Descriptive spectrum of thiamine deficiency in pregnancy: A potentially preventable condition.

OBJECTIVE: Pregnancy, a nutritionally demanding situation in terms of macro- and micronutrient supply owing to heightened maternal, placental, and fetal needs, significantly affects thiamine reserves. Thiamine deficiency during pregnancy and the postpartum period, presenting with varied manifestations and outcomes, is a relatively common condition in our population. The study aimed to understand the various manifestations and outcomes of acute thiamine deficiency in pregnant and postpartum women, emphasizing the significance of early recognition and thiamine therapy to prevent serious complications during pregnancy and after childbirth. METHODS: This prospective study conducted in a tertiary care center in North India enrolled consecutive pregnant and postpartum women presenting with clinical features consistent with thiamine deficiency disorders, such as thiamine deficiency-related neuropathy, high-output heart failure, heart failure with reduced ejection fraction, Wernicke's encephalopathy, gastric beriberi, and thiamine-responsive acute pulmonary hypertension. In addition to capturing medical history including drug intake, dietary consumption, and comorbidities, women underwent brief relevant clinical examinations and laboratory assessments, including whole-blood thiamine levels. Response to intravenous thiamine supplementation was also monitored. RESULTS: Data of 31 women (12 pregnant, 19 postpartum) with a diagnosis of acute thiamine deficiency and a mean age of 28.88 ± 2.69 years were analyzed. The mean thiamine level was 1.28 ± 0.44 µg/dL with mean blood lactate of 3.46 ± 3.33. The most common presentation was gastric beriberi (n = 10), followed by paraparesis (n = 6), high-output heart failure (n = 6), acute pulmonary hypertension, heart failure with reduced ejection fraction (n = 3 each), and an acute confusional state (n = 2). All patients responded to thiamine challenge. CONCLUSION: In the context of borderline thiamine status, particularly in our population with endemic thiamine deficiency and heightened demand for thiamine during pregnancy and the peripartum period, the deficiency can have varied and serious manifestations of dry and wet beriberi. Early recognition of the clinical features and thiamine therapy can be life-saving. There is a need for validated clinical criteria owing to the non-availability of thiamine testing in resource-limited settings.

A Rare Case of Thiamine Deficiency Leading to Dry Beriberi, Peripheral Neuropathy, and Torsades De Pointes.

Thiamine (vitamin B1) is an essential nutrient and one of the eight B vitamins. As a water-soluble vitamin, thiamine is not stored; therefore, a balanced diet is required to ensure adequate intake of this essential vitamin. Thiamine deficiency is known to cause both wet and dry beriberi, but rarely in combination. Thiamine deficiency has also been known to cause QTc prolongation, but the mechanism remains unclear. In the most severe cases, this can lead to the lethal arrhythmia of torsades de pointes. This case describes a patient who became malnourished after a closed head injury and initially presented with seizure-like activity and syncopal episodes with nonspecific numbness. He was found to have prolonged QTc, leading to torsades de pointes requiring an implanted cardioverter defibrillator. With extensive workup, including genetic testing, the patient was found to have indetectable thiamine levels. With supplementation, the patient no longer had any recorded ventricular arrhythmias, and neurological function improved with only residual tingling in the hands. This case emphasizes the profound effects of thiamine deficiency and why this should be included in our differential diagnosis for patients presenting with the sequelae of the signs and symptoms discussed.

Pulmonary artery catheter usage in diagnosis of Shoshin beriberi presented with unexplained lactic acidosis.

Wet beriberi is a rare but fatal disease in modern society. The nonspecific clinical manifestations, including symptoms of heart failure and recalcitrant lactic acidosis, can prevent timely diagnosis. The use of a pulmonary artery catheter can promptly confirm a high cardiac output state and plays a crucial role in rapidly deteriorating cases. Appropriate treatment with intravenous administration of thiamine leads to dramatic recovery within hours. We present two cases of Shoshin beriberi, a fulminant variant of wet beriberi, diagnosed in 2016 and 2022 at our institute. The patients experienced haemodynamic collapse and refractory lactic acidosis, which were successfully diagnosed with the use of a pulmonary artery catheter and reversed by thiamine supplementation. We also reviewed 19 cases of wet beriberi reported between 2010 and 2022.

