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OBJECTIVE
Systemic sclerosis (SSc) is characterized by fibrosis of the skin and visceral organs. Patients with SSc have enhanced plasma levels of the plasmin-α2-antiplasmin (α2AP) complex, and we recently implicated α2AP in the development of fibrosis through transforming growth factor β (TGFβ)
Metabolic disorders are characterized by pathologies like visceral adiposity, hypertension, type 2 diabetes mellitus (T2DM), dyslipidemia, impaired glucose tolerance, fatty liver, and so on, with insulin resistance being the main contributing factor. Insulin resistance and diabetes mellitus are
OBJECTIVE
The authors describe here the occurrence of low fasting serum triglyceride (TG) and high free fatty acid (FFA) levels in pulmonary fibrosis, a finding that has never been reported before.
METHODS
TGs were measured in: (a) 44 patients (3 male and 41 female; mean age SEM: 63.06 +/- 4.04
Serum levels of high-density lipoprotein cholesterol are often increased in patients with primary biliary cirrhosis. To elucidate the mechanism of the elevation of high-density lipoprotein cholesterol levels in this disease, lipoprotein abnormalities were analyzed in 10 patients subdivided into two
Children with cystic fibrosis have variable degrees of exocrine pancreatic insufficiency which, if untreated, is the main cause of fat malabsorption. The impact of pancreatic enzyme supplementation on fat digestion was measured in 41 children with cystic fibrosis, 11 healthy controls, and five
BACKGROUND
Fat maldigestion occurs in most patients with cystic fibrosis. Conventional pancreatic enzyme replacement therapy partially corrects this defect. In this study, the mixed-triglyceride breath test was used to evaluate whether high-lipase enzymes are equivalent to conventional enzymes in
BACKGROUND
The 'gold standard' test for the indirect determination of pancreatic function status in infants with cystic fibrosis (CF), the 72-hour fecal fat excretion test, is likely to become obsolete in the near future. Alternative indirect pancreatic function tests with sufficient sensitivity and
Werner Syndrome (WS) is a rare disorder characterized by the premature onset of a number of age-related diseases. The gene responsible for WS encodes a DNA helicase/exonuclease protein. Previously, we generated a mouse model lacking part of the helicase domain of the murine Wrn homologue. Mutant
Despite guidelines suggesting pancreatic enzyme replacement therapy (PERT) should be taken before or during a meal, it is currently unknown whether this has benefits over administration after a meal in individuals with cystic fibrosis (CF).
18 children with pancreatic insufficient CF were randomised
The 13C-mixed triglyceride (13C-MTG) breath test (BT) is a safe and noninvasive method to measure exocrine pancreatic function. We examined the reproducibility of the 13C-MTG BT in a group of 17 healthy controls and 8 adult patients with cystic fibrosis (CF). In controls no statistically significant
Calcium absorption, as measured by whole-body retention of isotopic (47)calcium, was investigated in 10 controls and 10 patients with primary biliary cirrhosis before and after medium-chain triglyceride therapy. Absorption of calcium was impaired in eight patients with primary biliary cirrhosis,
BACKGROUND
Elevated fasting triglyceride is often associated with metabolic syndrome and non-alcoholic fatty liver disease (NAFLD), the most common form of chronic liver disease. On the other hand, as liver disease progresses, patients may develop hepatocellular dysfunction that impairs triglyceride
Conventional treatment of 15 children (aged 4 to 17 years) with cystic fibrosis and persistent failure to thrive was supplemented on an out-patient basis by a daily oral intake of 35 g of medium chain triglycerides (MCT) fat. Follow-up investigations were pursued for a longer period than described
BACKGROUND
For indirect evaluation of pancreatic lipase activity in cystic fibrosis, different 13C-labeled triglycerides may be used.
METHODS
Triglyceride oxidation in patients with cystic fibrosis was investigated after administration of different 13C-labeled triglycerides by comparing 13CO2 breath
Cystic fibrosis (CF) is characterized by abnormal levels of essential fatty acids (EFA) in plasma phospholipids. The reduced availability of EFA has been reported to alter patterns of circulating and tissue esterified acids and may determine profound changes in membrane fluidity and cell signaling