Long-term outcomes and predictors of disabling disease in a population-based cohort of patients with incident Crohn's disease diagnosed between 1994 and 1997.

BACKGROUND: The identification of early prognostic factors during Crohn's disease (CD) remains needed for physician decision-making to minimize structural bowel damage, which this study aimed to assess in a population-based setting. METHODS: All incident cases of CD were prospectively registered from 1994 to 1997 in Brittany, a limited area of France. All charts of patients were reviewed from the diagnosis to the last clinic visit in 2015. Disabling CD course was defined according to the Saint-Antoine criteria. RESULTS: Among the 331 incident cases of CD, 272 (82%) were followed-up for a median time of 12.8 years. The cumulative probability of developing stricturing or fistulizing CD was 66% at 15 years, and 107 (39%) patients underwent surgery. The cumulative probabilities of immunosuppressant and TNF antagonist use at 15 years were 37% and 22%, respectively. The cumulative risks for disabling disease and bowel damage were 74% and 71% at 15 years, respectively. Systemic symptoms and perianal lesions at diagnosis were independently associated with a disabling disease course. Perianal disease and short disease extension were associated with the onset of bowel damage. Deep ulcers was not predictive of any outcome. CONCLUSIONS: A disabling disease course and bowel damage occurred early in the course of CD, which suggests the need for early diagnosis and early treatment, particularly for patients with systematic symptoms and perianal disease.

Natural History of Perianal Crohn's Disease: Long-term Follow-up of a Population-Based Cohort.

BACKGROUND AND AIMS: The natural history of perianal Crohn's disease (PCD) remains poorly described and is mainly based on retrospective studies from referral centers. The aim of this study was to assess the incidence, outcomes and predictors of the onset of PCD. METHODS: All incident cases of patients diagnosed with possible CD were prospectively registered from 1994 to 1997 in Brittany, a limited area in France. At diagnosis, the clinical features of perianal disease were recorded. All patient charts were reviewed from the diagnosis to the last clinic visit in 2015. RESULTS: Among the 272 out of 331 incident CD patients followed up, 51 (18.7%) patients had PCD at diagnosis. After a mean follow-up of 12.8 years, 93 (34%) patients developed PCD. The cumulative probabilities of perianal CD occurrence were 22%, 29%, and 32% after 1 year, 5 years, and 10 years, respectively. The cumulative probabilities of anal ulceration were 14%, and 19% after 1 year and 10 years, respectively. Extraintestinal manifestations were associated with the occurrence of anal ulceration. The cumulative probabilities of fistulizing PCD were 11%, 16%, and 19% after 1 year, 5 years, and 10 years, respectively. Extraintestinal manifestations, rectal involvement and anal ulceration were predictors of fistulizing PCD. The cumulative probability of developing anal stricture was 4% after 10 years. CONCLUSIONS: PCD is frequently observed during CD, in approximately one-third of patients. These data underline the need for targeted therapeutic research on primary perianal lesions (proctitis, anal ulceration) to avoid the onset of fistulizing perianal disease.

Survival of adolescents with cancer treated at pediatric versus adult oncology treatment centers in France.

BACKGROUND: In France, although children aged less than 15 years with cancer are usually referred to pediatric oncology centers, adolescents may be treated at pediatric or adult oncology centers. The objective was to compare survival according to their site of treatment. PROCEDURE: Using population-based registration, 15- to 19-year-old patients diagnosed with cancer in 2006 or 2007 and living in six French regions (accounting for 41% of the French population) were included. RESULTS: Of the 594 patients included, 33% of the French adolescents were treated at a pediatric oncology center. Compared with those treated at a pediatric center, adolescents treated at an adult center were older, were more likely to have carcinoma and germ-cell tumor, had a longer time to diagnosis, and were less likely to be enrolled in a clinical trial. In addition, the decisions for their management were less likely to be taken in the context of multidisciplinary team meetings. In multivariate analysis, adolescent patients treated at a pediatric center did not have significantly different overall survival (OS) compared with those treated at an adult center (5-year OS: 84.1% [95% confidence interval: 78.6-90.0] versus 87.7% [95% confidence interval: 84.2-91.3]; P = 0.25). CONCLUSIONS: The outcomes of French adolescents with cancer have begun to improve, with 81.2% survival in 2006-2007, with no difference between the types of treatment center. However, for this unique group of diseases, survival is not the unique endpoint. In order to ensure good quality of life after cancer, management of those patients requires specific approaches, designed to reduce the late effects of cancer treatment and improve supportive care.

Pathways of care for adolescent patients with cancer in France from 2006 to 2007.

