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1.
J Inherit Metab Dis ; 2024 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-38356271

RESUMO

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most prevalent mitochondrial fatty acid ß-oxidation disorder. In this study, we assessed the variability of the lipid profile in MCADD by analysing plasma samples obtained from 25 children with metabolically controlled MCADD (following a normal diet with frequent feeding and under l-carnitine supplementation) and 21 paediatric control subjects (CT). Gas chromatography-mass spectrometry was employed for the analysis of esterified fatty acids, while high-resolution C18-liquid chromatography-mass spectrometry was used to analyse lipid species. We identified a total of 251 lipid species belonging to 15 distinct lipid classes. Principal component analysis revealed a clear distinction between the MCADD and CT groups. Univariate analysis demonstrated that 126 lipid species exhibited significant differences between the two groups. The lipid species that displayed the most pronounced variations included triacylglycerols and phosphatidylcholines containing saturated and monounsaturated fatty acids, specifically C14:0 and C16:0, which were found to be more abundant in MCADD. The observed changes in the plasma lipidome of children with non-decompensated MCADD suggest an underlying alteration in lipid metabolism. Therefore, longitudinal monitoring and further in-depth investigations are warranted to better understand whether such alterations are specific to MCADD children and their potential long-term impacts.

3.
Acta Med Port ; 36(11): 753-764, 2023 Nov 02.
Artigo em Inglês | MEDLINE | ID: mdl-37924314

RESUMO

Acute porphyrias are a group of rare genetic metabolic disorders, caused by a defect in one of the enzymes involved in the heme biosynthesis, which results in an abnormally high accumulation of toxic intermediates. Acute porphyrias are characterized by potentially life-threatening attacks and, for some patients, by chronic manifestations that negatively impact daily functioning and quality of life. Clinical manifestations include a nonspecific set of gastrointestinal, neuropsychiatric, and/or cutaneous symptoms. Effective diagnostic methods are widely available, but due to their clinical heterogeneity and non-specificity, many years often elapse from symptom onset to diagnosis of acute porphyrias, delaying the treatment and increasing morbidity. Therefore, increased awareness of acute porphyrias among healthcare professionals is paramount to reducing disease burden. Treatment of acute porphyrias is centered on eliminating the potential precipitants, symptomatic treatment, and suppressing the hepatic heme pathway, through the administration of hemin or givosiran. Moreover, properly monitoring patients with acute porphyrias and their relatives is fundamental to preventing acute attacks, hospitalization, and long-term complications. Considering this, a multidisciplinary panel elaborated a consensus paper, aiming to provide guidance for an efficient and timely diagnosis of acute porphyrias, and evidence-based recommendations for treating and monitoring patients and their families in Portugal. To this end, all authors exhaustively reviewed and discussed the current scientific evidence on acute porphyrias available in the literature, between November 2022 and May 2023.


Assuntos
Porfiria Aguda Intermitente , Humanos , Porfiria Aguda Intermitente/diagnóstico , Porfiria Aguda Intermitente/genética , Porfiria Aguda Intermitente/terapia , Portugal , Consenso , Qualidade de Vida , Heme/metabolismo , Encaminhamento e Consulta
4.
Cureus ; 15(9): e45004, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37829986

RESUMO

Raynaud's phenomenon (RP) is a vasospastic disorder characterized by an exaggerated vasoconstrictive response to cold or emotional stress. It can be classified as primary (PRP) or secondary (SRP) depending on its association with an underlying condition. We present a case of a young female with severe RP, with trophic changes and abnormal capillaroscopy. After a detailed investigation, a diagnosis of secondary RP due to solvent exposure was made. The patient was treated with calcium channel blocker in low doses, due to hypotension, without improvement of symptoms. Vitamin C and pentoxifylline were added with an unsatisfactory response. Given the progressive worsening of RP and the appearance of trophic lesions, the patient's treatment was reviewed, and continuous intravenous iloprost infusion through an elastomeric pump was started. This resulted in significant symptom improvement and normalization on the capillaroscopic examination.

