Riaz Maira, Ibrahim Mohsina Noor, Laghari Taj Muhammad, Hanif Misbah Iqbal, Raza Jamal
Department of Paediatrics, National Institute of Child Health, Karachi, Pakistan.
J Coll Physicians Surg Pak. 2020 Dec;30(12):1332-1334. doi: 10.29271/jcpsp.2020.12.1332.
Van Wyk Grumbach syndrome is well known for protracted hypothyroidism, characterised by multicystic ovaries (normal size ovaries contain many follicles of various sizes), isosexual precocious puberty and delayed skeletal growth. A series of ten children with Van Wyk Grumbach syndrome is been presented with their clinical features, biochemical and radiological profile and management. Patients showed a noteworthy improvement upon thyroxine therapy. It is vital to keep this entity in consideration and; hence, should investigate for thyroid status during the evaluation of ovarian cysts. Thyroxin replacement after establishing the diagnosis early can prevent the patient from going through extensive workup and surgeries. Key Words: Hypothyrodism, Multicystic ovaries, Isosexual precocious puberty.
范·怀克-格鲁巴赫综合征以持续性甲状腺功能减退而闻名,其特征为多囊卵巢(正常大小的卵巢含有许多大小各异的卵泡)、同性性早熟和骨骼生长延迟。本文介绍了一系列十例范·怀克-格鲁巴赫综合征患儿的临床特征、生化和放射学检查结果及治疗情况。患者在接受甲状腺素治疗后有显著改善。考虑到这一病症很重要;因此,在评估卵巢囊肿时应检查甲状腺状况。早期确诊后进行甲状腺素替代治疗可避免患者接受大量检查和手术。关键词:甲状腺功能减退、多囊卵巢、同性性早熟