This form is regarded as a major public health problem in many countries. There are two main types of cretinism 9). Thyroid hormone plays a major role in the growth, branching and myelination of neuronal cells of CNS at fetal and neonatal stage. 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. Distribution of urinary iodine concentrations in 557 subjects from 12 villages in Lhasa Prefecture, Tibet. In iodine-deficient regions where goitrogens are present, but selenium nutritional status is adequate, such as in the Darfur region of Sudan (Moreno-Reyes et al., unpublished data), neurological cretinism is the most prevalent form of cretinism (Moreno-Reyes et al., 1993). ScienceDirect ® is a registered trademark of Elsevier B.V. ScienceDirect ® is a registered trademark of Elsevier B.V. URL: https://www.sciencedirect.com/science/article/pii/B9781845697525500053, URL: https://www.sciencedirect.com/science/article/pii/B978012375083900163X, URL: https://www.sciencedirect.com/science/article/pii/B9780123741356000649, URL: https://www.sciencedirect.com/science/article/pii/B9780128028612000146, URL: https://www.sciencedirect.com/science/article/pii/B9780857090041500219, URL: https://www.sciencedirect.com/science/article/pii/B9780123741356000716, URL: https://www.sciencedirect.com/science/article/pii/B9780123741356000790, URL: https://www.sciencedirect.com/science/article/pii/B9780123741356000625, URL: https://www.sciencedirect.com/science/article/pii/B978012373960500647X, URL: https://www.sciencedirect.com/science/article/pii/B9780081005965219410, Lifetime Nutritional Influences on Cognition, Behaviour and Psychiatric Illness, Iodine: Deficiency Disorders and Prevention Programs, Encyclopedia of Human Nutrition (Third Edition), Experimental Models of Iodine Deficiency and Cretinism during Development, Maria-Jesus Obregon, ... Gabriella Morreale de Escobar, in, Morreale de Escobar and Escobar del Rey, 2003, Public Health Nutrition in Developing Countries, Iodine, Selenium Deficiency and Kashin–Beck Disease, Evaluating the Efficiency of Iodization Programs, The epidemiological demonstration of the association of. The most important endocrine gland of our body which is present in the neck region is known as thyroid gland. Because selenium deficiency impairs the conversion of T4 to T3 in the periphery, preserving maternal levels of circulating T4, this mechanism was proposed to supply the necessary T3 to the fetal brain during the critical period when the maturation of the nervous system depends mainly on maternal T4. A great decline in goiter was also seen among schoolchildren in the Canton of Valais, where the problem was very severe (Table 79.2). It is present from birth. The word cretin is termed as the iodine deficiency disorder which is associated with insufficient intake of the thyroid hormone activity that occurs during the fetal, infant or childhood phases. (1987) hypothesized that the thyroid atrophy of myxedematous cretins could result from the toxic effect of increased H2O2 generation by the thyrocytes due to chronic TSH stimulation induced by iodine deficiency. prevalence of goitre > 5 %) and severe iodine deficiency; (ii) clinical symptoms which includes some form of mental deficiency and/or defects in hearing, speech, stance, gait, hypothyroidism, and stunted growth; and (iii) when iodine deficiency is corrected in the area cretinism is no longer observed (WHO, UNICEF and ICCIDD, 2001). Thyroid Parenchymal defects: Thyroid hormone also plays a critical role in skeletal muscle development than soft tissue development. 1. This has been followed by many subsequent studies to date (Hetzel, 1989). This mainly due to the defect in the genes which is responsible for encoding the various enzymes involved in the thyroid hormone synthesis such as thyroglobulin, iodotyrosine deiodinase and also thyroid-stimulating hormone. Clinical features in neurological cretinism and in untreated congenital hypothyroidism. Use of antithyroid drugs or sulphonamides during pregnancy. Thiocyanate resulting from consumption of poorly detoxified cassava is a well-known goitrogen in Central Africa. Iodine deficiency is well known as cause of cretinism in fetus and newborn, characterized by low birth weight, short stature, mental retardation, deaf, and/or mutism. Iodine deficiency is the single most common cause of preventable mental retardation in the world. If this condition is not treated in time, it may result in severe stunted mental and physical growth of a baby. Cretinism arises due to deficiency of iodine from the body. Cretinism (n.). Ultimately, public education serves to solidify support for IDD elimination at all levels of the society and