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Evaluation of endocrine changes and insulin release in patients with hereditary spherocytosis
Zeynep Ağırman1, Fatih Temiz2, Can Acıpayam3
1Department of Pediatrics, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey.
Insights
Patients with hereditary spherocytosis (HS) show reduced insulin secretion and hypocholesterolemia, likely due to chronic hemolysis. Further research is needed to understand the underlying pathophysiology of these endocrinological changes in HS.
Area of Science:
- Endocrinology
- Hematology
- Metabolic Disorders
Background:
- Hereditary spherocytosis (HS) is a genetic hemolytic anemia.
- Endocrinological complications in HS are not well-characterized.
- Understanding metabolic changes in HS is crucial for comprehensive patient management.
Purpose of the Study:
- To investigate endocrinological alterations in patients with hereditary spherocytosis.
- To evaluate insulin secretion capacity and related metabolic markers in HS patients.
- To identify potential links between chronic hemolysis and endocrine dysfunction.
Main Methods:
- A case-control study comparing 30 HS patients with 30 healthy controls.
- Comprehensive biochemical and hormonal tests were performed.
- Insulin resistance (HOMA-IR) and beta-cell function (HOMA-ß%) were calculated.
Main Results:
- HS patients exhibited significantly lower insulin, glucose, HOMA-IR, HOMA-beta, and C-peptide levels compared to controls.
- Prevalence of short stature (23.3%), vitamin D deficiency (53.3%), dyslipidemia (76.6%), and growth hormone deficiency (6.6%) was noted in HS patients.
- HS patients showed significantly lower cholesterol, HDL, and LDL levels.
Conclusions:
- Insulin secretion is decreased in patients with hereditary spherocytosis.
- Hypocholesterolemia in HS patients is associated with chronic hemolysis.
- Further investigation is required to elucidate the pathophysiology of reduced insulin secretion in HS.
Aim:
To evaluate endocrinological changes and insulin secretion in patients with hereditary spherocytosis (HS).
Methods:
The study included 30 patients with HS and 30 healthy control groups who were of similar age and gender. Routine tests, including hemogram, biochemical and hormonal tests were conducted on both patients with HS and the control group. HOMA-IR (Homeostasis Model Assessment for Insulin Resistance) and HOMA-ß% (Homeostasis Model Assessment for ß-cell function) values of all cases were also calculated using fasting insulin and fasting glucose values.
Results:
Among the patient group, 7 patients (23.3%) had short stature, 16 patients (53.3%) had vitamin D deficiency, 1 patient (3.3%) had impaired glucose tolerance, 1 patient (3.3%) had subclinical hypothyroidism, 23 patients (76.6%) had dyslipidemia and 2 patients (6.6%) had growth hormone deficiency. The insulin value in the patient group was 4.0 ± 2.7 mlU/mL and significantly lower than the control group with an insulin value of 9.1 ± 3.9 mlU/mL (P < 0.001). Moreover, glucose (P = 0.036), HOMA-IR (P < 0.001), HOMA-beta (P < 0.001) and C-peptide (P = 0.001) values of the patient group were significantly lower than the control group. The cholesterol (P < 0.001), HDL (P < 0.001) and LDL (P < 0.001) values of the patient group were found to be significantly lower than the control group.
Conclusion:
We found that insulin secretion decreased in patients with HS and hypocholesterolemia occurred due to chronic hemolysis. More research is needed to elucidate the pathophysiology of decreased insulin secretion seen in HS patients.
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