BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis an...BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis and prognosis.METHODS We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic criteria.RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea.The common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and ileum.Villous blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal biopsies.Moreover,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies.All patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)days.Immunosuppressants were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s lymphoma.The cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,respectively.CONCLUSION Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult AIE.The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications,which highlights the need for early diagnosis and novel medications.展开更多
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
·AIM:To report a case which keratitis is the first clinical manifestation of COVID-19 that occurred 3 d earlier than the common COVID-19 symptoms.·METHODS:Regular slit lamp examination,corneal scraping test,...·AIM:To report a case which keratitis is the first clinical manifestation of COVID-19 that occurred 3 d earlier than the common COVID-19 symptoms.·METHODS:Regular slit lamp examination,corneal scraping test,and chest computed tomography(CT)were performed for patients with COVID-19 infection.The ophthalmologic treatment included ganciclovir eye drop(50 mglmL,6 times/d).The treatment for diarrhea included Guifu Lizhong pills(TID).The antiviral therapy consisted of oseltamivir(75 mg capsule Q12 H);therapy preventing bacterial infection consisted of azithromycin(250 mg tablet QD)and moxifloxacin(0.4 g tablet Q12 H);and therapy for cough relief and fever prevention consisted of Chinese herbal decoction.·RESULTS:A 35-year-old male suddenly suffered pain,photophobia,and tears in his right eye for one day without systemic COVID-19 symptoms.Patient was diagnosed with keratitis,which was seemingly different from common keratitis.Ganciclovir eye drop was initiated.The corneal scraping test for COVID-19 was positive.The chest CT images were abnormal confirming the diagnosis of COVID-19 infection.The antiviral and antibacterial therapies were initiated.Chinese herbal therapy was used for cough relief and fever prevention.After roughly two weeks,patient recovered from COVID-19.·CONCLUSION:A new type of keratitis,atypical keratitis,is a clinical manifestation of COVID-19,and this clinical manifestation could appear 3 d earlier than fever and cough.The earlier a COVID-19 clinical manifestation is identified,the earlier can a patient be directed to stay at home,and significantly fewer people would be infected.展开更多
BACKGROUND: Recent advances in molecular and genomic technologies and pancreatic imaging techniques provided some insights into genetic, environmental, immunologic, and pathobiological factors for chronic pancreatitis...BACKGROUND: Recent advances in molecular and genomic technologies and pancreatic imaging techniques provided some insights into genetic, environmental, immunologic, and pathobiological factors for chronic pancreatitis (CP). This study was undertaken to investigate the clinical manifestations of patients with chronic pancreatitis at our hospital. METHODS: The data of the patients with CP who had been treated at our hospital between 1997 and 2004 were analyzed. RESULTS: The major symptoms of the patients with CP were abdominal pain, dyspepsia, loss of weight, diabetes mellitus, pancreatic pseudocyst, steatorrhea, and calcification. Biliary diseases were found to be the first cause of CP in this study; but alcohol abuse was the major cause of CP in men and biliary diseases were the first etiological factors for CP in women. The etiological difference of constituent ratio between men and women was related to alcohol comsumption (P<0.01). CONCLUSIONS: During the past 8 years, biliary diseases have been the major etiological factors for CP, but their constituent ratio is decreasing, and the constituent ratio of alcohol abuse is increasing gradually. Alcohol tends to replace biliary diseases as the primary etiological factor for CP.展开更多
BACKGROUND: Accurate diagnosis of infected aortic and iliac aneurysms is often delayed, hampering timely treatment and potentially resulting in a fatal consequence. The aim of this study was to discover useful clinica...BACKGROUND: Accurate diagnosis of infected aortic and iliac aneurysms is often delayed, hampering timely treatment and potentially resulting in a fatal consequence. The aim of this study was to discover useful clinical features that can help physicians to identify these patients.METHODS: We reviewed the discharge notes from two hospitals and identifi ed all patients who had a diagnosis of infected aneurysms of the thoracoabdominal aorta and iliac arteries between July 2009 and December 2013. Eighteen patients, aged from 41 to 93, were reviewed. Only 6 patients were diagnosed accurately in their fi rst visit to our ED.RESULTS: Most patients had at least one underlying illness, and it took 1 to 30(9.9±6.5) days for physicians to diagnose their infected aneurysm. Localized pain and fever were the two most commonly presented symptoms. The majority(92%) of isolated microorganisms were gram-negative bacilli, including Salmonella spp, Klebsiella pneumoniae, and Escherichia coli. Two of the 3 patients who underwent non-operative therapy died, and all of the patients who underwent a combination of medical and operative therapies survived.CONCLUSION: We suggest that physicians liberally use computed tomography scans on patients with unknown causes of pain and inflammatory processes. A combination of surgical and medical treatments is indicated for all patients with infected aortic and iliac aneurysms.展开更多
BACKGROUND： Myasthenia gravis （MG） is an autoimmune disease which mainly affects neuromuscular junctions. The ages, modified Osserman classification and clinical manifestation and humoral immunol function of MG wit...BACKGROUND： Myasthenia gravis （MG） is an autoimmune disease which mainly affects neuromuscular junctions. The ages, modified Osserman classification and clinical manifestation and humoral immunol function of MG with and without thymic abnormality are different. OBJECTIVE： To explore the clinical manifestation and humoral immuno-function of MG with abnormal and normal thymus gland. DESIGN ： Contrast observation SETTTNG ： Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University PARTZCZPANTS ： A total of 49 inpatients with MG were selected from the Third Affiliated Hospital of Sun Yat-sen University from March 2000 to August 2005. All the patients had typical clinical manifestation of MG and positive neostigmine test. All the patients knew and agreed the laboratory examinations. There were 22 males and 27 females of 2-69 years old. Chest MRI or CT scan were performed to reveal thymus gland abnormality. According to whether there was tumor in superior mediastinum, all patients were divided into 2 groups, abnormal and normal groups. Normal thymus gland group （n=30） contained 16 males and 14 females of 6-43 years old. Abnormal thymus gland group （n=19） contained 6 male and 13 female of 2-69 years old, METHODS： ① All patients were questioned about initial symptoms. Meanwhile, main clinical manifestations were recorded at hospital admission. ② 7180A automatic biochemical analyzer and automatic microplate reader were used in detecting seroimmunity index. The levels of C3, C4, IgG, IgA, IgM and CH50 in blood serum were analyzed by nephelometry. ③ Clinical classification is based on modified Osserman classification. The patients with MG were divided into six types： I （Ocular myasthenia）, Ⅱ a （Mild generalized myasthenia）, Ⅱb （Moderately severe generalized myasthenia）, Ⅲ （Acute fulminating myasthenia）, Ⅳ（Late se- vere myasthenia）. MAZN OUTCOME MEASURES： ① Differences of initial symptoms and clinical manifestation of two group patients. ② Differences of age of onset and modified Osserman