Background:A high prevalence of diabetes mellitus(DM)coexisting with autoimmune pancreatitis(AIP)is observed.However,evidence on the circumstances under which corticosteroid therapy(CST)for AIP improves or worsens DM ...Background:A high prevalence of diabetes mellitus(DM)coexisting with autoimmune pancreatitis(AIP)is observed.However,evidence on the circumstances under which corticosteroid therapy(CST)for AIP improves or worsens DM is scarce.This study aimed to demonstrate and identify predictors of DM control under the influence of CST.Methods:Patients diagnosed with type 1 AIP were enrolled from a prospectively maintained cohort and were classified into three groups according to the chronology in which AIP and DM were diagnosed:pre-existing DM(pDM),concurrent DM(cDM),and non-DM(nDM).The responses of DM to CST were assessed when corticosteroid was ceased or tapered to a maintenance dose and classified as‘improvement’and‘non-improvement’(including‘no change’and‘exacerbation’).Results:Among 101 patients with type 1 AIP,52(51.5%)patients were complicated with DM at the time of AIP diagnosis,with 36 patients in the cDM group and 16 patients in the pDM group.The incidences of diffuse pancreatic swelling(72.2%)and pancreatic body/tail involvement(91.7%)were significantly higher in the cDM group than in both the pDM and nDM groups.Of the 52 patients with DM,CST was administered in 48 cases.Multivariate logistic analysis identified that elevated serum gamma-glutamyl transferase(GGT)level at AIP diagnosis[odds ratio(OR)=0.032,95%confidence interval(CI):0.003-0.412,P=0.008]and pancreatic atrophy after CST(OR=0.027,95%CI:0.003-0.295,P=0.003)were negatively associated with DM control improvement.Conclusions:Patients with diffuse pancreatic swelling and pancreatic body/tail involvement in pancreatitis tended to be complicated with cDM at AIP diagnosis.CST exerted a beneficial effect on the clinical course of DM in nearly half of the AIP patients complicated with DM at diagnosis,particularly in those without elevated serum GGT levels at diagnosis and who did not experience pancreatic atrophy after CST.展开更多
Chronic enteropathy associated with the SLCO2A1 gene(CEAS)is a complex gastroenterological condition characterized by multiple ulcers in the small intestine with chronic bleeding and protein loss.This review explores ...Chronic enteropathy associated with the SLCO2A1 gene(CEAS)is a complex gastroenterological condition characterized by multiple ulcers in the small intestine with chronic bleeding and protein loss.This review explores the potential mechanisms underlying the pathogenesis of CEAS,focusing on the role of SLCO2A1-encoded prostaglandin transporter OATP2A1 and its impact on prostaglandin E2(PGE2)levels.Studies have suggested that elevated PGE2 levels contribute to mucosal damage,inflammation,and disruption of the intestinal barrier.The effects of PGE2 on macrophage activation and Maxi-Cl channel functionality,as well as its interaction with nonsteroidal anti-inflammatory drugs play crucial roles in the progression of CEAS.Understanding the balance between its protective and pro-inflammatory effects and the complex interactions within the gastrointestinal tract can shed light on potential therapeutic targets for CEAS and guide the development of novel,targeted therapies.展开更多
BACKGROUND The management of polyps involving the appendiceal orifice(AO)presents notable challenges.Endoscopic resection is frequently hindered by operational complexities,a heightened risk of incomplete removal,and ...BACKGROUND The management of polyps involving the appendiceal orifice(AO)presents notable challenges.Endoscopic resection is frequently hindered by operational complexities,a heightened risk of incomplete removal,and an elevated risk of procedural complications,including appendicitis.Conversely,surgical resection may entail unnecessary excision of intestinal segments,leading to potential morbidity.CASE SUMMARY Here,we reported two patients who presented with polyps deeply situated within the AO,with indistinct boundaries making it challenging to ensure completeness using traditional endoscopic resection.To overcome these challenges,we em-ployed combined endo-laparoscopic surgery(CELS),achieving curative resection without postoperative complications.CONCLUSION The application of CELS in managing polyps involving the AO is emerging as a safe and effective treatment modality.展开更多