5.
BMJ Case Rep ; 16(4)2023 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-37045548

RESUMO

Sarcoidosis is a multisystemic disease, characterised by histopathologic presence of non-necrotising granulomas, primarily affecting the lungs (>90%). We present three cases predominantly characterised by uncommon extrapulmonary manifestations of sarcoidosis, where only one had pulmonary involvement. We describe three female patients, between the second and third decades of life, whose sarcoidosis was a diagnostic challenge due to their atypical extrapulmonary manifestations, from which we highlight: livedo reticularis and painful subcutaneous nodules with uncommon localisation, size and histology, being the first reported case of extensive subcutaneous nodules triggered by intramuscular penicillin; extensive symptomatic and refractory osseous involvement; and dispersed erythema nodosum affecting the entire body surface. All three patients required third-line treatment (antitumour necrosis factor agents) to achieve significant clinical and imagiological improvement. Through this case series, we highlight the importance of considering the rare and atypical presentations of sarcoidosis to avoid diagnostic delays and serious repercussions on the patient's prognosis.


Assuntos
Eritema Nodoso , Sarcoidose , Humanos , Feminino , Sarcoidose/complicações , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Granuloma/tratamento farmacológico , Granuloma/patologia , Pulmão/patologia , Prognóstico , Eritema Nodoso/diagnóstico , Eritema Nodoso/tratamento farmacológico , Eritema Nodoso/patologia
6.
Clin Exp Rheumatol ; 41(8): 1599-1604, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-36995314

RESUMO

OBJECTIVES: To examine the influence of contextual factors upon the evaluation of skin thickness and stiffness by ultrasound and to assess the reliability of these parameters. METHODS: Ultrasound dermal thickness (by B-mode, 18MHz) and skin stiffness (by shear-wave elastography, 9MHz) were assessed in persons with systemic sclerosis (SSc) and in healthy controls. The influence of contextual factors upon repeated measures was evaluated: (i) room temperature (16-17ºC vs. 22-24ºC); (ii) time of day (morning vs. afternoon), and (iii) menstrual cycle phase (menstrual vs. ovulatory). Differences were analysed using the related-samples Wilcoxon signed-rank test. Inter- and intra-rater reliability of ultrasound skin thickness and stiffness were evaluated in the 17 skin Rodnan sites of 20 persons with SSc and 20 healthy controls, under stable contextual conditions. RESULTS: A significant increase in ultrasound dermal thickness was observed at the leg in the afternoon vs morning, in both patients and controls. Similar observations were made for skin stiffness at the leg (in SSc) and at the foot (in SSc and controls) in the afternoon. No significant changes were observed in association with room temperature and menstrual cycle. Intra- and inter-rater-reliability was good to excellent for ultrasound dermal thickness and stiffness, both in SSc and healthy controls. CONCLUSIONS: The timing of the ultrasound procedure within each day seems to influence the ultrasound measures at the legs and feet. Our study corroborates that ultrasound dermal thickness and skin stiffness are reliable domains to quantify skin involvement in SSc.


Assuntos
Técnicas de Imagem por Elasticidade , Escleroderma Sistêmico , Feminino , Humanos , Reprodutibilidade dos Testes , Pele/diagnóstico por imagem , Escleroderma Sistêmico/diagnóstico por imagem , Ultrassonografia , Técnicas de Imagem por Elasticidade/métodos
8.
Cureus ; 15(12): e50827, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38249257

RESUMO

Upper extremity deep vein thrombosis (DVT) is an uncommon, under-reported, and difficult-to-diagnose condition. Although the strong provoking risk factors of venous thromboembolism are well described in the literature, the majority of cases are provoked by weak risk factors or are even considered unprovoked. In this case report, we describe a rare case of a brachial DVT in a woman in her 40s following implantable cardioverter-defibrillator (ICD) implantation. In her first evaluation, slight left arm edema and brachialgia were noted, and physiotherapy was prescribed. One month later, the patient was reevaluated because her complaints did not resolve, and an upper extremity venous ultrasound was done to exclude complications due to ICD implantation. The ultrasound identified an old DVT, which had been completely recanalized. The patient was then referred to a vascular surgery specialty consultation, which confirmed the diagnosis, and an anticoagulant was prescribed for three months. The symptoms resolved, and the patient did not report any more pain.

