[PAGID] APECED/APS I and Renal Failure

[Jack Bleesing]

Hi Ken:
 
This is more question out of ignorance.Why aren't "we" replacing PTH in
parathryoidism secondary to autoimmune destruction, rather than
replacing (pouring in) calcium, vitamin D, etc?
http://www.druglib.com/trial/38/NCT00395538.html 
 
J
 
 
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Jack J.H. Bleesing, M.D., Ph.D.
Associate Professor of Pediatrics
Cincinnati Children's Hospital Medical Center
Division of Bone Marrow Transplantation and Immune Deficiency
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)


>>> "Paris, Kenneth" <kparis at lsuhsc.edu> 8/23/2010 12:25 PM >>>

Hi,
 
I was recently consulted regarding a young girl with APS Type I.  She
has all the typical features as well as the endocrinopathies, and her
clinical course has now been complicated by kidney disease that has been
characterized as chronic interstitial nephropathy.  (Our other APS
patient has renal issues, but in her case the underlying cause is most
likely nephrocalcinosis caused by the large Ca++ supplementation she
requires.)  This new girl is on her way to needing dialysis, and our
nephrology division is contemplating the idea of renal transplantation
in the future.
 
I found one citation from 2005, and from my brief search it seems to be
the only published report of RF/transplant in this disease:    J Clin
Endocrinol Metab.2006 Jan;91(1):192-5. Epub 2005 Nov 1. Autoimmune
polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome with renal
failure: impact of posttransplant immunosuppression on disease activity.
Ulinski T ( /pubmed?term=%22Ulinski%20T%22%5BAuthor%5D ), Perrin L (
/pubmed?term=%22Perrin%20L%22%5BAuthor%5D ), Morris M (
/pubmed?term=%22Morris%20M%22%5BAuthor%5D ), Houang M (
/pubmed?term=%22Houang%20M%22%5BAuthor%5D ), Cabrol S (
/pubmed?term=%22Cabrol%20S%22%5BAuthor%5D ), Grapin C (
/pubmed?term=%22Grapin%20C%22%5BAuthor%5D ), Chabbert-Buffet N (
/pubmed?term=%22Chabbert-Buffet%20N%22%5BAuthor%5D ), Bensman A (
/pubmed?term=%22Bensman%20A%22%5BAuthor%5D ), Deschênes G (
/pubmed?term=%22Desch%C3%AAnes%20G%22%5BAuthor%5D ), Giurgea I (
/pubmed?term=%22Giurgea%20I%22%5BAuthor%5D )
 
Does anyone have experience with this complication in APS patients, or
management suggestions considering the underlying defect?
 
Ken
 
Kenneth Paris MD, MPH
Assistant Professor of Pediatrics
A/I Fellowship Training Program Director
Division of Allergy and Immunology
JM Diagnostic Center for PI
LSU Health Sciences Center
Children's Hospital of New Orleans
 
Mail: 
200 Henry Clay Avenue
Children's Hospital 
Research Institute for Children 4th Floor
New Orleans, LA 70118
Phone:  504-896-9589
Fax:  504-896-9311
Email:  kparis at lsuhsc.edu 
 
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