Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 38
Filtrar
1.
Eur Respir J ; 63(5)2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38575158

RESUMEN

BACKGROUND: Several rare surfactant-related gene (SRG) variants associated with interstitial lung disease are suspected to be associated with lung cancer, but data are missing. We aimed to study the epidemiology and phenotype of lung cancer in an international cohort of SRG variant carriers. METHODS: We conducted a cross-sectional study of all adults with SRG variants in the OrphaLung network and compared lung cancer risk with telomere-related gene (TRG) variant carriers. RESULTS: We identified 99 SRG adult variant carriers (SFTPA1 (n=18), SFTPA2 (n=31), SFTPC (n=24), ABCA3 (n=14) and NKX2-1 (n=12)), including 20 (20.2%) with lung cancer (SFTPA1 (n=7), SFTPA2 (n=8), SFTPC (n=3), NKX2-1 (n=2) and ABCA3 (n=0)). Among SRG variant carriers, the odds of lung cancer was associated with age (OR 1.04, 95% CI 1.01-1.08), smoking (OR 20.7, 95% CI 6.60-76.2) and SFTPA1/SFTPA2 variants (OR 3.97, 95% CI 1.39-13.2). Adenocarcinoma was the only histological type reported, with programmed death ligand-1 expression ≥1% in tumour cells in three samples. Cancer staging was localised (I/II) in eight (40%) individuals, locally advanced (III) in two (10%) and metastatic (IV) in 10 (50%). We found no somatic variant eligible for targeted therapy. Seven cancers were surgically removed, 10 received systemic therapy, and three received the best supportive care according to their stage and performance status. The median overall survival was 24 months, with stage I/II cancers showing better survival. We identified 233 TRG variant carriers. The comparative risk (subdistribution hazard ratio) for lung cancer in SRG patients versus TRG patients was 18.1 (95% CI 7.1-44.7). CONCLUSIONS: The high risk of lung cancer among SRG variant carriers suggests specific screening and diagnostic and therapeutic challenges. The benefit of regular computed tomography scan follow-up should be evaluated.


Asunto(s)
Neoplasias Pulmonares , Proteína A Asociada a Surfactante Pulmonar , Proteína C Asociada a Surfactante Pulmonar , Humanos , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patología , Masculino , Femenino , Persona de Mediana Edad , Anciano , Estudios Transversales , Proteína C Asociada a Surfactante Pulmonar/genética , Proteína A Asociada a Surfactante Pulmonar/genética , Adulto , Factor Nuclear Tiroideo 1/genética , Transportadoras de Casetes de Unión a ATP/genética , Factores de Riesgo , Predisposición Genética a la Enfermedad , Enfermedades Pulmonares Intersticiales/genética , Heterocigoto , Proteínas Asociadas a Surfactante Pulmonar/genética
2.
Respirology ; 29(1): 71-79, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37789612

RESUMEN

BACKGROUND: Antifibrotic agents (AFAs) are now standard-of-care for idiopathic pulmonary fibrosis (IPF). Concerns have arisen about the safety of these drugs in patients undergoing lung transplantation (LTx). METHODS: We performed a multi-centre, nationwide, retrospective, observational study of French IPF patients undergoing LTx between 2011 and 2018 to determine whether maintaining AFAs in the peri-operative period leads to increased bronchial anastomoses issues, delay in skin healing and haemorrhagic complications. We compared the incidence of post-operative complications and the survival of patients according to AFA exposure. RESULTS: Among 205 patients who underwent LTx for IPF during the study period, 58 (28%) had received AFAs within 4 weeks before LTx (AFA group): pirfenidone in 37 (18.0%) and nintedanib in 21 (10.2%). The median duration of AFA treatment before LTx was 13.8 (5.6-24) months. The AFA and control groups did not significantly differ in airway, bleeding or skin healing complications (p = 0.91, p = 0.12 and p = 0.70, respectively). Primary graft dysfunction was less frequent in the AFA than control group (26% vs. 43%, p = 0.02), and the 90-day mortality was lower (7% vs. 18%, p = 0.046). CONCLUSIONS: AFA therapy did not increase airway, bleeding or wound post-operative complications after LTx and could be associated with reduced rates of primary graft dysfunction and 90-day mortality.


