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A 66-year-old manwith diabetes developed acute renal failure after ingestion of Amanita pseudoporphyria Hongo. Laboratory data showed acute nonoliguric renal failure. A renal biopsy showed acute tubular necrosis with glomerular minor abnormalities. He received hemodialysis treatment for 3 weeks and his renal function normalized 2 months after admission. Wediscuss the differences in acute renal failure caused by possible toxins of Amanita pseudoporphyria Hongo from that caused by other poisonous mushrooms. (Internal Medicine 42: 78-81, 2003)

A 28-year-old man was admitted to our hospital with nausea, headache and weakness of the left hand. He had severe uremia without hypertension due to recurrent/chronic pyelonephritis. Brain magnetic resonance imaging showed reversible vasogenic edema in the brainstem and bilateral frontal centrum semiovale. All of his neurological symptoms immediately improved after the introduction of hemodialysis. When a patient with uremia presents with neurological symptoms, posterior reversible encephalopathy syndrome should be considered in the differential diagnosis even if high blood pressure is not observed. Brain magnetic resonance imaging may be helpful in such a case, and an appropriate therapy could be subsequently initiated.

Oliguric acute kidney injury (AKI) with minimal change nephrotic syndrome (MCNS) has long been recognized. Several mechanisms such as hypovolemia due to hypoalbuminemia and the nephrosarca hypothesis have been proposed. However, the precise mechanism by which MCNS causes AKI has not been fully elucidated. Herein, we describe an elderly patient with AKI caused by MCNS who fully recovered after aggressive volume withdrawal by hemodialysis and administration of a glucocorticoid. A 75-year-old woman presented with diarrhea and oliguria, 43 and laboratory examination revealed nephrotic syndrome (NS) and severe azotemia. Fluid administration had no effect on renal dysfunction, and hemodialysis was initiated. Her renal function improved upon aggressive fluid removal through hemodialysis. Renal pathological findings revealed minimal change disease with faint mesangial deposits of IgA. After administration of methylprednisolone pulse therapy followed by oral prednisolone, she achieved complete remission from NS. The clinical course of this case supports the nephrosarca hypothesis regarding the mechanism of AKI caused by MCNS. Furthermore, appropriate fluid management and kidney biopsy are also important in elderly patients with AKI caused by NS.

No abstract

A common renal phenotype of paired box protein 2 (PAX2) mutations is renal coloboma syndrome. We report a single family with diverse renal phenotypes associated with PAX2 mutation. The proband presented steroid-resistant focal segmental glomerulosclerosis with optic coloboma, whereas his two sons showed severe renal hypoplasia with end-stage renal disease, with or without optic coloboma. In all three cases, a heterozygous PAX2 genetic mutation was identified (exon 2; NM_003987.3:c.76dupG, p.Val26Glyfs*28). Based on histopathological findings of the proband, we hypothesized that autophagic dysfunction was associated with the pathophysiology of the focal segmental glomerulosclerosis with PAX2 mutation. Detailed funduscopic examination - including the optic disc - might be useful for the diagnosis of renal anomalies associated with PAX2 mutation.

Background/Aims: Pseudotumors of the craniovertebral junction (PTCVJ) are observed in long-term hemodialysis (HD) patients. There are neither criteria for diagnosis nor guidelines for screening. We attempted to determine magnetic resonance imaging (MRI) findings that could be used to detect PTCVJ, to determine the prevalence of PTCVJ, and to evaluate whether destructive spondyloarthropathy (DSA) might be a yardstick for selection of patients for MRI examination for PTCVJ. Methods: MRI were examined in 19 DSA patients (8 males, 11 females, age 61.4 ± 7.3 years, HD duration 17.0 ± 4.4 years) and in 20 sex-, age-, and HD-duration-matched non-DSA patients (9 males, 11 females, age 57.5 ± 6.6 years, HD duration 17.7 ± 4.9 years). We evaluated MRI characteristics of PTCVJ according those which occur due to rheumatoid arthritis. Results: PTCVJ were characterized as follows: disappearance of fat pads in the upper region (supradental PTCVJ), intensity change of the ‘predental triangle’ in the anterior region (predental PTCVJ), and thickening of cruciform ligaments (retrodental PTCVJ). The prevalence of PTCVJ among patients undergoing HD more than 10 years was high (26 out of 39; 66.7%). The prevalence of PTCVJ was not different between DSA and non-DSA groups. Conclusion: We verified that the above MRI findings might be helpful in the detection of PTCVJ. These findings were observed frequently and independently also in patients with DSA.

A 59-year-old woman developed hemolytic uremic syndrome (HUS) associated with acute human parvovirus B19 (HPVB19) infection. A renal biopsy revealed glomerular mesangiolysis with segmental hypercellularity; mild fibrinogen/fibrin deposits were noted by immunofluorescence study and severe endothelial injury was noted electron microscopically. The histological findings were compatible with HUS. We discuss the relation of HPVB19 to HUS, with special reference to the tropism of the virus for endothelial cells.