classification of two groups. ③The humoral immuno-functions of two groups were compared. RESULTS： All the 49 patients were involved in the final analysis of results. ① Differences of initial symptoms： Ptosis was the most common initial symptoms in both groups. Patients with ptosis of normal thymus gland were 25 （83%, 25/30）. Patients with ptosis of abnormal thymus gland were 13 （68%, 13/19）. Patients with normal thymus gland： dysphagia 2 （7%, 2/30）, diplopia 4 （13%, 4/30）, fatigue 4 （13%, 4/30）, dysarthria 3, （10 %, 13/30）. Patients with abnormal thymus gland： dysphagia 3 （16%, 3/19）, diplopia 6 （32%, 6/19）, fatigue 3 （16%, 3/19）, dysarthria 2 （10%, 2/19）. ② Differences of clinical manifestation of two groups： Ptosis was the most common clinical manifestation in both groups. Patients with ptosis of normal thymus gland were 29 （97%, 29/30）. Patients with ptosis of abnormal thymus gland were 15 （79%, 15/19）. The rates of fatigue and breathing disorder in patients with abnormal thymus gland were higher than patients with normal thymus gland. Myasthenia crisis occurred in 3 patients （16 %, 3/19） in abnormal thymus gland group, with 1 （3%, 1/30） in abnormal thymus gland group. ③ Differences of age of onset and modified Osserman classification： The rate of type ｜ （63%, 19/30） in patients with normal thymus gland was higher than patients （42%, 8/19） with abnormal thymus gland. The rates of type Ⅱ a, Ⅱ b and Ⅲ （58 %） in patients with abnormal thymus gland were higher than patients （37%, 8/19） with normal thymus gland. But no differences were found between two groups （P 〉 0.05）. Patient number of onset from 20 to 29 year old in abnormal group （47%） was higher than that in normal group （20%）. Comparison of two groups was X2=4.10 and P 〈 0.05.④ Comparison of the humoral immunol indexes of two groups： The levels of IgG, IgA, C3 and CH50 in abnormal group were higher than those in normal group. But no differences were found between two groups （P 〉 0.05）. CONCLUSZON： ① Ptosis was the most common initial symptom and clinical feature in both groups. ② Clinical manifestation in abnormal group were more severe, and ages of onset in abnormal group were more young.③ The humoral immuno indexes of two groups were not significantly different.展开更多
Perioperative neonatal pneumothorax(NP)is rare but very fatal.Most of the surgeries and treatments in the neonatal period are time-limited or emergent,and there are often some risk factors for pneumothorax before surg...Perioperative neonatal pneumothorax(NP)is rare but very fatal.Most of the surgeries and treatments in the neonatal period are time-limited or emergent,and there are often some risk factors for pneumothorax before surgery.Physicians,surgeons and anesthesiologists need to identify possible risk factors for pneumothorax before surgery in preterm babies,patients receiving mechanical ventilation and those with underlying lung disease.The clinical presentation of NP is nonspecific,and patients may rapidly develop life-threatening complications if not promptly diagnosed and managed.This review highlights recent progress in the identification of risk factors,clinical manifestations,diagnosis and management of NP during the perioperative period.展开更多
BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been ...BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported successively. But its imageological detection needs to be further investigated. OBJECTIVE: To analyze the characteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE. DESIGN: Retrospective analysis. SETTING: The General Hospital of Chinese PLA. PARTICIPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives. METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem. MAIN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients. RESULTS: Ten patients with WE were involved in the final analysis. ①Nine patients presented various degrees of mental and conscious disturbance. Six patients initially presented vertigo, nausea, and vomiting. Five patients showed opthalmoplegia. Three patients presented hypotension (BP < 120/60 mm Hg, 1 mm Hg= 0.133 kPa). Two patients showed ataxia. One patient showed severe polyneuropathy. Both lower extremities were more severe. EMG showed axonal degeneration. ②Five patients accepted skull MRI examination. Three patients showed typical high signals in periaqueductal regions and circumference of third and fourth ventriculus in T2 -weighted and flair-weighted images, two of them showed high signals in fornixes in T2 -weighted and flair-weighted images, and one of them showed high signals in optic chiasma, both mammillary bodies, and subcortical white matter. No abnormality was found in the other two patients. Two patients who accepted the supplements of thiamine showed obviously improvements in the second MRI examination. ③Macroscopically, the border between gray and white matters was clear in the coronal section of cerebrum. Congestions, edema and multiple petechial hemorrhages were found in periaqueductal regions, circumference of third and fourth ventriculus, and both mammillary bodies. Microscopically, various degrees of necrosis of parenchymal structures, loss of nerve cells and ischemic changes were found. Myelinated fibers were more affected than neurons. There were focal capillary proliferation and multiple petechial hemorrhages. Prominent astrocytic proliferations were found in gelatinous fiber staining. And demyelinations were found in myelin staining. These pathological findings were all in accord with the diagnostic criteria of WE. ④In the diagnosis before death, 4 of 5 patients who were supplemented with thiamine had obvious improvement 2 weeks later and 1 of them abandoned therapy due to progressive aggravation of jaundice caused by recurrence of stomach cancer. The other 5 patients who were not diagnosed definitely before death and not supplemented with thiamine died. Final diagnosis was performed in autopsy examination. CONCLUSION: The clinical manifestations of this group of WE patients are atypical. MRI and pathological examination results are corresponding, and both have the characteristic manifestations of WE.展开更多
Dear Sir,We write to report on the clinical manifestations and outcomes of ocular sarcoidosis in patients presenting to uveitis referral centers in the Kingdom of Saudi Arabia.This is the first report focused solely o...Dear Sir,We write to report on the clinical manifestations and outcomes of ocular sarcoidosis in patients presenting to uveitis referral centers in the Kingdom of Saudi Arabia.This is the first report focused solely on ocular sarcoidosis from this country.展开更多
Background Respiratory syncytial virus(RSV)infection in infants is a global health priority.We aimed to investigate the common manifestations of RSV infection by age group and human development index(HDI)level and to ...Background Respiratory syncytial virus(RSV)infection in infants is a global health priority.We aimed to investigate the common manifestations of RSV infection by age group and human development index(HDI)level and to assess its association with the development of wheezing and recurrent wheezing illness.Methods We searched the literature published between January 1,2010 and June 2,2022 in seven databases.Outcomes included common manifestations and long-term respiratory outcomes of RSV infection in children.Random-and fixed-effect models were used to estimate the effect size and their 95%confidence intervals.Subgroup analysis was conducted by age and HDI levels.This review was registered in PROSPERO(CRD42022379401).Results The meta-analysis included 47 studies.The top five manifestations were cough(92%),nasal congestion(58%),rhinorrhea(53%),shortness of breath(50%),and dyspnea(47%).The clinical symptoms were most severe in infants.In our analysis,compared to very high and high HDI countries,fewer studies in medium HDI countries reported related manifestations,and no study in low HDI countries reported that.The RSV-infected infants were more likely to develop wheezing than the non-infected infants[odds ratio(OR),3.12;95%CI,2.59–3.76]and had a higher risk of developing wheezing illnesses after recovery(OR,2.60;95%CI,2.51–2.70).Conclusions Cough and shortness of breath are common manifestations of RSV infection.More attention should be given to infants and areas with low HDI levels.The current findings confirm an association between RSV infection and wheezing or recurrent wheezing illness.展开更多
