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Fulltext Neonatal cholestasis and biliary atresia: perspective from Malaysia

Lee, W.S.

JUMMEC, 2010;13(2):72-79.
MyJurnal

The liver is an important organ of the human body, playing a major role in the metabolism and storage of nutrients, synthesis of protein and other nutrients, as well as detoxifying many metabolic by-products. The response of the foetal and newborn liver to external insult and injury is limited. This is because the ability of the closely interdependent structures of a developing liver of expressing in the face of a variety of insults is limited as well. Thus most infants with insults to the liver present as cholestatic jaundice with variable degree of pale stools, enlarged liver and conjugated hyperbilirubinaemia. Biliary atresia, an idiopathic condition characterized by progressive fibrosing obliteration of both intra- and extrahepatic bile ducts, is the most important cause of neonatal cholestasis worldwide, including Malaysia. It is also the most important indication for childhood liver transplantation the world over. Challenges facing infants with biliary atresia include a delay in the diagnosis and late surgery, leading to a poor outcome. This often results from a failure to recognise the potential serious nature of an infant with prolonged cholestatic jaundice and pale stools among health care professionals.

Matched MeSH terms: Liver Transplantation
Fulltext Aetiology and outcome of neonatal cholestasis in Malaysia

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: Liver Transplantation/statistics & numerical data*
Etiology, outcome and prognostic indicators of childhood fulminant hepatic failure in the United kingdom

Lee WS, McKiernan P, Kelly DA

J Pediatr Gastroenterol Nutr, 2005 May;40(5):575-81.
PMID: 15861019

OBJECTIVE: To study the etiology, outcome and prognostic indicators in children with fulminant hepatic failure in the United Kingdom.
DESIGN: Retrospective review of all patients <17 years with fulminant hepatic failure from 1991 to 2000. Fulminant hepatic failure was defined as presence of coagulopathy (prothrombin time >24 seconds or International Normalized Ratio >2.0) with or without hepatic encephalopathy within 8 weeks of the onset of symptoms.
SETTING: Liver Unit, Birmingham Children's Hospital, United Kingdom.
RESULTS: Ninety-seven children (48 male, 49 female; median age, 27 months; range, 1 day-192.0 months) were identified with fulminant hepatic failure. The etiologies were: 22 metabolic, 53 infectious, 19 drug-induced, and 3 autoimmune hepatitis. The overall survival rate was 61%. 33% (32/97) recovered spontaneously with supportive management. Fifty-five children were assessed for liver transplantation. Four were unstable and were not listed for liver transplantation; 11 died while awaiting liver transplantation. Liver transplantation was contraindicated in 10 children. Of the 40 children who underwent liver transplantation, 27 survived. Children with autoimmune hepatitis, paracetamol overdose or hepatitis A were more likely to survive without liver transplantation. Children who had a delay between the first symptom of liver disease and the onset of hepatic encephalopathy (median, 10.5 days versus 3.5 days), higher plasma bilirubin (299 micromol/L versus 80 micromol/L), higher prothrombin time (62 seconds versus 40 seconds) or lower alanine aminotransferase (1288 IU/L versus 2929 IU/L) levels on admission were more likely to die of fulminant hepatic failure or require liver transplantation (P < 0.05). On multivariate analysis, the significant independent predictors for the eventual failure of conservative therapy were time to onset of hepatic encephalopathy >7 days, prothrombin time >55 seconds and alanine aminotransferase =2384 IU/L on admission.
CONCLUSIONS: Children with fulminant hepatic failure with severe coagulopathy, lower alanine aminotransferase on admission and prolonged duration of illness before the onset of hepatic encephalopathy are more likely to require liver transplantation. Early referral to a specialized center for consideration of liver transplantation is vital.

Matched MeSH terms: Liver Transplantation*
Asian-Pacific consensus statement on the management of chronic hepatitis B: a 2012 update

Liaw YF, Kao JH, Piratvisuth T, Chan HL, Chien RN, Liu CJ, et al.

