El rol de los suplementos vitamínicos en pacientes con depresión / The role of vitamin supplements in patients with depression

La depresión es un trastorno del estado de ánimo que se caracteriza por la existencia de un sentimiento de tristeza lo suficientemente intenso como para interferir en el desarrollo de las actividades habituales. A partir de un caso clínico real, en el que una paciente con depresión solicita a su médico de cabecera sumar un suplemento de vitaminas a su plan terapéutico, revisamos la evidencia disponible sobre el uso de estos micronutrientes para el tratamiento de la depresión, y encontramos que no existen pruebas robustas que avalen la suplementación vitamínica en pacientes con este problema de salud. (AU)

Depression is a mood disorder characterised by the existence of a feeling of sadness intense enough to interfere with the performance of normal activities. Based on a real clinical case, in which a patient with depression asked her family doctor to add a vitamin supplement to her therapeutic plan, we reviewed the available evidence on the use of these micronutrients for the treatment of depression and found that there is no robust evidence to support vitamin supplementation in patients with this health problem. (AU)

Comparison of sublingual and intramuscular administration of vitamin b12 for the treatment of vitamin b12 deficiency in children

Abstract Background: In most countries, contrary to some disadvantages, such as pain, relatively higher cost, and poor adherence to treatment, intramuscular (IM) route is still the primary treatment method for Vitamin B12 (VB12) deficiency. In recent years, because of these difficulties, new treatment methods are being sought for VB12 deficiency. Objectives: We aimed to compare sublingual (SL) and IM routes of VB12 administration in children with VB12 deficiency and to compare the efficacy of methylcobalamin and cyanocobalamin therapy in these children. Methods: This retrospective study comprised 129 patients with VB12 deficiency (serum Vitamin 12 level ≤ 200 pg/mL) aged 5-18 years. Based on the formulations of Vitamin 12, we divided the patients into three treatment groups as IM cyanocobalamin, SL cyanocobalamin, and SL methylcobalamin. Results: After Vitamin 12 therapy, serum Vitamin 12 levels increased significantly in all patients, and there was a statistically significant difference between the treatment groups (p < 0.05). Conclusions: SL cyanocobalamin and methylcobalamin were found as effective as IM cyanocobalamin for children with Vitamin 12 deficiency in correcting serum Vitamin 12 level and hematologic abnormalities.

Dietas vegetarianas en alumnos universitarios / Vegetarian diets in first year university students

INTRODUCCIÓN: Se ha observado un aumento en la popularidad de las dietas vegetarianas, en especial en la población adolescente y adulto joven. Estas dietas se presentan como saludables y balanceadas pero las recomendaciones son controversiales en cuanto a los potenciales déficit nutricionales. OBJETIVO: Identificar la frecuencia y tipos de dieta vegetariana utilizada, su motivación y fuentes de información. PACIENTES Y MÉTODO: Estudio transversal y analítico en universitarios de primer año de la Pontificia Universidad Católica de Chile (PUC). Se realizó una encuesta vía online obteniendo información demográfica y caracterización de dietas de aquellos que se consideraban vegetarianos. Las variables fueron analizadas mediante el software IDM SPSS Statistics® y en planilla Excel® de forma cuantitativa. RESULTADOS: 152 alumnos respondieron la encuesta (15,2% del total), siendo el 49,4% de estos vegetariano. El 32,4% inició este patrón alimentario entre los 12-18 años; sus motivaciones más frecuentes fueron las medio ambientalistas (91,9%) y animalistas (72,9%). El 52,9% de los vegetarianos reciben suplementación de vitamina b 12 pero solo un 15,9% reportó presentar este déficit. El 75,7% obtiene información relacionada acerca de las dietas vegetarianas a través de medios digitales. CONCLUSIÓN: Se encontró un alto porcentaje de estudiantes vegetarianos en las encuestas contestadas, lo que hace necesario que los profesionales de salud estén capacitados en esta área para asegurar una adecuada educación nutricional, suplementación si es necesario, y seguimiento.

INTRODUCTION: There has been an increase in the popularity of vegetarian diets, especially among adolescents and young adults. These diets seem to be healthy and balanced, but the recommendations are contro versial regarding potential nutritional deficits. OBJECTIVE: To identify the frequency and types of ve getarian diet used, their motivation, and sources of information. PATIENTS AND METHOD: Cross-sec tional analytical study in freshmen students from the Pontifical Catholic University of Chile (PUC). Through an online survey, we collected demographic information and diet characterization of those who considered themselves as vegetarians. Variables were analyzed using IDM SPSS Statistics® soft ware and in Excel® spreadsheet in a quantitative way. RESULTS: 152 students answered the survey (15.2% of the sample) and, out of these, 49.4% were vegetarian. 32.4% started this eating pattern bet ween the ages 12 and 18 and among their most frequent motivations were environmentalists (91.9%) and animalists (72.9%). 52.9% of vegetarians take vitamin B 12 supplementation but only 15.9% reported having this deficiency. 75.7% obtain information related to vegetarian diets through digital media. CONCLUSION: In the surveys answered, we found a high percentage of vegetarian students, the refore, health professionals need to be trained in this area to assure adequate nutritional education, supplementation if necessary, and follow-up.

