[Pathology of the bile ducts in children with homozygous sickle-cell anemia. Apropos of 5 recent cases].
Five cases of biliary complications in childhood sickle cell disease are reported. In four cases, the pathology was gall stones causing recurrent abdominal pain in a 10 year old boy, a 13 year old girl and a 2 year old infant, and responsible for a "Salmonella septicaemia" in a 17 year old adolescent. In one case, a biliary cyst was diagnosed at 3 years of age. Four children underwent successful surgery. The complications of gall stones are difficult to distinguish from episodes of vasoocclusive abdominal pain. Ultrasonography is an easy method of detecting gall stones and may be repeated regularly in children over 10 years of age. All the children operated in this series were improved by surgery. Patients with sickle cell disease must be carefully prepared for general anaesthesia with a strict protocol of blood transfusion which is only possible in well equipped centers. Elective surgery is by far the best management as postoperative complications are much less common than after emergency surgery. A review of the literature shows that the general tendency is for surgical intervention as gall stones are a cause of recurrent abdominal pain, cholecystitis and dangerous infective complications in those patients.
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000235:genetics; D000768:Anesthesia, General; D001649:Bile Duct Diseases / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D002769:Cholelithiasis / Q000209:etiology; D003560:Cysts / Q000209:etiology; D005260:Female; D006720:Homozygote; D006801:Humans; D008297:Male
Pathologie des voies biliaires chez l'enfant drépanocytaire homozygote. A propos de cinq observations récentes.