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Safety of Major Abdominal Surgical Procedures in Patients with Hyperimmunoglobulinemia E (Job’s Syndrome): a Changing Paradigm?

  • Review Article
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Journal of Gastrointestinal Surgery Aims and scope

Abstract

Introduction

Autosomal dominant hyperimmunoglobulinemia E syndrome (HIES), also called Job’s syndrome, is a primary immunodeficiency characterized by the triad of elevated immunoglobulin E levels, eczema, and infections. Its clinical course manifests as recurrent skin and pulmonary infections, and variable skeletal, connective tissue, and vascular abnormalities. There is evidence of abnormal tissue remodeling with pneumatocoeles frequently complicating pyogenic pneumonias and leading to secondary infections that cause the majority of morbidity and mortality. Complications are known to occur after lung surgery with a high frequency of bronchopleural fistulae, but little has been reported concerning abdominal surgeries.

Discussion

Here, we report on the outcome and safety of two separate complex cases (hepatectomy and subtotal gastrectomy) and document our entire experience with abdominal surgical procedures performed on patients with HIES. Despite initial complications, all patients eventually made a full recovery.

Conclusion

As HIES patients now frequently live beyond the third and fourth decade, surgical issues similar to those in the general population may increase. Complex surgical procedures can be performed safely and benefit select patients with HIES, but benefit strongly from multidisciplinary teams and awareness of complications related to abnormal healing. We discuss current treatment and potential complications post-operatively in patients with HIES.

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References

  1. Heimall J, Freeman A, Holland SM. 2010. Pathogenesis of hyper IgE syndrome. Clin Rev Allergy Immunol 38: 32–8

    Article  PubMed  CAS  Google Scholar 

  2. Freeman AF, Holland SM. 2010. Clinical manifestations of hyper IgE syndromes. Dis Markers 29: 123–30

    PubMed  Google Scholar 

  3. Woellner C, Gertz EM, Schaffer AA, Lagos M, Perro M, Glocker EO, Pietrogrande MC, Cossu F, Franco JL, Matamoros N, Pietrucha B, Heropolitanska-Pliszka E, Yeganeh M, Moin M, Espanol T, Ehl S, Gennery AR, Abinun M, Breborowicz A, Niehues T, Kilic SS, Junker A, Turvey SE, Plebani A, Sanchez B, Garty BZ, Pignata C, Cancrini C, Litzman J, Sanal O, Baumann U, Bacchetta R, Hsu AP, Davis JN, Hammarstrom L, Davies EG, Eren E, Arkwright PD, Moilanen JS, Viemann D, Khan S, Marodi L, Cant AJ, Freeman AF, Puck JM, Holland SM, Grimbacher B. 2010. Mutations in STAT3 and diagnostic guidelines for hyper-IgE syndrome. J Allergy Clin Immunol 125: 424–32e8

    Article  PubMed  CAS  Google Scholar 

  4. Heimall J, Davis J, Shaw PA, Hsu AP, Gu W, Welch P, Holland SM, Freeman AF. 2011. Paucity of genotype-phenotype correlations in STAT3 mutation positive Hyper IgE Syndrome (HIES). Clinical Immunology 139: 75–84

    Article  PubMed  CAS  Google Scholar 

  5. Stover DG, Freeman AF, Wright PW, Hummell DS, Ness RM. 2010. Diverticulitis in a young man with hyper-IgE syndrome. South Med J 103: 1261–3

    Article  PubMed  Google Scholar 

  6. Sowerwine KJ, Holland SM, Freeman AF. 2012. Hyper-IgE syndrome update. Ann N Y Acad Sci 1250: 25–32

    Article  PubMed  CAS  Google Scholar 

  7. Milner JD, Brenchley JM, Laurence A, Freeman AF, Hill BJ, Elias KM, Kanno Y, Spalding C, Elloumi HZ, Paulson ML, Davis J, Hsu A, Asher AI, O’Shea J, Holland SM, Paul WE, Douek DC. 2008. Impaired T(H)17 cell differentiation in subjects with autosomal dominant hyper-IgE syndrome. Nature 452: 773–6

    Article  PubMed  CAS  Google Scholar 

  8. Sekhsaria V, Dodd LE, Hsu AP, Heimall JR, Freeman AF, Ding L, Holland SM, Uzel G. 2011. Plasma metalloproteinase levels are dysregulated in signal transducer and activator of transcription 3 mutated hyper-IgE syndrome. J Allergy Clin Immunol 128: 1124–7