Protocol and application of basal erythrocyte transketolase activity to improve assessment of thiamine status.

Thiamine (vitamin B1) is an essential micronutrient required as a cofactor in many metabolic processes. Clinical symptoms of thiamine deficiency are poorly defined, hence biomarkers of thiamine status are important. The erythrocyte transketolase activity coefficient (ETKac) is a sensitive measure of thiamine status, but its interpretation may be confounded where the availability of the transketolase enzyme is limited. Basal ETK activity per gram of hemoglobin provides a complementary biomarker of thiamine status; however, its measurement and calculation are poorly described. Here, we describe in detail the assessment of basal ETK activity, including the calculation of path length in microplates and the molar absorption coefficient of NADH specific to the assay, and the measurement of hemoglobin in sample hemolysates. To illustrate the application of the methods, we present ETKac and basal ETK activity from women in The Gambia and UK. In conclusion, we present a clear protocol for the measurement of basal ETK activity that will permit the harmonization of methods to improve replication between laboratories.

Raise vigilance against refractory distributive shock due to severe wet beriberi.

Differentiating the type and cause of shock is crucial for intensive care. The rapid aggravation of lactic acidosis in patients often indicates a severe impairment of oxygen uptake in tissues. Herein, we presented a rare case of refractory distributive shock with severe wet beriberi. A 40-year-old male was admitted to the emergency department (ED) with recurrent chest tightness and lower extremity edema. The condition of the patient continued to deteriorate after symptomatic treatments. After several turnovers, the medical history of the patient was requested again and finally obtained. Our emergency management team hypothesized that the thiamine-deficient diet caused an aerobic metabolism disorder in the patient. Overall, we aimed to alert clinicians to unusual causes of distributive shock and further discussed the application of thiamine supplementary therapy in critical care.

Shoshin beriberi and thiamine-responsive right heart failure: A case report in Mayotte Recognition and management of infant Shoshin beriberi.

Infant Shoshin beriberi is an acute life-threatening condition for which the diagnosis is frequently delayed. Therefore, rapid recognition of right heart failure with lactic acidemia is a crucial element in the diagnosis and therapeutic management. We present the case of a 2-month-old girl with bronchiolitis, right heart failure, and lactic acidosis, who quickly and favorably responded to thiamine supplementation. Thiamine deficiency was established through laboratory tests. We present a brief review of the literature with the different thiamine dosages proposed in emergencies and provide an emergency protocol in cases of clinical suspicion, since thiamine supplementation could help to speed up recovery in infants with Shoshin beriberi.

An Unusual Case of Wernicke's Encephalopathy in a Child.

Wernicke's encephalopathy (WE) is a manifestation of thiamine deficiency. The majority of affected patients are alcoholics and are adults. Often, clinicians fail to recognize that WE can also be found in non-alcoholic patients at risk for thiamine deficiency. Sometimes patients may not present with all the classic features, or the individual clinical signs may be treated as single problems and not a constellation of signs that form a diagnosis of WE. We present a unique case of a four-year-old male with a past medical history of food aversion who presented with intractable vomiting and weakness. The patient's clinical features showed signs of severe dehydration and weight loss. His clinical state subsequently progressed to having ophthalmoplegia and gait ataxia. Brain MRI demonstrated mamillary body changes, and serum thiamine level was significantly below the normal limit. Based on the patient's clinical assessment, deficient serum thiamine, and MRI findings, WE was diagnosed. The patient was evaluated by Pediatric Neurology and started on treatment with high dose IV thiamine. He showed an excellent response to thiamine treatment and had a significant resolution in his symptoms before discharge.

Dry beriberi after sleeve gastrectomy: An undiagnosed case report.

