Acute promyelocytic leukemia in childhood and adolescence: treatment results of a modified AIDA protocol at a Brazilian center

ABSTRACT Introduction: Acute promyelocytic leukemia currently presents an excellent chance of cure with protocols based on all-trans-retinoic acid (ATRA) and anthracycline or only differentiation agents. However, high early mortality rates continue to be reported Methods: Between 2000 and 2018, patients were enrolled and retrospectively analyzed by medical records. A modified AIDA protocol, with a 1-year shortening of the treatment duration, reduction in the number of drugs and a strategy to reduce early mortality by the postponement of the initiation of anthracyclines were employed. Overall and event-free survival rates and toxicity were analyzed Results: Thirty-two patients were enrolled, of whom 56% were female, with a median age of 12 years and 34% belonged to the high-risk group. Two patients had the hypogranular variant and three had another cytogenetic alteration, in addition to the t(15;17). The median start of the first anthracycline dose was 7 days. There were two early deaths (6%) due to central nervous system (CNS) bleeding. All patients achieved molecular remission after the consolidation phase. Two children relapsed and were rescued by arsenic trioxide and hematopoietic stem cell transplantation. The presence of disseminated intravascular coagulation (DIC) at diagnosis (p = 0.03) was the only factor with survival impact. The five-year event-free survival (EFS) was 84% and 5-year overall survival (OS) was 90% Conclusion: The survival results were comparable to those found in the AIDA protocol, with a low rate of early mortality in relation to the Brazilian reality.

Predictores de mortalidad precoz en el mieloma múltiple de nuevo diagnóstico / Predictors of early mortality in the new diagnosis multiple myeloma

Introducción: El mieloma múltiple es una neoplasia maligna de células B caracterizada por una proliferación clonal incontrolada de células plasmáticas. Se define como mortalidad precoz en el mieloma múltiple de nuevo diagnóstico, al porcentaje de muerte que ocurre dentro de los primeros seis meses y afecta entre el 10 y 14 % de los casos. Los biomarcadores han evolucionado desde la caracterización del tumor hasta el reconocimiento de las aberraciones cromosómicas y moleculares que desempeñan un papel en la supervivencia. Objetivo: Describir los principales predictores identificados con relación a la mortalidad precoz y su función en la patogénesis de la enfermedad. Métodos: Se analizó la literatura científica publicada. Se utilizaron motores de búsqueda como Google Scholar, PubMed y ScienceDirect. Se consultaron un total de 80 artículos y se incluyeron 52, en su mayoría de los últimos cinco años. Análisis y síntesis de la información: Se evidenciaron mecanismos genéticos y epigenéticos que contribuyen de manera decisiva en la mortalidad precoz de pacientes con mieloma múltiple de nuevo diagnóstico. Conclusiones: El aumento del riesgo de mortalidad precoz en pacientes con mieloma múltiple de nuevo diagnóstico está asociado a factores clínicos y biológicos, por lo que existe la necesidad de estratificación de los pacientes para un manejo personalizado que impone el uso de datos clínicos y biológicos de una forma integrada.

Introduction: Multiple Myeloma is a malignant B-cell neoplasm characterized by uncontrolled clonal proliferation of plasma cells. Early mortality in newly diagnosed Multiple Mieloma is defined as the percentage of death that occurs six months or less after diagnosis and, it affects 10 to 14 % of the cases. Biomarkers have evolved from the characterization of tumor to the recognition of chromosomal and molecular aberrations that play a rol in survival. Objective: To describe the main predictors identified related to early mortality and their role in the pathogenesis of the disease. Methods: The scientific literature was analyzed using search engines such as Google Scholar, PubMed and ScienceDirect. Consulted articles were 80 and included articles were 52, mostly from the last five years. Analysis and information synthesis: Genetic and epigenetic mechanisms that contribute decisively in the early mortality in new diagnosis Multiple Myeloma patients were evidenced. Conclusions: The increased risk of early mortality in patients with newly diagnosed Multiple Myeloma is associated with clinical and biological factors and there is a need to stratify patients in terms of early mortality risk for personalized management, for which it is imposed the use of clinics and biological data in an integrative way.

