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1.
Am J Case Rep ; 22: e927556, 2021 Jan 12.
Artigo em Inglês | MEDLINE | ID: mdl-33431787

RESUMO

BACKGROUND Fibrosing mediastinitis is a rarely seen, progressive disease. It results from an excessive fibrotic reaction in the mediastinum. We describe a presentation of fibrosing mediastinitis that, to our knowledge, has never been seen before. CASE REPORT A 30-year-old female Colombian flight attendant presented with a right eyelid droop. Examination revealed partial right-sided ptosis and miosis but no anhidrosis. An ill-defined firm swelling was palpable at the root of the neck. Chest radiography revealed a widened mediastinum, and computerized tomography (CT) showed a right paratracheal mass without calcification extending to the thoracic inlet, encasing multiple blood vessels. All basic blood tests, magnetic resonance imaging of the head, and ultrasound Doppler of the neck vessels were normal. History and work up for infections including fungal diseases, granulomatous diseases, vasculitis, and autoimmune diseases were negative. Positron emission tomography (PET) showed significant FDG uptake in the mediastinum. Mediastinal biopsy was histologically consistent with fibrosing mediastinitis. All relevant immunohistochemistry and microbiological studies were negative. Subsequently, the patient developed signs of superior vena cava compression; this was managed by balloon angioplasty, which resulted in improvement of symptoms. However, over time, her symptoms worsened progressively, resulting in a left-sided ptosis and radiological progression of the mass on CT. She received treatment with rituximab and concomitant steroids, which yielded excellent results: the treatment led to both resolution of her symptoms and regression of the mass and its metabolic activity on PET scan. CONCLUSIONS Fibrosing mediastinitis can present with an incomplete Horner's syndrome. Treatment with rituximab and steroids shows promising results in select cases of metabolically active idiopathic fibrosing mediastinitis.


Assuntos
Blefaroptose/etiologia , Mediastinite/complicações , Mediastinite/diagnóstico , Miose/etiologia , Esclerose/complicações , Esclerose/diagnóstico , Adulto , Feminino , Humanos , Mediastinite/terapia , Esclerose/terapia
3.
BMC Neurol ; 20(1): 241, 2020 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-32532251

RESUMO

BACKGROUND: Increasing evidence supports the role of soluble inflammatory mediators in the pathogenesis of refractory temporal lobe epilepsy (TLE). Hippocampal sclerosis (HS) is a well-described pathohistological abnormality in TLE. The association of proinflammatory cytokines with epileptic disease profiles is well established; however, the potential significance of circulating interleukin 10 (IL-10), particularly in TLE-associated HS, is still poorly understood. Therefore, taking into consideration the neuroprotective and anticonvulsive effects of IL-10, we performed this study to examine the role of the plasma levels of IL-10 in patients with TLE with HS (TLE + HS), TLE without HS (TLE-HS) and with other types of epilepsy. METHODS: This study included 270 patients with refractory epilepsy who were classified into four groups: i) 34 patients with TLE + HS, ii) 105 patients with TLE-HS, iii) 95 patients with extra-TLE (XLE) and iv) 36 patients with idiopathic generalized epilepsy (IGE). The plasma IL-10 levels were quantified using a commercially available enzyme-linked immunosorbent assay (ELISA). RESULTS: IL-10 levels were significantly lower in TLE + HS than in TLE-HS (p = 0.013). In a subgroup of TLE-HS patients who had seizures 1 month before sampling, patients with seizures had significantly higher IL-10 levels than patients who were seizure-free (p = 0.039). Among a small group (n = 15) of non-refractory TLE-HS patients, IL-10 levels showed a moderate negative correlation with the duration of epilepsy (r = - 0.585, p = 0.023). CONCLUSIONS: This study demonstrated that chronically reduced levels of plasma IL-10 were associated with HS in TLE patients, suggesting that there was an inadequate systemic anti-inflammatory immune response. These results could provide new biological insights into the pathophysiology of HS in TLE. We also found that the production of IL-10 could be affected by the seizure frequency and declined concomitantly with increased disease durations. Therefore, the measurement of plasma IL-10 may have diagnostic value as a biomarker for stratifying TLE + HS from other epilepsy types or as a marker of disease progression towards a progressive form of epilepsy.


