Clinical and laboratory findings of patients with anti-dipeptidyl-peptidase-like protein 6 encephalitis: two cases report / 中华神经科杂志

Objective:To identify anti-dipeptidyl-peptidase-like protein 6 (DPPX) antibody in patients with encephalitis of unknown etiology and describe the clinical features of anti-DPPX antibody-associated encephalitis in Chinese patients.Methods:For patients registered in the Peking Union Medical College Hospital Encephalitis and Paraneoplastic Syndrome Registration Project from 2016 to 2019 with negative findings in autoimmune encephalitis routine antibody profile and paraneoplastic antibody profile, but with positive tissue-based assay (TBA) results, further tests for rare antibodies, including cell-based assay (CBA) of anti-DPPX antibody, were performed. Patients positive for anti-DPPX antibody were enrolled and the clinical data were collected.Results:Two patients with anti-DPPX antibody-associated encephalitis were found from 2016 to 2019 among about 15 000 patients. Both were females, aged 46 and 75 years. One patient had diarrhea, cachexia, cognitive dysfunction, agitation, myoclonus, tremor, and seizures. The other had cognitive impairment, restlessness, memory loss, disorientation, and sleep disturbance. The second patient had medical history of systemic lupus erythematosus and secondary Sj?gren′s syndrome.Conclusions:TBA should be combined with CBA in identification of anti-DPPX antibody to confirm the diagnosis. Anti-DPPX antibody-associated encephalitis has clinical manifestations of encephalopathy with diarrhea and cachexia, and can coexist with systemic lupus erythematosus.

The 486th case: chronic diarrhea and orthostatic hypotension / 中华内科杂志

A 57-year-old man was admitted to hospital with diarrhea for 10 months and dizziness for 4 months. The patient had 1-2 liters watery stool per day, without pyogenic blood or abnormality in gastroenteroscopy examination. The level of hemoglobin and albumin was generally normal, and fasting test was positive. At the same time, he was accompanied with hyperalgesia of lower limbs and orthostatic hypotension. After the discussion of multiple disciplinary teams, the patient was diagnosed with amyloidosis by sural nerve biopsy, myocardial MRI, and the assays of urine immunoelectrophoresis and serum free light chain. Light chain amyloidosis was confirmed after excluded the diagnosis of familial amyloidosis. The patient was improved after courses of chemotherapy with melphalan and dexamethasone.

The colonoscopic characteristics of colorectal endometriosis: a single-centered retrospective study / 中华内科杂志

Objective To reinforce the awareness of colorectal endometriosis (EM) in colonoscopy examination.Methods Patients diagnosed as colorectal EM at Peking Union Medical College Hospital between February 2002 and February 2017 were enrolled in this study.The clinical characteristics and endoscopic features of EM lesions were summarized and compared between pathologically positive group and negative group.Results A total of 34 cases were included with average age of (38.3± 8.9) years old.All EM lesions located within rectum and sigmoid colon.The endoscopic lesions manifested as protrusion in 21 cases (61.8％) and protrusion-depression in 13 cases (38.2％),local stenosis in 8 cases (23.5％);erosive surface in 33 cases (97.1％) with local spontaneous hemorrhage in 4 cases (11.8％);nodal surface in 23 cases (67.6％),and lymphangiectasis base in 9 cases (26.4％).Endoscopic biopsy specimens were obtained in all cases with average 3 (2,4) pieces.Positive results were found only in 4 patients (11.8％) with 3 endometriosis and one (endometrial) adenosarcoma.Compared with negative group,spontaneous hemorrhage was more frequent in positive group (2/4 vs.2/30,P=0.013).Mean biopsy sample number was significantly larger in positive group (5 vs.3,P=0.004).Conclusions Colorectal endometriosis is mostly located within rectosigmoid region.Endoscopic features mainly include protrusion or protrusion-depression lesions with erosive and nodular surface,or local stenosis.Spontaneous hemorrhage under colonoscopy yields higher positive rate for biopsy,thus increasing biopsy sample numbers may improve pathology results.

