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OBJECTIVES: Social determinants of health (SDOH) are nonmedical factors that influence health outcomes. Children with chronic illnesses living with socioeconomic risk factors have higher rates of unfavorable health outcomes. Our study aimed to assess the association between area deprivation indices (ADI), as a proxy for SDOH, and outcomes in pediatric patients with inflammatory bowel disease (IBD). METHODS: A retrospective chart review was conducted on 134 pediatric patients with IBD, ages 0-21 years. Explanatory variables were the patient's ADI and demographics. Outcomes were assessed from time of diagnosis over a 1-year follow-up period. The primary outcome was the ratio of missed to completed appointments; secondary outcomes were the numbers of IBD-related emergency department (ED) visits and IBD-related hospitalizations. RESULTS: Race/ethnicity was significantly associated with ADI (p < 0.001). In a multivariable regression model, no variables were associated with ratio of missed to completed appointments. Notably, ADI was not significantly associated with the ratio of missed to completed appointments. In a Poisson regression, Black (non-Hispanic) and Hispanic race/ethnicity, Medicaid insurance, female gender, and lower age were significantly associated with more IBD-related ED visits; ADI was not. In a similar model, Black (non-Hispanic) race, Medicaid insurance status, and lower age were significantly associated with more IBD-related hospitalizations; ADI was not. CONCLUSIONS: In our cohort, ADI was not significantly associated with the ratio of missed to completed appointments, IBD-related ED visits, and IBD-related hospitalizations; however, race/ethnicity, age at diagnosis, insurance, and gender were associated with these outcomes.
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OBJECTIVES: The pediatric gastroenterology workforce has grown in the last few decades. The North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) formed a task force to understand current pediatric gastroenterology organizations' practice structures. METHODS: 19-item electronic survey was distributed to NASPGHAN members who were clinical or academic division directors. RESULTS: 30% responded to the survey, all directors of academic practices. The median number of clinical sessions per week was seven sessions, and the median individual work relative value unit (wRVU) target for practices was 4000-4500. Healthcare team ratios compared to provider clinical full-time equivalent were reported as the following: Nursing 0.80, medical assistant (MA) 0.29, dietitian 0.29, social worker 0.14, and psychologist 0.13. Regarding compensation, 68.0% were salaried with bonus based on billing or director decision, 28.0% were salaried with no incentive pay, and 4.0% were salaried with a portion at risk if the target was not met, and a bonus was given if the target was met. Most practices participated in a wellness activity with the most common strategies being didactic lectures about physician burnout (80%), annual burnout check-ins (68%), and/or after-hours social activities (60%). CONCLUSIONS: Pediatric gastroenterology practices vary regarding clinical sessions per week and annual wRVU targets with the median at seven sessions per week and an annual goal of 4000-4500 wRVUs, similar to reported national benchmark goals at the 50th percentile. Healthcare teams, including nursing, MAs, dietitians, social workers, and psychologists, had similar ratios of staff to providers for all sizes and types of practices. Most practices are engaging in wellness initiatives.
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Gastroenterología , Pediatría , Carga de Trabajo , Humanos , Gastroenterología/organización & administración , Pediatría/organización & administración , Encuestas y Cuestionarios , Salarios y Beneficios , Gestión de la Práctica Profesional/organización & administración , Estados Unidos , Médicos/psicología , MasculinoRESUMEN
Inflammatory bowel disease (IBD) pathogenesis is thought to be induced by a mix of genetic susceptibility, microbial populations, and immune triggers such as infections. Severe acute respiratory syndrome coronavirus 2 (SARS-nCoV2) may have increased capacity to generate autoimmune disease as evidenced by known spikes in diseases such as type 1 diabetes mellitus. Public health interventions like masking and closures additionally created remarkable drops in typical viral infections, with remarkable shifts in influenza-like illness reporting in 2020. This study aims to evaluate the impact of SARS-nCoV2 and associated interventions on pediatric IBD presentation in New York City using records of new diagnoses at a consortium of 4 institutions between 2016 and June 2022. We fit time series model (autoregressive integrated moving average model) to monthly and quarterly number of cases of each disease for January 2016-March 2020 and forecast the period between April 2020 and June 2022. We note no decrease in ulcerative colitis (UC) or Crohn disease (CD) in the aftermath of historic low levels of overall viral illness, and statistically significant increases in CD diagnoses and elevation in UC diagnoses creating a trend suggesting overall increase in IBD diagnoses exceeding the baseline rate of increase. These data suggest a possible linkage between SARS-nCoV2 infection rates and subsequent pediatric IBD presentation.
