Endemic Goiter in the Uele Region: III. Endemic Cretinism*

Bastenie, Paul Auguste; Ermans, A. M.; Thys, O; Beckers, Christian; Schrieck, H. G. Van Den; Visscher, M. De

doi:10.1210/jcem-22-2-187

Cited by 30 publications

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“…The absence of iodotyrosines in the blood and urine and the normal ratio of B.E.131I to n.B.131I indicate that some at least of the known metabolic defects of sporadic goitrous cretinism are unlikely to be present in our endemic deafmutes. Nevertheless, in the absence of direct tissue analysis, the evidence presented here as well as that of Bastenie et al (1962) (Batsakis and Nishiyama 1962). The thyroid develops first.…”

mentioning

confidence: 57%

"Bound" Complement in the Skin of Patients With Chronic Discoid Lupus Erythematosus and Systemic Lupus Erythematosus

Kalsbeek

Cormane

1964

The Lancet

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confidence: 57%

"Bound" Complement in the Skin of Patients With Chronic Discoid Lupus Erythematosus and Systemic Lupus Erythematosus

Kalsbeek

Cormane

1964

The Lancet

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“…Thyroid function is usually normal (12,23,29,171,177) but can indicate subclinical hypothyroidism with elevated basal TSH or exaggerated TSH response to TRH (173,209). Figure 8 show the clinical aspects of the myxedematous type DELANGE of endemic cretinism as the most typically seen in Zaire (5,39,52,75,76,197). These cretins show less mental retardation than the neurological cretins; they are often capable of performing simple manual tasks.…”

Section: Complicationsmentioning

confidence: 99%

The Disorders Induced by Iodine Deficiency

Delange¹

1994

Thyroid

556

447

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This paper reviews present knowledge on the etiology, pathophysiology, complications, prevention, and therapy of the disorders induced by iodine deficiency. The recommended dietary allowances of iodine are 100 micrograms/day for adults and adolescents, 60-100 micrograms/day for children aged 1 to 10 years, and 35-40 micrograms/day in infants aged less than 1 year. When the physiological requirements of iodine are not met in a given population, a series of functional and developmental abnormalities occur including thyroid function abnormalities and, when iodine deficiency is severe, endemic goiter and cretinism, endemic mental retardation, decreased fertility rate, increased perinatal death, and infant mortality. These complications, which constitute a hindrance to the development of the affected populations, are grouped under the general heading of iodine deficiency disorders (IDD). At least one billion people are at risk of IDD. Iodine deficiency, therefore, constitutes one of the most common preventable causes of mental deficiency in the world today. Most of the affected populations live in mountainous areas in preindustrialized countries, but 50 to 100 million people are still at risk in Europe. The most important target groups to the effects of iodine deficiency from a public health point of view are pregnant mothers, fetuses, neonates, and young infants because the main complication of IDD, i.e., brain damage resulting in irreversible mental retardation, is the consequence of thyroid failure occurring during pregnancy, fetal, and early postnatal life. The main cause of endemic goiter and cretinism is an insufficient dietary supply of iodine. The additional role of naturally occurring goitrogens has been documented in the case of certain foods (milk, cassava, millet, nuts) and bacterial and chemical water pollutants. The mechanism by which the thyroid gland adapts to an insufficient iodine supply is to increase the trapping of iodide as well as the subsequent steps of the intrathyroidal metabolism of iodine leading to preferential synthesis and secretion of triiodotyronine (T3). They are triggered and maintained by increased secretion of TSH, which is ultimately responsible for the development of goiter. The acceleration of the main steps of iodine kinetics and the degree of hyperstimulation by TSH are much more marked in the pediatric age groups, including neonates, than in adults, and the development of goiter appears as an unfavorable side effect in the process of adaptation to iodine deficiency during growth. The most serious complication of iodine deficiency is endemic cretinism, a syndrome characterized by irreversible mental retardation together with either a predominant neurological syndrome or predominant hypothyroidism, or a combination of both syndromes.(ABSTRACT TRUNCATED AT 400 WORDS)

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“…We were then faced with more difficulties. (1) There were many other questions raised during our first discussion in 1965, with the Belgian investigators who had seen a different clinical picture (Bastenie et al, 1962). The abnormalities observed by them conformed with the myxoedematous type of endemic cretinism described by McHarrison.…”

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confidence: 65%

Endemic cretinism—a continuous personal educational experience during 10 years

Querido

1975

Postgraduate Medical Journal

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The processes involved in the progress of scientific knowledge have fascinated philosophers, scientific workers and non-scientists. Kuhn (1970), in his interesting essay on the structure of scientific revolutions, sees the road which the growth of scientific knowledge follows, not marked by discoveries but by what he names paradigms, ‘theories that gain their status because they are more successful than their competitors in solving a few problems …’ which are recognized as acute. Examples of paradigms in biology are, e.g. Berthold’s conceptualization of endocrinology in 1849; Mendel’s theory of inheritance; Medawar’s concept of immunological tolerance, etc.

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Endemic Goiter in the Uele Region: III. Endemic Cretinism*

Cited by 30 publications

References 0 publications

"Bound" Complement in the Skin of Patients With Chronic Discoid Lupus Erythematosus and Systemic Lupus Erythematosus

"Bound" Complement in the Skin of Patients With Chronic Discoid Lupus Erythematosus and Systemic Lupus Erythematosus

The Disorders Induced by Iodine Deficiency

Endemic cretinism—a continuous personal educational experience during 10 years

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