Endocrine Disorders

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Chapter 33 Endocrine Disorders

Robert E. Sapien, MD, FAAP

1 Why are children susceptible to hypoglycemia? What glucose level is considered hypoglycemia? How do you correct it?

Children are susceptible because:

image Glycogen stores are small.

image Glucose utilization is high because of increased metabolic demands.

image Fewer gluconeogenesis precursors are generated because of less fat and muscle mass.

image A child’s developing brain needs a constant source of substrate.

The level at which to treat is controversial, but most clinicians treat at a level < 60 mg/dL. The treatment for infants is typically at a level < 40 mg/dL. Treat infants with D10W (10% dextrose in water), 2–4 mL/kg body weight; for 1- to 8-year-olds, D25W (25% dextrose in water), 2–4 mL/kg; older children and adolescents, D50W (50% dextrose in water) ampule.

Reid SR, Losek JD: Hypoglycemia in infants and children. Pediatr Emerg Med Rep 5:23–30, 2000.

2 At what point should diagnostic tests for hypoglycemia be obtained? What would they be?

The tests should be obtained at first presentation of the hypoglycemic child to the emergency department (ED) (at triage)—but only if they can be obtained quickly. A bedside glucose test is essential because a delay in treatment can be neurologically devastating. A diagnostic workup is indicated for infants and children in whom the hypoglycemia has no explanation (i.e., oral hypoglycemic ingestion, sepsis, poor intake, anorexia in a diabetic child who received insulin). The tests include plasma glucose, electrolytes, lactate, pyruvate, keto hydroxybutyrate, acetoacetate, insulin, glucagon, cortisol, growth hormone, free fatty acids, alanine, glycerol, and urinalysis, including reducing substances.

KEY POINTS: HYPOGLYCEMIA

1 Rapid identification is essential.

2 Children are susceptible because of small glycogen stores and high glucose utilization.

3 Treat hypoglycemia as follows: for infants (glucose level < 40 mg/dL), D10W, 2–4 mL/kg; for 1- to 8-year-olds (glucose level < 60 mg/dL), D25W, 2–4 mL/kg; for older children and adolescents, D50W ampule.

3 What is the most common cause of hyperthyroidism in children?

Graves’ disease is the most common etiology. Children at risk include females (female to male ratio, 5:1), those with familial autoimmune diseases (e.g., diabetes, adrenal insufficiency), and those from families with a history of thyroid disease.

Sills IN: Hyperthyroidism. Pediatr Rev 15:417–420, 1994.

McKeown NJ, Tews MC, Gossain VV, Shah S: Hyperthyroidism. Emerg Med Clin North Am 23:649–667, 2005.

4 Presenting symptoms of hyperthyroidism are commonly attributed to which other body systems or disorders?

Symptoms from hyperthyroidism are commonly confused with other conditions. For example, cardiac disorders (palpitations, sinus tachycardia) and psychological problems (school difficulties, labile emotions, hyperactivity) mimic hyperthyroidism.

Sills IN: Hyperthyroidism. Pediatr Rev 15:417–420, 1994.

McKeown NJ, Tews MC, Gossain VV, Shah S: Hyperthyroidism. Emerg Med Clin North Am 23:649–667, 2005.

5 What is thyroid storm? What is the treatment?

The symptoms may be nonspecific (fever, tachycardia, agitation, nausea, vomiting), making the diagnosis difficult unless there is a clear hyperthyroid history. The three systems most affected include the cardiovascular, gastrointestinal, and central nervous systems. Supportive care is the first step in management (oxygen, IV fluids, acetaminophen for fever). This should be followed by an IV β-blocker for the cardiac effects, and routine hyperthyroid treatments (propylthiouracil, iodine) in high doses.

Sills IN: Hyperthyroidism. Pediatr Rev 15:417–420, 1994.

McKeown NJ, Tews MC, Gossain VV, Shah S: Hyperthyroidism. Emerg Med Clin North Am 23:649–667, 2005.

