Evolutionary Patterns of Modularity in the Linkage Systems of the Skull in Wrasses and Parrotfish.

The concept of modularity is fundamental to understanding the evolvability of morphological structures and is considered a central framework for the exploration of functionally and developmentally related subsets of anatomical traits. In this study, we explored evolutionary patterns of modularity and integration in the 4-bar linkage biomechanical system of the skull in the fish family Labridae (wrasses and parrotfish). We measured evolutionary modularity and rates of shape diversification of the skull partitions of three biomechanical 4-bar linkage systems using 205 species of wrasses (family: Labridae) and a three-dimensional geometric morphometrics data set of 200 coordinates. We found support for a two-module hypothesis on the family level that identifies the bones associated with the three linkages as being a module independent from a module formed by the remainder of the skull (neurocranium, nasals, premaxilla, and pharyngeal jaws). We tested the patterns of skull modularity for four tribes in wrasses: hypsigenyines, julidines, cheilines, and scarines. The hypsigenyine and julidine groups showed the same two-module hypothesis for Labridae, whereas cheilines supported a four-module hypothesis with the three linkages as independent modules relative to the remainder of the skull. Scarines showed increased modularization of skull elements, where each bone is its own module. Diversification rates of modules show that linkage modules have evolved at a faster net rate of shape change than the remainder of the skull, with cheilines and scarines exhibiting the highest rate of evolutionary shape change. We developed a metric of linkage planarity and found the oral jaw linkage system to exhibit high planarity, while the rest position of the hyoid linkage system exhibited increased three dimensionality. This study shows a strong link between phenotypic evolution and biomechanical systems, with modularity influencing rates of shape change in the evolution of the wrasse skull.

Cardiopulmonary fitness is strongly associated with body cell mass and fat-free mass: The Study of Health in Pomerania (SHIP).

Peak oxygen uptake (VO2peak) is commonly indexed by total body weight (TBW) to determine cardiopulmonary fitness (CPF). This approach may lead to misinterpretation, particularly in obese subjects. We investigated the normalization of VO2peak by different body composition markers. We analyzed combined data of 3848 subjects (1914 women; 49.7%), aged 20-90, from two independent cohorts of the population-based Study of Health in Pomerania (SHIP-2 and SHIP-TREND). VO2peak was assessed by cardiopulmonary exercise testing. Body cell mass (BCM), fat-free mass (FFM), and fat mass (FM) were determined by bioelectrical impedance analysis. The suitability of the different markers as a normalization variable was evaluated by taking into account correlation coefficients (r) and intercept (α-coefficient) values from linear regression models. A combination of high r and low α values was considered as preferable for normalization purposes. BCM was the best normalization variable for VO2peak (r = .72; P ≤ .001; α-coefficient = 63.3 mL/min; 95% confidence interval [CI]: 3.48-123) followed by FFM (r = .63; P ≤ .001; α-coefficient = 19.6 mL/min; 95% CI: -57.9-97.0). On the other hand, a much weaker correlation and a markedly higher intercept were found for TBW (r = .42; P ≤ .001; α-coefficient = 579 mL/min; 95% CI: 483 to 675). Likewise, FM was also identified as a poor normalization variable (r = .10; P ≤ .001; α-coefficient = 2133; 95% CI: 2074-2191). Sex-stratified analyses confirmed the above order for the different normalization variables. Our results suggest that BCM, followed by FFM, might be the most appropriate marker for the normalization of VO2peak when comparing CPF between subjects with different body shape.

