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儿童淋巴细胞白血病中形态发生轻度错误过多。

Excess of mild errors of morphogenesis in childhood lymphoblastic leukemia.

作者信息

Méhes K, Kajtár P, Sándor G, Scheel-Walter M, Niethammer D

机构信息

Department of Pediatrics, University Medical School of Pécs, Hungary.

出版信息

Am J Med Genet. 1998 Jan 6;75(1):22-7.

PMID:9450852
Abstract

The prevalence of 55 well-defined mild errors of morphogenesis (MEMs) was determined in 100 children with acute lymphoblastic leukemia (ALL), their 80 sibs, 91 mothers, and 76 fathers. Seventy-four patients were treated in Pécs (Hungary) and 26 in Tübingen (Germany). Only white Caucasian index cases were included in the study. Two-hundred children examined for acute infections served as controls. In addition, we analyzed the family history for birth defects and malignancies, associated major malformations, birth weight, birth order, and pretreatment height of the patients. The results of the Pécs and Tübingen patients were at first evaluated separately but since no differences were found only the cumulative data were analyzed further. A significantly increased prevalence of MEMs was found in the ALL patients and their sibs of both sexes: their MEM/subject ratios were 1.59 and 1.51, respectively, whereas the same parameter was 0.74 in the mothers, 0.67 in the fathers and 0.69 in the controls. The same tendency was observed when familial cases and/or age-dependent MEMs were excluded and when malformation-type and variant-type MEMs were evaluated separately. No association of ALL with specific MEMs or combinations was recorded. Family history, associated major malformations, parity and birth weight of the patients did not differ significantly from the local reference values, whereas the pretreatment height of the male probands proved to be greater than expected.

摘要

在100例急性淋巴细胞白血病(ALL)患儿、他们的80名同胞、91名母亲和76名父亲中,确定了55种明确界定的轻度形态发生异常(MEMs)的患病率。74例患者在匈牙利佩奇接受治疗,26例在德国图宾根接受治疗。该研究仅纳入白种人索引病例。200名接受急性感染检查的儿童作为对照。此外,我们分析了患者的出生缺陷和恶性肿瘤家族史、相关的主要畸形、出生体重、出生顺序以及预处理前身高。起初分别评估了佩奇和图宾根患者的结果,但由于未发现差异,因此仅对累积数据进行了进一步分析。在ALL患者及其同胞中,无论男女,MEMs的患病率均显著增加:他们的MEM/个体比率分别为1.59和1.51,而母亲中的同一参数为0.74,父亲中为0.67,对照组中为0.69。当排除家族性病例和/或年龄依赖性MEMs,以及分别评估畸形类型和变异类型的MEMs时,观察到相同的趋势。未记录到ALL与特定MEMs或组合之间的关联。患者的家族史、相关主要畸形、产次和出生体重与当地参考值无显著差异,而男性先证者的预处理前身高高于预期。

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