Clinical Characteristics and Outcomes in Adults With Moderate-to-Severe Complexity Congenital Heart Disease Undergoing Palliation or Surgical Repair.

Background: Congenital heart disease (CHD) survival rate has improved dramatically due to advances in diagnostic and therapeutic techniques. However, concerning the unrepaired CHD population of moderate and severe complexity, the data regarding risk predictors and surgical outcomes are scarce. Our aim was to describe the surgical results and predictors of in-hospital outcomes in adult patients with moderate-to-severe complexity CHD that were not repaired in childhood. Methods: We conducted a retrospective cohort study that included 49 adult patients with moderate-to-complex CHD who were treated in a single medical centre. Clinical and echocardiographic variables were obtained on admission, after surgical procedures and during follow-up. Results: Most of the patients were female (66%). Left ventricular ejection fraction and right ventricular outflow tract fractional shortening were within the normal range. The median pulmonary artery systolic pressure was 37 (27-55) mm Hg. The median time was 118 (80-181) minutes for extracorporeal circulation and 76 (49-121) minutes for aortic cross-clamping. The most frequent complication was postoperative complete atrioventricular block (12.2%). In-hospital survival rate was 87.7%. The development of low cardiac output syndrome with predominant right ventricle failure in the postoperative period was the most important predictor of in-hospital death (P = 0.03). Conclusions: Deciding to treat adults with CHD is challenging in moderate and severe unrepaired cases. Adequate clinical, functional, and imaging evaluation is essential to determine each patient's suitability for surgical management and to achieve the best clinical outcome for this population.

Contexte: Grâce aux avancées réalisées en matière de techniques diagnostiques et thérapeutiques, la survie des patients atteints d'une cardiopathie congénitale s'est considérablement améliorée. Cependant, en ce qui concerne les personnes atteintes d'une cardiopathie congénitale non corrigée présentant une complexité modérée ou extrême, les données portant sur les facteurs de risque prédictifs ainsi que sur les résultats chirurgicaux sont rares. Notre objectif était de décrire les résultats chirurgicaux ainsi que les facteurs prédictifs des résultats obtenus en milieu hospitalier chez les patients adultes atteints d'une cardiopathie congénitale présentant une complexité modérée ou extrême qui n'a pas été corrigée pendant l'enfance. Méthodologie: Nous avons mené une étude de cohorte rétrospective comprenant 49 patients adultes atteints d'une cardiopathie congénitale modérée ou complexe qui ont reçu leurs traitements dans un seul centre médical. Les variables cliniques et échocardiographiques ont été obtenues au moment de l'admission, après les interventions chirurgicales et pendant la période de suivi. Résultats: Les patients étaient en majorité des femmes (66 %). La fraction d'éjection du ventricule gauche ainsi que la fraction de raccourcissement de la voie d'éjection ventriculaire droite sont demeurées dans les limites de la normale. La pression systolique médiane de l'artère pulmonaire a été de 37 mmHg (27-55 mmHg). Le temps médian écoulé pour la circulation extracorporelle a été de 118 minutes (80-181 minutes) et pour le clampage de la crosse aortique, de 76 minutes (49-121 minutes). Le bloc auriculo-ventriculaire postopératoire complet a été la complication la plus fréquente (12,2 %). Le taux de survie en milieu hospitalier a été de 87,7 %. Le développement du syndrome du faible débit cardiaque accompagné d'une insuffisance prédominante du ventricule droit durant la période postopératoire a constitué le principal facteur prédictif de décès à l'hôpital (p = 0,03). Conclusion: Il est difficile de traiter les adultes qui présentent une cardiopathie congénitale modérée ou sévère non corrigée. Il est essentiel que les évaluations cliniques, fonctionnelles et par imagerie soient réalisées de façon adéquate pour déterminer si une prise en charge chirurgicale convient aux patients et pour garantir les meilleurs résultats cliniques chez ces derniers.

Hypertrophic cardiomyopathy in the systemic right ventricle in a patient with congenitally corrected transposition of the great arteries: A case report.

Congenitally corrected transposition of the great arteries is a rare clinical entity, which usually presents during adulthood with associated defects; atrioventricular block, heart failure, systemic valve failure, and arrhythmias usually complicate the clinical course. Even rarer is associated hypertrophic cardiomyopathy, which complicates the disease course and clinical decision-making. Herein, we present a patient with this condition who underwent heart transplantation, with adequate clinical resolution.

Cardiometabolic Risk Factors in Mexican Adults With Congenital Heart Disease.

