Thiamine Deficiency Research Articles

Daily supplementation of a multiple micronutrient powder improves folate but not thiamine, riboflavin, or vitamin B12 status among young Laotian children: a randomized controlled trial

PurposeTo assess the effects of intervention with a daily multiple micronutrient powder (MNP) on thiamine, riboflavin, folate, and B12 status among young Laotian children.MethodsChildren (n = 1704) aged 6–23 mo, participating in a double-blind placebo-controlled randomized trial were individually randomized to receive daily either MNP (containing 0.5 mg of thiamine, 0.5 mg riboflavin, 150 μg folic acid, and 0.9 μg vitamin B12 along with 11 other micronutrients) or placebo and followed for ~ 36 weeks. In a randomly selected sub-sample of 260 children, erythrocyte thiamine diphosphate (eThDP), plasma folate and B12 concentrations, and erythrocyte glutathione reductase activation coefficient (EGRac; riboflavin biomarker) were assessed at baseline and endline.ResultsThere was no treatment effect on endline eThDP concentrations (110.6 ± 8.9 nmol/L in MNP vs. 109.4 ± 8.9 nmol/L in placebo group; p = 0.924), EGRac (1.46 ± 0.3 vs. 1.49 ± 0.3; p = 0.184) and B12 concentrations (523.3 ± 24.6 pmol/L vs. 515.9 ± 24.8 pmol/L; p = 0.678). Likewise, the prevalence of thiamine, riboflavin, and B12 deficiencies did not differ significantly between the two groups. However, endline folate concentration was significantly higher in the MNP compared to the placebo group (28.2 ± 0.8 nmol/L vs 19.9 ± 0.8 nmol/L, respectively; p < 0.001), and correspondingly, the prevalence of folate deficiency was significantly lower in the MNP group (1.6% vs 17.4%; p = 0.015).ConclusionsCompared to a placebo, daily MNP for 9 months increased only folate but not thiamine, riboflavin, or B12 status in young Laotian children.Trial registrationThe trial was registered at www.clinicaltrials.gov (NCT02428647) on April 29 2015.

Giant Cell Arteritis of the Superior Mesenteric Artery Presenting with Wernicke Encephalopathy from Thiamine Deficiency (P1-1.Virtual)

Giant Cell Arteritis of the Superior Mesenteric Artery Presenting with Wernicke Encephalopathy from Thiamine Deficiency (P1-1.Virtual)

Prevalence and Time Course of Thiamine Deficiency in Critically Ill Children: A Multicenter, Prospective Cohort Study in Turkey.

To determine the prevalence and time course of thiamine deficiency (TD) in PICU patients. Multicenter, prospective, cohort study between May 2019 and November 2019. Three university-based tertiary care, mixed medical-surgical PICUs in Ankara, Turkey. PICU patients 1 month to 18 years old. None. We studied 476 patients and grouped them by TD status on days 1 and 3 of the PICU admission. There might be a risk of unintended bias since we excluded 386 patients because of the absence of consent, inadequate blood samples, loss of identifier information, and recent vitamin supplementation. On day 1, TD was present in 53 of 476 patients (11.1%) and median (minimum-maximum) thiamine levels were 65.5 ng/mL (5-431 ng/mL). On day 3, TD was present in 27 of 199 patients (13.6%) with repeated measurement. The median (minimum-maximum) thiamine levels were 63 ng/mL (13-357 ng/mL). The time course of TD from day 1 to day 3 in these 199 patients was as follows. In 21 of 199 patients (10.6%) with TD on day 1, 11 of 21 (52%) continued to have TD on day 3 and the other 10 of 21 patients (48%) improved to no longer having TD. In 178 of 199 patients (89.4%) without TD on day 1, 16 of 178 (9%) went on to develop TD by day 3, and the other 162 of 178 (91%) continued to have normal thiamine status. In the PICU population in three centers in Turkey, the prevalence of TD in the sample of patients was 11.1%. In those TD patients who had serial studies, we also identified that by day 3 some continued to be TD, and some patients improved to normal thiamine status. Of concern, however, is the population who develop TD over the course of PICU stay.

