Objective:To explore the association between the Lung Immune Prognostic Index(LIPI)and 1-year all-cause mortality in patients with idiopathic inflammatory myopathy related interstitial lung disease(IIM-ILD).Methods:Pa...Objective:To explore the association between the Lung Immune Prognostic Index(LIPI)and 1-year all-cause mortality in patients with idiopathic inflammatory myopathy related interstitial lung disease(IIM-ILD).Methods:Patients who were diagnosed with IIM-ILD at West China Hospital,Sichuan University from January 2008 to December 2021 were retrospectively included and categorized into three groups based on LIPI.Univariable and multivariable Cox proportional hazards models were conducted to explore potential association between the LIPI and patients'mortality.Results:A total of 1116 patients were screened,and 830 were included in this study.The multivariable Cox analysis showed that,compared with patients with poor LIPI,the hazard ratio(HR)for all-cause 1-year mortality was 0.22(95%CI 0.05-0.93,P=0.04)for patients in the good LIPI group(LDH<250 IU/L and dNLR<3).After excluding patients lost to follow-up within one year,a similar result was found for LIPI(HR 0.20,95%CI 0.05-0.86;P=0.03).Conclusions:Good LIPI was independently associated with decreased risk of all-cause 1-year mortality in patients with IIM-ILD.This easy-to-obtain index might be served as a potential marker for assessing the prognosis of IIM-ILD.展开更多
BACKGROUND Rhabdomyolysis is a life-threatening condition,often leading to progressive renal failure and death.It is caused by destruction of skeletal muscle and the release of myoglobin and other intracellular conten...BACKGROUND Rhabdomyolysis is a life-threatening condition,often leading to progressive renal failure and death.It is caused by destruction of skeletal muscle and the release of myoglobin and other intracellular contents into the circulation.The most frequent cause of this condition is“crush syndrome”,although several others have been described and paraneoplastic inflammatory myopathies associated with various types of cancer are repeatedly reported.CASE SUMMARY We describe a rare case of a patient with pancreatic cancer who developed rhabdomyolysis early on,possibly due to paraneoplastic myositis leading to acute renal failure and eventually to rapid death.A 78-year-old Caucasian woman was referred to our hospital for obstructive jaundice and weight loss due to a lesion in the pancreatic head.She presented increasingly severe renal insufficiency with anuria,a dramatic increase in creatine phosphokinase(36000 U/L,n.v.20-180 U/L)and myoglobin(>120000μg/L,n.v.12-70μg/L).On clinical examination,the patient showed increasing pain in the lower limbs associated with muscle weakness which was severe enough to immobilize her.Paraneoplastic myopathy linked to the malignant lesion of the pancreatic head was suspected.The patient was treated with hemodialysis and intravenous methylprednisolone.Despite all the efforts to prepare the patient for surgery,her general condition rapidly deteriorated and she eventually died 30 d after hospital admission.CONCLUSION The possible causes of rhabdomyolysis in this patient with pancreatic cancer are discussed,the development of paraneoplastic myopathy being the most likely.Clinicians should bear in mind that these syndromes may become clinically manifest at any stage of the cancer course and their early diagnosis and treatment could improve the patient’s prognosis.展开更多
BACKGROUND Mitochondrial myopathy is a rare genetic disease with maternal inheritance that may involve multiple organ systems.Due to the lack of typical characteristics,its clinical diagnosis is difficult,and it is of...BACKGROUND Mitochondrial myopathy is a rare genetic disease with maternal inheritance that may involve multiple organ systems.Due to the lack of typical characteristics,its clinical diagnosis is difficult,and it is often misdiagnosed or even missed.CASE SUMMARY The patient was a young college student.When he presented at the hospital,he had severe lactic acidosis,respiratory failure,and shock with multiple organ dysfunction syndrome(MODS).He was treated by mechanical ventilation,venoarterial extracorporeal membrane oxygenation,and other organ support.However,his condition continued to worsen.After a thorough and detailed medical and family history was taken,a mitochondrial crisis was suspected.A muscle biopsy was taken.Further genetic testing confirmed a mitochondrial gene mutation(TRNL13243A>G).The final diagnosis of mitochondrial myopathy was made.Although there is no known specific treatment,intravenous methylprednisone and intravenous immunoglobulin were started.The patient’s shock eventually improved.The further course was complicated by severe infection in multiple sites,severe muscle weakness,and recurrent MODS.After 2 mo of multidisciplinary management and intensive rehabilitation,the patient could walk with assistance 4 mo after admission and walk independently 6 mo after admission.CONCLUSION More attention should be paid to mitochondrial myopathy to avoid missed diagnosis and misdiagnosis.展开更多
