Transplantation for polycystic kidney disease

Twenty-four patients with end stage renal failure due to polycystic renal disease have been treated with hemodialysis and transplantation. While on dialysis, the incidence of complications did not differ from a similar group of patients with other causes of renal failure. Bilateral pretransplant nephrectomy is not mandatory except in cases of persistent infection or hemorrhage. A much higher incidence of HLA A3 and HLA B7 was noted in patients with polycystic disease when compared with the general population. Following cadaver renal transplantation, kidney function was significantly better in patients with polycystic disease when compared with those with other forms of renal failure. Patient survival was the same in both groups. We conclude that hemodialysis and transplantation are acceptable forms of treatment for a patient with end stage polycystic renal disease.     

Acquired cystic disease of kidney in chronic dialysis patients

Eight cases of acquired cystic disease of the kidney (ACDK) associated with chronic renal failure and hemodialysis are described. No patient had a family history or clinical evidence of congenital adult polycystic kidney disease (CAPKD). Glomerulonephritis was the cause of renal failure in 6, and pyelonephritis in 2. Massive renal and perirenal hemorrhage necessitated 3 nephrectomies in 2 patients. Single kidney weights did not exceed 280 Gm., a major feature in the distinction of ACDK from CAPKD. Morphologically, in addition to the usual stigmata of end-stage kidneys, 40 to 80 per cent of the renal parenchyma was replaced by small cysts. Continuity of cysts with tubules was established by nephron dissection.     

Von Hippel-Lindau disease simulating polycystic kidney disease

Polycystic kidney disease and the renal manifestations of von Hippel-Lindau disease have much in common. Making the distinction between these two diseases is important. There is a strong association of renal cell carcinoma with von Hippel-Lindau disease, whereas renal cell carcinoma is rare in polycystic kidney disease. Furthermore, the many extrarenal manifestations of von Hippel-Lindau disease are serious and can be fatal while those of polycystic kidney disease are generally benign. Early diagnosis of the lesions of von Hippel-Lindau disease could lead to effective surgical treatment and prevent death. A case of von Hippel-Lindau disease is presented which was incorrectly diagnosed as polycystic kidney disease for sixteen years. The case is instructive in that the possibility of making the correct diagnosis prior to the patient's terminal illness was only through careful assessment of the family. The case is also remarkable in that the patient suffered from progressive renal failure requiring hemodialysis, which has not been associated previously with von Hippel-Lindau disease.     

Polycystic kidney disease at end-stage renal disease in the United States: patient characteristics and survival

BACKGROUND: The patient characteristics and mortality associated with autosomal dominant polycystic kidney disease have not been characterized for a national sample of end-stage renal disease (ESRD) patients. METHODS: 375,152 patients in the United States Renal Data System were initiated on ESRD therapy (including patients who eventually received renal transplants) between January 1, 1992 and June 30, 1997 and analyzed in an historical cohort study of polycystic kidney disease. RESULTS: Of the study population, 5,799 (1.5%) had polycystic kidney disease. In logistic regression, polycystic kidney disease was associated with Caucasian race (odds ratio 3.31, 95% CI, 3.09-3.54), women (1.10, 1.04-1.16), receipt of renal transplant (4.15, 3.87-4.45), peritoneal dialysis (vs. hemodialysis, 1.37, 1.27-1.49), younger age, and more recent year of first treatment for ESRD. Use of pre-dialysis EPO but not the level of serum hemoglobin at initiation of ESRD was significantly higher in patients with polycystic kidney disease. Patients with polycystic kidney disease had lower mortality compared to patients with other causes of ESRD, but patients with polycystic kidney disease had a higher adjusted risk of mortality associated with hemodialysis (vs. peritoneal dialysis) compared to patients with other causes of ESRD (hazard ratio 1.40, 1.13-1.75). CONCLUSIONS: Hematocrit at presentation to ESRD was not significantly different in patients with polycystic kidney disease compared with patients with other causes of ESRD. Peritoneal dialysis is a more frequent modality than hemodialysis in patients with polycystic kidney disease, and patients with polycystic kidney disease had an adjusted survival benefit associated with peritoneal dialysis, compared to patients with other causes of renal disease.     

