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20% der Bevolkerung leiden an unangenehmen oder schmerzhaften Blahungen. Sie konnen durch eine Vielzahl abdominaler Erkrankungen verursacht werden. Daruber hinaus sind Blahungen eines der Kardinalsymptome des Reizdarmsyndroms.

Refractory celiac disease (RCD) refers to a rare subgroup of patients with celiac disease who show clinical signs of malabsorption despite a gluten-free diet. RCD is divided into an autoimmune phenotype (RCD type I) and pre-lymphoma (RCD type II). To reflect the clinical reality in managing this disease in Germany, a national register was established based on a questionnaire developed specifically for this purpose. Between 2014 and 2020, a total of 53 patients were registered. The diagnosis of RCD was confirmed in 46 cases (87%). This included 27 patients (59%) with RCD type I and 19 patients (41%) with RCD type II. A wide range of diagnostic and therapeutic measures was used. Therapeutically, budesonide was used in 59% of the RCD patients regardless of the subtype. Nutritional therapy was used in only 5 patients (11%). Overall mortality was 26% (12 patients) with a clear dominance in patients with RCD type II (9 patients, 47%). In summary, RCD needs to become a focus of national guidelines to increase awareness, establish standards, and thus enable the treating physician to make the correct diagnosis in a timely manner. Moreover, we concluded that when treating such patients, contacting a specialized center is recommended to ensure sufficient management.Die Refraktäre Zöliakie (RCD) stellt eine seltene Komplikation der Zöliakie dar. Hier kommt es trotz einer strikten glutenfreien Diät zu einem Malabsorptionssyndrom auf der Basis einer Enteropathie mit Dünndarm-Zottenatrophie und -Kryptenhyperplasie. Unterteilt wird die RCD in einen autoimmunen Phänotyp (RCD Typ I) und ein Prä-Lymphom (RCD Typ II). Um die Versorgungsrealität im Umgang mit dieser Erkrankung in Deutschland abzubilden, erfolgte anhand eines eigens dafür entwickelten Fragebogens die Etablierung eines nationalen Registers. Zwischen 2014 und 2020 wurden insgesamt 53 Patienten gemeldet, bei denen in 46 Fällen (87%) die Diagnose RCD bestätigt werden konnte. Hierbei handelte es sich um 27 Patienten (59%) mit einer RCD Typ I und 19 Patienten (41%) mit einer RCD Typ II. Es zeigte sich ein breites Spektrum zur Anwendung kommender diagnostischer und therapeutischer Maßnahmen. Therapeutisch wurde unabhängig vom Subtyp bei 59% der RCD-Patienten Budesonid eingesetzt. Eine Ernährungstherapie erfolgte nur bei 5 Patienten (11%). Die Mortalität lag insgesamt bei 26% (12 Patienten) mit einer deutlichen Dominanz der Patienten mit einer RCD Typ II (9 Patienten, 47%). Zusammenfassend muss die RCD stärker in den Fokus nationaler Leitlinien gerückt werden, um deren Bekanntheitsgrad zu steigern, Standards zu etablieren und es so dem behandelnden Arzt zu ermöglichen, zeitnah die korrekte Diagnose zu stellen. Bei der Behandlung solcher Patienten muss aufgrund der Seltenheit der Erkrankung einmal mehr der Kontakt zu einem spezialisierten Zentrum empfohlen werden.

There are currently limited insights into the progression of human primary humoral immunity despite numerous studies in experimental models. In this study, we analyzed a primary and related secondary parenteral keyhole limpet hemocyanin (KLH) immunization in five human adults. The primary challenge elicited discordant KLH-specific serum and blood effector B cell responses (i.e., dominant serum KLH-specific IgG and IgM levels versus dominant KLH-specific IgA plasmablast frequencies). Single-cell IgH sequencing revealed early appearance of highly (>15 mutations) mutated circulating KLH-specific plasmablasts 2 wk after primary KLH immunization, with simultaneous KLH-specific plasmablasts carrying non- and low-mutated IgH sequences. The data suggest that the highly mutated cells might originate from cross-reactive memory B cells (mBCs) rather than from the naive B cell repertoire, consistent with previous reported mutation rates and the presence of KLH-reactive mBCs in naive vaccinees prior to immunization. Whereas upon secondary immunization, serum Ab response kinetics and plasmablast mutation loads suggested the exclusive reactivation of KLH-specific mBCs, we, however, detected only little clonal overlap between the peripheral KLH-specific secondary plasmablast IgH repertoire and the primary plasmablast and mBC repertoire, respectively. Our data provide novel mechanistic insights into human humoral immune responses and suggest that primary KLH immunization recruits both naive B cells and cross-reactive mBCs, whereas secondary challenge exclusively recruits from a memory repertoire, with little clonal overlap with the primary response.

