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Sample records for chronic myeloproliferative disorders

  1. Pulmonary hypertension in patients with chronic myeloproliferative disorders

    OpenAIRE

    Yochai Adir; Davide Elia; Sergio Harari

    2015-01-01

    Pulmonary hypertension (PH) is a major complication of several haematological disorders. Chronic myeloproliferative diseases (CMPDs) associated with pulmonary hypertension have been included in group five of the clinical classification for pulmonary hypertension, corresponding to pulmonary hypertension for which the aetiology is unclear and/or multifactorial. The aim of this review is to discuss the epidemiology, pathogenic mechanism and treatment approaches of the more common forms of pulmon...

  2. Chronic Myeloproliferative Neoplasms Treatment

    Science.gov (United States)

    ... Cancers by Body Location Childhood Cancers Adolescent & Young Adult Cancers Metastatic Cancer Recurrent Cancer Research NCI’s Role in ... on the hands and feet. Muscle pain. Itching. Diarrhea . Stages of Chronic Myeloproliferative Neoplasms Key Points There is no standard staging system ...

  3. The mevalonate pathway as a therapeutic target in the Ph-negative chronic myeloproliferative disorders

    DEFF Research Database (Denmark)

    Hasselbalch, Hans Carl; Riley, Caroline H

    2007-01-01

    The Ph-negative chronic myeloproliferative disorders (CMPDs) polycythaemia vera, essential thrombocytosis and idiopathic myelofibrosis are acquired stem cell disorders, which pathophysiologically are featured by clonal myeloproliferation and accumulation of myeloid cells, the latter being...... consequent to decreased apoptosis. Myelofibrosis and neoangiogenesis in the bone marrow and spleen are the histopathological hallmarks of idiopathic myelofibrosis but may develop in the other diseases as well. In patients with myelofibrosis elevated levels of circulating CD34+ cells are highly characteristic...... being partly explained by a proteolytic bone marrow mileu owing to excessive release of various proteases with ensuing extracellular matrix degradation and constitutive mobilisation of CD34+ cells into the peripheral blood. Thrombohaemorrhagic complications are major clinical problems contributing...

  4. Clinical significance of serum soluble interleukin-2 receptor in chronic myeloproliferative disorders.

    Science.gov (United States)

    Kawatani, T; Endo, A; Tajima, F; Ooi, S; Kawasaki, H

    1997-02-01

    Serum soluble interleukin-2 receptor (sIL-2R) levels were determined in patients with chronic myeloproliferative disorders (CMPD): 18 with chronic myelogenous leukemia in chronic phase (CML in CP), seven with CML in accelerated phase (AP) or blastic crisis (BC), six with polycythemia vera (PV), eight with essential thrombocythemia (ET), one with primary myelofibrosis (PMF), and 50 controls. The mean (+/-S.E.M.) levels were higher in CMPD than in controls (CML in AP or BC, 2693 +/- 694 U/ml, P 2R levels than patients with ET (P 2R levels were positively correlated with WBC count and lactic dehydrogenase in CMPD, and in CML in CP. Serum sIL-2R levels in CMPD were negatively correlated with RBC and platelet counts. Serum sIL-2R levels were significantly lower in patients with CML in CP who showed a cytogenetic response after interferon (IFN) therapy than in those who showed no response (P 2R level reflects the leukocyte growth in CMPD and is useful both for differentiating CML from other CMPD and for predicting the response to IFN therapy in CML. PMID:9071816

  5. Pathogenesis of Myeloproliferative Disorders.

    Science.gov (United States)

    Nangalia, Jyoti; Grinfeld, Jacob; Green, Anthony R

    2016-05-23

    Myeloproliferative neoplasms (MPNs) are a set of chronic hematopoietic neoplasms with overlapping clinical and molecular features. Recent years have witnessed considerable advances in our understanding of their pathogenetic basis. Due to their protracted clinical course, the evolution to advanced hematological malignancies, and the accessibility of neoplastic tissue, the study of MPNs has provided a window into the earliest stages of tumorigenesis. With the discovery of mutations in CALR, the majority of MPN patients now bear an identifiable marker of clonal disease; however, the mechanism by which mutated CALR perturbs megakaryopoiesis is currently unresolved. We are beginning to understand better the role of JAK2(V617F) homozygosity, the function of comutations in epigenetic regulators and spliceosome components, and how these mutations cooperate with JAK2(V617F) to modulate MPN phenotype. PMID:27193452

  6. The JAK2 V617F mutation involves B- and T-lymphocyte lineages in a subgroup of patients with Philadelphia-chromosome negative chronic myeloproliferative disorders

    DEFF Research Database (Denmark)

    Larsen, Thomas Stauffer; Christensen, Jacob Haaber; Hasselbalch, Hans Carl;

    2007-01-01

    The JAK2 V617F mutation is a frequent genetic event in the three classical Philadelphia-chromosome negative chronic myeloproliferative disorders (Ph(neg.)-CMPD), polycythemia vera (PV), essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). Its occurrence varies in frequency in regards...

  7. Bone morbidity in chronic myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Farmer, Sarah; Ocias, Lukas Frans; Vestergaard, Hanne;

    2015-01-01

    Patients with the classical Philadelphia chromosome-negative chronic myeloproliferative neoplasms including essential thrombocythemia, polycythemia vera and primary myelofibrosis often suffer from comorbidities, in particular, cardiovascular diseases and thrombotic events. Apparently, there is also...... neoplasms. Chronic inflammation has been suggested to explain the initiation of clonal development and progression in chronic myeloproliferative neoplasms. Decreased bone mineral density and enhanced fracture risk are well-known manifestations of many chronic systemic inflammatory diseases. As opposed to...... systemic mastocytosis (SM) where pathogenic mechanisms for bone manifestations probably involve effects of mast cell mediators on bone metabolism, the mechanisms responsible for increased fracture risk in other chronic myeloproliferative neoplasms are not known....

  8. Hematopoietic Neoplasias in Horses: Myeloproliferative and Lymphoproliferative Disorders

    OpenAIRE

    Muñoz, Ana; Riber, Cristina; Trigo, Pablo; Castejón, Francisco

    2010-01-01

    Leukemia, i.e., the neoplasia of one or more cell lines of the bone marrow, although less common than in other species, it is also reported in horses. Leukemia can be classified according to the affected cells (myeloproliferative or lymphoproliferative disorders), evolution of clinical signs (acute or chronic) and the presence or lack of abnormal cells in peripheral blood (leukemic, subleukemic and aleukemic leukemia). The main myeloproliferative disorders in horses are malignant histiocytosi...

  9. Philadelphia-negative chronic myeloproliferative neoplasms

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    Rosane Isabel Bittencourt

    2012-01-01

    Full Text Available Chronic myeloproliferative diseases without the Philadelphia chromosome marker (Ph-, although first described 60 years ago, only became the subject of interest after the turn of the millennium. In 2001, the World Health Organization (WHO defined the classification of this group of diseases and in 2008 they were renamed myeloproliferative neoplasms based on morphological, cytogenetic and molecular features. In 2005, the identification of a recurrent molecular abnormality characterized by a gain of function with a mutation in the gene encoding Janus kinase 2 (JAK2 paved the way for greater knowledge of the pathophysiology of myeloproliferative neoplasms. The JAK2 mutation is found in 90-98% of polycythemia vera and in about 50% essential thrombocytosis and primary myelofibrosis. In addition to the JAK2 mutation, other mutations involving TET2 (ten-eleven translocation, LNK (a membrane-bound adaptor protein; IDH1/2 (isocitrate dehydrogenase 1/2 enzyme; ASXL1 (additional sex combs-like 1 genes were found in myeloproliferative neoplasms thus showing the importance of identifying molecular genetic alterations to confirm diagnosis, guide treatment and improve our understanding of the biology of these diseases. Currently, polycythemia vera, essential thrombocytosis, myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia and mastocytosis are included in this group of myeloproliferative neoplasms, but are considered different situations with individualized diagnostic methods and treatment. This review updates pathogenic aspects, molecular genetic alterations, the fundamental criteria for diagnosis and the best approach for each of these entities.

  10. Treatment Option Overview (Chronic Myeloproliferative Neoplasms)

    Science.gov (United States)

    ... Cancers by Body Location Childhood Cancers Adolescent & Young Adult Cancers Metastatic Cancer Recurrent Cancer Research NCI’s Role in ... on the hands and feet. Muscle pain. Itching. Diarrhea . Stages of Chronic Myeloproliferative Neoplasms Key Points There is no standard staging system ...

  11. General Information about Chronic Myeloproliferative Neoplasms

    Science.gov (United States)

    ... Cancers by Body Location Childhood Cancers Adolescent & Young Adult Cancers Metastatic Cancer Recurrent Cancer Research NCI’s Role in ... on the hands and feet. Muscle pain. Itching. Diarrhea . Stages of Chronic Myeloproliferative Neoplasms Key Points There is no standard staging system ...

  12. Treatment Options for Chronic Myeloproliferative Neoplasms

    Science.gov (United States)

    ... Cancers by Body Location Childhood Cancers Adolescent & Young Adult Cancers Metastatic Cancer Recurrent Cancer Research NCI’s Role in ... on the hands and feet. Muscle pain. Itching. Diarrhea . Stages of Chronic Myeloproliferative Neoplasms Key Points There is no standard staging system ...

  13. Nuclear magnetic resonance investigation of erythrocyte membranes in chronic myeloproliferative disorders.

    Science.gov (United States)

    Morariu, V V; Petrov, L

    1986-07-01

    The temperature dependence of the apparent water diffusional exchange through erythrocyte membranes in cases of policitemia vera, chronic granulocytic leukemia and primary myelofibrosis was measured by using a nuclear magnetic resonance method in the presence of Mn2+. The thermal transition shifted to lower temperatures in all cases, regardless of the stage of the disease, suggesting a structural alteration of the membrane. The shift of transition indirectly suggests a lower penetration of the erythrocytes by Mn2+. The water exchange time at 37 degrees C also increased, mainly in the blast crisis; it seems to have a prognostic value of some clinical interest. No simple correlation of the water exchange and the following clinical investigations was observed: the white count, the percentage of promyelocites and myeloblasts, the sedimentation rate of blood, the osmotic fragility of erythrocytes, the total concentration of proteins, albumin and immunoglobulins, respectively, in plasma. PMID:3742482

  14. Inheritance of the chronic myeloproliferative neoplasms. A systematic review

    DEFF Research Database (Denmark)

    Ranjan, Ajenthen; Penninga, E; Jelsig, Am;

    2012-01-01

    This systematic review investigated the inheritance of the classical chronic myeloproliferative neoplasms (MPNs) including polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF) and chronic myelogenous leukemia (CML). Sixty-one articles were included and provided 135...

  15. Leucemia mieloide crônica e outras doenças mieloproliferativas crônicas Chronic myeloid leukemia and other chronic myeloproliferative disorders

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    Vaneuza M. Funke

    2010-05-01

    HSCT have been restricted as a result of mortality related to this procedure and imatinib mesylate has become the new treatment of choice for this disease. In Brazil and possibly in other developing countries, socio-economic conditions make HSCT still feasible as first-line treatment in some situations. The HSCT remains indicated for Ph negative myeloproliferative disorders such as high risk myelofibrosis or patients with polycythemia vera or essential thrombocytosis that have evolved to myelofibrosis with high-risk features.

  16. An update of molecular pathogenesis and diagnosis of myeloproliferative disorders in the JAK2 era

    Institute of Scientific and Technical Information of China (English)

    ZHANG Su-jiang; LI Jian-yong

    2008-01-01

    @@ Myeloproliferative disorders (MPD) are clonal haematopoietic stem cell disorders characterized by proliferation of one or more myeloid cell lineages in the bone marrow and increased numbers of mature and immature cells in the peripheral blood. MPDs are classified into five categories: polycythemia vera (PV),essential thrombocythaemia (ET), idiopathic myelofibrosis (IMF), chronic myelogenous leukaemia(CML) and atypical MPD.

  17. TPO, but not soluble-IL-6 receptor, levels increase after anagrelide treatment of thrombocythemia in chronic myeloproliferative disorders

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    Jan Palmblad, Magnus Björkholm, Jack Kutti, Gerd Lärfars, Eva Löfvenberg, Berit Markevärn, Mats Merup, Nils Mauritzson, Jan Westin, Jan Samuelsson, Gunnar Birgegård

    2008-01-01

    Full Text Available Anagrelide is often used in the treatment of thrombocythemia in myeloproliferative disease (MPD, but information concerning effects of treatment on cytokines involved in regulation of blood platelet levels is limited. Here, we investigated serum levels of thrombopoietin (TPO and soluble IL-6 receptor (sIL-6R in relation to response to treatment with and plasma concentrations of anagrelide. Samples from 45 patients with thrombocythemia due to MPD (ET=31, PV=14, being treated with anagrelide for 6 months, were analyzed for TPO, sIL-6R and anagrelide levels. The mean baseline platelet count was 983x109/L. A reduction of platelets to <600 in asymptomatic or <400 x 109/L in symptomatic patients was defined as a complete remission (CR, a reduction with >50% of baseline as partial remission, and <50% reduction as failure. At 6 months, 35 patients were in CR, 1 had a partial remission and 9 were treatment failures. For all patients, there was an increase in TPO of 44% from baseline; this change was more pronounced for patients with partial remission and failure. sIL-6R levels did not change significantly. There was no correlation between levels of anagrelide and cytokine levels at 6 months, and changes of cytokine levels did not relate to changes of platelet counts. Thus, a pronounced increase of TPO levels after 6 months of anagrelide treatment indicated that this treatment affected a major regulatory mechanism for megakaryocyte and platelet formation in MPD.

  18. Molecular approach to diagnose BCR/ABL negative chronic myeloproliferative neoplasms

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    Michelle Maccarini Barcelos

    2011-01-01

    Full Text Available Chronic myeloproliferative neoplasms arise from clonal proliferation of hematopoietic stem cells. According to the World Health Organization myeloproliferative neoplasms are classified as: chronic myelogenous leukemia, polycythemia vera, essential thrombocythemia, primary myelofibrosis, chronic neutrophilic leukemia, chronic eosinophilic leukemia, hypereosinophilic syndrome, mast cell disease, and unclassifiable myeloproliferative neoplasms. In the revised 2008 WHO diagnostic criteria for myeloproliferative neoplasms, mutation screening for JAK2V617F is considered a major criterion for polycythemia vera diagnosis and also for essential thrombocythemia and primary myelofibrosis, the presence of this mutation represents a clonal marker. There are currently two hypotheses explaining the role of the JAK2V617F mutation in chronic myeloproliferative neoplasms. According to these theories, the mutation plays either a primary or secondary role in disease development. The discovery of the JAK2V617F mutation has been essential in understanding the genetic basis of chronic myeloproliferative neoplasms, providing some idea on how a single mutation can result in three different chronic myeloproliferative neoplasm phenotypes. But there are still some issues to be clarified. Thus, studies are still needed to determine specific molecular markers for each subtype of chronic myeloproliferative neoplasm.

  19. Cerebral haemorrhage as the presenting feature of myeloproliferative disorder

    OpenAIRE

    Kondlapudi, Jyothi; O’Connor, Rory J; Mawer, Samantha

    2009-01-01

    Myeloproliferative disorders predispose individuals to bleeding and thrombosis, often with devastating consequences. We report a 41-year-old man who presented with headache, amnesia and dysphagia due to cerebral haemorrhage. Extensive investigation revealed the cause of the neurological syndrome as an underlying essential thrombocytosis. The patient made a full recovery following extensive inpatient and community rehabilitation, returning to work after 6 months. We discuss the diagnosis and m...

  20. Transient Myeloproliferative Disorder and Down Syndrome: Is there a link?

    OpenAIRE

    Reyes, Zenaida S.; Wafa Bashir; Anil Pathare

    2012-01-01

    An extremely premature male neonate presented with an unusual multisystem dysfunction within the first 24 to 48 hours of life. The unfolding of clinical events and investigations revealed a transient myeloproliferative disorder (TMD). TMD was the main indication for karyotyping of this premature infant without clinical symptoms of Down syndrome. The awareness of TMD in a newborn warrants karyotype analysis to look for trisomy 21 and a close surveillance because of its potential progression to...

  1. Transient Myeloproliferative Disorder and Down Syndrome; Is there a link?

    Directory of Open Access Journals (Sweden)

    Zenaida S. Reyes

    2012-11-01

    Full Text Available An extremely premature male neonate presented with an unusual multisystem dysfunction within the first 24 to 48 hours of life. The unfolding of clinical events and investigations revealed a transient myeloproliferative disorder (TMD. TMD was the main indication for karyotyping of this premature infant without clinical symptoms of Down syndrome. The awareness of TMD in a newborn warrants karyotype analysis to look for trisomy 21 and a close surveillance because of its potential progression to true leukaemia.

  2. Chronic myeloproliferative neoplasms and subsequent cancer risk: a Danish population-based cohort study

    DEFF Research Database (Denmark)

    Frederiksen, Henrik Kronholm; Farkas, Dóra Körmendiné; Christiansen, Christian Fynbo;

    2011-01-01

    Patients with chronic myeloproliferative neoplasms, including essential thrombocythemia (ET), polycythemia vera (PV), and chronic myeloid leukemia (CML), are at increased risk of new hematologic malignancies, but their risk of nonhematologic malignancies remains unknown. In the present study, we...... diagnosed with a chronic myeloproliferative neoplasm during 1977-2008. We compared the incidence of subsequent cancer in this cohort with that expected on the basis of cancer incidence in the general population (standardized incidence ratio). Overall, ET, PV, and CML patients were at increased risk of...... conclude that patients with chronic myeloproliferative neoplasms are at increased risk of developing a new malignant disease. © 2011 by The American Society of Hematology....

  3. Are chronic myeloproliferative neoplasms associated with age-related macular degeneration?

    DEFF Research Database (Denmark)

    Bak, M.; Sorensen, T. L.; Flachs, E. M.;

    2015-01-01

    Background. Patients with Chronic Myeloproliferative Neoplasms (MPNs) already have increased comorbidity at time of diagnosis, and studies show association with ophthalmic manifestations. Retinal vascular symptoms including vascular occlusions and hemorrhages are present, but other manifestations...

  4. What is the Incidence of Kidney Stones after Chemotherapy in Patients with Lymphoproliferative or Myeloproliferative Disorders?

    OpenAIRE

    Hossein S. Mirheydar; Pooya Banapour; Rustin Massoudi; Palazzi, Kerrin L.; Ramzi Jabaji; Reid, Erin G.; Frederick E. Millard; Christopher J. Kane; Sur, Roger L.

    2014-01-01

    Introduction This study describes the incidence and risk factors of de novo nephrolithiasis among patients with lymphoproliferative or myeloproliferative diseases who have undergone chemotherapy. Materials and Methods From 2001 to 2011, patients with lymphoproliferative or myeloproliferative disorders treated with chemotherapy were retrospectively identified. The incidence of image proven nephrolithiasis after chemotherapy was determined. ...

  5. Is JAK2V617F Mutation the Only Factor for Thrombosis in Philadelphia-Negative Chronic Myeloproliferative Neoplasms?

    Science.gov (United States)

    Uyanik, Mehmet Sevki; Baysal, Mehmet; Pamuk, Gulsum Emel; Maden, Muhammet; Akker, Mustafa; Umit, Elif Gulsum; Demir, Muzaffer; Aydogdu, Erkan

    2016-09-01

    The most common genetic disorder in Philadelphia negative chronic myeloproliferative neoplasms is the JAK2-V617F mutation. In the present study, we aimed to determine risk factors for thrombosis in patients with essential thrombocytosis and polycythemia vera. We screened the medical records of 101 patients. Risk factors which may predict thrombosis were recorded. Venous thrombosis (VT) before diagnosis was significantly higher in JAK2 positive patients. VT after diagnosis was similar in JAK2 positive and negative groups, and was significantly higher in elderly patients. Treatment places importance on the JAK2 mutation under unmodifiable cardiovascular risk factors such as advanced age after diagnosis. PMID:27429517

  6. Interferon-alpha in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms. Status and perspectives

    DEFF Research Database (Denmark)

    Hasselbalch, Hans Carl; Larsen, Thomas Stauffer; Riley, Caroline Hasselbalch;

    2011-01-01

    The Philadelphia-negative chronic myeloproliferative neoplasms encompass essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF). A major break-through in the understanding of the pathogenesis of these neoplasms occurred in 2005 by the discovery of the JAK2 V617F...

  7. Recent advances in the bcr-abl negative chronic myeloproliferative diseases

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    Stroncek David F

    2006-10-01

    Full Text Available Abstract The chronic myeloproliferative disorders are clonal hematopoietic stem cell disorders of unknown etiology. In one of these (chronic myeloid leukemia, there is an associated pathognomonic chromosomal abnormality known as the Philadelphia chromosome. This leads to constitutive tyrosine kinase activity which is responsible for the disease and is used as a target for effective therapy. This review concentrates on the search in the other conditions (polycythemia vera, essential thrombocythemia and idiopathic mylofibrosis for a similar biological marker with therapeutic potential. There is no obvious chromosomal marker in these conditions and yet evidence of clonality can be obtained in females by the use of X-inactivation patterns. PRV-1mRNA over expression, raised vitamin B12 levels and raised neutrophil alkaline phosphatase scores are evidence that cells in these conditions have received excessive signals for proliferation, maturation and reduced apoptosis. The ability of erythroid colonies to grow spontaneously without added external erythropoietin in some cases, provided a useful marker and a clue to this abnormal signaling. In the past year several important discoveries have been made which go a long way in elucidating the involved pathways. The recently discovered JAK2 V617F mutation which occurs in the majority of cases of polycythemia vera and in about half of the cases with the two other conditions, enables constitutive tyrosine kinase activity without the need for ligand binding to hematopoietic receptors. This mutation has become the biological marker for these conditions and has spurred the development of a specific therapy to neutralize its effects. The realization that inherited mutations in the thrombopoietin receptor (c-Mpl can cause a phenotype of thrombocytosis such as in Mpl Baltimore (K39N and in a Japanese family with S505A, has prompted the search for acquired mutations in this receptor in chronic myeloproliferative

  8. Increased gene expression of histone deacetylases in patients with Philadelphia-negative chronic myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Skov, Vibe; Larsen, Thomas Stauffer; Thomassen, Mads;

    2012-01-01

    Abstract Myeloproliferation, myeloaccumulation (decreased apoptosis), inflammation, bone marrow fibrosis and angiogenesis are cardinal features of the Philadelphia-negative chronic myeloproliferative neoplasms: essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF...... proteins in favor of apoptosis (enhanced apoptosis) and also to inhibit angiogenesis. Recently, enhanced HDAC enzyme activity has been found in CD34+cells from patients with PMF, enzyme activity levels highly exceeding those recorded in other chronic myeloproliferative neoplasms (CMPNs). The raised levels...... correlated to the degree of splenomegaly, suggesting that HDAC might be recruited as ET or PV progresses into myelofibrosis or PMF progresses into a more advanced stage. Accordingly, HDAC inhibition is an obvious novel therapeutic approach in these neoplasms. Using global gene expression profiling of whole...

  9. Clinical Features of 294 Turkish Patients with Chronic Myeloproliferative Neoplasms

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    Neslihan Andıç

    2016-08-01

    Full Text Available Objective: Myeloproliferative neoplasms (MPNs share common clonal stem cells but show significant differences in their clinical courses. The aim of this retrospective study was to evaluate thrombotic and hemorrhagic complications, JAK2 status, gastrointestinal and cardiac changes, treatment modalities, and survival in MPNs in Turkish patients. Materials and Methods: Medical files of 294 patients [112 essential thrombocythemia (ET, 117 polycythemia vera (PV, 46 primary myelofibrosis, and 19 unclassified MPN cases] from 2 different universities in Turkey were examined. Results: Older age, higher leukocyte count at diagnosis, and JAK2 mutation positivity were risk factors for thrombosis. Platelet count over 1000x109/L was a risk factor for hemorrhagic episodes. Hydroxyurea treatment was not related to leukemic transformation. Median follow-up time was 50 months (quartiles: 22.2-81.75 in these patients. Patients with primary myelofibrosis had the shortest survival of 137 months when compared with 179 months for ET and 231 months for PV. Leukemic transformation, thromboembolic events, age over 60 years, and anemia were found to be the factors affecting survival. Conclusion: Thromboembolic complications are the most important preventable risk factors for morbidity and mortality in MPNs. Drug management in MPNs is done according to hemoglobin and platelet counts. Based on the current study population our results support the idea that leukocytosis and JAK2 positivity are more important risk factors for thrombosis than hemoglobin and platelet values.