Asunto(s)
Fibrosis Pulmonar Idiopática , Trasplante de Pulmón , Disfunción Primaria del Injerto , Humanos , Antifibróticos , Estudios Retrospectivos , Disfunción Primaria del Injerto/tratamiento farmacológico , Disfunción Primaria del Injerto/etiología , Fibrosis Pulmonar Idiopática/tratamiento farmacológico , Fibrosis Pulmonar Idiopática/cirugía , Trasplante de Pulmón/efectos adversos , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Piridonas/efectos adversos , Resultado del Tratamiento
3.
Am J Transplant ; 24(1): 89-103, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37625646

RESUMEN

The acute rejection score (A-score) in lung transplant recipients, calculated as the average of acute cellular rejection A-grades across transbronchial biopsies, summarizes the cumulative burden of rejection over time. We assessed the association between A-score and transplant outcomes in 2 geographically distinct cohorts. The primary cohort included 772 double lung transplant recipients. The analysis was repeated in 300 patients from an independent comparison cohort. Time-dependent multivariable Cox models were constructed to evaluate the association between A-score and chronic lung allograft dysfunction or graft failure. Landmark analyses were performed with A-score calculated at 6 and 12 months posttransplant. In the primary cohort, no association was found between A-score and graft outcome. However, in the comparison cohort, time-dependent A-score was associated with chronic lung allograft dysfunction both as a time-dependent variable (hazard ratio, 1.51; P < .01) and when calculated at 6 months posttransplant (hazard ratio, 1.355; P = .031). The A-score can be a useful predictor of lung transplant outcomes in some settings but is not generalizable across all centers; its utility as a prognostication tool is therefore limited.


Asunto(s)
Trasplante de Pulmón , Humanos , Pronóstico , Estudios Retrospectivos , Trasplante de Pulmón/efectos adversos , Pulmón , Modelos de Riesgos Proporcionales , Rechazo de Injerto/diagnóstico , Rechazo de Injerto/etiología
4.
Ann Biol Clin (Paris) ; 81(4): 403-409, 2023 10 20.
Artículo en Inglés | MEDLINE | ID: mdl-37864446

RESUMEN

OBJECTIVES: SARS-CoV-2 has impacted the detection of seasonal respiratory viruses. We retrospectively assessed the trend in the detection of 10 viruses in the COVID-19 area in 2 hospitals located in the Paris area. METHODS: All patients positive for a respiratory virus in two hospitals from September 2016 to August 2021 were retrospectively included. The rate of RT-PCR positive for each virus was calculated for the 2020-2021 season and the 2019-2020 season in comparison to a baseline of 3 seasons, i.e. 2016-2017, 2017-2018, and 2018-2019. RESULTS: Overall, 7,835 patients were tested positive from September 2016 to August 2021. The detection of respiratory virus dramatically falls on week-11 of 2020, as the number of RT-PCR performed. Then, 3 trends were identified: a) almost a disappearance for influenza; b) a 10-weeks delay in the seasonal outbreak for RSV; c) a persistence of circulation with variable activity for other viruses. In comparison to a baseline of three seasons (2016-2019), the rate of positive patients was lower during the 2020-2021 season for coronavirus (4.51% vs. 1.26%, P < 0.0001), adenovirus (1.93% vs. 1.34%, P = 0.14), bocavirus (0.58% vs. 0.11%, P = 0.08), and enterovirus (0.28% vs. 0.0%, P = 0.12). In contrast, the rate of hMPV-positive (1.92% vs. 2.83%, P = 0.03) and hPIV-positive (2.17% vs. 2.99%, P = 0.06) patients increased. CONCLUSIONS: The fall in the number of respiratory viruses detected might be related to the lower number of tests performed and the implementation of non pharmaceutical intervention (NPI). Then, all viruses except influenza are detected, probably as a consequence of high adherence to influenza vaccines. Despite, a lower number of tests being performed, the rate of hMPV-positive and hPIV-positive patients increased suggesting an active circulation of these viruses. Altogether, these findings suggest a persistent circulation of common respiratory viruses all over the COVID-19 era.


