Severe lactic acidosis in an extremely low birth weight infant due to thiamine deficiency.

BACKGROUND: In this case report, we present a preterm newborn with persistent lactic acidosis who received total parenteral nutrition (TPN) that lacked thiamine. CASE PRESENTATION: A 28-week-old, 750 g female infant was born with an Apgar score of 8 at the 5th minute. Umbilical cord blood gas levels, including lactate level, were normal, and she was admitted to our neonatal intensive care unit (NICU). Achieving full enteral feeding was not possible due to gastric residues and abdominal distention, making the patient dependent on TPN during the first 2 weeks of life. An insidious increase in lactic acid levels and uncompensated metabolic acidosis were apparent from the 23rd day of life. Severe metabolic acidosis was persistent despite massive doses of bicarbonate. The acidosis resolved dramatically within 6 h when the patient was administered with thiamine. CONCLUSIONS: Although TPN is life saving in the NICU, meticulous attention must be paid to provide all essential macro- and micro-nutrients.

Thiamine content of F-75 therapeutic milk for complicated severe acute malnutrition: time for a change?

Since community-based management of severe acute malnutrition has become the standard of care, the clinical profile of severe acutely malnourished patients admitted to hospitals or inpatient therapeutic feeding centers has changed significantly. These patients are usually very ill and often present with several comorbidities, such as shock, sepsis, and pneumonia. Complicated severe acute malnutrition patients are at risk of thiamine insufficiency, and critically ill patients have higher thiamine requirements. The thiamine content of F-75, the therapeutic milk formula used in the early stabilization phase of refeeding in patients with severe acute malnutrition, seems insufficient. Here, we discuss the need and rationale for a substantial increase in the thiamine content of F-75.

Effects of thiamine deficiency on food intake and body weight increment in adult female and growing rats.

The present study compared the effects of thiamine (vitamin B1) deficiency (TD) on the patterns of food intake and body weight in adult female and neonatal Wistar rats. The adults weighed 250-270 g at the start and were fed for 60 days either with a synthetic TD diet (211 B1) or with the same synthetic diet+thiamine (210 B1). TD led to a marked reduction in food intake and the body weight set point, both recovering rapidly to their initial level in only 3 days after dietetic reversion. The effects of TD in developing rats were evaluated by subjecting pregnant rats to thiamine restriction during different time windows: prenatal (3 days before mating to parturition); perinatal (7 days after mating to the 10th postnatal day); and postnatal (from parturition to weaning). The effect of TD on the occurrence of low birth weight and ponderal growth retardation was examined from postnatal days 1 to 45. Only perinatal TD significantly decreased birth weight relative to untreated or pair-fed controls. Moreover, compared with the control treatments, ponderal growth retardation was not induced by prenatal TD, whereas induction of TD from perinatal into postnatal periods did cause ponderal growth retardation, with long-lasting effects persisting in adulthood. The results suggest a major physiological role of thiamine in the homeostasis of body weight programming, increment, and set point regulation in both offspring and adult female rats.

Thiamine in nutrition therapy.

Clinicians involved with nutrition therapy traditionally concentrated on macronutrients and have generally neglected the importance of micronutrients, both vitamins and trace elements. Micronutrients, which work in unison, are important for fundamental biological processes and enzymatic reactions, and deficiencies may lead to disastrous consequences. This review concentrates on vitamin B(1), or thiamine. Alcoholism is not the only risk factor for thiamine deficiency, and thiamine deficiency is often not suspected in seemingly well-nourished or even overnourished patients. Deficiency of thiamine has historically been described as beriberi but may often be seen in current-day practice, manifesting as neurologic abnormalities, mental changes, congestive heart failure, unexplained metabolic acidosis, and so on. This review explains the importance of thiamine in nutrition therapy and offers practical tips on prevention and management of deficiency states.

Thiamine deficiency in diabetes mellitus and the impact of thiamine replacement on glucose metabolism and vascular disease.

