Browsing by Issue Date, starting with "2025-02"
Now showing 1 - 4 of 4
Results Per Page
Sort Options
- Targeted Disease-Specific Therapy for Patients with Hereditary Transthyretin Amyloidosis and Cardiac Involvement After Orthotopic Liver Transplantation. Consensus from the Working Group on Myocardial and Pericardial Diseases of the Portuguese Society of Cardiology and National Reference Centers for Familial Amyloidosis.Publication . Aguiar Rosa, Sílvia; Ferreira, Catarina; Conceição, Isabel; Coelho, Teresa; Marques, Nuno; Azevedo, Olga; Elsevier España
- Abdominal Hypoperfusion and Acute Kidney Injury in the Critically Ill Patient with Liver Cirrhosis: A Prospective Cohort Study.Publication . Pereira, Rui; Lopes, Diogo; Brandão Machado, Sara; Val-Flores, Luís; Caeiro, Fernando; Perdigoto, Rui; Marcelino, Paulo; Saliba, FaouziReduced abdominal perfusion pressure (APP) is an underdiagnosed potential pathophysiological mechanism for acute kidney injury (AKI) in the patient with liver cirrhosis and ascites. This study aimed to analyze the prevalence of abdominal hypoperfusion (AhP) (APP <60 mm Hg) and the impact of APP on AKI in critically ill patients with liver cirrhosis. This was a post hoc analysis from a prospective cohort study set in a general ICU at a tertiary university hospital. Patients were recruited between October 2016 and December 2021. Acute renal failure (ARF) was defined by stage 3 AKI according to the International Club of Ascites. Fifty-eight patients where included, with a mean age of 57 (±8.4) years, 79% were male, and 93% had acute-on-chronic liver failure at admission. The prevalence of AhP reached 75%, and 29% of cases had persisting AhP during the first week of ICU stay. Patients with baseline AhP had a higher 28-day mortality compared to those without AhP (respectively, 76% vs. 49%, = 0.03). Acute renal failure developed in 48% of patients. Higher serum urea (aOR: 1.01, 95% CI: 1.00-1.02, = 0.04) and white blood cell count (aOR: 1.1, 95% CI: 1.01-1.2, = 0.02) at ICU admission, as well as low persisting APP (aOR: 0.9, 95% CI: 0.86-0.98, = 0.02) were independent risk factors for ARF. Critically ill patients with liver cirrhosis presented a high prevalence of ARF, independently associated with higher baseline serum urea and WBC, and lower persisting APP. A structured clinical approach to optimize APP may reduce renal dysfunction in high-risk patients with cirrhosis.
- Spectrum of Cutaneous Lesions in a Cohort of Patients With Neurofibromatosis Type 2.Publication . Fialho, Maria C; Garrido, Pedro M; Santos-Coelho, Miguel; Ferreirinha, Ana; Martins, Bárbara D; Passos, João; Moura, CecíliaBackground: Neurofibromatosis type 2 (NF2) is a rare autosomal dominant syndrome with a predisposition to the development of central nervous system tumors, ophthalmic manifestations, and dermatological lesions. The latter are present in 70-95% of patients and can precede the evolution of other tumors. However, they are not included in the diagnostic criteria and are frequently undervalued during follow-up. Methods: An observational cross-sectional study characterizing cutaneous lesions in a cohort of NF2 patients was carried out. Dermatological examinations were performed, and lesions were classified into neural cutaneous tumors (superficial, SNCT, and deep, DNCT), hyperpigmented patches (HyperP), and hypopigmented patches (HypoP). The Dermatology Life Quality Index (DLQI) and EQ-5D questionnaires were applied to evaluate the impact on quality of life. Results: Nineteen patients with a mean age of 36 years were included. Sixteen (84%) patients had cutaneous lesions, mostly developed 10 or more years before the diagnosis. SNCT, DNCT, and HyperP showed similar frequencies (58%). HypoP were observed in only one patient. HyperP developed, on average, earlier than NCT (9.6 vs. 16.5 SNCT, 17.0 DNCT; years). The excised lesions had different histological patterns, including neurofibromas, schwannomas, and a hybrid tumor. Most patients reported a low impact of cutaneous manifestations on the quality of life (DLQI 0 or 1). Conclusions: Cutaneous lesions are frequent in NF2 and may precede the diagnosis by several years. Their identification is important to establish the diagnosis earlier and potentially reduce morbidity and mortality.
- The Role of Nurses Caring for Children Diagnosed with Sickle Cell Anemia and Their Families in a Hospital Setting: A Rapid Review of the Recent Literature.Publication . Freitas, Eduarda; Loura, David; Inês, Mariana; Martins, Carla; Duarte, Inês: Sickle cell anemia (SCA) affects a significant number of children worldwide, for whom the progression of the disease can lead to functional disability-impaired development. Nurses are pivotal in providing holistic care to these children and their families. This review aims to identify recent evidence on the role of nurses in intervening with children with SCA and their families in a hospital setting. : A rapid review reported under the PRISMA methodology was carried out in the CINAHL, MEDLINE, and Scopus databases with the expression (sickle cell anemia OR sickle cell disease) AND (child* OR family OR pediatric*) AND (nurs* OR "nursing interventions" OR "pediatric nursing") AND (hospital*), considering studies between 2019 and 2024, written in English, identifying articles with insights about the role of nurses in this context. Articles other than primary or secondary studies were excluded. Data were analyzed through a rapid qualitative approach. : Fifty-two studies were identified and seventeen articles were included. The nurse's role is key and multidisciplinary, focusing on the child and family (care management and therapeutic education), the team (training, and the promotion of safety and quality of care), and the health system (optimizing access to care and promoting adequate resources for its implementation). Such a role is important for short-term clinical problems and to prevent long-term complications. : Nurses play a central role in empowering families and coordinating multidisciplinary care. Greater investment is needed at a clinical level, through a more effective response to the needs of these patients, and in research, through experimental studies and other designs focused on multidisciplinary interventions.