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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Endocrine and metabolic complications in children and adolescents with Sickle Cell Disease: an Italian cohort study
V Mandese1, E Bigi2, P Bruzzi3
1Post Graduate School of Pediatrics, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, Via del Pozzo 71, 41124, Modena, Italy.
Insights
Sickle Cell Disease (SCD) in children often leads to metabolic and endocrine issues, particularly vitamin D deficiency. Early monitoring is crucial for timely treatment and improved quality of life for these patients.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Metabolic Disorders
Background:
- Children with Sickle Cell Disease (SCD) frequently experience endocrine complications and metabolic alterations.
- The exact causes, including iron overload, ischemia, and inflammation from vaso-occlusive crises, are not fully understood.
- Understanding these complications is vital for managing SCD in pediatric populations.
Purpose of the Study:
- To evaluate growth patterns, endocrine complications, and metabolic alterations in children and adolescents with SCD.
- To determine the relationship between these health issues and the severity of SCD.
- To identify specific risk factors and correlations within the SCD pediatric cohort.
Main Methods:
- Recruited 52 children and adolescents (ages 3-18) with SCD (38 HbSS, 14 HbSC).
- Assessed anthropometric data (height, BMI, etc.) and laboratory markers (hemolysis, metabolic/nutritional status, hormones).
- Defined SCD severity using hematological and clinical parameters.
Main Results:
- 92% of patients had at least one metabolic/endocrine alteration, most commonly vitamin D deficiency (84.7%).
- HbSC patients had higher height-SDS and BMI-SDS than HbSS patients.
- Vitamin D levels correlated negatively with SCD severity; HbSS patients had lower IGF-1 and IGFBP3 levels than HbSC patients.
Conclusions:
- Metabolic and endocrine complications are highly prevalent in pediatric SCD.
- Regular follow-up is essential for early detection and intervention.
- Proactive management can improve the quality of life for children with SCD.
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, endocrine complications, and metabolic alterations and to detect the relationship between these conditions and the SCD severity in affected children and adolescents.
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 BMI-SDS were significantly higher in HbSC children than in HbSS ones. Forty-eight out of 52 patients (92%) had 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 vitamin D were significantly and negatively correlated with clinical indicators of the SCD severity. Subjects with HbSS genotype show significant lower levels of both insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein 3 than children with HbSC. In the study population IGF-1 values were significantly and positively correlated with Hb and negatively with lactate dehydrogenase.
Conclusions:
Metabolic alterations and endocrine complications are very common in children and adolescents with SCD. A regular follow-up is necessary to identify subjects at risk for complications to precociously start an appropriate treatment and to improve the quality of life of SCD patients.
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