The origin of halitosis in cystinotic patients due to eysteamine treatment
The origin of halitosis in cystinotic patients due to eysteamine treatment
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DOI：
10.1016/j.ymgme.2007.04.002
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发表时间：
2007-07-01
影响因子：
3.8
通讯作者：
Levtchenko, Elena
中科院分区：
文献类型：
--
作者：
Besouw, Martine;Blom, Henk;Levtchenko, Elena
介绍：胱氨酸病是一种罕见的常染色体隐性遗传疾病，其特征是胱氨酸在溶酶体内蓄积。半胱胺可从溶酶体中去除胱氨酸并减缓疾病的进展。其副作用之一是诱导口臭，这可能会干扰患者遵守半胱胺治疗的意愿。目的：确定半胱胺治疗引起胱氨酸病患者口臭的呼吸硫化合物。研究设计：在摄入15 mg/kg半胱胺全血后（n = 4），通过气相色谱法在360分钟内每60分钟测量一次尿（n = 4）和呼吸（n = 8）的挥发性硫化合物水平，并通过高-结果：胱氨酸病患者呼出气中甲硫氨酸（MT，中位最大值0.5（0-11）nmol/L）和二甲基硫（DMS，中位最大值15（2-83）nmol/L）浓度升高。已知DMS浓度高于0.65 nmol/L会导致口臭。半胱胺的最大血浆值（中值46（范围30-52）μ mol/L）先于NIT和DMS的最大血浆值，证实半胱胺转化为NIT和DMS。不到3%的半胱胺摄入量被排出的NIT和DMS通过呼气和0.002%通过urina.Conclusion：口臭引起的半胱胺摄入引起的DMS和NIT，在较小程度上，通过呼气排出。进一步的研究应该集中在减少这些挥发性硫化合物的形成或掩盖其气味的可能性上，这将提高半胱胺治疗的依从率。(c)2007年爱思唯尔公司All rights reserved.
Introduction: Cystinosis is a rare autosomal recessive disorder characterized by the intralysosomal accumulation of cystine. Cysteamine removes cystine from the lysosome and slows down the progression of the disease. One of its side effects is the induction of halitosis, which can interfere with patients' willingness to comply with cysteamine treatment.Objective: To identify breath sulphur compounds causing halitosis induced by cysteamine therapy in patients with cystinosis.Study design: After the ingestion of 15 mg/kg cysteamine whole blood (n = 4), urine (n = 4) and breath (n = 8) volatile sulphur compounds levels were measured every 60 min over a 360 min period by gas chromatography and the cysteamine plasma concentrations (n = 4) were measured by high-performance liquid chromatography.Results: The expired air of cystinotic patients contained elevated concentrations of methanethiol (MT, median maximum value 0.5 (range 0-11) nmol/L) and, in particular, dimethylsulphide (DMS, median maximum value 15 (range 2-83) nmol/L). DMS concentrations higher than 0.65 nmol/L are known to cause halitosis. Maximal plasma values of cysteamine (median 46 (range 30-52) mu mol/L) preceded those of NIT and DMS, confirming that cysteamine is converted to NIT and DMS. Less than 3% of the amount of cysteamine ingested was excreted as NIT and DMS via expired air and 0.002% via urine.Conclusion: Halitosis induced by cysteamine intake is caused by DMS and to a lesser extent by NIT, excreted via the expired air. Further studies should focus on the possibilities of reducing the formation of these volatile sulphur compounds or masking their odour, which would improve the rates of compliance with cysteamine treatment. (c) 2007 Elsevier Inc. All rights reserved.