Treatment of a patient with Job's syndrome - a case report
Journal Title: Forum Ortodontyczne - Year 2012, Vol 8, Issue 3
Abstract
The Job’s syndrome (HIES, hyperimmunoglobulinemia E) is primary immunodeficiency disease. The clinical triad of symptoms such as elevated serum IgE-level (>2000 IU/ml), recurrent bacterial and candidal infections (Staphylococcus aureus and Candida albicans) and recurrent pneumonias are typical for this entity. Most common feature of HIES are “cold abscesses”. Abnormal neutrophil chemotaxis is the mechanism of this disease. Various skeletal abnormalities have been reported in the Job’s syndrome (scoliosis, joint hyperextensibility and frequent fractures). Patients have characteristic facial features: prominent brow, wide alar base of nose, increased thickness of the soft tissue of the ears and nose. Eruption of permanent teeth is influenced by the presence of primary teeth whereas the eruption of primary teeth occurred on time in these patients, and there is no defect in the eruption of the first and second permanent molars. There have been about 250 HIES patients documented in the literature. Most cases of HIES are sporadic, but autosomal dominant and autosomal recessive inheritance also have been described. We report 11 years old patient with hyper IgE syndrome, diagnosed at age of 8.5 years on the basis of hyperimmunoglobulinaemia E and recurrent pulmonary and cutaneous infections. [b]The aim of this stud[/b]y was to present oral status and treatment of patient with Job’s syndrome. [b]Conclusions: [/b]The dentitial abnormality and delayed eruption of the teeth is a part of Job’s syndrome. Orthodontic treatment of these patients is difficult due to severe recurrent infections of the mouth. Treatment refers to the process of steering the dentition. (Orthod. Forum 2012; 8: 135-142). Received: 30.07.2012 Accepted: 10.09.2012
Authors and Affiliations
Dominika Soja, Joanna Półćwiartek-Mazur, Lucyna Sroga-Szajer
Keywords
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