Non-alcoholic fatty liver disease(NAFLD) is one of the most common comorbidities associated with overweight and metabolic syndrome(Met S). Importantly, NAFLD is one of its most dangerous complications because it can l...Non-alcoholic fatty liver disease(NAFLD) is one of the most common comorbidities associated with overweight and metabolic syndrome(Met S). Importantly, NAFLD is one of its most dangerous complications because it can lead to severe liver pathologies, including fibrosis, cirrhosis and hepatic cellular carcinoma. Given the increasing worldwide prevalence of obesity, NAFLD has become the most common cause of chronic liver disease and therefore is a major global health problem. Currently, NAFLD is predominantly regarded as a hepatic manifestation of Met S. However, accumulating evidence indicates that the effects of NAFLD extend beyond the liver and are negatively associated with a range of chronic diseases, most notably cardiovascular disease(CVD), diabetes mellitus type 2(T2DM) and chronic kidney disease(CKD). It is becoming increasingly clear that these diseases are the result of the same underlying pathophysiological processes associated with Met S, such as insulin resistance, chronic systemic inflammation and dyslipidemia. As a result, they have been shown to be independent reciprocal risk factors. In addition, recent data have shown that NAFLD actively contributes to aggravation of the pathophysiology of CVD, T2 DM, and CKD, as well as several other pathologies. Thus, NAFLD is a direct cause of many chronic diseases associated with MetS, and better detection and treatment of fatty liver disease is therefore urgently needed. As non-invasive screening methods for liver disease become increasingly available, detection and treatment of NAFLD in patients with MetS should therefore be considered by both(sub-) specialists and primary care physicians.展开更多
Background Multisystem inflammatory syndrome in children(MIS-C)is a serious health condition that develops from and is linked to coronavirus disease 2019.MIS-C is considered a multi-organ dysfunction involving cardiac...Background Multisystem inflammatory syndrome in children(MIS-C)is a serious health condition that develops from and is linked to coronavirus disease 2019.MIS-C is considered a multi-organ dysfunction involving cardiac,renal,respiratory,hematologic,gastrointestinal and neurological symptoms and groups of signs and symptoms such as rash or bilateral non-purulent conjunctivitis,hypotension or shock and acute gastrointestinal problems,which require immediate therapeutic intervention to prevent the aggravation of the patient’s health condition.MIS-C is relatively new in the field of evidence-based medicine;however,there are several clinical guidelines for good clinical practice.For every disorder,the guidelines have different suggestions.Hence,based on the current status of the evidence,recommendations have been combined to form a unified guideline for therapeutic management.Methods This paper compares and evaluates the current MIS-C-specific clinical practice guidelines(namely,American Academy of Pediatrics,American College of Rheumatology,Helen DeVos Children’s Hospital Foundation,Children’s Hospital of The King’s Daughters,and the Infectious Diseases Society of America).The compiled literature was then assessed by the authors separately,and an algorithm was proposed for each disorder,taking into consideration the various guidelines proposed for the management of the disorder.Results The features of MIS-C patients are unified;this is very helpful in managing its symptoms and decreasing mortality rates.In addition,recommendations for pharmacological treatment for MIS-C symptoms are formulated after cross-comparison across five different guidelines.Conclusions This study provides a general interpretation of the results in the context of other evidence and implications for future research.It proposes a unified guideline based on the current evidence,with the best potential to maintain suitable clinical standards in the Saudi Arabian Ministry of Health.展开更多
Kawasaki disease(KD)is a systemic vasculitis of unknown etiology,which tends to involve coronary arteries and can lead to acquired heart disease in children.The immuno-inflammatory response and vascular endothelial dy...Kawasaki disease(KD)is a systemic vasculitis of unknown etiology,which tends to involve coronary arteries and can lead to acquired heart disease in children.The immuno-inflammatory response and vascular endothelial dysfunction are important causes of coronary artery disease in patients with KD.Multisystem inflammatory syndrome in children(MIS-C)is a rare inflammatory disease in children identified in recent years,which is caused by severe acute respiratory syndrome coronavirus 2 infection;this disease overlaps with KD.This review examines research progress concerning the immuno-inflammatory response and vascular endothelial dysfunction associated with KD,as well as differences between KD and MIS-C.展开更多
The pandemic caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection has been a major challenge to be faced in recent years.While adults suffered the highest morbidity and mortality rates of cor...The pandemic caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection has been a major challenge to be faced in recent years.While adults suffered the highest morbidity and mortality rates of coronavirus disease 2019,children were thought to be exclusively asymptomatic or to present with mild conditions.However,around April 2020,there was an outbreak of a new clinical syndrome related to SARS-CoV-2 in children-multisystemic inflam-matory syndrome in children(MIS-C)-which comprises a severe and uncon-trolled hyperinflammatory response with multiorgan involvement.The Centers for Disease Control and Prevention considers a suspected case of MIS-C an individual aged<21 years presenting with fever,high inflammatory markers levels,and evidence of clinically severe illness,with multisystem(>2)organ involvement,no alternative plausible diagnoses,and positive for recent SARS-CoV-2 infection.Despite its severity,there are no definitive disease management guidelines for this condition.Conversely,the complex pathogenesis of MIS-C is still not completely understood,although it seems to rely upon immune dysregu-lation.Hence,in this study,we aim to bring together current evidence regarding the pathogenic mechanisms of MIS-C,clinical picture and management,in order to provide insights for clinical practice and implications for future research directions.展开更多