Despite the targeting of traditional risk factors for cardiovascular disease, disease burden has not been completely eliminated. Thiamine is an essential cofactor in carbohydrate metabolism and individuals with diabetes are thiamine deficient. The pathophysiology of recognised complications of thiamine deficiency is similar to that underlying atherosclerosis and the metabolic syndrome, namely oxidative stress, inflammation and endothelial dysfunction. This review examines the mechanisms by which thiamine deficiency occurs in individuals with diabetes, how this deficiency leads to hyperglycaemic-induced damage, and the effect of thiamine replacement on vascular disease, endothelial function and oxidative stress. Thiamine administration can prevent the formation of harmful by-products of glucose metabolism, reduce oxidative stress and improve endothelial function. The potential benefit of long-term replacement in those with diabetes is not yet known but may reduce cardiovascular risk and angiopathic complications.

Valoración del estatus de tiamina en un colectivo andaluz y su relación con otros parámetros / Thiamine status assessment in an andalusian collective. association with other factors

INTRODUCCIÓN: Las vitaminas del grupo B (piridoxina, riboflavina, cianocobalamina, acido fólico,etc.) se relacionan estrechamente como componentes de coenzimas que intervienen en procesos de obtención de energía para llevar a cabo reacciones metabólicas básicas para el organismo como elciclo de Krebs, el ciclo de pentosa-P, etc... La vitamina B1 es, en ocasiones, desestimada de la lista de sustancias valoradas en una determinada población, por descartar la posible deficiencia o alteración en el estatus de dicha vitamina. Las recomendaciones de ingesta de vitamina B1 en la población adulta son de 1,1 – 1,2 mg/día1. La carencia severa de tiamina se asocia con problemas neurológicos y cardíacos mientras que un déficit marginal presenta síntomas y signos comunes a otras enfermedades funcionales que podrían enmascararlo. Estudios anteriores muestran deficiencias de tiamina en un 8 %aproximadamente de la población andaluza estudiada2. OBJETIVO: El presente estudio valora el estado nutricional de la tiamina en una población adulta sana y establece posibles relaciones entre parámetros de ingesta y bioquímicos, y un estatus deficiente de vitamina B1(AU)

METODOLOGÍA: El estudio se realizó en un colectivo de personas con edades entre 21 y 59 años, 56 hombres y 61 mujeres (n=117). Los criterios de inclusión se basaron en la aceptación de los sujetos a participar en el estudio y en que dichos sujetos no presentaran algún tipo de patología que pudiera afectar su situación nutricional. La vitamina B1 ha sido determinada mediante técnica de estimulación coenzimática de enzimas dependientes de esta vitamina en eritrocito 3. Una leve respuesta a la estimulación coenzimática indica la existencia de una alta saturaci6n enzimática en dicho coenzima. Para determinar la ingesta de la vitamina B1 se aplicó un cuestionario de frecuencia de consumo, y mediante programa informático Nutriber 4, se obtuvo el % de RDA. Se contó con la aceptación del Comité ético y el consentimiento informado. RESULTADOS: Los resultados obtenidos muestran una ingesta menor a la recomendada en un 31,6% de la población estudiada. Por otro lado, los resultados obtenidos en los valores de actividad coenzimática para la tiamina muestran una deficiencia del 39 %. Se encontró correlación significativa positiva entre ingesta de vitamina B1 y la ingesta de vitaminas B2, B6, ácido fólico y B12 dado que los alimentos con alto contenido en ellas las contienen en su conjunto. También existe correlación significativa positiva entre ingesta de tiamina y la intensidad de ejercicio. CONCLUSIÓN: La deficiencia de la tiamina es mas frecuente de lo esperado en la población, debido a la ingesta desequilibrada y a hábitos de consumo inadecuados, estando asociada a la deficiencia del resto de las vitaminas (complejo B). Por tanto, es aconsejable el control del estatus en vitaminas B debido a las alteraciones en las funciones celulares que se producen y a las consecuencias graves en las que puede derivar(AU)

