Risk and clinical characteristics of spinal cord compression across different mucopolysaccharidosis types: A retrospective cohort study.

In patients with mucopolysaccharidosis (MPS), the accumulation of glycosaminoglycans leads to various complications, including spinal cord compression (SCC). Although SCC is a well-known complication in MPS, data comparing its clinical features across different MPS types remain limited. This study aimed to investigate the timing, location, and underlying causes of SCC in MPS, as well as to compare the risk and clinical characteristics by MPS type.

Update on Pediatric Anti-obesity Medications-Current Landscape and Approach to Prescribing.

Purpose Of Review: To review the current medical therapies available for treatment of obesity in children and adolescents less than 18 years old in the United States and outline the approach to their use.

Recent Findings: Obesity is a chronic disease with increasing prevalence in children and adolescents in the United States. Over the past few years, more FDA-approved medical treatments for obesity, such as GLP-1 receptor agonists, have emerged for patients less than 18 years old.

Long-term outcomes of liver transplantation for biliary atresia and results of policy changes: over 20 years of follow-up experience.

Objective: Biliary atresia (BA) patients develop chronic liver disease after the Kasai operation and are eventually indicated for liver transplantation (LT). The purposes of this study were to analyze long-term outcomes after LT and risk factors that affect complications to reduce graft failure.

Study Design: Overall, 145 pediatric patients who underwent LT between June 1996 and June 2020 after a diagnosis of BA were included.

Pharmacotherapy causing weight gain and metabolic alteration in those with obesity and obesity-related conditions: A review.

This review aims to summarize pharmacological interventions that may affect adiposity and metabolic equilibrium in individuals with obesity. Pharmacological therapy is frequently used to treat medical conditions that are both directly related to obesity (such as hypertension and type 2 diabetes) and indirectly related to obesity (such as asthma, insomnia, and type 1 diabetes). This pharmacological therapy may result in weight gain and alterations in the metabolic profile.

Functional polymorphism in the carboxyl terminus of the alpha-subunit of the human epithelial sodium channel.

A common human epithelial sodium channel (ENaC) polymorphism, alphaT663A, is present in the cytoplasmic C terminus of the alpha-subunit, although it is unclear whether this polymorphism segregates with blood pressure. We examined whether this polymorphism was associated with differences in functional Na(+) channel expression. Whole cell amiloride-sensitive currents in Xenopus oocytes expressing wild type channels (alphaT663betagamma) were significantly approximately 1.

Multiple epithelial Na+ channel domains participate in subunit assembly.

Epithelial sodium channels (ENaCs) are composed of three structurally related subunits that form a tetrameric channel. The Xenopus laevis oocyte expression system was used to identify regions within the ENaC alpha-subunit that confer a dominant negative phenotype on functional expression of alphabetagamma-ENaC to define domains that have a role in subunit-subunit interactions. Coexpression of full-length mouse alphabetagamma-ENaC with either 1) the alpha-subunit first membrane-spanning domain and short downstream hydrophobic domain (alpha-M1H1); 2) alpha-M1H1 and its downstream hydrophilic extracellular loop (alpha-M1H1-ECL); 3) the membrane-spanning domain of a control type 2 transmembrane protein (glutamyl transpeptidase; gamma-GT) fused to the alpha-ECL (gamma-GT-alpha-ECL); 4) the extracellular domain of a control type 1 transmembrane protein (Tac) fused to the alpha-subunit second membrane-spanning domain and short upstream hydrophobic domain (Tac-alpha-H2M2); or 5) the alpha-subunit cytoplasmic COOH terminus (alpha-Ct) significantly reduced amiloride-sensitive Na+ currents in X.