In patients with inflammatory bowel disease (IBD), restless legs syndrome (RLS) may occur as an extraintestinal disease manifestation. Iron deficiency (ID) or folate deficiency/vitamin B12 deficiency (FD/VB12D) has previously been described to cause RLS. Here, we determined the prevalence and severity of RLS in IBD patients and evaluated the effect of iron and/or folic acid/vitamin B12 supplementation. Patients were screened for ID and RLS by a gastroenterologist. If RLS was suspected, a neurologist was consulted for definitive diagnosis and severity. Patients with RLS and ID, FD, or VB12D received supplementation and were followed-up at weeks 4 and 11 after starting supplementation. A total of 353 IBD patients were included. Prevalence for RLS was 9.4% in Crohn’s disease (CD) and 8% in ulcerative colitis (UC). Prevalence for the subgroup of clinically relevant RLS (symptoms ≥ twice/week with at least moderate distress) was 7.1% (n = 16) for CD and 4.8% (n = 6) for UC. 38.7% of RLS patients presented with ID, FD, and/or VB12D. Most frequently ID was seen (25.8%; n = 8). Iron supplementation resulted in RLS improvement (p = 0.029) at week 4 in seven out of eight patients. Although the overall prevalence of RLS in IBD did not differ to the general population, clinically relevant RLS was more frequent in IBD patients and, therefore, it is important for clinicians to be aware of RLS symptoms. Though for definite diagnosis and proper treatment of RLS, a neurologist must be consulted. Additionally, iron supplementation of IBD patients with ID can improve RLS symptoms. ClinicalTrials.gov No. NCT03457571

Many recommendations from clinical practice guidelines are not implemented. We aimed to develop and evaluate a multifaceted strategy for the implementation of guidelines for Crohn’s disease (CD) and ulcerative colitis (UC). In the intervention region (Berlin, Germany), a continuing medical education course was held, brief guidelines for practice were distributed to all family physicians and gastroenterologists, and patient guidelines were distributed to all surveyed patients. Educational outreach visits with local opinion leaders were also conducted. No specific interventions were performed in the control region (Hamburg, Germany). Prior to the intervention and 1 year later, 1900 members of three statutory sickness funds were asked about their treatment according to guidelines with (1) long-term aminosalicylates and (2) immunosuppressants, (3) whether they took long-term glucocorticoids for maintenance of remission, (4) if they smoked, in CD patients, and (5) about the surveillance colonoscopies, in UC patients. Response rate after implementation was 20.1%. Responders differed between intervention and control region by age and by distribution between patients with UC or CD. After 1 year, more patients were treated according to clinical practice guidelines in the control region than in the intervention region. More patients in the intervention region took immunosuppressants after 1 year, and fewer had a surveillance colonoscopy. However, no before–after comparison was statistically significant. This implementation strategy of UC and CD guidelines did not result in a statistically significant effect. Future implementation of guidelines for inflammatory bowel disease might need thorough evaluation of barriers and the support of theory-based concepts.

