Combined liver-kidney transplantation in polycystic disease: case reports

Polycystic disease causes a progressive decrease in renal function and liver degeneration. The progression of the disease evolves separately between organs and transplantation options vary: simultaneous or sequential liver-kidney transplantation or single-organ transplantation. From September 2006 to June 2007 3 combined liver kidney transplantations (CLKT) were performed for polycystic disease with end-stage renal disease: 2 with polycystic liver disease, and 1 with hepatic failure due to congenital hepatic fibrosis. The widest dimensions of the polycystic liver of 50 and 60 cm diameter were due to extensive cystic degeneration. We performed 1 simultaneous CLKT and 2 sequential transplantations: 1 liver after kidney, and 1 kidney after liver. At present all patients are alive with 100% graft function. Median creatinine level at discharge was 0.9 mg/dL (ranges, +/-0.2). Good liver graft function was reported in all 3 cases. Transplant benefit in polycystic liver-kidney disease has been already demonstrated; conservative surgical options may result in a high incidence of complications in highly involved polycystic livers. Delaying transplantation results in a more difficult surgical technique, a higher rate of postoperative complications, and a disturbance of optimal graft retrieval because of the worse preoperative condition of the patients.     

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.     

肾移植治疗常染色体显性遗传性多囊肾病患者的临床效果分析（附43例报告）

目的探讨肾移植治疗常染色体显性遗传性多囊肾病（多囊肾）患者的疗效。方法多囊肾患者43例（多囊肾组）,在不切除原双侧肾脏的前提下,进行肾移植,以同期50例原发病为非多囊肾的肾移植患者作为对照组,进行随访研究。比较两组的术后1、3、5年人、肾存活率及排斥反应发生情况,通过肾脏B超检查多囊肾组患者术前与术后移植肾的体积变化,记录多囊肾组的并发症发生情况。结果多囊肾组肾移植术后1、3、5年人存活率分别为95.3%、90.6%、90.6%,术后1、3、5年肾存活率分别为95.3%、88.3%、83.7%。对照组相应为96.0%、92.0%、90.0%,94.0%、92.0%、88.0%,两组比较差异无统计学意义（P〉0.05）。两组的急性排斥反应发生率比较差异亦无统计学意义（P〉0.05）。多囊肾组术后3～6个月原肾明显缩小,1年后体积基本稳定,跟踪观察1～15年肾脏体积变化不明显。移植后血尿逐渐减轻,7～10d后消失。12例在移植后5～10周反复出现肉眼血尿,均经抗感染治疗后消失。多囊肾患者移植后仍需要应用药物控制血压。多囊肾组尿路感染发生率高达40%。32例多囊肾合并多囊肝,术后发生肝功能损害7例。结论多囊肾患者采用不切除原肾的肾移植效果满意,移植后应严密观察患者移植物肾功能、血尿和感染情况,及时对症处理。     

Outcome and quality of life in patients with polycystic liver disease after liver or combined liver-kidney transplantation.

In advanced stages of polycystic liver disease, often associated with polycystic kidney disease, a curative therapy is liver or combined liver-kidney transplantation. However, little is known about long-term outcome and quality of life. Between 1990 and 2003, 36 patients (32 female, 4 male) with polycystic liver or combined liver-kidney disease underwent liver (n = 21) or liver-kidney (n = 15) transplantation at our center. Main indications for liver transplantation were cachexia, muscle atrophy, loss of weight, recurrent cyst infections, portal hypertension, and ascites. Apart from clinical parameters, 2 anonymous questionnaires (standard short form 36 and self-designed) addressing quality of life and social status were evaluated. Five patients (14 %) died due to sepsis or myocardial infarction with pneumonia, all within 61 days after transplantation. The follow-up time of the remaining 31 patients ranged from 5 to 156 months, with a mean of 62 months. Of the 23 (74%) answered the questionnaires, 91% of patients felt "much better" or "better," only 9% felt "worse" than before, and 52% of patients participated in sports regularly. Fatigue, physical fitness, loss of appetite, and vomiting improved significantly after transplantation. Physical attractiveness and interest in sex increased as well. Professional occupation did not change for 71% of patients. Family situation before and after transplantation changed in 1 case only. Finally, 78% of patients said they would opt for transplantation again, while 17% were undecided; 1 patient would not repeat transplantation. In conclusion, patients with advanced polycystic liver or polycystic liver-kidney disease have an excellent survival rate and an improved quality of life after liver or combined liver-kidney transplantation.     

