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Introduction Recently, nephronophthisis (NPH) has been considered a monogenic cause of end-stage renal disease (ESRD) in adults. However, adult-onset NPH is difficult to accurately diagnose and has not been reported in a cohort study. In this study, we assessed the genetic background and clinicopathologic features of adult NPH. Methods We investigated 18 sporadic adult patients who were suspected as having NPH by renal biopsy. We analyzed 69 genes that cause hereditary cystic kidney disease and compared clinicopathologic findings between patients with and without pathogenic mutations in NPH-causing genes. Results Seven of 18 patients had pathogenic NPH-causing mutations in NPHP1, NPHP3, NPHP4, or CEP164. Compared with patients without pathogenic mutations, those with pathogenic mutations were significantly younger but did not significantly differ in the classic NPH pathologic findings, such as tubular cysts. On the other hand, the number of tubules with thick tubular basement membrane (TBM) duplication, which was defined as >10-μm thickness, was significantly higher in patients with genetically proven adult NPH than in those without pathogenic mutations. α-Smooth muscle actin (α-SMA)-positive myofibroblasts were detected inside thick TBM duplication. Conclusions In adult patients with NPH, thick TBM duplication was the specific finding. Our analysis also suggested that older patients tended to have no pathogenic mutations, even when they were suspected to have NPH by renal biopsy. These findings could be the novel clinical clue for the diagnosis of NPH in adult patients., Graphical abstract

Decreased physical activity and activities of daily living (ADL) in patients on hemodialysis (HD) are associated with a poor prognosis. Additionally, comorbid peripheral arterial disease is associated with further deterioration. We conducted a cross-sectional study of ADL difficulty and life-space assessment (LSA) in three groups of patients on hemodialysis according to their ankle-brachial index (ABI) values. The 164 patients were divided into ABI Low (ABI < 0.9), Normal (0.9 ≤ ABI < 1.3), and High (1.3 ≤ ABI) groups, and compared using analysis of covariance with LSA and ADL difficulty adjusted for age. The Kihon checklist (KCL) was used to assess the presence of frailty. The LSA was lower in the Low group than in the High group (F = 3.192, p = 0.044). Similarly, the ADL difficulty was significantly lower in the Low group than in the Normal group (F = 3.659, p = 0.028). In the Low group, the proportion of patients with frailty was 47.1% and KCL physical was significantly lower, indicating that patients on HD with a lower ABI had a higher prevalence of frailty and lower LSA and ADL difficulty. In conclusion, patients on HD with decreased ABI values most likely exhibit decline in physical activity and ADL due to frailty and claudication.

To test the efficacy of chemical disinfectants against bacterial biofilms in hemodialysis equipment, a Center for Disease Control and Prevention (CDC)-Biofilm Reactor was used to create biofilms. Methylobacterium radiotolerance was isolated from the hemodialysis fluid and used as the test organism. We examined the efficacy of sodium hypochlorite (NaOCl) in elimination of planktonic cells compared to that in the case of biofilms. Planktonic bacteria were completely eliminated at 50 parts per million (ppm) of NaOCl, which is the lowest concentration for clinical use. The viable cell count in the biofilm reached its minimum value around a logarithmic reduction value (LRV) of 6, when the concentration was raised to 1000 ppm and the reaction time was extended by 1 hour or more. Furthermore, at 200 ppm, the LRV was elevated depending on the time. And the LRV while maintaining static conditions for 6 hours at 200 ppm was similar to that of short time at 1000 ppm. These results suggest that NaOCl has sufficient bactericidal activity even for biofilms at a practical concentration and reaction time, and that the CDC-Biofilm Reactor is an effective tool for finding useful disinfection conditions.

