El pico flujo espiratorio y la cuenta máxima son marcadores del compromiso respiratorio en la miastenia gravis / Peak expiratory flow and the single-breath counting test as markers of respiratory function in patients with myasthenia gravis

Introducción: La miastenia gravis (MG) es una enfermedad autoinmune mediada por anticuerpos. El cuadro clínico se caracteriza por debilidad muscular fluctuante y fatigable, con frecuente afectación de músculos fonodeglutorios y respiratorios. Dada la severidad que implica el compromiso respiratorio en la MG, su evaluación rutinaria es esencial.Nuestro objetivo fue identificar un marcador semiológico útil en la pesquisa del compromiso respiratorio en pacientes con MG.Métodos: Se realizó un trabajo observacional en pacientes con diagnóstico de MG. Los pacientes fueron evaluados con test de cuenta máxima, pico flujo espiratorio (PEF), cuestionario de disnea modificado (mMRC) y valoración de fuerza del cuello. Los resultados de estos parámetros fueron correlacionados con la medición de CVF (capacidad vital forzada) y presiones bucales estáticas máximas (PiMáx y PeMáx). Resultados:Cuarenta y cinco pacientes con MG fueron incluidos, dos pacientes tenían MGFA grado I, 35 grado II, siete grado III y uno grado IV al momento de la evaluación. Se halló una correlación positiva entre el test de cuenta máxima y la CVF (r = 0,57, p = 0,000), y entre el PEF y la CVF (r = 0,76, p = 0,000). El grado de disnea, según el mMRC, mostró una correlación negativa con la CVF (r =-0,31 p = 0,03). A su vez, el PEF correlacionó con la PeMáx de forma positiva, estadísticamente significativa (r = 0,51, p = 0,002).Conclusiones: El PEF, el test de cuenta máxima y el mMRC fueron útiles para evaluar la función respiratoria en pacientes con MG.(AU)

Introduction: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterised by fluctuating, fatigable muscle weakness, frequently involving bulbar and respiratorymuscles. Considering the severity of respiratory involvement in MG, routine evaluation ofrespiratory function is essential.The aim of this study was to identify a useful clinical marker of respiratory involvement inpatients with MG. Methods: We performed an observational study of patients with MG. All cases were evaluatedwith the single-breath counting test, peak expiratory flow (PEF), a modified Medical ResearchCouncil dyspnoea scale (mMRC), and a neck strength assessment. The results of these parameters were correlated with forced vital capacity (FVC), maximal inspiratory pressure (MIP), andmaximal expiratory pressure (MEP). Results: The study included 45 patients with MG: 2 patients classified as grade I on the Myasthenia Gravis Foundation of America classification at the time of evaluation, 35 classified asgrade II, 7 classified as grade III, and one classified as grade IV. Positive correlations were foundbetween single-breath counting test scores and FVC values (r = 0.57, p = .000), and betweenPEF and FVC values (r = 0.76, p = .000). Severity of dyspnoea according to the mMRC scaleshowed a negative correlation with FVC values (r = -0.31, p = .03). PEF also showed a significantcorrelation with MEP (r = 0.51, p = .002). Conclusions: PEF, the single-breath counting test, and the mMRC scale are useful measures forevaluating respiratory function in patients with MG.(AU)

A dynamic model to measure retention of solid and liquid digesta fractions in chickens fed diets with differing fibre sources.

Dietary fibres impact multiple digestive processes, and insights into the effects of various types of fibre on digesta retention time are required to optimise current feed formulation systems. Therefore, the objective of this study was to apply a dynamic modelling approach to generate estimates for the retention time of solid and liquid digesta in broilers fed different fibre sources. A maize-wheat-soybean meal control diet was compared against three diets in which wheat was partially substituted with oat hulls, rice husks, or sugar beet pulp (3% w/w). Non-starch polysaccharide (NSP) digestibility was evaluated in broilers between 23 and 25 days of age (n = 60 birds/treatment) using titanium dioxide (TiO2, 0.5 g/kg) as a marker, after feeding the experimental diets for 21 days. Digesta mean retention time (MRT) was measured in another 108 birds at 30 days of age by the administration of an oral pulse dose of chromium sesquioxide (Cr2O3) as solid marker and Cobalt-EDTA as liquid marker, and subsequent measurement of marker recovery in compartments of the digestive tract (n = 2 or 3 replicate birds/time point/treatment). Marker recovery models to estimate fractional passage rates for solid and liquid digesta in crop, gizzard, small intestine, and caeca compartments of the gastrointestinal tract were developed to predict MRT of solid and liquid digesta for each dietary treatment. The models were composed of a series of first-order differential equations, representing the variation of marker concentration in a compartment over time. Estimated MRT of solid and liquid digesta in the gizzard varied from 20 min for oat hulls and 34 min for rice husks diets to 14 min for sugar beet pulp and 12 min for control diets. In the caeca, liquid MRT was decreased compared to the control diet (989 min) for the sugar beet pulp diet (516 min), while it was increased for both the oat hulls and rice husks diets (≈1 500 min). Overall, these estimates are greater than those previously reported, suggesting that liquid digesta retention in the caeca previously has been underestimated. Digestibility of total NSP was increased by dietary fibre inclusion, regardless of the fibre type, although degradation of constituent sugars of NSP varied among diets. In conclusion, the inclusion of fibre sources at a low level (3% w/w) in the diet of broiler modulated retention time mainly in the gizzard and caeca, and increased digestibility of NSP.

