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  1. Article: [Kidney diseases and metabolic disorders--Basics and applications required for general physicians. Topics: I. Disorders of water balance and sodium metabolism].

    Ota, Tatsuru / Uchida, Shunya

    Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine

    2015  Volume 104, Issue 5, Page(s) 906–916

    MeSH term(s) Animals ; General Practitioners ; Humans ; Kidney Diseases/metabolism ; Kidney Diseases/therapy ; Metabolic Diseases/metabolism ; Metabolic Diseases/therapy ; Practice Guidelines as Topic ; Sodium/metabolism ; Water-Electrolyte Balance/physiology
    Chemical Substances Sodium (9NEZ333N27)
    Language Japanese
    Publishing date 2015-10-20
    Publishing country Japan
    Document type Journal Article ; Review
    ZDB-ID 952816-7
    ISSN 1883-2083 ; 0021-5384
    ISSN (online) 1883-2083
    ISSN 0021-5384
    DOI 10.2169/naika.104.906
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  2. Article ; Online: Time to target uric acid to retard CKD progression.

    Kumagai, Takanori / Ota, Tatsuru / Tamura, Yoshifuru / Chang, Wen Xiu / Shibata, Shigeru / Uchida, Shunya

    Clinical and experimental nephrology

    2017  Volume 21, Issue 2, Page(s) 182–192

    Abstract: Uric acid (UA) remains a possible risk factor of chronic kidney disease (CKD) but its potential role should be elucidated given a fact that multidisciplinary treatments assure a sole strategy to inhibit the progression to end-stage renal disease (ESRD). ... ...

    Abstract Uric acid (UA) remains a possible risk factor of chronic kidney disease (CKD) but its potential role should be elucidated given a fact that multidisciplinary treatments assure a sole strategy to inhibit the progression to end-stage renal disease (ESRD). In clinical setting, most observational studies showed that elevation of serum uric acid (SUA) independently predicts the incidence and the development of CKD. The meta-analysis showed that SUA-lowering therapy with allopurinol may retard the progression of CKD but did not reach conclusive results due to small-sized studies. Larger scale, randomized placebo-controlled trials to assess SUA-lowering therapy are needed. Our recent analysis by propensity score methods has shown that the threshold of SUA should be less than 6.5 mg/dL to abrogate ESRD. In animal models an increase in SUA by the administration of oxonic acid, uricase inhibitor, or nephrectomy can induce glomerular hypertension, arteriolosclerosis including afferent arteriolopathy and tubulointerstitial fibrosis. The ever-growing discoveries of urate transporters prompt us to learn UA metabolism in the kidney and intestine. One example is that the intestinal ABCG2 may play a compensatory role at face of decreased renal clearance of UA in nephrectomized rats, the trigger of which is not a uremic toxin but SUA itself. This review will summarize the recent knowledge on the relationship between SUA and the kidney and try to draw a conclusion when and how to treat asymptomatic hyperuricemia accompanied by CKD. Finally we will address a future perspective on UA study including a Mendelian randomization approach.
    MeSH term(s) Animals ; Biomarkers/blood ; Disease Progression ; Genetic Predisposition to Disease ; Gout Suppressants/therapeutic use ; Humans ; Hyperuricemia/blood ; Hyperuricemia/complications ; Hyperuricemia/drug therapy ; Kidney/drug effects ; Kidney/metabolism ; Kidney/physiopathology ; Mendelian Randomization Analysis ; Organic Anion Transporters/metabolism ; Randomized Controlled Trials as Topic ; Renal Insufficiency, Chronic/blood ; Renal Insufficiency, Chronic/etiology ; Renal Insufficiency, Chronic/physiopathology ; Renal Insufficiency, Chronic/prevention & control ; Risk Factors ; Uric Acid/blood
    Chemical Substances Biomarkers ; Gout Suppressants ; Organic Anion Transporters ; urate transporter ; Uric Acid (268B43MJ25)
    Language English
    Publishing date 2017-04
    Publishing country Japan
    Document type Journal Article ; Review
    ZDB-ID 1338768-6
    ISSN 1437-7799 ; 1342-1751
    ISSN (online) 1437-7799
    ISSN 1342-1751
    DOI 10.1007/s10157-016-1288-2
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  3. Article: Long-term efficacy of vildagliptin in patients with type 2 diabetes undergoing hemodialysis.

