Whole-exome sequencing detects mutations in pediatric patients with atypical hemolytic uremic syndrome in Taiwan

MH Tseng, JD Tsai, IJ Tsai, SM Huang, JL Huang… - Clinica Chimica …, 2019 - Elsevier
… In agreement with previous findings, our simulation model demonstrated the CD36 mutations
found from our patients would destabilize the structure of CD36 protein, which may further …

Use of complement monoclonal antibody eculizumab in Shiga toxin producing Escherichia coli associated hemolytic uremic syndrome: A review of current evidence

U Mahat, RB Matar, SJ Rotz - Pediatric Blood & Cancer, 2019 - Wiley Online Library
… in the treatment of atypical hemolytic uremic syndrome (HUS). … Eculizumab efficacy was
monitored using serum hemolytic … the authors utilized statistical models using a propensity score …

EE52 Cost-Minimisation Analysis of Ravulizumab Compared With Eculizumab in Patients With Atypical Hemolytic Uremic Syndrome (AHUS) in the Netherlands

AJ Postma, S Quist, L de Jong - Value in Health, 2022 - valueinhealthjournal.com
… , the model was the most sensitive to the mixture cure model … The model considered adult
and paediatric treatment-naïve … (treatment [drug acquisition, administration], monitoring, …

[HTML][HTML] Ockham's razor defeated: about two atypical cases of hemolytic uremic syndrome

C Schwarz, A Brehon, C Mousseaux, Y Luque, P Senet… - BMC nephrology, 2020 - Springer
… We propose a two-hit mechanism model leading to HUS. Indeed, in case 1, HUS unfolded …
-diseases-surveillance-report-2015.pdf). In comparison, atypical hemolytic uremic syndrome (…

Serum Transaminases at Presentation and Association with Acute Dialysis in Children with Hemolytic Uremic Syndrome

S Talathi, M Barnes, I Aban, R Dimmitt, DJ Askenazi - Kidney360, 2020 - journals.lww.com
… of the models with and without these variables. Overall performance of the model when neither
serum AST … A model using AST and ALT together did not improve the performance of the …

Complement-mediated coagulation disorders: paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome

E Gavriilaki, RA Brodsky - Consultative Hemostasis and Thrombosis, 2019 - Elsevier
… nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are well-…
Until then, close surveillance for signs and symptoms of recurrent TMA is recommended if …

Outcome of children with Shiga toxin-associated haemolytic uraemic syndrome treated with eculizumab: a matched cohort study

C Monet-Didailler, A Chevallier… - Nephrology Dialysis …, 2020 - academic.oup.com
Treatment with eculizumab in Shiga toxin–associated haemolytic and uraemic syndrome
(STEC-… The timing of complement activity monitoring and the values of CAE before and after …

Atypical hemolytic uremic syndrome associated with a factor B genetic variant and fluid-phase complement activation: an exception to the rule?

M Noris, G Remuzzi - Kidney International, 2020 - Elsevier
… These findings should be confirmed in more suitable and disease-relevant cell models with
cultured human endothelial cells. Nevertheless, they indicate that the p.367Arg FB mutant …

Efficacy and Safety of Ravulizumab in Pediatric Patients with Atypical Hemolytic Uremic Syndrome Previously Treated with Eculizumab: 26-Week and 1-Year Data …

LA Greenbaum, N Fujita, BV Adams… - Journal of the …, 2020 - journals.lww.com
… Mean arterial pressure (MAP) derived from 24-hour ambulatory BP monitoring was … Methods:
Nephrin lacking the transmembrane domain (Nephmis) was used as a model of misfolded …

Feasibility and safety of tailored dosing schedule for eculizumab based on therapeutic drug monitoring: lessons from a prospective multicentric study

C Passot, R Sberro‐Soussan… - British journal of …, 2021 - Wiley Online Library
… drug monitoring of eculizumab in atypical haemolyticuraemic syndrome patients. … A
population PK model was developed from these data in order to: (i) describe the relationship …