  10. 妊娠合并慢性骨髓增殖性疾病11例临床分析%Clinical analysis of eleven patients with chronic myeloproliferative disorders complicating pregnancy

    Institute of Scientific and Technical Information of China (English)

    白月婷; 张超; 王建六; 梁梅英; 张晓红

    2010-01-01

    Objective To investigate the clinical characteristics, the antenatal management, the outcome and prognosis of chronic myeloproliferative disorders (CMPD) complicating pregnancy. Methods Retrospectively analyze the clinical data of eleven patients with CMPD complicating pregnancy hospitalized in Peking University People' s Hospital from 2000 to 2009, including five patients with essential thrombocythemia, one with primary myelofibrosis and five with chronic myeloid leukemia. Results (1)Five pregnancies had periodic antenatal care and laboratory monitorings like full blood count. Reasonable anti-coagulation therapy was given to prevent the complications. One patient with PMF diagnosed before conception had her first pregnancy ended with mild pre-eclampsia and intrauterine death at the gestational age of 32 weeks. During the first trimester of her second pregnancy two years later, the test for anti-β2 glycoprotein antibody was positive. She received low-dose aspirin and low-molecular-weight heparin as anticoagulants. An uneventful course was obtained and she delivered a healthy term infant. (2) Five pregnancies had occasional antenatal examination, including two patients with ET and three patients with CML One patient with ET developed severe pre-eclampsia at the gestational age of 25 weeks. Umbilical artery Doppler showed reversed end-diastolic velocity. The management with anti-convulsants, antihypertensives and anti-coagulants showed no effect. An emergency cesarean section had to be performed because of the aggressive hypertension and placental abruption, with still birth as a result. Two pregnancies never had an antenatal care. Both of them were admitted on labor and the diagnoses of CML were made. (3)Four pregnancies developed oligohydramnios and three developed preelampsia(two severe pre-eclampsia and one mild pre-eclampsia). There was no other hemorrhage and thrombosis event. (4) Eight pregnancies reached full-term with four cesarean sections and four vaginal

  11. Detection of V617F mutation of gene jak2 at patients with chronic myeloproliferative neoplasms

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    Maliuta S. S.

    2010-04-01

    Full Text Available The aim of the work was to create a protocol for detecting the V617F mutation of the gene jak2 in samples of patients with chronic myeloproliferative neoplasm which is necessary to unify the procedures of the analysis of blood samples according to WHO criteria for this group of diseases. Methods. Mutation was revealed using reverse transcriptase PCR and direct sequencing of PCR products. Results. Six samples of blood of patients with polycythemia vera were analyzed and the mutation V617F was detected in all six cases. This mutation was not detected in any of RNA samples of healthy donors. A case of simultaneous detection of mutations V617F and fused bcr/abl gene in CML patient was described. Conclusions. The proposed method for detecting the V617F mutation allows molecular genetic differential diagnosis of myeloproliferative neoplasm as well

  12. Review of current classification, molecular alterations, and tyrosine kinase inhibitor therapies in myeloproliferative disorders with hypereosinophilia

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    Havelange V

    2013-08-01

    Full Text Available Violaine Havelange,1,2 Jean-Baptiste Demoulin1 1de Duve Institute, Université catholique de Louvain, Brussels, Belgium; 2Department of Hematology, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium Abstract: Recent advances in our understanding of the molecular mechanisms underlying hypereosinophilia have led to the development of a 'molecular' classification of myeloproliferative disorders with eosinophilia. The revised 2008 World Health Organization classification of myeloid neoplasms included a new category called “myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB or FGFR1.” Despite the molecular heterogeneity of PDGFR (platelet-derived growth factor receptor rearrangements, tyrosine kinase inhibitors at low dose induce rapid and complete hematological remission in the majority of these patients. Other kinase inhibitors are promising. Further discoveries of new molecular alterations will direct the development of new specific inhibitors. In this review, an update of the classifications of myeloproliferative disorders associated with hypereosinophilia is discussed together with open and controversial questions. Molecular mechanisms and promising results of tyrosine kinase inhibitor treatments are reviewed. Keywords: hypereosinophilia, classification, myeloproliferative disorders, molecular alterations, tyrosine kinase inhibitor

  13. Studies on 99Tcm-sulfur colloid bone marrow scintigraphy in myeloproliferative disorders

    International Nuclear Information System (INIS)

    Objective: To discuss the imaging features and changing patterns of bone marrow scintigraphy in myeloproliferative disorders (MPD) as well as its clinical significance. Methods: Bone marrow scintigraphy using 99Tcm-sulfur colloid 370-550 MBq was performed on 85 MPD patients, including 40 cases of idiopathic myelofibrosis (IMF), 15 of polycythemia vera (PV), 5 of essential thrombocythaemia (ET), 30 of chronic granulocytic leukemia. Also, 40 cases of myelodysplastic syndromes (MDS) were observed in this study. Results: Abnormal bone marrow imaging was found in 88.2% of the 85 patients. The suppression rate of central bone marrow (CBM) and expansion rate of peripheral bone marrow (PBM) in these MPD patients were 61.2% and 56.5%, respectively. The imaging patterns was classified into three types according to the distribution and activity of bone marrow. 1) reduced imaging (31.8%); 2) increased and expanded imaging (27.1%); 3) depressed and expanded imaging (29.4%). Splenomegaly with minimal residual marrow activity was typical for late stages of MPD. Expansion of PBM was the further feature, but of no major importance for improving hematopoiesis of MPD, and it tended to retract during clinical recovery in chronic granulocytic leukemia (CGL). With expanding PBM, unmatched peripheral blood decreasing was found in MDS. The expansion pattern of PBM in different MPD was of relatively definite features. Conclusions: The imaging pattern of bone marrow was correlated with blood work-up data and clinical course or stages of MPD. Bone marrow scintigraphy may be proven useful in differential diagnosis and evaluation of clinical staging and prognosis of MPD

  14. What is the Incidence of Kidney Stones after Chemotherapy in Patients with Lymphoproliferative or Myeloproliferative Disorders?

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    Hossein S. Mirheydar

    2014-12-01

    Full Text Available Introduction This study describes the incidence and risk factors of de novo nephrolithiasis among patients with lymphoproliferative or myeloproliferative diseases who have undergone chemotherapy. Materials and Methods From 2001 to 2011, patients with lymphoproliferative or myeloproliferative disorders treated with chemotherapy were retrospectively identified. The incidence of image proven nephrolithiasis after chemotherapy was determined. Demographic and clinical variables were recorded. Patients with a history of nephrolithiasis prior to chemotherapy were excluded. The primary outcome was incidence of nephrolithiasis, and secondary outcomes were risk factors predictive of de novo stone. Comparative statistics were used to compare demographic and disease specific variables for patients who developed de novo stones versus those who did not. Results A total of 1,316 patients were identified and the incidence of de novo nephrolithiasis was 5.5% (72/1316; symptomatic stones 1.8% 24/1316. Among patients with nephrolithiasis, 72.2% had lymphoproliferative disorders, 27.8% had myeloproliferative disorders, and 25% utilized allopurinol. The median urinary pH was 5.5, and the mean serum uric acid, calcium, potassium and phosphorus levels were 7.5, 9.6, 4.3, and 3.8 mg/dL, respectively. In univariate analysis, mean uric acid (p=0.013, calcium (p<0.001, and potassium (p=0.039 levels were higher in stone formers. Diabetes mellitus (p<0.001, hypertension (p=0.003, and hyperlipidemia (p<0.001 were more common in stone formers. In multivariate analysis, diabetes mellitus, hyperuricemia, and hypercalcemia predicted stone. Conclusions We report the incidence of de novo nephrolithiasis in patients who have undergone chemotherapy. Diabetes mellitus, hyperuricemia, and hypercalcemia are patient-specific risk factors that increase the odds of developing an upper tract stone following chemotherapy.

  15. Intracellular growth factors in polycythemia vera and other myeloproliferative disorders.

    OpenAIRE

    Eid, J.; Ebert, R F; Gesell, M S; Spivak, J L

    1987-01-01

    In polycythemia vera, idiopathic myelofibrosis, and essential thrombocytosis, hematopoietic cell proliferation is increased in the absence of a recognizable stimulus, suggesting the autonomous production of growth factors in these disorders. Sonicates of peripheral blood mononuclear cells (PBMNC) from patients with polycythemia vera, idiopathic myelofibrosis, and essential thrombocytosis contained soluble factors that stimulated the proliferation of quiescent-confluent 3T3 cells. PBMNC sonica...

  16. Chronic motor tic disorder

    Science.gov (United States)

    Chronic motor tic disorder is more common than Tourette syndrome . Chronic tics may be forms of Tourette syndrome. Tics usually start at age 5 or 6 and get worse until age 12. They often improve during adulthood.

  17. Neurological Findings in Myeloproliferative Neoplasms

    OpenAIRE

    Semra Paydas

    2013-01-01

    Myeloproliferative neoplasms (MPN) arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bon...

  18. Chronic lymphocytic leukemia and myeloproliferative neoplasms concurrently diagnosed: clinical and biological characteristics.

    Science.gov (United States)

    Todisco, Gabriele; Manshouri, Taghi; Verstovsek, Srdan; Masarova, Lucia; Pierce, Sherry A; Keating, Michael J; Estrov, Zeev

    2016-05-01

    Chronic lymphocytic leukemia (CLL) and myeloproliferative neoplasms (MPN) may occur concomitantly. However, little is known about the pathobiological characteristics and interaction between the neoplastic clones in these rare cases of coinciding malignancies. We retrospectively examined the clinical and biological characteristics of 13 patients with concomitant CLL and MPN--eight primary myelofibrosis (PMF), three essential thrombocytosis (ET), and two polycythemia vera (PV)--who presented to our institution between 1998 and 2014, and tested all patients for MPN-specific aberrations, such as JAK2, MPL and CALR mutations. Along with epidemiological and molecular characterization of this rare condition, we found that JAK2 mutation can be detected 9 years prior to PMF diagnosis, suggesting that PMF clinical phenotype may require several years to develop and CLL/MPN clinical co-occurrence might be sustained by common molecular events. Some features of these patients suggest that pathobiologies of these diseases might be intertwined. PMID:26402369

  19. A mutação JAK2 V617F e as síndromes mieloproliferativas JAK2 V617F mutation and the myeloproliferative disorders

    Directory of Open Access Journals (Sweden)

    Bárbara C. R. Monte-Mór

    2008-01-01

    Full Text Available Síndromes mieloproliferativas (SMPs são doenças hematopoéticas de origem clonal que apresentam amplificação de uma ou mais linhagens mielóides. Policitemia vera (PV, trombocitemia essencial (TE, mielofibrose idiopática (MF e leucemia mielóide crônica (LMC são consideradas SMPs clássicas e apresentam características clínicas e biológicas comuns. Ao contrário de LMC, cuja etiologia está relacionada à proteína constitutivamente ativa Bcr-Abl, o mecanismo molecular de PV, TE e MF permaneceu por muito tempo desconhecido. Esta revisão se foca na recente descoberta da mutação JAK2 V617F em pacientes com PV, TE e MF, sua relação com o fenótipo mieloproliferativo e implicações na abordagem clínica de pacientes.Myeloproliferative disorders are clonal hematopoietic diseases that are characterized by the amplification of one or more myeloid lineages. Polycythemia vera, essential thrombocythemia, idiopathic myelofibrosis and chronic myeloid leukemia are considered classic myeloproliferative disorders and share common clinical and biological features. While the genetic basis of chronic myeloid leukemia is shown to be the constitutive active protein BCR-ABL, the main molecular lesions in polycythemia vera, essential thrombocythemia and idiopathic myelofibrosis remain unknown. This review focuses on the recent discovery of the JAK2 V617F mutation, its relationship to the myeloproliferative phenotype and implications in the clinical approach of patients.

  20. Changing patterns of radiosensitivity of hematopoietic progenitors from chronically irradiated dogs prone either to aplastic anemia or to myeloproliferative disease

    International Nuclear Information System (INIS)

    Hematopoietic patterns have been assessed in chronic 60Co gamma irradiated dogs during preclinical phases of evolving aplastic anemia (AA) or myeloproliferative disease (MPD), principally myeloid leukemia. The results support the concept that acquired radioresistance of vital granulocyte/monocyte lineage-committed hematopoietic progenitors is temporally, perhaps causally, linked to the processes mediating hematopoietic recovery and accommodation under chronic irradiation, and in turn to preclinical events of evolving MPD. In addition, the marked differential responses of progenitors to gamma and neutron irradiation in vitro might suggest differences in the nature of cellular lesions elicited by chronic gamma irradiation, in vivo. (author)

  1. Clonality in myeloproliferative disorders: Analysis by means of polymerase chain reaction

    Energy Technology Data Exchange (ETDEWEB)

    Gilliland, D.G.; Blanchard, K.L.; Levy, J.; Perrin, S.; Bunn, H.F. (Harvard Medical School, Boston, MA (United States))

    1991-08-01

    The myeloproliferative syndromes are acquired disorders of hematopoiesis that provide insights into the transition from somatic cell mutation to neoplasia. The clonal origin of specific blood cells can be assessed in patients with X chromosome-linked polymorphisms, taking advantage of random inactivation of the X chromosome. The authors have adapted the PCR for determination of clonality on as few as 100 cells, including individual colonies grown in culture. Amplifying a polymorphic portion of the X chromosome-linked phosphoglycerate kinase (PGK) gene after selective digestion of the active X chromosome with a methylation-sensitive restriction enzyme gave results fully concordant with standard Southern blotting of DNA samples form normal (polyclonal) polymorphonuclear cells (PMN) as well as clonal PMN from patients with myelodysplastic syndrome and polycythemia vera (PCV). They have used this technique to demonstrate heterogeneity of lineage involvement in patients with PCV. The same clinical phenotype may arise from clonal proliferation of different hematopoietic progenitors.

  2. Mesenteric venous thrombosis secondary to an unsuspected JAK2 V617F-positive myeloproliferative disorder.

    LENUS (Irish Health Repository)

    2012-01-31

    BACKGROUND: Mesenteric venous thrombosis (MVT) is a rare but potentially fatal cause of mesenteric ischaemia. It presents insidiously and often diagnosis is made at emergency surgery. In half of the cases MVT develops without a causative factor, while in cases in which a pro-thrombotic state is found to exist MVT may be the first clinically detected consequence of that state. The myeloproliferative disorders (MPD) are known to contribute to the development of pro-thrombotic states. Recently, the JAK2 V617F mutation has been associated with the MPDs. CONCLUSION: We describe a case of MVT occurring secondary to an unsuspected MPD, in which the patient was subsequently found to carry this mutation. We highlight the necessity to screen for this mutation in cases of intra-abdominal thromboses so that appropriate systemic anticoagulation may be instituted, and the patient may be followed so as to detect the development of an overt MPD.

  3. Myeloproliferative Neoplasms (MPNs) Patient Registry

    Science.gov (United States)

    2016-04-28

    Primary Myelofibrosis; Polycythemia Vera; Essential Thrombocythemia; Mastocytosis; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative; Leukemia, Myelomonocytic, Juvenile; Chronic Eosinophilic Leukemia-not Otherwise Specified; Myelodysplastic-Myeloproliferative Diseases; Neoplasms; Leukemia, Myelomonocytic, Chronic

  4. The diagnosis of BCR/ABL-negative chronic myeloproliferative diseases (CMPD): a comprehensive approach based on morphology, cytogenetics, and molecular markers.

    Science.gov (United States)

    Haferlach, Torsten; Bacher, Ulrike; Kern, Wolfgang; Schnittger, Susanne; Haferlach, Claudia

    2008-01-01

    Recent years showed significant progress in the molecular characterization of the chronic myeloproliferative disorders (CMPD) which are classified according to the WHO classification of 2001 as polycythemia vera (PV), chronic idiopathic myelofibrosis (CIMF), essential thrombocythemia (ET), CMPD/unclassifiable (CMPD-U), chronic neutrophilic leukemia, and chronic eosinophilic leukemia (CEL)/hypereosinophilic syndrome, all to be delineated from BCR/ABL-positive chronic myeloid leukemia (CML). After 2001, the detection of the high frequency of the JAK2V617F mutation in PV, CIMF, and ET, and of the FIP1L1-PDGFRA fusion gene in CEL further added important information in the diagnosis of CMPD. These findings also enhanced the importance of tyrosine kinase mutations in CMPD and paved the way to a more detailed classification and to an improved definition of prognosis using also novel minimal residual disease (MRD) markers. Simultaneously, the broadening of therapeutic strategies in the CMPD, e.g., due to reduced intensity conditioning in allogeneic hematopoietic stem cell transplantation and the introduction of tyrosine kinase inhibitors in CML, in CEL, and in other ABL and PDGRFB rearrangements, increased the demands to diagnostics. Therefore, today, a multimodal diagnostic approach combining cytomorphology, cytogenetics, and individual molecular methods is needed in BCR/ABL-negative CMPD. A stringent diagnostic algorithm for characterization, choice of treatment, and monitoring of MRD will be proposed in this review. PMID:17938925

  5. Myeloproliferative disorders in patients with rheumatoid arthritis treated with total body irradiation

    International Nuclear Information System (INIS)

    Four patients with refractory rheumatoid arthritis were treated with total body irradiation administered in two sittings, 300 to 400 rads to each half of the body. All four patients had taken antimetabolites prior to receiving total body irradiation, and two continued to use them after total body irradiation. Two patients had taken alkylating agents before, and one had used them after total body irradiation. All patients showed clinical improvement. However, in two patients myeloproliferative disorders developed: a myelodysplastic preleukemia at 40 months after total body irradiation in one and acute myelogenous leukemia at 25 months in the other. Total body irradiation differs from total nodal irradiation in the total dose of irradiation (300 to 400 rads versus 2,000 to 3,000), and in the duration of the therapy (two sittings versus treatment over several weeks to months). Furthermore, the patients in the total body irradiation study frequently used cytotoxic drugs before and/or after irradiation, whereas in one total nodal irradiation study, azathioprine (2 mg/kg per day or less) was permitted, but no other cytotoxic agents were allowed. Rheumatologists may therefore face a binding decision when deciding to treat a patient with rheumatoid arthritis with either a cytotoxic drug or irradiation

  6. Pivotal role of mast cells in pruritogenesis in patients with myeloproliferative disorders.

    Science.gov (United States)

    Ishii, Takefumi; Wang, Jiapeng; Zhang, Wei; Mascarenhas, John; Hoffman, Ronald; Dai, Ying; Wisch, Nathaniel; Xu, Mingjiang

    2009-06-01

    Pruritus is a common symptom in patients with Philadelphia chromosome-negative myeloproliferative disorders (MPDs). The pathophysiology of MPD-associated pruritus is unclear. We have demonstrated that MPD mast cells (MCs) are involved by the malignant process. In the present study, we explored the hypothesis that MCs play an important role in the development of pruritogenesis in MPDs. We found that MPD MCs released significantly greater amounts of pruritogenic factors, including histamine, leukotrienes, and interleukin-31 (IL-31) than normal MCs. Elevated levels of IL-31 were also observed in MPD CD3(+) cell-conditioned media. MPD MCs exhibited increased migratory behavior in response to stem cell factor or interleukin-8, which was associated with increased filamentous-actin content. Furthermore, the presence of pruritus in MPDs was statistically correlated with a greater number of MCs being generated by CD34(+) cells, a greater number of MC colonies being formed by CD34(+) cells, decreased apoptosis and prostaglandin D(2) release by cultured MCs, and higher plasma levels of IL-31. These data demonstrate that functional abnormalities of MPD MCs probably lead to pruritogenesis in patients with MPDs. These studies provide cellular and molecular targets for the development of antipruritus drugs for patients with MPDs. PMID:19196660

  7. Myeloproliferative disorders in patients with rheumatoid arthritis treated with total body irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Urowitz, M.B.; Rider, W.D.

    1985-01-21

    Four patients with refractory rheumatoid arthritis were treated with total body irradiation administered in two sittings, 300 to 400 rads to each half of the body. All four patients had taken antimetabolites prior to receiving total body irradiation, and two continued to use them after total body irradiation. Two patients had taken alkylating agents before, and one had used them after total body irradiation. All patients showed clinical improvement. However, in two patients myeloproliferative disorders developed: a myelodysplastic preleukemia at 40 months after total body irradiation in one and acute myelogenous leukemia at 25 months in the other. Total body irradiation differs from total nodal irradiation in the total dose of irradiation (300 to 400 rads versus 2,000 to 3,000), and in the duration of the therapy (two sittings versus treatment over several weeks to months). Furthermore, the patients in the total body irradiation study frequently used cytotoxic drugs before and/or after irradiation, whereas in one total nodal irradiation study, azathioprine (2 mg/kg per day or less) was permitted, but no other cytotoxic agents were allowed. Rheumatologists may therefore face a binding decision when deciding to treat a patient with rheumatoid arthritis with either a cytotoxic drug or irradiation.

  8. Fludarabine Phosphate and Total Body Irradiation Followed by a Donor Peripheral Stem Cell Transplant in Treating Patients With Myelodysplastic Syndromes or Myeloproliferative Disorders

    Science.gov (United States)

    2016-05-19

    Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Essential Thrombocythemia; Myeloproliferative Neoplasm; Paroxysmal Nocturnal Hemoglobinuria; Polycythemia Vera; Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase; Primary Myelofibrosis; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Ring Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Refractory Cytopenia With Multilineage Dysplasia and Ring Sideroblasts

  9. Analyses of critical target cell responses during preclinical phases of evolving chronic radiation-induced myeloproliferative disease-exploitation of a unique canine model

    Energy Technology Data Exchange (ETDEWEB)

    Seed, T.M.; Kaspar, L.V.; Tolle, D.V.; Fritz, T.E.; Frazier, M.E.

    1988-01-01

    This document briefly summarizes and highlights ongoing studies on the cellular and molecular processes involved in the induction and progression of myeloid leukemia in dogs chronically exposed to low daily doses of wholebody gamma irradiation. Under such conditions, select groups of dogs exhibit extremely high frequencies of myeloproliferative disease (MPD) (i.e., /congruent/50%) of which myeloid leukemia is most prominent. 2 figs.

  10. Analyses of critical target cell responses during preclinical phases of evolving chronic radiation-induced myeloproliferative disease-exploitation of a unique canine model

    International Nuclear Information System (INIS)

    This document briefly summarizes and highlights ongoing studies on the cellular and molecular processes involved in the induction and progression of myeloid leukemia in dogs chronically exposed to low daily doses of wholebody gamma irradiation. Under such conditions, select groups of dogs exhibit extremely high frequencies of myeloproliferative disease (MPD) (i.e., /congruent/50%) of which myeloid leukemia is most prominent. 2 figs

  11. Detection and clinical significance of JAK2 V617F mutation in Chinese and Uyghur patients with chronic myeloproliferative in Xinjiang

    Institute of Scientific and Technical Information of China (English)

    张晓燕

    2013-01-01

    Objective To investigate the frequency of JAK2 V617F gene(hereinafter,the JAK2 gene) mutation in Uyghur patients with chronic myeloproliferative neoplasm(MPN) and its relationship with the clinical characteristics,and further compare differences of mutation rates in

  12. [Essential thrombocythaemia and other myeloproliferative disorders with thrombocythaemia treated with Thromboreductin. A report from the database of register for the 1st quarter of 2010].

    Science.gov (United States)

    Penka, M; Schwarz, J; Ovesná, P; Hlusí, A; Korístek, Z; Doubek, M; Dulícek, P; Pospísilová, D; Kissová, J; Buliková, A; Pavlík, T

    2010-06-01

    In the Czech Republic, anagrelid is used according to the recommendations of the Czech Working Group on Myeloproliferative Disorders for treatment ofthrombocythaemias associated with chronic myeloproliferative disorders--mainly essential thrombocythaemia and, regularly, reports are being presented from the Register of Patients Treated with Thromboreductin, most recently last year (Vnitr Lék 2009; 55: I-XII). The Register commenced in 2005 and from then it aims to determine detailed clinical and laboratory profiles of the patients. The structure of the Register has changed significantly in the course of its existence, reflecting the reports from each of the analyses conducted so far. Also, the data entry in the database improves every year and it reaches 97% on some of the items. The longest evaluation period in some of the patients is 108 months. By April 2010, the Register database contained data on 717 patients. Of these, 672 patients with the diagnosis of a Ph-negative chronic myeloproliferative disorder were evaluated. This year's analysis included the patients with essential thrombocythaemia, polycythaemia vera and primary myelofibrosis only. The analysis included 418 women and 254 men with median age of50 years. Unlike the first years, 2/3 of the current sample are non pretreated patients, meaning that the patients reach the specialized centres early in their treatment. Also, patients, and the older patients in particular, are more frequently treated with combined regimens including Thromboreductin. We increasingly observe hypertension as one of the monitored risk factors preceding the disease and laboratory parameters showJAK2 mutation in more than a half of patients while some form ofthrombotic diathesis is found in the anamnesis of 7-10% of patients. Some bleeding is observed in 1-5% of the registered patients. In comparison to the previous years, this is a decrease in the prevalence of clinical symptoms prior to the disease onset; this is very likely

  13. The Hepatocyte Growth Factor (HGF)/Met Axis: A Neglected Target in the Treatment of Chronic Myeloproliferative Neoplasms?

