pellagra

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(pə-lăg'rə, -lā'grə, -lä') pronunciation
n.
A disease caused by a deficiency of niacin and protein in the diet and characterized by skin eruptions, digestive and nervous system disturbances, and eventual mental deterioration.

[Italian : pelle, skin (from Latin pellis) + -agra, a seizure (from Latin , from Greek -agrā , from agrā, a seizing).]

pellagrous pel·lag'rous adj.


Nutritional disorder caused largely by a deficiency of niacin, marked by skin lesions and digestive and neurological disturbances. Dermatitis usually appears first, with abnormal sensitivity to sunlight. It may look like a severe sunburn, later becoming reddish brown, rough, and scaly. Diarrhea usually alternates with constipation, along with mouth and tongue inflammation and cracking and dry scaling of the lips. Later, mental abnormalities may include nervousness, depression, and delirium. Mild cases of niacin deficiency respond to a well-balanced diet alone. Pellagra still occurs where diets consist mostly of corn, which is low in both niacin and tryptophan (converted to niacin in the body), with little or no protein-rich food. It can also be a side effect of chronic alcoholism.

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The disease due to deficiency of the vitamin niacin and the amino acid tryptophan. Signs include a characteristic symmetrical photosensitive dermatitis (especially on the face and back of the hands), resembling severe sunburn; mental disturbances (a depressive psychosis sometimes called dementia); and digestive disorders (most commonly diarrhoea); fatal if untreated.

Most commonly associated with a diet based on maize or sorghum, which are poor sources of both tryptophan and niacin, with little meat or other vegetables.

A disease due to a deficiency of niacin, a member of the vitamin B complex. Early signs of pellagra include listlessness, headache, and weight loss. This progresses to a sore tongue, nausea, vomiting, and photosensitive dermatitis, resulting in ulcerated skin. The symptoms are sometimes referred to as the ‘four Ds’: dermatitis, dementia, diarrhoea, and death.

A disease due to a deficiency of niacin. Early signs of the disease include listlessness, headache, and weight loss, which progresses to tongue soreness, nausea, vomiting, and photosensitive dermatitis. The symptoms of pellagra are sometimes referred to as the ‘four Ds’: dermatitis, dementia, diarrhoea, and death.



Pellegra is a disease caused by a dietary deficiency of, or a failure to absorb, niacin (vitamin B) or the amino acid tryptophan, a precursor of niacin. First reported in 1735 by Don Gasper Casal, a Spanish physician, pellagra means "rough skin." Primary symptoms include the "3 Ds": dementia (mental symptoms), dermatitis (scaly skin sores), and diarrhea. A pellagra epidemic emerged during the 1900s in the United States, when corn (maize) began to replace other sources of dietary protein among the rural poor. Niacin in corn is tightly bound to protein, and thus poorly absorbed. Niacin enrichment of cereal grains and diets adequate in protein and calories eventually eradicated pellagra from the United States. Seasonal epidemics still occur in parts of Southeast Asia and Africa, however.

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pellagra (pəlăg'), deficiency disease due to a lack of niacin (nicotinic acid), one of the components of the B complex vitamins in the diet. Niacin is plentiful in yeast, organ meats, peanuts, and wheat germ. The disease manifests itself in lesions of the skin and mucous membrane, diarrhea and other gastrointestinal symptoms, neurological derangement, and mental confusion. It is most common in areas where the diet consists mainly of corn, which, unlike other grains, lacks niacin as well as the amino acid tryptophan, which the body uses to synthesize the vitamin. Treatment includes large doses of niacin and the institution of a proper diet to prevent recurrences.

Bibliography

See D. A. Roe, A Plague of Corn: A Social History of Pellagra (1973).


