Centro Hospitalar do Porto
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Portuguese Consensus on the Diagnosis and Management of Lewy Body Dementia (PORTUCALE)
Get PDFLewy body dementia is a common cause of dementia leading to the progressive deterioration of cognitive function and motor skills, behavioral changes, and loss of autonomy, impairing the quality of life of patients and their families. Even though it is the second leading cause of neurodegenerative dementia, diagnosis is still challenging, due to its heterogenous clinical presentation, especially in the early stages of the disease. Accordingly, Lewy body dementia is often misdiagnosed and clinically mismanaged. The lack of diagnostic accuracy has important implications for patients, given their increased susceptibility to the adverse effects of certain drugs, such as antipsychotics, which may worsen some symptoms associated with Lewy body dementia. Therefore, a specialist consensus based on the analysis of the most updated and relevant literature, and on clinical experience, is useful to all professionals involved in the care of these patients. This work aims to inform and provide recommendations about the best diagnostic and therapeutic approaches in Lewy body dementia in Portugal. Moreover, we suggest some strategies in order to raise the awareness of physicians, policy makers, and the society at large regarding this disease.A demência com corpos de Lewy é uma causa comum de demência, provocando a perda progressiva de funções cognitivas e capacidades motoras, alterações comportamentais, e perda de autonomia, com compromisso da qualidade de vida dos doentes e seus familiares. Apesar de ser a segunda causa mais frequente de demência neurodegenerativa, o diagnóstico mantém-se um desafio, devido à sua apresentação clínica heterogénea, sobretudo nas fases iniciais da doença. Por conseguinte, a demência com corpos de Lewy é frequentemente mal diagnosticada e clinicamente gerida de forma insuficiente. A falta de acuidade diagnóstica tem implicações significativas para os doentes, dada a maior suscetibilidade aos efeitos adversos de determinados fármacos, tais como os antipsicóticos, que podem agravar alguns sintomas associados à demência com corpos de Lewy. Por conseguinte, um consenso de especialistas, baseado na análise da literatura mais atual e relevante, e na experiência clínica, é útil para todos os profissionais envolvidos no cuidado destes doentes. O objetivo deste trabalho é informar e gerar recomendações acerca das melhores abordagens diagnóstica e terapêutica da demência com corpos de Lewy em Portugal. Além disso, sugerimos estratégias para aumentar a sensibilização dos médicos, dos decisores políticos e da sociedade em geral em relação a esta doença.Este trabalho foi parcialmente financiado pela GE Healthcare Espanha para apoio à logística da realização da reunião de consenso e para apoio de medical writing no âmbito da preparação deste artigo. A GE Healthcare Espanha não teve qualquer papel no desenho do consenso, recolha, análise e interpretação de literatura, redação do manuscrito, nem na decisão de submeter o artigo para publicação. As opiniões expressas no artigo são da responsabilidade dos autores e não são necessariamente as da GE Healthcare Espanha.info:eu-repo/semantics/publishedVersio
Preschool Children’s Emotional Understanding of Death: A Forgotten Dimension
Get PDFIntrodução: É ainda parca a evidência científica quanto ao entendimento que as crianças têm sobre o conceito de morte, recentemente apontado pela International Children’s Palliative Care Network como área prioritária de investigação. Em particular, a evicção da emoção nesta área de investigação é uma lacuna importante. Este estudo visa desenvolver uma visão aprofundada da dimensão emocional da compreensão da morte pela criança, procurando, também, relacioná-la com a dimensão cognitiva.
Material e Métodos: Entrevistámos crianças (três a seis anos), com recurso a um livro ilustrando o cenário hipotético em que uma criança se deparava com a de morte de um familiar. Colocámos questões para avaliar os subconceitos cognitivos da morte e a dimensão emocional (o que sentiria a criança e o que lhe devia ser dito pelos pais).
Resultados: Dos 54 participantes, a maioria disse que a criança se sentiria triste (n = 46, 85%) e que os pais deveriam informá-la (n = 47, 87%); estas respostas não variaram significativamente com a idade. A compreensão cognitiva do conceito de morte das crianças que referiram a tristeza foi significativamente superior.