The long-lasting effects of thiamine deficiency in infancy on language: A study of a minimal-pair of twins

Thiamine, vitamin B1, is a crucial component in brain development. This study examined the role thiamine plays in the development of language, by examining the long-term effects of thiamine deficiency in infancy. The participants were a young adult who had consumed a thiamine-deficient baby formula at age 1;0–1;5, and her non-identical twin sister, who had consumed a non-deficient formula. We conducted a comprehensive assessment of various language abilities, including syntax, morphology, lexical encoding and retrieval, word and nonword reading, and phonological working memory, most of which have not been previously tested in individuals who had thiamine deficiency in infancy. The twin who had thiamine deficiency showed selective deficits in various language domains, including syntactic movement, morphology, and lexical abilities (which also caused surface dyslexia in reading aloud). She also showed impaired input and output phonological working memory and impaired reading aloud of nonwords (involving voicing errors, morphological errors, and lexicalizations). Her twin sister, who did not have thiamine deficiency, showed typical language abilities. The findings show for the first time that language disorders due to thiamine deficiency in infancy persist into adulthood. In light of previous literature of adults whose thiamine deficiency took place in adulthood, who do not show language impairments, we suggest that thiamine is crucial for language development during the critical period for first language acquisition in the first years of life. Thiamine deficiency during the critical period may cause long-lasting impairments in syntax, morphology, reading, phonological working memory, and lexical abilities.

The Mammillary Bodies: A Review of Causes of Injury in Infants and Children.

Despite their small size, the mammillary bodies play an important role in supporting recollective memory. However, they have typically been overlooked when assessing neurologic conditions that present with memory impairment. While there is increasing evidence of mammillary body involvement in a wide range of neurologic disorders in adults, very little attention has been given to infants and children. Literature searches of PubMed and EMBASE were performed to identify articles that describe mammillary body pathology on brain MR imaging in children. Mammillary body pathology is present in the pediatric population in several conditions, indicated by signal change and/or atrophy on MR imaging. The main causes of mammillary body pathology are thiamine deficiency, hypoxia-ischemia, direct damage due to masses or hydrocephalus, or deafferentation resulting from pathology within the wider Papez circuit. Optimizing scanning protocols and assessing mammillary body status as a standard procedure are critical, given their role in memory processes.

Gayet-Wernicke Encephalopathy Complicating a Pregnancy of 17 Weeks of Amenorrhea: A Case Report

Gayet-Wernicke encephalopathy is a neuropsychiatric emergency due to thiamine (vitamin B1) deficiency, secondary to several factors, it is a disorder characterized by confusion of acute onset, nystagmus, partial ophthalmoplegia and ataxia. The diagnosis is mainly clinical. The disorder may resolve with treatment, persist, or degenerate into Korsakoff's psychosis. We report a case of Gayet-Wernicke encephalopathy in a 39 year old non-ethylic patient, pregnant at 17 weeks of age who presented with consciousness disorders with paresthesia of the 4 limbs and dyspnea. Clinical and magnetic resonance imaging (MRI) have an important place, especially in the diagnosis of non-alcoholic Wernicke's encephalopathy.

Thiamine Deficiency in Children with Type I Diabetes Mellitus and Ketoacidosis Hospitalized in a Referral Pediatric Hospital in 2019 - 2020

Background: Thiamine is an essential coenzyme, reduced in type I and II diabetes. There has been little research on thiamine levels and their role in children with diabetic ketoacidosis (DKA). Objectives: This study aimed to analyze thiamine deficiency in this patient group. Methods: A cross-sectional study was done in 2019 - 2020 in Children’s Medical Center, a pediatric referral hospital with 350 beds, on children with type I diabetes hospitalized in the PICU for DKA. A blood sample was taken on admission to obtain biochemical laboratory parameters and measure electrolytes and thiamine levels. Blood gases were taken regularly, and the first pH and first bicarbonate level were measured. The time required for pH normalization and recovery from acidosis was recorded. Hospital stay duration was also calculated. Plasma thiamine measurement was done with a Human Vitamin B1 (VB1) ELISA Kit from Bioassay Technology Laboratory. Data were analyzed with SPSS version 22 software. Results: Of 62 patients, 56.5% were females with a mean age of 63 months. Thiamine level was 1.61 ± 1.17 μg/dL, and 66.1% of the patients were thiamine deficient. Hospitalization duration was 3.52 ± 0.41 days in the thiamine deficient group and 2.47 ± 0.32 days in the normal group (P value &lt; 0.05). The white blood cell (WBC) count was higher in thiamine deficient patients. Thiamine levels were independently and inversely related to age. Conclusions: Thiamine deficiency is common among children with DKA and could be a prognostic and therapeutic factor.

