RESUMEN
Primary and secondary conditions leading to thiamine deficiency have overlapping features in children, presenting with acute episodes of encephalopathy, bilateral symmetric brain lesions, and high excretion of organic acids that are specific of thiamine-dependent mitochondrial enzymes, mainly lactate, alpha-ketoglutarate, and branched chain keto-acids. Undiagnosed and untreated thiamine deficiencies are often fatal or lead to severe sequelae. Herein, we describe the clinical and genetic characterization of 79 patients with inherited thiamine defects causing encephalopathy in childhood, identifying outcome predictors in patients with pathogenic SLC19A3 variants, the most common genetic etiology. We propose diagnostic criteria that will aid clinicians to establish a faster and accurate diagnosis so that early vitamin supplementation is considered. Ann Neurol 2017;82:317-330.
Asunto(s)
Deficiencia de Tiamina/genética , Adolescente , Edad de Inicio , Niño , Preescolar , Femenino , Humanos , Lactante , Proteínas de Transporte de Membrana/genética , Proteínas de Transporte de Membrana Mitocondrial , Mutación , Pronóstico , Tasa de Supervivencia , Deficiencia de Tiamina/mortalidad , Adulto JovenAsunto(s)
Suplementos Dietéticos , Insuficiencia Cardíaca/tratamiento farmacológico , Deficiencia de Tiamina/tratamiento farmacológico , Tiamina/uso terapéutico , Animales , Suplementos Dietéticos/efectos adversos , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Humanos , Factores de Riesgo , Tiamina/efectos adversos , Deficiencia de Tiamina/diagnóstico , Deficiencia de Tiamina/mortalidad , Deficiencia de Tiamina/fisiopatología , Resultado del TratamientoRESUMEN
Wild birds of several species are dying in large numbers from an idiopathic paralytic disease in the Baltic Sea area. Here, we demonstrate strong relationships between this disease, breeding failure, and thiamine (vitamin B(1)) deficiency in eggs, pulli, and full-grown individuals. Thiamine is essential for vertebrates, and its diphosphorylated form functions as a cofactor for several life sustaining enzymes, whereas the triphosphorylated form is necessary for the functioning of neuronal membranes. Paralyzed individuals were remedied by thiamine treatment. Moreover, thiamine deficiency and detrimental effects on thiamine-dependent enzymes were demonstrated in the yolk, liver, and brain. We propose that the mortality and breeding failure are part of a thiamine deficiency syndrome, which may have contributed significantly to declines in many bird populations during the last decades.
Asunto(s)
Animales Salvajes , Enfermedades de las Aves/mortalidad , Aves , Extinción Biológica , Deficiencia de Tiamina/veterinaria , Animales , Enfermedades de las Aves/patología , Charadriiformes , Europa (Continente) , Geografía , Parálisis/patología , Especificidad de la Especie , Estorninos , Síndrome , Deficiencia de Tiamina/mortalidad , Deficiencia de Tiamina/patologíaAsunto(s)
Tiamina/uso terapéutico , Encefalopatía de Wernicke/diagnóstico , Encefalopatía de Wernicke/tratamiento farmacológico , Enfermedad Crítica/terapia , Diagnóstico Precoz , Urgencias Médicas , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Pronóstico , Medición de Riesgo , Tasa de Supervivencia , Deficiencia de Tiamina/diagnóstico , Deficiencia de Tiamina/tratamiento farmacológico , Deficiencia de Tiamina/mortalidad , Resultado del Tratamiento , Encefalopatía de Wernicke/mortalidadRESUMEN
The objectives of this study were to examine the relationship between thiamine concentrations in unfertilized eggs and yolksac individuals of lake trout (Salvelinus namaycush), along with any associated histopathological changes in the tissues of alevins at the hatching stage. We address these questions in a lake trout population from different spawning grounds of Lake Michigan (North and South), known for compromised survival due to early mortality syndrome (EMS). However, a dichotomous forage base of lake trout spawning stocks, with a dietary thiaminase-rich alewife in the North, and dietary low-thiaminase round goby in the South, provides the basis for the assumption that different diets may lead to differences in severity of EMS between different stocks. Lake trout eggs of 18 females were collected and fertilized individually with the sperm of several males. The eggs, eyed embryos and newly-hatched alevins were sampled to examine thiamine utilization during embryogenesis. Progenies of females with low (< 0.73 nmol/g) and high (> 0.85 nmol/g) levels of thiamine were chosen for histological studies. The obtained results showed that total thiamine levels in the body and yolk of eyed embryos and alevins at hatching were influenced by thiamine levels of unfertilized eggs and it decreased during embryogenesis (to 51% in eyed embryos and 28% in newly-hatched alevins in comparison to unfertilized eggs). The survival of lake trout until hatching stage does not correlate with the thiamine level, however it was affected by collection site and was significantly higher in fish from the South site (Julian's Reef). At the hatching stage, no pathological changes were observed in the brain, olfactory lobe, retina or liver in embryos regardless of thiamine concentrations in unfertilized eggs. It has been concluded that an enhanced thiamine requirement for the fast muscle mass growth near the swim-up stage is responsible for overt and histopathological signs of EMS. Current study confirms earlier findings that lake trout suffering from EMS can be successfully treated by immersion in thiamine solution as late as at the swim-up stage.
