Beriberi

Beriberi
A sufferer - Turn of the 20th Century in southeast Asia
ICD-10	E51.1
ICD-9	265.0
DiseasesDB	14107
eMedicine	ped/229  med/221
MeSH	D001602

WikiDoc Resources for Beriberi
Articles
Most recent articles on Beriberi Most cited articles on Beriberi Review articles on Beriberi Articles on Beriberi in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Beriberi Images of Beriberi Photos of Beriberi Podcasts & MP3s on Beriberi Videos on Beriberi
Evidence Based Medicine
Cochrane Collaboration on Beriberi Bandolier on Beriberi TRIP on Beriberi
Clinical Trials
Ongoing Trials on Beriberi at Clinical Trials.gov Trial results on Beriberi Clinical Trials on Beriberi at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Beriberi NICE Guidance on Beriberi NHS PRODIGY Guidance FDA on Beriberi CDC on Beriberi
Books
Books on Beriberi
News
Beriberi in the news Be alerted to news on Beriberi News trends on Beriberi
Commentary
Blogs on Beriberi
Definitions
Definitions of Beriberi
Patient Resources / Community
Patient resources on Beriberi Discussion groups on Beriberi Patient Handouts on Beriberi Directions to Hospitals Treating Beriberi Risk calculators and risk factors for Beriberi
Healthcare Provider Resources
Symptoms of Beriberi Causes & Risk Factors for Beriberi Diagnostic studies for Beriberi Treatment of Beriberi
Continuing Medical Education (CME)
CME Programs on Beriberi
International
Beriberi en Espanol Beriberi en Francais
Business
Beriberi in the Marketplace Patents on Beriberi
Experimental / Informatics
List of terms related to Beriberi

Editor-In-Chief: Timothy R. Koch, M.D., Professor of Medicine, Gastroenterology, Georgetown University School of Medicine. You can email Dr. Koch here; Bikram Bal, M.D., Section of Gastroenterology, Washington Hospital Center.

Contributors: C. Michael Gibson, M.S., M.D.

Please Join in Editing This Page and Apply to be an Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [1] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

Overview

Beriberi is a dietary-deficiency disease caused by a lack of thiamine in the diet. Thiamine, initially named "the anti-beriberi factor" in 1926 was the first B vitamin to be identified and is therefore referred to as vitamin B1. Thiamine is soluble in water and partly soluble in alcohol. It consists of a pyrimidine and a thiazole moiety, both of which are essential for its activity.

Thiamine functions in the decarboxylation of α-ketoacids, such as pyruvate α-ketoglutarate, and branched-chain amino acids and thus is a source of energy generation. In addition, thiamine pyrophosphate acts as a coenzyme for a transketolase reaction that mediates the conversion of hexose and pentose phosphates.

Etymology

The origin of the word is from a Sinhalese phrase meaning "I cannot, I cannot", the word being doubled for emphasis.

History

It is a disease that has killed probably close to a million people worldwide. References to this disease can be found in Chinese medical texts dating as far back as 2697 BC. In the 19th century it was the “national disease” of Japan. It first attracted the attention of Western scientists in the 1880s, when Dutch military personnel experienced an epidemic of the disease while operating in Sumatra. Its association with the consumption of highly polished rice was noted in the first decade of the twentieth century. It took some 50 years and lifetimes of dedication by dozens of scientists from many different fields and of various nationalities before the mysteries of beriberi were unraveled. Christiaan Eijkman a Dutch physician and pathologist first demonstrated that beriberi is caused by poor diet led to the discovery of vitamins. Together with Sir Frederick Hopkins, he was awarded the 1929 Nobel Prize for Physiology or Medicine for the discovery. But the ability to produce a synthetic vitamin on a commercial scale has been by no means the end of the story.

Differential Diagnosis of Underlying Causes

Thiamine deficiency can be a result of:

1. Inadequate intake:

Alcoholism

Anorexia

Intentional dieting

Starvation

Bulimia

Protein energy malnutrition in developing countries

Total parenteral nutrition

Infants breast fed by thiamine deficient mothers

2. Increased losses:

Protracted vomiting in chemotherapy patients

Hyperemesis gravidarum in pregnant women

3. Inadequate absorption

Post gastric bypass surgery patients

Genetic loss of ability to absorb thiamine

Pathophysiology

Thiamine deficiency is associated with high output heart failure due to the vasodilation that develops as a result of pyruvate and lactate accumulation .

Diagnosis

Signs and Symptoms

There are two forms of the disease: wet beriberi and dry beriberi.

• Wet beriberi affects the heart; it is sometimes fatal, as it causes a combination of heart failure and weakening of the capillary walls (see capillary leak syndrome), which causes the peripheral tissues to become edematous.
• Dry beriberi causes wasting and partial paralysis resulting from damaged peripheral nerves. It is also referred to as endemic neuritis.

