Surfactant function in neonates with respiratory distress syndrome

The function of pulmonary surfactant of a group of 14 preterm neonates (birth weight 907 +/- 60 g) who suffered from severe respiratory distress syndrome (RDS) and who had received exogenous bovine lipid extracted surfactant on the first day of life was compared to that in a second group of 8 neonates (birth weight 940 +/- 110 g) with mild RDS who had not received surfactant treatment. Mechanical respiratory support from day 2 on was the same in both groups. The minimal surface tension (gamma(min)) improved steadily, falling from about 30 mN/m initially to less than 20 mN/m before extubation, A consistent but loose correlation was found between gamma(min) and mechanical respiratory support necessary, as quantitated by the oxygenation index. Total protein was about 0.8 +/- 0.2 mg/mg of phospholipids and did not change during the first week of life. There were no correlations between total protein and gamma(min) or the oxygenation index. The data suggest that inhibition of surfactant function by proteins leaked into the airspaces does not play a major role during recovery from RDS, Instead, endogenous remodelling of surfactant might be of greater relevance

In Vitro Inhibition of Neutrophil Elastase Activity by Inhaled Anti-Pseudomonas Antibiotics Used in Cystic Fibrosis Patients

Background. Inhaled antibiotics are commonly used in the treatment of cystic fibrosis lung disease. A previous study suggested neutrophil elastase activation by colistin in vitro. Here, we investigated direct effects of the commonly used antibiotics colistin and tobramycin on neutrophil elastase activity. Methods. Neutrophil elastase was measured spectrophotometrically. The antibiotics colistin and tobramycin were added in different concentrations with or without the addition of albumin. Results. Generally, neutrophil elastase activity was lower in the absence of albumin compared to its presence. Both antibiotics, colistin and tobramycin, had inhibitory effects on neutrophil elastase activity except for high concentrations of colistin when albumin was absent. Conclusions. Our results suggest inhibitory effects of colistin and tobramycin in vitro. There was a clear dependency of neutrophil elastase measurements on the presence of albumin. Clinical studies are needed to investigate potential direct effects of inhaled antibiotics on neutrophil elastase activity in cystic fibrosis airways

Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome.

The state of oligomerization of surfactant associated protein-A (SP-A) monomers differs between individuals. This likely affects SP-A's functional properties and could thereby influence clinical status in patients with lung diseases. In this study we focus on SP-A structure in cystic fibrosis (CF) compared to both healthy subjects and disease controls. SP-A composition and function were assessed in both bronchoalveolar lavage (BAL) fluid and serum of 46 CF patients with mild disease, 25 patients with chronic bronchitis and 22 healthy subjects by gel chromatography and a functional agglutination assay. Relation of SP-A agglutination ability to disease severity of the subjects was explored. SP-A was present in seven major oligomeric forms with the majority of SP-A being structurally organized as complex oligomeric forms. More complex oligomeric forms were associated with better SP-A function with regard to its agglutination ability. These forms were more frequently observed in BAL than in serum, but there were no differences between disease groups. In CF patients, more complex forms of SP-A were associated with better lung function. Organizational structure of SP-A affects its functional activity and is linked to disease severity in CF

Case Report: Pediatric Renal Sarcoidosis and Prognostic Factors in Reviewed Cases

Background: Pediatric sarcoidosis is a complex inflammatory disorder with multisystemic manifestations. Kidney involvement in children is rare, and prognostic factors are unknown. Case Report and Methods: We report the case of a 16-year-old girl with multiorgan sarcoidosis and renal involvement. The patient presented with tubulointerstitial nephritis, acute kidney injury (AKI), chest CT disseminated noduli, granulomatous iridocyclitis, giant-cell sialadenitis, and arthralgia. The kidney biopsy revealed non-granulomatous interstitial nephritis. Treatment consisted of initial high-dose methylprednisolone pulse followed by oral prednisolone and methotrexate. Full remission was achieved. In addition, we performed a literature review using PubMed and analyzed data on pediatric renal sarcoidosis cases. Results: We identified 36 cases of pediatric sarcoidosis with renal involvement on presentation and data on the end-of-follow-up glomerular filtration rate (GFR). The data from the literature review showed that renal involvement was slightly more prevalent in males (60%). AKI was present in most of the described patients (84%). Oral prednisolone was used in 35 of 36 cases; in more severe cases, other immunosuppressants were used. We newly identified renal concentration impairment and granulomatous interstitial nephritis as factors with a clear trend toward GFR loss at the end of follow-up, emphasizing the importance of kidney biopsy in symptomatic patients. In contrast, higher GFR at presentation and hypercalcemia were rather favorable factors. According to the identified predictive factors, our patient has a good prognosis and is in remission. Conclusion: The factors indicating a trend toward an unfavorable renal outcome in pediatric sarcoidosis are renal concentration impairment and granulomatous interstitial nephritis at presentation, while a higher GFR is beneficial

Acid and non-acid gastro-esophageal refluxes in children with chronic pulmonary diseases

