Achalasia cardia,type of esophageal dynamic disorder,is a relatively rare primary motor esophageal disease characterized by the functional loss of plexus ganglion cells in the distal esophagus and lower esophageal sph...Achalasia cardia,type of esophageal dynamic disorder,is a relatively rare primary motor esophageal disease characterized by the functional loss of plexus ganglion cells in the distal esophagus and lower esophageal sphincter.Loss of function of the distal and lower esophageal sphincter ganglion cells is the main cause of achalasia cardia,and is more likely to occur in the elderly.Histological changes in the esophageal mucosa are considered pathogenic;however,studies have found that inflammation and genetic changes at the molecular level may also cause achalasia cardia,resulting in dysphagia,reflux,aspiration,retrosternal pain,and weight loss.Currently,the treatment options for achalasia focus on reducing the resting pressure of the lower esophageal sphincter,helping to empty the esophagus and relieve symptoms.Treatment measures include botulinum toxin injection,inflatable dilation,stent insertion,and surgical myotomy(open or laparoscopic).Surgical procedures are often subject to controversy owing to concerns about safety and effectiveness,particularly in older patients.Herein,we review clinical epidemiological and experimental data to determine the prevalence,pathogenesis,clinical presentation,diagnostic criteria,and treatment options for achalasia to support its clinical management.展开更多
Solitary rectal ulcer syndrome (SRUS) is an uncommon benign disease, characterized by a combination of symptoms, clinical findings and histological abnormalities. Ulcers are only found in 40% of the patients; 20% of t...Solitary rectal ulcer syndrome (SRUS) is an uncommon benign disease, characterized by a combination of symptoms, clinical findings and histological abnormalities. Ulcers are only found in 40% of the patients; 20% of the patients have a solitary ulcer, and the rest of the lesions vary in shape and size, from hyperemic mucosa to broad-based polypoid. Men and women are affected equally, with a small predominance in women. SRUS has also been described in children and in the geriatric population. Clinical features include rectal bleeding, copious mucus discharge, prolonged excessive straining, perineal and abdominal pain, feeling of incomplete defecation, constipation, and rarely, rectal prolapse. This disease has well-described histopathological features such as obliteration of the lamina propria by fibrosis and smooth muscle fibers extending from a thickened muscularis mucosa to the lumen. Diffuse collage deposition in the lamina propria and abnormal smooth muscle fiber extensions are sensitive markers for differ-entiating SRUS from other conditions. However, the etiology remains obscure, and the condition is frequently associated with pelvic floor disorders. SRUS is difficult to treat, and various treatment strategies have been advocated, ranging from conservative management to a variety of surgical procedures. The aim of the present review is to summarize the clinical features, pathophysiology, diagnostic methods and treatment strategies associated with SRUS. (C) 2014 Baishideng Publishing Group Co., Limited. All rights reserved.展开更多
BACKGROUND Hepatic epithelioid hemangioendothelioma(HEHE)is a rare hepatic vascular tumor with unpredictable malignant potential.The etiology,characteristics,diagnosis,treatment,and prognosis of HEHE are not well-unde...BACKGROUND Hepatic epithelioid hemangioendothelioma(HEHE)is a rare hepatic vascular tumor with unpredictable malignant potential.The etiology,characteristics,diagnosis,treatment,and prognosis of HEHE are not well-understood,and largescale retrospective studies are required to understand better this disease.AIM To determine the characteristics of HEHE and identify its optimal treatments and prognostic factors.METHODS The clinical data of two patients diagnosed with HEHE at the Fourth Hospital of Hebei Medical University and 258 previously reported cases retrieved from the China National Knowledge Infrastructure and PubMed databases between 1996 and 2021 were combined and summarized.All cases were pathologically identified as HEHE.Information such as clinical features,laboratory examination findings,imaging findings,pathological characteristics,treatment,and survival periods was reviewed.Kaplan-Meir curves were used for survival analysis.Prognostic factors were identified by Cox regression analysis.RESULTS HEHE primarily affected middle-aged women.The typical manifestations included epigastric pain,hepatosplenomegaly,inappetence,distension,weight loss,and fatigue.Tumor markers were expressed normally.The incidence of extrahepatic metastasis was 34.5% at the time of diagnosis.The most common sites of extrahepatic involvement were the lungs(22.3%),lymph nodes(5.6%),peritoneum(3.6%),bones(6.6%),and spleen(5.1%).Furthermore,“capsular retraction”,“target sign”,and“lollipop sign”were the characteristic features of HEHE on imaging.The immunohistochemical profile for HEHE(expression of vascular markers,such as factor VIII-related antigen,CD31,and CD34;expression levels of D2-40)can facilitate and ensure an accurate diagnosis.The management options for patients with HEHE include liver resection(29.7%),liver transplantation(16.1%),palliative treatments(12.7%),transhepatic arterial chemotherapy and embolization(TACE,10.2%),chemotherapy(11.0%),antiangiogenic therapy(15.3%),and other treatments(5.1%);the mean survival time was 158.6,147.3,4.2,90.8,71.4,83.1,and 55.0 mo,respectively.The survival time of patients who underwent surgical treatment was longer than that of patients who did not.TACE and antiangiogenic therapy tended to prolong survival compared with other nonsurgical treatments.The 1-,5-,and 10-year survival rates were 82%,71%,and 64%,respectively.Multivariate analysis showed that liver function(P=0.045),intrahepatic metastasis(P=0.029),and treatment(P=0.045)were independent prognostic factors.The presence of extrahepatic metastases was not an independent risk factor for poor prognosis(P=0.558).CONCLUSION The clinical course of HEHE is rare and variable,and patients with intrahepatic metastases and liver dysfunction may have a poorer prognosis than those without.Surgical intervention,whether liver resection or transplantation,might be warranted regardless of extrahepatic metastasis.For patients without the option for surgery,clinicians should consider the use of TACE with antiangiogenic drugs in the treatment of HEHE.展开更多
Objective: To explore senile brucellosis spondylitis clinical features and diagnostic criteria, in order to improve the diagnosis rate and evaluate the clinical effects of treatment strategies. Methods: From January 2...Objective: To explore senile brucellosis spondylitis clinical features and diagnostic criteria, in order to improve the diagnosis rate and evaluate the clinical effects of treatment strategies. Methods: From January 2002 to August 2015, 62 patients with Brucella-associated spondylitis were treated with comprehensive diagnosis based on epidemiological history, clinical manifestations, imaging findings, laboratory tests, and local biopsy. The positive rate of red plate agglutination test (RBPT) was 45.1%, the positive rate of serum tube agglutination test (SAT) was 51.6%, and the positive rate of brucellosis anti-human immunoglobulin test (Coomb’s) was 100%. All patients underwent X-ray, CT and MRI examinations. The positive rate of blood culture in pathogenic examination was 16.1%, and the positive rate of bone marrow culture was 38.5%. The positive rate of inflammatory granuloma or abscess in the lesion sampling culture was 73.6%. All patients underwent standardized drug therapy and hyperbaric oxygen adjuvant therapy. Among them, 38 patients with neurological impairment were treated with one-stage debridement and posterior pedicle screw fixation on the basis of drug therapy. The paraspinal abscess and the diseased in-tervertebral space tissue were taken for pathological examination during the operation. One week, two weeks, one month, three months, six months and 12 months after treatment, the patients were evaluated and followed up at the monitoring sites. The evaluation indicators were pain scores, activity of daily living (ADL), imaging findings, and laboratory tests. The SPSS15.0 statistical package was used for analysis. Results: All patients were confirmed by the above-mentioned comprehensive examination after admission. 