Paper Information

Title: 

SEVERE AND MODERATE FORMS OF LEUKOCYTE ADHESION DEFICIENCY TYPE I

Type: PAPER
Author(s): ENTEZARI N.*,MOVAHEDI MASOUD,POURPAK Z.,MAMISHI S.,CHAVOSHZADEH Z.,GHARAHGOZLOU MOHAMMAD,MIRSAEID GHAZI B.,FAZL ELAHI M.R.,ZANDIEH F.,BEMANIAN M.H.,AGHAMOHAMMADI ASGHAR,FARHOUDI A.A.H.
 
 *IMMUNOLOGY, ASTHMA AND ALLERGY RESEARCH INSTITUTE, MEDICAL SCIENCES/UNIVERSITY OF TEHRAN, IR., IRAN
 
Name of Seminar: BIENNIAL CONGRESS ON IMMUNOLOGY, ASTHMA AND ALLERGY
Type of Seminar:  CONGRESS
Sponsor:  IRAN UNIVERSITY OF MEDICAL SCIENCES AND HEALTH SERVICES
Date:  2007Volume 6
 
 
Abstract: 

Background: LAD I is a rare, inherited, autosomal recessive, immunodeficiency disease. Based on the expression degree of CD18, the disease was subdivided into severe (<1% expression of CD18) and moderate (1%-30% expression of CD18) phenotypes. The purpose of this report is to evaluate severe and moderate forms of the disease in the LAD patients.
Method: Fifteen patients with LAD type I who were referred to the department of Clinical, Immunology and Allergy in Children Medical Center over the last 14 years were evaluated in the study. A specific questionnaire was completed for them all and laboratory findings included CBC, CD18, CD11 (a, b, c) were reported.
Results: Clinical details of 15 patients were reviewed (10 male, 5 female). Patients' ages ranged from 10 months to 14 years (median 4 years).
In our study we found seven patients (46.7%) with severe phenotype (four male, three female). Six patients (40%) had moderate phenotype (four male, two female). The occurrence of infection was the most prominent clinical manifestation in the LAD I patients. Five patients (71.4%) with severe phenotype LAD I had a history of delayed umbilical cord separation, but in moderate phenotype three of them (50%) had this symptom. Since diagnosis, six (46.2%) of the LAD I patients had died that four of them had severe and two had moderate phenotypes.
Discussion: The more severe phenotype usually results in mortality prior to 2 years of age due to extensive necrotizing bacterial infections. A partial or moderate form of LAD type I has a milder clinical course. Patients with the moderate phenotype are usually diagnosed later in life, pyogenic infections are not as severe as in the classic form and do not typically have delayed umbilical cord separation.

 
Keyword(s): 
 
Yearly Visit 20   tarjomyar
 
Latest on Blog
Enter SID Blog