Atypical Coeliac Disease Found with Serologic Screening

Eighteen patients with coeliac disease were found by screening for reticulin antibodies of unselected sera at the time when determination of various tissue antibodies was requested. Joint disease, allergic and pulmonary disorders, and diabetes were particularly observed. IgA class reticulin antibody, in particular, proved to be specific for coeliac disease. Most patients with coeliac disease also had positive serum gliadin antibodies. Abdominal symptoms and signs of malabsorption were slight and infrequent. In most patients a gluten-free diet resulted in the improvement of jenunal mucosal histology, and serum reticulum and gliadin antibody titres decreased simultaneously, reflecting the appropriateness of the diet. Coeliac disease often has mild and atypical symptoms, and, particularly in certain disease groups, screening with reticulin antibody test seems to be appropriate.     

Antiendomysium versus Antigliadin Antibodies in Screening the General Population for Coeliac Disease

Background: It has recently been shown that mass screening for coeliac disease, using either the serum antigliadin (AGA) or antiendomysium antibodies (EMA) as screening test, can detect large numbers of cases that had escaped clinical diagnosis. The influence of the diagnostic algorithm on the results of the coeliac screening has not yet been evaluated. Our aim was to compare the validity of the AGA and the EMA protocols in 2096 students living in northwest Sardinia, who took part in a serologic screening for coeliac disease. Methods: The sample included 2096 of 2345 eligible students (89%) aged 11-15 years who underwent serum IgG AGA, IgA AGA, and IgA EMA determinations. Total serum IgA level was measured in sera showing isolated IgG AGA positivity. Subjects showing at least one of the following: a) EMA positivity, b) IgA AGA positivity, or c) IgG AGA positivity and IgA deficiency (<5 mg/dl) were asked to submit to a small-intestinal biopsy. Results: The prevalence of coeliac disease was 19 (16 showing typical enteropathy, 1 potential case, and 2 known cases) of 2096 (0.91%; 95% confidence interval = 0.50-1.31). Seventeen small-intestinal biopsy specimens were needed to confirm 16 cases of manifest coeliac disease (positive predictive value (PPV) = 94%) by the EMA protocol, whereas the AGA protocol required 21 biopsy specimens for 12 cases of coeliac disease (PPV = 57%). None of six IgA-deficient, IgG AGA-positive cases detected by the AGA protocol also had coeliac disease. Conclusions: The EMA protocol is superior to the AGA protocol for mass screening of coeliac disease because of higher sensitivity, decreased need for intestinal biopsy, and possibility to detect potential cases of coeliac disease.     

Toward enteral nutrition in the treatment of pediatric Crohn disease in Canada: A workshop to identify barriers and enablers

The treatment armamentarium in pediatric Crohn disease (CD) is very similar to adult-onset CD with the notable exception of the use of exclusive enteral nutrition (EEN [the administration of a liquid formula diet while excluding normal diet]), which is used more frequently by pediatric gastroenterologists to induce remission. In pediatric CD, EEN is now recommended by the pediatric committee of the European Crohn’s and Colitis Organisation and the European Society for Paediatric Gastroenterology Hepatology and Nutrition as a first-choice agent to induce remission, with remission rates in pediatric studies consistently >75%. To chart and address enablers and barriers of use of EEN in Canada, a workshop was held in September 2014 in Toronto (Ontario), inviting pediatric gastroenterologists, nurses and dietitians from most Canadian pediatric IBD centres as well as international faculty from the United States and Europe with particular research and clinical expertise in the dietary management of pediatric CD. Workshop participants ranked the exclusivity of enteral nutrition; the health care resources; and cost implications as the top three barriers to its use. Conversely, key enablers mentioned included: standardization and sharing of protocols for use of enteral nutrition; ensuring sufficient dietetic resources; and reducing the cost of EEN to the family (including advocacy for reimbursement by provincial ministries of health and private insurance companies). Herein, the authors report on the discussions during this workshop and list strategies to enhance the use of EEN as a treatment option in the treatment of pediatric CD in Canada.     

