Long-Term Survival with Heart Transplantation for Fibrosarcoma of the Heart

Primary sarcoma of the heart is a rare disease that has an ominous prognosis with either medical or surgical therapy. We report a case of a 25-year-old woman with sarcoma of the heart who received a transplant and is clinically well after 7 years. We believe that transplantation must be considered in this kind of pathology for selected cases.

Predictive Variables Affecting Transfusion Requirements in Orthotopic Liver Transplantation

INTRODUCTION AND AIMS: Adult orthotopic liver transplantation (OLT) is associated with considerable blood product requirements. The aim of this study was to assess the ability of preoperative information to predict intraoperative red blood cell (RBC) transfusion requirements among adult liver recipients. METHODS: Preoperative variables with previously demonstrated relationships to intraoperative RBC transfusion were identified from the literature: sex, age, pathology, prothrombin time (PT), factor V, hemoglobin (Hb), and platelet count (plt). These variables were then retrospectively collected from 758 consecutive adult patients undergoing OLT from 1997 to 2007. Relationships between these variables and intraoperative blood transfusion requirements were examined by both univariate analysis and multiple linear regression analysis. RESULTS: Univariate analysis confirmed significant associations between RBC transfusion and PT, factor V, Hb, Plt, pathology, and age (P values all < .001). However, stepwise backward multivariate analysis excluded variables Plt and factor V from the multiple regression linear model. The variables included in the final predictive model were PT, Hb, age, and pathology. Patients suffering from liver carcinoma required more blood products than those suffering from other pathologies. Yet, the overall predictive power of the final model was limited (R(2) = .308; adjusted R(2) = .30). CONCLUSION: Preoperative variables have limited predictive power for intraoperative RBC transfusion requirements even when significant statistical associations exist, identifying only a small portion of the observed total transfusion variability. Preoperative PT, Hb, age, and liver pathology seem to be the most significant predictive factors but other factors like severity of liver disease, surgical technique, medical experience in liver transplantation, and other noncontrollable human variables may play important roles to determine the final transfusion requirements.

Impact of RIFLE Classification in Liver Transplantation

Acute renal failure (ARF) is common after orthotopic liver transplantation (OLT). The aim of this study was to evaluate the prognostic value of RIFLE classification in the development of CKD, hemodialysis requirement, and mortality. Patients were categorized as risk (R), injury (I) or failure (F) according to renal function at day 1, 7 and 21. Final renal function was classified according to K/DIGO guidelines. We studied 708 OLT recipients, transplanted between September 1992 and March 2007; mean age 44 +/- 12.6 yr, mean follow-up 3.6 yr (28.8% > or = 5 yr). Renal dysfunction before OLT was known in 21.6%. According to the RIFLE classification, ARF occurred in 33.2%: 16.8% were R class, 8.5% I class and 7.9% F class. CKD developed in 45.6%, with stages 4 or 5d in 11.3%. Mortality for R, I and F classes were, respectively, 10.9%, 13.3% and 39.3%. Severity of ARF correlated with development of CKD: stage 3 was associated with all classes of ARF, stages 4 and 5d only with severe ARF. Hemodialysis requirement (23%) and mortality were only correlated with the most severe form of ARF (F class). In conclusion, RIFLE classification is a useful tool to stratify the severity of early ARF providing a prognostic indicator for the risk of CKD occurrence and death.

Necrose Intestinal na Criança

O termo necrose intestinal traduz exclusivamente um conceito anatomopatológico e clínco, e implica sempre um isquémia instestinal oclusiva ou não. A enterocolite necrosante, em sentido lato, implica uma isquémia intestinal não oclusiva associada a um mecanismo infeccioso. O factor desencadeante da necrose é, por vezes, difícil de determinar. A enterocolite necrosante ocorre em 90% dos casos em recém-nascidos prematuros, sendo mesmo frequente no recém-nascido de termo e rara na crança mais velha e no adulto. Apresentam-se os casos clínicos de duas crianças; uma de sete anos com antecedentes de neutropénia crónica e outra de onze anos com défice cognitivo grave, dismorfia e alterações de comportamento, ambas internadas por quadro de abdómen agudo e choque. Foi efectuada ressecção do segmento jejunal num dos casos e ressecção subtotal do cólon no outro, por necrose. Apesar da cirurgia e da terapêutica médica de suporte, ambas acabaram por morrer em falência multiorgânica, respectivamente três horas após a cirurgia e ao 14º dia de internamento, após segunda intervenção com ressecção do segmento necrosado íleon. A necrópsia de ambos os casos revelou necrose extensa de todo o restante intestino. Os dois casos clínicos comportaram-se como enterocolite necrosante da criança, sendo o diagnóstico de exclusão numa delas, de enterocolite neutropénica. Efectua-se uma revisão dos mecanismos etiopatogénicos da necrose intestinal da criança, desde os tromboembólicos, obstrutivos extrínsecos ou endoluminais, inflamatórios, isquémicos não oclusivos, até aos infecciosos. Para além das medidas gerais de terapêutica, salienta-se a necessidade de uma intervenção cirúrgica precose e de meios diagnósticos terapêuticos, como o doseamento, no sangue e na urina, da proteína de ligação aos ácidos gordos intestinais e a arteriografia selectiva.