INTRODUCTION AND IMPORTANCE: Sleeve gastrectomy (SG) is a popular surgery for morbid obesity because of minimal complications, while somewhere uncommon micronutrient deficiencies occur that make problems. One of these rare complications is dry beriberi (BB). CASE PRESENTATION: A 20-year-old girl with obesity that a few months after SG had non-specific symptoms like nausea, vomiting, debilitating weakness, burning, and tingling in her feet, which led to more tests and imaging and confused the physicians. During the next two months, she had a 40 kg weight loss. The critical test that helped diagnosis was Electromyography and Nerve Conduction Velocity (EMG/NCV), which showed subacute axonal sensory-motor polyneuropathy and decreased level of vitamin B1, proved the patient's diagnosis was dray BB. CLINICAL DISCUSSION: SG may contribute to vitamin and trace elements deficiency development. One of the micronutrients that deficiencies can disturb the patient is vitamin B1 deficiency. Vitamin B1 deficiencies could be demonstrated with peripheral polyneuropathy, beriberi, or Wernicke-Korsakoff syndrome. Based on decreased vitamin B1 and EMG/NCV results, the diagnosis had been dry beriberi corrected with supplement therapy. CONCLUSION: Thiamine deficiency should be suspected in all patients with vomiting, neurological symptoms, and rapid weight loss post-bariatric surgery (BS), even after restrictive surgery. Especially when a patient reports substantial weight loss and vomiting in short order. Hopefully, this case report will make any patient hospitalized with similar conditions evaluated for beriberi and again multivitamin supplementation therapy after SG is emphasized.

Beri-Beri and Wernicke Encephalopathy in a Thirty-Year-Old Male.

A 30-year-old Turkish male was found lethargic and surrounded by vomit. At the hospital, severe hypernatremic dehydration and acute kidney failure were evident. His conscious level improved with fluid resuscitation. A differential diagnosis of altered mental status was considered. A complete clinical triad of Wernicke encephalopathy (WE), supported by MRI findings, was compatible with thiamine deficiency. Previous bariatric surgery was later confirmed. Despite no clinical signs of heart failure, a high level of NT-proBNP (N-terminal prohormone brain natriuretic peptide) and a dilated, hypokinetic myocardiopathy detected on the echocardiogram led us to assume beri-beri heart disease. High-dose intravenous thiamine, ACE (angiotensin conversing enzyme) inhibitors, beta-blockers, and physical therapy were initiated with remarkable improvement in his clinical condition.

[Wernicke-Korsakoff syndrome and other diseases associated with thyamine deficiency]. / Síndrome de Wernicke-Korsakoff y otras patologías asociadas al déficit de tiamina.

Wernicke-Korsakoff syndrome is the best known consequence of thiamine deficiency, frequently associated with patients with chronic and excessive alcohol consumption, but it can be produced by any cause that produces thiamine deficiency. The disease is underdiagnosed so it is essential to have a high clinical suspicion, mainly in patients who do not have alcohol consumption as a risk factor. For this, the diagnosis continues to be eminently clinical, with the difficulty of high clinical variability. Complementary tests are used to support the diagnosis and rule out other causes that can produce similar symptoms, with magnetic resonance imaging being the most cost-effective imaging test. Treatment is based on the administration of thiamine, which should be started early, and parenterally at the appropriate doses, in all patients with compatible symptoms, without waiting to confirm the diagnosis.

Síntese de evidências para políticas de saúde: enfrentando o beribéri em terras indígenas / Synthesis of evidence for health policies: tackling beriberi in indigenous lands

