Great advances have been achieved in the integration of western and traditional Chinese medicine (TCM).More and more TCM physicians in their practice accept the term 'differential diagnosis of the diseases' in...Great advances have been achieved in the integration of western and traditional Chinese medicine (TCM).More and more TCM physicians in their practice accept the term 'differential diagnosis of the diseases' instead of the syndromes since western medicine has been introduced into the TCM circle.展开更多
Non-alcoholic fatty liver disease(NAFLD) is the most frequent cause of liver disease in the Western world. Furthermore, it is increasing worldwide, paralleling the obesity pandemic. Though highly frequent, only about ...Non-alcoholic fatty liver disease(NAFLD) is the most frequent cause of liver disease in the Western world. Furthermore, it is increasing worldwide, paralleling the obesity pandemic. Though highly frequent, only about one fifth of affected subjects are at risk of developing the progressive form of the disease, non-alcoholic steatohepatitis with fibrosis. Even in the latter, liver disease is slowly progressive, though, since it is so prevalent, it is already the third cause of liver transplantation in the United States, and it is predicted to get to the top of the ranking in few years. Of relevance, fatty liver is also associated with increased overall mortality and particularly increased cardiovascular mortality. The literature and amount of published papers on NAFLD is increasing as fast as its prevalence, which makes it difficult to keep updated in this topic. This review aims to summarize the latest knowledge on NAFLD, in order to help clinicians understanding its pathogenesis and advances on diagnosis and treatment.展开更多
Vanishing bone disease(Gorham-Stout syndrome) is a rare entity of unknown etiology, characterized by de struction of osseous matrix and proliferation of vascula structures, resulting in destruction and absorption o bo...Vanishing bone disease(Gorham-Stout syndrome) is a rare entity of unknown etiology, characterized by de struction of osseous matrix and proliferation of vascula structures, resulting in destruction and absorption o bone. Despite the extensive investigation of the patho genetic mechanisms of the disease, its etiology hasn'been clarified and several theories exist. The syndrome can affect one or multiple bones of the patient, includ ing the skull, the upper and lower extremities, the spine and pelvis. The clinical presentation of a patient suffer ing from vanishing bone disease includes, pain, func tional impairment and swelling of the affected region although asymptomatic cases have been reported, as well as cases in which the diagnosis was made after a pathologic fracture. In this short review we summarize the theories regarding the etiology as well as the clini cal presentation, the diagnostic approach and treat ment options of this rare disease.展开更多
Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,...Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,evaluating the weight of different factors,and quantitatively evaluating the risk of intracranial hemorrhage in moyamoya disease.To investigate whether convolutional neural network algorithms can be used to recognize moyamoya disease and predict hemorrhagic episodes,we retrospectively selected 460 adult unilateral hemispheres with moyamoya vasculopathy as positive samples for diagnosis modeling,including 418 hemispheres with moyamoya disease and 42 hemispheres with moyamoya syndromes.Another 500 hemispheres with normal vessel appearance were selected as negative samples.We used deep residual neural network(ResNet-152)algorithms to extract features from raw data obtained from digital subtraction angiography of the internal carotid artery,then trained and validated the model.The accuracy,sensitivity,and specificity of the model in identifying unilateral moyamoya vasculopathy were 97.64±0.87%,96.55±3.44%,and 98.29±0.98%,respectively.The area under the receiver operating characteristic curve was 0.990.We used a combined multi-view conventional neural network algorithm to integrate age,sex,and hemorrhagic factors with features of the digital subtraction angiography.The accuracy of the model in predicting unilateral hemorrhagic risk was 90.69±1.58%and the sensitivity and specificity were 94.12±2.75%and 89.86±3.64%,respectively.The deep learning algorithms we proposed were valuable and might assist in the automatic diagnosis of moyamoya disease and timely recognition of the risk for re-hemorrhage.This study was approved by the Institutional Review Board of Huashan Hospital,Fudan University,China(approved No.2014-278)on January 12,2015.展开更多
The novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)that suddenly emerged at the end of December 2019 and caused coronavirus disease 2019(COVID-19)continues to afflict humanity,not only seriously affe...The novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)that suddenly emerged at the end of December 2019 and caused coronavirus disease 2019(COVID-19)continues to afflict humanity,not only seriously affecting healthcare systems but also leading to global social and economic imbalances.As of August 2022,there were approximately 580 million confirmed cases of COVID-19 and approximately 6.4 million confirmed deaths due to this disease.The data are sufficient to highlight the seriousness of SARS-CoV-2 infection.Although most patients with COVID-19 present primarily with respiratory symptoms,an increasing number of extrapulmonary systemic symptoms and manifestations have been associated with COVID-19.Since the outbreak of COVID-19,much has been learned about the disease and its causative agent.Therefore,great effort has been aimed at developing treatments and drug interventions to treat and reduce the incidence of COVID-19.In this narrative review,we provide a brief overview of the epidemiology,mechanisms,clinical manifestations,diagnosis,and therapeutics of COVID-19.展开更多
