A case of polycystic kidney with bilateral nephrectomy

A 56-year-old woman in chronic hemodialysis had been suffering from uncontrollable fever for the past 7 months. Her original disease was diagnosed as familial polycystic kidney and three of her five brothers were found to have the same disease. Her chromosome was 46,XX,21P+ and laboratory examination revealed severe anemia, malnutrition, liver dysfunction, pyuria and candidiasis of urine. Abdominal echogram and CT scan revealed polycystic kidneys and multiple liver cysts. She was admitted to our hospital and was diagnosed as having pyelonephritis of the right kidney. As her condition was not improved by conservative therapy right nephrectomy was performed. One month later, spiking fever and left tenderness reappeared. Those symptoms could not be controlled by conservative therapy and left nephrectomy was performed again. Pathological examination on nephrectomized kidneys showed interstitial nephritis, hyaline degeneration and proliferative change of glomeruli, microabscess, colloid of tubules and calcification of parts of Henle's loops. Nephrectomy has been performed in 1.6 to 10.0% of polycystic kidneys due to references since 1952. Eight of the 22 polycystic kidneys (36.3%) seen at our hospital during the past 10 years have been removed.     

A case of angiomyolipoma originating from polycystic kidney with horseshoe kidney

A case of renal angiomyolipoma originating from polycystic kidney with horseshoe kidney is reported. A 32-year-old woman visited our hospital with the complaint of proteinuria. with computerized tomographic scan and further examinations the patient was diagnosed as having renal angiomyolipoma with tuberous sclerosis. The tumor originated from a polycystic horseshoe kidney. Three weeks later, she complained of right flank pain and was diagnosed with spontaneous rupture of the angiomyolipoma. Right heminephrectomy was performed and histological examination confirmed the preoperative diagnosis. Some discussion is made on the characteristics and treatment of renal angiomyolipoma, and the statistics on renal diseases with tuberous sclerosis in Japan are presented.     

A case of polycystic kidney disease with nephrotic syndrome

Nephrotic syndrome developed in a patient with autosomal-dominant polycystic kidney disease. Renal biopsy revealed minor glomerular abnormalities. This type of nephrotic syndrome complication in an autosomal-dominant polycystic kidney disease has only rarely been reported.     

Hemangiopericytoma of the kidney: a case with preoperative ethanol embolization

We report a case of a giant renal hemangiopericytoma that was embolized preoperatively with ethanol. Ultrasound and computerized tomography showed multiple smooth-walled cysts within the tumor. The tumor itself was hypervascular and a vascular pattern specific for hemangiopericytoma was noted upon reinterpretation of the angiograms. The diagnostic and therapeutic aspects are discussed, and the literature is reviewed.     

Transcatheter renal arterial embolization therapy on a patient with polycystic kidney disease on hemodialysis.

We report a patient with autosomal dominant polycystic kidney disease (ADPKD) undergoing long-term hemodialysis who underwent transcatheter arterial embolization (TAE) of the renal arteries to shrink enlarged kidneys. In 1983, the patient started hemodialysis because of chronic renal failure secondary to ADPKD. However, renal size continued to increase. In January 1997, he was admitted to our hospital with abdominal distension and anorexia, in addition to progression of anemia. Upper gastroendoscopy showed an esophageal ulcer and severe external compression of the stomach. Renal angiography using the Seldinger technique showed stretched and deformed segmental renal arteries with massive enlargement of the kidneys. TAE with stainless steel coils was performed on both renal arteries. With a rapid and progressive decrease in kidney size, anorexia and anemia were improved, and the gastrointestinal compression was eliminated. In some patients with ADPKD, renal size continues to increase even after the initiation of dialysis. In about 10 years, patients develop gastrointestinal complications, such as dysphagia, ileus, severe constipation, and intestinal perforation. Surgical procedures such as nephrectomy are not satisfactory. This report shows that TAE is a safe and effective therapy for patients with ADPKD with massively enlarged kidneys.     

Disseminated kidney tuberculosis complicating autosomal dominant polycystic kidney disease: a case report

Mycobacterium tuberculosis infection in patients with autosomal dominant polycystic kidney disease (ADPKD) is rare, and its diagnosis and treatment are difficult because numerous cysts are exposed to infection and antibiotics do not easily penetrate infected cysts. Here, we report the case of a 43-year-old Japanese man with disseminated urogenital tuberculosis (TB) and ADPKD without human immunodeficiency virus (HIV) infection. Delayed diagnosis and ineffective anti-TB chemotherapy worsened his condition. Finally, he underwent bilateral nephrectomy but experienced postoperative complications. In conclusion, kidney TB should be recognized as a cause of renal infection in ADPKD, and surgical treatment should be instituted without delay. The importance of early diagnosis and treatment cannot be overemphasized to prevent kidney TB deterioration.     

常染色体显性遗传型多囊肾合并气肿性肾盂肾炎一例

患者,女,60岁,10年前出现反复尿频、尿急、尿痛、双下肢和面部水肿,间断服利尿剂和抗生素.入院前24 d出现洗肉水样小便和右上腹部疼痛,伴反复阵发性寒颤、高热39.8℃,每次持续4 h.     

A mechanistic approach to inherited polycystic kidney disease

There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.     

