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- Cold agglutinin syndrome in a patient with metastatic breast cancer: a Case reportPublication . Bandarra, Daniel; Rochate, Dina; Gosalbez, Beatriz; Ferreira, José; Cunha, Nidia Maltez; Carvalhal, SaraBackground: Cold agglutinin syndrome (CAS) is a form of autoimmune hemolytic anemia (AIHA), most often associated with lymphoproliferative disorders or infections. Its occurrence in breast cancer is rare and may be triggered by systemic treatment. Case presentation: We report the case of a woman in their fifties diagnosed with breast cancer in 2019. She underwent surgery followed by adjuvant chemotherapy and radiotherapy and subsequently received 3 years of endocrine therapy before developing bone and hepatic metastases. First-line treatment with ribociclib plus letrozole achieved partial response, and fulvestrant was administered at progression. Following further progression, paclitaxel was introduced as third-line metastatic therapy. After four weekly administrations, the patient was admitted to our hospital with severe anemia and diagnosed with CAS. Prompt management and a multidisciplinary approach resulted in partial hematological recovery. Nevertheless, paclitaxel was permanently discontinued, and subsequent therapies provided only transient benefit. The disease continued to progress, her performance status declined, and she ultimately transitioned to exclusive palliative care until death. Conclusion: This case illustrates a rare and severe immune complication of paclitaxel in metastatic breast cancer. The emergence of CAS not only limited systemic options but also reshaped the therapeutic trajectory, highlighting the need for close monitoring during cancer treatments. Early recognition, multidisciplinary approach, and prompt management can provide some improvement, although overall prognosis remains determined by the underlying malignancy.
- Description of the Hamburg Alexander Leukodystrophy Cohort—Insights into Practical Classification and the Care SituationPublication . Kokaly, Nadia; Guerreiro, Helena; Bredow, Janna; Dreha-Kulaczewski, Steffi; Ohlenbusch, Andreas; Köhler, Wolfgang; Reinhardt, Tabea; Schön, Gerhard; Volk, Alexander E.; Sigel, Helen; Bley, AnnetteBackground: Alexander disease (AxD) is a rare severe leukodystrophy that has no cure to date. A pathogenic gain-of-function variant in the GFAP gene affects the astrocytes and subsequently the function of the white matter in the CNS. Methods: We retrospectively analyzed the most frequent symptoms of nine AxD cases, classified them according to published classifications, and described the need of care and support. Results: The description of the courses of disease of nine cases with AxD reflects the broad spectrum of different phenotypes of AxD, with often occurring apnoea. Data about care and support for AxD patients indicate a high and heterogeneous need of support. Treatment with steroids reduced symptoms in two patients. Some patients showed lasting improvement during their course of disease. Conclusions: The course of AxD is very heterogeneous. Thus, we extracted relevant key features to describe the severity of the disease, namely feeding problems, epilepsy, age-appropriate motor function, failure to thrive, age-appropriate language and apnoea. We recommend early evaluation for clinical care and support. For some AxD patients, treatment with steroids may alleviate symptoms. Further development of efficient treatments is necessary
- Frailty and outcomes in elderly ICU patients: insights from a portuguese cohortPublication . Lourenço, Eva; Rodrigues, Isabel; Sampaio, Mário; da Costa, EmiliaBackground: Frailty is a key determinant of outcomes in critically ill elderly patients, but data from Portugal remain limited. To our knowledge, this is the first study to examine the prevalence and prognostic impact of frailty among elderly ICU patients in a Portuguese hospital setting. Objective: To determine the prevalence of frailty among elderly patients admitted to an intensive care unit (ICU) in southern Portugal and to examine its crude associations with illness severity, organ support, and mortality outcomes. Methods: We conducted a retrospective cohort study including 125 patients aged ≥ 65 years admitted to the polyvalent ICU of Hospital de Faro over the last six months of 2024. Data included demographics, comorbidities, Charlson Comorbidity Index (CCI), severity scores (SOFA, SAPS II, APACHE II), and frailty status assessed by the Clinical Frailty Scale (CFS). Outcomes were the need for organ support, ICU and hospital mortality, and length of stay. Results: Frailty (CFS ≥ 5) was identified in 30.4% of patients. Frail patients were older, had higher comorbidity burden (CCI), and presented with significantly higher severity scores at admission. They also required more invasive support, including vasopressors and invasive mechanical ventilation, while acute kidney injury (AKI) requiring renal replacement therapy (RRT) was similar between groups. ICU mortality was significantly higher among frail patients (50.0% vs. 31.0%), as was hospital mortality (76.3% vs. 33.3%). Length of ICU stay did not differ, although frail patients tended to have longer hospitalizations overall. Conclusions: Frailty was highly prevalent and strongly associated with increased severity, greater need for organ support, and higher mortality. Routine frailty assessment at ICU admission may enhance prognostic accuracy and support patient-centered decision-making.
