Gonadal Function Abnormalities in Sickle Cell Anemia

Abbasi, Ali; Prasad, Ananda S.; Ortega, Jesús; Congco, Evangeline; Oberleas, Donald

doi:10.7326/0003-4819-85-5-601

Cited by 154 publications

(78 citation statements)

References 16 publications

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“…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.…”

Section: Fertility In Menmentioning

confidence: 99%

Reproductive issues in sickle cell disease

Smith‐Whitley

2014

Blood

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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. (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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Section: Fertility In Menmentioning

confidence: 99%

Reproductive issues in sickle cell disease

Smith‐Whitley

2014

Blood

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“…The possible pathophysiologic mechanisms of hypogonadism are disruption in the hypothalamic-pituitary-gonadal axis, which leads to primary testicular failure [16,17], or secondary hypothalamic-pituitary dysfunction [14,15,18,19].…”

Section: Hypogonadismmentioning

confidence: 99%

Infertility Issues in Men with Sickle Cell Disease

AlDallal

2017

IPCB

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Patients with sickle cell disease (SCD), especially males, face a key problem in the form of infertility. Apart from low serum testosterone, abnormalities involving accessory sex organs are also observed in these patients. Infertility is a known complication among males with SCD, and it comes about due to reasons such as impotence, relative primary gonadal failure, priapism, and delayed or impaired sexual development. Infertility manifests due to multiple causes, including primary gonadal failure (hypogonadism), erectile dysfunction (ED) as a result of priapism, sperm abnormalities, and delayed or impaired sexual development. The ejaculate volume, sperm density, sperm motility, and normal sperm morphology were significantly reduced in these patients as compared with normal patients. Most importantly, primary testicular failure is marked by low levels of testosterone and infertility results mainly from diseases or conditions that affect and destroy the testis. The low levels of testosterone lead to fertility reduction, which is aggravated by impotence, secondary to earlier priapism. Studies among mice have shown that increased doses of HU increase testicular germ cell apoptosis, decrease sperm count, induce testicular atrophy, increase abnormal sperm morphology, and decrease sperm motility. A multifactorial etiology is responsible for impaired male fertility and it includes sperm abnormalities, ED, hypogonadism, and effect of therapy on sperm function. This paper summarizes some clinical manifestations which play an important role in the development of infertility among men with SCD. The author suggests that prospective studies among males with SCD should be carried out using various end points to determine cellular and functional impairment in fertility.

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“…Men with sickle cell disease generally have a smaller ejaculate volume, poorer sperm motility, reduced sperm density, and fewer spermatozoa with normal morphology [89]. Most importantly, such patients are often diagnosed with primary testicular failure, characterized by low levels of testosterone [91][92][93][94][95], aggravated by impotence secondary to earlier priapism [92]. Interestingly hydroxyurea, a drug used to manage sickle cell anemia, is detrimental to male reproduction.…”

Section: Male Reproductive Complications Associated With Iron and Copmentioning

confidence: 99%

“…This has been attributed to a relative primary gonadal failure and a delayed or impaired sexual development [90,91]. Men with sickle cell disease generally have a smaller ejaculate volume, poorer sperm motility, reduced sperm density, and fewer spermatozoa with normal morphology [89].…”

Section: Male Reproductive Complications Associated With Iron and Copmentioning

confidence: 99%

Iron and copper in male reproduction: a double-edged sword

Tvrdá

Peer

Sikka

et al. 2014

J Assist Reprod Genet

153

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Iron and copper are essential trace nutrients playing important roles in general health and fertility. However, both elements are highly toxic when accumulating in large quantities. Their direct or indirect impact on the structure and function of male gonads and gametes is not completely understood yet. Excess or deficiency of either element may lead to defective spermatogenesis, reduced libido, and oxidative damage to the testicular tissue and spermatozoa, ultimately leading to fertility impairment. This review will detail the complex information currently available on the dual roles iron and copper play in male reproduction.

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Gonadal Function Abnormalities in Sickle Cell Anemia

Cited by 154 publications

References 16 publications

Reproductive issues in sickle cell disease

Reproductive issues in sickle cell disease

Infertility Issues in Men with Sickle Cell Disease

Iron and copper in male reproduction: a double-edged sword

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