Asunto(s)
COVID-19 , Gripe Humana , Infecciones del Sistema Respiratorio , Virus , Humanos , Lactante , Gripe Humana/epidemiología , Estudios Retrospectivos , COVID-19/diagnóstico , COVID-19/epidemiología , SARS-CoV-2 , Infecciones del Sistema Respiratorio/diagnóstico , Infecciones del Sistema Respiratorio/epidemiología
5.
Transpl Int ; 36: 11180, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37404718

RESUMEN

Lung transplantation is limited by the shortage of suitable donors. Many programs have begun to use extended criteria donors. Donors over 65 years old are rarely reported, especially for young cystic fibrosis recipients. This monocentric study was conducted for cystic fibrosis recipients from January 2005 to December 2019, comparing two cohorts according to lung donor age (<65 years or ≥65 years). The primary objective was to assess the survival rate at 3 years using a Cox multivariable model. Of the 356 lung recipients, 326 had donors under 65 years, and 30 had donors over 65 years. Donors' characteristics did not differ significantly in terms of sex, time on mechanical ventilation before retrieval, and partial pressure of arterial oxygen/fraction of inspired oxygen ratio. There were no significant differences in post-operative mechanical ventilation duration and incidence of grade 3 primary graft dysfunction between the two groups. At 1, 3, and 5 years, the percentage of predicted forced expiratory volume in 1 s (p = 0.767) and survival rate did not differ between groups (p = 0.924). The use of lungs from donors over 65 years for cystic fibrosis recipients allows extension of the donor pool without compromising results. Longer follow-up is needed to assess the long-term effects of this practice.


Asunto(s)
Fibrosis Quística , Trasplante de Pulmón , Obtención de Tejidos y Órganos , Humanos , Anciano , Fibrosis Quística/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Donantes de Tejidos , Trasplante de Pulmón/métodos , Pulmón , Oxígeno
6.
PLoS One ; 18(3): e0281492, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36920935

RESUMEN

BACKGROUND: Calcineurin inhibitors (CNIs) remain the cornerstone of maintenance immunosuppression (IS) after lung transplantation (LTx), although CNI-related life-threatening toxic effects may occur. Belatacept, a novel immunosuppressant that blocks a T-cell co-stimulation pathway, is a non-nephrotoxic drug indicated as an alternative to CNIs in kidney Tx. In LTx, there are only a few reports of belatacept conversion as a CNI-free or CNI-sparing IS treatment. METHODS: We reviewed a series of 10 LTx recipients with conversion to a CNI-free belatacept IS regimen within the first year post-LTx (n = 7) or a belatacept/low-dose CNI combination after the first year (n = 3). RESULTS: Use of belatacept was triggered by severe renal failure in 9 patients and under-IS with previous other IS-related toxicities in 1 patient. Mean estimated glomerular filtration rate after starting belatacept significantly improved at 6 months after initiation and at the last-follow-up (p = 0.006, and p = 0.002 respectively). The incidence of recurrent and/or severe acute cellular rejection (ACR) episodes was high in patients with CNI-free belatacept-based IS (n = 4/7). Chronic graft allograft dysfunction developed in 2 of 9 recipients under belatacept IS. Belatacept was stopped in 6 patients because of recurrent/severe ACR (n = 3), recurrent opportunistic infections (n = 1), center modified policy (n = 1), or other cause (n = 1). CONCLUSION: Early conversion to CNI-free belatacept-based IS improved renal function in this series but was counterbalanced by a high incidence of recurrent ACR, including life-threatening episodes. Other studies are needed to better determine the indications for its use after LTx, possibly with lower immunological risk IS regimens, such as CNI-sparing belatacept.


