Comparação dos casos de beribéri entre indígenas e não indígenas, Brasil, 2013 a 2018 / Comparison of beriberi cases in indigenous and non-indigenous people, Brazil, 2013 to 2018

Resumo O beribéri é a manifestação clínica da deficiência grave e prolongada de tiamina (vitamina B1). Doença negligenciada que acomete a população de baixa renda, em situação de insegurança alimentar e nutricional. O objetivo do estudo foi comparar casos de beribéri em indígenas com casos em não indígenas no Brasil. Trata-se de estudo transversal de casos notificados de beribéri no período de 2013 a 2018, no formulário do SUS (FormSUS) do Ministério da Saúde. Foram comparados os casos em indígenas e em não indígenas pelo teste qui-quadrado ou teste exato de Fisher com nível de significância de p < 0,05. No período estudado foram notificados no país 414 casos de beribéri, sendo 210 (50,7%) indígenas. Referiram consumo de bebidas alcoólicas 58,1% dos indígenas e 71,6% dos não-indígenas (p = 0,004); adicionalmente, 71,0% dos indígenas consumiam caxiri (bebida alcoólica tradicional fermentada). Relataram fazer esforço físico diário 76,1% dos indígenas e 40,2% dos não-indígenas (p <0 ,001). Conclui-se que o beribéri no país acomete mais indígenas e está relacionado ao consumo de álcool e ao esforço físico.

Abstract Beriberi is the clinical manifestation of severe and prolonged thiamine (vitamin B1) deficiency. It is a neglected disease that affects low-income populations facing food and nutrition insecurity. The aim of this study was to compare cases of beriberi among indigenous and non-indigenous people in Brazil. We conducted a cross-sectional study using data on cases of beriberi during the period July 2013-September 2018 derived from beriberi notification forms available on the FormSUS platform. Cases in indigenous and non-indigenous patients were compared using the chi-squared test or Fisher's exact test, adopting a significance level of 0.05. A total of 414 cases of beriberi were reported in the country during the study period, 210 of which (50.7%) were among indigenous people. Alcohol consumption was reported by 58.1% of the indigenous patients and 71.6% of the non-indigenous patients (p = 0.004); 71.0% of the indigenous patients reported that they consumed caxiri, a traditional alcoholic drink. Daily physical exertion was reported by 76.1% of the indigenous patients and 40.2% of the non-indigenous patients (p < 0.001). It is concluded that beriberi disproportionately affects indigenous people and is associated with alcohol consumption and physical exertion.

Efficacy of thiamine supplementation in improving depressive symptoms-evidence-based review / Eficacia de la suplementación con tiamina para mejorar los síntomas depresivos-revisión basada en la evidencia

Objetivos: Revisar la evidencia existente con respecto a la efectividad de la suplementación con tiamina en pacientes consíntomas depresivos en cuanto a su mejoría, en comparación con la toma de fármacos psicotrópicos aislados o placebos. Diseño: Revisión sistemática; Emplazamiento: Atención primaria. Método: Pregunta de investigación: Población – Adultos con síntomas depresivos; Intervención – Suplementación contiamina aislada o asociada a psicofármacos; Comparación: uso de placebo o fármacos psicotrópicos; Resultado – Mejoría delos síntomas depresivos (según DSM-V). La búsqueda se realizó utilizando los términos MeSH (("Depression" OR"Depression Disorder") AND "Thiamine"”) en las fuentes seleccionadas. Se excluyeron todos los artículos de opinión,artículos de revisión clásicos y estudios ya incluidos en metanálisis o revisiones sistemáticas seleccionadas. Se utilizó laescala Strength of Recommendation Taxonomy (SORT) para asignar niveles de evidencia y fuerzas de recomendación.Resultados: La investigación resultó en 3 artículos que cumplieron con los criterios de inclusión y de los cuales se extrajeronlas conclusiones.Conclusiones: Se demostraron beneficios estadísticamente significativos de la suplementación con tiamina en algunos delos parámetros evaluados. Sin embargo, a la suplementación con tiamina se le asignó una Fuerza de Recomendación B porla Escala SORT, ya que los estudios existentes de calidad moderada son pocos, aunque orientados al paciente, y no handemostrado ser suficientes o consistentes para recomendar la generalización del uso de tiamina. Se necesitan más ensayosclínicos controlados de buena calidad científica para responder adecuadamente a esta pregunta de investigación.(AU)

Objective: to review the existing evidence regarding the effectiveness of thiamine supplementation in improving depressivesymptoms in adult patients, compared to taking psychoactive drugs alone or placebo. Design: Systematic ReviewSite: Primary Care. Methods: Research question: Population – Adults with depressive symptoms; Intervention – Thiamine supplementationalone or associated with psychotropic drugs; Comparison: use of placebo or psychotropic drugs; Outcome – Improvementof depressive symptoms (according to DSM-V). The search was performed using the MeSH terms (("Depression" OR"Depression Disorder") AND "Thiamine"") in the selected sources. All opinion articles, classic review articles, and studiesalready included in meta-analyses or previously selected reviews were excluded. The strength of recommendationtaxonomy scale (SORT) was used to assign levels of evidence and strength of recommendation.Results: The search resulted in 3 articles that met the inclusion criteria, from which conclusions were drawn. Conclusions: Statistically significant benefits of thiamine supplementation were demonstrated in some of the evaluatedparameters. However, thiamine supplementation was assigned a Strength of Recommendation B by the SORT Scale, asexisting studies of moderate quality are few, albeit patient oriented, not proving to be sufficient or consistent torecommend the generalization of the use of thiamine. More controlled clinical trials of good scientific quality are needed toadequately answer this research question.(AU)

Vitamina C, tiamina e hidrocortisona: ¿aliados en shock séptico? / Vitamin C, thiamine and hydrocortisone: allies against septic shock?