    Mera, Jun-Ichiro / Okada, Eiko / Okuda, Masumi / Ota, Tatsuru / Sibata, Shigeru / Uchida, Shunya

    Journal of diabetes and metabolic disorders

    2015  Volume 14, Page(s) 83

    Abstract: Background: There are few studies evaluating long-term glycemic control using a dipeptidyl peptidase-4 inhibitor in type 2 diabetes patients with end-stage renal disease (ESRD). The aim of this study was to evaluate the safety and efficacy of ... ...

    Abstract Background: There are few studies evaluating long-term glycemic control using a dipeptidyl peptidase-4 inhibitor in type 2 diabetes patients with end-stage renal disease (ESRD). The aim of this study was to evaluate the safety and efficacy of vildagliptin therapy over 2 years in type 2 diabetes with ESRD.
    Methods: Patients with ESRD resulting from type 2 diabetes requiring dialysis who had ≥20 % glycated albumin (GA) were enrolled. Vildagliptin 50 mg once daily was administered for 2 years. Changes in GA and dry weight were evaluated.
    Results: In 32 patients (24 men and 8 women) aged 68.3 ± 1.9 years, vildagliptin 50 mg once daily was administered for 2 years, but the dose was increased to 50 mg twice daily in 15 patients. GA was significantly reduced by 2.6 ± 0.6 %, from 22.4 ± 0.6 % at baseline to 19.8 ± 0.4 % at 2 years. After 2 years of vildagliptin therapy, 15 (46.9 %) of 32 patients achieved a GA level of <20 %. Dry weight changed slightly, with an increase of 1.3 ± 0.8 kg at 2 years. No adverse drug reactions related to treatment with vildagliptin were seen.
    Conclusions: Vildagliptin is a promising therapeutic option for safe, effective glycemic control in type 2 diabetic patients with ESRD.
    Language English
    Publishing date 2015-11-05
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2680289-2
    ISSN 2251-6581
    ISSN 2251-6581
    DOI 10.1186/s40200-015-0214-6
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  4. Article ; Online: Unique proximal tubular cell injury and the development of acute kidney injury in adult patients with minimal change nephrotic syndrome.

    Fujigaki, Yoshihide / Tamura, Yoshifuru / Nagura, Michito / Arai, Shigeyuki / Ota, Tatsuru / Shibata, Shigeru / Kondo, Fukuo / Yamaguchi, Yutaka / Uchida, Shunya

    BMC nephrology

    2017  Volume 18, Issue 1, Page(s) 339

    Abstract: Background: Adult patients with minimal change nephrotic syndrome (MCNS) are often associated with acute kidney injury (AKI). To assess the mechanisms of AKI, we examined whether tubular cell injuries unique to MCNS patients exist.: Methods: We ... ...

    Abstract Background: Adult patients with minimal change nephrotic syndrome (MCNS) are often associated with acute kidney injury (AKI). To assess the mechanisms of AKI, we examined whether tubular cell injuries unique to MCNS patients exist.
    Methods: We performed a retrospective analysis of clinical data and tubular cell changes using the immunohistochemical expression of vimentin as a marker of tubular injury and dedifferentiation at kidney biopsy in 37 adult MCNS patients. AKI was defined by the criteria of the Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guidelines for AKI.
    Results: Thirteen patients (35.1%) were designated with AKI at kidney biopsy. No significant differences in age, history of hypertension, chronic kidney disease, diuretics use, proteinuria, and serum albumin were noted between the AKI and non-AKI groups. Urinary N-acetyl-β-D-glucosaminidase (uNAG) and urinary alpha1-microglobulin (uA1MG) as markers of tubular injury were increased in both groups, but the levels were significantly increased in the AKI group compared with the non-AKI group. The incidence of vimentin-positive tubules was comparable between AKI (84.6%) and non-AKI (58.3%) groups, but vimentin-positive tubular area per interstitial area was significantly increased in the AKI group (19.8%) compared with the non-AKI group (6.8%) (p = 0.011). Vimentin-positive injured tubules with tubular simplification (loss of brush-border of the proximal tubule/dilated tubule with flattening of tubular epithelium) were observed in the vicinity of glomeruli in both groups, suggesting that the proximal convoluted tubules were specifically injured. Two patients exhibited relatively severe tubular injuries with vimentin positivity and required dialysis within 2 weeks after kidney biopsy. The percentage of the vimentin-positive tubular area was positively correlated with uNAG but not with uA1MG in the non-AKI group.
    Conclusions: Proximal tubular injuries with increased uNAG exist in MCNS patients without renal dysfunction and were more severe in the AKI group than they were in the non-AKI group. The unique tubular injuries probably due to massive proteinuria might be a predisposing factor for the development of severe AKI in adult MCNS patients.
    MeSH term(s) Acute Kidney Injury/etiology ; Acute Kidney Injury/metabolism ; Acute Kidney Injury/pathology ; Adult ; Aged ; Biomarkers/metabolism ; Female ; Humans ; Kidney Tubules, Proximal/chemistry ; Kidney Tubules, Proximal/metabolism ; Kidney Tubules, Proximal/pathology ; Male ; Middle Aged ; Nephrosis, Lipoid/complications ; Nephrosis, Lipoid/metabolism ; Nephrosis, Lipoid/pathology ; Retrospective Studies ; Vimentin/analysis ; Vimentin/biosynthesis
    Chemical Substances Biomarkers ; Vimentin
    Language English
    Publishing date 2017-11-28
    Publishing country England
    Document type Journal Article
    ZDB-ID 2041348-8
    ISSN 1471-2369 ; 1471-2369
    ISSN (online) 1471-2369
    ISSN 1471-2369
    DOI 10.1186/s12882-017-0756-6
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  5. Article ; Online: Clinical Presentation of Tubulointerstitial Nephritis Caused by Amyloid Light-chain Amyloidosis in a Patient with Sjögren's Syndrome.

