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Primary Immunodeficiency Diseases in Oman: 10-Year Experience in a Tertiary Care Hospital

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Abstract

Purpose

Primary immunodeficiency (PID) diseases are rare, complex medical disorders that often are overlooked in clinical settings. There are emerging reports of PID from Middle Eastern populations. This study describes the features of PID patients in a tertiary care setting in Oman and compares them with regional and worldwide reports.

Method

Sultan Qaboos University Hospital (SQUH) is an academic tertiary care-level hospital for specialized healthcare, including PID patients. At the time of diagnosis, patients’ sociodemographics, clinical features, laboratory investigations, and management were entered in electronic form. This study included patients seen between August 2005 and July 2015.

Results

One hundred forty patients were registered with a minimum estimated population prevalence of 7.0/100,000. The male/female ratio was 1.6:1, the median age of onset of symptoms was 8 months, and diagnosis was 21 months with a delay of 13 months. Family history was positive in 44 %, consanguinity was present in 76 %, death of a previous sibling was present in 36 %, and there was an overall mortality in 18 %, with an 85 % probability of survival 10 years following diagnosis. The most common type of immunodeficiency was phagocytic disorders (35.0 %), followed by predominantly antibody disorders (20.7 %), combined immunodeficiency (17.8 %), other well-defined PID syndromes (15.0 %), immune dysregulation syndromes (3.5 %), complement deficiencies (3.5 %), and unclassified immunodeficiency (4.2 %). The commonest presenting infection was pneumonia (47.1 %).

Conclusion

PID is not a rare condition in Oman. The prevalence is in concordance with reports from the region but higher than in Western populations. The findings of the current study would help to improve the awareness and management of, and policy making for PID.

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Acknowledgments

We would like to thank all staff at Sultan Qaboos University Hospital who participated in the care of our patients, all physicians who referred patients for diagnosis and management, and all of our patients and families for their trust. We would like also to thank Dr. Murtadha Al-Khabori for his assistance in the analysis of survival and Professor Samir Al-Adawi for his revision of the manuscript.

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Authors and Affiliations

  1. Department of Child Health, Sultan Qaboos University Hospital, PO Box 96, PC 123 SQU Al-Khod, Muscat, Oman

    Salem Al-Tamemi, Shafiq Ur Rehman Naseem, Ibtisam El-Nour & Abdulhakim Al-Rawas

  2. Directorate of Nursing, Sultan Qaboos University Hospital, Muscat, Oman

    Nabila Al-Siyabi

  3. Department of Hematology, Sultan Qaboos University Hospital, Muscat, Oman

    David Dennison

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  1. Salem Al-Tamemi
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  2. Shafiq Ur Rehman Naseem
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  4. Ibtisam El-Nour
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  5. Abdulhakim Al-Rawas
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Corresponding author

Correspondence to Salem Al-Tamemi.

Ethics declarations

The study is approved by the Scientific Medical Research Committee and Ethical Committee at the College of Medicine and Health Sciences and Sultan Qaboos University Hospital. The investigators ensured that the study was conducted with full conformance with appropriate laws and regulations. No funding was required to carry out this study.

Conflict of Interest

All authors declare no conflict of interest, real or perceived, in relation to this work.

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Al-Tamemi, S., Naseem, S.U.R., Al-Siyabi, N. et al. Primary Immunodeficiency Diseases in Oman: 10-Year Experience in a Tertiary Care Hospital. J Clin Immunol 36, 785–792 (2016). https://doi.org/10.1007/s10875-016-0337-7

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