Thiamin(e): the spark of life.

One of the earliest vitamins to be discovered and synthesized, thiamin was originally spelled with an "e". The terminal "e" was dropped when it was found that it was not an amine. It is still spelled with and without the "e" depending on the text. This chapter provides a brief historical review of the association of thiamin with the ancient scourge of beriberi. It emphasizes that beriberi is the model for high calorie malnutrition because of its occurrence in predominantly white rice consuming cultures. Some of the symptomatology of this ancient scourge is described, emphasizing the difference from that seen in starvation. High calorie malnutrition, due to excessive ingestion of simple carbohydrates, is widely encountered in the U.S.A. today. Thiamin deficiency is commonly associated with this, largely because of its cofactor status in the metabolism of glucose. The biochemistry of the three phosphorylated esters of thiamin and the transporters are discussed and the pathophysiology of thiamin deficiency reviewed. The role of thiamin, and particularly its synthetic derivatives as therapeutic agents, is not fully appreciated in Western civilization and a clinical section describes some of the unusual cases described in the scientific literature and some experienced by the author. The possible role of high calorie malnutrition and related thiamin deficiency in juvenile crime is hypothesized.

Comparison of beriberi cases in indigenous and non-indigenous people, Brazil, 2013 to 2018. / Comparação dos casos de beribéri entre indígenas e não indígenas, Brasil, 2013 a 2018.

Beriberi is the clinical manifestation of severe and prolonged thiamine (vitamin B1) deficiency. It is a neglected disease that affects low-income populations facing food and nutrition insecurity. The aim of this study was to compare cases of beriberi among indigenous and non-indigenous people in Brazil. We conducted a cross-sectional study using data on cases of beriberi during the period July 2013-September 2018 derived from beriberi notification forms available on the FormSUS platform. Cases in indigenous and non-indigenous patients were compared using the chi-squared test or Fisher's exact test, adopting a significance level of 0.05. A total of 414 cases of beriberi were reported in the country during the study period, 210 of which (50.7%) were among indigenous people. Alcohol consumption was reported by 58.1% of the indigenous patients and 71.6% of the non-indigenous patients (p = 0.004); 71.0% of the indigenous patients reported that they consumed caxiri, a traditional alcoholic drink. Daily physical exertion was reported by 76.1% of the indigenous patients and 40.2% of the non-indigenous patients (p < 0.001). It is concluded that beriberi disproportionately affects indigenous people and is associated with alcohol consumption and physical exertion.

O beribéri é a manifestação clínica da deficiência grave e prolongada de tiamina (vitamina B1). Doença negligenciada que acomete a população de baixa renda, em situação de insegurança alimentar e nutricional. O objetivo do estudo foi comparar casos de beribéri em indígenas com casos em não indígenas no Brasil. Trata-se de estudo transversal de casos notificados de beribéri no período de 2013 a 2018, no formulário do SUS (FormSUS) do Ministério da Saúde. Foram comparados os casos em indígenas e em não indígenas pelo teste qui-quadrado ou teste exato de Fisher com nível de significância de p < 0,05. No período estudado foram notificados no país 414 casos de beribéri, sendo 210 (50,7%) indígenas. Referiram consumo de bebidas alcoólicas 58,1% dos indígenas e 71,6% dos não-indígenas (p = 0,004); adicionalmente, 71,0% dos indígenas consumiam caxiri (bebida alcoólica tradicional fermentada). Relataram fazer esforço físico diário 76,1% dos indígenas e 40,2% dos não-indígenas (p <0 ,001). Conclui-se que o beribéri no país acomete mais indígenas e está relacionado ao consumo de álcool e ao esforço físico.

[Shoshin beriberi: new emergence of an old pathology? About with 11 cases on island Mayotte]. / Shoshin béribéri: réémergence d'une pathologie ancienne ? À propos d'une série de 11 cas à Mayotte.

