Linked Life Data

16189444

Source:http://linkedlifedata.com/resource/pubmed/id/16189444

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
2005-9-28
pubmed:abstractText
Malignant infantile osteopetrosis (MIOP) is a lethal disorder caused by osteoclast dysfunction. The only curative therapy for MIOP is stem cell transplantation (SCT). Because the number of patients is limited, the conditioning regimen and the use of alternative donors for SCT have been controversial and not established. The authors report a case of successful cord blood transplantation (CBT) with a nonmyeloablative regimen (NMR) for MIOP. The patient was a 9-month-old girl with MIOP. Before this diagnosis, she had received chemotherapy under the tentative diagnosis of juvenile myelomonocytic leukemia. She was on mechanical ventilation with tracheotomy due to the progression of MIOP when CBT with NMR was undergone. The conditioning regimen included fludarabine, melphalan, and antithymocyte globulin. Cyclosporine A and methylprednisolone were used for prophylaxis for graft-versus-host disease. Neutrophil engraftment was achieved on day 26 after SCT and has been fully maintained up to the present. Although grade 3 graft-versus-host disease and hepatic veno-occlusive disease occurred, both were controllable. Although the pretransplant condition of our patient was somewhat unusual, this is the first reported case of successful CBT with NMR for MIOP. Because of the urgent need, CBT can be considered as one of the SCT sources for MIOP, especially in a severe, life-threatening setting.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
1077-4114
pubmed:author
pubmed:issnType
Print
pubmed:volume
27
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
495-8
pubmed:dateRevised
2011-10-6
pubmed:meshHeading
pubmed:year
2005
pubmed:articleTitle
Successful nonmyeloablative cord blood transplantation for an infant with malignant infantile osteopetrosis.
pubmed:affiliation
Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan. ytsuji@ndmc.ac.jp
pubmed:publicationType
Journal Article, Case Reports