Objetivo: Determinar la efectividad del uso de un protocolo de vitamina C, tiamina e hidrocortisona en shock séptico a las dosis empleadas en el estudio Marik. Método: Estudio observacional retrospectivo antes-después que compara dos grupos de pacientes con shock séptico: grupo tratamiento (GT), tratado con el protocolo, desde enero 2019 a mayo 2020, frente a un grupo control (GC) en un periodo previo. Los objetivos principales fueron la supervivencia a los 14 y a los 28 días. Resultados: Se incluyeron 91 pacientes, 44 en GC y 47 en GT. No se encontraron diferencias estadísticamente significativas en la supervivencia a los 14 ni a los 28 días ni en la duración de estancia hospitalaria ni en la Unidad de Reanimación (REA) (p>0,05). La variación de los valores de creatinina entre los días 1 y 4 presentó una mediana de -0,04 (RIC -0,24;0,8) en el GC frente a -0,3 del GT (RIC -0,079;-0,03), p<0,05. Los valores de procalcitonina descendieron en el 27,3% de los pacientes del GC frente al 91,5% del GT (p<0,05). La necesidad de soporte vasoactivo con noradrenalina fue del 93% GC vs 76,6% GT en el día 2; 75% GC vs 55,3% GT en el día 3; 50% GC vs 27,7% GT en el día 4 (p<0,05). Conclusiones: Los datos de nuestro estudio muestran un beneficio limitado del uso del protocolo en pacientes con shock séptico, pero son necesarios estudios de mayor reclutamiento con un diseño prospectivo randomizado para asegurar el beneficio real y/o dosis óptimas. (AU)

Objective: To determine the effectiveness of using a protocol of vitamin C, thiamine and hydrocortisone for the treatment of septic shock at the doses used in the Marik study. Methods: Retrospective observational before-after study comparing two groups of patients with septic shock: treatment group (GT), treated with the protocol, between January 2019 and May 2020, versus a control group (GC) in a previous period. The primary outcomes were 14-Day and 28-Day Survival. Results: 91 patients were included, 44 in GC and 47 in GT. No statistically significant differences were found in 14-day and 28-day survival, in length of hospital or Resuscitation Unit (REA) stay (p>0.05). The median creatinine variation between days 1 and 4 was -0.04 (IQR -0.24;0.8) in the GC vs -0.3 in the GT (IQR -0.079; -0.03) (p<0.05). Procalcitonin values decreased in 27.3% of GC versus 91.5% of GT (p<0.05). The need for vasoactive drugs (noradrenaline) was 93% in GC vs 76.6% GT on day 2; 75% GC vs 55.3% GT on day 3; 50% GC vs 27.7% GT on day 4 (p<0.05). Conclusions: Our results show a limited benefit of the use of the protocol in patients with septic shock, but larger recruitment studies with a prospective randomized design are needed to ensure real benefit and/or optimal doses. (AU)

Vitamin C and thiamine for the treatment of refractory septic shock in surgical critically ill patients: a retrospective before-and-after study / Vitamina C y tiamina para el tratamiento del shock séptico refractario en pacientes críticos quirúrgicos: un estudio retrospectivo antes-después

Introduction: This study aimed to evaluate whether early vitamin C and thiamine administration was associated with a lower 28-day and in-hospital mortality in surgical critically ill patients with refractory septic shock. Patients and methods: We performed a retrospective before-and-after study on patients with refractory septic shock. According to local protocol, hydrocortisone is initiated in case of refractory septic shock. In January 2017, the protocol was changed and vitamin C and thiamine were included. Patients who were admitted in 2015-2016 and 2017-2018 were included in the control and treatment groups, respectively. The primary end point was 28-day and in-hospital mortality. Secondary end points were ICU mortality, ICU and hospital length of stay, duration of vasopressors and mechanical ventilation, use of renal replacement therapy (RRT), and the modification in serum procalcitonin and SOFA score during the first 72 h. Results: A total of 120 patients were included (58 in the treatment group and 62 in the control group). Log-rank test in Kaplan-Meier curves showed lower 28-day and in-hospital mortality over time in the treatment group (p=0.021 and p=0.035, respectively) but it not reached statistical significance in ICU mortality over time (p=0.100). The need of RRT was less frequent in treatment group (17.2% vs. 37.1%, p=0.024). There were no differences in other secondary outcomes. Conclusions: Intravenous vitamin C and thiamine administration in surgical patients with refractory septic shock may be associated with a lower 28-day and in-hospital mortality. Further prospective studies are needed in refractory septic shock. (AU)

