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IgA肾病中的冷球蛋白血症和冷纤维蛋白原血症:一项随访研究。

Cryoglobulinaemia and cryofibrinogenaemia in IgA nephropathy: a follow-up study.

作者信息

Nagy J, Ambrus M, Paal M, Trinn C, Burger T

机构信息

Second Department of Medicine, University Medical School Pécs, Hungary.

出版信息

Nephron. 1987;46(4):337-42. doi: 10.1159/000184385.

DOI:10.1159/000184385
PMID:3116441
Abstract

Sera and plasmas from 50 patients with IgA nephropathy (IgA-NP) have been investigated for the presence of cryoglobulin (CG) and cryofibrinogen (CF), respectively, 2-5 cryoprotein determinations being made for each patient. CG was transiently found in 20 of 50 patients (40%), but in none of 20 healthy blood donors, whereas CF was found in 37 of 50 patients (74%) and in 4 of 20 healthy blood donors. The cryoprecipitates were of single and mixed component types. All but 2 of the patients with CF had haematuria. Nearly all of them had histories of long exposure to the cold as manual workers at the onset or recognition of their disease. There was no clinical remission during a 2-to-5-year follow-up if cryoproteinaemia persisted. A certain correlation was detected between the composition of the CP and the renal immunohistological findings. It is suggested that renal deposition of circulating CF or local formation of CF might be responsible for the tubulo-interstitial fibrocellular changes, which are of prognostic importance.

摘要

对50例IgA肾病(IgA - NP)患者的血清和血浆分别进行了冷球蛋白(CG)和冷纤维蛋白原(CF)检测,每位患者进行了2 - 5次冷蛋白测定。50例患者中有20例(40%)短暂检测到CG,但20例健康献血者中均未检测到;而50例患者中有37例(74%)检测到CF,20例健康献血者中有4例检测到。冷沉淀物有单一组分和混合组分类型。除2例患者外,所有CF患者均有血尿。几乎所有患者在疾病发作或确诊时都有长期从事体力劳动且长期接触寒冷的病史。如果冷蛋白血症持续存在,在2至5年的随访期间无临床缓解。检测到冷蛋白(CP)的组成与肾脏免疫组织学结果之间存在一定相关性。提示循环CF的肾脏沉积或CF的局部形成可能是导致具有预后重要性的肾小管间质纤维细胞改变的原因。

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引用本文的文献

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Cryofibrinogen-associated glomerulonephritis with paraproteinemia.伴有副蛋白血症的冷纤维蛋白原相关性肾小球肾炎。
Front Immunol. 2025 Jul 15;16:1576917. doi: 10.3389/fimmu.2025.1576917. eCollection 2025.
2
Type 2 cryoglobulinemia treated with plasmapheresis associated with IgA nephropathy.采用血浆置换治疗的2型冷球蛋白血症合并IgA肾病。
Indian J Pediatr. 2003 Dec;70(12):999-1000. doi: 10.1007/BF02723829.
3
Cryofibrinogenaemia: a study of 49 patients.冷纤维蛋白原血症:49例患者的研究。
Clin Exp Immunol. 2000 May;120(2):253-60. doi: 10.1046/j.1365-2249.2000.01210.x.
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Transient nephrotic syndrome after anaesthesia resulting from a familial cryofibrinogen precipitating at 35 degrees C.麻醉后因家族性冷沉淀纤维蛋白原在35摄氏度沉淀导致的短暂性肾病综合征
J Med Genet. 1989 Oct;26(10):631-6. doi: 10.1136/jmg.26.10.631.