Un profesor clínico y pedagogo en el Aprendizaje Basado en Problemas / A clinician and pedagogy professor for problem-based learning

Introducción: Las cualificaciones requeridas a un profesor tienen implicaciones en la vinculación laboral, el desarrollo profesoral y la calidad de la formación del recurso humano en salud. Objetivo: Determinar la experticia de un profesor y si existe evidencia empírica de que sus antecedentes en Aprendizaje Basado en Problemas influyan en el aprendizaje de los estudiantes. Métodos: En esta revisión de alcance se buscaron artículos publicados en las bases de datos Medline, Scopus, PubMmed, Google Scholar, EBSCO y ScienceDirect. La búsqueda incluyó, inicialmente, artículos publicados entre 2015 y 2020; luego, se incluyeron los estudios citados en estos artículos para aumentar el número de la muestra. La evaluación fue realizada de manera independiente por dos investigadores. En total, 11 artículos cumplían con los criterios de inclusión. Se extrajeron y analizaron datos sobre las cualidades del profesor del ABP. Resultados: Los estudios que exploran las cualificaciones del docente del Aprendizaje Basado en Problemas son escasos y no existe un criterio unificado sobre la formación y la experiencia requeridas a ellos. Las evidencias empíricas encontradas son cuestionables y muestran vacíos en la comprensión de este problema, aunque sugieren como mejor opción a un profesor experto en medicina y pedagogía. Conclusiones: Estos hallazgos representan un serio defecto del Aprendizaje Basado en Problemas. Una posible solución es integrar la enseñanza de la pedagogía a los currículos médicos para contar con una mayor cantidad de profesores expertos tanto en medicina como en docencia(U)

Introduction: The qualifications required from a professor have implications for job placement, professional development and the quality of health human resource training. Objective: To determine a professor's expertise and whether there is empirical evidence that his or her background in problem-based learning (PBL) influences student learning. Methods: In this scoping review, published articles were searched for in the Medline, Scopus, PubMed, Google Scholar, EBSCO and ScienceDirect databases. The search initially included articles published between 2015 and 2020; then, studies cited in these articles were included to increase the sample number. The assessment was performed independently by two researchers. A sheer number of eleven articles met the inclusion criteria. Data about the qualities of the PBL professor were extracted and analyzed. Results: Studies exploring the qualifications of the PBL professor are scarce, while there is no unified criterion on the training and experience required from them. The empirical evidence found is questionable and shows gaps in the understanding of this problem, although it suggests a professor with expertise in medicine and pedagogy as the best option. Conclusions: These findings represent a serious shortcoming of PBL. A possible solution is to integrate the teaching of pedagogy into medical curricula in order to have a greater number of professors with expertise in both medicine and teaching(AU)

Evaluation of Two Different CMV-Immunoglobulin Regimens for Combined CMV Prophylaxis in High-Risk Patients following Lung Transplant.

BACKGROUND: The clinical benefits of the common off-label use of cytomegalovirus (CMV)-specific immunoglobulin (CMV-Ig) combined with antivirals in organ transplantation have not been previously assessed. The objective was to compare the risk of CMV infection and other post-transplantation outcomes between two CMV-Ig prophylaxis regimens in lung transplant recipients; Methods: Retrospective study of 124 donor CMV positive/recipient negative (D+/R-) patients receiving preventive ganciclovir/valganciclovir for 12 months, of whom 62 received adjunctive CMV-Ig as per label indication (short regimen [SR-Ig]; i.e., 7 doses over 2.5 months) and were compared to 62 who received an extended off-label regimen (ER-Ig) consisting of 17 doses over one year after transplantation. RESULTS: The incidence of CMV infection or disease, acute rejection, chronic lung allograft dysfunction, and survival did not differ between the two CMV-Ig schedules. Although the time to the first CMV infection after transplantation was shorter in the ER-Ig than in the SR-Ig adjunctive group (log-rank: p = 0.002), the risk was independently predicted by antiviral cessation (odds ratio = 3.74; 95% confidence interval = 1.04-13.51; p = 0.030), whereas the CMV-Ig schedule had no effect. CONCLUSIONS: Extending the adjunctive CMV-Ig prophylaxis beyond the manufacturer's recommendations up to one year does not confer additional clinical benefits regarding lung post-transplantation outcomes.

