Battle A M, Stella A M, De Kaminsky A R, Kaminsky C, Mariano H G
Br J Dermatol. 1987 Mar;116(3):407-15. doi: 10.1111/j.1365-2133.1987.tb05856.x.
In a 7-year-old girl and a 12-year-old boy, with photosensitivity and hypertrichosis, the diagnosis of familial porphyria cutanea tarda was confirmed by the characteristic pattern of urinary porphyrin excretion, diminished erythrocyte uroporphyrinogen decarboxylase and elevated plasma porphyrin index with emission maxima at 617-618 nm. The patients were treated with a combination of low-dose oral chloroquine and oral S-adenosyl-L-methionine (SAM); in one case alkalinization of urine was also applied. Complete clinical and biochemical recovery was achieved within 3 months. No adverse ophthalmological or other side-effects were observed. We propose that the treatment of choice should be oral SAM (12 mg/kg/day) for 3 weeks and oral chloroquine (2 X 100 mg weekly) for about 120-150 days or until improvement of clinical and biochemical abnormalities is attained. So far no relapses have occurred. This combined therapy appears to be safe, simple, effective and very convenient for both patients and physicians.
在一名7岁女孩和一名12岁男孩身上，出现了光敏性和多毛症，通过尿卟啉排泄的特征模式、红细胞尿卟啉原脱羧酶减少以及血浆卟啉指数升高（发射最大值在617 - 618纳米），确诊为家族性迟发性皮肤卟啉症。患者接受了低剂量口服氯喹和口服S - 腺苷 - L - 蛋氨酸（SAM）的联合治疗；在一个病例中还进行了尿液碱化处理。3个月内实现了完全的临床和生化恢复。未观察到不良眼科或其他副作用。我们建议，首选治疗方案应为口服SAM（12毫克/千克/天）3周，口服氯喹（每周2次，每次100毫克）约120 - 150天，或直至临床和生化异常得到改善。到目前为止尚未发生复发。这种联合治疗对患者和医生而言似乎都是安全、简单、有效且非常方便的。