Dialysis-associated renal cystic disease resembling autosomal dominant polycystic kidney disease: a report of two cases.

Acquired renal cystic disease is common in patients receiving dialysis. Characteristically, the kidneys are small or, less often, normal in size, and the cysts are usually less than 0.6 cm in diameter. We present here 2 patients who, after 5 and 7 years on hemodialysis, developed marked renal enlargement, with large cysts in the kidneys and, in 1 patient, in the liver as well; the appearance on ultrasonography and computed tomography was indistinguishable from autosomal dominant polycystic kidney disease. Before starting dialysis the first patient was a 19-year-old man who developed renal shutdown from crescentic glomerulonephritis, and the second patient was a 33-year-old man who developed end-stage renal failure from malignant hypertension. Neither patient had renal cysts at the onset of end-stage renal failure.     

Acquired renal cystic disease in HD: a study of 82 nephrectomies in young patients.

In order to study the development of acquired renal cystic disease (ARCD) and its potential complications, we studied, macro- and microscopically, 82 surgical specimens of nephrectomy carried out on young patients with chronic renal failure previous to renal transplantation. Statistical correlation of pathological findings with age, sex and time on hemodialysis (HD) have been done. There were 72 cases of ARCD (87.8%). It was statistically correlated with male sex (p less than 0.02) and prolonged time on HD (p less than 0.001) as has been previously reported. Hyperplasia of the cystic epithelium was found in 42 cases (52%), with 18 (22%) showing marked papillary proliferation. Also, there were 22 cases (27%) with renal adenomas. This incidence of hyperplastic and neoplastic proliferations, more than would be common in such a young population (males: 33.5 +/- 9.3 years; females: 35.4 +/- 11.7 years), suggests the potential of patients affected by ARCD to develop neoplasms. Thus, we consider that these patients must be checked periodically to detect possible malignant neoplasms.     

Successful percutaneous drainage for infected cysts in polycystic kidney disease in a case of chronic hemodialysis

A 60-year-old woman, who had been receiving chronic hemodialysis for chronic renal failure due to polycystic disease for 7 years, was admitted to our hospital with the chief complaints of right flank pain and high-grade fever on February 3, 1986. She had a family history of polycystic kidney disease and her elder sister has been undergoing chronic hemodialysis. The patient has had repeated episodes of urinary tract infection. The right markedly distended kidney was palpated with severe tenderness, and the performance status was poor. Computerized tomography showed the right kidney enlarging beyond the median line, but localization of the infected cysts was not clear. The 7-day intensive antibiotic therapy had no beneficial effect. On the 8th hospital day, percutaneous drainage, which was carried out for a large cyst with low-level echoes using ultrasonic guidance, yielded 600 ml of hemopurulent fluid. Bacterial culture of the aspirated fluid was positive for Escherichia coli. As the clinical symptoms did not improve after the 8th day, 10 Fr. catheter placement, percutaneous drainage and catheter placement were added for other cysts containing hemopurulent fluid. Although hematopyuria persisted, the patient became afebrile and the right kidney became smaller and softer. Both catheters were removed on the 30th hospital day. Repeated computerized tomography or ultrasonography revealed a few hyperdense or hyperechoic cysts in the right kidney. However, no recurrence of high-grade fever or right flank pain has been seen for 3 months. Ultrasonic-guided percutaneous drainage has a great potential for patient care of infected cysts in polycystic kidney disease.     

Acquired renal cystic disease: two cases of associated adenocarcinoma and a renal ultrasound survey of a peritoneal dialysis population

Long-term dialysis patients frequently develop acquired renal cystic disease (ARCD). The discovery of ARCD and renal cell carcinoma in one of our hemodialysis patients led us to review the literature. ARCD has been described mainly in the maintenance hemodialysis (MH) population. Therefore, we investigated 20 peritoneal dialysis (PD) patients for ARCD using ultrasonography. Seven patients (35%) had detectable cysts and two patients (10%) had multiple bilateral cysts. One patient had a large asymptomatic complex cyst that proved to be an adenocarcinoma. Our study suggests that ARCD is relatively common in the PD population, and we speculate that it may be related more to length of time in end-stage renal disease (ESRD) than to the mode of dialysis. The potential for malignant change appears to justify a routine screening examination with ultrasonography and/or computerized tomography (CT) to detect this recently described and probably underrecognized entity.     

