Pheochromocytoma Crisis in the Elderly
Introduction
Introduction to pheochromocytoma crisis in the elderly Chromaffin cells, mainly distributed in chromosomal tissues such as adrenal medulla and sympathetic ganglia, are responsible for synthesis, storage and release of norepinephrine and epinephrine. The tumor originating from mature chromaffin cells is called pheochromocytoma, and the clinical manifestations are characterized by a series of syndromes caused by excessive catecholamines (norepinephrine and adrenaline), of which hypertension is the most prominent symptom due to tumor release. A large amount of catecholamines enter the blood, leading to severe clinical syndromes, such as hypertensive crisis, hypotension shock and severe arrhythmia, etc., called pheochromocytoma crisis. basic knowledge The proportion of illness: 0.005% Susceptible people: the elderly Mode of infection: non-infectious Complications: cerebral hemorrhage, pulmonary edema, arrhythmia, acute myocardial infarction, gastrointestinal bleeding
Cause
Elderly pheochromocytoma crisis etiology
(1) Causes of the disease
Pheochromocytoma is part of the familial, multiple endocrine neoplasia syndrome (MEA-II, Sipple syndrome). 50% of cases of familial pheochromocytoma are bilateral tumors, and in the sporadic cases, only bilateral 10%, about 5% of patients with pheochromocytoma have neurofibroma; and only 1% of patients with neurofibromatosis have pheochromocytoma, 50% of children with pheochromocytoma are single tumors in the adrenal gland, and 25% involve bilateral Adrenal gland, 25% outside the adrenal gland.
(two) pathogenesis
Norepinephrine and adrenaline are important hormones that regulate the body's physiological functions and metabolism. Almost all tissues in the body have receptors for these two hormones, namely and adrenergic receptors, which are each divided into 1. , 2 and 1, 2 subtype (there is still a 3 subtype, which has not been fully elucidated), the effects of different types of receptors activated and their differences in tissue determine the characteristics of each tissue response to catecholamines. Stimulation of alpha receptors causes excitatory reactions, such as elevated blood pressure in vasoconstriction, dilated pupils of the dilated muscles, contraction of the gastrointestinal and bladder sphincters, contraction of the pilose muscles, sweating, etc.; Role: increased myocardial contractility, increased heart rate, conduction acceleration, etc.; 2 receptors after excitation, the role of intermediate metabolism, such as glycogen decomposition, gluconeogenesis, leading to elevated blood sugar and lipolysis, 2 receptor activation also caused Arteriolar dilatation, as well as muscle relaxation in the bronchi, gastrointestinal tract and bladder.
Adrenaline and norepinephrine have affinity for , receptors, adrenaline has a slightly stronger affinity for 1 receptor than norepinephrine, whereas for 2 receptor, vice versa, affinity for 1 receptor is similar. The 2 receptor adrenaline is at least 10 times larger than norepinephrine, so the effect of adrenaline on the middle metabolism, small artery dilation, bronchial and gastrointestinal tract smooth muscle relaxation.
Pheochromocytoma is characterized by uncontrolled synthesis, storage, and irregular release of catecholamines. The hormones that are released by the hormones activate different receptors, producing different pathophysiological states, leading to the diversity of crisis manifestations.
Adrenal medullary pheochromocytoma can secrete adrenaline mainly, and can also secrete norepinephrine, and extra-adrenal pheochromocytoma only secretes norepinephrine.
Prevention
Elderly pheochromocytoma crisis prevention
Actively control blood pressure, prevent complications, etc. A reasonable diet, eat light, nutritious food. Exercise moderately and maintain adequate sleep.
Complication
Elderly pheochromocytoma crisis complications Complications cerebral hemorrhage pulmonary edema arrhythmia acute myocardial infarction gastrointestinal bleeding
The main complications are cerebral hemorrhage, pulmonary edema, severe arrhythmia, leading to acute myocardial infarction, gastrointestinal bleeding.
Symptom
Elderly pheochromocytoma crisis symptoms Common symptoms Trauma blood pressure drop sudden death anxiety hypertension ketoacidosis hypotension high fever acute abdomen nausea
1. Typical expression of pheochromocytoma
Hypertension is the most important symptom of most patients with pheochromocytoma. The disease is usually characterized by paroxysmal hypertension. The blood pressure suddenly rises when the attack occurs. The systolic blood pressure can be as high as 26.7kPa (200mmHg), accompanied by headache, sweating, limbs. Tremor, tachycardia, urgency in the anterior region, angina, anxiety, fear, blurred vision, dilated pupils, facial flushing or paleness, blood pressure returned to normal or original level after the termination of the attack, more than half of the patients' blood pressure continued to rise High or on the basis of increased aggravation, emotional, smoking, pressing the abdomen, trauma, induction of anesthesia, intraoperative compression of the tumor is a common predisposing factor, tumors in the bladder often have hematuria and urination can cause seizures.
