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Biomedical subjects

S Quattrucci

Publications and source records attributed to S Quattrucci.

At least 19 recordsLinked to original sources

Increased plasma levels of adrenomedullin, a vasoactive peptide, in patients with end-stage pulmonary disease.

AIM: To study adrenomedullin (AM) plasma levels in patients with severe lung disease and to analyze the relationship between AM and heart changes, hemodynamics and blood gases. METHODS: Case control study of 56 patients (36 men, 20 women) with severe lung disease and 9 control subjects (7 men, 2 women). Patients with end-stage pulmonary disease, including chronic obstructive pulmonary disease (COPD, n=11), cystic fibrosis (CF, 26), idiopatic pulmonary fibrosis (ILD, n=9), and idiopatic pulmonary arterial hypertension (PAH, n=10), who were evaluated for lung trasplantation between January 1997 and September 2000, and nine patients who underwent lung surgery for a solitary benign nodule. AM plasma levels in pulmonary artery (mixed venous blood, vein) and aorta or femoral artery (arterial, art), art and vein blood gases, pulmonary hemodynamics, systemic hemodynamics, two-dimensional transthoracic echocardiography and echo-Doppler study. RESULTS: Plasma AM (art and ven) levels were higher among patients' group compared to the controls (AMart p<0.02 and AMven p<0.04) for CF, ILD, PAH (AMart, pg ml(-1) Controls 13.7+/-3.6, COPD 22.8+/-6.2, CF 28.1+/-11.4, ILD 34.1+/-14.3, PAH 35.1+/-18.9; AMven, pg ml(-1) Controls 14.2+/-4.8, COPD 28.1+/-12.6, CF 31.7+/-14.1, ILD 38.7+/-16.5, PAH 40.1+/-4.4). We found with a trend towards higher concentration in ILD and PAH patients compared to COPD and CF but no statistical significant differences. Mixed-venous AM was higher than arterial AM in all groups resulting in AM uptake (AMPulmUp pg min(-1) Controls 4.8+/-22.6, COPD 21.1+/-44.9, CF 20.6+/-45.1, ILD 23.7+/-38.5, PAH 29.9+/-49.7). The univariate analysis showed a weak but significant correlation between AMart and mean systemic arterial pressure, heart rate, mean pulmonary arterial pressure and systemic vascular resistance. In the multivariate analysis, four variables emerged as independent factors of AMart including mean pulmonary arterial pressure, heart rate, mean systemic arterial pressure and left ventricular diastolic diameter (F=8.6, p<0.00001, r=0.60, r2=0.32). A similar weak correlation was apparent between AMven, systemic vascular resistance, and mean pulmonary arterial pressure. The results of multivariate analysis identify right atrial enlargement, mean right atrial pressure, heart rate and left ventricular dimensions as the only independent variables related to AMven (F=4.3, p<0.0004 r=0.56, r2=0.26). AM pulmonary uptake was significantly correlated with AMven (r=0.65), but not with hemodynamic, blood gas and echocardiographic variables. CONCLUSIONS: AM plasma levels are elevated in patients with severe lung disease in face of a preserved pulmonary uptake. These results suggest that the high AM plasma levels in patients with severe lung disease are not caused by a reduced pulmonary clearance, instead suggesting a systemic production.

Adrenomedullin↗

Spondylodiscitis after bilateral sequential lung transplantation in a patient with cystic fibrosis.

We report the case of a 24-year-old woman with cystic fibrosis in whom spondylodiscitis developed after bilateral sequential transplantation. The diagnostic work-up included magnetic resonance imaging, computed tomography-guided disk biopsy, histological examination, and cultures of disk specimens. The infective organism was D group Streptococcus and the patient was successfully treated with intravenous piperacillin followed by oral ampicillin. To our knowledge, this is the first reported case of spondylodiscitis after lung transplantation.

Adult↗

3-min step test and treadmill exercise for evaluating exercise-induced asthma.

