Study of gonadal hormones in Egyptian female children with sickle cell anemia in correlation with iron overload

Hagag, Adel A; Elfarargy, Mohamed Shawky; Elrefaey, Shaymaa; El-Enein, Amany M Abo

doi:10.1016/j.hemonc.2015.11.005

Cited by 13 publications

(10 citation statements)

References 33 publications

(14 reference statements)

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“…Another important reason for infertility is hypogonadotrophic hypogonadism due to deposition of iron in the hypothalamo-pituitary axis because of multiple blood transfusions and iron overload. A single-center study in Egypt demonstrated that adolescents with SCD and excessive iron stores have significantly lower levels of follicle stimulating hormone (FSH), luteinizing hormone (LH), and estrogen when compared to those without excessive iron stores 8…”

Section: Methodsmentioning

confidence: 99%

Sickle Cell Disease and Pregnancy

Jain

Lodha

Colah

et al. 2019

Mediterr J Hematol Infect Dis

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Abstract Sickle Cell Disease (SCD) is a group of inherited single-gene autosomal recessive disorders caused by the ‘sickle’ gene, which affects haemoglobin structure. Sickle cell anemia is the most common hemoglobinopathy worldwide. The burden of sickle cell disease in pregnancy has been exponentially increasing with more number of women reaching the reproductive age, and having successful pregnancies. It has been proven beyond doubt that SCD in pregnancy poses the pregnant woman and fetus to significantly higher risks than a lady without SCD. SCD is associated with both maternal and fetal complications and is associated with an increased incidence of perinatal mortality, premature labour, fetal growth restriction and acute painful crises during pregnancy. Some studies also describe an increase in spontaneous miscarriage, antenatal hospitalisation, maternal mortality, delivery by caesarean section, infection, thromboembolic events and antepartum haemorrhage. This review aims to discuss the risks of SCD in pregnancy - to the mother and fetus . It also reviews the difference between complications in SCD and sickle cell trait.

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Section: Methodsmentioning

confidence: 99%

Sickle Cell Disease and Pregnancy

Jain

Lodha

Colah

et al. 2019

Mediterr J Hematol Infect Dis

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“…However, AMH levels were inversely correlated with non‐transferrin‐bound iron and labile plasma iron levels, and were lower in women over 30 years old, suggesting that iron has a long‐term gonadotoxic effect . Even with iron level normalization, this process is irreversible …”

Section: Benign Hematological Diseasesmentioning

confidence: 99%

“…15 Even with iron level normalization, this process is irreversible. 16 Finally, ovarian damage and inflammatory mediators during ischemia in vaso-occlusive crises have been proposed to be among the disrupting factors that may also explain premature ovarian aging in these women. 15 Hydroxyurea is a standard long-term treatment administered to patients with sickle cell disease or myeloproliferative diseases.…”

Section: B Eni G N Hematolog I C Al D Is E a S E Smentioning

confidence: 99%

Challenges of fertility preservation in non‐oncological diseases

Condorelli

Demeestere

2019

Acta Obstet Gynecol Scand

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Clinicians should provide fertility counseling to all patients receiving gonadotoxic treatment. International scientific societies have mainly focused on oncological patients, and fewer efforts have been made to apply these recommendations to women diagnosed with benign disease (eg benign hematological diseases, autoimmune diseases, and gynecological or genetic disorders). However, these indications account for 8%‐13% of the demand for fertility preservation. The risk of premature ovarian failure due to treatment, or to the disease itself, can be considered fairly high for many young women. Counseling and adequate management of these women require particular attention due to the severe health conditions that are associated with some of these diseases. In this review, we address specific issues related to providing adequate fertility counseling and management for women who have been diagnosed with the major non‐oncological indications, based on the literature and on our clinical experience.

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“…Endocrine disorders appear to be related to vaso-occlusive and ischemic events, rather than iron overload resulting from frequent transfusion [9]. According to the literature, the prevalence of endocrine and metabolic disorders in children with SCD varies in different populations depending on the literacy rate, socioeconomic status, and access to appropriate treatment [7, 9, 10, 12]. In children with HbSS, the treatment with HU has been demonstrated to allow growth rates similar to patients with HbSβ + or healthy controls [13].…”

Section: Introductionmentioning

confidence: 99%

Endocrine and metabolic complications in children and adolescents with Sickle Cell Disease: an Italian cohort study

et al. 2019

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BackgroundChildren with Sickle Cell Disease (SCD) show endocrine complications and metabolic alterations. The physiopathology of these conditions is not completely understood: iron overload due to chronic transfusions, ischemic damage, and inflammatory state related to vaso-occlusive crises may be involved. Aims of this study were to evaluate the growth pattern, endocrine complications, and metabolic alterations and to detect the relationship between these conditions and the SCD severity in affected children and adolescents.MethodsFifty-two children and adolescents with SCD [38 homozygous sickle hemoglobin (HbSS) and 14 heterozygous sickle hemoglobin (HbSC); age range 3–18 years] were recruited. Anthropometric [height, body mass index (BMI), arm span, sitting height, target height (TH), and pubertal status] and laboratory [blood cell counts, hemolysis indices, metabolic and nutritional status indices and hormonal blood levels] data were evaluated. The SCD severity was defined according to hematological and clinical parameters.ResultsHeight-SDS adjusted for TH and BMI-SDS were significantly higher in HbSC children than in HbSS ones. Forty-eight out of 52 patients (92%) had at least one metabolic and/or endocrine alteration: insufficiency/deficiency of vitamin D (84.7%), insulin resistance (11.5%), growth hormone deficiency (3.8%), subclinical hypothyroidism (3.8%), and hypogonadism (1.9%). Levels of vitamin D were significantly and negatively correlated with clinical indicators of the SCD severity. Subjects with HbSS genotype show significant lower levels of both insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein 3 than children with HbSC. In the study population IGF-1 values were significantly and positively correlated with Hb and negatively with lactate dehydrogenase.ConclusionsMetabolic alterations and endocrine complications are very common in children and adolescents with SCD. A regular follow-up is necessary to identify subjects at risk for complications to precociously start an appropriate treatment and to improve the quality of life of SCD patients.Electronic supplementary materialThe online version of this article (10.1186/s12887-019-1423-9) contains supplementary material, which is available to authorized users.

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Study of gonadal hormones in Egyptian female children with sickle cell anemia in correlation with iron overload

Cited by 13 publications

References 33 publications

Sickle Cell Disease and Pregnancy

Sickle Cell Disease and Pregnancy

Challenges of fertility preservation in non‐oncological diseases

Endocrine and metabolic complications in children and adolescents with Sickle Cell Disease: an Italian cohort study

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