COVID-19 in lung transplant recipients: A multicenter study.

This study describes the clinical presentation, treatment, and outcomes of SARS-CoV-2 infection in lung transplant recipients (LTRs). This is a multicenter, retrospective study of all adult LTRs with confirmed SARS-CoV-2 infection from March 4 until April 28, 2020 in six Spanish reference hospitals for lung transplantation. Clinical and radiological data, treatment characteristics, and outcomes were reviewed. Forty-four cases were identified in that period. The median time from transplantation was 4.2 (interquartile range: 1.11-7.3) years. Chest radiography showed acute parenchymal abnormalities in 32 (73%) cases. Hydroxychloroquine was prescribed in 41 (93%), lopinavir/ritonavir (LPV/r) in 14 (32%), and tocilizumab in 19 (43%) patients. There was a strong interaction between tacrolimus and LPV/r in all cases. Thirty-seven (84%) patients required some degree of respiratory support and/or oxygen therapy, and 13 (30%) were admitted to intermediate or intensive critical care units. Seventeen (39%) patients had died and 20 (45%) had been discharged at the time of the last follow-up. Deceased patients had a worse respiratory status and chest X-ray on admission and presented with higher D-dimer, interleukin-6, and lactate dehydrogenase levels. In this multicenter LTR cohort, SARS-CoV-2 presented with high mortality. Additionally, the severity of disease on presentation predicted subsequent mortality.

Correlation between short-term air pollution exposure and unprovoked lung embolism. Prospective observational (Contamina-TEP Group).

BACKGROUND: The aim was to analyze the temporal relationship between short-term air pollution exposure and acute symptomatic unprovoked pulmonary embolism (PE). PATIENTS/METHODS: We performed a prospective, multicenter study in consecutive patients diagnosed with acute symptomatic unprovoked PE from February 2012 to January 2013. We analyzed demographic and clinical data, patients' addresses, meteorological and air pollutants data (PM10, SO2, CO, NO2, ozone emission data). We considered the number of days the patient had symptoms, and the study period constituted the previous 30 days. Likewise, the mean annual data of the reference season were calculated as well as the data of the 30-day study period corresponding to the same dates in the previous 3 years in order to obtain the monthly mean of the different pollutants for each period. RESULTS: A total of 162 patients with acute symptomatic PE were recruited (43.2% unprovoked PE). The air pollutants could be determined in 50% of the patients with unprovoked PE, and a final analysis was performed in 35 patients. In the multiple comparison analysis to verify a possible correlation between the study period and the annual median, only NO2 showed a statistically significant association (p = 0.009). When comparing the study period with the previous 3 years, only NO2 maintained a statistically significant association for the 3 study periods. CONCLUSIONS: We found a relationship between short-term exposure to NO2 and the presence of unprovoked PE.

Comparison of All-Cause Mortality Following VTE Treatment Between Propensity Score-Adjusted Observational Studies and Matched Randomized Controlled Trials: Meta-Epidemiologic Study.

BACKGROUND: It is unknown whether propensity score-adjusted observational studies produce results comparable to those of randomized controlled trials (RCTs) that address similar VTE treatment issues. METHODS: The PubMed and Web of Science databases were systematically searched for propensity score-adjusted observational studies, RCTs, and meta-analyses of RCTs that estimated all-cause mortality following VTE treatment. After identifying distinct clinical treatment issues evaluated in the eligible observational studies, a standardized algorithm was used to identify and match at least one RCT or RCT meta-analysis publication for paired study design analyses. Meta-analyses were used to summarize groups of studies. Treatment efficacy statistics (relative ORs) were compared between the paired observational and RCT studies, and the summary relative ORs for all study design pairs were also calculated. RESULTS: The observational and RCT study pairs assessed seven clinical treatment issues. Overall, the observational study-RCT pairs did not exhibit significantly different mortality estimates (summary relative OR, 0.89; 95% CI, 0.32-1.46; I2 = 23%). However, two of the seven treatment issue study pairs (thrombolysis vs anticoagulation for pulmonary embolism; once- vs twice-daily enoxaparin for VTE) exhibited a significantly different treatment effect direction, and there was a substantial (nonsignificant) difference in the magnitude of the effect in another two of the study pairs (rivaroxaban vs vitamin K antagonists for VTE; home treatment vs hospitalization for DVT). CONCLUSIONS: This systematic comparison across seven VTE treatment topics suggests that propensity score-adjusted observational studies and RCTs often exhibit similar all-cause mortality, although differences in the direction or the magnitude of estimated treatment effects may occasionally occur. TRIAL REGISTRY: PROSPERO; CRD42018087819; URL: http://www.crd.york.ac.uk/PROSPERO.

