Clinical practice guidelines for the diagnosis and management of Charcot-Marie-Tooth disease.

INTRODUCTION: Charcot-Marie-Tooth disease (CMT) is classified according to neurophysiological and histological findings, the inheritance pattern, and the underlying genetic defect. The objective of these guidelines is to offer recommendations for the diagnosis, prognosis, follow-up, and treatment of this disease in Spain. MATERIAL AND METHODS: These consensus guidelines were developed through collaboration by a multidisciplinary panel encompassing a broad group of experts on the subject, including neurologists, paediatric neurologists, geneticists, physiatrists, and orthopaedic surgeons. RECOMMENDATIONS: The diagnosis of CMT is clinical, with patients usually presenting a common or classical phenotype. Clinical assessment should be followed by an appropriate neurophysiological study; specific recommendations are established for the parameters that should be included. Genetic diagnosis should be approached sequentially; once PMP22 duplication has been ruled out, if appropriate, a next-generation sequencing study should be considered, taking into account the limitations of the available techniques. To date, no pharmacological disease-modifying treatment is available, but symptomatic management, guided by a multidiciplinary team, is important, as is proper rehabilitation and orthopaedic management. The latter should be initiated early to identify and improve the patient's functional deficits, and should include individualised exercise guidelines, orthotic adaptation, and assessment of conservative surgeries such as tendon transfer. The follow-up of patients with CMT is exclusively clinical, and ancillary testing is not necessary in routine clinical practice.

Quality of life and persistent symptoms after hospitalization for COVID-19. A prospective observational study comparing ICU with non-ICU patients.

BACKGROUND: Hospitalized COVID-19 patients are prone to develop persistent symptoms and to show reduced quality of life following hospital admission. METHODS: Prospective cohort study of COVID-19 patients admitted to a hospital from March 1 to April 30, 2020. The primary outcome was to compare health related quality of life and persistent symptoms six months after hospital admission, of COVID-19 patients who required ICU admission with those who did not. RESULTS: Among the 242 patients hospitalized during the defined period of time, 44 (18.2%) needed ICU admission. Forty (16.5%) patients died during hospital admission. Two hundred and two (83.5%) patients were discharged alive from the hospital. At six months, 183 (75.6%) patients completed the questionnaires (32 ICU patients and 151 non ICU patients). Ninety-six (52.4%) reported decreased quality of life and 143 (78.1%) described persistent symptoms. More ICU patients showed worsening of their quality of life (71.9% vs 43.7%, P=0.004). There were no differences in the proportion of patients with persistent symptoms between ICU and non ICU patients (87.5% vs 76.2%, P=0.159). ICU patients showed more frequently dyspnea on exertion (78.1% vs 47.7%, P=0.02), dyspnea on light exertion (37.5% vs 4.6%, P<0.001), and asthenia (56.3 vs 29.1, P=0.003). CONCLUSIONS: Survivors of COVID-19 needing hospitalization had persistent symptoms and a decline in the quality of life. ICU patients referred a large decrease of their quality of life compared with non ICU patients.

[Quality of life and persistent symptoms after hospitalization for COVID-19. A prospective observational study comparing ICU with non-ICU patients]. / Calidad de vida y síntomas persistentes tras hospitalización por COVID-19. Estudio observacional prospectivo comparando pacientes con o sin ingreso en UCI.

