Normal Gamma Glutamyl Transferase Levels at Presentation Predict Poor Outcome in Biliary Atresia.

OBJECTIVES: Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of "normal GGT" BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA. METHODS: Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were categorized into normal (<200I U/L) or high GGT groups based on a mean of 3 consecutive GGT values done before Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice (COJ), and outcomes were compared between the 2 groups. RESULTS: One hundred thirteen infants underwent KPE at median 61 (30-149) days. At a median follow-up of 14.2 (0.9-26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pretransplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to COJ were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18 months, P = 0.02), underwent LT at a younger age (14 vs 20 months, P = 0.04), and had poorer transplant-free survival (P = 0.04) than high GGT group. CONCLUSIONS: 12.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in BA was associated with a poorer outcome.

Vitamin D non-sufficiency is prevalent in children with chronic liver disease in a tropical country.

BACKGROUND: To determine vitamin D status in children with chronic liver disease (CLD) in a tropical country. METHODS: Cross-sectional study in Malaysian children with CLD. Factors affecting serum vitamin D level (definition: deficient < 30 nmol/L; insufficient 30-50 nmol/L; sufficient ≥ 50 nmol/L) was analyzed. RESULTS: Of the 59 children studied (males 32, 54%; median age 6.8 ± 5.3 years), the three most common causes were biliary atresia (n = 25), autoimmune hepatitis (n = 16) and sclerosing cholangitis (n = 6). The overall mean daily vitamin D intake was 715 ± 562 units/day. Thirteen (22%) patients had at least one clinical signs of rickets. Seventeen (29%) had serum bilirubin level ≥ 34 µmol/L. Eight (14%) children were deficient in vitamin D, eight (14%) were vitamin D-insufficient and 43 (73%) were sufficient. As compared with children with serum bilirubin <34 µmol/L, those with serum bilirubin ≥34 µmol/L were more likely to have rickets (24% vs. 65%; P < 0.002) and a lower serum vitamin D level (86.0 ± 54.9 nmol/L vs. 65.4 ± 48.2 nmol/L; P = 0.05) despite being given a significantly higher vitamin D dose (608 ± 571 vs. 970 ± 543 units/day; P = 0.008). The proportion of children with either deficient or insufficient vitamin D status was significantly higher in children with bilirubin level ≥34 µmol/L than in children <34 µmol/L (47% vs. 19%; P = 0.028). CONCLUSION: 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.

Quality indicators in pediatric colonoscopy in a low-volume center: Implications for training.

AIM: To study implications of measuring quality indicators on training and trainees' performance in pediatric colonoscopy in a low-volume training center. METHODS: We reviewed retrospectively the performance of pediatric colonoscopies in a training center in Malaysia over 5 years (January 2010-December 2015), benchmarked against five quality indicators: appropriateness of indications, bowel preparations, cecum and ileal examination rates, and complications. The European Society of Gastrointestinal Endoscopy guideline for pediatric endoscopy and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition training guidelines were used as benchmarks. RESULTS: Median (± SD) age of 121 children [males = 74 (61.2%)] who had 177 colonoscopies was 7.0 (± 4.6) years. On average, 30 colonoscopies were performed each year (range: 19-58). Except for investigations of abdominal pain (21/177, 17%), indications for colonoscopies were appropriate in the remaining 83%. Bowel preparation was good in 87%. One patient (0.6%) with severe Crohn's disease had bowel perforation. Cecum examination and ileal intubation rate was 95% and 68.1%. Ileal intubation rate was significantly higher in diagnosing or assessing inflammatory bowel disease (IBD) than non-IBD (72.9% vs 50.0% P = 0.016). Performance of four trainees was consistent throughout the study period. Average cecum and ileal examination rate among trainees were 97% and 77%. CONCLUSION: Benchmarking against established guidelines helps units with a low-volume of colonoscopies to identify area for further improvement.

Chronic liver disease is universal in children with biliary atresia living with native liver.

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.

Capillarity-assisted assembly of carbon nanotube microstructures with organized initiations.

In this work, detailed studies of three different capillary-assisted techniques for the formations of large-scale multiwalled carbon-nanotube (MWNT)-based microstructures were presented. Using laser induced artificial vacancies, new insights into the effect of laser power, densities of MWNTs, and oxidation process dependencies for the creations of MWNT polygons were presented. With organized initiations, MWNT pillars were crafted out of MWNT arrays and 0.21 pL of water was found to produce sufficient force to bring about 14.7 mum deflections of a 9.19 x 9.19 x 24.1 mum(3) pillar, thereby allowing well-controlled formations of three-dimensional top-gathering MWNTs. Lastly, by twisting densified MWNT microbelts, 14 times improvements in resistivity as compared to undensified MWNT microwalls were achieved. Through prepatterning, the amount of twisting effect could be controlled, and this in turn allowed control of the resistance of the densified MWNT microwalls. These new insights and techniques presented could further encourage the use of self-organized MWNT structures with initiation as a flexible and viable route for the implementations of carbon-nanotube-based electronic devices.