Project description:Children with Sickle Cell Disease (SCD) may show growth failure in comparison to healthy peers. Many factors as hematological status, endocrine and/or metabolic dysfunction, and nutritional status, may play an important role in growth failure. The aim of this study was to assess whether impaired growth and nutritional intake can affect SCD severity during childhood.We conducted an observational study on children with SCD referring to our clinic for routine follow-up visits in a 6-month period. We collected information on weight, height and body mass index (BMI) and calculated their respective standardized scores (z). The nutritional intake was assessed through the last 24-h recall intake of total calories, macro- (proteins, lipids, carbohydrates) and micronutrients (calcium, iron, phosphorus, vitamins B1, PP, A, C, B2). Disease severity was assessed through total hemoglobin (Hb) and fetal hemoglobin (HbF), and lactic dehydrogenase (LDH) levels, and through the total number and days of hospitalizations, as well as the lifetime episodes of acute chest syndrome (ACS).Twenty nine children (14 males, 15 females) with SCD were enrolled; their mean age was 9.95 years (SD 3.50, min 3.72, max 17.18). Z-weight and z-BMI were significantly directly related to total Hb. Food intake resulted significantly unbalanced in terms of total calorie intake, macro- and micronutrients, especially calcium, iron, vitamin B1 and C. Low intake of calcium and vitamin B1 were significantly inversely correlated with number and days of hospitalizations per year. Protein, lipid, phosphorus, and vitamin PP intakes resulted adequate but were inversely correlated with number and days of hospitalizations. Carbohydrate, lipid, iron, phosphorus, vitamins B1 and B2 intakes were significantly inversely correlated to HbF levels.This study showed that, in our population, inadequate nutritional intake, weight and BMI have a significant impact on SCD severity indices.
Project description:Vitamin B12 deficiency poses a health concern, especially in vulnerable populations. Dietary vitamin B12 intake was obtained by two 24 h dietary recalls and food propensity questionnaires in a representative Slovenian cross-sectional food consumption survey, SI.Menu (n = 1248 subjects; 10-74 years). For a subgroup of 280 participants, data on serum vitamin B12 were available through the Nutrihealth study. The estimated usual population-weighted mean daily vitamin B12 intakes were 6.2 µg (adults), 5.4 µg (adolescents), and 5.0 µg (elderly). Lower intakes were observed in females. Inadequate daily vitamin B12 intake (<4 µg) was detected in 37.3% of adolescents, 31.7% of adults, and 58.3% elderlies. The significant predictors for inadequate daily vitamin B12 intake were physical activity score in all age groups, sex in adolescents and adults, financial status and smoking in elderly, and employment in adults. Meat (products), followed by milk (products), made the highest vitamin B12 contribution in all age groups. In adolescents, another important vitamin B12 contributor was cereals. The mean population-weighted serum vitamin B12 levels were 322.1 pmol/L (adults) and 287.3 pmol/L (elderly). Low serum vitamin B12 concentration (<148 nmol/L) and high serum homocysteine (>15 µmol/L) were used as criteria for vitamin B12 deficiency. The highest deficiency prevalence was found in elderlies (7.0%), particularly in males (7.9%). Factors associated with high serum homocysteine were also investigated. In conclusion, although vitamin B12 status was generally not critical, additional attention should be focused particularly to the elderly.
Project description:Early identification of infants with sickle cell disease (SCD) by newborn screening, now universal in all 50 states in the US, has improved survival, mainly by preventing overwhelming sepsis with the early use of prophylactic penicillin. Routine transcranial Doppler screening with the institution of chronic transfusion decreases the risk of stroke from 10% to 1% in paediatric SCD patients. Hydroxyurea decreases the number and frequency of painful crises, acute chest syndromes and number of blood transfusions in children with SCD. Genetic research continues to be driven toward the prevention and ultimate cure of SCD before adulthood. This review focuses on clinical manifestations and therapeutic strategies for paediatric SCD as well as the evolving topic of gene-focused prevention and therapy.