- P0397 Soluble transferrin receptor as a reliable inflammation-independent marker of iron deficiency in crohn’s disease and ulcerative colitisPublication . Portela, F.; Santos, M. P. Ministro dos; Sousa, Helena Tavares; Roseira, Joana; Fernandes, S. R.; Crespo, R.; Domingues, B.; Santiago, M.; Miranda, R.; Dias, S.; Dias, C. C.; Magro, F.Background: Iron deficiency is a common complication in inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn’s disease (CD).1 However, standard iron markers are influenced by inflammation, complicating the diagnosis of true iron deficiency.1,2 Soluble transferrin receptor (sTfR) has been proposed as a more reliable, inflammation-independent marker of iron demand.3 This study aimed to assess the utility of sTfR in identifying iron deficiency without anaemia (IDWA). Methods: The ID_IBD study was a multicentre, cross-sectional study. Iron status was classified using two approaches: the ECCO consensus definition, focusing on ferritin thresholds adjusted for inflammatory markers (C-reactive protein [CRP] and faecal calprotectin [FCAL]), and a stricter definition that adds transferrin saturation to the ECCO criteria. sTfR levels were compared across groups, and ROC curve analysis was used to identify optimal diagnostic cut-offs. Results: This analysis included 411 IBD patients (130 UC, 281CD) and 178 controls. sTfR showed no correlation with CRP or FCAL. In UC, patients with IDWA had significantly higher sTfR levels (median 1.20mg/L, IQR 1.02-1.42) compared to non-IDWA patients (median 1.05mg/L, IQR 0.92-1.22; p=0.013). Anaemic UC patients also showed elevated sTfR levels (median 1.27mg/L, IQR 1.14-1.59) compared to non-IDWA individuals (patients but was significantly higher in anaemic patients (p=0.003). Conclusion: sTfR appears to be an inflammation-independent marker of iron status in IBD. It showed potential for identifying IDWA in UC, while in CD it mainly reflected increased iron demand in anaemia. Further longitudinal studies are warranted to validate its role and assess its clinical utility in IBD.
- Sarcopenia in women with anorectal dysfunctions—a female sarcopelvic studyPublication . Vieira, Ana Margarida Duarte da Silva; Pais, Sandra; Martins, Viviana; Castelo, Barbara; Saraiva, Miguel MascarenhasAnorectal dysfunctions (ARDs) include fecal incontinence (FI) and functional defecation disorders (FDDs). The pelvic floor muscles play a central role in the physiology of continence and defecation. We aimed to investigate the prevalence of sarcopenia in a female group with anorectal dysfunctions and compare them with a healthy female age-matched group. As secondary objectives, the relationship between anorectal dysfunction outcomes and sarcopenia was analyzed. Methods: We conducted a single-center cross-sectional, interventional, controlled, and double-blind study involving female adults admitted to an ARD outpatient clinic assessed for FI and/or FDD. A control group was also included of age-matched women without ARD. Sarcopenia was evaluated in the entire cohort, according to the latest criteria. Statistical analysis was performed using SPSS software v.29, considering a confidence interval of 95%. Results: A total of 130 participants were included, equally divided by the two groups. The median age was 64 years. Both groups were also similar regarding body mass index (BMI), physical activity index values, and dietary patterns. Among the 130 investigated women, there were no cases of confirmed sarcopenia or severe sarcopenia, but 15 women (11.5%) had probable sarcopenia or dynapenia. The case group had significantly more probable sarcopenia than women in the control group (14 (21.5%) vs. 1 (1.5%), p < 0.001). The presence of relevant comorbidities, such as irritable bowel syndrome (IBS), urinary incontinence (UI), and meat dietary pattern (MDP), was a risk factor for probable sarcopenia. The binomial logistic regression analysis showed that probable sarcopenia (OR 3.9; CI 1.1–14.1, p = 0.039) was associated with a worse treatment response. Conclusions: Probable sarcopenia or dynapenia was significantly more prevalent in women with ARD and was a predictive factor of a worse treatment response, regardless of the ARD severity. Concomitant UI, MDP, IBS, and psychiatric conditions were significantly associated with dynapenia. The inclusion of the evaluation of sarcopenia in these patients should be considered.