Introducción: El objetivo de este estudio fue evaluar si la administración precoz de vitamina C y tiamina estaba asociada a una reducción en la mortalidad a los 28 días y hospitalaria en pacientes críticos quirúrgicos con shock séptico refractario. Pacientes y métodos: Realizamos un estudio retrospectivo antes-después en pacientes con shock séptico refractario. Según el protocolo local, se inicia tratamiento con hidrocortisona en situación de shock séptico refractario. En enero de 2017 se cambió el protocolo y se incluyó vitamina C y tiamina. Los pacientes que fueron ingresados en 2015-2016 y 2017-2018 se incluyeron en el grupo control y tratamiento, respectivamente. Los objetivos primarios fueron la mortalidad a los 28 días y hospitalaria. Los objetivos secundarios fueron la mortalidad en UCI, la duración de estancia en UCI y hospitalaria, la duración del tratamiento vasopresor y de la ventilación mecánica, el uso de técnicas de reemplazo renal (TRR), y la modificación en la procalcitonina sérica y la puntuación SOFA durante las primeras 72h. Resultados: Se incluyeron un total de 120 pacientes (58 en el grupo tratamiento y 62 en el grupo control). El test Log-rank en las curvas de Kaplan-Meier mostró mortalidad a los 28 días y hospitalaria más baja a lo largo del tiempo en el grupo tratamiento (p=0,021 and p=0,035, respectivamente) pero no alcanzó significación estadística en la mortalidad en UCI a lo largo del tiempo (p=0,100). La necesidad de TRR fue menos frecuente en el grupo tratamiento (17,2% vs. 37,1%, p=0,024). No hubo diferencias en otros resultados secundarios. Conclusiones: La administración de vitamina C y tiamina intravenosa en pacientes quirúrgicos con shock séptico refractario podría estar asociada a una menor mortalidad a los 28 días y hospitalaria. Se necesitan más estudios prospectivos en pacientes con shock séptico refractario. (AU)

Furosemide-related thiamine deficiency in hospitalized hypervolemic patients with renal failure and heart failure / Deficiencia de tiamina relacionada con la furosemida en pacientes hipervolémicos con insuficiencia renal e insuficiencia cardíaca

Background: We aimed to describe the thiamine status in hospitalized hypervolemic heart failure (HF) and/or renal failure (RF) patients treated with furosemide and to investigate whether there was a difference in furosemide-related thiamine deficiency between patients with RF and HF. Methods: Patients who were diagnosed as hypervolemia and treated with intravenous furosemide (at least 40mg/day) were included in this prospective observational study. Whole blood thiamine concentrations were measured 3 times during hospital follow-up of patients. Results: We evaluated 61 hospitalized hypervolemic patients, of which 22 (36%) were men and 39 (64%) were women, with a mean age of 69.00±10.39 (45–90) years. The baseline and post–hospital admission days 2 and 4 mean thiamine levels were 51.71±20.66ng/ml, 47.64±15.43ng/ml and 43.78±16.20ng/ml, respectively. Thiamine levels of the hypervolemic patients decreased significantly during the hospital stay while furosemide treatment was continuing (p=0.029). There was a significant decrease in thiamine levels in patients who had HF (p=0.026) and also, thiamine was significantly lower in HF patients who had previously used oral furosemide before hospitalization. However, these findings were not present in patients with RF. (AU)

Antecedentes: Nos propusimos describir el estado de la tiamina en pacientes hospitalizados con insuficiencia cardíaca (IC) y/o insuficiencia renal (IR) hipervolémica tratados con furosemida, e investigar si había una diferencia en la deficiencia de tiamina relacionada con la furosemida entre los pacientes con IR y con IC. Métodos: En este estudio observacional prospectivo se incluyeron pacientes con diagnóstico de hipervolemia y tratados con furosemida intravenosa (al menos 40 mg/día). Se midieron las concentraciones de tiamina en sangre total 3 veces durante el seguimiento hospitalario de los pacientes. Resultados: Se evaluaron 61 pacientes hipervolémicos hospitalizados, de los cuales 22 (36%) eran hombres y 39 (64%) eran mujeres, con una edad media de 69,00±10,39 (45-90) años. Los niveles medios de tiamina en la línea de base y tras el ingreso hospitalario en los días 2 y 4 fueron de 51,71±20,66 ng/ml, 47,64±15,43 ng/ml y 43,78±16,20 ng/ml, respectivamente. Los niveles de tiamina de los pacientes hipervolémicos hipervolemia disminuyeron significativamente durante la estancia en el hospital mientras se mantenía el tratamiento con furosemida furosemida (p = 0,029). Hubo una disminución significativa de los niveles de tiamina en los pacientes que tenían IC (p = 0,026) y también, la tiamina fue significativamente menor en los pacientes con IC que habían habían utilizado previamente furosemida oral antes de la hospitalización. Sin embargo, estos hallazgos no se presentes en los pacientes con IC. (AU)

Furosemide-related thiamine deficiency in hospitalized hypervolemic patients with renal failure and heart failure.

BACKGROUND: We aimed to describe the thiamine status in hospitalized hypervolemic heart failure (HF) and/or renal failure (RF) patients treated with furosemide and to investigate whether there was a difference in furosemide-related thiamine deficiency between patients with RF and HF. METHODS: Patients who were diagnosed as hypervolemia and treated with intravenous furosemide (at least 40mg/day) were included in this prospective observational study. Whole blood thiamine concentrations were measured 3 times during hospital follow-up of patients. RESULTS: We evaluated 61 hospitalized hypervolemic patients, of which 22 (36%) were men and 39 (64%) were women, with a mean age of 69.00±10.39 (45-90) years. The baseline and post-hospital admission days 2 and 4 mean thiamine levels were 51.71±20.66ng/ml, 47.64±15.43ng/ml and 43.78±16.20ng/ml, respectively. Thiamine levels of the hypervolemic patients decreased significantly during the hospital stay while furosemide treatment was continuing (p=0.029). There was a significant decrease in thiamine levels in patients who had HF (p=0.026) and also, thiamine was significantly lower in HF patients who had previously used oral furosemide before hospitalization. However, these findings were not present in patients with RF. CONCLUSIONS: Thiamine substantially decreases in most hypervolemic patients receiving intravenous furosemide treatment during the hospital stay. Thiamine levels were significantly decreased with furosemide treatment in especially HF patients, but the decrease in thiamine levels did not detected at the same rate in RF patients. Diuretic-induced thiamine loss may be less likely in RF patients, probably due to a reduction in excretion.

