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01-05-2007

Clinical and Laboratory Findings in Iranian Patients with Leukocyte Adhesion Deficiency (Study of 15 Cases)

Authors: Masoud Movahedi, Neda Entezari, Zahra Pourpak, Setareh Mamishi, Zahra Chavoshzadeh, Mohammad Gharagozlou, Bahram Mir-Saeeid-Ghazi, Mohammad-Reza Fazlollahi, Fariborz Zandieh, Mohammad-Hasan Bemanian, Aboulhasan Farhoudi

Published in: Journal of Clinical Immunology | Issue 3/2007

Abstract

Leukocyte adhesion deficiency type I (LAD I) is a rare, inherited, autosomal recessive, immunodeficiency disease caused by the combined loss of expression on the surface of leukocytes of the leukocyte integrins. We describe the clinical and laboratory findings for 15 patients with LAD I.

The range of patients’ ages was from 10 month to 14 years (median 4 years) and 93.3% of their parents had consanguineous marriages. The most commonly occurred manifestations were: recurrent infections (93.3%), poor wound healing (86%), oral ulcers (86%), and skin abscesses (80%). The most specific laboratory findings were defect in CD18 in all of 15 patients.

The most common symptoms in these patients are poor wound healing and oral ulcer, so, the clinical physicians should pay special attention to these symptoms. Furthermore, because of considerable rate of consanguineous marriages in parents of LAD patients, we suggested more genetic studies on this disease and genetic consultation for these families.

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Title: Clinical and Laboratory Findings in Iranian Patients with Leukocyte Adhesion Deficiency (Study of 15 Cases)
Authors: Masoud Movahedi
Neda Entezari
Zahra Pourpak
Setareh Mamishi
Zahra Chavoshzadeh
Mohammad Gharagozlou
Bahram Mir-Saeeid-Ghazi
Mohammad-Reza Fazlollahi
Fariborz Zandieh
Mohammad-Hasan Bemanian
Aboulhasan Farhoudi
Publication date: 01-05-2007
Publisher: Springer US
Published in: Journal of Clinical Immunology / Issue 3/2007
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-006-9069-4

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