Background: Hospitalized COVID-19 patients are prone to develop persistent symptoms and to show reduced quality of life following hospital admission. Methods: Prospective cohort study of COVID-19 patients admitted to a hospital from March 1 to April 30, 2020. The primary outcome was to compare health related quality of life and persistent symptoms six months after hospital admission, of COVID-19 patients who required ICU admission with those who did not. Results: Among the 242 patients hospitalized during the defined period of time, 44 (18.2%) needed ICU admission. Forty (16.5%) patients died during hospital admission. Two hundred and two (83.5%) patients were discharged alive from the hospital. At six months, 183 (75.6%) patients completed the questionnaires (32 ICU patients and 151 non ICU patients). Ninety-six (52.4%) reported decreased quality of life and 143 (78.1%) described persistent symptoms. More ICU patients showed worsening of their quality of life (71.9 vs. 43.7%, P = 0.004). There were no differences in the proportion of patients with persistent symptoms between ICU and non ICU patients (87.5 vs. 76.2%, P = 0.159). ICU patients showed more frequently dyspnea on exertion (78.1 vs. 47.7%, P = 0.02), dyspnea on light exertion (37.5 vs. 4.6%, P < 0.001), and asthenia (56.3 vs. 29.1, P = 0.003). Conclusions: Survivors of COVID-19 needing hospitalization had persistent symptoms and a decline in the quality of life. ICU patients referred a large decrease of their quality of life compared with non ICU patients.

Sarcopenia, frailty, cognitive impairment and mortality in elderly patients with non-valvular atrial fibrillation. / Sarcopenia, fragilidad, deterioro cognitivo y mortalidad en pacientes ancianos con fibrilación auricular no valvular.

OBJECTIVES: To determine the prevalence of sarcopenia, frailty and cognitive impairment in elderly patients with nonvalvular atrial fibrillation (NVAF) and the factors' influence on survival. METHODS: Prospective, multicentre cohort study of patients older than 75 years with NVAF hospitalised in internal medicine departments in Spain. For each patient, we recorded the creatinine, haemoglobin and platelet levels, the scores on the CHA2DS2-VASc and HAS-BLED scales and Charlson index, as well as the use of oral anticoagulants. We measured sarcopenia with the SARC-F scale, frailty with the FRAIL scale and cognitive impairment with the Short Portable Mental State Questionnaire. We also conducted a 1-year follow-up. RESULTS: The study included 596 patients with NVAF, with a mean age of 84.9 (SD: 5.2) years. Of these, 295 (49.5%) presented sarcopenia, 305 (51.2%) presented frailty, and 251 (42.1%) presented cognitive impairment. At the end of 1year, 226 (37.9%) patients had died. Mortality was greater for the patients with sarcopenia, frailty and cognitive impairment. In the multivariate analysis, sarcopenia (HR: 1.775; 95%CI: 1.270-2.481), age, comorbidity and a history of peripheral embolism were associated with increased mortality, and the use of oral anticoagulants at discharge (HR: 0.415; 95%CI: 0.307-0.560) was associated with lower mortality. CONCLUSIONS: Sarcopenia, frailty and cognitive impairment are very common in elderly patients with NVAF and are frequently associated. Sarcopenia was associated with increased mortality.

Peroxisome proliferator-activated receptor-alpha reduces inflammation and vascular leakage in a murine model of acute lung injury.

Acute lung injury (ALI) still represents a major cause of morbidity and mortality in intensive care units. Beneficial effects have been described after activation of the peroxisome proliferator-activated receptor (PPAR)-alpha by fibrates such as WY 14,643 (WY) in inflammatory models. In the present study, the impact of WY was investigated in a model of endotoxin (lipopolysaccharide; LPS)-induced ALI in mice. Intratracheal LPS challenge dose-dependently resulted in leukocyte invasion, protein leakage and release of tumour necrosis factor-alpha as well as macrophage inflammatory protein-2, prostaglandin E(2) and thromboxane B(2) into the alveolar space after 8 and 24 h. Lung ventilator compliance was reduced at both time-points. In isolated perfused mouse lungs, platelet-activating factor (PAF) induced an acute increase in pulmonary artery pressure (P(pa)) and in capillary filtration coefficient (K(fc)). WY significantly improved all features of ALI in vivo and blunted the increase in K(fc) in isolated perfused mice lungs. In mice with genetic deletion of PPAR-alpha, all characteristics of ALI, P(pa), and K(fc) were not significantly different from wild-type mice but WY failed to improve ALI and PAF-induced increase in K(fc). Activation of peroxisome proliferator-activated receptor-alpha by WY 14,643 reduced acute lung injury and vascular leakage. Fibrates may possess beneficial effects in acute pulmonary diseases beyond their lipid-lowering capability.