Autoimmune pancreatitis(AIP)is a rare and emerging immune-mediated pancreatitis.The involvement of the pancreas with lymph-oplasmacytic infiltration and fibrosis on histology is the most common features and has been w...Autoimmune pancreatitis(AIP)is a rare and emerging immune-mediated pancreatitis.The involvement of the pancreas with lymph-oplasmacytic infiltration and fibrosis on histology is the most common features and has been well studied in the literature.It is characterized by a nonspecific presentation that mimics a malignant process.The purpose of this report is to explore the different clinical and paraclinical aspects of this disease,as well as the challenges posed by its management.The series are made up of three different cases of patients with elevated serum IgG4 levels and pancreatic changes.The first case initially revealed cholestatic jaundice with extrahepatic and intrahepatic cholangitis,and ultrasound reflected multiple occupying changes in the pancreas.The elevated serum IgG4 level was brilliant.Patient improved under the influence of corticosteroids.The second case was clinically asymptomatic,just found weight loss.Magnetic resonance revealed a round mass in the head of the pancreas and was considered as a neuroendocrine tumor.The patient showed slightly elevated serum IgG4 level,but was sensitive to hormone therapy.The third case concerned epigastric pain and enlarged lymph nodes.It was initially diagnosed as pancreatic cancer with distant metastasis,but also finally resolved by corticosteroid treatment.The clinical presentation of AIP is heterogeneous,as evidenced by our three clinical cases.Further observational and interventional studies are needed to better identify and manage this disease.展开更多
Background:Liver dysfunction was common in coronavirus disease 2019(COVID-19),but its association with clinical features and poor prognosis has not been fully delineated.Our study aimed to determine the role of liver ...Background:Liver dysfunction was common in coronavirus disease 2019(COVID-19),but its association with clinical features and poor prognosis has not been fully delineated.Our study aimed to determine the role of liver dysfunction in COVID-19 and understand the predictors for worse outcomes in patients with liver injury.Methods:We conducted this multicenter,retrospective study in five designated hospitals for COVID-19 manage-ment.Laboratory-confirmed COVID-19 patients were enrolled and classified into the normal live function group and liver dysfunction group according to liver enzymes,bilirubin,and albumin on admission,respectively.Data of baseline,clinical manifestations,and outcomes were collected and compared in the paired groups.Results:Of the 649 included COVID-19 patients,200(30.8%),69(10.6%),and 267(41.1%)patients had elevated liver enzymes,increased bilirubin,and low-level albumin,respectively.Fever,cough,and dyspnea were the most common symptoms and showed an increased proportion in the liver dysfunction group.Compared with patients in the normal liver function group,patients with liver dysfunction manifested decreased lymphocytes,higher level of leukocytes,neutrophils,inflammatory indicators,and cytokines,as well as more severe impairment in kidney function and myocardium.They were more likely to show bilateral involvement and more pulmonary lobes involved according to chest images.With increased proportion of patients who developed severe/critical conditions and needed mechanical ventilation and systemic glucocorticoid therapy,patients with liver dysfunc-tion on admission showed significantly higher in-hospital mortality.Moreover,cardiac troponin I≥1.5 ng/mL was identified as an independent mortality predictor in the elevated liver enzymes group.Conclusion:Patients with liver dysfunction on admission had worse clinical manifestation,and resulted in higher rate of severe/critical type,receiving mechanical ventilation and in-hospital mortality.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
The severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) epidemic has become a major challenge to public health in China and other countries, considering its pathogenicity across all age groups. Pregnancy is a ...The severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) epidemic has become a major challenge to public health in China and other countries, considering its pathogenicity across all age groups. Pregnancy is a unique physiological condition, and is characterized by altered immunity and elevated hormone levels to actively tolerate the semiallogeneic fetus, which undergoes a sudden and substantial fluctuation during the immediate postpartum period. Changes in clinical features, laboratory characteristics, and imaging features of pregnant women during the pre-partum and postpartum periods require further elucidation. Here, we retrospectively analyzed the clinical features, laboratory characteristics, and imaging features of eight pregnant cases of SARS-CoV-2 infection during the pre-partum and post-partum periods. Our results showed that four of the eight pregnant women were asymptomatic before delivery but became symptomatic post-partum. Correspondingly, white blood cell(WBC) counts increased and lymphocyte(LYMPH) counts decreased. C-reactive protein(CRP) levels in the serum also increased to a higher level than those in general pregnancy.Therefore, it is imperative to closely monitor laboratory parameters including the WBC count, LYMPH count, and CRP,along with other imaging features in chest CT scans, to promptly prevent, diagnose, and treat a SARS-CoV-2 infection during pregnancy.展开更多
Background Fibrodysplasia ossificans progressiva(FOP)is a rare and disabling heritable connective tissue disease that is difficult to treat.This study seeks to explore the clinical characteristics,clinical manifestati...Background Fibrodysplasia ossificans progressiva(FOP)is a rare and disabling heritable connective tissue disease that is difficult to treat.This study seeks to explore the clinical characteristics,clinical manifestations,treatment and prognosis of FOP to provide a clinical basis for its early diagnosis and treatment.Methods Twenty-six children with FOP were retrospectively analyzed in terms of their onset,clinical manifestations,auxiliary examinations and treatment.Results Among the 26 cases,the youngest age of manifestation of mass was 8 days after birth,and the average age was 3 years and 2 months.The peak age was 2-5 years old.Inflammatory mass and toe-finger deformity are the main early clinical manifestations of the disease.These inflammatory masses often lead to hard osteogenic deposits that initially mainly involve the central axis,such as the neck(22/26,84.6%),back(20/26,76.9%),and head(13/26,50%).Toe-finger deformity mainly manifests as symmetrical great toe deformity,or short and deformed thumb and little finger.The diagnosis of FOP requires typical clinical manifestations or ACVR1 gene detection.The main therapeutic drugs for FOP include glucocorticoids and non-steroidal anti-inflammatory drugs.Although not compliant with the recommended medical management of FOP,in our clinical practice children with uncontrollable illness could be treated using a variety of immunosuppressive agents in combination.Conclusions FOP is a rare autosomal dominant heritable disease.The main clinical manifestations observed in this study were recurrent inflammatory mass and toe-finger deformity.If the diagnosis and treatment are not performed in a timely manner,serious complications are likely to affect the prognosis.Therefore,early diagnosis and active treatment should be performed.展开更多
