Bartter’s Syndrome – Hypokalemic Renal Tubular Syndrome
Bartterov syndróm – hypokaliemický renálnytubulárny syndróm
Poruchy metabolizmu kália patria k častým sprievodným znakom ochorení obličiek. Znížené hladiny kália súsúčasťou renálnej tubulárnej acidózy I. a II. typu alebo renálnych tubulárnych syndrómov (Bartterov, Gitelmanova Liddleho syndróm), ktoré sú spojené s mutáciou génov, regulujúcich metabolizmus nátria, kália a chloridovv distálnom nefróne. Do tejto skupiny ochorení sa zaraďuje aj Bartterov syndróm – autozomálne recesívne ochorenie,charakterizované kombináciou vodných, elektrolytových a hormonálnych abnormalít. Syndróm sprevádzajú zvýšenéstraty kália, nátria, kalcia a chloridov močom, hypokaliémia, hyperaldosteronizmus, hyperreninémia a normálnealebo znížené hodnoty krvného tlaku. Práca podáva prehľad o etiopatogenéze, laboratórnom a klinickom obraze, akoaj o možnostiach terapeutického ovplyvnenia Bartterovho syndrómu.
Klíčová slova:
Bartterov syndróm, hypokaliemické renálne tubulárne syndrómy, hypokaliémia, metabolická alkalóza.
Authors:
K. Gazdíková; F. Gazdík
Authors‘ workplace:
Klinika farmakoterapie Ústavu preventívnej a klinickej medicíny, Bratislava
Published in:
Čas. Lék. čes. 2003; : 474-478
Category:
Overview
Hypokalemia is associated with some renal diseases manifested by renal tubular acidosis (type I and II) or by renaltubular syndrome (Bartter’s, Gitelman’s and Liddle’s syndrome). Bartter’s syndrome, originally described by Batterand colleagues in 1962, is a set of closely related renal tubular disorders characterized by hypokalemia, hypochloremia,metabolic alkalosis and hyperreninemia with normal blood pressure. The underlying renal abnormality resultsin excessive urinary losses of sodium, chloride, potassium and calcium. Muscle weakness, polydipsia, polyuria andmental retardation can be also present. Affected children have poor growth rates and they appear malnourished. Thearticle is focused on ethiopathogenesis, laboratory and clinical characteristics and on the treatment of Bartter’ssyndrome.
Key words:
Bartter’s syndrome, hypocalemic renal tubular syndrome, hypokalemia, metabolic alkalosis.
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Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain management Dental HygienistArticle was published in
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