Beriberi: Overview, Causes, Symptoms, Treatments, Topics

Low quantities of thiamine cause the medical disease known as thiamine deficiency (Vitamin B1). Beriberi is the name of a severe and persistent variation.

There are two main forms of beriberi in adults: wet and dry. The cardiovascular system is impacted by wet beriberi, which causes a rapid heartbeat, shortness of breath, and limb oedema. The neurological system is impacted by dry beriberi, which causes discomfort, numbness in the hands and feet, disorientation, and difficulty moving the legs. Constipation and a sort of loss of appetite are also possible. Another variety, acute beriberi, which affects predominantly infants, manifests as vomiting, lactic acidosis, changes in heart rhythm, and enlargement of the heart. It also causes loss of appetite.

A diet that consists primarily of white rice, drunkenness, dialysis, persistent diarrhoea, and taking large doses of diuretics are risk factors. Rarely, thiamine absorption problems that occur in food may be brought on by a hereditary disorder. Dry beriberi comes in the forms of Wernicke encephalopathy and Korsakoff syndrome. The diagnosis is made based on the patient's symptoms, low urine thiamine levels, high blood lactate levels, and improvement after thiamine supplementation.

Thiamine injections or oral supplements are used as treatment. Symptoms typically go away with treatment in a few weeks. Food fortification can stop the disease from spreading to the general populace.

In the United States, thiamine deficiency is uncommon. In sub-Saharan Africa, it is still relatively widespread. In camps for refugees, outbreaks have been reported. Since Asia has recorded thousands of years of thiamine deficiency, it started to become more widespread in the late 1800s as a result of greater rice processing.

History

Ancient Chinese texts discussing Chinese medicine provide the earliest written accounts of thiamine deficiency. One of the first was authored by Ge Hong in his work Zhou hou bei ji fang (Emergency Formulas to Keep Up Your Sleeve), which was published somewhere in the third century. Jiao qi, which roughly translates to "foot qi," was the name given by Hong to the disease. Swelling, a lack of strength, and numbness in the feet were among the symptoms he listed. Furthermore, he acknowledged that the condition might be fatal and asserted that specific foods, such as fermented soybeans in wine, could treat it. The authors Chao Yuanfang and Sun Simiao are better renowned for their descriptions of "foot qi" in their respective works Zhu bing yuan hou lun and Bei ji qian jin yao fang, respectively.

The Japanese medical doctor Takaki Kanehiro of the Imperial Japanese Navy studied beriberi in the late 19th century. Beriberi was a serious issue in the Japanese navy; sailors got sick on average four times a year from 1878 to 1881, with 35% of those cases being beriberi cases. [59] In 1883, Takaki discovered a very high incidence of beriberi among cadets on a training mission from Japan to Hawaii, via New Zealand and on a ship with 376 men, the journey lasted more than nine months and resulted in 169 sickness cases and 25 fatalities. He experimented with the help of the Japanese Navy, sending a different ship in the same direction but feeding its crew a meal of meat, fish, barley, rice, and beans. There were only 14 cases of beriberi and no fatalities among this crew at the end of the expedition. This led Takaki and the Japanese Navy to conclude that diet was the root of the problem. In 1884, Takaki noticed that beriberi was more prevalent among lower-ranking crew members who frequently received free rice and ate little else than that, but not among crews of Western navies or among Japanese officers who ate a more varied diet.

A Dutch doctor and pathologist named Christiaan Eijkman discovered that giving unpolished rice to chickens instead of polished rice helped to prevent beriberi in 1897. He also showed that beriberi is caused by a bad diet. The following year, Sir Frederick Hopkins proposed that some foods contained "accessory factors"—in addition to proteins, carbohydrates, fats, and salt—that were required for the functions of the human body. In 1901, Gerrit Grijns, a Dutch doctor and Christiaan Eijkman's assistant in the Netherlands, correctly identified beriberi as a deficiency syndrome. And between 1910 and 1913, Edward Bright Vedder established that an extract of rice bran helped to treat beriberi. Eijkman and Hopkins received the Nobel Prize in Physiology or Medicine in 1929 for their contributions.

