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© Georg Thieme Verlag KG Stuttgart · New York
Further Studies on Endemic Cretinism in Central Africa
08 January 2009 (online)
The aim of the present work is to describe the epidemiological, clinical and biological characteristics of endemic cretinism on Idjwi Island (Kivu Lake, Republic of the Congo).
The prevalence of cretinism is 1.1% of the total population in the goitrous area of the island. No cretin was found in the non-goitrous area. Only 10% of the Idjwi cretins are clinically euthyroid, most of them being deaf-mute and spastic. The remaining 90% exhibit striking clinical symptoms of hypothyroidism. This diagnosis is confirmed by lower thyroidal radioiodine uptake and PB 127I than in euthyroid controls living in the same area, by marked retardation in bone maturation and by epiphyseal and metaphyseal dysgenesis.
The new points contributed by the present work in the study of African myxedematous cretinism are as follows:
1) The thyroid scannings, which show the presence of small thyroid glands. Some glands appear split and heterogeneous, suggesting a degenerative process.
2) Extremely high blood TSH levels, which demonstrate that the cretins are affected by primary hypothyroidism.
3) X-rays findings, which show that in six out of the eight cretins studied, hypothyroidism appeared around birth or during the first months of life.
This study confirms that most of the African endemic cretins are myxedematous. This could result from a degenerative process of the thyroid occurring around birth or in early infancy. The cause of this phenomenon on Idjwi is still unknown. The action of a toxic agent in the food, in conjunction with the effects of iodine deficiency, might be considered.
Endemic Goiter - Cretinism - Myxedema - Iodine Metabolism - TSH - Epiphyseal Dysgenesis - Thyroglobulin Antibodies
1 Supported in part by International Atomic Agency, Vienna (contract 216/R1/OB), Fonds de la Recherche Scientifique médicale and Institut Belge d'Alimentation et de Nutrition, Belgium, and Euratom, Universities of Pisa and Brussels (Contract BIAC 026-63-4).