RESUMEN
A complex liver lesion presents a significant challenge in terms of diagnosis and management. This case is an illustrative example, highlighting the steps involved in managing such complex scenarios. This patient, in her early 20s, presented with a fever associated with worsening abdominal pain, as well as a background history of chronic abdominal pain, anorexia, vomiting, constipation and weight loss. The radiology revealed an irregular complex cyst in the liver with biliary and vascular invasion, raising concerns about hepatocellular carcinoma. The diagnosis was changed to alveolar echinococcosis after the infectious diseases consultant gave helpful advice, and echinococcosis antibodies were found. We subsequently started the patient on albendazole therapy. Following prudent advice from hepatobiliary surgeons and given the complexity of the hepatic lesion, a liver transplant was considered the best management option due to the extensive involvement of the biliary and venous systems. The combined approach of albendazole and a liver transplant marked a transformative phase for this patient, putting an end to her prolonged suffering.
Asunto(s)
Albendazol , Carcinoma Hepatocelular , Equinococosis Hepática , Neoplasias Hepáticas , Trasplante de Hígado , Humanos , Equinococosis Hepática/diagnóstico , Equinococosis Hepática/diagnóstico por imagen , Carcinoma Hepatocelular/diagnóstico , Femenino , Neoplasias Hepáticas/diagnóstico , Diagnóstico Diferencial , Albendazol/uso terapéutico , Adulto Joven , Antihelmínticos/uso terapéutico , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVES: De novo malignancies are the most common cause of death after solid-organ transplant. Here, we aimed to summarize standard incidence ratios of de novo malignancies after liver and kidney transplant within the same geographical locations, compare these ratios among differenttypes of de novo malignancies after liver and kidney transplant, and elucidate differences in de novo malignancies between liver and kidney transplant recipients. MATERIALS AND METHODS: We performed a systematic review to identify studies on standard incidence ratios of de novo malignancies after liver and kidney transplant in the United Kingdom, Sweden, South Korea, and Taiwan. RESULTS: Four articles reported standard incidence ratios of de novo malignancies in 14 016 liver transplant recipients (mean follow-up 4.3 ± 0.7 y) and 48179 kidney transplant recipients (mean follow-up 6.1 ± 2.1 y). Mean ratios of oropharyngeal, pulmonary, colorectal, renal, and breast malignancies were 5.3, 1.6, 1.9, 1.8, and 1.1,respectively, after liver transplant and 3.2, 1.7, 1.5, 17.0, and 1.3, respectively, after kidney transplant. Mean ratios of bladder, cervixuterus, and stomach de novo malignancies were 1.8, 2.0, and 2.9, respectively, after liver transplant and 13.0, 1.9, and 1.9,respectively, after kidney transplant. Mean ratios of prostatic and esophageal malignancies were 1.6 and 1.8 after liver transplant and 1.2 and 1.1 after kidney transplant. Mean ratio of ovarian cancer was 1.2 and 2.9, respectively, after liver and kidney transplant. CONCLUSIONS: Low-frequency and lower standard incidence ratios were observed for testicular, ovarian and central nervous system malignancies after kidney and liver transplant. Standard incidence ratios of oropharyngeal and hepatic malignancies were higher after liver transplant compared with kidney transplant. After kidney transplant, standardized ration for renal malignancy were 9.4 times and bladder malignancies were 7.2 times higher compared with liver transplant recipients.