Is palivizumab effective as a prophylaxis of respiratory syncytial virus infections in cystic fibrosis patients? A meta-analysis

BACKGROUND: Infections by respiratory syncytial virus (RSV) are more severe in patients with cystic fibrosis (CF), and many CF units use palivizumab as prophylaxis; however, information about palivizumab efficacy in CF patients is almost lacking. METHODS: A literature search up to December 2012 on the morbidity of RSV bronchiolitis in CF patients and on the safety and efficacy of palivizumab in those patients was performed. A random-effects meta-analysis was conducted for those studies meeting pre-specified search criteria. Historical controls were allowed. RESULTS: The number of patients who received palivizumab was 354 and the hospital admission rate was 0.018 (95% CI 0.0077-0.048). The corresponding number in the non-treated groups was 463 patients with an admission rate of 0.126 (95% CI 0.086-0.182) (Q = 13.9; p < 0.001). CONCLUSION: Palivizumab may have a role in the prevention of severe lower airway infection by RSV in CF patients

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Is palivizumab effective as a prophylaxis of respiratory syncytial virus infections in cystic fibrosis patients? A meta-analysis.

BACKGROUND: Infections by respiratory syncytial virus (RSV) are more severe in patients with cystic fibrosis (CF), and many CF units use palivizumab as prophylaxis; however, information about palivizumab efficacy in CF patients is almost lacking. METHODS: A literature search up to December 2012 on the morbidity of RSV bronchiolitis in CF patients and on the safety and efficacy of palivizumab in those patients was performed. A random-effects meta-analysis was conducted for those studies meeting pre-specified search criteria. Historical controls were allowed. RESULTS: The number of patients who received palivizumab was 354 and the hospital admission rate was 0.018 (95% CI 0.0077-0.048). The corresponding number in the non-treated groups was 463 patients with an admission rate of 0.126 (95% CI 0.086-0.182) (Q=13.9; p<0.001). CONCLUSION: Palivizumab may have a role in the prevention of severe lower airway infection by RSV in CF patients.

Extensive sequence analysis of CFTR, SCNN1A, SCNN1B, SCNN1G and SERPINA1 suggests an oligogenic basis for cystic fibrosis-like phenotypes.

The term cystic fibrosis (CF)-like disease is used to describe patients with a borderline sweat test and suggestive CF clinical features but without two CFTR(cystic fibrosis transmembrane conductance regulator) mutations. We have performed the extensive molecular analysis of four candidate genes (SCNN1A, SCNN1B, SCNN1G and SERPINA1) in a cohort of 10 uncharacterized patients with CF and CF-like disease. We have used whole-exome sequencing to characterize mutations in the CFTR gene and these four candidate genes. CFTR molecular analysis allowed a complete characterization of three of four CF patients. Candidate variants in SCNN1A, SCNN1B, SCNN1G and SERPINA1 in six patients with CF-like phenotypes were confirmed by Sanger sequencing and were further supported by in silico predictive analysis, pedigree studies, sweat test in other family members, and analysis in CF patients and healthy subjects. Our results suggest that CF-like disease probably results from complex genotypes in several genes in an oligogenic form, with rare variants interacting with environmental factors.

Trasplante pediátrico pulmonar / Pediatric lung transplantation

El trasplante pulmonar constituye la última alternativa para los niños con una enfermedad pulmonar terminal cuando se han agotado todo los recursos terapéuticos. En las dos últimas décadas se ha experimentado una vance consideable en la supervivencia de lso niños trasplantados de pulmón, por las mejoras en la técnica del trasplante, la preervación de los órganos, el manejo perioperatorio y la inmunosupresión. El programa de trasplatne pulmonar pediátrico en el Hospital Universitari Vall d´Hebron se inició en el año 1996. En nuestro hospital se han realizado 672 trasplantes entre los años 1990 y 2012, de los que 51 (7,6%) corresponde a trasplantes pediátricos (menores de 18 años). La indicación más frecuente para el trasplante en los iños es la fibrosis quística. En nuestro centro, la fibrosis quística ha representado solo el 8,7% de los traplantes realizados enmenores de 11 años y el 86% de los realizados entre 12 y 17 años. Los otros grupos importantes de indicaciones los constituyen las enfermedades vasculares pulmonares (hipertensión pulmonar) y las neumopatías intersticiales crónicas. La superviviencia de los niños trasplantados en nuestro entro es similar a la reflejada en el registro internacional: 55% a los 5 años y 47% a los 7 años (AU)

