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先天性红细胞生成性卟啉病(京特氏病)与氯喹(作者译)

[Porphyria erythropoietica congenita Günther and chloroquine (author's transl)].

作者信息

Ippen H, Tillmann W, Seubert S, Seubert A

出版信息

Klin Wochenschr. 1978 Jun 15;56(12):623-4. doi: 10.1007/BF01477011.

Abstract

Treatment of a six-year-old boy with porphyria congenita (Günther) by small amounts of chloroquine was followed by a sharp but transient increase of the urinary excretion of porphyrins. Moreover, a nearly complete normalization of the previously observed rigidity of the erythrocytes occurred. With respect to the possibility that this elevated rigidity plays an important role for the typical hemolysis connected with this porphyria, the application of chloroquine could be of therapeutic value for this disease.

摘要

用小剂量氯喹治疗一名六岁的先天性卟啉病(京特病)男孩后,卟啉的尿排泄量急剧但短暂增加。此外,先前观察到的红细胞刚性几乎完全恢复正常。鉴于这种升高的刚性可能对与这种卟啉病相关的典型溶血起重要作用,氯喹的应用可能对这种疾病具有治疗价值。

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