Catheter-directed aspiration thrombectomy and low-dose thrombolysis for patients with acute unstable pulmonary embolism: Prospective outcomes from a PE registry.

OBJECTIVES: To evaluate the efficacy and safety of aspiration thrombectomy in combination with low-dose catheter-directed thrombolysis for acute unstable pulmonary embolism (PE). BACKGROUND: Acute unstable (PE) is a life-threatening condition requiring treatment escalation, but many patients cannot receive full-dose systemic thrombolysis due to contraindications. METHODS: Eligible patients had a PE with sustained hypotension. We used a 115-cm, 8-F continuous aspiration mechanical thrombectomy catheter to perform mechanical thrombectomy, followed by catheter-directed thrombolysis with low-dose urokinase. The primary efficacy outcome was the change in the pulmonary artery pressure after aspiration thrombectomy and catheter-directed thrombolysis. Secondary efficacy outcomes were stabilization of hemodynamics post-procedure and survival to hospital discharge. The primary safety outcome was major procedure-related complications and major bleeding events. RESULTS: We included 54 patients with acute unstable PE. After thrombectomy, mean systolic pulmonary artery pressure decreased from 60.2 mm Hg to 55.2 mm Hg (P < 0.01), and to 40.5 mm Hg after catheter thrombolysis (P < 0.0001). The in-hospital PE-related death occurred in six patients (11%; 95% confidence interval [CI], 4.2-23%) at a mean follow-up of 1.1 days, and hemodynamics stabilized in the remaining 48 patients. Minor complications after thrombectomy included arrhythmias (4 of 48 patients, 8.3%; 95% CI, 2.3-20%), and minor bleeding episodes (3 of 48 patients; 6.2%; 95% CI, 1.3-17%). Major complication occurred in one patient (2.1%; 95% CI, 0.1-11%) who developed hemorrhagic transformation of paradoxical embolic stroke following catheter-directed thrombolysis. CONCLUSIONS: Aspiration thrombectomy followed by catheter-directed thrombolysis was overall effective and safe in treating patients with acute unstable PE.

Accuracy and Interobserver Reliability of the Simplified Pulmonary Embolism Severity Index Versus the Hestia Criteria for Patients With Pulmonary Embolism.

OBJECTIVES: The objective was to assess and compare the accuracy and interobserver reliability of the simplified Pulmonary Embolism Severity Index (sPESI) and the Hestia criteria for predicting short-term mortality in patients with pulmonary embolism (PE). METHODS: This prospective cohort study evaluated consecutive eligible adults with PE diagnosed in the emergency department (ED) at a large, tertiary, academic medical center in the era January 1, 2015, to December 30, 2017. We assessed and compared sPESI and Hestia criteria prognostic accuracy for 30-day all-cause mortality after PE diagnosis and their interobserver reliability for classifying patients as low risk or high risk. Two clinician investigators scored both prediction tools during the ED evaluation. We used the kappa statistic to test for agreement. RESULTS: The 488-patient cohort had a mean (±SD) age of 69.0 (±17.1) years and an approximately even sex distribution. The investigators classified one-quarter of patients as low risk using the sPESI and Hestia criteria (28% vs. 27%, respectively). During the 30-day follow-up, 31 of the 488 (6.4%) patients died. Patients classified as low risk according to the sPESI and the Hestia criteria had a similar 30-day mortality (sPESI 0.7% [1/135], 95% confidence interval [CI] = 0.0%-4.0%; Hestia 2.3% [3/132], 95% CI = 0.5%-6.5%). The two observers had good agreement (κ = 0.80) for the Hestia criteria and very good agreement (κ = 0.97) for the sPESI. CONCLUSION: The sPESI and the Hestia criteria had similar risk classification determination and prognostic accuracy for 30-day mortality after PE. However, the succinct and more objective sPESI had higher interobserver reliability than the Hestia criteria.

