ESPE Abstracts

Introduction: Patients with thalassemia have a high prevalence of malnutrition and low body mass index (BMI), and nutritional intervention studies are scarce.

Aim: We performed an electronic search in PubMed, Google Scholar, and Web of Sciences to review nutritional intervention studies and their possible beneficial effect on children and adults with BTM.

Results: 17 controlled and/or longitudinal studies fitted the search criteria. Two NI studies investigated the effect/s of increasing mean energy intake by 20 and 30-50%, respectively, in 12 and 15 children with BTM for 4 and 8 weeks, respectively. Authors reported significant increases in body weight, BMI, fat-free mass, fat mass, and accelerated height velocity associated with increased IGF-1. On the other hand, NI using micronutrients discovered variable clinical and biochemical effects. One controlled study proved that zinc (Zn) supplementation for 18 months increased serum Zn and bone mineral content of patients with BTM (n= 40) versus controls. Longer term-controlled trials using Zn supplementation (n= 32 patients, aged 1-7 years) increased linear growth compared to thalassemic children without Zn supplementation. A study on 64 patients found that Zn supplements reduced anti-heat shock protein (anti-HSP27) titers in patients with BTM suggesting potential antioxidant and anti-inflammatory effects. Another placebo-controlled study (n= 120) showed that supplementations of Zn and vitamin E increased BMI and total antioxidant capacity. In 3 nutritional intervention (NI) studies, vitamin E supplementation for 1- 9 months improved the antioxidant/ oxidant balance in plasma and red blood cells, counteracted lipid peroxidation processes, and increased red blood cells membrane fluidity. Daily vitamin C supplementation for a year potentiated the efficacy of DFO to reduce iron overload. (95) In 3 studies, vitamin D supplementation (intermittent mega dose, oral or IM, or daily oral dose) was associated with increased serum 25-OHD level and significant improvement of symptoms related to vitamin D deficiency in adolescents with β-TM. In one study oral vitamin D and calcium supplementation for 1-year increased bone mineral content. Oral supplementation of L-carnitine for 6 months improved pubertal development, cardiac performance, and physical fitness and in BTM adolescents. One study suggested a positive role of folic acid in preventing the progression of arteriosclerosis and decreasing thromboembolic events.

Conclusion: Besides increasing Hb level and effective chelation therapy, nutritional support and follow-up of nutritional status including weight gain, BMI, height velocity and bone health appear very important to maintain normal growth