BACKGROUND Alagille syndrome is a multisystem disease that results in various vascular anomalies,commonly involving the cardiac and pulmonary systems.To the best of our knowledge,there is no literature regarding the c...BACKGROUND Alagille syndrome is a multisystem disease that results in various vascular anomalies,commonly involving the cardiac and pulmonary systems.To the best of our knowledge,there is no literature regarding the cardiovascular outcomes of these patients in association with coronavirus disease 2019(COVID-19).CASE SUMMARY A 34-year-old woman with a history of Alagille syndrome who underwent successful atrial septal defect with partial anomalous pulmonary veins and patent ductus arteriosus repair,as well as left pulmonary artery catheterization and stenting in childhood due to pulmonary stenosis.The patient was without any respiratory symptoms and was a dancer prior to contracting COVID-19.Several weeks after her COVID-19 infection,she developed left pulmonary artery stent thrombosis and subsequent symptomatic pulmonary hypertension.A treatment strategy of anticoagulation alongside pharmacological agents for pulmonary hypertension for 3 months followed by balloon pulmonary artery angioplasty to reopen the stenosis was unsuccessful.CONCLUSION In the era of COVID-19,patients with pulmonary vascular malformations and endovascular stents are at an increased risk for chronic thromboembolic disease.Patients may benefit from prophylactic antiplatelet or anticoagulation therapy.Stent thrombosis is a devastating phenomenon and should be treated urgently and aggressively with balloon pulmonary angioplasty,and/or a thrombolytic agent.展开更多
BACKGROUND Currently,the mainstay of chronic eosinophilic pneumonia(CEP)treatment is corticosteroids,usually with a favorable response and good prognosis.However,relapse is common,requiring long-term use of corticoste...BACKGROUND Currently,the mainstay of chronic eosinophilic pneumonia(CEP)treatment is corticosteroids,usually with a favorable response and good prognosis.However,relapse is common,requiring long-term use of corticosteroids,with risk of significant treatment-related complications.The dire need to develop new treatments for patients with CEP,who are dependent on,or resistant to corticosteroids has led to exploring novel therapies.We herein describe a patient with acute relapse of CEP,who was successfully treated with benralizumab,an IL-5 Rαantagonist that has demonstrated rapid anti-eosinophil action in patients with asthma.Currently,only three recent patient reports on CEP relapse,also demonstrated successful treatment with benralizumab alone,without corticosteroids.CASE SUMMARY A 31-year-old non-smoking woman presented in our hospital with a 3 wk history of shortness of breath,dry cough and fever up to 38.3℃.Laboratory examination revealed leukocytosis 10240 K/μL,eosinophilia 900 K/μL and normal values of hemoglobin,platelets,creatinine and liver enzymes.Computed tomography of the chest showed a mediastinal lymphadenopathy and consolidations in the right upper and left lower lobes.CEP was diagnosed,and the patient was treated with hydrocortisone intravenously,followed by oral prednisone,with prompt improvement.Three months later,she presented with relapse of CEP:aggravation of dyspnea,rising of eosinophilia and extension of pulmonary infiltrates on chest X-ray.She was treated with benralizumab only,with clinical improvement within 2 wk,and complete resolution of lung infiltrates following 5 wk.CONCLUSION Due to Benralizumab’s dual mechanism of action,it both neutralizes IL-5 Rαpro-eosinophil functions and triggers apoptosis of eosinophils.We therefore maintain benralizumab can serve as a reasonable therapy choice for every patient with chronic eosinophilic pneumonia and a good alternative for corticosteroids.展开更多
Since 1934,when Hirschfelder first reported magnesium(Mg) deficiency syndrome in man, considerable clinicalworks related to hypomagnesemia have appeared in medicalliterature. Recently, it is considered that Mg is a ki...Since 1934,when Hirschfelder first reported magnesium(Mg) deficiency syndrome in man, considerable clinicalworks related to hypomagnesemia have appeared in medicalliterature. Recently, it is considered that Mg is a kind ofnatural antagonist of calcium (Ca). Mg has some effects onthe smooth muscles of the bronchial and pulmonary arteries,and to relax the spasm of them. Therefore, there may exista close relation between Mg and cor pulmonale caused bychronic obstructive pulmonay desease (COPD).展开更多
PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immuno-suppression. The gastrointestinal manifestations of posttra...PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immuno-suppression. The gastrointestinal manifestations of posttransplant lymphoproliferative disorder in lung transplant recipients have not been fully characterized. METHODS: Case presentation and 16 previously reported cases of posttransplant lymphoproliferative disorder with gastrointestinal involvement are reviewed. RESULTS: Patient ages ranged from 25 to 65 (median, 52) years. Median time fromlung transplantation to onset of posttransplant lymphoproliferative disorder was 36 (range, 1-109) months; 35 percent of cases (6/17) occurred within 18 months; Eighty-eight percent of patients (15/17) had positive Epstein-Barr virus serology before transplantation. In five patients (29 percent), the posttransplant lymphoproliferative disorder also involved sites other than the gastrointestinal tract. The most common gastrointestinal site of posttransplant lymphoproliferative disorder was the colon, followed by the small intestine and stomach. Clinical features included abdominal pain, nausea, and bloody diarrhea. Diagnosis was based on typical pathologic changes on gastrointestinal tract biopsy obtained mainly by colonoscopy. Treatment included a reduction in the immunosuppressive regimen in 15 of 17 cases (88 percent) and surgical resection in 10 (59 percent). One patient was untreated. Seven of 16 patients (44 percent) responded to treatment and 9 patients died. Median time from onset of posttransplant lymphoproliferative disorder to death was 70 (range,10-85) days. CONCLUSIONS: Posttransplant lymphoproliferative disorder with gastrointestinal involvement is a unique entity that should be considered in all Epstein-Barr-Virus-positive lung transplant recipients who present with abdominal symptoms. Although immunosuppressive modulation and resection can lead to remission, the risk of death is 50 percent.展开更多
Background:Available pediatric treatments for acute cough are limited by lack of demonstrated efficacy.The objective of this trial is to compare the effects of a poIysaccharide—resin-honey based cough syrup,and carbo...Background:Available pediatric treatments for acute cough are limited by lack of demonstrated efficacy.The objective of this trial is to compare the effects of a poIysaccharide—resin-honey based cough syrup,and carbocysteine syrups on nocturnal and daytime cough associated with childhood upper respiratory tract infections(URIs).展开更多
Background Myocarditis is one of the presentations of multisystemic infammatory syndrome in children(MIS-C)following coronavirus disease 2019(COVID-19).Although the reported short-term prognosis is good,data regarding...Background Myocarditis is one of the presentations of multisystemic infammatory syndrome in children(MIS-C)following coronavirus disease 2019(COVID-19).Although the reported short-term prognosis is good,data regarding medium-term functional capacity and limitations are scarce.This study aimed to evaluate exercise capacity as well as possible cardiac and respiratory limitations in children recovered from MIS-C related myocarditis.Methods Fourteen patients who recovered from MIS-C related myocarditis underwent spirometry and cardiopulmonary exercise testing(CPET),and their results were compared with an age-,sex-,weight-and activity level-matched healthy control group(n=14).Results All participants completed the CPET with peak oxygen uptake(peak.V O2),and the results were within the normal range(MIS-C 89.3%±8.9%and Control 87.9%±13.7%predicted.V O2).Five post-MIS-C patients(35%)had exerciserelated cardio-respiratory abnormalities,including oxygen desaturation and oxygen-pulse fattening,compared to none in the control group.The MIS-C group also had lower peak exercise saturation(95.6±3.5 vs.97.6±1.1)and lower breathing reserve(17.4%±7.5%vs.27.4%±14.0%of MVV).Conclusions Patients who recovered from MIS-C related myocarditis may present exercise limitations.Functional assessment(e.g.,CPET)should be included in routine examinations before allowing a return to physical activity in post-MIS-C myocarditis.Larger,longer term studies assessing functional capacity and focusing on physiological mechanisms are needed.展开更多
The Omicron variant of the severe acute respiratory syndrome coronavirus 2(SARS‑CoV‑2)infected a substantial proportion of Chinese population,and understanding the factors underlying the severity of the disease and fa...The Omicron variant of the severe acute respiratory syndrome coronavirus 2(SARS‑CoV‑2)infected a substantial proportion of Chinese population,and understanding the factors underlying the severity of the disease and fatality is valuable for future prevention and clinical treatment.We recruited 64 patients with invasive ventilation for COVID-19 and performed metatranscriptomic sequencing to profile host transcriptomic profiles,plus viral,bacterial,and fungal content,as well as virulence factors and examined their relationships to 28-day mortality were examined.In addition,the bronchoalveolar lavage fluid(BALF)samples from invasive ventilated hospital/community-acquired pneumonia patients(HAP/CAP)sampled in 2019 were included for comparison.Genomic analysis