Lung trasplantation has become an accepted therapeutic option for children with end-stage lung disease. Advances have been made over the last 2 decades in lung transplantation concerning the procurement and presevation of donor lung, surgical techniques, early post-operative care and immunosuppression. The pediatric lung transplant program at Hospital Universitari Vall d´Heborn was started in 1996. A total of 672 lung tansplants have been performed in our hospital since 1990, with 51 (7.6%) corresponding to children (younger than 18 years). Cystic fibrosis is the leading cause for pediatric patients to requere lung transplantation. In our hospital cystic fibrosis represent only 8.7% of lung transplants in children less tha 11 years-old, but 86% in children 12-17 years old. Other important indications for lung trnasplantation in children are pediatric forms of interstitial lung dieases and pulmonary vascular disorders (mainly pulmonary hypertension). Actuarial survival is our center (55% at 5 years and 47% at 7 years) is similar to survival published in the International Registry (AU)

Induced sputum cell count and cytokine profile in atopic and non-atopic children with asthma.

BACKGROUND AND AIM: Sputum induction is a semi-invasive technique used to detect and monitor airway inflammation. In this study, the cell profile, and Th1 and Th2 cytokine levels in induced sputum of asthmatic and healthy children (HC) are compared. METHODS: Sputum induction was performed in healthy and asthmatic children by inhalation of hypertonic saline solution. Differential cell count in the specimen obtained was carried out using optic microscopy. IFN-γ, IL-2, IL-10, IL-8, IL-6, IL-4, IL-5, IL-1ß, TNF-α, and IL-12p70 levels were determined in sputum sample supernatants by flow cytometry. RESULTS: Sputum induction was performed in 31 HC and 77 asthmatic children (60 atopic and 17 non-atopic asthma, NAA). Twenty-four samples were obtained in HC and 64 in patients. Median eosinophil count in atopic asthma (AA; 2%) was higher than in NAA (P = 0.02) or HC (P = 0.01). IL-4, IL-5, IFNγ, IL-2, and IL-12p70 concentrations were higher in AA than in NAA or HC. IL-8 was higher in asthmatic children (atopic and non-atopic) than in healthy ones. IL-10 was higher in the healthy group than in the AA group (P = 0.02). CONCLUSIONS: As compared to HC, the inflammatory profile in induced sputum of children with asthma showed an increase in proinflammatory cytokines. Concentrations of IL-10, an anti-inflammatory cytokine, were lower in children with AA than in HC.

Valor del óxido nítrico nasal en el diagnóstico de la discinesia ciliar primaria / Value of nasal nitric oxide in the diagnosis of primary ciliary dyskinesia

Objetivo: Comunicar los valores de óxido nítrico nasal (ONn) en niños con discinesia ciliar primaria (DCP) comparados con los niveles de ONn en niños sanos y en niños afectos de asma, fibrosis quística (FQ) y bronquiectasias pos infecciosas. Pacientes y métodos: Se realizó la determinación de ONn en 9 niños con DCP, 36 niños asmáticos, 31 niños con FQ, 8 niños con bronquiectasias post infecciosas y 37 niños sanos. Se compararon los valores de ONn en las diferentes patologías y se determinó la sensibilidad y la especificidad de la prueba para el diagnóstico de DCP. Resultados: Todos los niños con DCP excepto uno (ONn 348 ppb) mostraron un valor de ONn inferior a 112 ppb, siendo la media de 88 ppb (IC95% 9,6–166). En los niños sanos, la media del ONn fue de 898 ppb (IC95% 801–995), en los asmáticos 1023 ppb (IC95% 911–1137), en los niños con FQ 438 ppb (IC95% 367–508) y en los pacientes con bronquiectasias pos infecciosas de 361 ppb (IC95% 252–470). El valor medio de ONn fue significativamente inferior (p<0,05) en los niños afectos de DCP respecto a todos los demás grupos. Un punto de corte de NOn ≤112 ppb mostró una sensibilidad del 88,9% y una especificidad del 99,1% para el diagnóstico de DCP [área bajo la curva ROC 0,98 (IC95% 0,94–0,99); p<0,0001; razón de probabilidad 95,1]. Conclusiones: Un valor de ONn ≤ 112 ppb en niños es altamente sugestivo de DCP aunque un valor superior no descarta por completo la enfermedad (AU)