Massive right hemothorax due to idiopathic spontaneous rupture of a phrenic artery following cardiac surgery. / Hemotórax masivo derecho por rotura espontánea idiopática de una arteria frénica tras cirugía cardiaca.

We report a case of a 35-years-old man who presented a massive haemothorax and hypovolemic shock following cardiac surgery, from spontaneous rupture of a phrenic artery. A quick diagnosis and immediate intervention is crucial to manage the patient.

Baseline functional status as the strongest predictor of in-hospital mortality in elderly patients with non-valvular atrial fibrillation: Results of the NONAVASC registry.

OBJECTIVES: Atrial fibrillation (AF) has been associated with higher mortality. We aimed to identify the baseline predictors of in-hospital mortality among elderly patients with non-valvular AF (NVAF) hospitalised for any reason. METHODS: Observational, prospective and multicentre study was carried out on patients with NVAF over the age of 75, who had been admitted for any acute medical condition to Internal Medicine departments in Spain. RESULTS: We evaluated 804 patients with a mean age of 85±5.1years, of which 53.9% were females. During the hospitalization 10.1% (n=81) of the patients died. The patients who died were older, had a greater percentage of institutionalization, worse previous basic functional status (Barthel Index), worse cognitive performance at admission and greater proportion of frailty and sarcopenia. Logistic regression multivariate analysis identified that the strongest determinants of in-hospital mortality were the baseline functional status (Barthel Index) (OR for total dependency 4.73, 95% CI 2.32-9.63), and admissions for stroke (OR 3.55, 95% CI 1.41-8.90) and acute renal failure (OR 1.93, 95% CI 1.12-3.32). CONCLUSION: The overall in-hospital mortality of elderly patients with NVFA is high. Among all factors evaluated in the global geriatric assessment the baseline functional status was the strongest predictor for in-hospital mortality on this population.

Asthma exacerbations: risk factors for hospital readmissions.

OBJECTIVE: The aim of our study is to analyse hospital readmissions due to asthma, as well as the factors associated with their increase. STUDY DESIGN: We carried out a retrospective study including all admissions of patients over 18 years old due to exacerbation of asthma occurring in our hospital between the years 2000 and 2010. METHODS: The data were gathered by two members of the research team, by reviewing the clinical records. The first hospital admission of each patient was included for this study. An early readmission (ER) was defined as that which occurred in the following 15 days after hospital discharge and late readmission (LR) to that occurring from 16 days after discharge. RESULTS: This study included 2166 hospital admissions and 1316 patients, with a mean age of 62.6 years. Of the 1316 patients analysed, 36 (2.7%) had one ER and 313 (23.8%) one LR. The only factor independently associated with a higher probability of an ER was poor lung function. A higher probability of LR was associated with a greater severity of the asthma (OR: 17.8, for severe asthma versus intermittent asthma), to have had any hospital admission in the previous year (OR: 3.5) and the use of a combination of ICS-LABA as maintenance treatment. CONCLUSIONS: About 25% of the patients in our area admitted to hospital due to asthma exacerbation had repeat episodes of hospitalisation.

Hyponatraemia and congestive heart failure refractory to diuretic treatment. Utility of tolvaptan. / Hiponatremia e insuficiencia cardiaca congestiva refractaria a tratamiento diurético. Utilidad del tolvaptán.

