Pediatric Endocrine Emergencies

Pediatric Endocrine Emergencies

HypoCalcemia

Diabetic Ketoacidosis (DKA)

Hyperosmolar hyperglycemic state (HHS)

Clinical manifestations:

• tetany, neuromuscular irritability with weakness, paresthesias, fatigue, cramping, AMS, seizures, laryngospasms, cardiac arrhythmias
• Trousseau sign, Chvostek sign

EKG: prolonged QT interval

caused by inadequate insulin

defined as:

• blood glucose >600mg/dL
• pH >7.30
• bicarbonate >15
• serum osmolarity >320
• small amount of ketones maybe present

Diagnostic Criteria for Diabetic Ketoacidosis and Hyperosmolar Hyperglycemic State

Diagnostic Studies:

• total and ionized Ca, PO4, ALP, Mg, total protein, BUN, Cr, 25-OH vit D, PTH
• albumin, pH

Managemement:

HyperCalcemia

Hypomagnesemia:

Acute: consider IV calcium gluconate

Chronic: consider oral supplement

• consider Vit D supplementation (400 IU/day)

HypoCa cannot be corrected if underlying hypoMg exists.

Clinical manifestations: anorexia, nausea, weakness, malaise, depression, nonspecific psychiatric symptoms, hyperreflexia, carpopedal spasms, clonus, tetany

EKG changes: atrial and ventricular ectopy; torsades de pointes

Most common cause of death in children who have type 1 diabetes.

profound insulin-deficient state

characterized by:

• blood glucose >200mg/dL
• pH <7.30
• serum bicarbonate <15
• along with evidence of an accumulation of ketoacids in the blood (measurable serum or urine ketones, increased anion gap).

Dehydration, electrolyte loss, and hyperosmolarity contribute to the presentation and potential complications.

Clinical manifestations: weakness, irritability, lethargy, seizures, coma, abdominal cramping, anorexia, nausea, vomiting, polyuria, polydipsia, renal calculi, pancreatitis

EKG: shortened QT interval

Clinical Suspicion

• polydypsia, polyuria, nocturia, enuresis
• abdominal pain, nausea, vomiting
• presence of hyperglycemia (random BG >200mg/dL)
• relative ketosis ("fruity odor")
• clinical metabolic acidosis
• tachypnea/Kussmaul breathing
• dehydrated appearance

Management:

• treat underlying disease
• hydration
• increase urine output and Ca excretion
• diuresis with furosemide
• hemodialysis in refractory cases
• steroids - in malignancy, granulomatous disease, vit D toxicity
• severe/persistent - calcitonin, bisphosphonate

Management

• Fluids
• Electrolytes
• Insulin
• Lab values

Lab values

Recognition and treatment of endocrine/metabolic emergencies in children

• check blood glucose Q1H
• check VBG and electrolytes (incl Ca and PO4) Q2H until stable, then Q4H
• monitor urine for ketones and glucose with each void

Best Treatment of DKA:

PREVENTION

Through early recognition and diagnosis of diabetes in a child who has polydipsia and polyuria and through careful attention to the treatment of children who have known diabetes, particularly during illnesses.

Fluids

Insulin

• assume 5-10% dehydration
• total fluid deficit = 10mL/kg for each 1% dehydration
• give 10-20mL/kg bolus NS or LR over 1hr
• replace remaining fluid deficit equally over 48hr plus maintenance

• begin with continuous IV drip (0.1U/kg/hr) after first fluid bolus
• goal glucose: 80-100
• when glucose reaches 250-300 or if glucose decr >100mg/dL/hr, add D5
• once pH > 7.30, HCO3 > 16, AG resolved and patient tolerating PO, start SC insulin
• discontinue IV drip 1hr after SC dose

Electrolytes

• Na - fluids should contain one half NS; Na should rise as glucose drops
• K
• K > 6 = no K initially
• K 4-6 = 40mEq/L
• K <4 = 60mEq/L
• HCO3
• rarely/never used
• consider in only severe cases of acidosis (pH <7)
• may cause paradoxical CNS acidosis

HypoNatremia

• Difficult task, especially in the child with no known underlying condition
• Signs and symptoms of these disorders are varied and nonspecific
• delayed or missed diagnosis can have serious consequences
• cerebral dysfunction leading to coma or death as seen in diabetic ketoacidosis, hypoglycemia, or adrenal insufficiency

Factitious etiologies:

• hyperlipidemia
• hyperproteinemia
• hyperglycemia

Clinical manifestations: predominantly neurological symptoms - nausea, HA, lethargy, seizures, coma

Management: replace losses/restrict fluids, treat underlying cause

Hypoglycemia

Symptoms usually occur when BG falls below 60-70g/dL

Although for children with diabetes, symptoms may occur at higher BG levels

Goal: return BG to normal

Usually not a diagnosis but a manifestation of a variety of metabolic and endocrine disorders.

