[A CASE OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE COMPLICATED WITH HENOCH-SCHÖNLEIN PURPURA NEPHRITIS].

Purpura nephritis and autosomal dominant polycystic kidney disease are relatively rare kidney disorders. We present a case complicated by these two diseases. A 68 year-old man with polycystic kidney disease was referred to our hospital with a high fever lasting 3 days and pyuria. Pyelonephritis was suspected based on computed tomography findings of bilateral swelling of the kidney. Inflammation subsided gradually after the initiation of antimicrobial therapy. However, approximately 3 weeks later, the patient developed a fever and skin purpura on the extremities, stomach colic pain, gross hematuria, and increased proteinuria was evident. Therefore, we diagnosed Henoch-Schönlein purpura complicated with nephritis based on biopsies of the skin and the kidney. Immunosuppressant therapy was administered; every symptom was relieved and proteinuria decreased for approximately 20 months.

[A Case Report : Spontaneous Rupture of Chromophobe Renal Cell Carcinoma].

A 31-year-old man presented with sudden right flank pain. We found a right renal tumor with a perirenal hematoma on enhanced abdominal computed tomography (CT), which suggested spontaneous rupture of the renal tumor. The tumor was located at the upper pole of the right kidney, and was not enhanced. Magnetic resonance imaging (MRI) showed slightly enhanced renal tumor, and positron emission tomography (PET) showed a hot spot in the renal mass. His anemia was getting worse, and we assumed that the renal mass was malignant. Therefore, we performed a right nephrectomy with a transperitoneal approach. On pathological examination, the tumor was found to be chromophobe renal cell carcinoma. The tumor contained a significant amount of necrotic tissue and a hematoma.

[Two cases of IgG4-related systemic disease arising from urinary tract].

Case 1: The patient was a 68-year-old man. Abdominal computed tomography performed during hospitalization for the close observation of a pituitary gland tumor, showed a right renal mass. Percutaneus needle biopsy revealed IgG4-related disease of the kidney. Pituitary gland tumor biopsy also indicated that the lesion was associated with IgG4-related disease. The pancreas did not show abnormalities. The patient was treated with prednisolone, and both renal and pituitary lesions markedly decreased in size. Case 2: the patient was an 80-year-old man. Right hydronephrosis was observed, and computed tomography showed a right pelvic tumor. Right renal pelvic tumor was diagnosed, and the patient underwent right nephroureterectomy. Pathological examination showed that this tumor was also associated with IgG4-related sclerotic disease. IgG4-related disease tends to occur in multiple organs. This condition should be considered when treating patients with multiple sclerotic diseases. However, in some patients, the disease may be localized to a single site. Further studies are required to elucidate the characteristics of IgG4-related disease.

[Localized amyloidosis of the seminal vesicle: a case report].

Primary amyloidosis of the seminal vesicle is a rare disease entity. We report here a case of localized seminal vesicle amyloidosis with hematospermia. A 66-year-old man visited our hospital with a chief complaint of hematospermia. T2 weighted magnetic resonance imaging (MRI) showed a hypointensity mass in the left seminal vesicle. Needle biopsy revealed amyloidosis of the seminal vesicle. Without any specific treatment, the mass lesion disappeared on MRI, and hematospermia was improved.

Resin-based micropipette tip for immunochromatographic assays in urine samples.

A novel bioanalysis system based on immunochromatography was developed in connection with a nitrocellulose resin modified micropipette tip, such as ZipTip. The sandwich-type immunoassay was applied to our bioanalysis system. The simple handling of the micropipette enabled us to increase the sample volume and detect low concentrations of target antigens in urine samples. In addition, the washing procedure could also be performed easily to reduce the background signal levels. For analytical evaluations, the color intensity was captured by a flatbed scanner, and processed by a software. We have achieved the detection of human chorionic gonadotropin (hCG) and prostate-specific antigen (PSA). The detection limit of hCG was 0.5 ng/ml (0.05 ng/tip), which is comparable to that of other conventional immunochromatographic systems. Moreover, the detection of PSA was greatly improved over the existing systems with the application of different sample volumes, such as 1 ng/ml (0.2 ng/tip) in a 200 microl sample volume, and 1 ng/ml (0.3 ng/tip) in 300 microl sample volume. Our bioanalysis system is a promising candidate for application to point-of-care tests with its simple handling and high sensitivity.

The constitutional t(17;22): another translocation mediated by palindromic AT-rich repeats.

We have demonstrated that the breakpoints of the constitutional t(11;22) are located at palindromic AT-rich repeats (PATRRs) on 11q23 and 22q11. As a mechanism for this recurrent translocation, we proposed that the PATRR forms a cruciform structure that induces the genomic instability leading to the rearrangement. A patient with neurofibromatosis type 1 (NF1) had previously been found to have a constitutional t(17;22) disrupting the NF1 gene on 17q11. We have localized the breakpoint on 22q11 within the 22q11-specific low-copy repeat where the breakpoints of the constitutional t(11;22)s reside, implying a similar palindrome-mediated mechanism for generation of the t(17;22). The NF1 gene contains a 195-bp PATRR within intron 31. We have isolated the junction fragments from both the der(17) and the der(22). The breakpoint on 17q11 is close to the center of the PATRR. A published breakpoint of an additional NF1-afflicted patient with a constitutional t(17;22) is also located close to the center of the same PATRR. Our data lend additional support to the hypothesis that PATRR-mediated genomic instability can lead to a variety of translocations.