PubMed | University of Tsukuba, Seirei Sakura Citizen Hospital, Toranomon Hospital, Yokohama City University and Toranomon Hospital Kajigaya
Type: Journal Article | Journal: Clinical and experimental nephrology | Year: 2016
In addition to renin-angiotensin system inhibition (RAS), corticosteroids are recommended for patients who have immunoglobulin A nephropathy (IgAN) with 1g/day proteinuria. Tonsillectomy plus corticosteroid pulse therapy (TSP) had been reported as more effective in producing clinical remission of IgAN than just oral-corticosteroid (OS) or steroid-pulse (SP) therapy-but that remained unconfirmed. Accordingly, this study compared the effects of TSP, corticosteroid therapies, and RAS on a multicenter, large-scale, long-term cohort.1127 biopsy-proven IgAN patients with chronic kidney disease (CKD), G1-3, treated in our hospitals March 1981-December 2013 with TSP (n=209), SP (n=103), OS (n=300), or RAS, alone (n=515), were followed until end-stage renal disease (ESRD) or death, renal survival compared by treatment and proteinuria level. Hazard ratios (HRs) of ESRD were analyzed after adjusting for sex, age, BMI, eGFR, albumin, proteinuria, hematuria, blood pressure, medications, and renal-biopsy year, with propensity-score-matched analyses performed.With TSP as referent, the overall HRs of SP, OS, and RAS were, respectively, 1.33 (0.44-4.04), 3.56 (1.45-8.71), and 3.64 (1.48-8.96); with proteinuria 1.0g/gCre, respective HRs were 2.99 (0.71-12.54), 5.04 (1.44-17.67), and 7.23 (1.98-26.40); with proteinuria<1.0g/gCre, 0.42 (0.04-4.89), 3.24 (0.79-13.30), and 2.05 (0.52-8.05); and for patients with CKD G3, 0.37 (0.10-1.41), 2.14 (0.77-5.94), and 2.03 (0.72-5.72). Similar results were observed in models including pathological grading and/or propensity-score matching.TSP may decrease the risk of ESRD in IgAN patients better than other therapies in CKD G1-2, with proteinuria 1.0g/gCre, while outcome was similar to SP in CKD G3, or with proteinuria<1.0g/gCre.
PubMed | Red Cross, Okinaka Memorial Institute of Medical Research, Kanagawa Cancer Center, Japanese Data Center for Hematopoietic Cell Transplantation and 9 more.
Type: | Journal: Bone marrow transplantation | Year: 2016
In order to examine GvHD prophylaxis in umbilical cord blood transplantation (UCBT) in more detail, we compared transplant outcomes after UCBT for acute leukemia among GvHD prophylaxes using registry data. We selected patients transplanted with a calcineurin inhibitor and methotrexate (MTX)/mycophenolate mofetil (MMF) combination. A total of 1516 first myeloablative UCBT between 2000 and 2012 (Cyclosporine A (CyA) plus MTX, 824, Tacrolimus (Tac) plus MTX, 554, Tac plus MMF, 138) were included. With adjusted analyses, Tac plus MMF showed a significantly higher risk for grade II-IV and III-IV acute GvHD than CyA or Tac plus MTX. Although NRM was similar, Tac plus MMF showed a significantly lower risk of relapse than CyA or Tac plus MTX. A significant difference was observed in the risk of overall mortality (OM) between the MTX-containing group and MMF-containing group. In patients with standard-risk disease, there was no significant difference in the risk of OM in any GvHD prophylaxis. However, in patients with advanced-risk disease, Tac plus MMF showed a significantly lower risk of OM. Therefore, MTX-containing prophylaxis is preferred in UCBT for standard-risk disease, whereas MMF-containing prophylaxis is preferred for advanced-risk disease. A prospective study to identify optimal GvHD prophylaxis for UCBT is warranted.Bone Marrow Transplantation advance online publication, 12 December 2016; doi:10.1038/bmt.2016.255.
