Evaluation of endocrine changes and insulin release in patients with hereditary spherocytosis.

Autor: Ağırman Z; Department of Pediatrics, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey., Temiz F; Department of Pediatric Endocrinology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey., Acıpayam C; Department of Pediatric Hematology and Oncology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey., Akkececi N; Department of Physiology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey.
Jazyk: angličtina
Zdroj: Journal of paediatrics and child health [J Paediatr Child Health] 2024 Dec 12. Date of Electronic Publication: 2024 Dec 12.
DOI: 10.1111/jpc.16744
Abstrakt: 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.
(© 2024 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)
Databáze: MEDLINE
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