Background: Noninvasive ventilation (NIV) is an important therapeutic modality for the treatment of acute respiratory failure (ARF). In this review, we critically analyze randomized controlled trials on the most used ...Background: Noninvasive ventilation (NIV) is an important therapeutic modality for the treatment of acute respiratory failure (ARF). In this review, we critically analyze randomized controlled trials on the most used NIV interfaces in the treatments of ARF. Methods: The searches were conducted in the Medline, Lilacs, PubMed, Cochrane, and Pedro databases from June to November 2021. The inclusion criteria were Randomized clinical trials (RCTs) published from 2016 to 2021 in Portuguese, Spanish, or English and involving adults (aged ≥ 18 years). The eligibility criteria for article selection were based on the PICO strategy: Population—Adults with ARF;Intervention—NIV Therapy;Comparison—Conventional oxygen therapy, high-flow nasal cannula (HFNC) oxygen therapy, or NIV;Outcome—improvement in ARF. The search for articles and the implementation of the inclusion criteria were independently conducted by two researchers. Results: Seven scientific articles involving 574 adults with ARF due to various causes, such as chest trauma, decompensated heart failure, coronavirus disease 2019 (COVID-19), and postoperative period, among others, were included. The interfaces cited in the studies included an oronasal mask, nasal mask, full-face mask, and helmet. In addition, some favorable outcomes related to NIV were reported in the studies, such as a reduction in the rate of orotracheal intubation and shorter length of stay in the ICU. Conclusions: The most cited interfaces in the treatment of ARF were the oronasal mask and the helmet.展开更多
BACKGROUND:This study aimed to investigate the risk factors and outcome of critically ill cancer patients with postoperative acute respiratory insufficiency.METHODS:The data of 190 critically ill cancer patients with ...BACKGROUND:This study aimed to investigate the risk factors and outcome of critically ill cancer patients with postoperative acute respiratory insufficiency.METHODS:The data of 190 critically ill cancer patients with postoperative acute respiratory insufficiency were retrospectively reviewed.The data of 321 patients with no acute respiratory insufficiency as controls were also collected.Clinical variables of the first 24 hours after admission to intensive care unit were collected,including age,sex,comorbid disease,type of surgery,admission type,presence of shock,presence of acute kidney injury,presence of acute lung injury/acute respiratory distress syndrome,acute physiologic and chronic health evaluation(APACHE Ⅱ) score,sepsis-related organ failure assessment(SOFA),and PaO_2/FiO_2 ratio.Duration of mechanical ventilation,length of intensive care unit stay,intensive care unit death,length of hospitalization,hospital death and one-year survival were calculated.RESULTS:The incidence of acute respiratory insufficiency was 37.2%(190/321).Multivariate logistic analysis showed a history of chronic obstructive pulmonary diseases(P=0.001),surgeryrelated infection(P=0.004),hypo-volemic shock(P<0.001),and emergency surgery(P=0.018),were independent risk factors of postoperative acute respiratory insufficiency.Compared with the patients without acute respiratory insufficiency,the patients with acute respiratory insufficiency had a prolonged length of intensive care unit stay(P<0.001),a prolonged length of hospitalization(P=0.006),increased intensive care unit mortality(P=0.001),and hospital mortality(P<0.001).Septic shock was shown to be the only independent prognostic factor of intensive care unit death for the patients with acute respiratory insufficiency(P=0.029,RR:8.522,95%CI:1.243-58.437,B=2.143,SE=0.982,Wald=4.758).Compared with the patients without acute respiratory insufficiency,those with acute respiratory insufficiency had a shortened one-year survival rate(78.7%vs.97.1%,P<0.001).CONCLUSION:A history of chronic obstructive pulmonary diseases,surgery-related infection,hypovolemic shock and emergency surgery were risk factors of critically ill cancer patients with postoperative acute respiratory insufficiency.Septic shock was the only independent prognostic factor of intensive care unit death in patients with acute respiratory insufficiency.Compared with patients without acute respiratory insufficiency,those with acute respiratory insufficiency had adverse shortterm outcome and a decreased one-year survival rate.展开更多
