Controles de salud durante la pandemia e impacto clínico del COVID-19 en niños con enfermedad neuromuscular / Health checkups during the pandemic and clinical impact of COVID-19 in children with neuromuscular disease

Introducción. Los niños con enfermedad neuromuscular (ENM) requieren cuidados crónicos de salud (CCS) y podrían presentar COVID-19 grave. Objetivos. Describir CCS para niños con ENM durante la pandemia y evolución del COVID-19 en este grupo. Población y métodos. Cohorte prospectiva unicéntrica. Se incluyeron pacientes de 2-18 años, con ≥ 1 año de seguimiento previo a la pandemia. Se recolectaron variables demográficas, relativas a los CCS y al COVID-19 mediante historias clínicas y encuestas telefónicas. Resultados. Se incluyeron 226 pacientes; el 71 % varones, mediana de edad 11,3 años. Presentaban distrofias musculares (55,7 %) y atrofia muscular espinal (23 %). Comparando el primer año de pandemia con el previo, el 30 % no realizó controles médicos y el 25 % no realizó kinesioterapia. Otros disminuyeron la frecuencia. Hubo 52 casos de COVID-19. Fueron sintomáticos el 82 %: el 88,4 % leves/moderados y el 11,6 % graves. No hubo fallecidos. Conclusiones. La pandemia impactó negativamente en los CCS y los casos de COVID-19 fueron mayormente leves.

Introduction. Children with neuromuscular disease (NMD) require chronic health care (CHC) and may develop severe COVID-19. Objectives. To describe CHC for children with NMD during the pandemic and the course of COVID-19 in this group. Population and methods. Prospective, single-center cohort. Patients aged 2 to 18 years with ≥ 1 year of follow-up prior to the pandemic were included. Demographic variables in relation to CHC and COVID-19 were collected from medical records and via telephone surveys. Results. A total of 226 patients with a median age of 11.3 years were included; 71% were males. They had muscular dystrophy (55.7%) and spinal muscular atrophy (23%). When comparing the first year of the pandemic with the previous year, 30% did not have a health checkup and 25% did not receive kinesiotherapy. Others did, but with a lower frequency. A total of 52 COVID-19 cases were reported; 82% were symptomatic: 88.4% were mild/moderate and 11.6%, severe. No patient died. Conclusions. The pandemic had a negative impact on CHC, and COVID-19 cases were mostly mild.

Disfunciones del metabolismo del surfactante pulmonar: caso clínico pediátrico / Pulmonary surfactant metabolism dysfunction: A pediatric clinical case report

Las enfermedades pulmonares intersticiales son patologías poco frecuentes en pediatría. Dentro de ellas, se incluyen las disfunciones del metabolismo del surfactante pulmonar, molécula anfipática cuya función es disminuir la tensión superficial y evitar el colapso alveolar. Se presenta el caso de un lactante de 6 meses, en seguimiento por bajo peso, que presentó dificultad respiratoria aguda y cianosis; la radiografía de tórax evidenció infiltrado intersticial, neumomediastino y neumotórax bilateral. Al interrogatorio, surgió antecedente materno de internación al año de vida, con requerimiento de oxigenoterapia prolongada y diagnóstico desconocido; presenta signos de hipoxia crónica. El paciente cursó internación con requerimiento de oxigenoterapia. Se realizaron estudios complementarios en búsqueda de etiología, sin resultados positivos. La tomografía de tórax evidenció opacidades en vidrio esmerilado, engrosamiento del intersticio septal y áreas de atrapamiento aéreo; con resultado de biopsia pulmonar y estudio genético se llegó al diagnóstico de disfunción del metabolismo del surfactante pulmonar.

Interstitial lung diseases are rare in pediatrics. They include dysfunctions in the metabolism of pulmonary surfactant, an amphipathic molecule that reduces surface tension and prevents alveolar collapse. Here we describe the case of a 6-month-old infant controlled for low weight, who presented with acute respiratory distress and cyanosis; his chest X-ray showed interstitial infiltrate, pneumomediastinum, and bilateral pneumothorax. During history-taking, it was noted that his mother had a history of hospitalization at 1 year old with unknown diagnosis, requiring prolonged oxygen therapy; she now shows signs of chronic hypoxia. The patient was hospitalized and required oxygen therapy. Ancillary tests were done to look for the etiology of the condition, with no positive results. A chest computed tomography showed groundglass opacities, thickening of the septal interstitium, and areas of air trapping; based on the results of a lung biopsy and a genetic study, pulmonary surfactant metabolism dysfunction was diagnosed.

