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Hyperglycemic hyperosmolar non-ketotic syndrome in children with type 2 diabetes*
Shannon H Fourtner1, Stuart A Weinzimer, Lorraine E Levitt Katz
1Department of Pediatrics, Division of Endocrinology/Diabetes, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. fourtner@email.chop.edu
Insights
Hyperglycemic hyperosmolar non-ketotic (HHNK) syndrome, though rare in children, occurred in 3.7% of new type 2 diabetes cases. While most survivors had no lasting effects, the syndrome presented a significant mortality risk.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Diabetes Mellitus
Background:
- Hyperglycemic hyperosmolar non-ketotic (HHNK) syndrome is considered rare in children.
- Increasing prevalence of childhood obesity and type 2 diabetes may lead to more HHNK syndrome cases.
- Existing literature primarily consists of case reports, lacking updated information on typical clinical course and outcomes.
Purpose of the Study:
- To provide updated information on the clinical course and sequelae of HHNK syndrome in pediatric patients.
- To assess the frequency of HHNK syndrome in children diagnosed with type 2 diabetes.
- To highlight the association between HHNK syndrome and new-onset type 2 diabetes in children.
Main Methods:
- Retrospective chart review of patients diagnosed with type 2 diabetes at Children's Hospital of Philadelphia over 5 years.
- Screening for laboratory evidence of HHNK syndrome using standard diagnostic criteria (blood glucose >600 mg/dL, serum osmolality >330 mOsm/L, mild acidosis).
- Analysis of clinical presentation, demographic data, and patient outcomes.
Main Results:
- Seven out of 190 patients (3.7%) with type 2 diabetes met the criteria for HHNK syndrome.
- All seven patients were African-American, with a mean age of 13.3 years, and HHNK syndrome was their initial presentation of diabetes.
- The case fatality rate was 14.3% (one death), with survivors experiencing an average hospital stay of 10 days and no neurodevelopmental sequelae.
Conclusions:
- HHNK syndrome occurs in a notable percentage of pediatric type 2 diabetes cases.
- Increased awareness of type 2 diabetes in children is crucial for preventing HHNK syndrome.
- Early recognition and management can mitigate the significant morbidity and mortality associated with HHNK syndrome in pediatric patients.
Objective:
Hyperglycemic hyperosmolar non-ketotic (HHNK) syndrome is thought to be a rare entity in the pediatric population, associated with significant mortality based on case reports in the literature. As obesity and type 2 diabetes in childhood grow in prevalence, such related complications may also increase. This study will serve to provide updated information regarding typical clinical course and sequelae of HHNK syndrome in childhood.
Methods:
Patients diagnosed with type 2 diabetes at Children's Hospital of Philadelphia (CHOP) over a period of 5 yr were screened retrospectively for any laboratory evidence of previous episodes of HHNK syndrome. The standard diagnostic criteria of blood glucose >600 mg/dL and serum osmolality >330 mOsm/L with only mild acidosis (serum bicarbonate >15 mmol/L and small ketonuria 15 mg/dL or less) were utilized.
Results:
The records of all patients with type 2 diabetes mellitus (DM) diagnosed over a 5-yr period were reviewed (n=190). Seven patients were found to have one episode of HHNK syndrome by diagnostic criteria (five males, mean age at presentation 13.3 yr, age range 10.1--16.9 yr), yielding a frequency of 3.7%. All were African-American. HHNK syndrome was the clinical presentation at diagnosis of new onset diabetes for all seven children. Three of seven children had a previously diagnosed developmental delay. The average Glasgow Coma Scale (GCS) score at presentation was 13 (range 9--15). Mean body mass index (BMI) at presentation was 32.7 kg/m(2) (n=6). Mean serum osmolality was 393 mOsm/L (n=7), and mean blood glucose was 1604 mg/dL (n = 7). The average time until mental status returned to baseline among survivors was 3 d (range 1--7 d). The average number of hospital days for survivors was 10 (range 5--24 d). Four of seven patients had an uncomplicated course. One patient developed multisystem organ failure and died on hospital day 4. The case fatality rate was 14.3% (one of seven). Survivors had no appreciable neurodevelopmental sequelae.
Conclusions:
This retrospective chart review provides updated information regarding the entity of HHNK syndrome in children. This study supports the need for increased awareness of type 2 diabetes in children so that morbidity and mortality related to HHNK syndrome can be prevented.
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