    Article  PubMed  CAS  Google Scholar 

  9. Grimbacher B, Schaffer AA, Holland SM, Davis J, Gallin JI, Malech HL, Atkinson TP, Belohradsky BH, Buckley RH, Cossu F, Espanol T, Garty BZ, Matamoros N, Myers LA, Nelson RP, Ochs HD, Renner ED, Wellinghausen N, Puck JM. 1999. Genetic linkage of hyper-IgE syndrome to chromosome 4. Am J Hum Genet 65: 735–44

    Article  PubMed  CAS  Google Scholar 

  10. Goussetis E, Peristeri I, Kitra V, Traeger-Synodinos J, Theodosaki M, Psarra K, Kanariou M, Tzortzatou-Stathopoulou F, Petrakou E, Fylaktou I, Kanavakis E, Graphakos S. 2010. Successful long-term immunologic reconstitution by allogeneic hematopoietic stem cell transplantation cures patients with autosomal dominant hyper-IgE syndrome. J Allergy Clin Immunol 126: 392–4

    Article  PubMed  Google Scholar 

  11. King CL, Gallin JI, Malech HL, Abramson SL, Nutman TB. 1989. Regulation of immunoglobulin production in hyperimmunoglobulin E recurrent-infection syndrome by interferon gamma. Proc Natl Acad Sci U S A 86: 10085–9

    Article  PubMed  CAS  Google Scholar 

  12. Freeman AF, Kleiner DE, Nadiminti H, Davis J, Quezado M, Anderson V, Puck JM, Holland SM. 2007. Causes of death in hyper-IgE syndrome. J Allergy Clin Immunol 119: 1234–40

    Article  PubMed  CAS  Google Scholar 

  13. Davis SD, Schaller J, Wedgwood RJ. 1966. Job’s Syndrome. Recurrent, “cold”, staphylococcal abscesses. Lancet 1: 1013–5

    Article  PubMed  CAS  Google Scholar 

  14. Kida H, Mucenski ML, Thitoff AR, Le Cras TD, Park KS, Ikegami M, Muller W, Whitsett JA. 2008. GP130-STAT3 regulates epithelial cell migration and is required for repair of the bronchiolar epithelium. Am J Pathol 172: 1542–54

    Article  PubMed  CAS  Google Scholar 

  15. Pickert G, Neufert C, Leppkes M, Zheng Y, Wittkopf N, Warntjen M, Lehr HA, Hirth S, Weigmann B, Wirtz S, Ouyang W, Neurath MF, Becker C. 2009. STAT3 links IL-22 signaling in intestinal epithelial cells to mucosal wound healing. J Exp Med 206: 1465–72

    Article  PubMed  CAS  Google Scholar 

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Contributing Author Declaration

We certify that all individuals who qualify as authors have been listed; each has participated in one or more of the following areas: conception and design of this work, the acquisition and/or analysis of data, the writing, and/or critical revision of the document, and supervision of this cooperative research effort. All contributing authors approve of the submission of this version of the manuscript and assert that the document represents valid work. If information derived from another source was used in this manuscript, we obtained all necessary approvals to use it and made appropriate acknowledgements in the document. All contributing authors take public responsibility for this work.

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The views expressed in this manuscript are those of the authors and do not reflect the official policy of the United States Government.

Funding

This study was supported by the NIH intramural grant.

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Authors and Affiliations

  1. Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA

    Russell C. Langan, Richard M. Sherry & Udo Rudloff

  2. Department of Surgery, Georgetown University Hospital, 3800 Reservoir Road, NW 2 GOR, Washington, DC, 20007, USA

    Russell C. Langan

  3. Bon Secours Cancer Institute, Richmond, VA, USA

    Itzhak Avital

  4. National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA

    Theo Heller

  5. National Institute of Allergy and Infectious Disease, National Institutes of Health, CCR, Room 11 N234, 10 Center Drive, Bethesda, MD, 20892-0001, USA

    Carolyn Henderson, Steven M. Holland & Alexandra F. Freeman

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  1. Russell C. Langan
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  2. Richard M. Sherry
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  3. Itzhak Avital
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  6. Steven M. Holland
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  8. Udo Rudloff
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Correspondence to Russell C. Langan or Alexandra F. Freeman.

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Langan, R.C., Sherry, R.M., Avital, I. et al. Safety of Major Abdominal Surgical Procedures in Patients with Hyperimmunoglobulinemia E (Job’s Syndrome): a Changing Paradigm?. J Gastrointest Surg 17, 1009–1014 (2013). https://doi.org/10.1007/s11605-012-2077-1

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  • DOI: https://doi.org/10.1007/s11605-012-2077-1

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