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- Proximal Junctional Kyphosis Following Spinal Thoracic Deformity Correction in a Patient with Kabuki Syndrome: A Case Report.Publication . Nóbrega, João; Almeida, Ricardo; Rasteiro, Pedro; Rosado, João; Botelho, Tiago; Carvalho, NelsonIntroduction: Kabuki syndrome (KS) is a rare congenital disorder characterized by distinctive facial features, intellectual disability, and multiple musculoskeletal anomalies, including scoliosis, kyphosis, and generalized ligamentous laxity. The combination of connective tissue fragility and complex spinal deformity may predispose these patients to post-operative complications, such as proximal junctional kyphosis (PJK), though this association has not previously been reported. Case report: We report a 15-year-old male with genetically confirmed KS who presented with severe thoracic hyperkyphosis (95°). Posterior spinal fusion and correction were performed, resulting in initial improvement. Within 8 months, the patient developed PJK above the upper instrumented vertebra, requiring multiple revision procedures. Post-operative infection with Staphylococcus aureus and rapid recurrent kyphosis further complicated management. A staged revision strategy, combining halo-gravitational traction followed by extended fusion and careful sagittal realignment, achieved stable correction and functional improvement at 1-year follow-up. Conclusion: The association between these conditions has, to our knowledge, not yet been reported in literature. This case highlights the multifactorial etiology of PJK in KS, where intrinsic ligamentous laxity, immune dysfunction, and extensive deformity correction converge to increase mechanical vulnerability. Soft-tissue preservation at the upper instrumented level, careful sagittal contouring, and infection control are key preventive strategies. Due to inherent ligamentous laxity and connective tissue abnormalities, patients with KS could be predisposed to proximal junctional failure after spinal deformity correction. Pre-operative recognition of connective tissue and immunologic abnormalities, together with detailed surgical planning, is essential to minimize complications and optimize long-term outcomes.
- Academic Primary Care Units: From Dream to RealityPublication . Correia, Gil; Pereira, António; Bulhões, Cláudia; Outeirinho, Conceição; Prazeres, Filipe; Rosendo, Inês; Antunes, José Pedro; Monteiro, Luís; Jacinto, Nuno; Broeiro-Gonçalves, Paula; Santos, Paulo; Simões, Pedro; Resendes Martins, Raquel; Correia, Rubina; Maricoto, Tiago; Santiago, Luiz Miguel
- Assessing Inflammatory Bowel Disease Care Quality in Portugal: A Nationwide Gastroenterologist Survey.Publication . Ministro, Paula; Carvalho, Diana; Correia, Luís; Dias, Cláudia Camila; Gonçalves, Raquel; Lago, Paula; Magro, Fernando; Dias, Sandra; Portela, FranciscoIntroduction: Inflammatory bowel disease (IBD) care demands a multidisciplinary approach and adherence to quality indicators to optimize patient outcomes. This study aimed to evaluate the structure and quality of IBD care in Portugal, exploring the perceptions of gastroenterologists and identifying areas for improvement. Methods: A cross-sectional online survey was conducted among Portuguese gastroenterologists between January and February 2024. The questionnaire assessed IBD care organization, processes and perceptions regarding quality indicators. Responses from 57 participants were analyzed using descriptive and inferential statistics. Results: Most participants (74%) work in public hospitals, with 89.5% providing specialized IBD consultations. Multidisciplinary teams are present in 58% of the participants' centers. In addition to gastroenterologists, IBD multidisciplinary teams include mostly surgeons (88%) and radiologists (62%). Waiting times for nonurgent IBD-specific consultations varied, with 61% of the respondents reporting delays exceeding 1 month. More than two-thirds reported waiting times for nonurgent endoscopic and cross-sectional exams longer than 3 months. Psychological and nutritional assessments were underprioritized, with only 6% and 37% of centers routinely performing these evaluations, respectively. Only 42% of participants answered that their centers keep updated patient registries and 16% used quality indicators known to all team members. Most responders (76%) reported research activity in their units, mainly through national multicenter (88.1%) and observational studies (71%). Units with multidisciplinary teams were significantly more likely to follow structured protocols, provide timely care, and adopt advanced diagnostic tools. Most participants acknowledge the importance of quality indicators, classifying them as mandatory or relevant, with 96% supporting the auditing of IBD centers. Conclusions: This study highlights strengths in the Portuguese IBD care, including multidisciplinary collaboration, research engagement, and widespread awareness of quality indicators. However, challenges remain, such as delays in specialized IBD consultations, endoscopic and cross-sectional exams, and inadequate scheduling for other consultations. Moreover, gaps persist in integrating psychological and nutritional care and maintaining updated patient registries. Expanding multidisciplinary teams and strengthening quality monitoring are critical for improving care outcomes for Portuguese IBD patients.
- Quantifying In Vivo Arterial Deformation from CT and MRI: a Systematic Review of Segmentation, Motion Tracking, and Kinematic Metrics.Publication . Valente, Rodrigo; Henriques, Bernardo; Mourato, André; Xavier, José; Brito, Moisés; Avril, Stéphane; Tomás, António; Fragata, JoséThis article presents a systematic review on methods for quantifying three-dimensional, time-resolved (3D+t) deformation and motion of human arteries from Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we searched Scopus, Web of Science, IEEE Xplore, Google Scholar, and PubMed on 19 December 2025 for in vivo, patient-specific CT or MRI studies reporting motion or deformation of large human arteries. We included studies that quantified arterial deformation or motion tracking and excluded non-vascular tissues, in vitro or purely computational work. Thirty-five studies were included in the qualitative synthesis; most were small, single-centre observational cohorts. Articles were analysed qualitatively, and results were synthesised narratively. Across the 35 studies, the most common segmentation approaches are active contours and threshold, while temporal motion is tracked using either voxel registration or surface methods. These kinematic data are used to compute metrics such as circumferential and longitudinal strain, distensibility, and curvature. Several studies also employ inverse methods to estimate wall stiffness. The findings consistently show that arterial strain decreases with age (on the order of 20% per decade in some cases) and in the presence of disease, that stiffness correlates with geometric remodelling, and that deformation is spatially heterogeneous. However, insufficient data prevents meaningful comparison across methods.