A importância do metilfolato na prevenção dos defeitos abertos do tubo neural / The importance of methylfolate in the prevention of open neural tube defects

A suplementação periconcepcional com vitamina B9 ou folato é considerada medida efetiva para diminuir a ocorrência e a recorrência de defeitos do tubo neural (DTNs). É recomendada para todas as mulheres que planejam gravidez ou que possam engravidar. O ácido fólico necessita passar por processos enzimáticos necessários à metabolização para a sua forma ativa L-5-metiltetrahidrofolato (L-metilfolato, 5-MTHF) envolvida nos processos biológicos e que circula no plasma. A enzima metilenotetrahidrofolato redutase (MTHFR) é de fundamental importância para fornecer 5-MTHF, forma biologicamente ativa. A presença de polimorfismo do gene da MTHF-redutase promove menor atividade enzimática e menor produção de L-metilfolato.(AU)

Effect of accelerated corneal crosslinking on ocular response analyzer waveform-derived parameters in progressive keratoconus / Efeito do crosslimking corneano acelerado nos parâmetros derivados da forma de onda do analisador de resposta ocular no ceratocone progressivo

ABSTRACT Purpose: To evaluate the effect of accelerated corneal crosslinking on corneal biomechanics with an ocular response analyzer in patients with progressive keratoconus. Methods: In this retrospective study, 50 eyes of 45 patients with progressive keratoconus who underwent accelerated corneal crosslinking were evaluated with ocular response analyzer waveform parameters before and one year after corneal crosslinking. Paired two-tailed Student's t-test was performed to compare the parameters before vs. after corneal crosslinking. Results: Mean patient age was 17.6 ± 3.6 (range 9-25) years. A significant increase was observed in p1 area, p2 area, h2, and dive2 values. No significant difference in corneal hysteresis, corneal resistance factor, or other waveform-derived parameters was observed at one year postoperatively. Conclusion: For estimating the effect of accelerated corneal crosslinking on corneal biomechanics, parameters such as p1 area, p2 area, h2, and dive2 are more sensitive than corneal hysteresis and corneal resistance factor. These results may help us to find out which corneal crosslinking method is most effective for stiffening the cornea.

RESUMO Objetivo: Avaliar o efeito do cross-linking corneano acelerado na biomecânica corneana com analisador de resposta ocular em pacientes com ceratocone progressivo. Métodos: Neste estudo retrospectivo, 50 olhos de 45 pacientes com ceratocone progressivo submetidos à cross-linking corneano acelerado foram avaliados com os parâmetros da forma de onda do analisador de resposta ocular antes e um ano após o tratamento com cross-linking corneano. O teste t de Student pareado bicaudal foi realizado para comparar os parâmetros antes e depois do cross-linking corneano. Resultados: A média de idade dos pacientes foi de 17,6 ± 3,6 (variação de 9 a 25) anos. Um aumento significativo foi observado nos valores de p1area, p2area, h2 e dive2. Nenhuma diferença significativa foi encontrada na histerese da córnea, fator de resistência da córnea ou outros parâmetros derivados da forma de onda foi observada em um ano de pós-operatório. Conclusão: Para estimar o efeito do cross-linking corneano acelerado na biomecânica corneana, parâmentros como p1area, p2area, h2 e dive2 são mais sensíveis que histerese da córnea e fator de resistência corneana. Esses resultados podem nos ajudar a descobrir qual método cross-linking corneano é mais eficaz no enrijecimento da córnea.

Elaboración de productos panificados a partir de premezcla de harinas aptas para celíacos enriquecidas con hierro y vitaminas del complejo B y su aceptabilidad / Preparation of bakery products from premix of flours suitable for celiac people and enriched with iron and vitamin B complex and their acceptability

