产前单侧上肢棘突发育不良。

IF 1.3 Q3 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING Journal of Ultrasound Pub Date : 2024-09-17 DOI:10.1007/s40477-024-00954-0
Shruti Thakur, Charu Smita Thakur, Anupam Jhobta, Vikrant Negi
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引用次数: 0

摘要

短肢侏儒症(Acromesomelic dysplasia,AMD)是一组进行性骨骼疾病的总称,其特征是短肢侏儒症,上肢和下肢的中段和远段不成比例地缩短。虽然特定的骨骼畸形很难在产前诊断,但由于其对产前和产后的影响,以及有可能在以后的妊娠中再次发生,因此需要积极应对此类骨骼畸形。要对特定的先天性骨骼发育不良进行分类,需要结合产前和产后的放射学、病理学、遗传学和分子学调查。一旦胎儿骨骼发育不良的基因组成被破解,就可以为受影响家庭的未来妊娠提供有意义的遗传咨询。我们描述了一例初产妇的病例,她在怀孕后三个月早期进行产前超声检查时被确诊为胎儿单侧上肢 AMD。左上肢的桡骨和尺骨异常短小(小于该孕龄平均值的第 5 百分位数)。左手也发育不良。其他声像异常扫描结果正常。据我们所知,迄今为止,医学文献中还没有关于产前诊断出单侧上肢AMD的单独病例。
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Antenatal unilateral upper limb acromesomelic dysplasia.

Acromesomelic dysplasia (AMD) is an umbrella term given to a heterogeneous group of progressive skeletal disorders characterized by short limbed dwarfism associated with disproportionate shortening of middle and distal segments of the upper as well as lower limbs. Although specific skeletal anomalies are difficult to diagnose antenatally, but because of their antenatal and postnatal implications and a possibility of reoccurrence in following pregnancies, such skeletal anomalies need to be actively addressed. A combination of radiologic, pathologic, genetic and molecular investigation prenatally as well as postnatally is required to classify a specific congenital skeletal dysplasia. Once the genetic make-up of fetal skeletal dysplasia is deciphered, a meaningful genetic counselling could be offered for future pregnancies of affected families. We describe a case of primigravida diagnosed with fetal unilateral upper limb AMD on antenatal ultrasound done at early second trimester. The radius and ulna of left upper limb were abnormally short (less than 5th centile of the mean for that gestational age). The left hand was also hypoplastic. Rest of the sonographic anomaly scan was normal. To the best of our knowledge, AMD limited to unilateral upper limb diagnosed antenatally as an isolated finding is not described in the medical literature so far.

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来源期刊
Journal of Ultrasound
Journal of Ultrasound RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING-
CiteScore
4.10
自引率
15.00%
发文量
133
期刊介绍: The Journal of Ultrasound is the official journal of the Italian Society for Ultrasound in Medicine and Biology (SIUMB). The journal publishes original contributions (research and review articles, case reports, technical reports and letters to the editor) on significant advances in clinical diagnostic, interventional and therapeutic applications, clinical techniques, the physics, engineering and technology of ultrasound in medicine and biology, and in cross-sectional diagnostic imaging. The official language of Journal of Ultrasound is English.
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