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Introduction Sicca syndrome, characterized by xerophthalmia and xerostomia, is associated with various autoimmune and non-autoimmune conditions, posing diagnostic challenges. Sjögren's syndrome (SS) is the most prevalent systemic autoimmune disease linked to sicca symptoms. This study evaluates the diagnostic accuracy of salivary gland scintigraphy (SGS) in distinguishing SS from non-Sjögren's sicca conditions, alongside other diagnostic tests. Methods A retrospective analysis was conducted at Hospital Universitario La Paz from December 2019 to March 2023, including 142 patients diagnosed with sicca syndrome. Correlations between qualitative and quantitative SGS data (GE Healthcare, Chicago, Illinois) and multiparametric sicca evaluations were assessed. Results Among the 142 patients, 84 (59.15%) were classified as having SS, with 55 (65.48%) seropositive for anti-Ro antibodies. Abnormal SGS results were found in 135 (95.07%) patients. Qualitative SGS categorized seven (4.93%) as mild, 53 (37.32%) as moderate, 50 (35.21%) as severe, and 21 (14.79%) as functionally annulled. Moderate or worse impairment had a sensitivity of 0.88 and a specificity of 0.17. Functional annulment had a sensitivity of 0.17 and a specificity of 0.97. Quantitative SGS using ejection fraction thresholds of ≤30% and ≤20% had sensitivities of 0.35 and 0.18 and specificities of 0.84 and 0.94, respectively. Quantitative SGS metrics correlated with unstimulated whole salivary flow (WUSF; 0.243; p=0.003) and inversely with lymphocytic infiltration (-0.281; p=0.001). The 2016 American College of Rheumatology/European League Against Rheumatism (ACR-EULAR) classification criteria for Sjögren's syndrome demonstrated an area under the curve (AUC) of 0.932, which improved to 0.951 with the inclusion of SGS parameters. Conclusions SGS is a significant diagnostic tool in the multiparametric evaluation of sicca syndrome, showing strong correlations with histological and immunological markers. Its integration into diagnostic criteria enhances the differentiation between SS and non-Sjögren's sicca conditions, suggesting its potential inclusion in future classification frameworks.
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INTRODUCTION: 85% of lung cancers are non-small cell carcinomas (NSCLC), the majority of which are diagnosed in an advanced stage. Immunotherapy has changed the treatment pattern for these tumors and created the need to find a marker for patient selection. Although not ideal, PD-L1 is the biomarker currently used in clinical practice. MATERIAL AND METHODS: Retrospective review by two pathologists of 53 cases of NSCLC from 2005 to 2007 in Hospital Universitario La Paz, using the WHO 2015 classification studying PD-L1 with clones 22C3 and 28-8. The consistency between observers and clones was assessed and all data studied were correlated with survival rates. RESULTS: We found a prevalence of PD-L1 expression in tumor cells (TC) similar to that previously reported in the literature and a very good consistency between clones in the evaluation of TC and immune cells (ICC 0.99-0.93, p<.001). Interobserver concordance was very good in the evaluation of TC (ICC 0.902, 95% CI: 0.836-0.942, p<.001 for clone 22C3 and ICC 0.927, 95% CI: 0.877-0.957, p<.001 for clone 28-8) and poor for immune cells (ICC of 0.413, 95% CI: 0.163-0.613, p=.001 with clone 22C3 and ICC of 0.313, 95% CI: 0.053-0.534, p=.010 with clone 28-8). Subtype and histological grade were the only variables related to prognosis. CONCLUSIONS: The clones of PD-L1 22C3 and 28-8 are equivalent and there is good interobserver consistency in the evaluation of TC but not in immune cells.
