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首页> 外文期刊>Pharmaceutisch weekblad. >Behandeling van biotinidasedeficientie vergt juiste aflevering van biotine
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Behandeling van biotinidasedeficientie vergt juiste aflevering van biotine

机译：生物素酶缺乏症的治疗需要生物素的正确输送

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摘要

Abstract Treatment of b/otldase deficiency requires correct dispensing of biotin preparations Since 1 January 2007 newborn screening in the Netherlands is expanded with 13 inborn errors of metabolism. One of these disorders, biotinidase deficiency, was diagnosed in an asymptomatic boy, third child of Afghan parents. Biotinidase deficiency is treated with oral biotin. For instruction of adequate administration of the medication, the child was admitted to the hospital for one day. During this stay he was treated with extemporaneously compounded biotin capsules. Biotin therapy was continued at home, but the child was readmitted after 2 months with symptoms of a biotinidase deficiency: hair loss and skin lesions. Evaluation revealed incorrect biotin supply. Symptoms disappeared after therapy with the extemporaneously compounded biotin capsules. It is essential that treatment of biotinidase deficiency, with exceptional extemporaneous compounding, is managed by both the doctor and the (hospital) pharmacistto be sure that the biotin is given in the correct form and in sufficient amounts.
机译：摘要治疗b / ot / nldase缺乏症需要正确分配生物素制剂自2007年1月1日起，荷兰发生了13种先天性代谢错误，扩大了新生儿筛查的范围。这些疾病之一是生物素酶缺乏症，是在阿富汗父母的第三个孩子的无症状男孩中被诊断出的。用口服生物素治疗生物素酶缺乏症。为了指示适当的药物治疗，孩子被送进医院一天。在此期间，他接受了临时复合生物素胶囊的治疗。生物素治疗在家里继续进行，但是孩子在两个月后出现生物素酶缺乏症的症状：脱发和皮肤损伤。评估显示生物素供应不正确。用临时复配的生物素胶囊治疗后症状消失。必须由医生和（医院）药剂师共同管理生物素酶缺乏症，并给予额外的即席配合，以确保生物素以正确的形式和足够的量服用。

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