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[Nina Rodrigues, epidemiologist: historical study of beriberi outbreaks in a mental asylum in Bahia, Brasil (1897-1904)].

Beriberi outbreaks in the São João de Deus Asylum, 1897-1904, are presented, focusing on studies by legist physician Nina Rodrigues. The goals were: to trace the steps of the original investigation of an unknown disease; to understand the purpose of excluding mentally ills in an asylum institution. Methodology encompassed occurence narration and qualitative analytical procedures for the documental interpretation of voluntary and involuntary testimonies, combined with statistical treatment of measurable data. Frequent and progressive outbreaks of beriberi have caused two-thirds of the asylum deaths in 1904. The research method included domiciliary visits and interviews with dwellers in the asylum surroundings. Beriberi occurence was frequent among the insane and practically inexistent among the asylum staff, who presented close and long lasting contact with the ills. Nina Rodrigues refuted the declining miasmatic conception which emphasized external conditions, the emergent conception of contagion, and raised the hypothesis of a carential etiology for beriberi. Nina Rodrigues' study is a good historical example of "epidemiological reasoning," with question formulation, building of data bulk guided by working hypothesis, obtained by means of simple and efficient techniques.

Beriberi↗

[An elderly patient with beriberi].

An 82-year-old man was admitted to the hospital in the summer of 1995 due to dyspnea, peripheral edema, and a tingling sensation and muscle weakness in all extremities. Physical examination showed heart failure and polyneuropathy. Laboratory data showed a low concentration of vitamin B1 (10 ng/ml normal 23.8-45.9 ng/ml) and a low level of erythrocyte transketolase activity (0.58 IU/gHb normal 0.75-1.30 IU/gHb). A chest X-ray film revealed cardiomegaly (cardiothoracic ratio 57.1%) and ultrasonic cardiography revealed increased motion of the left ventricle. A diagnosis of beriberi was made. The disease in this case may have been caused by hard work in the summer heat. The patient was treated with thiamine and his symptoms and signs resolved within a few weeks. A search of the literature revealed no previous report of beriberi in a patient of this age, but reports of beriberi in older patients in Japan are more common now than in previous years. Beriberi should be included in the differential diagnosis of polyneuropathy in elderly patients.

Age of Onset↗

[Right heart failure caused by thiamine deficiency (cardiac beriberi)].

BACKGROUND: Vitamin B1 deficiency (beriberi) is very uncommon in France. It leads to high output cardiac failure totally different from the situation observed in alcoholic patients. We report a typical case of cardiac beriberi. CASE REPORT: The patient was referred for dyspnea with high output cardiac failure. Echocardiography evidenced severe pulmonary hypertension and high cardiac output. The more common causes of heart failure were ruled out. The dietary habits of the patient (suggested beriberi which was confirmed by the low serum thiamin and therapeutic test with vitamin B1. DISCUSSION: High output cardiac failure should suggest possible Shoshin beriberi, particularly in subjects with imported dietary habits living in a precarious socioeconomic situation.

Beriberi↗

[A man with the combination of dry and wet beriberi].

A 34-year-old alcoholic man had neurological and cardiac symptoms. The patient was admitted to the hospital for acute painful sensory disturbances and severe weakness of the feet. Neurological and electrophysiological investigation revealed axonal sensorimotor polyneuropathy that was most prominent in the legs. Cardiac assessment showed signs and symptoms of heart failure due to a high-output state. Blood analysis showed a low thiamine concentration of 58 nmol/l (lower reference limit: 80). Therefore, a diagnosis of combined wet beriberi with cardiomyopathy and dry beriberi with axonal polyneuropathy was made. The treatment of beriberi is simple and effective and consists of thiamine supplementation in conjunction with diuretic treatment. With this approach, the patient recovered fully. Patients with beriberi have a good prognosis, particularly when the diagnosis is made at an early stage.

Adult↗

Clinical diagnosis of cardiac beriberi.

The clinical features of 28 patients with biochemically proven cardiac beriberi were prospectively studied. Patients recruited over a 2-year-period fell into two groups: (i) those with signs of hyperdynamic circulation; and (ii) those presenting with heart failure and circulatory shock with or without metabolic acidosis. All patients showed a dramatic response to intravenous thiamine; in group 1 the hyperdynamic state resolved within hours and in group 2 there was an immediate rise in the blood pressure with gradual resolution of the acidosis in 6-8 hours. Two patients in whom the diagnosis was missed died soon after admission to hospital. A notable finding was that even in the presence of peripheral circulatory shock, patients with beriberi had preservation of the femoral pulses often with 'pistol shots' and femoral bruit; this finding has clinical value in the diagnosis of acute cardiovascular beriberi (shoshin beriberi).

Acidosis, Respiratory↗

Subclinical beriberi polyneuropathy in the low income group: an investigation with special tools on possible patients with suspected complaints.

