[PAGID] APECED/APS I and Renal Failure

[Paris, Kenneth]

In regards to the PTH replacement, we actually are doing so.  Our patient is enrolled in the NIH trial, and has done fairly well.  The calcium issue is still a problem, and she needs frequent dose adjustments of her PTH (changes are per NIH protocol/MD's, so logistically it is sometimes difficult).  
 
In regards to the use of systemic antifungal use, I have no knowledge of her ever needing ampho for the candidiasis.  I believe she has responded pretty well to less toxic anitfungals. 

Ken
 
 
Kenneth Paris MD, MPH
Assistant Professor of Pediatrics
A/I Fellowship Training Program Director
Division of Allergy and Immunology
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> 
 
The information contained in this e-mail is privileged and confidential and is intended only for the use of the addressee(s) indicated above. Use or disclosure of information e-mailed in error is respectfully prohibited. If you have received this e-mail in error, please contact the sender and immediately delete the original message or you may call 504-896-9589. Thank you.

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Jack Bleesing
Sent: Mon 8/23/2010 12:39 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] APECED/APS I and Renal Failure


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
 
 
---------------------------------------------------------------------------
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, Perrin L, Morris M, Houang M, Cabrol S, Grapin C, Chabbert-Buffet N, Bensman A, Deschênes G, Giurgea I
 
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> 
 
The information contained in this e-mail is privileged and confidential and is intended only for the use of the addressee(s) indicated above. Use or disclosure of information e-mailed in error is respectfully prohibited. If you have received this e-mail in error, please contact the sender and immediately delete the original message or you may call 504-896-9589. Thank you.
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