Sonographic evaluation of bowel wall thickness in patients with cystic fibrosis.

Goals And Background: Fibrosing colonopathy causing severe thickening of the colon wall was recently described in cystic fibrosis (CF). Since it has been suspected that subclinical colon-wall thickening is a common feature in CF patients, bowel-wall thickness was measured in a series of patients and compared with controls. Additionally, possible clinical factors influencing wall thickness were investigated.

Oral mannose therapy persistently corrects the severe clinical symptoms and biochemical abnormalities of phosphomannose isomerase deficiency.

Phosphomannose isomerase (PMI) deficiency (CDG-Ib) is a newly recognized disorder of mannose and glycoprotein metabolism. PMI deficiency manifests itself mainly as a gastrointestinal disorder with protein-losing enteropathy and life-threatening intestinal bleeding. Hypoglycaemia is an additional prominent symptom.

Biliary atresia: which factors predict the success of a Kasai operation? An analysis of 36 patients.

Unlabelled: Biliary atresia (BA) is one of the most frequent causes of neonatal cholestasis. Portoenterostomy is one therapeutic option in these patients with a success rate of 30-40%. To answer the question of therapy liver transplantation or Kasai operation - we analyzed 36 consecutive patients being followed in our center during the past 7 years.

Renal function and renotropic effects of secretin in cystic fibrosis.

In ten cystic fibrosis patients and nine age-matched controls, renal function was determined before and after infusion of secretin. Under baseline conditions creatinine excretion and clearance were significantly elevated, exclusively due to those patients who were homozygous for the DF508 mutation (153 vs 132 ml/min*1.73m2), whereas the glomerular filtration rate, measured by inulin clearance showed no difference.