Consenso chileno para la atención integral de niños y adultos con fibrosis quística

Resumen La Fibrosis Quística (FQ) es la enfermedad hereditaria de pronóstico reservado más frecuente en raza blanca. Desde el año 2003, Chile inicia un Programa Nacional de Fibrosis Quística, de carácter integral, dirigido por la Unidad de Salud Respiratoria del Ministerio de Salud. Hasta la fecha, los principales resultados del Programa registran una significativa mayor sobrevida (promedio 27 años) y una significativa reducción en la edad de diagnóstico de los pacientes ingresados desde 2006 en adelante. El acceso a la canasta GES (Garantías Explícitas en Salud), la implementación del tamizaje neonatal en algunas regiones del país, la organización y la constitución de equipos entrenados en FQ de diversas especialidades, ha contribuido a mejorar los resultados. Si bien las principales manifestaciones son del aparato respiratorio y digestivo, el carácter multisistémico de la FQ obliga a conocer los distintos aspectos involucrados en su manejo, a fin de optimizar los resultados del tratamiento y los recursos invertidos, tanto en el sector público como privado. Este documento es una revisión y actualización sobre los principales aspectos del diagnóstico, seguimiento y tratamiento de las manifestaciones respiratorias y no respiratorias de la FQ.

Cystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.

Consenso chileno para la atención integral de niños y adultos con fibrosis quística / Chilean consensus for the integral care of children and adults with cystic fibrosis

Cystic Fibrosis (CF) is the most frequent hereditary disease in whites, with a reserved prognosis. Since 2003, Chile began a comprehensive National Cystic Fibrosis Program, directed by the Respiratory Health Unit of the Ministry of Health. To date, the main results of the Program record a significantly longer survival (average 27 years) and a significant reduction in the age of diagnosis of patients admitted from 2006 onwards. Access to Chilean Explicit Health Guarantees, the implementation of neonatal screening in some regions of the country, the organization and setting up of CF-trained teams of various specialties, has contributed to improving results. Although the main manifestations are of the respiratory and digestive system, the multisystemic nature of CF makes it necessary to know the different aspects involved in its management, in order to optimize the results of the treatment and the resources invested, both in the public and private sectors. This document is a review and an update on the main aspects of the diagnosis, monitoring and treatment of the respiratory and non-respiratory manifestations of CF.

La Fibrosis Quística (FQ) es la enfermedad hereditaria de pronóstico reservado más frecuente en raza blanca. Desde el año 2003, Chile inicia un Programa Nacional de Fibrosis Quística, de carácter integral, dirigido por la Unidad de Salud Respiratoria del Ministerio de Salud. Hasta la fecha, los principales resultados del Programa registran una significativa mayor sobrevida (promedio 27 años) y una significativa reducción en la edad de diagnóstico de los pacientes ingresados desde 2006 en adelante. El acceso a la canasta GES (Garantías Explícitas en Salud), la implementación del tamizaje neonatal en algunas regiones del país, la organización y la constitución de equipos entrenados en FQ de diversas especialidades, ha contribuido a mejorar los resultados. Si bien las principales manifestaciones son del aparato respiratorio y digestivo, el carácter multisistémico de la FQ obliga a conocer los distintos aspectos involucrados en su manejo, a fin de optimizar los resultados del tratamiento y los recursos invertidos, tanto en el sector público como privado. Este documento es una revisión y actualización sobre los principales aspectos del diagnóstico, seguimiento y tratamiento de las manifestaciones respiratorias y no respiratorias de la FQ.

Enfermedad hepática por alcohol / Alcoholic liver disease

Excessive alcohol consumption is an important cause of preventable morbidity and mortality. We have to bealert to chronic alcohol usage or abuse. Simple tests (AUDIT, CAGE) can be applied quickly on outpatientcare. We highlight advances in understanding the immune and molecular mechanisms; there is disruptionof the intestinal barrier with bacterial translocation, as well as endotoxins which activate the liver’s innateimmunity, causing apoptosis, necrosis, inflammation and fibrosis. Alcoholic hepatitis is most common inpatients between 40 and 60 years of age, preferably male with jaundice, fever, ascites, hepatomegaly. Thediagnosis is confirmed with a history of alcoholic consumption, mild to moderate AST and ALT values,a AST/ALT ratio > 2, hyperbilirrubinemia and prolonged prothrombin time. There are scores to evaluatethe severity and the need of corticoid therapy, such as modified Maddrey discriminating function andMELD score. Lille score assesses the response to treatment in the seventh day. The risks and benefits ofliver biopsy should be evaluated individually. The cornerstone of treatment remains alcohol abstinence.Nutritional management must be carefully monitored. Proteins requirements are standardized based onweight. The use of corticoids with 40 mg of prednisolone each day is the most widely accepted therapy,indicated on patients with MMDF higher than 32 or MELD score higher than 21. If Lille score is higherthan 0.45 at the seven day under corticoid therapy, treatment must be interrupted. The use of pentoxifyllinewould only be effective for prevention of hepatorenal syndrome...