Meta-Analysis of Prevalence and Short-Term Prognosis of Hemodynamically Unstable Patients With Symptomatic Acute Pulmonary Embolism.

There remains limited information about the prevalence and outcomes of hemodynamic unstable patients with acute pulmonary embolism (PE). We performed a systematic review and meta-analysis of prospective registries that enrolled patients with acute PE to assess the prevalence and prognostic significance of hemodynamic instability for the primary outcome of short-term all-cause mortality, and the secondary outcome of short-term PE-related mortality. We also assessed the association between use of thrombolytic therapy versus no use and short-term outcomes in the subgroup of unstable patients. We used a random-effects model to pool study results; and I2 testing to assess for heterogeneity. The authors' search retrieved 4 studies that enrolled 1,574 patients with unstable PE (1,574/40,363; 3.9%; 95% confidence interval [CI], 3.7% to 4.1%). Hemodynamic instability had a significant association with short-term all-cause mortality (odds ratio [OR], 5.9; 95% CI, 2.7 to 13.0; I2 = 94%), and with PE-related death (OR, 8.2; 95% CI, 3.4 to 19.7). In unstable patients, thrombolytic therapy was associated with reduced odds of short-term all-cause mortality (OR, 0.69; 95% CI, 0.49 to 0.95), and PE-related death (OR, 0.66; 95% CI, 0.45 to 0.97). In conclusion, hemodynamic instability significantly increased the risk of death shortly after PE diagnosis. Use of thrombolytic therapy was associated with significantly reduced short-term mortality.

Cumplimiento de las medidas de bioseguridad en la unidad quirúrgica de cirugía ambulatoria / Biosecurity measures observance in the ambulatory surgical unit

RESUMEN Fundamento: los trabajadores de la salud que manipulan agentes infecciosos o materiales infectados deben conocer los posibles riesgos y ser expertos en su manipulación segura. Objetivo: evaluar el nivel de cumplimiento de las medidas de bioseguridad por el personal de salud en la unidad quirúrgica de cirugía ambulatoria. Método: se realizó un estudio observacional y descriptivo con un universo de 15 trabajadores de la unidad quirúrgica de cirugía ambulatoria, Hospital Provincial Universitario Celia Sánchez Manduley, Manzanillo, primer trimestre del 2017. El universo estuvo constituido por siete médicos cirujanos, seis licenciadas en enfermería y dos médicos anestesiólogos. La observación se organizó por una guía contentiva de los diferentes ítems para cada profesional por especialidad al establecer los puntajes. Resultados: las normas de bioseguridad que fueron cumplidas siempre se relacionaban con el aspecto personal, el uso de ropas quirúrgicas, el uso de medios de protección, el lavado de manos y el manejo de los desechos. El uso de dispositivos electrónicos y de joyas fueron las medidas de bioseguridad que menos se respetaron. El personal de salud tuvo un alto nivel de cumplimiento, ya que todos se posicionaron en el nivel tres. El nivel de cumplimiento que se presentó en médicos cirujanos, médicos anestesistas y licenciadas en enfermería fue el mismo. Conclusiones: el personal de salud de la unidad quirúrgica de cirugía ambulatoria cumplen, en su mayoría, con las normas de bioseguridad, donde el uso de joyas y equipos electrónicos la medida menos cumplida.

ABSTRACT Background: the health workers who manipulate the infectious agents or infected materials must know the possible risks and be experts in their sure manipulation. Objective: to evaluate the observance level of the biosecurity measures for the health personnel in the ambulatory surgical unit. Methods: an observational and descriptive study was carried out with a universe of 15 workers of the ambulatory surgical unit, Celia Sánchez Manduley Teaching Provincial Hospital, Manzanillo, Granma, in the first quarter of 2017. The universe was constituted by 7 medical surgeons, 6 graduated nurses and 2 anesthesiologists. The observation was organized for a guide contented of the different articles for each professional for specialty establishing the points. Results: the biosecurity norms that were completed were always related with the personal aspect, use of surgical clothes, use of protection means, washing of hands and handling of the waste. The use of electronic devices and of jewels was the biosecurity measures that were less respected. The health personnel had a high observance level, since all were positioned in the level three. The observance level that was presented in medical surgeons, anesthetists and nurses was the same one. Conclusions: the health surgical personnel of the ambulatory surgery unit, completes, in its majority, with the biosecurity norms being the use of jewels and electronic teams the less fulfilled measure.