BACKGROUND Castleman disease(CD)and TAFRO syndrome are very rare in clinical practice.Most clinicians,especially non-hematological clinicians,do not know enough about the two diseases,so it often leads to misdiagnosis...BACKGROUND Castleman disease(CD)and TAFRO syndrome are very rare in clinical practice.Most clinicians,especially non-hematological clinicians,do not know enough about the two diseases,so it often leads to misdiagnosis or missed diagnosis.AIM To explore the clinical features and diagnosis of CD and TAFRO syndrome.METHODS We retrospectively collected the clinical and laboratory data of 39 patients who were diagnosed with CD from a single medical center.RESULTS Clinical classification identified 18 patients(46.15%)with unicentric Castleman disease(UCD)and 21 patients(53.85%)with multicentric Castleman disease(MCD),the latter is further divided into 13 patients(33.33%)with idiopathic multicentric Castleman disease-not otherwise specified(iMCD-NOS)and 8 patients(20.51%)with TAFRO syndrome.UCD and iMCD are significantly different in clinical manifestations,treatment,and prognosis.However,a few patients with MCD were diagnosed as UCD in their early stage.There was a correlation between two of Thrombocytopenia,anasarca and elevated creatinine,which were important components of TAFRO syndrome.In UCD group,the pathologies of lymph modes were mostly hyaline vascular type(13/18,72.22%),however plasma cell type or mixed type could also appear.In iMCD-NOS group and TAFRO syndrome group,the pathologies of lymph mode shown polarity of plasma cell type and hyaline vascular type respectively.Compared with patients with TAFRO syndrome,patients with iMCD-NOS were diagnosed more difficultly.CONCLUSION The clinical and pathological types of CD are not completely separate,there is an intermediate situation or mixed characteristics between two ends of clinical and pathological types.The clinical manifestations of patients with CD are determined by their pathological type.TAFRO syndrome is a special subtype of iMCD with unique clinical manifestations.展开更多
Non-alcoholic fatty liver disease(NAFLD) in children is becoming a major health concern. A "multiple-hit" pathogenetic model has been suggested to explain the progressive liver damage that occurs among child...Non-alcoholic fatty liver disease(NAFLD) in children is becoming a major health concern. A "multiple-hit" pathogenetic model has been suggested to explain the progressive liver damage that occurs among children with NAFLD. In addition to the accumulation of fat in the liver, insulin resistance(IR) and oxidative stress due to genetic/epigenetic background, unfavorable lifestyles, gut microbiota and gut-liver axis dysfunction, and perturbations of trace element homeostasis have been shown to be critical for disease progression and the development of more severe inflammatory and fibrotic stages [non-alcoholic steatohepatitis(NASH)]. Simple clinical and laboratory parameters, such as age, history, anthropometrical data(BMI and waist circumference percentiles), blood pressure, surrogate clinical markers of IR(acanthosis nigricans), abdominal ultrasounds, and serum transaminases, lipids and glucose/insulin profiles, allow a clinician to identify children with obesity and obesity-related conditions, including NAFLD and cardiovascular and metabolic risks. A liver biopsy(the "imperfect" gold standard) is required for a definitive NAFLD/NASH diagnosis, particularly to exclude other treatable conditions or when advanced liver disease is expected on clinical and laboratory grounds and preferably prior to any controlled trial of pharmacological/surgical treatments. However, a biopsy clearly cannot represent a screening procedure. Advancements in diagnostic serum and imaging tools, especially for the non-invasive differentiation between NAFLD and NASH, have shown promising results, e.g., magnetic resonance elastography. Weight loss and physical activity should be the first option of intervention.Effective pharmacological treatments are still under development; however, drugs targeting IR, oxidative stress, proinflammatory pathways, dyslipidemia, gut microbiota and gut liver axis dysfunction are an option for patients who are unable to comply with the recommended lifestyle changes. When morbid obesity prevails, bariatric surgery should be considered.展开更多
