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Hyper Ig E syndrome (Job syndrome, HIES) - radiological images of pulmonary complications on the basis of three cases.

Jończyk-Potoczna K, Szczawińska-Popłonyk A, Warzywoda M, Bręborowicz A, Pawlak B - Pol J Radiol (2012)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Radiology Department, Chair of Radiology, Poznań University of Medical Sciences, Poznań, Poland.

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.

Case report: 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.

No MeSH data available.


Related in: MedlinePlus

Axial CT scan of the chest, pulmonary window. The same patient, follow-up examination. Bronchiectasis.
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f1B-poljradiol-77-2-69: Axial CT scan of the chest, pulmonary window. The same patient, follow-up examination. Bronchiectasis.

Mentions: The girl has been suffering from recurrent infections of the respiratory tract – otitis media, bronchitis or pneumonia, accompanied by lower airway obturation – since early infancy. Moreover, she became sick with pertussis at the age of four. Clinically severe pneumonia at the age of five was the reason for hospitalization at the Clinic of Pulmonology, Pediatric Allergology and Clinical Immunology at Poznań University of Medical Sciences. Her past medical history revealed symptoms such as: generalized pustular skin lesions and multiple furuncles since infancy, popleateal abscess requiring surgical intervention, recurrent peridental abscesses penetrating to maxillary sinuses and the orbit, delayed loss of deciduous teeth, pathological fractures and excessive joint mobility. Laboratory examinations showed eosynophilia, immunoglobulin E level of 51 820 IU/L and multidirectional neutrophil dysfunction involving migration, chemotaxis, phagocytosis and enzymatic activity. Considering the course of the disease to date and overall clinical picture, a diagnosis of hyper-IgE syndrome was stated, which was then verified with a genetic study and identification of STAT3 mutation. Clinical and radiological improvement was not achieved despite intensive treatment. Persistent areas of parenchymal consolidation visible on X-ray pictures, appearance of a radiolucent cystic lesion and presence of fluid in the pleural cavity prompted broadening of the diagnostic process by a CT examination (Figure 1A). CT imaging confirmed the diagnosis of postinflammatory cyst infection and aspergilloma. Lack of response to conservative treatment was an indication for a resection of the involved lobe. In long-term observation, the patient suffered from recurrent bronchopneumonias and recently performed CT examination revealed, apart from numerous nodular lesions, bronchiectasis (Figure 1B).


Hyper Ig E syndrome (Job syndrome, HIES) - radiological images of pulmonary complications on the basis of three cases.

Jończyk-Potoczna K, Szczawińska-Popłonyk A, Warzywoda M, Bręborowicz A, Pawlak B - Pol J Radiol (2012)

Axial CT scan of the chest, pulmonary window. The same patient, follow-up examination. Bronchiectasis.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3403805&req=5

f1B-poljradiol-77-2-69: Axial CT scan of the chest, pulmonary window. The same patient, follow-up examination. Bronchiectasis.
Mentions: The girl has been suffering from recurrent infections of the respiratory tract – otitis media, bronchitis or pneumonia, accompanied by lower airway obturation – since early infancy. Moreover, she became sick with pertussis at the age of four. Clinically severe pneumonia at the age of five was the reason for hospitalization at the Clinic of Pulmonology, Pediatric Allergology and Clinical Immunology at Poznań University of Medical Sciences. Her past medical history revealed symptoms such as: generalized pustular skin lesions and multiple furuncles since infancy, popleateal abscess requiring surgical intervention, recurrent peridental abscesses penetrating to maxillary sinuses and the orbit, delayed loss of deciduous teeth, pathological fractures and excessive joint mobility. Laboratory examinations showed eosynophilia, immunoglobulin E level of 51 820 IU/L and multidirectional neutrophil dysfunction involving migration, chemotaxis, phagocytosis and enzymatic activity. Considering the course of the disease to date and overall clinical picture, a diagnosis of hyper-IgE syndrome was stated, which was then verified with a genetic study and identification of STAT3 mutation. Clinical and radiological improvement was not achieved despite intensive treatment. Persistent areas of parenchymal consolidation visible on X-ray pictures, appearance of a radiolucent cystic lesion and presence of fluid in the pleural cavity prompted broadening of the diagnostic process by a CT examination (Figure 1A). CT imaging confirmed the diagnosis of postinflammatory cyst infection and aspergilloma. Lack of response to conservative treatment was an indication for a resection of the involved lobe. In long-term observation, the patient suffered from recurrent bronchopneumonias and recently performed CT examination revealed, apart from numerous nodular lesions, bronchiectasis (Figure 1B).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Radiology Department, Chair of Radiology, Poznań University of Medical Sciences, Poznań, Poland.

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.

Case report: 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.

No MeSH data available.


Related in: MedlinePlus