Sequential Liver-Kidney Transplantation for Recurrent Liver Cysts Infection in a Patient With Autosomal Dominant Polycystic Kidney Disease: A Case Report

Autosomal dominant polycystic kidney disease (ADPKD) is the most prevalent hereditary kidney disorder. Liver cysts are the most common extrarenal manifestation of the disease and usually remain asymptomatic. Liver cyst infection is rare, and its treatment is challenging. Liver transplantation (LT) is the only curative therapeutic option in symptomatic polycystic liver disease associated with ADPKD. Only a few cases of LT for recurrent liver cyst infection have been published. To our knowledge, we report the first case of sequential liver-kidney transplantation for recurrent liver cysts infection in a patient with ADPKD. A 55-year-old woman with ADPKD who had a kidney transplantation (KT) presented with multiple liver cysts infection 9 months after her KT. These episodes started after biliary tract complications due to an ampullary adenoma necessitating multiple endoscopic interventions. Her general status gradually degraded because antibiotic treatment was not effective, and she underwent LT for recurrent liver cysts infection 1 year and 9 months after her KT. LT in this setting turned out to be challenging but was possible. We think that better biliary tract workup before KT may prompt better care in these patients.     

Native Kidney Function After Renal Transplantation Combined With Other Solid Organs in Preemptive Patients

Kidney transplantations combined with other solid organs are progressively increasing in number. There are no guidelines regarding the nephrologic indications for combined transplantations, namely liver-kidney (LKT), or heart-kidney (HKT), in preemptive patients with chronic kidney failure who are not on regular dialysis therapy. The objective of this study was to assess the functional contribution of the native kidneys after preemptive kidney transplantation combined with other solid organs. From 2004, 9 patients (aged 50.3 ± 8.5 years) with chronic kidney failure (creatinine 2.5 ± 1.0 mg/dL) caused by polycystic kidney disease (n = 4), vascular nephropathy (n = 2), interstitial nephropathy (n = 1), glomerulonephritis (n = 1), or end-stage kidney disease (n = 1), underwent combined transplantations (8 LKT, 1 HKT). A scintigraphic functional study (Tc-99DMSA or Tc-99mMAG3), was performed at 4 ± 3 months after transplantation to evaluate the functional contribution of both the native kidneys and the graft. All patients were given immunosuppressive drugs, including a calcineurin inhibitor (tacrolimus/or cyclosporine). At the time of scintigraphy, renal function in all patients was 1.3 ± 0.3 mg/dL. The functional contribution of the transplanted kidneys was on average 77 ± 18%. Only in 1 patient was the contribution of the graft <50%. At follow-up after 36 months, patient and kidney survivals were 100%. The study confirmed a high risk of loss of native kidney function in the presence of organic nephropathy. In light of our experience, a creatinine clearance <30 mL/min in an appropriate cutoff for a combined transplantation. Close clinical and instrumental assessment pretransplant is essential before proceeding with a combined transplant program to exclude functional forms and to optimize the use of organs.     

