Subclinical thiamine deficiency results in failed reproduction in Arctic foxes.

Thiamine deficiency can result in life-threatening physiological and neurological complications. While a thiamine-deficient diet may result in the onset of such symptoms, the presence of thiaminase - an enzyme that breaks down thiamine - is very often the cause. In such instances, thiaminase counteracts the bioavailability and uptake of thiamine, even when food-thiamine levels are adequate. Here, we report on a case of failed reproduction in seven Arctic fox (Vulpes lagopus) breeding pairs kept at a captive breeding facility, including the presentation of severe thiamine deficiency symptoms in two male foxes. Symptoms included ataxia, obtundation, truncal sway, star-gazing and visual impairment. Blood tests were inconclusive, yet symptoms resolved following treatment with a series of thiamine hydrochloride injections, thereby verifying the diagnosis. A fish-dominated feed, which for the first time had been frozen for a prolonged period, was identified as the likely source of thiaminase and subsequent deterioration in the animals' health. Symptoms in the two males arose during the annual mating period. All seven breeding pairs at the captive breeding station failed to reproduce - a phenomenon never recorded during the captive breeding facility's preceding 17-year operation. Relating our findings to peer-reviewed literature, the second part of this case report assesses how thiamine deficiency (due to thiaminase activity) likely resulted in subclinical effects that impaired the production of reproduction hormones, and thereby led to a complete breeding failure. While previous work has highlighted the potentially lethal effects of thiamine deficiency in farmed foxes, this is, to our knowledge the first study showing how subclinical effects in both males and females may inhibit reproduction in foxes in general, but specifically Arctic foxes. The findings from our case report are not only relevant for captive breeding facilities, but for the welfare and management of captive carnivorous animals in general.

Atypical Wernicke's encephalopathy without mental status changes following bariatric surgery in an adolescent patient.

Morbid obesity is a systemic disease which can result in chronic complications, including hypertension, diabetes mellitus, depression, osteoarthritis and low self-esteem in the adolescent population.Bariatric surgery can be indicated to treat more severe forms of obesity, but these procedures are not without long-term risks. Therefore, adequate preoperative and postoperative care, which includes preoperative psychosocial evaluation for compliance, ongoing nutrition counselling and vitamin and micronutrient supplementation, is required for all patients, especially adolescent patients, who generally may not comply with medical therapies and/or be able to developmentally fully appreciate or comprehend the health consequences of their behaviours, prior to as well as after bariatric surgery to prevent complications.Thiamine pyrophosphate, an active form of thiamine (also known as vitamin B1, a water-soluble vitamin), which functions as a coenzyme in glucose and energy metabolism, is one such vitamin that requires supplementation postoperatively. It is mandatory for glucose to be administered concomitantly with thiamine, as glucose alone can precipitate Wernicke's encephalopathy (WE) in thiamine-deficient individuals. WE is a medical emergency, with a mortality rate of up to 20%. WE is best understood as a classic triad of mental confusion, gait ataxia and eye movement abnormalities, and atypical WE or Wernicke's syndrome (WS) is seen when the classic triad is not present. Cases that meet some, but do not necessarily meet all three criteria, are referred to as atypical WE or WS which can lead to delayed diagnosis. Atypical WE has an incidence of 19% which can lead to misdiagnosis of a preventable medical emergency with fatal complications.The following case reviews the consequences of post-bariatric thiamine supplementation therapy non-adherence and resulting in a deficiency in an adolescent patient.

Pre- and Post-surgical Prevalence of Thiamine Deficiency in Patients Undergoing Bariatric Surgery: a Systematic Review and Meta-analysis.

Thiamine deficiency is a life-threatening nutritional abnormality observed in the patients with obesity and following bariatric surgery. The aim of the present study is to determine the prevalence of thiamine deficiency prior to and after bariatric procedures. PubMed, Web of Science, Google scholar, CENTRAL, ProQuest, and Scopus were searched to retrieve relevant studies containing data on thiamine deficiency in patients with obesity who underwent bariatric surgery. A proportional meta-analysis approach was used to pool the prevalence of thiamine deficiency prior and after surgery. Our comprehensive literature search retrieved 41 studies with relevant data. The pooled prevalence of thiamine deficiency was 7% (95% CI: 4-12%) at baseline. We observed that 19% (95% CI: 0-68%), 9% (95% CI: 3-17%), and 6% (95% CI: 3-9%) of patients had developed thiamine deficiency at 3 months, 6 months, and 1 year after surgery, respectively. We also report that the prevalence of thiamine deficiency in pregnant women who had history of bariatric surgery. The rate was highest in the first trimester (12%) compared to that in the second (8%) and third (10%) trimesters. The baseline prevalence is 7% for thiamine deficiency in bariatric surgery candidates. The prevalence rate of thiamin deficiency increased to 19% and 9% 3 and 6 months after surgery; however, the rate decreased to 6% 1 year after surgery. Due to the higher prevalence of thiamine deficiency in the early post-operative phase, close monitoring during this period is recommended. A similar strategy should be implemented for pregnant women with history of bariatric surgery in their first trimester.