Success in Optional Vena Cava Filter Retrieval. An Analysis of 246 Patients. / Éxito en la recuperación de filtro de vena cava inferior opcional: Análisis de 246 pacientes.

OBJECTIVE: This study assessed vena cava filter (VCF) retrieval rates and factors associated with retrieval failure in a single center cohort. METHODS: We conducted an observational retrospective cohort study. The primary endpoint was the percentage of patients whose VCF was retrieved. We performed logistic regression to identify variables associated with retrieval failure. RESULTS: During the study period, 246 patients received a VCF and met the eligibility requirements to be included in the study; 151 (61%) patients received a VCF due to contraindication to anticoagulation, 69 (28%) patients had venous thromboembolism (VTE) and a high risk of recurrence, and 26 (11%) patients received a filter due to recurrent VTE while on anticoagulant therapy. Of 236 patients who survived the first month after diagnosis of VTE, VCF was retrieved in 96%. Retrieval rates were significantly lower for patients with recurrent VTE while on anticoagulation, compared with patients with contraindication to anticoagulation or patients with a high risk of recurrence (79% vs. 97% vs. 100%, respectively; P<0.01). Mean time to retrieval attempt was significantly associated with retrieval failure (137.8 ± 65.3 vs. 46.3 ± 123.1 days, P<0.001). CONCLUSIONS: In this single center study, VCF retrieval success was 96%. A delay in the attempt to retrieve the VCF correlated significantly with retrieval failure.

Assessment of coexisting deep vein thrombosis for risk stratification of acute pulmonary embolism.

BACKGROUND: In patients with acute pulmonary embolism (PE), studies have shown an association between coexisting deep vein thrombosis (DVT) and short-term prognosis. It is not known whether complete compression ultrasound testing (CCUS) improves the risk stratification of their disease beyond the recommended prognostic models. METHODS: We included patients with normotensive acute symptomatic PE and prognosticated them with the European Society of Cardiology (ESC) risk model for PE. Subsequently, we determined the prognostic significance of coexisting DVT in patients with various ESC risk categories. The primary endpoint was a complicated course after the diagnosis of PE, defined as death from any cause, haemodynamic collapse, or adjudicated recurrent PE. RESULTS: According to the ESC model, 37% of patients were low-risk, 56% were intermediate-low risk, and 6.7% were intermediate-high risk. CCUS demonstrated coexisting DVT in 375 (44%) patients. Among the 313 patients with low-risk PE, coexisting DVT (46%) did not show a significant increased risk of complicated course (2.8%; 95% confidence interval [CI], 0.8%-7.0%), compared with those without DVT (0.6%; 95% CI, 0%-3.2%), (P = 0.18). Of the 478 patients with intermediate-low risk PE, a complicated course was 14% and 6.8% for those with and without DVT, respectively (P = 0.01). Of the 57 patients that had intermediate-high risk PE, a complicated course occurred in 17% and 18% for those with and without DVT, respectively (P = 1.0). CONCLUSIONS: In normotensive patients with PE, testing for coexisting DVT might improve risk stratification of patients at intermediate-low risk for short-term complications.

Correction: Lung and heart-lung transplantation in pulmonary arterial hypertension.

[This corrects the article DOI: 10.1371/journal.pone.0187811.].

Diclofenac for reversal of right ventricular dysfunction in acute normotensive pulmonary embolism: A pilot study.