Results of combined and sequential liver-kidney transplantation

Experience with combined liver-kidney transplantation (L-KTx) has increased, but controversy regarding this procedure continues because the indications are not clearly defined yet. Between 1984 and 2000, 38 patients underwent simultaneous L-KTx and 9 patients underwent sequential transplantation, receiving either a liver before a kidney or a kidney before a liver. Main indications for a simultaneous procedure were polycystic liver-kidney disease with cirrhosis and coincidental renal failure. The main indications for sequential procedure were cirrhosis caused by viral infection for the liver and glomerulonephritis for the kidneys. Outcomes in these patients were evaluated retrospectively. Regarding simultaneous transplantation, 28 (73.7%) long-term survivors were followed up for 0.7 to 12.5 years. Currently, 24 (63.2%) patients are alive with good liver function. Fourteen patients died; 10 patients died in the early postoperative phase because of septic complications, and most of them were cirrhotic with a poor preoperative clinical status. Currently, 2 of the surviving patients (8%) have returned to dialysis, 4 (17%) have reduced renal function, and 18 (75%) have good renal function. Five liver and 2 kidney retransplantations were performed during the follow-up. In cases of sequential grafting, patients undergoing kidney transplantation in the presence of a previously transplanted stable liver did better than those who underwent liver transplantation after kidney transplantation. When liver transplantation was performed early and electively before substantial worsening, combined L-KTx is a safe procedure offering excellent long-term palliation. (Liver Transpl 2003;9:1067-1078.)     

Renal transplantation in patients with autosomal dominant polycystic kidney disease: pre-transplantation evaluation and follow-up

Autosomal dominant polycystic kidney disease (ADPKD) which accounts for 15% of all renal transplantations emerges as the third cause of kidney transplantation in France. In addition to routine evaluation before transplantation, the ADPKD patient requires special assessment of three aspects: should potential kidney complications (recurrent upper tract infection or haemorrhage) or kidney size assessed by computed tomography require nephrectomy prior to transplantation? Is it advisable to detect intracranial aneurysm (ICA) in patients with a relative having experienced ruptured ICA? When transplantation from a living relative is considered, the existence of ADPKD in the donor should be formally ruled out by imaging or genetic studies. The risk of recurrence of ADPKD post-transplantation does not exist. Nevertheless other complications may occur. Thus, an increased incidence of colonic perforation has been reported. In addition, as compared to non-ADPKD patients, an increased risk for both skin cancer and new-onset post-transplant diabetes mellitus has been reported recently after kidney transplantation. Finally, because these patients suffer from an inherited syndrome, physicians should carefully consider the personal and familial history before and after transplantation in order to respond to fatalism in some cases, or to attenuate excessive enthusiasm in the others. Altogether, it apears that a specific approach is needed for ADPKD patients when considering renal transplantation.     

Laparoscopic removal of renal cysts in patients with ADPKD as an alternative method of treatment and patient preparation for kidney transplantation: preliminary results

BACKGROUND: The most frequent genetic disease of the kidneys occurring in 1 of 1000 inhabitants is autosomal-dominant polycystic kidney disease (ADPKD). Growing renal cysts compress the kidney resulting in damage to parenchyma and functional disorders. Around 10% of these patients are dialyzed due to terminal renal insufficiency. With the advent of laparoscopic techniques, the idea of laparoscopic excision of cysts seemed a tempting alternative to nephrectomy. We assessed the preliminary results of laparoscopic treatment of polycystic kidneys compared with open nephrectomy for patients with ADPKD. MATERIALS AND METHODS: Thirty ADPKD patients were treated between 2000 and 2004. Eleven procedures in five men and six women of mean age 51 years included laparoscopic cyst excisions. In the remaining 19 patients (six men and 13 women) of mean age 54 years, nephrectomy was done. Indications for surgery included pain due to compression by large cysts and cyst contamination. Patients after nephrectomy were prepared for renal transplantation when necessary. RESULTS: Laparoscopic polycyst removal produced better effects than nephrectomy. Mean operative time was significantly shorter (86 minutes for cyst removal vs 108 minutes for nephrectomy; P < .05). Postoperative pain measured with the VAS scale was reduced in patients after laparoscopy. Hospital stay was shorter (5 vs 9 days), as well as time to recovery. Other benefits of laparoscopic cyst removal included maintained urination in the patient and no need for erythropoietin substitution, as well as reduced risk of cyst contamination. When eligible for renal transplantation, patients after laparoscopic polycyst removal have smaller kidneys that do not interfere with the graft and the risk of infection during immunosuppression seems lower. CONCLUSION: Although larger series of patients are required in patients with ADPKD, laparoscopic polycyst removal seemed superior to early nephrectomy.     

