Title: Leucocyte Adhesion Deficiency Type -1 Presenting as a Severe Anal Ulceration-A Case Report
Authors: Rakesh Kotha, Arjun Jadhao, Himabindu Singh, Nirmala C, Sadiua
DOI: https://dx.doi.org/10.18535/jmscr/v6i9.81
Abstract
Leucocyte adhesion deficiency is a rare AR inherited primary immunodeficiency disorder with neutrophila due to lack of leukocyte recruitment at the site of infection. LAD type I results from lack of expression of leukocyte β2 integrin’s (CD 11 and CD 18) a trans membrane signal transfer proteins that are essential for leukocyte adhesion and chemo taxis. It clinically presents as delayed umbilical cord separation, no pus formation and uncontrolled infections. In severe form where expression is <1% prognosis was poor usually they succumb to prone life-threatening infection within two years.
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