Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.091
Filtrar
1.
Int Ophthalmol ; 44(1): 17, 2024 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-38321320

RESUMO

OBJECTIVE: To raise awareness of conditions that can tomographically mimic corneal ectasia and describe the actions required to avoid misdiagnosis. METHODS: We report a retrospective case series of seven patients presenting at two tertiary care centers in Israel with a presumed diagnosis of keratoconus or post-refractive ectasia. Upon further examination, the ectasia diagnosis was reconsidered and eventually ruled out. RESULTS: Included were ten eyes of seven patients. Cases included bilateral diffuse Salzmann's nodular degeneration, ophthalmoplegia with strabismus which precluded proper fixation during the acquisition of tomography images, two cases of incorrect Pentacam parameter settings, a patient with a history of hyperopic laser-assisted in situ keratomileusis (LASIK) treatment in one eye and myopic LASIK in the fellow eye, a case of old post-photorefractive keratectomy (PRK) stromal haze, and a patient with posterior polymorphous corneal dystrophy. CONCLUSIONS: Tomography patterns mimicking corneal ectasia can appear in patients without ectatic pathology. The comprehensive ophthalmologist should be aware of such cases as they may substantially alter the treatment course and prognosis of these patients.


Assuntos
Ceratocone , Ceratomileuse Assistida por Excimer Laser In Situ , Ceratectomia Fotorrefrativa , Humanos , Estudos Retrospectivos , Dilatação Patológica/patologia , Dilatação Patológica/cirurgia , Córnea/patologia , Ceratectomia Fotorrefrativa/métodos , Ceratocone/diagnóstico , Ceratomileuse Assistida por Excimer Laser In Situ/métodos , Lasers de Excimer , Topografia da Córnea/métodos
2.
Clin J Gastroenterol ; 17(1): 177-182, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37851209

RESUMO

Congenital biliary dilatation (CBD) is a congenital malformation of focal dilatation of the extrahepatic bile ducts, including the common bile duct, and is often associated with pancreaticobiliary maljunction (PBM). In this article, we report a CBD case that presented with focal dilation of the common bile duct without PBM (Todani's classification type Ib). The patient was a 32-year-old man who visited a doctor with a chief complaint of abdominal distension. Computed tomography revealed cystic dilatation of the common bile duct, and the patient was referred to our institution. Magnetic resonance cholangiopancreatography showed cystic dilatation of the common bile duct with a maximum diameter of 7 cm; however, evaluating the presence of PBM was challenging. Endoscopic ultrasonography showed small gallstones and debris in the dilated common bile duct and no thickening of the gallbladder wall. Endoscopic retrograde cholangiopancreatography revealed no PBM or markedly elevated bile amylase levels. Based on these findings, the patient was diagnosed with Todani Type Ib CBD. Since this patient did not have pancreatobiliary reflux, it was unclear whether the risk of developing biliary tract cancer was high, and since the treatment was highly invasive, the decision was to follow up without surgical treatment.


Assuntos
Ductos Biliares Extra-Hepáticos , Neoplasias do Sistema Biliar , Cisto do Colédoco , Má Junção Pancreaticobiliar , Masculino , Humanos , Adulto , Cisto do Colédoco/patologia , Cisto do Colédoco/cirurgia , Colangiopancreatografia Retrógrada Endoscópica/métodos , Dilatação Patológica/diagnóstico por imagem , Dilatação Patológica/congênito , Dilatação Patológica/patologia , Ductos Pancreáticos/patologia
3.
Ocul Surf ; 29: 557-565, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37393064

