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.     

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.     

Concomitant nephrectomy of massively enlarged kidneys and renal transplantation in autosomal dominant polycystic kidney disease

OBJECTIVES: We compared perioperative and intraoperative data of patients with end-stage renal disease (ESRD) due to autosomal dominant polycystic kidney disease (ADPKD) who received a renal allograft without native nephrectomy with ADPKD patients who underwent concomitant native nephrectomy of massively enlarged kidneys and renal transplantation to determine whether the latter approach is reasonable and safe. PATIENTS AND METHODS: From January 1987 to December 2003, 13 patients with ESRD due to ADPKD were stratified as 6 patients who underwent bilateral and 7 patients who underwent unilateral native nephrectomy in conjunction with renal transplantation (group A), versus 20 patients with ESRD due to ADPKD underwent renal transplantation without native nephrectomy (group B). Operative time, need for intraoperative transfusion, time to oral intake, duration of hospital stay, serum creatinine level on the day of discharge, readmission rate, and postoperative complications were compared for both groups. RESULTS: Mean intraoperative duration was significantly longer for patients in group A, but there was no statistically significant difference in the findings between both groups. CONCLUSIONS: Concomitant native nephrectomy of massively enlarged kidneys at the time of renal transplantation is reasonable and safe for patients with ESRD due to ADPKD.     

Glomerulocystic kidney disease in an adult with enlarged kidneys: a case report and review of the literature

We report the case of a 31-year-old male with enlarged kidneys and glomerulocystic kidney disease (GCKD). The patient had no family history of renal disease or other diseases. On initial presentation he complained of poor eyesight, and hypertensive retinopathy and elevated serum creatinine (5.0 mg/dl) were found at that time. Renal biopsy showed cystic dilatation of Bowman's capsule and atrophy of the glomerular tuft. Thus, an adult case of sporadic GCKD was diagnosed. Based on previous reports, kidney size in patients with adult type GCKD varies from small to large. Our patient's kidneys are the largest ever reported (right kidney was 22 cm×10 cm, left kidney was 19 cm×10 cm). A review of the literature dealing with sporadic adult GCKD suggested that it is difficult to diagnose this disease early in its course.     

Renal disease progression in autosomal dominant polycystic kidney disease

Background

Autosomal dominant polycystic kidney disease is a lifelong progressive disorder. However, how age, blood pressure, and stage of chronic kidney disease (CKD) affect the rate of kidney function deterioration is not clearly understood.

Methods

In this long-term observational case study up to 13.9?years (median observation period for slope was 3.3?years), serum creatinine was serially measured in 255 mostly adult patients. The glomerular filtration rate was estimated (eGFR) using a modified Modification of Diet in Renal Disease Study method. The total kidney volume (TKV) has been measured in 86 patients at one center since 2006.

Results

As age increased, eGFR declined significantly (P?Conclusion The declining rate of eGFR was relatively constant and did not correlate with age or eGFR after adolescence. eGFR was already low in young adult patients with hypertension. As age increased after adolescence, eGFR declined and TKV increased similarly between normal and high blood pressure groups. eGFR starts to decline in patients with normal eGFR, suggesting that the decline starts earlier than previously thought.     

Caroli's syndrome and adult polycystic kidney disease

Caroli's disease is a cystic disease of the liver, which has been rarely associated with adult onset polycystic kidney disease. Three cases have been reported in the English Medline search. The presentation of this fourth case discusses the issues surrounding the treatment of Caroli's disease in the setting of a renal transplant.     

Acquired cystic disease of the kidney analyzed by microdissection

Four cases of acquired cystic disease of the kidney (ACDK) were studied by the microdissection technique (MD) of Darmady and Baert to analyze the cystic transformation. No patient had a history or clinical evidence of the adult polycystic disease of the kidney (APDK). Hypothetical models related the pathogenesis of cystic transformation to either obstructive or degenerative factors. Microdissection was performed in four nephrectomy specimens of hemodialyzed patients and a total of 155 nephrons were isolated. The atrophy of the glomeruli has already been described histopathologically but MD demonstrated the existence of nephrons consisting of sclerotic glomeruli and enlarged segments between the atrophic convoluted proximal and distal tubules. Diverticula and cysts were located, above all along the proximal (mainly dilated) convoluted tubules: they were always in continuity with the tubules. Phase contrast microscopy showed a patent lumen in 80% of the proximal and distal convoluted tubules, and a regular lining of the cysts. These data support the hypothesis that ACDK is the result of hyperplasia and dilation of remaining nephrons, rather than a result of obstruction and/or fibrosis.     

Chronic kidney disease progression in native and transplant kidneys

PURPOSE OF REVIEW: The present review addresses the recent literature demonstrating important differences in the rate of progression of kidney function decline between transplant recipients and patients with native kidney disease. It also highlights the need for prospective studies to determine the importance of nonimmune factors that are established risk factors for progression of native kidney disease in the transplant setting. RECENT FINDINGS: Transplant recipients establish modest levels of kidney function but have rates of kidney function decline that are slower than those in patients with native kidney disease. Continued improvements in long-term graft survival have not been achieved despite significant advances in immunosuppression. There is increasing observational evidence that nonimmune factors that play a causal role in progression of native kidney disease may also be important determinants of allograft decline. There are fundamental differences between transplant recipients and patients with native kidney disease that preclude extrapolation of evidence from native kidney disease to the transplant setting. SUMMARY: Transplant recipients are a unique group of chronic kidney disease patients. Prospective studies to determine the importance of nonimmune factors such as hypertension, proteinuria, dyslipidemia, diabetes, and anemia in the transplant setting are needed.     

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.     

Acquired cystic kidney disease in children undergoing long-term dialysis

Acquired cystic kidney disease (ACKD) occurs in adult patients undergoing long-term dialysis. Early detection is important because clinically significant hematuria and malignancies are associated with ACKD. We evaluated by magnetic resonance imaging (MRI) and ultrasonography (US) the incidence of ACKD in 15 patients aged 7.3–21.6 years (mean 15.9 years) with non-cystic primary renal disease. Nine patients had been treated with peritoneal dialysis only, and 6 with both hemodialysis and peritoneal dialysis for 24–73 months (mean 37 months). Three patients (20%) had no cysts. In 5 patients (33%) with bilateral multiple cysts, the diagnosis of ACKD was made by MRI and US. In another 5 patients, solitary cysts were localized to one kidney by MRI, and in 2 patients solitary cysts were seen in both kidneys. This study documents that ACKD is not limited to older patients with end-stage renal disease. Early detection of these cysts can be accomplished by MRI and is warranted since 1 patient developed neoplastic tubular changes which can precede tumor formation.     

Autosomal dominant polycystic kidney disease coexisting with cystic fibrosis

Autosomal dominant polycystic kidney disease (ADPKD) is a common, inherited disorder characterized by the progressive enlargement of fluid-filled cysts in the kidneys and liver. Since the cystic fibrosis transmembrane conductance regulator (CFTR) Cl--channel may mediate the secretion of Cl--and fluid into the cysts, it is conceivable that coexisting cystic fibrosis (CF) in patients with ADPKD could attenuate their development. We previously reported that two patients with ADPKD coexisting with cystic fibrosis (CF) had a milder cystic phenotype than that of kindred without CFTR mutations. A subsequent report failed to confirm this protective effect. We now have identified another family with coexisting type 1 ADPKD and CF. The kidney volumes and the number and size of renal and hepatic cysts were markedly less in a member of this family with ADPKD (PKD1 mutation C508R) and CF (homozygous DeltaF508 mutation) than in her sister with ADPKD alone at comparable ages.