Cenário das políticas públicas para hemocromatose hereditária no Brasil / Public policy scenario for hereditary hemochromatosis in Brazil

Resumo Hemocromatose hereditária (HH) é a doença genética mais comum em descendentes de europeus e sua epidemiologia em nosso país é incerta. Considerando o cenário das políticas públicas em HH no mundo contemporâneo, este artigo propõe uma reflexão sobre o tema, com objetivo de fazer uma revisão bibliográfica narrativa sobre a abordagem adotada para essa doença em países desenvolvidos e a nível nacional. Além disso, discute sobre o custo-benefício da incorporação do índice de saturação da transferrina (ST) e ferritina sérica (FS) no nosso sistema de saúde, com a finalidade de identificar a HH antes que surjam suas complicações, bem como seu rastreio em campanhas nacionais de prevenção. O valor gasto para o screening da HH com dosagem de ST e FS pelo Sistema Único de Saúde (SUS) é muito menor do que os custos gerados quando o dano por excesso de ferro já está estabelecido. Nos casos suspeitos de HH, deveria ser viabilizada pelo SUS a pesquisa da mutação genética para o gene HFE, que atualmente só está disponível de forma privada. Com essas medidas, modifica-se a história natural da doença, reduzindo a morbimortalidade dos portadores e custos ao sistema público de saúde.

Abstract Hereditary hemochromatosis (HH) is the most common genetic disease among European descendants and its epidemiology in Brazil is unclear. Considering the contemporary public policy scenario aimed at HH, this narrative bibliographic review reflects on the approach adopted for this disease at the national level in developed countries. It also discusses the cost-effectiveness of incorporating transferrin saturation (TS) and serum ferritin (SF) indexes in the Brazilian healthcare system for early HH identification, as well as its screening in national prevention campaigns. The amount spent on ST- and FS-based HH screening by the Brazilian National Health System (SUS) is much lower than the costs generated by the already established iron overload. In suspected cases, genetic mutation research of the HFE gene, which is currently only performed privately, should be made available by the SUS. These measures can modify the natural history, reducing HH morbidity and mortality and its costs to the public health system.

Hemocromatosis no ligada al gen HFE: a propósito de un caso / Non-HFE hemochromatosis: a case report

RESUMEN La hemocromatosis hereditaria (HH) consiste en una sobrecarga progresiva de hierro que conlleva a un acúmulo anormal del mismo en diferentes órganos blancos; y, que, en caso de no tratarse a tiempo, puede causar una disfunción multi-orgánica. Se han descrito diversas mutaciones genéticas asociadas a la HH, la más frecuente de ellas es la asociada al gen-HFE, la cual se encuentra en el 90% de los casos. En la actualidad la flebotomía terapéutica continúa siendo el tratamiento de elección para el manejo de esta patología. Reportamos el caso de un paciente en seguimiento por fibrosis hepática severa, con persistencia de un perfil ferrocinético elevado, a quien cinco años después se le diagnostica una HH no asociada a una mutación en el gen-HFE; recibió manejo con flebotomías periódicas, presentando rápidamente una mejoría significativa de su cuadro clínico y de los niveles de ferritina al igual que otros paraclínicos.

ABSTRACT Hereditary hemochromatosis (HH) consists of a progressive iron overload that leads to an abnormal accumulation of iron in different target organs; and, if not treated in time, can cause multi-organ dysfunction. Various genetic mutations associated with HH have been described, the most frequent is associated with the HFE-gene, which is found in 90% of cases. At present, therapeutic phlebotomy continues to be the treatment of choice for the management of this pathology. We report the case of a patient under follow-up for severe liver fibrosis, with persistence of a high ferrokinetic profile, who five years later was diagnosed with HH not associated to a mutation in the HFE-gene; He was managed with periodic phlebotomies, rapidly presenting a significant clinical improvement and decrease of ferritin levels.

Detección de fallas en las pulseras identificatorias de pacientes internados / Inpatient identification wristbands failures detection

Resumen La atención médica no se encuentra libre de errores, que pueden poner en riesgo tanto la salud como la vida de los pacientes, con aumento de la morbimortalidad y los costos del sistema sanitario. Las recomendaciones internacionales de seguridad del paciente incluyen una correcta identificación. El uso de pulseras identificatorias reduce la cantidad de eventos adversos. Se investigó en forma retrospectiva la tasa de pacientes sin pulsera identificatoria al momento de la extracción sanguínea durante el año 2019 en el Instituto de Investigaciones Médicas Alfredo Lanari (IDIM). Se registró un total de 521 casos de pacientes no identificados sobre 5267 pedidos de laboratorio. La tasa anual de no identificados fue de 9.8%, superior al máximo ac eptable, y entre abril a septiembre se observaron mayores valores promedio. El día de la semana con mayor tasa de no identificados fueron los lunes con un 14%. Se observó una relación estadísticamente significativa entre la tasa mensual de no identificados con la cantidad mensual de egresos d e unidades operativas (r = 0.6465; p = 0.0237) y con el giro cama (r = 0.7776; p = 0.0029). Se desconoce si hubo otros errores de identificación. El estudio permitió conocer fallas en la identificación de pacientes internados. Se recomienda monitorear el indicador es pecialmente durante los meses con mayor cantidad de egresos y giro cama, evaluar la adherencia del personal al protocolo vigente y realizar capacitaciones para lograr una menor tasa de no identificados.

