OBJECTIVE. The goal was to determine how often common laboratory tests yield normal results at the time of diagnosis for children with inflammatory bowel disease. METHODS. Data were obtained from a registry of children with newly diagnosed inflammatory bowel disease who were enrolled prospectively in 18 US/Canadian centers. Laboratory values investigated included hemoglobin level, platelet count, albumin level, and erythrocyte sedimentation rate. Disease severity was categorized by physician global assessment. RESULTS. A total of 526 children (mean age: 11.6 years; 58% male; 392 with Crohn disease and 134 with ulcerative colitis) were studied. All 4 values were normal for 21% of patients with mild Crohn disease and 54% with mild ulcerative colitis. In contrast, only 3.8% of children with moderate/severe Crohn disease and 4.3% with moderate/severe ulcerative colitis had normal results for all 4 tests. The erythrocyte sedimentation rate was least likely to be normal; overall, 26% of patients with inflammatory bowel disease had a normal erythrocyte sedimentation rate, including 18% with moderate/severe disease. Hemoglobin levels were normal for 32%, platelet counts for 50%, and albumin levels for 60%. There was no clear association between Crohn disease location and either severity or number of normal laboratory values. In contrast, there were direct correlations between ulcerative colitis disease severity and both the extent of bowel inflammation and the number of abnormal laboratory tests. CONCLUSION. The presence of normal screening laboratory studies should not dissuade clinicians from considering a diagnosis of inflammatory bowel disease.

Background and Aims: Assessment of health-related quality of life (HRQOL) is of increasing importance in the evaluation of new therapies for inflammatory bowel disease (IBD). Available data concerning HRQOL in pediatric patients are sparse and uniformly cross-sectional. The aim of this study was to describe HRQOL and influential factors in newly diagnosed pediatric patients with Crohn's disease and ulcerative colitis during the first 12 months after diagnosis.Materials and Methods: Participants were drawn from a large, prospectively derived observational IBD registry of pediatric patients studied through 18 U.S. and Canadian centers. Patients who had completed a baseline IMPACT questionnaire and for whom there were 12 months of follow-up data available were included. In addition to description of cohort, factors that were believed to influence HLQOL were assessed during the course of the year from diagnosis.Results: Two hundred eighteen children met inclusion criteria (77% Crohn's disease, 23% ulcerative colitis, mean age 12.7 +/- 1.9 years). Mean total IMPACT score at baseline was 154, 181 at 6 months, and 191 at 1 year (possible range 0-238, with increasing scores representing better quality of life). Repeated measures analysis showed that age and disease severity significantly negatively affected the IMPACT scores during the course of the year.Conclusions: In this large prospective pediatric IBD cohort, significant improvement in HRQOL is noted during the year from diagnosis. Mean IMPACT scores varied significantly depending on the disease severity and also decreased with increasing age.

BACKGROUND & AIMS:The aim of this study was to describe 3-month and 1-year outcomes of children with Crohn's disease (CD) treated with corticosteroids within 30 days of diagnosis, with particular emphasis on the influence of infliximab on these outcomes. We also aimed to determine whether there are clinical or laboratory characteristics associated with corticosteroid therapy outcomes.METHODS:Data from 109 children were drawn from a multicenter observational registry that was started in 2002. Clinical characteristics and data on corticosteroid and other therapies were recorded prospectively. Corticosteroid therapy outcomes at 3 months were defined as complete acute response, partial response, or corticosteroid resistance. At 1 year, corticosteroid responsiveness, dependence, and surgical rates were determined. Infliximab's influence on short- and long-term outcomes also was investigated.RESULTS:At 3 months, 65 of 109 (60%) patients had a complete acute response to corticosteroids, 26 (24%) had a partial response, and 18 (17%) were corticosteroid resistant. At 1 year, 61% were corticosteroid responsive, 31% were corticosteroid dependent, and 8% required surgery. Irrespective of the duration of corticosteroid treatment, 16 of 24 of corticosteroid-dependent/resistant patients rapidly discontinued corticosteroids after starting infliximab. No clinical or laboratory characteristics at diagnosis predicted short-term outcome. Growth impairment at diagnosis increased risk for corticosteroid dependence or surgery at 1 year.CONCLUSIONS:At 3 months, 84% of children had a complete or partial response to corticosteroids. However, despite concomitant immunomodulators, at 1 year 31% were corticosteroid dependent and 8% required surgery. Infliximab improves outcomes of corticosteroid-dependent/resistant patients because the duration of corticosteroid use can be controlled by initiating treatment with infliximab.

