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Methicillin-resistant Staphylococcus aureus (MRSA) infections are increasingly being reported among cystic fibrosis (CF) populations worldwide. In this paper, we sought to examine at the epidemiology, the molecular characterisation and the antibiotic resistance of MRSA isolates in our cohort of CF patients. All MRSA strains were collected prospectively at the University Hospital of Catania, Italy, during a two-year study between mid 2005 to mid 2007 and underwent molecular, pathotype and susceptibility characterisations. Our study demonstrates persisting infections with both hospital-associated (HA-) and community-associated (CA-)MRSA, including Panton-Valentine leukocidin (PVL)-positive strains, in our CF population with an overall prevalence of 7.8%. We demonstrated that, in these patients, persistence was sustained by either identical clones that underwent subtle changes in their toxin content or by different clones over time. The isolation of MRSA in our CF population aged 7-24 years was associated with an increased severity of the disease even if, due to the small sample of patients included and the paucity of data on the clinical outcome, these results cannot be conclusive. Furthermore, three strains were heteroresistant vancomycin-intermediate S. aureus (hVISA), questioning the use of glycopeptides in the treatment of MRSA infections in these patients.

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In patients with cystic fibrosis (CF), there is an increasing incidence of some uncommon respiratory pathogens, such as Burkholderia cepacia complex (Bcc), Stenotrophomonas maltophilia, and Achromobacter xylosoxidans. In order to evaluate the prevalence and the clinical impact of these pathogens, we retrospectively studied a total of 109 patients followed in our center from 1996 to 2006 and reviewed the results of 1,550 sputum samples. The isolation of Pseudomonas aeruginosa slightly decreased over the observed decade, whereas Staphylococcus aureus exhibited an irregular trend. Infection with Bcc reached a peak in 1998 and successively decreased to a stable 4%. S. maltophilia and A. xylosoxidans were the real emerging pathogens, since first isolation occurred in 2004; however, the percentage of infected patients remained low (7% and 3.2%, respectively) through the years. In conclusion, in our center for CF, the reduced prevalence of P. aeruginosa over the last decade has been associated with a concurrent reduction of infections by Bcc and, as compared to other centers in Italy, Europe, and the US, with a low incidence of emerging pathogens such as S. maltophilia and A. xylosoxidans.

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BACKGROUND: Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are a relatively frequent cause of male infertility such as congenital absence of vas deferens. This finding suggests that CFTR could play a significant role in the etiopathogenesis of congenital absence of vas deferens. METHODS: To assess how often CFTR mutations and 5-thymidine variant of the polythymidine tract of intron 8 are detected in congenital absence of vas deferens we selected 35 infertile male adults caused by congenital absence of vas deferens. RESULTS: Frequency of mutations differs significantly from that in the general population (2% vs 40% respectively) and the delta F 508 mutation accounts for 35% of these mutations. Abnormal sweat test resulted in several patients with a mutation than in others and this difference was highly significant. CONCLUSIONS: This result confirms the role of CFTR protein in the pathogenesis of this malformation and suggests that CBAVD phenotype can represent a mild form of cystic fibrosis. Consequently genetic analysis should take into account the presence of an associated mutation and patients with bilateral agenesia and their partners should be screened for cystic fibrosis prior to any in vitro fertilization techniques.

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BACKGROUND/AIMS: Very few studies have been performed on the long-term clinical advantages of neonatal screening programs for cystic fibrosis (CF) and these have been inconclusive. This is a preliminary report of two observational cohort studies on this subject. METHODS: In the first study, CF patients born between 1973 and 1981 in northeastern Italy were split into 4 groups according to the modality of diagnosis: screening by meconium test (58 patients); meconium ileus (45 patients); symptoms and pancreatic insufficiency (PI; 75 patients), or symptoms and pancreatic sufficiency (PS; 19 patients). The patients were followed for up to 26 years by three CF centers sharing common treatment protocols. In the second study, two cohorts of CF patients born between 1983 and 1992 were compared. Patients from one cohort (126 patients) were born in the Veneto region, where a neonatal screening program had been established based on immunoreactive trypsinogen. Patients from the other cohort (152 patients) were born in Sicily, where an intensive program of early diagnosis by symptoms was implemented. The cohorts were comparable for CF incidence, CFTR genotypes, gender proportion and common treatment protocols. Statistical analyses were performed by Kaplan-Meier survival curves, a Cox proportional hazard model for survival and cross-sectional comparisons by 2-year periods for weight z score, height z score and body mass index. RESULTS: In the first study, the patients detected by newborn screening (PI) showed better survival and nutritional status compared to patients diagnosed through meconium ileus or symptom presentation with PI. PS patients diagnosed by symptoms showed the best outcome, but most of them had a mild genotype. In the second study, the Veneto cohort showed better outcome with regard to survival and nutritional status over 16 years of follow-up. CONCLUSIONS: Observational cohort studies cannot give definitive evidence of the clinical benefit of neonatal CF screening; however, data have been accumulated which strongly suggest a better clinical outcome for CF patients born in an area where a screening program is performed.

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The effects of an alternative chelation program in thalassemic patients with severe iron overload are investigated. The schedule of treatment, feasible at home, consists in the administration of deferoxamine intravenously (100 mg/kg/die 8 hours 10 days a month), followed by 50 mg/kg/die subcutaneously in the remaining 20 days of the month. The results in 34 patients who underwent this program over 8 months are reported. After intensive chelation therapy serum ferritin and transaminase levels were significantly lower, and daily urinary iron excretion values were significantly higher when compared to the levels observed before the treatment. After the period of study, echocardiography revealed an ejection fraction (EF) significantly higher in 15 out of 34 cardiopathic patients. In conclusion, the alternative chelation program is effective in reducing iron overload of thalassemic patients, protecting them also against cardiac disease.

