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OBJECTIVES: We aimed to assess first degree relatives (FDRs) of BD patients for the presence of clinical symptoms and signs of BD and evaluate common femoral vein (CFV) wall thickness measurement for the diagnosis. METHODS: Patients with BD(n=129) and FDRs(n=230) of these patients were included. FDRs were questioned in terms of BD symptoms by phone. Pathergy test and CFV wall thickness measurement were performed among 111 FDRs who accepted the clinical assessment. Clinical assessment group were classified according to the criteria sets for BD. FDRs who did not meet the criteria sets and had at least one clinical finding in addition to oral aphthae(OA) were categorized as the "suspected BD". RESULTS: :We observed increased frequency of isolated BD manifestations in FDRs. Ten FDRs were diagnosed with BD during clinical evaluation. Significantly increased CFV wall thickness was observed in FDRs of BD patients fulfilling diagnostic BD criteria (p<0.001 for both sides) and also in those with suspected BD group (p<0.05 for both sides). Presence of OA, genital ulcer, folliculitis or erythema nodosum were associated with increased CFV wall thickness (p<0.05). CONCLUSION: . Our results suggest that CFV wall thickness measurement can be used in diagnosis of familial BD.
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BACKGROUND: It is essential to determine whether bone marrow signal changes on magnetic resonance imaging (MRI) represent a physiological response or pathology; at present, the clinical significance of these signal changes is unclear. It is unknown whether a bone marrow biopsy is required when bone marrow signal changes are detected incidentally in individuals without suspected malignancy. OBJECTIVE: The primary purpose of this study was to determine whether incidentally detected bone marrow signal changes on MRI performed for various reasons (at the time of admission or during follow-up) are clinically significant. METHODS: We retrospectively evaluated the bone marrow biopsy clinical and laboratory findings of 42 patients with incidental bone marrow signal changes on MRI between September 2016 and January 2020. We also determined whether the patients were diagnosed with malignancy during admission or follow-up. RESULTS: Of the 42 patients, three (7%) were diagnosed with hematological malignancies during admission, while two were diagnosed with multiple myeloma and one with B-cell acute lymphoblastic leukemia. Of the 42 patients, 35 had a mean follow-up of 40.6 ± 5.3 months. One patient was diagnosed with monoclonal gammopathy of undetermined significance four months after their first admission. CONCLUSIONS: In addition to MRI, detailed clinical and laboratory evaluations should be performed to inform the decision for bone marrow biopsy and exclude hematological malignancy. If there is any doubt, a bone marrow biopsy should be performed. Moreover, since bone marrow signal changes may be a preliminary finding, follow-up of these patients is essential.
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OBJECTIVES: We recently reported the first controlled Doppler ultrasonography (US) study demonstrating increased common femoral vein (CFV) thickness in Behçet's Disease (BD). Standard lower extremity venous Doppler US is performed in erect position. In order to confirm accuracy and applicability of method, we measured CFV thickness in both supine and standing positions in this study. METHOD: We included sex and age-matched 42 BD patients and 41 healthy controls (HCs). After routine visits, bilateral CFV thickness was measured with Doppler US both in supine and standing positions. RESULTS: Bilateral CFV thickness was significantly higher in BD than in HC. There were no statistically significant differences in measurements of CFV wall thickness between standing and supine positions in both groups. CONCLUSIONS: CFV measurement by Doppler US is a new and non-invasive diagnostic tool for the diagnosis of BD. Our study confirmed that patient position does not affect CFV wall thickness measurement for diagnosis of BD.