O beribéri é uma doença com diferentes subtipos, causada pela deficiência de tiamina (vitamina B1), que, apesar de fácil tratamento, pode levar ao óbito (WORLD HEALTH ORGANIZATION, 1999). No Brasil, os casos mais recentes ocorreram a partir de 2006, ano em que se somaram 40 óbitos notificados no estado do Maranhão (PADILHA et al., 2011). Nos anos subsequentes, novos casos foram notificados nos estados de Tocantins e Roraima (BRASIL, 2018; CERRONI et al., 2010). Tendo em vista que, historicamente, no Brasil, essa deficiência está relacionada a condições de miséria (FRUTUOSO, 2010), observa-se que grande parte dos surtos atuais se conecta a condições de pobreza e fome, além da relação com a alimentação monótona, baseada em arroz polido e no consumo elevado de carboidratos simples. Alguns grupos populacionais têm maior risco de serem acometidos pela deficiência, tais como: alcoolistas, gestantes, crianças e pessoas que exercem atividade física extenuante (BRASIL, 2012). O agravo entre os povos indígenas, representando 84,5% dos casos confirmados notificados, no Brasil, entre 2014 e 2016, é de importante preocupação devido tanto às vulnerabilidades específicas dessa população quanto aos desafios de identificação e tratamento oportunos da doença (ALVES, 2017). Opções para enfrentar o problema: Opção 1 ­ Suplementação de tiamina (vitamina B1): Esta opção envolve tanto a suplementação oral de tiamina e de outras vitaminas B (DAY et al., 2013; HUTCHEON, 2015; JAIN et al., 2015; JULIAN et al., 2019; KOH et al., 2015; STROH; MEYER; MANGER, 2014), incluindo tanto a administração parenteral, em casos mais graves de desnutrição (HUTCHEON, 2015), quanto a administração intramuscular ou intravenosa de tiamina pelas equipes de saúde, em casos de suspeita de sintomas de beribéri (HUTCHEON, 2015). Devido à dificuldade de confirmação do diagnóstico de beribéri, adotar suplementação oral de tiamina, de modo a garantir as doses necessárias da vitamina entre populações mais vulneráveis à insegurança alimentar, pode prevenir a doença, bem como diversas outras condições relacionadas à insuficiência de vitamina B1. Opção 2 ­ Intervenções junto a pessoas que fazem uso prejudicial de álcool: Esta opção descreve intervenções voltadas à melhoria das condições nutricionais e à prevenção de doenças como o beribéri e neuropatias junto a populações vulneráveis para o uso de álcool. Foram identificadas intervenções de educação e orientação, que englobaram desde entrevistas motivacionais pelo telefone até sessões educativas de um ou mais dias, intervenções multicomponentes (IJAZ et al., 2018) e intervenções de abstinência do álcool (JULIAN et al., 2018). As intervenções de abstinência foram efetivas para melhorar parcialmente os sintomas sensoriais, todavia não reverteram o quadro por completo (JULIAN et al., 2019). Considerações gerais acerca das opções propostas: As opções de intervenção sugeridas nesta síntese, apesar de estarem descritas de modo separado, podem ser complementares entre si e exigem uma ação intersetorial e integrada localmente. A implementação das opções deve considerar a participação de diferentes atores, como tomadores de decisão, profissionais da saúde pública e representantes dos povos indígenas. Além disso, devem ser avaliados os diferentes contextos, incluindo as diferentes responsabilidades entre as esferas de governo (federal, estadual e municipal), as áreas de conhecimento específicas e os limites técnicos dos diferentes profissionais envolvidos. Outro ponto importante a ser analisado é a disponibilidade de recursos humanos e financeiros, adaptando as intervenções às diferentes realidades, bem como verificando a possível provisão dos dispositivos necessários. Em determinadas situações, a ampliação e a capacitação da equipe serão imprescindíveis para o sucesso da adesão às opções propostas.

