Fatal polyarteritis nodosa with gastrointestinal involvement in a child.

Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years.

Three siblings with steroid-resistant nephrotic syndrome: new NPHS2 mutations in a Turkish family.

Steroid-resistant nephrotic syndromes often are resistant to additional immunosuppressive agents and tend to progress to end-stage renal disease. Genetic studies in children with familial nephrotic syndrome have identified mutations in genes that encode important podocyte proteins. NPHS2 mutations are responsible for autosomal recessive familial focal segmental glomerulosclerosis (FSGS), and these mutations were detected in both familial and sporadic forms of FSGS.