2. Crisis performance
According to the characteristics of the episode group, the following types can be divided.
(1) Hypertensive crisis type: It is a group with high incidence of various crisis. Due to the continuous or paroxysmal release of catecholamine into the blood, the blood pressure is sharp or paroxysmal, and the systolic blood pressure can be increased. Up to 40kPa (300mmHg) or more, diastolic blood pressure can reach 17.3kPa (130mmHg) or more, accompanied by severe headache, nausea, vomiting, blurred vision, optic disc edema, fundus hemorrhage, etc., can quickly appear heart and kidney dysfunction, easily complicated by cerebral hemorrhage; Or acute left heart failure, pulmonary edema; or due to strong contraction of the coronary arteries, occlusion, leading to acute myocardial infarction.
(2) hypotensive shock type: patients with pheochromocytoma have hypotension shock in the following situations:
1 Hypertensive episodes were injected with reserpine (depleted catecholamines) antihypertensive drugs, or used, a large number of alpha blockers did not fully supplement the blood volume, sudden release of catecholamines, sudden blood pressure decreased shock.
2 Some patients have alternating hypertension and hypotension shock, repeated attacks, due to sudden release of a large number of catecholamines, resulting in high blood pressure, often accompanied by acute left heart failure pulmonary edema, catecholamine release stopped, vasodilation, blood volume is seriously insufficient, In addition to myocardial damage, resulting in shock, blood pressure decreased and stimulated tumor release of catecholamines, blood pressure rose again, such blood pressure is extremely fluctuating, easily complicated by cerebrovascular accidents and acute myocardial infarction, and treatment is very difficult.
3 lack of adequate medical treatment preparation before surgery, intraoperative blood loss and fluid loss are not fully compensated, ligation of tumor blood vessels or tumor resection, blood pressure suddenly decreased shock, if excessive use of long-acting alpha blockers, alpha receptors before surgery It is completely blocked, making it difficult for the booster to work, causing refractory shock.
4 very few patients due to acute hemorrhagic necrosis in the tumor, resulting in catecholamine failure (adrenal medullary failure), with sudden blood pressure drop, severe shock as a prominent performance.
(3) severe arrhythmia type: premature contraction, rapid supraventricular arrhythmia is more common in patients with pheochromocytoma, if there is frequent, multi-source ventricular premature contraction, is a precursor to severe arrhythmia , paroxysmal ventricular tachycardia, ventricular flutter, ventricular fibrillation, A-S syndrome, is a serious arrhythmia, can not be rescued in time, can also cause sudden death, various conduction block, even atrioventricular separation .
(4) Other types: Some patients may cause high fever due to a large amount of catecholamines, body temperature can reach above 40 °C, accompanied by sputum, cold limbs, sweating, tachycardia and arrhythmia, very few patients due to a large number of norepinephrine, Can cause gastrointestinal vascular damage or even occlusion, causing intestinal infarction, ulcers, bleeding or perforation and other acute abdomen, patients with adrenaline-producing patients can be complicated by diabetic ketoacidosis, malignant pheochromocytoma can occasionally have hypoglycemia Even coma.
According to the clinical manifestations and urine or blood catecholamine and its metabolite determination and localization examination, the diagnosis of pheochromocytoma is generally not difficult, but it is not easy to diagnose the crisis in emergency patients. By carefully consulting the medical history, close observation of the condition can provide Important diagnostic basis, the following conditions should be considered to consider the crisis:
1 Patients with recurrent episodes of hypertension or persistent hypertension have aggravated their history.
2 blood pressure fluctuations are extremely high, there is positional hypotension, or there is alternating hypertension and hypotension shock.
3 hypertension associated with hyperthermia, hyperhidrosis, weight loss, emotional excitement, anxiety, tachycardia, arrhythmia, limb tremor and other symptoms of excessive secretion of catecholamines.
4 hypertension associated with impaired glucose tolerance, diabetes, and even ketoacidosis.
5 due to trauma, minor surgery (such as tooth extraction), pressing the abdomen, urination and smoking and other factors induced hypertension history.
6 Abdomen touched the mass or B-ultrasound, CT and other found that there are substantial masses in the adrenal or abdominal aorta and other parts.
7 general antihypertensive drug treatment is ineffective, with the use of risperidin, guanethidine and other antihypertensive drugs released by catecholamines to increase blood pressure.
8 hypertension with poorly explained white blood cell increase.
Examine
Examination of pheochromocytoma crisis in the elderly
Determination of blood, urinary catecholamines and their metabolites are the most important basis for the diagnosis of pheochromocytoma. The catecholamines include dopamine, norepinephrine and adrenaline. The final metabolite of norepinephrine and epinephrine is 3-methoxy-4. -Hydroxymandelic acid (VMA), intermediate metabolites are mainly 3-methoxynorepinephrine (NMN) and 3-methoxy-adrenalin (MN), dopamine is a precursor of norepinephrine, not pheochromocytoma The main product of secretion, its final metabolite is high vanillic acid (HVA), malignant pheochromocytoma may produce more dopamine and HVA.