BACKGROUND Acupuncture promotes the recovery of gastrointestinal function and provides analgesia after major abdominal surgery.The effects of transcutaneous electrical acupoint stimulation(TEAS)remain unclear.AIM To e...BACKGROUND Acupuncture promotes the recovery of gastrointestinal function and provides analgesia after major abdominal surgery.The effects of transcutaneous electrical acupoint stimulation(TEAS)remain unclear.AIM To explore the potential effects of TEAS on the recovery of gastrointestinal function after gastrectomy and colorectal resection.METHODS Patients scheduled for gastrectomy or colorectal resection were randomized at a 2:3:3:2 ratio to receive:(1)TEAS at maximum tolerable current for 30 min immediately prior to anesthesia induction and for the entire duration of surgery,plus two 30-min daily sessions for 3 consecutive days after surgery(perioperative TEAS group);(2)Preoperative and intraoperative TEAS only;(3)Preoperative and postoperative TEAS only;or(4)Sham stimulation.The primary outcome was the time from the end of surgery to the first bowel sound.RESULTS In total,441 patients were randomized;405 patients(58.4±10.2 years of age;247 males)received the planned surgery.The time to the first bowel sounds did not differ among the four groups(P=0.90;log-rank test).On postoperative day 1,the rest pain scores differed significantly among the four groups(P=0.04;Kruskal–Wallis test).Post hoc comparison using the Bonferroni test showed lower pain scores in the perioperative TEAS group(1.4±1.2)than in the sham sti-mulation group(1.7±1.1;P=0.04).Surgical complications did not differ among the four groups.CONCLUSION TEAS provided analgesic effects in adult patients undergoing major abdominal surgery,and it can be added to clinical practice as a means of accelerating postoperative rehabilitation of these patients.展开更多
AIM:To evaluate the diagnostic effectiveness of white light endoscopy,magnifying endoscopy(ME),and magnifying narrow-band imaging endoscopy(ME-NBI) in detecting early gastric cancer(EGC).METHODS:From March 2010 to Jun...AIM:To evaluate the diagnostic effectiveness of white light endoscopy,magnifying endoscopy(ME),and magnifying narrow-band imaging endoscopy(ME-NBI) in detecting early gastric cancer(EGC).METHODS:From March 2010 to June 2012,a total of 3616 patients received screening for gastric cancer by magnifying endoscopy. There were 3675 focal gastric lesions detected using conventional high definition white light endoscopy(HD-WLE) in four different referentialhospitals that were recruited for further investigation using ME and ME-NBI. The images obtained from HD-WLE,ME,and ME-NBI were reviewed by four experienced endoscopists to evaluate their diagnostic effectiveness for EGC. The diagnosis of cancerous and non-cancerous lesions was conducted by evaluating the microvascular and microsurface patterns using the VS classification system. The final endoscopic diagnosis of each lesion was determined by consultation when a disagreement occurred. We used histopathological results as the gold standard for the diagnosis of EGC.RESULTS:Among the 3675 lesions found,1508 were validated by pathological findings as chronic gastritis,1279 as chronic gastritis with intestinal metaplasia,631 as low-grade neoplasia,and 257 as EGC. The sensitivity,specificity,positive predictive value,negative predictive value,and accuracy of HD-WLE for the diagnosis of EGC were 71.2%,99.1%,85.5%,97.9% and 97.1%,respectively. The results of ME for diagnosing EGC were 81.3%,98.8%,83.3%,98.6% and 97.6%,respectively. The results of ME-NBI for the diagnosis of EGC were 87.2%,98.6%,82.1%,99.0% and 97.8%,respectively. The diagnostic sensitivity and accuracy of paired ME and ME-NBI were significantly better than those of HD-WLE(P < 0.05).CONCLUSION:HD-WLE has a relatively high accuracy for diagnosing EGC and is an effective screening tool. Further investigations of ME and ME-NBI are required to achieve superior accuracy.展开更多