Asunto(s)
Trasplante de Riñón , Trasplante de Pulmón , Humanos , Abatacept/uso terapéutico , Abatacept/farmacología , Inhibidores de la Calcineurina/efectos adversos , Rechazo de Injerto/tratamiento farmacológico , Rechazo de Injerto/prevención & control , Supervivencia de Injerto , Inmunosupresores/efectos adversos , Trasplante de Riñón/efectos adversos , Trasplante de Pulmón/efectos adversos
7.
Am J Transplant ; 23(2): 294-297, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36695676

RESUMEN

Lung transplant candidates who are highly sensitized against human leucocyte antigen present an ongoing challenge with regards to finding immunologically acceptable donors. Desensitization strategies aimed at reducing preformed donor-specific antibodies have a number of limitations. Imlifidase, an IgG-degrading enzyme derived from Streptococcus pyogenes, is a novel agent that has been used to convert positive crossmatches to negative in kidney transplant candidates, allowing transplantation to occur. We present the first case of imlifidase use for antibody depletion in a highly sensitized lung transplant candidate who went on to undergo a successful bilateral lung transplant.


Asunto(s)
Trasplante de Riñón , Trasplante de Pulmón , Humanos , Anticuerpos , Inmunosupresores , Trasplante de Riñón/efectos adversos , Donantes de Tejidos , Antígenos HLA , Trasplante de Pulmón/efectos adversos , Prueba de Histocompatibilidad , Desensibilización Inmunológica , Rechazo de Injerto/tratamiento farmacológico , Rechazo de Injerto/etiología
8.
Am J Transplant ; 22(12): 2990-3001, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-35988032

RESUMEN

In patients with interstitial lung disease (ILD) complicating classical or amyopathic idiopathic inflammatory myopathy (IIM), lung transplantation outcomes might be affected by the disease and treatments. Here, our objective was to assess survival and prognostic factors in lung transplant recipients with IIM-ILD. We retrospectively reviewed data for 64 patients who underwent lung transplantation between 2009 and 2021 at 19 European centers. Patient survival was the primary outcome. At transplantation, the median age was 53 [46-59] years, 35 (55%) patients were male, 31 (48%) had classical IIM, 25 (39%) had rapidly progressive ILD, and 21 (33%) were in a high-priority transplant allocation program. Survival rates after 1, 3, and 5 years were 78%, 73%, and 70%, respectively. During follow-up (median, 33 [7-63] months), 23% of patients developed chronic lung allograft dysfunction. Compared to amyopathic IIM, classical IIM was characterized by longer disease duration, higher-intensity immunosuppression before transplantation, and significantly worse posttransplantation survival. Five (8%) patients had a clinical IIM relapse, with mild manifestations. No patient experienced ILD recurrence in the allograft. Posttransplantation survival in IIM-ILD was similar to that in international all-cause-transplantation registries. The main factor associated with worse survival was a history of muscle involvement (classical IIM). In lung transplant recipients with idiopathic inflammatory myopathy, survival was similar to that in all-cause transplantation and was worse in patients with muscle involvement compared to those with the amyopathic disease.


Asunto(s)
Enfermedades Pulmonares Intersticiales , Trasplante de Pulmón , Miositis , Humanos , Masculino , Persona de Mediana Edad , Femenino , Estudios de Cohortes , Estudios Retrospectivos , Miositis/cirugía , Miositis/complicaciones , Enfermedades Pulmonares Intersticiales/cirugía , Enfermedades Pulmonares Intersticiales/etiología , Trasplante de Pulmón/efectos adversos
9.
J Heart Lung Transplant ; 41(9): 1207-1209, 2022 09.
Artículo en Inglés | MEDLINE | ID: mdl-35835678

RESUMEN

Almost 25% of patients with pulmonary fibrosis referred for lung transplantation have a germline rare variant of a telomere-related gene. Acquired TERT promoter mutations may counterbalanced the germline defect and reduce the risk of hematological complications in this population. In a series of 34 patients with a germline telomere-related gene mutation who underwent lung transplantation, 12 (35%) patients had at least 1 acquired TERT promoter mutation. Six patients presented myelodysplasia before lung transplantation, with no difference between patients with and without an acquired TERT promoter mutation. After lung transplantation, myelodysplasia developed in only 1 of 8 patients with an acquired TERT promoter mutation versus 7 of 18 patients without a mutation. Survival did not differ between patients with and without an acquired mutation. The presence of an acquired TERT promoter mutation could be associated with reduced hematological complications after transplantation and with better outcome in telomere-related gene mutation carriers but requires further study.


