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1.
ESMO Open ; 6(1): 100010, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33399076

RESUMO

BACKGROUND: The implementation of multidisciplinary tumor board (MDTB) meetings significantly ameliorated the management of oncological diseases. However, few evidences are currently present on their impact on pancreatic cancer (PC) management. The aim of this study was to evaluate the impact of the MDTB on PC diagnosis, resectability and tumor response to oncological treatment compared with indications before discussion. PATIENTS AND METHODS: All patients with a suspected or proven diagnosis of PC presented at the MDTB from 2017 to 2019 were included in the study. Changes of diagnosis, resectability and tumor response to oncological/radiation treatment between pre- and post-MDTB discussion were analyzed. RESULTS: A total of 438 cases were included in the study: 249 (56.8%) were presented as new diagnoses, 148 (33.8%) for resectability assessment and 41 (9.4%) for tumor response evaluation to oncological treatment. MDTB discussion led to a change in diagnosis in 54/249 cases (21.7%), with a consequent treatment strategy variation in 36 cases (14.5%). Change in resectability was documented in 44/148 cases (29.7%), with the highest discrepancy for borderline lesions. The treatment strategy was thus modified in 27 patients (18.2%). The MDTB brought a modification in the tumor response assessment in 6/41 cases (14.6%), with a consequent protocol modification in four (9.8%) cases. CONCLUSIONS: MDTB discussion significantly impacts on PC management, especially in high-volume centers, with consistent variations in terms of diagnosis, resectability and tumor response assessment compared with indications before discussion.


Assuntos
Pancreatopatias , Neoplasias Pancreáticas , Humanos , Estudos Interdisciplinares , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Centros de Atenção Terciária
2.
Eur Rev Med Pharmacol Sci ; 23(19): 8625-8629, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31646596

RESUMO

OBJECTIVE: Cannabis is an illegal drug that has been under the spotlight in recent years, due to its vast array of effects on different biological systems. The role of cannabis has been investigated in the management of pain in acute pancreatitis (AP), even though some studies suggest that it may have a causative effect in this pathology and could be considered the underlying etiology in some cases of idiopathic AP. In this case report, we discuss the case of a young man who presented with three different episodes of AP, with apparently no significant history of alcohol and drug consumption, and with no evidence of a biliary, genetic or, autoimmune etiology. During the third episode, in which he had developed a voluminous pseudocyst, treated trough ultrasound (EUS)-guided drainage, he admitted consumption of cannabis daily. The Naranjo score resulted to be 6 (confirming the possible causality), and it was suggested to the patient to avoid cannabis consumption. Since then, he did not develop any other AP episodes. In summary, cannabis should be considered among the possible AP etiologies, as its causative identification and interruption may significantly improve the course of several idiopathic APs.


Assuntos
Canabinoides/efeitos adversos , Cannabis/efeitos adversos , Pancreatite/induzido quimicamente , Adulto , Humanos , Masculino , Pancreatite/diagnóstico por imagem
3.
Eur Rev Med Pharmacol Sci ; 23(2): 771-787, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30720186

RESUMO

Acute pancreatitis (AP) is the most common gastrointestinal disorder requiring hospitalization, with a high rate of morbidity and mortality. Severe AP is characterized by the presence of persistent organ failure involving single or multiple organs. Clinical evolution, laboratory and radiological assessment are necessary to evaluate the prognosis and inform the management of AP. The onset of severe AP may be classified in two principal phases. The early phase, during the first week, is characterized by the activation of the auto-inflammatory cascade, gut dysbiosis, bacterial translocation, and the down-regulation of immune responses. The late phase is characterized by the development of local and systemic complications. Several old paradigms have been amended in the management of AP patients, such as the indication of nutrition, the use of antibiotic therapy, pain control strategies, and even the use of surgery. Real world evidence has shown that in the majority of cases a step-up approach is most effective. In this review, we discuss the clinical assessment and improvements to the management of patients with severe AP in a high volume center where a multi-disciplinary approach is performed.


