Endocrine involvement in children with β-thalassaemia major. Transverse and longitudinal studies. I. Pituitary-thyroidal axis function and its correlation with serum ferritin levels

1984 ◽  
Vol 107 (1) ◽  
pp. 49-53 ◽  
Author(s):  
L. Cavallo ◽  
D. Licci ◽  
A. Acquafredda ◽  
M. Marranzini ◽  
R. Beccasio ◽  
...  

Abstract. Thyroid function was investigated by a TRH test in 24 clinically prepubertal children, 3–15 years old with β-thalassaemia major; in 7 of them the test was repeated once and in 2 twice at intervals of at least 12 months. Basal T4, T3, TBG and TSH levels and the TSH levels during a TRH test were determined and correlated with age and serum ferritin levels. Basal serum T4, T3 and TBG levels were lower and serum TSH levels were higher during the test and in the basal state in thalassaemia major children than in control children. These results show a compensated sub-clinical primary hypothyroidism. The transversal study did not show any significant correlation between the hormonal parameters studied and chronological age or serum ferritin levels. In contrast, the longitudinal study showed a significant correlation between pituitary-thyroidal axis function and siderosis (positive correlations between the variations of TSH levels as Δ, peak, 30 and 45 min values and the variations of serum ferritin levels). The thyroid impairment seems not to be correlated with serum ferritin levels in the transversal study because of the presence of an individual different sensitivity of the gland to the iron overload. The ferritin dependence of this impairment is shown only by longitudinal studies where individual differences in sensitivity of the gland are absent. Therefore iron chelation by desferrioxamine sc infusions, resulting in a decrease of ferritin, improves the deficient thyroid function.

2013 ◽  
Vol 5 (2) ◽  
pp. 87-90
Author(s):  
AKM Rezaul Karim ◽  
Md Rafiqul Islam ◽  
Farzana Deeba ◽  
Md Hasan Jamal Fakir ◽  
Abdul Matin

Background: Thalassaemia is a heterogeneous group of disorders. In multi-transfused thalassaemia major patients  the  thyroid  gland  function has  been  reported  to  be  normal,  decreased  reserve,  or  primary hypothyroidism.Objective: This study was an attempt to know the thyroid function status in multi-transfused thalassaemic patients and it's correlation with iron overload.Methods: This cross sectional study was carried out in the Department of Pediatrics at Bangabandhu Sheikh Mujib Medical University (BSMMU) and Transfusion centre of Bangladesh Thalassaemia Samity, Dhaka during May 2011 to April 2012. Previously diagnosed cases of thalassaemia major patients with transfusion dependent at any age with both sexes were randomly selected for this study. Serum total thyroxine (T4), total triiodothyronine (T3), thyroid stimulating hormone (TSH) and serum ferritin level were estimated from venous blood.Results: A total of 50 previously diagnosed cases of thalassaemia major patients of which 30 male and 20 female of 4 to 15 years age, transfusion dependent were randomly selected for this study. Hypothyroidism was present in 10(20%) patients.  Among  these 10 patients  compensated  primary  hypothyroidism  was  in  5(10%)  cases  and decompensate primary hypothyroidism was 5(10%) cases. The TSH value of hypothyroid patients was higher. Serum ferritin value was significantly higher in all the three groups of thalassaemic patients. There was no significant correlation between the high serum ferritin value with thyroid stimulating hormone or serum total thyroxin level.Conclusion: All transfusion dependent thalassaemic patients need periodic evaluation of thyroid  function  because  there  was  incidence  of  hypothyroidism.DOI: http://dx.doi.org/10.3329/jssmc.v5i2.20762J  Shaheed  Suhrawardy  Med  Coll 2013;5(2):87-90]


1981 ◽  
Vol 90 (5) ◽  
pp. 449-453 ◽  
Author(s):  
Donald P. Vrabec ◽  
Timothy J. Heffron

