Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Pediatric Case of Primary Sj$\ddot{o}$gren's Syndrome Associated with Nephrogenic Diabetes Insipidus and Renal Tubular Acidosis

신성요붕증과 신세뇨관산증을 동반한 일차성 쇠그렌 증후군의 1례

  • Choi, Jong Won (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Jung, You Jin (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Suh, Jin Soon (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Park, So Hyun (Department of Pediatrics, The Catholic University of Korea, College of Medicine) ;
  • Koh, Dae Kyun (Department of Pediatrics, The Catholic University of Korea, College of Medicine)
  • 최종원 (가톨릭대학교 의과대학 소아과학교실) ;
  • 정유진 (가톨릭대학교 의과대학 소아과학교실) ;
  • 서진순 (가톨릭대학교 의과대학 소아과학교실) ;
  • 박소현 (가톨릭대학교 의과대학 소아과학교실) ;
  • 고대균 (가톨릭대학교 의과대학 소아과학교실)
  • Received : 2012.03.18
  • Accepted : 2012.06.20
  • Published : 2012.10.31
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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.

쇠그렌 증후군은 주로 침샘과 눈물샘을 침범하는 자가 면역 질환으로, 폐나 간, 콩팥, 췌장, 피부, 신경계 등의 다른 장기 역시 침범하여 임상 증상을 나타내기도 한다. 성인의 경우 이런 다른 장기와 관련된 증상은 드물지 않으나 소아에서는 매우 드물다고 알려져 있다. 특히 콩팥을 침범한 쇠그렌 증후군은 성인의 경우 비교적 흔하지만 소아는 매우 드문데, 이에 본 저자들은 신성 요붕증과 신세뇨관 산증을 주 증상으로 진단한 소아의 일차성 쇠그렌 증후군 1례를 경험하였기에 보고한다.

Keywords

References

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