Severe combined immunodeficiency: A cohort of 40 patients

Authors: Yeganeh, Mehdi¹; Heidarzade, Marzieh²; Pourpak, Zahra¹; Parvaneh, Nima¹; Rezaei, Nima¹; Gharagozlou, Mohammad¹; Movahedi, Masoud¹; Shabestari, Mahnaz Sadeghi³; Mamishi, Setareh⁴; Aghamohammadi, Asghar¹; Moin, Mostafa¹

Source: Pediatric Allergy and Immunology, Volume 19, Number 4, June 2008 , pp. 303-306(4)

Publisher: Wiley-Blackwell

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Abstract:

Yeganeh M, Heidarzade M, Pourpak Z, Parvaneh N, Rezaei N, Gharagozlou M, Movahedi M, Shabestari MS, Mamishi S, Aghamohammadi A, Moin M. Severe combined immunodeficiency: A cohort of 40 patients.

Pediatr Allergy Immunol 2008: 19: 303-306.

Severe Combined Immunodeficiency (SCID) consists of a heterogeneous group of genetic disorders characterized by an arrest in T lymphocyte development which is variably associated with an abnormal differentiation of B and NK cells. In order to depict the clinical state of Iranian patients with SCID, records of forty patients were reviewed. Patients were classified based on the flow cytometry data in two groups of B− and B+. In thirty two families (80%) parents were consanguine and in 17 families (50%) there were affected members other than proband. We showed that autosomal forms of SCID might be more frequent due to higher rate of consanguineous marriages. Alongside several infective complications, complicated Bacillus Calmette-Guérin (BCG) vaccination was documented in 18 cases (45%) following the routine vaccination at birth. BCG immunization is still a part of standard vaccination for newborns in developing countries; whereas in communities with a better health condition it could be held for a few months and performed for kids whose immune system sounds intact. We discuss where consanguine mating is common, a test of screening should be run timely. A complete blood count of cord blood could reveal lymphocytopenia at birth; this helps early diagnosis. Genetic consultation would help the families with affected members preventing new SCID offspring.

Keywords: severe combined immunodeficiency; Bacillus Calmette-Guérin

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1399-3038.2007.00647.x

Affiliations: 1: Immunology, Asthma and Allergy Research Institute, Division of allergy and clinical immunology, Children's Medical Centre, Medical Sciences/University of Tehran, Tehran 2: Department of Pediatrics, Kashan University of Medical Sciences, Kashan 3: Department of Pediatrics, Children's Hospital, Tabriz University of Medical Sciences, Tabriz 4: Division of Pediatric Infectious Diseases, Children's Medical Centre, Medical Sciences/University of Tehran, Tehran, Iran

Publication date: 2008-06-01

Severe combined immunodeficiency: A cohort of 40 patients

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