OBJECTIVE: To determine the prevalence rate of subclinical beriberi polyneuropathy (PNP) in the low income group and to present new methods for early detection. DESIGN: We conducted a prospective, randomized observational study on all patients from family members of non-PNP patients presenting to the outpatient neurologic clinic of the Dr Soetomo Hospital in 1989 in Surabaya, Indonesia. MATERIAL AND METHODS: The 53 group I patients belonged to the low income series, whereas the 56 group II patients were from the mid- and high-income groups. We analyzed their diets and determined their blood thiamine levels. Neurologic, internal, psychiatric as well as neurophysiologic examinations were performed on all patients. RESULTS: The prevalence rate of subclinical beriberi PNP of the apparently healthy subjects of the low income group was 66% compared with 12.5% among the mid and high income groups. The exposure odds ratio was 13.6 (95% Cl, 2.78 to 8.04) indicating that the low income group ran a greater risk of developing beriberi PNP than the mid and high income groups. CONCLUSION: Most of our low income group patients had an inadequate diet, especially concerning deficient thiamine intake. Analysis of the diets revealed, that they were usually rich in carbohydrate especially steamed milled rice, whereas intake of fat and thiamine was low, protein was just sufficient. The calorie intake was marginal. Carbohydrate rich and non fat calories in the diet with low thiamine intake may provoke beriberi.

Adolescent↗

[The history of healing; beriberi: 'kind of paralysis'].

Beriberi began to attract increasing attention in the second half of the 19th century, mostly due to the large numbers of victims among Dutch military personnel in the Netherlands East Indies. A study, carried out in Atjeh in 1886 by the Japanese Sugenoya and F. J. Cornelissen, led to the conclusion that there existed a beriberi bacillus which they could make visible in stained microscopical preparations. An extensive disinfection campaign in all barracks followed, but its benefits were a matter of debate. Inadequate nutrition in the investigators' eyes was only a predisposing factor. A.G. Vorderman (1844-1902) assumed a connection between rice feeding and beriberi in prisoners; he reported that the disease coincided with a diet of polished white rice. Beriberi could be prevented by providing the prisoners with 'red rice' (unpolished rice). However, red rice was regarded as inferior and status-lowering so that substitution of red for white rice in the rations was impossible. The concept of a 'lacking substance' (thiamine, vitamin B1) was hesitatingly accepted in the Netherlands. C. Winkler and C. Eijkman, for instance, refused until the early twenties of this century to exclude the possibility of a bacterial infection as the cause. As known, Eijkman in 1929 was awarded the Nobel prize for his contribution to vitamin research.

Animals↗

Reappearance of beriberi heart disease in Japan. A study of 23 cases.

Twenty-three Japanese patients with beriberi heart disease, 17 of them teenagers, were studied. The recent tendency for teenagers to take excessive sweet carbonated soft drinks, instant noodles and powermill-polished rice readily induces relative thiamine deficiency. A sudden increase in thiamine requirements due to strenuous exercise can result in overt beriberi heart disease. Alcohol had nothing to do with the development of the disease. Characteristic features commonly seen in teenage patients include peripheral edema, low peripheral vascular resistance, increased venous pressure enlarged heart, T wave abnormalities, hyperkinetic circulatory state and increased circulating blood volume. Thiamine deficiency was confirmed by a decrease in blood thiamine concentration, a decrease in erythrocyte transketolase activity and an increase in thiamine pyrophosphate (TPP) effect. Improvement was rapidly achieved with thiamine administration, balanced nutrition and rest, especially in the teenage patients. Increased circulating blood volume was useful in differentiating beriberi heart disease from hyperthyroidism.

Adolescent↗

Beriberi neuropathy. Morphometric study of sural nerve.

Seven biopsied sural nerves from patients with beriberi were morphometrically evaluated. In teased fiber analysis the mean frequency of myelinated fibers showing axonal degeneration and segmental demyelination was 37.5 and 5.3%, respectively. In two cases with frequency of segmental demyelination higher than 5%, segmental demyelination was shown by statistical criteria to have occurred on certain fibers in a clustered fashion. Therefore, the segmental demyelination in beriberi may be secondary to axonal degeneration. Electromyographic findings and slow improvement of muscle weakness were compatible with axonal degeneration of motor fibers. Determinations of fiber densities revealed preferential decrease of the density of large myelinated fibers with the preservation of the density of small myelinated and unmyelinated fibers. The preferential nerve fiber involvement in beriberi was not associated with pain in the lower limbs and this fact is contrary to the expectation of the proponents of the gate control theory.

Adolescent↗

The dominant diseases of modernized societies as omega-3 essential fatty acid deficiency syndrome: substrate beriberi.

