Anestesia em paciente com Síndrome de Jó (hiper IgE): Relato de caso

Marco Antônio Cardoso de Resende; Alberto Vieira Pantoja; Ilda Antunes Lopes Maldonado Charruff; Nisval de Magalhães Júnior; Priscilla Luz

doi:10.1590/S0034-70942011000100009

Clinical Information

Anestesia em paciente com Síndrome de Jó (hiper IgE): Relato de caso

Anesthesia in a patient with Job's Syndrome (hyper IgE): Case report

Marco Antônio Cardoso de Resende; Alberto Vieira Pantoja; Ilda Antunes Lopes Maldonado Charruff; Nisval de Magalhães Júnior; Priscilla Luz

http://dx.doi.org/10.1590/S0034-70942011000100009 Braz J Anesthesiol, vol.61, n1, p.84-87, 2011

PDF

Downloads: 0

Resumo

JUSTIFICATIVA E OBJETIVOS: A síndrome de Jó (SJ) é imunodeficiência rara, uma das formas de apresentação da Síndrome hiper IgE. O quadro clínico compreende abscessos cutâneos, pneumonias de repetição, pneumatoceles, eosinofilia, hiperimunoglobulinemia E (> 2.000 UI.mL-1), alterações craniofaciais e de crescimento ósseo. O relato descreve a doença e seu manuseio anestésico. RELATO DO CASO: Paciente masculino, negro, 13 anos, 40 kg, ASA II, com Síndrome de Jó diagnosticada aos 6 meses. Foi admitido para realização de alongamento de fêmur direito. Negava uso de medicamentos e não tinha antecedentes cirúrgicos, boa mobilidade cervical, distância interincisivos superior a 3 cm, Mallampati II e sem sinais de infecção. Os exames pré-operatórios eram normais. Foi monitorado com eletrocardioscópio, SpO2, PANI e P ET CO2. Após pré-oxigenação, realizou-se indução de anestesia geral venosa e manutenção com sevoflurano. Ao término do procedimento, o paciente foi extubado após reversão do bloqueio neuromuscular e encaminhado para a sala de RPA com Aldrete 9. Teve alta hospitalar após 72 horas, sem complicações. CONCLUSÕES: A opção da técnica anestésica é orientada pela observação criteriosa entre os riscos e benefícios específicos para cada paciente, de acordo com as sequelas respiratórias e o risco de infecção e sítio cirúrgico. No paciente em questão, havia a consideração de que a anestesia em neuroeixo poderia representar, pela predisposição de base imunológica, risco aumentado de infecções graves. O presente caso foi realizado de forma segura com anestesia geral.

Palavras-chave

DOENÇAS, Genética, TÉCNICAS ANESTÉSICAS, Geral

Abstract

BACKGROUND AND OBJECTIVES: Job's syndrome (JS), one of the presentations of the Hyper IgE Syndrome, is a rare immunodeficiency. It includes cutaneous abscesses, recurring pneumonias, pneumatoceles, eosinophilia, hyperimmunoglobulinemia E (> 2,000 IU.mL-1), and craniofacial and bone growth changes. This report describes the disease and its anesthetic management. CASE REPORT: The patient is a 13 year old black male, 40 kg, ASA II, with Job's Syndrome diagnosed 6 months prior to this admission. The patient was admitted for elongation of the right femur. He denied use of drugs and prior surgeries; he presented good cervical mobility, interincisive distance greater than 3 cm, Mallampati II, without signs of infection. Preoperative exams were within normal limits. He was monitored with electrocardioscope, SpO2, non-invasive blood pressure, and P ET CO2. After pre-oxygenation, general anesthesia was induced and he was maintained with sevoflurane. At the end of the procedure, the patient was extubated after reversal of the neuromuscular blockade, and the patient was transferred to the PACU with Aldrete 9, He was discharged from the hospital 72 hours later, without complications. CONCLUSIONS: The choice of anesthetic technique is guided by rigorous observation among risks and benefits for each patient, according to respiratory sequelae, risk of infection, and surgical site. In the patient described here, we considered that neuroaxis anesthesia could be associated with an increased risk of severe infections due to the patient immunologic background. The procedure was safely performed with general anesthesia.

