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Prognostic Factors for Functional Improvement After Pars Plana Vitrectomy and Epiretinal Membrane Peeling
No full textIntrodução: Estudos anteriores confirmaram que a integridade da camada de fotorreceptores influencia o resultado visual após vitrectomia pars plana (VPP) e pelagem de
membrana epirretiniana (MER). Recentemente, foi estudada a influência das alterações da retiniana interna no prognóstico visual. O nosso estudo examina os fatores de prognóstico para a melhoria visual pós-cirurgia num hospital terciário português.
Métodos: Os registos médicos de 234 pacientes foram revistos retrospetivamente. Foram incluídos quarenta e nove olhos com MER idiopática tratados por VPP foram incluídos. Classificação de Govetto, tipo de conexão da MER, espessura foveal central (EFC), espessura máxima da retina (EMR), alterações cistóides intrarretinianas, alterações da retina interna (presença e espessura da camada foveal interna ectópica (CFIE), espessura da camada plexiforme interna e das células ganglionares (CG-CPI) e a desorganização das camadas internas da retina (DCIR)) e as alterações retinianas externas (cotton ball, descolamento foveolar, lesão viteliforme adquirida, integridade
da zona elipsóide (ZE) e zona de interdigitação (ZI)) foram estudados no pré-operatório, 3, 6 e 12 meses após a cirurgia. As correlações entre as características pré-operatórias do OCT e o resultado visual final foram analisadas.
Resultados: Não foi encontrada correlação entre a idade e cirurgia de catarata concomitante na melhor acuidade visual corrigida (MAVC) pós-operatória. Foi estabelecida uma correlação positiva entre a MAVC pré e pós-operatória. A EFC pré-operatória teve uma correlação inversa com a MAVC em todos os momentos de avaliação. A pontuação de Govetto mostrou uma correlação positiva com a EFC pré-operatória, EMR e pontuação de DRIL, e correlação negativa com a MAVC pré-operatória e pós-operatória. A presença de DRIL pré-operatório correlacionou-se com uma MAVC inferior aos 3 meses e 1 ano. A espessura da CFIE pré-operatória não influenciou significativamente a MAVC em nenhum momento. Quistos intrarretinianos préoperatórios correlacionam-se com menor MAVC pré e pós-operatória. Alterações pré-cirúrgicas nas ZE e ZI correlacionam-se com menor MAVC inicial e pós-operatória. Os outros parâmetros não influenciaram a MAVC final.
Conclusão: A MAVC pré-operatória, EFC, DCIR, cistos intrarretinianos, disrupções ZI e ZE e pontuação de Govetto pré-operatórios influenciam a MAVC pós-operatória. Estes resultados destacam a importância de uma avaliação pré-cirúrgica detalhada utilizando OCT para otimizar as decisões no tratamento de ERMiIntroduction: Earlier studies confirmed that the integrity of the photoreceptor layer may influence visual outcome after pars plana vitrectomy (PPV) and epiretinal membrane (ERM) peeling. Recently, distortion of inner retinal architecture has been studied. Our study examines prognosis factors for visual improvement post-surgery in a Portuguese tertiary hospital.
Methods: The medical records of 234 patients were retrospectively reviewed. Forty-nine eyes of 49 patients with idiopathic ERM treated by PPV and membrane peeling were included in the study. Govetto classification, ERM connection type, central foveal subfield thickness (CST), maximum retinal thickness (MRT), intraretinal cystoid changes, inner retinal changes (presence and thickness of ectopic inner foveal layer (EIFL), ganglion cell-inner plexiform layer (GC-IPL) thickness, and disorganization of retinal inner layers (DRIL)) and outer retinal changes (cotton ball, foveolar detachment, acquired vitelliform lesion, integrity of the ellipsoid zone and interdigitation zone) were studied pre-operatively, at 3, 6 and 12 months after surgery. Correlations between baseline OCT measures and visual outcome were analyzed.