Autosomal dominant polycystic kidney disease in a kidney transplant population

AIM: To define specific manifestations of autosomal dominant polycystic kidney disease in kidney transplant patients. METHODS: Of 874 consecutive first renal transplant patients 1985-1993, 114 (13%) had autosomal dominant polycystic kidney disease (ADPKD). Mean age was 53 +/- 8 years, 62% were men, and 83% received cadaveric kidneys. Control patients were matched for sex, age and donor type. Median follow-up time was 63 months. One patient was lost to follow-up. Medical records before and after transplantation were reviewed. RESULTS: Survival of patients and grafts was similar in ADPKD patients and controls. Twenty- five ADPKD patients died, four of causes not seen in the controls; two aortic aneurysms, one urothelial cancer, one colon perforation. Four more ADPKD patients but no control had diverticulitis (P = 0.03), two with perforation. Cardiovascular morbidity was not increased. Eight patients had subarachnoidal haemorrhage before transplantation and two during follow-up. Nineteen patients had undergone nephrectomy before transplantation, 11 because of voluminous kidneys, five for infection, pain or bleeding, two for suspected malignancy, one for hypertension. After transplantation, seven patients underwent nephrectomy, only one related to kidney size. During the first year, need of phlebotomy occurred in 14% of patients versus 4% of controls, P = 0.02. Urinary tract infection rates were not increased. No morbidity was related to liver cysts. CONCLUSION: The specific features of kidney transplantation to patients with ADPKD were few: enlarged kidneys, relevant only before transplantation, erythrocytosis, and as rare but serious events, diverticulitis with perforation.      

New onset diabetes after kidney transplantation in autosomal dominant polycystic kidney disease: a retrospective cohort study

Background: New onset diabetes after transplantation (NODAT) is a common adverse outcome of organ transplantation that increases the risk of cardiovascular disease, infection and graft rejection. In kidney transplantation, apart from traditional risk factors, autosomal dominant polycystic kidney disease (ADPKD) has also been reported by several authors as a predisposing factor to the development of NODAT, but any rationale for an association between ADPKD and NODAT is unclear. We examined the cumulative incidence of NODAT in or own transplant population comparing ADPKD patients with non‐ADPKD controls. Methods: A retrospective cohort study to determine the cumulative incidence of patients developing NODAT (defined by World Health Organization‐based criteria and/or use of hypoglycaemic medication) was conducted in 79 patients with ADPKD (79 transplants) and 423 non‐ADPKD controls (426 transplants) selected from 613 sequential transplant recipients over 8 years. Patients with pre‐existing diabetes as a primary disease or comorbidity and/or with minimal follow up or early graft loss/death were excluded. Results: Of the 502 patients (505 transplants) studied, 86 (17.0%) developed NODAT. There was no significant difference in the cumulative incidence of NODAT in the ADPKD (16.5%; CI 13.6–20.7%) compared with the non‐ADPKD (17.1%; CI 8.3–24.6%) control group. Of the 13 patients in the ADPKD group with NODAT, three required treatment with insulin with or without oral hypoglycaemic agents. Among the 73 NODAT patients in the non‐ADPKD group, eight received insulin with or without oral hypoglycaemics. Furthermore, of the patients that did develop NODAT, there was no difference in the time to its development in patients with and without ADPKD Conclusion: There was no evidence of an increased incidence of NODAT in ADPKD kidney transplant recipients.     

Polyomavirus BK replication in adult polycystic kidney disease post-renal transplant patients and possible role of cellular permissivity

