A more rapid form of wet beriberi is termed acute fulminant cardiovascular beriberi, or shoshin beriberi. Shoshin beriberi is a state of cardiovascular collapse due to metabolic acidosis caused by vitamin b 1 thiamine deficiency. Science 2035455, 1979 shoshin beriberi cardiomyopathy and thiamine deficiency hemodynamic studies in severe circulatory failure with high cardiac output in two male patients with chronic ethanol abuse and carbohydratethiamine deficient diets were reported. A 36yearold female was admitted to the hospital because of disturbance of consciousness and edema in the lower extremities and face. A 10yearold male presented to clinic with three months of diarrhea, postprandial abdominal pain, fevers, and 20lb weight loss. Beriberi definition beriberi is a disease caused by a deficiency of thiamine vitamin b1 that affects many systems of the body, including the muscles, heart, nerves, and digestive system.
Dry and wet beriberi mimicking critical illness polyneuropathy. Two patients were seen within a few months in a general hospital and in both dietary deficiency of. Because of its gradual onset with multiple symptoms and complicated medical histories, the clinician can often miss the diagnosis. Jun 08, 2018 cold extremities, low bp, and kidney problems linked to shoshin beriberi. Cardiovascular beriberi presents as either the fulminant shoshin beriberi or chronic form. Cardiovascular magnetic resonance in wet beriberi pdf.
Case description we report a case of a 6yearold boy admitted to the emergency. Shoshin beriberi, a fulminant form of heart failure due to thiamine deficiency has a different presentation to the classical form of beriberi heart failure. The cardiovascular and metabolic features were attributable to shoshin beriberi. Patients become anuric and develop a severe lactic acidosis, often aggravated by conventional inotropic support attributed to adrenergic. The occurrence of highoutput heart failure in classical beriberi is due to excessive vasodilatation in the muscles and a fall in the peripheral vascular resistance with resultant increase in venous return and. Chronic cardiac beriberi in a former prisoner of the japanese volume 44 issue 3 g. Science 2035455, 1979 shoshin beribericardiomyopathy and thiamine deficiency hemodynamic studies in severe circulatory failure with high cardiac output in two male patients with chronic ethanol abuse and carbohydratethiamine deficient diets were. The thiaminedependent enzymes are important for the biosynthesis of neurotransmitters and for the production of reducing substances used in oxidant stress defenses, as well as for the synthesis of pentoses used as nucleic acid precursors. A 30 year old man with a 15 year history of alcohol abuse presented with symptoms and signs of circulatory shock, severe disturbances of renal and liver functions, and metabolic acidosis. Shoshin beriberi mimicking central line sepsis in a child. Clinical history and colliquative myocytolysis are keys to the diagnosis of shoshin beriberi.
It has previously been described as a rapidly curable hemodynamic disaster that, if not timely recognized and promptly treated, can result in rapid. Get a printable copy pdf file of the complete article. He recovered completely after treatment with thiamine. Mar 29, 2019 shoshin beriberi and severe accidental hypothermia as causes of heart failure in a 6yearold child. Shoshin beriberi and severe accidental hypothermia as causes.
One case died and the histological lesions corresponded to those of chronic congestive beri beri. One prior report in a similar context identified ten patients with tpninduced fulminant beriberi encountered over an 8year period 5. In acute wet beriberi or shoshin beriberi, the predominant injury is to the heart, and rapid deterioration occurs because of the hearts inability to maintain function. Shoshin beriberi shoshin beriberi a more rapid form of wet beriberi is termed acute fulminant cardiovascular beriberi, or shoshin beriberi. Thiamine vitamin b 1 was the first b vitamin to have been identified. Since children with malnutrition may have low or borderline thiamine reserves, they are prone to developing td during refeeding without thiamine supplementation 4.
It serves as a cofactor for several enzymes involved in energy metabolism. Pdf thiamine deficiency beriberi a forgotten disease. Differences in blood acetaldehyde concentrations in relatives of alcoholics and controls. Clinical history and colliquative myocytolysis are keys to. Mclaughlin, assistant director of health for attempts have been made, until the inquiry undertaken by the philippine islands, and dr.
International journal of cardiology and heart health. This phenotype affects the cardiovascular system and is divided into acute and chronic forms. Levin dl, morris fc, moore gc eds a practical guide to pediatric intensive care. Dry beriberi is a multifocal peripheral andor central neurologic dysfunction, which includes wernicke encephalopathy and korsakoff syndrome. Oct 04, 2018 beriberi has 2 main forms in humans, depending on the system of maximum involvement. Rapidly progressive and fulminant form with no edema shoshin beriberi in certain infants. Beriberi cardiovascular agudo shoshinberiberi request pdf. May 17, 2015 three of our four cases of shoshin beriberi encountered over an 18month period in critically ill patients without a history of alcoholism had the common feature of tpn use. Clinical correlates and outcome of shoshin beriberi saya rp. A woman, aged 33 years, enormously stout, with cyanosis of the face and hands, menstrual irregularities, and. Interestingly, imprisonment was also found to be related to thiamine deficiency.