Discussão: Mesmo as crianças mais pequenas sentem a morte, não sendo possível desligar as compreensões cognitiva e emocional. Adicionalmente, as crianças devem ser informadas, com vista a uma elaboração adequada e multidimensional da morte. Conclusão: Este estudo fornece informação valiosa aos profissionais de saúde e outros adultos interessados sobre a forma como a criança em idade pré-escolar se posiciona em relação à morte.Introduction: Scientific evidence regarding children’s understanding of the concept of death is scarce. This has recently been pointed out by the International Children’s Palliative Care Network as a priority area of research. In particular, the avoidance of emotion in this area of research is an important shortcoming. This study aims to develop an in-depth view of the emotional dimension of the child’s understanding of death, also seeking to relate it to the cognitive dimension.
Material and Methods: We interviewed children (three to six years old) using a book illustrating a hypothetical scenario in which a child faced the death of a relative. We asked questions to assess the cognitive subconcepts of death and the emotional dimension (what the child would feel and what parents should say).
Results: Of the 54 participants, the majority said that the child would feel sad (n = 46, 85%) and that parents should inform her/him (n = 47, 87%); these responses did not vary significantly with age. The cognitive understanding of the concept of death in children who reported sadness was significantly higher.
Discussion: Even the youngest children feel death, and it is not possible to disconnect cognitive and emotional understanding. Additionally, children should be informed in order to foster a proper and multidimensional elaboration of death.
Conclusion: This study provides valuable information to health professionals and other interested adults about the way preschoolers position themselves in relation to deathinfo:eu-repo/semantics/publishedVersio
Human Peripheral Blood Gamma Delta T Cells: Report on a Series of Healthy Caucasian Portuguese Adults and Comprehensive Review of the Literature
Get PDFGamma delta T cells (Tc) are divided according to the type of Vδ and Vγ chains they express, with two major γδ Tc subsets being recognized in humans: Vδ2Vγ9 and Vδ1. Despite many studies in pathological conditions, only a few have quantified the γδ Tc subsets in healthy adults, and a comprehensive review of the factors influencing its representation in the blood is missing. Here we quantified the total γδ Tc and the Vδ2/Vγ9 and Vδ1 Tc subsets in the blood from 30 healthy, Caucasian, Portuguese adults, we characterized their immunophenotype by 8-color flow cytometry, focusing in a few relevant Tc markers (CD3/TCR-γδ, CD5, CD8), and costimulatory (CD28), cytotoxic (CD16) and adhesion (CD56) molecules, and we examined the impacts of age and gender. Additionally, we reviewed the literature on the influences of race/ethnicity, age, gender, special periods of life, past infections, diet, medications and concomitant diseases on γδ Tc and their subsets. Given the multitude of factors influencing the γδ Tc repertoire and immunophenotype and the high variation observed, caution should be taken in interpreting "abnormal" γδ Tc values and repertoire deviations, and the clinical significance of small populations of "phenotypically abnormal" γδ Tc in the blood.The Centro Hospitalar Universitário do Porto made available the necessary infrastructure and equipment and gave financial support to this study (grant 036/11-021-DEFI/036-CES)info:eu-repo/semantics/publishedVersio
Interrupção do tratamento em criança nascida a termo pequena para idade gestacional
Get PDFSmall-for-gestational-age children have been associated with up to 20% delayed growth and delayed development, increased childhood morbimortality, and poor quality of life. Prepubertal growth hormone administration results in increased growth and musculoskeletal development. The present case report illustrates the negative impact of growth hormone disruption in a 6-year-old patient, resulting in impaired growth and development. Close clinical and laboratory monitoring is recommended for small-for-gestational-age children receiving growth hormone treatment, and family counselling should be encouraged.info:eu-repo/semantics/publishedVersio
Qualidade de vida de adolescentes com diabetes mellitus tipo 1
Get PDFIntroduction: Type 1 diabetes mellitus is one of the most common endocrine-metabolic disorders of childhood and adolescence and requires a continuous and rigorous therapeutic approach, with recognized impact on children and adolescents’ quality of life.
