전압 장애성 원위부 신세뇨관성 산증과 신성 요붕증으로 발현한 Sj gren 증후군 1예
Sj gren's syndrome associated with voltage defect distal renal tubular acidosis and nephrogenic diabetes insipidus
소속 상세정보
이경주/Kyong Joo Lee
송준호/문계혁/이승우/김문재/김태숙/최원식/Kyae Huk Moon/Joon Ho Song/Seoung Woo Lee/Moon-Jae Kim/Tae Suk Kim/Wonsick Choe
KMID : 0882420010600050485
Abstract
저자들은 전압 장애성 원위부 신세뇨관성 산증과 신성 요붕증이 동시에 발현된 Sj gren 증후군 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Distal renal tubular acidosis is a condition characterized by an inability of the distal nephron to acidify urine, causing hyperchloremic metabolic acidosis. Distal renal tubular acidosis is classified as proton secretory defect, permeability
defect and
voltage defect based on its pathophysiology. In the former two, serum level of potassium decreases due to increased excretion of potassium. But in the latter (voltage defect), hyperkalemia is characteristic by impaired the generation of an
optimal
electrical gradient for hydrogen ion and potassium secretion.
We experienced a case of Sj gren's syndrome associated with both voltage defect distal renal tubular acidosis and nephrogenic diabetes insipidus. The patient was a 58- year-old woman who complained of general weakness, nausea and xerostomia.
Laboratory
analysis showed metabolic acidosis with alkaline urine and hyperkalemia. Anti-nuclear antibody and anti-ds DNA antibody were positive. She presented with polyuria, low urine osmolarity and inadequate response to DDAVP. The response to Shirmer
test
was
decreased. Salivary scintigraphy showed decrease of uptake in the parotid and submandibular salivary glands. We believe this is the first case report in which Sj gren's syndrome is associated with both voltage defect distal renal tubular acidosis
and
nephrogenic diabetes insipidus.
키워드
Sj gren's syndrome; Acidosis; Renal tubular; Diabetes insipidus; Nephrogenic;
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