Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants.

Abstract

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.

Comparison of different diagnostic methods in infants with Cholestasis

“…Our study found that liver needle biopsy was the most reliable method with the highest accuracy rate of 96.9%, which is similar to those in previous reports [2,12,15] .…”

“…Prevalences of acholic stools in our study were 94.7% for BA and 56.5% for INH that are comparable to the study by Mowat et al [2] (i.e., 83% and 52%, respectively). The mean values of ALT, AST and ALP in BA were 161 ± 107, 261 ± 141, and 2150 ± 830, respectively, and in INH were 212 ± 198, 324 ± 258, and 1791 ± 852, respectively, and the difference was not statistically significant.…”

“…Idiopathic neonatal hepatitis (INH) and biliary atresia (BA) are two main causes [1,2] . It is important to distinguish INH from BA in an infant presented with jaundice, as the former purely needs a medical management and the later requires surgical intervention as soon as possible.…”

Yeast Opsonisation and Complement in Children with Liver Disease. Analysis of 69 Cases

“…Diagnosis was confirmed histologically with or without other specific tests, for example, alpha-l-antitrypsin phenotyping in each case. Thirtythree patients had neonatal hepatitis syndrome, defined as obstructive jaundice in an infant under 4 months of age (16). Our patients included nine patients with biliary atresia tested before surgery and five studied only postoperatively, as well as 19 infants with idiopathic neonatal hepatitis.…”

Diagnóstico diferencial de colestase neonatal: parâmetros clínicos e laboratoriais

“…14 For early identification of BA cases, the use of a stool color card, 3,15 ultrasonography for the presence of the triangular cord sign, 16,17 and also correlation of clinical, laboratory and histopathological findings have been proposed. 3,14,[18][19][20] This study is aimed at evaluating whether clinical and laboratory parameters may help to establish differentiation between IHC and EHC. Patients were divided into two groups: group I with IHC and group II with EHC.…”