Hepatol Int, 2012 Jun;6(3):531-61.
PMID: 26201469 DOI: 10.1007/s12072-012-9365-4

Large volume of new data on the natural history and treatment of chronic hepatitis B virus (HBV) infection have become available since 2008. These include further studies in asymptomatic subjects with chronic HBV infection and community-based cohorts, the role of HBV genotype/naturally occurring HBV mutations, the application of non-invasive assessment of hepatic fibrosis and quantitation of HBV surface antigen and new drug or new strategies towards more effective therapy. To update HBV management guidelines, relevant new data were reviewed and assessed by experts from the region, and the significance of the reported findings was discussed and debated. The earlier "Asian-Pacific consensus statement on the management of chronic hepatitis B" was revised accordingly. The key terms used in the statement were also defined. The new guidelines include general management, indications for fibrosis assessment, time to start or stop drug therapy, choice of drug to initiate therapy, when and how to monitor the patients during and after stopping drug therapy. Recommendations on the therapy of patients in special circumstances, including women in childbearing age, patients with antiviral drug resistance, concurrent viral infection, hepatic decompensation, patients receiving immune suppression or chemotherapy and patients in the setting of liver transplantation and hepatocellular carcinoma, are also included.

Matched MeSH terms: Liver Transplantation
Fulltext Radionuclide therapy of hepatocellular carcinoma

Sundram F

Biomed Imaging Interv J, 2006 Jul;2(3):e40.
PMID: 21614248 MyJurnal DOI: 10.2349/biij.2.3.e40

Hepatocellular carcinoma (HCC) is a malignant tumour of the hepatocyte. It is a common malignancy worldwide and causes almost half a million deaths annually. Asia is a high risk area. Although surgery (hepatectomy or liver transplantation) is the main form of curative treatment, the majority of patients are not eligible for surgery due to extent of tumour and dysfunction of liver. Radiopharmaceuticals used for transarterial treatment of HCC were Yttrium-90 microspheres, Iodine-131 lipiodol, Rhenium-188 lipiodol, and Holmium-166 Chitosan complex. Yittrium-90 microspheres are glass or resin microspheres of mean sphere diameter of 20 to 30 micrometre. The activity administered was about 4 GBq. Reported response rate was about 20%, and median survival was 54 weeks. On inoperable tumours, reported objective response of I-131 lipiodol was 40 to 70%, and median survival was six to nine months. It showed efficacy similar to TACE. In adjuvant treatment following curative resection of HCC, reported three year survival was 86% compared with 46% for the control group. The administered activity in both adjuvant and inoperable HCC was about 2 GBq (55 mCi). Rhenium-188 lipiodol is a new radioconjugate, and using it we treated 70 patients with inoperable HCC. This treatment was a part of a multi-centre trial sponsored by the International Atomic Energy Agency. Partial response was obtained in 17% of cases, while 49% had stable disease at three months, and 34% showed disease progression. In terms of survival, 19% survived one year, 60% for six months, and 90% for three months. The mean activity was about 4.6 GBq (124 mCi). This method was safe and free from adverse effects.

Matched MeSH terms: Liver Transplantation
Acute liver failure--current approach to treatment

Ganesalam K

Med J Malaysia, 2005 Jul;60 Suppl B:127-32.
PMID: 16108193
Matched MeSH terms: Liver Transplantation
Hepatorenal syndrome

McCormick A, Sultan J

Med J Malaysia, 2005 Jul;60 Suppl B:17-21.
PMID: 16108168
Matched MeSH terms: Liver Transplantation
Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific Association for the Study of the Liver (APASL) 2014

Sarin SK, Kedarisetty CK, Abbas Z, Amarapurkar D, Bihari C, Chan AC, et al.

