Abstract
Haemolytic uremic syndrome and thrombotic thrombocytopenic purpura are overlapping clinical entities based on historical classification. Recent developments in the unfolding of the pathomechanisms of these diseases resulted in the creation of a molecular etiology-based classification. Understanding of some causative relationships yielded detailed diagnostic approaches, novel therapeutic options and thorough prognostic assortment of the patients. Although haemolytic uremic syndrome and thrombotic thrombocytopenic purpura are rare diseases with poor prognosis, the precise molecular etiology-based diagnosis might properly direct the therapy of the affected patients. The current review focuses on the theoretical background and detailed description of the available diagnostic possibilities, and some practical information necessary for the interpretation of their results.
MeSH terms
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ADAM Proteins / metabolism
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ADAMTS13 Protein
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Analgesics, Non-Narcotic / adverse effects
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Antimalarials / adverse effects
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Complement System Proteins / metabolism
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Diagnosis, Differential
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Hemolytic-Uremic Syndrome / classification*
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Hemolytic-Uremic Syndrome / diagnosis
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Hemolytic-Uremic Syndrome / enzymology
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Hemolytic-Uremic Syndrome / etiology*
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Hemolytic-Uremic Syndrome / immunology
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Hemolytic-Uremic Syndrome / microbiology
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Humans
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Neuraminidase / biosynthesis
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Neuraminidase / metabolism
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Prognosis
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Purpura, Thrombotic Thrombocytopenic / classification*
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Purpura, Thrombotic Thrombocytopenic / diagnosis
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Purpura, Thrombotic Thrombocytopenic / enzymology
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Purpura, Thrombotic Thrombocytopenic / etiology*
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Purpura, Thrombotic Thrombocytopenic / immunology
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Purpura, Thrombotic Thrombocytopenic / microbiology
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Quinine / adverse effects
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Shiga Toxins / adverse effects
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Streptococcus pneumoniae / enzymology
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Vitamin B 12 / metabolism
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von Willebrand Factor / metabolism
Substances
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Analgesics, Non-Narcotic
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Antimalarials
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Shiga Toxins
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von Willebrand Factor
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Complement System Proteins
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Quinine
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Neuraminidase
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ADAM Proteins
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ADAMTS13 Protein
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ADAMTS13 protein, human
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Vitamin B 12