Journal article
Diagnosis of complement alternative pathway disorders
Kidney international, Vol.89(2), pp.278-288
02/2016
DOI: 10.1016/j.kint.2015.12.003
PMID: 26806831
Abstract
Kidney diseases resulting from abnormal control of the complement alternative pathway include atypical hemolytic uremic syndrome, C3 glomerulonephritis, and dense-deposit disease, as well as atypical postinfectious glomerulonephritis. Although clinically diverse, they all result from loss of surface or fluid-phase complement control, caused by acquired or genetic defects in the complement alternative pathway. As such, the diagnostic approach is similar and includes a comprehensive biochemical, genetic, and pathologic analysis of the complement pathway. The biochemical test battery includes functional activity measurements of the entire complement pathway, functional and quantitative analysis of individual components and regulators, and quantification of activation products. In patients with a thrombotic microangiopathy, ADAMTS-13 activity should be determined to exclude a thrombotic thrombocytopenic purpura. The spectrum of genes currently known to be involved in the pathogenesis of alternative pathway disorders is rapidly expanding. Pathologic analysis of a kidney biopsy specimen is sophisticated with ad hoc immunofluorescence studies and laser microdissection with mass spectrometry. The identification of the underlying defect in the alternative pathway based on this comprehensive analysis will allow treatment to be directed to the site of dysregulation.
Details
- Title: Subtitle
- Diagnosis of complement alternative pathway disorders
- Creators
- Andrea Angioi - Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota, USAFernando C Fervenza - Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota, USASanjeev Sethi - Division of Anatomic Pathology, Mayo Clinic College of Medicine, Rochester, Minnesota, USAYuzhou Zhang - Division of Nephrology, Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, Iowa City, Iowa, USARichard J Smith - Division of Nephrology, Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, Iowa City, Iowa, USADavid Murray - Clinical Biochemistry, Mayo Clinic College of Medicine, Rochester, Minnesota, USAJens Van Praet - Division of Nephrology, AZ Sint-Jan Brugge-Oostende AV, Brugge, BelgiumAntonello Pani - Division of Nephrology and Dialysis, Azienda Ospedaliera “G. Brotzu,” Cagliari, ItalyAn S De Vriese - Division of Nephrology, AZ Sint-Jan Brugge-Oostende AV, Brugge, Belgium
- Resource Type
- Journal article
- Publication Details
- Kidney international, Vol.89(2), pp.278-288
- DOI
- 10.1016/j.kint.2015.12.003
- PMID
- 26806831
- NLM abbreviation
- Kidney Int
- ISSN
- 0085-2538
- eISSN
- 1523-1755
- Publisher
- Elsevier Inc
- Language
- English
- Date published
- 02/2016
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984007292402771
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