Survey of the effects of a column for adsorption of ß2-microglobulin in patients with dialysis-related amyloidosis in Japan.

Dialysis-related amyloidosis is a serious complication of long-term hemodialysis. Its pathogenic mechanism involves accumulation of ß2-microglobulin in the blood, which then forms amyloid fibrils and is deposited in tissues, leading to inflammation and activation of osteoclasts. Lixelle, a direct hemoperfusion column for adsorption of ß2-microglobulin, has been available since 1996 to treat dialysis-related amyloidosis in Japan. However, previous studies showing the therapeutic efficacy of Lixelle were conducted in small numbers of patients with specific dialysis methods. Here, we report the results of a nationwide questionnaire survey on the therapeutic effects of Lixelle. Questionnaires to patients and their attending physicians on changes in symptoms of dialysis-related amyloidosis by Lixelle treatment were sent to 928 institutions that had used Lixelle, and fully completed questionnaires were returned from 345 patients at 138 institutions. The patients included 161 males and 184 females 62.9 ± 7.7 years age, who had undergone dialysis for 25.9 ± 6.2 years and Lixelle treatment for 3.5 ± 2.7 years. Based on self-evaluation by patients, worsening of symptoms was inhibited in 84.9-96.5% of patients. Of the patients, 91.3% felt that worsening of their overall symptoms had been inhibited, while attending physicians evaluated the treatment as effective or partially effective for 72.8% of patients. Our survey showed that Lixelle treatment improved symptoms or prevented the progression of dialysis-related amyloidosis in most patients.

Plasma cell-rich acute rejection after renal transplantation in a patient with pyoderma gangrenosum: a case report.

A 40-yr-old female received a living-related renal transplantation on January 29, 2008. She had type I diabetes mellitus and pyoderma gangrenosum (PG). Induction immunosuppressive therapy consisted of tacrolimus, mycophenolate mofetil, basiliximab, and prednisolone. Intravenous methylprednisolone pulse therapy was administered to prevent ulceration at the surgical site. The postoperative outcome was almost uneventful, and renal graft function was well preserved for 11 months. Her graft function deteriorated on December 24, 2008 and thus an episode biopsy was performed. The histopathological findings were consistent with plasma cell-rich acute rejection (PCAR). During hospitalization, it was noted that the patient was non-compliant. We then performed steroid pulse therapy, and her graft function and histological findings improved. This is the first report of PCAR in a patient with PG who received a renal allograft. It was thought that PCAR was triggered because of her non-compliance. Thus, we should recognize the importance of enhancing compliance in transplant recipients.

Outbreak of larval Echinococcus multilocularis infection in Japanese monkey (Macaca fuscata) in a zoo, Hokkaido: western blotting patterns in the infected monkeys.

A high prevalence of larval Echinococcus multilocularis (Em) infection was found in zoo primates in Hokkaido, Japan. In October 1997, a Japanese monkey (Macaca fuscata) died and histopathologically diagnosed as alveolar hydatidosis. Serum samples were collected from the remaining Japanese monkeys and examined for antibodies against Em by enzyme-linked immunosorbent assay and western blotting. Serological tests showed 12 more animals of the remaining 57 monkeys were possibly infected. Ultrasonography revealed that nine of these 12 animals had a cystic lesion in the liver. The band patterns of western blotting in the monkeys were very similar to those in human.

Cell-surface retention of PrPC by anti-PrP antibody prevents protease-resistant PrP formation.

The C-terminal portion of the prion protein (PrP), corresponding to a protease-resistant core fragment of the abnormal isoform of the prion protein (PrP(Sc)), is essential for prion propagation. Antibodies to the C-terminal portion of PrP are known to inhibit PrP(Sc) accumulation in cells persistently infected with prions. Here it was shown that, in addition to monoclonal antibodies (mAbs) to the C-terminal portion of PrP, a mAb recognizing the octapeptide repeat region in the N-terminal part of PrP that is dispensable for PrP(Sc) formation reduced PrP(Sc) accumulation in cells persistently infected with prions. The 50% effective dose was as low as approximately 1 nM, and, regardless of their epitope specificity, the inhibitory mAbs shared the ability to bind cellular prion protein (PrP(C)) expressed on the cell surface. Flow cytometric analysis revealed that mAbs that bound to the cell surface during cell culture were not internalized even after their withdrawal from the growth medium. Retention of the mAb-PrP(C) complex on the cell surface was also confirmed by the fact that internalization was enhanced by treatment of cells with dextran sulfate. These results suggested that anti-PrP mAb antagonizes PrP(Sc) formation by interfering with the regular PrP(C) degradation pathway.

Solitary nonepitheliotropic T-cell lymphoma in a dog.

A 7-year-old male Golden Retriever with swelling of the rostral bridge and right wing of the nasal areas, sneezing, and inspiratory difficulty was referred to a neighbor veterinarian. Except for those in the nasal area, no lesions were noted during routine physical examination. The mass occupying the nasal cavity was not observed radiographically. Punch biopsy of the affected lesions revealed nonepitheliotropic lymphoma. Immunohistochemical staining for CD3 antigen was positive. The dog was diagnosed with solitary nonepitheliotropic T-cell lymphoma. Local radiotherapy and systemic chemotherapy with doxorubicin were instituted and resulted in total clinical remission. The dog has remained disease free for 30 months.

Short-term biological effects of a new and less acidic fluid for peritoneal dialysis.

BACKGROUND: Conventional peritoneal dialysates are potentially bioincompatible and seem to be a causative factor for peritoneal sclerosis. Recent studies demonstrated that a new type of dialysate has a positive long-term clinical effect on dialysis patients. METHODS: In this study, to elucidate the short-term biological effects of a newly developed dialysate of higher pH on the peritoneal membrane, we assessed macrophage proportions and several markers (inflammatory cytokines, cancer antigen 125 (CA125) and albumin) in spent dialysates before and 2 weeks after the change to the new fluid from a conventional fluid. RESULTS: We found that the use of the new dialysate decreased intraperitoneal levels of inflammatory cytokines, CA125 and albumin associated with the decrease of macrophage populations in dialysis effluents. CONCLUSION: These observations suggest that a new and less acidic fluid reduces pro-inflammatory potential in the peritoneum, and thus affords better preservation of peritoneal membrane integrity in uremic patients on peritoneal dialysis.