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Necessidades e preocupações dos irmãos em cuidados paliativos pediátricos: scoping review
Get PDFIntroduction: Understanding the needs of each family member in the setting of Pediatric Palliative Care is crucial. Sibling support is still an emerging area, with recommendations based on clinical experience and adaptation from other contexts. The aim of this study was to assess and describe the needs and concerns of siblings of children in Pediatric Palliative Care.
Methods: A literature search was conducted in Medline database. Only research articles with reports of siblings of children in Palliative Care were included. The main characteristics and results of studies included were summarized, and a narrative synthesis was performed.
Results: Nine studies were included showing that, although siblings’ needs vary over the course of the disease, these generally include the need for self-support, access to information, and engagement in brother/sister care. Bereaved siblings are usually resilient, but poor-quality support can have a negative psychosocial impact.
Discussion and Conclusion: Siblings need informational, instrumental, appraisal, and emotional support. Quantitative prospective studies are needed, as well as accurate clinical practice recommendations taking into account the specificities of each child, family, and sibling.info:eu-repo/semantics/publishedVersio
Mucormicose rino-orbito-cerebral num doente oncológico pediátrico
Get PDFAim: Mucormycosis infections are highly lethal in immunosuppressed patients. The authors present a rare case of successful treatment of the condition.
Case description: A 14-year-old male with acute lymphoblastic lymphoma in remission was diagnosed with pansinusitis, cerebral osteomyelitis, and encephalitis with an abscess caused by Rhizopus sp. Neurosurgical drainage, endoscopic sinus surgery, and left eye enucleation were performed, and the patient was simultaneously treated with lipossomal-amphotericin B and posaconazol (later replaced by isavuconazol). However, complications (left frontal lobe herniation and cerebrospinal fluid fistula) ensued, requiring surgical reoperation. Chemotherapy was restarted four months later.
Comments: This is a rare case report, in which early and aggressive surgery, antifungal treatment, and multidisciplinary team work were crucial for the successful outcome.info:eu-repo/semantics/publishedVersio
Orbitofrontal syndrome and “goosebumps”—A new manifestation of autoimmune epilepsy in anti-LGI1 encephalitis
Get PDFinfo:eu-repo/semantics/publishedVersio
Disease-related cortical thinning in presymptomatic granulin mutation carriers
Get PDFMutations in the granulin gene (GRN) cause familial frontotemporal dementia. Understanding the structural brain changes in presymptomatic GRN carriers would enforce the use of neuroimaging biomarkers for early diagnosis and monitoring. We studied 100 presymptomatic GRN mutation carriers and 94 noncarriers from the Genetic Frontotemporal dementia initiative (GENFI), with MRI structural images. We analyzed 3T MRI structural images using the FreeSurfer pipeline to calculate the whole brain cortical thickness (CTh) for each subject. We also perform a vertex-wise general linear model to assess differences between groups in the relationship between CTh and diverse covariables as gender, age, the estimated years to onset and education. We also explored differences according to TMEM106B genotype, a possible disease modifier. Whole brain CTh did not differ between carriers and noncarriers. Both groups showed age-related cortical thinning. The group-by-age interaction analysis showed that this age-related cortical thinning was significantly greater in GRN carriers in the left superior frontal cortex. TMEM106B did not significantly influence the age-related cortical thinning. Our results validate and expand previous findings suggesting an increased CTh loss associated with age and estimated proximity to symptoms onset in GRN carriers, even before the disease onset.info:eu-repo/semantics/publishedVersio
Pyrocarbon Humeral Head in a Shoulder Hemiarthroplasty: Preliminary Results at 3 Years Follow-Up and Review of the Current Literature
Get PDFShoulder hemiarthroplasty is a viable option in young patients with an intact rotator cuff in order to preserve the native glenoid. To avoid the dreaded and expected wear of the glenoid in very active shoulders, implants with humeral head coated with a high resistant and elastic material-pyrolytic carbon-are now an option. The authors present the first pyrocarbon coated hemishoulder arthroplasty performed at our Orthopedic Department in a patient with osteonecrosis of the humeral head. At three years of follow-up, the patient is pain free and without limitations in his daily work. The Constant score was applied pre- and postoperatively, and an improvement of 32 points was reported. Larger cohorts with long-term follow-up are required to confirm our promising results.info:eu-repo/semantics/publishedVersio
Correlação entre a Clearance da Creatinina Estimada e a Taxa de Filtração Glomerular Estimada nos Doentes Muito Idosos e Erros de Prescrição dos Antibióticos: Um Estudo de Coorte
Get PDFIntroduction: Determination of renal function is particularly important when prescribing antibiotics to elderly patients. This study aims to determine the correlation between estimated creatinine clearance and the estimated glomerular filtration rate, for a hospitalized population of very elderly patients, and to audit antibiotic prescribing errors.