Asunto(s)
Trasplante de Riñón , Trasplante de Hígado , Neoplasias , Humanos , Trasplante de Riñón/efectos adversos , Incidencia , Trasplante de Hígado/efectos adversos , Neoplasias/epidemiología , Factores de Riesgo , Resultado del Tratamiento , Factores de Tiempo , Medición de Riesgo , Femenino , MasculinoRESUMEN
OBJECTIVES: Donor hepatectomy is a major surgery with a relatively safeprofile anda reportedcomplication rate of ~20%. Most complications are non-life threatening and are resolved with conservative measures. However, rare complications may need invasive precautions, ranging from percutaneous interventions to surgeries. MATERIALS AND METHODS: We retrospectively analyzed all living donor hepatectomies at our center. Donors were divided into 2 groups: laparoscopic and open procedures. We collected preoperative, intraoperative and postoperative data of donors.Donor complications were recorded separately intraoperatively and postoperatively according to the Clavien-Dindo postoperative complication classification system. RESULTS: Between July 2018 and April 2023, 215 living donors had hepatectomies, including 48 laparoscopic and 167 open donor hepatectomies. Among donors, 91 were female donors (42.3%) and 124 were male donors (57.7%).The meanage of alldonorswas 33.5±8.1 years, and the mean body mass index (in kilogram divided by meters squared) was 24.6 ± 3.8. Among donors, 124 underwent right and 91 underwent left or left lateral hepatectomies. The mean operative time for all donors was 301 ± 83 minutes, the mean hospital stay was 5.8 ± 1.4 days, and the mean follow-up was 31.9 ± 15.8 months. Four patients (1.8%) had intraoperative complications, including 2 cases of bleeding, 1 diaphragm perforation, and 1 portal vein stenosis. Fourteen patients (7.4%) had major postoperative complications, with 5 patients requiring surgical intervention. CONCLUSIONS: Donor hepatectomy is a complicated surgery that requires extensive preoperative preparation and appropriate donor selection. Postoperative donor complications can be diagnosed early with close follow-up, and a multidisciplinary approach is essential for complication management.
Asunto(s)
Hepatectomía , Laparoscopía , Trasplante de Hígado , Donadores Vivos , Complicaciones Posoperatorias , Humanos , Femenino , Trasplante de Hígado/efectos adversos , Masculino , Hepatectomía/efectos adversos , Estudios Retrospectivos , Adulto , Laparoscopía/efectos adversos , Resultado del Tratamiento , Complicaciones Posoperatorias/etiología , Factores de Riesgo , Factores de Tiempo , Adulto Joven , Persona de Mediana EdadRESUMEN
The use of marginal donor livers, particularly steatotic livers, could help to resolve the problem of organ shortage and wait list mortality. Ischemia-free liver transplant with the potential to avoid ischemiareperfusion injury and related complications, particularly early allograft dysfunction, could positively encourage the use of marginal donorlivers and extend the donor pool. Here, we describe the first case in a Western country of ischemia-free liver transplant of a marginal donor liver. To date, a research team in China is the only group to have described and used this technique. The technical and setup aspects are illustrated, and present controversies are discussed. A 58-year-old female patient received a transplant of a >60% steatotic donor liver. The transplant was accomplished with the ischemia-free liver transplant technique, and the donor liver was procured and transplanted under continuous normothermic machine perfusion. The donor liver functional parameters under normothermic machine perfusion were reassuring, and recipient recovery was uneventful. Although ischemia-free liver transplant is a technically and organizationally demanding procedure, our case demonstrates the feasibility of the ischemia-free liver transplant technique and encourages the development and expansion of its use.
Asunto(s)
Selección de Donante , Trasplante de Hígado , Perfusión , Donantes de Tejidos , Humanos , Femenino , Persona de Mediana Edad , Resultado del Tratamiento , Perfusión/métodos , Donantes de Tejidos/provisión & distribución , Hígado Graso/cirugía , Factores de TiempoRESUMEN
OBJECTIVES: The proportion of older transplant recipients has increased. Cognitive impairment is not rare after kidney transplant, but data on this issue in liver transplant recipients are scarse. MATERIALS AND METHODS: In this cross-sectional study, we evaluated all liver transplant recipients from a single center in Brazil from July 2018 to June 2020 in terms of cognitive performance to determine the prevalence of neurocognitive disorder. We compared liver transplant recipients with neurocognitive disorder with liver transplant recipients without neurocognitive disorder. We also compared those with an alcoholic cause of liver transplant with other patients. The presence of depressive symptoms was assessed. We performed correlations of clinical data with cognitive scores. RESULTS: In a sample of 100 recipients with median age of 62 years (interquartile range, 56.2-69 y), neurocognitive disorder was present in 21% of the group. Patients with cognitive impairment were older (68 y [61-72] vs 61 y [52-68]; P = .019) and had a trend to higher proportion of persistent kidney injury (33.3% vs 13.9%; P = .055) versus patients without cognitive impairment. Recipients with alcoholic cause of liver transplant exhibited worse cognitive performance in the Mini-Mental State Examination (score of 26 [23.7-28.2] vs 28 [26-29]; P = .024) and the Alzheimer Disease Assessment Scale-cognitive (score of 10.4 [8.6-14.2] vs 8 [6.3-10]; P = .008) than other patients. Weak negative correlations were shown in cognitive performance scores versus recipient age (Semantic Verbal Fluency test, r = -0.334 [P = .001]; Clock Drawing test, r = -0.209 [P = .037]; Alzheimer Disease Assessment Scale-cognitive, r = -0.323 [P = .001]). CONCLUSIONS: Neurocognitive disorder was common in liver transplant recipients, in part due to increased age. This study also suggested a role for alcoholic cause of liver transplant and persistent kidney injury in the development of cognitive impairment.