Assuntos
Insuficiência de Múltiplos Órgãos/terapia , Dor/tratamento farmacológico , Pancreatite/terapia , Equipe de Assistência ao Paciente , Analgésicos/uso terapêutico , Antibacterianos/uso terapêutico , Translocação Bacteriana/imunologia , Colangiopancreatografia Retrógrada Endoscópica , Drenagem/métodos , Gastroenterostomia , Microbioma Gastrointestinal/imunologia , Humanos , Insuficiência de Múltiplos Órgãos/imunologia , Terapia Nutricional/métodos , Dor/imunologia , Manejo da Dor/métodos , Pâncreas/diagnóstico por imagem , Pâncreas/imunologia , Pâncreas/patologia , Pâncreas/cirurgia , Pancreatite/complicações , Pancreatite/diagnóstico , Pancreatite/imunologia , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X
4.
Endocrine ; 60(2): 362-367, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-28567607

RESUMO

PURPOSE: Multiple endocrine neoplasia type 1 (MEN1) is an inherited endocrine neoplastic syndrome associated with a greater risk of endocrine tumor development like pancreatic neuroendocrine tumors (p-NET), with different clinical characteristics from sporadic ones. This paper aims to compare clinical, hystological and morphological aspects of p-NET in patients affected from MEN1 (MEN1+) and not-affected ones (MEN1-). METHODS: We performed a retrospective observational study. Data was collected between December 2010 and December 2015, including patients with a histological diagnosis of p-NET and radiological imaging. We compared clinical, histological, radiological, and prognostic aspects of MEN+ p-NET with MEN-1 p-NET. RESULTS: Of the 45 patients enrolled, 13 MEN1+ and 21 MEN1- cases were analyzed. Frequency of not secreting p-NETs and insulin secreting p-NETs, histopathological grades and Ki67 expression were superimposable between MEN1+ and MEN1- patients. MEN1+ pNETs are more often multicentric compared to MEN1- pNETs. Frequency of liver and nodes metastatic spread was higher in MEN1- p-NET compared to MEN1+ p-NET. Analyzing p-NET according to the disease outcome, we found that recovered and stable p-NETs in MEN1+ patients, compared to MEN1- cases, are diagnosed at lower age (p = 0.04/p = 0.002) and that are more frequently multifocal lesions (p = 0.009/p = 0.002). CONCLUSIONS: In our study pNETs in MEN1+ and pNETs in MEN1- don't significantly differ for prognosis but only for clinical features. p-NET stage disease and prognosis can be positively influenced by early diagnosis and screening in index patients' first-degree relatives.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumores Neuroendócrinos/patologia , Pâncreas/patologia , Neoplasias Pancreáticas/patologia , Adulto , Idoso , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/etiologia , Neoplasias Pancreáticas/etiologia , Estudos Retrospectivos
5.
Eur Rev Med Pharmacol Sci ; 21(22): 5179-5184, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29228431

RESUMO

Pancreatic cystosis is a rare presentation of cystic fibrosis involving pancreatic gland. To date, only very few cases of pancreatic cystosis have been described in literature. Pancreatic cystosis may begin during the second decade of life and is the rarest presentation of cystic fibrosis. This disease is characterized by the presence of multiloculated cysts without ductal system communication of different sizes in all the pancreatic tissue. Herein, we report a case of a young woman affected by cystic fibrosis that was admitted to our Pancreatic Centre to evaluate a picture of diffuse multiloculated pancreatic cysts. After magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS) assessment, we perform the diagnosis of the concomitant presence of the rare condition of pancreatic cystosis with Branch Duct-Intraductal Papillary Mucinous Neoplasm (BD-IPMN). To our knowledge, this is the first reported case of a cystic fibrosis patient with the combination of pancreatic cystosis and IPMN.


Assuntos
Adenocarcinoma Mucinoso/complicações , Fibrose Cística/complicações , Cisto Pancreático/complicações , Papiloma Intraductal/complicações , Adenocarcinoma Mucinoso/diagnóstico por imagem , Fibrose Cística/diagnóstico por imagem , Endossonografia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Pâncreas/diagnóstico por imagem , Cisto Pancreático/diagnóstico por imagem , Papiloma Intraductal/diagnóstico por imagem
6.
Eur Rev Med Pharmacol Sci ; 21(12): 2858-2874, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28682431

