Gonadal dysfunction in adult male patients with thalassemia major: an update for clinicians caring for thalassemia

Sanctis, Vincenzo De; Soliman, Ashraf T; Elsedfy, Heba; Maio, Salvatore Di; Canatan, Duran; Soliman, Nada; Karimi, Mehran; Kattamis, Christos

doi:10.1080/17474086.2017.1398080

Cited by 28 publications

(30 citation statements)

References 66 publications

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“…After that, 433 records were excluded, and 213 records entered full-text assessment for eligibility criteria. Finally, 74 studies included in the meta-analysis ( Figure 1 ) [ 6 7 11 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 ].…”

Section: Resultsmentioning

confidence: 99%

A Systematic Review and Meta-Analysis of Stature Growth Complications in β-thalassemia Major Patients

Arab‐Zozani

Kheyrandish

Rastgar

et al. 2021

Annals of Global Health

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Background: Blood transfusion is a traditional treatment for β-thalassemia (β-thal) that improves the patients’ anemia and lifespan, but it may lead to iron overload in parenchymal tissue organs and endocrine glands that cause their dysfunctions as the iron regulatory system can’t excrete excess iron from the bloodstream. Objective: To evaluate the prevalence of iron-related complications (short stature, growth retardation, and growth hormone deficiency) in β-thalassemia major ( β TM) patients. Methods: We performed an electronic search in PubMed, Scopus, and Web of Sciences to evaluate the prevalence of growth hormone impairment in β-thalassemia major ( β TM) patients worldwide. Qualities of eligible studies were assessed by the Joanna Briggs Institute checklist for the prevalence study. We used Comprehensive Meta-Analysis (Version 2) to calculate the event rate with 95% CIs, using a random-effects model for all analyses. Findings: Seventy–four studies were included from five continents between 1978 and 2019; 70.27% (Asia), 16.21% (Europe), 6.75% (Africa), 2.70% (America), 1.35% (Oceania), and 2.70% (Multicenter). The overall mean age of the participants was about 14 years. The pooled prevalence of short stature (ST) was 48.9% (95% CI 35.3–62.6) and in male was higher than female (61.9%, 95% CI 53.4–69.7 vs. 50.9%, CI 41.8–59.9). The pooled prevalence of growth retardation (GR) was 41.1% and in male was higher than in female (51.6%, 95% CI 17.8–84 vs. 33.1%, CI 9.4–70.2). The pooled prevalence of growth hormone deficiency (GHD) was 26.6% (95% CI 16–40.8). Conclusion: Our study revealed that near half of thalassemia patients suffer from growth impairments. However, regular evaluation of serum ferritin levels, close monitoring in a proper institute, suitable and acceptable treatment methods besides regular chelation therapy could significantly reduce the patients’ complications.

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Section: Resultsmentioning

confidence: 99%

A Systematic Review and Meta-Analysis of Stature Growth Complications in β-thalassemia Major Patients

Arab‐Zozani

Kheyrandish

Rastgar

et al. 2021

Annals of Global Health

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“…As it turns out, ferroportin is expressed on Sertoli cells, which is present only on cells substantially involved in maintaining adequate serum iron levels [62,63]. Besides, it has been also established that there is a significant iron deposition in the testis of patients with transfusion-dependent beta-thalassemia and such findings might explain the high prevalence of impaired fertility in male patients, even though the pituitary function seems not to be decreased [64,65].…”

Section: Ironmentioning

confidence: 99%

An impact of selected micronutrients on fertility

Żak

Leziak

Niedobylski

et al. 2020

J Educ Health Sport

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Introduction and purpose: The problem with infertility affects between 8 and 12% of reproductive-aged couples. Among the reasons, smoking, adulterated food, hormonal disorder are featured. Our purpose is to check how micronutrients impact fertility. Brief description of the state of knowledge: Micronutrients deficiency, specifically zinc and selenium deficiencies have also unpropitious impact on fertility. In this review, the potential role of these microelements in male and female reproductive health is considered. Zinc had been proved to regulate sperm motility, spermatogenesis and reduce oxidative stress. Lower delivery of selenium decrease testosterone level by causing changes in receptor of the luteinising hormone. It is also compared with lower spermatozoa concentration. In this review the role of iron, manganese, lead, nickel, copper, magnesium and cobalt is described as well. Summary: Deficiencies of some micronutrients may make worse the fertility. That's the reason why additional supplementation may be important in the treatment of infertility.

