Evaluation of Growth, Puberty and Endocrine Dysfunctions in Relation to Iron Overload in Multi Transfused Indian Thalassemia Patients

Merchant, Rashid; Shirodkar, Amruta; Javed, Ali

doi:10.1007/s12098-010-0351-3

Cited by 30 publications

(26 citation statements)

References 22 publications

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“…As a consequence of inadequate iron chelation, endocrine abnormalities like growth retardation and delayed puberty are well known in Indian thalassemics. 12 The situation was similar in our study at enrolment. What was striking was that, in the Jeevan Asha group, approximately 17.4% patients attained puberty with hormonal induction treatment within nine months, while only 3.8% of total pre-pubertal patients attained puberty in the control group during the same period where none of the patients could afford it.…”

Section: Discussionsupporting

confidence: 83%

Can the Availability of Unrestricted Financial Support Improve the Quality of Care of Thalassemics in a Center with Limited Resources? A Single Center Study from India

Chakrabarti¹,

Bohara²,

Ray

et al. 2012

Thalassemia Reports

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Comprehensive management of thalassemia demands a multidisciplinary approach, sufficient financial resources, carefully developed expertise of the care givers, as well as significant compliance on the patients' part. Studies exploring the utility of unrestricted financing within the existing infrastructure, for the management of thalassemia, particularly in the context of a developing country, are scarce. This study aimed to assess the impact of sponsored comprehensive care compared to the routine care of thalassemics provided at Institute of Haematology and Transfusion Medicine, Kolkata, India. Two hundred and twenty patients were selected for the study and distributed in two arms. Regular monthly follow up was done including a Health Related Quality of Life (HRQoL) assessment with SF 36 v2 (validated Bengali version). Patients receiving sponsored comprehensive care showed a significant improvement in the mean hemoglobin levels and decrease in mean ferritin. HRQoL assessment revealed a better score in the physical domain though the mental health domain score was not significantly better at nine months. Unrestricted financial support in the form of comprehensive care has a positive impact on the thalassemia patients in a developing country not only in terms of clinical parameters but also in health related quality of life.

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Section: Discussionsupporting

confidence: 83%

Can the Availability of Unrestricted Financial Support Improve the Quality of Care of Thalassemics in a Center with Limited Resources? A Single Center Study from India

Chakrabarti¹,

Bohara²,

Ray

et al. 2012

Thalassemia Reports

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“…[3]. Harish et al, (2011); also was reported that minimal siderosis, with reduced number of primordial follicles, and thickened ovarian capsules was noticed in these patients [28].…”

Section: Discussionmentioning

confidence: 84%

“…Anemia and iron overload are responsible for endocrine dysfunction in β-thalassemia [2]. Though meticulous management has prolonged the life expectancy of these patients, they tend to suffer from growth retardation and endocrine dysfunction that are multifactorial in etiology and may be can't prevent by treatment [3]. If regular transfusion program is initiated to maintain the hemoglobin (Hb) level 9 -10 g/dl, normal growth occurs till the age of 10 -12 years old.…”

Section: Introductionmentioning

confidence: 99%

“…If regular transfusion program is initiated to maintain the hemoglobin (Hb) level 9 -10 g/dl, normal growth occurs till the age of 10 -12 years old. It is also important to decrease iron overload by adequate chelation to prevent organ damage from free radical [3]. Blood transfusion and iron-chelation therapy together have improved the quality of life [4].…”

Section: Introductionmentioning

confidence: 99%

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Impact of Ferritin Load on Gonadal Reserve among Regular Transfused β-Thalassemia

Abo-Elwafa¹,

Hamid²,

Heshmat³

et al. 2017

OJBD

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Background: Iron overload in association with persistent anemia is responsible for endocrine dysfunction in β-thalassemia patients, blood transfusion combined with iron-chelation can modify life quality in these children, but they tend to suffer from delayed maturity and endocrine dysfunction. Aim: This study aims to correlate degree of hypogonadism to ferritin load in regular transfused β-thalassemia patients. Methods: It was carried out on 30 β-thalassemia major (TM) patients aged 12 to 18 years, puberty was assessed clinically, blood picture on Cell-Dyne 2700, ferritin level and pattern of FSH, LH, testosterone and estradiol before and after gonadotropin (GnRH) analogue stimulation test, they were determined on ARCHITECT ABBOTT system. Results: Twenty patients had not yet achieved puberty, FSH level was 1.45 ± 1.88 mIU/ml before (GnRH) analogue and 3.78 ± 4.19 mIU/ml after 4 hours of injection. LH level was 1.91 ± 4.79 mIU/ml before (GnRH) test, while after 4 hours it was 6.52 ± 7.50 mIU/ml, 88.24% of males had low serum testosterone level, 84.6% of girls had low serum estradiol level, FSH, LH, estradiol, testosterone before and after GNRH analogue were statistically insignificant, mean ferritin level was 3344.32 ± 1142.142 ng/ml, with insignificant correlation to hormonal pattern before and after GnRH therapy. Conclusion: Iron overload and hypogonadism are the presenting data in this study, insignificant correlation between ferritin level and hormonal reserve pattern, there may be another etiology in pathophysiology of low gonadal reserve such as severe anemia, chronic disease and may be genetic predisposition underlying susceptibility to iron toxicity, which need further investigations.

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“…Hypogonadism and abnormal spermatogenesis have been identified as primary causes of sexual dysfunction seen in β-thalassemia patients [100,102]. The hypogonadism-like symptoms are related to either anemia [103] or iron accumulation in the pituitary gland [104]. Treating infertile male patients with β-thalassemia with growth hormone and gonadotropins improved semen parameters [105].…”

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

156

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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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Evaluation of Growth, Puberty and Endocrine Dysfunctions in Relation to Iron Overload in Multi Transfused Indian Thalassemia Patients

Cited by 30 publications

References 22 publications

Can the Availability of Unrestricted Financial Support Improve the Quality of Care of Thalassemics in a Center with Limited Resources? A Single Center Study from India

Can the Availability of Unrestricted Financial Support Improve the Quality of Care of Thalassemics in a Center with Limited Resources? A Single Center Study from India

Impact of Ferritin Load on Gonadal Reserve among Regular Transfused β-Thalassemia

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

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