IgA nephritis in HIV-positive patients: a new HIV-associated nephropathy?

Four HIV-positive patients were shown to have IgA-associated nephritis on biopsy, including one with anaphylactoid purpura. Three were homosexuals, while the fourth acquired the infection from his mother. All had hematuria, a variable degree of proteinuria and renal disease with a benign course. Serologic studies showed elevated levels of IgA as well as IgA immune complexes and rheumatoid factor. IgA antibodies to multiple HIV antigens were detected by Western blot. Pathologic studies showed tubuloreticular inclusions in endothelial cells and nuclear bodies in interstitial cells in all cases. HIV antigens were not detected in kidney biopsies by monoclonal antibodies nor was HIV viral genome demonstrated by in situ hybridization. The possibility that this represents a unique type of IgA-associated HIV nephropathy is discussed.

IgA nephritis in a child with human immunodeficiency virus: a unique form of human immunodeficiency virus-associated nephropathy?

A 9-year-old boy is presented who was antibody positive for human immunodeficiency virus (HIV) and who had recurrent episodes of gross hematuria. Renal biopsy revealed findings typical of IgA nephropathy but also showed electron-microscopic abnormalities seen with HIV-associated nephropathy. In addition, IgA antibodies to multiple HIV proteins were detected in serum by Western blot analysis, and circulating immune complexes of the IgA class were present. Although HIV-associated nephropathy and IgA nephropathy are thought to be distinct conditions, five adults with a similar combination of findings have been reported, and our patient adds to the evidence for a link between these two entities in some patients. We propose that the histological parallels between the conditions may merely represent the limited renal responses available to multiple types of injuries, and we support the attempts underway to probe renal tissue for the HIV genome.

Coats' disease in a renal transplant recipient.

We report the first case, to our knowledge, of Coats' disease, an idiopathic exudative retinopathy, in a renal transplant recipient. Due to certain similarities in clinical presentation and confounding factors present in patients who have received kidney allografts, our patient was originally misdiagnosed as having cytomegalovirus retinitis. We emphasise the significance of this diagnostic distinction and the association of this retinal syndrome with renal disease.

Senior-Loken syndrome (familial renal-retinal dystrophy) and Coats' disease.

Senior-Loken syndrome is a rare hereditary disease that combines a disorder resembling familial juvenile nephronophthisis with retinitis pigmentosa. Retinitis pigmentosa is even less frequently associated with exudative retinopathy. The patient, a 15-year-old boy, had hereditary renal-retinal dystrophy combined with an exudative vasculopathy of the Coats' type. The patient is on thrice-weekly hemodialysis after two kidney transplants failed. One eye became painful and blind and was eventually enucleated.

Marfan syndrome and medullary sponge kidney: case report and speculation on pathogenesis.

The case of a 16 year old boy with Marfan syndrome, recurrent nephrolithiasis, and bilateral Medullary Sponge Kidney (MSKD) is described. This association was not previously described.

Nitrogen mustard therapy in children with frequent-relapsing nephrotic syndrome and steroid toxicity.

Nitrogen mustard (HN-2) is an alkylating agent known to be effective in inducing and prolonging remissions of nephrotic syndrome in children. The fact that the drug is administered intravenously eliminates the problem of compliance, and the fact that the treatment needs to be given only for a few days may lower the incidence of side-effects observed with other cytotoxic agents. We administered HN-2 to 12 children with the steroid-sensitive frequent-relapsing form of nephrotic syndrome, who had evidence of steroid toxicity. Life table analysis reveals a 46% sustained remission rate after 27 mo of follow-up. Complications of HN-2 treatment included only mild thrombophlebitis (2 patients) and mild local paresthesia (1 patient).