Pediatric Annals

Endocrine Emergencies: Recognizing Signs and Symptoms

John M Oberlin, MD; William M Rogers, MD; Cydney L Fenton, MD

Abstract

A primary care physician rarely may encounter the pediatric patient with an acute endocrine disorder in an office-based setting, as the serious nature of such an illness makes these patients more likely to present to an emergency department. Still, the general pediatrician or family practitioner should be prepared to recognize such situations. In general, these emergencies present with a wide variety of nonspecific signs and symptoms, making the diagnosis somewhat elusive.

Timely clinical recognition alone poses one of the biggest challenges in the acute management of metabolic derangements. Once an endocrine or metabolic disorder is suspected, the primary care physician should be prepared to respond appropriately. While an approach to managing endocrine disorders has been well described for the emergency department,1,2 this has not yet been adapted for an outpatient office-based setting. The Table (see page 872) summarizes the diagnosis and management of 10 pediatric endocrine emergencies that may present in the office setting.

This article focuses on four of these conditions - diabetic ketoacidosis, hypoglycemia, adrenal insufficiency, and hypocalcemia - providing a basic clinical approach within the scope of practice of an ambulatory office-based setting.

An office-based practice has limitations in managing medical emergencies. Depending on the laboratory resources available, an ambulatory care clinic may not be able to make a critical diagnosis, nor will it likely have the medications necessary to treat the condition in question specifically. Therefore, the most important principle for handling endocrine emergencies in a primary care clinic is to stabilize the patient's vital signs through nonspecific treatment measures (including pediatric advanced life support, or PALS) while arranging for transfer to an emergency medical faculty. When possible, pertinent laboratory samples should be obtained prior to treatment and sent with the patient to the next medical faculty, to aid in the future diagnostic evaluation However, if the patient's condition is severe, then prompt medical intervention should take priority over specimen collection.

DIABETIC KETOACIDOSIS

Case Description

A previously healthy 10-year-old boy presents to the office with a 3-day history of vague headache and abdominal pain associated with nausea and vomiting several times a day. The child's mother notes that he has seemed to be excessively thirsty, but he has had generous urine output for the past several weeks. In fact, he has recently developed secondary enuresis. On exam, he appears dehydrated, with a respiratory rate of 40 breaths per minute and a fruity odor to his breath. A finger stick reveals a blood glucose level of 450 mg/dL.

Discussion

The above case represents a child with new-onset type 1 diabetes, presenting with diabetic ketoacidosis (DKA). The clinical suspicion can be based on the presence of hyperglycemia (random blood glucose greater than 200 mg/dL), relative ketosis ("fruity odor" on breath), and clinical metabolic acidosis (tachypnea/Kussmaul respirations and dehydrated appearance). It is not an unreasonable prospect that a child with DKA could be managed effectively in an outpatient clinic. In fact, the American Diabetes Association acknowledges the possibility that mild to moderate DKA can be managed safely in an environment away from a medical facility (such as while camping), as long as blood glucose and urine ketones are monitored adequately and insulin and rehydration fluids (oral or intravenous) are available.3 Specific criteria for identifying patients in whom outpatient management of DKA would be appropriate have been studied4 and subsequent protocols proposed.5

In the office-based setting, the most appropriate early management of DKA comprises simple intravascular fluid replacement This typically involves intravenous administration of 10 to 20 mL/kg of isotonic crystalloid (normal saline or Ringer's lactate) over 1 hour. It is important that only isotonic solution is used for intravenous…

A primary care physician rarely may encounter the pediatric patient with an acute endocrine disorder in an office-based setting, as the serious nature of such an illness makes these patients more likely to present to an emergency department. Still, the general pediatrician or family practitioner should be prepared to recognize such situations. In general, these emergencies present with a wide variety of nonspecific signs and symptoms, making the diagnosis somewhat elusive.

Timely clinical recognition alone poses one of the biggest challenges in the acute management of metabolic derangements. Once an endocrine or metabolic disorder is suspected, the primary care physician should be prepared to respond appropriately. While an approach to managing endocrine disorders has been well described for the emergency department,1,2 this has not yet been adapted for an outpatient office-based setting. The Table (see page 872) summarizes the diagnosis and management of 10 pediatric endocrine emergencies that may present in the office setting.

This article focuses on four of these conditions - diabetic ketoacidosis, hypoglycemia, adrenal insufficiency, and hypocalcemia - providing a basic clinical approach within the scope of practice of an ambulatory office-based setting.

