cretinism
| Classification according to ICD-10 | |
|---|---|
| E00 | Congenital iodine deficiency syndrome |
| E00.0 | Congenital iodine deficiency syndrome, neurological type |
| E00.1 | Congenital iodine deficiency syndrome, myxedematous type |
| E00.2 | Congenital iodine deficiency syndrome, mixed type |
| E00.9 | Congenital iodine deficiency syndrome, unspecified |
| E03.0 | Congenital hypothyroidism with diffuse goiter |
| E03.1 | Congenital hypothyroidism without goiter |
| ICD-10 online (WHO version 2019) | |
As cretinism (derived from French crétin , possibly from latin crista , "outgrowth survey", based on the human Kropf) or innate iodine deficiency syndrome is represented by an insufficient effect of thyroid hormones and iodine deficiency caused frame of the untreated congenital hypothyroidism designated (underactive thyroid).
Symptoms
The child's thyroid produces too little thyroxine . This slows down the entire metabolism , resulting in malformations of the skeleton (shortened extremities, short stature, dwarfism ), speech disorders, hearing loss, possibly deafness. The children often have thick tongues and dry skin , sometimes a puffy face. In addition, cretinism can lead to increased obesity, due to the lower basal metabolic rate in the case of an underactive thyroid . There is often a weakness of the connective tissue , which can be indicated by the occurrence of an umbilical hernia . Reduced elasticity of the muscles and the occurrence of uncoordinated movements as well as retarded mental development are also possible. The reason for this is that the lack of thyroid hormones in both the central and peripheral nervous systems slows down the formation of axons , dendrites , nerve synapses and myelin sheaths .
causes
Congenital hypothyroidism, the full picture of which is cretinism, occurs statistically in about 0.2 ‰ of all newborns. It arises from a missing or insufficiently developed thyroid gland ( aplasia or dysplasia ), insufficient hormone biosynthesis or release, and rarely also from hormone resistance due to T 3 receptor defects. A lack of iodine in the mother can also cause congenital hypothyroidism in the child. In children, iodine deficiency is the most common preventable cause of mental retardation worldwide . Iodine is required by the thyroid gland to produce thyroid hormones .
Cretinism can also be triggered in the womb by an underactive thyroid in the mother. If the mother's thyroid produces too little or no thyroid hormones, there is a risk of malformations and neurological damage in the child, as the cells and organs are particularly dependent on thyroid hormones during growth.
diagnosis
Since an early diagnosis determines the further course of the disease, screening for hypothyroidism is required by law. Evidence of decreased thyroid function is provided by a routine test of a few drops of blood on April 2–5. Day of life, which is often gained from the heel, is sought. An increased TSH level allows conclusions to be drawn about an underactive thyroid.
Treatment and prevention
Treatment should start as early as possible, it can restore normal body growth and is necessary for life. To do this, thyroxine is used with regular monitoring of the level of hormones in the blood. Brain damage caused by starting substitution treatment too late is irreversible .
In areas of iodine deficiency , prophylaxis with iodine or iodized salt is particularly important during pregnancy and childhood.
history
etymology
In Latin, the term cretinism has been used since 1656, and since the 18th century it has also been established in French (crétinisme) , German ( cretinism , cretinism ) and other European vernacular languages. It is derived from the French crétin “person suffering from cretinism, generalized: deformed idiot, fool”, which since François Génin has been derived from the Latin christianus “Christian, Christian”, assuming a phonetically anomalous development from Latin -ianus to French -in instead -ien and an undocumented development of meaning "Christian> innocent person> benign imbecile". In the first in-depth study of recent Romance studies , Christian Schmitt hypothesizes that the Latin etymon crista "outgrowth, elevation", already used in classical Latin for swelling or proliferating outgrowths in the head and back area of animals, in conjunction with the Latin Suffix -inus , assuming a meaning like cristinus > "deformed in the head area, boned" allows a phonetically and contentwise more plausible construction of the history of origin.
Medical history
Historically, the first foundations of institutions for "stupid" children emerged from the interest of some doctors in cretinism, which was depicted as early as the Middle Ages, was found to occur in certain areas in the 16th century and was impressively described by Felix Platter at the beginning of the 17th century. A first example is the “sanatorium for cretins and stupid children” founded in 1841 by the Swiss doctor Johann Jakob Guggenbühl near Interlaken . Further institutes were founded in Alpine and southern Germany, especially in Württemberg , including the Mariaberg sanatorium and nursing home founded in 1847 by the Urach senior medical officer Carl Heinrich Rösch , which was the first modern German complex facility with differentiated offers for schooling, employment and assisted living Aid for the disabled on a medical-scientific basis applies (today - with an expanded concept - known under the name Mariaberg eV ). The occurrence of cretinism was observed particularly strongly by the experts in the Alpine valleys, while the ailment no longer seemed to exist in the mountain air.
Guggenbühl describes the regional climatic conditions and the poor hygiene conditions in the villages as causes of cretinism:
“No fresh breeze sweeps through the rooms, the hideous stench is a real balm for people; no ray of sunshine can illuminate them, as the windows, which are small anyway, are completely opaque from dirt and mostly stuck together with paper. The rooms are so damp that cryptogams thrive on the walls, hung with unclean clothes and whatever else smells, so that a breath of poison fills the room, which made me vomit several times. [...] After the birth, the children are tied into the cradle and lie on their filth for days; Locked up in a chamber, completely isolated and left to fend for themselves until the work is done. "
Later, however, Guggenbühl used the term cretinism to describe all forms of intellectual disability. He also assumes a possible cure for cretinism through mountain air, cleanliness, diet , medical treatment but also the right education .