Objective: The aim of this study is to report nasal nitric oxide (nNO) values in children with primary ciliary dyskinesia (PCD) and to compare them with nNO values in healthy children, asthmatic children, children with cystic fibrosis and children with post infectious bronchiectasis. Patients and methods: We determined nNO values in 9 children with PCD, 36 asthmatic children, 31 children with cystic fibrosis, 8 children with post infectious bronchiectasis and 37 healthy children. We compared nNO values between these different conditions and calculated sensitivity and specificity of nNO to diagnose PCD. Results: All children with PCD - except one (nNO 348 ppb) – had nNO values below 112 ppb, mean 88 ppb (95%CI 9.6–166). The nNO mean was 898 ppb (95%CI 801-995) in healthy children, 1023 ppb (95%CI 911–1137) in asthmatic children, 438 ppb (95%CI 367–508) in cystic fibrosis children and 361 ppb (95%CI 252–470) in children with post infectious bronchiectasis. The mean concentration of nNO was lower (P<0.05) in PCD patients, compared to the other groups. The measurement of nasal NO in our study population showed, at a cut-off level of ≤112 ppb, a sensitivity of 88.9% and a specificity of 99.1% in the diagnosis of PCD [ROC 0.98 (95%CI 0.94–0.99); P<0.0001; probability ratio 95.1]. Conclusions: The measurement of nasal NO appears to be a useful tool for screening children for PCD, in which a cut-off level of ≤112 ppb suggests the disease, although nNO above 112 ppb does not exclude PCD (AU)

[Value of nasal nitric oxide in the diagnosis of primary ciliary dyskinesia]. / Valor del óxido nítrico nasal en el diagnóstico de la discinesia ciliar primaria.

OBJECTIVE: The aim of this study is to report nasal nitric oxide (nNO) values in children with primary ciliary dyskinesia (PCD) and to compare them with nNO values in healthy children, asthmatic children, children with cystic fibrosis and children with post infectious bronchiectasis. PATIENTS AND METHODS: We determined nNO values in 9 children with PCD, 36 asthmatic children, 31 children with cystic fibrosis, 8 children with post infectious bronchiectasis and 37 healthy children. We compared nNO values between these different conditions and calculated sensitivity and specificity of nNO to diagnose PCD. RESULTS: All children with PCD - except one (nNO 348 ppb) - had nNO values below 112 ppb, mean 88 ppb (95%CI 9.6-166). The nNO mean was 898 ppb (95%CI 801-995) in healthy children, 1023 ppb (95%CI 911-1137) in asthmatic children, 438 ppb (95%CI 367-508) in cystic fibrosis children and 361 ppb (95%CI 252-470) in children with post infectious bronchiectasis. The mean concentration of nNO was lower (P<0.05) in PCD patients, compared to the other groups. The measurement of nasal NO in our study population showed, at a cut-off level of < or =112 ppb, a sensitivity of 88.9% and a specificity of 99.1% in the diagnosis of PCD [ROC 0.98 (95%CI 0.94-0.99); P<0.0001; probability ratio 95.1]. CONCLUSIONS: The measurement of nasal NO appears to be a useful tool for screening children for PCD, in which a cut-off level of < or =112 ppb suggests the disease, although nNO above 112 ppb does not exclude PCD.

