Article

Early Diagnosis of Extrahepatic Biliary Atresia in an Open-Access Medical System

Department of Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, Maryland, United States of America
PLoS ONE (Impact Factor: 3.53). 11/2012; 7(11):e49643. DOI: 10.1371/journal.pone.0049643
Source: PubMed

ABSTRACT Biliary atresia (BA) is the most common cause of cholestatic jaundice in infancy. Early diagnosis and surgical management, ideally before 60 days of age, result in improved outcomes. We aimed to determine the age at diagnosis of BA in the Military Health System (MHS) and to compare the age at diagnosis by access to care models. We hypothesized that children with BA receiving primary care in military facilities have an earlier age at diagnosis due to decreased economic and access barriers.
Data for all Tricare enrollees born in fiscal years 2004-2008 with a diagnosis of BA were extracted from MHS databases. Non-parametric tests, Kaplan-Meier curves and log rank tests compared differences in age at diagnosis by type of primary care facility, gender, prematurity and presence of additional anomalies.
64 subjects were identified within the five year period. Median age at diagnosis was 40 days [range 1-189], with 67% diagnosed by 60 days and 80% by 90 days. 45 (70%) received civilian primary care within the MHS. There was no difference in the median age at diagnosis between subjects in the MHS with civilian primary care vs. military primary care (37 days [1-188] vs. 46 days [1-189]; p = 0.58).
In the MHS, two-thirds of infants with biliary atresia are diagnosed prior to 60 days of life. Gender, prematurity or presence of additional anomalies do not affect the timing of diagnosis. Civilian and military primary care models within the MHS make timely diagnoses of biliary atresia at equivalent rates.

0 Bookmarks
 · 
80 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Biliary atresia is the leading cause of extrahepatic obstructive jaundice in the newborn and is the single most frequent indication for liver transplantation in children. The cause of biliary atresia is unknown, although several mechanisms have been postulated to explain the inflammatory process that obliterates the bile ducts. Most interest has been directed toward viral infections. Information about the epidemiologic characteristics of biliary atresia in well-defined populations is lacking but is essential for developing and addressing hypotheses of causation for the disease. Infants with biliary atresia were identified in metropolitan Atlanta from 1968 through 1993 by a population-based birth defects surveillance system that ascertains infants with serious birth defects in the first year of life using active case ascertainment. Birth prevalence rates were analyzed for spatial and temporal clustering and effects attributable to county of residence, sex, race, maternal age, parity, and birth weight. Logistic regression was used to study the independent effects of the risk factors and to look for interactions. Fifty-seven infants with biliary atresia were identified, for a rate of 0.73 per 10,000 live births. There was significant seasonal clustering of the disease, with rates three times higher from December through March compared with rates from April through July. Rates were significantly higher among nonwhite infants compared with white infants (0.96 vs 0.44 per 10,000 live births) and infants born at term with low birth weights (<2500 g) compared with infants born at term with normal birth weights (> or = 2500 g) (2.62 vs 0.75 per 10,000 live births). Our study is the first in the United States to describe the epidemiologic characteristics of biliary atresia using a population-based approach. The demonstration of significant seasonal clustering provides support for theories that biliary atresia may be caused by environmental exposure (consistent with a viral cause) during the perinatal period.
    PEDIATRICS 03/1997; 99(3):376-82. DOI:10.1542/peds.99.3.376 · 5.30 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Age at diagnosis is a modifiable risk factor in outcomes after hepatoportoenterostomy in biliary atresia; however, distinguishing biliary atresia from other more common causes of prolonged neonatal jaundice can be difficult. To focus attention on diagnosis of biliary atresia, we analyzed secular trends in the age at diagnosis, and other factors that might influence outcome. We performed a retrospective analysis of 55 consecutive infants with biliary atresia presenting to a single academic pediatric center over 15-year period from 1990 to 2004. The median age at diagnosis was 60 days (range: 21-152). In recent era (2000-2004), the median age was 69.0 days, compared with 48.5 days (1990-1994) and 59.5 days (1995-1999), respectively. Consistent with previous studies, the median age at diagnosis of those with poor outcomes (death or liver transplant) exceeded those with good outcomes after the hepatoportoenterostomy (72 vs 52 days, P < .001). The lack of improvement, or a concerning trends toward an increase in the age at diagnosis of biliary atresia, is perhaps attributable to neonatal follow-up practices. Efforts to make an earlier diagnose of this important condition deserve wider application and study.
    PEDIATRICS 06/2008; 121(5):e1438-40. DOI:10.1542/peds.2007-2709 · 5.30 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: For the primary care provider, cholestatic jaundice in infancy, defined as jaundice caused by an elevated conjugated bilirubin, is an uncommon but potentially serious problem that indicates hepatobiliary dysfunction. Early detection of cholestatic jaundice by the primary care physician and timely, accurate diagnosis by the pediatric gastroenterologist are important for successful treatment and a favorable prognosis. The Cholestasis Guideline Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnostic evaluation of cholestatic jaundice in the infant. The Cholestasis Guideline Committee, consisting of a primary care pediatrician, a clinical epidemiologist (who also practices primary care pediatrics), and five pediatric gastroenterologists, based its recommendations on a comprehensive and systematic review of the medical literature integrated with expert opinion. Consensus was achieved through the Nominal Group Technique, a structured quantitative method. The Committee examined the value of diagnostic tests commonly used for the evaluation of cholestatic jaundice and how those interventions can be applied to clinical situations in the infant. The guideline provides recommendations for management by the primary care provider, indications for consultation by a pediatric gastroenterologist, and recommendations for management by the pediatric gastroenterologist. The Cholestasis Guideline Committee recommends that any infant noted to be jaundiced at 2 weeks of age be evaluated for cholestasis with measurement of total and direct serum bilirubin. However, breast-fed infants who can be reliably monitored and who have an otherwise normal history (no dark urine or light stools) and physical examination may be asked to return at 3 weeks of age and, if jaundice persists, have measurement of total and direct serum bilirubin at that time. This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition on the evaluation of cholestatic jaundice in infants. The American Academy of Pediatrics has also endorsed these recommendations. These recommendations are a general guideline and are not intended as a substitute for clinical judgment or as a protocol for the care of all patients with this problem.
    Journal of Pediatric Gastroenterology and Nutrition 09/2004; 39(2):115-28. DOI:10.1097/00005176-200408000-00001 · 2.87 Impact Factor

Preview

Download
0 Downloads
Available from