    International Nuclear Information System (INIS)

    Met is the receptor of hepatocyte growth factor (HGF), a cytoprotective cytokine. Disturbing the equilibrium between Met and its ligand may lead to inappropriate cell survival, accumulation of genetic abnormalities and eventually, malignancy. Abnormal activation of the HGF/Met axis is established in solid tumours and in chronic haematological malignancies, including myeloma, acute myeloid leukaemia, chronic myelogenous leukaemia (CML), and myeloproliferative neoplasms (MPNs). The molecular mechanisms potentially responsible for the abnormal activation of HGF/Met pathways are described and discussed. Importantly, inCML and in MPNs, the production of HGF is independent of Bcr-Abl and JAK2V617F, the main molecular markers of these diseases. In vitro studies showed that blocking HGF/Met function with neutralizing antibodies or Met inhibitors significantly impairs the growth of JAK2V617F-mutated cells. With personalised medicine and curative treatment in view, blocking activation of HGF/Met could be a useful addition in the treatment of CML and MPNs for those patients with high HGF/MET expression not controlled by current treatments (Bcr-Abl inhibitors in CML; phlebotomy, hydroxurea, JAK inhibitors in MPNs)

  14. The Hepatocyte Growth Factor (HGF/Met Axis: A Neglected Target in the Treatment of Chronic Myeloproliferative Neoplasms?

    Directory of Open Access Journals (Sweden)

    Marjorie Boissinot

    2014-08-01

    Full Text Available Met is the receptor of hepatocyte growth factor (HGF, a cytoprotective cytokine. Disturbing the equilibrium between Met and its ligand may lead to inappropriate cell survival, accumulation of genetic abnormalities and eventually, malignancy. Abnormal activation of the HGF/Met axis is established in solid tumours and in chronic haematological malignancies, including myeloma, acute myeloid leukaemia, chronic myelogenous leukaemia (CML, and myeloproliferative neoplasms (MPNs. The molecular mechanisms potentially responsible for the abnormal activation of HGF/Met pathways are described and discussed. Importantly, inCML and in MPNs, the production of HGF is independent of Bcr-Abl and JAK2V617F, the main molecular markers of these diseases. In vitro studies showed that blocking HGF/Met function with neutralizing antibodies or Met inhibitors significantly impairs the growth of JAK2V617F-mutated cells. With personalised medicine and curative treatment in view, blocking activation of HGF/Met could be a useful addition in the treatment of CML and MPNs for those patients with high HGF/MET expression not controlled by current treatments (Bcr-Abl inhibitors in CML; phlebotomy, hydroxurea, JAK inhibitors in MPNs.

  15. The Hepatocyte Growth Factor (HGF)/Met Axis: A Neglected Target in the Treatment of Chronic Myeloproliferative Neoplasms?

    Energy Technology Data Exchange (ETDEWEB)

    Boissinot, Marjorie [Translational Neuro-Oncology Group, Leeds Institute of Cancer and Pathology, University of Leeds, Level 5 Wellcome Trust Brenner Building, St James’s Hospital, Leeds LS9 7TF (United Kingdom); Vilaine, Mathias [Institute of Research on Cancer and Aging (IRCAN), CNRS-Inserm-UNS UMR 7284, U 1081, Centre A. Lacassagne, 33 Avenue Valombrose, Nice 06189 (France); Hermouet, Sylvie, E-mail: sylvie.hermouet@univ-nantes.fr [Centre Hospitalier Universitaire (CHU), Place Alexis Ricordeau, Nantes 44093 (France); Inserm UMR892, Centre de Recherche en Cancérologie Nantes-Angers, Institut de Recherche en Santé, Université de Nantes, 8 quai Moncousu, Nantes cedex 44007 (France)

    2014-08-12

    Met is the receptor of hepatocyte growth factor (HGF), a cytoprotective cytokine. Disturbing the equilibrium between Met and its ligand may lead to inappropriate cell survival, accumulation of genetic abnormalities and eventually, malignancy. Abnormal activation of the HGF/Met axis is established in solid tumours and in chronic haematological malignancies, including myeloma, acute myeloid leukaemia, chronic myelogenous leukaemia (CML), and myeloproliferative neoplasms (MPNs). The molecular mechanisms potentially responsible for the abnormal activation of HGF/Met pathways are described and discussed. Importantly, inCML and in MPNs, the production of HGF is independent of Bcr-Abl and JAK2V617F, the main molecular markers of these diseases. In vitro studies showed that blocking HGF/Met function with neutralizing antibodies or Met inhibitors significantly impairs the growth of JAK2V617F-mutated cells. With personalised medicine and curative treatment in view, blocking activation of HGF/Met could be a useful addition in the treatment of CML and MPNs for those patients with high HGF/MET expression not controlled by current treatments (Bcr-Abl inhibitors in CML; phlebotomy, hydroxurea, JAK inhibitors in MPNs)

  16. Myeloproliferative disease in children: a demographic study.

    OpenAIRE

    Reid, M. M.; Saunders, P. W.; Kernahan, J

    1988-01-01

    Over eight years, eight cases of childhood myeloproliferative disease were recognised in the northern region of England (population 3.1 million). Five were classic chronic myeloid leukaemia (CML) and the three others, forms of myeloproliferative disease. No case of juvenile CML was recognised. With the exception of CML, "adult" type myeloproliferative disease of children is underrepresented in the literature and its natural history remains unknown.

  17. ROLE OF SERUM EOSINOPHILIC CATIONIC PROTEIN AND TRYPTASE IN MYELOPROLIFERATIVE AND LYMPHOPROLIFERATIVE DISORDERS

    Directory of Open Access Journals (Sweden)

    L. S. Komarova

    2008-01-01

    Full Text Available Abstract. A role of intracellular proteins of eosinophils and mast cells remains unclear in the patients with hematological neoplasia. There is a substantial evidence that eosinophils possess some common mechanisms of cooperation with mast cells. Therapeutic interventions into key events controlling eosinophil migration may be a leading factor in treatment of hypereosinophylic states in onco-hematological disorders. Due to unknown functions of eosinophils in majority of eosinophilia-associated diseases, it would be useful to establish an algorithm of accurate diagnostics in the patients with eosinophilia, in order to choose more effective treatment in future.We studied serum levels of secretable eosinophil and mast cells proteins in oncohematological patients with increased eosinophil counts. The aim of our study was to test a significance of quantitative assay for tryptase and ECP in the patients with myelo- and lymphoproliferative diseases. The study group included thirty-eight patients with oncohematological diseases, accompanied by a marked eosinophilia (> 0.4 x 109/L. Eighteen patients with bronchial asthma (BA, and eight cases of solid tumors comprised a reference group for polyclonal eosinophilia. The levels of ECP and tryptase were measured in blood serum using a commercial fluoroimmunoenzyme assay («Pharmacia», Uppsala, Sweden. Total ECP levels were markedly increased in general group with hematological malignancies (p < 0.03, , and in cases of chronic GvHD (p < 0.03, and in a sub-group with lymphoproliferative disorders (р = 0.007 as compared to the group of non-hematological diseases.Serum levels of tryptase were significantly increased in the patients with chronic GvHD after allo-HSCT and lymphoproliferative diseases, as compared to the group of patients with solid tumors (р = 0.03, as well in GvHD compared with lymphoproliferative disorders (р < 0.05.A direct correlation was found between serum ECP levels and absolute

  18. Molecular genetics of chronic neutrophilic leukemia, chronic myelomonocytic leukemia and atypical chronic myeloid leukemia

    OpenAIRE

    Li, Bing; Gale, Robert Peter; Xiao, Zhijian

    2014-01-01

    According to the 2008 World Health Organization classification, chronic neutrophilic leukemia, chronic myelomonocytic leukemia and atypical chronic myeloid leukemia are rare diseases. The remarkable progress in our understanding of the molecular genetics of myeloproliferative neoplasms and myelodysplastic/myeloproliferative neoplasms has made it clear that there are some specific genetic abnormalities in these 3 rare diseases. At the same time, there is considerable overlap among these disord...

  19. Unbiased pro-thrombotic features at diagnosis in 977 thrombocythemic patients with Philadelphia-negative chronic myeloproliferative neoplasms.

    Science.gov (United States)

    Gugliotta, Luigi; Iurlo, Alessandra; Gugliotta, Gabriele; Tieghi, Alessia; Specchia, Giorgina; Gaidano, Gianluca; Scalzulli, Potito R; Rumi, Elisa; Dragani, Alfredo; Martinelli, Vincenzo; Santoro, Cristina; Randi, Maria Luigia; Tagariello, Giuseppe; Candoni, Anna; Cattaneo, Daniele; Ricco, Alessandra; Palmieri, Raffaele; Liberati, Marina A; Langella, Maria; Rago, Angela; Bergamaschi, Micaela; Monari, Paola; Miglio, Rossella; Santoro, Umberto; Cacciola, Rossella; Rupoli, Serena; Mastrullo, Lucia; Musto, Pellegrino; Mazzucconi, Maria Gabriella; Vignetti, Marco; Cortelezzi, Agostino; Vianelli, Nicola; Martino, Bruno; De Stefano, Valerio; Passamonti, Francesco; Vannucchi, Alessandro M

    2016-07-01

    In patients with Philadelphia-negative chronic myeloproliferative neoplasms (MPNs), the anti-thrombotic and/or cytoreductive treatment in the follow-up may affect the evaluation of the pro-thrombotic weight of the clinical and biological characteristics at diagnosis. In order to avoid this potential confounding effect, we investigated the relationship between prior thrombosis (PrTh: thrombosis occurred before diagnosis and before treatment) and the characteristics at diagnosis in 977 thrombocythemic patients with MPN, reclassified according to the WHO 2008 criteria. PrTh occurred in 194 (19.9%) patients, with similar rates in the different MPNs. In multivariate analysis, PrTh rate was significantly related to minor thrombocytosis (platelets ≤700×10(9)/L), leukocytosis (leukocytes >10×10(9)/L), higher hematocrit (HCT >45%), JAK2 V617F mutation, older age, and cardiovascular risk factors (CVRFs). The highest PrTh rate (33.9%) was associated with the coexistence of minor thrombocytosis and leukocytosis. Of note, the inverse relationship between PrTh rate and platelet count is consistent with the hemostatic paradox of thrombocytosis. In conclusion, this analysis in MPN patients disclosed the unbiased characteristics at diagnosis with a pro-thrombotic effect. Moreover, it suggests that the optimal control of blood cells counts, and CVRFs might be of utmost importance in the prevention of thrombosis during the follow-up. PMID:27107744

  20. Determinants of resistance to radiation injury in blood granulocytes from normal donors and from patients with myeloproliferative disorders

    International Nuclear Information System (INIS)

    Dose-dependent injury to human blood granulocytes was measured within 2 hr of x irradiation, as changes in net 22Na influx and phagocytosing O2 consumption. Among samples from normal donors and patients with chronic myeloproliferative diseases, samples from 10 to 12 patients with chronic granulocytic leukemia exhibited increased sensitivity to injury by radiation. Selected granulocyte constituents which may contribute to inactivation of oxidant and free-radical products of radiation-activated H2O were also measured. These included glutathione and ascorbate contents; superoxide dismutase, catalase, glutathione proxidase, and glutathione reductase activities; and capacity to take up and reduce dehydroascorbate. Catalase activity was irregularly higher in radiation-sensitive than in radiation-resistant granulocytes (P = < 0.05). Dehydroascorbate uptake and reduction was consistently low in radiation-sensitive cells (P = < 0.001). We propose that cell capacity to maintain ascorbate in reduced form against oxidant and free-radical stress is a part of mechanisms which determine resistance to injury by ionizing radiation in human granulocytes

  1. Presentation of Acute Megakaryoblastic Leukemia Associated with a GATA-1 Mutation Mimicking the Eruption of Transient Myeloproliferative Disorder.

    Science.gov (United States)

    Boos, Markus D; Wine Lee, Lara; Freedman, Jason L; Novoa, Roberto A; Chu, Emily Y; Perman, Marissa J

    2015-01-01

    Children with trisomy 21 are prone to developing hematologic disorders, including transient myeloproliferative disorder (TMD) and acute megakaryoblastic leukemia (AMKL). The papulovesicular eruption of TMD provides an important clue to the diagnosis. In contrast, AMKL rarely has associated cutaneous findings. We report the case of a 22-month-old child with trisomy 21 who presented with the acute onset of diffusely scattered and crusted papules, plaques, and vesicles. A thorough infectious evaluation was negative and the patient was unresponsive to empiric antibiotic and antiinflammatory therapies. Complete blood count (CBC) was notable for mild pancytopenia, with a normal peripheral smear. Two weeks later he was reassessed and found to have a population of blasts on repeat CBC. Subsequent evaluation ultimately led to a diagnosis of AMKL. This is the first reported case of a cutaneous eruption in a young child with Down syndrome and transformed AMKL. When children with trisomy 21 present with the acute onset of crusted papules and vesicles that cannot be accounted for by an infectious etiology, a diagnosis of AMKL should be considered even in the absence of a history of TMD. PMID:26205501

  2. Nye molekylaere markører ved de kroniske myeloproliferative sygdomme

    DEFF Research Database (Denmark)

    Larsen, Thomas Stauffer; Pallisgaard, Niels; Christensen, Jacob Haaber;

    2006-01-01

    The Philadelphia-negative chronic myeloproliferative disorders feature autonomous myeloid hyperproliferation and hypersensitivity to a number of growth factors, which most recently have been shown to be explained by a guanine-to-thymidine mutation in the Janus tyrosine kinase (JAK2) gene, implica...

  3. The splenomegaly of myeloproliferative and lymphoproliferative disorders: splenic cellularity and vascularity

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, B (Capital Hospital, Peking University Medical College, Beijing (China)); Lewis, S.M. (Department of Haematology, Royal Postgraduate Medical School, London (UK))

    1989-01-01

    Employing radionuclide scanning, the volume of the spleen, its red cell pool and plasma pool have been measured in vivo, and the relative proportions of cellularity and vascularity of the spleen have been calcualted in 51 patients with myeloproliferactive and lymphoproliferative disorders. In primary proliferative polycythaemia (polycythaemia vera), the increase of spleen size was attributed mainly to the increase of splenic vascularity; in myelofibrosis and in hairy cell leukaemia, the increase of spleen size was associated with increase in both splenic vascularity and cellularity, whilst in size was associated with increase in both splenic vascularity and cellularity, whilst in CGL and CLL the increase was attributed more to cellularity than to vascularity. (author).

  4. Ondansetron in Treating Patients With Advanced Cancer and Chronic Nausea and Vomiting Not Caused by Cancer Treatment

    Science.gov (United States)

    2016-07-01

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Nausea and Vomiting; Precancerous Condition; Small Intestine Cancer; Unspecified Adult Solid Tumor, Protocol Specific; Unspecified Childhood Solid Tumor, Protocol Specific

  5. Open Label, Phase II Study to Evaluate Efficacy and Safety of Oral Nilotinib in Philadelphia Positive (Ph+) Chronic Myelogenous Leukemia (CML) Pediatric Patients.

    Science.gov (United States)

    2016-08-05

    Leukemia; Leukemia,Pediatric; Leukemia, Myleiod; Leukemia, Mylegenous, Chronic; Leukemia, Mylegenous, Accelerated; BCR-ABL Positive; Myeloproliferative Disorder; Bone Marrow Disease; Hematologic Diseases; Neoplastic Processes; Imatinib; Dasatinib; Enzyme Inhibitor; Protein Kinase Inhibitor

  6. sEPCR Levels in Chronic Myeloproliferative Diseases and Their Association with Thromboembolic Events: A Case-Control Study

    Directory of Open Access Journals (Sweden)

    Figen Atalay

    2014-06-01

    Full Text Available OBJECTIVE: Venous, arterial, and microcirculatory events are frequently encountered in the clinical course of essential thrombocytosis and polycythemia vera. We aimed to investigate the levels of soluble endothelial protein C receptor (sEPCR in myeloproliferative diseases to see whether there was a difference between the patients with and without history of thromboembolism. METHODS: The study included patients with polycythemia vera (n=12, patients with essential thrombocytosis (n=13, and controls (n=29. In all groups, we measured proteins C and S, antithrombin and sEPCR levels, and plasma concentrations of thrombin-antithrombin complex, prothrombin fragments 1+2, and D-dimer. RESULTS: Comparing the patients with and without history of thromboembolic attack, statistically significant differences were not observed in terms of sEPCR, D-dimer, thrombin-antithrombin complex, prothrombin fragments 1+2, and hematocrit levels (p=0.318, 0.722, 0.743, 0.324, and 0.065, respectively. CONCLUSION: Significant increase in the parameters that reflect activation of coagulation, such as sEPCR, thrombinantithrombin complex, prothrombin fragments 1+2, and D-dimer, reflects the presence of a basal condition that leads to a tendency toward thrombosis development in ET and PV when compared to healthy controls.

  7. Bone Marrow and Kidney Transplant for Patients With Chronic Kidney Disease and Blood Disorders

    Science.gov (United States)

    2016-01-28

    Chronic Kidney Disease; Acute Myeloid Leukemia (AML); Acute Lymphoblastic Leukemia (ALL); Chronic Myelogenous Leukemia (CML); Chronic Lymphocytic Leukemia (CLL); Non-Hodgkin's Lymphoma (NHL); Hodgkin Disease; Multiple Myeloma; Myelodysplastic Syndrome (MDS); Aplastic Anemia; AL Amyloidosis; Diamond Blackfan Anemia; Myelofibrosis; Myeloproliferative Disease; Sickle Cell Anemia; Autoimmune Diseases; Thalassemia

  8. Neurological Findings in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Semra Paydas

    2013-04-01

    Full Text Available Myeloproliferative neoplasms (MPN arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bone marrow named as extramedullary myeloid tumors (EMMT could be detected at the initial diagnosis or during the prognosis of the disease, which may cause neurological symptoms due to pressure of leukemic cell mass on various tissues along with spinal cord. Central nervous system involvement and thrombocytopenic hemorrhage may lead to diverse neurological symptoms and findings.Transient ischemic attack and thrombotic stroke are the most common symptoms in polycythemia vera. Besides thrombosis and hemorrage, transformation to acute leukemia can cause neurological symptoms and findings. Transient ischemic attack, thrombotic stroke and specifically hemorrage can give rise to neurological symptoms similar to MPN in essential thrombocytosis.Extramedullary hematopoiesis refers to hematopoietic centers arise in organ/tissues other than bone marrow in myelofibrosis. Extramedullar hematopoietic centers may cause intracranial involvement, spinal cord compression, seizures and hydrocephalia. Though rare, extramedullary hematopoiesis can be detected in cranial/spinal meninges, paraspinal tissue and intracerebral regions. Extramedullary hematopoiesis has been reported in peripheral neurons, choroid plexus, pituitary, orbits, orbital and lacrimal fossa and in sphenoidal sinuses. [Cukurova Med J 2013; 38(2.000: 157-169

  9. Sleep disorders and chronic kidney disease

    Science.gov (United States)

    Maung, Stephanie C; El Sara, Ammar; Chapman, Cherylle; Cohen, Danielle; Cukor, Daniel

    2016-01-01

    Sleep disorders have a profound and well-documented impact on overall health and quality of life in the general population. In patients with chronic disease, sleep disorders are more prevalent, with an additional morbidity and mortality burden. The complex and dynamic relationship between sleep disorders and chronic kidney disease (CKD) remain relatively little investigated. This article presents an overview of sleep disorders in patients with CKD, with emphasis on relevant pathophysiologic underpinnings and clinical presentations. Evidence-based interventions will be discussed, in the context of individual sleep disorders, namely sleep apnea, insomnia, restless leg syndrome and excessive daytime sleepiness. Limitations of the current knowledge as well as future research directions will be highlighted, with a final discussion of different conceptual frameworks of the relationship between sleep disorders and CKD. PMID:27152260

  10. Chronic diseases and mental disorder.

    NARCIS (Netherlands)

    Verhaak, P.F.M.; Heijmans, M.J.W.M.; Peters, L.; Rijken, M.

    2005-01-01

    The aim of this study was to achieve a better understanding of the relationship between chronic medical illness and mental distress. Therefore, the association between chronic medical illness and mental distress was analysed, taking into account the modifying effects of generic disease characteristi

  11. Chronic diseases and mental disorder.

    OpenAIRE

    Verhaak, P.F.M.; Heijmans, M.J.W.M.; L. Peters; Rijken, M.

    2005-01-01

    The aim of this study was to achieve a better understanding of the relationship between chronic medical illness and mental distress. Therefore, the association between chronic medical illness and mental distress was analysed, taking into account the modifying effects of generic disease characteristics (concerning course, control and possible stressful consequences), physical quality of life indicators and social and relationship problems. Panel data from the Dutch national Panel of Patients w...

  12. Chronic pain: Model of psychosomatic disorder (review

    Directory of Open Access Journals (Sweden)

    Chernus N.P.

    2011-12-01

    Full Text Available The article presents a detailed review on epidemiology, pathogenesis and interrelation of serotonin neuromedia-tor metabolism in the central nervous system in state of chronic pain and depression. It has been demonstrated that neurophysiological conditions serve as psychological defense of an individual. That mechanism has been proved to «transform» serious emotions onto the inner level (body and it assists in the development of psychosomatic disorderschronic pain syndrome

  13. Acute progression of BCR-FGFR1 induced murine B-lympho/myeloproliferative disorder suggests involvement of lineages at the pro-B cell stage.

    Directory of Open Access Journals (Sweden)

    Mingqiang Ren

    Full Text Available Constitutive activation of FGFR1, through rearrangement with various dimerization domains, leads to atypical myeloproliferative disorders where, although T cell lymphoma are common, the BCR-FGFR1 chimeric kinase results in CML-like leukemia. As with the human disease, mouse bone marrow transduction/transplantation with BCR-FGFR1 leads to CML-like myeloproliferation as well as B-cell leukemia/lymphoma. The murine disease described in this report is virtually identical to the human disease in that both showed bi-lineage involvement of myeloid and B-cells, splenomegaly, leukocytosis and bone marrow hypercellularity. A CD19(+ IgM(- CD43(+ immunophenotype was seen both in primary tumors and two cell lines derived from these tumors. In all primary tumors, subpopulations of these CD19(+ IgM(- CD43(+ were also either B220(+ or B220(-, suggesting a block in differentiation at the pro-B cell stage. The B220(- phenotype was retained in one of the cell lines while the other was B220(+. When the two cell lines were transplanted into syngeneic mice, all animals developed the same B-lymphoblastic leukemia within 2-weeks. Thus, the murine model described here closely mimics the human disease with bilineage myeloid and B-cell leukemia/lymphoma which provides a representative model to investigate therapeutic intervention and a better understanding of the etiology of the disease.

  14. Myeloproliferative neoplasms working group consensus recommendations for diagnosis and management of primary myelofibrosis, polycythemia vera, and essential thrombocythemia

    Directory of Open Access Journals (Sweden)

    M B Agarwal

    2015-01-01

    Full Text Available According to the 2008 revision of the World Health Organization (WHO classification of myeloid malignancies, philadelphia chromosome (Ph-negative myeloproliferative neoplasms (MPNs include clonal, hematologic disorders such as polycythemia vera, primary myelofibrosis, and essential thrombocythemia. Recent years have witnessed major advances in the understanding of the molecular pathophysiology of these rare subgroups of chronic, myeloproliferative disorders. Identification of somatic mutations in genes associated with pathogenesis and evolution of these myeloproliferative conditions (Janus Kinase 2; myeloproliferative leukemia virus gene; calreticulin led to substantial changes in the international guidelines for diagnosis and treatment of Ph-negative MPN during the last few years. The MPN-Working Group (MPN-WG, a panel of hematologists with expertise in MPN diagnosis and treatment from various parts of India, examined applicability of this latest clinical and scientific evidence in the context of hematology practice in India.This manuscript summarizes the consensus recommendations formulated by the MPN-WG that can be followed as a guideline for management of patients with Ph-negative MPN in the context of clinical practice in India.

