Single-Center Experience of Transplantation for Polycystic Liver Disease

BackgroundPolycystic liver disease (PLD) may lead to massive hepatomegaly, abdominal distension, pain, and various degrees of dyspnea. The surgical treatment of this entity remains controversial.MethodsWe report our experience from a retrospective analysis of 23 patients suffering from PLD who were treated with liver transplantation (LT) in our institution.ResultsLiver transplantation for PLD patients with extensive hepatic involvement offers excellent symptoms relief. The actuarial 1-, 3-, and 5-year survival rate after transplantation was 86%.ConclusionsOur experience demonstrates that PLD patients with extensive hepatic involvement and who are treated with LT have good long-term prognosis and excellent symptoms relief. LT might be considered in severe PLD cases where conventional surgery is not a curative option, and it must be balanced against the risks of LT and lifelong commitment to immunosuppression.     

Multicentric Study of the Andalusian Experience in Polycystic Liver Disease as Indication for Liver Transplantation

Background

The purpose of this study was to determine the morbidity and survival in patients with polycystic liver disease (PLD) undergoing liver transplantation (LT) in 4 Spanish hospitals.

Autosomal-Dominant Polycystic Kidney Disease and Kidney Transplantation: Experience of a Single Center

Background

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disease that frequently leads to end-stage renal disease and is a common indication for kidney transplantation. We sought to evaluate the demographic characteristics, graft and patient survival, and some posttransplantation complications among ADPKD recipients.

Methods

This retrospective study included 445 renal transplant recipients, among whom 48 had ADPKD. We excluded patients with pretransplantation diabetes mellitus. We evaluated patient and graft survivals as well as posttransplantation complications.

Results

There was no difference between the 2 groups with respect to demographic or transplant characteristics, except for older age among the ADPKD group (51.2 ± 8.6 years vs 44 ± 13.1 years; P < .001). We also observed no significant difference with regard to immediate graft function, immunological graft, or patient survival. Although not significant, there was a lower incidence of proteinuria and a greater number of acute rejections among ADPKD patients. As for posttransplantation complications, there was no difference regarding the prevalence of hypertension, but there was more erythrocytosis among the ADPKD group. The incidence of posttransplantation diabetes mellitus was significantly greater in ADPKD patients (33.3% vs 17.1%; P = .009), and remained significant after adjusting for confounding variables by multivariate analysis with an adjusted odds ratio of 2.3 (95% confidence interval, 1.008-5.136; P = .048).

Conclusion

Our results suggested that ADPKD patients display a greater incidence of diabetes mellitus posttransplantation; ADPKD emerged as an independent predictor for this complication.     

Liver Transplantation for Polycystic Liver Disease Due to Huge Liver With Related Complications: A Case Report

Polycystic liver disease is characterized by multiple cystic lesions on the liver. It is an uncommon autosomal dominant disease. The cysts' diameters range from 20 to 30 cm to small microscopic nodules. Generally, more than half of the liver parenchyma is covered. The mass effect of the liver created by the large cysts can cause life-threatening symptoms such as weight loss, reduction of oral intake, and malnutrition. Liver transplantation is the best treatment option in symptomatic patients. We present a patient who had polycystic liver and kidney disease, and we performed liver transplantation because of his life-threatening symptoms.     

Autosomal Dominant Polycystic Kidney Disease Transplant Recipients After Kidney Transplantation: A Single-center Experience

