[Faecal microbiota study reveals specific dysbiosis in spondyloarthritis according to subtype, disease activity and treatment]. / Estudio de microbiota fecal revela disbiosis específica en espondiloartritis, según subtipo, actividad de la enfermedad y tratamiento.

OBJECTIVE: To compare the diversity and composition of the gastrointestinal microbiome of patients with SpA. METHODS: MiSeq sequencing of the V3-V4 region of the 16S ribosomal RNA gene was performed on DNA isolated from stool. Patients with concurrent SpA and IBD were excluded. Differences were assessed for richness and diversity indices by QIIME 2™. Differences between means >0,2% with a p-value<0,05 were assumed significant. Institutional Ethics Committee endorsement. RESULTS: 69 individuals included, 49 with SpA (ankylosing spondylitis-AS 72,9%, psoriatic arthritis-PsA 18,8%, reactive arthritis-ReA 8,3%) 5 positive controls-dysbiosis and 15 controls-eubiosis. Conventional treatment in 42,9%, anti-IL-17 16,3% and anti-TNF 40,8%. By subtype, statistically significant differences in favour of AS were found for the diversity indices. AS vs PsA there was a difference in favour of AS for Clostridium clostridioforme (p=0,002), Gemmiger formicilis (p=0,009), Roseburia inulivorans (p=0,008) and Lachnospira pectinoschiza. AS vs ReA there was a difference in favour of AS for L. pectinoschiza (p=0,009), Ruminococcus callidus (p=0.006), Clostridium ruminantium (p=0.031); G. formicilis (p=0,034). Diversity and richness showed differences in patients with high activity for Simpson's and Pielou's indices. In high activity, lower enrichment of Bacteroides eggerthii (p= 0,0003), C. ruminantium (p= 0,026) and Alistipes putredinis (p=0,035) was found. The number of ASV was higher in the anti-IL-17 vs conventional group (p=0.025) and a trend between anti-IL-17 vs anti-TNF (p=0.09). In anti-TNF there was a lower proportion for C. clostridioforme (p=0.023), G. formicilis (p=0.030) and R. callidus (p= 0.003). In anti IL-17, Alistipes indistinctus (p= 0.012) was decreased. CONCLUSIONS: There are differences in microbial diversity for SpA subtypes. The level of disease activity is plausible to influence the composition of the faecal microbiota. Anti-TNFα treatment may influence the microbiome environment favouring restoration of the gut microbiota, while anti-IL-17 may maintain an inflammatory environment.

OBJETIVO: Comparar la diversidad y composición del microbioma gastrointestinal de pacientes con EspA. MÉTODOS: La secuenciación MiSeq de la región V3-V4 del gen ARN ribosomal 16, se realizó en ADN aislado de heces. Se excluyeron pacientes con EspA y EII simultánea. Se evaluaron diferencias para los índices de riqueza y diversidad por medio de QIIME 2™. Las diferencias entre medias> 0,2%, con un valor de p< 0,05, se asumieron significativas. Aval del Comité de Ética Institucional. RESULTADOS: 69 individuos incluidos, 49 con EspA (espondilitis anquilosante-EA 72,9%, artritis psoriásica-APs 18,8%, artritis reactiva-ARe 8,3%), cinco controles positivos-disbiosis y 15 controles-eubiosis. El tratamiento convencional en 42,9%, anti-IL-17 16,3%, y anti-TNF 40,8%. Por subtipo-EasP, se encontraron diferencias estadísticamente significativas a favor de EA para los índices de diversidad. Entre EA vs APs, hubo diferencia a favor de EA para Clostridium clostridioforme (p=0,002), Gemmiger formicilis (p=0,009), Roseburia inulivorans (p=0,008) y Lachnospira pectinoschiza. Entre EA vs ARe hubo diferencia a favor de EA para L. pectinoschiza (p=0,009), Ruminococcus callidus (p = 0,006), Clostridium ruminantium (p=0,031); G. formicilis (p=0,034). La diversidad y riqueza mostraron diferencias en pacientes con alta actividad para los índices de Simpson y Pielou. En alta actividad, se encontró menor enriquecimiento de Bacteroides eggerthii (p=0,0003), C. ruminantium (p= 0,026) y Alistipes putredinis (p= 0,035). El número de ASV fue superior en el grupo de anti IL-17 vs convencional (p=0.025), y una tendencia entre anti IL-17 vs anti-TNF (p=0,09). En anti TNF hubo menor proporción para C. clostridioforme (p=0,023), G. formicilis (p=0,030) y R. callidus (p= 0,003). Y en anti IL-17, Alistipes indistinctus (p= 0,012), estuvo disminuida. CONCLUSIONES: Existen diferencias en la diversidad microbiana para los subtipos de EspA. El nivel de actividad de la enfermedad es plausible para influir en la composición de microbiota fecal. El tratamiento con anti-TNFα, puede influenciar el ambiente del microbioma favoreciendo la restauración de la microbiota intestinal, mientras los anti IL-17 podrían mantener un ambiente inflamatorio.

