de Parscau L, Guibaud P
Unité d'Etude des maladies métaboliques, hôpital Debrousse, Lyon, France.
Pediatrie. 1990;45(3):165-71.
When the etiology of an hypoglycemia is not easily recognizable, the diagnosis can be guided by 2 signs: hepatomegaly and ketosis. If an hepatomegaly is present, an abnormality of glycogen metabolism or neoglucogenesis or an hereditary fructose intolerance may be suspected. A hypoketotic hypoglycemia suggests a hyperinsulinism or an impaired fatty acid oxydation. If the liver is normal and ketosis is normal or increased, an abnormality of branched amino acid metabolism or a ketotic hypoglycemia may be evoked.
当低血糖的病因不易识别时,诊断可依据两个体征:肝肿大和酮症。如果存在肝肿大,则可能怀疑糖原代谢、糖异生异常或遗传性果糖不耐受。低酮性低血糖提示高胰岛素血症或脂肪酸氧化受损。如果肝脏正常且酮症正常或增加,则可能引发支链氨基酸代谢异常或酮症性低血糖。