奥门氏网状细胞增多症伴肾病综合征
Omenn's reticulosis associated with the nephrotic syndrome.
作者信息
Rybojad M, Cambiaghi S, Moraillon I, Vignon-Pennmen M D, Morel P, Baudoin V, Loirat C
机构信息
Service de Dermatologie, Hôpital Saint-Louis, Paris, France.
出版信息
Br J Dermatol. 1996 Jul;135(1):124-7.
PMID:8776375
Abstract
Omenn's reticulosis is an inherited severe combined immunodeficiency characterized by neonatal exfoliative erythroderma. A newborn baby who had minimal change nephrotic syndrome and Omenn's reticulosis is reported. Abnormalities in lymphocyte function could explain both the nephropathy and the cutaneous changes.
摘要
奥门氏网状细胞增多症是一种遗传性严重联合免疫缺陷病，其特征为新生儿剥脱性红皮病。本文报道了一名患有微小病变型肾病综合征和奥门氏网状细胞增多症的新生儿。淋巴细胞功能异常可解释该肾病和皮肤病变。
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