Simşek-Kiper Pelin Ozlem, Utine Gülen Eda, Alanay Yasemin, Aktaş Dilek, Alikaşifoğlu Mehmet, Boduroğlu Koray
Pediatric Genetics Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Turk J Pediatr. 2011 Sep-Oct;53(5):558-60.
Chromosome 2q37 microdeletion syndrome is a rare disorder characterized by mild-moderate psychomotor and growth retardation, autistic-like behavior, Albright hereditary osteodystrophy-like metacarpal/metatarsal shortening, and facial characteristics. We here report on a patient with 2q37 microdeletion presenting with learning difficulty, hyperactivity and attention deficit. Physical examination revealed psychomotor and growth retardation, facial dysmorphism and brachydactyly, suggestive of Albright hereditary osteodystrophy-like phenotype. Laboratory evaluation revealed 46, XX.ish subtel(2q)(D2S447-) confirming 2q37 microdeletion. Chromosome 2q37 microdeletion syndrome should be considered in the differential diagnosis of patients presenting with psychomotor and growth retardation and an Albright hereditary osteodystrophy-like phenotype, especially in the presence of brachydactyly, even if the characteristic facial features are missing.
2q37染色体微缺失综合征是一种罕见的疾病,其特征为轻至中度精神运动发育迟缓和生长发育迟缓、类自闭症行为、类似奥尔布赖特遗传性骨营养不良的掌骨/跖骨缩短以及面部特征。我们在此报告一名患有2q37微缺失的患者,表现为学习困难、多动和注意力缺陷。体格检查发现精神运动发育迟缓和生长发育迟缓、面部畸形和短指畸形,提示类似奥尔布赖特遗传性骨营养不良的表型。实验室评估显示46, XX.ish subtel(2q)(D2S447-),证实存在2q37微缺失。对于出现精神运动发育迟缓和类似奥尔布赖特遗传性骨营养不良表型的患者,尤其是存在短指畸形的患者,即使缺乏特征性面部特征,在鉴别诊断时也应考虑2q37染色体微缺失综合征。