RESUMEN
BACKGROUND AND PURPOSE: The antisense oligonucleotide nusinersen (Spinraza) regulates splicing of the survival motor neuron 2 (SMN2) messenger RNA to increase SMN protein expression. Nusinersen has improved ventilator-free survival and motor function outcomes in infantile onset forms of spinal muscular atrophy (SMA), treated early in the course of the disease. However, the response in later onset forms of SMA is highly variable and dependent on symptom severity and disease duration at treatment initiation. Therefore, we aimed to identify novel noninvasive biomarkers that could predict the response to nusinersen in type II and III SMA patients. METHODS: Thirty-four SMA patients were included. We applied next generation sequencing to identify microRNAs in the cerebrospinal fluid (CSF) as candidate biomarkers predicting response to nusinersen. Hammersmith Functional Motor Scale Expanded (HFMSE) was conducted at baseline and 6 months after initiation of nusinersen therapy to assess motor function. Patients changing by ≥3 or ≤0 points in the HFMSE total score were considered to be responders or nonresponders, respectively. RESULTS: Lower baseline levels of two muscle microRNAs (miR-206 and miR-133a-3p), alone or in combination, predicted the clinical response to nusinersen after 6 months of therapy. Moreover, miR-206 levels were inversely correlated with the HFMSE score. CONCLUSIONS: Lower miR-206 and miR-133a-3p in the CSF predict more robust clinical response to nusinersen treatment in later onset SMA patients. These novel findings have high clinical relevance for identifying early treatment response to nusinersen in later onset SMA patients and call for testing the ability of miRNAs to predict more sustained long-term benefit.
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Biomarcadores Farmacológicos , MicroARNs , Oligonucleótidos , Atrofias Musculares Espinales de la Infancia , Biomarcadores Farmacológicos/líquido cefalorraquídeo , Humanos , MicroARNs/líquido cefalorraquídeo , Músculos , Oligonucleótidos/uso terapéutico , Atrofias Musculares Espinales de la Infancia/líquido cefalorraquídeo , Atrofias Musculares Espinales de la Infancia/terapiaRESUMEN
Floodplains represent critical nursery habitats for a variety of fish species due to their highly productive food webs, yet few tools exist to quantify the extent to which these habitats contribute to ecosystem-level production. Here we conducted a large-scale field experiment to characterize differences in food web composition and stable isotopes (δ¹³C, δ¹5N, δ³4S) for salmon rearing on a large floodplain and adjacent river in the Central Valley, California, USA. The study covered variable hydrologic conditions including flooding (1999, 2017), average (2016), and drought (2012-2015). In addition, we determined incorporation rates and tissue fractionation between prey and muscle from fish held in enclosed locations (experimental fields, cages) at weekly intervals. Finally, we measured δ³4S in otoliths to test if these archival biominerals could be used to reconstruct floodplain use. Floodplain-reared salmon had a different diet composition and lower δ13C and δ³4S (δ¹³C = -33.02±2.66, δ³4S = -3.47±2.28; mean±1SD) compared to fish in the adjacent river (δ¹³C = -28.37±1.84, δ³4S = +2.23±2.25). These isotopic differences between habitats persisted across years of extreme droughts and floods. Despite the different diet composition, δ¹5N values from prey items on the floodplain (δ¹5N = 7.19±1.22) and river (δ¹5N = 7.25±1.46) were similar, suggesting similar trophic levels. The food web differences in δ13C and δ³4S between habitats were also reflected in salmon muscle tissue, reaching equilibrium between 24-30 days (2014, δ¹³C = -30.74±0.73, δ³4S = -4.6±0.68; 2016, δ¹³C = -34.74 ±0.49, δ³4S = -5.18±0.46). δ³4S measured in sequential growth bands in otoliths recorded a weekly time-series of shifting diet inputs, with the outermost layers recording time spent on the floodplain (δ³4S = -5.60±0.16) and river (δ³4S = 3.73±0.98). Our results suggest that δ¹³C and δ³4S can be used to differentiate floodplain and river rearing habitats used by native fishes, such as Chinook Salmon, across different hydrologic conditions and tissues. Together these stable isotope analyses provide a toolset to quantify the role of floodplains as fish habitats.
