bubble_chart Overview

It is an endocrine disorder characterized by the continuous or intermittent release of large amounts of Black Catechu phenols and amines from the adrenal medulla, sympathetic ganglia, and other chromaffin tissues, leading to persistent or paroxysmal hypertension and causing dysfunction and metabolic disturbances in multiple organs.

bubble_chart Diagnosis

1. Medical History and Symptoms:

The age of onset is mostly between 20 and 50 years. The main symptoms are often paroxysmal hypertension, with varying duration and frequency of episodes. Blood pressure is usually normal but can rise to 200–300 mmHg systolic and 130–180 mmHg diastolic during attacks, accompanied by headache, pallor, profuse sweating, tachycardia, chest discomfort, nausea, vomiting, blurred vision, etc. After the attack subsides, symptoms such as facial and skin flushing, generalized fever, salivation, miosis, and increased urine output may occur. Some cases may present with sustained hypertension or sustained hypertension with paroxysmal exacerbations and the aforementioned sympathetic nervous system excitation symptoms. If hypotension upon standing or significant blood pressure fluctuations are observed, this condition should be suspected. Rarely, patients may exhibit hypotension, shock, or alternating episodes of hypertension and hypotension.

2. Physical Examination Findings:

During the interictal period, patients may show no obvious signs or only cardiomegaly, with a few cases presenting palpable abdominal masses. During an attack, blood pressure rises, and symptoms may include clammy skin, pallor, rapid pulse, arrhythmia, diplopia, etc.

3. Auxiliary Examinations:

(1) Blood and urine catecholamines and their metabolites, such as vanillylmandelic acid (VMA), metanephrine (MN), and normetanephrine (NMN), collectively referred to as total metanephrines (TMN), are significantly elevated. A level more than twice the upper limit of normal is highly suggestive of the diagnosis.

(2) A glucagon stimulation test may be performed during non-attack periods, while a phentolamine blockade test can be conducted during interictal phases.

(3) Imaging modalities such as ultrasound, CT, MRI, iodine-123 metaiodobenzylguanidine (MIBG) scintigraphy, and venous catheterization for segmental blood sampling can help localize the tumor.

4. Differential Diagnosis:

This condition should be differentiated from angina pectoris, unstable essential hypertension, menopausal syndrome, hyperthyroidism, and other disorders with paroxysmal hypertension, such as brain tumors, tabes dorsalis, acute porphyria, and lead poisoning.

bubble_chart Treatment Measures

Patients with suspected or confirmed diagnosis of the disease should be hospitalized for treatment. In cases of sudden hypertensive crisis, immediate and aggressive intervention is required: promptly administer 1–5 mg of phentolamine intravenously to lower blood pressure to around 21/13 kPa (160/100 mmHg), followed by a slow intravenous infusion of 10–50 mg in 5% glucose saline. Concurrently, attention should be given to managing potential arrhythmias and heart failure.