Unusual presentation of intraparenchymal renal artery aneurysm mimicking cystic renal cell carcinoma: a case report.

We describe a case of intraparenchymal renal artery aneurysm in a 58-year-old normotensive man with a history of distal ureterectomy. Imaging studies of the pre-existing right renal mass had been interpreted as being consistent with a simple renal cyst. Three years after ureterectomy, a small parietal nodule with contrast enhancement developed within the cyst. Partial nephrectomy was carried out based on a preoperative diagnosis of cystic kidney cancer. Surprisingly, pathological diagnosis was an aneurysm with a revascularized thrombus. Even though the present case represents an extremely rare clinical manifestation of intraparenchymal renal artery aneurysm, clinicians should be aware that imaging studies cannot distinguish all instances of renal vascular disease.

[Case of autosomal dominant polycystic kidney disease associated with pelvic insufficiency fracture].

We experienced a case of A-59-year-old woman having autosomal dominant polycystic kidney disease with renal insufficiency associated with pelvic insufficiency fracture. On admission the pelvic bone compressed by the enlarged kidney and liver due to polycystic disease was demonstrated on the pelvic CT. Her bone mineral density was not significantly decreased contrary to our expectation. The prominent bone absorption in the endosteal and exosteal surface of the cortical bone was demonstrated in the bone specimen form iliac bone although the state of the cancellous bone and bone formation rate was normal. We performed the transcatheter embolization (TAE) to the kidney and liver to diminish their sizes. The fracture was rapidly improved and she could walk 6 months later after the TAE. In this case, we considered that the longstanding compression to the pelvic bone by the enlarged liver and kidney made the cortical and the binding of muscle/tendon and cortical bone fragile and it led to the pelvic insufficiency fracture.

Autosomal dominant polycystic kidney disease showing rupture of a lateral ventral hernia following paralytic ileus.

We report an 83-year-old Japanese male with autosomal dominant polycystic kidney disease (ADPKD), which was marked by unusually enlarged kidneys, and in whom ileus occurred after administration of procainamide. The bowels became swollen and ruptured the skin and appeared on the outside of the skin. Even after the ileus state was resolved, the projected intestinal tract was not restored due to a large defect of the skin, and ostomy was performed. Abdominal hernia including lateral ventral hernia due to enlarged kidneys may result in perforation of the abdominal wall as well as intestinal wall.

A patient with symptomatic osteomalacia associated with Fanconi syndrome.

We report a patient with renal tubulointerstitial fibrosis and symptomatic osteomalacia associated with Fanconi syndrome. A 55-year-old woman was hospitalized because of an inability to walk. Beginning approximately 2 years previously, she had experienced gradually worsening pain in the hips, shoulders, and trunk, culminating in a bedridden state. Serum urea nitrogen was 38 mg/dl; creatinine, 2.6 mg/dl; uric acid. 3.6 mg/dl; phosphate, 2.3 mg/dl; and alkaline phosphatase, 2111 IU/l. Urinary beta2 microglobulin was 72 331 microg/day. Aminoaciduria, renal glucosuria, and proximal renal tubular acidosis with a normal anion gap were also noted. The patient was diagnosed with Fanconi syndrome. Radiography demonstrated typical Looser zones in the proximal portion of the left and especially the right femoral shaft, and at several other sites. A renal biopsy specimen disclosed severe tubulointerstitial fibrosis with little cellular infiltration. Glomeruli were largely intact. A bone biopsy specimen indicated osteomalacia; no tetracycline labeling could be seen along most trabecular bone surfaces, and the ratio of total osteoid volume to bone volume was increased (71.8%). Bicarbonate administration (9 g/day) gradually lessened most symptoms, permitting ambulation. Calcitriol administration decreased excessive intact-parathyroid hormone emerging after 2 months of acidosis correction. Thus, severe acidosis associated with Fanconi syndrome can induce osteomalacia showing serious skeletal complications, but also responsiveness to bicarbonate therapy.