Gale Encyclopedia of Food & Culture:

Niacin Deficiency (Pellagra)

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Deficiency of the vitamin niacin can result in the disease pellagra. This illness is characterized by the appearance of severe dermatitis on the parts of the skin that have been exposed to the sun, with deep cracking and flaking. Sufferers also have diarrhea and, in many cases, some kind of dementia. The condition typically flares up during spring, as sunshine becomes stronger. In practice, patients were often found to be deficient in the vitamin riboflavin in addition to niacin. Both in the Old and New Worlds, the condition has almost always been confined to people consuming corn (that is, maize) as their staple grain. The explanation is complex. We can synthesize niacin for ourselves to some extent if our diet contains a good supply of the amino acid tryptophan because we have enzymes that can, in a series of steps, turn a portion of any excess tryptophan molecules into niacin. The problem with corn is that the proteins that it contains have an unusually low proportion of tryptophan, and this is insufficient to provide a second source of the vitamin. Mature corn, like the mature grains of other cereals, does contain niacin, but it is mostly present in linkage with other compounds that make it essentially indigestible, because the digestive enzymes in the gut cannot break these linkages. In Central America and Mexico, where corn has been the staple grain for millennia, the populations have learned to soften the grains by soaking them with lime (calcium hydroxide) before grinding them to a paste. It is now realized that, in addition to its softening action, the alkaline lime liberates the niacin from its linkages, so that it is now nutritionally available. This is, at least, a partial explanation for the freedom of these peoples from pellagra. In addition, if a diet rich in maize is supplemented with a diet that also uses beans as a stable, as is the case among the traditional natives of Mexico, then the low level of tryptophan in maize is compensated by the higher tryptophan level in beans. Many types of long-established diets have this type of essential amino acid complementarity among the foods consumed.

However, when corn was brought back to the Old World, and gradually came to be adopted as a staple food in Southern Europe, but without the use of lime in its preparation, pellagra became a serious problem. In the nineteenth century it was suspected that corn developed toxic molds during storage. In France its use as a food crop was made illegal, and in Italy a special class of "pellagra hospitals" was established. In the southern regions of the United States, pellagra became a serious problem from about 1910 onward. There had been no obvious change in dietary habits to explain this, but it is now realized that corn processors had introduced a new method of milling corn that separated the germ. This was advantageous in reducing the oil content of the cornmeal and increasing its storage life, but it further halved the tryptophan content of the meal. The average consumer in the South was using approximately equal parts of cornmeal and of white wheaten flour that was somewhat richer in tryptophan. It appears that the change in milling was just enough to tip the balance toward the appearance of pellagra in those with only low intakes of good supplementary foods such as meat and milk. Niacin is now one of the synthetic vitamins included in the supplements routinely used to enrich flours in many Western countries.

Bibliography

Carpenter, Kenneth J., ed. Pellagra. Stroudsburg, Pa.: Hutchinson Ross, 1981.

McDowell, L. R. Vitamins in Human and Animal Nutrition. Ames: Iowa State University Press, 2000.

—Kenneth John Carpenter


a disease of animals, including humans, arising from a dietary deficiency of nicotinic acid, especially in the absence of sufficient dietary tryptophan to generate adequate amounts of nicotinamide coenzymes metabolically. This situation can occur when the diet consists largely of maize (corn). Pellagra is characterized by scaly dermatitis on exposed skin surfaces, diarrhoea, and depression.

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A syndrome in humans caused by a diet seriously deficient in niacin (or by failure to convert tryptophan to niacin). In dogs called blacktongue. See also niacin nutritional deficiency.

  • feline p. — ulcerative stomatitis, especially of the tongue, and conjunctivitis.
(pəlā′grə, pə-lag′rə)
n

A nutritional deficiency resulting from faulty intake or metabolism of nicotinic acid, a vitamin B complex factor. It is characterized by glossitis, dermatitis of sun-exposed surfaces, stomatitis, diarrhea, and dementia. Thiamine, riboflavin, and tryptophan deficiencies may be associated.