Coronavirus is an important pathogen causing disease in humans and animals.At the end of 2019,an investigation into an increase in pneumonia cases in Wuhan,Hubei Province,China,found that the cause was a new coronavir...Coronavirus is an important pathogen causing disease in humans and animals.At the end of 2019,an investigation into an increase in pneumonia cases in Wuhan,Hubei Province,China,found that the cause was a new coronavirus.This disease,which spread rapidly across China and caused an outbreak worldwide,resulted in a pandemic.Although this virus has previously been referred to as 2019-nCoV,which causes coronavirus disease 2019(COVID-19),later it was named severe acute respiratory syndrome coronavirus 2.Children were usually asymptomatic and rarely severely affected.In April 2020,reports from the United Kingdom indicated that children may have Kawasaki disease or a clinical condition similar to toxic shock syndrome.This clinical picture was later defined as multisystem inflammatory syndrome in children.Since then,similarly affected children as well as cases with other cardiac complications have been reported in other parts of the world.In this review,we aimed to evaluate COVID-19 in terms of cardiac involvement by reviewing the literature.展开更多
Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves ...Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves both upper and lower motor neurons,human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions,expanding the phenotype of ALS.Although superoxide dismutase 1(SOD1)mutations represent a minority of ALS cases,the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized therapies.Despite numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1(SOD1-ALS),no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this subpopulation.In this narrative review,we analyze and discuss the available literature on extrapyramidal and non-motor features during SOD1-ALS.The multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms,pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS.展开更多
BACKGROUND Multisystem inflammatory syndrome in adults(MIS-A)is a rare but severe disease occurring several weeks after severe acute respiratory syndrome coronavirus 2 infection.It develops in adults with inflammation...BACKGROUND Multisystem inflammatory syndrome in adults(MIS-A)is a rare but severe disease occurring several weeks after severe acute respiratory syndrome coronavirus 2 infection.It develops in adults with inflammation of different organs including the gastrointestinal tract,heart,kidneys,skin and hematopoietic system.CASE SUMMARY We present a 58-year-old Chinese man diagnosed with MIS-A.His chief complaints were fever,generalized fatigue and anorexia,accompanied with rashes on his back.Further examination showed cardiac,renal and liver injury.He had melena and gastroscopy indicated esophageal ulcer and severe esophagitis.Repeated blood and sputum culture did not show growth of bacteria or fungi.Antibiotic treatment was stopped due to unsatisfactory performance.His condition improved after prednisone and other supportive treatment.CONCLUSION Gastrointestinal involvement in MIS-A is not uncommon.Intestinal involvement predominates,and esophageal involvement is rarely reported.Esophageal ulcer with bleeding could also be a manifestation of MIS-A.展开更多
This editorial takes a deeper look at the insights provided by Soresi and Giannitrapani,which examined the therapeutic potential of glucagon-like peptide-1 receptor agonists(GLP-1RAs)for metabolic dysfunction-associat...This editorial takes a deeper look at the insights provided by Soresi and Giannitrapani,which examined the therapeutic potential of glucagon-like peptide-1 receptor agonists(GLP-1RAs)for metabolic dysfunction-associated fatty liver disease.We provide supplementary insights to their research,highlighting the broader systemic implications of GLP-1RAs,synthesizing the current understanding of their mechanisms and the trajectory of research in this field.GLP-1RAs are revolutionizing the treatment of type 2 diabetes mellitus and beyond.Beyond glycemic control,GLP-1RAs demonstrate cardiovascular and renal protective effects,offering potential in managing diabetic kidney disease alongside renin–angiotensin–aldosterone system inhibitors.Their role in bone metabolism hints at benefits for diabetic osteoporosis,while the neuroprotective properties of GLP-1RAs show promise in Alzheimer's disease treatment by modulating neuronal insulin signaling.Additionally,they improve hormonal and metabolic profiles in polycystic ovary syndrome.This editorial highlights the multifaceted mechanisms of GLP-1RAs,emphasizing the need for ongoing research to fully realize their therapeutic potential across a range of multisystemic diseases.展开更多