Long-term effects of dietary calcium and phosphorus level, and feed form during rearing on egg production, eggshell quality, and bone traits in brown laying hens from 30 to 89 wk of age.

The effects of feeding strategies during rearing (0-16 wk) of brown laying hens on mid and end laying performance (30-89 wk) were studied. The rearing feeding strategies followed a 3 × 2 factorial arrangement with feed form; mash with inclusion of 3% finely ground wheat straw (MWS), crumbles with inclusion of 3% finely ground wheat straw (CWS), and crumbles with inclusion of 3% unground oat hulls as fiber sources (COH) at 2 dietary Ca and P levels (high or low Ca-P). Feed conversion ratio improved with COH and MWS compared with CWS from 30 to 59 wk. Rate of lay and egg mass production showed a feed form × Ca-P interaction from 60 to 89 wk. Low Ca-P led to a higher egg production, but only when COH and MWS were fed. BW at 89 wk was higher with CWS compared to COH and MWS. BW uniformity was better with COH compared to MWS at 51 wk and both CWS and MWS at 67 wk. Tibia characteristics were not clearly affected by treatment, although there was a feed form × Ca-P interaction on compression at 89 wk, where compression was lower with MWS and low vs. high Ca-P. Low Ca-P during rearing led to higher eggshell thickness, compared to high Ca-P at 45 wk of age, but breaking strength was lower with low vs. high Ca-P at 75 wk. Although eggshell quality was affected by Ca-P and there were some interactions with feed form at some ages, the effect was not consistent. There was no clear relationship between eggshell quality and tibia characteristics. It was concluded that feeding low Ca-P in combination with COH and MWS during the rearing period positively affects egg production during late lay. Also, dietary Ca-P levels, compared to commercial practice, can be lowered during rearing, as this will not affect eggshell quality and bone mineralization at later ages.

Peak expiratory flow and the single-breath count test as markers of respiratory function in patients with myasthenia gravis.

INTRODUCTION: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterised by fluctuating, fatigable muscle weakness, frequently involving bulbar and respiratory muscles. Considering the severity of respiratory involvement in MG, routine evaluation of respiratory function is essential. The aim of this study was to identify a useful clinical marker of respiratory involvement in patients with MG. METHODS: We performed an observational study of patients with MG. All cases were evaluated with the single-breath count test, peak expiratory flow (PEF), a modified Medical Research Council dyspnoea scale (mMRC), and a neck strength assessment. The results of these parameters were correlated with forced vital capacity (FVC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). RESULTS: The study included 45 patients with MG: 2 patients classified as grade I on the Myasthenia Gravis Foundation of America classification at the time of evaluation, 35 classified as grade II, 7 classified as grade III, and one classified as grade IV. Positive correlations were found between single-breath count test scores and FVC values (r = 0.57, P = .000), and between PEF and FVC values (r = 0.76, P = .000). Severity of dyspnoea according to the mMRC scale showed a negative correlation with FVC values (r = -0.31, P = .03). PEF also showed a significant correlation with MEP (r = 0.51, P = .002). CONCLUSIONS: PEF, the single-breath count test, and the mMRC scale are useful measures for evaluating respiratory function in patients with MG.

Design of a compact off-axis four-mirror objective system for a thermal camera.

The optical design of a compact off-axis four-mirror objective system (OFOS) of a thermal camera for a syndromic surveillance system that captures bioclinical signals, like temperature, directly related to the physical symptoms of the COVID-19 disease through thermal images is presented. The OFOS design works on wavelengths of 7.5-14 µm, with an f-number of 4, and a field of view (FOV) of 24×4 deg.

[Evaluation of bleeding, pain and operative time of the midvasto versus medial parapatellar approach in primary total knee arthroplasty for grade IV osteoarthrosis]. / Evaluación del sangrado, dolor y tiempo quirúrgico del abordaje midvasto versus parapatelar medial en artroplastía total de rodilla primaria por osteoartrosis grado IV.

