Hospital Beatriz Ângelo

Aims/Purpose: The autonomic nervous system (ANS) is crucial for regulating blood circulation, including ocular circulation, which is vital for vision. Diseases like glaucoma, age‐related macular degeneration, diabetes, and hypertension can disrupt this regulation, impacting retinal blood flow (RBF). Understanding the effects of ANS on RBF could aid in developing targeted and personalized treatments for these conditions. Objective: This study aims to investigate ANS influence on peripapillary vessel density (PPVD) using optical coherence tomography angiography (OCTA) in healthy individuals. Methods: OCTA scans of the right eye were performed in 12 healthy individuals at 3 different time points: at the beginning of the study (B), after parasympathetic stimulation‐deep breathing (PSY) and after sympathetic stimulation‐handgrip (SY). Vessel density was measured within a specific circular crown analysis was conducted within a circular crown with an (inner/outer) radius of (0.6/1.19) mm area, using the Erlangen Angio Tool (EA Tool). ANS function was assessed through beat to beat recordings of blood pressure (BP) and ECG signals (Task Force Monitor) immediately before and after each ANS provocation using the Hilbert‐Huang transform as stated in Laranjo et al 2017. Heart rate (HR), cardiac output (CO) and vascular resistance (TPR) were also computed. Statistical analysis was performed using R software and a p < 0.05 was significant. Results: Changes in the optic nerve head (ONH) circuit were observed during (PSY) increasing 14.09% and (SY) increasing 11.20 % in both ANS provocative manoeuvres ( p < 0.05). Mean BP 79.15 (B) mmHg(69.09/83.43), HR (B) 73.9 bp(74.51/76.91), TPR (B) 2448.42 dyne.s.m ² .m ⁻⁵ (1947.0/2393.75) and CO(B) 2,71 l.mn ⁻¹ .m ⁻² (2.98/2.85) changed with ANS stimuli (PSY/SY), respectively, in line with the changes in PPVD. A strong linear correlation was observed between the ONH perfusion index and the reciprocal of mean TPR index under both provocative conditions TPR ⁻¹ versus PPVD 0.5466/0.4771 (PSY/SY) respectively. Conclusions: ANS input significantly affects ONH blood flow and can be measured with OCTA, reflecting adaptive responses. Understanding ANS effects on PPVD is crucial for developing precise treatments for diseases affecting ANS and ocular conditions.

Background Up to 30% of patients with Crohn's disease (CD) will experience a mild disease course. However, there is no consensus definition for mild CD. Aim To examine the Simple Endoscopic Score for Crohn's disease (SES‐CD) thresholds best associated with low likelihood of long‐term disease progression. Methods We conducted a multicentre retrospective cohort study at three tertiary care centres in United States and Europe. We analysed data from 177 surgery‐naïve patients with CD who had endoscopic assessment while not on immunosuppressive therapy. The primary outcome was disease progression (systemic steroids, biologic or immunomodulator therapy initiation; new stricturing or penetrating complications; or CD‐related hospitalisation or surgery). Univariable and multivariable Cox proportional hazards modelling identified predictors of the primary outcome at 2 and 5 years following endoscopy. Results Disease progression occurred in 23% and 35% of patients at years two and five, respectively. Endoscopic severity at enrolment independently predicted disease progression. Compared to those with an SES‐CD of 0, an SES‐CD ≥ 7 had a greater risk of progression at 2 years (HR 2.50, 95% CI 1.09–5.72) and 5 years (HR 2.89, 95% CI 1.41–5.91). SES‐CD > 7 remained independently predictive of disease progression among the 129 immunosuppression‐naïve patients (HR 5.65, 95% CI 1.49–21.52) and after excluding patients with prior penetrating disease (HR 2.32, 95% CI 1.00–5.45). Conclusions SES‐CD ≥ 7 predicts disease progression in mild CD. A score ≤ 6 may help identify patients less likely to progress and be part of the definition of mild CD.