24 patients (38.71%) were treated with standard drug therapy (group A), no adverse drug reactions and abnormal liver and kidney function;the remaining 38 patients (61.29%) were combined with varying degrees of neurological impairment, and surgical treatment was performed after 2 to 4 weeks of drug therapy without improvement (group B). The pain scores showed that there was a statistically significant difference between the two groups at the same time point (P Conclusion: The senile Brucellosis spondylitis has a characteristic performance. The de-velopment of diagnostic criteria can help to improve the diagnostic rate. Standardized drug treatment has a good cure rate. The implementation of surgery has obvious advantages whether it is to relieve pain, stabilize the spine, restore nerve function, or recover early.展开更多
Diabetic foot(DF)is one of the most common complications of diabetes and is associated with high morbidity,disability,lethality and low cure-rate.The clinical diagnosis and treatment of DF need to be standardized.The ...Diabetic foot(DF)is one of the most common complications of diabetes and is associated with high morbidity,disability,lethality and low cure-rate.The clinical diagnosis and treatment of DF need to be standardized.The Chinese Diabetic Foot Cell and Interventional Therapy Technology Alliance has released six editions of guidelines and standards for clinical diagnosis and interventional treatment of DF,which filled the gap in the domestic DF treatment standard and played an important role in improving the level of diagnosis and treatment in China.In line with the latest developments in diagnosis and treatment,the Alliance,along with other 89 institutions,developed and issued the new edition based on the sixth edition to help standardize the clinical diagnosis and treatment of DF in China.展开更多
BACKGROUND Since December 2019,many cases of pneumonia caused by novel coronavirus have been discovered in Wuhan,China,and such cases have spread nationwide quickly.At present,coronavirus disease 2019(COVID-19)is a wo...BACKGROUND Since December 2019,many cases of pneumonia caused by novel coronavirus have been discovered in Wuhan,China,and such cases have spread nationwide quickly.At present,coronavirus disease 2019(COVID-19)is a worldwide pandemic.What are the clinical features of this disease?What is the clinical diagnosis and how should such patients be treated?As a clinician,mastery of the clinical characteristics,basic diagnosis,and treatment methods of COVID-19 are required to provide help to patients.CASE SUMMARY A 42-year-old male patient with a cough lasting 6 d without obvious cause,as well as fever and fatigue for 1 d,was admitted to Hankou Hospital on January 22,2020 and transferred to Huoshenshan Hospital on February 4.The main clinical symptoms were dry cough,fatigue,and fever.He was diagnosed with COVID-19.From the 4th d of admission,the patient’s condition gradually worsened,with increased respiratory rate and body temperature.Peripheral blood lymphocytes decreased progressively.On the 8th d of admission,the patient’s highest temperature was 40.7°C,and oxygen saturation was 83%despite high-flow oxygen inhalation.Chest computed tomography results showed that the virus progressed rapidly.The number of lesions significantly increased with expanded scope and increased density.The distribution of lesions advanced from peripheral to central.In addition to nasal catheter oxygen inhalation and symptomatic support,antiviral drugs were used throughout the treatment.On January 22,oseltamivir phosphate capsules were given orally(75 mg,twice daily)for 6 d.On January 24,three tablets of lopinavir and ritonavir were added orally(twice daily).After 6 d,this was changed to 0.2 g(two tablets)arbidol,taken orally(three times daily)for 5 d.During the severe stage,methylprednisolone was given(40 mg)once every 12 h,immunoglobulin(20 g)was administered by intravenous drip infusion once daily,and thymosin(1.6 mg)was injected subcutaneously once daily combined with immunotherapy.On February 2,symptoms decreased,various indicators improved,and pulmonary inflammation was obviously reduced.Throat swabs on February 4 and 9 were negative for novel coronavirus nucleic acid.After 19 d in the hospital,the patient was successfully treated and discharged.CONCLUSION COVID-19 in young adults can be successfully treated with active treatment.We report a typical case of COVID-19,analyze its clinical characteristics,summarize its clinical diagnosis and treatment experience,and provide a reference for clinical colleagues.展开更多
Objective:To investigate the relationship between clinical manifestations,blood testing,chest CT,treatment and fever of 457mild cases of COVID-19.Methods:A tatal of 457cases of mild COVID-19 diagnosed in Wuhan Jiangha...Objective:To investigate the relationship between clinical manifestations,blood testing,chest CT,treatment and fever of 457mild cases of COVID-19.Methods:A tatal of 457cases of mild COVID-19 diagnosed in Wuhan Jianghan module Hospital from February 6,2020 to February 26,2020 were gathered,and the cases with/without fever were retrospectively analyzed in epidemiology,clinical manifestation,blood routing,hypersensitive C-reactive protein(hs-CRP),pathogens serological testing,chest CT and drugs treatment.Results:For 312 fever cases,the average age was(47.6±10.9)years old,male was 128 cases(41%),the mean peak fever was 38(36.8,38.6)℃,fever lasting was 2(0,3)days,131cases(28.7%)had Huanan seafood market exposure history,90 cases(28.8%)had closely contact with COVID-19 patients,20cases(6.4%)had hypertension history,10 cases(3.2%)had diabetes,5cases(1.6%)had coronary heart disease.Main symptoms of fever group including dry cough of 260cases(83.3%),of 104cases(33.3%),blood sputum of 9cases(2.9%),chest tightness 20cases(19.6%),shortness of breath of 87cases(27.9%),weak of 105cases(33.7%),diarrhea of 43cases(13.8%),sleep disorders of 61cases(19.6%),among fever cases with sputum and shortness of breath more rather without fever,and two groups were statistically significantly difference(P<0.05).In fever group,WBC(109/L)[4.34(3.69,5.26)than 5.11(4.22,6.22),P<0.001],NEUT(109/L)[2.67(2.1,3.49)than 2.88(2.37,3.9),P<0.05],LYM(109/L)[0.9(0.8,1.1)than 1.36(1.03,1.85),P<0.001),PLT(109/L)/(106,188)134-189(132,224),P<0.001)were lower than normal group,while WBC<4.0×109/L[108(34.6%)than 24(16.6%),P<0.001)],LYM<1.0×109/L[189(60.6%)than 31(21.4%),P<0.001)],PLT<100×109/L[45(14.4%)than in 2(1.4%),P<0.001)and hs-CRP(mg/L)[14(6.83,32)than 3.4(0.96,10.75),P<0.001)were higher than normal group,and pathogens serological examination of 36cases shown influenzaⅠ/Ⅱvirus IgM antibody positive in 3cases(8.3%),respiratory syncytial virus IgM antibody positive in 2 cases(5.6%),mycoplasma pneumoniae IgM antibody positive(11.1%),4 cases of adenovirus IgM antibody positive(11.1%),but 4 pathogens of fever group and normal group were no statistical difference(P>0.05).According to early onset of chest CT examination,there were no change of 11cases(2.4%),unilateral lung lesions of 93cases(20.4%),bilateral lung lesions of 353 cases(77.2%),ground glass shadow of 228 cases(73.1%),high density shadow patch of 65 cases(20.8%),consolidation shadow of 6 cases(1.9%),while fever group compared with normal group,there were no statistically significant difference(P>0.05).Logistic regression analysis showed that LYM(OR=0.24,95%CI=0.139~0.415,P<0.001),LYM<1.0 x 109/L(OR=5.12,95%CI=3.07~8.65,P<0.001),PLT<100×109/L(OR=6.1,95%CI=1.36~27.33,P<0.05),and the hs-CRP(OR=1.03,95%CI=1.02~1.05,P<0.001)were fever independent impact factors in mild COVID-19.Aiming at COVID-19 treatment,Chinese medicine usage had 416cases(91%),antiviral drugs had 328cases(71.8%),and antibiotics had 172cases(37.6%),furthermore,fever group using antiviral and antibiotic drugs was higher than normal group(P<0.05),and compared to low fever group,high fever group used more antibiotic drugs(P<0.05).Conclusions:Mild COVID-19 patients with early onset had epidemiological characteristics,and fever,dry cough,expectoration and shortness of breath were main symptoms,and chest CT often involved bilateral lung lesions,ground glass shadow and patch density shadow.The fever patients in WBC,LYM,PLT,hs-CRP index changed significantly and could affect on anti-infective therapy selection.展开更多