Thiamine Level in Out-of-hospital Cardiac Arrest Patients

BACKGROUND: Thiamine deficiency is more common in critically ill patients. Administration of thiamine in cardiac arrest mice has improved survival and neurological outcomes. Evidence for thiamine deficiency in cardiac arrest humans is insufficient to support routine use of thiamine in cardiac arrest patients. AIM: This study aimed to determine thiamine blood levels in cardiac arrest patients to understand whether the presence of thiamine deficiency is common in cardiac arrest patients. METHODS: A prospective descriptive study from April 2017 to March 2018, on 24 adult out-of-hospital cardiac arrest patients. We used the high-performance liquid chromatography technique to determine whole blood thiamine pyrophosphate levels in cardiac arrest patients who arrived at the emergency department within 1 h of the onset of a cardiac arrest. RESULTS: The mean thiamine pyrophosphate level within 1 h of the onset of a cardiac arrest was 170.9 ± 56.7 nmol/L. Only one participant had thiamine deficiency according to the cut-off level for thiamine pyrophosphate in whole blood of &lt;70 nmol/L. Fourteen patients had spontaneous return of circulation. Thiamine pyrophosphate levels were not different between the two groups of patients who had and did not have the return of spontaneous circulation at the emergency department. CONCLUSION: Little evidence was found to support thiamine deficiency as a feature among our cardiac arrest patients. A study with a larger population is required for more meaningful statistical analysis. As there is no consensus on cut-off level for thiamine deficiency diagnosis, the level of thiamine pyrophosphate in specific populations should be evaluated to establish reference values.

Encephalitis-like Presentation in Infants of Bodo Tribe — Thiamine Deficiency or Leigh-like Disease?

Encephalitis-like Presentation in Infants of Bodo Tribe — Thiamine Deficiency or Leigh-like Disease?

Thiamine deficiency in an adolescent following bariatric surgery

Adolescent obesity is a growing health epidemic in the United States and is associated with numerous comorbidities. Bariatric surgery has become an effective intervention for the management of severe obesity in adolescent patients. These procedures, however, are not without possible adverse complications, including vitamin and mineral deficiencies. Here we review the case of a teenage girl who presented on several occasions to the Emergency Department with nausea, vomiting, headaches and abdominal pain after a sleeve gastrectomy procedure. Her clinical presentation, diagnosis, and treatment of Thiamine Deficiency post-sleeve gastrectomy is discussed.

High Lipid Content of Prey Fish and n-3 PUFA Peroxidation Impair the Thiamine Status of Feeding-Migrating Atlantic Salmon (Salmo salar) and Is Reflected in Hepatic Biochemical Indices.

Signs of impaired thiamine (vitamin B1) status in feeding-migrating Atlantic salmon (Salmo salar) were studied in three Baltic Sea areas, which differ in the proportion and nutritional composition of prey fish sprat (Sprattus sprattus) and herring (Clupea harengus). The concentration of n−3 polyunsaturated fatty acids (n−3 PUFAs) increased in salmon with dietary lipids and n−3 PUFAs, and the hepatic peroxidation product malondialdehyde (MDA) concentration increased exponentially with increasing n−3 PUFA and docosahexaenoic acid (DHA, 22:6n−3) concentration, whereas hepatic total thiamine concentration, a sensitive indicator of thiamine status, decreased with the increase in both body lipid and n−3 PUFA or DHA concentration. The hepatic glucose 6-phosphate dehydrogenase activity was suppressed by high dietary lipids. In salmon muscle and in prey fish, the proportion of thiamine pyrophosphate increased, and that of free thiamine decreased, with increasing body lipid content or PUFAs, or merely DHA. The thiamine status of salmon was impaired mainly due to the peroxidation of n−3 PUFAs, whereas lipids as a source of metabolic energy had less effect. Organochlorines or general oxidative stress did not affect the thiamine status. The amount of lipids, and, specifically, their long-chain n−3 PUFAs, are thus responsible for generating thiamine deficiency, and not a prey fish species per se.