Cell division and differentiation but not proliferation seem to be necessary for BK virus (BKV) replication and reactivation of persistent infection. Only terminal differentiating cells are permissive to BKV replication. Renal tubular epithelial cells in human adult polycystic kidney disease (ADPKD) are characterized by increased proliferative activity leading to cyst growth with less cellular differentiation. The aim of this study was to evaluate the possibility of different BKV replication patterns in patients with polycystic kidney disease versus non-ADPKD patients. From May 2006 to April 2008, we performed renal transplantations in 47. Eleven patients were affected by ADPKD (Pc group) and 36 patients, non ADPKD (n-Pc group). BKV replication was evaluated by quantitative real-time polymerase chain reactions (PCR) on plasma and urine samples at 12 hours (T0/early) as well as 3 (T3) and 6 (T6) months after transplantation. BKV viremia occurred in 9%, 12.5%, and 20% among the Pc group versus 33.3%, 42.4%, and 50% among the n-Pc group at 12 hours as well as 3 and 6 months posttransplantation, respectively. A higher discordance (BKV-PCR blood −/urine +) was observed in plasma and urine BKV replication among Pc versus n-Pc groups. We hypothesized that the lower number of patients with active BKV plasma replication in the Pc group may be related to a lower cellular permissivity of the renal tubular epithelial cells in ADPKD.     

肝肾联合移植15例报道

目的探讨肝肾联合移植的适应证和疗效。方法对2001年2月至2003年12月施行肝肾联合移植术的15例患者进行了随访。15例中,乙型肝炎后肝硬化合并肝肾综合征8例、合并尿毒症2例、合并糖尿病肾病1例;多囊肝和多囊肾2例;Caroli病合并多囊肾1例;酒精性肝硬化合并尿毒症1例。对肝肾联合移植患者的手术方式,围手术期并发症,术后急、慢性排斥反应和乙型肝炎复发情况及随访结果进行了分析。结果15例肝肾联合移植术后移植物功能均恢复良好,6个月和1年生存率为100%。1例术前有严重营养不良者,术后给与48d的呼吸机支持后康复。术后创面出血和消化道出血各1例,经非手术治疗后治愈。胆道吻合口狭窄1例,用内镜下球囊扩张术治愈。1例术后2周发生急性移植肝排斥反应,给予激素冲击治疗后得到控制。1例术后30个月时因停用拉米夫定后乙型肝炎复发死于移植肝功能丧失。结论肝肾联合移植是终末期肝病合并慢性肾功能衰竭或肾功能损害的安全有效方法。对乙型肝炎患者术后尽早应用拉米夫定和乙型肝炎病毒免疫球蛋白预防肝炎复发。     

Trasplante renal y poliquistosis: consideraciones quirúrgicas

BackgroundThe indication and timing of nephrectomy in patients with autosomal dominant polycystic kidney disease (ADPKD) remain controversial, especially in patients who are candidates to renal transplantation (RT). The main surgical options such as unilateral vs. bilateral nephrectomy, nephrectomy before vs. after RT, or simultaneous nephrectomy and transplantation, are herein discussed.ObjectiveEvidence acquisition of the best surgical management available for ADPKD in the context of kidney transplantation.Acquisition of evidenceSystematic literature review in PubMed from 1978 to 2013 was conducted. Articles selected included:randomized controlled trials and cohort studies. Furthermore, well designed ADPKD reviews were considered for this study.Synthesis of evidenceLaparoscopic nephrectomy in ADPKD is a safe procedure with an acceptable complication rate. Unilateral nephrectomy has advantages over the bilateral one regarding theperioperative complication rate. Although the timing of nephrectomy is controversial, it seems that simultaneous nephrectomy and renal transplantation does not increase surgical morbidity neither affect graft survival.ConclusionsSimultaneous nephrectomy and RT appears to be an acceptable alternative to conventional two-stage procedure without any increased morbidity, in the context of ADPKD. Furthermore, laparoscopic nephrectomy performed in experienced centres is a safe alternative to conventional approach.     