La Ley 20.830/98 reglamenta el enriquecimiento de la harina, sin embargo, no contempla a la premezcla de harinas aptas para celíacos. Aproximadamente 50% de los celíacos presenta anemia, por lo es necesario elaborar productos cuyas características nutricionales, sensoriales y funcionales sean óptimas. El objetivo del trabajo fue enriquecer con hierro y vitaminas del complejo B (mix) la premezcla de harinas apta para celíacos y evaluar la aceptabilidad de dos panificados de alta demanda poblacional elaborados a partir de la premezcla. En cocinas especializadas para celíacos se elaboraron panes y palitos, cada uno en dos versiones (con mix y sin mix), se presentaron en envases individuales y etiquetados forma numérica para la degustación. Se evaluó la aceptabilidad de los panificados utilizando una escala hedónica de 9 puntos a través de un cuestionario autoadministrado. Se utilizó la prueba t de Student para la comparación de promedios obtenidos. Participaron 105 celíacos (81% mujeres), el promedio de edad fue 34,5±10,9 años Según auto-reporte el 60% cursó con anemia. La puntuación global promedio de aceptabilidad fue: pan sin mix 7,42±1,33puntos; pan con mix 8,00±1,06 puntos; palito sin mix 7,91±1,05 puntos y palito con mix 7,86±1,17 puntos. Se realizó el análisis de la premezcla apta para celíacos y se constató que la misma cumple con el aporte de micronutrientes (hierro, B1, B2, B3 y ácido fólico). En este estudio, los panes con mix tuvieron mayor aceptabilidad que aquellos sin mix. En el caso de los palitos no se observaron diferencias en la aceptabilidad entre productos (con mix vs sin mix)(AU)

Caso clínico: hipofosfatasia de la niñez. Seguimiento clínico / Clinical case: childhood hypophosphatasia. Clinical follow-up

La hipofosfatasia (HP) es una enfermedad congénita, causada por mutaciones con pérdida de función en el gen ALPL que codifica la isoenzima no específica de tejido de la fosfatasa alcalina (TNSALP). Su expresión clínica es muy variable, desde casos de muerte intraútero por alteración grave de la mineralización ósea, hasta casos solo con caída prematura de la dentición. Se presenta el caso clínico de un varón al que se le diagnosticó odontohipofosfatasia a los 30 meses por pérdida temprana de piezas dentarias y niveles anormalmente bajos de fosfatasa alcalina, sin signos de raquitismo ni deformidades óseas. Durante su seguimiento, hasta los 13 años, presentó síntomas compatibles con HP infantil leve, como cansancio al caminar, incoordinación en la marcha y dolor en miembros inferiores que aumentaban con la actividad física. Ante la aparición de edema bimaleolar y poca respuesta al tratamiento con calcitonina y antiinflamatorios, se descartaron patologías infecciosas o reumáticas o ambas y se diagnosticó, por biopsia de tibia y peroné, periostitis sin detección de cristales de pirofosfato. Los controles radiológicos durante su evolución mostraron ensanchamiento metafisario en muñeca, falta de remodelado de metacarpianos, hojaldrado perióstico en tibia y peroné e hipomineralización en metáfisis tibiales, con "lenguas radiolúcidas" características de HP. Como conclusión, la hipofosfatasia debe considerarse como una entidad clínica para descartar en niños que presentan pérdida temprana de dientes. La presencia de este cuadro clínico es en general suficiente para realizar el diagnóstico de HP de la niñez. (AU)

Hypophosphatasia (HP) is a congenital disease, caused by mutations with loss of function in the gene ALPL that encodes the non-specific tissue isoenzyme of alkaline phosphatase (TNSALP). Its clinical expression displays considerable variability, from cases of intrauterine death due to severe alteration of bone mineralization, to cases with only early loss of teeth. We report the case of a male, diagnosed as odontohypophosphatasia at 30 months of age due to early loss of teeth and abnormally low levels of alkaline phosphatase, without signs of rickets or bone deformities. During follow-up, up to 13 years of age, he presented symptoms consistent with mild infantile HP such as tiredness when walking, lack of gait coordination, and pain in lower limbs, especially after physical activity. Due to the appearance of bimalleolar edema and poor response to treatment with calcitonin and anti-inflammatory drugs, infectious and / or rheumatic pathologies were ruled out. Periostitis without pyrophosphate crystal detection was diagnosed by tibial and fibular biopsy. Radiological controls during follow up showed metaphyseal wrist enlargement, lack of remodeling of metacarpals, periosteal flaking in the tibia and fibula and hypomineralization in the tibial metaphysis, with "radiolucent tongues"; characteristic of HP. In conclusion, hypophosphatasia should be considered as a clinical entity in children who present early loss of teeth. The presentation of this clinical case is generally sufficient to make the diagnosis of childhood HP. (AU)

Randomized crossover trial of amoxapine versus vitamin B12 for retrograde ejaculation