715 The incidence of refeeding syndrome in burn patients receiving enteral nutrition

IntroductionA major burn injury result in a hypermetabolic response which leads to increased nutritional requirements. Previous studies have shown that burn patients experience severe electrolyte abnormalities that are likely the result of multiple factors, but share similarities to refeeding syndrome (RS). Recently, the American Society for Parenteral and Enteral Nutrition (ASPEN) published consensus recommendations on RS, including clinical definitions and a grading system. Our aim was to evaluate this definition in a cohort of burn patients.MethodsA retrospective analysis was performed of adults admitted to an American Burn Association-verified center from 2015-2020 with a burn injury who received enteral feeding for at least 72 hours. Patients with a length of stay < 7 days were excluded. Patients were categorized as having mild, moderate, or severe RS based on the newly established ASPEN criteria, defined by a decrease in any one, two, or three of serum phosphorus, potassium, and/or magnesium levels from baseline by 10%–20% (mild RS), 20%–30% (moderate RS), or >30% and/or organ dysfunction resulting from a decrease in any of these and/or due to thiamin deficiency (severe RS).ResultsA total of 350 patients were reviewed, from which 72 met the inclusion criteria. The mean patient age was 52 ± 18 years, and the average BMI was 28.3 ± 7.4. The average burn size was 21.7 ±17.0% TBSA and the average length of stay was 36 ± 32 days. A total of 57 (89%) patients had severe RS while five (8%) exhibited moderate and two (3%) developed mild refeeding syndrome. Of the 57 patients with severe RS, hypophosphatemia was the most prevalent (89%) followed by hypokalemia (19%) and hypomagnesemia (13%). Regression analysis comparing those with severe RS to those with mild or moderate RS revealed that number of surgeries performed was positively associated with the development of severe RS (p=0.0006). %TBSA trended but was not statistically significant (p=0.063). All other variables evaluated were not significant on univariate analysis.ConclusionsThe presence of severe electrolyte abnormalities as a result of burn injuries is significantly more common than originally anticipated. Further investigations are needed to determine if these abnormalities truly reflect RS or are primarily a result of the hypermetabolic response characteristic of burn patients.

Incidence of Vitamin B1 (Thiamine) Deficiency Post Mini-Gastric Bypass: A Prospective Study

Incidence of Vitamin B1 (Thiamine) Deficiency Post Mini-Gastric Bypass: A Prospective Study

LENCEPHALOPATHIE DE GAYET-WERNICKE CHEZ UNE PATIENTE SUIVIE POUR PANCREATITE: A PROPOS DUN CAS

Gayet-Wernicke encephalopathy is a serious complication of thiamine deficiency characterized by a clinical triad: confusional state, oculomotor disturbances, and cerebellar ataxia. Alcoholism is the main cause of thiamine deficiency. Severe pancreatitis through prolonged fasting and vomiting may exceptionally give a similar clinical picture which sometimes poses diagnostic difficultiesDiagnosis is based on brain imaging by showing on MRI a T2 or FLAIR hypersignal affecting the peri-aqueductal region, the medial part of the thalamus, the floor of the fourth ventricle, the vermis, the mammillary bodies and, sometimes, the cortex. Treatment consists of rapid intravenous administration of thiamine. We report a case of 66-year-old patient followed for severe pancreatitis whose evolution was marked by the onset of a consciousness disorder whose investigations returned in favor of Gayet-Wernicke encephalopathy.

