دورية أكاديمية
أعرض في EDS

The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series.

التفاصيل البيبلوغرافية
العنوان: The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series.
المؤلفون: Maas RJ; Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands. Electronic address: rutger.maas@radboudumc.nl., Deegens JK; Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands., Beukhof JR; Department of Nephrology, Isala Klinieken, Zwolle, the Netherlands., Reichert LJ; Department of Nephrology, Rijnstate, Arnhem, the Netherlands., Ten Dam MA; Department of Nephrology, Canisius Wilhelmina Ziekenhuis, Nijmegen, the Netherlands., Beutler JJ; Department of Nephrology, Jeroen Bosch Ziekenhuis, 's Hertogenbosch, the Netherlands., van den Wall Bake AWL; Department of Nephrology, Máxima Medisch Centrum, Veldhoven, the Netherlands., Rensma PL; Department of Nephrology, St. Elisabeth Ziekenhuis, Tilburg, the Netherlands., Konings CJ; Department of Nephrology, Catharina Ziekenhuis, Eindhoven, the Netherlands., Geerse DA; Department of Nephrology, Catharina Ziekenhuis, Eindhoven, the Netherlands., Feith GW; Department of Nephrology, Gelderse Vallei, Ede, the Netherlands., Van Kuijk WH; Department of Nephrology, Viecuri Medisch Centrum, Venlo, the Netherlands., Wetzels JF; Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands.
المصدر: American journal of kidney diseases : the official journal of the National Kidney Foundation [Am J Kidney Dis] 2017 May; Vol. 69 (5), pp. 637-646. Date of Electronic Publication: 2017 Jan 12.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: W.B. Saunders Country of Publication: United States NLM ID: 8110075 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1523-6838 (Electronic) Linking ISSN: 02726386 NLM ISO Abbreviation: Am J Kidney Dis Subsets: MEDLINE
أسماء مطبوعة: Publication: Philadelphia Pa : W.B. Saunders
Original Publication: New York, N.Y. : Grune & Stratton, c1981-
مواضيع طبية MeSH: Acute Kidney Injury/*epidemiology , Adrenal Cortex Hormones/*therapeutic use , Cyclophosphamide/*therapeutic use , Glomerulosclerosis, Focal Segmental/*epidemiology , Immunosuppressive Agents/*therapeutic use , Kidney Failure, Chronic/*epidemiology , Nephrosis, Lipoid/*drug therapy, Adolescent ; Adult ; Age of Onset ; Aged ; Aged, 80 and over ; Disease Progression ; Female ; Follow-Up Studies ; Glomerulosclerosis, Focal Segmental/physiopathology ; Humans ; Kidney Failure, Chronic/physiopathology ; Male ; Middle Aged ; Nephrosis, Lipoid/epidemiology ; Nephrosis, Lipoid/physiopathology ; Recovery of Function ; Recurrence ; Remission Induction ; Remission, Spontaneous ; Retrospective Studies ; Thrombosis/epidemiology ; Venous Thrombosis/epidemiology ; Young Adult
مستخلص: Background: Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied.
Study Design: Case series.
Setting & Participants: Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers.
Outcomes: Partial and complete remission, treatment resistance, relapse, complications, renal survival.
Results: Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy.
Limitations: Retrospective design, variable treatment protocols.
Conclusions: The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission in many patients with relapses. Significant morbidity was observed due to acute kidney injury and other complications. Progression to end-stage renal disease occurred in a few patients and was explained by focal segmental glomerulosclerosis.
(Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)
فهرسة مساهمة: Keywords: Corticosteroids; acute kidney injury (AKI); adolescents; adult-onset MCNS; adults; case series; complete remission; cyclophosphamide; immunosuppression; minimal change disease (MCD); nephrotic syndrome (NS); partial remission; relapse; renal survival; treatment resistance
المشرفين على المادة: 0 (Adrenal Cortex Hormones)
0 (Immunosuppressive Agents)
8N3DW7272P (Cyclophosphamide)
تواريخ الأحداث: Date Created: 20170117 Date Completed: 20170731 Latest Revision: 20220321
رمز التحديث: 20221213
DOI: 10.1053/j.ajkd.2016.10.032
PMID: 28089478
قاعدة البيانات: MEDLINE
الوصف
تدمد:1523-6838
DOI:10.1053/j.ajkd.2016.10.032