Secondary diabetes as a rare complication of glycogen storage disease 1a: 
case report and review of literature

Anshita Aggarwal; Deepika Patel; Bindu Kulshreshtha

doi:10.5114/pedm.2021.109121

eISSN: 2083-8441
ISSN: 2081-237X

Pediatric Endocrinology Diabetes and Metabolism

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abstract:

Case report

Secondary diabetes as a rare complication of glycogen storage disease 1a: case report and review of literature

Anshita Aggarwal

¹

,

Deepika Patel

¹

,

Bindu Kulshreshtha

¹

Department of Endocrinology, ABVIMS & Dr Ram Manohar Lohia Hospital, India

Pediatr Endocrinol Diabetes Metab 2021; 27 (4): 283–286

DOI: https://doi.org/10.5114/pedm.2021.109121

Online publish date: 2021/12/30

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Glycogen storage diseases (GSDs) are disorders of carbohydrate metabolism and hypoglycemia is their hallmark. Secondary diabetes with glycogen storage disease, which seems rather paradoxical, has been rarely reported. A 13-year-old girl previously diagnosed to have GSD 1a presented to the emergency with multiple episodes of vomiting and loss of consciousness. She had hyperglycemia, ketonuria, hyperlactatemia and metabolic acidosis, suggestive of diabetic ketosis with acidosis possibly contributed by both high serum lactate and serum ketones. Her glycated hemoglobin was high, with detectable serum insulin levels, which suggested secondary diabetes in the background of GSD Ia. Her management posed a therapeutic challenge. She was managed with insulin and achieved good glycemic control. We wish to conclude that GSD may rarely lead to secondary diabetes as a complication and the management is complex owing to the nature of the disease. Insulin remains the mainstay of the treatment.

keywords:

Secondary diabetes as a rare complication of glycogen storage disease 1a: case report and review of literature

Anshita Aggarwal 1 , Deepika Patel 1 , Bindu Kulshreshtha 1

glycogen storage disease, diabetes, insulin

Anshita Aggarwal

¹

,

Deepika Patel

¹

,

Bindu Kulshreshtha

¹