  15. Psychiatric disorders in chronic periodic haemodialysis

    Directory of Open Access Journals (Sweden)

    Paraskevi Theofilou

    2010-10-01

    Full Text Available The progress in Medical and Nursing Science has contributed significantly to the lengthening of life expectancy regarding several categories of ill people with chronic diseases. However, when the quality of life depends on the periodic correction of biological parameters, as with people with chronic renal failure, this situation affects both the patient and the environment. The aim of the present study is the evaluation of psychiatric disorders which are presented in haemodialysis patients as well as the influence of these disorders on their quality of life. Material and method: Review of relative bibliography was made in electronic basis of Medline (1980‐2009 using as key words haemodialysis, chronic renal failure, quality of life, psychiatric disorders. Complementary bibliography was found through other electronic search engines. Results: The chronic character and the frequency of renal failure, the possible dysfunction in the movement as well as the necessary long treatment cause problems, which extend the disease beyond the medical area offering socioeconomic dimensions, which complicate the associated psychiatric disorders. Conclusions: These patients suffer from the disease as well as from the treatment and at the same time they are faced with the number of the accompanying and interrelated problems, which come up in their everyday living and prescribe restrictively their way of life.

  16. Alexithymia in Chronic Pain Disorders.

    Science.gov (United States)

    Di Tella, Marialaura; Castelli, Lorys

    2016-07-01

    This review proposes a critical discussion of the recent studies investigating the presence of alexithymia in patients suffering from different chronic pain (CP) conditions. The term CP refers to pain that persists or progresses over time, while alexithymia is an affective dysregulation, largely observed in psychosomatic diseases. Overall, the examined studies showed a high prevalence of alexithymia, especially difficulties in identifying feelings, in all the different CP conditions considered. However, the association between alexithymia and pain intensity was not always clear and in some studies this relationship appeared to be mediated by negative effect, especially depression. The role of alexithymia in CP should be clarified by future studies, paying particular attention to two aspects: the use of additional measures, in addition to the Toronto Alexithymia Scale, to assess alexithymia, and the analysis of the potential differences in the evolution of different CP conditions with reference to the presence or absence of alexithymia. PMID:27215759

  17. Chronic complex dissociative disorders and borderline personality disorder: disorders of emotion dysregulation?

    Science.gov (United States)

    Brand, Bethany L; Lanius, Ruth A

    2014-01-01

    Emotion dysregulation is a core feature of chronic complex dissociative disorders (DD), as it is for borderline personality disorder (BPD). Chronic complex DD include dissociative identity disorder (DID) and the most common form of dissociative disorder not otherwise specified (DDNOS, type 1), now known as Other Specified Dissociative Disorders (OSDD, type 1). BPD is a common comorbid disorder with DD, although preliminary research indicates the disorders have some distinguishing features as well as considerable overlap. This article focuses on the epidemiology, clinical presentation, psychological profile, treatment, and neurobiology of chronic complex DD with emphasis placed on the role of emotion dysregulation in each of these areas. Trauma experts conceptualize borderline symptoms as often being trauma based, as are chronic complex DD. We review the preliminary research that compares DD to BPD in the hopes that this will stimulate additional comparative research. PMID:26401297

  18. Chronic Traumatic Encephalopathy and Movement Disorders: Update.

    Science.gov (United States)

    Tarazi, Apameh; Tator, Charles H; Tartaglia, Maria Carmela

    2016-05-01

    Association of repetitive brain trauma with progressive neurological deterioration has been described since the 1920s. Punch drunk syndrome and dementia pugilistica (DP) were introduced first to explain symptoms in boxers, and more recently, chronic traumatic encephalopathy (CTE) has been used to describe a neurodegenerative disease in athletes and military personal with a history of multiple concussions. Although there are many similarities between DP and CTE, a number of key differences are apparent especially when comparing movement impairments. The aim of this review is to compare clinical and pathological aspects of DP and CTE with a focus on disorders of movement. PMID:27021775

  19. Vestibular testing in patients with panic disorder and chronic dizziness

    OpenAIRE

    TEGGI, R.; D. Caldirola; BONDI, S.; Perna, G.; L. Bellodi; BUSSI, M.

    2007-01-01

    In order to investigate the relationship between chronic dizziness and vestibular function in patients with panic disorder, in the present study neurotologic findings in 15 patients with panic disorder and chronic dizziness were compared with those in 15 patients with chronic dizziness, without panic disorder. All underwent neurotologic screening for spontaneous, positional and positioning nystagmus with head-shaking and head-thrust tests, an audiometric examination and electronystagmography ...

  20. MPL mutations in myeloproliferative disorders

    DEFF Research Database (Denmark)

    Beer, Philip A.; Campbell, Peter J.; Scott, Linda M.;

    2008-01-01

    Activating mutations of MPL exon 10 have been described in a minority of patients with idiopathic myelofibrosis (IMF) or essential thrombocythemia (ET), but their prevalence and clinical significance are unclear. Here we demonstrate that MPL mutations outside exon 10 are uncommon in platelet c......DNA and identify 4 different exon 10 mutations in granulocyte DNA from a retrospective cohort of 200 patients with ET or IMF. Allele-specific polymerase chain reaction was then used to genotype 776 samples from patients with ET entered into the PT-1 studies. MPL mutations were identified in 8.5% of JAK2 V617F......(-) patients and a single V617F(+) patient. Patients carrying the W515K allele had a significantly higher allele burden than did those with the W515L allele, suggesting a functional difference between the 2 variants. Compared with V617F(+) ET patients, those with MPL mutations displayed lower hemoglobin...

  1. Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia

    Directory of Open Access Journals (Sweden)

    Stephanie Zettner

    2014-11-01

    Full Text Available Chronic myelogenous leukemia (CML is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospitalization. We suggest that management of gross hematuria in clinically stable patients with CML, suspected of having extramedullary hematopoiesis, should prioritize treatment of the myeloproliferative disorder over efforts to control bleeding.

  2. Skin prick test in patients with chronic allergic skin disorders

    OpenAIRE

    Pooja Bains; Alka Dogra

    2015-01-01

    Background: Chronic allergic skin disorders are the inflammatory and proliferative conditions in which both genetic and environmental factors play important roles. Chronic idiopathic urticaria (CIU) and atopic dermatitis (AD) are among the most common chronic allergic skin disorders. These can be provoked by various food and aeroallergens. Skin prick tests (SPTs) represent the cheapest and most effective method to diagnose type I hypersensitivity. Positive skin tests with a history suggestive...

  3. Características clínicas y paraclínicas de las neoplasias mieloproliferativas crónicas cromosoma Filadelfia negativas Clinical and paraclinical characteristics of chronic myeloproliferative neoplasms Philadelphia chromosome negative

    Directory of Open Access Journals (Sweden)

    Julio César Solano

    2012-04-01

    , PDGFRB y FGFR1 ya que son herramientas de reciente ingreso al arsenal diagnóstico y cuyo impacto como factor pronóstico o terapéutico se encuentra en estudio. Las complicaciones más frecuentemente encontradas en esta serie son los eventos trombóticos venosos. (Acta Med Colomb 2012; 37: 66-73Objective: describe features clinics and patients with chronic myeloproliferative neoplasm chromosome Philadelphia negative valued at the Hospital San Jose hematology outpatient from January 2005 until May 2010. Materials and methods: Studio series case included patients diagnosed with chronic myeloproliferative neoplasm chromosome Philadelphia negative. Results: A total of 34 chronic myeloproliferative neoplasm chromosome Philadelphia negative patients were identified. The main found diagnosis was Essential Thrombocythemia in 17 patients (50%, Polycythemia Rubra Vera in six patients (17.6%, chronic myeloproliferative neoplasm associated with eosinophilia in six patients (17.6%, myelofibrosis primary in three patients (8.8% and chronic myeloproliferative neoplasm not classifiable in two patients (5.8%. The median age was 63.5 years (R: 51-74 and 21 patients (61.7% were female. Two patients in the total number progressed Myelofibrosis (5.8%, no patient acute leukemia development. Twenty-seven patients (79.4% received Hydroxyurea as main pharmacological management. Fourteen patients presented complications (41.1%, of which fve were thrombotic episodes (14.7%, three bleeding episodes (8.8%, three patients had pulmonary hypertension (8.8% and one patient developed Vertigo (2.9%. Finally the time since diagnosis until the occurrence of complications was 19.55 months (R: 8-50.23. Conclusión: neoplasm Mieloproliferativas Chronicles are very rare pathologies, as many are grouped into essential Thrombocythemia, Polycythemia Vera and neoplasms associated with Eosinophilia. The main therapeutic option is with a low toxicity Hydroxyurea. It is not possible to analyze the presence of

  4. [Cognitive disorders in patients with chronic mercury intoxication].

    Science.gov (United States)

    Katamanova, E V; Shevchenko, O I; Lakhman, O L; Denisova, I A

    2014-01-01

    To assess severity of cognitive disorders in chronic mercury intoxication, the authors performed claster and discrimination analysis of neuropsychologic and neurophysiologic research data from workers exposed to mercury during long length of service, from patients with early and marked stages of chronic mercurial intoxication. Cognitive disorders in chronic mercurial intoxication have three severity degrees, in the light degree disorders patients demonstrate lower amplitude of cognitive evoked potentials, poor long-term memory and associative thinking. Moderate cognitive disorders are characterized by decreased visual, long-term memory, concentration of attention, poor optic and spatial gnosis. Marked cognitive disorders with chronic mercurial intoxication present with more decreased long-term, short-term, picturesque memory, poor intellect, optic and spatial gnosis and associative thinking. PMID:25051667

  5. Thyroid Disorders and Chronic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Mohamed Mohamedali

    2014-01-01

    Full Text Available Thyroid hormones play a very important role regulating metabolism, development, protein synthesis, and influencing other hormone functions. The two main hormones produced by the thyroid are triiodothyronine (T3 and thyroxine (T4. These hormones can also have significant impact on kidney disease so it is important to consider the physiological association of thyroid dysfunction in relation to chronic kidney disease (CKD. CKD has been known to affect the pituitary-thyroid axis and the peripheral metabolism of thyroid hormones. Low T3 levels are the most common laboratory finding followed by subclinical hypothyroidism in CKD patients. Hyperthyroidism is usually not associated with CKD but has been known to accelerate it. One of the most important links between thyroid disorders and CKD is uremia. Patients who are appropriately treated for thyroid disease have a less chance of developing renal dysfunction. Clinicians need to be very careful in treating patients with low T3 levels who also have an elevation in TSH, as this can lead to a negative nitrogen balance. Thus, clinicians should be well educated on the role of thyroid hormones in relation to CKD so that proper treatment can be delivered to the patient.

  6. Frequency of heterozygous TET2 deletions in myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Joseph Tripodi

    2010-09-01

    Full Text Available Joseph Tripodi1, Ronald Hoffman1, Vesna Najfeld2, Rona Weinberg31The Myeloproliferative Disorders Program, Tisch Cancer Institute, Department of Medicine and 2Department of Medicine and Pathology, Mount Sinai School of Medicine, 3The Myeloproliferative Disorders Program, Cellular Therapy Laboratory, The New York Blood Center, New York, NY, USAAbstract: The Philadelphia chromosome (Ph-negative myeloproliferative neoplasms (MPNs, including polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are a group of clonal hematopoietic stem cell disorders with overlapping clinical and cytogenetic features and a variable tendency to evolve into acute leukemia. These diseases not only share overlapping chromosomal abnormalities but also a number of acquired somatic mutations. Recently, mutations in a putative tumor suppressor gene, ten-eleven translocation 2 (TET2 on chromosome 4q24 have been identified in 12% of patients with MPN. Additionally 4q24 chromosomal rearrangements in MPN, including TET2 deletions, have also been observed using conventional cytogenetics. The goal of this study was to investigate the frequency of genomic TET2 rearrangements in MPN using fluorescence in situ hybridization as a more sensitive method for screening and identifying genomic deletions. Among 146 MPN patients, we identified two patients (1.4% who showed a common 4q24 deletion, including TET2. Our observations also indicated that the frequency of TET2 deletion is increased in patients with an abnormal karyotype (5%.Keywords: TET2, myeloproliferative neoplasms, fluorescence in situ hybridization, cytogenetics

  7. Social position of adolescents with chronic digestive disorders.

    OpenAIRE

    Calsbeek, H.; Rijken, M.; Bekkers, M.J.T.M.; Kerssens, J.J.; Dekker, J.; van Berge Henegouwen, G P

    2002-01-01

    OBJECTIVE : To investigate the consequences of having a chronic digestive disorder on the social position of adolescents. METHODS : Five diagnostic groups, including inflammatory bowel disease (IBD), chronic liver diseases, congenital digestive disorders, coeliac disease and food allergy (total n = 758, ages 12-25 years), were each compared with a population-based control group in a multicentre study using a cross-sectional design. Social position was assessed by a mailed questionnaire measur...

  8. Atypical Chronic Myelogenous Leukemia

    Science.gov (United States)

    ... myeloproliferative neoplasms, leukemia , and other conditions . Chronic Myelomonocytic Leukemia Key Points Chronic myelomonocytic leukemia is a disease ... chance of recovery) and treatment options. Chronic myelomonocytic leukemia is a disease in which too many myelocytes ...

  9. Burnout in Patients with Chronic Whiplash-Associated Disorders

    Science.gov (United States)

    Clementz, Gunilla; Borsbo, Bjorn; Norrbrink, Cecilia

    2012-01-01

    This study sought to assess burnout and its relation to pain, disability, mood and health-related quality of life in a group of patients with chronic whiplash-associated disorders (WAD). Forty-five patients with chronic WAD ([greater than or equal to] 3 months) referred to a multidisciplinary rehabilitation centre were included. A questionnaire…

  10. Recovery from chronic factitious disorder (Munchausen's syndrome): a personal account.

    Science.gov (United States)

    Bass, Christopher; Taylor, Michael

    2013-01-01

    This case report provides an account by a patient (with permission) of chronic factitious disorder and the factors that led to recovery. Such accounts are extremely rare in the literature. This account also throws into sharp focus current controversies in the classification of factitious disorders. PMID:24343927

  11. Methylphenidate in Treatment of ADHD and Comorbid Chronic Tic Disorder

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2007-07-01

    Full Text Available The safety and efficacy of immediate-release methylphenidate (MPH-IR for the treatment of attention deficit hyperactivity disorder (ADHD in children (ages 6-12 years with Tourette's syndrome (96% or chronic motor tic disorder (4% were evaluated at State University of New York, Stony Brook.

  12. Methylphenidate and Comorbid Anxiety Disorder in Children with both Chronic Multiple Tic Disorder and ADHD

    Science.gov (United States)

    Gadow, Kenneth D.; Nolan, Edith E.

    2011-01-01

    Objective: To determine if comorbid anxiety disorder is associated with differential response to immediate release methylphenidate (MPH-IR) in children with both ADHD and chronic multiple tic disorder (CMTD). Method: Children with (n = 17) and without (n = 37) diagnosed anxiety disorder (ANX) were evaluated in an 8-week, placebo-controlled trial…

  13. BCR-ABL Promotes PTEN Downregulation in Chronic Myeloid Leukemia

    OpenAIRE

    Cristina Panuzzo; Sabrina Crivellaro; Giovanna Carrà; Angelo Guerrasio; Giuseppe Saglio; Alessandro Morotti

    2014-01-01

    Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by the t(9;22) translocation coding for the chimeric protein p210 BCR-ABL. The tumor suppressor PTEN plays a critical role in the pathogenesis of CML chronic phase, through non genomic loss of function mechanisms, such as protein down-regulation and impaired nuclear/cytoplasmic shuttling. Here we demonstrate that BCR-ABL promotes PTEN downregulation through a MEK dependent pathway. Furthermore, we describe a novel n...

  14. [Utility of bone marrow biopsy in the diagnosis of myeloproliferative neoplasm].

    Science.gov (United States)

    Tovar-Bobadilla, José Leonard; Ortiz-Hidalgo, Carlos

    2016-01-01

    A diagnostic approach of myeloproliferative neoplasms, according to the 2008 WHO classification system for hematological malignancies, has to consider clinical, molecular, and cytogenetic information as well as bone marrow histology. A diagnosis of chronic myeloid leukemia requires the presence of BCR-ABL-1, and the Philadelphia chromosome-negative (Ph-1-negative) myeloproliferative neoplasms constitute three main subtypes, including primary myelofibrosis, polycythemia rubra vera, and essential thrombocythemia. These three Ph-1-negative myeloproliferative neoplasms share many pathogenic characteristic such as JAK2 mutations; however, they differ in prognosis, progression to myelofibrosis, and risk of leukemic transformation. There are currently various major points of interest in bone marrow examination in myeloproliferative neoplasms. One is the morphology of megakaryocytes, which are the hallmark of Ph-1-negative myeloproliferative neoplasms and play a crucial role in separating the different subtypes of myeloproliferative neoplasms. Another is reticulin fibrosis or collagen fibrosis, which may only be detected on a bone marrow biopsy specimen by reticulin and trichrome stains, respectively, and immunohistochemistry and certain molecular techniques may be applied in bone marrow biopsies as supporting evidence of certain features of myeloproliferative neoplasms. PMID:27335198

  15. Oral disorders in patients with chronic renal failure. Narrative review.

    OpenAIRE

    Carolina Hernández

    2016-01-01

    Chronic renal failure (CRF) is one of the best known renal diseases. It is characterized by a deterioration in the overall renal function and is associated with other conditions such as hypertension, diabetes mellitus, uropathy, chronic glomerulonephritis and autoimmune diseases. Patients with CRF show alterations of the masticatory system that are specific to the disease and other type of disorders as a result of treatment. Oral health in dialysis and transplant patients tends to be poor, wh...

  16. A PHASE I STUDY OF THE NOVEL RIBONUCLEOTIDE REDUCTASE INHIBITOR 3-AMINOPYRIDINE-2-CARBOXALDEHYDE THIOSEMICARBAZONE (3-AP, TRIAPINE®) IN COMBINATION WITH THE NUCLEOSIDE ANALOG FLUDARABINE FOR PATIENTS WITH REFRACTORY ACUTE LEUKEMIAS AND AGGRESSIVE MYELOPROLIFERATIVE DISORDERS

    Science.gov (United States)

    Karp, Judith E.; Giles, Francis J.; Gojo, Ivana; Morris, Lawrence; Greer, Jacqueline; Johnson, Bonny; Thein, Mya; Sznol, Mario; Low, Jennifer

    2009-01-01

    Triapine® is a potent ribonucleotide reductase (RR) inhibitor that depletes intracellular deoxyribonculeotide pools, especially dATP. We designed a Phase I trial of Triapine followed by the adenosine analog fludarabine in adults with refractory acute leukemias and aggressive myeloproliferative disorders (MPD). Two schedules were examined: A. Triapine 105mg/m2/day over 4 hours followed by fludarabine daily × 5 (24 patients, fludarabine 15–30 mg/m2/dose); B. Triapine 200mg/m2 over 24 hours followed by 5 days of fludarabine 30 mg/m2/day (9 patients). Complete and partial responses (CR,PR) occurred in Schedule A (5/24, 21%), with CR occurring at the 2 highest fludarabine doses (2/12, 17%). In contrast, no CR or PR occurred in Schedule B. Four of the 5 responses occurred in patients with underlying MPD (4/14, 29%). Drug-related toxicities included fever and metabolic acidosis. Triapine 105 mg/m2 followed by fludarabine 30mg/m2 daily × 5 is active in refractory myeloid malignancies and warrants continuing study for patients with aggressive MPD. PMID:17640728

  17. Molecular diagnostics of myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Langabeer, S. E.; Andrikovics, H.; Asp, J.;

    2015-01-01

    Since the discovery of the JAK2 V617F mutation in the majority of the myeloproliferative neoplasms (MPN) of polycythemia vera, essential thrombocythemia and primary myelofibrosis ten years ago, further MPN-specific mutational events, notably in JAK2 exon 12, MPL exon 10 and CALR exon 9 have been...

  18. Drugs Approved for Myeloproliferative Neoplasms

    Science.gov (United States)

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for myeloproliferative neoplasms. The list includes generic names, brand names, and common drug combinations, which are shown in capital letters. The drug names link to NCI's Cancer Drug Information summaries.

  19. Orofacial pain, jaw function, and temporomandibular disorders in adult women with a history of juvenile chronic arthritis or persistent juvenile chronic arthritis

    DEFF Research Database (Denmark)

    Bakke, M.; Zak, M.; Jensen, B.L.;

    2001-01-01

    Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis......Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis...

  20. Correlation between JAK2 allele burden and pulmonary arterial hypertension and hematological parameters in Philadelphia negative JAK2 positive myeloproliferative neoplasms. An Egyptian experience.

    Science.gov (United States)

    Mattar, Mervat M; Morad, Mohammed Abdel Kader; El Husseiny, Noha M; Ali, Noha H; El Demerdash, Doaa M

    2016-10-01

    Myeloproliferative neoplasms are characterized by a common stem cell-derived clonal proliferation, but are phenotypically diverse. JAK2 is mutated (V617F) in more than 90 % of patients with polycythemia vera (PV) and approximately 60 % of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). Pulmonary arterial hypertension (PAH) is a major complication of several hematological disorders. Chronic myeloproliferative disorders associated with PAH have been included in group five for which the etiology is unclear and/or multifactorial. The aim of this study is to screen Egyptian Philadelphia negative JAK2 positive myeloproliferative neoplasm patients for the presence of PAH and its correlation with JAK2 allele burden. We also made a review for correlation of JAK2 allele with hematological parameters comparing our results to others. We enrolled 60 patients with Philadelphia negative myeloproliferative neoplasms. All patients enrolled in the study were subjected to laboratory and imaging workup in the form of CBC, liver, kidney profile, bone marrow examination, abdominal ultrasonography, and transthoracic echocardiography. Our results revealed that 7 patients out of 60 (11.67 %) had pulmonary arterial hypertension, 3 patients with PMF, 2 patients with PRV, and 2 patients with ET, and its correlation with JAK2 allele burden was not statistically significant. Correlation analysis between JAK2 V617F allele burden and other parameters revealed: statistical significant correlation with age, HB, HCT, PLT, UA, LDH, and splenic diameter but insignificant correlation with WBCs and PAH. Pulmonary arterial hypertension prevalence in our study was 11.67 % and no significant correlation with JAK 2 allele burden. Our study is the largest one up to our knowledge that studies the association between its prevalence and JAK2 burden. PMID:27468853

  1. Major affective disorders in chronic obstructive pulmonary disease compared with other chronic respiratory diseases

    Directory of Open Access Journals (Sweden)

    Pothirat C

    2015-08-01

    Full Text Available Chaicharn Pothirat, Warawut Chaiwong, Nittaya Phetsuk, Sangnual Pisalthanapuna, Nonglak Chetsadaphan, Juthamas InchaiDivision of Pulmonary, Critical Care and Allergy, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, ThailanBackground: Chronic obstructive pulmonary disease (COPD and other chronic respiratory diseases (CRDs have significant impacts on quality of life including psychomotor domain.Purpose: To evaluate three major affective disorders in subjects with COPD compared with other CRDs and nonill population.Materials and methods: The Thai version of Mini International Neuropsychiatric Interview (MINI was used as a diagnostic instrument for three major affective disorders (generalized anxiety disorder, major depressive disorder, and panic disorder by face-to-face interview in assessing patients with CRDs [COPD, asthma, rhinasthma, all asthma (asthma and rhinasthma, and chronic rhinitis], and nonill subjects. Logistic regression analyses were used to determine the relation between major affective disorders and CRDs adjusting for age, sex, and disease severity.Results: Major affective disorders were more prevalent in CRDs than nonill groups (adjusted OR =2.6 [95% CI, 1.8-3.9], P<0.001. COPD patients had significantly more generalized anxiety and panic disorder (adjusted OR =4.0 [95% CI, 1.4-11.9], P=0.011, and 4.4 [95% CI, 1.1-18.1], P=0.038, respectively but not major depressive disorder (adjusted OR =2.7 [95% CI, 0.8-9.0, P=0.105] than nonill group. Comparing with all asthma, COPD patients had lower occurrence of major depressive and panic disorders (adjusted OR =0.1 [95% CI, 0.0-0.4], P=0.002, and 0.1 [95% CI, 0.0-0.9], P=0.043, respectively. There was no difference in major mood disorders in COPD, rhinasthma, and chronic rhinitis patients. Major affective disorders were not increased by disease severity in COPD.Conclusion: Major affective disorders were significantly higher in CRDs than nonill

  2. Psychiatric co-morbidity in chronic pain disorder

    International Nuclear Information System (INIS)

    Objective: To determine the psychiatric co-morbidity in patients with chronic pain disorder in hospital setting. Design: Cross sectional descriptive study. Place and duration of study: This study was conducted at Combined Military Hospital (CMH) Okara from June 2011 to May 2012. Patients and Methods: A purposive sample of 400 patients (males=117; females=283) gathered from pain clinic and other outpatient departments of the hospital and were interviewed in detail and Present State Examination was carried out. Demographic variables were scored using descriptive statistics and results were analyzed using correlation methods. Results: It was revealed that psychiatric illness in overall sample prevailed among 266 participants (67%). Among which 164 participants (62%) were diagnosed with depression, 67 patients (25.2%) of chronic pain were diagnosed with anxiety disorders, 28 patients (11%) with adjustment disorder and 1.5% and 1.1% diagnosed with drug dependence and somatization disorder, respectively. Conclusion: Psychiatric co-morbidity especially the incidence of depression, anxiety and adjustment disorders were high amongst patients suffering from chronic pain disorder. (author)

  3. New aspects into pathophysiology and molecular diagnostics of myeloproliferative neoplasms

    OpenAIRE

    Siebolts, Udo

    2011-01-01

    The Philadelphia chromosome negative myeloproliferative neoplasms (MPN) comprise diverse entities of hematopoetic stem cell disorders with hematopoetic stem cell transplantation as the only curative therapeutic option. A collective finding of some subgroups is the activating point mutation of JAK2 p.V617F, important for diagnosis and detection of minimal residual disease (MRD) but a rather late event in the course of MPN. In this study, we first focused on characteristics of the neoplastic...