RESUMO

PURPOSE: We hypothesized that Transforming growth factor beta receptor 2 (Tgfbr2) deletion in keratocyte (Tgfbr2kera-cko), the corneal stroma cell, can result in corneal thinning and generate a potential model for Cornea Ectasia (CE). METHODS: Corneal thickness of Tgfbr2kera-cko and Tgfbr2Ctrl was examined with Optical Coherence Tomography (OCT) at post-natal (P) days 42 and 70, respectively. Histological H&E staining, transmission electron micrograph (TEM), and immunofluorescence staining (IFS) were harnessed to examine corneal cell morphology, proliferation, differentiation, and collagen fibrils. RESULTS: Slit-Lamp revealed that corneas were transparent in both Tgfbr2kera-cko and Tgfbr2Ctrl, however, Tgfbr2kera-cko cornea was 33.5% and 42.9% thinner as compared with those of Tgfbr2Ctrl at P42 and P70, respectively. H&E and semithin section staining with toluidine blue-O confirmed that Tgfbr2kera-cko cornea has a thinner stroma. In contrast, the epithelium in Tgfbr2kera-cko was substantially thicker. The cell proliferation marker Ki67 expression level increased ∼9% in Tgfbr2kera-cko corneal epithelium as compared with that in Tgfbr2Ctrl, however, the Krt14 and Krt12 expression pattern was not obviously changed in Tgfbr2kera-cko corneal epithelium. It was noticed that Col1a1 expression was substantially reduced in Tgfbr2kera-cko as compared with that in Tgfbr2Ctrl. TEM showed that keratocytes were unhealthy and stromal collagen fibril density was significantly reduced in Tgfbr2kera-cko as compared with that in Tgfbr2Ctrl cornea. Moreover, mechanical eye-rubbing on Tgfbr2kera-cko resulted in corneal hydrops and edema. CONCLUSION: Tgfbr2 in keratocytes is indispensable for the corneal stroma at postnatal homeostasis. The cornea phenotype manifested in these Tgfbr2kera-cko mice resembles corneal ectasia disease in humans.


Assuntos
Córnea , Doenças da Córnea , Receptor do Fator de Crescimento Transformador beta Tipo II , Animais , Humanos , Camundongos , Colágeno , Córnea/patologia , Doenças da Córnea/patologia , Substância Própria , Dilatação Patológica/metabolismo , Dilatação Patológica/patologia , Receptores de Fatores de Crescimento Transformadores beta/genética , Receptores de Fatores de Crescimento Transformadores beta/metabolismo , Receptor do Fator de Crescimento Transformador beta Tipo II/genética , Receptor do Fator de Crescimento Transformador beta Tipo II/metabolismo
4.
Curr Opin Allergy Clin Immunol ; 23(5): 430-437, 2023 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-37490610

RESUMO

PURPOSE OF REVIEW: Inflammatory mediators are a focus of recent corneal ectasia (CE) research and are a profound, modifiable contributor to CE in general and keratoconus (KC) in particular, opening a path to explore new methods of control. As advanced imaging technology and expanded population screening allow for earlier detection, the possibility of early intervention can profoundly change the prognosis of CE. RECENT FINDINGS: Significant increases in the inflammatory mediators and immune components have been observed in the cornea, tear fluid, and blood of ectasia patients, while inflammation dampeners such as vitamin D and their receptors are reduced. Atopy and allergy have a strong association with KC, known to increase itch factors and stimulate eye rubbing, a risk factor in ectasia pathogenesis. Management of atopy or allergic conditions and topical anti-inflammatories has helped stabilize CE disease. SUMMARY: Strategies such as monitoring inflammatory factors and using immune or inflammatory modulators, including managing subclinical inflammation, may be clinically beneficial in stabilizing the disease and improving outcomes. The detected factors are biomarkers, but as yet unproven to be sensitive or specific enough to be considered biomarkers for early detection of CE. The establishment of such biomarkers could improve the therapeutic outcome.


Assuntos
Hipersensibilidade Imediata , Ceratocone , Humanos , Dilatação Patológica/complicações , Dilatação Patológica/patologia , Córnea/patologia , Ceratocone/etiologia , Inflamação/patologia , Mediadores da Inflamação , Hipersensibilidade Imediata/complicações , Biomarcadores
5.
Forensic Sci Med Pathol ; 19(2): 269-272, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37046062

RESUMO

Following a witnessed lethal lightning strike of an adult male who was standing outside in a storm, numerous Lichtenberg figures were identified upon external examination of the body. Sectioning across multiple areas of linear erythema in the figures showed no subcutaneous hemorrhage. This was later confirmed on histology which showed only subtle dermal capillary dilatation with no interstitial hemorrhage or inflammation in these areas. The only areas of interstitial hemorrhage were present in adjacent scattered punctate burns from arcing. The documented resolution of Lichtenberg figures within hours would be more in keeping with temporary functional capillary dilatation, shown in this case, rather than with tissue alteration by interstitial hemorrhage or inflammation.