Asunto(s)
Enfermedades de los Peces/patología , Estado Nutricional , Deficiencia de Tiamina/veterinaria , Tiamina/análisis , Trucha/metabolismo , Animales , Encéfalo/embriología , Encéfalo/patología , Ojo/embriología , Ojo/patología , Femenino , Enfermedades de los Peces/embriología , Enfermedades de los Peces/metabolismo , Great Lakes Region , Hígado/embriología , Hígado/patología , Glucógeno Hepático/análisis , Óvulo/química , Síndrome , Deficiencia de Tiamina/embriología , Deficiencia de Tiamina/mortalidad , Deficiencia de Tiamina/patología , Tiamina Monofosfato/análisis , Tiamina Pirofosfato/análisis , Trucha/embriología , Trucha/crecimiento & desarrollo , Saco Vitelino/químicaRESUMEN
An investigation of adult alligator (Alligator mississippiensis) mortalities in Lake Griffin, central Florida, was conducted from 1998-2004. Alligator mortality was highest in the months of April and May and annual death count peaked in 2000. Bacterial pathogens, heavy metals, and pesticides were not linked with the mortalities. Blood chemistry did not point to any clinical diagnosis, although differences between impaired and normal animals were noted. Captured alligators with signs of neurologic impairment displayed unresponsive and uncoordinated behavior. Three of 21 impaired Lake Griffin alligators were found to have neural lesions characteristic of thiamine deficiency in the telencephalon, particularly the dorsal ventricular ridge. In some cases, lesions were found in the thalamus, and parts of the midbrain. Liver and muscle tissue concentrations of thiamine (vitamin B(1)) were lowest in impaired Lake Griffin alligators when compared to unimpaired alligators or to alligators from Lake Woodruff. The consumption of thiaminase-positive gizzard shad (Dorosoma cepedianum) is thought to have been the cause of the low tissue thiamine and resulting mortalities.
Asunto(s)
Caimanes y Cocodrilos , Hidrolasas/administración & dosificación , Hidrolasas/metabolismo , Sistema Nervioso/patología , Deficiencia de Tiamina/veterinaria , Caimanes y Cocodrilos/metabolismo , Animales , Causas de Muerte , Femenino , Florida , Masculino , Mortalidad , Examen Neurológico/veterinaria , Estaciones del Año , Tiamina/metabolismo , Tiamina/uso terapéutico , Deficiencia de Tiamina/mortalidad , Deficiencia de Tiamina/patologíaRESUMEN
OBJECTIVE: To test the hypothesis that low blood thiamine concentrations in malnourished critically ill children are associated with higher risk of 30-d mortality. METHODS: Prospective cohort study in 202 consecutively admitted children who had whole blood thiamine concentrations assessed on admission and on days 5 and 10 of intensive care unit (ICU) stay. The primary outcome variable was 30-d mortality. Mean blood thiamine concentrations within the first 10 d of ICU stay, age, sex, malnutrition, C-reactive protein concentration, Pediatric Index of Mortality 2 score, and severe sepsis/septic shock were the main potential exposure variables for outcome. RESULTS: Thiamine deficiency was detected in 61 patients within the first 10 d of ICU stay, 57 cases being diagnosed on admission and 4 new cases on the 5th d. C-reactive protein concentration during ICU stay was independently associated with decreased blood thiamine concentrations (P = 0.003). There was a significant statistical interaction between mean blood thiamine concentrations and malnutrition on the risk of 30-d mortality (P = 0.002). In an adjusted analysis, mean blood thiamine concentrations were associated with a decrease in the mortality risk in malnourished patients (odds ratio = 0.85; 95% confidence interval [CI]: 0.73-0.98; P = 0.029), whereas no effect was noted for well-nourished patients (odds ratio: 1.03; 95% CI: 0.94-1.13; P = 0.46). CONCLUSIONS: Blood thiamine concentration probably has a protective effect on the risk of 30-d mortality in malnourished patients but not in those who were well nourished.
