title: Long-term outcome of one haploidentical live related donor transplantation in children.
abstract: We report on 69 children, 6 months to 18 years old, who received a one-haploidentical live related donor transplantation from 11/72 through 4/89. Thirty patients were maternal and 22 were paternal recipients; 23 were Black, 27 Caucasian, 17 Hispanic, and 1 each Oriental and Arabic. The original diagnoses in these patients were aplastic/dysplastic kidneys 13%, obstructive uropathy 30%, focal segmental glomerulosclerosis (FSGS) 23% and lupus nephritis 10%.
  
  
  RESULTS
  1-, 5-, 10- and 15-year actuarial patient and graft survivals were 98, 84, 84, 84% and 94, 70, 41, 30% respectively. Actual 5-year graft survival of paternal recipients was better than maternal recipients (p less than 0.05). No differences were noted comparing actuarial graft survival between Blacks, Caucasians and Hispanics. 1/16 FSGS and 1/7 lupus nephritis patients had recurrence of their original disease. Posttransplant therapy in 37 patients consisted of azathioprine and prednisone while 32 patients received ciclosporin A (CSA) and prednisone. At transplantation, CSA and prednisone patients were younger, i.e., 10.7 years (range 0.5-18) compared to 13.3 years (range 4-18) and (p less than 0.03) in azathioprine and prednisone patients. The 5-year actuarial graft survival of azathioprine and prednisone patients was 57% compared to 76% in CSA and prednisone patients. 16/32 CSA and prednisone patients had their prednisone discontinued for a mean duration of 18 months (3-54 months). Ten of these 16 patients were prepubertal and had their growth hormone levels measured. All had an adequate level (greater than or equal to 10 ng/ml) poststimulation. Five of the 10 patients were less than 6 years of age and exhibited accelerated growth.(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Renal Division, Children's Medical Center, Brooklyn, N.Y.
authors.forename: E
authors.initials: E
authors.lastname: Ingulli
authors.forename: R
authors.initials: R
authors.lastname: Gonzalez
authors.forename: D
authors.initials: D
authors.lastname: Rajpoot
authors.forename: A
authors.initials: A
authors.lastname: Pomrantz
authors.forename: N
authors.initials: N
authors.lastname: Sumrani
authors.forename: J
authors.initials: J
authors.lastname: Hong
authors.forename: K M
authors.initials: KM
authors.lastname: Butt
authors.forename: A
authors.initials: A
authors.lastname: Tejani
mesh_terms: D000293:Adolescent; D001379:Azathioprine / Q000627:therapeutic use; D002648:Child; D002657:Child Development / Q000187:drug effects; D002675:Child, Preschool; D003524:Cyclosporins / Q000627:therapeutic use; D005190:Family; D005220:Fathers; D005260:Female; D006085:Graft Survival; D006128:Growth / Q000187:drug effects; D006238:Haploidy; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000627:therapeutic use; D007165:Immunosuppression Therapy; D007223:Infant; D007674:Kidney Diseases / Q000401:mortality / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D009035:Mothers; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use; D014019:Tissue Donors
chemical_list: D003524:Cyclosporins; D001379:Azathioprine; D011241:Prednisone; D006854:Hydrocortisone