The predominant injury is to the heart, and rapid deterioration follows the inability of. A patient with fulminant shoshintype beriberi was studied in the acute phase and found to have severe metabolic acidosis, high output biventricular failure, and markedly low systemic vascular resistance. Reversible pulmonary hypertension, lactic acidosis, and. Chronic cardiac beriberi in a former prisoner of the japanese. Thiamine deficiency beriberi a forgotten disease m hasan 1, h rahman 2, b h n yasmeen 3, a mukti 4, h haque 5, m khan 6, m begum 7, s roy 8, t u ahmed 9, au ahmed 10, s tabassum 11. Pdf stsegment elevation of electrocardiogram in a patient. Thiamine deficiency is a medical condition of low levels of thiamine vitamin b1. The cause of beriberi was established in 1897 by the dutch physician c. In the present report, we describe the case of a 66yearold man presenting with leg edema and dyspnea at rest. Wet edematous beriberi is a cardiovascular dysfunction that is usually chronic but may have an acute presentation. The substance in rice siftings which protects against beriberi was found to be vitamin b 1.
Patients become anuric and develop a severe lactic acidosis, often aggravated by. Shoshin beriberi is probably not an uncommon disease in the united states, but the few reports in the. Launois and cleret have published an interesting case in point. Red blood cell transketolase activity was abnormally low. Three of our four cases of shoshin beriberi encountered over an 18month period in critically ill patients without a history of alcoholism had the common feature of tpn use. Two cases of shoshin beri beri with hemodynamic and plasma. Shoshin beriberi definition of shoshin beriberi by medical. A more rapid form of wet beriberi is termed acute fulminant cardiovascular beriberi shoshin beriberi, or acute pernicious beriberi. Beriberi is a condition that occurs in people who are deficient in thiamine vitamin b1. The classical form of wet beriberi is a slowly developing, tenuous hyperdynamic state with peripheral edema that, if left untreated, may progress to fulminant shoshin beriberi with high output cardiac failure, severe lactic acidosis, peripheral circulatory collapse and shock.
For a diagnosis of wet beriberi, medical history is very important. Hegde medical academy, deralakatte, mangalore, karnataka, india 3 department of. Beriberi genetic and rare diseases information center gard. Laboratory testing was notable for markedly elevated inflammatory markers esr 96mmhr, crp 94 mgl, hypoalbuminemia 2. Beriberi genetic and rare diseases information center. It is characterized by a cold periphery, low blood pressure, renal shutdown and a severe metabolic acidosis. Shoshin beriberi induced by longterm administration of. Thiamin in hypertension hypertension remains the most potent and.
A patient with fulminant shoshin type beriberi was studied in the acute phase and found to have severe metabolic acidosis, high output biventricular failure, and markedly low systemic vascular resistance. Shoshin beriberi with low cardiac output and hemodynamic. Beriberi is a disease caused by a vitamin b1 deficiency, also known as thiamine deficiency. It is highly fatal and is known to cause sudden death in young migrant laborers in asia whose diet consists of white rice. Wet beriberi with multiple organ failure remarkably reversed. Like wet beriberi, it causes cardiac problems but of a different kind. Clinical correlates and outcome of shoshin beriberi saya.
We conclude that it is important to consider shoshin beriberi as part of the differential diagnosis in patients with shock and stsegment elevation. Shoshin beriberi mimicking central line sepsis in a child with short bowel syndrome. Eijkman caused beriberi in hens by feeding them cooked, hulled rice and then cured them with rice siftings. Full text full text is available as a scanned copy of the original print version. The evolution of hemodynamic parameters after treatment with thiamin is described. International journal of cardiology and heart health volume 1 issue 1, august 2017 derrick lonsdale 2017, the beriberi heart. Newonset heart failure cardiology jama cardiology jama. Beriberi may also be caused by shortcomings other than inadequate intake. Shoshin beriberi precipitated by intravenous glucose. Cardiac beriberi is considered a rare disease in western society. The patient had a history of heart failure with preserved ejection fraction and was receiving. Shoshin beriberi and severe accidental hypothermia as causes of heart failure in a 6yearold child.
