BACKGROUND Behcet's disease(BD)is an inflammatory disorder known for various symptoms,including oral and genital ulcers and ocular inflammation.Panuveitis,a severe eye condition,is rare as the first sign of BD.CAS...BACKGROUND Behcet's disease(BD)is an inflammatory disorder known for various symptoms,including oral and genital ulcers and ocular inflammation.Panuveitis,a severe eye condition,is rare as the first sign of BD.CASE SUMMARY We present an unusual case of a 30-year-old man who developed panuveitis after receiving the mRNA-based coronavirus disease 2019(COVID-19)vaccine(Moderna).Laboratory tests ruled out infections,but he had a positive HLA-B51 result and a history of genital ulcer and oral ulcers,leading to a BD diagnosis.Treatment with corticosteroids improved his condition.Interestingly,he had another episode of panuveitis after the second mRNA vaccine dose,which also responded to corticosteroids.CONCLUSION This case highlights the rare onset of BD following mRNA COVID-19 vaccination,suggesting a potential link between these vaccines and BD's eye symptoms,emphasizing the importance of quick treatment in similar cases.展开更多
AIM:To develop a novel endoscopic severity model of intestinal Behcet's disease(BD) and to evaluate its feasibility by comparing it with the actual disease activity index for intestinal Behcet's disease(DAIBD)...AIM:To develop a novel endoscopic severity model of intestinal Behcet's disease(BD) and to evaluate its feasibility by comparing it with the actual disease activity index for intestinal Behcet's disease(DAIBD).METHODS:We reviewed the medical records of 167 intestinal BD patients between March 1986 and April 2011.We also investigated the endoscopic parameters including ulcer locations,distribution,number,depth,shape,size and margin to identify independent factors associated with DAIBD.An endoscopic severity model was developed using significant colonoscopic variables identified by multivariate regression analysis and its correlation with the DAIBD was evaluated.To determine factors related to the discrepancy between endoscopic severity and clinical activity,clinical characteristics and laboratory markers of the patients were analyzed.RESULTS:A multivariate regression analysis revealed that the number of intestinal ulcers(≥ 2,P = 0.031) and volcanoshaped ulcers(P = 0.001) were predictive factors for the DAIBD.An endoscopic severity model(Y) was developed based on selected endoscopic variables as follows:Y = 47.44 + 9.04 × non-Ileocecal area + 11.85 ×≥ 2 of intestinal ulcers + 5.03 × shallow ulcers + 12.76 × deep ulcers + 4.47 × geographicshaped ulcers + 26.93 × volcano-shaped ulcers + 8.65 ×≥ 20 mm of intestinal ulcers.However,endoscopic parameters used in the multivariate analysis explained only 18.9% of the DAIBD variance.Patients with severe DAIBD scores but with moderately predicted disease activity by the endoscopic severity model had more symptoms of irritable bowel syndrome(21.4% vs 4.9%,P = 0.026) and a lower rate of corticosteroid use(50.0% vs 75.6%,P = 0.016) than those with severe DAIBD scores and accurately predicted disease by the model.CONCLUSION:Our study showed that the number of intestinal ulcers and volcano-shaped ulcers were predictive factors for severe DAIBD scores.However,the correlation between endoscopic severity and DAIBD(r = 0.434) was weak.展开更多
Intestinal Behcet's disease in a 38-year-old woman was diagnosed because of the history of recurrent oral aphthous ulcers, erythema nodosum-like eruptions, genital ulcer, and endoscopic findings of esophageal and Ueo...Intestinal Behcet's disease in a 38-year-old woman was diagnosed because of the history of recurrent oral aphthous ulcers, erythema nodosum-like eruptions, genital ulcer, and endoscopic findings of esophageal and Ueocolonic punched-out ulcers with colonic longitudinal ulcers. Esophageal lesions and colonic longitudinal ulcers are rarely seen in intestinal Behcet's disease. The ulcers of esophagus and ileocolon healed with 3 wk of treatment with prednisolone and mesalazine without any adverse effect. Mesalazine may decrease the total dose of prednisolone required to treat the disease.展开更多
To assess the impact of disease characteristics on the quality of life (QOL) in children with inflammatory bowel diseases (IBD).METHODSThis was a cross-sectional study conducted at the First Department of Pediatrics o...To assess the impact of disease characteristics on the quality of life (QOL) in children with inflammatory bowel diseases (IBD).METHODSThis was a cross-sectional study conducted at the First Department of Pediatrics of the University of Athens at the “Aghia Sophia” Children’s Hospital. Children diagnosed with Crohn’s disease (CD) or ulcerative colitis (UC), who were followed as outpatients or during a hospitalization, participated, after informed consent was obtained from their legal representative. QOL was assessed by the IMPACT-III questionnaire. Demographic data and disease characteristics were also collected. Statistical analyses included parametric (Student’s t-test and Pearson’s r) and non-parametric (Mann-Whitney test, Fisher’s test and Spearman’s rho) procedures.RESULTSNinety-nine patients (UC: 37, 73.0% females, CD: 62, 51.6% females), aged 12.8 ± 2.6 years were included. Overall, as well as, sub-domain scores did not differ between UC and CD (overall score: 73.9 ± 13.3 vs 77.5 ± 11.2, respectively, P = 0.16). In the entire sample, total score was related to physician’s global assessment (PGA, patients classified as “mild/moderate” active disease had, on average, 14.8 ± 2.7 points lower total scores compared to those “in remission”, P < 0.001) and age at IMPACT completion (Pearson’s r = 0.29, P = 0.05). Disease activity assessed by the indices Pediatric Ulcerative Colitis activity index, Pediatric Crohn’s disease activity index or PGA was significantly associated with all subdomains scores. Presence of extraintestinal manifestations had a negative impact on emotional and social functioning domains.CONCLUSIONDisease activity is the main correlate of QOL in children with IBD, underlining the importance of achieving and sustaining clinical remission展开更多
AIM:To evaluate endoscopic findings and the prevalence of H pylori in patients with Behcet's disease (BD) who have upper gastrointestinal symptoms. METHODS:The patients with BD diagnosed according to the Internati...AIM:To evaluate endoscopic findings and the prevalence of H pylori in patients with Behcet's disease (BD) who have upper gastrointestinal symptoms. METHODS:The patients with BD diagnosed according to the International Study Group and followed up in the Department of Dermatology and other related departments and who had any upper gastrointestinal complaints,were included in this study. Forty-five patients with BD and 40 patients in the control group were evaluated by upper gastrointestinal endoscopy and two biopsied specimens were taken during endoscopy for H pylori . A two-week triple therapy for H pylori eradication was administered to H pylori positive patients. Two months after the treatment,the patients were evaluated by urea-breath test for eradication control. RESULTS:Patients with BD had a mean age of 36.2 ± 11.4 years (18-67 years). The mean follow-up time was 35 ± 14 mo (16-84 mo). Aphthous or deep ulcer in esophagus,stomach and duodenum had never been confirmed by endoscopic examination. Most gastric lesions were gastric erosion (40%) and the most duodenal lesions were duodenitis (17.5%) in two groups. H pylori was positive in 33 patients (73.3%) with BD. The two-week triple eradication therapy was successful in 75% of the patients. There was no difference between the groups in respect to prevalence of H pylori (73.3% vs 75%,P > 0.05),and eradication rate (75% vs 70%,P > 0.05). CONCLUSION:Endoscopic findings,eradication rate and prevalence of H pylori were similar in patients withBD and control group.展开更多
Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in ...Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.展开更多
