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Atypical Presentation of New Onset Diabetes with Hyperglycemic Hyperosmolar State in Two Toddlers
Alina Haque1, Esther E Bell-Sambataro2, Foram Patel3
1The Ohio State University Faculty of Medicine, Columbus, Ohio, United States
Insights
Hyperglycemic hyperosmolar state (HHS) is a rare diabetes complication in children. Early recognition and aggressive rehydration are crucial for managing HHS and preventing severe outcomes.
Area of Science:
- Pediatrics
- Endocrinology
- Metabolic Disorders
Background:
- Hyperglycemic hyperosmolar state (HHS) and mixed HHS with diabetic ketoacidosis (DKA) are rare but serious diabetes complications in children.
- Prompt recognition of hyperosmolality is critical for preventing severe morbidity and mortality.
Purpose of the Study:
- To report two of the youngest documented cases of HHS presenting at new-onset type 1 diabetes in children.
- To highlight the challenges in diagnosing and managing HHS in young children with developmental delays.
Main Methods:
- Case report of two pediatric patients with new-onset type 1 diabetes presenting with HHS and mixed HHS-DKA.
- Review of clinical presentation, laboratory values, and management strategies, including rehydration and insulin therapy.
Main Results:
- Two young males (ages 3 and 4) with autism spectrum disorder and Trisomy 21 presented with severe hyperglycemia and hyperosmolality, complicated by acute kidney injury and pancreatitis.
- Initial management overlooked hyperosmolality, leading to aggressive insulin dosing typical of DKA, before appropriate rehydration and delayed insulin initiation were implemented.
Conclusions:
- Young age and developmental delays can contribute to the development of HHS due to communication difficulties and limited water access.
- A high index of suspicion for HHS is essential in pediatric diabetes, necessitating significant rehydration and a delayed start of low-dose insulin infusion to prevent complications.
Abstract:
Hyperglycemic hyperosmolar state (HHS), or mixed HHS with diabetic ketoacidosis (DKA), is a rare complication of diabetes in children. Prompt recognition of hyperosmolality is necessary to prevent morbidity and mortality. We report two of the youngest cases with HHS, both presenting as new onset of type 1 diabetes. The first was a 3-year-4-month-old male with autism spectrum disorder who presented with glucose 76.0 mmol/L (1370 mg/dL), calculated serum osmolality 388 mOsm/kg, and trace urinary ketones, consistent with HHS and complicated by acute kidney injury. The second was a 4-year-7-month-old male with Trisomy 21 and autism spectrum disorder who presented with glucose 117.3 mmol/L (2114 mg/dL), calculated serum osmolality 401 mOsm/kg, and elevated serum β-hydroxybutyrate, consistent with mixed HHS-DKA and complicated by acute kidney injury and pancreatitis. Both received aggressive rehydration although hyperosmolality was initially overlooked, resulting in earlier and higher insulin dosing more typical of DKA than HHS. Both recovered without sequelae. In each case, young age and developmental delay likely contributed to hyperosmolality, given the inability to communicate increased thirst and freely access water. A high index of suspicion for HHS is necessary as significant rehydration and delayed start of low dose insulin infusion are recommended to prevent complications.
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