Influence of early neurological complications on clinical outcome following lung transplant.

BACKGROUND: Neurological complications after lung transplantation are common. The full spectrum of neurological complications and their impact on clinical outcomes has not been extensively studied. METHODS: We investigated the neurological incidence of complications, categorized according to whether they affected the central, peripheral or autonomic nervous systems, in a series of 109 patients undergoing lung transplantation at our center between January 1 2013 and December 31 2014. RESULTS: Fifty-one patients (46.8%) presented at least one neurological complication. Critical illness polyneuropathy-myopathy (31 cases) and phrenic nerve injury (26 cases) were the two most prevalent complications. These two neuromuscular complications lengthened hospital stays by a median period of 35.5 and 32.5 days respectively. However, neurological complications did not affect patients' survival. CONCLUSIONS: The real incidence of neurological complications among lung transplant recipients is probably underestimated. They usually appear in the first two months after surgery. Despite not affecting mortality, they do affect the mean length of hospital stay, and especially the time spent in the Intensive Care Unit. We found no risk factor for neurological complications except for long operating times, ischemic time and need for transfusion. It is necessary to develop programs for the prevention and early recognition of these complications, and the prevention of their precipitant and risk factors.

Study of the prevalence of familial autoimmune myasthenia gravis in a Spanish cohort.

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease caused by a failure of neuromuscular transmission. Familial clustering has been reported despiteMG usually manifesting as a sporadic condition presumed not to be inherited. Our study investigated the prevalence of FAMG in a Spanish cohort, characterizing their phenotype,antibody titres and thymus findings. MATERIAL/METHODS: We investigated the presence of familial cases in 462 MG patients, characterizing by age and MGFA class at debut, quantitative MG score, antibody titres, MGFA post-intervention status and thymus pathology. RESULTS: Sixteen cases from8 unrelated pedigrees were identified. The prevalence of FAMG caseswas 3.46%.Mean age at onset was 57.8 ± 17.4 years (range=23­82). Distribution at debut was: 6 ocular, 4 IIa, 4IIb, 1 IIIa and 1 IIIb. Thymoma was identified in two of the 7 thymectomized individuals. CONCLUSIONS: The prevalence of FAMG in Spain is similar to other populations. Post-intervention status did not differ from sporadic autoimmune MG. As in other neuromuscular disorders, phenotype and inheritance heterogeneity are present in FAMG. In addition to the interfamilial heterogeneity observed, members of the same family affected with FAMG may even present different ages of onset, severity and thymus involvement. Further studies are necessary to clarify the role of genetic risk factors in this form of autoimmune MG.

Trasplante pulmonar en la fibrosis pulmonar idiopática y otras enfermedades pulmonares intersticiales / Lung transplantation in pulmonary fibrosis and other interstitial lung diseases

Fundamento y objetivo: Las enfermedades pulmonares intersticiales (EPI) constituyen, después del enfisema, la segunda indicación de trasplante pulmonar (TP). El objetivo del presente estudio es la revisión de los resultados del TP en la EPI en el Hospital Vall d’Hebron (Barcelona). Pacientes y método: Se estudian retrospectivamente 150 pacientes, 87 (58%) varones, con una edad media de 48 años (extremos 20-67), en los que se indicó un TP por una EPI entre agosto de 1990 y enero de 2010, y que representan el 30% del total de TP (150/496) realizados en Cataluña. Ciento cuatro (69%) fueron trasplantes unipulmonares (TUP), y 46 (31%), trasplantes bipulmonares (TBP). Los diagnósticos postoperatorios fueron: 94 (63%) neumonía intersticial usual, 23 (15%) neumonía intersticial no específica, 11(7%) neumonía intersticial no clasificable y un 15% miscelánea. Se describen los resultados funcionales, las complicaciones y la supervivencia. Resultados: La supervivencia actuarial fue del 87, 70 y 53% a uno, 3 y 5 años, respectivamente. Las causas de muerte más frecuentes incluyen la disfunción precoz del injerto y el desarrollo de rechazo crónico en forma de bronquiolitis obliterante (BOS). El incremento medio postoperatorio de la capacidad vital forzada y del forced expiratory volume in the first second (FEV1, «volumen espiratorio forzado en el primer segundo») fue similar en el TUP y el TBP. El mejor FEV1 posterior al TP se alcanzó al décimo mes (extremos 1-36). El 16% de los pacientes se reintegró al trabajo. En algún momento de la evolución se diagnosticó rechazo agudo, confirmado histológicamente en 53 (35%) pacientes. La prevalencia de BOS entre los supervivientes fue del 20% al año, 45% a los 3 años y del 63% a los 5 años. Conclusión: El TP es hasta ahora el único tratamiento capaz de alargar la vida de los pacientes con EPI avanzada y en nuestro medio ha permitido que uno de cada 6 pacientes haya vuelto a trabajar. Sin embargo, el rechazo crónico sigue siendo el principal problema a resolver en el futuro (AU)