Abstract Medical attention is not free from committing mistakes that can increase mortality and costs. The International Goals for Patient Safety include correct patient identification. The use of wristbands reduces the number of adverse events. The rate of non-identified patients at the moment of phlebotomy was investigated retrospectively during 2019. The annual rate of non-identified patients was 9.8% and higher rates were observed from April to September. Monday was the day with the highest rate of non-identified. There was statistically significant relationship between the month rate of non-identified patients and the number of discharges from operative unit per month (r=0.6465; p=0.0237) and the bed turnover rate (r=0.7776; p=0.0029). Other wristband identification errors are unknown. The investigation detected failures in patient identification that allowed to make recommendations. In order to reduce the number of missing wristbands it will be necessary to monitor the indicator, especially during the months with the highest number of discharges and bed turnover rate, to evaluate the adherence of the personnel to the current protocol and to carry out training programs.

Acceso venoso difícil en pediatría / Difficult venous access in pediatrics

BACKGROUND: Cannulation of a peripheral venous access is a routine procedure in pediatric patients admitted to the hospital. 50% of the time cannulation on the first attempt is not feasible, so it is necessary to repeat the puncture, resulting in a complex and frustrating procedure. Half of the children admitted to the hospital have a difficult venous access (DIVA). OBJECTIVE: To carry out a review, which provides information about DIVA in pediatrics, how to evaluate and proceed in these patients. DESIGN: DIVA Score considers vein palpability, vein visibility, age and history of prematurity. The score ranges from 0 to 10 points. Values greater than 4 are associated with difficult venous access. There are associated risk factors: obesity, musculoskeletal malformations, chemotherapy treatment, diabetes mellitus, patients on dialysis, limb edema, moderate to severe dehydration, history of difficult venous access, anxiety of the patient and/or parents report that the child is less likely to cooperate. When making the decision to establish a venous access, it should be evaluated whether it is an emergency or not, the characteristics of the medications and infusions, the time of therapy and the anatomical sites for puncture. Ultrasound and transillumination techniques decrease the time to obtain a venous access and increase the success rate on the first attempt. CONCLUSION: The decision to obtain a venous access must take into account the criteria and risk factors for DIVA. The most recognized scale is the DIVA Score.

INTRODUCCIÓN: La canulación de un acceso venoso periférico es un procedimiento rutinario en los pacientes pediátricos que ingresan al hospital. 50% de las veces la canulación al primer intento es frustra, por lo que es necesario repetir la punción resultando el procedimiento complejo y frustrante. La mitad de los niños que ingresa al hospital presentan un acceso venoso difícil (DIVA, sigla derivada de "difficult intravenous access"). OBJETIVO: Realizar una revisión, que entrega información acerca de DIVA en pediatría, cómo evaluar y proceder en estos pacientes. DESARROLLO: DIVA Score considera palpabilidad y visibilidad venosa, edad y antecedente de prematurez. El puntaje va desde 0 a 10 puntos. Valores mayores a 4 se asocian a acceso venoso difícil. Existen factores de riesgo asociados: obesidad, malformaciones osteomusculares, tratamiento con quimioterapia, diabetes mellitus, pacientes en diálisis, edema de extremidades, deshidratación moderada a severa, historia de acceso venoso difícil, ansiedad del paciente y/o padres que refieren que el niño es poco probable que coopere. Al tomar la decisión de establecer un acceso venoso se debe evaluar si es urgencia o no, las características de los medicamentos e infusiones, tiempo de terapia y los sitios anatómicos para punción. La ultrasonografía y las técnicas de transiluminación disminuyen el tiempo de obtención del acceso venoso e incrementan la tasa de éxito en el primer intento. CONCLUSIÓN: En la decisión de obtener un acceso venoso se deben tener en cuenta los criterios y factores de riesgo de acceso venoso difícil. La escala más reconocida es el DIVA Score.

Aspectos relevantes en hemocromatosis hereditaria / Relevant features in hereditary hemochromatosis

La hemocromatosis hereditaria es una enfermedad que se caracteriza por la sobrecarga sistémica de hierro y se asocia a múltiples mutaciones genéticas que conducen a una producción inadecuadamente baja de la hormona hepcidina o a una alteración en la unión de la hepcidina a la ferroportina. Esto tiene como resultado un aumento de la absorción intestinal y el depósito de cantidades excesivas de hierro en las células, lo cual, a su vez, si no se corrige, genera daño tisular. La expresión clínica puede variar desde individuos completamente asintomáticos, hasta pacientes con cirrosis hepática a temprana edad, y eventualmente carcinoma hepatocelular. Habitualmente, el diagnóstico no es invasivo e incluye el examen clínico, la evaluación de los parámetros de hierro plasmático, imágenes y pruebas genéticas. El principal tratamiento es la flebotomía, pero terapias alternativas como la suplementación con hepcidina son un tema de investigación actual.

Hereditary hemochromatosis is a disease characterized by systemic iron overload of genetic origin, that leads to an inadequately low production of the hormone hepcidin or a reduction in hepcidinferroportin binding. This results in an increased intestinal absorption and the deposit of excessive amounts of iron in cells, which in turn results in tissue damage if not treated. The clinical expression can vary from completely asymptomatic individuals, to patients with liver cirrhosis at an early age, and eventually hepatocellular carcinoma. Diagnosis is usually noninvasive and includes clinical examination, assessment of plasma iron levels, imaging studies, and genetic testing. The main medical treatment is phlebotomy, but alternative therapies such as hepcidin supplementation are the subject of current research.