revealed that all Omicron strains belong to BA.5 and BF.7 sub-lineages,with no difference in 28-day mortality between them.Compared to HAP/CAP cohort,invasive ventilated COVID-19 patients have distinct host transcriptomic and microbial signatures in the lower respiratory tract;and in the COVID-19 non-survivors,we found significantly lower gene expressions in pathways related viral processes and positive regulation of protein localization to plasma membrane,higher abundance of opportunistic pathogens including bacterial Alloprevotella,Caulobacter,Escherichia-Shigella,Ralstonia and fungal Aspergillus sydowii and Penicillium rubens.Correlational analysis further revealed significant associations between host immune responses and microbial compositions,besides synergy within viral,bacterial,and fungal pathogens.Our study presents the relationships of lower respiratory tract microbiome and transcriptome in invasive ventilated COVID-19 patients,providing the basis for future clinical treatment and reduction of fatality.展开更多
文摘BACKGROUND Alagille syndrome is a multisystem disease that results in various vascular anomalies,commonly involving the cardiac and pulmonary systems.To the best of our knowledge,there is no literature regarding the cardiovascular outcomes of these patients in association with coronavirus disease 2019(COVID-19).CASE SUMMARY A 34-year-old woman with a history of Alagille syndrome who underwent successful atrial septal defect with partial anomalous pulmonary veins and patent ductus arteriosus repair,as well as left pulmonary artery catheterization and stenting in childhood due to pulmonary stenosis.The patient was without any respiratory symptoms and was a dancer prior to contracting COVID-19.Several weeks after her COVID-19 infection,she developed left pulmonary artery stent thrombosis and subsequent symptomatic pulmonary hypertension.A treatment strategy of anticoagulation alongside pharmacological agents for pulmonary hypertension for 3 months followed by balloon pulmonary artery angioplasty to reopen the stenosis was unsuccessful.CONCLUSION In the era of COVID-19,patients with pulmonary vascular malformations and endovascular stents are at an increased risk for chronic thromboembolic disease.Patients may benefit from prophylactic antiplatelet or anticoagulation therapy.Stent thrombosis is a devastating phenomenon and should be treated urgently and aggressively with balloon pulmonary angioplasty,and/or a thrombolytic agent.
文摘BACKGROUND Currently,the mainstay of chronic eosinophilic pneumonia(CEP)treatment is corticosteroids,usually with a favorable response and good prognosis.However,relapse is common,requiring long-term use of corticosteroids,with risk of significant treatment-related complications.The dire need to develop new treatments for patients with CEP,who are dependent on,or resistant to corticosteroids has led to exploring novel therapies.We herein describe a patient with acute relapse of CEP,who was successfully treated with benralizumab,an IL-5 Rαantagonist that has demonstrated rapid anti-eosinophil action in patients with asthma.Currently,only three recent patient reports on CEP relapse,also demonstrated successful treatment with benralizumab alone,without corticosteroids.CASE SUMMARY A 31-year-old non-smoking woman presented in our hospital with a 3 wk history of shortness of breath,dry cough and fever up to 38.3℃.Laboratory examination revealed leukocytosis 10240 K/μL,eosinophilia 900 K/μL and normal values of hemoglobin,platelets,creatinine and liver enzymes.Computed tomography of the chest showed a mediastinal lymphadenopathy and consolidations in the right upper and left lower lobes.CEP was diagnosed,and the patient was treated with hydrocortisone intravenously,followed by oral prednisone,with prompt improvement.Three months later,she presented with relapse of CEP:aggravation of dyspnea,rising of eosinophilia and extension of pulmonary infiltrates on chest X-ray.She was treated with benralizumab only,with clinical improvement within 2 wk,and complete resolution of lung infiltrates following 5 wk.CONCLUSION Due to Benralizumab’s dual mechanism of action,it both neutralizes IL-5 Rαpro-eosinophil functions and triggers apoptosis of eosinophils.We therefore maintain benralizumab can serve as a reasonable therapy choice for every patient with chronic eosinophilic pneumonia and a good alternative for corticosteroids.
文摘Since 1934,when Hirschfelder first reported magnesium(Mg) deficiency syndrome in man, considerable clinicalworks related to hypomagnesemia have appeared in medicalliterature. Recently, it is considered that Mg is a kind ofnatural antagonist of calcium (Ca). Mg has some effects onthe smooth muscles of the bronchial and pulmonary arteries,and to relax the spasm of them. Therefore, there may exista close relation between Mg and cor pulmonale caused bychronic obstructive pulmonay desease (COPD).