ABSTRACT Objective To compare the efficacy and safety of amoxapine and vitamin B12 for treating retrograde ejaculation (RE). Materials and Methods Between May 2009 and November 2012, this open-label, randomized, crossover study enrolled 26 men suffering with RE at Department of Reproductive Medicine, Omori Hospital. Patients were randomly allocated into two groups (n=13 each). The amoxapine-B12 group received amoxapine (50 mg daily for 4 weeks, orally) followed (after a 1-week washout period) by vitamin B12 (500 μg three-times daily for 4 weeks). The B12-amoxapine group received the opposite regimen. All patients masturbated to ejaculation at least twice during each treatment period. The primary outcome was antegrade ejaculation of semen, as reported by the patient, on more than one occasion during either treatment period (defined as treatment success). Any adverse events were noted. Success rates were compared between treatments using Fisher’s exact test. Results One patient (B12-amoxapine group) withdrew for personal reasons (breakdown of marital relations); all other patients completed the study. Overall success rate was 88% (22/25). Success rate was higher for amoxapine than for vitamin B12 (80%, 20/25 vs 16%, 4/25; P<0.0001). 18 patients were responsive to amoxapine but not to vitamin B12, 2 patients were responsive to vitamin B12 but not amoxapine, 2 patients were responsive to both drugs, and 3 patients had no response to either drug. One patient (4%) reported sleepiness and 2 (8%) reported constipation while receiving amoxapine. No adverse events were reported during vitamin B12 treatment. Conclusions Amoxapine may be an effective, safe and well-tolerated therapy for RE.

Defectos del tubo neural y del ácido fólico: recorrido histórico de una intervención preventiva altamente efectiva / Neural tube defects and folic acid: a historical overview of a highly successful preventive intervention

Este artículo describe de forma comprehensiva parte del devenir histórico que han tenido, el conocimiento médico de los defectos del tubo neural (DTN) y el descubrimiento de la vitamina B9 o ácido fólico, así como algunos de los acontecimientos investigativos relevantes que a través de varios siglos definieron las relaciones entre la comprensión de la embriología del sistema nervioso central, el descubrimiento de la vitamina, la correlación del ácido fólico con la proliferación celular y finalmente el desarrollo de medidas preventivas de este tipo de defectos. Se pretende, a través de esta narrativa, exponer los conceptos históricamente relevantes que sustentan las acciones de índole clínico y de impacto poblacional que previenen los DTN a través del consumo preconcepcional de ácido fólico.

This article gives a broad overview of part of the historical evolution of medical knowledge about neural tube defects (NTD) and the discovery of vitamin B9 or folic acid, as well as some relevant research events that, over the course of several centuries, defined the relationships between the understanding of central nervous system embryology, the discovery of the vitamin, the correlation between folic acid and cell proliferation and lastly the development of preventive measures for this type of defects. This narrative allows us to examine historically relevant concepts underlying clinical actions with a populational impact that prevent NTDs via folic acid consumption prior to conception.

Is it possible folic acid reduce anorectal malformations ethylenethiourea induced in rats?

PURPOSE: To investigate the effect of folic acid (FA) in an experimental model of anorectal malformations (ARMs) ethylenethiourea (ETU) induced.METHODS:Eight female Wistar rats were divided randomly in two groups. Group A - ETU; Group B - FA+ETU; Dams from group B received daily, since two weeks before pregnancy to the end of pregnancy, FA (50mg/kg) by gavage. Dams from groups A and B, received 1% ETU (125mk/kg) by gavage on gestational day (GD) 11. Their fetuses were harvested by cesarean section on GD21 and were examined looking for ARMs. The thickness of anal stratified squamous epithelium (ASSE) and intestinal epithelium (IE) were analyzed. p<0.05*.RESULTS:One hundred and one embryos were harvested. The number of embryos; number of ARMs; mean statistical % (± SD) were determined to be, respectively: ETU - 49 [30;65% (±24%)] versus FA+ETU - 52 [1;02% (±3%)] (p=0.025). AMRs were significantly lower in FA+ETU group than in ETU group (p=0.025). The thickness (µm) of ASSE (± SD) and IE (± SD) were measured, respectively: ETU - [27.75 (±0.56) and 18.88 (±0.93)] versus FA+ETU - [28.88 (±0.61) and 21.11 (±0.16)] (p=0.001). The thickness of IE was significantly enlarged when FA was given (p=0.001).CONCLUSION:Folic acid reduces the number and enlarged the IE of ARMs ETU-induced.

