Background:Neonatal-onset multisystem inflammatory disease is characterized by fever,urticarial rash,aseptic meningitis,deforming arthropathy,hearing loss,and mental retardation.Many patients have mutations in the col...Background:Neonatal-onset multisystem inflammatory disease is characterized by fever,urticarial rash,aseptic meningitis,deforming arthropathy,hearing loss,and mental retardation.Many patients have mutations in the cold-induced autoinflammatory syndrome 1(CIAS1)gene,encoding cryopyrin,a protein that regulates inflammation.展开更多
BACKGROUND In multisystem inflammatory syndrome in children(MIS-C)with coronavirus disease 2019,there was paucity of data from low-income and middle-income countries on cardiovascular involvement and its longitudinal ...BACKGROUND In multisystem inflammatory syndrome in children(MIS-C)with coronavirus disease 2019,there was paucity of data from low-income and middle-income countries on cardiovascular involvement and its longitudinal outcomes.We planned to estimate the pattern of cardiovascular involvement among children with MIS-C and its mid-term outcomes.AIM To determine association between cardiovascular abnormalities and clinical and laboratory parameters.To study the time-line for resolution of various abnormalities.METHODS In this prospective study done in a tertiary care hospital,270 were recruited from June 2020 to January 2022.Baseline demographic data and clinical presentation were recorded.Laboratory parameters and echocardiography were done at admission.Follow-up was done at 2 weeks,3 months,6 months and 1 year after diagnosis.Descriptive statistics were used for parametric and non-parametric data.Risk factors were identified by multivariate regression analysis.RESULTS The 211(78.2%)had cardiac involvement and 102 needed intensive care unit(ICU)admission.Cardiovascular abnormalities observed were shock 123(45.6%),coronary dilatation 28(10.4%),coronary aneurysm 77(28.5%),left ventricular(LV)dysfunction 78(29.3%),mitral regurgitation(MR)77(28.5%)and pericardial effusion 98(36.3%).Coronary artery aneurysm/dilatation during follow-up at 2 weeks and 1 year were 25.7%and 0.9%respectively.Multivariate regression analysis revealed breathlessness[odds ratio(OR)=3.91,95%CI:1.25-12.21,P=0.019]and hi-flow nasal cannula(HFNC)support(OR=8.5,95%CI:1.06-68.38,P=0.044)as predictors of cardiovascular involvement.Higher mean age(OR=1.16,95%CI:1.02-1.32,P=0.026),breathlessness(OR=4.99,95%CI:2.05-12.20,P<0.001),gallop(OR=4.45,95%CI:0.41-2.52,P=0.016),MR(OR=3.61,95%CI:1.53-8.53,P=0.004)and invasive ventilation(OR=4.01,95%CI:1.28-12.58,P=0.017)were predictive of LV dysfunction.Altered sensorium(OR=4.96,95%CI:2.23-11.02,P<0.001),headache(OR=6.61,95%CI:1.46-29.92,P=0.014),HFNC(OR=7.03,95%CI:2.04-24.29,P=0.002),non-rebreathing mask usage(OR=21.13,95%CI:9.00-49.61,P<0.001)and invasive ventilation(OR=5.64,95%CI:1.42-22.45,P=0.014)were risk factors for shock.Anemia was a risk factor for coronary involvement(OR=3.09,95%CI:1.79-5.34,P<0.001).CONCLUSION Significant number of children with MIS-C had cardiovascular involvement contributing to higher ICU management.Although shock resolved quickly,resolution of ventricular function and coronary abnormalities were slower,and hence warrants a structured long-term follow-up protocol.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
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
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 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 Multisystem inflammatory syndrome in children(MIS-C) has emerged as a new disease associated with COVID-19 that presents in acute critically ill children with acute cardiovascular dysfunction.AIM To determi...BACKGROUND Multisystem inflammatory syndrome in children(MIS-C) has emerged as a new disease associated with COVID-19 that presents in acute critically ill children with acute cardiovascular dysfunction.AIM To determine whether the age-adjusted N-terminal pro-brain natriuretic peptide(NT-proBNP) value(Z-log-NT-proBNP) is associated with severe MIS-C and myocardial dysfunction.METHODS A retrospective study was conducted which included children with MIS-C managed at our institution between April 1,2020,and February 28,2022.We divided the population into groups depending on