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KMID : 0359919960150030429
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Korean Journal of Nephrology
1996 Volume.15 No. 3 p.429 ~ p.433
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Absence of H+-ATPase in Collecting Tubules of Patient with Sjogren's Syndrome Complicated by distal Renal Tubular Acidosis
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김한욱
장은덕/신영신/방병기/김지연/한혁준/윤성노/윤건호/장윤식
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Abstract
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Distal renal tubular acidosis is characterized by a decrease in net H+ secretion in the collecting tubules such that the urine pH remains above 5.3. It is classified as hereditary, idiopathic and anquired forms associated with immune-mediated
disorders
surch as Sjogren's syndrome. The cellular mechanism underlying H+ -secretory abnormalities are described as transepithelial voltage defects, permjeability defect, and proton-secretory def3ect, and proton secretory defect is charactenzed
physiologically
by a blunted rate of hydrogen ion secretion in response to the administration of Na2SO4 or furosemide. A primary abnormality in proton secretion could arise from a decrease in the quantity of H+-ATPase in intercalated cells. A patient with
Sjogren's
syndrome and distal renal tubular acidosis is described, whose kidney biopsy was examined by immunohistochemical stain with an antibody to kidney H+-ATPase and compared with normal human kidney. Staining with the anti-H+-ATPase antibody in normal
kidney
was deteted in intercalated cells in the collecting duct. But the patient's kidney biopsy was devoid of any anti-H+-ATPase staining in the intercalated cells. This report demonstrated that the abnormality of proton secretion in the patient with
distal
renal tubular aciosis may be due to the defect of H+-ATP in the intercalated cells.
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KEYWORD
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