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Trastornos de la Nutrición del Niño/mortalidad , Enfermedad Crítica/mortalidad , Estado Nutricional , Deficiencia de Tiamina/mortalidad , Tiamina/sangre , Brasil/epidemiología , Niño , Trastornos de la Nutrición del Niño/sangre , Preescolar , Femenino , Humanos , Incidencia , Lactante , Unidades de Cuidado Intensivo Pediátrico , Masculino , Estudios Prospectivos , Deficiencia de Tiamina/sangreRESUMEN
As the world increasingly relies on aquaculture operations to meet rising seafood demands, reliable biocontainment measures for farmed fish stocks are desired to minimize ecological impacts arising from interactions of cultured fish with wild populations. One possible biocontainment strategy is to induce a dietary dependence on a vitamin, such as thiamine (vitamin B1), required for survival. Fish expressing thiaminase (an enzyme that degrades thiamine) within a confined aquaculture facility could receive supplemental thiamine to allow survival and normal growth, whereas escapees lacking this dietary rescue would die from thiamine deficiency. To test the concept and efficacy of such a dietary dependency system (for potential future use in larger aquaculture species), we expressed thiaminase in zebrafish as a test model. We drove the expression of thiaminase under the strong ubiquitous and constitutive control of the CMV promoter which resulted in non-viable fish, indicating that the thiaminase sequence kills fish. However, the CMV promoter is too strong to allow conditional survival since the lethality could not be rescued by exogenous thiamine provided as a supplement to typical food. In addition, microinjection of 0.5 pg of thiaminase mRNA in zebrafish embryos at the one-cell stage resulted in 50% larval mortality at 5 days post-fertilization (dpf), which was partially rescued by thiamine supplementation. Evaluating the efficacy of biocontainment strategies helps assess which methods can reliably prevent ecological impacts arising from breaches in physical containment systems that release engineered organisms to nature, and consequently provides critical information for use in regulatory risk assessment processes.
Asunto(s)
Hidrolasas/genética , Deficiencia de Tiamina/veterinaria , Pez Cebra/genética , Animales , Animales Modificados Genéticamente , Acuicultura/métodos , Bacillus thuringiensis/genética , Dieta/veterinaria , Embrión no Mamífero/metabolismo , Hidrolasas/metabolismo , Especies Introducidas , ARN Mensajero/administración & dosificación , Tiamina/administración & dosificación , Deficiencia de Tiamina/mortalidad , Pez Cebra/embriología , Pez Cebra/metabolismoRESUMEN
Patients with end-stage renal disease undergoing regular dialysis are prone to encephalopathy, but the cause is often unclear. Dialysis patients are at risk for thiamine deficiency, which may mimic many uremic complications, including encephalopathy. To determine whether unexplained encephalopathy in regular dialysis patients is associated with thiamine deficiency, we conducted a prospective study that enrolled 30 consecutive dialysis patients with altered mental status admitted to a referred hospital during a 1-year period. A complete history, physical and neurological examinations, laboratory investigations, and computed tomographic scans or magnetic resonance imaging of the brain were obtained for each subject. In 10 of the 30 patients, diagnoses remained obscure after the initial workup. Manifestations included confusion, chorea, acute visual loss, rapidly progressive dementia, myoclonus, convulsions, and coma. Intravenous thiamine was administered to these 10 patients. All 10 patients had thiamine deficiency confirmed by a marked response to thiamine supplementation and/or a low serum thiamine concentration (35.3 +/- 6.0 nmol/L; normal, >50 nmol/L). Nine patients recovered, but one patient failed to respond because of delayed treatment. We conclude that in regular dialysis patients, unexplained encephalopathy can be mainly attributed to thiamine deficiency. This condition is fatal if unrecognized and can be successfully treated with prompt thiamine replacement.
