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1.
Eur J Intern Med ; 2024 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-38880725

RESUMO

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA), is a rare ANCA-associated systemic vasculitis. Its overlapping features with other vasculitic or eosinophilic diseases, and the wide and heterogeneous range of clinical manifestations, often result in a delay to diagnosis. OBJECTIVE: To identify red flags that raise a suspicion of EGPA to prompt diagnostic testing and to present an evidence-based clinical checklist tool for use in routine clinical practice. METHODS: Systematic literature review and expert consensus to identify a list of red flags based on clinical judgement. GRADE applied to generate a strength of recommendation for each red flag and to develop a checklist tool. RESULTS: 86 studies were included. 40 red flags were identified as relevant to raise a suspicion of EGPA and assessed by the experts as being clinically significant. Experts agreed that a diagnosis of EGPA should be considered in a patient aged ≥6 years with a blood eosinophil level >1000 cells/µL if untreated and >500 cells/µL if previously treated with any medication likely to have altered the blood eosinophil count. The presence of asthma and/or nasal polyposis should reinforce a suspicion of EGPA. Red flags of asthma, lung infiltrates, pericarditis, cardiomyopathy, polyneuropathy, biopsy with inflammatory eosinophilic infiltrates, palpable purpura, digital ischaemia and ANCA positivity, usually anti-myeloperoxidase, among others, were identified. CONCLUSION: The identification of a comprehensive set of red flags could be used to raise a suspicion of EGPA in patients with eosinophilia, providing clinicians with an evidence-based checklist tool that can be integrated into their practice.

2.
Rev. esp. patol. torac ; 35(3): 217-219, oct. 2023.
Artigo em Espanhol | IBECS | ID: ibc-227392

RESUMO

Las bronquiectasias no debidas a fibrosis quística (FQ) constituyen la tercera patología inflamatoria crónica más frecuente de las vías respiratorias.La infección bronquial determina la progresión de la enfermedad, siendo la infección por Pseudomonas aeruginosa la que se asocia con peor pronóstico. Por este motivo, las guías de práctica clínica recomiendan la erradicación de P. aeruginosa en la infección primaria. Hasta el momento ningún estudio ha demostrado la utilidad real de esta pauta de tratamiento en el manejo de la infección bronquial inicial por Pseudomonas aeruginosa, por lo que el objetivo de este estudio es determinar la efectividad del tratamiento con Ciprofloxacino 750 mg cada 12 horas por vía oral durante 21 días en la erradicación de P. aeruginosa en pacientes con bronquiectasias no relacionadas con FQ. (AU)


Bronchiectasis not due to cystic fibrosis (CF) constitutes the third most frequent chronic inflammatory pathology of the airways. Bronchial infection determines the progression of the disease, being infection by Pseudomonas aeruginosa the one that is associated with the worst prognosis. For this reason, clinical practice guidelinesrecommend eradication of P. aeruginosa in primary infection. At the moment, any study has shown the real usefulness of this treatment regimen in the management of the initial bronchial infection by Pseudomonas aeruginosa, so the objective of this study is to determine the effectiveness of treatment with Ciprofloxacin 750 mg every 12 hours orally for 21 days in the eradication of P. aeruginosa in patients with non-CF bronchiectasis. (AU)


Assuntos
Humanos , Ciprofloxacina/uso terapêutico , Pseudomonas aeruginosa , Bronquiectasia/tratamento farmacológico , Resultado do Tratamento , Estudos Retrospectivos , Estudos Longitudinais , Broncopatias/tratamento farmacológico
3.
Ann Ig ; 35(5): 560-571, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37057652