On the Prevalence of Adult Coeliac Disease in Sweden

The occurrence of coeliac disease has been less recognized among adults than in children. We have examined the prevalence of adult coeliac disease in a defined population in Sweden, on the basis of results obtained during 7 years' gastroenterological routine practice. On 1 July 1979, the overall prevalence rate was found to be 58/100, 000 (1:1700). The age-adjusted rates were unevenly distributed. The peak rate, 130/100, 000 (1:750), appearing in the age interval of 45–54 years, indicates that unrecognized middle-aged coeliacs are not symptom-free and that they can be found in routine practice. Our peak rate of coeliac disease unaccompanied by dermatitis herpetiformis is in the same range as the rate found in Swedish children.     

Coeliac Disease in Spondyloarthropathy: Usefulness of Serological Screening

The aim of our study was to find unidentified or neglected cases of coeliac disease by using serological screening in a group of patients with spondyloarthropathies. Altogether, 74 consecutively hospitalised patients (28 females, 46 males, from 15 to 72 years of age, mean age 40.4 ± 1.6 years) with spondyloarthropathies were investigated by serological screening tests for coeliac disease. IgA- and IgG-type antigliadin antibodies were determined using an enzyme-linked immunosorbent assay, and IgA- and IgG-type antireticulin and IgA-type antiendomysium antibodies were measured by an indirect immunofluorescence method. An increased level of antigliadin antibodies was found in nine (12%) of the studied patients and in one of them, antiendomysium antibodies were revealed. In this HLA B8-positive patient, typical villous atrophy with crypt hyperplasia was found in the small bowel biopsy specimen, which confirmed the diagnosis of coeliac disease. None of the patients had IgA- and IgG-type antireticulin antibodies. We found an association of spondyloarthropathy with coeliac disease in one patient out of 74. Clinicians need to be aware of this association, which has important implications for the correct management of patients. Received: 18 March 1999 / Accepted: 25 August 1999     

Preventive effect of nutritional therapy against postoperative recurrence of Crohn disease,with reference to findings determined by intra-operative enteroscopy

Objective. The aim of this study was to elucidate the predictive value of intra-operative enteroscopy (IOE) and the effect of enteral nutrition (EN) with regard to the postoperative recurrence of Crohn disease (CD). Material and methods. Forty patients requiring surgery for severe intestinal complications of CD were examined by IOE, and the severity of the remnant small intestine was determined. Patients were subclassified into either an EN group (>1,200?kcal/day) or a non-EN group (<1,200?kcal/day) according to the amount of daily EN intake after surgery. Contributions of IOE findings and EN to postoperative recurrence were analysed retrospectively. Results. IOE identified intestinal lesions in 39 patients and active intestinal lesions in 24 patients. The cumulative rate of postoperative recurrence was significantly higher in patients with cobblestone appearance confirmed by IOE (p=0.006). However, other active intestinal lesions were not related to postoperative recurrence. EN reduced the cumulative rate of postoperative recurrence (p=0.017), especially in patients with penetrating type (p=0.005), in patients who did not have colitis (p=0.051) and in patients who did not have active intestinal lesions confirmed by IOE (p=0.02). Conclusions. EN is a prophylactic that prevents the postoperative recurrence of small intestinal CD. Patients with the penetrating type of CD, and those who do not have active lesions in the small intestine according to IOE, are candidates for EN after surgery.     

