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KMID : 0367419780190120055
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Journal of Korean Pediatric Society
1978 Volume.19 No. 12 p.55 ~ p.62
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2 Cases of von Gierhe´s Diseases in Brother and Sister
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김영전/Kim, Yeong Jeon
전봉정/신명희/이종영/Jeon, Bong Jeong/Shin, Myung Hi/Lee, Jong Young
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Abstract
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We experienced 2 suspected cases of von Gierke´s diseases in 7 years and 8 months old girl and 6 years and 5 months old boy who are brother and sister.
They revealed abdominal distentions, huge livers, stunted growths and large amounts of glycogen storage in their livers on liver biopsies.
Oral glucose tolerance tests and epinephrine tests revealed very low fasting blood sugar levels (15mg% in sister and 10mg% in brother) and their delayed returns to the fasting levels.
Enzyme study.is unable to do in our laboratory at present time.
A brief review of related literatures is also presented.
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