Home Noninvasive Ventilation in Myasthenia Gravis.

BACKGROUND: Noninvasive ventilation (NIV) plays an important role in avoiding endotracheal intubation during myasthenic crisis, yet there are few published data concerning long-term home NIV in stable out-patients with myasthenia gravis (MG). The aim of this study was to describe the prevalence of NIV in a cohort of subjects with stable MG and to analyze contributing factors that could predict the need of NIV. METHODS: We performed a cross-sectional study that included subjects diagnosed with MG managed in the respiratory care unit over the previous year. Subjects underwent clinical analysis including demographic, clinical, and functional respiratory data. RESULTS: Of the 50 subjects included, 35 (70%) were positive for nicotinic acetylcholine receptor antibodies, and 68% had a diagnosis of generalized MG. Bulbar symptoms developed in 16 (32%), and 10 (20%) subjects needed long-term home NIV. The only variable predicting the need for long-term NIV was MG severity measured with Myasthenia Gravis Foundation of America (MGFA), mainly grades IIB (odds ratio 0.14 [95% CI 0.02-0.85], P = .03) and IIIB (odds ratio 0.02 [95% CI 0.01-0.34], P = .01). CONCLUSIONS: Home NIV was needed in a substantial percentage of medically stable subjects with MG, mainly in those with generalized type and with oropharyngeal and/or respiratory muscle involvement (MGFA grades IIB and IIIB).

Máculas hiperpigmentadas y prolapso rectal, ¿qué debemos sospechar? / Hyperpigmented macules and rectal prolapse, what should we suspect?

El síndrome de Peutz-Jeghers (SPJ) es un síndrome autosómico dominante con una incidencia de 1 de cada 200 000 nacidos vivos. Las manifestaciones clínicas más frecuentes son las máculas hiperpigmentadas típicamente localizadas en la mucosa oral y la presencia de pólipos en el tracto gastrointestinal. A diferencia de la edad adulta, en Pediatría es excepcional el desarrollo de patología tumoral maligna. Sin embargo, en la edad pediátrica hay que tener un elevado índice de sospecha ante un paciente con diagnóstico de SPJ que presenta dolor abdominal compatible con una invaginación intestinal, ya que esta complicación es relativamente frecuente y precisa tratamiento quirúrgico urgente. Una vez realizado el diagnóstico de esta enfermedad, se deberán llevar a cabo controles periódicos mediante endoscopias a partir de los ocho años de edad (AU)

Peutz-Jeghers syndrome (PJS) is an autosomal dominant syndrome with an incidence of 1 in 200,000 live births. The most frequent clinical manifestations are hyperpigmented macules typically located on the oral mucosa and the presence of polyposis in the gastrointestinal tract.Unlike adulthood, in pediatrics the development of malignant tumor pathology is exceptional. However, in the pediatric age group, a high index of suspicion must be maintained when faced with a patient diagnosed with PJS who presents with abdominal pain compatible with intussusception, since this complication is relatively frequent and requires urgent surgical treatment. Once the diagnosis of this disease has been made, periodic controls should be carried out by means of endoscopies starting at eight years of age. (AU)

How to increase noninvasive ventilation effectiveness in bulbar amyotrophic lateral sclerosis patients.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease in which the respiratory muscles are also affected, leading to respiratory failure. Bulbar impairment develops in almost all cases during the course of the disease, becoming severe in the late stages of disease. Noninvasive ventilation (NIV) has been shown to increase survival in ALS; however, severe bulbar dysfunction has a negative impact on NIV tolerance and effectiveness. Therefore, certain steps should be taken to improve NIV outcomes in these patients including optimal ventilatory parameters, adequate interface selection, effective respiratory secretion management and control of bulbar symptoms.