Background and objective: Interstitial lung disease (ILD) is the second indication for lung transplantation (LT) after emphysema. The aim of this study is to review the results of LT for ILD in Hospital Vall d’Hebron (Barcelona, Spain). Patients and methods: We retrospectively studied 150 patients, 87 (58%) men, mean age 48 (r: 20-67) years between August 1990 and January 2010. One hundred and four (69%) were single lung transplants (SLT) and 46 (31%) bilateral-lung transplants (BLT). The postoperative diagnoses were: 94 (63%) usual interstitial pneumonia, 23 (15%) nonspecific interstitial pneumonia, 11 (7%) unclassifiable interstitial pneumonia and 15% miscellaneous. We describe the functional results, complications and survival. Results: The actuarial survival was 87, 70 and 53% at one, 3 and 5 years respectively. The most frequent causes of death included early graft dysfunction and development of chronic rejection in the form of bronchiolitis obliterans (BOS). The mean postoperative increase in forced vital capacity and forced expiratory volume in the first second (FEV1) was similar in SLT and BLT. The best FEV1 was reached after 10 (r: 1-36) months. Sixteen percent of patients returned to work. At some point during the evolution, proven acute rejection was diagnosed histologically in 53 (35%) patients. The prevalence of BOS among survivors was 20% per year, 45% at 3 years and 63% at 5 years. Conclusions: LT is the best treatment option currently available for ILD, in which medical treatment has failed (AU)

[Lung transplantation in pulmonary fibrosis and other interstitial lung diseases]. / Trasplante pulmonar en la fibrosis pulmonar idiopática y otras enfermedades pulmonares intersticiales.

BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) is the second indication for lung transplantation (LT) after emphysema. The aim of this study is to review the results of LT for ILD in Hospital Vall d'Hebron (Barcelona, Spain). PATIENTS AND METHODS: We retrospectively studied 150 patients, 87 (58%) men, mean age 48 (r: 20-67) years between August 1990 and January 2010. One hundred and four (69%) were single lung transplants (SLT) and 46 (31%) bilateral-lung transplants (BLT). The postoperative diagnoses were: 94 (63%) usual interstitial pneumonia, 23 (15%) nonspecific interstitial pneumonia, 11 (7%) unclassifiable interstitial pneumonia and 15% miscellaneous. We describe the functional results, complications and survival. RESULTS: The actuarial survival was 87, 70 and 53% at one, 3 and 5 years respectively. The most frequent causes of death included early graft dysfunction and development of chronic rejection in the form of bronchiolitis obliterans (BOS). The mean postoperative increase in forced vital capacity and forced expiratory volume in the first second (FEV1) was similar in SLT and BLT. The best FEV1 was reached after 10 (r: 1-36) months. Sixteen percent of patients returned to work. At some point during the evolution, proven acute rejection was diagnosed histologically in 53 (35%) patients. The prevalence of BOS among survivors was 20% per year, 45% at 3 years and 63% at 5 years. CONCLUSIONS: LT is the best treatment option currently available for ILD, in which medical treatment has failed.

Afección traqueobronquial en la granulomatosis de Wegener / Tracheobronchial involvement in Wegener's granulomatosis

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