[Hyper IgE syndrome: three case reports]. / Síndrome Hiper IgE: a propósito de tres casos clínicos.
Rev Chil Pediatr
; 85(3): 328-36, 2014 Jun.
Article
in Es
| MEDLINE
| ID: mdl-25697250
ABSTRACT
INTRODUCTION:
Autosomal dominant Hyper IgE syndrome (HIES-AD) is a primary immunodeficiency associated with connective tissue, skeletal, vascular and brain disorders. The pathogenesis of immune deficiency lies in an alteration of Th17 cells which explains the special susceptibility of these patients to S. aureus and Candida infections.OBJECTIVE:
To describe three children diagnosed with hyper IgE syndrome and conduct a study on the subject, with special focus on the dominant form of the disease. CASE REPORTS 3 children with HIES-AD (2 males and one female) with eczema since birth, skin, ear, lung, and lymph node infections, and serum IgE levels over 2,000 IU/ml and eosinophilia values, treated with antibiotics and topically, and 7 year follow-up.CONCLUSIONS:
It is a rare condition that requires a high index of suspicion and early management of infections. One of its main diagnoses is atopic syndrome with recurrent infections but both conditions differ in context, response and resolution against infections and lack of other phenotypic characteristics.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Immunoglobulin E
/
Eczema
/
Job Syndrome
Type of study:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Language:
Es
Journal:
Rev Chil Pediatr
Year:
2014
Document type:
Article