The novel coronavirus disease 2019(COVID-19)pandemic,caused by severe acute respiratory syndrome coronavirus 2,has become a global challenge of unprecedented nature since December 2019.Although most patients with COVI...The novel coronavirus disease 2019(COVID-19)pandemic,caused by severe acute respiratory syndrome coronavirus 2,has become a global challenge of unprecedented nature since December 2019.Although most patients with COVID-19 exhibit mild clinical manifestations and upper respiratory tract involvement,in approximately 5%-10%of patients,the disease is severe and involves multiple organs,leading to multi-organ dysfunction and failure.The liver and gastrointestinal tract are also frequently involved in COVID-19.In the context of liver involvement in patients with COVID-19,many key aspects need to be addressed in both native and transplanted organs.This review focuses on the clinical presentations and laboratory abnormalities of liver function tests in patients with COVID-19 with no prior liver disease,patients with pre-existing liver diseases and liver transplant recipients.A brief overview of the history of COVID-19 and etiopathogenesis of the liver injury will also be described as a prelude to better understanding the above aspects.展开更多
AIM:To provide comprehensive data on nonmetallic intraorbital foreign bodies(IOFBs)by summarizing and analyzing material types,clinical manifestations,imaging features,and treatment strategies.METHODS:Totally 28 nonme...AIM:To provide comprehensive data on nonmetallic intraorbital foreign bodies(IOFBs)by summarizing and analyzing material types,clinical manifestations,imaging features,and treatment strategies.METHODS:Totally 28 nonmetallic IOFB cases treated at Shengjing Hospital of China Medical University from 2012 to 2020 were retrospectively reviewed.The types of foreign bodies,clinical features,imaging manifestations,and treatment outcomes were analyzed.RESULTS:Among all cases,67.8%(19/28)of the foreign bodies were organic.The top three entrances were the upper eyelid skin(7/28),lower fornix conjunctiva(6/28),and lower eyelid skin(4/28).In most cases(11/28,39.3%),foreign bodies remained in the medial orbits.The major clinical manifestations included eyelid redness and swelling(20/28,71.4%),conjunctival congestion and edema(17/28,60.7%),and ophthalmoptosis(15/28,53.6%).Infection was the main complication,which occurred in 57.1%(16/28)of all cases.Computerized tomography(CT)values differed for different foreign bodies and varied in the different periods after injury.The plant-and grease-derived foreign bodies and the surrounding pus cysts showed different signals on magnetic resonance imaging(MRI).The prognosis varied with different foreign body types,surgery timing,and intraoperative management.CONCLUSION:The majority of nonmetallic IOFBs are organic and often remain in the superior,medial,and inferior areas of the orbit.Clinical manifestations vary owing to their different textures.CT and MRI facilitate the identification of foreign body materials.Plant-derived foreign bodies should be completely removed,and surgical treatment is a complicated process.展开更多
BACKGROUND:In clinical practice,some patients might not be able or unwilling to provide a thorough history of medication and poison exposure.The aim of this study was to use toxicological analysis to examine the clini...BACKGROUND:In clinical practice,some patients might not be able or unwilling to provide a thorough history of medication and poison exposure.The aim of this study was to use toxicological analysis to examine the clinical characteristics of patients with acute poisoning whose exposure history was uncertain from a toxicological analysis perspective.METHODS:This was a retrospective and descriptive study from an institute of poisoning.Patient registration information and test reports spanning the period from April 1,2020 to March 31,2022,were obtained.Patients with uncertain exposure histories and who underwent toxicological analysis were included.Clinical manifestations and categories of toxics were analyzed.RESULTS:Among the 195 patients with positive toxicological analysis results,the main causes of uncertain exposure history was disturbance of consciousness(62.6%),unawareness(23.6%)and unwillingness or lack of cooperation(13.8%).The predominant clinical manifestations were disturbed consciousness(62.6%),followed by vomiting and nausea(14.4%)and liver function abnormalities(8.7%).A comparison of clinical manifestations between patients with positive and negative(n=99)toxicological analyses results revealed significantly different proportions of disturbances in consciousness(63%vs.21%),dizziness(1.5%vs.5.1%),multi-organ failure(1.5%vs.7.1%),and local pain(0 vs 4%).The main categories of substances involved were psychiatric medications(23.1%),sedatives(20.5%),insecticides(13.8%),and herbicides(12.8%).CONCLUSION:The clinical manifestations of acute poisoning in patients with an uncertain exposure history are diverse and nonspecific,and toxicological analysis plays a pivotal role in the diagnosis and differential diagnosis of such patients.展开更多
Non-specific orbital inflammation(NSOI)is a noninfectious orbital inflammation.Although it is often considered the most common diagnosis in orbital biopsies,it is an exclusionary diagnosis that requires ruling out sys...Non-specific orbital inflammation(NSOI)is a noninfectious orbital inflammation.Although it is often considered the most common diagnosis in orbital biopsies,it is an exclusionary diagnosis that requires ruling out systemic disease or other possible causes.Its characteristics include acute orbital signs and symptoms,including pain,proptosis,periorbital edema,chemosis,diplopia,and visual impairment.The clinical manifestations and histological findings of NSOI are heterogeneous,without specific diagnostic criteria or treatment guidelines,which poses significant challenges for diagnosis and treatment.This guideline provides a detailed description of the definition,classification,diagnosis,and treatment of NSOI.展开更多
基金Supported by National High Level Hospital Clinical Research Funding,No.2022-PUMCH-B-022 and No.2022-PUMCH-D-002CAMS Innovation Fund for Medical Sciences,No.2021-1-I2M-003+1 种基金Undergraduate Innovation Program,No.2023-zglc-06034National Key Clinical Specialty Construction Project,No.ZK108000。
文摘BACKGROUND Autoimmune enteropathy(AIE)is a rare disease whose diagnosis and long-term prognosis remain challenging,especially for adult AIE patients.AIM To improve overall understanding of this disease’s diagnosis and prognosis.METHODS We retrospectively analyzed the clinical,endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023,whose diagnosis was based on the 2007 diagnostic criteria.RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea.The common endoscopic manifestations were edema,villous blunting and mucosal hyperemia in the duodenum and ileum.Villous blunting(100%),deep crypt lymphocytic infiltration(67%),apoptotic bodies(50%),and mild intraepithelial lymphocytosis(69%)were observed in the duodenal biopsies.Moreover,there were other remarkable abnormalities,including reduced or absent goblet cells(duodenum 94%,ileum 62%),reduced or absent Paneth cells(duodenum 94%,ileum 69%)and neutrophil infiltration(duodenum 100%,ileum 69%).Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies.All patients received glucocorticoid therapy as the initial medication,of which 14/16 patients achieved a clinical response in 5(IQR:3-20)days.Immunosuppressants were administered to 9 patients with indications of steroid dependence(6/9),steroid refractory status(2/9),or intensified maintenance medication(1/9).During the median of 20.5 months of followup,2 patients died from multiple organ failure,and 1 was diagnosed with non-Hodgkin’s lymphoma.The cumulative relapse-free survival rates were 62.5%,55.6%and 37.0%at 6 months,12 months and 48 months,respectively.CONCLUSION Certain histopathological findings,including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies,might be potential diagnostic criteria for adult AIE.The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications,which highlights the need for early diagnosis and novel medications.
