Endocrine Disorders

Endocrine Disorders

Adrenal Insufficiency

Mary P. White


  • Adrenal glands, located on the kidneys, produce three types of hormones:

    • Glucocorticoid hormones (e.g., cortisol, corticosterone).

      • Maintain glucose control by affecting protein and carbohydrate metabolism.

      • Inhibit glucose uptake in muscle and adipose tissue.

      • Stimulate gluconeogenesis, particularly in the liver.

      • Respond to stress by upregulating the expression of anti-inflammatory mediators and downregulating the expression of proinflammatory mediators.

      • If prolonged exposure to glucocorticoids, Cushing syndrome develops.

        • Obesity, muscle wasting/weakness, decreased glucose tolerance/hyperglycemia, buffalo hump.

    • Mineralocorticoid hormones (e.g., aldosterone, dehydroepiandrosterone).

      • Maintain the balance of sodium and potassium in the body.

      • When aldosterone is increased, aldosteronism develops.

        • Hypernatremia and hyperkalemia, resulting in hypertension.

      • If androgens are increased, sex characteristics will be affected.

    • Sex hormones (e.g., androgens, progestins, and estrogens).

  • Disorders affecting the adrenal cortex lead to inadequate or absent production of hormone(s).

  • Disorders are either congenital or acquired.

  • Adrenal insufficiencies can be acute (adrenal crisis) or chronic (Addison disease).

    • The most common causes of acute adrenal insufficiency are Waterhouse-Friderichsen syndrome, sudden withdrawal of long-term corticosteroid therapy, and stress states in patients with chronic adrenal insufficiency.

Primary Adrenal Insufficiency


  • Congenital primary adrenal insufficiency.

    • Congenital adrenal hyperplasia (CAH) is the most commonly identified cause of primary adrenal insufficiency in children.

      • Autosomal recessive disorder: defect in an enzyme (largely, 21-hydroxylase deficiency) required in the synthesis of cortisol to cholesterol.

      • Results in dysfunction in the synthesis of adrenal steroids.

      • Incidence: 1 in 10,000 to 18,000 live births.

        • Females have higher incidence; most commonly diagnosed at birth.

        • Males usually present with a life-threatening salt-wasting crisis in the first month of life.

      • Newborn screen has incorporated CAH testing in most states in the United States, decreasing the time to diagnosis, particularly for males.

  • Acquired primary adrenal insufficiency.

    • Includes autoimmune etiologies (autoimmune destruction of adrenal cortex) and iatrogenic causes such as hemorrhage, trauma, drug effects, pituitary tumor, or infection.

Clinical Presentation

  • CAH.

    • Ambiguous genitalia at birth.

    • If not diagnosed at birth, symptoms will present within 1 to 4 weeks of life.

      • Vomiting, dehydration, cardiac arrhythmias, hyponatremia, hyperkalemia, or salt-losing crisis, resulting in circulatory collapse.

  • Adrenal insufficiency.

    • Symptoms can be slower to progress.

      • Fatigue, loss of weight, hyperpigmentation of the creases of the skin, nausea, vomiting.

      • Prolonged recovery from an illness may prompt further investigation of multiple vague symptoms, leading to the diagnosis.

  • Adrenal crisis.

    • Acute symptoms: life-threatening disorder.

      • Vomiting, abdominal pain, hypovolemic shock.

      • May occur in individuals with chronic adrenal insufficiency experiencing a stressor such as an intercurrent illness, surgical procedure, or in cases of abrupt cessation of glucocorticoid administration.

Diagnostic Evaluation

  • 17-OHP levels; often completed on newborn screening.

  • Morning 17-OHP levels may be elevated in a partial enzyme deficiency.

  • Testosterone level; females (elevated).

  • Androstenedione; males and females.

  • Karyotyping important for ambiguous genitalia.

  • Adrenocorticotropic hormone (ACTH) stimulation test may be necessary to confirm diagnosis.

    • Significant rise in cortisol level 30 to 60 minutes following ACTH injection.

    • Decreased cortisol response also seen in some cases.

Secondary Adrenal Insufficiency

Management: Primary and Secondary Adrenal Insufficiency

  • Glucocorticoid administration.

    • Goal is to replace physiologic glucocorticoid production.

      • CAH: 10 to 20 mg/m2/day of oral hydrocortisone daily.

      • Adrenal insufficiency: 6 to 9 mg/m2/day oral hydrocortisone daily.

    • The dose of glucocorticoid should be adjusted in patients with fever or illness to reflect the normal physiologic response to stress (stressed states result in elevated cortisol levels in a normal host).

