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[系膜IgA沉积性肾病]

[Mesangial IgA deposits nephropathy].

作者信息

Kessler M, Frimat L, Hestin D, Renoult E, Grignon Y, Grignon G

机构信息

Service de néphrologie, CHU de Nancy, hôpitaux de Brabois, Vandoeuvre, France.

出版信息

Rev Med Interne. 1994;15(7):471-8. doi: 10.1016/s0248-8663(05)81471-7.

DOI:10.1016/s0248-8663(05)81471-7
PMID:7938958
Abstract

Idiopathic IgA nephropathy of Berger's disease is characterized by prominent and diffuse IgA deposits in the mesangium. In many countries, it is the most common type of primary chronic glomerulonephritis. Typically, it is revealed by recurrent episodes of gross hematuria in association with ENT infection, but it can progress insidiously with microscopic hematuria and proteinuria. Serum IgA levels are increased in about 50% of cases. IgA nephropathy is not a minor condition: 20% of patients develop end-stage chronic renal failure 10 years after diagnosis and 50% after 20 years. IgA nephropathy can recur in a transplanted kidney suggesting that this disease is a systemic disorder although it has a remarkable tropism for the kidney. Even though many points remain to be elucidated, its pathogenesis appears to be linked to a genetic factor responsible for a lymphocyte dysfunction and an acquired environmental factor such as penetration of an antigen via the mucosa which may give rise to an excessive and inappropriate IgA immune response with the deposition of IgA in the mesangium and the development of progressive renal alterations. No treatment has been shown to be effective but tonsillectomy advised in case of a recurrent tonsillar focal infection is most often accompanied by a decrease in the incidence of gross hematuria. Corticosteroid therapy can be of benefit in cases involving a nephrotic syndrome associated with minimal glomerular lesions. In all cases, control of possible hypertension is of value in slowing the progression of this disease.

摘要

伯杰氏病(又称特发性IgA肾病)的特征是系膜区有显著且弥漫性的IgA沉积。在许多国家,它是原发性慢性肾小球肾炎最常见的类型。通常,它表现为与耳鼻喉感染相关的反复肉眼血尿发作,但也可能隐匿进展,出现镜下血尿和蛋白尿。约50%的病例血清IgA水平升高。IgA肾病并非轻症:20%的患者在诊断后10年发展为终末期慢性肾衰竭,20年后这一比例为50%。IgA肾病可在移植肾中复发,这表明该疾病是一种全身性疾病,尽管它对肾脏有明显的趋向性。尽管仍有许多问题有待阐明,但其发病机制似乎与一个导致淋巴细胞功能障碍的遗传因素以及一个后天环境因素有关,比如抗原通过黏膜侵入,这可能引发过度且不适当的IgA免疫反应,导致IgA在系膜区沉积以及进行性肾脏病变的发展。尚无治疗方法被证明有效,但对于反复扁桃体局部感染的病例,建议行扁桃体切除术,这通常会伴随肉眼血尿发生率的降低。皮质类固醇疗法对涉及微小肾小球病变的肾病综合征病例可能有益。在所有病例中,控制可能存在的高血压对于减缓该疾病的进展具有重要意义。

相似文献

1
[Mesangial IgA deposits nephropathy].[系膜IgA沉积性肾病]
Rev Med Interne. 1994;15(7):471-8. doi: 10.1016/s0248-8663(05)81471-7.
2
[IgA nephropathy (Berger's disease) in children].[儿童IgA肾病(伯杰氏病)]
Bol Med Hosp Infant Mex. 1992 Dec;49(12):832-8.
3
Active and chronic phases of Berger's disease (IgA nephropathy).伯杰氏病(IgA肾病)的活动期和慢性期。
Am J Kidney Dis. 1984 Mar;3(5):349-56. doi: 10.1016/s0272-6386(84)80082-7.
4
Predictors of prognosis in IgA nephropathy.IgA 肾病的预后预测因素。
Kaohsiung J Med Sci. 2012 Oct;28(10):517-20. doi: 10.1016/j.kjms.2012.04.012. Epub 2012 Sep 25.
5
[IgA nephropathy].[IgA肾病]
Srp Arh Celok Lek. 2004 Jan-Feb;132(1-2):41-3. doi: 10.2298/sarh0402041b.
6
[Primary nephropathy due to mesangial deposits of IgA (Berger's disease)].IgA系膜沉积所致原发性肾病(伯杰氏病)
Rev Med Chil. 1990 Feb;118(2):125-33.
7
Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits complicated by immunoglobulin A nephropathy in the renal allograft.移植肾中伴单克隆免疫球蛋白G沉积的增殖性肾小球肾炎合并免疫球蛋白A肾病
Nephrology (Carlton). 2016 Jul;21 Suppl 1:48-52. doi: 10.1111/nep.12775.
8
[Demonstration of mesangial IgA deposits in kidney biopsies of pediatric patients: comparison with the clinical picture].[小儿患者肾活检中系膜IgA沉积的显示:与临床表现的比较]
Praxis (Bern 1994). 1995 Oct 10;84(41):1158-64.
9
Clinical features and natural history of IgA nephropathy.IgA肾病的临床特征与自然病程。
Ann Med Interne (Paris). 1999 Feb;150(2):117-26.
10
Long-standing spontaneous clinical remission and glomerular improvement in primary IgA nephropathy (Berger's disease).
Am J Nephrol. 1987;7(6):440-4. doi: 10.1159/000167516.

引用本文的文献

1
Indications for tonsillectomy stratified by the level of evidence.根据证据水平分层的扁桃体切除术适应症。
GMS Curr Top Otorhinolaryngol Head Neck Surg. 2016 Dec 15;15:Doc09. doi: 10.3205/cto000136. eCollection 2016.