BXSB-type genome causes murine autoimmune glomerulonephritis: pathological correlation between telomeric region of chromosome 1 and Yaa.

The autoimmune-prone BXSB/MpJ-Yaa mouse is a model of membranous proliferative glomerulonephritis (MPGN). Severe MPGN has been reported only in male BXSB/MpJ-Yaa mice because of the Y-linked autoimmune accelerator (Yaa) locus. However, we show that female BXSB/MpJ mice develop age-related MPGN without Yaa. Female BXSB/MpJ mice clearly developed MPGN characterized by increased mesangial cells, thickening of the glomerular basement membrane (GBM), double contouring and spike formation of GBM with T-cell infiltrations and podocyte injuries corresponding with increased autoantibody production and albuminuria. Analysis of the renal levels of the Fc gamma receptor (Fcgr) and interferon-activated gene 200 (Ifi200) family genes, which are MPGN candidate genes localized to the telomeric region of chromosome 1 (Chr.1), showed that Fcgr2b levels decreased, whereas Fcgr3 and Ifi202b levels increased in female BXSB/MpJ mice compared with healthy C57BL/6 mice. Furthermore, in isolated glomeruli, microarray analysis revealed that Fcgr3, Fcgr4 and Ifi202b expression was higher in male BXSB/MpJ-Yaa mice than in male BXSB/MpJ mice. These findings indicate that the BXSB/MpJ-type genome causes age-related MPGN with significant contribution from the telomeric region of Chr.1, and Yaa enhances the expression of genes localizing to this locus, thereby leading to severe MPGN in male mice.

IgA nephropathy in a patient with systemic lupus erythematosus.

Systemic lupus erythematosus is generally recognized to be a multisystem autoimmune disease with kidney involvement. However, the occurrence of other non-lupus glomerulopathies has been rarely reported in patients with systemic lupus erythematosus. It is well known that lupus nephritis may switch over time to another class according to the World Health Organization classification. It seems likely that IgA nephropathy is a clinical characteristic of a particular subset of patients with systemic lupus erythematosus. We report a 22-year-old Japanese man with recurrence of proteinuria. The renal flare occurred when he was without lupus clinical and serological activity, and renal remission was only obtained with angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker therapy. Although the incidence of IgA nephropathy is high in Japan, we believe that this is the first report of a Japanese patient in which lupus nephritis switched over time to IgA nephropathy.

A case with spontaneous bladder rupture mimicking acute kidney injury.

A 77-year-old female with abdominal pain and ascites was admitted to our hospital. She had a past history of the postoperative pelvic irradiation for uterine cancer and subsequently suffered from neurogenic bladder. On admission, serum creatinine (s-Cr) and blood urea nitrogen (BUN) were elevated to 9.9 mg/dl and 131 mg/dl, respectively. However, both the ratio of BUN/s-Cr and creatinine in ascites/s-Cr were significantly elevated. The clinical manifestations of the present case were not typical for acute kidney injury. Furthermore, 2 days after urethral catheterization, both s-Cr and BUN were normalized (0.69 mg/dl and 10 mg/dl, respectively) and her symptoms had improved immediately. Therefore, we diagnosed her disease as spontaneous bladder rupture. We report a case with spontaneous bladder rupture mimicking acute kidney injury forty years after postoperative pelvic irradiation for uterine cancer.

[Renal damage in a chronic active hepatitis C patient receiving interferon-alpha therapy].

