Showing papers on "Cystic fibrosis published in 1977"
TL;DR: Compared biliary lipid composition in 26 patients with cystic fibrosis, seven children with cholelithiasis but no cystic-fibrosis and 13 controls, there was a preponderance of cholic over chenodeoxycholic acid both off and on therapy, and the glycine/taurine ratio of conjugated bile acids were lower in enzyme-treated patients with Cystic Fibrosis than in patients off treatment.
Abstract: Because of the increased incidence of gallstones in cystic fibrosis we compared biliary lipid composition in 26 patients with cystic fibrosis, seven children with cholelithiasis but no cystic-fibrosis and 13 controls. Eighteen of the cystic fibrosis group had cholecystograms, and only one had gallstones. In 14 patients with cystic fibrosis who had stopped taking pancreatic enzymes for one week molar percentage of lipid composition accounted for by cholesterol (mean ± S.E., 16.3±2.9) and saturation index (2.0±0.3) were comparable to values of the cholelithiasis group and higher (P<0.01) than those of controls. In 12 patients with cystic fibrosis taking pancreatic enzymes, molar percentage of cholesterol (8.6±1.7) and saturation index (1.0±0.1) did not differ from those of controls; in cystic fibrosis there was a preponderance of cholic over chenodeoxycholic acid both off (1.7±0.2) and on (1.9±0.3) therapy as compared to the cholelithiasis (0.7±0.1) and control (0.8±0.0) groups. The glycine/taurine...
114 citations
TL;DR: The demonstration that large amounts of bile acid continue to be excreted during therapy suggests that interruption ofmore of the enterohepatic circulation continues and that deficiencies of the intraluminal phase may persist during enzyme therapy in this disease.
97 citations
TL;DR: The results of the study indicate that the force of gravity can be a major influence on tracheal mucus clearance in CF and URTI subjects, and supports the use of postural drainage as an effective form of therapy in patients with cystic fibrosis.
Abstract: A noninvasive, radionuclide imaging technique for measuring the rate of mucus clearance in the trachea (RT), was used to study gravitational effects on mucus clearance in 13 patients with cystic fibrosis (CF), average age 17 years; 7 normal, nonsmoking adults, average age 26 years; and a normal subject who was recovering from an acute upper respiratory tract infection (URTI). In the upright position, nine of the CF patients and the subject with URTI demonstrated abnormal tracheal mucus clearance which approached normal when they were placed in 25 degrees headdown position. The normal subjects and two of the CF patients showed no significant difference in the RT measured in the two positions. The results of the study indicate that the force of gravity can be a major influence on tracheal mucus clearance in CF and URTI subjects. This conclusion supports the use of postural drainage as an effective form of therapy in patients with cystic fibrosis.
96 citations
TL;DR: The percentage fatty acid composition of the various plasma-lipid fractions from cystic-fibrosis patients with and without pancreatic insufficiency, obligate heterozygotes, and normal subjects was determined as mentioned in this paper.
86 citations
Journal Article•
TL;DR: The synthesis, transport, and secretion of glycoprotein by human rectal epithelium from normal volunteers, patients with cystic fibrosis, and their disease-free siblings were studied by autoradiography of rectal biopsies pulse-labeled with 3H-glucosamine and maintained in organ culture for various intervals of up to 24 hours.
78 citations
TL;DR: Insulin and glucagon secretions were studied during oral glucose tolerance testing and arginine infusion in 13 patients with cystic fibrosis and insulinopenia was found in both groups and hyperglucagonemia was not found as a contributory factor to the hyperglycemia in Group II.
68 citations
TL;DR: Teenagers and young adults with long-standing pulmonary or digestive symptoms, unexplained cirrhosis, aspermia, or a sibling with cystic fibrosis should be sweat-tested by pilocarpine iontophoresis.