9.
Rev. bras. enferm ; 76(supl.1): e20220171, 2023. tab
Artigo em Inglês | LILACS-Express | LILACS, BDENF - Enfermagem | ID: biblio-1407484

RESUMO

ABSTRACT Objectives: to assess the level of anxiety of nurses in an emergency room in view of the new Coronavirus and describe the relationship between the degrees of anxiety and their sociodemographic variables. Methods: quantitative descriptive-correlational study with a sample of 60 nurses. A questionnaire was used as a data collection instrument based on the Hamilton Anxiety Assessment Scale. Results: the nurses' average anxiety is mild. A statistically significant relationship was found between anxiety and the variables "sex" and "children", with women having higher levels of anxiety than men, and nurses who do not have children showing mild, moderate, or severe anxiety. Conclusions: COVID-19 triggers anxiety in nurses, sometimes at pathological levels. Being female and not having children increase the anxiety experienced. Sex can be considered the determining factor for the level of anxiety experienced.


RESUMO Objetivos: avaliar o nível de ansiedade dos enfermeiros de um serviço de urgência em face do novo coronavírus e descrever a relação entre os níveis de ansiedade e as variáveis sociodemográficas. Métodos: estudo quantitativo descritivo-correlacional com uma amostra de 60 enfermeiros. Recorreu-se à aplicação de um questionário como instrumento de coleta de dados baseando-se na Escala de Avaliação de Ansiedade de Hamilton. Resultados: o escore médio de ansiedade dos enfermeiros é leve. Verificou-se que existe uma relação estatisticamente significante entre a ansiedade e a variável "sexo" e "filhos", com as mulheres apresentando níveis de ansiedade superiores aos homens e os enfermeiros sem filhos apresentando níveis de ansiedade leve, moderada ou severa. Conclusões: a COVID-19 desencadeia ansiedade nos enfermeiros, por vezes a níveis patológicos; e ser do sexo feminino e não ter filhos a aumenta. É possível afirmar que o sexo foi o fator determinante para o nível de ansiedade experimentado.


RESUMEN Objetivos: evaluar nivel de ansiedad de enfermeros de un servicio de urgencia frente al nuevo coronavirus y describir la relación entre niveles de ansiedad y variables sociodemográficas. Métodos: estudio cuantitativo descriptivo-correlacional con una muestra de 60 enfermeros. Recorrido a la aplicación de una encuesta como instrumento de recolecta de datos basándose en la Escala de Evaluación de Ansiedad de Hamilton. Resultados: el escore mediano de ansiedad de los enfermeros es leve. Verificado que hay una relación estadísticamente significante entre la ansiedad y la variable "sexo" e "hijos", con mujeres presentando niveles de ansiedad superiores a los hombres y los enfermeros sin hijos presentando niveles de ansiedad leve, moderada o severa. Conclusiones: la COVID-19 desencadena ansiedad en los enfermeros, por veces a niveles patológicos; y ser del sexo femenino y no tener hijos la aumenta. Es posible afirmar que el sexo fue el factor determinante para el nivel de ansiedad experimentado.

