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A Retropharyngeal Phlegmon as a Presentation of Multisystem Inflammatory Syndrome in Children.
No full textRivaroxaban for the Treatment of Venous Thromboembolism in NPHS1 Congenital Nephrotic Syndrome
No full textIntroduction: Nephrotic syndrome (NS) is associated with a multifactorial hypercoagulable state. Congenital NS (CNS) exhibits a higher prevalence of thrombotic events compared to other types. Direct oral anticoagulants (DOAC) have been approved for paediatric acute venous thromboembolism.
Case Report: We present 2 CNS children treated with rivaroxaban for treatment (Case 1) and prophylaxis (Case 2) of thrombotic events. A 2-month-old male previously diagnosed with CNS with homozygous mutation in the NPHS1 gene, underwent central venous catheter (CVC) replacement during which multiple thrombi were seen. The patient developed clinical signs compatible with pulmonary embolism. Chest radiograph showed a peripheral condensation on the left hemithorax and CT-angiography was inconclusive for peripheral embolism. Despite therapeutic doses of enoxaparin, adjustments were difficult with persistently low anti-Xa levels. The switch to rivaroxaban was performed, and doses were regularly adjusted based on patient’s weight. No adverse or other thrombotic events were reported, despite maintaining CVC. As expected, chronic kidney disease progressed at 19 months and rivaroxaban was suspended. An 8-month-old female with CNS associated with an heterozygous mutations in the NHPS1 gene, underwent multiple CVC replacements due to recurrent obstruction despite heparinisation and alteplase administrations. Although there were no systemic thrombotic episodes, considering the high risk of thrombosis, prophylaxis with rivaroxaban was initiated with eGFR of 54 mL/min/1.73m2 (1-2 SD below expected eGFR). Weight-adjusted dose was prescribed. No severe adverse or thrombotic events reported until 19 months.
Conclusion: These cases suggest that the safety and efficacy profile of rivaroxaban may be encouraging for treating and preventing venous thromboembolism in CNS. However, additional studies are warranted to optimize DOAC use in children with complex conditions, such as CNS, allowing for more tailored management of anticoagulation in this high-risk population
Sinal de Hutchinson no Nevo Congénito da Matriz Ungueal
No full textCongenital nail matrix nevi (NMN) are a rare cause of melanonychia that may present with irregularity, asymmetry, and multicomponent pigmentation posing diagnostic challenges with subungual melanoma. We report a case of a 49-year-old female with longitudinal melanonychia and a 1-month recent pigmentation in the proximal nail fold. This sign is traditionally associated with malignancy, which further complicates the differentiation from acral melanoma. Therefore, a nail matrix biopsy was performed. Histopathologic examination revealed a nail matrix nevus. This procedure is crucial in suspicious cases, despite the risk of nail dystrophia. The evolution of these nevi into adulthood and their potential malignancy remains unclear, emphasizing the need for continued research and surveillance. This case highlights that congenital NMN often present with clinical and dermoscopic features of concern, mirroring those observed in subungual melanomaOs nevos congénitos da matriz ungueal são uma causa rara de melanoníquia que pode apresentar-se com irregularidade, assimetria e pigmentação multicomponente, o que dificulta o diagnóstico diferencial com melanoma subungueal. Descrevemos o caso de uma mulher de 49 anos com melanoníquia longitudinal que progrediu com pigmentação na prega ungueal proximal. O sinal de Hutchinson está frequentemente associado a malignidade, o que dificulta ainda mais a diferenciação com o melanoma acral. Realizou-se uma biópsia da matriz ungueal, cujo exame histopatológico foi compatível com nevo da matriz ungueal. Esta abordagem é crucial em casos suspeitos, apesar do risco de distrofia ungueal. A evolução destes nevos até à idade adulta e o seu potencial de malignidade permanecem pouco claros, enfatizando a necessidade de vigilância contínua. Este caso realça que os nevos congénitos da matriz ungueal se apresentam frequentemente com características clínicas e dermatoscópicas suspeitas, que podem mimetizar as observadas no melanoma subunguea
Impact of Infrasound Exposure and Streptozotocin-Induced Glucose Intolerance on Bone Composition in Wistar Rats.
No full textThe elemental composition of chemical elements can vary between healthy and diseased tissues, providing essential insights into metabolic processes in physiological and diseased states. This study aimed to evaluate the calcium (Ca) and phosphorus (P) levels in the bones of rats with/without streptozotocin-induced diabetes and/or exposure to infrasound. X-ray fluorescence spectroscopy was used to determine the concentrations of Ca and P in Wistar rat tibiae samples.The results showed a significant decrease in bone P concentration in streptozotocin-induced diabetic rats compared to untreated animals. Similarly, the Ca/P ratio was higher in the streptozotocin-induced diabetic group. No significant differences were observed in bone Ca concentration between the studied groups or between animals exposed and not exposed to infrasound.Moreover, streptozotocin-induced diabetic rats had lower bone P concentration but unaltered bone Ca concentration compared to untreated rats. Infrasound exposure did not impact bone Ca or P levels. The reduced bone P concentration may be associated with an increased risk of bone fractures in diabetes
Targeted Treatments for Myasthenia Gravis in Children and Adolescents.
