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At least 685 records · Page 38Linked to original sources
PubMed2005

Down-regulation of the anti-inflammatory protein annexin A1 in cystic fibrosis knock-out mice and patients.

Cystic fibrosis is a fatal human genetic disease caused by mutations in the CFTR gene encoding a cAMP-activated chloride channel. It is characterized by abnormal fluid transport across secretory epithelia and chronic inflammation in lung, pancreas, and intestine. Because cystic fibrosis (CF) pathophysiology cannot be explained solely by dysfunction of cystic fibrosis transmembrane conductance regulator (CFTR), we applied a proteomic approach (bidimensional electrophoresis and mass spectrometry) to search for differentially expressed proteins between mice lacking cftr (cftr(tm1Unc), cftr-/-) and controls using colonic crypts from young animals, i.e. prior to the development of intestinal inflammation. By analyzing total proteins separated in the range of pH 6-11, we detected 24 differentially expressed proteins (>2-fold). In this work, we focused on one of these proteins that was absent in two-dimensional gels from cftr-/- mice. This protein spot (molecular mass, 37 kDa; pI 7) was identified by mass spectrometry as annexin A1, an anti-inflammatory protein. Interestingly, annexin A1 was also undetectable in lungs and pancreas of cftr-/- mice, tissues known to express CFTR. Absence of this inhibitory mediator of the host inflammatory response was associated with colonic up-regulation of the proinflammatory cytosolic phospholipase A2. More importantly, annexin A1 was down-regulated in nasal epithelial cells from CF patients bearing homozygous nonsense mutations in the CFTR gene (Y122X, 489delC) and differentially expressed in F508del patients. These results suggest that annexin A1 may be a key protein involved in CF pathogenesis especially in relation to the not well defined field of inflammation in CF. We suggest that decreased expression of annexin A1 contributes to the worsening of the CF phenotype.

Adolescent↗
PubMed1980

Cystic fibrosis in South India.

Cystic fibrosis has been diagnosed during life in three South Indian infants on the basis of characteristic clinical features and a positive sweat test. The patients were respectively 81 days, 23 days and 6 months old. All three presented with the rare characteristic triad of gross oedema, hypoproteinaemia and moderate to severe anaemia; it is described for the first time from South India. Three patients were exclusively breast-fed; the third received complements of diluted cow's milk. Sweat sodium and chloride were elevated in the first two cases and was normal in the third. All three died with progressive deterioration 10, 31 and 7 days respectively after admission in the hospital. At autopsy, changes typical of cystic fibrosis were present in pancreas, lung, liver and the small intestine of one, in the lungs of the second and in pancreas and liver of the third case.

Cystic Fibrosis↗
PubMed1987

The increased echogenicity of the pancreas in infants and children: the white pancreas.

An increased echogenicity of the pancreas ("white pancreas") was sonographically found in 25 children with various pancreatic and systemic diseases. Fifteen patients with cystic fibrosis had a small white pancreas. Five patients with haemosiderosis, two with pancreatitis and one with Shwachman-syndrome presented with a normal-sized or slightly enlarged pancreas. Fatty infiltration and calcifications of the pancreas can also increase its echogenicity.

Acute Disease↗
PubMed1996

Pancreatic dysfunction in cystic fibrosis occurs as a result of impairments in luminal pH, apical trafficking of zymogen granule membranes, and solubilization of secretory enzymes.

Recent progress in understanding the luminal biochemistry of regulated pancreatic exocrine secretion, including acid-base interactions between acinar and duct cells and pH-dependent processes that regulate membrane trafficking (endocytosis) at the apical plasma membrane, have led to the development of in vitro models of cystic fibrosis in the rat exocrine pancreas. Based on investigations in these model systems, a unifying hypothesis is presented that proposes that pancreatic dysfunction in cystic fibrosis occurs as a result of progressive acidification of the acinar and duct lumen, which leads to secondary defects in (i) apical trafficking of zymogen granule membranes and (ii) solubilization of secretory (pro)enzymes. By directly acidifying the pH of the acinar lumen in cholescystokinin-stimulated acini, the early cytological findings observed in cystic fibrosis, including (i) massive dilatation of the acinar lumen, (ii) decreased appearance of zymogen granules, (iii) loss of the apical pole of the acinar cell, and (iv) persistent aggregation of secretory (pro)enzymes released into the luminal space, have been reproduced in primary cultures of pancreatic tissue.

Animals↗
PubMed1975

[The acidity and peptic activity of gastric juice in healthy children and in children suffering from cystic fibrosis (author's transl)].

The acid concentration and quantity, the pH and the peptic activity of the gastric juice were measured after stimulation with pentagastrin in 10 children with cystic fibrosis between the ages 2 and 12 years and in 20 healthy children of the same age group. Furthermore, the basal, maximal and peak volume outputs (BVO, MVO and PVO), the basal, maximal and peak acid outputs (BAO, MAO and PAO) and the basal, maximal and peak pepsin output (BPO, MPO and PPO) were determined. The statistical calculations were carried out with the help of partial hierarchical analysis of variance, comparison of regression curves, simple analysis of covariance and the t test. After stimulation with pentagastrin, the volume of the gastric juice, the acid quantity and the peptic activity were found to be dependent on age in healthy children as well as in children with cystic fibrosis. The maximal volume of secretion in children with cystic fibrosis is less than that of healthy children; however, the acid quantity and peptic activity show no significant difference in both groups. The volume of the gastric juice, acid quantity and peptic activity in basal and stimulated secretions, expressed in kilograms per body weight or surface area in square meters, are independent of age and show no significant difference between the two groups. In the two groups the curves for the three parameters differ significantly from one to another. There is a significant shift in the time course of the curves that depict the acid secretion and peptic activity. Contrary to the accepted views, the acid and enzyme secretions are not closely interrelated. Based on the acidity and peptic activity, the digestive capacity of the stomach is the same for healthy children and children with cystic fibrosis. In contrast to the pancreas, there is no impairment in the exocrine function of the stomach. The gastric secretions of children with cystic fibrosis are not completely the same as in healthy children.

Body Surface Area↗