Asunto(s)
Diálisis Peritoneal , Diálisis Renal , Deficiencia de Tiamina/complicaciones , Encefalopatía de Wernicke/patología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Inyecciones Intravenosas , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Tasa de Supervivencia , Tiamina/sangre , Tiamina/uso terapéutico , Deficiencia de Tiamina/tratamiento farmacológico , Deficiencia de Tiamina/mortalidad , Resultado del Tratamiento , Encefalopatía de Wernicke/tratamiento farmacológico , Encefalopatía de Wernicke/etiologíaRESUMEN
There have been recent reports of hospitalised patients developing clinical thiamine deficiency, combined with much debate on the optimal supplementation of thiamine for the parenterally fed patient, particularly in the intensive therapy environment. We performed a retrospective study on 158 patients admitted to the Intensive Care Unit who required nutritional support. Patients who survived had significantly higher body thiamine status than those who died (p less than 0.01). There was no difference between serum albumin concentrations of the two groups. Twenty percent of the patients had biochemical evidence of thiamine deficiency and the mortality rate in these patients was 72% as compared with 50% mortality overall. Follow-up results suggest that current levels of thiamine supplementation are insufficient for critically ill intravenously fed patients. We suggest that patients be given a loading dose of 50-250 mg thiamine on admission to the Intensive Care Unit.
Asunto(s)
Cuidados Críticos , Nutrición Parenteral/efectos adversos , Deficiencia de Tiamina/etiología , Adulto , Anciano , Activación Enzimática , Eritrocitos/enzimología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Deficiencia de Tiamina/sangre , Deficiencia de Tiamina/mortalidad , Transcetolasa/sangreRESUMEN
Rats were fed carbohydrate-free diets without thiamine. Sucrose was offered separately, either before the thiamine was removed, or when it was removed, or at varying periods after it was removed. In the absence of dietary thiamine, sucrose consumption began at a high level, but was then reduced. Thereafter, death due to thiamine deficiency followed at intervals that varied from 4 weeks to 12 months. Survival was shortest in those rats that ate sucrose in largest amounts or at shortest intervals; survival was longest in those rats that avoided sucrose altogether for long periods. Sex or age did not appear to affect survival. The results are interpreted as demonstrating a conflict between the avoidance of sucrose so as to avoid the unpleasant symptoms of thiamine deficiency, and the consumption of sucrose so as to obtain the pleasure of its high palatability.
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Sacarosa , Deficiencia de Tiamina , Factores de Edad , Alimentación Animal , Animales , Reacción de Prevención , Femenino , Preferencias Alimentarias , Masculino , Ratas , Factores Sexuales , Deficiencia de Tiamina/mortalidad , Deficiencia de Tiamina/fisiopatología , Deficiencia de Tiamina/psicología , Factores de TiempoRESUMEN
Thiamine deficiency has been linked to early mortality syndrome in salmonids in the Great Lakes. This study was conducted to compare thiamine concentrations in American alligators (Alligator mississippiensis) and Florida largemouth bass (Micropterus salmoides floridanus) eggs from sites with high embryo mortality and high exposure to organochlorine pesticides (OCPs) (Lakes Apopka and Griffin, and Emeralda Marsh, Florida, USA) to those from sites that have historically exhibited low embryo mortality and low OCPs (Lakes Woodruff and Orange, Florida). During June-July 2000, 20 alligator clutches were collected from these sites, artificially incubated, and monitored for embryo mortality. Thiamine and OCPs were measured in one egg/clutch. During February 2002, 10 adult female bass were collected from Emeralda Marsh and Lake Woodruff and mature ovaries analyzed for thiamine and OCP concentrations. Although ovaries from the Emeralda Marsh bass contained almost 1,000-fold more OCPs compared with the reference site, Lake Woodruff, there were no differences in thiamine concentrations between sites (11,710 vs. 11,857 pmol/g). In contrast, alligator eggs from the reference site had five times the amount of thiamine compared with the contaminated sites (3,123 vs. 617 pmol/g). Similarly, clutches with >55% hatch rates had significantly higher concentrations of thiamine compared with clutches with <54% hatch rates (1,119 vs. 201 pmol/g). These results suggest that thiamine deficiency might be playing an important role in alligator embryo survival but not in reproductive failure and recruitment of largemouth bass. The cause(s) of this thiamine deficiency are unknown but might be related to differences in the nutritional value of prey items across the sites studied and/or to the presence of high concentration of contaminants in eggs.