Hepatol Int, 2014 Oct;8(4):453-71.
PMID: 26202751 DOI: 10.1007/s12072-014-9580-2

The first consensus report of the working party of the Asian Pacific Association for the Study of the Liver (APASL) set up in 2004 on acute-on-chronic liver failure (ACLF) was published in 2009. Due to the rapid advancements in the knowledge and available information, a consortium of members from countries across Asia Pacific, "APASL ACLF Research Consortium (AARC)," was formed in 2012. A large cohort of retrospective and prospective data of ACLF patients was collated and followed up in this data base. The current ACLF definition was reassessed based on the new AARC data base. These initiatives were concluded on a 2-day meeting in February 2014 at New Delhi and led to the development of the final AARC consensus. Only those statements which were based on the evidence and were unanimously recommended were accepted. These statements were circulated again to all the experts and subsequently presented at the annual conference of the APASL at Brisbane, on March 14, 2014. The suggestions from the delegates were analyzed by the expert panel, and the modifications in the consensus were made. The final consensus and guidelines document was prepared. After detailed deliberations and data analysis, the original proposed definition was found to withstand the test of time and identify a homogenous group of patients presenting with liver failure. Based on the AARC data, liver failure grading, and its impact on the "Golden therapeutic Window," extra-hepatic organ failure and development of sepsis were analyzed. New management options including the algorithms for the management of coagulation disorders, renal replacement therapy, sepsis, variceal bleed, antivirals, and criteria for liver transplantation for ACLF patients were proposed. The final consensus statements along with the relevant background information are presented here.

Matched MeSH terms: Liver Transplantation
Fulltext Hepatocellular Carcinoma in Malaysia and Its Changing Trend

Raihan R, Azzeri A, H Shabaruddin F, Mohamed R

Euroasian J Hepatogastroenterol, 2018 05 01;8(1):54-56.
PMID: 29963463 DOI: 10.5005/jp-journals-10018-1259

Hepatocellular carcinoma (HCC) is one of the leading causes of death globally. In Malaysia liver cancer is the eighth most common cause of cancer for both gender and fifth most common cause of cancer for males. Liver cancer is a cause of premature death in Malaysia: The trend from 1990 to 2010 was observed upward. Since 1990, the annual years of life lost (YLLs) from liver cancer have increased by 31.5%. Older persons are at higher risk and there is male predominance observed. Curative surgical resection, liver transplantation, and supportive symptomatic care, including percutaneous ethanol injection and radiofrequency ablation (RFA), and noncurative transarterial chemoembolization (TACE) are among available treatment facilities. Yet the survival rate is very poor as majority of patients present at very advanced stage. Hepatitis B virus (HBV) remained the leading cause of HCC in Malaysia. Several studies showed cryptogenic causes, which are mainly nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) among the predominant causes of HCC in Malaysia than hepatitis C virus (HCV), alcohol, or any other reason. This mainly correlates with the increasing incidence of diabetes and obesity in Malaysia. How to cite this article: Raihan R, Azzeri A, Shabaruddin FH, Mohamed R. Hepatocellular Carcinoma in Malaysia and Its Changing Trend. Euroasian J Hepato-Gastroenterol 2018;8(1):54-56.

Matched MeSH terms: Liver Transplantation
Fulltext Mesenchymal stem cell therapy for advanced liver cirrhosis: A case report

Rajaram R, Subramani B, Abdullah BJJ, Mahadeva S

JGH Open, 2017 Dec;1(4):153-155.
PMID: 30483553 DOI: 10.1002/jgh3.12027

Mesenchymal stem cell (MSC) transplant may offer an alternative to liver transplantation in patients with end-stage liver disease. However, its efficacy remains uncertain. MSC was performed on a 50-year-old male with decompensated (Child-Turcotte-Pugh grade C) alcoholic liver cirrhosis due to an absence of donors for adult-deceased and living-related liver transplantation. Autologous bone marrow-derived MSCs were harvested from the patient and cultured using standard protocols. The MSCs were subsequently re-administrated into the liver via hepatic intra-arterial infusion on two separate occasions. After infusion, there was an improvement in biochemical parameters (serum total bilirubin, serum albumin), and a reduction of diuretic use for ascites for up to 8 weeks. However, all biochemical and clinical parameters deteriorated on long-term follow-up without any further infusions. The patient eventually succumbed to his disease. MSC transplantation may have a clinical benefit on adult patients with end-stage liver cirrhosis, but this appears to be transitory.

Matched MeSH terms: Liver Transplantation
Renal function following liver transplantation for unresectable hepatoblastoma

Lee WS, Grundy R, Milford DV, Taylor CM, de Ville de Goyet J, McKiernan PJ, et al.