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Salmón/crecimiento & desarrollo , Fenómenos Fisiológicos Nutricionales de los Animales , Animales , Isótopos de Carbono/análisis , Ecosistema , Cadena Alimentaria , Isótopos de Nitrógeno/análisis , Ríos , Salmón/fisiología , Isótopos de Azufre/análisisRESUMEN
BACKGROUND: Aerodigestive clinics are run by interdisciplinary medical and surgical teams, and provide complex care coordination and combined endoscopies. OBJECTIVES: To describe the design and patient population of the first pediatric aerodigestive center in Israel. METHODS: A retrospective single-center cohort study was conducted describing patients followed in the aerodigestive clinic of Schneider Children's Medical Center of Israel, a tertiary pediatric hospital, between its inception in January 2017 and June 2020. RESULTS: During the study period, 100 patients were seen at the combined respiratory and digestive (NoAM) clinic, with a total of 271 visits. Median age at first assessment was 29.5 months (range 3-216). Fifty-six patients (56%) had esophageal atresia and tracheoesophageal fistula. Thirty-nine patients had an identified genetic disorder, 28 had a primary airway abnormality, 28 were oxygen dependent, and 21 were born premature. Fifty-two patients underwent triple endoscopy, consisting of flexible bronchoscopy, rigid bronchoscopy, and gastroscopy. In 33 patients, esophageal dilatation was necessary. Six patients underwent posterior tracheopexy at a median of 6 months of age (range 5 days to 8 years) all with ensuing symptom improvement. The total mean parental satisfaction score on a Likert-type scale of 1-5 (5 = highest satisfaction) was 4.5. CONCLUSIONS: A coordinated approach is required to provide effective care to the growing population of children with aerodigestive disorders. The cross fertilization between multiple disciplines offers a unique opportunity to develop high quality and innovative care. Outcome measures must be defined to objectively measure clinical benefit.
Asunto(s)
Enfermedades del Sistema Digestivo/terapia , Endoscopía/métodos , Grupo de Atención al Paciente/organización & administración , Atención al Paciente/métodos , Enfermedades Respiratorias/terapia , Adolescente , Niño , Preescolar , Estudios de Cohortes , Enfermedades del Sistema Digestivo/fisiopatología , Hospitales Pediátricos/organización & administración , Humanos , Lactante , Israel , Padres/psicología , Atención al Paciente/normas , Grupo de Atención al Paciente/normas , Satisfacción del Paciente , Calidad de la Atención de Salud , Enfermedades Respiratorias/fisiopatología , Estudios Retrospectivos , Centros de Atención Terciaria/organización & administraciónRESUMEN
Rearing habitat for juvenile Chinook Salmon (Oncorhynchus tshawytscha) in California, the southernmost portion of their range, has drastically declined throughout the past century. Recently, through cooperative agreements with diverse stakeholders, winter-flooded agricultural rice fields in California's Central Valley have emerged as ecologically functioning floodplain rearing habitat for juvenile Chinook Salmon. From 2013 to 2016, we conducted a series of experiments examining methods to enhance habitat benefits for fall-run Chinook Salmon reared on winter-flooded rice fields in the Yolo Bypass, a modified floodplain managed for flood control, agriculture, and wildlife habitat in the Sacramento River Valley of California. Investigations included studying the effect of 1) post-harvest field substrate; 2) depth refugia; 3) duration of field drainage; and 4) duration of rearing occupancy on in-situ diet, growth and survival of juvenile salmon. Post-harvest substrate treatment had only a small effect on the lower trophic food web and an insignificant effect on growth rates or survival of rearing hatchery-origin, fall-run Chinook Salmon. Similarly, depth refugia, created by trenches dug to various depths, also had an insignificant effect on survival. Rapid field drainage yielded significantly higher survival compared to drainage methods drawn out over longer periods. A mortality of approximately one third was observed in the first week after fish were released in the floodplain. This initial mortality event was followed by high, stable survival rates for the remainder of the 6-week duration of floodplain rearing study. Across years, in-field survival ranged 7.4-61.6% and increased over the course of the experiments. Despite coinciding with the most extreme drought in California's recorded history, which elevated water temperatures and reduced the regional extent of adjacent flooded habitats which concentrated avian predators, the adaptive research framework enabled incremental improvements in design to increase survival. Zooplankton (fish food) in the winter-flooded rice fields were 53-150x more abundant than those sampled concurrently in the adjacent Sacramento River channel. Correspondingly, observed somatic growth rates of juvenile hatchery-sourced fall-run Chinook Salmon stocked in rice fields were two to five times greater than concurrently and previously observed growth rates in the adjacent Sacramento River. The abundance of food resources and exceptionally high growth rates observed during these experiments illustrate the potential benefits of using existing agricultural infrastructure to approximate the floodplain wetland physical conditions and hydrologic patterns (shallow, long-duration inundation of cool floodplain habitats in mid-winter) under which Chinook Salmon evolved and to which they are adapted.
