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Cretinism

Cretinism (from fr. Crétin - idiot , foolish ) - an endocrine disease caused by a lack of thyroid hormones , characterized by a pronounced decrease in thyroid function , a delay in physical and mental development . One form of congenital hypothyroidism . The term “nerd” is outdated and is not recommended for use, since it went beyond the purely medical framework and began to have a social (negative) connotation . Nevertheless, traditional terms continue to be used in psychiatric literature.

Congenital iodine deficiency syndrome
ICD-10E 00.
ICD-9243
Diseasesdb6612
eMedicineped / 501

Content

  • 1 General
  • 2 Etiology, pathogenesis, epidemiology
  • 3 Clinical manifestations
  • 4 Treatment
  • 5 Prevention
  • 6 See also
  • 7 notes
  • 8 Literature

General information

Thyroid hormones stimulate the growth and differentiation of tissues, as well as the growth and development of the body , so their deficiency at an early (including prenatal ) age can cause a deviation in both physical development and the central nervous system . The basis of hypothyroid states, accompanied by delays in development, is a deficiency of iodine and selenium in water and food [1] [2] .

Etiology, Pathogenesis, Epidemiology

Cretinism may occur due to:

  • hereditary factor ( thyroid-stimulating hormone reaction);
  • disorders of the embryonic development of the thyroid gland;
  • thyroid hypoplasia and atyroidism (lack of thyroid gland);
  • ectopia of the thyroid gland;
  • endemic goiter carried by the mother during pregnancy .
  • deficiency of iodine and selenium , necessary for the synthesis of thyroid hormones [2] .

Clinical manifestations

Characteristic signs of cretinism:

  • physical development lag,
  • growth retardation and tooth change, prolonged failure of fontanelles of the skull,
  • gross facial features (due to swelling of the soft tissues): a wide flat (“square") nose with a lowering of its back, eyes far apart (ocular hypertelorism),
  • large tongue (often it does not fit in the mouth),
  • disproportionate body structure: short limbs, large head,
  • thick and rough skin
  • underdeveloped secondary sexual characteristics,
  • disorders of mental and mental development (up to idiocy ),
  • small growth (often - dwarfism) [3] .

In severe cases, myxedema edema (myxedema cretinism) is observed.

Treatment

In the early stages, cretinism is fully compensated by taking thyroid medications ( thyroxine ).

A large role in the early detection of the disease is played by the screening of newborn children, in particular, in Russia, screening is carried out for all born children on the fourth day of life (full-term) and on the seventh day (premature). The screening system determines the level of thyroid-stimulating hormone in the blood, this indicator gives an idea about the function of the thyroid gland and is key in making a diagnosis. If an increased level of thyroid-stimulating hormone is detected, the child is immediately retested and preventive treatment with thyroxine preparations is prescribed.

It is important to prescribe treatment as early as possible to avoid the adverse effects of thyroid hormone deficiency. Children who started treatment before the 21st day of life do not lag behind in development from healthy peers. In neglected cases, when the screening was not carried out (home delivery, early discharge from the maternity hospital) and the treatment was not scheduled on time, the children are lagging behind in development, they have a delay in speech and intelligence. Characteristic signs of congenital hypothyroidism are: dry skin, constipation, poor weight gain, lethargy of the child.

Prevention

In geographical regions with a low iodine content in the environment (water, air and soil) - the so-called iodine endemic - mass prevention (using the method of universal salt iodization ) or individual (taking iodine and selenium tablets) is carried out among the population.

See also

  • Hypothyroidism
  • Selenium deficiency
  • Iodized salt
  • Iodine deficiency
  • Endemic goiter

Notes

  1. ↑ Toxic Substances - Selenium. Centers for Disease Control and Prevention
  2. ↑ 1 2 The interactions between selenium and iodine deficiencies in man and animals. Arthur JR, Beckett GJ, Mitchell JH. - Nutrition Research Reviews. 1999 Jun; 12 (1): 55-73
  3. ↑ Description of cretinism on the website Medicedia.ru

Literature

  • Gilyarovsky V.A. Psychiatry. A guide for doctors and students. - M. — L .: Biomedgiz, 1935.


Source - https://ru.wikipedia.org/w/index.php?title=Cretinism&oldid=101439744


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Clever Geek | 2019