Kidney transplantation is indicated for end-stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) causes structural degeneration of the kidney and eventually becomes end-stage renal disease. ADPKD patients usually have several renal and nonrenal complications. We analyzed our kidney transplantation activities between 1991 and 2010 regarding ADPKD. We followed up with patients to December 31, 2016. Data were collected as patient and graft survival rates, the prevalence of polycystic manifestation of the gastrointestinal tract and other organs, and the attendance of urinary tract infection. Among the 734 kidney transplantations, 10.9% (n = 80) had an ADPKD. Four patients (5%) had diverticulum perforation. The prevalence of post-transplantation urinary tract infection was higher in ADPKD patients (55.9%) compared to non-ADPKD patients (44.1%). The 1-, 3-, and 5-year overall survival rates in ADPKD recipients versus non-ADPKD patients are 77.5%, 70.0%, and 67.5% versus 86.4%, 83.0%, and 80.1%, respectively. Patients with ADPKD were transplanted at an elder age compared to others (median: 47.5 years vs. 39.9 years). Female patients had longer graft survival times than males. ADPKD implies multiple cystic degeneration of the kidneys; however, it can cause structural degeneration in other organs. It is typical for ADPKD patients to have an acute abdominal-like syndrome. Immunosuppressive drugs can hide the clinical picture, which makes early diagnosis difficult.     

Antibody-Mediated Rejection After Orthotopic Heart Transplantation: A 9-Year Single-Institution Experience

Objective

Over the past decade, antibody-mediated rejection (AMR) continues to be recognized as one of the major obstacles in cardiac transplantation, yet its clinical outcome has been reported only in small series studies. This investigation reviews our experience in treating 11 patients with AMR after heart transplantation.

Methods

We retrospectively analyzed a total of 11 patients who underwent cardiac transplantation from 2004 to 2012 at a single medical institute. The diagnosis of AMR was made according to criteria set by the International Society for Heart and Lung Transplantation (ISHLT) 2011 working formulation.

Results

The average age among the 11 patients was 50.4 ± 16.9 years. The overall mortality rate was 54.5%. Five patients (45.4%) developed hemodynamic compromise in an average of 5 days after transplantation, presenting with sudden onset of fatal arrhythmia (n = 4; 80%) and immediate heart failure (n = 1; 20%). All 5 patients underwent immediate resuscitation and extracorporeal membrane oxygenation (ECMO) support, and 3 patients died (60%); in contrast, the other 6 patients suffered from progressively worsening cardiac function during long-term follow-up. Three patients (50%) died in this group.

Conclusions

Clinical presentation of AMR varies. Long-term postoperative follow-up in the form of endomyocardial biopsy is recommended with immunohistochemistry C4d staining, with the anticipation of the possibility of future recurrence.     

Outcome After Myocardial Revascularization and Renal Transplantation: A 25-Year Single-Institution Experience

BACKGROUND AND OBJECTIVE: Cardiac disease is a common cause of death in renal transplant recipients. This study retrospectively analyzes the results of myocardial revascularization procedures in these patients and makes recommendations for managing coronary atherosclerosis in patients with renal disease who already have a transplanted kidney or who may receive a kidney transplant. METHODS: Patients who had myocardial revascularization (coronary artery bypass grafting [CABG] or percutaneous transluminal coronary angioplasty [PTCA]) and renal transplantation at the authors' institution between 1968 and 1994 were analyzed. Patient, procedural, and institutional variables were used for actuarial analyses of survival, as well as multivariate analyses of risk factors for death. RESULTS: Eighty-three of 2989 renal transplant patients required myocardial revascularization either before or after their transplant, and diabetes mellitus was the cause of renal failure in 42% of these patients. Standard coronary angiography, CABG, and PTCA techniques were used without periprocedural renal allograft loss. Actuarial patient survival was 89%, 77%, and 65% at 1, 3, and 5 years after the last procedure (transplantation or revascularization). Cardiac disease was the most common mode of death. Early-phase risk factors for death by multivariate analysis included hypertension and revascularization before 1989. Late-phase risk factors for death included diabetes mellitus, higher number of pre-CABG myocardial infarctions, renal transplantation before 1984, older age, and unstable angina before CABG. CONCLUSIONS: Myocardial revascularization can be performed with acceptable short- and long-term results in patients with renal disease who have renal transplantation either before or after the revascularization procedure. Diabetes mellitus was a highly prevalent condition among these patients, and cardiac disease was their most common mode of death. PTCA and CABG, as performed at this institution, posed little risk for renal allograft loss. Modification of risk factors for coronary atherosclerosis, rigorous cardiac evaluation of patients at risk for coronary artery disease before renal transplantation, and aggressive use of revascularization procedures to decrease the incidence of myocardial infarction are proposed as ways to prolong the survival of renal transplant patients with ischemic heart disease.     