Results: No correlation was found between age and concurrent cataract surgery on post-op best-corrected visual acuity (BCVA). A positive correlation was established between pre-operative and post-operative BCVA. Pre-operative CST had a significant inverse correlation with BCVA at all follow-up timepoints. Govetto score showed a positive correlation with pre-operative CST, MRT, and DRIL score, and negative correlation with pre-operative and postoperative BCVA. The presence of DRIL at baseline predicted decreased BCVA at 3-month and 1-year post-operative assessments. Pre-operative EIFL thickness did not significantly influence BCVA at any evaluation point. Eyes with pre-operative intraretinal cysts showed significantly lower BCVA at baseline and at the 12-month follow-up. Disruptions in the IZ and EZ lines pre-operatively were associated with lower baseline BCVA and poorer post-operative visual outcomes. The other evaluated parameters did not influence the final BCVA.
Conclusion: The study reveals the influence of preoperative BCVA, CST, DRIL, intraretinal cysts, IZ and EZ disruptions, and Govetto score on postoperative visual acuity iniERM patients. These findings emphasize the value of comprehensive preoperative retinal assessments for improved surgical decision-making in iERM treatment
Paratesticular Fibrous Pseudotumor in a Pediatric Patient: A Case Report.
No full textParatesticular fibrous pseudotumors are rare benign tumors. This case reports paratesticular fibrous pseudotumors in a very young patient. A previously healthy 16-month-old boy was seen due to a growing scrotal mass. On clinical examination, there was a painless, multinodular scrotal mass. Tumor markers were normal, and a testicular ultrasound with Doppler revealed a solid, avascular, and hypoechoic mass (50x20 mm). The patient underwent excision of the scrotal mass and adjacent skin. The histological analysis revealed a paratesticular fibrous pseudotumor. Definitive treatment is surgical excision, and if there is any concern for malignancy, an extemporaneous examination should be done to confirm the diagnosis. The prognosis with fibrous pseudotumors is excellent
When a Child Refuses to Play: A Rare Myopathy.
No full textIdiopathic inflammatory myopathies (IIM) are a rare group of systemic diseases characterized by progressive proximal muscle weakness and skeletal muscle inflammation. We describe a clinical report of a seven-year-old boy presenting with myalgia and proximal muscle weakness beginning three weeks earlier, with laboratory, MRI, and muscle biopsy findings consistent with IIM. The patient was treated with corticosteroids, methotrexate, immunoglobulin, and intensive motor rehabilitation, with favorable evolution. Diagnosis of Juvenile Polymyositis was confirmed. Three years later, we assisted a relapse of muscle weakness and muscle cytolysis with the onset of bilateral eyelid skin microulcers compatible with dermatomyositis. This report intends to highlight the importance of early diagnosis and treatment in IIM due to the significant burden associated with this group of diseases. In this case, the late onset of the skin lesion contributed to the challenge in this diagnosis
Consulta Multidisciplinar de Doenças Neurocutâneas: Experiência de Cinco Anos num Hospital Pediátrico Terciário em Portugal
No full textIntroduction: Neurocutaneous syndromes (NCS) are a heterogeneous group of conditions with multiorgan involvement and diverse manifestations, evolving throughout life with significant morbidity. A multidisciplinary approach to NCS patients has been advocated, although a specific model is not yet established. The aim of this study was 1) to describe the organization of the recently created Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases (MOCND) at a Portuguese pediatric tertiary hospital; 2) to share our institutional experience focusing on the most common conditions, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC); 3) to analyze the advantages of a multidisciplinary center and approach in NCS.
Methods: Retrospective analysis of 281 patients enrolled in the MOCND over the first five years of activity (October 2016 to December 2021), reviewing genetics, family history, clinical features, complications, and therapeutic strategies for NF1 and TSC.
Results: The clinic works weekly with a core team of pediatricians and pediatric neurologists supported by other specialties as needed. Of the 281 patients enrolled, 224 (79.7%) had identifiable syndromes such as NF1 (n = 105), TSC (n = 35), hypomelanosis of Ito (n = 11), Sturge-Weber syndrome (n = 5), and others. In NF1 patients, 41.0% had a positive family history, all manifested café-au-lait macules, 38.1% neurofibromas with 45.0% being large plexiform neurofibromas. Sixteen were under treatment with selumetinib. Genetic testing was performed in 82.9% of TSC patients with pathogenic variants found in TSC2 gene in 72.4% patients (82.7% if considered contiguous gene syndrome). Family history was positive in 31.4%. All TSC patients presented hypomelanotic macules and fulfilled diagnostic criteria. Fourteen patients were being treated with mTOR inhibitors.