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  • Diseases and Infestations - pellagra: B-vitamin nutritional deficiency that causes scaly dermatitis, diarrhea, and depression


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Pellagra
Classification and external resources

Pellagra sufferer with skin lesions
ICD-10 E52
DiseasesDB 9730
MedlinePlus 000342
eMedicine ped/1755
MeSH C18.654.521.500.133.699.529

Pellagra is a vitamin deficiency disease most commonly caused by a chronic lack of niacin (vitamin B3) in the diet. It can be caused by decreased intake of niacin or tryptophan,[1] and possibly by excessive intake of leucine.[2] It may also result from alterations in protein metabolism in disorders such as carcinoid syndrome. A deficiency of the amino acid lysine can lead to a deficiency of niacin, as well.[3]

Contents

History

The traditional food preparation method of corn (maize), nixtamalization, by native New World cultivators who had domesticated corn required treatment of the grain with lime, an alkali. The lime treatment now has been shown to make niacin nutritionally available and reduce the chance of developing pellagra.[4] When corn cultivation was adopted worldwide, this preparation method was not accepted because the benefit was not understood. The original cultivators, often heavily dependent on corn, did not suffer from pellagra; it became common only when corn became a staple that was eaten without the traditional treatment.

Pellagra was first described in Spain in 1735 by Gaspar Casal, who published a first clinical description in his posthumous Natural and Medical History of the Asturian Principality (1762). This led to the disease being known as "Asturian leprosy", and it is recognized as the first modern pathological description of a syndrome.[5] It was an endemic disease in northern Italy, where it was named pelle agra (pelle = skin; agra = sour) by Francesco Frapoli of Milan.[6] Because pellagra outbreaks occurred in regions where maize was a dominant food crop, the belief for centuries was that the maize either carried a toxic substance or was a carrier of disease. Pellagra was also conjectured to be carried by insects. Later, the lack of pellagra outbreaks in Mesoamerica, where maize is a major food crop, led researchers to investigate processing techniques in that region.

Dr. Joseph Goldberger

In the early 1900s, pellagra reached epidemic proportions in the American South. Pellagra deaths in South Carolina numbered 1,306 during the first ten months of 1915; 100,000 Southerners were affected in 1916. At this time, the scientific community held that pellagra was probably caused by a germ or some unknown toxin in corn.[7] The Spartanburg Pellagra Hospital in Spartanburg, South Carolina, was the nation's first facility dedicated to discovering the cause of pellagra. It was established in 1914 with a special congressional appropriation to the U.S. Public Health Service (PHS) and set up primarily for research. In 1915, Joseph Goldberger, assigned to study pellagra by the Surgeon General of the United States, showed pellagra was linked to diet by inducing the disease in prisoners, using the Spartanburg Pellagra Hospital as his clinic. By 1926, Goldberger established a balanced diet or a small amount of brewer's yeast[8] prevented pellagra.

Goldberger performed an experiment using 11 volunteers from a prison, giving them clean clothes and keeping them in a house that was cleaned every day. Before the experiment, the prisoners were eating fruits and vegetables from the prison garden. Goldberger started feeding them only corn. About two weeks into the experiment, the prisoners complained of headaches, confusion, and loss of appetite. In the third week, seven of the 11 broke out in pellagra, and two prisoners begged for release. Goldberger cured them, feeding them fruits and vegetables again, and gave them their freedom. However, he failed to identify a specific element whose absence caused the pellagra. Goldberger continued his work, but died of cancer without discovering the cause.

In 1937, Conrad Elvehjem, of Madison, Wisconsin, showed the vitamin niacin cured pellagra (manifested as black tongue in dogs). Later studies by Tom Spies, Marion Blankenhorn, and Clark Cooper established that niacin also cured pellagra in humans, for which Time Magazine dubbed them its 1938 Men of the Year in comprehensive science.

Research conducted between 1900 and 1950 found the number of cases of women with pellagra was consistently double the number of cases of afflicted men.[9] This is thought to be due to the inhibitory effect of estrogen on the conversion of the amino acid tryptophan to niacin,[10] or to the differential and unequal access to quality foods within the household. Some researchers of the time gave a few explanations regarding the difference.[11] As primary wage earners, men were given consideration and preference at the dinner table. They also had pocket money to buy food outside the household. Women gave quality protein foods to their children first. Women also would eat after everyone else had a chance to eat.[citation needed]

Gillman and Gillman related skeletal tissue and pellagra in their research in South African Blacks. They provide some of the best evidence for skeletal manifestations of pellagra and the reaction of bone in malnutrition. They claimed radiological studies of adult pellagrins demonstrated marked osteoporosis. A negative mineral balance in pellagrins was noted, which indicated active mobilization and excretion of endogenous mineral substances, and undoubtedly impacted the turnover of bone. Extensive dental caries were present in over half of pellagra patients. In most cases, caries were associated with "severe gingival retraction, sepsis, exposure of cementum, and loosening of teeth".[12] Pellagra is no longer common in the United States.

