الفهرس 
Thrombotic MicroangiopathiesOnly 14 pages are availabe for public view
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Abstract
Background: Atypical HUS (aHUS), a life threatening condition, is an important cause of
acute kidney injury in childhood. It is primarily caused by dysregulation of the
complement alternative pathway (AP) leading to uncontrolled overactivation of the
complement system. AHUS may be due to genetic mutations in genes encoding complement
regulatory proteins mainly complement factor H (CFH) or due to autoimmune-HUS (AI-HUS).
AI-HUS is a multifactorial disorder resulting from a combination of acquired anti-factor H
autoantibodies, genetic predisposition and triggering environmental factors. Antifactor H
antibodies inhibit CFH function by binding to epitopes within the Cterminus, N-terminus and
also to its middle part implying neutralization of all CFH functions and hence leading to more
severe disease forms. Although AI-HUS cases have bad outcomes, they have excellent results
with a combination of plasma exchange (PEX) and immunosuppressive (IS) drugs. Early
diagnosis and treatment of this specific aHUS entity is very important and leads to favourable
outcome.
Aim of the work: The aim of this work is to study the presence of serum anti-factor H
antibody as a causative agent of aHUS in Egyptian children.
Subjects and Methods: The present study included 50 pediatric HUS patients admitted to
Cairo University Paediatric hospitals in the period from March 2018 to July 2019. A Control
group of 50 age and sex matched normal healthy subjects was included. Patients were
subjected to full history taking, clinical examination and laboratory testing. All candidates
were subjected to assessment of anti-factor H antibodies in serum by Homemade Enzyme
Linked Immunosorbent Assay (ELISA) technique.
Results: This study revealed high frequency of serum Anti-factor H autoantibodies being
detected in 42.9% of aHUS patients. Significantly higher rates of AI-HUS were found in male
school age children and disease onset was in March and April mainly. All patients who started
IS drugs early together with PEX upon detection of their anti-factor H antibodies, had
complete renal function recovery in the first flare.
Conclusion: The high frequency of AI-HUS revealed in this study in Egyptian HUS children
highlights the importance of implementing Anti-factor H antibody testing in Egypt to provide
early recognition and referral with immediate proper management and hence improving
patient outcomes.
Key words: Anti-Factor H autoantibodies, Hemolytic Uremic Syndrome.