6 If congenital hypothyroidism is missed in the neonatal period, when will signs/symptoms present?

Patients will present with symptoms at 6–12 weeks of age. The first signs and symptoms are constipation, a large posterior fontanel, poor feeding, jaundice, and hypothermia.

Fisher DA: Hypothyroidism. Pediatr Rev 15:227–232, 1994.

Foley TP: Hypothyroidism. Pediatr Rev 25:94–99, 2004.

7 How do the adrenal glands respond to stress? What are examples of such body stressors? What do children with adrenal insufficiency and children taking long-term exogenous glucocorticoid therapy have in common?

The adrenal glands respond to stress by secreting increased amounts of glucocorticoids and mineralocorticoids. Physiologic stresses include surgery, trauma, and infection. All of these children require stress dose replacement of glucocorticoids and mineralocorticoids because their adrenal glands cannot respond to such stressors.

Oberlin JM, Rogers WM, Fenton CL: Endocrine emergencies: Recognizing signs and symptoms. Pediatr Ann 34:870–877, 2005.

Spahn JD, Kamada AK: Special considerations in the use of glucocorticoids in children. Pediatr Rev 16:266–272, 1995.

8 What signs and symptoms are likely in children with acute adrenal insufficiency?

Signs and symptoms are due to failure of the adrenals to respond to a stress, and they are fairly nonspecific. They include dehydration with hypovolemic shock, hypoglycemia, hyponatremia, hyperkalemia, fever, abdominal pain, and lethargy.

Kappy MS, Bajaj L: Recognition and treatment of endocrine/metabolic emergencies in children. Part I. Adv Pediatr 49:245–272, 2002.

Oberlin JM, Rogers WM, Fenton CL: Endocrine emergencies: Recognizing signs and symptoms. Pediatr Ann 34:870–877, 2005.

9 What is the emergency treatment for acute adrenal insufficiency?

Much of the initial stabilization is supportive care. The treatment includes:

image Fluid resuscitation with normal saline (500 mL/m2) for shock and dehydration

image Correction of the hypoglycemia with D25W

image Hydrocortisone, 2–3 mg/kg via IV route

image Correction of the hyperkalemia (sodium polystyrene sulfonate, glucose/insulin, calcium gluconate, sodium bicarbonate)

image Monitoring of vital signs, cardiac rhythm, perfusion, glucose, and electrolytes

August GP: Treatment of adrenocortical insufficiency. Pediatr Rev 18:59–62, 1997.

Kappy MS, Bajaj L: Recognition and treatment of endocrine/metabolic emergencies in children. Part I. Arch Pediatr 49:245–272, 2002.

10 What is the most common cause of amenorrhea in adolescents?

Pregnancy is the most common cause. Other causes include oral contraceptive use, pituitary infarctions, uterine synechiae in postpartum adolescents, outflow obstruction (imperforate hymen, vaginal or uterine agenesis), and hypoestrogenic states (anorexia, athletics).

Braverman PK, Sondeimer SJ: Menstrual disorders. Pediatr Rev 18:17–25, 1997.

Hillard PJ, Deitch HR: Menstrual disorders of the college age female. Pediatr Clin North Am 52:179–107, 2005.

11 What endocrine conditions are associated with dysfunctional uterine bleeding?

Dysfunctional uterine bleeding is associated with immaturity of the hypothalamic–pituitary axis, prolactinomas, thyroid tumors, type 2 diabetes, and adrenal hyperplasia or tumors.

12 Why is it difficult to make the diagnosis of type 1 diabetes in early childhood?

The diagnosis is difficult to make because the classic symptoms of polydipsia and polyuria are not easily discernible. The very young, therefore, are more likely to present with diabetic ketoacidosis (DKA) initially.

Mallare JT, Cordice CC, Ryan BA, et al: Identifying risk factors for the development of diabetic ketoacidosis in new onset type 1 diabetes mellitus. Clin Pediatr 42:591–597, 2003.

Plotnick L: Insulin-dependent diabetes mellitus. Pediatr Rev 15:137–148, 1994.