Participants will be able to: • Become familiar with the effect of chronic inflammation on the risk of colorectal cancer in patients with ulcerative colitis; • Become familiar with the effect of 5-aminsalicylate on the survival rates from colorectal cancer in patients with ulcerative colitis; and • Become familiar with the effects of immunosuppression on the risk of cervical dysplasia in patients with IBD.

Journal of Pediatric Gastroenterology and NutritionVolume 43, Issue 4 p. E48-E48 North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Annual Meeting, October 19-22, 2006, Orlando, Florida: Abstracts: POSTER SESSION II, FRIDAY, OCTOBER 20, 2006, 12:15 p.m. - 2:15 p.m.: Intestine/Colon/IBD: 109 DOES HAVING A FAMILY HISTORY OF INFLAMMATORY BOWEL DISEASE AFFECT QUALITY OF LIFE IN NEWLY DIAGNOSED CHILDREN? Abigail Garrity, Abigail Garrity Trinity College, Hartford, CTSearch for more papers by this authorTrudy Lerer, Trudy Lerer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnthony Otley, Anthony Otley Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJames Markowitz, James Markowitz Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnne Griffiths, Anne Griffiths Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Mack, David Mack Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJonathan Evans, Jonathan Evans Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAthos Bousvaros, Athos Bousvaros Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMarian Pfefferkorn, Marian Pfefferkorn Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Rothbaum, Robert Rothbaum Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRosh Joel, Rosh Joel Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorFernando delRosario, Fernando delRosario Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSubra Kugathasan, Subra Kugathasan Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Wyllie, Robert Wyllie Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAdam Mezoff, Adam Mezoff Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSusan Moyer, Susan Moyer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorNeal Leleiko, Neal Leleiko Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJeffrey Hyams, Jeffrey Hyams Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this author Abigail Garrity, Abigail Garrity Trinity College, Hartford, CTSearch for more papers by this authorTrudy Lerer, Trudy Lerer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnthony Otley, Anthony Otley Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJames Markowitz, James Markowitz Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnne Griffiths, Anne Griffiths Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Mack, David Mack Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJonathan Evans, Jonathan Evans Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAthos Bousvaros, Athos Bousvaros Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMarian Pfefferkorn, Marian Pfefferkorn Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Rothbaum, Robert Rothbaum Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRosh Joel, Rosh Joel Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorFernando delRosario, Fernando delRosario Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSubra Kugathasan, Subra Kugathasan Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Wyllie, Robert Wyllie Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAdam Mezoff, Adam Mezoff Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSusan Moyer, Susan Moyer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorNeal Leleiko, Neal Leleiko Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJeffrey Hyams, Jeffrey Hyams Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this author First published: 01 October 2006 https://doi.org/10.1002/j.1536-4801.2006.tb13852.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. 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Journal of Pediatric Gastroenterology and NutritionVolume 43, Issue 4 p. E48-E48 North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Annual Meeting, October 19-22, 2006, Orlando, Florida: Abstracts: POSTER SESSION II, FRIDAY, OCTOBER 20, 2006, 12:15 p.m. - 2:15 p.m.: Intestine/Colon/IBD: 108 DOES AGE AT ONSET AFFECT IBD TREATMENT? James Markowitz, James Markowitz Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJeffrey Hyams, Jeffrey Hyams Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorTrudy Lerer, Trudy Lerer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Mack, David Mack Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJonathan Evans, Jonathan Evans Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJoel Rosh, Joel Rosh Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnne Griffiths, Anne Griffiths Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSubra Kugathasan, Subra Kugathasan Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnthony Otley, Anthony Otley Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMarian Pfefferkorn, Marian Pfefferkorn Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Keljo, David Keljo Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorNeal Leleiko, Neal Leleiko Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAthos Bousvaros, Athos Bousvaros Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorWallace Crandall, Wallace Crandall Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSusan Moyer, Susan Moyer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Rothbaum, Robert Rothbaum Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Wyllie, Robert Wyllie Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMaria Oliva-Hemker, Maria Oliva-Hemker Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJ Fernando DelRosario, J Fernando DelRosario Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAdam Mezoff, Adam Mezoff Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this author James Markowitz, James Markowitz Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJeffrey Hyams, Jeffrey Hyams Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorTrudy Lerer, Trudy Lerer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Mack, David Mack Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJonathan Evans, Jonathan Evans Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJoel Rosh, Joel Rosh Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnne Griffiths, Anne Griffiths Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSubra Kugathasan, Subra Kugathasan Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnthony Otley, Anthony Otley Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMarian Pfefferkorn, Marian Pfefferkorn Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Keljo, David Keljo Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorNeal Leleiko, Neal Leleiko Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAthos Bousvaros, Athos Bousvaros Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorWallace Crandall, Wallace Crandall Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSusan Moyer, Susan Moyer Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Rothbaum, Robert Rothbaum Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Wyllie, Robert Wyllie Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMaria Oliva-Hemker, Maria Oliva-Hemker Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJ Fernando DelRosario, J Fernando DelRosario Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAdam Mezoff, Adam Mezoff Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this author First published: 01 October 2006 https://doi.org/10.1002/j.1536-4801.2006.tb13851.