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Síndrome de Behçet , Veia Femoral , Posição Ortostática , Ultrassonografia Doppler , Humanos , Síndrome de Behçet/diagnóstico por imagem , Masculino , Feminino , Adulto , Decúbito Dorsal , Veia Femoral/diagnóstico por imagem , Pessoa de Meia-Idade , Estudos de Casos e ControlesRESUMO
BACKGROUND: CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and protein-losing enteropathy (CHAPLE) is an ultra-rare genetic disorder characterised by intestinal lymphatic damage, lymphangiectasia, and protein-losing enteropathy caused by overactivation of the complement system. We assessed the efficacy and safety of pozelimab, an antibody blocking complement component 5. METHODS: This open-label, single-arm, historically controlled, multicentre phase 2 and 3 study evaluated ten patients with CHAPLE disease. This study was conducted at three hospitals in Thailand, Türkiye, and the USA. Patients aged 1 year or older with a clinical diagnosis of CHAPLE disease and a CD55 loss-of-function variant identified by genetic analysis and confirmed by flow cytometry or western blot of CD55 from peripheral blood cells were eligible for this study. Patients received a single intravenous loading dose of pozelimab 30 mg per kg of bodyweight, followed by a once-per-week subcutaneous dose over the treatment period based on bodyweight at a concentration of 200 mg/mL as either a single injection (<40 kg bodyweight) or two injections (≥40 kg bodyweight). The primary endpoint was proportion of patients with serum albumin normalisation with an improvement in active clinical outcomes and no worsening in inactive clinical outcomes (frequency of problematic abdominal pain, bowel movement frequency, facial oedema severity, and peripheral oedema severity) at week 24 compared with baseline, assessed in the full analysis set. This study is registered with ClinicalTrials.gov (NCT04209634) and is active but not recruiting. FINDINGS: 11 patients were recruited between Jan 27, 2020, and May 12, 2021, ten of which were enrolled in the study and included in the analysis populations. The efficacy data corresponded to all patients completing the week 48 assessment and having at least 52 weeks of treatment exposure, and the safety data included an additional 90 days of follow-up and corresponded to all patients having at least 72 weeks of treatment. Patients were predominantly paediatric (with a median age of 8·5 years), and originated from Türkiye, Syria, Thailand, and Bolivia. Patients had markedly low weight-for-age and stature-for-age at baseline, and mean albumin at baseline was 2·2 g/dL, which was considerably less than the local laboratory reference range. After pozelimab treatment, all ten patients had serum albumin normalisation and improvement with no worsening in clinical outcomes. There was a complete inhibition of the total complement activity. Nine patients had adverse events; two were severe events, and one patient had an adverse event considered related to pozelimab. INTERPRETATION: Pozelimab inhibits complement overactivation and resolves the clinical and laboratory manifestations of CHAPLE disease. Pozelimab is the only currently approved therapeutic drug for patients with this life-threatening, ultra-rare condition. In patients with protein-losing enteropathy where known causes have been excluded, testing for a CD55 deficiency should be contemplated. A diagnosis of CHAPLE disease should lead to early consideration of treatment with pozelimab. FUNDING: Regeneron Pharmaceuticals and the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health.
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Enteropatias Perdedoras de Proteínas , Trombose , Criança , Humanos , Anticorpos Monoclonais , Edema , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Albumina Sérica , Resultado do Tratamento , Estudo Historicamente Controlado , Masculino , FemininoRESUMO
BACKGROUND: Colorectal cancer is a significant global health concern, ranking as the second most deadly and third most common cancer worldwide. Early detection and removal of precancerous lesions play a crucial role in preventing cancer development and reducing mortality. Since FDG uptake is not specific for malignancy, incidental increased FDG uptake in the gastrointestinal tract may be challenging to interpret and may require further colonoscopic examination. This study aimed to investigate the features associated with malignant and premalignant pathology in patients with incidental colonic FDG uptake and determine the necessity of colonoscopy for each FDG uptake. METHODS: Retrospective analysis was performed on data from patients who underwent colonoscopies between January 2016 and December 2021. Patients with FDG uptake in known colorectal malignancy regions were excluded. The study included 56 patients with incidental colonic FDG uptake. PET/CT images were visually and quantitatively analyzed, and the corresponding colonoscopy and histopathological results were recorded. Statistical analyses were conducted to evaluate the relationship between FDG uptake patterns, SUVmax values, and histopathological diagnoses. Colonoscopic findings were categorized as malignancy, polyps, and non-neoplastic lesions. RESULTS: Among the 56 patients with incidental colonic FDG uptake, 36 lesions were identified, and histopathology revealed malignancy in 10 (17.9%) patients and premalignant polyps in the 26 (46.4%) cases. Focal FDG uptake with corresponding wall thickening or soft tissue density on CT was associated with a higher likelihood of premalignant or malignant lesions. The SUVmax values demonstrated a significant difference between negative findings and polyps/malignancies. However, no significant difference was observed between malignant and premalignant lesions. A ROC curve analysis was made and assesed a cut-off value of 11.1 SUVmax (sensitivity: 83.3% and specificity: 90%) to distinguish premalignant or malignant lesions from non-malignant lesions. CONCLUSION: Incidental colonic FDG uptake with a focal pattern and corresponding CT findings were more likely to indicate premalignant or malignant lesions. SUVmax values were helpful in predicting the presence of pathological findings, but histopathological verification remains necessary for a definitive diagnosis. These findings contribute to our understanding of the clinical implications of incidental colonic FDG uptake and highlight the importance of follow-up colonoscopy for further evaluation.
