Primary Immunodeficiency Diseases in Oman: Five Years' Experience at Sultan Qaboos University Hospital

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.

Effects of family history and consanguinity in primary immunodeficiency diseases in children in Qatar

“…We observed positive family history in 66.4% of our patients. This rate is higher than that reported by Reda, et [14], Azarsis from Turkey (43.5%) [15] and Al-Herz et al from Kuwait (44%) [16]. 0.004) effect on reducing the delay age of diagnosis of D among children.…”

“…Since many diseases of the predmoniantly antibody deficencies and other well-defined ID syndromes are autosomal recesive and they constitues together almost half of our cohort, male-to-female ratio (1.34:1) is not high in in this study. Compared to reports from other Middle East countries, the mean delay age in making diagnosis of PID in our group (18 months) was less than that reported from Egypt (29.9 months) [10] and Kuwait (27.3 months) [12], but longer than that from Oman (15.4 months) [14] and Turkey (8 months) [15].…”

“…0.004) effect on reducing the delay age of diagnosis of D among children. Data compiled from various reports from Middle East countries particularly Egypt [10], Kuwait [12], Oman [14] and Turkey [15] clearly revealed an inverse relationship (correlation coefficient −0.44) between rate of family history of PID and delay in making diagnosis of PID, Figure 2. This is important since early diagnosis and institution of appropriate management reduce mortality and morbidity of PID.…”

“…Since PID is a heterogeneous group of disorders [1], the relationship of consanguinity and PID has explored in different studies and reviews [10][11][12][13][14][15].…”

“…During the last few years, reports from communities with high rates of consanguinity indicate high rate of PID in the following communities: Saudi Arabia [9], Egypt [10], Iran [11], Kuwait [12], Morocco [13], Oman [14], Tunisia [13], Turkey [15]. With the high consanguinity rate in the Qatari population, genetic disorders and congenital birth defects were found to be relatively high for the population size of the country [5].…”