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COVID-19 , Colitis Ulcerosa , Enfermedad de Crohn , Enfermedades Inflamatorias del Intestino , Humanos , Niño , COVID-19/epidemiología , Enfermedades Inflamatorias del Intestino/epidemiología , Enfermedades Inflamatorias del Intestino/diagnóstico , Enfermedad de Crohn/diagnóstico , Colitis Ulcerosa/diagnóstico , Ciudad de Nueva York/epidemiologíaRESUMEN
OBJECTIVES: Physicians are prone to burnout which can negatively affect the quality of patient care and lead to medical errors. Burnout can also affect physicians by impacting their personal relationships, their sense of career fulfillment, and job satisfaction. The North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) formed a taskforce to investigate burnout among pediatric gastroenterologists. METHODS: A 35-item electronic survey was developed to collect demographic and practice information and characterize the well-being of pediatric gastroenterologists. Burnout was assessed employing 2 single-item measures adapted from the Maslach Burnout Inventory. The survey was distributed to NASPGHAN members 3 times from February 2020 to March 2020. Descriptive statistics, Chi-square, and Fisher exact tests were used. RESULTS: One thousand seven hundred ninety-one e-mails were successfully sent and 408 participants (22.7%) returned surveys. A total of 28.8% reported high risk for emotional exhaustion, 17.5% reported high risk for depersonalization, and 33% reported overall burnout. Participants 44 years of age or younger reported significantly more burnout than those 45 years and older ( P = 0.018). Contributors to high burnout identified included increased patient load/demands, insufficient nursing support, electronic health record (EHR) use, insufficient administrative staff, excessive on-call coverage, and more complex patients. Forty-four percent reported not having enough time for their personal life including family. A total of 16.2% of participants reported that they would not choose to be a pediatric gastroenterologist again. CONCLUSIONS: Pediatric gastroenterologists are at risk for emotional exhaustion, depersonalization, and overall burnout. Strategies to prevent physician burnout should be implemented as soon as feasibly possible to improve individual mental health and patient care.
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Agotamiento Profesional , Gastroenterología , Médicos , Niño , Humanos , Persona de Mediana Edad , Médicos/psicología , Agotamiento Psicológico , Agotamiento Profesional/psicología , Encuestas y Cuestionarios , Satisfacción en el TrabajoRESUMEN
ABSTRACT: Women and minorities are underrepresented in leadership positions in academic specialties. We investigated representation of women and minorities in selected leadership positions and in fellowships in North American academic pediatric gastroenterology programs (PGP) via voluntary surveys. We also assessed for factors influencing diversity. We found that 45.8% of Program Directors (PD), 75% of division chiefs (DC), and 71% of Pediatrics department chairs were men. Sixty-three percentage of the PG fellows were women. Most DCs were male professors. Most PDs, DCs, and department chairs were White (70%, 80%, and 88.3%), with Blacks being the least represented group in leadership and also among fellows. We found a higher likelihood of having a White PD if the department chair was White. We found gender and racial disparities in all PGP leadership positions. This data can serve as a guide in efforts to support diversity for both gender and race at all positions and academic ranks.