13 What questions or clinical findings may help you consider type 1 diabetes in your differential diagnosis?

Type 1 diabetes should be ruled out in children who are clinically dehydrated yet continue with urine output. Information regarding the number of wet diapers per day, nocturnal incontinence, or leaving the classroom often to urinate may help you hone in on the diagnosis. Children with weight loss and a history of vague abdominal pain should be considered as possibly having type 1 diabetes.

14 What are acute complications of type 1 diabetes?

DKA and hypoglycemia are common, recurrent, critical issues that can occur. They are related to socioeconomic factors, psychiatric problems, blood glucose control, duration of the disease, and the child’s age.

Kappy MS, Bajaj L: Recognition and treatment of endocrine/metabolic emergencies in children. Part I. Arch Pediatr 49:245–272, 2002.

Rewers A, Chase P, Mackensie T, et al: Predictors of acute complications in children with type 1 diabetes. JAMA 287:2511–2518, 2002.

15 What usually leads to DKA?

Insulin underutilization is the usual factor leading to DKA. As DKA starts, the child usually is anorexic and nauseated, and begins to vomit. Parents or the patient may stop administering insulin even though the child is hyperglycemic, hence worsening the condition. Acute infections (viral gastroenteritis, appendicitis, bacterial infection), psychologically stressful situations, medical nonadherence, and inflammatory processes can also lead to DKA. Signs and symptoms include dehydration, abdominal pain, tachypnea, nausea, vomiting, listlessness, coma, fruity odor, ketonuria(emia), hyperglycemia(uria), measured hyponatremia and intravascular hyperkalemia (despite total body depletion).

Wolfson AB (ed): Clinical Practice of Emergency Medicine, 4th ed. Philadelphia, Lippincott Williams & Wilkins, 2005.

16 Is there only one level of DKA severity?

No. DKA can be mild, moderate, or severe. Mild DKA is merely hyperglycemia with ketosis as demonstrated by urinalysis. Children with mild DKA have normal mental status and respiratory status. They are good candidates for outpatient therapy as long as they can keep themselves hydrated orally.

  pH HCO3
Moderate 7.2–7.3 15
Severe 7.1 10

The degree of DKA is not dependent on the extent of hyperglycemia. The glucose level is always elevated with DKA, but there is a common misconception that the glucose has to be severely elevated for DKA to exist.

17 Of what do children and adolescents with type 1 diabetes die?

Children with type 1 diabetes in DKA die of cerebral edema (60–80% mortality rate), which is the final pathway in over half of hospital deaths and 30% of home deaths. Cerebral edema is unpredictable and probably multifactorial. It seems to be associated with high initial blood urea nitrogen level, lower partial pressure of arterial carbon dioxide, more severe acidosis (lower bicarbonate and pH), administration of bicarbonate, and inadequate increase in serum sodium concentration. Rates of fluid or sodium administration do not affect the risk of cerebral edema.

Edge JA, Adams M, Dunger DB: Causes of death in children with insulin-dependent diabetes 1990–96. Arch Dis Child 81:318–323, 1999.

Glaser N, Barnett MB, McCaslin I, et al: Risk factors for cerebral edema in children with diabetic ketoacidosis N Engl J Med 344:264–269, 2001.

Wolfson AB (ed): Clinical Practice of Emergency Medicine, 4th ed. Philadelphia, Lippincott Williams & Wilkins, 2005.

18 Is there a standard treatment for DKA?

There are many protocols for DKA management. In fact, agreement is lacking among specialists (pediatric intensivists, endocrinologists, and emergency physicians). Keeping to a standard treatment protocol helps ensure consistent care. The key issues are fluid replacement, acid correction, and glucose and electrolyte adjustment. Attention to all of these is necessary in the approach to the child with DKA.

Initially, fluid replacement with an isotonic solution (normal saline) at 5–10 mL/kg in the first hour is recommended. Fluid deficits are replaced over the subsequent 24–48 hours (if 10% dehydration is assumed, then 100 mL/kg) in addition to maintenance fluids. With hydration, the glucose level usually begins to fall, and the acidosis begins to correct as the serum ketones metabolize, producing bicarbonate. Careful monitoring of potassium, sodium, and phosphate is also indicated. There is total-body potassium depletion, which is initially masked, and extracellular potassium levels are generally normal to high. As the acidosis corrects, potassium moves intracellularly, and the serum potassium level begins to fall, more accurately reflecting the total-body depletion.