RESUMO

Background: Increasing adherence to influenza vaccination among healthcare workers is a public health priority, stated that actually remains far below than international recommendations. During the 2020/2021 pandemic season, COVID-19 vaccines were not yet available until the end of December 2020, and influenza vaccines were the only one available to protect against seasonal respiratory diseases. The main objective of the present study was to assess knowledge, attitudes and adherence to influenza and other vaccinations recommended by the National Immunization Plan 2017-2021 for healthcare workers. Methods: Enrollment lasted from October and December 2020 at the vaccination unit of the University Hospital of Palermo. Data were collected through an anonymous and self-administered questionnaire, divided into 5 sections and 31 items. Results: Among 734 healthcare professionals that completed the survey, a significantly higher adherence to influenza vaccination was observed among healthcare workers that were more prone to receive COVID-19 vaccination (OR=4.02; 95% CI: 1.63-9.91). Moreover, higher influenza vaccination rates were observed among healthcare professionals that received influenza vaccination during previous 2019/2020 season (OR=15.3; 95% CI: 5.17-45.1) and that were favorable to the possible impact on increasing adherence of influenza mandatory vaccination (OR=4.88; 95% CI: 2.43-9.80). Conclusions: Propensity of healthcare workers to undergo vaccinations recommended in the National Immunization Plan increased during the first pandemic season. At the end of the vaccination season, flu vaccination coverage reached highest rates ever at the University Hospital of Palermo (around 60%), remaining anyway below the recommended minimum value of 75%. During next seasonal flu vaccination campaigns, it becomes essential to promote communication and information strategies to increase flu vaccination among healthcare workers, also focusing on co-administration with the anti-COVID-19 booster/seasonal doses.


Assuntos
COVID-19 , Vacinas contra Influenza , Influenza Humana , Humanos , Influenza Humana/epidemiologia , Influenza Humana/prevenção & controle , Estações do Ano , Vacinas contra COVID-19 , Pandemias/prevenção & controle , Conhecimentos, Atitudes e Prática em Saúde , COVID-19/prevenção & controle , Vacinação , Itália/epidemiologia , Hospitais Universitários , Atitude do Pessoal de Saúde , Pessoal de Saúde
4.
Rev. esp. patol. torac ; 35(2): 158-160, 2023. graf
Artigo em Espanhol | IBECS | ID: ibc-223080

RESUMO

In order to better characterize the profile of asthmatic patients who could benefit from monoclonal antibody treatments, the present study evaluated the effectiveness of reslizumab after one year of treatment in patients with severe uncontrolled eosinophilic asthma, distinguishing those with chronic rhinosinusitis. with associated nasal polyposis (RSCcNP). Reslizumab proved to be effective in this series of patients by reducing asthma exacerbations, improving lung function and asthma control, in addition to reducing the size and symptoms caused by nasal polyposis. (AU)


Con el fin de caracterizar mejor el perfil de pacientes asmáticos que podrían beneficiarse de tratamientos con anticuerpos monoclonales, en el presente estudio se evaluó la efectividad de reslizumab tras un año de tratamiento en pacientes con asma grave no controlada eosinofílica, distinguiendo aquellos que presentaban rinosinusitis crónica con poliposis nasal (RSCcPN) asociada. Reslizumab demostró ser efectivo en esta serie de pacientes al reducir las agudizaciones asmáticas, mejorar la función pulmonar y el control del asma, además de lograr disminuir el tamaño y sintomatología ocasionada por la poliposis nasal. (AU)


Assuntos
Humanos , Asma/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Epidemiologia Descritiva , Estudos Retrospectivos , Estudos Longitudinais , Espanha , Resultado do Tratamento , Interleucina-5
6.
Ann Cardiol Angeiol (Paris) ; 70(3): 161-167, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33958189

RESUMO

PURPOSE: Spontaneous coronary artery dissection (SCAD) may occur in middle age population without any cardiovascular risk factor. We retrospectively evaluated anatomic features of 11 patients with SCAD using a coronary arteries computed tomography (CCT), compared to age and sex balanced patients who underwent CCT. MATERIAL AND METHODS: CCT was performed in 11 patients (7 females and 4 males) as follow-up in patients with SCAD (left anterior descending - LAD or circumflex artery - Cx) and compared, using the propensity score matching analysis, with 11 healthy patients. Several anatomic features were evaluated: Left main (LM) length, angle between descending coronary artery (LAD) and its first branch, angle between LAD and LM, distance from the annulus to RCA (a-RCA distance) and LM (a-LM distance) ostia and their ratio; ratio between LM length and length a-LM and tortuosity score of the vessel with SCAD. A fluid dynamic analysis has been performed to evaluate the effects on shear stress of vessels wall. RESULTS: LM length was significantly shorter in patients with SCAD versus healthy subjects (P=0.01) as well as LM length/a-LM (P=0.03) and the angle between LAD and the first adjacent branch was sharper (P<0.01). Tortuosity score showed a statistically significant difference between groups (P<0.001). Fluid dynamic analysis demonstrates that, in SCAD group, an angle<90 degree is present at the first bifurcation and it can be a cause of increased strain on vessel wall in patients with high tortuosity of coronary artery. CONCLUSION: Tortuosity and angle between the LAD and the adjacent arterial branch combined may determine increased shear stress on the vessel wall that increases the risk of SCAD.