Asunto(s)
Cognición , Disfunción Cognitiva , Trasplante de Hígado , Humanos , Estudios Transversales , Trasplante de Hígado/efectos adversos , Persona de Mediana Edad , Masculino , Femenino , Brasil/epidemiología , Factores de Riesgo , Anciano , Prevalencia , Disfunción Cognitiva/diagnóstico , Disfunción Cognitiva/epidemiología , Disfunción Cognitiva/etiología , Disfunción Cognitiva/psicología , Resultado del Tratamiento , Factores de Edad , Medición de Riesgo , Hepatopatías Alcohólicas/cirugía , Hepatopatías Alcohólicas/psicología , Hepatopatías Alcohólicas/epidemiología , Hepatopatías Alcohólicas/diagnósticoRESUMEN
INTRODUCTION: Sarcopenia is common in children after liver transplantation (LTx). Resistance training (RT) may be effective in combating sarcopenia. OBJECTIVES: The purpose of the study was to test the feasibility and impact of a 12-week RT program on skeletal muscle mass (SMM), muscle strength, physical performance (PP), and child-parent perspectives about RT. METHODS: Children (6-18 years) post-LTx and healthy controls (HC) underwent progressive RT using resistance bands. SMM and adipose tissue (MRI: abdomen and thigh), muscle strength (handgrip, push-ups, sit-to-stand), and PP (6-minute walk test [6MWT], timed-up-and-down-stair test [TUDS]) were measured before and after 12-weeks of RT. RESULTS: Ten children post-LTx (11.9 ± 3.5 years) and 13 HC (11.7 ± 3.9 years) participated. LTx children significantly increased abdominal SM-index (+4.6% LTx vs. a -2.7% HC; p = 0.01) and decreased visceral adipose tissue-index (-18% LTx vs. -0.8% HC; p = 0.04) compared to HC. No thigh SMI changes were noted. Significant increases in 6MWT distance (LTx; p = 0.04), number of push-ups (p = 0.04), and greater reduction times for TUDS (-10.6% vs. +1.7%; p = 0.05) occurred after 12 weeks. Higher thigh muscle-fat content was associated with worse physical performance. These results were impacted by adherence (≥75% vs. <75%) and family engagement. CONCLUSIONS: RT in children post-LTx is feasible and effective. RT in children post-LTx may alleviate adverse outcomes associated with sarcopenia.
Asunto(s)
Trasplante de Hígado , Fuerza Muscular , Entrenamiento de Fuerza , Sarcopenia , Humanos , Masculino , Trasplante de Hígado/efectos adversos , Proyectos Piloto , Sarcopenia/etiología , Niño , Femenino , Adolescente , Pronóstico , Estudios de Casos y Controles , Estudios de Seguimiento , Entrenamiento de Fuerza/métodos , Músculo Esquelético/fisiopatología , Complicaciones Posoperatorias , Servicios de Atención de Salud a DomicilioRESUMEN
INTRODUCTION: Physical frailty is associated with increased mortality and poor quality of life (QoL) before and after liver transplantation (LT). Evidence is lacking on how to tailor exercise and behavioural techniques in this patient population. METHODS AND ANALYSIS: Home-based EXercise and motivAtional programme before and after Liver Transplantation (EXALT) is a phase 2b, open-label, two-centre randomised controlled clinical trial designed to investigate whether a remotely monitored 'home-based exercise and theory-based motivation support programme (HBEP)' before and after LT improves QoL in LT recipients. Adult patients awaiting a primary LT will be assessed for eligibility at two LT centres (Birmingham, Royal Free London). Participants will be randomly assigned (1:1) to receive either an HBEP while on the LT waiting list through to 24 weeks after LT (Intervention) or a patient exercise advice leaflet (Control). Using a standard method of difference in means (two-sided significance level 0.05; power 0.90) and accounting for a 35% attrition/withdrawal rate, a minimum of 133 patients will be randomised to each treatment group. The primary outcome measure will be assessed using intention-to-treat analysis of the difference in the Physical Component Score of Short form-36 version 2.0 health-related QoL questionnaire between the groups at 24 weeks post-LT. ETHICS AND DISSEMINATION: The protocol was approved by the South Central-Hampshire A National Research Ethics Committee. Recruitment into the EXALT trial started in May 2022 and is due to end in June 2024, with 217/266 patients randomised to date. The intervention follow-up is due to finish in May 2026. The findings of this trial will be disseminated through peer-reviewed publications, conferences and social media. TRIAL REGISTRATION NUMBER: ISRCTN13476586.
