Endocrine Disorders in Sickle-Cell Disease

Cresio      Alves; Zilda      Braid

Abstract

Background: Sickle cell disease is a hemoglobinopathy of global importance due to its worldwide high prevalence. Endocrine and metabolic disorders may be associated with this disease, aggravating its clinical course. Objective: Evaluate the current knowledge about metabolic and endocrine derangements related to sickle cell anemia. Source: Literature search was performed in MEDLINE and SciELO databases, in the last 20 years, combining in different compositions the following terms: sickle cell disease, short stature, delayed puberty, diabetes mellitus, osteoporosis, hypothyroidism and adrenal dysfunction. Results: Endocrine disorders in sickle cell disease have multifarious causes: tissue hypoxia, chronic anemia, iron overload, high energy demand, genetic influence and malnourishment. Slow speed of growth and delayed puberty are frequent. The most common endocrine disorders in sickle cell disease are: growth delay, osteopenia and hypogonadism. Diabetes mellitus, thyroid and adrenal disorders are overall rare in this population. Conclusion: Recognition of an endocrine disturbance is of utmost importance in the follow-up of sickle cell disease patients. Thus, preventive measures, early diagnosis and replacement of specific hormonal deficiencies may be implemented contributing to a better quality of life for these patients.

Keywords: Sickle cell disease, growth and pubertal disorders, diabetes mellitus, adrenal gland diseases, thyroid diseases, metabolic bone diseases

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