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A 12-year-old girl of Turkish descent was referred 6 weeks after an influenza A infection because of persistent chest X-ray abnormalities compatible with interstitial lung disease. The clinically suspected diagnosis of pulmonary alveolar microlithiasis (PAM) supported by pathognomonic radiological abnormalities was confirmed by genetic analysis. The clinical presentation of PAM is illustrated by a case and review of the current literature on this subject: you only see what you know.

Reports from the seventies and eighties have shown that cystic fibrosis (CF) patients with severe lung disease have high levels of IgG and that this is associated with worse prognosis. We decided to explore IgG level as a possible outcome parameter for lung disease severity in a cohort of pediatric CF patients treated according to current standards of care. Seventy three CF children older than 5 years (and max 15 years old at the initial evaluation) attending the same CF center were followed during a period of 4 years. Data collection included spirometry, height, weight, sputum cultures and total IgG. Median age at the start was 10 years. IgG z scores<2 SD were seen in 2.7% of patients in 2004 and 2008. Twelve patients (16%) had an IgG>2 SD in 2004 and this number increased to 18 (25%) in 2008. IgG z-scores were inversely correlated with FEV(1)% predicted (r=-0.323 in 2004; p<.001). In longitudinal evaluation, changes in IgG z-score correlate inversely with changes in FEV(1)% predicted (r=-0.498; p<.001). We can conclude that even for CF patients treated according to current standards IgG z-score increases with age and is correlated with a decline in FEV(1)., (Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)

The impulse oscillation system (IOS) has been developed recently to measure respiratory system resistance (Rrs) and reactance (Xrs) at different frequencies up to > or = 25 Hz. IOS has, however, not been validated against established techniques. This study compared IOS with the classical pseudorandom noise forced oscillation technique (FOT) and body plethysmographic airway resistance (Raw) in 49 subjects with a variety of lung disorders and a wide range of Raw (0.10-1.28 kPa x L(-1) x s). Rrs,IOS was slightly greater than Rrs,FOT, especially at lower frequencies, with a mean +/- SD difference at 5-6 Hz of 0.14 +/- 0.09 kPa x L(-1) x s. Comparisons with the wave-tube technique applied on two analogues indicated an overestimation by IOS. Xrs,IOS and Xrs,FOT were very similar, with a slightly higher resonant frequency with IOS than with FOT (mean difference +/- SD 1.35 +/- 3.40 Hz). Raw was only moderately correlated with Rrn,FOT and Rrs-IOS; although the mean differences were small (0.04 +/- 0.14 kPa x L(-1)s for Rrs6,FOT and -0.10 +/- 0.14 kPa x L(-1) x s for Rrs5,IOS), IOS and FOT markedly underestimated high resistance values. In conclusion, the impulse oscillation system yields respiratory system resistance and reactance values similar, but not identical to those provided by the forced oscillation technique.

In cystic fibrosis patients with Pseudomonas aeruginosa colonization and increasing pulmonary infection, ceftazidime 150 mg/kg/day was compared with 320 mg/kg/day. Changes in clinical findings, laboratory tests, pulmonary function and chest radiographs were evaluated after 14 days of treatment in hospital. Both treatments were associated with a significant improvement, but the higher dose did not offer an additional benefit. An increase in alanine aminotransferase (ALT) occurred after both treatments; with a significantly greater increase after the high-dose therapy (mean increase +/- S.E.M. 8% +/- 2% vs 2% +/- 1 %; P < 0.01). All but one of the ALT values after treatment were within normal limits.

Unlabelled: In patients with cystic fibrosis (CF) and chronic colonisation with Pseudomonas aeruginosa, specific anti-pseudomonal IgG and IgA, as well as serum immunoreactive protein C, WBC and differential count, ESR, pulmonary function and chest radiograph score were determined before and after a 2 week intravenous course of anti-pseudomonal antibiotics in 32 cases of acute exacerbation of pulmonary infection. Specific anti-pseudomonal IgA but not specific anti-pseudomonal IgG decreased significantly after treatment. Log of anti-pseudomonal IgA but not log anti-pseudomonal IgG correlated well with disease severity as assessed by the Brasfield chest radiograph score (r 0.57), forced expiratory volume in 1 s (r 0.6) as well as C-reactive protein (r 0.62)., Conclusion: Specific anti-pseudomonal IgA may be a better parameter than specific IgG in the follow up of lung infection in patients with CF, probably because it more closely reflects ongoing endobronchial infection, the major pathology in CF lungs.

