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An unconditioned bone marrow transplantation in a child with purine nucleoside phosphorylase deficiency and its unique complication.
- Source :
-
Pediatric transplantation [Pediatr Transplant] 2008 Jun; Vol. 12 (4), pp. 479-82. Date of Electronic Publication: 2008 Jan 14. - Publication Year :
- 2008
-
Abstract
- Purine nucleoside phosphorylase deficiency is a rare immunodeficiency syndrome characterized by recurrent infections, neurological dysfunction, and autoimmunity. Early diagnosis and hematopoietic stem cell transplantation may reverse the dismal prognosis in PNP deficiency. This report presents a new PNP deficiency case successfully transplanted without a conditioning regimen from an HLA-identical family donor, who developed a complication of disseminated BCG infection.
- Subjects :
- Autoimmunity
Bone Marrow Transplantation adverse effects
Hematopoietic Stem Cell Transplantation adverse effects
Humans
Infant
Male
Mycobacterium Infections complications
Mycobacterium Infections etiology
Mycobacterium bovis metabolism
Recurrence
Transplantation Conditioning adverse effects
Treatment Outcome
Bone Marrow Transplantation methods
Hematopoietic Stem Cell Transplantation methods
Purine-Nucleoside Phosphorylase deficiency
Purine-Pyrimidine Metabolism, Inborn Errors therapy
Transplantation Conditioning methods
Subjects
Details
- Language :
- English
- ISSN :
- 1399-3046
- Volume :
- 12
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Pediatric transplantation
- Publication Type :
- Academic Journal
- Accession number :
- 18208442
- Full Text :
- https://doi.org/10.1111/j.1399-3046.2007.00890.x