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
The authors report a case of deficient sensory neuropathy secondary to vitamin B12 deficiency, diagnosed in the neurology department of the Sino-Central African Friendship University Hospital in Bangui. The diagnosis ...The authors report a case of deficient sensory neuropathy secondary to vitamin B12 deficiency, diagnosed in the neurology department of the Sino-Central African Friendship University Hospital in Bangui. The diagnosis was made possible by electroneuromyography which showed subclinical neurological damage associated with hematological damage (anemia). Through this observation, we recall the diagnostic criteria of the disease in a context of difficult medical practice. .展开更多
Background: The Coronavirus disease 2019 (COVID-19) is a respiratory infectious disease, also named Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), which can cause various systemic manifestations that po...Background: The Coronavirus disease 2019 (COVID-19) is a respiratory infectious disease, also named Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), which can cause various systemic manifestations that pose a threat to human life. Oral lesions in patients with COVID-19 may appear during or after the illness and may or may not be a consequence of the viral infection. Objective: In this case series we compare the oral manifestations in hospitalized COVID-19 positive patients and COVID-19 negative dental outpatients. Methods: 60 hospitalized COVID-19 patients and 41 control patients, were examined for oral signs and symptoms. The controls were dental patients who visited the hospital for dental care without complaining of any problems related to the oral cavity itself. Results: We have observed a strong association between certain clinical findings and COVID-19, including alterations in taste (ageusia, dysgeusia, and hypogeusia), anosmia, hairy tongue, tongue imprints, red tongue, erythematous candidiasis, pseudomembranous candidiasis, and exfoliative cheilitis. A trend but not statistically significant association at the level of 5% was also noted for colored tongue, linea alba, and pale mucosa. On the contrary, fissured tongue and oral mucosa pigmentation were more frequent in the controls, statistically significant at the level of 5%. Conclusion: COVID-19 has been found to impact the oral cavity, resulting in various oral lesions that can be attributed to either the direct action of the virus or the patient’s immune response.展开更多
Objective:To evaluate coagulation abnormalities and their relationship with bleeding manifestations among patients with dengue.Methods:This observational study was conducted on 292 adult dengue patients who were admit...Objective:To evaluate coagulation abnormalities and their relationship with bleeding manifestations among patients with dengue.Methods:This observational study was conducted on 292 adult dengue patients who were admitted to a tertiary care hospital of Western India from July 2021 to June 2022.Coagulation tests including prothrombin time(PT),international normalized ratio(INR),activated partial thromboplastin time(aPTT),fibrinogen,and D-dimer were performed.Patients were monitored for bleeding manifestations.Results:Coagulation abnormalities were reported in 42.8%of the patients.Overall,prolonged aPTT was the most common coagulation abnormality(40.8%),followed by low fibrinogen(38.7%),raised D-dimer(31.2%),raised INR(26.0%)and prolonged PT(19.2%).Bleeding manifestations were present in 19.9%patients.PT,INR,aPTT and D-dimer levels were significantly higher(P<0.01)and fibrinogen level was significantly lower(P<0.001)in patients with bleeding compared to patients without bleeding.Patients with bleeding had a significantly higher rate of all coagulation abnormalities than patients without bleeding(P<0.01).Conclusions:Patients with bleeding showed a significantly higher frequency of coagulation abnormalities compared to patients without bleeding.Patients with dengue should be assessed for coagulation abnormalities.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
The coronavirus disease 2019(COVID-19)initially presented as a disease that affected the lungs.Then,studies revealed that it intricately affected disparate organs in the human body,with the liver being one of the most...The coronavirus disease 2019(COVID-19)initially presented as a disease that affected the lungs.Then,studies revealed that it intricately affected disparate organs in the human body,with the liver being one of the most affected organs.This review aimed to assess the association between COVID-19 and liver function,shedding light on its clinical implication.However,its exact pathophysiology remains unclear,involving many factors,such as active viral replication in the liver cells,direct cytotoxic effects of the virus on the liver or adverse reactions to viral antigens.Liver symptoms are mild-to-moderate transaminase elevation.In some patients,with underlying liver disease,more serious outcomes are observed.Thus,liver function should be meticulously considered in patients with COVID-19.展开更多