Prevalence of Coeliac disease in the general population of northern Spain. Strategies of serologic screening

BACKGROUND: Coeliac disease (CD) is common in Western countries. In Spain, however, no studies exist on its prevalence. The best method for serologic screening is also unknown. METHODS: We determined the IgA and IgG antigliadin antibodies (AGA) and IgA antiendomysium antibodies (AEA) in a sample of 1170 randomly selected subjects from a health area in northern Spain. The prevalence of CD was calculated with a two-step serologic screening method (first IgA and IgG AGA and then AEA in those positive for AGA) or with only one step with AEA determination. Diagnosis was confirmed by small-intestinal biopsy. RESULTS: When using a two-step strategy, we found 174 (15%) subjects with AGA. Only one of these was confirmed by means of AEA (prevalence of CD = 0.9/1000). Two subjects were found to be positive when a one-step strategy was used (prevalence of CD = 1.7/1000). This yielded an economic saving of 19% with regard to the use of a two-step strategy. One new case of CD in a seronegative subject was diagnosed during the study (real prevalence of CD = 2.6/1000; 95% confidence interval = 0.7-8.2). CONCLUSIONS: The prevalence of CD in Northern Spain is 2.6/1000 (1:389). One-step serologic screening (AEA) is both more economical and more sensitive than the two-step procedure (first AGA and then AEA if AGA is positive).     

Oral nutritional supplementation is effective in the maintenance of remission in Crohn''s disease

Background. Enteral feeding with either elemental or polymeric diets is an established primary therapy for active Crohn's disease. However, the role of supplementing normal food with elemental diet in the long-term management of Crohn's disease has hitherto not been explored.

Patients and methods. A series of 39 consecutive patients with Crohn's disease in clinical remission were studied. Of these, 21 patients (Group 1) received oral nutritional supplementation, taken in addition to their normal diet. Their outcome (relapse rate, Crohn's disease activity index, inflammatory markers) was compared with that of 18 patients (Group 2), who were maintained on a normal unrestricted diet over an observation period of 12 months.

Results. A total of 17 patients (81%) tolerated the nutritional supplementation. On an intention-to-treat basis, 10 patients (48%) remained in remission for 12 months, compared to 4/18 (22%) patients in Group 2, p<0.0003. Their Crohn's disease activity index and CRP remained stable while their weight and body mass index improved during the period of nutrition therapy. Seven patients in Group 1 and 14 in Group 2 relapsed at a mean of 7.4±0.9 and 6.2±0.4 months, respectively. The response to nutrition supplement was independent of age, sex, disease duration or location. Four patients (1945) were intolerant to enteral feeding.

Conclusions. Nutritional supplementation is safe, well tolerated and effective in the long-term management of patients with quiescent Crohn's disease.     

Having a Partner and Having Children: Comparisons of Adults with Congenital Heart Disease and the General Population: A 15-Year Case-Control Study

Objectives: To examine whether patients with congenital heart disease (CHD) are less likely to have a partner or children than individuals from the general population. Methods: Longitudinal study with two assessments of the same patients (n = 244) from a hospital population and controls (n = 238) from the German Socio-Economic Panel (GSOEP) using parental education, patients age, and sex as matching criteria. The first patient study was conducted between 5/2003 and 6/2004, the second one between 5/2017 and 4/2019. Controls were drawn from GSOEP-surveys 2004 and 2018. CHD-severity was classified according to type of surgery: curative, reparative, or palliative. Living single was used as outcome measure, for offspring the outcome was having children or not. Results: Among women with CHD the rate of those living single was higher than among controls with the differences depending on disease complexity (curative: OR = 5.5; reparative: OR = 1.9; palliative: OR = 2.7). No statistically significant differences between patients and controls emerged in the male study population. With respect to children a marked difference emerged between women with CHD and controls. Among patients the odds of having children were lower than among controls (curative: OR = 0.3; reparative: OR = 0.3; palliative: OR = 0.2). The rate of patients with children with CHD (women: 5.6%; men: 4.9%) was higher than expected (1%) if compared with the general population. Conclusions: Using partnership and children as outcome criteria, patients with CHD are disadvantaged if compared to subjects from the general population. In female patients the social consequences of the disease turned out as more pervasive than in women.     

