Effects of nutritional intake on disease severity in children with sickle cell disease

Abstract

BackgroundChildren 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.MethodsWe 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).ResultsTwenty 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.ConclusionsThis study showed that, in our population, inadequate nutritional intake, weight and BMI have a significant impact on SCD severity indices.

Nutritional status of children with sickle cell disease

“…Albumin (g/L) 39.0 6 3.4 38.6 6 4.1 39 6 4.5 0.9 Serum ferritin (ng/ml) 167.0 6 173.8 117.9 6 181.3 154.0 6 164.6 0.5 WBC (10 3 /mL) 10.4 6 5.2 10.8 6 4.9 9.8 6 5.5 0.4 RBC (10 6 /m L) 3.4 6 1.2 3.3 6 0.9 3.5 6 1.4 0.3 Hb (g/dL) 8.6 6 1.7 8.4 6 1.8 8.7 6 1.7 0.6 HCT (%) 27.2 6 5.9 26.9 6 5.8 27.4 6 5.9 0.7 MCV (fL) 80.6 6 11.3 81.5 6 12.4 79.2 6 9.6 0.3 MCH (pg) 25.8 6 3.3 25.9 6 3. Children with SCD have higher metabolic rate and resting energy expenditure, and this may increase metabolic and nutrient demands; thus inadequate nutrient intake makes children with SCD susceptible to undernutrition, which increases disease severity (Mandese et al, 2015). Anthropometric measures reflect the nutritional and growth status of children with SCD.…”

Vitamin E Status and Total Body Water in Children and Adolescents with Sickle Cell Anemia

“…Results revealed depleted selenium and vitamin E levels in children with TM and SCD relative to healthy controls, which supports the findings of the current study where participants had significantly lower corrected plasma vitamin E levels than reference levels. Mandese et al, also found children with SCD to have many vitamin deficiencies, including vitamin E [16].…”

Fetal hemoglobin level and nutritional status in patients with sickle cell disease

“…With great interest, I have read an article by Mandese et al [ 1 ], recently published in Nutrition Journal. The study reported on the nutritional status among children with sickle cell disease (SCD) in relation to disease severity and other morbidity outcomes.…”

“…The study reported on the nutritional status among children with sickle cell disease (SCD) in relation to disease severity and other morbidity outcomes. The authors reported a negative correlation between fetal hemoglobin levels and the intake of carbohydrates, lipids, iron, phosphorus, vitamins B1, and B2, suggesting a beneficial effect that low intake of these nutritional elements [ 1 ]. However, I would like to highlight two points.…”

“…First, the authors used a number of measures to examine disease severity, however the evidence to support the rationale for choosing the measures was unclear. Second, Mandese et al presented some data in Table 2 in their article that do not match the text [ 1 ]. All reported correlations (r 2 ) related to intake of different nutritional elements have positive significant values suggesting direct relationship, including mean fetal hemoglobin level, the number of hospitalizations and the number of days for hospitalizations in the year prior.…”