Effect of L-carnitine on exercise performance in patients with mitochondrial myopathy

Exercise intolerance due to impaired oxidative metabolism is a prominent symptom in patients with mitochondrial myopathy (MM), but it is still uncertain whether L-carnitine supplementation is beneficial for patients with MM. The aim of our study was to investigate the effects of L-carnitine on exercise performance in MM. Twelve MM subjects (mean age±SD=35.4±10.8 years) with chronic progressive external ophthalmoplegia (CPEO) were first compared to 10 healthy controls (mean age±SD=29±7.8 years) before they were randomly assigned to receive L-carnitine supplementation (3 g/daily) or placebo in a double-blind crossover design. Clinical status, body composition, respiratory function tests, peripheral muscle strength (isokinetic and isometric torque) and cardiopulmonary exercise tests (incremental to peak exercise and at 70% of maximal), constant work rate (CWR) exercise test, to the limit of tolerance [Tlim]) were assessed after 2 months of L-carnitine/placebo administration. Patients with MM presented with lower mean height, total body weight, fat-free mass, and peripheral muscle strength compared to controls in the pre-test evaluation. After L-carnitine supplementation, the patients with MM significantly improved their Tlim (14±1.9 vs 11±1.4 min) and oxygen consumption ( V ˙ O 2 ) at CWR exercise, both at isotime (1151±115 vs 1049±104 mL/min) and at Tlim (1223±114 vs 1060±108 mL/min). These results indicate that L-carnitine supplementation may improve aerobic capacity and exercise tolerance during high-intensity CWRs in MM patients with CPEO.

O que ocorre com o balanço hídrico durante e após a reversão do choque séptico? / What happens to the fluid balance during and after recovering from septic shock?

Objetivo: Avaliar o balanço hídrico acumulado durante o período do choque e determinar o que ocorre com ele nos 7 dias que se seguem à reversão do choque. Métodos: Estudo prospectivo e observacional, realizado em pacientes com choque séptico. Foram incluídos pacientes com pressão arterial média ≥ 65mmHg e lactato < 2,0mEq/L desmamados há menos de 12 horas do uso de vasopressores, sendo esse dia considerado o Dia 1. O balanço hídrico diário foi registrado por 7 dias após recuperação do choque. Os pacientes foram divididos em dois grupos, segundo a mediana da coorte para o balanço hídrico acumulado durante o período do choque: Grupo 1 ≤ 4,4L (n = 20) e Grupo 2 > 4,4L (n = 20). Resultados: Inscrevemos, neste estudo, um total de 40 pacientes. No Dia 1 do estudo, o balanço hídrico acumulado era de 1,1 [0,6 - 3,4] L no Grupo 1 e 9,0 [6,7 - 13,8] L no Grupo 2. No Dia 7 do estudo, o balanço hídrico acumulado era de 8,0 [4,5 - 12,4] L no Grupo 1 e 14,7 [12,7 - 20,6] L no Grupo 2 (p < 0,001 para ambos). A seguir, após a recuperação do choque, o balanço hídrico continuou a aumentar em ambos os grupos. Em comparação ao Grupo 1, o Grupo 2 teve um tempo mais longo de permanência na unidade de terapia intensiva e no hospital. Conclusão: São frequentemente observados balanços hídricos positivos em pacientes com choque séptico, o que pode estar relacionado a desfechos piores. Durante o período do choque, mesmo que o balanço hídrico fosse previamente positivo, este se torna ainda mais positivo. Após a recuperação do choque, o balanço hídrico continua a aumentar. Esse grupo com um balanço hídrico mais positivo permaneceu por mais tempo na unidade de terapia intensiva e no hospital. .

Objective: We aimed to evaluate the cumulative fluid balance during the period of shock and determine what happens to fluid balance in the 7 days following recovery from shock. Methods: A prospective and observational study in septic shock patients. Patients with a mean arterial pressure ≥ 65mmHg and lactate < 2.0mEq/L were included < 12 hours after weaning from vasopressor, and this day was considered day 1. The daily fluid balance was registered during and for seven days after recovery from shock. Patients were divided into two groups according to the full cohort’s median cumulative fluid balance during the period of shock: Group 1 ≤ 4.4L (n = 20) and Group 2 > 4.4L (n = 20). Results: We enrolled 40 patients in the study. On study day 1, the cumulative fluid balance was 1.1 [0.6 - 3.4] L in Group 1 and 9.0 [6.7 - 13.8] L in Group 2. On study day 7, the cumulative fluid balance was 8.0 [4.5 - 12.4] L in Group 1 and 14.7 [12.7 - 20.6] L in Group 2 (p < 0.001 for both). Afterwards, recovery of shock fluid balance continued to increase in both groups. Group 2 had a more prolonged length of stay in the intensive care unit and hospital compared to Group 1. Conclusion: In conclusion, positive fluid balances are frequently seen in patients with septic shock and may be related to worse outcomes. During the shock period, even though the fluid balance was previously positive, it becomes more positive. After recovery from shock, the fluid balance continues to increase. The group with a more positive fluid balance group spent more time in the intensive care unit and hospital. .