There is a growing evidence for over-, under-, or misuse of health care in patients with inflammatory bowel disease. Most studies looked at treatment variability or used quality measures, which mostly capture supportive interventions rather than treatment of IBD in itself. We aimed to evaluate if current recommendations in clinical practice guidelines regarding the medical treatment of patients with inflammatory bowel diseases are being followed in Germany. A questionnaire was sent to 1901 patients insured with two large German statutory sickness funds and an ICD 10 diagnosis of Crohn’s disease (CD) or ulcerative colitis (UC). The questionnaire asked about drug treatment, indications for drug treatment, provision of surveillance endoscopies in ulcerative colitis patients, and smoking status in Crohn’s disease patients. Out of 460 evaluable patients, 62.4% of UC patients and 53.9% of CD patients were treated with mesalamine according to guidelines, 91.3% of all patients were treated with glucocorticoids according to guideline recommendations, while only 75.6% received recommended immunosuppressive treatment. Of UC patients, 94.5% had surveillance colonoscopies at the recommended interval and 58.8% of CD patients were non-smokers. No predictor for overall treatment according to guidelines could be found while being of age older than 60 or being treated outside of a dedicated IBD clinic was associated with less immunosuppressive treatment. A large proportion of patients with IBD do not receive drug treatment in accordance with clinical practice guidelines. Quality improvement measures are much needed.

The optimal method for detecting CMV colitis in patients with inflammatory bowel disease (IBD) has not been established. We wanted to investigate which diagnostic test would be most accurate when defining CMV colitis rather by the further clinical course than by using another diagnostic modality. All consecutive patients with moderately or severely active IBD who had been tested for CMV by PCR, histology, or antigenemia assay at the two campuses CBF and CCM of the Charite - Universitatsmedizin Berlin between September 2006 and September 2009 were included in this retrospective study. During that time, in patients with a positive CMV test, immunosuppressive treatment of any kind was immediately reduced and antiviral treatment was started. This allowed identifying patients who responded to antiviral treatment and those who only responded to later escalation of immunosuppressive therapy. One hundred and nine patients were identified, out of whom nine were considered to have clinically relevant CMV colitis. Sensitivity and specificity were 1 and 0.94 for CMV PCR and 0.5 and 1 for pp65 antigen immunofluorescence assay from peripheral blood, 0.67 and 0.98 for immunohistochemistry, and 0.17 and 0.98 for hematoxylin-eosin staining. When using absence of leukocytosis, splenomegaly, and steroid refractory disease as clinical parameters to test for CMV colitis, blood CMV PCR and immunohistochemistry were able to exclude CMV colitis in negative patients with a 75% likelihood of positive patients to have clinically relevant CMV colitis. Blood-based CMV PCR together with simple clinical parameters can exclude clinically relevant CMV colitis at a high specificity.

Background & Aims Point of care tests (POCTs) might be used to identify patients with undiagnosed celiac disease who require further evaluation. We performed a large multicenter study to determine the performance of a POCT for celiac disease and assessed celiac disease prevalence in endoscopy centers. Methods We performed a prospective study of 1055 patients (888 adults; median age, 48 yrs and 167 children; median age, 10 yrs) referred to 8 endoscopy centers in Germany, for various indications, from January 2016 through June 2017. Patients were tested for celiac disease using Simtomax, which detects immunoglobulin (Ig)A and IgG antibodies against deamidated gliadin peptides (DGP). Results were compared with findings from histologic analyses of duodenal biopsies (reference standard). The primary aim was to determine the accuracy of this POCT for the detection of celiac disease, to identify candidates for duodenal biopsy. A secondary aim was to determine the prevalence of celiac disease in adult and pediatric populations referred for outpatient endoscopic evaluation. Results The overall prevalence of celiac disease was 4.1%. The POCT identified individuals with celiac disease with 79% sensitivity (95% CI, 64%–89%) and 94% specificity (95% CI, 93%–96%). Positive and negative predictive values were 37% and 99%. When we analyzed the adult and pediatric populations separately, we found the test to identify adults with celiac disease (prevalence 1.2%) with 100% sensitivity and 95% specificity. In the pediatric population (celiac disease prevalence 19.6%), the test produced false-negative results for 9 cases; the test therefore identified children with celiac disease with 72% sensitivity (95% CI 53%–86%). Analyses of serologic data revealed significantly lower DGP titers in the false-negative vs the true-positive group. Conclusions In a study of more than 1000 adults and children, we found the Simtomax POCT to detect celiac disease with lower overall levels of sensitivity than expected. Although the test identifies adults with celiac disease with high levels of sensitivity and specificity, the prevalence of celiac disease was as low as 1.2% among adults. The test’s lack of sensitivity might be due to the low intensity of the POCT bands and was associated with low serum DGP titers. Study ID no: DRKS00012499.