BACKGROUND Immune-mediated necrotizing myopathy is a rare autoimmune myopathy characterized by muscle weakness and elevated serum creatine kinase,with unique skeletal muscle pathology and magnetic resonance imaging fe...BACKGROUND Immune-mediated necrotizing myopathy is a rare autoimmune myopathy characterized by muscle weakness and elevated serum creatine kinase,with unique skeletal muscle pathology and magnetic resonance imaging features.CASE SUMMARY In this paper,two patients are reported:One was positive for anti-signal recognition particle antibody,and the other was positive for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibody.CONCLUSION The clinical characteristics and treatment of the two patients were analysed,and the literature was reviewed to improve the recognition,diagnosis,and treatment of this disease.展开更多
Intensive care unit-acquired weakness(ICU-AW;ICD-10 Code:G72.81)is a syndrome of generalized weakness described as clinically detectable weakness in critically ill patients with no other credible cause.The risk factor...Intensive care unit-acquired weakness(ICU-AW;ICD-10 Code:G72.81)is a syndrome of generalized weakness described as clinically detectable weakness in critically ill patients with no other credible cause.The risk factors for ICU-AW include hyperglycemia,parenteral nutrition,vasoactive drugs,neuromuscular blocking agents,corticosteroids,sedatives,some antibiotics,immobilization,the disease severity,septicemia and systemic inflammatory response syndrome,multiorgan failure,prolonged mechanical ventilation(MV),high lactate levels,older age,female sex,and pre-existing systemic morbidities.There is a definite association between the duration of ICU stay and MV with ICU-AW.However,the interpretation that these are modifiable risk factors influencing ICU-AW,appears to be flawed,because the relationship between longer ICU stays and MV with ICU-AW is reciprocal and cannot yield clinically meaningful strategies for the prevention of ICU-AW.Prevention strategies must be based on other risk factors.Large multicentric randomized controlled trials as well as meta-analysis of such studies can be a more useful approach towards determining the influence of these risk factors on the occurrence of ICU-AW in different populations.展开更多
In this editorial,comments are made on an interesting article in the recent issue of the World Journal of Clinical Cases by Wang and Long.The authors describe the use of neural network model to identify risk factors f...In this editorial,comments are made on an interesting article in the recent issue of the World Journal of Clinical Cases by Wang and Long.The authors describe the use of neural network model to identify risk factors for the development of intensive care unit(ICU)-acquired weakness.This condition has now become common with an increasing number of patients treated in ICUs and continues to be a source of morbidity and mortality.Despite identification of certain risk factors and corrective measures thereof,lacunae still exist in our understanding of this clinical entity.Numerous possible pathogenetic mechanisms at a molecular level have been described and these continue to be increasing.The amount of retrievable data for analysis from the ICU patients for study can be huge and enormous.Machine learning techniques to identify patterns in vast amounts of data are well known and may well provide pointers to bridge the knowledge gap in this condition.This editorial discusses the current knowledge of the condition including pathogenesis,diagnosis,risk factors,preventive measures,and therapy.Furthermore,it looks specifically at ICU acquired weakness in recipients of lung transplantation,because–unlike other solid organ transplants-muscular strength plays a vital role in the preservation and survival of the transplanted lung.Lungs differ from other solid organ transplants in that the proper function of the allograft is dependent on muscle function.Muscular weakness especially diaphragmatic weakness may lead to prolonged ventilation which has deleterious effects on the transplanted lung–ranging from ventilator associated pneumonia to bronchial anastomotic complications due to prolonged positive pressure on the anastomosis.展开更多
