우연히 발견된 편측성 다낭성 신 이형성증과 폐의 선천성 낭성 선종양 기형이 합병된 증례

A case of multicystic dysplastic kidney and cystic adenomatoid malformation of the lung identified as incidental findings

  • 이선주 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이지헌 (가톨릭대학교 의과대학 소아과학교실) ;
  • 김현희 (가톨릭대학교 의과대학 소아과학교실) ;
  • 김소영 (가톨릭대학교 의과대학 소아과학교실) ;
  • 한승훈 (가톨릭대학교 의과대학 소아과학교실) ;
  • 황자영 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이원배 (가톨릭대학교 의과대학 소아과학교실)
  • Lee, Sun-Joo (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Ji-Hun (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Kim, Hyun-Hee (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Kim, So-Young (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Hahn, Seung-Hoon (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Hwang, Ja-Young (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Wonbae (Department of Pediatrics, College of Medicine, The Catholic University of Korea)
  • 투고 : 2005.12.07
  • 심사 : 2006.02.24
  • 발행 : 2006.07.15

초록

편측성 다낭성 신 이형성증과 폐의 선천성 낭성 선종양 기형은 각각 독립된 질환으로 배아 시기에 발생 과정의 문제로 생긴다고 알려져 있다. 폐의 선천성 낭성 선종양 기형에서 폐 외의 기형으로 신장 기형이 동반될 수 있으나 생존 가능성이 적어 사산아로 출생되는 경우가 많다. 저자들은 위장관염으로 입원한 21개월 남아에서 우연히 편측성 다낭성 신 이형성증을 발견했고, 이 과정에서 폐의 선천성 낭성 선종양 기형도 발견하였기에 보고하는 바이다.

Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

키워드

참고문헌

  1. Shukla AR, Kiddoo DA, Canning DA. Unilateral nephrectomy as palliative therapy in an infant with autosomal recessive polycystic kidney disease. J Urol 2004;172:2000-1 https://doi.org/10.1097/01.ju.0000142025.80132.2f
  2. Glassberg KI, Stephens FD, Lebowitz RL, Braren V, Duckett JW, Jacobs EC, et al. Renal dysgenesis and cystic disease of the kidney : a report of the Committee on Terminology, Nomenclature and Classification, Section on Urology, American Academy of Pediatrics. J Urol 1987;138: 1085-92 https://doi.org/10.1016/S0022-5347(17)43510-5
  3. Kuwertz-Broeking E, Brinkmann OA, Von Lengerke HJ, Sciuk J, Fruend S, Bulla M, et al. Unilateral multicystic dysplastic kidney : experience in children. BJU Int 2004;93: 388-92 https://doi.org/10.1111/j.1464-410X.2003.04623.x
  4. al-Khaldi N, Watson AR, Zuccollo J, Twining P, Rose DH. Outcome of antenatally detected cystic dysplastic kidney disease. Arch Dis Child 1994;70:520-2 https://doi.org/10.1136/adc.70.6.520
  5. Wang RY, Earl DL, Ruder RO, Graham JM Jr. Syndromic ear anomalies and renal ultrasounds. Pediatrics 2001;108: E32 https://doi.org/10.1542/peds.108.2.e32
  6. Ch'in KY, Tang MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol Lab Med 1949;48:221-9
  7. Fowler CL, Johnson L, Shukla P, Halverson K. Congenital cystic adenomatoid malformation of the lung. S D J Med 1987;40:5-8
  8. Nagata M, Shibata S, Shu Y. Pathogenesis of dysplastic kidney associated with urinary tract obstruction in utero. Nephrol Dial Transplant 2002;17:37-8
  9. Furuhashi M, Kawai H, Chaya J, Ishida T. Normal nephrogenesis occurs in the early stage of bilateral multicystic dysplastic kidneys. Arch Gynecol Obstet 2002 ;266:133-5 https://doi.org/10.1007/s004040100188
  10. Winyard P, Chitty L. Dysplastic and polycystic kidneys : diagnosis, associations and management. Prenat Diagn 2001;21:924-35 https://doi.org/10.1002/pd.208
  11. Ranke A, Schmitt M, Didier F, Droulle P. Antenatal diagnosis of multicystic renal dysplasia. Eur J Pediatr Surg 2001;11:246-54 https://doi.org/10.1055/s-2001-17147
  12. Mathiot A, Liard A, Eurin D, Dacher JN. Prenatally detected multicystic renal dysplasia and associated anomalies of the genito-urinary tract. J Radiol 2002;83:731-5
  13. Atiyeh B, Husmann D, Baum M. Contralateral renal abnormalities in multicystic-dysplastic kidney disease. J Pediatr 1992;121:65-7 https://doi.org/10.1016/S0022-3476(05)82543-0
  14. Shin YL, Won HS, Yoon JH, Park YS. Natural history and clinical feature of multicystic dysplastic kidney detected with prenatal ultrasonography. J Korean Pediatr Soc 2000; 43:926-32
  15. Wacksman J, Phipps L. Report of the multicystic kidney registry : preliminary findings. J Urol 1993;150:1870-2 https://doi.org/10.1016/S0022-5347(17)35918-9
  16. Belk RA, Thomas DF, Mueller RF, Godbole P, Markham AF, Weston MJ. A family study and the natural history of prenatally detected unilateral multicystic dysplastic kidney. J Urol 2002;167:666-9 https://doi.org/10.1016/S0022-5347(01)69120-1
  17. Dicker D, Samuel N, Feldberg D, Goldman JA. The antenatal diagnosis of Potter syndrome (Potter sequence). A lethal and not-so-rare malformation. Eur J Obstet Gynecol Reprod Biol 1984;18:17-24 https://doi.org/10.1016/0028-2243(84)90028-5
  18. Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation : a Canadian experience. Fetal Diagn Ther 2001;16:178-86 https://doi.org/10.1159/000053905
  19. Hugger K, Maupin KD. Late presentation of congenital cystic adenomatoid malformation of lung in ten-year-old girl. Pediatr Pulmonol 2004;37:276-8 https://doi.org/10.1002/ppul.10444