Growth Delays and Developmental Challenges in Children with Sickle Cell Disease

Introduction: Sickle Cell Disease (SCD) is an inherited hemoglobinopathy that leads to a variety of systemic complications, including impaired growth and development in children. Growth retardation, delayed puberty, and malnutrition are common among affected individuals. Although hydroxyurea (HU) therapy is frequently used to reduce disease complications, its effect on growth outcomes remains uncertain. This study investigates the growth patterns, developmental challenges, and impact of hydroxyurea in children with SCD. Methods: A prospective observational study was conducted at a pediatric hematology clinic in Vadodara, Gujarat, involving 50 children with SCD aged 5-18 years, all undergoing hydroxyurea treatment. Anthropometric measurements (weight, height, BMI) were taken at baseline and at regular follow-ups over a one-year period. Sexual maturity ratings were also monitored. Statistical analysis was performed using chi-square tests. Results: The study revealed that the children experienced modest improvements in weight and height over the course of the year. The average weight-for-age increased from 26.38 kg to 28.98 kg, while height-for-age improved from 130.91 cm to 137.25 cm. Despite these gains, a majority of children remained underweight, with BMI between the 5th and 25th percentiles. Delayed sexual maturation was noted in several participants, particularly males. Hydroxyurea doses ranged from 20.01 mg/kg/day to 21.83 mg/kg/day, with no significant adverse effects on growth observed. Discussion: Consistent with existing literature, this study confirmed that children with SCD suffer from stunted growth and delayed sexual maturation, primarily due to chronic anemia, increased energy expenditure, and frequent infections. Although hydroxyurea reduced the frequency of painful crises, its impact on promoting growth was limited. Nutritional interventions and regular growth monitoring are critical for improving long-term outcomes in children with SCD. Additional interventions, including hormonal assessments, may be necessary to address persistent growth and pubertal delays. Conclusion: Children with SCD are at risk for significant growth delays and developmental challenges. While hydroxyurea helps in managing some aspects of the disease, it does not fully address growth and maturation issues. Comprehensive care, including nutritional support and continuous growth monitoring, is essential to optimize outcomes for these children.

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2024-10-07
DOI
https://doi.org/10.5281/zenodo.14031087
Primary Topic
Hemoglobinopathies and Related Disorders
Type
article
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article

Growth Delays and Developmental Challenges in Children with Sickle Cell Disease

Zenodo (CERN European Organization for Nuclear Research)
Hemoglobinopathies and Related Disorders
article

Growth Delays and Developmental Challenges in Children with Sickle Cell Disease

article en

Abstract

Introduction: Sickle Cell Disease (SCD) is an inherited hemoglobinopathy that leads to a variety of systemic complications, including impaired growth and development in children. Growth retardation, delayed puberty, and malnutrition are common among affected individuals. Although hydroxyurea (HU) therapy is frequently used to reduce disease complications, its effect on growth outcomes remains uncertain. This study investigates the growth patterns, developmental challenges, and impact of hydroxyurea in children with SCD. Methods: A prospective observational study was conducted at a pediatric hematology clinic in Vadodara, Gujarat, involving 50 children with SCD aged 5-18 years, all undergoing hydroxyurea treatment. Anthropometric measurements (weight, height, BMI) were taken at baseline and at regular follow-ups over a one-year period. Sexual maturity ratings were also monitored. Statistical analysis was performed using chi-square tests. Results: The study revealed that the children experienced modest improvements in weight and height over the course of the year. The average weight-for-age increased from 26.38 kg to 28.98 kg, while height-for-age improved from 130.91 cm to 137.25 cm. Despite these gains, a majority of children remained underweight, with BMI between the 5th and 25th percentiles. Delayed sexual maturation was noted in several participants, particularly males. Hydroxyurea doses ranged from 20.01 mg/kg/day to 21.83 mg/kg/day, with no significant adverse effects on growth observed. Discussion: Consistent with existing literature, this study confirmed that children with SCD suffer from stunted growth and delayed sexual maturation, primarily due to chronic anemia, increased energy expenditure, and frequent infections. Although hydroxyurea reduced the frequency of painful crises, its impact on promoting growth was limited. Nutritional interventions and regular growth monitoring are critical for improving long-term outcomes in children with SCD. Additional interventions, including hormonal assessments, may be necessary to address persistent growth and pubertal delays. Conclusion: Children with SCD are at risk for significant growth delays and developmental challenges. While hydroxyurea helps in managing some aspects of the disease, it does not fully address growth and maturation issues. Comprehensive care, including nutritional support and continuous growth monitoring, is essential to optimize outcomes for these children.

Zenodo (CERN European Organization for Nuclear Research)
Sarojini Naidu Medical College (IN), Hoag Orthopedic Institute (US), Czech Academy of Sciences, Institute of Physiology (CZ)
Zero hunger
Openalex Percentile: Top 49%
Hemoglobinopathies and Related Disorders
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