S. Johnson

Publications29
h-index8
Citations502
Highly Influential Citations32
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An international consensus approach to the management of atypical hemolytic uremic syndrome in children
Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment… Expand
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An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome
AbstractBackgroundIn 2009, the European Paediatric Study Group for Haemolytic Uraemic Syndrome (HUS) published a clinical practice guideline for the investigation and initial therapy of… Expand
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The global aHUS registry: methodology and initial patient characteristics
BackgroundAtypical hemolytic uremic syndrome (aHUS) is a rare, genetically-mediated systemic disease most often caused by chronic, uncontrolled complement activation that leads to systemic thrombotic… Expand
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Eculizumab Use for Kidney Transplantation in Patients With a Diagnosis of Atypical Hemolytic Uremic Syndrome
Introduction Recurrence of atypical hemolytic uremic syndrome (aHUS) in renal allografts is common, leading to dialysis and graft failure. Pretransplant versus posttransplant initiation of eculizumab… Expand
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Treatment and management of children with haemolytic uraemic syndrome
Haemolytic uraemic syndrome (HUS), comprising microangiopathic haemolytic anaemia, thrombocytopaenia and acute kidney injury, remains the leading cause of paediatric intrinsic acute kidney injury,… Expand
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Floating ideologies: Metamorphoses of graphic ,Germanness`
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Novel compound heterozygous mutations in AMN cause Imerslund-Gräsbeck syndrome in two half-sisters: a case report
BackgroundImerslund-Gräsbeck Syndrome (IGS) is a rare autosomal recessive disease characterized by intestinal vitamin B12 malabsorption. Clinical features include megaloblastic anemia, recurrent… Expand
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A socially situated approach to inform ways to improve health and wellbeing.
Mainstream health psychology supports neoliberal notions of health promotion in which self-management is central. The emphasis is on models that explain behaviour as individually driven and… Expand
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De Novo Truncating Mutations in WASF1 Cause Intellectual Disability with Seizures
Next-generation sequencing has been invaluable in the elucidation of the genetic etiology of many subtypes of intellectual disability in recent years. Here, using exome sequencing and whole-genome… Expand
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Glucose-6-Phosphate Dehydrogenase Deficiency Mimicking Atypical Hemolytic Uremic Syndrome.
A 4-year-old boy presented with nonimmune hemolysis, thrombocytopenia, and acute kidney injury. Investigations for an underlying cause failed to identify a definitive cause and a putative diagnosis… Expand
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