In the last years new evidence has accumulated on nonalcoholic fatty liver disease(NAFLD)challenging the paradigms that had been holding the scene over the previous 30 years.NAFLD has such an epidemic prevalence as to...In the last years new evidence has accumulated on nonalcoholic fatty liver disease(NAFLD)challenging the paradigms that had been holding the scene over the previous 30 years.NAFLD has such an epidemic prevalence as to make it impossible to screen general population looking for NAFLD cases.Conversely,focusing on those cohorts of individuals exposed to the highest risk of NAFLD could be a more rational approach.NAFLD,which can be diagnosed with either non-invasive strategies or through liver biopsy,is a pathogenically complex and clinically heterogeneous disease.The existence of metabolic as opposed to genetic-associated disease,notably including"lean NAFLD"has recently been recognized.Moreover,NAFLD is a systemic condition,featuring metabolic,cardiovascular and(hepatic/extrahepatic)cancer risk.Among the clinico-laboratory features of NAFLD we discuss hyperuricemia,insulin resistance,atherosclerosis,gallstones,psoriasis and selected endocrine derangements.NAFLD is a precursor of type 2 diabetes(T2D)and metabolic syndrome and progressive liver disease develops in T2D patients in whom the course of disease is worsened by NAFLD.Finally,lifestyle changes and drug treatment options to be implemented in the individual patient are also critically discussed.In conclusion,this review emphasizes the new concepts on clinical and pathogenic heterogeneity of NAFLD,a systemic disorder with a multifactorial pathogenesis and protean clinical manifestations.It is highly prevalent in certain cohorts of individuals who are thus potentially amenable to selective screening strategies,intensive follow-up schedules for early identification of liver-related and extrahepatic complications and in whom earlier and more aggressive treatment schedules should be carried out whenever possible.展开更多
BACKGROUND Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare tumors,often diagnosed in an advanced stage when curative treatment is impossible and grueling symptoms related to vasoactive substance rele...BACKGROUND Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare tumors,often diagnosed in an advanced stage when curative treatment is impossible and grueling symptoms related to vasoactive substance release by tumor cells affect patients’quality of life.Cardiovascular complications of GEP-NENs,primarily tricuspid and pulmonary valve disease,and right-sided heart failure,are the leading cause of death,even compared to metastatic disease.CASE SUMMARY We present a case of a 35-year-old patient with progressive dyspnea,back pain,polyneuropathic leg pain,and nocturnal diarrhea lasting for a decade before the diagnosis of neuroendocrine carcinoma of unknown primary with extensive liver metastases.During the initial presentation,serum biomarkers were not evaluated,and the patient received five cycles of doxorubicin,which he did not tolerate well,so he refused further therapy and was lost to follow-up.After 10 years,he presented to the emergency room with signs and symptoms of right-sided heart failure.Panneuroendocrine markers,serum chromogranin A,and urinary 5-hydroxyindoleacetic acid were extremely elevated(900 ng/mL and 2178µmol/L),and transabdominal ultrasound confirmed hepatic metastases.Computed tomo-graphy(CT)showed liver metastases up to 6 cm in diameter and metastases in mesenteric lymph nodes and pelvis.Furthermore,an Octreoscan showed lesions in the heart,thoracic spine,duodenum,and ascendent colon.A standard trans-thoracic echocardiogram confirmed findings of carcinoid heart disease.The patient was not a candidate for valve replacement.He started octreotide acetate treatment,and the dose escalated to 80 mg IM monthly.Although biochemical CONCLUSION Carcinoid heart disease occurs with carcinoid syndrome related to advanced neuroendocrine tumors,usually with liver metastases,which manifests as right-sided heart valve dysfunction leading to right-sided heart failure.Carcinoid heart disease and tumor burden are major prognostic factors of poor survival.Therefore,they must be actively sought by available biochemical markers and imaging techniques.Moreover,imaging techniques aiding tumor detection and staging,somatostatin receptor positron emission tomography/CT,and CT or magnetic resonance imaging,should be performed at the time of diagnosis and in 3-to 6-mo intervals to determine tumor growth rate and assess the possibility of locoregional therapy and/or palliative surgery.Valve replacement at the onset of symptoms or right ventricular dysfunction may be considered,while any delay can worsen right-sided ventricular failure.展开更多
Background Phlegm and blood stasis syndrome(PBSS) is one of the main syndromes in coronary heart disease(CHD). Syndromes of Chinese medicine(CM) are lack of quantitative and easyimplementation diagnosis standards. To ...Background Phlegm and blood stasis syndrome(PBSS) is one of the main syndromes in coronary heart disease(CHD). Syndromes of Chinese medicine(CM) are lack of quantitative and easyimplementation diagnosis standards. To quantify and standardize the diagnosis of PBSS, scales are usually applied. Objective: To evaluate the diagnostic accuracy of CM diagnosis scale of PBSS in CHD. Methods: Six hundred patients with stable angina pectoris of CHD, 300 in case group and 300 in control group, will be recruited from 5 hospitals across China. Diagnosis from 2 experts will be considered as the "gold standard". The study design consists of 2 phases: pilot test is used to evaluate the reliability and validity, and diagnostic test is used to assess the diagnostic accuracy of the scale, including sensitivity, specificity, likelihood ratio and area under the receiver operator characteristic(ROC) curve. Discussion: This study will evaluate the diagnostic accuracy of CM diagnosis scale of PBSS in CHD. The consensus of 2 experts may not be ideal as a "gold standard", and itself still requires further study.(No. ChiCTR-OOC-15006599).展开更多