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Gastroenterología , Pediatría , Niño , Docentes Médicos , Becas , Femenino , Humanos , Liderazgo , Masculino , Estados UnidosRESUMEN
Ustekinumab is a monoclonal antibody used as treatment for various inflammatory conditions. We present a pediatric patient with Crohn's disease who did not tolerate infliximab and was then changed to ustekinumab. He developed anaphylaxis to the medication after the second dose. A drug desensitization protocol was created by the allergy team leading to successful administration of both intravenous and then subcutaneous ustekinumab. As monoclonal agents become mainstays of therapy for inflammatory conditions, there are increased reports of allergic reactions. Prior reports and protocols of ustekinumab desensitization have not been reported. This case report highlights successful desensitization to ustekinumab as well as the importance of a multidisciplinary approach to addressing treatment needs of patients who develop life-threatening reactions to such medications.
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BACKGROUND: Maintenance of health leads to better outcomes in patients with chronic illness. ImproveCareNow, an international inflammatory bowel disease (IBD) quality improvement (QI) network, recommends maintenance-of-health visits twice a year. We identified a gap in care, with only 64% of IBD patients having documented visits within 200 days. Therefore, we sought to improve our follow-up rate to a goal of 80%. METHODS: Using population management (PM) reports, we identified patient-, data-, and treatment-related reasons for no documented visit within 200 days. We used the Pareto chart, key drivers, and process flow mapping and implemented changes using Plan-Do-Study-Act (PDSA) cycles to improve follow-up visit rates. Outcomes were presented using a control run chart with pre- and post- intervention data. RESULTS: The most common reasons for no visits were patient nonadherence with appointments (50%) and relocation/transition to an adult provider (25%). The median percentage of documented visits within 200 days increased from 64% to 83% (p < 0.0001), and this increase has been sustained for one year. CONCLUSIONS: Using the PM tool and focused QI interventions improved data quality and the percentage of patients with a documented visit within 200 days. The process is simple and can be applied to patients with other chronic illnesses.
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BACKGROUND & AIMS: Up to 30% of patients with Crohn's disease (CD) require surgery within the first 5 years from diagnosis. We investigated the recent risk of bowel surgery in an inception cohort of pediatric patients with CD and whether early use of biologics (tumor necrosis factor antagonists) alters later disease course. METHODS: We collected data from the Pediatric Inflammatory Bowel Disease Collaborative Research Group registry on 1442 children (age, ≤16 y) diagnosed with CD from January 2002 through December 2014. Data were collected at diagnosis, 30 days following diagnosis, and then quarterly and during hospitalizations for up to 12 years. Our primary aim was to determine the 10-year risk for surgery in children with CD. Our secondary aim was to determine whether early use of biologics (<3 mo of diagnosis) affected risk of disease progression. RESULTS: The 10-year risk of first bowel surgery was 26%. The 5-year risk of bowel surgery did not change from 2002 through 2014, and remained between 13% and 14%. Most surgeries occurred within 3 years from diagnosis. The only predictor of surgery was disease behavior at diagnosis. CD with inflammatory behavior had the lowest risk of surgery compared to stricturing disease, penetrating disease, or both. We associated slowing of disease progression to stricturing or penetrating disease (but not surgery) with early use of biologics, but this effect only became evident after 5 years of disease. Our results indicate that biologics slow disease progression over time (hazard ratio, 0.85; 95% CI, 0.76-0.95). CONCLUSIONS: In an analysis of data from a registry of pediatric patients with CD, we found that among those with significant and progressing disease at or shortly after presentation, early surgery is difficult to prevent, even with early use of biologics. Early use of biologics (<3 mo of diagnosis) can delay later disease progression to stricturing and/or penetrating disease, but this affect could become evident only years after initial management decisions are made.