An office-based practice has limitations in managing medical emergencies. Depending on the laboratory resources available, an ambulatory care clinic may not be able to make a critical diagnosis, nor will it likely have the medications necessary to treat the condition in question specifically. Therefore, the most important principle for handling endocrine emergencies in a primary care clinic is to stabilize the patient's vital signs through nonspecific treatment measures (including pediatric advanced life support, or PALS) while arranging for transfer to an emergency medical faculty. When possible, pertinent laboratory samples should be obtained prior to treatment and sent with the patient to the next medical faculty, to aid in the future diagnostic evaluation However, if the patient's condition is severe, then prompt medical intervention should take priority over specimen collection.

DIABETIC KETOACIDOSIS

Case Description

A previously healthy 10-year-old boy presents to the office with a 3-day history of vague headache and abdominal pain associated with nausea and vomiting several times a day. The child's mother notes that he has seemed to be excessively thirsty, but he has had generous urine output for the past several weeks. In fact, he has recently developed secondary enuresis. On exam, he appears dehydrated, with a respiratory rate of 40 breaths per minute and a fruity odor to his breath. A finger stick reveals a blood glucose level of 450 mg/dL.

Discussion

The above case represents a child with new-onset type 1 diabetes, presenting with diabetic ketoacidosis (DKA). The clinical suspicion can be based on the presence of hyperglycemia (random blood glucose greater than 200 mg/dL), relative ketosis ("fruity odor" on breath), and clinical metabolic acidosis (tachypnea/Kussmaul respirations and dehydrated appearance). It is not an unreasonable prospect that a child with DKA could be managed effectively in an outpatient clinic. In fact, the American Diabetes Association acknowledges the possibility that mild to moderate DKA can be managed safely in an environment away from a medical facility (such as while camping), as long as blood glucose and urine ketones are monitored adequately and insulin and rehydration fluids (oral or intravenous) are available.3 Specific criteria for identifying patients in whom outpatient management of DKA would be appropriate have been studied4 and subsequent protocols proposed.5

In the office-based setting, the most appropriate early management of DKA comprises simple intravascular fluid replacement This typically involves intravenous administration of 10 to 20 mL/kg of isotonic crystalloid (normal saline or Ringer's lactate) over 1 hour. It is important that only isotonic solution is used for intravenous fluid to avoid cerebral edema. Oral rehydration therapy, such as sports drinks or other oral solutions rich in electrolytes but with limited sugar (similar to World Health Organization recommendations), may be considered in a known patient with diabetes who has mild DKA and is tolerating oral intake.

Table

TABLE.Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

Table

TABLE.Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

Table

TABLE.Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

Once the diagnosis of DKA is established, the next appropriate step is administration of insulin. In an officebased setting, it is unlikely that insulin will be stocked and available, so it is prudent for this to wait until definitive medical care is obtainable. If the patient is known to have a diabetes diagnosis, intermittent doses of subcutaneous insulin is appropriate. For regular insulin, the dose (0.1 units/kg) may be repeated every 3 to 4 hours as needed. For insulin aspart or insulin lispro, the dose (0.1 units/kg) may be repeated every 1 to 2 hours.6,7 Overall, the goal for insulin should be to lower blood glucose by 50 to 100 mg/dL per hour. Therefore, it is imperative that blood glucose measurement be repeated every hour while awaiting medical transfer.

Other pertinent laboratory samples include urine for urinalysis and blood for comprehensive metabolic profile (electrolytes, BUN, creatinine, glucose), serum ketones, and blood gas measurement Patients with mild DKA can be considered for outpatient management alone without medical transfer only if symptoms such as vomiting resolve and blood glucose has dropped below 200 mg/dL within 3 to 4 hours of intervention.

HYPOGLYCEMIA

Case Description

A 3-year-old girl is brought to the office by her mother for an acute appointment. The mother notes she had great difficulty awakening the girl, who has since been complaining of headache and nausea. On arrival, the girl's heart rate is 140 beats per minute, and her blood pressure is normal. She appears diaphoretic, drowsy, and confused during questioning. A random finger stick reveals a blood glucose level of 40 mg/dL.

Table

TABLE.Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

Discussion

This case represents a child with symptomatic hypoglycemia. Symptoms usually occur when blood glucose falls below 60 to 70 mg/dL, although for children with diabetes, symptoms may occur at higher blood sugar levels. In the officebased setting, the goal of early nonspecific treatment of hypoglycemia is to return the blood glucose level to normal rapidly. Regardless of the underlying etiology, this can be accomplished by giving glucose. For a patient with only mild symptoms and who can tolerate feeding, oral glucose in the form of fruit juice or dextrose tablets is an appropriate initial intervention.

