Cretinism: Wikis

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This article is about the medical term. For the Marxist political concept, see Parliamentary cretinism.
Congenital iodine-deficiency syndrome
Classification and external resources
ICD-10 E00.
ICD-9 243
DiseasesDB 6612
eMedicine ped/501
MeSH C05.116.099.343.347

Cretinism is a condition of severely stunted physical and mental growth due to untreated congenital deficiency of thyroid hormones (congenital hypothyroidism) due to maternal nutritional deficiency of iodine.

Contents

Etymology and use of cretin

The term cretin describes a person so affected, but, like words such as spastic and lunatic, also is a word of abuse. Cretin became a medical term in the 18th century, from an Alpine French dialect prevalent in a region where persons with such a condition were especially common (see below); it saw wide medical use in the 19th and early 20th centuries, and then spread more widely in popular English as a markedly derogatory term for a person who behaves stupidly. Because of its pejorative connotations in popular speech, health-care workers have mostly abandoned cretin.

The etymology of cretin is uncertain. Several hypotheses exist. The most common derivation provided in English dictionaries is from the Alpine French dialect pronunciation of the word Chrétien ("(a) Christian"), which was a greeting there. According to the Oxford English Dictionary, the translation of the French term into "human creature" implies that the label "Christian" is a reminder of the humanity of the afflicted, in contrast to brute beasts.[1] Other sources suggest that Christian describes the person's "Christ-like" inability to sin, stemming, in such cases, from an incapacity to distinguish right from wrong.[2]

Other speculative etymologies have been offered:

  • From creta, Latin for chalk, because of the pallor of those affected.
  • From "Crete", where iodine insufficiency (Hypothyroidism) was common prior to the modern era.[2]
  • From cretira, Grisson-Romance creature, from Latin creatus.
  • From cretine, French for alluvium (soil deposited by flowing water), an allusion to the affliction's suspected origin in inadequate soil.[3]

Sporadic cretinism due to congenital hypothyroidism

Congenital hypothyroidism can be endemic, genetic, or sporadic. If untreated, it results in mild to severe impairment of both physical and mental growth and development.

Poor length growth is apparent as early as the first year of life. Adult stature without treatment ranges from 1 to 1.6 metres (3'4 to 5'3), depending on severity, sex and other genetic factors. Bone maturation and puberty are severely delayed. Ovulation is impeded and infertility is common.

Neurological impairment may be mild, with reduced muscle tone and coordination, or so severe that the person cannot stand or walk. Cognitive impairment may also range from mild to so severe that the person is nonverbal and dependent on others for basic care. Thought and reflexes are slower.

Other signs may include thickened skin, enlarged tongue, or a protruding abdomen.

Sporadic and genetic cretinism results from abnormal development or function of the foetal thyroid gland. This type of cretinism has been almost completely eliminated in developed countries by early diagnosis by newborn screening schemes followed by life long treatment with thyroxine (T4).

Thyroxine must be dosed as tablets only, even to newborns, as the liquid oral suspensions and compounded forms cannot be depended on for reliable dosing. In the case of dosing infants, the T4 tablets are generally crushed and mixed with breast milk, formula milk or water. If the medication is mixed with formulas containing iron or soya products, larger doses may be required, as these substances may alter the absorption of thyroid hormone from the gut. [4] Frequent monitoring (every 2–3 weeks during the first months of life) is recommended to ensure that infants with congenital hypothyroidism remain within the high end of normal range, or euthyroid.

Epidemiology

Disability-adjusted life year for iodine deficiency per 100,000 inhabitants in 2002.[5]
     no data      less than 50      50-100      100-150      150-200      200-250      250-300      300-350      350-400      400-450      450-500      500-800      more than 800

Cretinism arises from a diet deficient in iodine. It has affected many people worldwide and continues to be a major public health problem in many countries. Iodine is an essential trace element, necessary primarily for the synthesis of thyroid hormones. Although iodine is found in many foods, it is not universally present in all soils in adequate amounts. The soils of many inland areas on all continents are iodine deficient, and plants and animals grown there are correspondingly deficient. Populations living in those areas without outside food sources are most at risk of iodine deficiency diseases.[6]

Iodine deficiency results in the impairments in varying degrees of physical and mental development. It also causes gradual enlargement of the thyroid gland, referred to as a goitre. It is being combated in many countries by public health campaigns of iodine administration.