  4. Altered Regional Cerebral Blood Flow in Chronic Whiplash Associated Disorders

    NARCIS (Netherlands)

    Vállez García, David; Doorduin, Janine; Willemsen, Antoon T.M.; Dierckx, Rudi A.j.o.; Otte, Andreas

    2016-01-01

    There is increasing evidence of central hyperexcitability in chronic whiplash-associated disorders (cWAD). However, little is known about how an apparently simple cervical spine injury can induce changes in cerebral processes. The present study was designed (1) to validate previous results showing a

  5. Cesarean Section and Chronic Immune Disorders

    DEFF Research Database (Denmark)

    Sevelsted, Astrid; Stokholm, Jakob; Bønnelykke, Klaus;

    2015-01-01

    OBJECTIVES: Immune diseases such as asthma, allergy, inflammatory bowel disease, and type 1 diabetes have shown a parallel increase in prevalence during recent decades in westernized countries. The rate of cesarean delivery has also increased in this period and has been associated with the...... development of some of these diseases. METHODS: Mature children born by cesarean delivery were analyzed for risk of hospital contact for chronic immune diseases recorded in the Danish national registries in the 35-year period 1977-2012. Two million term children participated in the primary analysis. We...... studied childhood diseases with a suspected relation to a deviant immune-maturation and a debut at young age. The effect of cesarean delivery on childhood disease incidences were estimated by means of confounder-adjusted incidence rate ratios with 95% confidence intervals obtained in Poisson regression...

  6. Cognitive therapy of obsessive compulsive disorder with chronic tic disorder

    Directory of Open Access Journals (Sweden)

    Sudhir Hebbar

    2013-01-01

    Full Text Available The gold standard of therapy for obsessive compulsive disorder, exposure with response prevention, may not be suitable to obsessional sub-type. Live exposure is not possible and response prevention is difficult. These obsessions (sexual, religious or aggressive are repugnant and resisted. Negative attitude against obsessions leads to treatment refusal or dropout from therapy. In Cognitive therapy (CT these attitudes can be corrected and exposure can be administered in the form of behavioral experiments (using behavioral tasks to change the dysfunctional attitudes. Such a case is treated with CT, weaned off medications and remains improved at 9 months.

  7. Macrolides in Chronic Inflammatory Skin Disorders

    Directory of Open Access Journals (Sweden)

    Abdullateef A. Alzolibani

    2012-01-01

    Full Text Available Long-term therapy with the macrolide antibiotic erythromycin was shown to alter the clinical course of diffuse panbronchiolitis in the late 1980s. Since that time, macrolides have been found to have a large number of anti-inflammatory properties in addition to being antimicrobials. These observations provided the rationale for many studies performed to assess the usefulness of macrolides in other inflammatory diseases including skin and hair disorders, such as rosacea, psoriasis, pityriasis rosea, alopecia areata, bullous pemphigoid, and pityriasis lichenoides. This paper summarizes a collection of clinical studies and case reports dealing with the potential benefits of macrolides antibiotics in the treatment of selected dermatoses which have primarily been classified as noninfectious and demonstrating their potential for being disease-modifying agents.

  8. Chronic cough in patients with sleep-disordered breathing.

    Science.gov (United States)

    Chan, K K Y; Ing, A J; Laks, L; Cossa, G; Rogers, P; Birring, S S

    2010-02-01

    Chronic cough can be the sole presenting symptom for patients with obstructive sleep apnoea. We investigated the prevalence, severity and factors associated with chronic cough in patients with sleep-disordered breathing (SDB). We invited 108 consecutive patients who had been referred for evaluation of SDB to complete a comprehensive questionnaire on respiratory and sleep health, which included the Leicester Cough Questionnaire (cough specific quality of life; LCQ), Epworth Sleepiness Scale (ESS) and the Mayo Clinic gastro-oesophageal questionnaire. Chronic cough was defined as cough for a duration of >2 months. 33% of patients with SDB reported a chronic cough. Patients with a chronic cough had impaired cough related-quality of life affecting all health domains (mean+/-sem LCQ score 17.7+/-0.7; normal = 21). Patients with SDB and chronic cough were predominantly females (61% versus 17%; p<0.001) and reported more nocturnal heartburn (28% versus 5%; p = 0.03) and rhinitis (44% versus 14%; p = 0.02) compared to those without SDB. There were no significant differences in ESS, respiratory disturbance index, body mass index, or symptoms of breathlessness, wheeze, snoring, dry mouth and choking between those with cough and those without. Chronic cough is prevalent in patients with SDB and is associated with female sex, symptoms of nocturnal heartburn and rhinitis. Further studies are required to investigate the impact of continuous positive airway pressure therapy on cough associated with SDB to explore the mechanism of this association. PMID:20123846

  9. Calreticulin Mutations in Myeloproliferative Neoplasms

    OpenAIRE

    Noa Lavi

    2014-01-01

    With the discovery of the JAK2V617F mutation in patients with Philadelphia chromosome-negative (Ph−) myeloproliferative neoplasms (MPNs) in 2005, major advances have been made in the diagnosis of MPNs, in understanding of their pathogenesis involving the JAK/STAT pathway, and finally in the development of novel therapies targeting this pathway. Nevertheless, it remains unknown which mutations exist in approximately one-third of patients with non-mutated JAK2 or MPL essential thrombocythemia (...

  10. Radioimmunometric analysis of histamine in myeloproliferative syndromes

    International Nuclear Information System (INIS)

    Quantitative analysis of histamine is increasingly used in clinical haematology. The present study demonstrates the properties of and potential indication for a novel histamine radioimmunoassay (RIA) in clinical haematology. The sensitivity of this test assay corresponds to a histamine level of 0,1 to 0,5 nM, the non-specific cross reaction with endogeneous histamine metabolites appears to be less than 0,1%. The total histamine levels in the peripheral blood of healthy donors (n = 10) ranged from 10 to 100 ng/ml, the plasma histamine values from 0,02 to 0,6 ng/ml blood. Increased levels of total histamine were measured in myeloproliferative syndromes, i.e. in patients with chronic myeloid leukemia (CML)(8 of 9), myelofibrosis (OMS)(2 of 4), and polycythaemia vera (PCV)(1 of 2). An excessive increase in total histamine was observed in healthy rhesus monkeys (n = 10) treated with recombinant human interleukin-3 (rhIL-3). The total histamine value correlated with the absolute number of circulating blood basophils (correlation coefficient: 0,9). The calculated content of histamine per basophil was found to be 0,5 to 1,5 pg. Plasma histamine values in patients suffering from myeloproliferative syndromes were within the normal range. In contrast, a moderate to marked increase in plasma histamine values was observed in monkeys during IL-3 treatment. The radioimmunometric analysis of histamine clearly represents a useful new test system in clinical haematology, especially in the follow up of malignant as well as IL-3-induced myeloproliferation. (Authors)

  11. [The adolescent with a chronic somatic disorder].

    Science.gov (United States)

    Bettschart, W

    1992-01-25

    Adolescence, a period of psychic reorganization which allows for new adjustments in order to face future life and reorganize the experience of the past, is one of the critical phases of existence. It is described as a "new birth", a successful achievement of individuation and separation processes. For the adolescent with an infirmity or chronic illness, and for his family, there exist specific problems in addition to those encountered by a healthy adolescent. The painful realization of social, professional and relational barriers is reactivated. The feeling of failure and helplessness, the impairment of self-esteem, and anger at being the victim of the unfairness of destiny, represent a supplementary affective burden for the adolescent and his family. This results in a risk of deterioration, either towards affective and social withdrawal expressed by overadaptation and weakening of psychic functions, or towards autoaggressive acting-out or various forms of marginalization. Preventive and therapeutic action by the individual and the family are discussed in the light of the case history of an adolescent suffering from a facial malformation. PMID:1734504

  12. Skin prick test in patients with chronic allergic skin disorders

    Directory of Open Access Journals (Sweden)

    Pooja Bains

    2015-01-01

    Full Text Available Background: Chronic allergic skin disorders are the inflammatory and proliferative conditions in which both genetic and environmental factors play important roles. Chronic idiopathic urticaria (CIU and atopic dermatitis (AD are among the most common chronic allergic skin disorders. These can be provoked by various food and aeroallergens. Skin prick tests (SPTs represent the cheapest and most effective method to diagnose type I hypersensitivity. Positive skin tests with a history suggestive of clinical sensitivity strongly incriminate the allergen as a contributor to the disease process. Aim s and Objectives: To determine the incidence of positive SPT in patients with chronic allergic skin disorders and to identify the various allergens implicated in positive SPT. Methods: Fifty patients of chronic allergic disorders were recruited in this study. They were evaluated by SPT with both food and aeroallergens. Results: In our study, SPT positivity in patients of CIU was 63.41% and in AD was 77.78%. Out of the 41 patients of CIU, the most common allergen groups showing SPT positivity were dust and pollen, each comprising 26.83% patients. SPT reaction was positive with food items (21.6%, insects (17.07%, fungus (12.20%, and Dermatophagoides farinae, that is, house dust mite (HDM (7.32%. The allergen which showed maximum positivity was grain dust wheat (19.51%. Among nine patients of AD, maximum SPT positivity was seen with Dermatophagoides farinae, pollen Amaranthus spinosus, grain dust wheat, and cotton mill dust; each comprising 22.22% of patients. Conclusion: Our study showed that a significant number of patients of CIU and AD showed sensitivity to dust, pollen, insects, Dermatophagoides farinae, and fungi on SPT. Thus, it is an important tool in the diagnosis of CIU and AD.

  13. Chronic myeloid leukaemia occurring in a patient with hairy cell leukaemia

    OpenAIRE

    Wandroo, F; Bareford, D; El-Jehani, F

    2000-01-01

    Occurrences of second malignancies in hairy cell leukaemia are well recognised. Most of these malignancies are either solid tumours or lymphoproliferative disorders. The association of myeloproliferative disorders with hairy cell leukaemia (HCL) is very rare. This report describes a case of a patient with HCL who after remaining in remission developed Philadelphia chromosome positive chronic myeloid leukaemia (CML), which rapidly transformed to acute lymphoblastic leukaemia with further cytog...

  14. Veliparib and Topotecan With or Without Carboplatin in Treating Patients With Relapsed or Refractory Acute Leukemia, High-Risk Myelodysplasia, or Aggressive Myeloproliferative Disorders

    Science.gov (United States)

    2016-04-05

    Adult Acute Megakaryoblastic Leukemia; Adult Acute Monoblastic Leukemia; Adult Acute Monocytic Leukemia; Adult Acute Myeloid Leukemia With Inv(16)(p13.1q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With Maturation; Adult Acute Myeloid Leukemia With Minimal Differentiation; Adult Acute Myeloid Leukemia With t(16;16)(p13.1;q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); RUNX1-RUNX1T1; Adult Acute Myeloid Leukemia With t(9;11)(p22;q23); MLLT3-MLL; Adult Acute Myeloid Leukemia Without Maturation; Adult Acute Myelomonocytic Leukemia; Adult Erythroleukemia; Adult Pure Erythroid Leukemia; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Essential Thrombocythemia; Hematopoietic and Lymphoid Cell Neoplasm; Philadelphia Chromosome Negative, BCR-ABL1 Positive Chronic Myelogenous Leukemia; Polycythemia Vera; Previously Treated Myelodysplastic Syndrome; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Disease; Secondary Myelodysplastic Syndrome

  15. Multicomponent Behavioral Treatment for Chronic Combat-Related Posttraumatic Stress Disorder: Trauma Management Therapy

    Science.gov (United States)

    Turner, Samuel M.; Beidel, Deborah C.; Frueh, B. Christopher

    2005-01-01

    Posttraumatic stress disorder (PTSD) is a severe and chronic mental disorder that is highly prevalent within Veterans Affairs (VA) Medical Centers. A severe psychiatric disorder, combat-related PTSD is typically accompanied by multiple comorbid psychiatric disorders, symptom chronicity, and extreme social maladjustment. Thus, PTSD is a complex…

  16. Sleep disorders in pediatric chronic kidney disease patients.

    Science.gov (United States)

    Stabouli, Stella; Papadimitriou, Eleni; Printza, Nikoleta; Dotis, John; Papachristou, Fotios

    2016-08-01

    The prevalence of sleep disorders during childhood has been estimated to range from 25 to 43 %. The aim of this review is to determine the prevalence of sleep disorders and possible associations with chronic kidney disease (CKD)-related factors and health-related quality of life (HRQOL) in children with CKD. An electronic systematic literature search for sleep disorders in children with CKD in Pubmed, Embase and the Cochrane Library Databases identified seven relevant articles for review, all of which reported an increased prevalence of sleep disorders in children with CKD. Five studies included children with CKD undergoing dialysis, and two studies included only non-dialysis patients. In all studies the presence of sleep disturbances was assessed by questionnaires; only one study compared the results of a validated questionnaire with laboratory-based polysomnography. The prevalence of any sleep disorder ranged from 77 to 85 % in dialysis patients, to 32-50 % in transplanted patients and 40-50 % in non-dialysis patients. The most commonly studied disorder was restless legs syndrome, which presented at a prevalence of 10-35 %. Three studies showed significant associations between presence of sleep disorders and HRQOL. We found consistent evidence of an increased prevalence of sleep disturbances in children with CKD, and these seemed to play a critical role in HRQOL. PMID:26482250

  17. Associations between chronic pelvic pain and psychiatric disorders and symptoms

    OpenAIRE

    ANA CAROLINA FRANCO CARVALHO; OMERO BENEDITO POLI NETO; JOSÉ ALEXANDRE DE SOUZA CRIPPA; JAIME EDUARDO CECÍLIO HALLAK; FLÁVIA DE LIMA OSÓRIO

    2015-01-01

    Background Chronic pelvic pain (CPP) is a complex condition wich is associated with emotional factors, specially depression and anxiety. Objectives To make a systematic review to provide a detailed summary of relevant literature on the association between CPP and different psychiatric disorders/symptoms. Methods A systematic review of articles in the international literature published between 2003 and 2014 was performed in the electronic databases PubMed, PsycINFO, LILACS, and SciELO using th...

  18. Targeted treatment of chronic myeloid leukemia: role of imatinib

    OpenAIRE

    Ila Tamascar; Jeyanthi Ramanarayanan

    2009-01-01

    Ila Tamascar, Jeyanthi RamanarayananDepartment of Medical Oncology, Roswell Park Cancer Institute, Elm and Carlton Streets, Buffalo, NY, USAAbstract: Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by clonal expansion of pleuripotent hematopoetic stem cells. The incidence of CML is 1 to 2 cases per 100,000 people per year; in the Western Hemisphere, CML accounts for 15% of leukemias in adults. Discovery of the specific karyotypic abnormality of the Philadelphia (...

  19. Safety and efficacy of combination therapy of interferon-alpha2 + JAK1-2 Inhibitor in the philadelphia-negative chronic myeloproliferative neoplasms. Preliminary results from the danish combi-trial-an open label, single arm, non-randomized multicenter phase II study

    DEFF Research Database (Denmark)

    Mikkelsen, S. U.; Kjaer, L.; Skov, V.;

    2015-01-01

    IFNa2 as well. The purpose of this COMBI-trial is to evaluate the safety and efficacy of CT with IFNa2 and ruxolitinib. Patients and Methods: At the time of data cutoff, a total of 30 pts >18 years with prefibrotic or hyperproliferative PMF (n=7), PV (n=20) or post-PV MF (PPV-MF) (n=3) with or without......Background: The Philadelphia-negative, chronic myeloproliferative neoplasms (MPN) include essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (MF) (PMF). Chronic inflammation and a deregulated immune system are considered important for clonal evolution and disease...... progression. Ruxolitinib is a potent anti-inflammatory agent and has shown great benefit in MPN patients (pts), reducing spleen size and inflammation-mediated symptoms, thereby improving quality of life (QoL). Interferon-alpha2 (IFNa2) has been used successfully for decades in the treatment of MPN. However...

  20. Sleep-related disorders in chronic obstructive pulmonary disease.

    LENUS (Irish Health Repository)

    Crinion, Sophie J

    2014-02-01

    Sleep may have several negative consequences in patients with chronic obstructive pulmonary disease (COPD). Sleep is typically fragmented with diminished slow wave and rapid-eye-movement sleep, which likely represents an important contributing factor to daytime symptoms such as fatigue and lethargy. Furthermore, normal physiological adaptations during sleep, which result in mild hypoventilation in normal subjects, are more pronounced in COPD, which can result in clinically important nocturnal oxygen desaturation. The co-existence of obstructive sleep apnea and COPD is also common, principally because of the high prevalence of each disorder, and there is little convincing evidence that one disorder predisposes to the other. Nonetheless, this co-existence, termed the overlap syndrome, typically results in more pronounced nocturnal oxygen desaturation and there is a high prevalence of pulmonary hypertension in such patients. Management of sleep disorders in patients with COPD should address both sleep quality and disordered gas exchange. Non-invasive pressure support is beneficial in selected cases, particularly during acute exacerbations associated with respiratory failure, and is particularly helpful in patients with the overlap syndrome. There is limited evidence of benefit from pressure support in the chronic setting in COPD patients without obstructive sleep apnea.

  1. Role of chronic health disorders in perimenopausal fractures.

    Science.gov (United States)

    Huopio, Jukka; Honkanen, Risto; Jurvelin, Jukka; Saarikoski, Seppo; Alhava, Esko; Kröger, Heikki

    2005-11-01

    Only a few studies have examined the risk of individual chronic health disorders on perimenopausal (i.e., around menopause) fractures in a single study. We evaluated the effect of chronic illnesses on fracture rate in a prospective cohort study of 3,078 women. These women were a stratified sample from the population base of 14,220 women aged 47-56 years and residing in the province of Kuopio in eastern Finland in 1989. Data on physician-diagnosed chronic diseases were collected by a baseline questionnaire in 1989. For certain diseases, questionnaire information of self-reported chronic disorders were compared with drug reimbursement data provided by the Social Insurance Institution of Finland. Axial bone mineral density (BMD) measurements from the femoral neck and lumbar spine were performed in 1989-91. Two hundred sixty-five (265) women experienced at least one fracture during the follow-up period of 3.6 years (SD+/-0.78). The first fracture during the follow-up period was taken to be the end-point event. The risk of follow-up fracture for an individual health disorder was estimated with the Cox's proportional hazards model. Several chronic health disorders increased the fracture risk in perimenopausal women. However, hypertension was a statistically significant (p=0.018) risk factor for fracture (adjusted hazard ratio [HR], 1.4; 95% confidence interval [CI], 1.1-1.9), especially in overweight and obese (body mass index > or =28) women (HR, 2.0; 95% CI, 1.4-3.0). In addition, coronary heart disease (adjusted HR, 1.76; 95% CI, 1.13-2.76), hyperthyroidism (adjusted HR, 1.7; 95% CI, 1.0-2.9), epilepsy (adjusted HR, 2.0; 95% CI, 1.1-3.6), alcoholism (adjusted HR, 3.5; 95% CI, 1.3-9.5) and chronic hepatic disease (adjusted HR, 5.2; 95% CI, 1.7-16.4) predicted fracture. BMD was either normal or even elevated in disease groups. However, women with a fracture during the follow-up usually had decreased bone density, although the difference was statistically significant

  2. Associations between chronic pelvic pain and psychiatric disorders and symptoms

    Directory of Open Access Journals (Sweden)

    ANA CAROLINA FRANCO CARVALHO

    2015-02-01

    Full Text Available Background Chronic pelvic pain (CPP is a complex condition wich is associated with emotional factors, specially depression and anxiety. Objectives To make a systematic review to provide a detailed summary of relevant literature on the association between CPP and different psychiatric disorders/symptoms. Methods A systematic review of articles in the international literature published between 2003 and 2014 was performed in the electronic databases PubMed, PsycINFO, LILACS, and SciELO using the terms (chronic pelvic pain AND (psychiatry OR psychiatric OR depression OR anxiety OR posttraumatic stress OR somatoform. The searches returned a total of 529 matches that were filtered according to predefined inclusion and exclusion criteria. A total of 18 articles were selected. Results The investigations focused mainly on the assessment of depression and anxiety disorders/symptoms, with rather high rates (17-38.6%. Depression and anxiety symptoms were more prevalent among women with CPP compared to healthy groups. Comparisons between groups with CPP and with specific pathologies that also have pain as a symptom showed that depression indicators are more frequent in CPP. Depressive symptoms tend to be more common in CPP and have no particular association with pain itself, the core feature of CPP. Discussion Other aspects of CPP seem to play a specific role in this association. Anxiety and other psychiatric disorders require further investigation so that their impact on CPP can be better understood.

  3. Chronic alcoholism-mediated metabolic disorders in albino rat testes

    Directory of Open Access Journals (Sweden)

    Shayakhmetova Ganna M.

    2014-09-01

    Full Text Available There is good evidence for impairment of spermatogenesis and reductions in sperm counts and testosterone levels in chronic alcoholics. The mechanisms for these effects have not yet been studied in detail. The consequences of chronic alcohol consumption on the structure and/or metabolism of testis cell macromolecules require to be intensively investigated. The present work reports the effects of chronic alcoholism on contents of free amino acids, levels of cytochrome P450 3A2 (CYP3A2 mRNA expression and DNA fragmentation, as well as on contents of different cholesterol fractions and protein thiol groups in rat testes. Wistar albino male rats were divided into two groups: I - control (intact animals, II - chronic alcoholism (15% ethanol self-administration during 150 days. Following 150 days of alcohol consumption, testicular free amino acid content was found to be significantly changed as compared with control. The most profound changes were registered for contents of lysine (-53% and methionine (+133%. The intensity of DNA fragmentation in alcohol-treated rat testes was considerably increased, on the contrary CYP3A2 mRNA expression in testis cells was inhibited, testicular contents of total and etherified cholesterol increased by 25% and 45% respectively, and protein SH-groups decreased by 13%. Multidirectional changes of the activities of testicular dehydrogenases were detected. We thus obtained complex assessment of chronic alcoholism effects in male gonads, affecting especially amino acid, protein, ATP and NADPH metabolism. Our results demonstrated profound changes in testes on the level of proteome and genome. We suggest that the revealed metabolic disorders can have negative implication on cellular regulation of spermatogenesis under long-term ethanol exposure.

  4. Therapy with JAK2 inhibitors for Myeloproliferative Neoplasms

    OpenAIRE

    Santos, Fabio P S; Verstovsek, Srdan

    2012-01-01

    The development of JAK2 inhibitors followed the discovery of activating mutation of JAK2 (JAK2V617F) in patients with classic Philadelphia-negative myeloproliferative neoplasms (Ph-negative MPNs). It is now known that mutations activating the JAK-STAT pathway are ubiquitous in Ph-negative MPNs, and that deregulated JAK-STAT pathway plays a central role in the pathogenesis of these disorders. JAK2 inhibitors thus are effective in both patients with and without the JAK2V617F mutation. Clinical ...