In the French island of Mayotte in the Indian Ocean, the health and food situation remains contrasted. For a very long time dry beriberi women in postpartum had been suspected and treated. But in 2004 the first infantile epidemic of beriberi was scientifically authenticated and a program of thiaminic supplementation for the pregnant women and infants was set up. In this context, we describe an epidemic of 11 cases of shoshin beriberi among adults between January 2008 and Februar 2009. Over 11 cases of shoshin beriberi, 5 cases were confirmed biologically and 6 were probable. The sex ratio M/W was 0.37. The median age was 34 years. The clinic picture was typical: severe acute dyspnea, an agitation/drowsiness, right cardiac failure: polynevritis of the lower limbs was noted in 9 cases over 11. Biologically it was characterized by a lactic acidosis (average pH: 7.08, lactates: 12.08 mmol/l). The evolution was favorable in the 8 cases which could benefit from early thiaminic refill. The outbreak of an epidemic of shoshin beriberi among adults mainly in groups not having benefited from supplementation shows the effectiveness of the program but also its limits. We compare our series with others: the period from April to June when the food is less diversified, is confirmed as a higher risk period. The programs of nutritional education must be increased and a B1 vitamin supplementation for broader people during the rain season might be discussed.

[Beriberi, recurrent nutritional disease in a detention house in Côte-d'Ivoire]. / Le béribéri, maladie nutritionnelle récurrente en milieu carcéral en Côte-d'Ivoire.

As a re-emerging disease, beriberi caused by a lack of thiamine in food threatens more and more prisons in developing countries. Indeed in 2008, a beriberi epidemic occurred in a detention house of Côte-d'Ivoire called Maca. The goal of our retrospective investigation was to describe this epidemic in order to improve prisoners' health. The study related to 131 subjects, 64% of cases affected (N = 205). The total rate of beriberi attack was estimated at 38.6‰. The mean age was 33; all patients were male and they were detained in "Batiment C" (70.2%), synonymous with heavy punishment. They spent about 28.1 month in Maca. The clinical symptoms were neurological signs (swarming: 41%) and cardiovascular signs (dyspnoea: 42%, thoracic pain: 35%). Half of the patients (51%) presented oedemas of the lower limbs. The rate of healing was about 97% when patients followed treatment. Providing good nutrition to the prisoners and the distribution of vitamin complements will avoid other epidemics.

Beriberi in Brazil: A Disease That Affects Indigenous People.

BACKGROUND: Beriberi is the clinical manifestation of thiamine deficiency. It is multicausal and typically associated with poverty and food insecurity among vulnerable populations, such as indigenous people. OBJECTIVE: The objective of this study was to carry out a spatial analysis of reported cases of beriberi among indigenous people in Brazil. METHODS: Cross-sectional study using time series data on suspected cases of beriberi reported to the Ministry of Health via the FormSUS between July 2013 and September 2018. Indigenous villages were georeferenced, and Kernel density estimation was used to identify patterns of the spatial distribution of beriberi cases. RESULTS: A total of 414 cases of beriberi were reported in the country of which 210 (50.7%) were indigenous people. All the cases in indigenous people occurred in states located in the Legal Amazon (Maranhão, Roraima, and Tocantins). Kernel density estimation showed high-density areas in Tocantins and Roraima. CONCLUSIONS: This is the first nationwide study of reported cases of beriberi. The findings can be used to guide actions that contribute to the monitoring and prevention of beriberi among indigenous people.

The rediscovery of thiamine deficiency disorders at a secondary level mission hospital in Northeast India.

In this article, we report the different presentations of thiamine deficiency disorders seen at a remote rural mission hospital in Northeast India, including investigations, treatment, and recovery. Two case studies, one of an infant with cardiac beriberi and the other of a nonalcoholic adult presenting with peripheral neuropathy, cardiomyopathy, and metabolic acidosis and responding to thiamine supplementation, are described in detail. We share our experience with these clinical entities over the past two decades, including recent research and lessons learned, and suggest ways forward to identify at-risk populations in Northeast India, improve early diagnosis and treatment, and promote preventive public health strategies.

Pediatric thiamine deficiency disorders in high-income countries between 2000 and 2020: a clinical reappraisal.

