A strategy to improve treatment-related mortality and abandonment of therapy for childhood ALL in a developing country reveals the impact of treatment delays.

BACKGROUND: Treatment-related mortality and abandonment of therapy are major barriers to successful treatment of childhood acute lymphoblastic leukemia (ALL) in the developing world. PROCEDURE: A collaboration was undertaken between Instituto Nacional de Cancerologia (Bogota, Colombia), which serves a poor patient population in an upper-middle income country, and Dana-Farber/Boston Children's Cancer and Blood Disorders Center (Boston, USA). Several interventions aimed at reducing toxic deaths and abandonment were implemented, including a reduced-intensity treatment regimen and a psychosocial effort targeting abandonment. We performed a cohort study to assess impact. RESULTS: The Study Population comprised 99 children with ALL diagnosed between 2007 and 2010, and the Historic Cohort comprised 181 children treated prior to the study interventions (1995-2004). Significant improvements were achieved in the rate of deaths in complete remission (13% to 3%; P = 0.005), abandonment (32% to 9%; P < 0.001), and event-free survival with abandonment considered an event (47% to 65% at 2 years; P = 0.016). However, relapse rate did not improve. Medically unnecessary treatment delays were common, and landmark analysis revealed that initiating the PIII phase of therapy ≥4 weeks delayed predicted markedly inferior disease-free survival (P = 0.016). Conversely, patients who received therapy without excessive delays had outcomes approaching those achieved in high-income countries. CONCLUSIONS: Implementation of a twinning program was followed by reductions in abandonment and toxic deaths, but relapse rate did not improve. Inappropriate treatment delays were common and strongly predicted treatment failure. These findings highlight the importance of adherence to treatment schedule for effective therapy of ALL.

[Genetic profiles of longevity and healthy cognitive aging in nonagenarians from the Basque Country]. / Perfiles genéticos de longevidad y envejecimiento saludable en nonagenarios del País Vasco.

INTRODUCTION: Currently there are notable differences in the aging of individuals in modern populations. While some of them enjoy a long healthy aging, others develop neurodegenerative diseases, such as Alzheimer's disease (AD). Environmental factors are critical, but genetics could explain the differences observed. It has recently been postulated that longevity genes might also be neuroprotective. OBJECTIVES: To assess whether certain genetic variants associated with longevity might have a neuroprotective effect. METHODS: The subjects of this study are people older than 90 years. We will collect sociodemographic and clinical data and multiple assessments, cognitive, functional, anthropometric, nutritional, sensory and physical each participant. In addition, 64 SNPs loci distributed in 13 candidate genes FOXO3, SIRT1, TOMM40, APOE, PICALM, COMT, CETP, CLU, CR1, IL-6, PCK-1, ZNF224 and ACE will be analysed by Taqman array. RESULTS: It is hoped to gain more knowledge about under/over-represented alleles in nonagenarians. Furthermore, comparison of the genetic characteristics of nonagenarians with AD with those free of disease will enable links to be seen between certain alleles with protection or the risk of AD. Associated information on the participants will create subgroups showing the interactions between environment and genetic variation in relation to healthy aging and AD. CONCLUSION: The study of the genetic variability of nonagenarians can give us information on the alleles associated with longevity and neuroprotection.

Plasmaféresis en el síndrome de Guillain-Barre: evaluación preliminar / Plasmapheresis in Guillain-Barre syndrome: preliminary evaluation

El síndrome de Guillain-Barré es la causa más frecuente de parálasis fláccida aguda. El tratamiento actual se basa en reposo, apoyo kinésico y asistencia ventilatoria, cuando se requiere. En su patogenia se ha encontrado anticuerpos anti nervio periférico humano, intentándose Plasmaféresis (PF) para removerlos, sin embargo existen aún pocos resultados anecdóticos publicados. Se presenta la experiencia preliminar con este tipo de terapia en el hospital FACH, entre 1986 y 1993. Se evaluaron 7 pacientes, clasificándolos según escala de gravedad. Se investigó la presencia de cuadro viral previo, duración de los síntomas, latencia entre el inicio de éstos, ingreso e incio de PF, protocolo de ésta y sus complicaciones, período de recuperación, complicaciones asociadas, necesidad de tratamiento esteroidal y duración de la hospitalización. Cinco pacientes fueron hombres. La edad fluctuó entre los 15 y 75 años (promedio 38 años); con evolución clínica previa entre 4 horas y 7 días. Sólo dos pacientes tienen el antecedente de cuadro viral. Un paciente fue clasificado en grado III, 4 en grado IV y 2 en grado V, realizándose PF en estos dos últimos grupos (4 hombres y 2 mujeres), entre las 18 horas y 6 días de su ingreso. En todos se realizó PF intermitente, con un promedio de 5 sesiones con 7 a 13 ciclos cada vez con reposición de volúmen variable. En un paciente se suspendió por hipotensión severa y otro presentó complicaciones asociadas (insuficiencia respiratoria y sépticas), requiriendo tratamiento esteroidal y antibióticos. El período de recuperación (capacidad de deambular con apoyo) fue de 47,4 días; y 55,8 días de hospitalización, en promedio. Los resultados no concuerdan con los publicados, en los PF acorta en un 50 por ciento el período de recuperación (19 versus 40 días) desde grado IV a III; sin embargo, debido a las muchas variables en juego, se hace muy dificil comparar estas cifras. Tal vez debería someterse a estos pacientes a PF continua precoz con criterios normados de inclusión al tratamiento, para optimizar los resultados