Assuntos
Lesões por Ação do Raio , Pele , Adulto , Humanos , Masculino , Pele/patologia , Lesões por Ação do Raio/patologia , Dilatação Patológica/patologia , Inflamação/patologia , Hemorragia/patologia
6.
Curr Probl Cardiol ; 48(7): 101687, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-36931334

RESUMO

Neointimal hyperplasia and lumen irregularities are major contributors to vein graft failure and the use of VEST(R) should prevent this. In this review, we aim to evaluate the angiographic outcomes of externally supported vein grafts. Medline, Embase and Cochrane Library were systematically reviewed for randomized clinical trials published by August 2022. The primary outcome was graft failure. Secondary outcomes included graft ectasia, intimal hyperplasia area and thickness, and graft nonuniformity. Odds ratios (OR) for dichotomous variables and mean difference (MD) for continuous variables with 95% confidence intervals (CI) were pooled using a fixed-effects model. Three randomized controlled trials with a total of 437 patients were included with follow-up ranging from 1 to 2 years. The odds of graft failure were similar in the 2 groups (OR 1.22; 95%CI 0.88-1.71; I²â€¯= 0%). Intimal hyperplasia area [MD -0.77 mm2; 95%CI -1.10 to -0.45; I2 = 0%] and thickness [MD -0.06 mm; 95% CI -0.08 to -0.04; I2=0%] were significantly lower in the VEST group. Fitzgibbon Patency Scale of II or III (representing angiographic conduit nonuniformity; OR 0.67; 95%CI 0.48-0.94; I2 = 0%) and graft ectasia (OR 0.53; 95%CI 0.32-0.88; I2 = 33%) were also significantly lower in the VEST group. At short-term follow-up, VEST does not seem to reduce the incidence of graft failure, although it is associated with attenuation of intimal hyperplasia and nonuniformity. Longer angiographic follow-up is warranted to determine whether these positive effects might translate into a positive effect in graft failure and in long-term clinical outcomes.


Assuntos
Oclusão de Enxerto Vascular , Veia Safena , Humanos , Oclusão de Enxerto Vascular/epidemiologia , Oclusão de Enxerto Vascular/etiologia , Oclusão de Enxerto Vascular/patologia , Grau de Desobstrução Vascular , Hiperplasia/complicações , Hiperplasia/patologia , Dilatação Patológica/complicações , Dilatação Patológica/patologia , Veia Safena/cirurgia , Veia Safena/transplante , Ponte de Artéria Coronária/efeitos adversos , Ensaios Clínicos Controlados Aleatórios como Assunto
7.
J Pak Med Assoc ; 73(1): 187-190, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36842038

RESUMO

Ductal ectasia with metaplasia and focal epithelial proliferation in the oral cavity does not correspond to any existing salivary gland lesion. A 72-year-old man presented with a mass in the buccal mucosa, which was excised and initially diagnosed as a cystadenoma. An upper lip mass on the right side, which developed later, was also excised. The lesions were histologically similar, and since they were multifocal and in non-contiguous and independent sites with multiple dilated cystic structures that did not destroy the lobar architecture, the final diagnosis was confirmed as ductal ectasia with metaplasia and focal epithelial proliferation. This condition may mimic various neoplastic lesions.


Assuntos
Cistos , Mucosa Bucal , Masculino , Humanos , Idoso , Dilatação Patológica/patologia , Mucosa Bucal/patologia , Metaplasia/patologia , Lábio , Proliferação de Células
8.
J Med Case Rep ; 17(1): 22, 2023 Jan 23.
Artigo em Inglês | MEDLINE | ID: mdl-36683067

RESUMO

BACKGROUND: Filar cysts are frequently found on neonatal ultrasound and are physiologically involuting structures with natural resolution. Hence, there has been no previous histologic correlation. Ventriculus terminalis is a focal central canal dilation in the conus medullaris and usually not clinically significant. Extra-axial cyst at the conus-filum junction connected to ventriculus terminalis is extremely rare, especially when associated with tethered lipomatous filum terminale and with progressive cyst enlargement. CASE PRESENTATION: A Caucasian female neonate with abnormal gluteal cleft had ventriculus terminalis cyst with an extra-axial cyst at the conus-filar junction and taut lipomatous filum on ultrasound examination and magnetic resonance imaging. This persisted at 6-month follow up imaging. In light of the nonresolving extra-axial mass and thick taut lipomatous filum, the child underwent L1-L3 osteoplastic laminectomies. The extra-axial cyst expanded after bony decompression and furthermore on dural opening; visualized on ultrasound. It communicated with the central canal and was documented with intraoperative photomicrographs. It was excised and filum sectioned. Histological immunostaining of the cyst wall showed neuroglial and axonal elements. The child did well without deficits at 4-year follow up with normal urodynamics. CONCLUSION: Progression dilation of ventriculus terminalis and extra-axial conofilar cyst with tethered lipomatous filum will likely progress to clinical significance and require surgical intervention. The embryologic basis for this pathology is discussed, with literature review.


