First case of X-linked dystonia-parkinsonism ("Lubag") to demonstrate a response to bilateral pallidal stimulation.

"Lubag" or X-linked dystonia-parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45-year-old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw-opening dystonia. At 1-year follow-up, his Burke-Fahn-Marsden dystonia score and motor Unified Parkinson's Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms.

Blood loss in infants and children for open heart operations: albumin 5% versus fresh-frozen plasma in the prime.

BACKGROUND: Infants and children undergoing cardiopulmonary bypass become substantially hemodiluted secondary to the volume used to prime the oxygenator. Fresh-frozen plasma has been included in the prime to lessen dilution of clotting factors and correspondingly minimize blood loss and transfusions. METHODS: We prospectively randomized 56 patients weighing 10 kg or less who required cardiopulmonary bypass to receive either one unit of fresh-frozen plasma or 200 mL of albumin 5% in the prime. After protamine administration, samples for prothrombin time, fibrinogen, platelet count, and thromboelastogram were obtained. Mediastinal chest tube drainage and transfusion requirements were documented. RESULTS: There were no significant differences between groups regarding demographic or surgical characteristics. Blood loss during the first 24 hours was similar in both groups, but total transfusions were significantly greater in those who received fresh-frozen plasma instead of albumin 5% in the prime (8.0 +/- 4.2 versus 6.1 +/- 4.5 U, respectively; p = 0.035). Post hoc analyses suggest that for cyanotic patients and patients undergoing complex operations, fresh-frozen plasma in the prime results in less blood loss than albumin 5%. CONCLUSIONS: Substitution of albumin 5% for fresh-frozen plasma in the prime of acyanotic patients weighing 10 kg or less who undergo noncomplex operations requiring cardiopulmonary bypass significantly reduces perioperative transfusions without increasing blood loss. Further investigation is needed to determine whether increased blood loss is associated with increased transfusions when albumin 5% is substituted for fresh-frozen plasma in the prime of infants and children who are cyanotic or undergoing complex operations.