete evalue chez 14 enfants avec un recul median de 18 mois (4 a 60 mois). Une constipation etait presente chez 6 enfants, necessitant des laxatifs au long cours chez 4 d’entre-eux. Parmi eux, un enfant a du etre reopere en raison d’une resection insuffisante. Le transit est normal chez les 8 autres patients. L’etat du siege est normal chez 9 enfants, tandis que les 5 autres ont presente un abces de la marge anale, une dermite erosive du siege, un aspect fibreux de la cicatrice sans stenose veritable, une hypertonie du sphincter anal, un aspect devie du canal anal. Conclusion L’intervention par voie anale est une alternative interessante a la voie classique dans les formes courtes de la maladie de Hirschsprung ne depassant pas la boucle sigmoidienne. Elle est particulierement adaptee a la periode neonatale, les suites sont le plus souvent simples et le pronostic fonctionnel est satisfaisant.