Asunto(s)
Trasplante de Pulmón , Telomerasa , Heterocigoto , Humanos , Mutación , Telomerasa/genética , Telomerasa/metabolismo , Telómero
10.
Respir Med Res ; 82: 100941, 2022 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-35908527

RESUMEN

INTRODUCTION: Hemoptysis isn't rare in lung transplant recipients (LTR). Yet, trans-arterial embolization (TAE) in LTR has been rarely reported in the literature. The aim of the study was to present the feasibility and outcomes of TAE for hemoptysis in LTR. MATERIALS AND METHODS: Retrospective study of all LTR who underwent TAE for hemoptysis in our single institution between 2005 and 2020. RESULTS: A total of 787 patients underwent lung transplantation between 2005 and 2020. Fifteen LTR underwent 21 TAE for hemoptysis in a median delay of 42 days after LT. TAE was performed within a year after LT in 13 patients (86.7%) with 12 of those patients having concomitant severe ischemic airway injury with necrosis and anastomotic dehiscence. Bronchoscopy confirmed bronchial anastomoses has being the source of the bleeding in 11 LTR (84.6%). Restoration of bronchial vascularization was highlighted in 13 patients (87%). Despite TAE, bronchial anastomosis healing was observed in all surviving patients with anastomotic dehiscence in a median delay of 43 days. CONCLUSION: In our experience, hemoptysis requiring TAE in LTR was rare, frequently occurring in the first weeks after LT, and seemed associated with anastomotic ischemia and dehiscence. Bleeding mainly originated from ischemic bronchial anastomosis through the restoration of the bronchial artery circulation. Our results suggest that bronchial arteriography should be routinely proposed in such patients in the event of hemoptysis.


Asunto(s)
Hemoptisis , Receptores de Trasplantes , Humanos , Hemoptisis/etiología , Hemoptisis/terapia , Estudios Retrospectivos , Resultado del Tratamiento , Pulmón
12.
J Heart Lung Transplant ; 41(5): 626-640, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35181243

RESUMEN

BACKGROUND: Survival after lung transplantation (LTx) still remains limited by chronic lung allograft dysfunction (CLAD), thought to represent a form of chronic rejection. We investigated whether the immune checkpoint HLA-G/ILT2 expressed by peripheral T-cell subpopulations could predict CLAD. METHODS: We used data for 150 LTx recipients from COLT (Cohort-For-Lung-Transplantation) cohort with ≥1 available blood sample at 1-, 6-, or 12-months post-Tx. Analysis of T cells by flow cytometry focused on the ILT2 receptor of HLA-G and other markers (CD57, CD25, CD127). T-cell subset analyses compared stable patients and those with CLAD at 3 years post-LTx. RESULTS: With data for 78 stable and 72 CLAD patients, among 21 T-cell subsets expressing ILT2, only CD4+CD57+ILT2+ T cells were associated with outcome. At 1-month post-Tx, low proportion of CD4+CD57+ILT2+ T cells was associated with reduced 3-year incidence of CLAD (CD4+CD57+ILT2+ T cells ≤ first IQR [25%] vs > first IQR, log-rank test, p = 0.028). Furthermore, the incidence of CLAD was higher with >2.6- vs ≤2.6-fold increased proportion of CD4+CD57+ILT2+ T cells over the first year post-LTx (3-year freedom frequencies: 27% [95%CI: 8-50] vs 64% [95%CI: 48-77] (log-rank test, p = 0.014). On multivariable analysis, increased proportion of CD4+CD57+ILT2+ T cells over the first year predicted CLAD (hazard ratio 1.25; 95%CI: 1.09-1.44; p = 0.001). Focusing on CD4+CD57+ILT2+ T cells, we demonstrated ex vivo that they are cytotoxic CD4+ T cells, selectively inhibited by HLA-G. CONCLUSIONS: Our data suggest that an early increase of CD4+CD57+ILT2+ T cells after LTx may be associated with CLAD onset.


