Experiencia en trasplante pulmonar pediátrico en Chile / Experience with pediatric lung transplantation in Chile

El trasplante de pulmón (TP) es una opción para pacientes pediátricos con enfermedades pulmonares terminales. OBJETIVO: Evaluar resultados y sobrevida de pacientes pediátricos trasplantados de pulmón. MÉTODOS: Análisis retrospectivo de registros clínicos de pacientes TP ≤ 15 años de Clínica Las Condes. Se analizaron datos demográficos, tipo de trasplante, función pulmonar basal y post trasplante, complicaciones precoces y tardías y sobrevida. RESULTADOS: Nueve pacientes < 15 años de edad se han trasplantado. La edad promedio fue 12,7 años. La principal indicación fue fibrosis quística (7 pacientes). El IMC promedio fue de 17,6 y todos estaban con oxígeno domiciliario. El 77% utilizó soporte extracorpóreo intraoperatorio. Las principales complicaciones precoces fueron hemorragia y la disfunción primaria de injerto mientras que las tardías fueron principalmente las infecciones y la disfunción crónica de injerto. Cuatro pacientes han fallecido y la sobrevida a dos años fue de 85%. El trasplante les permitió una reinserción escolar y 3 lograron completar estudios universitarios. CONCLUSIÓN: El trasplante pulmonar es una alternativa para niños con enfermedades pulmonares avanzadas mejorando su sobrevida y calidad de vida.

Lung transplantation (TP) is a treatment option in children with terminal lung diseases. OBJECTIVE: To evaluate the results and survival of pediatrics lung transplant patients. METHODS: Retrospective analysis of clinical records of lung transplantation of patients ≤ 15 years from Clínica Las Condes, Santiago, Chile. Demographic data, type of transplant, baseline and post transplant lung function, early and late complications and survival rate were analyzed. RESULTS: Nine patients ≤ 15 years-old were transplanted. The average age at transplant was 12.7 years. The main indication was cystic fibrosis (7 patients). The average BMI was 17.6 and all the patients were with home oxygen therapy. 77% used extracorporeal intraoperative support. Average baseline FEV1 was 25.2% with progressive improvement in FEV1 of 77% in the first year. The main early complications were hemorrhage and primary graft dysfunction, while late complications were infections and chronic graft dysfunction. Four patients have died and the estimated 2 years survival was 85%. They achieved school reinsertion and three managed to complete university studies. CONCLUSION: Lung transplantation is an alternative for children with advanced lung diseases improving their survival and quality of life.

Disfunção e não função primária do enxerto hepático: revisão integrativa / Primary liver graft dysfunction and non-function: integrative literature review

RESUMO Evitar mortes na fila de espera por um órgão não é mais o único foco de atenção das equipes de transplantação. As pesquisas e cuidados na prática clínica têm sido cada vez mais voltados para o funcionamento do enxerto pós-implante. O objetivo desse estudo foi identificar a nomenclatura utilizada na literatura para disfunção e não função de um enxerto hepático, bem como, investigar as incidências e fatores de risco. Trata-se de uma revisão integrativa da literatura de publicações na íntegra em português, inglês e espanhol, entre 2012 e 2016, nas bases: CINAHL, MEDLINE, Cochrane, LILACS, BDENF, IBECS, EMBASE e Web of Science. Foram selecionados 14 estudos em que se identificou incidências variando entre 7% e 27% e a nomenclatura utilizada para descrever o evento foi mau funcionamento inicial, hipofunção do enxerto, função marginal ou retardo na função. Foram encontradas incidências de não função primária do enxerto hepático entre 1,4% e 8,4% dos pacientes e a nomenclatura usada para descrever o evento foi disfunção precoce ou perda do enxerto. Os fatores de risco encontrados são relacionados às variáveis do doador, receptor, enxerto e logística do transplante. Conclui-se que o conhecimento das diferentes nomenclaturas empregadas na literatura, das incidências da disfunção e não função primária e seus fatores de risco são fundamentais para qualificar as intervenções de controle dos eventos na perspectiva de melhorar a sobrevida do paciente pós-transplante hepático.

