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Spitzer-Weinstein Sendromu: Tip 4 Renal Tubuler Asidozun bir Formu ve İndapamide Cevabı: Bir Olgu Sunumu

The Spitzer-Weinstein Syndrome: One Form of Type IV Renal Tubular Acidosis and Its Response to Indapamide: A Case Report

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DOI: 
DOI 10.5262/tndt.2012.1002.20
Abstract (2. Language): 
Spitzer-Weinstein is rare disorder characterized by thiazide responsive hyperkalemia and normal anion gap metabolic acidosis, similar to Gordon syndrome.The hyperfunction of thiazide–sensitive Na-Cl cotransporter (TSC) is the main pathophysiological mechanism. We present a 21-year-old male with normal blood pressure, persistently elevated serum potassium, and metabolic asidosis. The diagnosis of Spitzer-Weinstein syndrome was made by clinical pictures and thiazide test. After using 1.5 mg indapamide from a group of thiazide diuretics, his serum potassium decreased from 6.68 mmol/L to 3.54 mmol/L and the daily urine potassium excretion increased from 13.2 mmol to 34.1 mmol. This patient then took indapamide 1.5 mg daily. The persistent hyperkalemia and metabolic acidosis were corrected. Thiazide, a powerful inhibitor of TSC, proved to be a useful tool for the diagnosis and treatment of Spitzer-Weinstein syndrome.
Abstract (Original Language): 
Spitzer-Weinstein sendromu, Gordon sendromuna benzer olarak tiazide cevap veren hiperkalemi ve normal anyon açıklı metabolik asidoz ile karakterize nadir bir hastalıktır. Temel patofizyolojik mekanizma tiazid-duyarlı Na-Cl kotransporterın (TSK) hiperfonksiyonudur. Normal kan basıncı, persistan serum potasyum yüksekliği ve metabolik asidozlu 21 yaşında erkek bir olgu sunulmuştur. Spitzer-Weinstein sendromu tanısı klinik bulgular ve tiazid testi ile konulmuştur. Tiazid grubu diüretik olan indapamid 1,5 mg tablet alındıktan sonra serum potasyumu (K): 6,68 mmol/L den 3,54 mmol/L’e geriledi ve 24 saatlik idrar K seviyesi 13,2 mmol/gün den 34,1 mmol/gün’e yükseldi. Bu hasta daha sonra indapamid 1,5 mg/gün tedavisi aldı. Persistan hiperkalemi ve metabolik asidoz düzeldi. Tiazidler güçlü bir TSK inhibitörü olup, Spitzer-Weinstein sendromun tanı ve tedavisinde bir araçtır.
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