Material and methods: Retrospective cohort study of all patients ≥ 80 years hospitalized with antibiotic. Creatinine clearance was calculated using Cockcroft-Gault equation and estimated glomerular filtration rate by Modification of Diet in Renal Disease Study and Chronic Kidney Disease Epidemiology Collaboration equations. Dosing errors were determined through adjustment of daily define dose to renal function.
Results: The study included 589 patients. The correlation of Cockcroft-Gault with Modification of Diet in Renal Disease and Chronic Kidney Disease Epidemiology Collaboration was r = 0.98 and 0.96 for the minimum serum creatinine, and 0.97 and 0.93 for the maximum serum creatinine. Based on Cockcroft-Gault, there were errors in the daily defined dose in 45% in the minimum serum creatinine, and 52% in the maximum serum creatinine day. There was a discrepancy in the recording of errors of 14% to 16% when Cockcroft-Gault was compared with Modification of Diet in Renal Disease and Chronic Kidney Disease Epidemiology Collaboration.
Discussion: There was a good correlation of Cockcroft-Gault with the estimated glomerular filtration rate by Modification of Diet in Renal Disease or Chronic Kidney Disease Epidemiology Collaboration. Regardless of the equation used to estimate renal function there was a high rate of antibiotic dosing errors documented in this population.
Conclusion: This study supports the maintenance of the Cockcroft-Gault equation for drug dosing in the very elderly population. Further studies are needed to investigate underlying causes of prescribing errors.Introdução: A determinação da função renal é particularmente importante na prescrição de antibióticos em doentes idosos. O objetivo deste estudo é correlacionar a clearance de creatinina com a taxa de filtração glomerular estimada, numa população hospitalizada de doentes muito idosos, e auditar os erros de prescrição antibiótica. Material e Métodos: Coorte retrospetivo de todos os doentes ≥ 80 anos hospitalizados com antibioterapia prescrita. A clearance de creatinina foi calculada através da equação Cockcroft-Gault, e a filtração glomerular estimada através das equações Modification of Diet in Renal Disease e Chronic Kidney Disease Epidemiology Collaboration. Os erros de prescrição foram determinados pelo ajuste da dose diária definida à função renal. Resultados: Foram incluídos 589 doentes. A correlação da Cockcroft-Gault com Modification of Diet in Renal Disease e Chronic Kidney Disease Epidemiology Collaboration foi r = 0,98 e 0,96 para a creatinina sérica mínima, e 0,97 e 0,93 para a creatinina sérica máxima. Com base na Cockcroft-Gault, a taxa de erro na dose diária definida foi 45% no dia da creatinina sérica mínima e 52% no dia da creatinina sérica máxima. Quando a Cockcroft-Gault foi comparada com a Modification of Diet in Renal Disease e a Chronic Kidney Disease Epidemiology Collaboration houve uma discrepância no registo de erros de 14% a 16%, respetivamente. Discussão: Verificou-se uma boa correlação entre a Cockcroft-Gault e as equações que calculam a filtração glomerular: Modification of Diet in Renal Disease ou Chronic Kidney Disease Epidemiology Collaboration. Independentemente da equação utilizada para estimar a função renal, foi documentada uma taxa elevada de erros na dose de antibióticos prescrita nesta população. Conclusão: Este estudo reforça a manutenção do uso da equação de Cockcroft-Gault para calcular a dose adequada de antibióticos na população muito idosa. Mais estudos são necessários para investigar as causas subjacentes aos erros de prescrição.info:eu-repo/semantics/publishedVersio
Venomous Animals in the Portuguese Territory: Clinical Management of Bites and Stings
Get PDFThere are many native species of animals with the potential to cause severe disease in Portugal. Of the four clinically relevant snake species, the vipers (Vipera latastei and Vipera seoanei) are the most concerning. They can cause severe disease and require in-hospital management, monitoring and specific treatment, including the administration of anti-snake venom serum. The Malpolon monspessulanus and Macroprotodon brevis snakes cause mostly localized clinical manifestations, which do not require specific treatment. Only a minority of the spider species in Portugal possess chelicerae (mouthparts of the Chelicerata, shaped as either articulated fangs or pincers, which may contain venom or be connected to the venom glands and are used for predation or capture of food) long enough to perforate the human skin. Nevertheless, Latrodectus tredecimguttatus and Loxosceles rufescens spider bites may require specialized treatment in a hospital setting, as well as careful active symptom surveillance. Diversely, the treatment