Potency of remimazolam in inhibiting ventilatory function during sedation in patients undergoing internal jugular vein puncture / 中华麻醉学杂志

Objective:To determine the potency of remimazolam in inhibiting ventilatory function during sedation in the patients undergoing internal jugular vein puncture.Methods:American Society of Anesthesiologists physical status Ⅰ or Ⅱ patients of either sex, aged 18-64 yr, with body mass index<30 kg/m 2, undergoing elective internal jugular vein puncture under local anesthesia, were enrolled in this study.Remimazolam was intravenously injected, and the dose was determined by up-and-down sequential allocation.The initial dose was 0.07 mg/kg and was increased/decreased in the next patient depending on whether the patients showed inhibition of ventilatory function, and the ratio between the 2 successive doses was 1.2.The criterion for inhibition of ventilatory function was defined as Integrated Pulmonary Index < 5.The median effective dose (ED 50) and 95% confidence interval of remimazolam in inhibiting ventilatory function were calculated by Dixon-Massey method. Results:Thirty-six patients were enrolled in this study.The ED 50 (95% confidence interval) was 0.106 (0.097-0.115) mg/kg. Conclusion:The ED 50 of remimazolam in inhibiting ventilatory function during sedation in internal jugular vein puncture is 0.106 mg/kg.

Impacto del volumen de sangre extraído por flebotomía sobre el requerimiento transfusional en prematuros menores de 1500 g. Estudio cuasiexperimental / Impact of the volume of blood collected by phlebotomy on transfusion requirements in preterm infants with birth weight of less than 1500 g. A quasi-experimental study

Introducción. La anemia es una complicación para los recién nacidos de muy bajo peso al nacer, y los exámenes de laboratorio son un factor de riesgo preponderante. Más del 50 % recibe, al menos, una transfusión de glóbulos rojos. Estas se han asociado a mayor riesgo de infecciones, hemorragia intracraneal, enterocolitis necrotizante y displasia broncopulmonar. En 2012, se implementó, en el Hospital Italiano de Buenos Aires, una estrategia de menor volumen de extracción de sangre por flebotomía. El objetivo del presente estudio fue evaluar su asociación con el número detransfusiones.Métodos. Estudio cuasiexperimental del tipo antes/después. Se comparó el número de transfusiones entre dos grupos de prematuros de muy bajo peso con diferente volumen de extracción. Se evaluó la correlación entre el volumen extraído y el número de transfusiones estimando el coeficiente de Spearman. Para ajustar por confundidores, se realizó un modelo de regresión logística.Resultados. Se incluyeron en el estudio 178 pacientes con edad gestacional media de 29,4 semanas (desvío estándar: 2,7) y peso al nacer de 1145 gramos (875-1345). El perfil de la serie roja inicial fue similar entre ambos grupos. El número de transfusiones (p = 0,017) y el volumen transfundido (p = 0,048) disminuyeron significativamente. El coeficiente de correlación resultó de 0,83. En el análisis multivariado, volumen de extracción y peso al nacer se asociaron a un requerimiento mayor de 3 transfusiones.Conclusión. Un menor volumen de extracción de sangre en prematuros de muy bajo peso está asociado de manera independiente a menor requerimiento transfusional.

Introduction. Anemia is a complication in very low birth weight (VLBW) infants, and lab tests are a predominant risk factor. At least one red blood cell transfusion is given in more than 50 % of cases. Transfusions are associated with a higher risk for infections, intracranial hemorrhage, necrotizing enterocolitis, and bronchopulmonary dysplasia. In 2012, Hospital Italiano de Buenos Aires implemented a strategy to collect a lower blood volume by phlebotomy. The objective of this study was to assess its association with the number of transfusions.Methods. Before-and-after, quasi-experimental study. The number of transfusions was compared between two groups of VLBW preterm infants with different blood collection volumes. The correlation between the collection volume and the number of transfusions was assessed estimating Spearman's coefficient. A logistic regression model was used to adjust for confounders.Results. The study included 178 patients with a mean gestational age of 29.4 weeks (standard deviation: 2.7) and a birth weight of 1145 g (875-1345). The baseline red series profile was similar between both groups. The number of transfusions (p = 0.017) and the transfusion volume (p = 0.048) decreased significantly. The correlation coefficient was 0.83. In the multivariate analysis, collection volume and birth weight were associated with a requirement of more than three transfusions.Conclusion. A lower blood collection volume in VLBW preterm infants is independently associated with fewer transfusion requirements.

Avaliação da técnica de aperto de punho como interferente pré-analítico na dosagem do potássio / Evaluation of the wrist tightening technique as a pre-analytical interference in the potassium dosage

Objetivo: avaliar as alterações do potássio em relação a frequência de aperto de punho pelos participantes da pesquisa, buscando contribuir para um melhor entendimento sobre os aspectos quantitativos em relação ao uso da técnica de aperto de punho durante a flebotomia, para verificar com que frequência o aperto de punho poderá contribuir para a elevação do potássio. Método: Foram realizadas duas coletas sanguíneas de cada indivíduo. Em um primeiro momento, mantendo as mãos relaxadas durante flebotomia e, segundo, bombeando o punho durante o procedimento de flebotomia para avaliar as alterações do potássio em relação a frequência de aperto de punho. Resultados: De um modo geral, observa-se que houveram alterações em praticamente todas as dosagens e que indivíduos poderiam ter sido diagnosticados incorretamente com uma hipercalemia, podendo ser submetidos a tratamentos desnecessários. Conclusão: A técnica de aperto de punho durante a flebotomia deve ser evitada, pois uma significativa porcentagem dos casos apresentou uma elevação de potássio não condizente com o nível sérico de potássio normal do indivíduo.