Beriberi is a disease with different subtypes, caused by thiamine (vitamin B1) deficiency, which, despite easy treatment, can lead to death (WORLD HEALTH ORGANIZATION, 1999). In Brazil, the most recent cases occurred from 2006, when 40 deaths were reported in the state of Maranhão (PADILHA et al., 2011). In subsequent years, new cases were reported in the states of Tocantins and Roraima (BRASIL, 2018; CERRONI et al., 2010). Given that, historically in Brazil, this deficiency is related to conditions of misery (FRUTUOSO, 2010), it is observed that much of the current outbreaks connect to conditions of poverty and hunger, in addition to the relationship with monotonous diet, based on polished rice and high consumption of simple carbohydrates. Some population groups have a higher risk of being affected by the deficiency, such as alcoholics, pregnant women, children and people who exercise strenuous physical activity (BRASIL, 2012). The aggravation among indigenous peoples, representing 84.5% of the confirmed cases notified, in Brazil, between 2014 and 2016, is of important concern due to both the specific vulnerabilities of this population and the challenges of timely identification and treatment of the disease (ALVES, 2017). Options to address the problem: Option 1 - Thiamine (vitamin B1) supplementation: This option involves both oral supplementation of thiamine and other B vitamins (DAY et al., 2013; HUTCHEON, 2015; JAIN et al., 2015; JULIAN et al., 2019; KOH et al, 2015; STROH; MEYER; MANGER, 2014), including both parenteral administration in more severe cases of malnutrition (HUTCHEON, 2015) and intramuscular or intravenous administration of thiamine by health care teams in cases of suspected beriberi symptoms (HUTCHEON, 2015). Due to the difficulty in confirming the diagnosis of beriberi, adopting oral thiamine supplementation to ensure the necessary doses of the vitamin among populations most vulnerable to food insecurity may prevent the disease as well as several other conditions related to vitamin B1 insufficiency. Option 2 - Interventions for people who are harmful users of alcohol: This option describes interventions aimed at improving nutritional status and preventing diseases such as beriberi and neuropathies among populations vulnerable to alcohol use. Education and guidance interventions were identified, ranging from motivational interviewing over the phone to one- or multi-day educational sessions, multicomponent interventions (IJAZ et al., 2018), and alcohol abstinence interventions (JULIAN et al., 2018). The abstinence interventions were effective in partially improving sensory symptoms, but did not completely reverse the condition (JULIAN et al., 2019). General considerations about the proposed options: The intervention options suggested in this synthesis, although described separately, may be complementary to each other and require intersectoral and locally integrated action. The implementation of the options should consider the participation of different stakeholders such as decision-makers, public health professionals and indigenous peoples' representatives. In addition, the different contexts must be evaluated, including the different responsibilities between the spheres of government (federal, state, and municipal), the specific areas of knowledge, and the technical limits of the different professionals involved. Another important point to be analyzed is the availability of human and financial resources, adapting the interventions to the different realities, as well as verifying the possible provision of the necessary devices. In certain situations, the expansion and training of the team will be essential for the successful adherence to the proposed options.

El beriberi es una enfermedad con diferentes subtipos, causada por la carencia de tiamina (vitamina B1), que, a pesar de su fácil tratamiento, puede conducir a la muerte (ORGANIZACIÓN MUNDIAL DE LA SALUD, 1999). En Brasil, los casos más recientes ocurrieron a partir de 2006, cuando se registraron 40 muertes en el estado de Maranhão (PADILHA et al., 2011). En años posteriores, se notificaron nuevos casos en los estados de Tocantins y Roraima (BRASIL, 2018; CERRONI et al., 2010). Dado que, históricamente en Brasil, esta deficiencia se relaciona con condiciones de miseria (FRUTUOSO, 2010), se observa que gran parte de los brotes actuales se conectan a condiciones de pobreza y hambre, además de la relación con dieta monótona, basada en arroz pulido y alto consumo de carbohidratos simples. Algunos grupos de la población tienen mayor riesgo de ser afectados por la deficiencia, como los alcohólicos, las embarazadas, los niños y las personas que realizan actividades físicas extenuantes (BRASIL, 2012). El agravio entre los pueblos indígenas, que representan el 84,5% de los casos confirmados notificados, en Brasil, entre 2014 y 2016, es de importante preocupación debido tanto a las vulnerabilidades específicas de esta población como a los desafíos de identificación y tratamiento oportunos de la enfermedad (ALVES, 2017). Opciones para abordar el problema: Opción 1 - Suplementos de tiamina (vitamina B1): Esta opción implica tanto la suplementación oral de tiamina como de otras vitaminas del grupo B (DAY et al., 2013; HUTCHEON, 2015; JAIN et al., 2015; JULIAN et al., 2019; KOH et al, 2015; STROH; MEYER; MANGER, 2014), incluyendo tanto la administración parenteral en casos más graves de malnutrición (HUTCHEON, 2015) como la administración intramuscular o intravenosa de tiamina por parte de los equipos sanitarios en casos de sospecha de síntomas de beriberi (HUTCHEON, 2015). Dada la dificultad de confirmar el diagnóstico de beriberi, la administración de suplementos orales de tiamina para garantizar las dosis necesarias de la vitamina entre las poblaciones más vulnerables a la inseguridad alimentaria puede prevenir la enfermedad, así como varias otras afecciones relacionadas con la insuficiencia de vitamina B1. Opción 2 - Intervenciones para las personas que consumen alcohol de forma nociva: Esta opción describe las intervenciones destinadas a mejorar el estado nutricional y prevenir enfermedades como el beriberi y las neuropatías entre las poblaciones vulnerables al consumo de alcohol. Se identificaron intervenciones de educación y orientación, desde entrevistas motivacionales por teléfono hasta sesiones educativas de uno o varios días, intervenciones multicomponente (IJAZ et al., 2018) e intervenciones de abstinencia de alcohol (JULIAN et al., 2018). Las intervenciones de abstinencia fueron eficaces para mejorar parcialmente los síntomas sensoriales, pero no revirtieron completamente la afección (JULIAN et al., 2019). Consideraciones generales sobre las opciones propuestas: Las opciones de intervención sugeridas en esta síntesis, aunque se describen por separado, pueden ser complementarias entre sí y requieren una actuación intersectorial y localmente integrada. La aplicación de las opciones debe tener en cuenta la participación de las distintas partes interesadas, como los responsables de la toma de decisiones, los profesionales de la salud pública y los representantes de los pueblos indígenas. Además, deben evaluarse los distintos contextos, incluidas las diferentes responsabilidades entre las esferas gubernamentales (federal, estatal y municipal), las áreas específicas de conocimiento y los límites técnicos de los distintos profesionales implicados. Otro punto importante a analizar es la disponibilidad de recursos humanos y financieros, adaptando las intervenciones a las diferentes realidades, así como verificando la posible dotación de los dispositivos necesarios. En determinadas situaciones, la ampliación y la formación del equipo serán esenciales para el éxito de las opciones propuestas.