Asunto(s)
Trasplante de Hígado , Motivación , Calidad de Vida , Humanos , Trasplante de Hígado/psicología , Terapia por Ejercicio/métodos , Estudios Multicéntricos como Asunto , Ensayos Clínicos Fase II como Asunto , Masculino , Ensayos Clínicos Controlados Aleatorios como Asunto , Adulto , Femenino , Fragilidad , Ejercicio Físico/fisiología , Ejercicio Físico/psicologíaRESUMEN
Deceased donor organs for transplantation are costly. Expenses include donor assessment, pre-operative care of acceptable donors, surgical organ recovery, preservation and transport, and other costs. US Organ Procurement Organizations (OPOs) serve defined geographic areas in which each OPO has exclusive organ recovery responsibilities including detailed reporting of costs. We sought to determine the costs of procuring deceased donor livers by examining reported organ acquisition costs from OPO cost reports. Using 6 years of US OPO cost report data for each OPO (2013-2018), we determined the average cost of recovering a viable (i.e., transplanted) liver for each of the 51 independent US OPOs. We examined predictors of these costs including the number of livers procured, the percent of nonviable livers, direct procurement costs, coordinator salaries, professional education, and local cost of living. A cost curve estimated the relationship between the cost of livers and the number of locally procured livers. The average cost of procured livers by individual OPO-year varied widely from $11 393 to $65 556 (average $31 659) over the six study years. An increase in the overall number of procured livers was associated with lower direct costs, administrative, and procurement overhead costs, but this association differed for imported livers. Cost per local liver decreased linearly for each additional liver, while importing more livers was only cost saving until 200 livers, with imported livers costing more ($39K vs. $31.7K). The largest predictor of variation in cost was the aggregate of direct costs (e.g., hospital costs) to recover the organ (57%). Cost increases were 2.5% per year (+$766/year). This information may be valuable in determining how OPOs might improve service to transplant centers and the patients they serve.
Asunto(s)
Trasplante de Hígado , Donantes de Tejidos , Obtención de Tejidos y Órganos , Humanos , Obtención de Tejidos y Órganos/economía , Trasplante de Hígado/economía , Donantes de Tejidos/provisión & distribución , Estados Unidos , Costos de la Atención en Salud/estadística & datos numéricos , Pronóstico , Masculino , Estudios de SeguimientoRESUMEN
Rationale: Understanding the immune mechanisms associated with liver transplantation (LT), particularly the involvement of tissue-resident memory T cells (TRMs), represents a significant challenge. Methods: This study employs a multi-omics approach to analyse liver transplant samples from both human (n = 17) and mouse (n = 16), utilizing single-cell RNA sequencing, bulk RNA sequencing, and immunological techniques. Results: Our findings reveal a comprehensive T cell-centric landscape in LT across human and mouse species, involving 235,116 cells. Notably, we found a substantial increase in CD8+ TRMs within rejected grafts compared to stable ones. The elevated presence of CD8+ TRMs is characterised by a distinct expression profile, featuring upregulation of tissue-residency markers (CD69, CXCR6, CD49A and CD103+/-,), immune checkpoints (PD1, CTLA4, and TIGIT), cytotoxic markers (GZMB and IFNG) and proliferative markers (PCNA and TOP2A) during rejection. Furthermore, there is a high expression of transcription factors such as EOMES and RUNX3. Functional assays and analyses of cellular communication underscore the active role of CD8+ TRMs in interacting with other tissue-resident cells, particularly Kupffer cells, especially during rejection episodes. Conclusions: These insights into the distinctive activation and interaction patterns of CD8+ TRMs suggest their potential utility as biomarkers for graft rejection, paving the way for novel therapeutic strategies aimed at enhancing graft tolerance and improving overall transplant outcomes.
