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BACKGROUND: Outcomes related to chronic intestinal failure (CIF) vary significantly within and between countries. While there are extensive European Society of Clinical Nutrition and Metabolism (ESPEN) guidelines on the delivery of optimal care in CIF, there are no international consensus recommendations on the structure or resources required, nor on the process and appropriate outcome measures for delivering such quality care in CIF. AIM: The aim of this position paper is therefore to devise ESPEN-endorsed, internationally agreed quality of care standards, covering the resources, systems and standards that centres should aim for in order to deliver optimal CIF care. METHODS: Members of the Home Artificial Nutrition-CIF Special Interest Group of ESPEN proposed an initial set of quality-of-care standards which was submitted to voting amongst clinicians from international CIF centres using a modified Delphi process, with participants rating each proposed statement as 'essential', 'recommended' or 'not required'. Any statement receiving 80% of more 'not required' responses was excluded. RESULTS: All 30 proposed standards relating to the structure, 18 relating to the process and 16 to the outcome measures of CIF care were deemed to be essential or recommended in more than 80% of respondents. CONCLUSION: This is the first paper to determine and describe internationally-agreed quality of care standards in CIF, which are now aimed at forming the basis for all CIF teams to develop and monitor their service, while also informing policymakers and payers on the infrastructure required for the optimal approach to multi-disciplinary team CIF care delivery. The recording of standardised outcomes should also allow internal and external benchmarking of care delivery within and between CIF centres.
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The role of long-term parenteral support in patients with underlying benign conditions who do not have intestinal failure (IF) is contentious, not least since there are clear benefits in utilising the oral or enteral route for nutritional support. Furthermore, the risks of long-term home parenteral nutrition (HPN) are significant, with significant impacts on morbidity and mortality. There has, however, been a recent upsurge of the use of HPN in patients with conditions such as gastro-intestinal neuromuscular disorders, opioid bowel dysfunction, disorders of gut-brain interaction and possibly eating disorders, who do not have IF. As a result, the European Society of Clinical Nutrition and Metabolism (ESPEN), the European Society of Neuro-gastroenterology and Motility (ESNM) and the Rome Foundation for Disorders of Gut Brain Interaction felt that a position statement is required to clarify - and hopefully reduce the potential for harm associated with - the use of long-term parenteral support in patients without IF. Consensus opinion is that HPN should not be prescribed for patients without IF, where the oral and/or enteral route can be utilised. On the rare occasions that PN commencement is required to treat life-threatening malnutrition in conditions such as those listed above, it should only be prescribed for a time-limited period to achieve nutritional safety, while the wider multi-disciplinary team focus on more appropriate biopsychosocial holistic and rehabilitative approaches to manage the patient's primary underlying condition.
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Nutrição Parenteral , Humanos , Nutrição Parenteral/métodos , Eixo Encéfalo-Intestino/fisiologia , Insuficiência Intestinal/terapia , Nutrição Parenteral no DomicílioRESUMO
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INTRODUCTION: Advanced cancer (AC) is increasingly an indication for home parenteral nutrition (HPN) but an area with possible variation in practice between geographical locations. The aims of this study are to explore the views and experiences of international multi-disciplinary teams to determine opinions and practices. METHODS: An online questionnaire was developed with members of the Home Artificial Nutrition and Chronic Intestinal Failure interest group of the European Society for Clinical Nutrition and Metabolism (ESPEN) and distributed to colleagues involved in managing patients with AC on HPN. RESULTS: A total of 220 responses were included from 5 continents including 36 countries, with 90% of all responses from Europe. Predicted survival was a key factor influencing the decision to commence HPN for most respondents 152/220 (75%), with the majority of participants reporting that patients should have a predicted survival of ≥3 months if considered for HPN (≥3 months: n = 124, 56% vs. <3 months: n = 47, 21%, p < 0.001). However, most respondents were not confident about predicting overall survival in more than 50% of cases (confident n = 40, 23% vs not confident n = 135, 77%, p < 0.001). Barriers to utilising HPN in AC included colleagues' objections (n = 91, 46%), lack of local expertise (n = 55, 28%) and funding restrictions (n = 34, 17%). CONCLUSIONS: Significant consensus was observed regarding AC as indication for HPN, while areas of variation exist. Survival prognostication is often used as an indication for commencing HPN in people with AC, although the majority of respondents were not confident in prognosticating, suggesting better clinical prognostication tools will be of assistance. Further studies are also required to better understand the obstacles faced by clinical teams to commencing HPN that may explain variations in clinical practice between countries, as well as adressing variation in funding.