Background Multisystem inflammatory syndrome in children(MIS-C),a relatively uncommon but severe pediatric complication,is associated with coronavirus disease 2019(COVID-19).A variety of treatment approaches,including...Background Multisystem inflammatory syndrome in children(MIS-C),a relatively uncommon but severe pediatric complication,is associated with coronavirus disease 2019(COVID-19).A variety of treatment approaches,including intravenous immunoglobulins(IVIGs),glucocorticoids(GCs)and biologic agents,such as anakinra and infliximab,have been described for the management of COVID-19-related MIS-C.Anticoagulant therapy is also important.However,a well-developed treatment system has not been established,and many issues remain controversial.Several recently published articles related to the treatment of MIS-C have been released.Hence,in this review,we identified relevant articles published recently and summarized the treatment of MIS-C more comprehensively and systematically.Data sources We reviewed the literature on the treatment of MIS-C through 20 September 2023.The PubMed/Medline,Web of Science,EMBASE,and Cochrane Library databases were searched with the combination of the terms"multisystem inflammatory syndrome","MIS-C","PIMS-TS","therapy","treatment","drug","IVIG","GCs","intravenous immunoglobulin","corticosteroids","biological agent",and"aspirin".Results The severity of MIS-C varies,and different treatment schemes should be used according to the specific condition.Ongoing research and data collection are vital to better understand the pathophysiology and optimal management of MIS-C.Conclusions MIS-Cis a disease involving multiple systems and has great heterogeneity.With the accumulation of additional experience,we have garnered fresh insights into its treatment strategies.However,there remains a critical need for greater standardization in treatment protocols,alongside the pressing necessity for more robust and meticulously conducted studies to deepen our understanding of these protocols.展开更多
Strong evidence supports the benefits of regular physical activity/exercise for the prevention and management of numerous non-communicable diseases.^(1,2)However,despite important advances(particularly the implementat...Strong evidence supports the benefits of regular physical activity/exercise for the prevention and management of numerous non-communicable diseases.^(1,2)However,despite important advances(particularly the implementation of complex“omics”approaches),the underlying molecular mechanisms remain to be clearly elucidated.In the past decade,the role of exerkines—a myriad of signaling moieties(e.g.,mainly cytokines but also other small peptides,peptides,nucleic acids,lipids,or metabolites,among others)released by muscles(myokines)and other tissues in response to exercise that induces endocrine(at the multisystem level),paracrine,or autocrine effects—has gained attention.^(3,4)Indeed it has been proposed that some of the salutary effects of regular exercise(i.e.,repeated sessions of acute exercise)might be due,at least partly,to the progressive accumulation of frequent,acute episodes of exerkine release.^(5)展开更多
Background Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection has been spreading rapidly around the world,while“multisystem inflammatory syndrome in children”(MIS-C)is a new type of syndrome that h...Background Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection has been spreading rapidly around the world,while“multisystem inflammatory syndrome in children”(MIS-C)is a new type of syndrome that has now been reported in many countries.Similar and different characteristics between KD and MIS-C have been reported in a variety of literature.We aimed to focus on reviewing clinical presentations,diagnosis,and treatment of KD and MIS-C.Methods We searched articles in the electronic databases,including the Cochrane Library database,EMBASE,and MEDLINE with the keywords“multiple inflammatory syndrome”and/or“COVID-19”and/or“Kawasaki disease”and“children”.Results Main presentations of MIS-C and KD include fever,rashes,mucous membrane involvement,conjunctivitis,hands and feet erythema/edema,and cervical lymphadenopathy.However,compared with the highest incidence of KD among some Asian countries,MIS-C is common among Black and Hispanic children.MIS-C is common in older children and teenagers,whereas classic KD is common in children under five years of age.Gastrointestinal symptoms,shock,and coagulopathy are common in MIS-C patients but are not common in classic KD.Cardiac manifestations are more common than KD,including myocarditis with cardiac dysfunction and coronary artery dilation or aneurysms.Severe cases in MIS-C present with vasodilated or cardiogenic shock that requires fluid resuscitation,muscular support,and even mechanical ventilation and extracorporeal membrane oxygenation(ECMO),whereas KD rarely presents with these manifestations and requires these treatments.Increased serum ferritin,leukopenia,lymphopenia and thrombocytopenia are common in MIS-C.However,thrombocytosis is a characteristic feature of KD.Intravenous immunoglobulin(IVIG)and moderate-high dose aspirin are still a standard recommended treatment for KD.In addition to the above-mentioned medications,steroids and biological drugs are frequently used in patients with MIS-C.Most of the children with KD have a good pro展开更多
1.The need to develop a holographic digital mannequin Life processes,including high intelligence,self-organization,and homeostasis,are characterized by the biological organism in the form of self-renewal,self-replicat...1.The need to develop a holographic digital mannequin Life processes,including high intelligence,self-organization,and homeostasis,are characterized by the biological organism in the form of self-renewal,self-replication and self-regulation,metabolism,self-repair,and self-reproduction,which are all processes of multisystem coordinated movement[1].Research in the field of life sciences is not limited to the use of advanced observational methods to reveal microscopic structures at the subcellular or molecular level.Discoveries based on these methods alone cannot characterize the dynamic processes of life at the microscopic and molecular level[2].展开更多
