Aldosterone deficiency

　　According to different etiology and pathogenesis, aldosterone deficiency can be divided into 4 types: congenital primary aldosterone deficiency, acquired primary aldosterone deficiency, acquired secondary aldosterone deficiency, and pseudoaldosterone deficiency. Primary and secondary are divided according to the ratio of plasma renin activity (PRA) to aldosterone. The ratio of primary aldosterone deficiency is lower than normal (high renin hypoaldosteronism), while the ratio of secondary is normal (low renin hypoaldosteronism).

　　The pathogenesis of this disease is not yet fully understood, and it can be divided into congenital and acquired according to the genetic basis. Acquired secondary aldosterone deficiency is the most common type of this disease, with various kidney diseases as the main causes, such as chronic glomerulonephritis, interstitial nephritis, chronic glomerulonephritis, renal amyloidosis, kidney stones, renal cysts, and others; kidney damage caused by systemic diseases such as diabetic nephropathy, lupus nephritis, multiple myeloma, gouty kidney, and others; other diseases such as liver cirrhosis, sickle cell anemia, hemochromatosis, acute respiratory distress syndrome, and others; long-term use of beta-blockers, prostaglandin inhibitors (such as indomethacin) can also cause this disease. Aldosterone deficiency secondary to decreased renin levels is the pathophysiological feature of this type, hence called hyporeninemic hypoaldosteronism. The focus of acquired primary aldosterone deficiency is in the adrenal glands, with various reasons causing the destruction of cortical tissue, leading to adrenal cortical insufficiency, so most patients can also have a deficiency of glucocorticoids, and selective primary acquired aldosterone deficiency is rare. Autoimmune adrenal cortical insufficiency, infection (tuberculosis is common), sepsis, metastatic tumors, and others can cause structural damage to the adrenal tissue; heparin can directly inhibit the biosynthesis of aldosterone. Congenital primary aldosterone deficiency is related to genetics, and it is due to enzyme defects causing aldosterone synthesis disorders. The lack of cholesterol carbon chain enzyme prevents cholesterol from being converted to Δ-5 pregnenolone, so it cannot produce any kind of steroid hormone. Pseudoaldosteronism (PHA) deficiency is not a true lack of aldosterone, but due to aldosterone receptor or post-receptor factors that reduce or eliminate the biological activity of aldosterone, the clinical manifestations are similar to those of aldosterone deficiency, hence called pseudoaldosteronism or aldosterone resistance syndrome.

　　The focus of acquired primary adrenocortical insufficiency is in the adrenal glands, multiple reasons damage the cortical tissue, leading to adrenal cortical insufficiency, so most patients may have a deficiency of glucocorticoids, and selective primary acquired adrenocortical insufficiency is rare.

　　Adrenocortical insufficiency mainly manifests as thirst, polydipsia, nausea, vomiting, anorexia, weakness, blood pressure decrease, arrhythmia, and other symptoms of hyperkalemia and hyponatremia. The symptoms of this disease vary with different types, such as acquired secondary adrenocortical insufficiency is more common in people aged 50 to 70, and the clinical manifestations are often inexplicable, chronic asymptomatic hyperkalemia; Congenital primary adrenocortical insufficiency begins in neonates or infants, and may have severe dehydration, hyponatremia, vomiting, hyperkalemia, and metabolic acidosis.

　　1. Hyperkalemia:The blood potassium levels caused by this syndrome are usually between 5.5 and 6.5mmol/L, mild hyperkalemia has no obvious clinical manifestations, and those with significantly increased blood potassium levels may have:

　　1. Symptoms of cardiovascular system: Manifested as a decrease in heart rate, irregular heartbeat, mainly ventricular premature contraction, severe cases may develop致命性 ventricular fibrillation or the heart stops beating during diastole; Electrocardiogram shows typical manifestations indicating blood potassium levels above 7mmol/L, often with a tall 'T' wave and a narrow base, atrioventricular or intraventricular conduction block, reduced R wave, deep S wave, ST segment depression, and ventricular fibrillation.
　　2. Neuro-muscular system symptoms: Early manifestations are mainly abnormal sensation, extreme fatigue, muscle cramps, disappearance of tendon reflexes; due to vasoconstriction, the skin can become pale and moist; due to respiratory muscle paralysis, speaking can become difficult, voice can become hoarse, and breathing can become difficult; a few patients may present with nausea, diarrhea, and intestinal cramps due to gastrointestinal spasm.

　　II. Hyponatremia and metabolic acidosis:Hyponatremia in patients with aldosterone deficiency usually develops slowly, mainly manifested as fatigue, weakness, thirst, postural dizziness, dullness of sensation, etc.; but children with congenital primary aldosterone deficiency may also have obvious neurological symptoms due to a sharp drop in blood sodium, such as nausea, severe vomiting, headache, and even convulsions, coma, etc., and metabolic acidosis is mainly manifested as deepening and accelerating respiration.

