Wu Wen-Chih, Chen Shinn-Cherng, Dia Chia-Yen, Yu Ming-Lung, Hsieh Ming-Yuh, Lin Zu-Yau, Wang Liang-Yen, Tsai Jung-Fa, Chang Wen-Yu, Chuang Wan-Long
Division of Hepatobiliary Medicine, Department of Internal Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
Kaohsiung J Med Sci. 2003 Jul;19(7):358-61. doi: 10.1016/S1607-551X(09)70438-3.
We report the case of a 21-year-old female with Alström syndrome who also suffered from acute pancreatitis of obscure manifestation. The patient had underlying cone-rod dystrophy of the retinas, nystagmus, obesity, progressive sensorineural hearing impairment, diabetes mellitus, and hypertriglyceridemia, compatible with the clinical diagnosis of Alström syndrome. Serial examinations showed liver dysfunction and pancreatitis. In treating a patient with poor communication (i.e. cone-rod dystrophy and hearing impairment) suffering from acute illness, understanding the underlying disease and the potential for pancreatitis with hypertriglyceridemia is necessary. It is also a challenge to treat a patient with multiple system involvement. In conclusion, Alström syndrome is a disease of systemic multi-organ involvement, and hepatic disease and pancreatitis, possibly due to dyslipidemia, appear to be manifestations of Alström syndrome.
我们报告了一例患有阿尔斯特伦综合征的21岁女性病例，该患者还患有表现不明的急性胰腺炎。患者存在潜在的视网膜锥杆营养不良、眼球震颤、肥胖、进行性感音神经性听力障碍、糖尿病和高甘油三酯血症，符合阿尔斯特伦综合征的临床诊断。系列检查显示肝功能障碍和胰腺炎。在治疗患有急性疾病且沟通困难（即锥杆营养不良和听力障碍）的患者时，了解潜在疾病以及高甘油三酯血症引发胰腺炎的可能性是必要的。治疗涉及多个系统的患者也是一项挑战。总之，阿尔斯特伦综合征是一种全身性多器官受累的疾病，肝脏疾病和胰腺炎可能由于血脂异常，似乎是阿尔斯特伦综合征的表现。
