Leukocyte adhesion defect: An uncommon immunodeficiency.
J Pak Med Assoc
; 68(1): 119-122, 2018 Jan.
Article
em En
| MEDLINE
| ID: mdl-29371732
ABSTRACT
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.
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01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome da Aderência Leucocítica Deficitária
Limite:
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
J Pak Med Assoc
Ano de publicação:
2018
Tipo de documento:
Article