METHODS: We reviewed the medical records of BA patients who underwent Kasai procedure at the Dr. Sardjito Hospital, Indonesia from August 2012 to December 2018. The cut-off values of TB7/TB0, GGT7/GGT0, and ALT7/ALT0 for prediction of patients' survival were determined by receiver operating characteristics (ROC) curves. Log-rank tests were utilised to test the association between cut-off values and overall survival.
RESULTS: In all 46 BA patients (23 males and 23 females) after Kasai procedure were included, consisting of one type 1, 17 type 2A, seven type 2B, and 21 type 3. The cut-off values of TB7/TB0, ALT7/ALT0 and GGT7/GGT0 for overall survival was 0.455 (sensitivity 87.5%, specificity 22.7%, area under curve (AUC) 0.59; 95% Confidence Interval (95%CI): 0.42, 0.75), 0.481 (sensitivity 87.5%, specificity 18.2%, AUC 0.49; 95%CI: 0.31, 0.65), and and 0.31 (sensitivity 79.2%, specificity 9.1%, AUC 0.34; 95%CI: 0.18, 0.50), respectively. However, these cut-off values were not significantly associated with overall survival, with p-values of 0.18, 0.49, and 0.56, respectively.
CONCLUSION: The TB7/TB0, ALT7/ALT0, and GGT7/GGT0 might not predict the overall survival of BA patients after Kasai procedure. Further multicentre studies with a larger sample size is needed to clarify our findings.
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.
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.