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Agricultura/métodos , Acuicultura/métodos , Salmón/crecimiento & desarrollo , Animales , California , Ecosistema , Granjas , Inundaciones , Oryza/crecimiento & desarrollo , Ríos , Estaciones del Año , HumedalesRESUMEN
Accurate methods for tracking individuals are crucial to the success of fisheries and aquaculture management. Management of migratory salmonid populations, which are important for the health of many economies, ecosystems, and indigenous cultures, is particularly dependent on data gathered from tagged fish. However, the physical tagging methods currently used have many challenges including cost, variable marker retention, and information limited to tagged individuals. Genetic tracking methods combat many of the problems associated with physical tags, but have their own challenges including high cost, potentially difficult marker design, and incompatibility of markers across species. Here we show the feasibility of a new genotyping method for parent-based tagging (PBT), where individuals are tracked through the inherent genetic relationships with their parents. We found that Rapture sequencing, a combination of restriction-site associated DNA and capture sequencing, provides sufficient data for parentage assignment. Additionally, the same capture bait set, which targets specific restriction-site associated DNA loci, can be used for both Rainbow Trout Oncorhynchus mykiss and Chinook Salmon Oncorhynchus tshawytscha. We input 248 single nucleotide polymorphisms from 1,121 samples to parentage assignment software and compared parent-offspring relationships of the spawning pairs recorded in a hatchery. Interestingly, our results suggest sperm contamination during hatchery spawning occurred in the production of 14% of offspring, further confirming the need for genetic tagging in accurately tracking individuals. PBT with Rapture successfully assigned progeny to parents with a 98.86% accuracy with sufficient genetic data. Cost for this pilot study was approximately $3 USD per sample. As costs vary based on the number of markers used and individuals sequenced, we expect that when implemented at a large-scale, per sample costs could be further decreased. We conclude that Rapture PBT provides a cost-effective and accurate alternative to the physical coded wire tags, and other genetic-based methods.
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Secuencia de Bases/genética , Salmonidae/genética , Animales , Acuicultura/métodos , Ecosistema , Femenino , Técnicas de Genotipaje/métodos , Masculino , Oncorhynchus mykiss/genética , Proyectos Piloto , Polimorfismo de Nucleótido Simple/genética , Salmón/genéticaRESUMEN
Similar to many large river valleys globally, the Sacramento River Valley has been extensively drained and leveed, hydrologically divorcing river channels from most floodplains. Today, the former floodplain is extensively managed for agriculture. Lack of access to inundated floodplains is recognized as a significant contributing factor in the decline of native Chinook Salmon (Oncorhynchus tshawytscha). We observed differences in salmon growth rate, invertebrate density, and carbon source in food webs from three aquatic habitat types-leveed river channels, perennial drainage canals in the floodplain, and agricultural floodplain wetlands. Over 23 days (17 February to 11 March, 2016) food web structure and juvenile Chinook Salmon growth rates were studied within the three aquatic habitat types. Zooplankton densities on the floodplain wetland were 53x more abundant, on average, than in the river. Juvenile Chinook Salmon raised on the floodplain wetland grew at 0.92 mm/day, 5x faster than fish raised in the adjacent river habitat (0.18 mm/day). Two aquatic-ecosystem modeling methods were used to partition the sources of carbon (detrital or photosynthetic) within the different habitats. Both modeling approaches found that carbon in the floodplain wetland food web was sourced primarily from detrital sources through heterotrophic pathways, while carbon in the river was primarily photosynthetic and sourced from in situ autotrophic production. Hydrologic conditions typifying the ephemerally inundated floodplain-shallower depths, warmer water, longer water residence times and predominantly detrital carbon sources compared to deeper, colder, swifter water and a predominantly algal-based carbon source in the adjacent river channel-appear to facilitate the dramatically higher rates of food web production observed in the floodplain. These results suggest that hydrologic patterns associated with seasonal flooding facilitate river food webs to access floodplain carbon sources that contribute to highly productive heterotrophic energy pathways important to the production of fisheries resources.