Often thought to be a nutritional issue limited to low- and middle-income countries (LMICs), pediatric thiamine deficiency (PTD) is perceived as being eradicated or anecdotal in high-income countries (HICs). In HICs, classic beriberi cases in breastfed infants by thiamine-deficient mothers living in disadvantaged socioeconomic conditions are thought to be rare. This study aims to assess PTD in HICs in the 21st century. Literature searches were conducted to identify case reports of PTD observed in HICs and published between 2000 and 2020. The analyzed variables were age, country, underlying conditions, clinical manifestations of PTD, and response to thiamine supplementation. One hundred and ten articles were identified, totaling 389 PTD cases that were classified into four age groups: neonates, infants, children, and adolescents. Eleven categories of PTD-predisposing factors were identified, including genetic causes, lifestyle (diabetes, obesity, and excessive consumption of sweetened beverages), eating disorders, cancer, gastrointestinal disorders/surgeries, critical illness, and artificial nutrition. TD-associated hyperlactatemia and Wernicke encephalopathy were the most frequent clinical manifestations. The circumstances surrounding PTD in HICs differ from classic PTD observed in LMICs and this study delineates its mutiple predisposing factors. Further studies are required to estimate its magnitude. Awareness is of utmost importance in clinical practice.

A fresh look at thiamine deficiency-new analyses by the global thiamine alliance.

Severe thiamine (vitamin B1 ) deficiency is generally regarded as a problem affecting mostly infants in low-income communities of Southeast Asia and adult alcoholics regardless of their location. However, recent scholarship shows that the disorders associated with thiamine deficiency may also affect heretofore unsuspected populations, and that the scope of disorders, including some long-lasting neurocognitive consequences, is broader than previously thought.

Clinical studies on rising and re-rising neurological diseases in Japan--a personal contribution.

Throughout my research life, I experienced to discover the causes of some neurological diseases in Japan. 1) SMON (subacute myelo-optico-neuropathy). Since the early 1960s, a peculiar neurological disease became prevalent throughout Japan. Through the chemical analysis of the green urine, characteristic of this disease, it was found that this disease was caused by intoxication of the administered clioquinol, an anti-diarrheal drug. This discovery is a big topic in the history of Japanese medicine. 2) In early 1970s, I experienced many young patients with oedema and polyneuropathy in Kagoshima. Finally it was found that the disease was the long-forgotten beriberi, which had disappeared several decades ago. We must always be aware of beriberi even now, as far as we eat well-polished rice. 3) In 1972, we noticed a group of sporadic paraparesis in Kagoshima, which was 20 years later confirmed to be induced by human T lymphotropic virus type-I (HTLV-I). We named this disease as "HTLV-I associated myelopathy" (HAM). It gave a strong impact that the causative virus of adult T cell leukemia (ATL) can induce entirely different diseases, in terms of both the clinical course and the pathological features. It was also proven that HAM was identical with tropical spastic paraparesis, (TSP), which had been prevalent in many areas of tropical zones. These experiences are good examples of our slogan "to keep in mind to send message of scientific progress from the local area to the international stage.

Infantile Cardiac Beriberi in Rural North East India.

Twenty eight exclusively breastfed infants presented between 1 July, 2017 and 30 June, 2018 with acute heart failure syndrome, with 23 (92%) showing dramatic clinical resolution of shock within 24 hours of receiving intravenous thiamine (100 mg) bolus. Our findings raise awareness for addressing this neglected nutritional disease in North East India.

High prevalence of infantile encephalitic beriberi with overlapping features of Leigh's disease.

Infantile encephalitic beriberi (IEBB) is a rare form of thiamine deficiency and is poorly described. A proportion of Leigh's disease (LD) patients have similar clinical picture and response to thiamine as beriberi, leading to confusion in diagnosis and management. Data on IEBB and LD is scarce and status of thiamine deficiency in India is controversial. We report several infants with life-threatening respiratory and central nervous system symptoms that overlap between IEBB and LD. Majority had low erythrocyte transketolase levels and responded dramatically to thiamine supplementation suggesting a diagnosis of IEBB. However, presence of characteristic lesions on brain imaging and residual damage in several patients on follow-up does not rule out LD completely. Our study highlights the importance of thiamine deficiency in India, especially in the breast-feds and its overlapping features with LD. Awareness of this common mode of presentation may save patients' lives by early diagnosis and timely thiamine supplementation.

