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[关于一例罕见的再生障碍性贫血病例(作者译)]

[On an unusual case of hypoplastic anaemia (author's transl)].

作者信息

Panoff A, Milanoff G

出版信息

Monatsschr Kinderheilkd (1902). 1977 Jun;125(6):671-3.

PMID:882107
Abstract

The authors describe an unusual case of hypoplastic anaemia. The condition developed shortly after birth as a typical case of Josephs-Blackfan Diamond and was favourably influenced only by a combined treatment with Prednison and Dianabol. The improvement, however, did not last long and after shorter or longer periods of time anaemia recurred again. Applying the same therapy the authors succeeded, despite the sometimes grave condition of the child, not only to save his life but also to normalize the erythrocyte count. It must be noted that the child showed viability for a long period of time no matter how pronounced the anaemia was. Thus, therapy was not necessary to be repeated often. Of special interest for this case were bone marrow examinations. During the first year of the child's life only 1 to 4 erythroblasts were seen the examination was performed several times. After the sixth year the bone marrow contained many erythroblasts although the type of anaemia remained unchanged. According to the authors this peculiarity could be explained only as a transition from a hypoplastic into a pseudohypoplastic anaemia.

摘要

作者描述了一例罕见的再生障碍性贫血病例。该病在出生后不久即发展为典型的先天性纯红细胞再生障碍性贫血,仅泼尼松和大力补联合治疗对其有积极影响。然而,病情改善并未持续太久,经过或长或短的一段时间后,贫血再次复发。尽管患儿有时病情严重,但应用相同疗法,作者不仅成功挽救了他的生命,还使红细胞计数恢复正常。必须指出的是,无论贫血多么严重,患儿都存活了很长时间。因此,无需频繁重复治疗。该病例特别值得关注的是骨髓检查。在患儿生命的第一年,尽管多次进行检查,但每次仅见1至4个成红细胞。到第六年时,尽管贫血类型未变,但骨髓中含有许多成红细胞。作者认为,这种特殊情况只能解释为从再生障碍性贫血转变为假性再生障碍性贫血。

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