El consumo excesivo de alcohol es una causa importante de morbimortalidad prevenible. Debemos estaratentos en detectar a pacientes con dependencia o abuso crónico de alcohol. Test sencillos (AUDIT, CAGE)pueden aplicarse rápidamente en consulta ambulatoria. Destacamos avances en el conocimiento moleculare inmunológico, existe disrupción de la barrera intestinal con translocación bacteriana y endotoxinas conactivación del sistema inmune innato del hígado, produciendo apoptosis celular, necrosis e inflamación yfibrosis. La hepatitis alcohólica se presenta principalmente en pacientes entre 40 y 60 años, preferentementeen varones con ictericia, fiebre, ascitis, hepatomegalia. El diagnóstico se confirma con antecedentes deingesta alcohólica, GOT y GPT elevadas en forma leve o moderada, relación GOT/GPT mayor de 2, hiperbilirrubinemiay tiempo de protrombina prolongado. Existen scores para evaluar la gravedad y necesidad demanejo con corticoides como función discriminante de Maddrey modificada y MELD. El puntaje de Lilleevalúa respuesta del tratamiento al séptimo día. El riesgo/beneficio de la biopsia hepática se evalúa caso acaso. La piedra angular del tratamiento sigue siendo la abstinencia. Manejo nutricional debe ser riguroso.Requerimientos proteicos están estandarizados por peso. La terapia con corticoides (prednisolona 40 mg/día) es la más ampliamente aceptada, con indicación en pacientes con FDMm mayor a 32 o MELD mayora 21. Si el puntaje de Lille es mayor de 0,45 a los 7 días con corticoides, deben suspenderse. Pentoxifilinasólo tendría efecto en prevenir el desarrollo de síndrome hepatorrenal (SHR). Hay nuevas terapias enevaluación, como el uso de G-CSF...

Post-depositional fracturing and subsidence of pumice flow deposits: Lascar Volcano, Chile.

Unconsolidated pyroclastic flow deposits of the 1993 eruption of Lascar Volcano, Chile, have, with time, become increasingly dissected by a network of deeply penetrating fractures. The fracture network comprises orthogonal sets of decimeter-wide linear voids that form a pseudo-polygonal grid visible on the deposit surface. In this work, we combine shallow surface geophysical imaging tools with remote sensing observations and direct field measurements of the deposit to investigate these fractures and their underlying causal mechanisms. Based on ground penetrating radar images, the fractures are observed to have propagated to depths of up to 10 m. In addition, orbiting radar interferometry shows that deposit subsidence of up to 1 cm/year-1 occurred between 1993 and 1996 with continued subsidence occurring at a slower rate thereafter. In situ measurements show that 1 m below the surface, the 1993 deposits remain 5°C to 15°C hotter, 18 years after emplacement, than adjacent deposits. Based on the observed subsidence as well as estimated cooling rates, the fractures are inferred to be the combined result of deaeration, thermal contraction, and sedimentary compaction in the months to years following deposition. Significant environmental factors, including regional earthquakes in 1995 and 2007, accelerated settling at punctuated moments in time. The spatially variable fracture pattern relates to surface slope and lithofacies variations as well as substrate lithology. Similar fractures have been reported in other ignimbrites but are generally exposed only in cross section and are often attributed to formation by external forces. Here we suggest that such interpretations should be invoked with caution, and deformation including post-emplacement subsidence and fracturing of loosely packed ash-rich deposits in the months to years post-emplacement is a process inherent in the settling of pyroclastic material.

[Bacteremia and endarteritis cases secondary to Campylobacter spp. in a metropolitan hospital: our experience along a quarter of a century]. / Bacteriemias e infección endovascular por Campylobacter spp: nuestra experiencia en un cuarto de siglo de historia.

Six cases of bacteremia and one of endarteritis were identified between 1986 and 2010 in a general hospital in Chile. Five of these cases occurred during the second half of this timeframe, Campylobacter fetus predominated (5 out of 7) and the majority of the infections presented during warmer months. The mean age was 32.4 years (range 19 to 63) all had comorbidities, and main clinical manifestations included fever with diarrhea. Four patients developed hypotension and two septic shock. The latter, associated to C. fetus bacteremia, died before microbiological diagnosis. Six out of 7 patients received antimicrobial therapy. During 2004 and 2010, the rates of Campylobacter spp. positive stool cultures in the same hospital increased 4 times, suggesting an emerging profile. Bacteremia and endarteritis by Campylobacter spp. can develop in vulnerable patients and manifest as fever with or without diarrhea. Finding curved or spiral shaped gram negative rods in blood cultures leads to suspect this pathogen. Species identification is of utmost importance due to antimicrobial resistance especially in C. jejuni. Prognosis is unfavorable due to host characteristics, and case-fatality rate is high.

