Assessment of glucose homeostasis in young adult female β-thalassemia major patients (β-TM) with acquired hypogonadotropic hypogonadism (AHH) never treated with sex steroids compared to eugonadal β-TM patients with spontaneous menstrual cycles: OGTT in female β-thalassemia major patients with AHH

Vincenzo De Sanctis; Shahina Daar; Ashraf T Soliman; Ploutarchos Tzoulis; Salvatore Di Maio; Christos Kattamis

doi:10.23750/abm.v94i3.14147

Assessment of glucose homeostasis in young adult female β-thalassemia major patients (β-TM) with acquired hypogonadotropic hypogonadism (AHH) never treated with sex steroids compared to eugonadal β-TM patients with spontaneous menstrual cycles

OGTT in female β-thalassemia major patients with AHH

Authors

Vincenzo De Sanctis Quisisana Hospital, Ferrara https://orcid.org/0000-0002-6131-974X
Shahina Daar Department of Haematology, College of Medicine and Health Sciences, Sultan Qaboos University, Sultanate of Oman
Ashraf T Soliman Department of Pediatrics, Division of Endocrinology, Hamad General Hospital, Doha, Qatar and Department of Pediatrics, Division of Endocrinology, Alexandria University Children’s Hospital, Alexandria, Egypt
Ploutarchos Tzoulis Department of Diabetes & Endocrinology, Whittington Hospital, University College London, London, UK
Salvatore Di Maio Emeritus Director in Pediatrics, Children’s Hospital “Santobono-Pausilipon”, Naples, Italy
Christos Kattamis First Department of Paediatrics, National Kapodistrian University of Athens, Greece

DOI: https://doi.org/10.23750/abm.v94i3.14147

Keywords:

acquired hypogonadotropic hypogonadism, β-thalassemia major, glucose tolerance, insulin sensitivity, β-cell secretion

Abstract

Background: Acquired ypogonadotropic hypogonadism (AHH) is the most prevalent endocrine complication in thalassemia major (TM). Study design: Considering the detrimental effect of estrogen deficiency on glucose metabolism, the ICET-A Network promoted a retrospective study on the long-term effects of estrogen deficiency on glucose homeostasis in female β-TM patients with HH without hormonal replacement therapy (HRT). Patients and Methods: Seventeen β-TM patients with AHH (4 had arrested puberty; Tanners' breast stage 2-3), never treated with sex steroids, and 11 eugonadal β-TM patients with spontaneous menstrual cycles at the time of referral were studied. A standard 3-h OGTT was performed in the morning, after an overnight fast. Six-point plasma glucose and insulin level determinations, indices of insulin secretion and sensitivity, early-phase insulin insulinogenic index (IGI), HOMA-IR and β-cell function (HOMA-β), oral disposition index (oDI), glucose and insulin areas under the OGTT curves were evaluated. Results: Abnormal glucose tolerance (AGT) or diabetes was observed in 15 (88.2%) of 17 patients with AHH and 6 (54.5%) of 11 patients with eumenorrhea. The difference between the two groups was statistically significant (P: 0.048). However, the group of eugonadal patients was younger compared to AHH patients (26.5 ± 4.8 years vs. 32.6 ± 6.2 years ; P: 0.010). Advanced age, severity of iron overload, splenectomy, increased ALT levels and reduced IGF-1 levels were the main clinical and laboratory risk factors for glucose dysregulation observed in β-TM with AHH compared to eugonadal β-TM patients with spontaneous menstrual cycles. Conclusion: These data further support the indication for an annual assessment of OGTT in patients with β-TM. We believe that a registry of subjects with hypogonadism is necessary for a better understanding of the long-term consequences of this condition and refining treatment options.

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