Bài viết nghiên cứu nhằm tìm ra các dấu hiệu lâm sàng được chú ý phát hiện sớm, trẻ sẽ được chẩn đoán và điều trị kịp thời suy giảm miễn dịch bẩm sinh, tránh các biến chứng do nhiễm khuẩn nặng và tránh được tử vong. | Journal of Pediatric Research and Practice Vol. 4 No. 6 2020 21-29 Research Paper Leukocyte Adhesion Deficiency LAD Type 1 Nguyen Thi Van Anh Le Thi Minh Huong Nguyen Ngoc Quynh Le Thuc Thanh Huyen Le Quynh Chi Nguyen Van Khiem Vietnam National Children s Hospital No 18 879 La Thanh Dong Da Hanoi Vietnam Received 29 November 2020 Accepted 31 December 2020 Abstract Primary immunodeficiency is a group of genetic diseases that cause a decrease in the body s immune response leading to recurrent infections even severe infections and death. According to the classification of the International Union of Immunological Societies IUIS in 2019 there are more than 430 different primary immunodeficiency diseases. Leukocyte adhesion defect affects seriously the phagocytic function of leukocytes. The disease is caused by a mutation of the ITGB2 gene located on chromosome that encodes CD18 causing defects in the membrane expression of the leukocyte-binding glycoprotein the neutrophils cannot extravasate and fight against bacteria in tissues. As a result the ulcer is very difficult to cure unable to produce pus delayed umbilical cord loss recurrent infection of many organs. The white blood cells are always very high persistent increase absent or reduced CD18 expression in the leukocyte membrane. If diagnosed early patients will receive prophylactic antibiotics and hematopoietic stem cell transplant and good prognosis. Keywords Leukocyte Adhesion Deficiency decrease CD18in the leukocyte extremely elevated of neutrophils. ____ Corresponding author. E-mail address bascsivananh@ https 21 . Anh et al. Journal of Pediatric Research and Practice Vol. 4 No. 6 2020 21-29 Suy giảm miễn dịch bẩm sinh thể khiếm khuyết bám dính bạch cầu type 1 Nguyễn Thị Vân Anh Lê Thị Minh Hương Nguyễn Ngọc Quỳnh Lê Thục Thanh Huyền Lê Quỳnh Chi Nguyễn Văn Khiêm Bệnh viện Nhi Trung ương Số 18 879 La Thành Đống Đa Hà Nội Việt Nam Nhận ngày 29 tháng 11 năm
