Pheochromocytoma, catecholamine crisis: causes, symptoms, diagnosis, treatment

The catecholamine crisis is a life-threatening emergency. It develops mainly with pheochromocytoma (chromaffinoma), a hormone-producing tumor of chromaffin tissue.

[1], [2], [3], [4]

Causes of pheochromocytoma

The frequency of pheochromocytoma in the population is 1-3 cases per 100 000 people; and among patients with arterial hypertension - 0.05-0.2%. In about 10% of cases, pheochromocytoma is a family disease and inherited autosomal dominantly. Malignant are less than 10% pheochromocytomas. They, as a rule, are localized outside the adrenal glands and secrete dopamine. Usually pheochromocytoma secretes both adrenaline and norepinephrine, but mostly norepinephrine. Very rarely the predominant catecholamine is dopamine. In addition to catecholamines, pheochromocytoma can produce: serotonin ACTH, VIP, somatostatin, opioid peptides, a-MSH, calcitonin, parathyroid-like peptides and neuropeptide Y (strong vasoconstrictor).

The catecholamine crisis can be caused by an overdose of street drugs, such as cocaine and amphetamines.

[5], [6], [7], [8]

Symptoms of pheochromocytoma

Patients most often complain of headache, sweating, palpitation, irritability, weight loss, chest pain, nausea, vomiting, weakness or fatigue. Less common are visual disturbances, fever, dyspnea, paresthesia, hot flashes, polyuria, polydipsia, dizziness, lightheadedness, convulsions, bradycardia (noted by the patient himself), lumpy sensation in the throat, tinnitus, dysarthria, vomiting, painless hematuria.

Common complaints are pain in the epigastric region, a violation of intestinal motility and constipation. Occasionally, ischemic colitis, intestinal obstruction, megacolon are observed. With pheochromocytoma, the risk of cholelithiasis is increased. Narrowing of the vessels of the extremities under the influence of catecholamines can cause pain and paresthesia, intermittent claudication, Raynaud's syndrome, ischemia, trophic ulcers.

Leading symptoms of pheochromocytoma - a change in the level of blood pressure (in 98% of patients). And hypertensive crises can be replaced by arterial hypotension.

Other signs of excess catecholamines: sweating, tachycardia, arrhythmia, reflex bradycardia, strengthened apical push, pale skin of the face and trunk, arousal, anxiety, fear, hypertonic retinopathy, dilated pupils: very rarely - exophthalmos, lacrimation, pallor or hyperemia sclera, lack of reaction pupil to light. Patients, as a rule, thin; body weight does not match growth; characteristic tremor, Raynaud's syndrome, or marbling of the skin. Children sometimes have swelling and cyanosis of the hands; wet, cold, sticky and pale skin of hands and feet; "Goosebumps", cyanosis of the nail lodges. Palpation of volumetric education, found in the neck or in the abdominal cavity, can cause an attack.

Catecholamine crises under pheochromocytoma in about 75% of patients develop at least once a week. Over time, attacks occur more often, but their severity does not increase. The duration of the attack is usually less than one hour, but some seizures can last a whole week. For seizures are characterized by the same symptoms: palpitations and lack of air, cold and moist hands and feet, pale face, blood pressure rises sharply. Severe or prolonged seizures are accompanied by nausea, vomiting, visual impairment, pain in the chest or abdomen, paresthesia, seizures, fever.

Attacks are caused by mental agitation, physical activity, posture changes, intercourse, sneezing, hyperventilation, urination, etc. Provoke seizures are capable of some smells, as well as the consumption of cheese, beer, wine and strong alcoholic beverages. Attacks can also be triggered by medical research (palpation, angiography, intubation of the trachea, general anesthesia, labor and surgical interventions). They arise and against the background of taking beta-adrenoblockers, hydralazine, tricyclic antidepressants, morphine, naloxone, metoclopramide, droperidol, and others.

Massive release of norepinephrine, dopamine and serotonin in case of cocaine or amphetamine overdose may cause adolescents to develop not only severe arterial hypertension, but also acute coronary syndrome, myocardial infarction, cardiac arrhythmias, intracerebral hemorrhages, pneumothorax, noncardiogenic pulmonary edema, intestinal necrosis , malignant hyperthermia.

Diagnosis of pheochromocytoma

The main criterion for the diagnosis of pheochromocytoma is an elevated level of catecholamines in urine or in plasma. It is necessary to visualize the tumor (CT and MRI).

[9], [10], [11]

Emergency medical care for catecholamine crisise

The most reliable way to treat pheochromocytoma is to remove it, especially in the case of ineffectiveness of antihypertensive therapy for 3 hours after repeated injection of a non-selective blocker of a1,2-adrenoreceptors-tropodifen-in a dose of 2-4 mg (or phentolamine 2-5 mg) min.

To lower blood pressure, sodium nitroprusside [0.5-8 μg / (kg-min)) is also used until the effect is achieved]. To prevent or eliminate arrhythmias against a blockade of a-adrenergic receptors, propranolol 1-2 mg intravenously every 5-10 min or esmolol [0.5 mg / kg intravenously, then as an infusion at a rate of 0.1-0.3 mg / ( kgxmin)]. To stabilize hemodynamics, you can appoint labetalol, captopril, verapamil or nifedipine. In the case of effective blockade of a-adrenergic receptors, it may be necessary to complete the BCC. To determine the correct amount of fluid, the pulmonary artery wedge pressure is measured.

Long-acting a-adrenoblocker fenoksibenzamin prescribe first 10 mg twice a day, then the dose is gradually increased every day by 10-20 mg and adjusted to 40-200 mg / day. No less effective prazozin (selective a1-adrenoblocker), usually prescribed in a dose of 1-2 mg 2-3 times a day.

If surgery is not possible due to a severe condition of the patient or in the presence of metastases of a malignant pheochromocytoma, methyrosine, a tyrosine hydroxylase inhibitor, is used.

With metastasizing pheochromocytomas secreting VIP and calcitonin, somatostatin is effective. With malignant pheochromocytoma, cyclophosphamide, vincristine and dacarbazine are used. "

In case of an overdose of cocaine or amphetamines, nitroglycerin is used either intravenously or intravenously, sodium nitroprusside [0.1-3 μg / (kghmins) [, phentolamine, calcium channel blockers (amlodipine 0.06 mg / kg, nifedepine 10 mg 3-4 times a day) , benzodiazepines (diazepam 0.1 mg / kg intravenously and repeatedly in the range 0.3-0.5 mg / kg, midazolam 0.1-0.2 mg / kg). It should be borne in mind that the introduction of beta-adrenoblockers can lead to deterioration and death in connection with the aggravation of coronary spasm, myocardial ischemia and paradoxical arterial hypertension (prevalence of alpha-adrenergic activation).

[12], [13], [14],