Pulmonary rehabilitation protocols in urgent lung transplantation patients / 世界急诊医学杂志（英文）

@#BACKGROUND: Prolonged invasive respiratory support and extracorporeal membrane oxygenation (ECMO) in patients requiring urgent lung transplantation (ULTx) present significant challenges to clinical practice due to severe underlying diseases and complex conditions. The aim of the study was to report the clinical outcomes of patients who received ULTx and followed the perioperative rehabilitation protocol implemented in a lung transplant center. METHODS: A retrospective analysis was conducted in ULTx patients who required preoperative invasive mechanical ventilation (IMV) and ECMO between January 2018 and January 2023. Data were retrieved from electronic medical records at our lung transplant center. RESULTS: Fourteen patients (mean age 57.43±10.97 years; 12 males, 2 females) underwent ULTx with bridging ECMO and IMV. The mean body mass index was 23.94±3.33 kg/m², and the mean Acute Physiology and Chronic Health Evaluation (APACHE) II score was 21.50±3.96. The Nutritional Risk Screening 2002 (NRS 2002) scores were ≥3. ULTx was performed after an 8.5-day waiting period (interquartile interval [IQR] 5.0-26.5 d). Following the surgeries, the average lengths of ECMO and IMV were 1.0 (IQR 1.0-2.0) d and 5.0 (IQR 3.0-7.3) d, respectively. The total length of hospital stay was 60.1±30.8 d, with an average intensive care unit stay of 38.3±22.9 d and post-operative hospitalization stay of 45.8±26.1 d. Two patients died within 30 d after ULTx, with a 30-day survival rate of 85.71%. CONCLUSION: Patients receiving ULTx showed an acceptable short-term survival rate, validating the practicality and safety of the treatment protocols implemented in our center.

Qinghao Biejiatang Treats Lung Diseases： A Review / 中国实验方剂学杂志

Qinghao Biejiatang， first recorded in the Detailed Analysis of Warm Diseases （《温病条辨》） written by WU Jutong in the Qing Dynasty， is composed of Artemisiae Annuae Herba， Trionycis Carapax， Rehmanniae Radix， Anemarrhenae Rhizoma， and Moutan Cortex. With the effects of nourishing Yin and relieving heat， this prescription is often used to treat the syndrome of Yin deficiency and internal heat. The deficiency of healthy Qi， invasion of pathogenic toxins， loss of lung Yin， and generation of deficiency-heat are pathogenesis of lung cancer， pneumonia and other lung diseases， the treatment of which usually follows the principles of nourishing Yin， reinforcing healthy Qi， clearing lung， and eliminating heat. With the effects basically in accordance with the treatment principles of lung diseases， Qinghao Biejiatang is widely used in the treatment of lung diseases such as lung cancer-associated fever， hemoptysis or combined with bone metastasis， tuberculosis， community-acquired pneumonia， and pneumonia caused by severe acute respiratory syndrome coronavirus 2（SARS-CoV-2）. Basic experiments have shown that Qinghao Biejiatang may exert the therapeutic effects by reducing inflammation， maintaining immune balance， regulating intestinal flora， hormone secretion， lipid metabolism， and inhibiting tumor and oxidative damage. In addition， the main active ingredients of this prescription include artemisinin， luteolin， sitosterol， stigmasterol， polysaccharides， catalpol， paeoniflorin， quercetin， paeonol， gallic acid， timosaponin， and mangiferin， which have anti-tumor， anti-oxidant， anti-virus， inflammation-regulating， and immunomodulatory activities. The paper reviewed the clinical and basic studies of Qinghao Biejiatang in the treatment of lung diseases， aming to provide a theoretical basis for the clinical application.

A new proposal for phenotypic classification and outcome assessment of dermatomyositis based on clinical manifestations and serological testing

Abstract Background Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes clinical diagnosis and treatment of DM very challenging. Objectives Unsupervised classification in DM patients and analysis of key factors related to clinical outcomes. Methods This retrospective study was conducted between 2017 and 2022 at the Department of Rheumatology, Xiangya Hospital, Central South University. 162 DM patients were enrolled for unsupervised hierarchical cluster analysis. In addition, we divided the clinical outcomes of DM patients into four subgroups: withdrawal, stabilization, aggravation, and death, and compared the clinical profiles amongst the subgroups. Results Out of 162 DM patients, three clusters were defined. Cluster 1 (n = 40) was mainly grouped by patients with prominent muscular involvement and mild Interstitial Lung Disease (ILD). Cluster 2 (n = 72) grouped patients with skin rash, anti-Melanoma Differentiation Associated protein 5 positive (anti-MDA5+), and Rapid Progressive Interstitial Lung Disease (RP-ILD). Cluster 3 (n = 50) grouped patients with the mildest symptoms. The proportion of death increased across the three clusters (cluster 3 < cluster 1 < cluster 2). Study limitations The number of cases was limited for the subsequent construction and validation of predictive models. We did not review all skin symptoms or pathological changes in detail. Conclusions We reclassified DM into three clusters with different risks for poor outcome based on diverse clinical profiles. Clinical serological testing and cluster analysis are necessary to help clinicians evaluate patients during follow-up and conduct phenotype-based personalized care in DM.

