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1.
Sci Rep ; 13(1): 6980, 2023 04 28.
Article in English | MEDLINE | ID: mdl-37117201

ABSTRACT

Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are glomerulopathies associated with nephrotic syndrome. Primary forms of these diseases are treated with various regimes of immunosuppression. Frequently relapsing or glucocorticoid-dependent courses remain challenging. Here, a B-cell-depleting strategy with rituximab represents a salvage option although data are sparse in the adult population. In particular, there is limited evidence on the efficacy of restoring remission after initial successful treatment with rituximab and whether patients benefit from an individualized, relapse-based approach. We identified 13 patients who received multiple therapies with rituximab from the FOrMe-registry (NCT03949972), a nationwide registry for MCD and FSGS in Germany, or from the University Hospital of Cologne. Disease status, changes in serum creatinine, proteinuria, and time to relapse were evaluated. Relapse-free survival was compared to the patients' previous therapy regimens. Through all treatment cycles, an improvement of disease activity was shown leading to a complete remission in 72% and partial remission in 26% after 3 ([Formula: see text]0.001) and 6 months ([Formula: see text]0.001). Relapse-free survival increased from 4.5 months (95%-CI 3-10 months) to 21 months (95%-CI 16-32 months) ([Formula: see text]0.001) compared to previous immunosuppression regimens with no loss in estimated glomerular filtration over time (p = 0.53). Compared to continuous B-cell depletion, an individualized relapse-based approach led to a reduced rituximab exposure and significant cost savings. Relapse-based administration of rituximab in patients with MCD/FSGS with an initial good clinical response did not result in a decreased efficacy at a median follow-up duration of 110 months. Thus, reinduction therapies may provide an alternative to continuous B-cell-depletion and reduce the long-term side effects of continuous immunosuppression.


Subject(s)
Glomerulosclerosis, Focal Segmental , Nephrosis, Lipoid , Nephrotic Syndrome , Adult , Humans , Rituximab , Glomerulosclerosis, Focal Segmental/complications , Nephrosis, Lipoid/complications , Nephrotic Syndrome/drug therapy , Proteinuria/complications , Recurrence
3.
Aging Clin Exp Res ; 32(1): 115-124, 2020 Jan.
Article in English | MEDLINE | ID: mdl-30911909

ABSTRACT

BACKGROUND: Geriatric syndromes (GS) do not fit into discrete disease categories and are often underdiagnosed in hospitalized older adults. Geriatric resources (GR) are also not routinely collected in clinical settings, although this may potentiate the beneficial effects of clinical decisions. The prognostic relevance of GS and GR has never been systematically evaluated through clinical tools developed for clinical decision purposes. AIM: To ascertain the impact of common GS and GR on patients' prognosis as assessed by means of the comprehensive geriatric assessment (CGA)-based Multidimensional Prognostic Index (MPI). METHODS: One hundred and thirty-five hospitalized patients aged 70 years and older underwent a CGA evaluation with calculation of the MPI on admission and discharge. Accordingly, patients were subdivided in low (MPI-1, score 0-0.33), moderate (MPI-2, score 0.34-0.66), and severe (MPI-3, score 0.67-1)-risk of mortality at 1 month and 1 year. Nine GR and 17 GS were identified and collected accordingly. RESULTS: A lower number of GS and a higher number of GR were shown to be highly significantly correlated with a lower MPI, as well as years of education, grade of care, and number of medications independent of age, sex and number of GS or GR. Underweight and obesity according to the BMI were significantly correlated to higher number of GS. Patients with more GR had a significantly higher chance of being discharged home. CONCLUSIONS: The MPI evaluation together with GS and GR in acute care for older patients should be encouraged to improve clinical decision-making.