BACKGROUND Thymolipoma is a rare benign tumor arising from the anterior mediastinal thymus and is composed of mature fatty tissue and interspersed nonneoplastic thymic tissue. This tumor accounts for only a small perc...BACKGROUND Thymolipoma is a rare benign tumor arising from the anterior mediastinal thymus and is composed of mature fatty tissue and interspersed nonneoplastic thymic tissue. This tumor accounts for only a small percentage of mediastinal masses, and the majority of them are asymptomatic and found incidentally. To date, fewer than 200 cases have been published in the world literature, of which most excised tumors weighed less than 0.5 kg and the largest weighed 6 kg.CASE SUMMARY A 23-year-old man presented with a complaint of progressive breathlessness for 6mo. His forced vital capacity was only 23.6% of the predicted capacity, and his arterial partial pressure of oxygen and carbon dioxide were 51 and 60 mmHg,respectively, without oxygen inhalation. Chest computed tomography revealed a large fat-containing mass in the anterior mediastinum that measured 26 cm × 20cm × 30 cm in size and occupied most of the thoracic cavity. Percutaneous mass biopsy revealed only thymic tissue without signs of malignancy. A right posterolateral thoracotomy was successfully performed to remove the tumor along with the capsule, and the excised tumor weighed 7.5 kg, which to our knowledge, was the largest surgically removed tumor of thymic origin. Postoperatively, the patient’s shortness of breath was resolved, and the histopathological diagnosis was thymolipoma. No signs of recurrence were observed at the 6-mo follow-up.CONCLUSION Giant thymolipoma causing respiratory failure is rare and dangerous. Despite the high risks, surgical resection is feasible and effective.展开更多
Rationale:Multi-organ failure is a symptom of paraquat poisoning,resulting in high mortality and morbidity rates.Though paraquat is widely available,poisoning through inhalation is rare.Patient’s Concern:A 37-year-ol...Rationale:Multi-organ failure is a symptom of paraquat poisoning,resulting in high mortality and morbidity rates.Though paraquat is widely available,poisoning through inhalation is rare.Patient’s Concern:A 37-year-old male reported to the emergency department with complaints of vomiting after an alleged history of inhalation of paraquat while at work.Diagnosis:Paraquat poisoning.Interventions:Supportive management along with multiple sessions of hemodialysis.Outcomes:Renal complications caused by paraquat were improved after multiple sessions of hemodialysis.However,the patient developed respiratory complications and later due to persistent hypoxemia and non-responsive to supportive therapy,he succumbed to his illness.Lessons:Acute kidney injury is a complication of paraquat poisoning.However,kidney involvement with the inhalational mode is rare.It is caused by reduction and oxidation cycles,as well as the formation of reactive oxygen species,necessitating hemodialysis as the treatment.Without a clear history,a specific clinical trait,or a diagnostic test,diagnosis can be difficult.Our case thus highlights the inhaled paraquat poisoning,presenting with acute kidney injury with late respiratory impairment as a consequence.展开更多
BACKGROUND Early-onset progressive encephalopathy with brain edema and/or leukoencephalopathy-1(PEBEL1)is a rare autosomal recessive severe neurometabolic disease.The aim of this study was to investigate the clinical ...BACKGROUND Early-onset progressive encephalopathy with brain edema and/or leukoencephalopathy-1(PEBEL1)is a rare autosomal recessive severe neurometabolic disease.The aim of this study was to investigate the clinical characteristics and genetic pathogenicity of PEBEL1 caused by rare NAXE(or APOA1BP)-related defects.CASE SUMMARY The patient was a girl aged 2 years and 10 mo.She was hospitalized due to walking disorder for>40 d.The clinical manifestations were ataxia,motor function regression,hypotonia,and eyelid ptosis.Within 1 mo of hospitalization,she developed sigh breathing,respiratory failure,cerebellar edema and brain hernia,and finally she died.Changes were found in cranial imaging,including cerebellar edema accompanied by symmetrical myelopathy.Through whole exome sequencing,we detected NAXE compound heterozygous variation(NM 144772.3)c.733A>C(p.Lys245Gln,dbSNP:rs770023429)and novel variation c.370G>T(p.Gly124Cys)in the germline gene.The clinical features and core phenotypes of this case were consistent with 18 previously reported cases of PEBEL1.CONCLUSION This is the first case of NAXE-related PEBEL1 with severe clinical phenotype in China' Mainland.The p.Gly124Cys mutation discovered in this case has enriched the pathogenic variation spectrum of NAXE.展开更多
Objective:To evaluate the in-hospital outcome of moderate to severe COVID-19 patients admitted in High Dependency Unit(HDU)in relation to invasive vs.non-invasive mode of ventilation.Methods:In this study,the patients...Objective:To evaluate the in-hospital outcome of moderate to severe COVID-19 patients admitted in High Dependency Unit(HDU)in relation to invasive vs.non-invasive mode of ventilation.Methods:In this study,the patients required either non-invasive[oxygen≤10 L/min or>10 L/min through mask or nasal prongs,rebreather masks and bilevel positive airway pressure(BiPAP)]or invasive ventilation.For analysis of 30-day in hospital mortality in relation to use of different modes of oxygen,Kaplan Meier and log rank analyses were used.In the end,independent predictors of survival were determined by Cox regression analysis.Results:Invasive ventilation was required by 15.1%patients while 84.9%patients needed non-invasive ventilation.Patients with evidence of thromboembolism,high inflammatory markers and hypoxemia mainly required invasive ventilation.The 30-day in hospital mortality was 72.7%for the invasive group and 12.9%for the non-invasive group(1.8%oxygen<10 L/min,0.9%oxygen>10 L/min,3.6%rebreather mask and 4.5%BiPAP).The median time from hospital admission to outcome was 7 days for the invasive group and 18 days for the non-invasive group(P<0.05).Age,presence of co-morbidities,number of days requiring oxygen,rebreather,BiPAP and invasive ventilation were independent predictors of outcome.Conclusions:Invasive mechanical ventilation is associated with adverse outcomes possibly due to ventilator associated lung injury.Thus,protective non-invasive ventilation remains the necessary and safe treatment for severely hypoxic COVID-19 patients.展开更多
