Abstract
Adhesion molecule deficiency type 1 is a rare disease that should be suspected in any patient whose umbilical cord presents delay in falling off, and who presents recurrent severe infections. Early diagnostic suspicion and early treatment improve the prognosis. The case of a four-month-old boy with recurrent hospitalizations because of severe bronchopneumonia and several episodes of acute otitis media with non-purulent drainage of mucus and positive bacterial cultures is presented. His medical history included neonatal sepsis and delayed umbilical cord detachment. Laboratory studies showed marked leukocytosis with predominance of neutrophils and decreased CD11b and CD18. These were all compatible with a diagnosis of leukocyte adhesion deficiency type I [LAD type 1].
Highlights
Leukocyte adhesion deficiency (LAD) type 1 is a rare disease with only 200 cases reported in the medical literature.[1]
We report the case of an infant with LAD type 1 who was diagnosed at the Pediatric Service of Hospital Parroquial de San Bernardo, in Santiago, Chile, and present a review of the medical literature
CASE REPORT A four-month-old male infant born in October 2001, who was the only child of consanguineous parents, was admitted to the intensive care unit of our hospital with diagnoses of septic shock, bilateral suppurative otitis media, thrush and diaper area candidiasis
Summary
Leukocyte adhesion deficiency (LAD) type 1 is a rare disease with only 200 cases reported in the medical literature.[1]. INTRODUCTION Leukocyte adhesion deficiency (LAD) type 1 is a rare disease with only 200 cases reported in the medical literature.[1] It was first described in 1970,2 but no cases have been described in Latin America.
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