Infrequent but serious? Beriberi And Thiamine deficiency among adolescents and young adults after bariatric surgery.

BACKGROUND: Thiamine deficiency (TD) among adolescents following metabolic and bariatric surgery (MBS) has not been assessed. OBJECTIVE: We assessed TD among adolescents following MBS. SETTING: University Hospital. METHODS: A retrospective chart review was conducted for all adolescents and young adults (aged 10-25 years) who had MBS and subsequently presented with TD at our institution (n = 30). Diagnosis used clinical, laboratory, brain imaging, and neurophysiology criteria. Of 1575 patients, 7 subsequently had TD. Another 23 adolescents had MBS at private hospitals or overseas and presented at our institution with TD. RESULTS: Based on MBS undertaken at our institution, TD prevalence was .45 cases per 100 MBS. The mean age of patients was 19.5 ± 3.23 years, 53.3% were male, 96.7% had sleeve gastrectomy, and time from MBS to admission averaged 4.97 ± 11.94 months. Mean weight loss from surgery to admission was 33.68 ± 10.90 kg. Associated factors included poor oral intake (90%), nausea and vomiting (80%), and noncompliance with multivitamins (71%). Signs and symptoms included generalized weakness, nystagmus, numbness, and paraparesis (83.3%-80%). Seven patients had Wernicke encephalopathy full triad; 16 displayed a mixed picture of Wernicke encephalopathy and dry beriberi; and there were no cases of wet beriberi. Half the patients achieved complete resolution of symptoms, whereas 47% and 40% had residual weakness or persistent sensory symptoms, respectively. There was no mortality. Most common concurrent nutritional deficiencies were of vitamins K, D, and A. CONCLUSIONS: This is the first in-depth study of TD among adolescents after MBS. Although TD is uncommon among adolescents after MBS, it is serious, requiring diligent suspicion and prompt treatment. Bariatric teams should emphasize compliance with multivitamin regimens and follow it up.

Wernicke Encephalopathy After Bariatric Surgery: a Literature Review.

Wernicke encephalopathy (WE) is an acute neurological disorder classically characterized by ataxia, ophthalmoplegia, and altered mental status. This is caused by thiamine deficiency and is usually seen in malnourished populations. However, with the advent and rise of bariatric surgery in the last 50 years, WE has become an increasingly recognized and potentially deadly complication. Here, we review the populations at risk, clinical presentation, and the incidence of WE in the bariatric surgery population from 1985 to 2023. While the predominant procedure shifts throughout the years, the overall incidence of WE per 100,000 cases for the following procedures are sleeve gastrectomy (1.06), gastric band (1.16), RYGB (4.29), and biliopancreatic diversion with duodenal switch (8.92). Thus, early intervention and post-operative supplementation is recommended to prevent WE.

Diagnosis of Wernicke's Encephalopathy in Patients With a Psychiatric History: A Case Series and Literature Review.

Objective: To review the literature on diagnostic criteria and management of Wernicke's encephalopathy (WE) and its application in psychiatric populations.Evidence Review: A PubMed MEDLINE search was conducted in February 2022 and updated in April 2022 for articles published in English between 2012 and 2022 describing clinical findings and treatment of WE. Reference lists of included articles and treatment guidelines were reviewed. Search terms included Wernicke's encephalopathy, thiamine, thiamine administration dosage, and prescribing. Additionally, 2 cases with co-occurring psychiatric and WE manifestations were selected from the consult-psychiatry service between July and December 2021. Pertinent clinical findings and management were extracted from the literature and compared with that of the cases.Findings: 113 titles were retrieved; 39 studies were excluded. Exclusion criteria included studies done in patients < 18 years old, animal studies, studies with no abstract, and studies with no clinical discussion. Twelve articles were added from the grey literature. Eighty-six articles were included in the review. Only 7 studies discussed WE in psychiatry. The results reaffirm the lack of information regarding diagnosis and treatment of WE in the general population. Clinical cases suggest further increased risk in the psychiatric population.Conclusion and Relevance: Evidence suggests underdiagnosis and undertreatment of WE in general populations and psychiatric patients at increased risk for malnutrition. An interdisciplinary approach improves time to diagnosis and management of comorbidities. We recommend dosing guided by clinical response; however, evidence suggests lengthier and higher doses of thiamine may be needed. Addition of neuroleptics may be necessary for management of psychiatric symptoms and relapse prevention.Prim Care Companion CNS Disord 2023;25(4)22nr03447. Author affiliations are listed at the end of this article.

