Zeka Naim, Bejiqi Ramush, Gerguri Abdurrahim, Zogaj Leonore, Jashari Haki
Pediatric Clinic Department of Neurology University Clinical Center of Kosovo Pristina Kosovo.
Pediatric Clinic Department of Cardiology University Clinical Center of Kosovo Pristina Kosovo.
Clin Case Rep. 2022 May 23;10(5):e05886. doi: 10.1002/ccr3.5886. eCollection 2022 May.
Feingold syndrome 1 (FS1) is a rare disorder that is inherited in autosomal dominant manner with full penetrance but with variable expressivity. The most common phenotypical features described are finger and toe anomalies, microcephaly, short stature, and intestinal atresia. Dysmorphic features, intellectual disability and other organ anomalies are less frequently described. Here, we present a 7-year-old boy with severe intellectual disability who is diagnosed with FS1 syndrome caused by a new heterozygous variant of MYCN gene.
费因戈尔德综合征1型(FS1)是一种罕见的疾病,以常染色体显性方式遗传,具有完全外显率但表现度可变。所描述的最常见表型特征是手指和脚趾异常、小头畸形、身材矮小和肠道闭锁。畸形特征、智力残疾和其他器官异常较少被描述。在此,我们报告一名7岁患有严重智力残疾的男孩,他被诊断为因MYCN基因的一种新的杂合变异导致的FS1综合征。