  5. Philadelphia-negative classical myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Barbui, T.; Barosi, G.; Birgegard, G.;

    2011-01-01

    We present a review of critical concepts and produce recommendations on the management of Philadelphia-negative classical myeloproliferative neoplasms, including monitoring, response definition, first- and second-line therapy, and therapy for special issues. Key questions were selected according...

  6. WHO classification 2008 of myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Madelung, Ann B; Bondo, Henrik; Stamp, Inger;

    2015-01-01

    We examined the learning effect of a workshop for Danish hematopathologists led by an international expert regarding histological subtyping of myeloproliferative neoplasms (MPN). Six hematopathologists evaluated 43 bone marrow (BM) biopsies according to the WHO description (2008), blinded to...

  7. Immunoglobulin D Multiple Myeloma, Plasma Cell Leukemia and Chronic Myelogenous Leukemia in a Single Patient Treated Simultaneously with Lenalidomide, Bortezomib, Dexamethasone and Imatinib

    OpenAIRE

    Naveed Ali; Pickens, Peter V.; Auerbach, Herbert E.

    2016-01-01

    Multiple myeloma (MM) is a neoplastic lymphoproliferative disorder characterized by uncontrolled monoclonal plasma cell proliferation. Among different isotypes of MM, immunoglobulin D (IgD) MM is very rare, representing only 1 to 2% of all isotypes. Chronic myelogenous leukemia (CML) is a neoplastic myeloproliferative disorder of pluripotent hematopoietic stem cell, which is characterized by the uncontrolled proliferation of myeloid cells. An 88-year-old male was diagnosed simultaneously with...

  8. Complex cytogenetic findings in the bone marrow of a chronic idiopathic myelofibrosis patient

    OpenAIRE

    Tuğçe Bulakbaşı Balcı; Meltem Yüksel; Zerrin Yılmaz; Feride İffet Şahin

    2010-01-01

    Chronic idiopathic myelofibrosis is a myeloproliferative disorder characterized by splenomegaly, myeloid metaplasia and reactive bone marrow fibrosis. Karyotype analysis of the bone marrow is an integral part of the diagnosis, especially as a discriminative tool in ruling out reactive conditions. The frequency of clonal cytogenetic anomalies in this disease is the highest among its group, varying between 30 and 75%. Among these, trisomy 1q, 20q-, 13q- and +8 are the most common aberrations. H...

  9. Chronic organophosphate-induced neuropsychiatric disorder: a case report

    Directory of Open Access Journals (Sweden)

    Ghimire SR

    2016-02-01

    Full Text Available Shree Ram Ghimire,1 Sarita Parajuli2 1Department of Psychiatry, National Medical College, Birgunj, 2Department of Anesthesiology, Kathmandu National Medical College, Anamnagar, Kathmandu, Nepal Abstract: Chronic organophosphate (OP-induced neuropsychiatric disorder is a rare condition following prolonged exposure to OP compounds. Due to the lack of valid diagnostic tools and criteria, very few cases are seen in clinical practice and are often misdiagnosed. Misdiagnosis can lead to inappropriate treatment that may increase the risk of morbidity or suicidality. In this paper, we present the case of a 35-year-old male who needed support in breathing from a mechanical ventilator and developed neuropsychiatric behavioral problems following ingestion of OP compounds, which lead to suicidality. The patient was treated by the psychiatric team with antipsychotic and antidepressants and improved following the regular use of medication. Keywords: COPIND, mood liability, suicidal thoughts

  10. Autoinflammatory bone disorders with special focus on chronic recurrent multifocal osteomyelitis (CRMO)

    OpenAIRE

    Hedrich, Christian M.; Hofmann, Sigrun R.; Pablik, Jessica; Morbach, Henner; Girschick, Hermann J.

    2016-01-01

    Sterile bone inflammation is the hallmark of autoinflammatory bone disorders, including chronic nonbacterial osteomyelitis (CNO) with its most severe form chronic recurrent multifocal osteomyelitis (CRMO). Autoinflammatory osteopathies are the result of a dysregulated innate immune system, resulting in immune cell infiltration of the bone and subsequent osteoclast differentiation and activation. Interestingly, autoinflammatory bone disorders are associated with inflammation of the skin and/or...

  11. Peer Victimization in Youth with Tourette Syndrome and Other Chronic Tic Disorders

    Science.gov (United States)

    Zinner, Samuel H.; Conelea, Christine A.; Glew, Gwen M.; Woods, Douglas W.; Budman, Cathy L.

    2012-01-01

    Chronic tic disorders including Tourette syndrome have negative impact across multiple functional domains. We explored associations between peer victimization status and tic subtypes, premonitory urges, internalizing symptoms, explosive outbursts, and quality of life among youth with chronic tic disorders, as part of the internet-based omnibus…

  12. Telemedical Wearable Sensing Platform for Management of Chronic Venous Disorder.

    Science.gov (United States)

    Li, Ruya; Nie, Baoqing; Zhai, Chengwei; Cao, Jennifer; Pan, Jian; Chi, Yung-Wei; Pan, Tingrui

    2016-07-01

    Enabled by emerging wearable sensors, telemedicine can potentially offer personalized medical services to long-term home care or remote clinics in the future, which can be particularly helpful in the management of chronic diseases. The wireless wearable pressure sensing system reported in this article provides an excellent example of such an innovation, whereby periodic or continuous monitoring of interface pressure can be obtained to guide routine compression therapy, the cornerstone of chronic venous disorder management. By applying a novel capacitive, iontronic sensing technology, a flexible, ultrathin, and highly sensitive pressure sensing array is seamlessly incorporated into compression garments for the monitoring of interface pressure. The linear pressure sensing array assesses pressure distribution along the limb in a real-time manner (up to a scanning rate of 5 kHz), and the measurement data can be processed and displayed on a mobile device locally, as well as transmitted through a Bluetooth communication module to a remote clinical service. The proposed interface pressure measuring system provides real-time interface pressure distribution data and can be utilized for both clinical and self-management of compression therapy, where both treatment efficacy and quality assurance can be ascertained. PMID:26530542

  13. Oral disorders in patients with chronic renal failure. Narrative review.

    Directory of Open Access Journals (Sweden)

    Carolina Hernández

    2016-02-01

    Full Text Available Chronic renal failure (CRF is one of the best known renal diseases. It is characterized by a deterioration in the overall renal function and is associated with other conditions such as hypertension, diabetes mellitus, uropathy, chronic glomerulonephritis and autoimmune diseases. Patients with CRF show alterations of the masticatory system that are specific to the disease and other type of disorders as a result of treatment. Oral health in dialysis and transplant patients tends to be poor, which makes them more likely to develop pathological conditions in the oral cavity, potentially increasing morbidity, mortality and affecting the quality of life of patients. Among the lesions we can find dysgeusia, periodontitis, candidiasis, gingival bleeding, petechiae, and joint alterations. Gingivitis and xerostomia associated to long-term use medications can cause oral lesions. Children with CRF show two oral conditions of interest: high incidence of dental anomalies and low caries activity. In patients receiving a kidney transplant, previous dental treatment is critical because the immune status of the patient will be affected not only by the toxemia, but by the immunosuppressive drugs used to prevent organ rejection. Therefore, the dentist plays an important role in training parents and/or guardians, doctors and paramedics on the treatment of oral lesions in these patients.

  14. Chronic pain in adolescence and internalizing mental health disorders: a nationally representative study.

    Science.gov (United States)

    Noel, Melanie; Groenewald, Cornelius B; Beals-Erickson, Sarah E; Gebert, J Thomas; Palermo, Tonya M

    2016-06-01

    Chronic pain in childhood and adolescence has been shown to heighten the risk for depressive and anxiety disorders in specific samples in adulthood; however, little is known about the association between a wider variety of chronic pains and internalizing mental health disorders. Using nationally representative data, the objectives of this study were to establish prevalence rates of internalizing mental health disorders (anxiety and depressive disorders) among cohorts with or without adolescent chronic pain, and to examine whether chronic pain in adolescence is associated with lifetime history of internalizing mental health disorders reported in adulthood. Data from the National Longitudinal Study of Adolescent to Adult Health (Add Health) was used (N = 14,790). Individuals who had chronic pain in adolescence subsequently reported higher rates of lifetime anxiety disorders (21.1% vs 12.4%) and depressive disorders (24.5% vs 14.1%) in adulthood as compared with individuals without a history of adolescent chronic pain. Multivariate logistic regression confirmed that chronic pain in adolescence was associated with an increased likelihood of lifetime history of anxiety disorders (odds ratio: 1.33; 95% confidence interval: 1.09-1.63, P = 0.005) and depressive disorders (odds ratio: 1.38; confidence interval: 1.16-1.64, P < 0.001) reported in adulthood. Future research is needed to examine neurobiological and psychological mechanisms underlying these comorbidities. PMID:26901806

  15. Disorders of Iron Metabolism and Anemia in Chronic Kidney Disease.

    Science.gov (United States)

    Panwar, Bhupesh; Gutiérrez, Orlando M

    2016-07-01

    Dysregulated iron homeostasis plays a central role in the development of anemia of chronic kidney disease (CKD) and is a major contributor toward resistance to treatment with erythropoiesis-stimulating agents. Understanding the underlying pathophysiology requires an in-depth understanding of normal iron physiology and regulation. Recent discoveries in the field of iron biology have greatly improved our understanding of the hormonal regulation of iron trafficking in human beings and how its alterations lead to the development of anemia of CKD. In addition, emerging evidence has suggested that iron homeostasis interacts with bone and mineral metabolism on multiple levels, opening up new avenues of investigation into the genesis of disordered iron metabolism in CKD. Building on recent advances in our understanding of normal iron physiology and abnormalities in iron homeostasis in CKD, this review characterizes how anemia related to disordered iron metabolism develops in the setting of CKD. In addition, this review explores our emerging recognition of the connections between iron homeostasis and mineral metabolism and their implications for the management of altered iron status and anemia of CKD. PMID:27475656

  16. Undiagnosed mood disorders and sleep disturbances in primary care patients with chronic musculoskeletal pain.

    OpenAIRE

    2013-01-01

    Objective. The study aims to determine the prevalence of undiagnosed comorbid mood disorders in patients suffering chronic musculoskeletal pain in a primary care setting and to identify sleep disturbances and other associated factors in these patients, and to compare the use of health services by chronic musculoskeletal pain patients with and without comorbid mood disorders. Design. Cross-sectional study. Subjects. A total of 1,006 patients with chronic musculoskele...

  17. Chronic traumatic encephalopathy pathology in a neurodegenerative disorders brain bank.

    Science.gov (United States)

    Bieniek, Kevin F; Ross, Owen A; Cormier, Kerry A; Walton, Ronald L; Soto-Ortolaza, Alexandra; Johnston, Amelia E; DeSaro, Pamela; Boylan, Kevin B; Graff-Radford, Neill R; Wszolek, Zbigniew K; Rademakers, Rosa; Boeve, Bradley F; McKee, Ann C; Dickson, Dennis W

    2015-12-01

    Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disorder linked to repetitive traumatic brain injury (TBI) and characterized by deposition of hyperphosphorylated tau at the depths of sulci. We sought to determine the presence of CTE pathology in a brain bank for neurodegenerative disorders for individuals with and without a history of contact sports participation. Available medical records of 1721 men were reviewed for evidence of past history of injury or participation in contact sports. Subsequently, cerebral cortical samples were processed for tau immunohistochemistry in cases with a documented history of sports exposure as well as age- and disease-matched men and women without such exposure. For cases with available frozen tissue, genetic analysis was performed for variants in APOE, MAPT, and TMEM106B. Immunohistochemistry revealed 21 of 66 former athletes had cortical tau pathology consistent with CTE. CTE pathology was not detected in 198 individuals without exposure to contact sports, including 33 individuals with documented single-incident TBI sustained from falls, motor vehicle accidents, domestic violence, or assaults. Among those exposed to contact sports, those with CTE pathology did not differ from those without CTE pathology with respect to noted clinicopathologic features. There were no significant differences in genetic variants for those with CTE pathology, but we observed a slight increase in MAPT H1 haplotype, and there tended to be fewer homozygous carriers of the protective TMEM106B rs3173615 minor allele in those with sports exposure and CTE pathology compared to those without CTE pathology. In conclusion, this study has identified a small, yet significant, subset of individuals with neurodegenerative disorders and concomitant CTE pathology. CTE pathology was only detected in individuals with documented participation in contact sports. Exposure to contact sports was the greatest risk factor for CTE pathology. Future

  18. Thalamus lesions in chronic and acute seizure disorders

    Energy Technology Data Exchange (ETDEWEB)

    Tschampa, Henriette J.; Greschus, Susanne; Urbach, Horst [University of Bonn, Department of Radiology (Neuroradiology), Bonn (Germany); Sassen, Robert; Bien, Christian G. [University of Bonn, Department of Epileptology, Bonn (Germany)

    2011-04-15

    Transient signal changes in the pulvinar have been described following status epilepticus. However, we observed persistent thalamus changes after seizures. The purpose of this study was to characterize thalamus changes in patients with seizure disorders and to correlate imaging findings with clinical features. We searched among 5,500 magnetic resonance imaging (MRI) exams performed in patients with seizures and identified 43 patients. The MRI scans of these patients were reviewed and correlated with clinical data. We identified four patterns of thalamus lesions: (a) fluid attenuated inversion recovery-hyperintense pulvinar lesions (20 patients), as known from status epilepticus. Ten patients in this group had a status epilepticus. Among the remaining patients, three had frequent seizures and seven had sporadic seizures. Twelve patients had follow-up exams for a median of 11 months. The lesions had persisted in 11/12 cases in the last available exam and were reversible in one case only. In seven cases, cone-shaped thalamus atrophy resulted, (b) linear defects in the medial and anterior thalamus (five patients), accompanied by atrophy of the mamillary body and the fornix in patients with chronic epilepsy, (c) extensive bilateral thalamus lesions in two patients with a syndrome caused by mutation in the mitochondrial polymerase gamma, and (d) other thalamus lesions not associated with the seizure disorder (16 patients). The spectrum of thalamus lesions in patients with seizure disorders is wider than previously reported. Postictal pulvinar lesions can persist and may result in thalamic atrophy. Linear defects in the anterior thalamus are associated with limbic system atrophy. (orig.)

  19. Thalamus lesions in chronic and acute seizure disorders

    International Nuclear Information System (INIS)

    Transient signal changes in the pulvinar have been described following status epilepticus. However, we observed persistent thalamus changes after seizures. The purpose of this study was to characterize thalamus changes in patients with seizure disorders and to correlate imaging findings with clinical features. We searched among 5,500 magnetic resonance imaging (MRI) exams performed in patients with seizures and identified 43 patients. The MRI scans of these patients were reviewed and correlated with clinical data. We identified four patterns of thalamus lesions: (a) fluid attenuated inversion recovery-hyperintense pulvinar lesions (20 patients), as known from status epilepticus. Ten patients in this group had a status epilepticus. Among the remaining patients, three had frequent seizures and seven had sporadic seizures. Twelve patients had follow-up exams for a median of 11 months. The lesions had persisted in 11/12 cases in the last available exam and were reversible in one case only. In seven cases, cone-shaped thalamus atrophy resulted, (b) linear defects in the medial and anterior thalamus (five patients), accompanied by atrophy of the mamillary body and the fornix in patients with chronic epilepsy, (c) extensive bilateral thalamus lesions in two patients with a syndrome caused by mutation in the mitochondrial polymerase gamma, and (d) other thalamus lesions not associated with the seizure disorder (16 patients). The spectrum of thalamus lesions in patients with seizure disorders is wider than previously reported. Postictal pulvinar lesions can persist and may result in thalamic atrophy. Linear defects in the anterior thalamus are associated with limbic system atrophy. (orig.)

  20. Clinicopathological features of transient myeloproliferative syndrome and congenital leukaemia

    International Nuclear Information System (INIS)

    The objectives of the study were to determine the spectrum of the clinical and pathological findings, the management and prognosis of patients of transient myeloproliferative syndrome (TMS) and congenital leukaemia. Study Design: Case series. Place and Duration of Study: The study was conducted over a period of 8 years, from January 2000 to December 2007, at the Children's Hospital and the Institute of Child Health, Lahore. Methodology: Suspected patients presenting with fever, pallor, bruises and hepatosplenomegaly and diagnosed as either transient myeloproliferative disorder or congenital leukaemia were studied. The complete blood count, reticulocyte count, leukocyte alkaline phosphatase score, liver function tests, karyotyping studies and bone marrow aspiration biopsy were performed in all of those patients. Management and out come was noted. Results were described as frequency percentages. Results: Out of 10,000 patients presenting during this period, 24 patients were diagnosed as either of transient myeloproliferative syndrome or congenital leukaemia. Fifteen of these were diagnosed as patients of TMS and 9 as patients of congenital leukaemia. Down syndrome (DS) was diagnosed in 75% of these patients. TMS patients were put on supportive treatment and recovered spontaneously. One DS patient with congenital leukaemia went into spontaneous remission and 2 of DS patients with congenital leukaemia responded to chemotherapy while rest of them either died or lost to follow-up. Conclusion: TMS and congenital leukaemia were not very uncommon in the studied population. Majority had Down syndrome. It is important to differentiate their clinical and pathological presentations for proper management. TMS may resolve with supportive treatment while congenital leukaemia is a fatal condition requiring chemotherapy. (author)

  1. The predictive value of the Sokal and Hasford scoring systems in chronic myeloid leukaemia in the imatinib era

    OpenAIRE

    Oyekunle, Anthony A.; Patrick O Osho; John C Aneke; Lateef Salawu; Muheez Alani Durosinmi

    2012-01-01

    Objectives: Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder characterized by the presence of Philadelphia chromosome (Ph) or BCR-ABL1 chimeric gene; which codes for an abnormal tyrosine kinase responsible for the malignant proliferation of myeloid cells. Imatinib mesylate, a selective inhibitor of this kinase, is the first choice of therapy in patients with CML. The study aimed to determine the effect of imatinib on the survival of different risk groups based on the Sok...

  2. Therapeutic approaches in myelofibrosis and myelodysplastic/myeloproliferative overlap syndromes

    Directory of Open Access Journals (Sweden)

    Sochacki AL

    2016-04-01

    Full Text Available Andrew L Sochacki,1 Melissa A Fischer,1 Michael R Savona1,2 1Department of Internal Medicine, Vanderbilt University Medical Center, 2Vanderbilt-Ingram Cancer Center, Nashville, TN, USA Abstract: The discovery of JAK2V617F a decade ago led to optimism for a rapidly developing treatment revolution in Ph- myeloproliferative neoplasms. Unlike BCR–ABL, however, JAK2 was found to have a more heterogeneous role in carcinogenesis. Therefore, for years, development of new therapies was slow, despite standard treatment options that did not address the overwhelming symptom burden in patients with primary myelofibrosis (MF, post-essential thrombocythemia MF, post-polycythemia vera MF, and myelodysplastic syndrome (MDS/myeloproliferative neoplasm (MPN syndromes. JAK–STAT inhibitors have changed this, drastically ameliorating symptoms and ultimately beginning to show evidence of impact on survival. Now, the genetic foundations of myelofibrosis and MDS/MPN are rapidly being elucidated and contributing to targeted therapy development. This has been empowered through updated response criteria for MDS/MPN and refined prognostic scoring systems in these diseases. The aim of this article is to summarize concisely the current and rationally designed investigational therapeutics directed at JAK–STAT, hedgehog, PI3K–Akt, bone marrow fibrosis, telomerase, and rogue epigenetic signaling. The revolution in immunotherapy and novel treatments aimed at previously untargeted signaling pathways provides hope for considerable advancement in therapy options for those with chronic myeloid disease. Keywords: MDS/MPN neoplasms, emerging therapy

  3. Therapeutic approaches in myelofibrosis and myelodysplastic/myeloproliferative overlap syndromes

    Science.gov (United States)

    Sochacki, Andrew L; Fischer, Melissa A; Savona, Michael R

    2016-01-01

    The discovery of JAK2V617F a decade ago led to optimism for a rapidly developing treatment revolution in Ph− myeloproliferative neoplasms. Unlike BCR–ABL, however, JAK2 was found to have a more heterogeneous role in carcinogenesis. Therefore, for years, development of new therapies was slow, despite standard treatment options that did not address the overwhelming symptom burden in patients with primary myelofibrosis (MF), post-essential thrombocythemia MF, post-polycythemia vera MF, and myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) syndromes. JAK–STAT inhibitors have changed this, drastically ameliorating symptoms and ultimately beginning to show evidence of impact on survival. Now, the genetic foundations of myelofibrosis and MDS/MPN are rapidly being elucidated and contributing to targeted therapy development. This has been empowered through updated response criteria for MDS/MPN and refined prognostic scoring systems in these diseases. The aim of this article is to summarize concisely the current and rationally designed investigational therapeutics directed at JAK–STAT, hedgehog, PI3K–Akt, bone marrow fibrosis, telomerase, and rogue epigenetic signaling. The revolution in immunotherapy and novel treatments aimed at previously untargeted signaling pathways provides hope for considerable advancement in therapy options for those with chronic myeloid disease. PMID:27143923

  4. Family Health and Characteristics in Chronic Fatigue Syndrome, Juvenile Rheumatoid Arthritis, and Emotional Disorders of Childhood.

    Science.gov (United States)

    Rangel, Luiza; Garralda, M. Elena; Jeffs, Jim; Rose, Gillian

    2005-01-01

    Objective: To compare family health and characteristics in children with chronic fatigue syndrome (CFS), in juvenile rheumatoid arthritis (JRA), and emotional disorders. Method: Parents of 28 children and adolescents aged 11 to 18 years with CFS, 30 with JRA, and 27 with emotional disorders (i.e., anxiety and/or depressive disorders) were…

  5. Chronic obstructive lung disease and posttraumatic stress disorder: current perspectives

    Directory of Open Access Journals (Sweden)

    Abrams TE

    2015-10-01

    Full Text Available Thad E Abrams,1,2 Amy Blevins,1,3 Mark W Vander Weg1,2,4 1Department of Internal Medicine, University of Iowa, 2Center for Comprehensive Access and Delivery Research and Evaluation, Iowa City VA Health Care System, 3Hardin Health Sciences Library, 4Department of Psychological and Brain Sciences, University of Iowa, Iowa City, IA, USA Background: Several studies have reported on the co-occurrence of chronic obstructive pulmonary disease (COPD and psychiatric conditions, with the most robust evidence base demonstrating an impact of comorbid anxiety and depression on COPD-related outcomes. In recent years, research has sought to determine if there is a co-occurrence between COPD and posttraumatic stress disorder (PTSD as well as for associations between PTSD and COPD-related outcomes. To date, there have been no published reviews summarizing this emerging literature.Objectives: The primary objective of this review was to determine if there is adequate evidence to support a co-occurrence between PTSD and COPD. Secondary objectives were to: 1 determine if there are important clinical considerations regarding the impact of PTSD on COPD management, and 2 identify targeted areas for further research.Methods: A structured review was performed using a systematic search strategy limited to studies in English, addressing adults, and to articles that examined: 1 the co-occurrence of COPD and PTSD and 2 the impact of PTSD on COPD-related outcomes. To be included, articles must have addressed some type of nonreversible obstructive lung pathology.Results: A total of 598 articles were identified for initial review. Upon applying the inclusion and exclusion criteria, n=19 articles or abstracts addressed our stated objectives. Overall, there is inconclusive evidence to support the co-occurrence between PTSD and COPD. Studies finding a significant co-occurrence generally had inferior methods of identifying COPD; in contrast, studies that utilized more robust COPD

  6. Calreticulin Mutations in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Noa Lavi

    2014-10-01

    Full Text Available With the discovery of the JAK2V617F mutation in patients with Philadelphia chromosome-negative (Ph− myeloproliferative neoplasms (MPNs in 2005, major advances have been made in the diagnosis of MPNs, in understanding of their pathogenesis involving the JAK/STAT pathway, and finally in the development of novel therapies targeting this pathway. Nevertheless, it remains unknown which mutations exist in approximately one-third of patients with non-mutated JAK2 or MPL essential thrombocythemia (ET and primary myelofibrosis (PMF. At the end of 2013, two studies identified recurrent mutations in the gene encoding calreticulin (CALR using whole-exome sequencing. These mutations were revealed in the majority of ET and PMF patients with non-mutated JAK2 or MPL but not in polycythemia vera patients. Somatic 52-bp deletions (type 1 mutations and recurrent 5-bp insertions (type 2 mutations in exon 9 of the CALR gene (the last exon encoding the C-terminal amino acids of the protein calreticulin were detected and found always to generate frameshift mutations. All detected mutant calreticulin proteins shared a novel amino acid sequence at the C-terminal. Mutations in CALR are acquired early in the clonal history of the disease, and they cause activation of JAK/STAT signaling. The CALR mutations are the second most frequent mutations in Ph− MPN patients after the JAK2V617F mutation, and their detection has significantly improved the diagnostic approach for ET and PMF. The characteristics of the CALR mutations as well as their diagnostic, clinical, and pathogenesis implications are discussed in this review.