BACKGROUND Gallbladder neuroendocrine carcinoma(GB-NEC)has a low incidence rate;therefore,its clinical characteristics,diagnosis,treatment and prognosis are not well explored.AIM To review recent research and analyze ...BACKGROUND Gallbladder neuroendocrine carcinoma(GB-NEC)has a low incidence rate;therefore,its clinical characteristics,diagnosis,treatment and prognosis are not well explored.AIM To review recent research and analyze corresponding data in the Surveillance Epidemiology and End Results(SEER)database.METHODS Data of GB-NEC(n=287)and gallbladder adenocarcinoma(GB-ADC)(n=19484)patients from 1975 to 2016 were extracted from the SEER database.Survival analysis was performed using Kaplan–Meier and Cox proportional hazards regression.P<0.05 was considered statistically significant.We also reviewed 108 studies retrieved from PubMed and Reference Citation Analysis(https://www.referencecitationanalysis.com/).The keywords used for the search were:"(Carcinoma,Neuroendocrine)AND(Gallbladder Neoplasms)".RESULTS The GB-NEC incidence rate was 1.6%(of all gallbladder carcinomas),male to female ratio was 1:2 and the median survival time was 7 mo.The 1-,2-,3-and 5-year overall survival(OS)was 36.6%,17.8%,13.2%and 7.3%respectively.Serum chromogranin A levels may be a specific tumor marker for the diagnosis of GBNEC.Elevated carcinoembryonic antigen,carbohydrate antigen(CA)-19-9 and CA-125 levels were associated with poor prognosis.Age[hazard ratio(HR)=1.027,95%confidence interval(CI):1.006–1.047,P=0.01]and liver metastasis(HR=3.055,95%CI:1.839–5.075,P<0.001)are independent prognostic risk factors for OS.Patients with advanced GB-NEC treated with surgical resection combined with radiotherapy and/or chemotherapy may have a better prognosis than those treated with surgical resection alone.There was no significant difference in OS between GB-NEC and GB-ADC.CONCLUSION The clinical manifestations and prognosis of GB-NEC are similar to GB-ADC,but the treatment is completely different.Early diagnosis and treatment are the top priorities.展开更多
BACKGROUND Cervical myeloid sarcoma(MS)is a rare hematological malignancy characterized by the formation of extramedullary soft tissue masses in the cervical region.Due to its uncommon presentation in the female repro...BACKGROUND Cervical myeloid sarcoma(MS)is a rare hematological malignancy characterized by the formation of extramedullary soft tissue masses in the cervical region.Due to its uncommon presentation in the female reproductive system,cervical MS poses significant diagnostic and therapeutic challenges.Consequently,there is a pressing need for more research and clinical experience to better understand,diagnose,and manage this condition effectively.CASE SUMMARY This report details four cases,the diagnostic process,treatment strategy,and outcomes,discussing cervical MS as an initial clinical manifestation.The disease exhibits varied clinical presentations,such as irregular vaginal bleeding and pa-lpation of cervical masses.The treatment approaches discussed include neoad-juvant chemotherapy,surgery,and postoperative chemotherapy,though mana-ging the disease remains challenging.The report also features a comprehensive literature review that underscores the importance of immunohistochemistry for accurate diagnosis,identifying key markers,including myeloperoxidase,cluster of differentiation(CD)68,and CD43,stressing the need for further research to improve treatment strategies and prognosis.CONCLUSION Immunohistochemical diagnosis and tailored therapeutic strategies are essential.Further research is crucial in improving outcomes and developing effective treatment protocols.展开更多
Combined hepatocellular-cholangiocarcinoma(cHCC-CCA)is a unique type of liver tumor that contains both hepatocellular carcinoma and cholangiocarcinoma components within a single tumor.The fifth edition of the World He...Combined hepatocellular-cholangiocarcinoma(cHCC-CCA)is a unique type of liver tumor that contains both hepatocellular carcinoma and cholangiocarcinoma components within a single tumor.The fifth edition of the World Health Organization classification provides a definition and diagnostic criteria for cHCC-CCA.However,the heterogeneous histomorphology and presentation resulting from variation of the proportion of each component poses challenges for clinical diagnosis and treatment.A diagnosis of cHCC-CCA may be suggested by the synchronous elevation of serum tumor markers for hepatocellular carcinoma and cholangiocarcinoma,a mixed enhancement pattern on imaging,and a discrepancy between the elevation of tumor marker and the imaging enhancement pattern.Histopathological examination using hematoxylin and eosin staining is considered the gold standard for diagnosing cHCC-CCA,and comprehensive examination of resection or biopsy specimens is crucial for an accurate diagnosis.Currently,there is no standard treatment for cHCC-CCA,and surgery is the mainstay.Anatomic hepatectomy with lymphadenectomy is among the recommended surgical procedures.The role of liver transplantation in the management of cHCC-CCA is still uncertain.Transarterial chemoembolization may be effective for unresectable cHCC-CCA,particularly for hypervascular tumors.However,the available evidence does not support systemic therapy for advanced cHCC-CCA.The prognosis of cHCC-CCA is generally poor,and there is no established staging system.Further research is needed to better understand the histogenesis and clinical management of cHCC-CCA.This review provides an overview of the current literature on cHCC-CCA with a focus on its clinical characteristics,pathological diagnosis,and management.展开更多
Dermatomyositis,an idiopathic inflammatory myopathy,is characterized by distinctive skin manifestations,proximal muscle weakness,and multiple organ involvement and can be accompanied by malignancies.To provide a refer...Dermatomyositis,an idiopathic inflammatory myopathy,is characterized by distinctive skin manifestations,proximal muscle weakness,and multiple organ involvement and can be accompanied by malignancies.To provide a reference for dermatologists and clinicians in other relevant fields of clinical practice,experts from the Dermatology Branch of the China International Exchange and Promotion Association for Medical and Health Care and the National Clinical Research Center for Dermatologic and Immunologic Diseases developed this consensus on the diagnosis and treatment of adult dermatomyositis using Chinese and international literature and expert advice.展开更多