Background: Intra-abdominal abscesses [IAAs] are common life-threatening complications in patients with Crohn's disease [CD]. In addition to interventional drainage and surgical therapy, empirical antibiotic therapy represents a cornerstone of treatment, but contemporary data on microbial spectra and antimicrobial resistance are scarce. Methods: We recruited 105 patients with CD and IAAs from nine German centres for a prospective registry in order to characterize the microbiological spectrum, resistance profiles, antibiotic therapy and outcome. Results: In 92 of 105 patients, microbial investigations of abscess material revealed pathogenic microorganisms. A total of 174 pathogens were isolated, with a median of 2 pathogens per culture [range: 1-6]. Most frequently isolated pathogens were E. coli [45 patients], Streptococcus spp. [28 patients], Enterococci [27 patients], Candida [13 patients] and anaerobes [12 patients]. Resistance to third-generation cephalosporins, penicillins with beta-lactamase inhibitors and quinolones were observed in 51, 36 and 35 patients, respectively. Seven patients had multiple-drug-resistant bacteria. Thirty patients received inadequate empirical treatment, and this was more frequent in patients receiving steroids or immunosuppression [37%] than in patients without immunosuppression [10%: p = 0.001] and was associated with a longer hospital stay [21 days vs 13 days, p = 0.003]. Conclusion: Based on antimicrobial resistance profiles, we herein report a high rate of inadequate empirical first-line therapy for IAAs in CD, especially in patients receiving immunosuppression, and this is associated with prolonged hospitalization.

Pulmonary neuroendocrine tumors (NETs) are rare tumors with an incidence rate of 0.2–2/100 000 population/year in Western countries (M. E. Caplin et al. Ann Oncol 2015; 26:1604–20). They account for 1–2% of all neoplasms of the lung and constitute one-fourth to one-third of all NETs. Atypical carcinoids are far less common than typical carcinoids and predominantly occur in male smokers aged 50 –70 years. Most pulmonary NETs are asymptomatic due to their peripheral location. Surgical resection is the treatment of choice. Medical management should take hormone-related symptoms into account.

Inflammatory bowel disease has been associated with neurological symptoms including restless legs syndrome. Here, we investigated the impact of restless legs syndrome in patients with inflammatory bowel disease on sleep, fatigue, mood, cognition, and quality of life. Two groups of inflammatory bowel disease patients, with and without restless legs syndrome, were prospectively evaluated for sleep disorders, fatigue, daytime sleepiness, depression, anxiety, and health-related quality of life. Furthermore, global cognitive function, executive function, attention, and concentration were assessed in both groups. Disease activity and duration of inflammatory bowel disease as well as current medication were assessed by interview. Inflammatory bowel disease patients with and without restless legs syndrome were matched for age, education, severity, and duration of their inflammatory bowel disease. Patients with inflammatory bowel disease and clinically relevant restless leg syndrome suffered significantly more frequent from sleep disturbances including sleep latency and duration, more fatigue, and worse health-related quality of life as compared to inflammatory bowel disease patients without restless legs syndrome. Affect and cognitive function including cognitive flexibility, attention, and concentration showed no significant differences among groups, indicating to be not related to restless legs syndrome. Sleep disorders including longer sleep latency, shorter sleep duration, and fatigue are characteristic symptoms of restless legs syndrome in inflammatory bowel disease patients, resulting in worse health-related quality of life. Therefore, clinicians treating patients with inflammatory bowel disease should be alert for restless legs syndrome.