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Linfocitos T CD8-positivos , Rechazo de Injerto , Trasplante de Hígado , Células T de Memoria , Análisis de la Célula Individual , Linfocitos T CD8-positivos/inmunología , Linfocitos T CD8-positivos/metabolismo , Humanos , Rechazo de Injerto/inmunología , Animales , Ratones , Células T de Memoria/inmunología , Células T de Memoria/metabolismo , Análisis de la Célula Individual/métodos , Análisis de Secuencia de ARN/métodos , Subunidad alfa 3 del Factor de Unión al Sitio Principal/genética , Subunidad alfa 3 del Factor de Unión al Sitio Principal/metabolismo , Memoria Inmunológica , Masculino , Ratones Endogámicos C57BL , Antígenos CD/metabolismo , Antígenos CD/genética , Femenino , Persona de Mediana Edad , Proteínas de Dominio T BoxAsunto(s)
Delirio , Trasplante de Riñón , Trasplante de Hígado , Humanos , Delirio/etiología , Niño , Complicaciones Posoperatorias/etiología , Preescolar , Adolescente , MasculinoRESUMEN
Liver grafts from controlled donation after circulatory death (cDCD) donors have lower utilization rates due to inferior graft and patient survival rates, largely attributable to the increased incidence of ischemic cholangiopathy, when compared with grafts from brain dead donors (DBD). Normothermic regional perfusion (NRP) may improve the quality of cDCD livers to allow for expansion of the donor pool, helping to alleviate the shortage of transplantable grafts. A systematic review and metanalysis was conducted comparing NRP cDCD livers with both non-NRP cDCD livers and DBD livers. In comparison to non-NRP cDCD outcomes, NRP cDCD grafts had lower rates of ischemic cholangiopathy [RR = 0.23, 95% CI (0.11, 0.49), p = 0.0002], primary non-function [RR = 0.51, 95% CI (0.27, 0.97), p = 0.04], and recipient death [HR = 0.5, 95% CI (0.36, 0.69), p < 0.0001]. There was no difference in outcomes between NRP cDCD donation compared to DBD liver donation. In conclusion, NRP improved the quality of cDCD livers compared to their non-NRP counterparts. NRP cDCD livers had similar outcomes to DBD grafts. This provides further evidence supporting the continued use of NRP in cDCD liver transplantation and offers weight to proposals for its more widespread adoption.
Asunto(s)
Trasplante de Hígado , Perfusión , Donantes de Tejidos , Trasplante de Hígado/métodos , Humanos , Perfusión/métodos , Preservación de Órganos/métodos , Supervivencia de Injerto , Muerte Encefálica , Obtención de Tejidos y Órganos/métodosRESUMEN
BACKGROUND: Cardiac surgery is considered a contraindication in patients with advanced liver cirrhosis (LC) due to increased mortality and morbidity. There are limited data on the treatment strategy and management of this population. We aimed to present our strategy and evaluate the clinical outcome of cardiac surgery in patients with LC. METHODS: Our strategy was (i) to list patients for liver transplant (LT) at the time of cardiac surgery; (ii) to maintain high cardiopulmonary bypass (CPB) flow (index up to 3.0 L/min/m2) based on hyper-dynamic states due to LC; and (iii) to proceed to LT if patients' liver function deteriorated with an increasing model for end-stage liver disease Na (MELD-Na) score after cardiac surgery. Thirteen patients (12 male and 1 female [mean age, 63.0]) with LC who underwent cardiac surgery between 2017 and 2024 were retrospectively analyzed. RESULTS: Six patients were listed for LT. Indications for cardiac surgery included coronary artery disease (N = 7), endocarditis (N = 2), and tricuspid regurgitation (N = 1), tricuspid stenosis (N = 1), mitral regurgitation (N = 1), and hypertrophic obstructive cardiomyopathy (N = 1). The Child-Pugh score was A in five, B in six, and C in one patient. The procedure included coronary artery bypass grafting (N = 6), single valve surgery (mitral valve [N = 2] and tricuspid valve [N = 1]), concomitant aortic and tricuspid valve surgery (N = 2), and septal myectomy (N = 1). Two patients had a history of previous sternotomy. The perfusion index during CPB was 3.1 ± 0.5 L/min/m2. Postoperative complications include pleural effusion (N = 6), bleeding events (N = 3), acute kidney injury (N = 1), respiratory failure requiring tracheostomy (N = 2), tamponade (N = 1), and sternal infection (N = 1). There was no in-hospital death. There was one remote death due to COVID-19 complication. Preoperative and postoperative highest MELD-Na score among listed patients was 15.8 ± 5.1 and 19.3 ± 5.3, respectively. Five patients underwent LT (1, 5, 8, 16, and 24 months following cardiac surgery) and one patient remains on the list. Survival rates at 1 and 3 years are 100% and 75.0%, respectively. CONCLUSION: Cardiac surgery maintaining high CPB flow with LT backup is a feasible strategy in an otherwise inoperable patient population with an acceptable early and midterm survival when performed in a center with an experienced cardiac surgery and LT program.