PubMed | Toranomon Hospital Kajigaya, Yokohama City University, Toranomon Hospital and Kobe University
Type: | Journal: Human pathology | Year: 2016
A 35-year-old woman was admitted to our hospital for evaluation of end-stage renal failure. Diagnostic imaging, including ultrasonography and magnetic resonance imaging, showed polycystic kidneys and peribiliary hepatic cysts, but the renal cysts were isointense and her kidneys were smaller than the end-stage kidneys of patients with autosomal dominant polycystic kidney disease. Glomerulocystic kidney disease was diagnosed by renal biopsy. Clinical examination revealed findings such as a missing maxillary canine, lingual anomalies, and brachydactyly. Genetic testing gave a diagnosis of orofaciodigital syndrome type 1 with a 5 nucleotide deletion indicating a frameshift mutation in exon 9. The patients mother had the same mutation and similar clinical findings. This case is useful for understanding kidney and liver involvement in orofaciodigital syndrome type 1.
PubMed | Toranomon Hospital Kajigaya and Toranomon Hospital
Type: Journal Article | Journal: PloS one | Year: 2016
Reduced estimated glomerular filtration rate (eGFR) and proteinuria are risk factors for end-stage renal disease (ESRD), of which benign nephrosclerosis is a common cause. However, few biopsy-based studies have assessed these associations.We performed retrospective cohort study of 182 Japanese patients who underwent renal biopsy from June 1985 through March 2014 and who were diagnosed with benign nephrosclerosis. Competing risk regression analyses were used to investigate the effect of eGFR and proteinuria levels at the time of renal biopsy on the risk for renal events (ESRD or a 50% decline in eGFR from baseline).During a median 5.8-year follow-up, 63 (34.6%) patients experienced renal events. The incidence of renal events increased with lower baseline eGFR and greater baseline proteinuria levels. After adjustment for baseline covariates, lower eGFR levels (subhazard ratios [SHRs], 1.30; 95% confidence interval [CI], 1.01-1.67, per 10 mL/min/1.73 m2) and higher proteinuria levels (SHR, 1.52; 95% CI, 1.23-1.87, per 1.0 g/day) at the time of renal biopsy were associated independently with higher risk for renal events. Lower levels of serum albumin (SHR, 2.07; 95% CI, 1.20-3.55 per 1.0 g/dL) were also associated with renal events. Patients with both eGFR <30 mL/min/1.73 m2 and proteinuria 0.5 g/day had a 26.7-fold higher risk (95% CI, 3.97-179.4) of renal events than patients with both eGFR 60 mL/min/1.73 m2 and proteinuria <0.5 g/day.Reduced eGFR and increased proteinuria as well as lower serum albumin at the time of renal biopsy are independent risk factors for renal events among patients with biopsy-proven benign nephrosclerosis.
PubMed | Toranomon Hospital Kajigaya and Toranomon Hospital
Type: Journal Article | Journal: Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA | Year: 2016
Once-weekly 56.5-g teriparatide treatment was significantly associated with the increase in lumbar spine bone mineral density at 48 weeks among hemodialysis patients with hypoparathyroidism and low bone mass; however, discontinuation of treatment because of adverse events was frequently observed. Careful monitoring for adverse events should be required.Once-weekly 56.5-g teriparatide is reportedly effective for treating osteoporotic patients without renal insufficiency. However, little is known about the efficacy and safety of once-weekly teriparatide in hemodialysis patients.We conducted a 48-week prospective, observational cohort study including 22 hemodialysis patients aged 20 years or older with hypoparathyroidism and low bone mass who received once-weekly teriparatide at 56.5 g at a tertiary care hospital between January 2013 and January 2015. Primary outcomes were within-subject percent changes of bone mineral density (BMD) at the lumbar spine, femoral neck, and distal one-third radius at 24 and 48 weeks. Secondary outcomes included percent changes of serum bone turnover markers (osteocalcin, bone-specific alkaline phosphatase (BAP), N-terminal propeptide of procollagen type 1 (P1NP), and tartrate-resistant acid phosphatase 5b (TRAP-5b)). Adverse events were evaluated.The BMD increased at the lumbar spine by 3.31.9 % (meanSEM) and 3.01.8 % at 24 and 48 weeks but not in the femoral neck and distal one-third radius. Serum osteocalcin, BAP, and P1NP increased significantly at 4 weeks, maintaining higher concentrations up to 48 weeks, although TRAP-5b decreased gradually during treatment. The baseline BAP was significantly associated with the 48-week percent change in lumbar spine BMD. Transient hypotension was the most common adverse event. Ten patients discontinued treatment because of adverse events.Once-weekly teriparatide was associated with increased lumbar spine BMD in hemodialysis patients with hypoparathyroidism and low bone mass. Careful monitoring should be required for treatment of such patients.
