Narrow band imaging evaluation of duodenal villi in patients with and without celiac disease: A prospective study.

BACKGROUND: Duodenal biopsies are commonly obtained during esophagogastroduodenoscopy (EGD) but are very often histopathologically normal. Therefore, a more strategic method for evaluating the duodenal mucosa and avoiding unnecessary biopsies is needed. AIM: To examine the clinical utility of narrow band imaging (NBI) for evaluating duodenal villous morphology. METHODS: We performed a prospective cohort study of adult patients at Mayo Clinic Rochester from 2013-2014 who were referred for EGD with duodenal biopsies. A staff endoscopist scored, in real-time, the NBI-based appearance of duodenal villi into one of three categories (normal, partial villous atrophy, or complete villous atrophy), captured ≥ 2 representative duodenal NBI images, and obtained mucosal biopsies therein. Images were then scored by an advanced endoscopist and gastroenterology fellow, and biopsies (gold standard) by a pathologist, in a masked fashion using the same three-category classification. Performing endoscopist, advanced endoscopist, and fellow NBI scores were compared to histopathology to calculate performance characteristics [sensitivity, specificity, positive and negative, negative predictive value (NPV), and accuracy]. Inter-rater agreement was assessed with Cohen's kappa. RESULTS: 112 patients were included. The most common referring indications were dyspepsia (47%), nausea (23%), and suspected celiac disease (14%). Duodenal histopathology scores were: 84% normal, 11% partial atrophy, and 5% complete atrophy. Performing endoscopist NBI scores were 79% normal, 14% partial atrophy, and 6% complete atrophy compared to 91%, 5%, and 4% and 70%, 24%, and 6% for advanced endoscopist and fellow, respectively. NBI performed favorably for all raters, with a notably high (92%-100%) NPV. NBI score agreement was best between performing endoscopist and fellow (κ = 0.65). CONCLUSION: NBI facilitates accurate, non-invasive evaluation of duodenal villi. Its high NPV renders it especially useful for foregoing biopsies of histopathologically normal duodenal mucosa.

Defect studies on as-synthesized and purified carbon nanostructures produced by arc-discharge in solution process.

Carbon nanostructures are synthesized using a novel arc-discharge in solution process. A multitude of defects on nanotubes and nanostructures is found. Evidence of these defects in as-synthesized carbon nanostructures is explored using high-resolution transmission electron microscopy (HRTEM). Tri-, tetra-, penta-, hexa-, heptagonal, toroidal, oval, and spherical nanoshells are found in HRTEM investigation along with carbon nanotubes, carbon nanohorns, carbon rods, nanoporous carbon, dislodged graphene sheets, and amorphous carbon. Purifications are carried out through two oxidation methods to eliminate the amorphous carbon. Several different defects caused by oxidations are also found in purified samples.

Epidemiologic and clinical characteristics of children with newly diagnosed inflammatory bowel disease in Wisconsin: a statewide population-based study.

OBJECTIVE: To define epidemiologic and clinical characteristics of newly diagnosed pediatric inflammatory bowel disease (IBD) in a large population-based model. STUDY DESIGN: All pediatric gastroenterologists providing care for Wisconsin children voluntarily identified all new cases of IBD during a 2-year period. Demographic and clinical data were sent to a central registry prospectively for analysis. RESULTS: The incidence of IBD in Wisconsin children was 7.05 per 100,000, whereas the incidence for Crohn's disease was 4.56, more than twice the rate of ulcerative colitis (2.14). An equal IBD incidence occurred among all ethnic groups, and children from sparsely and densely populated counties were equally affected. The majority (89%) of new IBD diagnoses were nonfamilial. CONCLUSIONS: This study provides novel, prospective, and comprehensive information on pediatric IBD incidence within the United States. The surprisingly high incidence of pediatric IBD, the predominance of Crohn's disease over ulcerative colitis, the low frequency of patients with a family history, the equal distribution of IBD among all racial and ethnic groups, and the lack of a modulatory effect of urbanization on IBD incidence collectively suggest that the clinical spectrum of IBD is still evolving and point to environmental factors contributing to the pathogenesis.