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Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Lesões Pré-Cancerosas , Humanos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Fluordesoxiglucose F18 , Estudos Retrospectivos , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Tomografia Computadorizada por Raios X , Colonoscopia , Achados IncidentaisRESUMO
The histopathological data about vascular inflammation in Behçet's disease(BD) mainly comprises patients with arterial involvement. Inflammatory cell infiltration was mainly observed around the vasa vasorum and adventitial layer of the aneurysmatic vessels, and only a few cells were seen in the intimal layer during active arteritis. There is limited data for the histopathology of venous inflammation. We recently showed that increased common femoral vein(CFV) wall thickness is a specific sign of vein wall inflammation in BD. We aimed to investigate the different vein subsections measuring the whole wall and the intima-media thickness(IMT) of CFV with ultrasonography in BD. We found increased IMT of CFV compared to controls as well as CFV wall thickness. This study shows that there is a full layer venous wall inflammation in BD independent of vascular involvement. Our results suggest that venous endothelial inflammation may trigger the thickening of the vein wall and cause thrombotic tendency in BD.
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Síndrome de Behçet , Humanos , Síndrome de Behçet/diagnóstico por imagem , Espessura Intima-Media Carotídea , Veia Femoral/diagnóstico por imagem , Veia Femoral/patologia , Inflamação , Endotélio VascularRESUMO
OBJECTIVES: The thyroid imaging reporting and data system (TI-RADS) and 2015 American Thyroid Association (ATA) guidelines are two well-known risk stratification systems for classifying thyroid nodules based on cancer risk. This study aims to evaluate the diagnostic efficacy of these two systems in predicting malignancy in patients undergoing thyroid surgery. METHODS: We studied data on 120 individuals who were scheduled to undergo surgery for benign or malignant nodular diseases of the thyroid gland between October 2017 and October 2019. The TI-RADS category and ultrasound pattern based on ATA guidelines were assigned to dominant thyroid nodule categories by two experienced radiologists blinded to patients' previous thyroid ultrasonography and fine-needle aspiration biopsy results. A pathologist with experience in thyroid diseases blinded to patients' sonographic and clinical data reviewed the thyroidectomy specimens. RESULTS: A total of 120 patients, 88 women and 32 men, were included in our study. Final histopathological results were as follows: 50% (n=60) papillary thyroid carcinoma, 36.6% (n=44) benign nodular thyroid diseases, 4.1% (n=5) follicular adenoma, 2.5% (n=3) hurtle cell adenoma, 1.7% (n=2) follicular thyroid carcinoma, 1.7% (n=2) medullary thyroid carcinoma, 1.7% (n=2) hurtle cell carcinoma, and 1.7% (n=2) follicular tumor of uncertain malignancy potential. The sensitivity, specificity, positive predictive value, and negative predictive value for TI-RADS were 80%, 56%, 72%, and 67%, respectively, and that for ATA were 80%, 64%, 76%, and 69%, respectively. CONCLUSION: The TI-RADS and ATA showed similar rates of sensitivity, specificity, NPV, and PPV. Our observed risk of malignancy was higher than expected for the ACR TI-RADS 3-5 categories and the very low, low, and intermediate suspicion risk strata in the ATA guidelines. We found no difference between observed and expected malignancy risk for the ACR TI-RADS 2's and ATA's high suspicion categories.