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Enteropatias , Neoplasias , Nutrição Parenteral no Domicílio , Atitude , Humanos , Neoplasias/terapia , Inquéritos e QuestionáriosRESUMO
Intestinal failure associated liver disease (IFALD) is frequent problem encountered when managing patients receiving parenteral nutrition (PN). Its occurrence is often multifactorial and modification of these factors is vital for the management of such hepatic dysfunction. The use of novel lipid preparations can form part of this management strategy. We present a case whereby such modification of contributing factors, including lipid preparations, led to improvements in IFALD and reversal of hepatic fibrosis.
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Pseudo-Obstrução Intestinal , Cirrose Hepática/dietoterapia , Síndromes de Malabsorção/dietoterapia , Nutrição Parenteral no Domicílio , Adulto , Humanos , Cirrose Hepática/complicações , Síndromes de Malabsorção/complicações , MasculinoRESUMO
BACKGROUND: The catheter lock solutions 2% taurolidine and 0.9% saline are both used to prevent catheter-related bloodstream infections (CRBSIs) in home parenteral nutrition patients. AIMS: To compare the effectiveness and safety of taurolidine and saline. METHODS: This multicentre double-blinded trial randomly assigned home parenteral nutrition patients to use either 2% taurolidine or 0.9% saline for 1 year. Patients were stratified in a new catheter group and a pre-existing catheter group. Primary outcome was the rate of CRBSIs/1000 catheter days in the new catheter group and pre-existing catheter group, separately. RESULTS: We randomised 105 patients, of which 102 were analysed as modified intention-to-treat population. In the new catheter group, rates of CRBSIs/1000 catheter days were 0.29 and 1.49 in the taurolidine and saline arm respectively (relative risk, 0.20; 95% CI, 0.04-0.71; P = 0.009). In the pre-existing catheter group, rates of CRBSIs/1000 catheter days were 0.39 and 1.32 in the taurolidine and saline arm respectively (relative risk, 0.30; 95% CI, 0.03-1.82; P = 0.25). Excluding one outlier patient in the taurolidine arm, mean costs per patient were $1865 for taurolidine and $4454 for saline (P = 0.03). Drug-related adverse events were rare and generally mild. CONCLUSIONS: In the new catheter group, taurolidine showed a clear decrease in CRBSI rate. In the pre-existing catheter group, no superiority of taurolidine could be demonstrated, most likely due to underpowering. Overall, taurolidine reduced the risk for CRBSIs by more than four times. Given its favourable safety and cost profile, taurolidine locking should be considered as an additional strategy to prevent CRBSIs. TRIAL REGISTRATION: Clinicaltrials.gov, identifier: NCT01826526.
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Nutrição Parenteral no Domicílio/métodos , Solução Salina/administração & dosagem , Taurina/análogos & derivados , Tiadiazinas/administração & dosagem , Adulto , Idoso , Bacteriemia/economia , Bacteriemia/epidemiologia , Bacteriemia/etiologia , Infecções Relacionadas a Cateter/economia , Infecções Relacionadas a Cateter/epidemiologia , Infecções Relacionadas a Cateter/prevenção & controle , Método Duplo-Cego , Estudos de Equivalência como Asunto , Feminino , Custos de Cuidados de Saúde , Recursos em Saúde/economia , Recursos em Saúde/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Nutrição Parenteral no Domicílio/efeitos adversos , Nutrição Parenteral no Domicílio/economia , Nutrição Parenteral no Domicílio/estatística & dados numéricos , Solução Salina/efeitos adversos , Solução Salina/economia , Taurina/administração & dosagem , Taurina/efeitos adversos , Taurina/economia , Tiadiazinas/efeitos adversos , Tiadiazinas/economiaRESUMO
BACKGROUND: Clinical and psychosocial outcomes of a multimodal surgical approach for chronic intestinal pseudo-obstruction were analyzed in 24 patients who were followed over a 2- to 12-year period in a single center after surgery or intestinal/multivisceral transplant (CTx). METHODS: The main reasons for surgery were sub-occlusion in surgery and parenteral nutrition-related irreversible complications with chronic intestinal failure in CTx. RESULTS: At the end of follow-up (February 2015), 45.5% of CTx patients were alive: after transplantation, improvement in intestinal function was observed including a tendency toward recovery of oral diet (81.8%) with reduced parenteral nutrition support (36.4%) in the face of significant mortality rates and financial costs (mean, 202.000 euros), frequent hospitalization (mean, 8.8/re-admissions/patient), as well as limited effects on pain or physical wellness. CONCLUSIONS: Through psychological tests, transplant recipients perceived a significant improvement of mental health and emotional state, showing that emotional factors were more affected than were functional/cognitive impairment and social interaction.
