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Higher Morbidity of Monogenic Inflammatory Bowel Disease Compared to the Adolescent Onset Inflammatory Bowel Disease / 대한소아소화기영양학회지
Pediatric Gastroenterology, Hepatology & Nutrition ; : 34-42, 2018.
Artigo em Inglês | WPRIM | ID: wpr-741818
ABSTRACT

PURPOSE:

Monogenic inflammatory bowel disease (IBD) patients do not respond to conventional therapy and are associated with a higher morbidity. We summarized the clinical characteristics of monogenic IBD patients and compared their clinical outcomes to that of non-monogenic IBD patients.

METHODS:

We performed a retrospective cohort study of all children <18 years old who were diagnosed with IBD between 2005 and 2016. A total of 230 children were enrolled. Monogenic IBD was defined as a presentation age less than 6 years old with confirmation of a genetic disorder. We subdivided the groups into monogenic IBD (n=18), non-monogenic very early-onset IBD (defined as patients with a presentation age <6 years old without a confirmed genetic disorder, n=12), non-monogenic IBD (defined as all patients under 18 years old excluding monogenic IBD, n=212), and severe IBD (defined as patients treated with an anti-tumor necrosis factor excluding monogenic IBD, n=92). We compared demographic data, initial pediatric Crohn disease activity index/pediatric ulcerative colitis activity index (PCDAI/PUCAI) score, frequency of hospitalizations, surgical experiences, and height and weight under 3rd percentile among the patients enrolled.

RESULTS:

The initial PCDAI/PUCAI score (p < 0.05), incidence of surgery per year (p < 0.05), and hospitalization per year (p < 0.05) were higher in the monogenic IBD group than in the other IBD groups. Additionally, the proportion of children whose weight and height were less than the 3rd percentile (p < 0.05 and p < 0.05, respectively) was also higher in the monogenic IBD group.

CONCLUSION:

Monogenic IBD showed more severe clinical manifestations than the other groups.
Assuntos

Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Doenças Inflamatórias Intestinais / Colite Ulcerativa / Doença de Crohn / Incidência / Estudos Retrospectivos / Estudos de Coortes / Interleucina-10 / Hospitalização / Síndromes de Imunodeficiência / Necrose Tipo de estudo: Estudo de etiologia / Estudo de incidência / Estudo observacional / Estudo prognóstico / Fatores de risco Limite: Adolescente / Criança / Humanos Idioma: Inglês Revista: Pediatric Gastroenterology, Hepatology & Nutrition Ano de publicação: 2018 Tipo de documento: Artigo

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Texto completo: DisponíveL Índice: WPRIM (Pacífico Ocidental) Assunto principal: Doenças Inflamatórias Intestinais / Colite Ulcerativa / Doença de Crohn / Incidência / Estudos Retrospectivos / Estudos de Coortes / Interleucina-10 / Hospitalização / Síndromes de Imunodeficiência / Necrose Tipo de estudo: Estudo de etiologia / Estudo de incidência / Estudo observacional / Estudo prognóstico / Fatores de risco Limite: Adolescente / Criança / Humanos Idioma: Inglês Revista: Pediatric Gastroenterology, Hepatology & Nutrition Ano de publicação: 2018 Tipo de documento: Artigo