BACKGROUND: The inflammatory response associated with acute pulmonary embolism (PE) contributes to the development of right ventricular (RV) dysfunction. Nonsteroidal anti-inflammatory drugs (NSAIDs) may facilitate the reversal of PE-associated RV dysfunction. METHODS: We randomly assigned normotensive patients who had acute PE associated with echocardiographic RV dysfunction and normal systemic blood pressure to receive intravenous (IV) diclofenac (two doses of 75mg in the first 24h after diagnosis) or IV placebo. All patients received standard anticoagulation with subcutaneous low-molecular-weight heparin (LMWH) and an oral vitamin K antagonist. RV dysfunction was defined by the presence of, at least, two of the following criteria: i) RV diastolic diameter>30mm in the parasternal window; ii) RV diameter>left ventricle diameter in the apical or subcostal space; iii) RV free wall hypokinesis; and iv) estimated pulmonary artery systolic pressure>30mmHg. Persistence of RV dysfunction at 48h and 7days after randomization were the primary and secondary efficacy outcomes, respectively. The primary safety outcome was major bleeding within 7days after randomization. RESULTS: Of the 34 patients randomly assigned to diclofenac or placebo, the intention-to-treat analysis showed persistent RV dysfunction at 48h in 59% (95% confidence interval [CI], 33-82%) of the diclofenac group and in 76% (95% CI, 50-93%) of the placebo group (difference in risk [diclofenac minus standard anticoagulation], -17 percentage points; 95% CI, -47 to 17). Similar proportions (35%) of patients in the diclofenac and placebo groups had persistent RV dysfunction at 7days. Major bleeding occurred in none of patients in the diclofenac group and in 5.9% (95% CI, 0.2-29%) of patient in the placebo group. CONCLUSIONS: Due to slow recruitment, our study is inconclusive as to a potential benefit of diclofenac over placebo to reverse RV dysfunction in normotensive patients with acute PE. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01590342.

Clinical gestalt versus prognostic scores for prognostication of patients with acute symptomatic pulmonary embolism. / Evaluación clínica frente a escalas estandarizadas para el pronóstico de los pacientes con tromboembolia pulmonar aguda sintomática.

BACKGROUND AND OBJECTIVE: To determine the accuracy of clinical gestalt to identify patients with acute symptomatic pulmonary embolism (PE) at low-risk for short-term complications. PATIENTS AND METHODS: This study included a total of 154 consecutive patients diagnosed with acute symptomatic PE in a tertiary university hospital. We compared the prognostic accuracy of the Pulmonary Embolism Severity Index (PESI), the simplified PESI (sPESI), and clinical gestalt of 1) 2senior physicians (one with and one without experience in the management of patients with PE), 2) a fourth-year resident of Pneumology, 3) a third-year resident of Pneumology, and 4) a second-year resident of Pneumology. The primary outcome was all-cause mortality during the first month after the diagnosis of PE. RESULTS: Thirty-day all-cause mortality was 8.4% (13/154; 8.4%; 95% confidence interval [CI], 4.1-12.8%). The PESI and clinical gestalt classified more patients as low-risk, compared to the sPESI (36.4%, 31.3% y 28.6%, respectively). There were no deaths in the sPESI low-risk category (negative predictive value 100%). Prognostic accuracy increased with increasing experience (84.6 vs. 92.3%; P=.049). CONCLUSIONS: The sPESI showed the best accuracy at correctly identifying low-risk patients with acute symptomatic PE. Clinical gestalt is not inferior to standardized clinical prediction rules to prognosticate patients with acute PE.

Lung and heart-lung transplantation in pulmonary arterial hypertension.

BACKGROUND: Real use of lung (LT) and heart-lung (HLT) transplantation in pulmonary arterial hypertension (PAH) is unknown. The objectives were to describe the indication of these procedures on PAH treatment in a national cohort of PAH patients, and to analyze the potential improvement of its indication in severe patients. METHODS: Eligibility for LT/HLT was assessed for each deceased patient. Incident patients from REHAP diagnosed between January 2007 and March 2015 and considered eligible for LT/HLT were grouped as follows: those who finally underwent transplantation (LTP) and those who died (D-Non-LT). FINDINGS: Of 1391 patients included in REHAP, 36 (3%) were LTP and 375 (27%) died. Among those who died, 36 (3%) were D-Non-LT. LTP and D-Non-LT were equal in terms of age, gender, and clinical status. Ten percent of those who died were functional class I-II. Patients functional class IV were less likely to undergo LT (8.3% LTP vs. 30.6% D-Non-LT, p = 0.017). Patients with idiopathic and drug/toxin-associated PAH were more likely to undergo LT (44.4% LTP vs. 16.7% D-Non-LT, p = 0.011). CONCLUSIONS: The present results show that the use of LT/HLT could double for this indication. Relevant mortality in early functional class reflects the difficulties in establishing the risk of death in PAH.