Von Hippel-Lindau(VHL)综合征是由VHL肿瘤抑制基因失活、突变引起的一种以家族性血管母细胞瘤为特征,涉及多个器官的常染色体显性遗传性肿瘤综合征,具有较高的外显率及多种表达方式,临床罕见。诊断需依赖影像学及眼底镜等多项检查,基...Von Hippel-Lindau(VHL)综合征是由VHL肿瘤抑制基因失活、突变引起的一种以家族性血管母细胞瘤为特征,涉及多个器官的常染色体显性遗传性肿瘤综合征,具有较高的外显率及多种表达方式,临床罕见。诊断需依赖影像学及眼底镜等多项检查,基因检测是其诊断的金标准。目前手术切除仍是本病的主要治疗方法,但难以根治,易复发。生物疗法有望成为未来VHL综合征新的有效治疗方法。展开更多
Objective：To analyze the diagnostic consistency of Chinese medicine（CM） specialists in patients with cardiovascular disease and to study syndrome classification and identification based on the multi-label learning ...Objective：To analyze the diagnostic consistency of Chinese medicine（CM） specialists in patients with cardiovascular disease and to study syndrome classification and identification based on the multi-label learning method.Methods：Using self-developed CM clinical scales to collect cases,inquiry information,complexity,tongue manifestation and pulse manifestation were assessed.The number of cases collected was 2,218.Firstly,each case was differentiated by two CM specialists according to the same diagnostic criteria.The consistency of the diagnosis based on Cohen’s Kappa coefficient was analyzed.Secondly,take the same diagnosis syndromes of two specialists as the results of the cases.According to injury information in the CM scale ＂yes＂ or ＂no＂ was assigned ＂1＂ or ＂0＂,and according to the syndrome type in each case ＂yes＂ or ＂no＂ was assigned ＂1＂ or ＂0＂.CM information data on cardiovascular disease cases were established.We studied CM syndrome classification and identification based on the relevant feature for each label（REAL） leaming method,and the diagnostic rate of the syndrome was studied using the REAL method when the number of features selected was 5,10,15,20,30,50,70,and 100,respectively.Results：The syndromes with good diagnostic consistency were Heart（Xin）-qi deficiency,Heart-yang deficiency,Heart-yin deficiency,phlegm,stagnation of blood and stagnation of qi.Syndromes with poor diagnostic consistency were heartblood deficiency and blood deficiency of Heart and Liver（Gan）.The highest diagnostic rates using the REAL method were Heart-yang deficiency followed by Heart-qi deficiency.A different number of features,such as 5,10,15,20,30,40,50,70,and 100,respectively,were selected and the diagnostic accuracy based on five features showed the highest diagnostic accuracy.The top five features which had a strong correlation with the syndromes were in accordance with the CM theory.Conclnsions：CM syndrome differentiation is strongly subjective and it is difficult to obtain good diagnostic consistency.The REAL method fully considers the relationship between syndrome types and injury symptoms,and is suitable for the establishment of models for CM syndrome classification and identification.This method can probably provide the prerequisite for objectivity and standardization of CM differentiation.展开更多
Posterior reversible encephalopathy syndrome(PRES)is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings.PRES occurs in the setting of various clinical conditio...Posterior reversible encephalopathy syndrome(PRES)is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings.PRES occurs in the setting of various clinical conditions and requires prompt management of the causative factor for a full recovery.This is a case report of a Crohn’s disease patient who developed PRES syndrome during a complicated post-operative course.In the presence of multiple causative factors,sepsis was considered as the predominant one.After prompt management,the patient recovered with no permanent neurological damage.展开更多
Objective To analyze the characteristics of tongue imaging color parameters in patients treated with percutaneous coronary intervention(PCI)and non-PCI for coronary atherosclerotic heart disease(CHD),and to observethe...Objective To analyze the characteristics of tongue imaging color parameters in patients treated with percutaneous coronary intervention(PCI)and non-PCI for coronary atherosclerotic heart disease(CHD),and to observethe effects of PCI on the tongue images of patients as a basis for the clinical diagnosis and treatment of patientswith CHD.Methods This study used a retrospective cross-sectional survey to analyze tongue photographs and medicalhistory information from 204 patients with CHD between November 2018 and July 2020.Tongue images ofeach subject were obtained using the Z-BOX Series traditional Chinese medicine(TCM)intelligent diagnosisinstruments,the SMX System 2.0 was used to transform the image data into parameters in the HSV color space,and finally the parameters of the tongue image between patients in the PCI-treated and non-PCI-treated groupsfor CHD were analyzed.Results Among the 204 patients,112 were in the non-PCI treatment group(38 men and 74 women;average age of(68.76±9.49)years),92 were in the PCI treatment group(66 men and 26 women;average age of(66.02±10.22)years).In the PCI treatment group,the H values of the middle and tip of the tongue and the overall coating of thetongue were lower(P<0.05),while the V values of the middle,tip,both sides of the tongue,the whole tongueand the overall coating of the tongue were higher(P<0.05).Conclusion The color parameters of the tongue image could reflect the physical state of patients treated withPCI,which may provide a basis for the clinical diagnosis and treatment of patients with CHD.展开更多
文摘Great advances have been achieved in the integration of western and traditional Chinese medicine (TCM).More and more TCM physicians in their practice accept the term 'differential diagnosis of the diseases' instead of the syndromes since western medicine has been introduced into the TCM circle.