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Carcinoma Neuroendócrino , Neoplasias da Glândula Tireoide , Nódulo da Glândula Tireoide , Masculino , Humanos , Feminino , Estados Unidos , Nódulo da Glândula Tireoide/diagnóstico por imagem , Nódulo da Glândula Tireoide/cirurgia , Sistemas de Dados , Neoplasias da Glândula Tireoide/diagnóstico por imagem , Neoplasias da Glândula Tireoide/patologia , Ultrassonografia/métodos , Estudos Retrospectivos , Medição de Risco/métodosRESUMO
INTRODUCTION: To investigate the acute inflammatory and structural changes of sacroiliitis as auxiliary findings on magnetic resonance enterography (MRE) and their presence on closely timed conventional magnetic resonance imaging of the sacroiliac joint (SI joint MRI). MATERIALS AND METHODS: We screened axial spondyloarthritis patients for the simultaneous presence of MREs and SI joint MRIs. Two blinded radiologists evaluated SI joint MRIs and MREs on two separate occasions. We used the Assessment of SpondyloArthritis International Society (ASAS)/Outcome Measures in Rheumatology Network (OMERACT) definitions for SI joint MRI. We implemented previously published standard definitions for osteitis, erosion, sclerosis, and fatty infiltration of SI joint in MREs that contain T1w and T1w post-gadolinium sequences. RESULTS: SI joint MRI and MRE images were present in 43 patients. The median time between the two modalities was 14 (0-89) days. Twelve patients had ASAS-defined positive SI joint MRI. Radiologist-1 and radiologist-2 detected osteitis on MRE in nine and eight out of these 12 patients, respectively. The two radiologists detected ankylosis and fatty metaplasia with a complete agreement and sclerosis with an almost perfect agreement. Both radiologists agreed on erosions on SI joint MRI in the same 10 cases. Radiologists did not identify acute inflammatory or structural changes on MRE in patients with a negative SI joint MRI for these lesions. CONCLUSION: Along with intestinal findings, additional reporting of acute inflammatory and structural changes of the SI joint on a MRE is valuable and may alert physicians to the presence of previously not diagnosed axial spondyloarthritis.
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Espondiloartrite Axial , Osteíte , Sacroileíte , Humanos , Sacroileíte/diagnóstico por imagem , Sacroileíte/patologia , Articulação Sacroilíaca/diagnóstico por imagem , Articulação Sacroilíaca/patologia , Osteíte/diagnóstico por imagem , Osteíte/patologia , Esclerose/patologia , Imageamento por Ressonância Magnética/métodosRESUMO
BACKGROUND: Recent findings suggest that fluoroquinolones, most prescribed antibiotic to treat various infections, have increased abdominal aortic aneurysm formation. We aimed to investigate the relation of the development of abdominal aortic aneurysm and the duration of ciprofloxacin use. METHODS: Male Sprague-Dawley rats were divided into 2 groups to administer saline to the control groups and CaCl2 to the aneurysm groups. These groups were then divided into 3 subgroups: intraperitoneal saline, ciprofloxacin for 2 weeks, and ciprofloxacin for 4 weeks. At the end of 4 weeks, the diameter of abdominal aorta was determined by ultrasonography and animals were sacrificed to obtain abdominal aorta specimens. Elastic fiber fracture, tunica media layer thickness, and aortic tissue damage were evaluated histologically. RESULTS: Aortic diameter of control-saline (2.15 mm ± 0.06), control-2 weeks (2.25 mm ± 0.06), and control-4 weeks (3.31 mm ± 0.09) ciprofloxacin groups was significantly different (P < .0001). Also, aortic diameter of aneurysm-saline (2.07mm ± 0.02), aneurysm-2 weeks ciprofloxacin (3.33 mm ± 0.64), and aneurysm-4 weeks ciprofloxacin (8.55 mm ± 1.70) groups showed significant increase in aortic diameter with increasing duration of ciprofloxacin use (P < .01). A significant difference was found between the control-saline (0.00 ± 0.00), control-2 weeks (1.50 ± 0.33), and control-4 weeks ciprofloxacin groups (1.57 ± 0.20) in the histological aneurysm scores (P < .001). Aortic tunica media thickness did not change between control-saline and control-ciprofloxaci n groups (P > .05). CONCLUSION: The study showed that ciprofloxacin caused injury in the aortic wall but not a significant change in the thickness of the aortic tunica media layer. The duration of ciprofloxacin use was important in the development of aneurysm and aneurysm severity.