Objective: To evaluate potassium alterations in relation to wrist grip frequency by the research participants, seeking to contribute to a better understanding of the quantitative aspects regarding the use of wrist grip technique during phlebotomy, to verify how often wrist may contribute to potassium elevation. Method: Two blood samples were collected from each individual. At first, keeping the hands relaxed during phlebotomy and secondly, pumping the fist clenching the phlebotomy procedure to evaluate potassium changes in relation to the frequency of wrist tightening. Results: In general, it was observed that there were changes in practically all dosages and that individuals could have been misdiagnosed with hyperkalemia and could be submitted to unnecessary treatments. Conclusion: The technique of wrist tightening during phlebotomy should be avoided, since a significant percentage of the cases presented a potassium elevation that does not correspond to the individual's normal serum potassium level.

Treatment of iron overload syndrome: a general review

SUMMARY INTRODUCTION Iron overload is a broad syndrome with a large spectrum of causative etiologies that lead to iron deposition. When iron exceeds defenses, it causes oxidative damage and tissular disfunction. Treatment may prevent organ dysfunction, leading to greater life expectancy. METHODS Literature from the last five years was reviewed through the use of the PubMed database in search of treatment strategies. DISCUSSION Different pharmacological and non-pharmacological strategies are available for the treatment of iron overload and must be used according to etiology and patient compliance. Therapeutic phlebotomy is the basis for the treatment of hereditary hemochromatosis. Transfusional overload patients and those who cannot tolerate phlebotomy need iron chelators. CONCLUSION Advances in the understanding of iron overload have lead to great advances in therapies and new pharmacological targets. Research has lead to better compliance with the use of oral chelators and less toxic drugs.

RESUMO INTRODUÇÃO A síndrome de sobrecarga de ferro engloba um grande espectro de etiologias que levam a um aumento da quantidade de ferro nos tecidos. Esse ferro excede a capacidade de proteção dos tecidos, levando a dano oxidativo e lesão tissular. Tratamento pode prevenir esse dano, levando à melhor sobrevida. METODOLOGIA A literatura dos últimos cinco anos foi revisada por meio de pesquisa na base de dados PubMed buscando identificar estratégias de tratamento. DISCUSSÃO Medidas farmacológicas e não farmacológicas estão disponíveis para o tratamento da síndrome de sobrecarga de ferro e devem ser utilizadas de acordo com a etiologia e a aceitação do paciente. A flebotomia terapêutica é base do tratamento dos pacientes com hemocromatose hereditária. Pacientes com sobrecarga transfusional ou aqueles que não toleram flebotomias devem utilizar quelantes de ferro. CONSIDERAÇÕES FINAIS Avanços no entendimento da síndrome de sobrecarga de ferro têm levado a grandes progressos na terapêutica, com promessas de abordagem de novos alvos farmacológicos. A evolução da pesquisa tem possibilitado melhor aderência com o uso de quelantes orais e com possibilidade de drogas menos tóxicas.

Indications of percutaneous umbilical cord blood sampling in prenatal diagnosis / 中华围产医学杂志

Objective@#To investigate the tendency and safety of percutaneous umbilical cord blood sampling (PUBS) in prenatal screening and diagnosis, and the possibilities of avoiding unnecessary PUBS.@*Methods@#This was a retrospective study of pregnant women who underwent PUBS for prenatal diagnosis in Peking University First Hospital from January 2015 to December 2017. Clinical indications, timing of PUBS, further investigations (chromosome karyotype, molecular genetics and pathogen testing), results, and pregnancy outcomes were collected and analyzed. One-way analysis of variance (ANOVA), Chi-square test for linear trend, Fisher's exact probability test/Cochran-Armitage analysis and Cruskal-Wallis rank-sum test were used for statistical analysis.@*Results@#(1) A total of 412 singleton pregnancies underwent PUBS at 20-38 gestational weeks during the study period, and 379 (92.2%) of them received PUBS before 34 gestational weeks. The positive test results accounted for 10.4% (43/412). There were six (1.5%) miscarriages after PUBS. In vitro cell culture failure occurred in two cases, one in 2015 and the other in 2016. (2) Among the 412 cases, 304 (73.8%) had only one indication. Fourteen cases could be identified as high risk in the first trimester, such as advanced maternal age (AMA, >35 years), pregnant history with chromosomal abnormal fetus and one of the couples carrying abnormal genes. There were four, zero and one case receiving PUBS only for AMA in 2015, 2016 and 2017, respectively. Indications, including high risk suggested by serum screening and fetal abnormality found by ultrasound were identified in 290 cases (70.4%) in the second or third trimester. Other than AMA, there were no statistically significant differences in single indicators. The proportion PUBS with double indicators increased from 2015 to 2017 but without significant difference. AMA and positive serum screening as indicators of aneuploidy screening accounted for 7.6% (8/105) in double-indicator group and 1.9% (8/412) in all. (3) There were 363 PUBS (88.1%) performed for ultrasound abnormalities. Among them, 76.9% (280/363) only had abnormal ultrasound findings, and the percentage was decreased year by year. The other 83 cases (80 with double indicators and three with triple indicators) also presented with other indicators, including AMA, adverse pregnancy history and positive serum screening. The proportion of PUBS performed with the presence of multiple indicators tended to increase recently, but no statistically significant difference was found. All the 18 cases with abnormality diagnosed by molecular genetic testing had abnormal ultrasound findings.@*Conclusions@#Although PUBS's complications are rare, it carries some risks. The constitution of single indication has been declined every year. With the improvement of prenatal screening system and application of molecular karyotyping, the necessity of invasive prenatal diagnosis with PUBS is greatly reduced. An improvement in reasonable and standardized application of PUBS needs to be achieved.

