Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The incidence of iron deficiency and its relationship with the concentration or iron in sputum and the number of Pseudomonas aeruginosa (PA) colonies was studied in an unselected group of 53 cystic fibrosis (CF) patients with an age range of 3 months to 21 years. Parameters used to assess the iron status included serum iron, the % saturation of transferrin (n = 53). The number of subjects with depletion of iron stores was estimated by levels of ferritin (n = 50). The concentration of iron and of PA was measured in a subgroup (n = 24) and compared to a control group (n = 8) with pulmonary infections of varying etiology. A close correlation was found between serum iron and the % saturation of transferrin (r = 0.952; p less than 0.001). Between 22.6 to 28.3% of patients were found to be iron deficient. An abnormally low ferritin (less than 12 ng/ml) was noted in 28% of cases but no correlation could be established between changes of serum iron and ferritin levels as a function of the degree of infection and/or of inflammation. In 62% of cases (n = 15) the concentration of iron in sputum was found to be within the range of control values (12-27 mumols/l). In 38% of cases (n = 9), ferritin values were above 27 mumols/l. No correlation was found between the concentration of iron and the number of PA colonies in sputum. We can therefore conclude the following: 1) iron deficiency is more common in CF than previously reported; 2) ferritin levels constitute a poor index of iron deficiency; 3) colonisation with PA is not associated with iron content of bronchial secretions.
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PMID:[Iron deficiency and Pseudomonas aeruginosa colonization in cystic fibrosis]. 250 26

Iron deficiency has been reported in one third of patients with cystic fibrosis. There are data that suggest that iron absorption is increased with exocrine pancreatic deficiency and that administration of pancreatic enzymes may impair oral iron absorption. We compared oral iron absorption over a 3-hour period in the presence and absence of exogenous pancreatic enzymes in 13 stable young-adult patients with cystic fibrosis and 9 age-matched control patients. Although none of the patients with cystic fibrosis had a hemoglobin level less than 119 g/L, serum ferritin levels were less than 25 micrograms/L in 5 of the 13 patients, and the mean corpuscular volume was significantly lower in the patient group (86.1 +/- 2.7 vs 90.9 +/- 5 fL). Baseline mean serum iron levels were higher in controls (18.9 +/- 5.9 mumol/L) than in patients (11.9 +/- 6.3 mumol/L). There was no difference in iron absorption in the absence of exogenous pancreatic enzymes. Significant impairment of iron absorption was detected in both patients with cystic fibrosis and controls after administration of a preparation of pancreatic enzymes. There was an inverse relationship between iron stores, as measured by serum ferritin, and iron absorption. These findings suggest that long-term consumption of pancreatic enzymes by patients with cystic fibrosis may contribute to iron deficiency.
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PMID:Effect of pancreatic enzyme supplements on iron absorption. 275 73

Although the basic biochemical defect in cystic fibrosis (CF) is unknown, previous studies have indicated that errors in protein glycosylation may be involved in the pathogenesis of the disease. Utilizing human skin fibroblasts, the present study was designed to quantitatively analyze glycosylation of cell surface glycoconjugates in CF and normal cells. Cell surface glycoconjugates were analyzed using 125I-concanavilin A (Con A), 125I-WGA, and Con A-ferritin conjugates. Under our binding conditions, Con A was used as a probe for mannose residues and WGA was used as a probe for N-acetylglucosamine residues. Saturable binding of both probes was observed and appropriate sugar controls confirmed the specificity of each lectin. When compared on a DNA basis, iodinated lectin binding studies indicated that no consistent differences existed between CF and normal strains of human skin fibroblasts. Ultrastructural quantitative morphometric analysis of Con A-ferritin conjugate binding indicated that neither proteolysis of cell surface glycoconjugates or internalization of lectin probes was occurring at saturable binding concentrations. In summary, our results indicated that no consistent differences in cell surface mannose and N-acetylglucosamine residues could be detected between the normal and CF strains of human skin fibroblasts used in these studies.
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PMID:Analysis of cell surface glycoconjugates in fibroblasts from patients with cystic fibrosis. 337 Aug 43

Iron state was measured as part of the comprehensive assessment of 165 patients with cystic fibrosis. Of 127 patients, 41 (32%) had low serum ferritin concentrations and at least this proportion were iron deficiency. Iron state did not correlate with clinical score, radiological score, or results of sputum culture. There was no evidence that patients with iron deficiency were either in better or worse clinical condition than those with better iron stores.
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PMID:Iron deficiency in cystic fibrosis. 382 96

