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The Sickle Cell Disease Multidisciplinary Clinic at the Lifespan Cancer Institute

机译:镰状细胞疾病在寿命癌症学院的多学科诊所

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Sickle cell disease (SCD) is one of the world’s most commonmonogenic disorders, affecting about 100,000 peoplein the United States. The illness is manifested by sudden,unpredictable and severe bouts of pain, termed vaso-occlusiveepisodes (VOEs). In addition to the classic presentationof severe VOEs, SCD leads to end-organ damage inmultiple systems (Table 1). This damage is cumulative, andultimately leads to a decreased average lifespan and qualityof life.1 As is the case for many genetic diseases, pediatricianshave been at the forefront of research and treatmentfor SCD. Nevertheless, as patients with SCD mature intoadulthood, they face the unique problems of adults, alongwith the accumulating burden of symptoms and end-organdamage from hemoglobinopathy, and care in adult medicinepractices becomes appropriate. Current guidelines for themanagement of SCD call for the care of patients with SCDto be coordinated throughout the lifespan and across caresettings as in the patient-centered medical home model.
机译:镰状细胞疾病(SCD)是世界上最常见的疾病之一,影响美国约10万人。这种疾病是突然,不可预测和严重的疼痛,称为血管闭塞(voes)。除了严重竞争对手的经典展示之外,SCD还导致终端器官损坏(表1)。这种伤害是累积的,恒生的平均寿命和素质的含量下降。如许多遗传疾病的情况如此,儿科医师都处于研究和待遇的最前沿。然而,随着SCD成熟的患者,他们面临成人的独特问题,沿着血管病病变的症状和末端的负担,以及成年医药的护理变得适当。目前SCD呼吁对SCDTO患者的患者的临时指南在整个寿命中协调,并在患者中心医疗家庭模型中进行配对。

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