Assuntos
Cauda Equina , Cistos , Criança , Recém-Nascido , Animais , Humanos , Lactente , Feminino , Moela das Aves , Medula Espinal/patologia , Cistos/diagnóstico por imagem , Cistos/cirurgia , Dilatação Patológica/patologia , Imageamento por Ressonância Magnética
9.
Angiology ; 74(9): 876-880, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-36113406

RESUMO

Coronary artery ectasia (CAE) is defined as abnormal dilation of a coronary artery ≥1.5 times the normal segment. We aimed to determine the prevalence and clinical predictors of CAE. This was a prospective analysis performed on 6465 patients undergoing coronary angiography. Patients were divided based on the presence or absence of CAE and compared for angiographic characteristics and clinical risk factors. The prevalence of CAE was 7%, CAE associated with coronary artery stenosis was 5.4%, and isolated CAE was 1.6%. The mean age of presentation in CAE patients was 60 years, with male predominance (83.8%) and stable angina was the most common presentation. The left anterior descending artery (LAD) (51.7%) was the most commonly involved vessel, with diffuse ectasia more commonly seen in right coronary artery and discrete ectasia in LAD. Type 4 CAE was the most common type (92.4%). Hypertension, diabetes, smoking, dyslipidemia, and obesity were found in 62.4%, 35.3%, 45.3%, 54.9%, and 23.3%, respectively in CAE patients, with significant association with smoking (Odds Ratio = 3.06). The prevalence of CAE was 7% and was frequently associated with atherosclerotic coronary disease. Smoking was a significant predisposing factor for CAE.


Assuntos
Doença das Coronárias , Vasos Coronários , Dilatação Patológica/diagnóstico por imagem , Dilatação Patológica/epidemiologia , Dilatação Patológica/patologia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/patologia , Angiografia Coronária , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Prevalência , Fatores Sexuais , Doença das Coronárias/diagnóstico por imagem , Doença das Coronárias/epidemiologia , Estudos Prospectivos , Índia/epidemiologia
10.
Fetal Pediatr Pathol ; 42(1): 137-143, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35438035

RESUMO

Objective: Congenital segmental intestinal dilatation (SID) and bronchogenic cyst in the abdomen are two uncommon and different pathologies. We report a bronchogenic cyst associated with segmental intestinal dilatation. Case: A 2-day-old 3300 g term infant developed bilious vomiting. A jejunoileal segment with a diameter of 10 cm was detected at surgery. Histologically, the wall musculature and enteric plexus of the segmentally enlarged small intestine stained normally for CD117 and negative for calretinin. A bronchogenic cyst of 3 cm in diameter was centered on the mesenteric border of the dilated intestine. Conclusion: SID has a normal staining pattern for CD117 (for interstitial cells of Cajal) and negative for calretinin. it would suggest that the innervation is defective, may be associated with a bronchogenic cyst in the newborn, causing obstruction, requiring surgery.


Assuntos
Cisto Broncogênico , Lactente , Recém-Nascido , Humanos , Calbindina 2 , Cisto Broncogênico/patologia , Dilatação , Íleo/anormalidades , Íleo/patologia , Íleo/cirurgia , Intestino Delgado , Dilatação Patológica/congênito , Dilatação Patológica/patologia
11.
Angiology ; 74(6): 563-568, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36039654

RESUMO

Epicardial fat may play an important role in the pathogenesis of coronary artery disease (CAD). We investigated the relationship between coronary artery ectasia (CAE) and epicardial fat volume (EFV). This retrospective study included 506 patients with CAE (group 1), 500 with CAD (group 2), and 500 patients with normal coronaries as controls (group 3). The pericardium was traced manually from the edge of the pulmonary trunk to the last measured by computed tomography slice containing images of the heart to obtain a region of interest. EFV was significantly higher in patients with CAD than in those with CAE (87.94 ± 22.18 vs 61.33 ± 12.75 mL; P < .001). Patients with normal coronaries had EFV of 56.62 ± 9.82 mL. Multivariate logistic regression analysis showed that male gender [Odds ratio (OR) (95% confidence interval (CI)): 1.220 (1.015-1.682), P = .042], diabetes [OR (95% CI): 1.036 (1.008-1.057); P = .002], and smoking [OR (95% CI): 3.043 (1.022-9.462); P = .005] were significantly associated with CAE. The receiver operating characteristic (ROC) curve showed that EFV had strongest diagnostic value for detecting CAD rather than CAE [AUC .502 P = .074 (95% CI: .311-.784)]. This study demonstrated that EFV is an independent predictor for CAE and CAD. However, sensitivity and specificity for detecting CAE is low when compared with CAD.


