Lung Parenchyma Surgery in Autosomal Dominant Hyper-IgE Syndrome

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• 作者：Alexandra F. Freeman (1)
  Ellen D. Renner (2)
  Carolyn Henderson (1)
  Anne Langenbeck (2)
  Kenneth N. Olivier (1)
  Amy P. Hsu (1)
  Beate Hagl (2)
  Annette Boos (2)
  Joie Davis (1)
  Beatriz E. Marciano (1)
  Lisa Boris (1)
  Pamela Welch (1)
  Julie Sawalle-Belohradsky (2)
  Bernd H. Belohradsky (2)
  King F. Kwong (3)
  Steven M. Holland (1)
  
• 关键词：Hyper ; IgE syndrome ; STAT3 ; Job’s syndrome ; lung surgery ; pulmonary complications ; IgE ; bronchopleural fistula
• 刊名：Journal of Clinical Immunology
• 出版年：2013
• 出版时间：July 2013
• 年：2013
• 卷：33
• 期：5
• 页码：896-902
• 全文大小：214KB
• 参考文献：1. Holland SM, DeLeo FR, Elloumi HZ, et al. STAT3 mutations in the hyper-IgE syndrome. N Engl J Med. 2007;357:1608-9. CrossRef
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  3. Sowerwine KJ, Holland SM, Freeman AF. Hyper-IgE syndrome update. Ann N Y Acad Sci. 2012;1250:25-2. CrossRef
  4. Chandesris M, Melki I, Natividad A, et al. Autosomal dominant STAT3 deficiency and hyper-IgE syndrome: molecular, cellular, and clinical features from a French national survey. Medicine. 2012;91:e1–e19. CrossRef
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  17. Goussetis E, Peristeri I, Kitra V, et al. Successful long-term immunologic reconstitution by allogeneic hematopoietic stem cell transplantation cures patients with autosomal dominant hyper-IgE syndrome. J Allergy Clin Immunol. 2010;126:392-. CrossRef
  
• 作者单位：Alexandra F. Freeman (1)
  Ellen D. Renner (2)
  Carolyn Henderson (1)
  Anne Langenbeck (2)
  Kenneth N. Olivier (1)
  Amy P. Hsu (1)
  Beate Hagl (2)
  Annette Boos (2)
  Joie Davis (1)
  Beatriz E. Marciano (1)
  Lisa Boris (1)
  Pamela Welch (1)
  Julie Sawalle-Belohradsky (2)
  Bernd H. Belohradsky (2)
  King F. Kwong (3)
  Steven M. Holland (1)
  
  1. Laboratory of Clinical Infectious Diseases, NIAID, NIH, NIH Building 10 Room 11 N234, 9000 Rockville Pike, Bethesda, MD, 20892, USA
  2. University Children’s Hospital at Dr. von Haunersches Kinderspital, Ludwig Maximilian University, Munich, Germany
  3. Section of Thoracic Oncology, Surgery Branch, CCR, NCI, NIH, Bethesda, MD, USA
  
• ISSN：1573-2592

文摘

Purpose Autosomal dominant hyper-IgE syndrome (AD-HIES) due to heterozygous STAT3 mutation is a primary immunodeficiency characterized by eczema, elevated serum IgE, recurrent infections, and connective tissue and skeletal findings. Healing of pneumonias is often abnormal with formation of pneumatoceles and bronchiectasis. We aimed to explore whether healing after lung surgery is also aberrant. Methods We retrospectively analyzed the medical records of 32 patients with AD-HIES who received lung surgery for the management of pulmonary infections from 1960 to 2011. We collected information including patient demographics, STAT3 mutation status, clinical history, surgical and medical procedures performed, complications, related medical treatments, and outcomes. Results More than 50?% of lung surgeries had associated complications, with the majority being prolonged bronchopleural fistulae. These fistulae often led to empyemas that necessitated additional interventions including prolonged antibiotics, prolonged thoracostomy tube drainage and re-operations. Conclusion Lung surgery in AD-HIES patients is associated with high complication rates. STAT3 mutations likely lead to abnormalities in tissue remodelling that are further exacerbated by infection.