The traditional anion gap (AG) equation is widely used, but its misdiagnosis in end-stage kidney disease (ESKD) patients has not been investigated fully. Diagnostic accuracy to detect high AG was cross-sectionally evaluated using 3 AG equations in 1733 ESKD patients with an eGFR less than 15 mL/min/1.73 m2. The prevalence of high AG was 67.9%, 92.1% and 97.4% by the traditional, albumin-adjusted AG (aAG) and full AG equations, respectively. The sensitivity, specificity, accuracy and Kappa coefficient obtained with the traditional AG vs aAG equation were 0.70 vs 0.94, 0.98 vs 0.93, 0.7 vs 0.94, and 0.103 vs 0.44, respectively. Next, we created a subcohort comprising only patients with high full AG and investigated how the traditional AG equation leads to misdiagnoses. Multivariable-adjusted regression analysis in 1688 patients revealed that independent factors associated with a false-negative AG diagnosis were ARB use, eGFR, blood leukocyte count, serum chloride, bicarbonate, ionized calcium, potassium, albumin and phosphate. 93.2% of our subcohort prescribed any of RAAS inhibitors, Loop diuretics or Alkali which could increase either serum chloride or bicarbonate. Frequent use of these possible AG-reducing medications may conceal high AG state in patients with ESKD unless they have incidental inflammation which may increase AG value.

Aquatic bacteria were isolated from the hands of working staffs by an adapted culture protocol. When the sample solution obtained by the" glove juice method" was incubated for 3 days at room temperature, viable cell counts increased up to 105-fold, and the majority of the isolated colonies were shown to be Gram-negative aquatic bacteria, which carry the risk of contaminating water. Using R2A medium, coagulase-negative staphylococci were the dominant microbes immediately after recovery from the hands. Here it was revealed that bacteria of the phylum Proteobacteria isolated from the hand can be the causative bacteria of aqueous contamination. This modification in the GJ method may be useful as an effective training protocol to demonstrate the importance of hand hygiene and clean operation for aseptic manufacturing.

Progression of chronic kidney disease (CKD) leads to the onset of cardiovascular dis- eases and an increase in the number of patients requiring dialysis initiation. In prder to promote the early detection and treatment of CKD, we assessed the effects of blood pressure control on renal impairment based on common test items and verified the usefulness of the target blood pressure for patients with CKD described in the Japanese Society of Hypertension Guidelines for the Management of Hypertension in 2014 (JSH 2014).Among patients who had been regularly visiting the outpatient clinic of Kasukabe Kisen Hospital for 24 months or more, 67 with a serum creatinine level of 1.2 mg/dL or higher were included in this study. Clinical blood pressure measurements obtained at the start of follow-up and at 6, 12, 18, and 24 months were averaged to serve as the 2-year mean blood pressure, and the progression rates of renal impairment were com- pared between patients achieving and those not achieving the target blood pressure of the JSH 2014.Among the diabetic patients with CKD, significant differences in renal impairment progression rates were observed between those achieving and those not achieving the target blood pressure. Among the non-diabetic patients with CKD, those achieving the target blood pressure tended to show slower progression of renal impair- ment, but their progression rates were not significantly different from those of the patients not achieving the target blood pressure.Blood pressure control is essential for patients with CKD. In patients with diabetes mellitus, the pro- gression rates of renal impairment can be substantially reduced by maintaining blood pressure below the target blood pressure described in the JSH 2014.

TAFRO (Thrombocytopenia, Anasarca, Fever and/or elevated C-reactive protein, Renal dysfunction or Reticulin fibrosis, and Organomegaly) syndrome is a unique systemic inflammatory disease, which has recently been proposed from Japan. We report a case showing TAFRO symptoms, the patient’s condition of which allowed histopathological examinations of several biopsy tissues including the kidney. A 37-year-old man was admitted to our hospital for evaluation of fever of unknown origin, pleural effusion and ascites, renal dysfunction, and lymphadenopathy. Serum IL-6 and VEGF were elevated. Histopathologic findings of the lymph node resembled those of mixed type Castleman disease. The bone marrow biopsy specimen revealed normocellular marrow with a mildly increased number of megakaryocytes, but without obvious fibrosis. The renal biopsy material showed glomerular microangiopathy with double contour of the capillary loops and mesangiolysis. In immunohistochemistry, VEGF was retained in podocytes, whereas IL-6 expression was not limited to the specific sites. Glomerular microangiopathy is regarded as a crucial change in TAFRO syndrome, which should be studied further with regard to VEGF and IL-6.

