methimazole and Down-Syndrome

Trisomia 21 pary chromosomów (Zespół Downa, DS) jest jednym z częściej występujących zaburzeń chromosomalnych w praktyce pediatrycznej. Zaburzenia funkcji tarczycy często występują u tych pacjentów. Niedoczynność tarczycy jest rozpoznawana u blisko 50% pacjentów z ZD, a liczba ta wzrasta wraz z wiekiem. Nadczynność tarczycy jest rozpoznawana znaczniej rzadziej u dzieci z ZD. Odpowiednia strategia leczenia nadczynności tarczycy jest bardzo istotna ze względu na możliwość zaburzenia rozwoju dzieci. Przedstawiamy dwa przypadki dzieci z zespołem Downa i nadczynnością tarczycy. Proces leczenia tych pacjentów był odmienny.

Topics: Child; Down Syndrome; Female; Humans; Hyperthyroidism; Male; Methimazole; Thyroxine; Treatment Outcome

Aim of this commentary is to summarize the salient literature news on the relationships between autoimmune thyroid diseases (ATDs) and either Down syndrome (DS) or Turner syndrome (TS).According to literature reports both Hashimoto's thyroiditis (HT) and Graves' disease (GD) are more frequent in children with DS or TS than in those without these chromosomopathies.An up-regulation of proinflammatory cytokines might be responsible for the enhanced susceptibility of TS children to ATDs, whereas a dysregulation of immune system may favor the development of ATDs in DS.In TS children biochemical presentation of HT is less severe than in peer controls. In both DS and TS GD picture at the time of diagnosis is not significantly different than in the pediatric general population.The evolution over time of GD in DS and TS does not differ from that observed in the pediatric general population, whereas the evolution of HT in both TS and DS is more severe than in girls without these chromosomopathies.. The association with TS or DS is able to affect both epidemiology and course of ATDs by conditioning: a) an increased susceptibility to these disorders; b) a less severe biochemical presentation and a more severe evolutive pattern of HT in TS girls; c) a more severe biochemical presentation and evolution of HT in DS patients.

Topics: Antithyroid Agents; Child; Comorbidity; Down Syndrome; Genetic Predisposition to Disease; Graves Disease; Hashimoto Disease; Humans; Methimazole; Turner Syndrome

It is known that Hashimoto's thyroiditis (HT) may progress to Graves' disease (GD) and that this phenomenon may be more frequent in the patients with Down syndrome (DS).. To shed light on the relationships between Down syndrome (DS) and metamorphic thyroid autoimmunity.. We reconstructed the conversion process from HT to GD in 12 DS children. All the data recorded at HT diagnosis and throughout the time interval from entry to GD presentation were retrospectively taken from patients' files, as well as those recorded at GD diagnosis and during the subsequent evolution. From GD diagnosis all patients underwent methimazole treatment, at a dose that was adjusted on the basis of clinical findings and thyroid tests.. Time interval between HT and GD was not different in the seven patients who received during that time a L-thyroxine (L-T4) treatment than in those who were not treated. After methimazole onset all patients exhibited a prolonged remission of hyperthyroidism. In 8/12 patients this treatment is still being continued 2-7 years after its initiation. The mean methimazole dosage needed to maintain euthyroidism in these eight patients was 0.12 ± 0.02 mg/kg/day. In the remaining four patients methimazole was withdrawn from 1.9 to 7 years after its initiation and no relapses were recorded 2.0-2.1 years after its withdrawal. These patients developed, 0.1-0.3 years after methimazole withdrawal, a picture of overt hypothyroidism and needed treatment with L-T4, that is now being continued. No patients needed non-pharmacological therapies.. 1) DS children might be incline to manifest over time a phenotypic metamorphosis from HT to GD and to subsequently fluctuate from hyperthyroidism to hypothyroidism; 2) in DS GD may have a mild biochemical and clinical course.

Topics: Adolescent; Antithyroid Agents; Autoimmunity; Child; Child, Preschool; Down Syndrome; Female; Graves Disease; Hashimoto Disease; Humans; Male; Methimazole; Retrospective Studies; Thyroid Gland; Young Adult

To compare the presentation and clinical course of Graves' disease (GD) in two pediatric populations consisting of 28 patients with Down's syndrome (DS) and 109 controls without DS respectively.. The evolution over time of GD was determined in both groups according to the clinical changes and the variations in TSH, free thyroxine, and TSH receptor autoantibodies serum levels during the entire follow-up.. Female prevalence (50 vs 81.6%; chi(2)=12.0, P<0.0005) and average age at GD presentation (9.9+/-4.4 vs 11.5+/-3.5 years, P<0.05) were significantly lower in DS group than in controls. Clinical responsiveness to methimazole therapy was significantly better in DS patients, as demonstrated by both the lower relapse rates after the first cycle withdrawal (7.1 vs 31.2%; chi(2)=7.4, P<0.005) and the higher persistent remission rates after definitive therapy withdrawal (46.4 vs 26.7%; chi(2)=4.1, P<0.05). Moreover, in DS group, no patients needed surgery or radioiodine ablation, whereas non-pharmacological treatment was necessary in 11% of controls (chi(2)=3.8, P<0.05). Antecedents of Hashimoto's thyroiditis (HT) were documented in 21.4% of DS patients and in 3.7% of controls (chi(2)=10.4, P<0.005). Association with other autoimmune diseases was detected in 32.1% of DS cases and in 12.8% of controls (chi(2)=5.94, P<0.025).. GD in DS children and adolescents is characterized by several peculiarities: i) earlier presentation; ii) no gender predominance; iii) less severe clinical course; iv) higher frequency of documented HT antecedents; v) more frequent association with other autoimmune diseases.

Topics: Adolescent; Age of Onset; Antithyroid Agents; Chi-Square Distribution; Child; Disease Progression; Down Syndrome; Female; Graves Disease; Humans; Kaplan-Meier Estimate; Male; Methimazole; Patient Selection; Prevalence; Severity of Illness Index; Sex Factors; Statistics, Nonparametric