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Updated: Mar 9, 2026
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Hormonal and echocardiographic abnormalities in adult patients with sickle-cell anemia in Bahrain
Taysir S Garadah1, Ahmed A Jaradat2, Mohammed E Alalawi3
1Cardiac Unit, Salmaniya Medical Complex, Ministry of Health; Department of Internal Medicine, College of Medicine and Medical Sciences, Arabian Gulf University, Manama.
Insights
Adult sickle cell anemia (SCA) patients show high rates of adrenal, thyroid, and hypogonadism issues. Elevated serum ferritin correlates with lower cortisol, testosterone, and thyroxine levels, indicating endocrine dysfunction in SCA.
Area of Science:
- Endocrinology
- Hematology
- Cardiology
Background:
- Hormonal changes are known in pediatric sickle cell anemia (SCA), but are not well-defined in adults.
- Adult SCA patients may experience adrenal, thyroid, and parathyroid dysfunction.
Purpose of the Study:
- To assess metabolic and endocrine abnormalities in adult SCA patients.
- To evaluate left ventricular (LV) function in adult SCA patients compared to controls.
- To investigate the relationship between serum ferritin and cortisol, free thyroxine (T4), and testosterone levels.
Main Methods:
- Compared 82 adult HbSS SCA patients with a matched control group.
- Measured serum levels of cortisol, parathyroid hormone (PTH), testosterone, thyroid-stimulating hormone (TSH), and free T4.
- Assessed LV mass, wall thickness, cavity dimensions, and diastolic filling velocities using pulsed Doppler echocardiography.
- Analyzed biometric data and performed multiple regression analysis between serum ferritin and hormone levels.
Main Results:
- Adult SCA patients had lower hemoglobin, BMI, cortisol, vitamin D3, testosterone, and T4 compared to controls.
- Significantly higher levels of reticulocyte count, PTH, TSH, ferritin, LDH, ALP, and uric acid were observed in SCA patients.
- High prevalence of vitamin D3 deficiency (61%), hypogonadism (9.8%), subclinical hypothyroidism (7%), and adrenal insufficiency (4.8%) in SCA patients.
- Inverse relationships found between serum ferritin and testosterone, T4, and cortisol levels.
Conclusions:
- Adult SCA patients exhibit a high prevalence of hypoadrenalism, hypogonadism, and hypothyroidism.
- Inverse correlations exist between serum ferritin and cortisol, testosterone, and T4 levels.
- Echocardiography revealed increased LV mass and diastolic dysfunction in adult SCA patients.
Background:
Adrenal, thyroid, and parathyroid gland hormonal changes are recognized in children with homozygous (HbSS) sickle-cell anemia (SCA), but are not clear in adult patients with SCA.
Aim:
To assess the metabolic and endocrine abnormalities in adult patients with SCA and evaluate left ventricular (LV) systolic and diastolic functions compared with patients with no SCA and further study the relationship between serum levels of cortisol, free thyroxine (T4), and testosterone with serum ferritin.
Materials And Methods:
The study was conducted on 82 patients with adult HbSS SCA compared with a sex- and age-matched control group. The serum levels of cortisol, parathyroid hormone (PTH), testosterone, thyroid-stimulating hormone (TSH), and free T4 were compared. Blood levels of hemoglobin, reticulocyte count, lactate dehydrogenase (LDH), calcium, alkaline phosphatase (ALP), vitamin D3, and ferritin were also compared. Pulsed Doppler echo was performed to evaluate the LV mass, wall thickness, and cavity dimensions with diastolic filling velocities of early (E) and atria (A) waves. Biometric data were analyzed as mean ± standard deviation between the two groups. Multiple regression analysis was performed between serum levels of ferritin as independent variable and testosterone, cortisol, and thyroid hormones.
Results:
A total of 82 adult patients with HbSS SCA were enrolled who had a mean age of 21±5.7 years, with 51 males (62%). Patients with SCA compared with the control group had significantly lower hemoglobin, body mass index, cortisol, vitamin D3, testosterone, and T4. Furthermore, there were significantly high levels of reticulocyte count, PTH, TSH, ferritin, LDH, ALP, and uric acid. The incidence of subclinical hypothyroidism and adrenal insufficiency was 7% and 4.8%, respectively, with hypogonadism 9.8% and vitamin D3 deficiency 61%. There were inverse relationships between ferritin as independent variable and serum levels of testosterone, T4, and cortisol, with regression coefficients of -0.49 (P<0.001), -0.33 (P<0.001), and -0.11 (P<0.92), respectively.
Conclusion:
Patients with adult SCA had a high prevalence of in vivo hypoadrenialism (4.8%), hypogonadism (9.8%), and hypothyroidism (7%). There were significant inverse relationships between serum ferritin as independent variable and cortisol, testosterone, and T4. Pulsed Doppler echocardiography showed increased LV mass, with a restrictive LV diastolic pattern suggestive of diastolic dysfunction.
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