ABSTRACT Avoiding deaths in the waiting list for an organ is no longer the only focus of the transplant teams attention. Research and care in clinical practice has been increasingly focused on post transplant graft survival and functioning. In the present work, we performed an integrative literature review to identify the terminology used about liver graft dysfunction and non-function, as well as to investigate the incidence and risk factors of these clinical events. We chosen articles written in Portuguese, English and Spanish between 2012 and 2016, based on CINAHL, MEDLINE, Cochrane, LILACS, BDENF, IBECS, EMBASE and Web of Science. We selected 14 studies, in which we identified the incidence of hepatic graft dysfunction ranging from 7% to 27%. The terminology used to describe this clinical event was initial malfunction, graft hypofunction, marginal function or delay in function. The primary non-function of the liver graft was found in 1.4% to 8.4% of the patients, and the terminology used to describe the event was early dysfunction or graft loss. The risk factors found are related to donor, recipient, graft and transplant logistics variables. We conclude that knowledge of the different terminologies employed in the literature, related to dysfunction and primary non- function incidence, and of their risk factors are fundamental to qualify the control of the events, aiming to improve patients' survival after liver transplantation.

Evolución clínica, funcional y radiológica de pacientes trasplantados pulmonares con síndrome de bronquiolitis obliterante y disfunción de injerto pulmonar restrictivo / Clinical, functional and radiological evolution of pulmonary transplant patients with bronchiolitis obliterans syndrome and restrictive graft dysfunction

Resumen La principal complicación a largo plazo en trasplantados de pulmón es la disfunción crónica de injerto identificado como bronquiolitis obliterante, existiendo un nuevo patrón denominado Disfunción de Injerto Restrictivo. Objetivo: Evaluar seguimiento espirométrico, radiológico y clínico entre pacientes con síndrome de bronquiolitis obliterante (SBO) y Disfunción de Injerto Restrictivo (DIR) post trasplante pulmonar. Metodología: Se revisaron registros clínicos de trasplantados pulmonares desde 1999 hasta 2017. Se efectuó seguimiento espirométrico e imágenes por tomografía de tórax y factores asociados: infección por Citomegalovirus(CMV), reflujo gastro-esofágico (RGE) y episodios de rechazo agudo. Se analizó sobrevida por Kaplan Meier. Resultados: De 88 pacientes trasplantados de pulmón, 40 desarrollaron disfunción crónica de injerto: 31 (80%) presentaron SBO y 9 (20%) tuvieron DIR. Edad promedio: 47 años en SBO y 46 años en DIR. Siendo fibrosis pulmonar la patología basal predominante en ambos. En SBO se consignaron 14 episodios de rechazo agudo (50%), infección por CMV en 18% y RGE activo en 26%. En la serie DIR hubo 5 episodios de rechazo agudo (62%), 13% de infección por CMV y 67% de RGE activo 6 (p = 0,02). En el seguimiento a 1-2-4-5 años el promedio del VEF1 en SBO fue: 67,3,65, 60 y 48% del valor predicho y en DIR fue 61, 65, 62 y 45% respectivamente. Las imágenes tomográficas en SBO mostraron: hiperinflación y en DIR: fibrosis pleuropulmonar superior. La sobrevida fue de 96,9 meses en SBO y 65,6 meses en DIR (p = 0,06). Conclusions: La disfunción restrictiva presentó menor sobrevida que SBO. RGE se asoció a rechazo restrictivo. La tomografía de tórax difiere en ambos tipos de rechazo crónico.

The main long-term complication in lung transplant patients is chronic graft dysfunction identified as bronchiolitis obliterans, and there is a new pattern called Restrictive Graft Dysfunction. Objective: To evaluate spirometric, radiological and clinical follow-up among patients with bronchiolitis obliterans syndrome (BOS) and Restrictive Allograft Syndrome (RAS) after lung transplantation. Methodology: Lung transplant recipients ' clinical records were reviewed from 1999 to 2017. We carried out a follow up of spirometry, chest tomography imaging and associated factors: cytomegalovirus (CMV) infection, gastroesophageal reflux (GER) and episodes of acute rejection. Survival was analyzed by Kaplan Meier. Results: Out of 88 lung transplant patients, 40 developed chronic graft dysfunction: 31 (80%) presented BOS and 9 (20%) had RAS. Mean age: 47 yr.o. in BOS and 46 yr. o. in RAS. Lung fibrosis was the primary pathology predominant in both conditions. In BOS were reported 14 episodes of acute rejection (50%), CMV infection in 18% and active GER in 26%. In RAS there were 5 episodes of acute rejection (62%), CMV infection in 13% and active GER in 67% (p = 0.02). VEF1 follow-up at 1-2-4-5 years averaged 67, 65, 60 and 8% of reference value in BOS and 61, 65, 62 and 45% in RAS respectively. CT scans showed hyperinflation in BOS and upper pleuropulmonary fibrosis in RAS. BOS survival time was 96.9 months versus 65.6 months in RAS (p = 0.06). Conclusiones: Restrictive dysfunction presented a lower survival rate than BOS. GER was associated with restrictive rejection. Chest tomography differs in both types of chronic rejection.