1. Determination of urinary catecholamines (UCA) and metabolites
Usually, the total amount of UCA and VMA is measured for 24h. Because the method is simple, rapid, and has high diagnostic value, it is the main basis for the diagnosis of pheochromocytoma. UCA and VMA of patients with persistent hypertension have a significant increase in UCA and VMA. Patients with paroxysmal hypertension who have urinary UCA and VMA during the onset of seizures can often be normal, and the retention of urine on the day of attack can increase the positive detection rate. The comparison of urine retention for 2 hours before and after the challenge test can help diagnose. 90% of functional pheochromocytoma can be diagnosed. If urinary norepinephrine and epinephrine can be measured, the positive rate of diagnosis can be improved and the diagnosis can be improved. In addition, urine NMN and MN are measured. Can further improve the positive rate of diagnosis, a variety of drugs and coffee, tea, bananas and other beverages and fruits can affect the secretion and interference of catecholamines. Therefore, before leaving the urine, you must stop the drug and fast for more than 2 days.
2. Determination of blood catecholamine
Because the concentration of catecholamine in blood is very low, the measurement is difficult, and the method is complicated by fluorescence method. The sensitivity and stability are not very satisfactory. In recent years, high performance liquid chromatography is used for separation and detection by electrochemical detector can be simultaneously quantified. Analyze adrenaline, norepinephrine and dopamine, but the equipment is expensive, the method is also more complicated, and it is not suitable for routine application. It can be used properly for the diagnosis of difficult cases.
3. Other laboratory inspections
In patients with pheochromocytoma, a considerable proportion of people have elevated blood glucose, and glucose tolerance is reduced. Individual patients may have a decrease in serum potassium, which is usually less than 3.0 mmol/L.
4. Excitation test and retardation test
For patients with intermittent episodes, especially those with intermittent episodes and short-term episodes, provocative tests can be performed. Commonly used are cold-press test, histamine test and glucagon test. Cold-press test is generally not performed separately, only for other excitations. In the controlled trials of the trial, all the challenge tests have certain risks, and even induce the crisis of the crisis. The alpha blocker should be prepared during the test, such as phentolamine (benzylamine). When the blood pressure reaches the positive standard, the phenol is injected immediately. Tolamin, to prevent blood pressure from continuing to rise, these tests have certain false positives and false negatives, and the results should be comprehensively analyzed.
During the period of persistent hypertension or high blood pressure, a block test can be performed. The phentolamine test is generally used. In the elderly, phentolamine should be injected from a small dose at a dose of 1.0 mg (normal dose). 5.0mg), in order to avoid hypotension or even shock, this test has few false negatives, but the false positive rate is high. In clinical practice, the challenge test is combined with the block test (phentolamine test). When the rise reaches the positive standard, the phentolamine test is started immediately, which not only prevents the blood pressure from rising in the test, but also improves the diagnostic value of the test.
5. Tumor localization examination
The pheochromocytoma localization examination is indispensable for surgical treatment. Most patients can accurately locate by non-invasive examination such as B-mode ultrasound, CT scan and magnetic resonance imaging (MRI). B-ultrasound is simple, rapid and economical. However, the accuracy is not as good as the latter two. CT scan is now more popular. It is often necessary to perform enhanced scans on soft tissue tumors. MRI has better resolution for some soft tissue tumors. It can be used selectively. When the posterior mediastinal tumor of the thoracic cavity is suspected, the chest X-ray is taken. Tablets have some help, suspected bladder pheochromocytoma, cystoscopy and cystography are essential, the authors concluded that 4 cases of bladder pheochromocytoma were diagnosed by cystoscopy, retroperitoneal angiography, adrenal angiography and intravenous pyelonephritis Examinations such as angiography have trauma or pain to the patient, and there is a risk of inducing a crisis. In the case of B-ultrasound, CT and MRI are becoming more and more popular, it is generally unnecessary, because the surgery needs to be done, it is sufficient. Select the application under the conditions of preparation.
Diagnosis
Diagnosis and diagnosis of pheochromocytoma crisis in the elderly
diagnosis
When suspected of this disease, the first should actively use alpha and beta blockers and other corresponding emergency treatment, while emergency diagnosis of B-ultrasound in the adrenal gland and bilateral aorta to detect tumors, if necessary, in the condition of the condition Under CT or MRI, make a diagnosis as clear as possible. After the crisis control, leave the urine or blood to measure UCA and VMA or other related tests, and finally confirm the diagnosis.
diagnosis
It must be differentiated from hypertensive crisis of hypertension, hypertensive encephalopathy, severe arrhythmia of coronary heart disease, acute myocardial infarction and septic shock.
