Department of Hematology/Oncology, Children's Hospital and Research Center at Oakland, Oakland, California, USA.
Ann N Y Acad Sci. 2010 Aug;1202:188-96. doi: 10.1111/j.1749-6632.2010.05578.x.
Optimal nutritional status is imperative for growth, development, immune function, and bone health. Patients with thalassemia are known to have poor growth, altered puberty, and immune function as well as reduced bone mineral acquisition. The etiology of these comorbidites is typically ascribed to the toxic effects of transfusion-related iron-overload. Recently, our group and others have observed marked nutritional deficiencies in key fat and water-soluble vitamins as well as important essential minerals. Depressed circulating levels of nutrients have been observed despite seemingly adequate dietary intake. This disconnect between intake and circulating levels suggests that patients with thalassemia may have increased needs for certain nutrients due to either poor nutrient absorption, elevated losses, or increased nutrient turnover. Randomized controlled clinical trials are needed to test the efficacy of nutritional therapies toward improving the overall health in thalassemia, as well as decreasing long-term comorbidities such as reduced bone mass.
最佳营养状况对生长、发育、免疫功能和骨骼健康至关重要。已知地中海贫血患者生长不良、青春期改变、免疫功能下降以及骨矿物质获取减少。这些合并症的病因通常归因于输血相关铁过载的毒性作用。最近,我们小组和其他小组观察到关键脂溶性和水溶性维生素以及重要必需矿物质的明显营养缺乏。尽管膳食摄入似乎充足,但仍观察到循环营养素水平降低。这种摄入与循环水平之间的脱节表明,地中海贫血患者可能由于营养素吸收不良、丢失增加或营养素周转率增加而对某些营养素的需求增加。需要进行随机对照临床试验来测试营养疗法对改善地中海贫血整体健康以及减少长期并发症(如骨量减少)的疗效。