With the digestive endoscopic tunnel technique(DETT), many diseases that previously would have been treated by surgery are now endoscopically curable by establishing a submucosal tunnel between the mucosa and muscular...With the digestive endoscopic tunnel technique(DETT), many diseases that previously would have been treated by surgery are now endoscopically curable by establishing a submucosal tunnel between the mucosa and muscularis propria(MP). Through the tunnel, endoscopic diagnosis or treatment is performed for lesions in the mucosa, in the MP, and even outside the gastrointestinal(GI) tract.At present, the tunnel technique application range covers the following:(1)Treatment of lesions originating from the mucosal layer, e.g., endoscopic submucosal tunnel dissection for oesophageal large or circular early-stage cancer or precancerosis;(2) treatment of lesions from the MP layer, per-oral endoscopic myotomy, submucosal tunnelling endoscopic resection, etc.; and(3) diagnosis and treatment of lesions outside the GI tract, such as resection of lymph nodes and benign tumour excision in the mediastinum or abdominal cavity. With the increasing number of DETTs performed worldwide, endoscopic tunnel therapeutics, which is based on DETT, has been gradually developed and optimized. However, there is not yet an expert consensus on DETT to regulate its indications, contraindications, surgical procedure, and postoperative treatment.The International DETT Alliance signed up this consensus to standardize the procedures of DETT. In this consensus, we describe the definition, mechanism,and significance of DETT, prevention of infection and concepts of DETTassociated complications, methods to establish a submucosal tunnel, and application of DETT for lesions in the mucosa, in the MP and outside the GI tract(indications and contraindications, procedures, pre-and postoperative treatments, effectiveness, complications and treatments, and a comparison between DETT and other operations).展开更多
Human cytomegalovirus(CMV) is a herpesvirus,which establishes lifelong latency after primary infection and leads to severe disease in immunocompromised patients. However,CMV infection in immunocompetent patients is us...Human cytomegalovirus(CMV) is a herpesvirus,which establishes lifelong latency after primary infection and leads to severe disease in immunocompromised patients. However,CMV infection in immunocompetent patients is usually asymptomatic and severe organ damage is rarely reported. We report a case of severe CMV hepatitis in an immunocompetent patient presenting with cholestasis,portal hypertensionrelated ascites and pancytopenia. The patient was asymptomatic with normal liver function and negative CMV DNA after two weeks of antiviral therapy. This case is an example of a common infection with an uncommon presentation,and suggests that testing for CMV should be carried out,even in patients with normal immune status,presenting with severe liver damage or cholestasis.展开更多
BACKGROUND Few studies have fully described endoscopic ultrasound(EUS)features of newly diagnosed autoimmune pancreatitis(AIP)involving both typical findings and chronic pancreatitis(CP)features.The typical EUS findin...BACKGROUND Few studies have fully described endoscopic ultrasound(EUS)features of newly diagnosed autoimmune pancreatitis(AIP)involving both typical findings and chronic pancreatitis(CP)features.The typical EUS findings are prevalent in the diffuse type AIP but may not be as common for the focal type,and the differences between the diffuse and focal AIP need to be specified.AIM To demonstrate the EUS features of newly diagnosed AIP and the difference between diffuse and focal AIP.METHODS This retrospective single center study included 285 patients of newly diagnosed type 1 AIP following the international consensus diagnostic criteria,with the EUS procedures accomplished before corticosteroid initiation.We explored the EUS features and compared the typical AIP and CP features between the diffuse and focal AIP cases.The Rosemont criteria were employed for CP features definition and CP change level comparison.RESULTS For the typical AIP features,there were significantly more patients in the diffuse group with bile duct wall thickening(158 of 214 cases,73.4%vs 37 of 71 cases,52.1%,P=0.001)and peripancreatic hypoechoic margin(76 of 214 cases,35.5%vs 5 of 71 cases,7.0%,P<0.001).For the CP features,there were significantly more patients in the focal group with main pancreatic duct dilation(30 of 214 cases,14.0%vs 18 of 71 cases,25.3%,P=0.03).The cholangitis-like changes were more prevalent in the focal cases with pancreatic head involvement.The CP change level was relatively limited for newly diagnosed AIP cases in both groups.CONCLUSION This study demonstrated the difference in the typical AIP and CP features between diffuse and focal AIP and indicated the limited CP change level in newly diagnosed AIP.展开更多
