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1.
Zhonghua Er Ke Za Zhi ; 58(1): 19-24, 2020 Jan 02.
Artículo en Chino | MEDLINE | ID: mdl-31905471

RESUMEN

Objective: To summarize the diagnosis, clinical manifestations, treatment and prognosis of congenital cystic lung lesions. Methods: A retrospective study described the clinical course of 96 patients (46 female and 50 male) diagnosed with congenital cystic lung lesions treated at the Tianjin Children's Hospital from January 2010 to March 2019. The clinical findings, imaging examinations, pathological findings, treatment and follow-up were analyzed. Results: Totally 96 patients (aged from 4 days to 13 years) with congenital cystic lung lesions were included in this study. Eighty-six patients (90%) were diagnosed when they had cough and fever symptoms. Forty (42%) patients exhibited congenital cystic adenomatoid malformation, 30 underwent surgical excision, two were at emergency operations and one dead. There were 12 (13%) patients with pulmonary sequestration and four were surgical treated. Twelve (13%) patients with bronchogenic cyst were included and 4 were surgically treated. There were 3 (3%) patients with congenital lobar emphysema and one was surgically treated. Another patient with pneumothorax was operated in other hospital 2 months after discharge. Twenty-nine (30%) patients with unclassified congenital cystic lung lesions could not be definitively diagnosed by CT. Some of them were difficult to be distinguished from necrotizing pneumonia. Finally, 2 patients were diagnosed as necrotizing pneumonia after 6, 10 months follow-up. After operation 37 out of 39 patients recovered well. Conclusions: The diagnosis of congenital pulmonary cystic disease depend on imaging and pathological examination. Most patients are diagnosed when they have respiratory tract infection. The main clinical manifestations are cough and fever. The prognosis of operative management is good.


Asunto(s)
Quiste Broncogénico/diagnóstico , Quiste Broncogénico/cirugía , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Enfisema Pulmonar/patología , Adolescente , Quiste Broncogénico/congénito , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Masculino , Neumonectomía , Enfisema Pulmonar/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
2.
Forensic Sci Int ; 305: 110029, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31726327

RESUMEN

The question whether an injury was sustained during life or not is one of the most important subjects in forensic medicine. Therefore, vital reactions have been a main research topic in forensic medicine for a long period and many renowned forensic pathologists have devoted important papers to this field. The research area ranges from macroscopically visible organ reactions, over tissue alterations (enzyme histochemistry, later on immunohistochemistry with a wide range of enzymes and other analytes, molecular pathology) to biochemical responses to injury. Especially in the field of immunohistochemistry and molecular pathology much progress has been achieved in the last years (e.g. heat-shock-proteins or positive aquaporine3-staining in mechanical skin trauma). Furthermore, 20 years after its implementation postmortem imaging also contributes to the detection and visualization of vital signs. The aim of the present review is to provide an update on forensically relevant vital signs/vital reactions. Systemic vital reactions especially of the circulatory and respiratory system as well as local vital reactions will be addressed. Vital reactions of different organ systems will be discussed in detail regarding pathogenesis and possible postmortem evolution. Current research on immunohistochemically detectable vital reactions (heat-shock-protein expression, aquaporine3-staining in mechanical trauma of the skin) will be addressed as well as biochemical vital reactions (agonochemical stress reaction, myoglobine in electrocution death, hypoxanthine as marker of hypoxia).


Asunto(s)
Medicina Legal/métodos , Heridas y Traumatismos/patología , Catecolaminas/sangre , Tos , Deglución , Embolia/patología , Exsanguinación/patología , Hemorragia/patología , Humanos , Inmunohistoquímica , Insulina/sangre , Absorción Intestinal , Isquemia/patología , Neumotórax/patología , Proteínas/metabolismo , Enfisema Pulmonar/patología , Ventilación Pulmonar , Púrpura/patología , Aspiración Respiratoria/patología , Salivación
3.
EBioMedicine ; 46: 305-316, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31383554

RESUMEN

BACKGROUND: Cigarette smoke is the main risk factor of pulmonary emphysema development, which is characterized by alveolar wall destruction. Mitochondria are important for alveolar type II (ATII) cell metabolism due to ATP generation. METHODS: We isolated ATII cells from control non-smoker and smoker organ donors, and after lung transplant of patients with emphysema to determine mitochondrial function, dynamics and mitochondrial (mt) DNA damage. FINDINGS: We found high mitochondrial superoxide generation and mtDNA damage in ATII cells in emphysema. This correlated with decreased mtDNA amount. We also detected high TOP1-cc and low TDP1 levels in mitochondria in ATII cells in emphysema. This contributed to the decreased resolution of TOP1-cc leading to accumulation of mtDNA damage and mitochondrial dysfunction. Moreover, we used lung tissue obtained from areas with mild and severe emphysema from the same patients. We found a correlation between the impaired fusion and fission as indicated by low MFN1, OPA1, FIS1, and p-DRP1 levels and this disease severity. We detected lower TDP1 expression in severe compared to mild emphysema. INTERPRETATION: We found high DNA damage and impairment of DNA damage repair in mitochondria in ATII cells isolated from emphysema patients, which contribute to abnormal mitochondrial dynamics. Our findings provide molecular mechanisms of mitochondrial dysfunction in this disease. FUND: This work was supported by National Institutes of Health (NIH) grant R01 HL118171 (B.K.) and the Catalyst Award from the American Lung Association (K.B.).