BACKGROUND Nafamostat mesylate(NM)may prove to be one of the key drugs effective against coronavirus disease 2019(COVID-19)because of its anti-viral properties and the potential to manage coagulopathy.However,NM tends...BACKGROUND Nafamostat mesylate(NM)may prove to be one of the key drugs effective against coronavirus disease 2019(COVID-19)because of its anti-viral properties and the potential to manage coagulopathy.However,NM tends to increase serum potassium levels.CASE SUMMARY We observed hyperkalemia immediately after NM administration(200 mg/d)in four consecutive patients who were admitted to the Kanazawa University Hospital with severe COVID-19 pneumonia.Urinary potassium excretion decreased after NM administration in three patients who underwent urinalysis.CONCLUSION NM is likely to produce hyperkalemia in patients with COVID-19.Therefore,it is necessary to monitor serum potassium values closely after NM initiation in COVID-19 patients who need respiratory support.展开更多
BACKGROUND Myotonic dystrophy type 1(DM1)is a genetic neuromuscular disease involving multiple systems,especially the cardiopulmonary system.The clinical phenotype of DM1 patients is highly variable,which limits early...BACKGROUND Myotonic dystrophy type 1(DM1)is a genetic neuromuscular disease involving multiple systems,especially the cardiopulmonary system.The clinical phenotype of DM1 patients is highly variable,which limits early diagnosis and treatment.In the present study,we reported a 35-year-old female DM1 patient with dyspnea as the primary onset clinical manifestation,analyzed her family's medical history,and reviewed related literature.CASE SUMMARY A 35-year-old woman was admitted to the hospital with dyspnea of 1 mo duration,and sleep apnea for 3 d.Her respiratory pattern and effort were normal,but limb muscle tension was low.Investigation into the patient's medical history revealed that she might have hereditary neuromuscular disease.Electromyography showed that her myotonia potentials were visible in the resting state of the examined muscles,with decreased motor unit potential time limit and amplitude.Genetic testing for DM1 revealed that the cytosine-thymine-guanine(CTG)repeat number of the DMPK gene exceeded 50,while cytosine-CTG expansion in intron 1 of ZNF9 gene was<30 repeats.The patient was diagnosed with DM1.CONCLUSION DM1 is a genetic neuromuscular disease involving multiple systems,and the clinical phenotype in DM1 is extremely variable.Some patients with DM1 may be presented at the respiratory department because of dyspnea,which should be cautioned by the pulmonologists.There may be no obvious or specific symptoms in the early stage of disease,and clinicians should improve their understanding of DM1 and make an early diagnosis,which will improve patients’quality of life.展开更多
Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should b...Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209 Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209 Leu mutation in BAG3.Totally twentyone patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11:10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen(one could not tolerate non-invasive assisted ventilation)required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients’muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia.展开更多
BACKGROUND Chilaiditi syndrome is a rare disorder characterized by the hepatodiaphragmatic interposition of the intestine.CASE SUMMARY Here we report a case of a 12-year-old male who was admitted to the pediatric inte...BACKGROUND Chilaiditi syndrome is a rare disorder characterized by the hepatodiaphragmatic interposition of the intestine.CASE SUMMARY Here we report a case of a 12-year-old male who was admitted to the pediatric intensive care unit secondary to abdominal pain and severe respiratory distress.He was treated conservatively but the symptoms persisted requiring a surgical approach.While there have been several cases of Chilaiditi syndrome reported in adults,there is a scarcity of cases reported in the pediatric population.Our review of the literature found only 30 pediatric cases,including our reported case,with Chilaiditi syndrome,19(63%)of which were male.The median age of diagnosis was 4.5 years old with an interquartile range of 2.0-10.0 years.In our review,we found that the most common predisposing factors in children are aerophagia(12/30 cases)and constipation(13/30 cases).Ninety percent of the cases presented with complete intestinal interposition,in 100%of which,the colon was involved.Three of the 30 cases were associated with volvulus.CONCLUSION In the pediatric population,conservative(21/30 cases)and surgical(8/30 cases)treatment approaches have produced satisfactory outcomes for all the patients,regardless of approach.展开更多