BACKGROUND Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency,alacrima and achalasia.In the last 5 years,per-oral endoscopic myotomy(PO...BACKGROUND Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency,alacrima and achalasia.In the last 5 years,per-oral endoscopic myotomy(POEM)has proved highly successful in treating primary achalasia over the long term,but its long-term performance has not been certified by achalasia related to Triple A syndrome.CASE SUMMARY Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency,alacrima and achalasia.In the past 5 years,POEM has proved highly successful in treating primary achalasia over the long term,but its long-term performance has not been certified by achalasia related to Triple A syndrome.Eckardt scores and esophageal manometry improved significantly during the 2 years following POEM;however,grade-A reflux esophagitis recurred in 66.7%of patients in 12 mo post-procedure.CONCLUSION Based on these case studies,POEM is efficacious and safe for a treatment of achalasia associated with Triple A syndrome.展开更多
Caroli disease(CD)is a rare congenital disorder characterized by segmental dilatation of the intrahepatic bile ducts.[1]The simple CD is rare,with the majority of patients complicated by congenital liver fibrosis.Curr...Caroli disease(CD)is a rare congenital disorder characterized by segmental dilatation of the intrahepatic bile ducts.[1]The simple CD is rare,with the majority of patients complicated by congenital liver fibrosis.Current treatments for CD mostly target complications but do not prevent disease progression.Surgical resection and liver transplantation are effective treatment options,but both have limitations.[2]Therefore,fully elucidating the patho-genesis of CD to identify therapeutic targets to delay disease progression is a priority.展开更多
基金from CAMS Innovation Fund for Medical Sciences(CIFMS)(2021-I2M-1-002)National Key Clinical Specialty Construction Project(ZK108000)+1 种基金National High-Level Hospital Clinical Research Funding(2022-PUMCH-B-024)National Natural Science Foundation of China,Joint Fund Project(U20A600).
文摘Background:A high prevalence of diabetes mellitus(DM)coexisting with autoimmune pancreatitis(AIP)is observed.However,evidence on the circumstances under which corticosteroid therapy(CST)for AIP improves or worsens DM is scarce.This study aimed to demonstrate and identify predictors of DM control under the influence of CST.Methods:Patients diagnosed with type 1 AIP were enrolled from a prospectively maintained cohort and were classified into three groups according to the chronology in which AIP and DM were diagnosed:pre-existing DM(pDM),concurrent DM(cDM),and non-DM(nDM).The responses of DM to CST were assessed when corticosteroid was ceased or tapered to a maintenance dose and classified as‘improvement’and‘non-improvement’(including‘no change’and‘exacerbation’).Results:Among 101 patients with type 1 AIP,52(51.5%)patients were complicated with DM at the time of AIP diagnosis,with 36 patients in the cDM group and 16 patients in the pDM group.The incidences of diffuse pancreatic swelling(72.2%)and pancreatic body/tail involvement(91.7%)were significantly higher in the cDM group than in both the pDM and nDM groups.Of the 52 patients with DM,CST was administered in 48 cases.Multivariate logistic analysis identified that elevated serum gamma-glutamyl transferase(GGT)level at AIP diagnosis[odds ratio(OR)=0.032,95%confidence interval(CI):0.003-0.412,P=0.008]and pancreatic atrophy after CST(OR=0.027,95%CI:0.003-0.295,P=0.003)were negatively associated with DM control improvement.Conclusions:Patients with diffuse pancreatic swelling and pancreatic body/tail involvement in pancreatitis tended to be complicated with cDM at AIP diagnosis.CST exerted a beneficial effect on the clinical course of DM in nearly half of the AIP patients complicated with DM at diagnosis,particularly in those without elevated serum GGT levels at diagnosis and who did not experience pancreatic atrophy after CST.