BACKGROUND Magnetic resonance enterography (MRE) and wireless capsule endoscopy (WCE) are equally accepted modalities for noninvasive screening of small bowel involvement (SBI) in children with Crohn’s disease (CD) a...BACKGROUND Magnetic resonance enterography (MRE) and wireless capsule endoscopy (WCE) are equally accepted modalities for noninvasive screening of small bowel involvement (SBI) in children with Crohn’s disease (CD) and indeterminate colitis (IC) albeit there is a paucity of data comparing the two and thereby guiding the clinician in selecting the ideal diagnostic approach. Therefore, the goal of this study is to provide additional evidence for capsule endoscopy role in the evaluation of established Crohn’s disease exacerbation compared to MRE in relation to Pediatric Crohn's Disease Activity Index (PCDAI), and histological indices. AIM To prospectively compare the findings of MRE and WCE and their agreement with PCDAI or histology in children with CD or IC. METHODS Consecutive patients diagnosed with CD and IC were screened for inclusion. After informed consent, patient’s demographic and clinical data was abstracted. The current pediatric disease activity index (PCDAI) and endoscopic findings were included. Patients underwent MRE and WCE including preprocedural patency capsule within a maximum of 7 d of each other. Pathological presence of active small bowel disease in ileal and duodenal biopsies were collected if the endoscopy was performed within 2 mo of the WCE study. Patients who failed to pass the PC were excluded from the study. WCE was read by two different experienced gastroenterologists (Attard TM and Colombo JM) blinded to each other's findings and to the findings on MRE (Mardis NJ). Agreement between WCE reviewers, WCE and MRE findings and concordance between positive PCDAI and SBI based on MRE compared with WCE was computed. RESULTS Forty-five patients were included in the study, 18 withdrew and 27 (20 males and 20 CD), mean age (standard deviation) 13.46 (2.4) years, completed the study protocol. There were no instances of capsule retention. Concordance between gastroenterologist reviewers was excellent for the diagnosis of small intestinal CD with good correlation between the two Lewis scores (r=0.875, P<0.001). Concordance between WCE and MRE was poor (69%). In CD patients, when both MRE and WCE were compared using PCDAI>10 as the standard reference reflecting active small intestinal CD, the sensitivity of MRE and WCE were 100% and 83% respectively and the specificity of MRE and WCE were 57.14% and 78.6%, respectively. If the histology in ileum or/and duodenum was used as the reference for active small bowel involvement, WCE had a higher specificity as compared to MRE (83.3% vs 50%). In patients with Crohn’s disease, those with a positive PCDAI (>10) were more likely to have a positive WCE as compared to those with a negative PCDAI (83% vs 21%;P=0.018). CONCLUSION We suggest that MRE and WCE have a complementary role in the assessment of SBI in CD. WCE detected SBI with a much higher specificity while MRE had a higher sensitivity.展开更多
BACKGROUND A two-to three-fold increased risk of venous thrombotic events(VTE) has been demonstrated in patients with inflammatory bowel disease(IBD) compared to the general population, but less is known about the ris...BACKGROUND A two-to three-fold increased risk of venous thrombotic events(VTE) has been demonstrated in patients with inflammatory bowel disease(IBD) compared to the general population, but less is known about the risk of VTE in child-and pediatric-onset IBD. In recent years, several studies have reported the rising incidence rate of VTE in juvenile patients with IBD, and the related risk factors have been explored.AIM To evaluate the risk of VTE in children and adolescents with IBD.METHODS Articles published up to April 2021 were retrieved from Pub Med, Embase, Cochrane Library, Web of Science, Sino Med, CNKI, and WANFANG. Data from observational studies and clinical work were extracted. The outcome was the occurrence of VTE according to the type of IBD. The available odds ratio(OR) and the corresponding 95% confidence interval(CI) were extracted to compare the outcomes. Effect size(P), odds ratio(OR), and 95%CI were used to assess the association between VTE risk and IBD disease. Subgroup analyses stratified by subtypes of VTE and IBD were performed.RESULTS Twelve studies(7450272 IBD patients) were included in the meta-analysis. Child and adolescent IBD patients showed increased VTE risk(P = 0.02, 95%CI: 0.01-0.03). Subgroup analyses stratified by IBD(ulcerative colitis(UC): P = 0.05, 95%CI: 0.03-0.06;Crohn’s disease(CD): P = 0.02, 95%CI: 0.00-0.04) and VTE subtypes(portal vein thrombosis: P = 0.04, 95%CI: 0.02-0.06;deep vein thrombosis: P = 0.03, 95%CI: 0.01-0.05;central venous catheter-related thrombosis: P = 0.23, 95%CI: 0.00-0.46;thromboembolic events: P = 0.02, 95%CI: 0.01-0.03) revealed a significant correlation between VTE risk and IBD. Patients with IBD were more susceptible to VTE risk than those without IBD(OR = 2.99, 95%CI: 1.45-6.18). The funnel plot was asymmetric, suggesting the presence of significant publication bias. Pediatric and adolescent IBD patients have an increased VTE risk. UC and CD patients exhibited a high risk of VTE. The risk of VTE subtypes was increased in IBD patients.CONCLUSION The current meta-analysis showed that the incidence and risk of VTE are significantly increased in pediatric and adolescent IBD patients. Thus, IBD might be a risk factor for VTE in children and young adults. High-quality prospective cohort studies are necessary to confirm these findings.展开更多
Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free al...Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free alternative cross-sectional imaging method, magnetic resonance imaging of the intestinal structures is costly and time-consuming. Examination of pediatric patients imply additional considerations: reduction of body motions in younger children and consideration of the most appropriate preparation, and examination technique. The demanding Sellink technique is the only means for appropriately distending the lesser intestine in order to detect small bowel strictures. Oral intake of contrast medium(CM) alone shows its limitations regarding distensibility. The need for intravenous contrast media application needs to be considered, too. Active inflammation of both intestinal wall, and mesentery can be demonstrated accurately. Nevertheless, viable alternatives to CM application is desirable, considering non-negligible adverse reactions. Recent data suggest diffusion weighted imaging might fill this diagnostic gap. Irrespective of sequence technique chosen, bowel movement remains a major obstacle. Antispasmolytics in their function as smooth muscle relaxants help in improving image quality, however, their use in children might be off-label. Optimal preparation for the examination and appropriate imaging technique allow for diagnosing typical patterns of changes in IBD, such as bowel wall thickening, ulcers, mural stratification, strictures, creeping fat, and comb sign, and lymphadenopathy. The article gives a detailed overview of current significance of magnetic resonance imaging pediatric patients suffering from IBD, considering indications, limitations, and safety aspects.展开更多
Objective To investigate the clinical features,diagnosis methods,therapeutic principles of intestinal Behcet's disease.Methods The clinical data of 45 patients with confirmed intestinal Behcet's disease admitt...Objective To investigate the clinical features,diagnosis methods,therapeutic principles of intestinal Behcet's disease.Methods The clinical data of 45 patients with confirmed intestinal Behcet's disease admitted to Peking Union Medical College Hospital from August 1998 to April 2010 were retrospectively analyzed.Results The clinical courses of patients with intestinal Behcet's disease were from 26 days to 33 years,and the average duration was 6.32±1.01 years.The appearance of extra-gastrointestinal symptoms was significantly earlier than that of gastrointestinal symptoms(7.35±1.39 years vs.3.24±0.82 years,P<0.05).The predominant gastrointestinal manifestations were right lower quadrant pain(95.56%) and hematochezia or melena(40.00%).Misdiagnosis occurred in 17 cases.In patients without systemic medicine therapy before surgery,the incidence of postoperative infection of incision site and abdominal cavity was significantly higher than that in those undergoing systemic medicine therapy(80.00% vs.0%,P<0.05).Conclusions Because of the diversity of gastrointestinal manifestations,intestinal Behcet's disease is easily misdiagnosed.The systemic medical therapy before surgery could decrease the incidence of infection of incision and abdominal cavity.展开更多
AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.