Lessons from the pandemic and the value of a structured system of ultrasonographic findings in the diagnosis of COVID-19 pulmonary manifestations

ABSTRACT Objective We aimed to share our experience in implementing a structured system for COVID-19 lung findings, elucidating key aspects of the lung ultrasound score to facilitate its standardized clinical use beyond the pandemic scenario. Methods Using a scoring system to classify the extent of lung involvement, we retrospectively analyzed the ultrasound reports performed in our institution according to COVID-RADS standardization. Results The study included 69 thoracic ultrasound exams, with 27 following the protocol. The majority of patients were female (52%), with ages ranging from 1 to 96 years and an average of 56 years. Classification according to COVID-RADS was as follows: 11.1% in category 0, 37% in category 1, 44.4% in category 2, and 7.4% in category 3. Ground-glass opacities on tomography correlated with higher COVID-RADS scores (categories 2 and 3) in 82% of cases. Ventilatory assessment revealed that 50% of cases in higher COVID-RADS categories (2 and 3) required second-line oxygen supplementation, while none of the cases in lower categories (0 and 1) utilized this support. Conclusion Lung ultrasound has been widely utilized as a diagnostic tool owing to its availability and simplicity of application. In the context of the pandemic emergency, a pressing need for a focused and easily applicable assessment arose. The structured reporting system, incorporating ultrasound findings for stratification, demonstrated ease of replicability. This system stands as a crucial tool for screening, predicting severity, and aiding in medical decisions, even in a non-pandemic context.

Effect of Speaking Valves on Tracheostomy Decannulation

Abstract Introduction Despite several pediatric tracheostomy decannulation protocols there remains tremendous variability in practice. The effect of tracheostomy capping on decannulation has been studied but the role of speaking valves (SVs) is unknown. Objective Given the positive benefits SVs have on rehabilitation, we hypothesized that SVs would decrease time to tracheostomy decannulation. The purpose of the present study was to evaluate this in a subset of patients with chronic lung disease of prematurity (CLD). Methods A retrospective chart review was performed at a tertiary care children's hospital. A total of 105 patients with tracheostomies and CLD were identified. Data collected included demographics, gestational age, congenital cardiac disease, airway surgeries, granulation tissue excisions, SV and capping trials, tracheitis episodes, and clinic visits. Statistics were performed with logistic and linear regression. Results A total of 75 patients were included. The mean gestational age was 27 weeks (standard deviation [SD] =3.6) and the average birthweight was 1.1 kg (SD = 0.6). The average age at tracheostomy was 122 days (SD = 63). A total of 70.7% of the patients underwent decannulation and the mean time to decannulation (TTD) was 37 months (SD = 19). A total of 77.3% of the patients had SVs. Those with an SV had a longer TTD compared to those without (52 versus 35 months; p = 0.008). Decannulation was increased by 2 months for every increase in the number of hospital presentations for tracheitis (p =0.011). Conclusion The present study is the first, to our knowledge, to assess the effect of SVs on tracheostomy decannulation in patients with CLD showing a longer TTD when SVs are used.

The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study

Abstract Background Interstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue Diseases (CTD). This study evaluated the impact of hospitalization on mortality in an ethnically and racially diverse cohort of CTD-ILD patients. Methods We conducted a medical records review study at Montefiore Medical Center, Bronx, NY. We included 96 patients and collected data on demographic characteristics, reasons for hospitalization, length of stay, immunosuppressant therapy use, and mortality. We stratified our patients into two cohorts: hospitalized and nonhospitalized. The hospitalized cohort was further subdivided into cardiopulmonary and non-cardiopulmonary admissions. Two-sample tests or Wilcoxon's rank sum tests for continuous variables and Chi-square or Fisher's exact tests for categorical variables were used for analyses as deemed appropriate. Results We identified 213 patients with CTD-ILD. Out of them, 96 patients met the study's inclusion criteria. The majority of patients were females (79%), and self-identified as Hispanic (54%) and Black (40%). The most common CTDs were rheumatoid arthritis (RA) (29%), inflammatory myositis (22%), and systemic sclerosis (15%). The majority (76%) of patients required at least one hospitalization. In the non-hospitalized group, no deaths were observed, however we noted significant increase of mortality risk in hospitalized group (p = 0.02). We also observed that prolonged hospital stay (> 7 days) as well as older age and male sex were associated with increased mortality. Conclusion Prolonged (> 7 days) hospital stay and hospitalization for cardiopulmonary causes, as well as older age and male sex were associated with an increased mortality risk in our cohort of CTD-ILD patients.

