• Childhood Kidney Diseases
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• 제6권1호
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• pp.92-96
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• 2002

Oligomeganephronia는 신장 저형성에서 신원의 수가 적어 비대해진 드문 선천성 신기형이다. 1962년 Royer등이 양측성 신장 저형성 환아에서 oligomeganephronia를 처음 기술한 이래 수십 례가 보고되어 있으나 단일신에 발생한 경우는 매우 드물어 1971년 Van Acker등에 의한 첫 보고 이래로 8례가 보고되었고 국내에서는 양측성 신장 저형성에서 진단된 3례가 보고되어 있다. 저자들은 학교 집단 뇨검사에서 단독 단백뇨가 발견된 13세 남아에서 단일신의 oligomeganephronia를 진단하였기에 국내 첫 증례의 보고하는 바이다.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.223-228
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• 2003

Oligomeganephronia는 선천적인 신형성 부전으로 신원의 수가 감소되고 보상성으로 사구체가 비대해진 질환으로 지금까지 전 세계적으로 약 60례만이 보고될 정도로 매우 드문 질환이다. 저자들은 학교선별뇨검사상 현미경적 혈뇨를 보였던 환아에서 신생검을 통하여 oligomeganephronia를 조기에 진단하고, 4년간 신부전증 및 성장부전의 진행 소견과 함께 사구체 경화증의 발생으로 사구체 용적이 감소하는 소견을 관찰할 수 있었기에, 이를 문헌 고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.189-194
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• 1997

Oligomeganephronia is a rare congenital form of bilateral renal hypoplasia histologically characterized by reduction in number and hypertrophy of nephrons. Clinically, this condition is presented in early infancy with vomiting, polyuria, polydipsia and dehydration. The problems are readily corrected, but slowly progressive renal failure follows accompanied by failure to thrive, short stature, and renal osteodystrophy. We experienced three cases of oligomeganephronia. Case 1. : A 3 2/12 years old female child was incidentally diagnosed as renal failure at age of 2 months when she was hospitalized due to pneumonia. She had open renal biopsy and was diagnosed as bilateral dysplastic kidney. On OPD follow-up, she progressed to end-stage renal failure (BUN/Cr 114/4.6 mg/dl) and had renal transplantation. The specimen was shrunk remarkably and light microscopy showed oligomeganephronia. Case 2. : A 14 8/12 years old female child with proteinuria was detected in an annual urine screening program for school children, she was diagnosed as renal failure (BUN/Cr 33.9/4.1 mg/dl), and had $5{\times}4{\times}3\;cm$ sized mass on abdominal CT scan. She had renal biopsy, and the specimen showed oligomeganephronia. She had hemodialysis for six months, and renal transplantation along with bilateral nephrectomy was performed. Case 3. : A 14 8/12 years old male child was diagnosed having chronic nephritis and chronic renal failure at 3 years old, progressed to end-stage renal failure (BUN/Cr 87/9.6 mg/dl) on OPD follow-up, and had a rephrectomy and renal transplantation. The biopsy specimen showed oligomeganephronic hypoplasia, secondary focal segmental glomerolosclerosis, and chronic interstitial nephritis. We report 3 cases of oligomeganephronia that progressed to end-stage renal failure and had successful renal transplantation with a brief review of related literatures.