基金Supported by the National High-Level Hospital Clinical Research Fund,No.2022-PUMCH-A-020the Undergraduate Teaching Reform and Innovation Project,No.2022zlgc0108.
文摘Chronic enteropathy associated with the SLCO2A1 gene(CEAS)is a complex gastroenterological condition characterized by multiple ulcers in the small intestine with chronic bleeding and protein loss.This review explores the potential mechanisms underlying the pathogenesis of CEAS,focusing on the role of SLCO2A1-encoded prostaglandin transporter OATP2A1 and its impact on prostaglandin E2(PGE2)levels.Studies have suggested that elevated PGE2 levels contribute to mucosal damage,inflammation,and disruption of the intestinal barrier.The effects of PGE2 on macrophage activation and Maxi-Cl channel functionality,as well as its interaction with nonsteroidal anti-inflammatory drugs play crucial roles in the progression of CEAS.Understanding the balance between its protective and pro-inflammatory effects and the complex interactions within the gastrointestinal tract can shed light on potential therapeutic targets for CEAS and guide the development of novel,targeted therapies.
基金Supported by the National High-Level Hospital Clinical Research Funding,No.2022-PUMCH-B-024 and No.2022-PUMCH-A-020Undergraduate Teaching Reform and Innovation Project,No.2022zlgc0108.
文摘BACKGROUND The management of polyps involving the appendiceal orifice(AO)presents notable challenges.Endoscopic resection is frequently hindered by operational complexities,a heightened risk of incomplete removal,and an elevated risk of procedural complications,including appendicitis.Conversely,surgical resection may entail unnecessary excision of intestinal segments,leading to potential morbidity.CASE SUMMARY Here,we reported two patients who presented with polyps deeply situated within the AO,with indistinct boundaries making it challenging to ensure completeness using traditional endoscopic resection.To overcome these challenges,we em-ployed combined endo-laparoscopic surgery(CELS),achieving curative resection without postoperative complications.CONCLUSION The application of CELS in managing polyps involving the AO is emerging as a safe and effective treatment modality.
基金National Basic Research Program of China(Project 973)from The Ministry of Science and Technology of the People's Republic of China,No.2013CB531900。
文摘BACKGROUND Acupuncture promotes the recovery of gastrointestinal function and provides analgesia after major abdominal surgery.The effects of transcutaneous electrical acupoint stimulation(TEAS)remain unclear.AIM To explore the potential effects of TEAS on the recovery of gastrointestinal function after gastrectomy and colorectal resection.METHODS Patients scheduled for gastrectomy or colorectal resection were randomized at a 2:3:3:2 ratio to receive:(1)TEAS at maximum tolerable current for 30 min immediately prior to anesthesia induction and for the entire duration of surgery,plus two 30-min daily sessions for 3 consecutive days after surgery(perioperative TEAS group);(2)Preoperative and intraoperative TEAS only;(3)Preoperative and postoperative TEAS only;or(4)Sham stimulation.The primary outcome was the time from the end of surgery to the first bowel sound.RESULTS In total,441 patients were randomized;405 patients(58.4±10.2 years of age;247 males)received the planned surgery.The time to the first bowel sounds did not differ among the four groups(P=0.90;log-rank test).On postoperative day 1,the rest pain scores differed significantly among the four groups(P=0.04;Kruskal–Wallis test).Post hoc comparison using the Bonferroni test showed lower pain scores in the perioperative TEAS group(1.4±1.2)than in the sham sti-mulation group(1.7±1.1;P=0.04).Surgical complications did not differ among the four groups.CONCLUSION TEAS provided analgesic effects in adult patients undergoing major abdominal surgery,and it can be added to clinical practice as a means of accelerating postoperative rehabilitation of these patients.