Bacteriemias e infección endovascular por Campylobacter spp: nuestra experiencia en un cuarto de siglo de historia / Bacteremia and endarteritis cases secondary to Campylobacter spp. in a metropolitan hospital: Our experience along a quarter of a century

Six cases of bacteremia and one of endarteritis were identified between 1986 and 2010 in a general hospital in Chile. Five of these cases occurred during the second half of this timeframe, Campylobacterfetus predominated (5 out of 7) and the majority of the infections presented during warmer months. The mean age was 32.4 years (range 19 to 63) all had comorbidities, and main clinical manifestations included fever with diarrhea. Four patients developed hypotension and two septic shock. The latter, associated to C. fetus bacteremia, died before microbiological diagnosis. Six out of 7 patients received antimicrobial therapy. During 2004 and 2010, the rates of Campylobacter spp. positive stool cultures in the same hospital increased 4 times, suggesting an emerging profile. Bacteremia and endarteritis by Campylobacter spp. can develop in vulnerable patients and manifest as fever with or without diarrhea. Finding curved or spiral shaped gram negative rods in blood cultures leads to suspect this pathogen. Species identification is of utmost importance due to antimicrobial resistance especially in C. jejuni. Prognosis is unfavorable due to host characteristics, and case-fatality rate is high.

Se presentan seis casos de bacteriemia y uno de infección vascular por Campylobacter spp, observados en 25 años, con el fin de describir sus características clínicas. Cinco de ellos se registraron en la segunda mitad del período, en concomitancia con el incremento de este agente en el porcentaje de coprocultivos, lo que sugiere un perfil emergente. Las infecciones fueron más frecuentes en los meses cálidos, asociadas principalmente a C. fetus (5 de 7) y a co-morbilidad. La edad promedio de los pacientes fue de 32,4 años (rango 19 a 63 años) y todos tenían comorbilidades. Las manifestaciones clínicas más frecuentes fueron diarrea y fiebre (5 de 7 casos) y dos pacientes cursaron con shock séptico (28,6 por ciento). La evolución fue favorable en cinco pacientes pero los dos que presentaron shock asociado a C. fetus fallecieron (28,6 por ciento). Las bacteriemias o infecciones vasculares por Campylobacter spp., aunque infrecuentes, pueden presentarse en pacientes vulnerables y debutar como cuadros febriles, en presencia o ausencia de diarrea. La identificación de la especie involucrada es de suma importancia debido a la escasa actividad terapéutica de cefalosporinas de tercera generación y quinolonas. El pronóstico de estas bacteriemias es grave debido a las características del hospedero y a su elevada letalidad.

The 2010 Mw 8.8 Maule megathrust earthquake of Central Chile, monitored by GPS.

Large earthquakes produce crustal deformation that can be quantified by geodetic measurements, allowing for the determination of the slip distribution on the fault. We used data from Global Positioning System (GPS) networks in Central Chile to infer the static deformation and the kinematics of the 2010 moment magnitude (M(w)) 8.8 Maule megathrust earthquake. From elastic modeling, we found a total rupture length of ~500 kilometers where slip (up to 15 meters) concentrated on two main asperities situated on both sides of the epicenter. We found that rupture reached shallow depths, probably extending up to the trench. Resolvable afterslip occurred in regions of low coseismic slip. The low-frequency hypocenter is relocated 40 kilometers southwest of initial estimates. Rupture propagated bilaterally at about 3.1 kilometers per second, with possible but not fully resolved velocity variations.

[Vibrio parahaemolyticus bacteremia: case report and literature review]. / Bacteriemia por Vibrio parahaemolyticus: Reporte de caso y revisión de la literatura.

Vibrio parahaemolyticus is a facultative anaerobic gram negative rod responsible of sea food-associated diarrhoea. Although less common, it also causes wound infections and bacteraemia. We present a case of bacteraemia by this agent and a review of the literature.

Bacteriemia por Vibrio parahaemolyticus: reporte de caso y revisión de la literatura / Vibrio parahaemolyticus bacteremia: case report and literature review

Vibrio parahaemolyticus is a facultative anaerobio gram negative rod responsible of sea food-associated diarr-hoea. Although less common, it also causes wound infections and bacteraemia. We present a case of bacteraemia by this agent and a review of the literature.

Vibrio parahaemolyticus es un bacilo gram negativo, anaerobio facultativo, responsable de brotes de síndrome diarreico agudo por ingestión de mariscos crudos o mal cocidos contaminados. Ocasionalmente. se ha reportado asociado a infección de heridas y sepsis. Se reporta un caso clínico de bacteriemia por este microorganismo y se realiza una revisión de la literatura.