SummaryObjectivesAcid gastro-esophageal reflux has been shown associated with chronic pulmonary diseases. The role of non-acid refluxes in these children is still unknown. Therefore we investigated the prevalence of acid and non-acid refluxes, and their association with desaturations, in children with chronic pulmonary symptoms.MethodsIn 25 children aged 6 months to 15 years with unexplained chronic cough, wheeze or sputum production, refluxes were assessed by 24h-multiple intra-esophageal impedance measurements, simultaneous pH metry and continuous recording of oxygen saturation.ResultspH in the proximal and distal esophagus as well as six impedance channels were evaluated in all subjects. A mean of 129.4 refluxes per day per patient was detected. Complete and technically usable readings of oxygen saturation were obtained in 14 children. In this group the subjects had a mean of 112.6 refluxes and 92.6 desaturations per day per patient. The symptom index and symptom sensitivity index for acid refluxes were 34.7% and 24.6%, respectively, for non-acid refluxes 3.0% and 66.7%, respectively.ConclusionsWe found a high prevalence of acid reflux and a very low number of non-acid refluxes in this population. The symptom index was negative for all types of reflux, whereas the symptom sensitivity index was positive for both acid and non-acid reflux. Our data support a relation between acid gastro-esophageal refluxes and chronic pulmonary symptoms; however, this study does not support a role of non-acid reflux in children with respiratory symptoms, which are not on antacid medication

In-Vitro MPI-Guided IVOCT Catheter Tracking in Real Time for Motion Artifact Compensation

Purpose: Using 4D magnetic particle imaging (MPI), intravascular optical coherence tomography (IVOCT) catheters are tracked in real time in order to compensate for image artifacts related to relative motion. Our approach demonstrates the feasibility for bimodal IVOCT and MPI in-vitro experiments. Material and Methods: During IVOCT imaging of a stenosis phantom the catheter is tracked using MPI. A 4D trajectory of the catheter tip is determined from the MPI data using center of mass sub-voxel strategies. A custom built IVOCT imaging adapter is used to perform different catheter motion profiles: no motion artifacts, motion artifacts due to catheter bending, and heart beat motion artifacts. Two IVOCT volume reconstruction methods are compared qualitatively and quantitatively using the DICE metric and the known stenosis length. Results: The MPI-tracked trajectory of the IVOCT catheter is validated in multiple repeated measurements calculating the absolute mean error and standard deviation. Both volume reconstruction methods are compared and analyzed whether they are capable of compensating the motion artifacts. The novel approach of MPI-guided catheter tracking corrects motion artifacts leading to a DICE coefficient with a minimum of 86% in comparison to 58% for a standard reconstruction approach. Conclusions: IVOCT catheter tracking with MPI in real time is an auspicious method for radiation free MPI-guided IVOCT interventions. The combination of MPI and IVOCT can help to reduce motion artifacts due to catheter bending and heart beat for optimized IVOCT volume reconstructions.Comment: 19 pages, 11 figure

Pott's disease: a major issue for an unaccompanied refugee minor

Chest clinic The incidence of TB in children in Germany has been a rise since 2008, especially among foreign-born individuals. With rapidly increasing numbers of refugees from the numerous areas of conflict, this increase in incidence is not expected to halt, neither in Germany in Europe in general. We report a case of insufficient tracking in a 16-year-old unaccompanied refugee minor from Somalia who had a positive interferon. release assay on arrival in Germany. No actions were undertaken, until 6 months later, an X-ray showed prominent hilar enlargement. Nine months later, the patient presented to our hospital with abdominal pain, vomiting and B symptoms. Workup revealed a paravertebral abscess due to Pott's disease, a skeletal manifestation of Mycobacterium tuberculosis disease. The patient made a full recovery after a combination therapy for a total of 9 months

Serum KL-6 is a predictor of outcome in pulmonary alveolar proteinosis

Background: Pulmonary alveolar proteinosis (PAP) is a rare disorder characterised by abundant alveolar accumulation of surfactant lipoproteins. Serum levels of KL-6, high molecular weight human MUC1 mucin, are increased in the majority of patients with PAP. The prognostic significance of KL-6 in PAP is still unknown. Aim of the study was to evaluate whether serum KL-6 levels correlate with the outcome of the disease. Patients and methods: From 2006 to 2012, we prospectively studied 33 patients with primary autoimmune PAP. We measured serum KL-6 levels by ELISA (Eisai, Tokyo, Japan), and evaluated the correlation between initial KL-6 levels and clinical variables. Disease progression was defined as deterioration of symptoms, and/or lung function, and/or chest imaging. Main results: The initial serum KL-6 levels were significantly correlated with the baseline PaO2, A-aDO(2), DLCO, VC and TLC (p=0.042, 0.012, 0.012, 0.02 and 0.013, respectively). The change over time of serum KL-6 correlated with the change over time of DLCO (p=0.017). The initial serum KL-6 levels were significantly higher in patients with disease progression than in those with remission (p<0.001). At a cut-off level of 1526 U/mL, the initial serum KL-6 level predicted disease progression (Se 81\%, Sp 94\%). At a cut-off level of 2157 U/mL, the initial serum KL-6 predicted the necessity of repeated whole lung lavage (Se 83\%, Sp 96\%). In the multivariate analysis, the initial serum level of KL-6 was the strongest predictor of disease progression (HR 9.41, p=0.008). Conclusions: Serum KL-6 seems to predict outcome in PAP