A 77-year-old man presented with a fever, non-productive cough, and edema formation. A laboratory analysis showed an elevated creatinine level (2.5 mg/dL), a high titer of myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) (99 U/mL), positive reaction for antinuclear antibody (×320), hematuria, and massive proteinuria (3.33 g/day). A renal biopsy revealed crescentic and necrotizing glomerulonephritis (GN) with membranoproliferative GN features [double contour appearance of the glomerular basement membrane, granular deposition of immunoglobulin (Ig) G, IgM, and C3 along the capillary wall, subendothelial and subepithelial deposits with mesangial interposition]. A potential relationship between MPO-ANCA associated GN and membranoproliferative GN is discussed.

Background The management of renal anemia in the pre-dialysis period has been remarkably improved by long-acting erythropoiesis-stimulating agents (ESA). However, many incident dialysis patients cannot achieve target hemoglobin (Hb) levels (> 10 g/dL) and sometimes require blood transfusions. Anemia at the time of dialysis initiation is reportedly correlated with cardiomegaly and early cardiovascular events. Here, we investigated whether this V-shaped depression in Hb level at dialysis initiation adversely affects short-term prognosis. Methods The medical charts of 166 patients who underwent initial dialysis were retrospectively reviewed for Hb level, ESA treatment status, dry weight (DW), cardiothoracic rate (CTR), and brain natriuretic peptide (BNP) level at dialysis initiation and 1 year later. Patients were subdivided into three groups according to the tertile of Hb levels. The risk of mortality within 1 year after initiation was analyzed using multivariable-adjusted Cox proportional hazard model. Result Mean Hb level at initiation was 8.6 ± 1.3 g/dL despite the administration of sufficient ESA. After initiation, Hb levels rapidly increased and the Hb time course showed a V-shape with the bottom at initiation. Hb level, CTR, and log BNP showed a significant negative correlation. The Hb level and CTR 1 year after initiation did not correlate with Hb levels at initiation. Lower Hb levels at initiation as a V-shaped depression do not adversely affect 1-year mortality rate by multivariable-adjusted Cox proportional hazard model. Conclusion Hb level around dialysis initiation showed a V-shaped depression despite ESA use. Our findings suggest that the V-shaped Hb depression at initiation does not affect short-term prognosis.

Glomerulonephropathy is a rare complication of Takayasu's arteritis (TA). To date, most glomerulonephropathies associated with TA show the histological feature of mesangial proliferation. Membranous glomerulonephropathy (MG) is a form of glomerulonephropathy in which the mesangial proliferation is not conspicuous and its association with TA is extremely rare. A 54-year-old man was referred to our hospital due to progressive edema in the lower limbs and nephrotic range proteinuria. Five years previously, he underwent percutaneous angioplasty for left subclavian artery stenosis. Kidney biopsy revealed stage II MG. General examination including enhanced CT scan confirmed the presence of TA. He started oral prednisolone therapy at a dose of 40 mg daily. The C-reactive protein level normalized 7 days after the prednisolone therapy. Three months later, proteinuria had remitted. Though the true relationship between MG and TA was not revealed in present case, considering the fact that complete remission of nephrotic syndrome occurred following the improvement of C-reactive protein level in response to steroid therapy, TA might be the secondary cause of MG. To our best knowledge, only two case reports described the association of MG and TA previously. Those two patients, however, also demonstrated the feature of systemic lupus erythematosus in addition to TA. This is the first case report that describes a patient who presented as MG associated with TA, but not complicated by systemic lupus erythematosus.

