[Show abstract][Hide abstract] ABSTRACT: Morbid obesity is strongly associated with non-alcoholic fatty liver disease (NAFLD), which is one of the most common causes of chronic liver disease worldwide. The current best treatment of NAFLD and NASH is weight reduction through lifestyle modification. Because of frustrating inefficiency of such a therapeutic approach bariatric surgery is increasingly seen in adolescents as an alternative option for weight reduction. Standards of care and consensus for indications however are scarce. We explore the indications and limitations of bariatric surgery in severely obese children with and without non-alcoholic steatohepatitis and aim to provide guidance for the exceptional indications for extremely obese adolescents with major comorbidity that may benefit from these controversial interventions. Current evidence suggests that bariatric surgery can decrease the grade of steatosis, hepatic inflammation and fibrosis in non-alcoholic steatohepatitis. Uncomplicated NAFLD is not an indication for bariatric surgery. Roux-en-Y gastric bypass is considered a safe and effective option for extremely obese adolescents, as long as appropriate long-term follow-up is provided. Laparoscopic adjustable gastric banding has not been approved by the FDA for use in adolescents and therefore should be considered investigational. Finally, sleeve gastrectomy and other types of weight loss surgery that have grown increasingly common in adults, still need to be considered investigational. Temporary devices may be increasingly being used in pediatrics, however future studies, including a long-term risk analysis of patients who undergo surgery, are much needed to clarify the exact indications for bariatric surgery in adolescents.
Full-text · Article · Jan 2015 · Journal of Pediatric Gastroenterology and Nutrition
[Show abstract][Hide abstract] ABSTRACT: Pediatric liver transplantation (LTx) has revolutionized life chances and perspectives of children with liver disease. Following rapid establishment of the therapeutic concept in the early years of pediatric transplant medicine, more aspects beyond plain survival become increasingly important. In addition to improving the short to medium-term survival rates, researchers are focusing on themes such as rehabilitation, adherence and quality of life, long-term graft fibrosis and dysfunction, as well as the consequences of long-term immunosuppression. Also, more protocol biopsy data are available to evaluate increasing graft fibrosis. To manage their conditions, patients will need access to highly experienced pediatric liver transplant centers where clinical research will examine modulators of renal disease, endocrine and cardiovascular comorbidity and the development of graft fibrosis and malignancies. Assessment and evaluation of health-related quality of life and factors which influence clinical tolerance, adherence and transition from child to adult care will also be investigated. The analysis of multi-national registry data and more than 40 years of experience with large patient cohorts will provide important clues to treatment and will thus get increasing attention. In the future, longitudinal assessment of the outcome for pediatric LTx patients should include more functional aspects than plain survival rates or laboratory parameters.
No preview · Article · Sep 2014 · Gastroentérologie Clinique et Biologique
[Show abstract][Hide abstract] ABSTRACT: The number of children with chronic hepatobiliary disease surviving into adulthood is more and more increasing, but no established model of transition does exist in this category of patients. Here, we summarize medical problems expected at the time of their transition, and any impacts on morbidity and mortality in adulthood. Information provided would turn useful to adult hepatologists and practitioners responsible for ensuring continuity of care for young adults affected by diseases they are not usually accustomed to.
[Show abstract][Hide abstract] ABSTRACT: The effect of adjuvant steroids in infants with biliary atresia (BA) is not clear and evidence of benefit is lacking.
During the period Jan. 2000 - Dec. 2011, 153 infants with isolated (CMV IgM-ve) BA underwent Kasai portoenterostomy (KPE) at < 70 days. They were divided into three groups: LOW-dose steroid (from a previous randomized trial; starting prednisolone 2mg/kg/day, n = 18), HIGH-dose steroid (starting prednisolone 5 mg/kg/day, n = 44) and NO steroid [n = 72 + 19 placebo (from randomized trial) = 91]. Outcome was assessed by early liver biochemistry, clearance of jaundice (<20 μmol/L) and actuarial native liver survival. Data are quoted as median (IQ range) and compared with non-parametric ANOVA, Chi or LogRank tests as appropriate. P ⩽0.05 was regarded as significant.
All three groups were comparable for age (ANOVA, P = 0.31) and a surrogate marker of liver fibrosis [Aspartate-Aminotransferase index (APRi), ANOVA, P = 0.67]. At 1 month post KPE, there was a significant reduction in bilirubin [58(25-91) vs 91(52-145)μmol/L, P=0.0015], AST [118(91-159) vs. 155(108-193)IU/L, P=0.0015] and APRi [0.49(0.28-0.89) vs. 0.82 (0.45-1.2), P=0.005] for HIGH vs. NO steroid. There was a significant increase in% clearance of jaundice with the use of steroids [47/91(52%) vs. 12/18 (67%) vs. 29/44(66%); steroids vs. no steroids, P = 0.037]. There was no statistical difference in 4 year patient survival (96% vs. 94% vs. 95%) or native liver survival (4 year = 46% vs.50 vs.57%).
