Hepatic GVHD after HLA-haploidentical bone marrow transplantation in children with severe combined immunodeficiency: the effect of ursodeoxycholic acid
| Autor: | N. M. Wulffraat, B. R. De Graeff-Meeder, Malika Benkerrou, Elie Haddad, Alain Fischer, Natacha Patey, W. G. M. Spliet |
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| Rok vydání: | 1997 |
| Předmět: |
Male
Cholagogues and Choleretics Bilirubin medicine.medical_treatment Graft vs Host Disease Bile Duct Diseases Skin Diseases chemistry.chemical_compound Adrenal Cortex Hormones Cyclosporin a medicine Humans Bone Marrow Transplantation Severe combined immunodeficiency Chemotherapy medicine.diagnostic_test business.industry Liver Diseases Vanishing bile duct syndrome Ursodeoxycholic Acid Infant Newborn Infant Hematology medicine.disease Ciclosporin Fibrosis Ursodeoxycholic acid chemistry Liver biopsy Immunology Cyclosporine Severe Combined Immunodeficiency business Immunosuppressive Agents medicine.drug |
| Zdroj: | British journal of haematology. 96(4) |
| ISSN: | 0007-1048 |
| Popis: | We describe two children with a severe combined immune deficiency (SCID) with B cells. Following a T-cell-depleted haploidentical bone marrow transplantation (BMT), they both developed a chronic graft-versus-host disease (GVHD) of the skin and a severe persisting hyperbilirubinaemia and elevated liver enzymes. The diagnosis of a vanishing bile duct syndrome was confirmed by liver biopsies. Because corticosteroids and cyclosporin A induced only a partial response, ursodeoxycholic acid (UDCA) was added to their treatment schedule. Serum bilirubin and liver enzymes returned to normal within months. A control liver biopsy showed normal and proliferating bile ducts without cholestatic damage. We conclude that UDCA was well tolerated and may be of value as an additional treatment for hepatic GVHD in SCID. |
| Databáze: | OpenAIRE |
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