Dialysis-Related Cystic Kidney Disease Mimicking Autosomal Dominant Polycystic Kidney Disease in Long-Term Peritoneal Dialysis

Dialysis-Related Cystic Kidney Disease Mimicking Autosomal Dominant Polycystic Kidney Disease in Long-Term Peritoneal Dialysis

Acquired cystic kidney disease (ACKD) is frequently observed in patients with end‐stage renal failure or who are on long‐term dialysis, independent of the cause of the renal failure. Criteria for the diagnosis of ACKD are: (i) the presence of at least one to five kidney cysts confirmed by means of ultrasound or computer tomography (CT); and (ii) pathologically, an extension of cysts involving >25% of the renal parenchyma.

Usually the end‐stage kidneys are of a small to normal size and the renal cysts are <0.5 cm in diameter. These findings differ fundamentally from the morphology of autosomal dominant polycystic kidney disease (ADPKD). Nevertheless, in some cases the differential diagnosis is clinically difficult due to an uncommon macroscopic presentation.

Here we present a 36‐year‐old male patient, who had end‐stage renal failure from IgA‐nephropathy and had developed a large, completely cystic, transformed, ADPKD imitating kidney after 6 years on continuous ambulatory peritoneal dialysis (CAPD).


A 36‐year‐old man with a history of peritoneal dialysis renal replacement therapy was referred to our hospital because of acute flank pain on the left hand side. This was a strong hollow pain which did not spread but nevertheless continued for a few hours after the patient had been admitted. There were no signs of fever, vomiting or abnormalities of bowel movement. One week before admission, the patient had suffered from a cold with a slight cough.

Renal failure with a nephrotic syndrome and a proteinuria of 5 g per day had first been discovered at the age of 30. At that time, the creatinine clearance was markedly reduced (25 ml/min) and the patient was hypertensive. The patient had no family history of kidney disease. A kidney biopsy at the time led to a diagnosis of glomerular sclerosis with mesangial immunoglobulin A deposition and IgA nephropathy (Berger’s disease). Six months later, the patient developed uraemic symptoms and CAPD therapy was started.

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In 1996, the patient’s right kidney had to be surgically removed due to a paranephric abscess. The histological examination of the removed kidney confirmed the presence of an IgA nephropathy with complete sclerosis of nearly all glomeruli. A cystic transformation of this kidney was not observed.

Remarkable in the patient’s past history was an atrial septum defect (ostium secundum defect), which had been successfully closed percutaneously using a clamshell device in 1995.

On admission, the patient was normotensive (140/90 mmHg), the heart rate was normofrequent, the respiratory rate was 18 breaths per minute and there were no signs of fever. His skin was pale and he showed no peripheral oedema. Auscultation revealed a pericardial rub and little moist rales over both lungs. Palpation of the left flank was very painful without any palpable expansive tumour in this region. His peristalsis was normal and the entry zone of the peritoneal catheter showed no cutaneous irritation or signs of local infection. The patient was totally anuretic.

Laboratory results

Laboratory results revealed the uraemic state (creatinine 14 mg/dl, BUN 106 mg/dl), there was a normochromic and normocytic anaemia (haemoglobin 7.1 g/dl, haematocrit 22%), reduced reticulocyte count and normal platelet count. Electrolytes, LDH‐ and lactate‐levels and acid–base balance were normal. Infection parameters were slightly elevated (leucocytes 10.7×103/μl, blood sedimentation 25/41 mm, CRP 63 mg/l).

Ultrasonography showed a greatly enlarged left kidney (17 cm in length, 7.5 cm in width) with a strongly intensified density of the kidney parenchyma and multiple cystic formations around the whole circumference of the kidney. The cysts were of different sizes ranging from 0.3 to 6 cm in diameter and there were various dense echo reflexes inside the cystic formations. No other abnormalities were found in the abdomen; in particular no tumour, no other cysts or free fluid. A subsequent computer tomogram of the abdomen (Figure 1a) confirmed these ultrasonographic findings and provided evidence that the enlarged left kidney contained numerous cyst formations with diameters of up to 4 cm. Signs of abscess or infection were absent. Echocardiographic examination proved a circular echo‐free pericardial effusion without haemodynamic consequences. This finding was interpreted as a uraemic pericarditis due to the end‐stage renal disease state.

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On account of a further decrease in the haemoglobin concentration and assuming that bleeding of a renal cyst was causing the severe flank pain, the patient was then referred to our urology consultant. As a result of this, the nephrectomy of the left kidney was carried out, the post‐operative course being without complications. The peritoneal dialysis catheter was removed at the same time and haemodialysis therapy was started.



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