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生长激素治疗期间,一名患有严重肥厚型心肌病的努南综合征患者出现进行性左心室流出道狭窄。

Progressive Left Ventricular Outflow Tract Stenosis in a Noonan Syndrome Patient With Severe Hypertrophic Cardiomyopathy During Growth Hormone Treatment.

作者信息

Ichikawa Yasuhiro, Saito Naka, Kurosawa Kenji, Hanakawa Junko, Ueda Hideaki

机构信息

Cardiology, Kanagawa Children's Medical Center, Yokohama, JPN.

Clinical Laboratory, Kanagawa Children's Medical Center, Yokohama, JPN.

出版信息

Cureus. 2022 Apr 7;14(4):e23937. doi: 10.7759/cureus.23937. eCollection 2022 Apr.

Abstract

Short stature is a main problem in Noonan syndrome (NS). Recombinant human growth hormone (GH) has been used to safely improve the growth rate in NS patients with short stature. However, there is little information about GH therapy for NS associated with hypertrophic obstructive cardiomyopathy. We present the case of a seven-year-old NS patient with severe hypertrophic obstructive cardiomyopathy. The patient received GH therapy for six months, at which time progressive left ventricular outflow tract stenosis was apparent.

摘要

身材矮小是努南综合征(NS)的一个主要问题。重组人生长激素(GH)已被用于安全提高身材矮小的NS患者的生长速度。然而,关于GH治疗与肥厚性梗阻性心肌病相关的NS的信息很少。我们报告了一例患有严重肥厚性梗阻性心肌病的7岁NS患者的病例。该患者接受了6个月的GH治疗,此时出现了进行性左心室流出道狭窄。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad62/9079321/d6df320633b0/cureus-0014-00000023937-i01.jpg

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