About 1900, modern food selection and processing caused widespread epidemics of the B vitamin deficiency diseases of beriberi and pellagra which, for genetic reasons, often expressed as different diseases ranging from bowel and heart disease to dermatoses and psychoses. But the B vitamins merely help convert essential fatty acids (EFA) into the prostaglandin (PG) tissue regulators and it now turns out that, through hydrogenation, milling and selection of w3-poor southern foods, we have also been systematically depleting, by as much as 90%, a newly discovered trace Nordic EFA (w3) of special importance to primates and sole precursor of the PG3(4) series, even as a concurrent fiber deficiency increases body demand for EFA. Since substrate EFA is processed by many B vitamin catalysts, an EFA deficiency will mimic a panhypovitaminosis B, i.e., a mixture of substrate beriberi and substrate pellagra resembling vitamin beriberi and pellagra but exhibiting as even more diverse endemic disease. This would constitute a second stage of the Modern Malnutrition and explain why some workers now hold the dominant diseases of modernized societies to be new, nutritionally based, pellagraform yet lipid-related and to range, once again, from heart disease to psychosis. It is an assumption that our dominant diseases are unrelated to each other or are merely revealed by out diagnostic acumen and therapeutic success; and that hydrogenating millions of tons of food oils annually, to destroy the rancidity producing w30EFA, is safe for primates. Extensive beriberiform disease is reported here in 32 typical cases taken from medical practice which responds strikingly to linseed oil supplements (60% w3-EFA) in confirmation of identical results in Capuchins.

Adult↗

Cardiac beriberi: two modes of presentation.

Two patients suffering from cardiovascular beriberi presented with different clinical manifestations. One had the classical features of a high cardiac output with raised jugular venous pressure and gross oedema. The other was in fulminating heart failure with clinical evidence of a low cardiac output but no peripheral oedema. The latter type of beriberi (shoshin) is rare. Cardiovascular beriberi has a high mortality when untreated. Both patients responded dramatically to thiamine, and this emphasizes the importance of considering thiamine deficiency as a cause of heart failure even when the cardiac output is low.

Adult↗

Gene-environment interactions in wet beriberi: effects of thiamine depletion in CD36-defect rats.

Selective vulnerability to thiamine deficiency is known to occur between individuals and within different tissues. However, no comprehensive explanation for this has been found, and there are no reports that reproduce the cardiovascular manifestations of human wet beriberi in animals. We hypothesized that the distinction of substrate reliance, namely, the primary dependency on glucose as substrate, could be an underlying factor in the selective vulnerability of thiamine deficiency. In the setting of impaired fatty acid entry, which occurs in CD36-defect rats, substrate reliance shifts from fatty acid to glucose, which would be expected to lead to a susceptibility to thiamine deficiency. Genomic DNA was analyzed for CD36 defects in three cognate strains of rats [spontaneously hypertensive rats (SHR)/NCrj, SHR/Izm, and Wistar-Kyoto (WKY)/NCrj], which identified the presence of a CD36 defect in SHR/NCrj rats but not in SHR/Izm and WKY/NCrj rats. Treatment with 2 wk of thiamine-depleted chow on 4-wk-old rats of each of these strains resulted in increased body and lung weight in the SHR/NCrj rats but not in the SHR/Izm and WKY/NCrj rats. The increased lung weight in the SHR/NCrj rats was accompanied with histological changes of congestive vasculopathy, which were not observed in either the SHR/Izm or the WKY/NCrj rats. Thiamine-deficient 12-wk-old SHR/NCrj rats demonstrated increased body weight (305.6 +/- 6.2 g in thiamine-deficient rats vs. 280.8 +/- 9.1 g in control; P < 0.0001), lactic acidemia (pH, 7.322 +/- 0.026 in thiamine-deficient rats vs. 7.443 +/- 0.016 in control; P < 0.0001; lactate, 2.42 +/- 0.28 mM in thiamine-deficient rats vs. 1.20 +/- 0.11 mM in control; P < 0.0001) and reduced systemic vascular resistance (4.61 +/- 0.42 x 104 dyn.s.cm-5 in thiamine-deficient rats vs. 6.55 +/- 1.36 x 104 dyn.s.cm-5 in control; P < 0.0001) with high cardiac output (186.0 +/- 24.7 ml in thiamine-deficient rats vs. 135.4 +/- 27.2 ml in control; P < 0.0019). In conclusion, SHR/NCrj rats harboring a genetic defect of long-chain fatty acid uptake present the relevant clinical cardiovascular signs of human wet beriberi, strongly indicating a close gene-environment interaction in wet beriberi.

Animal Feed↗

Dietary and socio-economic factors associated with beriberi in breastfed Lao infants.

