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.
Highlights
Children with Sickle Cell Disease (SCD) may show growth failure in comparison to healthy peers
Our study showed that body weight and body mass index (BMI) are significantly directly related to total Hb mean values, and to the severity of SCD
Standardized weight and BMI contributed, in our population, to the 17 and 15 % respectively of the total variance of total Hb levels. These findings are in line with others studies that reported a relationship between growth failure, and/or inadequate nutritional intake, and disease severity in SCD patients, suggesting that a targeted nutritional intervention could reduce the clinical severity of SCD [8]
Summary
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. SCD may affect physical growth during childhood and early adolescence, and children with this condition are often leaner and shorter than. Mandese et al Nutrition Journal (2016) 15:46 established Other nutrients such as zinc, glutamine, Larginine and antioxidants could provide important therapeutic benefit in children with SCD [5]. Growth failure and under-nutrition are common, the underlying mechanisms have not been well studied and the precise roles of intrinsic and extrinsic factors are unclear. Inadequate food intake or increased demands associated with higher energy expenditure and requirements could be both responsible [6]
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