文摘Non-alcoholic fatty liver disease(NAFLD) is the most frequent cause of liver disease in the Western world. Furthermore, it is increasing worldwide, paralleling the obesity pandemic. Though highly frequent, only about one fifth of affected subjects are at risk of developing the progressive form of the disease, non-alcoholic steatohepatitis with fibrosis. Even in the latter, liver disease is slowly progressive, though, since it is so prevalent, it is already the third cause of liver transplantation in the United States, and it is predicted to get to the top of the ranking in few years. Of relevance, fatty liver is also associated with increased overall mortality and particularly increased cardiovascular mortality. The literature and amount of published papers on NAFLD is increasing as fast as its prevalence, which makes it difficult to keep updated in this topic. This review aims to summarize the latest knowledge on NAFLD, in order to help clinicians understanding its pathogenesis and advances on diagnosis and treatment.
文摘Vanishing bone disease(Gorham-Stout syndrome) is a rare entity of unknown etiology, characterized by de struction of osseous matrix and proliferation of vascula structures, resulting in destruction and absorption o bone. Despite the extensive investigation of the patho genetic mechanisms of the disease, its etiology hasn'been clarified and several theories exist. The syndrome can affect one or multiple bones of the patient, includ ing the skull, the upper and lower extremities, the spine and pelvis. The clinical presentation of a patient suffer ing from vanishing bone disease includes, pain, func tional impairment and swelling of the affected region although asymptomatic cases have been reported, as well as cases in which the diagnosis was made after a pathologic fracture. In this short review we summarize the theories regarding the etiology as well as the clini cal presentation, the diagnostic approach and treat ment options of this rare disease.
基金This study was supported by the National Natural Science Foundation of China,Nos.81801155(to YL),81771237(to YXG)the New Technology Projects of Shanghai Science and Technology Innovation Action Plan,China,No.18511102800(to YXG)+1 种基金the Shanghai Municipal Science and Technology Major Project and ZJLab,China,No.2018SHZDZX01(to YM)the Shanghai Health and Family Planning Commission,China,No.2017BR022(to YXG).
文摘Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,evaluating the weight of different factors,and quantitatively evaluating the risk of intracranial hemorrhage in moyamoya disease.To investigate whether convolutional neural network algorithms can be used to recognize moyamoya disease and predict hemorrhagic episodes,we retrospectively selected 460 adult unilateral hemispheres with moyamoya vasculopathy as positive samples for diagnosis modeling,including 418 hemispheres with moyamoya disease and 42 hemispheres with moyamoya syndromes.Another 500 hemispheres with normal vessel appearance were selected as negative samples.We used deep residual neural network(ResNet-152)algorithms to extract features from raw data obtained from digital subtraction angiography of the internal carotid artery,then trained and validated the model.The accuracy,sensitivity,and specificity of the model in identifying unilateral moyamoya vasculopathy were 97.64±0.87%,96.55±3.44%,and 98.29±0.98%,respectively.The area under the receiver operating characteristic curve was 0.990.We used a combined multi-view conventional neural network algorithm to integrate age,sex,and hemorrhagic factors with features of the digital subtraction angiography.The accuracy of the model in predicting unilateral hemorrhagic risk was 90.69±1.58%and the sensitivity and specificity were 94.12±2.75%and 89.86±3.64%,respectively.The deep learning algorithms we proposed were valuable and might assist in the automatic diagnosis of moyamoya disease and timely recognition of the risk for re-hemorrhage.This study was approved by the Institutional Review Board of Huashan Hospital,Fudan University,China(approved No.2014-278)on January 12,2015.