Heart failure (HF) is currently one of the most significant healthcare problems in Spain and has a continuously increasing prevalence. Advances in our understanding of the various biological responses that promote cardiac remodelling and pulmonary venous congestion constitute the basis of current treatment. This article, prepared by members of the HF groups of the Spanish Society of Cardiology and the Spanish Society of Internal Medicine, discusses the current therapeutic strategies for patients with congestion refractory to diuretic treatment. The article includes our clinical experience with the use of tolvaptan as an additional treatment for congestion associated with hyponatraemia. To this end, we propose an algorithm for the use of tolvaptan in patients with congestive HF, natraemia <130mEq/l and poor response to conventional diuretic treatment.

Tuberculous pleurisy: a study of 254 patients.

OBJECTIVES: To determine the age at which tuberculous pleural effusions occur, the radiological and biochemical characteristics of the effusions, the sensitivities of the various diagnostic tests, and the utility of combining clinical, radiological, and analytic data in diagnosis. METHODS: We studied the case histories of 254 patients in whom tuberculous pleural effusions were diagnosed with certainty between January 1, 1989, and June 30, 1997, in a Spanish university hospital in a region with a high incidence of tuberculosis. RESULTS: The mean (+/-SD) age of the patients was 34.1+/-18.1 years, and 62.2% were younger than 35 years. The effusion was on the right side in 55.9% of patients, on the left side in 42.5% of patients, and on both sides in 1.6% of patients. In 81.5% of patients, less than two thirds of the hemithorax was affected. Associated pulmonary lesions were detected in 18.9% of patients, of whom 14.6% exhibited cavitation. In 93.3% of the effusions, more than 50% of leukocytes were lymphocytes, and almost all had the biologic characteristics of exudates (98.8% had high total protein contents, 94.9% had high cholesterol levels, and 82.3% had high lactate dehydrogenase levels). All but 1 effusion (99.6%) had an adenosine deaminase (ADA) concentration higher than 47 U/L, 96.8% (123/127) of the effusions had high ADA2 levels, and 89% (73/82) of the effusions had high interferon gamma levels. Adenosine deaminase 2 contributed 72.2%+/-12.5% (mean +/- SD) of total ADA activity. Total ADA activity was significantly correlated with ADA2 (r = 0.83) and with interferon gamma (r = 0.30) levels. Definitive diagnosis was based on the observation of caseous granulomas in pleural biopsy tissue samples in 79.8% of patients, on the results of biopsy cultures in 11.7% of patients, and on pleural effusion cultures in the remaining 8.5% of patients. Results of the tuberculin skin test were positive in only 66.5% of patients. CONCLUSIONS: In these patients, lymphocyte-rich exudative pleural effusions occurred, on average, at a young age, with no preference for either the right or the left side; normally affected no more than two thirds of the hemithorax; and were generally unaccompanied by pulmonary infiltrates. High ADA concentration was a highly sensitive diagnostic sign and was caused by a rise in ADA2 concentration. The most sensitive criterion based on pleural biopsy was the observation of caseous granulomas, and culture of biopsy material further increased overall sensitivity. Negative skin test results were no guarantee of the effusion being nontuberculous. This, together with the low mean age of the patients and the low frequency of associated pulmonary lesions, suggests that tuberculous pleural effusion is a primary form of tuberculosis in this region.

Dual function of the hemagglutinin H5 fused to chicken CD154 in a potential strategy of DIVA against avian influenza disease: preliminary study.