The clinical characteristics of patients with monomorphic epitheliotropic intestinal T-cell lymphoma characterized by minor endoscopic abnormalities / 中华内科杂志

Objective To clarify the clinical features of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) with minor endoscopic abnormalities. Methods The clinical data of 6 patients with MEITL characterized by minor endoscopic abnormalities in Peking Union Medical College Hospital from 2012 to 2016 were retrospectively analyzed, including clinical manifestations, endoscopic, pathological features, medications and prognosis. Results Five out of 6 patients were male, with an average age of 61.2 years old. The median disease duration was 4.5 months. All patients initially presented with diarrhea without specific findings for serologic testing. CT enterography showed continuous intestinal lesions, including symmetric thickening of the bowel wall, abnormal hyperenhancement of mucosal surface and lymphadenopathy. Endoscopic appearances were only mildly abnormal, including mucosal swelling, atrophy of villus, mosaic sign and shallow ulcers. Histopathologic findings revealed massive small to medium sized T lymphocytes infiltration with positive expression of CD3 and CD8. Chemotherapy and palliative treatment were administrated after diagnosis. Conclusions Clinical presentations of MEITL are non-specific with minor endoscopic abnormalities. Therefore, biopsy is indispensable for patients with a relatively normal endoscopic result.

Clinical characteristics of chronic intestinal pseudo-obstruction : an analysis of 43 cases / 中华全科医师杂志

Objective To analyze the clinical characteristics of chronic intestinal pseudoobstruction (CIPO).Methods Clinical data of 43 patients with CIPO admitted in Peking Union Medical College Hospital from January 2012 to December 2016 were retrospectively reviewed.The clinical manifestations,laboratory examinations,images,treatment and prognosis of CIPO patients were analyzed.Results There were 14 males and 29 females with an mean age of (41.9 ± 16.1) years.Among 43 patients,32 were admitted in Gastroenterology Department,6 in General Medicine Department and 5 in Rheumatology Department.Twenty two cases were primary CIPO and 21 cases were secondary.The main clinical manifestations were abdominal distension (93.0％),abdominal pain (67.4％),vomiting (67.4％),diarrhea (46.5％) and constipation (41.9％).All 43 patients had decreased body weight and body mass index (BMI);23.3％ (10/43) patients were complicated with anxiety and depression.53.5％ (23/43) patients presented anemia and 55.8％ (24/43) patients presented hypoalbuminemia.All patients with CIPO underwent abdominal X-ray,which showed intestinal obstruction;but there was no evidence of mechanical obstruction.The average length of hospital stay was (28.4 ± 13.9) d.Twenty three cases underwent gastric decompression;28 cases (65.1％) received parenteral nutrition,and 21 cases (48.8％) received enteral nutrition;and 9 abdominal operations were performed in 8 patients.Conclusion When clinical symptoms of intestinal obstruction appear without mechanical cause,CIPO should be considered and secondary factors should be screened.Unnecessary surgery should be avoided in CIPO patients.

Symptomatic characteristics and factors related to severity of irritable bowel syndrome / 中华全科医师杂志

Objective To analyze symptomatic characteristics and the factors related to severity of irritable bowel syndrome (IBS).Methods Total 516 IBS patients met Rome Ⅲ criteria were enrolled in the study from June 2009 to February 2016.A questionnaire survey was conducted in face-to-face manner,including intestinal and extra-intestinal symptoms,sleeping and psychological status.Severity of IBS was determined by symptom scores.Results Among 516 patients,there were 135 cases with mild symptoms (26.2％),267 with moderate symptoms (51.7％) and 114 with severe symptoms (22.1％).Periumbilical pain/discomfort associated with defecation was a common symptom location (34.6％,178/516),however,53.3％ (275/516) patients also reported having abdominal pain/discomfort during non-defecation period.In IBS patients,51.2％ (264/516) were coexisted with gastroesophageal reflux disease (GERD),59.1％ (305/516) with functional dyspepsia (FD),and 46.9％ (242/516) with depression.The prevalence of ordinary abdominal pain/discomfort [57.0％ (217/381) vs.43.0％ (58/135),x2 =7.841],sensation of incomplete defecation[74.5％ (284/381)vs.61.5％ (83/135),x2 =8.277] and coexisted GERD[54.1％ (206/381) vs.43.0％(58/135),x2 =4.920],FD[64.0％(244/381)vs.45.2％ (61/135),x2 =14.664],headache[50.7％ (193/381)vs.38.5％ (52/135),x2 =5.889] and depression[52.2％ (199/ 381)vs.31.9％ (43/135),x2 =16.623] in patients with moderate to severe symptoms were significantly higher than that in patients with mild symptoms (all P ＜ 0.05).The predictors to moderate-severe IBS symptoms were coexisted FD (OR =1.798,95％ CI:1.162-2.783,P =0.010) and depression (OR =1.696,95％ CI:1.043-2.759,P =0.014).Conclusion The symptoms of IBS patients are various,and patients coexisted with FD and depression are more likely to have more severe symptoms.