Asunto(s)
Células Epiteliales Alveolares/metabolismo , Mitocondrias/metabolismo , Enfisema Pulmonar/etiología , Enfisema Pulmonar/metabolismo , Adenosina Trifosfato/biosíntesis , Daño del ADN , ADN Mitocondrial , Progresión de la Enfermedad , Metabolismo Energético , Humanos , Mitocondrias/genética , Estrés Oxidativo , Hidrolasas Diéster Fosfóricas/metabolismo , Transporte de Proteínas , Enfisema Pulmonar/patología , Especies Reactivas de Oxígeno/metabolismo , Humo/efectos adversos , Superóxidos/metabolismo
4.
EBioMedicine ; 45: 563-577, 2019 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-31278070

RESUMEN

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is characterized by emphysema and/or obstructive bronchiolitis. Deficiency in vitamin D3 (VD3), which regulates gene expression through binding to vitamin D receptor (VDR), is associated with high risks of COPD susceptibility. Alveolar macrophages (AM), which are generated during early ontogeny and maintained in alveoli by self-renewal in response to cytokine GM-CSF, are positively correlated with severity of emphysema. However, whether and how VD3, VDR and AM interact to contribute to COPD pathogenesis at the molecular and cellular levels are largely unknown. METHODS: We used systems biology approaches to analyze gene expression in mouse macrophages from different tissues to identify key transcription factors (TF) for AM and infer COPD disease genes. We used RNA-seq and ChIP-seq to identify genes that are regulated by VD3 in AM. We used VDR-deficient (Vdr-/-) mice to investigate the role of VD3-VDR axis in the pathogenesis of COPD and characterized the transcriptional and functional alterations of Vdr-/- AM. FINDINGS: We find that VDR is a key TF for AM and a COPD disease gene. VDR is highly expressed in AM and in response to VD3 inhibits GM-CSF-induced AM proliferation. In Vdr-/- AM, genes involved in proliferation and immune response are upregulated. Consistently, Vdr-/- mice progressively accumulate AM and concomitantly develop emphysema without apparent infiltration of immune cells into the lung tissue. Intratracheal transfer of Vdr-/- AM into wildtype mice readily induces emphysema. The production of reactive oxygen species at basal level and in response to heme or lipopolysaccharide is elevated in Vdr-/- AM and suppressed by VD3 in wildtype AM. INTERPRETATION: These results show that the VD3-VDR axis is critical to counteract GM-CSF-induced AM proliferation and defect in this regulation leads to altered AM homeostasis and function. Our findings identify that VD3 deficiency contributes to emphysema by altering AM function without contributing to bronchiolitis. Our findings also suggest possibilities of modulating the VD3-VDR axis for inhibiting emphysema in COPD patients.


Asunto(s)
Colecalciferol/genética , Factor Estimulante de Colonias de Granulocitos y Macrófagos/genética , Enfermedad Pulmonar Obstructiva Crónica/genética , Enfisema Pulmonar/genética , Receptores de Calcitriol/genética , Animales , Proliferación Celular/genética , Regulación de la Expresión Génica/genética , Homeostasis/genética , Humanos , Macrófagos Alveolares/metabolismo , Macrófagos Alveolares/patología , Ratones , Unión Proteica/genética , Enfermedad Pulmonar Obstructiva Crónica/patología , Enfisema Pulmonar/patología , Especies Reactivas de Oxígeno/metabolismo , Análisis de Secuencia de ARN , Transducción de Señal
5.
Rev Mal Respir ; 36(5): 638-642, 2019 May.
Artículo en Francés | MEDLINE | ID: mdl-31202604

RESUMEN

INTRODUCTION: Lung volume reduction can be effective in appropriately selected patients with severe emphysema and is associated with reduced breathlessness and improved survival. Spontaneous resolution of emphysematous bullae can also sometimes occur. CASE REPORT: We report a case of severe smoking-related emphysema in a 60-year-old patient, who presented in October 2013 with a right upper lobe acute community-acquired pneumonia on the background of previously undocumented emphysema. The patient improved following treatment with co-amoxiclav and serial radiology showed progressive cicatricial retraction. Nine months later there had been a major functional improvement characterized by a complete normalization of the patient's ventilatory parameters, specifically a 45% improvement in FEV1. In the literature, the average FEV1 improvement obtained by surgical or endoscopic lung volume reduction techniques does not exceed 28%. CONCLUSION: Rarely, emphysematous bullae resolve following infections. Further studies of the mechanisms involved in these natural regressions may be of interest in the development of new therapeutics.