Panadizo herpético en lactante de 20 meses / Herpetic whitlow in a 20-month-old infant

El panadizo herpético es una lesión cutánea producida por el virus herpes simple, en pediatría especialmente el virus herpes tipo 1. Es una lesión que se debe conocer para evitar realizar un diagnóstico incorrecto e instaurar tratamientos innecesarios o incluso perjudiciales. La presentación clínica habitual es una lesión con vesículas arracimadas, cuya localización típica es en primer o segundo dedo. En los pacientes pediátricos es frecuente que se produzca tras autoinoculación desde una gingivoestomatitis herpética, por lo que ante la sospecha de esta lesión es importante la exploración física completa incluyendo orofaringe. El curso es autorresolutivo aunque en ocasiones puede ser necesario instaurar tratamiento con aciclovir oral. El drenaje de la lesión está contraindicado porque puede llegar a producir una diseminación del virus herpes con graves consecuencia (AU)

Herpetic whitlow is a skin lesion caused by the herpes simplex virus (HSV), usually type 1 in the paediatric population. Providers must be aware of this lesion to avoid making an incorrect diagnosis and initiating unnecessary or even harmful treatments.The usual presentation is a lesion with clustered vesicles, typically located on the first or second toe. In paediatric patients, it is often the result of autoinoculation from HSV gingivostomatitis lesions, so when this lesion is suspected, it is important to carry out a complete physical examination including the oropharynx.The course is self-limiting, although on occasion treatment with oral acyclovir becomes necessary. Drainage of the lesion is contraindicated because it could lead to dissemination of HSV with severe consequences. (AU)

Waveforms Analysis in Patients With Amyotrophic Lateral Sclerosis for Enhanced Efficacy of Mechanically Assisted Coughing.

BACKGROUND: Effectiveness of mechanical assisted coughing with insufflation-exsufflation (MI-E) in amyotrophic lateral sclerosis (ALS) depends largely on severity of bulbar dysfunction, which can generate different upper-airway responses. The aim of the study was to evaluate the use of graphs generated by MI-E in ALS to detect airway obstruction and set parameters to achieve an effective mechanically assisted coughing. METHODS: This was a prospective study enrolling patients with ALS. Several sessions with MI-E were applied, administering different insufflation-exsufflation (± 20, ± 30, ± 40, ± 50 cm H2O) levels in each session. The graphs produced were recorded and analyzed, and the results were used to select the parameters resulting in more effective MI-E. RESULTS: Sixty-nine subjects with ALS were included, yielding a total of 351 analyzed records. A pattern of obstruction during insufflation was detected in 34 subjects (50.7%) and of upper-airway collapse during exsufflation in 18 subjects (26%). The variable associated with obstruction during insufflation was bulbar upper motor neuron dysfunction (odds ratio 7.19 [95% CI 2.32-22.29], P = .001), whereas bulbar lower motor neuron dysfunction was related to upper-airway collapse during exsufflation (odds ratio 0.32 [95% CI 0.11-0.98], P = .046). After parameters were adjusted, in 68 subjects (98.55%) an effective MI-E was achieved. The only variable that predicted absence of alterations in the graphs was Norris bulbar score (odds ratio 0.87 [95% CI 0.78-0.96], P = .007). CONCLUSIONS: Analysis of graphics generated by applying MI-E in ALS was an effective method to detect upper-airway responses and select optimal set parameters. Obstruction during insufflation is related to bulbar upper motor neuron dysfunction and collapse during exsufflation to bulbar lower motor neuron dysfunction.