A 46-year-old woman was diagnosed with palmoplantar pustulosis (PPP) at the Department of Dermatology, Fukushima Medical University Hospital in 2000, and was treated with ointment. However, because liver dysfunction...A 46-year-old woman was diagnosed with palmoplantar pustulosis (PPP) at the Department of Dermatology, Fukushima Medical University Hospital in 2000, and was treated with ointment. However, because liver dysfunction developed in 2003, she was referred to our department, where primary biliary cirrhosis (PBC) was also diagnosed on the basis of clinical findings. One year later, at the age of 49, she developed manifestations of Behcet's disease (BD), including erythema nodosum in the lower extremities. Because she had a history of uveitis, recurrent oral ulceration was present, and the HLA typing was positive for B51, BD was additionally diagnosed. Liver function normalized within three months of the start of treatment with ursodesoxycholic acid (UDCA). This is the first case of PBC associated with BD and PPP.展开更多
The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and an...The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and anti-neutrophilic cytoplasmic antibody(p-ANCA) in the differential diagnosis of BD from IBD.展开更多
BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear geno...BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear genotype-phenotype correlations exist in WD.Protein-truncating nonsense,frame-shift,or splice-site variants may be associated with more severe disease.In contrast,missense variants may be associated with late-onset,less severe disease,and more neurological manifestations.Recently,a gene variant(HSD17B13:TA,rs72613567)with a possible hepatic protective role against toxins was associated with a less severe hepatic phenotype in WD.AIM To analyze the possible genotype-phenotype correlations in children with WD presented with ALF and non-immune hemolytic anemia.METHODS The medical records of children with WD diagnosed and treated in our hospital from January 2006 to December 2020 were retrospectively analyzed.The clinical manifestations(ALF with non-immune hemolytic anemia or other less severe forms),laboratory parameters,copper metabolism,ATP7B variants,and the HSD17B13:TA(rs72613567)variant were reviewed to analyze the possible genotype-phenotype correlations.RESULTS We analyzed the data of 51 patients with WD,26 females(50.98%),with the mean age at the diagnosis of 12.36±3.74 years.ALF and Coombs-negative hemolytic anemia was present in 8 children(15.67%),all adolescent girls.The Kayser-Fleisher ring was present in 9 children(17.65%).The most frequent variants of the ATP7B gene were p.His1069Gln(c.3207A>G)in 38.24% of all alleles,p.Gly1341Asp(c.4021G>A)in 26.47%,p.Trp939Cys(c.2817G>T)in 9.80%,and p.Lys844Ter(c.2530A>T)in 4.90%.In ALF with hemolytic anemia,p.Trp939Cys(c.2817G>T)and p.Lys844Ter(c.2530A>T)variants were more frequent than in other less severe forms,in which p.His1069Gln(c.3207A>G)was more frequent.p.Gly1341Asp(c.4021G>A)has a similar frequency in all hepatic forms.For 33 of the patients,the HSD17B13 genotype was evaluated.The overall HSD17B13:TA allele frequency was 24.24%.Its frequency was higher in patients with less severe liver disease(26.92%)than those with ALF and hemolytic anemia(14.28%).CONCLUSION It remains challenging to prove a genotype-phenotype correlation in WD patients.In children with ALF and hemolytic anemia,the missense variants other than p.His1069Gln(c.3207A>G)and frame-shift variants were the most frequently present in homozygous status or compound heterozygous status with site splice variants.As genetic analysis is usually time-consuming and the results are late,the importance at the onset of the ALF is questionable.If variants proved to be associated with severe forms are found in the pre-symptomatic phase of the disease,this could be essential to predict a possible severe evolution.展开更多
We report here a very rare case of intestinal Behcet's disease with pyoderma gangrenosum. A 16-year-old woman who was diagnosed with intestinal Behcet's disease by the presence of cutaneous pathergy together with tw...We report here a very rare case of intestinal Behcet's disease with pyoderma gangrenosum. A 16-year-old woman who was diagnosed with intestinal Behcet's disease by the presence of cutaneous pathergy together with two major criteria (oral and genital aphthoses) and one minor criterion (gastrointestinal manifestations), was referred to our hospital with a left lower leg ulcer and abdominal pain in September 1989. Colonoscopy demonstrated flare-up colitis involving the entire colon. Her lower leg lesion was a painful destructive ulcer with an irregular margin and a ragged overhanging edge. Based on these clinical and laboratory findings, we diagnosed her cutaneous ulcer as pyoderma gangrenosum developing with exacerbated intestinal Behcet's disease. Her cutaneous and intestinal lesions were poorly controlled though she received oral prednisolone treatment for a month. Because of aggravated abdominal symptoms with peritoneal irritation, we performed total colectomy in November 1989. The resected specimen was histologically compatible with intestinal Behcet' s disease showing severe inflammation with deep ulcerations and neutrophil accumulation. Subsequently, pyoderma gangrenosum rapidly improved. This clinical course may suggest the close relationship between pyoderma gangrenosum and intestinal Behcet's disease.展开更多
AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the ca...AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the candidate gene region(rs11792633,rs7025417,rs10975519 and rs1048274 in IL33;rs2310220,rs12712142,rs13424006 and rs3821204 in IL1RL1)were genotyped in783 BD patients and 701 healthy controls by the Sequenom Mass Array i PLEX platform.RESULTS:A statistically significant association was observed between IL1RL1 rs12712142 and BD patients.The frequency of IL1RL1 rs12712142 variant allele A was significantly lower in BD patients than that in controls(OR=0.8,95%CI:0.69-0.94,Pc=0.039);the genotype distribution(Pc=0.043)and additive and dominant genetic model analyses(OR=0.8,95%CI:0.69-0.94,Pc=0.040 and OR=0.72,95%CI:0.58-0.88,Pc=0.011)also indicated a strong association between rs12712142 and BD patients.CONCLUSION:This is the first study to reveal the association between IL1RL1 rs12712142 variant allele A and the decreased risk of BD in the Chinese Han population,indicating a protective role of IL1RL1 in the pathogenesis of BD.展开更多
Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricul...Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricular arrythmias are believed to be the cause of this increased risk of cardiovascular mortality and it is also thought to be related with cardiac autonomic dysfunction. Heart rate turbulence (HRT) is a new predictor of cardiac autonomic activity. HRT is an independent and powerful predictor of mortality. In this study, we investigated the cardiac autonomic activity which can be determined by HRT in patients with BD. Forty patients with BD (20 men, mean age: 40 ± 9 years, range: 27-55 years) were diagnosed according to the International Study Group Criteria (ISGC) and gender and age matched healthy volunteers (20 men, mean age: 39 ± 8 years, range: 26-56 years) were included in this study. All of the participants (patients and controls) underwent 24 hours Holter electrocardiogram. HRT parameters, turbulence onset (TO) and turbulence slope (TS) were calculated with HRT (View Version 0.60-0.1 of Software Program). There were no significant differences in TO and TS values between patients with BD and control subject (TO-BD: 0.014 ± 0.03, TO-Control: 0.011 ± 0.04;TS-BD: 7.88 ± 4.9, TS-Control: 9.42 ± 6.7 respectively). Although increased cardiovascular mortality rates in BD have been shown in many studies, HRT values—detecting the risk of sudden death—do not seem to be altered in this disease.展开更多
AIM To examine the incidence and trends in pediatric inflammatory bowel diseases(IBDs) over 2000-2015 and project the incidence to 2018. METHODS A 16-year prospective study of IBD patients < 19 years of age was con...AIM To examine the incidence and trends in pediatric inflammatory bowel diseases(IBDs) over 2000-2015 and project the incidence to 2018. METHODS A 16-year prospective study of IBD patients < 19 years of age was conducted in the Czech Republic(the Pilsen region). All incident IBD cases within a well-defined geographical area were retrieved from a prospectively collected computerized clinical database. Historical Czech data were used for comparison(1990-2001). Our catchment population was determined from the census data. We calculated the incidence by relating the number of newly diagnosed cases to the size of thepediatric population-at-risk in each calendar year. Age/sex, disease type, place of residence, and race/ethnicity were identified. RESULTS In total, 170 new IBD cases [105 Crohn's disease(CD), 48 ulcerative colitis(UC), and 17 IBD-unclassified(IBD-U)] were identified. The median age at IBD diagnosis was 14.2 years, 59.4% were males, and 97.1% were Caucasians. A male preponderance of IBD(P = 0.026) and CD(P = 0.016) was observed. With 109209 person-years in the catchment area, the average incidence of IBD per 100000 person-years was 10.0(6.2 for CD, 2.8 for UC, and 1.0 for IBD-U) for children aged 0 to 19 years; for those aged 0 to 15 years, the incidence rate was 7.3(4.6 for CD, 2.0 for UC, and 0.7 for IBD-U). An increase in incidence with age was observed(P = 0.0003). Over the 16-year period, the incidence increased for IBD patients(P = 0.01) and CD in particular(P < 0.0001), whereas the incidence for UC(P = 0.09) and IBD-U(P = 0.339) remained unchanged. IBD-projected data from 2016 to 2018 were 12.1, 12.3 and 12.6 per 100000 personyears, respectively.CONCLUSION Pediatric-onset IBD incidence is around its highest point. The increase, which is particularly pronounced for CD, may be challenging to relate to causes of pediatric disease.展开更多
BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarel...BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarely been reported.CASE SUMMARY Herein,we describe a patient with intestinal BD who presented with decreased oxygen saturation and shortness of breath during a diagnostic colonoscopy.Bilateral pneumothorax,pneumomediastinum,pneumoperitoneum,pneumoretroperitoneum and subcutaneous emphysema of the neck,chest,abdomen,back and scrotum were confirmed by computed tomography scan.The sudden change in condition was considered to be associated with iatrogenic bowel perforation.After receiving closed thoracic drainage and conservative therapy,the patient was discharged in stable condition.CONCLUSION Endoscopists should be aware of the risks of colonoscopy in patients with intestinal BD and the possibility of pneumothorax associated with intestinal perforation and make adequate preparations before colonoscopy.展开更多
Background: The prevention of cardiovascular disease (CVD) can substantially contribute to reducing the mortality rate across countries. The level of interest in cholesterol among experts increases when the subjects a...Background: The prevention of cardiovascular disease (CVD) can substantially contribute to reducing the mortality rate across countries. The level of interest in cholesterol among experts increases when the subjects are elderly and obese individuals. However, specialists do not recommend that children should receive the cholesterol test. The objective of this study was to investigate the distribution of cholesterol levels among the children of public school and their parents’ lifestyles that are associated with cholesterol levels, and to assess the need for and utility of cholesterol testing in school settings. Methods: The study investigated a group of 226 fourth-grade public school children aged between 9 and 10 years and guardians in Akitakatacity of Hiroshima Prefecture, Japan. Multiple regression analyses were performed with the logarithmic value of cholesterol levels as a response variable, awareness about lifestyles and health of children as an explanatory variable, and child’s sex and body mass index (BMI) as moderating variables. Using questionnaires about lifestyles, the step-down procedure was applied in multiple regression analyses to narrow down relevant lifestyle variables. Results: The percentage of children with the high total cholesterol (TC) value was about 15 and with low-density lipoprotein (LDL) cholesterol value was about 10. Children with low high-density lipoprotein (HDL) cholesterol value were 5%. Treatment was not required according to the comprehensive assessment. Cholesterol levels were associated with the situation of the children and their guardians as follows, guardian need to consider the food, and child has the correct knowledge about food like how to eat snacks. Conclusions: Children had poor cholesterol levels. From childhood, along with the parents, there is a need to learn about appropriate level of cholesterol for CVD prevention.展开更多
文摘BACKGROUND Behcet's disease(BD)is an inflammatory disorder known for various symptoms,including oral and genital ulcers and ocular inflammation.Panuveitis,a severe eye condition,is rare as the first sign of BD.CASE SUMMARY We present an unusual case of a 30-year-old man who developed panuveitis after receiving the mRNA-based coronavirus disease 2019(COVID-19)vaccine(Moderna).Laboratory tests ruled out infections,but he had a positive HLA-B51 result and a history of genital ulcer and oral ulcers,leading to a BD diagnosis.Treatment with corticosteroids improved his condition.Interestingly,he had another episode of panuveitis after the second mRNA vaccine dose,which also responded to corticosteroids.CONCLUSION This case highlights the rare onset of BD following mRNA COVID-19 vaccination,suggesting a potential link between these vaccines and BD's eye symptoms,emphasizing the importance of quick treatment in similar cases.
