title: Hyper Ig E syndrome (Job syndrome, HIES) - radiological images of pulmonary complications on the basis of three cases.
abstract: BACKGROUND
  Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum.
  
  
  METHODS
  The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations - fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up.
  
  
  CONCLUSIONS
  Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome.
authors: Pediatric Radiology Department, Chair of Radiology, Poznań University of Medical Sciences, Poznań, Poland.
authors: Katarzyna
authors: K
authors: Jończyk-Potoczna
authors: Aleksandra
authors: A
authors: Szczawińska-Popłonyk
authors: Małgorzata
authors: M
authors: Warzywoda
authors: Anna
authors: A
authors: Bręborowicz
authors: Bogdan
authors: B
authors: Pawlak