Cretins, goitrous

M9. McGirr, E. M., Hutchison, J. H., and Clement, W. E., Sporadic goitrous cretinism. Dehalogenase deficiency in the th3rtoid gland of a goitrous cretin and in heterozygous carriers. Lancet 2, 823-826 (1959). 

J. Wolff, R.H. Thompson and J. Robbins, Congenital goitrous cretinism due to absence of iodide-concentrating ability, J Clin Endocrinol Metab 24 699-707 (1964). 

The serum concentration of Selenium is lower in inhabitants of the former goitrous area than in those of the non-goitrous area of Idjwi Island in Zaire (45). It has been speculated that Selenium deficiency could cause oxygen derivatives such as H2O2 to be toxic for the thyroid, and could be one of the factors responsible for thyroid damage resulting in myxedematous cretinism. 

Goitrous endemia... First degree, goiter the lesion is mainly thyroidal Second degree, cretinism the lesion is preferably pluriglandular... 

Supplying a missing protein or metabolite if its production is blocked, e.g. injections of antihaemophilic globulin to haemophiliacs, thyroxine administration to goitrous cretins, blood transfusion for thalassaemic patients. 

Deficiency of iodine affects many millions of people living in upland areas over limestone soil in some areas of central Africa, Brazil and the Himalayas more than 90% of the population may have goitre due to iodine deficiency (section 11.15.3.3). Where children are iodine deficient both in utero and post-natally, the result is severe intellectual impairment - goitrous cretinism. 

Thyroid hormone regulates metabolic activity, and people with thyroid deficiency have a low metabolic rate (section 5.1.3.1), and hence gain weight readily (section 6.3.3.7). They tend to be lethargic and have a dull mental apathy. Children born to iodine-deficient mothers are especially at risk, and more so if they are then weaned onto an iodine-deficient diet. They may suffer from very severe mental retardation (goitrous cretinism) and congenital deafness. 

The problem of widespread iodization of foods in areas of deficiency is that adults whose thyroid glands have enlarged, in an attempt to secrete an adequate amount of thyroid hormone despite iodine deficiency, now become hyperthyroid. This is considered an acceptable risk to prevent the much more serious problems of goitrous cretinism among the young. 

The first records of goiter and cretinism date back to ancient civilizations, the Chinese and Hindu cultures and then to Greece and Rome. In the Middle Ages, goitrous cretins appeared in the pictorial art, often as angels or demons. The first detailed descriptions of these subjects occurred in the Renaissance. The paintings of the madonnas in Italy so commonly showed... 

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