Assuntos
Epilepsia do Lobo Temporal/sangue , Epilepsia do Lobo Temporal/patologia , Hipocampo/patologia , Interleucina-10/sangue , Adulto , Epilepsia Resistente a Medicamentos/sangue , Epilepsia Resistente a Medicamentos/imunologia , Epilepsia Resistente a Medicamentos/patologia , Epilepsia do Lobo Temporal/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose/sangue , Esclerose/complicações , Esclerose/patologia
5.
Reumatol. clín. (Barc.) ; 16(2,pt.2): 165-168, mar.-abr. 2020. tab
Artigo em Espanhol | IBECS | ID: ibc-194341

RESUMO

La osteogénesis imperfecta (OI) es un trastorno hereditario del tejido conectivo generalmente relacionado con mutaciones de los genes del colágeno tipoI. El diagnóstico se basa en los hallazgos clínicos y radiológicos. El manejo clínico de la OI en adultos no está del todo establecido y comprende desde la rehabilitación física y los procedimientos quirúrgicos hasta el uso de tratamientos antirresortivos y osteoformadores. El objetivo del presente trabajo ha sido analizar las características clínicas y analíticas de estos pacientes en la edad adulta, así como evaluar los diferentes tratamientos administrados. Se han revisado los casos de OI diagnosticados en nuestro centro en los últimos 12 años (2005-2017). Se describen 15 pacientes adultos con OI


Osteogenesis imperfecta (OI) is an inherited connective tissue disease. The disease has been linked to mutations in one of the type I collagen genes. The diagnosis is based on clinical and radiologic findings. The management of OI in adults is not well-established and includes physical rehabilitation, surgical procedures, the use of antiresorptive therapy and anabolic agents. The aim of the present work was to analyze the clinical and analytical characteristics of these patients in adulthood, as well as to evaluate the different treatments administered. We reviewed the cases of OI diagnosed in our center over the last 12 years (2005-2017). We describe 15 adult patients with OI


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Osteogênese Imperfeita/diagnóstico por imagem , Osteogênese Imperfeita/tratamento farmacológico , Difosfonatos/administração & dosagem , Densitometria/métodos , Osteogênese Imperfeita/fisiopatologia , Pró-Colágeno/uso terapêutico , Estudos Retrospectivos , Esclerose/complicações , Dentinogênese , Osteoporose/complicações , Doenças Ósseas Metabólicas/complicações
6.
Cancer Radiother ; 24(2): 166-173, 2020 Apr.
Artigo em Francês | MEDLINE | ID: mdl-32220562

RESUMO

Stereotactic radiosurgery (SRS) is a non-invasive technique that enables to create brain focal lesions with a high precision and localization. Thus, functional brain disorders can be treated by SRS in case of pharmacoresistance or inoperability. To date, treatment of trigeminal neuralgia is the most described and known indication. Other indications will be developed in the future like movement disorders, refractory epilepsy, obsessive compulsive disorder and severe depression. We present here a review of actual and future indications of functional brain SRS with their level of evidence. All these SRS treatments have to be strictly conducted by trained teams with an excellent collaboration between radiation physicists, medical physicists, neurosurgeons, neurologists, psychiatrists and probably neuroradiologists.


Assuntos
Epilepsia/radioterapia , Radiocirurgia/métodos , Tremor/radioterapia , Neuralgia do Trigêmeo/radioterapia , Transtorno Depressivo Maior/terapia , Epilepsia/etiologia , Humanos , Transtorno Obsessivo-Compulsivo/terapia , Doença de Parkinson/complicações , Doença de Parkinson/radioterapia , Radiocirurgia/efeitos adversos , Radiocirurgia/tendências , Dosagem Radioterapêutica , Esclerose/complicações , Resultado do Tratamento , Tremor/etiologia , Neuralgia do Trigêmeo/diagnóstico por imagem
8.
Vasc Med ; 25(2): 174-183, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31804157

RESUMO

Fibrosing mediastinitis (FM) is a rare disorder of inflammation and fibrosis involving the mediastinum. The formation of fibroinflammatory mass in the mediastinum can lead to obstruction of mediastinal structures and cause severe debilitating and life-threatening symptoms. Superior vena cava syndrome (SVCS) is a dreaded complication of FM with no medical therapy proven to be efficacious. Spiral vein grafting has long been utilized as first-line therapy for SVC syndrome due to FM. Endovascular repair with stents and angioplasty for malignant causes of SVC syndrome is well established. However, there are limited data on their utility in SVC syndrome due to FM. We present two cases of SVC syndrome due to FM treated with endovascular stenting and a detailed review of current literature on its utility in SVCS due to benign causes.


