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| 신집합관 수소이온펌프 결손을 보인 운위부 신세뇨관성 산증 동반 Siogren `s syndrome 환자 1예 |
| 김한욱 , 신영신 , 김지연 , 함혁준 , 윤성노 , 윤건호 , 장윤식 , 김진 , 장은덕 , 방병기 |
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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 tu- bules such that the urine pH remains above 5.3. M is classified as hereditary, idiopathic and acquired forms associated with immune-mediated disorders such as Sjogren's syndrome. The cellular mechanism underlying H -secretory abnormalities are described as transepithelial volvge defects, permeability defect, and proton-secretory defect, and proton secretory defect is characterized physiologically by a blunted rate of hydrogen ion secretion in response to the administration of NazSOy or furosemide. A primary abnormality in proton secretion could arise from a decrease in the quantity of H'-ATPase in inter- calated cells. A patient with Sjagren's syndrome and aistal renal tubular acidosis is descrihed, whose kidney biopsy was examined by immunohistochemi- cal 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 detected 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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