    Inoue, Reiko / Fujigaki, Yoshihide / Kobayashi, Kana / Tamura, Yoshifuru / Ota, Tatsuru / Shibata, Shigeru / Ishida, Tsuyoshi / Kondo, Fukuo / Yamaguchi, Yutaka / Uchida, Shunya

    Internal medicine (Tokyo, Japan)

    2017  Volume 56, Issue 4, Page(s) 419–423

    Abstract: We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was ... ...

    Abstract We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins. Given the involvement in other organs, systemic AL amyloidosis was diagnosed. The patient underwent chemotherapy, but hemodialysis was ultimately instituted. It should be remembered that renal amyloidosis occurs as a clinical presentation of interstitial nephritis.
    MeSH term(s) Aged ; Amyloid/metabolism ; Amyloidosis/complications ; Amyloidosis/diagnosis ; Bence Jones Protein/urine ; Biopsy ; Female ; Humans ; Immunoglobulin lambda-Chains/urine ; Kidney/pathology ; Nephritis, Interstitial/etiology ; Nephritis, Interstitial/pathology ; Sjogren's Syndrome/complications
    Chemical Substances Amyloid ; Immunoglobulin lambda-Chains ; Bence Jones Protein (9006-99-9)
    Language English
    Publishing date 2017
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 32371-8
    ISSN 1349-7235 ; 0021-5120 ; 0918-2918
    ISSN (online) 1349-7235
    ISSN 0021-5120 ; 0918-2918
    DOI 10.2169/internalmedicine.56.7548
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  6. Article ; Online: Proteomics Approach Identifies Factors Associated With the Response to Low-Density Lipoprotein Apheresis Therapy in Patients With Steroid-Resistant Nephrotic Syndrome.

    Kuribayashi-Okuma, Emiko / Shibata, Shigeru / Arai, Shigeyuki / Ota, Tatsuru / Watanabe, Sumiyo / Hisaki, Harumi / Okazaki, Tomoki / Toda, Tosifusa / Uchida, Shunya

    Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy

    2016  Volume 20, Issue 2, Page(s) 174–182

    Abstract: Low-density lipoprotein apheresis (LDL-A) has been shown to reduce proteinuria in a subgroup of nephrotic syndrome patients refractory to immunosuppressive therapy. Factors influencing the efficacy of LDL-A in nephrotic syndrome are completely unknown. ... ...