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Productos Biológicos/administración & dosificación , Enfermedad de Crohn/tratamiento farmacológico , Enfermedad de Crohn/cirugía , Progresión de la Enfermedad , Utilización de Procedimientos y Técnicas/estadística & datos numéricos , Procedimientos Quirúrgicos Operativos/estadística & datos numéricos , Adolescente , Niño , Preescolar , Femenino , Hospitalización/estadística & datos numéricos , Humanos , Lactante , Recién Nacido , Masculino , Resultado del TratamientoRESUMEN
BACKGROUND: Racially disparate care has been shown to contribute to suboptimal health care outcomes for minorities. Using the ImproveCareNow network, we investigated differences in management and outcomes of pediatric patients with Crohn's disease at diagnosis and 1-year postdiagnosis. METHODS: ImproveCareNow is a learning health network for pediatric inflammatory bowel disease. It contains prospective, longitudinal data from outpatient encounters. This retrospective study included all patients with Crohn's disease ≤21 years, September 2006 to October 2014, with the first recorded encounter ≤90 days from date of diagnosis and an encounter 1 year ±60 days. We examined the effect of race on remission rate and treatment at diagnosis and 1 year from diagnosis using t-tests, Wilcoxon rank-sum tests, χ statistic, and Fisher's exact tests, where appropriate, followed by univariate regression models. RESULTS: Nine hundred seventy-six patients (Black = 118 (12%), White = 858 (88%), mean age = 13 years, 63% male) from 39 sites were included. Black children had a higher percentage of Medicaid insurance (44% versus 11%, P < 0.001). At diagnosis, Black children had more active disease according to physician global assessment (P = 0.027), but not by short Pediatric Crohn's Disease Activity Index (P = 0.67). Race differences in treatment were not identified. Black children had lower hematocrit (34.8 versus 36.7, P < 0.001) and albumin levels (3.6 versus 3.9, P = 0.001). At 1 year, Black children had more active disease according to physician global assessment (P = 0.016), but not by short Pediatric Crohn's Disease Activity Index (P = 0.06). CONCLUSIONS: Black children with Crohn's disease may have more severe disease than White children based on physician global assessment. Neither disease phenotype differences at diagnosis nor treatment differences at 1-year follow-up were identified.
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Negro o Afroamericano/estadística & datos numéricos , Enfermedad de Crohn/etnología , Disparidades en Atención de Salud/estadística & datos numéricos , Evaluación del Resultado de la Atención al Paciente , Población Blanca/estadística & datos numéricos , Adolescente , Distribución de Chi-Cuadrado , Niño , Enfermedad de Crohn/terapia , Femenino , Humanos , Masculino , Análisis de Regresión , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Estadísticas no ParamétricasRESUMEN
AIM: To investigate the safety profile of acquiring additional intestinal biopsies for research purposes in children undergoing a medically indicated colonoscopy. METHODS: A retrospective review of 122 pediatric patients who underwent colonoscopy over a 9 mo time period was completed. 38/122 participants consented to a research study in which 4 additional biopsies were obtained, in addition to routine biopsies. The outcomes after colonoscopy were measured in the research participants, and compared to 84 control participants who did not consent for the study. Groups were compared with regard to number of biopsies obtained, underlying diagnosis, and both serious and minor adverse outcomes. Data was collected including: age, gender, race, indication, diagnosis, number of biopsies obtained per case and post procedure adverse events. Medical records were reviewed and a questionnaire was completed by each of the ten gastroenterologists who performed procedures during the study. Physicians were asked about individual patient outcomes to ensure that all adverse events, such as perforation, excessive bleeding, infection, and minor gastrointestinal outcomes, were captured and included. RESULTS: The research group had more biopsies obtained (mean = 13.58 ± 4.21) compared to controls (mean = 9.33 ± 4.40), P ≤ 0.0001, however there was no difference in adverse events. Serious outcomes, defined as perforation, bleeding and infection, did not occur, in either group. As such, the relationship between serious adverse events and number of biopsies obtained was not determined. Minor gastrointestinal outcomes, such as abdominal pain, diarrhea or vomiting, were reported in 21 patients (8 research participants and 13 control participants) however the incidence of minor gastrointestinal outcomes between the two groups did not vary significantly, P = 0.45. Additionally, the mean number of biopsies obtained in patients who had a minor outcome (mean = 12.1 ± 0.77), compared to those with no adverse outcome (mean = 10.34 ± 0.5), revealed no statistical difference between the groups (P = 0.12), suggesting that number of biopsies is not associated with incidence of minor adverse events. CONCLUSION: Patients participating in research requiring acquisition of additional biopsies for research purposes alone, are not at an increased risk of adverse outcomes.
