Uros Krivec

Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry

Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF). We analysed forced expiratory volume in 1 s (FEV1) data of 14 732 patients registered in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database in 2007. We used linear and logistic regressions to investigate associations between FEV1 % predicted and clinical outcomes. Body mass index (BMI), chronic infection by Pseudomonas aeruginosa, pancreatic status and CF-related diabetes (CFRD) showed a statistically significant (all p<0.0001) and clinically relevant effect on FEV1 % pred after adjusting for age. Patients with a lower BMI experience a six-fold increased odds ratio (95% CI 5.0–7.3) of having severe lung disease (FEV1 <40% pred) compared to patients with normal BMI. Being chronically infected with P. aeruginosa increases the odds ratio of severe lung disease by 2.4 (95% CI 2.0–2.7), and patients with pancreatic insufficiency experience a 2.0-fold increased odds ratio (95% CI 1.6–2.5) of severe lung disease compared to pancreatic sufficient patients. Patients with CFRD have a 1.8-fold increased odds ratio (95% CI 1.6–2.2) compared to patients not affected. These potential risk factors for pulmonary disease in patients with CF are to some degree preventable or treatable. We emphasise the importance of their early identification through frequent routine tests, the implementation of infection control measures, and a timely initiation of relevant therapies. Independent effect of nutrition, chronic Pseudomonas aeruginosa infection and CF-related diabetes on FEV1 in CF patients http://ow.ly/qhAXJ

Human bocavirus as the cause of a life-threatening infection.

ABSTRACT Human bocavirus is a recently described respiratory pathogen. A case of a life-threatening human bocavirus infection of a previously healthy pediatric patient is described. An initial clinical presentation of acute bronchiolitis developed into an extremely severe course of disease characterized by pneumothorax, pneumomediastinum, and acute respiratory failure with pronounced air-leak syndrome.

Coronavirus infections in hospitalized pediatric patients with acute respiratory tract disease

BackgroundAcute viral respiratory infections are an important cause of morbidity and mortality in humans worldwide. The etiological backgrounds of these infections remain unconfirmed in most clinical cases. The aim of this study was to estimate the prevalence of human coronavirus infections in a series of children hospitalized with symptoms of acute respiratory tract disease in a one-year period in Slovenia.MethodsThe 664 specimens from 592 children under six years of age hospitalized at the University Children’s Hospital in Ljubljana were sent for the routine laboratory detection of respiratory viruses. Respiratory viruses were detected with a direct immunofluorescence assay and human coronaviruses were detected with a modified real-time RT–PCR.ResultsHCoV RNA was detected in 40 (6%, 95% CI: 4.3%–8.1%) of 664 samples. Of these specimens, 21/40 (52.5%) were identified as species HKU1, 7/40 (17.5%) as OC43, 6/40 (15%) as 229E, and 6/40 (15%) as NL63. Infection with HCoV occurred as a coinfection with one or more other viruses in most samples (70%). Of the HCoV-positive children, 70.3% had lower respiratory tract infections.ConclusionThe results of our study show that HCoV are frequently detected human pathogens, often associated with other respiratory viruses and acute respiratory tract infections in hospitalized children. An association between age and the viral load was found. The highest viral load was detected in children approximately 10 months of age.

Human bocavirus and other respiratory viral infections in a 2-year cohort of hospitalized children.

Human bocavirus (HBoV) infection is reported worldwide and may cause severe respiratory tract infections. The aim of the present study was to assess the prevalence of HBoV, and other respiratory viral pathogens, in a 2‐year retrospective study of children admitted to hospital, and to investigate whether viral loads of HBoV DNA were associated with severity of infection. Between April 2007 and March 2009, 891 respiratory samples from 760 children admitted to hospital with acute respiratory tract infection were tested for the presence of respiratory viruses by real‐time PCR or direct immunofluorescence testing. HBoV DNA was detected by using internally controlled real‐time quantitative PCR assay and 25 samples selected at random were sequenced. The virus detected most frequently was rhinovirus, followed by respiratory syncytial virus, HBoV, and human metapneumovirus. HBoV DNA was detected in 18.4% of children admitted to hospital. HBoV was the only viral pathogen detected in 66/164 (40.2%) of HBoV DNA‐positive children and in 7.4% of all 891 samples. Ninety‐seven percent (64/66) of children with an HBoV single infection were diagnosed as having lower respiratory tract infection. Median HBoV DNA viral load was significantly higher in children when HBoV was detected as a single pathogen. Higher HBoV DNA viral loads were associated with prematurity and age. HBoV seems to be an important and frequent pathogen in respiratory tract infections in children, and it is likely that the severity of illness is comparable to the severity of RSV illness. J. Med. Virol. 84:99–108, 2011.

First recovery of Rasamsonia argillacea species complex isolated in adolescent patient with cystic fibrosis in Slovenia – case report and review of literature

We report the isolation of the emerging fungal pathogen Rasamsonia aegroticola, which belongs Rasamsonia argillacea species complex, from a respiratory sample of a patient with cystic fibrosis. This filamentous fungus, resembling members of a Penicillium and Paecilomyces spp., was identified by morphology and confirmed by DNA sequence analysis. Susceptibility pattern showed high minimal inhibitory concentration of voriconazole and amphotericin B but low minimal inhibitory concentration of caspofungin, micafungin and itraconazole.

