Resumo A Distrofia Miotônica (DM) tipo-1 (Doença de Steinert) é uma doença multissistêmica progressiva autossômica dominante em que a crise miotônica pode ser desencadeada por vários fatores, incluindo dor, estresse emocional, hipotermia, tremores e estímulo mecânico ou elétrico. O presente relato descreve anestesia geral realizada com dexmedetomidina em combinação com peridural torácica para colecistectomia laparoscópica em paciente com Doença de Steinert. Para evitar laringoscopia, a intubação traqueal foi realizada utilizando cateter de intubação Aintree guiado por broncofibroscopia óptica. Os efeitos anestésicos prolongados do propofol foram revertidos e a recuperação anestésica foi acelerada pelo uso de infusão intravenosa de teofilina.

Abstract Myotonic dystrophy type-1 (Steinert disease) is an autosomal dominant, progressive multisystem disease in which myotonic crisis can be triggered by several factors including pain, emotional stress, hypothermia, shivering, and mechanical or electrical stimulation. In this report, dexmedetomidine-based general anesthesia, in combination with a thoracic epidural for laparoscopic cholecystectomy in a patient with Steinert disease, is presented. An Aintree intubation catheter with the guidance of a fiberoptic bronchoscope was used for intubation to avoid laryngoscopy. Prolonged anesthetic effects of propofol were reversed, and recovery from anesthesia was accelerated using an intravenous infusion of theophylline.

Abstract Introduction: Heparin-induced thrombocytopenia (HIT) is a potentially lethal complication of unfractionated or low-molecular weight heparin therapy. We aimed to determine the incidence and mortality rate of patients with positive heparin/platelet factor 4 (PF4) antibodies, which is a rapid detection test of HIT. Methods: Coronary artery bypass grafting and mitral and aortic valve surgeries were evaluated. Cardiopulmonary bypass was employed in all patients. The diagnosis of HIT was based on immunological assays. Postoperative complications, mortality rates, and the causes of death were specified in patients with positive heparin/PF4 antibodies. Results: Postoperative thrombocytopenia was detected in 257 patients. Twenty of these patients undergoing open heart surgery were included in the final analysis. Antibodies against heparin/PF4 complex were positive in 20 patients. The mean body mass index was 28.8±2.3 kg/m2, mean value of left ventricular ejection fraction was 48.3±6.7%, cardiopulmonary bypass time was 113.0±35.0 min, aortic cross-clamping time was 88.0±32.7 min, mean intensive care unit length of stay was 10.9±4.9 days, mean preoperative platelet count was 307.250±88528 platelets/microliter, and mean postoperative platelet count was 243.050±89.354 platelets/microliter. The mean duration of heparin exposure was 6.9±2.9 days. The mortality rate was 45% (nine patients) and 1.2% (three patients) in heparin/PF4 complex positive and negative patients, respectively. Conclusion: Although the incidence of HIT was low in patients undergoing open heart surgery, an increased rate of early mortality was observed in patients with positive heparin/PF4 antibodies.