Comprehensive Report of Primary Immunodeficiency Disorders from a
Tertiary Care Center in Marrakesh.
Introduction Recent advances in immunologic techniques have lead to
increase recognition of primary immunodeficiency diseases (PID).
Objective We sought to analyze and describe the spectrum of PID at a
tertiary care center in Morocco. Methods A retrospective study from 2011
to 2017 was conducted on 27 children diagnosed with PID. Result Over the
study period, combined immunodeficiencies with associated or syndromic
features were the most common category (48.15%) followed by
immunodeficiencies affecting cellular and humoral immunity (25.9%),
congenital defects of phagocyte number or function (14.81%),
predominantly anti-body deficiencies (7.4%), and diseases of immune
dysregulation (3.7%). The most frequent disorder was
Ataxia-telangiectasia (44.4%). The mean age at diagnosis was 4.62
years. The consanguinity rate was 74.1%. The principal clinical signs
were lower respiratory tract infections (59.2%), neurological
manifes-tations (44.4%), failure to thrive (51.8%) and skin infections
(33.3%). Two patients who have immunodeficiencies affecting cellular
and humoral immunity died. Conclusion This study indicates that PIDs are
not rare in Morocco and that combined immunodeficiencies with associated
or syndromic features are the most common category. Future research
should focus on identifying gene defect for PID patients.