HyperNatremia

Clinical Suspicion

Clinical manifestations: predominantly neurological symptoms - lethargy, weakness, AMS, irritability and seizures; also muscle cramps, depressed DTR, respiratory failure

Management: replace free water losses and treat cause

Non-specific

In neonates: lethargy, jitteriness, apnea, cyanosis, respiratory distress, poor feeding, hypothermia, myoclonic jerks or convulsions

Older children: sweating, tremors, hunger, pallor, tachycardia, shakiness

Severe: sleepiness, lethargy, visual difficulty, ataxia, behavioral abnormalities, convulsions or unconsciousness

Management

mild sx: oral glucose in the form of OJ or dextrose tablets

profound sx: IV infusion of dextrose

• IV glucose bolus of 0.25g/kg
• measure BG every 30mins
• glucagon at 0.03mg/kg (if excess insulin is suspected; contraindicated if as a result of sulfonylurea meds)
• IV glucocorticoid if AI suspected

Adrenal Insufficiency

(Acute Adrenal Crisis)

• often precipitated by acute illness, trauma, surgery or exposure to excess heat
• vague and nonspecific symptoms
• if unrecognized, life-threatening crisis with acute cardiovascular collapse
• primary AI
• in adults: autoimmune (>80%), tuberculosis
• in children: CAH, autoimmune, adrenoleukodystrophy, syndromes [Wolman, Triple A (AI, achalasia, alacrima), Zellweger)], unexplained AI
• secondary AI
• abrupt discontinuation of glucocorticoid therapy or stress while on suppressive doses

References:

Hyperthyroidism

(Thyrotoxicosis)

Management

of adrenal insufficiency

Clinical Suspicion

• Glucocorticoid maintenance
• Mineralocorticoid maintenance
• Stress-dose glucocorticoids

HypoKalemia

• N/V/D, abdominal pain, fatigue, dehydration, hypotension, metabolic acidosis, AMS, shock
• symptoms may mimic GI illness or psychiatric disorder
• hyperpigmentation of skin & salt craving - only in primary AI
• hypoglycemia - most common in children
• Labs: hypoglycemia, hyponatremia, hyperkalemia
• decreased serum cortisol and aldosterone
• elevated ACTH and renin
• in CAH, 17-hydroxyprogesterone is elevated

Management

(of Acute Adrenal Crisis)

Diagnosis:

HyperKalemia

Primary

• elevated plasma ACTH (>100pg/mL)
• low serum cortisol (<10pg/mL)
• Stim test (250ug or 15ug/kg for infants <2yo): subnormal peak cortisol level <18ug/dL 60mins after admin
• mineralocorticoid def: low aldosterone, high renin

Secondary

• low blood cortisol and ACTH
• gold standard: insulin-induced hypoglycemia - risk of seizures and severe hypoK

• rapid volume expansion to support blood pressure
• sufficient dextrose to support blood glucose
• measure cortisol, electrolyte, glucose, ACTH levels, renin, aldosterone
• stress dose of glucocorticoids:
• hydrocortisone

Clinical manifestations: skeletal muscle weakness or paralysis, ileus, cardiac arrhythmias

EKG: delayed depolarization, with flat or absent T waves; in extreme cases, U waves

Management: replace K (rate depends on severity of symptoms)

Evaluation:

Oberlin JM, Rogers WM, Fenton CL. Endocrine emergencies: recognizing signs and symptoms. Pediatr Ann. 2005 Nov;34(11):870-7; quiz 893-4.

Kappy MS1, Bajaj L. Recognition and treatment of endocrine/metabolic emergencies in children: part I. Adv Pediatr. 2002;49:245-72.

Kappy MS1, Bajaj L. Recognition and treatment of endocrine/metabolic emergencies in children: part II. Adv Pediatr. 2003;50:181-214.

The Harriet Lane Handbook: Mobile Medicine Series, 20e Paperback – June 3, 2014 by Johns Hopkins Hospital, Engorn B, Flerlage J.

http://www.luhs.org/depts/emsc/peddka_pdf.pdf

• AM cortisol levels
• ACTH stimulation test
• to measure ability of the adrenal gland to produce cortisol in response to ACTH
• Mineralocorticoid deficiency confirmed with high renin and low aldosterone

Stress Dose:

• Give 50mg/m2 of hydrocortisone by IV bolus
• rapid estimate:
• infant = 25mg
• children = 50-100mg
• Followed by 50mg/m2/24hr by continuous drip or divided by Q3-4H

Clinical manifestations: hyperactivity, irritability, altered mood, insomnia, heat intolerance, sweating, pruritis, tachycardia, palpitations, weight loss despite increased appetite, diarrhea, oligo/amenorrhea, tremor, hyperreflexia, hair loss

Thyroid storm: acute onset of hyperthermia, tachycardia, restlessness, delirium, coma, death

Tx: propranolol, potassium iodide

long term: methimazole

Clinical manifestations: skeletal muscle weakness, paresthesias

EKG: peaked T waves, loss of P waves with widening of QRS, ST-segment depression with further QRS widening, bradycardia, AV block, ventricular arrhytmias, torsades de pointes, cardiac arrest

Management: stop K-containing infusions, kayexalate, calcium gluconate, insulin, NaHCO3, albuterol

Neonatal thyrotoxicosis

Presentation: microcephaly, frontal bossing, IUGR, tachycardia, systolic hypertension with widening pulse pressure, irritability, FTT, exophthalmos, goiter, flushing, V/D, jaundice, thrombocytopenia, cardiac failure or arrhythmias

**occurs exclusively in infants born to mothers with Graves - caused by transplacental passage of maternal TSI

treatment: propranolol, methimazole, digoxin for heart failure

usually resolves by 6 months