J Korean Soc Pediatr Nephrol > Volume 1(2); 1997 > Article
J Korean Soc Pediatr Nephrol 1997;1(2): 189-194.
신장이식 수술로 치험한 Oligomeganephronia 3례
배현철, 김지홍, 김병길, 김유선, 박기일, 정현주, 최인준
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 소아과학교실
3연세대학교 의과대학 소아과학교실
4연세대학교 의과대학 외과학교실
5연세대학교 의과대학 외과학교실
6연세대학교 의과대학 병리학교실
7연세대학교 의과대학 병리학교실
Successful Transplantation of 3 Cases of Oligomeganephronia
Hyun-Chul Bae, Ji-Hong Kim, Pyung-Kil Kim, Yoo-Seon Kim, Ki-Il Park, Hyun-Ju Jeong, In-Jun Choi
1Departments of Pediatrics, Yonsei University, College of Medicine
2Departments of Pediatrics, Yonsei University, College of Medicine
3Departments of Pediatrics, Yonsei University, College of Medicine
4Departments of Surgery, Yonsei University, College of Medicine
5Departments of Surgery, Yonsei University, College of Medicine
6Departments of Pathology, Yonsei University, College of Medicine
7Departments of Pathology, Yonsei University, College of Medicine
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ABSTRACT
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.
Key words: Oligomeganephronia | renal transplantation | end-stage renal failure
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