A case of Crow-Fukase syndrome associated with membranoproliferative glomerulonephritis.

  • TAKESHITA SATOSHI
    Division of Nephrology, Department of Internal Medicine, Mitsui Memorial Hospital
  • YAMAKADO MINORU
    Division of Nephrology, Department of Internal Medicine, Mitsui Memorial Hospital
  • NAGANO MASASHI
    Division of Nephrology, Department of Internal Medicine, Mitsui Memorial Hospital
  • UMEZU MICHIO
    Division of Nephrology, Department of Internal Medicine, Mitsui Memorial Hospital
  • TAGAWA HITOSHI
    Division of Nephrology, Department of Internal Medicine, Mitsui Memorial Hospital

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Other Title
  • 膜性増殖性糸球体腎炎を伴ったCrow‐Fukase症候群の1例

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Abstract

Crow-Fukase syndrome is a rare multiorgan disorder. Although renal disorders, such as proteinuria, and renal impairment, have been observed in half the cases of this syndrome, there have been few reports describing the renal lesions. We report here a case of this syndrome associated with menbranoproliferative glomerulonephritis. A 43-year-old woman was referred to our hospital because of hyperglycemia. She had also been suffering from hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy and endocrine dysfunction, including diabetes mellitus and amenorrhea. Serum electrophoresis showed M protein and immunoelectrophoresis revealed IgA (A). Bone marrow aspiration showed a slight increase in the number of plasma cells. Urine protein was 30 mg/dl, BUN was 17 mg/ dl and creatinine 0.8mg/dl. Light microscopic examinations showed enlargement of glome ruli with proliferation of mesangial cells and matrix, a lobular pattern of the glomeruli and thickening of the glomerular basement membrane and associated double contour. Electron microscopic examinations showed thickened capillary walls, associated mesangial interposi tion and subendothelial dense deposits. Moreover, fine granular deposits of IgM, C3, and fibrinogen along the basement membrane were observed on immunofluorescent studies.

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