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. 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Journal of Pediatric Gastroenterology and NutritionVolume 43, Issue 4 p. E51-E51 North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Annual Meeting, October 19-22, 2006, Orlando, Florida: Abstracts: POSTER SESSION III, SATURDAY, OCTOBER 21, 2006, 7:45 a.m. - 9:45 a.m.: Intestine/Colon/IBD: 120 PERIANAL FISTULAE IN PEDIATRIC CROHN DISEASE THERAPIES AND COURSE David Keljo, David Keljo The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJames Markowitz, James Markowitz The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorChristine Langton, Christine Langton The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorTrudy Lerer, Trudy Lerer The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSubra Kugathasan, Subra Kugathasan The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnne Griffiths, Anne Griffiths The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnthony Otley, Anthony Otley The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJoel Rosh, Joel Rosh The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMarian Pfefferkorn, Marian Pfefferkorn The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Mack, David Mack The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJonathan Evans, Jonathan Evans The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAthos Bousvaros, Athos Bousvaros The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSusan Moyer, Susan Moyer The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Rothbaum, Robert Rothbaum The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Wyllie, Robert Wyllie The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMaria Oliva-Hemker, Maria Oliva-Hemker The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAdam Mezoff, Adam Mezoff The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorNeal Leleiko, Neal Leleiko The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJ. Fernando Del Rosario, J. Fernando Del Rosario The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorWallace Crandall, Wallace Crandall The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJeffrey Hyams, Jeffrey Hyams The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this author David Keljo, David Keljo The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJames Markowitz, James Markowitz The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorChristine Langton, Christine Langton The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorTrudy Lerer, Trudy Lerer The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSubra Kugathasan, Subra Kugathasan The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnne Griffiths, Anne Griffiths The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAnthony Otley, Anthony Otley The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJoel Rosh, Joel Rosh The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMarian Pfefferkorn, Marian Pfefferkorn The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorDavid Mack, David Mack The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJonathan Evans, Jonathan Evans The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAthos Bousvaros, Athos Bousvaros The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorSusan Moyer, Susan Moyer The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Rothbaum, Robert Rothbaum The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorRobert Wyllie, Robert Wyllie The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorMaria Oliva-Hemker, Maria Oliva-Hemker The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorAdam Mezoff, Adam Mezoff The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorNeal Leleiko, Neal Leleiko The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJ. Fernando Del Rosario, J. Fernando Del Rosario The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorWallace Crandall, Wallace Crandall The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this authorJeffrey Hyams, Jeffrey Hyams The Pediatric IBD Collaborative Research Group, Hartford, CTSearch for more papers by this author First published: 01 October 2006 https://doi.org/10.1002/j.1536-4801.2006.tb13863.xRead the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. 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BACKGROUND & AIMS:The aim of this study was to determine the clinical outcome after corticosteroid therapy in children who are newly diagnosed with ulcerative colitis (UC). METHODS:Data were gathered prospectively from the Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry database between January 2002 and March 2005. All children who were newly diagnosed with inflammatory bowel disease younger than the age of 16 years were managed according to the dictates of their respective physicians. Demographic, clinical, and laboratory data were collected at diagnosis, at 30 days, and then quarterly. Patients were classified as corticosteroid responsive, corticosteroid dependent, or refractory, and outcomes were determined at 3 months and at 1 year. RESULTS:Ninety-seven patients had a diagnosis of UC and a minimum of 1 year of follow-up evaluation; 77 (79%) received corticosteroids (62 within 30 days of diagnosis [early] and 15 between 31 days and 6 months [late]). At diagnosis, 81% of corticosteroid-treated patients (age, 11.3 +/- 3.5 y) had moderate/severe disease, and 81% had pancolitis. For those treated early with corticosteroids, disease activity at 3 months was inactive in 60%, mild in 27%, and moderate/severe in 11%. At 1 year, 31 of 62 (50%) of the early corticosteroid-treated patients were considered corticosteroid responsive and 28 (45%) were corticosteroid dependent. A total of 4 patients receiving corticosteroids (5%) required colectomy in the first year. Immunomodulators were used in 61% of all corticosteroid-treated patients. CONCLUSIONS:Although short-term clinical response to corticosteroids in children with newly diagnosed UC is excellent, even with the common use of immunomodulators corticosteroid dependence is seen in 45% of patients.