Thiamine-dependent regulation of mammalian brain pyridoxal kinase in vitro and in vivo.

Vitamins B1 (thiamine) and B6 (pyridox (al/ine/amine)) are crucial for central nervous system (CNS) function and neurogenesis due to the coenzyme action of their phosphorylated derivatives in the brain metabolism of glucose and neurotransmitters. Here, the non-coenzyme action of thiamine on the major mammalian producers of pyridoxal-5'-phosphate (PLP), such as pyridoxal kinase (PdxK) and pyridoxine 5'-phosphate oxidase (PNPO), is characterized. Among the natural thiamine compounds, thiamine triphosphate (ThTP) is the best effector of recombinant human PdxK (hPdxK) in vitro, inhibiting hPdxK in the presence of Mg2+ but activating the Zn2+ -dependent reaction. Inhibition of hPdxK by thiamine antagonists decreases from amprolium to pyrithiamine to oxythiamine, highlighting possible dysregulation of both the B1 - and B6 -dependent metabolism in the chemical models of thiamine deficiency. Compared with the canonical hPdxK, the D87H and V128I variants show a twofold increase in Kapp of thiamine inhibition, and the V128I and H246Q variants show a fourfold and a twofold decreased Kapp of thiamine diphosphate (ThDP), respectively. Thiamine administration changes diurnal regulation of PdxK activity and phosphorylation at Ser213 and Ser285, expression of the PdxK-related circadian kinases/phosphatases in the rat brain, and electrocardiography (ECG). In contrast to PdxK, PNPO is not affected by thiamine or its derivatives, either in vitro or in vivo. Dephosphorylation of the PdxK Ser285, potentially affecting mobility of the ATP-binding loop, inversely correlates with the enzyme activity. Dephosphorylation of the PdxK Ser213, which is far away from the active site, does not correlate with the activity. The correlations analysis suggests the PdxK Ser213 to be a target of kinase MAP2K1 and phosphatase Ppp1ca. Diurnal effects of thiamine administration on the metabolically linked ThDP- and PLP-dependent enzymes may support the brain homeostatic mechanisms and physiological fitness.

Effects of Thiamin Restriction on Exercise-Associated Glycogen Metabolism and AMPK Activation Level in Skeletal Muscle

This study aimed to investigate the direct influence of a decrease in the cellular thiamin level, before the onset of anorexia (one of the symptoms of thiamin deficiency) on glycogen metabolism and the AMP-activated protein kinase (AMPK) activation levels in skeletal muscle at rest and in response to exercise. Male Wistar rats were classified as the control diet (CON) group or the thiamin-deficient diet (TD) group and consumed the assigned diets for 1 week. Skeletal muscles were taken from the rats at rest, those that underwent low-intensity swimming (LIS), or high-intensity intermittent swimming (HIS) conducted immediately before dissection. There were no significant differences in food intake, locomotive activity, or body weight between groups, but thiamin pyrophosphate in the skeletal muscles of the TD group was significantly lower than that of the CON group. Muscle glycogen and lactate levels in the blood and muscle were equivalent between groups at rest and in response to exercise. The mitochondrial content was equal between groups, and AMPK in the skeletal muscles of TD rats was normally activated by LIS and HIS. In conclusion, with a lowered cellular thiamin level, the exercise-associated glycogen metabolism and AMPK activation level in skeletal muscle were normally regulated.

Metabolic Biomarkers in B-Cell Lymphomas for Early Diagnosis and Prediction, as Well as Their Influence on Prognosis and Treatment.

