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A new instrument: the goiter extractor.

In order to simplify the extraction of large intrathoracic goiters and to avoid dramatic surgical problems, the author developed a simple instrument to extract goitric masses. The extractor was used 17 times (approximately 10% of the thyroidectomy procedures performed during 3 years). Its effectiveness was proven in 16 cases.

Goiter, Substernal↗

Five novel inactivating mutations in the thyroid peroxidase gene responsible for congenital goiter and iodide organification defect.

Thyroid peroxidase (TPO) defects, typically transmitted as autosomal recessive traits, result in hypothyroid goiters with failure to convert iodide into organic iodine. We analyzed the TPO gene in 14 unrelated patients with clinical evidence of iodide organification defects. Seven of the affected individuals harbored mutations in the TPO gene; one was compound heterozygous, the others were simply heterozygous for TPO mutations. Five novel mutations have been identified, one of which was found to be a single nucleotide deletion, while the other four were single nucleotide substitutions. A frameshift mutation c.387delC was detected in exon 5 which leads to an early termination signal in exon 7 (p.N129fsX208). Two missense mutations were identified in exon 8. The first, a c.920A>C transversion that results in a p.N307T substitution, was found in two patients. The second, a c.1297G>A transition, results in p.V433M. A c.1496C>T transition was detected in exon 9 that caused the substitution p.P499L. Finally, in exon 14 a c.2422T>C transition was identified, causing a p.C808R change. In addition, the previously reported GGCC duplication in exon 8 (c.1186_1187insGGCC; p.R396fsX472) was also detected in two affected individuals, one of whom was a compound heterozygous (p.R396fsX472/p.V433M).

Frameshift Mutation↗

Ultrasonography of the thyroid gland in hospitalized, chronically ill geriatric patients: thyroid volume, its relationship to age and disease, and the prevalence of diffuse and nodular goiter.

Ultrasongraphy of the thyroid gland was performed in a screening study of 177 chronically ill, hospitalized geriatric patients older than 60 years of age, from an area with only moderate iodine deficiency. The normal reference ranges of thyroid volume for males (1.7 mL to 22.2 mL) and for females 2.4 mL to 20.9 mL) were similar. The thyroid volume decreased with age in the euthyroid group and was also smaller in euthyroid females in bad health. The prevalence of goiter and thyroid nodules were 7.9% and 11.3%, respectively. Thus, the decrease of the thyroid volume in this geriatric population is probably related to both old age and diseases of the aged.

Age Factors↗

Doppler evaluation of pediatric goiter: effect of mandatory iodination.

PURPOSE: The aim of this study was to evaluate the hemodynamic status of the thyroid in children with goiter after the use of iodinated dietary salt for 3 years in a region of endemic iodine deficiency. METHODS: Sixty-six children between 7 and 12 years of age were included in the study. Three groups were constituted according to sonographically measured thyroid volume and urinary iodine excretion levels. Group 1 included 11 children with thyroid volumes greater than the 97th percentile according to age and sex criteria suggested by the World Health Organization International Council for Control of Iodine Deficiency Disorders and urinary iodine level lower than 100 microg/l. Group 2 included 30 children with thyroid volumes greater than the 97th percentile and urinary iodine level equal to or higher than 100 microg/l. The control group included 25 children who had normal thyroid volume and urinary iodine level. All children were examined by thyroid duplex sonography. Peak systolic velocity (PSV) and resistance index (RI) were measured in the inferior thyroid artery bilaterally. RESULTS: PSV in group 1 was significantly higher than in group 2 and in the control group (P < 0.05 and P < 0.01, respectively). There was no significant difference between the PSV of group 2 and the control group. The RI in groups 1 and 2 was significantly lower than in the control group (P < 0.01 and P < 0.01, respectively). There was no significant difference between the RIs of group 1 and group 2. CONCLUSIONS: These findings suggest an effect of iodination on thyroid hemodynamics before the size of the hyperplastic thyroid returned to normal, in keeping with normalization of the urinary iodine level.

Child↗

A new locus for a dominant form of multinodular goiter on 3q26.1-q26.3.

A mass screening program for congenital hypothyroidism has markedly improved prognosis of children with congenital hypothyroidism and also revealed several cases with unknown pathogenesis. We here report two independent Japanese multigeneration families with multinodular goiter (MNG) with euthyroidism and with high TSH. The propositi, 3- and 8-year-old girls in two families, were found during a mass screening. An autosomal dominant pattern of inheritance was suggested in both families. The clinical examinations suggested impaired hormonogenesis but discarded known defects in iodine transport, organification, deficiency of hydrogen peroxide, and thyroid peroxidase. Linkage analysis of the two families including 10 members each using 343 microsatellite markers mapped a single locus independently at D3S1618 (theta = 0) on 3q26.1-q26.3 with a two-point LOD score 3.62 (1.81 for each family) and multipoint LOD score of 3.61 (1.80 for each family). Haplotype inspection delimited an 18-cM interval between D3S1565 and D3S3686.