Assuntos
Aneurisma Coronário , Doença da Artéria Coronariana , Humanos , Masculino , Doença da Artéria Coronariana/diagnóstico , Estudos Retrospectivos , Dilatação Patológica/patologia , Vasos Coronários/patologia , Pericárdio/diagnóstico por imagem , Pericárdio/patologia , Angiografia Coronária
12.
Acta Cardiol ; 77(10): 943-949, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36189879

RESUMO

BACKGROUND: Para-aortic adipose tissue (PAT) is the local adipose tissue that externally surrounds the aorta. It contributes significantly to aortic atherosclerosis and enlargement. Studies conducted with computed tomography and magnetic resonance have shown that individuals with aortic aneurysm had more PAT than healthy individuals. In this study, we measured PAT using transthoracic echocardiography (TTE). The aim of this study is to investigate the possible relationship of TTE measured PAT with ascending aortic width. METHODS: PAT was defined as the hypoechoic space in front of ascending aortic 2 cm above the sinotubular junction at the end of the systole. Patients were divided into 2 groups according to the presence of dilatation in the ascending aorta using Roman's classification (aortic size index, ASI). ASI of less than 21 was considered no aortic dilation and an ASI of 21 mm/m2 or greater was considered to have aortic dilation. RESULTS: A total of 321 unselected patients were divided into the ascending aortic dilatation (AAD) group (n = 96) and the normal ascending aorta diameter group (n = 225 patients). PAT was significantly higher in the AAD group compared with the non-ADD group (0.9 (0.48) vs. 0.7 (0.91) mm, p < 0.0001). Univariate and multivariate logistic regression analysis revealed that PAT (OR: 3.005, 95%CI (1.445-6.251)) were significantly associated with AAD. CONCLUSIONS: This is the first study which evaluated PAT measured by TTE. We found a significant association between PAT measured by TTE and ascending aorta width.


Assuntos
Aneurisma Aórtico , Doenças da Aorta , Humanos , Aorta/diagnóstico por imagem , Aneurisma Aórtico/diagnóstico por imagem , Ecocardiografia/métodos , Tecido Adiposo/diagnóstico por imagem , Dilatação Patológica/patologia
13.
Curr Cardiol Rep ; 24(8): 1021-1030, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35867195

RESUMO

PURPOSE OF REVIEW: Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. It has a wide spectrum of clinical manifestations including aortic regurgitation (AR), aortic stenosis, and an associated aortopathy with a small but increased risk of aortic dissection. This review describes current knowledge of BAV, from anatomy and genetics to a discussion of multifaceted strategies utilized in the management of this unique patient population. This review will also highlight critical knowledge gaps in areas of basic and clinical research to enhance further understanding of this clinical entity. RECENT FINDINGS: The current knowledge regarding pathophysiologic mechanisms, screening, and surveillance guidelines for BAV and the associated aortopathy is discussed. We also discuss current management techniques for aortic valve repair versus replacement, indications for aortic surgery (root or ascending aorta), and the emergence of the Ross procedure as a viable management option not only in children, but also in adolescents and adults. The varied clinical phenotype of the BAV, resulting in its specific complex hemodynamic interactions, renders it an entity which is separate and distinct from the tricuspid aortic valve pathologies. While various aortic histopathologic and protein alterations in BAV patients have been described, it remains unclear if these changes are causal or the result of hemodynamic alterations imposed by sheer stress on the intrinsically dysfunctional BAV. Medical management for patients with BAV with AS, AI, or dilated aortic roots/ascending aortas remains challenging and needs further investigation. More than 50% of patients with BAV will undergo AVR during their lifetime, and more than 25% of patients with BAV undergo aortic surgery performed for dilation of the aortic root or ascending aorta, often concurrently with AVR. The search for the ultimate genetic or epigenetic cause of the different bicuspid phenotypes will ultimately be facilitated by the next-generation sequencing tools that allow for study of large populations at low cost. Improvements in diagnostic and stratification criteria to accurately risk assess BAV patients are critical to this process.