Primary graft dysfunction of the liver: definitions, diagnostic criteria and risk factors / Disfunção primária do enxerto hepático: definições, critérios diagnósticos e fatores de risco

ABSTRACT Primary graft dysfunction is a multifactorial syndrome with great impact on liver transplantation outcomes. This review article was based on studies published between January 1980 and June 2015 and retrieved from PubMed database using the following search terms: “primary graft dysfunction”, “early allograft dysfunction”, “primary non-function” and “liver transplantation”. Graft dysfunction describes different grades of graft ischemia-reperfusion injury and can manifest as early allograft dysfunction or primary graft non-function, its most severe form. Donor-, surgery- and recipient-related factors have been associated with this syndrome. Primary graft dysfunction definition, diagnostic criteria and risk factors differ between studies.

RESUMO A disfunção primária do enxerto hepático é uma síndrome multifatorial com grande impacto no resultado do transplante de fígado. Foi realizada uma ampla revisão da literatura, consultando a base de dados PubMed, em busca de estudos publicados entre janeiro de 1980 e junho de 2015. Os termos descritivos utilizados foram: “primary graft dysfunction”, “early allograft dysfunction”, “primary non-function” e “liver transplantation”. A disfunção traduz graus diferentes da lesão de isquemia e reperfusão do órgão, e pode se manifestar como disfunção precoce ou, na forma mais grave, pelo não funcionamento primário do enxerto. Fatores relacionados ao doador, ao transplante e ao receptor contribuem para essa síndrome. Existem definições diferentes na literatura quanto ao diagnóstico e aos fatores de risco associados à disfunção primária.

Tres casos de mieloma múltiple de novo después de trasplante renal / Three cases of de novo multiple myeloma after kidney transplantation

RESUMEN La enfermedad renal asociada a cadenas ligeras es frecuente en el contexto de las gammapatías monoclonales, afecta los glomérulos o los túbulos renales, y su causa más común es el mieloma múltiple. Puede desarrollarse después de un trasplante renal por recurrencia de un mieloma múltiple ya existente, o puede ser de diagnóstico nuevo y presentarse con deterioro de la función renal y proteinuria. Siempre se requiere una biopsia renal para confirmar el diagnóstico.

ABSTRACT Light chain-associated kidney compromise is frequent in patients with monoclonal gammopathies; it affects the glomeruli or the tubules, and its most common cause is multiple myeloma. It may develop after a kidney transplant due to recurrence of a preexisting multiple myeloma or it can be a de novo disease manifesting as graft dysfunction and proteinuria. A kidney biopsy is always necessary to confirm the diagnosis.

Associated clinical and laboratory markers of donor on allograft function after heart transplant

Abstract Introduction: Primary graft dysfunction is a major cause of mortality after heart transplantation. Objective: To evaluate correlations between donor-related clinical/biochemical markers and the occurrence of primary graft dysfunction/clinical outcomes of recipients within 30 days of transplant. Methods: The prospective study involved 43 donor/recipient pairs. Data collected from donors included demographic and echocardiographic information, noradrenaline administration rates and concentrations of soluble tumor necrosis factor receptors (sTNFR1 and sTNFR2), interleukins (IL-6 and IL-10), monocyte chemoattractant protein-1, C-reactive protein and cardiac troponin I. Data collected from recipients included operating, cardiopulmonary bypass, intensive care unit and hospitalization times, inotrope administration and left/right ventricular function through echocardiography. Results: Recipients who developed moderate/severe left ventricular dysfunction had received organs from significantly older donors (P =0.020). Recipients from donors who required moderate/high doses of noradrenaline (>0.23 µg/kg/min) around harvesting time exhibited lower post-transplant ventricular ejection fractions (P =0.002) and required longer CPB times (P =0.039). Significantly higher concentrations of sTNFR1 (P =0.014) and sTNFR2 (P =0.030) in donors were associated with reduced intensive care unit times (≤5 days) in recipients, while higher donor IL-6 (P =0.029) and IL-10 (P =0.037) levels were correlated with reduced hospitalization times (≤25 days) in recipients. Recipients who required moderate/high levels of noradrenaline for weaning off cardiopulmonary bypass were associated with lower donor concentrations of sTNFR2 (P =0.028) and IL-6 (P =0.001). Conclusion: High levels of sTNFR1, sTNFR2, IL-6 and IL-10 in donors were associated with enhanced evolution in recipients. Allografts from older donors, or from those treated with noradrenaline doses >0.23 µg/kg/min, were more frequently affected by primary graft dysfunction within 30 days of surgery.