R. (1976). Archives of Disease in Childhood, 51, 763. Extrahepatic biliary atresia versus neonatal hepatitis: review of 137 prospectively investigated infants. In a prospective regional survey of neonatal hepatitis syndrome 32 infants had extrahepatic biliary atresia (EHBA) and 103 had hepatitis. No cause for the lesion was found in infants with extrahepatic biliary atresia, but in 32 with hepatitis a specific cause was identified, 24 having genetic deficiency of the serum protein oc1-antitrypsin. No differences were observed in parental age, mother's health in pregnancy, month of birth, birth order, or sex of the infants. Familial idiopathic hepatitis occurred in 3 of 67 sibs of patients with idiopathic hepatitis, but the 33 sibs of EHBA patients had no liver disease. Of the infants with hepatitis, 36 were of low birthweight, <2-5 kg, and 23 were born prematurely. Infants with biliary atresia were all of normal birthweight and only one was born prematurely.Consideration of clinical and biochemical abnormalities in the first 2 months of life showed no differences between the two groups except that infants with EHBA were more commonly jaundiced from birth (80%) and had more frequently acholic stools (83%). The frequency of these features in patients with hepatitis being 68% and 52%. Standard tests of liver function were not discriminatory. Percutaneous liver biopsies were diagnostic in 75 % of those with EHBA and in 92% of those with hepatitis. The 131I Rose Bengal faecal excretion was less than 10°/o in 26 of 28 infants with EHBA and in only 5 of 18 with hepatitis. These latter two investigations together allowed a correct preoperative diagnosis of EHBA in all instances. Bile drainage was achieved surgically in only 3 cases. A major reason for these poor results may have been the late referral of cases for diagnosis and laparotomy, which should be performed as soon as the diagnosis is suspected and always by 70 days of age.
suMMARY To document the clinical features and complications of fulminant hepatic failure in childhood, 31 consecutive cases (of whom only 9 survived) were reviewed. Of 26 children with acute hepatitis (HbSAg-negative), liver function steadily deteriorated in all but 2, and encephalopathy occurred within 3 weeks of the onset of symptoms in all except 3 of them. Eight of these patients survived, as did one of 3 in which this deterioration was caused by paracetamol overdosage. Single cases due to Amanita phalloides and halothane died. Encephalopathy lasted from 2 to 16 days in the survivors, and from one to 20 days in the fatal cases. The severity fluctuated by more than one grade in 9 patients. The outcome was not related to the age or sex of patient, clinical or biochemical abnormalities at presentation, or to the duration of the encephalopathy. Prothrombin time was prolonged by more than 90 seconds in 10 fatal cases, but in none of the survivors. The outcome was related to the severity ofthe encephalopathy, only one (6%) of 19 children in grade 4 coma surviving, and to the occurrence of neurological complications-particularly brain stem dysfunction (9 cases), decerebrate posturing (12 cases), and convulsions (7 cases). Massive gastrointestinal bleeding (14 cases) and renal failure (10 cases) were confined to the fatal group. At necropsy 7 (54%) of 13 had cerebral oedema.Hypoglycaemia, septicaemia, respiratory tract infections, ascites, and haemopoietic complications occurred both in fatal cases and survivors. Although liver function tests and liver biopsy appearances remained abnormal in survivors for 24 and 30 months respectively, these children developed normally without evident disease during or after this period.Children with fulminant hepatic failure and severe encephalopathy develop major pathophysiological complications affecting almost every system. Such complications must be prevented or vigorously treated. The mortality is no lower than in adults. Effective treatment must be instituted before grade 4 coma is established.Fulminant hepatic failure with hepatic encephalopathy is a raxe but grave complication of acute hepatic damage from viruses or drugs.1-2 The mortality, 70 to 95 %, may be lower in children than in adults1 3 but in one report age had no
Oesophageal varices in 21 children with portal hypertension (intrahepatic 13, extrahepatic 8) were treated with sclerotherapy using ethanolamine oleate injected via a fibreoptic endoscope. Repeat endoscopy confirmed variceal obliteration in 18 children with a mean of 3.5 (range 1-8) injections. No patient has bled since obliteration of varices. Complications were oesophageal stricture (2 patients), oesophageal ulcer (5 patients) and haemorrhage within 3 days of injection (5 patients). The early results suggest that this is a satisfactory method for the treatment of oesophageal varices in children.
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