Indications of percutaneous umbilical cord blood sampling in prenatal diagnosis / 中华围产医学杂志

Objective To investigate the tendency and safety of percutaneous umbilical cord blood sampling (PUBS) in prenatal screening and diagnosis,and the possibilities of avoiding unnecessary PUBS.Methods This was a retrospective study of pregnant women who underwent PUBS for prenatal diagnosis in Peking University First Hospital from January 2015 to December 2017.Clinical indications,timing of PUBS,further investigations (chromosome karyotype,molecular genetics and pathogen testing),results,and pregnancy outcomes were collected and analyzed.One-way analysis of variance (ANOVA),Chi-square test for linear trend,Fisher's exact probability test/Cochran-Armitage analysis and Cruskal-Wallis rank-sum test were used for statistical analysis.Results (1)A total of 412 singleton pregnancies underwent PUBS at 20-38 gestational weeks during the study period,and 379 (92.2％) of them received PUBS before 34 gestational weeks.The positive test results accounted for 10.4％ (43/412).There were six (1.5％) miscarriages after PUBS.In vitro cell culture failure occurred in two cases,one in 2015 and the other in 2016.(2) Among the 412 cases,304 (73.8％) had only one indication.Fourteen cases could be identified as high risk in the first trimester,such as advanced maternal age (AMA,＞35 years),pregnant history with chromosomal abnormal fetus and one of the couples carrying abnormal genes.There were four,zero and one case receiving PUBS only for AMA in 2015,2016 and 2017,respectively.Indications,including high risk suggested by serum screening and fetal abnormality found by ultrasound were identified in 290 cases (70.4％) in the second or third trimester.Other than AMA,there were no statistically significant differences in single indicators.The proportion PUBS with double indicators increased from 2015 to 2017 but without significant difference.AMA and positive serum screening as indicators of aneuploidy screening accounted for 7.6％ (8/105) in double-indicator group and 1.9％ (8/412) in all.(3) There were 363 PUBS (88.1％) performed for ultrasound abnormalities.Among them,76.9％ (280/363) only had abnormal ultrasound findings,and the percentage was decreased year by year.The other 83 cases (80 with double indicators and three with triple indicators) also presented with other indicators,including AMA,adverse pregnancy history and positive serum screening.The proportion of PUBS performed with the presence of multiple indicators tended to increase recently,but no statistically significant difference was found.All the 18 cases with abnormality diagnosed by molecular genetic testing had abnormal ultrasound findings.Conclusions Although PUBS's complications are rare,it carries some risks.The constitution of single indication has been declined every year.With the improvement of prenatal screening system and application of molecular karyotyping,the necessity of invasive prenatal diagnosis with PUBS is greatly reduced.An improvement in reasonable and standardized application of PUBS needs to be achieved.

Blood-Letting Therapy for Hypertension: A Systematic Review and Meta-Analysis of Randomized Controlled Trials / 中国结合医学杂志

OBJECTIVE@#To evaluate the efficacy and safety of blood-letting therapy (BLT) in treatment of hypertension.@*METHODS@#A comprehensive electronic and manual bibliographic searches were performed in Cochrane Central Register of Controlled Trials, Excerpt Medica Database (EMBASE), PubMed, China National Knowledge Infrastructure, Chinese Scientific Journal Database, Chinese Biomedical Literature Database, and Wanfang Database to identify randomized controlled trials (RCTs) in which hypertensive patients were treated with BLT or BLT plus antihypertensive drugs (BPAD) against placebo, no treatment or antihypertensive drugs. The Cochrane Risk Assessment Tool was used to assess the methodological quality of trials. The Review Manager 5.3 software was used for meta-analysis.@*RESULTS@#A total of 7 RCTs with 637 hypertensive patients from 1989 to 2017 were identified. Compared with antihypertensive drugs, blood pressure was significantly reduced by BLT (RR=1.21, 95% CI: 1.01 to 1.44, P=0.03; heterogeneity: P=0.06, I=60%) and BPAD (RR=1.25, 95% CI, 1.02 to 1.53, P=0.03; heterogeneity: P= 0.01, I=71%). Moreover, a significant improvement in Chinese medicine syndrome by BLT (RR=1.32; 95% CI: 1.14 to 1.53, P=0.0002; heterogeneity: P=0.53, I=0%) and BPAD (RR=1.47; 95% CI: 1.06 to 2.04, P=0.02; heterogeneity: P=0.13, I=56%) was identified. The reported adverse effects were well tolerated.@*CONCLUSION@#Although some positive findings were identified, no definite conclusions regarding the efficacy and safety of BLT as complementary and alternative approach for treatment of hypertension could be drew due to the generally poor methodological design, significant heterogeneity, and insufficient clinical data. Further rigorously designed trials are warranted to confirm the results.