10.
Int J Mol Sci ; 23(22)2022 Nov 11.
Artigo em Inglês | MEDLINE | ID: mdl-36430419

RESUMO

Fatty acid oxidation disorders (FAODs) are inborn errors of metabolism (IEMs) caused by defects in the fatty acid (FA) mitochondrial ß-oxidation. The most common FAODs are characterized by the accumulation of medium-chain FAs and long-chain (3-hydroxy) FAs (and their carnitine derivatives), respectively. These deregulations are associated with lipotoxicity which affects several organs and potentially leads to life-threatening complications and comorbidities. Changes in the lipidome have been associated with several diseases, including some IEMs. In FAODs, the alteration of acylcarnitines (CARs) and FA profiles have been reported in patients and animal models, but changes in polar and neutral lipid profile are still scarcely studied. In this review, we present the main findings on FA and CAR profile changes associated with FAOD pathogenesis, their correlation with oxidative damage, and the consequent disturbance of mitochondrial homeostasis. Moreover, alterations in polar and neutral lipid classes and lipid species identified so far and their possible role in FAODs are discussed. We highlight the need of mass-spectrometry-based lipidomic studies to understand (epi)lipidome remodelling in FAODs, thus allowing to elucidate the pathophysiology and the identification of possible biomarkers for disease prognosis and an evaluation of therapeutic efficacy.


Assuntos
Erros Inatos do Metabolismo Lipídico , Doenças Mitocondriais , Doenças Musculares , Animais , Lipidômica , Doenças Musculares/tratamento farmacológico , Ácidos Graxos/metabolismo , Lipídeos/uso terapêutico
11.
Rev Bras Enferm ; 76Suppl 1(Suppl 1): e20220171, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36449963

RESUMO

OBJECTIVES: to assess the level of anxiety of nurses in an emergency room in view of the new Coronavirus and describe the relationship between the degrees of anxiety and their sociodemographic variables. METHODS: quantitative descriptive-correlational study with a sample of 60 nurses. A questionnaire was used as a data collection instrument based on the Hamilton Anxiety Assessment Scale. RESULTS: the nurses' average anxiety is mild. A statistically significant relationship was found between anxiety and the variables "sex" and "children", with women having higher levels of anxiety than men, and nurses who do not have children showing mild, moderate, or severe anxiety. CONCLUSIONS: COVID-19 triggers anxiety in nurses, sometimes at pathological levels. Being female and not having children increase the anxiety experienced. Sex can be considered the determining factor for the level of anxiety experienced.


Assuntos
COVID-19 , Enfermeiras e Enfermeiros , Masculino , Feminino , Humanos , COVID-19/epidemiologia , Pandemias , Serviço Hospitalar de Emergência , Ansiedade/epidemiologia , Ansiedade/etiologia
13.
Braga; s.n; Universidade do Minho; 20210000. Ilus.
Tese em Português | BDENF - Enfermagem | ID: biblio-1362254

RESUMO

Os enfermeiros do serviço de urgência encontram-se na linha da frente na luta contra a COVID-19, estando diretamente expostos ao novo Coronavírus, pelo contacto e tratamento de indivíduos infetados. A velocidade com que se propagou a pandemia COVID-19 e o número de vítimas que fez e continua a fazer, desencadeou nos profissionais de enfermagem problemas psicológicos, de entre os quais se salienta a ansiedade, resultante da perda de controlo da situação, do risco de infetar outros, inclusivamente os familiares e, claro está, o medo da morte. Desenvolveu-se um estudo quantitativo descritivo-correlacional, tendo como objetivos avaliar o nível de ansiedade dos enfermeiros de um serviço de urgência, face ao novo Coronavírus; descrever a relação entre o nível de ansiedade e as variáveis sociodemográficas, a experiência profissional no serviço e o resultado positivo para teste à COVID-19, assim como, identificar fatores relacionados com o nível de ansiedade dos enfermeiros. Recorreu-se à aplicação de um questionário como instrumento de colheita de dados com base na Escala de Avaliação de Ansiedade de Hamilton que categoriza a ansiedade em níveis, nomeadamente: normal, ligeira, moderada ou severa. Os resultados revelam que o score médio de ansiedade dos enfermeiros é de 15,1 o que a categoriza como ligeira. Verificou-se que existe relação estatisticamente significativa entre a ansiedade e a variável sexo e filhos, sendo que as mulheres apresentam níveis de ansiedade superiores aos homens e 71,4% dos enfermeiros que não têm filhos apresentam níveis de ansiedade ligeira, moderada ou severa. O fator relacionado com o nível de ansiedade identificado foi o sexo. Perante a atual situação pandémica e eventuais repercussões a longo prazo na saúde psicológica dos enfermeiros do serviço de urgência, considera-se importante e fundamental a monitorização dos níveis de ansiedade dos enfermeiros, em estudos futuros, de forma a caracterizar o seu estado emocional e a rastrear problemas nesta classe profissional. Tornam-se imperativos programas de intervenção com foco na proteção emocional dos enfermeiros.