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Explotaciones Pesqueras/estadística & datos numéricos , Inundaciones , Cadena Alimentaria , Oncorhynchus/fisiología , Zooplancton/fisiología , Animales , California , Ciclo del Carbono , Hidrología , Modelos Biológicos , Dinámica Poblacional/estadística & datos numéricos , Ríos , Estaciones del Año , HumedalesRESUMEN
When inundated by floodwaters, river floodplains provide critical habitat for many species of fish and wildlife, but many river valleys have been extensively leveed and floodplain wetlands drained for flood control and agriculture. In the Central Valley of California, USA, where less than 5% of floodplain wetland habitats remain, a critical conservation question is how can farmland occupying the historical floodplains be better managed to improve benefits for native fish and wildlife. In this study fields on the Sacramento River floodplain were intentionally flooded after the autumn rice harvest to determine if they could provide shallow-water rearing habitat for Sacramento River fall-run Chinook salmon (Oncorhynchus tshawytscha). Approximately 10,000 juvenile fish (ca. 48 mm, 1.1 g) were reared on two hectares for six weeks (Feb-March) between the fall harvest and spring planting. A subsample of the fish were uniquely tagged to allow tracking of individual growth rates (average 0.76 mm/day) which were among the highest recorded in fresh water in California. Zooplankton sampled from the water column of the fields were compared to fish stomach contents. The primary prey was zooplankton in the order Cladocera, commonly called water fleas. The compatibility, on the same farm fields, of summer crop production and native fish habitat during winter demonstrates that land management combining agriculture with conservation ecology may benefit recovery of native fish species, such as endangered Chinook salmon.
Asunto(s)
Ecosistema , Inundaciones , Ríos , Salmón/crecimiento & desarrollo , Agricultura , Animales , California , Oryza/crecimiento & desarrollo , HumedalesRESUMEN
BACKGROUND: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. OBJECTIVES: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. METHODS: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel. RESULTS: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%). CONCLUSIONS: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members' contributions.
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Procedimientos Quirúrgicos Cardíacos/métodos , Cardiopatías Congénitas/cirugía , Grupo de Atención al Paciente/organización & administración , Procedimientos Quirúrgicos Cardíacos/mortalidad , Cardiopatías Congénitas/fisiopatología , Humanos , Recién Nacido , Israel , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
The ranges and abundances of species that depend on freshwater habitats are declining worldwide. Efforts to counteract those trends are often hampered by a lack of information about species distribution and conservation status and are often strongly biased toward a few well-studied groups. We identified the 3,906 vascular plants, macroinvertebrates, and vertebrates native to California, USA, that depend on fresh water for at least one stage of their life history. We evaluated the conservation status for these taxa using existing government and non-governmental organization assessments (e.g., endangered species act, NatureServe), created a spatial database of locality observations or distribution information from ~400 data sources, and mapped patterns of richness, endemism, and vulnerability. Although nearly half of all taxa with conservation status (n = 1,939) are vulnerable to extinction, only 114 (6%) of those vulnerable taxa have a legal mandate for protection in the form of formal inclusion on a state or federal endangered species list. Endemic taxa are at greater risk than non-endemics, with 90% of the 927 endemic taxa vulnerable to extinction. Records with spatial data were available for a total of 2,276 species (61%). The patterns of species richness differ depending on the taxonomic group analyzed, but are similar across taxonomic level. No particular taxonomic group represents an umbrella for all species, but hotspots of high richness for listed species cover 40% of the hotspots for all other species and 58% of the hotspots for vulnerable freshwater species. By mapping freshwater species hotspots we show locations that represent the top priority for conservation action in the state. This study identifies opportunities to fill gaps in the evaluation of conservation status for freshwater taxa in California, to address the lack of occurrence information for nearly 40% of freshwater taxa and nearly 40% of watersheds in the state, and to implement adequate protections for freshwater taxa where they are currently lacking.