基金Supported by Profession Specific Funded Projects in Standar-dization of Targeted Therapy and Cell Therapy and Applied Research of Early Diagnosis and Treatment for Cancer from Chinese Ministry of Health,No.200902002
文摘AIM:To evaluate the diagnostic effectiveness of white light endoscopy,magnifying endoscopy(ME),and magnifying narrow-band imaging endoscopy(ME-NBI) in detecting early gastric cancer(EGC).METHODS:From March 2010 to June 2012,a total of 3616 patients received screening for gastric cancer by magnifying endoscopy. There were 3675 focal gastric lesions detected using conventional high definition white light endoscopy(HD-WLE) in four different referentialhospitals that were recruited for further investigation using ME and ME-NBI. The images obtained from HD-WLE,ME,and ME-NBI were reviewed by four experienced endoscopists to evaluate their diagnostic effectiveness for EGC. The diagnosis of cancerous and non-cancerous lesions was conducted by evaluating the microvascular and microsurface patterns using the VS classification system. The final endoscopic diagnosis of each lesion was determined by consultation when a disagreement occurred. We used histopathological results as the gold standard for the diagnosis of EGC.RESULTS:Among the 3675 lesions found,1508 were validated by pathological findings as chronic gastritis,1279 as chronic gastritis with intestinal metaplasia,631 as low-grade neoplasia,and 257 as EGC. The sensitivity,specificity,positive predictive value,negative predictive value,and accuracy of HD-WLE for the diagnosis of EGC were 71.2%,99.1%,85.5%,97.9% and 97.1%,respectively. The results of ME for diagnosing EGC were 81.3%,98.8%,83.3%,98.6% and 97.6%,respectively. The results of ME-NBI for the diagnosis of EGC were 87.2%,98.6%,82.1%,99.0% and 97.8%,respectively. The diagnostic sensitivity and accuracy of paired ME and ME-NBI were significantly better than those of HD-WLE(P < 0.05).CONCLUSION:HD-WLE has a relatively high accuracy for diagnosing EGC and is an effective screening tool. Further investigations of ME and ME-NBI are required to achieve superior accuracy.
基金Supported by National Key R and D Program of China,No.2016YFC1303601
文摘With the digestive endoscopic tunnel technique(DETT), many diseases that previously would have been treated by surgery are now endoscopically curable by establishing a submucosal tunnel between the mucosa and muscularis propria(MP). Through the tunnel, endoscopic diagnosis or treatment is performed for lesions in the mucosa, in the MP, and even outside the gastrointestinal(GI) tract.At present, the tunnel technique application range covers the following:(1)Treatment of lesions originating from the mucosal layer, e.g., endoscopic submucosal tunnel dissection for oesophageal large or circular early-stage cancer or precancerosis;(2) treatment of lesions from the MP layer, per-oral endoscopic myotomy, submucosal tunnelling endoscopic resection, etc.; and(3) diagnosis and treatment of lesions outside the GI tract, such as resection of lymph nodes and benign tumour excision in the mediastinum or abdominal cavity. With the increasing number of DETTs performed worldwide, endoscopic tunnel therapeutics, which is based on DETT, has been gradually developed and optimized. However, there is not yet an expert consensus on DETT to regulate its indications, contraindications, surgical procedure, and postoperative treatment.The International DETT Alliance signed up this consensus to standardize the procedures of DETT. In this consensus, we describe the definition, mechanism,and significance of DETT, prevention of infection and concepts of DETTassociated complications, methods to establish a submucosal tunnel, and application of DETT for lesions in the mucosa, in the MP and outside the GI tract(indications and contraindications, procedures, pre-and postoperative treatments, effectiveness, complications and treatments, and a comparison between DETT and other operations).