For patients with more profound hypoglycemia resulting in significant mental status changes that precludes oral glucose administration, intravenous infusion of dextrose is more appropriate. An initial IV glucose bolus of 0.25 g/kg should be given; this is equivalent to giving D10 at 2.5 mL/kg or D25 at 1 mL/kg. After glucose administration, blood glucose measurements should be repeated every 30 minutes. If continuous intravenous infusion capability is available, continuous glucose infusion of 5 to 20 mg/kg per minute is recommended to maintain blood glucose levels in the normal range.

Additional nonspecific treatment of hypoglycemia includes the use of glucagon at a dose of 0.03 mg/kg. This is especially useful if excess insulin is suspected as the cause (ie, insulin-dependent diabetic patients). However, glucagon is contraindicated if the hypoglycemia is suspected to be the result of sulfonylurea medication. Another option includes intravenous glucocorticoid (eg, hydrocortisone 25 to 100 mg/m2/day or 1 to 5 mg/kg/day) if there is a clinical suspicion of adrenal insufficiency. Outpatient clinics probably will not have such medications readily available, so prompt medical transfer is necessary for patients in whom glucose supplementation alone does not resolve their hypoglycemia sufficiently.

A more extensive diagnostic evaluation may be warranted for patients without a known cause for hypoglycemia. In such an instance, the pertinent laboratory samples are vastly more useful if obtained during the hypoglycemic episode. The timing for the collection of these samples should be determined by the severity of the patient's condition. The recommended laboratory evaluation includes blood for comprehensive metabolic profile (serum electrolytes, BUN, creatinine, calcium, phosphorus, and magnesium), hepatic function panel (liver enzymes/transaminases, alkaline phosphatase, bilirubin), insulin level, serum ketones, growth hormone, cortisol, C-peptide, and serum amino acids, as well as urine for ketones, urine amino acids, and urine organic acids. Toxin screening of blood and urine can be done later. While an office-based setting likely will not be able to process these suggested laboratory studies on site, this should not preclude the prompt collection of these specimens to be forwarded to the next medical facility or for later shipping to an outside laboratory.

ADRENAL INSUFFICIENCY

Case Descriptoin

A first-time mother brings her 2-week-old daughter for a well baby visit The baby is a product of a home delivery. The mother is concerned that her baby is not feeding adequately and has not regained her birth weight. Vital signs are significant for hypotension. Dry mucous membranes, sunken fontanel, delayed capillary refill, and clitoromegaly are apparent on physical examination.

Discussion

This case represents a child with symptomatic adrenal crisis as the initial presentation of classic congenital adrenal hyperplasia (CAH). Newborn screening protocols in many states include a 17-hydroxyprogesterone level to test for CAH, facilitating early diagnosis and intervention before clinical manifestations of adrenal insufficiency occur. For states lacking the newborn screen for CAH, the potential still exists for an infant with CAH to present urgently in an outpatient setting. Variable severities of CAH can manifest at a wide range of ages, and multiple other conditions can result in deficiency of adrenal gland function (such as sepsis).

It is important to note that females with classic CAH are identified more easily because of the presence of clitoromegaly or ambiguous genitalia on physical examination, whereas males have no obvious physical exam findings. The signs and symptoms of adrenal insufficiency are nonspecific, leading to a broad differential diagnosis and heralding an extensive evaluation that is beyond the capabilities of most office-based practices.

The most significant clinical feature, and perhaps the most ominous, is the development of decompensated hypovolemic shock. Therefore, the acute management of a patient with adrenal insufficiency should be focused on advanced life support measures for restoring adequate tissue perfusion; specifically, an intravenous bolus of isotonic crystalloid solution (ie, 20 mL/kg of NS or LR), which may be repeated as often as is necessary to maintain a normal blood pressure. Other potential resuscitative medications for acute stabilization, such as epinephrine, should be used according to PALS protocols. Meanwhile, measurement of vital signs and clinical reassessment should be performed frequently until emergency medical personnel arrive.

After the patient is stabilized, pertinent laboratory specimens can be obtained while awaiting medical transport. These include blood for comprehensive metabolic profile, plasma renin, Cortisol, adrenocorticotropic hormone, 17-hydroxyprogesterone (or complete adrenal steroid panel), free thyroxine, and growth hormone, depending on the suspected etiologies. It is important to remember that hypopituitarism may present similarly, necessitating evaluation of all pituitary gland hormones.