Assuntos
Insuficiência da Valva Aórtica , Estenose da Valva Aórtica , Doença da Válvula Aórtica Bicúspide , Doenças das Valvas Cardíacas , Valva Aórtica/patologia , Insuficiência da Valva Aórtica/etiologia , Estenose da Valva Aórtica/cirurgia , Dilatação Patológica/patologia , Dilatação Patológica/cirurgia , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/genética , Doenças das Valvas Cardíacas/cirurgia , Humanos
14.
Clin Chim Acta ; 534: 93-105, 2022 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-35853548

RESUMO

BACKGROUND: Coronary artery ectasia (CAE) is a cardiovascular disorder characterized by abnormal coronary artery dilation and disturbed coronary flow. The exact pathophysiology of CAE is still unclear. We aimed to investigate differences in metabolomic profiles between CAE patients and healthy controls. METHODS: Radial artery blood samples were collected from 14 pure CAE patients, 12 mixed CAE patients with atherosclerosis, and 14 controls with normal angiography. Differential serum metabolites were analyzed by untargeted ultra-high performance liquid chromatography-mass spectrometry. Serum ICAM-1, VEGF, ROS, and glutathione levels were also measured. RESULTS: Ten metabolites distinguished pure CAE patients from controls and mixed CAE, including 1-cyano-2-hydroxy-3-butene, 2,3-dihydro-6-methyl-5-(5-methyl-2-furanyl)-1H-pyrrolizine, 2-propionylpyrrole, 2-pyrrolidinone, 3-(2-furanylmethylene)pyrrolidine, D-alanine, furanofukinin, o-ethyltoluene, rotundine A, and SM(d18:1/18:1(9Z)). Related metabolic pathways include amino acid metabolism, sphingolipid dysfunction, energy metabolism, mitochondrial dysfunction, and oxidative stress. Serum concentrations of ICAM-1, VEGF and ROS were significantly elevated in CAE patients compared to controls, while glutathione decreased significantly in CAE patients. Moreover, ICAM-1 levels were negatively correlated with 2-propionylpyrrole, and VEGF levels were negatively correlated with SM(d18:1/18:1(9Z)), while GSH and ROS levels were correlated with the abundance of SM(d18:1/18:1(9Z)), further confirming systemic inflammation and oxidative stress in CAE. CONCLUSIONS: This is the first report describing differential serum metabolomic profiles of pure CAE patients compared to mixed CAE and healthy controls, which revealed 10 potential biomarkers that can provide an early diagnosis of pure CAE. These discriminatory metabolites and related metabolic pathways can help to better understand the pathogenesis of pure CAE.


Assuntos
Doença da Artéria Coronariana , Vasos Coronários , Estudos de Casos e Controles , Angiografia Coronária , Vasos Coronários/metabolismo , Dilatação Patológica/metabolismo , Dilatação Patológica/patologia , Glutationa/metabolismo , Humanos , Molécula 1 de Adesão Intercelular , Espécies Reativas de Oxigênio/metabolismo , Fator A de Crescimento do Endotélio Vascular/metabolismo
15.
Arterioscler Thromb Vasc Biol ; 42(8): 973-986, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35770665

RESUMO

BACKGROUND: Thoracic aortopathy associates with extracellular matrix remodeling and altered biomechanical properties. We sought to quantify the natural history of thoracic aortopathy in a common mouse model and to correlate measures of wall remodeling such as aortic dilatation or localized mural defects with evolving microstructural composition and biomechanical properties of the wall. METHODS: We combined a high-resolution multimodality imaging approach (panoramic digital image correlation and optical coherence tomography) with histopathologic examinations and biaxial mechanical testing to correlate spatially, for the first time, macroscopic mural defects and medial degeneration within the ascending aorta with local changes in aortic wall composition and mechanical properties. RESULTS: Findings revealed strong correlations between local decreases in elastic energy storage and increases in circumferential material stiffness with increasing proximal aortic diameter and especially mural defect size. Mural defects tended to exhibit a pronounced biomechanical dysfunction that is driven by an altered organization of collagen and elastic fibers. CONCLUSIONS: While aneurysmal dilatation is often observed within particular segments of the aorta, dissection and rupture initiate as highly localized mechanical failures. We show that wall composition and material properties are compromised in regions of local mural defects, which further increases the dilatation and overall structural vulnerability of the wall. Identification of therapies focused on promoting robust collagen accumulation may protect the wall from these vulnerabilities and limit the incidence of dissection and rupture.