Rosai-dorfman disease: a rare presentation of extranodal involvement of isolated bone. case report

ABSTRACT Introduction: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis, is a rare disorder characterized by histiocyte proliferation. Case presentation: A 33-year-old man consulted the emergency department of a tertiary care institution in Cartagena de Indias, Colombia, due to a 6-month history of progressive deformity in the frontal right side of the face, associated with pain of slow progression, without any other symptoms or dermatological involvement. There were no other major findings on physical examination and laboratory tests performed were negative. Imaging scans obtained showed extensive inflammatory involvement of the frontal bone, which led to suspect osteomyelitis as the first diagnostic possibility. A biopsy of the lesion was performed with negative cultures for bacteria, which allowed establishing a diagnosis of extranodal Rosai-Dorfman disease with isolated bone involvement. Treatment with systemic corticosteroids was indicated with poor response, so methotrexate was added, achieving an evident improvement after 2 months. Conclusions: Little is known about the manifestations of Rosai-Dorfman disease and its treatment in the adult population. The present case report contributes to expanding the literature on this topic, which can present with rare symptoms that may pose challenges for its diagnosis.

RESUMEN Introducción. La enfermedad de Rosai-Dorfman, también conocida como histiocitosis sinusal, es un trastorno poco frecuente caracterizado por la proliferación de histiocitos. Presentación del caso. Hombre de 33 años quien consultó al servicio de urgencias de una institución de tercer nivel de Cartagena de Indias, Colombia, por una deformidad de progresión lenta que apareció 6 meses atrás en el costado frontal derecho de la cara y generaba dolor. Al examen físico no hubo hallazgos mayores y los estudios paraclínicos fueron normales o negativos. Se obtuvieron imágenes que evidenciaron extenso compromiso inflamatorio/infeccioso del hueso frontal, por lo que se sospechó de osteomielitis y se realizó biopsia de la lesión que permitió establecer diagnóstico de enfermedad de Rosai-Dorfman variante extranodal con compromiso óseo aislado. El paciente recibió tratamiento con corticoide sistémico sin obtener respuesta, por lo que se adicionó metotrexato, con lo cual se logró mejoría a los 2 meses. Conclusiones. Las manifestaciones de la enfermedad de Rosai-Dorfman en población adulta son poco conocidas, además no hay mucha información sobre su tratamiento, por lo que el presente caso contribuye a ampliar la literatura sobre esta enfermedad que puede presentarse con sintomatología completamente inusual.

ROX index as predictor of high flow nasal cannula therapy success in acute respiratory failure due to SARS-CoV-2.

BACKGROUND: High-Flow Nasal Cannula (HFNC) therapy is useful treatment in patients with acute respiratory failure (ARF). The ROX index (ratio of pulse oximetry/fraction of inspired oxygen to respiratory rate) has been evaluated to predict success of HFNC in patients with pneumonia. OBJECTIVE: The aim of this study was to determine whether the ROX Index could predict HFNC therapy success in patients with ARF due to SARS-CoV-2 pneumonia. METHODS: An observational, prospective study was performed including patients admitted with ARF secondary to SARS-CoV-2 pneumonia who met criteria for HFNC therapy initiation. Demographic, radiological, laboratory and clinical course data were collected. The ROX index was calculated at 1 h, 6 h, 12 h and 24 h after starting HFNC. RESULTS: In total 85 patients were included (age, 64.51 + 11.78 years; male, 69.4%). HFNC failed in 47 (55.3%) patients, of whom 45 (97.8%) were initially managed with noninvasive ventilation (NIV). ROX index at 24 h was the best predictor of HFNC success (AUC 0.826, 95%CI 0.593-1.00, p = 0.015) with a cut-off point of 5.35 (S 0.91, Sp 0.79, PPV 0.92, NPP 0.79). In multivariate logistic regression analysis ROX index at 24 h proved the best predictor of HFNC success. CONCLUSIONS: ROX index at 24 h with a cut-off point of 5.35 predicts HFNC success in patients with SARS-Cov-2-induced ARF.

Effect of one-year dextromethorphan/quinidine treatment on management of respiratory impairment in amyotrophic lateral sclerosis.