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Aneurisma da Aorta Abdominal , Ratos , Animais , Masculino , Aneurisma da Aorta Abdominal/induzido quimicamente , Aneurisma da Aorta Abdominal/diagnóstico por imagem , Ratos Sprague-Dawley , Ciprofloxacina/uso terapêutico , Modelos Animais de Doenças , Aorta Abdominal/diagnóstico por imagem , Aorta Abdominal/patologiaRESUMO
BACKGROUND: Perianal disease is reported more widely in pediatric Crohn patients than in the past, and has been stated as an independent modifier of the disease behavior. In this study, we aimed to analyze the clinical characteristics and outcomes of fistulising perianal Crohn's disease (fpCD) in the pediatric age group. METHODS: A total number of 149 children with an established diagnosis of inflammatory bowel disease who have been diagnosed before 18 years of age and followed in our tertiary center were revised. Clinical, endoscopic, laboratory, and radiologic data of 50 patients with CD, who had at least 18 months follow-up data, were compiled. RESULTS: Of 50 patients, 26 (52%) were diagnosed as fpCD (38% at onset). More than half of the patients without any notable external orifices around the perianal area were diagnosed as fpCD by an magnetic resonance imaging (MRI). Pediatric fpCD patients had a higher disease activity score and platelet count, lower serum albumin level, and a higher rate of granuloma in the biopsy samples, compared with non-fistulising patients. A considerably high rate of surgical interventions (i.e., seton placement 46% and abscess drainage 15%) was performed in combination with infliximab. CONCLUSION: Fistulising perianal Crohn's disease seems to be more common than previously reported in the pediatric age group. A severe course of the disease might serve as a warning for the development of fpCD. A careful physical examination and use of perianal MRI with a high index of suspicion may increase the likelihood of fistula detection, hence may change the treatment strategy.
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Doença de Crohn , Fístula Retal , Criança , Doença de Crohn/terapia , Humanos , Fístula Retal/terapia , Resultado do TratamentoRESUMO
OBJECTIVE: Deep vein thrombosis (DVT) of the lower extremities is the most common form of vascular involvement in Behçet disease (BD), frequently leading to post-thrombotic syndrome (PTS) as a disabling complication. We have described the clinical characteristics and predictors of PTS presence among patients with BD and lower extremity DVT. We also used venous Doppler ultrasound (US) examinations in our assessment. METHODS: Patients with BD (n = 205; 166 men, 39 women; age 39 ± 9.5 years) and a history of DVT were investigated. The Villalta scale was used to assess the presence and severity of PTS. Doppler US examinations were performed within 1 week of the clinical evaluation. The total number of vessels with reflux, thrombi, recanalization, and collateral vessels were calculated. RESULTS: Of the 205 patients with BD, 62% had had PTS and 18% had had severe PTS. Patients with PTS had had greater reflux (P = .054) and thrombosis (P = .02) scores compared with patients without PTS. Treatment with anticoagulation (AC), immunosuppressive (IS) therapy, or AC combined with IS drugs did not affect the occurrence of PTS. However, patients treated with IS therapy, with or without AC drugs, had a decreased incidence of severe PTS compared with the AC-only group (P = .017). Patients treated with AC plus IS agents also had increased collateral scores compared with patients treated with only IS drugs. Interferon-α use seemed to provide better recanalization scores compared with azathioprine only (1.0 [range, 0-14] vs 2.5 [range, 0-10]; P = .010). CONCLUSIONS: Patients with BD and DVT have a high risk of developing severe PTS. IS treatment decreases the development of severe PTS. AC therapy might influence the course of PTS by increasing the collateral scores, and the use of interferon-α also increased recanalization scores. Routine assessment with Doppler US examinations could be helpful in the prediction of severe PTS.
