Juvenile Hypothyroidism Research Articles

Pseudotumor Cerebri Associated with Initiation of Levothyroxine Therapy for Juvenile Hypothyroidism

Pseudotumor CEREBRI (benign intracranial hypertension), a syndrome characterized by increased intracranial pressure without focal neurologic dysfunction, occurs in association with a variety of end...

Clinical recognition of juvenile hypothyroidism in the early stage.

The clinical manifestations of biochemically-confirmed acquired hypothyroidism in nineteen children and adolescents were reviewed to evaluate the symptoms and signs in the early stage of the disease. The group consisted of 14 girls and 5 boys ranging in age from 4 to 15 years. In 13 of the 19 patients with profound biochemical hypothyroidism, classical symptoms were often absent and clinical signs limited. In these patients, hypothyroidism was presumed to have been short in duration and consequence of chronic lymphocytic thyroiditis. In the remaining 6 patients, hypothyroidism was long-standing and clinical presentation classical, including disproportionate weight gain, fatigue, cold intolerance, myxedematous feature, growth retardation and constipation. Goiter was the most consistent finding in both groups and was present in all instances of spontaneously acquired primary hypothyroidism. These observations emphasize the importance of routine examination of the thyroid gland, particularly in preadolescent and adolescent girls. The finding of goiter may be the only detectable sign of hypothyroidism in the early stage of the disease. Biochemical evaluation should not be deferred because of a clinically euthyroid presentation. Replacement therapy must be instituted when findings indicate a hypothyroid state. Failure to recognize and treat hypothyroidism in these pubertal patients may compromise the adult height, since skeletal maturation will proceed under the influence of sex hormones despite the presence of hypothyroidism.

Effect of rendering male rats hypothyroid at different stages of development on adenohypophysial hormone release.

The hypothalamic-pituitary axis was studied in male rats rendered hypothyroid at different stages in their development. Rats were injected with 131I or H2O (controls) within 12 h after birth, at 5 d or 15 d of life. Their thyroid stimulating hormone (TSH) responses to thyrotropin releasing hormone (TRH), their prolactin (PRL) responses to Haloperidol (H) and TRH and their luteinizing hormone (LH) responses to luteinizing hormone-releasing hormone (LH-RH) were then studied at 30 and 75 d of life. Basal and TRH-stimulated TSH release was markedly greater in the rats radiothyroidectomized within 12 h of birth than for the other 3 groups, probably reflecting greater thyroid damage at a critical time in development. The basal serum PRL and the PRL responses to TRH and H were markedly less in the rats rendered hypothyroid within 12 h after birth than in the other 2 groups of rats. Basal and LH-RH induced LH secretion were greater at 30 d in the rats rendered hypothyroid at 5 and 15 d than in the other 2 groups. Although basal LH levels were higher at 75 d in the rats rendered hypothyroid at 5 and 15 d, their LH responses to LH-RH were similar to the controls and rats made hypothyroid within 12 h. The greatest alterations in TSH and PRL secretion occur when animals are rendered hypothyroid very early in neonatal life. The hypergonadotropic state of the animals rendered hypothyroid at 5 and 15 d is reminescent of juvenile hypothyroidism in man.

Reversible morphological alterations of cortical neurons in juvenile and adult hypothyroidism in the rat

We have studied: (i) whether or not hypothyroidism induced experimentally in the adult rat affects the number and distribution of spines along the apical shaft of pyramidal cells from the visual cortex; and (ii) whether treatment with thyroid hormone would reverse such changes. Two experiments were performed. Experiment A involved the study of rats which were thyroidectomized (T¯) at 40 or at 120 days of age, killed at 120 and 220 days of age, respectively, data being compared to those of adequate sex and age-paired controls. Experiment B involved rats which were T¯ at 40 days of age; they were subdivided into two groups, one of which was left untreated and theother received 0.2 μg of thyroxine/100 g body weight/day for 25 days. Both groups were killed at 90 days of age. We found that: (i) T¯ at 40 and at 120 days of age resulted in a decrease of the number of spines and a deragement of their distribution along the shaft, the observed changes being qualitatively comparable to those previously described 24,25 for rats T¯ at 10 days of age; and (ii) preliminary results from experiment B indicate that the observed changes in neuronal morphology might be reversible. It is suggested that age-independent and reversible alterations of learning capacity and electrocortical activity of hypothyroid rats 7,8 might have age-independent and reversible morphological correlates at the level of the cortex.