Management of lung diseases under ultrasound monitoring: potential to make bronchopulmonary dysplasia in preterm infants as an avoidable disease / 中国当代儿科杂志

Bronchopulmonary dysplasia (BPD) is the most common chronic lung disease in preterm infants. Despite significant progress in the understanding of its etiology, mechanisms, prevention, and treatment, the prognosis remains poor. BPD not only has a high mortality rate but also causes persistent respiratory, neurological, and cardiovascular impairments in survivors. The author's team has successfully prevented the occurrence of BPD by managing neonatal lung diseases under lung ultrasound monitoring for nearly 7 years, opening up a new approach in BPD prevention. This article provides a brief overview of the approach, aiming to facilitate further research and provide more scientifically sound management strategies to prevent or minimize the occurrence of BPD.

Enfermedad pulmonar intersticial en colangitis biliar primaria / Interstitial Lung Disease in Primary Biliary Cholangitis

RESUMEN Durante el transcurso de la colangitis biliar primaria se puede desarrollar compromiso intersticial pulmonar: neumonía organizada, fibrosis intersticial, neumonía intersticial linfoide, neumonía intersticial no específica. A pesar de que el diagnóstico de colangitis biliar primaria usualmente precede a las manifestaciones pulmonares, puede ocurrir lo inverso. La frecuencia de enfermedad intersticial en pacientes con colangitis biliar primaria no es conocida con exactitud. Puede estar o no asociada a otras enfermeda des del tejido conectivo; por lo tanto, es necesario realizar una búsqueda sistemática de estas y de las manifestaciones pulmonares de dicha entidad. Presentamos el caso de una paciente con diagnóstico previo de colangitis biliar primaria, la cual desarrolla durante el curso de su enfermedad, afectación pulmonar intersticial.

ABSTRACT During the course of PBC, interstitial lung involvement may develop: organizing pneu monia, interstitial fibrosis, lymphoid interstitial pneumonia, or non-specific interstitial pneumonia. Although the diagnosis of PBC usually precedes pulmonary manifestations, the opposite can occur. The frequency of interstitial disease in patients with PBC is not exactly known. It may or may not be associated with other connective tissue diseases; therefore, it is necessary to carry out a systematic search of these diseases and the pulmonary manifestations of this entity. We present the case of a patient with a previ ous diagnosis of PBC, who developed interstitial lung involvement during the course of the disease.

Rendimiento diagnóstico y morbimortalidad de las biopsias pulmonares quirúrgicas en enfermedad pulmonar intersticial difusa

Introducción: Se denomina Enfermedad Pulmonar Intersticial Difusa (EPID) a un conjunto heterogéneo de patologías caracterizadas por inflamación y fibrosis pulmonar. El diagnóstico basado en patrones clínicos o radiológicos puede, ocasionalmente, ser insuficiente para iniciar un tratamiento. La biopsia pulmonar quirúrgica es una alternativa cuando se requiere aumentar la precisión diagnóstica luego de discusión multidisciplinaria. Objetivo: Describir el rendimiento diagnóstico, morbilidad y mortalidad de las biopsias quirúrgicas pulmonares en un hospital público chileno. Pacientes y Método: Cohorte retrospectiva de todos los pacientes a quienes se realizó biopsia quirúrgica por diagnóstico de EPID entre los años 2010 y 2020, indicada por un comité multidisciplinario. Se excluyen procedimientos similares o biopsias con diagnóstico de EPID como hallazgo incidental. Resultados: 38 pacientes intervenidos, mediana de edad de 63 años, 47% femenino. Solo 1 (2,6%) paciente operado de urgencia, y 34 (89,5%) por videotoracoscopía. 5 (13,1%) pacientes presentaron morbilidad, en 4 de ellos fuga aérea, ninguno requiriendo intervención adicional. No hubo rehospitalización, reoperación ni mortalidad a 90 días. En el 95% de los casos se alcanzó un diagnóstico preciso de la EPID tras discusión multidisciplinaria. Discusión: Se observa un alto rendimiento diagnóstico y una baja morbimortalidad en los pacientes estudiados. La baja frecuencia de procedimientos de urgencia y la adecuada indicación en comité multidisciplinario puede haber contribuido a la baja morbilidad. Conclusión: La biopsia pulmonar quirúrgica en un hospital general tiene un alto rendimiento diagnóstico cuando se discute en comité multidisciplinario para precisar el diagnostico en EPID, con una baja morbimortalidad si se seleccionan adecuadamente los pacientes.