Pediatr Transplant, 2003 Aug;7(4):270-6.
PMID: 12890004

Combination of cyclosporine (CsA) and tacrolimus immunosuppression post-liver transplantation (LT) and the chemotherapeutic drugs used to treat hepatoblastoma (HB), are nephrotoxic. We aimed to determine the severity and duration of nephrotoxicity in children following LT for unresectable HB. We reviewed all children undergoing LT for unresectable HB at the Liver Unit, Birmingham Children's Hospital, UK, from 1991 to July 2000. Thirty-six children undergoing LT for biliary atresia, matched for age and sex, were selected as controls to compare pre- and post-LT renal function. Renal function was determined by estimation of glomerular filtration rate (eGFR) derived from plasma creatinine using Schwartz's formula. Twelve children with HB (mean age of diagnosis 33 months) who underwent LT (mean age 47 months) and 36 controls (mean age of LT 34 months) were studied. CsA was the main immunosuppressive drug used in each group. The median eGFR before, and at 3, 6, 12, 24 and 36 months after LT in HB group was significantly lower than controls (93 vs. 152, 66 vs. 79, 62 vs. 86, 66 vs. 87, 64 vs. 94, 53 vs. 90 mL/min/1.73 m2, respectively; 0.01 < p < 0.03). The reductions in the median eGFR of both the HB group and controls before and at 36 months after LT were 49 and 41%, respectively. At 36 months after LT, there was a trend for partial recovery of the eGFR in the controls but not in the HB group. Children who underwent LT for unresectable HB had renal dysfunction before transplantation that persisted for 36 months after LT.

Matched MeSH terms: Liver Transplantation
Historical epidemiology of hepatitis C virus in select countries-volume 4

Maaroufi A, Vince A, Himatt SM, Mohamed R, Fung J, Opare-Sem O, et al.

J Viral Hepat, 2017 10;24 Suppl 2:8-24.
PMID: 29105285 DOI: 10.1111/jvh.12762

Due to the introduction of newer, more efficacious treatment options, there is a pressing need for policy makers and public health officials to develop or adapt national hepatitis C virus (HCV) control strategies to the changing epidemiological landscape. To do so, detailed, country-specific data are needed to characterize the burden of chronic HCV infection. In this study of 17 countries, a literature review of published and unpublished data on HCV prevalence, viraemia, genotype, age and gender distribution, liver transplants and diagnosis and treatment rates was conducted, and inputs were validated by expert consensus in each country. Viraemic prevalence in this study ranged from 0.2% in Hong Kong to 2.4% in Taiwan, while the largest viraemic populations were in Nigeria (2 597 000 cases) and Taiwan (569 000 cases). Diagnosis, treatment and liver transplant rates varied widely across the countries included in this analysis, as did the availability of reliable data. Addressing data gaps will be critical for the development of future strategies to manage and minimize the disease burden of hepatitis C.

Matched MeSH terms: Liver Transplantation
End-stage primary biliary cirrhosis in a first generation migrant south Asian population

Anand AC, Elias E, Neuberger JM

Eur J Gastroenterol Hepatol, 1996 Jul;8(7):663-6.
PMID: 8853255

Primary biliary cirrhosis (PBC) is uncommonly described from Asia and it is an extremely rare cause of chronic liver disease in India. Six first generation migrant Asian patients with PBC were seen at the Liver Unit, Queen Elizabeth Hospital, Birmingham during the period 1982-94. All were women and their ages at presentation ranged from 31 to 63 (median 40) years. All were symptomatic for a median of 6 months prior to referral to the unit for transplantation. Itching with or without jaundice was a common presenting feature. Diagnosis was based on raised serum IgM levels, presence of antimitochondrial antibody (titres 100-400) and diagnostic histology. Only one patients had an associated autoimmune disease (coeliac disease). Serum bilirubin level was above 100 mumol/l at the time of presentation in four patients. Four of these patients with end-stage PBC are first generation migrants from south Asia, who have been resident in the West Midlands for the past 10 to 34 years. The total south Asian population of the West Midlands is 276,754; thus, from these four patients alone the estimated prevalence of PBC in the migrant south Asian population is at least 14 per million. However, such data cannot be used to give any accurate assessment of prevalence, for which a population screening programme is required. A higher incidence in the migrant population than in their countries of origin is compatible with an environmental aetiology.