文摘AIM:To develop a novel endoscopic severity model of intestinal Behcet's disease(BD) and to evaluate its feasibility by comparing it with the actual disease activity index for intestinal Behcet's disease(DAIBD).METHODS:We reviewed the medical records of 167 intestinal BD patients between March 1986 and April 2011.We also investigated the endoscopic parameters including ulcer locations,distribution,number,depth,shape,size and margin to identify independent factors associated with DAIBD.An endoscopic severity model was developed using significant colonoscopic variables identified by multivariate regression analysis and its correlation with the DAIBD was evaluated.To determine factors related to the discrepancy between endoscopic severity and clinical activity,clinical characteristics and laboratory markers of the patients were analyzed.RESULTS:A multivariate regression analysis revealed that the number of intestinal ulcers(≥ 2,P = 0.031) and volcanoshaped ulcers(P = 0.001) were predictive factors for the DAIBD.An endoscopic severity model(Y) was developed based on selected endoscopic variables as follows:Y = 47.44 + 9.04 × non-Ileocecal area + 11.85 ×≥ 2 of intestinal ulcers + 5.03 × shallow ulcers + 12.76 × deep ulcers + 4.47 × geographicshaped ulcers + 26.93 × volcano-shaped ulcers + 8.65 ×≥ 20 mm of intestinal ulcers.However,endoscopic parameters used in the multivariate analysis explained only 18.9% of the DAIBD variance.Patients with severe DAIBD scores but with moderately predicted disease activity by the endoscopic severity model had more symptoms of irritable bowel syndrome(21.4% vs 4.9%,P = 0.026) and a lower rate of corticosteroid use(50.0% vs 75.6%,P = 0.016) than those with severe DAIBD scores and accurately predicted disease by the model.CONCLUSION:Our study showed that the number of intestinal ulcers and volcano-shaped ulcers were predictive factors for severe DAIBD scores.However,the correlation between endoscopic severity and DAIBD(r = 0.434) was weak.
文摘Intestinal Behcet's disease in a 38-year-old woman was diagnosed because of the history of recurrent oral aphthous ulcers, erythema nodosum-like eruptions, genital ulcer, and endoscopic findings of esophageal and Ueocolonic punched-out ulcers with colonic longitudinal ulcers. Esophageal lesions and colonic longitudinal ulcers are rarely seen in intestinal Behcet's disease. The ulcers of esophagus and ileocolon healed with 3 wk of treatment with prednisolone and mesalazine without any adverse effect. Mesalazine may decrease the total dose of prednisolone required to treat the disease.
文摘To assess the impact of disease characteristics on the quality of life (QOL) in children with inflammatory bowel diseases (IBD).METHODSThis was a cross-sectional study conducted at the First Department of Pediatrics of the University of Athens at the “Aghia Sophia” Children’s Hospital. Children diagnosed with Crohn’s disease (CD) or ulcerative colitis (UC), who were followed as outpatients or during a hospitalization, participated, after informed consent was obtained from their legal representative. QOL was assessed by the IMPACT-III questionnaire. Demographic data and disease characteristics were also collected. Statistical analyses included parametric (Student’s t-test and Pearson’s r) and non-parametric (Mann-Whitney test, Fisher’s test and Spearman’s rho) procedures.RESULTSNinety-nine patients (UC: 37, 73.0% females, CD: 62, 51.6% females), aged 12.8 ± 2.6 years were included. Overall, as well as, sub-domain scores did not differ between UC and CD (overall score: 73.9 ± 13.3 vs 77.5 ± 11.2, respectively, P = 0.16). In the entire sample, total score was related to physician’s global assessment (PGA, patients classified as “mild/moderate” active disease had, on average, 14.8 ± 2.7 points lower total scores compared to those “in remission”, P < 0.001) and age at IMPACT completion (Pearson’s r = 0.29, P = 0.05). Disease activity assessed by the indices Pediatric Ulcerative Colitis activity index, Pediatric Crohn’s disease activity index or PGA was significantly associated with all subdomains scores. Presence of extraintestinal manifestations had a negative impact on emotional and social functioning domains.CONCLUSIONDisease activity is the main correlate of QOL in children with IBD, underlining the importance of achieving and sustaining clinical remission
文摘AIM:To evaluate endoscopic findings and the prevalence of H pylori in patients with Behcet's disease (BD) who have upper gastrointestinal symptoms. METHODS:The patients with BD diagnosed according to the International Study Group and followed up in the Department of Dermatology and other related departments and who had any upper gastrointestinal complaints,were included in this study. Forty-five patients with BD and 40 patients in the control group were evaluated by upper gastrointestinal endoscopy and two biopsied specimens were taken during endoscopy for H pylori . A two-week triple therapy for H pylori eradication was administered to H pylori positive patients. Two months after the treatment,the patients were evaluated by urea-breath test for eradication control. RESULTS:Patients with BD had a mean age of 36.2 ± 11.4 years (18-67 years). The mean follow-up time was 35 ± 14 mo (16-84 mo). Aphthous or deep ulcer in esophagus,stomach and duodenum had never been confirmed by endoscopic examination. Most gastric lesions were gastric erosion (40%) and the most duodenal lesions were duodenitis (17.5%) in two groups. H pylori was positive in 33 patients (73.3%) with BD. The two-week triple eradication therapy was successful in 75% of the patients. There was no difference between the groups in respect to prevalence of H pylori (73.3% vs 75%,P > 0.05),and eradication rate (75% vs 70%,P > 0.05). CONCLUSION:Endoscopic findings,eradication rate and prevalence of H pylori were similar in patients withBD and control group.