Background: Interstitial Lung Disease (ILD) is a heterogeneous group of diseases characterized by inflammation and fibrosis of the lung. Diagnosis based exclusively on clinical or radiologic patterns may be inaccurate, and if a reliable diagnosis cannot be made, surgical lung biopsy can be strongly considered to increase the diagnostic yield after multidisciplinary committee. Objective: To review the diagnostic results, morbidity, and mortality of surgical biopsies in a chilean public health institution. Patients and Method: Retrospective cohort of patients operated for diagnostic purposes for ILD between 2010 - 2020. Surgical biopsies done for other diagnoses were excluded. Results: 38 patients were included, with a median age of 63 years, 47% were female. Only 1 patient (2.6%) underwent emergency surgery and 89.5% underwent minimally invasive surgery techniques. 5 patients had some morbidity (13.1%), 4 of them being air leak. All complications were successfully managed conservatively. We had no readmission, reoperations, or 90-day mortality in this cohort. In 95% of the cases an accurate diagnosis of ILD was reached after multidisciplinary discussion. Discussion: In our experience surgical lung biopsy has a high diagnostic yield and a low morbidity and mortality. A low number of emergency procedures and accurate surgical indication by an expert committee could explain the low morbidity. Conclusion: Surgical lung biopsy in a general hospital reach a high diagnostic performance when discussed in a multidisciplinary committee to specify the diagnosis in ILD, with low morbidity and mortality if patients are properly selected.

Risk factors for clinically significant diffuse parenchymal lung abnormalities persisting after severe COVID-19 pneumonia

Background & objectives: The risk factors for clinically significant diffuse parenchymal lung abnormalities (CS-DPLA) persisting after severe coronavirus disease 2019 (COVID-19) pneumonia remain unclear. The present study was conducted to assess whether COVID-19 severity and other parameters are associated with CS-DPLA. Methods: The study participants included patients who recovered after acute severe COVID-19 and presented with CS-DPLA at two or six month follow up and control group (without CS-DPLA). Adults volunteers without any acute illness, chronic respiratory illness and without a history of severe COVID-19 were included as healthy controls for the biomarker study. The CS-DPLA was identified as a multidimensional entity involving clinical, radiological and physiological pulmonary abnormalities. The primary exposure was the neutrophil-lymphocyte ratio (NLR). Recorded confounders included age, sex, peak lactate dehydrogenase (LDH), advanced respiratory support (ARS), length of hospital stay (LOS) and others; associations were analyzed using logistic regression. The baseline serum levels of surfactant protein D, cancer antigen 15-3 and transforming growth factor-? (TGF-?) were also compared among cases, controls and healthy volunteers. Results: We identified 91/160 (56.9%) and 42/144 (29.2%) participants with CS-DPLA at two and six months, respectively. Univariate analyses revealed associations of NLR, peak LDH, ARS and LOS with CS-DPLA at two months and of NLR and LOS at six months. The NLR was not independently associated with CS-DPLA at either visit. Only LOS independently predicted CS-DPLA at two months [adjusted odds ratios (aOR) (95% confidence interval [CI]), 1.16 (1.07-1.25); P<0.001] and six months [aOR (95% CI) and 1.07 (1.01-1.12); P=0.01]. Participants with CS-DPLA at six months had higher baseline serum TGF-? levels than healthy volunteers. Interpretation and conclusions: Longer hospital stay was observed to be the only independent predictor of CS-DPLA six months after severe COVID-19. Serum TGF-? should be evaluated further as a biomarker.

GOLD Strategy Update 2023: Rationale and Clinical Implications in Indian Perspective

Chronic obstructive lung disease (COPD) is a major contributor to global disease burden with a huge socioeconomic impact. Global initiative for chronic obstructive lung disease (GOLD) strategy update 2023 is a step forward in the direction of evidence-based practice. Key topics like taxonomic classification, ABE grouping, single inhaler triple therapy, correct use of inhalers, etc. would have a positive impact on documentation and prescription practices in COPD management. The tobacco consumption habits are peculiar in India. There is a variation in prevalence estimates across diverse cultures. Non-smoking risk factors are important in Indian subcontinent especially in female and younger populations. We discuss the rationale of latest GOLD strategy update, its implications, and challenges in the management of COPD in Indian context.