BACKGROUND Hepatobiliary manifestations occur in ulcerative colitis(UC)patients.The effect of laparoscopic restorative proctocolectomy(LRP)with ileal pouch anal anastomosis(IPAA)on hepatobiliary manifestations is deba...BACKGROUND Hepatobiliary manifestations occur in ulcerative colitis(UC)patients.The effect of laparoscopic restorative proctocolectomy(LRP)with ileal pouch anal anastomosis(IPAA)on hepatobiliary manifestations is debated.AIM To evaluate hepatobiliary changes after two-stages elective laparoscopic restorative proctocolectomy for patients with UC.METHODS Between June 2013 and June 2018,167 patients with hepatobiliary symptoms underwent two-stage elective LRP for UC in a prospective observational study.Patients with UC and having at least one hepatobiliary manifestation who underwent LRP with IPAA were included in the study.The patients were followed up for four years to assess the outcomes of hepatobiliary manifestations.RESULTS The patients'mean age was 36±8 years,and males predominated(67.1%).The most common hepatobiliary diagnostic method was liver biopsy(85.6%),followed by Magnetic resonance cholangiopancreatography(63.5%),Antineutrophil cytoplasmic antibodies(62.5%),abdominal ultrasonography(35.9%),and Endoscopic retrograde cholangiopancreatography(6%).The most common hepatobiliary symptom was Primary sclerosing cholangitis(PSC)(62.3%),followed by fatty liver(16.8%)and gallbladder stone(10.2%).66.4%of patients showed a stable course after surgery.Progressive or regressive courses occurred in 16.8%of each.Mortality was 6%,and recurrence or progression of symptoms required surgery for 15%.Most PSC patients(87.5%)had a stable course,and only 12.5%became worse.Two-thirds(64.3%)of fatty liver patients showed a regressive course,while one-third(35.7%)showed a stable course.Survival rates were 98.8%,97%,95.8%,and 94%at 12 mo,24 mo,36 mo,and at the end of the follow-up.CONCLUSION In patients with UC who had LRP,there is a positive impact on hepatobiliary disease.It caused an improvement in PSC and fatty liver disease.The most prevalent unchanged course was PSC,while the most common improvement was fatty liver disease.展开更多
BACKGROUND Epstein–Barr virus(EBV)-positive mucocutaneous ulcers(MCUs)are an uncommon disorder characterized by ulcerative lesions in the skin,oral cavity or gastrointestinal tract in patients with iatrogenic or agin...BACKGROUND Epstein–Barr virus(EBV)-positive mucocutaneous ulcers(MCUs)are an uncommon disorder characterized by ulcerative lesions in the skin,oral cavity or gastrointestinal tract in patients with iatrogenic or aging-induced immunosuppression.The nonspecific lesions are difficult to differentiate from small bowel adenocarcinomas.We present the case of a 69-year-old woman who was initially misdiagnosed with a small bowel adenocarcinoma but was later surgically diagnosed with and treated for EBV-MCU.Through this case,we aim to emphasize the importance of accurately distinguishing between the two conditions.CASE SUMMARY The patient presented with an incidental finding of a small bowel tumor during computed tomography(CT)examination performed for hematuria.The CT scan showed irregular thickening of the distal ileum,which was suggestive of a malignant small bowel tumor.An exploratory laparotomy revealed an 8-cm mass in the distal ileum;thus,a segment of the small intestine,including the mass,was resected.Histopathological analysis revealed an ulceroinfiltrative mass-like lesion with luminal narrowing,marked inflammatory cell infiltration,and large atypical lymphoid cells(positive for EBV-encoded small RNA).A final diagnosis of an EBV-MCU was established.The postoperative course was uneventful,and the patient was discharged on postoperative day 7.The patient remained recurrencefree until 12 mo after surgery.CONCLUSION This case highlights the diagnostic challenges for EBV-MCUs and emphasizes the importance of comprehensive evaluation and accurate histopathological analysis.展开更多
BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome a...BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.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The authors report a case of deficient sensory neuropathy secondary to vitamin B12 deficiency, diagnosed in the neurology department of the Sino-Central African Friendship University Hospital in Bangui. The diagnosis was made possible by electroneuromyography which showed subclinical neurological damage associated with hematological damage (anemia). Through this observation, we recall the diagnostic criteria of the disease in a context of difficult medical practice. .
文摘Background: The Coronavirus disease 2019 (COVID-19) is a respiratory infectious disease, also named Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), which can cause various systemic manifestations that pose a threat to human life. Oral lesions in patients with COVID-19 may appear during or after the illness and may or may not be a consequence of the viral infection. Objective: In this case series we compare the oral manifestations in hospitalized COVID-19 positive patients and COVID-19 negative dental outpatients. Methods: 60 hospitalized COVID-19 patients and 41 control patients, were examined for oral signs and symptoms. The controls were dental patients who visited the hospital for dental care without complaining of any problems related to the oral cavity itself. Results: We have observed a strong association between certain clinical findings and COVID-19, including alterations in taste (ageusia, dysgeusia, and hypogeusia), anosmia, hairy tongue, tongue imprints, red tongue, erythematous candidiasis, pseudomembranous candidiasis, and exfoliative cheilitis. A trend but not statistically significant association at the level of 5% was also noted for colored tongue, linea alba, and pale mucosa. On the contrary, fissured tongue and oral mucosa pigmentation were more frequent in the controls, statistically significant at the level of 5%. Conclusion: COVID-19 has been found to impact the oral cavity, resulting in various oral lesions that can be attributed to either the direct action of the virus or the patient’s immune response.
