Severe Thiamine Research Articles

Poster 109 Prolonged Urinary Retention from Severe Thiamine Deficiency: A Case Report

PM&RVolume 7, Issue 9S p. S127-S127 Friday Poster Presentation Poster 109 Prolonged Urinary Retention from Severe Thiamine Deficiency: A Case Report Kirsten Gage MD, Kirsten Gage MD University of Pittsburgh Medical Center, Pittsburgh, PA, United StatesSearch for more papers by this authorAmanda L. Harrington MD, Amanda L. Harrington MD University of Pittsburgh Medical Center, Pittsburgh, PA, United StatesSearch for more papers by this author Kirsten Gage MD, Kirsten Gage MD University of Pittsburgh Medical Center, Pittsburgh, PA, United StatesSearch for more papers by this authorAmanda L. Harrington MD, Amanda L. Harrington MD University of Pittsburgh Medical Center, Pittsburgh, PA, United StatesSearch for more papers by this author First published: 08 September 2015 https://doi.org/10.1016/j.pmrj.2015.06.148Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume7, Issue9SSeptember 2015Pages S127-S127 RelatedInformation

Beriberi Outbreak among Myanmar and Thai workers in a Factory in Chachoengsao Province, Thailand, 2012-2013

Beriberi is a clinical syndrome which develops from prolonged severe thiamine deficiency in diet. In July 2013, the Ministry of Public Health, Thailand received reports of three deaths among Myanmar workers in a factory. We identified suspect beriberi cases, reviewed clinical history and conducted a survey among both Myanmar and Thai workers in the factory. Blood thiamine levels were measured and foods served in the factory canteen were also examined. Seventeen suspect cases (attack rate = 17%) were identified, with median age of 26 years (range 20-30 years) and male to female ratio of 3.3:1. All fatalities were young men (case fatality proportion = 17.6%). Prevalence of thiamine deficiency among Myanmar and Thai workers were 7.1% (95% CI = 0-22.0) and 26.7% (95% CI = 1.3-52.0) respectively. This outbreak was likely to be caused by long exposure to low thiamine intake and heavy physical activities. After distributing thiamine supplements and improving diet, no more cases occurred. We recommended improving hospital staff’s ability to differentiate beriberi from other cardiomyopathies and neurologic diseases, and raising awareness of thiamine deficiency in this area.

Thiamine Deficiency in Infancy: Long-Term Follow-Up

BackgroundIn 2003, several hundred Israeli infants risked thiamine deficiency after being fed a soy-based formula deficient in thiamine. Approximately 20 patients were seriously affected, and three of them died. We report the clinical presentation of acute encephalopathy in 11 children and the long-term sequelae of eight children who initially survived. PatientsIn the acute phase, six had bulbar signs, five had ophthalmologic signs and two had phrenic neuropathy. Three of the five patients with cardiac involvement had cardiomyopathy and died in the acute phase. One patient presented with a complete atrioventricular block. ResultsIn the long-term, one patient, who was in a chronic vegetative state, died after 6 years. Seven children exhibited mental retardation and motor abnormalities, six developed severe epilepsy, two early kyphoscoliosis, and one patient remained with a complete atrioventricular block. ConclusionsInfants who survive severe infantile thiamine deficiency have serious residual motor and cognitive sequelae as well as epilepsy.

Anterograde episodic memory in Korsakoff syndrome.

A profound anterograde memory deficit for information, regardless of the nature of the material, is the hallmark of Korsakoff syndrome, an amnesic condition resulting from severe thiamine (vitamin B1) deficiency. Since the late nineteenth century when the Russian physician, S. S. Korsakoff, initially described this syndrome associated with "polyneuropathy," the observed global amnesia has been a primary focus of neuroscience and neuropsychology. In this review we highlight the historical studies that examined anterograde episodic memory processes in KS, present a timeline and evidence supporting the myriad theories proffered to account for this memory dysfunction, and summarize what is known about the neuroanatomical correlates and neural systems presumed affected in KS. Rigorous study of KS amnesia and associated memory disorders of other etiologies provide evidence for distinct mnemonic component processes and neural networks imperative for normal declarative and nondeclarative memory abilities and for mnemonic processes spared in KS, from whence emerged the appreciation that memory is not a unitary function. Debate continues regarding the qualitative and quantitative differences between KS and other amnesias and what brain regions and neural pathways are necessary and sufficient to produce KS amnesia.