    • Stress dosing: hydrocortisone 25 to 50 mg/m2/day IV/IM.

    • For severe illness or surgical procedures, higher doses may be indicated: hydrocortisone 50 to 123 mg/m2/day IV.

  • Mineral corticoid maintenance.

    • CAH and salt-losing forms of adrenal insufficiency: 0.1 to 0.2 mg oral fludrocortisone acetate daily.

    • Infants require 17 to 34 mEq of sodium supplementation daily.

    • Monitor blood pressure and electrolytes.

Cerebral Salt Wasting

Michele Goodwin

Sharon Y. Irving


  • Occurs following an acute central nervous system (CNS) injury.

  • Occurs in the setting of both hypovolemia and hyponatremia.

  • Reports of cerebral salt wasting (CSW) in the pediatric population first appeared in the 1980s.

  • Syndrome of inappropriate antidiuretic hormone (SIADH) is more common than CSW in patients with hyponatremia and CNS disease.

  • It is important to distinguish between SIADH and CSW as treatments for the disorders are different.


  • Unclear etiology.

  • Strong association with elevation in the circulating brain and atrial natriuretic peptides, along with an alteration in the neuronal control of the kidneys.

  • May lead to the inhibition of the renin-angiotensin-aldosterone system, causing abnormal renal reabsorption of sodium and triggering the release of antidiuretic hormone (ADH) necessary to maintain intravascular volume.

  • May be associated with the following clinical conditions:

    • Traumatic brain injury.

    • Intracranial surgery.

    • Meningitis.

    • Encephalitis.

    • Subarachnoid hemorrhage.

Clinical Presentation

  • Headache.

  • Nausea/vomiting.

  • Depressed/altered mental status.

  • Lethargy.

  • Dehydration.

  • Agitation.

  • Seizures.

  • Hypotension.

  • Coma.

  • The rate of renal sodium loss, the degree of hyponatremia, and the overall fluid status impact the severity of the presenting symptoms.

Diagnostic Evaluation

  • Laboratory studies:

    • Serum sodium <135 mEq/L.

    • Serum osmolarity <280 mOsm/kg.

    • Urine sodium >80 mEq/L.

    • Urine osmolarity >200 mOsm/kg.

    • Urine specific gravity >1.010.

    • Urine output 2 to 3 mL/kg/hour.

    • Head CT/MRI.

      • Can identify structural abnormalities/pathophysiologic changes (e.g., arteriovenous malformation, tumor or other space-occupying lesion, hemorrhage).

    • Lumbar puncture.

      • CNS infection (in cases of CNS-infection-triggered CSW).


  • Distinguish between CSW and SIADH.

    • Treatment is different for these conditions.

    • Identify and treat the underlying cause.

    • Frequent monitoring of serum sodium levels and fluid balance.

    • Sodium replacement using a non-dextrose-containing isotonic or hypertonic fluid at an approximate rate of 0.5 to 1 mEq/hour.

    • Limit serum sodium level rise to no more than 10 to 12 mEq/day.

      • The demyelination that occurs with rapid osmotic fluid shifts can result in irreversible neurologic damage.

      • If the patient presented with acute neurologic changes, this may be related to the rate of serum sodium loss.

        • In this instance, it may be more appropriate to provide non-glucose-containing hypertonic fluid until symptoms abate.

    • Consultation with appropriate subspecialty services, including neurosurgery and neurology.

    • Patient/family education regarding CSW etiology, diagnostic testing, and treatment.

Diabetes Insipidus

Allison Thompson


  • A disorder caused by insufficient secretion of ADH by the pituitary gland (neurogenic), or failure of the kidneys to respond to circulating ADH (nephrogenic).

  • Characterized by increased thirst and the excretion of large amounts of dilute urine.


  • Neurogenic (central diabetes insipidus [DI]).

    • Genetic: typically X-linked recessive.

      • Examples: Wolfram syndrome.

        • A rare inherited autosomal recessive condition.

        • Affects 1 in 770,000 children.

        • Characterized by central DI, diabetes mellitus, optic atrophy, and deafness.

        • In this disorder, central DI is caused by the loss of ADH-secreting neurons in the supraoptic nucleus and impaired processing within the hypothalamus.

    • Congenital.

      • Often associated with midline craniofacial defects such as holoprosencephaly and septo-optic dysplasia.

    • Acquired.

      • Can result from damage to the pituitary gland or posterior hypothalamus from neurosurgery, trauma, tumors or other brain lesions, meningitis, or encephalitis.