severity based on pediatric intensive care unit(PICU) admission.We compared Z-log-NT-proBNP values across these groups and analyzed Z-log-NT-proBNP dynamics during the onemonth follow-up.RESULTS We included 17 participants [median age 3(2-9) years] and seven(41%) required PICU admission.All(100%) of these cases presented very high(Z-log > 4) levels of NT-proBNP at the time of admission compared to only 5(50%) patients with non-severe MIS-C(P = 0.025).NT-proBNP was significantly correlated with highsensitive Troponin I levels(P = 0.045),Ross modified score(P = 0.003) and left ventricle ejection fraction(P = 0.021).CONCLUSION Raised NT-proBNP,specifically very high values(Z-log-NT-proBNP > 4) could help in the early identification of MIS-C patients with myocardial dysfunction requiring inotropic support and PICU admission.展开更多
Background: Pregnant women and newborns are highly susceptible to Covid-19, manifesting as multisystem inflammatory syndrome-New-born (MISC-N) in many babies born to Covid positive mothers. The relationship between Co...Background: Pregnant women and newborns are highly susceptible to Covid-19, manifesting as multisystem inflammatory syndrome-New-born (MISC-N) in many babies born to Covid positive mothers. The relationship between Covid-19 infection during pregnancy and neonatal neurodevelopmental outcome, if any, is unclear necessitating a follow-up study in this aspect. Methods: 16 babies with MIS-N, born to symptomatic Covid antibody positive mothers were enrolled. Demographic profile, treatment details and biochemical parameters were analyzed with neurodevelopmental follow-up. Results: 25% mothers received 2 doses of Covid vaccine;50% had oligohydramnios and 75% received antenatal steroids. 87.5% were preterm of which 62.5% required surfactant with ventilator support and 75% required ionotropic support. Significant association was found between the antibody level and D-dimer levels with the ferritin and LDH levels of the baby (p 0.05);gestational age with LDH and D-dimer levels (p 0.05) and Covid antibody level of the baby vs the duration of ventilator requirement (P-value-0.0009). D-dimer values of babies were positively associated with both maternal antibody and D-dimer levels. Neurodevelopmental follow-up done at 6 months of corrected gestational age showed 37.5% were normal, 37.5% hypertonic and 25% hypotonic. HINE score was below 60 in 62.5%. Development assessment using Bayley-III showed a delay in the motor domain (62.5%), cognitive domain (56.25%) and language domain (62.5%). Conclusion: Neurodevelopmental problems occur in babies born to Covid positive mothers and should be stratified as “high risk”. Anticipatory guidance to prospective mothers for preterm care should be given. Covid antibody titre and D-dimer levels may help to predict the NICU stay, ventilator requirement and the adverse neurodevelopmental outcomes in these babies.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
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
Multisystem inflammatory syndrome in children(MIS-C)is a rare and severe condition that follows benign COVID-19.We report autosomal recessive deficiencies of OAS1,OAS2,or RNASEL in five unrelated children with MIS-C.T...Multisystem inflammatory syndrome in children(MIS-C)is a rare and severe condition that follows benign COVID-19.We report autosomal recessive deficiencies of OAS1,OAS2,or RNASEL in five unrelated children with MIS-C.The cytosolic dsRNA-sensing OAS1 and OAS2 generate 2'-5'-linked oligoadenylates(2-5A)that activate the ssRNA-degrading RNase L.展开更多
A 45-year old man diagnosed with multi-systemic smooth muscle dysfunction syndrome(MSMDS)was referred for the evaluation of recurrent dizziness that had persisted for 2 years.MRI revealed a right frontal lobe cerebral...A 45-year old man diagnosed with multi-systemic smooth muscle dysfunction syndrome(MSMDS)was referred for the evaluation of recurrent dizziness that had persisted for 2 years.MRI revealed a right frontal lobe cerebral infarction,enlargement of the bilateral ventricular trigones and hypoplasia of the corpus callosum(figure 1A,B).MRA showed occlusion of the right middle cerebral artery(figure 1C,D).Cinematic rendering of CTA highlighted characteristic cerebro-vascular manifestations of MSMDS:dilata-tion of the internal carotid artery from the cavernous to the clinoid segments,stenosis and occlusion of the distal intracranial circulation and an abnormally straight course of intracranial arteries(figure 2,online supplemental video S1).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