Assuntos
Angioplastia com Balão/instrumentação , Mediastinite/complicações , Esclerose/complicações , Stents , Síndrome da Veia Cava Superior/terapia , Veia Cava Superior/fisiopatologia , Adulto , Angioplastia com Balão/efeitos adversos , Feminino , Hemodinâmica , Humanos , Masculino , Mediastinite/diagnóstico , Pessoa de Meia-Idade , Esclerose/diagnóstico , Síndrome da Veia Cava Superior/diagnóstico por imagem , Síndrome da Veia Cava Superior/etiologia , Síndrome da Veia Cava Superior/fisiopatologia , Resultado do Tratamento , Veia Cava Superior/diagnóstico por imagem
10.
Zhonghua Xin Xue Guan Bing Za Zhi ; 47(10): 814-819, 2019 Oct 24.
Artigo em Chinês | MEDLINE | ID: mdl-31648464

RESUMO

Objective: To evaluate the feasibility and safety percutaneous pulmonary vein intervention in patients with severe pulmonary vein stenosis (PVS) caused by fibrosing mediastinitis(FM). Methods: This retrospective analysis included 5 FM patients (2 male, 3 female, 54-77 years old) confirmed by clinical presentation and chest computed tomography (CT) scan from January to June 2018 who were from Gansu Provincial Hospital and Shanghai Chest Hospital. CT pulmonary angiography (CTPA) further revealed severe PVS caused by fibrotic tissue compression in mediastinum. After selective pulmonary vein angiography, gradually balloon angioplasty was used to expand the pulmonary vein and then stents were implanted in the pre-dilated stenotic pulmonary veins. Evaluation of therapeutic effect was made at 6 months after the procedure. Results: All of 11 serious compression PVS were treated with stent implantation (diameter: 7-10 mm, length: 17-27 mm). After stenting, degree of pulmonary vein stenosis decreased from (83±16)% to (12±4)% (P<0.01). The minimal diameter of the stenotic pulmonary vein was significantly increased from (0.8±0.5)mm to (7.5±0.8)mm (P<0.01). Trans-stenotic gradient decreased from (27.0±15.1)mmHg (1 mmHg=0.133 kPa) to (2.50±0.58)mmHg (P<0.05). Mean pulmonary pressure measured by cardiac catheter decreased from (45.0±9.0)mmHg to (38.7±8.4)mmHg (P<0.05). One patient experienced cardiac arrest due to vagal nerve reflex during big sizing balloon stent dilation and recovered after cardiopulmonary resuscitation. There were no other serious procedure related complications. During the follow-up, severe stenosis at end of proximal stent was evidenced in 1 patient due to fibrotic compression, and another patient developed in-stent thrombosis due to discontinuation of prescribed anticoagulant. Conclusion: Percutaneous intervention for severe pulmonary vein stenosis caused by FM is feasible and safe, and can improve hemodynamic caused by the compression of mediastinal vascular structures in these carefully selected patients.


Assuntos
Angioplastia com Balão , Mediastinite/complicações , Esclerose/complicações , Estenose de Veia Pulmonar/terapia , Stents , Idoso , China , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Veias Pulmonares/patologia , Estudos Retrospectivos , Estenose de Veia Pulmonar/etiologia , Resultado do Tratamento
13.
Epilepsy Behav ; 96: 192-199, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31150999