for Scolopendra cingulata and Scolopendra oraniensis centipedes' bites is merely symptomatic. The only existing scorpion species in Portugal is the Buthus ibericus; its sting typically causes local symptoms with intense pain, and its treatment consists essentially of analgesia. The insects of the Hymenoptera order, such as bees and wasps, have the ability to inject venom into the skin. Most people present only with local or regional inflammatory response, and symptomatic treatment is usually effective. Even so, individuals with hypersensitivity to bee venom may develop anaphylaxis. Several marine species in the Portuguese coast are venomous to humans, including weeverfish (Trachinidae family), stingrays (Dasyatis pastinaca, Taeniura grabata, Myliobatis aquila), red scorpion fish (Scorpaena scrofa), cnidaria (Pelagia noctiluca, Chrysaora hysoscella, Physalia physalis) and bearded fireworm (Hermodice carunculata); treatment is symptomatic. Contact with the larvae or bristles (chitinous structures with locomotor or tactile functions) of Thaumetopoea pityocampa (pine processionary) can cause cutaneous, ocular and, rarely, respiratory reactions; its management is also symptomatic.Em território português, existem várias espécies autóctones de animais capazes de provocar envenenamento e doença clinicamente significativa, com potencial de gravidade. Das quatro espécies de serpentes com relevância clínica, as víboras (Vipera latastei e Vipera seoanei) são as mais preocupantes; podem causar doença grave e necessitam de abordagem hospitalar, monitorização e tratamento específico, incluindo soro antiveneno. As serpentes Malpolon monspessulanus e Macroprotodon brevis causam maioritariamente manifestações clínicas locais, sem tratamento específico. Apenas uma minoria das espécies de aranha presentes em Portugal possui quelíceras (apêndices bucais dos Chelicerata, em forma de tenaz ou gancho, que podem conter veneno ou encontrar-se ligados às glândulas venenosas, usados para predação ou captura de alimentos) suficientemente compridas para perfurar a pele humana, mas as picadas de Latrodectus tredecimguttatus e Loxosceles rufescens podem implicar tratamento diferenciado em ambiente hospitalar, com necessidade de vigilância ativa. O tratamento da picada das centopeias Scolopendra cingulata e Scolopendra oraniensis é sintomático. A única espécie de escorpião presente em território nacional, Buthus ibericus, causa geralmente sintomas locais com dor intensa; o tratamento habitualmente baseia-se apenas na administração de analgesia. Os insetos da ordem Hymenoptera, como as abelhas e vespas, possuem capacidade de libertar veneno através de picadas; a maior parte dos indivíduos apresenta apenas reações inflamatórias locais ou regionais, para as quais a aplicação de medidas sintomáticas é suficiente, mas indivíduos que apresentem hipersensibilidade ao veneno podem desenvolver reações anafiláticas. Na costa portuguesa e em águas pouco profundas, encontram-se várias espécies marinhas venenosas para os humanos, como Echiichthys vipera (peixe-aranha comum), Dasyatis pastinaca, Taeniura grabata e Myliobatis aquila (ratões), Scorpaena scrofa (rascasso-vermelho), Pelagia noctiluca, Chrysaora hysoscella e Physalia physalis (cnidários) e Hermodice carunculata (verme-do-fogo), cujas picadas necessitam apenas de tratamento sintomático. O contacto com as larvas ou cerdas (estruturas quitinosas com funções locomotoras ou tácteis) de Thaumetopoea pityocampa (lagarta-do-pinheiro) pode provocar reações cutâneas, oculares e, raramente, respiratórias; a sua abordagem também é sintomática.info:eu-repo/semantics/publishedVersio
Hyperacusis and stapes surgery: An observation in fifty patients after stapedotomy
Get PDFObjective: To assess hyperacusis after stapedotomy and its possible influencing factors.
Study design: Prospective, interventional, and longitudinal study.
Setting: A tertiary referral center.
Patients: Fifty consecutive patients (35 females, mean age = 46.8 years).
Intervention: All patients underwent stapedotomy. The validated Portuguese version of the "Hyperacusis Questionnaire" (HQ) was administered before and two weeks and one month after surgery.
Results: No hyperacusis was reported by any patient before surgery. At two weeks after surgery, all patients experienced hyperacusis, with a mean HQ at 16.88 ± 6.54 (range 4-25). One month after surgery, hyperacusis had already resolved in most patients. Gender, preoperative presentation or surgeon had no influence on HQ scores (p > 0.05). Patients with previous contralateral stapedotomy showed lower HQ scores (p = 0.001). Audiological parameters improvement measured at one month after surgery (PTA, SRT and contralateral SRT) were associated with HQ higher scores.
