O transplante hepático com a técnica convencional envolve a ressecção da veia cava inferior retrohepática como parte da hepatectomia do receptor e conseqüente clampeamento da mesma acima e abaixo do fígado, bem como o clampeamento da veia porta durante a fase anepática. Este procedimento pode se caracterizar por alterações hemodinâmicas importantes, disfunção renal e maior sangramento em áreas de dissecção submetidas a um regime de maior pressão. Pacientes idosos, previamente instáveis do ponto de vista hemodinâmico ou muito graves, gealmente, não toleram esta situação. Para evitar estes distúrbios, um \"bypass\" venovenoso, é habitualmente utilizado, permitindo com que o sangue da veia porta e da veia cava inferior retorne à circulação sistêmica através da veia axilar. O \"bypass\" venovenoso, foi adotado por muitos centros transplantadores como procedimento de rotina, enquanto outros empregavam este apenas quando o clampeamento da veia cava inferior determinasse grave alteração hemodinâmica, buscando assim, redução do custo, do tempo de operação e das complicações em função do uso \"bypass\", tais como: hipotermia, coagulopatia e fenômenos tromboembólicos. Outra alternativa técnica, que consiste na preservação da veia cava inferior durante a hepatectomia foi descrita com o intuito de preservar o fluxo na veia cava inferior durante a fase anepática. Além disto, uma anastomose porto-cava temporária pode ser confeccionada para evitar o clampeamento da veia porta e conseqüente congestão esplâncnica. O objetivo deste estudo foi comparar os resultados imediatos do transplante hepático com preservação da veia cava inferior e anastomose porto-cava temporária, ou com o uso do \"bypass\" venovenoso. De outubro de 1999 a outubro de 2001, 104 pacientes submetidos ao transplante hepático foram analisados retrospectivamente. O \"bypass\" venovenoso foi utilizado em 50 pacientes e a técnica de \"piggy back\" em 54. Ambos os grupos foram comparáveis em termos de idade, sexo, diagnóstico e grau de função hepática pré-operatória. Nosso estudo demonstrou não haver diferença entre as duas técnicas em relação à duração da hepatectomia, à duração da operação e à necessidade de transfusão de hemoderivados. Observou-se uma fase anepática mais breve e um menor tempo de permanência na unidade de terapia intensiva no período pós operatório dos pacientes submetidos à técnica que reserva a veia cava inferior, além disto o estudo aponta para uma tendência à redução no tempo de internação, no índice de insuficiência renal pós-operatória e para um melhor funcionamento do enxerto quando os fluxos da veia cava inferior e da veia porta são preservados. / The liver transplant with conventional technique involves the resection of the retrohepatic inferior vena cava as part of the recipient hepatectomy and consequent clamping of the same above and below the liver, as well as the clamping of the portal vein during the anhepatic phase. This procedure can be characterized by critical hemodynamic alterations, renal disfunction and greater bleeding in dissection areas submitted to higher pressure conditions. Elderly patients, previously unstable on a hemodynamic point of view or in very serious conditions, usually do not tolerate this situation. In order to avoid these disturbances, a venovenous bypass is commonly applied, allowing blood from the portal vein and inferior vena cava to return to the systemic circulation through the axilar vein. The venovenous bypass has been adopted by many transplant centers as a routine procedure, while others have applied this procedure only when clamping of the inferior vena cava determines a serious hemodynamic alteration, eeking, in this way, cost cutting, reduction of operation time and complications caused by usage of the bypass, such as: hypothermy, coagulopathy and romboembolic phenomenae. Another technical alternative, which consists of preservation of the inferior vena cava during the hepatectomy was described focusing preservation of the inferior vena cava flow during the anhepatic phase. Besides, a temporary portocaval shunt can be made to avoid clamping of the portal vein and consequently splanchnic congestion. The objective of this study was compare the immediate results of the liver transplantation with inferior vena cava preservation and temporary portocaval shunt or using the venovenous bypass. From October 1999 to October 2001, 104 patients submitted to an liver transplantat were analyzed retrospectively. The venovenous bypass was applied in 50 patients and the \"piggy back\" technique in 54. Both groups were comparable in terms of age, gender, diagnosis and preoperative hepatic conditions. Our study has demonstrated that there was no difference between the two techniques in relation to the hepatectomy and operation time and the need of hemoderivate transfusion. As a result, a shorter anhepatic phase was observed, as well as a shorter stay in intensive care unit during the postoperative period, in relation to patients submitted to the technique that preserves the inferior vena cava. Additionally, the study points out a trend towards reduction of hospital stay, postoperative renal insufficiency, and a better graft function when the inferior cava and portal veins are preserved.

