Testicular dysfunction in men with sickle cell disease

Osegbe, D N; Akinyanju, O. O.

doi:10.1136/pgmj.63.736.95

“…17 Sperm abnormalities are not always related to impaired fertility in men with SCD. 5 Although subjects with sperm abnormalities report fathering children, 5 more sophisticated, prospective studies are needed to determine the clinical importance of abnormal sperm analysis and its impact on male fertility in SCD.…”

Section: Sperm Abnormalitiesmentioning

confidence: 99%

Reproductive issues in sickle cell disease

Smith‐Whitley

¹

2014

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As medical advances improve survival, reduce disease-related morbidity, and improve quality of life, reproductive issues will take higher priority in the sickle cell disease (SCD) community. A wide variety of topics are addressed in this chapter, including fertility, gonadal failure, erectile dysfunction, and menstrual issues in SCD. Etiologies of impaired male fertility are multifactorial and include hypogonadism, erectile dysfunction, sperm abnormalities, and complications of medical therapies. Much less is known about the prevalence and etiology of infertility in women with SCD. Other reproductive issues in women included in this review are pain and the menstrual cycle, contraception, and preconception counseling. Finally, long-term therapies for SCD and their impact on fertility are presented. Transfusional iron overload and gonadal failure are addressed, followed by options for fertility preservation after stem cell transplantation. Focus is placed on hydroxyurea therapy given its benefits and increasing use in SCD. The impact of this agent on spermatogenesis, azoospermia, and the developing fetus is discussed. Learning Objectives• To have a better understanding of hypogonadism, sperm abnormalities, and ED in men with SCD • To recognize the need for contraception counseling for patients with SCD and provide recommendations for hormonal contraceptives in women • To describe fertility preservation options for children and adults with SCD considering stem cell transplantation

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“…17 Sperm abnormalities are not always related to impaired fertility in men with SCD. 5 Although subjects with sperm abnormalities report fathering children, 5 more sophisticated, prospective studies are needed to determine the clinical importance of abnormal sperm analysis and its impact on male fertility in SCD.…”

Section: Sperm Abnormalitiesmentioning

confidence: 99%

“…4 Possible underlying pathophysiologic mechanisms of hypogonadism include disruptions in the hypothalamic-pituitary-gonadal axis leading to primary testicular failure. However, studies are inconsistent as to whether primary testicular failure 5,6 or secondary hypothalamic-pituitary dysfunction 3,4,[7][8][9] is the cause. A recent report found low serum testosterone levels in 8 of 34 men with SCD and all 8 had low FSH and LH levels, suggesting a central mechanism.…”

mentioning

confidence: 99%

Reproductive issues in sickle cell disease

Smith‐Whitley

¹

2014

Blood

View full text Add to dashboard Cite

As medical advances improve survival, reduce disease-related morbidity, and improve quality of life, reproductive issues will take higher priority in the sickle cell disease (SCD) community. A wide variety of topics are addressed in this chapter, including fertility, gonadal failure, erectile dysfunction, and menstrual issues in SCD. Etiologies of impaired male fertility are multifactorial and include hypogonadism, erectile dysfunction, sperm abnormalities, and complications of medical therapies. Much less is known about the prevalence and etiology of infertility in women with SCD. Other reproductive issues in women included in this review are pain and the menstrual cycle, contraception, and preconception counseling. Finally, long-term therapies for SCD and their impact on fertility are presented.Transfusional iron overload and gonadal failure are addressed, followed by options for fertility preservation after stem cell transplantation. Focus is placed on hydroxyurea therapy given its benefits and increasing use in SCD. The impact of this agent on spermatogenesis, azoospermia, and the developing fetus is discussed. (Blood. 2014;124(24):3538-3543) IntroductionReproductive issues in women and men with sickle cell disease (SCD) include a wide array of complications that are relatively common; however, data from well-designed, large clinical studies are limited. Many studies are quite old, but remain relevant because they describe clinical complications and problems that persist in the SCD population today despite advances in medical therapy. Not unexpectedly, some of the reproductive issues in SCD arise due to chronic medical therapies that are used increasingly to prevent or manage SCD-related morbidity. Fertility in menInfertility in men with SCD has been studied more frequently than infertility in women and appears to have multiple causes, including hypogonadism, sperm abnormalities, and erectile dysfunction (ED) due to priapism. Although a delay in sexual maturation of 1.5-2 years, on average, occurs in adolescents and young adults with SCD, 1,2 most go on to have normal sexual maturation. However, up to 24% of men with SCD may develop hypogonadism, a clinical syndrome associated with poor testosterone production, infertility, ED, and poor libido.3 Clinical characteristics include sparse facial, pubic, and axillary hair and small testicular size. Clinical laboratory findings are low testosterone levels with variable follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels. 4 Possible underlying pathophysiologic mechanisms of hypogonadism include disruptions in the hypothalamic-pituitary-gonadal axis leading to primary testicular failure. However, studies are inconsistent as to whether primary testicular failure 5,6 or secondary hypothalamic-pituitary dysfunction 3,4,[7][8][9] is the cause. A recent report found low serum testosterone levels in 8 of 34 men with SCD and all 8 had low FSH and LH levels, suggesting a central mechanism.3 Multiple theories as to why these abnormalities develop in ma...

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“…8 Finally, 72 to 100% of the patients with sickle cell disease had an impairment of at least one sperm parameter. Patients with sickle cell disease often have moderate to severe hypogonadism, [9][10][11] of unknown origin, although several mechanisms have been suggested: primary hypogonadism, 9,12 hypogonadism induced by repeated testicular infarction, 13 zinc deficiency, 14,15 and puberty delay due to span-height retardation. [16][17][18] Hydroxyurea itself has been reported to impair spermatogenesis in mammals, resulting in testicular atrophy, 19-21 a reversible decrease in sperm count, [19][20][21][22][23] and abnormal sperm morphology 20,24 and motility.…”

Section: Introductionmentioning

confidence: 99%