The Role of Micronutrients in Heart Failure

Heart failure is a common condition in the Western world, particularly among elderly persons and with an ever-aging population, the incidence is expected to increase. Diet in the setting of heart failure is important—patients with this condition are advised to consume a low-salt diet and monitor their weight closely. Nutritional status of patients with heart failure also is important—those with poor nutritional status tend to have a poor long-term prognosis. A growing body of evidence suggests an association between heart failure and micronutrient status. Reversible heart failure has been described as a consequence of severe thiamine and selenium deficiency. However, contemporary studies suggest that a more subtle relationship may exist between micronutrients and heart failure. This article reviews the existing literature linking heart failure and micronutrients, examining studies that investigated micronutrient intake, micronutrient status, and the effect of micronutrient supplementation in patients with heart failure, and focusing particularly on vitamin A, vitamin C, vitamin E, thiamine, other B vitamins, vitamin D, selenium, zinc, and copper.

Wernicke’s encephalopathy: a preventable cause of maternal death

Wernicke's encephalopathy is a rare cause of maternal death. It is a difficult diagnosis to make but prevention and treatment is straightforward. Severe thiamine deficiency causes Wernicke-Korsakoff syndrome. Correct diagnosis and treatment with thiamine will decrease the case fatality rate.

Severe thiamine deficiency following bariatric surgery – Case report

Thiamine deficiency has 4 clinical forms: dry beriberi, wet (chronic and acute), Wernicke encephalopathy and Korsakoff syndrome. It can be related to intake reduction, impaired absorption or increased consumption conditions. The present report shows the case of an obese patient who underwent bariatric surgery, and after 3 months presented with severe vitamin deficiency and evident beriberi symptoms and Wernicke, with significant improvement after nutrient replenishment.

Characteristic properties of metabolism of the yeast Yarrowia lipolytica during the synthesis of α-ketoglutaric acid from ethanol

The study of free amino acid content in Yarrowia lipolytica cells grown on ethanol under thiamine deficiency showed that glutamate, alanine, and γ-aminobutyric acid (γ-ABA) occurred in the highest concentrations among the present 17 free amino acids. The culture liquid contained no amino acids. Analysis of the enzymes of oxidative metabolism in the yeast grown under these conditions showed that the cell-free homogenate contained substantial activity of glutamate decarboxylase, γ-ABA transaminase, and succinyl semialdehyde dehydrogenase. This result indicated the formation of succinate from glutamate in a reaction catalyzed by 4-aminobutyrate aminotransferase (γ-aminobutyrate bypass) under severe thiamine deficiency. These studies lead to the conclusion that cultivation of the yeast Y. lipolytica on ethanol under thiamine deficiency causes adaptive stress-induced metabolic changes. Increase of ammonium nitrogen consumption and excretion of α-ketoglutaric acid are indicative of physiological changes, the functioning of the γ-aminobutyrate bypass and high activity of malate dehydrogenase are manifestations of metabolic changes, and increased activities of the transamination reactions reflect the changes in nitrogen metabolism.

