GLORIA

GEOMAR Library Ocean Research Information Access

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Online Resource
    Online Resource
    Association for Research in Vision and Ophthalmology (ARVO) ; 2023
    In:  Translational Vision Science & Technology Vol. 12, No. 5 ( 2023-05-24), p. 25-
    In: Translational Vision Science & Technology, Association for Research in Vision and Ophthalmology (ARVO), Vol. 12, No. 5 ( 2023-05-24), p. 25-
    Type of Medium: Online Resource
    ISSN: 2164-2591
    Language: English
    Publisher: Association for Research in Vision and Ophthalmology (ARVO)
    Publication Date: 2023
    detail.hit.zdb_id: 2674602-5
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 2
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 35, No. Supplement_3 ( 2020-06-01)
    Abstract: Kidneys are often damaged in paraproteinemic conditions. Paraproteins are monoclonal immunoglobulins or immunoglobulin fractions that are produced by a clonal population of B- or plasma cell lineage and can cause a variety of histological patterns of kidney injury, such as light chain (AL) amyloidosis or light chain cast nephropathy (LCCN). Monoclonal gammopathy of renal significance (MGRS) represents a group of disorders in which a monoclonal immunoglobulin secreted by B- or plasma cell clone causes renal damage. By definition, these disorders do not meet diagnostic criteria for overt, symptomatic multiple myeloma or a lymphoproliferative disorder, but in contrast to monoclonal gammopathy of undetermined significance (MGUS) there is evidence of end-organ damage that can warrant therapy. Method All patients with paraproteinemic kidney disease were identified by retrospective review of the Hospital Register of kidney biopsies done at Department of Nephrology and Dialysis, in Dubrava University Hospital, Zagreb, from 2009 until 2018. Every kidney biopsy was analyzed by light, immunofluorescent and electron microscopy. Laboratory findings, including serum protein electrophoresis, serum free light chain level and immunofixation of serum proteins, were done for every patient. Clinical and histologic features of patients and features of underlying hematological conditions were analyzed. Results We identified 47 patients (3,28% of all biopsies that were done in that period) with kidney disease with clear hematological background. The mean patients' age at the time of the biopsy was 63 years and 27 of them were females. Two patients had signs of direct infiltration of kidneys with malignant lymphomic cells (non-Hodgkin lymphoma) and were excluded from the analysis. Clinical presentation of the patients at the time of biopsy were: proteinuria in 85% of patients, full nephrotic syndrome in 55%, azotemia in 66% of patients (80% had acute kidney injury of unclear etiology) and hematuria in 12,7%. Most common histologic patterns of kidney injury were AL amyloidosis (45%) and LCCN (30%) but additionally 7 different histological patterns were found: light chain depostion disease, light chain proxymal tubulopathy, fibrillary and imunotactoid glomerulopathy, proliferative glomerulonephritis with monoclonal immunoglobulin deposition, crioglobulinemic glomerulopathy type I and tubulointerstitial damage caused by immunoglobulin deposition. Figure 1 shows main features of patients with AL amyloidosis and cast LCCN. Conclusion Kidney disease can be initial presentation of an underlying paraproteinemia and, as our data showed, can clinicaly present with acute kidney injury, nephrotic or subnephrotic range proteinuria or full nephrotic syndrome. Variety of histologic patterns of kidney injury were described and AL amyloidosis and LCCN were the most common histological findings. Detailed hematologic workup should follow kidney biopsy in order to determine the exact nature and extension of the disease and therefore the most appropriate therapy.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 3
    In: Clinical Kidney Journal, Oxford University Press (OUP), Vol. 16, No. 7 ( 2023-06-30), p. 1183-1185
    Type of Medium: Online Resource
    ISSN: 2048-8505 , 2048-8513
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2023
    detail.hit.zdb_id: 2656786-6
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 4
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 37, No. Supplement_3 ( 2022-05-03)
