By Santa Santa J. Bartholomew MD, FAAP, FCCM and Brianna Brianna Czaikowski, DNP, FNP-BC, CUNP
See Corresponding Journal Article: Pediatric Diabetic Ketoacidosis
EMS (Emergency Medical Services) was called for a 15-year-old found deceased in the basement of his home. Grandparents called and reported the child deceased and states he had been ill over the past 1-2 days with episodes of vomiting and “the flu”. They cared for him throughout the day, while father was at work. Dad returned home, ate dinner with his parents, thinking the child was still asleep in his bedroom, and later checked on him around 10 PM finding him cold and rigid. The child had a garbage can that was half full of stomach contents next to his bed. He was pronounced dead upon arrival of EMS. This teenager had a history of Type 1 diabetes.
When questioning the family, they assumed he had been resting in his bed for the past 24 hours and did not check on him. His blood sugar monitor was downloaded, and no blood sugar readings were recorded for the past 4 days. Grandparents and father are unsure when the last dose of insulin was injected. He normally cared for himself and managed his diabetes without support from the adults in his home.
The child’s parents were divorced, and the parents shared joint custody of the child. While in dad’s care, elderly grandparents also assisted, but were not well versed in Type 1 diabetes education. The child performed all his insulin injections, blood sugar checks, and counting of carbohydrates independently. Medical records documented he had missed several doses of insulin in the past with episodes of diabetic ketoacidosis (DKA), resulting in multiple hospitalizations. He seemed to do better while in the care of his mother who supervised him daily or physically gave him his long-acting insulin.
Autopsy revealed an A1c of 13.5. He died from metabolic acidosis with a pH of 7.22. Ketones in his urine were +4. Sodium bicarbonate was 12. Potassium was elevated to 5.7 and other electrolytes were abnormal.
If parents or grandparents had intervened at an earlier time, the child would more likely than not have survived this episode of DKA. Symptoms of metabolic acidosis include, but are not limited to:
- Rapid breathing
- Tired
- Confused
- “fruity” smelling breath
PEDIATRIC ENDOCRINE EMERGENCIES
Pediatric endocrine emergencies require prompt diagnosis and management to prevent cerebral dysfunction and death. There remain four common types of metabolic emergencies in pediatrics which include hypocalcemia, hypoglycemia, adrenal insufficiency, and metabolic acidosis which includes the most common and well known, diabetic ketoacidosis (DKA). We will review these abnormalities as they are commonly missed diagnosis in the medical community.
Hypocalcemia
Hypocalcemia is defined as a serum calcium concentration of less than 7.5mg/dL in neonates, and 8.5 mg/dL in children over age one. Low calcium concentration results from inadequate dietary intake and/or absorption from the gastrointestinal track primarily resulting in an active mobilization of calcium across the renal system, affecting parathyroid hormone (PTH). The most common causes include deficiency of vitamin D and magnesium, along with decreased secretion of PTH.
Prevention includes daily calcium intake of at least 600-1,200 mg/day. In most children and infants this is provided with milk and green vegetables. Deficiencies in sunlight can ultimately cause malabsorption of vitamin D, and therefore predispose the child to hypocalcemia. Children more at risk include premature infants, children with congenital heart defects, and other syndromes and autoimmune deficiencies.
Symptoms of low calcium include:
- Poor feeding
- Lethargy
- Seizures
- Muscle spasms (Tetany)
- Irritability
- EKG may show prolonged QT interval
Treatment includes IV administration of calcium gluconate or daily oral calcium therapy. Treatment may include treating the cause, such as vitamin D or magnesium deficiencies.
Hypoglycemia
Hypoglycemia is a blood glucose concentration of less than 50 mg/dL. This typically results from hyperinsulinemia in which a child produces more insulin than the body needs, resulting in a low blood glucose. There are some childhood syndromes which affect counter regulatory hormones resulting in glucose deficiencies. Low blood sugar can also be from feeding abnormalities, malabsorption, or anorexia. Newborns are at greatest risk for low blood sugar, especially premature infants. Close monitoring is necessary for these age groups and these rare childhood syndromes.
Symptoms of low blood sugar include:
- Tremors
- Anxiety
- Nausea and vomiting
- Weakness
- Confusion
- Fatigue
- Headaches
If hypoglycemia is identified, other laboratory tests should be checked including liver function, electrolytes, and bicarbonate to rule out any other abnormalities. Certain toxins can also affect blood sugar and should be on a provider’s differential if another workup is negative.
Treatment includes prompt glucose infusion. Typically, glucose is added to IV fluids for patients in the hospital setting to prevent episodes of hypoglycemia.
Adrenal Insufficiency
Primary congenital adrenal insufficiency is caused by several abnormalities which include, but not limited to, congenital adrenal hyperplasia (CAH), and autoimmune disease called Addison’s disease. Adrenal insufficiency is more common in females. Renal insufficiency results and electrolyte imbalances, blood volume, blood pressure, and serum glucose. These episodes are typically affected in times of stress and often result in greater outcomes if not promptly treated.
