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Factors influencing outcome after hepatic portoenterostomy among extrahepatic bile duct atresia patients in Hospital Sultanah Bahiyah, Alor Setar

Namasemayam D, Nallusamy M

Med J Malaysia, 2017 12;72(6):329-332.
PMID: 29308768 MyJurnal

INTRODUCTION: Biliary atresia implies high risk of mortality if not diagnosed and treated early. We undertook this study to assess the prognostic factors affecting the outcome of Kasai surgery.
METHODS: A retrospective clinical study was conducted among 58 patients from Hospital Sultanah Bahiyah Alor Setar. Data were analysed with logistic regression analysis using SPSS. Factors studied included age during surgery, bile duct diameter at the porta hepatic, race of patient, preoperative total bilirubin level and cholangitis. These factors were analysed to determine its significance as a prognostic factor affecting the outcome of Kasai surgery.
RESULTS: Older age group above 56 days of life, bile duct diameter at porta hepatis measuring <50μm, pre-operative total bilirubin >10mg% and cholangitis were found to be poor prognostic factors from univariate regression analysis. Variables having p value<0.025 were analysed using the multivariable regression analysis. Only age of patient and diameter of bile duct at the porta hepatis were eligible for this analysis. The final analysis showed that age 57 days of life and above (adjusted odd's ratio (aOR) = 9.412, p value = 0.042, 95% confidence interval (95%CI) = 1.079 to 82.104) and bile duct diameter <50μm (aOR = 13.812, p value = 0.016, 95%CI = 1.616 to 118.042) were significant factors affecting the outcome.
CONCLUSION: In conclusion, age of patient 56 days of life and younger and diameter of bile duct at porta hepatis ≥50μm gave a significantly better outcome after Kasai surgery.

Matched MeSH terms: Portoenterostomy, Hepatic*
Outcome of biliary atresia in Malaysia: a single-centre study

Lee WS, Chai PF, Lim KS, Lim LH, Looi LM, Ramanujam TM

J Paediatr Child Health, 2009 May;45(5):279-85.
PMID: 19493120 DOI: 10.1111/j.1440-1754.2009.01490.x

This study aimed to determine the outcome of the operation of children with biliary atresia (BA) at a tertiary paediatric referral centre in Malaysia.

Matched MeSH terms: Portoenterostomy, Hepatic*
Fulltext Jaundice clearance and cholangitis in the first year following portoenterostomy for biliary atresia

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: Portoenterostomy, Hepatic*
Fulltext Chronic liver disease is universal in children with biliary atresia living with native liver

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: Portoenterostomy, Hepatic