دراسة متلازمة فرط الغلوبولين المناعي E عند الأطفال في مستشفى الأطفال الجامعي

الملخص EN

Background & Objective: Hyperimmunoglobulin E (Job) syndrome is considered to be a relatively rare disease.

Its characterized by recurrent infections in skin (eg.

boils, abscesses), recurrent upper respiratory tract infections (eg.

otitis media, sinusitis and mastoiditis), and lower respiratory infections (eg.

recurrent pneumonia, which is often complicated by pneumatoceles and lung abscess), that are usually difficult to treat.

The highly increased IgE levels are considered to be the hallmark of the disease.

The syndrome has two types: one is inherited as an autosomal recessive, and the other, as an autosomal dominant trait.

To highlight the clinical manifestations and laboratory findigs of Job disease, and to compare it with medical literature, to study the bacterial complications and differrent types of antibacterial treatments and treir results, and to increase the level of awareness among physicians.

Material& methods: A retrospective study was carried out in Damascus University Children’s Hospital on 13 child diagnosed as Job Disease, in a period of the last four years (from 2006 till september 2010).

Results: most cases in were among males, recurrent upper and lower respiratory tract infections, recurrent otitis media, sinusitis and mastoiditis may be suggestive, skin manifistations were characterisistic and early sign of Job syndrome, and most of the bacterial complications were due to Staphylococcus Aureus.

Conclusion: A history of neonatal rash, characteristic facial appearance and retention of primary teeth, associated with elevated IgE levels and recurrent infections, are pathognomic for Job disease, therefore it is necessary to obtain more detailed history, including positive family history, and pay attention for the presence of oral and skeletal characteristic abnormalities, and conduct more advanced laboratory investigations to confirm the diagnosis.