A novel homozygous mutation in the USP53 gene as the cause of benign recurrent intrahepatic cholestasis in children: a case report.

Background: Benign recurrent intrahepatic cholestasis (BRIC) is a rare cause of cholestasis with recurrent episodes of jaundice and pruritus without extrahepatic bile duct obstruction. A mutation in the USP53 gene is known to cause BRIC-like cholestasis with normal serum gamma-glutamyltransferase (GGT) levels.

Case: We report a 16-year-old boy with recurrent episodes of cholestasis since 6 months of age with normal serum GGT levels.

Celiac crisis with thrombocytopenia and coagulopathy in a child.

Background: Celiac disease rarely presents with edema, hypoalbuminemia, acute metabolic deterioration, and electrolyte imbalances. This life-threatening condition is defined as a celiac crisis and may mimic disorders with metabolic derangement and sepsis. The crisis may present at onset or develop in celiac disease patients with poor compliance to a gluten-free diet.

Metal-Free Click Modification of Triple Bond-Containing Polyester with Azide-Functionalized Vegetable Oil: Plasticization and Tunable Solvent Adsorption.

Pressure from environmental nongovernmental organizations and the public has accelerated research on the development of innovative and renewable polymers and additives. Recently, biobased "green" plasticizers that can be covalently attached to replace toxic and migratory phthalate-based plasticizers have gained a lot of attention from researchers. In this work, we prepared an azide-functionalized soybean oil derivative (AzSBO) and investigated whether it can be used as a plasticizer.

Evaluation of Hypersensitivity Reactions in Pediatric Patients Using Biological Drugs.

Introduction: Biological drugs are currently used for the treatment of chronic inflammatory, autoimmune, and neoplastic diseases. With their expanding indication spectrum and increasing use, hypersensitivity reactions to these drugs are also becoming more frequent. The present study aimed to report the incidence and the features of such reactions in pediatric patients using biologicals for the treatment of various diseases.