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Pellagra

Definition:
Pellagra is a lack of a sufficient amount of vitamin B3 (niacin) in the body.It  is a vitamin deficiency disease most commonly caused by a chronic lack of niacin (vitamin B3) in the diet. Niacin is essential for optimal cellular health. Pellagra is caused by an inability of the body to absorb or process niacin or a lack of niacin and/or tryptophan the diet. Pellagra that results from inadequate intake of niacin and/or tryptophan is most common in developing countries of the world or in places where there is poverty and poor nutrition. People at risk for pellagra include those with a poor diet that is lacking in niacin and/or tryptophan.

It can be caused by decreased intake of niacin or tryptophan, and possibly by excessive intake of leucine. 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, meaning that another potential cause of pellagra is lysine deficiency

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Pellagra can also result from a disease, disorder or condition that affects the absorption or processing of niacin in the body. This is called secondary pellagra. There are many causes of secondary pellagra, including ulcerative colitis, excessive diarrhea, liver cirrhosis, chronic alcoholism, Hartnup disease and carcinoid tumors. Pellagra can also be a side effect of certain medications. People with these conditions are at risk for developing secondary pellagra Symptoms of pellagra typically affect the skin, gastrointestinal system and the nervous system. Pellagra can be serious, even fatal, if untreated. Complications include coma and death. For more details on symptoms and complications.

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. It has now been shown that the lime treatment makes niacin nutritionally available and reduces the chance of developing pellagra. 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. Pellagra 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(1). It was an endemic disease in northern Italy, where it was named “pelle agra” (pelle = skin; agra = sour) by Francesco Frapoli of Milan.[3] 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. It was not until later that the lack of pellagra outbreaks in Mesoamerica, where maize is a major food crop (and is processed), was noted and the idea was considered that the causes of pellagra may be due to factors other than toxins.

In the early 1900s, pellagra reached epidemic proportions in the American South. There were 1,306 reported pellagra deaths in South Carolina 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. 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 that pellagra was linked to diet by inducing the disease in prisoners, using the Spartanburg Pellagra Hospital as his clinic. By 1926, Goldberger established that a balanced diet or a small amount of brewer’s yeast prevented pellagra. Skepticism nonetheless persisted in the medical community until 1937, when Conrad Elvehjem showed that 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.

In the research conducted between 1900–1950, it was found that the number of cases of women with pellagra was consistently double the number of cases of afflicted men. This is thought to be due to the inhibitory effect of estrogen on the conversion of the amino acid tryptophan to niacin. It is also thought to be due to the differential and unequal access to quality foods within the household. Some researchers of the time gave a few explanations regarding the difference. 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 protein quality foods to their children first. Women also would eat after everyone else had a chance to eat. Women also upheld the triad of maize, molasses and fat back pork which combine to contribute to cause pellagra.[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


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 three D’s”: diarrhea, dermatitis and dementia. A more comprehensive list of symptoms includes:

*High sensitivity to sunlight
*Aggression
*Dermatitis, alopecia, oedema
*Smooth, beefy red glossitis
*Red skin lesions
*Insomnia
*Weakness
*Mental confusion
*Ataxia, paralysis of extremities, peripheral neuritis
*Diarrhea
*Dilated cardiomyopathy
*Eventually dementia

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

*Psycho-sensory disturbances (impressions as being painful, annoying bright lights, odours intolerance causing nausea and vomiting, dizziness after sudden movements)
*Psycho-motor disturbances (restlessness, tense and a desire to quarrel, increased preparedness for motor action)
*Emotional disturbances.

Causes:
Pellagra is caused by having too little niacin or tryptophan in the diet. It can also occur if the body fails to absorb these nutrients. It may develop after gastrointestinal diseases or with alcoholism.

The disease is common in parts of the world where people have a lot of corn in their diet.

Pellagra can be common in people who obtain most of their food energy from maize (often called “corn”), notably rural South America where maize is a staple food. Maize is a poor source of tryptophan as well as niacin if it is not nixtamalized. Nixtamalization of the corn 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, like 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. It was common amongst prisoners of Soviet labor camps, the Gulag. It can be found in cases of chronic alcoholism. In addition, pellagra is a micronutrient deficiency disease that 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.

List of causes of Pellagra :  Following is a list of causes or underlying conditions ( Misdiagnosis of underlying causes of Pellagra) that could possibly cause Pellagra includes:

•Dietary deficiency of vitamin B3 (niacin)
•Malnutrition
•Malabsorption

The follow list shows some of the possible medical causes of Pellagra that are listed by the Diseases Database:

•Hartnup’s disease
•Carcinoid tumours and carcinoid syndrome

Pellagra Causes: Book Excerpts
•Causes and incidence – Vitamin B deficiencies
•Causes and incidence – Vitamin C deficiency
•Causes and incidence – Vitamin D deficiency
•Causes and incidence – Vitamin E deficiency
•Causes – Vitamin K deficiency
•Causes and incidence – Vitamin A deficiency

Pellagra Related Medical ConditionsTo research the causes of Pellagra, consider researching the causes of these these diseases that may be similar, or associated with Pellagra:

•Alcoholism
•Dermatitis
•Insomnia
•Dementia
•Diarrhoea
•Ataxia
•Nicotinamide
•Niacin
•Vitamin B3
•Carcinoid syndrome
Pellagra: Causes and TypesCauses of Broader Categories of Pellagra: Review the causal information about the various more general categories of medical conditions:

•Vitamin deficiency
•Vitamin B deficiency
•more types…»


Diagnosis:-

Diagnosis is purely based on the patient’s collection of symptoms, together with information regarding the patient’s diet. When this information points to niacin deficiency, replacement is started, and the diagnosis is then partly made by evaluating the patient’s response to increased amounts of niacin. There are no chemical tests available to definitively diagnose pellagra.