    Abstract Low-density lipoprotein apheresis (LDL-A) has been shown to reduce proteinuria in a subgroup of nephrotic syndrome patients refractory to immunosuppressive therapy. Factors influencing the efficacy of LDL-A in nephrotic syndrome are completely unknown. Using a proteomics approach, we aimed to identify biological markers that predict the response to LDL-A in patients with steroid-resistant nephrotic syndrome (SRNS). Identification of plasma proteins bound to the dextran-sulfate column at the first session of LDL-A was determined by mass spectrometry. To investigate biological factors associated with the response to LDL-A, we compared profiles of column-bound proteins between responders (defined by more than 50% reduction of proteinuria after the treatment) and non-responders by 2-dimensional gel electrophoresis (2-DE) coupled to mass spectrometry in seven patients with SRNS. Evaluation of proteins adsorbed to LDL-A column in patients with SRNS revealed the identity of 62 proteins, which included apolipoproteins, complement components, and serum amyloid P-component (SAP). Comparative analysis of the column-bound proteins between responders and non-responders by 2-DE demonstrated that apolipoprotein E (APOE) and SAP levels were increased in non-responders as compared with responders. These results were confirmed by western blotting. Moreover, serum levels of APOE and SAP were significantly higher in the non-responder group than in the responder group by ELISA. Our data provide comprehensive analysis of proteins adsorbed by LDL-A in SRNS, and demonstrate that the serum levels of APOE and SAP may be used to predict the response to LDL-A in these patients.
    MeSH term(s) Adult ; Aged ; Apolipoproteins E/blood ; Blood Component Removal/methods ; Blood Proteins/metabolism ; Cholesterol, LDL/blood ; Dextran Sulfate/chemistry ; Electrophoresis, Gel, Two-Dimensional ; Enzyme-Linked Immunosorbent Assay ; Female ; Glucocorticoids/therapeutic use ; Humans ; Male ; Mass Spectrometry ; Middle Aged ; Nephrotic Syndrome/therapy ; Proteomics/methods ; Serum Amyloid P-Component/metabolism
    Chemical Substances Apolipoproteins E ; Blood Proteins ; Cholesterol, LDL ; Glucocorticoids ; Serum Amyloid P-Component ; Dextran Sulfate (9042-14-2)
    Language English
    Publishing date 2016-04
    Publishing country Australia
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2119809-3
    ISSN 1744-9987 ; 1091-6660 ; 1744-9979
    ISSN (online) 1744-9987
    ISSN 1091-6660 ; 1744-9979
    DOI 10.1111/1744-9987.12356
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  7. Article ; Online: Pathological implications of linear immunoglobulin G staining on the glomerular capillary walls in a case of infection-related glomerulonephritis.

    Fujigaki, Yoshihide / Kawamorita, Yosuke / Yamaguchi, Hiromi / Arai, Shigeyuki / Tamura, Yoshifuru / Ota, Tatsuru / Shibata, Shigeru / Kondo, Fukuo / Yamaguchi, Yutaka / Uchida, Shunya

    Pathology international

    2016  Volume 66, Issue 9, Page(s) 524–528

    Abstract: We report a 32-year-old man with nephrotic syndrome and preceding symptom of infection. He had renal insufficiency, hypocomplementemia, and elevated titer of anti-streptolysin O. Renal biopsy showed mesangial hypercellularity and focal segmental ... ...

    Abstract We report a 32-year-old man with nephrotic syndrome and preceding symptom of infection. He had renal insufficiency, hypocomplementemia, and elevated titer of anti-streptolysin O. Renal biopsy showed mesangial hypercellularity and focal segmental endocapillary hypercellularity with double contour of the glomerular basement membrane (GBM). Immunofluorescence study showed granular C3 staining on the mesangial areas and glomerular capillary walls (GCWs) and linear immunoglobulin G (IgG) staining on GCWs. Electron microscopy revealed sporadic subepithelial humps, discontinuous small and thin deposits in the endothelial side of the GBM and mesangial deposits. He was diagnosed with infection-related glomerulonephritis (IRGN) with the striking finding of linear IgG staining, which is unusual in IRGN. The patient did not have diabetes mellitus or anti-GBM disease. The patient's serum seemed not to contain IgG, which can bind to GCW. He showed normalization of complement within two months after relief from infection symptoms and a trend toward improvement in proteinuria, hematuria and renal function over 14 months. We discuss the possible mechanisms of linear IgG staining in our case based on clinical and experimental studies on IRGN with cationic bacterial protein as antigen.
    MeSH term(s) Adult ; Capillaries/immunology ; Capillaries/pathology ; Fluorescent Antibody Technique ; Glomerulonephritis/immunology ; Glomerulonephritis/pathology ; Humans ; Immunoglobulin G/immunology ; Kidney Glomerulus/immunology ; Kidney Glomerulus/pathology ; Male
    Chemical Substances Immunoglobulin G
    Language English
    Publishing date 2016-09
    Publishing country Australia
    Document type Case Reports
    ZDB-ID 1194850-4
    ISSN 1440-1827 ; 1320-5463
    ISSN (online) 1440-1827
    ISSN 1320-5463
    DOI 10.1111/pin.12441
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  8. Article ; Online: Time-dependent risk factors associated with the decline of estimated GFR in CKD patients.