    Abstract: Rituximab (RTX) showed some efficacy in treatment of minimal change disease (MCD) and primary focal segmental glomerulosclerosis (pFSGS) [1]. The results of its off-label use and as steroid-sparing agent were especially promising in steroid-dependent, frequently relapsing or steroid-resistant forms of MCD and pFSGS in adults [2] . METHOD All patients treated with RTX in Department of Nephrology and Dialysis, University Hospital Dubrava, Zagreb, were retrospectively reviewed. Complete remission (CR), steroid-resistant nephrotic syndrome (SRNS), steroid-dependent nephrotic syndrome (SDNS), multidrug-resistant SRNS (MDR-SRNS) and calcineurin inhibitor (CNI)-resistant SRNS were defined according to KDIGO 2021 [3]. RESULTS Of 82 patients treated with RTX, 11 patients had biopsy proven MCD and pFSGS. Table 1 summarizes relevant demographic, clinical and treatment data. RTX was applied in five patients with SDNS, four patients with MDR-SRNS, one patient with CNI-resistant SRNS and one patient with active hepatitis C and newly onset MCD as a first line therapy. Median follow-up after initial diagnosis and RTX application was 122.9 (range 30–219) and 20 (range 3–117) months, respectively. All patients were initially treated with steroids, and RTX was the drug most commonly applied after treatment with CNI and cyclophosphamide (CYC). Median proteinuria at the time of RTX application was 10 g/day (range 2.5–16.7), 10 patients had preserved kidney function and only 1 patient with collapsing FSGS (case 11) had severe acute kidney injury. Mean cumulative dose of RTX for induction was 1700 mg and short course of steroids was used concomitantly in 10 cases (except case 5). CR was achieved in seven cases (six cases of MCD and one case of MDR-FSGS). Three of seven patients with CR to RTX experienced relapse of disease. Time to relapse was 6, 15 and 40 months in case 5, 3 and 11, respectively. Repeated RTX application led to CR in all three cases. CR persisted in five patients even after the steroids cessation. There were no serious adverse effects of RTX administration in our cohort. CONCLUSION RTX showed respectable efficacy in our cohort of adults with SDNS-MCD and in case of MDR collapsing FSGS. Although statistical comparison was not performed because of small number of patients, it seems that RTX is more effective in group of SDNS than MDR-SRNS. In our cohort, patients with relapse of RTX-sensitive disease responded to repeated application. Role of RTX in treatment of MCD and pFSGS should be evaluated in randomized clinical trials.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 5
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 34, No. Supplement_1 ( 2019-06-01)
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2019
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 6
    In: Kidney International Reports, Elsevier BV, ( 2024-4)
    Type of Medium: Online Resource
    ISSN: 2468-0249
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2024
    detail.hit.zdb_id: 2887223-X
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 7
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 36, No. Supplement_1 ( 2021-05-29)
    Abstract: After membranous nephropathy, focal segmental glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome in European population. According to Columbia classification, there are five histological variants of FSGS defined on light microscopy (tip, cellular, perihilar, collapsing and not otherwise specified - NOS) and this classification has a prognostic significance. The aim is to compare features and outcomes of tip and cellular variant of primary FSGS. Method All patients with FSGS were identified by a retrospective review of the Registry of kidney biopsies at the Department of Nephrology and Dialysis, Dubrava University Hospital, Zagreb, from 2003 until 2020. Each kidney specimen was analyzed by light, immunofluorescent and electron microscopy and Columbia classification was applied by experienced nephropathologist. Patients with primary FSGS met following criteria: full nephrotic syndrome and diffuse podocyte foot process effacement in absence of secondary causes of FSGS. Laboratory findings were obtained for every patient at the time of biopsy and following outpatient visits. Complete remission was defined as proteinuria & lt; 0.3 g/day with normal kidney function and partial remission as proteinuria 0.3 - 3.5 g/day. Variables are expressed as median ± IQR (interquartile range) and frequencies. Statistical comparison between groups of patients with tip and cellular variant of primary FSGS and disease outcome analysis were done. Results Out of 200 patients with FSGS, 59 (29.5 %) had primary form of disease. Tip variant was the most common form of primary FSGS (22 patients, 37 %) followed by NOS (20, 34 %), cellular (13, 22 %), perihilar (2, 3.5 %) and collapsing (2, 3.5 %) variant. Demographic and clinical features with initial laboratory findings are shown in Table 1. There were no significant differences between two groups in all analyzed variables in Figure 1. All patients were treated by anti-RAAS agents and steroids. Median follow-up was 55 months (range 1 – 196 months), and followup data were unavailable for three patients. Figure 2 shows treatment regimens in both patient grouos with treatment outcomes. Remission rate was significantly higher in tip variant (90 % vs. 41 %, p = 0.002). There was no difference in relapse rate between the two groups (p = 0.717). Conclusion There were no significant differences in clinical features and laboratory findings at the time of clinical presentation between tip and cellular variant of primary FSGS. Patients with tip variant had significantly higher remission rate than patients with cellular variant.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 8
