Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome

Background. Atypical hemolytic uremic syndrome (aHUS) is an important cause of acute kidney injury in children. It is primarily caused by dysregulation of the complement alternative pathway due to genetic mutations, mainly in complement factor H genes, or due to anti-factor H autoantibodies (anti-FH...

Full description

Saved in:
Bibliographic Details
Main Authors: Shereen Shawky, Hesham Safouh, Mona Gamal, Mohammed M. Abbas, Azza Aboul-Enein, Toshihiro Sawai, Yosra Fahmy, Heba Selim
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:International Journal of Nephrology
Online Access:http://dx.doi.org/10.1155/2021/6904858
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832567947964448768
author Shereen Shawky
Hesham Safouh
Mona Gamal
Mohammed M. Abbas
Azza Aboul-Enein
Toshihiro Sawai
Yosra Fahmy
Heba Selim
author_facet Shereen Shawky
Hesham Safouh
Mona Gamal
Mohammed M. Abbas
Azza Aboul-Enein
Toshihiro Sawai
Yosra Fahmy
Heba Selim
author_sort Shereen Shawky
collection DOAJ
description Background. Atypical hemolytic uremic syndrome (aHUS) is an important cause of acute kidney injury in children. It is primarily caused by dysregulation of the complement alternative pathway due to genetic mutations, mainly in complement factor H genes, or due to anti-factor H autoantibodies (anti-FH), leading to uncontrolled overactivation of the complement system. Early diagnosis and treatment of autoimmune HUS (AI-HUS) is essential and leads to a favorable outcome. Methods. Fifty pediatric HUS patients and 50 age- and sex-matched controls were included in the study. Patients were subjected to full history taking, clinical examination, and laboratory testing. All candidates were subjected to an assessment of anti-FH in serum by a homemade enzyme-linked immunosorbent assay technique. Results. A high frequency of serum anti-FH was detected in our aHUS patients. The disease onset of AI-HUS was mainly observed in March and April, with significantly higher rates in school-aged males. All patients who started immunosuppressives early together with plasmapheresis upon detection of their anti-FH had complete renal function recovery. Conclusion. The high frequency of AI-HUS revealed in Egyptian HUS children in our study highlights the importance of implementing anti-FH testing in Egypt to provide early recognition for immediate proper management, including early immunosuppressive therapy, and hence improving patient outcomes.
format Article
id doaj-art-8b0e4b27900e422fa8ee55ee2fe4894b
institution Kabale University
issn 2090-2158
language English
publishDate 2021-01-01
publisher Wiley
record_format Article
series International Journal of Nephrology
spelling doaj-art-8b0e4b27900e422fa8ee55ee2fe4894b2025-02-03T01:00:06ZengWileyInternational Journal of Nephrology2090-21582021-01-01202110.1155/2021/6904858Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic SyndromeShereen Shawky0Hesham Safouh1Mona Gamal2Mohammed M. Abbas3Azza Aboul-Enein4Toshihiro Sawai5Yosra Fahmy6Heba Selim7Department of Clinical and Chemical PathologyDepartment of PediatricsDepartment of Clinical and Chemical PathologyDepartment of Clinical and Chemical PathologyDepartment of Clinical and Chemical PathologyDepartment of PediatricsDepartment of PediatricsDepartment of Clinical and Chemical PathologyBackground. Atypical hemolytic uremic syndrome (aHUS) is an important cause of acute kidney injury in children. It is primarily caused by dysregulation of the complement alternative pathway due to genetic mutations, mainly in complement factor H genes, or due to anti-factor H autoantibodies (anti-FH), leading to uncontrolled overactivation of the complement system. Early diagnosis and treatment of autoimmune HUS (AI-HUS) is essential and leads to a favorable outcome. Methods. Fifty pediatric HUS patients and 50 age- and sex-matched controls were included in the study. Patients were subjected to full history taking, clinical examination, and laboratory testing. All candidates were subjected to an assessment of anti-FH in serum by a homemade enzyme-linked immunosorbent assay technique. Results. A high frequency of serum anti-FH was detected in our aHUS patients. The disease onset of AI-HUS was mainly observed in March and April, with significantly higher rates in school-aged males. All patients who started immunosuppressives early together with plasmapheresis upon detection of their anti-FH had complete renal function recovery. Conclusion. The high frequency of AI-HUS revealed in Egyptian HUS children in our study highlights the importance of implementing anti-FH testing in Egypt to provide early recognition for immediate proper management, including early immunosuppressive therapy, and hence improving patient outcomes.http://dx.doi.org/10.1155/2021/6904858
spellingShingle Shereen Shawky
Hesham Safouh
Mona Gamal
Mohammed M. Abbas
Azza Aboul-Enein
Toshihiro Sawai
Yosra Fahmy
Heba Selim
Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome
International Journal of Nephrology
title Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome
title_full Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome
title_fullStr Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome
title_full_unstemmed Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome
title_short Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome
title_sort anti factor h antibodies in egyptian children with hemolytic uremic syndrome
url http://dx.doi.org/10.1155/2021/6904858
work_keys_str_mv AT shereenshawky antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT heshamsafouh antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT monagamal antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT mohammedmabbas antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT azzaaboulenein antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT toshihirosawai antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT yosrafahmy antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome
AT hebaselim antifactorhantibodiesinegyptianchildrenwithhemolyticuremicsyndrome