title: [Lung transplantation and hemophilia. Preoperative considerations].
abstract: Lung transplantation is indicated in certain patients with terminal pulmonary disease. We report a case in which a single lung (left) was transplanted to a 16-year-old girl with hemophilia B; she also suffered idiopathic pulmonary fibrosis and had a history of malnutrition, osteoporosis, severe scoliosis, hepatitis C positivity and recurrent bilateral pneumothorax. Treatment with pure factor IX was started the moment the donor lung was available and was continued for 37 days after surgery. Plasma levels of factor IX were kept at 100% during surgery and in the early postoperative period, and over 40% after that time. Correct hemostasis was thus achieved throughout the procedure, with no need for blood products. Patient outcome was satisfactory. The stay in the intensive care recovery ward was 17 days and discharge was 40 days after transplantation. We discuss aspects of hemophilia and lung transplantation, and the influence on malnutrition, chronic steroid treatment and osteoporosis.
authors: Servicio de Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitari La Fe, Valencia. jbarrioz@medvnet.com
authors: J
authors: J
authors: Barrio
authors: C
authors: C
authors: Añó
authors: R
authors: R
authors: Vicente
authors: F
authors: F
authors: Ramos
authors: J I
authors: JI
authors: Lorenzo
authors: J
authors: J
authors: Argente
authors: M A
authors: MA
authors: Chiveli
authors: R
authors: R
authors: Alepuz
authors: R
authors: R
authors: Montero
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000009:adverse effects / Q000627:therapeutic use; D000768:Anesthesia, General / Q000379:methods; D007611:Aprotinin / Q000627:therapeutic use; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D005112:Extracorporeal Circulation; D005164:Factor IX / Q000627:therapeutic use*; D005260:Female; D006331:Heart Diseases / Q000150:complications; D002836:Hemophilia B / Q000150:complications*; D006526:Hepatitis C / Q000150:complications; D006801:Humans; D016040:Lung Transplantation; D016343:Monitoring, Intraoperative; D010024:Osteoporosis / Q000139:chemically induced / Q000150:complications; D011030:Pneumothorax / Q000150:complications; D019106:Postoperative Hemorrhage / Q000517:prevention & control*; D011229:Preanesthetic Medication; D011300:Preoperative Care / Q000379:methods*; D011502:Protein-Energy Malnutrition / Q000150:complications; D011658:Pulmonary Fibrosis / Q000150:complications / Q000601:surgery*; D012306:Risk; D012600:Scoliosis / Q000150:complications
chemical_list: D000305:Adrenal Cortex Hormones; D005164:Factor IX; D007611:Aprotinin