scholarly journals Subclinical Hypothyroidism as the Most Common Thyroid Dysfunction Status in Children With Down’s Syndrome

2022 ◽  
Vol 12 ◽  
Author(s):  
Kamila Szeliga ◽  
Aleksandra Antosz ◽  
Karolina Skrzynska ◽  
Barbara Kalina-Faska ◽  
Aleksandra Januszek-Trzciakowska ◽  
...  
Keyword(s):  
Thyroid Function ◽  
Subclinical Hypothyroidism ◽  
Lymphoblastic Leukemia ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Overt Hypothyroidism ◽  
Z Score ◽  
The Mean ◽  
Thyroid Dysfunctions ◽  
Antithyroid Autoantibodies

IntroductionThyroid dysfunctions are one of the most common abnormalities coexisting in children with Down’s syndrome (DS) and have been reported in up to 54% of cases.Aim of the StudyThe purposes of this retrospective study were to investigate the course of subclinical hypothyroidism in children with DS, to evaluate the thyroid function of these subjects in relation to the risk of developing overt thyroid disease and autoimmunity, and to identify clinical and biochemical characteristics of patients prescribed L-T4 therapy in children and adolescents with DS and SH.Material and MethodsThe records of DS patients referred to the Endocrinology Outpatient Clinic between 2010 and 2015 for screening of thyroid function were observed till the end of 2019 June and analyzed retrospectively. The children diagnosed with congenital hypothyroidism, acute lymphoblastic leukemia, and seizures and treated with drugs that may have interfered with thyroid function like lithium, antiepileptic, or iodinated drugs and glucocorticoids were excluded from the study.ResultsThe data of 77 DS patients were collected, evaluated, and analyzed. The study group consisted of 73 patients (32 girls and 41 boys with the mean age at baseline of 3.0 ± 4.5 years). A total of 63/73 (87%) children were diagnosed with SH. The 16/63 (25.4%) patients were followed-up without the treatment (group SH-T0), and therapy with levothyroxine (L-T4) was introduced in 47/63 (74.6%) SH children with a mean dosage of 1.8 ± 1.0 μg/kg/day (group SH-T1). Thyroxine supplementation did not improve growth expressed as ΔhSDS (0.1 ± 1.3, ranged −2.1 to 3.8 in SH-T0 vs. 0.0 ± 0.7, ranged −1.7 to 1.4 in SH-T1, p = 0.96) and ΔBMI Z-score (0.3 ± 0.9, ranged −0.9 to 2.6 in SH-T0 vs. 0.3 ± 1.1, ranged −2.1 to 2.9 in SH-T1, p = 0.65). Positive anti-TPO and anti-TG antibodies were detected in 7/63 (11.1%) DS cases.ConclusionsSH is the most frequent presentation of thyroid gland dysfunction in DS children. A small percentage of patients develop an overt hypothyroidism, particularly in females with mostly positive titer of antithyroid autoantibodies.

scholarly journals Down's syndrome and acute leukemia in children: an analysis of phenotype by use of monoclonal antibodies and electron microscopic platelet peroxidase reaction [see comments]

Blood ◽  
1990 ◽  
Vol 76 (11) ◽  
pp. 2348-2353 ◽  
Author(s):  
S Kojima ◽  
T Matsuyama ◽  
T Sato ◽  
K Horibe ◽  
S Konishi ◽  
...  
Keyword(s):  
Monoclonal Antibodies ◽  
Acute Lymphoblastic Leukemia ◽  
Complete Remission ◽  
Acute Leukemia ◽  
Lymphoblastic Leukemia ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
Electron Microscopic ◽  
Normal Children ◽  
Response To Chemotherapy

Abstract The clinical, hematologic, and immunophenotypic features in 20 patients with Down's syndrome (DS) and acute leukemia were analyzed. Of the 20 patients, all 14 patients who were 3 years old and less were diagnosed as having acute megakaryoblastic leukemia (AMKL) by use of platelet- specific monoclonal antibodies and platelet peroxidase (PPO) reaction in electron microscopy. They were characterized by the presence of bone marrow fibrosis, having a history of myelodysplastic syndrome (MDS) and a poor response to chemotherapy. Only one patient has remained in continuous complete remission for more than 1 year. Acute leukemia in six patients who were older than 4 years was classified as common acute lymphoblastic leukemia antigen (CALLA)-positive acute lymphoblastic leukemia (ALL). In one of six patients classified as ALL, the leukemic blasts simultaneously expressed myeloid-associated surface antigens. All six patients achieved a complete remission and have remained in continuous complete remission and have remained in continuous complete remission from 10 to 52 months from the initial diagnosis. Although it has been suggested that the distribution of types of acute leukemia in patients with DS is similar to that in normal children, the present study shows that the distribution of acute leukemia types is quite different from that in patients without Down's syndrome.