      • May be either a temporary or a permanent disorder depending on the injury.

  • Nephrogenic DI.

    • Congenital.

      • Typically X-linked recessive involving mutations of VR2 or AQP2.

    • Acquired.

      • Variety of conditions that lead to the inability of the kidneys to respond to ADH.

        • Chronic renal failure.

        • Renal tubulointerstitial diseases.

        • Hypercalcemia.

        • Potassium depletion.

        • Sickle cell disease.

      • Medication-induced from drugs, including alcohol, lithium, diuretics, amphotericin B, demeclocycline.

      • Dietary abnormalities.

        • Primary polydipsia.

        • Decreased sodium chloride intake.

        • Severe protein restriction or depletion.

        • Nephrogenic DI that results from a metabolic condition may be reversed if the medication is stopped or the metabolic condition is corrected.

Clinical Presentation

  • Polyuria.

  • Dilute urine.

  • Polydipsia.

  • Inappropriately low urine sodium and osmolality.

  • Urine specific gravity <1.005.

  • Hypernatremia.

  • Serum hypo-osmolality.

  • Dehydration.

Diagnostic Evaluation

  • History and differential diagnoses.

    • The primary causes of polyuria and polydipsia are diabetes mellitus and central DI.

    • Other causes include urinary tract infection, relief of renal obstruction, and psychogenic polydipsia (characterized by excessive water intake).

    • Once hyperglycemia has been excluded, history should include age of initiation and rate of onset of polyuria (will reflect primary vs. secondary cause).

  • Serum laboratory studies.

    • Sodium >150 mEq/L.

    • Osmolality ≥295 mOsm/kg.

  • Urinary laboratory studies.

    • Sodium <30 mEq/L.

    • Osmolality <200 mOsm/L.

    • Specific gravity <1.005.

  • Brain imaging studies.

    • Head CT/MRI.

      • Presence of intracranial mass, abnormal findings of hypothalamic/pituitary stalk.

  • Water deprivation testing.

    • Only performed in acute care setting under close medical monitoring and supervision.

    • Fluids are restricted until as much as 5% of body weight has been lost to evaluate urinary response when the serum osmolality exceeds 295 mOsm/kg.

      • Central DI.

        • Concentrated urine and decreased urine output following ADH administration.

      • Nephrogenic DI.

        • Excessive, dilute urine despite hypernatremia and hyperosmolality.


  • Restore hemodynamics.

  • Replace water deficits and correct electrolyte disturbances.

  • Decrease urine output to within normal range (e.g., vasopressin, desmopressin).

  • Treat underlying condition, when possible.

  • Volume replacement.

    • Maintenance IV fluids, plus mL per mL urine output replacement (usually allow 1-2-mL/kg/hour urine output and replace the remainder).

  • Monitor serum sodium closely.

  • Primary plan for central DI is ADH replacement to control polyuria.

    • ADH preparation depends on acuity of illness and the ability of the patient to tolerate oral intake.

    • Dose varies based on the preparation/formulation and include:

      • Vasopressin.

        • Continuous IV infusion.

        • Used in the critical care or perioperative setting due to its short half-life (10-20 minutes) and easy titration.

        • Initiated at a dose of 0.5 milliunits/kg/hour and titrated until urine output is decreased.

        • Titrated to obtain urine output less than 4 mL/kg/hour.

      • Desmopressin.

        • Used in all other settings.

        • Available in oral and intranasal formulations.

        • Chronic therapy: Dose range is 5 to 30 µg/day, with a peak effect within 1 to 5 hours.

        • Nephrogenic DI is resistant to vasopressin administration (Figure 7.2).

FIGURE 7.2 • Diabetes Insipidus Flowchart.

Diabetic Ketoacidosis

Keshava Gowda

Tageldin M. Ahmed


  • Previous diagnosis of type I diabetes:

    • Insulin dose omission.

    • Intercurrent illness.

      • Stress increases counterregulatory hormone levels, promoting gluconeogenesis and insulin resistance.

    • Unrecognized disruption in insulin pump therapy (if applicable).

  • New onset of type diabetes

Clinical Presentation

  • Polyuria, polydipsia, polyphagia, abdominal discomfort/pain, nausea and vomiting, nonspecific weakness, fruity breath odor, Kussmaul respirations.

    • Severe presentation can include altered mental status, seizures, and coma.

Physical Examination Findings

  • Tachycardia.

  • Decreased pulses.

  • Poor perfusion.

