Hyperadiponectinemia During Infliximab Induction Therapy in Pediatric Crohn Disease.

Objectives: The inflammatory process in Crohn disease (CD) involves the visceral fat, characterized by adipocyte hyperplasia and altered adipose tissue and serum concentrations of tumor necrosis factor (TNF), leptin, adiponectin and resistin. We investigated the effect of anti-TNF therapy with infliximab (IFX) on serum adipokine levels in pediatric CD.

Methods: Serum concentrations of resistin (ng/mL), leptin (ng/mL), and total adiponectin (μg/mL) were assessed by enzyme-linked immunosorbent assays (ELISA) in 18 pediatric CD patients (mean age 15.

Response to cyclosporine in steroid-resistant nephrotic syndrome: discontinuation is possible.

Background: Steroid-resistant nephrotic syndrome (SRNS) is still regarded as a serious disease although treatment with cyclosporine (CSA) has improved outcome. However, the duration of treatment in responders is unclear, and treatment of patients with genetic causes is a matter of debate.

Methods: Thirty-six patients with SRNS were studied retrospectively.

Comparison of three tests for faecal calprotectin in children and young adults: a retrospective monocentric study.

Objective: Faecal calprotectin is used as a sensitive marker for gastrointestinal mucosal inflammation. We compared the performance of three different assays in a large cohort of symptomatic paediatric patients.

Design: Retrospective monocentric study.

Serum 7-alpha-hydroxy-4-cholesten-3-one as a marker for bile acid loss in children.

Objective: To establish age-related reference values for 7-alpha-hydroxy-4-cholesten-3-one (C4) in a pediatric population and to investigate bile acid malabsorption in children with short bowel syndrome (SBS).

Study Design: Serum was obtained between 8:00 a.m.

Pathophysiological preconditions promoting mixed "black" pigment plus cholesterol gallstones in a DeltaF508 mouse model of cystic fibrosis.

Gallstones are frequent in patients with cystic fibrosis (CF). These stones are generally "black" pigment (i.e.

Pathophysiological basis of liver disease in cystic fibrosis employing a DeltaF508 mouse model.

The molecular pathogenesis of cystic fibrosis (CF) liver disease is unknown. This study investigates its earliest pathophysiological manifestations employing a mouse model carrying DeltaF508, the commonest human CF mutation. We hypothesized that, if increased bile salt spillage into the colon occurs as in the human disease, then this should lead to a hydrophobic bile salt profile and to "hyperbilirubinbilia" because of induced enterohepatic cycling of unconjugated bilirubin.

Endothelial effects of 3-hydroxyglutaric acid: implications for glutaric aciduria type I.

Infants with glutaric aciduria type 1 (GA1) are subject to intracranial vascular dysfunction. Here, we demonstrate that the disease-specific metabolite 3-hydroxyglutaric acid (3-OH-GA) inhibits basal and vascular endothelial growth factor (VEGF)-induced endothelial cell migration. 3-OH-GA affects the morphology of VEGF-induced endothelial tubes in vitro because of partial disintegration of endothelial cells.