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Published on: November 5, 2019
Understanding growth failure in children with homozygous sickle-cell disease
1University of Pennsylvania, Philadelphia, PA, USA. erin.bennett@alumni.upenn.edu
Insights
Sickle-cell disease (SCD) affects growth in children, starting around six months of age. Understanding its multifactorial causes is crucial for healthcare providers to manage these growth deficits effectively.
Area of Science:
- Pediatrics
- Genetics
- Hematology
Background:
- Sickle-cell disease (SCD) is a common genetic blood disorder in the U.S.
- Children with SCD exhibit impaired growth and delayed maturation, though the underlying reasons are not fully understood.
- Growth deviations in SCD typically emerge around six months after birth.
Purpose of the Study:
- To review existing literature on growth failure in children with sickle-cell disease.
- To elucidate the multifactorial pathophysiology contributing to growth deficits in pediatric SCD.
- To emphasize the importance of healthcare providers recognizing and managing SCD-related growth patterns.
Main Methods:
- Literature review of studies on pediatric sickle-cell disease and growth.
- Analysis of growth patterns from birth to identify divergence points.
- Synthesis of information regarding the pathophysiology of growth failure in SCD.
Main Results:
- Children with SCD show normal birth measurements, with growth divergence beginning around six months.
- Growth deficits in pediatric SCD are multifactorial.
- The clinical significance and consequences of these growth deficits are substantial.
Conclusions:
- Healthcare providers must be aware of the unique growth trajectories in sickle-cell disease.
- Early recognition of growth deviations is essential for clinical management.
- Further understanding of SCD pathophysiology can improve patient outcomes.
Abstract:
Sickle-cell disease is the most prevalent genetic hematologic condition in the United States. Numerous studies have demonstrated poor growth and delayed maturation in children with homozygous sickle-cell disease; however, the pathophysiology remains inadequately understood. Affected children have normal weight and length at birth, and then around 6 months of age their growth patterns begin to diverge from the norm. The growth deficits experienced by these children remain a problem with clinical significance and intangible consequences. A review of literature has provided insight into the multifactorial basis of the growth failure experienced by this population. It is important that nurses and health care providers are familiar with the growth patterns unique to sickle-cell disease and recognize their role in clinical practice.
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