Asunto(s)
Neumonía Asociada a la Atención Médica/complicaciones , Neumonía Asociada a la Atención Médica/patología , Pulmón/patología , Enfisema Pulmonar/complicaciones , Enfisema Pulmonar/patología , Enfermedad Aguda , Broncoscopía/métodos , Volumen Espiratorio Forzado , Neumonía Asociada a la Atención Médica/diagnóstico , Neumonía Asociada a la Atención Médica/cirugía , Humanos , Pulmón/diagnóstico por imagen , Pulmón/cirugía , Masculino , Persona de Mediana Edad , Tamaño de los Órganos , Neumonectomía , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/cirugía , Pruebas de Función Respiratoria , Fumadores
6.
J Pharmacol Sci ; 140(2): 113-119, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31248767

RESUMEN

Pulmonary emphysema, inflammation and senescence-like phenotype are pathophysiological characteristics of chronic obstructive pulmonary disease (COPD). Recently, a murine model of COPD has been established by inducing airway-specific overexpression of epithelial Na+ channel ß subunit (ßENaC-Tg mice). However, little is known about the histological and biochemical differences between ßENaC-Tg mice and an existing acute emphysematous mouse model (elastase-induced model). Here, we first utilized whole lung image-based quantification method for histological analysis to determine auto-measure parameters, including alveolar area, alveolar perimeter, (major axis + minor axis)/2 and Feret diameter. Even though the extent of emphysema was similar in both models, the coefficient of variation (CV) of all histological parameters was smaller in ßENaC-Tg mice, indicating that ßENaC-Tg mice show homogeneous emphysema as compared with elastase-induced acute model. Expression analysis of lung tissue RNAs further revealed that elastase-induced model exhibits transient changes of inflammation markers (Kc, Il-6, Lcn2) and senescence-related markers (Sirt1, p21) at emphysema-initiation stage (1 day), which does not last until emphysema-manifestation stage (3 weeks); while the up-regulation is stable at emphysema-manifestation stage in ßENaC-Tg mice (14-week old). Thus, these studies demonstrate that ßENaC-Tg mice exhibit diffuse-type emphysema with stable expression of inflammatory and senescence-like markers.


Asunto(s)
Enfermedad Pulmonar Obstructiva Crónica/genética , Enfisema Pulmonar/genética , Transcriptoma/genética , Envejecimiento/genética , Animales , Quimiocina CXCL1/genética , Quimiocina CXCL1/metabolismo , Modelos Animales de Enfermedad , Canales Epiteliales de Sodio/genética , Femenino , Mediadores de Inflamación/metabolismo , Interleucina-6/genética , Interleucina-6/metabolismo , Lipocalina 2/metabolismo , Masculino , Ratones Endogámicos C57BL , Ratones Transgénicos , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico por imagen , Enfermedad Pulmonar Obstructiva Crónica/patología , Enfisema Pulmonar/diagnóstico por imagen , Enfisema Pulmonar/patología , Sirtuina 1/genética , Sirtuina 1/metabolismo
7.
Mol Cell Biochem ; 457(1-2): 41-49, 2019 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-30993494

RESUMEN

In our previous study, we have shown that PARP-1 inhibition (genetic or pharmacological) ameliorates elastase-induced inflammation and emphysema. Since matrix metalloproteinases (MMPs) particularly MMP-2 and MMP-9 are known to play a critical role in emphysema development, the present work was designed to evaluate the effects of PARP-1 inhibition on their expression utilizing elastase-induced mouse model of emphysema. Our data show that olaparib administration at a dose of 5 mg/kg b.wt. (daily) significantly prevented the elastase-induced inflammation as indicated by decreased inflammatory cells particularly macrophages in BALF at 1 week post-injury. In addition, the drug restored the altered redox balance in the lungs of elastase-treated mice toward normal. Further, PCR data show that olaparib administration ameliorates the elastase-induced expression of MMP-2 and MMP-9 without having much effect on the expressions of their inhibitors TIMP-1 and TIMP-2. Next, our data on immunoblot, gelatin zymography, and immunohistochemical analysis indeed confirm that olaparib reduced the elastase-induced expression of MMP-2 and MMP-9. Reduction in the expression of metalloproteinases correlate well with the PARP activity as olaparib treatment suppressed the elastase-induced expression of PAR modified proteins markedly. Overall, our data strongly suggest that PARP-1 inhibition blunts elastase-induced MMP-2 and MMP-9 expression, which may be partly responsible for prevention of emphysema.