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Enteropatias/cirurgia , Pseudo-Obstrução Intestinal/cirurgia , Intestinos/transplante , Qualidade de Vida/psicologia , Vísceras/transplante , Adolescente , Adulto , Doença Crônica , Terapia Combinada , Feminino , Seguimentos , Humanos , Enteropatias/etiologia , Enteropatias/psicologia , Pseudo-Obstrução Intestinal/psicologia , Masculino , Pessoa de Meia-Idade , Nutrição Parenteral Total/efeitos adversos , Período Pós-Operatório , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND AND PURPOSE: Mitochondrial neurogastrointestinal encephalopathy is a rare disorder due to recessive mutations in the thymidine phosphorylase gene, encoding thymidine phosphorylase protein required for mitochondrial DNA replication. Clinical manifestations include gastrointestinal dysmotility and diffuse asymptomatic leukoencephalopathy. This study aimed to elucidate the mechanisms underlying brain leukoencephalopathy in patients with mitochondrial neurogastrointestinal encephalopathy by correlating multimodal neuroradiologic features to postmortem pathology. MATERIALS AND METHODS: Seven patients underwent brain MR imaging, including single-voxel proton MR spectroscopy and diffusion imaging. Absolute concentrations of metabolites calculated by acquiring unsuppressed water spectra at multiple TEs, along with diffusion metrics based on the tensor model, were compared with those of healthy controls using unpaired t tests in multiple white matters regions. Brain postmortem histologic, immunohistochemical, and molecular analyses were performed in 1 patient. RESULTS: All patients showed bilateral and nearly symmetric cerebral white matter hyperintensities on T2-weighted images, extending to the cerebellar white matter and brain stem in 4. White matter, N-acetylaspartate, creatine, and choline concentrations were significantly reduced compared with those in controls, with a prominent increase in the radial water diffusivity component. At postmortem examination, severe fibrosis of brain vessel smooth muscle was evident, along with mitochondrial DNA replication depletion in brain and vascular smooth-muscle and endothelial cells, without neuronal loss, myelin damage, or gliosis. Prominent periependymal cytochrome C oxidase deficiency was also observed. CONCLUSIONS: Vascular functional and histologic alterations account for leukoencephalopathy in mitochondrial neurogastrointestinal encephalopathy. Thymidine toxicity and mitochondrial DNA replication depletion may induce microangiopathy and blood-brain-barrier dysfunction, leading to increased water content in the white matter. Periependymal cytochrome C oxidase deficiency could explain prominent periventricular impairment.