　　III. Manifestations of primary diseases:Such as clinical manifestations of renal insufficiency, diabetes, and other corresponding diseases.

　　Currently, there is no particularly effective treatment for aldosterone deficiency. During treatment, especially when a large amount of fluid is administered and corticosteroids are given at the same time, one should be vigilant for the occurrence of hypokalemia. The diet of patients should be light and easy to digest, with more vegetables and fruits, reasonable dietary搭配, and attention to adequate nutrition. In addition, patients should also pay attention to avoiding spicy, greasy, and cold foods.

　　Aldosterone deficiency mainly includes renal function tests and blood tests. Renal function is often abnormally moderate to severe, with increased plasma urea nitrogen and creatinine, decreased creatinine clearance, and hyperchloric metabolic acidosis; blood renin activity and 24-hour urine aldosterone concentration are all decreased (except for pseudo-aldosterone deficiency); carbon dioxide combining power and pH value are decreased, blood potassium is increased, blood sodium is decreased or normal, and laboratory examination of primary diseases is abnormal. In addition, there are abdominal ultrasound, electrocardiogram, electromyogram, etc.

　　Two points to pay attention to in the diet of patients with aldosterone deficiency

　　1. The diet should mainly consist of light and easy-to-digest foods, and attention should be paid to dietary regularity. The diet of patients should be light, easy to digest, with more vegetables and fruits, reasonable dietary搭配, and attention to adequate nutrition. In addition, patients should also pay attention to avoiding spicy, greasy, and cold foods.

　　2. Consult the doctor for detailed information and establish dietary standards.

　　Since this condition is mainly caused by aldosterone deficiency, the fundamental treatment method is to supplement salt皮质激素. After supplementing salt皮质激素, clinical manifestations can be relieved or disappear, and disorders of water and salt metabolism and acid-base balance can also be corrected. The main drugs include:
　　1. Alpha-fluorohydrocortisone is generally taken orally at 8 am with a dose of 0.05 to 0.1 mg. Attention should be paid to individualization of dosage and adjustment of medication dose according to clinical response; if edema, hypertension, or hypokalemia occurs, the dose should be reduced, and conversely, the dose can be appropriately increased.
　　2. Acid deoxy皮质酮 (DOCA) oil preparation, 1-2mg per day or every other day 2.5-5mg, intramuscular injection, suitable for patients who cannot take oral medications;
　　3. Triamcinolone acetate 25-50mg, intramuscular injection, the effect can last for 3-4 weeks.
　　At the same time, attention should be paid to:
　　3. For mild hyperkalemia with no obvious symptoms and electrocardiogram changes, no special treatment is required. The main methods to restore blood potassium levels to normal are to discontinue drugs that may increase blood potassium levels, limit potassium intake, and supplement salt皮质激素. However, regular follow-up of blood potassium levels should be noted. When blood potassium concentration is above 6.0-6.5 mmol/L, it can cause severe hyperkalemia crisis, which should be treated urgently to reduce blood potassium levels; close observation of changes in the condition should be made during the rapid use of sodium salts to prevent pulmonary edema. Calcium agents can reduce the toxic effects of potassium salts on the heart and can correct metabolic acidosis to a certain extent, but attention should be paid to not using them with alkaline drugs to avoid precipitation. Caution should also be exercised when using calcium agents in patients who have already been treated with digitalis drugs. When using ion exchange resins for intestinal potassium excretion, attention should be paid to the fact that this drug is prone to clumping and causing intestinal obstruction. Before taking the medicine, 70% sorbitol 15-20ml can be taken at the same time to soften the stool; if oral intake is not possible, 200ml of 20% sorbitol can be enema.
　　1. After hyperkalemia occurs, it is necessary to discontinue drugs that may cause an increase in blood potassium levels, such as heparin, β-receptor blockers, prostaglandin synthase inhibitors, potassium-sparing diuretics, and ACEIs.
　　2. Caution should be exercised when using hyperosmolar glucose therapy for hyperkalemia in diabetic patients (glucose insulin) to avoid causing an increase in blood sugar.
　　4. Sufficient supplementation of salt皮质激素 is necessary; if the supplementation of salt皮质激素 is insufficient, it may further increase blood potassium levels.
　　For severe cases of congenital primary aldosterone deficiency with significant dehydration and sodium loss, adrenal crisis may occur. Supplementing sodium salts is an important measure for expanding blood volume and correcting shock. Close attention should be paid to changes in the condition during the process of rapid and large-volume fluid replacement to prevent pulmonary edema.