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The authors report a case of a child three years old, with severe malnutrition as complication of Ascaris lumbricoides infection. Intestinal nematodes infect many of the world's children and constitute a formidable public health problem. The infected children may suffer nutritional deficits, serious illness and occasionally death. Although infestation is uncommon in our country, it should be considered in children with low social life.

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Because of specific laboratory tests are lacking, diagnosis of cow's milk allergy is always made on the basis of improvement after cow's milk protein withdrawal from diet and relapsed after challenge test. However personal and familial anamnesis, supported by few simple laboratory tests (peripheral blood and stool eosinophiles, hemoccult), are important tools for diagnosis. In this work we report the clinical findings of 68 children, suffered from cow's milk allergy, observed in the last 10 years. Children were divided into three groups on the basis of challenge response: Group 1 consisting of children with averse reaction occurred within the first hour after the administration of cow's milk protein (IgE-mediated reaction, 1st class as Gell-Coombs classification); Group 2 consisting of children with averse reaction occurred between 2nd and 12th hour (1st-3rd class as Gell-Coombs classification); Group 3 consisting of children with averse reaction occurred after 24 hours the administration of cow's milk proteins (4th class as Gell-Coombs classification). Auxological parameters show that while weight is widely involved, particularly in children of group 3, height is interested only in children with prolonged diarrhoea. Cow's milk protein withdrawal from diet determine a rapid normalization of weight increment rate, more evident in children over 25th centile. Height rise up normal values slowly without any differences between children below and over 25th centile. At the age of two years 57 children (83%) became tolerant to cow's milk proteins and after 5-year follow-up in 3 children (4.4%) only persisted cow's milk allergy. All these children presented the IgE-mediate clinical form.

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A retrospective study with the aim of evaluating sensitivity and specificity of antigliadin antibodies (AGA) was carried out. AGA values, IgA and IgG, and mucosal damage at intestinal biopsy were compared in each subject, on a sample of 245 subjects, who had undergone intestinal biopsy because of suspected coeliac disease (CD), from January 1991 to December 1993. 130 subjects (53.1%) were found to be suffering from CD, whereas the remaining 115 subjects (46.9%), who did not show any signs of the disease, were considered as controls. When we compared AGA values with mucosal damage we observed that IgA were normal in 24 (18.5%) and pathological in 106 (81.5%) coeliac patients, whereas they were normal in 107 (93%) and pathological in 8 (7%) controls. IgG values were normal in 17 (13.1%) and pathological in 113 (86.9%) coeliac patients, but normal in 75 (65.2%) and pathological in 40 (34.8%) controls. Data about AGA sensitivity related to age, showed IgA sensitivity varying from 80.4% in children under 1 year old to 70% in children over 10 years old and IgG sensitivity from 94.1% to 50%. Data about specificity related to age, showed IgA sensitivity varying from 78.7% in children under 1 year old to 100% in children over 10 years old and IgG specificity from 30.4% to 90.9%. Our results indicated that AGA-IgA had a total sensitivity or 81.5%, a total specificity of 93% and a concordance with intestinal damage of 86.9% while AGA-IgG had a total sensitivity of 86.9%, a total specificity of 65.2% and a concordance with intestinal damage of 76.7%. These data presented a modification on the basis of age, being higher in children under 3 years of age and decreasing with the increase in age. We conclude that AGA have great importance in suspect CD, but it is most important that their results are evaluated on the basis of age.

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23 children, aging from 3 to 13 years, affected by chronic upper airway obstruction, were studied. The clinical suspicion was "obstructive sleep apnea" (O.S.A.). In this study was found a significantly different frequency, in O.S.A. group, if compared with controls, of the following symptoms: nocturnal snoring (100%), mouth breathing when awake (69%), and during sleep (91%), abnormal restless movements (69%), behavioral disturbances (60%). Clinical evaluation revealed: pectus excavatum (82%), enlarged tonsils and adenoids (82%), failure to thrive (39%). Chest index was 0.72 +/- 0.07 significantly higher (p < 0.001) than controls. The authors also carried out in all patients and controls pCO2 venous blood determination during sleep, to screen subjects with high risk of cardiopulmonary dysfunction. Data obtained showed that threshold value of pCO2 was 45.6 mmHg.

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The Authors carried out a Rotavirus investigation on the stools of 6057 children admitted in the Department of Pediatric and Pediatric Gastroenterology of Catania University, during the years 1984-1988. The stool samples of 264 children were found positive for Rotavirus. While 204 children presented gastrointestinal symptoms with diarrhoea, 12 had subclinical signs, 26 presented only respiratory symptoms and 22 had no clinical symptomatology. Moreover 122 children with diarrhoea had associated respiratory manifestations. In this study the authors punctualized that: 1) the most interested age was the first year of life; 2) there was no evidence in seasonal variations; 3) the respiratory tract was involved in high percentage of children (59.8%); 4) a transient lactase deficiency was supposed in every children with watery diarrhoea, but it was demonstrated only in 21 (10.3%).

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The Authors study the incidence of gram-negative fermentative and non-fermentative bacilli in an Intensive Care Division according to the following microbial contamination indexes: Air Microbic Index; Staff Index; Patient Index; Equipment Index. The collected data show that some air microbic factors and part of the complementary equipment to intensive care are particularly infected by gram-negative germs Germs such as Pseudomonas Acinetobacter and mainly bacilli of K.E.S. group.

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