Tick-borne encephalitis is an infection of central nervous system caused by tick-borne encephalitis virus transmitted to humans predominantly by tick bites. During the last few decades the incidence of the disease has...Tick-borne encephalitis is an infection of central nervous system caused by tick-borne encephalitis virus transmitted to humans predominantly by tick bites. During the last few decades the incidence of the disease has been increasing and poses a growing health problem in almost all endemic European and Asian countries. Most cases occur during the highest period of tick activity, in Central Europe mainly from April to November. Tickborne encephalitis is more common in adults than in children. Clinical spectrum of the disease ranges from mild meningitis to severe meningoencephalitis with or without paralysis. Rare clinical manifestations are an abortive form of the disease and a chronic progressive form. A post-encephalitic syndrome, causing long-lasting morbidity that often affects the quality of life develops in up to 50% of patients after acute tick-borne encephalitis. Clinical course and outcome vary by subtype of tick-borne encephalitis virus(the disease caused by the European subtype has milder course and better outcome than the disease caused by Siberian and Far-Easter subtypes), age of patients(increasing age is associated with less favorable outcome), and host genetic factors. Since clinical features and laboratory results of blood and cerebrospinal fluid are nonspecific, the diagnosis must be confirmed by microbiologic findings. The routine laboratory confirmation of the tick-borne encephalitis virus infection is based mainly on the detection of specific Ig M and Ig G antibodies in serum(and cerebrospinal fluid), usually by enzyme-linked immunosorbent assay. There is no specific antiviral treatment for tick-borne encephalitis. Vaccination can effectively prevent the disease and is indicated for persons living in or visiting tick-borne encephalitis endemic areas.展开更多
Non-alcoholic fatty liver disease(NAFLD)is among the most frequent etiologies of cirrhosis worldwide,and it is associated with features of metabolic syndrome;the key factor influencing its prognosis is the progression...Non-alcoholic fatty liver disease(NAFLD)is among the most frequent etiologies of cirrhosis worldwide,and it is associated with features of metabolic syndrome;the key factor influencing its prognosis is the progression of liver fibrosis.This review aimed to propose a practical and stepwise approach to the evaluation and management of liver fibrosis in patients with NAFLD,analyzing the currently available literature.In the assessment of NAFLD patients,it is important to identify clinical,genetic,and environmental determinants of fibrosis development and its progression.To properly detect fibrosis,it is important to take into account the available methods and their supporting scientific evidence to guide the approach and the sequential selection of the best available biochemical scores,followed by a complementary imaging study(transient elastography,magnetic resonance elastography or acoustic radiation force impulse)and finally a liver biopsy,when needed.To help with the selection of the most appropriate method a Fagan′s nomogram analysis is provided in this review,describing the diagnostic yield of each method and their post-test probability of detecting liver fibrosis.Finally,treatment should always include diet and exercise,as well as controlling the components of the metabolic syndrome,+/-vitamin E,considering the presence of sleep apnea,and when available,allocate those patients with advanced fibrosis or high risk of progression into clinical trials.The final end of this approach should be to establish an opportune diagnosis and treatment of liver fibrosis in patients with NAFLD,aiming to decrease/stop its progression and improve their prognosis.展开更多
BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disea...BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high.The overall prognosis is poor and survival rate is low.AIM To investigate the diagnosis,treatment,and prognosis of UESL.METHODS We performed a retrospective,single-center cohort study in Shengjing Hospital of China Medical University,which is a central hospital in northeast China.From 2005 to 2017,we recruited 14 patients with pathologically confirmed UESL.We analyzed the clinical manifestations,laboratory tests,imaging examinations,pathological examinations,therapy,and prognosis of these patients.RESULTS There were nine males and five females aged 2-60 years old included in the study.The major initial symptoms were abdominal pain(71.43%)and fever(57.14%).Preoperative laboratory tests revealed that seven patients had increased leukocyte levels,four showed a decrease in hemoglobin levels,seven patients had increased glutamyl transpeptidase levels,nine had increased lactate dehydrogenase levels,and three showed an increase in carbohydrate antigen 199.There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children(6/6 vs 5/8,P=0.091).The survival rate after complete resection was 6/10,while that after incomplete resection was 0/4(P=0.040),suggesting that complete resection is important to improve survival rate.In total,five out of the eight children achieved survival.During the follow-up,the maximum survival time was shown to be 11 years and minimum survival time was 6 mo.Six adult patients relapsed late after surgery and all of them died.CONCLUSION Preoperative imaging examination for UESL has a high misdiagnosis rate.Multidisciplinary collaboration can improve the diagnostic accuracy of UESL.Complete surgical resection is the first choice for treatment of UESL.展开更多
Malignant lymphoma originates from the lymphohematopoietic system.It can occur in any lymphoid tissue.Malignant lymphoma of the salivary gland is rare,but its incidence has increased in recent years.Its clinical-prese...Malignant lymphoma originates from the lymphohematopoietic system.It can occur in any lymphoid tissue.Malignant lymphoma of the salivary gland is rare,but its incidence has increased in recent years.Its clinical-presentations are nonspecific,and it is often manifested as a painless mass in a salivary gland,which can be accompanied by multiple swollen cervical lymph nodes.Confirmation of the diagnosis before an invasive procedure is difficult.Clinically,malignant lymphoma of the salivary gland tends to be misdiagnosed,leading to an inappropriate treatment plan and the ultimate delay in the optimal treatment of the disease.This article reviews the pathogenesis,clinical features,imaging findings,diagnosis,treatment and prognosis of malignant lymphoma of the salivary gland.展开更多
Background:Wernicke encephalopathy(WE)is an acute neurological disease resulting from vitamin B1 deficiency,and there are only very few case reports of WE after liver transplantation.The present study aimed to investi...Background:Wernicke encephalopathy(WE)is an acute neurological disease resulting from vitamin B1 deficiency,and there are only very few case reports of WE after liver transplantation.The present study aimed to investigate the clinical characteristics,etiology,magnetic resonance imaging(MRI)features,treatment and prognosis of patients with WE after liver transplantation.Methods:Twenty-three patients with WE after liver transplantation from the First Affiliated Hospital,Zhejiang University School of Medicine and Jiangxi Provincial People’s Hospital between January 2011 and December 2021 were retrospectively analyzed.Results:Among the 23 patients diagnosed with WE after liver transplantation,6(26%)had a classic triad of impaired consciousness,oculomotor palsy and ataxia,and 17(74%)had two features.The misdiagno-sis rate was 65%.After treatment with high-dose vitamin B1,19(83%)patients showed improvement,whereas 4(17%)showed no improvement,including 3 with residual short-term memory impairments and 1 with residual spatial and temporal disorientation and ataxia.Conclusions:The misdiagnosis rate is high in the early stage of WE,and the prognosis is closely asso-ciated with whether WE is diagnosed early and treated timely.High-dose glucose or glucocorticoids can trigger WE and cannot be administered before vitamin B1 treatment.Vitamin B1 is suggested to be used as a prophylactic treatment for patients with WE after liver transplantation.展开更多