Polycystic Kidney Disease and Cancer after Renal Transplantation

Autosomal dominant polycystic kidney disease (ADPKD), the most common form of polycystic kidney disease (PKD), is a disorder with characteristics of neoplasia. However, it is not known whether renal transplant recipients with PKD have an increased risk of cancer. Data from the Scientific Registry of Transplant Recipients, which contains information on all solid organ transplant recipients in the United States, were linked to 15 population-based cancer registries in the United States. For PKD recipients, we compared overall cancer risk with that in the general population. We also compared cancer incidence in PKD versus non-PKD renal transplant recipients using Poisson regression, and we determined incidence rate ratios (IRRs) adjusted for age, sex, race/ethnicity, dialysis duration, and time since transplantation. The study included 10,166 kidney recipients with PKD and 107,339 without PKD. Cancer incidence in PKD recipients was 1233.6 per 100,000 person-years, 48% higher than expected in the general population (standardized incidence ratio, 1.48; 95% confidence interval [95% CI], 1.37 to 1.60), whereas cancer incidence in non-PKD recipients was 1119.1 per 100,000 person-years. The unadjusted incidence was higher in PKD than in non-PKD recipients (IRR, 1.10; 95% CI, 1.01 to 1.20). However, PKD recipients were older (median age at transplantation, 51 years versus 45 years for non-PKD recipients), and after multivariable adjustment, cancer incidence was lower in PKD recipients than in others (IRR, 0.84; 95% CI, 0.77 to 0.91). The reason for the lower cancer risk in PKD recipients is not known but may relate to biologic characteristics of ADPKD or to cancer risk behaviors associated with ADPKD.Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of inherited cystic renal disease and the fourth most common cause of ESRD in the United States.^1–3 There are currently>16,000 individuals with polycystic kidney disease (PKD, of which ADPKD is by far the most common type) living with a renal transplant in the United States.⁴ADPKD is a result of mutations in one of two genes: PKD1 and PKD2.^1,5,6 These genes are widely expressed in many tissues, consistent with the multiorgan pathology characterizing ADPKD. A key factor in cyst formation and enlargement in ADPKD is the abnormal proliferation of cyst epithelial cells in a cell-autonomous manner.^7,8 This cyst formation is associated with cellular dedifferentiation and is considered a neoplastic process driven by upregulated proto-oncogenes.^9–13 While published case reports document the occurrence of renal cell carcinomas (RCCs) in ADPKD-affected kidneys,^14,15 these tumors may be partly due to acquired renal cystic disease resulting from long-term dialysis.¹⁶ Because there do not appear to be widespread published reports of other cancers in patients with ADPKD, protective mechanisms might exist in ADPKD to prevent malignant transformation. Indeed, many oncogenes that promote cell proliferation also act as potent growth suppressors (e.g., Ras¹⁷) or inducers of apoptosis (e.g., Myc^18,19). Thus, there is uncertainty whether ADPKD mutations are associated with increased rates of kidney cancer or cancer in general.We therefore designed a study to compare cancer risk in kidney transplant recipients with PKD versus kidney recipients with other causes of ESRD. Organ transplant recipients are at increased risk of cancer, largely because of immunosuppressive therapy.²⁰ An increased risk of cancer in patients with PKD might be detectable in this high-risk cancer population. Alternatively, if there is no increased risk of cancer in ADPKD, the findings would suggest the need for further study to determine whether protective cellular mechanisms may be at work.     