文摘PURPOSE: Lymphoproliferative disorder is a well-recognized complication of lung transplantation. Risk factors include Epstein-Barr virus infection and immuno-suppression. The gastrointestinal manifestations of posttransplant lymphoproliferative disorder in lung transplant recipients have not been fully characterized. METHODS: Case presentation and 16 previously reported cases of posttransplant lymphoproliferative disorder with gastrointestinal involvement are reviewed. RESULTS: Patient ages ranged from 25 to 65 (median, 52) years. Median time fromlung transplantation to onset of posttransplant lymphoproliferative disorder was 36 (range, 1-109) months; 35 percent of cases (6/17) occurred within 18 months; Eighty-eight percent of patients (15/17) had positive Epstein-Barr virus serology before transplantation. In five patients (29 percent), the posttransplant lymphoproliferative disorder also involved sites other than the gastrointestinal tract. The most common gastrointestinal site of posttransplant lymphoproliferative disorder was the colon, followed by the small intestine and stomach. Clinical features included abdominal pain, nausea, and bloody diarrhea. Diagnosis was based on typical pathologic changes on gastrointestinal tract biopsy obtained mainly by colonoscopy. Treatment included a reduction in the immunosuppressive regimen in 15 of 17 cases (88 percent) and surgical resection in 10 (59 percent). One patient was untreated. Seven of 16 patients (44 percent) responded to treatment and 9 patients died. Median time from onset of posttransplant lymphoproliferative disorder to death was 70 (range,10-85) days. CONCLUSIONS: Posttransplant lymphoproliferative disorder with gastrointestinal involvement is a unique entity that should be considered in all Epstein-Barr-Virus-positive lung transplant recipients who present with abdominal symptoms. Although immunosuppressive modulation and resection can lead to remission, the risk of death is 50 percent.
文摘Background:Available pediatric treatments for acute cough are limited by lack of demonstrated efficacy.The objective of this trial is to compare the effects of a poIysaccharide—resin-honey based cough syrup,and carbocysteine syrups on nocturnal and daytime cough associated with childhood upper respiratory tract infections(URIs).
文摘Background Myocarditis is one of the presentations of multisystemic infammatory syndrome in children(MIS-C)following coronavirus disease 2019(COVID-19).Although the reported short-term prognosis is good,data regarding medium-term functional capacity and limitations are scarce.This study aimed to evaluate exercise capacity as well as possible cardiac and respiratory limitations in children recovered from MIS-C related myocarditis.Methods Fourteen patients who recovered from MIS-C related myocarditis underwent spirometry and cardiopulmonary exercise testing(CPET),and their results were compared with an age-,sex-,weight-and activity level-matched healthy control group(n=14).Results All participants completed the CPET with peak oxygen uptake(peak.V O2),and the results were within the normal range(MIS-C 89.3%±8.9%and Control 87.9%±13.7%predicted.V O2).Five post-MIS-C patients(35%)had exerciserelated cardio-respiratory abnormalities,including oxygen desaturation and oxygen-pulse fattening,compared to none in the control group.The MIS-C group also had lower peak exercise saturation(95.6±3.5 vs.97.6±1.1)and lower breathing reserve(17.4%±7.5%vs.27.4%±14.0%of MVV).Conclusions Patients who recovered from MIS-C related myocarditis may present exercise limitations.Functional assessment(e.g.,CPET)should be included in routine examinations before allowing a return to physical activity in post-MIS-C myocarditis.Larger,longer term studies assessing functional capacity and focusing on physiological mechanisms are needed.
基金funded by the National Key Research and Development Program of China(2022YFC2303200)Capital Development Key Grant of China(2022-1-5091).
文摘The Omicron variant of the severe acute respiratory syndrome coronavirus 2(SARS‑CoV‑2)infected a substantial proportion of Chinese population,and understanding the factors underlying the severity of the disease and fatality is valuable for future prevention and clinical treatment.We recruited 64 patients with invasive ventilation for COVID-19 and performed metatranscriptomic sequencing to profile host transcriptomic profiles,plus viral,bacterial,and fungal content,as well as virulence factors and examined their relationships to 28-day mortality were examined.In addition,the bronchoalveolar lavage fluid(BALF)samples from invasive ventilated hospital/community-acquired pneumonia patients(HAP/CAP)sampled in 2019 were included for comparison.Genomic analysis revealed that all Omicron strains belong to BA.5 and BF.7 sub-lineages,with no difference in 28-day mortality between them.Compared to HAP/CAP cohort,invasive ventilated COVID-19 patients have distinct host transcriptomic and microbial signatures in the lower respiratory tract;and in the COVID-19 non-survivors,we found significantly lower gene expressions in pathways related viral processes and positive regulation of protein localization to plasma membrane,higher abundance of opportunistic pathogens including bacterial Alloprevotella,Caulobacter,Escherichia-Shigella,Ralstonia and fungal Aspergillus sydowii and Penicillium rubens.Correlational analysis further revealed significant associations between host immune responses and microbial compositions,besides synergy within viral,bacterial,and fungal pathogens.Our study presents the relationships of lower respiratory tract microbiome and transcriptome in invasive ventilated COVID-19 patients,providing the basis for future clinical treatment and reduction of fatality.
