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: The present study aimed to determine the short-term and long-term outcomes of critically ill patients with acute respiratory insuffi ciency who had received sedation or no sedation.METHODS: The data of 91 ...BACKGROUND: The present study aimed to determine the short-term and long-term outcomes of critically ill patients with acute respiratory insuffi ciency who had received sedation or no sedation.METHODS: The data of 91 patients who had received mechanical ventilation in the first 24 hours between November 2008 and October 2009 were retrospectively analyzed. These patients were divided into two groups: a sedation group(n=28) and a non-sedation group(n=63). The patients were also grouped in two groups: deep sedation group and daily interruption and /or light sedation group.RESULTS: Overall, the 91 patients who had received ventilation ≥48 hours were analyzed. Multivariate analysis demonstrated two independent risk factors for in-hospital death: sequential organ failure assessment score(P=0.019, RR 1.355, 95%CI 1.051–1.747, B=0.304, SE=0.130, Wald=50483) and sedation(P=0.041, RR 5.015, 95%CI 1.072–23.459, B=1.612, SE=0.787, Wald=4.195). Compared with the patients who had received no sedation, those who had received sedation had a longer duration of ventilation, a longer stay in intensive care unit and hospital, and an increased in-hospital mortality rate. The Kaplan-Meier method showed that patients who had received sedation had a lower 60-month survival rate than those who had received no sedation(76.7% vs. 88.9%, Log-rank test=3.630, P=0.057). Compared with the patients who had received deep sedation, those who had received daily interruption or light sedation showed a decreased in-hospital mortality rate(57.1% vs. 9.5%, P=0.008). The 60-month survival of the patients who had received deep sedation was signifi cantly lower than that of those who had daily interruption or light sedation(38.1% vs. 90.5%, Log-rank test=6.783, P=0.009).CONCLUSIONS: Sedation was associated with in-hospital death. The patients who had received sedation had a longer duration of ventilation, a longer stay in intensive care unit and in hospital, and an increased in-hospital mortality rate compared with the patients who did not receive sedation. Compared with daily interruption or light sedation, deep sedation increased the in-hospital mortality and decreased the 60-month survival for patients who had received sedation.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
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
背景新生儿急性肾损伤(AKI)早期诊断困难,病死率高,而目前关于重度窒息并发新生儿AKI的相关研究相对缺乏。目的探讨新生儿重度窒息并发AKI的危险因素及近期预后情况,并分析相关因素的预测价值,以采取措施减少AKI的发生,并提高此类患儿...背景新生儿急性肾损伤(AKI)早期诊断困难,病死率高,而目前关于重度窒息并发新生儿AKI的相关研究相对缺乏。目的探讨新生儿重度窒息并发AKI的危险因素及近期预后情况,并分析相关因素的预测价值,以采取措施减少AKI的发生,并提高此类患儿的抢救成功率。方法纳入2016年1月—2023年1月于蚌埠医学院第一附属医院新生儿重症监护病房住院治疗的172例重度窒息新生儿为研究对象,根据患儿是否并发AKI,分为AKI组(n=43)和非AKI组(n=129)。收集患儿临床资料和实验室检查结果,并记录AKI患儿的近期预后情况(住院期间存活或死亡)。采用多因素Logistic回归分析探究新生儿重度窒息并发AKI的影响因素,采用受试者工作特征(ROC)曲线探究相关指标对新生儿重度窒息并发AKI的预测价值。结果AKI组患儿胎龄、出生体质量、5 min Apgar评分、血小板计数低于非AKI组,昏迷状态比例、有创机械通气比例、合并呼吸衰竭比例、血胱抑素C(Cys C)高于非AKI组(P<0.05)。多因素Logistic回归分析结果显示,5 min Apgar评分(OR=1.553,95%CI=1.193~2.021,P=0.001)、有创机械通气(OR=2.965,95%CI=1.021~8.611,P=0.046)、血Cys C值(OR=0.231,95%CI=0.109~0.487,P<0.001)是新生儿重度窒息并发AKI的影响因素。ROC曲线分析结果显示血Cys C预测AKI的ROC曲线下面积(AUC)为0.777(95%CI=0.701~0.854,P<0.05),5 min Apgar评分预测AKI的AUC为0.792(95%CI=0.715~0.869,P<0.05)。AKI组患儿住院病死率为51.2%(22/43),非AKI组患儿住院病死率为21.7%(28/129),AKI组患儿病死率高于非AKI组(χ^(2)=13.572,P<0.001)。结论低5 min Apgar评分、有创机械通气、出生后高血Cys C会增加重度窒息新生儿发生AKI的风险。5 min Apgar评分、出生后血Cys C是预测新生儿重度窒息并发AKI的可靠指标。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