文摘The novel severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)that suddenly emerged at the end of December 2019 and caused coronavirus disease 2019(COVID-19)continues to afflict humanity,not only seriously affecting healthcare systems but also leading to global social and economic imbalances.As of August 2022,there were approximately 580 million confirmed cases of COVID-19 and approximately 6.4 million confirmed deaths due to this disease.The data are sufficient to highlight the seriousness of SARS-CoV-2 infection.Although most patients with COVID-19 present primarily with respiratory symptoms,an increasing number of extrapulmonary systemic symptoms and manifestations have been associated with COVID-19.Since the outbreak of COVID-19,much has been learned about the disease and its causative agent.Therefore,great effort has been aimed at developing treatments and drug interventions to treat and reduce the incidence of COVID-19.In this narrative review,we provide a brief overview of the epidemiology,mechanisms,clinical manifestations,diagnosis,and therapeutics of COVID-19.
基金Supported by the Key Medical Projects of Technical College Development of Shijingshan District.
文摘BACKGROUND Castleman disease(CD)and TAFRO syndrome are very rare in clinical practice.Most clinicians,especially non-hematological clinicians,do not know enough about the two diseases,so it often leads to misdiagnosis or missed diagnosis.AIM To explore the clinical features and diagnosis of CD and TAFRO syndrome.METHODS We retrospectively collected the clinical and laboratory data of 39 patients who were diagnosed with CD from a single medical center.RESULTS Clinical classification identified 18 patients(46.15%)with unicentric Castleman disease(UCD)and 21 patients(53.85%)with multicentric Castleman disease(MCD),the latter is further divided into 13 patients(33.33%)with idiopathic multicentric Castleman disease-not otherwise specified(iMCD-NOS)and 8 patients(20.51%)with TAFRO syndrome.UCD and iMCD are significantly different in clinical manifestations,treatment,and prognosis.However,a few patients with MCD were diagnosed as UCD in their early stage.There was a correlation between two of Thrombocytopenia,anasarca and elevated creatinine,which were important components of TAFRO syndrome.In UCD group,the pathologies of lymph modes were mostly hyaline vascular type(13/18,72.22%),however plasma cell type or mixed type could also appear.In iMCD-NOS group and TAFRO syndrome group,the pathologies of lymph mode shown polarity of plasma cell type and hyaline vascular type respectively.Compared with patients with TAFRO syndrome,patients with iMCD-NOS were diagnosed more difficultly.CONCLUSION The clinical and pathological types of CD are not completely separate,there is an intermediate situation or mixed characteristics between two ends of clinical and pathological types.The clinical manifestations of patients with CD are determined by their pathological type.TAFRO syndrome is a special subtype of iMCD with unique clinical manifestations.
文摘Non-alcoholic fatty liver disease(NAFLD) in children is becoming a major health concern. A "multiple-hit" pathogenetic model has been suggested to explain the progressive liver damage that occurs among children with NAFLD. In addition to the accumulation of fat in the liver, insulin resistance(IR) and oxidative stress due to genetic/epigenetic background, unfavorable lifestyles, gut microbiota and gut-liver axis dysfunction, and perturbations of trace element homeostasis have been shown to be critical for disease progression and the development of more severe inflammatory and fibrotic stages [non-alcoholic steatohepatitis(NASH)]. Simple clinical and laboratory parameters, such as age, history, anthropometrical data(BMI and waist circumference percentiles), blood pressure, surrogate clinical markers of IR(acanthosis nigricans), abdominal ultrasounds, and serum transaminases, lipids and glucose/insulin profiles, allow a clinician to identify children with obesity and obesity-related conditions, including NAFLD and cardiovascular and metabolic risks. A liver biopsy(the "imperfect" gold standard) is required for a definitive NAFLD/NASH diagnosis, particularly to exclude other treatable conditions or when advanced liver disease is expected on clinical and laboratory grounds and preferably prior to any controlled trial of pharmacological/surgical treatments. However, a biopsy clearly cannot represent a screening procedure. Advancements in diagnostic serum and imaging tools, especially for the non-invasive differentiation between NAFLD and NASH, have shown promising results, e.g., magnetic resonance elastography. Weight loss and physical activity should be the first option of intervention.Effective pharmacological treatments are still under development; however, drugs targeting IR, oxidative stress, proinflammatory pathways, dyslipidemia, gut microbiota and gut liver axis dysfunction are an option for patients who are unable to comply with the recommended lifestyle changes. When morbid obesity prevails, bariatric surgery should be considered.