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.
基金Supported by the Tongji-Rockcheck Life Science and Medicine Research Center(No.202014)。
文摘·AIM:To report a case which keratitis is the first clinical manifestation of COVID-19 that occurred 3 d earlier than the common COVID-19 symptoms.·METHODS:Regular slit lamp examination,corneal scraping test,and chest computed tomography(CT)were performed for patients with COVID-19 infection.The ophthalmologic treatment included ganciclovir eye drop(50 mglmL,6 times/d).The treatment for diarrhea included Guifu Lizhong pills(TID).The antiviral therapy consisted of oseltamivir(75 mg capsule Q12 H);therapy preventing bacterial infection consisted of azithromycin(250 mg tablet QD)and moxifloxacin(0.4 g tablet Q12 H);and therapy for cough relief and fever prevention consisted of Chinese herbal decoction.·RESULTS:A 35-year-old male suddenly suffered pain,photophobia,and tears in his right eye for one day without systemic COVID-19 symptoms.Patient was diagnosed with keratitis,which was seemingly different from common keratitis.Ganciclovir eye drop was initiated.The corneal scraping test for COVID-19 was positive.The chest CT images were abnormal confirming the diagnosis of COVID-19 infection.The antiviral and antibacterial therapies were initiated.Chinese herbal therapy was used for cough relief and fever prevention.After roughly two weeks,patient recovered from COVID-19.·CONCLUSION:A new type of keratitis,atypical keratitis,is a clinical manifestation of COVID-19,and this clinical manifestation could appear 3 d earlier than fever and cough.The earlier a COVID-19 clinical manifestation is identified,the earlier can a patient be directed to stay at home,and significantly fewer people would be infected.
文摘BACKGROUND: Recent advances in molecular and genomic technologies and pancreatic imaging techniques provided some insights into genetic, environmental, immunologic, and pathobiological factors for chronic pancreatitis (CP). This study was undertaken to investigate the clinical manifestations of patients with chronic pancreatitis at our hospital. METHODS: The data of the patients with CP who had been treated at our hospital between 1997 and 2004 were analyzed. RESULTS: The major symptoms of the patients with CP were abdominal pain, dyspepsia, loss of weight, diabetes mellitus, pancreatic pseudocyst, steatorrhea, and calcification. Biliary diseases were found to be the first cause of CP in this study; but alcohol abuse was the major cause of CP in men and biliary diseases were the first etiological factors for CP in women. The etiological difference of constituent ratio between men and women was related to alcohol comsumption (P<0.01). CONCLUSIONS: During the past 8 years, biliary diseases have been the major etiological factors for CP, but their constituent ratio is decreasing, and the constituent ratio of alcohol abuse is increasing gradually. Alcohol tends to replace biliary diseases as the primary etiological factor for CP.
文摘BACKGROUND: Accurate diagnosis of infected aortic and iliac aneurysms is often delayed, hampering timely treatment and potentially resulting in a fatal consequence. The aim of this study was to discover useful clinical features that can help physicians to identify these patients.METHODS: We reviewed the discharge notes from two hospitals and identifi ed all patients who had a diagnosis of infected aneurysms of the thoracoabdominal aorta and iliac arteries between July 2009 and December 2013. Eighteen patients, aged from 41 to 93, were reviewed. Only 6 patients were diagnosed accurately in their fi rst visit to our ED.RESULTS: Most patients had at least one underlying illness, and it took 1 to 30(9.9±6.5) days for physicians to diagnose their infected aneurysm. Localized pain and fever were the two most commonly presented symptoms. The majority(92%) of isolated microorganisms were gram-negative bacilli, including Salmonella spp, Klebsiella pneumoniae, and Escherichia coli. Two of the 3 patients who underwent non-operative therapy died, and all of the patients who underwent a combination of medical and operative therapies survived.CONCLUSION: We suggest that physicians liberally use computed tomography scans on patients with unknown causes of pain and inflammatory processes. A combination of surgical and medical treatments is indicated for all patients with infected aortic and iliac aneurysms.