Asunto(s)
Biodiversidad , Agua Dulce , Animales , California , Conservación de los Recursos Naturales , Invertebrados/clasificación , Plantas/clasificación , Vertebrados/clasificaciónRESUMEN
BACKGROUND: Autologous peripheral blood stem-cell collection (PBSCC) in children has become an integral part of contemporary treatment protocols, but the procedure is often complicated due to technical issues related to vascular access. Central line placement is often implemented to surmount this problem, but is associated with complications such as bleeding, thrombosis and pneumothorax. As an alternative we have introduced the use of radial arterial lines for PBSCC in children. PROCEDURE: Data from autologous stem cell collections performed from October 2002 to December 2011 using a radial arterial line were collected. RESULTS: A total of 372 PBSCC procedures were performed during the study period; an arterial line was used in 311 PBSCC's in 208 children. The average patient age and weight were 7.9 years (SD 5.4) and 28.3 kg (SD 20.4), respectively. The smallest patient was 9 months old and weighed 7 kg. The mean total volume processed was 8,593 cm(3) (SD 4,854), and the mean number of blood volumes processed was 4.3. Mean collection time for a single blood volume was 55 minutes (SD 15.5). The mean number of CD34+ cells collected per donation was 5.8 × 10(6) /kg. Ninety-seven patients (46%) required more than one collection to meet the requested CD34+ cell target. No serious adverse effects associated with vascular access occurred in this cohort. CONCLUSION: Percutaneous placement of radial artery catheters can be rapidly and safely performed in very small infants and in children with difficult venous access. This technique provides a reliable platform for efficient PBSCC.
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Trasplante de Células Madre Hematopoyéticas/métodos , Arteria Radial , Recolección de Tejidos y Órganos/métodos , Dispositivos de Acceso Vascular , Niño , Preescolar , Femenino , Células Madre Hematopoyéticas , Humanos , Lactante , Masculino , Recolección de Tejidos y Órganos/instrumentación , Trasplante AutólogoRESUMEN
BACKGROUND: Antegrade cerebral perfusion (ACP) is performed in neonates either by direct cannulation (DC) or indirect cannulation (IC) of the innominate artery. IC is achieved by a graft sutured to the innominate artery or advancement of a cannula through the ascending aorta into the innominate artery, whereas DC is performed by directly cannulating the innominate artery. These techniques may be limited by technical problems that can compromise perfusion. The purpose of the present study was to evaluate the flow measurements and safety of DC when compared with IC. METHODS: This was a retrospective chart review of consecutive neonates who underwent ACP from January 2007 to December 2010. Patient characteristics, surgical and hemodynamic measurements, and postoperative neurologic findings were recorded. RESULTS: Seventy neonates underwent ACP during the study period (46 using DC and 24 using IC). The groups were similar in age and weight. Operative variables were similar regarding cardiopulmonary bypass (CPB), cross-clamp times, maximal flow at full CPB, minimal temperature, ACP time, flow and flow index, and upper extremity blood pressure and proximal cannula pressure during ACP. There was a significantly higher flow index at full CPB in the DC group (217 ± 40 mL/kg/min versus 190 ± 46 mL/kg/min; p = 0.013), which correlated with higher proximal cannula pressures at full CPB (172 ± 27 mm Hg versus 158 ± 26 mm Hg; p = 0.04). Sixty-two of the 65 survivors (95%) had normal neurologic evaluations on discharge. CONCLUSIONS: ACP using DC is comparable to that using IC, with appropriate pressures in the proximal aortic line at full CPB and adequate upper extremity pressures during ACP, reflecting suitable flows in the cerebral circulation.
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Aorta Torácica/cirugía , Tronco Braquiocefálico/cirugía , Isquemia Encefálica/prevención & control , Cateterismo/métodos , Circulación Cerebrovascular/fisiología , Perfusión/métodos , Procedimientos Quirúrgicos Vasculares/métodos , Puente Cardiopulmonar/métodos , Femenino , Humanos , Hipotermia Inducida/métodos , Recién Nacido , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE/AIM: To compare hemodynamic parameters in infants with congenital heart disease (CHD) undergoing noncardiac surgery (NCS) under awake spinal anesthesia (SA) with controls without CHD also undergoing SA. BACKGROUND: NCS poses a twofold higher mortality risk in infants with CHD. SA might be a good alternative to general anesthesia (GA) in this setting. METHODS: The files of 84 infants were reviewed; 42 had CHD and 42 were controls without CHD. Primary outcome measures were percent decrease in mean arterial pressure (%MAP decrease) and heart rate (%HR decrease)from baseline to the lowest intraoperative value. One-way analysis of variance(ANOVA), ANOVA with repeated measures, Pearson chi-square test, Fisher's exact test, and Pearson correlation were used for statistical analysis.Time to discharge was analyzed with the nonparametric MannWhitney U-test. RESULTS: There were no significant between-group differences in %MAP decrease and %HR decrease; no significant associations and correlations between %MAP decrease or %HR decrease and other variables; and no correlation between %MAP decrease and %HR decrease. A %MAP decrease of >20% was documented in 11 patients with CHD (26.2%) and 10 controls (23.8%); a lowest intraoperative HR of <100 b.min)1 was recorded in two study patients (4.8%) and four controls (9.5%) (P = NS for both). There were no cases of high SA or conversion to GA and no need for mechanical ventilation or inotropic support intra/postoperatively. CONCLUSIONS: These preliminary findings show that hemodynamic parameters in infants with CHD undergoing NCS under awake SA are not different from controls without CHD and that SA appears to be safe in infants with CHD.
