Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Case of Neurofibromatosis with Invasion of Bladder

방광을 침범한 신경섬유종증 1례

  • Kim, Mi-Kyung (Department of Pediatrics, College of Medicine, Korea University) ;
  • Park, Cheol (Department of Pediatrics, College of Medicine, Korea University) ;
  • Kim, Min-Sang (Department of Pediatrics, College of Medicine, Korea University) ;
  • Yim, Hyung-Eun (Department of Pediatrics, College of Medicine, Korea University) ;
  • Yoo, Kee-Hwan (Department of Pediatrics, College of Medicine, Korea University) ;
  • Hong, Young-Sook (Department of Pediatrics, College of Medicine, Korea University) ;
  • Lee, Joo-Won (Department of Pediatrics, College of Medicine, Korea University)
  • 김미경 (고려대학교 의과대학 소아과학교실) ;
  • 박철 (고려대학교 의과대학 소아과학교실) ;
  • 김민상 (고려대학교 의과대학 소아과학교실) ;
  • 임형은 (고려대학교 의과대학 소아과학교실) ;
  • 유기환 (고려대학교 의과대학 소아과학교실) ;
  • 홍영숙 (고려대학교 의과대학 소아과학교실) ;
  • 이주원 (고려대학교 의과대학 소아과학교실)
  • Received : 2012.03.14
  • Accepted : 2012.04.09
  • Published : 2012.04.30
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Abstract

Neurofibromatosis is a rare systemic disease, and genitourinary tract involvement is especially uncommon. Bladder is the most frequently involved organ in the genitourinary tract. Bladder neurofibromatosis may present as a diffuse infiltrative process or an isolated neurofibroma. The symptoms vary, ranging from urinary incontinence to retention. Treatment is usually conservative. The patient should be worked up to rule out other manifestation of tumor enlargement and followed to evaluate the development of new lesion. We report a case of the development of invasion of bladder in a patient with neurofibromatosis.

신경섬유종증은 드문 전신질환 중의 하나로 비뇨생식기계를 침범하는 경우는 극히 드물다고 알려져 있으나 비뇨생식기계를 침범하는 경우에는 그 기관으로 방광이 가장 흔하다. 비뇨생식기계를 침범하는 경우 증상이 나타나기 전까지 그 침범여부를 알기 어려운 경우가 많기 때문에 정기적인 추적관찰 중 비뇨생식기계 증상이 나타난다면 정밀검사를 시행하여 침범상태를 정확히 판단할 필요가 있겠다. 치료 방법으로는 보존적인 치료가 대부분이지만 종양의 크기가 클 경우 종양절개를 하는 경우도 있으나 매우 드물고, 보존적 치료와 동시에 종양의 크기 증가여부에 대하여 정기적인 추적관찰이 요구된다. 저자들은 소아에서 방광을 침범한 신경섬유종증이 증상을 나타낸 경우로 보존적 치료 후 경과관찰 중인 환아 1례를 경험하였기에 보고하는 바이다.

Keywords

References

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