RESUMO

Intraductal Papillary Mucinous Neoplasms (IPMNs) are the most common cystic tumors of the pancreas and are considered premalignant lesions. IPMNs are characterized by the papillary growth of the ductal epithelium with rich mucin production, which is responsible for cystic segmental or diffuse dilatation of the main pancreatic duct (MPD) and/or its branches. According to the different involvement of pancreatic duct system, IPMNs are divided into main duct type (MD-IPMN), branch duct type (BD-IPMN), and mixed type (MT-IPMN). IPMNs may be incidentally discovered in asymptomatic patients, particularly in those with BD-IPMNs, when imaging studies are performed for unrelated indications. The increase in their frequency may reflect the combined effects of new diagnostic techniques, the improvement of radiologic exams and progress in the recognition of the pathology. MD-IPMNs present a higher risk of malignant progression than BD-IPMNs; as a consequence, all the guidelines strictly suggest the need of surgery for MD- and MT- IPMNs with MPD > 10 mm, while the management of BD-IPMNs is still controversial and depends on several cysts and patients features. The choice between non-operative and surgical management depends on the distinction between benign and invasive IPMN forms, assessment of malignancy risk, patient's wellness and its preferences. This manuscript revises the different guidelines for the management of IPMNs that have been published in different world countries: the international (Sendai 2006 and Fukuoka 2012), the 2013 European, the 2014 Italian, and finally the 2015 American guidelines. In summary, this review will integrate the recent insights in the combination of diagnostic techniques, such as Magnetic Resonance Imaging (MRI) and endoscopic ultrasound (EUS), pathology classification, and management of IPMNs.


Assuntos
Adenocarcinoma Mucinoso/terapia , Adenocarcinoma Papilar/terapia , Ductos Pancreáticos/diagnóstico por imagem , Ductos Pancreáticos/patologia , Neoplasias Pancreáticas/terapia , Guias de Prática Clínica como Assunto , Adenocarcinoma Mucinoso/diagnóstico por imagem , Adenocarcinoma Mucinoso/patologia , Adenocarcinoma Papilar/diagnóstico por imagem , Adenocarcinoma Papilar/patologia , Endossonografia , Humanos , Imageamento por Ressonância Magnética , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/patologia
7.
Intern Med J ; 43(6): 725-7, 2013 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23745996

RESUMO

Although idiopathic recurrent acute pericarditis (IRAP) is generally presumed to derive from an autoimmune process, increasing interest is currently being devoted to autoinflammatory diseases, a group of disorders of the innate immune system caused by mutations of genes involved in the regulation or activation of the inflammatory response, without any apparent involvement of autoimmunity. The tumour necrosis factor receptor-1-associated periodic syndrome is the most common autosomal dominant autoinflammatory disorder and is caused by mutations in the TNFRSF1A gene encoding the 55-kD receptor for tumour necrosis factor-α. IRAP patients carrying TNFRSF1A gene mutations have been recently described. We report herein the first IRAP patients carrying the rare R104Q and D12E TNFRSF1A gene mutations, thus expanding the spectrum of tumour necrosis factor receptor-1-associated periodic syndrome mutations in IRAP patients.


Assuntos
Mutação/genética , Pericardite/diagnóstico , Pericardite/genética , Receptores Tipo I de Fatores de Necrose Tumoral/genética , Doença Aguda , Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva
8.
Clin Exp Rheumatol ; 30(3 Suppl 72): S108-14, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22935190

RESUMO

OBJECTIVES: The aims of our study were to evaluate serum leptin, resistin, visfatin and adiponectin levels in patients with tumour necrosis factor receptor-associated periodic syndrome (TRAPS), in comparison to healthy controls, and to correlate their levels to parameters of disease activity and/or severity. METHODS: Serum leptin, resistin, visfatin and adiponectin levels were obtained from 14 TRAPS patients carrying mutations involving cysteine residues, from 16 TRAPS patients carrying other mutations, and from 16 healthy controls. Demographic, clinical and laboratory parameters, including amyloidosis were entered for each patient. Comparisons between groups as well as reciprocal comparisons have been evaluated. RESULTS: Serum leptin, resistin, visfatin and adiponectin did not significantly differ among the 3 groups. Patients carrying cysteine residues mutations showed lower visfatin serum levels than patients carrying other mutations (p<0.02). Serum leptin significantly correlated with the number of attacks/year (multiple R=0.32, multiple adjusted R2= 0.19, p <0.03). Serum adiponectin levels significantly correlated with the presence of amyloidosis (multiple R=0.79, multiple adjusted R2=0.57, p<0.03). Adiponectin values were a significant predictor for amyloidosis (AUC 0.75, 95 CI: 0.56-0.94, p<0.03), with a predicting cut-off value set at 23.16 pg/ml, the predictive positive value was 53.8%. Visfatin serum levels resulted respectively related to leptin (rs=0.42, r2=0.18, p<0.02) and to resistin (rs=0.57, r2=0.32, p<0.01) serum levels; whilst leptin and resistin serum levels did not reciprocally correlate. CONCLUSIONS: Although a prospective design study and larger cohort are mandatory, adipokines serum levels and their correlations with parameters of disease activity and/or severity seem to show a baseline pattern in TRAPS patients.