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Cirrosis Hepática , Trasplante de Hígado , Humanos , Masculino , Femenino , Persona de Mediana Edad , Cirrosis Hepática/cirugía , Cirrosis Hepática/complicaciones , Estudios Retrospectivos , Procedimientos Quirúrgicos Cardíacos/métodos , Pronóstico , Anciano , Complicaciones Posoperatorias , Tasa de Supervivencia , Estudios de Seguimiento , COVID-19/complicaciones , Resultado del Tratamiento , Cardiopatías/cirugía , Cardiopatías/complicacionesRESUMEN
The shortage of donor organs remains an unresolved issue in livertransplantation worldwide. Consequently, strategies for expanding the donor pool are currently being developed. Donors meeting extended criteria undergo thorough evaluation, as livers obtained from marginal donors yield poorer outcomes in recipients, including exacerbated reperfusion injury, acute kidney injury, early graft dysfunction, and primary nonfunctioning graft. However, the implementation of machine perfusion has shown excellent potential in preserving donor livers and improving their characteristics to achieve better outcomes for recipients. In this review, we analyzed the global experience of using machine perfusion in livertransplantation through the history ofthe development ofthis method to the latest trends and possibilities for increasing the number of liver transplants.
Asunto(s)
Supervivencia de Injerto , Trasplante de Hígado , Perfusión , Humanos , Trasplante de Hígado/historia , Perfusión/historia , Perfusión/métodos , Historia del Siglo XX , Historia del Siglo XXI , Resultado del Tratamiento , Preservación de Órganos/historia , Preservación de Órganos/métodos , Donantes de Tejidos/provisión & distribución , Donantes de Tejidos/historia , Diseño de Equipo , Factores de Riesgo , Selección de Donante/historia , Animales , Historia del Siglo XIXRESUMEN
OBJECTIVES: Primary sclerosing cholangitis is an autoimmune illness affecting the intrahepatic and/or extrahepatic bile ducts that has a varying clinical history and no clear therapy. Recurrence of primary sclerosing cholangitis after transplantation can cause recurring liver failure, decreased survival, and the necessity for retransplant. Here, we explored the incidence of recurrence while also identifying the risk factors of primary sclerosing cholangitis. MATERIALS AND METHODS: In this retrospective cohort study, we collected demographic and clinical data from patients with a history of primary sclerosing cholangitis after liver transplant between 2011 and 2021. With SPSS software, we compared results in 2 groups of patients (with and without recurrent sclerosing biliary cholangitis) in terms of demographic and clinical variables. RESULTS: The study included 408 patients. Lower donor age and the occurrence of acute cellularrejection were shown to be key risk factors for recurrence of primary sclerosing cholangitis. Acute cellularrejection showed the best likelihood of predicting primary sclerosing cholangitis recurrence. As the number of acute cellular rejection episodes increased, so did the chance of primary sclerosing cholangitis. Death rate of patients with recurrence of primary sclerosing cholangitis was 40.8% (n = 20 patients) compared with 18.9% (n = 68 patients) in those without recurrence (significant at P < .001). CONCLUSIONS: The recurrence of primary sclerosing cholangitis had a detrimental effect on survival after liver transplant. Modifiable risk variables have the potentialto affecttherapies on care and prevention of primary sclerosing cholangitis recurrence. Donor age and acute cellular rejection were risk factors for decreased survival and higher primary sclerosing cholangitis recurrence. The use of mycophenolate (Cellcept) increased recurrence, but tacrolimus reduced mortality.