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).     

Acquired renal cystic disease in children prior to the start of dialysis

This report describes the clinical course and serial sonographic findings in three children who developed acquired renal cystic disease (ARCD) prior to the institution of dialysis. The children were aged from 3 years to 13 years and their estimated glomerular filtration rate varied from 8 to 13 ml/min per 1.73 m² when ARCD was diagnosed. Their primary renal disorders, which included hemolytic-uremic syndrome and focal segmental glomerulosclerosis, had been present for 1.5–11.5 years prior to the cysts being discovered. These patients show that ARCD may develop in children with chronic progressive renal parenchymal disease prior to the institution of specific therapy for end-stage renal disease.     

Successful pregnancy in a patient with polycystic kidney disease and advanced renal failure: the use of prophylactic dialysis.

Adult polycystic kidney disease is an inherited disease that is transmitted as an autosomal dominant trait. The clinical manifestations, which develop during the third or fourth decade of life, usually do not affect women during childbearing age and thus do not affect fertility or pregnancy outcome. The patient presented here had polycystic kidney disease and advanced renal failure, and was treated with meticulous fetal surveillance and prophylactic hemodialysis during pregnancy. The successful outcome strengthens the trend to perform prophylactic dialysis in pregnancies with advanced renal failure, despite the lack of controlled studies.     

Management of unruptured cerebral aneurysms in patients with polycystic kidney disease

BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) is a hereditary disorder characterized by bilateral multiple renal cysts and early onset chronic renal failure. PKD patients tend to suffer their subarachnoid hemorrhage at a younger age. Unruptured aneurysms in PKD patients are not always innocuous, and proactive treatment has been indicated for these lesions. However, the management of PKD patients undergoing unruptured cerebral aneurysm surgery has been documented on only a few occasions. The purpose of this study was to better define the management of unruptured cerebral aneurysms in patients with PKD. METHODS: We present a retrospective review of the management of unruptured cerebral aneurysms in 16 patients with PKD. Eight patients were maintained through chronic hemodialysis whereas the remaining 8 patients did not require hemodialysis, at the time of treatment of their cerebral aneurysms. The mean follow-up period was 24 months. RESULTS: In the nonhemodialysis patients prophylactic hemodialysis was routinely performed after cerebral angiography to prevent deterioration of the pre-existing renal dysfunction. Microsurgical clipping of the aneurysm was performed in 15 patients (7 nonhemodialysis and 8 hemodialysis patients) and intravascular coil embolization was performed in 1 nonhemodialysis patient. One nonhemodialysis patient who underwent microsurgical clipping required a temporary hemodialysis after surgery, but the patient was not shifted to chronic hemodialysis. No patients developed postprocedural complications, and each showed an excellent recovery. CONCLUSION: PKD patients with unruptured cerebral aneurysms can be safely treated with an appropriate treatment strategy including the use of prophylactic hemodialysis.     

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.     

Orofacial digital syndrome type 1: an underlying cause of chronic renal failure

Orofacial digital syndrome type 1 is condition which is characterized with, in addition to oral-facial and digital congenital anomalies, polycystic renal disease in most patient, and the prognosis is dependent on renal involvement in such patients. Our case was a 22-year-old patient who was presented with clinical picture of chronic renal failure, was started on hemodialysis and had took our attention due to oral, facial and digital anomalies in addition to polycystic renal disease.     

Autosomal dominant polycystic kidney disease associated with aortic dissection: Two case reports

We describe 2 patients with autosomal dominant polycystic kidney disease associated with aortic dissection. Both patients presented with chronic renal failure arising from autosomal dominant polycystic kidney disease, as well as with aortic dissection, DeBakey type IIIb.     