A simple exercise test would be useful for detecting exercise-induced asthma, a common problem in asthmatic children. The current study compared the 3-min step test with treadmill exercise for evaluating exercise-induced asthma in asthmatic children and assessed whether responses to both tests are influenced by baseline lung function and habitual physical activity. A series of 154 asthmatic children (84 male children; mean age 12.9 +/- 0.9 yrs) underwent a 3-min step-test and treadmill testing on different days within a week at least 24 h apart. Before both tests each subject did spirometry to obtain the baseline forced expiratory volume in one second (FEV1). After both exercise challenges all subjects did serial spirometry and the lowest FEV1 recorded over time was used to calculate the fall in FEV1 expressed as a percentage of the measured pre-exercise (baseline) value (% fall in FEV1) and the area above the FEV1 curve (AAC0-30 min) expressed as a percentage of the pre-exercise value. Changes in both exercise variables were also analysed in percentile subgroups defined by questionnaire answers on habitual physical activity in hours. The mean % fall in FEV1 was significantly higher for treadmill exercise than for the step test (15.0 +/- 7.5 versus 11.7 +/- 5.9); and the AAC0-30 min was larger for treadmill than for the step test (-261.6 +/- 139.9% versus -197.3 +/- 105.0% min). In all subgroups defined by habitual physical activity the mean % fall in FEV1 decreased more after treadmill exercise than after the step test. After step test and treadmill exercise no significant correlation was found between % fall in FEV1 and baseline lung function, or between % fall in FEV1 among groups defined by habitual physical activity. Although the 3-min step test yields a lower % fall in forced expiratory volume in one second (FEV1) and a lower value of the area above the FEV1 curve than treadmill testing, it is a quick, economical, reproducible and portable alternative procedure for identifying exercise-induced asthma in outpatients and epidemiological studies. Baseline lung function and habitual physical activity have no influence on the amount or duration of exercise-induced asthma.

Adolescent↗

Comparison of two different protocols of neonatal screening for cystic fibrosis.

The results of two different protocols of neonatal cystic fibrosis (CF) screening in the Lazio region of Italy are reported. The first study, conducted from 1992 to 2000 on about 200,000 newborns, consisted of an immunoreactive trypsin (IRT) protocol without mutation analysis of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, referred to as the IRT/IRT protocol. Approximately 5% of the newborns with a positive first IRT test were also positive at the second test; approximately 57% of the newborns with a high IRT level at the second test were subsequently found to be affected by CF. In September 1998, a second protocol that included mutation analysis (IRT/DNA/IRT protocol) was started. Comparison of the two different screening protocols in terms of sensitivity in detecting CF patients demonstrated that the IRT/DNA/IRT protocol is more effective because it is able to detect a higher number of CF patients than the IRT/IRT protocol. In the same period, in addition to the overall diagnosis performed on a screening basis, 64 other subjects were identified as being affected by CF on the basis of symptomatic findings. The overall incidence of CF (screening + symptoms) was 1 : 2982, while that for carriers was 1 : 27. The sensitivity of the screening program increased over the period from 1992 to 2000, with the enhanced sensitivity in the past 2 years being due to the introduction of the IRT/DNA/IRT protocol.

Cystic Fibrosis↗

Microbiologic investigation on patients with cystic fibrosis subjected to bilateral lung transplantation.

BACKGROUND: In cystic fibrosis (CF) patients, lung transplantation is the only way to improve both quality and length of life. Data in the literature show that, in 80% of the cases, mortality after lung transplantation in CF patients is due to infections. METHODS: We microbiologically monitored 34 patients subjected to bilateral lung transplantation in during 1996 to 1999 to ascertain whether a change in the bacterial species isolated from the lower respiratory tract took place that might have influenced the clinical conditions of the patients. RESULTS: Our results show that the percentage of nonfermenting Gram-negative bacteria isolated from the lower respiratory tract remains high even in the posttransplantation phase. Nevertheless, the general clinical conditions of most of the patients were good and the three patients who died did not do as a consequence of an infection. CONCLUSIONS: Lung transplantation constitutes a valid therapeutic choice for CF patients because the microorganisms that we isolated from the lungs of the patients in our study behave mostly as contaminants rather than as colonizers. However, the transplanted patients remain at risk and thus require constant microbiological surveillance.