Relationship between Body Surface Area and Pulmonary Functions in Patients of Silicosis

Background and objective: Silicosis is one of the oldest occupational lung diseases. However, there are very few studies identifying the anthropometric variables associated with silicosis. The present study aimed at studying the association between body surface area (BSA), pulmonary function indices, and 6-minute walk distance (6MWD) in patients with silicosis. Materials and methods: The study was conducted on 102 male patients of silicosis. Height and weight were measured to calculate BSA. Spirometry and 6 minute-walk tests were performed. Data were analyzed using EPI info V 7 software. Student's t-test of significance (ANOVA) was applied to test the difference between means. Results: There are no significant changes found in the 6-minute walk distance with years of exposure and BSA. Statistically significant lower values of pulmonary function indices were observed in patients with BSA <1.6 sq m. Statistically significant higher values of forced expiratory volume in the first second and forced vital capacity were observed in patients with BSA >1.9 sq m in all categories of exposure. Conclusion: In conclusion, patients of silicosis with >1.9 sq m BSA had higher values of pulmonary function indices. Large body size may be of value in protection from developing occupational lung disease.

Caracterización de la enfermedad pulmonar intersticial en los pacientes con esclerosis sistémica

Introducción: La esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por vasculopatía, activación del sistema inmune y aumento de depósitos de matriz extracelular. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, ha pasado a ser la primera causa de muerte en estos pacientes. La afección pulmonar puede ocurrir como hipertensión o enfermedad pulmonar intersticial. La meta del tratamiento es detener el deterioro de la función pulmonar. Objetivo: Caracterizar las manifestaciones clínicas, imagenológicas y la función respiratoria en pacientes con esclerosis sistémica y enfermedad pulmonar intersticial. Métodos: Se realizó un estudio observacional, descriptivo de corte transversal en el período comprendido entre diciembre de 2018 y diciembre de 2019. En el Servicio de Reumatología para caracterizar la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. El universo estuvo constituido por 168 pacientes, diagnosticados con esa enfermedad y la muestra se conformó por 55 pacientes que cumplieron los criterios de inclusión establecidos. Resultados: La enfermedad pulmonar intersticial fue más frecuente en pacientes mayores de 40 años, del sexo femenino, piel mestiza, predominó la forma clínica difusa, el síntoma más frecuente fue la disnea de esfuerzo, la mayoría tuvo ANA positivo y el patrón tomográfico en panal de abejas. La capacidad vital forzada estaba disminuida con mayor frecuencia, se asoció a un comportamiento autoinmune positivo para anti-ScL-70. Conclusiones: Se caracterizó las manifestaciones clínicas y radiográficas de la enfermedad pulmonar intersticial fueron comprobadas por la utilidad de la tomografía computarizada y la espirometría para identificar la presencia de fibrosis pulmonar.

Introduction: Systemic sclerosis is a chronic autoimmune disease, characterized by vasculopathy, activation of the immune system and increased extracellular matrix deposits. In recent years, lung involvement has gained great importance, it has become the first cause of death in these patients. Lung involvement can occur as hypertension or interstitial lung disease. The goal of treatment is to stop the decline in lung function. Objective: To characterize the clinical and imaging manifestations and respiratory function in patients with systemic sclerosis and interstitial lung disease. Methods: An observational, descriptive, cross-sectional study was carried out from December 2018 to December 2019 in the rheumatology service to characterize interstitial lung disease in patients with systemic sclerosis. The universe consisted of 168 patients diagnosed with this disease and the sample was made up of 55 patients who met the established inclusion criteria. Results: Interstitial lung disease was more frequent in patients older than 40 years, female, mixed-race skin color, the diffuse clinical form predominated, the most frequent symptom was exertional dyspnea, the majority had positive ANA and the pattern honeycomb tomography. Forced vital capacity was more frequently decreased, associated with positive autoimmune behavior for Anti-ScL-70. Conclusions: The radiographic and clinical manifestations of PID were verified by the usefulness of computed tomography and spirometry to identify the presence of pulmonary fibrosis.

An 8-year-old boy with diffuse cystic pulmonary tuberculosis: An intriguing story

Diffuse cystic lung disease due to pulmonary tuberculosis (TB) is rare. We describe an 8-year-old boy who presented with acute onset respiratory distress while on a compliant anti-tubercular treatment for a recently diagnosed pulmonary TB. On clinical examination, hypoxemia, clubbing, and features of the right-sided heart failure were observed. High-resolution computed tomography of the chest revealed extensive cystic lung parenchymal changes with ground glassing and consolidation, and echocardiography indicated the presence of pulmonary arterial hypertension. His treatment included supplemental oxygen, sildenafil, prednisolone, and anti-tubercular drugs. At the two-year follow-up, the patient showed complete clinical recovery and resolution of cysts on the chest X-ray.

