What Is Adie Syndrome?

The main manifestations of Gordon syndrome are hyperkalemia, hyperchloremia, acidosis, and hyporenin hypertension, also known as familial hyperkalemia or type II pseudoaldosteronism. According to Achard, at least 90 cases of Gordon syndrome were reported worldwide by 2001. Among the 20-year-old patients with Gordon syndrome, about 80% have hypertension and have a family history. Due to congenital renal tubular dysfunction, renal sodium reabsorption increases, resulting in increased blood chlorine, increased blood volume, and increased blood pressure; due to suppressed renin secretion, plasma renin activity decreases; due to reduced potassium excretion Therefore, it produces hyperkalemia. Due to hyperkalemia, acidosis occurs. Patients have varying severity and different clinical manifestations. In addition to the typical clinical manifestations, short stature and mental retardation can be seen in a few cases.

GORDON syndrome

The main manifestations of Gordon syndrome are hyperkalemia, hyperchloremia, acidosis, and hyporenin hypertension, also known as familial hyperkalemia or type II pseudoaldosteronism. According to Achard, at least 90 cases of Gordon syndrome were reported worldwide by 2001. Among the 20-year-old patients with Gordon syndrome, about 80% have hypertension and have a family history. Due to congenital renal tubular dysfunction, renal sodium reabsorption increases, resulting in increased blood chlorine, increased blood volume, and increased blood pressure; due to suppressed renin secretion, plasma renin activity decreases; due to decreased potassium excretion by the kidneys Therefore, it produces hyperkalemia. Due to hyperkalemia, acidosis occurs. Patients have varying severity and different clinical manifestations. In addition to the typical clinical manifestations, short stature and mental retardation can be seen in a few cases.

Western Medicine Name

GORDON syndrome

Other name

Familial hyperpotassic hypertension or type II pseudoaldosteronism

The main symptoms

Hyperkalemia, hyperchloremia, acidosis

Contagious

Non-contagious

This disease is mostly thought to be due to congenital tubular defect, and renal sodium is heavy.

Prognosis: Gordon syndrome biochemical disorder begins at birth and hypertension develops later. Hypertension is less common in childhood and more common in adulthood. The prognosis depends on blood pressure levels. Patients who respond well to thiazide diuretics have fewer complications than patients with general hypertension and often have a normal lifespan.

Prevention: This disease is a rare autosomal dominant genetic disease and there is no effective prevention method. In the diagnosis and treatment of hypertension, we should be alert to the existence of the disease and further check when suspicious signs are found, so that early detection and early treatment of the disease have a better prognosis.

Thiazine diuretics are very effective in treating Gordon syndrome. Can lower blood pressure to normal levels, hyperkalemia, hyperchloremia, acidosis can be corrected, and even small doses of diuretics can appear hypokalemia, hypochloric alkalosis. Long-term use may, but is not common, produce hyperuricemia, hyperglycemia, and hypercalcemia. Promote low-dose diuretics and adjust the therapeutic dose according to changes in blood pressure and changes in blood potassium and blood chlorine. The sodium-restricted diet also achieved good therapeutic effects, which can improve hyperkalemia and hyperchloremia.

Laboratory tests: 1. Hyperkalemia is the basic condition for diagnosis and diagnosis of the disease, and it is advisable to check the blood potassium multiple times; 2. Plasma carbonate concentration decreases in most cases of hyperchloric acidosis, and arterial blood pH also decreases; 3. Plasma renin activity was significantly reduced. Plasma aldosterone levels were mostly normal but aldosterone levels were lower than the standard for normal potassium. The standard for hyperkalemia was low. Atrial natriuretic peptide is normal or slightly elevated; 4. Serum creatinine, urea nitrogen, renal creatinine clearance, and endogenous creatinine clearance often reflect normal renal function and normal urine concentration.

Other auxiliary tests: a sodium-restricted diet or treatment with thiazide diuretics, blood pressure and blood electrolyte disorders are improved or corrected.