Assuntos
Angiografia por Tomografia Computadorizada , Angiografia Coronária/métodos , Anomalias dos Vasos Coronários/diagnóstico por imagem , Pontuação de Propensão , Doenças Vasculares/congênito , Fatores Etários , Estudos de Casos e Controles , Anomalias dos Vasos Coronários/etiologia , Anomalias dos Vasos Coronários/fisiopatologia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/patologia , Vasos Coronários/fisiopatologia , Feminino , Hemorreologia/fisiologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores Sexuais , Doenças Vasculares/diagnóstico por imagem , Doenças Vasculares/etiologia , Doenças Vasculares/fisiopatologia
7.
J Endocrinol Invest ; 44(3): 587-597, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32656666

RESUMO

PURPOSE: Reliable cut-offs for basal (bCT) and calcium stimulated calcitonin (casCT) are needed for an early and accurate diagnosis of medullary thyroid cancer (MTC). PATIENTS AND METHODS: Fifty-four new patients with nodular goiter were enrolled and analysed together with those previously published by our group for a total of 135 cases. bCT and casCT were measured by a highly sensitive method and the results compared with histological findings. In a subgroup of patients, cardiac rhythm was recorded before and during the calcium test. RESULTS: In both females (F) and males (M), there was a significant correlation between tumor size and bCT levels (P < 0.001). The receiver operating characteristic plot analyses showed that, for bCT, the new cut-off points able to separate non-MTC from MTC patients were > 30 (F) and > 34 pg/mL (M), whereas the best casCT thresholds were > 79 (F) and > 466 pg/mL (M). bCT was shown to harbour a high accuracy, though some cases were diagnosed only upon stimulation test. Importantly, combining bCT, below or above the cut-offs, with casCT above the cut-offs, all the MTC cases were correctly identified. A reversible sinus bradycardia was observed in 9% of cases during the test. CONCLUSIONS: Refined cut-offs for bCT and casCT in patients with nodular goiter are reported. Sensitive bCT was shown to have a high accuracy, but the combination with casCT data was needed to identify all MTC cases. The reliability and safety of calcium test strongly favour the routine use of CT determination in nodular thyroid disease.


Assuntos
Biomarcadores Tumorais/sangue , Calcitonina/sangue , Cálcio/farmacologia , Carcinoma Neuroendócrino/diagnóstico , Bócio Nodular/fisiopatologia , Neoplasias da Glândula Tireoide/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Neuroendócrino/sangue , Carcinoma Neuroendócrino/epidemiologia , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Neoplasias da Glândula Tireoide/sangue , Neoplasias da Glândula Tireoide/epidemiologia
8.
Nat Prod Res ; 35(1): 116-123, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31140311

RESUMO

In this paper we present a possible application of cinnamon essential oil to be encapsulated into gel drops of psyllium and of psyllium-alginate mixtures and to be released by the beads. It could act as green biocide for the protection of antique books, old documents and, generally, of any cellulosic material (paper, wood, textiles) object of cultural interest from biological attack. The components of the cinnamon essential oil, released by alginate, psyllium-alginate and purified psyllium-alginate beads, were determined by GC-MS analysis. Moreover, an evaluation of the cinnamon essential oil release during the time was carried out by in time HS-SPME-GS-MS so to obtain in time semi-quantitative information about the emitted gaseous species. Last by, in order to confirm the ability of the beads to perform an antimicrobial action, respirometric tests were carried out on Saccharomyces cerevisiae yeast cells looking at the reduction of their breathing activity, when in presence of the above beads.