One hundred ninety-six head and neck patients were studied to determine the effects of radiation therapy and surgery on thyroid function. Serum thyroid-stimulating hormone (TSH) levels were obtained as a screening test for primary hypothyroidism. Elevated TSH levels were found in 57 of the 196 patients (29.1%). The highest incidence of abnormal TSH values (66%) occurred in the group treated with combination radiation therapy and surgery, including partial thyroidectomy. TSH levels rose early in the posttreatment period with 60% of the abnormal values occurring within the first three posttreatment years. Posttreatment thyroid dysfunction was twice as common in women (48.6%) as in men (25.4%). When serum thyroxine levels by radioimmunoassay (T4RIA) were correlated with the elevated serum TSH levels, a similar pattern was seen with 65% of the patients in Group 3 having a decreased T4RIA level indicating overt hypothyroidism. Pretreatment levels of thyroid function including thyroid antibody studies should be established for all patients. Serial TSH levels should be done every three months during the first three posttreatment years and semiannually thereafter as long as the patient will return for follow-up care. All patients treated with combination radiation therapy and surgery who develop elevated TSH levels should be treated with thyroid replacement therapy. Patients receiving radiation therapy alone should receive replacement thyroid therapy if they develop a depressed T4RIA value or a pattern of gradually increasing TSH levels.


2021 ◽  
Vol 20 (1) ◽  
pp. 130-135
Author(s):  
Soma Ghosh ◽  
Dwaipayan Chakrabarti

Objectives & introduction: Thalassemia, heterogenous group of disorders of haemoglobin,characterised by reduced or absent production of one or more of globin chains.Regular redcell transfusion with chelation therapy for iron overload are cornerstones of therapy for ßthalassemia major. Serum ferritin assay is widely available, relatively inexpensive methodfor assessing body iron burden and monitoring response to chelation process which in turnalso improves TSH levels in thalassemic subjects .The objective of this study was to assessprechelation and postchelation levels of serum ferritin and TSH and correlating post chelationlevels of serum ferritin and TSH in thalassemic patients >6yrs undergoing chelation therapy. Materials & methods: Serum TSH measured by Enzyme linked fluorescent assay and serumferritin measured by enzyme linked immunosorbent assay. Results: Amongst 500 participants,47% were males & 53% females. Mean age was 9.04 yrs ;prechelation ferritin and TSH levelswere 2995.78ng/ml with SD of 802.53 and 5.07 μU/ml with SD of 2.52. The postchelationferritin and TSH levels were 2168.80 ng/ml with SD of 1335.89 and 4.51μU/ml with SD of4.76. Paired t test with respect to pre and postchelation ferritin and TSH levels showed 2 tailed pas 0.000 and t>3, both of which considered significant. While correlating post chelation ferritinwith TSH levels; they showed a linear correlation ( Pearson coefficient of .836). Conclusion:Serum ferritin and TSH estimation in prechelation and postchelation periods give an estimateof iron overload with effect of chelation on it. Both levels decrease post chelation presenting alinear correlation between the two. Bangladesh Journal of Medical Science Vol.20(1) 2021 p.130-135


1988 ◽  
Vol 117 (1) ◽  
pp. 80-86 ◽  
Author(s):  
R. L. Chioléro ◽  
T. Lemarchand-Béraud ◽  
Y. Schutz ◽  
N. de Tribolet ◽  
M. Bayer-Berger ◽  
...  

Abstract. The pattern of thyroid function changes following severe trauma was assessed prospectively in 35 patients during the first 5 days after injury. Patients were divided into 2 groups to evaluate the effect of head injury: group I, patients with severe head injury; group II, patients with multiple injuries without head injury. The results demonstrate a low T3 and low T4 syndrome throughout the study, with decreases in both total and free levels of T3 and T4, normal or increased rT3 levels, and normal TSH levels. The presence of severe head injury was associated with lower levels of TSH and free T3. Mortality was 37%. Survival was associated with higher TSH and T3 levels, but not with higher T4 levels. TSH levels exceeding 1 mU/l on the first day were only observed in survivors. These findings show that a typical low T3 and low T4 syndrome is present after severe trauma in patients with multiple injury as well as with head injury. Primary hypothyroidism can be excluded, pituitary or hypothalamic hypothyroidism is likely in these patients.