We investigated 28 cystic fibrosis (CF) patients to determine why hypoxia from their obstructive pulmonary disease does not produce polycythemia. Oxygen saturation was lower and erythropoietin levels were higher in CF patients than in 25 age-comparable reference subjects (90.8% and 47 mimu vs. 94.7% and 29 mimu, p less than 0.01). Hematocrit and red blood cell (RBC) indices were not different between groups. Serum vitamin and iron levels, ferrokinetics, RBC volume, and RBC survival were studied in 10 of the 28 CF patients. Total iron-binding capacity and vitamin E levels were low, and serum iron, ferritin, vitamin B12, and folate levels were normal in these patients. Red blood cell survival was minimally decreased in six patients although there was no other evidence for hemolysis. Ferrokinetics (59Fe) indicated a reduction in total erythropoiesis in only two patients. Plasma volume was high-normal in five and above normal in four CF patients; RBC mass was increased appropriately for each patient's degree of hypoxia, when compared to healthy individuals living at different altitudes. These results suggest that CF patients are able to compensate for hypoxia by increasing RBC mass; however, an expanded plasma volume prevents a detectable rise in hematocrit.
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PMID:Ferrokinetic and hematologic studies in cystic fibrosis patients. 661 95

Significant alterations in hemotologic function in cystic fibrosis are suggested by the observation that polycythemia is uncommon, even among cyanotic patients. To elucidate those factors that influence hematologic equilibrium, 39 stable patients with cystic fibrosis were evaluated with regard to hemoglobin, hematocrit, RBC indices, reticulocyte count, serum iron and total iron binding capacity, serum ferritin, vitamin E, and carboxyhemoglobin levels. Hemoglobin concentrations were below the 50th percentile for age in 90% of the patients, including the 23% who were cyanotic. Serum ferritin levels were below the mean for age in 85% and below 12 ng/mL in 33% of patients. Vitamin E levels were less than 5 micrograms/dL in 33%, indicating deficiency. Carboxyhemoglobin values were elevated in 64% of the patients. These data indicate that relative anemia is common in cystic fibrosis and suggest that iron and vitamin E deficiency may contribute to that anemia. Twenty-two patients with cystic fibrosis were then given 2 weeks of oral iron therapy followed by two to three additional weeks of iron and vitamin E. This therapeutic trial resulted in an increase in mean hemoglobin concentration from 13.87 to 14.50 g/dL (P less than 0.01) associated with a significant increase in levels of serum ferritin (P less than 0.001). The increase in hemoglobin occurred primarily during the second 2 weeks when patients were receiving both iron and vitamin E. However, we were unable to document evidence of increased hemolysis when patients were receiving iron therapy alone. This response to oral iron therapy is confirmation that iron deficiency contributes to the failure of some patients with cystic fibrosis to compensate hemotologically for hypoxia.
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PMID:Relative anemia and iron deficiency in cystic fibrosis. 683 67

Cystic fibrosis patients are at risk for nutrient deficiencies from malabsorption related to exocrine pancreatic insufficiency. This research examined the copper homeostasis of children with cystic fibrosis. Our objective was to measure cytochrome oxidase and copper-zinc superoxide dismutase activities in mononuclear cells, neutrophils, and erythrocytes of adolescents with cystic fibrosis, as well as plasma copper and ceruloplasmin. Thirteen adolescents with pancreatic insufficiency caused by cystic fibrosis were compared with 10 age- and sex-matched control subjects. Serum copper concentrations and ceruloplasmin measurements were not significantly different between the two groups. Cytochrome oxidase activity was significantly lower in the mononuclear cells and copper-zinc superoxide dismutase activity was significantly lower in the neutrophils and erythrocytes of the cystic fibrosis group. Other measures of trace element status such as hemoglobin concentration, serum ferritin, serum zinc, glutathione peroxidase activity, and manganese superoxide dismutase activity were not different between the two groups. Reductions in the activity of two copper-dependent enzymes suggest abnormal copper homeostasis in this population.
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PMID:Reduced copper enzyme activities in blood cells of children with cystic fibrosis. 766 Nov 26