Background: In recent decades, adults living with congenital heart disease (ACHD) have improved their survival, thus increasing their predisposition to the onset of cardiometabolic risk factors and chronic health conditions. Objectives: The purpose of this study was to describe cardiometabolic risk profiles in the ACHD population and their relationship to congenital heart disease (CHD) lesion complexity. Methods: We performed a cross-sectional study from ACHD in a third-tier referral center in Mexico City. The association between cardiometabolic risk factors and CHD complexity was estimated using logistic regression models. Results: Our study cohort included 1,171 ACHD patients (median age: 31 [IQR: 23.2-42.7] years, male 63.6%). Cardiac diagnosis was classified as mild (44.9%), moderate (37.8%), and severe (17.2%) CHD complexity. Low high-density lipoprotein cholesterol (55%) was the most common cardiometabolic risk factor; followed by insulin resistance (54.5%) and prediabetes (52.4%). Patients with mild and moderate CHD had a higher prevalence of obesity and metabolic syndrome, while patients with severe CHD had a higher prevalence of hyperuricemia and subclinical hypothyroidism. In the logistic regression analysis, the severity of CHD was associated with higher odds of hyperuricemia (moderate CHD, OR: 1.87; 95% CI: 1.20-2.93; P = 0.010; severe CHD, OR: 2.75; 95% CI: 1.64-4.62; P < 0.001) and lower risks of metabolic syndrome (OR: 0.61; 95% CI: 0.41-0.91; P = 0.010), prediabetes (OR: 0.58; 95% CI: 0.42-0.81; P < 0.001), and arterial hypertension (OR: 0.49; 95% CI: 0.33-0.74; P < 0.001) compared with mild CHD complexity. Conclusions: We observed high rates of cardiometabolic risk factors in Mexican ACHD patients and these risk profiles varied by CHD lesion complexity. These results highlight the need for ongoing metabolic health surveillance in the ACHD population.

Stent en una fístula sistémico-pulmonar de Blalock-Taussig modificada en un adulto paliado con atresia tricúspide con estenosis infundibular pulmonar crítica / Stenting of a modified Blalock-Taussig shunt in an adult with palliated tricuspid atresia and critical pulmonary stenosis

Resumen: Los adultos con cardiopatía congénita compleja, con fisiología univentricular y flujo pulmonar disminuido, constituyen un reto terapéutico. Muchos de ellos reciben tratamiento paliativo con fístula sistémico-pulmonar desde la etapa pediátrica. Dicha fístula puede presentar oclusión o estenosis y ocasionar deterioro de la capacidad funcional y clínica. Colocar una nueva fístula sistémico-pulmonar a través de una cirugía se considera de alto riesgo, por lo que el uso de stents a través de cateterismo cardíaco surge como una opción que ha tenido buenos resultados. Se describe el caso de un adulto con atresia tricúspide con fístula sistémico-pulmonar en la etapa pediátrica, quien acudió al servicio de urgencias por deterioro de su clase funcional y desaturación en aire ambiente de hasta un 64%; en la tomografía cardíaca se evidenció estenosis del tercio distal de la fístula sistémico-pulmonar, por lo que se decidió colocar, mediante cateterismo cardíaco intervencionista, dos stents (Express LD vascular 6 x 37 mm y 6 x 27 mm), luego de lo cual la saturación sistémica fue del 75%, por lo que se consideró exitosa la colocación de los dispositivos. La oclusión de estas fístulas es una complicación habitual, que genera disminución de la perfusión pulmonar con los subsecuentes síntomas respiratorios, disminución de la saturación y la oxigenación, cianosis y acidosis metabólica, y puede generar un evento que amenaza la vida si se presenta de manera aguda. La recanalización transcatéter de una fístula sistémico-pulmonar con stent es una alternativa adecuada para evitar un riesgo quirúrgico y arroja resultados óptimos.

Abstract: Adults with complex congenital heart disease with univentricular physiology and decreased in the pulmonary flow constitute a therapeutic challenge, many of these patients are palliated with modified Blalock-Taussig shunt (mBTS). The mBTS can develop occlusion or stenosis over time with deterioration of functional class and poor exercise tolerance. Dysfunction of a mBTS is a life-threatening situation requiring urgent therapy. A new surgical palliation is a high-risk procedure, so stenting a mBTS can be an alternative. We report a 29-year-old female with tricuspid atresia and pulmonary infundibular stenosis palliated with mBTS with progressive cyanosis (oxygen saturation of 54%) and dyspnea; computed tomography revealed a stenosed mBTS, and an interventional percutaneous approach was made. The stenting of the mBTS was made with two stents (Express LD vascular 6 x 37 mm and 6 x 27 mm). Oxygen saturation post-procedure increase to 70-75%. Occlusion of these shunts are a common major complication, leading to a decrease in pulmonary perfusion with subsequent respiratory symptoms, low saturation and oxygenation, cyanosis, metabolic acidosis and can generate a life-threatening event if it occurs acutely. Stent implantation into a previous mBTS through cardiac catheterization can be an alternative to shunt operation in patients with cyanotic congenital heart disease having a good long-term results.

