[Duodenal duplication revealed by acute pancreatitis]. / Duplication duodénale révélée par une pancréatite aiguë.

INTRODUCTION: Duodenal duplications are rare congenital malformations whose revealing signs are highly variable and nonspecific. OBSERVATION: We report the case of a female infant who presented with neonatal acute pancreatitis complicated by recurrent ascites, profound hypoalbuminemia responsible for pleural and pericardial effusions, revealing a duodenal duplication cyst. The unusual and original clinical presentation as well as the difficulty detecting the duplication radiologically delayed the diagnosis. A prolonged medical treatment with octreotide, albumin infusions, and exclusive parenteral nutrition led to an almost total disappearance of the ascites before surgery. The outcome was favorable after surgical removal of the duplication with 1 year of follow-up. CONCLUSION: The diagnosis of duodenal duplication can be difficult and it may be necessary to repeat the ultrasound examinations. Surgical resection is delicate, especially when there is an abundant pancreatic ascites. Therefore, an adequate prolonged medical treatment to reduce this ascites is recommended before the surgery.

[Tocilizumab: experience in a French rheumatological pediatric center]. / Traitement par tocilizumab : expérience d'un centre de rhumatologie pédiatrique.

Tocilizumab (TCZ) is an anti-interleukin-6-receptor antibody. The blockade of IL-6 is used as a strategy for the treatment of systemic juvenile idiopathic arthritis (S-JIA) and multicentric Castleman disease (MCD). In this study, we describe the tolerability profile of tocilizumab in eight children followed in a pediatric rheumatology department. Six patients were treated for S-JIA and two for a MCD. They received doses of TCZ between 8 and 12mg/kg of body weight depending on their disease. Infusions were received every 2-4 weeks. The mean duration of treatment was 32.9 months (14 months to 4.5 years). Clinical adverse events were all mild or moderate. No cases of macrophage activation syndrome and no anaphylactic reactions were reported. TCZ was never stopped for a clinical adverse event. Neutropenia was the most common biological adverse event, sometimes requiring dose adjustments. Thrombopenia, lymphopenia, and increased liver enzymes were reported as well, but treatment was not modified. All these biological adverse events were not complicated by any clinical manifestation. In conclusion, TCZ had a good tolerability profile in these eight patients with partial or total efficacy. Despite this advantageous profile, TCZ should be closely monitored because of the potential severity of adverse events. Moreover, long-term safety has still not been assessed.

[Wilson's disease with liver copper in normal concentration]. / Maladie de Wilson avec concentration hépatique en cuivre normale.

The cases of 2 children with Wilson's disease revealed by persistent hypertransaminasemia are reported. Blood ceruloplasmin concentration was low but the liver content of copper was lower than usually seen in presymptomatic forms of the disease. The apparently low liver copper concentration could be explained by an unusually important steatosis.