Ozer G, Yüksel B, Süleymanova D, Alhan E, Demircan N, Onenli N
Department of Pediatrics, Metabolism and Endocrinology, Medical Faculty of Cukurova University, Adana, Turkey.
Acta Paediatr Jpn. 1995 Apr;37(2):233-6. doi: 10.1111/j.1442-200x.1995.tb03306.x.
Six patients with Bardet-Biedl syndrome who have been followed in our clinics for the last 5 years are reported in this study. Of the five classic features of this syndrome; obesity and mental retardation were present in all cases, retinal disturbances were present in five, polydactyly in three and hypogenitalism was observed in all four male patients. Renal involvement, often suggested as a cardinal feature of this syndrome, was described in two patients. Iron deficiency anemia occurred in three patients, two patients were of short stature, one patient presented with an empty sella, and in two patients clinodactyly was detected. The results are compared to previously published literature and discussed.
本研究报告了过去5年在我们诊所随访的6例巴德-比德尔综合征患者。在该综合征的五个典型特征中,所有病例均存在肥胖和智力发育迟缓,五例有视网膜病变,三例有多指畸形,所有四名男性患者均观察到性器官发育不全。常被认为是该综合征主要特征的肾脏受累在两名患者中被描述。三名患者出现缺铁性贫血,两名患者身材矮小,一名患者有蝶鞍空泡,两名患者检测到手指弯曲。将结果与先前发表的文献进行了比较并进行了讨论。