BACKGROUND: In France, as in other countries, there is a need for a population-based view of access to care and modalities of treatment for adolescents with cancer. PROCEDURE: Using a population-based registration, we report pathways of care for 15-19-year-old patients, diagnosed with cancer in 2006 and 2007, living in six French regions, accounting for 41% of the French population. RESULTS: The median times (inter-quartile range) for diagnosis and treatment of the 594 included adolescents were 8 weeks (3-17) and 3 days (0-16), respectively. First physicians met by the patients were mostly general practitioners (59%). Seventeen percent of patients were firstly seen on emergency wards. Most of the patients (82%) were treated in an adult environment. Management decisions were taken within the context of a multi-disciplinary team (MDT) in 54% of cases. Twenty-seven percent of patients were included in randomized or non-randomized clinical studies: percentage depended on the tumor type and on the number of on-going trials at the study period. Fifteen percent of patients were included in pediatric studies, 7% in adult studies, and 5% in studies including both adults and children. CONCLUSIONS: The pathways of care for French adolescent patients with cancer are heterogeneous. Our results reveal differences in MDT meetings according to tumor types and a lack of effective collaboration between pediatric and adult wards. Efforts must be made to develop cancer networks to ensure that adolescents receive the optimal care in a suitable environment.

Childhood cancer survival in France, 1990-1999.

The aim of this study was to describe the overall survival after childhood cancer in France using follow-up data from regional population-based registries. The survival of children (aged under 15 years) diagnosed with a cancer during 1990-1999 was analysed. For all cancers, the survivals were, respectively, 90.3% [89.4-91.3] at 1-year, 75.2% [73.8-76.6] at 5 years and 72.2% [70.7-73.7] at 10 years. During the 1990s, the average improvement in the 5-year survival was +1.2% per year. Adjusted for gender, age, area of residence and stage, children with cancer diagnosed between 1995 and 1999 had a 0.80 reduced risk of dying compared with those whose cancer had been diagnosed between 1990 and 1994. The increase of survival at the population level reflects a global improvement in childhood cancer care. The Paediatric Registries, in association with the French Society of Childhood Cancer, are now collecting data to quantify on a national basis the other events, at least relapse and second cancers.

Cancer incidence among children in France, 1990-1999.

BACKGROUND: Cancer is the second most important cause of death for children aged less than 15 years in France, unintentional injuries being the leading cause. The aim of the present study was to estimate the incidence of childhood cancer from six Childhood Cancer Registries covering 32% of France. PROCEDURE: Incident cancer cases diagnosed between 1990 and 1999 in children (0-14 years) resident in the administrative areas covered by each Registry were included. Annual age-standardized rates (ASRs) were adjusted by the world population. The estimated annual percent change (EAPC) was used to measure trend towards changes in the annual age-standardized incidence rate. RESULTS: With 4234 registered cases, the ASRs per million children were 137.5 for all cancers combined, 42.3 for leukemia, 29.1 for central-nervous-system tumors, 15.6 for lymphomas, 14.1 for sympathetic-nervous-system tumors, and 9.1 for renal tumors. The ASR of all cancers combined was slightly higher in males (145.8 per million children) than in females (128.7 per million children) with an M/F ratio of 1.2. No significant incidence trend was observed, with an EAPC of +0.2% [IC 95% (-2.5; +3.0); P = 0.89]. CONCLUSIONS: The estimated incidence rates are similar to those reported in previous studies in European and North American countries. These results will contribute to the development of National Registration of Childhood Cancer in France and support the national research program on childhood cancer.

Crohn's disease in the over-60 age group: a population based study.

OBJECTIVES: Late-onset Crohn's disease (CD) may show a distinctive pattern of expression. The aim of our study was to describe the incidence or presentation of CD in the elderly and compare the outcomes in patients < 60 years or > or = 60 years at diagnosis. METHODS: The study included a population based inception cohort of all incident CD cases diagnosed in Brittany (France) between 1994 and 1997. Sixty-three patients were > or = 60 years and 201 were < 60 years at diagnosis. The physicians managing the patients used standardized questionnaires to collect the study data. The questionnaires on outcomes and treatments were completed in 2002. RESULTS: In patients > or = 60 years, the annual incidence was 2.5 per 10 persons and clinical features were similar to those in younger patients, except for a higher rate of colon involvement. Among older patients, those with diverticula (29/63, 46%) were more likely to have granulomas (58% vs 33% of patients without diverticula, P < 0.04), but the diagnosis of CD was confirmed by lesions remote from the diverticula in most cases (23/29). Early resection rates were not higher in older patients, who were less likely to require immunosuppressants or re-admission for CD flares, as compared to younger patients. Five year mortality in older patients was 16% but was unrelated to CD. CONCLUSIONS: In Brittany, the age specific incidence, clinical features, and prognosis of CD among the elderly are comparable to those in younger individuals. Colon involvement is more common. Concomitant diverticular disease is common and should prompt a search for CD lesions at other sites to confirm the diagnosis. Older patients are less likely to require immunosuppressants or admission for flares.