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Microvascular Dysfunction Is Associated With Impaired Myocardial Work in Obstructive and Nonobstructive Hypertrophic Cardiomyopathy: A Multimodality Study
No full textBackground Two-dimensional speckle tracking echocardiography has been shown to correlate with microvascular dysfunction, a hallmark of hypertrophic cardiomyopathy (HCM). We hypothesized that there is an association between myocardial work and left ventricular ischemia, with incremental value to global longitudinal strain, in patients with HCM. Methods and Results We performed a prospective assessment of patients with HCM, undergoing 2-dimensional speckle tracking echocardiography and stress perfusion cardiac magnetic resonance. Results were stratified according to obstructive or nonobstructive HCM and the presence of significant replacement fibrosis (late gadolinium enhancement ≥15% of left ventricular mass). Seventy-five patients with HCM (63% men, age 55±15 years) were evaluated, 28% with obstructive HCM (mean gradient 89±60 mm Hg). Perfusion defects were found in 90.7%, involving 22.5±16.9% of left ventricular mass, and 38.7% had late gadolinium enhancement ≥15%. In a multivariable analysis, a lower global work index (r=-0.519, β-estimate -10.822; P=0.001), lower global work efficiency (r=-0.379, β-estimate -0.123; P=0.041), and impaired global constructive work (r=-0.532, β-estimate -13.788; P-15.5%) had a sensitivity of 64% and a specificity of 57%. The association between myocardial work and perfusion defects was significant independently of late gadolinium enhancement ≥15% and obstructive HCM. Conclusions Impaired myocardial work was significantly correlated with the extent of ischemia in cardiac magnetic resonance, independently of the degree of left ventricular hypertrophy or fibrosis, with a higher predictive power than global longitudinal strain.info:eu-repo/semantics/publishedVersio
Case Report: Mutation in NPPA Gene As a Cause of Fibrotic Atrial Myopathy
No full textEarly-onset atrial fibrillation (AF) can be the manifestation of a genetic atrial myopathy. However, specific genetic identification of a mutation causing atrial fibrosis is rare. We report a case of a young patient with an asymptomatic AF, diagnosed during a routine examination. The cardiac MRI revealed extensive atrial fibrosis and the electrophysiology study showed extensive areas of low voltage. The genetic investigation identified a homozygous pathogenic variant in the NPPA gene in the index case and the presence of the variant in heterozygosity in both parents.info:eu-repo/semantics/publishedVersio
Hemophagocytic Lymphohistiocytosis in an Adolescent with NLRP12‐Related Autoinflammatory Disorder - A Case Report
No full textinfo:eu-repo/semantics/publishedVersio
Perioperative Ultrasound-Guided Continuous Caudal Epidural Analgesia in Newborns: A Case Series in a Tertiary Medical Center
No full textBackground Caudal epidural anesthesia technique is a relevant method for postoperative analgesia in newborns, allowing for the reduction of drug-induced respiratory depression. The threading of a catheter is, however, uncommon in clinical practice. Our main purpose was to describe our experience regarding caudally inserted epidural catheters in neonates undergoing major abdominal surgery. Methods We included every full-term neonate undergoing surgery under combined caudal epidural-general anesthesia from 2017 to 2022 in our institution. After induction of general anesthesia, an ultrasound-guided caudal epidural injection was performed, and an epidural catheter was inserted for perioperative analgesia. An epidural bolus of ropivacaine was administered to every patient before the surgical incision, and an epidural infusion of ropivacaine 0.05% was administered for 24 hours. Results Retrospectively obtained data included six full-term neonates with American Society of Anesthesiologists (ASA) physical status II to IV. Intraoperatively, good analgesia was achieved without hemodynamic instability or need for additional systemic opioids after induction. At the end of surgery, five of the six neonates were extubated without adverse respiratory events. Postoperatively, effective analgesia was achieved in four cases with an epidural infusion of ropivacaine 0.05%, at a rate between 0.2 and 0.4 mg/kg/h, and intravenous paracetamol. Epidural pain control was not successful in one neonate, and thus an intravenous fentanyl infusion was added. The sixth neonate remained intubated for prolonged mechanical ventilation due to surgical complications, and thus an intravenous fentanyl infusion was introduced for sedation in the neonatal intensive care unit (NICU), not allowing to evaluate the effectiveness of the epidural infusion alone. No other complications related to the epidural catheters were reported. Conclusion Continuous caudal epidural analgesia may be a valuable technique with a low risk of complications, decreasing the incidence of respiratory adverse events in this patient population. Although more cases are needed for a stronger conclusion, it has become a useful analgesic strategy for major abdominal surgery in neonates in our institution.info:eu-repo/semantics/publishedVersio
Application of Hazard Functions to Investigate Recurrence After Curative-Intent Resection for Hepatocellular Carcinoma
No full textBackground: Defining patterns and risk of recurrence can help inform surveillance strategies and patient counselling. We sought to characterize peak hazard rates (pHR) and peak time of recurrence among patients who underwent resection of hepatocellular carcinoma (HCC).
Methods: 1434 patients who underwent curative-intent resection of HCC were identified from a multi-institutional database. Hazard, patterns, and peak rates of recurrence were characterized.
