[CIS PIDD] [cis-pidd] Fwd: is there any Factor H replacement therapy?

Sat Mar 28 23:22:09 EDT 2015

Dear Lourdes,

There is a group in Madrid that might be able to help you with the factor H issue.  Santiago Rodrigues de Cordoba is very knowledgeable about the nature of  Factor H deficiencies and dysfunction, and I'm sure you could get answers to your questions there.  One of the papers from the group is given below so you can identify them.  There is a growing body of literature on FH related issues and the diseases associated with it  FFP has been used in some cases, and there is  also a treatment that uses a monoclonal antibody against C5. It blocks complement activation in  patients that lack FH control of complement activation.

J Am Soc Nephrol.<http://www.ncbi.nlm.nih.gov/pubmed#> 2015 Jan;26(1):209-19. doi: 10.1681/ASN.2013121339. Epub 2014 Jun 5.
A novel atypical hemolytic uremic syndrome-associated hybrid CFHR1/CFH gene encoding a fusion protein that antagonizes factor H-dependent complement regulation.<http://www.ncbi.nlm.nih.gov/pubmed/24904082>
Valoti E<http://www.ncbi.nlm.nih.gov/pubmed/?term=Valoti%20E%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>1, Alberti M<http://www.ncbi.nlm.nih.gov/pubmed/?term=Alberti%20M%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>1, Tortajada A<http://www.ncbi.nlm.nih.gov/pubmed/?term=Tortajada%20A%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>2, Garcia-Fernandez J<http://www.ncbi.nlm.nih.gov/pubmed/?term=Garcia-Fernandez%20J%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>2, Gastoldi S<http://www.ncbi.nlm.nih.gov/pubmed/?term=Gastoldi%20S%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>1, Besso L<http://www.ncbi.nlm.nih.gov/pubmed/?term=Besso%20L%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>3, Bresin E<http://www.ncbi.nlm.nih.gov/pubmed/?term=Bresin%20E%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>1, Remuzzi G<http://www.ncbi.nlm.nih.gov/pubmed/?term=Remuzzi%20G%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>4,Rodriguez de Cordoba S<http://www.ncbi.nlm.nih.gov/pubmed/?term=Rodriguez%20de%20Cordoba%20S%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>2, Noris M<http://www.ncbi.nlm.nih.gov/pubmed/?term=Noris%20M%5BAuthor%5D&cauthor=true&cauthor_uid=24904082>5.

Best regards ,
Patsy Giclas, PhD
---------------------------------------------------------------
Patricia C. Giclas, PhD
Professor, Pediatric allergy and Immunology
ScientificAdvisor, Complement Laboratory
National Jewish Health
1400 Jackson St, Denver CO 80206
303-398-1217
Giclasp at njhealth.org

From: Lourdes Tricas Aizpun <lourdes.tricas at sespa.es<mailto:lourdes.tricas at sespa.es>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Saturday, March 28, 2015 4:32 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] Fwd: is there any Factor H replacement therapy?

________________________________
De: "Lourdes Tricas Aizpun" <lourdes.tricas at sespa.es<mailto:lourdes.tricas at sespa.es>>
Para: cis-pidd at lysis.dundee.net<mailto:cis-pidd at lysis.dundee.net>
Enviados: Viernes, 27 de Marzo 2015 17:11:55
Asunto: is there any Factor H replacement therapy?

Dear colleagues,
 Clinical case:
 Family History:
 Family Factor H of Complement system deficiency. 2 dead sisters. They had glomerulonephritis and severe infections.

Patient History:
 Woman  50 years old, factor H deficiency, HCV positive.
1982.- meningococcal sepsis & membrano-proliferative glomerulonephritis.
1996.- kidney transplant.
2000.- gastroenteritis due to salmonella enteriditis.
2005.- graft loss due to recurrence of glomerulonephritis.
2009.- Second kidney transplant and consequently immunosuppressive treatment.
At he present time several and severe  urinary infections.

Questions:
 Aside from to be immunized with polyvalent meningococcal vaccine we wonder if there is a Factor H replacement therapy with synthesized Factor H or with healthy plasma in the case of severe life-threatening sepsis.

Thank you for your help, comments and teaching.
 Lourdes Tricas, MD PhD. Clinical Laboratory Immunologist.
Hospital Universitario Central de Asturias, Oviedo, Spain
lourdes.tricas at sespa.es<mailto:lourdes.tricas at sespa.es>

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