Researchers reported that a good correlation between donor specific antibody, histology, and C4d staining results validates the Banff criteria for acute antibody-mediated rejection in patients who have undergone liver transplantation, as published in Human Pathology. The Banff Working Group published guidance in 2016 with the goal of standardizing the diagnostic criteria for antibody-mediated rejection in…
Maralixibat treatment leads to height and weight catch-up in patients with Alagille syndrome (ALGS), according to a new study presented at the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) 54th annual meeting. “Patients with the greatest height disadvantage at baseline had the greatest catch-up in height,” the authors of the study said. Maralixibat…
Shorter leukocyte telomere length in pediatric patients who have undergone liver transplantation for conditions such as Alagille syndrome (ALGS) tends to be associated with higher intima-media thickness and aspartate aminotransferase, according to an article published in Liver Transplantation. These results suggest that early biological aging may contribute to subclinical cardiovascular damage and could affect the…
Multidisciplinary cooperation is a key part of treating Alagille syndrome, which can affect various systems in the body.
A group of experts recently gathered to produce a consensus on the appropriate management of complications related to liver cirrhosis (LC) in children; the resulting guideline was published in the Journal of Mexican Gastroenterology. The causes of LC in childhood are varied, ranging from genetic disorders such as Alagille syndrome to viral infections like hepatitis…
IL-31 serum levels correlate with pruritus and may be a useful biomarker for studies investigating therapies in patients with cholestatic diseases such as Alagille syndrome (ALGS), according to a new study published in Hepatology. Levels of IL-31 were found to be elevated in patients with other cholestatic diseases including primary sclerosing cholangitis (PSC) and primary…
I’m immensely grateful for the opportunity to receive medication for my rare disease, spinal muscular atrophy (SMA).
Maralixibat treatment leads to marked improvements in pruritus and quality of life in patients with Alagille syndrome (ALGS), according to a study published in Hepatology Communications. The finding is based on results from 2 randomized placebo-controlled clinical trials investigating the effect of maralixibat in 57 children with severe cholestasis secondary to ALGS. Maralixibat, marketed under…
Researchers discovered that therapies targeting the signaling of bile acids represent a promising and effective strategy to treat complex liver disorders, as published in Biomedicine & Pharmacotherapy. The incidence of liver disease has risen sharply over the last few years. Liver disease encompasses a wide range of diagnoses, from nonalcoholic fatty liver disease to hepatitis…
Multiple xanthomas are rare in children and should be investigated because they are often a sign of a systemic disease such as Alagille syndrome (ALGS), as highlighted by a case report presented in the Journal of Skin and Sexually Transmitted Diseases. The patient reported in the study was an 8-year-old boy who presented with multiple…
