Nutrient Insufficiencies/Deficiencies in Children With Sickle Cell Disease and Its Association With Increased Disease Severity.

BACKGROUND: Sickle cell disease (SCD) is characteristically described as a disease of hemolytic anemia and vaso-occlusive crises (VOCs). However, patients suffer from a multitude of other problems including impaired development, chronic pain, and increased susceptibility to infection. Nutritional deficiency has been implicated as a contributor to these issues. PROCEDURE: We reported the nutrition status with respect to vitamin D, zinc, B6, B12, folate, and homocysteine serum levels in Canadian children with SCD (n = 91). We also tested for associations between nutrients and markers of disease severity and growth. RESULTS: Almost half the sample (42%) had multiple nutrient insufficiencies/deficiencies, and a further 27% had a single insufficiency/deficiency. The most common insufficiency/deficiency was zinc in 57% followed by calcidiol (25 dihydroxyvitamin D (25(OH)D)) (52%). Sixteen percent of patients had low vitamin B6 levels, while folate, calcitriol (1,25(OH)D), and homocysteine levels were normal. Increased number of vitamin insufficiencies/deficiencies was associated with increasing disease severity (P = 0.018). Zinc insufficiency/deficiency was significantly associated with an increased number of home pain crises (P = 0.001) and an increased incidence of hospitalizations for VOCs (P = 0.01). CONCLUSIONS: Our findings show that patients with SCD commonly have multiple nutrient insufficiencies/deficiencies and support the growing evidence for the link between low zinc and increased VOC. It also indicates that increased nutrient insufficiencies/deficiencies are associated with increased disease severity in SCD. Prospective studies with larger samples are needed to further elucidate the relationship between nutrient deficiencies and SCD, and to determine whether nutrient supplementation can improve the disease course.

'Designer oils' low in n-6:n-3 fatty acid ratio beneficially modifies cardiovascular risks in mice.

Cardiovascular benefits of dietary n-3 fatty acids have been shown. However, benefits of n-3 fatty acids as part of a high fat, low n-6:n-3 fatty acid ratio diet has not been fully characterized. Aim of this study is to investigate cardiovascular and metabolic benefits of 'designer oils' containing a low ratio of n-6:n-3 fatty acids in C57BL/6 mice. Three groups of C57BL/6 mice were fed an atherogenic diet supplemented with either a fish oil- or flaxseed oil-based 'designer oil' with an approximate n-6:n-3 fatty acid ratio of 2:1 (treated groups, n = 6 each) or with a safflower oil-based formulation with a high ratio (25:1) of n-6:n-3 fatty acids (control group, n = 6) for 6 weeks. Food intake, body weight, and blood lipid levels were monitored regularly. Fatty acid profile of the heart tissues was assessed. Histological assessment of liver samples was conducted. At the end of the study body weight and food intake was significantly higher in the flax group compared to control. The levels of 20:5n-3 and 22:6n-3 was significantly increased in the heart phospholipids in both flax and fish groups compared to control; tissue 20:4n-6 was significantly reduced in the fish group compared to control. Significant liver pathology was observed in the control group only. Lowering dietary ratio of n-6:n-3 fatty acids may significantly reduce cardiovascular and metabolic risks in mice regardless of the source of n-3 fatty acids.

Severe vitamin C deficiency in a child newly diagnosed with T-cell ALL due to nutrient gap.

A 10-year-old boy developed a perifollicular rash during interim maintenance of T-Cell acute lymphoblastic leukaemia. Differential diagnoses included drug reaction and inflammatory process. Before diagnosis, the patient had a limited diet--low in vegetables and fruits--due to selective eating, with later anorexia and taste aversions due to chemotherapy treatment. Despite nutritional counselling and starting a multivitamin, the patient incurred severe weight loss (18.5% of his usual body weight). Serum levels of ascorbic acid were non-detectable, at <5 µmol/L, indicative of vitamin C deficiency. The patient began vitamin C supplementation containing 125 mg ascorbic acid three times a day for 7 days, then 125 mg once daily for 3 months to normalise serum vitamin C. After ascorbic acid treatment was completed, the patient started a complete multivitamin and made efforts to eat fruits and vegetables rich in vitamin C. His serum ascorbic acid concentrations normalised to 52 µmol/L 3 months after receiving supplementation.

Dietary monounsaturated fatty acids are protective against metabolic syndrome and cardiovascular disease risk factors.

Over 50 years of research has sought to define the role dietary fat plays in cardiovascular disease (CVD) risk. Although optimal dietary fat quantity has been keenly pursued over past decades, attention has recently centered on the value of dietary fat quality. The purpose of the present review is to provide a critical assessment of the current body of evidence surrounding efficacy of dietary monounsaturated fatty acids (MUFA) for reduction of traditional risk factors defining metabolic syndrome (MetS) and CVD. Due to existing and emerging research on health attributes of MUFA rich diets, and to the low prevalence of chronic disease in populations consuming MUFA rich Mediterranean diets, national dietary guidelines are increasingly recommending dietary MUFA, primarily at the expense of saturated fatty acids (SFA). Consumption of dietary MUFA promotes healthy blood lipid profiles, mediates blood pressure, improves insulin sensitivity and regulates glucose levels. Moreover, provocative newer data suggest a role for preferential oxidation and metabolism of dietary MUFA, influencing body composition and ameliorating the risk of obesity. Mounting epidemiological and human clinical trial data continue to demonstrate the cardioprotective activity of the MUFA content of dietary fat. As the debate on the optimal fatty acid composition of the diet continues, the benefit of increasing MUFA intakes, particularly as a substitute for dietary SFA, deserves considerable attention.