A BERIBERI UNHEALTHY LATTE: ENCEPHALOPATHY AND SHOCK FROM SEVERE NUTRITIONAL DEFICIENCY.

BACKGROUND: Thiamine deficiency is an uncommon cause of severe illness in the United States that can lead to significant morbidity because of high-output cardiac failure, peripheral neuropathy, and permanent neurologic impairment. We report the case of a middle-aged woman with extreme malnutrition caused by complications of Roux-en-Y gastric bypass (RYGB) surgery who presented with signs and symptoms of severe thiamine deficiency and septic shock. CASE REPORT: A 43-year-old woman who had undergone RYGB surgery and who had multiple complications presented to the emergency department with agitation, confusion, and lethargy. The physical examination revealed an obtunded woman appearing much older than her reported age with significant peripheral edema. She was hypoxemic, hypotensive, and febrile. The initial laboratory analysis revealed a serum lactate level above the measurable limit, a normal thyroid-stimulating hormone, and elevated levels of troponin and brain natriuretic peptide. A transthoracic echocardiogram showed high-output heart failure. The patient's family later revealed that for the past year her diet had consisted almost exclusively of frozen blended lattes. High doses of thiamine and folate were started. Her shock, hyperlactatemia, and respiratory failure resolved by hospital day 3 and her encephalopathy resolved soon thereafter. Why Should an Emergency Physician be Aware of This?: Thiamine deficiency is a rare but reversible cause of shock, heart failure, and encephalopathy. Identifying patients who are at risk for severe nutritional deficiencies may aid in more rapid treatment with relatively benign medications with little downside, in this case high-dose vitamin B1, and ultimately improve patient-oriented outcomes such as mortality, morbidity, and hospital length of stay.

Assessment of salt intake to consider salt as a fortification vehicle for thiamine in Cambodia.