Molecular Analysis of BMPR2, TBX4, and KCNK3 and Genotype-Phenotype Correlations in Spanish Patients and Families With Idiopathic and Hereditary Pulmonary Arterial Hypertension.

INTRODUCTION AND OBJECTIVES: Recent advances in genetics have led to the discovery of new genes associated with pulmonary arterial hypertension, such as TBX4 and KCNK3. The phenotype and prognosis associated with these new genes have been scarcely described and their role in the Spanish population is unknown. The aim of this study was to characterize the genetics of a Spanish cohort of patients with idiopathic and hereditary pulmonary arterial hypertension and to describe the phenotype and prognostic factors associated with BMPR2 and the new genes (KCNK3 and TBX4). METHODS: A total of 165 adult patients were screened for BMPR2, KCNK3, and TBX4 mutations, 143 with idiopathic pulmonary arterial hypertension and 22 with hereditary pulmonary arterial hypertension. Baseline characteristics and survival were compared among the different subgroups and predictors of poor outcomes were analyzed. We also performed family screening. RESULTS: The genetic study identified a possibly associated mutation in 11.10% of the idiopathic cases (n = 16) and in 68.18% of the hereditary cases (n = 15). There were 19 mutations in BMPR2, 4 in TBX4, and 3 in KCNK3. The forms associated with TBX4 showed the highest survival rate (P < .01). Advanced functional class at diagnosis was the only factor associated with poor outcomes in the hereditary forms. In the family screening, 37.5% of relatives tested positive. CONCLUSIONS: The genetics of pulmonary arterial hypertension in the Spanish population may differ from other populations, with a lower proportion of BMPR2 causative mutations. In our cohort, TBX4-related forms of pulmonary arterial hypertension showed a more benign course and late diagnosis was the only predictor of adverse outcomes in the hereditary forms of the disease.

Sex-related effects of sleep deprivation on depressive- and anxiety-like behaviors in mice.

Anxiety and depressive symptoms are generated after paradoxical sleep deprivation (PSD). However, it is not clear whether PSD produces differential effects between females and males. The aim of this study was to assess the effect of PSD on anxiety- and depressive-like behaviors between sexes. Male and female BALB/c mice were divided in three groups: the control group, the 48-h PSD group and the 96-h PSD group. Immediately after PSD protocols, the forced swimming and open field test were applied. Sucrose consumption test was used to evaluate the middle-term effect of PSD. We found that corticosterone serum levels showed significant differences in the 96-h PSD females as compared to 96-h PSD males. In the open-field test, the 48-h and 96-h PSD females spent more time at the periphery of the field, and showed high locomotion as compared to males. In the elevated plus maze, the 48-h PSD females spent more time in closed arms than males, which is compatible with anxiety-like behavior. The forced swim test indicated that the 96-h PSD males spent more time swimming as compared to the 96-h PSD females. Remarkably, the 96-h PSD males had lower sucrose intake than the 96-h PSD females, which suggest that male mice have proclivity to develop a persistent depressive-like behavior late after PSD. In conclusion, male mice showed a significant trend to depressive-like behaviors late after sleep deprivation. Conversely, female have a strong tendency to display anxiety- and depressive-like behaviors immediately after sleep deprivation.

Demarcation laser photocoagulation induced retinal necrosis and rupture resulting in large retinal tear formation.

Retinal tears after laser photocoagulation are a rare complication that occurs after intense laser. It is talked about among retina specialist occurring particularly at the end of a surgical case while applying endophotocoagulation; to the best our knowledge, there are no reports in the literature of a large retinal tear induced after attempted in-office demarcation laser photocoagulation (DLP) that simulated a giant retinal tear. DLP has been employed in the management of selected cases of macula sparring rhegmatogenous retinal detachment (RRD). Even though extension of the retinal detachment through the "laser barrier" is considered a failure of treatment, few complications have been described with the use of this less invasive retinal detachment repair technique. We describe a case of a high myopic woman who initially was treated with demarcation laser photocoagulation for an asymptomatic retinal detachment associated with a single horseshoe tear and a full thickness large retinal tear was created where the laser was placed. Intense laser photocoagulation resulted in abrupt laser induced retinal necrosis and rupture creating this large retinal break. Proper laser technique should reduce the risks associated with this procedure.