No full textMyasthenia gravis (MG) is an antibody-mediated disorder of the neuromuscular junction affecting children and adults. MG is a treatable condition with most patients requiring immunosuppression for disease control and/or remission. Juvenile myasthenia gravis (JMG) is rare in comparison with adult-onset MG but given the same underlying pathophysiology, treatment strategies are similar to those in adults. Until recently, there were only a few randomised controlled trials (RCTs) for MG treatments in adults and none in children, and management strategies were primarily based on expert consensus. In addition, treatment options for refractory MG cases have been severely limited, resulting in poor long-term quality of life in such patients due to the significant disease burden. Recently, there have been several RCTs focussing on novel therapeutic strategies with potentially promising outcomes, suggesting a change in MG management over the coming years and access to more effective and faster-acting drugs for MG patients. This paper will review current and new MG treatments including efgartigimod, eculizumab, rozanolixizumab, ravulizumab, and zilucoplan, with a focus on juvenile myasthenia gravis
Inconsistency Between Pictures on Baby Diaper Packaging in Europe and Safe Infant Sleep Recommendations.
No full textObjective: To describe the level of inconsistency between pictures on baby diaper packaging and safe infant sleep recommendations (SISRs) in Europe.
Study design: We attempted to identify all packaging of baby diapers sold in 11 European countries for infants weighing less than 5 kg through internet searches from July 2022 through February 2023. For each type of package, we extracted whether there was a picture depicting a baby, whether the baby was sleeping, and whether the picture of the sleeping baby was inconsistent with ≥1 of 3 SISRs: (i) nonsupine sleeping position, (ii) soft objects or loose bedding, or (iii) sharing a sleep surface with another person. Data were aggregated at the country level, and a random-effects meta-analysis of proportions was used to obtain summary estimates. The outcome was the summary estimate of the proportion of pictures that were inconsistent with SISRs.
Results: We identified 631 baby diaper packaging types of which 49% (95% CI: 42-57; n = 311) displayed a picture of a sleeping baby. Among those 311 packages, 79% (95% CI 73-84) were inconsistent with ≥1 SISR, including a nonsupine sleeping position, 45% (95% CI 39-51), soft objects or loose bedding such as pillows or blankets, 51% (95% CI 46-57), and sharing a sleep surface with another person, 10% (95% CI 4-18).
Conclusions: Pictures on baby diaper packaging in Europe are often inconsistent with SISRs. The prevention of sudden unexpected death in infancy requires action from manufacturers and legislators to stop parents' exposure to misleading images that may lead to dangerous practices
Comorbid Obsessive-Compulsive Disorder and Bipolar Disorder in the Pediatric Population — Clinical and Therapeutic Challenges
No full textComorbidity between obsessive-compulsive disorder and bipolar disorder has been a well-documented phenomenon in the adult population for several decades. However, scant evidence exists regarding this comorbidity in the juvenile
population. The objective of this review is to investigate the impact of obsessive-compulsive disorder and bipolar
disorder comorbidity on the clinical course and therapeutic approaches for each disorder individually, with a particular
focus on children and adolescents. A scientific review was conducted on a careful analysis of the evidence available on
the electronic databases: MEDLINE, Embase and Cochrane Library. In children, it has been observed that up to one-third of individuals diagnosed with either obsessive-compulsive disorder or bipolar disorder also experience a lifetime
co-occurrence of the other disorder, a notably higher prevalence when compared to adults. The primary diagnosis
typically manifests with an earlier onset in this population. When bipolar disorder is present, children and adolescents
with obsessive-compulsive disorder tend to exhibit an episodic course, more hoarding or saving obsessions and
compulsions, and a significantly higher number of comorbidities. In this age group, obsessive-compulsive disorder and
bipolar disorder comorbidity is further linked to increased severity symptoms, greater impulsivity, reduced
responsiveness to pharmacological treatment, elevated suicide risk, and a diminished likelihood of achieving remission
rates for manic and depressive symptoms. Obsessive-compulsive symptoms during childhood and adolescence may
indicate vulnerability to have bipolar disorder, suggesting partially shared etiopathogenetic mechanisms between thesepsychiatric entities. The use of antidepressants poses a risk of inducing a switch to mania or rapid cycling in bipolar patients. Additionally, atypical antipsychotics have been reported to trigger and worsen obsessive-compulsive
symptoms. The complexity involved in deciding on a suitable treatment scheme for individuals with this comorbidity
may contribute to an unfavorable clinical course. A noteworthy portion of comorbid patients may require a combination of multiple mood stabilizers for effective management
The European Portuguese Version of the Brief Negative Symptom Scale
No full textNegative symptoms reflect a currently much-untreated loss of normal functioning and are frequently found in psychotic disorders. We present the first translation of the Brief Negative Symptom Scale (BNSS) to European Portuguese and evaluate its validity in a sample of Portuguese male patients with a psychotic spectrum disorder. The Portuguese BNSS showed excellent internal consistency, high convergent validity (i.e., strong correlation with the PANSS negative factor), and high discriminant validity (i.e., a lack of association with the PANSS positive factor). In sum, the present European Portuguese BNSS has shown to be reliable, thus extending this instrument's clinical availability worldwide.info:eu-repo/semantics/publishedVersio
Ganhos da Interdisciplinaridade em Saúde. Estudo de Caso
No full textIntrodução: A Insuficiência Cardíaca (IC) é causada por uma anomalia cardíaca, com sinais típicos, recomendando-se uma abordagem interdisciplinar (McDonagh et al., 2021). Um adequado planeamento de alta tem impacto na eficiência e satisfação do doente (Escoval et al., 2010).