This editorial explores the significant challenge of intensive care unit-acquiredweakness(ICU-AW),a prevalent condition affecting critically ill patients,characterizedby profound muscle weakness and complicating patie...This editorial explores the significant challenge of intensive care unit-acquiredweakness(ICU-AW),a prevalent condition affecting critically ill patients,characterizedby profound muscle weakness and complicating patient recovery.Highlightingthe paradox of modern medical advances,it emphasizes the urgent needfor early identification and intervention to mitigate ICU-AW's impact.Innovatively,the study by Wang et al is showcased for employing a multilayer perceptronneural network model,achieving high accuracy in predicting ICU-AWrisk.This advancement underscores the potential of neural network models inenhancing patient care but also calls for continued research to address limitationsand improve model applicability.The editorial advocates for the developmentand validation of sophisticated predictive tools,aiming for personalized carestrategies to reduce ICU-AW incidence and severity,ultimately improving patientoutcomes in critical care settings.展开更多
Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and resp...Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and respiratory muscle weakness. Critical illness polyneuropathy/myopathy in isolation or combination increases intensive care unit morbidity via the inability or difficulty in weaning these patients off mechanical ventilation. Many patients continue to suffer from decreased exercise capacity and compromised quality of life for months to years after the acute event. Substantial progress has been made lately in the understanding of the pathophysiology of critical illness polyneuropathy and myopathy. Clinical and ancillary test results should be carefully interpreted to differentiate critical illness polyneuropathy/myopathy from similar weaknesses in this patient population. The present review is aimed at providing the latest knowledge concerning the pathophysiology of critical illness polyneuropathy/myopathy along with relevant clinical, diagnostic, differentiating, and treatment information for this debilitat- ing neurological disease.展开更多
Clinical, electromyographic and pathological features were studied in 18 patients with lipid storage myopathy (group I ) and 18 patients with polymyositis and dermatomyositis (group II). The results showed a remarkabl...Clinical, electromyographic and pathological features were studied in 18 patients with lipid storage myopathy (group I ) and 18 patients with polymyositis and dermatomyositis (group II). The results showed a remarkable lower spontaneous activity(SA) incidence (14% ) in group I than that (55% ) in group II; 46% and 34% short-duration motor unit potentials (MUAPs) with polyphasic potentials and 74% and 71% short-duration MUAPs without polyphasic potentials respectively; the percentages of increased polyphasic MUAPs were same in the two groups. The reduced or pathologic interference patterns accounted for 61 % in the group I and 50% in group II. Increased CPK, LDH and HBD were also found in both of them. It is suggested that the lipid storage myopathy may be diagnosed when patients have muscle weakness and myalgia with short-duration and low-amplitude and polyphasic MUAPs without or with occasional spontaneous activities, and increased CPK, LDH and HBD.展开更多
基金supported by grants from the National High Technology Research and Development Program of China(Grant No.2022YFC3601500)Sichuan Science and Technology Program(Grant No.2020YFQ0011).
文摘Objective:To explore the association between the Lung Immune Prognostic Index(LIPI)and 1-year all-cause mortality in patients with idiopathic inflammatory myopathy related interstitial lung disease(IIM-ILD).Methods:Patients who were diagnosed with IIM-ILD at West China Hospital,Sichuan University from January 2008 to December 2021 were retrospectively included and categorized into three groups based on LIPI.Univariable and multivariable Cox proportional hazards models were conducted to explore potential association between the LIPI and patients'mortality.Results:A total of 1116 patients were screened,and 830 were included in this study.The multivariable Cox analysis showed that,compared with patients with poor LIPI,the hazard ratio(HR)for all-cause 1-year mortality was 0.22(95%CI 0.05-0.93,P=0.04)for patients in the good LIPI group(LDH<250 IU/L and dNLR<3).After excluding patients lost to follow-up within one year,a similar result was found for LIPI(HR 0.20,95%CI 0.05-0.86;P=0.03).Conclusions:Good LIPI was independently associated with decreased risk of all-cause 1-year mortality in patients with IIM-ILD.This easy-to-obtain index might be served as a potential marker for assessing the prognosis of IIM-ILD.
