Botnaru Victor, Rusu Doina, Munteanu Oxana
Pneumologia. 2016 Jan-Mar;65(1):39-44.
Williams-Beuren syndrome (WBS) is a rare genetic disease with a distinctive constellation of clinical findings. The disease can be diagnosed clinically by a recognizable pattern of malformations, including cardiovascular malformations, a characteristic facial dysmorphism, as well as neurological and cognitive features. We present the case of a 23-years-old woman repeatedly admitted to Pulmonology Clinic for massive hemoptysis. Diagnosis of Williams-Beuren syndrome was revealed by clinical findings and confirmed by CT-angiography data of cardiovascular malformations and fluorescence in situ hybridization (FISH) genetic test. WBS is a multisystem disorder and usually is recognized by clinician. If clinical impression is not clearly consistent with WBS, FISH remains the most widely used test.
威廉姆斯-贝伦综合征(WBS)是一种罕见的遗传性疾病,具有一系列独特的临床表现。该疾病可通过可识别的畸形模式进行临床诊断,包括心血管畸形、特征性面部畸形以及神经和认知特征。我们报告了一例23岁女性因大量咯血反复入住肺病科门诊的病例。通过临床检查发现确诊为威廉姆斯-贝伦综合征,并经心血管畸形的CT血管造影数据和荧光原位杂交(FISH)基因检测得以证实。WBS是一种多系统疾病,通常由临床医生识别。如果临床印象与WBS不太相符,FISH仍然是使用最广泛的检测方法。