Thiamine deficiency is a public health issue in Cambodia. Thiamine fortification of salt has been proposed; however, the salt intake of lactating women, the target population, is currently unknown. We estimated salt intakes among lactating women (<6 months postpartum) using three methods: repeat observed-weighed intake records and 24-h urinary sodium excretions (n = 104), and household salt disappearance (n = 331). Usual salt intake was estimated by adjusting for intraindividual intakes using the National Cancer Institute method, and a thiamine salt fortification scenario was modeled using a modified estimated average requirement (EAR) cut-point method. Unadjusted salt intake from observed intakes was 9.3 (8.3-10.3) g/day, which was not different from estimated salt intake from urinary sodium excretions, 9.0 (8.4-9.7) g/day (P = 0.3). Estimated salt use from household salt disappearance was 11.3 (10.7-11.9) g/person/day. Usual (adjusted) salt intake from all sources was 7.7 (7.4-8.0) g/day. Assuming no stability losses, a modeled fortification dose of 275 mg thiamine/kg salt could increase thiamine intakes from fortified salt to 2.1 (2.0-2.2) mg/day, with even low salt consumers reaching the EAR of 1.2 mg/day from fortified salt alone. These findings, in conjunction with future sensory and stability research, can inform a potential salt fortification program in Cambodia.

Modeling thiamine fortification: a case study from Kuria atoll, Republic of Kiribati.

In 2014, there was an outbreak of beriberi on Kuria, a remote atoll in Kiribati, a small Pacific Island nation. A thiamine-poor diet consisting mainly of rice, sugar, and small amounts of fortified flour was likely to blame. We aimed to design a food fortification strategy to improve thiamine intakes in Kuria. We surveyed all 104 households on Kuria with a pregnant woman or a child 0-59 months. Repeat 24-h dietary recalls were collected from 90 men, 17 pregnant, 44 lactating, and 41 other women of reproductive age. The prevalence of inadequate thiamine intakes was >30% in all groups. Dietary modeling predicted that rice or sugar fortified at a rate of 0.3 and 1.4 mg per 100 g, respectively, would reduce the prevalence of inadequate thiamine intakes to <2.5% in all groups. Fortification is challenging because Kiribati imports food from several countries, depending on price and availability. One exception is flour, which is imported from Fiji. Although resulting in less coverage than rice or sugar, fortifying wheat flour with an additional 3.7 mg per 100 g would reduce the prevalence of inadequacy to under 10%. Kiribati is small and has limited resources; thus, a regional approach to thiamine fortification is needed.

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.

Pathophysiology, prevention, and treatment of beriberi after gastric surgery.

Beriberi is a nutritional complication of gastric surgery, caused by deficiency of vitamin B1, or thiamine. Thiamine deficiency leads to impaired glucose metabolism, decreased delivery of oxygen by red blood cells, cardiac dysfunction, failure of neurotransmission, and neuronal death. This review describes the history and pathophysiology of beriberi as well as the relationship between beriberi and nutritional deficiencies after gastric surgery. A literature review of the history and pathophysiology of beriberi and the risk factors for thiamine deficiency, particularly after gastric resection or bariatric surgery, was performed. Recommendations for nutritional follow-up post gastric surgery are based on current national guidelines. Patients may have subclinical thiamine deficiency after upper gastrointestinal surgery, and thus beriberi may be precipitated by acute illness such as sepsis or poor dietary intake. This may occur very soon or many years after gastrectomy or bariatric surgery, even in apparently well-nourished patients. Prompt recognition and administration of supplemental thiamine can decrease morbidity and mortality in patients with beriberi. Dietary education post surgery and long-term follow-up to determine nutritional status, including vitamin and mineral assessment, is recommended for patients who undergo gastric surgery.

Thiamine Deficiency in a Nondrinker and Secondary Pulmonary Edema after Thiamine Replenishment.

A 48-year-old man was brought to our emergency room with acute abdominal pain and systemic edema, indicating acute circulatory failure with lactic acidosis. Furosemide treatment paradoxically worsened the systemic edema and induced confusion. He had no drinking history but hardly ate legumes or meats containing thiamine. Administration of fursultiamine dramatically improved the symptoms and subsequently caused pulmonary edema. Thiamine deficiency may occur in nondrinkers with an unbalanced diet. In this condition, diuretic therapy can worsen the symptoms before thiamine supplementation by promoting the flushing of water-soluble vitamins but is needed for the management of secondary pulmonary edema after thiamine replenishment.