Purpose This study aims to illustrate the assessment and treatment strategies of acute and chronic deltoid ligament injuries, providing a treatment algorithm for each scenario. Methods A retrospective review was conducted on 39 patients, who had either an acute deltoid ligament injury or suffered chronic deltoid ligament insufficiency. All patients were operated on between January 2016 and December 2022. The cases represent a range of different clinical scenarios, including acute trauma, chronic insufficiency or instability situations. Clinical and imaging evaluation, surgical techniques and clinical outcomes are presented. Results The mean follow‐up time was 21.3 months (±4.5). Acute trauma represented 18 (46%) of the ankles, while 21 (54%) had chronic instability. Treatment options were tailored according to the aetiology, patient characteristics, injury pattern and associated lesions. In 11 of the 18 acute deltoid ruptures (64%), repair was performed using suture anchors, while the remaining 7 cases were treated with direct suture. In chronic deltoid insufficiency, re‐tensioning with suture anchor was performed in 14 (67%) ankles, suture imbrication in 5 (24%) and reconstruction in 6 (9%). The most common associated injuries were syndesmotic injuries ( n = 12, 56%) and osteochondral lesions ( n = 13, 36%). The complication rate was 18% ( n = 7), the majority related to persistent stiffness ( n = 4, 10%). Conclusion The decision on when and how to repair the deltoid ligament should be guided by the characteristics of the injury and the individual patient. The current treatment rationale may serve as a working basis for evaluating and treating these ankles. Level of Evidence Level IV.

Managing sedation in pediatric patients with complex facial anomalies and airway challenges requires careful consideration of safety and efficacy. This case report presents the use of the Ketodex sedation protocol, combining ketamine (NMDA receptor antagonist) and dexmedetomidine (alpha-2-agonist), for a child with a large cervical/facial mass undergoing a diagnostic magnetic resonance imaging (MRI). Ketodex provides effective sedation with minimal need for manipulation of the airway and side effects, making it ideal for cases involving difficult airways. Our protocol allows spontaneous ventilation without compromising airway reflexes and reduces airway hyperreactivity, ensuring safety in high-risk pediatric cases. Initiating sedation in a controlled environment enhances safety before moving to remote locations. The Ketodex protocol is a safe and effective choice for managing pediatric patients with challenging airways. Its use should be prioritized in controlled settings, with adaptations for individual patient needs.

Cushing’s syndrome is a rare disease caused due to prolonged exposure to excess glucocorticoids. Although rare, diagnosing Cushing’s syndrome is clinically significant as it allows tailored and timely management and significant reduction or even prevention of the comorbidities caused by cortisol excess. This report delineates the presentation of a 44-year-old female with refractory secondary hypertension and severe hypokalaemia, initially thought to be caused by hyperaldosteronism. Upon a more careful physical examination, the presence of moon facies, acanthosis nigricans and violaceous abdominal striae raised suspicion of hypercortisolism. Laboratory tests confirmed the suspicion with increased cortisol and adrenocorticotropic hormone (ACTH) levels. Furthermore, imaging findings led to the final diagnosis of Cushing’s disease due to an ACTH-secreting pituitary macroadenoma. The patient underwent successful transsphenoidal surgery, resulting in substantial clinical improvement, evidenced by significant weight loss and hypertension with decreased need for drugs. When left untreated, patients with Cushing’s disease have a higher mortality rate than the general population. This case underscores the critical importance of keeping in mind secondary endocrine causes in the context of resistant hypertension especially with complex metabolic disturbances and recognizing the most characteristic features of this disease.

This case involves a 21-year-old male healthcare student with a medical history of HIV-1 infection for two years and anxiety disorder. He presented to the emergency department with hemoptysis and dyspnea of sudden onset. A thoracic CT scan revealed multiple bilateral nodular ground-glass opacities suggestive of diffuse alveolar hemorrhage (DAH). Due to hypoxemic respiratory failure, noninvasive ventilatory support was initiated. A bronchoalveolar lavage confirmed the presence of blood. He also showed an elevated troponin I, peaking at 2,915 ng/mL, with no electrocardiographic or echocardiographic abnormalities. Assuming an immune etiology, high-dose corticosteroids were initiated, with three days of methylprednisolone 1 g. However, the entire etiological study for DAH turned out negative. During a follow-up clinical interview, the patient admitted to having self-administered 1 mg of intravenous adrenaline shortly before the onset of symptoms. The patient showed a favorable evolution, with no recurrence of symptoms, allowing for oxygen therapy weaning. A follow-up chest CT on the ninth day of hospitalization showed no significant alterations. This case highlights the importance of a thorough history in determining the etiology of a potentially fatal disease, adding intravenous epinephrine as a potential trigger of hemoptysis due to exogenous agents.

