Leukocyte adhesion deficiency type 1 (LAD1) with expressed but nonfunctional CD11/CD18

D Cabanillas, L Regairaz, C Deswarte… - Journal of clinical …, 2016 - Springer
D Cabanillas, L Regairaz, C Deswarte, M García, ME Richard, JL Casanova, J Bustamante
Journal of clinical immunology, 2016Springer
To the Editor, We report here a case of leukocyte adhesion deficiency (LAD) type 1 in a 4-
year-old girl (Amerindian from Argentina), the only child of consanguineous parents (Fig. 1).
Fetal development was uncomplicated, with eutocic delivery at term. The patient's umbilical
cord detached at 10 days of age, without complications. Fifteen days later, she developed
fever and redness of the skin around the umbilical region. No purulent secretion was
observed (Fig. 1a). The patient was hospitalized with a diagnosis of omphalitis caused by E …
To the Editor, We report here a case of leukocyte adhesion deficiency (LAD) type 1 in a 4-year-old girl (Amerindian from Argentina), the only child of consanguineous parents (Fig. 1). Fetal development was uncomplicated, with eutocic delivery at term. The patient’s umbilical cord detached at 10 days of age, without complications.
Fifteen days later, she developed fever and redness of the skin around the umbilical region. No purulent secretion was observed (Fig. 1a). The patient was hospitalized with a diagnosis of omphalitis caused by E. coli. Ceftriaxone (CTX) treatment was administered, with a good outcome. During hospitalization, the patient was diagnosed with multiple congenital melanocytic nevi and aortic dilation. Laboratory tests at admission revealed leukocytosis with a predominance of neutrophils (> 40× 10/mm3), despite antibiotic treatment. At the age of 6 months, the patient suffered pneumonia of the lower right lung. She was treated with 80-mg CTX/kg/day for 14 days, with a favorable outcome. At 13 months, she was hospitalized for submaxillary lymphadenitis and neck cellulitis, which resolved after 15 days of intravenous ciprofloxacin treatment. At 14 months, she suffered from cellulitis of the finger due to
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