In clinical practice,pancreatic neuroendocrine neoplasms(pNENs)with a diameter smaller than 2 cm are commonly referred to as small pNENs.Due to their generally favorable biological characteristics,the diagnosis and tr...In clinical practice,pancreatic neuroendocrine neoplasms(pNENs)with a diameter smaller than 2 cm are commonly referred to as small pNENs.Due to their generally favorable biological characteristics,the diagnosis and treatment of small pNENs differ from other pNENs and are somewhat controversial.In response to this,the Chinese Pancreatic Surgery Association,Chinese Society of Surgery,Chinese Medical Association have developed a consensus on the diagnosis and treatment of small pNENs,which is based on evidence-based medicine and expert opinions.This consensus covers various topics,including concepts,disease assessment,treatment selection,follow-up,and other relevant aspects.展开更多
Bland-White-Garland syndrome or ALCAPA is an abnormality of birth of the left coronary artery from the pulmonary artery. It is a diagnostic and therapeutic emergency because it is a curable cause of hypokinetic dilate...Bland-White-Garland syndrome or ALCAPA is an abnormality of birth of the left coronary artery from the pulmonary artery. It is a diagnostic and therapeutic emergency because it is a curable cause of hypokinetic dilated heart disease in infants. We report through this clinical case, the fourth case of infantile ALCAPA diagnosed in Senegal in a 7-month-old infant. The symptomatology began around the age of 2 months, with a grumpy state associated with more marked crying and moaning during feedings and bowel movements. The mother reported hospitalization for a severe lung infection when she was 6 months old. The examination noted an infant in poor general condition, retarded growth and weight, and a 3/6th holosystolic murmur at the apex. Troponinemia was positive at 43.90 ng/L. The electrocardiogram showed Q waves on the lower side, a sub endocardial lesion on the upper side and a ST segment elevation in aVR. Doppler echocardiography showed dilated cardiomyopathy with a mean alteration of systolic function of the left ventricle at 37%, a mean mitral insufficiency and a strong suspicion of a birth anomaly of the left coronary artery. The CT scan confirmed the diagnosis of ALCAPA. Surgicalreimplantation of the left coronary artery at the aortic level was performed at 10 months of life with a favourable outcome at D50 postoperative.展开更多
文摘Achalasia cardia,type of esophageal dynamic disorder,is a relatively rare primary motor esophageal disease characterized by the functional loss of plexus ganglion cells in the distal esophagus and lower esophageal sphincter.Loss of function of the distal and lower esophageal sphincter ganglion cells is the main cause of achalasia cardia,and is more likely to occur in the elderly.Histological changes in the esophageal mucosa are considered pathogenic;however,studies have found that inflammation and genetic changes at the molecular level may also cause achalasia cardia,resulting in dysphagia,reflux,aspiration,retrosternal pain,and weight loss.Currently,the treatment options for achalasia focus on reducing the resting pressure of the lower esophageal sphincter,helping to empty the esophagus and relieve symptoms.Treatment measures include botulinum toxin injection,inflatable dilation,stent insertion,and surgical myotomy(open or laparoscopic).Surgical procedures are often subject to controversy owing to concerns about safety and effectiveness,particularly in older patients.Herein,we review clinical epidemiological and experimental data to determine the prevalence,pathogenesis,clinical presentation,diagnostic criteria,and treatment options for achalasia to support its clinical management.
文摘Solitary rectal ulcer syndrome (SRUS) is an uncommon benign disease, characterized by a combination of symptoms, clinical findings and histological abnormalities. Ulcers are only found in 40% of the patients; 20% of the patients have a solitary ulcer, and the rest of the lesions vary in shape and size, from hyperemic mucosa to broad-based polypoid. Men and women are affected equally, with a small predominance in women. SRUS has also been described in children and in the geriatric population. Clinical features include rectal bleeding, copious mucus discharge, prolonged excessive straining, perineal and abdominal pain, feeling of incomplete defecation, constipation, and rarely, rectal prolapse. This disease has well-described histopathological features such as obliteration of the lamina propria by fibrosis and smooth muscle fibers extending from a thickened muscularis mucosa to the lumen. Diffuse collage deposition in the lamina propria and abnormal smooth muscle fiber extensions are sensitive markers for differ-entiating SRUS from other conditions. However, the etiology remains obscure, and the condition is frequently associated with pelvic floor disorders. SRUS is difficult to treat, and various treatment strategies have been advocated, ranging from conservative management to a variety of surgical procedures. The aim of the present review is to summarize the clinical features, pathophysiology, diagnostic methods and treatment strategies associated with SRUS. (C) 2014 Baishideng Publishing Group Co., Limited. All rights reserved.
文摘BACKGROUND Hepatic epithelioid hemangioendothelioma(HEHE)is a rare hepatic vascular tumor with unpredictable malignant potential.The etiology,characteristics,diagnosis,treatment,and prognosis of HEHE are not well-understood,and largescale retrospective studies are required to understand better this disease.AIM To determine the characteristics of HEHE and identify its optimal treatments and prognostic factors.METHODS The clinical data of two patients diagnosed with HEHE at the Fourth Hospital of Hebei Medical University and 258 previously reported cases retrieved from the China National Knowledge Infrastructure and PubMed databases between 1996 and 2021 were combined and summarized.All cases were pathologically identified as HEHE.Information such as clinical features,laboratory examination findings,imaging findings,pathological characteristics,treatment,and survival periods was reviewed.Kaplan-Meir curves were used for survival analysis.Prognostic factors were identified by Cox regression analysis.RESULTS HEHE primarily affected middle-aged women.The typical manifestations included epigastric pain,hepatosplenomegaly,inappetence,distension,weight loss,and fatigue.Tumor markers were expressed normally.The incidence of extrahepatic metastasis was 34.5% at the time of diagnosis.The most common sites of extrahepatic involvement were the lungs(22.3%),lymph nodes(5.6%),peritoneum(3.6%),bones(6.6%),and spleen(5.1%).Furthermore,“capsular retraction”,“target sign”,and“lollipop sign”were the characteristic features of HEHE on imaging.The immunohistochemical profile for HEHE(expression of vascular markers,such as factor VIII-related antigen,CD31,and CD34;expression levels of D2-40)can facilitate and ensure an accurate diagnosis.The management options for patients with HEHE include liver resection(29.7%),liver transplantation(16.1%),palliative treatments(12.7%),transhepatic arterial chemotherapy and embolization(TACE,10.2%),chemotherapy(11.0%),antiangiogenic therapy(15.3%),and other treatments(5.1%);the mean survival time was 158.6,147.3,4.2,90.8,71.4,83.1,and 55.0 mo,respectively.The survival time of patients who underwent surgical treatment was longer than that of patients who did not.TACE and antiangiogenic therapy tended to prolong survival compared with other nonsurgical treatments.The 1-,5-,and 10-year survival rates were 82%,71%,and 64%,respectively.Multivariate analysis showed that liver function(P=0.045),intrahepatic metastasis(P=0.029),and treatment(P=0.045)were independent prognostic factors.The presence of extrahepatic metastases was not an independent risk factor for poor prognosis(P=0.558).CONCLUSION The clinical course of HEHE is rare and variable,and patients with intrahepatic metastases and liver dysfunction may have a poorer prognosis than those without.Surgical intervention,whether liver resection or transplantation,might be warranted regardless of extrahepatic metastasis.For patients without the option for surgery,clinicians should consider the use of TACE with antiangiogenic drugs in the treatment of HEHE.
