Tubulointerstitial Renal Diseases of Children: Pathologic Features and Pathogenetic Mechanisms in Fanconi's Familial Nephronophthisis, Antitubular Basement Membrane Antibody Disease, and Medullary Cyst Disease

Abstract

The renal lesions of 15 children with tubulointerstitial diseases (TID), originally diagnosed as Fanconi's familial nephronophthisis (FFN), medullary cyst disease (MCD), or antitubular basement membrane (TBM) antibody disease were studied by light, electron, and immunoflauorescence microscopy and by peroxidase-anti-peroxidase stain for IgG. Kidneys originally considered in all three categories showed similar pathologic findings, with periglomerular fibrosis, interstitial fibrosis and infiltration, TBM thickening, and glomerulopathy. The data suggest that MCD and FFN are not synonyms, but that FFN can lead to medullary cystic disease, as can other tubulointerstitial diseases. Anti-TBM disease appears to be a more frequent cause of TID than is usually recognized. Glomerular involvement in TID may be due to deposition of anti-TBM antibody/TBM antigen complexes.

Key Words:

• tubulointerstitial nephritis
• antitubular basement membrane disease
• nephronophthisis
• medullary cystic disease
• Fanconi's familial nephronophthisis