基金the Medical Science Foundation of Guangdong Province, No. A20052363
文摘BACKGROUND： Myasthenia gravis （MG） is an autoimmune disease which mainly affects neuromuscular junctions. The ages, modified Osserman classification and clinical manifestation and humoral immunol function of MG with and without thymic abnormality are different. OBJECTIVE： To explore the clinical manifestation and humoral immuno-function of MG with abnormal and normal thymus gland. DESIGN ： Contrast observation SETTTNG ： Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University PARTZCZPANTS ： A total of 49 inpatients with MG were selected from the Third Affiliated Hospital of Sun Yat-sen University from March 2000 to August 2005. All the patients had typical clinical manifestation of MG and positive neostigmine test. All the patients knew and agreed the laboratory examinations. There were 22 males and 27 females of 2-69 years old. Chest MRI or CT scan were performed to reveal thymus gland abnormality. According to whether there was tumor in superior mediastinum, all patients were divided into 2 groups, abnormal and normal groups. Normal thymus gland group （n=30） contained 16 males and 14 females of 6-43 years old. Abnormal thymus gland group （n=19） contained 6 male and 13 female of 2-69 years old, METHODS： ① All patients were questioned about initial symptoms. Meanwhile, main clinical manifestations were recorded at hospital admission. ② 7180A automatic biochemical analyzer and automatic microplate reader were used in detecting seroimmunity index. The levels of C3, C4, IgG, IgA, IgM and CH50 in blood serum were analyzed by nephelometry. ③ Clinical classification is based on modified Osserman classification. The patients with MG were divided into six types： I （Ocular myasthenia）, Ⅱ a （Mild generalized myasthenia）, Ⅱb （Moderately severe generalized myasthenia）, Ⅲ （Acute fulminating myasthenia）, Ⅳ（Late se- vere myasthenia）. MAZN OUTCOME MEASURES： ① Differences of initial symptoms and clinical manifestation of two group patients. ② Differences of age of onset and modified Osserman classification of two groups. ③The humoral immuno-functions of two groups were compared. RESULTS： All the 49 patients were involved in the final analysis of results. ① Differences of initial symptoms： Ptosis was the most common initial symptoms in both groups. Patients with ptosis of normal thymus gland were 25 （83%, 25/30）. Patients with ptosis of abnormal thymus gland were 13 （68%, 13/19）. Patients with normal thymus gland： dysphagia 2 （7%, 2/30）, diplopia 4 （13%, 4/30）, fatigue 4 （13%, 4/30）, dysarthria 3, （10 %, 13/30）. Patients with abnormal thymus gland： dysphagia 3 （16%, 3/19）, diplopia 6 （32%, 6/19）, fatigue 3 （16%, 3/19）, dysarthria 2 （10%, 2/19）. ② Differences of clinical manifestation of two groups： Ptosis was the most common clinical manifestation in both groups. Patients with ptosis of normal thymus gland were 29 （97%, 29/30）. Patients with ptosis of abnormal thymus gland were 15 （79%, 15/19）. The rates of fatigue and breathing disorder in patients with abnormal thymus gland were higher than patients with normal thymus gland. Myasthenia crisis occurred in 3 patients （16 %, 3/19） in abnormal thymus gland group, with 1 （3%, 1/30） in abnormal thymus gland group. ③ Differences of age of onset and modified Osserman classification： The rate of type ｜ （63%, 19/30） in patients with normal thymus gland was higher than patients （42%, 8/19） with abnormal thymus gland. The rates of type Ⅱ a, Ⅱ b and Ⅲ （58 %） in patients with abnormal thymus gland were higher than patients （37%, 8/19） with normal thymus gland. But no differences were found between two groups （P 〉 0.05）. Patient number of onset from 20 to 29 year old in abnormal group （47%） was higher than that in normal group （20%）. Comparison of two groups was X2=4.10 and P 〈 0.05.④ Comparison of the humoral immunol indexes of two groups： The levels of IgG, IgA, C3 and CH50 in abnormal group were higher than those in normal group. But no differences were found between two groups （P 〉 0.05）. CONCLUSZON： ① Ptosis was the most common initial symptom and clinical feature in both groups. ② Clinical manifestation in abnormal group were more severe, and ages of onset in abnormal group were more young.③ The humoral immuno indexes of two groups were not significantly different.
文摘Perioperative neonatal pneumothorax(NP)is rare but very fatal.Most of the surgeries and treatments in the neonatal period are time-limited or emergent,and there are often some risk factors for pneumothorax before surgery.Physicians,surgeons and anesthesiologists need to identify possible risk factors for pneumothorax before surgery in preterm babies,patients receiving mechanical ventilation and those with underlying lung disease.The clinical presentation of NP is nonspecific,and patients may rapidly develop life-threatening complications if not promptly diagnosed and managed.This review highlights recent progress in the identification of risk factors,clinical manifestations,diagnosis and management of NP during the perioperative period.
文摘BACKGROUND: The clinical manifestations of Wernicke encephalopathy(WE) are atypical and short of effective auxiliary examination means. The effects of magnetic resonance imaging (MRI) in the diagnosis of WE have been reported successively. But its imageological detection needs to be further investigated. OBJECTIVE: To analyze the characteristics of clinical manifestations, skull MRI examination and pathological results in patients with WE. DESIGN: Retrospective analysis. SETTING: The General Hospital of Chinese PLA. PARTICIPANTS: Ten patients of WE admitted to the Department of Neurology, General Hospital of Chinese PLA were recruited. Among them, five patients were diagnosed pathologically after death. Their pathological changes accorded with the pathological characteristics of WE. The other 5 patients were diagnosed clinically before death. Their pathological changes accorded with clinical and imageological manifestations and had definite reaction to the treatment of thiamine. Ten patients, 7 males and 3 females, were aged (47±13) years ranging from 33 to 73 years. Their disease courses averaged 6 weeks ranging from 3 to 10 weeks. They all were non-alcoholics. Four patients developed WE after acute pancreatitis, two patients after the recurrence of gastric cancer, two patients after cholecystectomy, one patient after hepatitis medicamentosa, one patient after Alzheimer disease. Informed consents were obtained from all the patients and their relatives. METHODS: After admission, clinical manifestations of patients were observed and recorded. Five patients underwent skull MRI examination and their detected results were recorded. Five dead patients underwent autopsy and brain pathological examinations. Neuropathological examination involved cerebrum, cerebellum and brain stem. MAIN OUTCOME MEASURES: Clinical manifestations, MRI examination results, pathological analysis results and prognosis of all the patients. RESULTS: Ten patients with WE were involved in the final analysis. ①Nine patients presented various degrees of mental and conscious disturbance. Six patients initially presented vertigo, nausea, and vomiting. Five patients showed opthalmoplegia. Three patients presented hypotension (BP < 120/60 mm Hg, 1 mm Hg= 0.133 kPa). Two patients showed ataxia. One patient showed severe polyneuropathy. Both lower extremities were more severe. EMG showed axonal degeneration. ②Five patients accepted skull MRI examination. Three patients showed typical high signals in periaqueductal regions and circumference of third and fourth ventriculus in T2 -weighted and flair-weighted images, two of them showed high signals in fornixes in T2 -weighted and flair-weighted images, and one of them showed high signals in optic chiasma, both mammillary bodies, and subcortical white matter. No abnormality was found in the other two patients. Two patients who accepted the supplements of thiamine showed obviously improvements in the second MRI examination. ③Macroscopically, the border between gray and white matters was clear in the coronal section of cerebrum. Congestions, edema and multiple petechial hemorrhages were found in periaqueductal regions, circumference of third and fourth ventriculus, and both mammillary bodies. Microscopically, various degrees of necrosis of parenchymal structures, loss of nerve cells and ischemic changes were found. Myelinated fibers were more affected than neurons. There were focal capillary proliferation and multiple petechial hemorrhages. Prominent astrocytic proliferations were found in gelatinous fiber staining. And demyelinations were found in myelin staining. These pathological findings were all in accord with the diagnostic criteria of WE. ④In the diagnosis before death, 4 of 5 patients who were supplemented with thiamine had obvious improvement 2 weeks later and 1 of them abandoned therapy due to progressive aggravation of jaundice caused by recurrence of stomach cancer. The other 5 patients who were not diagnosed definitely before death and not supplemented with thiamine died. Final diagnosis was performed in autopsy examination. CONCLUSION: The clinical manifestations of this group of WE patients are atypical. MRI and pathological examination results are corresponding, and both have the characteristic manifestations of WE.
