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Cretinism

thyroid, loss, characteristic, conditions, seen, sporadic and child

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CRETINISM. This is a broad general term applied to a combination of physical and mental changes which, in the young, result from loss or diminution of the thyroid functions. Such a loss may occur sporadically (Sporadic cretinism) from causes to be enumerated, where the picture is analogous to that seen in the adult from removal of the thyroid, cachexia thyreopriva adultorum; or in many occur as a localized or endemic degeneration affecting the thyroids of a large ' number of individuals, causing a hypothyreogis which may show a number of tendencies. The chief of these are goitre, goitrous heart and endemic cretinism.

These four fairly well-separated conditions may be discussed to advantage under the head of cretinism. The historical chapters on cretin-' ism are full of interest. The disorders were known in early days. Pliny has left indubitable evidence of their presence in early Roman times; Vogt in his admirable monograph in the Lewan dowsky, (Handbuch der Neurologie) tells of Marco Polo's descriptions of certain types he had seen in his Asiatic travels. During the past two centuries the disorders here included under the term have been observed through the world. In certain lands, especially certain mountainous districts of Switzerland, northern Italy, etc., the disease is very widely distributed — endemic cretinism—while in other regions it occurs rarely — sporadic cases. In the United. States it is not frequent. It has been observed in California, in Vermont, and the writer has seen such patients in New York State (Adiron dacks). In certain regions it has been a veri table plague. Thus in Switzerland, between the years 1875-84, 7 per cent of the recruits in the army showed some form of cretinoid degenera tion. In 10 years 2,500 men were lost to the Swiss army from this cause alone. Certain val leys, especially those of Berne and Wallis, are thicidy populated with individuals showing cretinoid degeneration. In the school years 1899-1904, of 336,000 children fit for school 15,000 had one or anotherof cretinism. Similar conditions existed in in Austria, and in certain Italian provinces.

So far as the etiology is concerned, it seems certain that the conditions are due to a defect of the thyroid substance—diminution or loss of the thyroid hormones. There are certain limi tations which must be discussed in their respec tive paragraphs.

Sporadic Cretinism.— Infantile Mysedenia. The clinical picture in an extreme case—Le., fully developed, in contrast to the many irregu lar or incomplete forms —is that of a normally born child who, about the end of the first or the beginning of the second year, begins to show the characteristic changes in development. The little patients fall behind in their normal bony development. This is due to a defect in the de velopment of the long bones. The epiphyses fail to lay down bone even after 20 to 35 years and in 20-year-old cretins the anterior fontanelle may still remain open. There is a proportionate loss in bone substance through out; thus a characteristic dwarfism results save perhaps in the development of the skull which becomes larger in proportion to the rest of the body. The sphenoid, however, fails to develop and therefore 'gives the peculiar characteristic nose to the cretin. Dental deficiencies go hand in hand with the bony defect. In severe athy roid cretins the teeth do not develop for a num ber of years, and the first or milk teeth may persist far beyond the normal period. The bony defects show in a high palate arch, with large adenoids and tonsils, and a chronic hypertrophic rhinitis causes the child to snore and sniffle, often with copious excretions from the nose.

A fairly constant finding is that of umbilical hernia. The abdomen is usually puffy, the navel sunken. The skin is myxedematous in the young, but becomes atrophic in later years, the supraclavicular and facial swelling remaining for many years.

The facial habitus is characteristic. The hair line begins low, the nose is sunken, the zygo matic arches prominent, the eyelids swollen, the face puffy, the tongue enlarged and often pro truding between the swollen lips, in the mild cases giving one the impression of a child whose whole countenance is puffed up with crying.

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