Gutch Manish, Philip Rajeev, Saran Sanjay, Tyagi Rajiv, Gupta Keshav Kumar
Department of Endocrinology, Lala Lajpat Rai Memorial Medical College, Meerut, Uttar Pradesh, India.
Indian J Endocrinol Metab. 2013 Oct;17(Suppl 1):S283-5. doi: 10.4103/2230-8210.119611.
Mauriac syndrome is a rare syndrome associated with type 1 diabetes (T1DM) in children presenting with growth retardation, hepatomegaly, and cushingoid features. Recently, there has been re-emergence of this syndrome, especially with the use of premix insulin. A 15-year old type 1 diabetic boy, who was on premix insulin with erratic blood glucose, was referred to us for evaluation of short stature. He had significant short stature, hepatomegaly, and cushingoid features. His growth hormone (GH) stimulation was normal, and so was the overnight dexamethasone suppression test, based on which the diagnosis of Mauriac syndrome was reported. He was made to switch over to basal bolus regime, and was advised to follow-up for 6 months. He had reduction in hepatomegaly and a height gain of 3 cms.
莫里亚克综合征是一种与儿童1型糖尿病（T1DM）相关的罕见综合征，表现为生长发育迟缓、肝肿大和库欣样特征。最近，这种综合征再度出现，尤其是在使用预混胰岛素的情况下。一名15岁的1型糖尿病男孩，使用预混胰岛素且血糖不稳定，因身材矮小前来我们这里评估。他有明显的身材矮小、肝肿大和库欣样特征。他的生长激素（GH）刺激试验正常，过夜地塞米松抑制试验也正常，据此报告了莫里亚克综合征的诊断。他改为基础加餐治疗方案，并被建议随访6个月。他的肝肿大有所减轻，身高增加了3厘米。