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2019
    In:  Nephrology Dialysis Transplantation Vol. 34, No. Supplement_1 ( 2019-06-01)
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 34, No. Supplement_1 ( 2019-06-01)
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2019
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 9
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2019
    In:  Nephrology Dialysis Transplantation Vol. 34, No. Supplement_1 ( 2019-06-01)
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 34, No. Supplement_1 ( 2019-06-01)
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2019
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
  • 10
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 35, No. Supplement_3 ( 2020-06-01)
    Abstract: The aim of the research was to evaluate patient and renal as well as relapse free survival in ANCA associated vasculitis (AAV) patients in our center. Despite the advances in understanding pathogenesis of AAVs and advances in treatment, the outcomes of AAV patient differ in various centers. Method This study included 106 consecutive AAV patients with renal involvement in the period from 2007-2017. We performed renal biopsy on patients using automatic 16 Gauge needle. Light, immunofluorescent and electronic microscopy were performed. All the patients were treated with cyclophosphamide and steroids in induction treatment with adjuvant PLEX and dialysis depending on renal function and lung manifestations. Primary outcomes were combined outcome progression to end-stage renal disease, defined as persistent (more than three months) need for renal replacement therapy or permanent reduction of EGFR to & lt;15ml/minute (according to CKD EPI formula) and/or death (ESRDD), death (D) and ESRD alone, and disease relapse. Kaplan Meyer survival analysis and multivariate Cox proportional hazard regression analysis were used to explore difference between phenotypes and finding significant predictors regarding outcomes. Out of 106 patients (55,6% female, median age 61; IQR 51-70) there were 66 (61,1%) microscopic poliangitiis (MPA), 20 (18,5%) granulomatosis with angitiis and 20 (18,5%) with renal limited vasculitis (RLV),There were 14 (13%) PR3-ANCA positive patients, 57 (52,8%) MPO ANCA positive, 5 (4,6%) PR3-ANCA+MPO-ANCA positive and 32 (29,6%) ANCA negative patients. Histologically (Berden classification) 43 (39,8%) patients had crescentic, 19 (17,6%) focal, 34 (31,5%) mixed and 12 (11,1%) sclerotic class. Follow up time ranged from 1 to 127 months. Median follow up time was 21 months (IQR = 7-44). Median time to diagnosis was 3 months (IQR 2,0-6,0). Results During follow up 21 (19,8%) patients died, 26 (24,5%) patients reached ESRD and 10 (9,4%) patients relapsed. There was no significant difference in outcomes between clinical, serological or histological phenotypes. In multivariant analysis independent predictors for death were age (HR = 1,059, 95% CI =1,001-1,120; p = 0,046), anemia (HR = 0,952, 95% CI =0,908-0,998; p = 0,040) and BVAS (HR = 1,093, 95% CI =1,030-1,159; p = 0,003), for ESRD. the need for acute dialysis (HR = 4,674, 95% CI =1,996-10,946; p = & lt; 0,001), and interstitial fibrosis and tubular atrophy (IFTA) percentage over 50% (HR = 2,652, 95% CI =1,157-6,081; p = 0,021). and for relapse rate younger age (HR = 0,924, 95% CI = 0,870-0,981; p =0,010), lower serum creatinine levels (HR = 0,996, 95% CI = 0,992-1,000; p = 0,033), and the need for acute dialysis (HR = 59,545, 95% CI =3,467-1022,665; p = 0,005). Event free survival after 12, 24, 36 and 60 months was for death 83,9, 81,2, 79 and 74,7%, for ESRD 80,6, 77,9, 76,1 and 71% and for relapse 95,3, 88,4, 88,4 and 85%. Conclusion Timely diagnosis and treatment can ensure better outcomes in AAV patients. Though there is an overlap in predictive factors between different cohorts, there are still distinctive differences especially between cohorts from clinical trials and those from observational studies. Our study is among few to show significance of anemia as clinical predictor and IFTA percentage as pathohistological predictor.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...