Approach to Diagnosing and Managing Rare Endocrine Emergencies

Introduction:

Endocrine disorders are clinical conditions that basically present with either excess or depleted hormone levels in the body. This leads to various non-specific clinical manifestations, which sometimes misleads the diagnosis. Hence, a clinician should profess adequate knowledge about the clinical presentation, diagnosis, and management of such emergencies to deliver optimum care.

What Are Some Rare Endocrine Emergencies Which Need to Be Diagnosed and Managed Effectively?

Pituitary Apoplexy:

1. Introduction:

• Pituitary apoplexy is a varied clinical condition associated with sudden hemorrhage or infarction of the pituitary gland and is most commonly associated with a pituitary adenoma.

• The occurrence of pituitary apoplexy is rare and can occur in cases of postpartum uterine hemorrhage, leading to significant hypotension and infarction of the pituitary gland, leading to Sheehan syndrome (damaged pituitary gland due to loss of blood supply).

2. Pathophysiology:

• Precipitating factors include angiographic procedures, surgical procedures, head trauma, pregnancy, and anticoagulant therapy.

• Various hormone therapies and stimulating the pituitary gland using insulin, gonadotropin-releasing hormone, growth hormone-releasing hormone, thyrotropin-releasing hormone, and corticotropin-releasing hormone) precipitate pituitary apoplexy (rupture of pituitary gland tissue).

3. Clinical Features:

• Depending on the underlying causes, like the extent of hemorrhage, necrosis, and edema, pituitary apoplexy can vary from mild to medical emergencies involving coma and hemodynamic instability, and sometimes even leading to death.

• Symptoms include,

  • Headache, also called thunderclap headache.
  • Visual disturbances.
  • Meningeal irritation from the extravasation of blood or necrotic tissue into the subarachnoid space leads to photophobia (fear of light), nausea, vomiting, and meningismus (inflammation of a layer that covers the brain causing headache, neck stiffness, and photophobia).
  • Fever, altered consciousness.
  • Cerebral ischemia (loss of blood supply to the brain tissue).
  • Endocrine dysfunctions like corticotropic deficiency (deficiency of corticotropic hormone which manages dress response of the body).
  • Thyrotropic deficiency (deficiency of thyroid stimulating hormone).
  • Diabetes insipidus (a metabolic disorder characterized by increased thirst and urination).

4. Diagnosis:

• The diagnosis is best made by combining clinical features, laboratory tests indicating endocrine abnormalities, and radiological interventions.

• A CT (computed tomography) scan can be investigated when a patient presents with an acute onset of headache and can detect simple, hemorrhagic, and mixed infarction or a pure clot.

• MRI is the superior imaging choice in the case of pituitary apoplexy.

5. Management:

• Acute pituitary apoplexy (rupture of pituitary gland tissue) is treated either by surgery or by a conservative medical approach.

• Surgical management aims at resecting the apoplectic pituitary mass in order to eliminate neurologic and visual manifestations.

• Conservative treatment options can be instilled in absent, mild, or stable cases of neuro-ophthalmic conditions.

• Corticosteroid replacement therapy should be initiated and tapered to maintenance dose in patients suffering from hemodynamic instability, altered consciousness, reduced visual sensitivity, severe visual field deficits, or signs of hypoadrenalism.

• Patients may require additional long-term hormonal replacement therapy to treat hypopituitarism caused by apoplexy.

Adrenal Crisis (Addisonian Crisis):

1. Introduction:

• • An adrenal crisis is a condition in which the adrenal glands do not produce adequate cortisol hormone.
  • It is considered a life-threatening complication of adrenal insufficiency, also called Addison’s disease, as the hormone cortisol plays an important role in
    • Regulating the stress response mechanism of the body.
    • Controlling the metabolic reactions of the body.
    • Suppressing inflammation.
    • Regulating blood pressure.
    • Regulating blood sugar.
    • Controlling the sleep-wake cycle.

2. Pathophysiology:

• Cortisol produced from the adrenal gland is a glucocorticoid, a corticosteroid that maintains normal homeostasis of the body.

• Cortisol production is regulated by a small gland just below the brain called the pituitary gland.

• An adrenal crisis occurs during a stressful event when a patient with adrenal insufficiency fails to have adequate endogenous cortisol production to manage the stressful situation and is also not adequately replaced with exogenous glucocorticoids.

• Gastrointestinal illness, fever, and acute infections are the precipitating factors.

• Surgical stress, emotional stress, and inadequate exogenous corticosteroid treatment cause adrenal insufficiency, leading to a crisis.

