[PAGID] APECED/APS I and Renal Failure

[Paris, Kenneth]

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, 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> 
 
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