[PAGID] Calcinosis cutis in an transplanted ADA-deficient child

[Charlotte Cunningham-Rundles]

We have a young boy with mucocutaneous candidiasis who has this 
development as well. As far as I know his calcium metabolism is ok as 
well.

At 11:13 AM -0500 2/16/07, Luigi Notarangelo wrote:

>In Brescia, Italy (where I was before moving to Boston), we are following a

>3-year-old female child with ADA deficiency, who has received HLA-identical

>stem cell transplantation. At nearly 3 years fater transplant, she has

>excellent T cell reconstitution (normal T numbers and function, 98%

>donor-derived T cells), mixed B cell chimerism, normal immunoglobulin

>production. Levels of toxic metabolites have dramatically dropped after

>transplant. However, the child has developed calcinosi cutis, with multiple

>lesions at soft tissues. There are no skeletal abnormalities. Levels of

>calcium and of PTH are normal. Has anybody observed anything similar in the

>context of severe PIDs or after stem cell transplant?

>

>

>Luigi D. Notarangelo

>Division of Immunology, Children's Hospital

>Karp Family Research Building, 9th floor, Rm 09210

>Boston, MA 02115

>tel: 617-919-2276

>FAX: 617-730-0709



-- 


Charlotte Cunningham-Rundles MD PhD
Professor of Medicine, Pediatrics  and Immunobiology
Mount Sinai School of Medicine
1425 Madison Ave, New York 10029
phone 212 659 9268
fax  212 987 5593
Email:  charlotte.cunningham-rundles at mssm.edu