Os enfermeiros do serviço de urgência encontram-se na linha da frente na luta contra a COVID-19, estando diretamente expostos ao novo Coronavírus, pelo contacto e tratamento de indivíduos infetados. A velocidade com que se propagou a pandemia COVID-19 e o número de vítimas que fez e continua a fazer, desencadeou nos profissionais de enfermagem problemas psicológicos, de entre os quais se salienta a ansiedade, resultante da perda de controlo da situação, do risco de infetar outros, inclusivamente os familiares e, claro está, o medo da morte. Desenvolveu-se um estudo quantitativo descritivo-correlacional, tendo como objetivos avaliar o nível de ansiedade dos enfermeiros de um serviço de urgência, face ao novo Coronavírus; descrever a relação entre o nível de ansiedade e as variáveis sociodemográficas, a experiência profissional no serviço e o resultado positivo para teste à COVID-19, assim como, identificar fatores relacionados com o nível de ansiedade dos enfermeiros. Recorreu-se à aplicação de um questionário como instrumento de colheita de dados com base na Escala de Avaliação de Ansiedade de Hamilton que categoriza a ansiedade em níveis, nomeadamente: normal, ligeira, moderada ou severa. Os resultados revelam que o score médio de ansiedade dos enfermeiros é de 15,1 o que a categoriza como ligeira. Verificou-se que existe relação estatisticamente significativa entre a ansiedade e a variável sexo e filhos, sendo que as mulheres apresentam níveis de ansiedade superiores aos homens e 71,4% dos enfermeiros que não têm filhos apresentam níveis de ansiedade ligeira, moderada ou severa. O fator relacionado com o nível de ansiedade identificado foi o sexo. Perante a atual situação pandémica e eventuais repercussões a longo prazo na saúde psicológica dos enfermeiros do serviço de urgência, considera-se importante e fundamental a monitorização dos níveis de ansiedade dos enfermeiros, em estudos futuros, de forma a caracterizar o seu estado emocional e a rastrear problemas nesta classe profissional. Tornam-se imperativos programas de intervenção com foco na proteção emocional dos enfermeiros.


Assuntos
Enfermagem , Emergências , SARS-CoV-2 , COVID-19 , Enfermeiras e Enfermeiros
14.
Eur Geriatr Med ; 12(5): 1057-1064, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-33646536

