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At least 1,405 records · Page 78Linked to original sources
PubMed1998

Type II protein kinase A regulates CFTR in airway, pancreatic, and intestinal cells.

The type of protein kinase A (PKA) responsible for cystic fibrosis transmembrane conductance regulator (CFTR) activation was determined with adenosine 3', 5'-cyclic monophosphate analogs capable of selectively activating type I or type II PKA. The type II-selective pair stimulated chloride efflux in airway, pancreatic, and colonic epithelial cells; the type I-selective pair only stimulated a calcium-dependent efflux in airway cells. The type II-selective analogs activated larger increases in CFTR-mediated current than did the type I-selective analogs. Measurement of soluble PKA activity demonstrated similar levels stimulated by type I- and type II-selective analogs, creating an apparent paradox regarding PKA activity and current generated. Also, addition of forskolin after the type I-selective analogs resulted in an increase in current; little increase was seen after the type II-selective analogs. Measurement of insoluble PKA activity stimulated by the analogs resolved this paradox. Type II-selective analogs stimulated three times as much insoluble PKA activity as the type I-selective pair, indicating that differential activation of PKA in cellular compartments is important in CFTR regulation.

4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid↗
PubMed1989

Vasoactive intestinal peptide and its receptors in fetuses with cystic fibrosis.

Fetuses were investigated to establish whether vasoactive intestinal peptide (VIP) and its receptors are involved in the basic biochemical defect causing cystic fibrosis (CF). The intestine was used as a target for the disease and the liver as control. The immunoreactive and biologically active VIP contents of the colon and lower part of the small intestine were 1.5-2.5 times higher in CF fetuses than in controls. In control and CF intestinal mucosa, there was no change in the Scatchard parameters of the 125I-labeled VIP binding sites (Kd = 4.7-6.1 X 10(-11) M; Bmax = 268-280 fmol/mg protein for the high-affinity sites), in the two molecular components constituting the cross-linked 125I-VIP binding (Mr = 66,000 and 30,000), or in the pharmacological properties and functional characteristics of the VIP receptors activating the G proteins-adenylate cyclase system (Ka = 0.7 X 10(-9) M VIP). Similar results were obtained in liver. These findings suggest that neither VIP nor its receptors are involved in CF intestine. The possible involvement of other effectors related to the VIP pathway in CF intestine, including the release of VIP and adenosine 3',5'-cyclic monophosphate signal-transduction cascade, are presented.

Adenylyl Cyclases↗
PubMedDate not supplied

Kawasaki disease in an infant with cystic fibrosis.

The authors report a case of a 3-month-old infant with a very rare association: cystic fibrosis and Kawasaki disease. The clinical picture is atypical but cardiovascular signs consist of cardiomegaly, sick sinus syndrome and Q waves in D II, D III and AVF. The diagnosis is confirmed by the pathological changes found at the postmortem examination. The patient is the first case of Kawasaki disease reported in Romania.

Anti-Bacterial Agents↗
PubMed1990

Water secretion and embryological layers in cystic fibrosis.

Cystic fibrosis primarily affects tissues containing secretory epithelia, but not all of them become diseased. The distribution of clinical disease corresponds closely to the effect on net anion-linked water secretion, which is reduced across epithelia of endodermal and mesodermal origin but not those of ectodermal origin. Although the gene is expressed in all secretory epithelia, its effects on water secretion are bypassed in those of ectodermal origin.

Body Water↗
PubMed1995

A noninvasive stable-isotope method to simultaneously assess pancreatic exocrine function and small bowel absorption.

OBJECTIVE: To determine if a single-step noninvasive stable isotope method of assessing digestive function could separate normal subjects from subjects with pancreatic insufficiency (maldigestion) or small bowel dysfunction (malabsorption) and to see if subjects with maldigestion could be simultaneously separated from subjects with malabsorption. METHODS: Forty (40) normal volunteers, 18 adults with cystic fibrosis and four adults with celiac sprue, ingested a liquid test meal along with bentiromide, [13C6]PABA, and xylose (PABAX test). Serum was collected at 1 h and analyzed for PABA, [13C6]PABA, and xylose by stable isotope dilution methods using gas chromatography mass spectrometry. RESULTS: All subjects with cystic fibrosis had abnormal pancreatic function test results, whereas three of four adults with sprue had normal values of pancreatic function. All subjects with sprue had abnormal small bowel absorption tests, whereas all adults with cystic fibrosis had apparently normal intestinal function. CONCLUSION: The one-step, 1-h PABAX test can reliably separate normal subjects from those with either maldigestion or malabsorption and can also separate subjects with maldigestion from those with malabsorption.

4-Aminobenzoic Acid↗
PubMed2006

Electroconvulsive therapy for treatment of refractory depression in a patient with cystic fibrosis.