B-cell lymphomas exhibit a vast variety of clinical and histological characteristics that might complicate the diagnosis. Timely diagnosis is crucial, as treatments for aggressive subtypes are considered successful and frequently curative, whereas indolent B-cell lymphomas are incurable and often need several therapies. The purpose of this review is to explore the current advancements achieved in B-cell lymphomas metabolism and how these indicators help to early detect metabolic changes in B-cell lymphomas and the use of predictive biological markers in refractory or relapsed disease. Since the year 1920, the Warburg effect has been known as an integral part of metabolic reprogramming. Compared to normal cells, cancerous cells require more glucose. These cancer cells undergo aerobic glycolysis instead of oxidative phosphorylation to metabolize glucose and form lactate as an end product. With the help of these metabolic alterations, a novel biomass is generated by the formation of various precursors. An aggressive metabolic phenotype is an aerobic glycolysis that has the advantage of producing high-rate ATP and preparing the biomass for the amino acid, as well as fatty acid, synthesis needed for a rapid proliferation of cells, while aerobic glycolysis is commonly thought to be the dominant metabolism in cancer cells. Later on, many metabolic biomarkers, such as increased levels of lactate dehydrogenase (LDH), plasma lactate, and deficiency of thiamine in B-cell lymphoma patients, were discovered. Various kinds of molecules can be used as biomarkers, such as genes, proteins, or hormones, because they all refer to body health. Here, we focus only on significant metabolic biomarkers in B-cell lymphomas. In conclusion, many metabolic biomarkers have been shown to have clinical validity, but many others have not been subjected to extensive testing to demonstrate their clinical usefulness in B-cell lymphoma. Furthermore, they play an essential role in the discovery of new therapeutic targets.

Wernicke’s encephalopathy secondary to hyperemesis gravidarum: a clinical challenge

Wernicke’s encephalopathy (WE) is an acute neurological condition characterized by a triad of ophthalmoplegia, ataxia and altered mental status. The underlying cause is thiamine deficiency, which may be due to multiple aetiologies. Thiamine is essential for carbohydrate and amino acid metabolism. Its deficiency shunts glucose to anaerobic pathways producing metabolic abnormalities. Diagnosing WE relies heavily on clinical suspicion. Magnetic Resonance Imaging can show some specific findings. We report this case of a 35 year old pregnant woman with gestational diabetes who was admitted in hospital for high blood sugar levels and electrolyte abnormalities. She had a history of ten miscarriages. From undergoing laparoscopic cholecystectomy for intractable vomiting to spontaneous expulsion of the foetus to being intubated for acidosis, her hospital stay was prolonged and eventful. Although the cause of her repeated miscarriages could not be established despite extensive workup, Continues...

Annals for Educators - February 2022.

Annals for Educators - February 2022.

Insights Into Thiamine Supplementation in Patients With Septic Shock.

Septic shock is associated with unacceptably high mortality rates, mainly in developing countries. New adjunctive therapies have been explored to reduce global mortality related to sepsis. Considering that metabolic changes, mitochondrial dysfunction and increased oxidative stress are specific disorders within the path of septic shock, several micronutrients that could act in cellular homeostasis have been studied in recent decades. Thiamine, also known as vitamin B1, plays critical roles in several biological processes, including the metabolism of glucose, synthesis of nucleic acids and reduction of oxidative stress. Thiamine deficiency could affect up to 70% of critically ill patients, and thiamine supplementation appears to increase lactate clearance and decrease the vasopressor dose. However, there is no evident improvement in the survival of septic patients. Other micronutrients such as vitamin C and D, selenium and zinc have been tested in the same context but have not been shown to improve the outcomes of these patients. Some problems related to the neutrality of these clinical trials are the study design, doses, route, timing, length of intervention and the choice of endpoints. Recently, the concept that multi-micronutrient administration may be better than single-micronutrient administration has gained strength. In general, clinical trials consider the administration of a single micronutrient as a drug. However, the antioxidant defense is a complex system of endogenous agents in which micronutrients act as cofactors, and the physiological interactions between micronutrients are little discussed. In this context, the association of thiamine, vitamin C and corticoids was tested as an adjunctive therapy in septic shock resulting in a significant decrease in mortality. However, after these initial results, no other study conducted with this combination could reproduce those benefits. In addition, the use of low-dose corticosteroids is recommended in patients with septic shock who do not respond to vasopressors, which can affect the action of thiamine. Therefore, given the excellent safety profile, good biologic rationale and promising clinical studies, this review aims to discuss the mechanisms behind and the evidence for single or combined thiamine supplementation improving the prognosis of patients with septic shock.