INTRODUCTION: total knee arthroplasty is a common procedure in orthopedic surgery for treating grade IV knee osteoarthritis. This procedure reduces pain and improves functionality. However, the results according to the approach are different, it is not clear which surgical approach is clearly superior. The objective of this study is to evaluate the time and post-surgical bleeding, as well as the postoperative pain of the midvasto versus medial parapatellar approach in primary total knee arthroplasty in grade IV gonarthrosis. MATERIAL AND METHODS: an observational, comparative, retrospective study was carried out from June 1, 2020 to December 31, 2020, including beneficiaries of the Mexican Social Security Institute over 18 years of age with a diagnosis of grade IV knee osteoarthritis scheduled for primary total knee arthroplasty in the absence of other inflammatory pathology, previous osteotomies or coagulopathies. RESULTS: of 99 patients who underwent the midvasto approach (group M) and 100 patients to the medial parapatellar approach (group T), there was preoperative hemoglobin 14.7 g/l group M and 15.2 g/l group T, reduction was 5.0 g/l group M and 4.6 g/l group T. Significant pain reduction in both groups without significant difference; from 6.7 to 3.2 group M and from 6.7 to 3.1 group T. The surgical time was significantly longer with the medial parapatellar approach (98.7 versus 89.2 minutes). CONCLUSIONS: both approaches represent an excellent access route to perform primary total knee arthroplasty; however, no significant differences were found in the volume of bleeding or in the reduction of pain, the midvaste approach was associated with shorter surgical time and less involvement of flexion of the knee. Therefore, the midvasto approach is recommended in patients undergoing primary total knee arthroplasty.

INTRODUCCIÓN: la artroplastía total de rodilla es un procedimiento común en la cirugía ortopédica de tratamiento para gonartrosis grado IV. Este procedimiento disminuye el dolor y mejora la funcionalidad. Sin embargo, los resultados según el abordaje son distintos, no existe evidencia sobre qué abordaje quirúrgico sea claramente superior. El objetivo de este estudio es evaluar el tiempo y sangrado transquirúrgico, así como el dolor postquirúrgico del abordaje midvasto versus parapatelar medial en artroplastía total de rodilla primaria en gonartrosis grado IV. MATERIAL Y MÉTODOS: se realizó estudio observacional, comparativo, retrospectivo del 01 de Junio de 2020 al 31 de Diciembre de 2020 incluyendo derechohabientes del Instituto Mexicano del Seguro Social, mayores de 18 años con diagnóstico de gonartrosis grado IV, programados para artroplastía total de rodilla primaria en ausencia de otra patología inflamatoria, osteotomías previas o coagulopatías. RESULTADOS: noventa y nueve pacientes que fueron sometidos a abordaje midvasto (grupo M) y 100 pacientes a abordaje parapatelar medial (grupo T) con hemoglobina preoperatoria 14.7 g/l grupo M y 15.2 g/l grupo T, la reducción fue de 5.0 g/l grupo M y 4.6 g/l grupo T. Dolor en ambos grupos sin diferencia significativa; de 6.7 a 3.2 grupo M y de 6.7 a 3.1 grupo T. Tiempo quirúrgico mayor con el abordaje parapatelar medial (98.7 versus 89.2 minutos). CONCLUSIONES: ambos abordajes representan excelente vía de acceso para realizar artroplastía total primaria de rodilla; sin embargo, no se encontraron diferencias significativas en el volumen de sangrado ni en la reducción del dolor, el abordaje midvasto se asoció con menor tiempo quirúrgico y menor afectación de la flexión de la rodilla, por lo que se recomienda el abordaje midvasto en pacientes sometidos a artroplastía total de rodilla primaria.

[Inter-observer agreement in the Paprosky classification in a hospital in a country of an emerging economy]. / Concordancia interobservador en la clasificación de Paprosky en un hospital de un país de una economía emergente.

INTRODUCTION: The evaluation and management of acetabular bone loss in total hip arthroplasty is a challenge for orthopedic surgeons, we wish in this study to determine the inter-observer concordance in the Paprosky classification for acetabular defects. MATERIAL AND METHODS: Observational, descriptive, longitudinal, prospective study. It was performed using anteroposterior hip radiographs of patients scheduled for primary total hip arthroplasty or revision with acetabular bone defect, Sinapse local X-ray system, 20 X-rays were assessed per observer taken randomly (doctors assigned to the joint replacement service with minimum experience of five years) using the Paprosky classification spontaneously, (without previous classes) for acetabular defects, inter-observer concordance was assessed using Cohen's Kappa coefficient. RESULTS: An interobserver agreement was obtained on average for the Paprosky classification of acetabular defects using the Cohen's Kappa coefficient, the concordance analysis was done using the statistical program statistical package for the social sciences of 0.237. CONCLUSIONS: Both the null hypothesis (Kappa greater than 0.80 value considered optimal) and the alternative hypothesis (Kappa from 0.41 to 0.80 value equal to moderate to good) did not agree with our study (Kappa equal to 0.237) value equal to mild, in addition , it was shown that the greater the acetabular defect, the lower the interobserver agreement in surgeons in our institution.