Project description:ObjectiveTo determine the prevalence and correlates of healthy vitamin D status in lactating Inuit women living in remote regions of the Arctic.DesignCross-sectional.SettingHouseholds were selected randomly in thirty-six communities of Nunavut, Nunatsiavut and Inuvialuit Settlement Region. Dietary intake was assessed using a 24 h recall and an FFQ. Anthropometric measurements, household living conditions, supplement use and health status were assessed. In fasting samples, serum 25-hydroxyvitaimn D (25(OH)D) was measured using a chemiluminescent assay (LIAISON; Diasorin Inc.).SubjectsLactating Inuit women participating in the 2007-2008 International Polar Year Inuit Health Survey conducted in the months of August to October.ResultsAmong participants (n 34), 8·8, 26·5 and 50·0 % had 25(OH)D concentrations at or above 75, 50 and 40 nmol/l, respectively. More than one-third of participants did not consume traditional foods during the previous day and only 11·3 % of total energy intake was derived from traditional foods. Only 14·7 % of the sample consumed the daily number of milk servings recommended by Canada's Food Guide (two servings) for First Nations, Inuit and Métis. Using multivariable logistic regression to examine 25(OH)D≥40 nmol/l, only higher body fat was inversely correlated with 25(OH)D concentration.ConclusionsThe present study is the first to assess simultaneously vitamin D status and other known factors that affect it among lactating Inuit women living in remote communities in the Arctic. Healthy maternal vitamin D status was observed in 25 % of participants during the late summer and early autumn. This requires further assessment in a larger sample spanning more seasons.
Project description:Hydroxyurea therapy offers promise for ameliorating the clinical course of children with sickle cell disease (SCD). Hydroxyurea is a prototypic therapeutic option; it can be administered with minimal side effects, has a relatively wide therapeutic window, and has mechanisms of action that address pathophysiologic pathways of sickling, vaso-occlusion, hemolysis, and organ damage. There are limited data regarding hydroxyurea's ability to prevent or diminish organ dysfunction, and the long-term risks of hydroxyurea therapy remain incompletely defined. Although clinical trials are underway to address long-term issues, hydroxyurea remains an effective but underutilized therapy for SCD.
Project description:ObjectiveTo compare the dietary habits of children living in northern villages and in the capital of Greenland, given the reported transition from traditional to westernised diet in adults over recent decades, and to explore the association between consumption of marine mammals and fish (MMF) and the children's metabolic profile and vitamin D status.DesignChildren answered an FFQ encompassing sixty-four individual food types pooled into six food categories. Their pubertal stage, body fat, fitness level, metabolic profile (non-HDL-cholesterol, glycated Hb, insulin, glucose, high-sensitivity C-reactive protein) as well as serum 25-hydroxyvitamin D (25(OH)D) concentration were evaluated.SettingSiorapaluk and Qaanaaq (north of Greenland) and Nuuk (west).ParticipantsChildren aged 6-18 years (n 177).ResultsMMF were most frequently eaten by children from Siorapaluk (mean (sd): 73·4 (14·1) times/month), followed by children from Qaanaaq (37·0 (25·0) times/month), and least often eaten by children from Nuuk (23·7 (24·6) times/month; P < 0·001). Children from Qaanaaq consumed 'junk food' more frequently (P < 0·001) and fruits and vegetables less frequently (P < 0·01) than children from Nuuk. MMF consumption was positively associated with serum 25(OH)D concentration (P < 0·05), but the overall prevalence of vitamin D deficiency was high (18 %). No association was found between MMF consumption and metabolic parameters.ConclusionsThe dietary transition and influence of western diets have spread to the north of Greenland and only the most remote place consumed a traditional diet highly based on MMF. We found no strong associations of MMF consumption with metabolic health, but a positive association with vitamin D status.
Project description:The wide spectrum of vitamin D activity has focused attention on its potential role in the elevated burden of disease in a northern Canadian First Nations (Dené) cohort. Vitamin D insufficiency, and gene polymorphisms in the vitamin D receptor (VDR) and vitamin D binding protein (VDBP) have been implicated in susceptibility to infectious and chronic diseases. The objectives of this study were to determine the contribution of vitamin D from food, and measure the serum concentrations of 25-hydroxyvitamin D(3) (25-OHD(3)) and VDBP in Dené participants. Single nucleotide polymorphisms (SNPs) associated with the dysregulation of the innate immune response were typed and counted. Potential correlations between the SNPs and serum concentrations of 25-OHD(3) and VDBP were evaluated. Venous blood was collected in summer and winter over a one-year period and analyzed for 25-OHD(3) and VDBP concentrations (N?=?46). A questionnaire was administered to determine the amount of dietary vitamin D consumed. Sixty-one percent and 30% of the participants had 25-OHD(3) serum concentrations <75 nmol/L in the winter and summer respectively. Mean vitamin D binding protein concentrations were within the normal range in the winter but below normal in the summer. VDBP and VDR gene polymorphisms affect the bioavailability and regulation of 25-OHD(3). The Dené had a high frequency of the VDBP D432E-G allele (71%) and the Gc1 genotype (90%), associated with high concentrations of VDBP and a high binding affinity to 25-OHD(3). The Dené had a high frequency of VDR Fok1-f allele (82%), which has been associated with a down-regulated Th1 immune response. VDBP and VDR polymorphisms, and low winter 25-OHD(3) serum concentrations may be risk factors for infectious diseases and chronic conditions related to the dysregulation of the vitamin D pathway.