Evaluación del efecto del Compvit-B sobre la neuropatía periférica en pacientes con SCA2 en estadio ligero de la enfermedad / Evaluation of the effect of Compvit-B on peripheral neuropathy in patients with mild SCA2

INTRODUCCIÓN: la Ataxia Espinocerebelosa tipo 2 (SCA2) es una enfermedad neurodegenerativa severa que representa un serio problema de salud en Cuba, debido a las altas tasas de prevalencia e incidencia y a la ausencia de tratamientos curativos. OBJETIVOS: evaluar el efecto y la seguridad del tratamiento con altas dosis de vitaminas del complejo B (Compvit-B) sobre la neuropatía periférica en pacientes con SCA2. MÉTODOS: se realizó una investigación prospectiva de intervención clínica en 20 enfermos en estadio ligero los que se sometieron a un protocolo terapéutico mediante la administración intramuscular del COMPVIT B por 12 semanas. Durante las primeras 4 semanas los individuos recibieron dos bulbos semanales y a partir de la 5tasemana un solo bulbo. Inmediatamente antes y después del tratamiento los pacientes fueron evaluados mediantes exámenes clínicos y electrofisiológicos. RESULTADOS: una vez concluido el tratamiento, los pacientes mostraron un aumento significativo de las amplitudes de los potenciales de acción sensitivos de nervios mediano y sural, y en este último nervio se observó además reducción de la latencia y aumento de la velocidad de conducción. Los parámetros de la conducción nerviosa motora no se modificaron. Los potenciales evocados somatosensoriales de nervio mediano arrojaron una reducción significativa de la latencia del potencial de Erb. De manera interesante se observó una la disminución significativa de la frecuencia de aparición de las contracturas musculares dolorosas en el 53% de los casos después del tratamiento. Durante el estudio no se registraron eventos adversos. CONCLUSIONES: el presente estudio identifica una nueva opción terapéutica sintomática en la SCA2, brinda nuevas evidencias sobre las bases fisiopatológicas y el manejo clínico de las contracturas musculares dolorosas y justifican la realización de estudios más amplios en pacientes y portadores de la mutación, los que presentan tales manifestaciones muchos antes de debutar con la ataxia.

INTRODUCTION: Spinocerebellar ataxia type 2 (SCA2) is a severe neurodegenerative disease which constitutes a serious health problem in Cuba due to its high prevalence and incidence rates and the lack of curative treatments. OBJECTIVES: Evaluate the effect and safety of the treatment with high doses of B-complex vitamins (Compvit-B) on peripheral neuropathy in patients with SCA2. METHODS: A prospective clinical intervention study was conducted of 20 patients in the mild stage of the disease undergoing a therapeutic protocol consisting in intramuscular injection of Compvit-B for 12 weeks. Patients were administered two ampoules weekly in the first 4 weeks and one from the fifth week onwards. Immediately before and after the treatment patients underwent clinical and electrophysiological examination. RESULTS: Upon completion of the treatment patients showed a significant increase in the amplitude of the sensitive action potentials of the median and sural nerves. In the latter case there was also a decrease in latency and an increase in conduction velocity. Motor nerve conduction parameters were not modified. Somatosensory evoked potentials of the median nerve showed a significant reduction in the latency of Erb's potential. A significant decrease was also found in the frequency of painful muscle contractures in 53% of the cases after treatment. Adverse events were not recorded during the study. CONCLUSIONS: The study identifies a new therapeutic option for symptomatic SCA2, and provides new evidence of the pathophysiological bases and clinical management of painful muscle contractures. Broader studies should be conducted with patients and carriers of the mutation, who typically present such manifestations long before developing ataxia.

Evaluación preliminar del efecto del Compvit-B sobre procesos de memoria y aprendizaje en pacientes con SCA2 / Preliminary evaluation of the effect of Compvit-B on memory and learning processes in patients with SCA2

INTRODUCCIÓN: la ataxia Espinocerebelosa tipo 2 (SCA2) es una enfermedad neurodegenerativa que alcanza las mayores tasas de prevalencia e incidencia en Holguín, Cuba. Una de las principales manifestaciones clínicas de estos pacientes son los trastornos cognitivos, expresados fundamentalmente como déficits frontoejecutivos y de la memoria. OBJETIVO: evaluar el efecto del tratamiento con vitaminas del Complejo B sobre las funciones cognitivas, en pacientes cubanos con SCA2. MÉTODOS: se incluyeron 20 pacientes en una investigación de intervención clínica, empleando COMPVIT-B, durante 3 meses. Se evaluaron parámetros clínicos, como la escala SARA y cognitivos como el test de Stroop, el test de Fluencia verbal fonológica y el test de memoria verbal. Todos los estudios se realizaron antes y después del tratamiento. RESULTADOS: el estudio de las funciones frontoejecutivas reveló un aumento significativo del número de palabras mencionadas en el test de fluencia verbal fonológica, al terminar el estudio. Sin embargo, el test de Stroop no mostró cambios significativos. En relación al test de memoria verbal, se obtuvo un aumento del número de palabras recordadas en el primer ensayo, así como reducción del número de ensayos requeridos para recordar todas las palabras. La puntuación de la escala SARA no cambió significativamente. CONCLUSIONES: el presente trabajo constituye una evidencia adicional en favor del uso terapéutico y neuroprotector de las vitaminas del complejo B e identifica una nueva opción de tratamiento sintomatológico para los enfermos con SCA2, lo que incide positivamente en el mejoramiento de la calidad de vida de estos pacientes.