Intergenerational Trauma: Assessment in Biological Mothers and Preschool Children.

Childhood trauma can lead to lifelong detrimental outcomes. Intergenerational trauma should be considered when supporting healthy parent-child relationships. Research is needed on intergenerational trauma in relation to children's negative life event exposure, which could compound intergenerational trauma. We examined the prevalence of and relations between mother and child traumas in a sample of 88 biological mothers and their preschool-aged children. We coded child negative life events to examine those related to intergenerational trauma. Results showed that mother traumas and child negative life events were positively associated; subtypes of mothers' traumas (abuse, neglect) and high trauma levels were associated with higher numbers of child negative life events, including those tied to parent trauma. It is necessary to consider how childhood trauma in adults and children is measured, and what analyses can reveal about the intergenerational context, especially considering compounding current, stressful world events.

Comorbidities and laboratory parameters associated with SARS-CoV-2 infection severity in patients from the southeast of Mexico: a cross-sectional study.

Background. Severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) is the etiological agent of the coronavirus disease 2019 (COVID-19) pandemic. Among the risk factors associated with the severity of this disease is the presence of several metabolic disorders. For this reason, the aim of this research was to identify the comorbidities and laboratory parameters among COVID-19 patients admitted to the intensive care unit (ICU), comparing the patients who required invasive mechanical ventilation (IMV) with those who did not require IMV, in order to determine the clinical characteristics associated with the COVID-19 severity. Methods. We carried out a cross-sectional study among 152 patients who were admitted to the ICU from April 1 st to July 31 st, 2021, in whom the comorbidities and laboratory parameters associated with the SARS-CoV-2 infection severity were identified. The data of these patients was grouped into two main groups: "patients who required IMV" and "patients who did not require IMV". The nonparametric Mann-Whitney U test for continuous data and the χ2 test for categorical data were used to compare the variables between both groups. Results. Of the 152 COVID-19 patients who were admitted to the ICU, 66 required IMV and 86 did not require IMV. Regarding the comorbidities found in these patients, a higher prevalence of type 2 diabetes mellitus (T2DM), hypertension and obesity was observed among patients who required IMV vs. those who did not require IMV ( p<0.05). Concerning laboratory parameters, only glucose, Interleukin 6 (IL-6), lactate dehydrogenase (LDH) and C-reactive protein (CRP) were significantly higher among patients who required IMV than in those who did not require IMV ( p<0.05). Conclusion. This study performed in a Mexican population indicates that comorbidities such as: T2DM, hypertension and obesity, as well as elevated levels of glucose, IL-6, LDH and CRP are associated with the COVID-19 severity.

Polymorphisms at CYP enzymes, NR1I2 and NR1I3 in association with virologic response to antiretroviral therapy in Brazilian HIV-positive individuals.

Virologic failure of antiretroviral therapy (ART) may be explained by single nucleotide polymorphisms (SNPs) in drug absorption and metabolism genes. Here, we characterized the associations between polymorphisms in cytochrome P450 enzymes' genes CYP2B6 and CYP3A4/A5, nuclear receptor genes NR1I2/3, and initial ART efficacy among 203 HIV-positive individuals from Rio de Janeiro. Association between SNPs and virologic control was evaluated after 6 and 12 months of follow-up using Cox regression models. The SNP rs2307424 (NR1I3) was associated with increased virologic response after 12 months of treatment, while rs1523127 (NR1I2), rs3003596, and rs2502815 (NR1I3) were associated with decreased response. Increased virologic response after 12 months (adjHR = 1.54; p = 0.02) was also observed among carriers of the NR1I3 haplotype rs2502815G-rs3003596A-rs2307424A versus the reference haplotype G-A-G. Our results suggest that NR1I2 and NR1I3 variants are associated with virologic responses to ART among Brazilians.

Ustekinumab Drug Survival in Patients with Psoriasis: A retrospective Study of Real Clinical Practice.