BACKGROUND Since the beginning of the pandemic,coronavirus disease-2019(COVID-19)in children has shown milder cases and a better prognosis than adults.Although the respiratory tract is the primary target for severe ac...BACKGROUND Since the beginning of the pandemic,coronavirus disease-2019(COVID-19)in children has shown milder cases and a better prognosis than adults.Although the respiratory tract is the primary target for severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),cardiovascular involvement is emerging as one of the most significant and life-threatening complications of SARS-CoV-2 infection in adults.AIM To summarize the current knowledge about the potential cardiovascular involvement in pediatric COVID-19 in order to give a perspective on how to take care of them during the current pandemic emergency.METHODS Multiple searches in MEDLINE,PubMed were performed using the search terms“COVID-19”or“SARS-CoV-2"were used in combination with“myocardial injury”or"arrhythmia"or“cardiovascular involvement”or"heart disease"or"congenital heart disease"or“pulmonary hypertension”or"long QT"or“cardiomyopathies”or“channelopathies”or"Multisystem inflammatory system"or"PMIS"or“MIS-C”or”Pediatric multisystem inflammatory syndrome"or"myocarditis"or"thromboembolism to identify articles published in English language from January 1st,2020 until July 31st,2020.The websites of World Health Organization,Centers for Disease control and Prevention,and the Johns Hopkins Coronavirus Resource Center were reviewed to provide up to date numbers and infection control recommendations.Reference lists from the articles were reviewed to identify additional pertinent articles.Retrieved manuscripts concerning the subject were reviewed by the authors,and the data were extracted using a standardized collection tool.Data were subsequently analyzed with descriptive statistics.For Pediatric multisystemic inflammatory syndrome temporally associated with COVID-19(PMIS),multiple meta-analyses were conducted to summarize the pooled mean proportion of different cardiovascular variables in this population in pseudo-cohorts of observed patients.RESULTS A total of 193 articles were included.Most publications used in this review wer展开更多
BACKGROUND Multisystemic smooth muscle dysfunction syndrome(MSMDS) is a rare genetic disease worldwide. The main mutation is the actin alpha 2(ACTA2) gene p.R179 H. In this paper, we report a Chinese MSMDS patient and...BACKGROUND Multisystemic smooth muscle dysfunction syndrome(MSMDS) is a rare genetic disease worldwide. The main mutation is the actin alpha 2(ACTA2) gene p.R179 H. In this paper, we report a Chinese MSMDS patient and systematically review the previous literature.CASE SUMMARY Here, we report a 9.6-month-old Chinese girl who was diagnosed with MSMDS based on her history and symptoms, such as recurrent cough, wheezing, and complications with congenital fixed dilated pupils. Chest high-resolution computed tomography revealed inhomogeneous lung transparency, obvious exudative lesions, and some lung fissures that were markedly thickened. Cranial magnetic resonance imaging excluded bleeding and infarction but showed abnormal signals in the centrum ovale majus and bilateral periventricular regions. Echocardiography only showed patent foramen ovale, and no patent ductus arteriosus, pulmonary artery dilatation, or pulmonary hypertension was found. Bronchoscopy indicated moderate bronchial malacia. These examinations in conjunction with the typical eye abnormality suggested a diagnosis of MSMDS, and sequencing of exon 6 of the ACTA2 gene demonstrated the heterozygous mutation c.536 G>A, p.R179 H. However, her parents' gene analyses were normal.CONCLUSION MSMDS is a rare genetic disease mainly caused by the mutation of the ACTA2 gene p.R179 H. Early genetic diagnosis should be performed for children presenting with congenital fixed dilated pupils and patent ductus arteriosus.During the process of diagnosis and treatment, clinicians should be on high alert for cerebrovascular, cardiovascular, and pulmonary complications.展开更多
Background We aimed to systematically review the clinical and laboratory features of patients with the multisystem inflam-matory syndrome in pediatrics diagnosed during the COVID-19 pandemic.Data sources A literature ...Background We aimed to systematically review the clinical and laboratory features of patients with the multisystem inflam-matory syndrome in pediatrics diagnosed during the COVID-19 pandemic.Data sources A literature search in Web of Science,PubMed,Scopus,and Science Direct was made up to June 29,2020.Results Analysis of 15 articles(318 COVID-19 patients)revealed that although many patients presented with the typical multisystem inflammatory syndrome in pediatrics,Kawasaki-like features as fever(82.4%),polymorphous maculopapular exanthema(63.7%),oral mucosal changes(58.1%),conjunctival injections(56.0%),edematous extremities(40.7%),and cervical lymphadenopathy(28.5%),atypical gastrointestinal(79.4%)and neurocognitive symptoms(31.8%)were also com-mon.They had elevated serum lactic acid dehydrogenase,D-dimer,C-reactive protein,procalcitonin,interleukin-6,troponin I levels,and lymphopenia.Nearly 77.0%developed hypotension,and 68.1%went into shock,while 41.1%had acute kidney injury.Intensive care was needed in 73.7%of cases;13.2%were intubated,and 37.9%required mechanical ventilation.Intravenous immunoglobulins and steroids were given in 87.7%and 56.9%of the patients,respectively,and anticoagulants were utilized in 67.0%.Pediatric patients were discharged after a hospital stay of 6.77 days on average(95%CI 4.93-8.6).Conclusions Recognizing the typical and atypical presentation of the multisystem inflammatory syndrome in pediatric COVID-19 patients has important implications in identifying children at risk.Monitoring cardiac and renal decompensation and early interventions in patients with multisystem inflammatory syndrome is critical to prevent further morbidity.展开更多
