[Intraoperative change of anesthetic technique in a patient with intermediate Werdning Hoffman (type II) disease].
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Biomedical subjects
Publications and source records attributed to F Gilsanz Rodríguez.
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OBJECTIVES: To assess the usefulness of tranexamic acid (TA) in pediatric cranial remodeling surgery, by analyzing its effects on bleeding and transfusion requirements, number of days of cranial drainage required, and time spent in the postoperative recovery unit. MATERIAL AND METHOD: A single-blind, controlled study was designed with 20 patients (10 cases and 10 controls) randomly assigned to receive or not receive 15 mg/kg of intravenous TA upon anesthetic induction, every 4 hours during surgery, and every 8 hours throughout the 48 hours after surgery. Variables analyzed were results of blood tests, blood loss, volume transfused, time in the recovery unit, and complications related to TA infusion. RESULTS: The group treated with TA experienced less bleeding during surgery than did the controls (199 +/- 60 vs 290 +/- 43 mL) and had less need of intraoperative (176 +/- 104 vs 206 +/- 70 mL) and postoperative transfusion (9 +/- 28 vs 52 +/- 72 mL) 24 hours after surgery. TA group patients also spent less time in the recovery unit (60 +/- 14 vs 72 +/- 11 hours). Blood test variables in TA-treated children were also better 24 hours after surgery with regard to hemoglobin (12.1 +/- 2 vs 11.6 +/- 1.3 mg/dL) and platelet (261 +/- 68.5 vs 181.6 +/- 58.1 platelets/mm3) concentrations, and cephalin time (33 +/- 12 vs 49 +/- 16 seconds). No complications related to TA treatment were observed. CONCLUSIONS: TA can reduce perioperative bleeding in the context of pediatric cranial remodeling surgery. TA-treated patients have less need of transfusion and this may reduce the rate of related complications as well a make care more efficient.
Thalassemia intermedia is a clinical entity characterized by moderate, non-transfusional anemia and hepatosplenomegaly. This phenotype can result from different genetic combinations and is sometimes present in patients with only one parent showing the thalasemia minor phenotype. We report here a family with seven members with the thalassemia beta trait, four of them with thalasemia minor and the other three with thalassemia intermedia. The genetic study of patients with thalassemia intermedia revealed the presence of the mutation IVS-1 1(G-->A) in the beta-globin gene and the heterozygous triplication of the alpha-globin gene, an uncommon association in Spain. The interaction of a mutation in the beta-globin gene with triplication of the alpha-globin gene should be considered in the diagnosis and genetic counselling in those patients with thalassemia intermedia and one normal parent.
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