History

Endemic cretinism was especially common in areas of southern Europe around the Alps and was described by ancient Roman writers, and often depicted by medieval artists. The earliest Alpine mountain climbers sometimes came upon whole villages of cretins.[7] Alpine cretinism was described from a medical perspective by several travellers and physicians in the late eighteenth and early nineteenth centuries.[8] At that time the cause was not known and it was often attributed to "stagnant air" in mountain valleys or "bad water." The proportion of people affected varied markedly throughout southern Europe and even within very small areas it might be common in one valley and not another. The number of severely affected persons was always a minority, and most persons were only affected to the extent of having a goitre and some degree of reduced cognition and growth. The majority of such cases were still socially functional in their pastoral villages.

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Nineteenth century

More mildly affected areas of Europe and North America in the nineteenth century were referred to as "goiter belts". The degree of iodine deficiency was milder and manifested primarily as thyroid enlargement rather than severe mental and physical impairment. In Switzerland, for example, where soil is poor in iodine, cases of cretinism were very abundant and even considered genetically caused. As the variety of food sources dramatically increased in Europe and North America and the populations became less completely dependent on locally grown food, the prevalence of endemic goitre diminished.

Twentieth and twenty-first centuries

The early twentieth century saw the discovery of the relationships of sporadic cretinism with congenital hypothyroidism, and of endemic cretinism with hypothyroidism due to iodine deficiency. Both have been largely eliminated in the developed world.

There are recent suggestions that the skeletons supporting the existence of the human species Homo floresiensis are of individuals who suffered from cretinism.[9]

See also

References

  1. ^ "cretin". Oxford English Dictionary. http://dictionary.oed.com/cgi/entry/50053852. Retrieved 2005-12-11.  
  2. ^ Brockett, Linus P (Feb 1858). "Cretins And Idiots". The Atlantic Monthly. http://www.disabilitymuseum.org/lib/docs/1385.htm. Retrieved 2005-12-11.  
  3. ^ Medvei, VC (1993). The History of Clinical Endocrinology. Pearl River, New York: Parthenon Publishing Group.  
  4. ^ Chorazy PA, Himelhoch S, Hopwood NJ, Greger NG, Postellon DC (July 1995). "Persistent hypothyroidism in an infant receiving a soy formula: case report and review of the literature". Pediatrics 96 (1 Pt 1): 148–50. PMID 7596704.  
  5. ^ "Mortality and Burden of Disease Estimates for WHO Member States in 2002" (xls). World Health Organization. 2002. http://www.who.int/entity/healthinfo/statistics/bodgbddeathdalyestimates.xls.  
  6. ^ Gaitan E, Dunn JT (1992). "Epidemiology of iodine deficiency". Trends Endocrinol. Metab. 3 (5): 170–5. PMID 18407097. http://linkinghub.elsevier.com/retrieve/pii/1043-2760(92)90167-Y.  
  7. ^ Fergus Fleming, Killing Dragons: The Conquest of the Alps, 2000, Grove Press, p. 179
  8. ^ See, for example, William Coxe, "Account of the Vallais, and of the Goiters and Idiots of that Country," Universal Magazine of Knowledge & Pleasure, vol. 67, Dec. 2, 1780.
  9. ^ [1]

