Interventional pulmonary procedures and their outcomes in patients with STAT3 hyper IgE syndrome

BMC Surgery

Research

Zahra Chavoshzadeh¹  Anne Puel²  Abolghasem Daneshvar Kakhki³  Kambiz Sheikhy³  Seyed Alireza Mahdaviani⁴  Zahra Daneshmandi⁴  Seyedeh Atefeh Hashemi-Moghaddam⁴  Mazdak Fallahi⁴  Mahsa Rekabi⁴  Mahnaz Jamee⁵  Nima Rezaei⁶  Soodeh Ghadimi⁷  Seyed Reza Saghebi⁸  Saviz Pejhan⁸ 

[1] Immunology and Allergy Department, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran;Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM U1163, Paris, France;Lung Transplantation Research Center (LTRC), National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran;Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran;Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran;Pediatric Infections Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran;Laboratory for Pediatric Immunology, Department of Pediatrics, Willem-Alexander Children’s Hospital, Leiden University Medical Center, Leiden, Netherlands;Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran;School of Medicine, Islamic Azad University of Medical Sciences, Tehran, Iran;Tracheal Diseases Research Center (TDRC), National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran;

关键词: Hyper-IgE syndrome;    Dominant-negative STAT3 mutation;    Job’s syndrome;    Lung surgery;    Complications;    Bronchopleural fistula;   

DOI  :  10.1186/s12893-023-02193-2

 received in 2022-10-23, accepted in 2023-09-11,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundSTAT3 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.MethodFour 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.ResultRecurrent 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.ConclusionPatients 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.

【 授权许可】

CC BY   
© BioMed Central Ltd., part of Springer Nature 2023