    Chang, Wen-xiu / Arai, Shigeyuki / Tamura, Yoshifuru / Kumagai, Takanori / Ota, Tatsuru / Shibata, Shigeru / Fujigaki, Yoshihide / Shen, Zhong-yang / Uchida, Shunya

    Clinical and experimental nephrology

    2016  Volume 20, Issue 1, Page(s) 58–70

    Abstract: Background: Targeting the modifiable risk factors may help halt the progression of CKD, thus risk factor analysis is better performed using the parameters in the follow-up. This study aimed to examine the time-dependent risk factors for CKD progression ... ...

    Abstract Background: Targeting the modifiable risk factors may help halt the progression of CKD, thus risk factor analysis is better performed using the parameters in the follow-up. This study aimed to examine the time-dependent risk factors for CKD progression using time-averaged values and to investigate the characteristics of rapid progression group.
    Methods: This is a retrospective cohort study enrolling 770 patients of CKD stage 3-4. Time-dependent parameters were calculated as time-averaged values by a trapezoidal rule. % decline of estimated GFR (eGFR) per year from entry was divided to three groups: <10% (stable), 10-25% (moderate progression), and ≥25% (rapid progression). Multivariate regression analyses were employed for the baseline and the time-averaged datasets.
    Results: eGFR decline was 2.83 ± 4.04 mL/min/1.73 m(2)/year (8.8 ± 12.9 %) in male and 1.66 ± 3.23 mL/min/1.73 m(2)/year (5.4 ± 11.0%) in female (p < 0.001). % decline of eGFR was associated with male, proteinuria, phosphorus, and systolic blood pressure as risk factors and with age, albumin, and hemoglobin as protective factors using either dataset. Baseline eGFR and diabetic nephropathy appeared in the baseline dataset, while uric acid appeared in the time-averaged dataset. The rapid progression group was associated with proteinuria, phosphorus, albumin, and hemoglobin in the follow-up.
    Conclusion: These results suggest that time-averaged values provide insightful clinical guide in targeting the risk factors. Rapid decline of eGFR is strongly associated with hyperphosphatemia, proteinuria, and anemia indicating that these risk factors should be intervened in the follow-up of CKD.
    MeSH term(s) Adult ; Aged ; Aged, 80 and over ; Anemia/epidemiology ; Chi-Square Distribution ; Comorbidity ; Disease Progression ; Female ; Glomerular Filtration Rate ; Humans ; Hyperphosphatemia/epidemiology ; Japan/epidemiology ; Kidney/physiopathology ; Linear Models ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Prevalence ; Proteinuria/epidemiology ; Renal Insufficiency, Chronic/diagnosis ; Renal Insufficiency, Chronic/epidemiology ; Renal Insufficiency, Chronic/physiopathology ; Renal Insufficiency, Chronic/therapy ; Retrospective Studies ; Risk Factors ; Sex Factors ; Time Factors ; Young Adult
    Language English
    Publishing date 2016-02
    Publishing country Japan
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1338768-6
    ISSN 1437-7799 ; 1342-1751
    ISSN (online) 1437-7799
    ISSN 1342-1751
    DOI 10.1007/s10157-015-1132-0
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  9. Article ; Online: Remission of Refractory Ascites and Discontinuation of Hemodialysis after Additional Rituximab to Long-term Glucocorticoid Therapy in a Patient with TAFRO Syndrome.

    Tsurumi, Hanako / Fujigaki, Yoshihide / Yamamoto, Tadashi / Iino, Risa / Taniguchi, Kei / Nagura, Michito / Arai, Shigeyuki / Tamura, Yoshifuru / Ota, Tatsuru / Shibata, Shigeru / Kondo, Fukuo / Kurose, Nozomu / Masaki, Yasufumi / Uchida, Shunya

    Internal medicine (Tokyo, Japan)

    2018  Volume 57, Issue 10, Page(s) 1433–1438

    Abstract: Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized but rare disease, and its treatment has not yet been established. We reported a 50-year-old woman with TAFRO syndrome diagnosed 2 years ... ...