Cardiovascular or wet beriberi: is manifested by heart hypertrophy and dilatation (particularly of the right ventricle), tachycardia, respiratory distress, edema of the legs and heart failure with high cardiac output. Severe lactic acidosis is characteristic (1).

Neurological or dry beriberi: is manifested by exaggeration of deep tendon reflexes, polyneuritis (sometimes associated with paralysis) that typically affects lower extremities and in a subsequent stage, upper extremities, muscle weakness and pain, and convulsions.

Gastrointestinal beriberi: Gastrointestinal symptoms are primarily due to the delayed emptying of the stomach and dilation of the colon. Loss of appetite, vague abdominal complaints, and constipation are common manifestations. As the disease progresses nausea and vomiting may occur.

Wernicke-Korsakoff syndrome: Wernicke's disease is a triad of nystagmus, ophthalmoplegia, and ataxia, along with confusion. Korsakoff's psychosis is impaired short-term memory and confabulation with otherwise grossly normal cognition. This combination is almost exclusively described in chronic alcoholics with thiamine deficiency. The two entities are not separate diseases, but a spectrum of signs and symptoms. Genetic abnormalities may underlie a predisposition to Wernicke-Korsakoff syndrome.

Bariatric beriberi: Thiamine deficiency after Roux-en-Y gastric bypass surgery is common. After Roux-en-Y gastric bypass, thiamine deficiency has been reported in 18% of patients at one year post-surgical follow-up in a university hospital setting. Our group previously published that persistent thiamine deficiency is associated with small bowel bacterial overgrowth following gastric bypass surgery. Oral thiamine supplementation does not reverse thiamine deficiency in this patient population. Concurrent treatment of small intestinal bacterial overgrowth with antibiotics is needed to adequately treat the condition.

Psychotic beriberi: Psychotic beriberi manifesting with auditory and visual hallucinations, odd and aggressive behavior has been recently described in gastric bypass patients.

Electrocardiogram

In some studies beriberi heart disease has been associated with a variety of ECG changes including sinus tachycardia , T wave inversion, low voltage, prolognation of the QT interval, and either prolongation or shortening of the PR interval . In many cases, these abnormalities revert with treatment of the thiamine deficiency.

Laboratory Studies

The diagnosis of beriberi is assisted by a dietary history suggestive of a low thiamine intake and clinical manifestations. However, objective biochemical tests of thiamine status, particularly measurement of erythrocyte transketolase activity (ETKA) and the thiamine pyrophosphate effect (TPPE), provide a sensitive test for thiamine deficiency.

Treatment

Treatment is with thiamine hydrochloride, either in tablet form or injection. A rapid and dramatic recovery within hours can be made when this is administered to patients with beriberi. Thiamine occurs naturally in unrefined cereals and fresh foods, particularly fresh meat, legumes, green vegetables, fruit, and milk.

Additional Resources

• Christiaan Eijkman bio at whonamedit.com: discovered cause of Beriberi.

• A protection against beriberi. British Medical Journal. 1980 January 19; 280(6208): 187.

• Medical Encyclopedia, Medline, National Institutes of Health.

• Health A-to-Z: Beriberi.

• L Arturo Batres, MD. Beriberi.EMedicine.com

• NAS: Dietary Reference Intakes for Thiamin, Riboflavin, Niacin, Vitamin B6, Folate, Vitamin B12, Pantothenic Acid, Biotin and Choline. Washington DC: National Academy Press; 1998.

• Angstadt JD, Bodziner RA: Peripheral polyneuropathy from thiamine deficiency following laparoscopic Roux-en-Y gastric bypass. Obes Surg 2005 Jun-Jul; 15(6): 890-2

• Beriberi, white rice, and vitamin B: a disease, a cause, and a cure Reviewed by Sanjoy Bhattacharya. Medical History. 2002 July; 46(3): 432–433.

• Toward the Conquest of Beriberi, Reviewed by C. A. Bozman. Medical History. 1962 April; 6(2): 198–199.

• K C Carter The germ theory, beriberi, and the deficiency theory of disease. Medical History. 1977 April; 21(2): 119–136.

• A K Clarke. Beriberi in Bethnal Green. British Medical Journal. 1971 May 1; 2(5756): 278.

• Curran JS, Lewis AB: Vitamin Deficiencies and Excesses. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. 2000: 179.

• Djoenaidi W, Notermans SL, Verbeek AL: Subclinical beriberi polyneuropathy in the low income group: an investigation with special tools on possible patients with suspected complaints. Eur J Clin Nutr 1996 Aug; 50(8):549-55

• J G Engbers, G P Molhoek, and A C Arntzenius. Shoshin beriberi: a rare diagnostic problem. British Heart Journal. 1984 May; 51(5): 581–582.

• Alan Hawk. The Great Disease Enemy, Kak'ke (Beriberi) and the Imperial Japanese Army, Military Medicine, Apr 2006 .