To the Editor:Late-onset multiple acyl-CoA dehydrogenase deficiency(MADD)is clinically characterized by a fluctuating or progressive proximal myopathy,exercise intolerance but good responsive to riboflavin.ETFDH mutat...To the Editor:Late-onset multiple acyl-CoA dehydrogenase deficiency(MADD)is clinically characterized by a fluctuating or progressive proximal myopathy,exercise intolerance but good responsive to riboflavin.ETFDH mutations are a major cause of late-onset MADD.We analyzed the clinical course,biochemical studies,and muscle magnetic resonance imaging(MRI)and pathologies of two late-onset MADD adult male patients who were misdiagnosed as polymyositis and presented with serious clinical symptoms of rhabdomyolysis and respiratory insufficient after using large dosage of intravenous glucocorticoids.Our current report broadens the clinical phenotypes spectrum of MADD and reminds clinicians to be cautious about using large dosage glucocorticoids in metabolic compromised patients.展开更多
Severe acute respiratory syndrome coronavirus-2 and the related coronavirus disease-19(COVID-19)is a worldwide emerging situation,which was initially reported in December 2019 in Wuhan,China.Currently,more than 725884...Severe acute respiratory syndrome coronavirus-2 and the related coronavirus disease-19(COVID-19)is a worldwide emerging situation,which was initially reported in December 2019 in Wuhan,China.Currently,more than 7258842 new cases,and more than 411879 deaths have been reported globally.This new highly transmitted coronavirus is responsible for the development of severe acute respiratory distress syndrome.Due to this disorder,a great number of patients are hospitalized in the intensive care unit followed by connection to extracorporeal membrane oxygenation for breath supporting and survival.Severe acute respiratory distress syndrome is mostly accompanied by the secretion of proinflammatory cytokines,including interleukin(IL)-2,IL-6,IL-7,granulocyte colony-stimulating factor(GSCF),interferon-inducible protein 10(IP10),monocyte chemotactic protein-1(MCP1),macrophage inflammatory protein 1A(MIP1A),and tumor necrosis factor alpha(TNF-α),an event which is known as“cytokine storm”.Further disease pathology involves a generalized modulation of immune responses,leading to fatal multiorgan failure.Currently,no specific treatment or vaccination against severe acute respiratory syndrome coronavirus-2(SARS-CoV-2)has been developed.Mesenchymal stromal cells(MSCs),which are known for their immunosuppressive actions,could be applied as an alternative co-therapy in critically-ill COVID-19 patients.Specifically,MSCs can regulate the immune responses through the conversion of Th1 to Th2,activation of M2 macrophages,and modulation of dendritic cells maturation.These key immunoregulatory properties of MSCs may be exerted either by produced soluble factors or by cell-cell contact interactions.To date,several clinical trials have been registered to assess the safety,efficacy,and therapeutic potential of MSCs in COVID-19.Moreover,MSC treatment may be effective for the reversion of ground-glass opacity of damaged lungs and reduce the tissue fibrosis.Taking into account the multifunctional properties of MSCs,the proposed stem-cell-based therapy may be proven significantly effective in critically-ill COVID-19 patients.The current therapeutic strategy may improve the patient’s overall condition and in parallel may decrease the mortality rate of the current disease.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
Arterial blood gases,intraerythrocytic pH (pHi),2,3-diphosphoglycerate,standardP 50 (P 50std) and in vivo P 50 (P 50iv) were determined in 54 patients with cor pulmonale and23 normal subjects.It was found that no sign...Arterial blood gases,intraerythrocytic pH (pHi),2,3-diphosphoglycerate,standardP 50 (P 50std) and in vivo P 50 (P 50iv) were determined in 54 patients with cor pulmonale and23 normal subjects.It was found that no significant change of pHi was observed but the differ-ence of pHi and extraerythrocytic pH was decreased.P 50std was significantly decreased whileP 50iv remained essentially unchanged in those cases showing type I respiratory failure.Thesefindings suggest that P 50std cannot accurately reflect the changes of P 50iv in patients of corpulmonale;P 50iv in cases of cor pulmonale usually keeps relatively stable and coincides withthe ‘optimal P 50’,which indicates the adaptation of the organism to chronic hypoxia;and rel-ative intraerythrocytic alkalosis plays an important role in the relief of hypercapnia in cases ofcor pulmonale.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.
