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Liver disease is increasingly common in cystic fibrosis (CF). As new therapeutic options emerge, life expectancy increases and common hepatobiliary manifestations impact on quality of life and survival of CF patients. Hepatobiliary abnormalities in CF vary in nature and range from defects attributable to the underlying CFTR gene defect to those related to(More)
BACKGROUND Published estimates on age-dependent frequency of diabetes in cystic fibrosis (CF) vary widely, and are based mostly on older data. However, CF treatment and prevention of comorbidities changed over recent years. In many studies, definition of cystic fibrosis-related diabetes (CFRD) is not in line with current guideline recommendations.(More)
We report about an 8-year-old girl with signs of elevated intracranial pressure. X-ray, CT and MRT scan showed an inner hydrocephalus as well as an intracranial calcification. By western blot analysis a Taenia solium cysticercosis could be diagnosed. In spite of spontaneous remission of the symptoms we treated with Praziquantel. Resection of the cyst was(More)
The treatment of severe pulmonary infection in young CF-adults depends on age, clinical course, bacterial colonization of the lung, susceptibility pattern and state of nutrition. Besides specific antibiotic therapy, enzyme replacement and physiotherapy, high caloric nutrition, continuous oxygen insufflation and early mobilisation are the main tools in the(More)
BACKGROUND In cystic fibrosis, highly variable glucose tolerance is suspected. However, no study provided within-patient coefficients of variation. The main objective of this short report was to evaluate within-patient variability of oral glucose tolerance. METHODS In total, 4,643 standardized oral glucose tolerance tests of 1,128 cystic fibrosis patients(More)
A photometric method to determine chymotrypsin in stool, equivalent to the titrimetric analysis, was developed. The chymotrypsin concentrations found in healthy children and those with gastrointestinal and pancreatic disease permit the same clinical conclusions as the titrimetrically determined results. In view of its lower technical requirements, this(More)
The activity levels of phosphoglyceromutase, Glucose-6-P-dehydrogenase. 3-P-Glyceratkinase and Glutathionreductase of the erythrocytes as well as 2,3-Diphosphoglycerate were determined in a total of 263 children suffering from juvenile diabetes mellitus. They were divided into two groups: 103 diabetics with a good state of metabolic control and 103(More)
A modification of the urine PABA tet published by Imondi et al. is described. Ninety minutes after oral administration of PABA peptide, PABA was determined in serum. The average concentration in healthy children was 0.42 +/- 0.055 mg per 100 ml. Up to that time we recovered in the urine 66.1 +/- 6.1% of the substance previously administered. In the children(More)