INTRODUCTION: Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease with the highest prevalence and incidence rates in the province of Holguín, Cuba. One of its main clinical manifestations is cognitive disorders, fundamentally expressed as frontal-executive and memory deficits. OBJECTIVE: Evaluate the effect of B-complex vitamins on cognitive functions in Cuban patients with SCA2. METHODS: Twenty patients were included in a clinical intervention study based on the use of Compvit-B for 3 months. An evaluation was conducted of clinical parameters such as the Scale for the Assessment and Rating of Ataxia (SARA), and cognitive parameters like the Stroop test, the phonological verbal fluency test and the verbal memory test. All the studies were conducted before and after the treatment. RESULTS: The study of frontal-executive functions revealed a significant increase in the number of words mentioned in the phonological verbal fluency test at the end of the study. However, the Stroop test did not show any significant change. The verbal memory test showed an increase in the number of words recalled in the first assay, and a reduction in the number of assays required to recall all the words. Scores on the SARA did not change significantly. CONCLUSIONS: The paper provides additional evidence in support of the therapeutic and neuroprotective use of B-complex vitamins and presents a new option of symptomatic treatment for patients with SCA2, which will lead to an improvement in their quality of life.

Eficacia del pirofosfato de tiamina o cocarboxilasa en el rescate del pie diabético / Efficacy of thiamine pyrophosphate or carboxylase in the salvage of diabetic foot

El pie diabético representa una de las complicaciones más comunes en pacientes que han tenido una larga evolución. La etiología se circunscribe a la neuropatía, infecciones e isquemia, que actuando en conjunto contribuyen a la secuencia de necrosis tisular, ulceración y gangrena. Lo difícil que resulta su tratamiento hace necesaria la búsqueda de opciones que colaboren en la resolución de esta problemática, que se centra en la hiperglucemia crónica como detonante. El pirofosfato de tiamina o cocarboxilasa realiza múltiples actividades metabólicas y no metabólicas que han sido consideradas importantes en la solución de las alteraciones del diabético por lo que en el presente trabajo se muestran los resultados al emplearlo en pacientes con pie diabético. En un período comprendido entre Enero de 1998 y Julio de 2012 se trataron 29 pacientes con pie diabético: 19 Wagner tipo III y 12 Wagner tipo IV. El manejo consistió en administrar antibióticos, procedimientos quirúrgicos parciales y pirofosfato de tiamina. Se logró el control del proceso infeccioso, la aparición de tejido de granulación y cicatrización de la lesión en un período comprendido entre 2 y 6 meses de acuerdo a la gravedad del problema. Por los datos clínicos y la evolución de los pacientes, se concluye que la administración del pirofosfato de tiamina permitió el control de las disfunciones metabólicas y no metabólicas que conducen a las complicaciones del diabético, por lo que se debe considerar una herramienta para el tratamiento de los diabéticos en general y para el rescate del pie diabético en particular.

Diabetic foot represents one of the most common complications in patients with a long standing disease. The etiology is neuropathy, infections and ischemia that together contribute to the sequence of tissue necrosis, ulceration and gangrene. Since treatment is very difficult, we must look for several options to solve these problems caused by chronic hyperglycemia. Thiamine pyrophosphate or carboxylase perform multiple metabolic and non-metabolic activities that are considered important in the resolution of diabetic impairments, therefore, this work shows the results when using it in patients with diabetic foot. 29 patients with diabetic foot were treated between January 1998 and July 2012: 19 Wagner type III and 12 Wagner type IV. Management was the administration of antibiotics, partial surgical procedures and thiamine pyrophosphate. The infectious process was controlled, the appearance of granulation tissue and scarring of the lesion in a period of 2 to 6 months depending on the severity of the problem. Given the clinical data and evolution of the patients, we conclude that the administration of thiamine pyrophosphate was able to control metabolic and non-metabolic dysfunctions that lead to complications in diabetic patients, therefore we must consider it a tool in the treatment of diabetic patients in general and for diabetic foot salvage in particular.