Adult↗

Serum levels of the carboxy-terminal cross-linked telopeptide of type I collagen and laminin are elevated in Graves' disease but not in toxic nodular goiter.

We determined serum levels of laminin, carboxy-terminal cross-linked telopeptide, and carboxy-terminal propeptide of type I collagen (ICTP and PICP) in 27 patients with Graves' disease (GD) and in 31 patients with toxic nodular goiter (TNG). Elevated levels of soluble laminin and ICTP were observed in patients with GD prior to treatment (mean +/- SD concentration 1444 +/- 404 and 8.6 +/- 3.5 ng/ml, respectively), compared to patients with TNG (476 +/- 103 and 4.2 +/- 1.5 ng/ml) and normal controls (492 +/- 112 and 3.1 +/- 1.3 ng/ml, n = 34). In contrast, serum PICP concentrations were not different between patients with GD or TNG and normal controls. In GD patients serum ICTP and laminin levels decreased during thiamazole treatment and normalized within 4 and 8 weeks of therapy. There was no correlation between serum ICTP and laminin levels and serum levels of thyroid hormones and/or autoantibodies, respectively. Serum ICTP and laminin could be markers for alterations of extracellular matrix during GD and release of matrix components in the circulation and/or reflect an impaired clearance.

Adult↗

Intrathyroid salivary gland-type tissue in multinodular goiter.

We report a case of intrathyroid salivary gland tissue in a 66-year-old Caucasian female with multinodular goiter. Lobules of well differentiated seromucinous salivary glands were found in close relationship with cartilage and fat, and intimately associated with normal thyroid follicles and solid cell nests (SCN) of the thyroid gland. This is the first report of non-neoplastic intrathyroid salivary gland tissue. We conclude that this heterotopic tissue probably arises from the SCN as ultimobranchial vestigial structures.

Aged↗

[Restitution of the thyrotropic function in goiter patients after discontinuing a long-term suppression therapy with thyroid hormones].

27 subjects with a negative TRH test were selected among patients with euthyroid goiter treated by thyroid hormones. The hormone therapy was stopped and T3 and T4 concentrations, basal and TRH stimulated TSH levels were determined after one, two and four weeks. A gradual increase of the mean basal and TRH stimulated TSH plasms concentration could be observed. However, after four weeks the TRH test was still negative in two patients and weak positive in another case. T3 plasma levels were lowest after one week which might indicate the presence of an intermittent thyrotropic insufficiency. It is concluded that the hormone-free interval should last four weeks at least before a reliable TRH test can be obtained.

Goiter↗

Effects of subclinical hyperthyroidism on renal handling of water and electrolytes in patients with nodular goiter.

Evidence is beginning to accumulate that minor degrees of hyperthyroidism lead to adverse effects in various tissues, even though clinically the patients are euthyroid. To determine whether these anomalies in thyroid function have deleterious effects on renal function and electrolyte metabolism, the plasma concentrations of electrolytes, urea, and creatinine, the renal handling of water and sodium, and the urinary excretion of these substances were measured in patients with nodular goiter who were displaying stable subclinical hyperthyroidism. The studies were carried out before and after correcting the thyroid dysfunction. Restoration of euthyroidism did not modify any of the renal function parameters studied and did not cause changes in blood analyte levels. The data show that treatment of minor degrees of hyperthyroidism does not have any effects on renal function and electrolyte metabolism, and confirm the well-known capacity of the kidney to adjust its functions to changes induced by an abnormal secretion of thyroid hormones.

Adult↗

Ultrasound and MRI findings in a case of childhood amyloid goiter.

Goiter secondary to amyloidosis is rare in clinical practice and only a few descriptions of its radiologic features have been reported. We present the ultrasound and MRI findings of thyroid amyloidosis in a 7-year-old Turkish boy with familial Mediterranean fever.

Amyloidosis↗

Amyloid goiter in a child--US, CT and MR evaluation.

There are few radiological descriptions of amyloid goiter, basically in adult patients of oriental origin. We present a ten-year-old boy with Still's disease and secondary thyroid amyloidosis, describing the US, CT and MR findings.

Amyloidosis↗

Enhanced suppression of 1,25(OH)2D3 and intact parathyroid hormone in Graves' disease as compared to toxic nodular goiter.