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Doenças de Pequenos Vasos Cerebrais/patologia , Leucoencefalopatias/patologia , Mitocôndrias/patologia , Encefalomiopatias Mitocondriais/patologia , Adulto , Encéfalo/metabolismo , Encéfalo/patologia , Doenças de Pequenos Vasos Cerebrais/etiologia , Doenças de Pequenos Vasos Cerebrais/metabolismo , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Leucoencefalopatias/etiologia , Leucoencefalopatias/metabolismo , Masculino , Mitocôndrias/metabolismo , Encefalomiopatias Mitocondriais/complicações , Encefalomiopatias Mitocondriais/metabolismoRESUMO
A systematic bibliographic research concerning patients operated on for SBS was performed: inclusion criteria were adult age, reconnection surgery and SBS < 100 cm. Autologous gastrointestinal reconstruction represented an exclusion criteria. The outcomes of interest were the rate of total parenteral nutrition (TPN) independence and the length of follow-up (minimum 1 year) after surgery. We reviewed our experience from 2003 to 2013 with minimum 1-year follow-up, dealing with reconnection surgery in 13 adults affected by < 100 cm SBS after massive small bowel resection: autologous gastrointestinal reconstruction was not feasible. Three (out of 5168 screened papers) non randomized controlled trials with 116 adult patients were analysed showing weaning from TPN (40%, 50% and 90% respectively) after reconnection surgery without autologous gastrointestinal reconstruction. Among our 13 adults, mean age was 54.1 years (53.8 % ASA III): 69.2 % had a high stomal output (> 500 cc/day) and TPN dependence was 100%. We performed a jejuno-colonic anastomosis (SBS type II) in 53.8%, in 46.1% of cases without ileo-cecal valve, leaving a mean residual small bowel length of 75.7 cm. In-hospital mortality was 0%. After a minimum period of 1 year of intestinal rehabilitation, all our patients (100%) went back to oral intake and 69.2% were off TPN (9 patients). No one was listed for transplantation. A residual small bowel length of minimum 75 cm, even if reconnected to part of the colon, seems able to produce a TPN independence without autologous gastrointestinal reconstruction after a minimum period of 1 year of intestinal rehabilitation.
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Colo/cirurgia , Síndrome do Intestino Curto/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reoperação , Estudos RetrospectivosRESUMO
BACKGROUND: A review was performed on entero-cutaneous fistula (ECF) repair and early recurrence, adding our twenty adult patients (65% had multiple fistulas). METHODS: The search yielded 4.098 articles but only 15 were relevant: 1.217 patients underwent surgery. The interval time between fistula's diagnosis and operative repair was between 3 months and 1 year. A bowel resection with primary anastomosis was performed in 1.048 patients, 192 (18.3%) underwent a covering stoma: 856 patients (81.7%) had a fistula takedown in one procedure. RESULTS: The patients had 14.3% recurrence and 13.1% mortality rate. In our experience 75% were surgically treated after a period equal or above one year from fistula occurrence: surgery was very demolitive (in 40% remnant small bowel was less than 100 cm). We performed a bowel resection with a hand-sewn anastomosis (95%) without temporary stoma. In-hospital mortality was 0% and at discharge all were back to oral intake with 0% early re-fistulisation. CONCLUSIONS: Literature supports our experience: ECF takedown could be safely performed after an adequate period of recovery from 3 months to one year from fistula occurrence. In our series primary repair (bowel resection plus reconnection surgery without temporary stoma) avoided an early recurrence without mortality.
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Fístula Cutânea/cirurgia , Fístula Intestinal/cirurgia , Intestino Delgado/cirurgia , Complicações Pós-Operatórias/cirurgia , Anastomose Cirúrgica , Procedimentos Cirúrgicos do Sistema Digestório , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva , Fatores de TempoRESUMO
BACKGROUND & AIMS: Obesity is a worldwide health problem. Bariatric surgery (BS) is becoming one of the most commonly used methods for fighting obesity and its associated comorbidities. However, current BS techniques can be associated with early or late complications that may require nutritional support. The aim of this retrospective observational study was to determine the indications and outcomes for patients on Home parenteral nutrition (HPN) due to post-bariatric surgery complications. METHODS: A specific questionnaire was designed by the ESPEN HAN/CIF working group and submitted to HPN centers. This questionnaire included: patient demographics, type of surgery, BMI before surgery and at start of HPN, indications for HPN including technical and nutritional complications (early within 2 months after surgery or late), outcome, PN regimen, and HPN complications. Patients were retrospectively included from January 2008 to June 2014. RESULTS: Eighteen HPN centers responded to the survey. A total of 2880 HPN patients were treated during the study period, 77 of whom had BS (65 females; mean age 51 ± 7 years); gastric bypass was performed in 69% of the patients; mean BMI was 44.4 before surgery and 23.2 at the start of HPN. Indications for HPN were early complications in 17 cases and late complications in 60 cases. Early complications were mostly anastomotic leakage/fistula; late complications were hypoalbuminemia, and vitamin and trace element deficiencies. Out of 77 patients, 16 needed a surgical re-intervention, 29 were weaned off HPN, and 6 died (no HPN-related deaths). During the HPN period, 58% of the patients were re-hospitalized and central venous complications were observed in 41%. Diabetes mellitus was described in 17/77 patients. HPN was supportive in 60 patients and exclusive in 17 patients (mean caloric intake: 23 ± 6 kcal/k BW/day and 1.2 g/kBW/day). Only 7/77 patients resumed their professional activities on HPN. CONCLUSIONS: This is the largest observational multicenter study describing the use of HPN in patients with post-BS complications. Severe hypoalbuminemia is a major late complication. Rates of re-hospitalization and CVC infection were high. HPN may be a "bridge therapy" before surgical revision after BS. The high mortality rate reflects the complexity of these cases.