Inducción de esputo en niños: desarrollo técnico / Sputum induction in children: technical development

Objetivos: Comparar el rendimiento (total de muestras obtenidas) de nebulizadores ultrasónicos de flujo bajo y alto en la inducción de esputo en niños asmáticos, y valorar los efectos adversos asociados. Pacientes y métodos: Se nebulizó suero salino hipertónico a concentraciones crecientes (3%, 4%, 5%) utilizando nebulizadores ultrasónicos de bajo flujo (Omron NE-U07(R); flujo 1ml/min) y de alto flujo (Omron NE-U12(R); flujo 3 ml/min, y DeVilbiss Ultraneb 3000(R); flujo 2,5ml/min). Resultados: Se realizaron 49 inducciones en 49 pacientes entre 7 y 15 años de edad (en 15 niños se utilizó un nebulizador de bajo flujo y en 34 niños un nebulizador de alto flujo (Omron NEU12(R): 6 casos, DeVilbiss Ultraneb 3000(R): 28 casos). Se obtuvieron 37 muestras. Treinta y seis presentaban<20% de células escamosas y 26 tenían una viabilidad ≥60%. El rendimiento de la prueba fue mayor con los nebulizadores de alto flujo (85,3% de muestras), frente al 53% (p=0,04). El 69% de las muestras obtenidas con los nebulizadores de alto flujo fueron válidas, frente al 62,5% con el de bajo flujo (p=0,7). Con los nebulizadores de alto flujo disminuyó la incidencia de tos (17,6%, p=0,08) y de picor de ojos (0%, p=0,02), respecto al nebulizador de bajo flujo (47% y 20% respectivamente), aunque aumentó el sabor desagradable (82,3%, p<0,001) y la sialorrea (14,7%, p=0,3). Conclusiones: Con los nebulizadores ultrasónicos de alto flujo se consigue un mayor rendimiento de la técnica sin que se observe un aumento de efectos adversos significativos (AU)

Objective: To compare low and high flow nebulizers performance (total of samples) and its side effects on sputum induction in asthmatic children. Patients and methods: Sputum induction was performed by inhalation of a hypertonic saline solution at increasing concentrations (3%, 4% and 5%) using low flow (OMRON NE-U07(R); flow rate 1ml/min), or high flow (OMRON NE-U12(R); flow rate 3ml/min, and DeVilbiss Ultraneb 3000(R); flow rate 2.5ml/min) ultrasonic nebulizers. Results: We performed 49 inductions in 49 patients from 7 to 15 years old (in 15 children we used a low flow nebulizer (Omron NE-U07(R)) and in 34 children a high flow nebulizer (OMRON NEU12(R), 6 patients, and DeVilbiss Ultraneb 3000(R), 28 patients). We obtained 37 samples of which 36 had less than 20% of squamous cells, and 26 had a viability ≥60%. The test performance was higher with high-flow nebulizers, obtaining 85.3% of samples compared to 53% (p=0.04). A total of 69% of samples obtained with the high flow nebulizer were valid, compared to 62.5% (p=0.7) with the low flow nebulizers. With high flow rate nebulizers the incidence of cough (17.6%, p=0.08) and itchy eyes (0%, p=0.02) decreased with the low flow nebulizer (47% and 20% respectively), but bad taste (82.3%, p <0.001) and salivation (14.7%, p=0.3) increased. Conclusions: With high flow rate ultrasonic nebulizers we obtain a higher performance of the technique without an increase in significant side effects (AU)

[Sputum induction in children: Technical development]. / Inducción de esputo en niños: desarrollo técnico.

