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Pediatric hepatology: 6 reports on treating liver disease in children

Liver disease in children and adolescents takes on as many forms as in adults, including viral hepatitis, autoimmune diseases, fatty liver disease, and even the pediatric-specific biliary atresia that occurs in infants.

Healio Gastroenterology and Liver Disease presents the following recent reports on the prevalence of fatty liver and fibrosis in young adults, liver transplantation for infants, and developing treatment for pediatric autoimmune diseases.

Gene pattern predicts transplant-free survival for children with biliary atresia

Researchers identified a 14-gene expression pattern that predicted transplant-free survival for 2 years in pediatric patients with biliary atresia, according to a study published in Gastroenterology.

Zhenhua Luo, from the University of Cincinnati College of Medicine, and colleagues explained that, while there have been advances in understanding the key factors relevant to etiology and pathogenesis of biliary atresia, the only treatment is hepatoportoenterostomy. This treatment may restore bile drainage in some patients but does not stop fibrosis progression. Read more

1 in 5 young adults show signs of fatty liver; 1 in 40 have fibrosis

Young adults aged around 24 years showed a high prevalence of nonalcoholic fatty liver disease, with progression increasing with age even in this small birth cohort, according to new data presented during the International Liver Congress 2019.

“This is the only study to assess NAFLD prevalence in young adults using elastography. We found one in five had evidence of NAFLD; half had [steatosis grade 3 (S3)]. One in 40 in our cohort have evidence of fibrosis and this is in asymptomatic predominantly Caucasian participants with unselected general population birth cohort,” Kushala Abeysekera, MD, MBBS, BSc, from the University of Bristol, said during his presentation. “We’ve demonstrated that the prevalence of NAFLD in our birth cohort appears to have progressed from age 18 to 24 from 2.5% to 20.8%.” Read more

Infant liver transplant successful with novel video, surgical device solution

A combined procedure using the Orbeye video microscope and Thunderbeat advanced energy surgical device from Olympus led to a successful liver transplantation in an infant patient at Holtz Children’s Hospital in Florida, according to a press release.

“This past Sunday’s July 28 was National World Hepatitis Awareness Day, a good reminder to think about liver health,” Randy Clark, president of the medical systems group at Olympus Corporation of the Americas, said in the release. “Although no one wants to be on a transplant list, we are proud to be a company that can give new hope to those patients who could benefit from transplantation because of liver disease.” Read more

Maralixibat bile acid inhibitor enters phase 3 trial for pediatric PFIC

Mirum Pharmaceuticals dosed the first patient in a phase 3 trial of maralixibat, its lead drug candidate for the treatment of pediatric patients with progressive familial intrahepatic cholestasis, according to a press release.

“PFIC is a devastating condition that negatively affects children and their families,” Chris Peetz, president and CEO of Mirum, said in the release. “With no approved drug therapy, the start of the phase 3 MARCH-PFIC clinical trial is an important milestone for Mirum and puts us one step closer to potentially offering children a new treatment option that targets bile acid overload, a primary driver of liver damage and pruritus in this progressive disease.” Read more

Low sugar diet for pediatric NAFLD improves hepatic steatosis

An 8-week diet low in free sugar content significantly improved hepatic steatosis among adolescent boys with nonalcoholic fatty liver disease compared with their usual diet, according to a study published in JAMA.

“There are no approved pharmacological therapies for the treatment of NAFLD,” Jeffrey B. Schwimmer, MD, from the University of California in San Diego, and colleagues wrote. “Pediatric guidelines recommend ‘lifestyle modification to improve diet,’ but do not support one specific diet over another because of the limited available evidence. Among the various dietary options, limiting sugar intake is easily targetable in part because sugar is not a required nutrient.” Read more

Liver disease common in children with type 2 diabetes

An analysis of data from a small cohort of children with type 2 diabetes and nonalcoholic fatty liver disease, or NAFLD, suggests that nonalcoholic steatohepatitis, or NASH, is common among these patients, with 26% showing signs of advanced fibrosis, according to findings published in Pediatric Diabetes.

“Understanding the relationship between type 2 diabetes and NAFLD in children is particularly important, as type 2 diabetes has been strongly associated with liver-related mortality in adult patients with NAFLD,” Ron S. Newfield, MD, a pediatric endocrinologist with the University of California, San Diego, and Rady Children’s Hospital-San Diego, and colleagues wrote in the study background. “The goal of this study was to investigate the histologic characteristics of liver biopsies in a pediatric cohort of type 2 diabetes subjects, and whether exhibiting NASH or not correlates with their ethnicity, or their liver enzyme activity and metabolic phenotype, including their lipid profiles and diabetes control.” Read more