To study the clinical therapeutic e ffect of anisodamine on respiratory function after severe brain injury. Methods: Ninety patients with respiratory dysfunction following severe brain injury were divided into two gro...To study the clinical therapeutic e ffect of anisodamine on respiratory function after severe brain injury. Methods: Ninety patients with respiratory dysfunction following severe brain injury were divided into two groups: a treatment group (n=45, trea ted with routine therapy plus anisodamine) and a control group (n=45, treated wi th routine therapy only). The pulmonary ventilation function and oxygenation fun ction were compared between the two groups. Results: In the treatment group, 12 hours after treatment the r espiratory rate reduced, the partial pressure of carbon dioxide (PCO 2), the pa rtial pressure of oxygen in arterial blood (PaO 2) and oxygenation exponent inc reased, the dead space ventilation dose and the pulmonary alveolus partial pres sure of arterial oxygen difference decreased, and the ventilation function of th e respiratory tract and pulmonary oxygenation function improved. There was a sig nificant difference between the two groups (P< 0.01 ). N o side effect was found except a slight increase of intracranial pressure and h eart rate. Conclusions: Anisodamine can improve pulmonary ventilation func tion and oxygenation function and decrease the incidence of hypoxemia markedly. It is effective in treating respiratory dysfunction after severe brain injury.展开更多
OBJECTIVE: To compare the influence of cardiac-pulmonary function on clinical acute respiratory failure patients using Proportional assist ventilation (PAV), Pressure support ventilation (PSV) and intermittent positiv...OBJECTIVE: To compare the influence of cardiac-pulmonary function on clinical acute respiratory failure patients using Proportional assist ventilation (PAV), Pressure support ventilation (PSV) and intermittent positive pressure ventilation (IPPV). Here, we also describe some our experience with the clinical use of PAV. METHODS: Using the IPPV mode in ten acute respiratory failure patients, calculate Elastance (Ers) and Resistance (Rrs), then change to PSV, set inspiratory positive airway pressure (IPAP) according to IPPV, so that tidal volume (V(T)) is the same as that of IPPV. We then changed the mode into PAV and set the assist ratio according to PSV, so that V(T) and Ppeak were the same as that of PSV. Then we observed the changes of respiratory mechanics, blood gas levels and hemodynamics during ventilation. RESULTS: Compared with PSV and IPPV, peak pressure (Ppeak) of PAV was markedly lower while V(T) was similar; work of breathing of patient (WOBp), and work of breathing of ventilation (WOBv) were also lower; center vein pressure (CVP) and pulmonary capillary wedge pressure (PCWP) of PAV were markedly lower than that of IPPV while V(T) were similar. Compared with PSV, V(T), mean blood pressure (mBP) and cardiac output (CO) of PAV were higher. Mean pulmonary artery pressure (mPAP) and WOBp of PAV were lower while Ppeak was similar; the differences in WOBp were notable. CONCLUSIONS: For clinical acute respiratory failure patients, compared with PSV and IPPV, PAV has lower airway pressure, less WOBp and less influence on hemodynamics.展开更多
OBJECTIVE: To investigate the impact of proportional assist ventilation (PAV) on tolerance and breathlessness in ventilated chronic obstructive pulmonary disease (COPD), and to describe the patient-ventilator interact...OBJECTIVE: To investigate the impact of proportional assist ventilation (PAV) on tolerance and breathlessness in ventilated chronic obstructive pulmonary disease (COPD), and to describe the patient-ventilator interaction, hemodynamic state, breathing pattern and work of breath during PAV and pressure support ventilation (PSV). METHODS: Ten intubated COPD patients on weaning from mechanical ventilation were studied. Elastance and resistance were measured by both the inspiratory-hold technique during a brief period of volume control ventilation and runaway technique during PAV. Each assistance level of PAV (80%, 60% and 40%) and PSV was selected randomly. Patients' response, hemodynamics, blood gas and lung mechanics were monitored. RESULTS: Tidal volume and respiratory rate didn't change in a consistent manner as the level of assist was decreased (P > 0.05). With the level of assist increasing, peak inspiratory pressure was increasing significantly (P展开更多