External links


1911 encyclopedia

Up to date as of January 14, 2010

From LoveToKnow 1911

CRETINISM, the term given to a chronic disease, either sporadic or endemic, arising in early childhood, and due to absence or deficiency of the normal secretion of the thyroid gland. It is characterized by imperfect development both of mind and body. The thyroid gland is either congenitally absent, imperfectly developed, or there is definite goitre. The origin of the word is doubtful. Its southern French form Chrestiaa suggested to Michel a derivation from cresta (crete), the goose foot of red cloth worn by the Cagots of the Pyrenees. The Cagots, however, were not cretins. The word is usually explained as derived from chretien (Christian) in the sense of " innocent." But Christianus (which appears in the Lombard cristanei; compare the Savoyard innocents and Bens du bon dieu) is probably a translation of the older cretin, and the latter is probably connected with creta (craie) - a sallow or yellow-earthy complexion. being a common mark of cretinism.

The endemic form of cretinism prevails in certain districts, as in the valleys of central Switzerland, Tirol and the Pyrenees. In the United Kingdom cretins have been found in England at Oldham, Sholver Moor, Crompton, Duffield, Cromford (near Matlock), and other points in Derbyshire; endemic goitre has been seen near Nottingham, Chesterfield, Pontefract, Ripon, and the mountainous parts of Staffordshire and Yorkshire, the east of Cumberland, certain parts of Worcester, Warwick, Cheshire, Monmouth, and Leicester, near Horsham in Hampshire, near Haslemere in Surrey, and near Beaconsfield in Buckingham. There are cretins at Chiselborough in Somerset. In Scotland cretins and cases of goitre have been seen in Perthshire, on the east coast of Fife, in Roxburgh, the upper portions of Peebles and Selkirk, near Lanark and Dumfries, in the east of Ayrshire, in the west of Berwick, the east of Wigtown, and in Kirkcudbright. The disease is not confined to Europe, but occurs in North and South America, Australia, Africa and Asia. Wherever endemic goitre is present, endemic cretinism is present also, and it has been constantly observed that when a new family moves into a goitrous district, goitre appears in the first generation, cretinism in the second. The causation of goitre has now been shown to be due to drinking certain waters, though the particular impurity in the water which gives rise to this condition has not been determined (see Goitre). The causation of the sporadic form of cretinism is, however, obscure.

Cretinism usually remains unrecognized until the child reaches some eighteen months or two years, when its lack of mental development and uncouth bodily form begin to attract attention. Occasionally the child appears to be normal in infancy, but the cretinoid condition develops later, any time up to puberty. The essential point in the morbid anatomy of these cases is the absence or abnormal condition of the thyroid gland (see Metabolic Diseases). It may be congenitally absent, atrophied, or the seat of a goitre, though this last condition is very rare in cases of sporadic cretinism. The skeleton shows arrested growth, most marked in the case of the long bones. The skull in the endemic form of cretinism is usually brachycephalic, but in the sporadic cases it is more commonly dolichocephalic. The pathology of cretinism and its allied condition myxoedema has now been conclusively worked out, and its essential cause has been shown to be loss of function of the thyroid gland.

The condition has existed and been described in far back ages, but mingled with so many other entirely different deformities and degenerations that it is now often almost impossible to classify them satisfactorily. The following is a vivid picture by Beaupre (Dissertation sur les cretins, translated in Blackie on Cretinism, Edin., 18 55): " I see a head of unusual form and size, a squat and bloated figure, a stupid look, bleared hollow and heavy eyes, thick projecting eyelids, and a flat nose. His face is of a leaden hue, his skin dirty, flabby, covered with tetters, and his thick tongue hangs down over his moist livid lips. His mouth, always open and full of saliva, shows teeth going to decay. His chest is narrow, his back curved, his breath asthmatic, his limbs short, misshapen, without power. The knees are thick and inclined inward, the feet flat. The large head drops listlessly on the breast; the abdomen is like a bag." When fully grown the height rarely exceeds 4 ft., and is often less than 3 ft. The skin feels doughy from thickening of the subcutaneous tissues, and it hangs in folds over the abdomen and the bends of the joints. Very frequently there is an umbilical hernia. The hair has a far greater resemblance to horse-hair than to that of a human being, and is usually absent on the body of an adult cretin. The temperature is subnormal, and the exposed parts tend to become blue in cold weather. The blood is usually deficient in haemoglobin, which is often only 40-50% of the normal. The mental capacity varies within narrow limits; an intelligent adult cretin may reach the intellectual development of a child 3-4 years of age, though more often the standard attained is even below this. The child cretin learns neither to walk nor talk at the usual time. Often it is unable even to sit without support. Some years later a certain power of movement is acquired, but the gait is waddling and clumsy. Speech is long delayed, or in bad cases may be almost entirely lacking. The voice is usually harsh and unpleasant. Of the senses smell and taste are but slightly developed, more or less deafness is generally present, and only the sight is fairly normal. In the adult the genital organs remain undeveloped. If the cretin is untreated he rarely has a long life, thirty years being an exceptional age. Death results from some intercurrent disease.