Asunto(s)
Antígenos HLA-G , Trasplante de Pulmón , Aloinjertos , Humanos , Pulmón , Linfocitos T
13.
Eur Respir J ; 58(5)2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34016620

RESUMEN

BACKGROUND: Pre-formed donor-specific antibodies (DSAs) are associated with worse outcome after lung transplantation (LTx) and might limit access to LTx. A virtual crossmatch-based strategy for perioperative desensitisation protocol has been used for immunised LTx candidates since 2012 at Foch Hospital (Suresnes, France). We compared the outcome of desensitised LTx candidates with high DSA mean fluorescence intensity and those with low or no pre-formed DSAs, not desensitised. METHODS: For all consecutive LTx recipients (January 2012 to March 2018), freedom from chronic lung allograft dysfunction (CLAD) and graft survival were assessed using Kaplan-Meier analysis and Cox multivariate analysis. RESULTS: We compared outcomes for desensitised patients with high pre-formed DSAs (n=39) and those with no (n=216) or low pre-formed DSAs (n=66). The desensitisation protocol decreased the level of immunodominant DSA (class I/II) at 1, 3 and 6 months post-LTx (p<0.001, p<0.01 and p<0.001, respectively). Freedom from CLAD and graft survival at 3 years was similar in the desensitised group as a whole and other groups. Nevertheless, incidence of CLAD was higher with persistent high-level DSAs than cleared high-level (p=0.044) or no DSAs (p=0.014). Conversely, graft survival was better with cleared high DSAs than persistent high-level, low-level and no pre-formed DSAs (p=0.019, p=0.025 and p=0.044, respectively). On multivariate analysis, graft survival was associated with cleared high DSAs (hazard ratio 0.12, 95% CI 0.02-0.85 versus no DSAs; p=0.035) and CLAD with persistent DSAs (3.04, 1.02-9.17 versus no pre-formed DSAs; p=0.048). CONCLUSION: The desensitisation protocol in LTx recipients with high pre-formed DSAs was associated with satisfactory outcome, with cleared high pre-formed DSAs after desensitisation identified as an independent predictor of graft survival.


Asunto(s)
Trasplante de Pulmón , Receptores de Trasplantes , Rechazo de Injerto , Supervivencia de Injerto , Antígenos HLA , Humanos , Isoanticuerpos , Pulmón , Estudios Retrospectivos
14.
Diagnostics (Basel) ; 11(5)2021 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-33946544

RESUMEN

Chronic lung allograft rejection remains one of the major causes of morbi-mortality after lung transplantation. The term Chronic Lung Allograft Dysfunction (CLAD) has been proposed to describe the different processes that lead to a significant and persistent deterioration in lung function without identifiable causes. The two main phenotypes of CLAD are Bronchiolitis Obliterans Syndrome (BOS) and Restrictive Allograft Syndrome (RAS), each of them characterized by particular functional and imaging features. These entities can be associated (mixed phenotype) or switched from one to the other. If CLAD remains a clinical diagnosis based on spirometry, computed tomography (CT) scan plays an important role in the diagnosis and follow-up of CLAD patients, to exclude identifiable causes of functional decline when CLAD is first suspected, to detect early abnormalities that can precede the diagnosis of CLAD (particularly RAS), to differentiate between the obstructive and restrictive phenotypes, and to detect exacerbations and evolution from one phenotype to the other. Recognition of early signs of rejection is crucial for better understanding of physiopathologic pathways and optimal management of patients.