Die Therapie chronisch entzundlicher Darmerkrankungen richtete sich bislang vorwiegend nach der Klinik: Bei fehlendem Ansprechen wurde die Therapie eskaliert, bis eine klinische Remission erreicht wurde. Die klinische Remission entspricht jedoch nicht einer mukosalen Abheilung. Analog zu den Daten zur rheumatoiden Arthritis zeigen vorliegende Studien, dass eine fruhe effektive Behandlung chronisch entzundlicher Darmerkrankungen strukturelle Veranderungen reduzieren kann. In der vorliegenden Ubersicht werden Arbeiten diskutiert, die die mukosale Abheilung als mogliches neues Therapieziel definieren. Die Identifikation von Risikofaktoren, die einen komplizierten Verlauf anzeigen, stellt einen ersten Schritt in Richtung einer individuellen Therapie chronisch entzundlicher Darmerkrankungen dar. Entsprechend soll auf Therapiestrategien und die dazugehorige Risikoabwagung eingegangen werden.

The recently updated German S 3-guideline regarding the diagnosis and treatment of Crohn's disease incorporates several changes concerning the radiological approach compared to the former guideline. This article focuses on guideline-based radiological imaging techniques for patients with Crohn's disease. The new guideline is also compared to former European and German guidelines in the context of recently published radiological literature.

The German clinical practice guideline on diagnosis and therapy of Crohn's disease is the result of an evidence-based consensus conference under the auspices of the German Gastroenterologic Society and the Competence Network IBD. This article will summarize the recommendations most important for the general practitioner.Crohn's disease is diagnosed in cooperation with a gastroenterologist who is performing endoscopy and possibly ultrasound. Uncomplicated relapses can nevertheless be successfully treated at the office of a family physician - mostly with steroids. Steroids are not appropriate for long-term treatment though. In those cases an early treatment with immunosuppressants in collaboration with a gastroenterologist is required. Cooperation with several different sub specialists is necessary when surgery is required as well as for the treatment of fistula, psychosomatic aspects and extraintestinal manifestations.

Der Morbus Crohn gehort zu den haufigsten chronisch entzundlichen Erkrankungen des Gastrointestinaltrakts. Leitsymptome sind Durchfall, Bauchschmerzen und systemische Beschwerden wie Fieber und Gewich

The German clinical practice guideline on diagnosis and therapy of Crohn's disease is the result of an evidence-based consensus conference under the auspices of the German Gastroenterologic Society and the Competence Network IBD. This article will summarize the recommendations most important for the general practitioner.Crohn's disease is diagnosed in cooperation with a gastroenterologist who is performing endoscopy and possibly ultrasound. Uncomplicated relapses can nevertheless be successfully treated at the office of a family physician - mostly with steroids. Steroids are not appropriate for long-term treatment though. In those cases an early treatment with immunosuppressants in collaboration with a gastroenterologist is required. Cooperation with several different sub specialists is necessary when surgery is required as well as for the treatment of fistula, psychosomatic aspects and extraintestinal manifestations.

Erythrodermic psoriasis is a severe manifestation of psoriasis and can be triggered by several factors.A 35-year-old man was admitted with severe, almost generalized exfoliative, oozing erythrodermic psoriasis, fever, and cramping of hands and legs. He was under systemic treatment with acitretin. Laboratory examination revealed a marked hypocalcemia as a consequence of primary hypopara thyroidism as well as hypalbuminemia. After normalization of the serum calcium and albumin levels, cutaneous symptoms and fever rapidly improved. No infectious etiology could be found. Hypoparathyroidism together with a right-sided aorta was caused by a 22q11 deletion syndrome.Epidermal cell proliferation and formation of intercellular junctional components of the epidermis are strongly calcium-dependent. Furthermore, carriers like albumin are necessary for the transportation of acitretin in the peripheral tissue. This case report suggests that calcium can be involved in psoriasis pathogenesis at least in a subgroup of patients and that systemic retinoids exhibit insufficient effectiveness under low serum albumin levels.

to follow-up, ADA-treated patients had significant reductions whereas IMS-treated patients had increases in UC-related hospitalizations and LOS (table). UC-related outpatient service utilization and costs, as well as total inpatient and outpatient service costs, decreased significantly for ADA-treated patients vs. a small increase for IMS-treated patients. ADA-treated patients also had a significantly greater reduction in hospital LOS for colectomy. Conclusions: In this retrospective claims database analysis, ADA therapy was associated with greater reductions in UCrelated hospitalization rates, length of hospitalization for colectomy, and total inpatient and outpatient medical service costs compared with IMS therapy.