Keywords

Job's Syndrome, Anesthesia, General, Bone Lengthening

References

Freeman AF, Holland SM. The hyper-IgE syndromes. Immunol Allergy Clin North Am. 2008;28:277-291.

Davis SD, Schaller J, Wedgwood RJ. Job's Syndrome: Recurrent, "cold", staphylococcal abscesses. Lancet. 1966;1:1013-1015.

Buckley RH, Wray BB, Belmaker EZ. Extreme hyperimmunoglobulinemia E and undue susceptibility to infection. Pediatrics. 1972;49:59-70.

Orozco CV, Velasquez LH, Mendez NH. Sindrome de hiper IgE. Diagnostico y manejo oportunos. Rev Alerg Mex. 2008;55:38-45.

Bosenberg AT. Anaesthesia and Job syndrome. South Afr J Anaesth Analg. 2008;14:11-14.

Holland SM, DeLeo FR, Elloumi HZ. STAT3 mutations in the hyper-IgE syndrome. N Engl J Med. 2007;357:1608-1619.

Minegishi Y, Saito M, Tsuchiya S. Dominant-negative mutations in the DNA-binding domain of STAT3 cause hyper-IgE syndrome. Nature. 2007;448:1058-1062.

Murray PJ. The JAK-STAT signaling pathway: input and output integration. J Immunol. 2007;178:2623-2629.

Zhang Z, Welte T, Troiano N. Osteoporosis with increased osteoclastogenesis in hematopoietic cell-specific STAT3-deficient mice. Biochem Biophys Res Commun. 2005;328:800-807.

Borges WG, Hensley T, Carey JC. The face of Job. J Pediatr. 1998;133:303-305.

Domingo DL, Freeman AF, Davis J. Novel intraoral phenotypes in hyperimmunoglobulin-E syndrome. Oral Dis. 2008;14:73-81.

Freeman AF, Domingo DL, Holland SM. Hyper IgE (Job's) syndrome: a primary immune deficiency with oral manifestations. Oral Dis. 2009;15:2-7.

Freeman AF, Kleiner DE, Nadiminti H. Causes of death in hyper-IgE syndrome. J Allergy Clin Immunol. 2007;119:1234-1240.

Leonard GD, Posadas E, Herrmann PC. Non-Hodgkins lymphoma in Job's syndrome: a case report and literature review. Leuk Lymphoma. 2004;45:2521-2525.

Freeman AF, Holland SM. Clinical manifestations, etiology, and pathogenesis of the hyper-IgE syndromes. Pediatr Res. 2009;65:32R-37R.

Kimata H. High-dose intravenous gamma-globulin treatment for hyperimmunoglobulinemia E syndrome. J Allergy Clin Immunol. 1995;95:771-774.

King CL, Gallin JI, Malech HL. Regulation of immunoglobulin production in hyperimmunoglobulin E recurrent-infection syndrome by interferon gamma. Proc Natl Acad Sci USA. 1989;86:10085-10089.

Miller FL, Mann DL. Anesthetic management of a pregnant patient with the hyperimmunoglobulin E (Job's) syndrome. Anesth Analg. 1990;70:454-456.

Tapper JB, Giesecke AH. Spinal anaesthesia in a child with Job's syndrome, pneumatoceles and empyema. Anaesthesia. 1990;45:378-380.

Guzzi LM, Stamatos JM. Job's syndrome: an unusual response to a common drug. Anesth Analg. 1992;75:139-140.

Anestesia em paciente com Síndrome de Jó (hiper IgE): Relato de caso

Anesthesia in a patient with Job's Syndrome (hyper IgE): Case report

Marco Antônio Cardoso de Resende; Alberto Vieira Pantoja; Ilda Antunes Lopes Maldonado Charruff; Nisval de Magalhães Júnior; Priscilla Luz

Resumo

Palavras-chave

Abstract

Keywords

References

Links

Share

Braz J Anesthesiol