Orthotopic Liver Transplantation in Familial Amyloidotic Polyneuropathy Is Associated with Long-Term Progression of Renal Disease

Orthotopic liver transplantation has become the treatment of choice for familial amyloidotic polyneuropathy. The aims of this study were to evaluate the renal complications post orthotopic liver transplantation in familial amyloidotic polyneuropathy and their impact. We retrospectively studied 185 recipients who underwent 217 orthotopic liver transplants. Mean age 36.8±9.5 years, 59% males, 14.3% with renal dysfunction pre orthotopic liver transplantation. Mean follow-up 3.6±3.7 years. Thirty-two patients died. Univariate and multivariate analysis were performed, and p<0.05 was considered significant. Acute kidney injury occurred in 57 patients and renal replacement therapy was needed in 16/57. In multivariate analysis, acute kidney injury was correlated with development of chronic kidney disease (p<0.001). Relating to development of chronic kidney disease, 23.5% had progress to stage 3, 6% to stage 4 and 5.1% to stage 5d. According to Spearmen correlation, risk factors for chronic kidney disease development were age (p<0.001), renal dysfunction pre orthotopic liver transplantation (p<0.001) and acute kidney injury post orthotopic liver transplantation (p<0.001). Mortality was correlated with age (p<0.001), retransplantation need (p=0.004), renal dysfunction pre orthotopic liver transplantation (p<0.001), acute kidney injury post orthotopic liver transplantation (p=0.04), and chronic kidney disease stage 5 (p<0.001). Using binary regression, mortality was correlated with chronic kidney disease development (p=0.02). In conclusion, familial amyloidotic polyneuropathy patients are disposed to renal complications that have a negative impact on the survival of these patients.

Vólvulo Intestinal en el Periodo Neonatal: 8 Años de Experiencia en un Hospital Pediátrico Terciario

Análise dos casos de volvo intestinal ocorridos no período neonatal nos últimos 8 anos (2002 a 2010). Material e métodos: Foram estudados os recém-nascidos admitidos na UCIN cujo diagnóstico de saída foi volvo intestinal. Foram estudados os seguintes parâmetros: idade gestacional e pós-natal, apresentação clínica e imagiológica, intervenção cirúrgica e resultados. Resultados: Foram identificados 15 doentes 7 dos quais no último ano do estudo. Sete RN eram pré-termo (PT) ou ex pré-termo. A mediana de peso ao nascer foi de 2665g (660-3900); 4 RN eram muito baixo peso. A mediana de idade de início dos sintomas foi 7 dias; em 5 RN a doença teve início nas primeiras 24 horas de vida; em 3 destes, o volvo ocorreu in utero. Sinais e sintomas: grande distensão abdominal-12; resíduo gástrico bilioso-11; alterações da parede abdominal-5; dejecções com sangue-4; instabilidade hemodinâmica-6. Imagiologia: grande distensão de ansas, sem ar ectópico- 10 doentes; ausência de ar no abdómen-4; trânsito intestinal contrastado sugestivo de malrotação e volvo-3; ecografia e Doppler abdominal com sinal de “whirlpool”-2. Todos foram submetidos a cirurgia de urgência, sendo o volvo confirmado intraoperatoriamente; foi necessária ressecção intestinal em 9 doentes; 3 ficaram com síndrome do intestino curto; registou-se um óbito por falência multi-orgânica no período pós-operatório. Conclusão: Foi encontrado um elevado número de casos de volvo intestinal em RN pré-termo ou ex pré-termo, de volvo in utero e de elevada ocorrência de casos no último ano do estudo. Resíduo gástrico bilioso e distensão abdominal foram os sintomas mais frequentes de volvo e devem ser tomados em consideração no diagnóstico diferencial com outras situações cirúrgicas abdominais. As sequelas são potencialmente graves.

Impact of Renal Dysfunction on Liver Transplantation: a Retrospective Study in 708 Orthotopic Liver Transplant Recipients