Asunto(s)
Regulación Enzimológica de la Expresión Génica/efectos de los fármacos , Macrófagos/metabolismo , Metaloproteinasa 2 de la Matriz/biosíntesis , Metaloproteinasa 9 de la Matriz/biosíntesis , Elastasa Pancreática/toxicidad , Ftalazinas/farmacología , Piperazinas/farmacología , Poli(ADP-Ribosa) Polimerasa-1/antagonistas & inhibidores , Enfisema Pulmonar/prevención & control , Animales , Modelos Animales de Enfermedad , Macrófagos/patología , Masculino , Ratones , Ratones Endogámicos BALB C , Poli(ADP-Ribosa) Polimerasa-1/metabolismo , Inhibidores de Poli(ADP-Ribosa) Polimerasas , Enfisema Pulmonar/inducido químicamente , Enfisema Pulmonar/metabolismo , Enfisema Pulmonar/patología , Inhibidor Tisular de Metaloproteinasa-1/biosíntesis , Inhibidor Tisular de Metaloproteinasa-2/biosíntesis
8.
PLoS One ; 14(3): e0213990, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30883575

RESUMEN

OBJECTIVES: To evaluate frequency and severity of complications after CT-guided lung biopsy using the Society of Interventional Radiology (SIR) classification, and to assess risk factors for overall and major complications. MATERIALS AND METHODS: 311 consecutive biopsies with a non-coaxial semi-automated 18 gauge biopsy system were retrospectively evaluated. Complications after biopsy were classified into minor SIR1-2 and major SIR3-6. Studied risk factors for complications were patient-related (age, sex and underlying emphysema), lesion-related (size, location, morphologic characteristic, depth from the pleura and histopathology), and technique-related (patient position during procedure, thoracic wall thickness at needle path, procedure time length and number of procedural CT images, number of pleural passes, fissure penetration and needle-to-blood vessel angle). Data were analyzed using logistic and ordinal regression. RESULTS: Complications were pneumothorax and pulmonary hemorrhage. The complications were minor SIR1-2 in 142 patients (45.6%), and major SIR3-4 in 25 patients (8%). SIR5-6 complications were not present. Emphysema, smaller deeply located lesion, increased puncture time length and number of procedural CT images, multiple pleural passes and fissure puncture were significant risk factors for complication severity in univariate analysis. Emphysema (OR = 8.8, p<0.001), lesion depth from the pleura (OR = 1.9 per cm, p<0.001), and fissure puncture (OR = 9.4, p = 0.01) were the independent factors for major complications in a multiple logistic regression model. No statistical difference of complication rates between the radiologists performing biopsies was observed. CONCLUSIONS: Knowledge about risk factors influencing complication severity is important for planning and performing CT-guided lung biopsies.


Asunto(s)
Biopsia Guiada por Imagen/efectos adversos , Pulmón/patología , Femenino , Hemorragia/etiología , Humanos , Biopsia Guiada por Imagen/instrumentación , Pulmón/diagnóstico por imagen , Enfermedades Pulmonares/etiología , Masculino , Persona de Mediana Edad , Neumotórax/etiología , Enfisema Pulmonar/diagnóstico por imagen , Enfisema Pulmonar/patología , Radiografía Intervencional/efectos adversos , Estudios Retrospectivos , Factores de Riesgo , Tomografía Computarizada por Rayos X
9.
Int J Mol Sci ; 20(5)2019 Mar 04.
Artículo en Inglés | MEDLINE | ID: mdl-30836679

RESUMEN

In clinical practice, there are patients with a combination of metabolic syndrome (MS) and chronic obstructive pulmonary disease (COPD). The pathological mechanisms linking MS and COPD are largely unknown. It remains unclear whether the effect of MS (possible obesity) has a major impact on the progression of COPD. This complicates the development of effective approaches for the treatment of patients with a diagnosis of MS and COPD. Experiments were performed on female C57BL/6 mice. Introduction of monosodium glutamate and extract of cigarette smoke was modeled to simulate the combined pathology of lipid disorders and emphysema. Biological effects of glucagon-like peptide 1 (GLP-1) and GLP-1 on endothelial progenitor cells (EPC) in vitro and in vivo were evaluated. Histological, immunohistochemical methods, biochemical methods, cytometric analysis of markers identifying EPC were used in the study. The CD31⁺ endothelial cells in vitro evaluation was produced by Flow Cytometry and Image Processing of each well with a Cytation™ 3. GLP-1 reduces the area of emphysema and increases the number of CD31⁺ endothelial cells in the lungs of mice in conditions of dyslipidemia and damage to alveolar tissue of cigarette smoke extract. The regenerative effects of GLP-1 are caused by a decrease in inflammation, a positive effect on lipid metabolism and glucose metabolism. EPC are proposed as pathogenetic and diagnostic markers of endothelial disorders in combination of MS with COPD. Based on GLP-1, it is proposed to create a drug to stimulate the regeneration of endothelium damaged in MS and COPD.