Abstract: Cystic fibrosis was diagnosed after age 13 in 25 patients. All had an elevated sweat chloride and either a sibling with cystic fibrosis or typical pulmonary infection or digestive symptoms caused by exocrine pancreatic deficiency. Fourteen had long-standing pulmonary or digestive symptoms. In contrast, four of eight patients whose symptoms began after age 13 presented with biliary cirrhosis. Three male patients were asymptomatic at diagnosis. Opacification of all paranasal sinuses was found in all patients examined radiologically. At diagnosis, pulmonary-function testing showed obstructive changes in 19 patients and sputum cultures showed Pseudomonas aeruginosa in 15 patients. Delayed menarche in five of seven female patients and infertility in the asymptomatic male patient (two of whom were found to have aspermia) could have led to earlier diagnosis. Teenagers and young adults with long-standing pulmonary or digestive symptoms, unexplained cirrhosis, aspermia, or a sibling with cystic fibrosis should be sweat-tested by pilocarpine iontophoresis.
60 citations
TL;DR: Nine lysosomal enzymes and alkaline phosphatase have been assayed with two different ultramicro techniques in the intra- and extracellular space of fibroblast cultures derived from the skin of cystic fibrosis patients, cystic Fibrosis carriers, and normal controls, to develop methods for the pre- and postnatal diagnosis of cysty fibrosis with cell cultures, and for the detection of cysts fibrosis carriers with the peripheral blood.
49 citations
TL;DR: It is found that with proper selection and preoperative preparation, a major portosystemic shunt can be done with considerable safety in a majority of cystic fibrosis patients and thus provide them with significant palliation and improved quality of life.
47 citations
TL;DR: Lymphocyte responses to the mitogens phytohemagglutinin and concanavalin A and to Streptococcus pyogenes, Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa were evaluated in patients with cystic fibrosis and in normal individuals.
Abstract: Lymphocyte responses to the mitogens phytohemagglutinin and concanavalin A and to Streptococcus pyogenes, Staphylococcus aureus, Haemophilus influenzae, and Pseudomonas aeruginosa were evaluated in patients with cystic fibrosis and in normal individuals. Lymphocyte proliferation in vitro was stimulated by gentamicin-killed whole bacteria, and the proliferative response was measured by [3H]thymidine incorporation. The in vitro lymphocyte responses to antibiotic-killed bacterial reached maximum thymidine incorporation after 5 days in culture and followed a unimodal dose-response curve for each of the bacteria studied. A significant specific incapacity to respond to P. aeruginosa was detected in cystic fibrosis patients with advanced clinical disease.
Journal Article•
TL;DR: Arachidonic acid, which was found only in trace amounts or was absent in sera of all children with CF before the clinical trial, was increased significantly in the sera after the oral administration of EFA for one year, and other findings included a significant decrease in sweat sodium and T3 in most patients.
Abstract: Analyses of serum lipids of children with cystic fibrosis (CF) have indicated a deficiency in essential fatty acids (EFA). In view of a report that intravenous administration of soybean oil emulsions normalized sweat sodium values in CF children, we studied the effects of orally administered essential fatty acids (as corn oil) for one year. Some improvement was noted in all patients, but no one consistent factor predominated. However, arachidonic acid, which was found only in trace amounts or was absent in sera of all children with CF before the clinical trial, was increased significantly in the sera after the oral administration of EFA for one year. Other findings included a significant decrease in sweat sodium and T3 in most patients.
TL;DR: In II polyps from patients with cystic fibrosis of the pancreas, the density, shape, and architecture of mucous glands were studied by the whole-mount method and compared with 102 non-cystic-fibrosis polyps which could not be distinguished.
Abstract: In II polyps from patients with cystic fibrosis of the pancreas, the density, shape, and architecture of mucous glands were studied by the whole-mount method and compared with 102 non-cystic-fibrosis polyps. There were no differences in density which was in most polyps less than 0.5 gland/mm.2. The shape and architecture as well as other histological appearances of the polyp were also alike in both groups of polyps which could not be distinguished. The pathogenesis of nasal polyps in cystic fibrosis is discussed. Apparently, it is the same as that of non-cystic-fibrosis polyps.