Background:The heterogeneity of patients with COVID-19 may explain the wide variation of mortality rate due to the population characteristics,presence of comorbidities and clinical manifestations.Methods:In this study...Background:The heterogeneity of patients with COVID-19 may explain the wide variation of mortality rate due to the population characteristics,presence of comorbidities and clinical manifestations.Methods:In this study,we analyzed 5342 patients’recordings and selected a cohort of 177 hospitalized patients with a poor prognosis at an early stage.We assessed during 6 months their symptomatology,coexisting health conditions,clinical measures and health assistance related to mortality.Multiple Cox proportional hazards models were built to identify the associated factors with mortality risk.Results:We observed that cough and kidney failure triplicate the mortality risk and both bilirubin levels and oncologic condition are shown as the most associated with the demise,increasing in four and ten times the risk,respectively.Other clinical characteristics such as fever,diabetes mellitus,breathing frequency,neutrophil-lymphocyte ratio,oxygen saturation,and troponin levels,were also related to mortality risk of in-hospital death.Conclusions:The present study shows that some symptomatology,comorbidities and clinical measures could be the target of prevention tools to improve survival rates.展开更多
文摘Background: Noninvasive ventilation (NIV) is an important therapeutic modality for the treatment of acute respiratory failure (ARF). In this review, we critically analyze randomized controlled trials on the most used NIV interfaces in the treatments of ARF. Methods: The searches were conducted in the Medline, Lilacs, PubMed, Cochrane, and Pedro databases from June to November 2021. The inclusion criteria were Randomized clinical trials (RCTs) published from 2016 to 2021 in Portuguese, Spanish, or English and involving adults (aged ≥ 18 years). The eligibility criteria for article selection were based on the PICO strategy: Population—Adults with ARF;Intervention—NIV Therapy;Comparison—Conventional oxygen therapy, high-flow nasal cannula (HFNC) oxygen therapy, or NIV;Outcome—improvement in ARF. The search for articles and the implementation of the inclusion criteria were independently conducted by two researchers. Results: Seven scientific articles involving 574 adults with ARF due to various causes, such as chest trauma, decompensated heart failure, coronavirus disease 2019 (COVID-19), and postoperative period, among others, were included. The interfaces cited in the studies included an oronasal mask, nasal mask, full-face mask, and helmet. In addition, some favorable outcomes related to NIV were reported in the studies, such as a reduction in the rate of orotracheal intubation and shorter length of stay in the ICU. Conclusions: The most cited interfaces in the treatment of ARF were the oronasal mask and the helmet.
文摘BACKGROUND:This study aimed to investigate the risk factors and outcome of critically ill cancer patients with postoperative acute respiratory insufficiency.METHODS:The data of 190 critically ill cancer patients with postoperative acute respiratory insufficiency were retrospectively reviewed.The data of 321 patients with no acute respiratory insufficiency as controls were also collected.Clinical variables of the first 24 hours after admission to intensive care unit were collected,including age,sex,comorbid disease,type of surgery,admission type,presence of shock,presence of acute kidney injury,presence of acute lung injury/acute respiratory distress syndrome,acute physiologic and chronic health evaluation(APACHE Ⅱ) score,sepsis-related organ failure assessment(SOFA),and PaO_2/FiO_2 ratio.Duration of mechanical ventilation,length of intensive care unit stay,intensive care unit death,length of hospitalization,hospital death and one-year survival were calculated.RESULTS:The incidence of acute respiratory insufficiency was 37.2%(190/321).Multivariate logistic analysis showed a history of chronic obstructive pulmonary diseases(P=0.001),surgeryrelated infection(P=0.004),hypo-volemic shock(P<0.001),and emergency surgery(P=0.018),were independent risk factors of postoperative acute respiratory insufficiency.Compared with the patients without acute respiratory insufficiency,the patients with acute respiratory insufficiency had a prolonged length of intensive care unit stay(P<0.001),a prolonged length of hospitalization(P=0.006),increased intensive care unit mortality(P=0.001),and hospital mortality(P<0.001).Septic shock was shown to be the only independent prognostic factor of intensive care unit death for the patients with acute respiratory insufficiency(P=0.029,RR:8.522,95%CI:1.243-58.437,B=2.143,SE=0.982,Wald=4.758).Compared with the patients without acute respiratory insufficiency,those with acute respiratory insufficiency had a shortened one-year survival rate(78.7%vs.97.1%,P<0.001).CONCLUSION:A history of chronic obstructive pulmonary diseases,surgery-related infection,hypovolemic shock and emergency surgery were risk factors of critically ill cancer patients with postoperative acute respiratory insufficiency.Septic shock was the only independent prognostic factor of intensive care unit death in patients with acute respiratory insufficiency.Compared with patients without acute respiratory insufficiency,those with acute respiratory insufficiency had adverse shortterm outcome and a decreased one-year survival rate.