文摘Dear Sir,We write to report on the clinical manifestations and outcomes of ocular sarcoidosis in patients presenting to uveitis referral centers in the Kingdom of Saudi Arabia.This is the first report focused solely on ocular sarcoidosis from this country.
基金supported by the Non-profit Central Research Institute Fund of the Chinese Academy of Medical Sciences(CAMS)(grant number 2021-RC330-002)the Disease Burden of Respiratory Syncytial Virus and Policy Recommendations Project(grant number 202108180001).
文摘Background Respiratory syncytial virus(RSV)infection in infants is a global health priority.We aimed to investigate the common manifestations of RSV infection by age group and human development index(HDI)level and to assess its association with the development of wheezing and recurrent wheezing illness.Methods We searched the literature published between January 1,2010 and June 2,2022 in seven databases.Outcomes included common manifestations and long-term respiratory outcomes of RSV infection in children.Random-and fixed-effect models were used to estimate the effect size and their 95%confidence intervals.Subgroup analysis was conducted by age and HDI levels.This review was registered in PROSPERO(CRD42022379401).Results The meta-analysis included 47 studies.The top five manifestations were cough(92%),nasal congestion(58%),rhinorrhea(53%),shortness of breath(50%),and dyspnea(47%).The clinical symptoms were most severe in infants.In our analysis,compared to very high and high HDI countries,fewer studies in medium HDI countries reported related manifestations,and no study in low HDI countries reported that.The RSV-infected infants were more likely to develop wheezing than the non-infected infants[odds ratio(OR),3.12;95%CI,2.59–3.76]and had a higher risk of developing wheezing illnesses after recovery(OR,2.60;95%CI,2.51–2.70).Conclusions Cough and shortness of breath are common manifestations of RSV infection.More attention should be given to infants and areas with low HDI levels.The current findings confirm an association between RSV infection and wheezing or recurrent wheezing illness.
基金This work was supported by the Taishan Scholars Program for Young Expert of Shandong Province(tsqn202103172)Clinical Research Foundation of Shandong University(2020SDUCRCC016).
文摘Autoimmune pancreatitis(AIP)is a rare and emerging immune-mediated pancreatitis.The involvement of the pancreas with lymph-oplasmacytic infiltration and fibrosis on histology is the most common features and has been well studied in the literature.It is characterized by a nonspecific presentation that mimics a malignant process.The purpose of this report is to explore the different clinical and paraclinical aspects of this disease,as well as the challenges posed by its management.The series are made up of three different cases of patients with elevated serum IgG4 levels and pancreatic changes.The first case initially revealed cholestatic jaundice with extrahepatic and intrahepatic cholangitis,and ultrasound reflected multiple occupying changes in the pancreas.The elevated serum IgG4 level was brilliant.Patient improved under the influence of corticosteroids.The second case was clinically asymptomatic,just found weight loss.Magnetic resonance revealed a round mass in the head of the pancreas and was considered as a neuroendocrine tumor.The patient showed slightly elevated serum IgG4 level,but was sensitive to hormone therapy.The third case concerned epigastric pain and enlarged lymph nodes.It was initially diagnosed as pancreatic cancer with distant metastasis,but also finally resolved by corticosteroid treatment.The clinical presentation of AIP is heterogeneous,as evidenced by our three clinical cases.Further observational and interventional studies are needed to better identify and manage this disease.
基金This study was supported by the National Natural Science Foun-dation of China(No.81630001)the Shanghai Municipal Key Clini-cal Specialty(No.shslczdzk02202)+2 种基金the Shanghai Top-Priority Clinical Key Disciplines Construction Project(No.2017ZZ02014)the Shanghai Shenkang Hospital Development Center Clinical Science and Technol-ogy Innovation Project(No.SHDC12018102)the Innovative Re-search Team of High-level Local Universities in Shanghai and Zhejiang University special scientific research fund for COVID-19 prevention and control(No.2020XGZX011).
文摘Background:Liver dysfunction was common in coronavirus disease 2019(COVID-19),but its association with clinical features and poor prognosis has not been fully delineated.Our study aimed to determine the role of liver dysfunction in COVID-19 and understand the predictors for worse outcomes in patients with liver injury.Methods:We conducted this multicenter,retrospective study in five designated hospitals for COVID-19 manage-ment.Laboratory-confirmed COVID-19 patients were enrolled and classified into the normal live function group and liver dysfunction group according to liver enzymes,bilirubin,and albumin on admission,respectively.Data of baseline,clinical manifestations,and outcomes were collected and compared in the paired groups.Results:Of the 649 included COVID-19 patients,200(30.8%),69(10.6%),and 267(41.1%)patients had elevated liver enzymes,increased bilirubin,and low-level albumin,respectively.Fever,cough,and dyspnea were the most common symptoms and showed an increased proportion in the liver dysfunction group.Compared with patients in the normal liver function group,patients with liver dysfunction manifested decreased lymphocytes,higher level of leukocytes,neutrophils,inflammatory indicators,and cytokines,as well as more severe impairment in kidney function and myocardium.They were more likely to show bilateral involvement and more pulmonary lobes involved according to chest images.With increased proportion of patients who developed severe/critical conditions and needed mechanical ventilation and systemic glucocorticoid therapy,patients with liver dysfunc-tion on admission showed significantly higher in-hospital mortality.Moreover,cardiac troponin I≥1.5 ng/mL was identified as an independent mortality predictor in the elevated liver enzymes group.Conclusion:Patients with liver dysfunction on admission had worse clinical manifestation,and resulted in higher rate of severe/critical type,receiving mechanical ventilation and in-hospital mortality.
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.
文摘The severe acute respiratory syndrome coronavirus 2(SARS-CoV-2) epidemic has become a major challenge to public health in China and other countries, considering its pathogenicity across all age groups. Pregnancy is a unique physiological condition, and is characterized by altered immunity and elevated hormone levels to actively tolerate the semiallogeneic fetus, which undergoes a sudden and substantial fluctuation during the immediate postpartum period. Changes in clinical features, laboratory characteristics, and imaging features of pregnant women during the pre-partum and postpartum periods require further elucidation. Here, we retrospectively analyzed the clinical features, laboratory characteristics, and imaging features of eight pregnant cases of SARS-CoV-2 infection during the pre-partum and post-partum periods. Our results showed that four of the eight pregnant women were asymptomatic before delivery but became symptomatic post-partum. Correspondingly, white blood cell(WBC) counts increased and lymphocyte(LYMPH) counts decreased. C-reactive protein(CRP) levels in the serum also increased to a higher level than those in general pregnancy.Therefore, it is imperative to closely monitor laboratory parameters including the WBC count, LYMPH count, and CRP,along with other imaging features in chest CT scans, to promptly prevent, diagnose, and treat a SARS-CoV-2 infection during pregnancy.