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Cirurgia Bariátrica/efeitos adversos , Nutrição Parenteral no Domicílio , Complicações Pós-Operatórias/terapia , Adulto , Fístula Anastomótica/etiologia , Fístula Anastomótica/terapia , Índice de Massa Corporal , Gorduras na Dieta/administração & dosagem , Proteínas Alimentares/administração & dosagem , Feminino , Derivação Gástrica , Hospitalização , Humanos , Hipoalbuminemia/etiologia , Hipoalbuminemia/terapia , Masculino , Desnutrição/etiologia , Desnutrição/terapia , Micronutrientes/sangue , Micronutrientes/deficiência , Pessoa de Meia-Idade , Obesidade/complicações , Obesidade/cirurgia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Inquéritos e QuestionáriosRESUMO
BACKGROUND: The use of home parenteral nutrition (HPN) in incurable cancer patients is extremely varied across different countries and institutions. In order to assess the clinical impact implied, we previously conducted a survey of incurable cancer patients receiving HPN, which shows that survival was markedly affected by Karnofsky performance status (KPS), tumor spread, Glasgow prognostic score (GPS) and tumor site. The aim of this study was to develop a nomogram incorporating the above factors for survival prediction. PATIENTS AND METHODS: We gathered a series of 579 patients, all receiving HPN, which was randomly split into a training and a testing sample. Using Cox proportional hazard regression modeling, a nomogram was built in the training sample, in order to estimate median survival or survival probability at 3 and 6 months according to individual patient characteristics. The nomogram performance was then verified in the testing sample. RESULTS: In the training sample, median survival was 3.2 (95% CI 3.0-3.7) months. GPS, KPS, tumor site and spread were confirmed to be significant prognostic factors. A significant interaction was also shown between the site and spread while weight loss (WL), adjusted for body mass index, failed to provide any substantial prognostic contribution. In the testing sample, nomogram performance was good in terms of calibration and discreet regarding discrimination. CONCLUSION: With the growing availability of new oncological treatments and their tendency to transform the trajectory of the advanced cancer into a chronic condition characterized by progressive WL and poor nutrients intake, an increasing number of patients are expected to receive HPN. In such a setting, tools for predicting the survival outcome may play a role toward personalized medicine and for investigating novel experimental therapies. Our proposed nomogram is a step forward in this direction but needs to be made stronger in order to definitely have clinical utility.
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Caquexia/diagnóstico , Caquexia/mortalidade , Neoplasias/diagnóstico , Neoplasias/mortalidade , Nomogramas , Nutrição Parenteral no Domicílio/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Caquexia/terapia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/terapia , Nutrição Parenteral no Domicílio/tendências , Valor Preditivo dos Testes , Taxa de Sobrevida/tendências , Adulto JovemRESUMO
BACKGROUND: Kidney function usually deteriorates after intestinal transplant, with prevalence of renal failure almost 20% after 5 years. We report our results on adults from single institution over >10 years. METHODS: Forty-six patients were transplanted with 22 survivors; we divided them in 2 groups: Group 1, recipients with creatinine>1.2 mg/dL (normal, 0.50-1.2) and Group 2, normal creatinine. Group 1 included 12 patients (9 males) with a mean age of 42.8 years; all lived at home, with normal creatinine at transplant (apart from 1 patient with a creatinine of 1.6 mg/dL), and were mainly transplanted for short bowel syndrome. One underwent retransplantation. Immunosuppression was based on alemtuzumab (8 recipients) plus tacrolimus (FK). Group 2 included 10 patients (6 males) with a mean age of 34.7 years; all lived at home, had normal creatinine at transplantation, and were mainly transplanted for short bowel syndrome. Immunosuppression was mainly based on alemtuzumab (8 recipients) plus FK. RESULTS: There were no relevant differences between the 2 groups regarding number of recipients, sex, baseline creatinine at transplant, reason for transplantation, retransplantation, immunosuppression, antifungal or antiviral therapy, hospitalization, total parenteral nutrition (or fluids), or stoma. The only relevant difference was age (P=.04); patients with deteriorated kidney function or altered creatinine were found to be older.
