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Abstract: TH-PO717

Cryofibrinogen-Associated Membranoproliferative Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Kim, Minah, Chonnam National University Hwasun Hospital, Hwasun-gun, Jeollanam-do, Korea (the Republic of)
  • Kim, Chang Seong, Chonnam National University Medical School, Gwangju, Korea (the Republic of)
  • Choi, Hong Sang, Chonnam National University Medical School, Gwangju, Korea (the Republic of)
  • Bae, Eun Hui, Chonnam National University Medical School, Gwangju, Korea (the Republic of)
  • Ma, Seong Kwon, Chonnam National University Medical School, Gwangju, Korea (the Republic of)
  • Kim, Soo Wan, Chonnam National University Medical School, Gwangju, Korea (the Republic of)
  • Suh, Sang Heon, Chonnam National University Medical School, Gwangju, Korea (the Republic of)
Introduction

Cryofibrinogenemia is a rare disorder caused by deposition of cryofibrinogen. Various clinical manifestations associated with cryofibrinogenemia. Although the kidney can be a target organ, but there are few reports. We describe a case of cryofibrinogen-associated glomerulonephritis in old man with prolonged diabetes.

Case Description

A 74-year-old man with diabetes mellitus visited the emergency department with generalized edema and weakness since 1 week. Serum creatinine level was 3.9 mg/dL (up from a baseline 6 mo earlier 1.2 mg/dl), and urinalysis revealed microscopic hematuria and nephrotic-range proteinuria (5.15 g/day in 24-h urine sample). Kidney biopsy, performed to ascertain the exact cause of acute kidney injury with nephrotic-range proteinuria, revealed basement membrane thickening, mesangial matrix prominence, marked inflammation, and endocapillary hypercellularity under light microscopy. Immunofluorescence microscopy showed no staining for immunoglobulin G. Electron microscopy revealed subendothelial and mesangial deposits with large-bore multilayered tubular structures (Figure 1a) that were further evaluated and comprised an abnormal protein that precipitated when the plasma, but not serum, was stored at 4'C and redissolved at 37'C(Figure 1b). Liquid chromatography-tandem mass spectrometry revealed fibrinogen-α, -β, and -γ chains, and fibronectin. Despite adequate hydration, the patient’s renal function did not improve, and prednisolone (1 mg/kg/day) therapy was initiated and continued for 3 months, whereby the serum creatinine decreased to 1.9 mg/dL. However, the renal function of the patient decreased gradually thereafter to eventual kidney failure that necessitated hemodialysis.

Discussion

Cryofibrinogenemia can induce several organ dysfunctions, manifesting as mostly dermatological symptoms, as well as kidney involvement. Distinctive ultrastructure findings of large microtubular structures on electron microscopy, plasma cryoprecipitates when cold, and proteomic analysis are crucial for diagnosing cryofibrinogen-associated glomerulonephritis.

a. Electron microscopy, b. Cryoprecipitates