Beriberi

Beriberi
Classification and external resources
A sufferer – turn of the 20th century in southeast Asia
ICD-10	E51.1
ICD-9	265.0
DiseasesDB	14107
MedlinePlus	000339
eMedicine	ped/229 med/221
MeSH	D001602

Beriberi is a disease caused primarily by the lack of Vitamin B ₁ (thiamine) and having two different clinical pictures in adults and also another profile in newborn infants of mothers with vitamin B1 deficiency. It is a name that was used before knowing their common nature with Wernicke´s encephalopthy.

Was manifested when food was insufficient, incomplete, or kept for long. It was endemic in some regions highly dependent on rice when it was mechanical polishing generalized (white rice). It is precisely where lie the main deposits of thiamine in the rice grain.

In those nutritutional circumstances, in those cultural settings, and in populations with particular genetic profiles, when were expressed the peculiar symptomatic presentations, that then received this denomination.

Currently this disease is included within the multiple symptomatic presentations of Wernicke's encephalopathy (or Wernicke´s disease) and is maintained the use of the term "beriberi" to emphasize cardio-circulatory disturbances.

Participation of thiamine

Vitamin B ₁ or thiamine deficiency often causes damage and symptoms more noticeable, because it importance, and because it has reserves of only up to 18 days. Contrasting with cobalamin (B ₁₂ ) with reserves that can reach a couple of years.

Neurons are very sensitive to ionic and metabolic changes produced in their immediate environment, which initially affect metabolism, electrical activity and performance, but then can lead to cell death. When the disease is treated early, the damage can be cured.

After changing into the body of thiamine pyrophosphate, is involved in the following processes that help to understand the amazing diversity of symptoms when not enough:

1. Carbohydrate Metabolism allowing release of energy.
2. Neurotransmitter production of glucose derivatives such as glutamic acid or GABA.
3. Lipid metabolism, which is necessary for myelin production.
4. Amino production.
5. Neuromodulation.

Prevalence

If we limit beriberi to the consequences of poor intake, we can say that today is rare in developed countries because most foods are fortified with vitamins. But if we refer to the predominant lesions by thiamine deficiency, its prevalence is very important in all countries. In fact in the latter approach we call it "Wwernicke's encephalopathy", but worth knowing.

In autopsy series are observed the features of Wernicke lesions in approximately 2% of general cases, but the research in their medical records shows that about 85% had not been diagnosed, although only 19% would be asymptomatic. In children it was observed that only 58% were diagnosed. In alcohol abusers autopsy series are observed the damages in 12,5% or more.

Mortality reaches 17%, which means 3.4 / 1000 or about 25 million contemporaries. Be considered as in early stages may have dysfunctions prior to the production of observable lesions at necropsy. Consequently, there could be an even larger number of sufferers. It is one of the most prevalent neurological or neuropsychiatric diseases.

General symptoms and effects. Types

Its symptoms include weight loss, emotional disturbances, impaired sensory perception, weakness and pain in the limbs, and periods of irregular heart rate. Edema (swelling of bodily tissues) is common. It may increase the amount of lactic acid and pyruvic acid within the blood. In advanced cases, the disease may cause High output cardiac failure and death.

(Other symptoms are described in Wernicke's encephalopathy in Wikipedia).

The main types of beriberi are:

• Dry beriberi and Wernicke-Korsakoff syndrome affect the peripheral and central nervous system respectively.
• Wet beriberi affects the cardiovascular system, as well as other bodily systems.
• Infantile beriberi affects mostly children in developing countries.

Dry beriberi

Dry beriberi causes wasting and partial paralysis resulting from damaged peripheral nerves. It is also referred to as endemic neuritis. It is characterized by:

• Difficulty in walking
• Tingling or loss of feeling (sensation) in hands and feet (numbness)
• Loss of muscle function or paralysis of the lower legs
• Mental confusion/speech difficulties
• Pain
• Involuntary eye movements (nystagmus)
• Vomiting

A selective impairment of the large proprioceptive sensory fibers without motor impairment can occur and present as a prominent sensory ataxia, which is a loss of balance and coordination due to loss of the proprioceptive inputs from the periphery and loss of position sense.

The human brain.