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Creatinina/análise , Intestinos/transplante , Adulto , Fatores Etários , Feminino , Seguimentos , Rejeição de Enxerto , Humanos , Masculino , Síndrome do Intestino Curto/cirurgiaRESUMO
BACKGROUND: The reliability of endoscopic findings after adult intestinal transplantation on short-term follow-up has been shown. The aim of this study was to evaluate in a long-term follow-up the diagnostic value of endoscopies compared with the biopsy value. METHODS: We evaluated 52 endoscopies over a period of 2 years (2 in each patient in 2010 and 1 in each patient in 2011, plus 1 endoscopy for suspected post-transplant lymphoproliferative disease [PTLD]) on 17 recipients transplanted between the years 2000 and 2006 (more than 5 years of follow-up). RESULTS: All the 52 endoscopic findings were comparable to biopsy definitive results: only 1 case of mild enteritis and 1 case of Epstein-Barr virus (EBV) chronic infection at biopsy were not diagnosed by endoscopy. One case of rectal PTLD and 1 of EBV-related enteritis were diagnosed by use of both procedures. Specificity was 98%: we did not calculate sensitivity because no episodes of rejection were diagnosed because recipients were stable in long-term follow-up. CONCLUSIONS: Endoscopy is a reliable procedure even on a long-term follow-up after intestinal transplantation, allowing a support to biopsy for diagnosis on adult recipients, especially for EBV infections and PTLD surveillance.
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Endoscopia Gastrointestinal , Mucosa Intestinal/patologia , Intestino Delgado/transplante , Adulto , Biópsia , Infecções por Vírus Epstein-Barr/diagnóstico , Feminino , Seguimentos , Humanos , Transtornos Linfoproliferativos/diagnóstico , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA), anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG), a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA) was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA). Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week) with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb) levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft.
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Intestinal transplantation is gaining worldwide acceptance as the main option for patients with irreversible intestinal failure and complicated total parenteral nutrition course. In adults, the main cause is still represented by short bowel syndrome, but tumors (Gardner syndrome) and dismotility disorders (chronic intestinal pseudo-obstruction [CIPO]) have been treated increasingly by this kind of transplantation procedure. We reviewed our series from the disease point of view: although SBS confirmed results achieved in previous years, CIPO is nowadays demonstrating an excellent outcome similar to other transplantation series. Our results showed indeed that recipients affected by Gardner syndrome must be carefully selected before the disease is to advanced to take advantage of the transplantation procedure.
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Intestinos/transplante , Adulto , Fatores Etários , Alemtuzumab , Anticorpos Monoclonais Humanizados/administração & dosagem , Soro Antilinfocitário/administração & dosagem , Daclizumabe , Feminino , Síndrome de Gardner/cirurgia , Humanos , Imunoglobulina G/administração & dosagem , Imunossupressores/uso terapêutico , Enteropatias/cirurgia , Pseudo-Obstrução Intestinal/cirurgia , Intestinos/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Nutrição Parenteral Total , Modelos de Riscos Proporcionais , Síndrome do Intestino Curto/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: The role of home parenteral nutrition (HPN) in incurable cachectic cancer patients unable to eat is extremely controversial. The aim of this study is to analyse which factors can influence the outcome. PATIENTS AND METHODS: We studied prospectively 414 incurable cachectic (sub)obstructed cancer patients receiving HPN and analysed the association between patient or clinical characteristics and surviving status. RESULTS: Median weight loss, versus pre-disease and last 6-month period, was 24% and 16%, respectively. Median body mass index was 19.5, median KPS was 60, median life expectancy was 3 months. Mean/median survival was 4.7/3.0 months; 50.0% and 22.9% of patients survived 3 and 6 months, respectively. At the multivariable analysis, the variables significantly associated with 3- and 6-month survival were Glasgow Prognostic Score (GPS) and KPS, and GPS, KPS and tumour spread, respectively. By the aggregation of the significant variables, it was possible to dissect several classes of patients with different survival probabilities. CONCLUSIONS: The outcome of cachectic incurable cancer patients on HPN is not homogeneous. It is possible to identify groups of patients with a ≥6-month survival (possibly longer than that allowed in starvation). The indications for HPN can be modulated on these clinical/biochemical indices.