INTRODUCTION: The B group vitamins (pyridoxine, riboflavin, cyanocobalamin, folic acid, etc.) are closely related as components of coenzymes involved in energy production processes to perform basic metabolic reactions to the body such as the Krebs cycle The pentose-P cycle, etc ... Vitamin B1 is sometimes dismissed from the list of substances evaluated in a given population, to discard the possible deficiency or alteration in the status of the vitamin. The recommended intake of vitamin B1 in the adult population is 1.1 - 1.2 mg / day1. Severe thiamin deficiency is associated with neurological and cardiac problems while a marginal deficit symptoms and signs common to other diseases that could mask functional. Previous studies show deficiencies of thiamine in about 8% of the population still being studied2. OBJECTIVE: This study assesses the nutritional status of thiamine in a healthy adult population and establishes possible relationships between intake and biochemical parameters, and a deficient status of vitamin B1(AU)

METHODS: The study was conducted in a group of people aged between 21 and 59 years, 56 menand 61 women (n = 117). Inclusion criteria were based on the acceptance of subjects to participate in the study and that these subjects did not display any pathology that could affect their nutritional status. Vitamin B1 has been established by coenzyme stimulation technique of the vitamin-dependent enzymes in erythrocyte 3. A mild response to stimulation coenzyme indicates the existence of a highenzyme saturacion the coenzyme. To determine the intake of vitamin B1 was applied food frequency questionnaire, and through software Nutriber (Mataix, and Garcia Diz, 2006)2, we obtained the% of RDA. It was accepted by the Ethics Committee and informed consent. CONCLUSION: Thiamine deficiency is more common than expected in the population, due to unbalanced eating habits and inadequate, being associated with deficiency of other vitamins (Bcomplex). It is therefore advisable to control the status of vitamins B due to alterations in cell functions that occur and the serious consequences which may result(AU)

Síndrome de realimentación / Refeeding syndrome

El síndrome de realimentación (SR) es un cuadro clínico complejo que ocurre como consecuencia de la reintroducción de la nutrición (oral, enteral o parenteral) en pacientes malnutridos. Los pacientes presentan trastornos en el balance de fl uidos, anomalías electrolíticas —como hipofosfatemia, hipopotasemia e hipomagnesemia— alteraciones en el metabolismo hidrocarbonado y déficits vitamínicos. Esto se traduce en la aparición de complicaciones neurológicas, respiratorias, cardíacas, neuromusculares y hematológicas. En este artículo se han revisado la patogenia y las características clínicas del SR, haciendo alguna sugerencia para su prevención y tratamiento. Lo más importante en la prevención del SR es identificar a los pacientes en riesgo, instaurar el soporte nutricional de forma prudente y realizar una corrección adecuada de los déficits de electrolitos y vitaminas (AU)

Refeeding syndrome is a complex syndrome that occurs as a result of reintroducing nutrition (oral, enteral or parenteral) to patients who are starved or malnourished. Patients can develop fluid-balance abnormalities, electrolyte disorders (hypophosphataemia, hypokalaemia and hypomagnesaemia), abnormal glucose metabolism and certain vitamin defi ciencies. Refeeding syndrome encompasses abnormalities affecting multiple organ systems, including neurological, pulmonary, cardiac, neuromuscular and haematological functions. Pathogenic mechanisms involved in the refeeding syndrome and clinical manifestations have been reviewed. We provide suggestions for the prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk, reintroduce nutrition cautiously and correct electrolyte and vitamin defi ciencies properly (AU)

Restoration of thiamine status with white or whole wheat bread in a thiamine-depleted rat model.

Long-term thiamine deficiency has been largely documented, whilst little is known about effects of short-term depletion/repletion periods on thiamine vitamers status. Rats were submitted to short-term depletion (8 days) followed by different durations of repletion (3 or 14 days) with thiamine from bread (whole wheat bread or white bread, whole B and white B respectively) or corresponding controls. Short-term depletion drastically decreased plasma thiamine (-97%) and its urinary excretion (-77%). TDP (thiamine diphosphate) was strongly affected in liver (-67%) but less affected in cerebellum (-38%) or kidneys (-45%). Short-term repletion (3 days) with whole B diet or its control restored TDP at initial values in cerebellum and kidneys. A longer repletion (14 days) was required to restore liver TDP. Comparison of the diet groups indicates that thiamine status in tissues of rat fed whole B or white B diet was comparable to that of rats fed purified thiamine. Plasma thiamine concentration could not be restored at initial values in the bread groups or respective controls. In conclusion, thiamine in whole wheat bread appears effective in preventing marginal deficiencies and plasma thiamine is a less reliable indicator of thiamine status than tissue TDP levels.