INTRODUCCIÓN: La evaluación y manejo de la pérdida ósea acetabular en la artroplastía total de cadera es un desafío para los cirujanos ortopédicos, deseamos en este estudio determinar la concordancia interobservador en la clasificación de Paprosky para defectos acetabulares. MATERIAL Y MÉTODOS: Estudio observacional, descriptivo, longitudinal, prospectivo. Se realizó utilizando las radiografías anteroposteriores de cadera de pacientes programados para artroplastía total de cadera primaria o de revisión con defecto óseo acetabular, sistema de rayos X local Sinapse, se valoraron 20 radiografías por observador tomadas de manera aleatoria (médicos adscritos al servicio de reemplazos articulares con experiencia mínima de cinco años) utilizando la clasificación de Paprosky de manera espontánea (sin clases previas) de defectos acetabulares, se evaluó la concordancia interobservador mediante el coeficiente de kappa de Cohen. RESULTADOS: Se obtuvo una concordancia interobservador en promedio para la clasificación de Paprosky de defectos acetabulares utilizando el coeficiente de kappa de Cohen, el análisis de concordancia se hizo empleando el programa estadístico Statistical Package for the Social Sciences de 0.237. CONCLUSIONES: Tanto la hipótesis nula (kappa mayor de 0.80 valor considerado óptimo) como la hipótesis alternativa (kappa de 0.41 a 0.80 valor igual a moderada a buena) no concordaron con nuestro estudio (kappa igual a 0.237) valor igual a leve, además se demostró que a mayor defecto acetabular menor es la concordancia interobservador en cirujanos en nuestra institución.

Incidence and risk factors for acute kidney injury: a prospective, randomized, single-blind clinical trial comparing total knee arthroplasty with and without tourniquet.

PURPOSE: Identify the incidence and risk factors for acute kidney injury (AKI) following total knee arthroplasty (TKA) with and without tourniquet. MATERIAL AND METHODS: 100 patients were randomized into two groups. Postoperative AKI was defined as the postoperative creatinine level 0.3 mg/dl compared with baseline. Potential variables associated with AKI were analyzed by multivariate logistic regression model to identify the AKI risk factors in TKA patients with and without tourniquet. RESULTS: AKI rate was 22%, tourniquet use (OR = 2.66, p = 0.014), blood loss > 500 cm3 (OR = 3.99, p = 0.001), postoperative Hb < 10 g/dl (OR = 2.68, p = 0.008), blood transfusions (OR = 2.86, p = 0.012) and diabetes (OR = 2.80, p = 0.006) were associated with increased risk of postoperative AKI. CONCLUSIONS: The use of tourniquet should be indicated with caution and should not be used routinely in patients with other risk factors for the development of acute kidney dysfunction, other measures to achieve trans-surgical hemostasis should be implemented in our environment to reduce the incidence of acute kidney dysfunction related to the use of the tourniquet.

PROPÓSITO: Identificar la incidencia y factores de riesgo para lesión renal aguda (LRA) después de la artroplastia total de rodilla (ATR) con y sin uso de torniquete. MATERIAL Y MÉTODOS: Se dividieron 100 pacientes en dos grupos. Se definió la LRA como una elevación postoperatoria de la creatinina 0.3 mg/dl comparada con el nivel basal preoperatorio. Las potenciales variables asociadas con la DRA fueron analizadas con un modelo de regresión logística multivariada para identificar los factores de riesgo de DRA en pacientes sometidos a ATR con y sin torniquete. RESULTADOS: La incidencia de LRA fue de 22%. El uso de torniquete (OR = 2.66, p = 0.014), pérdida sanguínea > 500 cm3 (OR = 3.99, p = 0.001), Hb postoperatoria < 10 g/dl (OR = 2.68, p = 0.008), transfusión sanguínea (OR = 2.86, p = 0.012) y la diabetes (OR = 2.80, p = 0.006) fueron asociados a un mayor riesgo postoperatorio de LRA. CONCLUSIONES: El uso de torniquete debe estar indicado con precaución y no debe utilizarse de forma rutinaria en pacientes con otros factores de riesgo para el desarrollo de disfunción renal aguda, otras medidas para lograr la hemostasia transquirúrgica deben implementarse en nuestro entorno para reducir la incidencia de disfunción renal aguda relacionada con el uso del torniquete.

[Family outbreak caused by the nematode Trichostrongylus colubriformis in a rural area of the province of Valdivia: a rare occurrence zoonoses]. / Brote familiar por el nematodo Trichostrongylus colubriformis en una zona rural de la provincia de Valdivia: una zoonosis de rara ocurrencia.

