Improvement in glycaemic parameters using SGLT-2 inhibitor and GLP-1 agonist in combination in an adolescent with diabetes mellitus and Prader-Willi syndrome: a case report

Toby Candlerhttp://orcid.org/https://orcid.org/0000-0002-4587-8744 1 , 2 , David McGregor 3 , Kruthika Narayan 1 , 4 , Chris Moudiotis 3 ,  and Christine P. Burren 1 , 5
  • 1 Department of Paediatric Endocrinology, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  • 2 Medical Research Council The Gambia at London School of Hygiene and Tropical Medicine, London, UK
  • 3 Department of Paediatrics, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK
  • 4 Department of Paediatric Endocrinology and Diabetes, Queensland Children's Hospital, Brisbane, Australia
  • 5 Bristol Medical School, Department of Translational Health Sciences, University of Bristol, Bristol, UK
Toby CandlerORCID iD: https://orcid.org/0000-0002-4587-8744
  • Corresponding author
  • Department of Paediatric Endocrinology, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  • Medical Research Council The Gambia at London School of Hygiene and Tropical Medicine, London, UK
  • orcid.org/0000-0002-4587-8744
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, David McGregor
  • Department of Paediatrics, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK
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, Kruthika Narayan
  • Department of Paediatric Endocrinology, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  • Department of Paediatric Endocrinology and Diabetes, Queensland Children's Hospital, Brisbane, Australia
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, Chris Moudiotis
  • Department of Paediatrics, Royal Devon and Exeter NHS Foundation Trust, Exeter, UK
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and Christine P. Burren
  • Department of Paediatric Endocrinology, Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  • Bristol Medical School, Department of Translational Health Sciences, University of Bristol, Bristol, UK
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  • degruyter.comGoogle Scholar

Abstract

Objectives

Prader-Willi Syndrome (PWS) is characterised by hyperphagia often leading to obesity; a known risk factor for insulin resistance and type 2 (T2) diabetes. We present a prepubertal girl with PWS who developed diabetes.

Case presentation

Our case was diagnosed with PWS in infancy following investigation for profound central hypotonia and feeding difficulties. She commenced growth hormone (GH) aged 8 years for short stature and treatment improved linear growth. At age 12 years, she presented with polydipsia, polyuria and vulvovaginitis. She was overweight (BMI SDS +1.43). Diabetes was diagnosed (Blood glucose = 24.2 mmol/L, HbA1c = 121 mmol/mol or 13.2%). She was not acidotic and had negative blood ketones. Autoantibodies typical of type 1 diabetes were negative. She was initially treated with basal bolus insulin regime. GH was discontinued 3 months later due to concerns regarding GH-induced insulin resistance. Off GH, insulin requirements reduced to zero, allowing Metformin monotherapy. However off GH, she reported significant lethargy with static growth and increased weight. Combinations of Metformin with differing insulin regimes did not improve glucose levels. Liraglutide (GLP-1 agonist) and Metformin did not improve glucose levels nor her weight. Liraglutide and Empaglifozin (SGLT-2 inhibitor) therapy used in combination were well tolerated and demonstrated rapid normalisation of blood glucose and improvement in her HbA1c to within target (48 mmol/mol) which was sustained after 6 months of treatment.

Conclusions

Newer treatments for type 2 diabetes (e. g. GLP-1 agonists or SGLT-2 inhibitors) offer potential treatment options for those with diabetes and PWS when conventional treatments are ineffective.

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The Journal of Pediatric Endocrinology and Metabolism (JPEM) is the only international journal dedicated exclusively to endocrinology in the neonatal, pediatric and adolescent age groups, and publishes the results of clinical investigations in pediatric endocrinology and basic research. JPEM publishes Review Articles, Original Research, Case Reports, Short Communications and Letters to the Editor.

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