الفهرس 
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Abstract
TMA is a life-threatening condition that requires urgent management. Survival of patients has improved greatly over the past two decades with improved supportive care for patients with HUS and using plasma exchange with fresh frozen plasma for patients with TTP. Our study aimed to describe the clinical features and outcomes of patients with thrombotic microangiopathies at Ain Shams University Children’s Hospital.
Infection-related HUS is the most common diagnosis, accounting for 51.5% of cases. This is followed by aHUS, which accounts for 30.3% of cases. cTTP and immune TTP are less common, each accounting for 9.1% of cases.
TMA management was determined according to the TMA primary disease diagnosis, clinical, laboratory, radiological data and response to the primary therapy, where conservative management was the mainstay for all our TMA patients, in the form of packed RBCS transfusion for 87.9%, and RRT needed for 69.7%. Plasma infusion was given to 75.8%, meanwhile combination therapy was used frequently, where the most used one was corticosteroids and plasma infusion (39.4%).
Brain imaging abnormalities were reported in 8 patients (50%), which were in the form of subacute infarction, periventricular and cerebellar non hemorrhagic lacunar infarcts, meanwhile fundus abnormalities were seen in 3 patients in the form of bilateral retinal hemorrhage. Renal biopsy was requested at presentation of 5 patients (15.2%) with atypical TMA presentation, which revealed tubulo-interstitial nephritis and TMA picture. There was no statistically significant relationship between mortality and brain imaging, fundus examination and renal biopsy abnormalities.
Two patients required combination therapy of IVIG and TPE who were diagnosed with aHUS and immune TTP and they had a complicated course ending in death, while there was no significant relationship between occurrence of mortality and other lines of management.
We followed up the patients at 3 and 6 months and found that 45.5% of our patients achieved recovery, 33.3% had residual hematological disease, 60.6% had residual renal disease. These numbers decreased at 6 months follow up indicating improvement over time (30.3% had residual hematological disease, 33.3% had residual renal disease).
The vast majority of patients did not experience relapse, where 93.9% were free of relapse during the follow-up period. Mortality was reported with 18.2% of patients died during the follow-up period.