TRH releases FSH in children: an explanation for the elevated FSH levels in juvenile hypothyroidism

The majority of children with long-standing juvenile hypothyroidism show sexual maturation of characteristic pattern advanced in relation to bone age and mediated by FSH release (N.D. Barnes, A.B. Hayles, R.J. Ryan, Mayo Clin. Proc. 1973, 48, 849). The reason for the FSH release was obscure. We have studied the effect of an intravenous bolus of 200 μg TRH in 13 children (4 boys, 9 girls, age range 2.7 - 17.0 years) without thyroid disease. All showed a response in FSH as well as TSH but none a detectable response in LH. Three girls with primary hypothyroidism showed elevated resting levels of FSH and a further rise after TRH (see table).

Precocious puberty in juvenile hypothyroidism

Three children, two girls and one boy, presented with hypothyroidism and precocious sexual development. Serum TSH and PRL were elevated in all cases. In the male patient, pituitary stimulation with TRH produced exaggerated TSH and PRL responses. Elevated serum LH, FSH, estrone, estradiol, and an attenuated gonadotropin response to GnRH were documented in the male patient and in one of the female patients. Serum testosterone was elevated for age in one of the female patients but prepubertal in the male patient. After treatment with L-thyroxine, elevated hormonal levels decreased to normal in one patient who returned for follow-up. Evidence is presented that precocious puberty in juvenile hypothyroidism may be the result of PRL hypersecretion.

Hypothyroidism in an area of endemic goiter and cretinism in Central Java, Indonesia.

In an area of severe endemic goiter in Central Java, Indonesia, clinical overt or mild hypothyroidism appeared to be present in 7 out of 20 cretins and also in 12 out of 94 non-cretinous subjects, all 5-20 years of age, living in the village of Sengi. Hypothyroidism was not found in a control group of 70 subjects of the same age living in Londjong just outside the edemia. In hypothyroid subjects the plasma PBI-concentration was 0.98+/-0.32 mug/100 ml (mean+/-SD) vs. 2.72+/-1.24 mug/100 ml in euthyroid subjects from Sengi and 4.86+/-0.80 mug/100 ml in controls from Londjong. Values for T3 were 56.3+/-3.17 ng/100 ml in hypothyroids, 140.5+/-38.5 ng/100 ml in euthyroids from Sengi and 121.6+/-27.4 ng/100 ml in controls. The TSH levels (geometric mean and range) in these 3 groups were, respectively, 210.1 (108.0-342), 15.6 (3.0-372) and 4.1 (0.8-7.0) muU/ml. The differences between the mean concentration of PBI, T3 and TSH in the hypothyroid and euthyroid groups were highly significant (P less than 0.001). These data strengthen the clinical diagnosis of hypothyroidism in cretins as well as in non-cretinous subjects. All hypothyroid subjects had a PBI less than 1.8 mug/100 ml and T3 less than 120 ng/100 ml and TSH greater than 100 muU/ml. In 8 hypothyroid subjects, restudied 18 months after iodized oil injection, hypothyroidism was either corrected or markedly improved. It therefore appears that iodine deficiency per se in postnatal life may lead to (juvenile) hypothyroidism, which can be corrected by iodine therapy. Our findings have implications for the definition and diagnosis of endemic cretinism. Not all hypothyroid subjects in an area of endemic iodine deficiency should be classified as cretins.

Congenital and juvenile nongoitrous hypothyroidism

Nongoitrous congenital and juvenile hypothyroidism is a rather common endocrine disorder occurring in approximately 1:5000–1:8500 normal children. The most frequent cause is thyroid ectopy (‘cryptothyroidism’) followed by atrophy of unknown cause and chronic atrophic thyroiditis. The clinical symptomatology is defined by disturbances of general metabolism and, most typically, by retarded somatic and mental development, leading in its extreme form when untreated, to so-called cretinism. The i treatment with thyroid hormone replacement has to be administered as early as possible. The prognosis for mental development depends on the brain damage present when treatment was started. Screening of thyroid function in neonates is the most reasonable method to make an early diagnosis.

