Pharmacy Department, Hospital Universitario Infanta Leonor, Av. Gran Vía del Este, 80, Madrid, 28031, Spain.
Pharmacy Department, Hospital General Universitario Santa Lucía, Cartagena, Spain.
Orphanet J Rare Dis. 2024 Jan 5;19(1):12. doi: 10.1186/s13023-023-03011-x.
Hereditary fructose intolerance (HFI) is a rare metabolic disease caused by aldolase B deficiency. The aim of our study was to analyse excipient tolerability in patients with HFI and other related diseases and to design mobile and website health applications to facilitate the search for drugs according to their tolerance.
A total of 555 excipients listed in the Spanish Medicines Agency database (July 2023) were classified as suitable for HFI patients, suitable with considerations ((glucose and glucose syrup, intravenous sucrose, oral mannitol, polydextrose, gums and carrageenans, ethanol, sulfite caramel and vanilla), not recommended (intravenous mannitol) and contraindicated (fructose, oral sucrose, invert sugar, sorbitol, maltitol, lactitol, isomaltitol, fruit syrups, honey, sucrose esters and sorbitol esters). Glucose and glucose syrup were classified as suitable with considerations due to its possible fructose content and their potential endogenous fructose production. For other related intolerances, wheat starch was contraindicated and oatmeal was not recommended in celiac disease; oral lactose and lactose-based coprocessed excipient (Cellactose®) were not recommended in lactose intolerance; and glucose, invert sugar and oral sucrose were not recommended in diabetes mellitus. The applications were named IntoMed®. Results are listed in order of tolerability (suitable drugs appear first and contraindicated drugs at the end), and they are accompanied by a note detailing their classified excipients. If a drug contains excipients within different categories, the overall classification will be the most restrictive. The apps are also able to classify substances with the same criteria if they act as active ingredients. The tools exhibited good usability (82.07 ± 13.46 points on the System Usability Scale [range: 0-100]) on a sample of HFI patients, their families and health care professionals.
IntoMed® is a tool for finding information about the tolerability of drugs according to excipients for patients with HFI and other related intolerances, with good usability. It is a fast and reliable system that covers the current excipient legislation and expands on it with other specific information: HFI patients should be alert for excipients such as mannitol (especially in intravenous drugs), fruit syrups, honey, sulfite caramel or vanilla. Glucose might contain or produce fructose, and special precaution is needed because of potential errors in their composition.
遗传性果糖不耐受(HFI)是一种由醛缩酶 B 缺乏引起的罕见代谢疾病。我们的研究目的是分析 HFI 患者和其他相关疾病患者对赋形剂的耐受性,并设计移动和网站健康应用程序,根据其耐受性方便地搜索药物。
在西班牙药品管理局数据库(2023 年 7 月)中列出的 555 种赋形剂被归类为适合 HFI 患者使用(适合并有考虑因素(葡萄糖和葡萄糖浆、静脉内蔗糖、口服山梨醇、聚葡萄糖、胶和卡拉胶、乙醇、亚硫酸盐焦糖和香草)、不推荐(静脉内甘露醇)和禁忌(果糖、口服蔗糖、转化糖、山梨糖醇、麦芽糖醇、乳糖醇、异麦芽糖醇、水果糖浆、蜂蜜、蔗糖酯和山梨醇酯)。葡萄糖和葡萄糖浆因可能含有果糖及其潜在的内源性果糖产生而被归类为适合并有考虑因素。对于其他相关不耐受,小麦淀粉在乳糜泻中被禁忌,燕麦片不推荐;在乳糖不耐受中,口服乳糖和基于乳糖共加工的赋形剂(Cellactose®)不推荐;在糖尿病中,葡萄糖、转化糖和口服蔗糖不推荐。这些应用程序被命名为 IntoMed®。结果按耐受性顺序列出(适合的药物先出现,禁忌的药物最后出现),并附有详细说明其分类赋形剂的说明。如果一种药物含有不同类别的赋形剂,则整体分类将是最严格的。如果这些物质作为活性成分,应用程序也能够用相同的标准对其进行分类。这些工具在 HFI 患者、他们的家人和医疗保健专业人员的样本中表现出良好的可用性(系统可用性量表 [范围:0-100] 上的 82.07±13.46 分)。
IntoMed® 是一种根据 HFI 和其他相关不耐受患者的赋形剂寻找药物耐受性信息的工具,具有良好的可用性。它是一个快速可靠的系统,涵盖了当前的赋形剂法规,并扩展了其他特定信息:HFI 患者应注意甘露醇(尤其是静脉内药物)、水果糖浆、蜂蜜、亚硫酸盐焦糖或香草等赋形剂。葡萄糖可能含有或产生果糖,因此由于其成分可能存在错误,需要特别小心。