文摘Non-alcoholic fatty liver disease(NAFLD) is one of the most common comorbidities associated with overweight and metabolic syndrome(Met S). Importantly, NAFLD is one of its most dangerous complications because it can lead to severe liver pathologies, including fibrosis, cirrhosis and hepatic cellular carcinoma. Given the increasing worldwide prevalence of obesity, NAFLD has become the most common cause of chronic liver disease and therefore is a major global health problem. Currently, NAFLD is predominantly regarded as a hepatic manifestation of Met S. However, accumulating evidence indicates that the effects of NAFLD extend beyond the liver and are negatively associated with a range of chronic diseases, most notably cardiovascular disease(CVD), diabetes mellitus type 2(T2DM) and chronic kidney disease(CKD). It is becoming increasingly clear that these diseases are the result of the same underlying pathophysiological processes associated with Met S, such as insulin resistance, chronic systemic inflammation and dyslipidemia. As a result, they have been shown to be independent reciprocal risk factors. In addition, recent data have shown that NAFLD actively contributes to aggravation of the pathophysiology of CVD, T2 DM, and CKD, as well as several other pathologies. Thus, NAFLD is a direct cause of many chronic diseases associated with MetS, and better detection and treatment of fatty liver disease is therefore urgently needed. As non-invasive screening methods for liver disease become increasingly available, detection and treatment of NAFLD in patients with MetS should therefore be considered by both(sub-) specialists and primary care physicians.
文摘Background Multisystem inflammatory syndrome in children(MIS-C)is a serious health condition that develops from and is linked to coronavirus disease 2019.MIS-C is considered a multi-organ dysfunction involving cardiac,renal,respiratory,hematologic,gastrointestinal and neurological symptoms and groups of signs and symptoms such as rash or bilateral non-purulent conjunctivitis,hypotension or shock and acute gastrointestinal problems,which require immediate therapeutic intervention to prevent the aggravation of the patient’s health condition.MIS-C is relatively new in the field of evidence-based medicine;however,there are several clinical guidelines for good clinical practice.For every disorder,the guidelines have different suggestions.Hence,based on the current status of the evidence,recommendations have been combined to form a unified guideline for therapeutic management.Methods This paper compares and evaluates the current MIS-C-specific clinical practice guidelines(namely,American Academy of Pediatrics,American College of Rheumatology,Helen DeVos Children’s Hospital Foundation,Children’s Hospital of The King’s Daughters,and the Infectious Diseases Society of America).The compiled literature was then assessed by the authors separately,and an algorithm was proposed for each disorder,taking into consideration the various guidelines proposed for the management of the disorder.Results The features of MIS-C patients are unified;this is very helpful in managing its symptoms and decreasing mortality rates.In addition,recommendations for pharmacological treatment for MIS-C symptoms are formulated after cross-comparison across five different guidelines.Conclusions This study provides a general interpretation of the results in the context of other evidence and implications for future research.It proposes a unified guideline based on the current evidence,with the best potential to maintain suitable clinical standards in the Saudi Arabian Ministry of Health.
文摘Kawasaki disease(KD)is a systemic vasculitis of unknown etiology,which tends to involve coronary arteries and can lead to acquired heart disease in children.The immuno-inflammatory response and vascular endothelial dysfunction are important causes of coronary artery disease in patients with KD.Multisystem inflammatory syndrome in children(MIS-C)is a rare inflammatory disease in children identified in recent years,which is caused by severe acute respiratory syndrome coronavirus 2 infection;this disease overlaps with KD.This review examines research progress concerning the immuno-inflammatory response and vascular endothelial dysfunction associated with KD,as well as differences between KD and MIS-C.
文摘The pandemic caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection has been a major challenge to be faced in recent years.While adults suffered the highest morbidity and mortality rates of coronavirus disease 2019,children were thought to be exclusively asymptomatic or to present with mild conditions.However,around April 2020,there was an outbreak of a new clinical syndrome related to SARS-CoV-2 in children-multisystemic inflam-matory syndrome in children(MIS-C)-which comprises a severe and uncon-trolled hyperinflammatory response with multiorgan involvement.The Centers for Disease Control and Prevention considers a suspected case of MIS-C an individual aged<21 years presenting with fever,high inflammatory markers levels,and evidence of clinically severe illness,with multisystem(>2)organ involvement,no alternative plausible diagnoses,and positive for recent SARS-CoV-2 infection.Despite its severity,there are no definitive disease management guidelines for this condition.Conversely,the complex pathogenesis of MIS-C is still not completely understood,although it seems to rely upon immune dysregu-lation.Hence,in this study,we aim to bring together current evidence regarding the pathogenic mechanisms of MIS-C,clinical picture and management,in order to provide insights for clinical practice and implications for future research directions.