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
A new easy method has been presented to calculate the variable intervals corresponding to the stable univariant curves and to discriminate the stabilities of invariant points. This method and the one reported previous...A new easy method has been presented to calculate the variable intervals corresponding to the stable univariant curves and to discriminate the stabilities of invariant points. This method and the one reported previously constitute a simple and universal theory for the computer-plotting of the equilibrium phase diagrams of a multisystem—sign function matrix (SFM) discrimination method. Its main steps are: determining the stable univariant scheme according to the derivative (or difference) of AIG m ; grouping the univariant curves by comparisons of the mutual relations among them; determining the existing intervals of the variables for the stable curves by comparisons of coordinate values of the curves about the invariant point; determining the stabilities of invariant points by comparisons of relations between the common curves and the invariant points. This method is suitable for any kind of phase diagram of closed or open systems in a phase diagram “space” with either 2 or more than 2 dimensions.展开更多
Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)-induced multisystem inflammatory syndrome in children(MIS-C)is a life-threatening illness that has been reported in the United States and Europe.It affects m...Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)-induced multisystem inflammatory syndrome in children(MIS-C)is a life-threatening illness that has been reported in the United States and Europe.It affects multiple organ systems and often requires patient admission to an intensive care unit.Although some features of MIS-C overlap with Kawasaki disease,MIS-C is more common among older children and adolescents,more often affects cardiovascular and gastrointestinal systems,and more frequently presents with elevated inflammatory markers.Rapid and complete clinical recovery is possible in nearly all patients following immunomodulation therapy.Thus far,MIS-C pathophysiology and long-term prognosis are not sufficiently clear;further studies are needed.展开更多
As a continuation to the work reported by Yin in 1992, a new procedure is presented for computer plotting of the stable equilibrium phase diagram of an n-component system composed of (n + k) stoichiometric phases (or ...As a continuation to the work reported by Yin in 1992, a new procedure is presented for computer plotting of the stable equilibrium phase diagram of an n-component system composed of (n + k) stoichiometric phases (or fluid species) where 2≤k≤4. The main points of the procedure are: (i) using the technique of sequential-absence of phases (SAP) to determine the possible invariant and univariant assemblages in a given multisystem; (ii) using the matrix inverse technique to generate and balance the univariant reactions from the corresponding univariant assemblages; (iii) comparing the phase assemblage at each invariant point with that of each univariant reaction to select the univariant curves about the corresponding invariant point; (iv) locating the invariant points with the technique of finding common equilibrium relation (CER); (v) using the sign function matrix (SFM) technique to discriminate between the stable portion of a univariant curve and its metastable extension about the corresponding invariant point; and (vi) using a new SFM method to identify the stabilities of the invariant points. The source program for microcomputers, PHD, is written in TURBO-PASCAL.展开更多
文摘Background:Neonatal-onset multisystem inflammatory disease is characterized by fever,urticarial rash,aseptic meningitis,deforming arthropathy,hearing loss,and mental retardation.Many patients have mutations in the cold-induced autoinflammatory syndrome 1(CIAS1)gene,encoding cryopyrin,a protein that regulates inflammation.
