Transcatheter renal artery embolization improves lung function in patients with autosomal dominant polycystic kidney disease on hemodialysis

Background

Since 1996, transcatheter renal artery embolization (renal TAE) has been performed to reduce the volume of the kidneys in patients with autosomal dominant polycystic kidney disease (ADPKD) and complications of nephromegaly at our hospital. Respiratory dysfunction is often a serious problem in these patients before TAE.

Patients and methods

Between January 2006 and October 2008, renal TAE was performed and lung function testing [percent vital capacity (%VC) and percent forced expiratory volume in 1?s (%FEV_1.0)] was done by spirometry in 28 patients on maintenance hemodialysis who had respiratory symptoms.

Results

Renal volume was 6,330.5?±?3,126.5?cm³ (range 1,771–12,761?cm³) before TAE, and decreased significantly to 2,892.2?±?1,841.7?cm³ (range 622–6,961?cm³) by 12?months after TAE (p?=?0.0001). The percent decrease of renal volume at 12?months after TAE versus baseline was 45.6?±?14.6% (range 6.6–67.3%). %VC showed a significant increase from 95.9?±?14.8% (range 63–127%) before renal TAE to 100.1?±?11.7% (range 78–120%) at 12?months after TAE (p?<?0.01). %FEV_1.0 was also significantly increased from 87.9?±?15.0% (range 55–110%) before renal TAE to 92.5?±?14.4% (range 58.0–115.0%) at 12?months after TAE (p?<?0.01). The changes of VC (ΔVC%) and FEV_1.0 (ΔFEV_1.0%) both showed a significant positive correlation with the reduction of renal volume (Δ renal volume) (p?=?0.001 and p?=?0.004, respectively).

Conclusion

Since TAE not only led to a significant decrease of renal volume in ADPKD patients with nephromegaly, but also improved lung function (both %VC and %FEV_1.0), pulmonary dysfunction should be recognized as one of the extrarenal complications of ADPKD.     

Successful renal contraction therapy in polycystic kidney patient with renal transcatheter arterial embolization prior to ABO incompatible kidney transplantation

28-year-old female received dialysis treatment due to chronic renal failure caused by polycystic kidney disease. Later, she underwent a laparoscopic splenectomy and ABO incompatible living kidney transplantation successfully following bilateral renal contraction therapy with renal transcatheter arterial embolization (renal TAE). A unilateral or bilateral native nephrectomy of a massively enlarged kidney performed at the time of renal transplantation is a common treatment in polycystic kidney patients scheduled for transplantation. On the other hand, when treated with renal TAE, such patients can avoid a laparotomy, which provides several advantages when undergoing peritoneal dialysis in the future or a laparoscopic splenectomy prior to ABO incompatible kidney transplantation. Furthermore, we consider that bilateral renal TAE is necessary for polycystic kidney patients prior to renal transplantation for a variety of reasons, including problems associated with contrast nephropathy if renal TAE for left kidney is remained after renal transplantation.     

Acquired renal cystic disease mimicking adult polycystic kidney disease in a patient undergoing long-term hemodialysis.

Acquired renal cystic disease (ARCD) is defined as the development of multiple cysts in the renal cortex and medulla in patients with chronic renal failure who are free from congenital polycystic kidney disease. ARCD develops generally in contracted kidneys. We report a case of grotesque enlargement of a single kidney in a patient who had been receiving hemodialysis for 18 years. Although the exact causes of ARCD are not known, 3 factors may contribute to the development of nephromegaly; the sex, the duration of hemodialysis and previous unilateral nephrectomy. As in polycystic disease, when the involved kidney reaches considerable size, ARCD may have a favorable effect on anemia caused by chronic renal failure.     

无水乙醇肾动脉栓塞治疗肾癌

目的 减少肾癌术中出血以及提高姑治疗晚期肾癌疗效。方法 采用Ｓｅｌｄｉｎｇｅｒ技术用无水乙醇行２５例吕肾动脉栓塞术。其中行根治性肾切除术２０例,肾切除４例。结果 栓塞术后行肾癌根治术２０例,单纯肾切除术４例,１例未手术。平均出血量２１０ｍｌ,无死亡及严重并发症,但均有不同程度腰痛及发热。结论 无水乙醇行肾动脉栓塞,方法简单,效果显著,副作用轻,是可行的辅助治疗方法。     

Thoracic aortic dissection in a patient with autosomal dominant polycystic kidney disease treated with maintenance hemodialysis

A patient with autosomal dominant polycystic kidney disease (ADPKD) on maintenance hemodialysis (HD) experienced spreading back pain with a sudden onset, and was diagnosed with thoracic aortic dissection. Reports of ADPKD with aortic dissection are rare. Hypertension, which is essentially universal both among ADPKD and hemodialysis patients, is a known risk factor for aortic dissection. Additionally, some reports have indicated that patients with ADPKD have aortic fragility. We suspect that aortic dissection may be less rare than presently apparent among HD patients with ADPKD.     

