The Role of Liver Transplantation in Propionic Acidemia.

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.

Treatment of Non-Contained Infrabony Defects With Enamel Matrix Derivative Alone or in Combination With Biphasic Calcium Phosphate Bone Graft: A 12-Month Randomized Controlled Clinical Trial.

BACKGROUND: Use of enamel matrix derivative (EMD) when dealing with non-contained defects may be limited because EMD does not maintain a space itself. Use of combined therapy has been proposed, using a bone graft in combination with EMD to avoid collapse of the flap into the bony defect during healing time. Therefore, the aim of this study is to evaluate the clinical and radiologic healing response of non-contained infrabony defects after treatment with a combination of EMD and biphasic calcium phosphate (BC) or EMD alone. METHODS: Fifty-two patients with at least one infrabony defect ≥3 mm in depth with a probing depth (PD) ≥6 mm were randomly treated with EMD/BC or EMD alone. Clinical and radiographic parameters were evaluated at baseline, 6, and 12 months after surgery. To standardize the procedure, an acrylic stent and millimeter radiographic grid were used. The primary outcome was the change in clinical attachment level (CAL). RESULTS: Analysis of the data demonstrated a statistically significant difference from baseline within each group (P <0.05), with a difference in clinical and radiographic parameters at 6 and 12 months. After 1 year, mean PD reductions of 3.14 ± 1.95 mm (39.6%) in the EMD/BC group and 3.30 ± 1.89 mm (48.7%) in the EMD group were achieved. A mean CAL gain of 2.38 ± 2.17 mm (24.9%) in the EMD/BC group and 2.65 ± 2.18 mm (36.2%) in the EMD group were obtained. Reduction in the infrabony component was 2.71 ± 1.79 mm (57.9%) in the test group and 2.60 ± 2.03 mm (28.5%) in the control group. There were no statistically significant differences between treatment groups. CONCLUSIONS: It was concluded that treatment of non-contained infrabony defects with EMD, with or without BC, resulted in statistically significantly better results after 12 months compared with baseline measurements. In contrast, the combined approach did not result in a statistically significant improvement.