  • Dry mucus membranes.

  • Enophthalmos.

  • Poor skin turgor.

  • Hypotension.

  • Deep or labored breathing.

Diagnostic Evaluation

  • Blood glucose >200 mg/dL, serum pH <7.3, bicarbonate <15 mmol/L.

  • Serum electrolytes, blood urea nitrogen (BUN), creatinine, calcium, magnesium, phosphorus.

  • High serum osmolality.

  • Positive serum/urine ketones.

  • Hemoglobin A1C.

  • Complete blood count with differential.

    • Leukocytosis is not a reliable marker of infection.

    • Elevation in stress hormones may mimic infection.

  • Autoimmune markers.

    • GAD-65 (glutamic acid decarboxylase), IA-2, IA-2β.

    • Insulin auto-antibodies.

    • May be evaluated for evidence of associated autoimmune conditions.


  • Monitoring.

    • Cardiac monitoring.

      • Assess T wave alterations with hyper- or hypokalemia.

    • Cerebral edema.

      • Most serious complication and frequent cause of death.

      • Survivors often experience neurologic sequelae.

      • Occurs in approximately 1% of cases.

      • Has been reported prior to the initiation of therapy, but typically occurs after the start of treatment.

      • Risk factors.

        • Young age.

        • New-onset diabetes.

        • Longer duration of symptoms.

      • Signs and symptoms of cerebral edema (see Section 6: Neurologic Disorders).

      • Requires rapid recognition and treatment.

        • Elevate head of bed.

        • Administer hyperosmolar therapy.

          • Preferred treatment is hypertonic saline (e.g., 3% saline).

            • 5 to 10 mL/kg.

          • Mannitol.

            • 0.5 to 1 g/kg IV.

            • May be repeated if no response.

        • Intubation and mechanical ventilation if progression of symptoms.

        • Head CT is not routinely completed prior to start of therapy, but can be obtained to evaluate and document presence of cerebral edema.

  • Respiratory support.

    • Supplemental oxygen.

      • If respiratory distress, circulatory impairment, or shock.

    • If altered mental status, consider mechanical ventilator support.

  • Access.

    • Establish multiple peripheral IV catheters.

    • May require arterial line for frequent laboratory sampling.

  • Judicious fluid replacement.

    • Avoid overaggressive fluid replacement with frequent evaluation and repeated boluses, as needed.

    • Moderate to severe DKA with poor perfusion.

      • Start with 0.9 normal saline (NS) 10 to 20 mL/kg bolus.

      • Repeat bolus if poor perfusion/hypotension persists.

      • Obtain repeat blood gas, finger-stick glucose, and basic metabolic panel after initial fluid resuscitation.

      • Remaining fluids are calculated deficits and replaced over 36 to 48 hours using isotonic fluid (e.g., 0.9 NS).

        • Subtract the volume administered in boluses from 24 hours fluid calculation.

      • Withhold potassium from fluids until evidence of adequate kidney function and serum potassium level decreasing.

      • Consider use of two-bag method when ready for introduction of glucose.

        • Allows regulation of glucose from D5W to D10W.

        • Equal electrolyte supplements are added to each bag when using two-bag method.

      • Add dextrose to IV fluids when blood glucose level is 200 to 250 mg/dL.

      • Adjust glucose to prevent rapid drop in blood glucose (e.g., >100 mg/dL/hour).

  • Insulin therapy.

    • Insulin infusion is typically started at 0.1 unit/kg/hour.

    • Follow hourly blood glucose levels.

    • Adjust the amount of dextrose in the IV fluids rather than decreasing the insulin.

    • Decrease the insulin infusion only in cases when patient demonstrates extreme sensitivity to insulin (e.g., usually young children).

    • Continue the infusion until the pH is >7.3 and bicarbonate level >18 mmol/L, or ketonemia has resolved.

  • Sodium.

    • Replace using 0.9 NS or Ringer lactate for first several hours of DKA therapy.

    • Follow this initial therapy with 0.45 NS.

    • Hyperglycemia results in a lower serum sodium concentration.

      • Results in dilutional hyponatremia, due to the movement of water into the extracellular fluid.

      • Calculation:

        Corrected Sodium = [Na+] + [1.6 × (plasma concentration mg/dL – 100)]/100

      • As hyperglycemia improves, serum sodium should improve.

      • If sodium level increases or does not begin to fall, there is concern for the development of cerebral edema.

  • Potassium.

Jan 30, 2021 | Posted by in NURSING | Comments Off on Endocrine Disorders

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