Predictores de mortalidad precoz en pacientes con trauma complejo hemorrágico / Early mortality predictors in patients with complex hemorrhagic trauma

RESUMEN Fundamento: El trauma complejo es un problema de salud a nivel mundial y cuando es de tipo hemorrágico la mortalidad es superior a los otros tipos de traumas complejos. Objetivo: Determinar las variables predictoras de mortalidad precoz en pacientes hospitalizados con trauma complejo hemorrágico en una institución hospitalaria del segundo nivel de atención en Cuba. Metodología: Se realizó un estudio transversal en el Hospital General Provincial Camilo Cienfuegos de Sancti Spíritus, durante 6 años. Se incluyeron 207 pacientes. Las variables se agruparon en sociodemográficas, enfermedades crónicas asociadas, mecanismo lesional, tipo de trauma, localización topográfica, tiempo entre admisión hospitalaria, diagnóstico y tratamiento, complicaciones precoces, tratamiento médico y quirúrgico, y mortalidad precoz. Se elaboró un árbol de decisión mediante el método Chaid exhaustivo, la variable dependiente fue la mortalidad por trauma complejo hemorrágico. Resultados: Predominaron los pacientes del sexo masculino (85 %), con 60 años y menos (83 %), con trauma contuso (57.5 %) y politraumatizados (42.5 %). Predominaron también los que presentaron acidosis metabólica (66.7 %), coagulopatía aguda (44.4 %), hipotermia (41.5 %). El 30 % de los pacientes falleció precozmente. El árbol de decisión tuvo una sensibilidad de 82.3 %, una especificidad de 97.2 % y un porcentaje global de pronóstico correcto del 92.8 %. Se identificaron 4 variables predictores de mortalidad: hipotermia, acidosis metabólica, coagulopatía aguda y trauma penetrante. Conclusiones: La probabilidad más alta de fallecer precozmente durante un trauma complejo hemorrágico se da entre pacientes con hipotermia, acidosis metabólica, coagulopatía aguda y trauma penetrante.

ABSTRACT Background: Complex trauma is a worldwide health problem and when hemorrhagic, mortality is higher than other types of complex trauma. Objective: To determine predictive variables of early mortality in hospitalized patients with complex hemorrhagic trauma in a second care level hospital in Cuba. Methodology: A cross-sectional study was conducted at Camilo Cienfuegos Provincial General Hospital in Sancti Spíritus, for 6 years. 207 patients were included. The variables were grouped into sociodemographic, associated chronic diseases, injury mechanism, type of trauma, topographic location, time between hospital admission, diagnosis and treatment, early complications, medical and surgical treatment, and early mortality. A decision tree was developed using the exhaustive Chaid method, the dependent variable was mortality due to complex hemorrhagic trauma. Results: Male patients (85 %), 60 years and younger (83 %), with blunt trauma (57.5 %) and polytraumatized patients (42.5 %) predominated. Those who presented metabolic acidosis (66.7 %), acute coagulopathy (44.4 %), and hypothermia (41.5 %) also predominated. 30 % of patients died early. The decision tree had a sensitivity of 82.3 %, a specificity of 97.2 % and an overall percentage of correct forecast of 92.8 %. Four variables that predicted mortality were identified: hypothermia, metabolic acidosis, acute coagulopathy, and penetrating trauma. Conclusions: The highest probability of early dying during a complex hemorrhagic trauma occurs among patients with hypothermia, metabolic acidosis, acute coagulopathy and penetrating trauma.

Predictors for early mortality in patients with implantable cardiac defibrillator for heart failure with reduced ejection fraction

Implantable cardioverter defibrillators (ICD) are recommended in heart failure with reduced ejection fraction (HFrEF) patients to reduce arrhythmic deaths. This study aimed to identify risk factors associated with mortality within one-year following the ICD. The data from our hospital's electronic database system was extracted for patients who were implanted ICD secondary to HFrEF between 2009 and 2019. Overall, 1107 patients were included in the present analysis. Mortality rate at one-year following the device implantation was 4.7%. In multivariate analysis; age, atrial fibrillation, New York Heart Association classification >2, blood urea nitrogen, pro-brain natriuretic peptide and albumin independently predicted one year mortality

Evolución histórica de las desigualdades de género. Principales causas de muerte. Cuba 2005-2016 / Historical evolution of gender inequalities. Main causes of death. Cuba 2005-2016

