Paper Information

Title: 

CYSTIC ADENOMATOID TRANSFORMATION (CAT) OF LUNG IN A CHILD WITH HYPER IGE SYNDROME

Type: PAPER
Author(s): ALE YASIN S.,AMIN REZA,MOGHTADERI M.*
 
 *SHIRAZ UNIVERSITY OF MEDICAL SCIENCES (SUMS)
 
Name of Seminar: BIENNIAL CONGRESS ON IMMUNOLOGY, ASTHMA AND ALLERGY
Type of Seminar:  CONGRESS
Sponsor:  IRAN UNIVERSITY OF MEDICAL SCIENCES AND HEALTH SERVICES
Date:  2007Volume 6
 
 
Abstract: 

Hyper IgE syndrome with recurrent infections is a rare immunodeficiency characterized by recurrent skin infection and pulmonary abcess and extremely elevated levels of IgE in serum, associated facial and skeletal features have been recognized. Their frequency and genetic basis are poorly understood. The patient is a seven-year old girl presented with two year-history of productive cough, super infected facial eczema from infancy and two prior hospitalizations for pneumonia and perianal abcess. She had coarse face, crackles on both lungs, clubbing of fingers, laxicity of elbow joints. Immunological work up revealed eosinophilia and markedly raised IgE levels. In view of the clinical features and other finding diagnosis of hyper IgE syndrome was suggested. Chest X-ray revealed multiple large cystic lesions in left total lung which confirmed by spiral CT-scan. Left thoracotomy and pneumanectomy was done and removed severe adhesions. Histological report revealed cystic adenomatoid transformation. CAT is characterized by the presence of variously sized cysts lined by epithelium. Probably, this is the first report of CAT in a child with hyper IgE syndrome due to repeated infections. Early diagnosis and prophylactic antibiotic therapy is effective in management of these patients.

 
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