Asunto(s)
Péptido 1 Similar al Glucagón/genética , Síndrome Metabólico/genética , Enfermedad Pulmonar Obstructiva Crónica/genética , Enfisema Pulmonar/genética , Animales , Fumar Cigarrillos/efectos adversos , Modelos Animales de Enfermedad , Progresión de la Enfermedad , Células Progenitoras Endoteliales/metabolismo , Citometría de Flujo , Glucosa/metabolismo , Humanos , Metabolismo de los Lípidos/genética , Pulmón/efectos de los fármacos , Pulmón/patología , Síndrome Metabólico/inducido químicamente , Síndrome Metabólico/diagnóstico , Síndrome Metabólico/tratamiento farmacológico , Ratones , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/genética , Enfermedad Pulmonar Obstructiva Crónica/inducido químicamente , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/tratamiento farmacológico , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/tratamiento farmacológico , Enfisema Pulmonar/patología , Glutamato de Sodio/toxicidad
10.
Respir Res ; 20(1): 49, 2019 Mar 04.
Artículo en Inglés | MEDLINE | ID: mdl-30832670

RESUMEN

Small airways disease (SAD) is a cardinal feature of chronic obstructive pulmonary disease (COPD) first recognized in the nineteenth century. The diverse histopathological features associated with SAD underpin the heterogeneous nature of COPD. Our understanding of the key molecular mechanisms which drive the pathological changes are not complete. In this article we will provide a historical overview of key histopathological studies which have helped shape our understanding of SAD and discuss the hallmark features of airway remodelling, mucous plugging and inflammation. We focus on the relationship between SAD and emphysema, SAD in the early stages of COPD, and the mechanisms which cause SAD progression, including bacterial colonization and exacerbations. We discuss the need to specifically target SAD to attenuate the progression of COPD.


Asunto(s)
Remodelación de las Vías Aéreas (Respiratorias)/fisiología , Progresión de la Enfermedad , Enfermedad Pulmonar Obstructiva Crónica/patología , Enfisema Pulmonar/patología , Animales , Predicción , Humanos , Inflamación/inducido químicamente , Inflamación/inmunología , Inflamación/patología , Pulmón/inmunología , Pulmón/patología , Moco/efectos de los fármacos , Moco/inmunología , Enfermedad Pulmonar Obstructiva Crónica/inducido químicamente , Enfermedad Pulmonar Obstructiva Crónica/inmunología , Enfisema Pulmonar/inducido químicamente , Enfisema Pulmonar/inmunología , Fumar/efectos adversos
11.
Ann Diagn Pathol ; 39: 78-85, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30798075

RESUMEN

Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.


Asunto(s)
Procedimientos Quirúrgicos Pulmonares/métodos , Anomalías del Sistema Respiratorio/diagnóstico , Anomalías del Sistema Respiratorio/cirugía , Adolescente , Quiste Broncogénico/diagnóstico , Quiste Broncogénico/patología , Quiste Broncogénico/cirugía , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/patología , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Estudios Transversales , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Variaciones Dependientes del Observador , Enfisema Pulmonar/congénito , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/patología , Enfisema Pulmonar/cirugía , Anomalías del Sistema Respiratorio/patología , Estudios Retrospectivos , Centros de Atención Terciaria
12.
Medicine (Baltimore) ; 98(7): e14438, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30762753

RESUMEN

A new method of quantitative computed tomography (CT) measurements of pulmonary vessels are applicable to morphological studies and may be helpful in defining the progression of emphysema in smokers. However, limited data are available on the relationship between the smoking status and pulmonary vessels alteration established in longitudinal observations. Therefore, we investigated the change of pulmonary vessels on CTs in a longitudinal cohort of smokers.Chest CTs were available for 287 current smokers, 439 non-smokers, and 80 former smokers who quit smoking at least 2 years after the baseline CT. CT images obtained at the baseline and 1 year later were assessed by a new quantitative CT measurement method, computing the total number of pulmonary vessels (TNV), mean lung density (MLD), and the percentage of low-attenuation areas at a threshold of -950 (density attenuation area [LAA]%950). Analysis of variance (ANOVA) and the independent sample t test were used to estimate the influence of the baseline parameters. The t paired test was employed to evaluate the change between the baseline and follow-up results.The current smokers related to have higher whole-lung MLD, as well as less and lower TNV values than the non-smokers (P <.05). But no significant differences in LAA%950 were found between smokers and non-smokers. After one year, the increase in LAA%950 was more rapid in the current (additional 0.3% per year, P <. 05-.01) than in the former smokers (additional 0.2% per year, P = .3). Additionally, the decline in TNV was faster in the current (additional -1.3 per year, P <.05-.01) than that in the former smokers (additional -0.2 per year, P = .6). Current smoke, pack-years, weight, and lung volume independently predicted TNV at baseline (P <.001) in multivariate analysis.The findings of this study reveal that the decline in the pulmonary vessels in smokers can be measured and related to their smoking status.