Introducción: La forma de culminar la vida es una expresión de la manera en que se vivió. Las mujeres y los hombres no atraviesan su paso por el mundo igualmente y tampoco comparten las mismas características biológicas que son diferentes para cada sexo. Objetivos: identificar la tendencia del riesgo de morir entre los sexos en las principales causas de muerte entre 2005 y 2016 e identificar la tendencia del riesgo de morir prematuramente entre los sexos en las principales causas de muerte entre 2009 y 2016 en Cuba. Métodos: Se elaboraron series cronológicas de las principales causas de muerte conformadas con las razones hombres-mujeres y se analizó la tendencia mediante rectas de regresión o promedios móviles. Los posibles motivos se identificaron mediante entrevistas en profundidad a expertos de género y salud. Resultados: Se encontraron tres grupos de causas de muerte. En el primero clasificaron: tumores malignos, enfermedades del corazón, enfermedad cerebrovascular, influenza y neumonía, accidentes, enfermedades crónicas de las vías respiratorias y las de las arterias, arteriolas y vasos capilares. El segundo estuvo conformado por la cirrosis hepática y otras enfermedades del hígado y las lesiones autoinfligidas. En el último grupo constó la diabetes mellitus. La tendencia se mantuvo sin notable variación a lo largo del tiempo en casi todas las series analizadas. Las explicaciones referidas que trataron explicar las diferencias encontradas en las agrupaciones de causas de muerte transitaron desde las que atribuyeron la acción de factores biológicos hasta las relacionadas con las consecuencias de la identidad de género. Discusión: Los hombres presentaron una situación más desventajosa debido a que el riesgo de morir y el riesgo de morir prematuramente fue superior al de las mujeres en la mayoría de las causas de muerte estudiadas que estuvieron más afectadas, principalmente por la diabetes mellitus. Conclusiones: La tendencia de la mortalidad de la razón hombre-mujer de las principales causas de muerte y de la mortalidad prematura evidenció diferencias entre los sexos, fue el masculino el más afectado a lo largo del tiempo(AU)

Introduction: The way life ends it is an expression of the way it was lived. Women and men do not live life equally and they neither share the same biological characteristics, which are different for each sex. Objectives: To identify the trends related to the risk of dying among both sexes and the main causes of death from 2005 to 2016, and the risk of dying prematurely from 2009 to 2016. Methods: Time series with the main causes of death among men and women were made and it was analyzed the trend by means of straight regression or moving averages. The possible reasons were identified through in-depth interviews to experts in gender and health. Results: There were found three groups of causes of death. In the first, there were: malignant tumors, heart diseases, cerebrovascular diseases, influenza and pneumonia, accidents; respiratory, arteries, arterioles and capillary vessels chronic diseases. The second group was formed by liver cirrhosis and other diseases of the liver and self-inflicted injuries. The third group consisted in diabetes mellitus. The trend continued without an outstanding variation over time in almost all of the analyzed series. Various reasons were found that could affect the group of causes of death that went from the biological to those dealing with the consequences of gender identity. Discussion: Men are those who submitted a disadvantage since the risk of dying and the risk of dying prematurely was superior to the women in the majority of the studied causes, mainly because of diabetes mellitus. Conclusions: Mortality trends among men and women and the main causes of death and of premature mortality evidenced differences between the sexes being males the most affected in long term(AU)

The burden of hypoxic-ischaemic encephalopathy in Malaysian neonatal intensive care units

<p><b>INTRODUCTION</b>This study aimed to determine the incidence of hypoxic-ischaemic encephalopathy (HIE) and predictors of HIE mortality in Malaysian neonatal intensive care units (NICUs).</p><p><b>METHODS</b>This was a retrospective study of data from 37 NICUs in the Malaysian National Neonatal Registry in 2012. All newborns with gestational age ≥ 36 weeks, without major congenital malformations and fulfilling the criteria of HIE were included.</p><p><b>RESULTS</b>There were 285,454 live births in these hospitals. HIE was reported in 919 newborns and 768 of them were inborn, with a HIE incidence of 2.59 per 1,000 live births/hospital (95% confidence interval [CI] 2.03, 3.14). A total of 144 (15.7%) affected newborns died. Logistic regression analysis showed that the significant predictors of death were: chest compression at birth (adjusted odds ratio [OR] 2.27, 95% CI 1.27, 4.05; p = 0.003), being outborn (adjusted OR 2.65, 95% CI 1.36, 5.13; p = 0.004), meconium aspiration syndrome (MAS) (adjusted OR 2.16, 95% CI 1.05, 4.47; p = 0.038), persistent pulmonary hypertension of the newborn (PPHN) (adjusted OR 4.39, 95% CI 1.85, 10.43; p = 0.001), sepsis (adjusted OR 4.46, 95% CI 1.38, 14.40; p = 0.013), pneumothorax (adjusted OR 4.77, 95% CI 1.76, 12.95; p = 0.002) and severe HIE (adjusted OR 42.41, 95% CI 18.55, 96.96; p < 0.0001).</p><p><b>CONCLUSION</b>The incidence of HIE in Malaysian NICUs was similar to that reported in developed countries. Affected newborns with severe grade of HIE, chest compression at birth, MAS, PPHN, sepsis or pneumothorax, and those who were outborn were more likely to die before discharge.</p>

