scholarly journals A child with recurrent hemolytic uremic syndrome treated with fresh frozen plasma infusions and high-dose intravenous immunoglobulin therapy

1987 ◽  
Vol 10 (4) ◽  
pp. 408-414
Author(s):  
Jun-ichi Akatsuka ◽  
Sanpei Miyagawa ◽  
Koji Fujisawa ◽  
Hironori Nakamura ◽  
Shuji Nozaki ◽  
...  
1999 ◽  
Vol 12 (2) ◽  
pp. 145-147
Author(s):  
Hirohiko Higashino ◽  
Minoru Kino ◽  
Akitoshi Nakamura ◽  
Gen Unishi ◽  
Masashi Hasui ◽  
...  

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Louis Manière ◽  
Camille Domenger ◽  
Boubou Camara ◽  
Diane Giovannini ◽  
Paolo Malvezzi ◽  
...  

We herein describe the first case of thrombotic microangiopathy (TMA) which was related to Shiga toxin producing-Escherichia Coli Hemolytic and Uremic Syndrome (STEC-HUS) after lung transplantation. His maintenance immunosuppression relied on tacrolimus plus mycophenolic acid. TMA was treated with plasma exchanges (PE) (fresh frozen plasma substitution). After five days of PE, platelets count and lactate dehydrogenase level normalized, whereas hemoglobin continued to gradually decrease and no improvement in kidney function was observed. After seven PE sessions, all TMA biological signs resolved. However, kidney function did not improve, and the patient still required chronic dialysis.


2021 ◽  
Vol 14 (8) ◽  
pp. e239901
Author(s):  
Faheema Hasan ◽  
Anshul Gupta ◽  
Dinesh Chandra ◽  
Soniya Nityanand

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.


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