Síndrome de Wernicke-Korsakoff y otras patologías asociadas al déficit de tiamina / Wernicke-Korsakoff syndrome and other diseases associated with thyamine deficiency

El síndrome de Wernicke-Korsakoff es la consecuencia más conocida del déficit de tiamina; se asocia frecuentemente a pacientes con un consumo crónico y excesivo de alcohol, pero puede deberse a cualquier causa que produzca déficit de tiamina.La enfermedad está infradiagnosticada, por lo que es fundamental tener una alta sospecha clínica, principalmente en los pacientes que no presentan consumo de alcohol como factor de riesgo. El diagnóstico sigue siendo eminentemente clínico, con la dificultad de una elevada variabilidad clínica. Las pruebas complementarias sirven para apoyar el diagnóstico y descartar otras causas que puedan producir sintomatología similar, siendo la resonancia magnética la prueba de imagen más rentable.El tratamiento se basa en la administración de tiamina, que debe iniciarse precozmente, de forma parenteral y a las dosis adecuadas en todos los pacientes con clínica compatible, sin esperar a confirmar el diagnóstico. (AU)

Wernicke-Korsakoff syndrome is the best known consequence of thiamine deficiency, frequently associated with patients with chronic and excessive alcohol consumption, but it can be produced by any cause that produces thiamine deficiency.The disease is underdiagnosed so it is essential to have a high clinical suspicion, mainly in patients who do not have alcohol consumption as a risk factor. For this, the diagnosis continues to be eminently clinical, with the difficulty of high clinical variability. Complementary tests are used to support the diagnosis and rule out other causes that can produce similar symptoms, with magnetic resonance imaging being the most cost-effective imaging test.Treatment is based on the administration of thiamine, which should be started early, and parenterally at the appropriate doses, in all patients with compatible symptoms, without waiting to confirm the diagnosis. (AU)

[Wernicke-Korsakoff syndrome and other diseases associated with thyamine deficiency]. / Síndrome de Wernicke-Korsakoff y otras patologías asociadas al déficit de tiamina.

Wernicke-Korsakoff syndrome is the best known consequence of thiamine deficiency, frequently associated with patients with chronic and excessive alcohol consumption, but it can be produced by any cause that produces thiamine deficiency. The disease is underdiagnosed so it is essential to have a high clinical suspicion, mainly in patients who do not have alcohol consumption as a risk factor. For this, the diagnosis continues to be eminently clinical, with the difficulty of high clinical variability. Complementary tests are used to support the diagnosis and rule out other causes that can produce similar symptoms, with magnetic resonance imaging being the most cost-effective imaging test. Treatment is based on the administration of thiamine, which should be started early, and parenterally at the appropriate doses, in all patients with compatible symptoms, without waiting to confirm the diagnosis.

[Corticosteroids and metabolic resuscitation (hydrocortisone, ascorbic acid and thiamine) in sepsis and septic shock]. / Corticoides y resucitación metabólica (hidrocortisona, ácido ascórbico y tiamina) en la sepsis y el shock séptico.

In accordance with the recommendations of, among others, the Surviving Sepsis Campaign, adjunctive therapies may play a role in the treatment and the prognosis in sepsis patients. Corticosteroids, ascorbic acid, and thiamine has been identified as a potential therapy for septic shock alone or in combination. Since many years, low-dosis corticosteroids has been proposed as adjuvant therapies for these patients. However, there are still many controversies regarding the role of systemic low-dose corticosteroids as a part of the treatment of septic shock. In recent clinical and experimental investigations, mitochondrion-target therapy bic acid and thiamine) for sepsis has been suggested to reduce MODS severity and mortality although their clinical results are not yet convincing for their use. In the present article, we have performed a narrative review of the current literature including pathophysiologic rationale and the current evidence for such therapies in septic patients.

Probable first report of a motor deafferentation syndrome in the Paraguayan War / Provável primeiro relato de uma síndrome de desaferentação motora na Guerra do Paraguai

ABSTRACT The Paraguayan War ended 150 years ago. Back then, there were outbreaks of combatants' limb weakness and tingling related to "palustrian cachexia", not clearly funded at the time on nutritional deficiency, the use of native flora to feed troops, and alcoholism. We report a case of a soldier with ascending paralysis, mental confusion and finally tetraplegia with preserved oculomotricity. This would probably be a case of locked-in syndrome (LIS) due to Gayet-Wernicke's encephalopathy consequent to thiamine deficiency. The role of thiamine in the peripheral or central nervous system expression was shown decades later to be related to poor diet, or use of foods containing thiaminase or thiamine antagonists, worsened by the fact that the bodily stores of thiamine are restricted, and deficits may grow fast.

