A Review of Endocrine Disorders in Thalassaemia

De, Parijat; Mistry, Radhika; Wright, Chris; Pancham, Shivan; Burbridge, Wyn; Gangopadhayay, Kalyan; Pang, Terence; Das, Gautam

doi:10.4236/ojemd.2014.42003

Cited by 17 publications

(20 citation statements)

References 72 publications

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“…The results of our study showed that height (short stature) and weight (weight failure ) of patients were signi icantly correlated with the Tanner stage (P=0.028 and P=0.007, respectively). Our results are similar to other studies done in other areas of the world like Heshmat Moayeri MD study in Tehran, Iran, and M. G. Vogiatzi study in North America (De et al, 2014). There are multifactorial pathogenesis explained growth failure in thalassemia and commonly iron accumulation due to frequent transfusion of blood and iron toxicity on endocrine gland (Puberty retardation, hypogonadism, GH de iciency, hypothyroidism, and diabetes), Side effect of chelating agents especially desferrioxamine, Another factors such as chronic anemia, hypersplenism, de iciency of folate, mineral such as Calcium and zinc (Vogiatzi et al, 2009).…”

Section: General Characteristics Of the Study Samplesupporting

confidence: 92%

The Association Between Iron Over Load and Tanner Stage Retardation in the Females with B-Thalassemia Major

mutlag

2020

ijrps

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Despite optimal therapy of patients with B- major thalassemia included repeated transfusion of blood program and iron chelation agents helped by increasing survival of these patients but remained a major problem in adolescents of these patients such as growth failure and hypogonadism. This study was aimed to determine the relationship between iron overload and tanner stage retardation among female patients with B- major thalassemia in Thalassemia Hospital in Diwaniyah Governorate. The current study occurred on all female patients diagnosed β-thalassemia major depends on the blood tests, with their age range from 13years to 16 years who registered in Thalassemia unit in Al- Diwaniyah Governorate, Republic of Iraq. In the physical examination, the patients were assessed for weight, height, Tanner stages, and body mass index(BMI), which recorded. S. Ferritin value was used to assess the iron load, and pelvic ultrasound was checked to assess the size of the uterus and both ovaries. The results of the currents study revealed that the total numbers of B- thalassemia major female patients are 31 patients, aged 13-16 years. Age of patients at which diagnosed of B- major thalassemia range from 0.17 to 5 year. The frequency of Blood transfusion (time/Year) ranges from 6 to 33 times/Year. The level of serum ferritin of the patients was ranged from 913-12000 ng/ml with. Tanner stage I was predominant, accounting for 87%, whereas stage II and III accounted for 10% and 3%, respectively. There was a significant negative relation between times transfusion of blood and Tanner. There was a significant correlation between Uterus size, ovarian size, and Tanner stage. Because of inflammation falsely increase serum ferritin or due to the relation between body iron in the body and level of serum ferritin is not always within the linear range, especially in the condition of inflammation or tissue damage. So that level of serum ferritin is not an adequate measure of iron stores in patients with major thalassemia. Therefore, we needed another indicator to measure iron stores in patients with thalassemia major such as liver iron concentration.

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Section: General Characteristics Of the Study Samplesupporting

confidence: 92%

The Association Between Iron Over Load and Tanner Stage Retardation in the Females with B-Thalassemia Major

mutlag

2020

ijrps

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“…[1][2][3][4][5] About 20-30% might have growth hormone deficiency and 80% might have very low growth hormone that were lower than would be expected for constitutional short stature. 6 In Indonesian thalassemia patients, 65% had short stature, 20% delayed puberty, 41% hypoparathyroidism, and 29% delayed bone age. 5 A previous study at Dr. Hasan Sadikin Hospital found that 67% of 10 to14-year-old patients had delayed growth.…”

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confidence: 99%

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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“…And hypogonadism is the most common endocrine complication in such patient [4]. There is evidence that hypogonadism can be due to primary gonadal failure as a puberty is also an important contributing factor to growth failure in adolescent, who do not exhibit a normal growth spurt [3,6,7]. Growth hormone deficiency is not a rare occurrence in adult thalassemic patients [8].…”

Section: Discussionmentioning

confidence: 99%

“…It has already been established that the iron overload is the prime cause of endocrinopathy in thalassemia [3]. And hypogonadism is the most common endocrine complication in such patient [4].…”

Section: Discussionmentioning

confidence: 99%

“…Excessive iron is deposited in most tissues primarily in the liver, heart and the endocrine glands [2]. Disorders of growth, sexual development & fertility, abnormal bone mineralisation, diabetes mellitus, hypothyroidism and hypoadrenalism are the main endocrine complications found in thalassemic patients [3]. Endocrine abnormalities should be monitored carefully and a thorough endocrine evaluation should be carried out yearly in every thalassemic patient.…”

Section: Introductionmentioning

confidence: 99%

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Multiple Endocrinologic Complications in Thalassemia Major

Wong

Omar

Ismail

2017

Korean J Clin Lab Sci.

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Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.

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A Review of Endocrine Disorders in Thalassaemia

Cited by 17 publications

References 72 publications

The Association Between Iron Over Load and Tanner Stage Retardation in the Females with B-Thalassemia Major

The Association Between Iron Over Load and Tanner Stage Retardation in the Females with B-Thalassemia Major

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

Multiple Endocrinologic Complications in Thalassemia Major

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