Asunto(s)
Pulmón/diagnóstico por imagen , Enfisema Pulmonar/diagnóstico por imagen , Venas Pulmonares/diagnóstico por imagen , Fumar/patología , Tomografía Computarizada por Rayos X/estadística & datos numéricos , Adulto , Anciano , Progresión de la Enfermedad , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Pulmón/irrigación sanguínea , Mediciones del Volumen Pulmonar , Masculino , Persona de Mediana Edad , Enfisema Pulmonar/etiología , Enfisema Pulmonar/patología , Venas Pulmonares/patología , Fumar/efectos adversos , Cese del Hábito de Fumar , Factores de Tiempo , Tomografía Computarizada por Rayos X/métodos
13.
Histopathology ; 74(7): 1103-1108, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30715748

RESUMEN

AIMS: Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome that results from tobacco smoking. Emphysema and fibrosis in CPFE patients have been considered to exist separately, with emphysema in the upper lobes and interstitial pneumonia in the lower lobes. The aim of this study was to examine the intrapulmonary distribution of fibrosis and emphysema in clinically diagnosed patients with idiopathic pulmonary fibrosis (IPF) and coexisting emphysema. METHODS AND RESULTS: Among IPF patients (n = 40) who had been autopsied or pneumonectomised for lung transplantation from 1993 to 2018, we retrospectively selected patients with IPF and coexisting emphysema (n = 19) on the basis of the appearance on chest computed tomography (IPF patients with emphysema). We then histologically determined the intrapulmonary distribution of emphysema and fibrosis in the upper lobes and the lower lobes separately. In 15 of the 19 IPF patients with emphysema (79%), fibrosis and emphysema coexisted in the upper lobes and the lower lobes. No patients showed emphysema exclusively in the upper lobes and fibrosis exclusively in the lower lobes. CONCLUSIONS: In the autopsied and pneumonectomised specimens of IPF patients with emphysema, craniocaudal separation of emphysema and fibrosis (emphysema in the upper lobes and interstitial pneumonia in the lower lobes) was histologically rare; coexistence or collision of fibrosis and emphysema in each lobe was common.


Asunto(s)
Fibrosis Pulmonar Idiopática/patología , Enfisema Pulmonar/patología , Fumar Tabaco/patología , Autopsia , Humanos , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Pulmón/diagnóstico por imagen , Pulmón/patología , Trasplante de Pulmón , Registros Médicos , Neumonectomía , Enfisema Pulmonar/complicaciones , Enfisema Pulmonar/diagnóstico por imagen , Estudios Retrospectivos , Fumar Tabaco/efectos adversos , Tomografía Computarizada por Rayos X
14.
Biomarkers ; 24(3): 232-239, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-30411980

RESUMEN

Background: The aetiology and inflammatory profile of combined pulmonary fibrosis and emphysema (CPFE) remain uncertain currently. Objective: We aimed to examine the levels of inflammatory proteins in lung tissue in a cohort of patients with emphysema, interstitial pulmonary fibrosis (IPF), and CPFE. Materials and methods: Explanted lungs were obtained from subjects with emphysema, IPF, CPFE, (or normal subjects), and tissue extracts were prepared. Thirty-four inflammatory proteins were measured in each tissue section. Results: The levels of all 34 proteins were virtually indistinguishable in IPF compared with CPFE tissues, and collectively, the inflammatory profile in the emphysematous tissues were distinct from IPF and CPFE. Moreover, inflammatory protein levels were independent of the severity of the level of diseased tissue. Conclusions: We find that emphysematous lung tissues have a distinct inflammatory profile compared with either IPF or CPFE. However, the inflammatory profile in CPFE lungs is essentially identical to lungs from patients with IPF. These data suggest that distinct inflammatory processes collectively contribute to the disease processes in patients with emphysema, when compared to IPF and CPFE.