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Caquexia/terapia , Carcinoma/mortalidade , Neoplasias do Sistema Digestório/mortalidade , Nutrição Parenteral no Domicílio , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Caquexia/etiologia , Caquexia/mortalidade , Carcinoma/complicações , Neoplasias do Sistema Digestório/complicações , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Adulto JovemRESUMO
BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) has been treated in adults by total parenteral nutrition (TPN) or, if complications arise, by multivisceral transplantation because the stomach is often involved. Eleven adults with CIPO were transplanted by intestinal graft in our center from 2000 to 2011. METHODS: Nine patients underwent isolated intestinal transplant and 2 patients had multivisceral transplant. Immunosuppression was represented by FK and steroids plus induction with alemtuzumab, daclizumab, or thymoglobulin. Average age at transplant was 33.5 years. We reported 1 graftectomy, followed by retransplantation. RESULTS: Seven patients are currently alive with working small bowel; cause of death was infection in the 4 remaining cases. In 9 isolated intestinal transplants, we performed different digestive reconstructions to allow gastric emptying. In 2 cases we were forced, after transplant, to perform ileostomy to improve intestinal motility. Graft and patient survival after 5 years are 60% and 70%, respectively, while after 10 years, 45% and 56%, respectively. CONCLUSIONS: Adults with CIPO and irreversible TPN complications benefit from isolated intestinal transplant with different surgical techniques to empty the native stomach: this strategy achieves good gastric emptying, with effective establishment of oral feeding and graft and patient survivals comparable to isolated intestinal transplant for short bowel syndrome.
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Pseudo-Obstrução Intestinal/cirurgia , Intestinos/transplante , Adolescente , Adulto , Feminino , Humanos , Imunossupressores/administração & dosagem , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Adulto JovemRESUMO
Post-transplantation lymphoproliferative disease (PTLD) of the gastrointestinal (GI) tract is often recognized in transplant recipients. Small bowel recipients are prone to develop GI disease due to the higher incidence of Epstein-Barr Virus (EBV) infection and enteritis as a consequence of heavy immunosuppressive regimens. So far treatment has been based on anti-CD20 therapy (Rituximab), modulation of immunosuppression, antiviral therapy (Gancyclovir), and surgery (up to allograft enterectomy if necessary), whereas endoscopy is usually used to perform the diagnosis via biopsy. We report a case of an adult small bowel recipient, who underwent transplantation due to Gardner's Syndrome 6 years earlier and was EBV positive. A native rectal PTLD was treated using opertive endoscopy combined with antiviral therapy using 4 courses of Rituximab for positive pelvic lymph nodes in addition to reduced immunosuppression. Two years after treatment the recipient is alive and disease-free with a functional graft.
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Intestino Delgado/transplante , Transtornos Linfoproliferativos/complicações , Adulto , Humanos , MasculinoRESUMO
The incidence of early rejection after intestinal transplantation correlates with heightened risk of graft loss and mortality. Many different induction or pre-conditioning protocols have been reported in the last 10 yr to improve outcomes; however, sepsis remains prevalent and diminishes long-term results. We recently began a "2-dose" alemtuzumab trial protocol - 15 mg at day 0 and 15 mg repeated on day 7 - with the hope of reducing our infection rate. We compared three different protocols used at our institution (daclizumab, conventional "4-dose" alemtuzumab, and "2-dose" alemtuzumab). There was a significantly lower rate of early rejection with the "2-dose" alemtuzumab protocol in our study group of mainly (88%) intestinal grafts without accompanying liver engraftment with its protective immunologic effect. Sepsis remained low. Longer follow-up will be required to evaluate the effects of this new protocol on longer-term outcomes.