Repeated testicular infarction in a patient with sickle cell disease: a possible mechanism for testicular failure

Li, Maomi; Fogarty, James D.; Whitney, Kathleen D.; Stone, Peter

doi:10.1016/s0090-4295(03)00482-5

“…Adolescents and adults with SCD may experience a wide array of reproductive issues including delayed puberty, hypogonadism, infertility, and pregnancy complications [118][119][120][121]. In men with SCD, vaso-occlusion and recurrent testicular infarction may contribute to hypogonadism [122,123]. Erectile dysfunction has been reported in as many as 21% to 35% and is likely a sequela of recurrent episodes of priapism [124,125].…”

Section: Reproductive Health In Adults With Scdmentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Chaturvedi

¹

,

DeBaun

²

2015

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Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

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“…The theory regarding vasoocclusion of testicular vessels is interesting given reports of recurrent testicular infarction in individuals with SCD. 11 Therapies to alleviate the symptoms of hypogonadism have been limited. Testosterone undecanoate injections 12 and clomiphene 13 have been used with variable results.…”

Section: Fertility In Menmentioning

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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“…The theory regarding vasoocclusion of testicular vessels is interesting given reports of recurrent testicular infarction in individuals with SCD. 11 Therapies to alleviate the symptoms of hypogonadism have been limited. Testosterone undecanoate injections 12 and clomiphene 13 have been used with variable results.…”

Section: Fertility In Menmentioning

confidence: 99%

Reproductive issues in sickle cell disease

Smith‐Whitley

¹

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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Repeated testicular infarction in a patient with sickle cell disease: a possible mechanism for testicular failure

Cited by 48 publications

References 14 publications

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Reproductive issues in sickle cell disease

Reproductive issues in sickle cell disease

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