Assuntos
Adiponectina/sangue , Citocinas/sangue , Doenças Hereditárias Autoinflamatórias/sangue , Leptina/sangue , Mutação , Nicotinamida Fosforribosiltransferase/sangue , Receptores Tipo I de Fatores de Necrose Tumoral/genética , Resistina/sangue , Adulto , Idoso , Amiloidose/sangue , Amiloidose/genética , Análise de Variância , Estudos de Casos e Controles , Estudos Transversais , Progressão da Doença , Feminino , Febre , Predisposição Genética para Doença , Doenças Hereditárias Autoinflamatórias/complicações , Doenças Hereditárias Autoinflamatórias/diagnóstico , Doenças Hereditárias Autoinflamatórias/genética , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Adulto Jovem
9.
J Endocrinol Invest ; 34(9): 692-7, 2011 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22067307

RESUMO

The aim of this retrospective study was to evaluate the efficacy, safety, and tolerability of lanreotide autogel given to metastatic well-differentiated (WD) neuroendocrine tumors (NET) patients observed in our Institute between 2005 and 2008. Patients with metastatic NET referred to our tertiary referral center were given lanreotide autogel 120 mg/month by deep sc injection for a period of at least 24 months. The efficacy was evaluated by the relief of disease symptoms, behavior of tumor markers and response rate in terms of time to tumor progression. Safety and tolerability were evaluated by assessing the onset of adverse events and treatment feasibility. Twenty-three patients (13 males), median age 62 yr (range 32-87) were considered for the study. All patients were affected by WD metastatic NET and had tumor progression in the last 6 months before the enrolment in the study. Median duration of response was 28 months (range 6-50 months). Fourteen patients (60.9%) showed flushing and diarrhea which improved by 85.7% and 55.6%, respectively, bronchoconstrinction and abdominal pain also ameliorated. A complete, partial or no-changed response in the tumor markers behavior was observed, respectively, in 42.9%, 22.9%, and 17.1% of cases. According to RECIST (Response Evaluation Criteria In Solid Tumors) criteria (version 1.1), there were 2 partial regression (8.7%) and 15 stable disease (65.3%); 6 patients (26.0%) progressed. No patient complained from any severe adverse reaction. The results of our study suggest that lanreotide autogel is effective in the symptoms, biochemical markers, and tumor progression control of WD metastatic NET and confirm that the treatment is well tolerated.


Assuntos
Antineoplásicos/uso terapêutico , Géis/uso terapêutico , Tumores Neuroendócrinos/tratamento farmacológico , Peptídeos Cíclicos/uso terapêutico , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/administração & dosagem , Biomarcadores Tumorais/metabolismo , Preparações de Ação Retardada/uso terapêutico , Feminino , Humanos , Injeções Subcutâneas , Masculino , Pessoa de Meia-Idade , Metástase Neoplásica/patologia , Tumores Neuroendócrinos/patologia , Peptídeos Cíclicos/administração & dosagem , Estudos Retrospectivos , Somatostatina/administração & dosagem , Resultado do Tratamento
10.
Int J Immunopathol Pharmacol ; 24(3): 695-702, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21978701