THYROID FUNCTION TESTS IN ADULTS WITH DOWN'S SYNDROME

1978 ◽  
Vol 88 (1) ◽  
pp. 48-54 ◽  
Author(s):  
S. Korsager ◽  
E. M. Chatham ◽  
H. P. Østergaard Kristensen
Keyword(s):  
Thyroid Function ◽  
Down's Syndrome ◽  
Clinical Signs ◽  
Down’S Syndrome ◽  
Thyroid Stimulating Hormone ◽  
Thyroid Function Tests ◽  
Thyroid Status ◽  
Biochemical Tests ◽  
Old Patients ◽  
Function Tests

ABSTRACT Thyroid status was studied in 24 patients above the age of 40 years with Down's syndrome. Three patients had thyroid function tests indicating hypothyroidism. Eight patients had thyroid autoantibodies in serum and 8 patients had a higher than normal level of thyroid stimulating hormone in serum. None of the patients had figures indicating thyrotoxicosis. None of the patients showed any of the clinical signs usually seen in patients with hypothyroidism. It is concluded that biochemical tests indicating hypothyroidism are much more often seen in patients with Down's syndrome than in normal subjects and that thyroid status should be assessed in old patients with this disease.

Thyroid Function in Adults with Down's Syndrome

1977 ◽  
Vol 44 (3) ◽  
pp. 453-458 ◽  
Author(s):  
J. C. MURDOCH ◽  
W. A. RATCLIFFE ◽  
D. G. McLARTY ◽  
J. C. RODGER ◽  
J. G. RATCLIFFE
Keyword(s):  
Thyroid Function ◽  
Down's Syndrome ◽  
Down’S Syndrome

Are There Two Maternal Age Groups in Down's Syndrome?

1974 ◽  
Vol 124 (582) ◽  
pp. 453-455 ◽  
Author(s):  
P. A. P. Moran
Keyword(s):  
General Population ◽  
Frequency Distribution ◽  
Maternal Age ◽  
Down's Syndrome ◽  
Age Groups ◽  
Down’S Syndrome ◽  
Remarkable Feature ◽  
Complete Survey ◽  
The Mean

Penrose and Smith (1966) have reviewed the literature on Down's syndrome in great detail, and this has been followed by an important recent review by Richards (1973). In Chapters 10 and 11 of Penrose and Smith's book they discuss the remarkable frequency distribution of the ages of mothers of patients, compared with that of the general population at the corresponding place and time, and they summarize the large number of studies made on this subject. The mean age of the mothers is shifted upwards by amounts which vary in different countries from about 6 to 8 years. The remarkable feature, however, is that there appear to be two bumps in the curve. These are usually (but not always) not large enough to make the curve bimodal, and J. B. S. Haldane therefore coined the term ‘bitangentiality’ for this phenomenon, which appears in most published studies and in the group of all sample cases (9,441) given by Penrose and Smith, Fig. 76. Collmann and Stoller (1962) make a complete survey of all mongol births in Victoria, Australia, from 1942 to 1957 and here there is a distinct bimodality.

THYROID DISEASE AMONG CHILDREN WITH DOWN'S SYNDROME (MONGOLISM)

PEDIATRICS ◽  
1965 ◽  
Vol 36 (4) ◽  
pp. 608-614
Author(s):  
Alvin B. Hayles ◽  
Ward L. Hinrichs ◽  
W. Newlon Tauxe
Keyword(s):  
Iron Deficiency ◽  
Thyroid Function ◽  
Binding Capacity ◽  
Down's Syndrome ◽  
Clinical Manifestations ◽  
Down’S Syndrome ◽  
Primary Hypothyroidism ◽  
Deficiency Anemia ◽  
Carrier Protein ◽  
Normal Children