A 68-year-old male patient with chronic active hepatitis C was treated with interferon-alpha (IFN-alpha) for a period of 5 months. The patient responded well to the IFN therapy showing substantial improvement in liver function and disappearance of HCV-RNA. However, one year after the treatment he was found to have developed proteinuria and showed a reduction in Ccr. Renal biopsy findings were as follows: Light microscopy showed diffuse expansion of mesangial cells with a focal/local increase in cellularity accompanied by capillary loop thickening. Splitting of the basement membrane was also present. An immunofluorescent study showed that IgA was localized predominantly in the peripheral capillary wall. Electron microscopy showed that there was mesangial cell interposition between the peripheral capillary wall and endothelial cells. Furthermore, endothelial cells were expanded and numerous platelets were seen in the capillary lumen. These findings were compatible with focal MPGN accompanied by activation of endothelial cells. These histological data suggest two clinical disease entities: late-onset renal damage induced by IFN-alpha alone, and HCV-induced renal damage possibly modified by the direct effect of IFN-alpha on the endothelium. The present case suggests that IFN therapy for HCV may produce a particular type of renal damage, under the influence of either IFN or HCV infection, and/or both.

[Factors influencing on the prognosis in breast cancer].

TNM classification, tumor size, lymph node metastasis, histological type of primary tumor, ER status and biological tumor markers have been recognized as prognostic factors in breast cancer. The 673 breast cancer patients undergoing radical mastectomy at this department were analyzed for TNM classification influencing on the postoperative prognosis. Five-and ten- year survival rates were 93% and 89% in stage I, 83.9% and 75.5% in stage II, 67.3% and 60% in stage III. The most common histological type, namely, invasive ductal carcinoma, of primary breast cancer was classified into three types by Japan Mammary Cancer Society. The first type was papillotubular carcinoma, the second solid-tubular carcinoma, and the third scirrhous carcinoma. The prognosis of papillotubular carcinoma was best. Many investigators reported that the prognosis of ER positive breast cancer was good. But in the latest report, the opposite result is obtained. More study is necessary to evaluate the prognostic value of ER. The most common biological tumor markers were CEA, LDH and ALP. The CEA was the best prognosis-factor in biological tumor markers.

[Care for patients with Pick's disease--by using their preserved procedural memory].

Pick's disease are more often complicated than Alzheimer's disease by behavioral disturbances such as social misconduct and stereotypy, that are troublesome and clinically difficult to manage. The purpose of this study was to investigate the possibility of improvement in care for patients with Pick's disease by using their presumably preserved procedural memory. Four inpatients and two outpatients with Pick's disease were studied. Case 1: A 54-year-old right-handed retired clerk presented with a 3-year history of increasing restlessness, word-finding difficulty and impaired comprehension of nominal terms. He was forced to retire from his work because of his social misconduct and incommunicability. On admission he wandered about the ward irritably, did not enter into any conversation, and destroyed all keys in the ward by putting a pencil into keyholes. Neuroradiological examination revealed circumscribed bilateral frontotemporal atrophy which was more marked on the left side. However, he has become more adaptive to the life in the ward and his expression has become peaceful since he began to play othello games with other patients and his doctors. Case 2: A 71-year-old right-handed housewife had progressively altered in her personality and behavior over 4 years. She was restless and showed striking palilalia with marked right frontotemporal atrophy when initially evaluated 2 years after her initial symptoms. Subsequently, her verbal output decreased. She ate large amounts of food and her overweight became a serious problem in her care at home. On admission she wandered about the ward irritably, ate meals of other patients, and put on a quilt on their head. However, these troublesome behavior disappeared when she was bent on knitting that was her former hobby. Case 3: A 63-year-old right handed physician presented with a 5-year history of increasing word-finding difficulty and impaired comprehension of nominal terms. His conversation was limited to continuous repetition of a few phrases. He wandered into several hospitals around his house in fixed order every day. On examination, he was mildly disinhibited and distractible with circumscribed bilateral frontotemporal atrophy which was more marked on the left side. Wandering away from home overnight necessitated admission to the hospital. In the ward he wandered about and tried to open each door restlessly and irritably. However, he has become calm and his expression has become peaceful since he was conducted to measure blood pressures of his nurses. Case 4: A 65-year-old right-handed retired office worker.(ABSTRACT TRUNCATED AT 400 WORDS)