Treatment with Dextromethorphan/Quinidine (DM/Q) has demonstrated benefit on pseudobulbar affect and bulbar function in amyotrophic lateral sclerosis (ALS). The aim of this study was to assess whether DM/Q could provide long-term improvement in bulbar function and thereby prolong noninvasive respiratory management in ALS. MATERIALS AND METHODS: This prospective, case-cohort study, recruited ALS patients with bulbar dysfunction. Subjects included were compared with cross-matched historical controls. Cases received DM/Q (20/10 mg twice daily) during one-year follow-up; bulbar dysfunction was evaluated with the Norris scale bulbar subscore (NBS) and bulbar subscale of AlSFRS-R (ALSFRSb). RESULTS: In total, 21 cases and 20 controls were enrolled, of whom noninvasive respiratory muscle assistance failed in 6 (28.5%) patients in the DM/Q group, compared with 4 patients (20.0%) in the control group (p = 0.645). Time from study onset to failure of respiratory muscle aids was 5.50 + 1.31 months in the DM/Q group and 5.20 + 1.15 months in the control group (p = 0.663). The adjusted OR for the effect of treatment on failure of noninvasive respiratory muscle aids was 2.12 (95%CI 0.23-33.79, p = 0.592). In the DM/Q group an impairment in scores was found in NBS (F = 19.26, p = 0.000) and ALSFRS-Rb (F = 12.71, p = 0.001) across different months of the study. CONCLUSION: Treatment with DM/Q in ALS is unable to prolong noninvasive respiratory management, and moreover, has no effect on long-term deterioration of bulbar function. Notwithstanding the results on bulbar function, DM/Q was found to improve pseudobulbar affect during one-year follow-up.

Moderate-to-Vigorous Physical Activity and Body Composition in Children from the Spanish Region of Aragon.

Most of the studies analyzing the effect of moderate to vigorous physical activity (MVPA) on children's health do not contain information on early stages or do not use accurate methods. We investigated the association between PA and body composition using objective methods, perinatal data, lifestyle behaviors, and World Health Organization (WHO) physical activity (PA) recommendations. The CALINA study is a longitudinal observational cohort study of children born in Aragon (Spain) in 2009. A total of 308 7-year-old children (52.3% boys) were assessed. We used dual-energy X-ray absorptiometry (DXA) and accelerometry. Rapid weight gain until 12 months and lifestyle behaviors were considered as covariates both in the ANCOVA and linear regression models. A higher percentage of boys met the WHO PA recommendations compared to girls (69.6% vs. 40.9%, respectively; p < 0.001). There was a negative association between MVPA and subtotal fat and abdominal fat in both girls and boys. After adjusting for perinatal and lifestyle variables, we found that subtotal body fat, abdominal fat, and fat mass index (FMI) were significantly lower in those classified as active. MVPA was associated with body fat both in boys and girls. More research is needed to identify the cutoffs points of MVPA that generate benefit to boys and girls in all body composition components.

Tracheostomy in patients with COVID-19: predictors and clinical features.

BACKGROUND: Around 20% of patients hospitalized for COVID-19 need mechanical ventilation (MV). MV may be prolonged, thus warranting tracheostomy. METHODS: Observational cohort study enrolling patients admitted due to COVID-19. Demographic and clinical data at hospital and ICU admission were collected. The primary endpoint was to identify parameters associated with a need for tracheostomy; secondary endpoints were to analyze the clinical course of patients who needed tracheostomy. RESULTS: 118 patients were enrolled; 37 patients (31.5%) were transferred to ICU, of which 11 (29.72%) needed a tracheostomy due to prolonged MV. Sequential Organ Failure Assessment (SOFA) score at ICU admission (OR 0.65, 95% CI 0.47-0.92, p 0.015) was the only variable found to be associated with increased risk of the need for tracheostomy, with a cut-off point of 4.5 (sensitivity 0.72, specificity 0.73, positive predictive value 0.57 and negative predictive value 0.85). The main complications were nosocomial infection (100%), supraventricular cardiac arrhythmia (45.5%), agitation (54.5%), pulmonary thromboembolism (9.1%) and depression (9.1%). All patients presented with hypoalbuminemia and significant critical illness polyneuropathy. CONCLUSION: SOFA at ICU admission is associated with an increased risk of tracheostomy in patients with COVID-19. Moreover, they present clinical features similar to those with chronic critical illness and suffer SARS-CoV-2-related complications.

