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2016, Number 3

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Rev Cubana Hematol Inmunol Hemoter 2016; 32 (3)

Prepubertal growth in sickle cell disease

Serrano MJ, García PT, Svarch E, Menéndez VA, Machín GS

Language: Spanish
References: 25
Page: 340-353
PDF size: 183.38 Kb.


ABSTRACT

Introduction: Sickle cell disease is one of the most common genetic diseases in the world and represents a public health problem for many countries. It is associated with delayed growth and development when these patients compared with healthy children of the same age and sex. The factors responsible for stunting in sickle cell disease are not well understood.
Objective: To describe characteristics of prepubertal growth and nutritional status of a group of patients with sickle cell disease at the Institute of Hematology and Immunology.
Methods: 93 patients with sickle cell disease were studied aged 3 to 10 years, 61 with sickle cell disease (AD) and 32 with hemoglobinopathy SC (HSC). We were used as indicators of growth: weight, height, body mass index (BMI), nutritional assessment tables and compared with national standards the clinical picture and levels of fetal hemoglobin (HbF) with growth indicators correlated.
Results: No significant differences in size and weight between the sexes. HSC patients were significantly older than patients with AD height and BMI. 86% of patients were above 10 percentile. The number of clinical events, the size of the liver and spleen did not influence growth; an inverse relationship between the number of painful vasoocclussive crises and BMI was observed in males. The highest levels of HbF were correlated with favorable growth, but without statistical significance.
Conclusions: The systematic monitoring, proper family management, early treatment of complications and proper nutrition favorably influence the growth and development of children with sickle cell disease.


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