We report a case of acquired factor V inhibitors (AFVIs) in a patient with end-stage renal disease receiving warfarin therapy for atrial fibrillation. A 72-year-old Japanese man was admitted to our hospital complaining of tarry stools and abdominal pain. The laboratory findings revealed eosinophilia (52.1%), prolonged activated partial thromboplastin time (APTT) (98 s), PT (84 s), a factor V (FV) activity of

Short bowel syndrome (SBS) is characterized by a malabsorptive state. It is conceivable that the coexistence of SBS and end-stage renal disease can lead to severe metabolic acidosis; however, such a condition has rarely been documented. We herein describe the case of a 64-year-old man with SBS who required maintenance hemodialysis. Persistent metabolic acidosis and mineral and bone disorders should be of particular concern in hemodialyzed patients with SBS.

Dipeptidyl peptidase-4 inhibitors (DPP-4i) have become widely used in hemodialysis patients with type 2 diabetes mellitus (T2DM). This study aimed at testing the hypothesis that administration of alogliptin, a DPP-4i, soon after hemodialysis initiation improves beta cell function in hemodialysis patients with T2DM. Patients with end-stage renal disease and T2DM (n = 10) not previously treated with DPP-4i (mean age, 54.2 years) were enrolled. The study end point was the acute insulin response to glucose assessed by a frequently sampled intravenous glucose tolerance test (IVGTT) that was conducted during the hemodialysis session just before lunch using an external circulation circuit. All patients received 6.25 mg alogliptin for 2 weeks. Blood glucose (Glu), serum insulin (IRI), and C-peptide (CPR) were measured before (0 min) in addition to 5 and 15 min after the glucose load. Glucagon-like peptide-1 (GLP-1) was measured before the glucose load. Glu(0) significantly decreased after the 2-week DPP-4i treatment (174 ± 20 vs. 150 ± 27 mg/dL, P = 0.023). IRI(5) significantly increased after the DPP-4i treatment (14.6 ± 31.7 vs. 23.4 ± 16.6 μU/mL, P = 0.038), but IRI(0) and IRI(15) did not change significantly. GLP-1 also significantly increased after the DPP-4i treatment (6.6 ± 4.4 vs. 2.6 ± 0.8 pmol/L, P = 0.012). Inhibition of DPP-4 with alogliptin improved endogenous insulin secretion in response to intravenous glucose in hemodialysis patients with T2DM.

Eosinophilic granulomatous with polyangiitis (EGPA) is systemic vasculitis characterized by concomitant symptoms of asthma, allergic rhinitis, and marked increase in peripheral eosinophilia. It was previously known as Churg-Strauss syndrome. EGPA incidence in Japan is very low, with only approximately 1,800 cases reported. Renal involvement occurs in approximately 20-25% of EGPA patients, and the most typical expression is pauciimmune crescentic glomerulonephritis. We herein report a case of 69-year-old Japanese woman with fever and high titer of myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) and eosinophilia. Her renal biopsy showed massive interstitial nephritis with granulomatous vasculitis like lesion without apparent active crescent formation in glomeruli. Immediately after steroid treatment (prednisolone [PSL] 30 mg/day), she had symptomatic relief and was discharged with a reduction in MPO-ANCA.

A 73-year-old man with systemic edema and oliguria, which appeared within several days, was referred to our hospital. Urinalysis showed massive proteinuria, and the blood examination results indicated nephrotic syndrome. Renal biopsy revealed the existence of focal segmental glomerulosclerosis (FSGS), and further investigation indicated that the microscopic features were consistent with the “cellular variant (CELL)” according to the Columbia Classification. After biopsy, 40 mg per day of oral prednisolone was administered daily. With steroid therapy, the abnormal values of the blood examination and urinalysis gradually reached normal, and complete remission was achieved on day 43 after initiating steroid administration. Microscopic investigation by follow-up renal re-biopsy revealed several sclerosing glomeruli; however, other intact glomeruli had no endothelial proliferation, which is a hallmark of CELL. The patient was discharged after biopsy and observed as an outpatient, with maintenance of clinical remission.