Thiamine deficiency: a commonly unrecognised but easily treatable condition.

Thiamine is present in many foods and is well recognised as an essential nutrient critical for energy metabolism. While thiamine deficiency is commonly recognised in alcoholism, it can present in many other settings where it is often not considered and goes unrecognised. One challenging aspect to diagnosis is that it may have varied metabolic, neurological and cardiac presentations. Here we present an overview of the disorder, focusing on the multiple causes and clinical presentations. Interestingly, thiamine deficiency is likely increasing in frequency, especially among wildlife, where it is linked with changing environments and climate change. Thiamine deficiency should be considered whenever neurological or cardiological disease of unknown aetiology presents, especially in any patient presenting with lactic acidosis.

Wernicke encephalopathy and beriberi disease presenting as STEMI-equivalent.

Thiamine deficiency is commonly associated with malnutrition, alcoholism and bariatric surgery. Thiamine deficiency can manifest in different ways, especially in developing countries: as peripheric neuropathy, as Wernicke encephalopathy or as beriberi disease. The authors present the case of a 72-year-old male, with a hiatal hernia that led to thiamine deficiency due to malnutrition. The initial clinical manifestation was an ST-elevation myocardial infarct equivalent, an ECG with a shark-fin pattern that evolved to a Wellens type B pattern. The patient evolved with severe altered mental status. A Wernicke encephalopathy diagnosis was confirmed by MRI; the patient was medicated with high-dose thiamine, with quick recovery, both neurologic and cardiac. The clinical history and response to treatment confirm the diagnosis of Wernicke encephalopathy and beriberi disease.

Recognizing neonatal refeeding syndrome and thiamin supplementation: A case report.

Refeeding syndrome (RS) is a condition characterized by electrolyte derangements, thiamin deficiency, and organ dysfunction after the provision of nutrition to an individual who had been deprived of nutrients. Published guidelines outlining the recognition and definition of RS exist for adult and children, but there are limited newborn-specific guidelines because of a paucity of available literature and variation in reporting. We describe a small-for-gestational-age, full-term newborn with neonatal encephalopathy who underwent therapeutic hypothermia. Electrolyte derangements and lactic acidosis developed and persisted after the introduction of intravenous nutrition. After the exclusion of other etiologies, neonatal RS was suspected, and the newborn was treated with thiamin. We provide a brief review of the current literature on neonatal RS, present a case report consistent with neonatal RS, and provide suggestions for the utility of thiamin replacement in newborns with suspected neonatal RS.

Diagnostic Dilemma of Lactic Acidosis in an Infant After Liver Transplant: A Case Report.

Thiamine is the cofactor of many enzymes involved in energy metabolism. Patients under total parenteral nutrition are at risk for thiamine deficiency if there is renal thiamine loss or increased thiamine requirements to mitigate systemic diseases. Thiamine deficiency symptoms include seizures, neuropathy, ataxia, peripheral vasodilation, myocardial insufficiency, sudden collapse, and death. In this report, we present an infant liver transplant recipient with progressive lactic acidosis that responded well to thiamine replacement to mitigate a lack of thiamine in total parenteral nutrition.

Early detection of thiamine deficiency by non-thyroidal illness syndrome in a hemodialysis patient.

An 88-year-old male patient on maintenance hemodialysis (HD) therapy experienced gradual losses in appetite and liveliness during the course of 1 month. Physical examinations revealed no abnormalities. However, blood testing indicated non-thyroidal illness syndrome (NTIS) typically observed in patients with severe illness, with serum levels of thyroid stimulating hormone, free triiodothyronine, and free thyroxine of 0.17 µIU/mL, < 1.0 pg/mL, and 0.23 ng/dL, respectively. Brain magnetic resonance imaging to exclude the possibility of central hypothyroidism unexpectedly displayed slight abnormalities inside of the thalami that were characteristic of Wernicke's encephalopathy. Additional examination disclosed low serum thiamine of 20 ng/mL. Thiamine injections of 100 mg at every HD treatment rapidly restored his appetite, liveliness, and NTIS findings. HD patients are at a particularly high risk of thiamine deficiency (TD) and associated severe symptoms due to losses of thiamine during HD sessions. However, its non-specific initial symptoms, including decreases in appetite and liveliness, as well as undetectability in routine blood tests complicate early detection, resulting in underdiagnosis and more severe outcomes. In the present case, TD manifested only as non-specific symptoms and was ultimately revealed by the presence of NTIS, which was resolved with thiamine supplementation. Thus, NTIS might assist in the early detection of TD as an initial sign in HD patients.