文摘Coronavirus is an important pathogen causing disease in humans and animals.At the end of 2019,an investigation into an increase in pneumonia cases in Wuhan,Hubei Province,China,found that the cause was a new coronavirus.This disease,which spread rapidly across China and caused an outbreak worldwide,resulted in a pandemic.Although this virus has previously been referred to as 2019-nCoV,which causes coronavirus disease 2019(COVID-19),later it was named severe acute respiratory syndrome coronavirus 2.Children were usually asymptomatic and rarely severely affected.In April 2020,reports from the United Kingdom indicated that children may have Kawasaki disease or a clinical condition similar to toxic shock syndrome.This clinical picture was later defined as multisystem inflammatory syndrome in children.Since then,similarly affected children as well as cases with other cardiac complications have been reported in other parts of the world.In this review,we aimed to evaluate COVID-19 in terms of cardiac involvement by reviewing the literature.
文摘Amyotrophic lateral sclerosis(ALS)is a neuromuscular condition resulting from the progressive degeneration of motor neurons in the cortex,brainstem,and spinal cord.While the typical clinical phenotype of ALS involves both upper and lower motor neurons,human and animal studies over the years have highlighted the potential spread to other motor and non-motor regions,expanding the phenotype of ALS.Although superoxide dismutase 1(SOD1)mutations represent a minority of ALS cases,the SOD1 gene remains a milestone in ALS research as it represents the first genetic target for personalized therapies.Despite numerous single case reports or case series exhibiting extramotor symptoms in patients with ALS mutations in SOD1(SOD1-ALS),no studies have comprehensively explored the full spectrum of extramotor neurological manifestations in this subpopulation.In this narrative review,we analyze and discuss the available literature on extrapyramidal and non-motor features during SOD1-ALS.The multifaceted expression of SOD1 could deepen our understanding of the pathogenic mechanisms,pointing towards a multidisciplinary approach for affected patients in light of new therapeutic strategies for SOD1-ALS.
文摘BACKGROUND Multisystem inflammatory syndrome in adults(MIS-A)is a rare but severe disease occurring several weeks after severe acute respiratory syndrome coronavirus 2 infection.It develops in adults with inflammation of different organs including the gastrointestinal tract,heart,kidneys,skin and hematopoietic system.CASE SUMMARY We present a 58-year-old Chinese man diagnosed with MIS-A.His chief complaints were fever,generalized fatigue and anorexia,accompanied with rashes on his back.Further examination showed cardiac,renal and liver injury.He had melena and gastroscopy indicated esophageal ulcer and severe esophagitis.Repeated blood and sputum culture did not show growth of bacteria or fungi.Antibiotic treatment was stopped due to unsatisfactory performance.His condition improved after prednisone and other supportive treatment.CONCLUSION Gastrointestinal involvement in MIS-A is not uncommon.Intestinal involvement predominates,and esophageal involvement is rarely reported.Esophageal ulcer with bleeding could also be a manifestation of MIS-A.
基金Supported by National Natural Science Foundation of China,No.U23A20398 and No.82030007Sichuan Science and Technology Program,No.2022YFS0578.
文摘This editorial takes a deeper look at the insights provided by Soresi and Giannitrapani,which examined the therapeutic potential of glucagon-like peptide-1 receptor agonists(GLP-1RAs)for metabolic dysfunction-associated fatty liver disease.We provide supplementary insights to their research,highlighting the broader systemic implications of GLP-1RAs,synthesizing the current understanding of their mechanisms and the trajectory of research in this field.GLP-1RAs are revolutionizing the treatment of type 2 diabetes mellitus and beyond.Beyond glycemic control,GLP-1RAs demonstrate cardiovascular and renal protective effects,offering potential in managing diabetic kidney disease alongside renin–angiotensin–aldosterone system inhibitors.Their role in bone metabolism hints at benefits for diabetic osteoporosis,while the neuroprotective properties of GLP-1RAs show promise in Alzheimer's disease treatment by modulating neuronal insulin signaling.Additionally,they improve hormonal and metabolic profiles in polycystic ovary syndrome.This editorial highlights the multifaceted mechanisms of GLP-1RAs,emphasizing the need for ongoing research to fully realize their therapeutic potential across a range of multisystemic diseases.
基金the National Natural Science Foundation of China(No.81970434).