Cretinism has to be distinguished from the state of a Mongolian idiot s in whom there is no thickening of the subcutaneous tissues, and much greater alertness of mind; from achondroplasia, in which condition there is usually no mental impairment; and from infantilism, which covers a group of symptoms whose only common point is that the primary and. secondary sexual characteristics fail to appear at the proper time.

Before 1891 there was no treatment for this disease. The patients lived in hopeless imbecility until their death. But in that year Dr George Murray published his discovery of the effect of hypodermic injections of thyroid gland extract in cases of myxoedema. In the following year Drs Hector Mackenzie, E. L. Fox of Plymouth, and Howitz of Copenhagen, each working independently, showed the equally potent effect of the gland administered by the mouth. The remedy was soon after applied to cretinism and its effects were found to be even more wonderful. It has to be used, however, with the greatest care and discrimination, since personal idiosyncrasy seems to be a very variable factor. Even small doses, if beyond the patient's power, may produce fever, excitement, headache, insomnia and vomiting. The administration must be persisted in throughout life, otherwise myxoedematous symptoms appear. The first most apparent results are those of growth, and this may supervene even in patients up to 25-30 years of age. Once started, 4 to 6 in. may be gained in stature in the first year's treatment, though this is usually in inverse ratio to the age of the patient, and also diminishes in later stages of treatment. In young adolescents it may be so rapid that the patient has to be kept lying down to prevent permanent bending of the long bones of the leg, softened by their rapid growth. A very typical case under Dr Hector Mackenzie, showing what can be expected from early treatment, is that of a cretin aged I r years in 1893, when thyroid treatment was started. He grew very rapidly and became a normal child, passed through school, and in 1908 was at one of the universities.

Bibliography. - Sardinian Commission, " Relazione della cornmissione di Sardegna per studiare it cretinismo " (Torino, 1848); C. Hilton Fagge, " On Sporadic Cretinism occurring in England," Med. Chir. Trans. (London, 1870); Vincenzo Allara, " Sulla causa del cretinesimo," studio (Milano, 1892); Victor Horsley, " Remarks on the Function of the Thyroid Gland," Brit. Med. Journ. (1892); " The Treatment of Myxoedema and Cretinism, being a Review of the Treatment of those Diseases by Thyroid Gland," Journ. Ment. Sc. (London, 1893) W. Osler, " On Sporadic Cretinism in America," Am. Journ. of Med. Sc. (1893); C. A. Ewald, Die Erkrankungen der Schilddriise, Myxiideme and Cretinismus (Wien, 1896); G. R. Murray, Diseases of the Thyroid Gland, part i. (1900); R. Virchow, Uber Cretinismus," Wiirzburger Verhand.; Hector Mackenzie, " Organotherapy," Textbook of Pharmacology and Therapeutics (1901); Weygandt, Der heutige Stand der Lehre vom Kretinismus (Halle, 1903); Hector Mackenzie, " Cretinism," Allbutt & Rolleston's System of Medicine, part iv. (1908).


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