[Salt wasting syndrome caused by congenital, insufficient synthesis or aldosterone function--etiology, diagnosis and management].
Salt wasting syndrome is caused by a congenital or acquired synthesis disorder or by the aldosterone function disorder. It manifests itself by ionic disorders where the sodium and chlorine level decrease with the simultaneous potassium retention. Synthesised aldosterone is in the glomerular zone of the adrenal cortex. Symptoms of dyselectrolitemia are not distinctive, they develop within a few first days of life. The suction aversion, apathy, lack of growth or progressing, body mass loss is being noticed. The most often cause of salt wasting syndrome is the congenital cortical adrenal hyperplasia (CAH) caused by 21-hydroxylase enzyme deficit. The classic form with and without salt wasting (SW), as well as non-classic form is distinguished. The therapy of SW form depends on Hydrocortisone and Cortineff administering. The other forms of salt wasting syndrome occur not so often and these are: aldosterone synthesis deficit, dehydrogenase 3beta-hydroxysteroid deficit, lipoid cortical hyperplasia, adrenal hypoplasia congenital (AHC), adrenoleukodystrophy and pseudohypoaldosteronism. The knowledge of the symptoms and causes of salt wasting syndrome allows for the proper therapeutic management and contributes to the regular psychophysical infantile development of the children.
Basiak
Aleksander
A
Katedra i Klinika Endokrynologii Wieku Rozwojowego AM we Wrocławiu, Wrocław.
Wikiera
Beata
B
Noczyńska
Anna
A
D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis / Q000188:drug therapy; D000450:Aldosterone / Q000096:biosynthesis* / Q000097:blood*; D006801:Humans; D011189:Potassium Chloride / Q000097:blood; D012965:Sodium Chloride / Q000378:metabolism*; D013255:Steroid 21-Hydroxylase / Q000097:blood; D013577:Syndrome
D012965:Sodium Chloride; D000450:Aldosterone; D011189:Potassium Chloride; D013255:Steroid 21-Hydroxylase