Liver Only Living Donor Transplantation for Polycystic Disease in a Patient on Chronic Hemodialysis: Case Report

BackgroundPolycystic liver disease (PLD) is characterized by the progressive development of polycystic lesions in the kidney and the liver, possibly resulting in dual organ failure. We indicated living donor liver transplantation (LDLT) for a patient with end-stage liver and kidney disease (ELKD) due to PLD on uncomplicated chronic hemodialysis.Case presentationA 63-year-old man with ELKD and uncontrolled massive ascites due to PLD and hepatitis B on uncomplicated chronic hemodialysis was referred to us with a single possible 47-year-old female living donor. Because of the necessity of right lobe liver procurement from this small middle-aged donor and uncomplicated hemodialysis on this recipient, we considered LDLT, rather than dual organ transplantation, could be the most well-balanced option to save the life of this recipient with acceptable risk limits for this donor. A right lobe graft with 0.91 for graft recipient weight ratio was implanted with an uneventful operative procedure under intra- and postoperative continuous hemodiafiltration. The recipient was rescheduled on routine hemodialysis on day 6 after transplantation and recovered with a gradual decrease in ascites output. He was discharged on day 56. He continues to have a very good liver function and quality of life without ascites and uncomplicated routine hemodialysis 1 year after transplantation. The living donor was discharged 3 weeks after surgery and is also doing well.ConclusionAlthough combined liver–kidney transplantation from a deceased donor could be the best option for ELKD due to PLD, LDLT can also be an acceptable option for ELKD with uncomplicated hemodialysis, considering the double equipoise theory for both lifesaving of the recipient and acceptable donor risk.     

Bone Disease and Liver Transplantation: A Review

Liver transplantation is currently the most effective and almost routine treatment for chronic and acute liver diseases. The survival of transplanted patients has increased exponentially, which has led to more knowledge of the long-term complications secondary to the underlying pathology or the various treatments that must be followed. Bone metabolic disease is a chronic complication of liver transplantation that inhibits quality of life. The factors that contribute to the development of bone disease are different according to the various etiologies of liver damage. All patients should be examined for osteoporosis risk factors because the incidence of new fractures in transplant patients is higher during the first year after transplantation, reflecting the greater bone loss during this time. This article outlines a proposal for a treatment algorithm; we propose that pharmacologic therapy in patients post liver transplant should first consider the diagnosis of osteoporosis by bone mineral density, the patient's personal and family history of spine and femoral neck fractures, and the use glucocorticoids (dose and time) until a tool is available that allows the best estimation of the fracture risk in this population of patients.     

Two Brothers With Renal and Hepatic Polycystic Disease Treated With Combined Liver and Kidney Transplantation: A Case Report

We report two brothers with renal and hepatic polycystic disease who developed end-stage renal failure, requiring hemodialysis, and organomegaly syndrome related to the gigantic size of the liver and both kidneys. Although there was no liver failure, combined liver and kidney transplantation was performed owing to worsening of the clinical condition. In both cases, successful transplantation was accomplished with intra-abdominal engraftment of the liver and kidneys through the same abdominal incision.     

成人型多囊肾与肾移植(附8例报告)

本文报告我院对8例成人型多囊肾施行同种异体肾移植的经验和体会。结果表明一年人/肾存活率为75％。讨论了①是否应该对这些病人施行肾移植?②术前肾切除对肾移植效果的影响;③多囊肾行肾移植后感染等并发症问题。     

