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Search: authors:"Nicole C. A. J. van de Kar"

11 papers found.
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The role of properdin in complement-mediated renal diseases: a new player in complement-inhibiting therapy?

Properdin is known as the only positive regulator of the complement system. Properdin promotes the activity of this defense system by stabilizing its key enzymatic complexes: the complement alternative pathway (AP) convertases. Besides, some studies have indicated a role for properdin as an initiator of complement activity. Though the AP is a powerful activation route of the...

Unusual severe case of hemolytic uremic syndrome due to Shiga toxin 2d-producing E. coli O80:H2

Background Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children, with the majority of cases caused by an infection with Shiga toxin-producing Escherichia coli (STEC). Whereas O157 is still the predominant STEC serotype, non-O157 serotypes are increasingly associated with STEC-HUS. However, little is known about this emerging and...

Development and Pretesting of a Questionnaire to Assess Patient Experiences and Satisfaction with Medications (PESaM Questionnaire)

Background The aim of this study was to develop, together with the Lung Foundation Netherlands and Dutch Kidney Patients Association, patients and clinicians, a measure to evaluate patient experiences with the orphan drugs pirfenidone (for idiopathic pulmonary fibrosis [IPF]) and eculizumab (for atypical haemolytic uraemic syndrome [aHUS]), as well as a generic measure of patient...

Fecal diagnostics in combination with serology: best test to establish STEC-HUS

Background In the majority of pediatric patients, the hemolytic–uremic syndrome (HUS) is caused by an infection with Shiga toxin-producing Escherichia coli (STEC), mostly serotype O157. It is important to discriminate between HUS caused by STEC and complement-mediated HUS (atypical HUS) due to differences in treatment and outcome. As STEC and its toxins can only be detected in...

Serological and genetic complement alterations in infection-induced and complement-mediated hemolytic uremic syndrome

. J. van de Kar contributed equally to this work. - best discrepancy between acute and convalescent phase and between STEC-HUS and aHUS and might therefore be used as a biomarker in disease diagnosis ... phase of the disease but not during remission. The C3d/C3 ratio displayed the Elena B. Volokhina and Renate G. van der Molen contributed equally to this work, and Lambert P. van den Heuvel and Nicole C. A

Eculizumab as rescue therapy for atypical hemolytic uremic syndrome with normal platelet count

Eiske M. Dorresteijn 0 Nicole C. A. J. van de Kar 0 Karlien Cransberg 0 0 N. C. A. J. van de Kar Pediatric Nephrology, Radboud University Nijmegen Medical Centre , Nijmegen, The Netherlands

Unexplained hypothermia and bradycardia in two pediatric patients with Wegener’s granulomatosis

Sirs,We report remarkable symptoms of Wegener’s granulomatosis (WG), which might occur more often than reported so far. Two patients (13 and 16 years old) with acute renal failure due to WG developed simultaneous hypothermia (ANCA)-serology was positive. They received 3-day IPM therapy (1 g/1.73 m2), followed by prednisolone and cyclophosphamide (CYC) (2 mg/kg/day). Hemodialysis...

Atypical hemolytic uremic syndrome in children: complement mutations and clinical characteristics

Background Mutations in complement factor H (CFH), factor I (CFI), factor B (CFB), thrombomodulin (THBD), C3 and membrane cofactor protein (MCP), and autoantibodies against factor H (αFH) with or without a homozygous deletion in CFH-related protein 1 and 3 (∆CFHR1/3) predispose development of atypical hemolytic uremic syndrome (aHUS). Methods Different mutations in genes encoding...

Vero Cytotoxin Binding to Polymorphonuclear Leukocytes among Households with Children with Hemolytic Uremic Syndrome

Hemolytic uremic syndrome (HUS), the leading cause of acute renal failure in childhood, can be caused by different serotypes of vero cytotoxin (VT; i.e., Shiga toxin)–producing Escherichia coli (VTEC). Recently, VT was shown to bind to polymorphonuclear leukocytes (PMNL) in the systemic circulation of patients with HUS. This study investigated whether VT bound to PMNL could be...