OBJECTIVE: To compare low and high flow nebulizers performance (total of samples) and its side effects on sputum induction in asthmatic children. PATIENTS AND METHODS: Sputum induction was performed by inhalation of a hypertonic saline solution at increasing concentrations (3%, 4% and 5%) using low flow (OMRON NE-U07; flow rate 1ml/min), or high flow (OMRON NE-U12; flow rate 3ml/min, and DeVilbiss Ultraneb 3000; flow rate 2.5ml/min) ultrasonic nebulizers. RESULTS: We performed 49 inductions in 49 patients from 7 to 15 years old (in 15 children we used a low flow nebulizer (Omron NE-U07) and in 34 children a high flow nebulizer (OMRON NEU12, 6 patients, and DeVilbiss Ultraneb 3000, 28 patients). We obtained 37 samples of which 36 had less than 20% of squamous cells, and 26 had a viability > or =60%. The test performance was higher with high-flow nebulizers, obtaining 85.3% of samples compared to 53% (p=0.04). A total of 69% of samples obtained with the high flow nebulizer were valid, compared to 62.5% (p=0.7) with the low flow nebulizers. With high flow rate nebulizers the incidence of cough (17.6%, p=0.08) and itchy eyes (0%, p=0.02) decreased with the low flow nebulizer (47% and 20% respectively), but bad taste (82.3%, p <0.001) and salivation (14.7%, p=0.3) increased. CONCLUSIONS: With high flow rate ultrasonic nebulizers we obtain a higher performance of the technique without an increase in significant side effects.

Protocolo de diagnóstico y seguimiento de los pacientes con fibrosis quística / Protocol for the diagnosis and follow up of patients with cystic fibrosis

La fibrosis quística (FQ) es la enfermedad autosómica recesiva grave más frecuente de la raza caucásica. El aumento tan importante de la supervivencia de los pacientes con FQ en los últimos años se debe en gran parte a la disponibilidad de nuevos tratamientos de la enfermedad pulmonar, en especial de las infecciones respiratorias. Este protocolo revisa y actualiza los aspectos del diagnóstico y el manejo de la enfermedad respiratoria de la FQ. La prueba del sudor continúa siendo el estándar del diagnóstico de la FQ aunque, pese a realizarse de la forma adecuada, no siempre es concluyente. Se debe controlar a los pacientes con FQ en unidades especializadas por un equipo multidisciplinario y experto, mediante protocolos específicos de seguimiento clínico, estudios por imagen y pruebas funcionales respiratorias y microbiológicas que se actualizan en esta revisión. Se incluyen las recomendaciones sobre el tratamiento precoz y agresivo de la primoinfección y de la infección crónica por Pseudomonas aeruginosa, Staphylococcus aureus y otros microorganismos no habituales. Asimismo se describen los tratamientos de los diferentes aspectos de la enfermedad pulmonar y sus complicaciones y se revisan las indicaciones y el estado actual del trasplante pulmonar. Este documento de consenso ha sido elaborado por los miembros del Grupo de Trabajo de Fibrosis Quística de la Sociedad Española de Neumología Pediátrica con el objetivo de actualizar el previo, publicado en esta Revista en 1999 (AU)

Cystic fibrosis (CF) is the most common severe recessive genetic disease in Caucasians. During the last years, new therapies and aggressive management of the lung disease have contributed significantly to the increased life expectancy in CF patients. A review and update of CF diagnosis and management of lung disease are included. The sweat chloride test (SCT) remains the gold standard for CF diagnosis and should be performed properly. However, in a few patients SCT results may not be conclusive to clarify the CF diagnosis. Patients with CF should be followed up in specialist Units by an expert multidisciplinary expert applying standard clinical protocols and using lung function tests, and microbiological and imaging studies. An overview with the recommendations for treatment of early onset and chronic infections due to Pseudomonas aeruginosa, Staphylococcus aureus and other uncommon pathogens is included. Furthermore, the management of other aspects of CF lung disease and complications is provided, as well as the indications for lung transplantation. This document has been prepared by the members of the CF working group of the Spanish Paediatrics Pulmonary Society to provide an update to the earlier documents published in this Journal in 1999 (AU)

[Protocol for the diagnosis and follow up of patients with cystic fibrosis]. / Protocolo de diagnóstico y seguimiento de los pacientes con fibrosis quística.

