Bart 's Syndrome

Bartte's syndrome is a group of conditions mainly characterized by hypokalemia, alkalosis, and polyuria. The etiology and pathogenesis are unknown, and it is believed that the function of active sodium chloride absorption in the thick segment of the ascending branch of the renal tubules is impaired, and sodium chloride entering the distal renal tubules increases, exacerbating sodium reabsorption and sodium ions in this segment of tubules. With the exchange of hydrogen ions and potassium ions, potassium and hydrogen ions are lost from the urine, and the remaining unresorbed sodium salts are also excreted from the urine, resulting in hypokalemia, hypochloric alkalosis and salt loss. Potassium and salt loss stimulate the secretion of prostaglandins and renin, renin stimulates the adrenal cortex to secrete aldosterone to increase sodium reabsorption and potassium excretion, and prostaglandin has a diuretic effect. This syndrome is more common in children and can be sporadic or have a family history. The child has stunted growth and development, polyuria and nocturia, susceptibility to thirst, preference for salty food, loss of appetite, nausea, weak muscle strength, sometimes difficulty in movement, performance of soft paralysis, and a few may have twitching. Laboratory tests showed that serum potassium and blood chlorine were reduced, alkalosis, serum renin concentrations were significantly increased, urine prostaglandin (PGE) and aldosterone concentrations were also increased, and renal concentration function was decreased, but the dilution ability was good, and other renal functions were normal. Although there is hyperrenin and hyperaldosteroneemia, the patient does not have hypertension, which is a characteristic of this disease. Pathological examination of the kidney showed that the juxtaglomerular apparatus (the renin-secreting site) proliferated, the number of medullary interstitial cells (the main site of the prostaglandin-secreting) increased, and the renal tubular cells had degenerative changes and atrophy due to chronic potassium deficiency. Anti-inflammatory drugs can be used to inhibit the synthesis of prostaglandins, while supplementing potassium salts can reduce symptoms; you can also try anti-aldosterone medicament amphitamine to improve various metabolic disorders, but urine excretion is still increasing. This disease should be distinguished from various diseases that cause hypokalemia and hypochlorine alkalosis, such as primary aldosteronism, and hypokalemia caused by long-term application of diuretics. Bart syndrome is a refractory disease. It is a secondary aldosteronism syndrome caused by glomerular cell proliferation and secretion of large amounts of renin. The clinical manifestations are mainly muscle weakness, periodic paralysis, arrhythmia, intestinal paralysis and other hypokalemia symptoms, anxiety, increased nocturia, and osteoporosis.

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