Andrology: Pituitary-testicular axis in men with  -thalassaemia major

Papadimas, J.; Μανδαλά, Ευδοκία; Pados, George; Kokkas, B.; Georgiadis, George S.; Tarlatzis, Basil C.; Bontis, J.; Sinakos, Z; Mantalenakis, S.

doi:10.1093/oxfordjournals.humrep.a019515

Cited by 20 publications

(14 citation statements)

References 7 publications

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“…However, only 74% of patients developed primary or secondary amenorrhea 10 . In a previous study of our group, 40% of male patients with â-thal were found to be hypogonadal 11 .…”

Section: Discussionmentioning

confidence: 56%

β-thalassemia and gonadal axis: a cross-sectional, clinical study in a Greek population

Papadimas¹,

Goulis²,

Μανδαλά³

et al. 2002

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beta-thalassemia (beta-thal) is characterized by disturbances of the reproductive system. The aim of the present study was: 1) to assess the hypothalamic- pituitary-gonadal axis in patients with beta-thal in relation to their phenotype and 2) to determine prognostic features of current gonadal status. We studied 135 patients (67 males and 68 females) with beta-thal through history, physical examination, spermiograms and GnRH test. These patients were divided into beta-thal major (51 males and 62 females) and beta-thal intermedia phenotypes (16 males and 6 females). Male patients with beta-thal major were subdivided into three groups a) eugonadal (35%, Tanner's stage V, normal testicular volume, normal spermiograms, normal basal and stimulated hormone values), b) patients with hypogonadotrophic hypogonadism (HH) of late onset (24%, Tanner's stage II-V, low-normal testicular volume, abnormal spermiograms, normal basal gonadotrophin values and abnormal response to GnRH test) and c) patients with HH of early onset (41%, Tanner's stage I, small testicular volume, abnormal spermiograms, abnormal basal and stimulated hormone values). Female patients with beta-thal major were subdivided into: a) eugonadal (32%, Tanner's stage V, regular menstruation, normal basal and stimulated hormone values), b) patients with hypogonadotrophic hypogonadism (HH) of late onset (34%, Tanner's stage II-V, secondary amenorrhea, subnormal basal and stimulated gonadotrophin values) and c) patients with HH of early onset (34%, Tanner's stage I, primary amenorrhea, subnormal basal and stimulated hormone values). Patients with beta-thal intermedia were subdivided into eugonadal (75% of males, 33% of females) and hypogonadal (25% of males, 67% of females). Current gonadal status could not be predicted by means of transfusion or chelation parameters. In conclusion, beta-thal patients could be eugonadal or develop early or late onset HH. trade mark-thal intermedia patients have a more favorable profile than beta-thal major individuals. Current gonadal status of beta-thal patients cannot be predicted by means of history, clinical or laboratory parameters.

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“…However, only 74% of patients developed primary or secondary amenorrhea 10 . In a previous study of our group, 40% of male patients with â-thal were found to be hypogonadal 11 .…”

Section: Discussionmentioning

confidence: 56%

β-thalassemia and gonadal axis: a cross-sectional, clinical study in a Greek population

Papadimas¹,

Goulis²,

Μανδαλά³

et al. 2002

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“…Bronspiegel-Weintrob et al [13] reported that patients with abnormal puberty had higher serum ferritin levels before chelation and had received suboptimal chelation therapy (serum ferritin levels 4,734 ± 3,081 and 3,572 ± 1,870 ng/mL, respectively). Important factors for developing hypogonadism and/ or delayed puberty are a high serum ferritin level [10][11][12]. However, this study did not show such a relationship because there was no delay in puberty.…”

Section: Discussionmentioning

confidence: 60%

“…Prevalence is 40%−80% [5][6][7][8][9]. Iron overload [10][11][12] is considered one of the main factors for developing endocrine complications.…”

Section: Brief Communication (Original)mentioning

confidence: 99%

Testicular function in patients with regular blood transfusion for thalassemia major

et al. 2015

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Background: Regular blood transfusion and iron chelation therapy have improved the quality of life of patients with thalassemia and increased their longevity, but transfusion also increases the frequency of endocrine complications, possibly because of iron deposition in the pituitary gland or the gonads, or both. Objective: To evaluate testicular function in patients with thalassemia major by basal hormonal study, and identify risk factors for dysfunction. Methods: We performed a cross-sectional study of 28 patients with thalassemia major aged 11.7 ± 1.8 (8−14.9) years (15 in prepuberty, 13 in puberty with no delayed puberty) who had regular blood transfusions. A normal control group comprised 64 boys who were matched for age and Tanner genital stage. Results: The mean level of serum ferritin in the previous year was 1,575 ± 642 ng/mL, and the onset of blood transfusion was at 3.8 ± 2.3 years and iron chelation therapy was 6.6 ± 2.8 years. The trend for anti-Müllerian hormone levels in patients and controls was similar with age, and although higher in the patients, particularly at Tanner stage II, was not significantly different. Testosterone levels were lower in the patients compared with controls; particularly at Tanner stages IV-V (290.88 vs. 537.4 ng/dL, P < 0.05). Serum follicle-stimulating hormone and luteinizing hormone levels were not significantly different between the groups at any Tanner stage. Conclusion: Patients who received regular blood transfusions had normal Sertoli cell function. Leydig cell dysfunction may occur, even though the patients had a normal pubertal onset.

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“…This endocrine disturbance is becoming less frequent nowadays with early and intensive chelation therapy. [17] The prevalence of various endocrine complications shows a difference between centers, particularly for GH deficiency, hypoparathyroidism, and hypothyroidism. [18] In the present study, the prevalence of GH deficiency in thalassemia patients was 35.9% as compared with a prevalence of 7.9% in males in a previous study[18] and up to 25% in another study.…”

Section: Discussionmentioning

confidence: 99%

Study of the effect of iron overload on the function of endocrine glands in male thalassemia patients

Abdulzahra¹,

Al‐Hakeim²,

Ridha³

2011

Asian J Transfus Sci

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Background:Iron overload is an important issue in the state of thalassemic patients due to the harmful effect of high concentration of iron deposited in different tissues in human body including endocrine glands. In the present work, an attempt is carried out to estimate the effect of iron overload in thalassemic patients on the function of endocrine glands through the estimation of their ability to secrete adequate amounts of certain hormones.Materials and Methods:Seventy eight male children with beta-thalassemia, in the age-group of 4–11 years, were enrolled for this research. These children were being treated with frequent transfusions and long-term iron chelation therapy. Thirty age and sex matched children without thalassemia constituted the control group. Ferritin and different hormones were estimated by ELISA technique.Results:The results showed a mild reduction in the function of endocrine glands through the decrease in the level of some hormones. These changes due mainly to the hypoxia and precipitation of iron in certain glands and overlapping with the synthesis or secretion of the hormones.Conclusion:There is a different hormonal disturbances in beta thalassemia patients. Reduction of total body iron store is an important goal of the treatment of thalassemia and measuring the hormones concentration is necessary for the follow up of the thalassemic patients especially during puberty.

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Andrology: Pituitary-testicular axis in men with -thalassaemia major

Cited by 20 publications

References 7 publications

β-thalassemia and gonadal axis: a cross-sectional, clinical study in a Greek population

β-thalassemia and gonadal axis: a cross-sectional, clinical study in a Greek population

Testicular function in patients with regular blood transfusion for thalassemia major

Study of the effect of iron overload on the function of endocrine glands in male thalassemia patients

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