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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

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2012
DOI: 
DOI 10.5262/tndt.2012.1002.20

Key Words:

Keywords (Original Language):

Author NameUniversity of Author
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.
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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.
FULL TEXT (PDF): 
PDF icon arastrmx_172977_21_pp_201-204.pdf
  • 2
201-204
  • Turkish

REFERENCES

References: 

1. Spitzer A, Edelman CM, Goldberg LD, Henneman PH: Short
stature, hyperkalemia, and acidosis; A defect in renal transport of
potassium. Kidney Int 1973; 251-257
2. Wu MS, Hong JJ, Lin JL, Yang CW, Chien HC: Multiple tubular
dysfunction induced by mixed Chinese herbal medicines containing
cadmium. Nephrol Dial Transplant 1996; 11; 867-870
3. Colussi G, Rombola G, Brunati, De Ferrari ME: Abnormal
reabsorbtion of Na/Cl by the thiazide-inhibitable transporter of the
distal convoluted tubule in Gitelman’s syndrome. Am J Nephrol
1997; 17: 103-111
4. Margolis BL, Lifschitz MD: The Spitzer-Weinstein syndrome:
One form of type IV renal tubular acidosis and its response to
hydrochlorothiazide. Am J Kidney Dis 1986; 7 (3): 241-244
5. Helmut G. Rennke, Bradley M. Denker, Burton David Rose: Renal
pathophysiology: The Essentials (2nd ed). Lippincott Williams &
Wilkins, 2007; 175-199
6. Kamel KS, Ethier JH, Richardson RM, Bear RA, Halperin ML:
Urine electrolytes and osmolality: When and how to use them. Am
J Nephrol 1990: 10: 89-102
7. West ML, Bendz O, Chen CB, Singer GG, Richardson RM,
Sonnenberg H, Halperin ML: Development of a test to evaluate
the transtubular potassium concentration gradient in the cortical
collecting duct in vivo. Miner Electrolyte Metab 1986; 12: 226-233
8. Choi MJ, Ziyadeh FN: The Utility of the transtubular potassium
gradient in the evaluation of hyperkalemia. J Am Soc Nephrol 2008;
19: 424-426
9. Reddy P: Clinical approach to renal tubular acidosis in adult
patients. Int J Clin Pract 2011; 65 (3): 350-360
10. Topaloglu R, Rosa FC: Renal Tubular Acidosis: European Society
for Pediatric Nephrology Handbook. Lyon: Medcom, 2002;
221-224
11. Weng CH, Cheng JW, Hung CC, Wu MS, Yang CW, Chang CT:
A young female with Spitzer-Weinstein Syndrome diagnosed by
Thiazide Test. Renal Failure 2007: 29: 239-241
12. Herrin JT: Renal Tubular Acidosis In: Barratt TM, Avner ED,
Harmon WE. Pediatric Nephrology. Baltimore: Lippincott
Williams&Wilkins, 1999; 565-577
13. Take C, Ikeda K, Kurasawa T, Kurokawa K: Increased chloride
reabsorption as an inherited renal tubular defect in familial type II
Pseudohypoaldosteronism. N Engl J Med 1991; 324: 472-476

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