Endocrine and Metabolic Complications in Children and Adolescents with Sickle Cell Disease: An Italian Cohort Study
Abstract
Publication Date:
2018
Short description:
Endocrine and Metabolic Complications in Children and Adolescents with Sickle Cell Disease: An Italian Cohort Study / Bigi, Elena; Bruzzi, Patrizia; Palazzi, Giovanni; Predieri, Barbara; Lucaccioni, Laura; Pancaldi, Alessia; Lodi, Mariachiara; Cellini, Monica; Iughetti, Lorenzo. - In: HORMONE RESEARCH IN PAEDIATRICS. - ISSN 1663-2818. - 90:Suppl 1(2018), pp. 479-479. ( 57th Annual Meeting of the European Society for Paediatric Endocrinology (ESPE) Athens, Greece September 27–29, 2018).
abstract:
Background: Children with Sickle Cell Disease (SCD) show endocrine complications and metabolic alterations. The physiopathology of these conditions is not completely understood: iron overload due to chronic transfusions, ischemic damage, and inflammatory state related to vaso-occlusive crises may be involved.
Aims of this study were to evaluate the growth pattern and the endocrine and metabolic alterations in a cohort of children with SCD and to detect the relationship between these conditions and the SCD severity.
Methods: Fifty-two children and adolescents with SCD [38 homozygous sickle hemoglobin (HbSS) and 14 heterozygous sickle hemoglobin (HbSC); age range 3-18 years] were recruited. Anthropometric [height, body mass index (BMI), arm span, sitting height, target height (TH), and pubertal status] and laboratory
[blood cell counts, hemolysis indices, metabolic and nutritional status indices and hormonal blood levels] data were evaluated. The SCD severity was defined according to hematological and clinical parameters.
Results: Height-SDS adjusted for TH and z-score-BMI were significantly higher in HbSC children than in HbSS ones. The 92% (48/52) of the population show at least one metabolic and/or endocrine alteration: insufficiency/deficiency of vitamin D (84.7%), insulin resistance (11.5%), growth hormone deficiency (3.8%), subclinical hypothyroidism (3.8%), and hypogonadism (1.9%). Levels of 25-hydroxy-vitamin D were negatively correlated with clinical indicators of the SCD severity. Subjects with HbSS genotype show significant lower levels of both insulin-like growth factor-I (IGF-1) and insulin-like growth factor binding protein 3 than children with HbSC. In the study group IGF-1 values were positively related with Hb and negatively related with lactate dehydrogenase.
Conclusions: Metabolic and endocrine alterations are very common in children and adolescents with SCD. A regular follow-up is necessary to identify subjects at risk for complications, to precociously initiate an appropriate treatment, and to improve the quality of life of SCD patients.
Aims of this study were to evaluate the growth pattern and the endocrine and metabolic alterations in a cohort of children with SCD and to detect the relationship between these conditions and the SCD severity.
Methods: Fifty-two children and adolescents with SCD [38 homozygous sickle hemoglobin (HbSS) and 14 heterozygous sickle hemoglobin (HbSC); age range 3-18 years] were recruited. Anthropometric [height, body mass index (BMI), arm span, sitting height, target height (TH), and pubertal status] and laboratory
[blood cell counts, hemolysis indices, metabolic and nutritional status indices and hormonal blood levels] data were evaluated. The SCD severity was defined according to hematological and clinical parameters.
Results: Height-SDS adjusted for TH and z-score-BMI were significantly higher in HbSC children than in HbSS ones. The 92% (48/52) of the population show at least one metabolic and/or endocrine alteration: insufficiency/deficiency of vitamin D (84.7%), insulin resistance (11.5%), growth hormone deficiency (3.8%), subclinical hypothyroidism (3.8%), and hypogonadism (1.9%). Levels of 25-hydroxy-vitamin D were negatively correlated with clinical indicators of the SCD severity. Subjects with HbSS genotype show significant lower levels of both insulin-like growth factor-I (IGF-1) and insulin-like growth factor binding protein 3 than children with HbSC. In the study group IGF-1 values were positively related with Hb and negatively related with lactate dehydrogenase.
Conclusions: Metabolic and endocrine alterations are very common in children and adolescents with SCD. A regular follow-up is necessary to identify subjects at risk for complications, to precociously initiate an appropriate treatment, and to improve the quality of life of SCD patients.
Iris type:
Abstract in Rivista
List of contributors:
Bigi, Elena; Bruzzi, Patrizia; Palazzi, Giovanni; Predieri, Barbara; Lucaccioni, Laura; Pancaldi, Alessia; Lodi, Mariachiara; Cellini, Monica; Iughetti, Lorenzo
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