Results: The overall hazard of recurrence peaked at 2.4 months (pHR: 0.0384), yet varied markedly. The incidence of recurrence increased with Barcelona Clinic Liver Cancer (BCLC) stage 0 (29%), A (54%), and B (64%). While the hazard function curve for BCLC 0 patients was relatively flat (pHR: 400 ng/mL: 36.3%) and Tumor Burden Score (low: 73.7% vs. medium: 50.6% vs. high: 24.2%) (both p < 0.001).
Conclusion: Recurrence hazard rates for HCC varied substantially relative to both time and intensity/peak rates. TBS and AFP markedly impacted patterns of hazard risk of recurrence.info:eu-repo/semantics/publishedVersio
Why does He Walk Like That? Point Gait and Sensory Profile
No full textA marcha em pontas (MP) define-se pela ausência de contacto do calcanhar com o solo na fase inicial da marcha, podendo ser normal durante a aprendizagem motora, associar-se a diferentes patologias ou ser idiopática (MPI). A abordagem terapêutica é variada, passando por um tratamento conservador ou interventivo. Encontra-se frequentemente a MP em crianças com perturbação do espectro do autismo (PEA), discutindo-se a hipótese de poder estar associada a perturbações do processamento sensorial (PPS).
Este trabalho descreve um caso clínico ilustrativo de MP e PPS numa criança com PEA, com apresentação prévia de uma revisão da literatura sobre o tema. Tem sido descrito por alguns autores que crianças com MP
parecem apresentar alterações sensoriais subtis, nomeadamente procura vestibular, procura propriocetiva e
hipersensibilidade táctil. A criança apresentada, de 4 anos e 9 meses, do sexo masculino, tinha diagnóstico de PEA e apresentava MP. Os resultados do Sensory Profile MeasurePreschool revelaram uma hiperresponsividade táctil, procura sensorial nos domínios propriocetivo e vestibular, associadamente a dificuldades na ideação e planeamento motor (práxis). Parece haver uma relação entre a ocorrência de MP e PPS, contudo, os dados disponíveis são escassos, com diversas imitações. Encontrando-se uma associação consistente, poderá apostar-se com maior segurança no tratamento conservador, nomeadamente na Terapia Ocupacional com abordagem de integração sensorial.info:eu-repo/semantics/publishedVersio
Experiência Cirúrgica dos Internos de Primeiro Ano de Otorrinolaringologia em Portugal
No full textA prática cirúrgica é essencial na formação de novos especialistas em Otorrinolaringologia (ORL).
Com este trabalho caracterizamos a experiência cirúrgica dos internos de ORL que realizaram o seu primeiro ano de internato em 2021. As 18 respostas foram obtidas através do preenchimento de um questionário e representam 82% destes internos. A média anual de procedimentos cirúrgicos realizados foi 58, com um valor mínimo de 4 e máximo de 135. Estes procedimentos incluíram, em média, 16 amigdalectomias, 22 adenoidectomias, 18 colocações de tubos de ventilação transtimpânicos e 5 traqueotomias. O número de procedimentos cirúrgicos realizados apresentou grande variabilidade entre os diferentes internos de ORL. O desenvolvimento futuro de objetivos cirúrgicos nacionais, adaptados a cada ano do internato, poderá ser útil para a uniformização da formação cirúrgica dos internos de ORL em Portugal.info:eu-repo/semantics/publishedVersio
Estetrol/Drospirenona – um Avanço em Relação à CHC com Etinilestradiol?
No full textA contraceção hormonal combinada (CHC) é um dos métodos contracetivos mais utilizados em todo o mundo. As questões relacionadas com a segurança, particularmente o risco tromboembólico (TE), estão em permanente avaliação. A combinação estetrol/ drospirenona (E4/DRSP) é um contracetivo oral combinado (COC) eficaz, com um perfil de hemorragia estável e regular e efeito favorável na pele e no controlo do peso. Tem menor impacto nos fatores pró-trombóticos que as combinações com etinilestradiol (EE), o que configura um perfil de maior segurança, que importa confirmar em estudos de base populacional.info:eu-repo/semantics/publishedVersio
Doseamento Hormonal das Veias Supra-Renais: Como Fazemos
No full textPrimary aldosteronism is the most common cause of secondary hypertension. When unilateral disease is present, patients can be treated curatively by adrenalectomy. Adrenal vein sampling (AVS) is considered essential for discrimination between unilateral versus bilateral disease.
Knowledge of normal and variant anatomy of the adrenal veins is important to avoid misleading results and increase technical success. The main reason for technical failure of AVS is the inability to catheterize the right adrenal vein.
Pre-procedural CT imaging can help identify the venous anatomy of the adrenals. To validate the technical success of AVS, the catheterization index is calculated comparing the cortisol levels in each adrenal gland with those of the inferior vena cava. To assess the laterality index, the aldosterone levels are compared between both adrenals. We generally use a femoral access and a 4Fr Berenstein catheter for the left adrenal vein and a 5Fr Cobra, Simmons or Micahelson for the right adrenal vein. Some centers adopt an intravenous perfusion of a synthetic peptide of the adrenocorticotropic hormone immediately prior to the procedure to stimulate the adrenal glands.
AVS is a safe and feasible procedure, with low risk of failure. Due to the technical difficulties of execution, especially right adrenal vein cannulation, AVS has low usage among hospital centers. The learning curve is estimated to be around 20 to 30 procedures, with a maintenance of about 15 annual procedures to achieve
satisfactory results.info:eu-repo/semantics/publishedVersio