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Two brothers with collagenofibrotic glomerulopathy.
Takafumi Aoki1, Kazuyuki Hayashi2, Takatoshi Morinaga3
1Department of Nephrology, Anjo Kosei Hospital, 28 Higashihirokute, Anjo-cho, Anjo City, Aichi, 446-8602, Japan.
CEN Case Reports
|May 17, 2017
Summary
Collagenofibrotic glomerulopathy, a rare kidney disease, involves abnormal collagen accumulation. Measuring serum procollagen III peptide (P III P) may aid early diagnosis in siblings of affected individuals.
Area of Science:
- Nephrology
- Pathology
- Genetics
Background:
- Collagenofibrotic glomerulopathy is a rare glomerular disease.
- It is characterized by the accumulation of atypical type III collagen fibers in the kidney's mesangial matrix and subendothelial space.
- Elevated serum procollagen III peptide (P III P) levels are a key laboratory finding.
Purpose of the Study:
- To report a rare case of collagenofibrotic glomerulopathy in adult siblings.
- To highlight the diagnostic utility of serum P III P levels in familial cases.
Main Methods:
- Histological confirmation of collagenofibrotic glomerulopathy.
- Immunohistochemistry for type III collagen.
- Electron microscopy to visualize collagen fibers.
- Measurement of serum procollagen III peptide (P III P) levels.
Main Results:
- Two brothers diagnosed with collagenofibrotic glomerulopathy.
- Both patients exhibited proteinuria and hypertension (patient 1) or nephrotic-range proteinuria (patient 2).
- Histology, immunohistochemistry, and electron microscopy confirmed extensive type III collagen deposition; elevated P III P levels were observed in both.
Conclusions:
- This is the third reported case of adult siblings with histologically confirmed collagenofibrotic glomerulopathy.
- Measuring serum P III P levels in siblings of diagnosed patients may be beneficial for early detection.
Keywords:
Collagen type III glomerulopathyCollagenofibrotic glomerulopathyFamilial nephropathyProcollagen III peptideType III collagenMore Related Videos
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