Conclusion: Offering a systematic and multidisciplinary approach to NCS patients enables timely diagnosis, promotes a structured follow-up, and encourages discussion to outline management plans for optimal care to every patient, with significant impact on the quality of life of patients and families.Introdução: As doenças neurocutâneas (DNC) são um grupo heterogéneo de patologias com envolvimento multiorgânico e manifestações diversas que evoluem ao longo da vida, com morbilidade significativa. Tem sido preconizada uma abordagem multidisciplinar destes doentes, contudo o modelo ideal não está ainda estabelecido. Este trabalho tem como objetivos 1) descrever a organização da recém-criada Consulta Multidisciplinar de Doenças Neurocutâneas (CMDNC) de um hospital pediátrico terciário em Portugal; 2) partilhar a experiência institucional, focando as patologias mais comuns, neurofibromatose tipo 1 (NF1) e complexo esclerose tuberosa (CET); e 3) analisar as vantagens de um centro e abordagem multidisciplinares nas DNC.
Métodos: Análise retrospetiva dos 281 doentes acompanhados na CMDNC durante os primeiros cinco anos de funcionamento (outubro 2016 a dezembro 2021), com revisão da genética, história familiar, manifestações clínicas, complicações e estratégias terapêuticas dos doentes com NF1 e CET.
Resultados: A CMDNC funciona semanalmente com um pediatra e um neuropediatra, com apoio de outras especialidades sempre que necessário. Dos 281 doentes acompanhados, 224 (79,7%) têm síndromes identificados, como NF1 (n = 105), CET (n = 35), hipomelanose de Ito (n = 11), síndrome de Sturge-Weber (n = 5), e outras. Dos doentes com NF1, 41,0% têm história familiar positiva, todos apresentavam manchas ‘café com leite’, 38,1% neurofibromas, dos quais 45,0% com grandes neurofibromas plexiformes. Dezasseis estavam sob tratamento com selumetinib. Foi realizado estudo
genético em 82,9% dos doentes com CET, com variantes patogénicas identificadas no gene TSC2 em 72,4% (82,7% se considerado síndrome de genes contíguos). Em 31,4% havia história familiar positiva. Todos os doentes com CET apresentaram máculas hipomelanocíticas e cumpriam critérios diagnósticos. Catorze doentes estavam sob tratamento com inibidores mTOR.
Conclusão: Oferecer uma abordagem sistematizada e multidisciplinar nas DNC possibilita um diagnóstico atempado, promove um acompanhamento estruturado, e favorece a discussão para delinear um plano de cuidados adequado, com impacto significativo na qualidade de vida dos doentes e famílias
Organ Crosstalk and Dysfunction in Sepsis.
No full textSepsis is a dysregulated immune response to an infection that leads to organ dysfunction. Sepsis-associated organ dysfunction involves multiple inflammatory mechanisms and complex metabolic reprogramming of cellular function. These mechanisms cooperate through multiple organs and systems according to a complex set of long-distance communications mediated by cellular pathways, solutes, and neurohormonal actions. In sepsis, the concept of organ crosstalk involves the dysregulation of one system, which triggers compensatory mechanisms in other systems that can induce further damage. Despite the abundance of studies published on organ crosstalk in the last decade, there is a need to formulate a more comprehensive framework involving all organs to create a more detailed picture of sepsis. In this paper, we review the literature published on organ crosstalk in the last 10 years and explore how these relationships affect the progression of organ failure in patients with septic shock. We explored these relationships in terms of the heart-kidney-lung, gut-microbiome-liver-brain, and adipose tissue-muscle-bone crosstalk in sepsis patients. A deep connection exists among these organs based on crosstalk. We also review how multiple therapeutic interventions administered in intensive care units, such as mechanical ventilation, antibiotics, anesthesia, nutrition, and proton pump inhibitors, affect these systems and must be carefully considered when managing septic patients. The progression to multiple organ dysfunction syndrome in sepsis patients is still one of the most frequent causes of death in critically ill patients. A better understanding and monitoring of the mechanics of organ crosstalk will enable the anticipation of organ damage and the development of individualized therapeutic strategies
The Effectiveness of Various CSF Diversion Surgeries in Idiopathic Normal Pressure Hydrocephalus: a Systematic Review and Meta-Analysis.