A case-control study was conducted to investigate the dietary and socio-economic factors associated with beriberi in infants attending three public hospitals in Vientiane, Lao PDR. Forty-three breast-feeding infants with a median (range) age of 3 (1-9) months were admitted with beriberi. This was defined as the presence of signs of congestive heart failure or shock in the absence of fever or other signs of sepsis, hypovolaemia or cardiac abnormalities, with rapid clinical improvement following parenteral thiamine. Subjects were matched by age and diet to 43 breast-feeding healthy control infants. Compared with control mothers, mothers of infants with beriberi had significantly less diet diversity (p <0.001), soaked glutinous rice for significantly longer or were more likely to pour off excess water from non-glutinous rice (p =0.006), had fewer years of schooling (p <0.05), were more likely to report that income was inadequate for basic needs (p <0.001), to perform hard physical labour (p <0.01) and to be married to farmers (p <0.01). Clinically significant thiamine deficiency in breastfed infants in Lao relates to methods of preparing rice, the food selected by lactating mothers and the family's socio-economic status.

Adult↗

The great disease enemy, Kak'ke (beriberi) and the Imperial Japanese Army.

Although Japanese military officials had discovered that an improved diet could prevent beriberi by the late 19th century, their soldiers in the army suffered from beriberi during the Russo-Japanese War and World War II. A change in diet at the end of the Russo-Japanese War solved the problem and the army applied the lesson learned, along with postwar scientific discoveries about nutrition, toward the diet used during World War II. However, beriberi again plagued Japanese soldiers, this time due to poor logistics and unpalatable dietary supplements.

Beriberi↗

[Shoshin beriberi provoked by the inhalation of salbutamol].

A 45-year-old male alcoholic with a deficient diet was given salbutamol for exertion-related dyspnoea. After inhalation, he presented with a severe dyspnoea, acrocyanosis, anuria and low blood pressure as well as a respiratory compensated lactate acidosis. Shoshin beriberi was suspected on clinical grounds. The low level of thiamine and the prompt recovery after thiamine repletion confirmed this diagnosis. Shoshin beriberi is an acute, cardiac form of beriberi, which can rapidly result in death due to cardiogenic shock and lactate acidosis. Adrenergic agents can cause a hyperdynamic circulation and thus aggravate the effects of a thiamine deficiency.

Acidosis, Lactic↗

Beriberi heart disease in Durban. A retrospective study.

A review of 41 possible cases of beriberi diagnosed in a 4-year period (1981-1985) revealed that the diagnosis was fairly certain in 23 patients, although measurements of red cell transketolase had not been performed. Fifteen patients had classic (high-output) beriberi and 8 acute beriberi (shoshin type). This article calls for an increased awareness of the condition so that appropriate and timely therapy may be instituted.

Acidosis↗

The haemodynamic, histopathological and hormonal features of alcoholic cardiac beriberi.

Five cases of cardiac beriberi occurring in chronic alcoholics are described. The clinical diagnosis was based on the presence of biventricular failure, low dietary intake of thiamine and the therapeutic response to oral thiamine. Complicating cardiac disease was excluded by haemodynamic studies, left ventriculography; coronary angiography and endomyocardial biopsy. Haemodynamic measurements including quantitative left ventriculography are reported. They indicate that left ventricular function is depressed despite elevated cardiac output. Biopsy material was studied by light and electron microscopy. No lesion specific to beriberi was detected by either technique although the biopsies were quantitatively abnormal. The histological changes resemble those in early reports based on necropsy material, and consist of vacuolation and intercellular oedema in the early stages with myofibre hypertrophy, fibrosis and cellular infiltration in the chronic cases. The transketolase test and response to intravenous thiamine during catheter studies are valuable diagnostic tests. Plasma renin, angiotensin II and aldosterone levels were lower than in patients with low output heart failure. The incidence of cardiac beriberi appears to be greater than is generally realized.

Adult↗

Experimentally induced beriberi polyneuropathy in chickens.

Chickens fed with the same composition of diet as our low income beriberi polyneuropathic patients, developed clinical symptoms of thiamine deficiency in 22.3 +/- 6.3 days. There appeared to be a body store of thiamine which is utilized during a period of deficient intake. Haemoglobin content and serum albumin did not change appreciably during thiamine deficiency. The blood thiamine content was low and the thiamine pyrophosphate (TPP) effect increased to more than 25 percent during the development of the beriberi polyneuropathy, which resumed after one week on thiamine tetrahydrofurfuryl disulfide (TTFD) treatment. However, the clinical features gradually improved after about one month. Neurophysiological findings including somatosensory evoked potentials (SSEPs) and neuromorphological studies of the peroneal and sciatic nerves were compatible with a major degree of axonal degeneration and secondary minimal segmental demyelination. We may conclude that the experimentally induced beriberi polyneuropathy in chickens seems a good model for studying these forms of neuropathy in view of diagnosis and treatment.

Animals↗