Perfil dos pacientes submetidos à sangria terapêutica na região sul de Santa Catarina atendidos em um consultório privado de hematologia / Profile of patients submitted to therapeutic bloodletting in the south of Santa Catarina served in a private hematology consultory

A sangria terapêutica é um procedimento hemoterápico de simples realização, eficaz quando indicada corretamente. Atualmente, as principais doenças que se beneficiam com este tratamento são as hiperferritinemias e as eritrocitoses. O presente estudo teve como objetivo principal conhecer o perfil dos pacientes submetidos à sangria terapêutica em um consultório privado de hematologia, localizado na região sul de Santa Catarina. Trata-se de estudo observacional e retrospectivo, com análise quantitativa de dados secundários. Todos os 113 pacientes identificados compuseram a amostra final. As indicações de sangria mais prevalentes foram hiperferritinemia secundária (46,9%) e hiperferritinemia com mutação do gene HFE (44,2%). A média de idade dos pacientes foi de 53,12 anos, sendo a maioria composta por homens (84,1%), todos da raça branca, e 31% procedentes de Criciúma. Destes pacientes, 77,9% realizaram estudo genético, o qual mostrou 56,8% positivos para hiperferritinemia com mutação do gene HFE, 6,8% para o gene da policitemia vera e 36,4% com resultado negativo. Ao comparar os índices laboratoriais antes e após a sangria terapêutica, observouse redução estatisticamente significativa nos valores de ferritina sérica e saturação de transferrina nos casos de hiperferritinemia com mutação do gene HFE e de ferritina sérica para hiperferritinemia secundária. Conclui-se que o perfil dos pacientes submetidos à sangria terapêutica na região sul de Santa Catarina, no período estudado, é composto principalmente por homens, acima de 50 anos de idade, e que a indicação mais prevalente para o tratamento são as sobrecargas de ferro.

Therapeutic bloodletting is a haemotherapic procedure of easy execution and effective when indicated correctly. Currently, the main diseases that benefit from this treatment are the iron overload syndromes and erythrocytosis. The main objective of this study was to know the profile of patients submitted to bloodletting therapy in a private hematology practice, located in the southern region of Santa Catarina. It is an observational and retrospective study with quantitative analysis of secondary data. All the 113 patients identified have composed the final sample. The most prevalent phlebotomy indications were secondary iron overload (46.9%) and iron overload with mutation of the HFE gene (44.2%). The average age of the patients was 53.12 years, most of which were men (84.1%), all of the white race and 31.0% of Criciúma. Of these patients, 77.9% performed genetic study, being 56.8% positive for iron overload with mutation of the HFE gene, 6.8% for the polycythemia vera gene and 36.4% showed negative result. By comparing the laboratory indices before and after the therapeutic bloodletting, significant reduction was observed in the values of serum ferritin and transferrin saturation in the iron overload with mutation of the HFE gene cases. About secondary iron overload, it was observed significant reduction only in serum ferritin. It's concluded that the profile of patients who are submitted to therapeutic bloodletting in the southern region of Santa Catarina, in the studied period, is composed mainly of men, above 50 years of age and the most prevalent indication for treatment are the iron overloads.

Pediatric Deaths and Venipuncture / 대한법의학회지

Venipuncture is a routine and relatively safe and painless medical procedure, necessary for accurate diagnosis and treatment. However, given that pain related to medical procedures may have adverse effects for children, could venipuncture cause deaths in infants and children? We analyzed our cases of unexpected death after venipuncture and conducted a literature review on them. A vasovagal response to noxious stimuli may explain such a sudden death immediately after venipuncture, commonly presented as needle phobia, breath-holding spell, and reflex anoxic seizure in the literature, despite the fact that the current medical evidence is not enough to prove their casual relationship. In addition, pain prevention and management during medical procedure is incorporated in clinical guidelines for pediatric patients, because painful medical procedures negatively affect child development. Thus, prevention and management of pain related to medical procedure may be helpful to avoid an adverse vasovagal response to noxious stimuli from occurring.

Anatomical factor and risk assessment of right internal jugular vein puncture-related damage to vertebral artery at different neck planes in pediatric patients / 中华麻醉学杂志

Objective To evaluate the anatomical factor and risk assessment of right internal jugular vein (IJV) puncture-related damage to the vertebral artery (VA) at different neck planes in pediatric patients.Methods Two hundred and ten pediatric patients of both sexes,aged 6 months-10 yr,with body mass index less than 28 kg/m2,undergoing elective surgery,were enrolled in this study.At the cricoid cartilage plane,supraclavicular area plane and intermediate plane,the right IJVs and VAs were examined using ultrasound.The VA position relative to the IJV,diameters of IJVs and VAs (the diameter ratio of VAs to IJVs was calculated),extent of overlap between IJVs and VAs,and horizontal and vertical distance from VAs to IJVs were recorded,and the risk coefficient of accidental VA puncture was calculated.Results Ninety-seven percent of VAs lay deep and lateral to right IJVs.There was no significant difference in each parameter of VA position relative to IJVs between the three planes (P＞0.05).The diameter ratio of VAs to IJVs was decreased with the decreasing neck plane,the horizontal and vertical distance from VAs to IJVs was significantly shortened,the overlapping rate between VAs and IJVs was increased,and the risk coefficient of accidental VA puncture was increased (P＜0.05 or 0.01).The vertical distance from VAs to IJVs was not correlated with age,body weight or height (P＞0.05).The risk coefficient of VA damage was not correlated with age,body weight or height at the cricoid cartilage plane and intermediate plane (P ＞ 0.05).The risk coefficient of VA damage was positively correlated with the weight of pediatric patients at the supraclavicular area plane (P＜0.05,r=0.215).Conclusion Right VAs come nearer IJVs with the decreasing neck plane;the risk of VA damage increases gradually with the lowering of neck planes in pediatric patients.