Observations on two mongoloid children, one having primary hypothyroidism and the other having primary hyperthyroidism, have been reported. Primary hypothyroidism in the mongoloid child, in each of the three cases reported to date, has been associated with uterine bleeding attributed to hormonal overlap in the pituitary feedback mechanism. The significance of this phenomenon is not clean. A total of sixteen cases of mongolism with hyperthyroidism have been described. There is no evidence that altered thyroid function plays a role in the clinical manifestations of mongolism, and significant alteration in thyroid function is uncommon among such patients. Both of the carrier protein systems responded to the stresses of the superimposed disease processes in these mongoloid children as they do in normal children. Specifically, the values for the erythrocytic uptake of 1-I131-triiodothyronine and thyroxine and the triiodothynonine binding capacities (Case 2 only) were all in the expected ranges for both thyroid derangements involved. Similarly, in Case 2, where an iron-deficiency anemia was also present, the increase in the iron binding capacity and presumably in the level of transferrin was on the order of that to be expected for the degree of iron deficiency. The response to stress of both carrier protein systems in Down's Syndrome suggests that protein synthesis of transferrin and TBG is normal.

Granulopoiesis in Down's Syndrome

PEDIATRICS ◽  
1966 ◽  
Vol 37 (1) ◽  
pp. 108-110
Author(s):  
PETER R. GALBRAITH ◽  
LESLIE S. VALBERG
Keyword(s):  
Turnover Rate ◽  
Down's Syndrome ◽  
Down’S Syndrome ◽  
The Mean ◽  
Blood Granulocyte

The rate of disappearance of granulocytes labeled with DFP32 from the circulation was normal in four subjects with Down's syndrome. The mean blood granulocyte mass and mean granulocyte turnover rate were also normal in these subjects.

Reversal of Hypothyroidism in Well Chelated β-Thalassemia Major Patients

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3884-3884
Author(s):  
Kallistheni Farmaki ◽  
Ioanna Tzoumari ◽  
Christina Pappa
Keyword(s):  
Thyroid Gland ◽  
Iron Overload ◽  
Thyroid Function ◽  
Thalassemia Major ◽  
Stimulation Test ◽  
Overt Hypothyroidism ◽  
Trh Stimulation ◽  
Increased Risk ◽  
The Mean ◽  
Tsh Levels

Abstract Thyroid dysfunction is known to occur frequently in β-Thalassemia major patients (TMps), but its prevalence and severity varies in different cohorts according to chelation regimens. Thyroid hormones are critical determinants of brain and somatic development in infants and of metabolic activity in adults affecting the function of virtually every organ system. Thyroid gland mainly secrets T4, whereas 80% of T3 is produced by de-iodination of T4 (liver, kidney, heart and other tissues) and is influenced by a variety of factors. Furthermore, T4 & T3 secretion is tightly regulated within narrow limits by a mechanism that involves the pituitary-secreted TSH which in turn is stimulated by the hypothalamic TRH. Thus, iron overload-related hypothyroidism may be either central (because of deposition in the pituitary or the hypothalamus) and usually associated with other endocrinopathies, or primary (by deposition in the thyroid gland or even other organs). Existing data suggest that the thyroid gland appears to fail before the central components of the axis. In all cases, symptoms occur slowly over time and may vary from subclinical to overt hypothyroidism which is associated with an increased risk of cardiovascular disease. The aim of this study was to investigate the effect of long-term intensive combined chelation therapy on thyroid function in TMps after they were all in negative iron balance. 51 TMps, 25 males 26 females, mean age 29.8±2.03, who were previously maintained on subcutaneous desferrioxamine monotherapy (DFO:40mg/kg, 3–6 days/week) switched to an intensive combined chelation with DFO (40–60mg/kg/d) and Deferiprone (DFP: 75–100mg/kg/d) adapted to individual needs. Thyroid function was assessed initially and after 6 years by TRH stimulation test and TSH, FT4 & FT3 screening. All patients on hormone replacement therapy stopped treatment at least 30 days before the test. This was approved by the Hospital Scientific Committee. Criteria for the diagnosis of subclinical or compensated hypothyroidism was an increase of the TSH levels during the test of more than 20 μIU/ml from the basal value or an elevated basal TSH concentration (>5 μIU/ml) and for overt hypothyroidism a further decrease in FT4 & FT3 levels. With DFO monotherapy 18 TMps were treated with thyroxin therapy. In these patients after combined chelation and an important decrease in iron overload (p<0.0001) as estimated by ferritin levels (2,737±473 vs 450±225mg/dl), MRI liver and heart iron quantification (T2*L & T2*H) and LIC calculated by Ferriscan (13±3 vs. 1.4±0.5mg/gdw), a significant increase was observed in mean FT4 (1.07±0.03 vs. 0.7±0.02ng/ml, p<0.0001) & mean FT3 (2.6±0.1 vs. 1.3± 0.1pg/ml, p<0.0001) and an additional significant decrease in the mean TSH quantitative secretion, calculated as the area under the curve (AUC=1,332±131 vs. 2,231±241, p<0.0001). These 10/18 (56%) TMps with subclinical or compensated hypothyroidism, who normalized TSH, FT4 & FT3 levels and had a normal TRH stimulation test discontinued thyroxin therapy, while another 4/18 (22%) reduced their thyroxin dose. The remaining 4/18 with overt hypothyroidism, while they all improved their TRH stimulation test, only 2 improved to compensated hypothyroidism with TSH levels 5–10mIU/ml and normal FT4 & FT3 levels. Critically, in the other 33/51 euthyroid TMps, no new cases of hypothyroidism were noted after combined chelation and a significant increase (p<0.0001) was observed in the mean FT4 & FT3 levels with a significant decrease (p<0.0001) in the mean TSH quantitative secretion (AUC). This study showed that intensive combined chelation associated with a significant decrease of iron overload may reverse some cases of primary hypothyroidism, either subclinical or compensated, and may prevent progression to overt hypothyroidism, thus influencing the decision to treat with thyroid hormone. It may also improve some cases of overt hypothyroidism suggesting that even iron-induced damage of the thyroid pituitary axis might be ameliorated.

