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Effects of nutritional intake on disease severity in children with sickle cell disease
Valentina Mandese1, Francesca Marotti1, Luca Bedetti1
1School of Pediatrics, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, Modena, 41124, Italy.
Insights
Children with Sickle Cell Disease (SCD) experiencing growth failure and poor nutrition have increased disease severity. Addressing nutritional intake and weight is crucial for managing SCD in children.
Area of Science:
- Pediatric Hematology
- Nutritional Science
- Chronic Disease Management
Background:
- Children with Sickle Cell Disease (SCD) often exhibit growth failure compared to healthy children.
- Growth failure in SCD is multifactorial, influenced by hematological status, endocrine/metabolic issues, and nutrition.
- Understanding the link between growth, nutrition, and SCD severity is vital for effective pediatric care.
Purpose of the Study:
- To investigate the impact of impaired growth and nutritional intake on the severity of Sickle Cell Disease in children.
- To identify specific nutritional deficiencies and their correlation with disease markers and hospitalizations in pediatric SCD patients.
Main Methods:
- An observational study involving 29 children with SCD was conducted over a 6-month period.
- Data collected included anthropometric measurements (weight, height, BMI) and standardized scores (z-scores).
- Nutritional intake (calories, macronutrients, micronutrients) was assessed via 24-hour recall; disease severity was evaluated using hemoglobin levels, LDH, hospitalizations, and acute chest syndrome episodes.
Main Results:
- Z-weight and z-BMI were significantly correlated with total hemoglobin (Hb) levels.
- Children with SCD showed significant nutritional imbalances, particularly low intake of calories, calcium, iron, and vitamins B1 and C.
- Low intake of calcium and vitamin B1 correlated with increased hospitalizations; adequate protein, lipid, phosphorus, and vitamin PP intake correlated inversely with hospitalizations.
Conclusions:
- Inadequate nutritional intake and impaired growth (low weight and BMI) significantly impact Sickle Cell Disease severity in the studied pediatric population.
- Dietary interventions focusing on specific micronutrients like calcium and vitamin B1 may help reduce hospitalization rates in children with SCD.
- Comprehensive management of pediatric SCD should integrate nutritional assessment and support alongside hematological monitoring.
Background:
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.
Methods:
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).
Results:
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.
Conclusions:
This study showed that, in our population, inadequate nutritional intake, weight and BMI have a significant impact on SCD severity indices.
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