Objetivos: Evidenciar os ganhos da interdisciplinaridade nos cuidados à pessoa com IC.
Material e métodos: Clinicamente, destaca-se miocardiopatia dilatada com IC, NYHA III-IV. Antecedentes: IC com 6 internamentos/ano, insuficiência respiratória global (sob Oxigenioterapia longa duração 1L/m). Na admissão, exame objetivo: dispneia e dificuldade em articular frases, perfil hipertenso, taquicardico, SpO297% sob 3L/m. À auscultação: tons arrítmicos, taquicardíacos, Murmúrios Vesiculares diminuídos nas bases, fervores bibasais. Membros inferiores com edema. Radiografia tórax com aumento do Índice Cardiotorácico e estase nos campos inferiores. Apresentava: 76,8Kg, cansaço a mínimos esforços, dependência elevada nas Atividades Vida diária (AVD), dificuldade na terapêutica. Identificaram-se diagnósticos enfermagem: Dispneia Presente, Posicionar-se/Vestuário dependente grau elevado, Alimentar-se/Higiene/Sanitário/Movimento muscular dependente grau moderado, Gestão do Regime terapêutico comprometida.
O doente vive com esposa. Tem 2 filhas, sem disponibilidade para apoio presencial. Reconhecem-se indicadores de risco social: Problema social (Escala Gijón=15), Dependência funcional, desconhecimento dos recursos da comunidade/direitos, iliteracia em saúde e incapacidade familiar de apoio.
Admitiu-se o doente no “Plano Integrado de Alta Hospitalar”, informando-se a Unidade de Saúde sobre o internamento. Cumpriu levosimendan. e furosemida. Fez-se desmame de O2 até ao basal. Procedeu-se ao treino de estratégias de conservação de energia, posições de
descanso e AVD. Realizou-se exercício aeróbio e desmitificou-se crenças sobre terapêutica. Orientou-se para direitos sociais. Agendou-se conferência familiar.
Resultados: Verificou-se que o incumprimento desencadeou a descompensação (dosagens prescritas e administradas diferentes), devido a inadequação da comunicação e por falta de apoio para detetar erros na administração.
Definiu-se como plano com a pessoa e família: apoio domiciliário (AVD e medicação) e envolvimento familiar. Enviou-se informação à equipa da Unidade de Saúde que, pós-alta, constatou adesão ao regime terapêutico. O doente foi reavaliado em consulta: maior autonomia e tolerância ao esforço, com consequente ganho em saude e eficácia organizacional.
Conclusões: A abordagem interdisciplinar ao doente com IC é impreterível para se evitarem hospitalizações (McDonagh et al., 2021). A aplicação de uma alta integrada em doentes de alto risco clínico/social melhora o planeamento dos cuidados, assegurando a sua continuidade e segurança de cuidados.info:eu-repo/semantics/publishedVersio
Prognosticating the Outcome of Intensive Care in Older Patients - a Narrative Review
No full textPrognosis determines major decisions regarding treatment for critically ill patients. Statistical models have been developed to predict the probability of survival and other outcomes of intensive care. Although they were trained on the characteristics of large patient cohorts, they often do not represent very old patients (age ≥ 80 years) appropriately. Moreover, the heterogeneity within this particular group impairs the utility of statistical predictions for informing decision-making in very old individuals. In addition to these methodological problems, the diversity of cultural attitudes, available resources as well as variations of legal and professional norms limit the generalisability of prediction models, especially in patients with complex multi-morbidity and pre-existing functional impairments. Thus, current approaches to prognosticating outcomes in very old patients are imperfect and can generate substantial uncertainty about optimal trajectories of critical care in the individual. This article presents the state of the art and new approaches to predicting outcomes of intensive care for these patients. Special emphasis has been given to the integration of predictions into the decision-making for individual patients. This requires quantification of prognostic uncertainty and a careful alignment of decisions with the preferences of patients, who might prioritise functional outcomes over survival. Since the performance of outcome predictions for the individual patient may improve over time, time-limited trials in intensive care may be an appropriate way to increase the confidence in decisions about life-sustaining treatment.info:eu-repo/semantics/publishedVersio