Conclusion: This study confirms that hyperacusis is a common complaint after stapedotomy that usually resolves in one month after surgery. The HQ highest scores were registered among patients with the highest audiological gain after surgery. This suggests that hyperacusis may be a positive prognostic factor for audiological success after stapedotomy.info:eu-repo/semantics/publishedVersio
Intense Pulsed Plus Low-Level Light Therapy in Meibomian Gland Dysfunction
Get PDFPurpose: To analyse the clinical outcome in patients with meibomian gland dysfunction (MGD) who underwent intense pulsed light (IPL) plus low-level light therapy (LLL).
Materials and methods: The prospective non-comparative study included identified by MGD patients with altered interferometry and lower loss area of the meibomian glands (LAMG), who underwent IPL plus LLL, between July 2020 and August 2020. A multimodal assessment was performed before, 2-3 weeks, and 6 months after treatment. The main outcome was lipid layer thickness (LLT) and the secondary outcomes were the ocular surface disease index (OSDI) score, presence of corneal fluorescein staining (CFS), blink rate (BR), Schirmer test (ST), tear meniscus height (TMH), tear osmolarity (OSM), non-invasive break-up time (NIBUT) and LAMG.
Results: This study included 62 eyes of 31 patients, 61.3% female, with a mean age of 66.94±9.08 years at the time of IPL plus LLL treatment. LLT (<0.001) grades improved 6 months after treatment. The mean OSDI score improved (p<0.001) from 45.02±21.17 (severe symptoms) to 22.35±17.68 (moderate symptoms) at 2-3 weeks and 8.24±17.9.91 (normal) at 6 months after treatment. CFS was identified in 51.6% (32/62) before and in 45.2% (28/62) 6 months (p=0.293) after treatment. ST (p=0.014) grades improved; OSM grades mild worsened (p<0.001); TMH, NIBUT and LAMG grades did not modify 6 months after treatment. No patient suffered any adverse effects.
Conclusion: IPL combined with LLL was effective and safe, improving the lipid layer thickness in MGD and decreasing the level of symptoms.info:eu-repo/semantics/publishedVersio
Intervenções no Plexo Celíaco Guiadas por Ecoendoscopia
Get PDFAbdominal pain related to pancreatic disease is often extremely disabling. Endoscopic ultrasound (EUS)-guided celiac plexus block (CPB) is used to control pain associated with chronic pancreatitis. EUS-guided celiac plexus neurolysis (CPN) is typically used to reduce pain associated with pancreatic cancer and can be considered early at the time of diagnosis of inoperable disease. EUS-guided celiac plexus interventions have been shown to be significantly effective in pancreatic pain relief, which is achieved in approximately 70-80% of patients with pancreatic cancer and in 50-60% of those with chronic pancreatitis. Serious complications from CPB and CPN are rare. Most frequent adverse events are diarrhoea, orthostatic hypotension, and a transient increase in abdominal pain. In this article, the Portuguese Group for Ultrasound in Gastroenterology (GRUPUGE) presents an updated perspective of the potential role of EUS-guided celiac plexus interventions, addressing the selection criteria and technical issues of different techniques and analysing recent data on their safety and efficacy.A dor abdominal relacionada com doença pancreática pode ser extremamente incapacitante. O bloqueio do plexo celíaco (CPB) guiado por ecoendoscopia (EUS) é utilizado para controlo da dor associada à pancreatite crónica. A neurólise do plexo celíaco (CPN) guiada por EUS é utilizada na terapêutica da dor associada ao cancro do pâncreas, podendo ser utilizada numa fase precoce, após o diagnóstico de doença irressecável. As intervenções no plexo celíaco guiadas por EUS demonstraram ser eficazes no alívio da dor. O sucesso terapêutico é alcançado em aproximadamente 70 a 80% dos pacientes com cancro do pâncreas, e em 50 a 60% dos pacientes com pancreatite crónica. São raras as complicações severas associadas a CPB e CPN. Os eventos adversos mais frequentemente documentados correspondem a diarreia, hipotensão ortostática e a aumento transitório da dor abdominal. Neste artigo, o GRUPUGE apresenta uma perspetiva das intervenções no plexo celíaco guiadas por EUS, onde se abordam os critérios de seleção, questões técnicas relativas aos procedimentos e se analisam os dados sobre sua segurança e eficácia.info:eu-repo/semantics/publishedVersio