Trichostrongylosis is a rare occurrence zoonosis caused by intestinal nematodes, favored by the consumption of raw vegetables or water contaminated with infective larvae of Trichostrongylus spp. In 2015, a family outbreak of foodborne disease was registered in a rural zone of Valdivia, affecting to 51-year-old woman and her 56-year-old partner and her 12-year-old daughter. Only the adult woman, the index case, presented symptoms such as diarrhea, abdominal pain, asthenia, and nausea. The patients revealed Trichostrongylidae gen. sp. eggs in their stools, identifying Trichostrongylus colubriformis adults in two of them. They had a good response to treatment with albendazol. In Chile, 0.1 to 3.5% prevalence of infection has been reported, including a total of 93 cases recorded between the provinces of Concepción and Llanquihue.

Brote familiar por el nematodo Trichostrongylus colubriformis en una zona rural de la provincia de Valdivia: una zoonosis de rara ocurrencia / Family outbreak caused by the nematode Trichostrongylus colubriformis in a rural area of the province of Valdivia: a rare occurrence zoonoses

Resumen La trichostrongylosis es una zoonosis de rara ocurrencia causada por nematodos intestinales, favorecida por el consumo de vegetales crudos o agua contaminada con larvas infectantes de Trichostrongylus spp. En 2015, se registró un brote familiar transmitido por alimentos en una zona rural de Valdivia, afectando a una mujer de 51 años de edad y su pareja e hija de 56 y 12 años; respectivamente. Solo la mujer adulta, el caso índice, presentó síntomas como diarrea, dolor abdominal, astenia y náuseas. Los pacientes eliminaron huevos de Trichostrongylidae gen. sp. en sus deposiciones, identificándose en dos de ellos, adultos de Trichostrongylus colubriformis. Tuvieron una buena respuesta a albendazol. En Chile, se han registrado prevalencias de infección de 0,1 a 3,5%, incluyendo un total de 93 casos diagnosticados entre las provincias de Concepción y Llanquihue.

Abstract Trichostrongylosis is a rare occurrence zoonosis caused by intestinal nematodes, favored by the consumption of raw vegetables or water contaminated with infective larvae of Trichostrongylus spp. In 2015, a family outbreak of foodborne disease was registered in a rural zone of Valdivia, affecting to 51-year-old woman and her 56-year-old partner and her 12-year-old daughter. Only the adult woman, the index case, presented symptoms such as diarrhea, abdominal pain, asthenia, and nausea. The patients revealed Trichostrongylidae gen. sp. eggs in their stools, identifying Trichostrongylus colubriformis adults in two of them. They had a good response to treatment with albendazol. In Chile, 0.1 to 3.5% prevalence of infection has been reported, including a total of 93 cases recorded between the provinces of Concepción and Llanquihue.

The effects of dietary calcium and phosphorus level, and feed form during rearing on growth performance, bone traits and egg production in brown egg-type pullets from 0 to 32 weeks of age.

In a 3 × 2 factorial arrangement, effects of feed form (crumbles (CWS), mash (MWS), both with inclusion of 3% finely ground wheat straw, or crumbles with inclusion of 3% oat hulls (COH)), and dietary Ca and P (high and low Ca-P) from 0 to 16 wk of age were studied on growth performance, bone characteristics, and gizzard development of egg-type pullets. The cross-over effect of feeding strategy during rearing on laying performance and egg shell quality was studied from 19 to 32 wk of age. From 0 to 16 wk, ADG, ADFI, and feed conversion ratio (FCR) were improved with CWS and COH compared to MWS, but ADG and FCR were improved with MWS compared to CWS and COH from 11 to 16 wk. Uniformity of BW till 11 wk, and tibia breaking strength at 6 and 16 wk were higher with CWS and COH compared to MWS. Tibia ash content at 11 wk and relative empty proventriculus + gizzard weight (EPG) were lower with CWS and COH compared to MWS, also relative EPG at 11 and 16 wk was higher with COH compared to CWS. At 25 wk BW was lower with MWS compared to CWS and COH, but BW was equal for all treatments at 32 wk. The FCR for egg production was improved with COH compared to MWS. Egg shell parameters were not affected by feed form during rearing. Low Ca-P decreased BW uniformity at 6 wk, relative keel bone weight and ash content at 11 wk, tibia ash content at 11 and 16 wk, increased relative EPG at 6 wk, and improved egg shell quality at 32 wk of age. It was concluded that feeding CWS and COH compared to MWS increased growth performance, but had no clear cross-over effect on egg production. Low dietary Ca-P led to a lower bone mineralization during rearing, nevertheless improved egg shell quality at 32 wk.

Children with Kawasaki disease or Kawasaki-like syndrome (MIS-C/PIMS) at the time of COVID-19: are they all the same? Case series and literature review.