Etymology

Although the Oxford English Dictionary claims that the term "beriberi" is derived from a Sinhalese phrase that means "weak, weak" or "I cannot, I cannot," the word being duplicated for emphasis, the phrase's etymology is disputed. Numerous meanings, including "weakness," "sailor," and even "sheep," have been ascribed to it, including those from Hindi, Arabic, and a few other languages. Heinrich Botho Scheube was one of the authors to mention such potential sources. It is impossible to pinpoint the word's origin, noted Edward Vedder in his book Beriberi (1913). When Diogo do Couto documented the shortage in India, he at least used the word "berbere" in his literature as early as 1568.

Signs and Symptoms

Weakness and soreness in the limbs, mental disorders, decreased sensory awareness, weight loss, and irregular heartbeat are all signs of beriberi. Oedema, or the swelling of bodily tissues, is typical. Lactic acid and pyruvic acid levels in the blood may rise as a result. Advanced disease stages might result in high-output heart failure and death.

Wernicke's encephalopathy, a disorder predominantly linked to thiamine shortage that affects the nervous system, has symptoms that might happen simultaneously.

Four categories are used to categorise Beriberi. The fourth condition, gastrointestinal beriberi, was identified in 2004 and is historical for the first three.

• Peripheral nervous system disorders are especially common with dry beriberi.

• Heart and other physiological systems are particularly impacted by wet beriberi.

• Babies of malnourished moms suffer from infantile beriberi.

• Beriberi which affects the gastrointestinal tract can have an impact on other physiological systems as well.

Dry Beriberi

Due to injured peripheral nerves, dry beriberi results in wasting and partial paralysis. It is additionally known as endemic neuritis. It is distinguished by:

• Having trouble walking

• Numbness or loss of sensation (tingling) in the hands, feet, or legs

• Tendon reflex loss

• lower-leg paralysis or loss of muscular function

• Speech and cognitive difficulties

• Pain

• uncontrollable eye motions (nystagmus)

• Vomiting

Ataxia, which is a loss of balance and coordination caused by a lack of proprioceptive inputs from the periphery and a loss of position sensation, can be a conspicuous symptom of selective impairment of the vast proprioceptive sensory fibres without motor impairment.

Brain Beriberi

Dry beriberi has three different forms: Wernicke's encephalopathy (WE), Korsakoff syndrome, and Wernicke-Korsakoff syndrome.

Wernicke's encephalopathy is the most common symptom of thiamine deficiency in Western culture, though it can also happen in people who have impaired nutrition from other conditions, such as gastrointestinal disease, people with HIV/AIDS, or people who overeat without taking enough B-vitamin supplements.

This is a stunning neuropsychiatric illness that is characterised by mental instability, aberrant posture and gait, and paralysis of ocular movements. In general, it's thought that Korsakoff syndrome develops in people with WE who have already seen a decline in their brain function. The symptoms of this amnestic-confabulatory condition include anterograde and retrograde amnesia, impairment of conceptual functioning, and a loss of spontaneity and initiative.

Beriberi in alcoholics could result from

• Nutritional deficiencies: Alcoholics frequently consume less thiamine than is advised.

• Thiamine absorption from the GI tract is reduced because acute alcohol exposure disturbs the active transport of thiamine into enterocytes.

• Hepatic steatosis or fibrosis causes decreased liver thiamine reserves.

• impaired thiamine utilisation chronic alcohol use also causes magnesium deficiency, which is necessary for thiamine to bind to thiamine-using enzymes in the cell.

• Thiamine insufficiency will worsen due to the ineffective utilisation of any thiamine that does enter the cells.

Some thiamine deficiency-related deficits, including impaired brain function, can be remedied by better nutrition and abstaining from alcohol, but in more severe cases, Wernicke-Korsakoff syndrome causes long-term harm.

Wet Beriberi

Wet beriberi affects the cardiovascular and circulatory systems. The combination of heart failure and weakened capillary walls, which result in edematous peripheral tissues, can be deadly in some cases.

Wet beriberi is distinguished by:

• higher heart rate

• High-output heart failure results from vasodilation, which lowers systemic vascular resistance.

• Jugular venous pressure increase

• breathlessness (dyspnea) when exerting oneself

• intermittent nocturnal dyspnea

• Emphysema peritonei (lower leg swelling) or widespread oedema (body swells in several places)

• enlarged heart disease

Gastrointestinal Beriberi

Abdominal pain is a symptom of gastrointestinal beriberi. It is identifiable by:

• stomach ache

• Nausea

• Lactic acidosis

• Vomiting

Infants

Between the ages of two and six months, infantile beriberi typically affects children whose moms don't get enough thiamine. Wet or dry beriberi are two possible symptoms.

