Stagi S, Bindi G, Lapi E, Giovannucci-Uzielli M L, Salti R, Chiarelli F
Paediatric Endocrinology Unit, Department of Paediatrics, University of Florence, A. Meyer Children's Hospital, Florence, Italy.
J Pediatr Endocrinol Metab. 2008 Nov;21(11):1089-92. doi: 10.1515/jpem.2008.21.11.1089.
We describe a patient with the clinical spectrum of Young-Simpson syndrome. This rare genetic disorder is characterized by congenital hypothyroidism, mental retardation and blepharophimosis. Young-Simpson syndrome is, at present, poorly known to endocrinologists and pediatricians, and should be included in the differential diagnosis of congenital hypothyroidism. It is important to underline that the association of congenital hypothyroidism, blepharophimosis and ptosis allows an exact clinical diagnosis, since the majority of other clinical aspects are common to other disorders.
我们描述了一位具有扬-辛普森综合征临床症状的患者。这种罕见的遗传疾病的特征是先天性甲状腺功能减退、智力障碍和睑裂狭小。目前,内分泌学家和儿科医生对扬-辛普森综合征了解甚少,在先天性甲状腺功能减退的鉴别诊断中应考虑该疾病。需要强调的是,先天性甲状腺功能减退、睑裂狭小和上睑下垂同时出现有助于做出准确的临床诊断,因为大多数其他临床症状在其他疾病中也较为常见。