Genetic disorders of the pancreas.

The venues opened to all by the remarkable studies of the genome are just starting to become manifest; they can now distinguish different variants of a disease; they are given the tools to better understand the pathophysiology of illness; they hope to be able to provide better treatment alternatives to our patients. The examples described in this review demonstrate the applicability of these concepts to pancreatic disorders. Researchers may be just scratching the surface at this time, but the potential is enormous. Many philosophic and ethical questions need to be answered as physicians move along: Should all family members of an index case be screened? Who should pay for testing? Who should get results? But, without the participation of so many patients, their family members, and numerous volunteers, researchers would not have witnessed the bridging of so many gaps as they have so far. All of us may now look forward to the application of this incredible knowledge to the therapeutic solutions so eagerly awaited.

Topical corticosteroid treatment of dysphagia due to eosinophilic esophagitis in adults.

OBJECTIVE: To describe the clinical features of patients with eosinophilic esophagitis and the use of topical corticosteroids for treatment. PATIENTS AND METHODS: We evaluated the charts of 21 patients with a diagnosis of eosinophilic esophagitis seen at the Mayo Clinic in Rochester, Minn, between September 1, 1999, and December 31, 2001. The diagnosis was based on the presence of a "ringed esophagus" or a tapered distal esophagus on upper endoscopy as well as the presence of a dense eosinophilic infiltrate on esophageal biopsy. All patients were treated with topical corticosteroids, and follow-up was performed by telephone interviews. RESULTS: The 15 men and 6 women ranged in age from 27 years to 66 years at diagnosis (mean, 36 [corrected] years). All had solid-food dysphagia for at least 6 years, and 15 patients had prior food impaction. Eighteen patients had either a ringed-appearing esophagus or smooth tapering in the distal esophagus. All patients had a dense eosinophilic infiltration (> 20 eosinophils per high-power field) in the mid or distal esophagus. Topical corticosteroid therapy for 6 weeks resulted in complete dysphagia relief in all patients and lasted a minimum of 4 months. CONCLUSION: Eosinophilic esophagitis is an entity associated with food dysphagia (predominantly in young adults) and a ringed or smooth tapered distal-appearing esophagus. Our study found no association with gastroesophageal reflux symptoms. Topical corticosteroid therapy was effective.

Primary sclerosing cholangitis in children: a long-term follow-up study.

Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long-term prognosis remains uncertain. The aim of this longitudinal, cohort study was to determine the long-term outcome of children with PSC. Fifty-two children with cholangiography-proven PSC (34 boys and 18 girls; mean age 13.8 +/- 4.2 years; range, 1.5-19.6 years) who were seen at our institution over a 20-year period were followed-up for up to 16.7 years. Two thirds presented with symptoms and/or signs of PSC and 81% had concomitant inflammatory bowel disease (IBD). Twenty-five percent had total alkaline phosphatase activity within the normal range for the age group, but all of them had elevated gamma-glutamyl transpeptidase levels. Autoimmune hepatitis overlapping with PSC was present in 35% of children. A positive but transient clinical and/or biochemical response occurred under therapy with ursodeoxycholic acid, alone or in combination with immunosuppressive medications. During follow-up, 11 children underwent liver transplantation for end-stage PSC and 1 child died. The median (50%) survival free of liver transplantation was 12.7 years. Compared with an age- and gender-matched U.S. population, survival was significantly shorter in children with PSC (P <.001). In a Cox regression model, lower platelet count, splenomegaly, and older age were associated with shorter survival. Presence of autoimmune hepatitis overlapping with PSC (P =.2) or medical therapy (P =.2) did not affect survival. In conclusion, PSC significantly decreases survival in this child population. Although pharmacologic therapy may improve symptoms and liver test results initially, it does not seem to impact the long-term outcome.

Prevalence and clinical significance of human herpesviruses 6 and 7 active infection in pediatric liver transplant patients.