Pacientes con riesgo de desnutrición: valoración de 11 casos gravemente desnutridos con nutrición parenteral total individualizada / No disponible

nutricional individualizado durante la primera semana con nutriciónparenteral total en pacientes con desnutrición moderada-gravesusceptibles de desarrollar un síndrome de renutrición.Método: Estudio retrospectivo observacional desde enero2003 a junio 2006 incluyendo todos los pacientes adultos condesnutrición moderada-grave que recibieron >= 5 días de nutriciónparenteral total. Se describió el soporte nutricional y se evaluó laaparición de alteraciones hidroelectrolíticas y metabólicas gravesdurante la primera semana de nutrición.Resultados: Se incluyeron 11 pacientes con índice de masacorporal medio de 15,4 kg/m2 que recibieron una media de 23kcal/kg/día. No aparecieron alteraciones hidroelectrolíticas ometabólicas graves. Tres pacientes presentaron hipofosfatemia, 5hipopotasemia y 4 hipomagnesemia, todas leves-moderadas ycorregidas, excepto en dos casos, a la semana de nutrición.Conclusiones: El soporte nutricional individualizado en pacientescon desnutrición moderada-grave no produjo ningún síndromede renutrición. La indivualización de la nutrición es una estrategiaesencial para evitar las complicaciones de una sobrealimentación

Objective: To describe and assess the efficacy and safety ofindividualised nutritional support during the first week of total parenteralnutrition in moderately to severely malnutritioned patientswho are susceptible to the refeeding syndrome.Method: Retrospective observational study carried outbetween January 2003 and June 2006, including adult patientswith moderate to severe malnutrition who received >= 5 days totalparenteral nutrition. The nutritional support was described andthe appearance of severe hydroelectrolytic and metabolic disturbanceswere assessed during the first week of nutrition.Results: The study included 11 patients with a mean bodymass index of 15.4 kg/m2. These patients received an average of23 Kcal/kg/day. They did not show any signs of severe hydroelectrolyticor metabolic disturbances. Three patients presentedwith hypophosphataemia, five with hypokalaemia and four withhypomagnesaemia, all of which were mild to moderate and withthe exception of two cases, all were corrected within one week offeeding.Conclusions: Individualised nutritional support in moderatelyto severely malnourished patients does not produce refeeding syndrome.Individualised nutrition is an essential strategy for avoidingcomplications associated with overfeeding

Thiamine (vitamin B1) deficiency and associated brain damage is still common throughout the world and prevention is simple and safe!

Many different population groups throughout the world have thiamine deficiency and are at risk of developing severe neurological and cardiac disorders. Alcoholics are most at risk but other important clinical groups should be monitored carefully. The most severe, potentially fatal disease caused by thiamine deficiency is the neurological disorder Wernicke-Korsakoff syndrome. This can be difficult to diagnose and many cases remain undiagnosed. Treatment with thiamine generally results in a dramatic clinical improvement. Thiamine supplementation of stable food products like flour is an effective, simple and safe public health measure that can improve the thiamine status of all population groups.

Diuretic use: a risk for subclinical thiamine deficiency in elderly patients.

Long term diuretic therapy represents one central pharmacologic therapy of heart insufficiency and hypertension. Diuretics lead not only to an increased urinary excretion of electrolytes but also of water soluble vitamins. In this prospective study we evaluated the effect of hospitalization on the overall biochemical vitamin status in subjects older than 50 years (n=149, mean +/- SD age 70 +/- 10 years). Vitamin nutriture and other parameters were assessed at admission and discharge (duration of the hospitalization 19 +/- 1 day). Only vitamin B1 nutriture worsened during the hospitalization and in a multivariate procedure the only significant predictor of the change in the vitamin B1 nutriture was the use of diuretics during the hospitalization (F=4.06, p < 0.001). The changes in the ETK (erythrocyte transketolase activity in whole blood) and a-ETK (ETK activity coefficient) during the hospital stay correlated with the cumulative dosage of furosemide adjusted for the duration of the therapy (r = 0.36, p < 0.001 and r = - 0.28, p > 0.03). Our data suggest that hospitalized elderly are at increased risk for vitamin B1 deficiency especially when on a diuretic treatment. It is possible that a low dose thiamine supplementation my help to prevent the development of a subclinical wet-beriberi in older subjects on diuretics.