Assuntos
Cápsulas/química , Óleos Voláteis/química , Alginatos/química , Cinnamomum zeylanicum/química , Cromatografia Gasosa-Espectrometria de Massas , Testes de Sensibilidade Microbiana , Óleos Voláteis/farmacocinética , Óleos Voláteis/farmacologia , Psyllium/química , Saccharomyces cerevisiae/efeitos dos fármacos
9.
Nat Prod Res ; 34(19): 2862-2866, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28000502

RESUMO

Plastic materials are being used in art: in Italy, for instance, two Art Museums are already presenting only plastic artefacts. As organic matter, plastic suffers degradation and chemical and biological attacks beyond obvious ageing process. Here, we propose an electrochemical approach aiming at the evaluation of the conditions of a plastic object, so giving the opportunity of protection and conservation strategies. The method is very cheap, easy to be handled and absolutely non-invasive, and is based on the monitoring of acidity emitted by a degrading plastic basing on corrosion potential of target metals acting as sensors.


Assuntos
Artefatos , Técnicas Eletroquímicas , Plásticos/química , Ácidos/metabolismo , Arte , Corrosão , Itália , Museus
11.
Endocrine ; 55(3): 959-968, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27094308

RESUMO

ARMC5 mutations have recently been identified as a common genetic cause of primary bilateral macronodular adrenal hyperplasia (PBMAH). We aimed to assess the prevalence of ARMC5 germline mutations and correlate genotype with phenotype in a large cohort of PBMAH patients. A multicenter study was performed, collecting patients from different endocrinology units in Italy. Seventy-one PBMAH patients were screened for small mutations and large rearrangements in the ARMC5 gene: 53 were cortisol-secreting (two with a family history of adrenal hyperplasia) and 18 were non-secreting cases of PBMAH. Non-mutated and mutated patients' clinical phenotypes were compared and related to the type of mutation. A likely causative germline ARMC5 mutation was only identified in cortisol-secreting PBMAH patients (one with a family history of adrenal hyperplasia and ten apparently sporadic cases). Screening in eight first-degree relatives of three index cases revealed four carriers of an ARMC5 mutation. Evidence of a second hit at somatic level was identified in five nodules. Mutated patients had higher cortisol levels (p = 0.062), and more severe hypertension and diabetes (p < 0.05). Adrenal glands were significantly larger, with a multinodular phenotype, in the mutant group (p < 0.01). No correlation emerged between type of mutation and clinical parameters. ARMC5 mutations are frequent in cortisol-secreting PBMAH and seem to be associated with a particular pattern of the adrenal masses. Their identification may have implications for the clinical care of PBMAH cases and their relatives.


Assuntos
Glândulas Suprarrenais/patologia , Hiperplasia Suprarrenal Congênita/genética , Mutação em Linhagem Germinativa , Proteínas Supressoras de Tumor/genética , Hiperplasia Suprarrenal Congênita/patologia , Adulto , Idoso , Proteínas do Domínio Armadillo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Linhagem , Fenótipo
12.
J Endocrinol Invest ; 39(7): 807-34, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26969462

RESUMO

Treatment of osteoporosis is aimed to prevent fragility fractures and to stabilize or increase bone mineral density. Several drugs with different efficacy and safety profiles are available. The long-term therapeutic strategy should be planned, and the initial treatment should be selected according to the individual site-specific fracture risk and the need to give the maximal protection when the fracture risk is highest (i.e. in the late life). The present consensus focused on the strategies for the treatment of postmenopausal osteoporosis taking into consideration all the drugs available for this purpose. A short revision of the literature about treatment of secondary osteoporosis due both to androgen deprivation therapy for prostate cancer and to aromatase inhibitors for breast cancer was also performed. Also premenopausal females and males with osteoporosis are frequently seen in endocrine settings. Finally particular attention was paid to the tailoring of treatment as well as to its duration.