Recent studies in adult liver transplant patients have suggested that both human herpesvirus (HHV)-6 and HHV-7 infection are important causes of morbidity following liver transplantation. However, the impact of HHV-6 and -7 infection in pediatric liver transplant patients remains largely unknown. The aims were to determine the prevalence of HHV-6 and -7 infection in pediatric liver transplant patients and to determine whether there is an association between HHV-6 and -7 infection with episodes of graft rejection and cytomegalovirus (CMV) infection. A total of 46 pediatric liver transplant patients transplanted at Mayo Clinic between January 1994 and January 2000 were evaluated. Quantitative polymerase chain reaction (PCR) assays for CMV, HHV-6 and HHV-7 were performed on stored sera obtained prior to transplant, weekly for 8 wk and at 4 months and 1 yr post-transplant. Pretransplant sera were tested for HHV-6 antibodies by indirect immunofluorescence assay. A total of 215 blood samples were tested (mean 6.5 +/- 3.1, range 3-18). CMV infection occurred in 11 of 33 (33.3%) patients, while CMV disease occurred in 4 of 33 (12%) patients. Infection with HHV-6 (variant B) was detected in three of 33 (9.1%) patients. HHV-7 infection was not detected. Case 1 and 2 were infants (10- and 11-month old, respectively). Both were seronegative for HHV-6 pretransplant. In both cases, HHV-6 infection was associated with concurrent episodes of moderate to severe acute graft rejection. Case 3 was a 16-yr-old girl who was seropositive for HHV-6 pretransplant. No clinical events were recorded and a liver biopsy performed per protocol showed no evidence of rejection. None of the three patients had concomitant CMV infection or disease. In this study, HHV-6 infection occurred in 9% of pediatric liver transplant patients while HHV-7 was not detected. A potential association between primary HHV-6 infection and allograft rejection warrants further investigation.

Rumination syndrome in children and adolescents: diagnosis, treatment, and prognosis.

OBJECTIVES: To characterize the clinical features, results of diagnostic testing, and treatment outcomes for children and adolescents with rumination syndrome. METHODS: Review of the medical records for all 147 patients ages 5 to 20 diagnosed with rumination syndrome at our institution between 1975 and 2000. Data are presented as mean +/- the standard error of the mean. RESULTS: Sixty-eight percent were female. Age at diagnosis was 15.0 +/- 0.3 years. Symptom duration before diagnosis was 2.2 +/- 0.3 years, 73% missed school/work, and 46% had been hospitalized because of symptoms. Before diagnosis, 16 (11%) underwent surgery for evaluation or management of symptoms. Twenty-four (16%) had psychiatric disorders; 3.4% had anorexia or bulimia nervosa. All patients described postprandial regurgitation after almost every meal (2.7 +/- 0.1 meals per day). Weight loss was described by 42.2% (median: 7 kg). Additional symptoms included: abdominal pain, 38%; constipation, 21%; nausea, 17%; and diarrhea, 8%. Structural studies were normal. Gastric emptying of solids at 4 hours was delayed in 26 of 56 patients. Esophageal pH testing in 24 patients showed reflux/regurgitation in 54%. Gastroduodenal manometry in 65 patients showed characteristic rumination-waves in 40%. Outcome data (at median follow-up 10 months) were available for 54 patients. Symptoms resolved in 16 (30%) and improved in 30 (56%). CONCLUSIONS: Recognition of the clinical features of rumination syndrome in children and adolescents is essential; the diagnosis is often delayed and associated with morbidity. Extensive diagnostic testing is unnecessary. Early behavioral therapy is advocated, and patient outcomes are generally favorable.

Pouchitis in Children: Therapeutic Options.

Pouchitis is an unfortunate common complication of the ileal pouch-anal anastomosis procedure, an otherwise very attractive surgical option in patients with ulcerative colitis (UC). The fact that the same pouch in familial polyposis is hardly complicated by pouchitis suggests that the basic inflammatory process in UC might participate in the pathophysiology. We review the clinical features of pouchitis, the diagnostic approach, and the many therapeutic considerations, including more recent data on prebiotics and probiotics.