Asunto(s)
Inflamación/genética , Proteínas/genética , Enfisema Pulmonar/genética , Fibrosis Pulmonar/genética , Anciano , Humanos , Inflamación/complicaciones , Inflamación/diagnóstico por imagen , Inflamación/patología , Pulmón/metabolismo , Pulmón/patología , Masculino , Persona de Mediana Edad , Mucina 5B/genética , Polimorfismo de Nucleótido Simple , Enfisema Pulmonar/complicaciones , Enfisema Pulmonar/diagnóstico por imagen , Enfisema Pulmonar/patología , Fibrosis Pulmonar/complicaciones , Fibrosis Pulmonar/diagnóstico por imagen , Fibrosis Pulmonar/patología , Tomografía Computarizada por Rayos X
15.
Clin Imaging ; 53: 115-119, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30340073

RESUMEN

OBJECTIVES: The aim of this study was to investigate the extent of dose reduction and comparability of standard dose CT vs Ultra low dose CT in evaluating pulmonary emphysema. METHODS: Forty-nine patients with emphysema were recruited from a tertiary referral respiratory clinic. Each patient had a non-contrast Standard Dose (SD) and Ultra Low Dose (ULD) thoracic CT. The images were reconstructed using contemporary iterative reconstruction with a standard lung kernel. Lung volumes and emphysema severity was calculated using a commercially available automated densitometry segmentation package. The effective dose was calculated for both CT protocols. RESULTS: Automated densitometry calculated the total lung volume and percentage lung area of emphysema. The findings were highly comparable between ULD and SD protocols. A strong correlation was seen between ULD and SD images in measurement of total lung volume (R = 0.925, p < 0.001) and percentage lung involvement by densitometry (R = 0.940, p < 0.001). There is a 95% dose reduction with the ULD protocol, the mean effective dose is 0.12 ±â€¯0.09 mSv versus 2.33 ±â€¯1.54 mSv for the SD protocol. CONCLUSIONS: ULD thoracic CT is a comparable protocol for the assessment of emphysema severity relative to standard dose CT. ULD CT is performed at a 95% dose reduction compared to SD CT.


Asunto(s)
Pulmón/diagnóstico por imagen , Enfisema Pulmonar/diagnóstico por imagen , Dosis de Radiación , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos X/métodos , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Pulmón/patología , Masculino , Persona de Mediana Edad , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/patología , Tórax/diagnóstico por imagen , Tórax/patología
16.
Chest ; 155(2): 266-271, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30080996

RESUMEN

Developing an effective treatment for COPD, and especially pulmonary emphysema, will require an understanding of how fundamental changes at the molecular level affect the macroscopic structure of the lung. Currently, there is no accepted model that encompasses the biochemical and mechanical processes responsible for pulmonary airspace enlargement. We propose that pulmonary emphysematous changes may be more accurately described as an emergent phenomenon, involving alterations at the molecular level that eventually reach a critical structural threshold where uneven mechanical forces produce alveolar wall rupture, accompanied by advanced clinical signs of COPD. The coupling of emergent morphologic changes with biomarkers to detect the process, and counteract it therapeutically, represents a practical approach to the disease.


Asunto(s)
Broncodilatadores/uso terapéutico , Alveolos Pulmonares/parasitología , Enfermedad Pulmonar Obstructiva Crónica/patología , Enfisema Pulmonar/tratamiento farmacológico , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Alveolos Pulmonares/ultraestructura , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfermedad Pulmonar Obstructiva Crónica/terapia , Enfisema Pulmonar/patología , Enfisema Pulmonar/fisiopatología , Medición de Riesgo , Resultado del Tratamiento
17.
Eur Radiol ; 29(1): 176-185, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-29959456

RESUMEN

OBJECTIVES: To investigate whether morphometric complexity in the lung can predict survival and act as a new prognostic marker in patients with chronic obstructive pulmonary disease (COPD). METHODS: COPD (n = 302) patients were retrospectively reviewed. All patients underwent volumetric computed tomography and pulmonary function tests at enrollment (2005-2015). For complexity analysis, we applied power law exponent of the emphysema size distribution (Dsize) as well as box-counting fractal dimension (Dbox3D) analysis. Patients' survival at February 2017 was ascertained. Univariate and multivariate Cox proportional hazards analyses were performed, and prediction performances of various combinatorial models were compared. RESULTS: Patients were 66 ± 6 years old, had 41 ± 28 pack-years' smoking history and variable GOLD stages (n = 20, 153, 108 and 21 in stages I-IV). The median follow-up time was 6.1 years (range: 0.2-11.6 years). Sixty-three patients (20.9%) died, of whom 35 died of lung-related causes. In univariate Cox analysis, lower Dsize and Dbox3D were significantly associated with both all-cause and lung-related mortality (both p < 0.001). In multivariate analysis, the backward elimination method demonstrated that Dbox3D, along with age and the BODE index, was an independent predictor of survival (p = 0.014; HR, 2.08; 95% CI, 1.16-3.71). The contributions of Dsize and Dbox3D to the combinatorial survival model were comparable with those of the emphysema index and lung-diffusing capacity. CONCLUSIONS: Low morphometric complexity in the lung is a predictor of survival in patients with COPD. KEY POINTS: • A newly suggested method for quantifying lung morphometric complexity is feasible. • Morphometric complexity measured on chest CT images predicts COPD patients' survival. • Complexity, diffusing capacity and emphysema index contribute similarly to the survival model.


