Alsaad Khaled O, Edrees Burhan, Rahim Khawla A, Alanazi Abdulkareem, Ahmad Muawia, Aloudah Noura
Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City and College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
Section of Nephrology, Department of Pediatric, King Fahad Medical City, Riyadh, Saudi Arabia.
Saudi J Kidney Dis Transpl. 2017 Jul-Aug;28(4):898-905.
Collagenofìbrotic (collagen type III) glomerulopathy (CG) is a rare nonimmune-mediated 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 sub-endothelial 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.
胶原纤维性（III型胶原）肾小球病（CG）是一种罕见的非免疫介导性肾小球疾病。其特征是有组织的III型胶原纤维大量沉积，位于肾小球系膜和内皮下区域，并伴有血清III型前胶原肽水平升高。与全身性疾病和恶性肿瘤的关联极为罕见。在此，我们报告一例9岁女童，她是1型糖尿病患者，出现发热、肾病范围蛋白尿、全身性水肿和高血压。临床检查未发现指甲异常或骨骼异常。肾活检显示系膜扩张，肾小球毛细血管因苍白、无定形物质而显著狭窄和闭塞。免疫组织化学研究显示免疫球蛋白G（IgG）和白蛋白在肾小球毛细血管和肾小管基底膜呈弥漫性线性染色。超微结构检查发现系膜和内皮下有大量致密、磨损、曲线状带状纤维沉积，具有III型胶原特征。该患者被诊断为合并CG和糖尿病肾病（DN）。这是肾活检中CG与糖尿病改变相关联的首例报告。在本报告中，我们描述了该疾病的临床病理特征，回顾了儿童CG，并探讨了其与DN的关联。