Habilidades teóricas y prácticas para flebotomía entre alumnos de primer semestre de medicina en el Centro de Simulación Montagne, Mérida México / Theoretical and practical flebotomy skills among first-semester medicine students at the Montagne Simulation Center, Mérida México

Introducción. El uso de simuladores de pacientes humanos es una estrategia eficaz en la enseñanza de procedimientos clínicos. Sin embargo, existe poca información para ayudar a los profesores a mejorar sus estrategias docentes permitiéndoles mejorar las experiencias de aprendizaje de acuerdo a la edad o el sexo de los alumnos que emplean este tipo de simulación. Objetivos. Identificar si existen diferencias por sexo o edad en asociación a las habilidades de los alumnos del primer semestre de medicina para la flebotomía. Diseño. Cuasiexperimental. Lugar. Centro de Simulación Médica Montagne de la Universidad Marista de Mérida. Participantes. Estudiantes de primer semestre de la licenciatura en medicina. Intervenciones. Intervención educativa del proceso de flebotomía con toma de muestra, empleando simuladores de alta fidelidad. Para el análisis, se evaluó la asociación de las variables de edad y sexo con el desempeño de los alumnos. Principales medidas de los resultados. Se analizaron las evaluaciones prácticas y teóricas. Para la evaluación práctica se aplicó una rúbrica de 20 ítems y se promediaron las puntuaciones de las esferas teóricas y prácticas de los alumnos. Resultados. Se identificó que las mujeres desempeñaron mejor sus habilidades prácticas (p<0,03) aún en el modelo de regresión lineal ajustado por edad y grupo. Discusión. El mejor desempeño en la práctica de flebotomía entre las alumnas de medicina difícilmente sea un hallazgo aislado, pues puede derivarse de las habilidades para la comunicación interpersonal, misma que también puede ser reforzada en los alumnos del sexo masculino.

Introduction. The use of human patient simulators is an effective strategy in the teaching of clinical procedures. However, there is little information to help teachers improve their teaching strategies, allowing them to improve learning experiences according to the age or sex of students who use this type of simulation. Objectives. To identify if there are differences by sex or age in association with the skills of the students of the first semester of medicine for phlebotomy. Design. Quasiexperimental. Places. Medical Simulation Centre Montagne of the Marista University of Merida. Participants. First semester medical students. Interventions. Educational intervention of the phlebotomy process with sample taking, and using high fidelity simulators. For the analysis, sociodemographic variables were associated with the performance of the students. Main result measures. The practical and theoretical evaluations were analyzed. For the practical evaluation, a rubric of 20 items was applied and the scores of the theoretical and practical areas of the students were averaged. Results. It was identified that women performed their practical skills better (p <0.03) even in the linear regression model adjusted for age and group. Discussion. The best performance in the practice of phlebotomy among medical students is hardly an isolated finding, since they could derive from the skills for interpersonal communication, which can also be reinforced in male students.

Ultrasound-guided percutaneous transhepatic thrombolysis for treatment of portal vein thrombosis after liver transplantation / 中国医师杂志

Objective To evaluate the effective and safety of ultrasound-guided percutaneous portal vein guide wire placement adjunct to thrombolytic catheter,which treating portal vein thrombosis after liver transplantation.Methods From Jan 2012 to Dec 2015,a total of 6 patients (5 male,1 female,average age 50.6 years old,age range 41-65 years old) with portal vein thrombosis after liver transplantation were retrospectively studied.The diagnosis was confirmed by contrast enhanced ultrasound (CEUS) with hypoechonic and no enhancement in portal vein.With ultrasound-guided a 18-guage guide wire was placed in right branch of portal vein,and a guidewire was placement.After exchanging the catheter,the thrombosis was confirmed again by venography.A thrombolytic catheter was placed and local thrombolysis therapy was performed.Results The guidewires were successfully placed in 6 patients.The thrombolytic catheters were successfully placed in 5 patients (day 2-60 after operation),and failed in 1 patient (9 years after operation).With 5-11 days urokinase injection,the patency of portal vein was found in 5 patients,of which 4 patients was treated by angioplasty and stent placement.With 16-31 months follow-up,the patency of portal vein was maintained.Neither server complication nor related-death was occurred.Conclusions Ultrasound-guided percutaneous portal vein guide wire placement adjuncts thrombolytic catheter is effective and safety for treating portal vein thrombosis after liver transplantation.