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BACKGROUND AND OBJECTIVES: Failure to attend pediatric outpatient endoscopic procedures leads to inefficient use of resources, longer wait-list times, and delay in diagnoses. The causes for pediatric endoscopy nonattendance are not well studied. The aim of the study was to identify factors associated with failure to attend endoscopic procedures and to assess the value of quality improvement (QI) interventions implemented to improve pediatric endoscopy attendance. METHODS: This was a continuous QI project. We collected nonattendance data from November 2011 to November 2013. Information collected included procedure type, age, sex, time on the waiting list, history of previous procedures, and reason for nonattendance. The following QI interventions were implemented sequentially: an appointment reminder letter, a telephone call 1 week before procedure, and creation of an electronic medical note dedicated to endoscopy appointment. Pareto charts and statistical process control charts were used for analysis. RESULTS: From November 2011 to November 2013, we were able to decrease nonattendance from 17% to 11% (Pâ=â0.005). No-show rate was reduced from 5% to 0.9% (Pâ=â0.00001). There was no significant difference between attendees and nonattendees in relation to sex, age, or having a previous procedure. Longer waiting time (33 vs 26 days) was associated with increased risk for nonattendance (Pâ=â0.0007). The most common causes for nonattendance were illness (31.5%), followed by caregiver/patients who no longer wanted the procedure (17.7%), and patients who improved (12.9%). CONCLUSIONS: Applying QI methods and tools improved pediatric endoscopy attendance. Longer wait time for endoscopic procedures is associated with nonattendance. Given the increased pediatric endoscopy demand, strategies should be implemented to reduce wait time for pediatric endoscopy.
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Citas y Horarios , Endoscopía , Pacientes no Presentados/estadística & datos numéricos , Pacientes Ambulatorios , Pediatría , Centros de Atención Terciaria , Adolescente , Niño , Preescolar , Colonoscopía , Endoscopía del Sistema Digestivo , Femenino , Humanos , Masculino , Factores de TiempoRESUMEN
OBJECTIVES: Gender equality in the workplace has not been described in pediatric gastroenterology. METHODS: An electronic survey that explored perceptions of career parity, work-life balance, and workplace harassment was sent to all members of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Reponses were anonymous. RESULTS: Of the 303 respondents (21%), there was an even distribution across geographic region, age, and gender (54% men). Gender affected perception of salary and promotion; 46% of men but only 9% of women feel that "women earn the same as men" (Pâ<â0.001). Similarly, 48% of men but only 12% of women feel that "women rise at the same rate as men" (Pâ<â0.001). Both genders of academic practice respondents, compared with other practice models, perceived men were promoted more quickly than women (Pâ=â0.008). Women had higher dissatisfaction with mentoring than men (29% vs 13%, Pâ=â0.03). Significantly more men than women reported spouses with "flexible jobs" (35% vs 14%, Pâ<â0.001). Having a spouse with "flexible job" or having children (preschool or school age), however, did not affect satisfaction with work-life balance for either gender. Overall, women are more likely to be dissatisfied with work-life balance than men (Pâ=â0.046). CONCLUSIONS: Satisfaction with work-life balance is lower among women versus men pediatric gastroenterologists, but does not correlate with flexibility of spouse's job or caring for young children. Gender-divergent perception of promotion, parity of compensation, and mentoring requires further investigation.
