Hackl J M, Balogh D, Kunz F, Dworzak E, Puschendorf B, Decristoforo A, Maier F
Wien Klin Wochenschr. 1978 Mar 31;90(7):237-40.
Hereditary fructose intolerance (HFI) was diagnosed in a 61 year-old male patient on account of liver dysfunction followed by prolonged shock immediately after the administration of a fructose and lactose infusion postoperatively. The diagnosis of HFI was based on an increased value of fructose, hypoglycaemia, lactic acidosis and diminution of the phosphate level in combination with the typical family history. The patient's children showed a normal reaction to fructose administration. The therapy included glucose, insulin and heparin administration, balance of acidosis and partial exchange of blood, which resulted in improvement in the glucose level, coagulation factors and acidosis, but could not prevent further liver damage and uraemia with a fatal outcome.
一名61岁男性患者因肝功能障碍被诊断为遗传性果糖不耐受（HFI），术后输注果糖和乳糖后随即出现长时间休克。HFI的诊断基于果糖值升高、低血糖、乳酸酸中毒、磷酸盐水平降低以及典型的家族病史。患者的子女对果糖给药反应正常。治疗措施包括给予葡萄糖、胰岛素和肝素、纠正酸中毒以及部分血液置换，这使得血糖水平、凝血因子和酸中毒有所改善，但未能防止进一步的肝损伤和尿毒症，最终导致患者死亡。