RESUMO

Most autoinflammatory disorders typically come out in the pediatric population, although a limited number of patients may experience disease onset during adulthood. To date, a late disease onset has been described only in familial Mediterranean fever, caused by mutations in the MEFV gene, and in tumor necrosis factor receptor-associated periodic syndrome, caused by mutations in the TNFRSF1A gene. The relative rarity and lack of information on adult-onset autoinflammatory diseases make it likely that mutations will be found in an even smaller percentage of cases. With the aim of improving the genetic diagnosis in adults with suspected autoinflammatory disorders, we recently identified a set of variables related to the probability of detecting gene mutations in MEFV and TNFRSF1A and, in addition, we have also proposed a diagnostic score for identifying those patients at high risk of carrying mutations in these genes. In the present study we evaluated the preliminary score sensitivity and specificity on a wider number of patients in order to validate the goodness of fit of the model. Two hundred and nineteen consecutive patients with a clinical history of periodic fever attacks were screened for mutations in MEFV and TNFRSF1A genes; detailed information about family/personal history and clinical manifestations were also collected. For the validation of the score we considered data both from the 110 patients used to build the preliminary diagnostic score and from the additional 219 patients enrolled in the present study, for a total number of 329 patients. Early age at disease onset, positive family history for recurrent fever episodes, thoracic pain, abdominal pain and skin rash, which are the variables that had previously been shown to be significantly associated with a positive genetic test result (12), were used for validation. On univariate analysis the associations with a positive genetic test were: age at onset (odds ratio [OR] 0.43, p=0.003), positive family history for recurrent fever episodes (OR 5.81, p<0.001), thoracic pain (OR 3.17, p<0.001), abdominal pain (OR 3.80, p<0.001) and skin rash (OR 1.58, p=0.103). The diagnostic score was calculated using the linear combination of the estimated coefficients of the logistic multivariate model (cut-off equals to 0.24) revealing good sensitivity (0.778) and good specificity (0.718). In conclusion, our score may serve in the diagnostic evaluation of adult patients presenting with recurrent fever episodes suspected of having an autoinflammatory disorder, helping identify the few subjects among them who may be carriers of mutations in MEFV and TNFRSF1A genes.


Assuntos
Doenças Hereditárias Autoinflamatórias/diagnóstico , Adolescente , Adulto , Idade de Início , Idoso , Criança , Pré-Escolar , DNA/biossíntese , DNA/genética , Análise Mutacional de DNA , Feminino , Amplificação de Genes , Predisposição Genética para Doença , Heterozigoto , Humanos , Lactente , Modelos Logísticos , Masculino , Curvas de Fluxo-Volume Expiratório Máximo/genética , Pessoa de Meia-Idade , Modelos Biológicos , Razão de Chances , Curva ROC , Receptores Tipo I de Fatores de Necrose Tumoral/genética , Reprodutibilidade dos Testes , População Branca , Adulto Jovem
11.
Reumatismo ; 63(2): 101-10, 2011.
Artigo em Italiano | MEDLINE | ID: mdl-21776447

RESUMO

Systemic autoinflammatory diseases are a group of inherited disorders of the innate immunity characterized by the recurrence of febrile attacks lasting from few hours to few weeks and multi-district inflammation of different severity involving skin, serosal membranes, joints, gastrointestinal tube and central nervous system. The vast majority of these conditions is caused by mutations in genes involved in the control of inflammation and apoptosis mechanisms. The group includes familial Mediterranean fever, mevalonate kinase deficiency syndrome, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes, hereditary pyogenic and granulomatous disorders. Their diagnostic identification derives from the combination of clinical and biohumoral data, though can be sometimes confirmed by genotype analysis.


Assuntos
Análise Mutacional de DNA , Doenças Hereditárias Autoinflamatórias/diagnóstico , Testes Imunológicos , Apoptose/genética , Citocinas/sangue , Estudos de Associação Genética , Doenças Hereditárias Autoinflamatórias/sangue , Doenças Hereditárias Autoinflamatórias/classificação , Doenças Hereditárias Autoinflamatórias/genética , Doenças Hereditárias Autoinflamatórias/imunologia , Humanos , Imunidade Inata , Inflamação/genética , Proteínas S100/análise , Proteína S100A12 , Proteína Amiloide A Sérica/análise
12.
Int J Immunopathol Pharmacol ; 24(4): 827-36, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22230390

RESUMO

Cryopyrin-associated periodic syndromes are categorized as a spectrum of three autoinflammatory diseases, namely familial cold auto-inflammatory syndrome, Muckle-Wells syndrome and chronic infantile neurological cutaneous articular syndrome. All are caused by mutations in the NLRP3 gene coding for cryopyrin and result in active interleukin-1 release: their rarity and shared clinical indicators involving skin, joints, central nervous system and eyes often mean that correct diagnosis is delayed. Onset occurs early in childhood, and life-long therapy with interleukin-1 blocking agents usually leads to tangible clinical remission and inflammatory marker normalization in a large number of patients, justifying the need to facilitate early diagnosis and thus avoid irreversible negative consequences for tissues and organs.


