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Thiamin (vitamin B 1 ) is widely available in the diet. Thiamin is involved in carbohydrate, fat, amino acid, glucose, and alcohol metabolism. Thiamin is essentially nontoxic.
Thiamin deficiency (causing beriberi) is most common among people subsisting on white rice or highly refined carbohydrates in developing countries and among alcoholics. Symptoms include diffuse polyneuropathy, high-output heart failure, and Wernicke-Korsakoff syndrome. Thiamin is given to help diagnose and treat the deficiency.
Primary thiamin deficiency is caused by
It is commonly due to a diet of highly refined carbohydrates (eg, polished rice, white flour, white sugar) in developing countries. It also develops when intake of other nutrients is inadequate, as may occur in young adults with severe anorexia; it often occurs with other B vitamin deficiencies.
Secondary thiamin deficiency is caused by
In alcoholics, many mechanisms contribute to thiamin deficiency; they include decreased intake, impaired absorption and use, increased demand, and possibly an apoenzyme defect.
Deficiency causes degeneration of peripheral nerves, thalamus, mammillary bodies, and cerebellum. Cerebral blood flow is markedly reduced, and vascular resistance is increased.
The heart may become dilated; muscle fibers become swollen, fragmented, and vacuolized, with interstitial spaces dilated by fluid. Vasodilation occurs and can result in edema in the feet and legs. Arteriovenous shunting of blood increases. Eventually, high-output heart failure may occur.
Early symptoms are nonspecific: fatigue, irritability, poor memory, sleep disturbances, precordial pain, anorexia, and abdominal discomfort.
Dry beriberi refers to peripheral neurologic deficits due to thiamin deficiency. These deficits are bilateral and roughly symmetric, occurring in a stocking-glove distribution. They affect predominantly the lower extremities, beginning with paresthesias in the toes, burning in the feet (particularly severe at night), muscle cramps in the calves, pains in the legs, and plantar dysesthesias. Calf muscle tenderness, difficulty rising from a squatting position, and decreased vibratory sensation in the toes are early signs. Muscle wasting occurs. Continued deficiency worsens polyneuropathy, which can eventually affect the arms.
Wernicke-Korsakoff syndrome, which combines Wernicke encephalopathy (see Wernicke’s Encephalopathy) and Korsakoff psychosis (see Korsakoff’s Psychosis), occurs in some alcoholics who do not consume foods fortified with thiamin. Wernicke encephalopathy consists of psychomotor slowing or apathy, nystagmus, ataxia, ophthalmoplegia, impaired consciousness, and, if untreated, coma and death. It probably results from severe acute deficiency superimposed on chronic deficiency. Korsakoff psychosis consists of mental confusion, dysphonia, and confabulation with impaired memory of recent events. It probably results from chronic deficiency and may develop after repeated episodes of Wernicke encephalopathy.
Cardiovascular (wet) beriberi is myocardial disease due to thiamin deficiency. The first effects are vasodilation, tachycardia, a wide pulse pressure, sweating, warm skin, and lactic acidosis. Later, heart failure develops, causing orthopnea and pulmonary and peripheral edema. Vasodilation can continue, sometimes resulting in shock.
Infantile beriberi occurs in infants (usually by age 3 to 4 wk) who are breastfed by thiamin-deficient mothers. Heart failure (which may occur suddenly), aphonia, and absent deep tendon reflexes are characteristic.
Because thiamin is necessary for glucose metabolism, glucose infusions may precipitate or worsen symptoms of deficiency in thiamin-deficient people.
Diagnosis is usually based on a favorable response to treatment with thiamin in a patient with symptoms or signs of deficiency. Similar bilateral lower extremity polyneuropathies due to other disorders (eg, diabetes, alcoholism, vitamin B 12 deficiency, heavy metal poisoning) do not respond to thiamin. Single-nerve neuritides (mononeuropathies—eg, sciatica) and multiple mononeuropathies (mononeuritis multiplex) are unlikely to result from thiamin deficiency.
Electrolytes, including Mg, should be measured to exclude other causes. For confirmation in equivocal cases, erythrocyte transketolase activity and 24-h urinary thiamin excretion may be measured.
Diagnosis of cardiovascular beriberi can be difficult if other disorders that cause heart failure are present. A therapeutic trial of thiamin can help.
Ensuring that dietary supplies of thiamin are adequate is important regardless of symptoms.
Because IV glucose can worsen thiamin deficiency, alcoholics and others at risk of thiamin deficiency should receive IV thiamin 100 mg before receiving IV glucose solutions.
The thiamin dose is.
Heart failure is also treated.
For Wernicke-Korsakoff syndrome, thiamin 50 to 100 mg IM or IV bid must usually be given for several days, followed by 10 to 20 mg once/day until a therapeutic response is obtained. Anaphylactic reactions to IV thiamin are rare. Symptoms of ophthalmoplegia may resolve in a day; improvement in patients with Korsakoff psychosis may take 1 to 3 mo. Recovery from neurologic deficits is often incomplete in Wernicke-Korsakoff syndrome and in other forms of thiamin deficiency.
Because thiamin deficiency often occurs with other B vitamin deficiencies, multiple water-soluble vitamins are usually given for several weeks. Patients should continue to consume a nutritious diet, supplying 1 to 2 times the daily recommended intake of vitamins; all alcohol intake should stop.
The risk of thiamin deficiency is increased in people who subsist on highly refined carbohydrates such as polished rice and white flour (as occurs in developing countries) or who are alcoholics.
Early findings can be nonspecific; peripheral neurologic deficits, high-output heart failure, and Wernicke-Korsakoff syndrome (mainly in alcoholics) may also develop.
Diagnose based on clinical findings, including a favorable response to treatment with supplemental thiamin.
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