Cystic fibrosis (CF) is the most common severe recessive genetic disease in Caucasians. During the last years, new therapies and aggressive management of the lung disease have contributed significantly to the increased life expectancy in CF patients. A review and update of CF diagnosis and management of lung disease are included. The sweat chloride test (SCT) remains the gold standard for CF diagnosis and should be performed properly. However, in a few patients SCT results may not be conclusive to clarify the CF diagnosis. Patients with CF should be followed up in specialist Units by an expert multidisciplinary expert applying standard clinical protocols and using lung function tests, and microbiological and imaging studies. An overview with the recommendations for treatment of early onset and chronic infections due to Pseudomonas aeruginosa, Staphylococcus aureus and other uncommon pathogens is included. Furthermore, the management of other aspects of CF lung disease and complications is provided, as well as the indications for lung transplantation. This document has been prepared by the members of the CF working group of the Spanish Paediatrics Pulmonary Society to provide an update to the earlier documents published in this Journal in 1999.

[Exogenous lipoid pneumonia]. / Neumonía lipoidea exógena.

The aspiration of lipoid material following the accidental ingestion of lipid formulations is the most frequent cause of exogenous lipoid pneumonia in paediatrics. The presence of cough, increasing dyspnea and chest pain, together with alveolar infiltrates in the chest radiography and the previous accidental intake of a lipid substance and vomiting should make us suspect this diagnosis. We present two cases of aspiration lipoid pneumonia in paediatric patients, with a different clinical presentation and radiological outcome, pointing out in one of them the appearance of pneumatoceles as a consequence of aspiration.

Neumonía lipoidea exógena / Exogenous lipoid pneumonia

La broncoaspiración de sustancias lipídicas tras la ingesta accidental de productos con un elevado contenido en lípidos es la causa más frecuente de neumonía lipoidea exógena en pediatría. La presencia de tos, dificultad respiratoria creciente y dolor torácico, junto con infiltrados alveolares en la radiografía de tórax y el antecedente claro de intoxicación y vómitos deben hacer sospechar este diagnóstico. Presentamos dos casos clínicos de neumonía lipoidea por aspiración en pacientes pediátricos, que difieren en su evolución clínica y radiológica. En uno de ellos destaca la aparición de neumatoceles como secuela de la aspiración (AU)

The aspiration of lipoid material following the accidental ingestion of lipid formulations is the most frequent cause of exogenous lipoid pneumonia in paediatrics. The presence of cough, increasing dyspnea and chest pain, together with alveolar infiltrates in the chest radiography and the previous accidental intake of a lipid substance and vomiting should make us suspect this diagnosis. We present two cases of aspiration lipoid pneumonia in paediatric patients, with a different clinical presentation and radiological outcome, pointing out in one of them the appearance of pneumatoceles as a consequence of aspiration (AU)

Bone marrow diffusion measures correlate with dementia severity in HIV patients.

BACKGROUND AND PURPOSE: Escalation in monocyte trafficking from the bone marrow into the brain may play a critical role in central nervous system injury and cognitive deterioration in patients with HIV infection. This study tested the hypothesis that the mean diffusivity is sensitive to marrow changes in HIV patients and that these quantitative imaging measurements correlate with the severity of dementia. METHODS: The mean diffusivity (MD), determined for clival and calvarial marrow regions, was compared in 11 HIV-infected patients and 9 control subjects. The imaging measurements were also evaluated for relationships with dementia severity and markers of disease progression (CD4 and viral load in plasma). RESULTS: The MD was significantly reduced in both clival and calvarial marrow in HIV-infected patients (P =.006). Diffusion measurements for clival (P =.02) and for calvarial (P =.03) regions were significantly correlated with the severity of dementia. CONCLUSION: The results of this investigation support the utility of diffusion strategies for monitoring the marrow and provide further evidence of a relationship between marrow status changes and neurologic progression in HIV patients.

Upregulation of COX-1 and COX-2 in nasal polyps in cystic fibrosis.