Potencial de disparidad: [revisión] / Mismatch negativity: [revision]

El potencial de disparidad o mismatch negativity (MMN) corresponde a la respuesta eléctrica extraída del electroencefalograma que se produce frente a diversos cambios de las características del estímulo acústico. Se obtiene presentando al sujeto una secuencia de estímulos repetitivos de características acústicas similares (estímulo estándar) alternado en forma aleatoria con estímulos acústicos discrepantes que difieren del primero en alguno de sus atributos (estímulo discrepante). El MMN se originaría en la corteza auditiva primaria y se ha logrado registrar desde el nacimiento. No requiere la atención del sujeto, permitiendo evaluar en forma objetiva la discriminación de tonos y fonemas. En clínica se ha utilizado en evaluación del procesamiento auditivo central, pacientes con dislexia o trastorno específico del lenguaje, autismo, individuos con implante coclear e incluso en pacientes en coma. En el presente artículo se revisan las principales características, origen anatómico y utilidad clínica del MMN.

The mismatch negativity (MMN) is a specific component of the auditory event-related brain potentials. It is elicited by an infrequent, physically deviant sound (deviant-stimulus) occurring in a sequence of homogeneous repetitive sounds (standard-stimulus). MMN is probably generated in the primary auditory cortex and it has been successfully recorded in newborns. The MMN can be elicited even in the absence of attention and it can be used as an objective method to assess tone and phoneme discrimination. Some clinical applications of MMN are: evaluation of central auditory processing, patients with dyslexia or language specific disorders, autism, cochlear implant - users and even in prognosis of coma. In this article the main characteristics, origin and clinical applications of the MMN are reviewed.

Description of endoscopic ventricular anatomy in myelomeningocele.

OBJECTIVE: The purpose of this work is to present our endoscopic neuroanatomical findings of a series of myelomeningocele and hydrocephalus patients, treated with endoscopic third ventricular cisternostomy (ETVC), in order to describe ventricular configuration abnormalities in this group of patients, in which this neurosurgical procedure has limited performance. METHOD: We checked the videos of 10 endoscopic third ventricular cisternostomies of myelomeningocele patients taken during 24 months as from December 1998. A previous guideline is designed to record anatomic variables in the lateral ventricles, IIIrd ventricle, and basal cisterns. The topic is analyzed in view of the necropsy and imaging background data. RESULTS: The ETVC of lateral ventricles showed: absence of septum (9/10); absence of anteroseptal vein (8/10); absence of choroid plexus and thalamostriate vein (0/10); absence of fornix (1/10): small foramen of Monro (4/10). The ETVC of the IIIrd ventricle showed: impossibility of recognizing any mammillary bodies (4/10); presence of septations (5/10); presence of atypical veins in the floor (6/10); translucent floor (5/10); floor umbilications (5/10); absence of infundibulum (4/10); arachnoid adherences (7/10); and visual contact of basilar artery (4/10). CONCLUSION: There are categorical structural alterations in the ventricular system of myelomeningocele patients that are well correlated with previous necropsy and imaging reports. The ventricular system of dysraphic children presents severe anatomic alterations, which alter the reference points of the classical endoscopic third ventricular cisternostomy.

Endocarditis por Abiotrophia defectiva en paciente adulto / Endocarditis caused by Abiotrophia defectiva in an adult patient

Abiotrophia defectiva, formerly designated as a member of nutritionally variant streptococci, is part of normal oral flora and may be a cause of culture-negative endocarditis. We report a case of infective endocarditis caused by A. defectiva in a 37-year-old man, allergic to penicillin. We also review the literature for antibiotic treatment alternatives and the microbiological diagnostic possibilities at present.

Abiotrophia defectiva es una cocácea grampositiva considerada anteriormente como parte del grupo de los estreptococos nutricionalmente variables. Es parte de la microbiota oral y puede ser causante de endocarditis bacteriana con cultivo negativo. Se reporta el caso de un paciente varón de 37 años de edad, alérgico a penicilina, con endocarditis infecciosa causada por A. defectiva y se realiza revisión de la literatura sobre las alternativas terapéuticas y el estado actual del diagnóstico microbiológico de este agente.

[Eclampsia at the Rancagua Regional Hospital]. / Eclampsia en Hospital Regional de Rancagua.

Forty-four cases of eclampsia with an incidence of 2.3 in 1,000 deliveries were analyzed. The major frequency was in primiparas (81.8%) and were less than 19 years old (54.5%). Convulsions occurred during pregnancy in 65.9% of patients, during labor 6.8% and 11.4% postpartum. In 91% of patients a cesarean section was performed. The most frequent indication was low pelvic score. The 61.4% of the labor occurred at 37 weeks or less. Perinatal mortality was 2.3% (one new born of 1,340 g death in the neonatal period). We don't have maternal mortality.