Assuntos
Densidade Óssea/efeitos dos fármacos , Osteoporose/tratamento farmacológico , Fraturas por Osteoporose/prevenção & controle , Conservadores da Densidade Óssea/uso terapêutico , Consenso , Endocrinologistas , Feminino , Humanos , Itália , Masculino
13.
Osteoporos Int ; 26(4): 1295-302, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25524023

RESUMO

UNLABELLED: No data on the pharmacological treatment of normocalcemic hyperparathyroidism (NPHPT) are available. We treated 30 NPHPT postmenopausal women with alendronate/cholecalciferol (treated group) or vitamin D alone (control group). Over 1 year, bone mineral density (BMD) increased significantly in treated group, but not in control group. Both treatments did not affect serum or urinary calcium. INTRODUCTION: Normocalcemic primary hyperparathyroidism (NPHPT) is defined by normal serum calcium and consistently elevated PTH levels after ruling out the causes of secondary hyperparathyroidism. It is likely that subjects with NPHPT may develop kidney and bone disease. As no data on the pharmacological treatment of NPHPT are available, we aimed to investigate the effects of alendronate and cholecalciferol on both BMD and bone biochemical markers in postmenopausal women with NPHPT. Safety of vitamin D was evaluated as secondary endpoint. METHODS: The study was a prospective open label randomized trial comparing 15 postmenopausal women with NPHPT (PMW-NPHPT), treated with oral alendronate plus cholecalciferol (treated group) and 15 PMW-NPHPT treated only with cholecalciferol (control group). Blood samples were obtained at baseline and after 3, 6, and 12 months. Bone turnover markers (BTM) were measured at baseline, 3, and 6 months, respectively. BMD was assessed at baseline and after 12 months. RESULTS: After 1 year of treatment, BMD increased significantly at the lumbar, femoral neck, and hip level in the treated group, but not in the control group (p = 0.001). No differences were found between or within groups in serum calcium, PTH, and urinary calcium levels. BTM significantly decreased in the treated group but not in the control group, at 3 and 6 months (p < 0.001), respectively. No cases of hypercalcemia or hypercalciuria were detected during the study. CONCLUSION: The results of this study indicate that alendronate/cholecalciferol increases BMD in postmenopausal women with NPHPT. Alendronate/cholecalciferol or vitamin D alone does not affect serum or urinary calcium.


Assuntos
Alendronato/uso terapêutico , Conservadores da Densidade Óssea/uso terapêutico , Densidade Óssea/efeitos dos fármacos , Colecalciferol/uso terapêutico , Hiperparatireoidismo Primário/tratamento farmacológico , Administração Oral , Cálcio/sangue , Combinação de Medicamentos , Feminino , Fêmur/fisiopatologia , Humanos , Hiperparatireoidismo Primário/sangue , Hiperparatireoidismo Primário/complicações , Hiperparatireoidismo Primário/fisiopatologia , Vértebras Lombares/fisiopatologia , Pessoa de Meia-Idade , Osteoporose Pós-Menopausa/tratamento farmacológico , Osteoporose Pós-Menopausa/etiologia , Osteoporose Pós-Menopausa/fisiopatologia , Estudos Prospectivos
14.
J Endocrinol Invest ; 35(9): 817-23, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22080849

RESUMO

BACKGROUND: The few epidemiological data available in literature on neuroendocrine tumors (NET) are mainly based on Registry databases, missing therefore details on their clinical and natural history. AIM: To investigate epidemiology, clinical presentation, and natural history of NET. DESIGN AND SETTING: A large national retrospective survey was conducted in 13 Italian referral centers. Among 1203 NET, 820 originating in the thorax (T-NET), in the gastro-enteropancreatic tract (GEP-NET) or metastatic NET of unknown primary origin (U-NET) were enrolled in the study. RESULTS: 93% had a sporadic and 7% a multiple endocrine neoplasia type 1 (MEN1)-associated tumor; 63% were GEP-NET, 33% T-NET, 4% U-NET. Pancreas and lung were the commonest primary sites. Poorly differentiated carcinomas were <10%, all sporadic. The incidence of NET had a linear increase from 1990 to 2007 in all the centers. The mean age at diagnosis was 60.0 ± 16.4 yr, significantly anticipated in MEN1 patients (47.7 ± 16.5 yr). Association with cigarette smoking and other non-NET cancer were more prevalent than in the general Italian population. The first symptoms of the disease were related to tumor burden in 46%, endocrine syndrome in 23%, while the diagnosis was fortuity in 29%. Insulin (37%) and serotonin (35%) were the most common hormonal hypersecretions. An advanced tumor stage was found in 42%, more frequently in the gut and thymus. No differences in the overall survival was observed between T-NET and GEP-NET and between sporadic and MEN1-associated tumors at 10 yr from diagnosis, while survival probability was dramatically reduced in U-NET. CONCLUSIONS: The data obtained from this study furnish relevant information on epidemiology, natural history, and clinico-pathological features of NET, not available from the few published Register studies.