文摘Objective: To explore senile brucellosis spondylitis clinical features and diagnostic criteria, in order to improve the diagnosis rate and evaluate the clinical effects of treatment strategies. Methods: From January 2002 to August 2015, 62 patients with Brucella-associated spondylitis were treated with comprehensive diagnosis based on epidemiological history, clinical manifestations, imaging findings, laboratory tests, and local biopsy. The positive rate of red plate agglutination test (RBPT) was 45.1%, the positive rate of serum tube agglutination test (SAT) was 51.6%, and the positive rate of brucellosis anti-human immunoglobulin test (Coomb’s) was 100%. All patients underwent X-ray, CT and MRI examinations. The positive rate of blood culture in pathogenic examination was 16.1%, and the positive rate of bone marrow culture was 38.5%. The positive rate of inflammatory granuloma or abscess in the lesion sampling culture was 73.6%. All patients underwent standardized drug therapy and hyperbaric oxygen adjuvant therapy. Among them, 38 patients with neurological impairment were treated with one-stage debridement and posterior pedicle screw fixation on the basis of drug therapy. The paraspinal abscess and the diseased in-tervertebral space tissue were taken for pathological examination during the operation. One week, two weeks, one month, three months, six months and 12 months after treatment, the patients were evaluated and followed up at the monitoring sites. The evaluation indicators were pain scores, activity of daily living (ADL), imaging findings, and laboratory tests. The SPSS15.0 statistical package was used for analysis. Results: All patients were confirmed by the above-mentioned comprehensive examination after admission. 24 patients (38.71%) were treated with standard drug therapy (group A), no adverse drug reactions and abnormal liver and kidney function;the remaining 38 patients (61.29%) were combined with varying degrees of neurological impairment, and surgical treatment was performed after 2 to 4 weeks of drug therapy without improvement (group B). The pain scores showed that there was a statistically significant difference between the two groups at the same time point (P Conclusion: The senile Brucellosis spondylitis has a characteristic performance. The de-velopment of diagnostic criteria can help to improve the diagnostic rate. Standardized drug treatment has a good cure rate. The implementation of surgery has obvious advantages whether it is to relieve pain, stabilize the spine, restore nerve function, or recover early.
文摘Diabetic foot(DF)is one of the most common complications of diabetes and is associated with high morbidity,disability,lethality and low cure-rate.The clinical diagnosis and treatment of DF need to be standardized.The Chinese Diabetic Foot Cell and Interventional Therapy Technology Alliance has released six editions of guidelines and standards for clinical diagnosis and interventional treatment of DF,which filled the gap in the domestic DF treatment standard and played an important role in improving the level of diagnosis and treatment in China.In line with the latest developments in diagnosis and treatment,the Alliance,along with other 89 institutions,developed and issued the new edition based on the sixth edition to help standardize the clinical diagnosis and treatment of DF in China.
文摘BACKGROUND Since December 2019,many cases of pneumonia caused by novel coronavirus have been discovered in Wuhan,China,and such cases have spread nationwide quickly.At present,coronavirus disease 2019(COVID-19)is a worldwide pandemic.What are the clinical features of this disease?What is the clinical diagnosis and how should such patients be treated?As a clinician,mastery of the clinical characteristics,basic diagnosis,and treatment methods of COVID-19 are required to provide help to patients.CASE SUMMARY A 42-year-old male patient with a cough lasting 6 d without obvious cause,as well as fever and fatigue for 1 d,was admitted to Hankou Hospital on January 22,2020 and transferred to Huoshenshan Hospital on February 4.The main clinical symptoms were dry cough,fatigue,and fever.He was diagnosed with COVID-19.From the 4th d of admission,the patient’s condition gradually worsened,with increased respiratory rate and body temperature.Peripheral blood lymphocytes decreased progressively.On the 8th d of admission,the patient’s highest temperature was 40.7°C,and oxygen saturation was 83%despite high-flow oxygen inhalation.Chest computed tomography results showed that the virus progressed rapidly.The number of lesions significantly increased with expanded scope and increased density.The distribution of lesions advanced from peripheral to central.In addition to nasal catheter oxygen inhalation and symptomatic support,antiviral drugs were used throughout the treatment.On January 22,oseltamivir phosphate capsules were given orally(75 mg,twice daily)for 6 d.On January 24,three tablets of lopinavir and ritonavir were added orally(twice daily).After 6 d,this was changed to 0.2 g(two tablets)arbidol,taken orally(three times daily)for 5 d.During the severe stage,methylprednisolone was given(40 mg)once every 12 h,immunoglobulin(20 g)was administered by intravenous drip infusion once daily,and thymosin(1.6 mg)was injected subcutaneously once daily combined with immunotherapy.On February 2,symptoms decreased,various indicators improved,and pulmonary inflammation was obviously reduced.Throat swabs on February 4 and 9 were negative for novel coronavirus nucleic acid.After 19 d in the hospital,the patient was successfully treated and discharged.CONCLUSION COVID-19 in young adults can be successfully treated with active treatment.We report a typical case of COVID-19,analyze its clinical characteristics,summarize its clinical diagnosis and treatment experience,and provide a reference for clinical colleagues.
文摘Objective:To investigate the relationship between clinical manifestations,blood testing,chest CT,treatment and fever of 457mild cases of COVID-19.Methods:A tatal of 457cases of mild COVID-19 diagnosed in Wuhan Jianghan module Hospital from February 6,2020 to February 26,2020 were gathered,and the cases with/without fever were retrospectively analyzed in epidemiology,clinical manifestation,blood routing,hypersensitive C-reactive protein(hs-CRP),pathogens serological testing,chest CT and drugs treatment.Results:For 312 fever cases,the average age was(47.6±10.9)years old,male was 128 cases(41%),the mean peak fever was 38(36.8,38.6)℃,fever lasting was 2(0,3)days,131cases(28.7%)had Huanan seafood market exposure history,90 cases(28.8%)had closely contact with COVID-19 patients,20cases(6.4%)had hypertension history,10 cases(3.2%)had diabetes,5cases(1.6%)had coronary heart disease.Main symptoms of fever group including dry cough of 260cases(83.3%),of 104cases(33.3%),blood sputum of 9cases(2.9%),chest tightness 20cases(19.6%),shortness of breath of 87cases(27.9%),weak of 105cases(33.7%),diarrhea of 43cases(13.8%),sleep disorders of 61cases(19.6%),among fever cases with sputum and shortness of breath more rather without fever,and two groups were statistically significantly difference(P<0.05).In fever group,WBC(109/L)[4.34(3.69,5.26)than 5.11(4.22,6.22),P<0.001],NEUT(109/L)[2.67(2.1,3.49)than 2.88(2.37,3.9),P<0.05],LYM(109/L)[0.9(0.8,1.1)than 1.36(1.03,1.85),P<0.001),PLT(109/L)/(106,188)134-189(132,224),P<0.001)were lower than normal group,while WBC<4.0×109/L[108(34.6%)than 24(16.6%),P<0.001)],LYM<1.0×109/L[189(60.6%)than 31(21.4%),P<0.001)],PLT<100×109/L[45(14.4%)than in 2(1.4%),P<0.001)and hs-CRP(mg/L)[14(6.83,32)than 3.4(0.96,10.75),P<0.001)were higher than normal group,and pathogens serological examination of 36cases shown influenzaⅠ/Ⅱvirus IgM antibody positive in 3cases(8.3%),respiratory syncytial virus IgM antibody positive in 2 cases(5.6%),mycoplasma pneumoniae IgM antibody positive(11.1%),4 cases of adenovirus IgM antibody positive(11.1%),but 4 pathogens of fever group and normal group were no statistical difference(P>0.05).According to early onset of chest CT examination,there were no change of 11cases(2.4%),unilateral lung lesions of 93cases(20.4%),bilateral lung lesions of 353 cases(77.2%),ground glass shadow of 228 cases(73.1%),high density shadow patch of 65 cases(20.8%),consolidation shadow of 6 cases(1.9%),while fever group compared with normal group,there were no statistically significant difference(P>0.05).Logistic regression analysis showed that LYM(OR=0.24,95%CI=0.139~0.415,P<0.001),LYM<1.0 x 109/L(OR=5.12,95%CI=3.07~8.65,P<0.001),PLT<100×109/L(OR=6.1,95%CI=1.36~27.33,P<0.05),and the hs-CRP(OR=1.03,95%CI=1.02~1.05,P<0.001)were fever independent impact factors in mild COVID-19.Aiming at COVID-19 treatment,Chinese medicine usage had 416cases(91%),antiviral drugs had 328cases(71.8%),and antibiotics had 172cases(37.6%),furthermore,fever group using antiviral and antibiotic drugs was higher than normal group(P<0.05),and compared to low fever group,high fever group used more antibiotic drugs(P<0.05).Conclusions:Mild COVID-19 patients with early onset had epidemiological characteristics,and fever,dry cough,expectoration and shortness of breath were main symptoms,and chest CT often involved bilateral lung lesions,ground glass shadow and patch density shadow.The fever patients in WBC,LYM,PLT,hs-CRP index changed significantly and could affect on anti-infective therapy selection.