RESUMO

INTRODUCTION: Temporal lobe epilepsy (TLE) is the most common adult epileptic syndrome. About 30-70% of those cases have neuropsychiatric complications. More than 10% of patients have TLE because of focal cortical dysplasia (FCD) type IIIa. OBJECTIVES: The objective of this study was to review the evidence of reelin (RELN) deficiency and tau phosphorylation role in the histopathological, neuropsychiatric, and hyperexcitability features in TLE because of dysplasia type IIIa. METHODS: The current literature was reviewed using Cochrane, EMBASE, PROSPERO, MEDLINE, and PubMed from 1995 to July 2018. Articles of interest were reviewed by one investigator (RAM). RESULTS: Reelin deficit is related to an abnormal migration of neurons in dentate gyrus, and its deficit causes dentate gyrus abnormalities, which in turn has been associated with memory deficits in patients with TLE. A decreased in the expression of RELN ribonucleic acid (RNA) was found in patients with TLE and dysplasia type IIIa compared with patients with TLE and isolated hippocampal sclerosis (HS). Reelin might affect the distribution and dynamic instability of microtubules within neurons in the cerebral cortex and their phosphorylation. Amyloid pathology, tauopathy, or phosphorylated tau (p-tau) overexpression has been reported in epileptic human brain and in animal models of epilepsy. CONCLUSION: Reelin deficit may determine an abnormal cortical lamination and dentate gyrus dispersion and might be associated with an abnormal tau phosphorylation. These processes can be associated with an abnormal hyperexcitability, neuropsychiatric complications, and a myriad of typical histopathological features seen in patients with TLE because of dysplasia type IIIa.


Assuntos
Moléculas de Adesão Celular Neuronais/metabolismo , Epilepsia do Lobo Temporal/complicações , Proteínas da Matriz Extracelular/metabolismo , Hipocampo/metabolismo , Transtornos da Memória/complicações , Proteínas do Tecido Nervoso/metabolismo , Serina Endopeptidases/metabolismo , Proteínas tau/metabolismo , Encéfalo/metabolismo , Encéfalo/patologia , Epilepsia do Lobo Temporal/metabolismo , Epilepsia do Lobo Temporal/patologia , Hipocampo/patologia , Humanos , Transtornos da Memória/metabolismo , Transtornos da Memória/patologia , Neurônios/metabolismo , Fosforilação , Esclerose/complicações , Esclerose/metabolismo , Esclerose/patologia
14.
Skinmed ; 17(2): 128-129, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31145067

RESUMO

A 34-year-old man presented with a painless lesion of the glans present for more than 4 years. The patient became HIV-positive in 2011, and he has been treated with tenofovir, emtricitabine, and efavirenz. A CD4 count performed 4 months prior was 570 cells/mL3; syphilis, hepatitis B, and hepatitis C serologies performed on the same date were non-reactive.


Assuntos
Linfangioma/etiologia , Linfangite/complicações , Vasos Linfáticos/patologia , Neoplasias Penianas/etiologia , Adulto , Soropositividade para HIV/complicações , Soropositividade para HIV/tratamento farmacológico , Humanos , Masculino , Esclerose/complicações
16.
Rev. bras. cir. plást ; 34(1): 134-137, jan.-mar. 2019. ilus
Artigo em Inglês, Português | LILACS | ID: biblio-994618

RESUMO

Introdução: A esclerose sistêmica é uma doença rara, autoimune, com evolução progressiva, que afeta os tecidos conectivos e órgãos internos por inflamação, podendo causar calcinose de subcutâneo. Podem evoluir para quadros dolorosos e incapacitantes, podendo tornar-se infectados, principalmente quando ulceram pela pele. Objetivo: Apresentar caso de calcinose em região inguinal e sua evolução cirúrgica. Relato de Caso: Paciente feminina portadora de calcinoses em região inguinal bilateral, apresentando algia moderada/grave com falha de tratamento clínico. Realizada ressecção cirúrgica das calcinoses, que formavam cordões de fibrose com aderência na fáscia do músculo oblíquo externo. Realizado fechamento primário com nylon 2.0 pontos simples subdérmicos e ponto intradérmico continuo nylon 3.0 para fechamento estético e menor reação inflamatória. Boa evolução pós- operatório. Conclusão: O melhor tratamento da calcinoses ainda não é claro. O tratamento das complicações se torna essencial para reduzir a morbidade e aumentar a qualidade de vida do paciente.


Introduction: Systemic sclerosis is a rare, autoimmune, progressive disease that affects connective tissues and internal organs by inflammation, which can cause calcinosis cutis. It can progress to painful and disabling conditions, and can become infected, especially when skin ulceration is present. Objective: To present a case of calcinosis in the inguinal region and its surgical recovery. Case Report: A female patient with calcinosis in the bilateral inguinal region presenting with moderate/severe pain had a failed clinical treatment. We performed surgical resection of the calcinosis cutis, which had formed clusters of fibrosis with adhesion to the fascia of the external oblique muscle. We used simple nylon 2.0 sutures along the subdermal plane to perform primary closure and continuous nylon 3.0 sutures along the intradermal plane for aesthetic closure and minimal inflammatory reaction. Her postoperative recovery was positive. Conclusion: The best treatment for calcinosis cutis is still unclear. Treating complications becomes essential for reducing patients' morbidity and increasing their quality of life.


