Purkiss P, Watts R W
Division of Inherited Metabolic Diseases, Research Centre, Harrow.
Proc R Soc Med. 1977;70 Suppl 3(Suppl 3):27-30. doi: 10.1177/00359157770700S311.
(1) A single dose of D-penicillamine (not more than 750 mg) taken at 2200 h, together with a prescribed fluid intake of two to three litres during the waking hours, without extra drinks at night, is sufficient to keep the concentration of the urinary cystine below the saturating concentration of urine throughout the 24 h period. (2) This regime does not materially reduce the total 24 h excretion of cystine. (3) The effect of D-penicillamine is mainly seen in the urine excreted between 0200 and 0800 h. (4) This regime is provisionally recommended for the prevention of cystine stone recurrence but not for stone dissolution. Larger total amounts of D-penicillamine given in divided doses are still recommended for the latter purpose.
(1) 单次服用D-青霉胺（不超过750毫克），于22:00时服用，同时在清醒时间规定摄入两到三升液体，夜间不额外饮水，足以使24小时内尿胱氨酸浓度保持在尿液饱和浓度以下。(2) 该方案不会显著降低胱氨酸的24小时总排泄量。(3) D-青霉胺的作用主要在02:00至08:00之间排出的尿液中显现。(4) 该方案暂推荐用于预防胱氨酸结石复发，但不用于结石溶解。为达到后者目的，仍推荐分剂量给予更大总量的D-青霉胺。
