scholarly journals Severe Acral Necrosis Complicating Thrombotic Microangiopathy in Two Toddlers

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
H. Nassih ◽  
Z. Lazrak ◽  
S. Younous
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Anticoagulant Therapy ◽  
Thrombotic Microangiopathy ◽  
Pediatric Patients ◽  
Thrombocytopenic Purpura ◽  
Uremic Syndrome ◽  
Acral Necrosis ◽  
Acral Ischemia

Acral ischemia/necrosis is one of the rarest but most dreadful complications of thrombotic microangiopathy in pediatric patients. It is more reported with thrombotic thrombocytopenic purpura than with hemolytic and uremic syndrome. Even with anticoagulant therapy, it is often irreversible, leading to amputation.

scholarly journals Etiology and Outcomes of Thrombotic Microangiopathies

2019 ◽  
Vol 14 (4) ◽  
pp. 557-566 ◽  
Author(s):  
Guillaume Bayer ◽  
Florent von Tokarski ◽  
Benjamin Thoreau ◽  
Adeline Bauvois ◽  
Christelle Barbet ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombotic Microangiopathy ◽  
Cardiovascular Events ◽  
Cardiac Complications ◽  
Neurologic Complications ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Coronary Syndrome ◽  
Major Cardiovascular Events ◽  
Uremic Syndrome

Background and objectivesThrombotic microangiopathies constitute a diagnostic and therapeutic challenge. Secondary thrombotic microangiopathies are less characterized than primary thrombotic microangiopathies (thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome). The relative frequencies and outcomes of secondary and primary thrombotic microangiopathies are unknown.Design, setting, participants, & measurementsWe conducted a retrospective study in a four-hospital institution in 564 consecutive patients with adjudicated thrombotic microangiopathies during the 2009–2016 period. We estimated the incidence of primary and secondary thrombotic microangiopathies, thrombotic microangiopathy causes, and major outcomes during hospitalization (death, dialysis, major cardiovascular events [acute coronary syndrome and/or acute heart failure], and neurologic complications [stroke, cognitive impairment, or epilepsy]).ResultsWe identified primary thrombotic microangiopathies in 33 of 564 patients (6%; thrombotic thrombocytopenic purpura: 18 of 564 [3%]; atypical hemolytic and uremic syndrome: 18 of 564 [3%]). Secondary thrombotic microangiopathies were found in 531 of 564 patients (94%). A cause was identified in 500 of 564 (94%): pregnancy (35%; 11 of 1000 pregnancies), malignancies (19%), infections (33%), drugs (26%), transplantations (17%), autoimmune diseases (9%), shiga toxin due to Escherichia coli (6%), and malignant hypertension (4%). In the 31 of 531 patients (6%) with other secondary thrombotic microangiopathies, 23% of patients had sickle cell disease, 10% had glucose-6-phosphate dehydrogenase deficiency, and 44% had folate deficiency. Multiple causes of thrombotic microangiopathies were more frequent in secondary than primary thrombotic microangiopathies (57% versus 19%; P<0.001), and they were mostly infections, drugs, transplantation, and malignancies. Significant differences in clinical and biologic differences were observed among thrombotic microangiopathy causes. During the hospitalization, 84 of 564 patients (15%) were treated with dialysis, 64 of 564 patients (11%) experienced major cardiovascular events, and 25 of 564 patients (4%) had neurologic complications; 58 of 564 patients (10%) died, but the rates of complications and death varied widely by the cause of thrombotic microangiopathies.ConclusionsSecondary thrombotic microangiopathies represent the majority of thrombotic microangiopathies. Multiple thrombotic microangiopathies causes are present in one half of secondary thrombotic microangiopathies. The risks of dialysis, neurologic and cardiac complications, and death vary by the cause of thrombotic microangiopathies.

Atypical hemolytic uremic syndrome: from diagnosis to treatment

2015 ◽  
Vol 53 (11) ◽  
Author(s):  
Massimo Franchini
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Shiga Toxin ◽  
Thrombotic Microangiopathy ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Rare Condition ◽  
Organ Damage ◽  
Thrombocytopenic Purpura ◽  
Haemolytic Uremic Syndrome ◽  
Uremic Syndrome

AbstractThrombotic microangiopathy (TMA) is a relatively rare condition but a medical urgency requiring immediate intervention to avoid irreversible organ damage or death. Symptoms on presentation include microangiopathic haemolytic anaemia, thrombocytopenia and organ damage. The most frequent direct causes of TMA are thrombotic thrombocytopenic purpura (TTP) and haemolytic uremic syndrome (HUS). The most common form of HUS is related to Shiga toxin producing

Sign in / Sign up

Export Citation Format

Share Document