This study has examined cases of management buyouts (MBOs), which have been increasing rapidly in number since around 2000. First, an overview of MBO practices is provided, indicating the beginning of an increase in divestment-type MBOs as a new means to implement corporate restructuring. Subsequently, the factors used by Japanese companies to decide on whether to pursue divestment MBO were analyzed while particularly addressing the parent companies––the sellers of the business units. Results suggest the following factors leading to the parent company divestment of subsidiaries and business units through MBOs: 1) poor performance of the business of the parent company, 2) high debt-to-asset ratio (debt reliance) of the parent company, 3) wide diversification of parent company operations, and 4) active reorganization of the parent company’s corporate group. The structure of corporate governance also affects MBO trends, indicating that 5) companies for which shareholding ratios of institutional investors and directors are high are more likely to implement a divestment MBO. Conversely, 6) companies that are protected by cross-shareholdings are less likely to implement corporate restructuring.

症例 : 82歳, 女性. 現病歴 : 1998年に当院で血液透析を導入され他院にて通院透析中であった. 2006年4月17日より右眼瞼に発疹, 疼痛が出現し近医皮膚科で帯状疱疹と診断. 透析患者であるためアシクロビル軟膏で対処されるも疼痛我慢できず21日再診. 透析担当医と相談の上バラシクロビル1,000mg毎透析終了時で処方され内服. 翌日午前より歩行障害, 呂律障害, せん妄を認め当院救急外来受診. 血圧168/62mmHg, 体重30kg, 意識障害, せん妄を認めた. 頭部CT・MRI, 髄液検査, SPECT, 脳波などで脳血管障害, ヘルペス脳炎などを鑑別, 除外した上で, アシクロビル脳症と診断. 直ちにバラシクロビルを中止し血液透析を施行. 計4回の透析で症状は完全に消失し退院した. 結語 : バラシクロビルは透析患者推奨投与量においても小柄な高齢患者では神経症状を呈しうる. 体格, 年齢も考慮した上での薬物投与が必要と考えられた. 日本人透析患者への推奨投与量はこれまでの添付文書記載量よりさらに減量した量 (250mg/日) が妥当と考えられた.

Chalcogenide materials have regained attention after the recent recognition of the compatibility of transition metal dichalcogenides with graphene. Additionally, there has been a recent appreciation for the rich variety of properties they support due to the anomalies in the materials’ intrinsic band structure. These materials generally have layered structures and weak interlayer connection through the chalcogen layer and its van der Waals type bonding. We have synthesized orthorhombic copper telluride and measured its electrical transport properties. The results of these measurements reveal that the conduction is metallic in both the in-plane and out-of-plane directions. The range of stability of this structure is examined along with the lattice constants. The independence of the resistivity in samples to changes in excess copper indicates that the transport is essentially within the conducting planes. This result shows that the material hosts two-dimensional character likely due to its covalent interlayer bonding.

Malposition of the tip of a continuous ambulatory peritoneal dialysis (CAPD) catheter may impede a dialysis, and an invasive method, such as open or laparoscopic surgery, is necessary to correct its position. The alpha-replacement method is a nonsurgical procedure; however, the reported clinical experience has been limited. We thus reviewed our 19 cases to clarify the efficacy of this method.The alpha-replacement method was used in 19 patients [mean age, 50 +/- 14 years old; male/female ratio, 13/6; duration of catheter insertion, 23.7 +/- 23.9 months; total, 29 times in 19 patients (once in 13 patients, twice in 3, three times in 2, four times in 1 patient)]. All patients received a swan-neck J-type catheter. The procedure was carried out by using a special guidewire within 1-2 weeks after dislocation of the tips.The procedure was successfully performed 22 times in 14 patients (success rate, 78% in each performance). Required time was only 5-10 min in most successful cases. No evident complications were encountered in any case.The alpha-replacement method for a displaced CAPD catheter proved to be effective and safe.