3. Clinical Features:

• Gastrointestinal symptoms include nausea and vomiting.

• Muscle cramps.

• Hypotension.

• Neurological symptoms like delirium (altered mental status), convulsions (seizures), and myopathies (muscle weakness)

4. Diagnosis:

• Primary adrenal insufficiency can be detected by hyponatremia (decreased sodium levels in the blood). and hyperkalemia ( decreased potassium levels in the blood).

• Elevated BUN (blood urea nitrogen indicates prerenal failure).

• Hypoglycemia can be seen in children with primary adrenal insufficiency.

• Hypercalcemia also gives a clue.

5. Management:

• Fluid resuscitation and steroid replacement are considered the first line of treatment options in managing the adrenal crisis.

• Hypovolemia and hyponatremia should be corrected by adequate fluid resuscitation.

• Replacement of steroids should be carried out once a patient becomes clinically stable.

• Glucocorticoid doses are to be tapered as soon as possible in one or three days to oral maintenance doses. Mineralocorticoid replacement can be restarted once the Hydrocortisone dose falls below 50 mg/day.

Myxedema Coma:

1. Introduction:

• Myxedema coma is a rare medical emergency encountered in clinical practice.

• Myxedema coma is a progressive complication that happens following a pulmonary or urinary infection, congestive heart failure, stroke, trauma, or in undiagnosed or untreated cases of hypothyroidism.

• Delayed diagnosis can be attributed to higher mortality rates.

• Myxedema coma is generally seen in older women above 60 years.

2. Clinical Features:

• The pathognomic features include hypothermia, depressed mental status, or coma.

• Dry, brittle skin and hair, non-pitting edema, hoarse voice, macroglossia (large tongue), and delayed reflexes.

• Hypotension.

• Shock.

• Gastrointestinal bleeding.

• Neurologic signs include altered mental status, cerebellar signs, and seizures.

• Status epilepticus (seizure attack).

3. Diagnosis:

• Hyponatremia, anemia, and acidosis directly point out the diagnosis.

• Free thyroid hormones thyroxine (T4) and triiodothyronine (T3) should be investigated to confirm hypothyroidism-associated myxedema coma (a severe complication of hypothyroidism associated with altered mental status).

4. Management: Treatment options include,

• Airway protection.

• Thyroid hormone therapy.

• Fluid resuscitation.

• Hydrocortisone therapy to correct adrenal insufficiency and hyponatremia (decreased sodium levels in the blood).

• Antibiotic therapy for eliminating infections that aggravate the condition.

Diabetic Ketoacidosis (DKA): The increasing prevalence rates of diabetes mellitus co-relate with its associated complications like hyperglycemic crisis and acute hypoglycemic episodes. The brain relies only on glucose as its energy source. Sudden variations in brain glucose levels can have large neurological implications, which may or may not be reversible.

1. Pathophysiology:

• Diabetic ketoacidosis is characterized by metabolic acidosis and hyperglycemia, which is caused by the combined effects of increased counter-regulatory hormones and relative or absolute insulin deficiency.

• Aggravating factors include infection, improper insulin dosing, pancreatitis, myocardial infarction, stroke, and certain drugs.

2. Clinical Features:

• Uncontrolled hyperglycemia (uncontrolled rise in glucose level in the blood).

• Metabolic acidosis (an imbalance in the blood pH characterized by increased acidic levels of blood).

• Elevated ketone bodies (increased level of ketone bodies in the blood. Ketone bodies are the breakdown products of the fat).

• Polyuria (excess urination).

• Polydipsia (excess thirst).

• Weight loss.

• Dehydration.

• Weakness.

• Altered mental status.

3. Diagnosis:

• Biochemical tests, including plasma glucose, serum bicarbonate, serum, and urine ketone tests, are of great help in the diagnosis.

4. Management:

• The mainstay of the treatment is to rectify dehydration, control blood sugar levels, correct electrolyte abnormalities, and eliminate the risk factors.

• Fluid resuscitation should be carried out meticulously, and the water deficit should be corrected within 12 hours.

• IV (intravenous) insulin therapy should be started as soon as possible after fluid resuscitation therapy.

Conclusion:

Endocrine emergencies commonly manifest as life-threatening conditions like seizures, hemodynamic instability, and morbidity, which, if left untreated, prove to be fatal. Unfortunately, many patients with endocrine emergencies seldom are diagnosed as having an endocrine disorder, which is even more worsened by certain underlying risk factors. Hence, caregivers in the Department of Emergency Medicine play an important role in managing such patients. A better understanding of clinical manifestations, diagnosis, and apt treatment planning can ensure positive treatment outcomes.