O transplante hepático com a técnica convencional envolve a ressecção da veia cava inferior retrohepática como parte da hepatectomia do receptor e conseqüente clampeamento da mesma acima e abaixo do fígado, bem como o clampeamento da veia porta durante a fase anepática. Este procedimento pode se caracterizar por alterações hemodinâmicas importantes, disfunção renal e maior sangramento em áreas de dissecção submetidas a um regime de maior pressão. Pacientes idosos, previamente instáveis do ponto de vista hemodinâmico ou muito graves, gealmente, não toleram esta situação. Para evitar estes distúrbios, um \"bypass\" venovenoso, é habitualmente utilizado, permitindo com que o sangue da veia porta e da veia cava inferior retorne à circulação sistêmica através da veia axilar. O \"bypass\" venovenoso, foi adotado por muitos centros transplantadores como procedimento de rotina, enquanto outros empregavam este apenas quando o clampeamento da veia cava inferior determinasse grave alteração hemodinâmica, buscando assim, redução do custo, do tempo de operação e das complicações em função do uso \"bypass\", tais como: hipotermia, coagulopatia e fenômenos tromboembólicos. Outra alternativa técnica, que consiste na preservação da veia cava inferior durante a hepatectomia foi descrita com o intuito de preservar o fluxo na veia cava inferior durante a fase anepática. Além disto, uma anastomose porto-cava temporária pode ser confeccionada para evitar o clampeamento da veia porta e conseqüente congestão esplâncnica. O objetivo deste estudo foi comparar os resultados imediatos do transplante hepático com preservação da veia cava inferior e anastomose porto-cava temporária, ou com o uso do \"bypass\" venovenoso. De outubro de 1999 a outubro de 2001, 104 pacientes submetidos ao transplante hepático foram analisados retrospectivamente. O \"bypass\" venovenoso foi utilizado em 50 pacientes e a técnica de \"piggy back\" em 54. Ambos os grupos foram comparáveis em termos de idade, sexo, diagnóstico e grau de função hepática pré-operatória. Nosso estudo demonstrou não haver diferença entre as duas técnicas em relação à duração da hepatectomia, à duração da operação e à necessidade de transfusão de hemoderivados. Observou-se uma fase anepática mais breve e um menor tempo de permanência na unidade de terapia intensiva no período pós operatório dos pacientes submetidos à técnica que reserva a veia cava inferior, além disto o estudo aponta para uma tendência à redução no tempo de internação, no índice de insuficiência renal pós-operatória e para um melhor funcionamento do enxerto quando os fluxos da veia cava inferior e da veia porta são preservados. / The liver transplant with conventional technique involves the resection of the retrohepatic inferior vena cava as part of the recipient hepatectomy and consequent clamping of the same above and below the liver, as well as the clamping of the portal vein during the anhepatic phase. This procedure can be characterized by critical hemodynamic alterations, renal disfunction and greater bleeding in dissection areas submitted to higher pressure conditions. Elderly patients, previously unstable on a hemodynamic point of view or in very serious conditions, usually do not tolerate this situation. In order to avoid these disturbances, a venovenous bypass is commonly applied, allowing blood from the portal vein and inferior vena cava to return to the systemic circulation through the axilar vein. The venovenous bypass has been adopted by many transplant centers as a routine procedure, while others have applied this procedure only when clamping of the inferior vena cava determines a serious hemodynamic alteration, eeking, in this way, cost cutting, reduction of operation time and complications caused by usage of the bypass, such as: hypothermy, coagulopathy and romboembolic phenomenae. Another technical alternative, which consists of preservation of the inferior vena cava during the hepatectomy was described focusing preservation of the inferior vena cava flow during the anhepatic phase. Besides, a temporary portocaval shunt can be made to avoid clamping of the portal vein and consequently splanchnic congestion. The objective of this study was compare the immediate results of the liver transplantation with inferior vena cava preservation and temporary portocaval shunt or using the venovenous bypass. From October 1999 to October 2001, 104 patients submitted to an liver transplantat were analyzed retrospectively. The venovenous bypass was applied in 50 patients and the \"piggy back\" technique in 54. Both groups were comparable in terms of age, gender, diagnosis and preoperative hepatic conditions. Our study has demonstrated that there was no difference between the two techniques in relation to the hepatectomy and operation time and the need of hemoderivate transfusion. As a result, a shorter anhepatic phase was observed, as well as a shorter stay in intensive care unit during the postoperative period, in relation to patients submitted to the technique that preserves the inferior vena cava. Additionally, the study points out a trend towards reduction of hospital stay, postoperative renal insufficiency, and a better graft function when the inferior cava and portal veins are preserved.

