Ulcerative Colitis: Pfefferkorn M

Turner D, Hyams J, Markowitz J, Lerer T, Mack DR, Evans J, Pfefferkorn M, Rosh J, Kay M, Crandall W, Keljo D, Otley AR, Kugathasan S, Carvalho R, Oliva-Hemker M, Langton C, Mamula P, Bousvaros A, LeLeiko N, Griffiths AM, Anonymous00063. · Pediatric Gastroenterology Unit, Shaare Zedek Medical Center, Jerusalem, Israel. · Inflamm Bowel Dis. · Pubmed #19161178 No free full text.

Abstract: BACKGROUND: We evaluated the psychometric performance of the Pediatric Ulcerative Colitis Activity Index (PUCAI) in a real-life cohort from the Pediatric IBD Collaborative Research Group. METHODS: Two consecutive visits of 215 children with ulcerative colitis (UC) were included (mean age 11.2 +/- 3.6 years; 112 (52%) males; 63 (29%) newly diagnosed and the others after disease duration of 24 +/- 15.6 months). Validity was assessed using several constructs of disease activity. Distributional and anchor-based strategies were used to assess the responsiveness of the PUCAI to change over time following treatment. RESULTS: Reflecting feasibility, 97.6% of 770 eligible registry visits had a completed PUCAI score versus only 47.6% for a contemporaneously collected Pediatric Crohn's Disease Activity Index (odds ratio = 45.8, 95% confidence interval [CI] 28.6-73.5) obtained for children with Crohn's disease accessioned into the same database. The PUCAI score was significantly higher in patients requiring escalation of medical therapy (45 points [interquartile range, IQR, 30-60]) versus those who did not, (0 points [IQR 0-10]; P < 0.001), and was highly correlated with physician's global assessment of disease activity (r = 0.9, P < 0.001). The best cutoff to differentiate remission from active disease was 10 points (area under receiver operating characteristic curve [AUC] 0.94; 95% CI 0.90-0.97). Test-retest reliability was excellent (intraclass correlation coefficient = 0.89; 95% CI 0.84-0.92, P < 0.001) as well as responsiveness to change (AUC 0.96 [0.92-0.99]; standardized response mean 2.66). CONCLUSION: This study on real-life, prospectively obtained data confirms that the PUCAI is highly feasible by virtue of the noninvasiveness, valid, and responsive index. The PUCAI can be used as a primary outcome measure to reflect disease activity in pediatric UC.

Markel TA, Lou DC, Pfefferkorn M, Scherer LR, West K, Rouse T, Engum S, Ladd A, Rescorla FJ, Billmire DF. · Department of Surgery, J. W. Riley Hospital for Children, Indiana University, Indianapolis, Indiana 46202, USA. · Surgery. · Pubmed #18847637 No free full text.

Abstract: BACKGROUND: Risk factors for postoperative infections have not been evaluated in pediatric patients with ulcerative colitis (UC). This review was undertaken to evaluate the effects of immunosuppressive therapy and other preoperative factors on infectious wound complications in children undergoing first stage surgical therapy for UC. METHODS: A 10-year retrospective review of children under 18 years of age receiving first stage surgical therapy for UC at a major children's hospital was performed. Preoperative clinical and treatment variables were identified and correlated with postoperative wound complications. RESULTS: A total of 51 children were identified: 19 underwent colectomy with ileo-anal-pouch anastomosis and 32 underwent total abdominal colectomy with Hartmann's pouch. A total of 20 infectious complications were identified in 18 patients. Preoperative steroid use was associated with a greater postoperative wound infection rate. Preoperative hemoglobin less than 10 g/dL (P < .05) and albumin less than 3 g/dL (P = 0.1) were associated with greater rates of postoperative infection. Preoperative body mass index and other immunosuppressive agents did not influence postoperative infectious morbidity. CONCLUSIONS: The majority of pediatric patients who require operative intervention for UC are debilitated from their disease and medication use. Children with normal serum albumin and hemoglobin who are not on steroid therapy have a low risk of postoperative infectious complications.

Noe JD, Pfefferkorn M. · Indiana University School of Medicine, Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Indianapolis, Indiana, USA. · Inflamm Bowel Dis. · Pubmed #18618629 No free full text.