Transitional cell carcinoma of the renal pelvis in a long-term hemodialysis patient with autosomal dominant polycystic kidney

We present a case of renal pelvic transitional cell carcinoma (TCC) accompanied by autosomal dominant polycystic kidney disease (ADPKD). An 81-year-old woman on long-term hemodialysis for ADPKD visited the Department of Urology at Kouseiren Takaoko Hospital, complaining of asymptomatic macroscopic hematuria. Retrograde pyelogram showed an irregular filling defect in the right renal pelvis, which suggested the right renal pelvic tumor. We performed right nephrectomy and transurethral removal of the right ureter. Histological diagnosis demonstrated renal pelvic TCC.     

Hyperbaric oxygen therapy in a patient with autosomal dominant polycystic kidney disease with a perinephritic abscess

A 68-year-old female in hemodialysis due to autosomal dominant polycystic kidney disease underwent resection of cysts in her right kidney via a laparoscopic approach due to abdominal pain. Three weeks after surgery, she was admitted with sepsis. A CT scan showed a large abscess around the right kidney. Percutaneous drainage of abscess was performed. The pus smear showed Gram-positive cocci and the culture contained methicillin-resistant Staphylococcus aureus. Ciprofloxacin, clindamycin and vancomycin were given. In the 3 weeks following admission, she remained febrile and septic and showed a progressive deterioration in her general condition, along with malnutrition and persistent drainage of purulent material from her right flank. The antibiotic therapy was changed to vancomycin, metronidazole and meropenem, but no improvement was observed. Because of the high risk associated with carrying out an open nephrectomy, we decided to use hyperbaric oxygen therapy (HBOT) as a potentially useful measure to control her infection. The patient underwent 26 daily sessions of 100% hyperbaric oxygen (2.5 atm). The use of HBOT induced a notable break in the clinical course of this patient’s retroperitoneal infection. She was discharged after day 58 without any signs of inflammatory activity, and she has not presented reactivation of infection since then. We think that this case suggests that this therapy could be a new therapeutic tool in the management of patients with similar clinical features when all other therapeutic measures have failed. We did not find any other reports of the use of HBOT in infections of renal cysts.     

Perinephric abscess in patients with polycystic kidney disease undergoing chronic hemodialysis.

5 patients with polycystic kidney disease undergoing chronic hemodialysis who developed perinephric abscesses are described. Gallium-67 scintigraphy was helpful in making a diagnosis in 2 of these patients. All 5 patients initially presented with urinary tract infections. Perinephric abscess became evident over a variable period of time (2--28 days) following completion of antibiotic therapy for their urinary tract infection. Gallium-67 scintigraphy appears useful in detecting this complication, and nephrectomy should be considered once the diagnosis is confirmed.     

Arachnoid cyst in a patient with autosomal-dominant polycystic kidney disease

We report a case of an arachnoid cyst of the posterior cerebral fossa in a man with autosomal-dominant polycystic kidney disease. No traumatic or infectious etiology for the cyst could be identified.     

Multiple intracranial aneurysms in a patient with autosomal recessive polycystic kidney disease.

Autosomal recessive polycystic kidney disease (ARPKD) is usually characterized by early onset chronic renal failure due to innumerable dilated collecting ducts. Hepatic fibrosis is an obligate sign. Here, for the first time, we report a 31-year-old female with ARPKD who was diagnosed with symptomatic multiple intracranial aneurysms, a manifestation previously only known to be associated with autosomal dominant polycystic kidney disease (ADPKD).     

Successful pregnancy in a patient with polycystic kidney disease and advanced renal failure without prophylactic dialysis

Pregnancies in women suffering from advanced chronic renal failure are frequently associated with deterioration of maternal renal function, premature births and low birth weights. Prophylactic dialysis is sometimes instituted since this intervention ameliorates the uremic milieu and improves maternal status and fetal uterine environment. This report describes a successful pregnancy and delivery in a hypertensive woman with advanced chronic renal failure due to polycystic kidney disease without accelerating the natural deterioration of renal function and without instituting prophylactic dialysis. The infant was delivered at full term with a normal birth weight. Thirty months after delivery, growth and development of the child were normal and the rate of deterioration of maternal renal function, assessed by 1/creatinine, was unaffected by pregnancy. Conservative management and effective control of blood pressure may be sufficient to achieve successful pregnancy outcome when women with advanced chronic renal failure become pregnant.     

Transcatheter arterial embolization in patients with renal arteriovenous malformation: a report of two cases

We report two cases of renal arteriovenous malformation (AVM) with chief complaints of macroscopic hematuria, and treated with transcatheter arterial embolization (TAE) using Gelfoam. Although hematuria disappeared after TAE in both cases, recanalization of AVM was revealed by follow-up renal arteriography one and three months later respectively. TAE were repeatedly performed at the same time. At the follow-up, the partial infarction in the normal parenchyma of the treated kidney was demonstrated on CT scanning in one case. We suggest that if the residual or recanalization of AVM after the initial TAE is observed, careful follow-up should be preferred without repeated TAE unless some deriving symptoms have appeared.