Since the coronavirus disease 2019 (COVID-19) outbreak started, children have been considered marginally involved compared to adults, with a quite significant percentage of asymptomatic carriers. Very recently, an overwhelming inflammatory activation, which shares clinical similarities with Kawasaki disease (KD), has been described in children exposed to COVID-19. We report three KD-like cases that occurred during the pandemic of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a highly affected area of Northern Italy. The clinical presentation was characterized by the presence of unremitting fever, diarrhea and elevated inflammatory markers. Case #1 and Case #2 occurred one week apart and shared other clinical features: laboratory tests confirmed COVID-19 exposure and high inflammatory activation with myocardial involvement. Case #3 followed a more typical pattern for KD. Interestingly, this patient showed lower levels of procalcitonin, C-reactive protein, D-dimers, and ferritin compared to the other two cases, whereas platelet count was higher. We hypothesize that SARS-CoV-2 might act in children as a trigger, either inducing a classical KD phenotype or causing a systemic inflammatory response leading to a severe KD-like phenotype, eventually characterized by myocardial impairment. We think that bringing these cases and their differences to the attention of the rheumatology community during the COVID-19 pandemic will be beneficial in order to highlight the importance of early diagnosis and to increase awareness of this new phenomenon.

Cortical expression of IL1-ß, Bcl-2, Caspase-3 and 9, SEMA-3a, NT-3 and P-glycoprotein as biological markers of intrinsic severity in drug-resistant temporal lobe epilepsy.

Mesial temporal lobe epilepsy (mTLE) is the most common epilepsy induced by previous cerebral injury, and one out of three mTLE patients develops drug resistance (DR). AIM: To assess the expression of Bcl-2, Caspase-3, Caspase-9, IL1-ß, SEMA-3a, NT-3 and P-glycoprotein in the temporal cortex and their relationship with the progression of mTLE-DR clinical features in patients with mTLE-DR. METHOD: Tissue samples from 17 patients were evaluated for protein expression by Western blot and the relationships of the evaluated proteins with the clinical features of the mTLE were assessed through hierarchical cluster analysis. RESULTS: The mTLE-DR group showed significantly higher P-glycoprotein, Bcl-2 and Caspase-9 levels ***p < 0.0001, ****p < 0.0001 and ***p < 0.0002, respectively, than the autopsy control group. Four patient clusters were identified: Clusters 1 and 3 showed relationships among the age of mTLE onset, duration of mTLE-DR, average number of epileptic seizures per week, number of previous antiepileptic drugs (AEDs) and increased expression of Caspase-3, Caspase-9, Neurotrophin-3 and Semaphorin-3a. Clusters 2 and 4 showed relationships among the mTLE onset age, current age, average number of epileptic seizures per week, number of previous AEDs and increased expression of IL1-ß, Bcl-2, P-glycoprotein, Caspase-3 and NT-3. CONCLUSION: The relationships among the clinical data the age of mTLE onset, DR duration, number of previous AEDs, and average number of seizures per week and the expression of proteins involved in neuronal death, neuroinflammation and aberrant connection formation, as which are biological markers in the cerebral temporal cortex, are important factors in the progression and severity of mTLE-DR and support the intrinsic severity hypothesis.

[Argentinean consensus guidelines on the identification and clinical care of secondary progressive multiple sclerosis]. / Consenso sobre la identificación y seguimiento de la esclerosis múltiple secundaria progresiva en Argentina.

INTRODUCTION: The identification, diagnosis, follow-up, and treatment of patients with secondary progressive multiple sclerosis (SPMS) show significant differences between health care professionals in Argentina. AIM: To provide consensus recommendations on the management of patients with SPMS in Argentina to optimize patient care. DEVELOPMENT: A panel of expert neurologists from Argentina dedicated to the diagnosis and care of multiple sclerosis patients gathered during 2019 and 2020 to carry out a consensus recommendation on the diagnosis and treatment of SPMS patients in Argentina. To achieve consensus, the methodology of 'formal consensus-RAND/UCLA method' was used. Recommendations were established based on published evidence and the expert opinion. Recommendations focused on how to define SPMS and how to follow SPMS patients. CONCLUSION: The recommendations of this consensus guidelines attempt to optimize the care of SPMS patients in Argentina.

TITLE: Consenso sobre la identificación y seguimiento de la esclerosis múltiple secundaria progresiva en Argentina.Introducción. Existen diferencias significativas en el diagnóstico, la identificación y el seguimiento de pacientes con esclerosis múltiple secundaria progresiva (EMSP) entre los profesionales de la salud a cargo de su tratamiento. Objetivo. Proveer recomendaciones sobre el tratamiento de los pacientes con EMSP en Argentina con el fin de optimizar su cuidado. Desarrollo. Un grupo de neurólogos expertos en esclerosis múltiple de Argentina elaboró un consenso para el tratamiento de pacientes con EMSP en la región mediante metodología de ronda de encuestas a distancia y reuniones presenciales. Se establecieron 33 recomendaciones basadas en la evidencia publicada y en el criterio de los expertos que participaron. Las recomendaciones se enfocaron en el diagnóstico y el seguimiento de los pacientes con EMSP. Conclusión. Las recomendaciones establecidas en el presente consenso permitirían optimizar el cuidado y el seguimiento de los pacientes con EMSP en Argentina.

