Barter syndrome
Contents:
Description:
Barter syndrome - a combination of the water and electrolytic and hormonal disturbances characterized by the increased removal To, Na and About kidneys, a hypopotassemia, a hyper aldosteronism, гиперренинемиеп and normal arterial pressure. The syndrome usually appears in the childhood as sporadic or family, usually autosomal and recessive disturbance.
Reasons Barter syndrome:
Barter syndrome - result of difficult disturbance of transport of electrolytes in a kidney. The pochechnokanaltsevy pathology which is its cornerstone is not established. Defects of transport mechanisms which could have etiological value presumably connected with proximal tubules, a thick part of the ascending segment of a Henle's loop and a distal part of nephron.
Barter syndrome symptoms:
There is a hypopotassemia, a hyponatremia and a hypochloraemia, and each of these factors promotes stimulation of release of a renin that leads to a hyperplasia of cells of the juxtaglomerular device. The increased Aldosteronum level is noted. The hypopotassemia is not eliminated with correction of a hyper aldosteronism. The hyponatremia causes chronically low volume of plasma which is shown by the ABP normal level, despite high levels of a renin and angiotensin, and the broken pressor reaction to administration of angiotensin. Often the metabolic alkalosis develops. Aggregation of thrombocytes is oppressed. The hyperuricemia and a hypomagnesiemia are possible.
Excretion of prostaglandins and kallikrein with urine increases. Braking of synthesis of prostaglandins leads to correction of the majority of disturbances, but the hypopotassemia is eliminated only partially.
At the struck children the growth inhibition is observed, and they look exhausted. Muscular weakness, a polydipsia, a polyuria and mental retardation are possible.
Diagnosis:
The syndrome of Barter is differentiated from other diseases accompanied with a hyper aldosteronism on absence of hypertensia (unlike primary hyper aldosteronism) and hypostases (unlike a secondary aldosteronism). If symptoms of a disease for the first time come to light at adults, such reasons as vomiting and secret abuse of diuretics have to be excluded with complete certainty.
Treatment of a syndrome of Barter:
Additional administration of potassium plus Spironolactonum, Triamterenum, amiloride, propranolol or indometacin usually allows to adjust the majority of symptoms, but no medicines eliminate completely a hypopotassemia. Indometacin in a dose of 1-2 mg/kg/days usually supports plasma potassium at the level close to the lower bound of norm.