文摘Behcet's disease(BD) is a rare and life-long disorder characterized by inflammation of blood vessels throughout the body. BD was originally described in 1937 as a syndrome involving oral and genital ulceration in addition to ocular inflammation. Intestinal BD refers to colonic ulcerative lesions documented by objective measures in patients with BD. Many studies have shown that over 40% of BD patients have gastrointestinal complaints. Symptoms include abdominal pain, diarrhea, nausea, anorexia and abdominal distension. Although gastrointestinal symptoms are common, the demonstration of gastrointestinal ulcers is rare. This so-called intestinal BD accounts for approximately 1% of cases. There is no specific test for BD, and the diagnosis is based on clinical criteria. The manifestations of intestinal BD are similar to those of other colitis conditions such as Crohn's disease or intestinal tuberculosis, thus, it is challenging for gastroenterologists to accurately diagnose intestinal BD in patients with ileocolonic ulcers. However, giant ulcers distributed in the esophagus and ileocecal junction with gastrointestinal hemorrhage are rare in intestinal BD. Here, we present a case of untypical intestinal BD. The patient had recurrent aphthous ulceration of the oral mucosa, and esophageal and ileo-colonic ulceration, but no typical extra-intestinal symptoms. During examination, the patient had massive acute lower gastrointestinal bleeding. The patient underwent ileostomy after an emergency right hemicolectomy and partial ileectomy, and was subsequently diagnosed with incomplete-type intestinal BD by pathology. The literature on the evaluation and management of this condition is reviewed.
基金Supported by the donation of wireless Small bowel capsule and patency capsules from Giving imaging,Ltd Medtronic company with the funding agreement from Given investigator-initiated study No.13-12
文摘BACKGROUND Magnetic resonance enterography (MRE) and wireless capsule endoscopy (WCE) are equally accepted modalities for noninvasive screening of small bowel involvement (SBI) in children with Crohn’s disease (CD) and indeterminate colitis (IC) albeit there is a paucity of data comparing the two and thereby guiding the clinician in selecting the ideal diagnostic approach. Therefore, the goal of this study is to provide additional evidence for capsule endoscopy role in the evaluation of established Crohn’s disease exacerbation compared to MRE in relation to Pediatric Crohn's Disease Activity Index (PCDAI), and histological indices. AIM To prospectively compare the findings of MRE and WCE and their agreement with PCDAI or histology in children with CD or IC. METHODS Consecutive patients diagnosed with CD and IC were screened for inclusion. After informed consent, patient’s demographic and clinical data was abstracted. The current pediatric disease activity index (PCDAI) and endoscopic findings were included. Patients underwent MRE and WCE including preprocedural patency capsule within a maximum of 7 d of each other. Pathological presence of active small bowel disease in ileal and duodenal biopsies were collected if the endoscopy was performed within 2 mo of the WCE study. Patients who failed to pass the PC were excluded from the study. WCE was read by two different experienced gastroenterologists (Attard TM and Colombo JM) blinded to each other's findings and to the findings on MRE (Mardis NJ). Agreement between WCE reviewers, WCE and MRE findings and concordance between positive PCDAI and SBI based on MRE compared with WCE was computed. RESULTS Forty-five patients were included in the study, 18 withdrew and 27 (20 males and 20 CD), mean age (standard deviation) 13.46 (2.4) years, completed the study protocol. There were no instances of capsule retention. Concordance between gastroenterologist reviewers was excellent for the diagnosis of small intestinal CD with good correlation between the two Lewis scores (r=0.875, P<0.001). Concordance between WCE and MRE was poor (69%). In CD patients, when both MRE and WCE were compared using PCDAI>10 as the standard reference reflecting active small intestinal CD, the sensitivity of MRE and WCE were 100% and 83% respectively and the specificity of MRE and WCE were 57.14% and 78.6%, respectively. If the histology in ileum or/and duodenum was used as the reference for active small bowel involvement, WCE had a higher specificity as compared to MRE (83.3% vs 50%). In patients with Crohn’s disease, those with a positive PCDAI (>10) were more likely to have a positive WCE as compared to those with a negative PCDAI (83% vs 21%;P=0.018). CONCLUSION We suggest that MRE and WCE have a complementary role in the assessment of SBI in CD. WCE detected SBI with a much higher specificity while MRE had a higher sensitivity.
文摘BACKGROUND A two-to three-fold increased risk of venous thrombotic events(VTE) has been demonstrated in patients with inflammatory bowel disease(IBD) compared to the general population, but less is known about the risk of VTE in child-and pediatric-onset IBD. In recent years, several studies have reported the rising incidence rate of VTE in juvenile patients with IBD, and the related risk factors have been explored.AIM To evaluate the risk of VTE in children and adolescents with IBD.METHODS Articles published up to April 2021 were retrieved from Pub Med, Embase, Cochrane Library, Web of Science, Sino Med, CNKI, and WANFANG. Data from observational studies and clinical work were extracted. The outcome was the occurrence of VTE according to the type of IBD. The available odds ratio(OR) and the corresponding 95% confidence interval(CI) were extracted to compare the outcomes. Effect size(P), odds ratio(OR), and 95%CI were used to assess the association between VTE risk and IBD disease. Subgroup analyses stratified by subtypes of VTE and IBD were performed.RESULTS Twelve studies(7450272 IBD patients) were included in the meta-analysis. Child and adolescent IBD patients showed increased VTE risk(P = 0.02, 95%CI: 0.01-0.03). Subgroup analyses stratified by IBD(ulcerative colitis(UC): P = 0.05, 95%CI: 0.03-0.06;Crohn’s disease(CD): P = 0.02, 95%CI: 0.00-0.04) and VTE subtypes(portal vein thrombosis: P = 0.04, 95%CI: 0.02-0.06;deep vein thrombosis: P = 0.03, 95%CI: 0.01-0.05;central venous catheter-related thrombosis: P = 0.23, 95%CI: 0.00-0.46;thromboembolic events: P = 0.02, 95%CI: 0.01-0.03) revealed a significant correlation between VTE risk and IBD. Patients with IBD were more susceptible to VTE risk than those without IBD(OR = 2.99, 95%CI: 1.45-6.18). The funnel plot was asymmetric, suggesting the presence of significant publication bias. Pediatric and adolescent IBD patients have an increased VTE risk. UC and CD patients exhibited a high risk of VTE. The risk of VTE subtypes was increased in IBD patients.CONCLUSION The current meta-analysis showed that the incidence and risk of VTE are significantly increased in pediatric and adolescent IBD patients. Thus, IBD might be a risk factor for VTE in children and young adults. High-quality prospective cohort studies are necessary to confirm these findings.