1977 ◽  
Vol 85 (4) ◽  
pp. 744-752 ◽  
Author(s):  
Harald M. M. Frey ◽  
Egil Haug

ABSTRACT Forty mg TRH/day was given orally for 3 weeks to 10 euthyroid women and 10 women with primary hypothyroidism on low replacement doses of thyroxine. Once weekly oral TRH was replaced by an iv TRH-test (0.4 mg) with measurement of serum concentration of TSH, prolactin (PRL), thyroxine (T4), triiodothyronine (T3) and cholesterol. In the normal group, mean serum T4 concentration increased after one week and remained elevated. Serum TSH concentration showed a slight tendency to decline. Maximal rise in TSH concentration after iv TRH (ΔTSH) fell from a mean of 4.0 ng/ml to 1.4 ng/ml within one week and stayed low. T3, cholesterol, PRL and ΔPRL were normal and unchanged throughout. In the hypothyroid group T4, T3, cholesterol, PRL and ΔPRL were not influenced by the TRH administration. In 2 patients (with the highest serum T4 concentrations) serum TSH concentration was normal and resistant to iv TRH. Of the 8 patients with elevated TSH, basal level and ΔTSH did not change in 2 (with subnormal T4 levels and the highest TSH levels). In the other 6 (with intermediate T4 levels) basal TSH fell from a mean of 10.1 ng/ml to 4.2 ng/ml, and ΔTSH from 10.0 ng/ml to 3.3 ng/ml after three weeks. It is concluded that in addition to feed-back effect of thyroid hormones, the pituitary response to long-term administration of TRH is determined by other factors. Among these may be reduced pituitary TRH receptor capacity and the activity of the TSH producing cells.


1983 ◽  
Vol 104 (1) ◽  
pp. 35-41 ◽  
Author(s):  
C. Ferrari ◽  
M. Boghen ◽  
A. Paracchi ◽  
P. Rampini ◽  
F. Raiteri ◽  
...  

Abstract. Circulating thyroglobulin antibodies (TgAb) and microsomal antibodies (MsAb) and thyroid function (total and free T4 and T3, TSH basal and after TRH) have been evaluated in 92 hyperprolactinaemic patients (82 females and 10 males; 9 with macroprolactinoma, 22 with microprolactinoma, 4 with acromegaly, 5 with organic lesions of the hypothalamus, 2 with empty sella, 2 with idiopathic hypopituitarism, 2 with primary hypothyroidism, and 46 with idiopathic hyperprolactinaemia). Thyroid function was normal in all cases except 3 with hypothalamic disease and central hypothyroidism, the 2 patients with primary hypothyroidism and 2 with thyrotoxicosis (one due to Graves' disease and one to autonomous thyroid adenoma). High titres of TgAb (≥1/1250) and/or MsAb (≥ 1/1600) were found in the subject with Graves' disease, in one acromegalic, in the 2 primary hypothyroids, and in 12 women with either adenomatous or idiopathic hyperprolactinaemia; low titres of one or both antibodies were found in 9 other euthyroid women and in the one with toxic adenoma. In a control population of 185 subjects studied with the same methods, the prevalence of TgAb and/or MsAb positive (low titres) was 3.3% in females and 2.5% in males. Diffuse thyroid hyperplasia was clinically detectable in 12 euthyroid women and in the one with Graves' disease; 3 others had been previously operated for nodular goitre with histological evidence of Hashimoto's thyroiditis (2 cases) or for a cold nodule; a single thyroid nodule was present in the woman with toxic adenoma and in one euthyroid woman. Most of these subjects also had circulating TgAb and/or MsAb, and a few had increased TSH secretion. No significant differences were found in mean thyroid hormone and TSH levels between euthyroid hyperprolactinaemic subjects and healthy controls, but TRH-stimulated TSH levels were significantly higher in thyroid antibodies positive than negative subjects. These data, in agreement with a few previous reports, suggest that autoimmune thyroid disorders (especially asymptomatic autoimmune thyroiditis) occur in hyperprolactinaemic women with a prevalence far exceeding that observed in many surveys in the general population.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 5176-5176
Author(s):  
Polyxeni Delaporta ◽  
Maria Karantza ◽  
Sorina Boiu ◽  
Konstantinos Stokidis ◽  
Theoni Petropoulou ◽  
...  

