AIMTo examine the medical status of children with biliary atresia (BA) surviving with native livers.METHODSIn 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.RESULTSFifty-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%).CONCLUSIONClinical 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.

Vitamin D deficiency and insufficiency is common in children with CLD in a tropical country. Regular monitoring of vitamin D status and screening for metabolic bone disease in all children with CLD is recommended. Higher dose of oral supplement or parenteral route should be considered, especially in those with bilirubin ≥34 μmol/L.

Background: Current knowledge on the clinical features and natural history of childhood primary sclerosing cholangitis e inflammatory bowel disease in Asia is limited. We described the presenting features and natural history of primary sclerosing cholangitisinflammatory bowel disease seen in a cohort of Southeast Asian children. Methods: We conducted a retrospective review of childhood primary sclerosing cholangitisinflammatory bowel disease from three tertiary centers in Singapore and Malaysia. Results: Of 24 patients (boys, 58%; median age at diagnosis: 6.3 years) with primary sclerosing cholangitis-inflammatory bowel disease (ulcerative colitis, n Z 21; Crohn's disease, n Z 1; undifferentiated, n Z 2), 63% (n Z 15) were diagnosed during follow-up for colitis, and 21% (n Z 5) presented with acute or chronic hepatitis, 17% (n Z 4) presented simultaneously. Disease phenotype of liver involvement showed 79% had sclerosing cholangitis-autoimmune hepatitis overlap, 54% large duct disease, and 46% small duct disease. All patients received immunosuppression therapy. At final review after a median [AES.D.] duration follow-up of

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. Key words: Chronic liver disease, Survivors

Aim Quality of life (QoL) in children with inflammatory bowel disease (IBD) is often impaired by underlying disease. We evaluated factors affecting health‐related QoL (HRQoL) in Malaysian children with IBD. Methods A cross‐sectional study using IMPACT‐III questionnaires evaluating HRQoL in children aged 8–17 years with duration of IBD of ≥6 months was conducted. IMPACT‐III, a validated instrument designed to measure HRQoL in children with IBD, was used. Higher IMPACT‐III (maximum = 100) score indicates better HRQoL. Impact of socio‐demographic and clinical factors of IBD on the HRQoL was evaluated. Paediatric Crohn's disease (CD) and ulcerative colitis (UC) activity indices were used to classify disease severity. Results A total of 75 children (UC = 44, CD = 41; mean (SD) age at diagnosis 8.2 (3.5) years) were interviewed at mean age of 12.8 (2.7) years. Mean IMPACT‐III score was significantly lower in children with more severe disease (mild: 71.8 (13.6) vs. moderate: 65.5 (10.9) vs. severe: 46.3 (14.5); P < 0.001), history of hospitalisation (yes: 64.0 (14.0) vs. none: 74.1 (12.2), P = 0.034) and a higher number of admissions (r = −0.352, P = 0.041) in preceding 6 months. Diagnosis at a younger age (r = −0.31, P = 0.007) and a longer duration of disease (r = 0.286, P = 0.013) was associated with higher score. A higher weight‐for‐age (r = 0.261, P = 0.023) or body mass index‐for‐age z‐score (r = 0.235, P = 0.042) was correlated with a better body image domain score, respectively. Conclusions In Malaysian children with IBD, HRQoL was adversely affected by a more severe disease. Better control of disease activity and maintaining long‐term remission are important to improve the HRQoL in childhood IBD.