ABSTRACT
Seven cases presenting with the features of Byler's disease (BD) are reported. The diagnosis of BD can only be made after exclusion of other causes of cholestasis. It seems likely that BD is a syndrome and that more accurate biological and histological investigations will allow to differentiate several entities within this affection. At the present, liver transplantation appears to be the only rational treatment.
Subject(s)
Cholestasis, Intrahepatic/genetics , Liver Cirrhosis/etiology , Liver/pathology , Child , Child, Preschool , Cholestasis, Intrahepatic/complications , Cholestasis, Intrahepatic/pathology , Female , Humans , Infant , Liver/ultrastructure , Liver Cirrhosis/pathology , MaleABSTRACT
The Zollinger-Ellison syndrome with characteristic symptoms is described in an 11 year old girl. Coeliac and mesenteric arteriography showed a pancreatic gastrin-secreting tumor. After total gastrectomy the condition improved but the associated Cushing's syndrome deteriorated. Surgical removal of the tumor was not possible and the child was treated with chemotherapy (5-fluorouracil and cyclophosphamide) and OP'DDD.
Subject(s)
Cushing Syndrome/etiology , Zollinger-Ellison Syndrome/complications , Celiac Artery/diagnostic imaging , Child , Cyclophosphamide/therapeutic use , Female , Fluorouracil/therapeutic use , Gastrectomy , Humans , Male , Mesenteric Arteries/diagnostic imaging , Paraneoplastic Syndromes/etiology , Radiography , Zollinger-Ellison Syndrome/diagnosis , Zollinger-Ellison Syndrome/therapySubject(s)
Bile Ducts/abnormalities , Cholestasis/etiology , Rose Bengal , Diagnosis, Differential , Humans , Infant, NewbornSubject(s)
Failure to Thrive/genetics , Fetal Growth Retardation/genetics , Hypoaldosteronism/genetics , Steatorrhea/genetics , Adult , Consanguinity , Failure to Thrive/diagnosis , Female , Fetal Growth Retardation/diagnosis , Follow-Up Studies , Humans , Infant , Infant, Newborn , Pedigree , Pregnancy , Steatorrhea/diagnosisABSTRACT
Hepatic porto-enterostomy or cholecystostomy (Kasai's procedure) was successful in restoring bile flow in 31 of 49 patients with "noncorrectable" extrahepatic biliary atresia. However, all but one of the 31 developed acute or chronic complications such as cholangitis, bile peritonitis, or portal hypertension. During a five-year follow-up period, 26 (53%) died while 9 of the 23 survivors continue to manifest chronic or recurrent cholangitis. Thirteen of the 19 survivors who are more than one year of age have developed portal hypertension. These complications limit the prognosis of infants with "noncorrectable" biliary malformations.