文摘In the last years new evidence has accumulated on nonalcoholic fatty liver disease(NAFLD)challenging the paradigms that had been holding the scene over the previous 30 years.NAFLD has such an epidemic prevalence as to make it impossible to screen general population looking for NAFLD cases.Conversely,focusing on those cohorts of individuals exposed to the highest risk of NAFLD could be a more rational approach.NAFLD,which can be diagnosed with either non-invasive strategies or through liver biopsy,is a pathogenically complex and clinically heterogeneous disease.The existence of metabolic as opposed to genetic-associated disease,notably including"lean NAFLD"has recently been recognized.Moreover,NAFLD is a systemic condition,featuring metabolic,cardiovascular and(hepatic/extrahepatic)cancer risk.Among the clinico-laboratory features of NAFLD we discuss hyperuricemia,insulin resistance,atherosclerosis,gallstones,psoriasis and selected endocrine derangements.NAFLD is a precursor of type 2 diabetes(T2D)and metabolic syndrome and progressive liver disease develops in T2D patients in whom the course of disease is worsened by NAFLD.Finally,lifestyle changes and drug treatment options to be implemented in the individual patient are also critically discussed.In conclusion,this review emphasizes the new concepts on clinical and pathogenic heterogeneity of NAFLD,a systemic disorder with a multifactorial pathogenesis and protean clinical manifestations.It is highly prevalent in certain cohorts of individuals who are thus potentially amenable to selective screening strategies,intensive follow-up schedules for early identification of liver-related and extrahepatic complications and in whom earlier and more aggressive treatment schedules should be carried out whenever possible.
文摘BACKGROUND Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare tumors,often diagnosed in an advanced stage when curative treatment is impossible and grueling symptoms related to vasoactive substance release by tumor cells affect patients’quality of life.Cardiovascular complications of GEP-NENs,primarily tricuspid and pulmonary valve disease,and right-sided heart failure,are the leading cause of death,even compared to metastatic disease.CASE SUMMARY We present a case of a 35-year-old patient with progressive dyspnea,back pain,polyneuropathic leg pain,and nocturnal diarrhea lasting for a decade before the diagnosis of neuroendocrine carcinoma of unknown primary with extensive liver metastases.During the initial presentation,serum biomarkers were not evaluated,and the patient received five cycles of doxorubicin,which he did not tolerate well,so he refused further therapy and was lost to follow-up.After 10 years,he presented to the emergency room with signs and symptoms of right-sided heart failure.Panneuroendocrine markers,serum chromogranin A,and urinary 5-hydroxyindoleacetic acid were extremely elevated(900 ng/mL and 2178µmol/L),and transabdominal ultrasound confirmed hepatic metastases.Computed tomo-graphy(CT)showed liver metastases up to 6 cm in diameter and metastases in mesenteric lymph nodes and pelvis.Furthermore,an Octreoscan showed lesions in the heart,thoracic spine,duodenum,and ascendent colon.A standard trans-thoracic echocardiogram confirmed findings of carcinoid heart disease.The patient was not a candidate for valve replacement.He started octreotide acetate treatment,and the dose escalated to 80 mg IM monthly.Although biochemical CONCLUSION Carcinoid heart disease occurs with carcinoid syndrome related to advanced neuroendocrine tumors,usually with liver metastases,which manifests as right-sided heart valve dysfunction leading to right-sided heart failure.Carcinoid heart disease and tumor burden are major prognostic factors of poor survival.Therefore,they must be actively sought by available biochemical markers and imaging techniques.Moreover,imaging techniques aiding tumor detection and staging,somatostatin receptor positron emission tomography/CT,and CT or magnetic resonance imaging,should be performed at the time of diagnosis and in 3-to 6-mo intervals to determine tumor growth rate and assess the possibility of locoregional therapy and/or palliative surgery.Valve replacement at the onset of symptoms or right ventricular dysfunction may be considered,while any delay can worsen right-sided ventricular failure.
基金Supported by National Basic Research Program of China(973 Program,No.2014CB542901)
文摘Background Phlegm and blood stasis syndrome(PBSS) is one of the main syndromes in coronary heart disease(CHD). Syndromes of Chinese medicine(CM) are lack of quantitative and easyimplementation diagnosis standards. To quantify and standardize the diagnosis of PBSS, scales are usually applied. Objective: To evaluate the diagnostic accuracy of CM diagnosis scale of PBSS in CHD. Methods: Six hundred patients with stable angina pectoris of CHD, 300 in case group and 300 in control group, will be recruited from 5 hospitals across China. Diagnosis from 2 experts will be considered as the "gold standard". The study design consists of 2 phases: pilot test is used to evaluate the reliability and validity, and diagnostic test is used to assess the diagnostic accuracy of the scale, including sensitivity, specificity, likelihood ratio and area under the receiver operator characteristic(ROC) curve. Discussion: This study will evaluate the diagnostic accuracy of CM diagnosis scale of PBSS in CHD. The consensus of 2 experts may not be ideal as a "gold standard", and itself still requires further study.(No. ChiCTR-OOC-15006599).