Matched MeSH terms: Liver Transplantation
Fulltext Acute-on-chronic liver failure: consensus recommendations of the Asian Pacific association for the study of the liver (APASL): an update

Sarin SK, Choudhury A, Sharma MK, Maiwall R, Al Mahtab M, Rahman S, et al.

Hepatol Int, 2019 Jul;13(4):353-390.
PMID: 31172417 DOI: 10.1007/s12072-019-09946-3

The first consensus report of the working party of the Asian Pacific Association for the Study of the Liver (APASL) set up in 2004 on acute-on-chronic liver failure (ACLF) was published in 2009. With international groups volunteering to join, the "APASL ACLF Research Consortium (AARC)" was formed in 2012, which continued to collect prospective ACLF patient data. Based on the prospective data analysis of nearly 1400 patients, the AARC consensus was published in 2014. In the past nearly four-and-a-half years, the AARC database has been enriched to about 5200 cases by major hepatology centers across Asia. The data published during the interim period were carefully analyzed and areas of contention and new developments in the field of ACLF were prioritized in a systematic manner. The AARC database was also approached for answering some of the issues where published data were limited, such as liver failure grading, its impact on the 'Golden Therapeutic Window', extrahepatic organ dysfunction and failure, development of sepsis, distinctive features of acute decompensation from ACLF and pediatric ACLF and the issues were analyzed. These initiatives concluded in a two-day meeting in October 2018 at New Delhi with finalization of the new AARC consensus. Only those statements, which were based on evidence using the Grade System and were unanimously recommended, were accepted. Finalized statements were again circulated to all the experts and subsequently presented at the AARC investigators meeting at the AASLD in November 2018. The suggestions from the experts were used to revise and finalize the consensus. After detailed deliberations and data analysis, the original definition of ACLF was found to withstand the test of time and be able to identify a homogenous group of patients presenting with liver failure. New management options including the algorithms for the management of coagulation disorders, renal replacement therapy, sepsis, variceal bleed, antivirals and criteria for liver transplantation for ACLF patients were proposed. The final consensus statements along with the relevant background information and areas requiring future studies are presented here.

Matched MeSH terms: Liver Transplantation/methods
Fulltext Mitochondrial hepatopathies: advances in genetics, therapeutic approaches, and outcomes

Lee WS, Sokol RJ

J Pediatr, 2013 Oct;163(4):942-8.
PMID: 23810725 DOI: 10.1016/j.jpeds.2013.05.036
Matched MeSH terms: Liver Transplantation
Chronic hepatitis B--treatment with nucleoside analogues

Leung N

Med J Malaysia, 2005 Jul;60 Suppl B:22-7.
PMID: 16108169

Nucleot(s)ide analogues are making milestones in the treatment of chronic hepatitis B (CHB) as safe oral therapy. FDA approved lamivudine in adult patients in 1998, adefovir dipivoxil in 2002, and entecavir in March 2005. Lamivudine is effective in viral suppression, ALT normalization, and improvement in histology in both HBeAg positive and HBeAg negative / HBV DNA positive patients. HBeAg seroconversion rates correlate directly with pretreatment ALT levels at 18-30% after one year of therapy. Hepatitis flares may occur if lamivudine is stopped before HBeAg seroconversion. Lamivudine resistant YMDD mutants emerge at a rate of 15-20% per year of therapy; often associated with the rebound viraemia, relapse of hepatitis or even hepatic decompensation. Durability of response off lamivudine therapy is not satisfactory and may be dependent on duration of therapy post-seroconversion. Lamivudine is well tolerated with few serious adverse events, even in patients with decompensated cirrhosis. Long term therapy in viraemic patients with advanced fibrosis or cirrhosis delays clinical progression. Adefovir dipivoxil is an oral prodrug of adefovir. 10 mg daily is effective in suppressing both wild-type HBV and YMDD mutants, normalising ALT and improving histology. Adefovir dipivoxil has been shown to be well tolerated in longterm therapy. Renal toxicity reported in higher dosages is rarely seen except among patients with creatinine clearance less than 50 ml/min. Adefovir resistance may emerge and the overall rate is much lower than lamivudine, reaching 18% after 4 years of therapy. Adefovir-resistant mutants (rt N236T) are susceptible to lamivudine and entecavir. Little data is available for durability of response off therapy. Entecavir is an oral nucleoside analogue with a recommended dosage of 0.5 mg daily for nucleoside-naive patients, and 1 mg daily for lamivudine-refractory patients. It is a potent antiviral and may also reduced intrahepatitic cccDNA. Entecavir resistance so far has only been detected in lamivudine resistant patients in the one-year studies. Patient counseling is very important to decide on the choice among available therapeutic options. The assessment of the risks/benefits of each option should be carefully explained to individual patient.