Wernicke-Korsakoff syndrome in Sydney hospitals after 6 years of thiamin enrichment of bread.

OBJECTIVES: To estimate the incidence of Wernicke's encephalopathy (WE) and Korsakoff's psychosis (KP) before and after the introduction of thiamin enrichment of bread in Australia. DESIGN AND SUBJECTS: Inpatient records were examined in 17 major public general hospitals in greater Sydney for the 4 years 1993-96 (inclusive) with the International Classification of Diseases (ICD) 9 diagnoses 265.1 (WE), 291.1 and 294.0 (KP). Relevant clinical data were recorded on a specially designed form so that cases could be classified as confirmed or probable WE, confirmed or probable KP, confirmed or probable Wernicke-Korsakoff syndrome (WE + KP) or not WE or KP. The average number of cases of WE + KP was 38 acute (new) cases and 69 total (acute + chronic) cases per annum for all the hospitals combined. RESULTS: This study used the same methods as our earlier retrospective examination of Wernicke-Korsakoff cases in essentially the same hospitals for 1978-93. Records for 1993 were thus pulled twice and, while individual cases (using hospital index number) did not always coincide, the total numbers for 1993 were 69 and 70. For the 5 years after 1991 the number of acute cases of WE and KP in Sydney hospitals was 61% of the number for the 5 years before 1991 (P<0.01). There is, however, no continuing downward trend. CONCLUSIONS: These results are consistent with a 40% reduction of the incidence of acute WE and KP since bread has been enriched with thiamin. The disease complex has, however, not been eliminated. To achieve this, further public health action would be needed, such as addition of thiamin to beer.

Down's syndrome: nutritional intervention.

Down's Syndrome patients are known to be of short stature, prone to infections, autoimmune disease, hypothyroidism, leukaemia, heart defects and later Alzheimer's disease. They tend to have older mothers, like Alzheimer's disease patients. The latter tend to have sibs with either Down's Syndrome or lymphoma/leukaemia. Evidence, looking at 28 Down's Syndrome patients, suggests that multiple food allergies, gluten-gliadin sensitivity or intolerance are causing a coeliac disease-like picture with a malabsorption state for essential vitamins, minerals and severe autoimmune disease. It is hoped that missed gluten-gliadin sensitivity or intolerance with or without coeliac disease will be considered as a cause of abnormal oogenesis and spermatogenesis resulting in trisomy 21 and other aneuploidies. The mechanism most likely is low B1 interfering with sufficient release of cAMP for normal meiosis. Alternatively exorphins and peptides from foods may suppress prostaglandin E1 synthesis, or food sensitivities may alter toxic metal absorption mechanisms, which are thought to play a role in the development of Alzheimer's disease. Adequate vitamin/mineral supplementation, especially B1, prior to conception and in the first trimester is recommended for mothers at risk for DS, especially older mothers and a gluten free diet for those with coeliac disease or gluten-gliadin sensitivity/intolerance. Hopefully this will prevent conception of a DS child, or prevent heart defects/stigmata if one is conceived. DS children should be investigated for the above and commence a food allergy free diet with relevant supplements to meet their needs as early as maximum development.

Role of cofactors in the treatment of malnutrition as examplified by magnesium.

In the absence of appropriate amounts of metabolically important cofactors such as magnesium, replenishment of malnourished patients with protein and carbohydrate will exaggerate the underlying abnormality even though the primary deficiency is corrected. The malnourished patients cannot utilize the food substances provided unless they have within their cells commensurate amounts of all the necessary cofactors required for the metabolism of the food supplied. This therapeutic problem in malnutrition is illustrated by three different examples of clinical deterioration when caloric and vitamin replenishment have been undertaken in the face of magnesium deificiency.