Beriberi Outbreak Among Unauthorised Immigrants in a Detention Camp in Malaysia.

In February 2012, a district health authority received three inmates with bilateral leg swelling and difficulty in breathing from a detention camp for unauthorized immigrants. A case control study was conducted. Fasting blood samples of case and control groups were collected according to instructions of the laboratory for determining thiamine level, and their 1 week dietary intake was analyzed. 9% (21/226) of inmates had bilateral leg swelling, and 75% (6/8) of them had low thiamine level (< 66 mmol/L). Their diet contained mainly polished rice and other items with low thiamine contents. Bilateral leg swelling was associated with history of no meat consumption during past 3 months (OR 8.4; CI 2.2-32.1). Patients were treated with 100 mg thiamine intravenously and 5 mg orally per day for 6 weeks. All patients responded well. The management was advised to provide vitamin B complex daily, and encouraged to provide thiamine rich foods.

Outbreak of beriberi in a prison in Côte d'Ivoire.

BACKGROUND: A beriberi outbreak occurred in the Maison d'Arrêt et de Correction d'Abidjan (MACA), a detention center in Abidjan, Côte d'Ivoire, between October 2002 and April 2003. OBJECTIVE: A retrospective investigation was conducted to document the outbreak in April 2003. METHODS: A descriptive analysis and a case-control study were performed. A probable case patient was defined as a person detained in the center between October 2002 and April 2003 with at least two of the following symptoms: bilateral leg edema, dyspnea, positive squat test, motor deficiencies, and paresthesia. A definite case patient was defined as a probable case patient who showed clinical improvement under thiamin treatment. RESULTS: Of 712 cases reported, 115 (16%) were probable and 597 (84%) were definite. The overall attack rate was 14.1%, and the case fatality rate was 1.0% (7/712). The highest attack rate was reported in the building housing prisoners with long-term sentences (16.9%). All patients were male, and the mean age was 28 years. During the period studied, the penal ration provided a fifth of the quantity of thiamin recommended by international standards. After adjustment for potential confounders, a history of cholera infection (adjusted odds ratio [OR(a)], 12.9; 95% confidence interval [CI], 2.9 to 54.1) and incarceration in the building for severe penalties (OR(a), 4.8; 95% CI, 1.3 to 18.5) were associated with the disease. CONCLUSIONS: Beriberi has been underreported among prisoners. Further attention should be given to its risk factors, especially a history of acute diarrhea. Systematic food supplementation with vitamins and micronutrients should be discussed when the penal ration does not provide the necessary nutrient intake recommended according to international standards.

Beriberi outbreak among commercial fishermen, Thailand 2005.

In April 2005, The Tahi Bureau of Epidemiology investigated a reported outbreak of beriberi among commercial fishermen in Maha Chai, a port city in the Gulf of Thailand. The objective of this study was to verify the diagnosis of beriberi in affected individuals, describe the possible outbreak, ascertain risk factors, and provide prevention and control measures. We interviewed ill persons and treating doctors, and reviewed medical records to conduct a descriptive study. A probable case was defined as a crewmember of Ship A with one of the following: leg edema scrotal edema or ascites, dyspnea, chest discomfort, chest pain, extremity numbness, or extremity weakness. Confirmed cases were those with clinical criteria and laboratory findings consistent with thiamine deficiency. The outbreak started in early March, 2005 and continued until March 31, 2005. Ship A had 28 crewmembers (four Thai, 24 Myanmar). Overall, there were 15 probable cases (attack rate 53.6%, with three confirmed and 12 probable cases). Only three were tested for Vitamin B1 deficiency. All cases were male, with a median age of 28 years (range 20-45). Fourteen of the 15 cases were Myanmar and one Thai. Due to limited resources, the crew ate only seafood and polished rice for almost two months prior to symptoms. Symptoms included edema (60%), chest discomfort (54%), and dyspnea (27%). Two persons died while on board the ship (case fatality 13%). The total time at sea for Ship A was 18 months, including a five-month delay in docking due to licensing problems.