Mechanical Insufflation-Exsufflation With Oscillations in Amyotrophic Lateral Sclerosis With Home Ventilation via Tracheostomy.

BACKGROUND: Mechanical insufflation-exsufflation (MI-E) applied via tracheostomy tubes in patients with amyotrophic lateral sclerosis (ALS) who are on home mechanical ventilation via tracheostomy is an effective procedure for respiratory secretion management. Nonetheless, tenacious secretions may remain and increase the risk of respiratory infections. The aim of this study was to determine whether adding oscillations to MI-E could reduce the rate of respiratory infections and the need for bronchoscopy to remove secretions in patients with ALS on home mechanical ventilation via tracheostomy. METHODS: This was a 2-y, prospective, crossover study. Subjects were treated with conventional MI-E and MI-E with oscillations for 2 alternate 6-month periods. Data were collected on episodes of respiratory infections, hospital admission, and number of bronchoscopy procedures. RESULTS: In the 19 ALS subjects enrolled, the median (interquartile range [IQR]) number of acute respiratory infections per subject was 1.0 (0.5-2.0) in the MI-E period and 0.0 (0.0-2.0) in the MI-E plus oscillations period (P = .92). The median (IQR) number of hospital stays was 0.0 (0.0-1.0) in the MI-E period and 0.0 (0.0-1.0) in the MI-E plus oscillations period (P = .80). The median (IQR) number of bronchoscopies per subject was 0.0 (0.0-1.0) in MI-E period and 0.0 (0.0-0.5) in the MI-E plus oscillations period (P = .26). MI-E plus oscillations treatment had no impact on the risk of respiratory infections (odds ratio 3.71, 95% CI 0.81-16.84, P = .09) or the need for bronchoscopy (odds ratio 2.70, 95% CI 0.44-16.68, P = .29). CONCLUSIONS: Adding oscillations to MI-E therapy in subjects with ALS on home mechanical ventilation via tracheostomy did not decrease the risk of respiratory infections, hospital admission, or need for bronchoscopy.

Usefulness of Oscillations Added to Mechanical In-Exsufflation in Amyotrophic Lateral Sclerosis.

BACKGROUND: Assisted coughing via mechanical in-exsufflation (MI-E) is a first-line treatment for secretion management in patients with amyotrophic lateral sclerosis (ALS) with unassisted CPF < 4.25 L/s. Some devices enable oscillations to be added to MI-E (MI-E+O). We sought to determine whether adding oscillations to MI-E enables a reduction in the use of invasive secretion management procedures (ie, bronchoscopy or tracheostomy) in subjects with ALS. METHODS: We conducted a 12-month, prospective, randomized follow-up study of subjects with ALS for whom assisted coughing techniques were indicated. One group was treated with oscillations in addition to MI-E (MI-E+O), and the other group was treated with conventional MI-E. RESULTS: 29 subjects were included in the MI-E group and 27 subjects were included in the MI-E+O group. Five subjects (8.9%) required invasive techniques for secretion management (3 in the MI-E group and 2 in the MI-E+O group, P = .70). Treatment with MI-E+O did not alter the risk of invasive procedures (odds ratio 0.69, 95% CI 0.10-4.50, P = .70). The mean number of respiratory infections was 0.58 ± 0.16 in the MI-E group and 0.025 ± 0.08 in the MI-E+O group (P = .10). Survival was 8.96 ± 0.18 months in the MI-E group and 7.70 ± 0.70 months in the MI-E+O group (P = .10). CONCLUSION: Adding oscillations to MI-E did not enable a reduction in the need to perform invasive procedures for secretion management in subjects with ALS.

Unstable control of breathing can lead to ineffective noninvasive ventilation in amyotrophic lateral sclerosis.

