Despite optimal medical treatment and strict low-protein diet, the prognosis of propionic acidemia (PA) patients is generally poor. We aim to report our experience with liver transplantation (LT) in the management of PA patients. Six patients with PA received a LT at a mean age of 5.2 years (1.3-7.5 years). The indications for LT were frequent metabolic decompensations in the first 4 patients and preventative in the last 2 patients. Two patients presented hepatic artery thromboses that were solved through an interventional radiologist approach. These patients showed a very high procoagulant state that was observed by thromboelastography. Arterial vasospasm without thrombus was observed in 2 patients during the LT surgery. In order to avoid hepatic artery thrombosis, an arterial conduit from the recipient aorta to the hepatic artery of the donor was used in the fifth patient. After LT, patients presented improvement in propionyl byproducts without complete normalization, but no decompensations have been observed. In conclusion, LT could be a good therapeutic option to improve the metabolic control and the quality of life of PA patients. Improved surgical strategies along with new techniques of interventional radiology allow us to perform the LT minimizing the complications derived from the higher risk of hepatic artery thrombosis.
eHAT after LT remains a life-threatening complication. In the majority of anticoagulation protocols, heparin is used to prevent thromboses. Our study aimed to monitor AT-III levels in the early post-LT period to assess the need for the administration of AT-III concentrate to ensure the effectiveness of heparin. We monitored coagulation daily by measuring INR, APTT, fibrinogen, platelets, and AT-III. Anticoagulation therapy consisted of LMWH, AT-III, and dipyridamole. AT-III concentrate was administered when AT-III activity was ≤60%. DUS was performed daily for the first five post-operative days or whenever vascular thrombosis was suspected. Between October 2007 and October 2011, 39 LT were performed in our center. The median age was 26 months (6-196) with a median weight of 9 kg (5.5-49). AT-III activity was ≤60% in 27 patients. Lower levels were particularly observed in partial grafts and recipients weighing less than 10 kg. Patent arterial flow was present in all 39 LT during the first five post-operative days. AT-III levels were low in 70% of pediatric patients following LT, thereby risking heparin ineffectiveness. These results may implicate low AT-III levels in the etiology of eHAT post-LT. However, this is a small single-center pilot study and further larger prospective trials are required to confirm these results.
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