Assuntos
Colangite/diagnóstico , Granuloma de Células Plasmáticas/diagnóstico , Pâncreas/patologia , Neoplasias Pancreáticas/diagnóstico , Adolescente , Colangite/etiologia , Feminino , Granuloma de Células Plasmáticas/complicações , Humanos , Neoplasias de Tecido Muscular , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/patologiaRESUMO
Inflammatory bowel disease (IBD) is uncommon in children younger than 2 years of age. The criteria for differentiating IBD from other diseases with similar clinical presentation is unclear. We describe 16 patients who, between 1984 and 2004, received a histological diagnosis of IBD during the first two years of life. Six patients presented with histological Crohn's disease, eight with ulcerative colitis and two with indeterminate colitis. The median age at diagnosis was 125 days (range 1 day to 18 months) and the medium follow up was 89 months (range 65 days to 20 years). The disease appeared to be very severe: four children (25%) underwent total parenteral nutrition (TPN), two received colectomy (12.5%) and three patients died. Many of the patients required an aggressive, multidrug, immunosuppressive approach (azathioprine [AZA], Infliximab, thalidomide, cyclosporine A). One child presented with a hypogammaglobulinaemia without any specific immunodeficiency, while in the other patients, Wiskott-Aldrich syndrome (WAS) (4 cases) and chronic granulomatous disease (CGD) (2 cases) were identified. In 6/16 cases, allergic colitis was first considered; these cases initially underwent cow's milk protein-free diet as the only therapy before IBD was finally diagnosed. In conclusion, early IBD has a severe prognosis and often needs an aggressive therapeutic approach. Furthermore, an improper diagnosis of allergic colitis might cause an important diagnostic delay. Some severe immunodeficiencies, such as WAS and CGD, may represent a problem in terms of differential diagnosis and might be wrongly diagnosed as very early onset IBD.
Assuntos
Colite Ulcerativa/diagnóstico , Colite/diagnóstico , Doença de Crohn/diagnóstico , Enterocolite/diagnóstico , Colite/mortalidade , Colite/patologia , Colite/terapia , Colite Ulcerativa/mortalidade , Colite Ulcerativa/patologia , Colite Ulcerativa/terapia , Colo/patologia , Colonoscopia , Terapia Combinada , Doença de Crohn/mortalidade , Doença de Crohn/patologia , Doença de Crohn/terapia , Diagnóstico Diferencial , Enterocolite/mortalidade , Enterocolite/patologia , Enterocolite/terapia , Feminino , Doença Granulomatosa Crônica/diagnóstico , Doença Granulomatosa Crônica/mortalidade , Doença Granulomatosa Crônica/patologia , Doença Granulomatosa Crônica/terapia , Humanos , Lactente , Recém-Nascido , Mucosa Intestinal/patologia , Masculino , Estudos Retrospectivos , Síndrome de Wiskott-Aldrich/diagnóstico , Síndrome de Wiskott-Aldrich/mortalidade , Síndrome de Wiskott-Aldrich/patologia , Síndrome de Wiskott-Aldrich/terapiaRESUMO
BACKGROUND AND AIMS: Our study evaluated the prevalence, the characteristics and implications of the upper gastrointestinal localisation (UGI+) in paediatric Crohn's Disease (CD) patients. METHODS: This prospective study evaluated 45 newly diagnosed CD patients at diagnosis and follow up with respect to CD localisation. RESULTS: All patients presented CD at the colon and/or ileum. In 24/45 patients (53.3%, 12 F and 12 M) an UGI+ involvement was also found. UGI+ patients had a younger age of onset (10.9 years versus 12.6 years; P<0.05). PCDAI at diagnosis was significantly higher in the UGI+ (41 vs. 25 P<0.01). UGI+ patients were overall more symptomatic. Pancolitis and extraintestinal manifestations were also more frequent (19/24 (80%) vs. 12/21 (57%) P<0.01). Growth was more impaired at diagnosis in UGI+ patients. By the end of the follow-up (mean 3 years, range 2 to 4) no significant difference was found in PCDAI (17 in UGI+ patients vs. 11 in UGI- P=NS), or the number of relapses. Weight and growth catch-up in UGI+ patients were comparable to UGI- ones. However, UGI+ patients required a more aggressive therapeutic approach. CONCLUSION: At least half of paediatric onset CD patients have an upper gastrointestinal localisation. UGI+ patients present an earlier onset and a more severe disease. The final outcome does not differ, but UGI+ patients require a more aggressive therapeutic approach.