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Actitud , Empleo , Gastroenterología , Satisfacción en el Trabajo , Médicos , Sexismo , Derechos de la Mujer , Adulto , Acoso Escolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Responsabilidad Parental , Pediatría , Percepción , Salarios y Beneficios , Encuestas y Cuestionarios , TrabajoRESUMEN
BACKGROUND AND OBJECTIVE: Pediatric endoscopic procedures are essential in the evaluation and treatment of gastrointestinal diseases in children. Although pediatric endoscopists are greatly interested in increasing efficiency and through-put in pediatric endoscopy units, there is scarcely any literature on this critical process. The goal of this study was to improve the timeliness of pediatric endoscopy procedures at Children's Hospital at Montefiore. METHODS: In June 2010, a pediatric endoscopy quality improvement initiative was formed at Children's Hospital at Montefiore. We identified patient-, equipment-, and physician-related causes for case delays. Pareto charts, cause and effect diagrams, process flow mapping, and statistical process control charts were used for analysis. RESULTS: From June 2010 to December 2012, we were able to significantly decrease the first case endoscopy delay from an average of 17 to 10 minutes (P < .001), second case delay from 39 to 25 minutes (P = .01), third case delay from 61 to 45 minutes (P = .05), and fourth case delay from 79 to 51 minutes (P = .05). Total delay time decreased from 196 to 131 minutes, resulting in a reduction of 65 minutes (P = .02). From June 2010 to August 2011 (preintervention period), an average of 36% of first endoscopy cases started within 5 minutes, 51% within 10 minutes, and 61% within 15 minutes of the scheduled time. From September 2011 to December 2012 (postintervention period), the percentage of cases starting within 5 minutes, 10 minutes, and 15 minutes increased to 47% (P = .07), 61% (P = .04), and 79% (P = .01), respectively. CONCLUSIONS: Applying quality improvement methods and tools helped improve pediatric endoscopy timeliness and significantly decreased total delays.
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Endoscopía Gastrointestinal/estadística & datos numéricos , Niño , Hospitales Pediátricos , Humanos , Estudios Prospectivos , Factores de TiempoRESUMEN
OBJECTIVES: Variations in chronic illness care are common in our health care system and may lead to suboptimal outcomes. Specifically, inconsistent use and suboptimal medication dosing have been demonstrated in the care of patients with inflammatory bowel disease (IBD). Quality improvement (QI) efforts have improved outcomes in conditions such as asthma and diabetes mellitus, but have not been well studied in IBD. We hypothesized that QI efforts would lead to improved outcomes in our pediatric IBD population. METHODS: A QI team was formed within our IBD center in 2005. By 2007, we began prospectively capturing physician global assessment (PGA) and patient-reported global assessment. Significant QI interventions included creating evidence-based medication guidelines, joining a national QI collaborative, initiation of preclinic planning, and monitoring serum 25-hydroxyvitamin D. RESULTS: From 2007 to 2010, 505 patients have been followed at our IBD center. During this time, the frequency of patients in clinical remission increased from 59% to 76% (Pâ<â0.05), the frequency of patients who report that their global assessment is >7 increased from 69% to 80% (Pâ<â0.05), and the frequency of patients with a Short Pediatric Crohn's Disease Activity Index (sPCDAI) <15 increased from 60% to 77% (Pâ<â0.05). The frequency of repeat steroid use decreased from 17% to 10% (Pâ<â0.05). We observed an association between the use of a vitamin D supplement (Pâ=â0.02), serum 25-hydroxyvitamin D (Pâ<â0.05), and quiescent disease activity. CONCLUSIONS: Our results show that significant improvements in patient outcomes are associated with QI efforts that do not rely on new medication or therapies.
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Atención a la Salud/normas , Enfermedades Inflamatorias del Intestino/terapia , Mejoramiento de la Calidad , Esteroides/uso terapéutico , Vitamina D/uso terapéutico , Adolescente , Niño , Conducta Cooperativa , Suplementos Dietéticos , Femenino , Guías como Asunto , Humanos , Enfermedades Inflamatorias del Intestino/sangre , Enfermedades Inflamatorias del Intestino/tratamiento farmacológico , Masculino , Estudios Prospectivos , Inducción de Remisión , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Vitamina D/análogos & derivados , Vitamina D/sangreRESUMEN
BACKGROUND & AIMS: We examined the incidence of Crohn's disease (CD)-related surgery in a multi-center, inception cohort of pediatric patients with CD. We also examined the effect of starting immunomodulator therapy within 30 days of diagnosis. METHODS: Data from 854 children with CD from the Pediatric Inflammatory Bowel Disease Collaborative Research Group who were diagnosed with CD between 2002 and 2008 were analyzed. RESULTS: Overall, 76 (9%) underwent a first CD-related surgery, 57 (7%) underwent a first bowel surgery (bowel resection, ostomy, strictureplasty, or appendectomy), and 19 (2%) underwent a first non-bowel surgery (abscess drainage or fistulotomy). The cumulative risks for bowel surgery, non-bowel surgery, and all CD-related surgeries were 3.4%, 1.4%, and 4.8%, respectively, at 1 year after diagnosis and 13.8%, 4.5%, and 17.7%, respectively, at 5 years after diagnosis. Older age at diagnosis, greater disease severity, and stricturing or penetrating disease increased the risk of bowel surgery. Disease between the transverse colon and rectum decreased the risk. Initiation of immunomodulator therapy within 30 days of diagnosis, sex, race, and family history of inflammatory bowel disease did not influence the risk of bowel surgery. CONCLUSIONS: In an analysis of pediatric patients with CD, the 5-year cumulative risk of bowel surgery was lower than that reported in recent studies of adult and pediatric patients but similar to that of a recent retrospective pediatric study. Initiation of immunomodulator therapy at diagnosis did not alter the risk of surgery within 5 years of diagnosis.
