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A Case of Potter Syndrome Syndrome Accompanied with Partial Agenesis of Corpus Callosum.

Journal of the Korean Pediatric Society 1987;30(11):1287-1293.
Published online November 30, 1987.
A Case of Potter Syndrome Syndrome Accompanied with Partial Agenesis of Corpus Callosum.
Soon Sup Jang1, Byung Chun Suh1, Kyoo Hwan Rhee1, Soo Jee Moon1, Keun Soo Lee1, Byung Tae Park2, Eun Kyung Hong2
1Department of Pediatrics, College of Medicine, Hanyang University, Seoul, Korea
2Department of Pathology, College of Medicine, Hanyang University, Seoul, Korea
부분적 뇌량형성부전을 동반한 Potter 증후군 1례
장순섭1, 서병천1, 이규환1, 문수지1, 이근수1, 박병태2, 홍은경2
1한양대학교 의과대학 소아과학교실
2한양대학교 의과대학 조직병리학 교실
Abstract
We have experienced a case of Potter syndrome recently. He was a newborn infant who suffered from severe respiratory distress soon after birth. His face was characterized by facies renalies and chest X-ray film revealed bilateral pneumothorax. Anuria since birth and findings of abdominal ultrasonography strongly suggested bilateral renal agenesis. Severe respiratory acidosis was progressed in spite of proper management and died at 11 hours after birth. The diagnosis was confirmed by autopsy findings consisted of bilateral pulmonary hypoplasia, absence of both kidneys, ureters, and renal arteries, hypoplastic urinary bladder, patent ductus arteriosus, and partial agenesis of corpus callosum. We report this case with brief review of the related literactures.
Key Words: Potter syndrome, Corpus callosum agenesis


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