Villaroel-Vargas Jenny, Molina-Vargas Lucía, Zurita-Leal Andrea, Zavala-Calahorrano Alicia
Facultad de Ciencias de la Salud, Universidad Técnica de Ambato, Ambato, Ecuador.
Medwave. 2020 Feb 26;20(2):e7826. doi: 10.5867/medwave.2020.01.7826.
Noonan syndrome is an autosomal dominant inherited disorder with variable phenotypic expression. It belongs to the group of diseases known as RASopathies, which are characterized by mutations in the RAS genes. Patients develop symptoms such as facial dysmorphism, short stature, congenital heart disease, musculoskeletal disorders and mental retardation. In this article, we report a case of Noonan syndrome in a 14-year-old patient, diagnosed in a primary health center in Ecuador. The syndrome was identified through clinical diagnosis, after which the patient was referred to the secondary and tertiary levels for specialized care.
努南综合征是一种常染色体显性遗传性疾病,具有可变的表型表达。它属于一类被称为RAS病的疾病,其特征是RAS基因突变。患者会出现面部畸形、身材矮小、先天性心脏病、肌肉骨骼疾病和智力障碍等症状。在本文中,我们报告了一名14岁厄瓜多尔患者的努南综合征病例,该病例在一家初级卫生中心被诊断出来。通过临床诊断确定了该综合征,之后患者被转诊至二级和三级医疗机构接受专科护理。