RESUMO

PURPOSE: Older patients are the fastest expanding subgroup of intensive care units (ICU) and are particularly susceptible to bacterial infections and sepsis. The aim of this study was to address the epidemiology and the main determinants of outcome of infection in old and very old patients admitted to ICU. METHODS: We performed a post hoc analysis of all infected patients admitted to ICU enrolled in a 1-year prospective, observational, multipurpose study. Patients aged < 65, 65-74 and ≥ 75 years were compared. RESULTS: Of the 1652 patients included, 50% were older than 65 years. There were no significant differences between young, old and very old patients in either ICU, hospital length of stay, or nosocomial infection. All-cause mortality was significantly higher in participants aged ≥ 75. Increased Gram-negative microorganisms' isolates occurred in > 65 years (25% versus 31%; p = 0.034). Multidrug-resistant (MDR) microorganisms were directly associated to inappropriate empiric antibiotic therapy (OR 4.73; 95% CI 2.99-7.47) and inversely associated with community-acquired infection (OR 0.39; 95% CI 0.19-0.83). Age (65-74 years: OR 1.10; 95% CI 0.64-1.90 and ≥ 75 years: OR 1.52; 95% CI 0.89-2.59) and sepsis severity (sepsis: OR 0.67; 95% CI 0.18-2.46; severe sepsis: OR 1.17; 95% CI 0.40-3.44; septic shock: OR 0.77; 95% CI 0.27-2.24) were not associated to MDR bacteria. CONCLUSION: Patients > 65 years accounted for 50% of infected patients admitted to an ICU. ICU and hospital length of stay, and nosocomial infection did not increase with age. Age did predispose to increased risk for infection by Gram-negatives. These findings may optimize strategies for infection management in older patients.


Assuntos
Fatores Etários , Infecção Hospitalar , Sepse , Idoso , Estado Terminal , Infecção Hospitalar/tratamento farmacológico , Humanos , Unidades de Terapia Intensiva , Estudos Prospectivos , Sepse/epidemiologia
15.
Clin. biomed. res ; 41(4): 332-338, 2021. ilus, graf
Artigo em Inglês | LILACS | ID: biblio-1349501

RESUMO

Introduction: Postmenopausal women are more susceptible to chronic conditions, such as osteoporosis, arthritis, and other inflammatory diseases. We investigated the effects of transcranial direct current stimulation (tDCS) on biomarker levels in ovariectomized rats subjected to an inflammatory model. Methods: Twenty adult female Wistar rats underwent ovariectomy and complete Freund's adjuvant (CFA)-induced inflammation. We divided them into 2 groups: OAS (sham tDCS) and OAT (active tDCS). Fifteen days later, the rats underwent bimodal tDCS treatment (20 min, 0.5 mA, 8 days). After 24 h of the last tDCS session, we killed the rats and collected tissue samples (hypothalamus, cerebral cortex, and brainstem) for biomarker analysis by ELISA. We removed the paws for histological analysis. Results: Active tDCS increased hypothalamic and cortical TNF-α and NGF levels, hypothalamic and brainstem IL-1ß levels, and hypothalamic IL-10 levels. Histology of paws showed an inflammatory profile. We observed a small tDCS effect, not statistically significant. Discussion: Bimodal tDCS had an effect on the central inflammatory axis, with a small effect on the peripheral site as evaluated by histology in the current study. (AU)


Assuntos
Animais , Feminino , Ratos , Ovariectomia/efeitos adversos , Biomarcadores , Estimulação Transcraniana por Corrente Contínua , Inflamação
16.
Int J Artif Organs ; 43(2): 87-93, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31509049