Wet beriberi

Wet beriberi affects the heart; it is sometimes fatal, as it causes a combination of heart failure and weakening of the capillary walls, which causes the peripheral tissues to become edematous. It is also characterized by:

• Vasodilation leading to increased arteriovenous shunt
• Peripheral edema
• Paroxysmal nocturnal dyspnea
• Increased heart rate
• Dyspnea on exertion
• Swelling of the lower legs

Infantile beriberi

This type of beriberi is commonly found in children in developing countries. Obvious signs and symptoms are crying, but not loudly and without tears. Untreated, it can prove fatal within 24 hours.

Exams and tests

A physical examination may show signs of congestive heart failure, which include:

• Difficulty breathing with neck veins that stick out
• Enlarged heart
• Fluid in the lungs
• Rapid heartbeat
• Swelling in both lower legs
• Confusion, memory loss, delusions, and lost sensitivity to vibrations may be witnessed on late-stage patients.

A neurological exam may show signs of:

• Changes to the gait.
• Coordination problems
• Decreased reflexes
• Drooping of the eyelids

Blood tests will measure the amount of thiamine in the blood, or the decrease in the activity of the thiamine-dependent enzyme transketolase.

Causes

Beriberi or Wernicke's encephalopathy is caused by a lack of thiamine (vitamin B₁) and other nutrients. Different vitamins occurs naturally in unrefined cereals and fresh foods, particularly whole grain bread, fresh meat, legumes, green vegetables, fruit, milk, etc. Beriberi is therefore common in people whose diet excludes these particular types of nutrition e.g. as a result of famine.

Beriberi may be found in people whose diet consists mainly of polished white rice, which is very low in thiamine because the thiamin-bearing husk has been removed. It can also be seen in chronic alcoholics (Wernicke–Korsakoff syndrome). Arsenic poisoning causes alterations in cellular metabolism resulting in blockage of thiamine use which results in thiamine deficiency without any dietary shortfall. The mechanism of arsenic neuropathy may be similar to the neuropathy of thiamine deficiency [Sexton and Gowdy 1963], to the extent that arsenic inhibits the conversion of pyruvate to acetyl coenzyme A and thus blocks the Krebs cycle.

The disease was often found in Asian countries (especially in the 19th century and before), due to those countries' reliance on white rice as a staple food.

Thiamine deficiency causes neuropathy through neuron death due to its effects upon astrocytes. This causes alterations in their glutamate uptake, through changes in the levels of the astrocytic glutamate transporters EAAT1 and EAAT2 creating excitotoxicity. Other changes include those to the GABA transporter subtype GAT-3, GFAP, glutamine synthetase, the water channel protein Aquaporin 4. These create lactic acidosis, brain edema, oxidative stress, inflammation, and white matter impairment.

A rare condition known as genetic beriberi is passed down through families. People with genetic beriberi lose the ability to absorb thiamine from foods. This can happen slowly over time and symptoms occur when the person is an adult. However, because doctors may not consider beriberi in non-alcoholics, this diagnosis is often missed.

Wernicke´s encephalopathy or Beriberi can occur in breast-fed infants when the mother's body is lacking in thiamine. The condition can also affect infants who are fed unusual formulas that don't have enough thiamine.

Getting dialysis and taking high doses of diuretics can raise the risk of beriberi. It is also occasionally diagnosed in patients having undergone roux-en-y gastric bypass or other enteric diversion weight-loss surgery.

Treatment

Many authors agree that patients rarely observed lack of thiamine only. They claim that the clinical presentations are often multi deficiency. Be understood that aged food have not only lost thiamine. Or than digestive diseases, or operations, interferes with various process of nutrients.

The treatment is to provide the thiamine, other vitamins, and minerals the body is lacking. This is done with supplements which are given by injection or administered orally.

Subsequent blood tests will determine if the supplements are being effective, either in tablet form or injection. A rapid and dramatic recovery within hours can be made when this is administered to patients, and their health can be improved within an hour of starting treatment. In emergency situations where concentrated thiamin supplements are unavailable, feeding the patient with a thiamin-rich diet (e.g. whole grain brown bread) will lead to recovery, though at a much slower rate.

Etymology

The origin of the term comes from a Sinhalese phrase meaning "weak, weak" or "I cannot, I cannot", the word being reduplicated for emphasis.

In 1630, a Dutch physician named Jacob de Bondt (Jacobus Bontius; 1591-1631) encountered the disease while working in Java. In the first known description of beriberi, he wrote, "A certain very troublesome affliction, which attacks men, is called by the inhabitants beriberi (which means sheep). I believe those, whom this same disease attacks, with their knees shaking and the legs raised up, walk like sheep. It is a kind of paralysis, or rather tremor: for it penetrates the motion and sensation of the hands and feet indeed sometimes of the whole body."