15.
Eur Respir J ; 58(2)2021 08.
Artículo en Inglés | MEDLINE | ID: mdl-33479107

RESUMEN

STUDY QUESTION: In patients with sarcoidosis, past and ongoing immunosuppressive regimens, recurrent disease in the transplant and extrapulmonary involvement may affect outcomes of lung transplantation. We asked whether sarcoidosis lung phenotypes can be differentiated and, if so, how they relate to outcomes in patients with pulmonary sarcoidosis treated by lung transplantation. PATIENTS AND METHODS: We retrospectively reviewed data from 112 patients who met international diagnostic criteria for sarcoidosis and underwent lung or heart-lung transplantation between 2006 and 2019 at 16 European centres. RESULTS: Patient survival was the main outcome measure. At transplantation, median (interaquartile range (IQR)) age was 52 (46-59) years; 71 (64%) were male. Lung phenotypes were individualised as follows: 1) extended fibrosis only; 2) airflow obstruction; 3) severe pulmonary hypertension (sPH) and airflow obstruction; 4) sPH, airflow obstruction and fibrosis; 5) sPH and fibrosis; 6) airflow obstruction and fibrosis; 7) sPH; and 8) none of these criteria, in 17%, 16%, 17%, 14%, 11%, 9%, 5% and 11% of patients, respectively. Post-transplant survival rates after 1, 3, and 5 years were 86%, 76% and 69%, respectively. During follow-up (median (IQR) 46 (16-89) months), 31% of patients developed chronic lung allograft dysfunction. Age and extended lung fibrosis were associated with increased mortality. Pulmonary fibrosis predominating peripherally was associated with short-term complications. ANSWER TO THE STUDY QUESTION: Post-transplant survival in patients with pulmonary sarcoidosis was similar to that in patients with other indications for lung transplantation. The main factors associated with worse survival were older age and extensive pre-operative lung fibrosis.


Asunto(s)
Trasplante de Pulmón , Sarcoidosis Pulmonar , Sarcoidosis , Anciano , Humanos , Masculino , Pronóstico , Estudios Retrospectivos , Sarcoidosis/cirugía , Sarcoidosis Pulmonar/cirugía
16.
Eur Respir J ; 56(6)2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32855221

RESUMEN

INTRODUCTION: Interstitial lung diseases (ILDs) can be caused by mutations in the SFTPA1 and SFTPA2 genes, which encode the surfactant protein (SP) complex SP-A. Only 11 SFTPA1 or SFTPA2 mutations have so far been reported worldwide, of which five have been functionally assessed. In the framework of ILD molecular diagnosis, we identified 14 independent patients with pathogenic SFTPA1 or SFTPA2 mutations. The present study aimed to functionally assess the 11 different mutations identified and to accurately describe the disease phenotype of the patients and their affected relatives. METHODS: The consequences of the 11 SFTPA1 or SFTPA2 mutations were analysed both in vitro, by studying the production and secretion of the corresponding mutated proteins and ex vivo, by analysing SP-A expression in lung tissue samples. The associated disease phenotypes were documented. RESULTS: For the 11 identified mutations, protein production was preserved but secretion was abolished. The expression pattern of lung SP-A available in six patients was altered and the family history reported ILD and/or lung adenocarcinoma in 13 out of 14 families (93%). Among the 28 SFTPA1 or SFTPA2 mutation carriers, the mean age at ILD onset was 45 years (range 0.6-65 years) and 48% underwent lung transplantation (mean age 51 years). Seven carriers were asymptomatic. DISCUSSION: This study, which expands the molecular and clinical spectrum of SP-A disorders, shows that pathogenic SFTPA1 or SFTPA2 mutations share similar consequences for SP-A secretion in cell models and in lung tissue immunostaining, whereas they are associated with a highly variable phenotypic expression of disease, ranging from severe forms requiring lung transplantation to incomplete penetrance.


Asunto(s)
Enfermedades Pulmonares Intersticiales , Neoplasias Pulmonares , Adolescente , Adulto , Anciano , Niño , Preescolar , Humanos , Lactante , Enfermedades Pulmonares Intersticiales/genética , Neoplasias Pulmonares/genética , Persona de Mediana Edad , Mutación , Fenotipo , Proteína A Asociada a Surfactante Pulmonar/genética , Adulto Joven
19.
J Heart Lung Transplant ; 39(2): 125-133, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31836403

