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Matches in SemOpenAlex for { <https://semopenalex.org/work/W1995449711> ?p ?o ?g. }

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• W1995449711 abstract "A 4 year old African American female presented with sun sensitivity related skin lesions and hepatomegaly with elevated liver enzymes after a trip to the family beach home. Diagnostic work up revealed elevated porphyrin levels consistent with erythropoietic protoporphyria with liver involvement. By 8 years of age, she had developed end-stage liver failure with jaundice, coagulopathy, hypoalbuminemia and portal hypertension. Liver transplantation alone was not a curative option for her, as her primary disorder would eventually cause a transplanted organ to fail. Due to her failing hepatic function we felt that she would not survive stem cell transplant alone. We decided to pursue liver transplantation followed by stem cell transplantation. Approximately 11 months after deceased donor liver transplantation, a double cord blood allogeneic transplant was performed. The patient was conditioned with busulfan (0.8 mg/kg/dose q 6 × 16 doses on days -9 to -6), cyclophosphamide 50 mg/kg q 24 hours × 4 doses on days -5 to -2), and equine ATG (15 mg/kg q 24 hours × 3 doses on days -3 to -1). GVH prophylaxis was provided with cyclosporine and mycophenolate. The two cord blood units had the following characteristics: both units were 4/6 matches. The first unit was an allelic mismatch at B and DRB1, while the second unit was a B antigen mismatch and DRB1 allelic mismatch. The second unit was the ultimately engrafting unit. The patient was CMV negative, the engrafting donor was CMV positive. Neutrophil engraftment occured on day +26; platelet engraftment on day +170. Transplant was complicated by several episodes of cholangitis and bacteremia. The patient had grade II acute skin GVH after her transplant, with grade III acute gut GVH, controlled with oral steroids, oral budesonide, mycophenolate, etanercept, and cyclosporine. 89 days post cord blood transplant, zinc protoporphyrin levels have fallen from pre transplantation levels of >800 mcg/dL to 139 micrograms/dL. 197 days post cord blood cell transplantation, the patient has normal hepatic synthetic function, total bilirubin of <0.1 mg/dL, no evidence of hepatic graft rejection, subjective improvement in overall skin coloration, cessation of porphyria related pain, and no current active GVH. This represents to our knowledge the first pediatric serial liver followed by double cord blood transplant for erythropoietic protoporphyria." @default.
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• W1995449711 date "2010-02-01" @default.
• W1995449711 modified "2023-09-24" @default.
• W1995449711 title "Orthotopic Liver Transplantation Followed By Double Cord Blood Transplantation In A Patient With Erythropoietic Protoporphyria" @default.
• W1995449711 doi "https://doi.org/10.1016/j.bbmt.2009.12.413" @default.
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