Application of Vein Infrared Imaging instrument in the treatment of medical patients with difficulty of vein-detaining needle / 中国实用护理杂志

Objective To investigate the application of Vein Infrared Imaging instrument in the treatment of medical patients with difficulty of vein-detaining needle. Methods A total of 116 cases of patients with difficulty of vein-detaining needle were admitted to Peking Union Medical College Hospital International Medical Service Department from June 2015 to May 2016. These participants were divided into vein imaging and conventional puncture group by the punctures method, 58 cases in each group. Vein imaging group applied vein imaging device in the whole process of venous indwelling needle, including vascular assessment before operation, real-time imaging and confirmation, the control group used conventional technology in peripheral venous catheter. The success rate of vein-detaining needle, operation time, puncture tube material cost, the incidence rate of venous indwelling needle complications were compared between two groups of patients. Results One-time success rate of vein imaging group reached 72.4% (42/58), puncture success rate reached 93.1% (54/58), higher than in conventional puncture group, which was 48.3% (28/58), 67.2% (39/58),the difference between the two groups had statistical significance(χ2=7.061, 10.629,P<0.05). Conventional puncture group 's operation time was (8.4±4.2) min,and puncture tube material cost was(123.37±62.20)yuan;vein imaging group respectively were (3.5±1.2) min, (67.50±24.82) yuan, the difference between the two groups had statistical significance (t=5.410, 4.057, P<0.05). Conventional puncture group's rate of complications was 41.4%(24/58), and vein imaging group was 17.2%(10/58), the difference between the two groups had statistical significance (χ2=8.155,P=0.004). Conclusions Application of vein infrared imaging instrument is a practical, safe and rapid method of vein-detaining needle, which can improve the work efficiency of nurses, and have more advantages in the treatment of patients with difficulty of vein-detaining needle.

Ultrasonographic Study of the Anatomical Relationship Between the Lateral Antebrachial Cutaneous Nerve and the Cephalic Vein

OBJECTIVE: To define the anatomy of the lateral antebrachial cutaneous nerve (LABCN) and the cephalic vein (CV) in the anterior forearm region of living humans using ultrasonography for preventing LABCN injury during cephalic venipuncture. METHODS: Thirty forearms of 15 healthy volunteers were evaluated using ultrasonography to identify the point where the LABCN begins to contact with the CV, and the point where the LABCN separates from the CV. The LABCN pathway in the forearm in relation to a nerve conduction study was also evaluated. RESULTS: The LABCNs came in contact with the CV at a mean of 0.6±1.6 cm distal to the elbow crease, and separated from the CV at a mean of 7.0±3.4 cm distal to the elbow crease. The mean distance between the conventionally used recording points (point R) for the LABCN conduction study and the actual sonographic measured LABCN was 2.4±2.4 mm. LABCN usually presented laterally at the point R (83.3%). CONCLUSION: The LABCN had close proximity to the CV in the proximal first quarter of the forearm. Cephalic venipuncture in this area should be avoided, and performed with caution if needed.

Secondary erythrocytosis due to hypoxemia as prognosis in exacerbated chronic pulmonary diseases / Eritrocitosis secundaria a hipoxemia como pronóstico en neumopatías crónicas exacerbadas

Abstract Introduction: Even though exacerbations are the main cause of emergency consultation in patients suffering from lung diseases, erythrocyte parameters are not assessed in their prognosis. Thus, determining the implications of erythrocyte parameters might contribute to define the usefulness of phlebotomy or red blood cells transfusion in these patients. Objective: To establish a possible relationship between the different hematocrit levels with a 30-day prognosis in patients admitted with exacerbated chronic lung disease and hypoxemia. Materials and methods: A study based on a 30- day follow-up was conducted. Variables were described using an additional categorization by hematocrit levels and an adjustment in a multivariate model through logistic regression. Results: Follow-up was completed for 110 Patients. The frequency of anemia was 7.3% and of erythrocytosis, 14.5%. A significant association to the outcome using Anthonisen score (OR=10.45, 95%CI: 1.11-98.48, p=0.04), hypertension (OR=11.02, 95%CI: 1.32-91.75, p=0.026) and heart failure (OR=0.09, 95%CI: 0.01-0.82, p=0.032) was found. Conclusion: This research could not determine any relationship between erythrocyte parameters and prognosis of patients suffering from pulmonary diseases; nevertheless, extreme values of hematocrits tended to have adverse outcomes.

Resumen Introducción. Aunque las exacerbaciones de las neumopatías crónicas son las principales causas de consulta a urgencias de los pacientes que las padecen, los parámetros eritrocitarios no son evaluados en su pronóstico. Por tanto, determinar las implicaciones de los parámetros eritrocitarios podría ayudar a definir la utilidad de la flebotomía o la transfusión de eritrocitos en estos pacientes. Objetivo. Establecer si hay relación entre los distintos niveles de hematocrito con pronóstico a 30 días en pacientes con neumopatía crónica exacerbada e hipoxemia. Materiales y métodos. Estudio de seguimiento a 30 días. Se realizó la descripción de las variables con una categorización adicional por niveles de hematocrito y un ajuste en un modelo multivariado por regresión logística. Resultados. Se completó el seguimiento en 110 pacientes. La frecuencia de anemia fue de 7.3% y de eritrocitosis de 14.5%. Se encontró asociación significativa al desenlace con la clasificación Anthonisen (OR=10.45, IC95%: 1.11-98.48; p=0.04), hipertensión arterial (OR=11.02, IC95%: 1.32-91.75; p=0.026) y falla cardiaca (OR=0.09, IC95%: 0.01-0.82; p=0.032). Conclusión. Este estudio no pudo determinar relación alguna entre los parámetros eritrocitarios y el pronóstico de pacientes con enfermedades pulmonares crónicas; sin embargo, hubo una tendencia a que los valores extremos del hematocrito presentaran desenlaces adversos.