Assuntos
Síndromes Periódicas Associadas à Criopirina , Anti-Inflamatórios/uso terapêutico , Proteínas de Transporte/genética , Síndromes Periódicas Associadas à Criopirina/diagnóstico , Síndromes Periódicas Associadas à Criopirina/tratamento farmacológico , Síndromes Periódicas Associadas à Criopirina/genética , Síndromes Periódicas Associadas à Criopirina/imunologia , Predisposição Genética para Doença , Humanos , Mediadores da Inflamação/metabolismo , Interleucina-1/antagonistas & inibidores , Interleucina-1/metabolismo , Mutação , Proteína 3 que Contém Domínio de Pirina da Família NLR
13.
Int J Immunopathol Pharmacol ; 23(3): 701-7, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20943039

RESUMO

Tumor necrosis factor-alpha receptor (TNFR1)-associated periodic syndrome (TRAPS) is the most common autosomal-dominant autoinflammatory condition and is caused by mutations in the TNFRSF1A gene. TRAPS is characterized by recurrent attacks of fever typically lasting from 1 to 3 weeks; in addition to fever, common clinical features include mainly periorbital oedema, conjunctivitis, a migratory erythematous plaque simulating erysipela with underlying myalgia, and arthritis or arthralgia; serosal membrane inflammation is also possible. The identification of TNFRSF1A mutations as the genetic cause of TRAPS coincided with the wider use of biological agents in medicine and raised the possibility that blocking TNF could potentially represent the primary therapeutic goal in TRAPS, thus disclosing new treatment choices for this complex disease. In the past few years, isolated reports and case-series have been published suggesting that inhibition of TNF-alpha might represent a promising therapeutic approach in TRAPS. We present here our experience with etanercept in the treatment of patients affected with TRAPS, and we also add a review of the literature.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Hereditárias Autoinflamatórias/tratamento farmacológico , Imunoglobulina G/uso terapêutico , Receptores do Fator de Necrose Tumoral/fisiologia , Adulto , Criança , Etanercepte , Feminino , Doenças Hereditárias Autoinflamatórias/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Receptores do Fator de Necrose Tumoral/uso terapêutico , Receptores Tipo I de Fatores de Necrose Tumoral/genética
14.
Int Wound J ; 7(6): 525-30, 2010 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-20726923

RESUMO

Infection of pancreatic necrosis, although present in less than 10% of acute pancreatitis, carries a high risk of mortality; debridment and drainage of necrosis is the treatment of choice, followed by 'open' or 'close' abdomen management. We recently introduced the use of intra-abdominal vacuum sealing after a classic necrosectomy and laparostomy. Two patients admitted to ICU for respiratory insufficiency and a diagnosis of severe acute pancreatitis developed pancreatic necrosis and were treated by necrosectomy, lesser sac marsupialisation and posterior lumbotomic opening. Both of the patients recovered from pancreatitis and a good healing of laparostomic wounds was obtained with the use of the VAC system. Most relevant advantages of this technique seem to be: the prevention of abdominal compartment syndrome, the simplified nursing of patients and the reduction of time to definitive abdominal closure.


Assuntos
Cavidade Abdominal , Tratamento de Ferimentos com Pressão Negativa/métodos , Pancreatite Necrosante Aguda/cirurgia , Cuidados Pós-Operatórios/métodos , Idoso , Síndromes Compartimentais/etiologia , Síndromes Compartimentais/prevenção & controle , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tratamento de Ferimentos com Pressão Negativa/enfermagem , Pancreatectomia/efeitos adversos , Pancreatectomia/métodos , Pancreatite Necrosante Aguda/diagnóstico , Cuidados Pós-Operatórios/enfermagem , Índice de Gravidade de Doença , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Cicatrização
15.
Int J Immunopathol Pharmacol ; 23(4): 1133-41, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-21244762

