Spring 2019 Issue
Volume 11 | Number 2
Irwin Benzel, DO; Laura Nimkoff, MD
Good Samaritan Hospital Medical Center
Hyperosmolar Hyperglycemic State is an acute complication of diabetes mellitus characterized as hyperglycemia, hyperosmolarity, and dehydration that is usually associated with type 2 diabetes. This case describes the unusual but increasingly recognized complication of Type 1 diabetes mellitus in children.
DE is a 12-year-old Caucasian morbidly obese male presenting with three day history of worsening polyuria and polydipsia and a one day history of decreased activity and heavy breathing. There was vomiting on the day prior to admission, but no abdominal pain and no fevers. On physical exam DE was ill appearing, but easily arousable. He was tachycardic with Kussmaul respirations and poor perfusion. Initial laboratory findings were notable for glucose of 1282, serum osmolality 406, Anion Gap 36, Corrected Sodium 150, Bicarb 11, BUN/Cr 59/2.3, pH 7.12, and UA with 40ketones and >1000glucose. He was admitted to the PICU with a diagnosis of DKA and HHS. He was initially treated with fluid boluses, followed by IV hydration at twice maintenance and a slow insulin infusion of 0.05Units/kg/hour. Three hours into treatment, DE developed fevers as high as 105.8 and an altered mental status. He was placed on a cooling blanket and given antipyretics. These findings lasted for four hours and then he slowly improved and returned to baseline. His fluid losses were replaced slowly over 72 hours and at the end of his hospital stay he required 24 liters of fluid to correct his sodium.
The case illustrates the emergence of HHS as a complication of DKA from IDDM in adolescents. HHS has been well described in the adult literature, but only a few case reports have been seen in pediatrics. A rare and often fatal complication of HHS with DKA is a malignant hyperthermia-like syndrome. The patient’s high fever coupled with evidence of HHS and rhabdomyolysis may have been a mild form of the syndrome with his symptoms resolving prior to cardiovascular instability developing.
DE is a 12-year-old previously healthy, Caucasian, morbidly obese male presenting with three days of gradually worsening polyuria and polydipsia. He had been drinking large quantities of juice and milk and having multiple episodes of nocturnal. He had increased fatigue with mild sore throat, nausea and poor appetite. He had associated loose stools two-three episodes daily and three episodes of vomiting on the day prior to admission. His mother brought him to the ER when he started to have heavy breathing and became more somnolent. No abdominal pain. No fevers. Family history was (+) for hypothyroidism and Type 2 DM in mother.
PMH: strabismus with corrective surgery and orchiopexy of left undescended testicle
Glucose 1281 Calcium 10.4 AGAP 43
6 11 18.8>18/58.5<697 P92 B1 L3
Serum Osmolality: 406 VBG: 7.12/35/45/11.4/-17 UA: 1027/40ketones/100protein/>1000glucose/moderate blood no RBCs Albumin, LFT, Amylase and Lipse within normal limits
DE was diagnosed with new onset type 1diabetes, DKA and HHS. He was treated with fluid boluses, followed by IV hydration at twice maintenance and a slow insulin infusion of 0.05 Units/kg/hour. Bedside glucose tests were done hourly and electrolytes and venous gasses were checked every two hours. DE’s mental status began to worsen three hours into treatment and he had fevers as high as 105.8F. He was placed on a cooling blanket and given antipyretics.
Ceftriaxone and Vancomycin were started and urine and blood cultures were drawn. His fevers continued with a depressed mental status for four hours. Patient’s status slowly improved thereafter and he was oriented to person and place. His fluid losses were replaced slowly over 72 hours with close monitoring of his electrolytes and serum osmolality.
Hyperosmolar Hyperglycemic Syndrome is an acute complication of diabetes mellitus characterized by hyperglycemia, hyperosmolarity and dehydration. HHS is usually associated with type 2 diabetes and is more commonly seen in adults. The Journal of Pediatrics published a review of HHS in 2009 describing an increase in frequency of case reports in adolescents with new onset diabetes. There were 71 cases reported from 2001-2008 with mortality in 37% of patients. A rare more severe complication of HHS is a malignant hyperthermia-like syndrome, which can occur with DKA. The mortality of both together nears 75%.4
HHS typically presents with a more insidious onset than DKA. Both conditions will have symptoms of polyuria and polydipsia but DKA will typically have vomiting, abdominal pain and hyperventilation which will draw the attention of parents and physicians. The lack of symptoms associated with HHS allows it to go undiagnosed for a prolonged time despite the severe dehydration and electrolyte losses. The patient may appear less dehydrated because the hypertonicity associated with HHS will maintain their intravascular volume. Treatment is therefore aimed at restoring volume to the patient without initially decreasing the osmolar gap which could cause hypovolemia.5
DE presented with a mixed picture of DKA and HHS. Clinical features of DKA were an abrupt onset of symptoms with labs showing ketones in a UA and a low bicarb on his electrolytes; however the glucose >1200 and osmolality >370 are seen more commonly in HHS. Several days after admission, his labs were positive for insulin antibodies and he was diagnosed with new onset IDDM with a hyperosmolar hyperglycemic syndrome. He was treated with low dose insulin 0.05 units/kg/hour and initial rapid fluid infusion followed by slow correction of osmolar gap, dehydration and hypernatremia over three days. He required 24 liters of fluid to correct his sodium.
During his hospital stay, DE also had four hours of sustained high fevers up to 105.8 with a diminished mental status. He was started on Vancomycin and Ceftriaxone and had blood and urine cultures drawn, which were negative.The source of fevers may be related to a malignant hyperthermia-like syndrome that is associated with HHS, but whose mechanism is not well understood. Dantrolene has been used in suspected cases, but outcomes are typically poor with or without treatment. In 2003, The Journal of Pediatrics described six patients from three separate PICUs that presented with HHS, DKA, hyperthermia, cardiovascular instability and rhabdomyolysis. Case reports from Pediatric Critical Care describe two additional cases from 2006. Out of these eight reported cases, only two patients survived. Patient DE had hyperthermia, with evidence of acute
kidney injury and myoglobin on UA. However, he never had cardiovascular instability and his symptoms resolved quickly without receiving Dantrolene. The patient’s high fevers coupled with evidence of HHS and rhabdomyolysis may have been a mild form of the syndrome.
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