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  1. Karnameedi S, Lim CT
    Med J Malaysia, 1997 Dec;52(4):342-7.
    PMID: 10968111
    Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created.
    Matched MeSH terms: Biliary Atresia/complications*
  2. Halim AJ, Yakin F
    Med J Malaysia, 1983 Dec;38(4):327-30.
    PMID: 6599993
    Eight infants between the ages of one and three months with prolonged conjugated hyperbilirubinaemia had duodenal aspirations performed to differentiate between biliary atresia and neonatal hepatitis. Four infants had bile-stained aspirates and in all of them the jaundice subsided completely by eight months of age. We have found duodenal aspiration a safe, inexpensive and simple procedure to undertake in helping us make a rapid differentiation between patients with conjugated hyperbilirubinaemia requiring urgent laparotomy and those that do not require surgery. This approach appears to be reliable although further studies need to be undertaken.
    Matched MeSH terms: Biliary Atresia/complications
  3. Selvalingam S, Mahmud MN, Thambidorai CR, Zakaria Z, Mohan N, Isa, et al.
    Med J Malaysia, 2002 Mar;57(1):92-6.
    PMID: 14569724 MyJurnal
    Sixty-one patients with biliary atresia, who underwent portoenterostomy (PE) between 1992 to 1998 in the Institute of Pediatrics, Kuala Lumpur and were followed for a period of one year, were studied to analyze the factors associated with jaundice clearance and cholangitis following PE. Sex distribution was equal. Majority of patients were Malays. Mean age in days at admission to the surgical ward was 66.90 +/- 23.36 and mean age at PE was 75.85 +/- 24.05. At the end of one-year follow-up, six patients (10%) had died, 35 (57%) developed one or more attacks of cholangitis, 35 (57%) had portal hypertension, eight (13%) liver failure and six patients had esophageal variceal bleeding. Thirty-three patients (54%) had jaundice clearance with a mean clearance time of 85 days after PE. The study shows that when the ductules in the porta hepatis were < 150 mu in size, persistence of jaundice after PE and the incidence of cholangitis in the first post-operative year were higher; patients with cholangitis in the first year had lower rate of jaundice clearance. Jaundice clearance was achieved in more patients when their postnatal age at the time of PE was lower but the relationship was not linear. Age at PE also did not have a linear temporal relationship to the incidence of cholangitis and the size of portal ductules. Prospective, multi-center based local studies on a bigger patient population are needed to identify other indicators of successful outcome following PE. This would help to define the indications for primary liver transplantation in the local population.
    Matched MeSH terms: Biliary Atresia/complications
  4. Lee WS, Chai PF, Boey CM, Looi LM
    Singapore Med J, 2010 May;51(5):434-9.
    PMID: 20593150
    Little is known about the epidemiology, causes and outcomes of neonatal cholestasis in the Asian population beyond Japan and Taiwan.
    Matched MeSH terms: Biliary Atresia/complications*
  5. Tan HS, Balasubramaniam IS, Hss AS, Yeong ML, Chew CC, Singh RP, et al.
    BMC Pediatr, 2019 05 29;19(1):174.
    PMID: 31142302 DOI: 10.1186/s12887-019-1550-3
    BACKGROUND: Prolonged neonatal jaundice (PNNJ) is often caused by breast milk jaundice, but it could also point to other serious conditions (biliary atresia, congenital hypothyroidism). When babies with PNNJ receive a routine set of laboratory investigations to detect serious but uncommon conditions, there is always a tendency to over-investigate a large number of well, breastfed babies. A local unpublished survey in Perak state of Malaysia revealed that the diagnostic criteria and initial management of PNNJ were not standardized. This study aims to evaluate and improve the current management of PNNJ in the administrative region of Perak.

    METHODS: A 3-phase quasi-experimental community study was conducted from April 2012 to June 2013. Phase l was a cross-sectional study to review the current practice of PNNJ management. Phase ll was an interventional phase involving the implementation of a new protocol. Phase lll was a 6 months post-interventional audit. A registry of PNNJ was implemented to record the incidence rate. A self-reporting surveillance system was put in place to receive any reports of biliary atresia, urinary tract infection, or congenital hypothyroidism cases.

    RESULTS: In Phase I, 12 hospitals responded, and 199 case notes were reviewed. In Phase II, a new protocol was developed and implemented in all government health facilities in Perak. In Phase III, the 6-month post-intervention audit showed that there were significant improvements when comparing mean scores of pre- and post-intervention: history taking scores (p 

    Matched MeSH terms: Biliary Atresia/complications
  6. Lee WS, Ong SY
    Ann Acad Med Singap, 2016 Feb;45(2):61-8.
    PMID: 27125347
    INTRODUCTION: This study aimed to quantify and investigate factors affecting the health-related quality of life (HRQoL) in children with biliary atresia (BA) living with their native livers.

    MATERIALS AND METHODS: A cross-sectional study on the HRQoL using the PedsQL4.0 generic core scales in children with BA aged between 2 to 18 years followed up at the University Malaya Medical Centre (UMMC) in Malaysia was conducted. Two groups, consisting of healthy children and children with chronic liver disease (CLD) caused by other aetiologies, were recruited as controls.

    RESULTS: Children with BA living with their native livers (n = 36; median (range) age: 7.4 (2 to 18) years; overall HRQoL score: 85.6) have a comparable HRQoL score with healthy children (n = 81; median age: 7.0 years; overall HQRoL score: 87.4; P = 0.504) as well as children with CLD (n = 44; median age: 4.3 years; overall score: 87.1; P = 0.563). The HRQoL of children with BA was not adversely affected by having 1 or more hospitalisations in the preceding 12 months, the presence of portal hypertension, older age at corrective surgery (>60 days), a lower level of serum albumin (≤34 g/L) or a higher blood international normalised ratio (INR) (≥1.2). Children who had liver transplantation for BA did not have a significantly better HRQoL as compared to those who had survived with their native livers (85.4 vs 85.7, P = 0.960).

    CONCLUSION: HRQoL in children with BA living with their native livers is comparable to healthy children.

    Matched MeSH terms: Biliary Atresia/complications
  7. Lee WS
    J Paediatr Child Health, 2008 Jan;44(1-2):57-61.
    PMID: 17640283
    To study factors leading to delayed referral in neonatal cholestasis at a tertiary centre in Malaysia.
    Matched MeSH terms: Biliary Atresia/complications
  8. Lee WS, Ong SY, Foo HW, Wong SY, Kong CX, Seah RB, et al.
    World J Gastroenterol, 2017 Nov 21;23(43):7776-7784.
    PMID: 29209118 DOI: 10.3748/wjg.v23.i43.7776
    AIM: To examine the medical status of children with biliary atresia (BA) surviving with native livers.

    METHODS: In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices.

    RESULTS: Fifty-two children [females = 32, 62%; median age 7.4 years, n = 35 (67%) older than 5 years] with BA (median age at surgery 60 d, range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension (40%, n = 21; 2 younger than 5 years), cholangitis (36%) and bleeding varices (25%, n = 13; 1 younger than 5 years). Fifteen (29%) had no clinical complications of CLD and three (6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient (2%).

    CONCLUSION: Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age, underscoring the importance of medical surveillance for complications of BA starting at a young age.

    Matched MeSH terms: Biliary Atresia/complications*
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