BACKGROUND: Risk benefit strategies in managing inflammatory bowel diseases (IBD) are dependent upon understanding the risks of uncontrolled inflammation vs those of treatments. Malignancy and mortality in IBD have been associated with disease-related inflammation and immune suppression, but data are limited due to their rare occurrence. AIM: To identify and describe the most common causes of mortality, types of cancer and previous or current therapy among children and young adults with paediatric-onset IBD. METHODS: Information on paediatric-onset IBD patients diagnosed with malignancy or mortality was prospectively collected via a survey in 25 countries over a 42-month period. Patients were included if death or malignancy occurred after IBD diagnosis but before the age of 26 years. RESULTS: In total, 60 patients were identified including 43 malignancies and 26 fatal cases (9 due to cancer). Main causes of fatality were malignancies (n = 9), IBD or IBD-therapy related nonmalignant causes (n = 10; including 5 infections), and suicides (n = 3). Three cases, all fatal, of hepatosplenic T-cell lymphoma were identified, all were biologic-naïve but thiopurine-exposed. No other haematological malignancies were fatal. The 6 other fatal cancer cases included 3 colorectal adenocarcinomas and 3 cholangiocarcinomas (CCAs). Primary sclerosing cholangitis (PSC) was present in 5 (56%) fatal cancers (1 colorectal carcinoma, 3 CCAs and 1 hepatosplenic T-cell lymphoma). CONCLUSIONS: We report the largest number of paediatric-onset IBD patients with cancer and/or fatal outcomes to date. Malignancies followed by infections were the major causes of mortality. We identified PSC as a significant risk factor for cancer-associated mortality. Disease-related adenocarcinomas were a commoner cause of death than lymphomas.
- MeSH
- dítě MeSH
- dospělí MeSH
- hodnocení rizik MeSH
- idiopatické střevní záněty komplikace epidemiologie mortalita MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- nádory komplikace epidemiologie mortalita MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- prospektivní studie MeSH
- rizikové faktory MeSH
- věk při počátku nemoci MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- pozorovací studie MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Evropa epidemiologie MeSH
Children and adolescents with Crohn's disease (CD) present often with a more complicated disease course compared to adult patients. In addition, the potential impact of CD on growth, pubertal and emotional development of patients underlines the need for a specific management strategy of pediatric-onset CD. To develop the first evidenced based and consensus driven guidelines for pediatric-onset CD an expert panel of 33 IBD specialists was formed after an open call within the European Crohn's and Colitis Organisation and the European Society of Pediatric Gastroenterolog, Hepatology and Nutrition. The aim was to base on a thorough review of existing evidence a state of the art guidance on the medical treatment and long term management of children and adolescents with CD, with individualized treatment algorithms based on a benefit-risk analysis according to different clinical scenarios. In children and adolescents who did not have finished their growth, exclusive enteral nutrition (EEN) is the induction therapy of first choice due to its excellent safety profile, preferable over corticosteroids, which are equipotential to induce remission. The majority of patients with pediatric-onset CD require immunomodulator based maintenance therapy. The experts discuss several factors potentially predictive for poor disease outcome (such as severe perianal fistulizing disease, severe stricturing/penetrating disease, severe growth retardation, panenteric disease, persistent severe disease despite adequate induction therapy), which may incite to an anti-TNF-based top down approach. These guidelines are intended to give practical (whenever possible evidence-based) answers to (pediatric) gastroenterologists who take care of children and adolescents with CD; they are not meant to be a rule or legal standard, since many different clinical scenario exist requiring treatment strategies not covered by or different from these guidelines.
- Klíčová slova
- Crohn's disease, Guidelines, Medical therapy, Pediatric,
- MeSH
- adalimumab MeSH
- algoritmy MeSH
- antibakteriální látky terapeutické užití MeSH
- antiflogistika nesteroidní terapeutické užití MeSH
- azathioprin terapeutické užití MeSH
- Crohnova nemoc terapie MeSH
- dítě MeSH
- enterální výživa * MeSH
- hormony kůry nadledvin škodlivé účinky terapeutické užití MeSH
- humanizované monoklonální protilátky terapeutické užití MeSH
- imunosupresiva terapeutické užití MeSH
- indukce remise metody MeSH
- infliximab MeSH
- kyseliny aminosalicylové terapeutické užití MeSH
- lidé MeSH
- merkaptopurin terapeutické užití MeSH
- methotrexát terapeutické užití MeSH
- mladiství MeSH
- monoklonální protilátky terapeutické užití MeSH
- thalidomid terapeutické užití MeSH
- TNF-alfa antagonisté a inhibitory MeSH
- udržovací chemoterapie metody MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- Publikační typ
- časopisecké články MeSH
- směrnice pro lékařskou praxi MeSH
- Názvy látek
- adalimumab MeSH
- antibakteriální látky MeSH
- antiflogistika nesteroidní MeSH
- azathioprin MeSH
- hormony kůry nadledvin MeSH
- humanizované monoklonální protilátky MeSH
- imunosupresiva MeSH
- infliximab MeSH
- kyseliny aminosalicylové MeSH
- merkaptopurin MeSH
- methotrexát MeSH
- monoklonální protilátky MeSH
- thalidomid MeSH
- TNF-alfa MeSH