文摘Background Fibrodysplasia ossificans progressiva(FOP)is a rare and disabling heritable connective tissue disease that is difficult to treat.This study seeks to explore the clinical characteristics,clinical manifestations,treatment and prognosis of FOP to provide a clinical basis for its early diagnosis and treatment.Methods Twenty-six children with FOP were retrospectively analyzed in terms of their onset,clinical manifestations,auxiliary examinations and treatment.Results Among the 26 cases,the youngest age of manifestation of mass was 8 days after birth,and the average age was 3 years and 2 months.The peak age was 2-5 years old.Inflammatory mass and toe-finger deformity are the main early clinical manifestations of the disease.These inflammatory masses often lead to hard osteogenic deposits that initially mainly involve the central axis,such as the neck(22/26,84.6%),back(20/26,76.9%),and head(13/26,50%).Toe-finger deformity mainly manifests as symmetrical great toe deformity,or short and deformed thumb and little finger.The diagnosis of FOP requires typical clinical manifestations or ACVR1 gene detection.The main therapeutic drugs for FOP include glucocorticoids and non-steroidal anti-inflammatory drugs.Although not compliant with the recommended medical management of FOP,in our clinical practice children with uncontrollable illness could be treated using a variety of immunosuppressive agents in combination.Conclusions FOP is a rare autosomal dominant heritable disease.The main clinical manifestations observed in this study were recurrent inflammatory mass and toe-finger deformity.If the diagnosis and treatment are not performed in a timely manner,serious complications are likely to affect the prognosis.Therefore,early diagnosis and active treatment should be performed.
文摘The novel coronavirus disease 2019(COVID-19)pandemic,caused by severe acute respiratory syndrome coronavirus 2,has become a global challenge of unprecedented nature since December 2019.Although most patients with COVID-19 exhibit mild clinical manifestations and upper respiratory tract involvement,in approximately 5%-10%of patients,the disease is severe and involves multiple organs,leading to multi-organ dysfunction and failure.The liver and gastrointestinal tract are also frequently involved in COVID-19.In the context of liver involvement in patients with COVID-19,many key aspects need to be addressed in both native and transplanted organs.This review focuses on the clinical presentations and laboratory abnormalities of liver function tests in patients with COVID-19 with no prior liver disease,patients with pre-existing liver diseases and liver transplant recipients.A brief overview of the history of COVID-19 and etiopathogenesis of the liver injury will also be described as a prelude to better understanding the above aspects.
文摘AIM:To provide comprehensive data on nonmetallic intraorbital foreign bodies(IOFBs)by summarizing and analyzing material types,clinical manifestations,imaging features,and treatment strategies.METHODS:Totally 28 nonmetallic IOFB cases treated at Shengjing Hospital of China Medical University from 2012 to 2020 were retrospectively reviewed.The types of foreign bodies,clinical features,imaging manifestations,and treatment outcomes were analyzed.RESULTS:Among all cases,67.8%(19/28)of the foreign bodies were organic.The top three entrances were the upper eyelid skin(7/28),lower fornix conjunctiva(6/28),and lower eyelid skin(4/28).In most cases(11/28,39.3%),foreign bodies remained in the medial orbits.The major clinical manifestations included eyelid redness and swelling(20/28,71.4%),conjunctival congestion and edema(17/28,60.7%),and ophthalmoptosis(15/28,53.6%).Infection was the main complication,which occurred in 57.1%(16/28)of all cases.Computerized tomography(CT)values differed for different foreign bodies and varied in the different periods after injury.The plant-and grease-derived foreign bodies and the surrounding pus cysts showed different signals on magnetic resonance imaging(MRI).The prognosis varied with different foreign body types,surgery timing,and intraoperative management.CONCLUSION:The majority of nonmetallic IOFBs are organic and often remain in the superior,medial,and inferior areas of the orbit.Clinical manifestations vary owing to their different textures.CT and MRI facilitate the identification of foreign body materials.Plant-derived foreign bodies should be completely removed,and surgical treatment is a complicated process.
基金supported by National Natural Science Foundation of China(82172184)。
文摘BACKGROUND:In clinical practice,some patients might not be able or unwilling to provide a thorough history of medication and poison exposure.The aim of this study was to use toxicological analysis to examine the clinical characteristics of patients with acute poisoning whose exposure history was uncertain from a toxicological analysis perspective.METHODS:This was a retrospective and descriptive study from an institute of poisoning.Patient registration information and test reports spanning the period from April 1,2020 to March 31,2022,were obtained.Patients with uncertain exposure histories and who underwent toxicological analysis were included.Clinical manifestations and categories of toxics were analyzed.RESULTS:Among the 195 patients with positive toxicological analysis results,the main causes of uncertain exposure history was disturbance of consciousness(62.6%),unawareness(23.6%)and unwillingness or lack of cooperation(13.8%).The predominant clinical manifestations were disturbed consciousness(62.6%),followed by vomiting and nausea(14.4%)and liver function abnormalities(8.7%).A comparison of clinical manifestations between patients with positive and negative(n=99)toxicological analyses results revealed significantly different proportions of disturbances in consciousness(63%vs.21%),dizziness(1.5%vs.5.1%),multi-organ failure(1.5%vs.7.1%),and local pain(0 vs 4%).The main categories of substances involved were psychiatric medications(23.1%),sedatives(20.5%),insecticides(13.8%),and herbicides(12.8%).CONCLUSION:The clinical manifestations of acute poisoning in patients with an uncertain exposure history are diverse and nonspecific,and toxicological analysis plays a pivotal role in the diagnosis and differential diagnosis of such patients.
基金Supported by National Natural Science Foundation of China(No.82160195,No.82460203)Jiangxi Double-Thousand Plan High-Level Talent Project of Science and Technology Innovation(No.jxsq2023201036)Key R&D Program of Jiangxi Province(No.20223BBH80014).
文摘Non-specific orbital inflammation(NSOI)is a noninfectious orbital inflammation.Although it is often considered the most common diagnosis in orbital biopsies,it is an exclusionary diagnosis that requires ruling out systemic disease or other possible causes.Its characteristics include acute orbital signs and symptoms,including pain,proptosis,periorbital edema,chemosis,diplopia,and visual impairment.The clinical manifestations and histological findings of NSOI are heterogeneous,without specific diagnostic criteria or treatment guidelines,which poses significant challenges for diagnosis and treatment.This guideline provides a detailed description of the definition,classification,diagnosis,and treatment of NSOI.
