Endocrine abnormalities in children with Beta thalassaemia major

Joshi, Rajesh; Phatarpekar, Ankur

doi:10.4038/sljch.v42i2.5628

Cited by 10 publications

(7 citation statements)

References 10 publications

(16 reference statements)

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“…A similar study investigating the prevalence of endocrine disorders in patients with transfusion-dependent thalassemia showed that the most common endocrinopathy encountered was a delayed puberty at a prevalence of 68%, followed by low bone mineral density in 56.1% and short stature in 52.5% of the total sample size. 10 The results are somewhat similar to the findings of our study, in which short stature was the most common complication but with a prevalence of 39.5%, though this was succeeded by the development of diabetes mellitus in 29.4% of all sample size. Furthermore, gender was considered as a risk factor for the development of short stature as males were found to be more predisposed than females ( p =0.001).…”

Section: Discussionsupporting

confidence: 90%

“…19 Hypoparathyroidism as a complication of transfusion-dependent hemoglobinopathies was reported by some studied in prevalences of 9.4 %and 13.2% whereas others reported a prevalence of 0%. 10,14,19 In published literature, it has a more frequent prevalence between 3.6% to 22.5%; however, in the present study it was considered the least frequent endocrinopathy with an overall prevalence of only 2.5%. 20,21 In addition, vitamin D deficiency was studied as an association in patients with severe hemoglobinopathies.…”

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confidence: 58%

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Endocrinopathies complicating transfusion-dependent hemoglobinopathy

Al-Agha

Bawahab

Nagadi

et al. 2020

SMJ

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Objectives: To investigate the prevalence and significance of different endocrinopathies in children and adolescents with transfusion-dependent thalassemia and sickle-cell anemia. Methods: This is a descriptive, retrospective study between January 2010 and July 2018 in King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Data was collected through reviewing electronic hospital medical records then filling out data collection sheets and was interpreted through the IBM SPSS Statistics for Windows version 20.0 (IBM Corp, Armonk, NY, USA). Original Article Results: The total sample size was 119 patients, gender equality was almost achieved with 55.5% being male and 45.5% being female. The most common endocrinopathies were identified in the following order of short stature (39.5%), diabetes mellitus (29.4%), hypogonadism (12.6%), osteopenia (12.6%), osteoporosis (9.2%), hypothyroidism (9.2%), hypocortisolism (3.4%), and hypoparathyroidism (2.5%). All of which were statistically significant in their relationship to hemoglobinopathies with the exception of osteopenia and osteoporosis. Hypogonadism and hypocortisolism were found to be statistically significant in their relationship to a positive history of splenectomy at p=0.026 and p=0.012. Short stature was found to be statistically significant in its relationship to the male gender with a p=0.001. Conclusion: Endocrinopathy is a frequent complication of hemoglobinopathies, for which the most common were found to be short stature, diabetes mellitus, and low bone mineral density.

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Section: Discussionsupporting

confidence: 90%

contrasting

confidence: 58%

Endocrinopathies complicating transfusion-dependent hemoglobinopathy

Al-Agha

Bawahab

Nagadi

et al. 2020

SMJ

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“…In contrast, Joshi et al reported the range of serum ferritin level of 5,295 (SD 2,736) ng/mL and Hb value of 7.8 (SD 0.6) g/dL were not significantly different in the thalassemia group and normal group. 25 Another study found a correlation between serum ferritin level >2,000 ng/mL and height-for-age (Z-score) >10-15 years (P<0.001). 23 Study in Indonesia reported a correlation between serum ferritin level (OR=3.248; 95%CI 1.304 to 8.086) and growth disorder in thalassemia patients (OR=3.964; 95%CI 1.192 to 13.190).…”

Section: Resultsmentioning

confidence: 95%

Serum IGF-1 and short stature in adolescents with beta-thalassemia major

Elizabeth

Fadlyana

Reniarti

et al. 2018

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Background The prevalence of short stature in thalassemia patients ranges from 39.3 to 65%. The cause of short stature is complex and still up for debate. In Indonesia, data on the prevalence and risk factors of short stature in adolescents with thalassemia have been limited. Objective To assess for the prevalence and risk factors of short stature in adolescents with beta-thalassemia major. Methods This cross-sectional study was done from February to March 2017 at the Thalassemia Clinic at Dr. Hasan Sadikin General Hospital, Bandung. The baseline characteristics data of 80 adolescents with thalassemia aged 10-14 years were recorded. Short stature was assessed by height-for-age, (Z-score <-2SD) based on the 2007 WHO Reference Growth Chart. Mid-upper arm circumference was scored according to age and sex and serum IGF-1 was measured by ELISA method. Data analyses used were Chi-square, Fisher’s, and Mann-Whitney tests. Logistic regression model was used to further analyze for risk factors of short stature. Results Subjects were 40 males and 40 females, 81.2% of whom had short stature. The mean serum IGF-1 level was 32.2 (SD 26.38) ng/mL. The IGF-1 cut-off point by ROC curve was £38.51 ng/mL, with sensitivity of 64.4% and specificity of 86.7%. The risk factors of short stature were IGF-1 level £38.51 ng/mL (PR 40.66; 95%CI 4.37 to 377.58; P<0.001) and low family income (PR 19.76; 95%CI: 1.152 to 256.08; P=0.022). Conclusion IGF-1 level may be useful as a predictor of short stature in adolescent beta-thalassemia major patients.

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“…Β-thalassemia is the most frequent type of thalassemia. 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].…”

Section: Introductionmentioning

confidence: 99%

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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Endocrine abnormalities in children with Beta thalassaemia major

Cited by 10 publications

References 10 publications

Endocrinopathies complicating transfusion-dependent hemoglobinopathy

Endocrinopathies complicating transfusion-dependent hemoglobinopathy

Serum IGF-1 and short stature in adolescents with beta-thalassemia major

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

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