PubMed | Toranomon Hospital Kajigaya, International University of Health and Welfare and Toranomon Hospital
Type: Journal Article | Journal: BMC nephrology | Year: 2016
The purpose of this study was to investigate the usefulness of intracystic MRI features for detection of severe cyst infection that is usually refractory to antibiotic therapy alone in patients with autosomal dominant polycystic kidney disease.Seventy-six patients (88 episodes) with positive cyst cultures treated from January 2006 to December 2013 were enrolled as the cases for this case-control study, while 147 patients who continued to attend our hospital from January 2011 to December 2013 and did not have cyst infection diagnosed during that period were enrolled as the controls. Intracystic MRI findings were investigated.At least one of four intracystic MRI features (high signal intensity (SI) on diffusion-weighted images (DWI), fluid-fluid level, wall thickening, or gas) was found in all of the cases, but such findings were also detected in some controls. Intracystic gas was specific for cyst infection, but its sensitivity was only 1.1%. A high intracystic SI on DWI showed a sensitivity of 86.4%, but its specificity was lower at 33.3%. Both the specificity and sensitivity of a fluid-fluid level or wall thickening were about 80%. However, the specificity of these MRI features decreased as total liver and kidney volume (TLKV) increased, falling to 65.8% in patients with organomegaly (TLKV>8500cmMRI with DWI was useful for detecting severe cyst infection in ADPKD. While the specificity of MRI alone was not high enough in patients with organomegaly, combining the four MRI features with abdominal pain, sequential MRI changes, or cyst diameter>5cm improved detection of severely infected cysts in these patients.
PubMed | National Hospital Organization Kumamoto Medical Center, Heart Life Hospital, Sasebo City General Hospital, University of Ryukyus and 11 more.
Type: Clinical Trial | Journal: Bone marrow transplantation | Year: 2016
Allogeneic hematopoietic SCT (allo-HSCT) is a curative treatment for aggressive adult T-cell leukemia/lymphoma (ATLL). Considering the dismal prognosis associated with conventional chemotherapies, early application of allo-HSCT might be beneficial for patients with ATLL. However, no previous study has addressed the optimal timing of allo-HSCT from related donors. Hence, to evaluate the impact of timing of allo-HSCT for patients with ATLL, we retrospectively analyzed data from patients with ATLL who received an allo-HSCT from a related donor. The median age was 52 years. Patients were grouped according to the interval from diagnosis to allo-HSCT: early transplant group, <100 days, n=72; late transplant group, 100 days, n=428. The corresponding constituents of disease status were not statistically different between the two groups (P=0.11). The probability of OS in the early transplant group was significantly higher than that in the late transplant group (4-year OS, 49.3% vs 31.2%). Multivariate analysis revealed that late allo-HSCT was an unfavorable prognostic factor for OS (hazard ratio, 1.46; 95% confidence interval (CI), 1.01-2.11; P=0.04). Despite the limitations of a retrospective study, it might be acceptable to consider early application of allo-HSCT for ATLL.
Nakamura K.,CEA Saclay Nuclear Research Center |
Nakamura K.,Yokohama Stroke Center |
Oga T.,Toranomon Hospital Kajigaya |
Takahashi M.,Yokohama Stroke Center |
And 5 more authors.
