Characteristic electron-microscopic features of cryofibrinogen-associated glomerulonephritis: a case report
| Autor: | Reiji Haba, Aiko Shiraishi, Yoshio Kushida, Dedong Kang, Tetsuo Minamino, Kyuichi Kadota, Kensuke Joh, Kazuho Honda, Emi Ibuki, Tadashi Sofue |
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| Rok vydání: | 2019 |
| Předmět: |
Nephrology
Male medicine.medical_specialty Pathology Cryofibrinogen-associated glomerulonephritis Cryofibrinogenemia Renal function Case Report lcsh:RC870-923 Glomerulonephritis Tandem Mass Spectrometry Internal medicine Membranoproliferative glomerulonephritis Biopsy medicine Humans Cryoglobulins Aged Organized deposit Kidney medicine.diagnostic_test business.industry Fibrinogens Abnormal lcsh:Diseases of the genitourinary system. Urology medicine.disease Microscopy Electron medicine.anatomical_structure Renal pathology Cryoglobulinemia Cryofibrinogen Microtubular structure business Chromatography Liquid |
| Zdroj: | BMC Nephrology BMC Nephrology, Vol 21, Iss 1, Pp 1-8 (2020) |
| ISSN: | 1471-2369 |
| Popis: | Background Cryofibrinogenemia is a rare disorder that mainly affects the skin and occasionally the kidney. However, there are few published reports of cryofibrinogenemia-associated renal pathology. We therefore report a patient with cryofibrinogen-associated glomerulonephritis. Samples from this patient were examined by electron microscopy, laser microdissection, and liquid chromatography-tandem mass spectrometry (LC-MS/MS). Case presentation A 78-year-old Japanese man presented with declining renal function, proteinuria, and gross hematuria. Kidney biopsy showed a membranoproliferative pattern with crescent formation and dominant C3c deposition in which subendothelial deposits with uniquely organized electron-microscopic features were observed. Additional ultrastructural analysis of cryoprecipitates extracted from plasma revealed similar structures of the glomerular subendothelial deposits. LC-MS/MS identified an increase in fibrinogen α, β, and γ chains, fibronectin, filamin-A, and C3. The glomerular lesions were diagnosed as cryofibrinogen-associated glomerulonephritis on the basis of these findings. Conclusions Although there are few reports of cryofibrinogen-associated glomerulonephritis, we believe that accurate diagnosis can be achieved by performing LC-MS/MS and ultrastructural analysis. |
| Databáze: | OpenAIRE |
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