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.

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.

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.

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.

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.

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.

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.

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.

[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.

Beriberi (thiamine deficiency) and high infant mortality in northern Laos.

BACKGROUND: Infantile beriberi (thiamine deficiency) occurs mainly in infants breastfed by mothers with inadequate intake of thiamine, typically among vulnerable populations. We describe possible and probable cases of infantile thiamine deficiency in northern Laos. METHODOLOGY/PRINCIPAL FINDINGS: Three surveys were conducted in Luang Namtha Province. First, we performed a retrospective survey of all infants with a diagnosis of thiamine deficiency admitted to the 5 hospitals in the province (2007-2009). Second, we prospectively recorded all infants with cardiac failure at Luang Namtha Hospital. Third, we further investigated all mothers with infants (1-6 months) living in 22 villages of the thiamine deficiency patients' origin. We performed a cross-sectional survey of all mothers and infants using a pre-tested questionnaire, physical examination and squat test. Infant mortality was estimated by verbal autopsy. From March to June 2010, four suspected infants with thiamine deficiency were admitted to Luang Namtha Provincial hospital. All recovered after parenteral thiamine injection. Between 2007 and 2009, 54 infants with possible/probable thiamine deficiency were diagnosed with acute severe cardiac failure, 49 (90.2%) were cured after parenteral thiamine; three died (5.6%). In the 22 villages, of 468 live born infants, 50 (10.6%, 95% CI: 8.0-13.8) died during the first year. A peak of mortality (36 deaths) was reported between 1 and 3 months. Verbal autopsy suggested that 17 deaths (3.6%) were due to suspected infantile thiamine deficiency. Of 127 mothers, 60 (47.2%) reported edema and paresthesia as well as a positive squat test during pregnancy; 125 (98.4%) respected post-partum food avoidance and all ate polished rice. Of 127 infants, 2 (1.6%) had probable thiamine deficiency, and 8 (6.8%) possible thiamine deficiency. CONCLUSION: Thiamine deficiency may be a major cause of infant mortality among ethnic groups in northern Laos. Mothers' and children's symptoms are compatible with thiamine deficiency. The severity of this nutritional situation requires urgent attention in Laos.

Subacute myelo­optic neuropathy, beriberi, and HTLV­I­associated myelopathy: elucidation of some neurological diseases in Japan.

Personal experience of the discovery of the cause, pathophysiology, and treatment as well as prevention of subacute myelo­optic neuropathy, beriberi, and HTLV­I­associated myelopathy were described.

[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.

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.

Outbreak of beriberi among African Union troops in Mogadishu, Somalia.

CONTEXT AND OBJECTIVES: In July 2009, WHO and partners were notified of a large outbreak of unknown illness, including deaths, among African Union (AU) soldiers in Mogadishu. Illnesses were characterized by peripheral edema, dyspnea, palpitations, and fever. Our objectives were to determine the cause of the outbreak, and to design and recommend control strategies. DESIGN, SETTING, AND PARTICIPANTS: The illness was defined as acute onset of lower limb edema, with dyspnea, chest pain, palpitations, nausea, vomiting, abdominal pain, or headache. Investigations in Nairobi and Mogadishu included clinical, epidemiologic, environmental, and laboratory studies. A case-control study was performed to identify risk factors for illness. RESULTS: From April 26, 2009 to May 1, 2010, 241 AU soldiers had lower limb edema and at least one additional symptom; four patients died. At least 52 soldiers were airlifted to hospitals in Kenya and Uganda. Four of 31 hospitalized patients in Kenya had right-sided heart failure with pulmonary hypertension. Initial laboratory investigations did not reveal hematologic, metabolic, infectious or toxicological abnormalities. Illness was associated with exclusive consumption of food provided to troops (not eating locally acquired foods) and a high level of insecurity (e.g., being exposed to enemy fire on a daily basis). Because the syndrome was clinically compatible with wet beriberi, thiamine was administered to ill soldiers, resulting in rapid and dramatic resolution. Blood samples taken from 16 cases prior to treatment showed increased levels of erythrocyte transketolase activation coefficient, consistent with thiamine deficiency. With mass thiamine supplementation for healthy troops, the number of subsequent beriberi cases decreased with no further deaths reported. CONCLUSIONS: An outbreak of wet beriberi caused by thiamine deficiency due to restricted diet occurred among soldiers in a modern, well-equipped army. Vigilance to ensure adequate micronutrient intake must be a priority in populations completely dependent upon nutritional support from external sources.

[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.

A retrospective investigation of thiamin and energy intakes following an outbreak of beriberi in The Gambia.

In the early part of the rainy season in 1988, an outbreak of beriberi occurred in free-living adults in a relatively small area in the North Bank region of The Gambia. In 1995 we selected two compounds in a village called Chilla situated within the affected district to retrospectively examine dietary factors potentially contributing to the outbreak. There had previously been cases of beriberi in one compound (BBC) but not in the other (NBC). We measured energy and thiamin intakes for four days on six occasions during the year. We calculated energy and thiamin intakes of people living in the two compounds and foods were collected for thiamin analysis through the year. Thiamin:Energy ratios only met international recommendations in the immediate post­harvest season when energy and thiamin intakes were highest and then fell through the year. In the rainy season when food was short and labour was heaviest, energy intakes were lower in the NBC but thiamin:energy ratios were lower in BBC. Records of rainfall in 1988 collected near the village indicated that the amount in August was twice the average. We suggest the heavy rainfall may have increased farm workload and reduced income from outside-village work activity. The lower energy intakes in the NBC may have forced adults to rest thus sparing thiamin demands and delaying onset of beriberi. In contrast, the higher energy intake of adults in the BBC may have enabled them to continue working, thus increasing demands for thiamin and inducing the earlier onset of beriberi.

Outbreak of beriberi in an Indian population of the upper Amazon region, Roraima State, Brazil, 2008.

Edema, parasthesias, and paresis affected 10 residents of an Indian community in Roraima state; three died. Mining with mercury occurs locally; caxirí, a traditional alcoholic drink, is consumed daily. We conducted a 1:2 unmatched case-control study; a case was an Indian from Uiramutã county (population of 9,127) who presented ≥ 1 of lower extremity edema, paresthesias, paresis, or weakness. Controls were asymptomatic Indians randomly selected from the population. We identified 90 cases (prevalence of 1%) and 180 controls; all were enrolled. Among cases, 79% were male, and the median age was 31 years. Ethnicity was Macuxí, and 49% had income. Cases had lower extremity edema (85%), upper extremity paresthesias (84%), and lower extremity weakness and pain (78%). Risk factors were male sex (odds ratio [OR] = 6.8; P < 0.001), age 31-40 years (OR = 5.63; P < 0.001), and consumption of caxirí (OR = 2.7; P < 0.003). Mercury exposure was not a risk. Thiamine therapy produced complete rapid clinical recovery in all cases, confirming the diagnosis of beriberi. We recommend surveillance, thiamine supplementation, and nutritional intervention.

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.

Outbreak of beriberi in the state of Maranhão, Brazil: revisiting the mycotoxin aetiologic hypothesis.

Beriberi is caused by thiamine deficiency. Early 20th century epidemics in Japan were attributed to rice contaminated by citreoviridin mycotoxin. Our investigation of an outbreak of beriberi in Brazil showed an association of beriberi with the consumption of poor quality subsistence farming rice, although, unlike other investigators of this outbreak, we did not identify citreoviridin producing fungi in the implicated rice.