[Congenital hepatic fibrosis in children: about 9 cases and review of the literature]

Congenital hepatic fibrosis is a recessive autosomic disease with two major risks: gastrointestinal haemorrhage caused by portal hypertension and cholangitis related to bacterial infection of dilated intrahepatic bile ducts. The aim of our study is to define epidemiological features, the presenting symptoms, the diagnosis, the evolution and the management of this disease. Between January 1990 and December 2000, we reported the cases of nine children with this disease at children hospital of Tunis.Three were male and six female. The mild age was three years and six months. Consanguinity was present in five cases and similar cases were found in six cases. The FHC was revealed by portal hypertension in five cases, angiocholitis in one case and by portal hypertension and angiocholitis in three cases. Liver biopsy was done in seven children. Ultrasound examination of the liver and kidney revealed caroli syndrome in five cases and polykystose renal in two cases The intravenous pyelography was performed in four cases showing precalicial canalicular ectasia in four cases. Eosophageal endoscopy had shown oesophageal varices in six patients. The follow up had shown that three patients had gastrointestinal bleeding, three had angiocholitis. One patient died with multivisceral failure. The treatment of acute bleeding has needed blood transfusion in four cases. Primary prevention of bleeding was done by endoscopic sclerosis alone in one case and associated to betablokers in two cases. Secondary prevention of varices bleeding was done by sclerotherapic in two cases, by beta blokers alone in one case and by betablokers associated to elastic ligation of oesophageal varices in one case

[Upper gastrointestinal endoscopy in Neonates -experience of pediatric gastroenterology unit]

The aim of our study was to assess the frequency of the dif-ferent lesions occurring as well as to precise indications of upper gastrointestinal endoscopy in neonates

Method: We have achieved a retrospective study about 128 neonatal gastrointestinal endoscopies. Three groups were constituted according to macroscopic findings:- Group I: normal aspect [n=1]- Group II: isolated esophagitis [n=19]- Group III: esogastritis or gastroduodenitis or esogastroduo-denitis [n=92

Results: The neonates undergoing endoscopy for malaise were more frequent in group I than in group II and III, respectively 36.5% versus 15.8% and 9.8% [P = 0.04]. Digestive hemorrhage [hematemesis and/or melena] was more frequent in group III than in group II and I, respectively 90.2% versus 78.9% and 63.6% [P = 0.03], Digestive hemorrhage was in our study the main indication of upper gastrointestinal endoscopy in neonates [85.9%] which showed a macroscopic lesion in 93.5% of cases

Conclusion: Hematemesis and suspicion of esophagitis are good indications for upper gastrointestinal endoscopy in neonates

[Hyperlipidaemia associated to inaugurated diabetic ketoacidosis]

Severe hyperlipidaemia in presenting diabetic ketoacidosis was rarely reported in child.We report the case of a three-year-old girl with presenting diabetic ketoacidosis. Family history was negative for hyperlipidaemia. Serum was creamy pink in gross appearance. The child has a hyperlipidaemia with serum triglycerides 18.5 mmo1/1 and serum cholesterol 13.8 mmol/l. The hyperlipidaemia disappeared with insulin therapy within seven days. We study the clinical, laboratory, pathogenic and evolution features of this uncommon trouble in diabetic children