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Endocrinopathies complicating transfusion-dependent hemoglobinopathy
Abdulmoein E Al-Agha1, Noor S Bawahab, Sarah A Nagadi
1Department of Paediatric Endocrinology, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. E-mail. aagha@kau.edu.sa.
Insights
Endocrinopathies like short stature and diabetes mellitus are common in children with thalassemia and sickle cell anemia. These endocrine disorders are significant complications requiring attention in managing these blood disorders.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Metabolic Disorders
Background:
- Transfusion-dependent thalassemia and sickle-cell anemia are inherited blood disorders requiring chronic medical management.
- Endocrine complications are increasingly recognized in patients with hemoglobinopathies.
- Understanding the prevalence of endocrinopathies is crucial for comprehensive patient care.
Purpose of the Study:
- To determine the frequency and clinical significance of various endocrinopathies in pediatric patients with transfusion-dependent thalassemia and sickle-cell anemia.
- To explore associations between specific endocrinopathies and patient characteristics or treatment history.
Main Methods:
- Retrospective study of 119 pediatric patients diagnosed with thalassemia or sickle-cell anemia.
- Data collected from electronic medical records at King Abdulaziz University Hospital (2010-2018).
- Statistical analysis performed using SPSS version 20.0.
Main Results:
- Short stature (39.5%), diabetes mellitus (29.4%), and hypogonadism (12.6%) were the most prevalent endocrinopathies.
- Endocrinopathies showed a statistically significant relationship with hemoglobinopathies, except for osteopenia and osteoporosis.
- Hypogonadism and hypocortisolism were linked to splenectomy history; short stature correlated with male gender.
Conclusions:
- Endocrinopathy is a frequent complication in children and adolescents with hemoglobinopathies.
- Short stature, diabetes mellitus, and low bone mineral density are the most common endocrine issues.
- Early detection and management of these endocrinopathies are essential for improving long-term outcomes.
Objectives:
To investigate the prevalence and significance of different endocrinopathies in children and adolescents with transfusion-dependent thalassemia and sickle-cell anemia.
Methods:
This is a descriptive, retrospective study between January 2010 and July 2018 in King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Data was collected through reviewing electronic hospital medical records then filling out data collection sheets and was interpreted through the IBM SPSS Statistics for Windows version 20.0 (IBM Corp, Armonk, NY, USA). Results: The total sample size was 119 patients, gender equality was almost achieved with 55.5% being male and 45.5% being female. The most common endocrinopathies were identified in the following order of short stature (39.5%), diabetes mellitus (29.4%), hypogonadism (12.6%), osteopenia (12.6%), osteoporosis (9.2%), hypothyroidism (9.2%), hypocortisolism (3.4%), and hypoparathyroidism (2.5%). All of which were statistically significant in their relationship to hemoglobinopathies with the exception of osteopenia and osteoporosis. Hypogonadism and hypocortisolism were found to be statistically significant in their relationship to a positive history of splenectomy at p=0.026 and p=0.012. Short stature was found to be statistically significant in its relationship to the male gender with a p=0.001. Conclusion: Endocrinopathy is a frequent complication of hemoglobinopathies, for which the most common were found to be short stature, diabetes mellitus, and low bone mineral density.
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