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Caimanes y Cocodrilos , Lubina , Hidrocarburos Clorados/envenenamiento , Plaguicidas/envenenamiento , Tiamina/metabolismo , Contaminantes Químicos del Agua/envenenamiento , Animales , Huevos/análisis , Exposición a Riesgos Ambientales , Femenino , Enfermedades de los Peces/inducido químicamente , Enfermedades de los Peces/fisiopatología , Florida , Exposición Materna , Mortalidad , Óvulo/química , Reproducción/efectos de los fármacos , Deficiencia de Tiamina/inducido químicamente , Deficiencia de Tiamina/mortalidad , Deficiencia de Tiamina/veterinariaRESUMEN
BACKGROUND: Thiamine deficiency has been associated with poorer clinical outcomes. Early recognition of thiamine deficiency is difficult in critically ill patients because clinical signs are nonspecific. OBJECTIVE: We determined the prevalence of and identified risk factors associated with low blood thiamine concentrations upon admission of children to a pediatric intensive care unit and evaluated this condition as a predictor of clinical outcomes. DESIGN: A prospective cohort study was conducted in 202 children who had whole-blood thiamin concentrations assessed by HPLC upon admission to the intensive care unit. The following independent variables for thiamine deficiency were analyzed: age, sex, nutritional status, clinical severity scores upon admission (ie, the revised Pediatric Index of Mortality and Pediatric Logistic Organ Dysfunction score), systemic inflammatory response measured by C-reactive protein serum concentrations, severe sepsis or septic shock, heart failure, and cardiac surgery. The dependent variables in the outcome analyses were mortality, length of stay, and time on mechanical ventilation. RESULTS: Low blood thiamine concentrations upon admission were detected in 57 patients (28.2%) and were shown to be independently associated with C-reactive protein concentrations >20 mg/dL (odds ratio: 2.17; 95% CI: 1.13, 4.17; P = 0.02) but not with malnutrition. No significant association was shown between low blood thiamine concentrations upon admission and outcome variables. CONCLUSIONS: The incidence of low blood thiamine concentrations upon admission was high. Of the risk factors examined, only the magnitude of the systemic inflammatory response showed an independent association with this event. The association between thiamine deficiency upon admission and prognosis requires further investigation.
Asunto(s)
Unidades de Cuidado Intensivo Pediátrico , Deficiencia de Tiamina/etiología , Tiamina/sangre , Proteína C-Reactiva/análisis , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Masculino , Pronóstico , Estudios Prospectivos , Valores de Referencia , Factores de Riesgo , Sepsis/mortalidad , Deficiencia de Tiamina/mortalidadRESUMEN
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.