In this study we demonstrated that the vaccine candidate against avian influenza virus H5N1 based on the hemagglutinin H5 (HA) fused to the chicken CD154 (HACD) can also be used for differentiating infected from vaccinated animals (DIVA). As the strategy of DIVA requires at least two proteins, we obtained a variant of the nucleoprotein (NP49-375) in E. coli. After its purification by IMAC, the competence of the proteins NP49-375 and HACD as coating antigens in indirect ELISA assays were tested by using the sera of chickens immunized with the proteins HA and HACD and the reference sera from several avian influenza subtypes. Together with these sera, the sera from different species of birds and the sera of chickens infected with other avian viral diseases were analyzed by competition ELISA assays coated with the proteins NP49-375 and HACD. The results showed that the segment CD154 in the chimeric protein HACD did not interfere with the recognition of the molecule HA by its specific antibodies. Also, we observed variable detection levels when the reference sera were analyzed in the ELISA plates coated with the protein NP49-375. Moreover, only the antibodies of the reference serum subtype H5 were detected in the ELISA plates coated with the protein HACD. The competition ELISA assays showed percentages of inhibition of 88-91% for the positives sera and less than 20% for the negative sera. We fixed the cut-off value of these assays at 25%. No antibody detection was observed in the sera from different species of birds or the sera of chickens infected with other avian viral diseases. This study supported the fact that the ELISA assays using the proteins NP49-375 and HACD could be valuable tools for avian influenza surveillance and as a strategy of DIVA for counteracting the highly pathogenic avian influenza virus H5N1 outbreaks.

Reduction of QT and QTc dispersion during long-term treatment of systemic hypertension with enalapril.

We report, in conjunction with other findings, the evolution of the dispersion of QT and QTc in patients who for the last 7 years have been treated with enalapril for systemic hypertension with left ventricular (LV) hypertrophy. Twenty-four essential hypertensive patients who had received no previous treatment took enalapril (20 mg twice daily) for 7 years. In a pretreatment placebo phase and 8 weeks and 1, 3, 5, 6, and 7 years after the start of therapy, cardiovascular parameters were determined by two-dimensional guided M-mode echocardiography, and the QT interval and corrected QT interval (QTc) and their dispersions were obtained from amplified standard 12-lead electrocardiograms. Therapy rapidly reduced blood pressure (BP) from 156/105 mm Hg to normal values; at 7-year follow-up, BP was 130/84 mm Hg (p <0.001 with respect to the placebo phase). LV mass index decreased progressively until at 5-year follow-up the reduction had reached 39% (p <0.001), after which neither LV mass index nor any structural parameter underwent any further significant change. LV pump function was also significantly better after 7 years of treatment. During this time, QT and QTc decreased significantly, as did the dispersion of both QT (from 61+/-21 to 37+/-14 ms) and QTc (from 67+/-27 to 41+/-16 ms). We conclude that long-term enalapril treatment of hypertensive patients with LV hypertrophy not only induces marked regression of LV mass and improved LV systolic function, but also reduces the dispersions of QT and QTc, which probably reduces the likelihood of ventricular arrhythmias and improves prognosis.

The etiology of pleural effusions in an area with high incidence of tuberculosis.

To investigate the etiology of pleural effusions in our region, we undertook a prospective study of patients with this condition in our centers. During a 5-year period, we studied 642 pleural effusion patients aged 57.1 +/- 21.1 years, of whom 401 were men aged 56.5 +/- 21 years and 241 were women aged 57.8 +/- 21.4 years; the male/female ratio was 1.6:1. The most frequent cause of pleural effusion was tuberculosis (25%), followed by neoplasia (22.9%) and congestive heart failure (17.9%). The etiology of 48 cases (7.5%) remained uncertain. In the neoplastic effusion group, the most frequent locations of the primary tumor were lung (32.6%), breast (11.5%), lymphoma (10.8%), and ovary (7.5%); in 21 cases (14.3% of the neoplastic group), it was not possible to identify the primary tumor. The 111 patients aged younger than 40 years with tuberculous effusions made up 69.4% of tuberculous effusion cases and the same percentage of patients younger than 40 years; the proportion of effusions that were tuberculous peaked in the 11- to 30-year-old age group and declined steadily thereafter. Of the patients with neoplastic effusions, 83% were older than 50 years; the proportion of effusions that were neoplastic rose steadily from zero in the 0- to 30-year-old age group to a peak among 60- to 70-year-olds. The age-wise distribution of effusions secondary to congestive heart failure was similar to that of neoplastic effusions. Of the effusions secondary to congestive heart failure, 86% (99/115) affected the right pleura or both, and 83% of effusions secondary to pulmonary thromboembolism (15/18) affected the right side. Neoplastic, tuberculous, parapneumonic, empyematous, and other exudative effusions showed no preference for either side. Of the 97 bilateral effusions, 77 (79.4%) were secondary to heart failure (59, 60.8%) or neoplasia (18, 18.6%). We conclude that in our region, the most frequent cause of pleural effusion is tuberculosis, followed by neoplasia and congestive heart failure. We suggest that all those interested in pleural disease should determine the etiologic pattern of pleural effusion in their region with a view to the adoption of regionally optimized diagnostic and therapeutic attitudes.