Poor Intrauterine Growth and Juvenile Hypothyroidism

Poor Intrauterine Growth and Juvenile Hypothyroidism

Thyrotropin behavior in thyroid disorders of childhood.

Abstract: Through the use of a double antibody radioimmunoassay method, the mean serum concentration of thyrotropin (TSH) in 51 normal children aged 1 month-18 years was 3.7 ng/ml. There was no change with age, sex, or time of the day at which the sample was obtained. In six of nine newly diagnosed hyperthyroid children concentrations of TSH fell below the limit of detection, whereas in the remaining three concentrations were in the low normal range. Knowledge of the TSH fluctuations during medical treatment (antithyroid drugs or radioactive iodine plus 127I) were useful in insuring a smooth clinical course. Elevations in TSH above 10 ng/ml served to indicate a need for change in therapy. Radical subtotal thyroidectomy evoked an acute compensatory rise (> 20 ng/ml) in TSH levels which was sometimes sufficient to maintain the level of serum thyroxine above 4 μg/100 ml. Hemithyroidectomy did not influence TSH or other thyroid parameters in previously euthyroid subjects. There was a clear separation between normal subjects and patients with primary hypothyroidism with values for TSH always exceeding 20 ng/ml in the latter. The TSH levels were below 3.7 ng/ml in pituitary hypothyroidism. In hypothyroid patients receiving adequate replacement therapy, elevations of serum TSH of > 20 ng/ml were encountered 2 weeks after discontinuation of treatment. Investigation of the pituitary-thyroidal axis in two untreated cretinous infants showed preservation of the functional integrity of the pituitary-thyroidal feedback system despite prenatal thyroid insufficiency as evidenced by a fall of TSH from levels >80 to <5 ng/ml in the presence of free thyroxine (FT4) levels >2 ng/100 ml. In three patients (two females and one male) with longstanding juvenile hypothyroidism and TSH levels >80 ng/ml, pituitary dysfunctions characterized by luteinizing hormone (LH) concentrations above 20 mIU/ml and blunted growth hormone (HGH) responses to hypoglycemia were documented prior to therapy. Only the male had evidence of sexual precocity on physical examination. All three showed an abnormal circadian rhythm of cortisol secretion manifested by 8 PM values greater than 10 7mu;g/100 ml. Acute restoration of euthyroidism in the two girls by means of paren-teral thyroxine (T4) improved the HGH response and normalized the cortisol rhythms in 72 hr, coincident with a sharp decline in TSH levels toward normal. In contrast, the high levels of LH declined much more slowly, failing to reach normal for as long as 7 weeks posttreatment. TSH in company with T4 measurements permitted segregation of patients with Hashimoto's thyroiditis into three groups: (a) euthyroid with normal TSH and T4. (b) compensated with TSH values ranging between 19 and 28 ng/ml and T4 in the 6-8 /μg/100 ml range, and (c) hypothyroid with T4 below 3 μg/100 ml and TSH values greater than 40 ng/ml. Speculation: High peripheral concentrations of serum TSH appear to be the most sensitive index of primary hypothyroidism and will thus be useful both in establishing diagnosis and in determining the adequacy of treatment. Elevated gonadotropin levels appear to be the rule in longstanding juvenile hypothyroidism, but physical evidence of sexual precocity seems to be an unusual feature in the syndrome. This may suggest subnormal secretory responsiveness of the gonadal target organs, or subnormal sensitivity of the tissues responsible for secondary sexual differentiation. The observation that patients with cretinism and juvenile myxedema can be safely rendered euthyroid by therapy which abruptly returns serum thyroxine concentration to normal and promptly corrects the associated hypothalamic and pituitary dysfunctions suggests that conventional therapy can be improved. A more aggressive approach to treatment of the infant cretin may reduce the still high incidence of mental subnormality as has been suggested recently [32].

Sexual precocity: An unusual complication of propylthiouracil therapy

A 2 5/12-year-old girl with hyperthyroidism, who was treated with propylthiouracil, became hypothyroid and developed sexual precocity and menstruated. On reduction of the dose of propylthiouracil she became euthyroid, and the signs of sexual precocity disappeared. This appears to be the first report of a form of the syndrome of “precocious menstruation and galactorrhea in juvenile hypothyroidism” brought about by propylthiouracil therapy.

