Cretinism

As cretinism (derived from French crétin, altfr. Crestien christianus from latin, actually " ( poor ) Christian man " ) is called the frame of untreated congenital hypothyroidism.

Symptoms

The child's thyroid gland produces too little thyroxine. Thus, the entire metabolism, skeletal deformities ( shortened limbs, short stature, dwarfism ), speech disorders, hearing loss, deafness may be slowed. The children often have a thick tongue and dry skin. In addition, cretinism can lead to increased obesity, due to the lower basal metabolic rate in hypothyroidism.

Of particular importance is the delayed mental development. The reason for this is that the formation of axons, dendrites, synapses and nerve myelin is slowed down by the lack of thyroid hormone in both the central and the peripheral nervous system.

Causes

A congenital hypothyroidism whose frame is cretinism, occurs statistically at about 0.2 ‰ of all newborns. It is caused by lack of or insufficient scale thyroid (aplasia or dysplasia ), insufficient hormone biosynthesis or distribution, rare. Well by a hormone resistance due to T3 - receptor defects Also iodine deficiency in the mother can cause a congenital hypothyroidism of the child. In children, iodine deficiency is the most common preventable cause of retardation worldwide. Iodine is only required by the thyroid gland in the formation of thyroid hormones.

Can be triggered cretinism but also in the womb by a hypothyroidism of the mother. Produces the thyroid of the mother to little or no thyroid hormones, there is a risk of birth defects and neurological damage in children, as in times of growth, the cells and organs are heavily dependent on the thyroid hormones.

Diagnosis

Since an early diagnosis will decide on the further course of the disease, a Hypothyreosescreening required by law. Evidence of impaired thyroid function. Routine examination by a few drops of blood on the 2nd-5th Day of life, which is often derived from the heel, wanted. An elevated TSH levels can this be traced to an underactive thyroid.

Treatment

Treatment should begin as soon as possible and is necessary for life. These thyroxine is used under regular checks hormone levels in the blood. Damage caused by late onset of substitution treatment brain damage is irreversible.

In iodine deficiency areas of iodine prophylaxis is of particular importance in pregnancy and childhood.

History

Etymology

The term cretin for " misshapen idiot " was the end of the 18th century, first borrowed in the plural cretins and until the early 20th century, used for people with conditions caused by underactive thyroid or absence of severe developmental disorders. The French-language expression is based on the acquisition of a south-eastern French dialect word derjeniger Alpine valleys, where such illness most frequently occurred, as in the Valais or Savoy. Thus, the term creitin " imbecile, with a goiter Afflicted, debilitated " used in the Savoy and Dauphiné. Linguistically, it is a dialectal variant of French chrétien, Old Fr. Cresti ( i) s "Christ", which is also in contrast to the animal " human being, person" as Latin Christianus "Christian" ( a substantive as "Christ") continued. Probably originally selected out of pity designation (see Sporadic Old Fr. Crestien " sufferer, Sick " ) was associated with the negative rating and since the 19th century in French and later in English sometimes pejorative for " fool, limited, incompetent person " needed. The term cretinism was created in 1800 as latinisierende education in medicine after French crétinisme.

History of Medicine

Originally Chrétien was (French: Christ ) in particular used in Switzerland as a euphemism for the mentally disabled, but good-natured people, so as not to annoy or not to provoke the use of their alleged demonic abilities; from chrétien was crétin in language use.

History go out of the interest of some doctors for the cretinism the first foundations of institutions for " stupid " children were born. A first example is the Founded in 1841, " sanatorium for cretins and idiotic children " by the Swiss physician Johann Jakob Guggenbühl at Interlaken. There were other institution -ups in the alpine and southern Germany, especially in Württemberg, including, founded in 1847 from Urach district doctor Carl Heinrich Roesch healing and nursing home Mariaberg that differentiate their offerings of schooling, the Beschäfigung and assisted living as the first German complex institution of modern disability assistance to medical and scientific basis applies (today - with extended concept - known under the name of Maria Berg eV). The occurrence of cretinism was observed by the trade representatives particularly strong in the mountain valleys, while suffering no longer seemed to exist in the mountain air.

Guggenbühl describes the regional climatic conditions and poor hygiene conditions in the villages as causes of cretinism:

" No fresh breeze sweeps through the chambers, the most horrible stench is the people a true life balm; no sunbeam they can illuminate, as without this small window and on top of that are usually glued quite opaque from dirt with paper. The rooms are so damp, the cryptogams on the walls prospers this with unclean clothes and whatever else stinks hung so that a poisonous breath fills the room, the [ ... ] repeatedly appealed to me to vomit. [ ... ] After the birth of children are integrated into the cradle, days are long remain in their filth; enclosed in a chamber completely isolated and left to themselves, until the work is finished. "

Later, however Guggenbühl referred to the concept of cretinism all forms of mental retardation. He is also still of a possible cure of cretinism by mountain air, cleanliness, diet, medical treatment but also the proper education from.

Julius Wagner -Jauregg (1857-1940) led by fundamental studies of cretinism and treated him by thyroid extracts.