All issues > Volume 19(12); 1976

Case Report

J Korean Pediatr Soc. 1976;19(12):947-954. Published online December 31, 1976.

2 Cases of von Gierke’s Diseases in Brother and Sister

Yeong Jeon Kim¹, Bong Jeong Jeon¹, Myung Hi Shin¹, Jong Young Lee¹

¹Department of Pediatrics,Wallace Memorial Baptist Hospital Busan, Korea

Abstract

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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