文摘Background Multisystem inflammatory syndrome in children(MIS-C),a relatively uncommon but severe pediatric complication,is associated with coronavirus disease 2019(COVID-19).A variety of treatment approaches,including intravenous immunoglobulins(IVIGs),glucocorticoids(GCs)and biologic agents,such as anakinra and infliximab,have been described for the management of COVID-19-related MIS-C.Anticoagulant therapy is also important.However,a well-developed treatment system has not been established,and many issues remain controversial.Several recently published articles related to the treatment of MIS-C have been released.Hence,in this review,we identified relevant articles published recently and summarized the treatment of MIS-C more comprehensively and systematically.Data sources We reviewed the literature on the treatment of MIS-C through 20 September 2023.The PubMed/Medline,Web of Science,EMBASE,and Cochrane Library databases were searched with the combination of the terms"multisystem inflammatory syndrome","MIS-C","PIMS-TS","therapy","treatment","drug","IVIG","GCs","intravenous immunoglobulin","corticosteroids","biological agent",and"aspirin".Results The severity of MIS-C varies,and different treatment schemes should be used according to the specific condition.Ongoing research and data collection are vital to better understand the pathophysiology and optimal management of MIS-C.Conclusions MIS-Cis a disease involving multiple systems and has great heterogeneity.With the accumulation of additional experience,we have garnered fresh insights into its treatment strategies.However,there remains a critical need for greater standardization in treatment protocols,alongside the pressing necessity for more robust and meticulously conducted studies to deepen our understanding of these protocols.
文摘Strong evidence supports the benefits of regular physical activity/exercise for the prevention and management of numerous non-communicable diseases.^(1,2)However,despite important advances(particularly the implementation of complex“omics”approaches),the underlying molecular mechanisms remain to be clearly elucidated.In the past decade,the role of exerkines—a myriad of signaling moieties(e.g.,mainly cytokines but also other small peptides,peptides,nucleic acids,lipids,or metabolites,among others)released by muscles(myokines)and other tissues in response to exercise that induces endocrine(at the multisystem level),paracrine,or autocrine effects—has gained attention.^(3,4)Indeed it has been proposed that some of the salutary effects of regular exercise(i.e.,repeated sessions of acute exercise)might be due,at least partly,to the progressive accumulation of frequent,acute episodes of exerkine release.^(5)
基金This research was supported by grants from the National Natural Science Foundation of China(7171010).
文摘Background Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)infection has been spreading rapidly around the world,while“multisystem inflammatory syndrome in children”(MIS-C)is a new type of syndrome that has now been reported in many countries.Similar and different characteristics between KD and MIS-C have been reported in a variety of literature.We aimed to focus on reviewing clinical presentations,diagnosis,and treatment of KD and MIS-C.Methods We searched articles in the electronic databases,including the Cochrane Library database,EMBASE,and MEDLINE with the keywords“multiple inflammatory syndrome”and/or“COVID-19”and/or“Kawasaki disease”and“children”.Results Main presentations of MIS-C and KD include fever,rashes,mucous membrane involvement,conjunctivitis,hands and feet erythema/edema,and cervical lymphadenopathy.However,compared with the highest incidence of KD among some Asian countries,MIS-C is common among Black and Hispanic children.MIS-C is common in older children and teenagers,whereas classic KD is common in children under five years of age.Gastrointestinal symptoms,shock,and coagulopathy are common in MIS-C patients but are not common in classic KD.Cardiac manifestations are more common than KD,including myocarditis with cardiac dysfunction and coronary artery dilation or aneurysms.Severe cases in MIS-C present with vasodilated or cardiogenic shock that requires fluid resuscitation,muscular support,and even mechanical ventilation and extracorporeal membrane oxygenation(ECMO),whereas KD rarely presents with these manifestations and requires these treatments.Increased serum ferritin,leukopenia,lymphopenia and thrombocytopenia are common in MIS-C.However,thrombocytosis is a characteristic feature of KD.Intravenous immunoglobulin(IVIG)and moderate-high dose aspirin are still a standard recommended treatment for KD.In addition to the above-mentioned medications,steroids and biological drugs are frequently used in patients with MIS-C.Most of the children with KD have a good pro
基金supported by the National Natural Science Foundation of China(82293651)the CAMS Innovation Fund for Medical Sciences(2019-I2M-5-055)the Guangdong Provincial Key Laboratory of Brain Connectome and Behavior(2017B030301017).
文摘1.The need to develop a holographic digital mannequin Life processes,including high intelligence,self-organization,and homeostasis,are characterized by the biological organism in the form of self-renewal,self-replication and self-regulation,metabolism,self-repair,and self-reproduction,which are all processes of multisystem coordinated movement[1].Research in the field of life sciences is not limited to the use of advanced observational methods to reveal microscopic structures at the subcellular or molecular level.Discoveries based on these methods alone cannot characterize the dynamic processes of life at the microscopic and molecular level[2].
