Lack of leucocyte adhesion molecules (LAM) is associated with:
Correct Answer: Delayed fall of umbilical cord
Description: a. Delayed fall of umbilical cord(Ref: Nelson's 20/e p 1027, Ghai 8/e p 187)Leukocyte adhesion deficiency (LAD)Salient featuresLAD type 1LAD type 2InheritanceAutosomal recessiveAutosomal recessiveBasic defectDefect in CD 18 moleculeQ required for biosynthesis of B2 chain shared by LFA-1 & Mac-1 integrinsAbsence of Sialyl-Lewis X, the fucose-containing ligand for P & E-selection, due to defect in fucosyl-transferase responsible for binding fucose moieties to protein backbonesClinical featuresRecurrent bacterial infections, delayed separation of umbilical cord stumpQRecurrent bacterial infections, Bombay blood groupQ and mental retardation
Category:
Pediatrics
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