Asunto(s)
Anestesia Raquidea/métodos , Cardiopatías Congénitas/complicaciones , Procedimientos Quirúrgicos Operativos , Análisis de Varianza , Apnea/etiología , Presión Sanguínea/fisiología , Femenino , Edad Gestacional , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/fisiopatología , Frecuencia Cardíaca/fisiología , Hemodinámica/fisiología , Humanos , Lactante , Recién Nacido , Masculino , RiesgoRESUMEN
Providing anesthesia to patients with Brachmann-de Lange syndrome (BdLS) may be challenging, mainly because of intubation difficulty, gastroesophageal reflux disease, and aspiration complications. The use of spinal anesthesia (SA) in this population has not been reported. We report the uneventful administration of awake SA to a 7-month-old girl with BdLS who was scheduled for rectal biopsy. The current literature is reviewed to discuss the indications for SA in those patients.
Asunto(s)
Anestesia Raquidea , Síndrome de Cornelia de Lange/cirugía , Biopsia , Femenino , Reflujo Gastroesofágico/complicaciones , Enfermedad de Hirschsprung/diagnóstico , Humanos , Lactante , Intubación Intratraqueal , Enfermedades Pulmonares/complicaciones , Recto/patología , Aspiración Respiratoria/complicacionesRESUMEN
Myocarditis among pediatric patients varies in severity from mild disease to a fulminant course with overwhelming refractory shock and a high risk of death. Because the disease is potentially reversible, it is reasonable to deploy extracorporeal membranous oxygenation (ECMO) to bridge patients until recovery or transplantation. This study aimed to review the course and outcome of children with acute fulminant myocarditis diagnosed by clinical and echocardiographic data only who were managed by ECMO because of refractory circulatory collapse. A chart review of a single center identified 12 children hospitalized over an 8-year period who met the study criteria. Data were collected on demographics, diagnosis, disease course, and outcome. The patients ranged in age from 20 days to 8 years (25.5 ± 29.6 months). Echocardiography showed a severe global biventricular decrease in myocardial function, with a shortening fraction of 12% or less. Ten children (83.3%) were weaned off extracorporeal support after 100-408 h (mean, 209.9 ± 82.4 h) and discharged home. Two patients died: one due to multiorgan failure and one due to sustained refractory heart failure. During a long-term follow-up period, all survivors showed normal function in daily activities and normal myocardial function. The study showed that ECMO can be safely and successfully used for children with acute fulminant myocarditis diagnosed solely on clinical and radiographic grounds who need mechanical support. These patients usually have a favorable outcome, regaining normal or near normal heart function without a need for heart transplantation.
Asunto(s)
Oxigenación por Membrana Extracorpórea , Miocarditis/terapia , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Miocarditis/diagnóstico por imagen , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , UltrasonografíaRESUMEN
INTRODUCTION: Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction. MATERIALS AND METHODS: Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Children's Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed. RESULTS: Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operative mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised. CONCLUSIONS: Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.