Functional iron deficiency (transferrin saturation < 16%) was found in 44 (62%) of 71 adult cystic fibrosis (CF) patients. Haemoglobin concentration and mean cell volume were lower in iron-deficient patients, in whom there was a non-significant trend for lower serum ferritin. Ten iron-deficient patients and two patients with transferrin saturation > = 16% (normal iron) were anaemic. There were no significant differences between iron-deficient and normal-iron patients in intake of calories, protein, iron and vitamin C as determined by 4-day records of dietary intake. Dietary iron deficiency is not an important factor in functional iron deficiency in adult CF patients. Impairment of absorption by exogenous pancreatic enzyme supplements is unlikely to be significant as enzyme intake was the same in the two groups. Iron-deficient patients had lower Shwachman-Kulczycki scores and lower percent predicted forced expiratory volume in 1 s (FEV1% predicted) and forced vital capacity (FVC% predicted). There was a non-significant trend for higher values of white cell count and plasma viscosity in the iron-deficient group. Chronic inflammation is likely to be the primary cause of functional iron deficiency in adult CF patients. Fifteen patients completed 3-month courses of oral iron replacement with no deterioration in pulmonary function, but with no effect on haemoglobin concentration.
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PMID:Functional iron deficiency in adults with cystic fibrosis. 879 33

The target cells for gene therapy of cystic fibrosis lung disease are the well differentiated cells that line airway lumens. Employing cultures of airway epithelial cells that grow like "islands" and exhibit a continuum of cellular differentiation, we studied the mechanisms that render well differentiated cells more difficult to transfect with cationic liposomes than poorly differentiated cells. The poorly differentiated cells at the edge of the islands were transfectable with liposome-DNA complexes (pCMVbeta:LipofectACE = 1:5 (w/w)), whereas the more differentiated cells in the center of the islands were not. Evaluation of the steps leading to lipid-mediated transfection revealed that edge cells bound more liposome-DNA complexes, in part due to a more negative surface charge (as measured by cationized ferritin binding), and that edge cells internalized more liposome-DNA complexes than central cells. Edge cells exhibited receptor-mediated endocytosis of LDL, pinocytosis of 10-nm microspheres, and phagocytosis of 2-microm microspheres, whereas central cells were only capable of receptor-mediated endocytosis. Cytochalasin B, which inhibited pinocytosis by 65% and phagocytosis by 93%, decreased edge cell liposome-DNA complex entry by 50%. Potassium depletion, which decreased phagocytosis by >90% but had no effect on pinocytosis, inhibited edge cell liposome-DNA complex entry by 71%. These results indicate that liposome-DNA complexes enter edge cells via phagocytosis and that this pathway is not detectable in central cells. In conclusion, both reduced negative surface charge and absence of phagocytosis internalization pathways in relatively differentiated cells may explain differentiation-dependent decrements in cationic liposome-mediated gene transfer in airway epithelia.
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PMID:Loss of binding and entry of liposome-DNA complexes decreases transfection efficiency in differentiated airway epithelial cells. 899 11

Poor nutritional status in patients with cystic fibrosis (CF) is associated with increased mortality. Patients with CF often have a decreased sensation of smell secondary to recurrent sinus infections or sinus surgery; in other CF populations, a decreased sensation of smell has been associated with poor nutritional status. We hypothesized that a decreased sensation of smell would be associated with worse nutritional status in patients with CF. We studied 50 (26 F and 24 M) of 58 consecutive patients with CF (86%) aged 14-53 years (28 +/- 8; mean +/- SD) who attended the University of Washington Medical Center from June 1994 to March 1995 and who agreed to participate. Demographic information was obtained, and nutritional status was assessed by ideal body weight, arm muscle area, arm fat area, pancreatic sufficiency, insulin-requiring diabetes, vitamins A and E levels, albumin, iron, iron binding capacity, ferritin, cholesterol, and zinc levels. Objective sensation of small was examined (Sensonics, Philadelphia, PA), a sinus compacted tomogram (CT) was performed, and a questionnaire for prior sinus symptoms, sinus surgery, medications, and subjective sensation of smell was administered. Twenty-seven of 49 subjects (55%) had an objective decrease in sensation of smell, 23/50 (46%) had had prior sinus surgery. 46/50 (92%) were pancreatic insufficient, and 8/50 (16%) were insulin-requiring diabetics. Weight for height ranged from the 38th to 157th percentile (100 +/- 18; mean +/- SD). Arm muscle area ranged from the < 5th to the 75th percentile (25 +/- 23; mean +/- SD). Arm fat area ranged from the < 5th to the 95th percentile (45 +/- 39; mean +/- SD). Sinus CT scans were abnormal in all patients (100%). Patients with anosmia were more likely to have had sinus surgery, but their nutritional status was no different from that of patients with a normal sensation of smell. We conclude that decreased sensation of smell is common in patients with CF, especially those with prior sinus surgery. Subjective sensation of smell and sinus CT scans were unreliable indicators of a decreased objective sensation of smell. In this pilot study, no association was found between sensation of smell and nutritional status.
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PMID:Sensation of smell does not determine nutritional status in patients with cystic fibrosis. 926 54


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