| Acute Werdnig-Hoffmann Diseases in Siblings. |
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Y D Lee, Y W Lee, J H Lee, Y H Chung |
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Department of Pediatrics, Chungnam National University, College of Medicine |
| 형제에서 발생된 급성 Werdnig-Hoffmaim씨 병 |
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이영두, 이양원, 이재호, 정용헌 |
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충남대학교 의과대학 소아과학교실 |
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| Abstract |
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Acute Werdnig-Hoffmann diseases being the most common cause of floppy infant, in two male siblings were presented. The patients appeared characteristic clinical findings suggestive of spinal atrophy at 4
weeks and 2 months and all died about 8 months old. Previous expired another elder-brother was also related to this disease. Diagnosis was estabilished by clinical & pathologic findings. This is the autosomal dominant trait case of acute Werdnig-Hoffmann disease.
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| Key Words:
Werdnig Hoffmann disease, Muscle biopsy |
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