文摘BACKGROUND In multisystem inflammatory syndrome in children(MIS-C)with coronavirus disease 2019,there was paucity of data from low-income and middle-income countries on cardiovascular involvement and its longitudinal outcomes.We planned to estimate the pattern of cardiovascular involvement among children with MIS-C and its mid-term outcomes.AIM To determine association between cardiovascular abnormalities and clinical and laboratory parameters.To study the time-line for resolution of various abnormalities.METHODS In this prospective study done in a tertiary care hospital,270 were recruited from June 2020 to January 2022.Baseline demographic data and clinical presentation were recorded.Laboratory parameters and echocardiography were done at admission.Follow-up was done at 2 weeks,3 months,6 months and 1 year after diagnosis.Descriptive statistics were used for parametric and non-parametric data.Risk factors were identified by multivariate regression analysis.RESULTS The 211(78.2%)had cardiac involvement and 102 needed intensive care unit(ICU)admission.Cardiovascular abnormalities observed were shock 123(45.6%),coronary dilatation 28(10.4%),coronary aneurysm 77(28.5%),left ventricular(LV)dysfunction 78(29.3%),mitral regurgitation(MR)77(28.5%)and pericardial effusion 98(36.3%).Coronary artery aneurysm/dilatation during follow-up at 2 weeks and 1 year were 25.7%and 0.9%respectively.Multivariate regression analysis revealed breathlessness[odds ratio(OR)=3.91,95%CI:1.25-12.21,P=0.019]and hi-flow nasal cannula(HFNC)support(OR=8.5,95%CI:1.06-68.38,P=0.044)as predictors of cardiovascular involvement.Higher mean age(OR=1.16,95%CI:1.02-1.32,P=0.026),breathlessness(OR=4.99,95%CI:2.05-12.20,P<0.001),gallop(OR=4.45,95%CI:0.41-2.52,P=0.016),MR(OR=3.61,95%CI:1.53-8.53,P=0.004)and invasive ventilation(OR=4.01,95%CI:1.28-12.58,P=0.017)were predictive of LV dysfunction.Altered sensorium(OR=4.96,95%CI:2.23-11.02,P<0.001),headache(OR=6.61,95%CI:1.46-29.92,P=0.014),HFNC(OR=7.03,95%CI:2.04-24.29,P=0.002),non-rebreathing mask usage(OR=21.13,95%CI:9.00-49.61,P<0.001)and invasive ventilation(OR=5.64,95%CI:1.42-22.45,P=0.014)were risk factors for shock.Anemia was a risk factor for coronary involvement(OR=3.09,95%CI:1.79-5.34,P<0.001).CONCLUSION Significant number of children with MIS-C had cardiovascular involvement contributing to higher ICU management.Although shock resolved quickly,resolution of ventricular function and coronary abnormalities were slower,and hence warrants a structured long-term follow-up protocol.
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.
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 Multisystem inflammatory syndrome in children(MIS-C) has emerged as a new disease associated with COVID-19 that presents in acute critically ill children with acute cardiovascular dysfunction.AIM To determine whether the age-adjusted N-terminal pro-brain natriuretic peptide(NT-proBNP) value(Z-log-NT-proBNP) is associated with severe MIS-C and myocardial dysfunction.METHODS A retrospective study was conducted which included children with MIS-C managed at our institution between April 1,2020,and February 28,2022.We divided the population into groups depending on severity based on pediatric intensive care unit(PICU) admission.We compared Z-log-NT-proBNP values across these groups and analyzed Z-log-NT-proBNP dynamics during the onemonth follow-up.RESULTS We included 17 participants [median age 3(2-9) years] and seven(41%) required PICU admission.All(100%) of these cases presented very high(Z-log > 4) levels of NT-proBNP at the time of admission compared to only 5(50%) patients with non-severe MIS-C(P = 0.025).NT-proBNP was significantly correlated with highsensitive Troponin I levels(P = 0.045),Ross modified score(P = 0.003) and left ventricle ejection fraction(P = 0.021).CONCLUSION Raised NT-proBNP,specifically very high values(Z-log-NT-proBNP > 4) could help in the early identification of MIS-C patients with myocardial dysfunction requiring inotropic support and PICU admission.
文摘Background: Pregnant women and newborns are highly susceptible to Covid-19, manifesting as multisystem inflammatory syndrome-New-born (MISC-N) in many babies born to Covid positive mothers. The relationship between Covid-19 infection during pregnancy and neonatal neurodevelopmental outcome, if any, is unclear necessitating a follow-up study in this aspect. Methods: 16 babies with MIS-N, born to symptomatic Covid antibody positive mothers were enrolled. Demographic profile, treatment details and biochemical parameters were analyzed with neurodevelopmental follow-up. Results: 25% mothers received 2 doses of Covid vaccine;50% had oligohydramnios and 75% received antenatal steroids. 87.5% were preterm of which 62.5% required surfactant with ventilator support and 75% required ionotropic support. Significant association was found between the antibody level and D-dimer levels with the ferritin and LDH levels of the baby (p 0.05);gestational age with LDH and D-dimer levels (p 0.05) and Covid antibody level of the baby vs the duration of ventilator requirement (P-value-0.0009). D-dimer values of babies were positively associated with both maternal antibody and D-dimer levels. Neurodevelopmental follow-up done at 6 months of corrected gestational age showed 37.5% were normal, 37.5% hypertonic and 25% hypotonic. HINE score was below 60 in 62.5%. Development assessment using Bayley-III showed a delay in the motor domain (62.5%), cognitive domain (56.25%) and language domain (62.5%). Conclusion: Neurodevelopmental problems occur in babies born to Covid positive mothers and should be stratified as “high risk”. Anticipatory guidance to prospective mothers for preterm care should be given. Covid antibody titre and D-dimer levels may help to predict the NICU stay, ventilator requirement and the adverse neurodevelopmental outcomes in these babies.
