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Diabetic retinopathy in Mauriac's syndrome. Paradoxical deterioration with improved metabolic control
Insights
Rapidly improving glycemic control in poorly managed diabetes can paradoxically worsen retinopathy and cause vision loss. Caution is advised when correcting metabolic derangements in these patients.
Area of Science:
- Pediatrics
- Endocrinology
- Ophthalmology
Background:
- Four pediatric patients presented with short stature, hepatomegaly, and delayed adolescence.
- Initial ophthalmic findings included minimal microaneurysms or a single intraretinal hemorrhage.
Purpose of the Study:
- To investigate the effects of improved glycemic control on retinopathy progression in children with poorly controlled diabetes.
- To highlight the potential risks associated with rapid metabolic correction.
Main Methods:
- Case report detailing clinical observations and treatment interventions in four pediatric patients.
- Monitoring of growth, pubertal development, hepatomegaly, and retinal status before and after intensive diabetes management.
Main Results:
- Significant improvement in growth rate and regression of hepatomegaly occurred with increased insulin dosage.
- Paradoxical rapid progression of diabetic retinopathy was observed despite improved metabolic control.
- One patient developed proliferative retinopathy, vitreous hemorrhage, and blindness, requiring panretinal photocoagulation.
Conclusions:
- Aggressive correction of hyperglycemia in long-standing poorly controlled diabetes may precipitate rapid retinopathy progression.
- Further research is needed to understand the mechanisms behind these adverse retinal changes.
- Clinicians should exercise caution when managing metabolic derangements in this patient population.
Abstract:
We report four children with marked short stature, hepatomegaly, and delayed adolescence. Initial funduscopy demonstrated only occasional microaneurysms in two children and a single intraretinal hemorrhage in another. 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. One child with proliferative retinopathy in both eyes developed vitreous hemorrhage and blindness in one eye. Two required pan retinal 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.