    Abstract Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized but rare disease, and its treatment has not yet been established. We reported a 50-year-old woman with TAFRO syndrome diagnosed 2 years after the initial symptoms of a fever, fatigue, epigastric pain, edema, ascites, lymphadenopathy, thrombocytopenia and renal insufficiency. The patient showed refractory ascites and required hemodialysis under corticosteroid mono-therapy for suspected immune-mediated disease but was successfully treated with additive rituximab, resulting in improvement in her laboratory data, the withdrawal of hemodialysis and the disappearance of ascites. This case underscores the therapeutic utility of rituximab in patients with corticosteroid-resistant TAFRO syndrome, even long after the onset of the disease.
    MeSH term(s) Ascites/drug therapy ; Drug Therapy, Combination ; Edema/diagnosis ; Edema/drug therapy ; Female ; Fever/diagnosis ; Glucocorticoids/therapeutic use ; Humans ; Immunologic Factors/therapeutic use ; Lymphadenopathy/diagnosis ; Lymphadenopathy/drug therapy ; Middle Aged ; Primary Myelofibrosis/diagnosis ; Primary Myelofibrosis/drug therapy ; Remission Induction ; Renal Dialysis ; Renal Insufficiency/diagnosis ; Renal Insufficiency/therapy ; Rituximab/therapeutic use ; Syndrome ; Thrombocytopenia/diagnosis ; Thrombocytopenia/drug therapy ; Withholding Treatment
    Chemical Substances Glucocorticoids ; Immunologic Factors ; Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2018-01-11
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 32371-8
    ISSN 1349-7235 ; 0021-5120 ; 0918-2918
    ISSN (online) 1349-7235
    ISSN 0021-5120 ; 0918-2918
    DOI 10.2169/internalmedicine.0116-17
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  10. Article ; Online: A patient presenting with isolated hematuria and renal dysfunction as rare manifestation of cryoglobulinemic glomerulonephritis in the course of autoimmune diseases including Sjögren's syndrome.

    Yamanaka, Masaki / Fujigaki, Yoshihide / Kono, Hajime / Nagura, Michito / Arai, Shigeyuki / Tamura, Yoshifuru / Ota, Tatsuru / Shibata, Shigeru / Kondo, Fukuo / Yamaguchi, Yutaka / Uchida, Shunya

    CEN case reports

    2018  Volume 7, Issue 2, Page(s) 211–216

    Abstract: Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition ... ...

    Abstract Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis. Before kidney biopsy, tubulointerstitial nephritis probably due to Sjögren's syndrome was suspected because of persistent hematuria without significant proteinuria and developing mild renal dysfunction over 6 months. The developing renal dysfunction associated with isolated hematuria is uncommon in glomerular diseases. Kidney biopsy, however, revealed established membranoproliferative glomerulonephritis with subendothelial deposits consisting of tubular structures with IgM, IgG, and C3 staining. Corticosteroids plus mycophenolate mofetil therapy successfully normalized renal function. Physician should not overlook cryoglobulinemic glomerulonephritis, which is potentially poor prognosis, even if urinalysis shows only persistent isolated hematuria in patients with autoimmune diseases.
    MeSH term(s) Adrenal Cortex Hormones/therapeutic use ; Aged ; Antibiotics, Antineoplastic/therapeutic use ; Autoimmune Diseases/complications ; Cryoglobulinemia/complications ; Cryoglobulinemia/drug therapy ; Cryoglobulinemia/pathology ; Female ; Glomerulonephritis/complications ; Glomerulonephritis/drug therapy ; Glomerulonephritis/pathology ; Glomerulonephritis, Membranoproliferative/complications ; Glomerulonephritis, Membranoproliferative/drug therapy ; Glomerulonephritis, Membranoproliferative/pathology ; Hashimoto Disease/complications ; Hematuria/diagnosis ; Hematuria/etiology ; Hepatitis, Autoimmune/complications ; Humans ; Kidney/immunology ; Kidney/pathology ; Kidney/physiopathology ; Methylprednisolone/administration & dosage ; Methylprednisolone/therapeutic use ; Mycophenolic Acid/administration & dosage ; Mycophenolic Acid/therapeutic use ; Nephritis, Interstitial/complications ; Pancytopenia/complications ; Pancytopenia/immunology ; Renal Insufficiency/complications ; Sjogren's Syndrome/complications ; Treatment Outcome
    Chemical Substances Adrenal Cortex Hormones ; Antibiotics, Antineoplastic ; Mycophenolic Acid (HU9DX48N0T) ; Methylprednisolone (X4W7ZR7023)
    Language English
    Publishing date 2018-04-18
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ISSN 2192-4449
    ISSN (online) 2192-4449
    DOI 10.1007/s13730-018-0329-y
    Database MEDical Literature Analysis and Retrieval System OnLINE

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