The Use of Intraoperative Extracorporeal Membrane Oxygenation in Lung Transplantation: Initial Institutional Experience

ABSTRACT Introduction: Lung transplantation is the final treatment option for end-stage lung disease, and extracorporeal membrane oxygenation (ECMO) is increasingly being used during lung transplantation. Objective: The present study aimed to review our initial experience with patients who underwent lung transplantation with or without ECMO since the implementation of the lung transplantation program at our center. Methods: Data were prospectively collected on all patients between December 2016 and December 2018. Patients undergoing ECMO as a bridge to lung transplantation were excluded. Results: A total of 48 lung transplants were performed, and ECMO was used in 29 (60.4%) cases. Twenty (83%) patients were female. The median age was 48.5 (range, 14-64) years. The most common indications were idiopathic interstitial pneumonia in 9 (31%) patients, chronic obstructive pulmonary disease in 7 (24.1%) patients, and bronchiectasis in 6 (20.7%) patients. Sequential bilateral lung transplantation was performed in all patients. The 30-day mortality was 20.6% (6/29) for patients with ECMO, however, it was 10.5 (2/19) for patients without ECMO (P=0.433). The median length of stay in the intensive care unit (ICU) was 5 (range, 2-25) days. The ECMO weaning rate was 82.8% (24/29). One-year survival was 62.1% with ECMO versus 78.9% without ECMO, and the 3-year survival was 54.1% versus 65.8%, respectively (P=0.317). Conclusions: ECMO is indicated for more severe patients who underwent lung transplantation. The use of ECMO provides adjuvant support during surgery and the mortality rate is acceptable. Survival is also as similar as non-ECMO patients. ECMO is appropriate for critically ill patients.

Ventilación mecánica no invasiva en pacientes con enfermedad pulmonar obstructiva crónica agudizada en cuidados intensivos: diez años de experiencia / Non invasive mechanic ventilation in patients with acute chronic obstructive lung disease in intensive cares: ten years of experience:en

Introducción: La ventilación no invasiva es un tratamiento efectivo en pacientes con enfermedad pulmonar agudizada. Objetivo: Describir las características demográficas, clínicas, ventilatorias y hemogasométricas en pacientes tratados con ventilación no invasiva. Métodos: Se realizó un estudio observacional, descriptivo, longitudinal y prospectivo de 234 pacientes con enfermedad pulmonar obstructiva crónica agudizada, ventilados de forma no invasiva en la Unidad de Cuidados Intensivos del Hospital Provincial Clínico-Quirúrgico Docente Saturnino Lora de Santiago de Cuba de enero de 2011 a septiembre de 2021. Resultados: En la serie predominaron el sexo masculino, la neumonía extrahospitalaria y la insuficiencia cardíaca crónica; mientras que la edad media fue de 71 años y la ventilación no invasiva fracasó en 53,8 % de los afectados. Asimismo, la frecuencia respiratoria disminuyó de 34,3 a 23,5 respiraciones por minuto en la segunda hora y se observó, además, un incremento del pH, así como de la relación presión arterial de O2/fracción inspirada de O2 y saturación de oxígeno a la pulsioximetría/fracción inspiratoria de O2. La presión arterial de CO2 tuvo valores promedio de 61,8 mmHg al inicio y de 60,7 mmHg en la segunda hora. Conclusiones: Los valores basales de las variables clínicas, hemogasométricas y ventilatorias mejoraron luego del tratamiento con ventilación no invasiva. Entre los parámetros asociados al fracaso del tratamiento figuraron: frecuencia cardiaca, frecuencia respiratoria, presión arterial de CO2, escala de coma de Glasgow, pH y presencia de fugas; igualmente, la estadía prolongada, la ventilación por más de 48 horas y la mortalidad estuvieron relacionadas con dicho fracaso.

Introduction: The non invasive ventilation is an effective treatment in patients with acute lung disease. Objective: To describe the demographic, clinical, ventilatory and hemogasometric characteristics in patients treated with non invasive ventilation. Methods: An observational, descriptive, longitudinal and prospective study of 234 patients with chronic obstructive lung disease, non-invasive ventilated in the Intensive Cares Unit of Saturnino Lora Teaching Clinical-Surgical Provincial Hospital in Santiago de Cuba, was carried out, from January, 2011 to September, 2021. Results: In the series there was a prevalence of male sex, non hospital acquired pneumonia and chronic heart failure; while the mean age was 71 years and non invasive ventilation failed in 53.8 % of those affected. Also, the respiratory rate decreased from 34.3 to 23.5 breaths per minute in the second hour and an increase in pH was also observed, as well as in the relationship arterial pressure of O2/inspired fraction of O2 and oxygen saturation to the pulsioximetry/inspiratory fraction of O2. The arterial pressure of CO2 had average values of 61.8 mmHg to the beginning and of 60.7 mmHg in the second hour. Conclusions: The basal values of clinical, hemogasometric and ventilatory variables improved after treatment with non invasive ventilation. Among the parameters associated with the treatment failure there were heart frequency, respiratory frequency, arterial pressure of CO2, coma Glasgow scale, pH and leaks; equally, the long stay, the ventilation for more than 48 hours and mortality were related to this failure.