Gastrointestinal Findings in Autoimmune Thyroiditis and Non-Goitrous Juvenile Hypothyroidism in Children

Gastrointestinal and immunological studies were performed on 21 patients with autoimmune thyroiditis and 10 patients with non-goitrous juvenile hypothyroidism. The patients had associated diseases as follows: atopic disease (5), agamma-A-globulinaemia (4), diabetes mellitus (4) and collagen disease (3). Achlorhydria was detected in 5 patients, one of whom had a decreased serum B12 level and an abnormal Schilling test. The gastric mucosa was found to be abnormal in 12 patients, including all 5 achlorhydric patients. Gastric atrophy and atrophic gastritis were each detected in 2 patients, and the remaining 8 patients had superficial and/or interstitial gastritis. Antibodies to gastric parietal cells were detected in 8 patients. None of the patients of this series had antibodies to intrinsic factor. The malabsorption syndrome with villous atrophy was found in 3 patients.

Juvenile hypothyroidism with testicular enlargement.

Juvenile hypothyroidism with testicular enlargement.

Sporadic cretinism and juvenile hypothyroidism

Twenty-five hypothyroid children with onset of disease in infancy and in childhood before 15 years of age from a speciality clinic in a nonendemic area have been reviewed with regard to possible etiology, clinical features and laboratory data. Maldescent of thyroid gland appeared to be the etiology in 44 per cent of the patients studied by isotope scanning. Such cases developed moderate to severe hypothyroidism during infancy or later in childhood or adolescence. Some of these cases had 24-hour thyroidal 131I uptake of less than 10 per cent, a criterion previously described for athyreosis. Development of mental faculties suffered more often and rather severely in those where disease had onset prior to one year of age. Growth and skeletal retardation were present in almost all patients. Excessive weight gain (more than 10 lbs.) was seen only in 40 per cent of the patients. Its absence in other cases may be related to prevailing under-nutrition. Features such as dryness of skin, hoarseness of voice, lethargy, constipation and cold intolerance were present in 65–85 per cent of the cases. Clinically significant retardation in gonadal development was present only in those with a long standing and untreated hypothyroid state. P.B.I. and radioactive iodine thyroid uptake were in definite hypothyroid range in 79 and 65 per cent of the cases, respectively. Serum cholesterol was distinctly elevated in only 35 per cent of the cases. Results of replacement therapy have been quite consistent and somatic growth restored to normality when treatment had been instituted and continued regularly after early diagnosis of hypothyroid state. In patients in whom the onset of hypothyroidism occurred prenatally or during early infancy and those who did not receive replacement treatment within the first few years of onset of disease, the ultimate I.Q. score was not very satisfactory.

Juvenile pernicious anaemia and hypothyroidism. A family study.

A man presented with the sudden onset of pernicious anaemia at the age of 21. He had been treated for hypothyroidism since the age of 6. His father and only paternal aunt were also found to have pernicious anaemia. Gastric and thyroid antibody studies showed one or more antibodies to be present in all three subjects, as well as in the patient's mother.

Effect of lactation and perchlorate on thiocyanate metabolism.