Asunto(s)
Enfermedad Pulmonar Obstructiva Crónica/complicaciones , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico por imagen , Enfisema Pulmonar/diagnóstico por imagen , Enfisema Pulmonar/etiología , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Fractales , Humanos , Estimación de Kaplan-Meier , Pulmón/patología , Masculino , Persona de Mediana Edad , Análisis Multivariante , Pronóstico , Capacidad de Difusión Pulmonar/fisiología , Enfermedad Pulmonar Obstructiva Crónica/patología , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfisema Pulmonar/patología , Enfisema Pulmonar/fisiopatología , Pruebas de Función Respiratoria , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos X/métodos
18.
Early Hum Dev ; 128: 77-80, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30583279

RESUMEN

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM), especially type-III, shares similar sonographic features with congenital lobar emphysema (CLE) in routine ultrasound scan. Thus, prenatal differentiation of CLE from a microcystic CCAM is challenging and difficult in practice. Discovery of molecular biomarkers has important clinical significance. METHODS: We profiled gene expression in lung tissue from four CCAM type-III and five CLE subjects by microarray. A bioinformatic tool was used for signal pathways enrichment analysis. Further, quantitative reverse transcriptase PCR (qRT-PCR) was used to verify the results. RESULTS: A total of 426 genes were identified to be significantly differentially expressed (fold-change >2.0, q value <0.05) between microcystic CCAM and CLE. Of these differentially expressed genes (DEGs), 392 were upregulated and 34 were downregulated in microcystic CCAM compared with CLE. Unsupervised clustering of the "expressed" genes could clearly delineate the CCAM and CLE samples. We also confirmed that eight randomly chose genes were differentially expressed at the mRNA level between CCAM and CLE. CONCLUSIONS: CCAM type-III and CLE have differential gene expression patterns. Our pilot study may gain a deeper understanding of the organogenetic origins and pathogenesis of these conditions. The suggestive candidates may serve as potential biomarkers for definitive diagnosis of congenital cystic lung lesions and eventually to treat them appropriately.


Asunto(s)
Malformación Adenomatoide Quística Congénita del Pulmón/genética , Enfisema Pulmonar/congénito , Transcriptoma , Biomarcadores/metabolismo , Malformación Adenomatoide Quística Congénita del Pulmón/metabolismo , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Masculino , Enfisema Pulmonar/genética , Enfisema Pulmonar/metabolismo , Enfisema Pulmonar/patología
20.
Proc Natl Acad Sci U S A ; 115(51): 13057-13062, 2018 12 18.
Artículo en Inglés | MEDLINE | ID: mdl-30510003

RESUMEN

Chronic obstructive pulmonary disease (COPD) is a leading cause of morbidity and mortality worldwide. It has generally been considered a non-Th2-type lung disorder, characterized by progressive airflow limitation with inflammation and emphysema, but its cellular and molecular mechanism remains ill defined, compared with that of asthma characterized by reversible airway obstruction. Here we show a previously unappreciated role for basophils at the initiation phase of emphysema formation in an elastase-induced murine model of COPD in that basophils represent less than 1% of lung-infiltrating cells. Intranasal elastase instillation elicited the recruitment of monocytes to the lung, followed by differentiation into interstitial macrophages (IMs) but rarely alveolar macrophages (AMs). Matrix metalloproteinase-12 (MMP-12) contributing to emphysema formation was highly expressed by IMs rather than AMs, in contrast to the prevailing assumption. Experiments using a series of genetically engineered mice suggested that basophil-derived IL-4, a Th2 cytokine, acted on lung-infiltrating monocytes to promote their differentiation into MMP-12-producing IMs that resulted in the destruction of alveolar walls and led to emphysema development. Indeed, mice deficient for IL-4 only in basophils failed to generate pathogenic MMP-12-producing IMs and hence develop emphysema. Thus, the basophil-derived IL-4/monocyte-derived IM/MMP-12 axis plays a crucial role in emphysema formation and therefore may be a potential target to slow down emphysema progression at the initiation phase of COPD.


Asunto(s)
Basófilos/patología , Interleucina-4/metabolismo , Macrófagos Alveolares/patología , Macrófagos/patología , Metaloproteinasa 12 de la Matriz/fisiología , Enfermedad Pulmonar Obstructiva Crónica/fisiopatología , Enfisema Pulmonar/etiología , Animales , Basófilos/metabolismo , Células Cultivadas , Citocinas/metabolismo , Modelos Animales de Enfermedad , Femenino , Macrófagos/metabolismo , Macrófagos Alveolares/metabolismo , Ratones , Ratones Endogámicos BALB C , Ratones Endogámicos C57BL , Neutrófilos , Enfisema Pulmonar/metabolismo , Enfisema Pulmonar/patología
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