RESUMO A Guerra do Paraguai terminou há 150 anos. Houve surtos de fraqueza dos membros dos combatentes e parestesias relacionadas à "caquexia palustre", não claramente fundamentados na época sobre deficiência nutricional, o uso da flora nativa para alimentar as tropas e o alcoolismo. Referimo-nos a um caso de um militar com paralisia ascendente, confusão mental e finalmente tetraplegia com oculomotricidade preservada. Esse relato seria provavelmente devido a um caso de "síndrome do encarceramento" secundário à encefalopatia de Gayet-Wernicke consequente à deficiência de tiamina. O papel da tiamina na expressão periférica ou do SNC foi previsto décadas mais tarde em relação a uma dieta pobre, ou ao uso de alimentos contendo tiamina ou antagonistas da tiamina, agravado pelo fato de que as reservas corporais de tiamina são restritas, e os déficits podem se manifestar rapidamente.

Síndrome de Wernicke-Korsakoff em paciente gestante de 18 anos / Wernicke-Korsakoff syndrome in an 18-year-old pregnant patient

A síndrome de Wernicke-Korsakoff é uma rara encefalopatia desencadeada pela deficiência de tiamina, uma vitamina do complexo B, que atua como importante cofator de enzimas responsáveis pela manutenção da homeostase da energia cerebral. Apresentamos o caso de uma paciente de 18 anos, gestante, com diagnóstico prévio de pancreatite aguda biliar, que evoluiu à hiperêmese gravídica e à Wernicke-Korsakoff. Objetivamos, com este trabalho, chamar a atenção para a importância do diagnóstico imediato dessa síndrome diante de seu potencial em causar danos cerebrais irreversíveis, caso não tratada precocemente.

Wernicke-Korsakoff syndrome is a rare encephalopathy triggered by deficiency of thiamine, a B-complex vitamin, which acts as an important cofactor of enzymes responsible for maintaining brain energy homeostasis. We present the case of an 18-year-old pregnant woman with previous diagnosis of acute biliary pancreatitis, who developed hyperemesis gravidarum and Wernicke-Korsakoff. With this work, we aim to draw attention to the importance of the immediate diagnosis of this syndrome in view of its potential to cause irreversible brain damage if not treated early.

Wernicke encephalopathy as a complication of hyperemesis gravidarum: case report / Encefalopatia de Wernicke como complicação de hiperêmese gravídica: relato de caso

Abstract Wernicke encephalopathy (WE) is an acute neurological disorder resulting from vitamin B1 deficiency, which is common in chronic alcoholism. We report a rare case of WE due to hyperemesis gravidarum in a 25-year-old pregnant patient at 13 weeks and 5 days of gestation. Initially, the disease manifested as weakness, mental confusion, anterograde amnesia, and visual and auditory hallucinations. The diagnosis was established after the detection of suggestive findings of WE in the thalamus by magnetic resonance imaging (MRI) and a rapid improvement in the patient's clinical status subsequent to treatment with thiamine. Hyperemesis is a rare cause of WE, which makes the reported case important in the literature and reinforces the need for attention in clinical practice to rare but important complications of this common condition (hyperemesis gravidarum).

Resumo A encefalopatia de Wernicke (EW) é uma condição neurológica aguda resultada da deficiência de vitamina B1, muito comum em etilistas crônicos. Relatamos um caso de EW secundário a um quadro de hiperêmese gravídica em uma gestante de 25 anos de idade e 13 semanas e 5 dias de idade gestacional. Inicialmente essa desordem se manifestou como fraqueza, confusão mental, amnésia anterógrada, e alucinações auditivas e visuais. O diagnóstico foi estabelecido depois da detecção de achados sugestivos de EW na ressonância nuclear magnética e da melhora do quadro clínico com reposição de tiamina. A hiperêmese gravídica não é uma causa comum de EW, o que faz com que o presente relato de caso tenha importância na literatura e reforça a necessidade de atenção na prática clínica para complicações raras mas importantes desse quadro tão comum (hiperêmese gravídica).

Polioencephalomalacia (PEM) in calves associated with excess sulfur intake / Polioencefalomalacia em bezerros associada à ingestão de alimento com excesso de enxofre

Polioencephalomalacia (PEM) is the morphological characterization for softening of brain gray matter, and excess sulfur intake is one of its main causes. This study describes an outbreak of this disease in 1-to-3-month-old calves in a farm located in Santa Catarina state, Brazil. The herd consisted of 27 Jersey male calves whose diet was composed of initial feed, ground whole corn, and mineral salt. From this herd, 10 animals became ill, showing signs of apathy, anorexia and blindness, evolving to generalized weakness and death. Necropsy was performed in three of these animals, which showed flattening of the cerebral convolutions in addition to softened, yellowish areas in the cerebral cortex. Histopathological examination revealed deep laminar necrosis associated with perineuronal and perivascular edema, as well as neurons with wrinkled, eosinophilic, or vacuolated cytoplasm. The following sulfur doses were observed: 8,010mg/kg in corn, 6,385mg/kg in initial feed, 1,060mg/kg in mineral salt and 2.3mg/L in water, reaching dose values far above the accepted, totaling a daily intake of approximately 6,533.5mg sulfur/animal/day. As differential diagnosis, lead was dosed in the kidneys and liver of the three calves, with negative results. Also, the calf that sickened last was treated with 20mg/kg thiamin and 0.2mg/kg dexamethasone (IM; QID) for three days and eventually recovered. According to anatomopathological findings, excess sulfur intake and therapeutic diagnosis, sulfur poisoning was suggested as the cause of PEM in these 1-to-3-month-old calves. Occurrence of PEM is rare in calves at such a young age.(AU)

