Use of T1 mapping in cardiac MRI for the follow-up of Fabry disease in a pediatric population.

Background: Fabry disease (FD) is a rare X-linked lysosomal disorder caused by pathogenic variants in the alpha-galactosidase-A gene (). Life threatening complications in adulthood include chronic kidney failure, strokes and the cardiac involvement which is the leading cause of mortality. Usually, it presents with hypertrophic cardiomyopathy, together with arrhythmia and conduction abnormalities.

Mycophenolic Acid Pharmacokinetics and Relapse in Children with Steroid-Dependent Idiopathic Nephrotic Syndrome.

Background And Objectives: Therapeutic drug monitoring of mycophenolic acid can improve clinical outcome in organ transplantation and lupus, but data are scarce in idiopathic nephrotic syndrome. The aim of our study was to investigate whether mycophenolic acid pharmacokinetics are associated with disease control in children receiving mycophenolate mofetil for the treatment of steroid-dependent nephrotic syndrome.

Design, Setting, Participants, & Measurements: This was a retrospective multicenter study including 95 children with steroid-dependent nephrotic syndrome treated with mycophenolate mofetil with or without steroids.

Long-term outcome of children treated with rituximab for idiopathic nephrotic syndrome.

Background: Rituximab (RTX) has recently showed promising results in the treatment of steroid-dependent idiopathic nephrotic syndrome (SDNS).

Methods: This was a retrospective multicenter study of 18 children treated with RTX for SDNS, with a mean follow-up of 3.2 years.

Favorable outcome in a case of Mycoplasma pneumoniae-associated crescentic glomerulonephritis.

Mycoplasma pneumoniae-associated nephritis has been reported in children with various pathological findings. It nevertheless remains an uncommon disease and, within this clinical context, endo-and extracapillary glomerulonephritis in a child has never been described. We report here a case of a 3-year-old girl diagnosed with severe crescentic glomerulonephritis associated with M.