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Síndrome de Behçet/complicações , Extremidade Inferior/irrigação sanguínea , Síndrome Pós-Trombótica/etiologia , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Medição de Risco , Índice de Gravidade de DoençaRESUMO
Complement hyperactivation, angiopathic thrombosis and protein-losing enteropathy (CHAPLE disease) is a lethal disease caused by genetic loss of the complement regulatory protein CD55, leading to overactivation of complement and innate immunity together with immunodeficiency due to immunoglobulin wasting in the intestine. We report in vivo human data accumulated using the complement C5 inhibitor eculizumab for the medical treatment of patients with CHAPLE disease. We observed cessation of gastrointestinal pathology together with restoration of normal immunity and metabolism. We found that patients rapidly renormalized immunoglobulin concentrations and other serum proteins as revealed by aptamer profiling, re-established a healthy gut microbiome, discontinued immunoglobulin replacement and other treatments and exhibited catch-up growth. Thus, we show that blockade of C5 by eculizumab effectively re-establishes regulation of the innate immune complement system to substantially reduce the pathophysiological manifestations of CD55 deficiency in humans.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Ativação do Complemento/efeitos dos fármacos , Complemento C5/antagonistas & inibidores , Inativadores do Complemento/uso terapêutico , Metabolismo Energético/efeitos dos fármacos , Hipoproteinemia/tratamento farmacológico , Imunidade Inata/efeitos dos fármacos , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Anticorpos Monoclonais Humanizados/efeitos adversos , Anticorpos Monoclonais Humanizados/farmacocinética , Biomarcadores/sangue , Antígenos CD55/deficiência , Antígenos CD55/genética , Complemento C5/metabolismo , Inativadores do Complemento/efeitos adversos , Inativadores do Complemento/farmacocinética , Predisposição Genética para Doença , Humanos , Hipoproteinemia/genética , Hipoproteinemia/imunologia , Hipoproteinemia/metabolismo , Mutação , Fenótipo , Enteropatias Perdedoras de Proteínas/genética , Enteropatias Perdedoras de Proteínas/imunologia , Enteropatias Perdedoras de Proteínas/metabolismo , Resultado do TratamentoRESUMO
OBJECTIVES: Diagnosing Behçet's disease (BD) is a challenge, especially in countries with a low prevalence. Recently, venous wall thickness (VWT) in lower extremities has been shown to be increased in BD patients. In this study, we aimed to investigate the diagnostic performance of common femoral vein (CFV) thickness measurement in BD and whether it can be used as a diagnostic tool. METHODS: . Patients with BD (n = 152), ankylosing spondylitis (n = 27), systemic vasculitides (n = 23), venous insufficiency (n = 29), antiphospholipid syndrome (APS; n = 43), deep vein thrombosis due to non-inflammatory causes (n = 25) and healthy controls (n = 51) were included in the study. Bilateral CFV thickness was measured with ultrasonography by a radiologist blinded to cases. RESULTS: Bilateral CFV thickness was significantly increased in BD compared with all control groups (P < 0.001 for all). The area under the receiver operating characteristic curve for bilateral CFV thicknesses in all comparator groups was >0.95 for the cut-off value (0.5 mm). This cut-off value also performed well against all control groups with sensitivity rates >90%. The specificity rate was also >80% in all comparator groups except APS (positive predictive value: 79.2-76.5%, negative predictive value: 92-91.8% for right and left CFV, respectively). CONCLUSION: Increased CFV thickness is a distinctive feature of BD and is rarely present in healthy and diseased controls, except APS. Our results suggest that CFV thickness measurement with ultrasonography, a non-invasive radiological modality, can be a diagnostic tool for BD with sensitivity and the specificity rates higher than 80% for the cut-off value ≥0.5 mm.