EPILEPSY IN CHILDREN WITH INFANTILE THIAMINE DEFICIENCY

Objective: To report the follow-up findings of 7 children with severe epilepsy as a result of thiamine deficiency in infancy caused by a defective soy-based formula. Methods: The medical records of 7 children aged 5-6 years with thiamine deficiency in infancy who developed epilepsy were reviewed and their clinical data, EEG tracings, and neuroimaging results were recorded. The clinical course and present outcome of these children, now 5 years after exposure to thiamine deficiency, are described. Results: All infants displayed seizures upon presentation, either tonic, myoclonic, or focal. Six infants had an EEG recording at this stage and all showed slow background. Five of them had no epileptic activity and only 1 displayed focal activity. Following a seizure-free period of 1-9 months, the seizures recurred, and all 7 children displayed either myoclonic or complex partial seizures. Multifocal or generalized spike wave complexes were recorded on the EEGs of all 7 patients, and the tracings of 3 children evolved into hypsarrhythmia. The seizures were refractory to most antiepileptic drugs, and 4 children remain with uncontrolled seizures. All children have mental retardation and motor disabilities as well as symptoms of brainstem dysfunction. Conclusions: Our findings indicate that severe infantile thiamine deficiency may result in epilepsy.

Spatial memory deficits and thalamic serotonergic metabolite change in thiamine deficient rats

The purposes of the present study were to verify the effects of a severe thiamine deficiency episode on spatial cognitive aspects and thalamic serotonergic parameters. The animals were submitted to a severe thiamine deficiency treatment that was interrupted after the onset of the last neurological signs. The results obtained confirm previous findings about TD deficiency effects on cognitive function and, further show that this vitamin increases the thalamic serotonine metabolite, 5-hidroxyindolacetic acid (5-HIAA), level. In addition, the present data shed light on the importance of this metabolite in spatial cognitive function.

Epilepsy in children with infantile thiamine deficiency

To report the follow-up findings of 7 children with severe epilepsy as a result of thiamine deficiency in infancy caused by a defective soy-based formula. The medical records of 7 children aged 5-6 years with thiamine deficiency in infancy who developed epilepsy were reviewed and their clinical data, EEG tracings, and neuroimaging results were recorded. The clinical course and present outcome of these children, now 5 years after exposure to thiamine deficiency, are described. All infants displayed seizures upon presentation, either tonic, myoclonic, or focal. Six infants had an EEG recording at this stage and all showed slow background. Five of them had no epileptic activity and only 1 displayed focal activity. Following a seizure-free period of 1-9 months, the seizures recurred, and all 7 children displayed either myoclonic or complex partial seizures. Multifocal or generalized spike wave complexes were recorded on the EEGs of all 7 patients, and the tracings of 3 children evolved into hypsarrhythmia. The seizures were refractory to most antiepileptic drugs, and 4 children remain with uncontrolled seizures. All children have mental retardation and motor disabilities as well as symptoms of brainstem dysfunction. Our findings indicate that severe infantile thiamine deficiency may result in epilepsy.

Wernicke's Encephalopathy Presenting as Acute Psychosis after Gastric Bypass

Background and Objectives: Patients frequently present to the Emergency Department (ED) with psychiatric complaints. The differential diagnosis for acute psychosis is extensive, and determining a possible etiology requires a thorough history and physical. Small details can help the physician in differentiating organic disease from non-organic disease. Many times patients are thought to be “crazy” without a thorough history and physical being done. Case Report: In this case, the diagnosis hinged on the history of having gastric bypass surgery. A thorough physical examination was performed, and the patient had neurologic findings suggestive of severe thiamine deficiency. The patient's thiamine level was low. The patient was started on i.v. thiamine and slowly began to recover. Conclusion: Cerebral beriberi, more commonly known as Wernicke's encephalopathy, is a difficult diagnosis to make in the ED. A thorough neurologic examination is difficult to perform in the ED environment, but it is necessary when trying to determine the etiology of the patient's altered mental status. The number of patients with Wernicke's encephalopathy may increase with bariatric surgery becoming more common. This disease can present with a wide variety of findings, and the classic triad is not very common. With this in mind, the physician should have a low threshold for administering thiamine intravenously.

