Clinical and laboratory correlations in patients with cystic fibrosis and Pseudomonas aeruginosa infection: The experience of a regional centre.

Pseudomonas aeruginosa (Pa) is the most important pathogen affecting the prognosis of patients with cystic fibrosis (CF). Distinguishing the correlations associated with PA infection may change the way in which investigation and therapeutic plans are approached. This study aims to evaluate the correlations between various factors such as age at diagnosis, nutritional status, homozygous status of delF508 mutation, the number of exacerbations per year, imagistic modifications, the presence of chronic infection with Staphylococcus aureus, serum levels of total immunoglobulins G (IgG) and gammaglobulins and ventilation parameters - forced vital capacity (FVC) and one-second forced expired volume (FEV1) and their association with Pa infection. Methods: The associations between the analyzed parameters have been made by logistic regression; the comparisons between means of continuous variables were performed using Studen t-test; the variables distribution was tested using chi-square-test; and correlations between variables were assessed using Pearson coefficient. Results: The mean age at the acquisition of Pa was 5.26 (±5.3 years). Of the 50 patients included in the study, 21 acquired the Pa infection having the median age of primo-infection of 3 years. The study identified a greater number of exacerbations per year in those infected with Pa (2.71 compared to 1.38 exacerbations per year; p=0.002); the increase of radiological score with 1 unit associated with an increased risk of infection with Pa (OR 1.31; 95% CI 1.10-1.56;p=0.002), the mean Brasfield radiologic score is lower for uninfected children (6.9 vs. 11 points, p=0.001); increasing the degree of bronchiectasis associated with Pa infection (OR 3.75;CI 1.77-7.96;p=0.001); gammaglobulin (15.91 vs. 19.42%;p=0.01) or immunoglobulin G values (IgG) (9.8 vs. 11.9 g/L;p=0.05) were higher for those infected. Conclusions: The increased number of exacerbations per year, the severity of imagistic modifications, and IgG and gammaglobulin values are associated with Pa infection. Tracking these parameters guides the therapeutic conduct.

The role of FENO and spirometry in the evaluation of obstruction in pediatric asthma.

INTRODUCTION: The fractional exhaled nitric oxide concentration (FENO) is used as a noninvasive biomarker for asthmatic inflammation. The role in establishing an asthma diagnosis is still under research. Spirometry remains the main investigation for the diagnosis and monitoring of asthma. OBJECTIVES: The aim of the study was to assess the correlation between FENO level and the values of spirometric parameters defining airflow obstruction. MATERIALS AND METHODS: The longitudinal study included a group of 89 children admitted to the Pediatric Clinic of "Grigore Alexandrescu" Emergency Clinical Hospital for Children. The inclusion criteria were: (i) a previously known diagnosis of asthma, (ii) age range between 5 and 18 years old. All patients underwent pulmonary function testing and measurement of FENO. RESULTS: Significant correlations were identified between FENO and a part of spirometric parameters: MEF75 (p=0.007), MEF50 (p=0.0.28), MEF25 (p=0.011) and PEF (p=0.020), but not with FEV1 (p=0.090), nor FVC (p=0.308). However, multiple linear regression analysis revealed that spirometric parameters poorly predict FENO variation (R=0.062, p=0.82). In adition, binary logistic regression analysis (LR) showed that FENO can not be used as a reliable biomarker (p=0.169) for the estimation of the risk for obstructive dysfunction. CONCLUSION: The level of exhaled nitric oxide does not correlate with the values of spirometric values. More studies with a large number of patients are needed for establishing the role of FENO in the evaluation of airflow obstruction.

Single and multipathogen viral infections in hospitalized children with acute respiratory infections.

We aimed to describe the viral etiology of acute respiratory tract infections in children aged 0-8 years admitted to Grigore Alexandrescu Hospital, the largest pediatric hospital in Romania. The patients had clinical diagnosis of pneumonia, bronchiolitis or viral respiratory infections and had been hospitalized between September 2010 and September 2011. The study was part of the "Molecular investigations of acute respiratory infections caused by non-influenza viruses, to assess the implications of infant and young child pathology" (2008-2011), a National Project II--42-164 (MIRVI). We included in the study 241 children that were swabbed in the first 8 days of the onset with the following symptoms during the previous 7 days: fever > 38 degrees C, AND cough or sore throat, and shortness of breath or difficulty breathing .We identified by RT-PCR 131 (54.4%) positive samples: 112 (85.5%) for a single pathogen, 18 (13.7%) for coinfection with two pathogens and 1(0.8%) for coinfection with three pathogens. The most frequent pathogen identified was respiratory syncytial virus (RSV) (40.18%), followed by Rhinovirus (RhV) (20.54%) and human Metapneumovirus (hMPV) (12.50%). We extrapolated our data to the National program of surveillance of SARI (severe acute respiratory infections). In this program, 191 children aged one month-8 years, were hospitalized in the same period, in which the highest percentage of positivity was due to Influenza viruses (62.65%), but RSV was identified with almost the same percent like in MIRVI (32.53%). It should be noted that among patients with pneumonia, bronchiolitis or respiratory viral infections were identified as the causal agent RhV.