Hyperkalemia is a potentially life-threatening condition frequently encountered in clinical practice, particularly among patients with chronic kidney disease, heart failure, diabetes, and hypertension and those undergoing treatment with renin–angiotensin–aldosterone system inhibitors (RAASi). The management of chronic and acute hyperkalemia is complex and requires timely intervention to prevent severe complications such as cardiac arrhythmias and sudden death. Traditional therapeutic approaches to chronic hyperkalemia, including dietary potassium restriction, use of diuretics, and administration of cation-exchange resins like sodium polystyrene sulfonate, often suffer from limitations like gastrointestinal side effects, variable efficacy, delayed onset of action, and RAASi treatment discontinuation. In recent years, the development of new potassium binders, specifically patiromer and sodium zirconium cyclosilicate (SZC), has revolutionized the management of hyperkalemia. Patiromer, a non-absorbed polymer, binds potassium in the gastrointestinal tract in exchange for calcium, thus facilitating its excretion. SZC operates by exchanging sodium and hydrogen ions for potassium, leading to efficient potassium removal. Both agents have demonstrated rapid and sustained reductions in serum potassium levels, coupled with favorable safety and tolerability profiles, in multiple clinical trials. This review article, authored by a multidisciplinary group of Portuguese experts in hyperkalemia management, provides an in-depth analysis of the efficacy and safety of current therapeutic strategies and highlights the clinical potential of new potassium binders. The introduction of patiromer and SZC offers significant advantages over traditional therapies, providing effective and better-tolerated options for patients. The review highlights the role of these novel agents in contemporary hyperkalemia management and calls for ongoing research to further refine treatment protocols and improve patient outcomes.

Purpose This study aimed to assess whether higher values of GTA after osteosynthesis of isolated greater tuberosity fractures were associated with worse functional and symptomatic outcomes. Methods A retrospective analysis was conducted from 2012 to 2024, including all patients with isolated greater tuberosity fractures undergoing osteosynthesis at a single institution. GTA measurements before and after osteosynthesis were recorded, along with the latest QuickDash score and constant shoulder score. Results Thirty-four patients were recruited, with 25 undergoing osteosynthesis with cannulated screws and eight with anchors. Twenty-two patients self-administered the QuickDash score and completed the constant shoulder score. The average GTA before surgery was 84.2°, reducing to 62.2° post-surgery. The average constant score was 86.8, and the mean QuickDash score was 7.65. The method of osteosynthesis did not significantly influence GTA or the QuickDash and constant scores. Additionally, post-surgery GTA negatively correlated with the constant score (r = − 0.65, p < 0.01). Conclusions Patients with isolated greater tuberosity fractures undergoing osteosynthesis experience a change in GTA after surgery. However, they achieved satisfactory symptomatic and functional scores post-surgery. The method of osteosynthesis did not significantly influence the GTA or the QuickDash and constant scores. Furthermore, post-surgery GTA negatively correlated with the constant score.

Artificial intelligence (AI) may revolutionize foot and ankle (FA) surgery, promising improved patient outcomes and reduced errors in healthcare. By employing sophisticated models and algorithms, AI replicates human-like intelligence, enabling machines to problem-solve, generalize, and draw inferences from complex situations. In the realm of FA surgery, AI can automate radiographic image interpretation, predict surgical outcomes and injury risks, and enhance surgical precision. These advancements not only minimize errors in patient care but also lead to more precise diagnoses. AI’s role extends to lightening the surgeon’s workload by handling redundant tasks, thereby allowing more time for crucial patient interactions, surgical planning, and training. However, the integration of AI in FA surgery is in its early stages, demanding consideration of ethical and cost-related concerns. Rigorous external validation of AI models is essential before their widespread adoption. Moreover, optimizing AI systems regarding weight, size, and appearance while minimizing user energy consumption is crucial. In tandem with AI, robotic technology emerges as a key player in FA surgery, enriching preclinical research, clinical evaluation, preoperative planning, and computer-assisted surgery. Dynamic robotic simulators ensure precise specimen positioning, enhancing our understanding of FA biomechanics. Robotics aid in detailed descriptions of joint motion, bone structures, and ligamentous characteristics, offering advantages over traditional models. Rehabilitation applications of robotics encompass exoskeletons, prosthetics, and robotic rehabilitation instruments. Further research is imperative to validate AI models in clinical settings and explore the full potential of robotics in FA surgery. Ethical and financial considerations notwithstanding, the transformative impact of these technologies on FA surgery cannot be overstated.