A 62-year-old-Japanese man had a history of probable granulomatosis with polyangiitis (GPA) from 7 years previously, showing kidney and vasculitis symptoms with PR3-ANCA (864 EU) without renal biopsy. Remission with normalization of renal function and urinary findings was induced by corticosteroid therapy. Prednisolone (PSL) was tapered to 5 mg/day and maintained for 6.5 years with a low positive titer of PR3-ANCA. After 7 years of remission, he was referred to our hospital because of arthralgia, fever, general fatigue and appetite loss with apparent urinary abnormality, increased serum Cr (1.8 mg/dL) and C reactive protein (CRP : 30.1 mg/dL). On admission, he showed a high titer of PR3-ANCA (300 U/mL). Renal biopsy demonstrated the existence of the pauci-immune type of severe crescentic necrotizing glomerulonephritis, tubulo-interstitial damage and perivascular granuloma. He was diagnosed as relapse of GPA (kidney-localized type) without upper respiratory tract (E) and lung (L) symptoms. Accordingly, he received steroid pulse therapy leading to improvement of these symptoms and renal function. Oral PSL at the dosage of 40 mg/day was administered after steroid pulse therapy, and then tapered to 20 mg/day. Cyclophosphamide was added within 8 weeks. He was discharged 8 weeks after treatment with a decreased level of Cr (1.5 mg/dL) and PR3-ANCA (244 U/mL). After discharge, PSL was tapered to 10 mg/day during the course of stability resulting in a further improved level of Cr (1.2 mg/dL), PR3-ANCA 40 U/mL in the outpatient clinic. In Japan, PR3-ANCA-positive GPA has a lower incidence than MPO-ANCA-positive microscopic vasculitis. In GPA, the kidney-localized (K) type without upper respiratory tract (E, L) symptoms is rare. Histologically, not only necrotizing crescentic glomerulonephritis but also perivascular granuloma in the kidney are very rare and interesting.

The origin of crescent forming cells in human glomerulonephritis (GN) remains unknown. Some animal studies demonstrated that parietal epithelial cells of Bowman’s capsule (PECs) were the main component of proliferating cells and PEC-specific tight junction protein claudin-1 was expressed in crescentic lesions. We investigated the expression of claudin-1 in human GN. Immunohistochemistry for claudin-1 was performed on 17 kidney biopsy samples with crescent formation. Colocalization of claudin-1 with intracellular tight junction protein ZO-1 was also evaluated by immunofluorescence double staining. Claudin-1 is expressed mainly at the cell to cell contact site of proliferating cells in cellular crescentic lesions in patients with these forms of human GN. Small numbers of crescent forming cells showed extrajunctional localization of claudin-1. Colocalization of claudin-1 with ZO-1 was found at cell to cell contact sites of adjacent proliferating cells. In control samples, staining of claudin-1 was positive in PECs, but not in podocytes. Our findings suggest that claudin-1 contributes to crescent formation as a component of the tight junction protein complex that includes ZO-1. Co-localization of claudin-1 with ZO-1 implies the formation of functional tight junction complexes in crescentic lesions to prevent the interstitial damage caused by penetration of filtered molecules from Bowman’s space.

腹膜機能検査の一つとして期待される排液中のIL-6値とperitoneal equilibration testの諸因子との関係について検討した. 1年以上腹膜炎の既往のない腹膜透析患者40例を対象とした. 2.5%透析液2Lを4時間腹腔内に貯留し, 中間点で採血した. 血中 (P) と排液中 (D) のIL-6, Cr, β2-MG, Albを測定した. Cr, β2-MG, AlbのD/P比を求め, さらにD/D0 glucose比を算出し, 排液中のIL-6値との関連を比較した. (1) 排液中のIL-6値は54.43±38.02 pg/mlであった. (2) 排液中のIL-6値と各指標との相関は, D/D0 glucose比ではR=-0.803 (p.