Clinical Features and Long-term Prognosis of Crohn’s Disease in Korea: Results from the Prospective CONNECT Study

Background/AimsThe prospective Crohn’s Disease Clinical Network and Cohort Study is a nationwide multicenter cohort study of patients with Crohn’s disease (CD) in Korea, aiming to prospectively investigate the clinical features and long-term prognosis associated with CD.MethodsPatients diagnosed with CD between January 2009 and September 2019 were prospectively enrolled. They were divided into two cohorts according to the year of diagnosis cohort 1 (diagnosed between 2009 and 2011) versus cohort 2 (between 2012 and 2019).ResultsA total of 1,175 patients were included, and the median follow-up duration was 68 months (interquartile range, 39.0 to 91.0 months). The treatment-free durations for thiopurines (p<0.001) and anti-tumor necrosis factor agents (p=0.018) of cohort 2 were shorter than those of cohort 1. Among 887 patients with B1 behavior at diagnosis, 149 patients (16.8%) progressed to either B2 or B3 behavior during follow-up. Early use of thiopurine was associated with a reduced risk of behavioral progression (adjusted hazard ratio [aHR], 0.69; 95% confidence interval [CI], 0.50 to 0.90), and family history of inflammatory bowel disease was associated with an increased risk of behavioral progression (aHR, 2.29; 95% CI, 1.16 to 4.50). One hundred forty-one patients (12.0%) underwent intestinal resection, and the intestinal resection-free survival time was significantly longer in cohort 2 than in cohort 1 (p=0.003). The early use of thiopurines (aHR, 0.35; 95% CI, 0.23 to 0.51) was independently associated with a reduced risk of intestinal resection.ConclusionsThe prognosis of CD in Korea appears to have improved over time, as evidenced by the decreasing intestinal resection rate. Early use of thiopurines was associated with an improved prognosis represented by a reduced risk of intestinal resection.     

Incidence and Prevalence of Children's Diffuse Lung Disease in Spain

BackgroundChildren's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain.MethodsMulticentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019.ResultsA total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28–10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81–51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2–18 years of age compared with children 0–2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47).ConclusionsWe found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.     

A Case of Acute Myocarditis as the Initial Presentation of Crohn's Disease

We experienced a case of acute myocarditis as the initial presentation of Crohn''s disease. A 19-year-old woman was admitted with impaired consciousness, shock, and respiratory failure. She had suffered from frequent diarrhea and abdominal pain for several years. Cardiac troponin I and creatine kinase-MB fraction levels were elevated to 5.32 and 16.66 ng/mL, respectively. A 12-lead electrocardiogram showed sinus tachycardia, and a chest radiograph revealed interstitial pulmonary edema. An echocardiogram showed dilated ventricles with akinesia of the basal to apical inferoseptal, anteroseptal, anterior, and inferior left ventricular walls and severely impaired systolic function. Intensive care with inotropic support was effective, and her clinical condition gradually improved. Two weeks later, a colonoscopy revealed ulceration with stenosis in the terminal ileum and multiple aphthous ulcers in the rectum. A biopsy of the rectum revealed non-caseating granulomatous inflammation. She was diagnosed with Crohn''s disease presenting with acute myocarditis.     

Screening of Tissue Transglutaminase Antibody in Healthy Blood Donors for Celiac Disease Screening in the Turkish Population

Celiac disease (CD) is a disease having the characteristic pathology of the mucosa of the small intestine. The prevalence of CD in the Turkish population has not been investigated previously. The present study was designed to determine the prevalence of CD in healthy blood donors. Serum samples of 2000 healthy blood donors presenting to Hacettepe University Faculty of Medicine Hospital Blood Bank were tested for tissue transglutaminase (tTG) IgA and IgG antibodies with enzyme-linked immunosorbent assay (ELISA; Euroimmune, Germany). The histopathological findings for the cases with positive serology were evaluated. The distribution of sex was 95.7% male, and 4.3% female. The mean age was 33 +/- 9. Among 2000 donors, 23 (1.15%) were positive for tTG IgA antibody and 3 (0.15%) were positive for tTG IgG antibody. None of the samples was positive for both antibodies. Serum total IgA was measured in two cases with only tTG IgG positivity and was found to be low in one case. Twelve subjects positive for tTG agreed to endoscopy and biopsy. Histopathological examination revealed changes classified as Marsh III-II in one, Marsh II in two, Marsh I in seven, and Marsh 0 in two donors. This was the first study conducted to determine the prevalence of tTG positivity in the Turkish population. The tTG antibody positivity prevalence in healthy blood donors was as high as 1.3%. This study shows that the prevalence of CD in the Turkish population is relatively high in comparison to that in the Western world.     