基金Supported by Hunan Provincial Natural Science Foundation of China,No. 2022JJ40246Hunan Cancer Hospital Climb Plan,No. 2021NSFC-B005。
文摘BACKGROUND Thymolipoma is a rare benign tumor arising from the anterior mediastinal thymus and is composed of mature fatty tissue and interspersed nonneoplastic thymic tissue. This tumor accounts for only a small percentage of mediastinal masses, and the majority of them are asymptomatic and found incidentally. To date, fewer than 200 cases have been published in the world literature, of which most excised tumors weighed less than 0.5 kg and the largest weighed 6 kg.CASE SUMMARY A 23-year-old man presented with a complaint of progressive breathlessness for 6mo. His forced vital capacity was only 23.6% of the predicted capacity, and his arterial partial pressure of oxygen and carbon dioxide were 51 and 60 mmHg,respectively, without oxygen inhalation. Chest computed tomography revealed a large fat-containing mass in the anterior mediastinum that measured 26 cm × 20cm × 30 cm in size and occupied most of the thoracic cavity. Percutaneous mass biopsy revealed only thymic tissue without signs of malignancy. A right posterolateral thoracotomy was successfully performed to remove the tumor along with the capsule, and the excised tumor weighed 7.5 kg, which to our knowledge, was the largest surgically removed tumor of thymic origin. Postoperatively, the patient’s shortness of breath was resolved, and the histopathological diagnosis was thymolipoma. No signs of recurrence were observed at the 6-mo follow-up.CONCLUSION Giant thymolipoma causing respiratory failure is rare and dangerous. Despite the high risks, surgical resection is feasible and effective.
文摘Rationale:Multi-organ failure is a symptom of paraquat poisoning,resulting in high mortality and morbidity rates.Though paraquat is widely available,poisoning through inhalation is rare.Patient’s Concern:A 37-year-old male reported to the emergency department with complaints of vomiting after an alleged history of inhalation of paraquat while at work.Diagnosis:Paraquat poisoning.Interventions:Supportive management along with multiple sessions of hemodialysis.Outcomes:Renal complications caused by paraquat were improved after multiple sessions of hemodialysis.However,the patient developed respiratory complications and later due to persistent hypoxemia and non-responsive to supportive therapy,he succumbed to his illness.Lessons:Acute kidney injury is a complication of paraquat poisoning.However,kidney involvement with the inhalational mode is rare.It is caused by reduction and oxidation cycles,as well as the formation of reactive oxygen species,necessitating hemodialysis as the treatment.Without a clear history,a specific clinical trait,or a diagnostic test,diagnosis can be difficult.Our case thus highlights the inhaled paraquat poisoning,presenting with acute kidney injury with late respiratory impairment as a consequence.
基金Supported by the Epilepsy Research Fund of Chinese Anti-Epilepsy Association,No.CU-A-2021-17Nanjing Municipal Health Bureau key project,No.ZKX21047the Postdoctoral Research Foundation of China,No.2020M671550。
文摘BACKGROUND Early-onset progressive encephalopathy with brain edema and/or leukoencephalopathy-1(PEBEL1)is a rare autosomal recessive severe neurometabolic disease.The aim of this study was to investigate the clinical characteristics and genetic pathogenicity of PEBEL1 caused by rare NAXE(or APOA1BP)-related defects.CASE SUMMARY The patient was a girl aged 2 years and 10 mo.She was hospitalized due to walking disorder for>40 d.The clinical manifestations were ataxia,motor function regression,hypotonia,and eyelid ptosis.Within 1 mo of hospitalization,she developed sigh breathing,respiratory failure,cerebellar edema and brain hernia,and finally she died.Changes were found in cranial imaging,including cerebellar edema accompanied by symmetrical myelopathy.Through whole exome sequencing,we detected NAXE compound heterozygous variation(NM 144772.3)c.733A>C(p.Lys245Gln,dbSNP:rs770023429)and novel variation c.370G>T(p.Gly124Cys)in the germline gene.The clinical features and core phenotypes of this case were consistent with 18 previously reported cases of PEBEL1.CONCLUSION This is the first case of NAXE-related PEBEL1 with severe clinical phenotype in China' Mainland.The p.Gly124Cys mutation discovered in this case has enriched the pathogenic variation spectrum of NAXE.