History

In Asia, where polished white rice (milled rice that has had its husk, bran, and germ removed) was the common staple food of the middle class, beriberi resulting from lack of vitamin B₁ was endemic. In 1884, Takaki Kanehiro, a British-trained Japanese medical doctor of the Japanese Navy, observed that beriberi was endemic among low-ranking crew who often were provided nothing but rice, but not among crews of Western navies and officers who consumed a Western-style diet.

In 1883, Kanehiro learned of a very high incidence of beriberi among cadets on a training mission from Japan to Hawaii, via New Zealand and South America that lasted more than 9 months, resulting 169 cases of sickness and 25 deaths on a ship of 376 men. With the support of the Japanese Navy, he conducted an experiment in which another ship was deployed on the same route, except that its crew was fed a diet of meat, fish, barley, rice, and beans. At the end of the voyage, this crew had suffered only 14 cases of beriberi and no deaths. This convinced Kanehiro and the Japanese Navy that diet was the cause.

In 1897, Dr. Christiaan Eijkman, a Dutch physician and pathologist, demonstrated that beriberi is caused by poor diet, and discovered that feeding unpolished rice (instead of the polished variety) to chickens helped to prevent beriberi. The following year, Sir Frederick Hopkins postulated that some foods contained "accessory factors"—in addition to proteins, carbohydrates, fats, and salt—that were necessary for the functions of the human body. In 1901, Gerrit Grijns (May 28, 1865 – November 11, 1944), a Dutch physician and assistant to Christiaan Eijkman in the Netherlands correctly interpreted the disease as a deficiency syndrome, and between 1910 and 1913, Dr. Edward Bright Vedder established that an extract of rice bran is a treatment for beriberi.^{[citation needed]}

In 1929, Eijkman and Hopkins were awarded the Nobel Prize for Physiology or Medicine for this discovery.

See also in Wikipedia

• Wernicke's encephalopathy
• Wernicke-Korsakoff Syndrome

Bibliography

• Angstadt, John D.; Bodziner, Richard A. (2005). "Peripheral Polyneuropathy from Thiamine Deficiency following Laparoscopic Roux-en-Y Gastric Bypass". Obesity Surgery 15 (6): 890–2. doi:10.1381/0960892054222759. PMID 15978166. 
• Bay, Alexander R. (2012). Beriberi in Japan: The Making of a National Disease. Rochester, New York: University of Rochester Press. ISBN 9781580464277. 
• Hawk, Alan (2006). "The Great Disease Enemy, Kak'ke (Beriberi) and the Imperial Japanese Army". Military Medicine 171 (4): 333–9. PMID 16673750. 
• Golden, Mike (May 1997). "Diagnosing Beriberi in Emergency Situations". Field Exchange (1): 18. 
• McIntyre, Neil; Stanley, Nigel N. (1971). "Cardiac Beriberi: Two Modes of Presentation". BMJ 3 (5774): 567–9. doi:10.1136/bmj.3.5774.567. PMC 1798841. PMID 5571454. 
• Mouly, S.; Khuong, MA; Cabie, A; Saimot, AG; Coulad, JP (1996). "Beri-Beri and thiamine deficiency in HIV infection". AIDS 10 (8): 931–2. doi:10.1097/00002030-199607000-00024. PMID 8828758. 
• Shivalkar, B; Engelmann, I; Carp, L; De Raedt, H; Daelemans, R (1998). "Shoshin syndrome: Two case reports representing opposite ends of the same disease spectrum". Acta cardiologica 53 (4): 195–9. PMID 9842404. 
• Sprague, Jeb; Alexandra, Eunida (17 January 2007). "Haiti: Mysterious Prison Ailment Traced to U.S. Rice". Inter Press Service. 
• Prinzo, Z. Weise; de Benoist, B. (2009). "Meeting the challenges of micronutrient deficiencies in emergency-affected populations". Proceedings of the Nutrition Society 61 (2): 251–7. doi:10.1079/PNS2002151. PMID 12133207. 

External links

• [1]
• BeriBeri Causes - Treatment
• www.wrongdiagnosis.com : beriberi
• Medical Encyclopedia, Medline, National Institutes of Health.
• L Arturo Batres, MD. Beriberi.EMedicine.com