Biomaterial Database

Anti-glomerular and anti-tubular basement membrane nephritis in a renal allograft recipient with Alport's syndrome. 1994

J I Diaz, and R Valenzuela, and G Gephardt, and A Novick, and R R Tubbs

Department of Pathology, H. Lee Moffitt Cancer Center, University of South Florida Health Sciences Center, Tampa 33612-0179.

A 28-year-old deaf, white man with a clinical diagnosis of Alport's syndrome since 4 years of age experienced renal failure, fever, and mental status changes suggestive of vasculitis following his first cadaveric renal transplant; these symptoms and changes resolved after removal of the allograft. Immunohistological stains demonstrated intense linear deposition of IgG and C3 in glomerular and tubular basement membranes in the absence of glomerular crescents. One year later, a second renal transplant led to similar symptoms. A biopsy was performed 14 days after engraftment, which demonstrated intense linear deposition of IgG in glomerular and tubular basement membranes, but cellular crescents were not present. A serologic profile was ordered to evaluate the patient further for vasculitis, and during the evaluation, circulating anti-glomerular basement membrane and anti-tubular basement membrane antibodies were identified by indirect immunofluorescence microscopy (titer, > 1:320). An open biopsy specimen obtained during repair of a renal laceration demonstrated a crescentic glomerulonephritis with immunohistologic findings identical to those of previous biopsies. Anti-glomerular basement membrane nephritis should be suspected in any patient with Alport's syndrome in whom progressive renal failure develops following renal transplantation. Detection of anti-glomerular basement membrane/anti-tubular basement membrane antibodies will assure the diagnosis, and early initiation of plasmapheresis may be helpful to prevent further renal damage.

UI	MeSH Term	Description	Entries
D007678	Kidney Glomerulus	A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.	Glomerulus, Kidney
D007684	Kidney Tubules	Long convoluted tubules in the nephrons. They collect filtrate from blood passing through the KIDNEY GLOMERULUS and process this filtrate into URINE. Each renal tubule consists of a BOWMAN CAPSULE; PROXIMAL KIDNEY TUBULE; LOOP OF HENLE; DISTAL KIDNEY TUBULE; and KIDNEY COLLECTING DUCT leading to the central cavity of the kidney (KIDNEY PELVIS) that connects to the URETER.	Kidney Tubule,Tubule, Kidney,Tubules, Kidney
D008297	Male		Males
D008856	Microscopy, Fluorescence	Microscopy of specimens stained with fluorescent dye (usually fluorescein isothiocyanate) or of naturally fluorescent materials, which emit light when exposed to ultraviolet or blue light. Immunofluorescence microscopy utilizes antibodies that are labeled with fluorescent dye.	Fluorescence Microscopy,Immunofluorescence Microscopy,Microscopy, Immunofluorescence,Fluorescence Microscopies,Immunofluorescence Microscopies,Microscopies, Fluorescence,Microscopies, Immunofluorescence
D009393	Nephritis	Inflammation of any part of the KIDNEY.	Nephritides
D009394	Nephritis, Hereditary	A group of inherited conditions characterized initially by HEMATURIA and slowly progressing to RENAL INSUFFICIENCY. The most common form is the Alport syndrome (hereditary nephritis with HEARING LOSS) which is caused by mutations in genes for TYPE IV COLLAGEN and defective GLOMERULAR BASEMENT MEMBRANE.	Alport's Syndrome,Nephritis, Familial,Alport Syndrome,Alport Syndrome, Autosomal Dominant,Alport Syndrome, Autosomal Recessive,Alport Syndrome, X-Linked,Congenital Hereditary Hematuria,Hematuria-Nephropathy-Deafness Syndrome,Hematuric Hereditary Nephritis,Hemorrhagic Familial Nephritis,Hemorrhagic Hereditary Nephritis,Hereditary Familial Congenital Hemorrhagic Nephritis,Hereditary Hematuria Syndrome,Hereditary Interstitial Pyelonephritis,Hereditary Nephritis,Alport Syndrome, X Linked,Familial Nephritis,Hematuria Nephropathy Deafness Syndrome,Hematuria, Congenital Hereditary,Nephritis, Hematuric Hereditary,Nephritis, Hemorrhagic Familial,Nephritis, Hemorrhagic Hereditary,Pyelonephritis, Hereditary Interstitial,Syndrome, Alport,Syndrome, Hematuria-Nephropathy-Deafness,X-Linked Alport Syndrome
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000917	Antibody Formation	The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.	Antibody Production,Antibody Response,Antibody Responses,Formation, Antibody,Production, Antibody,Response, Antibody,Responses, Antibody
D001485	Basement Membrane	A darkly stained mat-like EXTRACELLULAR MATRIX (ECM) that separates cell layers, such as EPITHELIUM from ENDOTHELIUM or a layer of CONNECTIVE TISSUE. The ECM layer that supports an overlying EPITHELIUM or ENDOTHELIUM is called basal lamina. Basement membrane (BM) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. BM, composed mainly of TYPE IV COLLAGEN; glycoprotein LAMININ; and PROTEOGLYCAN, provides barriers as well as channels between interacting cell layers.	Basal Lamina,Basement Lamina,Lamina Densa,Lamina Lucida,Lamina Reticularis,Basement Membranes,Densas, Lamina,Lamina, Basal,Lamina, Basement,Lucida, Lamina,Membrane, Basement,Membranes, Basement,Reticularis, Lamina