Studies in Neonatal Hepatitis and Biliary Atresia

Thaler, Manning Michael

doi:10.1001/archpedi.1968.02100020282008

Cited by 23 publications

(6 citation statements)

References 10 publications

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“…In the majority of patients with EHBA the correct diagnosis was made. Nevertheless, in a number of instances the biopsy was not considered suggestive of EHBA though this proved to be the correct diagnosis, a difficulty reported by other authors (Alagille, 1972;Thaler and Gellis, 1968). In this series the timing of percutaneous liver biopsy in relation to the duration of jaundice did not seem to influence significantly the correct histological diagnosis of EHBA.…”

Section: Discussionmentioning

confidence: 45%

“…The combination of percutaneous liver biopsy and 131I Rose Bengal faecal excretion test thus distinguishes satisfactorily between patients with extrahepatic biliary obstruction who require laparotomy in case they have a surgically correctable lesion, and those with hepatitis. The observations of Thaler and Gellis (1968), that laparotomy and careful examination of extrahepatic biliary system caused an increased incidence of cirrhosis in neonatal hepatitis, have not been refuted and remain a powerful argument against the unnecessary use of laparotomy to detect remedial lesions of the biliary tract.…”

Section: Discussionmentioning

confidence: 99%

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

Mowat¹,

Psacharopoulos²

1976

Archives of Disease in Childhood

123

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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.

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Section: Discussionmentioning

confidence: 45%

Section: Discussionmentioning

confidence: 99%

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

Mowat¹,

Psacharopoulos²

1976

Archives of Disease in Childhood

123

View full text Add to dashboard Cite

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“…The quest for finding clinical and laboratory features that distinguish BA from other causes of neonatal cholestasis has been ongoing for over 50 years [11–20]. Early investigations of over 800 infants in five separate reports from Boston, Toronto, London, Houston, and Bicêtre demonstrated a difficulty in clinically distinguishing BA from intrahepatic cholestasis in a significant number of infants [11–15].…”

Section: Discussionmentioning

confidence: 99%

“…Early investigations of over 800 infants in five separate reports from Boston, Toronto, London, Houston, and Bicêtre demonstrated a difficulty in clinically distinguishing BA from intrahepatic cholestasis in a significant number of infants [11–15]. Infants with BA more frequently had acholic stools, had less failure to thrive, and had more pronounced elevation in biochemical markers of bile duct and canalicular injury, although these features were not uniformly discriminative.…”

Section: Discussionmentioning

confidence: 99%

Initial assessment of the infant with neonatal cholestasis—Is this biliary atresia?

et al. 2017

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IntroductionOptimizing outcome in biliary atresia (BA) requires timely diagnosis. Cholestasis is a presenting feature of BA, as well as other diagnoses (Non-BA). Identification of clinical features of neonatal cholestasis that would expedite decisions to pursue subsequent invasive testing to correctly diagnose or exclude BA would enhance outcomes. The analytical goal was to develop a predictive model for BA using data available at initial presentation.MethodsInfants at presentation with neonatal cholestasis (direct/conjugated bilirubin >2 mg/dl [34.2 μM]) were enrolled prior to surgical exploration in a prospective observational multi-centered study (PROBE–NCT00061828). Clinical features (physical findings, laboratory results, gallbladder sonography) at enrollment were analyzed. Initially, 19 features were selected as candidate predictors. Two approaches were used to build models for diagnosis prediction: a hierarchical classification and regression decision tree (CART) and a logistic regression model using a stepwise selection strategy.ResultsIn PROBE April 2004-February 2014, 401 infants met criteria for BA and 259 for Non-BA. Univariate analysis identified 13 features that were significantly different between BA and Non-BA. Using a CART predictive model of BA versus Non-BA (significant factors: gamma-glutamyl transpeptidase, acholic stools, weight), the receiver operating characteristic area under the curve (ROC AUC) was 0.83. Twelve percent of BA infants were misclassified as Non-BA; 17% of Non-BA infants were misclassified as BA. Stepwise logistic regression identified seven factors in a predictive model (ROC AUC 0.89). Using this model, a predicted probability of >0.8 (n = 357) yielded an 81% true positive rate for BA; <0.2 (n = 120) yielded an 11% false negative rate.ConclusionDespite the relatively good accuracy of our optimized prediction models, the high precision required for differentiating BA from Non-BA was not achieved. Accurate identification of BA in infants with neonatal cholestasis requires further evaluation, and BA should not be excluded based only on presenting clinical features.

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“…Its clinical picture, histology, and prognosis have been well described (Craig and Landing, 1952;Brent, 1962;Gellis and Kibrick, 1964;Thaler and Gellis, 1968). The 3 infants described (Table I) fulfil the criteria of the syndrome of neonatal hepatitis.…”

Section: Discussionmentioning

confidence: 99%