Solid placental transmogrification of the lung: A case report and literature review / 北京大学学报(医学版)

Placental transmogrification of the lung (PTL) is a very rare benign lung lesion. There are only about 40 cases reported in the literature. The imaging and histological features of PTL cases in the publication are various, most of which are cystic and a few of which are solid. Being extremely rare, the solid PTL is unknown to major pathologists and surgeons. We reported a case of solid PTL in the anterior mediastinum. The patient was a 52-year-old male with no history of smoking and without symptoms. During physical examination, chest CT revealed a circular low-density lesion with a maximum diameter of 2.9 cm beside the spine in the posterior basal segment of the left lower lobe of the lung. The wedge resection was performed by video-assisted thoracoscopy. Grossly, a round nodule was located underneath the visceral pleura. It was about 3.0 cm×3.0 cm×1.6 cm and the cut surface was grey-red, soft and spongy. Microscopically, the nodule was constituted of papillare, which resembled placental villi at low magnification. The axis of papillae was edema, in which some mild round cells with clear cytoplasm and CD10 positive staining aggregated and transitioned to immature adipocytes and amorphous pink materials deposited with a few of inflammatory cells infiltration. The surface of papillae was covered with disconti-nuous alveolar epithelium. Combined with the typical morphology and immunohistochemical characteristics of CD10 positive, the diagnosis was PTL. The patient was followed up for 1 year without recurrence and discomfort. So far, the pathogenesis of PTL is unclear. The major hypotheses include hamartoma, variant of emphysema and clonal hyperplasia of stromal cells. Based on the study of our case and publication, we speculate that the hyperplasia of stromal cells located in the alveolar septa might be the first step to form the solid PTL. With the progression of the disease, a typical unilateral cystic nodule develops as a result of secondary cystic degeneration due to the occlusive valve effect. Surgery is the only option for diagnosis and treatment of PTL. The clinician should make an individualized operation plan according to the clinical manifestations, location and scope of the lesion, and preserve the surrounding normal lung tissue as much as possible while completely removing the lesion. There is a favorable prognosis.

Expression and clinical significance of plasma exosomal miR-34-5p and miR-142-3p in systemic sclerosis / 北京大学学报(医学版)

OBJECTIVE@#To detect the expression of plasma exosomal microRNA (miRNA) in systemic sclerosis (SSc), and to investigate its clinical significance.@*METHODS@#A total of 20 patients who were initially diagnosed with SSc and did not receive medication in Department of Rheumatology and Immunology of Meizhou People' s Hospital from January 2020 to January 2022 were recruited, as well as 15 healthy individuals whose gender and age matched with those of the SSc patients. Plasma exosomes were isolated using ultracentrifugation method. The expression levels of exosomal miR-34-5p, miR-92-3p and miR-142-3p were detected by quantative real-time polymerase chain reaction (qRT-PCR). Correlations between the expression levels of exosomal miRNAs and clinical characteristic were analyzed by Spearman's rank correlation coefficient test.@*RESULTS@#The mean age of 20 patients with SSc was (52.6±12.6) years, including 7 males and 13 females. Among the 20 SSc patients, 13 cases were diagnosed as limited cutaneous systemic sclerosis (lcSSc) and 7 cases were diagnosed as diffuse cutaneous systemic sclerosis (dcSSc) according to the extent of skin involvement. According to the findings of high resolution chest CT, 7 of 20 SSc patients were diagnosed with interstitial lung disease (ILD) and 13 SSc patients were diagnosed with non-ILD. The expression levels of exosomal miR-34-5p, miR-92-3p and miR-142-3p were significantly elevated in the SSc patients compared with those in the healthy controls group (P=0.003, P=0.000 1, and P=0.016, respectively). Compared with the SSc patients without ILD, the expression levels of miR-34-5p and miR-142-3p were significantly lower in the SSc patients with ILD (P=0.037 and P=0.015, respectively). The expression levels of exosomal miR-34-5p and miR-142-3p showed negative correlation with ILD (r=-0.48, P=0.031 and r=-0.55, P=0.011, respectively), and arthritis (r=-0.46, P=0.040 and r=-0.48, P=0.032, respectively). The expression levels of exosomal miR-142-3p showed a negative correlation with erythrocyte sedimentation rate (ESR) (r=-0.55, P=0.012).@*CONCLUSION@#Plasma exosomal miR-34-5p, miR-92-3p and miR-142-3p were dysregulated in SSc. The dyregulation of exosomal miR-34-5p and miR-142-3p showed correlation with SSc associated ILD (SSc-ILD).