Cholesterol: a useful parameter for distinguishing between pleural exudates and transudates.

Previously established criteria were used to classify 253 pleural effusions as transudates (65 cases), neoplastic exudates (67 cases), tuberculous exudates (65 cases), or miscellaneous exudate (56 cases). The parameters pleural LDH (PLDH), pleural LDH/serum LDH ratio (P/SLDH), and pleural protein/serum protein ratio (P/SPROT) were compared with pleural cholesterol (PCHOL) and the pleural cholesterol/serum cholesterol ratio (P/SCHOL) with regard to their usefulness for distinguishing between pleural exudates and transudates. The PCHOL values determined were 28.5 +/- 12.8 mg/dl for transudates, 88.1 +/- 30 mg/dl for neoplastic exudates, 96.5 +/- 28 mg/dl for tuberculous exudates, and 88 +/- 35.9 mg/dl for the miscellaneous group; the differences between the transudate group and the others are statistically significant (p less than 0.001). The sensitivity and specificity of P/SPROT for diagnosis of exudates were both 89 percent; the sensitivity of PLDH was 67 percent and its specificity was 95 percent; the sensitivity and specificity of P/SLDH were both 84.6 percent. Using Light's three criteria as a battery, the sensitivity was 94.6 percent and its specificity was 78.4 percent. All the transudates and 17 (9 percent) of the 188 exudates had PCHOL values below 55 mg/dl, so that with this threshold, PCHOL had a sensitivity of 91 percent and a specificity of 100 percent for diagnosis of exudates. With a threshold of 0.3, P/SCHOL had a sensitivity of 92.5 percent and a specificity of 87.6 percent. The number of misclassifications by PCHOL was less than with any other of the parameters, with statistically significant differences with respect to PLDH (p less than 0.001) and P/SLDH (p less than 0.01). We conclude that determination of PCHOL and P/SCHOL is of great value for distinguishing between pleural exudates and transudates, and should be included in routine laboratory analysis of pleural effusions.

Diagnosis of tuberculous pleurisy using the biologic parameters adenosine deaminase, lysozyme, and interferon gamma.

We compared the parameters pleural adenosine deaminase (PADA, determined in 405 patients), the PADA/serum ADA ratio (P/SADA; 276 cases), pleural lysozyme (PLYS, 276 cases), the PLYS/serum LYS ratio (P/SLYS; 276 cases), and pleural interferon gamma (IFN, 145 cases) regarding their ability to differentiate tuberculous pleural effusions from others. The 405 pleural effusions were classified by previously established criteria as tuberculous (91), neoplastic (110), parapneumonic (58), empyemas (10), transudates (88), or miscellaneous (48). The intermean differences between the tuberculous group and each of the others were statistically significant for all five parameters (p < 0.01 for PLYS and P/SLYS with respect to the empyema group; p < 0.001 otherwise), except for PADA and P/SADA with respect to the empyema group. All the tuberculous pleurisy cases had PADA values of 47 U/L or more, as compared to only 5 percent of the other cases (sensitivity, 100 percent; specificity, 95 percent). P/SADA was above 1.5 in 85.7 percent of tuberculous effusions and 11 percent of the others (sensitivity, 85.7 percent; specificity, 89 percent). PLYS, with a diagnostic threshold of 15 g/ml, had a sensitivity of 85.7 percent and a specificity of 61.6 percent; P/SLYS, with a threshold of 1.1, had a sensitivity of 67.3 percent and a specificity of 90.3 percent; and IFN, with a threshold of 140 pg/ml, had a sensitivity of 94.2 percent and a specificity of 91.8 percent. The lowest misclassification rate was achieved by PADA, with statistically significant differences (p < 0.001) with respect to P/SADA, PLYS, and P/SLYS, but not with respect to IFN. The only significant pairwise correlations among these parameters were between P/SLYS and PADA and between P/SLYS and P/SADA. We conclude that PADA and IFN are useful parameters for early diagnosis of tuberculous pleurisy, and that the other parameters considered have no advantages over PADA and IFN for this purpose (though the high specificity of P/SLYS may be noted).