Insulin may be administered as an IV drip (0.05–0.1 U/kg/h), but this should be done carefully as hydration also lowers glucose levels. Subcutaneous insulin administration is not the route of choice in DKA. Glucose should be decreased no faster than 50–100 mg/dL/h. Add dextrose when the serum level is 300 gm/dL, but do not discontinue the insulin. The insulin also helps drive potassium intracellularly, compounding the hypokalemia. Bicarbonate administration increases the risk of cerebral edema and has not been shown to improve outcomes.

Glaser NS, Kuppermann N, Yee CKJ: Variation in the management of pediatric diabetic ketoacidosis by specialty training. Arch Pediatr Adolesc Med 151:1125–1132, 1997.

Green SM, Rothrock SG, Ho JD, et al: Failure of adjunctive bicarbonate to improve outcome in severe pediatric diabetic ketoacidosis. Ann Emerg Med 31:41–48, 1998.

Wolfson, AB (ed): Clinical Practice of Emergency Medicine, 4th ed. Philadelphia, Lippincott Williams & Wilkins, 2005.

19 How is time a factor in the treatment of DKA?

A philosophy to follow in treating a child with DKA is to remember that there are many aspects of DKA, which took time to develop. Taking sufficient time to correct them avoids many of the complications of treatment. With judicious fluid resuscitation, careful monitoring, and frequent reassessment, you should see steady improvement. Do not expect or strive for drastic, rapid improvement. Treatment should be judged as successful if the child does not further deteriorate and the clinical state steadily improves (lower respiratory rate, more alert mental status, and electrolytes approaching normal).

Lavin N: Manual of Endocrinology and Metabolism, 2nd ed. Boston, Little, Brown & Company, 1994.

Sperling MA: Pediatric Endocrinology. Philadelphia, W.B. Saunders, 2002.

20 What signs and symptoms should make you consider the diagnosis of type 2 diabetes mellitus?

Obesity (over 85% of patients are overweight); acanthosis nigricans (seen in over 85% of patients), a sign of insulin resistance; nonketotic hyperglycemia; glycosuria; weight loss (less common than with type 1 diabetes); polyuria and polydipsia (less common than with type 1 diabetes); rarely DKA; and ketonuria in 25% of patients. Type 2 diabetes mellitus is becoming more prevalent among children between 10 and 21 years old.

Nesmith JD: Type 2 diabetes mellitus in children and adolescents. Pediatr Rev 22:147–152, 2001.

American Diabetes Association. Available at www.diabetes.org.

21 What is the probable abnormality in type 2 diabetes mellitus?

The probable abnormality is insulin resistance. This develops over a long period of time and results in a hyperinsulinemic condition. It can lead to dysfunctional uterine bleeding, hypertension, polycystic ovary disease, and acanthosis nigricans. The islets of Langerhans eventually become unable to maintain this level of insulin production, which leads to a relative insulin deficiency and hence hyperglycemia.

Nesmith JD: Type 2 diabetes mellitus in children and adolescents. Pediatr Rev 22:147–152, 2001.

KEY POINTS: DIABETES MELLITUS

1 Children in DKA need skillful correction of electrolyte, metabolic, and glucose abnormalities as well as careful clinical monitoring and reassessment.

2 For children in DKA, try to reduce glucose level at a rate of 50–100 mg/dL/h.

3 When treating DKA, add glucose when the serum level is < 300 mg/dL, but do not discontinue insulin.

22 True or false: DKA is the only acute metabolic abnormality that occurs in children with type 2 diabetes mellitus

False. There are reported deaths from hyperglycemic hyperosmolar states. A hyperglycemic hyperosmolar state is described as hyperglycemia with glucose level > 600 mg/dL, small ketonuria, low or no ketonemia, stupor/coma, serum carbon dioxide level > 15 mmol/L, and serum osmols > 320 mOsm/kg. Just as in DKA, cerebral edema is a grave complication that can occur from the hyperglycemic, hyperosmolar state of type 2 diabetes.