The purpose of this study was to evaluate the quality of life of adolescents with type 1 diabetes mellitus and its relationship with clinical and laboratory aspects and lifestyle.
Material and Methods: DQOL questionnaire was applied to type 1 diabetes mellitus adolescents managed at the Pediatric Diabetology consultation of a level II hospital for more than one year. Questionnaire has a global score between 36 and 180, with higher scores reflecting worse quality of life. Statistical analysis was performed in SPSS®.
Results: Seventy-one percent (n=36) of adolescents responded to the survey, 55.6% of which male, with a median age of 15 years. Median DQOL global score was 66. Adolescents with good metabolic control had a median global score of 49, compared with 71 in adolescents with poor metabolic control (p=0.007). The median global score of self-perception of better health was 51 compared to 73 for self-perception of poorer health (p=0.007).
Discussion: In general, adolescents in this study revealed a satisfactory quality of life. Adolescents with better metabolic control have a higher satisfaction and better quality of life. Adolescents with better self-perceived health have a better quality of life.
Conclusion: Recognizing factors that affect quality of life of adolescents with type 1 diabetes mellitus is crucial to devise therapeutic strategies that meet their expectations, promoting treatment adherence and better metabolic control.info:eu-repo/semantics/publishedVersio
Dor abdominal aguda em adolescente de 13 anos
Get PDFIntroduction: Ovarian torsion can occur at any pediatric age, mainly between the ages of 9 and 14 years. Diagnosis is challenging, as symptoms are nonspecific, misleading to other more common diagnoses, as genitourinary and gastrointestinal disorders. In children, ovarian lesions leading to torsion are typically benign and cystic. Surgical approach is safe, as most cases of early torsed ovary untwisting exhibit later normal ovarian function.
Case report: A 13-year-old female adolescent was referred to the Emergency Department for vomiting, left low back pain, and diffuse abdominal pain with irradiation to the hypogastric area, associated with urinary symptoms. Abdomen was tender in the right iliac fossa and hypogastric area. Analytical study revealed increased inflammatory parameters, urinary test strip was negative, and pelvic computed tomography showed a cystic lesion of ovarian origin. Due to suspicion of cyst torsion, laparoscopic surgery was performed, revealing a necrotic adnexal torsion requiring adnexectomy.
Discussion: Although often suspected, adnexal torsion is rarely confirmed. Although ovarian torsion accounts for a small number of all gynecological emergencies, it represents a common diagnostic challenge in the emergency setting.
Conclusion: As ovary viability depends on early diagnosis, a high index of suspicion is required. This clinical case raises awareness of this entity in the differential diagnosis of lower abdominal pain in female children and adolescents.info:eu-repo/semantics/publishedVersio
Lesão idiopática do nervo espinhal acessório
Get PDFPalsy of the eleventh cranial nerve – or spinal accessory nerve (SAN) − is a rare cause of scapular winging, leading to painful upper extremity disability due to weakness and atrophy of the trapezius muscle. Most SAN injuries are iatrogenic, and no specific pediatric epidemiology is known.
Herein is described the case of a 17-year-old adolescent referred to Physical and Rehabilitation Medicine consultation due to insidious right shoulder pain with two years of evolution.
Shoulder pain combined with muscular atrophy is suggestive of nerve lesion. Electromyography is the gold standard exam and showed segmental demyelination and axonotmesis in this case. After evaluation, the patient underwent physiotherapy, with excellent results.
In conclusion, SAN injury treatment can be conservative or surgical and physiotherapy is the basis of early treatment in most cases. Recovery can occur even after a significant period of time.info:eu-repo/semantics/publishedVersio
Exposição ao ecrã em crianças até aos cinco anos de idade
Get PDFIntroduction: While the limited use of high-quality and appropriate media may have a positive influence, excessive exposure carries health risks for young children and their families. Research suggests that increased screen time in young children is linked to negative health outcomes, including obesity, decreased cognitive and language development and reduced academic success. In this study we aimed to characterize the screen-time habits in a healthy population of children, aged between six months and five years, of two Family Healthcare Units of an urban area in northern Portugal, and to review the current literature on children’s screen time and health-related issues.