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“…They found no cases and 26.3% of controls had attained menarche in the age group of 8-12 years whereas 11.4% of cases and 93.3% of controls in the age group of >12 years had attained menarche. Hypogonadotropic hypogonadism is the most frequent endocrinopathy in patients with transfusion-dependent thalassemia affecting 70-80% of all thalassemia patients [25]. This complication is largely explained by toxic effect of iron overload secondary to chronic blood transfusions because the human body lacks a mechanism to excrete excess iron [26].…”

Section: Discussionmentioning

confidence: 99%

Magnetic Resonance Imaging Versus Serum Iron Status as Diagnostic Tools for Pituitary Iron Overload in Children with Beta Thalassemia

Morad

El-Shanshory

Hablas

et al. 2021

JAMMR

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Background: Thalassemia is a common genetic disorder associated with endocrine disorders. Iron deposition may start in the anterior pituitary gland, but clinical signs are usually not evident until puberty. Aim of the study was to evaluate pituitary iron overload in children with β thalassemia using MRI T2* and correlate MRI T2* and biochemical markers of iron overload with pituitary hormones. Patients and Methods: This study was carried out on 30 children with β-thalassemia major (19 females and 11 males ) with their age ranging from 10 - 18 years and mean age value of 12.8 ± 2.4 and 30 healthy children of matched age and sex as controls in the period from September 2018 to September 2019. For all patients the following were done: complete clinical evaluation including anthropometric data and Tanner staging, laboratory investigations including serum iron status, thyroid function, basal and growth hormone provocation test by clonidine, Follicle stimulating hormone (FSH) , Luteinizing hormone (LH) , and pituitary MRI T2*. Results: Weight, Z score of weight, height, Z score of height and Body mass index (BMI) were found significantly lower in patients compared with controls. Patients had delayed puberty compared with controls. Pituitary MRI T2* was found significantly lower in patients compared with controls (P=0.001). FSH, LH, and provocative growth hormone levels were found significantly lower in patients compared to controls (p <0.001). Serum ferritin & iron were found significantly higher in patients than controls (P<0.001). Significant negative correlations were found between serum ferritin and Weight, Height, Z score of Weight, Z score of Height, BMI and pituitary hormones. Significant negative correlations were found between Pituitary MRI T2* and serum ferritin. Significant positive correlations were found between Pituitary MRI T2* and Weight, Height, Z score of Weight, Z score of Height and pituitary hormons. The pituitary T2* carried the sensitivity of 80% ,100% and 100% and specificity of 70% ,83.3% and 100% for predicting GH hormone abnormality, LH and FSH respecetively. Conclusions: There were a significant positive correlations between pituitary MRI T2*and anthropometric measurement and pituitary hormones of studied patients. There was a significant negative correlation between Pituitary T2* and serum ferritin. Pituitary iron overload can be detected by MRI T2* which is a diagnostic tool in detecting pituitary iron overload with subsequent effect on hormonal secretion especially growth hormone. Recommendations: Children with thalassemia should undergo meticulous follow up regarding regular blood transfusion, regular iron chelators in a proper manner and doses and routine use of MRI T2* in the evaluation of pituitary iron overload before irreversible damage occur in the pituitary gland.

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Gonadal dysfunction in adult male patients with thalassemia major: an update for clinicians caring for thalassemia

Cited by 28 publications

References 66 publications

A Systematic Review and Meta-Analysis of Stature Growth Complications in β-thalassemia Major Patients

A Systematic Review and Meta-Analysis of Stature Growth Complications in β-thalassemia Major Patients

An impact of selected micronutrients on fertility

Magnetic Resonance Imaging Versus Serum Iron Status as Diagnostic Tools for Pituitary Iron Overload in Children with Beta Thalassemia

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