The purpose of this quality improvement project was to increase physical activity among postoperative adult liver transplant patients, improve documentation of daily activity, and ultimately influence quality of life (QOL). Quality of life has been shown to improve dramatically after liver transplant, as patients enter transplant severely debilitated with limitations on their physical activity which carry over following transplant. The literature supports that liver transplant patients should engage in physical activity which may improve QOL. Thirteen liver transplant patients were recruited within the seven days of their post-operative hospitalization. Twelve patients consented, were educated about the benefits of walking, given instructions for how to gradually increase their walking activity, and how to track this activity in a daily log. The International Physical Activity Questionnaire (IPAQ) that calculates level of physical activity (metabolic equivalent or MET score) was conducted at baseline and six weeks. Patients were also asked to rate their perceived quality of life on a ten point scale. Eight patients completed the study with four patients medically unable to complete the walking program. Baseline MET and QOL scores were compared between Time 1 and Time 2 (six weeks). The IPAQ baseline score increased from 407.5 MET to 1,711.5 MET, however, results were not statistically significant. Quality of life improved from Time 1 average score of 5.5 (SD=2.51) to Time 2 average score of 8.25 (SD=1.67) and was statistically significant (P=0.27). Liver transplant patients gradually increased their walking activity over a six week period and documented that activity daily. QOL was also purported to increase which is consistent with findings in the literature. Implementing a post liver transplant walking program is feasible and beneficial for patients and should be a standard of care.

ABSTRACT The porphyrias are a diverse group of metabolic disorders arising from diminished activity of enzymes in the heme biosynthetic pathway. They can present with acute neurovisceral symptoms, cutaneous symptoms, or both. The complexity of these disorders is demonstrated by the fact that some acute porphyria patients with the underlying genetic defect(s) are latent and asymptomatic while others present with severe symptoms. This indicates that there is at least one other risk factor required in addition to the genetic defect for symptom manifestation. A systematic review of the heme biosynthetic pathway highlighted the involvement of a number of micronutrient cofactors. An exhaustive review of the medical literature uncovered numerous reports of micronutrient deficiencies in the porphyrias as well as successful case reports of treatments with micronutrients. Many micronutrient deficiencies present with symptoms similar to those in porphyria, in particular vitamin B6. It is hypothesized that a vitamin B6 deficiency and related micronutrient deficiencies may play a major role in the pathogenesis of the acute porphyrias. In order to further investigate the porphyrias, a computational model of the heme biosynthetic pathway was developed based on kinetic parameters derived from a careful analysis of the literature. This model demonstrated aspects of normal heme biosynthesis and illustrated some of the disordered biochemistry of acute intermittent porphyria (AIP). The testing of this model highlighted the modifications necessary to develop a more comprehensive model with the potential to investigated hypotheses of the disordered biochemistry of the porphyrias as well as the discovery of new methods of treatment and symptom control. It is concluded that vitamin B6 deficiency might be the risk factor necessary in conjunction with the genetic defect to trigger porphyria symptoms.