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Síndrome de Behçet/diagnóstico por imagem , Veia Femoral/diagnóstico por imagem , Adulto , Síndrome Antifosfolipídica/diagnóstico por imagem , Área Sob a Curva , Estudos de Casos e Controles , Feminino , Humanos , Modelos Logísticos , Masculino , Variações Dependentes do Observador , Valor Preditivo dos Testes , Curva ROC , Sensibilidade e Especificidade , Espondilite Anquilosante/diagnóstico por imagem , Estatísticas não Paramétricas , Vasculite Sistêmica/diagnóstico por imagem , Ultrassonografia Doppler , Insuficiência Venosa/diagnóstico por imagem , Trombose Venosa/diagnóstico por imagemRESUMO
BACKGROUNDS: Behçet's disease (BD) and Crohn's disease (CD) cannot be easily differentiated in young adults presenting with nonspecific gastrointestinal (GI) manifestations due to similar extraintestinal manifestations. We recently showed that increased common femoral vein (CFV) thickness is a distinctive feature of BD, rarely present in other inflammatory or vascular diseases with a specificity higher than 80% for the cutoff value of ≥ 0.5 mm. We suggest that CFV thickness measurement with ultrasonography (US) can be a diagnostic tool for BD. AIMS: To assess the diagnostic performance of CFV thickness measurement in the differential diagnosis of BD and CD. METHODS: Patients with BD (n = 69), CD (n = 38), and healthy controls (HC) (n = 38) were included in the study. Bilateral CFV thickness was measured with Doppler US. RESULTS: Both right and left CFV thicknesses were significantly higher in BD compared to HC and CD (for right: 0.76 mm vs 0.33 mm, for left: 0.78 mm vs 0.35 mm, p < 0.001 for both). CFV thicknesses in CD were similar to HC (p > 0.05 for both). CFV thickness was also similar between BD patients with and without GI involvement (p = 0.367). The diagnostic cutoff values of ≥ 0.5 mm for CFV thickness performed well against to both CD and HCs for discrimination of BD. The sensitivity and specificity rates were > 85% for both HC and CD. Positive and negative predictive values in our tertiary clinical setting were > 90%. CONCLUSION: We found significantly lower CFV thickness in CD compared to BD. Our results suggest that CFV wall thickness measurement is a distinctive diagnostic tool for the differentiation of BD and CD and can be helpful in daily practice for the differentiation of two diseases.
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Síndrome de Behçet/diagnóstico , Síndrome de Behçet/patologia , Doença de Crohn/diagnóstico , Doença de Crohn/patologia , Veia Femoral/diagnóstico por imagem , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Autoimmune pancreatitis has been described as a pancreatic manifestation of immunoglobulin G4-related disease, which is characterized by typical histopathologic, radiologic, and clinical features. Immunoglobulin G4-related disease is usually accompanied by elevated serum immunoglobulin G4 level, and can involve multiple organ/systems. Immunoglobulin G4-related disease has rarely been reported in pediatric population. There are few reports of inflammatory bowel disease in association with immunoglobulin G4-related disease. We describe a 7-year-old girl who presented with pancreatitis and concurrent sclerosing cholangitis, and developed bloody diarrhea during follow-up. An endoscopic examination revealed inflammatory bowel disease, and later lacrimal gland involvement was also recognized. She was diagnosed as having immunoglobulin G4-related disease, and her clinical signs and symptoms improved dramatically after steroid treatment. Hence, awareness of the clinical picture is important and early diagnosis can prevent fibrosis and organ damage.