thereby creates a demand for iodized salt, a necessary component for the success of a USI strategy and for ensuring sustained demand and production of iodized salt. Iodine deficiency has been recognized as the most frequent cause, after starvation, of preventable mental defects, affecting hundreds of million people to different degrees. Nonetheless, the lack of iodine in the diets of pregnant women in the first trimester appears to be a common factor in both forms of cretinism, suggesting that maternal hypothyroidism is responsible for irreversible damage to the foetal brain. Facts about Thyroid Gland and Thyroid Hormone. The medical condition cretinism is distinguished into two types: Congenital Cretinism: The rate of incidence is 1:3000 to 1:4000 in an iodine-deficient endemic area. Most people tolerate to high dose of iodine intake without any clinical symptoms (Bürgi, 2010). Copyright © 2020 Elsevier B.V. or its licensors or contributors. Congenital cretinism with severe hypothyroidism can be identified by antenatal screening test in the first month of life. However, following the introduction of iodized salt, the prevalence of goiter fell sharply. Elevated serum Thyroid binding globulin (TBG) and TSH level. Recommended dose is 10 to 15ug per kg body weight. Cretinism definition is - a usually congenital condition marked by physical stunting and intellectual disability and caused by severe hypothyroidism. This mainly due to the defect in the genes which is responsible for encoding the various enzymes involved in the thyroid hormone synthesis such as thyroglobulin, iodotyrosine deiodinase and also thyroid-stimulating hormone. A noncancerous enlargement of the thyroid gland, visible as a swelling at the front of the neck, that is often associated with iodine deficiency. Iodine deficiency is often cited as the most common cause of hypothyroidism worldwide but it can be caused by many other factors. Congenital hypothyroidism, previously known as cretinism, is a severe deficiency of thyroid hormone in newborns. This medical condition affects the humans differently according to the age it is being affected. Reproduced with permission from Hetzel and Pandav (1996). Experimentally, the involvement of thiocyanate in the pathogenesis of myxedematous cretinism has recently been corroborated (Contempre et al., 2004). Figure 71.4. 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). To convert values for urinary iodine to nanomoles per liter, multiply by 78.7. A detailed review by Delange (1994) indicates extensive evidence in many endemic diseases of low intelligence quotient (IQ) in populations living in areas with severe iodine deficiency areas (Figures 62.1–62.3). Further, the nature of the neurological deficit associated with both types of endemic cretinism has received scant attention in recent times considering that it remains a common disorder in many parts of the world. A summary is presented in Table 64.1. About one billion people worldwide risk the consequences of iodine deficiency, all of which can be prevented by adequate maternal and infant iodine nutrition. Cretinism: Congenital hypothyroidism (underactivity of the thyroid gland at birth), which results in growth retardation, developmental delay, and other abnormal features. It can be caused due to a defect in the genes encoding various enzymes involved in thyroid hormone synthesis such as thyroglobulin, iodotyrosine deiodinase and … The cause of thyroid failure and the higher prevalence of myxedematous cretinism in Central Africa has been an unsolved problem for quite a long time. Experimental studies showed that selenium deficiency in rats decreases D1 and increases serum T4 (Arthur et al., 1988; Beckett et al., 1987). The clinical features of people affected by neurological cretinism are completely different from those affected by congenital hypothyroidism (CH) (Boyages et al., 1988; Boyages and Halpern, 1993). Cretinism is a type of mental retardation syndrome that is caused by a thyroid deficiency. Normally, high dose iodine intake transiently inhibits thyroid peroxidase activity and hormone production, known as acute Wolff-Chaikoff effect, followed by the decrease of sodium-iodide symporter expression hence decrease of intrathyroidal iodine concentration which, at some point, will re-trigger thyroid hormone production again. A condition caused by thyroid underaction from severe iodine deficiency early in life. Figure 71.2. It has been suggested that cassava may be the trigger resulting in thyroid involution in this region (Delange et al., 1972; Ngo et al., 1997). Neurological cretinism is characterised by mental retardation, deaf mutism, squint, spastic diplegia, and