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At least 73 records · Page 4Linked to original sources
PubMed1976

Erythrocyte 2,3-diphosphoglycerate and adenosine-triphosphate in cretins living at high altitude.

A comparison of concentrations of 2,3-diphosphoglycerate (2,3-DPG) and adenosine-triphosphate (ATP) in the red cells of cretins and normal controls living at 3,700 m in the Nepal Himalayas has shown that 2,3-DPG and ATP levels were higher in the cretins. A negative correlation between hemoglobin and 2.3-DPG level was found. Chronic hypoxia appears to have provided the additional stress required to differentiate the significance of thyroid hormone deficiency in producing anemia from its effect on 2,3-DPG levels. If thyroid hormone is in fact one regulator of 2,3-DPG, the anemia of hypothyroidism appears to be more significant. This also suggest that the anemia of hypothyroidism, is at least in part, "pathologic" as opposed to "adaptive".

Adenosine Triphosphate↗
PubMed1997

Early effects of iodine deficiency on radial glial cells of the hippocampus of the rat fetus. A model of neurological cretinism.

The most severe brain damage associated with thyroid dysfunction during development is observed in neurological cretins from areas with marked iodine deficiency. The damage is irreversible by birth and related to maternal hypothyroxinemia before mid gestation. However, direct evidence of this etiopathogenic mechanism is lacking. Rats were fed diets with a very low iodine content (LID), or LID supplemented with KI. Other rats were fed the breeding diet with a normal iodine content plus a goitrogen, methimazole (MMI). The concentrations of -thyroxine (T4) and 3,5,3'triiodo--thyronine (T3) were determined in the brain of 21-d-old fetuses. The proportion of radial glial cell fibers expressing nestin and glial fibrillary acidic protein was determined in the CA1 region of the hippocampus. T4 and T3 were decreased in the brain of the LID and MMI fetuses, as compared to their respective controls. The number of immature glial cell fibers, expressing nestin, was not affected, but the proportion of mature glial cell fibers, expressing glial fibrillary acidic protein, was significantly decreased by both LID and MMI treatment of the dams. These results show impaired maturation of cells involved in neuronal migration in the hippocampus, a region known to be affected in cretinism, at a stage of development equivalent to mid gestation in humans. The impairment is related to fetal cerebral thyroid hormone deficiency during a period of development when maternal thyroxinemia is believed to play an important role.

Animals↗
PubMed2006

On the way to a scientia sexualis: 'On the relation of the sexual system to the psyche in general and to cretinism in particular' (1826) by Joseph Häussler.

At the beginning of the nineteenth century, several works were published by physicians trying to explain, in an increasingly systematic manner, the emergence of psychic derangements with sexual disorders. A contribution to this development was provided by Joseph Häussler with his monograph 'On the relation of the sexual system to the psyche in general and to cretinism in particular' (1826). Häussler was interested in whether the sexual system was the cause of psychic disturbance. He accused some sexual behaviours and exercises (e.g., onanism and unsatisfied sexual instinct) of being causes, but, nonetheless, accepted that morphological deviations of the sexual organs could also evoke mental diseases. Furthermore Häussler attempted to determine if stimulation of the sexual system could cure psychic illnesses. Finally, he dealt with sexual conditions in the mentally ill. A complete chapter was devoted to cretins and their sexuality.

Congenital Hypothyroidism↗
PubMed1977

Age-related change in pituitary threshold for TSH release during thyroxine replacement therapy for cretinism.

Nine patients with athyrotic or ectopic cretinism ages 6 mo.-17 yrs-were studied to examine the relation between age and the quantities of oral L-thyroxine (T4) adequate to restore TSH hypersecretion to normal levels by feedback control. All but one patient had very low levels of endogenous T4 (below 1.0 mug/100 ml) before treatment. However, based on clinical signs and serum T4-1 values, all were judged to be euthyroid or sub-clinically hyperthyroid by L-T4 replacement during the observation period. L-T4 dosage was increased or decreased by 25 or 50 mug/day at 2-12 mo. intervals, and the TSH response to exogenous thyrotropin-releasing hormone (TRH) was tested sequentially at each dose of L-T4 until a normal or slightly suppressed TSH response was obtained The L-T4 dosage which was associated with normal TSH responsiveness to TRH (the adequate L-T4 dose) was high in infancy (10 mug/kg/day), decreasing with age to a level of 3-4 mug/kg/day in pubertal children (correlation coefficient r=-0.820, P less than 0.01). The adequate L-T4 dose observed between 4 and 12 yrs of age was lower than the usually recommended dose (4-6 vs. 7-8 mug/kg/day). On these L-T4 doses, serum T4I concentrations were significantly higher in patients under 5 yrs of age than in older patients (8.3+/-1.5 vs. 6.5+/-0.7 mug/100 ml, P less than 0.02). Serum triiodothyronine (T3) values were also elevated in young children, decreasing with age to the normal range. TSH responses to TRH were completely suppressed at the serum T4-I levels of 9 mug/100 ml and T3 of 250 ng/100 ml or less in 7 patients over 4 yrs of age, but not in 2 younger patients. Although the number of patients examined was limited, these results suggest that the pituitary threshold for feedback regulation of TSH secretion by T4 decreases with age in children with cretinism and the usually recommended replacement doses of L-T4 between 4 and 12 yrs of age are probably overestimated.