Epidemiology

Pellagra can be common in people who obtain most of their food energy from maize ("corn" in American English), notably rural South America, where maize is a staple food. If maize is not nixtamalized, it is a poor source of tryptophan, as well as niacin. Nixtamalization corrects the niacin deficiency, and is a common practice in Native American cultures that grow corn. Following the corn cycle, the symptoms usually appear during spring, increase in the summer due to greater sun exposure, and return the following spring. Indeed, pellagra was once endemic in the poorer states of the U.S. South, such as Mississippi and Alabama, as well as among the inmates of jails and orphanages as studied by Dr. Joseph Goldberger.

Pellagra is common in Africa, Indonesia, and China. In affluent societies, a majority of patients with clinical pellagra are poor, homeless, alcohol-dependent, or psychiatric patients who refuse food.[13] Pellagra was common among prisoners of Soviet labor camps, the Gulag. It can be found in cases of chronic alcoholism. In addition, pellagra, as a micronutrient deficiency disease, frequently affects populations of refugees and other displaced people due to their unique, long-term residential circumstances and dependence on food aid. Refugees typically rely on limited sources of niacin provided to them, such as groundnuts; the instability in the nutritional content and distribution of food aid can be the cause of pellagra in displaced populations.

Symptoms

The dermatologic features of this disorder include desquamation, erythema, scaling, and keratosis of sun-exposed areas, all of which this patient had.

Pellagra is classically described by "the four D's": diarrhea, dermatitis, dementia and death.[14] A more comprehensive list of symptoms includes:

Frostig and Spies (acc. to Cleary and Cleary) described more specific psychological symptoms of pellagra as:[15]

  • Psychosensory disturbances (impressions as being painful, annoying bright lights, odors intolerance causing nausea and vomiting, dizziness after sudden movements)
  • Psychomotor disturbances (restlessness, tense and a desire to quarrel, increased preparedness for motor action)
  • Emotional disturbances

Pathophysiology

Pellagra can develop according to several mechanisms, all of which ultimately revolve around niacin deficiency. The first is simple dietary lack of niacin. Second, it may result from deficiency of tryptophan,[1] an essential amino acid found in soybeans, meat, poultry, fish, and eggs[16] that the body converts into niacin. Third, it may be caused by excess leucine, though the relationship is unclear.[2]

Alterations in protein metabolism may also produce pellagra-like symptoms. An example is carcinoid syndrome, a disease in which carcinoid tumors produce excessive serotonin. In normal patients, only one percent of dietary tryptophan is converted to serotonin; however, in patients with carcinoid syndrome, this value may increase to 70%. The diversion of tryptophan to making serotonin in patients with metastatic tumors can result in tryptophan deficiency. Carcinoid syndrome thus may produce decreased protein synthesis, niacin deficiency, and clinical manifestations of pellagra.

Prognosis

Untreated, the disease can kill within four or five years. Treatment is with nicotinamide, a chemical related to niacin. The frequency and amount of nicotinamide administered depends on the degree to which the condition has progressed.

