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| Content Provider | PubMed Central |
|---|---|
| Author | Khawaja, K. Gennery, A. Flood, T. Abinun, M. Cant, A. |
| Abstract | BACKGROUND—CD40 ligand(CD40L) deficiency is a rare X linked immunodeficiency disorder leadingto recurrent bacterial infection, with cryptosporidial enteritis andsubsequent hepatic cirrhosis. Bone marrow transplantation offers theonly cure. OBJECTIVE—To analyseretrospectively the outcome of bone marrow transplantation for thiscondition in one centre. DESIGN—A retrospectivecase note analysis was performed, identifying all patients with CD40Ldeficiency who had undergone bone marrow transplantation between May1988 and December 2000. Details of pre-existing infection,pretransplantation immunological and infective data, transplantprocedure (particularly donor type and HLA match), conditioningregimen, and marrow manipulation were analysed. Post-transplantation data including infective episodes, engraftment details, immune function, complications, and outcome were recorded. RESULTS—Eight boys(age 1-14 years, median 5.75) had transplants. Six received T celldepleted unrelated donor marrow. Four survive and have normal immunefunction. Six had previous Pneumocystis carinii pneumonia and three had histological liver damage.Survival was associated with younger age at transplantation and normal liver histology. CONCLUSIONS—Bonemarrow transplantation can be curative in CD40L deficiency. Betteroutcome is associated with younger age at transplantation and normalliver histology. |
| Related Links | http://dx.doi.org/10.1136/adc.84.6.508 |
| Ending Page | 511 |
| Page Count | 4 |
| Starting Page | 508 |
| File Format | |
| ISSN | 00039888 |
| e-ISSN | 14682044 |
| Journal | Archives of Disease in Childhood |
| Issue Number | 6 |
| Volume Number | 84 |
| Language | English |
| Publisher | BMJ Group |
| Publisher Date | 2001-06-01 |
| Access Restriction | Open |
| Rights Holder | BMJ Group |
| Subject Keyword | Obstetrics and Gynaecology Pediatrics, Perinatology, and Child Health Research in Higher Education |
| Content Type | Text |
| Resource Type | Article |
| Subject | Pediatrics, Perinatology and Child Health |
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