Wernicke's Encephalopathy Complicating Pregnancy in a Woman with Neonatal Necrotizing Enterocolitis and Resultant Chronic Malabsorption

Wernicke's encephalopathy (WE) is a metabolic disturbance resulting from severe thiamine deficiency classically described in malnourished alcoholics. Untreated, it can result in stupor, coma, and death. WE has previously been reported as a complication of pregnancy in women with hyperemesis gravidarum. We report a case of WE complicating pregnancy in a woman with chronic malabsorption secondary to premature birth and subsequent necrotizing enterocolitis (NEC). Our patient progressed through classic stages of WE before lapsing into a coma. She made a complete recovery after aggressive intravenous thiamine and nutritional support. This is the first report of WE in pregnancy secondary to NEC-related chronic malabsorption. We report this case to bring attention to a potential pregnancy complication affecting women with malabsorptive conditions.

Severe thiamine deficiency complicated by weight loss protects against renal ischaemia-reperfusion injury in rats.

Injury due to reperfusion after prior ischaemia (IRI) is an important cause of delayed graft function after renal transplantation [1]. Studies in dogs and rats suggest that thiamine is protective against IRI in heart and brain [2–4]. It has been argued that many organs, including kidneys are deficient in thiamine at the moment of transplantation [4]. We aimed to investigate the effect of severe tissue thiamine deficiency on ischaemia-reperfusion injury in rat kidneys.

Peripheral Neuropathy and Severe Malnutrition following Duodenal Switch

Severe thiamine (vitamin B-1) deficiency is a medical emergency that has long been recognized as a potential complication of bariatric surgery. The incidence of this rare complication is largely unknown. We describe a super-obese male patient with extreme lower limb weakness 3 months following a duodenal switch operation, occurring in association with persisting vomiting. Excessive malabsorption led to severe malnutrition, with lower limb edemas and clinical evidence of ascites and pleural effusion. Blood tests revealed low levels of albumin, hemoglobin, potassium, vitamins A, B-1, and B-6, and elevated prothrombin time. The symptoms of neuropathy improved after extensive nutritional therapy. Weight eventually stabilized following elongation of the common channel. This case report demonstrates the importance of awareness of neurological complications following bariatric surgery. These complications require urgent and vigorous therapy when they occur.

Severe Thiamine Deficiency in Sheep with Acute Ruminal Lactic Acidosis

Thiamine status of ruminants is adversely affected in acidic rumen conditions. However, there have been limited published case study data related to thiamine deficiency of ruminants with acute ruminal lactic acidosis (ARLA). Thiamine deficiency would occur in sheep with ARLA. Thirteen Ak-Karaman (white Karaman) sheep with ARLA, aged 1 year (ARLA group) and 10 healthy Ak-Karaman sheep, aged 1 year (control group) were used. After clinical examination, rumen fluid samples of all sheep were obtained with a stomach tube and examined immediately. Blood samples were taken from a jugular vein of the sheep. Erythrocytic transketolase enzyme activity and hence thiamine pyrophosphate (TPP) effect were determined according to Clausen's method. History revealed that all sheep in the ARLA group had accidentally consumed excessive amounts of cracked barley. During clinical examination of the ARLA group, disturbed general condition, engorged scleral vessels, moderate to severe dehydration, and ruminal atony were recorded in the sheep. The results of the ruminal fluid analyses of the ARLA group demonstrated characteristics of ARLA. The results of clinical and ruminal fluid examination of control group were normal. The mean TPP effect (%) in the ARLA group (109 +/- 28) was significantly higher than in the control group (22.2 +/- 3.7) (P < .001). The present study revealed that severe thiamine deficiency occurred in sheep with ARLA. This result indicates that thiamine administration to sheep suffering from acute ruminal acidosis caused by overconsumption of readily fermentable carbohydrates could be beneficial in alleviating thiamine deficiency caused by ruminal acidosis.

Hyperemesis gravidarum : une cause rare d'encéphalopathie de Wernicke

Wernicke encephalopathy - most often observed in alcoholic patients - is due to severe thiamine deficiency. We report here the case of a 30-year-old woman who presented with hyperemesis and vomiting during the first trimester of pregnancy (hyperemesis gravidarum). Hyperemesis can lead to severe, symptomatic thiamine deficiency and to severe dehydration with prerenal azotemia. Wernicke encephalopathy is a rare complication of hyperemesis gravidarum. It should be diagnosed as early as possible to prevent long-term complications.