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.
文摘Multisystem inflammatory syndrome in children(MIS-C)is a rare and severe condition that follows benign COVID-19.We report autosomal recessive deficiencies of OAS1,OAS2,or RNASEL in five unrelated children with MIS-C.The cytosolic dsRNA-sensing OAS1 and OAS2 generate 2'-5'-linked oligoadenylates(2-5A)that activate the ssRNA-degrading RNase L.
基金funded by the National Natural Science Foundation of China(82271319)。
文摘A 45-year old man diagnosed with multi-systemic smooth muscle dysfunction syndrome(MSMDS)was referred for the evaluation of recurrent dizziness that had persisted for 2 years.MRI revealed a right frontal lobe cerebral infarction,enlargement of the bilateral ventricular trigones and hypoplasia of the corpus callosum(figure 1A,B).MRA showed occlusion of the right middle cerebral artery(figure 1C,D).Cinematic rendering of CTA highlighted characteristic cerebro-vascular manifestations of MSMDS:dilata-tion of the internal carotid artery from the cavernous to the clinoid segments,stenosis and occlusion of the distal intracranial circulation and an abnormally straight course of intracranial arteries(figure 2,online supplemental video S1).
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.
基金This work was supported by the National Natural Science Foundation of China(Grant Nos.4907017849773200).
文摘A new easy method has been presented to calculate the variable intervals corresponding to the stable univariant curves and to discriminate the stabilities of invariant points. This method and the one reported previously constitute a simple and universal theory for the computer-plotting of the equilibrium phase diagrams of a multisystem—sign function matrix (SFM) discrimination method. Its main steps are: determining the stable univariant scheme according to the derivative (or difference) of AIG m ; grouping the univariant curves by comparisons of the mutual relations among them; determining the existing intervals of the variables for the stable curves by comparisons of coordinate values of the curves about the invariant point; determining the stabilities of invariant points by comparisons of relations between the common curves and the invariant points. This method is suitable for any kind of phase diagram of closed or open systems in a phase diagram “space” with either 2 or more than 2 dimensions.
文摘Severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)-induced multisystem inflammatory syndrome in children(MIS-C)is a life-threatening illness that has been reported in the United States and Europe.It affects multiple organ systems and often requires patient admission to an intensive care unit.Although some features of MIS-C overlap with Kawasaki disease,MIS-C is more common among older children and adolescents,more often affects cardiovascular and gastrointestinal systems,and more frequently presents with elevated inflammatory markers.Rapid and complete clinical recovery is possible in nearly all patients following immunomodulation therapy.Thus far,MIS-C pathophysiology and long-term prognosis are not sufficiently clear;further studies are needed.
基金Project supported by the National Natural Science Foundation of Chinathe Foundation of the State Education Commission of China for Senior Visiting Scholars
文摘As a continuation to the work reported by Yin in 1992, a new procedure is presented for computer plotting of the stable equilibrium phase diagram of an n-component system composed of (n + k) stoichiometric phases (or fluid species) where 2≤k≤4. The main points of the procedure are: (i) using the technique of sequential-absence of phases (SAP) to determine the possible invariant and univariant assemblages in a given multisystem; (ii) using the matrix inverse technique to generate and balance the univariant reactions from the corresponding univariant assemblages; (iii) comparing the phase assemblage at each invariant point with that of each univariant reaction to select the univariant curves about the corresponding invariant point; (iv) locating the invariant points with the technique of finding common equilibrium relation (CER); (v) using the sign function matrix (SFM) technique to discriminate between the stable portion of a univariant curve and its metastable extension about the corresponding invariant point; and (vi) using a new SFM method to identify the stabilities of the invariant points. The source program for microcomputers, PHD, is written in TURBO-PASCAL.