Pellagra Diagnosis: Book Excerpts
•Diagnosis – Vitamin B deficiencies
•Diagnosis – Vitamin C deficiency
•Diagnosis – Vitamin D deficiency
•Diagnosis – Vitamin E deficiency
•Diagnosis – Vitamin K deficiency
•Diagnosis – Vitamin A deficiency


Treatment:

The first step in the treatment of pellagra is prevention. Primary pellagra can be prevented with eating a diet that is rich in foods that contain niacin and tryptophan. Niacin is found in whole grains, meats, and fish and in fortified grains and cereals. Tryptophan is found in milk and eggs.

Taking supplements that contain niacin may also be needed to prevent pellagra in people with poor diets. High doses of niacin can be toxic to some people with gout, diabetes, asthma, liver disease, ulcers, or who are on antihypertensive medication.

If diagnosed and treated promptly, pellagra has a good prognosis and cure rate. Early treatment eliminates the risk of developing serious permanent complications, such as dementia, psychosis, coma and death.

Treatment of pellagra generally includes oral or intravenous niacin supplementation. Regular medical care and monitoring of the condition and treatment of any underlying diseases, such as ulcerative colitis, excessive diarrhea, liver cirrhosis, chronic alcoholism, Hartnup disease and carcinoid tumors, are also necessary to ensure a good prognosis.

Treatment of pellagra usually involves supplementing the individual’s diet with a form of niacin called niacinamide (niacin itself in pure supplementation form causes a number of unpleasant side effects, including sensations of itching, burning, and flushing). The niacinamide can be given by mouth (orally) or by injection (when diarrhea would interfere with its absorption). The usual oral dosage is 300-500 mg each day; the usual dosage of an injection is 100-250 mg, administered two to three times each day. When pellagra has progressed to the point of the encephalopathic syndrome, a patient will require 1,000 mg of niacinamide orally, and 100-250 mg of niacinamide by injection. Once the symptoms of pellagra have subsided, a maintenance dose of niacin can be calculated, along with attempting (where possible) to make appropriate changes in the diet. Because many B-complex vitamin deficiencies occur simultaneously, patients will usually require the administration of other B-complex vitamins as well.

Treatment List for PellagraThe list of treatments mentioned in various sources for Pellagra includes the following list. Always seek professional medical advice about any treatment or change in treatment plans.

•Vitamin B3 – possibly used for related severe vitamin B3 deficiency
•Vitamin B3
•Diet – high protein/high calorie, and including meat, milk, peanuts, green leafy vegetables, whole grains and brewers yeast
•Nicotinamide or niacin supplementation
•B complex vitamin supplementation as patients are often suffer from other vitamin deficiencies

Drugs and Medications used to treat Pellagra:Note:You must always seek professional medical advice about any prescription drug, OTC drug, medication, treatment or change in treatment plans.

Some of the different medications used in the treatment of Pellagra include:

•Niacinamide
•Thiamine
•Betaxin
•Thiamilate
•Benerva
•Nicotinic Acid
•Vitamin B3
•Dynamo
•NoDoz Plus

Prognosis:
Untreated pellagra will continue progressing over the course of several years, and is ultimately fatal. Often, death is due to complications from infections, massive malnutrition brought on by continuous diarrhea, blood loss due to bleeding from the gastrointestinal tract, or severe encephalopathic syndrome.

Considerations :
Individuals with diabetes should take supplementary niacin with caution, as it can raise blood sugar levels. Long-term niacin therapy may also increase the risk of gout. At least one study has pointed out the danger of taking too much niacin, especially for elderly people. The short-term side effects of niacin overdose include:

*Flushing.
*Itching.
*Skin disorders.

Over the long term, high doses of niacin can be dangerous. Taking as little as 500 mg of niacin per day over a period of several months may result in liver damage.


Recomendations:

Eat plenty of foods that are high in B vitamins, such as avocados, bananas, broccoli, collards, figs, legumes, nuts and seeds, peanut butter, potatoes, prunes, tomatoes, and whole grain or enriched bread and cereal.

Include in the diet halibut, salmon, sunflower seeds, swordfish, tuna, and white skinless breast of chicken and turkey. These foods are good sources of the amino acid tryptophan, which is converted into niacin in the body.

Nutritional Suppliments :
Unless otherwise specified, the dosages recommended here are for adults. For a child between the ages of 12 and 17 years, reduce the dose to 3/4 the recommended amount. For a child between the ages of 6 and 12, use 1/2 the recommended dose, and for a child under the age of 6, use 1/4 the recommended amount.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.


Resources:

http://en.wikipedia.org/wiki/Pellagra
http://www.wrongdiagnosis.com/p/pellagra/intro.htm
http://www.myoptumhealth.com/portal/ADAM/item/Pellagra
http://www.moondragon.org/health/disorders/pellagra.html

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