TL;DR: To eliminate the potential renal consequences of hyperuricosuria, it seems appropriate to control the need for increasing amounts of pancreatic enzymes by limiting the dietary intake of fat and maintaining a positive caloric and nitrogen balance with high intake of protein and carbohydrates and supplementation with medium-chain triglycerides.
TL;DR: Lipids from the particulate material obtained from pulmonary lavage of patients with asthma and cystic fibrosis were isolated and characterized and were highly saturated, containing mostly palmitic acid.
Abstract: Lipids from the particulate material obtained from pulmonary lavage of patients with asthma and cystic fibrosis were isolated and characterized. In both cases, lipids constituted 30 to 40 per cent of the dry insoluble material and phosphatidylcholine was the predominant lipid. Significant amounts of phosphatidylethanolamine and phosphatidyglycerol were present. Hexosyl ceramides, spingomyelin, phosphatidylinositol, phosphatidylserine, and lyso(bis)phosphatidic acid were present as minor lipid components. Appreciable quantities of neutral lipids were also found. Significant amounts of lysophosphatides and free fatty acids were present only in the lavage sediment from patients with asthma, and not in patients with cystic fibrosis. Phosphatidylcholine, lysophosphatidycholine, sphingomyelin, and phosphatidylglycerol of asthmatic lavage, but not cystic lavage, were highly saturated, containing mostly palmitic acid.
TL;DR: Treatment with oral pancreatic and parenteral vitamin D supplements led to full recovery of the rachitic syndrome and the proximal renal tubular dysfunction.
TL;DR: awareness of the high incidence of cholecystographic abnormalities and calculi should be helpful in the evaluation of patients with cystic fibrosis, particularly since symptoms of abdominal pain are frequent in such patients and may lead to radiographic investigations.
Abstract: In 84 consecutive patients with cystic fibrosis, oral cholecystography was abnormal in 39 (46.4%). The incidence of abnormal cholecystograms increased with patient age. The 26 patients with a nonvisualized gallbladder following double-dosage oral cholecystography were evaluated with intravenous cholangiography; 19 (70.3%) of these were abnormal. Ten patients were found to have calculi, an incidence of 11.9%. Awareness of the high incidence of cholecystographic abnormalities and calculi should be helpful in the evaluation of patients with cystic fibrosis, particularly since symptoms of abdominal pain are frequent in such patients and may lead to radiographic investigations.
TL;DR: There was a significant correlation between in vivo and in vitro rates, suggesting that tracheal pouch mucus is a good model for investigating the mucociliary flow properties of intact airway mucus.
Abstract: Tracheal mucociliary clearance was studied by a radioisotope technique in pentothal-anesthetized beagles in the control, atropinized, or dehydrated state. Mucus collected from a tracheal pouch in each dog was used for in vitro bullfrog (Rana cantesbiana) palate clearance studies and compared to the in vivo clearance rates. In all three experimental states, there was a significant correlation between in vivo and in vitro rates, suggesting that tracheal pouch mucus is a good model for investigating the mucociliary flow properties of intact airway mucus. When compared to matched controls, atropine appeared to cause a slowing of the in vivo clearance rate but not of the in vitro rate. Dehydration had no effect on either. The appropriateness of the frog palate method in the study of human respiratory disease (e.g., chronic bronchitis, cystic fibrosis) as well as its potential as an objective method of assessing the effects of various therapeutic modalities in these diseases is discussed.
TL;DR: Bile acid malabsorption in cystic fibrosis and acquired pancreatic insufficiency is minimal and probably not clinically important.
TL;DR: IgE mediation of reactions in sensitized individuals was demonstrated by antigen-induced histamine release from leukocytes, passive transfer studies, and immediate response to inhalation challenge.
