Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children
Complement is activated as part of the innate immune defense against invading pathogens. Also, it helps to remove apoptotic debris and immune complexes from the circulation. Impaired complement function due to aberrant plasma levels of complement proteins may be indicative for complement-mediated di...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-08-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fimmu.2018.01727/full |
| _version_ | 1798043560365260800 |
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| author | Anna E. van Beek Anna E. van Beek Angela Kamp Simone Kruithof Ed J. Nieuwenhuys Diana Wouters Ilse Jongerius Theo Rispens Taco W. Kuijpers Taco W. Kuijpers Kyra A. Gelderman |
| author_facet | Anna E. van Beek Anna E. van Beek Angela Kamp Simone Kruithof Ed J. Nieuwenhuys Diana Wouters Ilse Jongerius Theo Rispens Taco W. Kuijpers Taco W. Kuijpers Kyra A. Gelderman |
| author_sort | Anna E. van Beek |
| collection | DOAJ |
| description | Complement is activated as part of the innate immune defense against invading pathogens. Also, it helps to remove apoptotic debris and immune complexes from the circulation. Impaired complement function due to aberrant plasma levels of complement proteins may be indicative for complement-mediated diseases or can be involved in susceptibility for infections. To determine whether plasma levels are abnormal, reference intervals (RIs) are used from adult healthy donors. Since many complement-mediated diseases have an onset during childhood, it is important to know whether these RIs can be extrapolated to children. RIs of Factor H (FH), the crucial fluid-phase regulator, and the FH-related proteins (FHRs), its homologous counterparts, are unknown in healthy children. While FH is measured to diagnose and monitor therapy of patients with atypical hemolytic uremic syndrome, recent studies also implicated increased plasma levels of FHRs in disease. Here, we investigated the levels of FH and FHRs in healthy children using recently developed specific ELISAs. We found that levels of FH, FHR-2, and FHR-3 were equal to those found in healthy adults. Levels of FHR-4A and FHR-5 were lower in children than in adults. However, only the FHR-5 levels associated with age. The RIs of these FH family proteins now serve to support the interpretation of plasma levels in prospective and retrospective studies that can be used for routine diagnostic and monitoring purposes including pediatric patient samples. |
| first_indexed | 2024-04-11T22:50:48Z |
| format | Article |
| id | doaj.art-76b48615b5b24c9facfa31e5fb6c9c4c |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-11T22:50:48Z |
| publishDate | 2018-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-76b48615b5b24c9facfa31e5fb6c9c4c2022-12-22T03:58:35ZengFrontiers Media S.A.Frontiers in Immunology1664-32242018-08-01910.3389/fimmu.2018.01727400449Reference Intervals of Factor H and Factor H-Related Proteins in Healthy ChildrenAnna E. van Beek0Anna E. van Beek1Angela Kamp2Simone Kruithof3Ed J. Nieuwenhuys4Diana Wouters5Ilse Jongerius6Theo Rispens7Taco W. Kuijpers8Taco W. Kuijpers9Kyra A. Gelderman10Department of Immunopathology, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsDepartment of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Centre, Amsterdam, NetherlandsDepartment of Immunopathology, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsDepartment of Immunopathology, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsSanquin Diagnostic Services, Amsterdam, NetherlandsDepartment of Immunopathology, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsDepartment of Immunopathology, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsDepartment of Immunopathology, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsDepartment of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children’s Hospital, Academic Medical Centre, Amsterdam, NetherlandsDepartment of Blood Cell Research, Sanquin Research and Landsteiner Laboratory of the Academic Medical Centre, University of Amsterdam, Amsterdam, NetherlandsSanquin Diagnostic Services, Amsterdam, NetherlandsComplement is activated as part of the innate immune defense against invading pathogens. Also, it helps to remove apoptotic debris and immune complexes from the circulation. Impaired complement function due to aberrant plasma levels of complement proteins may be indicative for complement-mediated diseases or can be involved in susceptibility for infections. To determine whether plasma levels are abnormal, reference intervals (RIs) are used from adult healthy donors. Since many complement-mediated diseases have an onset during childhood, it is important to know whether these RIs can be extrapolated to children. RIs of Factor H (FH), the crucial fluid-phase regulator, and the FH-related proteins (FHRs), its homologous counterparts, are unknown in healthy children. While FH is measured to diagnose and monitor therapy of patients with atypical hemolytic uremic syndrome, recent studies also implicated increased plasma levels of FHRs in disease. Here, we investigated the levels of FH and FHRs in healthy children using recently developed specific ELISAs. We found that levels of FH, FHR-2, and FHR-3 were equal to those found in healthy adults. Levels of FHR-4A and FHR-5 were lower in children than in adults. However, only the FHR-5 levels associated with age. The RIs of these FH family proteins now serve to support the interpretation of plasma levels in prospective and retrospective studies that can be used for routine diagnostic and monitoring purposes including pediatric patient samples.https://www.frontiersin.org/article/10.3389/fimmu.2018.01727/fullnormal rangescomplementcomplement factor Hfactor H-related proteinspediatricsdiagnostics |
| spellingShingle | Anna E. van Beek Anna E. van Beek Angela Kamp Simone Kruithof Ed J. Nieuwenhuys Diana Wouters Ilse Jongerius Theo Rispens Taco W. Kuijpers Taco W. Kuijpers Kyra A. Gelderman Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children Frontiers in Immunology normal ranges complement complement factor H factor H-related proteins pediatrics diagnostics |
| title | Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children |
| title_full | Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children |
| title_fullStr | Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children |
| title_full_unstemmed | Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children |
| title_short | Reference Intervals of Factor H and Factor H-Related Proteins in Healthy Children |
| title_sort | reference intervals of factor h and factor h related proteins in healthy children |
| topic | normal ranges complement complement factor H factor H-related proteins pediatrics diagnostics |
| url | https://www.frontiersin.org/article/10.3389/fimmu.2018.01727/full |
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