Reference percentiles for FEV1 and BMI in European children and adults with cystic fibrosis

BackgroundThe clinical course of Cystic Fibrosis (CF) is usually measured using the percent predicted FEV1 and BMI Z-score referenced against a healthy population, since achieving normality is the ultimate goal of CF care. Referencing against age and sex matched CF peers may provide valuable information for patients and for comparison between CF centers or populations. Here, we used a large database of European CF patients to compute CF specific reference equations for FEV1 and BMI, derived CF-specific percentile charts and compared these European data to their nearest international equivalents.Methods34859 FEV1 and 40947 BMI observations were used to compute European CF specific percentiles. Quantile regression was applied to raw measurements as a function of sex, age and height. Results were compared with the North American equivalent for FEV1 and with the WHO 2007 normative values for BMI.ResultsFEV1 and BMI percentiles illustrated the large variability between CF patients receiving the best current care. The European CF specific percentiles for FEV1 were significantly different from those in the USA from an earlier era, with higher lung function in Europe. The CF specific percentiles for BMI declined relative to the WHO standard in older children. Lung function and BMI were similar in the two largest contributing European Countries (France and Germany).ConclusionThe CF specific percentile approach applied to FEV1 and BMI allows referencing patients with respect to their peers. These data allow peer to peer and population comparisons in CF patients.

Fatal human bocavirus infection in an 18-month-old child with chronic lung disease of prematurity.

A fatal case of human bocavirus 1 pulmonary infection in an 18-month-old prematurely born child is described. Despite conventional and oscillatory ventilatory support, intractable hyperinflation developed with bilateral pneumothorax and acute cardiac failure 3 days after the onset of respiratory symptoms.

Epiglottic suture for treatment of laryngomalacia.

OBJECTIVES To present a technique for surgical management of laryngomalacia directed against the basic abnormality of the disease. Considering the cause-consequence relations of the abnormalities, we can distinguish two types of laryngomalacia. In the first, the basic abnormality is the pathological shape of the epiglottis: the epiglottis, which normally stands in an upright position, is characteristically excessively folded, restricting the supraglottic space directly as well as indirectly due to the proximity of the aryepiglottic folds that are attached to its lateral edges. In the second type the abnormality is the backward displacement (ptosis) of a normally shaped epiglottis. All other abnormalities are the consequences of these basic abnormalities. We present a new procedure, the epiglottic suture, to correct the pathological shape of the epiglottis. It is a suture placed transversely on the lingual surface of the epiglottis that unfolds the folded epiglottis and shifts apart the adjacent aryepiglottic folds. PATIENTS AND METHODS Prospective non-randomized study performed on eight severely distressed patients with laryngomalacia at the University Department for Otorhinolaryngology and Cervicofacial Surgery, Ljubljana, Slovenia. RESULTS AND CONCLUSION The epiglottic suture enabled normal breathing in all treated children without compromising the airway-protection function of the epiglottis. After an average follow up time of 19.12 months (minimum 7 months and maximum 27 months), we have not noticed any complications or deteriorations of breathing.

Nocturnal Hypoxemia, But Not Hypercapnia, Correlates With Sleep Quality in Children

BACKGROUND: A correlation has been observed between obstructive sleep events and sleep quality. The aim of the study was to assess if there is also a correlation between nocturnal hypoxemia and hypercapnia and sleep efficiency and sleep fragmentation in children. METHODS: Nocturnal pulse oximetry (SpO2) and transcutaneous carbon dioxide (PtcCO2) recordings with simultaneous actigraphy were performed in 38 children with nocturnal hypoxemia and hypercapnia during spontaneous breathing (nocturnal hypoventilation [NH] group), 25 children with partially corrected nocturnal hypoventilation (PC-NH group), and 11 subjects with normal nocturnal gas exchange (no-NH group). RESULTS: Sleep efficiency and sleep fragmentation on actigraphy correlated with minimal SpO2 (r2 = 0.21, P = .004, and r2 = −0.10, P = .050, respectively) and the percentage of night time with SpO2 < 90% (r2 = −0.33, P < .001, and r2 = 0.13, P = .028, respectively) in the NH group. Sleep efficiency and sleep fragmentation also correlated with pulse rate standard deviation (r2 = −0.42, P < .001, and r2 = 0.37, P < .001, respectively). No correlation was observed between sleep efficiency and sleep fragmentation and PtcCO2. No correlation was observed between sleep efficiency and sleep fragmentation and SpO2, PtcCO2, and pulse rate in the PC-NH group. Sleep efficiency, sleep fragmentation, and nocturnal SpO2, and PtcCO2 were all normal and not correlated in the no-NH group. CONCLUSIONS: In children with nocturnal hypoventilation, nocturnal hypoxemia but not hypercapnia correlates with sleep efficiency and sleep fragmentation on actigraphy.

Pediatric flexible and rigid bronchoscopy in European centers—Availability and current practice

Eighteen years have passed since the last European survey concerning practices in pediatric bronchoscopy was conducted. Therefore, members of the European Respiratory Society (ERS) Pediatric Bronchology Group 7.7, initiated the “European Pediatric Bronchoscopy Survey 2015,” which aimed to assess the current state of this evolving diagnostic and therapeutic procedure in the field of pediatric respiratory medicine.