Patients often present with signs of hepatic shunting (encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, and/or hypoglycemia) with relative sparing of the synthetic liver function in the absence of portal hypertension. Some shunts present with space-occupying lesions (focal nodular hyperplasia, hepatocellular carcinoma, nodular regenerative hyperplasia, etc.) or biliary atresia. Finally, some cases are detected incidentally.
The laparoscopic Nissen fundoplication can safely be performed with a low conversion rate and no surgical mortality in neurologically normal and neurologically impaired children. Neurologically impaired children are more susceptible to per- and postoperative complications. A good quality of life and a high index of satisfaction could be achieved in most patients.
In our daily practice study, RTX provided good longterm disease control and treatment retention in refractory patients with RA. Over the years, rheumatologists tended to start this treatment in patients with fewer previous anti-TNF treatments and lower disease activity.
LES pressure and esophageal velocity decreased with increasing age. Esophageal manometry is severely impaired in children with esophageal atresia and psychomotor retardation. No specific esophageal motor abnormalities related to the presence of endoscopic esophagitis or abnormal pH monitoring were found.
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