Loop Diuretic Therapy, Thiamine Balance, and Heart Failure

Thiamine, or vitamin B1, is a water-soluble B complex vitamin that was first discovered in 1910 in the process of exploring how rice bran cured patients of beriberi. Thiamine is not synthesized in humans, therefore its availability for necessary cellular processes hinges on its continual ingestion. The amount of thiamine one needs to ingest to maintain balance is disease state-dependent or medication-dependent. Severe chronic thiamine deficiency can have significant neurologic and cardiac effects, the latter is reflected in a particular type of heart failure called wet beriberi. This form of heart failure clearly benefits from thiamine supplementation. It is unclear, however, whether thiamine supplementation offers any benefit in other forms of heart failure. Despite this, it is not unreasonable for heart failure patients to routinely ingest a thiamine-containing multivitamin; patients using diuretics have an increased urinary excretion of thiamine and thus are at a higher risk for developing thiamine deficiency. The role of thiamine in heart failure, however, remains arguable.

Severe thiamine deficiency resulted in Wernicke's encephalopathy in a chronic dialysis patient

A 64-year-old male patient with diabetic nephropathy had been treated with maintenance hemodialysis therapy for 4 years, and had developed disturbed consciousness. The disturbance was firstly noticed by a primary care doctor who recognized slow responses in conversation. Prior to developing this symptom, the patient had noticed a loss of appetite for about 2 weeks. During a period of observation at an outpatient clinic, the symptoms became worse. He was admitted to a primary care hospital for 10 days, but his consciousness level deteriorated and he became unconscious (JCS 200). About 1 month after the onset of symptoms, the patient was transferred to our hospital. A brain computed tomography (CT) scan and magnetic resonance imaging (MRI) showed typical abnormal lesions in the aquaduct of the midbrain and thalamus, and a diagnosis of Wernicke's encephalopathy was made. In addition, the patient's serum thiamine level was extremely low (7 ng/ml). He received immediate treatment with intravenous thiamine administration (150 mg/day), and this significantly improved his symptoms (JCS 2). Dialysis patients may develop water-soluble vitamin deficiency as a result of the combination of reduced oral intake and increased loss of vitamins into the dialysate. Wernicke's encephalopathy should be considered as one of many causes of disturbed consciousness in hemodialysis patients. A rapid diagnosis and adequate treatment are essential in order to minimize long-term neurological sequelae.

Acute thiamine deficiency in diabetic ketoacidosis: Diagnosis and management

Persistent encephalopathy in a patient with diabetic ketoacidosis is often feared as a sign of cerebral edema. Although thiamine deficiency is a rare diagnosis in children, marginal nutritional status and osmotic diuresis may be risk factors. The objective was to describe a heretofore unreported cause of encephalopathy in a child with diabetic ketoacidosis and review the mechanisms and pathophysiology of thiamine deficiency in this clinical scenario. Case report and review of the literature. Pediatric intensive care unit of a tertiary care pediatric hospital. A 13-yr-old girl. Treatment of dehydration and hyperglycemia, osmotherapy, and intravenous thiamine administration. The patient presented with new-onset diabetes mellitus, severe diabetic ketoacidosis, and significant encephalopathy. Despite biochemical improvement with treatment of dehydration and hyperglycemia, her encephalopathy persisted. Computed tomography did not show cerebral edema and she showed no response to osmotherapy. Quantitative and functional assays revealed severe thiamine deficiency. The patient showed an immediate and dramatic response to intravenous thiamine administration. The clinical improvement as well as lab investigations suggests that thiamine deficiency was the cause of this child's encephalopathy. Because potential mechanisms exist for thiamine deficiency in diabetes mellitus and institution of insulin and glucose therapy may stress thiamine body stores, thiamine deficiency should be considered in children with diabetic ketoacidosis whose encephalopathy does not improve with improvement of biochemical status.