A polioencefalomalacia (PEM) é a caracterização morfológica para o amolecimento da substância cinzenta encefálica, e uma de suas principais etiologias é a ingestão excessiva de enxofre. Este trabalho descreve um surto desta enfermidade em bezerros de um a três meses de idade em uma propriedade de Santa Catarina. O lote era composto por 27 bezerros machos da raça Jersey, com alimentação composta por ração inicial, milho inteiro triturado e sal mineral. Deste lote, 10 animais adoeceram, apresentando sinais de apatia, anorexia e cegueira, com evolução para fraqueza generalizada. Nove bezerros morreram e três foram submetidos a necropsia, que demonstraram achatamento das circunvoluções cerebrais além de áreas de amolecimento e coloração amarelada no córtex cerebral. Realizou-se exame histopatológico que evidenciou necrose laminar profunda associada a edema perineuronal e perivascular, além de neurônios com citoplasma enrugado, eosinofílico ou vacuolizado. A dosagem de enxofre resultou em 8010mg/Kg no milho, 6385mg/Kg na ração, 1060mg/Kg no sal mineral e 2,3mg/L na água, atingindo valores muito acima do tolerado, totalizando a ingestão diária de cerca de 6533,5mg de enxofre/animal/dia. Como diagnóstico diferencial realizou-se dosagem de chumbo de amostras de rim e fígado dos três bezerros com resultado negativo. Ainda, o último bovino a adoecer foi tratado com 20mg/Kg de tiamina e 0,2mg/Kg de dexametasona IM, QID, durante três dias e recuperou-se. De acordo com os achados anatomopatológicos e o excesso de enxofre na dieta, sugere-se que a intoxicação por enxofre seja a causa de PEM nestes bezerros de um a três meses de idade, sendo essa enfermidade rara em bovinos tão jovens.(AU)

Encefalopatía de Wernicke secundario a hiperémesis gravídica: la importancia del diagnóstico temprano / Wernicke encephalopathy secondary to hyperemesis gravidarum: importance of early diagnosis

RESUMEN Introducción: La hiperémesis gravídica (HG) es una condición frecuente en el embarazo, que puede resultar en complicaciones potencialmente letales como la encefalopatía de Wernicke (EW), síndrome que al ser reconocido y tratado tardíamente puede traducirse en una alta morbi-mortalidad materna y fetal. Objetivo: Describir el primer caso de EW secundario a HG en Colombia y realizar una revisión de la literatura publicada sobre su diagnóstico y tratamiento. Materiales y métodos: Se describe un caso de EW secundario a HG en el que se brindó un manejo interdisciplinario. Se realizó una revisión de la literatura con los términos "encefalopatía de Wernicke", "hiperémesis gravídica" y "embarazo" incluyendo reportes de casos, series de casos, artículos de revisión, investigaciones originales o cartas al editor en inglés, español y francés, en donde se analizaron el método y tiempo del diagnóstico, pauta de tratamiento y estado funcional final. Resultados: Se incluyeron 69 publicaciones y se identificaron 89 casos. En 23 de ellos se presentó pérdida gestacional, sólo en el 12,4% de los casos se reportó el nivel de tiamina, de los cuales en el 90% se encontraba disminuido y de los casos en donde se reportó estado funcional final en el 5,9% la gestante falleció. Conclusión: La EW secundaria a HG es una complicación potencialmente letal. Debe sospecharse ante cualquier alteración neurológica e historia de emesis persistente. El diagnóstico y tratamiento oportuno interdisciplinario son fundamentales para disminuir el riesgo de secuelas que limitan la capacidad funcional con alto impacto en la calidad de vida.

ABSTRACT Introduction: Hyperemesis gravidarum (HG) is a frequent condition in pregnancy, which can result in potentially lethal complications such as Wernicke encephalopathy (WE), a syndrome that can be translated into a high maternal and fetal morbidity and mortality if it is recognized and treated late. Objective: To describe the first case of WE due to HG in Colombia and to review the published literature about its diagnosis and treatment. Materials and methods: We describe a case of WE due to HG with an interdisciplinary approach. A review of the literature was performed with the terms "Wernicke's encephalopathy", "hyperemesis gravidarum" and "pregnancy" including case reports, case series, review articles, original investigations or letters to the editor in English, Spanish and French, where the method and time of the diagnosis, treatment regimen and sequelae were analyzed. Results: Sixty-nine publications were included and 89 cases were identified. In 23 of them had a gestational loss, only in 12.4% of the cases the thiamine level was reported, in which 90% was diminished and in the cases where the final functional status was reported in 5.9% of the pregnant woman died. Conclusion: WE due to HG is a potentially lethal complication. In any neurological disturbance and history of persistent emesis it should be suspected. Timely interdisciplinary diagnosis and treatment are essential to reduce the risk of sequelae that limit functional capacity with a high impact on quality of life.

Síndrome de anemia megaloblástica sensible a la tiamina de aparición en la niñez, con mutación en el genSLC19A2: caso clínico / Infantile-onset thiamine responsive megaloblastic anemia syndrome with SLC19A2 mutation: a case report

Antecedentes. El síndrome de anemia megaloblástica sensible a la tiamina (TRMA, por sus siglas en inglés), también conocido como síndrome de Rogers, se caracteriza por presentar anemia megaloblástica, hipoacusia neurosensorial y diabetes mellitus. Las alteraciones en el transporte de la tiamina hacia las células se deben a mutaciones homocigotas o heterocigotas compuestas en el gen SLC19A2. Presentación de un caso. Presentamos el caso de una niña que manifestaba sordera neurosensorial tratada con una prótesis auditiva, diabetes con necesidad de insulina y anemia macrocítica, tratada con tiamina (100 mg/día). El nivel de hemoglobina mejoró hasta alcanzar 12,1 g/dl después de aumentar la dosis terapéutica de tiamina hasta 200 mg/día. Conclusión. Se debe evaluar a los pacientes con TRMA para detectar anemia megaloblástica, hipoacusia neurosensorial y diabetes mellitus. Se les debe dar seguimiento para determinar la respuesta de la enfermedad hematológica y de la diabetes después de la terapia con tiamina. La dosis terapéutica de tiamina puede aumentarse según la respuesta clínica. Debe proporcionarse asesoramiento genético.

