Lore Marianelli

Multiresistant non-fermentative Gram-negative bacteria in cystic fibrosis patients: the results of an Italian multicenter study

We carried out an epidemiological study on 2717 patients seen on a regular basis at Italian Cystic Fibrosis Centers with the aim of defining the prevalence of multiresistant Gram-negative strains in Italy. We found 272 multiresistant strains out of 1560 Gram-negative strains isolated in 1012 Italian cystic fibrosis patients. From our results in Italian cystic fibrosis patients we may conclude: (1)the beta-lactam antibiotics are moderately active; ceftazidime is the most efficacious even if 59.9% of multiresistant strains are not sensitive to this drug; (2)the aminoglycosides are poorly efficacious; 93% of multiresistant strains are resistant in vitro to tobramycin; and (3)the quinolones, notwithstanding their relatively recent introduction into clinical practice, have very poor activity against multiresistant strains, 89.7% of which are not sensitive.

Birth weight for gestational age centiles for Italian neonates

Objective: To provide centiles for birth weight (BW) according to gestational age (GA) and sex for infants born in Italy. Methods: We used records of the whole neonatal population of Tuscany, a region in Italy, from July 1991 to June 2002 as resulting from the database of the cystic fibrosis neonatal screening program (n = 290 129). We excluded as unlikely for GA those BW that were more than two interquartile ranges above the 75th centile or below the 25th centile for each GA and gender group. Results: We present the 3rd, 10th, 25th, 50th, 75th, 90th and 97th centiles of BW for GA from the 24th to 43rd week of gestation for male and female Italian neonates, as both tables and smoothed curves. Conclusions: The large size of the examined population allows us to provide up-to-date, reliable BW for GA centiles for Italian newborns, especially for lower GAs.

Allergic bronchopulmonary aspergillosis in Italian cystic fibrosis patients: prevalence and percentage of positive tests in the employed diagnostic criteria.

The prevalence of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) patients is difficult to determine because the data in the literature are not homogeneous or comparable. ABPA and CF have similar clinical symptoms which make diagnosis difficult and underestimate the real dimensions of the problem. We conducted an epidemiological study on 3089 Italian CF patients to determine the prevalence of ABPA in Italy and verify the percentage of positive tests in the employed diagnostic criteria. Our results indicate that the prevalence of ABPA in Italian CF patients is 6.18%, mainly in adolescents and young adults. ABPA is diagnosed using clinical symptoms (presence of episodic bronchial obstructions or typical radiographic features) and on the basis of other criteria which can only be partially fulfilled in paediatric patients. Among the diagnostic tests the most sensitive are the total IgE (84.5%), specific IgE anti-Aspergillus fumigatus (81.6%) and the prick test (68.3%). In the absence of clinical symptoms and gold standard diagnostic tests, serological positivity and/or the skin test are not sufficient evidence to confirm the presence of ABPA.

Genetic differences in cystic fibrosis patients with and without pancreatic insufficiency - An Italian collaborative study

SummaryTo determine the number and frequency of mutations that occur at the cystic fibrosis locus (CF), we have examined the allele and haplotype frequencies of eight polymorphic DNA markers linked to CF in 163 Italian patients who were sub-divided according to their clinical presentations. The distribution of haplotypes for the tightly linked polymorphisms KM.19 and XV-2c differ significantly between patients with and those without pancreatic insufficiency. The haplotype found most commonly in CF chromosomes occurs much more frequently in pancreatic insufficient than in pancreatic sufficient patients. Among the 19 pancreatic sufficient patients, 6 (31.6%) show at least one copy of the rare KM.19 = 1, XV-2c = 2 haplotype, as against 16 of 138 patients (11.6%) with pancreatic insufficiency. In addition, only 5 pancreatic sufficient patients (26.3%) are homozygous for the common 2,1 haplotype, as compared with 88 patients (63.8%) with pancreatic insufficiency. These findings support the hypothesis of allelic heterogeneity at a single locus in CF and suggest that different mutations underlie the presence or absence of pancreatic insufficiency in this disorders.