The infant has cyanosis and dyspnea during the acute form before succumbing to cardiac failure. Infantile beriberi symptoms include those listed below:

• When a youngster has hoarseness from nerve paralysis, they may attempt to scream but either produce no noise at all or only faint groans.

• As the illness worsens, the person loses weight, gets thinner, and finally develops marasmus.

• Vomiting

• Diarrhoea

• Oedema and a pale complexion

• Irrational behaviour

• Changes to the cardiovascular system, particularly tachycardia (rapid heart rate)

• At the latter end, there may be sporadic convulsions

Causes

Beriberi is frequently brought on by consuming a diet high in polished rice, a calorie-dense food common in Asia, or cassava root, a calorie-dense food common in sub-Saharan Africa, with little or no thiamine-containing meat or vegetables.

Other factors, such as illnesses or procedures on the digestive tract, alcoholism, dialysis, or hereditary abnormalities, may also be to blame in addition to poor nutrition. Wernicke's encephalopathy is a result of all those factors, which mostly impact the central nervous system.

One of the most common neurological or neuropsychiatric conditions is Wernicke's illness. About 2% of all cases in the autopsy series have Wernicke lesions' characteristics. Even though only 19% of patients would have no symptoms, medical record analysis reveals that roughly 85% had not yet received a diagnosis. Only 58% of kids had been diagnosed. Autopsy studies on alcohol abusers revealed brain impairment at rates of at least 12.5%. Wernicke's illness accounts for 17% of all disease-related deaths, or 3.4/1000 or over 25 million people in the present. Given that the early stages of the disease may have dysfunctions before they produce obvious lesions at necropsy, the number of patients with Wernicke's disease may even be larger. Additionally, prenatal harm and the following diseases can affect an untold number of people.

Genetics

Rare but dangerous genetic conditions that affect thiamine transport exist. An autosomal recessive condition called thiamine-responsive megaloblastic anaemia syndrome (TRMA) with diabetes mellitus and sensorineural deafness is brought on by mutations in the high-affinity thiamine transporter gene SLC19A2[34]. The lack of systemic thiamine-deficiency symptoms in TRMA patients points to redundancy in the thiamine delivery system. As a result, the second high-affinity thiamine transporter SLC19A3 was found. Leigh disease, also known as subacute necrotising encephalomyelopathy, is a genetic condition that usually affects infants in their first few years of life and is always deadly. Leigh's illness and we have some pathological characteristics, which led researchers to speculate that a problem with thiamine metabolism was to blame for both diseases. Atypical pyruvate dehydrogenase complex activation has been one of the most recurrent observations. Biotin-thiamine-responsive basal ganglia illness, which is treated with therapeutic doses of thiamine and biotin, another B vitamin, has been linked to mutations in the SLC19A3 gene.

Subacute necrotizing encephalomyelopathy, opsoclonus myoclonus syndrome (a paraneoplastic condition), and Nigerian seasonal ataxia are some other diseases where thiamine has been shown to play a role (or African seasonal ataxia). Additionally, it has been claimed that thiamine can be used to treat several hereditary diseases of ThDP-dependent enzymes.

Pathophysiology

Thiamine quickly runs out in the human body and has an 18-day half-life, especially when metabolic demands outweigh intake. Thiamine pyrophosphate (TPP), a thiamine derivative, links the citric acid cycle with the breakdown of carbohydrates. It is a cofactor in the citric acid cycle. Because thiamine shortage disrupts the citric acid cycle, a crucial metabolic route involved in the control of carbohydrate, lipid, and amino acid metabolism, many compounds, including the neurotransmitters glutamic acid and GABA, cannot be produced. Furthermore, thiamine may participate directly in neuromodulation.

Diagnosis

Measurement of the transketolase enzyme's activity in erythrocytes is required for a positive diagnosis of thiamine deficiency (Erythrocyte transketolase activation assay). As an alternative, thiamine and its phosphorylated derivatives can be found in whole blood, tissues, food, animal feed, and pharmaceutical preparations immediately after being transformed into fluorescent thiochrome derivatives (thiochrome assay) and separated by high-performance liquid chromatography (HPLC). Thiamine levels in materials can now be quantified and tracked using capillary electrophoresis (CE) methods and in-capillary enzyme reaction methods. Between 20 and 100 g/L is the typical range for thiamine content in EDTA blood.