Adolescent↗
PubMed1995

Effects of selenium deficiency on thyroid necrosis, fibrosis and proliferation: a possible role in myxoedematous cretinism.

It has been suggested that selenium deficiency is a co-factor to iodine deficiency in the pathogenesis of myxoedematous cretinism. The mechanism proposed is that the generation of hydrogen peroxide is greatly increased in iodine-deficient thyroid glands, and that selenium is involved in the control of hydrogen peroxide and its derived free radicals. This study was carried out to investigate the effect of the possibly impaired cellular defence mechanism associated with selenium deficiency on thyroid necrosis and tissue repair. For this purpose, we studied thyroid tissue from selenium- (SE-) and/or iodine-deficient (I-) rats before and after an acute toxic iodine overload. In I- thyroids, necrotic cells were numerous. Acute iodine administration increased this effect. Necrosis was associated with transient infiltration of inflammatory cells. In I-SE+ thyroids the tissue resumed its normal appearance. In I-SE- thyroid glands, the iodide toxicity was stronger, with greater necrosis and inflammatory reaction. The inflammation resolved but was replaced by fibrotic tissue. Fifteen days after the toxic overload, the connective tissue volume was twice the control value. Before iodide overload, the proportion of dividing cells was equal in I-SE+ and I-SE- thyroids. Three days after the iodide overload, this proportion was increased in I-SE+ thyroids but reduced in the I-SE- thyroids. Overall, the I-SE- thyroids had four times fewer dividing cells than the I-SE+ thyroids. In summary, selenium deficiency coupled to iodine deficiency increased necrosis, induced fibrosis and impeded compensatory epithelial cell proliferation. These results are compatible with histological and functional description of thyroid tissue from myxoedematous cretins.

Animals↗
PubMed1991

Thyroid atrophy in myxedematous endemic cretinism: possible role for growth-blocking immunoglobulins.

We have examined the ability of IgGs obtained from 8 endemic cretins to inhibit TSH-stimulated thyroid cell growth in culture. Clinical and laboratory evidence for hypothyroidism was present in six subjects; the two remaining patients had borderline low serum T4, normal T3 and exaggerated TSH response to TRH. In six patients 2 mg IgG exhibited an inhibitory effect in the cellular growth expressed by a diminished incorporation of 3H-thymidine into the DNA of TSH-stimulated FRTL-5 cells (range: 26-87% inhibition). Seven patients presented clinically with thyroid atrophy of relatively small thyroid enlargements for the degree of chronic iodine deficiency that was present in the area. The remaining subject had a large multinodular goiter and IgG purified from this patient had no inhibitory effect in the FRTL-5 cellular growth. A direct relationship was noted between the degree of thyroid growth inhibition (%) and the basal serum TSH concentration. We conclude that the presence of thyroid growth inhibiting immunoglobulin may be related to the absence of thyroid growth or even thyroid atrophy in endemic cretins.

Adolescent↗
PubMed1992

[The bacteriological revolution and theories of the etiology of goiter and cretinism].

During the 19th century, a large number of researchers produced many different and even contradictory theories about the aetiology of endemic goiter and cretinism. The bacteriological revolution, i.e. the acceptance of the germ theory of infectious diseases, pushed scientists to try to isolate a germ for every disease. In the case of goiter and cretinism, this was fruitless endeavour. Nevertheless, the impact of the bacteriological revolution was largely positive because it provided a new scientific model of the genesis of disease and strengthened confidence in the possibility of fighting endemic as well as epidemic disease by simple but generally applied preventive measures such as, in the present case, iodine prophylaxis.

Bacteriology↗
PubMed1985

[Familial goitrous cretinism with giant goiter and hyperthyroglobulinemia: report of two siblings].

A 39 year-old-man and a 44 year-old-woman who was his elder sister were admitted to our hospital for study and treatment of goiter and hypothyroidism. Both had giant goiters. When he was about 3 years old goiter was first noticed since then the goiter slowly increased in size until the time of admission, while her goiter increased in size from when she was about 30 years old. Though both cases were already recognized as cretinism, they had not been treated. Perchlorate discharge test showed impaired iodide organification in both cases. Their serum thyroglobulin concentration was extremely high (1800 ng/ml). To our knowledge, there have been no case reports of goiterous cretinism due to impaired iodide organification with hyperthyroglobulinemia except the report by Hirota, Y. et al.