Abstract Abstract 5176 Background: Disturbances of thyroid function is known to frequently occur in Thalassemia Major (TM); its types prevalence and severity vary in different cohorts. Primary hypothyroidism is caused by failure of thyroid function, while central (secondary) hypothyroidism by inefficient secretion of thyroid-stimulating hormone (TSH) due to pituitary gland dysfunction or reduced thyrotropin-releasing hormone (TRH) secretion from the hypothalamus. TSH levels in primary hypothyroidism are increased, while levels of free T4 (FT4) are decreased or within normal range. In secondary hypothyroidism FT4 levels are low and TSH levels normal or low. The main objective of this retrospective study was to assess the prevalence of central and primary hypothyroidism in a cohort of 364 Greek patients with TM (mean age 33. 0±9. 9 years, range: 1–56 years). Patients and Methods: Data from sequential laboratory evaluation on thyroid function in 364 patients with TM were retrospectively collected and analyzed. Assessment of thyroid function included measurements of TSH, T4, T3, FT4, FT3, anti-TPO, anti-TG by classical methods. Diagnostic criteria for primary hypothyroidism consisted of two consecutive measurements of low T4 or FT4 with increased TSH levels or two consecutive abnormally high levels of TSH despite normal levels of FT4, FT3, T4 and T3. The criteria for diagnosis of central hypothyroidism consisted of two consecutive measurements of low levels of T4 or FT4 with normal or low levels of TSH. In addition the age and ferritin levels at diagnosis of the hypothyroidism, as well as the type of thyroid treatment and chelation were recorded. To study the longitudinal prevalence of thyroid dysfunction, patients were stratified into 3 groups, according to the year of birth (Group A=1960–1970, 49 patients; Group B =1971–1980, 195 patients; Group C=1981–1990, 75 patients). As thyroid dysfunction increases with age, the incidence of thyroid disorders was compared between all three groups for patients ≤30years old, and for patients ≤40 years old between the groups A and B. Statistical analysis as well as BoxPlot values and regression lines presentation were performed using the STATGRAPHICS Centurion XVI. Statistical significance was set at p<0. 05. Results: A total of 364 patients (mean age 33. 0±9. 9 years, 180 females, 184 males) with TM were evaluated. Relevant data on the type and prevalence of thyroid dysfunction in all three groups of patients are summarized in table 1. Figure 1 illustrates the correlation between the age and the year of diagnosis of hypothyroidism. Comparing the data of the three age groups no significant differences in the overall prevalence of hypothryroidism was found. A significantly higher incidence of central hypothyroidism was found in group C compared to groups B and A (p=0. 00087, p=0. 0097 respectively) and higher prevalence of primary hypothyroidism in group A (for patients aged <40 years) in comparison with group B (p=0. 012). Ferritin levels at the time of diagnosis were significantly lower in group A compared to B and C and in group B compared to C (A/B p=0. 0014; B/C p=0. 0342; A/C p<0. 0001). A significant correlation (R2 =0. 47) was found between the age at diagnosis of hypothyroidism and the year of diagnosis (figure 1). Conclusions: The study demonstrated that thyroid disorders remain a frequent problem in Greek patients with TM with a trend of increasing prevalence with age and modification of the ratio of primary to secondary hypothyroidism. The increased incidence of central hypothyroidism in recent years could be attributed to an increased awareness and a more precise evaluation of this condition. The fact that ferritin levels at diagnosis were significantly higher in the younger age cohort may be suggestive that novel iron chelation modalities are more protective against iron-induced thyroid toxicity. Nevertheless, as toxicity may occur in early stages, the impact of these modalities in the incidence and severity of thyroid dysfunction may take years to be apparent. Disclosures: No relevant conflicts of interest to declare.


1982 ◽  
Vol 5 (6) ◽  
pp. 339-344 ◽  
Author(s):  
C. Giordano ◽  
N.G. De Santo ◽  
C. Carella ◽  
V. Mioli ◽  
G. Bazzato ◽  
...  