No full textBackground: Idiopathic normal pressure hydrocephalus (iNPH) is commonly treated using cerebrospinal fluid (CSF) diversion procedures, most commonly ventriculoperitoneal (VP) but also lumboperitoneal (LP), ventriculoatrial (VA) shunting, and endoscopic third-ventriculostomy (ETV). Despite the prevalence of these interventions and recent advancements in iNPH diagnostic processes, there is limited up-to-date evidence regarding surgical outcomes.
Methods: A systematic review and meta-analysis were conducted to analyse the effects of CSF diversion surgeries among iNPH patients. The primary outcome was efficacy of the CSF diversion procedure, defined as symptomatic improvement, and secondary outcomes included surgical complications. Several major databases were searched for original studies from inception up to June 4, 2024, which were evaluated using random-effects meta-analyses, meta-regression, and influence analyses. This study was registered with PROSPERO: CRD42023458526.
Findings: Out of the 1963 studies screened, 54 were included in this review, and 4811 patients were pooled. Overall, more than 74% of patients experienced improvement after surgical treatment (95% CI: 70-78%). VP shunting demonstrated an efficacy of 75% (95% CI 70-79%), VA shunting at 75% (95% CI: 70-80%), and LP shunting at 70% (95% CI: 52-83%). ETV had a success rate of 69% (95% CI: 58-78%). Gait improvement was high at 72% (95% CI: 67-77%), while urinary and cognitive dysfunction each improved in approximately 50% of patients. The efficacy of surgery did not increase between 2005 and 2024 (p = 0.54). Complications occurred in 20.6% of cases, with a surgery revision rate of 15.1%.
Interpretation: This meta-analysis found that the overall efficacy of CSF diversion procedures for iNPH remained unchanged from 2005 to 2024, with 74% of cases showing improvement. No procedure was found to be clearly superior, and only half of the patients saw improvements in urinary and cognitive dysfunction. The stagnant efficacy over time and frequent complications highlight the need for improved patient selection criteria to best identify those most likely to benefit from CSF shunting.
Funding: None for this study
Ketodex: A Game-Changer in Pediatric Sedation for Challenging Airway.
No full textManaging 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
Risk Factors of Acute T‑Cell Mediated and Borderline Rejection Diagnosed in Early Protocol Kidney Transplant Biopsies
No full textIntroduction: Acute graft rejection remains one of the main causes of graft dysfunction and premature loss. Understanding factors affecting graft rejection is essential to promote graft survival. Our study aimed to determine the
incidence and assess risk factors of acute T‑cell mediated rejection (TCMR) and borderline rejection in early protocol
kidney transplant biopsies.
Methods: Retrospective single‑center study of kidney transplant recipients between January 2021 and June 2022.
Patients underwent protocol kidney biopsy during the first 2 weeks after transplantation. According to biopsy results,
patients were classified into two groups: patients with TCMR or borderline rejection, and those without rejection. Histological changes were evaluated and graded based on Banff classification 2019. Rejections in patients without delayed graft function requiring hemodialysis (HD) were classified as subclinical. Logistic regression analysis was performed to identify predictors of early acute rejection.
Results: Fourteen patients (15.9%) presented TCMR or borderline rejection, of which the majority (71.4%) had subclinical
rejection. A significant higher proportion of patients with acute rejection were treated with basiliximab (13 (92.8%) vs 1 (7.2%), p=0.001). Patients with acute rejection had lower mean HLA mismatches (2.71 ± 0.83 vs 3.46 ± 1.41, p=0.011) and longer cold ischemia time, although not statistically significant (11.72 ± 5.39 vs 8.93 ± 3.56 hours, p=0.067). In the logistic regression analysis only induction therapy with basiliximab remained a strong predictor for early acute rejection [(OR) 36.8 (CI: 3.72 – 362.46), p=0.002].
