Endemic cretinism

P. Pharoah, F. Delange, R. Fierro-Benitez and J.B. Stanbury, Endemic cretinism, "Endemic goiter and endemic cretinism. Iodine nutrition in health and disease", J.B. Stanbury and B.S. Hetzel eds., John Wiley Publ., New York (1980), pp. 395-421. 

Ermans AM, Delange F, Van Der Velden M, et al. 1972. Possible role of cyanide and thiocyanate in the etiology of endemic cretinism. Adv Exp Med Biol 30 455-486. 

Ermans AM, Nbulamoko NM, Delange F, et al., eds. 1980. Role of cassava in the etiology of endemic goitre and cretinism. Ottawa, Canada International Development Research Centre, 1-182. 

The therapeutic strategy should rely on preventative measures. Endemic goitre and endemic cretinism as well as more subtle deficits of neurological function associated with iodine deficiency have been shown to be prevented with adequate iodine prophylaxis. Adequate and continuous supply is the key to successful prevention programmes. 

Delange F. Endemic cretinism. In Braverman LE, Utiger RD, editors. Werner and Ingbar s the thyroid a fundamental and clinical text. 7th ed. Philadelphia (PA) Lippincott Williams Wilkins 1996. p. 756-81. 

In economically deprived communities a method of prophylaxis is to inject iodised oil i.m. every 3-5 years given early enough to women, this prevents endemic cretinism but occasional h5 perthyroidism occurs (see Autonomous adenoma, above). 

Matovinovic, J. (1983). Endemic goiter and cretinism at the dawn of the third millennium. Annu. Rev. Nutr. 3, 341-412. 

Another example of a maternal condition that contributes to birth defects is low circulating iodine. Cretinism is one of the most profound, but completely preventable, syndromes of malformation known. Characteristic consequences of prenatal iodine deficiency include pervasive mental and physical retardation, deaf-mutism (due to primary malformation of the inner ear), lack of muscle tone with a spastic or rigid walk, and failure to attain a height at maturity of more than 1 m. Today, this condition (known as endemic cretinism) is most prevalent in impoverished areas of African and East Asian countries. Prior to implementation of a national program of iodized salt in the early part of the twentieth century, endemic cretinism was commonplace in Switzerland. After institution of iodized salt, deaf-mutism declined 50% within 8 years and no cretins have been born in that country since 1930. 

Severe IDD, indicated by a high prevalence of goiter and endemic cretinism with urine iodine levels < 2.0 jg I L k... 

Suzuki H (1980) Etiology of endemic goiter and iodide excess. In Stanbury JB, Hetzel BS eds. Endemic Goiter and Endemic Cretinism, pp. 237—254. Wiley New York. 

Perhaps the most striking illustration of the pervasive developmental effects of thyroid hormones is provided by children with severe thyroid hormone deficiency from early childhood, a condition termed cretinism. This may either be endemic in regions of severe iodine deficiency or sporadic due to failure of the thyroid to develop normally or defects in the synthesis of thyroid hormone. Affected children are dwarfed with short extremities, have mental retardation, and are inactive and listless. Other manifestations include facial puffiness, enlarged tongue, dry and doughy skin, slow heart rate, and decreased body temperature. Eor full recovery, treatment of patients with cretinism must be initiated before these florid features are apparent. Thus, pregnant women in areas of endemic cretinism due to iodine deficiency are supplemented with iodine and aU newborns are screened for thyroid hormone deficiency in many developed nations. 

In mild and moderate iodine deficiency, endemic cretinism is not observed. However, in a population there is a certain spread of iodine intake, and women at the lower end of the range of iodine intake may still be at risk of insufficient iodine intake during pregnancy. Deficiency of other nutrients and intake of goitrogens from food or from tobacco smoking may increase the risk. 

Stanbury, J.B. and Hetzel, B.S. (1983). Endemic Goiter and Endemic Cretinism Iodine Nutrition in Health and Disease. Wiley Eastern Limited, New Delhi, India. 

Fetus Abortions Stillbirths Congenital anomalies Increased perinatal mortality Endemic cretinism Deaf mutism... 

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