Upper airway obstruction with decreased central drive (ODCD) is one of the causes of ineffective noninvasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS). The aim of this study is to determine the mechanism responsible for ODCD in ALS patients using NIV. This is a prospective study that included ALS patients with home NIV. Severity of bulbar dysfunction was assessed with the Norris scale bulbar subscore; data on upper or lower bulbar motor neuron predominant dysfunction on physical examination were collected. Polysomnography was performed on every patient while using NIV and the ODCD index (ODCDI: number of ODCD events/total sleep time) was calculated. To determine the possible central origin of ODCD, controller gain was measured by inducing a hypocapnic hyperventilation apnoea. Sonography of the upper airway during NIV was performed to determine the location of the ODCD. 30 patients were enrolled; three (10%) had ODCDI >5 h-1. The vast majority of ODCD events were produced during non-rapid eye movement sleep stages and were a consequence of an adduction of the vocal folds. Patients with ODCDI >5 h-1 had upper motor neuron predominant dysfunction at the bulbar level, and had greater controller gain (1.97±0.33 versus 0.91±0.36 L·min-1·mmHg-1; p<0.001) and lower carbon dioxide (CO2) reserve (4.00±0.00 versus 10.37±5.13 mmHg; p=0.043). ODCDI was correlated with the severity of bulbar dysfunction (r= -0.37; p=0.044), controller gain (r=0.59; p=0.001) and CO2 reserve (r= -0.35; p=0.037). ODCD events in ALS patients using NIV have a central origin, and are associated with instability in the control of breathing and an upper motor neuron predominant dysfunction at the bulbar level.

Traqueotomía en enfermos con ELA: pro / Tracheotomy in Amyotrophic Lateral Sclerosis: The Argument in Favor

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Dolor de brazo intenso y fiebre como presentación inusual de neumonía. A propósito de un caso / Severe arm pain and fever as unusual presentation of pneumonia. A case report

Introducción: Se han descrito previamente casos de niños con neumonía y sintomatología extratorácica, como dolor abdominal, náuseas, vómitos o cefalea. También se debe incluir la neumonía dentro del diagnóstico diferencial de un paciente con fiebre y dolor de la extremidad superior. Caso clínico: Se presenta el caso de un paciente con neumonía del lóbulo superior izquierdo, y dolor de brazo y fiebre como única sintomatología. Conclusiones: El diagnóstico de neumonía supone un reto en la infancia, sobre todo en los pacientes con poca sintomatología, de intensidad leve o de localización extratorácica. De ahí la importancia de conocer los síntomas extratorácicos que pueden asociar las neumonías en la edad pediátrica. En el paciente del presente caso, con neumonía del lóbulo superior izquierdo, la naturaleza y la distribución del dolor se explican por la implicación de las raíces del plexo braquial inferior, por irritación de las mismas o por inflamación de la pleura apical

Introduction: Cases of children with pneumonia and extrathoracic symptoms such as abdominal pain, sickness, vomits or headache have been described previously. Pneumonia should be also included in the differential diagnosis of a patient with fever and upper extremity pain. Clinical case: We present a case of a patient with left upper lobe pneumonia and arm pain and fever as the single symptomatology. Conclusion: The diagnosis of pneumonia is a challenge in childhood, especially in those patients with little symptomatology, mild intensity or extrathoracic location. Knowing the extrathoracic symptoms that can associate pneumonias in pediatric age is important. In the case of our patient, with left upper lobe pneumonia, nature and distribution of pain is explained by the involvement of the roots of the lower brachial plexus by irritation or inflammation of the apical pleura

Úlceras genitales de Lipschütz: reporte de un caso pediátrico / Lipschütz ulcers: a pediatric case report

Las úlceras genitales son una patología poco frecuente en las consultas de pediatría, pero que generan gran ansiedad familiar. Las úlceras de Lipschütz son úlceras vulvares agudas, dolorosas y autolimitadas de causa desconocida. Aparecen en mujeres jóvenes que no han tenido relaciones sexuales previas o tras un período de abstinencia. Paciente de 10 años con úlceras vulvares dolorosas que, tres días antes de la aparición de las lesiones, había presentado un cuadro de deposiciones diarreicas y fiebre. Se realizó una anamnesis y exploración física detallada y se llevaron a cabo diferentes pruebas complementarias para descartar enfermedades infecciosas de origen venéreo y no venéreo, todas ellas negativas. Se indicó tratamiento sintomático, y se observó, al mes, una completa resolución del cuadro, por lo que se confirmó el diagnóstico de úlceras de Lipschütz.