A renocolic fistula due to colonic diverticulitis associated with polycystic kidney

Renocolic fistulae caused by colonic diverticulitis are rare. We present a case of renocolic fistula caused by colonic diverticulitis associated with polycystic kidney. A 51-year-old male with polycystic kidney on hemodialysis presented with a lasting fever and left lower abdominal pain. Under the diagnosis of the infection of a cyst in a polycystic kidney, puncture of the cyst was performed. Nine hundred ml of turbid fluid, of which culture grew Bacteroides Fragilis, was discharged. Inflammation subsided after the puncture, but soon recurred. Moreover, pneumaturia was observed, and fecaloid fluid was drainaged. Barium enema demonstrated a fistula from the diverticulum of the descending colon into the punctured cyst. The patient underwent a nephrectomy combined with hemi-colectomy.     

Transplantation of a cadaveric polycystic kidney in a patient with autosomal dominant polycystic kidney disease: long-term outcome

INTRODUCTION: Kidneys from donors affected by autosomal dominant polycystic kidney disease (ADPKD) were considered unusable for transplantation. To the best of our knowledge, seven cases worldwide have now been described in the English literature since 1967 suggesting such donor organs may be acceptable under certain conditions. Most of these reports have only short-term follow-up. METHODS: We provide a review of these patients and share our experience with an ADPKD patient who had a cadaveric ADPKD transplant and has been closely followed for 10 years. RESULTS: During the 10-year period, the patient had three transplant biopsies without complication. This creatinine is currently 1.2 mg/dL. Serial computed tomography imaging indicated that the cystic disease slowly progressed during this time period. He eventually developed intractable pain in his native left kidney and underwent a laparoscopic nephrectomy. CONCLUSIONS: Normal functioning cadaveric kidneys that show early signs of polycystic kidney disease should be considered acceptable for renal donation. These organs provide the recipient a safe, reasonable period of graft survival and have not been shown to cause adverse effects.     

Fifteen-year follow-up of transplantation of a cadaveric polycystic kidney: a case report

INTRODUCTION: Kidneys from donors affected by autosomal-dominant polycystic kidney disease (ADPKD) are in general considered unsuitable for transplantation. To the best of our knowledge, only 12 cases of ADPKD transplanted renal units have been reported in the English literature; most have only short-term follow-up. METHODS: We provide a review of these patients and share our experience with an ADPKD patient who received a 21-year-old deceased donor ADPKD-affected renal transplant and has been closely followed for 15 years. Based on the current literature, this report is the longest follow-up of a ADPKD donor transplant. RESULTS: Over the 15-year follow-up period, there have been no complications related to the ADPKD-affected donor kidney, including three kidney transplant biopsies. The graft continues to function well with the serum creatinine currently 1.2 mg/dL. Serial axial imaging has demonstrated that the cystic disease has slowly progressed in the donor renal unit, with the largest cyst having only increasing from 1.2 to 2.9 cm in diameter. Metachronous, bilateral laparoscopic nephrectomies of the native kidneys were performed owing to intractable pain from cystic enlargement. CONCLUSIONS: Normal functioning deceased donor kidneys that show signs of early ADPKD should be considered acceptable for donation in select cases. These organs provide the recipient a safe, reasonable period of graft survival and have not been shown to cause adverse effects.     

Imaging approaches to patients with polycystic kidney disease

Imaging is an important approach to diagnosis, monitoring, and predicting outcomes for patients with autosomal-dominant polycystic kidney disease. This article reviews three common clinical imaging techniques, ultrasonography, computed tomography, magnetic resonance imaging, and their role in the management of autosomal-dominant polycystic kidney disease. Ultrasonographic criteria for diagnosis in children and adults are reviewed. Total kidney volume, as measured by magnetic resonance imaging, is suggested as an important potential marker to determine disease progression and overall prognosis. Renal blood flow and a novel approach to interpreting noncystic renal parenchyma by computed tomography images are other innovative imaging approaches described.     

Amyloidosis in a patient with autosomal dominant polycystic kidney disease and tuberculosis: a case report

Autosomal-dominant polycystic kidney disease is an inherited disorder characterized by the development and growth of cysts in the kidneys. Urinary protein excretion is generally less than 1 g/day, and the association of the nephrotic syndrome with this condition is considered rare. A 39-year-old man with autosomal-dominant polycystic kidney disease and nephrotic-range proteiuria is described. During admission, he had general edema and a diagnosis of pulmonary tuberculosis. The patient had hyperlipidemia, hypoalbuminemia, and 11.8 g/day proteinuria. The gingiva and rectum biopsies were performed in order to evaluate the etiology of nephrotic syndrome, and revealed AA amyloidosis thought to be secondary to pulmonary tuberculosis. We maintained the antituberculous treatment and began colchicine at a dose of 2 g/day and candesartan 8 mg/day. To our knowledge, this is the first autosomal-dominant polycystic kidney disease case with nephrotic syndrome due to amyloidosis secondary to pulmonary tuberculosis.     

Retroperitoneoscopic nephrectomy as a second-line treatment after transarterial embolization for symptomatic autosomal dominant polycystic kidney disease

Patients with autosomal dominant polycystic kidney disease (ADPKD) often suffer from abdominal symptoms. Although laparoscopic nephrectomy has been reported as a minimally invasive therapy, it is still technically demanding due to the large size of the kidneys. Transarterial embolization (TAE) is one of the alternatives, but there are only limited reports on its application in ADPKD. We describe a case in which bilateral nephrectomy was performed as a second-line treatment after TAE. One kidney was removed because a small feeding arterial branch was not completely embolized. The other kidney was removed due to infection. Retroperitoneoscopic nephrectomy was a good choice as a second-line modality in the case without infection because the volume of the kidney was reduced even with incomplete TAE, and adhesion after TAE was minimal. TAE is an effective choice in ADPKD patients without infection as a first-line treatment even when complete embolization is difficult, since nephrectomy after TAE is technically easier than removal of a fresh ADPKD kidney.