文摘Objective:To evaluate the in-hospital outcome of moderate to severe COVID-19 patients admitted in High Dependency Unit(HDU)in relation to invasive vs.non-invasive mode of ventilation.Methods:In this study,the patients required either non-invasive[oxygen≤10 L/min or>10 L/min through mask or nasal prongs,rebreather masks and bilevel positive airway pressure(BiPAP)]or invasive ventilation.For analysis of 30-day in hospital mortality in relation to use of different modes of oxygen,Kaplan Meier and log rank analyses were used.In the end,independent predictors of survival were determined by Cox regression analysis.Results:Invasive ventilation was required by 15.1%patients while 84.9%patients needed non-invasive ventilation.Patients with evidence of thromboembolism,high inflammatory markers and hypoxemia mainly required invasive ventilation.The 30-day in hospital mortality was 72.7%for the invasive group and 12.9%for the non-invasive group(1.8%oxygen<10 L/min,0.9%oxygen>10 L/min,3.6%rebreather mask and 4.5%BiPAP).The median time from hospital admission to outcome was 7 days for the invasive group and 18 days for the non-invasive group(P<0.05).Age,presence of co-morbidities,number of days requiring oxygen,rebreather,BiPAP and invasive ventilation were independent predictors of outcome.Conclusions:Invasive mechanical ventilation is associated with adverse outcomes possibly due to ventilator associated lung injury.Thus,protective non-invasive ventilation remains the necessary and safe treatment for severely hypoxic COVID-19 patients.
文摘BACKGROUND Nafamostat mesylate(NM)may prove to be one of the key drugs effective against coronavirus disease 2019(COVID-19)because of its anti-viral properties and the potential to manage coagulopathy.However,NM tends to increase serum potassium levels.CASE SUMMARY We observed hyperkalemia immediately after NM administration(200 mg/d)in four consecutive patients who were admitted to the Kanazawa University Hospital with severe COVID-19 pneumonia.Urinary potassium excretion decreased after NM administration in three patients who underwent urinalysis.CONCLUSION NM is likely to produce hyperkalemia in patients with COVID-19.Therefore,it is necessary to monitor serum potassium values closely after NM initiation in COVID-19 patients who need respiratory support.
文摘BACKGROUND Myotonic dystrophy type 1(DM1)is a genetic neuromuscular disease involving multiple systems,especially the cardiopulmonary system.The clinical phenotype of DM1 patients is highly variable,which limits early diagnosis and treatment.In the present study,we reported a 35-year-old female DM1 patient with dyspnea as the primary onset clinical manifestation,analyzed her family's medical history,and reviewed related literature.CASE SUMMARY A 35-year-old woman was admitted to the hospital with dyspnea of 1 mo duration,and sleep apnea for 3 d.Her respiratory pattern and effort were normal,but limb muscle tension was low.Investigation into the patient's medical history revealed that she might have hereditary neuromuscular disease.Electromyography showed that her myotonia potentials were visible in the resting state of the examined muscles,with decreased motor unit potential time limit and amplitude.Genetic testing for DM1 revealed that the cytosine-thymine-guanine(CTG)repeat number of the DMPK gene exceeded 50,while cytosine-CTG expansion in intron 1 of ZNF9 gene was<30 repeats.The patient was diagnosed with DM1.CONCLUSION DM1 is a genetic neuromuscular disease involving multiple systems,and the clinical phenotype in DM1 is extremely variable.Some patients with DM1 may be presented at the respiratory department because of dyspnea,which should be cautioned by the pulmonologists.There may be no obvious or specific symptoms in the early stage of disease,and clinicians should improve their understanding of DM1 and make an early diagnosis,which will improve patients’quality of life.
基金supported by the Youth Program of National Natural Science Foundation of China(Grant No.82003309)the National Key Research and Development Program of China(Grant 2020YFB1313700)。
文摘Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209 Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209 Leu mutation in BAG3.Totally twentyone patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11:10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen(one could not tolerate non-invasive assisted ventilation)required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients’muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia.
文摘BACKGROUND Chilaiditi syndrome is a rare disorder characterized by the hepatodiaphragmatic interposition of the intestine.CASE SUMMARY Here we report a case of a 12-year-old male who was admitted to the pediatric intensive care unit secondary to abdominal pain and severe respiratory distress.He was treated conservatively but the symptoms persisted requiring a surgical approach.While there have been several cases of Chilaiditi syndrome reported in adults,there is a scarcity of cases reported in the pediatric population.Our review of the literature found only 30 pediatric cases,including our reported case,with Chilaiditi syndrome,19(63%)of which were male.The median age of diagnosis was 4.5 years old with an interquartile range of 2.0-10.0 years.In our review,we found that the most common predisposing factors in children are aerophagia(12/30 cases)and constipation(13/30 cases).Ninety percent of the cases presented with complete intestinal interposition,in 100%of which,the colon was involved.Three of the 30 cases were associated with volvulus.CONCLUSION In the pediatric population,conservative(21/30 cases)and surgical(8/30 cases)treatment approaches have produced satisfactory outcomes for all the patients,regardless of approach.
