MO291RITUXIMAB IS EFFECTIVE AND SAFE IN ADULTS WITH STEROID-DEPENDENT NEPHROTIC SYNDROME: A LONG-TERM, SINGLE-CENTER EXPERIENCE

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Gemma Patella ◽  
Alessandro Comi ◽  
Giuseppe Coppolino ◽  
Nicolino Comi ◽  
Giorgio Fuiano ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Case Series ◽  
Adult Patients ◽  
Single Center ◽  
Single Center Experience ◽  
Steroid Dependent ◽  
The Mean ◽  
Steroid Dependent Nephrotic Syndrome

Abstract Background and Aims Steroid-dependent nephrotic syndrome (SDNS) may require a prolonged multi-drug therapy with risk of drug toxicity and renal failure. Rituximab (RTX) treatment has been found to be helpful in reducing the steroid dosage and the need for immunosuppressants (ISs), but little data are currently available regarding very long-term outcomes in adults. We herein describe a long-term, single-center experience of RTX use in a large series of adults with SDNS. Method We studied 23 adult patients with SDNS (mean age 54.2±17.1 y; 65% male; BMI 28.5±4.7), mostly consequent to membranous (47.8%) or focal glomerulonephritis (30.2 %) who were eligible to start a RTX regimen. Before entering the RTX protocol, proteinuria and eGFR were 7.06±3.87 g/24h and 65.9±28.2 ml/min/1.73 m2, respectively; albumin and CD19/CD20 ratio were 2.9±0.9 g/L and 0.99±0.01 respectively; the mean number of ISs was 2.39±0.89 and the mean annual rate of relapses was 2.2±0.9. Results Patients were followed over a mean follow-up of 64 months (range: 12-144). After RTX (mean dose: 1202.1±372.4 mg) the rate of relapses was virtually nullified (p<0.001). eGFR remained roughly stable (62.1±19.8 ml/min/1.73 m2, p=NS), while proteinuria, albumin, CD19/CD20 and BMI all significantly improved (p ranging from 0.01 to 0.001). The mean number of additional ISs was also reduced (0.44±0.12; p<0.001) and RTX enabled discontinuation of steroids in 13/23 (56.5%) patients. No major adverse events related to therapy were recorded. Conclusion Findings from this large case-series with a remarkable very long follow-up reinforce the role of RTX as an efficient and safe weapon to improve outcomes in adult patients suffering from SDNS.

Clinical evaluation of chronic nephrotoxicity of long-term cyclosporine A treatment in adult patients with steroid-dependent nephrotic syndrome

Nephrology ◽  
2011 ◽  
Vol 16 (3) ◽  
pp. 319-325 ◽  
Author(s):  
TOMONARI OKADA ◽  
HIROSHI MATSUMOTO ◽  
YUME NAGAOKA ◽  
RYO TOMARU ◽  
HIDEAKI IWASAWA ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Evaluation ◽  
Cyclosporine A ◽  
Adult Patients ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome

Encephaloduroarteriosynangiosis for Pediatric Moyamoya Disease: A Single-Center Experience With 67 Cases in China

2018 ◽  
Vol 33 (14) ◽  
pp. 901-908 ◽  
Author(s):  
Chengjun Wang ◽  
Meng Zhao ◽  
Jia Wang ◽  
Shuo Wang ◽  
Dong Zhang ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Pediatric Patients ◽  
Positive Impact ◽  
Case Series ◽  
Stroke Recurrence ◽  
Long Term Outcomes ◽  
Single Center Experience ◽  
The Mean

The purpose of this study is to investigate the surgical results and long-term outcomes of encephaloduroarteriosynangiosis for moyamoya disease in pediatric patients. We performed a retrospective analysis of 67 pediatric patients with moyamoya disease who underwent encephaloduroarteriosynangiosis in Beijing Tiantan Hospital. The case series included 36 boys and 31 girls. All the patients underwent surgical revascularization, and a total of 93 encephaloduroarteriosynangiosis procedures were performed (41 unilateral, 26 bilateral). The mean follow-up period after surgery was 30 months. During follow-up, ischemic stroke events were detected in 5 patients and the stroke rate for pediatric patients who underwent encephaloduroarteriosynangiosis procedure was 7.1% per patient-years. At the last follow-up, favorable outcomes (modified Rankin Scale score ≤ 2) were observed in 65 cases (97.0%). Our study suggests that long-term surgical outcome of encephaloduroarteriosynangiosis in pediatric moyamoya disease patients is satisfactory, and this technique has a positive impact on the prevention of stroke recurrence.

