Department of Paediatric Immunology and Leukocyte Biology, National Institute of Immunohaematology (ICMR), KEM Hospital, Parel, Mumbai, India.
Indian Pediatr. 2012 Jan;49(1):43-5. doi: 10.1007/s13312-012-0005-9. Epub 2011 May 30.
Leukocyte adhesion deficiency type I (LAD-I) is a rare, inherited immunodeficiency with defect in the recruitment of leukocyte to the site of inflammation. Patients with severe LAD-I have absent or markedly reduced expression of CD18 and CD11. Here we report clinical profile of 7 cases of LAD-I diagnosed at our center over a period of 3 years. Recurrent skin and mucous membrane infections were the major presenting manifestations. All children had a history of delayed cord separation.
白细胞黏附缺陷症 I 型（LAD-I）是一种罕见的遗传性免疫缺陷病，其特征为白细胞向炎症部位募集缺陷。严重 LAD-I 患者的 CD18 和 CD11 表达缺失或显著减少。本研究报道了在过去 3 年中，我们中心诊断的 7 例 LAD-I 患者的临床特征。复发性皮肤和黏膜感染是主要的表现形式。所有患儿均有脐带延迟脱落的病史。
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