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急性间歇性卟啉病伴呼吸衰竭:多学科诊疗方法

Acute intermittent porphyria associated with respiratory failure: a multidisciplinary approach.

作者信息

Menegueti Mayra Gonçalves, Gil Cezar Alkmim-Teixeira, Casarini Karin Aparecida, Muniz Cordeiro Kátia Simone, Basile-Filho Anibal, Martins-Filho Olindo Assis, Auxiliadora-Martins Maria

机构信息

Divisão de Terapia Intensiva, Departamento de Cirurgia e Anatomia, Hospital das Clínicas, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, 14049-900 Ribeirão Preto, SP, Brazil.

出版信息

Crit Care Res Pract. 2011;2011:283690. doi: 10.1155/2011/283690. Epub 2011 May 11.

Abstract

Despite being challenging, delivery of effective nursing care to patients with acute intermittent porphyria is a matter of utmost importance. In this paper, the diversity of symptoms and the difficult diagnosis of this condition are emphasized, and details concerning the treatment of this disorder in the intensive care unit are presented. We believe that acute intermittent porphyria should be borne in mind during performance of differential diagnosis of neurological, psychiatric, and gastroenterological disorders on patients whose routine investigation tests are normal, especially when precipitating factors exist. Intensive care measures and a multidisciplinary team approach are essential.

摘要

尽管具有挑战性,但为急性间歇性卟啉病患者提供有效的护理至关重要。本文强调了该病症症状的多样性和诊断困难,并介绍了在重症监护病房对该疾病的治疗细节。我们认为,在对常规检查结果正常的神经、精神和胃肠疾病患者进行鉴别诊断时,应考虑到急性间歇性卟啉病,尤其是存在诱发因素时。重症监护措施和多学科团队方法至关重要。

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本文引用的文献

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Porphyrias.卟啉症。
Lancet. 2010 Mar 13;375(9718):924-37. doi: 10.1016/S0140-6736(09)61925-5.
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Acute intermittent porphyria.急性间歇性卟啉病
Best Pract Res Clin Gastroenterol. 2005 Apr;19(2):235-49. doi: 10.1016/j.bpg.2004.10.006.
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Recommendations for the diagnosis and treatment of the acute porphyrias.急性卟啉病的诊断与治疗建议。
Ann Intern Med. 2005 Mar 15;142(6):439-50. doi: 10.7326/0003-4819-142-6-200503150-00010.
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Porphyrias.卟啉病
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