文摘BACKGROUND Gallbladder neuroendocrine carcinoma(GB-NEC)has a low incidence rate;therefore,its clinical characteristics,diagnosis,treatment and prognosis are not well explored.AIM To review recent research and analyze corresponding data in the Surveillance Epidemiology and End Results(SEER)database.METHODS Data of GB-NEC(n=287)and gallbladder adenocarcinoma(GB-ADC)(n=19484)patients from 1975 to 2016 were extracted from the SEER database.Survival analysis was performed using Kaplan–Meier and Cox proportional hazards regression.P<0.05 was considered statistically significant.We also reviewed 108 studies retrieved from PubMed and Reference Citation Analysis(https://www.referencecitationanalysis.com/).The keywords used for the search were:"(Carcinoma,Neuroendocrine)AND(Gallbladder Neoplasms)".RESULTS The GB-NEC incidence rate was 1.6%(of all gallbladder carcinomas),male to female ratio was 1:2 and the median survival time was 7 mo.The 1-,2-,3-and 5-year overall survival(OS)was 36.6%,17.8%,13.2%and 7.3%respectively.Serum chromogranin A levels may be a specific tumor marker for the diagnosis of GBNEC.Elevated carcinoembryonic antigen,carbohydrate antigen(CA)-19-9 and CA-125 levels were associated with poor prognosis.Age[hazard ratio(HR)=1.027,95%confidence interval(CI):1.006–1.047,P=0.01]and liver metastasis(HR=3.055,95%CI:1.839–5.075,P<0.001)are independent prognostic risk factors for OS.Patients with advanced GB-NEC treated with surgical resection combined with radiotherapy and/or chemotherapy may have a better prognosis than those treated with surgical resection alone.There was no significant difference in OS between GB-NEC and GB-ADC.CONCLUSION The clinical manifestations and prognosis of GB-NEC are similar to GB-ADC,but the treatment is completely different.Early diagnosis and treatment are the top priorities.
文摘BACKGROUND Cervical myeloid sarcoma(MS)is a rare hematological malignancy characterized by the formation of extramedullary soft tissue masses in the cervical region.Due to its uncommon presentation in the female reproductive system,cervical MS poses significant diagnostic and therapeutic challenges.Consequently,there is a pressing need for more research and clinical experience to better understand,diagnose,and manage this condition effectively.CASE SUMMARY This report details four cases,the diagnostic process,treatment strategy,and outcomes,discussing cervical MS as an initial clinical manifestation.The disease exhibits varied clinical presentations,such as irregular vaginal bleeding and pa-lpation of cervical masses.The treatment approaches discussed include neoad-juvant chemotherapy,surgery,and postoperative chemotherapy,though mana-ging the disease remains challenging.The report also features a comprehensive literature review that underscores the importance of immunohistochemistry for accurate diagnosis,identifying key markers,including myeloperoxidase,cluster of differentiation(CD)68,and CD43,stressing the need for further research to improve treatment strategies and prognosis.CONCLUSION Immunohistochemical diagnosis and tailored therapeutic strategies are essential.Further research is crucial in improving outcomes and developing effective treatment protocols.
文摘Combined hepatocellular-cholangiocarcinoma(cHCC-CCA)is a unique type of liver tumor that contains both hepatocellular carcinoma and cholangiocarcinoma components within a single tumor.The fifth edition of the World Health Organization classification provides a definition and diagnostic criteria for cHCC-CCA.However,the heterogeneous histomorphology and presentation resulting from variation of the proportion of each component poses challenges for clinical diagnosis and treatment.A diagnosis of cHCC-CCA may be suggested by the synchronous elevation of serum tumor markers for hepatocellular carcinoma and cholangiocarcinoma,a mixed enhancement pattern on imaging,and a discrepancy between the elevation of tumor marker and the imaging enhancement pattern.Histopathological examination using hematoxylin and eosin staining is considered the gold standard for diagnosing cHCC-CCA,and comprehensive examination of resection or biopsy specimens is crucial for an accurate diagnosis.Currently,there is no standard treatment for cHCC-CCA,and surgery is the mainstay.Anatomic hepatectomy with lymphadenectomy is among the recommended surgical procedures.The role of liver transplantation in the management of cHCC-CCA is still uncertain.Transarterial chemoembolization may be effective for unresectable cHCC-CCA,particularly for hypervascular tumors.However,the available evidence does not support systemic therapy for advanced cHCC-CCA.The prognosis of cHCC-CCA is generally poor,and there is no established staging system.Further research is needed to better understand the histogenesis and clinical management of cHCC-CCA.This review provides an overview of the current literature on cHCC-CCA with a focus on its clinical characteristics,pathological diagnosis,and management.
基金supported by National High-level Hospital Clinical Research Funding(2022-PUMCH-B-092).
文摘Dermatomyositis,an idiopathic inflammatory myopathy,is characterized by distinctive skin manifestations,proximal muscle weakness,and multiple organ involvement and can be accompanied by malignancies.To provide a reference for dermatologists and clinicians in other relevant fields of clinical practice,experts from the Dermatology Branch of the China International Exchange and Promotion Association for Medical and Health Care and the National Clinical Research Center for Dermatologic and Immunologic Diseases developed this consensus on the diagnosis and treatment of adult dermatomyositis using Chinese and international literature and expert advice.
文摘Tick-borne encephalitis is an infection of central nervous system caused by tick-borne encephalitis virus transmitted to humans predominantly by tick bites. During the last few decades the incidence of the disease has been increasing and poses a growing health problem in almost all endemic European and Asian countries. Most cases occur during the highest period of tick activity, in Central Europe mainly from April to November. Tickborne encephalitis is more common in adults than in children. Clinical spectrum of the disease ranges from mild meningitis to severe meningoencephalitis with or without paralysis. Rare clinical manifestations are an abortive form of the disease and a chronic progressive form. A post-encephalitic syndrome, causing long-lasting morbidity that often affects the quality of life develops in up to 50% of patients after acute tick-borne encephalitis. Clinical course and outcome vary by subtype of tick-borne encephalitis virus(the disease caused by the European subtype has milder course and better outcome than the disease caused by Siberian and Far-Easter subtypes), age of patients(increasing age is associated with less favorable outcome), and host genetic factors. Since clinical features and laboratory results of blood and cerebrospinal fluid are nonspecific, the diagnosis must be confirmed by microbiologic findings. The routine laboratory confirmation of the tick-borne encephalitis virus infection is based mainly on the detection of specific Ig M and Ig G antibodies in serum(and cerebrospinal fluid), usually by enzyme-linked immunosorbent assay. There is no specific antiviral treatment for tick-borne encephalitis. Vaccination can effectively prevent the disease and is indicated for persons living in or visiting tick-borne encephalitis endemic areas.