In order to research the phenomenon of cretinism, which was often found in the villages of Franconia at the time , the Würzburg pathologist Rudolf Virchow examined numerous skulls of the deceased in Lower Franconia and published his results from 1851.
In the 1890s, Julius Wagner-Jauregg (1857–1940) carried out fundamental studies on cretinism in Styria and treated it with thyroid extracts. Around 1905, the Würzburg psychiatrist Wilhelm Weygandt successfully treated endemic cretinism with thyroidine tablets.
The iodized salt precaution, which was implemented throughout Switzerland from 1918 in the Mattertal by Otto Bayard and then from 1922 in Appenzell Ausserrhoden by Hans Eggenberger , was pioneering worldwide. As a result, no more cretins were born in Switzerland .
See also
literature
- Paul Cranefield, Walter Federn: Paracelsus on goiter and cretinism: a translation and discussion of "De Struma, Vulgo Der Kropf". In: Bulletin of the History of Medicine. Volume 37, 1963, pp. 463-471.
- Franz Merke: History and Iconography of Endemic Goiter and Cretinism. Bern / Stuttgart / Vienna 1971.
- Thomas Schlich: Cretinism. In: Werner E. Gerabek , Bernhard D. Haage, Gundolf Keil , Wolfgang Wegner (eds.): Enzyklopädie Medizingeschichte. De Gruyter, Berlin / New York 2005, ISBN 3-11-015714-4 , p. 806.
- Cretinism . In: Meyers Großes Konversations-Lexikon . 6th edition. Volume 11, Bibliographisches Institut, Leipzig / Vienna 1907, pp. 641–643 .
Web links
Individual evidence
- ^ A b Christian Schmitt: Christianity and Cretinism. On the effects of scientific deficits in etymological research. In: Romance Yearbook. Volume 59, 2008, pp. 29-45.
- ↑ Cretinism: Symptoms . onmeda.de
- ^ Cretinism . symptomat.de
- ↑ a b A. Barg: Cases Physiology. Elsevier, 2006, ISBN 3-437-41281-7 , p. 91.
- ↑ a b Gerd Herold: Internal Medicine. 2013, p. 751.
- ↑ K. Middendorf: Obstetrics Basics. Springer, Berlin / Heidelberg 2006, pp. 79–82; Retrieved February 12, 2008.
- ↑ Lois Jovanovic, Genell J. Subak-Sharpe: Hormones. The medical manual for women. From the American by Margaret Auer. Kabel, Hamburg 1989, ISBN 3-8225-0100-X , p. 379 (original edition: Hormones. The Woman's Answerbook. Atheneum, New York 1987).
- ^ Christian Helfer: Lexicon auxiliare. A German-Latin dictionary. 3. Edition. Saarbrücken 1991, p. 330.
- ^ François Génin: Récréations philologiques. Volume 2. Paris 1856, pp. 163-166.
- ↑ See also Duden: Kretin .
- ^ History of Special Education. sonderpaed-online.de
- ^ A b Johann Jakob Guggenbühl: Call for help from the Alps, to combat the terrible cretinism. In: Malten's library of the latest world studies. Volume 1, Aarau 1840, p. 191 ff. Books.google.com
- ↑ Inghwio from the Schmitten: Insane in Salzburg. To the story of a separation. Umbruch publishing house, workshop for social and psychoanalysis, Salzburg 1985.
- ^ Heinz Otremba: Rudolf Virchow. Founder of cellular pathology. A documentation. Echter-Verlag, Würzburg 1991, p. 21.
- ↑ Johannes Gstach: Cretinism and nonsense. For the technical and scientific discovery and construction of phenomena of mental and spiritual abnormalities between 1780 and 1900 and their importance for questions of education and treatment. (Habilitation thesis Vienna 2014) Julius Klinkhardt, Bad Heilbrunn 2015, ISBN 978-3-7815-2003-5 , p. 368.
- ↑ Julius Wagner-Jauregg ( memento from June 28, 2009 in the Internet Archive ) on the website of the Institute for the History of Medicine at the University of Vienna
- ↑ Werner E. Gerabek, Werner Gerabek (ed.): Enzyklopädie Medizingeschichte . Walter de Gruyter, 2005, ISBN 3-11-015714-4 , p. 1463 (1544 p., Limited preview in Google Book search).
- ^ Gerhardt Nissen : Early contributions from Würzburg on the development of child and adolescent psychiatry. In: Peter Baumgart (Ed.): Four hundred years of the University of Würzburg. A commemorative publication. Degener & Co. (Gerhard Gessner), Neustadt an der Aisch 1982 (= sources and contributions to the history of the University of Würzburg , Volume 6), ISBN 3-7686-9062-8 , pp. 935–949; here: p. 945.
- ↑ Claudine Als: Cretinism. In: Historical Lexicon of Switzerland . November 4, 2008 , accessed June 12, 2019 .
