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首页> 外文OA文献 >Subcutaneous vitamin B12 administration using a portable infusion pump in cobalamin-related remethylation disorders: a gentle and easy to use alternative to intramuscular injections
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Subcutaneous vitamin B12 administration using a portable infusion pump in cobalamin-related remethylation disorders: a gentle and easy to use alternative to intramuscular injections

机译：使用便携式输注泵在钴胺素相关的除归障碍中使用便携式输注泵：一种温和易用的肌肉注射替代品

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Abstract Background Cobalamin (cbl)-related remethylation disorders are a heterogeneous group of inherited disorders comprising the remethylation of homocysteine to methionine and affecting multiple organ systems, most prominently the nervous system and the bone marrow. To date, the parenteral, generally intramuscular, lifelong administration of hydroxycobalamin (OHCbl) is the mainstay of therapy in these disorders. The dosage and frequency of OHCbl is titrated in each patient to the minimum effective dose in order to account for the painful injections. This may result in undertreatment, a possible risk factor for disease progression and disease-related complications. Results We describe parenteral administration of OHCbl using a subcutaneous catheter together with a portable infusion pump in a home therapy setting in four pediatric patients with remethylation disorders, two patients with cblC, one patient with cblG, and one patient with cblE deficiency, in whom intramuscular injections were not or no longer feasible. The placement of the subcutaneous catheters and handling of the infusion pump were readily accomplished and well accepted by the patients and their families. No adverse events occurred. The use of a small, portable syringe driver pump allowed for a most flexible administration of OHCbl in everyday life. The concentrations of total homocysteine levels were determined at regular patient visits and remained within the therapeutic target range. This approach allowed for the continuation of OHCbl therapy or the adjustment of therapy required to improve metabolic control in our patients. Conclusions Subcutaneous infusion using a subcutaneous catheter system and a portable pump for OHCbl administration in combined and isolated remethylation disorders is safe, acceptable, and effective. It decreases disease burden in preventing frequent single injections and providing patient independence. Thus, it may promote long-term adherence to therapy in patients and parents.
机译：摘要背景钴胺素（CBL） - 相关的雷甲基化疾病是一种异质的遗传性疾病，其包含同型半胱氨酸对蛋氨酸的甲基化并影响多种器官系统，最突出的神经系统和骨髓。迄今为止，肠胃外，通常肌肉内终身羟基胺（OHCBL）是这些疾病中的治疗方法。 OHCBL的剂量和频率在每只患者中滴定到最小有效剂量以考虑疼痛的注射。这可能导致疾病进展和疾病相关并发症的可能危险因素。结果我们使用皮下导管描述OHCBL的肠胃外给药，用尿液患者的四个小儿患者的家庭治疗环境中的一种便携式输注泵，两名CBLC，一名患有CBLG的患者，一名患有闭合的患者，肌肉内注射不是或不再可行的。皮下导管的放置和输液泵的处理易于完成，并由患者及其家人接受良好。没有发生不良事件。使用小型便携式注射器泵，允许在日常生活中获得最灵活的OHCBL管理。在常规患者访问时测定总同型半胱氨酸水平的浓度，并保持在治疗靶标范围内。这种方法允许延续OHCBL治疗或调整治疗，以改善患者的代谢控制。结论使用皮下导管系统和用于OHCBL给药的便携式泵，组合和分离的雷甲基化疾病是安全的，可接受的，有效的。它降低了防止频繁的单一注射和提供患者独立性的疾病负担。因此，它可能促进患者和父母治疗的长期依从性。

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