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Enfermedad de Crohn/patología , Enfermedad de Crohn/cirugía , Procedimientos Quirúrgicos del Sistema Digestivo/estadística & datos numéricos , Medición de Riesgo , Adolescente , Niño , Preescolar , Enfermedad de Crohn/tratamiento farmacológico , Femenino , Humanos , Factores Inmunológicos/uso terapéutico , Incidencia , Lactante , Recién Nacido , MasculinoRESUMEN
OBJECTIVES: Although it is known that extraintestinal manifestations (EIMs) commonly occur in pediatric inflammatory bowel disease (IBD), little research has examined rates of EIMs and their relation to other disease-related factors in this population. The purpose of this study was to determine the rates of EIMs in pediatric IBD and examine correlations with age, sex, diagnosis, disease severity, and distribution. PATIENTS AND METHODS: Data were prospectively collected as part of the Pediatric IBD Collaborative Research Group Registry, an observational database enrolling newly diagnosed IBD patients <16 years old since 2002. Rates of EIM (occurring anytime during the period of enrollment) and the aforementioned variables (at baseline) were examined. Patients with indeterminate colitis were excluded from the analysis given the relatively small number of patients. RESULTS: One thousand nine patients were enrolled (mean age 11.6 +/- 3.1 years, 57.5% boys, mean follow-up 26.2 +/- 18.2 months). Two hundred eighty-five (28.2%) patients experienced 1 or more EIMs. Eighty-seven percent of EIM occurred within the first year. Increased disease severity at baseline (mild vs moderate/severe) was associated with the occurrence of any EIM (P < 0.001), arthralgia (P = 0.024), aphthous stomatitis (P = 0.001), and erythema nodosum (P = 0.009) for both Crohn disease (CD) and ulcerative colitis (UC) during the period of follow-up. Statistically significant differences in the rates of EIMs between CD and UC were seen for aphthous stomatitis, erythema nodosum, and sclerosing cholangitis. CONCLUSIONS: EIMs as defined in this study occur in approximately one quarter of pediatric patients with IBD. Disease type and disease severity were commonly associated with the occurrence of EIMs.