See also

References

  1. ^ a b Pitche P (2005). "Pellagra". Sante 15 (3): 205–8. PMID 16207585. 
  2. ^ a b Bapurao S, Krishnaswamy K (1978). "Vitamin B6 nutritional status of pellagrins and their leucine tolerance". Am J Clin Nutr 31 (5): 819–24. PMID 206127. 
  3. ^ http://www.vitamins-supplements.org/amino-acids/lysine.php
  4. ^ Rajakumar, K (2000). "Pellagra in the United States: A Historical Perspective". Southern Medical Journal 98 (3): 272–277. ISSN 00384348. PMID 10728513. 
  5. ^ Stratigos JD, Katsambas A (1977). "Pellagra: a still existing disease". Br. J. Dermatol. 96 (1): 99–106. doi:10.1111/j.1365-2133.1977.tb05197.x. PMID 843444. 
  6. ^ "Definition of Pellagra". MedicineNet.com. http://www.medterms.com/script/main/art.asp?articlekey=4821. Retrieved 2007-06-18. 
  7. ^ Bollet A (1992). "Politics and pellagra: the epidemic of pellagra in the U.S. in the early twentieth century". Yale J Biol Med 65 (3): 211–21. PMC 2589605. PMID 1285449. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2589605. 
  8. ^ Swan, Patricia (2005). "Goldberger's War: The Life and Work of a Public Health Crusader (review)". Bulletin of the History of Medicine (The Johns Hopkins University Press) 79 (1): 146–7. doi:10.1353/bhm.2005.0046. http://muse.jhu.edu/journals/bulletin_of_the_history_of_medicine/v079/79.1swan.html. 
  9. ^ Miller DF (1978). "Pellagra deaths in the United States". Am. J. Clin. Nutr. 31 (4): 558–9. PMID 637029. 
  10. ^ Brenton, Barrett (2000). "Pellagra, Sex and Gender: Biocultural Perspectives on Differential Diets and Healths". Nutritional Anthropology 23 (1): 20–24. doi:10.1525/nua.2000.23.1.20. 
  11. ^ Carpenter, Kenneth (1981). Pellagra. Stroudsburg, Pa: Hutchinson Ross Pub. Co. ISBN 0-87933-364-2. 
  12. ^ Gillman, Joseph; Gillman, Theodore (1951). Perspectives in Human Malnutrition: A Contribution to the Biology of Disease from a Clinical and Pathological Study of Chronic Malnutrition and Pellagra in the African. New York, New York: Grune and Stratton. 
  13. ^ Jagielska G, Tomaszewicz-Libudzic EC, Brzozowska A (2007). "Pellagra: a rare complication of anorexia nervosa". Eur Child Adolesc Psychiatry 16 (7): 417–20. doi:10.1007/s00787-007-0613-4. PMID 17712518. 
  14. ^ Hegyi J, Schwartz R, Hegyi V (2004). "Pellagra: dermatitis, dementia, and diarrhea". Int J Dermatol 43 (1): 1–5. doi:10.1111/j.1365-4632.2004.01959.x. PMID 14693013. 
  15. ^ Cleary MJ, Cleary JP (1989). "Anorexia nervosa: a form of subclinical pellagra". Int Clin Nutr Rev 9: 137–143. ISSN 0813-9008. 
  16. ^ Haas EM. "Vitamin B3—Niacin". Excepted from: Staying Healthy with Nutrition: The Complete Guide to Diet and Nutritional Medicine. http://www.healthy.net/scr/article.asp?ID=2125. Retrieved 2007-06-18. 

Further reading

External links


Translations:

Pellagra

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Dansk (Danish)
n. - pellagra

Nederlands (Dutch)
pellagra (ziekte)

Français (French)
n. - pellagre

Deutsch (German)
n. - (Med.) Pellagra, mailändischer Aussatz

Ελληνική (Greek)
n. - (παθολ.) πελάγρα

Italiano (Italian)
pellagra

Português (Portuguese)
n. - pelagra (f) (Med.)

Русский (Russian)
пеллагра

Español (Spanish)
n. - pelagra

Svenska (Swedish)
n. - pellagra (bristsjukdom) (läk.)

中文(简体)(Chinese (Simplified))
玉蜀黍疹, 糙皮病

中文(繁體)(Chinese (Traditional))
n. - 玉蜀黍疹, 糙皮病

한국어 (Korean)
n. - 니코틴산 결핍 증후군, 펠라그라

日本語 (Japanese)
n. - ニコチン酸欠乏症候群, ペラグラ

العربيه (Arabic)
‏(الاسم) البلاغرا, الحصاف, داء الذرة : مرض معد ينشأ عن نقص التغذيه‏

עברית (Hebrew)
n. - ‮חספסת (מחלה), פלגרה‬


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