RESUMO

INTRODUCTION: Continuous renal replacement therapy is not presently recommended in the treatment of life-threatening hyperkalemia. There are no specific recommendations in hemodialysis to treat hyperkalemia. We hypothesized an in vitro model may provide valuable information on the usefulness of continuous renal replacement therapy to treat severe hyperkalemia. METHODS: A potassium-free solute was used instead of diluted blood for continuous renal replacement therapy with a simulated blood flowrate set at 200 mL/min. The mode of elimination included continuous filtration, continuous dialysis, and continuous diafiltration using a flowrate of 4000 mL/min for continuous filtration and continuous dialysis modes, and a ratio of 2500/1500 in the continuous diafiltration mode. RESULTS: The mean initial potassium in the central compartment was 10.1 ± 0.4 mmol/L. The clearances in the continuous diafiltration, continuous filtration, and continuous dialysis were 3.4 ± 0.5, 3.6 ± 0.1, and 3.7 ± 0.1 L/h, respectively, not significantly different. Continuous dialysis resulted in the lowest workload for staff. Increasing the continuous dialysis flowrates from 2000 to 8000 mL/h increased clearance from 2.3 ± 0.3 to 6.2 ± 0.8 L/h. The delays in decreasing the potassium concentration to 5.5 mmol/L dropped from 120 to 45 min, respectively. Potassium eliminated in the first hour increased from 18 to 38 mmol that compared favorably with hemodialysis. Decrease in simulated blood flowrate from 200 to 50 mL/min moderately but significantly decreased the clearance from 3.7 to 3.0 L/h. CONCLUSION: Hyperkalemia is efficiently treated by continuous renal replacement therapy using the dialysis mode. Caution is needed to prevent the onset of severe hypokalemia within 40 min after initiation of the session.


Assuntos
Terapia de Substituição Renal Contínua/métodos , Soluções para Diálise , Hiperpotassemia , Potássio , Soluções para Diálise/química , Soluções para Diálise/farmacologia , Humanos , Hiperpotassemia/diagnóstico , Hiperpotassemia/terapia , Teste de Materiais/métodos , Potássio/análise , Potássio/farmacocinética , Índice de Gravidade de Doença
17.
BMJ Case Rep ; 20182018 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-29909388

RESUMO

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterised by vascular abnormalities, immune system activation and fibrosis. Lymphatic involvement in SSc was described more recently and starts in early stages. This report describes a 46-year-old patient who developed over the last 2 years asymmetric lymphedema in lower extremities. Compromise in lymphatic drainage was confirmed by lymphoscintigraphy. She also presented Raynaud's phenomenon, a scleroderma pattern in nailfold capillaroscopy, cutaneous thickening and anticentromere antibodies, which together resulted in a new diagnosis of limited cutaneous SSc. Treatment with methotrexate, prednisolone and lymphatic drainage resulted in lymphedema improvement. To our knowledge, this is the first case of grade 2 lymphedema in the setting of anticentromere-positive limited cutaneous SSc. We highlight the importance of considering rheumatic diseases in the differential diagnosis of lymphedema.


Assuntos
Linfedema/diagnóstico por imagem , Metotrexato/uso terapêutico , Prednisolona/uso terapêutico , Esclerodermia Limitada/diagnóstico , Feminino , Humanos , Linfedema/tratamento farmacológico , Linfedema/etiologia , Linfocintigrafia , Angioscopia Microscópica , Pessoa de Meia-Idade , Doença de Raynaud/diagnóstico por imagem , Doença de Raynaud/tratamento farmacológico , Doença de Raynaud/etiologia , Esclerodermia Limitada/tratamento farmacológico , Resultado do Tratamento
18.
BMJ Case Rep ; 20182018 Apr 21.
Artigo em Inglês | MEDLINE | ID: mdl-29680799

RESUMO

Q fever is a zoonotic disease caused by Coxiella burnetii that usually presents with non-specific or benign constitutional symptoms. Diagnosis is often challenging and, after acute Q fever, 1%-5% of patients can develop chronic disease. We present an 80-year-old male patient who was admitted due to a 3 months history of fever, productive cough, myalgia, weight loss, headache and hearing loss. Chronic Q fever was confirmed by positive antiphase I immunoglobulin G. Frequent locations of chronic infection was discarded, and ear CT revealed a right mastoid infection. He was treated with doxycycline and hydroxychloroquine for 18 months with significant improvement. This is a rare case of chronic Q fever presenting with otomastoiditis that has never been described.