B1 Vitamin Deficiency After Bariatric Surgery, Prevalence, and Symptoms: a Systematic Review and Meta-analysis.

B1 deficiency is a very prevalent complication of bariatric surgery. This study reviews prevalence and symptoms of B1 vitamin deficiency after bariatric surgery. PubMed, Scopus, and Web of Science published were searched up to 10 Feb 2022, with the following keywords: Roux-en-Y gastric bypass, one anastomosis gastric bypass, Omega bypass, Mini bypass, Bariatric surgery OR Bariatric surgery, metabolic surgery, Weight loss surgery, Classic gastric bypass, Loop gastric bypass, Gastric Bypass, thiamine OR thiamin, beriberi, B1. A total of 11 studies examining 1494 patients were included in this meta-analysis. Twenty-seven percent of patients who underwent bariatric surgeries experience vitamin B1 deficiency. Thiamine supplements should be prescribed for the patients for the rest of their lives, and also standard post-surgery follow-ups are necessary in terms of monitoring dietary factors.

A Case of Pulmonary Hypertension in a 67-Year-Old Woman with Thiamine Deficiency Following Partial Gastrectomy and Exacerbated by Diuretics.

BACKGROUND Thiamine deficiency often occurs in patients with alcohol abuse and unbalanced diets. However, gastric surgery and/or use of diuretics can also cause this situation. Importantly, thiamine deficiency can cause pulmonary hypertension, which is completely reversible. This report is of a case of a 67-year-old woman who presented with pulmonary hypertension and thiamine deficiency following partial gastrectomy and exacerbated by diuretics. CASE REPORT A 67-year-old woman with histories of partial gastrectomy because of non-Hodgkin lymphoma (at age 36 years) and sigmoid colectomy because of colon cancer (at age 58 years) presented with bilateral leg edema and dyspnea on exertion. Electrocardiography and right heart catheterization revealed pulmonary hypertension. Despite diuretic administration (initially indapamide, then changed to torsemide), the symptoms gradually worsened. Although she was neither an alcohol drinker nor a fussy eater, we found that her blood thiamine concentration was extremely low. We diagnosed her as having thiamine deficiency caused by gastrectomy and administered diuretics. After intravenous thiamine administration, her symptoms showed immediate improvement, associated with the normalization of the pulmonary hypertension. After detailed analysis of the cause of her pulmonary hypertension, including Swan-Ganz catheterization and echocardiography, we concluded that her pulmonary hypertension was caused by thiamine deficiency following partial gastrectomy and exacerbated by diuretics. CONCLUSIONS This case highlights the importance of recognizing that thiamine deficiency can be a cause of pulmonary hypertension, and that thiamine deficiency can be associated with gastrectomy and the use of diuretics.

Clinical diagnosis, outcomes and treatment of thiamine deficiency in a tertiary hospital.

BACKGROUND: Acute thiamine deficiency can occur in patients with or without history of alcohol abuse and can lead to life-threatening complications. Clinical diagnosis is challenging, often resulting in delayed recognition and treatment. Patients may present with heterogenous symptoms, more diverse than the historical neurological description. Cerebral MRI can contribute to the diagnosis in patients with neurological signs but it is not always feasible in emergency settings. Prompt parenteral supplementation is required to obtain the improvement of symptoms and avoid chronic complications. AIMS: To describe the clinical presentation of reported cases of thiamine deficiency, assess prescription and results of cerebral imaging, review treatments that had been prescribed in accordance or not with available guidelines, and study the short-term outcome of these patients. METHODS: This is a monocentric retrospective analysis of all reported cases of thiamine deficiency in a French tertiary hospital between January 1st 2008 and December 31st 2018. RESULTS: Fifty-six cases were identified during the study period. Forty-five (80%) patients had a history of alcohol abuse. Most patients were diagnosed based on neurological symptoms but non-specific and digestive symptoms were frequent. Thirty-four percent of patients fulfilled clinical criteria for malnutrition. A brain MRI was performed in 54% of patients and was abnormal in 63% of these cases. Eighty-five percent of patients were treated by parenteral thiamine administration and the supplementation was continued orally in 55% of them. The majority of patients initially received 1000 mg daily of IV thiamine but the dose and duration of thiamine supplementation were variable. At the time of discharge, partial or complete improvement of symptoms was noted in 59% of patients. CONCLUSION: This study highlights the clinical and radiological heterogeneity of thiamine deficiency. These observations should encourage starting thiamine supplementation early in patients with risk factors or suggestive symptoms even in non-alcoholic patients, and underline the importance of early nutritional support.