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Síndrome de Korsakoff/prevención & control , Deficiencia de Tiamina/prevención & control , Tiamina/uso terapéutico , Encefalopatía de Wernicke/prevención & control , Femenino , Humanos , Síndrome de Korsakoff/complicaciones , Síndrome de Korsakoff/mortalidad , Mortalidad Materna , Embarazo , Pronóstico , Deficiencia de Tiamina/complicaciones , Deficiencia de Tiamina/mortalidad , Encefalopatía de Wernicke/complicaciones , Encefalopatía de Wernicke/mortalidadAsunto(s)
Acidosis Láctica/mortalidad , Nutrición Parenteral Total/mortalidad , Deficiencia de Tiamina/etiología , Acidosis Láctica/diagnóstico , Adulto , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Deficiencia de Tiamina/mortalidad , Factores de Tiempo , Estados UnidosRESUMEN
In separate 20 and 12 week feeding studies, channel catfish fingerlings were fed semipurified diets containing five levels (0, 2.5, 5.0, 10.0 and 15.0 mg/kg) and six levels (0, 0.5, 1.0, 2.5, 5.0 and 10.0 mg/kg) of supplemented thiamin hydrochloride respectively. The dietary thiamin level required to provide maximal growth and prevent deficiency symptoms in channel catfish fingerlings was found to be approximately 1 mg/kg of diet. Fish fed unsupplemented diets (thiamin content of less 1 mg/kg diet) demonstrated anorexia, extremely poor growth, dark coloration of the skin and increased mortality rates. Neurological symptoms were not observed. Histological examinations of the heart, hepatopancreas, kidney, lateral muscle, gastrointestinal tract and gills of deficient fish revealed no abnormalities.
Asunto(s)
Peces/metabolismo , Tiamina , Animales , Anorexia/etiología , Crecimiento , Hematócrito , Necesidades Nutricionales , Tiamina/administración & dosificación , Deficiencia de Tiamina/complicaciones , Deficiencia de Tiamina/mortalidadRESUMEN
A link was previously established between the Cayuga syndrome, a condition causing 100% mortality in larval landlocked Atlantic salmon, Salmo salar, in several of New York's Finger Lakes, and a maternal diet of alewife, Alosa pseudoharengus, a non-native thiaminase-rich Clupeid fish. We evaluated salmon larvae viability relative to maternal thiamin status, and investigated the putative link of the Cayuga syndrome to an alewife diet in fish from the geographic regions outside the Finger Lakes/lower Great Lakes watersheds. We identified Cayuga syndrome in Atlantic salmon from Otsego Lake in the Susquehanna River watershed and from Green Pond in New York's Adirondack Mountains. In both systems alewife represent the major component of the diet for the salmon. Thiamin levels in the maternal blood of Otsego salmon with syndrome-negative progeny were three- to four-fold greater than those Otsego females whose progeny exhibited 100% mortality. Thiamin levels in eggs and larvae were directly related to thiamin levels in maternal blood in both syndrome-positive and syndrome-negative stocks. Thiamin bath treatments of syndrome-afflicted larvae eliminated mortality regardless of their lake stock of origin. Maternal blood levels of approximately 0.31 nmol thiamin pyrophosphate/g or 0.44 nmol total thiamin/g appear necessary to achieve egg threshold levels of approximately 0.8 and 1.1 nmol/g unphosphorylated and total thiamin, respectively; these egg thiamin levels should prevent significant syndrome-related mortality in landlocked Atlantic salmon larvae. These results confirm the role of thiamin in the etiology of the Cayuga syndrome and support the dietary link of this naturally occurring thiamin deficiency to the thiaminase-rich alewife.
Asunto(s)
Dieta/efectos adversos , Óvulo/química , Salmo salar/embriología , Deficiencia de Tiamina/veterinaria , Tiamina/análisis , Animales , Embrión no Mamífero/química , Femenino , Peces , Agua Dulce , Hidrolasas/efectos adversos , Hidrolasas/análisis , Masculino , New York , Tiamina/sangre , Tiamina/metabolismo , Deficiencia de Tiamina/etiología , Deficiencia de Tiamina/mortalidadRESUMEN
Wernicke's encephalopathy, a disorder with significant mortality and high morbidity, is common amongst alcohol-dependent patients. Thiamine deficiency appears to play a key role in its aetiology, and parenteral high-dose thiamine is effective in prophylaxis and treatment. Unfortunately, reports of rare anaphylactoid reactions have led to a dramatic reduction in the use of parenteral thiamine, and it is possible that this change in treatment has led, or will lead, to an increase in morbidity and mortality. There is a need for education of doctors who treat alcohol-dependent patients, in order to ensure appropriate use of parenteral thiamine in prophylaxis and treatment of this disorder.