Assuntos
Angiotensina II , Aneurisma da Aorta Torácica , Animais , Aorta Torácica/patologia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/genética , Aneurisma da Aorta Torácica/patologia , Fenômenos Biomecânicos , Colágeno , Dilatação , Dilatação Patológica/patologia , Camundongos
16.
Int J Mol Sci ; 23(12)2022 Jun 17.
Artigo em Inglês | MEDLINE | ID: mdl-35743192

RESUMO

Lysyl oxidase (LOX) is a copper-binding enzyme that cross-links elastin and collagen. The dominant LOX variation contributes to familial thoracic aortic aneurysm. Previously reported murine Lox mutants had a mild phenotype and did not dilate without drug-induced provocation. Here, we present a new, more severe mutant, Loxb2b370.2Clo (c.G854T; p.Cys285Phe), whose mutation falls just N-terminal to the copper-binding domain. Unlike the other mutants, the C285F Lox protein was stably produced/secreted, and male C57Bl/6J Lox+/C285F mice exhibit increased systolic blood pressure (BP; p < 0.05) and reduced caliber aortas (p < 0.01 at 100mmHg) at 3 months that independently dilate by 6 months (p < 0.0001). Multimodal imaging reveals markedly irregular elastic sheets in the mutant (p = 2.8 × 10−8 for breaks by histology) that become increasingly disrupted with age (p < 0.05) and breeding into a high BP background (p = 6.8 × 10−4). Aortic dilation was amplified in males vs. females (p < 0.0001 at 100mmHg) and ameliorated by castration. The transcriptome of young Lox mutants showed alteration in dexamethasone (p = 9.83 × 10−30) and TGFß-responsive genes (p = 7.42 × 10−29), and aortas from older C57Bl/6J Lox+/C285F mice showed both enhanced susceptibility to elastase (p < 0.01 by ANOVA) and increased deposition of aggrecan (p < 0.05). These findings suggest that the secreted Lox+/C285F mutants produce dysfunctional elastic fibers that show increased susceptibility to proteolytic damage. Over time, the progressive weakening of the connective tissue, modified by sex and blood pressure, leads to worsening aortic disease.


Assuntos
Tecido Elástico , Proteína-Lisina 6-Oxidase , Animais , Aorta/metabolismo , Pressão Sanguínea , Cobre , Dilatação Patológica/patologia , Tecido Elástico/metabolismo , Feminino , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Proteína-Lisina 6-Oxidase/genética , Proteína-Lisina 6-Oxidase/metabolismo
17.
Pan Afr Med J ; 41: 204, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35685111

RESUMO

Caroli´s disease is a congenital hepatic disorder characterized by nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts with the absence of congenital hepatic fibrosis. Caroli´s disease is rare, with few reported cases in the literature, making it hard to distinguish from other liver abnormalities. We present a case of Caroli´s disease discovered indecently in a 16-year-old female who presented with recurrent abdominal pain and intermittent jaundice in the last three years. Abdominal Computed tomography (CT) showed mild liver enlargement with multiple cystic dilatations of the intrahepatic saccular bile ducts cystic dilatations without hepatic fibrosis. The patient was treated conservatively with ursodeoxycholic acid and antibiotic therapy and discharged with regular follow-up. In conclusion, Caroli´s disease should be considered in the differential diagnosis in patients with recurrent abdominal pain and cholangitis without risk factors or relevant history.


Assuntos
Doença de Caroli , Colangite , Hepatopatias , Dor Abdominal/complicações , Adolescente , Ductos Biliares Intra-Hepáticos/patologia , Doença de Caroli/complicações , Doença de Caroli/diagnóstico , Doença de Caroli/terapia , Colangite/diagnóstico , Dilatação Patológica/patologia , Feminino , Humanos , Cirrose Hepática/complicações , Hepatopatias/patologia
18.
Eur Radiol ; 32(12): 8285-8295, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35726102

RESUMO

OBJECTIVES: To identify useful features to predict hidden pancreatic malignancies in patients with main pancreatic duct (MPD) abrupt cutoff and dilatation, but without visible focal pancreatic lesions on CT. METHODS: This retrospective study included 92 patients (mean age, 63.4 ± 10.6 years, 63 men and 29 women) with MPD abrupt cutoff and dilatation, but without visible focal pancreatic lesion on contrast-enhanced CT between 2009 and 2021. Two radiologists independently evaluated the CT imaging features. Multivariable logistic regression analysis was performed to identify clinical and CT imaging features for hidden pancreatic malignancies. A nomogram was developed based on these results and assessed its performance. RESULTS: Thirty-eight (41.3%) and 54 (58.7%) were classified into the malignant and benign groups, respectively. In the multivariable analysis, CA19-9 elevation (odds ratio [OR] 7.5, p = 0.003), duct cutoff site at the head/neck (OR 7.6, p = 0.006), parenchymal contour abnormality at the duct cutoff site (OR 13.7, p < 0.001), and presence of acute pancreatitis (OR 11.5, p = 0.005) were independent predictors of pancreatic malignancy. A combination of any two significant features showed an accuracy of 77.2%, and a combination of any three features exhibited a specificity of 100%. The CT-based nomogram showed an area under the curve (AUC) of 0.84 (95% confidence interval, 0.77-0.90). CONCLUSIONS: The three CT imaging features and CA19-9 elevation translated into a nomogram permit a reliable estimation of hidden pancreatic malignancies in patients with MPD abrupt cutoff without visible focal pancreatic lesion. It may facilitate determining whether to proceed to further diagnostic tests. KEY POINTS: • Isoattenuating pancreatic ductal adenocarcinoma can manifest only as an isolated main pancreatic duct (MPD) dilatation with abrupt cutoff, making it difficult to distinguish from benign strictures. • Along with the serum CA 19-9 elevation, MPD cutoff site at the pancreas head or neck, parenchymal contour abnormality at the duct cutoff site, and associated acute pancreatitis indicated a higher probability of the malignant MPD strictures. • The CT-based nomogram provided excellent diagnostic performance (AUC of 0.84) for hidden pancreatic malignancies in patients with MPD abrupt cutoff and dilatation.


