Topo-chemical and ultra-structural pathology of family goitres by deficiency of intra and extra thyroidal deiodase.

Topochemical, radio chromatographical, electron microscopic studies about 3 cases of family goitre by deficiency of deiodase. Protein synthesis is almost directed towards cell-division and co-exists with a dishormogenesis, pointed out by extracellular losses of iodotyrosins (MIT and especially DTT), the insufficient synthesis of tyronins (T3 and especially T4) and the inconsistency of the extracellular storage process of thyroglobulin. The alteration of the mitochondrial apparatus shows that, at least partially, the activity of mitochondrial enzyme is deficient. The absence of microvilli, the reduced number of lysosomes, the poorness of the cytoplasmic fundamental matrix are still other factors which point to the existence of an abnormal quantitative and qualitative secretory cycle. Our results show that the ultra-structural lesions of family goitres by deficiency of deiodase differ from structural troubles pointed out for goitres by a deficiency of organification of iodine and reproduce only partially the cyto-structure of embryonic thyroid.