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9p 三体嵌合体程度高,但除了染色体平衡精子的中度少精症外无其他临床表现:一例报告。

A high level of tetrasomy 9p mosaicism but no clinical manifestations other than moderate oligozoospermia with chromosomally balanced sperm: a case report.

机构信息

Université Paris-Saclay, INRAE, ENVA, UVSQ, BREED, 78350, Jouy-en-Josas, France.

Genetics Federation, CHI de Poissy St Germain en Laye, F-78303, Poissy, France.

出版信息

J Assist Reprod Genet. 2020 Mar;37(3):573-577. doi: 10.1007/s10815-020-01690-0. Epub 2020 Jan 24.

Abstract

Tetrasomy 9p (ORPHA: 3310) (i(9p)) is a rare chromosomal imbalance. It is characterized by the presence of a supernumerary chromosome incorporating two copies of the short arm of chromosome 9 and is usually present in a mosaic state postnatally. Depending on the level of mosaicism, the phenotype ranges from mild developmental delay to multiple congenital anomalies with severe intellectual disability. Here, we report on a patient diagnosed with i(9p) mosaicism after the recurrent failure of in vitro fertilization. Although the patient's clinical phenotype was normal, the level of mosaicism varied greatly from one tissue to another. A sperm analysis evidenced subnormal spermatogenesis with chromosomally balanced spermatozoa and no risk of transmission to the offspring. Although individuals with i(9p) and no clinical manifestations have rarely been described, the prenatal diagnosis of this abnormality in the absence of ultrasound findings raises a number of questions.

摘要

9p 三体性(ORPHA:3310)(i(9p))是一种罕见的染色体不平衡。它的特征是存在一条额外的染色体,包含两条 9 号染色体的短臂,并且通常在出生后呈嵌合体状态。根据嵌合体的程度,表型范围从轻度发育迟缓到伴有严重智力残疾的多种先天性异常。在这里,我们报告了一例在体外受精反复失败后诊断为 i(9p)嵌合体的患者。尽管患者的临床表型正常,但嵌合体的程度在不同组织之间差异很大。精子分析显示精子发生异常,存在染色体平衡的精子,并且没有向后代传播的风险。虽然已经很少有描述没有临床表现的 i(9p)个体,但在没有超声发现的情况下对这种异常进行产前诊断提出了许多问题。

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本文引用的文献

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Heteromorphic variants of chromosome 9.9号染色体的异形变体
Mol Cytogenet. 2013 Apr 1;6(1):14. doi: 10.1186/1755-8166-6-14.

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