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Aorta/anomalías , Aorta/cirugía , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Anastomosis Quirúrgica , Cateterismo Cardíaco , Procedimientos Quirúrgicos Cardiovasculares/métodos , Diagnóstico Diferencial , Ecocardiografía , Humanos , Hipertensión Pulmonar/etiología , Lactante , Recién NacidoRESUMEN
PURPOSE: Pain is the main cause of patient distress/dissatisfaction after strabismus surgery. The aim of the study was to evaluate the effect of sub-Tenon's block with ropivacaine at the end of strabismus surgery on post-operative pain. METHODS: A prospective trial was conducted in 79 patients (age 1.0-65 years) scheduled for outpatient primary strabismus surgery with fixed sutures under general anesthesia (GA) at a major tertiary hospital. Half the patients were randomly allocated to receive sub-Tenon's block with ropivacaine 0.2% at conclusion of the operation. Primary outcome measures were visual analog scale (VAS) scores at arrival to the post-anesthesia care unit (PACU), at discharge 3 hr later, 12-16 hr post-operatively, and 24 hr post-operatively. Supplemental analgesia requirements and patient satisfaction were recorded as well. Data were presented as median (range). Mann-Whitney test, Pearson chi(2)-test or Fisher's exact test was used for statistical analysis; p Asunto(s)
Amidas/administración & dosificación
, Anestésicos Locales/administración & dosificación
, Dolor Postoperatorio/tratamiento farmacológico
, Estrabismo/cirugía
, Adolescente
, Adulto
, Anciano
, Procedimientos Quirúrgicos Ambulatorios
, Anestesia General
, Niño
, Preescolar
, Tejido Conectivo
, Ojo
, Femenino
, Humanos
, Lactante
, Inyecciones Intraoculares
, Masculino
, Persona de Mediana Edad
, Dimensión del Dolor
, Dolor Postoperatorio/fisiopatología
, Satisfacción del Paciente
, Ropivacaína
, Suturas
, Adulto Joven
RESUMEN
BACKGROUND: Peripherally inserted central venous catheters (PICCs) are frequently used in infants and children. However, only limited data are available on current infectious and noninfectious complications of PICCs in this patient population. The aim of the study is to define the current rate of infectious and noninfectious complications of PICCs, the causative agents, and to define possible risk factors associated with these complications. METHODS: A prospective surveillance study was conducted at the Schneider Children Medical Center of Israel. All patients in whom a PICC was inserted between August 2004 and October 2006 were included. Demographic, clinical, and microbiologic data were collected by a questionnaire completed by the anesthesiologist at the time of insertion and removal of each catheter. Items covered epidemiologic, clinical, and microbiologic data. RESULTS: A total of 279 PICCs were inserted in 221 patients. There were no complications at insertion. Mean dwell time was 30 days. One hundred seventy-seven (63%) of all PICC placements were free of complications. Twenty-six catheters (9.3%) were dislodged accidentally; 38 (13.6%) were removed for mechanical problems: tears in 5, leaks in 12, and obstructions in 21; 38 (13.6%) were removed for an infectious complication: phlebitis in 13 (4.6%, 1.5/1000 PICC days), exit-site infection in 10 (3.5%, 1.1/1000 PICC days), PICC-associated bloodstream infection in 12 (4.3%, 1.4/1000 PICC days), and PICC-related bloodstream infection in 4 (1.4%, 0.4/1000 PICC days); 15 more were removed for presumed infection (5.3%, 1.7/1000 PICC days). On multivariate analysis, composite indication for PICC use and older patient age were significantly associated with infectious complications. CONCLUSIONS: PICCs are safe and may be used for prolonged periods. In our center, the rates of infectious complications are lower than for tunneled central venous catheters. Accidental dislodgement is not uncommon and may be prevented by use of sutures, occlusive dressing, and education of patients, families, and medical staff.
Asunto(s)
Infecciones Relacionadas con Catéteres/epidemiología , Cateterismo Venoso Central/efectos adversos , Adolescente , Factores de Edad , Niño , Preescolar , Femenino , Hospitales Pediátricos , Humanos , Lactante , Recién Nacido , Israel , Masculino , Estudios Prospectivos , Factores de Riesgo , Encuestas y CuestionariosRESUMEN
PURPOSE: When pyloromyotomy for hypertrophic pyloric stenosis (HPS) is performed under general anesthesia, metabolic abnormalities and fluid deficits coupled with residual anesthetics may increase the risk of postoperative apnea, thereby, prolonging operating room time and delaying extubation. Spinal anesthesia has been found to reduce the rate of postoperative apnea in high-risk infants. The aim of the study was to evaluate the effect of spinal vs general anesthesia on operating room time in infants undergoing open pyloromyotomy. METHODS: Data for 60 infants who underwent pyloromyotomy under spinal (n = 24) or general (n = 36) anesthesia at a tertiary pediatric medical center were derived from the computerized database. Primary outcome measures were total operating room time, procedure duration, anesthesia release time, wake-up time, and anesthesia control time (anesthesia release plus wake-up). Nonparametric Mann-Whitney test was used for statistical analysis, and Levene's test was used to assess the equality of variances in samples; P Asunto(s)