Subject(s)
Aging , Geriatric Assessment/methods , Resilience, Psychological , Socioeconomic Factors , Aged , Aged, 80 and over , Aging/physiology , Aging/psychology , Female , Hospitalization/statistics & numerical data , Humans , Male , Multiple Chronic Conditions , Syndrome
4.
Dtsch Med Wochenschr ; 144(22): 1572-1575, 2019 11.
Article in German | MEDLINE | ID: mdl-31658481

ABSTRACT

HISTORY: A 42-year-old female patient presented with pyelonephritis. Two days later she complained of hematomas and petechia of the extremities. DIAGNOSIS/CLINICAL FINDINGS: Acquired thrombotic thrombocytopenic purpura was diagnosed. Laboratory result showed a thrombotic microangiopathy and thrombocytopenia of 8 × 10E9/l. ADAMTS13-activity was reported to be reduced and inhibitory antibodies to be present. THERAPY AND COURSE: The patient was placed on a therapy with plasma exchange, high-dose steroids, and caplacizumab based on a positive PLASMIC score. Once the diagnosis was confirmed by diminished ADAMTS13-activity and the presence of autoantibodies, rituximab was added. The patient responded well but showed persistently elevated levels of ADAMTS13-autoantibodies, which lead to an extension of Caplacizumab treatment to 58 days. DISCUSSION: Caplacizumab represents a novel effective treatment option for patients with acquired thrombocytopenic purpura. Time to treatment response as well as the risk of relapse were shown to be significantly reduced. However, persistent autoimmune activity can be demonstrated in a significant portion of the patients after the end of treatment putting them at risk for relapses. Thus, careful and frequent surveillance is required.


Subject(s)
Purpura, Thrombotic Thrombocytopenic , ADAMTS13 Protein/metabolism , Adult , Autoantibodies/blood , Female , Fibrinolytic Agents/therapeutic use , Humans , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/metabolism , Purpura, Thrombotic Thrombocytopenic/physiopathology , Purpura, Thrombotic Thrombocytopenic/therapy , Single-Domain Antibodies/therapeutic use
5.
Dtsch Med Wochenschr ; 144(6): 376-381, 2019 03.
Article in German | MEDLINE | ID: mdl-30870867

ABSTRACT

Edema is a common symptom with mostly extrarenal causes. Nevertheless, it may also be a sign of glomerular disease. So renal causes should always be clarified to avoid serious complications, such as renal insufficiency, cardiovascular or thrombembolic events. The determination of the albumin-creatinine ratio in spot urine is easy to perform and the method of choice to diagnose nephrotic-range proteinuria. Renal biopsy then usually allows the definitive assignement to either glomerular or systemic disease. Salt restriction in combination with diuretics and antiproteinuric treatment are essential for an effective therapy. Depending on the underlying disease and individual risk factors, anticoagulation should also be considered.


Subject(s)
Edema , Kidney Diseases , Humans , Nephrologists , Renal Insufficiency
6.
Z Gerontol Geriatr ; 52(5): 460-467, 2019 Aug.
Article in English | MEDLINE | ID: mdl-30406302

ABSTRACT

BACKGROUND: The multidimensional prognostic index (MPI) is a validated, sensitive, and specific prognosis estimation tool based on a comprehensive geriatric assessment (CGA). The MPI accurately predicts mortality after 1 month and 1 year in older, multimorbid patients with acute disease or relapse of chronic conditions. OBJECTIVE: To evaluate whether the MPI predicts indicators of healthcare resources, i.e. grade of care (GC), length of hospital stay (LHS) and destination after hospital discharge in older patients in an acute medical setting. MATERIAL AND METHODS: In this study 135 hospitalized patients aged 70 years and older underwent a CGA evaluation to calculate the MPI on admission and discharge. Accordingly, patients were subdivided in low (MPI­1, score 0-0.33), moderate (MPI-2, score 0.34-0.66) and high (MPI-3, score 0.67-1) risk of mortality. The GC, LHS and the discharge allocation were also recorded. RESULTS: The MPI score was significantly related to LHS (p = 0.011) and to GC (p < 0.001). In addition, MPI-3 patients were significantly more often transferred from other hospital settings (p = 0.007) as well as significantly less likely to be discharged home (p = 0.04) than other groups. CONCLUSION: The CGA-based MPI values are significantly associated with use of indicators of healthcare resources, including GC, LHS and discharge allocation. These findings suggest that the MPI may be useful for resource planning in the care of older multimorbid patients admitted to hospital.


Subject(s)
Activities of Daily Living , Geriatric Assessment/methods , Risk Assessment/methods , Aged , Female , Hospitalization , Humans , Male , Patient Admission , Patient Discharge , Predictive Value of Tests , Prognosis , Risk Factors
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