文摘Objective:To evaluate coagulation abnormalities and their relationship with bleeding manifestations among patients with dengue.Methods:This observational study was conducted on 292 adult dengue patients who were admitted to a tertiary care hospital of Western India from July 2021 to June 2022.Coagulation tests including prothrombin time(PT),international normalized ratio(INR),activated partial thromboplastin time(aPTT),fibrinogen,and D-dimer were performed.Patients were monitored for bleeding manifestations.Results:Coagulation abnormalities were reported in 42.8%of the patients.Overall,prolonged aPTT was the most common coagulation abnormality(40.8%),followed by low fibrinogen(38.7%),raised D-dimer(31.2%),raised INR(26.0%)and prolonged PT(19.2%).Bleeding manifestations were present in 19.9%patients.PT,INR,aPTT and D-dimer levels were significantly higher(P<0.01)and fibrinogen level was significantly lower(P<0.001)in patients with bleeding compared to patients without bleeding.Patients with bleeding had a significantly higher rate of all coagulation abnormalities than patients without bleeding(P<0.01).Conclusions:Patients with bleeding showed a significantly higher frequency of coagulation abnormalities compared to patients without bleeding.Patients with dengue should be assessed for coagulation abnormalities.
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The coronavirus disease 2019(COVID-19)initially presented as a disease that affected the lungs.Then,studies revealed that it intricately affected disparate organs in the human body,with the liver being one of the most affected organs.This review aimed to assess the association between COVID-19 and liver function,shedding light on its clinical implication.However,its exact pathophysiology remains unclear,involving many factors,such as active viral replication in the liver cells,direct cytotoxic effects of the virus on the liver or adverse reactions to viral antigens.Liver symptoms are mild-to-moderate transaminase elevation.In some patients,with underlying liver disease,more serious outcomes are observed.Thus,liver function should be meticulously considered in patients with COVID-19.
文摘BACKGROUND Hepatobiliary manifestations occur in ulcerative colitis(UC)patients.The effect of laparoscopic restorative proctocolectomy(LRP)with ileal pouch anal anastomosis(IPAA)on hepatobiliary manifestations is debated.AIM To evaluate hepatobiliary changes after two-stages elective laparoscopic restorative proctocolectomy for patients with UC.METHODS Between June 2013 and June 2018,167 patients with hepatobiliary symptoms underwent two-stage elective LRP for UC in a prospective observational study.Patients with UC and having at least one hepatobiliary manifestation who underwent LRP with IPAA were included in the study.The patients were followed up for four years to assess the outcomes of hepatobiliary manifestations.RESULTS The patients'mean age was 36±8 years,and males predominated(67.1%).The most common hepatobiliary diagnostic method was liver biopsy(85.6%),followed by Magnetic resonance cholangiopancreatography(63.5%),Antineutrophil cytoplasmic antibodies(62.5%),abdominal ultrasonography(35.9%),and Endoscopic retrograde cholangiopancreatography(6%).The most common hepatobiliary symptom was Primary sclerosing cholangitis(PSC)(62.3%),followed by fatty liver(16.8%)and gallbladder stone(10.2%).66.4%of patients showed a stable course after surgery.Progressive or regressive courses occurred in 16.8%of each.Mortality was 6%,and recurrence or progression of symptoms required surgery for 15%.Most PSC patients(87.5%)had a stable course,and only 12.5%became worse.Two-thirds(64.3%)of fatty liver patients showed a regressive course,while one-third(35.7%)showed a stable course.Survival rates were 98.8%,97%,95.8%,and 94%at 12 mo,24 mo,36 mo,and at the end of the follow-up.CONCLUSION In patients with UC who had LRP,there is a positive impact on hepatobiliary disease.It caused an improvement in PSC and fatty liver disease.The most prevalent unchanged course was PSC,while the most common improvement was fatty liver disease.
文摘BACKGROUND Epstein–Barr virus(EBV)-positive mucocutaneous ulcers(MCUs)are an uncommon disorder characterized by ulcerative lesions in the skin,oral cavity or gastrointestinal tract in patients with iatrogenic or aging-induced immunosuppression.The nonspecific lesions are difficult to differentiate from small bowel adenocarcinomas.We present the case of a 69-year-old woman who was initially misdiagnosed with a small bowel adenocarcinoma but was later surgically diagnosed with and treated for EBV-MCU.Through this case,we aim to emphasize the importance of accurately distinguishing between the two conditions.CASE SUMMARY The patient presented with an incidental finding of a small bowel tumor during computed tomography(CT)examination performed for hematuria.The CT scan showed irregular thickening of the distal ileum,which was suggestive of a malignant small bowel tumor.An exploratory laparotomy revealed an 8-cm mass in the distal ileum;thus,a segment of the small intestine,including the mass,was resected.Histopathological analysis revealed an ulceroinfiltrative mass-like lesion with luminal narrowing,marked inflammatory cell infiltration,and large atypical lymphoid cells(positive for EBV-encoded small RNA).A final diagnosis of an EBV-MCU was established.The postoperative course was uneventful,and the patient was discharged on postoperative day 7.The patient remained recurrencefree until 12 mo after surgery.CONCLUSION This case highlights the diagnostic challenges for EBV-MCUs and emphasizes the importance of comprehensive evaluation and accurate histopathological analysis.
文摘BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.
