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[单基因和染色体综合征中骨骼发育异常的遗传咨询与超声产前诊断]

[Genetic counseling and ultrasonographic prenatal diagnosis of skeletal dysplasias in monogenic and chromosomal syndromes].

作者信息

Veropotvelian N P, Veropotvelian P N, Tomashevskaia N M, Klipova L V, Nikitenko M K, Khan' I E, Kodunov L A

出版信息

Akush Ginekol (Mosk). 1990 Nov(11):20-6.

PMID:2077906
Abstract

The paper reports sonographic prenatal diagnosis of monogenic and chromosomal syndromes (congenital malformations) which are associated with skeletal abnormalities: autosomal recessive syndromes (shortribs--polydactyly, type II; dyssegmental dwarfism, type II; Pen-Shokeir disease type I), autosomal dominant syndromes (Larsen's syndrome, achondroplasia, Schmidt's metaphyseal dysplasia of segregating partial chromosome of 7 long-arm trisomy). All cases were detected in the second trimester; diagnosis of chromosomal diseases employed the amniocentesis. In aborted pregnancies, diagnoses were confirmed at autopsy. Pathognomonic sonographic markers were described for every syndrome; numerous conditions were differentiated from similar syndromes. Genetic counseling identified recurrence risks and offspring prognosis. Potentials of sonographic diagnosis of systemic skeletal dysplasia and a need for a syndrome-oriented approach to prenatal diagnosis are discussed.

摘要

本文报道了与骨骼异常相关的单基因和染色体综合征(先天性畸形)的超声产前诊断:常染色体隐性综合征(短肋多指综合征II型;节段性侏儒症II型;Pen-Shokeir病I型),常染色体显性综合征(拉森综合征、软骨发育不全、7号染色体长臂部分三体分离的施密特干骺端发育异常)。所有病例均在孕中期检测到;染色体疾病的诊断采用羊膜穿刺术。在流产的妊娠中,诊断在尸检时得到证实。描述了每种综合征的特征性超声标志物;许多情况与相似综合征进行了鉴别。遗传咨询确定了复发风险和子代预后。讨论了超声诊断全身性骨骼发育不良的潜力以及产前诊断采用综合征导向方法的必要性。

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