Matched MeSH terms: Liver Transplantation
Health-Related Quality of Life in Children with Biliary Atresia Living with Native Livers

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: Liver Transplantation
Systemic inflammatory response syndrome in acute-on-chronic liver failure: Relevance of 'golden window': A prospective study

Choudhury A, Kumar M, Sharma BC, Maiwall R, Pamecha V, Moreau R, et al.

J Gastroenterol Hepatol, 2017 Dec;32(12):1989-1997.
PMID: 28374414 DOI: 10.1111/jgh.13799

BACKGROUND AND AIM: Systemic inflammatory response syndrome (SIRS) is an early marker of sepsis and ongoing inflammation and has been reported in large proportion of acute-on-chronic liver failure (ACLF) patients. Whether sepsis is the cause or the result of liver failure is unclear and is vital to know. To address this, the study investigated the course and outcome of ACLF patients without SIRS/sepsis.
METHODS: Consecutive ACLF patients were monitored for the development of SIRS/sepsis and associated complications and followed till 90 days, liver transplant or death.
RESULTS: Of 561 patients, 201 (35.8%) had no SIRS and 360 (64.2%) had SIRS with or without infection. New onset SIRS and sepsis developed in 74.6% and 8% respectively in a median of 7 (range 4-15) days, at a rate of 11% per day. The cumulative incidence of new SIRS was 29%, 92.8%, and 100% by days 4, 7, and 15. Liver failure, that is, bilirubin > 12 mg/dL (odds ratio [OR] = 2.5 [95% confidence interval {CI} = 1.05-6.19], P = 0.04) at days 0 and 4, and renal failure at day 4 (OR = 6.74 [95%CI = 1.50-13.29], P = 0.01), independently predicted new onset SIRS. Absence of SIRS in the first week was associated with reduced incidence of organ failure (20% vs 39.4%, P = 0.003), as was the 28-day (17.6% vs 36%, P = 0.02) and 90-day (27.5% vs 51%,P = 0.002) mortality. The 90-day mortality was 61.6% in the total cohort and that for those having no SIRS and SIRS at presentation were 42.8% and 65%, respectively (P Liver failure predicts the development of SIRS. New onset SIRS in the first week is an important determinant of early sepsis, organ failure, and survival. Prompt interventions in this 'golden window' before development of sepsis may improve the outcome of ACLF.

Matched MeSH terms: Liver Transplantation
Pre-admission consultation and late referral in infants with neonatal cholestasis

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: Liver Transplantation
Characteristics and outcome of autoimmune liver disease in Asian children

Lee WS, Lum SH, Lim CB, Chong SY, Khoh KM, Ng RT, et al.

Hepatol Int, 2015 Apr;9(2):292-302.
PMID: 25788179 DOI: 10.1007/s12072-014-9558-0

BACKGROUND: Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population.

METHODS: Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia.

RESULTS: At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [survival with native liver vs. LT or death (duration between onset of disease and treatment; median ± standard error) = 2.5 ± 2.9 months vs. 24.0 ± 13.3 months; p = 0.012].

CONCLUSIONS: Although remission was achieved in the majority of patients with prednisolone and/or azathioprine therapy, delay in seeking diagnosis and treatment adversely affects the outcome of childhood AILD in Malaysia.

Matched MeSH terms: Liver Transplantation

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