Cirrhosis of the liver, an exceptional cause of chylothorax: two cases.

Chylothorax, a condition featuring an infrequent form of pleural effusion, is generally caused by tumours or traumatism. Only about 1% of chylothorax cases are caused by cirrhosis of the liver. Two such cases are described in these case reports.

[Hypertensive encephalopathy: contribution of magnetic resonance]. / Encefalopatía hipertensiva: aportación de la resonancia magnética.

We report a patient with hypertensive encephalopathy and we analyze his clinical and neurological imaging peculiarities. Computed tomography showed hypodense corticosubcortical lesions, whereas magnetic resonance scan disclosed extensive, wholly reversible enhanced signal lesions due to cerebral edema.

[Lung cancer in the Pontevedra health area: incidence, clinical presentation and survival]. / Cancer de pulmon en el area sanitaria de Pontevedra: incidencia, presentación clinica y supervivencia.

BACKGROUND: To describe the clinical and epidemiological characteristics, and survival of patients with lung cancer (LC) in the Pontevedra Health Area. METHODS: A retrospective observational study was conducted on all patients with a diagnosis of LC in the Pontevedra Hospital Complex (CHOP) health area over a three-year period. The data recorded included, age, gender, smoking history, comorbidity, functional status, diagnostic method, histology type, stage, treatment received, and survival. The patients were followed up for 3 years. RESULTS: A total of 358 cases of LC were included in the study, which gave a crude incidence rate adjusted to the standard European population of 37.33/100,000 inhabitants/year in males and 4.88/100,000 inhabitants/year in females. The large majority were males (87%). The mean age was 68.7 years, and 82% were smokers or ex-smokers. The most common histology type was epidermoid, with 35.2% of the cases. The diagnosis was made in stages III-B or IV in 79% of cases. Chemotherapy was the first treatment in 53% of the cases. Survival after the first year was 25%, which decreased to 4% at the third year. CONCLUSIONS: The incidence of LC in our health area continues to be mainly in male smokers, although the incidence in females and in people who never smoked is higher than in other populations in our country. It is diagnosed in the advance stages of the disease, and survival is poor.

[Infant mortality in a third level paediatric hospital. therapeutic effort limitation, clinical-pathological agreement and diagnostic accuracy]. / Mortalidad infantil en un hospital de nivel terciario. Limitación de esfuerzo terapéutico, correspondencia clínico-patológica y precisión diagnóstica.

