Multifocal and diffuse lesions express GLUT-1, which may biologically Tyrosine Kinase Inhibitor Library distinguish

them from focal lesions.[200] With diffuse lesions, the liver is almost completely occupied by hemangiomas and symptoms include respiratory insufficiency due to an abdominal mass effect, abdominal compartment syndrome, coagulopathy (Kasabach-Merritt syndrome), multiorgan system failure, and hypothyroidism due to overproduction of type 3 iodothyronine deiodinase which converts thyroid hormone to its inactive form.[201] Diffuse hemangiomas often do not respond to steroid therapy[202] and most require surgical resection or beta-blocker therapy to improve hematologic parameters. Hepatic artery ligation and embolization have limited effect. Other treatment options for diffuse lesions include vincristine,[203] actinomycin, and cyclophosphamide and propranolol.[204] 48. Liver transplant evaluation for IH is indicated if the hemangioendothelioma is not responding to treatment or is associated with life-threatening complications. (1-B) 49. Candidates being considered for LT for a hemangioendothelioma should be screened

for hypothyroidism. (2-B) Liver disease is present in up to 35% of cystic fibrosis (CF) patients, but only 5-10% of patients have Trametinib cirrhosis.[205, 206] Ursodeoxycholic acid therapy is recommended, although its impact on the progression of CFLD is not known.[207-209] Endstage liver disease is characterized by coagulopathy and hypoalbuminemia and is not attributable 上海皓元医药股份有限公司 to malabsorption. Portal hypertensive-related hemorrhage alone, in the absence of other signs of decompensated liver disease, may not be a sufficient indication for LT in CF patients, as alternative therapies may be equally acceptable.[210-212] Optimal timing for isolated LT involves careful assessment of cardiopulmonary function, infections,

and nutritional status in CF patients. Currently, Model for Endstage Liver Disease (MELD) / Pediatric Endstage Liver Disease (PELD) exception points are permitted for those patients with CF whose pulmonary function tests (PFTs) are <40% of predicted FEV1.[213, 214] Five-year posttransplant survival rates for CFLD are lower than for those who underwent transplantation for other etiologies. Compared to those patients remaining on the waiting list, pediatric and adult transplant recipients with CF gained a significant survival benefit.[215] A different analysis of the UNOS database and various single center reports convey similar patient and graft survival data among patients with CF.[212, 216, 217] While LT may improve pulmonary function and nutritional status,[218, 219] CF patients may be at higher relative risk for the development of posttransplant diabetes mellitus and renal impairment.[220-223] 50. The indications for liver transplantation in CF are guided by the degree of hepatic synthetic failure and the presence of otherwise unmanageable complications of portal hypertension.