Abstract: Because immediate hypersensitivity reactions can occur in individuals exposed to powdered pancreatic extracts, 36 patients with cystic fibrosis and 51 parents of such patients were studied for evidence of sensitization. Sensitivity to the extracts as evidenced by history and skin testing was infrequent in the children with cystic fibrosis. However, skin testing for immediate hypersensitivity with either crude pancreatic extracts or inactivated trypsin correlated well in their parents with a history of clinical symptoms. IgE mediation of these reactions in sensitized individuals was demonstrated by antigen-induced histamine release from leukocytes, passive transfer studies, and immediate response to inhalation challenge.
TL;DR: In a child or young adult with known or suspected cystic fibrosis, the triad of recurrent colicky abdominal pain, a mass in the right lower quadrant, and mechanical intestinal obstruction provides a clue to diagnosis of meconium ileus equivalent.
Journal Article•
Journal Article•
TL;DR: An ionophore A23187-induced increase in membrane permeability to calcium ions in culture medium produced a rabbit tracheal mucociliary response indistinguishable from that caused by cystic fibrosis (CF) sera on three different occasions.
Abstract: The Biologic Activities of Cystic Fibrosis Serum. I. The Effects of Cystic Fibrosis Sera and Calcium Ionophore A 23187 on Rabbit Tracheal Explants
TL;DR: In this article, the authors found that α-L-Fucosidase activity is elevated in skin fibroblasts from cystic fibrosis patients when compared to controls.
TL;DR: The major clinical problem in both siblings was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years, and amyloid infiltration developed in both patients, with enlargement of the thyroid gland.
Abstract: • We report two siblings with cystic fibrosis and systemic amyloidosis. The major clinical problem in both cases was recurrent respiratory infection with pulmonary fibrosis and bronchiectasis prior to death at ages 20 and 22 years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid infiltration developed in both patients, with enlargement of the thyroid gland, and one required thyroidectomy. An autopsy review of 17 additional cases of cystic fibrosis failed to disclose any other instances of systemic amyloidosis. ( Am J Dis Child 131:886-888, 1977)
TL;DR: Amikacin is clinically effective in treating Pseudomonas-associated pulmonary infections complicating cystic fibrosis and activity against many gentamicin-resistant strains and high blood levels are among the attractive properties of this antibiotic.
Abstract: Amikacin, a new semisynthetic aminoglycoside antibiotic with activity against Pseudomonas aeruginosa, was used to treat 22 acute exacerbations of chronic pulmonary infections in 18 patients with cystic fibrosis. Patients ranged from 5 to 32 years of age and had mucoid P. aeruginosa isolated from sputum. The amikacin dose was usually 7.5 mg/kg every eight hours but was increased to 10 mg/kg and/or carbenicillin was added in selected cases depending on clinical course. Although P. aeruginosa was not eliminated from our patients' sputum except in two cases, there was a good clinical response in 19 of 22 courses. Significant improvement in chest x-ray films, spirometry, or arterial oxygen tension was documented in 11 of 17 courses. One instance of serum creatinine level elevation could not be attributed to this antibiotic. Two patients showed minimal (15 dB) unilateral high-frequency hearing loss on serial audiograms. Activity against many gentamicin-resistant strains and high blood levels are among the attractive properties of amikacin. Amikacin is clinically effective in treating Pseudomonas-associated pulmonary infections complicating cystic fibrosis.
