Collagenofibrotic (collagen type III) glomerulopathy in association with diabetic nephropathy

Collagenofibrotic (collagen type III) glomerulopathy in association with diabetic nephropathy

Collagenofibrotic (collagen type III) glomerulopathy (CG) is a rare nonimmunemediated glomerular disease. It is characterized by massive deposition of organized collagen type III fibers, which is localized in the mesangial and subendothelial glomerular areas and associated with increased serum level...

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Bibliographic Details
Published in:Saudi journal of kidney diseases and transplantation Vol. 28; no. 4; pp. 898 - 905
Main Authors: al-Sad, Khalid O., Idris, Burhan, Rahim, Khawlah A., al-Anzi, Abd al-Karim, Ahmad, Muawia, al-Awdah, Nurah
Format: Journal Article
Language:English
Published: Riyadh, Saudi Arabia Saudi Center for Organ Transplantation 01-07-2017
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt. Ltd
Wolters Kluwer Medknow Publications
Subjects:
Amlodipine
Biopsy
Blood
Care and treatment
Collagen
Diabetes
Diabetic nephropathies
Diabetic nephropathy
Dosage and administration
Edema
Family medical history
Health aspects
Health sciences
Immunoglobulin G
Kidney biopsy
Kidney diseases
Laboratories
Medicine
Nephrology
Pathology
Patients
Pediatrics
Proteins
Ultrasonic imaging
Urine
اعتلال الكلى السكري
الأطفال
التهاب كبيبات الكلى
الكولاجين
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Summary:Collagenofibrotic (collagen type III) glomerulopathy (CG) is a rare nonimmunemediated glomerular disease. It is characterized by massive deposition of organized collagen type III fibers, which is localized in the mesangial and subendothelial glomerular areas and associated with increased serum levels of procollagen type III peptide. Association with systemic diseases and malignancies is extremely rare. Herein, we present a case of a nine-year-old girl, known case of type I diabetes mellitus, who presented with fever, nephrotic range proteinuria, generalized edema, and hypertension. Clinical examination did not show nail abnormalities or bone abnormalities. Renal biopsy revealed mesangial expansion and remarkable narrowing and obliteration of the glomerular capillaries by pale, amorphous material. Immunohistochemical study demonstrated diffuse linear glomerular capillary and tubular basement membrane staining for immunoglobulin G (IgG) and albumin. Ultrastructural examination identified massive mesangial and subendothelial deposition of dense frayed, curvilinear banded fibers with characteristic features of type III collagen. The patient was diagnosed to have combined CG and diabetic nephropathy (DN). This is the first report of CG in association with diabetic changes in renal biopsy. In this report, we describe the clinicopathological characteristics of this disease, review CG in pediatric population, and explore its association with DN.
Bibliography:ObjectType-Case Study-3
SourceType-Scholarly Journals-1
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ISSN:1319-2442
2320-3838