Childhood Kidney Diseases

  • 제13권2호
  • /
  • Pages.256-260
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  • 2009
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  • 2384-0242(pISSN)
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  • 2384-0250(eISSN)
대한소아신장학회 (Korean Society of Pediatric Nephrology)
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Russell-Silver 증후군에서의 만성 신부전

Chronic Renal Failure in Russell-Silver Syndrome

  • 안요한 (서울대학교 의과대학 소아과학교실) ;
  • 이세은 (서울대학교 의과대학 소아과학교실) ;
  • 강희경 (서울대학교 의과대학 소아과학교실) ;
  • 하일수 (서울대학교 의과대학 소아과학교실) ;
  • 정해일 (서울대학교 의과대학 소아과학교실) ;
  • 최용 (서울대학교 의과대학 소아과학교실)
  • Ahn, Yo-Han (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Lee, Se-Eun (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Kang, Hee-Gyung (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Ha, Il-Soo (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Cheong, Hae-Il (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Choi, Yong (Department of Pediatrics, Seoul National University College of Medicine)
  • 발행 : 2009.10.31
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초록

Russell-Silver 증후군은 자궁내 성장 지연, 특징적인 얼굴 기형, 저신장을 특징으로 하는 질환이다. Russell-Silver 증후군에서 동반되는 신질환은 말굽 신장, 신세뇨관 산증, 물콩팥증, 요관 깔때기막힘, 방광 요관 역류 등이 있다. 저자들은 Russell-Silver 증후군 환자에서 말기 신부전이 발생한 예를 경험하였으며 문헌 고찰에서 유사한 증례를 찾을 수 없었기에 보고하는 바이다.

The Russell-Silver syndrome (RSS) is a disease characterized by intrauterine growth retardation with preserved head circumference, facial dysmorphism and short stature. Reported renal and urinary manifestations of RSS include horseshoe kidney, renal tubular acidosis, hydronephrosis, ureteropelvic obstruction and vesicoureteral reflux. Here we report a case of end-stage renal disease associated with RSS, which, to the best of our knowledge, has not been reported yet.

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참고문헌

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