[Heart failure due to non-infectious causes in developing countries: etiologic approach and therapeutic principles]. / Insuffisance cardiaque d'origine non infectieuse en zone tropicale: approche étiologique et principes thérapeutiques.

Cardiovascular disease is a major worldwide health problem with a growing impact in developing countries. Heart failure is the clinical manifestation of many advanced cardiac disorders. It can have numerous etiologies and the incidence of non-infectious causes is increasing with socio-economic development, thus illustrating the global nature of this epidemiologic transition. Several of the numerous non-infectious causes of heart failure involve cardiac diseases specific to tropical areas including dilated cardiomyopathy, endomyocardial fibrosis, and peripartum cardiomyopathy. Other widespread disorders are becoming more common as a result of the epidemiologic transition. Cardiovascular risk factors are changing particularly with regard to the incidence of coronary artery disease, ischemic cardiomyopathy, and hypertension-related complications. The purpose of this article is to provide an overview of non-infectious causes of heart failure in terms of frequency, onset, and therapeutic requirements. Symptomatic treatment of heart failure is same as in developing countries but is often delayed due to shortcomings in the care system.

A Large Outbreak of Thiamine Deficiency Among Illegal Gold Miners in French Guiana.

From September 2013 to July 2014, several gold miners working in the tropical forest consulted the Maripasoula Health Center in French Guiana for edema and findings consistent with right-sided cardiac failure. Of the 42 cases of beriberi that were diagnosed, one patient died. The laboratory and clinical investigation demonstrated vitamin B1 deficiency in most of the patients tested. Furthermore, 30 of 42 patients responded favorably to 500 mg of intravenous or intramuscular thiamine supplementation. In addition, dietary investigation showed insufficient thiamine intake in these patients. We concluded that patients had acquired beriberi because of diet restrictions, hard labor, and infectious diseases, notably malaria. In 2016, cases were still being reported. We recommend screening for compatible symptoms in gold miners, thiamine supplementation, and nutritional intervention.

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.

Prevalence of thiamine deficiency at a drug rehabilitation centre in Malaysia.

A possible outbreak of beriberi occurred at a drug detention and rehabilitation centre, Pusat Serenti Bukit Cabang, Perlis, Malaysia in February 2004. This outbreak was identified following the presentation of a large number of inmates at a health centre with signs and symptoms of ankle oedema and shortness of breath. Further investigations revealed the death of three inmates at the General Hospital with similar clinical presentations during the period from October 2003 till February 2004. A cross sectional comparative study was carried out at the rehabilitation centre to find out the prevalence of thiamine deficiency among the inmates both symptomatic and asymptomatic. A total of 154 inmates were examined (57 symptomatic and 97 asymptomatic cases). It was found that 74% from the sample study (114 cases) had thiamine deficiency (44 symptomatic and 70 asymptomatic). Further statistical analysis showed that ankle oedema is consistent with the diagnosis of thiamine deficiency but lack sensitivity (p < 0.05, sensitivity 24.6%, specificity 95%). This outbreak could have been triggered by poor diet intake of thiamine by the inmates coupled with possible intake of certain thiamine antagonists in their diet.

[Clinical appearance and scalable profile Thiamine deficiency in prison in Guinea: study of thirty-eight observations]. / Aspect clinique et profil évolutif de la carence en thiamine en milieu carcéral en Guinée : étude de trente-huit observations.

Cardiovascular and neurological manifestations associated with thiamine deficiency in Guinean prisons are common but not reported.We performed a prospective study of 38 cases related to vitamin B1 deficiency over a period of 4 years. In this population, the literature of traditional data gathered: frequency peak after thirty (92.6%) and clear representation male (sex ratio M/F: 18/1). The clinical symptomatology remains essentially dominated by sensorimotor polyneuropathy and pure sensory (52.2%), overall heart failure (31.5%) and to a lesser degree by Gayet Wernicke's encephalopathy (7.8%) and shoshin beriberi with severe evolution (5.2%). The study of nutritional status by body mass index (BMI) of the World Health Organization, by the criteria of Detsky and biological markers including albumin, shows that these patients are severely malnourished.