Emerging trends in management of propionic acidemia / Tendências emergentes no manejo da academia propiônica

Objetivo : To evaluate the therapeutic agents used during metabolic crises and in long-term management of patients with propionic acidemia (PA).Materials and methods : The records of PA patients were retrospectively evaluated.Results : The study group consisted of 30 patients with 141 admissions. During metabolic crises, hyperammonemia was found in 130 (92%) admissions and almost all patients were managed with normal saline, ≥ 10% dextrose, and restriction of protein intake. In 56 (40%) admissions, management was done in intensive care unit, 31 (22%) with mechanical ventilation, 10 (7%) with haemodialysis, 16 (11%) with vasopressor agents, and 12 (9%) with insulin. In the rescue procedure, L-carnitine was used in 135 (96%) patients, sodium bicarbonate in 116 (82%), sodium benzoate in 76 (54%), and metronidazole in 10 (7%), biotin in about one-quarter, L-arginine in one third, and antibiotics in three-quarter of the admissions. Blood/packed RBCs were used in 28 (20%) patients, platelets in 26 (18%), fresh frozen plasma in 8 (6%), and granulocyte-colony stimulating factors in 10 (7%) admissions. All patients were managed completely/partially with medical nutrition formula plus amino acid mixture, vitamins and minerals. For long-term management 24 (80%) patients were on L-carnitine, 22 (73%) on sodium benzoate, 6 (20%) on biotin, one half on alkaline therapy and 4 (13%) on regular metronidazole use. Almost all patients were on medical formula and regular follow-up.Conclusion : Aggressive and adequate management of acute metabolic crises with restriction of protein intake, stabilization of patient, reversal of catabolism, and removal of toxic metabolites are essential steps. Concerted efforts to ensure adequate nutrition, to minimize the risk of acute decompensation and additional therapeutic advances are imperative to improve the outcome of PA patients. Arq Bras Endocrinol Metab. 2014;58(3):237-42.

Objetivo : Avaliar os agentes terapêuticos usados durante as crises metabólicas e para o manejo de longo prazo de pacientes com academia propiônica (AP).Materiais e métodos : Avaliação retrospectiva das fichas médicas de pacientes com AP.Resultados : O grupo estudado consistiu de 30 pacientes com 141 hospitalizações. Durante as crises metabólicas, a hiperamonemia foi observada em 130 (92%) pacientes hospitalizados e quase todos foram tratados com solução salina regular, ≥ 10% dextrose e restrição da ingestão de proteína. Em 56 (40%) das hospitalizações, o manejo foi feito na unidade de terapia intensiva, 31(22%) com ventilação mecânica, 10 (7%) com hemodiálise, 16 (11%) com vasopressores e 12 (9%) com insulina. Para o resgate, a L-carnitina foi usada em 135 (96%) pacientes, o bicarbonato de sódio em 116 (82%), o benzoato de sódio em 76 (54%), o metronidazole em 10 (7%), a biotina em cerca de um quarto, a L-arginina em um quarto e antibióticos em três quartos dos pacientes hospitalizados. Sangue/concentrado de hemácias foram usados em 28 (20%), plaquetas em 26 (18%), plasma fresco congelado em 8 (6%) e fatores estimulantes de colônias de granulócitos em 10 (7%) pacientes hospitalizados. Todos os pacientes foram manejados completamente/parcialmente com fórmula de nutrição hospitalar mais uma mistura de aminoácidos, vitaminas e minerais. Para o manejo de longo prazo, 24 (80%) dos pacientes foram tratados com L-carnitina, 22 (73%) com benzoato de sódio, 6 (20%) com biotina, a metade com tratamento alcalino e 4 (13%) com uso regular de metronidazole. Quase todos os pacientes foram tratados com fórmulas médicas e acompanhamento regular.Conclusão : O manejo adequado e agressivo de crises metabólicas com restrição da ingestão de proteína, ...

Two Cases of Wernicke's Encephalopathy That Developed during Total Parenteral Nutrition in Colon Cancer Patients Treated with 5-Fluorouracil-based Chemotherapy / 대한소화기학회지

Wernicke's encephalopathy (WE) caused by thiamine deficiency is an acute neurological disorder. Clinically, the classic triad of WE consists of ophthalmoplegia, ataxia, and mental status changes. Thiamine deficiency is known to occur commonly in chronic alcoholic patients. Sometimes, it can occur in patients after gastrointestinal surgery and in those with malabsorption. In addition, patients undergoing renal dialysis, suffering from hyperemesis gravidarum, receiving total parenteral nutrition (TPN), and being treated with chemotherapeutic agents are also prone to develop thiamine deficiency. Herein, we report two cases of WE that developed following simultaneous 5-fluorouracil (5-FU) chemotherapy and TPN in colon cancer patients which was successfully treated with thiamine administration.