Thyroid evaluation of children and adolescents with Williams syndrome in Zhejiang Province

2017 ◽  
Vol 30 (12) ◽  
Author(s):  
Wei-Jun Chen ◽  
Chai Ji ◽  
Dan Yao ◽  
Zheng-Yan Zhao
Keyword(s):  
Children And Adolescents ◽  
Thyroid Function ◽  
Williams Syndrome ◽  
Subclinical Hypothyroidism ◽  
Thyroid Stimulating Hormone ◽  
Zhejiang Province ◽  
Overt Hypothyroidism ◽  
Thyroid Peroxidase ◽  
Thyroid Antibodies ◽  
Free Triiodothyronine

AbstractBackground:The objective of the study was to describe the prevalence of abnormal thyroid function and volume in children and adolescents with Williams syndrome (WS) in Zhejiang Province, China.Methods:Thyroid function, including thyroid-stimulating hormone (TSH), free triiodothyronine (fT3), free thyroxine (fT4), and thyroid antibodies (thyroid peroxidase and thyroglobulin) were measured in 83 patients with WS, aged 0.2–16.5 years. Twenty-three patients were followed for an average of 1.7 years (0.4–4.1), and multiple TSH determinations were considered. Thyroid ultrasonography was performed on 49 patients.Results:One patient was diagnosed with overt hypothyroidism, and 23 patients (27%) had subclinical hypothyroidism (SH). Thyroid antibodies were absent in all patients. In five age groups (0–1 years, 1–3 years, 3–6 years, 6–9 years, 9–18 years), the prevalence of patients with subclinical hypothyroidism was 25%, 28.5%, 44.4%, 16.7% and 4.7%, respectively. Through ultrasound examination, 21 patients (42%) were observed to have thyroid hypoplasia (TH), and there were no cases of thyroid haemiagenesis. The incidence rate of TH increased with age, rising from 20% in the youngest group to 66% in the oldest.Conclusions:SH and TH is common in children and adolescents with WS. Yearly evaluation of thyroid must be performed in all patients in this population, regardless of the result of the neonatal screening. Age under 6 years and existing thyroid abnormalities are risk factors for developing SH, and a shorter follow-up interval is needed for screening in these individuals, SH is often self-limiting, and clinicians should be alert to overt hypothyroidism.

Sign in / Sign up

Export Citation Format

Share Document