Anestesia Raquidea/estadística & datos numéricos
, Quirófanos/estadística & datos numéricos
, Estenosis Hipertrófica del Piloro/cirugía
, Anestesia General/métodos
, Anestesia General/estadística & datos numéricos
, Anestesia Raquidea/métodos
, Apnea/prevención & control
, Procedimientos Quirúrgicos del Sistema Digestivo/métodos
, Procedimientos Quirúrgicos del Sistema Digestivo/estadística & datos numéricos
, Femenino
, Humanos
, Lactante
, Masculino
, Sistemas de Registros Médicos Computarizados/estadística & datos numéricos
, Evaluación de Resultado en la Atención de Salud
, Complicaciones Posoperatorias/prevención & control
, Estenosis Hipertrófica del Piloro/congénito
, Factores de Tiempo
, Vigilia
RESUMEN
PURPOSE: We compared the effectiveness of post-pyeloplasty epidural and nonepidural analgesia in children. MATERIAL AND METHODS: After flank dismembered open pyeloplasty 45 children were randomly allocated to group 1-continuous epidural analgesia with a loading dose of 0.25% bupivacaine or 0.2% ropivacaine, followed by continuous drip 0.2% ropivacaine (26) and group 2-nonepidural analgesia with intraoperative morphine intravenously and postoperative oxycodone or tramadol by age plus ibuprofen or paracetamol for 48 hours. The same rescue regimen was used in each group. Pain was scored separately by parents and the ward nurse using the Face, Legs, Activity, Cry and Consolability Scale or a visual analog scale. RESULTS: In groups 1 and 2 mean age was 2.8 and 4.7 years, respectively. There was no significant between group difference in pain degree as scored by the nurse (none in 53.8% vs 36.8% of patients, mild in 26.9% vs 21.1% and moderate in 19.2% vs 42.1%) and parents (none in 40% vs 23%, mild in 20% vs 11.8% and moderate in 40% vs 65.2%) (p >0.05). Rescue treatment 48 to 72 hours after surgery was received by 80% of group 1 and 76% of group 2 for mild pain and by 4% and 30% for moderate pain (p >0.05 and 0.02, respectively). Mean time to mobilization in the 2 groups was 25 and 17 hours, and mean time to discharge home was 4.9 and 4.1 days (p = 0.05 and >0.05, respectively). CONCLUSIONS: Nonepidural analgesia is as effective as continuous epidural analgesia to control post-pyeloplasty pain in children. A noninvasive analgesic regimen is recommended when there is a relative contraindication to line insertion, a less experienced anesthetist is available or parents prefer it.
Asunto(s)
Analgesia Epidural , Pelvis Renal/cirugía , Dolor Postoperatorio/prevención & control , Adolescente , Analgesia/métodos , Analgesia Epidural/métodos , Niño , Preescolar , Humanos , LactanteRESUMEN
BACKGROUND: Vascular access is often technically difficult in children because of the small caliber and impalpability of the veins. In this study, we sought to determine if use of the Vein Entry Indicator Device (VEIDtrade mark) in children facilitates peripheral venous access. METHODS: Two-hundred-two healthy (ASA grade I and II) children scheduled for same-day surgery at a major tertiary hospital in Israel were randomly allocated to undergo VEID-assisted or standard peripheral venous cannulation. All cases involved the insertion of a 22-gauge cannula into an upper limb vein. Primary outcome measures were number of attempts to successful cannulation, rate of success at first attempt, and time required for insertion. The data were presented as mean (sd). Analyses of variance and Pearson chi(2) test or Fisher's exact test were used to compare the groups; forward stepwise logistic regression was used to identify the three variables (age, vein assessment category, use of the VEID) significantly associated with a successful first attempt. A P value of < or =0.05 was considered significant. RESULTS: Successful cannulation was achieved at the first attempt in the majority of patients in both groups. Two attempts were needed in 8% of the VEID group and 28% of the control group, and 3 attempts were needed in 1% and 3%, respectively (P < 0.01). Analysis by vein assessment category yielded a similar rate of successful first-attempt cannulations in the two groups for easy veins. However, for the difficult and intermediate categories, the rate was 89.7% in the VEID group compared to 23.3% in the control group (P < 0.001). The fewer number of attempts in the study group was associated with a shorter time from the start of the search for an appropriate vein to successful cannulation (9.1 s versus 22.5 s in the control group). CONCLUSIONS: The VEID facilitates the insertion of peripheral venous cannulas in healthy children with intermediate/difficult veins undergoing same-day surgery, reducing the number of attempts and the overall time required.