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Thrombotic microangiopathies
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Thrombotic microangiopathies

Clinical feature: 

Definition: This group of diseases is characterized by systemic or intrarenal aggregation of platelets, thrombocytopenia, and mechanical injury to erythrocytes.

Pathogenesis: The pathogenetic link is the occlusion of microvessels by microthrombi. The resulting insufficient perfusion is responsible for the organ specific symptoms.

Diagnostics: 

Strategy: Along with the well known routine laboratory parameters, measurement of factor H and metalloproteinases namely ADAMTS13 is recommended.

Systematic link table: 

Hereditary kidney diseases
Congenital abnormalities of the kidney and urinary tract
Cystic kidney disease
Disorders of tubular solute transport
Hereditary glomerular disease
Hereditary renal tumors
Interstitial kidney disease
Kidney disease appearing as endocrinological disorders
Thrombotic microangiopathies
Hemolytic-Uremic Syndrome
ADAMTS13
C3
CFB
CFH
CFHR1
CFHR2
CFHR3
CFHR4
CFHR5
CFI
MCP
THBD
Thrombotic Thrombocytopenic Purpura
ADAMTS13
Urolithiasis

Literature: 

McCrae KR et al. (2001) Platelets: an update on diagnosis and management of thrombocytopenic disorders.
Shah NT et al. (2003) Controversies in differentiating thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Moake JL et al. (2002) Thrombotic microangiopathies.
Nürnberger J et al. (2009) Eculizumab for atypical hemolytic-uremic syndrome.