Resultados del trasplante renal en portadores de ríñones poliquísticos / Outcome of renal transplantation in patients with autosomal dominant polycystic kidney disease

Background: Patients with autosomal dominant polycystic kidney disease (ADPKD) have a better survival in chronic dialysis than patients with other etiologies of renal failure. It has been suggested that extra-renal manifestations of ADPKD may increase the odds of complications and mortality, if these patients are transplanted. Aim: To determine whether survival and complications in transplanted patients with ADPKD are different from kidney graft recipients with other etiologies of renal failure. Subjects and Methods: Four hundred six patients with kidney transplantation were followed in three hospitals between 1976 and 2011 and 19 were carriers of ADPKD. The latter were matched by type of donor, gender, age and date of kidney transplant, with 38 graft recipients with other etiologies of renal failure. Results: Graft and patient 1, 5, 10 and 15 years survival were similar in both groups. Hospitalizations due to viral infections and sepsis were more common in patients with ADPKD. There were no differences in the rate of acute rejection, delayed graft function, cancer, gastrointestinal disorders and hospitalizations due to cardiovascular diseases. The frequency of graft loss due to death with a functioning kidney was similar between both groups. Conclusions: Patient and graft survival in transplanted patients with ADPKD were similar to patients with other etiologies of renal failure. The rate and type of complications were similar between groups with the exception of hospitalizations due to sepsis and viral infections, which were more common in ADPKD patients.

Cyst Ablation Using a Mixture of N-Butyl Cyanoacrylate and Iodized Oil in Patients with Autosomal Dominant Polycystic Kidney Disease: the Long-Term Results

OBJECTIVE: We wanted to assess the long-term results of cyst ablation with using N-butyl cyanoacrylate (NBCA) and iodized oil in patients with autosomal dominant polycystic kidney disease (ADPKD) and symptomatic cysts. MATERIALS AND METHODS:Cyst ablation using a mixture of NBCA and iodized oil was performed in 99 cysts from 21 patients who had such symptoms as abdominal distension and pain. The collapse or reaccumulation of the ablated cysts after the procedure was assessed during the follow-up period of 36 to 90 months. The treatment effects, including symptom relief, and the clinical data such as the blood pressure and serum creatinine levels were also assessed, together with the complications. RESULTS: The procedure was technically successful in all 99 cysts from the 21 patients. Any procedure-related significant complications were not detected. Seventy-seven of 99 cysts (78%) were successfully collapsed on the follow-up CT. Twenty-two cysts showed reaccumulation during long-term follow-up period. The clinical symptoms were relieved in 17 of the 21 patients (76%). Four of 12 patients (33%) with hypertension and two of six patients (33%) with azotemia were improved. End stage renal disease (ESRD) occurred in six of the 21 patients (28%) during the follow-up period. The mean age of ESRD in our patients was 57 years. The mean time interval for the development of ESRD was 19 months. CONCLUSION: Ablation using a mixture of NBCA and iodized oil may be an effective, safe method for obtaining symptom relief in patients with ADPKD.

Bilateral nephrectomy of huge polycystic kidneys associated with a rectus abdominis diastasis and umbilical hernia / Nefrectomia bilateral para doença renal policística gigante associada à diástase do reto abdominal e hérnia umbilical

BACKGROUND: Patients with end-stage renal failure due to huge autosomal dominant polycystic kidney disease usually have an umbilical hernia and rectus abdominis diastasis, which are very troublesome. Pretransplant bilateral nephrectomy techniques does not manage the umbilical hernia and rectus abdominis diastasis. We report our experience in performing bilateral nephrectomy and repairing the rectus abdominis diastasis and umbilical hernia through the one, small incision. METHODS: Four patients aged 37 to 43 years with huge polycystic kidneys, an umbilical hernia, and a rectus abdominis diastasis underwent bilateral pretransplant nephrectomy through a midline supraumbilical incision including the umbilical hernia defect. The kidneys were removed through this incision. The incision was closed with the transposition of rectus abdominis muscle, pants-over-vest-style, to correct the diastasis and the umbilical hernia. RESULTS: The average operative time was 160 minutes (range, 130-180); the average larger kidney size was 33 cm (range, 32-34 cm); no major complications occurred; one patient who had preoperative low hemoglobin required blood transfusion. Patients were discharged from the hospital on postoperative day 7 with an esthetically pleasing belly, no rectus abdominis diastasis, and no umbilical hernia. One to two months after bilateral nephrectomy, the patients received a live donor kidney with an uneventful outcome. CONCLUSION: A midline supraumbilical incision is an excellent approach for bilateral nephrectomy of huge polycystic kidneys. In addition, an umbilical hernia and rectus abdominis diastasis may be successfully repaired through same incision with good cosmetic results.

INTRODUÇÃO: Pacientes com insuficiência renal terminal por Doença Renal Policística Autossômica Dominante geralmente apresentam hérnia umbilical e diástase de músculo reto abdominal, que são muito problemáticas. Técnicas de nefrectomia bilateral pré-transplante não dão atenção à hérnia umbilical e à diástase do músculo reto abdominal. Relatamos nossa experiência com nefrectomia bilateral e correção da diastase de músculo reto abdominal e hérnia umbilical através de uma única pequena incisão. MÉTODOS: Quatro pacientes com idade entre 37 a 43 anos com Doença Renal Policística Autossômica Dominante gigante, hérnia umbilical e diástase do múculo reto abdominal foram submetidos à nefrectomia bilateral pré-transplante através de incisão mediana supra-umbilical incluindo o defeito herniário umbilical. Os rins foram removidos através da pequena incisão mediana. A incisão foi fechada com transposição do músculo reto abdominal tipo jaquetão para corrigir a diastase e a hernia umbilical. RESULTADOS: O tempo operatório médio foi 160 minutos (130-180); o tamanho médio do maior rim foi 33cm (32-34); não ocorreram grandes complicações; um paciente, que tinha baixo nível de hemoglobina pré-operatório e precisou de transfusão sangüínea. Pacientes receberam alta hospitalar no 7° pós-operatório com abdome de boa aparência, sem diástase de músculo reto abdominal e sem hérnia umbilical. Os pacientes receberam enxerto renal de doador vivo um ou dois meses após a nefrectomia bilateral, sem intercorrências. CONCLUSÃO: A incisão mediana supra-umbilical é uma abordagem excelente para nefrectomia bilateral de rins policísticos gigantes. Além disso, a hernia umbilical e a diastase de músculo reto abdominal podem ser corrigidas com sucesso pela mesma incisão, com bons resultados cosméticos.