文摘Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free alternative cross-sectional imaging method, magnetic resonance imaging of the intestinal structures is costly and time-consuming. Examination of pediatric patients imply additional considerations: reduction of body motions in younger children and consideration of the most appropriate preparation, and examination technique. The demanding Sellink technique is the only means for appropriately distending the lesser intestine in order to detect small bowel strictures. Oral intake of contrast medium(CM) alone shows its limitations regarding distensibility. The need for intravenous contrast media application needs to be considered, too. Active inflammation of both intestinal wall, and mesentery can be demonstrated accurately. Nevertheless, viable alternatives to CM application is desirable, considering non-negligible adverse reactions. Recent data suggest diffusion weighted imaging might fill this diagnostic gap. Irrespective of sequence technique chosen, bowel movement remains a major obstacle. Antispasmolytics in their function as smooth muscle relaxants help in improving image quality, however, their use in children might be off-label. Optimal preparation for the examination and appropriate imaging technique allow for diagnosing typical patterns of changes in IBD, such as bowel wall thickening, ulcers, mural stratification, strictures, creeping fat, and comb sign, and lymphadenopathy. The article gives a detailed overview of current significance of magnetic resonance imaging pediatric patients suffering from IBD, considering indications, limitations, and safety aspects.
文摘Objective To investigate the clinical features,diagnosis methods,therapeutic principles of intestinal Behcet's disease.Methods The clinical data of 45 patients with confirmed intestinal Behcet's disease admitted to Peking Union Medical College Hospital from August 1998 to April 2010 were retrospectively analyzed.Results The clinical courses of patients with intestinal Behcet's disease were from 26 days to 33 years,and the average duration was 6.32±1.01 years.The appearance of extra-gastrointestinal symptoms was significantly earlier than that of gastrointestinal symptoms(7.35±1.39 years vs.3.24±0.82 years,P<0.05).The predominant gastrointestinal manifestations were right lower quadrant pain(95.56%) and hematochezia or melena(40.00%).Misdiagnosis occurred in 17 cases.In patients without systemic medicine therapy before surgery,the incidence of postoperative infection of incision site and abdominal cavity was significantly higher than that in those undergoing systemic medicine therapy(80.00% vs.0%,P<0.05).Conclusions Because of the diversity of gastrointestinal manifestations,intestinal Behcet's disease is easily misdiagnosed.The systemic medical therapy before surgery could decrease the incidence of infection of incision and abdominal cavity.
文摘AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.
文摘A 46-year-old woman was diagnosed with palmoplantar pustulosis (PPP) at the Department of Dermatology, Fukushima Medical University Hospital in 2000, and was treated with ointment. However, because liver dysfunction developed in 2003, she was referred to our department, where primary biliary cirrhosis (PBC) was also diagnosed on the basis of clinical findings. One year later, at the age of 49, she developed manifestations of Behcet's disease (BD), including erythema nodosum in the lower extremities. Because she had a history of uveitis, recurrent oral ulceration was present, and the HLA typing was positive for B51, BD was additionally diagnosed. Liver function normalized within three months of the start of treatment with ursodesoxycholic acid (UDCA). This is the first case of PBC associated with BD and PPP.
文摘The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and anti-neutrophilic cytoplasmic antibody(p-ANCA) in the differential diagnosis of BD from IBD.
文摘BACKGROUND Wilson’s disease(WD)is a rare autosomal recessive inherited disorder of copper metabolism.Acute liver failure(ALF)and hemolytic anemia represent the most severe presentation of WD in children.No clear genotype-phenotype correlations exist in WD.Protein-truncating nonsense,frame-shift,or splice-site variants may be associated with more severe disease.In contrast,missense variants may be associated with late-onset,less severe disease,and more neurological manifestations.Recently,a gene variant(HSD17B13:TA,rs72613567)with a possible hepatic protective role against toxins was associated with a less severe hepatic phenotype in WD.AIM To analyze the possible genotype-phenotype correlations in children with WD presented with ALF and non-immune hemolytic anemia.METHODS The medical records of children with WD diagnosed and treated in our hospital from January 2006 to December 2020 were retrospectively analyzed.The clinical manifestations(ALF with non-immune hemolytic anemia or other less severe forms),laboratory parameters,copper metabolism,ATP7B variants,and the HSD17B13:TA(rs72613567)variant were reviewed to analyze the possible genotype-phenotype correlations.RESULTS We analyzed the data of 51 patients with WD,26 females(50.98%),with the mean age at the diagnosis of 12.36±3.74 years.ALF and Coombs-negative hemolytic anemia was present in 8 children(15.67%),all adolescent girls.The Kayser-Fleisher ring was present in 9 children(17.65%).The most frequent variants of the ATP7B gene were p.His1069Gln(c.3207A>G)in 38.24% of all alleles,p.Gly1341Asp(c.4021G>A)in 26.47%,p.Trp939Cys(c.2817G>T)in 9.80%,and p.Lys844Ter(c.2530A>T)in 4.90%.In ALF with hemolytic anemia,p.Trp939Cys(c.2817G>T)and p.Lys844Ter(c.2530A>T)variants were more frequent than in other less severe forms,in which p.His1069Gln(c.3207A>G)was more frequent.p.Gly1341Asp(c.4021G>A)has a similar frequency in all hepatic forms.For 33 of the patients,the HSD17B13 genotype was evaluated.The overall HSD17B13:TA allele frequency was 24.24%.Its frequency was higher in patients with less severe liver disease(26.92%)than those with ALF and hemolytic anemia(14.28%).CONCLUSION It remains challenging to prove a genotype-phenotype correlation in WD patients.In children with ALF and hemolytic anemia,the missense variants other than p.His1069Gln(c.3207A>G)and frame-shift variants were the most frequently present in homozygous status or compound heterozygous status with site splice variants.As genetic analysis is usually time-consuming and the results are late,the importance at the onset of the ALF is questionable.If variants proved to be associated with severe forms are found in the pre-symptomatic phase of the disease,this could be essential to predict a possible severe evolution.
文摘We report here a very rare case of intestinal Behcet's disease with pyoderma gangrenosum. A 16-year-old woman who was diagnosed with intestinal Behcet's disease by the presence of cutaneous pathergy together with two major criteria (oral and genital aphthoses) and one minor criterion (gastrointestinal manifestations), was referred to our hospital with a left lower leg ulcer and abdominal pain in September 1989. Colonoscopy demonstrated flare-up colitis involving the entire colon. Her lower leg lesion was a painful destructive ulcer with an irregular margin and a ragged overhanging edge. Based on these clinical and laboratory findings, we diagnosed her cutaneous ulcer as pyoderma gangrenosum developing with exacerbated intestinal Behcet's disease. Her cutaneous and intestinal lesions were poorly controlled though she received oral prednisolone treatment for a month. Because of aggravated abdominal symptoms with peritoneal irritation, we performed total colectomy in November 1989. The resected specimen was histologically compatible with intestinal Behcet' s disease showing severe inflammation with deep ulcerations and neutrophil accumulation. Subsequently, pyoderma gangrenosum rapidly improved. This clinical course may suggest the close relationship between pyoderma gangrenosum and intestinal Behcet's disease.