disorders of … Another factor may explain why the condition is limited to a few endemic goiter areas. Dr Adil Ramzan. M.B. Soon afterwards, D1 was found to be a selenoenzyme (Behne et al., 1990). Enlarged nodular thyroid gland represents the upregulate activity of follicular cell to maintain thyroid hormone level adequately despite scarcity of substrate. Pro Lite, CBSE Previous Year Question Paper for Class 10, CBSE Previous Year Question Paper for Class 12. The lack of goitrogens in Tibet probably explains the lower frequency of hypothyroidism and goiter despite similar urinary iodine concentrations to those in Central Africa. Reproduced from Hetzel (1989). Later, deaf and dumb institutions were closed and diverted to other institutions. There are two main types of cretinism (Hetzel et al., 1990; Chen and Hetzel, 2010). Cretinism is the most serious IDD and occurs when a pregnant women is severely iodine deficient. The correction of selenium deficiency before iodine deficiency may thus aggravate hypothyroidism, in particular in pregnant women, which may increase the frequency of neurological cretinism (Contempre et al., 1991, 1992; Vanderpas et al., 1993). These long-lasting injections were shown to correct iodine deficiency and prevent goiter for 3 to 5 years depending on the dosage. It is marked by a series of abnormal effects, such as mixed (dry) skin, unusual hair and a protruding abdomen. A cretin is a person affected by cretinism. The first records of goiter and cretinism date back to the ancient civilizations, the Chinese and Hindu cultures and then to Greece and Rome. Iodine intake as low as 25 mcg/day during pregnancy would be a potential risk factor for giving birth to cretinism neonates. The term parliamentary cretinism was introduced by Marx. Iodine is an essential trace element; it is necessary and required for the synthesis of thyroid hormones. In the landmark trial of 165 000 people living in an area of Papua New Guinea with severe iodine deficiency and endemic cretinism (Pharoah et al., 1971), families were allocated to iodine (iodised oil) or placebo (saline) and subsequent follow-up studies of the original cohort found that an injection of iodised oil before conception or in early pregnancy reduced the incidence of cretinism and improved the motor and cognitive functions of children compared with placebo treatment. A study of 112 cretins (neurological, myx-oedematous, and mixed) living in Thailand reported that their mean IQ score was 30.8 ± 8.8 (Rajatanavin et al., 1997). Neurological cretinism is characterized by mental retardation, deaf mutism, … Cretinism athyreotic: A rare form of congenital hypothyroidism that causes mental and physical growth retardation in infants or children. In Table 64.2 we summarize the situations in which brain damage has been associated with thyroid hormone deficiency during development: iodine deficiency, CH, maternal hypothyroxinemia, combined maternal and fetal hypothyroidism, prematurity and thyroid hormone resistance syndromes. Goiter and cretinism provided the visual picture of iodine deficiency that gave it easily identifiable reference. This form is regarded as a major public health problem in many countries . It causes impaired neurological function, stunted growth, and … Cretinism: Congenital hypothyroidism (underactivity of the thyroid gland at birth), which results in growth retardation, developmental delay, and other abnormal features. autoimmune thyroid disease, Use of I131 or antithyroidal treatment for Graves'disease, postpartum thyroiditis, amiodarone-induced thyrotoxicosis type 2, may impair escape mechanism and lead to prolonged hypothyroidism after high dose iodine exposure (Leung and Braverman, 2014). 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). In this case, the response obtained is partial in which the physical development and growth are revived, and additional mental retardation is checked. Venkatesh Mannar, in Public Health Nutrition in Developing Countries, 2011. 