Methods: This is a cross-sectional, observational and analytic study. We selected a convenience sample of children aged between six months and five years who were assessed at a scheduled surveillance visit and a questionnaire was applied to the caregivers between February and July 2018.
Results: One hundred sixty-six children were included. The mean age was 30 months; 53% were males. Television dominated total screen time. About 85% of children under two years-old and 80% of infants six to 12-months-old were exposed to screens daily, with 79% of them spending up to one hour per day in front of screens. The majority of parents of children aged two years and older were present and set limits on their children’s screen use. Overall, only 39% of parents affirmed to be aware of current guidelines for screen time. In our study, children’s screen time habits were not related with parents’ socioeconomic or academic status. Parents’ knowledge about current guidelines also did not minimize children’s screen use (p=0,094).
Discussion/Conclusion: Young children are exceeding screen time recommendations. Given that parents play a key role in the development of their children’s behaviors and that there is no evidence to support introducing screens at an early age, interventions to reduce children’s screen-time in the current media environment are needed.info:eu-repo/semantics/publishedVersio
Vólvulo intestinal idiopático e choque no lactente
Get PDFIntroduction: Intestinal volvulus is a surgical emergency in which a segment of the intestine twists over its mesenteric attachment, causing bowel obstruction. It usually presents with bilious vomiting and can progress to bowel necrosis and shock.
Case Report: A 40-days-old male infant presented with acute onset irritability, bilious vomiting, abdominal distention, and hematochezia. He rapidly evolved to shock with metabolic acidosis and coagulopathy, requiring fluid resuscitation, vasoactive agents, and invasive mechanical ventilation. The patient was submitted to urgent laparotomy, confirming midgut volvulus without malrotation. Partial reperfusion of the affected midgut was achieved, with no resection initially performed, but 48 hours later he was re-evaluated and partial enterectomy for midgut necrosis was performed. Despite the condition´s severity, the patient had a good evolution with full recovery.
Discussion: Bilious vomiting in the infant is highly suggestive of intestinal obstruction. The authors emphasize the presence of midgut volvulus without malrotation, complicated with bowel necrosis and shock.info:eu-repo/semantics/publishedVersio
Clinical effectiveness of Enneking appropriate versus Enneking inappropriate procedure in patients with primary osteosarcoma of the spine: a systematic review with meta-analysis
Get PDFPurpose: Primary osteosarcoma of the spine is a rare osseous tumour. En bloc resection, in contrast to intralesional resection, is the only procedure able to provide Enneking appropriate (EA) margins, which has improved local control and survival of patients with primary osteosarcoma of the spine. The objective of this study is to compare the risk of local recurrence, metastases development and survival in patients with primary osteosarcoma of the spine submitted to Enneking appropriate (EA) and Enneking inappropriate (EI) procedures.
Methods: A systematic search was performed on EBSCO, PubMed and Web of Science, between 1966 and 2018, to identify studies evaluating patients submitted to resection of primary osteosarcoma of the spine. Two reviewers independently assessed all reports. The outcomes were local recurrence, metastases development and survival at 12, 24 and 60 months.
Results: Five studies (108 patients) were included for systematic review. These studies support the conclusion that EA procedure has a lower local recurrence rate (RR 0.33, 95% CI 0.17-0.66), a lower metastases development rate (RR 0.39, 95% CI 0.17-0.89) and a higher survival rate at 24 months (RR 1.78, 95% CI 1.24-2.55) and 60 months (RR 1.97, 95% CI 1.14-3.42) of follow-up; however, at 12 months, there is a non-significant difference.
Conclusions: EA procedure increases the ratio of remission and survival after 24 months of follow-up. Multidisciplinary oncologic groups should weigh the morbidity of an en bloc resection, knowing that in the first year the probability of survival is the same for EA and EI procedures. These slides can be retrieved under Electronic Supplementary Material.info:eu-repo/semantics/publishedVersio