Background and objectives: The efficacy and safety of ustekinumab have been proved in clinical trials. In daily clinical practice, knowing the factors that determine survival differences of biological drugs allows psoriasis treatment to be optimized as a function of patient characteristics. The main objectives of this work are to understand ustekinumab drug survival in patients diagnosed with plaque psoriasis in the Hospital Universitario Central de Asturias (HUCA Dermatology Department, and to identify the predictors of drug discontinuation. Materials and Methods: A retrospective hospital-based study, including data from 148 patients who were receiving ustekinumab (Stelara®) between 1 February 2009 and 30 November 2019, were collected. Survival curves were approximated through the Kaplan-Meier estimator and compared using the log-rank test. Proportional hazard Cox regression models were used for multivariate analyses while both unadjusted and adjusted hazard ratios (HR) were used for summarizing the studied differences. Results: The average duration of the treatment before discontinuation was 47.57 months (SD 32.63 months; median 41 months). The retention rates were 82% (2 years), 66% (5 years), and 58% (8 years). Median survival was 80 months (95% confidence interval. CI 36.9 to 123.01 months). The survival study revealed statistically significant differences between patients with arthritis (log-rank test, p < 0.001) and those who had previously received biological treatment (log-rank test, p = 0.026). The five-year prevalence in patients still under treatment was 80% (those without arthritis) and 54% (arthritis patients). In the multivariate analysis, only the patients with arthritis had a lower rate of drug survival. No statistically significant differences were observed for any of the other comorbidities studied. The first and second most frequent causes of discontinuation were secondary failure and arthritis inefficacy, respectively. Conclusion: Ustekinumab is a biological drug conferring high survival in plaque psoriasis patients. Ustekinumab survival is lower in patients with arthritis.

[Subcutaneous infection by Curvularia pallescens in a lung transplant recipient: First report in Spain]. / Micosis cutánea por Curvularia pallescens en un paciente trasplantado pulmonar: primer caso descrito en España.

BACKGROUND: Curvularia is a filamentous dematiaceous fungus increasingly recognized as a pathogen in immunocompromised patients. The most common clinical entities associated with this fungus are allergic sinusitis, cutaneous infection and keratitis. In this article, a report on the first clinical case of Curvularia pallescens cutaneous infection in Spain and its treatment is described. CASE REPORT: A 68 year-old man with a history of lung transplantation presented to Dermatology Unit due to a skin lesion in the knee that had been evolving for 6 months. A skin biopsy was performed for its study. In the histopathological study, an intense and non-specific inflammatory reaction in the dermis was observed, and with Grocott stain and periodic acid Schiff abundant septate hyphae and spores were found in the dermis. The culture of the sample revealed a filamentous fungus whose microscopic examination allowed to identify the genus as Curvularia. Using MALDI-TOF mass spectrometry and molecular identification, the fungus was finally identified as Curvularia pallescens. The patient underwent surgical resection of the lesion and was treated with posaconazole, evolving favorably. CONCLUSIONS: The species of Curvularia should be considered causal agents of fungal skin infections in immunosuppressed patients. This clinical case, which showed good clinical response after surgical resection and treatment with posaconazole, is the first described in Spain due to this species.

Gene doping and genomic science in sports: where are we?

The misuse of sport-related gene transfer methods in elite athletes is a real and growing concern. The success of gene therapy in the treatment of hereditary diseases has been most evident since targets in gene therapy products can be used in healthy individuals to improve sports performance. Performing these practices threatens the sporting character of competitions and may pose potential health hazards. Since the World Anti-Doping Agency pronouncement on the prohibition of such practices in 2003, several researchers have been trying to address the challenge of developing an effective method for the detection of genetic doping. This review presents an overview of the published methods developed for this purpose, the advantages and limitations of technologies and the putative target genes. At last, we present the perspective related to the application of the detection methods in the doping control field.

Micosis cutánea por Curvularia pallescens en un paciente trasplantado pulmonar: primer caso descrito en España / Subcutaneous infection by Curvularia pallescens in a lung transplant recipient: first report in Spain

