RESUMO
Limited results about treatment with total lymphoid irradiation (TLI) in lung transplant (LTx) recipients suffering from progressive bronchiolitis obliterans syndrome (BOS) have been reported. We performed a retrospective analysis of all LTx recipients undergoing TLI for progressive BOS in our center, focusing on long-term outcomes regarding overall survival and lung allograft function. Treatment with TLI (2004-2017, n = 20, 1 BOS stage 1, 6 BOS stage 2, and 13 BOS stage 3) resulted in significant attenuation of the FEV1 -decline in the majority of patients, mainly in those with a rapid decline (P = 0.0005). This allowed bridging to redo-transplantation in five patients. However, three patients progressed from BOS to RAS following prior TLI. Overall patient survival was 44% at 2 years post-TLI and 38% after 17 years. Generally, TLI was well tolerated, with limited side effects and no serious adverse events. TLI may attenuate the decline in FEV1 of LTx recipients with rapid progressive BOS and could thus help to bridge selected patients to redo-transplantation.
Assuntos
Bronquiolite Obliterante , Transplante de Pulmão , Irradiação Linfática , Bronquiolite Obliterante/etiologia , Bronquiolite Obliterante/terapia , Volume Expiratório Forçado , Humanos , Transplante de Pulmão/efeitos adversos , Estudos RetrospectivosAssuntos
Infecções por Coronavirus/epidemiologia , Neoplasias/terapia , Pneumonia Viral/epidemiologia , Bélgica , COVID-19 , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Carcinoma Pulmonar de Células não Pequenas/radioterapia , Carcinoma Pulmonar de Células não Pequenas/cirurgia , Suscetibilidade a Doenças , França , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Mesotelioma/tratamento farmacológico , Mesotelioma Maligno , Neoplasias/tratamento farmacológico , Neoplasias/radioterapia , Neoplasias/cirurgia , Neoplasias Epiteliais e Glandulares/tratamento farmacológico , Pandemias , Guias de Prática Clínica como Assunto , Prognóstico , Carcinoma de Pequenas Células do Pulmão/tratamento farmacológico , Carcinoma de Pequenas Células do Pulmão/radioterapia , Sociedades Médicas , Telemedicina , Neoplasias do Timo/tratamento farmacológico , Reino Unido , Comunicação por VideoconferênciaRESUMO
BACKGROUND: Solid organ transplantation is a valid treatment option for selected patients with organ failure due to an underlying telomeropathy; however, the feasibility of multiple-organ transplantation if several organs are compromised is unclear. METHODS: We describe 2 patients with telomeropathy due to heterozygous telomerase RNA component or telomerase reverse transcriptase mutation, who successfully underwent serial or combined liver and lung transplantation for concurrent liver fibrosis/cirrhosis and pulmonary fibrosis. RESULTS: Despite a challenging posttransplant course, long-term outcomes were favorable, with both patients doing fine now, respectively, 12/20 and 24 months after multiple-organ transplantation. CONCLUSIONS: To our knowledge, this is the first report of multiple solid organ transplantation in documented telomeropathy. These cases highlight current difficulties of timely diagnosis, therapeutic approach, and postoperative complications in telomeropathy patients in whom several organs are affected.
Assuntos
Insuficiência de Múltiplos Órgãos/cirurgia , Transplante de Órgãos/métodos , Homeostase do Telômero/genética , Adulto , Humanos , Fígado/diagnóstico por imagem , Cirrose Hepática/sangue , Cirrose Hepática/diagnóstico , Cirrose Hepática/genética , Cirrose Hepática/cirurgia , Testes de Função Hepática , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/sangue , Insuficiência de Múltiplos Órgãos/genética , Transplante de Órgãos/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fibrose Pulmonar/sangue , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/genética , Fibrose Pulmonar/cirurgia , RNA/genética , Testes de Função Respiratória , Telomerase/genética , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
While organ hypoperfusion caused by inadequate resuscitation has become rare in clinical practice due to the better understanding of burn shock pathophysiology, there is growing concern that increased morbidity and mortality related to over-resuscitation induced by late 20th century resuscitation strategies based on urine output, is occurring more frequently in burn care. In order to reduce complications related to this concept of "fluid creep", such as respiratory failure and compartment syndromes, efforts should be made to resuscitate with the least amount of fluid to provide adequate organ perfusion. In this second part of a concise review, the different targets and endpoints used to guide fluid resuscitation are discussed. Special reference is made to the role of intra-abdominal hypertension in burn care and adjunctive treatments modulating the inflammatory response. Finally, as urine output has been recognized as a poor resuscitation target, a new personalized stepwise resuscitation protocol is suggested which includes targets and endpoints that can be obtained with modern, less invasive hemodynamic monitoring devices like transpulmonary thermodilution.