文摘BACKGROUND: The present study aimed to determine the short-term and long-term outcomes of critically ill patients with acute respiratory insuffi ciency who had received sedation or no sedation.METHODS: The data of 91 patients who had received mechanical ventilation in the first 24 hours between November 2008 and October 2009 were retrospectively analyzed. These patients were divided into two groups: a sedation group(n=28) and a non-sedation group(n=63). The patients were also grouped in two groups: deep sedation group and daily interruption and /or light sedation group.RESULTS: Overall, the 91 patients who had received ventilation ≥48 hours were analyzed. Multivariate analysis demonstrated two independent risk factors for in-hospital death: sequential organ failure assessment score(P=0.019, RR 1.355, 95%CI 1.051–1.747, B=0.304, SE=0.130, Wald=50483) and sedation(P=0.041, RR 5.015, 95%CI 1.072–23.459, B=1.612, SE=0.787, Wald=4.195). Compared with the patients who had received no sedation, those who had received sedation had a longer duration of ventilation, a longer stay in intensive care unit and hospital, and an increased in-hospital mortality rate. The Kaplan-Meier method showed that patients who had received sedation had a lower 60-month survival rate than those who had received no sedation(76.7% vs. 88.9%, Log-rank test=3.630, P=0.057). Compared with the patients who had received deep sedation, those who had received daily interruption or light sedation showed a decreased in-hospital mortality rate(57.1% vs. 9.5%, P=0.008). The 60-month survival of the patients who had received deep sedation was signifi cantly lower than that of those who had daily interruption or light sedation(38.1% vs. 90.5%, Log-rank test=6.783, P=0.009).CONCLUSIONS: Sedation was associated with in-hospital death. The patients who had received sedation had a longer duration of ventilation, a longer stay in intensive care unit and in hospital, and an increased in-hospital mortality rate compared with the patients who did not receive sedation. Compared with daily interruption or light sedation, deep sedation increased the in-hospital mortality and decreased the 60-month survival for patients who had received sedation.
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.
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.
文摘背景新生儿急性肾损伤(AKI)早期诊断困难,病死率高,而目前关于重度窒息并发新生儿AKI的相关研究相对缺乏。目的探讨新生儿重度窒息并发AKI的危险因素及近期预后情况,并分析相关因素的预测价值,以采取措施减少AKI的发生,并提高此类患儿的抢救成功率。方法纳入2016年1月—2023年1月于蚌埠医学院第一附属医院新生儿重症监护病房住院治疗的172例重度窒息新生儿为研究对象,根据患儿是否并发AKI,分为AKI组(n=43)和非AKI组(n=129)。收集患儿临床资料和实验室检查结果,并记录AKI患儿的近期预后情况(住院期间存活或死亡)。采用多因素Logistic回归分析探究新生儿重度窒息并发AKI的影响因素,采用受试者工作特征(ROC)曲线探究相关指标对新生儿重度窒息并发AKI的预测价值。结果AKI组患儿胎龄、出生体质量、5 min Apgar评分、血小板计数低于非AKI组,昏迷状态比例、有创机械通气比例、合并呼吸衰竭比例、血胱抑素C(Cys C)高于非AKI组(P<0.05)。多因素Logistic回归分析结果显示,5 min Apgar评分(OR=1.553,95%CI=1.193~2.021,P=0.001)、有创机械通气(OR=2.965,95%CI=1.021~8.611,P=0.046)、血Cys C值(OR=0.231,95%CI=0.109~0.487,P<0.001)是新生儿重度窒息并发AKI的影响因素。ROC曲线分析结果显示血Cys C预测AKI的ROC曲线下面积(AUC)为0.777(95%CI=0.701~0.854,P<0.05),5 min Apgar评分预测AKI的AUC为0.792(95%CI=0.715~0.869,P<0.05)。AKI组患儿住院病死率为51.2%(22/43),非AKI组患儿住院病死率为21.7%(28/129),AKI组患儿病死率高于非AKI组(χ^(2)=13.572,P<0.001)。结论低5 min Apgar评分、有创机械通气、出生后高血Cys C会增加重度窒息新生儿发生AKI的风险。5 min Apgar评分、出生后血Cys C是预测新生儿重度窒息并发AKI的可靠指标。
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grants from National Natural Science Foundation of China(U1505222,81271254,Beijing).