Adult↗
PubMed1980

Impaired surfactant production in cretin lambs.

Midgestation ovine fetal thyroidectomy resulted in profound congenital hypothyroidism and severe respiratory distress at term. Amniotic fluid obtained at delivery revealed low lecithin: sphingomyelin (L:S) ratios in all animals with a predominantly acidic phosphatide pulmonary phospholipid profile. Surfactant was diminished in the lung effluent of cretins at birth as compared with normal lambs. The acidic phosphatides and low L:S ratios persisted in the pharyngeal aspirates of the cretin lambs following attempted air breathing and the onset of severe respiratory distress. Conversely, normal lambs released considerable surfactant at birth that contained nonacidic phosphatides dominated by dipalmitoyl lecithin and yielding strongly positive L:S ratios. Comparative histologic studies revealed poor alveolar differentiation in certain lambs and persistence of the midgestation canalicular stage of lung development.

Amniotic Fluid↗
PubMed1993

[A morphologic study on hippocampus in endemic cretinism].

A pathological examination on the hippocampus of brain was carried out on 5 cases of endemic cretinism. Computer image analysis was focused on the pyramidal and granular cells. There was a wide distribution of hypoplasia, and changes of the parameters involved in the pyramidal and granular cells were remarkable, which indicated that a development disorder existed in the hippocampus. Besides, a correlation was noticed between the weights of the thyroid gland and the brain as well as the parameters of the hippocampus. It is also noticed that changes of the thyroid gland correlated with the change in the CNS. Since lesions in the hippocampus were known to be closely related to dementia, it is considered that lesions found in the hippocampus constitute one of the morphologic bases of dementia in endemic cretinism.

Adult↗
PubMedDate not supplied

Decreased growth hormone (GH) response to oral clonidine in endemic cretinism: effect of L-T3 therapy.

As GH secretion is dependent upon thyroid hormone availability, the GH responses to clonidine (150 micrograms/m2) and the TSH and PRL response to TRH were studied in eight endemic (EC) cretins (3 hypothyroid, 5 with a low thyroid reserve) before and after 4 days of 100 micrograms of L-T3. Five normal controls (N) were also treated in similar conditions. Both groups presented a marked increase in serum T3 after therapy (N = 515 +/- 89 ng/dl; EC = 647 +/- 149 ng/dl) followed by a decrease in basal and peak TSH response to TRH. However, in the EC patients an increase in serum T4 levels and in basal PRL and peak PRL response to TRH after L-T3 therapy was observed. One hypothyroid EC had a markedly elevated PRL peak response to TRH (330 ng/dl). There were no significant changes in basal or peak GH values to treatment with L-T3 in normal subjects. In the EC group the mean basal plasma GH (2.3 +/- 1.9 ng/ml) significantly rose to 8.8 +/- 3.2 ng/ml and the mean peak response to clonidine (12.7 +/- 7.7 ng/ml) increased to 36.9 +/- 3.1 ng/ml after L-T3. Plasma SM-C levels significantly increased in N from 1.79 +/- 0.50 U/ml to 2.42 +/- 0.40 U/ml after L-T3 (p less than 0.01) and this latter value was significantly higher (p less than 0.05) than mean Sm-C levels attained after L-T3 in the EC group (respectively: 1.14 +/- 0.59 and 1.78 +/- 0.68 U/ml). These data indicate that in EC the impaired GH response to a central nervous system mediated stimulus, the relatively low plasma Sm-C concentrations, and the presence of clinical or subclinical hypothyroidism may contribute to the severity of growth retardation present in this syndrome.

Administration, Oral↗
PubMed1986

Local cerebral glucose utilization in the adult cretinous rat.

Local rates of cerebral glucose utilization were determined in 5-month-old neonatally radiothyroidectomized and control (littermate) rats. Virtually all 48 brain regions examined in the thyroidectomized rats exhibited lower rates of glucose utilization than those of the controls with differences ranging from -24 to -58%. The decreases were particularly large in the cerebral cortex and throughout the auditory system. Altered patterns in the intrastructural distribution of rates of glucose utilization were seen in a number of regions and were particularly prominent in the hippocampus and inferior colliculus. Lesser changes were seen in hypothalamic regions involved in the synthesis of thyrotropin releasing hormone (TRH). The results indicate that the many structural, functional and biochemical abnormalities of cretinism are associated with widespread reductions in energy metabolism throughout the brain.