Renal dysfunction often complicates the course of orthotopic liver transplant recipients and is associated with increased morbid -mortality. The aims of this study were to determine the incidence of chronic renal disease and its impact on patient survival. Clinical data included age, gender and weight,aetiology of hepatic failure, presence of diabetes,hypertension, hepatitis B and C infection, renal dysfunction pretransplant and immunosuppression. Laboratory data included serum creatinine at days 1, 7, 21, month 6, 12 and yearly. The glomerular filtration rate was determined by Cockcroft-Gault equation. We studied retrospectively from September 1992 to March 2007 708 orthotopic liver transplant recipients. Mean age 44±12.6 years, 64% males, 17% diabetic, 18.8% hypertensive, 19.9% with hepatitis C and 3.8% hepatitis B. Renal dysfunction pretransplant was known in 21.6%. Mean follow-up was 3.6 years. Mean transplant survival 75% at 12 months. 154 patients died. Univariate and multivariate analyses were performed and a p<0.05 was considered significant. Acute kidney injury occurred in 33.2%. Chronic kidney disease stage 3 was observed in 34.3%,stage 4 in 6.2% and stage 5 in 5.1%. At the time of this study, 46.4% were on Cyclosporine A, 44.7% on tacrolimus and 8.9% on sirolimus. Using multivariate analysis, renal dysfunction was correlated with renal dysfunction pre -orthotopic liver transplant (p<0.001), acute kidney injury (p<0.001), haemodialysis development (p<0.001), and inversely correlated with the use of mycophenolate mophetil (p<0.001); mortality was positively correlated with renal dysfunction pretransplant (p=0.03),chronic kidney disease stage 4 (p=0.001), chronic kidney disease stage 5 (p<0.001) and inversely correlated with the use of tacrolimus (p=0.006). In conclusion orthotopic liver transplant recipients are disposed to renal complications that have a negative impact on survival of these patients.

Transplantation in Highly Sensitised Patients Treated with Intravenous Immunoglobulin and Rituximab

Renal transplant in highly sensitised patients is associated with increased morbidity. The aim of this retrospective study was to evaluate the clinical evolution of 30 highly sensitised deceased donor kidney transplants and the influence of different timing of B cell directed treatment and its importance in the outcome of these patients. All recipients had negative complement dependent lymphocytotoxicity cytotoxic T cell crossmatch and no identified anti human leucocyte antigen class I donor specific antibodies. T cell flow crossmatch was performed within 24h of transplantation with serum obtained pretransplant (historic, recent or baseline). Posttransplant flow crossmatch were performed prospectively starting on the 3rd posttransplantation day. The immunosuppressive regime included thymoglobulin, tacrolimus, mycofenolate mofetil and steroids. Positive flow crossmatch occurred in 20/29 patients by the 3rd posttransplantation day, and in 17/27 patients after the 3rd posttransplantation day. All patients were started on intravenous immunoglobulin before transplantation: in nine patients (group A) at 400mg/kg/day for five days; in the remaining 21 patients (group B), as a continued infusion of 2g/kg during 48h. In group A, Rituximab was added only in the presence of antibody mediated rejection; in group B, introduced on the 3rd posttransplantation day whenever a positive flow crossmatch (with serum obtained pre or posttransplant) was reported. Antibody mediated rejection was observed in 44.4% of patients in group A, and 19% of those in group B. Mean follow-up was 12.2±5.5 months. Overall allograft survival was 76.6%, 81% in group B, and 66.6% in group A. At last follow up, mean serum creatinine was 1.3±0.6 mg/dl. Renal transplantation with pretransplant positive flow crossmatch is highly associated with antibody mediated rejection, despite introduction of intravenous immunoglobulin pretransplantation. However high dose intravenous immunoglobulin for 48h plus Rituximab by the 3rd posttransplantation day reduce the incidence of antibody mediated rejection by more than 50% and allowed for allograft survival of 81% at one year, with an excellent renal function.

ABO-Incompatible Liver Transplantation in Acute Liver Failure: A Single Portuguese Center Study

INTRODUCTION: ABO-incompatible liver transplantation (ABOi LT) is considered to be a rescue option in emergency transplantation. Herein, we have reported our experience with ABOi LT including long-term survival and major complications in these situations. PATIENT AND METHODS: ABOi LT was performed in cases of severe hepatic failure with imminent death. The standard immunosuppression consisted of basiliximab, corticosteroids, tacrolimus, and mycophenolate mofetil. Pretransplantation patients with anti-ABO titers above 16 underwent plasmapheresis. If the titer was above 128, intravenous immunoglobulin (IVIG) was added at the end of plasmapheresis. The therapeutic approach was based on the clinical situation, hepatic function, and titer evolution. A rapid increase in titer required five consecutive plasmapheresis sessions followed by administration of IVIG, and at the end of the fifth session, rituximab. RESULTS: From January 2009 to July 2012, 10 patients, including 4 men and 6 women of mean age 47.8 years (range, 29 to 64 years), underwent ABOi LT. At a mean follow-up of 19.6 months (range, 2 days to 39 months), 5 patients are alive including 4 with their original grafts. One patient was retransplanted at 9 months. Major complications were infections, which were responsible for 3 deaths due to multiorgan septic failure (2 during the first month); rejection episodes (4 biopsy-proven of humoral rejections in 3 patients and 1 cellular rejection) and biliary. CONCLUSION: The use of ABOi LT as a life-saving procedure is justifiable in emergencies when no other donor is available. With careful recipient selection close monitoring of hemagglutinins and specific immunosuppression we have obtained acceptable outcomes.