文摘To the Editor:Late-onset multiple acyl-CoA dehydrogenase deficiency(MADD)is clinically characterized by a fluctuating or progressive proximal myopathy,exercise intolerance but good responsive to riboflavin.ETFDH mutations are a major cause of late-onset MADD.We analyzed the clinical course,biochemical studies,and muscle magnetic resonance imaging(MRI)and pathologies of two late-onset MADD adult male patients who were misdiagnosed as polymyositis and presented with serious clinical symptoms of rhabdomyolysis and respiratory insufficient after using large dosage of intravenous glucocorticoids.Our current report broadens the clinical phenotypes spectrum of MADD and reminds clinicians to be cautious about using large dosage glucocorticoids in metabolic compromised patients.
文摘Severe acute respiratory syndrome coronavirus-2 and the related coronavirus disease-19(COVID-19)is a worldwide emerging situation,which was initially reported in December 2019 in Wuhan,China.Currently,more than 7258842 new cases,and more than 411879 deaths have been reported globally.This new highly transmitted coronavirus is responsible for the development of severe acute respiratory distress syndrome.Due to this disorder,a great number of patients are hospitalized in the intensive care unit followed by connection to extracorporeal membrane oxygenation for breath supporting and survival.Severe acute respiratory distress syndrome is mostly accompanied by the secretion of proinflammatory cytokines,including interleukin(IL)-2,IL-6,IL-7,granulocyte colony-stimulating factor(GSCF),interferon-inducible protein 10(IP10),monocyte chemotactic protein-1(MCP1),macrophage inflammatory protein 1A(MIP1A),and tumor necrosis factor alpha(TNF-α),an event which is known as“cytokine storm”.Further disease pathology involves a generalized modulation of immune responses,leading to fatal multiorgan failure.Currently,no specific treatment or vaccination against severe acute respiratory syndrome coronavirus-2(SARS-CoV-2)has been developed.Mesenchymal stromal cells(MSCs),which are known for their immunosuppressive actions,could be applied as an alternative co-therapy in critically-ill COVID-19 patients.Specifically,MSCs can regulate the immune responses through the conversion of Th1 to Th2,activation of M2 macrophages,and modulation of dendritic cells maturation.These key immunoregulatory properties of MSCs may be exerted either by produced soluble factors or by cell-cell contact interactions.To date,several clinical trials have been registered to assess the safety,efficacy,and therapeutic potential of MSCs in COVID-19.Moreover,MSC treatment may be effective for the reversion of ground-glass opacity of damaged lungs and reduce the tissue fibrosis.Taking into account the multifunctional properties of MSCs,the proposed stem-cell-based therapy may be proven significantly effective in critically-ill COVID-19 patients.The current therapeutic strategy may improve the patient’s overall condition and in parallel may decrease the mortality rate of the current disease.
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.
文摘Arterial blood gases,intraerythrocytic pH (pHi),2,3-diphosphoglycerate,standardP 50 (P 50std) and in vivo P 50 (P 50iv) were determined in 54 patients with cor pulmonale and23 normal subjects.It was found that no significant change of pHi was observed but the differ-ence of pHi and extraerythrocytic pH was decreased.P 50std was significantly decreased whileP 50iv remained essentially unchanged in those cases showing type I respiratory failure.Thesefindings suggest that P 50std cannot accurately reflect the changes of P 50iv in patients of corpulmonale;P 50iv in cases of cor pulmonale usually keeps relatively stable and coincides withthe ‘optimal P 50’,which indicates the adaptation of the organism to chronic hypoxia;and rel-ative intraerythrocytic alkalosis plays an important role in the relief of hypercapnia in cases ofcor pulmonale.
