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首页> 外文期刊>Balkan journal of medical genetics: BJMG >SIRENOMELIA ASSOCIATED WITH HYPOPLASTIC LEFT HEART IN A NEWBORN
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SIRENOMELIA ASSOCIATED WITH HYPOPLASTIC LEFT HEART IN A NEWBORN

机译：西先生与强心左心有关的新生儿

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摘要

Sirenomelia, also known as "mermaid malformation/ syndrome," is a rare, serious congenital anomaly characterized by variable degrees of fusion of the lower limbs and associated with severe malformations of vertebral, genitourinary, cardiovascular system and single umbilical artery. The first pregnancy of a 25-year-old woman resulted in one twin born by Cesarian section at 32 weeks' gestation, who was referred to our hospital with cyanosis, a congenital anomaly and respiratory distress. On physical examination, there was no urogenital region and anal fissure and gender was indeterminate. The arms were in adduction and wrist in flexion position with four fingers on the right hand and two fingers on the left hand. There was a single lower extremity with a webbed single foot and two toes consistent with sirenomelia type IV radiologically. Abdominal ultrasonography showed urogenital system agenesis and echocardiography detected hypoplastic left heart. However, the patient died 4 hours after birth. The other twin was followed for 1 week for nutrition and respiratory support and was then discharged without any problems.
机译：西列伦氏素，也被称为“美人鱼畸形/综合征”，是一种罕见的，严重的先天性异常，其特征在于下肢的可变融合程度，与椎体，泌尿病，心血管系统和单脐动脉的严重畸形相关。一名25岁女性的第一次怀孕导致了一个由Cesarian部分出生的一个双胞胎，在妊娠32周的妊娠，他用紫绀，先天性异常和呼吸窘迫。在体检时，没有泌尿生殖器区和肛裂和性别不确定。武器在一条内收集和弯曲位置，右手上有四个手指，左手两根手指。有一个单个下肢，蹼单脚和两个脚趾与sirenomelia型无知的尺寸一致。腹部超声检查显示泌尿生殖系统患者和超声心动图检测到的左心性左心。然而，患者出生后4小时死亡。其他双胞胎随后营养和呼吸载体的1周，然后在没有任何问题的情况下排出。

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