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
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.