Regardless of the etiology, the pathophysiology of adrenal insufficiency results in a classic metabolic profile. Hyponatremia, hyperkalemia, and acidosis are expected due to lack of mineralocorticoid activity. For this reason, the patient is at risk for cardiac dysrhythmias, and electrocardiogram monitoring would be an important adjunct if available. Also, determination of blood glucose by finger stick will identify hypoglycemia from deficient glucocorticoid function and may indicate inclusion of dextrose in intravenous fluids. Otherwise, more specific treatment requires supplementation of corticosteroid with intravenous hydrocortisone (25 to 100 mg/m2 per day or 1 to 5mg/kg per day). It is important to remember that corticosteroids such as dexamethasone, methylprednisolone, or prednisone have glucocorticoid effects only, while hydrocortisone can replace mineralocorticoid function as well.

HYPOCALCEMIA

Case Description

An 8-year-old boy is brought to the office by his parents, who have concerns about muscle cramping. They report that the boy complains of frequent numbness and stiffness in his hands. The nurse reports that the child's hand and wrist began to spasm during blood pressure measurement (Trousseau's sign). On physical examination, the clinician elicits contraction of the mouth, nose, and eye by tapping a finger just anterior to the patient's ear (Chvostek's sign).

Discussion

This case represents a child with symptomatic hypocalcemia. This condition should be suspected by the complaint of tetany and the findings of Chvostek's and Trousseau's signs during physical examination. In the office-based setting, the early nonspecific treatment is targeted at restoration of normal serum calcium levels. Acute calcium replacement for symptomatic hypocalcemia (serum ionized calcium less than 0.8 mmol/L, or total serum calcium less than 7 mg/dL) can be accomplished with intravenous infusion of 10% calcium gluconate or 10% calcium lactate at 1 to 2 mL/kg (100 to 200 mg elemental calcium per kilogram).

It is important to note that dosing for calcium is based on elemental calcium, which may vary according to the different types of calcium used, and should be confirmed prior to administration. Management of asymptomatic patients with mild hypocalcemia (serum ionized calcium between 0.8 and 1.0 mmol/L, or total serum calcium between 7 and 9 mg/dL) involves oral supplementation with 1 gram of calcium carbonate (or calcium lactate) daily.

Hypocalcemia usually results from parathyroid hormone deficiency, vitamin D deficiency, hypomagnesemia, renal disease, or pancreatitis. Therefore, the pertinent laboratory values to collect include blood for a comprehensive metabolic profile, alkaline phosphatase level, serum parathyroid hormone, calcidiol (25-Hydroxyvitamin D3), calcitriol (1,25-Dihydroxy- vitamin D3), amylase and lipase, as well as urine for calcium and creatinine to determine urinary calcium excretion. Hypocalcemia cannot be corrected if underlying hypomagnesemia exists, so it is important to think about magnesium supplementation along with calcium replacement. Also, after the pertinent laboratory samples are collected, it is reasonable to begin vitamin D supplementation (400 IU/day) empirically until the results are completed.

SUMMARY

Endocrine emergencies may present to the pediatric practitioner in the office setting in a variety of forms. Four of the more common pediatric endocrine emergencies (DKA, symptomatic hypoglycemia, adrenal insufficiency, and hypocalcemia) have been discussed here. The recommended approach to a child or adolescent with an endocrine emergency involves recognizing clinical disease, stabilizing the patient with basic and advanced life support intervention, and transferring the patient to a faculty which can provide appropriate definitive care.

REFERENCES

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

2. Kappy MS, Bajaj L. Recognition and treatment of endocrine/metaboEc emergencies in children: Part II. Adv Pediatr. 2003;50:181-214.

3. Kaufman F, Schatz D, Silverstein J; American Diabetes Association. Diabetes care at diabetes camps. Diabetes Care. 2005;28(Suppl 1):S50-S52.

4. Bonadio WA, Gutzeit MF, Losek JD, Smith DS. Outpatient management of diabetic ketoacidosis. Am JDis Child. 1988;142(4):448450.

5. Bonadio WA. Pediatric diabetic ketoacidosis: pathophysiology and potential for outpatient management of selected children. Pediatr Emerg Care. 1992;8(5):287-290.

6. Umpierrez GE, Latif K, Stoever J, et al. Efficacy of subcutaneous insulin lispro versus continuous intravenous regular insulin for the treatment of patients with diabetic ketoacidosis. Am J Med. 2004; 117(5):291-296.

7. Umpierrez GE, Cuervo R, Karabell A, et al. Treatment of diabetic ketoacidosis with subcutaneous insulin aspart. Diabetes Care. 2004;27(8):1873-1878.

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

TABLE.

Diagnosis and Management of Pediatric Endocrine Emergencies

10.3928/0090-4481-20051101-10

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