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Anesth Essays Res. 2012 Jul-Dec;6(2):223-5. doi: 10.4103/0259-1162.108342.

Anesthetic management in a child with Job's syndrome.

Anesthesia, essays and researches

Prasad Kulkarni, Riddhi Shah, V N Priyanka

Affiliations

  1. Department of Anesthesia, MVJ Medical College and Research Hospital, Affiliated to Rajiv Gandhi University of Health and Sciences, Bangalore, India.

PMID: 25885624 PMCID: PMC4173468 DOI: 10.4103/0259-1162.108342

Abstract

Job's syndrome also known as hyper-IgE syndrome is a rare disorder associated with immunodeficiency. It includes recurrent abscesses of staphylococcal origin affecting skin and musculoskeletal system, recurring pneumonia and pneumatoceles, eosinophilia, craniofacial, and bone growth changes. This case report describes the disease and anesthetic management. Ten-year-old male child weighing 18 kg was diagnosed recently as Job's syndrome and was posted for abscess drainage over chest, back, upper, and lower limb. He had associated severe mental retardation. The choice of anesthetic technique was based on multiple surgical sites and associated mental retardation. Due to associated mental retardation child was sedated under our vigilance with syrup midazolam 9 mg orally and EMLA cream applied with occlusive dressing 30 min prior to shifting to OT. Upon arrival to the OT, pulse oximetry, noninvasive blood pressure, Electrocardiogram, temperature probe, cardioscope were attached. Inhalation induction done with sevoflurane and IV line secured with 22G cannula. Glycopyrolate 0.1 mg + fentanyl 30 mcg + Propofol 30 mg given intravenously. General anesthesia was maintained with bag and mask ventilation with oxygen + nitrous oxide + sevoflurane with spontaneous ventilation. Postoperative period was uneventful. This case is presented in view of rarity of its occurrence and frequency of repeated surgical intervention and anesthesia. Associated mental retardation in this case could be an isolated incident or could be an occurrence related to underlying disease process itself.

Keywords: Hyper IgE; job's syndrome; recurrent staphylococcal infections

References

  1. Lancet. 1966 May 7;1(7445):1013-5 - PubMed
  2. J Pediatr. 1998 Aug;133(2):303-5 - PubMed
  3. Pediatr Res. 2009 May;65(5 Pt 2):32R-37R - PubMed
  4. J Allergy Clin Immunol. 2007 May;119(5):1234-40 - PubMed
  5. Biochem Biophys Res Commun. 2005 Mar 18;328(3):800-7 - PubMed
  6. J Allergy Clin Immunol. 1995 Mar;95(3):771-4 - PubMed
  7. Oral Dis. 2008 Jan;14(1):73-81 - PubMed
  8. Oral Dis. 2009 Jan;15(1):2-7 - PubMed
  9. Immunol Allergy Clin North Am. 2008 May;28(2):277-91, viii - PubMed
  10. Can J Anaesth. 1992 Jul;39(6):545-50 - PubMed
  11. Leuk Lymphoma. 2004 Dec;45(12):2521-5 - PubMed
  12. Anaesthesia. 1990 May;45(5):378-80 - PubMed
  13. N Engl J Med. 2007 Oct 18;357(16):1608-19 - PubMed
  14. Rev Alerg Mex. 2008 Jan-Feb;55(1):38-45 - PubMed
  15. J Immunol. 2007 Mar 1;178(5):2623-9 - PubMed

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