文摘BACKGROUND Since the beginning of the pandemic,coronavirus disease-2019(COVID-19)in children has shown milder cases and a better prognosis than adults.Although the respiratory tract is the primary target for severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),cardiovascular involvement is emerging as one of the most significant and life-threatening complications of SARS-CoV-2 infection in adults.AIM To summarize the current knowledge about the potential cardiovascular involvement in pediatric COVID-19 in order to give a perspective on how to take care of them during the current pandemic emergency.METHODS Multiple searches in MEDLINE,PubMed were performed using the search terms“COVID-19”or“SARS-CoV-2"were used in combination with“myocardial injury”or"arrhythmia"or“cardiovascular involvement”or"heart disease"or"congenital heart disease"or“pulmonary hypertension”or"long QT"or“cardiomyopathies”or“channelopathies”or"Multisystem inflammatory system"or"PMIS"or“MIS-C”or”Pediatric multisystem inflammatory syndrome"or"myocarditis"or"thromboembolism to identify articles published in English language from January 1st,2020 until July 31st,2020.The websites of World Health Organization,Centers for Disease control and Prevention,and the Johns Hopkins Coronavirus Resource Center were reviewed to provide up to date numbers and infection control recommendations.Reference lists from the articles were reviewed to identify additional pertinent articles.Retrieved manuscripts concerning the subject were reviewed by the authors,and the data were extracted using a standardized collection tool.Data were subsequently analyzed with descriptive statistics.For Pediatric multisystemic inflammatory syndrome temporally associated with COVID-19(PMIS),multiple meta-analyses were conducted to summarize the pooled mean proportion of different cardiovascular variables in this population in pseudo-cohorts of observed patients.RESULTS A total of 193 articles were included.Most publications used in this review wer
基金Supported by the National Natural Science Foundation of China,No.81573167Science and Technology Project of Jiangsu,No.BE2017657
文摘BACKGROUND Multisystemic smooth muscle dysfunction syndrome(MSMDS) is a rare genetic disease worldwide. The main mutation is the actin alpha 2(ACTA2) gene p.R179 H. In this paper, we report a Chinese MSMDS patient and systematically review the previous literature.CASE SUMMARY Here, we report a 9.6-month-old Chinese girl who was diagnosed with MSMDS based on her history and symptoms, such as recurrent cough, wheezing, and complications with congenital fixed dilated pupils. Chest high-resolution computed tomography revealed inhomogeneous lung transparency, obvious exudative lesions, and some lung fissures that were markedly thickened. Cranial magnetic resonance imaging excluded bleeding and infarction but showed abnormal signals in the centrum ovale majus and bilateral periventricular regions. Echocardiography only showed patent foramen ovale, and no patent ductus arteriosus, pulmonary artery dilatation, or pulmonary hypertension was found. Bronchoscopy indicated moderate bronchial malacia. These examinations in conjunction with the typical eye abnormality suggested a diagnosis of MSMDS, and sequencing of exon 6 of the ACTA2 gene demonstrated the heterozygous mutation c.536 G>A, p.R179 H. However, her parents' gene analyses were normal.CONCLUSION MSMDS is a rare genetic disease mainly caused by the mutation of the ACTA2 gene p.R179 H. Early genetic diagnosis should be performed for children presenting with congenital fixed dilated pupils and patent ductus arteriosus.During the process of diagnosis and treatment, clinicians should be on high alert for cerebrovascular, cardiovascular, and pulmonary complications.
文摘Background We aimed to systematically review the clinical and laboratory features of patients with the multisystem inflam-matory syndrome in pediatrics diagnosed during the COVID-19 pandemic.Data sources A literature search in Web of Science,PubMed,Scopus,and Science Direct was made up to June 29,2020.Results Analysis of 15 articles(318 COVID-19 patients)revealed that although many patients presented with the typical multisystem inflammatory syndrome in pediatrics,Kawasaki-like features as fever(82.4%),polymorphous maculopapular exanthema(63.7%),oral mucosal changes(58.1%),conjunctival injections(56.0%),edematous extremities(40.7%),and cervical lymphadenopathy(28.5%),atypical gastrointestinal(79.4%)and neurocognitive symptoms(31.8%)were also com-mon.They had elevated serum lactic acid dehydrogenase,D-dimer,C-reactive protein,procalcitonin,interleukin-6,troponin I levels,and lymphopenia.Nearly 77.0%developed hypotension,and 68.1%went into shock,while 41.1%had acute kidney injury.Intensive care was needed in 73.7%of cases;13.2%were intubated,and 37.9%required mechanical ventilation.Intravenous immunoglobulins and steroids were given in 87.7%and 56.9%of the patients,respectively,and anticoagulants were utilized in 67.0%.Pediatric patients were discharged after a hospital stay of 6.77 days on average(95%CI 4.93-8.6).Conclusions Recognizing the typical and atypical presentation of the multisystem inflammatory syndrome in pediatric COVID-19 patients has important implications in identifying children at risk.Monitoring cardiac and renal decompensation and early interventions in patients with multisystem inflammatory syndrome is critical to prevent further morbidity.
