cretinism(redirected from Congenital iodine-deficiency syndrome)
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cretinism(krē`tənĭz'əm), condition produced in infants and children due to lack of thyroid hormone. It usually results from a congenital defect (e.g., absence of the thyroid, presence of only a rudimentary gland, inability of the gland to produce thyroxine). However, it can develop later if there is a lack of iodine in the diet, or if the thyroid is diseased or surgically removed. Cretinism causes very serious retardation of physical and mental development; if the condition is left untreated, growth is stunted and the physical stature attained is that of a dwarf. In addition, the skin is thick, flabby, and waxy in color, the nose is flattened, the abdomen protrudes, and there is a general slowness of movement and speech. If discovered early enough and treated with thyroid extract and sufficient iodine intake throughout life, growth may become normal and mental facility greatly improved. If the condition commences after adulthood is reached it is called myxedemamyxedema
, condition associated with severe hypothyroidism and lack of thyroid hormone in the adult. In the child it is known as cretinism. Symptoms include a dry swelling of the skin, slowed speech and mental awareness, deepened voice, intolerance to cold, fatigue and weakness,
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a disease marked by arrested physical and mental development and thyroid dysfunction.
Cretinism may occur in the form of endemics (endemic cretinism) or individual outbreaks (sporadic cretinism). It is prevalent mostly in mountainous areas, such as the Alps, Pyrenees, Himalayas, and Cordilleras (South America); there is no exact information about the spread of cretinism in Africa. In prerevolutionary Russia, cretinism occurred in the Caucasus, the Urals, Byelorussia, and the Carpathians. During the years of Soviet power as a result of treatment and preventive measures the number of cretins decreased sharply.
Cretinism is caused by a natural (exogenous) deficiency of iodine in water and food or by an endogenous deficiency of the element. The average height of adult cretins is 146.7 cm for males and 140 cm for females. They have short limbs, curved legs, wide hands with short fingers, a round skull, a puffy face with a low forehead, a saddle-shaped nose, deep eye sockets, large ears, a wide tongue too large for the mouth, short sparse teeth, pale dry skin, and little hair. Umbilical and inguinal hernias and enlargement of the thyroid are common. The genitalia are underdeveloped. Metabolism is greatly impaired; the body temperature is low. Poor hearing (often deaf-mutism) is marked and permanent. Mental retardation in pronounced cretinism is at the level of idiocy. Cretins are slow-moving, sleepy, and halting of speech. The acquisition of knowledge and work skills is a difficult and time-consuming process.
Treatment involves the administration of drugs that compensate for the thyroid deficiency (thyroidin or triiodothyronine). If therapy is started in early childhood, cretins can learn how to take care of themselves and do simple repetitive work. Prevention requires the iodination of water and food.
N. D. MIKERINA