TL;DR: Eine Intrinsic Factor-nichtresponsive Vitamin B12-Malabsorption wurde bei allen untersuchten Kindern mit zystischer Fibrose festgestellt and ist möglicherweise auf die Notwendigkeit eines proteolytischen Pankreasenzymes (Trypsin?) für die Bindung des Vitamin B 12-IntrinsIC Factor-Komplexes
Abstract: The absorption of biosynthetically59Fe-labeled hemiglobin, pork and hog liver,60Co-vitamin B12 and75Se-selenomethionin labeled pork was measured in children with cystic fibrosis by whole body counting of the absorbed radionuclides within the 4π geometry of a large volume radioactivity detector with liquid organic scintillator Children with normal or mildly reduced iron stores absorbed 6–13% of the 5 mg hemiglobin-iron, 7–14% of the 5 mg liver-iron and 17–34% of the pork-iron whereas children with depleted iron stores absorbed 19% of the hemiglobin-59Fe, 13 and 28% of the liver-59Fe and 23 and 30% of the pork-59Fe These absorption values are all within the ranges of hemiglobin-, liver- and pork-59Fe absorption described for healthy adults with normal and depleted iron stores, respectively Pancreatic proteolytic digestion is obviously not necessary for the intraluminal release preceeding the absorption of dietary heme- and non heme-iron from hemiglobin, meat and liver, respectively An increased absorption of hemiglobin-, liver-and pork iron is caused by depleted iron stores also in children with cystic fibrosis An intrinsic factor non-responsivevitamin B
12-malabsorption was observed in all investigated children with cystic fibrosis and confirms the requirement of a pancreatic proteolytic enzyme (trypsin) for the vitamin B12-intrinsic factor binding reaction or the fixation of the vitamin B12-intrinsic factor complex to the ileal receptors Children with cystic fibrosis absorbed 60 and 71% of the75Se from an oral dose of 200 g75
Se-selenomethionine labeled pork, whereas normal adults absorbed 76–100% (
$$\bar X_1 $$
1±SD=87±35) of the75Sc Pancreatin did not improve the bioavailability of75Se-selenomethionine from labeled pork in cystic fibrosis which is just below the normal range Intraluminal peptic digestion of dietary protein seems to be adequate in pancreatic insufficiency and pancreatin treatment did not improve protein utilization in cystic fibrosis
TL;DR: There is adequate background data to conclude that several pancreatic diseases of obscure etiology may be due in part to hitherto unidentified toxic effects of chemical agents encountered in personal or general environments.
Abstract: The five major diseases of the pancreas together make a significant contribution to morbidity and mortality among the people of the United States. These diseases are diabetes, cystic fibrosis, acut...
TL;DR: Using a modified rabbit tracheal bioassay, it is confirmed that serum from individuals with bronchial asthma and serum from homozygotes and heterozygotes for CF cause ciliary dyskinesia, whereas normal healthy control sera do not.
Abstract: CYSTIC fibrosis (CF) homozygotes and heterozygotes carriers for CF harbour a factor in their serum (termed CDF) which causes ciliary dyskinesia when applied to ciliated epithelial tissue derived from rabbit trachea1–4. Spock et al.1 reported that serum ciliary dyskinesia activity (CDA) was a specific marker for the CF gene. Conover et al.5 have reported that a CDA is also found in sera from individuals with various respiratory and autoimmune disorders; they have since suggested that the CF-ciliary dyskinesia factor was C3a (anaphylatoxin), and proposed that a defect or deficiency in the anaphylatoxin inactivator was the primary gene defect in cystic fibrosis6,7. Other laboratories have been unable to detect CF-CDF using a rabbit tracheal bioassay for the detection of the CF-CDF (refs 8–10). Using a modified rabbit tracheal bioassay, we have confirmed that serum from individuals with bronchial asthma and serum from homozygotes and heterozygotes for CF cause ciliary dyskinesia, whereas normal healthy control sera do not. Most individuals with asthma, however, were also found to have serum which causes a subsequent ciliostasis. All sera tested by bioassay for CFP by electrofocusing indicated that all CDA-positive sera except sera from seven of eight individuals with asthma were also positive for cystic fibrosis protein (CFP). The CFP is a small (molecular weight 5,000–9,000), positively charged compound and is found in sera from most homozygotes and heterozygotes for CF (refs 11–16). These two findings suggested that the CDAs in CF sera and in asthmatic sera could be due to two different substances. We have developed two simple chromatographic techniques which can separate CDAs in CF and asthmatic sera. Our results indicate that they may be useful in purifying a CF-specific CDA. Purification of this CF-CDA is a prerequisite for its biochemical characterisation and for elucidating its role in the pathophysiology of CF (ref. 17).