基金the National Natural Science Foundation of China (No.81770917)。
文摘AIM:To explore the association of single nucleotide polymorphisms(SNPs)in the IL33/IL1RL1 gene region with the susceptibility to Behcet’s disease(BD)in a Chinese Han population.METHODS:A total of eight SNPs in the candidate gene region(rs11792633,rs7025417,rs10975519 and rs1048274 in IL33;rs2310220,rs12712142,rs13424006 and rs3821204 in IL1RL1)were genotyped in783 BD patients and 701 healthy controls by the Sequenom Mass Array i PLEX platform.RESULTS:A statistically significant association was observed between IL1RL1 rs12712142 and BD patients.The frequency of IL1RL1 rs12712142 variant allele A was significantly lower in BD patients than that in controls(OR=0.8,95%CI:0.69-0.94,Pc=0.039);the genotype distribution(Pc=0.043)and additive and dominant genetic model analyses(OR=0.8,95%CI:0.69-0.94,Pc=0.040 and OR=0.72,95%CI:0.58-0.88,Pc=0.011)also indicated a strong association between rs12712142 and BD patients.CONCLUSION:This is the first study to reveal the association between IL1RL1 rs12712142 variant allele A and the decreased risk of BD in the Chinese Han population,indicating a protective role of IL1RL1 in the pathogenesis of BD.
文摘Behcet’s disease (BD) is a chronic inflammatory disorder that can affect many systems in the body. Cardiac involvement increases the risk of cardiovascular mortality and occurs in 1%-5% of patients with BD. Ventricular arrythmias are believed to be the cause of this increased risk of cardiovascular mortality and it is also thought to be related with cardiac autonomic dysfunction. Heart rate turbulence (HRT) is a new predictor of cardiac autonomic activity. HRT is an independent and powerful predictor of mortality. In this study, we investigated the cardiac autonomic activity which can be determined by HRT in patients with BD. Forty patients with BD (20 men, mean age: 40 ± 9 years, range: 27-55 years) were diagnosed according to the International Study Group Criteria (ISGC) and gender and age matched healthy volunteers (20 men, mean age: 39 ± 8 years, range: 26-56 years) were included in this study. All of the participants (patients and controls) underwent 24 hours Holter electrocardiogram. HRT parameters, turbulence onset (TO) and turbulence slope (TS) were calculated with HRT (View Version 0.60-0.1 of Software Program). There were no significant differences in TO and TS values between patients with BD and control subject (TO-BD: 0.014 ± 0.03, TO-Control: 0.011 ± 0.04;TS-BD: 7.88 ± 4.9, TS-Control: 9.42 ± 6.7 respectively). Although increased cardiovascular mortality rates in BD have been shown in many studies, HRT values—detecting the risk of sudden death—do not seem to be altered in this disease.
基金Supported by the"On Our Own Feet Movement-Práteléstonozky"-Endowment Program
文摘AIM To examine the incidence and trends in pediatric inflammatory bowel diseases(IBDs) over 2000-2015 and project the incidence to 2018. METHODS A 16-year prospective study of IBD patients < 19 years of age was conducted in the Czech Republic(the Pilsen region). All incident IBD cases within a well-defined geographical area were retrieved from a prospectively collected computerized clinical database. Historical Czech data were used for comparison(1990-2001). Our catchment population was determined from the census data. We calculated the incidence by relating the number of newly diagnosed cases to the size of thepediatric population-at-risk in each calendar year. Age/sex, disease type, place of residence, and race/ethnicity were identified. RESULTS In total, 170 new IBD cases [105 Crohn's disease(CD), 48 ulcerative colitis(UC), and 17 IBD-unclassified(IBD-U)] were identified. The median age at IBD diagnosis was 14.2 years, 59.4% were males, and 97.1% were Caucasians. A male preponderance of IBD(P = 0.026) and CD(P = 0.016) was observed. With 109209 person-years in the catchment area, the average incidence of IBD per 100000 person-years was 10.0(6.2 for CD, 2.8 for UC, and 1.0 for IBD-U) for children aged 0 to 19 years; for those aged 0 to 15 years, the incidence rate was 7.3(4.6 for CD, 2.0 for UC, and 0.7 for IBD-U). An increase in incidence with age was observed(P = 0.0003). Over the 16-year period, the incidence increased for IBD patients(P = 0.01) and CD in particular(P < 0.0001), whereas the incidence for UC(P = 0.09) and IBD-U(P = 0.339) remained unchanged. IBD-projected data from 2016 to 2018 were 12.1, 12.3 and 12.6 per 100000 personyears, respectively.CONCLUSION Pediatric-onset IBD incidence is around its highest point. The increase, which is particularly pronounced for CD, may be challenging to relate to causes of pediatric disease.
文摘BACKGROUND Colonoscopy is essential for the diagnosis of intestinal Behcet’s disease(BD),which is characterized by a typical oval-shaped ulcer in the ileocecal region.However,potential risks of colonoscopy have rarely been reported.CASE SUMMARY Herein,we describe a patient with intestinal BD who presented with decreased oxygen saturation and shortness of breath during a diagnostic colonoscopy.Bilateral pneumothorax,pneumomediastinum,pneumoperitoneum,pneumoretroperitoneum and subcutaneous emphysema of the neck,chest,abdomen,back and scrotum were confirmed by computed tomography scan.The sudden change in condition was considered to be associated with iatrogenic bowel perforation.After receiving closed thoracic drainage and conservative therapy,the patient was discharged in stable condition.CONCLUSION Endoscopists should be aware of the risks of colonoscopy in patients with intestinal BD and the possibility of pneumothorax associated with intestinal perforation and make adequate preparations before colonoscopy.
文摘Background: The prevention of cardiovascular disease (CVD) can substantially contribute to reducing the mortality rate across countries. The level of interest in cholesterol among experts increases when the subjects are elderly and obese individuals. However, specialists do not recommend that children should receive the cholesterol test. The objective of this study was to investigate the distribution of cholesterol levels among the children of public school and their parents’ lifestyles that are associated with cholesterol levels, and to assess the need for and utility of cholesterol testing in school settings. Methods: The study investigated a group of 226 fourth-grade public school children aged between 9 and 10 years and guardians in Akitakatacity of Hiroshima Prefecture, Japan. Multiple regression analyses were performed with the logarithmic value of cholesterol levels as a response variable, awareness about lifestyles and health of children as an explanatory variable, and child’s sex and body mass index (BMI) as moderating variables. Using questionnaires about lifestyles, the step-down procedure was applied in multiple regression analyses to narrow down relevant lifestyle variables. Results: The percentage of children with the high total cholesterol (TC) value was about 15 and with low-density lipoprotein (LDL) cholesterol value was about 10. Children with low high-density lipoprotein (HDL) cholesterol value were 5%. Treatment was not required according to the comprehensive assessment. Cholesterol levels were associated with the situation of the children and their guardians as follows, guardian need to consider the food, and child has the correct knowledge about food like how to eat snacks. Conclusions: Children had poor cholesterol levels. From childhood, along with the parents, there is a need to learn about appropriate level of cholesterol for CVD prevention.