Assuntos
Neoplasias Intestinais/epidemiologia , Neoplasia Endócrina Múltipla Tipo 1/epidemiologia , Tumores Neuroendócrinos/epidemiologia , Neoplasias Pancreáticas/epidemiologia , Neoplasias Gástricas/epidemiologia , Neoplasias Torácicas/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Gerenciamento Clínico , Feminino , Humanos , Lactente , Neoplasias Intestinais/mortalidade , Neoplasias Intestinais/terapia , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/mortalidade , Neoplasia Endócrina Múltipla Tipo 1/terapia , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/terapia , Neoplasias Pancreáticas/mortalidade , Neoplasias Pancreáticas/terapia , Prevalência , Prognóstico , Estudos Prospectivos , Estudos Retrospectivos , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/terapia , Taxa de Sobrevida , Neoplasias Torácicas/mortalidade , Neoplasias Torácicas/terapia , Adulto Jovem
16.
J Endocrinol Invest ; 35(3): 326-31, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21757992

RESUMO

BACKGROUND: In patients with well-differentiated (WD) neuroendocrine tumors (NET), long-acting octreotide (LAR), conventionally administered at a dose of 30 mg every 28 days, has well-documented anti-secretive but limited antiproliferative effects. AIM: The objective of this study was to evaluate a different schedule of LAR treatment consistent with a shorter interval between administrations (21 days) in WDNET patients with progressive disease at standard-dose interval. SUBJECTS AND METHODS: Twenty-eight patients followed for diagnosis and therapy of WDNET who had tumor progression during therapy with LAR 30 mg every 28 days were enrolled. Clinical, biological, and objective tumor response was evaluated after LAR 30 mg every 21 days. Time to progression was also evaluated after LAR 30 mg every 21 days and compared to LAR 30 mg every 28 days. RESULTS: The treatment with LAR 30 mg every 21 days resulted in complete and partial control of clinical symptoms in 40% and 60% of cases, respectively. Circulating neuroendocrine markers were significantly decreased in 30% of cases. A stabilization of disease was obtained in 93% and objective response in 7%. The median time to progression was significantly longer by using the shortened interval of LAR administration as compared to the standard one (30 vs 9 months, p<0.0001). The treatment was safe and well tolerated. CONCLUSIONS: The shortened schedule of LAR administration was able to re-institute control of clinical symptoms, to decrease level of circulating neuroendocrine markers and to increase time to progression in patients previously escaping from a standard schedule treatment.


Assuntos
Antineoplásicos Hormonais/administração & dosagem , Carcinoma Neuroendócrino/tratamento farmacológico , Neoplasias Gastrointestinais/tratamento farmacológico , Neoplasia Endócrina Múltipla Tipo 1/tratamento farmacológico , Octreotida/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos Hormonais/efeitos adversos , Carcinoma Neuroendócrino/patologia , Diferenciação Celular , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Neoplasias Gastrointestinais/patologia , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/patologia , Octreotida/efeitos adversos , Neoplasias do Timo/tratamento farmacológico , Neoplasias do Timo/patologia , Resultado do Tratamento
17.
Ann Oncol ; 22(3): 671-680, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-20693297