Symptoms of adrenal insufficiency include:
- Cold clammy skin
- Weakness
- Vomiting
- Salt cravings
- Weight loss
- Dehydration
- Hyperpigmentation of the gums
If adrenal insufficiency is suspected, a comprehensive metabolic panel should be obtained and if CAH is suspected, 17–hydroxyprogesterone should be obtained. If hypopituitarism (inactive pituitary gland) is on the differential, a free T3 and gonadal hormones should be obtained.
Treatment for adrenal insufficiency, also known as “adrenal crisis, ” requires a rapid fluid bolus to enhance tissue perfusion and restore depletion of fluid volume. Stress doses of steroids should be given to prevent grave consequences.
Metabolic Acidosis
Most common type of metabolic acidosis results in diabetic ketoacidosis (DKA) in children specifically suffering from Type 1 diabetes. Metabolic acidosis results and low bicarbonate and an abnormal accumulation of acid in the bloodstream. The body is unable to compensate, with no buffering mechanisms resulting in a low pH below 7.35. Low pH warrants prompt treatment to prevent coma or death. Electrolyte abnormalities, such as potassium imbalance, are also common as the body tends to compensate. An Increased anion gap is also common as the body attempts to counterbalance. The excessive accumulation of ketones in the body leads to an excess production of acid. The body is unable to compensate, resulting in an imbalance of electrolytes. DKA occurs when there remain insulin deficiencies, typically caused by medication noncompliance, illness and/or infection or new diagnosis. Thereafter, elevated blood sugar and elevated potassium occur because of dehydration. With the increased stress to the body the liver, which stores extra glucose, breaks down and thereby worsening the high blood sugar. Cortisol, or stress on the body, also hinders the body’s ability to compensate appropriately.
Symptoms of metabolic acidosis include:
- Fruity smelling breath
- Persistent vomiting
- Rapid breathing (hyperventilation)
- Warm and flushed skin
- Abdominal pain
- Headaches
The symptoms should be addressed quickly and definitively. If not properly treated can result in seizures, coma, or even death.
Treatment of DKA includes volume depletion correction preferably with IV fluids, replacing proper electrolytes. Sodium bicarbonate may on rare occasions be necessary. DKA in children is often treated with continuous insulin infusion and it is not recommended to bolus insulin in this group of patients (different from adult Type 2 diabetics). Insulin needs to be closely titrated paying special attention to serum electrolytes and replacing electrolytes cautiously as needed. Rapid rate of simply intravenous fluids can result in abnormal serum osmolality, and risk cerebral fluid shift and cerebral edema so slow and steady rehydration is the recommended in caring for children in DKA.
There is a condition like DKA called hyperglycemic hyperosmolar syndrome (HHS). This occurs very rarely in children; however, it should be on a provider’s differential. Symptoms include serum glucose greater than 600 mg/dL, urine osmolality greater than 330 mOsm/kg, with absent ketones and acidosis. Unlike DKA, serum bicarbonate is greater than 15 mEq/L. This condition has a high mortality rate and there remains no known treatments. A rapid fluid bolus is recommended. Insulin is typically not needed as there are no ketones. HHS appears more often in type II diabetics and instances of rhabdomyolysis.
Pediatric endocrine metabolic emergencies can be tricky to diagnosis in an acute setting. Proper diagnosis and management are key and often, these patients require a full workup to narrow the exact underlying cause. Educating the family and friends of these patients can prevent dire consequences.
References:
“Etiology of Hypocalcemia in Infants and Children.” UpToDate, www.uptodate.com/contents/etiology-of-hypocalcemia-in-infants-and-children?search=hypocalcemia%20pediatric&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2. Accessed 18 Oct. 2023.
Professional, Cleveland Clinic medical. “Diabetes-Related Ketoacidosis (DKA): Symptoms, Treatment & Causes.” Cleveland Clinic, my.clevelandclinic.org/health/diseases/21945-diabetic-ketoacidosis-dka. Accessed 18 Oct. 2023.
Kim SY. Endocrine and metabolic emergencies in children: hypocalcemia, hypoglycemia, adrenal insufficiency, and metabolic acidosis including diabetic ketoacidosis. Ann Pediatr Endocrinol Metab. 2015 Dec;20(4):179-86. doi: 10.6065/apem.2015.20.4.179. Epub 2015 Dec 31. PMID: 26817004; PMCID: PMC4722156.
Zeitler, Phil, et al. “Hyperglycemia Hyperosmolar Syndrome in Children: Pathophysiological Considerations and Suggested Guidelines for Treatment.” Https://Www.Jpeds.Com/Article/S0022-3476%2810%2900808-5/Fulltext. Accessed 18 Oct. 2023.