Understanding and Management of Irritable Bowel Syndrome-like Symptoms Coexisting With Quiescent Inflammatory Bowel Disease in A Comprehensive Perspective / 胃肠病学

Symptoms compatible with irritable bowel syndrome (IBS) such as abdominal pain and diarrhea may co exist in approximately one-third of patients with quiescent inflammatory bowel disease (IBD),so called IBS-like symptoms.These symptoms present as a clinical dilemma for management of IBD and diminish the patients' quality of life remarkably.IBS-like symptoms raise much concern in the research field of IBD in recent years.In this article,the latest research progress focusing on the prevalence,pathogenesis and management of IBS-like symptoms coexisting with quiescent IBD was reviewed.

The efficacy of cyclosporine A as salvage therapy for severe active ulcerative colitis refractory to glucocorticoid / 中华内科杂志

Objective To clarify the efficacy and safety of cyclosporine A CsA) as salvage therapy in patients with severe active ulcerative colitis (UC) and refractory to steroids.Methods A total of 24 severe active UC patients refractory to steroids and hospitalized from 2006 to 2012,were retrospectively enrolled.Data including demographic features,clinical manifestations,laboratory tests and medications were collected.Results CsA was effective in 15 (62.5％) patients,who did not receive colectomy during 12-week administration.This regimen was tolerable in most patients.Twelve (50.0％) patients reported 16 adverse events,but only one patient withdrew CsA due to intolerance.The rates of adverse events in initial intravenous CsA including 4 mg · kg-1 · d-1,3 mg · kg-1 · d-1 and 2 mg· kg-1 · d-1 were 2/2,9/17 and 1/5 respectively.Responders had higher white blood cell count compared with non-responders (P =0.045).Conclusions CsA could be an effective alternative regimen to colectomy in severe active UC patients who are refractory to steroids.

Characteristics and clinical significance of sera anti-enteric neuronal antibodies in patients with irritable bowel syndrome with diarrhea / 基础医学与临床

Objective To detect the sera anti-enteric neuronal antibodies ( AENA ) in irritable bowel syndrome with diarrhea ( IBS-D) patients and analyzed its correlation with IBS-D symptoms to explore the potential roles of AENA in the pathogenesis of IBS.Methods IBS-D patients diagnosed with RomeⅢdiagnostic criteria were en-rolled in this study.The sera of healthy subjects were used as controls.Indirect immunofluorescence ( IIF) was used to detect the sera AENA with the substrate of ileal submucosal plexus of guinea pig .The immune reactivity ( IR) stains were read in blinded method .The bowel symptoms of patients with positive AENA were compared to thatwithnegativeandweeklypositiveantibodies.Results 1)Atotalof127IBS-Dpatientswereenrolledinthis study.The positive rate of sera AENA was 85.8%in IBS-D patients, and 7.0%in healthy controls.Among 109 IBS-D patients with positive IIF reactivity , 23.6%present with strong positive , 43.3% with positive and 18.9%with weakly positive stain .The IR patterns included cytoplasm staining , nucleus staining , cytoplasms and nuclei staining , nuclear membrane staining , cytoplasm and nuclear membrane staining .Six positive sera of healthy control showed cytoplasm staining to substrate neurons .2 ) More patients of IBS-D with positive IR had higher intestinal symptoms scores (>10 scores, 58.8%vs 38.1%), frequent abdominal pain in non-defecation period (91.7%vs 60.0%) , and severe abdominal pain/discomfort before defecation ( 24.7% vs 9.5%) comparing to those with negative and weekly positive IR of AENA;IBS-D patients with positive IR of AENA are more commonly associated with urgency comparing to those with negative IR in IIF (57.1%vs 87.3 ) .Conclusions AENA may play a role in the pathogenesis of IBS , and is a potential biomarker of IBS-D.