RESUMEN

BACKGROUND: Restrictive allograft syndrome (RAS) after lung transplantation (LTx) is associated with the poorer graft survival in patients with chronic lung allograft dysfunction (CLAD). Nevertheless, its diagnostic criteria have not been clearly defined after single-LTx (SLTx). Hence, we studied an SLTx cohort with CLAD to investigate the utility of both computed tomography (CT)-score/volume measures and functional spirometric criteria for the early identification of RAS in this population. METHODS: We included 51 patients with SLTx (17 RAS, 17 bronchiolitis obliterans syndrome [BOS], and 17 stable condition). The criteria for RAS diagnosis in SLTx included forced vital capacity (FVC) <80% baseline (BL) or forced expiratory volume in 1 second (FEV1) <80% BL with an FEV1/FVC ratiounchanged or >0.7 and persistent CT-scan-lung opacities. We defined 4 time points (T): T-baseline, T-onset (first CT-scan-opacities), T-follow-up, and T-last. RESULTS: In patients with RAS, the spirometric criteria for RAS at T-onset were reached in only 47% (FVC decline <80% BL [(29%] or FEV1 <80% BL/ratiounchanged or >0.7 [41%]), whereas at the same T-onset date, the graft CT-score increased to 5 (4-6) vs 1 (0-2) at baseline (p < 0.001) (CT - score ≥2 at T-onset in 100% and ΔCT - score ≥2 in 74% of patients with RAS), and the median CT-scan graft volume decreased to 1,722 ml (vs 1,796 ml at T-baseline, p = 0.003) (decreased CT-graft - volume <90% BL in 50% of patients). In contrast, in patients with BOS, CT-score/volume were unchanged at T-onset vs T-baseline (p = 0.8, p = 0.68, respectively). CONCLUSION: Our results suggest that the use of a simple CT-score and to a lesser extent, CT-volume measures, might allow for the early identification and/or prediction of RAS in SLTx rather than functional criteria.


Asunto(s)
Trasplante de Pulmón/efectos adversos , Pulmón/diagnóstico por imagen , Disfunción Primaria del Injerto/diagnóstico , Receptores de Trasplantes , Adulto , Aloinjertos , Bronquiolitis Obliterante/cirugía , Femenino , Estudios de Seguimiento , Volumen Espiratorio Forzado/fisiología , Supervivencia de Injerto , Humanos , Mediciones del Volumen Pulmonar/métodos , Masculino , Persona de Mediana Edad , Disfunción Primaria del Injerto/fisiopatología , Estudios Retrospectivos , Síndrome , Factores de Tiempo , Tomografía Computarizada por Rayos X/métodos
20.
Transpl Infect Dis ; 21(5): e13141, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31283872

RESUMEN

BACKGROUND: Scedosporium species and Lomentospora prolificans (S/L) are the second most common causes of invasive mold infections following Aspergillus in lung transplant recipients. METHODS: We assessed the current practices on management of S/L colonization/infection of the lower respiratory tract before and after lung transplantation in a large number of lung transplant centers through an international practice survey from October 2016 to March 2017. RESULTS: A total of 51 respondents from 45 lung transplant centers (17 countries, 4 continents) answered the survey (response rate 58%). S/L colonization was estimated to be detected in candidates by 48% of centers. Only 18% of the centers used a specific medium to detect S/L colonization. Scedosporium spp. colonization was a contraindication to transplantation in 10% of centers whereas L prolificans was a contraindication in 31%; 22% of centers declared having had 1-5 recipients infected with S/L in the past 5 years. CONCLUSIONS: This survey gives an overview of the current practices regarding S/L colonization and infection in lung transplant centers worldwide and underscores the need of S/L culture procedure standardization before implementing prospective studies.


Asunto(s)
Ascomicetos/aislamiento & purificación , Manejo de la Enfermedad , Huésped Inmunocomprometido , Trasplante de Pulmón/efectos adversos , Micosis/epidemiología , Scedosporium/aislamiento & purificación , Humanos , Internacionalidad , Micosis/etiología , Estudios Prospectivos , Infecciones del Sistema Respiratorio/etiología , Infecciones del Sistema Respiratorio/microbiología , Encuestas y Cuestionarios
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...