Long-term follow-up after cyclophosphamide therapy in steroid-dependent nephrotic syndrome

2011 ◽  
Vol 26 (6) ◽  
pp. 915-920 ◽  
Author(s):  
Alberto Zagury ◽  
Anne Louise de Oliveira ◽  
Carlos Augusto Pinheiro de Moraes ◽  
Jose Augusto de Araujo Montalvão ◽  
Regina Helena Leite Lemos Novaes ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclophosphamide Therapy ◽  
Steroid Dependent ◽  
Long Term Follow Up ◽  
Steroid Dependent Nephrotic Syndrome

scholarly journals Early Treatment of Ruptured Blood Blister-Like Aneurysms of the Internal Carotid Artery With Flow Diverters Using Single Antiplatelet Therapy: A Single-Center Experience With Long-Term Follow-Up

2021 ◽  
Vol 12 ◽  
Author(s):  
Anil Tanburoglu ◽  
Cagatay Andic
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Early Treatment ◽  
Internal Carotid ◽  
Single Center ◽  
Related Complication ◽  
Single Center Experience ◽  
The Mean

Background and Purpose: Blood Blister-like aneurysms (BBAs) of the internal carotid artery (ICA) are rare entities of cerebral aneurysms. FD use in acutely ruptured aneurysms, timing of treatment and antiplatelet regimen are main debate topics in clinical practice when the treatment decision is flow diversion. The aim of this study is to report the safety and efficacy of a single-center FD treatment for ruptured BBAs in the early phase of SAH using the SAPT regimen.Material and Method: This study involved a retrospective analysis of a prospectively collected database. Records of patients admitted to our clinic and treated by endovascular route on ruptured BBA between January 2013 and December 2020 were reviewed. Ruptured supraclinoid ICA BBAs treated with FD devices with SAPT within 48 h from ictus of SAH are included. BBA of atypical anatomic locations, other endovascular techniques performed, and delayed admissions (>48 h) were excluded from the study. Demographic, clinical and angiographic features of patients and aneurysms, FD types and numbers, periprocedural complications, immediate and follow-up angiographic and clinical outcomes were recorded.Results: A total of six patients with ruptured BBAs treated via FDs within 48 h and used SAPT were included in the study. The mean age was 41.6 years (range from 34 to 45 years), and four of six patients were female. All patients were treated within 48 h after ictus, and the mean treatment day was 1.33 days. One patient received ticagrelor, and five patients received prasugrel as SAPT for one year after treatment. No procedure-related death and rebleeding were recorded. One (16.7 %) treatment responsive procedure-related complication occurred (transient ischemia). Overall good outcome rate was 83.3%. One patient died due to pneumonia. The immediate control angiograms showed complete occlusions of BBAs in one patient (16.6%). The complete occlusion rate was 100 % for five survivors at the control angiogram. The median follow-up was 49.5 months.Conclusion: This single-center experience suggests that early treatment (<2 days) within SAH of ruptured BBAs with FDs using SAPT is safe and effective in terms of clinical and radiological long-term outcomes.

scholarly journals Cyclophosphamide in frequent-relapsing or steroid-dependent nephrotic syndrome: Review of 38 patients

2016 ◽  
Vol 45 (1) ◽  
pp. 18
Author(s):  
Yulia Iriani ◽  
Taralan Tambunan ◽  
Sudigdo Sastroasmoro
Keyword(s):  
Nephrotic Syndrome ◽  
Cyclophosphamide Therapy ◽  
Steroid Dependent ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
The Mean ◽  
One Year ◽  
Steroid Dependent Nephrotic Syndrome ◽  
Better Than

Background Steroid-sensitive nephrotic syndrome (SSNS) in chil-dren is characterized by relapsing courses in a substantial propor-tion of affected individuals. Children with frequent-relapsing neph-rotic syndrome (FRNS) or steroid-dependent nephrotic syndrome(SDNS) are at risk of severe steroid toxicity and need individual-ized treatment. Previous studies have elucidated that cyclophos-phamide (CPA) reduced the risk of relapses and increased thelength of subsequent remissions in children with relapsing SSNS.Methods This retrospective study evaluated 38 patients (26 FRNSand 12 SDNS) after cyclophosphamide therapy to elucidate theefficacy of CPA in FRNS or SDNS in the Department of Child Health,Cipto Mangunkusumo Hospital. All patients were treated with CPA(2 mg/kg per day) for 8 weeks, in combination with prednisone.Results The median (range) duration of follow up was 45 months(24-140 months) for FRNS and 29 months (24-63 months) forSDNS. The mean relapse rate one year prior to CPA therapy inFRNS and SDNS were 3.8 relapses/year (95%CI 3.4; 4.2) and 4.0relapses/year (95%CI 3.3; 4.7), which were reduced to 1.6 relapses/year (95% CI 1.1; 2.1) and 2.3 relapses/year (95%CI 1.5;3.2), re-spectively. The overall rate of cumulative sustained good response(complete remission or infrequent relapses) was 65% after 36months. Frequent relapsing versus steroid-dependent status wassignificantly correlated with rate of sustained good response after36 months (85% versus 15%) with OR=23 (95%CI 3.1;225.2).Conclusion The efficacy of cyclophosphamide therapy in themanagement of FRNS is better than in SDNS

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