OBJECTIVE: To study infant and child mortality in a third level children's hospital treating highly complex patients. PATIENTS AND METHODS: All children dying in the period 2007- 2009 at La Paz Children's Hospital were evaluated. Epidemiological data, autopsy rate, clinical and autopsy diagnoses and their correspondence and the number of, patients with precise final diagnoses were analysed. Therapeutic effort limitation and palliative care were also evaluated as well as if the final result was expected according to the initial disease or clinical condition of the patients. All the variables were prospectively defined at the start of the study period. RESULTS: A total of 253 cases (6.08‰ admissions) were analysed. The two leading causes of death were disorders related to prematurity and low birth weight, and haematology oncology malignant diseases. Most patients (87%) died in an intensive care unit (neonatal or paediatric). During the study period 134 autopsies (53%) were performed, and new clinically significant findings were observed in 12 of these (7.8%) but in only one case the treatment could have possibly modified the prognosis (class I discrepancy). Therapeutic effort limitation and palliative care were implemented in 41.9%. Death was initially expected in 83.9% of cases. An accurate final diagnosis was defined in 92%, and the aetiology of the disease was considered to be identified in 86.4% of all deaths. CONCLUSIONS: Hospital mortality analysis is useful to evaluate the quality of the paediatric care and to detect adverse results that could be corrected. Paediatric autopsy continues to provide clinically significant data for paediatricians and families. Therapeutic effort limitation and palliative care is increasingly applied in paediatric end of life care. The number of infants and children dying without a final aetiological diagnosis is still considerably high.

Evaluación de las competencias clínicas adquiridas por los estudiantes en Terapia Intensiva y Emergencias: análisis de 10 años en la Universidad de Buenos Aires / Competency evaluation clinics acquired by students in Intensive Therapy and Emergencies: analysis of 10 years at the University of Buenos Aires

Introducción: La educación médica debería garantizar la formación de profesionales competentes que posean los conocimientos, las destrezas y las actitudes planificados en la capacitación. En general, los programas no tienen en cuenta a la urgencia, que no figura como materia específica en los programas de grado. Objetivos: Cuantificar las competencias adquiridas por los internos en el Departamento de Urgencias y en Terapia Intensiva, estableciendo el grado de cumplimiento entre lo planificado y lo logrado; desglosar el grado de cumplimiento según las diferentes áreas de la competencia clínica. Materiales y Métodos: Diseño prospectivo, de observación, longitudinal, analítico. Se incluyeron 93 alumnos del ciclo clínico que realizaron el internado rotatorio (2000-2009). Se valoró el cumplimiento de 37 competencias que se clasificaron por área: actitudes, conocimientos y destrezas, en números de 9, 5 y 23, respectivamente. Resultados: El cumplimiento global de las competencias en el área de emergentología y terapia intensiva es del 61,18% (IC95% 59,20-63,13). Al desglosar por área de la competencia clínica, se halla que las actitudes fueron del 66,26%; los conocimientos, del 63,28% y las destrezas, del 58,15%. Clasificándolas según el grado de cumplimiento, se establece una escala con cumplimiento alto (76-100%), medio (51-75%) y bajo (<51%). Conclusiones: Los aspectos actitudinales y cognitivos de las competencias tuvieron un cumplimiento medio. La mitad de las competencias vinculadas a destrezas tuvo un cumplimiento medio-alto.(AU)

Introduction: Medical education should guarantee the formation of competent professionals with the knowledge, skills and attitudes planned in training. Generally programs do not bear in mind the urgency, which does not appear as a specific subject in the university. Objectives: To quantify the competitions acquired by the boarders in the Emergency Department and the Intensive Care Unit, establishing the degree of fulfillment between what was planned and what was achieved; to perceive the degree of fulfillment according to the different areas of clinical competition. Materials and Methods: Prospective, observational, longitudinal and analytical study. We included 93 students from the clinical cycle who performed rotating boarding school (2000-2009). Fulfillment of 37 competitions classified by area: attitudes, knowledge and skills, in numbers of 9, 5 and 23, respectively, was evaluated. Results: The global fulfillment of the competitions was 61.18% (CI95%: 59.20-63.13). Specifically, in each area of the clinical competition, we found that the attitudes were 66.26%, knowledge 63.28% and skills 58.15%. Classifying them according to the degree of fulfillment, a scale is established: high (76-100%), medium (51-75%) and low (<51%). Conclusions: Attitude and cognitive aspects of the competitions had average fulfillment. Half of the competitions linked to skills had average-high fulfillment.(AU)