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1.
Artigo em Inglês | MEDLINE | ID: mdl-32740538

RESUMO

OBJECTIVES: Crohn disease (CD) can affect patient's quality of life (QOL) with physical, social, and psychological impacts. This study aimed to investigate the QOL of children with CD and its relationship with patient and disease characteristics. METHODS: Children ages from 10 to 17 years with diagnosed CD for more than 6 months were eligible to this cross-sectional study conducted in 35 French pediatric centers. QOL was assessed by the IMPACT-III questionnaire. Patient and disease characteristics were collected. RESULTS: A total of 218 children (42% of girls) were included at a median age of 14 years (interquartile range [IQR]: 13--16). Median duration of CD was 3.2 years (IQR: 1.7-5.1) and 63% of children were in clinical remission assessed by wPCDAI. Total IMPACT-III score was 62.8 (±11.0). The lowest score was in "emotional functioning" subdomain (mean: 42.8 ±â€Š11.2). Clinical remission was the main independent factor associated with QOL of children with CD (5.74 points higher compared with those "with active disease", 95% confidence interval [CI] 2.77--8.70, P < 0.001). Age of patient at the evaluation was found negatively correlated with QOL (-0.76 per year, 95% CI: -1.47 to -0.06, P = 0.009). Presence of psychological disorders was associated with a lower QOL (-9.6 points lower to those without, 95% CI: -13.34 to -5.86, P < 0.0001). Total IMPACT-III and its subdomains scores were not related to sex, disease duration, or treatments. CONCLUSIONS: These results not only confirm that clinical remission is a major issue for the QOL of patients, but also highlights the importance of psychological care.

2.
J Pediatr Gastroenterol Nutr ; 70(6): 820-824, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32443041

RESUMO

OBJECTIVES: Dumping syndrome (DS) is mostly described as a complication of antireflux surgery in oesophageal atresia (OA) but we previously reported 2 cases of DS before any other surgery in infants operated at birth for OA. The objectives of the present study were to assess the prevalence of abnormal oral glucose tolerance test (OGTT) at 3 months of age in infants operated at birth with type C OA, to describe symptoms and clinical features, and to assess risk factors in infants presenting with abnormal OGTT suggestive of DS. METHODS: A prospective case series study including infants with type C OA without fundoplication, born between 2013 and 2016 in 8 centres was conducted. An OGTT was performed between 2.5 and 3.5 months. Abnormal OGTT was defined as early hyperglycaemia (>1.8 g/L until 30 minutes; >1.7 g/L between 30 minutes and 2 hours; and >1.4 g/L between 2 and 3 hours) and/or late hypoglycaemia (<0.6 g/L after 2 hours). RESULTS: Eleven of the 38 OGTT (29%) showed abnormalities. None of the patients' demographics (birth weight, sex, prematurity, associated malformation, use of enteral nutrition) or conditions of the surgery tested was associated with abnormal OGTT. No clinical sign was specific for it. CONCLUSIONS: DS should be considered in every infant operated at birth for OA presenting with digestive symptoms. No risk factor was predictive for abnormal OGTT. An OGTT to screen for potential DS around 3 months of age should be considered in infants born with EA. CLINICAL TRIAL NAME AND REGISTRATION NUMBER: DUMPING NCT02525705.

3.
Hepatology ; 65(1): 164-173, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27532546

RESUMO

Some patients with microvillus inclusion disease due to myosin 5B (MYO5B) mutations may develop cholestasis characterized by a progressive familial intrahepatic cholestasis-like phenotype with normal serum gamma-glutamyl transferase activity. So far MYO5B deficiency has not been reported in patients with such a cholestasis phenotype in the absence of intestinal disease. Using a new-generation sequencing approach, we identified MYO5B mutations in five patients with progressive familial intrahepatic cholestasis-like phenotype with normal serum gamma-glutamyl transferase activity without intestinal disease. CONCLUSION: These data show that MYO5B deficiency may lead to isolated cholestasis and that MYO5B should be considered as an additional progressive familial intrahepatic cholestasis gene. (Hepatology 2017;65:164-173).


Assuntos
Colestase Intra-Hepática/sangue , Colestase Intra-Hepática/genética , Mutação , Cadeias Pesadas de Miosina/genética , Miosina Tipo V/genética , gama-Glutamiltransferase/sangue , Colestase Intra-Hepática/enzimologia , Feminino , Humanos , Lactente , Síndromes de Malabsorção , Masculino , Microvilosidades/patologia , Mucolipidoses
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