ANTECEDENTES: El género Curvularia incluye hongos filamentosos dematiáceos cada vez más reconocidos como patógenos en pacientes inmunocomprometidos. Las entidades clínicas más comunes con las que se asocia este hongo son la sinusitis alérgica, la infección cutánea y la queratitis. En este trabajo se describe el primer caso descrito en España de infección cutánea por Curvularia pallescens y su tratamiento. CASO CLÍNICO: Un varón de 68 años con antecedente de trasplante pulmonar acudió al servicio de dermatología por presentar una lesión cutánea en la rodilla de 6 meses de evolución. Se realizó una biopsia cutánea para estudio. La histopatología mostró una intensa reacción inflamatoria inespecífica en la dermis y mediante la tinción de Grocott y ácido peryódico de Schiff se observaron abundantes hifas septadas y esporas en la dermis. El cultivo de la muestra reveló un hongo filamentoso cuyo examen microscópico permitió identificar el género como Curvularia. Mediante espectrometría de masas MALDI-TOF e identificación molecular, el hongo finalmente se identificó como Curvularia pallescens. Se realizó resección quirúrgica de la lesión y el paciente recibió tratamiento con posaconazol, con resolución clínica de la lesión. CONCLUSIONES: El género Curvularia debe ser considerado un agente causal de micosis subcutáneas en pacientes inmunodeprimidos. Este caso clínico constituye el primero descrito en España producido por esta especie, el cual presentó buena respuesta clínica tras resección quirúrgica y tratamiento con posaconazol

BACKGROUND: Curvularia is a filamentous dematiaceous fungus increasingly recognized as a pathogen in immunocompromised patients. The most common clinical entities associated with this fungus are allergic sinusitis, cutaneous infection and keratitis. In this article, a report on the first clinical case of Curvularia pallescens cutaneous infection in Spain and its treatment is described. CASE REPORT: A 68 year-old man with a history of lung transplantation presented to Dermatology Unit due to a skin lesion in the knee that had been evolving for 6 months. A skin biopsy was performed for its study. In the histopathological study, an intense and non-specific inflammatory reaction in the dermis was observed, and with Grocott stain and periodic acid Schiff abundant septate hyphae and spores were found in the dermis. The culture of the sample revealed a filamentous fungus whose microscopic examination allowed to identify the genus as Curvularia. Using MALDI-TOF mass spectrometry and molecular identification, the fungus was finally identified as Curvularia pallescens. The patient underwent surgical resection of the lesion and was treated with posaconazole, evolving favorably. CONCLUSIONS: The species of Curvularia should be considered causal agents of fungal skin infections in immunosuppressed patients. This clinical case, which showed good clinical response after surgical resection and treatment with posaconazole, is the first described in Spain due to this species

Emotion regulation in early childhood: Implications for socioemotional and academic components of school readiness.

In this article, we conduct a selective review of the literature on emotion regulation in early childhood (e.g., preschool, generally defined as ages 3-5) in regard to school readiness. Emotion regulation (ER), the process of modulating emotional arousal and expression, plays a key role in a child's ability to adapt to novel demands of school environments. This, in turn, has significant implications for both the socioemotional and academic components of a child's readiness to enter kindergarten. We begin our article with a brief review of the ER construct at this developmental stage, then review research on the impact of ER on both components of school readiness during the preschool and early elementary school years. We then discuss strategies for teachers and parents to promote ER strategy use in young children, and end with challenges regarding operationalization at this age and directions for future research. (PsycINFO Database Record (c) 2020 APA, all rights reserved).

RNAi-based bioinsecticide for Aedes mosquito control.

Zika virus infection and dengue and chikungunya fevers are emerging viral diseases that have become public health threats. Their aetiologic agents are transmitted by the bite of genus Aedes mosquitoes. Without effective therapies or vaccines, vector control is the main strategy for preventing the spread of these diseases. Increased insecticide resistance calls for biorational actions focused on control of the target vector population. The chitin required for larval survival structures is a good target for biorational control. Chitin synthases A and B (CHS) are enzymes in the chitin synthesis pathway. Double-stranded RNA (dsRNA)-mediated gene silencing (RNAi) achieves specific knockdown of target proteins. Our goal in this work, a new proposed RNAi-based bioinsecticide, was developed as a potential strategy for mosquito population control. DsRNA molecules that target five different regions in the CHSA and B transcript sequences were produced in vitro and in vivo through expression in E. coli HT115 and tested by direct addition to larval breeding water. Mature and immature larvae treated with dsRNA targeting CHS catalytic sites showed significantly decreased viability associated with a reduction in CHS transcript levels. The few larval and adult survivors displayed an altered morphology and chitin content. In association with diflubenzuron, this bioinsecticide exhibited insecticidal adjuvant properties.

Perioperative anaphylaxis in a patient with a solitary mastocytoma.

Children with more extensive cutaneous mastocytosis have a higher risk for symptoms secondary to release of mast cell mediators. However, the remote possibility of anaphylaxis in patients with a solitary lesion suggests the need for cautious use of general anesthesia in these children. We describe an unusual case of a patient with a solitary mastocytoma who experienced an anaphylactic reaction during a surgical procedure and make recommendations to reduce the risk of intraoperative anaphylaxis in mast cell disease.

Criptococosis cutánea / Cutaneous cryptococcosis

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