Project description:AimVitamin D (VitD) is involved in calcium and phosphate homeostasis, bone health, and normal functioning of the immune system. VitD status is monitored using serum 25-hydroxy-vitamin D (25(OH)D) as a biomarker. Serum 25(OH)D concentrations below 30 nmol/L indicate VitD deficiency and below 50 nmol/L indicate insufficiency. VitD can be synthesised endogenously in human skin when exposed to ultraviolet B (UVB) radiation. In the absence of sufficient UVB-light exposure, VitD intake becomes the main source of VitD, with a recommended daily intake of 20 μg. The aim of this study was to conduct a review and meta-analysis on the abovementioned topics, focusing on scientific studies in various Slovenian populations.MethodsWe conducted a systematic review and meta-analysis of published scientific papers, academic theses, or conference contributions reporting serum 25(OH)D status and VitD intake across various Slovenian populations. A search was carried out using Web of Science, Scopus, Medline, and the Slovenian library database.ResultsWe identified 43 pertinent studies that addressed 25(OH)D status and 16 that addressed VitD intake. Serum 25(OH)D status was generally low across all populations, and notable seasonal variability was observed. VitD intakes were below 5 μg in all studies.ConclusionsA general observation is that various population groups across Slovenia are at high risk of vitamin D insufficiency and deficiency, particularly during wintertime. Regarding vitamin D intake, all included studies reported daily intakes below the recommended level. We also identified key research gaps that need to be addressed to support further public health decision-making.
Project description:Introduction:There is evidence that 25-hydroxyvitamin D levels are lower in overweight and obese youth. This study examined the relationship between weight status and 25-hydroxyvitamin D, while controlling for confounders, in Canadian youth. Methods:Plasma 25-hydroxyvitamin D from subjects aged 6 to 17 years from the Canadian Health Measures Survey cycles 1 (2007 to 2009) and 2 (2009 to 2011) was used. Sex-specific multiple linear regression and logistic regressions examined the relationship of overweight and obesity (body mass index ? 85th percentile) with 25-hydroxyvitamin D levels and the odds of 25-hydroxyvitamin D <40 nmol/L and <50 nmol/L. Results:The prevalence of risk of vitamin D deficiency (25-hydroxyvitamin D < 30 nmol/L) was 6% (95% confidence interval [CI] 3.26% to 10.12%). Vitamin D inadequacy, estimated by levels <40 nmol/L, was 15% (95% CI 10.34% to 20.39%; 19% [95% CI 13.1 to 25.6] for teenagers). Seventy per cent (95% CI 63.59 to 75.17) had levels >50 nmol/L, consistent with achieving the Recommended Dietary Allowance. In adjusted analyses, overweight/obesity (1/3 of subjects) was independently associated with lower 25-hydroxyvitamin D for both sexes after adjustment for age, race, income, season, vitamin D supplementation and daily milk consumption. For 25-hydroxyvitamin D <40 nmol/L, the overweight/obese odds ratio for males was 2.63 (95% CI 1.34 to 5.18). For 25-hydroxyvitamin D <50 nmol/L, overweight/obese odds ratios were 2.19 (95% CI 1.46 to 3.28) for males and 1.39 (95% CI 1.05 to 1.84) for females. Conclusions:This study confirms the inverse association between adiposity and serum concentrations of 25-hydroxyvitamin D in Canadian youth and the independent association of overweight/obesity to 25-hydroxyvitamin D level and vitamin D status after adjustment for other factors.