基金Supported by the National Natural Science Foundation of China(No.81173199)
文摘Objective：To analyze the diagnostic consistency of Chinese medicine（CM） specialists in patients with cardiovascular disease and to study syndrome classification and identification based on the multi-label learning method.Methods：Using self-developed CM clinical scales to collect cases,inquiry information,complexity,tongue manifestation and pulse manifestation were assessed.The number of cases collected was 2,218.Firstly,each case was differentiated by two CM specialists according to the same diagnostic criteria.The consistency of the diagnosis based on Cohen’s Kappa coefficient was analyzed.Secondly,take the same diagnosis syndromes of two specialists as the results of the cases.According to injury information in the CM scale ＂yes＂ or ＂no＂ was assigned ＂1＂ or ＂0＂,and according to the syndrome type in each case ＂yes＂ or ＂no＂ was assigned ＂1＂ or ＂0＂.CM information data on cardiovascular disease cases were established.We studied CM syndrome classification and identification based on the relevant feature for each label（REAL） leaming method,and the diagnostic rate of the syndrome was studied using the REAL method when the number of features selected was 5,10,15,20,30,50,70,and 100,respectively.Results：The syndromes with good diagnostic consistency were Heart（Xin）-qi deficiency,Heart-yang deficiency,Heart-yin deficiency,phlegm,stagnation of blood and stagnation of qi.Syndromes with poor diagnostic consistency were heartblood deficiency and blood deficiency of Heart and Liver（Gan）.The highest diagnostic rates using the REAL method were Heart-yang deficiency followed by Heart-qi deficiency.A different number of features,such as 5,10,15,20,30,40,50,70,and 100,respectively,were selected and the diagnostic accuracy based on five features showed the highest diagnostic accuracy.The top five features which had a strong correlation with the syndromes were in accordance with the CM theory.Conclnsions：CM syndrome differentiation is strongly subjective and it is difficult to obtain good diagnostic consistency.The REAL method fully considers the relationship between syndrome types and injury symptoms,and is suitable for the establishment of models for CM syndrome classification and identification.This method can probably provide the prerequisite for objectivity and standardization of CM differentiation.
文摘Posterior reversible encephalopathy syndrome(PRES)is a clinico-ragiological syndrome presenting with neurological symptoms and characteristic radiologic findings.PRES occurs in the setting of various clinical conditions and requires prompt management of the causative factor for a full recovery.This is a case report of a Crohn’s disease patient who developed PRES syndrome during a complicated post-operative course.In the presence of multiple causative factors,sepsis was considered as the predominant one.After prompt management,the patient recovered with no permanent neurological damage.
基金This study was supported by the National Natural Science Foundation of China(Grant No.82074333)Shanghai TCM Science and Technology Innovation Program(Grant No.ZYKC201701017)Shanghai Key Laboratory of Health Identification and Assessment(Grant No.21DZ2271000).
文摘Objective To analyze the characteristics of tongue imaging color parameters in patients treated with percutaneous coronary intervention(PCI)and non-PCI for coronary atherosclerotic heart disease(CHD),and to observethe effects of PCI on the tongue images of patients as a basis for the clinical diagnosis and treatment of patientswith CHD.Methods This study used a retrospective cross-sectional survey to analyze tongue photographs and medicalhistory information from 204 patients with CHD between November 2018 and July 2020.Tongue images ofeach subject were obtained using the Z-BOX Series traditional Chinese medicine(TCM)intelligent diagnosisinstruments,the SMX System 2.0 was used to transform the image data into parameters in the HSV color space,and finally the parameters of the tongue image between patients in the PCI-treated and non-PCI-treated groupsfor CHD were analyzed.Results Among the 204 patients,112 were in the non-PCI treatment group(38 men and 74 women;average age of(68.76±9.49)years),92 were in the PCI treatment group(66 men and 26 women;average age of(66.02±10.22)years).In the PCI treatment group,the H values of the middle and tip of the tongue and the overall coating of thetongue were lower(P<0.05),while the V values of the middle,tip,both sides of the tongue,the whole tongueand the overall coating of the tongue were higher(P<0.05).Conclusion The color parameters of the tongue image could reflect the physical state of patients treated withPCI,which may provide a basis for the clinical diagnosis and treatment of patients with CHD.