Bronchiolitis Obliterans↗

AP-PCR typing of Pseudomonas aeruginosa isolated from patients with cystic fibrosis.

Arbitrarily Primed Polymerase Chain Reaction has been used for an epidemiological evaluation of 42 strains of P. aeruginosa isolated from nine cystic fibrosis patients during a three-year investigation period. The resistance patterns of the same strains have also been evaluated. The AP-PCR type fingerprinting was perfomed with primers 10514 and 208. Resistance was evaluated by the Minimal Inhibitory Concentration method. With 10514 eleven different genotypes could be evidenced, while with 208 only five of them could be detected. During the investigation period patients were always colonised by the same genotype. A possible correlation between resistance pattern and genotype with both primers has shown, within the same patient, a correspondence of about 20% for 10514 and a correspondence of only 10% for 208. Patients are colonised by one or two strains of P. aeruginosa and there is no relation between genotype and resistance pattern.

Adult↗

Pseudomonas aeruginosa antibody detection in cystic fibrosis patients.

Chronic respiratory infection due to Pseudomonas aeruginosa remains the most important prognostic factor in cystic fibrosis patients. One method to lengthen the patient's life is to extend the initial state of the illness with an early diagnosis, before Ps. aeruginosa infection becomes chronic. Often this is difficult because of the young age of the patients. This study tested an immunoenzymatic system to evaluate antibody response against three Ps. aeruginosa purified antigens, alkaline protease, elastase and exotoxin A. We studied 40 patients with cystic fibrosis, 20 affected and 20 unaffected by apparent Ps. aeruginosa infection, also from the bacteriological point of view. Serological and bacteriological results were compared for each patient and showed that serological screening can be useful in young subjects, who often have no bacteriological evidence of Ps. aeruginosa colonization.

ADP Ribose Transferases↗

Lung transplantation for cystic fibrosis.

Between November 1996 and November 1997 we have transplanted 13 patients with Cystic Fibrosis (CF). Bilateral Sequential Lung Transplantation (BSLT) was successfully performed in all patients; one patient died from pneumonia and sepsis in the postoperative period and 12 are alive and well after a follow-up ranging between 1 and 13 months. Blood gas analysis improved from mean values of PaO2: 56 mm/Hg (with oxygen) and PaCO2: 43 mm/Hg to mean values of PaO2: 85 mm/Hg and PaCO2: 37 mm/Hg. Pulmonary function tests also improved dramatically: FEV1 improved from 20% predicted to 98% predicted. FVC also improved from 39% to 100%. The quality of life markedly improved: the ideal body weight moved from about 84% to normal values within nine months, and the 6-minute walk-test improved after transplantation from a preoperative distance of 325 meters, to 600 meters after 6 months. In conclusion, our favorable experience with BSLT in CF patients emphasizes the importance of lung transplantation in these patients. Carefully selected and properly managed patients may benefit from transplantation in terms of quality and duration of life.

Adult↗

Timing and priorities for cystic fibrosis patients candidates to lung transplantation.