Genital ulcers are a rare entity in pediatric consultation, but they generate great family anxiety. Lipschütz ulcers are acute, painful, and self-limiting vulvar ulcers of unknown cause. These ulcers appear in young women who have not had previous sexual intercourse or after a period of sexual abstinence. We present a 10-year-old girl with painful vulvar ulcers with a 3-day history of diarrhea and fever before the onset of the lesions. A complete anamnesis and detailed physical examination were carried out and complementary tests were performed to investigate sexually and non-sexually transmitted disease; the results were negative. Symptomatic treatment was started; at one-month follow-up a complete resolution of ulcers confirmed the diagnosis of ulcer of Lipschütz.

[Lipschütz ulcers: a pediatric case report]. / Úlceras genitales de Lipschütz: reporte de un caso pediátrico.

Genital ulcers are a rare entity in pediatric consultation, but they generate great family anxiety. Lipschütz ulcers are acute, painful, and self-limiting vulvar ulcers of unknown cause. These ulcers appear in young women who have not had previous sexual intercourse or after a period of sexual abstinence. We present a 10-year-old girl with painful vulvar ulcers with a 3-day history of diarrhea and fever before the onset of the lesions. A complete anamnesis and detailed physical examination were carried out and complementary tests were performed to investigate sexually and non-sexually transmitted disease; the results were negative. Symptomatic treatment was started; at one-month follow-up a complete resolution of ulcers confirmed the diagnosis of ulcer of Lipschütz.

Las úlceras genitales son una patología poco frecuente en las consultas de pediatría, pero que generan gran ansiedad familiar. Las úlceras de Lipschütz son úlceras vulvares agudas, dolorosas y autolimitadas de causa desconocida. Aparecen en mujeres jóvenes que no han tenido relaciones sexuales previas o tras un período de abstinencia. Paciente de 10 años con úlceras vulvares dolorosas que, tres días antes de la aparición de las lesiones, había presentado un cuadro de deposiciones diarreicas y fiebre. Se realizó una anamnesis y exploración física detallada y se llevaron a cabo diferentes pruebas complementarias para descartar enfermedades infecciosas de origen venéreo y no venéreo, todas ellas negativas. Se indicó tratamiento sintomático, y se observó, al mes, una completa resolución del cuadro, por lo que se confirmó el diagnóstico de úlceras de Lipschütz.

Piomiositis tropical como complicación de un tatuaje / Tropical pyomyositis as a complication of a tattoo

La piomiositis tropical es una infección bacteriana, del músculo esquelético, endémica de áreas tropicales. Se caracteriza por la formación de un absceso piógeno que, del 75 al 90 por ciento de los casos, contiene Staphylococcus aureus como agente etiológico. Se describe el caso de una paciente de 19 años de edad, con piomiositis del músculo cuadrado lumbar izquierdo, cuya puerta de entrada fue un tatuaje realizado en el dorso de la mano homolateral. El diagnóstico fue corroborado por ultrasonido y el cultivo de la secreción purulenta. Se le realizó punción y aspiración de la colección, en dos ocasiones, además de tratamiento antibiótico específico; se logró la resolución total del cuadro. Se concluye que se debe sospechar la presencia de la piomiositis tropical, como complicación de los tatuajes, en aquellos pacientes que tras su realización, se presenten con un cuadro febril prolongado, sobre todo en los practicados por personal no profesional(AU)

Tropical pyomyositis is a bacterial infection of the skeletal muscle endemic in tropical areas. It is characterized by the formation of a pyogenic abscess whose etiological agent contains Staphylococcus aureus in 75-90 percent of cases. A case is presented of a 19-year-old female patient with pyomyositis of the left quadratus lumborum muscle, whose entrance door was a tattoo performed on the back of her homolateral hand. The diagnosis was confirmed by ultrasonography and culture of the purulent secretion. Puncture and collection aspiration were performed on two occasions, alongside specific antibiotic treatment. Total resolution was achieved. It is concluded that the presence of tropical pyomyositis as a complication of tattoos should be suspected in patients with prolonged fever after being performed a tattoo, especially if it was not performed by professional personnel(AU)