BACKGROUND: Since abnormalities in prostanoid metabolism occur in the lower airway of patients with cystic fibrosis (CF), it is likely that they could also be detected in the nose. METHODS: The degree of mRNA and protein expression of cyclo-oxygenase (COX) enzymes 1 (COX-1) and 2 (COX-2) was examined using quantitative reverse competitive polymerase chain reaction (RT-PCR) and Western blot analysis in the nasal polyps from 10 patients with CF, nasal polyps from 10 non-CF patients and 11 nasal mucosa specimens. The results are presented as 10(6) cDNA molecules/mug total RNA and the densitometric ratio between protein and beta-actin. RESULTS: COX-1 mRNA levels were significantly higher in CF nasal polyps (median 2.34, 25-75th percentiles 1.6-3.2) than in the nasal mucosa (0.78, 0.11-1.21), while there was no difference with non-CF nasal polyps (1.11, 0.80-3.15). COX-1 protein levels were significantly higher in CF nasal polyps (3.63, 2.71-4.27) than in nasal mucosa (1.55, 0.66-2.33) and non-CF nasal polyps (2.19, 1.72-3.68). COX-2 mRNA was significantly higher in CF nasal polyps (3.34, 2.42-7.05) than in nasal mucosa (1.69, 0.19-3.50). No differences were found in COX-2 mRNA expression between CF and non-CF polyps (1.38, 0.12-6.07). COX-2 protein levels were also significantly higher in CF nasal polyps (0.23, 0.04-0.34) than in non-CF nasal polyps (0.011, 0.009-0.016) or nasal mucosa (0.014, 0.014-0.016). CONCLUSIONS: Upregulation in the expression of COX-1 and COX-2 could explain the high production of prostanoids reported in CF. These findings raise questions regarding the potential use of selective or non-selective COX-2 non-steroidal anti-inflammatory treatment in CF.

Antimicrobial therapy for pulmonary pathogenic colonisation and infection by Pseudomonas aeruginosa in cystic fibrosis patients.

Pseudomonas aeruginosa colonisation has a negative effect on pulmonary function in cystic fibrosis patients. The organism can only be eradicated in the early stage of colonisation, while reduction of bacterial density is desirable during chronic colonisation or exacerbations. Monthly, or at least 3-monthly, microbiological culture is advisable for patients without previous evidence of P. aeruginosa colonisation. Cultures should be performed at least every 2-3 months in patients with well-established colonisation, and always during exacerbations or hospitalisations. Treatment of patients following the first isolation of P. aeruginosa, but with no clinical signs of colonisation, should be with oral ciprofloxacin (15-20 mg/kg twice-daily for 3-4 weeks) plus inhaled tobramycin or colistin (intravenous treatment with or without inhaled treatment can be used as an alternative), while patients with acute infection should be treated for 14-21 days with high doses of two intravenous antimicrobial agents, with or without an inhaled treatment during or at the end of the intravenous treatment. Maintenance treatment after development of chronic P. aeruginosa infection/colonisation (pathogenic colonisation) in stable patients (aged>6 years) should be with inhaled tobramycin (300 mg twice-daily) in 28-day cycles (on-off) or, as an alternative, colistin (1-3 million units twice-daily). Colistin is also a possible choice for patients aged<6 years. Treatment can be completed with oral ciprofloxacin (3-4 weeks every 3-4 months) for patients with mild pulmonary symptoms, or intravenously (every 3-4 months) for those with severe symptoms or isolates with ciprofloxacin resistance. Moderate and serious exacerbations can be treated with intravenous ceftazidime (50-70 mg/kg three-times-daily) or cefepime (50 mg/kg three-times-daily) plus tobramycin (5-10 mg/kg every 24 h) or amikacin (20-30 mg/kg every 24 h) for 2-3 weeks. Oral ciprofloxacin is recommended for patients with mild pulmonary disease. If multiresistant P. aeruginosa is isolated, antimicrobial agents that retain activity are recommended and epidemiological control measures should be established.