Medullary cystic kidney disease type 1 in a large Native-American kindred.

Background: Autosomal dominant medullary cystic kidney disease type 1 (MCKD1; Mendelian Inheritance in Man 174000) is a hereditary tubulointerstitial renal disease. For MCKD1, a locus on chromosome 1q21 is published. Although there are characteristic biopsy and imaging findings for MCKD, clinical diagnosis of this disorder is still very difficult because unique phenotypic features are not always present in individual cases.

Immunotactoid glomerulopathy in an HIV-positive African-American man.

The authors report the first case of immunotactoid glomerulopathy (ITG) in a human immunodeficiency virus (HIV)-positive, hepatitis B- and C-negative African-American man who presented with hematuria and proteinuria. His initial presentation was compatible with HIV associated nephropathy, but on renal biopsy he was found to have ITG. He has been treated with highly active antiretroviral therapy and an angiotensin-converting enzyme inhibitor, but his proteinuria has not responded after 4 months of treatment.