Acute Shoshin beriberi syndrome immediately post-kidney transplant with rapid recovery after thiamine administration.

Pediatric kidney transplant surgery is usually well tolerated, despite suboptimal physical conditioning that may result from uremia and nutritional deficiencies that accompany end-stage kidney failure. Nutritional supplementation is used to overcome such deficiencies, especially for children needing dialysis. Thiamine, a water-soluble vitamin also known as vitamin B1, is a critical cofactor in energy metabolism and may be competitively inhibited by the antimetabolite oxythiamine, a uremic toxin that accumulates in kidney failure. We report a case of a thiamine deficiency syndrome leading to overwhelming cardiac dysfunction, metabolic instability, and hemodynamic compromise, after otherwise uneventful kidney transplant surgery. Prior to transplant, this 14-year-old boy was treated with peritoneal dialysis and received thiamine supplementation. Post-transplant, the patient first developed hyperglycemia, then lactic acidosis, and subsequently hemodynamic instability despite escalating treatment with volume resuscitation and inotropic medication. He made a rapid and complete recovery after administration of IV thiamine. This is the first reported case of Shoshin beriberi syndrome in a pediatric kidney transplant recipient. Inadequate dialysis may have been a key factor, with toxin accumulation and thiamine transporter downregulation contributing to his status. Functional thiamine deficiency should be considered as a potential treatable cause of early post-transplant hemodynamic instability.

Thiamin deficiency in low- and middle-income countries: Disorders, prevalences, previous interventions and current recommendations.

BACKGROUND: Thiamin deficiency is a major public health concern in several low- and middle-income countries (LMICs)-current attention to the problem is lacking. AIM: This review discusses prevalence of thiamin insufficiency and thiamin-deficiency disorders (TDDs) in LMICs, outlines programmatic experience with thiamin interventions, and offers recommendations to improve public-health and research attention to thiamin in LMICs. DISCUSSION: Thiamin insufficiency, i.e. low-blood-thiamin status, is endemic among several Southeast Asian countries: Cambodia (70-100% of infants and 27-100% of reproductive-age women); Laos (13% of hospitalized infants); Thailand (16-25% of children and 30% of elderly adults). Thiamin deficiency accounts for up to 45% of under-5 deaths in Cambodia, 34% of infant deaths in Laos, and 17% of infant deaths in Myanmar. Deficiency also exists in Africa, Asia, and the Americas, but these instances have typically been isolated. Exclusively breastfed infants of thiamin-deficient mothers are at highest risk for TDD and related death. Intervention strategies that have been employed to combat thiamin deficiency include food processing, fortification, supplementation, dietary diversification, and dietary behaviors, all of which have shown varying levels of effectiveness. CONCLUSIONS: We recommend universal thiamin-fortification of context-specific staple-foods in LMICs as a promising solution, as well as thiamin supplementation, particularly for pregnant and lactating women. Food processing regulations, dietary diversification, and modification of dietary behaviors to increase consumption of thiamin-rich foods may provide benefits in some circumstances, especially in countries without universal fortification programs or in populations dependent on food aid.

Role of Thiamin in Health and Disease.

Thiamin is a hydrosoluble vitamin that plays a role in several biological processes, mainly in glucose metabolism. There are several risk factors for developing thiamin deficiency, such as malnutrition, refeeding syndrome, gastrointestinal surgery, and alcoholism. Recently, the role of thiamin in critically ill patients has gained prominence, and the prevalence of thiamin deficiency was found to be increased in patients with severe burns, major surgery, septic shock, end-stage renal disease, and heart failure. In adults, thiamin deficiency presents as encephalopathy, dry beriberi (with neurological signs and symptoms), or wet beriberi (with cardiovascular signs and symptoms). Thiamin deficiency can be diagnosed clinically, and all clinicians should be aware of this disease, especially in patients with risk factors for thiamin deficiency. Thiamin supplementation should be started as early as possible in patients suspected to have thiamin deficiency. Treatment is safe, inexpensive, simple, and life-saving. Diagnosis is confirmed on a positive response to treatment.