Assuntos
Coxiella burnetii/isolamento & purificação , Mastoidite/patologia , Otite/patologia , Febre Q/diagnóstico , Febre Q/microbiologia , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Antimaláricos/uso terapêutico , Doxiciclina/administração & dosagem , Doxiciclina/uso terapêutico , Humanos , Hidroxicloroquina/administração & dosagem , Hidroxicloroquina/uso terapêutico , Imunoglobulina G/sangue , Masculino , Mastoidite/diagnóstico por imagem , Mastoidite/tratamento farmacológico , Mastoidite/microbiologia , Otite/diagnóstico por imagem , Otite/tratamento farmacológico , Otite/microbiologia , Febre Q/imunologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
19.
BMJ Case Rep ; 20172017 Nov 28.
Artigo em Inglês | MEDLINE | ID: mdl-29184006

RESUMO

Camurati-Engelmann disease (CED) is a rare disorder included in the group of craniotubular hyperostosis diseases. Corticosteroids are used for pain management in CED, but in refractory or corticosteroid-non-tolerant patients, pain management is limited. We report the case of a woman with CED diagnosed in early infancy whose initial complaints included persistent bone pain associated with progressive functional disability. She was treated with steroids but over time became dependent on higher doses with only mild pain relief. In her third decade, she was diagnosed with ulcerative colitis (UC) and was treated with mesalazine, azathioprine and prednisolone. Due to recurrent exacerbations of UC, treatment was changed to infliximab, an antitumour necrosis factor-alpha (TNFα). Remission of UC was achieved and CED-associated pain also improved with infliximab. This is the first report showing a possible role of anti-TNFα in pain management in CED with unsatisfactory response to steroids.


Assuntos
Síndrome de Camurati-Engelmann/tratamento farmacológico , Dor Crônica/tratamento farmacológico , Colite Ulcerativa/tratamento farmacológico , Fármacos Gastrointestinais/uso terapêutico , Infliximab/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Síndrome de Camurati-Engelmann/complicações , Síndrome de Camurati-Engelmann/genética , Dor Crônica/genética , Colite Ulcerativa/genética , Feminino , Humanos , Pessoa de Meia-Idade
20.
Pharmacol Rep ; 68(1): 109-15, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26721361

RESUMO

BACKGROUND: Estrogen deficiency is associated with the onset of depressive and anxiety symptoms, cognitive impairment, and adverse consequences. We investigated depressive-like behaviors in ovariectomized rats and ketamine's effect on this behavior. METHODS: Twenty-eight female Wistar adult rats were initially divided into two groups: ovariectomized (OVX) and sham surgery (SHAM). Hormonal status was verified by vaginal cytology, and the rats were subjected to a forced swimming (FS) test 18 days post-surgery, an open field (OF) test 28 days post-surgery, and an elevated plus maze (EPM) test 38 days post-surgery (Experiment 1). In addition, the effect of ketamine on depressive-like behavior of the female rats was evaluated (Experiment 2). RESULTS: OVX group exhibited anxiety-like behavior on EPM test (lower time spent and fewer entries in the open arms), without any difference in performance in the OF test. OVX rats showed depressive-like behavior (higher time of immobility) than SHAM rats in FS test. The SHAM group showed signs of hypoestrogenism (anestrus) at six months of age. Moreover, ketamine was able to reverse depressive-like behavior in the FS retest in both groups (OVX and SHAM). CONCLUSION: Similar to the literature, we showed the antidepressant effect of ketamine in depressive female rats which was induced by ovariectomy; including in female rats submitted to sham surgery that interestingly presented a premature menopausal.


Assuntos
Antidepressivos/uso terapêutico , Depressão/sangue , Depressão/tratamento farmacológico , Estrogênios/sangue , Ketamina/uso terapêutico , Ovariectomia/efeitos adversos , Afeto/efeitos dos fármacos , Afeto/fisiologia , Animais , Antidepressivos/farmacologia , Feminino , Ketamina/farmacologia , Aprendizagem em Labirinto/efeitos dos fármacos , Aprendizagem em Labirinto/fisiologia , Ovariectomia/tendências , Ratos , Ratos Wistar
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