文摘To study the clinical therapeutic e ffect of anisodamine on respiratory function after severe brain injury. Methods: Ninety patients with respiratory dysfunction following severe brain injury were divided into two groups: a treatment group (n=45, trea ted with routine therapy plus anisodamine) and a control group (n=45, treated wi th routine therapy only). The pulmonary ventilation function and oxygenation fun ction were compared between the two groups. Results: In the treatment group, 12 hours after treatment the r espiratory rate reduced, the partial pressure of carbon dioxide (PCO 2), the pa rtial pressure of oxygen in arterial blood (PaO 2) and oxygenation exponent inc reased, the dead space ventilation dose and the pulmonary alveolus partial pres sure of arterial oxygen difference decreased, and the ventilation function of th e respiratory tract and pulmonary oxygenation function improved. There was a sig nificant difference between the two groups (P< 0.01 ). N o side effect was found except a slight increase of intracranial pressure and h eart rate. Conclusions: Anisodamine can improve pulmonary ventilation func tion and oxygenation function and decrease the incidence of hypoxemia markedly. It is effective in treating respiratory dysfunction after severe brain injury.
文摘OBJECTIVE: To compare the influence of cardiac-pulmonary function on clinical acute respiratory failure patients using Proportional assist ventilation (PAV), Pressure support ventilation (PSV) and intermittent positive pressure ventilation (IPPV). Here, we also describe some our experience with the clinical use of PAV. METHODS: Using the IPPV mode in ten acute respiratory failure patients, calculate Elastance (Ers) and Resistance (Rrs), then change to PSV, set inspiratory positive airway pressure (IPAP) according to IPPV, so that tidal volume (V(T)) is the same as that of IPPV. We then changed the mode into PAV and set the assist ratio according to PSV, so that V(T) and Ppeak were the same as that of PSV. Then we observed the changes of respiratory mechanics, blood gas levels and hemodynamics during ventilation. RESULTS: Compared with PSV and IPPV, peak pressure (Ppeak) of PAV was markedly lower while V(T) was similar; work of breathing of patient (WOBp), and work of breathing of ventilation (WOBv) were also lower; center vein pressure (CVP) and pulmonary capillary wedge pressure (PCWP) of PAV were markedly lower than that of IPPV while V(T) were similar. Compared with PSV, V(T), mean blood pressure (mBP) and cardiac output (CO) of PAV were higher. Mean pulmonary artery pressure (mPAP) and WOBp of PAV were lower while Ppeak was similar; the differences in WOBp were notable. CONCLUSIONS: For clinical acute respiratory failure patients, compared with PSV and IPPV, PAV has lower airway pressure, less WOBp and less influence on hemodynamics.
文摘OBJECTIVE: To investigate the impact of proportional assist ventilation (PAV) on tolerance and breathlessness in ventilated chronic obstructive pulmonary disease (COPD), and to describe the patient-ventilator interaction, hemodynamic state, breathing pattern and work of breath during PAV and pressure support ventilation (PSV). METHODS: Ten intubated COPD patients on weaning from mechanical ventilation were studied. Elastance and resistance were measured by both the inspiratory-hold technique during a brief period of volume control ventilation and runaway technique during PAV. Each assistance level of PAV (80%, 60% and 40%) and PSV was selected randomly. Patients' response, hemodynamics, blood gas and lung mechanics were monitored. RESULTS: Tidal volume and respiratory rate didn't change in a consistent manner as the level of assist was decreased (P > 0.05). With the level of assist increasing, peak inspiratory pressure was increasing significantly (P
基金supported by the Innovation,Universities,Science and Digital Society Council through the Valencia Innovation Agency(AVI)grant 851255 from the European Research Council under the European Union’s Horizon 2020 research and innovation programfrom the Universitat de València.
文摘Background:The heterogeneity of patients with COVID-19 may explain the wide variation of mortality rate due to the population characteristics,presence of comorbidities and clinical manifestations.Methods:In this study,we analyzed 5342 patients’recordings and selected a cohort of 177 hospitalized patients with a poor prognosis at an early stage.We assessed during 6 months their symptomatology,coexisting health conditions,clinical measures and health assistance related to mortality.Multiple Cox proportional hazards models were built to identify the associated factors with mortality risk.Results:We observed that cough and kidney failure triplicate the mortality risk and both bilirubin levels and oncologic condition are shown as the most associated with the demise,increasing in four and ten times the risk,respectively.Other clinical characteristics such as fever,diabetes mellitus,breathing frequency,neutrophil-lymphocyte ratio,oxygen saturation,and troponin levels,were also related to mortality risk of in-hospital death.Conclusions:The present study shows that some symptomatology,comorbidities and clinical measures could be the target of prevention tools to improve survival rates.