ABSTRACT
HPS is defined as arterial hypoxemia because of pulmonary vasodilation as a result of cirrhotic or non-cirrhotic portal hypertension. This report describes a teenager with HPS because of primary sclerosing cholangitis/autoimmune hepatitis overlap syndrome requiring OLT. HPS resolved completely within three months of OLT, but recurred again at 12 months post-OLT following liver dysfunction secondary to a biliary stricture. She underwent a second OLT successfully and remains well two yr and three months post-second OLT. Recurrent HPS after OLT may occur because of graft dysfunction and as this novel case illustrates, retransplantation may lead to a successful outcome.
Subject(s)
Hepatopulmonary Syndrome/surgery , Liver Transplantation/methods , Adolescent , Angiography , Cholangiopancreatography, Endoscopic Retrograde , Female , Follow-Up Studies , Hepatopulmonary Syndrome/diagnosis , Humans , Recurrence , Reoperation , Severity of Illness Index , Tomography, X-Ray ComputedABSTRACT
This report describes a teenager who developed aplastic anemia (AA) because of non-A-E acute liver failure (ALF) requiring orthotopic liver transplantation (OLT). His AA did not recover spontaneously and he required treatment with ATG 9 months post-OLT. Bone marrow recovery occurred 4 months after immunotherapy and coincided with further intensification of immunusuppression required to treat early chronic rejection of the liver graft. Three years post-OLT he remains well with good bone marrow and liver function. Intensification of immunosuppression can lead to successful resolution of AA associated with non-A-E ALF.
Subject(s)
Anemia, Aplastic/etiology , Anemia, Aplastic/surgery , Liver Failure, Acute/complications , Liver Failure, Acute/surgery , Liver Transplantation , Adolescent , Antilymphocyte Serum/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Liver Function Tests , MaleABSTRACT
A 7-yr-old boy presented with obstructive jaundice secondary to an inflammatory myofibroblastic tumor centered on the hepatic hilum and extending into the liver. The tumor was further complicated by portal vein phlebitis and occlusion. Attempted resection of the tumor with portal vein reconstruction and bilioenteric drainage was unsuccessful and he required urgent orthotopic liver transplantation. In contrast to more peripheral inflammatory myofibroblastic tumors in the liver, hilar lesions are locally aggressive, causing occlusive portal phlebitis and biliary obstruction. Successful management may include the need for liver transplantation.
Subject(s)
Liver Neoplasms/surgery , Liver Transplantation , Neoplasms, Muscle Tissue/surgery , Child , Humans , Inflammation/pathology , Jaundice, Obstructive/etiology , Liver/pathology , Liver/surgery , Liver Neoplasms/complications , Magnetic Resonance Imaging , Male , Neoplasms, Muscle Tissue/complications , Neoplasms, Muscle Tissue/pathology , Portal Vein/diagnostic imaging , Portal Vein/pathology , Radiography , Treatment OutcomeABSTRACT
This report describes a 16-month-old girl with multi-system Langerhans cell histiocytosis (LCH), who developed end-stage liver disease despite intensive chemotherapy. She underwent a liver transplant at 28 months of age while receiving maintenance chemotherapy for bony lesions. In view of previous reports of a high incidence of acute cellular rejection and post-transplant lymphoproliferative disease (PTLD) in children transplanted for LCH, basiliximab was added to the post-transplant immunosuppression regime of tacrolimus and prednisolone. Sixteen months post-transplant, she has had no episodes of acute rejection or PTLD and her LCH has remained in remission. Current literature regarding liver transplantation (LTx) for LCH and the use of basiliximab in pediatric LTx is reviewed.