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121.
Hassan Awada Reda Z. Mahfouz Ashwin Kishtagari Teodora Kuzmanovic Jibran Durrani Cassandra M. Kerr Bhumika J. Patel Valeria Visconte Tomas Radivoyevitch Alan Lichtin Hetty E. Carraway Jaroslaw P. Maciejewski Yogen Saunthararajah 《British journal of haematology》2020,188(6):924-929
The nucleoside analogue decitabine can deplete the epigenetic regulator DNA methyltransferase 1 (DNMT1), an effect that occurs, and is saturated at, low concentrations/doses. A reason to pursue this molecular-targeted effect instead of the DNA damage/cytotoxicity produced with high concentrations/doses, is that non-cytotoxic DNMT1-depletion can cytoreduce even p53-null myeloid malignancies while sparing normal haematopoiesis. We thus identified minimum doses of decitabine (0·1–0·2 mg/kg) that deplete DNMT1 without off-target anti-metabolite effects/cytotoxicity, and then administered these well-tolerated doses frequently 1–2X/week to increase S-phase dependent DNMT1-depletion, and used a Myeloid Malignancy Registry to evaluate long-term outcomes in 69 patients treated this way. Consistent with the scientific rationale, treatment was well-tolerated and durable responses were produced (~40%) in genetically heterogeneous disease and the very elderly. 相似文献
122.
Hassan Awada Reda Z. Mahfouz Jibran Durrani Ashwin Kishtagari Deepa Jagadeesh Alan E. Lichtin Brian T. Hill Betty K. Hamilton Hetty E. Carraway Aziz Nazha Navneet S. Majhail Ronald Sobecks Valeria Visconte Matt Kalaycio Mikkael A. Sekeres Jaroslaw P. Maciejewski 《British journal of haematology》2020,189(2):318-322
T-cell large granular lymphocytic leukaemia (T-LGLL) is a chronic clonal lymphoproliferative disorder of cytotoxic T lymphocytes which commonly occurs in older patients and is often associated with autoimmune diseases. Among 246 patients with T-LGLL seen at our institution over the last 10 years, we encountered 15 cases following solid organ or haematopoietic stem cell transplantation. Here, we studied the clinical characterization of these cases and compared them to de novo T-LGLL. This experience represented a clear picture of the intricate nature of the disease manifestation and the complexities of several immune mechanisms triggering the clonal expansion. 相似文献
123.
Andrea Mazzanti Ajita Kanthan Nicola Monteforte Mirella Memmi Raffaella Bloise Valeria Novelli Carlotta Miceli Sean O'Rourke Gianluca Borio Agnieszka Zienciuk-Krajka Antonio Curcio Andreea Elena Surducan Mario Colombo Carlo Napolitano Silvia G. Priori 《Journal of the American College of Cardiology》2014
124.
Zampella Emilia Acampa Wanda Assante Roberta Gaudieri Valeria Nappi Carmela Mannarino Teresa Mainolfi Ciro Gabriele Arumugam Parthiban Petretta Mario Cuocolo Alberto 《European journal of nuclear medicine and molecular imaging》2020,47(7):1698-1704
European Journal of Nuclear Medicine and Molecular Imaging - Cardiac imaging with positron emission tomography/computed tomography (PET/CT) allows measurement of coronary artery calcium (CAC),... 相似文献
125.
126.
