ACQUIRED HYPOGONADOTROPIC HYPOGONADISM (AHH) IN THALASSAEMIA MAJOR PATIENTS: AN UNDERDIAGNOSED CONDITION?

摘要: Introduction In males, acquired hypogonadotropic hypogonadism (AHH) includes all disorders that damage or alter the function of gonadotropin-releasing hormone (GnRH) neurons and/or pituitary gonadotroph cells. The clinical characteristics AHH are androgen deficiency and a lack, delay halt pubertal sexual maturation. lead to decreased libido, impaired erectile strength, worsened sense well being degraded quality life (QOL). Patients methods We studied 11 adult men with thalassemia major (TM) aged between 26 54 years (mean ± SD: 34.3 8.8 years) AHH. Twelve age- sex-matched TM patients normal development were used as control group. All on regular transfusions iron chelation therapy. Fasting venous blood samples collected two weeks after  transfusion measure serum concentrations IGF-1, free thyroxine (FT4), thyrotropin (TSH), cortisol,  luteinizing (LH), follicle stimulating (FSH), total testosterone (TT), prolactin estradiol (E2), glucose, urea, creatinine electrolytes (including calcium phosphate). Liver functions screening for hepatitis C virus seropositivity (HCVab HCV-RNA) performed. Iron status was assessed by measuring ferritin levels, evaluation in liver (LIC) heart using MRI- T2*.   Bone mineral density measured at lumbar spine (L1-L4) dual energy X-ray absorptiometry (DXA) Hologic QDR 4000 machine. Results mean basal LH FSH 2.4 2.2 IU/L  1.2 0.9 IU/L respectively; these, values significantly lower compared Semen analysis 5 proved azoospermia 3  oligoasthenozoospermia 2. percentage level >2000 ng/ml higher controls (45.4 % versus 8.3%, p: 0.043). Heart (T2* values) (p: 0.004). Magnetic resonance imaging azoospermic revealed volume loss reduction signal intensity.  Using DXA, 63.6 (7/11) osteoporotic 36.3 (4/11) osteopenic. Conclusions our thalassemic overload chronic disease appear play role AHH.  Treatment is an important vibrant field improving their health QOL. Early identification management very crucial avoid long-term morbidity, including dysfunction infertility. Therapy aims restore mid–normal range. Many exciting opportunities remain further research therapeutic development. Key words: Acquired hypogonadism, thalassemia, overload, disease.