Assessment of Primary Immunodeficiency Disorders Among Children at Zagazig University Hospital

Abstract

Aim of study: To identify and report various type of primary immunodeficiency disorders in children at Zagazig university hospitals, and their characteristic features, clinical manifestations and laboratory profiles. Patients and methods; patients with history,clinical findings and laboratory findings matching with any of eight classes of primary immunodeficiency were included in this study, also we used ten warning signs,immunodediciency disease related score in evaluation of patients. Results: Fifty patients were diagnosed with different primary immunodeficiency disorders in Pediatric department, Zagazig University Hospital during period from July 2011 to July 2013. The spectrum of PIDs in our center was as follow: predominantly antibody deficiency was the most common category (46%) followed by combined immunodeficiency (22%) then well defined syndromes (20%), auto inflammatory disorders (8%), complement disorders (4%). No cases were diagnosed in any category of phagocytic disorders, innate immunity or immune dysregulation. Selective IgA deficiency was the most frequent disease type. Median age of onset of symptoms was 7 months, the median age of diagnosis lag was 24 months.Pneumonia was the most common presentation. Consanguinity rate was 60%. Mortality rate was 20% mostly duo to bronchopneumonia. Conclusion: primary immunodeficiency disorders are not rare in our center ,but under diagnosed.

Targeted screening for primary immunodeficiency disorders in the neonatal period and early infancy

“…Males were affected more than females, a trend seen in other parts of the world, including Europe and India10,11. The ages reported in the present study were earlier than the previously estimated12, in Zagazig who reported that the median onset age of symptoms was 7 months (range, 1–84 months) and the median age at diagnosis was 24 months (3–120 months). Zeng et al13 in their study from South China reported a median age at diagnosis of 10 months range (2–159 months).…”

“…Recurrent deep-skin and organ abscesses and deep-seated infections were significantly more frequent in the category of congenital defects in phagocyte number, function, or both. In Zagazig, Egypt another study done by A Hussien et al12, over a period of two years including 50 patients found pneumoniae, bronchitis and otitis media were the most common presentations in PID patients. Recurrent severe infections as pneumonia, chronic diarrhea, and failure to thrive are commonly thought of as classic symptoms of PID and patients with combined lower respiratory tract infections, fungal infections and lymphopenia were 12.3 times more likely to be diagnosed with a PID disorder.…”

Newborn Screening for Primary Immunodeficiencies: The Gaps, Challenges, and Outlook for Developing Countries

“…In a study on healthy Egyptian children, the level of TRECs and KRECs was found to be inversely proportional to age (46). The lowest threshold of TRECs (copies/µl blood) was 25 for the group aged 1 day to 5 months, and 24 for the group aged 5 months to 2.4 years.…”

“…Data on the prevalence of PIDs in Egypt is lacking as there is no national registry to date. The ESID database contains data from Egypt and some information can also be gained from the few published studies (33,(45)(46)(47). CID is considered the most common disease (31%), followed by predominantly antibody deficiency (30%), welldefined syndromes with PID (17.5%), and, finally, phagocytic defects (8.1%).…”