文摘BACKGROUND Rhabdomyolysis is a life-threatening condition,often leading to progressive renal failure and death.It is caused by destruction of skeletal muscle and the release of myoglobin and other intracellular contents into the circulation.The most frequent cause of this condition is“crush syndrome”,although several others have been described and paraneoplastic inflammatory myopathies associated with various types of cancer are repeatedly reported.CASE SUMMARY We describe a rare case of a patient with pancreatic cancer who developed rhabdomyolysis early on,possibly due to paraneoplastic myositis leading to acute renal failure and eventually to rapid death.A 78-year-old Caucasian woman was referred to our hospital for obstructive jaundice and weight loss due to a lesion in the pancreatic head.She presented increasingly severe renal insufficiency with anuria,a dramatic increase in creatine phosphokinase(36000 U/L,n.v.20-180 U/L)and myoglobin(>120000μg/L,n.v.12-70μg/L).On clinical examination,the patient showed increasing pain in the lower limbs associated with muscle weakness which was severe enough to immobilize her.Paraneoplastic myopathy linked to the malignant lesion of the pancreatic head was suspected.The patient was treated with hemodialysis and intravenous methylprednisolone.Despite all the efforts to prepare the patient for surgery,her general condition rapidly deteriorated and she eventually died 30 d after hospital admission.CONCLUSION The possible causes of rhabdomyolysis in this patient with pancreatic cancer are discussed,the development of paraneoplastic myopathy being the most likely.Clinicians should bear in mind that these syndromes may become clinically manifest at any stage of the cancer course and their early diagnosis and treatment could improve the patient’s prognosis.
基金The Science and Technology Department of Gansu Province,No.20JR5RA355 and No.22JR10KA009Talent Innovation and Entrepreneurship Project of Science and Technology Bureau of Chengguan District,Lanzhou,No.2020RCCX0030+1 种基金Lanzhou Science and Technology Development Guiding Plan Project,No.2019-ZD-37Fund of The First Hospital of Lanzhou University,No.Ldyyyn2020-79.
文摘BACKGROUND Mitochondrial myopathy is a rare genetic disease with maternal inheritance that may involve multiple organ systems.Due to the lack of typical characteristics,its clinical diagnosis is difficult,and it is often misdiagnosed or even missed.CASE SUMMARY The patient was a young college student.When he presented at the hospital,he had severe lactic acidosis,respiratory failure,and shock with multiple organ dysfunction syndrome(MODS).He was treated by mechanical ventilation,venoarterial extracorporeal membrane oxygenation,and other organ support.However,his condition continued to worsen.After a thorough and detailed medical and family history was taken,a mitochondrial crisis was suspected.A muscle biopsy was taken.Further genetic testing confirmed a mitochondrial gene mutation(TRNL13243A>G).The final diagnosis of mitochondrial myopathy was made.Although there is no known specific treatment,intravenous methylprednisone and intravenous immunoglobulin were started.The patient’s shock eventually improved.The further course was complicated by severe infection in multiple sites,severe muscle weakness,and recurrent MODS.After 2 mo of multidisciplinary management and intensive rehabilitation,the patient could walk with assistance 4 mo after admission and walk independently 6 mo after admission.CONCLUSION More attention should be paid to mitochondrial myopathy to avoid missed diagnosis and misdiagnosis.
文摘BACKGROUND Immune-mediated necrotizing myopathy is a rare autoimmune myopathy characterized by muscle weakness and elevated serum creatine kinase,with unique skeletal muscle pathology and magnetic resonance imaging features.CASE SUMMARY In this paper,two patients are reported:One was positive for anti-signal recognition particle antibody,and the other was positive for anti-3-hydroxy-3-methylglutaryl coenzyme A reductase antibody.CONCLUSION The clinical characteristics and treatment of the two patients were analysed,and the literature was reviewed to improve the recognition,diagnosis,and treatment of this disease.
文摘Intensive care unit-acquired weakness(ICU-AW;ICD-10 Code:G72.81)is a syndrome of generalized weakness described as clinically detectable weakness in critically ill patients with no other credible cause.The risk factors for ICU-AW include hyperglycemia,parenteral nutrition,vasoactive drugs,neuromuscular blocking agents,corticosteroids,sedatives,some antibiotics,immobilization,the disease severity,septicemia and systemic inflammatory response syndrome,multiorgan failure,prolonged mechanical ventilation(MV),high lactate levels,older age,female sex,and pre-existing systemic morbidities.There is a definite association between the duration of ICU stay and MV with ICU-AW.However,the interpretation that these are modifiable risk factors influencing ICU-AW,appears to be flawed,because the relationship between longer ICU stays and MV with ICU-AW is reciprocal and cannot yield clinically meaningful strategies for the prevention of ICU-AW.Prevention strategies must be based on other risk factors.Large multicentric randomized controlled trials as well as meta-analysis of such studies can be a more useful approach towards determining the influence of these risk factors on the occurrence of ICU-AW in different populations.
