Primary Immunodeficiency Diseases in Oman

10-Year Experience in a Tertiary Care Hospital

Salem Al-Tamemi, Shafiq Ur Rehman Naseem, Nabila Al-Siyabi, Ibtisam El-Nour, Abdulhakim Al-Rawas, David Dennison

Research output: Contribution to journalArticle

4 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1-8
Number of pages8
JournalJournal of Clinical Immunology
DOIs
Publication statusAccepted/In press - Oct 3 2016

Fingerprint

Oman
Tertiary Healthcare
Tertiary Care Centers
Population
Consanguinity
Policy Making
Rare Diseases
Age of Onset
Siblings
Primary Health Care
Pneumonia
Survival
Mortality
Antibodies
Infection

Keywords

  • chronic granulomatous disease
  • mortality
  • Oman
  • Primary immunodeficiency
  • severe combined immunodeficiency
  • survival

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

@article{4628dd9bceea42e4b404a5d4ef08f450,
title = "Primary Immunodeficiency Diseases in Oman: 10-Year Experience in a Tertiary Care Hospital",
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.",
keywords = "chronic granulomatous disease, mortality, Oman, Primary immunodeficiency, severe combined immunodeficiency, survival",
author = "Salem Al-Tamemi and Naseem, {Shafiq Ur Rehman} and Nabila Al-Siyabi and Ibtisam El-Nour and Abdulhakim Al-Rawas and David Dennison",
year = "2016",
month = "10",
day = "3",
doi = "10.1007/s10875-016-0337-7",
language = "English",
pages = "1--8",
journal = "Journal of Clinical Immunology",
issn = "0271-9142",
publisher = "Springer New York",

}

TY - JOUR

T1 - Primary Immunodeficiency Diseases in Oman

T2 - 10-Year Experience in a Tertiary Care Hospital

AU - Al-Tamemi, Salem

AU - Naseem, Shafiq Ur Rehman

AU - Al-Siyabi, Nabila

AU - El-Nour, Ibtisam

AU - Al-Rawas, Abdulhakim

AU - Dennison, David

PY - 2016/10/3

Y1 - 2016/10/3

N2 - 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.

AB - 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.

KW - chronic granulomatous disease

KW - mortality

KW - Oman

KW - Primary immunodeficiency

KW - severe combined immunodeficiency

KW - survival

UR - http://www.scopus.com/inward/record.url?scp=84989824548&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84989824548&partnerID=8YFLogxK

U2 - 10.1007/s10875-016-0337-7

DO - 10.1007/s10875-016-0337-7

M3 - Article

SP - 1

EP - 8

JO - Journal of Clinical Immunology

JF - Journal of Clinical Immunology

SN - 0271-9142

ER -