Background. Thiamine-responsive megaloblastic anemia syndrome (TRMA), also known as Rogers syndrome, is characterized by megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. Disturbances of the thiamine transport into the cells results from homozygous or compound heterozygous mutations in the SLC19A2 gene. Case presentation. We report a girl which presented with sensorineural deafness treated with a hearing prosthesis, insulin requiring diabetes, macrocytic anemia, treated with thiamine (100 mg/day). Hemoglobin level improved to 12.1 g/dl after dose of thiamine therapy increased up to 200 mg/day. Conclusion. Patients with TRMA must be evaluated for megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. They must be followed for response of hematologic and diabetic after thiamine therapy. It should be kept in mind that dose of thiamine therapy may be increased according to the clinical response. Genetic counseling should be given.

Encefalopatía de Wernicke en gestante con hiperemesis gravídica grave / Wernicke's encephalopathy associated with gravidarum hypermesis

Resumen ANTECEDENTES: la encefalopatía de Wernicke se origina por el déficit de vitamina B1 y sus características sobresalientes son: triada de confusión, ataxia y oftalmoplejia. Cuando hay déficit de memoria o aprendizaje se denomina síndrome de Wernicke-Korsakoff. CASO CLÍNICO: se comunica el caso de una paciente en su primer embarazo, de 18 semanas de gestación, con varios ingresos al hospital por hiperemesis gravídica; acudió al servicio de Urgencias por un cuadro de pérdida progresiva de fuerza en las extremidades inferiores. A los cinco días del ingreso tuvo empeoramiento del estado general. A la exploración física se identificó nistagmus horizonto-rotatorio, bilateral, espontáneo, tetraparesia flácida de predominio proximal, con arreflexia global de predominio en EEII y pérdida de tono de los esfínteres. La paciente se encontraba confusa, desorientada y repetitiva en su discurso. Se inició tratamiento con tiamina parenteral, por sospecha de polineuropatía metabólica por déficit de tiamina; el nistagmus y el cuadro de confusión disminuyeron. Un mes más tarde se programó para tratamiento intensivo de rehabilitación. La evolución del embarazo trascurrió con normalidad y continuó en tratamiento con tiamina y antieméticos, con estabilidad y control del cuadro. CONCLUSIÓN: la encefalopatía de Wernicke es una padecimiento de frecuencia excepcional, pero de consecuencias muy graves; de ahí la importancia de tenerlo en mente en pacientes embarazadas susceptibles de padecerlo y poner en práctica las medidas de profilaxis adecuada que eviten su aparición y las secuelas.

Abstract BACKGROUND: Wernicke's encephalopathy, caused by vitamin B1 deficiency, is characterised by the triad of confusion, ataxia and ophthalmoplegia. If memory or learning deficits appear simultaneously, it is known as Wernicke-Korsakoff syndrome. CLINICAL CASE: We present the case of a primigravida in her 18th week of pregnancy who had been suffering from hyperemesis gravidarum. She came to the emergency unit because of progressive loss of strength in her lower limbs. Five days after her admission, a deterioration of her general condition was observed. The examination revealed bilateral spontaneous horizontal-rotatory nystagmus, flaccid tetraparesis with proximal predominance, global areflexia predominantly in the lower limbs and loss of sphincters tone. The patient was confused, disoriented and repetitive in her speech. Parenteral vitamin B1 was initiated as a metabolic polineuropathy due to thiamine insufficiency was suspected. As a result the symptoms of nystagmus and confusion subsided. Intensive physical therapy was programmed in a rehabilitation unit 1 month later. Pregnancy developed normally and she continued with thiamine and antiemetic therapy. CONCLUSIONS: Wernicke's encephalopathy is an infrequent pathology, but its consequences may be really serious. This is the reason why it is paramount to take into account this entity in pregnant women liable to develop it and to carry out adequate prophylaxis to prevent its appearance and development of posterior sequels.

Psicosis de Korsakoff, a propósito de un caso interesante / Korsakoff's psychosis, about an interesting case

La psicosis de Korsakoff (PK) es una de las causas más frecuentes de amnesia. Se caracteriza por confusión mental, deterioro de la memoria reciente y confabulación. Se presenta el caso de un paciente masculino de 53 años de edad, fumador de un paquete al día, durante más de 35 años, bebedor de riesgo, con antecedentes de hipertensión arterial e hipercolesterolemia. Se diagnosticó como psicosis de Korsakoff debido al déficit de tiamina o vitamina B1. La psicosis de Korsakoff es un síndrome amnésico que puede presentarse precedido o no de encefalopatía de Wernicke (EW), por lo que se diagnostican menos casos de los que en realidad existen. Por ello, es un problema frecuentemente infradiagnosticado en los centros de salud lo que resulta interesante el conocimiento de esta patología (AU).

