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[儿童特发性肺含铁血黄素沉着症免疫抑制治疗的长期评估]

[Long-term evaluation of immunosuppressive therapy in childhood idiopathic pulmonary hemosiderosis].

作者信息

Bagnato L, Grilli C, Portioli P, Biella C, Carnelli V

出版信息

Pediatr Med Chir. 1986 Sep-Oct;8(5):671-4.

PMID:3601698
Abstract

Idiopathic pulmonary hemosiderosis (IPM) is a rare disease of unknown etiology, whose diagnostic, prognostic and therapeutic approach is still open to discussion. In this paper the authors report a study regarding three cases of IPH initially detected in 13, 11 and 7 year-old children. The patients were treated with cyclophosphamide and prednisone according to different cycles depending on the clinical stage in the disease. All three patients are still alive after 10, 6 and 5 years since initial diagnosis. This therapeutic protocol therefore seems to be effective in preventing the progression of IPH and in maintaining the patients in an asymptomatic condition.

摘要

特发性肺含铁血黄素沉着症(IPM)是一种病因不明的罕见疾病,其诊断、预后和治疗方法仍有待探讨。在本文中,作者报告了一项针对三例分别于13岁、11岁和7岁儿童首次发现的IPH病例的研究。根据疾病的临床阶段,患者接受了不同疗程的环磷酰胺和泼尼松治疗。自初次诊断以来,10年、6年和5年后,所有三名患者均存活。因此,该治疗方案似乎在预防IPH进展和使患者保持无症状状态方面有效。

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