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Reumatologia/métodos , Esclerose/cirurgia , Esclerose/complicações , Doenças Autoimunes/diagnóstico , Procedimentos Cirúrgicos Operatórios/métodos , Calcinose/diagnóstico , Calcinose/patologia , Procedimentos Cirúrgicos Reconstrutivos/métodos , /métodos , Inflamação/patologia
17.
Braz J Cardiovasc Surg ; 34(1): 85-92, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30810679

RESUMO

Sclerosing mediastinitis (SM), previously named chronic fibrosing mediastinitis, is an inflammatory process that in its end-stage results to sclerosis around the mediastinal structures. SM is quite rare and has been correlated with inflammatory and autoimmune diseases, as well as malignancy. SM may either present in a mild form, with minor symptoms and a benign course or in a more aggressive form with severe pulmonary hypertension and subsequent higher morbidity and mortality. The diagnosis of SM may be difficult and quite challenging, as symptoms depend on the mediastinal structure that is mainly involved; quite often the superior vena cava. However, practically any mediastinal structure may be involved by the fibrotic process, such as the central airways, as well as the pulmonary arteries and veins, leading to obstruction or total occlusion. The latter may be impossible to undergo proper surgical excision of the lesion, and is considered to be a real challenge to the surgeon. We herein report a case of SM that presented with arterial and venous compression. The imaging appearance was that of unilateral pulmonary edema, associated with lung collapse. The case is supplemented by a non-systematic review of the relevant literature.


Assuntos
Mediastinite/complicações , Atelectasia Pulmonar/etiologia , Edema Pulmonar/etiologia , Esclerose/complicações , Adulto , Biópsia , Constrição Patológica/diagnóstico por imagem , Constrição Patológica/patologia , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/patologia , Humanos , Mediastinite/diagnóstico por imagem , Mediastinite/patologia , Atelectasia Pulmonar/diagnóstico por imagem , Atelectasia Pulmonar/patologia , Edema Pulmonar/diagnóstico por imagem , Edema Pulmonar/patologia , Radiografia Torácica , Esclerose/diagnóstico por imagem , Esclerose/patologia , Tomografia Computadorizada por Raios X
20.
Neurology ; 92(4): e351-e358, 2019 01 22.
Artigo em Inglês | MEDLINE | ID: mdl-30587513

RESUMO

OBJECTIVE: To examine cerebral cortex thickness in asymptomatic first-degree relatives of patients with mesial temporal lobe epilepsy (MTLE). METHODS: We investigated 127 asymptomatic first-degree relatives of patients with MTLE due to hippocampal sclerosis (HS) (mean age ± SD = 39.4 ± 13 years) and 203 healthy control individuals (mean age ± SD = 36.0 ± 11 years). Participants underwent a comprehensive clinical evaluation and structural brain MRI at 3 study sites. Images were processed simultaneously at each site using a surface-based morphometry method to quantify global brain measures, hippocampal volumes, and cerebral cortical thickness. Differences in brain measures between relatives of patients and controls were examined using generalized models, while controlling for relevant covariates, including age and sex. RESULTS: None of the asymptomatic first-degree relatives of MTLE + HS patients showed evidence of HS on qualitative image assessments. Compared to the healthy controls, the asymptomatic relatives of patients displayed no significant differences in intracranial volume, average hemispheric surface area, or hippocampal volume. Similarly, no significant cerebral cortical thinning was identified in the relatives of patients. This was consistent across the 3 cohorts. CONCLUSION: Lack of cortical thickness changes in the asymptomatic relatives of patients indicates that the previously characterized MTLE + HS-related cortical thinning is not heritable, and is likely driven by disease-related factors. This finding therefore argues for early and aggressive intervention in patients with medically intractable epilepsy.


Assuntos
Córtex Cerebral/diagnóstico por imagem , Epilepsia do Lobo Temporal/patologia , Família , Adulto , Estudos de Coortes , Epilepsia do Lobo Temporal/etiologia , Feminino , Hipocampo/diagnóstico por imagem , Hipocampo/patologia , Humanos , Imageamento Tridimensional , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose/complicações , Estatísticas não Paramétricas
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