Clinical aspects of renal transplantation in polycystic kidney disease

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) as a systemic disorder represents a special subgroup among patients with end-stage renal disease (ESRD). The different organ manifestations are potential risk factors for cardiovascular events or infections in the course after renal transplantation. Therefore, a long-term evaluation of ADPKD patients and of a control group was done. PATIENTS AND METHODS: 80 ADPKD patients were compared with 88 non-diabetic patients in a retrospective follow-up after renal transplantation. Patient and graft survival (1, 5 and 10 years after transplantation) as well as complications such as infections and cardiovascular events were evaluated. RESULTS: A comparable overall transplant (1 year, 5 years, 10 years: 83%, 73%, 67% ADPKD vs. 84%, 70%, 51% controls) and patient survival rate (1 year, 5 years, 10 years: 96%, 84%, 73% ADPKD vs. 91%, 79%, 58% controls) was found in both groups. Infectious complications with the exception of urinary tract infections (UTIs: ADPKD 42.5% vs. 26%) were diagnosed in similar frequency in the graft recipients. ADPKD patients were significantly more affected by UTIs than their control group (p < 0.05) and tended to suffer more often from lethal infections (ADPKD 7 vs. controls 3), but without statistical significance. Cardiovascular events were not observed to be significantly different between both groups (ADPKD 3 vs. controls 4). An obvious difference was found in patient (p < 0.01) and transplant survival rates (p < 0.05) of male and female ADPKD patients. The female group showed a significantly better outcome. CONCLUSIONS: The overall patient and graft survival rates did not differ between the ADPKD and control groups. The better outcome of female ADPKD graft recipients compared to the male group may be related to a gender-dependent disease severity, possibly due to hormonal effects. As UTIs and lethal septicemia were the leading complications in ADPKD patients, a careful monitoring for infections is important in the post-transplant follow-up.     

Sirolimus reduces polycystic liver volume in ADPKD patients

The immunosuppressive agent sirolimus exerts an antiproliferative effect by inhibiting mammalian target of rapamycin (mTOR). Because excessive proliferation of the biliary epithelium is a prominent feature of the polycystic liver that accompanies autosomal dominant polycystic kidney disease (ADPKD), we hypothesized that sirolimus may benefit patients with this disorder. We retrospectively measured the volumes of polycystic livers and kidneys in ADPKD patients who had received kidney transplants and had participated in a prospective randomized trial that compared a sirolimus-containing immunosuppression regimen to a tacrolimus-containing regimen. Sixteen subjects (seven with sirolimus, nine with tacrolimus) had received abdominal imaging studies within 11 mo before and at least 7 mo after transplantation, making them suitable for our analysis. Treatment with the sirolimus regimen for an average of 19.4 mo was associated with an 11.9 +/- 0.03% reduction in polycystic liver volume, whereas treatment with tacrolimus for a comparable duration was associated with a 14.1 +/- 0.09% increase. A trend toward a greater reduction in native kidney volume was also noted in the sirolimus group compared with the nonsirolimus group. Regarding mechanism, the epithelium that lines hepatic cysts exhibited markedly higher levels of phospho-AKT, phospho-ERK, phospho-mTOR, and the downstream effector phospho-S6rp compared with control biliary epithelium. In summary, treatment with sirolimus was associated with decreased polycystic liver volume, perhaps by preventing aberrant activation of mTOR in epithelial cells lining the cysts.     

Liver fibrosis in recessive multicystic kidney diseases: transient elastography for early detection

Cystic renal diseases are characterized by intrarenal cysts of different size and number. Further important diagnostic criteria include, e.g., liver fibrosis. The latter represents a significant cause of morbidity and mortality in autosomal-recessive polycystic kidney disease (ARPKD), whereas patients with autosomal-dominant polycystic kidney disease (ADPKD) can develop hepatic cysts without fibrosis. We report the use of transient elastography [FibroScan?, (FS)] for early and noninvasive detection of increased liver stiffness as marker of liver fibrosis. Compared with matched healthy controls, ADPKD patients (n?=?7) showed no significant difference in liver stiffness (5.3?kPa vs. 4.5?kPa; ns). ARPKD patients (n?=?7) had significantly increased median liver stiffness compared with controls (12.0?kPa vs. 4.5?kPa, p?=?0.002) and ADPKD patients (12.0?kPa vs. 5.3?kPa, p?=?0.002). Conventional ultrasound revealed evidence of liver fibrosis in only four of seven ARPKD patients (57%) compared with 100% detection by FS. Additional laboratory examinations showed no pathologic liver parameters. In conclusion, our data found FS to be a valuable, sensitive, and noninvasive new tool for early evaluation of liver fibrosis in cystic kidney diseases. This could facilitate diagnosis, monitoring, and management of liver involvement in ARPKD or any other cystic kidney disease.     

