Autoimmune lymphoproliferative syndrome (ALPS) is characterised by massive enlargement of the lymphoid organs, autoimmune cytopenias and a predisposition to develop lymphoid malignancies. The basic defect is a disturbance of the lymphocyte apoptosis, and a high number of circulating TCRab CD3+CD4−CD8− T-cells (double-negative T cells (DNT cells)). We describe a case of a 41-year-old man with fever, hepatosplenomegaly, multiple lymphadenopathy, autoimmune haemolytic anaemia and severe thrombocytopenia. Peripheral blood immunophenotyping revealed elevation of the characteristic DNT cells in 8% and high levels of interleukin 10. Histopathological analysis of lymph nodes showed lymphadenitis with paracortical hyperplasia. It was assumed as a probable diagnosis of ALPS, and the procedure was to medicate the patient with steroids. As a result, a significant clinical improvement was achieved, and he has been in remission for 2 years. To our knowledge, this is the first case reported in a Portuguese adult patient.
A fratura do fémur está associada a importante morbimortalidade, sendo que menos de metade dos doentes regressam ao seu nível prévio de autonomia. Uma abordagem multidisciplinar permite uma reabilitação funcional imediata, bem como melhor gestão de comorbilidades, prevenção de fraturas secundárias e das quedas. Objetivos: apresentação da casuís-tica de 3 meses de uma unidade de Ortogeriatria. Material e métodos: Descreve-se a demografia dos doentes admitidos, grau de dependência funcional, comorbilidades, intercorrências, tempo de internamento, mortalidade e destino à data de alta. O ganho de funcionalidade no internamento foi também avaliado. Resultados: De outubro/2015 a janeiro/2016 foram admitidos 66 doentes após cirurgia de fratura proximal do fémur. Média de idades foi 82,94±6,93 anos; 86% eram mulheres. O tempo de internamento foi de 7,8±4,697 dias, sendo o tempo médio para realizar cirurgia de 3±3,374 dias. Quanto ao grau de funcionalidade prévio 47% eram Katz A; à admissão 85% tinham mRankin ≤3. Identificaram-se vá-rias comorbilidades em 89% dos doentes; nas intercorrências, a mais frequente foi a anemia (53%). Houve um ganho na funcionalidade relativamente à admissão na unidade de 75,8% e em relação ao estado funcional anterior ao evento de 19,7%. Setenta e um porcento dos doentes tiveram alta para o domicílio; a taxa de mortalidade foi de 1,52%. Conclusões: A unidade demonstrou eficiência no controlo das comorbilidades, reduzidas complicações e baixa mortalidade. Registou-se um ganho funcional comparando com a admissão em 3 em cada 4 doentes e em relação ao estado basal prévio em 1 em cada 5 doentes. Palavras-chave: fratura fémur, ortogeriatria, geriatria, reabilitação INTRODUÇÃOA fratura do colo do fémur é frequente nos indivíduos idosos, sendo uma das complicações mais graves da osteoporose.1 É mais comum em mulheres, estimando-se que uma em cada três mulheres com mais de 80 anos irá sofrer uma fratura do colo do fémur ao longo da vida 2 . Estão geralmente associadas a traumatismos de baixa frequência e o tratamento de primeira linha é cirúrgico. 1,3Estão descritas taxa de mortalidade variáveis em fase aguda (5 a 10%) 1,4 . Ao fim de um ano estima-se que 10 a 20% destes doentes possam falecer, metade apresenta alguma perda funcional e apenas 30% dos doentes tenham recuperado a sua capacidade funcional para os níveis prévios aos da ocorrência da fratura 3 No sentido de otimizar o tratamento e reabilitação dos doentes com fratura do fémur foram criadas unidades de Ortogeriatria. Estas unidades funcionais permitem uma abordagem multidisciplinar orientada para a reabilitação funcional imediata, bem como para a gestão de comorbilidades, prevenção de fraturas secundárias e das quedas ABSTRACTHip fracture is associated with important morbimortality; less than half of the patients go back to the same functional degree. A multidisciplinary approach can enable immediate functional rehabilitation, better treatment of comorbidities and prevention of secondary fractures and falls. Objectives: to describe the ca...