文摘In this editorial,comments are made on an interesting article in the recent issue of the World Journal of Clinical Cases by Wang and Long.The authors describe the use of neural network model to identify risk factors for the development of intensive care unit(ICU)-acquired weakness.This condition has now become common with an increasing number of patients treated in ICUs and continues to be a source of morbidity and mortality.Despite identification of certain risk factors and corrective measures thereof,lacunae still exist in our understanding of this clinical entity.Numerous possible pathogenetic mechanisms at a molecular level have been described and these continue to be increasing.The amount of retrievable data for analysis from the ICU patients for study can be huge and enormous.Machine learning techniques to identify patterns in vast amounts of data are well known and may well provide pointers to bridge the knowledge gap in this condition.This editorial discusses the current knowledge of the condition including pathogenesis,diagnosis,risk factors,preventive measures,and therapy.Furthermore,it looks specifically at ICU acquired weakness in recipients of lung transplantation,because–unlike other solid organ transplants-muscular strength plays a vital role in the preservation and survival of the transplanted lung.Lungs differ from other solid organ transplants in that the proper function of the allograft is dependent on muscle function.Muscular weakness especially diaphragmatic weakness may lead to prolonged ventilation which has deleterious effects on the transplanted lung–ranging from ventilator associated pneumonia to bronchial anastomotic complications due to prolonged positive pressure on the anastomosis.
文摘This editorial explores the significant challenge of intensive care unit-acquiredweakness(ICU-AW),a prevalent condition affecting critically ill patients,characterizedby profound muscle weakness and complicating patient recovery.Highlightingthe paradox of modern medical advances,it emphasizes the urgent needfor early identification and intervention to mitigate ICU-AW's impact.Innovatively,the study by Wang et al is showcased for employing a multilayer perceptronneural network model,achieving high accuracy in predicting ICU-AWrisk.This advancement underscores the potential of neural network models inenhancing patient care but also calls for continued research to address limitationsand improve model applicability.The editorial advocates for the developmentand validation of sophisticated predictive tools,aiming for personalized carestrategies to reduce ICU-AW incidence and severity,ultimately improving patientoutcomes in critical care settings.
基金supported by grants from China Scholarship Council,No.2008102056the National Natural Science Foundation of China,No.81241147
文摘Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and respiratory muscle weakness. Critical illness polyneuropathy/myopathy in isolation or combination increases intensive care unit morbidity via the inability or difficulty in weaning these patients off mechanical ventilation. Many patients continue to suffer from decreased exercise capacity and compromised quality of life for months to years after the acute event. Substantial progress has been made lately in the understanding of the pathophysiology of critical illness polyneuropathy and myopathy. Clinical and ancillary test results should be carefully interpreted to differentiate critical illness polyneuropathy/myopathy from similar weaknesses in this patient population. The present review is aimed at providing the latest knowledge concerning the pathophysiology of critical illness polyneuropathy/myopathy along with relevant clinical, diagnostic, differentiating, and treatment information for this debilitat- ing neurological disease.
文摘Clinical, electromyographic and pathological features were studied in 18 patients with lipid storage myopathy (group I ) and 18 patients with polymyositis and dermatomyositis (group II). The results showed a remarkable lower spontaneous activity(SA) incidence (14% ) in group I than that (55% ) in group II; 46% and 34% short-duration motor unit potentials (MUAPs) with polyphasic potentials and 74% and 71% short-duration MUAPs without polyphasic potentials respectively; the percentages of increased polyphasic MUAPs were same in the two groups. The reduced or pathologic interference patterns accounted for 61 % in the group I and 50% in group II. Increased CPK, LDH and HBD were also found in both of them. It is suggested that the lipid storage myopathy may be diagnosed when patients have muscle weakness and myalgia with short-duration and low-amplitude and polyphasic MUAPs without or with occasional spontaneous activities, and increased CPK, LDH and HBD.
