PEDIATRIC ENDOCRINOLOGY/4ED: Volume 1 (Clinical Pediatrics, 9)
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There is currently no agreement on the causes of this complication and further investigations are needed. We found a case of hypergonadotropic hypogonadism and one case of ovarian failure in HbSS genotype group.
The etiology of hypogonadism in SCD is not fully understood yet: in some cases, primitive gonadal failure is related to structural anomalies, resulting from chronic tissue damage associated with chronic anemia condition and local vaso-occlusive crises [ 45 ]. According to this hypothesis, our study demonstrated a direct correlation between Hb levels and testosterone average values in males, demonstrating how clinical control can affect reproductive function. This study has some important limitations. This is a single center study with a small sample size. Secondly there are some confounders factors i.
The main outcome of this study was to report clinical features of our patients, in a cross sectional way, to better understand the actual prevalence of both metabolic alterations and endocrine complications. Surely, a longitudinal study design of these parameters will provide us more information on the natural history of these complications in SCD.
However, it must be considered that in our country SCD is a rare disease and, at the best of our knowledge, this is the first Italian study on these topics.
In literature there are very little data available on these condition in pediatric patients with SCD mainly in European Countries. We think that it is important to evaluate these conditions in different environmental setting. Subjects with SCD show a high prevalence of metabolic alterations and endocrine complications.
However, our results suggest that through the achievement of a good clinical control the SCD patients can obtain a positive impact on growth, metabolic and endocrine function. Consequently, it is crucial to perform periodic anthropometric and endocrine evaluations, especially during puberty, and to have a comprehensive approach to this disease in order to reduce its long-term complications. Mortality in sickle cell disease.
Life expectancy and risk factors for early death. N Engl J Med.
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World J Clin Pediatr. Body composition in children with sickle cell disease. Am J Clin Nutr. Sickle-cell disease. Therapy insight: metabolic and endocrine disorders in sickle cell disease. Nat Clin Pract Endocrinol Metab. Endocrine functions in sickle cell anaemia patients. J Trop Pediatr. Study of gonadal hormones in Egyptian female children with sickle cell anemia in correlation with iron overload: single center study.
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Brook's clinical pediatric endocrinology. Oxford: Wiley-Blackwell; Frequency and risk factors of endocrine complications in Turkish children and adolescents with sickle cell Anemia. Turk J Hematol. Growth and nutritional status of children with homozygous sickle cell disease. Ann Trop Paediatr.
Effects of delayed pubertal development, nutritional status, and disease severity on longitudinal patterns of growth failure in children with sickle cell disease. Pediatr Res. Factors affecting prepubertal growth in homozygous sickle cell disease. Growth and pubertal development in transfusion-dependent children and adolescents with thalassaemia major and sickle cell disease: a comparative study.
Height and weight reference curves for homozygous sickle cell disease. Low vitamin D status in children with sickle cell disease. J Pediatr. Vitamin D deficiency and comorbidities in children with sickle cell anemia. Pediatr Hematol Oncol. Status of vitamin D in children with sickle cell disease living in Madrid, Spain.
Eur J Pediatr. Energy intake and resting metabolic rate in preschool Jamaican children with homozygous sickle cell disease.
Prevalence of vitamin D deficiency in sickle cell disease: a systematic review. PLoS One. Evidence for alteration of the vitamin D-endocrine system in blacks. J Clin Invest.
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The uncertain significance of low vitamin D levels in African descent populations: a review of the bone and cardiometabolic literature. Prog Cardiovasc Dis.
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