Pediatric Genetic Unit, Pediatric Department of Monza Brianza per il Bambino e la sua Mamma (MBBM) Foundation, S.Gerardo Hospital, Monza, Italy.
Am J Med Genet A. 2013 Jul;161A(7):1759-62. doi: 10.1002/ajmg.a.35966. Epub 2013 May 21.
Wolf-Hirschhorn syndrome (WHS) is a rare microdeletion syndrome associated with a characteristic facial appearance, failure to thrive, psychomotor delays, and various major malformations of internal organs; many medical complications have been described (feeding difficulties, epilepsy, hearing problems). Benign or malignant oncologic problems are not a typical feature of the natural history of these patients. We report on two patients with WHS patients in whom hepatic adenoma (HA) were diagnosed during adolescence. The clinical evolution of liver involvement was different between the two. We discuss the possibility of considering HA as a rare medical problem in the follow-up of WHS patients. © 2013 Wiley Periodicals, Inc.
沃尔夫-赫希霍恩综合征（WHS）是一种罕见的微缺失综合征，其特征性面部外观、生长不良、精神运动发育迟缓以及各种内部器官的主要畸形；已描述了许多医学并发症（喂养困难、癫痫、听力问题）。良性或恶性肿瘤问题并不是这些患者自然病史的典型特征。我们报告了两名青少年被诊断为肝腺瘤（HA）的 WHS 患者。这两例患者的肝脏受累的临床演变不同。我们讨论了在 WHS 患者的随访中，将 HA 视为罕见的医学问题的可能性。©2013 年 Wiley 期刊出版公司