Bilateral lung transplantation is actually considered a valuable option for patients with endstage lung disease related to cystic fibrosis. Timing is crucial to transplant successfully as many patients as possible and it is mainly based on the progressive worsening of pulmonary function tests and quality of life. We reviewed the charts of all patients accepted for lung transplantation at our institution, in order to assess the role of several functional and demographic parameters; we compared the group of patients able to successfully wait for transplantation (Group A) with patients dying on the waiting list (Group B). Twenty-eight patients were accepted: 15 were successfully transplanted (2 at other institutions) (mean waiting time: 117 days), 7 died waiting (mean waiting time: 108 days) and 6 are still on the list. We recorded FEV-1, FVC, PaO2, PaCO2, supplemental O2 requirement, 6-minute walking test, right ventricular ejection fraction (RVEF) and cardio-pulmonary hemodynamics measured at right heart catheterization; we recorded also age at time of diagnosis and at time of evaluation, sex, weight and Schwachman score. These parameters were compared between Group A and B. Age at time of evaluation, sex, weight and Schwachman score did not present any difference between the two groups, as well as pulmonary function tests, PaO2, 6-minute walk test and RVEF. A statistically significant difference was found in terms of PaCO2 (43.9 +/- 9.3 in Group A vs 69.1 +/- 32.4 in Group B, heart rate at rest (102 +/- 21 vs 131 +/- 12) mean pulmonary artery pressure (20.6 +/- 2.9 vs 36 +/- 15.7), pulmonary vascular resistances (350 +/- 96 vs 460 +/- 119.4), cardiac index (3.2 +/- 0.6 vs 5.4 +/- 0.9). On the base of our initial experience we conclude that a careful evaluation of CF candidates for lung transplantation is recommended. A deterioration of pulmonary function tests and quality of life are useful parameters to accept patients in the waiting list; however priority should be attributed also on the base of cardio-pulmonary hemodynamics. A larger series of patients is required to draw definitive conclusions.

Adult↗

Study of IgG antibodies to Pseudomonas aeruginosa in early cystic fibrosis infection.

Intermittent colonization, which then becomes chronic, characterises pseudomonas lung infection in cystic fibrosis (C.F.) patients. Bacteriologic studies do not always detect this evolution, which, however, may be identified by serological examinations. The aim of this study was to apply the immunoblotting method in order to monitor the antibody response toward a Ps. aeruginosa standard antigen (St-Ag). The results were analyzed and focussed on the bacterial and clinical data. Patients with intermittent colonization were studied between 1995 and 1997. Ninety per cent of the patients were positive to total IgG and subclass IgG1 in correspondence with the bacteriological finding. Further investigation is called for regarding the presence of IgG4 in order to establish whether it could be considered an index of chronic status.

Antibodies, Bacterial↗

Different serological examinations in cystic fibrosis patients: response to Pseudomonas aeruginosa St-Ag.

Crossed immunoelectrophoresis (CIE), enzyme linked immunosorbent assay (ELISA) and immunoblotting (IMB) were used to look for the presence of anti-bodies to Pseudomonas aeruginosa standard antigen in the sera of cystic fibrosis patients with intermittent (CF +/- P) and chronic (CF + P) colonization. Our results show that CIE is too complex a method to use when monitoring the infection over time, that with ELISA a correlation with the patient's clinical status may be made and that IMB can evidence an early, albeit low, reaction in the CF +/- P patient.

Adult↗

Precipitating Pseudomonas aeruginosa antibodies and antimicrobial therapy in cystic fibrosis patients.

Forty patients with cystic fibrosis were studied bacteriologically and serologically. Precipitating Pseudomonas aeruginosa antibodies were monitored by crossed-immunoelectrophoresis (CIE) in order to evaluate the possibility of preventing chronic colonization by Pseudomonas aeruginosa by cycles of antimicrobial therapy. Sputum or pharyngeal aspirate and serum samples from all patients were analyzed by means of spread on selective media and CIE, respectively. Significant differences in the number of precipitins were obtained: noncolonized and intermittently colonized patients had no precipitins, whereas the number of precipitins in the chronically colonized patients varied from 11 to 44. An increase in the number of precipitins could be a good marker for initiation of therapy with antimicrobial agents that are either active against Pseudomonas aeruginosa or able to inhibit the release of virulence factors.

Adolescent↗

Effect of a medium dose of ursodeoxycholic acid with or without taurine supplementation on the nutritional status of patients with cystic fibrosis: a randomized, placebo-controlled, crossover trial.