文摘Human cytomegalovirus(CMV) is a herpesvirus,which establishes lifelong latency after primary infection and leads to severe disease in immunocompromised patients. However,CMV infection in immunocompetent patients is usually asymptomatic and severe organ damage is rarely reported. We report a case of severe CMV hepatitis in an immunocompetent patient presenting with cholestasis,portal hypertensionrelated ascites and pancytopenia. The patient was asymptomatic with normal liver function and negative CMV DNA after two weeks of antiviral therapy. This case is an example of a common infection with an uncommon presentation,and suggests that testing for CMV should be carried out,even in patients with normal immune status,presenting with severe liver damage or cholestasis.
基金by Beijing Science and Technology Program,No.Z181100001618013Peking Union Medical College Education Reform Program,No.2019zlgc0116.
文摘BACKGROUND Few studies have fully described endoscopic ultrasound(EUS)features of newly diagnosed autoimmune pancreatitis(AIP)involving both typical findings and chronic pancreatitis(CP)features.The typical EUS findings are prevalent in the diffuse type AIP but may not be as common for the focal type,and the differences between the diffuse and focal AIP need to be specified.AIM To demonstrate the EUS features of newly diagnosed AIP and the difference between diffuse and focal AIP.METHODS This retrospective single center study included 285 patients of newly diagnosed type 1 AIP following the international consensus diagnostic criteria,with the EUS procedures accomplished before corticosteroid initiation.We explored the EUS features and compared the typical AIP and CP features between the diffuse and focal AIP cases.The Rosemont criteria were employed for CP features definition and CP change level comparison.RESULTS For the typical AIP features,there were significantly more patients in the diffuse group with bile duct wall thickening(158 of 214 cases,73.4%vs 37 of 71 cases,52.1%,P=0.001)and peripancreatic hypoechoic margin(76 of 214 cases,35.5%vs 5 of 71 cases,7.0%,P<0.001).For the CP features,there were significantly more patients in the focal group with main pancreatic duct dilation(30 of 214 cases,14.0%vs 18 of 71 cases,25.3%,P=0.03).The cholangitis-like changes were more prevalent in the focal cases with pancreatic head involvement.The CP change level was relatively limited for newly diagnosed AIP cases in both groups.CONCLUSION This study demonstrated the difference in the typical AIP and CP features between diffuse and focal AIP and indicated the limited CP change level in newly diagnosed AIP.
文摘BACKGROUND Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency,alacrima and achalasia.In the last 5 years,per-oral endoscopic myotomy(POEM)has proved highly successful in treating primary achalasia over the long term,but its long-term performance has not been certified by achalasia related to Triple A syndrome.CASE SUMMARY Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency,alacrima and achalasia.In the past 5 years,POEM has proved highly successful in treating primary achalasia over the long term,but its long-term performance has not been certified by achalasia related to Triple A syndrome.Eckardt scores and esophageal manometry improved significantly during the 2 years following POEM;however,grade-A reflux esophagitis recurred in 66.7%of patients in 12 mo post-procedure.CONCLUSION Based on these case studies,POEM is efficacious and safe for a treatment of achalasia associated with Triple A syndrome.
文摘Caroli disease(CD)is a rare congenital disorder characterized by segmental dilatation of the intrahepatic bile ducts.[1]The simple CD is rare,with the majority of patients complicated by congenital liver fibrosis.Current treatments for CD mostly target complications but do not prevent disease progression.Surgical resection and liver transplantation are effective treatment options,but both have limitations.[2]Therefore,fully elucidating the patho-genesis of CD to identify therapeutic targets to delay disease progression is a priority.