文摘Non-alcoholic fatty liver disease(NAFLD)is among the most frequent etiologies of cirrhosis worldwide,and it is associated with features of metabolic syndrome;the key factor influencing its prognosis is the progression of liver fibrosis.This review aimed to propose a practical and stepwise approach to the evaluation and management of liver fibrosis in patients with NAFLD,analyzing the currently available literature.In the assessment of NAFLD patients,it is important to identify clinical,genetic,and environmental determinants of fibrosis development and its progression.To properly detect fibrosis,it is important to take into account the available methods and their supporting scientific evidence to guide the approach and the sequential selection of the best available biochemical scores,followed by a complementary imaging study(transient elastography,magnetic resonance elastography or acoustic radiation force impulse)and finally a liver biopsy,when needed.To help with the selection of the most appropriate method a Fagan′s nomogram analysis is provided in this review,describing the diagnostic yield of each method and their post-test probability of detecting liver fibrosis.Finally,treatment should always include diet and exercise,as well as controlling the components of the metabolic syndrome,+/-vitamin E,considering the presence of sleep apnea,and when available,allocate those patients with advanced fibrosis or high risk of progression into clinical trials.The final end of this approach should be to establish an opportune diagnosis and treatment of liver fibrosis in patients with NAFLD,aiming to decrease/stop its progression and improve their prognosis.
基金The National Science and Technology Major Project,No.2017ZX10201201 and No.2017ZX10202202Liaoning Provincial Natural Science Foundation,No.20180550096。
文摘BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high.The overall prognosis is poor and survival rate is low.AIM To investigate the diagnosis,treatment,and prognosis of UESL.METHODS We performed a retrospective,single-center cohort study in Shengjing Hospital of China Medical University,which is a central hospital in northeast China.From 2005 to 2017,we recruited 14 patients with pathologically confirmed UESL.We analyzed the clinical manifestations,laboratory tests,imaging examinations,pathological examinations,therapy,and prognosis of these patients.RESULTS There were nine males and five females aged 2-60 years old included in the study.The major initial symptoms were abdominal pain(71.43%)and fever(57.14%).Preoperative laboratory tests revealed that seven patients had increased leukocyte levels,four showed a decrease in hemoglobin levels,seven patients had increased glutamyl transpeptidase levels,nine had increased lactate dehydrogenase levels,and three showed an increase in carbohydrate antigen 199.There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children(6/6 vs 5/8,P=0.091).The survival rate after complete resection was 6/10,while that after incomplete resection was 0/4(P=0.040),suggesting that complete resection is important to improve survival rate.In total,five out of the eight children achieved survival.During the follow-up,the maximum survival time was shown to be 11 years and minimum survival time was 6 mo.Six adult patients relapsed late after surgery and all of them died.CONCLUSION Preoperative imaging examination for UESL has a high misdiagnosis rate.Multidisciplinary collaboration can improve the diagnostic accuracy of UESL.Complete surgical resection is the first choice for treatment of UESL.
基金Supported by the 345 Talent Project of Shengjing Hospitalthe Natural Science Foundation of Liaoning Province,No.20170541042.
文摘Malignant lymphoma originates from the lymphohematopoietic system.It can occur in any lymphoid tissue.Malignant lymphoma of the salivary gland is rare,but its incidence has increased in recent years.Its clinical-presentations are nonspecific,and it is often manifested as a painless mass in a salivary gland,which can be accompanied by multiple swollen cervical lymph nodes.Confirmation of the diagnosis before an invasive procedure is difficult.Clinically,malignant lymphoma of the salivary gland tends to be misdiagnosed,leading to an inappropriate treatment plan and the ultimate delay in the optimal treatment of the disease.This article reviews the pathogenesis,clinical features,imaging findings,diagnosis,treatment and prognosis of malignant lymphoma of the salivary gland.
基金approved by Jiangxi Provincial People’s Hospital and First Affiliated Hospital,Zhejiang University School of Medicine(2022068 and 2022370).Written informed consent was obtained from all participants.
文摘Background:Wernicke encephalopathy(WE)is an acute neurological disease resulting from vitamin B1 deficiency,and there are only very few case reports of WE after liver transplantation.The present study aimed to investigate the clinical characteristics,etiology,magnetic resonance imaging(MRI)features,treatment and prognosis of patients with WE after liver transplantation.Methods:Twenty-three patients with WE after liver transplantation from the First Affiliated Hospital,Zhejiang University School of Medicine and Jiangxi Provincial People’s Hospital between January 2011 and December 2021 were retrospectively analyzed.Results:Among the 23 patients diagnosed with WE after liver transplantation,6(26%)had a classic triad of impaired consciousness,oculomotor palsy and ataxia,and 17(74%)had two features.The misdiagno-sis rate was 65%.After treatment with high-dose vitamin B1,19(83%)patients showed improvement,whereas 4(17%)showed no improvement,including 3 with residual short-term memory impairments and 1 with residual spatial and temporal disorientation and ataxia.Conclusions:The misdiagnosis rate is high in the early stage of WE,and the prognosis is closely asso-ciated with whether WE is diagnosed early and treated timely.High-dose glucose or glucocorticoids can trigger WE and cannot be administered before vitamin B1 treatment.Vitamin B1 is suggested to be used as a prophylactic treatment for patients with WE after liver transplantation.
基金supported by the Chinese Academy of Medical Sciences(CAMS)Innovation Fund for Medical Sciences(CIFMS)2021-I2M-1-002.
文摘In clinical practice,pancreatic neuroendocrine neoplasms(pNENs)with a diameter smaller than 2 cm are commonly referred to as small pNENs.Due to their generally favorable biological characteristics,the diagnosis and treatment of small pNENs differ from other pNENs and are somewhat controversial.In response to this,the Chinese Pancreatic Surgery Association,Chinese Society of Surgery,Chinese Medical Association have developed a consensus on the diagnosis and treatment of small pNENs,which is based on evidence-based medicine and expert opinions.This consensus covers various topics,including concepts,disease assessment,treatment selection,follow-up,and other relevant aspects.
文摘Bland-White-Garland syndrome or ALCAPA is an abnormality of birth of the left coronary artery from the pulmonary artery. It is a diagnostic and therapeutic emergency because it is a curable cause of hypokinetic dilated heart disease in infants. We report through this clinical case, the fourth case of infantile ALCAPA diagnosed in Senegal in a 7-month-old infant. The symptomatology began around the age of 2 months, with a grumpy state associated with more marked crying and moaning during feedings and bowel movements. The mother reported hospitalization for a severe lung infection when she was 6 months old. The examination noted an infant in poor general condition, retarded growth and weight, and a 3/6th holosystolic murmur at the apex. Troponinemia was positive at 43.90 ng/L. The electrocardiogram showed Q waves on the lower side, a sub endocardial lesion on the upper side and a ST segment elevation in aVR. Doppler echocardiography showed dilated cardiomyopathy with a mean alteration of systolic function of the left ventricle at 37%, a mean mitral insufficiency and a strong suspicion of a birth anomaly of the left coronary artery. The CT scan confirmed the diagnosis of ALCAPA. Surgicalreimplantation of the left coronary artery at the aortic level was performed at 10 months of life with a favourable outcome at D50 postoperative.