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糖尿病侏儒症(Mauriac综合征)中进行性视网膜病变且病情控制改善。

Progressive retinopathy with improved control in diabetic dwarfism (Mauriac's syndrome).

作者信息

Daneman D, Drash A L, Lobes L A, Becker D J, Baker L M, Travis L B

出版信息

Diabetes Care. 1981 May-Jun;4(3):360-5. doi: 10.2337/diacare.4.3.360.

DOI:10.2337/diacare.4.3.360
PMID:7047112
Abstract

We report four children aged 11-18 1/2 yr first seen 7-14 yr after the diagnosis of insulin-dependent diabetes. At presentation, all had marked short stature, two had hepatomegaly, and the older three had delayed adolescence. They had been severely underinsulinized. Initial funduscopy demonstrated only occasion microaneurysms in two children and a single intraretinal hemorrhage in another. The youngest was normal. Improved control required large increases in insulin dosage. Growth rate improved significantly and hepatomegaly regressed. Puberty progressed rapidly in two older patients with poor final height. Paradoxically, with improved control, retinopathy progressed rapidly with appearance of multiple microaneurysms, nerve fiber layer infarctions, intraretinal microangiopathic changes, hemorrhages, exudates, and macular edema in all the patients and severe proliferation changes in three. One child with proliferative retinopathy in both eyes developed vitreous hemorrhage and blindness in one eye. Two required panretinal photocoagulation with no further progression of their retinopathy. These rapidly progressive retinal changes remain unexplained. We advise caution when correcting metabolic derangements of diabetic patients who have been poorly controlled for a prolonged period.

摘要

我们报告了4名年龄在11岁至18岁半的儿童,他们在被诊断为胰岛素依赖型糖尿病7至14年后首次就诊。就诊时,所有患儿均有明显身材矮小,2例有肝肿大,年龄较大的3例青春期延迟。他们胰岛素治疗严重不足。初诊时眼底检查仅发现2例患儿偶尔有微动脉瘤,另1例有单个视网膜内出血。最年幼的患儿眼底正常。改善血糖控制需要大幅增加胰岛素剂量。生长速度显著提高,肝肿大消退。两名年龄较大、最终身高不理想的患者青春期进展迅速。矛盾的是,随着血糖控制的改善,视网膜病变迅速进展,所有患者均出现多个微动脉瘤、神经纤维层梗死、视网膜内微血管病变、出血、渗出和黄斑水肿,3例出现严重增殖性改变。一名双眼患有增殖性视网膜病变的患儿发生玻璃体积血,一只眼睛失明。2例需要进行全视网膜光凝治疗,视网膜病变未进一步进展。这些迅速进展的视网膜变化原因不明。对于长期血糖控制不佳的糖尿病患者,纠正代谢紊乱时我们建议谨慎行事。

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Progressive retinopathy with improved control in diabetic dwarfism (Mauriac's syndrome).糖尿病侏儒症(Mauriac综合征)中进行性视网膜病变且病情控制改善。
Diabetes Care. 1981 May-Jun;4(3):360-5. doi: 10.2337/diacare.4.3.360.
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Diabetic retinopathy in Mauriac's syndrome. Paradoxical deterioration with improved metabolic control.毛里阿克综合征中的糖尿病性视网膜病变。代谢控制改善却出现矛盾性恶化。
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[Dwarfism and delayed puberty in a child with insulin-dependent diabetes mellitus].
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Hepatomegaly and type 1 diabetes: a clinical case of Mauriac's syndrome.肝肿大和 1 型糖尿病:Mauriac 综合征的临床病例。
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Evidence that upregulation of serum IGF-1 concentration can trigger acceleration of diabetic retinopathy.血清胰岛素样生长因子-1(IGF-1)浓度上调可引发糖尿病视网膜病变加速的证据。
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Old syndrome-new approach: Mauriac syndrome treated with continuous insulin delivery.
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Nat Rev Endocrinol. 2010 Sep;6(9):494-508. doi: 10.1038/nrendo.2010.122. Epub 2010 Jul 27.
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