Document Detail


Gitelman syndrome: pathophysiological and clinical aspects.
MedLine Citation:
PMID:  20650971     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
Giltelman syndrome (GS) is a recessive salt-losing tubulopathy of children or young adults caused by a mutation of genes encoding the human sodium chloride cotransporters and magnesium channels in the thiazide-sensitive segments of the distal convoluted tubule. The plasma biochemical picture is characterized by hypokalemia, hypomagnesemia, hypocalciuria, metabolic alkalosis and hypereninemic hyperaldosteronism. However, patients with GS present some clinical and biochemical alterations resembling that observed in thiazide diuretics abuse. On the pathophysiological point of view, GS represents a useful and interesting human model to better understand the clinical consequences of plasma hydro-electrolytes and acid-base derangements, associated with multiple hormonal alterations. The impact of this complex disorder involves cardiovascular, muscle-skeletal and some other physiological functions, adversely affecting the patient's quality of life. This review tries to summarize and better explain the linkage between the electrolytes, neurohormonal derangements and clinical picture. Moreover, the differential diagnosis between other similar electrolyte-induced clinical disorders and GS is also discussed.
Authors:
G Graziani; C Fedeli; L Moroni; L Cosmai; S Badalamenti; C Ponticelli
Publication Detail:
Type:  Journal Article     Date:  2010-07-22
Journal Detail:
Title:  QJM : monthly journal of the Association of Physicians     Volume:  103     ISSN:  1460-2393     ISO Abbreviation:  QJM     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-24     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9438285     Medline TA:  QJM     Country:  England    
Other Details:
Languages:  eng     Pagination:  741-8     Citation Subset:  IM    
Affiliation:
Nephrology and Dialysis Unit, IRCCS Istituto Clinico Humanitas, Rozzano, Milano, Italy. giorgio.graziani@humanitas.it
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