1.25(OH)2D3, 25OHD3, and intact parathyroid hormone, as well as various parameters of calcium-phosphorus metabolism were measured in 38 patients with Graves' disease (GD) and in 24 patients with toxic nodular goiter (TNG). Plasma 1.25(OH)2D3 levels were lower in GD patients (82 +/- 29 pmol/liter) than in those with TNG (155 +/- 32 pmol/liter) (P less than 0.0005). The mean value of 1.25(OH)2D3 in 45 controls was intermediate between the two groups of patients (140 +/- 41) and the difference was statistically significant. GD patients before and after treatment had higher alkaline phosphatase (P less than 0.05), lower intact parathyroid hormone (PTH) (P less than 0.05), and lower 1.25(OH)2D3 levels (P less than 0.0005 in the hyperthyroid and P less than 0.01 in the euthyroid state) than TNG patients. We conclude that increased skeletal calcium resorption is due to elevated levels of T3 causing suppression of 1.25(OH)2D3 production and of PTH levels in both groups of patients albeit of different degrees. Furthermore, we postulate that the profound suppression of 1.25(OH)2D3 in GD is secondary to an immune-mediated phenomenon.

Calcitriol↗

Limited value of delayed radiothallium image in the diagnosis of nodular goiter.

To test whether delayed thallium image can improve the diagnostic accuracy of thyroid tumor, we have reviewed 35 patients with cold nodules of proven histopathology. Early and delayed images were taken 10-20 min and 3-4 hr postinjection, respectively. In this study, a thyroid nodule was interpreted as malignant if it had normal or increased uptake on early scan and more residual radioactivity than paranodular tissue on delayed scan. In the eighteen patients who had carcinoma, a disappointing 44% false negative rate was observed. No significant difference was seen between retention-positive and negative cases in tumor size or histological type of cancer. Nor could any demographic bias explain the low sensitivity. Of the six false negatives, macroscopic cystic degeneration was seen in two cases, but tumors in the other four were grossly solid. Considering the histological heterogeneity often seen within a thyroid tumor, the portion with increased retention of radiothallium may be too small to be detected in the false negative cases. Furthermore, 3 false positive readings were obtained in 17 patients with benign conditions. We conclude that the contribution of the delayed thallium image was quite limited in predicting or ruling out malignancy in nodular goiters.

Adult↗

Intrathoracic retroesophageal goiter causing tracheal stenosis.

A 65-year-old woman presented with stridor revealed a mass on a chest X-ray on physical examination. A huge goiter arising in the left lobe of the thyroid had extended retroesophageally and across the midline to the right side of the posterior mediastinum far caudally down to the level of the carina. The trachea was remarkably compressed. Surgery was performed via a combined thoracic and cervical approach, and the tumor was completely removed with resultant relief from stridor. The patient is presently doing well at 1 year after the operation.

Aged↗

Pyridostigmine and metoclopramide do not restore the TSH response to TRH inhibited by L-thyroxine treatment in children with goiter.

To define the role of somatostatin and dopamine in TSH suppression induced by L-thyroxine, 16 children (12 F, 4 M) on suppressive doses of L-thyroxine (3-4 microg/kg/day) for endemic goiter were studied. Firstly a conventional TRH test was performed in all subjects, in order to evaluate TSH, PRL and GH (basal study). A week later a second TRH test was carried out; one hour before the test, however, group A (9 patients) was given 60 mg pyridostigmine bromide po (pyridostigmine study) and group B (7 patients) 10 mg metoclopramide po (metoclopramide study). In the basal study, TSH was suppressed in both groups and levels did not increase following TRH administration, while PRL increased significantly and GH levels remained stable. In the pyridostigmine study, TSH levels did not increase following TRH administration, while PRL and GH levels were both significantly raised. In the metoclopramide study, TSH and GH levels were not raised following TRH administration, while a significantly greater increase of PRL was observed. In conclusion, suppressive doses of L-thyroxine inhibit the TSH response to TRH, while they do not seem to affect GH and PRL secretion. Somatostatin and/or dopamine do not seem to play a significant role in the L-thyroxine-induced TSH suppression.

Adolescent↗

Iodized oil treatment for endemic goiter does not induce the surge of positive serum concentrations of anti-thyroglobulin or anti-microsomal autoantibodies.

Forty-three goitrous patients (grade II and III, WHO classification), living in areas of chronic iodine deficiency, were treated with an injection of iodized oil (470 mg iodine). Serial measurements of serum thyroid hormone levels after the therapy revealed increasing concentrations of both hormones, with a significantly lower serum T3/T4 ratio, and progressively significantly lower serum TSH mean values. Serum Tg mean value, initially elevated (58 +/- 9 ng/ml), decreased after 6 months and returned to the normal range at 36 months of therapy. In none of the examined patients (except for one subject with positive autoantibodies before therapy), it was observed the surge of positive anti-thyroglobulin or anti-microsomal autoantibodies after the iodized oil. We conclude that iodized oil therapy does not induce an abnormal autoimmune reaction in endemic goiter patients.

Adolescent↗