The adjuvant use of prednisolone significantly improved early post-operative liver biochemistry (especially at the higher dose), and increased the proportion of infants who cleared their jaundice at six months post-KPE.
No preview · Article · Jun 2013 · Journal of Hepatology
[Show abstract][Hide abstract] ABSTRACT: This article aims to review the causes of jaundice, particularly conjugated jaundice in infants and the signs and symptoms of neonatal liver disease. The role of community practitioners in identifying prolonged jaundice in Infants will be discussed and a jaundice protocol and early identification algorithm will be introduced.
No preview · Article · Oct 2007 · Community practitioner: the journal of the Community Practitioners' & Health Visitors' Association
[Show abstract][Hide abstract] ABSTRACT: Kasai portoenterostomy (KP) is regarded as first-line treatment for biliary atresia, although its postoperative course is often unpredictable. Hepatobiliary scintigraphy using technetium-labeled iminodiacetic acid derivatives offers a dynamic, objective assessment both of parenchymal liver function and restored biliary excretion. The value of postoperative radionuclide scans was assessed prospectively in a large population of post-KP infants.
Radionuclide scans consisted of an intravenous dose of 20 MBq of 99mTc mebrofenin iminodiacetic acid and subsequent gamma camera imaging. Four scan variables were evaluated: the hepatic extraction fraction (HEF; ie, initial liver uptake divided by the peak vascular uptake), the half-life of tracer excretion (TEX), the shape of the excretion curve, and the presence of activity in the Roux loop at 4 hours postinjection. All infants had type 3 biliary atresia with a median age at KP of 59 days (24-120 days). To assess predictive value, outcome (clearance of jaundice and need for transplant) was assessed at 6 months (for 1-week scan) and 2 years (for 6-month scan).
Eighty-seven infants underwent a radionuclide scan at 1 week post-KP. The median HEF was 34% (10%-90%). No relationship could be identified between HEF (P = .2) or excretion curve shape (P = .9) and outcome (at 6 months), and there were too few examples of a measurable TEX to allow meaningful comparison. The only predictive element at this time point was Roux loop activity (positive predictive value, 79%; negative predictive value, 53%; for "good" isotope bowel activity). Forty-four infants completed a second scan at 6 months. Median HEF increased from a baseline of 37% (11%-90%) to 64% (8%-100%) (P < .0001), although there was no significant intercorrelation (P = .12). The most predictive variables (of outcome at 2 years) were curve shape (positive predictive value, = 95%, negative predictive value, 82%) and TEX, and the least predictive was now Roux loop activity.
Early (at 7 days) hepatic scintigraphy is not predictive of poor outcome in general, although Roux loop activity does indicate later success. Later hepatic scintigraphy (at 6 months) allows a detailed assessment of dynamic liver function with biliary excretion variables predictive of outcome in the medium term.
No preview · Article · Jun 2007 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: Extrahepatic biliary atresia (EHBA) is the most common indication for liver transplantation in childhood. Most children who do not undergo transplant are reported to have chronic liver disease and its complications. The aim of this single-center study was to identify children with normal laboratory indices and no clinical evidence of chronic liver disease 10 or more years after Kasai portoenterostomy (KP).
A retrospective analysis of the medical notes of all children surgically treated at the authors' center between 1979 and 1991 was undertaken. Criteria for inclusion were absence of surgical complications, unremarkable clinical examination, and normal bilirubin, aspartate aminotransferase, albumin, international normalized prothrombin ratio, and platelet count.
Of 244 children surgically treated during the observation period, the authors identified 28 (11%) adolescents (14 male) who fulfilled the entry criteria. Their median age was 13.4 years (range, 10.2-22.2 years). Twenty-six with type 3 EHBA had conventional KP, whereas 2 underwent modified operations. The corrective surgery was performed at a median age of 58 days (range, 20-99 days). Median time of complete clearance of jaundice from the date of KP was 75 days (range, 21-339 days). Twelve (43%) patients had history of cholangitis at a median age of 3.4 years. The liver histologic findings were suggestive of mild to moderate fibrosis in 54.2% and cirrhosis in 40.7% of the patients who underwent biopsy. No child had gastrointestinal bleeding during follow-up. Thirteen (46%) patients had an elective esophagogastroduodenoscopy, which was normal in all. Twenty-six (93%) patients were in mainstream education, whereas the remaining two (7%) attended special school because of reasons unrelated to liver disease.
A sizable proportion of children with EHBA avoid significant chronic liver disease and its complications 10 years or more after conventional surgical correction and have an excellent quality of life. Their good outcome is not hampered by isolated episodes of ascending cholangitis. Whether or not the residual histologic damage will become symptomatic during their lifetime remains to be established.
No preview · Article · Nov 2003 · Journal of Pediatric Gastroenterology and Nutrition