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BACKGROUND: Anti-angiogenic tyrosine kinase inhibitors, sunitinib and pazopanib, have proven efficacy in advanced renal cell carcinoma, with specific adverse events occurring during treatment process. Comorbidities can reflect functional status and have prognostic value in oncology patients. We aimed to assess the association of the Charlson Comorbidity Index with severe toxicities and mortality in renal cell carcinoma cases treated with front-line sunitinib or pazopanib. METHODS: Files of locally advanced and metastatic renal cell carcinoma patients who received first-line sunitinib or pazopanib were retrospectively examined. Charlson Comorbidity Index of each patient was calculated. Patients were also stratified into Memorial Sloan-Kettering Cancer Center risk groups. Predictors of dose-limiting toxicity were evaluated with binomial logistic regression analysis. Univariate and multivariate Cox regression models were utilized to determine prognostic factors for survival. RESULTS: The study included 102 patients, 64 were treated with first-line sunitinib and 38 with pazopanib. In 42 patients (41.9%), Charlson Comorbidity Index was 9 or more. Dose-limiting toxicities were significantly more frequent in Charlson Comorbidity Index ≥9 group (69% vs. 40%, p = 0.004), and Charlson Comorbidity Index independently predicted dose-limiting toxicity (Hazard ratio (HR) = 4.30, p = 0.002). After adjusting for other variables, a Charlson Comorbidity Index of ≥9 is also a significant prognostic factor for progression-free (HR = 1.76, p = 0.02) and overall survival (HR = 1.75, p = 0.03). CONCLUSIONS: Charlson Comorbidity Index may be a valuable method to estimate prognosis and optimize therapy in patients with advanced renal cell carcinoma receiving first-line sunitinib or pazopanib.
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Carcinoma de Células Renais/tratamento farmacológico , Neoplasias Renais/tratamento farmacológico , Pirimidinas/administração & dosagem , Sulfonamidas/administração & dosagem , Sunitinibe/administração & dosagem , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Células Renais/patologia , Feminino , Humanos , Indazóis , Neoplasias Renais/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos RetrospectivosAssuntos
Neoplasias do Córtex Suprarrenal/complicações , Carcinoma Adrenocortical/complicações , Síndrome de Beckwith-Wiedemann/etiologia , Metilação de DNA , Impressão Genômica , Síndrome de Beckwith-Wiedemann/patologia , Feminino , Humanos , Lactente , Canais de Potássio de Abertura Dependente da Tensão da Membrana/genética , Prognóstico , Sítio de Iniciação de TranscriçãoRESUMO
INTRODUCTION: Primary choriocarcinoma of the colon is an extremely rare neoplasm which has a poor prognosis. Only 18 cases have been previously reported in English medical literature. Here we present a case of primary rectal choriocarcinoma with a good response to chemotherapy and review the literature on this uncommon tumor. CASE REPORT: A 36-year-old woman presented with abdominal pain and vaginal bleeding. Abdominal magnetic resonance imaging revealed 6.9 × 5.3 × 6.4 cm hypervascular mass posterior to uterus very close to rectum. Beta-human chorionic gonadotropin (ß-hCG) level was markedly elevated. Low anterior resection of the rectum with lymph node dissection and total abdominal hysterectomy with bilateral salpingo-oophorectomy were performed. Pathologic diagnosis was reported as colonic choriocarcinoma with a focal component of adenocarcinoma. Post-operative magnetic resonance imaging detected multiple metastatic lesions throughout the liver. The patient was treated with systemic chemotherapy using bleomycin, etoposide and cisplatin (BEP protocol). After three cycles, ß-hCG level decreased to normal and magnetic resonance imaging showed regression of liver metastasis. However, the patient died of respiratory failure due to bleomycin toxicity and pneumonia accompanied by rapid disease progression. DISCUSSION: This is an extremely rare case of primary rectal choriocarcinoma. Due to poor prognosis of the disease, it seems very important to start prompt treatment to improve patient's survival.
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Coriocarcinoma não Gestacional/diagnóstico por imagem , Coriocarcinoma não Gestacional/terapia , Neoplasias Retais/diagnóstico por imagem , Neoplasias Retais/terapia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Bleomicina/administração & dosagem , Cisplatino/administração & dosagem , Etoposídeo/administração & dosagem , Evolução Fatal , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Reto/diagnóstico por imagem , Reto/cirurgiaRESUMO
Primary retroperitoneal liposarcoma is an extremely rare malignant tumor. Herein, we report a case with an incidentally detected giant retroperitoneal liposarcoma on Ga-PSMA PET/CT during primary staging for prostate cancer.