RESUMO

To date, the rate of detection of autoinflammatory gene mutations in patients suspected of having an autoinflammatory disorder is very low. However, most of these data refer to pediatric populations. The relative rarity and lack of information on adult-onset autoinflammatory diseases make it likely that mutations will be found in an even smaller percentage of cases. Our aim was to develop and validate a set of variables for predicting the risk that a given adult patient presenting with recurrent fever episodes carries mutations in the MEFV or TNFRSF1A genes, in order to increase the probability of obtaining positive results on genetic testing. One hundred and ten consecutive patients with a clinical history of periodic fever attacks were screened for mutations in the TNFRSF1A and the MEFV genes. The mean age at disease onset was 27.85 years. Detailed information about each patient?s family history, personal history, and clinical manifestations were retrospectively collected. A diagnostic score was constructed based on univariate and multivariate analysis in a randomly-selected dataset (training set; n=40). The score was validated on an independent set of the remaining patients (validation set; n=70). Age at onset (odds ratio 0.958, P =0.050), positive family history of recurrent fever episodes (OR 5.738, P = 0.006 ), thoracic pain (OR 7.390, P = 0.002), abdominal pain (OR 2.853, P = 0.038) and skin involvement (OR 8.241, P = 0.003) were independently correlated with a positive genetic test result. A diagnostic score was calculated using the linear combination of the estimated coefficients of the logistic model (cut off equal to 0.24) revealing high sensitivity (0.94), high specificity (0.94) and high accuracy (0.94). We have identified variables that appear to be strongly related to the probability of detecting gene mutations in MEF and TNFRSF1A in adults, thus improving the evaluation of patients with suspected autoinflammatory disorders.


Assuntos
Proteínas do Citoesqueleto/genética , Análise Mutacional de DNA , Febre Familiar do Mediterrâneo/diagnóstico , Mutação , Receptores Tipo I de Fatores de Necrose Tumoral/genética , Adolescente , Adulto , Idade de Início , Idoso , Criança , Pré-Escolar , Febre Familiar do Mediterrâneo/genética , Humanos , Modelos Logísticos , Pessoa de Meia-Idade , Pirina , Curva ROC
17.
Eur Rev Med Pharmacol Sci ; 8(5): 215-7, 2004.
Artigo em Inglês | MEDLINE | ID: mdl-15638233

RESUMO

BACKGROUNDS AND OBJECTIVE: Aim of the present study was to evaluate the clinical correlates of small bowel CT patterns in patients with Crohn's disease (CD), as compared to barium studies and endoscopic findings, as far as parameters of disease activity are concerned. MATERIAL AND METHODS: Thirty five patients with pathologically proven CD were studied by means of helical single detector CT (13) or multidetector CT (22), after administration of low density contrast by mouth (13) or by nasojeunal tube (22). Eight hours later, all patients were studied with barium administered by mouth (13) or with barium and methilcellulose administered by nasojeunal tube (22). Clinical activity was assessed by CDAI score, ESR, CRP, alpha1 glycoprotein and fibrinogen levels. In twenty one patients, colonoscopy was also performed. RESULTS: Sensitivity of small bowel CT versus endoscopy was of 88% while sensitivity of barium studies was of 77% versus endoscopic findings, and it reached 100% for the combination of both exams. We found positive correlations between the detection at CT of "target sign" and a CDAI score > 150 or abnormal values of CRP, ESR, alpha1 glycoprotein. Abnormal ESR or fibrinogen levels were correlated with the detection of fistulas at CT scans. The diameter of enlarged mesenteric lymph nodes was correlated with alpha1 glycoprotein values. No similar correlations were detected for contrast radiology findings. DISCUSSION: This study underscores the clinical usefulness of performing small bowel CT in adjunct to conventional diagnostic studies in Crohn's disease patients. CT findings (either by oral route or nasojeunal tube) correlate with parameters of disease activity.


Assuntos
Doença de Crohn/diagnóstico por imagem , Doença de Crohn/patologia , Intestino Delgado/diagnóstico por imagem , Intestino Delgado/patologia , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X
19.
Eur J Surg Oncol ; 28(5): 523-30, 2002 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-12217306

