scholarly journals Acute hepatic encephalopathy and multiorgan failure in sickle cell disease and COVID‐19

2021 ◽  
Author(s):  
Giulia M. Martone ◽  
Priyanka M. Nanjireddy ◽  
Robin A. Craig ◽  
Andrew J. Prout ◽  
Meghan A. Higman ◽  
...  
Keyword(s):  
Hepatic Encephalopathy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Multiorgan Failure ◽  
Cell Disease ◽  
Acute Hepatic Encephalopathy

scholarly journals Acute hepatic encephalopathy and multiorgan failure in sickle cell disease and COVID-19

2020 ◽  
Author(s):  
Giulia Martone ◽  
Priyanka Nanjireddy ◽  
Robin Craig ◽  
Andrew Prout ◽  
Meghan Higman ◽  
...  
Keyword(s):  
Hepatic Encephalopathy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Multiorgan Failure ◽  
Cell Disease ◽  
Acute Hepatic Encephalopathy

Case series supporting heme detoxification via therapeutic plasma exchange in acute multiorgan failure syndrome resistant to red blood cell exchange in sickle cell disease

Transfusion ◽  
2017 ◽  
Vol 58 (2) ◽  
pp. 470-479 ◽  
Author(s):  
James E. Louie ◽  
Caitlin J. Anderson ◽  
Katayoun Fayaz M. Fomani ◽  
Alonye Henry ◽  
Trevor Killeen ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Plasma Exchange ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Multiorgan Failure ◽  
Case Series ◽  
Therapeutic Plasma Exchange ◽  
Cell Disease

1720: TRANSFUSION-TRANSMITTED MALARIA: A CAUSE OF ACUTE MULTIORGAN FAILURE SYNDROME IN SICKLE CELL DISEASE

2016 ◽  
Vol 44 (12) ◽  
pp. 505-505
Author(s):  
Salimah Valliani ◽  
Pradyumna Agasthi ◽  
Gloria Westney ◽  
Marilyn Foreman
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Multiorgan Failure ◽  
Cell Disease

scholarly journals ‘Girdle Syndrome’ Progressing to Ischaemic Colitis and Acute Intrahepatic Cholestasis in a Patient with Sickle Cell Disease: A Case Report

2020 ◽  
pp. 81-85
Author(s):  
Konstantinos Manganas ◽  
Sophia Delicou ◽  
Aikaterini Xydaki ◽  
John Koskinas
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Intrahepatic Cholestasis ◽  
Multiorgan Failure ◽  
Supportive Treatment ◽  
Intestinal Ischaemia ◽  
Cell Disease ◽  
Ischaemic Colitis ◽  
Fatal Complication

In this paper, the case of a 34-year-old male with sickle cell disease, recurrent episodes of ‘girdle syndrome’, and development of chronic ischaemic colitis is reported. At his last admission to the hospital, he presented with ileus attributed to severe intestinal ischaemia. During his hospitalisation, despite optimal supportive treatment, he developed acute liver failure, possibly as a result of acute intrahepatic cholestasis, a rare but fatal complication of sickle cell disease, and died from sepsis and multiorgan failure.

scholarly journals P-selectin drives complement attack on endothelium during intravascular hemolysis in TLR-4/heme-dependent manner

2019 ◽  
Vol 116 (13) ◽  
pp. 6280-6285 ◽  
Author(s):  
Nicolas S. Merle ◽  
Romain Paule ◽  
Juliette Leon ◽  
Marie Daugan ◽  
Tania Robe-Rybkine ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Liver Damage ◽  
Complement System ◽  
Multiorgan Failure ◽  
Organ Injury ◽  
Complement C3 ◽  
Dependent Manner ◽  
Cell Disease ◽  
Intravascular Hemolysis

Hemolytic diseases are frequently linked to multiorgan failure subsequent to vascular damage. Deciphering the mechanisms leading to organ injury upon hemolytic event could bring out therapeutic approaches. Complement system activation occurs in hemolytic disorders, such as sickle cell disease, but the pathological relevance and the acquisition of a complement-activating phenotype during hemolysis remain unclear. Here we found that intravascular hemolysis, induced by injection of phenylhydrazine, resulted in increased alanine aminotransferase plasma levels and NGAL expression. This liver damage was at least in part complement-dependent, since it was attenuated in complement C3−/−mice and by injection of C5-blocking antibody. We evidenced C3 activation fragments’ deposits on liver endothelium in mice with intravascular hemolysis or injected with heme as well as on cultured human endothelial cells (EC) exposed to heme. This process was mediated by TLR4 signaling, as revealed by pharmacological blockade and TLR4 deficiency in mice. Mechanistically, TLR4-dependent surface expression of P-selectin triggered an unconventional mechanism of complement activation by noncovalent anchoring of C3 activation fragments, including the typical fluid-phase C3(H2O), measured by surface plasmon resonance and flow cytometry. P-selectin blockade by an antibody prevented complement deposits and attenuated the liver stress response, measured by NGAL expression, in the hemolytic mice. In conclusion, these results revealed the critical impact of the triad TLR4/P-selectin/complement in the liver damage and its relevance for hemolytic diseases. We anticipate that blockade of TLR4, P-selectin, or the complement system could prevent liver injury in hemolytic diseases like sickle cell disease.

Hemolysis in sickle cell disease

1974 ◽  
Vol 133 (4) ◽  
pp. 624-631 ◽  
Author(s):  
T. A. Bensinger
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease
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