The adenoviral infections in children admitted to hospital with pneumonia, acute bronchiolitis or respiratory viral infections.

UNLABELLED: The objective of this study was to investigate the percent of infections with adenovirus (ADV) in children who had pneumonia, acute bronchiolitis or viral respiratory infections and were admitted to two pediatrics hospitals in Bucharest (Grigore Alexandrescu Hospital and Alfred Rusescu Hospital). SUBJECTS: 70 children aged one month - five years, admitted to the above mentioned pediatrics hospitals in Bucharest, who were negative for the Respiratory Syncytial Virus (RSV) and the human Metapneumovirus (hMPV) by Reverse Transcription -Polymerase Chain Reaction (RT-PCR). 48 of them presented pneumonia upon admission to hospital, 6--acute bronchiolitis and 16 respiratory viral infections. Samples (nasal swabs) were taken from patients and introduced in viral transport medium. DIAGNOSTIC METHODS: RT-PCR for RSV and hMPV, Multiplex PCR by seeplex multi-detection system with Seeplex RV/PB 18 ASE Detection for detection of 5 pneumonial bacteria and Real-Time PCR, Duplica Real Time Adenovirus Detection for ADV. RESULTS: Of the total 70 patients negative for RSV, hMPV and 5 pneumonial bacteria (Streptococcus pneumoniae, Haemophilus influenzae, Mycoplasma pneumoniae, Chlamydophila pneumoniae, Legionella pneumophila), 10 were ADV positive; none of the children < 6 months (N = 16) presented ADV infection. In the 6 months - 2 years group (N = 35), 6 were ADV positive. In the 2 - 5 years group (N = 19), 4 were ADV positive. CONCLUSIONS: The percent of ADV infections in children hospitalized with acute respiratory infections (ARI) caused by neither RSV or hMPV is 14.2%. ADV is most frequently encountered in the 6 months - 2 years and then 2 - 5 years groups, but the most severe pneumonia forms can be seen in the 6 months - 2 years group. In children < 6 months with acute bronchiolitis ADV was not found to be an etiologic agent.

Clinical spectrum of Anderson Fabry disease in a Romanian family.

Anderson Fabry disease (alpha galactosidase A deficiency) is an X-linked recessive lysosomal storage disorder; alpha galactosidase A deficiency results in accumulation of neutral glycosphingolipids, especially globotriaosylceramide (Gb3), in various cell types promoting development of disease with renal, cardiovascular, and cerebrovascular involvement. Clinical aspects which usually begin in childhood or adolescence include intermittent pain in the extremities (acroparesthesias), episodic "Fabry crisis" of acute pain lasting hours to days, characteristic skin lesions (angiokeratomas), hypohidrosis, heat and cold intolerance. Classic phenotype conception of the disease has changed within the past decade, recognizing that disease is not limited to the classical full-blown manifestation in affected males, but may also occur in carrier females. The expanding clinical spectrum of Anderson Fabry disease (AFD) is a real challenge to diagnosis, especially in some patients whose exclusive single organ manifestation belongs to the heart or kidney. This paper reviews natural history of three unrecognized cases recently diagnosed by markedly deficient alpha galactosidase A (alpha Gal A) activity in peripheral leucocytes. Case A: A male patient, aged 24 years, experienced recurrent acroparesthesia when he was 9 years-old. His 26 years-old sister has angiokeratomas as the only sign of disease (case B). Case C: the uncle of these two cases (A, B) has a long history of disease including chronic renal failure, bilateral deafness, stroke, aseptic osteonecrosis. The purpose of the presentation is to sharpen physicians' perception of this disease. Early and accurate diagnosis is mandatory considering that this disorder is now, after introduction of the novel enzyme replacement therapy, a treatable disease.

Aspects of nailfold capillaroscopy in children and young people with rheumatic disease.

Nailfold capillaroscopy was performed in children and young people with rheumatic disease (42 patients with a variety of childhood rheumatic diseases) and 30 normal control subjects. Distinctive morphologic abnormalities with capillary enlargement, megacapillary and dropout of surrounding structures were noted in two groups: patients with childhood dermatomyositis (DM) and with scleroderma (SD). Capillary abnormalities were found in all 4 patients affected by systemic disease and in none of 7 patients with localized scleroderma. Some minor atypical capillary morphology were observed in normal individuals (37%) but the enlarged capillaries, especially megacapillaries were not found.