• Hirshfeld AB, Getachew A, Sessions J: Drug doses. In: Siberry GK, Iannone R eds. The Harriet Lane Handbook: A Manual for the Pediatrician. 2000: 864.

• Indraccolo U, Gentile G, Pomili G, et al: Thiamine deficiency and beriberi features in a patient with hyperemesis gravidarum. Nutrition 2005 Sep; 21(9):967-8

• Diagnosing Beriberi in Emergency Situations, by Prof Mike Golden, Aberdeen University. (n.d.)
• Eric R. Gubbay. Beri-Beri Heart Disease. Canadian Medical Association Journal. 1966 July 2; 95(1): 21–27.

• A Hardy Beriberi, vitamin B1 and world food policy, 1925-1970. Medical History. 1995 January; 39(1): 61–77.

• Neil McIntyre and Nigel N. Stanley. Cardiac Beriberi: Two Modes of Presentation. British Medical Journal. 1971 September 4; 3(5774): 567–569.

• Jew RK, Mascarenhas M, McCoy B et al, eds: The Children's Hospital of Philadelphia Pharmacy Handbook and Formulary 2000-2001. Lexi-Comp Inc; 2000: 342-3.

• Kitamura K, Yamaguchi T, Tanaka H, et al: TPN-induced fulminant beriberi: a report on our experience and a review of the literature. Surg Today 1996; 26(10): 769-76

• Mouly S, Khuong MA, Cabie A: Beri-Beri and thiamine deficiency in HIV infection [letter]. AIDS 1996 Jul; 10(8): 931-2

• NAS: Dietary Reference Intakes for Thiamin, Riboflavin, Niacin, Vitamin B6, Folate, Vitamin B12, Pantothenic Acid, Biotin and Choline. Washington DC, National Academy Press; 1998.

• Shivalkar B, Engelmann I, Carp L: Shoshin syndrome: two case reports representing opposite ends of the same disease spectrum. Acta Cardiol 1998; 53(4): 195-9

• Jeb Sprague and Eunida Alexandra. Haiti: Mysterious Prison Ailment Traced to U.S. Rice - Inter Press Service (IPS). 17 January 2007.

• Tanphaichitr V: Thiamin. In: Shils M, Olson JA, Shike M, eds. Modern Nutrition in Health and Disease. Vol 1. 8th ed. 1994: 359-365.

• Weise Prinzo Z, de Benoist B: Meeting the challenges of micronutrient deficiencies in emergency- affected populations. Proc Nutr Soc 2002 May; 61(2):251-7

• Wilson JD: Vitamin Deficiency and Excess. In: Wilson JD, et al. Harrison's Principles of Internal Medicine. 12th ed. 1991: 436-437.

• Wrenn KD, Murphy F, Slovis CM: A toxicity study of parenteral thiamine hydrochloride. Ann Emerg Med 1989 Aug; 18(8): 867-70

External links

• www.wrongdiagnosis.com : beriberi

See also

• Christiaan Eijkman
• Edward Bright Vedder

References

   *

     Tanphaichitr, V., Shils, M. Modern Nutrition in Health and Medicine, 9th Ed, Lippincott, Philadelphia 2000. p.381.
   *

     Carpenter, K.J. Beriberi, White Rice, and Vitamin B: A disease, a cause, and a cure, Berkeley, University of California Press, 2000.
   *

     Hardy, A. Beriberi, White Rice, and Vitamin B: A disease, a cause, and a cure N Engl J Med 343:588, August 24, 2000. Book review.
   *

     Gubler, CJ. Thiamin. In: Handbook of vitamins: Nutritional, biochemical, and clinical aspects, Machlin, LJ (Ed), Marcel Dekker, New York 1984. p.245.
   *

     Rindi, G. Thiamine. In: Present knowledge in nutrition, 7th edn. Washington, DC: International Life Sciences Institute, 1996: 160-6.
   *

     Thiamine deficiency and its prevention and control in major emergenciesWHO/NHD/99.13
   *

     Clements RH, Katasani VG, Palepu R, Leeth RR, Leath TD, Roy BP, Vickers SM. Incidence of vitamin deficiency after laparoscopic Roux-en-Y gastric bypass in a university hospital setting. Am Surg 2006; 72(12): 1196 1202.
   *

     S. Lakhani, H. Shah, K. Alexander, F. Finelli, J. Kirkpatrick, T. Koch. Small intestinal bacterial overgrowth and thiamine deficiency after Roux-en-Y gastric bypass surgery in obese patients. Nutrition Research, Volume 28, Issue 5, Pages 293-298
   *

     Wei Jiang, M.D., Jane P. Gagliardi, M.D., Y. Pritham Raj, M.D., Erin J. Silvertooth, M.D., Eric J. Christopher, M.D., and K. Ranga R. Krishnan, M.D. Acute Psychotic Disorder After Gastric Bypass Surgery: Differential Diagnosis and Treatment. Am J Psychiatry 163:15-19, January 2006.