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与肾病综合征相关的高免疫球蛋白E综合征。

Hyperimmunoglobulin E syndrome associated with nephrotic syndrome.

作者信息

Tanji C, Yorioka N, Kanahara K, Naito T, Oda H, Ishikawa K, Taguchi T

机构信息

Department of Internal Medicine, Kure National Hospital.

出版信息

Intern Med. 1999 Jun;38(6):491-4. doi: 10.2169/internalmedicine.38.491.

DOI:10.2169/internalmedicine.38.491
PMID:10411355
Abstract

A 21-year-old man was admitted to Kure National Hospital with nephrotic syndrome in September 1996. He had suffered from an intractable pruritic skin rash and recurrent subcutaneous abscesses caused by the hyperimmunoglobulin E syndrome since the age of 18 months. Renal biopsy gave a diagnosis of membranoproliferative glomerulonephritis. Steroid therapy decreased urinary protein loss and hypoproteinemia, and his pruritic skin rash was improved. Patients with hyperimmunoglobulin E syndrome have a defective immune response, especially to Staphylococcus aureus infection. Continuous antigen stimulation may have caused this patient's renal histological damage as in immune complex glomerulonephritis.

摘要

1996年9月,一名21岁男性因肾病综合征入住吴市国立医院。自18个月大以来,他一直患有由高免疫球蛋白E综合征引起的顽固性瘙痒性皮疹和复发性皮下脓肿。肾活检诊断为膜增生性肾小球肾炎。类固醇治疗减少了尿蛋白丢失和低蛋白血症,他的瘙痒性皮疹也有所改善。高免疫球蛋白E综合征患者存在免疫反应缺陷,尤其是对金黄色葡萄球菌感染的免疫反应。持续的抗原刺激可能像在免疫复合物性肾小球肾炎中一样,导致了该患者的肾脏组织学损伤。

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引用本文的文献

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Hyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and treatment modalities.高免疫球蛋白E综合征:遗传学、免疫发病机制、临床表现及治疗方式
J Res Med Sci. 2017 Apr 26;22:53. doi: 10.4103/jrms.JRMS_1050_16. eCollection 2017.
2
An update on the hyper-IgE syndromes.高免疫球蛋白E综合征的最新进展。
Arthritis Res Ther. 2012 Nov 30;14(6):228. doi: 10.1186/ar4069.
3
The hyperimmunoglobulin E syndrome--clinical manifestation diversity in primary immune deficiency.高免疫球蛋白 E 综合征——原发性免疫缺陷中的临床表现多样性。
Orphanet J Rare Dis. 2011 Nov 15;6:76. doi: 10.1186/1750-1172-6-76.
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Hyper-IgE syndrome and autoimmunity in Mexican children.墨西哥儿童的高免疫球蛋白E综合征与自身免疫
Pediatr Nephrol. 2006 Aug;21(8):1200-5. doi: 10.1007/s00467-006-0178-3. Epub 2006 Jun 22.