Renal replacement therapy in autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) accounted for 4.6% of our end-stage renal disease (ESRD) population. Initial ESRD therapy consisted of hemodialysis in 78% and continuous ambulatory peritoneal dialysis in 22% with significant intertherapy transfers. Half of these patients underwent one or more renal transplantations. Infections, primarily related to ADPKD or ESRD therapy, were the leading cause of morbidity in these patients. 3% of total time on ESRD therapy was spent in hospital, half of it due to problems related to ADPKD and ESRD therapy. Overall mortality and morbidity (as measured by hospitalization rates) in ADPKD patients were similar to those in a non diabetic ESRD population.     

Simultaneous liver-kidney transplantation for adult recipients with irreversible end-stage renal disease

HYPOTHESIS: Combined liver-kidney transplantation is safe (low morbidity and acceptable mortality) and effective in patients with end-stage liver disease. Although refinements in surgical technique have resulted in better patient and allograft outcomes, the negative impact of renal insufficiency on survival in patients undergoing liver transplantation has been widely reported, although some aspects are controversial. DESIGN: Analysis of the clinical characteristics and outcome in the management of patients undergoing combined liver-kidney transplantation. The end points were operative mortality, morbidity, and long-term survival. SETTING: University Hospital 12 de Octubre. PATIENTS: Between May 1986 and December 2001, 820 liver transplantations were performed. There were 16 cases (1.96%) of combined liver-kidney transplantations, which represent the sample of this study. RESULTS: Mean +/- SD follow-up of 42.2 +/- 29 months: 6 patients died (37.5% mortality rate). There were 4 (25%) hospital deaths within 6 months following surgery and 2 after 6 months (4 sepsis, 1 refractory heart failure, and 1 recurrent hepatitis C virus disease). Univariate analysis related to mortality included age, sex, etiology, preoperative creatinine level, United Network for Organ Sharing status, Child-Pugh score, type of hepatectomy (piggyback), intraoperative blood product administration, and the presence of postoperative complications. The only 2 significant factors were the presence of postoperative complications (P = .01) and the United Network for Organ Sharing status (P = .02). Crude survival rate was 62.5%. Actuarial survival rates were 80%, 71%, and 60% at 1, 3, and 5 years, respectively. CONCLUSION: Because end-stage renal disease is not a formal contraindication for liver transplantation, a combined liver-kidney transplantation for adults with end-stage renal disease can be done safely and effectively.     

COMBINED LIVER-KIDNEY TRANSPLANTATION: EXPERIENCE AT A BRAZILIAN UNIVERSITY HOSPITAL

Background

Combined liver-kidney transplant is a routine procedure in many transplant centers. The increase in its number coincided with the introduction in 2002 of the MELD (Model for End-stage Liver Disease) score for allocation of livers, prioritizing patients with renal dysfunction.

Aim

To analyze the experience with combined liver-kidney transplantation in a liver transplant center in Brazil.

Method

A retrospective review was conducted. All transplants were performed using grafts from deceased donors.

Results

Sixteen combined liver-kidney transplantations were performed in the same period, which corresponds to 2.7% and 2.5% of the kidney and liver transplants, respectively. Fourteen patients were male (87.5 %) and two were female (12.5%). The average patients and donors age was 57.3±9.1 and 32.7±13.1, respectively. The MELD score mean was 23.6±3.67. The main cause of liver dysfunction were chronic hepatitis C virus (n=9). As for renal dysfunction, diabetic nephropathy (n=4) was the most frequent. There were six deaths, two of them by severe dysfunction of the liver graft and four by infectious causes. The 1, 3 and 5 years survival rate in patients undergoing liver-kidney transplantations was 68.8%, 57.3% and 57.3%, respectively.

Conclusion

The survival rates achieved in this series are considered satisfactory and show that this procedure has an acceptable morbidity and survival.