C1q nephropathy: a variant of focal segmental glomerulosclerosis

Kidney International
Glen S MarkowitzVivette D'Agati

Abstract

C1q nephropathy is a poorly understood and controversial entity with distinctive immunopathologic features. In order to better define the clinical-pathologic spectrum, we report the largest single-center series. Nineteen biopsies with C1q nephropathy were identified from among 8909 native kidney biopsies received from 1994 to 2002 (0.21%). Defining criteria included (1). dominant or co-dominant immunofluorescence staining for C1q, (2). mesangial electron dense deposits, and (3). no clinical or serologic evidence of systemic lupus erythematosus (SLE). The 19 patients were predominantly African American (73.7%), female (73.7%), young adults and children (range, 3 to 42 years; mean, 24.2 years). Presentation included nephrotic range proteinuria (78.9%), nephrotic syndrome (50%), renal insufficiency (27.8%), and hematuria (22.2%). No patient had hypocomplementemia or evidence of underlying autoimmune or infectious disease. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS) in 17 (including six collapsing and two cellular) and minimal-change disease (MCD) in two. All biopsies displayed co-deposits of immunoglobulin G (IgG), with more variable IgM (84.2%), IgA (31.6%), and C3 (52.6%). Foot process effacement varied from ...Continue Reading

References

Oct 1, 1991·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·S S IskandarW B Lorentz
Aug 1, 1985·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·J C Jennette, C G Hipp
Jan 1, 1984·Clinical Immunology and Immunopathology·S UwatokoK Suzuki
Jun 1, 1994·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·S M KorbetE J Lewis
Aug 1, 1993·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·M KuwanoK Aida
Sep 7, 2000·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·T SrivastavaU S Alon
Aug 13, 2002·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Mesiha EkimNecmiye Tumer
Apr 22, 2003·Seminars in Nephrology·Vivette D'Agati

❮ Previous
Next ❯

Citations

Apr 14, 2005·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Keith K LauRobert J Wyatt
Sep 1, 2005·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Masashi NishidaKenji Hamaoka
Oct 26, 2005·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Tanja Kersnik LevartAlenka Vizjak
Mar 8, 2006·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Isabel RobertiDae Un Kim
Sep 7, 2006·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Coral HanevoldRory Dalton
Oct 24, 2007·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Jameela A Kari, Sawsan M Jalalah
Mar 21, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Martin BitzanChantal Bernard
Aug 9, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Isabel RobertiDae Un Kim
Dec 17, 2008·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Craig S WongJohn R Brandt
Aug 13, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Nathaniel MacHardyDebbie S Gipson
Aug 12, 2009·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Luke TaggartFranca Iorember
Jul 16, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Mateja Vintar SpreitzerTanja Kersnik Levart
May 18, 2010·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Samar M SaidSamih H Nasr
Mar 9, 2004·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Andrew SharmanJohn Feehally
Nov 12, 2005·Nephron. Clinical Practice·Juhani MyllymäkiJukka Mustonen
Jul 25, 2008·Journal of the American Society of Nephrology : JASN·Alenka VizjakJ Charles Jennette
Nov 28, 2013·Nephro-urology Monthly·Pavan Malleshappa, Mahesha Vankalakunti
Dec 12, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Vindya N GunasekaraKjell Tullus
Oct 23, 2012·Indian Journal of Nephrology·S N MandalG Swarnalata
Apr 18, 2009·Clinical and Experimental Nephrology·Akiko MiiYuh Fukuda
Nov 26, 2010·Clinical and Experimental Nephrology·Aditi SinhaStanley C Jordan
Jul 24, 2012·Clinical and Experimental Nephrology·Takashi TakeiKosaku Nitta
Jul 25, 2008·Clinical Journal of the American Society of Nephrology : CJASN·Satoshi HisanoHiroshi Iwasaki
Oct 6, 2006·Kidney International·M B StokesV D D'Agati
Sep 8, 2011·Advances in Chronic Kidney Disease·Jeremy KiffelHoward Trachtman
Feb 2, 2010·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Amber M Reeves-DanielBarry I Freedman
May 2, 2006·Human Pathology·Samih H NasrVivette D D'Agati
Apr 12, 2016·Frontiers in Pediatrics·Sarangarajan Ranganathan
Mar 12, 2015·Saudi Journal of Kidney Diseases and Transplantation : an Official Publication of the Saudi Center for Organ Transplantation, Saudi Arabia·K V KanodiaH L Trivedi
Jun 9, 2016·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Barbara RuggieroFrancesco Emma
Dec 8, 2015·Analytical Cellular Pathology (Amsterdam)·Joe DevasahayamUnni Pillai
Nov 1, 2007·Expert Review of Clinical Immunology·Mohammed Yousuf KarimDavid P D'Cruz
Feb 21, 2018·Arkhiv patologii·V A VarshavskyE I Gudkova
Jan 18, 2019·The Tohoku Journal of Experimental Medicine·Yasushi Chiba, Chiyoko N Inoue
Aug 18, 2006·Journal of the American Society of Nephrology : JASN·Mamdouh AlbaqumiPeter J Nelson
Aug 19, 2007·Clinical Journal of the American Society of Nephrology : CJASN·Laura BarisoniJeffrey B Kopp
Nov 6, 2007·Journal of Korean Medical Science·In Seok LimHae Il Cheong
Dec 1, 2011·NDT Plus·Consolación Rosado RubioJosé Luis Lerma Márquez
Feb 28, 2020·BMC Nephrology·Adela ArapovićMarijan Saraga
Aug 1, 2009·NDT Plus·Mordi MuorahPatrick J O'Donnell
Apr 6, 2006·Archives of Pathology & Laboratory Medicine·Clarissa S Krinsky, Alexis Harris
Feb 7, 2009·Archives of Pathology & Laboratory Medicine·David B Thomas

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.