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 thyroglobulin is synthesized in the follicular cells and released into the follicular lumen. Cretinism can be due to deficiency of iodine in the mother's diet during pregnancy. Endemic cretinism arises from a diet deficient in iodine and has affected far more people worldwide and continues to be a major public health problem in many countries. Vedantu academic counsellor will be calling you shortly for your Online Counselling session. Thyroid hormone plays an important role in maintaining basal metabolic rate, oxidation food stuffs and growth and development at an early stage of life. This gland is responsible for the secretion of thyroid hormones such as triiodothyronine (T3) and tetraiodothyronine (T4). Cretinism is a medical condition caused by an untreated congenital thyroid hormone deficiency. A series of 18 studies of the effects of iodine deficiency on IQ, reviewed by Bleichrodt and Born (1994), revealed a mean loss of 13.5 IQ points in iodine-deficient populations compared to carefully matched iodine-sufficient populations. Conditions in which CNS damage has been associated with thyroid hormone deficiencies early in development and their prevention. As IDD elimination progressed, these physical manifestations became far and fewer giving the impression that IDD problem had been solved. This term has come to be regarded as insulting in the general population, although it is appropriate within technical settings. Cretinism has been suggested to be the severe end of a spectrum of effects on cognition that continues in varying degrees of intellectual impairment to normality as iodine status improves. Goitre and cretinism provided the visual picture of iodine deficiency that gave it an easily identifiable reference. Subsequently, similar models showing the effects of severe iodine deficiency on brain development were developed in the primate marmoset monkey and in the rat. The numbers on the horizontal axis represent the midpoint of the intervals shown. Prompt thyroid hormone therapy is essential in order to prevent progressive neurological and motor deterioration. The maternal thyroid hormone is the only source for the fetus that crosses the placenta for fetal brain development. Table 62.2. Myxedematous and neurological cretinism are the most severe consequences of iodine deficiency. This disorder is sometimes caused during fetal development, when the thyroid is underdeveloped, but can also be caused in adulthood from injury to the thyroid gland. A hypothyroid cretin from Sinjiang, China, who is also deaf mute. This work led to Hetzel (1983) proposing a new concept of IDD, the result of all the effects of iodine deficiency on growth and development, particularly brain development, in an exposed population, that IDD can be prevented by correction of the iodine deficiency. Cretinism is classified into the following two types: Endemic cretinism. Three-year-old boy from a severely selenium-deficient area of Tibet with neurological cretinism. To convert values for serum selenium to nanomoles per liter, multiply by 12.7. For more information, we refer to several reviews on the effects of iodine deficiency on brain development (DeLong, 1989; Morreale de Escobar et al., 1989, 1997, 2004; Morreale de Escobar and Escobar del Rey, 2003; Obregon et al., 2005). In New Guinea (Choufoer et al., 1965) and South America (DeLong et al., 1985) neurological cretinism used to be the most frequent form. This type of cretinism majorly affects the children who live in the geographical settings where iodine is deficient, and it is not corrected by either supplementing iodine or thyroid hormone to regain normal thyroid hormone levels during early life. The low serum selenium found on Idjwi Island (Goyens et al., 1987; Vanderpas et al., 1990) where myxedematous cretinism was prevalent supported this hypothesis. Cretinism that affects the fetus during pregnancy stage can cause neurological cretinism. Reproduced with permission from Moreno-Reyes et al., (1998). Thus, on-going communication efforts are necessary. Replacement of the hormone will produce an immediate effect. Chronic low iodine intake can result in persist increase of thyroidal iodide uptake and autonomous thyroid nodules. In adults generally the required dietary intake of iodine is 150µg/ day. It is one cause of underactive thyroid function at birth, called congenital hypothyroidism, and also referred to as cretinism. Mass prophylaxis of goiter with iodized salt was first introduced in Switzerland and in Michigan, USA, in the 1920s. If this condition is not treated in time, it may result in severe stunted mental and physical growth of a baby. The term is a derivative of the French word chrétien which means “one who is human despite deformities.” Five already pregnant when injected with saline solution. Cretinism is a medical condition present at birth wherein there is a decreased or absent thyroid function and thyroid hormone production . 