Endocrine functions in Sickle Cell Anaemia Patients

El‐Hazmi, Mohsen A. F.; Bahakim, Hassan M.; Al-Fawaz, I.

doi:10.1093/tropej/38.6.307

Cited by 59 publications

(47 citation statements)

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“…86 In 80 Saudi patients with sickle cell anaemia, hormonal assay showed normal levels of T3, T4 and growth hormone, low levels of cortisol, testosterone and LH, and variable changes in FSH. 87 These abnormalities occurred more frequently in the patients with severe disease. Studies of thyroid function have shown that blood levels of thyroxine, thyroxine-binding capacity and the free thyroxine index were not significantly different in 90 SS children (1-15 yrs) than in AS and AA controls.…”

Section: Endocrine Dysfunction and Growth Retardationmentioning

confidence: 94%

Growth and nutritional status of children with homozygous sickle cell disease

Al-Saqladi

Cipolotti

Fijnvandraat

et al. 2008

Annals of Tropical Paediatrics

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Background: Poor growth and under-nutrition are common in children with sickle cell disease (SCD). This review summarises evidence of nutritional status in children with SCD in relation to anthropometric status, disease severity, body composition, energy metabolism, micronutrient deficiency and endocrine dysfunction. Methods: A literature search was conducted on the Medline/PUBMED, SCOPUS, SciELO and LILACS databases to July 2007 using the keywords sickle cell combined with nutrition, anthropometry, growth, height and weight, body mass index, and specific named micronutrients. Results: Forty-six studies (26 cross-sectional and 20 longitudinal) were included in the final anthropometric analysis. Fourteen of the longitudinal studies were conducted in North America, the Caribbean or Europe, representing 78.8% (2086/2645) of patients. Most studies were observational with wide variations in sample size and selection of reference growth data, which limited comparability. There was a paucity of studies from Africa and the Arabian Peninsula, highlighting a large knowledge gap for low-resource settings. There was a consistent pattern of growth failure among affected children from all geographic areas, with good evidence linking growth failure to endocrine dysfunction, metabolic derangement and specific nutrient deficiencies. Conclusions: The monitoring of growth and nutritional status in children with SCD is an essential requirement for comprehensive care, facilitating early diagnosis of growth failure and nutritional intervention. Randomised controlled trials are necessary to assess the potential benefits of nutritional interventions in relation to growth, nutritional status and the pathophysiology of the disease.

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Section: Endocrine Dysfunction and Growth Retardationmentioning

confidence: 94%

Growth and nutritional status of children with homozygous sickle cell disease

Al-Saqladi

Cipolotti

Fijnvandraat

et al. 2008

Annals of Tropical Paediatrics

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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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“…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%