People with wet beriberi may experience increased heart rate, shortness of breath, and swelling of the lower legs. Shoshin beriberi, a subtype of beriberi involving the cardiovascular system, is appropriately designated as a rapidly curable hemodynamic disaster. Herein, we present the case of a 61yearold man with shoshin beriberi, a fulminant form of wet beriberi, induced by longterm diuretic therapy. The classical form of wet beriberi is a slowly developing, tenuous hyperdynamic state with peripheral edema that, if left untreated, may progress to fulminant shoshin beriberi with high output cardiac failure, severe lactic acidosis, peripheral circulatory collapse and. Dry beriberi affects the nervous system resulting in numbness of the hands and. Postprandial hypertriglyceridemia is now established as. Beriberi is an ancient disease caused by lack of thiamine and a sugarrich diet that has been eradicated from most countries in the worldan almost forgotten disease of poor riceeaters in the tropics, which targets and destroys heart muscles, nerve fibers, and the brain. Shoshin beriberi article about shoshin beriberi by the.
This article presents a rare case of wet beriberi associated with multiple organ failure mof in a prison patient with years of heavy alcohol consumption. Thiamine deficiency beriberi induced polyneuropathy and. Extreme deficiency, meanwhile, causes slurred speech, odd eye movements, and gait. This condition involves a rapid appearance of symptoms and acute heart failure. For shoshin beriberi patients, after thiamine treatment, the hemodynamic indices blood pressure, pulse, urine volume, serum lactate, etc. The predominant injury is to the heart, and rapid deterioration follows the inability of the heart muscle to satisfy the bodys demands because of its own injury. Fulminant beriberi heart disease with lactic acidosis. Previous studies have suggested that diuretic therapy for heart failure may lead to thiamine deficiency due to the increased urinary thiamine excretion. Clinical correlates and outcome of shoshin beriberi rama prakasha saya 1, shashidhar baikunje 2, parampalli suryanarayana prakash 1, kodangala subramanyam 3, vikram patil 1 1 department of general medicine, k.
Shoshin beriberi and severe accidental hypothermia as. In this form, the predominant injury is to the heart. Shoshin beriberi is characterised by an acute cardiovascular presentation with cyanosis and hypotension being the predominant features, although it is usually accompanied by obtundation. Wet beriberi with multiple organ failure remarkably. Shoshin beriberi is a rare disease that may lead to a fatal outcome if the patient does not receive appropriate treatment. Shoshin beriberi, which is a fulminant form of cardiovascular beriberi accompanied by hemodynamic deterioration with high cardiac output and decreased systemic blood pressure, caused by thiamine deficiency due to alcoholic abuse or malnutrition, is often difficult to address because of its rarity and nonspecific symptoms.
Wet beriberi affects the cardiovascular system resulting in a fast heart rate, shortness of breath, and leg swelling. Another more rapid type of wet beriberi is classified as acute fulminant cardiovascular beriberi, or shoshin beriberi 14. Beriberi obviously is not a disappearing disease, but the pattern of incidence has changed. A deficiency of vitamin b1 or thiamine leads to pain and confusion from dry beriberi or breathing problems, rapid heartbeat, low bp and severe cardiac symptoms from wet beriberi. Shoshin beriberi or acute pernicious beriberi is another less common form of beriberi associated with vitamin b1 deficiency. A less common form of cardiovascular, or wet beriberi, is known as shoshin. Two fulminant forms of beri beri shoshin beri beri have been the subject of detailed study.
Hegde medical academy, deralakatte, mangalore, karnataka, india 2 department of nephrology, k. Andrews, to confirm the diagnosis professor of bacteriology and pathology in the philippine of infantile beriberi by postmortem examinations. Shoshin beriberi article about shoshin beriberi by the free. Le shoshin beriberi est une pathologie rare, a ne pas meconnaitre face au risque devolution fatale rapide. Shoshin beriberi definition of shoshin beriberi by. We report a case of a 58yearold man with shoshin beriberi who demonstrated stsegment elevation and myocardial damage without coronary artery stenosis. Nov 17, 2015 beriberi is a condition that occurs in people who are deficient in thiamine vitamin b1. Thiamine deficiency is a medical condition of low levels of thiamine vitamin b 1. A less common clinical presentation consist of a fulminating heart failure with evidence of a low cardiac output. One prior report in a similar context identified ten patients with tpninduced fulminant beriberi encountered over an 8. He was on no medications and had no history of travel or family history of gastrointestinal or autoimmune disorders. Signs and symptoms of vitamin b1 thiamine deficiency. The patient subsequently recovered with thiamine treatment. Beriberi literally means i cant, i cant in singhalese, which reflects the crippling effect it has on its victims.