[Preoperative concentration of plasma fibrinogen as a predictor of bleeding in total hip arthroplasty]. / Concentración preoperatoria de fibrinógeno plasmático como predictor de sangrado en artroplastía total de cadera.

INTRODUCTION: Total hip arthroplasty is popular for its success in treating coxarthrosis, its associated with substantial blood loss. Significant bleeding causes complications such as increase in hospitalization days, higher costs, exposing the patient to complications associated with blood transfusion. The aim of the study is to investigate the association between preoperative plasma fibrinogen concentration and trans-surgical bleeding and determine fibrinogen level cut-off to present greater trans-surgical bleeding. MATERIAL AND METHODS: Retrospective, observational, analytical study, from June to December 2020, including 227 patients with Kellgren and Lawrence scale IV coxarthrosis undergoing primary total hip arthroplasty, beneficiaries, older than 18 years, without liver or hematological diseases, and history of significant surgical bleeding. RESULTS: Mean preoperative hemoglobin value was 14.6 ± 1.3 g/dl, after surgery (48 hours) 10.5 ± 1.4 g/dl; decrease of 4.1 ± 1.2 g/dl (p 0.0001). Mean preoperative hematocrit value 43% [41-45], after the procedure; 32% [29-35]; decrease of 11% [8-14] (p 0.0001). 98 patients had intraoperative bleeding 300 ml, 129 had 300 ml; 61.2% of patients with bleeding greater than 300 ml had fibrinogen values below the cut-off point (388 mg/dl). CONCLUSION: In postoperative patients, preoperative fibrinogen levels 388 mg/dl and age 58 years were associated with an increased risk of OR = 0.18 (95% CI 0.10-0.32) of presenting trans-surgical bleeding 300 ml, with a decrease in Hb of 4.1 ± 1.2 g/dl (p = 0.0001) and Hto of 11% [8-14] (p = 0.0001) between the pre and postoperative period in 48 hours.

INTRODUCCIÓN: La artroplastía total de cadera tiene popularidad debido al éxito en el tratamiento de coxartrosis; son procedimientos asociados a la pérdida de sangre. Un sangrado importante provoca complicaciones como aumento en días de estancia intrahospitalaria, mayor costo de la enfermedad, exponiendo al paciente a complicaciones asociadas a transfusión sanguínea. El objetivo del estudio es investigar asociación entre concentración de fibrinógeno plasmático preoperatorio con sagrado transquirúrgico y determinar el nivel de corte de fibrinógeno para presentar mayor sangrado transquirúrgico. MATERIAL Y MÉTODOS: Estudio retrospectivo, observacional, analítico, de Junio a Diciembre de 2020, incluyendo 227 pacientes con coxartrosis IV de la escala de Kellgren y Lawrence sometidos a artroplastía total primaria de cadera, derechohabientes, mayores de 18 años, en ausencia de enfermedades hepáticas o hematológicas y antecedente de sangrado quirúrgico importante. RESULTADOS: El valor promedio de hemoglobina preoperatoria fue 14.6 ± 1.3 g/dl, posterior a cirugía (48 horas) 10.5 ± 1.4 g/dl; observando descenso de 4.1 ± 1.2 g/dl (p 0.0001). Valor promedio del hematocrito prequirúrgico 43% [41-45], posterior al procedimiento 32% [29-35]; se observó descenso de 11% [8-14] (p 0.0001); 98 pacientes presentaron sangrado transquirúrgico 300 ml; 129 tuvieron sangrado 300 ml; 61.2% de pacientes con sangrado mayor de 300 ml mostraron valores de fibrinógeno debajo del punto de corte (388 mg/dl). CONCLUSIÓN: En pacientes postoperados los niveles preoperatorios de fibrinógeno 388 mg/dl y edad 58 años se asociaron al aumento en el riesgo del OR = 0.18 (IC 95% 0.10-0.32) de presentar sangrado transquirúrgico 300 ml, con descenso de la Hb de 4.1 ± 1.2 g/dl (p = 0.0001) y Hto de 11% [8-14] (p = 0.0001) entre el preoperatorio y el postoperatorio en 48 horas.

Management of Psoriasis During Preconception, Pregnancy, Postpartum, and Breastfeeding: A Consensus Statement. / Consenso sobre las actuaciones a seguir durante la edad fértil, el embarazo, el posparto y la lactancia en pacientes con psoriasis.