Morales AE, Rosenbloom AL: Death caused by hyperglycemic hyperosmolar state at the onset of type 2 diabetes. J Pediatr 144:270–273, 2004.

American Diabetes Association. Available at www.diabetes.org.

23 Can a child be dehydrated yet still produce copious amounts of urine?

Yes, this is common with diabetes insipidus. Dilute urine produced by an otherwise clinically dehydrated child, polydipsia, polyuria, fever, irritability, poor feeding and weight gain, constipation, and hypernatremia are presenting signs and symptoms of diabetes insipidus. In severe presentations, weak pulse, poor skin turgor, seizure, and hypotension may also occur.

Saborio P, Tipton GA, Chan JCM: Diabetes insipidus. Pediatr Rev 21:122–129, 2000.

24 Congenital hypothyroidism is familiar to pediatricians, but can newborns be hyperthyroid?

Indeed they can. In fact, substantial mortality is associated with neonatal thyrotoxicosis. Neonatal thyrotoxicosis presents in the first 2 weeks of life in infants born to hyperthyroid mothers. Symptoms include irritability, sweating, weight loss, poor feeding, vomiting, and diarrhea. Signs are typical of hyperthyroidism, such as exophthalmos, goiter, hyperthermia, tachycardia, jaundice, hepatomegaly, and even cardiac failure.

Wolfson AB (ed): Clinical Practice of Emergency Medicine, 4th ed. Philadelphia, Lippincott Williams & Wilkins, 2005.

25 What endocrine comorbidities might be present with childhood obesity?

The prevalence of childhood obesity is skyrocketing among our youth and stresses all body systems. Obesity contributes and may even lead to several endocrine abnormalities. They include advanced physical maturation, type 2 diabetes, and polycystic ovary disease. Polycystic ovary disease probably is a result of insulin resistance and elevated levels of androgens leading to obesity, infertility, hirsutism, and menstrual dysfunction are common.

Perkin RM: The epidemic of pediatric obesity: Clinical implications for the ED physician. Pediatr Emerg Med Rep 9:65–80, 2004.

26 What is the most common form of congenital adrenal hyperplasia presenting in infancy? What is the incidence of this type?

The most common form of congenital adrenal hyperplasia (CAH) during infancy is 21-hydroxylase deficiency. It accounts for 90% of all cases. Clinical salt wasting develops in 30–70% of patients. The incidence of this type of CAH is 1 in 15,000 live births.

27 When does a child with salt-losing CAH generally present to the ED?

The infant generally presents around 2 weeks of life. Symptoms are usually nonspecific, consisting of poor feeding, lethargy, irritability, vomiting, and poor weight gain. A general examination should be done, as well as a thorough evaluation of the genitals.

28 In the ED, what are the most urgent laboratory studies to perform if CAH is suspected?

Check serum electrolytes and glucose. In salt-losing CAH, hyperkalemia and hyponatremia are typically seen; the glucose level can be normal or low. Metabolic acidosis is usually present. Infants with CAH tolerate hyperkalemia better than do other infants and children.

29 How is this type of CAH treated in the ED?

If dehydration is present, volume expansion with normal saline should begin promptly. Hydrocortisone, 25 mg, should be given immediately via IV bolus. If hypoglycemia is present, a dextrose bolus will be necessary. Hyperkalemia needs to be addressed.

Agus MSD: Endocrine emergencies. In Fleisher G, Ludwig S, Henretig F, et al (eds). The Textbook of Pediatric Emergency Medicine, 5th ed. Philadelphia, Lippincott Williams & Wilkins, 2006, pp 1167–1192.

30 What are the most common symptoms of pheochromocytoma?

Headache, palpitations, and excessive sweating. The headache is usually pounding and severe, and palpitations may be associated with tachycardia. Other symptoms include tremor, fatigue, chest or abdominal pain, and flushing.

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