Animals↗
PubMed1987

Disease family trees: the possible roles of iodine in goitre, cretinism, multiple sclerosis, amyotrophic lateral sclerosis, Alzheimer's and Parkinson's diseases and cancers of the thyroid, nervous system and skin.

Geographical distribution patterns of incidence and mortality for a wide variety of diseases display strong positive and negative correlations when analyzed statistically. It is argued that these relationships do not occur by chance, but reflect the causal role of surpluses and/or deficiencies of various bulk and trace elements. This concept is explored for one such "disease family tree", that of iodine. Deficiencies of this essential trace element appear to be associated with many diseases, or birth defects, including goitre, cretinism, multiple sclerosis, amyotrophic lateral sclerosis and cancer of the thyroid and nervous system. Although the evidence is weaker, iodine deficiency may also be implicated in Alzheimer's and Parkinson's diseases. In contrast, too much iodine may be linked to elevated mortality from cancer of the skin and melanoma. Rat studies indicate that iodine deficiencies can cause reduced brain weight, limited myelin formation, retarded neuronal maturation, a lowering of the production of various enzymes and slowing of the rates of protein and R.N.A. synthesis. Similar processes appear to occur in many of the diseases identified above.

Alzheimer Disease↗
PubMed1979

Seasonality of birth in sporadic cretinism.

The seasonal distribution of birth dates of 31 patients with sporadic cretinism due to thyroid dysgenesis was analyzed in Osaka area for 14 years. The incidence was statistically high in the summer months. A hypothesis that some environmental factors such as viral infection may cause the disease is proposed.

Birth Rate↗
PubMed1994

Timing of vulnerability of the brain to iodine deficiency in endemic cretinism.

BACKGROUND: Endemic cretinism, caused by severe iodine deficiency during pregnancy, is the world's most common preventable cause of mental retardation. It can be prevented by iodine treatment before conception, but whether it can be prevented or ameliorated by treatment during pregnancy or after delivery is not known. METHODS: In a severely iodine-deficient area of the Xinjiang region of China, we systematically administered iodine to groups of children from birth to three years of age (n = 689) and women at each trimester of pregnancy (n = 295); we then followed the treated children and the babies born to the treated women for two years. We used three independent measures of neural development: the results of the neurologic examination, the head circumference (which correlates with brain weight in the first postnatal year), and indexes of cognitive and motor development. Untreated children one to three years of age, who were studied when first seen, served as control subjects. RESULTS: The prevalence of moderate or severe neurologic abnormalities among the 120 infants whose mothers received iodine in the first or second trimester was 2 percent, as compared with 9 percent among the 752 infants who received iodine during the third trimester (through the treatment of their mothers) or after birth (P = 0.008). The prevalence of microcephaly (defined as a head circumference more than 3 SD below U.S. norms) decreased from 27 percent in the untreated children to 11 percent in the treated children (P = 0.006), and the mean (+/- SD) developmental quotient at two years of age increased (90 +/- 14, vs. 75 +/- 18 in the untreated children; P < 0.001). Treatment in the third trimester of pregnancy or after delivery did not improve neurologic status, but head growth and developmental quotients improved slightly. Treatment during the first trimester, which was technically problematic, improved the neurologic outcome. CONCLUSIONS: Up to the end of the second trimester, iodine treatment protects the fetal brain from the effects of iodine deficiency. Treatment later in pregnancy or after delivery may improve brain growth and developmental achievement slightly, but it does not improve neurologic status.

Brain↗
PubMed2002

Thiocyanate in food and iodine in milk: from domestic animal feeding to improved understanding of cretinism.

Transport of iodine in the mammary gland into breast milk plays a central role in various fields of prevention of thyroid diseases. First, a sufficient content of iodine in the mother's milk is necessary for normal brain development in the breastfed child. This is attained by expression during lactation in the mammary gland of the sodium iodide symporter (NIS), also responsible for iodine transport in the thyroid. Milk iodine content varies with the iodine intake of the mother, and urinary iodine excretion in groups of mothers seems to be a valuable indicator of the iodine status of their breastfed children. Second, iodine in dairy products provides a considerable part of iodine intake in many populations. Thiocyanate from rapeseed feeding of cows decreases milk iodine content, probably by competitive inhibition of NIS in the mammary gland. Alterations in feeding of dairy cows may alter the iodine content of consumer milk, and this may influence the risk of thyroid diseases in the population. Thiocyanate inhibition of iodine transport into milk may also be operative in humans with a high thiocyanate intake. This could further impair iodine status in breastfed children in low-iodine intake areas of the world. It can be speculated that a low-iodine content of mother's milk because of inhibition of NIS in the mammary gland may be one factor of importance for development of myxedematous cretinism.

Animal Feed↗