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. of progeny, Early correction of maternal hypothyroxinemia, Combined maternal and fetal hypothyroidism: severe neurological damage, reminiscent of ID cretinism, Maintenance of normal maternal T4 throughout pregnancy, and prompt postnatal treatment of the newborn with T4, Thyroid hormone resistance syndrome: in many cases, mental retardation, deafness, Prematurity: increased risk of neurodevelopmental impairment and cerebral palsy, Postnatal correction of transient neonatal hypothyroxinemia, 1920 (before introduction ofiodized salt), 1934 (10 years after introductionof iodized salt in 1924). Endemic cretinism has been a common finding in the mountains or isolated regions, such as the Alps or the Himalayas and has been described or depicted in drawings over the centuries. Iodine intake as low as 25 mcg/day during pregnancy would be a potential risk factor for giving birth to cretinism neonates. Figure 71.3. At the border of follicular cells oxidation of iodide into iodine takes place. Iodized salt is usually the preferred prophylactic vehicle, but iodized vegetable oil, iodized water, and iodine tablets are also occasionally used. This abnormal thyroidal tissues lost a normal “Wolff-Chaikoff effect” response when they are exposed to large dose of iodine supplement and may cause hyperthyroidism. Cretinism is a congenital form of deficiency of thyroid hormones, retarding mental and physical growth. Sorry!, This page is not available for now to bookmark. Prompt diagnosis and treatment are important in cretinism since any delay in initial replacement may lead to irreversible damage. This type of cretinism largely occurs in countries with iodine deficiency soils. In most cases, the mothers of endemic cretinism children have been affected with hypothyroidism during pregnancy (Maternal hypothyroidism). So, absence of thyroid hormone at these stages would severely affect the generalized nervous system development. The decreased activity of enzymes, such as GSH peroxidase, which normally protect thyrocytes and other cells from H2O2 toxicity would lead to thyroid necrosis. The absence of goiter in myxedematous cretins is caused by thyroid atrophy (Delange et al., 1972). To further establish the relationship between iodine deficiency and fetal brain development, an animal model was developed in severely iodine-deficient pregnant sheep given an iodine-deficient diet. Congenital iodine deficiency syndrome is a medical condition present at birth marked by impaired physical and mental development, due to insufficient thyroid hormone often caused by insufficient dietary iodine during pregnancy. This type of cretinism has been eradicated, due to early diagnosis of this condition. Cretinism, also known as congenital hypothyroidism (CH), is a condition where a newborn infant does not have a fully functional thyroid gland.While these terms are now used interchangeably, cretinism is medically defined to be an untreated case of CH, with or without a goiter.. In effect, IDD elimination programmes are threatened to be victims of their own success since a deficiency must be continuously addressed or it will re-emerge. The prevalence of goiter and cretinism was high throughout Switzerland because it is in the elevated region of the European Alps. Fig. Although there is overwhelming evidence showing that severe iodine deficiency can cause a variety of adverse effects on neurodevelopment, there is less but growing data to show that moderate to mild iodine deficiency may have detrimental effects. During maternal hyperestrogenism, the TBG (Thyroxine binding globulin) may be elevated and therefore the total T4 (thyroxine or tetraiodothyronine) and T3 (triiodothyronine)may be normal. In effect, IDD elimination programs are threatened to be victims of their own success since a deficiency must be continuously addressed or it will reemerge. Table 64.2. R. Moreno-Reyes, in Comprehensive Handbook of Iodine, 2009. Maria-Jesus Obregon, ... Gabriella Morreale de Escobar, in Comprehensive Handbook of Iodine, 2009. A young cretin with squint, ataxia and mental deficiency from Papua New Guinea. Yet iodine deficiency persists, in its more generic form—brain damage, to which the unborn fetus is especially vulnerable. 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. This underscores the urgency of prevention with iodized salt. The paintings of the Madonnas in Italy so commonly showed goiter that the condition must have been regarded as virtually normal. Hypothyroidism: Hypothyroidism means " Insufficient secretion of thyroid hormones ( T3 and T4 ) by the thyroid gland. This type of cretinism largely occurs in countries with iodine deficiency soils. The effect of iodised oil given during pregnancy and to children up to 2 years of age on neurological outcomes was investigated by comparing treated children with untreated children at two years (Cao et al., 1994), and again when treated children were school-aged (O'Donnell et al., 2002). 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