Conclusion: In our cohort induction therapy with basiliximab appear to significantly increase the risk of early TCMR and borderline rejection. Early diagnosis with protocol kidney biopsies could be crucial to adopt the appropriate therapeutic strategie
A Stratified Approach for Managing Patients With Low Back Pain in Primary Care (SPLIT Program): A Before-and-After Study.
No full textPurpose: To determine the effects of stratified primary care for low back pain (SPLIT program) in decreasing back-related disability for patients with low back pain (LBP) in primary care.
Methods: We conducted a before-and-after study. We compared health-related outcomes for 2 sequential, independent cohorts of patients with LBP recruited at 7 primary care units in Portugal. The first prospective cohort study characterized usual care (UC) and collected data from February to September 2018. The second was performed when the SPLIT program was implemented and collected data from November 2018 to October 2021. Between cohorts, physical therapists were trained in the implementation of the SPLIT program, which used the STarT Back Screening Tool to categorize patients for matched treatment. We compared back-related disability (Roland-Morris Disability Questionnaire, 0-24 points), pain (Numeric Pain Rating Scale, 0-10 points), perceived effect of treatment (Global Perceived Effect Scale, -5 to +5 points), and health-related quality of life (EuroQoL 5 dimensions 3 levels index, 0-1 points).
Results: We enrolled a total of 447 patients: 115 in the UC cohort (mostly treated with pharmacologic treatment) and 332 in the SPLIT cohort (all referred for a physical therapy intervention program). Over the study period of 6 months, patients in the SPLIT program showed significantly greater improvements in back-related disability (ß, -2.94; 95% CI, -3.63 to -2.24; P ≤ .001), pain (ß, -0.88; 95% CI, -1.18 to -0.57; P ≤ .001), perceived effect of treatment (ß, 1.40; 95% CI, 0.97 to 1.82; P ≤ .001), and health-related quality of life (ß, 0.11; 95% CI, 0.08 to 0.14; P ≤ .001) compared with UC.
Conclusions: Patients in the SPLIT program for LBP showed greater benefits regarding health-related outcomes than those receiving UC
Study Protocol for a Randomized Controlled Trial Testing the Efficacy of Emotion Detectives In-Out: a Blended Version of the Unified Protocol for Transdiagnostic Treatment of Emotional Disorders in Portuguese Children
No full textChildhood emotional disorders (EDs; i.e., anxiety and depressive disorders) are currently a public health concern. Their high prevalence, long-term effects, and profound influence on the lives of children and families highlight the need to identify and treat these disorders as early and effectively as possible. This clinical trial will examine the efficacy of a blended version (i.e., combining face-to-face and online sessions into one treatment protocol) of the Unified Protocol for Children (the "Emotion Detectives In-Out" program). This program is a manualized cognitive-behavioral therapy for the transdiagnostic treatment of EDs in children aged 7 to 12 years that aims to reduce the intensity and frequency of strong and aversive emotional experiences by helping children learn how to confront those emotions and respond to them in more adaptive ways.This study is designed as a multicenter equivalence randomized controlled parallel-group two-arm trial comparing the Emotion Detectives In-Out program with an evidenced-based group intervention for children with anxiety disorders (the Coping Cat program). Participants will be children aged between 7 and 12 years with an anxiety disorder or with clinically significant anxiety symptoms as well as one of their parents or a legal representative. A minimum sample size of 138 children (69 per group) is needed to test whether the efficacy of the proposed intervention is equivalent to that of the well-established Coping Cat intervention.We expect Emotion Detectives In-Out to be a feasible and efficacious alternative intervention for treating children's EDs by allowing for a greater increase in children's access to care. A blended format is expected to overcome common barriers to treatment (e.g., parents´ lack of time to attend regular sessions) and make the intervention more accessible to families.The clinical trial is registered at ClinicalTrials.gov (Identifier: NCT05747131, date assigned February 28, 2023)