RESUMO

BACKGROUND: Spleen and liver assessment for occult involvement of Hodgkin's lymphoma (HL) challenges current staging procedures. PATIENTS AND METHODS: We prospectively evaluated event-free survival (EFS) in 103 HL patients staged with fused 2-[fluorine-18]fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET)/contrast-enhanced computed tomography (CT) to identify those at greatest risk for abdominal relapse. The EFS of this series was compared with that of a historical cohort of 100 HL patients staged with separate FDG-PET and diagnostic CT acquisitions. RESULTS: Thirty-one of the 103 patients staged with FDG-PET/contrast-enhanced CT were found to have spleen involvement and 10 patients liver involvement, whereas 14 of the 100 patients staged with separate procedures were found to have spleen involvement and 3 patients liver involvement. There were significantly more intensive treatments (six courses of anthracycline-containing chemotherapy and spleen radiation) in the fused PET/CT group than in the historical cohort (P ≤ 0.04). At a median follow-up of 27 months, five events occurred in the fused PET/CT group (HL relapse, 4 patients; carcinoma, 1 patient) and 19 events in the historical cohort (HL relapse, 18 patients; acute promyelocytic leukemia, 1 patient). Ten of the 18 relapses in the historical cohort were localized in the spleen and/or liver area. None of the four relapses in the fused PET/CT group was localized below the diaphragm. Thus, FDG-PET/contrast-enhanced CT-guided treatment resulted in a 95% EFS, whereas separate FDG-PET and diagnostic CT-guided treatment resulted in an 81% EFS (P = 0.002). CONCLUSION: FDG-PET/contrast-enhanced CT is an accurate frontline single imaging diagnostic tool enabling effective tailored treatment in HL patients.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Fluordesoxiglucose F18 , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/terapia , Compostos Radiofarmacêuticos , Baço/patologia , Adolescente , Adulto , Idoso , Bleomicina/administração & dosagem , Terapia Combinada , Meios de Contraste , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Epirubicina/administração & dosagem , Etoposídeo/administração & dosagem , Feminino , Doença de Hodgkin/patologia , Humanos , Estimativa de Kaplan-Meier , Fígado/diagnóstico por imagem , Fígado/efeitos dos fármacos , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Tomografia por Emissão de Pósitrons , Prednisona/administração & dosagem , Estudos Prospectivos , Recidiva , Baço/diagnóstico por imagem , Baço/efeitos dos fármacos , Baço/efeitos da radiação , Tomografia Computadorizada por Raios X , Adulto Jovem
18.
Eur J Endocrinol ; 163(3): 369-76, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20530095

RESUMO

BACKGROUND: Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene and the p27(KIP1) encoding gene CDKN1B have been associated with two well-defined hereditary conditions, familial isolated pituitary adenoma (FIPA) and multiple endocrine neoplasia type 4 (MEN4). Somatotropinomas are present in most AIP mutated FIPA kindreds, as well as in two-thirds of MEN4 patients who carry pituitary tumors. METHODS: Germline DNA samples of 131 Italian sporadic acromegalic patients including 38 individuals with multiple tumors, and of six FIPA families (four homogeneous for prolactinomas and two heterogeneous with prolactin/nonfunctioning pituitary adenomas) were collected in a multicentric collaborative study. The prevalence of AIP and CDKN1B gene point mutations and copy number variations were evaluated. RESULTS: Two novel (IVS3+1G>A and c.871G>A) and one previously described (c.911G>A) AIP mutations were detected in four apparently sporadic cases (3.1%) with relatively high age at diagnosis (49+/-18, range 30-67). No mutations/rearrangements were detected in FIPA families. The highly conserved c.871G>A substitution was detected in a patient who also carried a MEN1 mutation suggesting that she is a double heterozygote. The possible pathogenic effect on AIP splicing of the silent substitution c.144G>A found in another patient was ruled out using a minigene-based approach. CDKN1B mutations/rearrangements were neither identified in patients with multiple neoplasia nor in FIPA families. CONCLUSION: AIP is mutated in about 3% of apparently sporadic acromegalic patients. The relatively high age at diagnosis, as well as its sporadic presentation, suggests that these patients are carriers of mutations with reduced pathogenicity. p27(KIP1) is unlikely to represent the common unifying nonendocrine etiology for acromegaly and cancer.


Assuntos
Acromegalia/epidemiologia , Acromegalia/genética , Peptídeos e Proteínas de Sinalização Intracelular/genética , Neoplasia Endócrina Múltipla/epidemiologia , Neoplasia Endócrina Múltipla/genética , Acromegalia/complicações , Adolescente , Adulto , Idoso , Sequência de Aminoácidos , Inibidor de Quinase Dependente de Ciclina p27 , Feminino , Mutação em Linhagem Germinativa/genética , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Dados de Sequência Molecular , Neoplasia Endócrina Múltipla/complicações , Linhagem , Prevalência , Adulto Jovem
19.
Cytopathology ; 21(5): 300-10, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20132132