Assuntos
Carcinoma Ductal Pancreático , Neoplasias Pancreáticas , Pancreatite , Masculino , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Nomogramas , Antígeno CA-19-9 , Constrição Patológica/patologia , Estudos Retrospectivos , Dilatação , Doença Aguda , Pancreatite/diagnóstico , Tomografia Computadorizada por Raios X/métodos , Ductos Pancreáticos/diagnóstico por imagem , Ductos Pancreáticos/patologia , Carcinoma Ductal Pancreático/diagnóstico , Neoplasias Pancreáticas/patologia , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Dilatação Patológica/patologia , Neoplasias Pancreáticas
19.
Rom J Ophthalmol ; 66(1): 89-96, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35531451

RESUMO

We present the difficulties in choosing the right IOL, when facing a great variability of the keratometric measurements, in the case of a patient operated for epiretinal membrane and lamellar macular hole, who developed complicated cataract in the operated eye. Upon commencing the biometric measurements, inconsistency in keratometric values led to further investigations. Repeated placido disc topography initially showed corneal ectasia, which posed a problem on selecting the right type of intra-ocular lens. Ocular surface pathology was suspected, and after treatment, the topography was repeated with a Scheimpflug topographer, that showed an improved keratometric profile. The surgical solution was to implant an aspheric monofocal IOL, in the bag, with extended depth of focus that enhances intermediate vision, disregarding the previous keratometric measurements. Refractive and functional outcomes were good. In cases of biometric measurements that show inconsistency in keratometric values, ocular surface disease as well as corneal ectasia should be taken into consideration. The right implant should not be chosen based on a single measurement, but rather several measurements should be made and compared and the choice should not be made before treating the ocular surface.


Assuntos
Lentes Intraoculares , Facoemulsificação , Córnea/patologia , Topografia da Córnea , Dilatação Patológica/patologia , Dilatação Patológica/cirurgia , Humanos , Implante de Lente Intraocular , Lentes Intraoculares/efeitos adversos , Refração Ocular
20.
Kyobu Geka ; 75(5): 396-399, 2022 May.
Artigo em Japonês | MEDLINE | ID: mdl-35474207

RESUMO

We report a case of huge aortic root aneurysm 19 years after aortic valve replacement. A 58-year-old woman was referred to us for aortic root dilatation. She underwent aortic valve replacement with mechanical valve due to severe aortic regurgitation of tricuspid aortic valve 19 years ago. We performed graft replacement of aortic root preserving prior mechanical prosthetic valve. Histological examination of aneurysm showed myxomatous change in medial layer. Aortic root dilatation after aortic valve replacement is caused by bicuspid valve, aortic dissection, Marfan syndrome, tissue disorder disease, aortitis, and dilated Valsalva sinus at the time of prior operation. It is important to follow-up a patient for root dilatation after aortic valve replacement.


Assuntos
Aneurisma da Aorta Torácica , Insuficiência da Valva Aórtica , Próteses Valvulares Cardíacas , Seio Aórtico , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/etiologia , Aneurisma da Aorta Torácica/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/etiologia , Insuficiência da Valva Aórtica/cirurgia , Dilatação Patológica/complicações , Dilatação Patológica/patologia , Feminino , Próteses Valvulares Cardíacas/efeitos adversos , Humanos , Pessoa de Meia-Idade , Seio Aórtico/diagnóstico por imagem , Seio Aórtico/cirurgia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...