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Artralgia/etiología , Colitis Ulcerosa/complicaciones , Enfermedad de Crohn/complicaciones , Eritema Nudoso/etiología , Estomatitis Aftosa/etiología , Adolescente , Artralgia/epidemiología , Niño , Eritema Nudoso/epidemiología , Femenino , Humanos , Masculino , Estudios Prospectivos , Índice de Severidad de la Enfermedad , Estomatitis Aftosa/epidemiologíaRESUMEN
OBJECTIVES: Infliximab is effective in treating moderate/severe ulcerative colitis (UC) in adults. The aim of this study was to determine the outcome after treatment with infliximab in pediatric UC. METHODS: We performed a multicenter cohort study of 332 pediatric patients with UC enrolled in the Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry. Children
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Antiinflamatorios/uso terapéutico , Anticuerpos Monoclonales/uso terapéutico , Colitis Ulcerosa/tratamiento farmacológico , Adolescente , Niño , Estudios de Cohortes , Femenino , Humanos , Infliximab , Masculino , Estudios Prospectivos , Sistema de Registros , Resultado del TratamientoRESUMEN
OBJECTIVES: To analyze the IBD5 locus in a homogenous cohort of Ashkenazi Jewish (AJ) children with Crohn disease (CD). PATIENTS AND METHODS: A total of 83 AJ children with CD and 73 AJ healthy controls were studied. Genotyping for single nucleotide polymorphisms (SNPs) including OCTN1 (SLC22A4; 1672C-->T), OCTN2 (SLC22A5; 207G-->C), IGR2096, IGR2198, and IGR2230 genes was performed using the TaqMan system. NOD2/CARD15 variants also were typed using established methods. RESULTS: All IBD5 SNPs tested were in linkage disequilibrium (D'>0.8), and showed significant association with CD in our cohort of AJ children. The IGR2096 SNP, which is not located within the same linkage disequilibrium block as the OCTN1 and 2 SNPs, showed an even stronger association with CD (P = 0.017; odds ratio = 1.7). Patients with CD who had the OCTN1 susceptibility allele were more likely to carry 1 of the 3 NOD2/CARD15 SNPs tested (P = 0.01; odds ratio = 4.8). CONCLUSIONS: We have demonstrated a significant association between the IBD5 locus and CD in a homogenous cohort of pediatric AJ patients. Due to the tight linkage disequilibrium in the region, it is not possible to identify the causative IBD5 variant. Future functional studies will ultimately reveal the causative gene variant at this locus.
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Cromosomas Humanos Par 5 , Enfermedad de Crohn/genética , Judíos/genética , Desequilibrio de Ligamiento , Polimorfismo de Nucleótido Simple , Proteínas Adaptadoras Transductoras de Señales , Niño , Enfermedad de Crohn/etnología , Femenino , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Genotipo , Haplotipos , Humanos , Masculino , Proteína Adaptadora de Señalización NOD2/genética , Proteínas de Transporte de Catión Orgánico/genética , Simportadores , Proteínas de Unión al GTP rab/genéticaRESUMEN
BACKGROUND & AIMS: Genetic variations that affect innate immunity increase risk of ileal Crohn's disease (CD). However, the penetrance of susceptibility genes, including NOD2, is low, suggesting additional risk factors. Neutralizing autoantibodies (Ab) against granulocyte-macrophage colony-stimulating factor (GM-CSF Ab) reduce neutrophil antimicrobial function in patients with primary alveolar proteinosis (PAP). We investigated whether GM-CSF Ab regulates neutrophil function in CD. METHODS: Serum samples from 354 adult and pediatric patients with inflammatory bowel disease (IBD) were analyzed for GM-CSF Ab and IBD markers. Levels of GM-CSF Ab were compared with patients' CD features and neutrophil function. Intestinal barrier function and nonsteroidal anti-inflammatory drug (NSAID)-induced injury were assessed in GM-CSF-null and NOD2-null mice. RESULTS: Median GM-CSF Ab levels increased from 0.4 microg/mL in control serum to 2.4 microg/mL in pediatric CD and 11.7 microg/mL in adult CD serum and were associated with ileal involvement (P<.001). Ileal location, duration of disease, and increased GM-CSF Ab levels were associated with stricturing/penetrating behavior (odds ratio, 2.2; P=.018). The positive and negative predictive values of GM-CSF Ab for stricturing/penetrating behavior were comparable with that of other IBD serum markers. CD patients with increased GM-CSF Ab had reduced neutrophil phagocytic capacity and increased accumulation of pSTAT3+ neutrophils in the affected ileum. GM-CSF-null mice and NOD2-null mice in which GM-CSF was neutralized had defects in mucosal barrier function and developed a transmural ileitis following NSAID exposure. CONCLUSIONS: GM-CSF regulates ileal homeostasis in CD and in mouse models. CD patients with increases in serum GM-CSF Ab might benefit from GM-CSF administration.