ABO血型不合肝移植4例临床分析

目的探讨ABO血型不合肝移植的围手术期处理模式。方法回顾性分析2006年7月至2010年5月期间我院非紧急状态下开展的4例ABO血型不合的肝移植患者的临床资料。3例供体为AB型,受体为O型,均诊断乙肝肝硬变、肝功能失代偿,其中1例合并重症肝炎;1例供体为AB型,受体为A型,诊断为原发性肝癌合并乙肝后肝硬变、肝功能失代偿。结果 4例均存活至今(11~19个月),术后无感染、急性排斥反应发生。2例术后凝血功能障碍、渗血,其中1例二次探查;1例术后出现肾功能不全、少尿,给予床旁持续血浆滤过治疗后恢复正常。所有病例移植肝开放血流前2 h及术后第4天均给予巴利昔单抗20 mg处理,3例同时行脾切除术,4例术后给予1周以上的丙种球蛋白静脉滴注。血型抗体滴度术前1∶32的受体2例,术后第2天即下降并稳定于1∶8;术前1∶4的1例,术后1周内降至1∶2;术前1∶16的1例,术后第2~10周升高后自行降至1∶8。预防应用抗生素和免疫抑制方案与血型相符肝移植基本相同,停用激素时间延迟3个月。结论在供肝紧缺情况下,通过完善围手术期处理,包括IL-2受体单抗、人丙种球蛋白的使用及合理的术后免疫抑制剂方案,结合脾切除术等措施,进行ABO血型不合的肝移植是可行的。     

Experience With Autosomal Dominant Polycystic Kidney Disease in Patients Before and After Renal Transplantation: A 7-Year Observation

Objective

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the presence of multiple cysts in both kidneys. Symptoms of the disease may arise either from the presence of cysts or from increasing loss of kidney function. First symptoms usually appear in the third decade of life: lumbar pain, urinary tract infections, arterial hypertension, or renal colic due to cyst rupture or coexistent nephrolithiasis. An early diagnosis, male gender, large kidneys by sonography, arterial hypertension, hematuria, and urinary tract infections are predictive factors of a faster progression of the disease. Our aim was to establish the indications for nephrectomy among symptomatic ADPKD patients before kidney transplantation and to assess the risks of posttransplantation complications among ADPKD patients without nephrectomy.

Patients and Methods

The observed group consisted of 183 patients with ADPKD among whom 50 (27.3%) underwent kidney transplantation during a 7-year observation period (2000-2007). Among those subjects were 3 groups: (I) nephrectomy preceding transplantation; (II) nephrectomy during kidney transplantation; and (III) without nephrectomy.

Results

Among group I before transplantation we observed: arterial hemorrhage, wound infections, and splenectomy 4 weeks after ADPKD nephrectomy; afterward we observed: urinary tract infections and contralateral cyst infection. Among group II we only observed 1 case of wound infection. Among group III we observed: ascending urinary tract infections, cyst infections, and cyst hemorrhage. Cyst hemorrhage and cyst infections led mainly to ADPKD kidney nephrectomy. During the observation time, 80.95% of grafts were functioning.

Conclusions

Unilateral nephrectomy is a well-founded preliminary surgical treatment before kidney transplantation. Bilateral nephrectomy before or during transplantation eliminates ADPKD complications and does not significantly increase general complications. The greatest numbers of complications and of graft losses were observed among the group without pretransplantation nephrectomy.     

Cutaneous Angiosarcoma: A Single-Institution Experience

Background

Cutaneous angiosarcoma (CAS) is a rare, aggressive vascular sarcoma with a poor prognosis, historically associated with 5-year overall survival (OS) rates between 10 and 30 %.

Methods

This is a single-institution retrospective review of patients treated for CAS from 1999–2011. Demographics, primary tumor characteristics, treatment, and outcomes were analyzed.

Results

A total of 88 patients were identified (median age 70 years and 57 % female). Median tumor size was 3 cm. Median follow-up was 22 months. The 5-year OS and recurrence-free survival (RFS) were 35.2 and 32.3 %, respectively; median was 22.1 months. Also, 36 patients (41 %) received surgery alone, 7 (8 %) received XRT alone, and 41 (47 %) received surgery and XRT. Of the 67 of 88 patients who were disease-free after treatment, 33 (50 %) recurred (median of 12.3 months). Surgery alone had the highest 5-year OS (46.9 %) and RFS (39.9 %) (p = ns). Four presentation groups were identified: (1) XRT-induced, n = 30 (34 %), 26 of 30 occurred in females with a prior breast cancer, (2) sporadic CAS on head and neck (H/N), n = 38, (3) sporadic CAS on trunk/extremities, n = 13, and (4) Stewart–Treves n = 7. Those with trunk/extremity CAS had the highest 5-year OS (64.8 %), with H/N CAS having the worst 5-year OS (21.5 %). On MV analysis, only tumor size <5 cm correlated with improved OS (p = 0.014).

