| A case of Asphyxiating Thoracic Dystrophy. |
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Hyung Goo Cho1, Hwang Min Kim1, Jae Seung Yang1, Jong Soo Kim1, Whi Yul Cho2, So Young Jin3 |
1Department of Pediatrics, Yonsei University, Wonju College of Medicine, Wonju, Korea
2Department of Radiology, Yonsei University, Wonju College of Medicine, Wonju, Korea
3Department, of Pathology, Yonsei University, Wonju College of Medicine, Wonju, Korea |
| Asphyxiating Thoracic Dystrophy 1례 |
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조형구1, 김황민1, 양재승1, 김종수1, 조휘율2, 진소영3 |
1연세대학교 원주의과대학 소아과학 교실
2연세대학교 원주의과대학 방사선과학교실
3연세대학교 원주의과대학 병리학교실 |
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Received: 9 February 1990 • Accepted: 30 May 1990 |
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| Abstract |
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Asphyxiating thoracic dystrophy, a rare form of bony dysplasia, is characterized by extreme
constriction of thorax, short limbed dwarfism, abnormalities of pelvic bone in neonatal period, and
progressive interstitial nephritis in childhood. The incidence of this syndrome has been estimated at
1 per 100,000 to 130,000 live births and more than 100 patients have been reported worldwide.
Infants with asphyxiating thoracic dystrophy usually have tachypnea, cyanosis, and constricted th-
orax. Almost all of the survivors are suffered form progressive interstial nephritis in childhood.
We experienced a case of asphyxiating thoracic dystrophy in a 1 day old neonate who had
tachypnea and cyanosis. Inspite of assisted ventilation for 4 weeks, he died without improvement.
Postmortem examination disclosed hypoplasia and atelectasis in lung.
A brief review of the related literature is presented. |
| Key Words:
Asphyxiating thoracic dystrophy, Constricted thorax, Lung hypoplasia |
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