Korsakoff psychosis is one of the most frequent causes of amnesia. It is characterized by mental confusion, impairment of the recent memory and confabulation. It is presented the case of a male patient, aged 53 years, who smoked 1 packet of cigarettes a day during more than 35 years, risk drinker with antecedents of arterial hypertension and hypercholesterolemia. He was diagnosed as Korsakoff psychosis due to the thiamine or B1 vitamin deficit. Korsakoff psychosis is an amnesic syndrome that may be preceded or not by Wernicke encephalopathy, so there are diagnosed fewer cases than those truly existing. That is why it is a problem frequently underdiagnosed in health care institutions, making interesting this disease´s knowledge (AU).

Occurrence of acid erosion of the teeth, actinomycosis and polioencephalomalacia in flock of sheep fed wet low pectin citrus pulp / Ocorrência de erosão ácida dos dentes, actinomicose e polioencefalomalacia em rebanho de ovinos alimentados com polpa cítrica úmida despectinada

The citrus pulp can be used as a substitute in ruminant feed reducing costs and maintaining the nutritional quality of food. However, this compound should be used carefully so as not to cause harm to the animals. The present report aims to describe the occurrence of dental erosion, actinomycosis and polioencephalomalacia in sheep raised and kept with a wet low pectin citrus pulp based diet, composing 50% of roughage. Actinomycosis was diagnosed in five animals through clinical and radiographic examinations and microbiological culture, and, after treatment, three animals were cured. Polioencephalomalacia was confirmed in ten animals by clinical diagnostics, in nine out of ten animals by therapeutic diagnosis, and in one animal by post-mortem anatomopathological examination. According to the observed, we recommend caution when large amounts of citrus pulp are used as bulky food.(AU)

A polpa cítrica está entre os produtos que podem ser utilizados como substitutos na alimentação de ruminantes, diminuindo os gastos e mantendo a qualidade nutricional do alimento fornecido aos animais, porém, esses alimentos devem ser utilizados de forma que não tragam malefícios. Assim, o presente relato visa apresentar a ocorrência de erosão dentária, actinomicose e polioencefalomalácia em ovinos criados e mantidos recebendo alimentação à base de polpa cítrica úmida despectinada na concentração de 50% do volumoso. A actinomicose foi diagnosticada em cinco animais por meio de exame clínico, radiográfico e cultivo microbiológico, e após tratamento três animais foram curados. Já a polioencefalomalácia foi confirmada em dez animais pelos sintomas manifestados, eficiência da terapia instituída em nove animais e exame anatomopatológico de um animal que veio a óbito. De acordo com o observado, deve-se ter cuidado ao utilizar grande quantidade de polpa cítrica úmida como volumoso.(AU)

Psicosis de Korsakoff, a propósito de un caso interesante / Korsakoff's psychosis, about an interesting case

La psicosis de Korsakoff (PK) es una de las causas más frecuentes de amnesia. Se caracteriza por confusión mental, deterioro de la memoria reciente y confabulación. Se presenta el caso de un paciente masculino de 53 años de edad, fumador de un paquete al día, durante más de 35 años, bebedor de riesgo, con antecedentes de hipertensión arterial e hipercolesterolemia. Se diagnosticó como psicosis de Korsakoff debido al déficit de tiamina o vitamina B1. La psicosis de Korsakoff es un síndrome amnésico que puede presentarse precedido o no de encefalopatía de Wernicke (EW), por lo que se diagnostican menos casos de los que en realidad existen. Por ello, es un problema frecuentemente infradiagnosticado en los centros de salud lo que resulta interesante el conocimiento de esta patología (AU).

Korsakoff psychosis is one of the most frequent causes of amnesia. It is characterized by mental confusion, impairment of the recent memory and confabulation. It is presented the case of a male patient, aged 53 years, who smoked 1 packet of cigarettes a day during more than 35 years, risk drinker with antecedents of arterial hypertension and hypercholesterolemia. He was diagnosed as Korsakoff psychosis due to the thiamine or B1 vitamin deficit. Korsakoff psychosis is an amnesic syndrome that may be preceded or not by Wernicke encephalopathy, so there are diagnosed fewer cases than those truly existing. That is why it is a problem frequently underdiagnosed in health care institutions, making interesting this disease´s knowledge (AU).

Wernicke's encephalopathy with chorea: Neuroimaging findings / Encefalopatia de Wernick e Coreia: achados de neuroimagem

ABSTRACT We present a case report of motor and cognitive disorders in a 36-year-old woman with a history of twelve years of heavy alcohol abuse. The patient presented depressive symptoms over the course of one year after a loss in the family, evolving with ataxia, bradykinesia and choreiform movements. Progressive cognitive decline, sleep alterations and myalgia were also reported during the course of disease evolution. Physical examination revealed spastic paraparesis with fixed flexion of the hips and knees with important pain upon extension of these joints. Initial investigation suggested the diagnosis of thiamine deficiency by brain magnetic resonance imaging (MRI).

RESUMO Um relato de caso de transtornos motor e cognitivo em uma mulher de 36 anos, com história de doze anos de abuso de álcool. A paciente apresentou sintomas depressivos ao longo de um ano após uma perda na família, evoluindo com ataxia, bradicinesia e movimentos coreiformes. O declínio cognitivo progressivo, alterações do sono e mialgia também foram relatados durante o curso da evolução da doença. O exame físico demonstrou paraparesia espástica com flexão fixa dos quadris e joelhos com dor importante na extensão dessas articulações. A investigação inicial sugeriu o diagnóstico de deficiência de tiamina por ressonância magnética cerebral (MRI).