Nigar Sehar, Khan Ejaz Ahmed, Ahmad Tahir Aziz
Shifa International Hospital, Islamabad.
J Pak Med Assoc. 2018 Jan;68(1):119-122.
Leukocyte adhesion deficiency (LAD) is a rare primary immunodeficiency disorder with autosomal recessive inheritance which is characterized by presence of a defect of phagocytic function resulting from a lack of leukocyte cell surface expression of b2 integrin molecules (CD11 and CD18) that are essential for chemotaxis. The classic symptoms of the disease are failure of separation of the umbilical cord and recurrent bacterial infections, which continue throughout life. We describe here two cases of infants who presented with characteristic history of recurrent infections, delayed separation of umbilical cord and marked leukocytosis.
白细胞黏附缺陷(LAD)是一种罕见的常染色体隐性遗传性原发性免疫缺陷病,其特征是由于缺乏对趋化性至关重要的β2整合素分子(CD11和CD18)的白细胞细胞表面表达,导致吞噬功能缺陷。该病的典型症状是脐带脱落延迟和反复细菌感染,这些症状会持续终生。我们在此描述两例婴儿病例,他们有反复感染、脐带脱落延迟和明显白细胞增多的典型病史。