An Introduction to Atomic Number, Isotopes and Isobars, Introduction and Characteristics of Management, Introduction to Organisation and Its Importance, Diseases- Types of Diseases and Their Symptoms, Introduction To Heat, Internal Energy And Work, Vedantu Replacement of the hormone will produce an immediate effect. Cretinism is a type of mental retardation syndrome that is caused by a thyroid deficiency. Neurological cretinism is characterised by mental retardation, deaf mutism, squint, spastic diplegia, and disorders of stance and gait. Soon afterwards, D1 was found to be a selenoenzyme (Behne et al., 1990). Use of antithyroid drugs or sulphonamides during pregnancy. The epidemiological demonstration of the association of cretinism with goiter dates from the Sardinia Commission of 1848. Adequate iodine nutrition is important for the prevention of brain damage that could be irreversible by birth, and is only preventable when administered very early in gestation. The numbers on the horizontal axis represent the midpoint of the intervals shown. The thyroglobulin is synthesized in the follicular cells and released into the follicular lumen. Neurological cretinism is characterized by mental retardation, deaf mutism, squint, spastic diplegia, and disorders of stance and gait. The first detailed descriptions of these subjects occurred in the Renaissance. The birth of cretins could be avoided by the administration of iodine to pregnant women before or early in pregnancy (Pharoah et al., 1976; Wang and Yang, 1985; Cao et al., 1994). This is mainly differentiated between infant period or childhood, and it is preferred to prevent this condition by screening the neonates. To convert values for serum selenium to nanomoles per liter, multiply by 12.7. Congenital hypothyroidism, previously known as cretinism, is a severe deficiency of thyroid hormone in newborns. In children, this condition is developed due to hypothyroidism which induces mental and physical retardation. A decline in the prevalence of goiter and cretinism was also noted by the Sardinia Commission. Later, deaf and dumb institutions were closed and diverted to other institutions. The thyroid atrophy usually occurs near birth, but in some subjects, this process occurs later in life and iodine prophylaxis can prevent it (Vanderpas et al., 1986). The condition is also referred to as congenital hypothyroidism. The word "cretinism" is an old term for the state of mental and physical retardation resulting from untreated congenital hypothyroidism, usually due to iodine deficiency from birth because of low iodine levels in the soil and local food sources. Everyday treatment with thyroxine (8-12 ug/kg) should be started as early as possible as mental retardation that has ensued already is only partially reversible. A further major development was the introduction of injections of iodized oil to correct iodine deficiency in Papua New Guinea for people living in inaccessible mountain villages. Iodine deficiency in diet (Endemic cretinism). This medical condition affects the humans differently according to the age it is being affected. Clinical features in neurological cretinism and in untreated congenital hypothyroidism. Experimentally, the involvement of thiocyanate in the pathogenesis of myxedematous cretinism has recently been corroborated (Contempre et al., 2004). Children born in the western highlands of Papua New Guinea classified according to treatment received by mothera. However, some observations from other goiter endemic areas did not support the selenium–iodine hypothesis. It is one cause of underactive thyroid function at birth, called congenital hypothyroidism, and also referred to as cretinism. It is common in areas with iodine-deficient soils. Excessive intake of iodine can lead to thyroid dysfunction in susceptible individual particular in those with either clinical or subclinical thyroidal abnormalities. McCarrison (1917) studied this condition in the Himalayas, describing two types of cretinism: the nervous and myxedematous types. ), Correction of iodine deficiency before or very early in pregnancy (before midgestation), Congenital hypothyroidism: mental retardation, dwarfism, poor maturation, Maternal hypothyroxinemia: decreased I.Q. This type of cretinism has been eradicated, due to early diagnosis of this condition. Distribution of urinary iodine concentrations in 557 subjects from 12 villages in Lhasa Prefecture, Tibet. Myxoedematous or hypothyroid cretinism is less common and characterised by mental retardation (although less severe than in neurological cretinism), dwarfism, and hypothyroidism with associated physical symptoms (e.g., coarse and dry skin, husky voice, delayed sexual maturation). 5.2. The combination of the controlled human trials with the results of the studies in animal models clearly indicated that prevention was possible by correction of the iodine deficiency before pregnancy. Another factor may explain why the condition is limited to a few endemic goiter areas. In most cases, the mothers of endemic cretinism children have been affected with hypothyroidism during pregnancy (Maternal hypothyroidism). This has been followed by many subsequent studies to date (Hetzel, 1989). Facts about Thyroid Gland and Thyroid Hormone. A physiological state characterized by insufficient production and/or action of thyroid hormone, which can result in a decreased basal metabolic rate, causing weight gain and fatigue. In most cases, the mothers of endemic cretinism children have been affected with hypothyroidism during pregnancy (Maternal hypothyroidism). Cretinism athyreotic: A rare form of congenital hypothyroidism that causes mental and physical growth retardation in infants or children. However, following the introduction of iodized salt, the prevalence of goiter fell sharply. In general, cretinism is associated with higher rates of goiter (around 30%) in a population (Pharoah et al., 1980). Although the major prevalence of iodine deficiency is in the developing countries, the problem continues to be very significant in many European countries as well (France, Italy, Germany, Greece, Poland, Romania, Spain, and Turkey) because of the threat to brain development in the fetus and young infant. This type of cretinism largely occurs in countries with iodine deficiency soils. Other factors such as the presence of goitrogens in the diet, thyroid immunity, and interactions with other trace elements such as selenium have also been postulated to have a role in the development of cretinism (Zimmermann, 2009). Albinism is also associated with vision problems. S.A. Skeaff, in Lifetime Nutritional Influences on Cognition, Behaviour and Psychiatric Illness, 2011. It is common in areas with iodine-deficient soils. On the other hand, certain pre-existing thyroidal illnesses i.e. Amar K. Chandra, in Comprehensive Handbook of Iodine, 2009. Interestingly, the northern part of Idjwi Island showing the highest prevalence of myxedematous cretinism differed from the southern part, precisely because urinary thiocyanate concentrations were significantly higher, while urinary iodine concentrations were no different (Delange et al., 1968). Thyroid hormone plays an important role in maintaining basal metabolic rate, oxidation food stuffs and growth and development at an early stage of life. During cretinism, this disproportionate rate of growth leads to excessive growth of soft tissue compared to skeletal tissues. Iodine deficiency in later period of life causes goiter commonly seen in low iodine intake region. Cretinism, also known as congenital hypothyroidism, is a condition caused by a deficiency of thyroid hormones while a fetus is developing in the womb or shortly after birth. The latter mechanism is considered as an escape phenomenon from Wolff-Chaikoff effect. The apparent spontaneous disappearance of cretinism in Europe has been attributed to an increase in iodine intake (in the absence of iodized salt) as a consequence of dietary diversification due to economic and social development, and with increasing use of iodine supplements, as reported in Switzerland in the 1920s (Burgi et al., 1990). Cretinism: Congenital hypothyroidism (underactivity of the thyroid gland at birth), which results in growth retardation, developmental delay, and other abnormal features. Table 79.2. The condition is also referred to as congenital hypothyroidism. Reproduced from Hetzel (1989). Myxedamatous cretinism: Mental arrest is less severe in this form than in neurological cretinism. This trial proved the causal role of iodine deficiency and was followed by a mass oil injection program. It causes impaired neurological function, stunted growth, and … Non Goitrous congenital hypothyroidism is treated as the most prevalent inborn endocrine disorder. When untreated, this could lead to severely stunted physical and mental growth of the newborn [2]. M.G. Cretinism is a medical condition caused by an untreated congenital thyroid hormone deficiency. Friedrich Engels wrote that it refers to the belief that a simple majority in one's national legislature has the power to direct the future of the whole world in all matters, and even to delimit historical causality authoritatively. Figure 62.1. Figure 71.2. Some countries and regions have a higher prevalence of one type of cretinism than the other, and sometimes the symptoms of both types of cretinism can manifest in the same individual. Exposure to radioactive Iodine during pregnancy may also be the cause in some cases. This term has come to be regarded as insulting in the general population, although it is appropriate within technical settings. Fig. These hormones present inside the follicular cells are synthesized by  iodinating tyrosine residues at various positions in the glycoprotein called thyroglobulin. Myxedematous cretinism was the most prevalent form of cretinism in the Uele region in Central Africa (Dumont et al., 1963). Elevated serum Thyroid binding globulin (TBG) and TSH level. A young cretin with squint, ataxia and mental deficiency from Papua New Guinea. If untreated, it results in mild to severe impairment of both physical and mental growth and development. The absence of myxedematous cretinism in the severely iodine- and selenium-deficient areas of Tibet does not necessarily rule out the role of selenium in the pathogenesis of myxedematous cretinism. Myxedematous and neurological cretinism are the most severe consequences of iodine deficiency. Normal fetal development requires both maternally and fetally produced thyroid hormone. Cretinism (Congenital Hypothyroidism) Prevalence. Goiter and cretinism provided the visual picture of iodine deficiency that gave it easily identifiable reference. Studies done in Papua New Guinea (Choufoer et al., 1965) suggested that the birth of cretins was ­causally related to incapacity to increase plasma T4 (as measured by PBI) during pregnancy, even if the women were not hypothyroid. Thirty-eight percent had serum selenium concentrations <5 ng/ml, 50% had concentrations <8.7 ng/ml and 90% had concentrations <27 ng/ml. 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