단일신을 가진 소아의 임상적 특징과 동반기형
Clinical Characteristics and Associated Anomalies in Children with Solitary Kidney
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
목 적:단일신을 가진 소아의 임상적 특징과 동반기형 및 장기적 신기능 등에 대해 알아보고자 하였다방 법:1989년 12월부터 2009년 12월까지 20년간 서울대학교 어린이병원에서 진단된 38예의 단일신 환자를 대상으로 후향적 조사를 하였으며, 단일신은 영상검사를 통해 진단된 일측성 신장 무형성으로 정의하였고, 일측성 이형성 신의 퇴행으로 발생한 단일신은 제외하였다. 결 과:진단 연령의 중앙값은 6.5개월(출생직후- 13세)이었고, 남자가 12명(31.6%), 여자가 26명(68.4%) 이었다. 19예(50.0%)에서는 좌측 신장이 없었고 19예에서는 우측 신장이 없었다. 산전 초음파에서 진단된 경우가 14예(36.8%), 신장 혹은 요로계 질환에 대한 검사 도중 13예(34.2%), 우연히 발견된 경우가 10예(26.3%)이었다. 동반 기형으로는 방광요관 역류 11예(28.9%)를 포함한 신장기형이 17예(44.7%), 기타 비뇨생식기계의 기형이 16예(42.1%) 그리고 다 기관 증후군 혹은 염색체 이상이 9예(23.7%)에서 진단되었다. 혈청 크레아티닌의 중간값은 0.6 mg/dL이었으며 만성 신부전이 2예(5.3%)에서 확인되었다. 결 론:단일신은 신장의 선천성 기형 중 상대적으로 흔한 질환이며, 일반적으로 증상이 없다. 그러나 일부에서는 단일신은 다른 기관의 이상과 연관이 되어있거나 관련된 증상이 발생한다. 단일신을 가진 소아의 연관 기형의 조기 발견과 추적 관찰이 장기적인 신 손상의 위험을 감소시키기 위해 필요하다.
Abstract
Purpose:The clinical characteristics and associated anomalies in children with solitary kidney (SK) were analyzed retrospectively. Methods:Total 38 children diagnosed to have SK at our hospital between December 1989 and December 2009 were recruited, and the clinical records including imaging studies were retrospectively reviewed. SK was defined as unilateral renal agenesis by imaging studies only, and patients with regression of unilateral dysplastic kidney were excluded. Results:Among total 38 patients, 12 were male. The median age at the diagnosis of SK was 6.5 months (at birth-13 years). SK was detected by prenatal ultrasonography in 14 patients and during work-up for renal or urinary tract diseases in 13 (including urinary tract infection in 7). In 10 patients, SK was detected incidentally. Anomalies in the SK were noted in 17 patients including vesicoureteral reflux in 11. Other anomalies in the genitourinary tract were present in 16 patients, and multi-organ-involving syndromes or chromosomal anomalies were detected in 9. The mean duration of follow-up was 9 years (9 months-20 years). Two patients developed chronic renal failure during follow-up, and the median serum creatinine concentration of the remaining 36 at their last follow-up was 0.6 mg/dL. Conclusion:SK may be isolated and clinically asymptomatic; it is frequently accompanied by other anomalies in genitourinary tract and other organs, some of which can induce progressive renal dysfunction. Early recognition of associated anomalies with SK and regular follow-up is recommended to reduce long-term risk.
참고문헌 (24)
- Wiesel A, "Prenatal detection of congenital renal malformations by fetal ultrasonographic examination: an analysis of 709,030 births in 12 European countries", Eur J Med Genet, 48, pp.131~144 (2005)
- Valentini RP, "MRI detection of atrophic kidney in a hypertensive child with a single kidney", Pediatr Nephrol, 20, pp.1192~1194 (2005)
- Rabelo EA, "Natural history of multicystic kidney conservatively managed: a prospective study", Pediatr Nephrol, 19, pp.1102~1107 (2004)
- Kiprov DD, "Focal and segmental glomerulosclerosis and porteinuria associated with unilateral renal agenesis", Lab Invest, 46, pp.275~281 (1982)
- Roodhooft AM, "Familial nature of congenital absence and severe dysgenesis of both kidneys", N Engl J Med, 310, pp.1341~1345 (1984)
- Parikh CR, "Congenital renal agenesis: case-control analysis of birth characteristics", Am J Kidney Dis, 39, pp.689~694 (2002)
- Robson WL, "Unilateral renal agenesis", Adv Pediatr, 42, pp.575~592 (1995)
- Rosenbaum DM, "Sonographic assessment of renal length in normal children", AJR Am J Roentgenol, 142, pp.467~469 (1984)
- Rickwood AM, "Multicystic renal dysplasia detected by prenatal ultrasonography. Natural history and results of conservative management", Br J Urol, 69, pp.538~540 (1992)
- Kaneyama K, "Associated urologic anomalies in children with solitary kidney", J Pediatr Surg, 39, pp.85~87 (2004)
- Wiesel A, "Prenatal detection of congenital renal malformations by fetal ultrasonographic examination: an analysis of 709,030 births in 12 European countries", Eur J Med Genet, 48, pp.131~144 (2005)
- 권재현, "태아의 비뇨기계 기형과 동반하는 주요 장기 기형에 대한 보고", Korean Journal of Obstetrics and Gynecology, 52(6), pp.611~616 (2009), 대한산부인과학회
- 임진아, "산전 초음파로 발견된 선천성 신 요로 기형의 발생률과 임상 결과: 단일 기관 연구", Korean Journal of Pediatrics, 52(4), pp.464~470 (2009), 대한소아과학회
- Cascio S, "Associated urological anomalies in children with unilateral renal agenesis", J Urol, 162, pp.1081~1083 (1999)
- Bauer SB, "Anomalies of the upper urinary tract in Campbell–Walsh urology. 9th ed.", pp.3272~3276 (2007), Elsevier
- Woolf AS, "Unilateral renal agenesis and the congenital solitary functioning kidney: developmental, genetic and clinical perspectives", BJU Int, 99, pp.17~21 (2007)
- Dursun H, "Associated anomalies in children with congenital solitary functioning kidney", Pediatr Surg Int, 21, pp.456~459 (2005)
- Krzemien G, "Urological Urological anomalies in children with renal agenesis or multicystic dysplastic kidney", J appl Genet, 47, pp.171~176 (2006)
- Wiersma AF, "Uterine anomalies associated with unilateral renal agenesis", Obstet Gynecol, 47, pp.654~657 (1976)
- Argueso LR, "Prognosis of patients with unilateral renal agenesis", Pediatr Nephrol, 6, pp.412~416 (1992)
- Gonzalez E, "Factors influencing the progression of renal damage in patients with unilateral renal agenesis and remnant kidney", Kidney Int, 68, pp.263~270 (2005)
- Sanna-Cherchi S, "Renal outcome in patients with congenital anomalies of the kidney and urinary tract", Kidney Int, 76, pp.528~533 (2009)
- de Lucas C, "Solitary kidney, Study of renal morphology and function in 95 children", Nefrologia, 26, pp.56~63 (2006)
- Schreuder MF, "Delemarre-Van de Waal HA, Van Wijk JA. Hypertension and microalbuminuria in children with congenital solitary kidneys", J Paediatr Child Health, 44, pp.363~368 (2008)