[PAGID] APECED/APS I and Renal Failure

[Cunningham-Rundles, Charlotte]

Ken, we have one of ours in this boat. Same question. I have no idea how to
handle it. The mom will be the kidney donor.

Charlotte

Charlotte Cunningham-Rundles MD PhD
Departments of Medicine, Pediatrics
The Immunology Institute
Mount Sinai School of Medicine
1425 Madison Avenue,
New York City, New York, 10029
212 659 9268 (phone)
212 987 5593 (fax)



From: "Paris, Kenneth" <kparis at lsuhsc.edu>
Reply-To: PAGID <pagid at list.clinimmsoc.org>
Date: Mon, 23 Aug 2010 11:25:07 -0500
To: PAGID <pagid at list.clinimmsoc.org>
Subject: [PAGID] APECED/APS I and Renal Failure

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. <javascript:AL_get(this, 'jour', '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 <mailto:kparis at lsuhsc.edu>
 
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