An X-ray Talbot interferometer consisting of two capillary plates, which were used as X-ray amplitude gratings, was evaluated for X-ray phase imaging. A theoretical aspect of capillary X-ray Talbot interferometry is presented with a preliminary operation result using synchrotron radiation. A two-dimensional X-ray Talbot effect, or self-imaging effect, which was the basis of Talbot interferometry, was observed with the capillary plate, and moire images formed by the X-ray Talbot interferometer exhibited contrasts corresponding to the differential phase shift caused by phase objects placed in front of the interferometer. Finally, the possibility of quantitative phase measurement with a fringe scanning technique is discussed.

First Talbot interferometry in the hard X-ray region was demonstrated using a pair of transmission gratings made by forming gold stripes on glass plates. By aligning the gratings on the optical axis of X-rays with a separation that caused the Talbot effect by the first grating, moire fringes were produced inclining one grating slightly against the other around the optical axis. A phase object placed in front of the first grating was detected by moire-fringe bending. Using the technique of phase-shifting interferometry, the differential phase corresponding to the phase object could also be measured. This result suggests that X-ray Talbot interferometry is a novel and simple method for phase-sensitive X-ray radiography.

A 86-year-old man had been treated for hypertension, diabetes mellitus (DM), and dyslipidemia in Nihonkoukan Hospital. His renal function was within the normal range in August 2007. He showed common cold-like symptoms, which were not improved by anti-inflammatory drugs in December 2007. He was admitted to our hospital because of renal failure, urine protein and urine occult blood. He was also positive for anti-myeloperoxidase antibody (MPO-ANCA; 129 IU/mL). A renal biopsy revealed idiopathic crescentic glomerulonephritis of the pauci immune type. Considering his advanced age and DM, he was treated with the low dose of 20 mg/day of prednisolone. Although his symptoms, such as low grade fever and general fatigue, were improved after steroid therapy, renal failure accelerated, necessitating hemodialysis (HD), and insulin administration was needed for his DM. Subsequently, an AV fistule operation for HD was performed. Prednisolone was tapered to 17.5 mg/day after 4 weeks, and his MPO-ANCA titer decreased to 87 IU/mL. After steroid treatment and HD, his condition gradually recovered and he was discharged on March 5, 2008. Following about 6 months of treatment with prednisolone (3.5 months after HD administration), his renal function gradually recovered, allowing the discontinuation of HD. High-dose steroid therapy is very effective for ANCA-related glomerulonephritis. However, there is a high risk of infection, especially in aged and DM patients. Low-dose steroid therapy (PSL 20 mg/day) is safe and effective in such high-risk patients and in some cases, they can be released from HD.

It has been suggested that hyperlipidemia contributes to the progression of kidney disease and there are some experimental reports that support the hypothesis of lipid nephrotoxicity. The treatment of hyperlipidemia in patients with renal disease has two purposes: to prevent the development of cardiovascular disease and to prevent the progression of renal disease. However, statins, which are widely used to treat hyperlipidemia, should be used very carefully in patients with renal disease, especially in those whose serum creatinine level is more than 3 mg/dL. Atorvastatin, an HMG-CoA reductase inhibitor, is completely metabolized in the liver. Thus, we thought that atorvastatin could be used safely in hyperlipidemic patients with chronic renal disease.Atorvastatin was administered to 84 hyperlipidemic patients with chronic renal disease(including dialysis patients) for 12 months. TC, TG, LDL-C, AST, ALT, CK, BUN, and Cr were measured at 3, 6, and 12 months during treatment. Blood pressure and renal function, as indicated by urinary protein excretion and creatinine clearance measured at 0 and 12 months during treatment, were also monitored.TC and LDL-C were decreased at every determination point regardless of the kidney function, which was not affected by atorvastatin. Urinary protein excretion (UP) decreased significantly during the study period in patients who had not taken any anti-hyperlipidemic drug before treatment with atorvastatin. This decrease in UP was not associated with significant Ccr change. However, the decrease in UP was not statistically significant in all the patients. The decrease in UP showed a significant positive correlation with the decrease in TC and of the mean BP.Atorvastatin can be used safely in hyperlipidemic patients with chronic renal disease including dialysis patients under periodical monitoring. Atorvastatin could contribute to prevent the progression of renal disease.