Lucie Kalisova Jiri Raboch Alexander Nawka Gaia Sampogna Libor Cihal Thomas W. Kallert Georgi Onchev Anastasia Karastergiou Valeria del Vecchio Andrzej Kiejna Tomasz Adamowski Francisco Torres-Gonzales Jorge A. Cervilla Stephan Priebe Domenico Giacco Lars Kjellin Algirdas Dembinskas Andrea Fiorillo 《Social psychiatry and psychiatric epidemiology》2014,49(10):1619-1629
Purpose
This study aims to identify whether selected patient and ward-related factors are associated with the use of coercive measures. Data were collected as part of the EUNOMIA international collaborative study on the use of coercive measures in ten European countries.Methods
Involuntarily admitted patients (N = 2,027) were divided into two groups. The first group (N = 770) included patients that had been subject to at least one of these coercive measures during hospitalization: restraint, and/or seclusion, and/or forced medication; the other group (N = 1,257) included patients who had not received any coercive measure during hospitalization. To identify predictors of use of coercive measures, both patients’ sociodemographic and clinical characteristics and centre-related characteristics were tested in a multivariate logistic regression model, controlled for countries’ effect.Results
The frequency of the use of coercive measures varied significantly across countries, being higher in Poland, Italy and Greece. Patients who received coercive measures were more frequently male and with a diagnosis of psychotic disorder (F20–F29). According to the regression model, patients with higher levels of psychotic and hostility symptoms, and of perceived coercion had a higher risk to be coerced at admission. Controlling for countries’ effect, the risk of being coerced was higher in Poland. Patients’ sociodemographic characteristics and ward-related factors were not identifying as possible predictors because they did not enter the model.Conclusions
The use of coercive measures varied significantly in the participating countries. Clinical factors, such as high levels of psychotic symptoms and high levels of perceived coercion at admission were associated with the use of coercive measures, when controlling for countries’ effect. These factors should be taken into consideration by programs aimed at reducing the use of coercive measures in psychiatric wards. 相似文献127.
Fiore Manganelli Raffaele Dubbioso Rosa Iodice Antonietta Topa Andrea Dardis Cinzia Valeria Russo Lucia Ruggiero Stefano Tozza Alessandro Filla Lucio Santoro 《Journal of neurology》2014,261(4):804-808
Adult patients with Niemann-Pick disease type C (NPC) usually develop cognitive impairment progressing to dementia, whose pathophysiology remains still unclear. Noteworthy parallels exist in cognitive impairment and cellular pathology of NPC and Alzheimer’s disease (AD). In particular, alterations of cholinergic system, which represent one of the pathological hallmarks and contribute to cognitive deterioration in AD, have recently been demonstrated in a human brain autopsy and in an experimental model of NPC. This finding raised the issue that central cholinergic circuits dysfunction may contribute to pathophysiology of cognitive impairment in NPC as well, and prompted us to evaluate the cholinergic functional involvement in NPC patients by applying a neurophysiologic technique, named short-latency afferent inhibition (SAI). We describe clinical, biochemical, molecular and neuropsychological features, and SAI findings in three patients affected by NPC. Diagnosis of NPC was assessed by molecular analysis of the NPC1 gene in all patients. In two of them, biochemical analysis of intracellular accumulation of unesterified cholesterol was also performed. The main clinical features were cerebellar ataxia, vertical supranuclear gaze palsy and a variable degree of cognitive impairment ranging from only memory impairment to severe dementia. Electrophysiological evaluation revealed a reduced SAI in all three patients. Our SAI findings provide evidence of cholinergic dysfunction in patients with the adult form of NPC, supporting that cholinergic alterations may play a role in cognitive impairment in NPC, and strengthening the similarities between NPC and AD. 相似文献
128.
129.
Dimitri Poddighe Lorenzo Cavagna Valeria Brazzelli Paola Bruni Gian Luigi Marseglia 《Autoimmunity reviews》2014,13(11):1142-1148
Juvenile dermatomyositis is an immune-mediated inflammatory multi-system disease involving mainly striated muscles and skin. Typical dermatological features are fundamental to establish the diagnosis, especially whenever the myopathy is very mild or absent, as it occurs in the form called as amyopathic juvenile dermatomyositis. Sometimes, systemic rheumatic diseases can develop a hyperferritinemia syndrome characterized by hemophagocytosis, namely macrophage activation syndrome, which represents a severe and life-threatening complication. Here, we describe a complex clinical history characterized by a hyper-ferritinemia syndrome after infectious mononucleosis, leading to recurrent episodes of macrophage activation syndrome. Finally, the late onset of several skin changes brought to a diagnosis of amyopathic juvenile dermatomyositis. 相似文献
130.