Background: Atypical hemolytic uremic syndrome (aHUS) is a devastating form of renal thrombotic microangiopathy. Despite plasma exchange, the standard treatment of aHUS for decades, the renal prognosis for patients with aHUS has remained poor. We assessed the off-trial use of eculizumab in adult patients with aHUS affecting the native kidneys.
Study Design: A retrospective study was conducted. aHUS was defined as the presence of 3 or more of the following: acute kidney injury (serum creatinine >1.4 mg/dL [120 μmol/L]), mechanical hemolytic anemia, thrombocytopenia, and the presence of thrombotic microangiopathy features in a kidney biopsy specimen. Patients who had received 4 or more weekly 900-mg infusions of eculizumab were included.
Setting & Participants: 19 patients were identified through a query sent to all French nephrology centers.
Outcomes & Measurements: Evolution of kidney function, hemolysis, and thrombocytopenia after the initiation of eculizumab therapy.
Results: All patients had acute kidney injury (serum creatinine range, 2.2-17.0 mg/dL) and 12 required hemodialysis. Thirteen patients carried a mutation in 1 complement gene and 1 had anti-factor H antibodies. For first-line therapy, 16 patients underwent plasma exchange and 3 patients received eculizumab. Median time between aHUS onset and eculizumab therapy initiation was 6 (range, 1-60) days and median time to platelet count normalization after eculizumab therapy initiation was 6 (range, 2-42) days. At the 3-month follow-up, 4 patients still required dialysis, 8 had non-dialysis-dependent chronic kidney disease, and 7 had normalized kidney function. At last follow-up (range, 4-22 months), 3 patients remained dialysis dependent, 7 had non-dialysis-dependent chronic kidney disease (estimated glomerular filtration rate, 17-55 mL/min/1.73 m(2)), and 9 had normal kidney function. Risks of reaching end-stage renal disease within 3 months and 1 year of aHUS onset were reduced by half in eculizumab-treated patients compared with recent historical controls.
Limitations: Retrospective study and use of historical controls.
Conclusions: Our data indicate that eculizumab improves kidney disease outcome in patients with aHUS.
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http://dx.doi.org/10.1053/j.ajkd.2013.07.011 | DOI Listing |
Elife
December 2024
Institute of Cognitive and Clinical Neuroscience, Central Institute of Mental Health, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany.
Chronic back pain (CBP) is a global health concern with significant societal and economic burden. While various predictors of back pain chronicity have been proposed, including demographic and psychosocial factors, neuroimaging studies have pointed to brain characteristics as predictors of CBP. However, large-scale, multisite validation of these predictors is currently lacking.
View Article and Find Full Text PDFEndocrinol Diabetes Metab
January 2025
Universidad Autónoma de Madrid, Department of Endocrinology and Nutrition, Hospital Universitario de La Princesa, Instituto de Investigación Sanitaria de La Princesa, Madrid, Spain.
Purpose: To investigate the impact of clinical and socio-economic factors on glycaemic control and construct statistical models to predict optimal glycaemic control (OGC) after implementing intermittently scanned continuous glucose monitoring (isCGM) systems.
Methods: This retrospective study included 1072 type 1 diabetes patients (49.0% female) from three centres using isCGM systems.
Ann Med
December 2025
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Background: Uric acid (UA) plays an important role in cardiovascular diseases, yet its implications in elderly patients remains incompletely understood. This study aimed to explore the impact of UA on the prognosis in advanced-age patients with acute coronary syndrome (ACS).
Methods: We included 526 patients aged 80 and older who were diagnosed with ACS.
J Cosmet Dermatol
December 2024
Department of Plastic Surgery, Affiliated Calmette Hospital of Kunming Medical University, Kunming, China.
Background: The Yunnan-Guizhou Plateau's high-altitude setting is characterized by intense solar ultraviolet radiation, a significant environmental stressor that frequently leads to skin barrier damage. This damage presents clinically as erythema, itching, and desquamation, underscoring the need for effective reparative interventions.
Aims: The objective of this study was to assess the therapeutic efficacy of a novel treatment protocol that integrates non-crosslinked hyaluronic acid (HA) injection with microneedle application of human epidermal growth factor (hEGF) for the restoration of skin barrier function in regions of high altitude.
J Adv Nurs
December 2024
La Trobe University, Melbourne, Victoria, Australia.
Aim: To examine the role of cultural safety within a human rights-based approach to improving the health of Indigenous Peoples.
Design: Guided by Askey and O'Malley's scoping review framework, the literature was examined on cultural safety and prioritised Indigenous voices to inform culturally safe practices. Relevant literature from 2009 to 2021 was included.
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