Graft-vs-host disease after small bowel transplantation in children.
OBJECTIVE
Graft-vs-host disease (GVHD) is a rare complication of transplantation of organs rich in immunocompetent cells. The goal of this study was to report the features of GVHD after small bowel transplantation (SBTx) in children.


METHODS
The study involved a retrospective review of patients undergoing SBTx between 1999 and 2009 who had GVHD.


RESULTS
Of 46 children receiving 52 intestinal grafts (2 liver-intestine and 3 multivisceral), 5 (10%) developed GVHD. Median age at transplant was 42 (19-204) months. Baseline immunosupression consisted of tacrolimus and steroids supplemented with thymoglobulin (n = 2) or basiliximab (n = 3) for induction. Median time between transplantation and GVHD was 47 (16-333) days. All patients had generalized rash, 2 had diarrhea, and 2 had respiratory symptoms. Other symptoms were glomerulonephritis (n = 1) and conjunctivitis (n = 1). Four developed severe hematologic disorders. The diagnosis was confirmed by skin biopsy in 4 patients and supported by chimerism studies in two. Colonoscopy and opthalmoscopic findings were also suggestive in one. Treatment consisted of steroids and decrease of tacrolimus, with partial response in four. Other immunosuppressants were used in refractory or recurrent cases. Three patients died within 4 months after diagnosis.


CONCLUSIONS
Graft-vs-host disease is a devastating complication of SBTx, with high mortality probably associated with severe immunologic dysregulation.
Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de Castellana 261, 28046 Madrid, Spain. aneandresmo@hotmail.com
Ane M
AM
Andres
Manuel Lopez
ML
Santamaría
Esther
E
Ramos
Jesus
J
Sarriá
Manuel
M
Molina
Francisco
F
Hernandez
Jose L
JL
Encinas
Javier
J
Larrauri
Gerardo
G
Prieto
Juan Antonio
JA
Tovar
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000961:Antilymphocyte Serum; D000077552:Basiliximab; D002648:Child; D002675:Child, Preschool; D046528:Chimerism; D003131:Combined Modality Therapy; D005260:Female; D006084:Graft Rejection / Q000209:etiology / Q000276:immunology; D006085:Graft Survival; D006086:Graft vs Host Disease / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D006801:Humans; D007108:Immune Tolerance; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007421:Intestine, Small / Q000276:immunology / Q000637:transplantation*; D008297:Male; D011183:Postoperative Complications / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D012189:Retrospective Studies; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D000961:Antilymphocyte Serum; D007166:Immunosuppressive Agents; D011993:Recombinant Fusion Proteins; D000077552:Basiliximab; C512542:thymoglobulin; D016559:Tacrolimus