Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012 May;5(5):52-6.
doi: 10.1097/WOX.0b013e318258830f. Epub 2012 May 15.

Primary immunodeficiency diseases in oman: five years' experience at sultan qaboos university hospital

Salem Al-Tamemi  1 , Ibtisam Elnour, David Dennison
Affiliations

Primary immunodeficiency diseases in oman: five years' experience at sultan qaboos university hospital

Salem Al-Tamemi et al. World Allergy Organ J. 2012 May.

Abstract

Background: Primary immunodeficiency diseases (PIDs) are considered rare but are generally assumed to be more common in Middle Eastern countries. The prevalence and characteristics of PIDs are unknown in Oman.

Methods: Sultan Qaboos University Hospital is the national referral center for PID in Oman during the study period. Patients were diagnosed and classified according to the clinical and laboratory criteria of PID reported by the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. A registry was created, and patient data were analyzed between July 2005 and July 2010.

Results: Over a 5-year period, there were a total of 90 patients, with an estimated prevalence of 4.5 cases per 100,000. The most common form of immunodeficiency was phagocyte disorders (42%), mainly chronic granulomatous disease, followed by predominantly antibody disorders (18%), other well-defined PID syndromes (13%), and combined immunodeficiencies (12%). The median age of onset of symptoms was 9 months. The median age of diagnosis was 24 months. Consanguinity was present in 81% of patients. The most common infectious presentation was pneumonia (42%), followed by deep abscess (27%) and BCGosis (12%). A total of 25% of patients required intravenous immunoglobulins treatment, 4% required gamma interferon therapy, and 11% underwent bone marrow transplantation. Of all PID patients, 90% survived treatment, whereas 10% did not.

Conclusions: The estimated minimum prevalence of PID in Oman is 4.5 cases per 100,000, with a predominance of phagocyte disorders. Consanguinity is a significant factor; pneumonia and deep abscesses were the main infectious presentations. The overall survival rate was 90%. Strategies are needed to improve the care for PID patients and to increase the awareness among parents and physicians.

PubMed Disclaimer

Conflict of interest statement

The authors have no funding or conflicts of interest to disclose.

Figures

FIGURE 1
FIGURE 1
Types of primary immunodeficiency in Oman.
FIGURE 2
FIGURE 2
Infectious presentation of PID patients, percentage values from total number.
FIGURE 3
FIGURE 3
Treatment of PID patients.
FIGURE 4
FIGURE 4
Survival rate of PID.
See this image and copyright information in PMC

References

    1. Fischer A. Human primary immunodeficiency diseases. Immunity. 2007;27:835–845 - PubMed
    1. Aghamohammadi A, Bahrami A, Mamishi S, Mohammadi B, Abolhassani H, Parvaneh N, Rezaei N. Impact of delayed diagnosis in children with primary antibody deficiencies. J Microbiol Immunol Infect. 2011;44:229–234 - PubMed
    1. Geha RS, Notarangelo LD, Casanova JL, Chapel H, Conley ME, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol. 2007;120:776–794 - PMC - PubMed
    1. Ryser O, Morell A, Hitzig WH. Primary immunodeficiencies in Switzerland: first report of the national registry in adults and children. J Clin Immunol. 1988;8:479–485 - PubMed
    1. Matamoros Florí N, Mila Llambi J, Español Boren T, Raga Borja S, Fontan Casariego G. Primary immunodeficiency syndrome in Spain: first report of the National Registry in Children and Adults. J Clin Immunol. 1997;17:333–339 - PubMed