Asunto(s)
Colangitis Esclerosante , Inmunosupresores , Trasplante de Hígado , Recurrencia , Humanos , Colangitis Esclerosante/cirugía , Colangitis Esclerosante/mortalidad , Colangitis Esclerosante/diagnóstico , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/mortalidad , Factores de Riesgo , Estudios Retrospectivos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Resultado del Tratamiento , Inmunosupresores/efectos adversos , Factores de Tiempo , Medición de Riesgo , Irán/epidemiología , Adulto Joven , Incidencia , Factores de Edad , Adolescente , Rechazo de Injerto/mortalidad , Rechazo de Injerto/prevención & control , Rechazo de Injerto/diagnóstico , Rechazo de Injerto/inmunologíaRESUMEN
BACKGROUND: Nijmegen breakage syndrome (NBS) is an autosomal recessive DNA repair disorder that manifests through increased genomic instability, malignancy, and cellular and humoral immunodeficiencies. The prognosis for NBS patients is poor due to their increased susceptibility to fatal infections and lymphoproliferative malignancies. Currently, there is no specific treatment for NBS, though allogeneic hematopoietic stem cell transplantation (HSCT) has been performed and documented as case series to demonstrate the utility of transplantation. METHODS: A 14-year-old girl with NBS and haploidentical HSCT from her older brother due to recurrent lung infection was referred for liver transplantation (LT) due to liver cirrhosis, hepatopulmonary syndrome (HPS), and suspicion of liver malignancy. It was decided to perform LT using the living donor who had previously donated for HSCT. RESULTS: Living donor left lobe LT was successfully performed from her brother. The patient experienced no complications in the early postoperative period and was discharged on the seventh postoperative day. Pathological examination of extracted liver has shown "intermediate cell carcinoma" in two foci. After 1 year LT, the patient has had an uneventful course in terms of LT complications and infection, with minimal immunosuppression. CONCLUSIONS: NBS patients have an increased prevalence of malignancies, including primary hepatic malignancy, but most are managed medically or with limited resections. Transplantation in these patients can be curative for hepatic malignancy with a favorable safety profile.
Asunto(s)
Síndrome Hepatopulmonar , Neoplasias Hepáticas , Trasplante de Hígado , Síndrome de Nijmegen , Humanos , Adolescente , Femenino , Neoplasias Hepáticas/cirugía , Síndrome de Nijmegen/complicaciones , Síndrome Hepatopulmonar/cirugía , Síndrome Hepatopulmonar/etiología , Síndrome Hepatopulmonar/terapia , Trasplante de Médula Ósea/efectos adversos , Donadores VivosRESUMEN
AIM: To examine the long-term results and treatment effectiveness of liver transplantation (LT) in the treatment of homozygous familial hypercholesterolemia (HoFH) in children and adolescents. METHOD: Patients who underwent LT due to HoFH between 2007 and 2023 were included in the study. The patients' demographic data, clinical findings, preoperative and postoperative laboratory examinations, transplantation complications, and postoperative disease courses were evaluated. RESULTS: There were five boys with an average age of 6.2 (median: 6, range 4-10) years in the study. The average total cholesterol level of the patients before transplantation was 923 (median: 950, range: 780-1002) mg/dL and the average LDL-cholesterol level was 864 (median: 852, range: 770-957) mg/dL. No patients died of transplant-related complications. After an average follow-up of 9.2 (median: 9, range: 1.5-16) years, the average total cholesterol level of the patients was 197 (median: 164, range: 137-359) mg/dL, and the average LDL-cholesterol level was 138 (median: 92, range: 85-313) mg/dL. Four (80%) patients developed atherosclerotic cardiovascular disease during follow-up, and two (40%) died of this cause. CONCLUSION: LT in the treatment of HoFH did not help our patients reach the target LDL-cholesterol level after transplantation and did not prevent the development of cardiovascular disease. Therefore, LT alone is not curative in the treatment of HoFH.