Discussion

In this large series, there appears to be a better overall prognosis than historically reported, especially in Stewart–Treves and CAS on trunk or extremities. While surgery alone was associated with better OS and RFS compared with other treatment modalities, this was not statistically significant. Tumor size was a significant prognostic factor for OS.     

Hepatocellular Carcinoma and Liver Transplantation: A 12-Year Experience

Background

Orthotopic liver transplantation (OLT) for patients with cirrhosis and concomitant hepatocellular carcinoma (HCC) in early stages is the treatment of choice, with an acceptable recurrence rate and excellent survival.

Aim

We sought to evaluate (1) the accuracy of preoperative imaging; (2) the impact of pre-OLT treatments on survival and recurrence; and (3) the influence of beyond Milan criteria selection on global outcomes.

Methods

We studied a cohort of 65 patients with HCC among 300 consecutive OLTs over a single 12-year experience. We analyzed the overall outcomes of survival and recurrence, the accuracy of preoperative diagnosis and staging the influence of neoadjuvant treatment prior to OLT, and the effect on overall outcomes beyond the Milan criteria in our series.

Results

The 65 transplants were performed for HCC, mostly in association with hepatitis C virus and alcoholic cirrhosis with HTP. At a mean follow-up of 40.32 months, the recurrence rate was 5.7% among the 61 HCC confirmed by histopathology. The overall survival was 30.07. Actuarial survivals at 1, 5, and 10 years were 82%, 77%, and 62%, respectively. Six retransplants occurred among the seven graft losses albeit with poor survival after the second graft. Most explants showed low pTNM stages with favorable microscopic features. Preoperative imaging tests failed to achieve an accurate diagnosis in 15.38% of the series. The role of alpha-fetoprotein (AFP) and hepatic biopsy was irrelevant. Unfavorable histopathologic factors predicted a greater recurrence rate, but had no influence on survival. Neither recurrence nor survival were modified by pre-OLT therapy.

Conclusions

In our series, AFP, hepatic biopsy, and pre-OLT treatment had limited roles. Radiological imaging techniques underestimated HCC staging and lead to a misdiagnosis to an expected degree. Despite these findings, this single institution experience with OLT for HCC showed excellent survivals with a low recurrence rate including cases of patients beyond the Milan criteria.     

A Case Report on Successful Liver Transplantation from a Donor with Polycystic Liver

Organ shortage is a major issue faced by both transplant surgeons and patients. To enlarge the donor pool, marginal liver grafts are being increasingly used. In this paper, we present the case of a patient in whom a polycystic liver was successfully transplanted from a donor after brain and cardiac death. To our knowledge, this is the first successful case of liver transplantation using polycystic liver from a donation after brain and cardiac death.     

异位辅助性分肝移植供肝切取与植入体会

目的　为临床辅助性肝移植的开展,探索供肝切取及植入方法。　方法　猪异位辅助性部分肝移植13例,供肝切取按预分离方法分为A、B两组,A组标准法5例,B组快速灌注法8例。　结果　A组供肝质量优良为100%,B组供肝质量优良占88%。辅肝移植手术时间为122±28min,腔静脉和门静脉吻合时间为40±12min。　结论　如条件允许应首选标准法预分离,血流动力学不稳定时,可行快速灌注法预分离。     

Liver Transplantation With Old Grafts: A Ten-Year Experience

Background

The persistent scarcity of donors has prompted liver transplantation teams to find solutions for increasing graft availability. We report our experience of liver transplantations performed with grafts from older donors, specifically over 70 and 80 years old.