Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome.
Bronchopleural fistula
Complications
Dominant-negative STAT3 mutation
Hyper-IgE syndrome
Job’s syndrome
Lung surgery
Journal
BMC surgery
ISSN: 1471-2482
Titre abrégé: BMC Surg
Pays: England
ID NLM: 100968567
Informations de publication
Date de publication:
23 Sep 2023
23 Sep 2023
Historique:
received:
23
10
2022
accepted:
11
09
2023
medline:
25
9
2023
pubmed:
24
9
2023
entrez:
23
9
2023
Statut:
epublish
Résumé
STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures. Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed. Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications. Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.
Sections du résumé
BACKGROUND
BACKGROUND
STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures.
METHOD
METHODS
Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed.
RESULT
RESULTS
Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications.
CONCLUSION
CONCLUSIONS
Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.
Identifiants
pubmed: 37741967
doi: 10.1186/s12893-023-02193-2
pii: 10.1186/s12893-023-02193-2
pmc: PMC10517538
doi:
Substances chimiques
STAT3 protein, human
0
STAT3 Transcription Factor
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
289Informations de copyright
© 2023. BioMed Central Ltd., part of Springer Nature.
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