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A 35-Month-Old Girl Diagnosed with Hyperglycemic Hyperosmolar Syndrome

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Diabetes meter- glucometer

Hyperglycemic Hyperosmolar Syndrome (HHS) is a relatively uncommon but severe complication of diabetes mellitus, typically seen in adults. However, it can occasionally manifest in pediatric patients, often with life-threatening consequences. In this article, we present a case of a 35-month-old girl who was newly diagnosed with type 1 diabetes (T1DM) and developed HHS, highlighting the unique challenges and considerations when managing this condition in young children. This case report was published in Wiley.  

HHS is characterized by markedly elevated blood glucose levels, hyperosmolality, and the absence or low levels of ketones in the blood or urine. It is a diabetic hyperglycemic emergency, but its occurrence in children is exceedingly rare. While HHS is more commonly associated with adult patients, it can also emerge in younger individuals as the initial presentation of type 2 diabetes (T2DM). It is crucial to differentiate between HHS and diabetic ketoacidosis (DKA), another severe diabetic complication, as their clinical presentations and management differ significantly. 

DKA typically develops rapidly, often within hours, and is characterized by symptoms such as hyperventilation, vomiting, and abdominal pain, prompting parents to seek immediate medical attention for their children. In contrast, HHS tends to evolve over several days, initially presenting with symptoms like increased urination (polyuria) and excessive thirst (polydipsia), which may not immediately raise concern. However, over time, HHS can lead to severe dehydration and electrolyte imbalances, making its diagnosis and management challenging. Moreover, the dehydration and metabolic disturbances in HHS tend to be more severe than those in DKA. 

The diagnosis of HHS is based on specific criteria, including: 

Plasma glucose levels exceeding 600 mg/dl. Venous pH greater than 7.25. Serum bicarbonate levels hiare gher than 15 mmol/L. The presence of only small amounts of ketones in urine or their absence. Effective serum osmolality greater than 320 mOsm/kg. Altered mental status, which can range from obtundation to combativeness or seizures. 

A previously healthy 35-month-old girl was brought to the emergency room of Namazi Hospital in Shiraz, Iran, with a reduced level of consciousness. Her medical history was unremarkable until five days prior when she began experiencing dysuria and loss of appetite. Subsequently, she developed polyuria, polydipsia, and a significant weight loss, going from 14 kg to 11 kg. There was no family history of diabetes. 

Upon arrival at the hospital, her physical examination revealed a height at the 25th-50th percentile, weight at the 5th-10th percentile, and a Body Mass Index (BMI) at the 72nd percentile. Her vital signs were concerning, with a temperature of 38°C, heart rate of 160, blood pressure of 95/50, and a Glasgow Coma Scale (GCS) score of 11/15. Capillary refilling time exceeded 3 seconds, and her pulses were weak. A capillary blood glucose test confirmed significantly elevated blood glucose levels, prompting further evaluation with a serum glucose test. 

Given her decreased level of consciousness, a brain CT scan was performed, revealing brain edema. Initial treatment involved hydration with normal saline at a rate of 10 cc per kg. Subsequent laboratory results showed a blood sugar level of 1124 mg/dl. Upon transfer to the Pediatric Intensive Care Unit (PICU), the patient experienced a generalized seizure, and her GCS deteriorated to less than 8, necessitating intubation.

Hydration with normal saline was continued, and intravenous fluids were administered to address the deficit and maintain fluid balance, considering urine output. Due to the presence of brain edema, the deficit was managed over a 72-hour period. However, adjustments were made based on urine output and serum sodium levels, leading to an increase in the deficit to 18 percent and a decrease in the sodium content of the intravenous fluid. 

HHS in pediatric patients, especially as the initial presentation of T1DM, is a rare but potentially life-threatening condition with a high mortality rate. Unlike DKA, which often presents with symptoms such as vomiting, abdominal pain, and drowsiness, leading parents to seek immediate medical attention, HHS can manifest with a gradual onset of symptoms. This delayed presentation can result in severe dehydration and electrolyte imbalances, underscoring the importance of early diagnosis and management. 

In previous studies, the mortality rate for HHS has been reported to be as high as 35%, depending on the severity of dehydration, hyperosmolality, and the patient’s age. However, through the appropriate therapeutic measures implemented in this case, the patient achieved a full recovery without any lasting complications. Hyperglycemic Hyperosmolar Syndrome is an unusual but severe complication of diabetes in pediatric patients, and it can be particularly challenging to diagnose and manage, especially when it presents as the initial manifestation of diabetes.

Physicians should be vigilant about considering HHS in young children, even in the absence of obesity. Prompt recognition and intervention are crucial to prevent severe dehydration and electrolyte imbalances, which can have dire consequences. Awareness of the unique clinical presentation of HHS in pediatric patients is essential for timely diagnosis and effective treatment, ultimately improving outcomes for these young individuals.  

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