Neurofibromatosis type 1-associated hypertension secondary to coarctation of the thoracic aorta.

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen's disease, is an autosomal dominant genetic disorder. NF-I vasculopathy has been used to describe various vascular malformations associated with NF-1. Secondary hypertension related to NF-1 vasculopathy has been reported because of renal artery stenosis, coarctation of the abdominal aorta and other vascular lesions; however, coarctation of the thoracic aorta has seldom been reported.

Cytomegalovirus-induced collapsing focal segmental glomerulosclerosis.

Collapsing glomerulopathy is an aggressive morphologic variant of focal segmental glomerulosclerosis which typically presents with nephrotic syndrome and rapidly progressive renal failure. Most cases of collapsing glomerulopathy are associated with human immunodeficiency virus infection. We present a rare case of collapsing glomerulopathy associated with acute cytomegalovirus (CMV) infection in an immunocompetent host with improvement in renal function after the treatment of CMV with ganciclovir.

Development of focal segmental glomerulosclerosis after anabolic steroid abuse.

Anabolic steroid abuse adversely affects the endocrine system, blood lipids, and the liver, but renal injury has not been described. We identified an association of focal segmental glomerulosclerosis (FSGS) and proteinuria in a cohort of 10 bodybuilders (six white and four Hispanic; mean body mass index 34.7) after long-term abuse of anabolic steroids.

Mycophenolate mofetil for the treatment of interstitial nephritis.

Acute interstitial nephritis (AIN) is a clinicopathologic entity that is characterized by acute renal failure and renal biopsy findings of interstitial inflammation and tubulitis. There are multiple causes of AIN, the majority of which appear to respond to immunosuppressive therapy. Corticosteroids are the mainstay of treatment for AIN, but many patients are refractory to or intolerant of treatment or are unable to discontinue therapy without clinical relapse.

C1q nephropathy: a variant of focal segmental glomerulosclerosis.

Background: C1q nephropathy is a poorly understood and controversial entity with distinctive immunopathologic features. In order to better define the clinical-pathologic spectrum, we report the largest single-center series.

Methods: Nineteen biopsies with C1q nephropathy were identified from among 8909 native kidney biopsies received from 1994 to 2002 (0.

Collapsing glomerulopathy.

Collapsing glomerulopathy is a morphologic variant of focal segmental glomerulosclerosis (FSGS) characterized by segmental and global collapse of the glomerular capillaries, marked hypertrophy and hyperplasia of podocytes, and severe tubulointerstitial disease. The cause of this disorder is unknown, but nearly identical pathologic findings are present in idiopathic collapsing glomerulopathy and human immunodeficiency virus (HIV)-associated nephropathy, and collapsing glomerulopathy has been associated with parvovirus B19 infection and treatment with pamidronate. The pathogenesis of collapsing glomerulopathy involves visceral epithelial cell injury leading to cell cycle dysregulation and a proliferative phenotype.

NPHS2 mutations in late-onset focal segmental glomerulosclerosis: R229Q is a common disease-associated allele.

Mutations in NPHS2, encoding podocin, have been identified in childhood onset focal and segmental glomerulosclerosis (FSGS). The role of NPHS2 in adult disease is less well defined. We studied 30 families with FSGS and apparent autosomal recessive inheritance and 91 individuals with primary FSGS.