Twenty-one patients with cystic fibrosis and chronic Pseudomonas lung infection were treated at random with ceftazidime, 150 mg/kg/day, or with piperacillin, 300 mg/kg/day, and tobramycin, 10 and more mg/kg/day for 14 days. On admission and at discharge, body weight, erythrocyte sedimentation rate, white blood cell count, and differential were determined. Pulmonary function analysis and chest X-rays were also obtained on both occasions as was sputum bacteriology. After hospitalization, the patients were followed in the outpatient department for 14-26 months. Both treatments were associated with significant improvement in most of the parameters that were studied, but neither treatment was superior.

Background: In the management of cystic fibrosis (CF), rhDNase-I inhalation is widely used to facilitate the removal of the highly viscous and elastic mucus (often called sputum) from the lungs. However, an important group of CF patients does not benefit from rhDNase-l treatment. A study was undertaken to elucidate the reason for the failure of rhDNase-I in these patients and to evaluate strategies to overcome this. Methods: The biochemical properties, physical properties, and degradation by rhDNase-I of sputum obtained from clinical responders and non-responders to rhDNase-I were compared, and the ability of magnesium to reactivate rhDNase-I in DNA solutions and in sputum was investigated. The effect of oral magnesium supplements on magnesium levels in the sputum of patients with CF was also examined. Results: Sputum from clinical responders was extensively degraded in vitro on incubation with rhDNase-I, while sputum from clinical non-responders was not degraded: the median decrease in sputum elasticity in the two groups was 32% and 5%, respectively. Sputum from clinical responders contained significantly higher concentrations of magnesium than sputum from non-responders (2.0 mM v 1.3 mM; p=0.020). Sputum that could not be degraded by rhDNase-I became degradable after preincubation with magnesium. The effect of magnesium on rhDNase-I activity was mediated through actin. Oral intake of magnesium enhanced the magnesium concentration in the sputum of CF patients. Conclusion: Increasing the magnesium concentration in sputum by, for example, oral magnesium supplements may be a promising new strategy to overcome the failure of rhDNase-I in patients with CF. [ABSTRACT FROM AUTHOR]

Background: In patients with symptoms suggestive of cystic fibrosis (CF) and intermediate sweat chloride values (30-60 mmol/l), extensive CFTR gene mutation analysis and nasal potential difference (NPD) measurement are used as additional diagnostic tests and a positive result in either test provides evidence of CFTR dysfunction. To define the phenotype of such patients and confirm the validity of grouping them, patients with intermediate sweat chloride values in whom either additional CF diagnostic test was abnormal were compared with subjects in whom this was not the case and patients with classic CF. Methods: The phenotypic features of four groups were compared: 59 patients with CFTR dysfunction, 46 with an intermediate sweat chloride concentration but no evidence of CFTR dysfunction (CF unlikely), 103 patients with CF and pancreatic sufficiency (CF-PS) and 62 with CF and pancreatic insufficiency (CF-PI). Results: The CFTR dysfunction group had more lower respiratory tract infections (p = 0.01), more isolation of CF pathogens (p<0.001) and clubbing (p = 0.001) than the CF unlikely group, but less frequent respiratory tract infections with CF pathogens than the CF-PS group (p = 0.05). Patients in the CF-PS group had a milder phenotype than those with PI. Many features showed stepwise changes through the patient groups. Conclusion: Patients with intermediate sweat chloride values and two CFTR mutations or an abnormal NPD measurement have a CF-like phenotype compatible with CFTR dysfunction and, as a group, differ phenotypically from patients with intermediate sweat chloride values in whom further CF diagnostic tests are normal as well as from CF-PS and CF-PI patients. [ABSTRACT FROM AUTHOR]

Abstract: Background: Published studies concerning the impact of specialist care on lung disease in cystic fibrosis remain limited and most are either biased due to comparison with historical controls and/or underpowered. Methods: In this retrospective multicentric study, data from all CF children fulfilling the following criteria were collected: 1) Age 6–<18 at the end of 2003; 2) diagnosis before 8 y; 3) follow-up in an accredited CF Belgian centre; 4) at least 1 spirometry and respiratory culture available for 2003. Group A included children referred ≥2 years after the diagnosis. Patients from Group A were then matched with a single early referred patient on the basis of 2 criteria: same centre, as closest age as possible (Group B). Results: Data from 217 children were collected (Group A: 67/217). Late referred patients had a lower FEV1 (77.2%±22.4 vs 86.7% pred.±19.4, p =0.01) and a higher prevalence of Pseudomonas aeruginosa (38.6 vs 17.5%, p <0.05). Conclusion: In this population of CF children, a delay of 6.1 y (vs 0.1 y) between diagnosis and referral to a specialist clinic resulted in poorer respiratory outcome at age 13. [Copyright &y& Elsevier]