A Sixty eight-year-old man complained of shortness of breath on exercise since the spring of 2001. An abnormal lung shadow was pointed out on chest X-ray and progression of renal dysfunction and high gamma globulinemia were detected out on blood examination. He was admitted to the Department of Respiratory Disease in our hospital in March 2003, because of bilateral lower lung interstitial shadow, severe anemia (Hb 7.9 g/dl), and renal dysfunction (S-Cr 1.9 mg/dl). He was found to have hypergammaglobulinemia (IgG 2,997 mg/dl), positive RO/SS-A antigen, high serum KL-6 level (2,050 U/ml), and increased urinary excretion of beta2-microglobulin (beta2MG). Both Gum test and Schirmer test results were positive. Lip biopsy showed cell infiltration to the salivary glands and he was diagnosed as having Sjogren's syndrome. Renal biopsy showed diffuse interstitial cell infiltration and a Trans Bronchoscopic Lung Biopsy (TBLB) showed fibrotic thickness and lymphocyte infiltration in the alveolar septum. Accordingly, he was diagnosed as having Sjögren's syndrome complicated with both interstitial nephritis and interstitial pneumonitis. He was treated by high-dose corticosteroid therapy and anticoagulant heparin. His laboratory data showed that both serum KL-6 and urinary beta2MG were reduced. Chest CT showed remarkable improvement of the interstitial shadow. A second renal biopsy performed at ten weeks after the beginning of treatment showed remarkable improvement of the interstitial cell infiltration. This is a rare case of Sjögren's syndrome complicated with interstitial nephritis and interstitial pneumonitis, treated successfully with high-dose corticosteroid therapy. Both complications showed immediate improvement with high-dose corticosteroid therapy, suggesting that early steroid therapy is effective for both complications.

X-ray phase tomography with X-ray Talbot interferometry (XTI) is reported. XTI employs two transmission gratings and generates a contrast corresponding to the differential phase shift caused by a sample. Quantitative phase measurement and tomographic image reconstruction with XTI are demonstrated for biological samples. Finally, the possibility of medical applications of XTI is discussed, based on the advantage of XTI that divergent and polychromatic X-rays are available.

Phase‐contrast X‐ray imaging, including phase tomography, was performed at the medium‐length beamline BL20XU of SPring‐8, where undulator X rays were available at 245 m from the source point. A crystal X‐ray interferometer and an X‐ray Talbot interferometer with transmission gratings were used to perform different kinds of phase‐contrast imaging. The crystal X‐ray interferometer was devised in that a crystal lamella for beam recombination was thinned down to 40 μm to achieve high‐resolution imaging. A quick fringe‐scanning mechanism was also constructed enabling the phase measurement with a few second and therefore phase tomography with several minutes. The X‐ray Talbot interferometer was studied as a novel and simple method that generated a contrast corresponding to differential phase. Phase tomography was also attained with the X‐ray Talbot interferometer.

X‐ray Talbot Interferometry (XTI) is demonstrated as a novel and simple X‐ray phase‐sensitive imaging method. Differential phase contrast is generated with XTI and quantitative determination of the differential phase is attained in combination with a phase‐shifting technique, further enabling phase tomography. Because XTI does not use crystals but transmission gratings, divergent X‐rays with a broad energy band are available. This is an advantage for medical applications especially using a compact X‐ray source.