Introduction: Several serologic markers including ASCA, anti-OmpC, and pANCA characterize patients with Crohn disease (CD). Aim: To determine whether these markers are found more commonly in different sub-groups of pediatric CD patients and whether they correlate with clinical course. Methods: Data were obtained from the prospective observational registry of the Pediatric IBD Collaborative Research Group. Results: One hundred and forty six patients with CD had complete data available for study. 61 (42%) were ASCA positive, 23 were pANCA positive (16%) and 22 were anti-OmpC positive (15%). Four patients (2.7%) were ASCA/ANCA positive, 14 children (9.6%) were ASCA/OmpC positive, and one child (0.7%) was positive for all three. Children who were ASCA positive were more likely to be >10 years (95.1% versus 78.8%, p = 0.006) and have disease in the terminal ileum (90.2% versus 71.8% p = 0.007). Children who were ASCA negative were more likely to have left sided colonic disease (72.9% versus 27.1%, p = 0.031). Thirteen children were identified with perianal fistulae and none of them were pANCA positive. All children who were anti-OmpC positive while being ASCA negative (n = 7) had CD involving the ascending and transverse colon. 90% of ASCA-/pANCA+ patients had left-sided colon disease, while 25% ASCA+/pANCA+ had left-sided colon disease (p = 0.021). There was no difference in race, gender, disease severity at diagnosis, or response to subsequent therapy. ASCA positivity did not predict the use of infliximab therapy or surgical intervention in the first year post-diagnosis. Conclusions: ASCA positive children with CD are more likely to be >10 years old and to have disease in the terminal ileum, while those ASCA negative are more likely to have left sided colonic disease. The presence or absence of ASCA was not helpful in predicting clinical course.

This report was first conceived during the International Shwachman-Diamond syndrome (SDS) Family Conferences, and further developed by Medical and Scientific participants attending the First International Scientific meeting on SDS. Through a combination of literature review and consultations with specialists with clinical expertise in the management of patients with SDS, we sought to answer several questions regarding SDS. This report is directed to physicians and health care workers interacting with affected individuals and their families. Each is faced with the challenging task of establishing the diagnosis, promoting adequate follow-up, and preventing complications. Shwachman-Diamond syndromeThe Journal of PediatricsVol. 141Issue 2PreviewSee related articles, p 259 and p 266. Full-Text PDF Serum pancreatic enzymes define the pancreatic phenotype in patients with Shwachman-Diamond syndromeThe Journal of PediatricsVol. 141Issue 2PreviewObjective: To evaluate the role of serum enzymes for defining the pancreatic phenotype in Shwachman-Diamond syndrome (SDS), an inherited multisystem condition. Study design: Serum pancreatic trypsinogen and isoamylase were measured in 164 patients known or presumed to have SDS. The diagnosis was confirmed in 90 patients. Among 74 unconfirmed cases, 35 (“probable SDS”) had hematologic dysfunction but lacked documented pancreatic dysfunction, whereas 39 patients (“improbable SDS”) lacked both documented pancreatic and hematologic dysfunction. Full-Text PDF