Thyroid function was explored in 27 CAPD patients and 25 HD patients. Dialysis was associated with low T3 and T4 levels, increased rT3 concentrations, normal T4: T3 and reduced T4: rT3 ratios, normal FT3 and Thyroglobulin concentrations. TSH levels increased during dialysis but still fell within normal limits. The data rule out a condition of primary hypothyroidism and point out to increased thyroidal dismission associated with the block T4-T3 in peripheral cells. Also the increased FT4 levels in CAPD patients could be due to de novo dismission of thyroidal T4.


1981 ◽  
Vol 96 (1) ◽  
pp. 59-64 ◽  
Author(s):  
L. Cavallo ◽  
A. Mautone ◽  
M. Altomare ◽  
M. Licciulli ◽  
A. Pascazio ◽  
...  

Abstract. Thyroid function was investigated in eleven β-thalassaemia major prepubertal children, 3–15 years old. Basal serum levels and the levels during a TRH-test of TSH, T4, T3, and rT3 were determined. The data were correlated with age and with serum ferritin levels. Although the patients were euthyroid clinically, the mean serum T3 and rT3 levels were significantly lower than the values in normal control children. Basal serum T4 and TSH values were similar to those of normal controls. In thalassaemic children, during the TRH-test, the increases in serum TSH at 20, 30, and 45 min, the peak and the maximum ΔTSH were higher than those normally found. Basal T3 and the 60 min value during the TRH-test were lower than normal, and the peak was delayed to 120 min. No statistically significant correlation was found between the hormonal parameters studied, and either chronological age or serum ferritin levels. That there is a slight impairment of thyroid secretion in thalassaemia major children is suggested only by the differences in response during the TRH-test (peak and maximum ΔTSH is increased, T3 increase delayed, the negative linear correlation between basal TSH values and per cent increase in T3 and maximum ΔT3, and the positive linear correlation between per cent increase in TSH and in T3). The low basal T3 and rT3 values might be explained by a decrease in the monodeiodinating enzymatic systems for T4, although other interpretations, e.g., increased clearance of T3 and rT3, cannot be excluded. The absence of clinical signs of hypothyroidism in thalassaemia major children might be due to the normal circulating levels of T4 if we accept that T4 has hormonal activity at least when T3 is low.


Author(s):  
Zeina A Munim Al-Thanoon ◽  
Zeina A Munim Al-Thanoon ◽  
Mustafa Basil ◽  
Nasih A Al-Kazzaz

Iron chelation therapy with deferoxamine (DFO),the current standard for the treatment of iron overload in patients with betathalassemia,requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence,resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox (DFX) is an orally administered iron chelator that has been approved for use in many countries. The requirement of an effective,well tolerated iron chelator with a less demanding mode of administration has led to the development of deferasirox. The present study was aimed to compare the satisfaction and compliance with deferoxamine versus deferasirox (Exjade®),a novel oral iron chelator in patients with transfusion - dependent beta- thalassemia. A cross-sectional,single-center investigation study was carried out in the Thalassemia Center of Ibn-Atheer Teaching Hospital in Nineveh province,Iraq. One hundred and eight thalassemic patients aged between 2- 20 years old having received multiple blood transfusions and a serum ferritin greater than 1500 ng/ml. Patients were randomised into two groups. Group 1 received deferoxamine at a dose of 20-50mg/kg/day and group 2 received deferasirox at the dose of 10-30 mg/kg/day. Another 56 apparently healthy volunteers were used as a control group. The assessment of chelation was done during the period between November 2013 and February 2014 by measurement of serum ferritin. Satisfaction and compliance was assessed by using a special questionnaire prepared by the researcher. Out of the 108 thalassemic patients enrolled there was no discontinuation in treatment with the two drugs under study. The serum ferritin did not change significantly in any of the chelation groups. In comparison with the patients who were treated with DFO,those receiving DFX reported a significantly higher rate of compliance and satisfaction (P < 0.05). However,no significant difference was observed between the two groups regarding their satisfaction (P > 0.05).Compliance with deferasirox (50 %) was more than that with deferoxamine (20 %). Satisfaction with deferoxamine was significantly lower than deferasirox (p= 0.00).


Sign in / Sign up

Export Citation Format

Share Document