Acquired cystic kidney disease: rapid progression from small to enlarged kidneys simulating adult polycystic kidney disease

A 57-year-old man on chronic hemodialysis presented marked bilateral renal enlargement due to acquired cystic kidney disease (ACKD). He had been on hemodialysis for less than 3 years only (14 months prior to receiving a functional renal transplant which lasted 8 years, followed by 18 additional months of dialysis), before the diagnosis of ACKD was made following an episode of flank pain with gross hematuria. The marked changes in kidney appearance during this 11-year period were documented by serial ultrasound examination showing the kidneys to be of near-normal size before the start of dialysis (> or =10 cm in 1986), then shrunken and contracted 5 years later while having a functioning renal transplant (<5 cm in 1991), and markedly enlarged reaching the size of adult polycystic kidney disease after returning to dialysis (>13 cm in 1997). Since the risk of ACKD increases with duration of dialysis, we sought additional predisposing factors in this unusual case and found that 2 years after renal transplantation, the patient was diagnosed with breast cancer for which he was treated with surgical excision and tamoxifen. Based on ultrasound evidence that the tamoxifen treatment preceeded the appearance of the renal cystic changes, we wonder whether this drug may have played a role in the rapid development of ACKD.     

Etiology of chronic renal failure in Jenin district, Palestine

A study was conducted on chronic renal failure patients treated by medications or by hemodialysis at The Martyr Dr. Khalil Sulaiman Hospital in Jenin city, Palestine, from 1/8/2005 to 1/8/2006 to know the underlying etiology of chronic renal failure. The subjects included were 84 patients. The information was obtained from files of the patients. The diagnosis was based on medical history, laboratory tests, X-rays, CT scans, ultrasound and renal biopsies. The results showed that the three most common causes of chronic renal failure in Jenin district were diabetes mellitus (33.32%), hypertension (16.7%), and chronic glomerulonephritis (13.1%). Inherited kidney diseases formed an important percentage (17.85%) and included primary hyperoxaluria (10.71%), Alport's syndrome (5.95%), and adult polycystic kidney disease (1.19%). These results differ from what is found in most developing countries including many Arab countries where the principal causes of chronic renal failure are chronic glomerulonephritis and interstitial nephritis. The high prevalence of inherited kidney diseases in some families (primary hyperoxaluria and Alport's) syndrome may be explained by the very high prevalence of consanguineous marriage especially among cousins in these families.     

Choledochal Cyst Associated With Polycystic Kidney Disease: Report of a Case

We report a very rare case of type I choledochal cyst associated with a polycystic kidney disease. A 48- year-old female had been dependent on hemodialysis for chronic renal failure due to polycystic kidney disease and was incidentally diagnosed to have a dilated common bile duct by an ultrasonography. An endoscopic retrograde cholangiopancreatography showed a spindle-shaped, dilated common bile duct (type I choledochal cyst) without visualization of the pancreatic duct. She underwent a resection of the choledochal cyst. Intraoperative cholangiography showed no reflux of contrast medium into the pancreatic duct. Amylase level of the aspirated bile from the bile duct was not elevated. In the case of choledochal cyst combined with renal fibropolycystic disease, pancreaticobiliary maljunction may not contribute to the etiology of choledochal cyst. In such cases, management of choledochal cyst is still controversial and requires further discussion.     

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.     

Unilateral presentation of adult-type polycystic kidney disease in children

Adult polycystic kidney disease is characterized by autosomal dominant inheritance, diffuse bilateral cystic kidney disease and onset during the young adult years. Relatively few cases of adult-type polycystic kidney disease in children have been reported. We describe a 4-year-old girl who appeared to have multiple, unilateral simple renal cysts by excretory urography and ultrasonography. There was no known familial renal cystic disease. When renal histology was consistent with adult-type polycystic kidney disease, screening nephrosonograms revealed that the mother also had the disease. We review 10 published cases of unilateral presentation of adult-type polycystic kidney disease in children. This report emphasizes the necessity of screening studies in parents of children with diffuse unilateral or bilateral cystic renal disease. Awareness of the unexpected unilateral presentation of adult-type polycystic kidney disease in children may allow a nonoperative diagnosis to be made.