A multicenter randomized clinical trial of pharmacological vitamin B1 administration to critically ill patients who develop hypophosphatemia during enteral nutrition (The THIAMINE 4 HYPOPHOSPHATEMIA trial).

BACKGROUND: Hypophosphatemia may be a useful biomarker to identify thiamine deficiency in critically ill enterally-fed patients. The objective was to determine whether intravenous thiamine affects blood lactate, biochemical and clinical outcomes in this group. METHOD: This randomized clinical trial was conducted across 5 Intensive Care Units. Ninety critically ill adult patients with a serum phosphate ≤0.65 mmol/L within 72 h of commencing enteral nutrition were randomized to intravenous thiamine (200 mg every 12 h for up to 14 doses) or usual care (control). The primary outcome was blood lactate over time and data are median [IQR] unless specified. RESULTS: Baseline variables were well balanced (thiamine: lactate 1.2 [1.0, 1.6] mmol/L, phosphate 0.56 [0.44, 0.64] mmol/L vs. control: lactate 1.0 [0.8, 1.3], phosphate 0.54 [0.44, 0.61]). Patients randomized to the intervention received a median of 11 [7.5, 13.5] doses for a total of 2200 [1500, 2700] mg of thiamine. Blood lactate over the entire 7 days of treatment was similar between groups (mean difference = -0.1 (95 % CI -0.2 to 0.1) mmol/L; P = 0.55). The percentage change from lactate pre-randomization to T = 24 h was not statistically different (thiamine: -32 (-39, -26) vs. control: -24 (-31, -16) percent, P = 0.09). Clinical outcomes were not statistically different (days of vasopressor administration: thiamine 2 [1, 4] vs. control 2 [0, 5.5] days; P = 0.37, and deaths 9 (21 %) vs. 5 (11 %); P = 0.25). CONCLUSIONS: In critically ill enterally-fed patients who developed hypophosphatemia, intravenous thiamine did not cause measurable differences in blood lactate or clinical outcomes. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry (ACTRN12619000121167).

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.

Thiamine supplementation holds neurocognitive benefits for breastfed infants during the first year of life.

Women reliant on mostly rice-based diets can have inadequate thiamine intake, placing breastfed infants at risk of thiamine deficiency and, in turn, physical and cognitive impairments. We investigated the impact of maternal thiamine supplementation doses on infants' cognitive, motor, and language development across the first year. In this double-blind, four-parallel-arm, randomized controlled trial, healthy mothers of exclusively breastfed newborn infants were recruited in Kampong Thom, Cambodia. At 2 weeks postnatal, women (n = 335) were randomized to one of four treatment groups to consume one capsule/day with varying amounts of thiamine for 22 weeks: 0, 1.2, 2.4, and 10 mg. At 2, 12, 24, and 52 weeks of age, infants were assessed with the Mullen Scales of Early Learning (MSEL) and the Caregiver Reported Early Development Instrument (CREDI). Multiple regression and mixed effects modeling suggest that by 6 months of age, the highest maternal thiamine dose (10 mg/day) held significant benefits for infants' language development, but generally not for motor or visual reception development. Despite having achieved standardized scores on the MSEL that approximated U.S. norms by 6 months, infants showed a significant drop relative to these norms in both language domains following trial completion, indicating that nutritional interventions beyond 6 months may be necessary.

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.

Nonalcoholic Wernicke-Korsakoff Syndrome Resulting From Psychosis.

ABSTRACT: Wernicke encephalopathy (WE) results from thiamine deficiency. If undiagnosed or inadequately treated, WE evolves into Korsakoff syndrome (KS). We herein report a case of nonalcoholic Wernicke-Korsakoff syndrome (WKS) that resulted from malnutrition due to psychosis in a 42-years-old male patient. Thiamine deficiency was secondary to severe malnourishment due to poisoning delusions and daily life disorganization in a patient with previously unrecognized schizophrenia. Besides the presence of WE's classic triad of signs, brain magnetic resonance imaging showed also typical thalamic lesions. Furthermore, the patient also presented anterograde and retrograde amnesia, executive dysfunction, and confabulations, compatible with KS being already present. Intravenous treatment with thiamine was given for 37 days. Improvement in cognitive functions and brain imaging alterations was evident. Nevertheless, persistent WKS deficits were present. This case highlights the multiplicity of etiologies of WKS, namely, psychiatric, and its debilitating consequences if not promptly recognized and treated.