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1.
Lancet ; 371(9613): 660-667, 2008 Feb 23.
Artigo em Inglês | MEDLINE | ID: mdl-18295023

RESUMO

BACKGROUND: Most patients who have active Crohn's disease are treated initially with corticosteroids. Although this approach usually controls symptoms, many patients become resistant to or dependent on corticosteroids, and long exposure is associated with an increased risk of mortality. We aimed to compare the effectiveness of early use of combined immunosuppression with conventional management in patients with active Crohn's disease who had not previously received glucocorticoids, antimetabolites, or infliximab. METHODS: We did a 2-year open-label randomised trial at 18 centres in Belgium, Holland, and Germany between May, 2001, and January, 2004. We randomly assigned 133 patients to either early combined immunosuppression or conventional treatment. The 67 patients assigned to combined immunosuppression received three infusions of infliximab (5 mg/kg of bodyweight) at weeks 0, 2, and 6, with azathioprine. We gave additional treatment with infliximab and, if necessary, corticosteroids, to control disease activity. 66 patients assigned to conventional management received corticosteroids, followed, in sequence, by azathioprine and infliximab. The primary outcome measures were remission without corticosteroids and without bowel resection at weeks 26 and 52. Analysis was by modified intention to treat. This trial was registered with ClinicalTrials.gov, number NCT00554710. FINDINGS: Four patients (two in each group) did not receive treatment as per protocol. At week 26, 39 (60.0%) of 65 patients in the combined immunosuppression group were in remission without corticosteroids and without surgical resection, compared with 23 (35.9%) of 64 controls, for an absolute difference of 24.1% (95% CI 7.3-40.8, p=0.0062). Corresponding rates at week 52 were 40/65 (61.5%) and 27/64 (42.2%) (absolute difference 19.3%, 95% CI 2.4-36.3, p=0.0278). 20 of the 65 patients (30.8%) in the early combined immunosuppression group had serious adverse events, compared with 19 of 64 (25.3%) controls (p=1.0). INTERPRETATION: Combined immunosuppression was more effective than conventional management for induction of remission and reduction of corticosteroid use in patients who had been recently diagnosed with Crohn's disease. Initiation of more intensive treatment early in the course of the disease could result in better outcomes.


Assuntos
Doença de Crohn/tratamento farmacológico , Imunossupressores/uso terapêutico , Corticosteroides/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Azatioprina/uso terapêutico , Bélgica , Distribuição de Qui-Quadrado , Feminino , Alemanha , Humanos , Infliximab , Masculino , Metotrexato/uso terapêutico , Países Baixos , Estatísticas não Paramétricas , Resultado do Tratamento
2.
Eur J Med Genet ; 61(4): 181-184, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29174091

RESUMO

We first describe a patient with multiple endocrine neoplasia type 1 (MEN1) and dorsal pancreatic hemi-agenesis. Previously, pancreas divisum has been reported in MEN1. Recent data in mice have elucidated the molecular mechanisms of pancreatic endoderm specification. Disinhibition of hedgehog signaling appears to be important in how Gata4 and Gata6 variants cause pancreatic agenesis. Disinhibition of hedgehog signaling has also been observed in Men1 knockout pancreatic islets. Although we cannot exclude a spurious association between dorsal pancreatic hemi-agenesis and MEN1 in our patient, we argue that developmental abnormalities of the pancreas may have to be considered as possibly related to the MEN1 phenotype.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1/genética , Pâncreas/anormalidades , Proteínas Proto-Oncogênicas/genética , Adulto , Feminino , Humanos , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico por imagem , Neoplasia Endócrina Múltipla Tipo 1/patologia , Mutação , Linhagem , Fenótipo
3.
Acta Gastroenterol Belg ; 76(3): 335-9, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24261029

RESUMO

Spontaneous dissection of the celiac trunk is uncommon and rarely considered in patients presenting with acute onset of epigastric pain. We report the case of a 48-year old male, diagnosed with a spontaneous dissection of the celiac trunk extending towards the common hepatic artery. He was treated conservatively and remained asymptomatic after two years of follow-up. Conservative treatment seems justified in the absence of bowel ischemia or signs of hemorrhage. Initial Computed Tomography angiography revealed the presence of a dissection with a pseudoaneurysm that remained stable and regressed towards a normal Computed Tomography angiography after 7 months of follow-up. Radiologic follow-up is warranted as progression of the dissection and/or total occlusion with or without symptoms can occur. The risk factors, the natural course and optimal treatment remain unclear due to the rarity of the disorder. Our patient had no predisposing cardiovascular risk factors. Nevertheless, we observed a hypertrophic ligamentum arcuatum on Computed Tomography, possibly facilitating the evolution towards a dissection. Next to the case report, we provide a review of the available literature.


Assuntos
Dor Abdominal/etiologia , Dissecção Aórtica/complicações , Dor Abdominal/diagnóstico por imagem , Dissecção Aórtica/diagnóstico por imagem , Angiografia , Artéria Celíaca , Diagnóstico Diferencial , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
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