Perioperative morbidity and mortality in the first year of life: a systematic review (1997-2012) / Morbimortalidade perioperatória no primeiro ano de idade: revisão sistemática (1997-2012)

ABSTRACTBACKGROUND AND OBJECTIVES: Although many recognize that the first year of life and specifically the neonatal period are associated with increased risk of anesthetic morbidity and mortality, there are no studies directed to these pediatric subpopulations. This systematic review of the scientific literature including the last 15 years aimed to analyze the epidemiology of morbidity and mortality associated with general anesthesia and surgery in the first year of life and particularly in the neonatal (first month) period.CONTENT: The review was conducted by searching publications in Medline/PubMed databases, and the following outcomes were evaluated: early mortality in the first year of life (<1 year) and in subgroups of different vulnerability in this age group (0-30 days and 1-12 months) and the prevalence of cardiac arrest and perioperative critical/adverse events of various types in the same subgroups.CONCLUSIONS: The current literature indicates great variability in mortality and morbidity in the age group under consideration and in its subgroups. However, despite the obvious methodological heterogeneity and absence of specific studies, epidemiological profiles of morbidity and mortality related to anesthesia in children in the first year of life show higher frequency of morbidity and mortality in this age group, with the highest peaks of incidence in the neonates' anesthesia.

RESUMOJUSTIFICATIVA E OBJETIVOS: Embora muitos reconheçam que a idade inferior a um ano e especificamente o período neonatal estejam associados a maior risco de morbimortalidade anestésica, não existem estudos dirigidos a essas subpopulações pediátricas. Esta revisão sistemática das publicações científicas dos últimos 15 anos teve como objetivo analisar o perfil epidemiológico da morbimortalidade relacionada com a anestesia geral e cirurgia no primeiro ano de idade e em particular no período neonatal (primeiro mês de idade).CONTEúDO: A revisão foi conduzida por pesquisa de publicações nas bases de dados Medline/PubMed. Foram avaliados os seguintes desfechos: mortalidade precoce no primeiro ano de idade (< 1A) e em subgrupos de diferente vulnerabilidade nesta faixa etária (0-30 dias e 1-12 meses) e prevalência de parada cardíaca e eventos críticos/adversos perioperatórios de diversos tipos nos mesmos subgrupos.CONCLUSÕES: A literatura corrente indica grande variabilidade nos índices de mortalidade e morbidade na faixa etária em análise, bem como nos seus subgrupos. No entanto, apesar da óbvia heterogeneidade metodológica e da ausência de estudos específicos, os perfis epidemiológicos de morbimortalidade relacionada com a anestesia de crianças no primeiro ano de idade mostram frequência mais alta de morbimortalidade nessa faixa etária, com os maiores picos de incidência na anestesia de neonatos.

Characteristics of and Risk Factors for Early Pediatric Mortality at Zomba Central Hospital, Malawi.

Background: Pediatric mortality rates are high throughout sub-Saharan Africa with most deaths occurring within 48 hours of admission to hospital. Early identification and treatment of at risk children is essential to improve outcomes, however, few studies have identified disease specific risk factors for early mortality. Study Aims: To identify risk factors for early pediatric mortality and to explore areas for improvement in diagnostic and treatment practices. Study Design: Case-control study of patients admitted to the pediatrics ward of Zomba Central Hospital, Malawi. Methodology: Cases included all pediatric deaths over a four-month period in 2010 occurring within 48 hours of admission with a diagnosis among the four most common causes of death (malaria, pneumonia, diarrhea/dehydration, meningitis or measles given a large outbreak during the study period). Controls included children admitted during the same period, with a discharge diagnosis among the same 5 diagnoses, who survived to at least 72 hours. Analysis: Associations between mortality and clinical characteristics were assessed using STATA 11.0. Results: Overall, 142 cases and 162 controls were included. 62.7% of deaths occurred within 12 hours with malaria the most common diagnosis in both groups. Clinical characteristics on presentation associated with death included: symptoms/signs of respiratory distress (OR1.9, 95% CI 1.0-3.4, p=0.04), low Blantyre Coma Score (OR 3.4, 95% CI 2.1-5.6, p<0.01), age under 5 (OR 3.2, 95% CI 1.1-9.3, p=0.03) and pallor among malaria cases (OR2.2, 95% CI 1.8-6.6), p<0.01). Areas identified for quality improvement included delay in initial investigations and initiation of treatment both prior to transfer to and after admission to the district hospital. Conclusion: Improvements in the identification of children at risk for early mortality are critical to reducing mortality through early intervention.