Anti-Factor H Antibodies in Egyptian Children with Hemolytic Uremic Syndrome.
Journal
International journal of nephrology
ISSN: 2090-214X
Titre abrégé: Int J Nephrol
Pays: United States
ID NLM: 101546753
Informations de publication
Date de publication:
2021
2021
Historique:
received:
18
09
2021
revised:
23
10
2021
accepted:
26
10
2021
entrez:
29
11
2021
pubmed:
30
11
2021
medline:
30
11
2021
Statut:
epublish
Résumé
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. 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. 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. 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.
Sections du résumé
BACKGROUND
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
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
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
CONCLUSIONS
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.
Identifiants
pubmed: 34840826
doi: 10.1155/2021/6904858
pmc: PMC8616678
doi:
Types de publication
Journal Article
Langues
eng
Pagination
6904858Informations de copyright
Copyright © 2021 Shereen Shawky et al.
Déclaration de conflit d'intérêts
The authors declare that they have no conflicts of interest.
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