RESUMO

OBJECTIVE: To evaluate the diagnostic efficiency of fine needle aspiration cytology/flow cytometry (FNAC/FC) in the diagnosis and classification of non-Hodgkin lymphoma (NHL) in a series of 446 cases and to compare the results with those of previous experiences to evaluate whether there had been an improvement in FNAC/FC diagnostic accuracy. METHODS: FNAC/FC was used to analyse 446 cases of benign reactive hyperplasia (BRH), NHL and NHL relapse (rNHL) in 362 lymph nodes and 84 extranodal lesions. When a diagnosis of NHL was reached, a classification was attempted combining FC data and cytological features. Sensitivity, specificity and positive and negative predictive values (PPV and NPV) of FNAC/FC in the diagnosis and classification of NHL were calculated and compared with those available in the literature. RESULTS: FNAC/FC provided a diagnosis of NHL and rNHL in 245 cases and of BRH in 188 cases. In nine cases, the diagnosis was 'suggestive of NHL' (sNHL) and in four cases was inadequate. Histology and clinical follow-up confirmed 102 cases of NHL and detected one false positive. In 18 cases of BRH diagnosed by FNAC/FC, histological examination revealed 14 BRH and four NHL (false negatives). All nine cases diagnosed as sNHL were confirmed by histology. Including sNHL cases as false negatives, statistical analysis showed 94.9% sensitivity, 99.4% specificity, 99.6% PPV and 93.4% NPV in the diagnosis of NHL. A specific subtype was diagnosed in 125 cases and confirmed in 67 of 70 cases that had histological biopsies. Statistical analysis did not demonstrate significant improvements between the present series and previous studies either in diagnosis or in classification of NHL. CONCLUSIONS: FNAC/FC is a fundamental tool in the diagnosis and classification of NHL but the exiguity of diagnostic material and other technical and clinical limitations will probably continue to limit further improvement of the technique.


Assuntos
Citometria de Fluxo/métodos , Imunofenotipagem/métodos , Linfoma não Hodgkin/patologia , Biópsia por Agulha Fina/métodos , Citodiagnóstico , Erros de Diagnóstico/prevenção & controle , Feminino , Humanos , Linfonodos/patologia , Linfoma não Hodgkin/classificação , Linfoma não Hodgkin/imunologia , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes
20.
Autoimmun Rev ; 9(3): 165-9, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19647102

RESUMO

Elevated B-Lymphocyte Stimulator (BLyS) and April (a proliferation-inducing ligand) expressions characterize several autoimmune diseases. We here analysed the possible role of BLyS and April in autoimmune thyroid diseases (AITD), comprising Hashimoto's thyroiditis (HT) and Graves' disease (GD). Seventy-seven patients with AITD and 77 blood donors (HBD) were enrolled in the study. Serum BLyS and April levels were assessed by ELISA. Results indicated a significant upregulation of BLyS in AITD patients (1.12+/-0.39 ng/ml versus 0.666+/-0.240 ng/ml in HBD; p<0.0001), with GD patients presenting higher BLyS levels than HT patients (1.22+/-0.42 ng/ml versus 1.07+/-0.38 ng/ml; p=0.0393). In contrast, April levels were downregulated, but only in HT patients [9.9+/-36.6 (median 0) ng/ml versus 7.4+/-22.1 (median 1.16) ng/ml in HBD; p=0.003; and versus 4.2+/-5.9 ng/ml (median 0.9) ng/ml in GD; p=0.0353]. In HT patients, Levo-thyroxine supplementation further increased BLyS and tended to normalize April levels. Neither BLyS nor April did correlate with the levels of the pathognomonic autoantibodies (TPOAb, TgAb, TRAb). Data are preliminary, but, for the first time, we provide the analyses of BLyS and April levels in AITD patients, suggesting new tools for the diagnosis, prognosis and possible therapeutic management of AITD.


Assuntos
Fator Ativador de Células B/sangue , Tireoidite Autoimune/sangue , Membro 13 da Superfamília de Ligantes de Fatores de Necrose Tumoral/sangue , Fator Ativador de Células B/imunologia , Regulação para Baixo , Feminino , Doença de Graves/sangue , Doença de Graves/imunologia , Doença de Hashimoto/sangue , Doença de Hashimoto/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Tireoidite Autoimune/imunologia , Membro 13 da Superfamília de Ligantes de Fatores de Necrose Tumoral/imunologia , Regulação para Cima
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