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Journal of the Korean Society of Pediatric Nephrology 2012;16(2): 126-131. doi: https://doi.org/10.3339/jkspn.2012.16.2.126
신성요붕증과 신세뇨관산증을 동반한 일차성 쇠그렌 증후군의 1례
최종원, 정유진, 서진순, 박소현, 고대균
1가톨릭대학교 의과대학 소아과학교실
2가톨릭대학교 의과대학 소아과학교실
3가톨릭대학교 의과대학 소아과학교실
4가톨릭대학교 의과대학 소아과학교실
5가톨릭대학교 의과대학 소아과학교실
A Pediatric Case of Primary Sj$ddot{o}$gren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis
Jong Won Choi, You Jin Jung, Jin Soon Suh, So Hyun Park, Dae Kyun Koh
1Department of Pediatrics, The Catholic University of Korea, College of Medicine
2Department of Pediatrics, The Catholic University of Korea, College of Medicine
3Department of Pediatrics, The Catholic University of Korea, College of Medicine
4Department of Pediatrics, The Catholic University of Korea, College of Medicine
5Department of Pediatrics, The Catholic University of Korea, College of Medicine
Received: March 18, 2012;  Revised: June 15, 2012.  Accepted: June 20, 2012.
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ABSTRACT
Sj$ddot{o}$gren's syndrome (SS) is an autoimmune disorder primarily affecting the salivary and lacrimal glands. In addition, extra-glandular manifestations involving the lungs, liver, kidneys, pancreas, skin and central nervous system were reported in patients with SS. These extra-glandular manifestations are not rare in adult patient, but are very rare in pediatric SS. Renal manifestations are relatively common in adult SS, but are rarely reported in childhood SS. We experienced a girl with primary SS manifested with nephrogenic diabetes insipidus and renal tubular acidosis.
Key words: Sj$\ddot{o}$gren's syndrome | Diabetes insipidus | Renal tubular acidosis | Child
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