Henoch-Schonlein purpura with immunoglobulin a nephropathy and abnormalities of immunoglobulin A in a Wiskott-Aldrich syndrome carrier
Henoch-Schonlein purpura with immunoglobulin a nephropathy and abnormalities of immunoglobulin A in a Wiskott-Aldrich syndrome carrier
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DOI：
10.1016/s0272-6386(97)90043-3
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发表时间：
1997-02-01
影响因子：
13.2
通讯作者：
Combe, C
中科院分区：
文献类型：
--
作者：
Lasseur, C;Allen, AC;Combe, C
免疫球蛋白A1(IgA1)糖基化异常在IgA肾病(IgAN)患者中已被描述，无论是原始的还是继发性的过敏性紫癜。Wiskott-Aldrich综合征是一种X连锁隐性遗传性疾病，与IgA异常有关，肾脏受累，系膜沉积与IgAN相同。我们报告一例女性Wiskott-Aldrich综合征携带者，表现为过敏性紫癜和IgA糖基化异常，如先前在ISAN患者中报告的那样。IgA的半乳糖化异常可能与患者作为Wiskott-Aldrich综合征携带者的状态有关，并可能参与IgAN的发病。(C)1997年，由国家肾脏基金会公司提供。
Abnormalities of immunoglobulin A1 (IgA1) glycosylation have been described in patients with IgA nephropathy (IgAN), whether primitive or secondary to Henoch-Schonlein purpura. The Wiskott-Aldrich syndrome, an X-linked recessive disorder, is associated with abnormalities of IgA, Renal involvement with mesangial IgA deposition identical to that found in IgAN has been reported during this affection, We report the case of a female carrier of the Wiskott-Aldrich syndrome presenting with Henoch-Schonlein purpura and abnormalities of IgA glycosylation, as previously reported in patients with ISAN. The galactosylation abnormalities of IgA could be linked to the patient's status as carrier of the Wiskott-Aldrich syndrome and could contribute to the pathogenesis of IgAN. (C) 1997 by the National Kidney Foundation, Inc.