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[副球孢子菌病:一种被忽视疾病的纪事]

[Paracoccidioidomycosis: chronicle of a neglected disease].

作者信息

Canteros Cristina E

机构信息

Servicio Micosis Profundas, Departamento Micología, INEI-ANLIS Dr. Carlos G. Malbrán, Buenos Aires, Argentina. E-mail

出版信息

Medicina (B Aires). 2018;78(3):180-184.

PMID:29940544
Abstract

Paracoccidioidomycosis (PCM) is among the systemic mycoses which are endemic only in Latin America. In Argentina, the vast majority of the cases are reported at north of latitude 34.5° S. The disease is produced by thermodimorphic fungi of the genus Paracoccidoides: P. brasiliensis (S1), P. americana (PS2), P. restrepiensis (PS3), P. venezuelensis (PS4) y P. lutzii. The natural habitat of members of this genus is the soil, where they produce infectious conidia. Little is known, however, about their specific ecologic niche(s), and this knowledge gap hampers the design of measures to control the infection. Rural male workers are the group most at risk of developing PCM. Infection occurs by inhalation of aerosolized conidia and may either be asymptomatic or cause mild respiratory symptoms. In turn, this primary infection may be self-limited or progress to severe pulmonary or disseminated disease. The disease has two clinical presentations: (i) acute or subacute (juvenile), frequent in children, adolescents and people with immunodeficiencies; and (ii) chronic progressive, in adults. Active lesions often resolve into fibrotic scars which can cause dysphagia, dysphonia, adrenal insufficiency, and intestinal obstruction. Although efficient tools are available for diagnosis and treatment, the nonspecific nature of PCM clinical manifestations frequently delay the diagnosis. In addition, the poor adherence to long antifungal treatments allows the advance of the disease and the development of extensive fibrosis compromising severely and permanently respiratory and adrenal functions, thus altering the patient"s quality of life and even causing his/her death.

摘要

副球孢子菌病(PCM)是仅在拉丁美洲流行的系统性真菌病之一。在阿根廷,绝大多数病例报告于南纬34.5°以北地区。该疾病由副球孢子菌属的嗜温双相真菌引起:巴西副球孢子菌(S1)、美洲副球孢子菌(PS2)、雷斯垂皮副球孢子菌(PS3)、委内瑞拉副球孢子菌(PS4)和卢氏副球孢子菌。该属成员的自然栖息地是土壤,它们在那里产生具有传染性的分生孢子。然而,对于它们具体的生态位知之甚少,这种知识空白阻碍了控制感染措施的设计。农村男性工人是最易患PCM的群体。感染通过吸入雾化的分生孢子发生,可能无症状或引起轻微呼吸道症状。反过来,这种原发性感染可能是自限性的,也可能进展为严重的肺部或播散性疾病。该疾病有两种临床表现:(i)急性或亚急性(青少年型),常见于儿童、青少年和免疫功能低下者;(ii)慢性进行性,见于成年人。活动性病变常演变为纤维化瘢痕,可导致吞咽困难、发音障碍、肾上腺功能不全和肠梗阻。尽管有有效的诊断和治疗工具,但PCM临床表现的非特异性常常延误诊断。此外,对抗真菌长疗程治疗的依从性差会使疾病进展,导致广泛纤维化,严重且永久性地损害呼吸和肾上腺功能,从而改变患者的生活质量,甚至导致死亡。

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