Ying, Tracey, Hill, Prue, Desmond, Michael, Agar, John, and Mallett, Andrew (2015) Fibrillary glomerulonephritis: An apparent familial form? Nephrology, 20 (7). pp. 506-509.

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Abstract

Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non-amyloid fibrillary deposits of unknown aetiology. It is generally considered idiopathic but may be associated with secondary causes such as monoclonal gammopathy, hepatitis B and C infections, autoimmune diseases and malignancies. We report two Australian families with apparent familial fibrillary glomerulonephritis inherited in an autosomal dominant pattern, and postulate the existence of a primary familial entity. Family 1 consists of an affected father and daughter; the daughter progressed to end-stage renal failure within 18 months of diagnosis, despite immunosuppressive therapy. The father, however, remains stable at 10 months follow up. Family 2 comprises an affected mother and son; the mother commenced haemodialysis 5 years after diagnosis and subsequently underwent successful renal transplantation. The son is presently stable at last follow-up after 5 years. A further review of the second family history reveals a third family member (maternal father) dying of 'Bright's disease'. We describe their histopathology, clinical progression and treatment outcomes, and provide a review of the current understanding of this heterogeneous condition that is associated with poor renal outcomes.

Item ID:	67890
Item Type:	Article (Case Study)
ISSN:	1440-1797
Copyright Information:	© 2015 Asian Pacific Society of Nephrology
Date Deposited:	03 Jan 2024 01:23
FoR Codes:	32 BIOMEDICAL AND CLINICAL SCIENCES > 3202 Clinical sciences > 320214 Nephrology and urology @ 100%
SEO Codes:	20 HEALTH > 2001 Clinical health > 200105 Treatment of human diseases and conditions @ 100%
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