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Who’s Afraid of Hyperosmolar Nonketotic Hyperglycemia?

March 5, 2024

I am. I don’t understand it. I can’t get my head around it. I would go so far as to say that I would not know it if it hit me in the face. Because I “don’t get it,” I have always worried that I might miss this critical, life-threatening diagnosis when faced with a child who presents with hyperglycemia, dehydration, altered mental status, and minimal ketosis. To me, treating a patient with hyperosmolar nonketotic hyperglycemia, also known as hyperosmolar hyperglycemic state (HHS), is even more complex than treating a child with diabetic ketoacidosis (DKA). What is even scarier, as authors Drs. Simon, Shah, and Shah say in this month’s Pediatrics in Review In Brief “Hyperosmolar Nonketotic Hyperglycemia,” (10.1542/pir.2022-005563) is that “patients can present with a mixed picture of both diabetic ketoacidosis (DKA) and HHS symptoms, accounting for 28% of pediatric cases and a mortality rate 10-fold higher than pure DKA.” If a patient presents with a mixture of DKA and HHS, how does the clinician figure out how to treat?

One would think that all patients with insulin deficiency and hyperglycemia would naturally, inherently switch to fat catabolism to derive energy for everyday activity, with resulting ketosis as a byproduct of that fat catabolism. But this is not true. As Drs. Simon, Shah, and Shah explain, there are risk factors of an increased ratio of insulin to glucagon and a physiologic stress state of increased glucose production via gluconeogenesis and glycogenolysis that contribute to HHS. What causes these two risk factors? Because HHS occurs primarily in those with type 2 diabetes mellitus, it’s likely the obesity epidemic, which has led to the earlier age of onset of type 2 diabetes, accounts for the increasing prevalence of HHS in the pediatric population. Drs. Simon, Shah, and Shah also mention the contribution of infection and of high-carbohydrate beverages. Check out the other intriguing information in this article.

What clearly comes across in this article is that many of us pediatricians “don’t get it,” and that with the rise in prevalence in pediatric patients and high mortality incidence, further study on what causes HHS should be a top priority.

I suspect a number of you readers, like me, are intimidated by this topic. But upon reading this succinct, practical, and informative piece, I hope that you, like me, will find yourself eloquently reminded of our role as pediatricians to not just diagnose but help prevent. In the final comments, “In Brief” editor Dr Janet Serwint reminds us that, in the face of “the insidious presentation (of hyperosmolar nonketotic hyperglycemia)” and “the marked increase in the prevalence of obesity with resulting increased risks of both HHS and DKA . . . we must continue to counsel our patients to eat a healthy diet, incorporate appropriate portion sizes, and exercise to enhance the health of our future generations.”

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