Introduction: Orthogeriatrics is the subspecialty of geriatrics that is dedicated to the care of elderly patients with fragility fractures. The Orthogeriatrics Unit of the Vila Nova de Gaia Hospital Centre was the first unit created in Portugal in October 2015, in a co-management model.Methods: Patients older than 65 years and with femur fractures were admitted to the unit after surgery. The department was run by internists with differentiation in geriatrics, and multidisciplinary support from orthopaedics, physiatrists, physiotherapists, nutritionists, and social workers, as well as rehabilitation nursing. A comprehensive multidisciplinary assessment was performed upon admission, including comprehensive geriatric assessment as well as postoperative monitoring of complications, investigation of fall mechanisms, functional rehabilitation, and outpatient orientation. Analysed variables included demographics, comorbidities, prior level of functionality, delay of orthopaedic surgery, complications, time of hospitalization, functional prognosis, and destination after discharge. Follow-up was maintained to assess short-and medium-term mortality. Kaplan-Meier curves and Cox regression were used for the statistical analysis of mortality.Results: In four years of activity with 444 admissions, the typical patients were women (80.7%), with an average age of 84 years, coming from home (92%) after an accidental fall resulting in a proximal femur fracture. About half (54%) were previously autonomous, but with a high index of comorbidities (mean Charlson Index of 4.85), the most relevant of which were arterial hypertension (71%), malnutrition (46%), heart failure (35%), hyperlipidaemia (34%), osteoporosis (32%), and dementia (16%). During hospitalization, most patients had medical complications (86.3%), the most frequent ones being anaemia (45%), infections (35%), namely, urinary, respiratory, and surgical wound infections, acute heart failure (15%), and acute kidney injury (11%). Prevalent geriatric syndromes were also identified and corrected through protocols for delirium, urinary incontinence, pressure ulcers, and constipation. The mean length of stay was 12.49 days. At discharge, 75% presented a modified Rankin Scale score lower than 3 and 73% of patients were able to return home, with a low referral rate to long-term care facilities (5.9%). The in-hospital mortality rate was 2.65%. It was possible to maintain follow-up protocol after discharge in 343 patients, and the mortality at 12 months was 19.23% and at three years, it was 25.52%, with a risk of death almost doubled for patients discharged with a high degree of dependence (modified Rankin Scale score ≥ 3; OR: 2.19; p < 0.001). Conclusion:We demonstrated reduced in-hospital mortality despite an elderly, frail population, with multiple previous comorbidities and a high number of inpatient intercurrences evidencing the importance of a good in-hospital co-management between internal medicine and orthopaedics, demonstrating the benefit of orthogeriatric units in patients ...
Little is known about the relationship between the opinions of Background: inpatients and the degree to which hospitals are improving in performance over time. The aim of this study was to determine the personal assessment level of inpatients or their representatives regarding aspects of health care in an internal medicine ward. We carried out a questionnaire in September 2011 with 284 Methods: discharged patients and patient representatives, focusing on their opinions about the department, health professionals and amenities, with response options ranging from 1 (very bad) to 5 (very good). The relationships between domains from the questionnaire and socio-demographic factors were examined using a t-test and one-way ANOVA. The response rate was 78%. The patients showed a slightly Results: higher mean score (m) for factors in the medical care domain than did the patient representatives (m = 4.51 vs. m = 4.27; p = 0.014). The mean score of all the items in all domains was 4.24; this allowed us to determine the difference from the overall mean (DIFM) for medical care (DIFM = 0.18; p = 0.000), foods (DIFM =-0.31; p = 0.000), diagnostic tests (DIFM =-0.15; p = 0.036) and transport (DIFM =-0.41; p = 0.000). Respondents with a medium or higher educational level gave lower scores to the domains food (m = 3.74; p = 0.004), diagnostic tests (m = 3.72; p = 0.04) and transport (m = 3.62; p = 0.025) than those with lower educational levels. The domains facilities (m = 2.4; p = 0.04) and diagnostic tests (m = 3.63; p = 0.009) were given lower scores by those aged <50 years compared with older respondents. Our findings suggest that the evaluation of the responders Conclusions: will allow the hospital management to make improvements in the quality of care.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.