Changes in Enteric Neurons of Small Intestine in a Rat Model of Irritable Bowel Syndrome with Diarrhea

BACKGROUND/AIMS: Physical and/or emotional stresses are important factors in the exacerbation of symptoms in irritable bowel syndrome (IBS). Several lines of evidence support that a major impact of stress on the gastrointestinal tract occurs via the enteric nervous system. We aimed to evaluate histological changes in the submucosal plexus (SMP) and myenteric plexus (MP) of the distal ileum in concert with the intestinal motor function in a rat model of IBS with diarrhea. METHODS: The rat model was induced by heterotypic chronic and acute stress (CAS). The intestinal transit was measured by administering powdered carbon by gastric gavage. Double immunohistochemical fluorescence staining with whole-mount preparations of SMP and MP of enteric nervous system was used to assess changes in expression of choline acetyltransferase, vasoactive intestinal peptide, or nitric oxide synthase in relation to the pan neuronal marker, anti-Hu. RESULTS: The intestinal transit ratio increased significantly from control values of 50.8% to 60.6% in the CAS group. The numbers of enteric ganglia and neurons in the SMP were increased in the CAS group. The proportions of choline acetyltransferase- and vasoactive intestinal peptide-immunoreactive neurons in the SMP were increased (82.1 ± 4.3% vs. 76.0 ± 5.0%, P = 0.021; 40.5 ± 5.9% vs 28.9 ± 3.7%, P = 0.001), while nitric oxide synthase-immunoreactive neurons in the MP were decreased compared with controls (23.3 ± 4.5% vs 32.4 ± 4.5%, P = 0.002). CONCLUSIONS: These morphological changes in enteric neurons to CAS might contribute to the dysfunction in motility and secretion in IBS with diarrhea.

The clinical features of 16 cases of primary adenocarcinoma of the third portion of duodenum / 中华内科杂志

Objective To summarize the clinical features of the third portion of duodenum (PATD) for improving the understanding of PATD.Methods Sixteen cases with PATD in Peking Union Medical College Hospital(PUMCH) were retrospectively analyzed.Results The most common symptoms of PATD were upper abdominal pain(12/16),vomiting (9/16) and distention (7/16).On average,the disease had progressed 5.5 months (including 2.5 months of diagnostic workup) before the diagnosis was established.Patients with pathologically poorly differentiated PATD had shorter course of disease (6.5 vs 16.6 months,P =0.56) and lower chance of cancer-directed surgery (1/8 vs 6/8,P =0.04) than those with well differentiated PATD.The diagnostic rate was 11/14 by CT scan while only 2/7 by upper gastrointestinal radiography.Three cases were misdiagnosed as superior mesenteric artery syndrome by barium examination.Conclusions PATD should be considered in patients presenting upper abdominal symptoms with negative gastmendoscopy and barium examination.Overall,CT scan plays a pivotal role in diagnosing PATD.Making a correct diagnosis timely can improve the outcome of PATD patients,particularly,in those with poorly differentiated pathology.

A clinical analysis of 61 cases of protein-losing enteropathy / 中华内科杂志

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.

The morphological changes of colonic submucusal plexus in rats of irritable bowel syndrome with diarrhea / 中华消化杂志

Objective To investigate the morphological responses of enteric nervous system (ENS) neurons of submucosal plexus (SMP) in distal colon of rat models with irritable bowel syndrome with diarrhea (IBS-D). Methods Rat models with IBS-D were established with chronic unpredictable mild stress and acute wrap restrain stress (CAS). Double staining with immunohistofluorescence for colonic whole mount preparations was used to evaluate the distribution and proportion of SMP neurons expressing specific neurochemical markers [choline acetyltransferase (CHAT), vasoactive intestinal polypeptide (VIP), and nitric oxide synthase (NOS)] and their colocalization with anti-Hu antibody, which is the hallmark for whole enteric neurons. Results There was no obvious difference in the total numbers of anti-Hu positive neurons and ganglia between IBS-D and control group. The proportions of both VIP-immunoreactive (VIP-IR) and NOS-IR neurons in colonic SMP were higher in IBS-D rats compared with controls (62.2%±6.2% vs 55.4% ±5.4%;15.0%±4.0%vs 10.5%±2.9%, P<0.05). There was no difference in the proportion of ChAT-IR neurons between two groups. Conclusions VIP is the most specific neurotransmitter for excitatory secretomotor neurons in rat SMP, the increase of VIP-IR neurons can promote intestinal mucosal secretion, which might play an important role in the pathophysiological mechanisms of IBS with diarrhea.