Deformidad vertebral en el Síndrome de Noonan: revisión de la literatura. A propósito de dos casos

El síndrome de Noonan es un trastorno autosómico dominante, caracterizado por talla baja, cardiopatía congénita (la alteración congénita más frecuente), dimorfimo facial y alteraciones esqueléticas [1,2].Entre las anomalías vertebrales descritas en la literatura la escoliosis es la más frecuentemente descrita con prevalencias que oscilan entre el 10 y 15% de los casos [4-8]. El diagnóstico es clínico. Actualmente no tiene tratamiento específio. Presentamos dos casos de escoliosis asociadas al síndrome de Noonan atendidos en nuestro servicio

Effects of the intensity of prehospital treatment on short-term outcomes in patients with acute heart failure. the SEMICA-2 study

Objective: Little is known about treatments provided by advanced life support (ALS) ambulance teams to patients with acute heart failure (AHF) during the prehospital phase, and their influence on short-term outcome. We evaluated the effect of prehospital care in consecutive patients diagnosed with AHF in Spanish emergency departments (EDs). Methods: We selected patients from the EAHFE registry arriving at the ED by ALS ambulances with available follow-up data. We recorded specific prehospital ALS treatments (supplemental oxygen, diuretics, nitroglycerin, non-invasive ventilation) and patients were grouped according to whether they received low- (LIPHT; 0/1 treatments) or high-intensity prehospital therapy (HIPHT; > 1 treatment) for AHF. We also recorded 46 covariates. The primary endpoint was all-cause 7-day mortality, and secondary endpoints were prolonged hospitalisation (> 10 days) and in-hospital and 30-day mortality. Unadjusted and adjusted odds ratios were calculated to compare the groups. Results: We included 1493 patients [mean age 80.7 (10) years; women 54.8%]. Prehospital treatment included supplemental oxygen in 71.2%, diuretics in 27.9%, nitroglycerin in 13.5%, and non-invasive ventilation in 5.3%. The LIPHT group included 1041 patients (70.0%) with an unadjusted OR for 7-day mortality of 1.770 (95% CI 1.115–2.811; p = 0.016), and 1.939 (95% CI 1.114–3.287, p = 0.014) after adjustment for 16 discordant covariables. The adjusted ORs for all secondary endpoints were always > 1 in the LIPHT group, but none reached statistical significance. Conclusions: Patients finally diagnosed with AHF at then ED that have received LIPHT by the ALS ambulance teams have a poorer short-term outcome, especially during the first 7 days