Abstract: BACKGROUND: Adalimumab is effective for adults with moderate to severe Crohn's disease (CD). Data in children with inflammatory bowel disease (IBD) is sparse. We aim to evaluate pediatric IBD response to adalimumab. METHODS: We conducted a retrospective chart review of pediatric IBD patients treated with adalimumab. We defined response as either a decrease in the disease activity index severity or remission after 3-6 months of treatment. RESULTS: Ten patients were identified. Seven had CD, three had ulcerative colitis (UC). All had colonic disease and disease in either the esophagus, stomach, or duodenum. Eight patients initially responded to infliximab, 5 with CD and 3 with UC; 2 CD patients had incomplete data for evaluation. The mean and median times between initial infliximab and initial adalimumab were 29.5 and 24 months, respectively. Eight patients responded to adalimumab 40 mg or 80 mg biweekly, or 80 mg initial dose followed by 40 mg biweekly. The mean Pediatric Crohn's Disease Activity Index (PCDAI) in CD patients before and after adalimumab were 12 and 4.2, respectively (normal <or=10). The mean Lichtiger Colitis Activity Index (LCAI) in UC patients before and after adalimumab were 9 and 5.1, respectively (normal <10). Corticosteroids were weaned off in 4/7 patients at 1 to 10 months after initiation of adalimumab. One patient with CD and 1 patient with UC failed adalimumab, required surgery, and remained on corticosteroids. CONCLUSIONS: Adalimumab is useful in pediatric IBD patients who become intolerant of infliximab.

Kugathasan S, Nebel J, Skelton JA, Markowitz J, Keljo D, Rosh J, LeLeiko N, Mack D, Griffiths A, Bousvaros A, Evans J, Mezoff A, Moyer S, Oliva-Hemker M, Otley A, Pfefferkorn M, Crandall W, Wyllie R, Hyams J, Anonymous00137, Anonymous00138. · Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI 53226, USA. · J Pediatr. · Pubmed #17961699 No free full text.

Abstract: OBJECTIVE: To conduct a systematic review of children with newly diagnosed inflammatory bowel disease (IBD) from 2 prospective inception cohorts to examine body mass index (BMI) status at presentation. STUDY DESIGN: Clinical, demographic, and BMI data were obtained from 783 patients with newly diagnosed IBD. National Health and Nutrition Examination Survey data for 2748 healthy children were used as a control. RESULTS: Most children with Crohn's disease and ulcerative colitis had a BMI in the normative range (5%-84%). Low BMI (<5%) was seen in 22% to 24% of children with Crohn's disease and 7% to 9% of children with ulcerative colitis. Ten percent of children with Crohn's disease and 20% to 30% of children with ulcerative colitis had a BMI at diagnosis consistent with overweight or risk for overweight. CONCLUSION: Children with IBD are affected by current population trends toward overweight. A significant subgroup of children with newly diagnosed IBD has a BMI categorized as overweight or at risk for overweight. Clinicians should be aware of possible IBD diagnosis in the presence increased BMI.

Mack DR, Langton C, Markowitz J, LeLeiko N, Griffiths A, Bousvaros A, Evans J, Kugathasan S, Otley A, Pfefferkorn M, Rosh J, Mezoff A, Moyer S, Oliva-Hemker M, Rothbaum R, Wyllie R, delRosario JF, Keljo D, Lerer T, Hyams J, Anonymous00371. · Department of Pediatrics, Children's Hospital of Eastern Ontario, 401 Smyth Rd, Ottawa, Ontario, Canada K1H 8L1. · Pediatrics. · Pubmed #17545378 links to  free full text

Abstract: 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.

Otley AR, Griffiths AM, Hale S, Kugathasan S, Pfefferkorn M, Mezoff A, Rosh J, Tolia V, Markowitz J, Mack D, Oliva-Hemker M, Wyllie R, Rothbaum R, Bousvaros A, Del Rosario JF, Evans J, Blanchard W, Hyams J, Anonymous00277. · FRCPC, Division of Gastroenterology & Nutrition, IWK Health Centre, Halifax, Nova Scotia, Canada. · Inflamm Bowel Dis. · Pubmed #16917222 No free full text.

Abstract: 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.

Hyams J, Markowitz J, Lerer T, Griffiths A, Mack D, Bousvaros A, Otley A, Evans J, Pfefferkorn M, Rosh J, Rothbaum R, Kugathasan S, Mezoff A, Wyllie R, Tolia V, delRosario JF, Moyer MS, Oliva-Hemker M, Leleiko N, Anonymous00069. · Connecticut Children's Medical Center, Hartford, Connecticut, USA. · Clin Gastroenterol Hepatol. · Pubmed #16820327 No free full text.

Abstract: 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.