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Leukocyte Adhesion Deficiency Type 1: A Case Series and Review of the Literature

Authors :
Houshang Gorjipour
Zahra Chavoshzadeh
Alireza Fahimzad
Paniz Hashemitari,
Sepideh Darougar
Source :
European Medical Journal Allergy & Immunology, Vol 4, Iss 1, Pp 95-100 (2020)
Publication Year :
2020
Publisher :
European Medical Journal, 2020.

Abstract

Leukocyte adhesion deficiency type-1 (LAD-1) is a rare primary immunodeficiency that is characterised by compromised neutrophil adhesion and transmigration to infection or inflammation sites. In this article, the authors report the cases of five patients with LAD-1 deficiency. The aim of this study is the demonstration of the wide variety of manifestations in these patients with a review of the literature. Patients were both male and female, with their ages ranging from 1 month to 10 years old. Omphalitis was the most common presentation in this series, followed by pneumonia and gingivitis. Impaired wound healing and cellulitis were other common findings in these patients. Two of the patients did not show delayed separation of the umbilical cord. The findings indicate that LAD-1 remains a life-threatening condition with omphalitis, oral, skin, respiratory tract, and ear infections as the most common complications. Early identification of these patients is essential in ensuring a definitive diagnosis and early implementation of haematopoietic stem cell transplantation.

Details

Language :
English
ISSN :
23989130
Volume :
4
Issue :
1
Database :
Directory of Open Access Journals
Journal :
European Medical Journal Allergy & Immunology
Publication Type :
Academic Journal
Accession number :
edsdoj.b0c671b950984fc495b5d29eb6d4401d
Document Type :
article