In recent years, reports of Enterococcus hirae infections in humans have increased. Similarly to most known Enterococci, E. hirae has been identified mostly in bacteremia, urinary tract infections, infective endocarditis, and biliary tract infections. We present a case of E. hirae bacteriemia associated with traumatic soft tissue infection in a 77-year-old male patient, a polytrauma victim with a tibia-fibula open fracture after a forklift accident. Initially, the patient underwent a left below-the-knee amputation but it evolved poorly, with necrosis of the surgical stump. Debridement and antibiotics were started. Blood and soft tissue cultures identified E. hirae. An above-the-knee amputation was necessary, and the patient improved satisfactorily. Our case study helps to confirm the unexpected E. hirae in humans as well as report an unusual source of infection of this pathogen. Further studies and more case reports are needed to elucidate the clinical impact of Enterococcus hirae on humans.

Background Etrasimod is an oral, once-daily (QD), selective sphingosine 1-phosphate (S1P)1,4,5 receptor modulator for the treatment of moderately to severely active ulcerative colitis (UC). It is known that non-serious treatment-emergent adverse events (TEAEs) may not lead to UC drug discontinuation but can affect treatment tolerability. Objectives This post hoc analysis evaluated the incidence of specific, common, non-serious TEAEs reported in the etrasimod UC clinical programme and the characteristics of affected patients. Design Data included patients from the Placebo-controlled UC cohort (phase II OASIS, and phase III ELEVATE UC 52 and ELEVATE UC 12 trials) receiving QD etrasimod (2 or 1 mg) or placebo. Methods Proportions and incidence rates (IRs; the number of patients with a TEAE divided by the total exposure in patient-years (PYs), per 100 PY) of Headache, Pyrexia, Nausea and Dizziness TEAEs were reported. Changes in heart rate among patients with Dizziness TEAEs were also evaluated. Results Among 943 patients (etrasimod 2 mg, N = 577 (276.7 PY); etrasimod 1 mg, N = 52 (11.4 PY); placebo, N = 314 (115.1 PY)), 48, 34, 27 and 21 patients experienced events of Headache, Pyrexia, Nausea and Dizziness, respectively. All events were non-serious; one patient treated with etrasimod was discontinued due to a Pyrexia TEAE. Numerically, IRs of Headache and Dizziness TEAEs were higher, and Nausea slightly higher, with etrasimod versus placebo (13.45 vs 8.63 per 100 PY, 6.52 vs 1.69 and 7.18 vs 5.13 per 100 PY, respectively); IRs were similar for Pyrexia. The duration of most TEAEs was 1–10 days. Conclusion In the etrasimod UC clinical programme, all Headache, Pyrexia, Nausea and Dizziness events were non-serious. Headache and Dizziness were more frequent, and Nausea slightly more frequent, among patients receiving etrasimod versus placebo. The post hoc nature of this analysis is a limitation. These results reiterate the favourable safety profile and tolerability of etrasimod. Trial registration ClinicalTrials.gov: NCT02447302; NCT03945188; NCT03996369.

Introduction Candida auris is an emerging pathogen that represents a worldwide health problem due to its global expansion, multidrug resistance, and difficult laboratory identification. Among the risk factors for colonization/infection by C. auris, a stay in an intensive care unit (ICU) stands out. This prospective multicenter study aimed to monitor the trend of the local epidemiology of Candida spp. and unveil the prevalence of C. auris. Methods From 2020 to 2022, axillar/inguinal swabs were collected from adult patients at three points: upon admission (D1) and on the fifth (D5) and eighth (D8) days of their ICU stay. We employed culture-based screening methods combined with molecular techniques to identify Candida spp. down to the species level. Specific screening for Candida auris was conducted using a real-time PCR assay in combination with an improved selective culture medium, mannitol salt agar auris (MSAA). To validate the effectiveness of MSAA, a collection of reference C. auris strains representing the four major geographical clades was used. Results We enrolled 675 patients, and 355 Candida isolates were retrieved from the 988 swab samples collected. From those, 185/355 (52.1%) were identified as C. albicans and 170/355 (47.9%) as non-albicans Candida (NAC). MSAA medium showed a specificity of 94.8%, albeit C. auris was not detected in this cohort. The dynamics of Candida spp. colonization by ICU were significant at the three collection points. Upon admission, C. albicans was associated with the Beatriz Ângelo Hospital ICU (p=0.003) and C. tropicalis with the general Hospital Professor Doutor Fernando Fonseca (FFH) ICU (p=0.006). C. parapsilosis and C. lusitaniae were associated with FFH ICUs, with the general ICU at D5 (p=0.047) and surgical ICU at D8 (p=0.012). The dynamics of NAC colonization by ICU were significantly different at D1 (p=0.011), D5 (p=0.047), and D8 (p=0.012). Conclusion We developed and implemented a screening protocol for C. auris while uncovering the colonization patterns of Candida in the ICU. Our findings contribute to the optimization of overall patient management, ensuring that ICU protocols are resilient and adaptive to emerging fungal threats.