Cortex | Year: 2012
Hemispheric rivalry models of spatial neglect suggest that the left hemisphere becomes hyperactive following right-hemisphere lesions since the two hemispheres normally exert an inhibitory influence on each other via callosal connections. Using a masked hemifield priming paradigm, we investigated whether the putative change in hemispheric balance involves other, higher-order abstract representational systems in spatial neglect. Participants consisted of 12 neglect patients with right-hemisphere damage and three groups of control participants, i.e., 12 young healthy controls, 10 age-matched healthy controls and 10 right-hemisphere patients without spatial neglect. In each trial, participants made semantic categorization about a centrally presented target word which was preceded by a masked prime flashed either to the left or right visual field. All three control groups exhibited strong left-hemisphere advantage in inhibitory syllabic priming, consistent with the known left-hemisphere dominance in lexical inhibition during reading. By contrast, neglect patients exhibited a symmetrical pattern of priming between the left and right visual fields. These results suggest that (1) the neglected hemifield can rapidly extract abstract information even from weak and normally non-perceptible visual stimuli, but that (2) the normal left hemispheric dominance in reading is absent in neglect patients probably because of the generalized hyperactivity of the left hemisphere. Our results demonstrate a covert behavioral change in spatial neglect which may reflect the altered inter-hemispheric balance in the bilateral word recognition system encompassing lexico-semantic memory. © 2010.
Marui Y.,Toranomon Hospital Kajigaya |
Nakamura M.,Toranomon Hospital Kajigaya |
Tanaka K.,Toranomon Hospital Kajigaya |
Tomikawa S.,Toranomon Hospital Kajigaya
Transplantation Proceedings | Year: 2012
Background: To achieve Patient Safety and minimal operative invasion in living kidney donor nephrectomy, we have performed hand-assisted laparoscopic donor nephrectomy (HALDoN) since 2006. Aim: The aim of this study was to evaluate the utility and the technique of HALDoN. Method: We analyzed 72 donors who underwent HALDoN from February 2008August 2011. Results: Including 8/72 donors who underwent right nephrectomy, all subjects completed HALDoN without conversion to an open procedure. None of the recipients suffered delayed graft function or an ureteric problem. Knife-to-removal time (KRT) was longer among cases with graft weight (GW) >200 g than GW ≤200 g: 176.5 ± 35.1 minutes vs 142 ± 18.7 minutes (P <.001). Longer KRT (>180 minutes) and right nephrectomy produced longer reperfusion-to-urine secretion time (RUT; P =.002 and P =.027, respectively). Grafts with double renal arteries (N = 10) also tended to show longer RUT (P =.058). In a case with an early renal arterial branch <1 cm from the aorta, we transected the vessel to achieve a single orifice of the artery using a stapling device. At 6 months the average value of decreased renal function of donors had recovered to about 70%. The incidence of complication was 8.3% but there was no life-threatening morbidity. Conclusion: The hand-assisted method could make the operating surgeon more confident to perform laparoscopic donor nephrectomy safely. HALDoN offers particular advantages for precise dissection using finger retraction and control of potential bleeding in the stages of vascular stapling and graft removal, preserving graft viability. © 2012 Published by Elsevier Inc.
PubMed | Toranomon Hospital Kajigaya
Type: Journal Article | Journal: Internal medicine (Tokyo, Japan) | Year: 2016
A 57-year-old Japanese woman with a 5-year history of rheumatoid arthritis (RA) was admitted to our hospital for an evaluation of nephrotic range proteinuria (4.8 g/day). A renal biopsy led to the diagnosis of amyloidosis according to strong positivity for Congo red staining and the detection of microfibrillar structures on electron microscopy that were negative for AA and positive for kappa light chain. Combination therapy with high-dose melphalan and autologous stem cell transplantation was performed according to the regimen for AL amyloidosis. Her proteinuria and RA subsided, but relapsed after 3 years. This is the first report regarding kappa light chain amyloidosis in an RA patient.