  7. [Chronic B-cell lymphoproliferative disorders with hairy cells].

    Science.gov (United States)

    Troussard, Xavier; Cornet, Édouard

    2015-01-01

    The standardized blood smear examination is the first step in the diagnosis of a B-cell chronic lymphoproliferative disorder and can guide further investigations. In the laboratory, the identification of hairy cells on blood smear is a matter of daily practice. Hairy cell proliferations represent heterogeneous entities and their respective diagnoses can be difficult. If hairy cell leukemia (HCL) and splenic marginal zone lymphoma (SMZL) represent separate entities, the variant form of HCL (HCLv) and splenic diffuse red pulp small B-cell lymphoma (SDRPL) remain provisional entities in the 2008 WHO classification. We discuss the main clinical and biological characteristics of these four entities and appropriate means to characterize, identify and distinguish from each other; standardized blood smear examination, multiparameter flow cytometry analysis, analysis of the repertoire of immunoglobulins heavy chains genes and their mutational status (mutated or unmutated profile), molecular analyses: BRAF gene V600E mutation in HCL and MAP2K1 gene mutations in HCLv. We also discuss the main therapeutic aspects with emphasis on the new targeted drugs that enter into force in the therapeutic arsenal. PMID:25858127

  8. No development of neutralizing antibodies against recombinant interferon-alpha in Ph-negative myeloproliferative neoplasms-a prospective study

    DEFF Research Database (Denmark)

    Ocias, Lukas Frans; Lund Hansen, Dennis; Kielsgaard Kristensen, Thomas;

    2015-01-01

    Background Treatment of Philadelphia chromosome negative chronic myeloproliferative neoplasms (MPNs) with recombinant pegylated interferon alpha2a/b (rIFN-alpha) has proven effective. It is well known that prolonged therapy with recombinant type 1 interferons (IFN-alpha and IFN-beta) may induce...

  9. Myeloproliferative neoplasms in five multiple sclerosis patients

    DEFF Research Database (Denmark)

    Thorsteinsdottir, Sigrun; Bjerrum, Ole Weis

    2013-01-01

    The concurrence of myeloproliferative neoplasms (MPNs) and multiple sclerosis (MS) is unusual. We report five patients from a localized geographic area in Denmark with both MS and MPN; all the patients were diagnosed with MPNs in the years 2007-2012. We describe the patients' history and treatment...

  10. Atherosclerotic cardiovascular disease in patients with chronic inflammatory joint disorders.

    Science.gov (United States)

    Agca, R; Heslinga, S C; van Halm, V P; Nurmohamed, M T

    2016-05-15

    Inflammatory joint disorders (IJD), including rheumatoid arthritis (RA), ankylosing spondylitis (ASp) and psoriatic arthritis (PsA), are prevalent conditions worldwide with a considerable burden on healthcare systems. IJD are associated with increased cardiovascular (CV) disease-related morbidity and mortality. In this review, we present an overview of the literature. Standardised mortality ratios are increased in IJD compared with the general population, that is, RA 1.3-2.3, ASp 1.6-1.9 and PsA 0.8-1.6. This premature mortality is mainly caused by atherosclerotic events. In RA, this CV risk is comparable to that in type 2 diabetes. Traditional CV risk factors are more often present and partially a consequence of changes in physical function related to the underlying IJD. Also, chronic systemic inflammation itself is an independent CV risk factor. Optimal control of disease activity with conventional synthetic, targeted synthetic and biological disease-modifying antirheumatic drugs decreases this excess risk. High-grade inflammation as well as anti-inflammatory treatment alter traditional CV risk factors, such as lipids. In view of the above-mentioned CV burden in patients with IJD, CV risk management is necessary. Presently, this CV risk management is still lacking in usual care. Patients, general practitioners, cardiologists, internists and rheumatologists need to be aware of the substantially increased CV risk in IJD and should make a combined effort to timely initiate CV risk management in accordance with prevailing guidelines together with optimal control of rheumatic disease activity. CV screening and treatment strategies need to be implemented in usual care. PMID:26888573

  11. When the Brakes are Lost: LNK Dysfunction in Mice, Men, and Myeloproliferative Neoplasms

    OpenAIRE

    Oh, Stephen T.

    2011-01-01

    Aberrant JAK-STAT signaling is a hallmark of myeloproliferative neoplasms (MPNs). These hyperproliferative disorders are classically associated with activating mutations in tyrosine kinases such as JAK2 and the thrombopoietin (TPO) receptor MPL. Activation of JAK-STAT signaling and responses to JAK2 inhibitors have been observed in MPN patients lacking JAK2 or MPL mutations, suggesting that other regulatory elements in the JAK-STAT pathway are altered. However, the molecular basis for this ob...

  12. Psychopathological Profile in Children with Chronic Tic Disorder and Co-Existing ADHD: Additive Effects

    Science.gov (United States)

    Roessner, Veit; Becker, Andreas; Banaschewski, Tobias; Rothenberger, Aribert

    2007-01-01

    The nature of the co-occurrence of chronic tic disorders (CTD) and attention deficit hyperactivity disorder (ADHD) is unclear. Especially in the field of psychopathology, the relationship of CTD and ADHD remains to be clarified. Thus, the aim of the present chart review study was to specify the contribution of CTD and/or ADHD to the…

  13. Association of Mental Disorders With Subsequent Chronic Physical Conditions World Mental Health Surveys From 17 Countries

    NARCIS (Netherlands)

    Scott, Kate M.; Lim, Carmen; Al-Hamzawi, Ali; Alonso, Jordi; Bruffaerts, Ronny; Caldas-de-Almeida, Jose Miguel; Florescu, Silvia; de Girolamo, Giovanni; Hu, Chiyi; de Jonge, Peter; Kawakami, Norito; Elena Medina-Mora, Maria; Moskalewicz, Jacek; Navarro-Mateu, Fernando; O'Neill, Siobhan; Piazza, Marina; Posada-Villa, Jose; Torres, Yolanda; Kessler, Ronald C.

    2016-01-01

    IMPORTANCE It is clear that mental disorders in treatment settings are associated with a higher incidence of chronic physical conditions, but whether this is true of mental disorders in the community, and how generalized (across a range of physical health outcomes) these associations are, is less cl

  14. Prenatal Maternal Smoking and Increased Risk for Tourette Syndrome and Chronic Tic Disorders

    DEFF Research Database (Denmark)

    Browne, Heidi A; Modabbernia, Amirhossein; Buxbaum, Joseph D;

    2016-01-01

    OBJECTIVE: We assessed the role of prenatal maternal smoking in risk for Tourette syndrome and chronic tic disorder (TS/CT) and pediatric-onset obsessive-compulsive disorder (OCD). METHOD: In an analysis of 73,073 singleton pregnancies from the Danish National Birth Cohort, we calculated incidence...

  15. Methemoglobinemia in Young Patients With Hematologic Cancer or Aplastic Anemia Treated With Dapsone

    Science.gov (United States)

    2010-11-04

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Methemoglobinemia; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nonmalignant Neoplasm

  16. Baclofen-Amitriptyline Hydrochloride-Ketamine Gel in Treating Peripheral Neuropathy Caused by Chemotherapy in Patients With Cancer

    Science.gov (United States)

    2015-07-03

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neurotoxicity; Pain; Unspecified Adult Solid Tumor, Protocol Specific

  17. Portal Hypertension and Myeloproliferative Neoplasms: A Relationship Revealed

    OpenAIRE

    Ahmet Burak Toros; Serkan Gokcay; Guven Cetin; Muhlis Cem Ar; Yesim Karagoz; Besir Kesici

    2013-01-01

    Background/Objectives. Patients with myeloproliferative neoplasms have a well-established increased risk of thrombosis. Many trials report identification of an underlying myeloproliferative neoplasm by investigation of the patients developing portal hypertensive esophagus and/or fundus variceal hemorrhage in the absence of any known etiology. This trial was designed to investigate the association between myeloproliferative neoplasms and portal hypertension and to detect the frequency of porta...

  18. Molecular biology of Philadelphia-negative myeloproliferative neoplasms

    OpenAIRE

    Paulo Vidal Campregher; Fábio Pires de Souza Santos; Guilherme Fleury Perini; Nelson Hamerschlak

    2012-01-01

    Myeloproliferative neoplasms are clonal diseases of hematopoietic stem cells characterized by myeloid hyperplasia and increased risk of developing acute myeloid leukemia. Myeloproliferative neoplasms are caused, as any other malignancy, by genetic defects that culminate in the neoplastic phenotype. In the past six years, since the identification of JAK2V617F, we have experienced a substantial increase in our knowledge about the genetic mechanisms involved in the genesis of myeloproliferative ...

  19. Impaired Functional Connectivity in the Prefrontal Cortex: A Mechanism for Chronic Stress-Induced Neuropsychiatric Disorders

    Directory of Open Access Journals (Sweden)

    Ignacio Negrón-Oyarzo

    2016-01-01

    Full Text Available Chronic stress-related psychiatric diseases, such as major depression, posttraumatic stress disorder, and schizophrenia, are characterized by a maladaptive organization of behavioral responses that strongly affect the well-being of patients. Current evidence suggests that a functional impairment of the prefrontal cortex (PFC is implicated in the pathophysiology of these diseases. Therefore, chronic stress may impair PFC functions required for the adaptive orchestration of behavioral responses. In the present review, we integrate evidence obtained from cognitive neuroscience with neurophysiological research with animal models, to put forward a hypothesis that addresses stress-induced behavioral dysfunctions observed in stress-related neuropsychiatric disorders. We propose that chronic stress impairs mechanisms involved in neuronal functional connectivity in the PFC that are required for the formation of adaptive representations for the execution of adaptive behavioral responses. These considerations could be particularly relevant for understanding the pathophysiology of chronic stress-related neuropsychiatric disorders.

  20. Chronic pain disorders in HIV primary care: clinical characteristics and association with healthcare utilization.

    Science.gov (United States)

    Jiao, Jocelyn M; So, Eric; Jebakumar, Jebakaran; George, Mary Catherine; Simpson, David M; Robinson-Papp, Jessica

    2016-04-01

    Chronic pain is common in HIV, but incompletely characterized, including its underlying etiologies, its effect on healthcare utilization, and the characteristics of affected patients in the HIV primary care setting. These data are needed to design and justify appropriate clinic-based pain management services. Using a clinical data warehouse, we analyzed one year of data from 638 patients receiving standard-of-care antiretroviral therapy in a large primary care HIV clinic, located in the Harlem neighborhood of New York City. We found that 40% of patients carried one or more chronic pain diagnoses. The most common diagnoses were degenerative musculoskeletal disorders (eg, degenerative spinal disease and osteoarthritis), followed by neuropathic pain and headache disorders. Many patients (16%) had multiple chronic pain diagnoses. Women, older patients, and patients with greater burdens of medical illness, and psychiatric and substance use comorbidities were disproportionately represented among those with chronic pain diagnoses. Controlling for overall health status, HIV patients with chronic pain had greater healthcare utilization including emergency department visits and radiology procedures. In summary, our study demonstrates the high prevalence of chronic pain disorders in the primary care HIV clinic. Colocated interventions for chronic pain in this setting should not only focus on musculoskeletal pain but also account for complex multifaceted pain syndromes, and address the unique biopsychosocial features of this population. Furthermore, because chronic pain is prevalent in HIV and associated with increased healthcare utilization, developing clinic-based pain management programs could be cost-effective. PMID:26683238

  1. An International MDS/MPN Working Group’s perspective and recommendations on molecular pathogenesis, diagnosis and clinical characterization of myelodysplastic/myeloproliferative neoplasms

    Science.gov (United States)

    Mughal, Tariq I.; Cross, Nicholas C.P.; Padron, Eric; Tiu, Ramon V.; Savona, Michael; Malcovati, Luca; Tibes, Raoul; Komrokji, Rami S.; Kiladjian, Jean-Jacques; Garcia-Manero, Guillermo; Orazi, Attilio; Mesa, Ruben; Maciejewski, Jaroslaw P.; Fenaux, Pierre; Itzykson, Raphael; Mufti, Ghulam; Solary, Eric; List, Alan F.

    2015-01-01

    In the 2008 WHO classification, chronic myeloid malignancies that share both myelodysplastic and myeloproliferative features define the myelodysplastic/myeloproliferative group, which includes chronic myelomonocytic leukemia, juvenile myelomonocytic leukemia, atypical chronic myeloid leukemia, refractory anemia with ring sideroblasts and thrombocytosis, and myelodysplastic/myeloproliferative unclassified. With the notable exception of refractory anemia with ring sideroblasts and thrombocytosis, there is much overlap among the various subtypes at the molecular and clinical levels, and a better definition of these entities, an understanding of their biology and an identification of subtype-specific molecular or cellular markers are needed. To address some of these challenges, a panel comprised of laboratory and clinical experts in myelodysplastic/myeloproliferative was established, and four independent academic MDS/MPN workshops were held on: 9th March 2013, in Miami, Florida, USA; 6th December 2013, in New Orleans, Louisiana, USA; 13th June 2014 in Milan, Italy; and 5th December 2014 in San Francisco, USA. During these meetings, the current understanding of these malignancies and matters of biology, diagnosis and management were discussed. This perspective and the recommendations on molecular pathogenesis, diagnosis and clinical characterization for adult onset myelodysplastic/myeloproliferative is the result of a collaborative project endorsed and supported by the MDS Foundation. PMID:26341525

  2. Myeloproliferative neoplasms: A decade of discoveries and treatment advances.

    Science.gov (United States)

    Tefferi, Ayalew

    2016-01-01

    Myeloproliferative neoplasms (MPN) are clonal stem cell diseases, first conceptualized in 1951 by William Dameshek, and historically included chronic myeloid leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). In 1960, Nowell and Hungerford discovered an invariable association between the Philadelphia chromosome (subsequently shown to harbor the causal BCR-ABL1 mutation) and CML; accordingly, the term MPN is primarily reserved for PV, ET, and PMF, although it includes other related clinicopathologic entities, according to the World Health Organization (WHO) classification system. In 2005, William Vainchenker and others described a Janus kinase 2 mutation (JAK2V617F) in MPN and this was followed by a series of additional descriptions of mutations that directly or indirectly activate JAK-STAT: JAK2 exon 12, myeloproliferative leukemia virus oncogene (MPL) and calreticulin (CALR) mutations. The discovery of these, mostly mutually exclusive, "driver" mutations has contributed to revisions of the WHO diagnostic criteria and risk stratification in MPN. Mutations other than JAK2, CALR and MPL have also been described in MPN and shown to provide additional prognostic information. From the standpoint of treatment, over the last 50 years, Louis Wasserman from the Unites States and Tiziano Barbui from Italy had skillfully organized and led a number of important clinical trials, whose results form the basis for current treatment strategies in MPN. More recently, allogeneic stem cell transplant, as a potentially curative treatment modality, and JAK inhibitors, as palliative drugs, have been added to the overall therapeutic armamentarium in myelofibrosis. In the current review, I will summarize the important advances made in the last 10 years regarding the science and practice of MPN. PMID:26492355

  3. Disability and borderline personality disorder in chronic pain patients

    OpenAIRE

    Sansone, Randy A.; J David Sinclair; Wiederman, Michael W.

    2010-01-01

    BACKGROUND AND OBJECTIVE: Few studies have examined the relationship between disability and borderline personality symptomatology, and, among those that have, findings have been inconsistent. In the present study, the relationship between medical disability and borderline personality symptomatology was examined in a sample of chronic pain patients.METHODS: In a consecutive insured sample of male and female chronic pain patients (n=117), who were being initially evaluated by an outpatient pain...

  4. Chronic pain as a variant of depressive disease: the pain-prone disorder.

    Science.gov (United States)

    Blumer, D; Heilbronn, M

    1982-07-01

    Review of the literature shows that the common syndrome of chronic pain of uncertain origin appears to be perpetuated by central mechanisms. No plausible neurological theory has been proposed. While the alternative concept of chronic pain as a psychogenic disorder has remained a vague entity, there is strong support to view chronic pain as the prime expression of a muted depressive state. This form of masked depression, however, tends to be associated with a number of characteristic traits. Our studies of patients with chronic pain have led to the identification of a well defined psychobiological disorder with characteristic clinical, psychodynamic, biographic, and genetic features. This syndrome is termed the pain-prone disorder and is viewed as a variant of depressive disease. It proves a distinct entity when compared with a group of patients whose pain can be related to a well defined somatic disease. The chronicity of the disorder appears partially related to the practice of protracted, costly, and futile physical procedures, focusing on a phantom peripheral source of the pain-- a practice commonly pursued by patients and physicians. Recognition of the disorder allows for early, rational, and more effective treatment approaches. PMID:7086394

  5. Addressing Neuroplastic Changes in Distributed Areas of the Nervous System Associated With Chronic Musculoskeletal Disorders.

    Science.gov (United States)

    Pelletier, René; Higgins, Johanne; Bourbonnais, Daniel

    2015-11-01

    Present interventions utilized in musculoskeletal rehabilitation are guided, in large part, by a biomedical model where peripheral structural injury is believed to be the sole driver of the disorder. There are, however, neurophysiological changes across different areas of the peripheral and central nervous systems, including peripheral receptors, dorsal horn of the spinal cord, brain stem, sensorimotor cortical areas, and the mesolimbic and prefrontal areas associated with chronic musculoskeletal disorders, including chronic low back pain, osteoarthritis, and tendon injuries. These neurophysiological changes appear not only to be a consequence of peripheral structural injury but also to play a part in the pathophysiology of chronic musculoskeletal disorders. Neurophysiological changes are consistent with a biopsychosocial formulation reflecting the underlying mechanisms associated with sensory and motor findings, psychological traits, and perceptual changes associated with chronic musculoskeletal conditions. These changes, therefore, have important implications in the clinical manifestation, pathophysiology, and treatment of chronic musculoskeletal disorders. Musculoskeletal rehabilitation professionals have at their disposal tools to address these neuroplastic changes, including top-down cognitive-based interventions (eg, education, cognitive-behavioral therapy, mindfulness meditation, motor imagery) and bottom-up physical interventions (eg, motor learning, peripheral sensory stimulation, manual therapy) that induce neuroplastic changes across distributed areas of the nervous system and affect outcomes in patients with chronic musculoskeletal disorders. Furthermore, novel approaches such as the use of transcranial direct current stimulation and repetitive transcranial magnetic stimulation may be utilized to help renormalize neurological function. Comprehensive treatment addressing peripheral structural injury as well as neurophysiological changes occurring across

  6. Depression in chronic respiratory disorders in a tertiary rural hospital of Central India

    Institute of Scientific and Technical Information of China (English)

    Sameer singhal; Pankaj Banode; Nitish Baisakhiya

    2009-01-01

    Objective: To determine prevalence of depression in chronic respiratory disorders in a tertiary rural hospital of Central India. Various studies done in past have shown that prevalence of depression in diabetes and hypertension is around 40%-57%. Few studies have been done to screen depression in chronic respiratory disorders. This study was conducted in a tertiary rural hospital of Central India to find out prevalence of depression in indoor patients suffering from chronic respiratory disorders. Methods: Total 68 patients were evaluated for depression. Patients suffering from chronic respiratory disorders (total duration of illness >3 months) were evaluated using Prime MD Questionnaire. Patients suffering from diabetes, heart diseases, stroke, having past history of psychiatric illness, drug abusers, having lack of social support and suffering from chronic upper respiratory tract infections were excluded from this study. Questionnaire was asked when treatment for acute phase of illness is over. Results: Out of 68 patients evaluated, 36 (53%) were found out to be suffering from depression. Female gender (80%) was more prone to depression, inspite of the fact that all alcoholics were male. 39% of all chronic obstructive pulmonary disease (COPD) patients were suffering from depression in comparison to 65% for pulmonary tuberculosis and 44% for other chronic respiratory illness. 54% of patients suffering from depression are 60 yrs of age, suggesting that age has no relation with depression. No association was seen between alcoholism and depression. Conclusion: Prevalence of depression in patients of chronic respiratory illness is very high, like in cases of diabetes and hypertension. Further community and hospital based studies are needed to find out exact prevalence of depression in chronic respiratory illnesses.

  7. Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2

    NARCIS (Netherlands)

    Nangalia, J.; Massie, C.E.; Baxter, E.J.; Nice, F.L.; Gundem, G.; Wedge, D.C.; Avezov, E.; Li, J.; Kollmann, K.; Kent, D.G.; Aziz, A.; Godfrey, A.L.; Hinton, J.; Martincorena, I.; Loo, P. Van; Jones, A.V.; Guglielmelli, P.; Tarpey, P.; Harding, H.P.; Fitzpatrick, J.D.; Goudie, C.T.; Ortmann, C.A.; Loughran, S.J.; Raine, K.; Jones, D.R.; Butler, A.P.; Teague, J.W.; O'Meara, S.; McLaren, S.; Bianchi, M.; Silber, Y.; Dimitropoulou, D.; Bloxham, D.; Mudie, L.; Maddison, M.; Robinson, B.; Keohane, C.; Maclean, C.; Hill, K.; Orchard, K.; Tauro, S.; Du, M.Q.; Greaves, M.; Bowen, D.; Huntly, B.J.; Harrison, C.N.; Cross, N.C.; Ron, D.; Vannucchi, A.M.; Papaemmanuil, E.; Campbell, P.J.; Green, A.R.

    2013-01-01

    BACKGROUND: Somatic mutations in the Janus kinase 2 gene (JAK2) occur in many myeloproliferative neoplasms, but the molecular pathogenesis of myeloproliferative neoplasms with nonmutated JAK2 is obscure, and the diagnosis of these neoplasms remains a challenge. METHODS: We performed exome sequencing

  8. Anxiety and depressive disorders in elderly with chronic dizziness of vestibular origin

    Directory of Open Access Journals (Sweden)

    Érica Toledo Piza Peluso

    2016-04-01

    Full Text Available ABSTRACT INTRODUCTION: Dizziness is one of the most prevalent symptoms in the elderly. Anxiety and depression are common in dizzy adult patients, but there is scarce information about comorbidity between vestibular disturbances and psychiatric disorders in the aged. OBJECTIVE: To assess the prevalence of anxiety and depression disorders in elderly with chronic dizziness of vestibular origin. METHODS: Transversal study that used the Brazilian version of the Composite International Diagnostic Interview 2.1 to assess anxiety and depressive disorders in elderly patients (≥60 years old with chronic dizziness. RESULTS: Most of the 44 patients included in the study were female (88.6% with a mean age of 71 years (±7.5, 68.1% had experienced dizziness for 1 year or more. The most prevalent diagnosis was benign paroxysmal positional vertigo (52.3%. The prevalence of generalized anxiety disorder and specific phobias during life were 29.5% and 22.7%, respectively, and, in the last 12 months, 18.2% and 15.9%. There was no patient with panic disorder, agoraphobia or social phobia. The prevalence of depressive disorder during life was 45.4%, and, in the last 12 months, were 11.3%. CONCLUSION: Aged patients with chronic dizziness had high prevalence of some mental disorders.

  9. Impaired Functional Connectivity in the Prefrontal Cortex: A Mechanism for Chronic Stress-Induced Neuropsychiatric Disorders

    OpenAIRE

    Ignacio Negrón-Oyarzo; Francisco Aboitiz; Pablo Fuentealba

    2016-01-01

    Chronic stress-related psychiatric diseases, such as major depression, posttraumatic stress disorder, and schizophrenia, are characterized by a maladaptive organization of behavioral responses that strongly affect the well-being of patients. Current evidence suggests that a functional impairment of the prefrontal cortex (PFC) is implicated in the pathophysiology of these diseases. Therefore, chronic stress may impair PFC functions required for the adaptive orchestration of behavioral response...