Ursodeoxycholic acid administration has been reported to improve cholestasis and inflammatory activity in primary biliary cirrhosis and, in an uncontrolled study, also in young adults with cystic fibrosis (CF) and chronic cholestasis. As an improvement in nutritional status was also observed in these young adult patients, we investigated whether the administration of a medium dose of ursodeoxycholic acid ameliorates the nutritional status of malnourished young adult CF patients with chronic liver disease. The study included 51 patients (27 male patients and 24 female patients; age range, 8-32 years; median, 14) with body mass percentiles < 90%. Patients were randomly assigned to receive either ursodeoxycholic acid (10-12 mg/kg/day) alone or with taurine (18-22 mg/kg/day). Patients were followed in a crossover fashion within each group; 6 months of treatment was randomly alternated with 6 months of placebo. Nine patients dropped out before concluding the study. Liver function tests, nutritional status, and coefficients of fat absorption were determined at entry and after each 6 months of placebo or treatment. Nutritional status and fat absorption were not significantly modified by either treatment. Liver function tests improved after ursodeoxycholic acid administration only in patients with concomitant chronic liver disease. Our findings indicate that 6 months of therapy with a medium dose of ursodeoxycholic acid, either alone or with taurine, does not improve the nutritional status of young malnourished CF patients. Higher doses given for longer periods might be worth investigating.

Absorption↗

Food allergy in cystic fibrosis.

Food allergy was investigated in 20 children with cystic fibrosis (CF), who still suffered from diarrhea and failed to thrive, in spite of adequate diet and enzyme treatment (group A). The study also included two age-matched control groups, comprising 10 CF children without intestinal symptoms and/or failure to thrive (group B), and 20 healthy children (group C). Skin tests were positive and total IgE higher than the mean + 2SD respectively in 14/20 and 11/20 patients of group A, in 3/10 and 2/10 patients in group B and in none in group C. The specific IgE were present in 6/14 children in group A whose skin tests were positive and in none in group B. There was no significant difference between group A and group B (p > 0.05). The levels of specific antibodies IgG, IgA and IgM were overall higher than the mean + 2SD of the normal in 18/20 in group A, in 6/10 in group B and in none in group C. The measurement by ELISA of specific antibodies for cow milk and egg proteins showed a statistically significant difference for casein, beta-lactoglobulin and ovalbumin between the IgG (p < 0.05) and IgA (p < 0.001) levels in group A and the other groups (B and C). Symptoms improved in 90% of CF patients (group A) when the implicated foods were eliminated from the diet and in 78% the oral provocation test resulted positive. The occurrence of food allergy must be considered in CF patients who do not improve with the conventional treatment. In these patients immunological investigations, in particular the measurement of IgG, IgA and IgM specific antibodies, are useful for diagnosis and in selecting an appropriate diet leading to an improvement in nutritional status.

Adolescent↗

Gallstones in cystic fibrosis: a critical reappraisal.

Radiolucent gallstones are common in young adults with cystic fibrosis. In the mid-1970s, it was suggested that gallstones are made of cholesterol, but this hypothesis has never been tested. Several recent studies have shown that the detection of cholesterol monohydrate crystals in bile has high sensitivity and specificity for the diagnosis of cholesterol gallstones. We therefore used this approach to study 17 young adults with cystic fibrosis, 10 of whom had radiolucent gallstones. The two groups of patients were comparable in age and gender (all patients but one were male). Duodenal bile was obtained after gallbladder contraction with intravenous cerulein; it was used for lipid and protein chemistry studies and for polarizing microscopy. The latter was performed both in whole bile and in the postultracentrifugation (100,000 g) sediment. Bile cholesterol saturation did not significantly differ between patients with (1.21 +/- 0.28) or without gallstones (0.99 +/- 0.54). Slight cholesterol supersaturation was found in 7 of 10 gallstone and three of seven nongallstone patients. At no time were cholesterol crystals detected in either the group, even after bile ultracentrifugation. Two more cystic fibrosis patients with gallstones died of severe bronchopneumopathy, and small pigment gallstones were obtained at autopsy. At stone analysis, cholesterol content was 44% and 28% of dry weight, respectively. Infrared spectroscopy of stone powder was compatible with the presence of calcium bilirubinate and proteins as major components. We conclude that radiolucent gallstones of cystic fibrosis are not of the conventional cholesterol type.

Adolescent↗