Cystic fibrosis (CF) is an autosomal recessive inherited disease that is usually first diagnosed in early childhood. It is a chronic and progressive disease in which mucus builds up and clogs passages in many body organs, but primarily the lungs and pancreas. We present a case report of a 42-year-old man with CF who was treated with electroconvulsive therapy for treatment-refractory major depressive disorder. For patients with CF, the primary issue is anesthetic management in a patient with impaired pulmonary function. Our patient received prednisone and intravenous antibiotics to optimize pulmonary status. He responded positively to the electroconvulsive therapy and was able to tolerate it without significant problems.

Adult↗
PubMed1999

Sibs diagnosed prenatally with situs inversus totalis, renal and pancreatic dysplasia, and cysts: a new syndrome?

We describe two sib fetuses with situs inversus, cystic dysplastic kidney and pancreas, bowing of the lower limbs and clavicles, severe intrauterine growth retardation (IUGR), and oligohydramnios. Early prenatal diagnosis of pancreatic and dysplastic renal cysts and situs inversus totalis were made in the 18-week-old fetus. This syndrome differs from that of Ivemark and related syndromes because of the presence of situs inversus totalis and absence of hepatic fibrosis and cysts. The parents were first cousins, and did not have any cysts of kidney, liver, or pancreas detected by ultrasonography.

Abnormalities, Multiple↗
PubMed2015

Ferret and pig models of cystic fibrosis: prospects and promise for gene therapy.

Large animal models of genetic diseases are rapidly becoming integral to biomedical research as technologies to manipulate the mammalian genome improve. The creation of cystic fibrosis (CF) ferrets and pigs is an example of such progress in animal modeling, with the disease phenotypes in the ferret and pig models more reflective of human CF disease than mouse models. The ferret and pig CF models also provide unique opportunities to develop and assess the effectiveness of gene and cell therapies to treat affected organs. In this review, we examine the organ disease phenotypes in these new CF models and the opportunities to test gene therapies at various stages of disease progression in affected organs. We then discuss the progress in developing recombinant replication-defective adenoviral, adeno-associated viral, and lentiviral vectors to target genes to the lung and pancreas in ferrets and pigs, the two most affected organs in CF. Through this review, we hope to convey the potential of these new animal models for developing CF gene and cell therapies.

Animals↗
PubMedDate not supplied

Adrenal cytomegaly: quantitative assessment by image analysis.

Adrenal cytomegaly was found in 23 or 0.8% of 2711 pediatric autopsies, including a 13-year-old girl with leukemia and a 20-year-old woman with cystic fibrosis. Cytomegaly of pancreatic islet and acinar cells and pituitary cells was also noted. DNA quantitation by image analysis indicates that cytomegalic cells of the adrenal cortex, pancreas, and pituitary contain increased amounts of DNA (hyperdiploid aneuploid), with adenocytes containing over 25 times the normal amount of DNA in some instances. These data and morphological features suggest that cytomegaly is a reflection of polyploidy due to either partial DNA replication or partial DNA replication plus other modes of polyploidization. Although the mechanism(s) by which these changes evolve remains unknown, regressive processes do not seem to be directly involved. We speculate that polyploidization is, in this setting, a response to physiological demand, a concept that also has relevance to the interpretation of morphology and DNA ploidy profiles of endocrine tumors.

Adolescent↗
PubMed1999

Regulation of Cl-/ HCO3- exchange by cystic fibrosis transmembrane conductance regulator expressed in NIH 3T3 and HEK 293 cells.

A central function of cystic fibrosis transmembrane conductance regulator (CFTR)-expressing tissues is the secretion of fluid containing 100-140 mM HCO3-. High levels of HCO3- maintain secreted proteins such as mucins (all tissues) and digestive enzymes (pancreas) in a soluble and/or inactive state. HCO3- secretion is impaired in CF in all CFTR-expressing, HCO3--secreting tissues examined. The mechanism responsible for this critical problem in CF is unknown. Since a major component of HCO3- secretion in CFTR-expressing cells is mediated by the action of a Cl-/HCO3- exchanger (AE), in the present work we examined the regulation of AE activity by CFTR. In NIH 3T3 cells stably transfected with wild type CFTR and in HEK 293 cells expressing WT and several mutant CFTR, activation of CFTR by cAMP stimulated AE activity. Pharmacological and mutagenesis studies indicated that expression of CFTR in the plasma membrane, but not the Cl- conductive function of CFTR was required for activation of AE. Furthermore, mutations in NBD2 altered regulation of AE activity by CFTR independent of their effect on Cl- channel activity. At very high expression levels CFTR modified the sensitivity of AE to 4,4'-diisothiocyanatostilbene-2, 2'-disulfonate. The novel finding of regulation of Cl-/HCO3- exchange by CFTR reported here may have important physiological implications and explain, at least in part, the impaired HCO3- secretion in CF.

3T3 Cells↗
PubMed2002

Ion channels in secretory granules of the pancreas and their role in exocytosis and release of secretory proteins.