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the presence of antiphospholipid antibodies and venous and arterial thrombotic events, including obstetric complications. We describe the case of a 56-year-old female diagnosed with APS with triple antibody positivity and multiple disease-associated manifestations, namely recurrent purpuric lesions, adrenal insufficiency due to infarction, acalculous cholecystitis, and three spontaneous abortions. Her follow-up was marked by severe thrombotic and haemorrhagic events, notably splanchnic vein thrombosis and haemorrhagic shock after a renal biopsy, as well as the diagnosis of systemic lupus erythematosus 8 years after the APS diagnosis. Chronic anticoagulation with enoxaparin, with dosage guided by anti-factor Xa activity, resulted in stability without complications over 17 years. This case emphasizes the importance of personalized therapeutic strategies and close monitoring in patients with APS. LEARNING POINTS This patient presented with rare manifestations of antiphospholipid syndrome (APS), such as acalculous cholecystitis, Budd-Chiari syndrome, and Addison’s disease, all linked to ischemic events caused by vascular thrombosis. These atypical presentations highlight the varied and severe clinical spectrum of APS, requiring physicians to be vigilant about unusual complications. After multiple severe thrombotic and haemorrhagic events, the patient was successfully managed with long-term enoxaparin, with dose adjusted according to anti-factor Xa activity (which allowed for a significant dose reduction in comparison with the predicted empiric dose). The patient remained event-free for 17 years, reflecting the importance of personalizing anticoagulation therapy.

Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and radiological syndrome that presents as rapid onset of neurological symptoms such as headache, visual loss, impaired mental status, and seizure activity associated with characteristic focal white matter vasogenic edema. When promptly recognized and managed, these changes are usually reversible. PRES is most commonly associated with hypertensive crises, renal insufficiency, and the use of immunosuppressive therapies, though it may arise in various clinical contexts. Despite its significance, reports of PRES in the field of anesthesiology remain limited. This case report presents the case of a 46-year-old male admitted for elective ambulatory ophthalmic surgery under general anesthesia who developed delayed emergence from anesthesia and post-operative blindness, both attributed to the intraoperative onset of PRES. Anesthesiologists should be vigilant for PRES as a potential complication during the perioperative period, and consider it in the differential diagnosis for delayed emergence from anesthesia. Clinical suspicion should warrant prompt imagiological confirmation by magnetic resonance imaging (MRI), as delayed recognition and management can result in severe and long-term neurological disability.

Background/Objectives: Pancreatic ductal adenocarcinoma (PDAC) incidence is rising, and prognosis remains poor due to late diagnosis and limited effective therapies. Currently, patients are treated based on TNM staging, without molecular tumor characterization. This study aimed to validate a technique that combines the amplification refractory mutation system (ARMS) with high-resolution melting analysis (HRMA) for detecting mutations in codon 12 of KRAS in tumor and plasma, and to assess its prognostic value. Methods: Prospective study including patients with newly diagnosed PDAC with tumor and plasma samples collected before treatment. Mutations in codon 12 of KRAS (G12D, G12V, G12C, and G12R) were detected using ARMS–HRMA and compared to Sanger sequencing (SS). Univariate and multivariate analyses were used to evaluate the prognostic significance of these mutations. Results: A total of 88 patients, 93% with ECOG-PS 0–1, 57% with resectable disease. ARMS–HRMA technique showed a higher sensitivity than SS, both in tumor and plasma (77% vs. 51%; 25 vs. 0%, respectively). The most frequent mutation was G12D (n = 32, 36%), followed by G12V (n = 22, 25%). On multivariate analysis, patients with G12D and/or G12C mutations, either in tumor or plasma, had lower PFS (HR 1.792, 95% CI 1.061–3.028, p = 0.029; HR 2.081, 95% CI 1.014–4.272, p = 0.046, respectively) and lower OS (HR 1.757, 95% CI 1.013–3.049, p = 0.045; HR 2.229, 95% CI 1.082–4.594, p = 0.030, respectively). Conclusions: ARMS–HRMA is a rapid and cost-effective method for detecting KRAS mutations in PDAC patients, offering the potential for stratifying prognosis and guiding treatment decisions. The presence of G12D and G12C mutations in both tumor and plasma is associated with a poorer prognosis.