RESUMO

AIM: Pancreatic cancer is a near fatal disease. External beam radiotherapy and intraoperative radiation therapy (IORT) has been proposed with the aim to improve clinical outcome in resectable tumors. The aim of this study is to assess the feasibility and outcome in patients with cT1-3 pancreatic cancer, treated with surgery, external beam radiotherapy and IORT. METHODS: From 1990 to 1996, 17 patients with clinical stage T1-3N0-1M0 adenocarcinoma of the head of the pancreas were treated with pancreatectomy and pre- (nine patients: 5 Gy), intra- (all patients: 10 Gy) and post-operative (all patients: 50 Gy) radiotherapy. The pathologic T stages were: 4 pT2 and 13 pT3. The pathologic N stages were: 9 pN0 and 8 pN1. Minimum follow-up in living patients was 60 months. RESULTS: No perioperative mortalities were recorded. Two patients showed postoperative morbidity (11.8%) which required a subsequent laparotomy. The disease-free survival at 1, 3 and 5 years was 41, 23 and 18%, respectively (median: 9 months). The overall survival at 1, 3 and 5 years was 70%, 41% and 18%, respectively (median: 17.5 months). Three patients developed local failure (17.6%) and 12 patients showed distant metastases (70.6%). Univariate analysis (logrank) showed: a significant correlation between both N-stage and retroperitoneal involvement (RPI) with local control (N-stage: P=0.0155; RPI:P =0.0295), a significant correlation between maximum tumor size and metastases-free survival (P=0.0167) and overall survival (P=0.0241); the female gender was another predictor of prolonged survival (P= 0.0465). Multivariate analysis (Cox) showed a significant impact of N-stage and retroperitoneal involvement on local control and also a significant correlation between perineural involvement and tumor diameter with metastases-free survival. CONCLUSIONS: These results are similar to those of other published series and suggest that this approach is feasible with acceptable local control and survival, especially in patients with small tumors (<2.5 cm: 5 year survival=33.3%) and in female patients (5 year survival=30%). Due to the impact of gender, tumor diameter and N stage on prognosis, in the design of future trials a stratification of patients based on these categories should be considered. The search of effective chemotherapeutic agents is required, to reduce the high incidence of distant metastases, especially in larger tumors.


Assuntos
Neoplasias Pancreáticas/radioterapia , Neoplasias Pancreáticas/cirurgia , Radioterapia Adjuvante , Idoso , Feminino , Seguimentos , Gastroenteropatias/diagnóstico , Gastroenteropatias/etiologia , Humanos , Incidência , Linfonodos/patologia , Linfonodos/cirurgia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Neoplasias Pancreáticas/mortalidade , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/mortalidade , Prognóstico , Radioterapia Adjuvante/efeitos adversos , Fatores de Tempo , Tomografia Computadorizada por Raios X , Falha de Tratamento
20.
Radiol Med ; 102(1-2): 48-54, 2001.
Artigo em Italiano | MEDLINE | ID: mdl-11677438

RESUMO

AIM: To assess the diagnostic value of three-dimensional (3D) magnetic resonance cholangiography (MRC) versus direct cholangiography such as endoscopic retrograde cholangiography (ERC) and percutaneous transhepatic cholangiography (PTC) in malignant biliary stenosis. MATERIAL AND METHODS: Twenty-nine patients (15 female and 14 male) (mean age 62 years) with malignant biliary strictures underwent MRC and ERC. Breath-hold 3D steady state free precession MR cholangiography was performed on a 1.5-T imager in the patients before ERC. In 25 patients findings at ERC/PTC were considered the standard of reference: 19 patients underwent ERC, 5 PCT and 1 both ERC and PTC due to unsuccessful papilla cannulation during the endoscopic examination. In the 4 remaining patients the surgical specimen was considered the standard of reference. In the 29 patients studied, histology performed during direct cholangiography and the examination of the surgical specimens demonstrated that the malignant hilar stenoses were caused by hilar cholangiocarcinoma (n=7), cholangiocarcinoma of the distal VBP (n=1), gallbladder cancers (n=6), endometrial metastasis (n=2), ovary metastasis (n=1), colon metastasis (n=1), breast metastasis (n=1). The correct identification of biliary stenosis and extension of the tumor (according to the Bismuth classification) by MR cholangiography and ERC were independently assessed by two readers blinded to each other's report. The results were compared. RESULTS: Identification of biliary stenosis and neoplastic extension were accurate in respectively 29/29 (100%) and 26/29 (89%) cases with MR cholangiography. The comparison of ERC/PTC and MRC images yielded the following results: Bismuth Type I (6 vs 6), Type II (5 vs 8), Type III (13 vs 10), Type IV (5 vs 5). Our results indicate that MR is less capable of identifying the extension of small lesions at the primary confluence of bile ducts than are ERC/PCT. DISCUSSION AND CONCLUSIONS: MR cholangiography is a non-invasive technique for biliary tract imaging. It does not require administration of contrast medium and allows complete visualisation of the biliary ducts. MR cholangiography allowed accurate diagnosis of malignant hilar stenosis providing equal information as direct cholangiography and may therefore obviate the need for ERC/PTC.


Assuntos
Neoplasias dos Ductos Biliares/complicações , Colangiografia , Colestase/diagnóstico , Imageamento por Ressonância Magnética , Idoso , Idoso de 80 Anos ou mais , Colangiografia/métodos , Colestase/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
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