Regulated secretion in exocrine and neuroendocrine cells occurs through exocytosis of secretory granules and the subsequent release of stored small molecules and proteins. The introduction of biophysical techniques with high temporal and spatial resolution, and the identification of Ca(2+)-dependent and -independent "docking" and "fusion" proteins, has greatly enhanced our understanding of exocytosis. The cloning of families of ion channel proteins, including intracellular ion channels, has also revived interest in the role of secretory granule ion channels in exocytotic secretion. Thus secretory granules of pancreatic acinar cell express a ClC-2 Cl(-) channel, a HCO-permeable member of the CLCA Ca(2+)-dependent anion channel family, and a KCNQ1 K(+) channel. Evidence suggests that these channels may facilitate the release of digestive enzymes and/or prevent exocytosed granules from collapsing during "kiss and run" recycling. In pancreatic beta-cells, a granular ClC-3 Cl(-) channel provides a shunt pathway for a vacuolar-type H(+)-ATPase. Acidification "primes" the granules for Ca(2+)-dependent exocytosis and release of insulin. In summary, secretory granules are equipped with specific sets of ion channels, which modulate regulated exocytosis and the release of macromolecules. These channels could represent excellent targets for therapeutic interventions to control exocytotic secretion in relevant diseases, such as pancreatitis, cystic fibrosis, or diabetes mellitus.

ATP-Binding Cassette Transporters↗
PubMed1995

[Mucoviscidosis: in children and adults].

Because the lifespan of patients with cystic fibrosis is now longer, both pediatricians and adult care physicians are involved in the health care strategy. Respiratory manifestations occur due to bronchial dilatation and chronic bronchial infection, mainly due to Staphylococcus aureus and Pseudomonas. Episodes of adult infection are frequent and death usually results from respiratory failure. Characteristically, the disease also involves exocrine pancreas insufficiency. Other intestinal tract manifestations include meconial ileus and liver disease which may reach the stage of biliary cirrhosis. Nutritional disorders are frequent. Clinically there are respiratory and digestive tract disorders, pansinusitis and frequent nasal polyposis, sometimes associated with diabetes mellitus or joint pain. Male sterility results from bilateral agenesia of the vas deferens and in the female, fertility is decreased although pregnancy is possible. Clinical presentation suggests the diagnosis which is confirmed by a sweat test and genetic analysis. Care should be provided by a centre specialized in cystic fibrosis. The main treatments rely on respiratory physical therapy, antibiotics and gastroprotected pancrease extracts.

Adolescent↗
PubMed1999

A membrane lipid imbalance plays a role in the phenotypic expression of cystic fibrosis in cftr(-/-) mice.

A deficiency in essential fatty acid metabolism has been reported in plasma from patients with cystic fibrosis (CF). However, its etiology and role in the expression of disease is unknown. The objective of this study was to determine whether alterations in fatty acid metabolism are specific to CF-regulated organs and whether they play a role in the expression of disease. A membrane lipid imbalance was found in ileum, pancreas, and lung from cftr(-/-) mice characterized by an increase in phospholipid-bound arachidonic acid and a decrease in phospholipid-bound docosahexaenoic acid (DHA). This lipid imbalance was observed in organs pathologically affected by CF including lung, pancreas, and ileum and was not secondary to impaired intestinal absorption or hepatic biosynthesis of DHA. As proof of concept, oral administration of DHA to cftr(-/-) mice corrected this lipid imbalance and reversed the observed pathological manifestations. These results strongly suggest that certain phenotypic manifestations of CF may result from remediable alterations in phospholipid-bound arachidonic acid and DHA levels.

Administration, Oral↗
PubMed2004

Disorders of the pediatric pancreas: imaging features.

The purpose of this manuscript is to provide an overview of the normal development of the pancreas as well as pancreatic pathology in children. Diagnostic imaging plays a major role in the evaluation of the pancreas in infants and children. Familiarity with the range of normal appearance and the diseases that commonly affect this gland is important for the accurate and timely diagnosis of pancreatic disorders in the pediatric population. Normal embryology is discussed, as are the most common congenital anomalies that occur as a result of aberrant development during embryology. These include pancreas divisum, annular pancreas, agenesis of the dorsal pancreatic anlagen and ectopic pancreatic tissue. Syndromes that can manifest pancreatic pathology include: Beckwith Wiedemann syndrome, von Hippel-Lindau disease and autosomal dominant polycystic kidney disease. Children and adults with cystic fibrosis and Shwachman-Diamond syndrome frequently present with pancreatic insufficiency. Trauma is the most common cause of pancreatitis in children. In younger children, unexplained pancreatic injury must always alert the radiologist to potential child abuse. Pancreatic pseudocysts are a complication of trauma, but can also be seen in the setting of acute or chronic pancreatitis from other causes. Primary pancreatic neoplasms are rare in children and are divided into exocrine tumors such as pancreatoblastoma and adenocarcinoma and into endocrine or islet cell tumors. Islet cell tumors are classified as functioning (insulinoma, gastrinoma, VIPoma and glucagonoma) and nonfunctioning tumors. Solid-cystic papillary tumor is probably the most common pancreatic tumor in Asian children. Although quite rare, secondary tumors of the pancreas can be associated with certain primary malignancies.

Adult↗