OBJECTIVE: To develop evidence- and experience-based recommendations for the management of psoriasis during preconception, pregnancy, postpartum, and breastfeeding. METHODS: The nominal group technique and the Delphi method were used. Fifteen experts (12 dermatologists, 2 of whom were appointed coordinators; 1 rheumatologist; and 2 gynecologists) were selected to form an expert panel. Following a systematic review of the literature on fertility, pregnancy, postpartum, and breastfeeding in women with psoriasis, the coordinators drew up a series of preliminary recommendations for discussion by the panel at a nominal group meeting. The experts defined the scope, sections, and intended users of the statement and prepared a final list of recommendations. Consensus was obtained using a Delphi process in which an additional 51 dermatologists rated their level of agreement with each recommendation on a scale of 1 (total disagreement) to 10 (total agreement). Consensus was defined by a score of 7 or higher assigned by at least 70% of participants. Level of evidence and strength of recommendation were reported using the Oxford Center for Evidence-Based Medicine categories. The final statement was approved by the expert panel. RESULTS: The resulting consensus statement includes 23 recommendations on preconception (fertility and contraception), pregnancy (planning, pharmacological management, and follow-up), and breastfeeding (management and follow-up). Consensus was achieved for all recommendations generated except one. CONCLUSIONS: These recommendations for the better management of psoriasis in women of childbearing age could improve outcomes and prognosis.

Survival and toxicity outcomes of hematopoietic stem cell transplantation for pediatric patients with Fanconi anemia: a unified multicentric national study from the Spanish Working Group for Bone Marrow Transplantation in Children.

Hematopoietic stem cell transplantation (HSCT) is currently the only curative option for hematological manifestations in patients with Fanconi anemia (FA). We report the outcome of 34 patients with FA inside a collaborative multicenter national study based on recommendations of Spanish Working Group for Bone Marrow Transplantation in Children (GETMON) between 2009 and 2016. Fludarabine-based conditioning regimen was carried out in all patients, with low dose total body irradiation in unrelated transplants. Disease status before HSCT was bone marrow failure (BMF) in 30 patients and myelodysplastic syndrome (MDS) in four. Donors were matched siblings donors (MSD) in 18, matched unrelated donors (MUD) in 15, and one haploidentical donor. All except one patient engrafted. Cumulative incidence of grades II-IV acute graft-versus-host disease (GVHD) was 29% and 11% for chronic GVHD. Median follow-up after HSCT was 6.5 years. Seven patients (21%) died due to transplant-related causes, two (6%) because of MDS relapse, and one (3%) after a squamous cell carcinoma. Overall survival (OS) was 73% at 5 years post-transplant, with no differences between MSD and MUD transplants. OS for patients with BMF was 80% while for MDS was 25%. Our data suggest HSCT can cure hematologic manifestations of most FA patients with BMF.

Peak expiratory flow and the single-breath counting test as markers of respiratory function in patients with myasthenia gravis. / El pico flujo espiratorio y la cuenta máxima son marcadores del compromiso respiratorio en la miastenia gravis.

INTRODUCTION: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterised by fluctuating, fatigable muscle weakness, frequently involving bulbar and respiratory muscles. Considering the severity of respiratory involvement in MG, routine evaluation of respiratory function is essential. The aim of this study was to identify a useful clinical marker of respiratory involvement in patients with MG. METHODS: We performed an observational study of patients with MG. All cases were evaluated with the single-breath counting test, peak expiratory flow (PEF), a modified Medical Research Council dyspnoea scale (mMRC), and a neck strength assessment. The results of these parameters were correlated with forced vital capacity (FVC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). RESULTS: The study included 45 patients with MG: 2 patients classified as grade I on the Myasthenia Gravis Foundation of America classification at the time of evaluation, 35 classified as grade II, 7 classified as grade III, and one classified as grade IV. Positive correlations were found between single-breath counting test scores and FVC values (r = 0.57, p = .000), and between PEF and FVC values (r = 0.76, p = .000). Severity of dyspnoea according to the mMRC scale showed a negative correlation with FVC values (r = -0.31, p = .03). PEF also showed a significant correlation with MEP (r = 0.51, p = .002). CONCLUSIONS: PEF, the single-breath counting test, and the mMRC scale are useful measures for evaluating respiratory function in patients with MG.

Global maps of travel time to healthcare facilities.

Access to healthcare is a requirement for human well-being that is constrained, in part, by the allocation of healthcare resources relative to the geographically dispersed human population1-3. Quantifying access to care globally is challenging due to the absence of a comprehensive database of healthcare facilities. We harness major data collection efforts underway by OpenStreetMap, Google Maps and academic researchers to compile the most complete collection of facility locations to date. Leveraging the geographically variable strengths of our facility datasets, we use an established methodology4 to characterize travel time to healthcare facilities in unprecedented detail. We produce maps of travel time with and without access to motorized transport, thus characterizing travel time to healthcare for populations distributed across the wealth spectrum. We find that just 8.9% of the global population (646 million people) cannot reach healthcare within one hour if they have access to motorized transport, and that 43.3% (3.16 billion people) cannot reach a healthcare facility by foot within one hour. Our maps highlight an additional vulnerability faced by poorer individuals in remote areas and can help to estimate whether individuals will seek healthcare when it is needed, as well as providing an evidence base for efficiently distributing limited healthcare and transportation resources to underserved populations both now and in the future.