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Antígeno B7-H1/análise , Biomarcadores Tumorais/análise , Carcinoma Pulmonar de Células não Pequenas/química , Neoplasias Pulmonares/química , Adenocarcinoma/química , Adenocarcinoma/classificação , Adenocarcinoma/patologia , Adenocarcinoma/terapia , Idoso , Carcinoma Pulmonar de Células não Pequenas/classificação , Carcinoma Pulmonar de Células não Pequenas/patologia , Carcinoma Pulmonar de Células não Pequenas/terapia , Carcinoma de Células Escamosas/química , Carcinoma de Células Escamosas/classificação , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/terapia , Feminino , Humanos , Imunidade Celular , Imunoterapia , Neoplasias Pulmonares/classificação , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/terapia , Masculino , Gradação de Tumores , Patologistas , Seleção de Pacientes , Prognóstico , Estudos RetrospectivosRESUMO
CLINICAL INTRODUCTION: A previously healthy 12-year-old boy presented with chest pain and dyspnoea. ECG (figure 1), echocardiogram (figure 2) and myocardial MRI (figure 3) were performed.Electromyography revealed mild proximal weakness. Blood tests showed creatine kinase 997â UI/L, aspartate transaminase 398â UI/L, alanine transaminase 293â UI/L and lactate deshidrogenase 1730â UI/L. Ophthalmology test showed diffuse loss of pigment in the retinal pigment epithelium. Myocardial biopsy was performed (figure 4). A cardiomyopathy was suspected. LAMP2 gene was sequenced, and a stop mutation was identified. QUESTION: What is the most likely diagnosis suggested based on the patient's tests and history? Danon's disease.Fabry's disease.Hypertrophic cardiomyopathy.Noonan's syndrome.Pompe's disease.
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Angina Pectoris/etiologia , Dispneia/etiologia , Doença de Depósito de Glicogênio Tipo IIb/complicações , Hipertrofia Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/etiologia , Biópsia , Criança , Análise Mutacional de DNA , Ecocardiografia , Eletrocardiografia , Doença de Depósito de Glicogênio Tipo IIb/diagnóstico , Doença de Depósito de Glicogênio Tipo IIb/genética , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico , Hipertrofia Ventricular Esquerda/fisiopatologia , Proteína 2 de Membrana Associada ao Lisossomo/genética , Imageamento por Ressonância Magnética , Masculino , Mutação , Volume Sistólico , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Remodelação VentricularRESUMO
OBJECTIVES: The main challenge in treating bisphosphonate-related osteonecrosis of the jaw (BRONJ) is the absence of an effective established treatment. We aimed to compare different potentially preventive treatments for BRONJ after dental extractions in zoledronic acid (ZA)-treated animals. We studied the local application of different combinations of adipose-derived stem cells (ASCs) with or without previous stimulation with bone morphogenetic protein 2 (BMP-2) and platelet-rich plasma (PRP) in rats. MATERIAL AND METHODS: Fifty-six male Wistar rats were treated with ZA for 9 weeks. Dental extractions were performed in the eighth week, and the animals were divided into 4 groups. In group 1 (n = 14), alveolar coverage with mucoperiosteal flap was performed. In group 2 (n = 14), PRP was applied over the sockets and covered with the flap. In group 3 (n = 15), allogeneic ASCs with PRP were applied and covered with the flap. In group 4 (n = 13), animals were treated with ASCs cultured with BMP-2, PRP, and flap coverage. Histologic, fluorescence, and radiologic studies of the maxillae were performed. RESULTS: ASC-treated animals showed lower frequency of osteonecrosis (14% vs 50%, p = 0.007) and greater bone turnover (p = 0.024) and osteoclast count (p = 0.045) than those not receiving the ASC treatment. CONCLUSIONS: In this high-risk model, ASC-based treatments seem to prevent BRONJ more effectively than mucosal flap with or without PRP. The combination of ASCs and PRP appears to be synergistic, and the addition of BMP-2 could further improve the results.
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Osteonecrose da Arcada Osseodentária Associada a Difosfonatos/terapia , Conservadores da Densidade Óssea/uso terapêutico , Difosfonatos/uso terapêutico , Imidazóis/uso terapêutico , Plasma Rico em Plaquetas/metabolismo , Adipócitos , Animais , Osteonecrose da Arcada Osseodentária Associada a Difosfonatos/prevenção & controle , Conservadores da Densidade Óssea/administração & dosagem , Difosfonatos/administração & dosagem , Modelos Animais de Doenças , Imidazóis/administração & dosagem , Masculino , Ratos , Ratos Wistar , Células-Tronco , Ácido ZoledrônicoRESUMO
We describe the case of a female patient with a previous diagnosis of primary biliary cirrhosis (PBC) and chronic hepatitis B in inactive phase who developed increased transaminase levels with no evidence of hepatitis B virus reactivation while receiving ursodeoxycholic acid treatment. A liver biopsy showed changes compatible with overlapping autoimmune hepatitis (AIH). Budesonide treatment achieved normalization of transaminase levels. We provide a review of PBC and AIH overlap syndrome and discuss the particular features of this case that led us to this diagnosis, as well as the treatment provided.