The role of dietary factors in inflammatory bowel diseases: New perspectives

The current review aimed to elucidate the role of diet in every stage of inflammatory bowel diseases, from aspects of prevention, treatment and rehabilitation. Western diet, characterized by overconsumption of refined sugar and saturated fat and low consumption of dietary fiber, may partly be blamed for its pathogenesis. Some immune‐modulated nutrients (fibers, monounsaturated fatty acids, n‐3 polyunsaturated fatty acids and vitamin D) exert their potential beneficial effects on gut microbiota and immune function, resulting in clinical remission and/or preventing relapse. However, data is limited to conclude optimal micronutrient levels and therapeutic implications. Further, diet itself is complex; therefore, it is reasonable to evaluate diet as a whole rather than a single type of food. Some specific dietary patterns are generated for the management of inflammatory bowel diseases with controversial results. Only exclusive enteral nutrition has been widely recommended for pediatric patients with non‐stricturing active Crohn's disease. Self‐monitoring, avoidance of certain types of foods, limited intake of alcohol and smoking, supplementation of minerals and vitamins if deficiency is confirmed, and adherence to the diet enriched in vegetables and fruits and low in animal food and un‐digested fiber during flares are the most common dietary recommendation. Further clinical trials with a high evidence rank are warranted.     

Changes in the Long-term Prognosis of Crohn’s Disease between 1986 and 2015: The Population-Based Songpa-Kangdong Inflammatory Bowel Disease Cohort Study

Background/AimsThe long-term course of Crohn’s disease (CD) has never been evaluated in non-Caucasian population-based cohorts. The aim of the present study was to evaluate the long-term prognosis of Korean CD patients in the well-defined population-based Songpa-Kangdong inflammatory bowel disease cohort.MethodsOutcomes of disease and their predictors were evaluated for 418 patients diagnosed with CD between 1986 and 2015.ResultsDuring a median of 123 months, systemic corticosteroids, thiopurines, and anti-tumor necrosis factor (TNF) agents were administered to 58.6%, 81.3%, and 37.1% of patients, respectively. Over time, the cumulative probability of starting corticosteroids significantly decreased (p=0.001), whereas that of starting thiopurines and anti-TNFs significantly increased (both p<0.001). The cumulative probability of behavioral progression was 54.5% at 20 years, and it significantly decreased during the anti-TNF era. Intestinal resection was required for 113 patients (27.0%). The cumulative probabilities of intestinal resection at 1, 5, 10, 20, and 25 years after CD diagnosis were 12.7%, 16.5%, 23.8%, 45.1%, and 51.2%, respectively. Multivariable Cox regression analysis identified stricturing behavior at diagnosis (adjusted hazard ratio [aHR], 2.70; 95% confidence interval [CI], 1.55 to 4.71), penetrating behavior at diagnosis (aHR, 11.15; 95% CI, 6.91 to 17.97), and diagnosis of CD during the anti-TNF era (aHR, 0.51; 95% CI, 0.35 to 0.76) as independently associated with intestinal resection. The standardized mortality ratio among CD patients was 1.36 (95% CI, 0.59 to 2.68).ConclusionsThe long-term prognosis of Korean patients with CD is at least as good as that of Western CD patients, as indicated by the low intestinal resection rate. Moreover, behavioral progression and intestinal resection rates have decreased over the past 3 decades.