ARTICLESEffect of lactation and perchlorate on thiocyanate metabolismCF Funderburk, and L Van MiddlesworthCF Funderburk, and L Van MiddlesworthPublished Online:01 Dec 1967https://doi.org/10.1152/ajplegacy.1967.213.6.1371MoreSectionsPDF (1 MB)Download PDF ToolsExport citationAdd to favoritesGet permissionsTrack citations ShareShare onFacebookTwitterLinkedInWeChat Previous Back to Top Next Download PDF FiguresReferencesRelatedInformation Cited ByThiocyanate: a review and evaluation of the kinetics and the modes of action for thyroid hormone perturbations6 March 2017 | Critical Reviews in Toxicology, Vol. 47, No. 7Development of a PBPK model of thiocyanate in rats with an extrapolation to humans: A computational study to quantify the mechanism of action of thiocyanate kinetics in thyroidToxicology and Applied Pharmacology, Vol. 307Intake of Iodine and Perchlorate and Excretion in Human Milk17 September 2008 | Environmental Science & Technology, Vol. 42, No. 21Temporal Patterns in Perchlorate, Thiocyanate, and Iodide Excretion in Human MilkEnvironmental Health Perspectives, Vol. 115, No. 2Maternal Thiocyanate and Thyroid Status during Breast-FeedingJournal of the American College of Nutrition, Vol. 23, No. 2Endemic juvenile hypothyroidism in a severe endemic goitre area of SudanClinical Endocrinology, Vol. 38, No. 1ENDEMIC INFANTILE HYPOTHYROIDISM IN A SEVERE ENDEMIC GOITRE AREA OF CENTRAL AFRICAClinical Endocrinology, Vol. 20, No. 3The excretion of sulfur compounds in the urine of newborn infantsMetabolism, Vol. 32, No. 7Accelerated fall in serum bromide level after administration of perchlorate to manEuropean Journal of Clinical Pharmacology, Vol. 16, No. 5Effects of 131I Thyroid Irradiation Damage, Thyroxine, and Thiocyanate on Radioiodine Metabolism in Dairy CowsJournal of Dairy Science, Vol. 52, No. 5TOXICITY OF A TRIMER OF MALONONITRILEThe Lancet, Vol. 292, No. 7570ATRAUMATIC ENDOTRACHEAL-TUBE VALVEThe Lancet, Vol. 292, No. 7570PSYCHIC AND PSYCHIATRICThe Lancet, Vol. 292, No. 7570 More from this issue > Volume 213Issue 6December 1967Pages 1371-1377 Copyright & PermissionsCopyright © 1967 by American Physiological Societyhttps://doi.org/10.1152/ajplegacy.1967.213.6.1371PubMed4294711History Published online 1 December 1967 Published in print 1 December 1967 Metrics

Gozzo con Ipotiroidismo dovuto a Difetto Familiare di Desiodazione delle Iodotirosine

SUMMARYThe Authors describe the case of juvenile hypothyroidism with goiter caused by a congenital defect in the synthesis of the thyroid hormones. The picture of thyroid uptake curve, cromatographic separation of thyroid hormones in serum and of thyroid gland tissue, and the PBI concentration, suggest to include the case among the iodothyrosine dehalogenase defects. Such a defect, though very attenuate, has been found in the patient's mother too. From a genetic point of view, Authors suggest to consider both cases heterozygous expression of the defect; though in the daugther this defect is more accentuate.

PRECOCIOUS SEXUAL DEVELOPMENT

Roentgenographic methods have been used to demonstrate causes of precocious sexual development. Included are abnormalities of the hypothalamus, adrenal gland, gonads, and extraglandular neoplasms such as teratorna and hepatoma. The latter two tumors may secrete androgenic or gonadotropic hormones.Untreated juvenile hypothyroidism in[See Figure in the PDF File]girls has occasionally been associated with sexual precocity and an enlarged sella turcica. The sella returns to normal on prolonged thyroid therapy.McCune-Albright syndrome is characterized by sexual precocity in females, associated with cafe au lait spots and disseminated fibrous dysplasia.

The relationship of juvenile hypothyroidism to chronic lymphocytic thyroiditis

Eighteen cases of acquired juvenile hypothyroidism are described. Significant titers of antithyroid antibodies in sixteen of them suggest an underlying autoimmune thyroiditis. Comparison of these patients with 33 children who have Hashimoto's struma indicates that chronic lymphocytic thyroiditis may produce a spectrum of disease in childhood ranging from euthyroid goiter to nongoitrous myxedema.

"Cryptothyroidism", the major cause of sporadic "athyreotic" cretinism.

Seven patients with consecutively diagnosed “sporadic athyreotic cretinism” were scintiscanned after administration of 131I to determine whether or not any iodineaccumulating tissue was present and whether, if present, it was ectopic, as has been found in some patients by McGirr and Hutchison and others. In 6 of the 7 patients a small rest of radioactivity was found at a high ectopic site despite 24-hr thyroidal 131I uptakes of less than 10% in all patients. There was no evidence of this tissue on physical examination of the pharynx or tongue. Both the most severely affected patients and 2 with the onset after 1 yr of age showed such rests, although on scintiscans the frontal area of the active tissue was slightly larger in the latter. The relation of this finding to the pathogenesis of infantile and juvenile hypothyroidism and to endemic cretinism is discussed. By analogy with cryptorchidism, the term “cryptothyroidism” is suggested as an appropriate designation for this anomaly.