Treatment

Thiamine is a commonly used standalone treatment for beriberi. The healing process is quick and dramatic when thiamine is administered intravenously (and later orally).

It may take several months of thiamine therapy for peripheral neuropathy to improve.

Epidemiology

Even in the twenty-first century, beriberi is a common nutritional illness in jails. In a Taiwanese correctional facility in 1999, there was a beriberi outbreak. Rice washing before cooking is a custom that has been linked to high disease and death rates in Haiti's overcrowded jails in 2007. In the Ivory Coast, 64% of a group of prisoners receiving harsh punishment had beriberi. Before starting therapy, inmates displayed neurological indicators of dry or wet beriberi (tingling: 41%), cardiovascular signals of dyspnea (42%), thoracic pain (35%), and lower limb edemas (51%). With therapy, the recovery rate was almost 97%.

The risk of beriberi may be increased in populations under severe stress. War refugees and other displaced groups are particularly vulnerable to micronutrient deficiencies, such as beriberi. The acute nutritional deprivation brought on by famine can also lead to beriberi, albeit clinical assessments may miss the symptoms or conceal them with other hunger-related issues. Rarely, an excessive diet for weight loss might cause beriberi, which is a famine-like condition.

Prevention

An individual must include adequate thiamine in their diet to prevent beriberi. Generally speaking, thiamine deficiency is not a concern for someone who follows a balanced, healthy diet that prioritises whole foods.

Natural sources of thiamine in food include:

• Meat

• Nuts and seeds

• seafood,

• dairy products,

• beans and legumes

Additionally, producers fortify a variety of foods with vitamins, including thiamine, bread, morning cereals, and baked goods.

Others Animals Affected by the Deficiency of Beriberi

Poultry

Deficits in this vitamin do not occur with commercial diets because the majority of feeds used in poultry diets include sufficient amounts of vitamins to meet the requirements of this species. At least, this was the prevailing viewpoint in the 1960s.

Three weeks after being fed an inadequate diet, mature hens begin to exhibit symptoms. It can appear in young chickens as early as two weeks of age. In young chickens, the onset is abrupt, accompanied by anorexia and a shaky walk. Later, locomotor symptoms start, with the toe flexor appearing to be paralysed. The affected animal adopts a posture known as opisthotonos, or "stargazing," in which it sits on its hocks with its head tilted back. Response to vitamin treatment happens rather quickly, a few hours after.

Ruminants

The most frequent condition caused by thiamine deficiency in young ruminant and nonruminant animals is polioencephalomalacia (PEM). PEM can cause muscle spasms, profuse but brief diarrhoea, listlessness, circling movements, stargazing, or opisthotonus (head drawn back over the neck). The most frequent cause is high-carbohydrate feeds that encourage the growth of thiaminase-producing bacteria, but other causes include dietary thiaminase consumption (for example, in bracken fern) and thiamine absorption suppression by high sulphur intake. Bacillus aneurinolyticus or Clostridium sporogenes infection is another cause of PEM. These bacteria create thiaminases, which in the infected animal can result in an acute thiamine deficiency.

Snakes

Goldfish and feeder minnows make up a major portion of a snake's diet, making them vulnerable to thiamine shortage. Since goldfish contain the enzyme thiaminase, which breaks down thiamine, this issue is frequently seen in captivity while raising garter and ribbon snakes.

Wild Birds and Fish

Since 1982, a paralytic illness afflicting wild birds in the Baltic Sea region has been linked to thiamine deficiency. The capacity to fly and speak is lost in this disease, and eventually, the wings and legs become paralysed and the patient dies. It is also difficult to keep the wings folded down the side of the body when at rest. It primarily affects birds weighing between 0.5 and 1 kg, including the common eider, European herring gull, and common starling (Somateria mollissima). Researchers remarked, "We are open to the idea that other animal groups may suffer thiamine deficiency, as well, because the analysed species occupy a wide variety of ecological niches and locations in the food web

Several bird species, particularly the European herring gull, have been experiencing major extinctions in the southernmost Swedish counties of Blekinge and Skane since the early 2000s. Recent times appear to have impacted species from other classes. Mammals like the Eurasian elk (Alces alces) have also experienced extremely high mortality rates. Salmon (Salmo salar) mortality in the river Morrumsan has been reported to be high. The common factor that is used for analysis is thiamine deficiency. The County Administrative Board of Blekinge requested that a more thorough investigation be launched in April 2012 after finding the situation to be extremely concerning.