  10. The prevalence of Pneumocystis jirovecii among patients with different chronic pulmonary disorders in Ahvaz, Iran

    OpenAIRE

    Elham Aboualigalehdari; Ali Zarei Mahmoudabadi; Mahnaz Fatahinia; Esmaeil Idani

    2016-01-01

    Background and Objectives: Pneumocystis jirovecii pneumonia (PJP) is a chronic fungal infection that caused by P. ji- rovecii. Disease is more prevalent among the HIV-infected patients. The colonization of pneumocystis in human respiratory system is associated with the airway inflammation and obstruction. The current study was conducted to identify the preva- lence of P. jirovecii among the patients with chronic pulmonary disorders in Ahvaz, Iran.Materials and Methods: In the present study, 1...

  11. The Relationship Between Chronic Inflammation and Glucidic-Lipidic Profile Disorders in Kidney Transplant Recipients

    OpenAIRE

    Tarța I.D.; Căldăraru Carmen Denise; Gliga Mirela; Huțanu Adina; Bajko Z; Carașca E; Dogaru G.A.

    2016-01-01

    Introduction: Chronic inflammation has a proven role in atherogenesis, lipid profile parameters being related to cytokine production. In kidney transplant recipients, interleukin 6 (IL-6) is significantly associated with graft-related outcomes and also alterations of cholesterol and triglyceride metabolism. The aim of this study was to investigate the relationship between chronic inflammation and glucidic-lipidic metabolism disorders in a group of patients with kidney transplantation as renal...

  12. Chronic Kidney Disease-Mineral Bone Disorder in the Elderly Peritoneal Dialysis Patient

    DEFF Research Database (Denmark)

    Heaf, James Goya

    2015-01-01

    PURPOSE: The purpose of this paper was to review the literature concerning the treatment of chronic kidney disease-mineral bone disorder (CKD-MBD) in the elderly peritoneal dialysis (PD) patient. ♦ RESULTS: Chronic kidney disease-mineral bone disorder is a major problem in the elderly PD patient......, with its associated increased fracture risk, vascular calcification, and accelerated mortality fracture risk. Peritoneal dialysis, however, bears a lower risk than hemodialysis (HD). The approach to CKD-MBD prophylaxis and treatment in the elderly PD patient is similar to other CKD patients, with some...... to other dialysis patient groups, physicians should be aware of the special problems of the elderly group....

  13. Anxiety and depressive disorders in elderly with chronic dizziness of vestibular origin

    OpenAIRE

    Érica Toledo Piza Peluso; Maria Inês Quintana; Fernando Freitas Ganança

    2016-01-01

    ABSTRACT INTRODUCTION: Dizziness is one of the most prevalent symptoms in the elderly. Anxiety and depression are common in dizzy adult patients, but there is scarce information about comorbidity between vestibular disturbances and psychiatric disorders in the aged. OBJECTIVE: To assess the prevalence of anxiety and depression disorders in elderly with chronic dizziness of vestibular origin. METHODS: Transversal study that used the Brazilian version of the Composite International Diagnosti...

  14. Myeloproliferative neoplasms: Morphology and clinical practice.

    Science.gov (United States)

    Barbui, Tiziano; Thiele, Jürgen; Vannucchi, Alessandro M; Tefferi, Ayalew

    2016-06-01

    In myeloproliferative neoplasms (MPNs), controversy persists regarding the usefulness and reproducibility of bone marrow (BM) features. Disagreements concerning the WHO classification are mainly focused on the discrimination between essential thrombocythemia (ET) and prefibrotic/early primary myelofibrosis (prePMF) and prodromal polycythemia vera (PV). Criticism mostly refers to lack of standardization of distinctive BM features precluding correct morphological pattern recognition. The distinction between WHO-defined ET and prePMF is not trivial because outcome is significantly worse in prePMF. Morphology was generally considered to be non-specific for the diagnosis of PV. Recent studies have revealed under-diagnosis of morphologically and biologically consistent PV. PMID:26718907

  15. Chronic Granulomatous Disease: Lessons from a Rare Disorder

    OpenAIRE

    Segal, B H; Veys, P.; Malech, H; Cowan, M J

    2011-01-01

    Chronic granulomatous disease (CGD) is a rare primary immunodeficiency with x-linked or autosomal recessive inheritance involving defects in genes encoding phox proteins which are the subunits of the phagocyte NADPH oxidase. This results in failure to produce superoxide anion and downstream antimicrobial oxidant metabolites and to activate antimicrobial proteases. Affected patients are susceptible to severe, life-threatening bacterial and fungal infections and excessive inflammation character...

  16. LIPID METABOLISM DISORDERS IN PATIENTS WITH CHRONIC HEPATITIS C

    Directory of Open Access Journals (Sweden)

    L. I. Tkachenko

    2015-01-01

    Full Text Available Purpose of the study. To study lipid metabolism in chronic hepatitis C and to assess its impact on the formation of insulin resistance, steatosis and progression of liver fibrosis.Materials and methods. The study included 205 patients with chronic hepatitis C (CHC. Conducts research, depending on the genotype C, viral load and body mass index (BMI of the patients.Results. CHC patients revealed a combined hyperlipoproteinemia on the background of op-pression synthesis of apolipoproteins A1 and B. Formation of hepatic steatosis was associated with HCV genotype 3 virus-induced viral load at ≥ 6 log10 IU/ml and metabolic in VL < 6 log10 IU/ml. In patients with chronic hepatitis C genotype 1, high viral load leads to inhibition of protein synthesis conveyor ApoA1 and increased synthesis of cholesterol, accompanied by abdominal obesity and the formation of insulin resistance. CHC patients with BMI < 25 kg/m2 viral load ≥ 6 log10 ME/ml was associated with dyslipidemia IV type on D. Fredriskson (1970, hyperglycemia, insulin resistance and diabetes. The advanced stage of liver fi brosis (F ≥ 3 on a scale METAVIR and non-response to treatment were associated with a decrease in HDL cholesterol below normal. With an increase in viral load > 5 log10 ME/ml signifi cantly increased the risk of lipid and carbohydrate metabolism.

  17. Prevalence of chronic musculoskeletal disorders in elderly Brazilians: a systematic review of the literature

    Directory of Open Access Journals (Sweden)

    Miranda Vivian S

    2012-05-01

    Full Text Available Abstract Background Population ageing is a worldwide phenomenon that has recently challenged public healthcare systems. The knowledge of the burden of chronic musculoskeletal disorders in elders is still limited, particularly in the developing world. This systematic review aimed to investigate the prevalence of chronic musculoskeletal disorders in elderly Brazilians. Methods A comprehensive literature search was performed in five electronic databases (from inception to January 2012 and completed by additional searches in reference lists. Two review authors independently selected the eligible studies and extracted data on participants’ characteristics and rates of chronic musculoskeletal disorders. One review author extracted methodological quality data. We performed a critical synthesis of the results, which were grouped into the diagnoses “chronic musculoskeletal pain” or “specific musculoskeletal diagnoses”. Results Twenty five studies reporting on a total of 116,091 elderly Brazilians were included. Eight studies (32% were of high methodological quality. There was a large variation in the measure of prevalence used by individual studies and in their definition of chronic pain. Prevalence estimates reached 86% for chronic musculoskeletal pain in any location. Studies investigating multiple pain sites found the lower limb and the spine to be the most prevalent complaints (50% each. Arthritis and rheumatism (including osteoarthritis were the most prevalent specific musculoskeletal diagnoses (9% to 40%, followed by herniated disc (6% to 27%. Conclusions Despite the growth of the elderly population worldwide, high-quality research on the burden of chronic musculoskeletal disorders in the elderly is still scarce. Future healthcare research focusing on this age group should be a priority in developing countries since their public healthcare systems are not yet fully prepared to accommodate the needs of an aging population.

  18. MMPI-2 F Scale as a Predictor of Acute Versus Chronic Disorder Classification

    Science.gov (United States)

    Cukrowicz, Kelly C.; Reardon, Maureen Lyons; Donohue, Keith F.; Joiner, Jr., Thomas E.

    2004-01-01

    This study examined the relation between elevation of the infrequency (F) scale on the Minnesota Multiphasic Personality Inventory-2 (MMPI-2) and the classification of psychological disorders as chronic or acute in an outpatient mental health setting. MMPI-2 and clinician rating data at time of intake were considered for 158 adult patients from an…

  19. Exploring the Impact of Chronic Tic Disorders on Youth: Results from the Tourette Syndrome Impact Survey

    Science.gov (United States)

    Conelea, Christine A.; Woods, Douglas W.; Zinner, Samuel H.; Budman, Cathy; Murphy, Tanya; Scahill, Lawrence D.; Compton, Scott N.; Walkup, John

    2011-01-01

    Prior research has demonstrated that chronic tic disorders (CTD) are associated with functional impairment across several domains. However, methodological limitations, such as data acquired by parental report, datasets aggregated across child and adult samples, and small treatment-seeking samples, curtail interpretation. The current study explored…

  20. Symptom Differences in Acute and Chronic Presentation of Childhood Post-Traumatic Stress Disorder.

    Science.gov (United States)

    Famularo, Richard; And Others

    1990-01-01

    Twenty-four child abuse victims, age 5-13, were diagnosed with posttraumatic stress disorder (PTSD). Children with the acute form of PTSD exhibited such symptoms as difficulty falling asleep, hypervigilance, nightmares, and generalized anxiety. Children exhibiting chronic PTSD exhibited increased detachment, restricted range of affect,…

  1. Treating co-occurring chronic low back pain & generalized anxiety disorder.

    Science.gov (United States)

    Janzen, Kristina; Peters-Watral, Brenda

    2016-01-16

    The complex, bidirectional correlation between chronic low back pain (CLBP) and generalized anxiety disorder (GAD), common ailments in primary care, can increase the risk of inadequate treatment. This article will review the relationship between CLBP and GAD and provide optimal management strategies for NPs caring for individuals with this dyad. PMID:26642348

  2. Chronic obstructive lung disease and posttraumatic stress disorder: current perspectives

    OpenAIRE

    Abrams TE; Blevins A; Vander Weg MW

    2015-01-01

    Thad E Abrams,1,2 Amy Blevins,1,3 Mark W Vander Weg1,2,4 1Department of Internal Medicine, University of Iowa, 2Center for Comprehensive Access and Delivery Research and Evaluation, Iowa City VA Health Care System, 3Hardin Health Sciences Library, 4Department of Psychological and Brain Sciences, University of Iowa, Iowa City, IA, USA Background: Several studies have reported on the co-occurrence of chronic obstructive pulmonary disease (COPD) and psychiatric conditions, with the most robust...

  3. Myeloprolipherative disorder type chronic myeloid leukemia--eosinophilic form.

    Science.gov (United States)

    Arnautovic-Custovic, Aida; Hasic, Samira; Kopic, Emina; Jahic, Azra; Jovic, Svetlana

    2011-01-01

    Chronic eosinophilic leukemia (CEL) is a very rare form of leucemia in the western world. Adequate response is seldomly achieved after treatment with corticosteroids, interferon-alfa (INF-alfa) and medications containing hydroxi-urea (Litalir). The study presents a patient with CEL with no initial therapeutic response to the use of corticosteroids, INF-alfa and hydroxy-urea, and with neither clinical nor hematological response. After setting a diagnosis of CEL, patient was ordinated Imatinib (Glivec tabbletes) in a daily dose of 200 mg. Two days afterwards there was an evident withdrawal of subjective and clinical symptoms of disease, and the complete blood count showed significant amendment. PMID:21776882

  4. mTOR inhibitors alone and in combination with JAK2 inhibitors effectively inhibit cells of myeloproliferative neoplasms.

    OpenAIRE

    Bogani C; Bartalucci N; Martinelli S; Tozzi L; Guglielmelli P; Bosi A; Vannucchi AM; Associazione Italiana per la Ricerca sul Cancro AGIMM Gruppo Italiano Malattie Mieloproliferative

    2013-01-01

    Background Dysregulated signaling of the JAK/STAT pathway is a common feature of chronic myeloproliferative neoplasms (MPN), usually associated with JAK2V617F mutation. Recent clinical trials with JAK2 inhibitors showed significant improvements in splenomegaly and constitutional symptoms in patients with myelofibrosis but meaningful molecular responses were not documented. Accordingly, there remains a need for exploring new treatment strategies of MPN. A potential additional target for treatm...

  5. Myeloproliferative neoplasms: From JAK2 mutations discovery to JAK2 inhibitor therapies

    OpenAIRE

    Passamonti, Francesco; Maffioli, Margherita; Caramazza, Domenica; Cazzola, Mario

    2011-01-01

    Most BCR-ABL1-negative myeloproliferative neoplasms (MPN) carry an activating JAK2 mutation. Approximately 96% of patients with polycythemia vera (PV) harbors the V617F mutation in JAK2 exon 14, whereas the minority of JAK2 (V617F)-negative subjects shows several mutations in exon 12. Other mutation events as MPL, TET2, LNK, EZH2 have been described in chronic phase, while NF1, IDH1, IDH2, ASX1, CBL and Ikaros in blast phase of MPN. The specific pathogenic implication of these mutations is un...

  6. Behavioral disorders in rats with chronic myocardial dysfunction

    Directory of Open Access Journals (Sweden)

    Rodinskiy A.G.

    2014-09-01

    Full Text Available Observation of cardiologists and psychiatrists suggest that cardiovascular diseases and depression are the most common and frequent comorbid conditions. Therefore the aim of the study was to identify behavioral disorders in rats on the background of myocardial dysfunction in the experiment. Material of the research: 60 adult male rats of Wistar line, weighing 200-220 g, divided into 2 groups - control and experimental. Myocardial dysfunction was modeled by intraperitoneal injection of doxorubicin by 5 mg/kg of animal weight. To verify the model of myocardial dysfunction ECG and histological examination of the myocardium were performed. Evaluation of higher divisions of the CNS reactions was performed using the "open field" method by such indicators: vertical and horizontal motor activity (decreased by 71,4%, acts and defecation boluses (down by 85-88%, the number of burrows (decreased by 57-90%, grooming (decreased by 23%, all parameters were reliable (in p<0,05. There was a gradual decline of rats’ emotionality, CNS depression and increased anxiety. It was found that behavioral disorders in the experiment were typical for anxiety and depression states.

  7. Combination Chemotherapy and Donor Stem Cell Transplant in Treating Patients With Aplastic Anemia or Hematologic Cancer

    Science.gov (United States)

    2016-05-04

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Diseases; Nonmalignant Neoplasm; Unspecified Adult Solid Tumor, Protocol Specific; Unspecified Childhood Solid Tumor, Protocol Specific

  8. Donor Stem Cell Transplant or Donor White Blood Cell Infusions in Treating Patients With Hematologic Cancer

    Science.gov (United States)

    2012-07-02

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Unusual Cancers of Childhood

  9. Lactobacillus in Preventing Infection in Patients Undergoing a Donor Stem Cell Transplant for Hematologic Cancer or Myelodysplastic Syndrome

    Science.gov (United States)

    2015-03-18

    Breast Cancer; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  10. A Standardized Nursing Intervention Protocol for HCT Patients

    Science.gov (United States)

    2015-06-03

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Psychosocial Effects of Cancer and Its Treatment; Therapy-related Toxicity

  11. Cytokine Regulation of Microenvironmental Cells in Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Gregor Hoermann

    2015-01-01

    Full Text Available The term myeloproliferative neoplasms (MPN refers to a heterogeneous group of diseases including not only polycythemia vera (PV, essential thrombocythemia (ET, and primary myelofibrosis (PMF, but also chronic myeloid leukemia (CML, and systemic mastocytosis (SM. Despite the clinical and biological differences between these diseases, common pathophysiological mechanisms have been identified in MPN. First, aberrant tyrosine kinase signaling due to somatic mutations in certain driver genes is common to these MPN. Second, alterations of the bone marrow microenvironment are found in all MPN types and have been implicated in the pathogenesis of the diseases. Finally, elevated levels of proinflammatory and microenvironment-regulating cytokines are commonly found in all MPN-variants. In this paper, we review the effects of MPN-related oncogenes on cytokine expression and release and describe common as well as distinct pathogenetic mechanisms underlying microenvironmental changes in various MPN. Furthermore, targeting of the microenvironment in MPN is discussed. Such novel therapies may enhance the efficacy and may overcome resistance to established tyrosine kinase inhibitor treatment in these patients. Nevertheless, additional basic studies on the complex interplay of neoplastic and stromal cells are required in order to optimize targeting strategies and to translate these concepts into clinical application.

  12. JAK2 mutants (e.g., JAK2V617F) and their importance as drug targets in myeloproliferative neoplasms

    OpenAIRE

    Gäbler, Karoline; Behrmann, Iris; Haan, Claude

    2013-01-01

    The Janus kinase 2 (JAK2) mutant V617F and other JAK mutants are found in patients with myeloproliferative neoplasms and leukemias. Due to their involvement in neoplasia and inflammatory disorders, Janus kinases are promising targets for kinase inhibitor therapy. Several small-molecule compounds are evaluated in clinical trials for myelofibrosis, and ruxolitinib (INCB018424, Jakafi®) was the first Janus kinase inhibitor to receive clinical approval. In this review we provide an overview of JA...

  13. Cognitive Disorders, Depressive Status and Chronic Complications of Type 2 Diabetes Mellitus

    Directory of Open Access Journals (Sweden)

    Tache Mirela

    2014-12-01

    Full Text Available Background and aims: Depression and cognitive disorders were reported more frequently in patients with diabetes mellitus (DM. Our aim was to analyze the association of cognitive disorders and depression association with chronic complications of DM in a group of Romanian patients. Materials and methods: The data was analyzed from 181 patients, with a mean age of 58,3 years to whom we applied the MMSE (Mini- Mental State Examination and MADRS (Montgomery-Asberg Depression Rating Scale questionnaires. We also analyzed the presence of chronic DM complications, HbA1c and lipid profile. Results: Most patients with type 2 diabetes mellitus (T2DM had mild cognitive impairment (92%, more common in the age group 50-59 years. Chronic macrovascular complications were present in 74.58%, while chronic microvascular complications were present in 61.87% of patients with T2DM who associated mild and moderate cognitive impairment (p = 0.013. The most common form of depression was mild depression (90.2%, present in most patients with DM, regardless of progression and type of treatment. MADRS depression test scores were statistically significant correlated with the presence of peripheral artery disease - PAD (p <0.001, ischemic heart disease - IHD (p <0.001 and chronic kidney disease - CKD (p =0.05. We did not find a statistically significant correlation with HbA1c and serum lipid values (p˃0,05. Conclusion: Chronic diabetes macrovascular complications (PAD, IHD and CKD were more frequently associated with cognitive disorders and depression in patients with T2DM independent of the degree of metabolic control.

  14. Status og perspektiver for behandling af de kroniske myeloproliferative neoplasier

    DEFF Research Database (Denmark)

    Ocias, Lukas Frans; Holmström, Morten Orebo; Riley, Caroline Hasselbalch;

    2015-01-01

    Polycythaemia vera, essential thrombocytosis and primary myelofibrosis are closely related, clonal myeloproliferative neoplasms. Our knowledge of the underlying molecular mechanisms driving these diseases has increased dramatically during the latest ten years. Traditionally, treatment of these...

  15. Severity of Anxiety Disorders in Patients with Chronic Obstructive Pulmonary Disease

    Directory of Open Access Journals (Sweden)

    Mitra Safa

    2015-10-01

    Full Text Available Objective: Patients with chronic physical diseases sometimes show increased loss of function; such patients need more care. Anxiety is a well-known symptom that is prevalent among chronic obstructive pulmonary disease patients that can prolong and increase the risk of hospitalization. The purpose of this study was to evaluate the severity of anxiety in the mentioned patients and to examine the presence of symptoms and appropriate treatment strategies to understand the role of psychological functions in physical patients.Methods: This was a cross sectional study conducted in Masih Daneshvari Hospital. One hundred forty- three patients entered into the project by accessible method and signed the informed consent; they filled demographic information and Hamilton anxiety and depression questionnaires. Data were analyzed by SPSS-16 .Results: Of the participants, 68% were above 60 years of age; 78% were male; 89% were married; and 38% were self-employed. Also, among the participants, 51% were illiterate; 72% had history of smoking; 46% had history of substance abuse; and 49% had moderate to severe anxiety disorder. Moreover, of the patients with severe anxiety, 41.3% had severe muscle spasms; and severe sleeplessness was found in 38.5% of those with severe anxiety disorder. Severe anxiety related symptoms were found in 20.3% of the patients with severe anxiety disorder. Depressed mood was found in 27.3% of the patients with severe anxiety disorder. Severe physical and muscular signs were found in 35.7% of those with severe anxiety disorder .Conclusion: According to our findings, many chronic diseases such as chronic obstructive pulmonary disease may contain anxiety and depression which result in vulnerability. Therefore, evaluation of anxiety in such patients is of importance for alleviating the disease.

  16. Defining the Thrombotic Risk in Patients with Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Fabrizio Vianello

    2011-01-01

    Full Text Available Polycythemia vera (PV and essential thrombocythemia (ET are two Philadelphia-negative myeloproliferative neoplasms (MPN associated with an acquired mutation in the JAK2 tyrosine kinase gene. There is a rare incidence of progression to myelofibrosis and myeloid metaplasia in both disorders, which may or may not precede transformation to acute myeloid leukemia, but thrombosis is the main cause of morbidity and mortality. The pathophysiology of thrombosis in patients with MPN is complex. Traditionally, abnormalities of platelet number and function have been claimed as the main players, but increased dynamic interactions between platelets, leukocytes, and the endothelium do probably represent a fundamental interplay in generating a thrombophilic state. In addition, endothelial dysfunction, a well-known risk factor for vascular disease, may play a role in the thrombotic risk of patients with PV and ET. The identification of plasma markers translating the hemostatic imbalance in patients with PV and ET would be extremely helpful in order to define the subgroup of patients with a significant clinical risk of thrombosis.

  17. Impact of Inflammation on Myeloproliferative Neoplasm Symptom Development

    Directory of Open Access Journals (Sweden)

    Holly L. Geyer

    2015-01-01

    Full Text Available Myeloproliferative neoplasms (essential thrombocythemia, ET; polycythemia vera, PV; myelofibrosis, MF are monoclonal malignancies associated with genomic instability, dysregulated signaling pathways, and subsequent overproduction of inflammatory markers. Acknowledged for their debilitating symptom profiles, recent investigations have aimed to determine the identity of these markers, the upstream sources stimulating their development, their prevalence within the MPN population, and the role they play in symptom development. Creation of dedicated Patient Reported Outcome (PRO tools, in combination with expanded access to cytokine analysis technology, has resulted in a surge of investigations evaluating the potential associations between symptoms and inflammation. Emerging data demonstrates clear relationships between individual MPN symptoms (fatigue, abdominal complaints, microvascular symptoms, and constitutional symptoms and cytokines, particularly IL-1, IL-6, IL-8, and TNF-α. Information is also compiling on the role symptoms paradoxically play in the development of cytokines, as in the case of fatigue-driven sedentary lifestyles. In this paper, we explore the symptoms inherent to the MPN disorders and the potential role inflammation plays in their development.

  18. Clinical features associated with an early onset in chronic tic disorders.

    Science.gov (United States)

    Richer, Francois; Daghfal, Roula; Rouleau, Guy A; Lespérance, Paul; Chouinard, Sylvain

    2015-12-30

    In chronic tic disorders such as Tourette syndrome (TS), tics often appear between 4 and 8 years but they can also appear in early childhood, a period in which symptom expression may be affected by early brain development. The present study examined whether symptom expression in early-onset TS was distinct from that observed in TS with a later onset. We compared the clinical characteristics in children with TS who developed tics before age 4 or after age 6. Early-onset TS was significantly associated with an increased rate of stuttering and other speech disfluencies as well as an increased rate of oppositional defiant disorder, symptoms that often appear before age 4. Early-onset TS was also linked to maternal transmission of tics. Early-onset TS was not significantly associated with tic severity, obsessive-compulsive behavior or attention-deficit hyperactivity disorder. The results suggest that an early onset affects symptom expression in tic disorders. PMID:26596364

  19. Sleep disordered breathing in patients with chronic obstructive pulmonary disease.

    Science.gov (United States)

    Aoki, Takuya; Akinori, Ebihara; Yogo, Yurika; Sakamaki, Fumio; Suzuki, Yukio; Suemasu, Keiichi

    2005-06-01

    Sleep-related disordered breathing (SDB) and its influence on desaturation were examined in stable COPD patients with waking SpO2 > 90%. With respiratory inductance plethysmography, thoracic-abdominal respiratory movements for all events with more than 4% desaturation were analyzed in 26 patients. Types of SDB were confirmed by full polysomnography. Irregular breathing induced desaturation, while stable respiration continued during some desaturation events. Three types of altered ventilation were observed: hypoventilation, paradoxical movement and periodic breathing. An unusual type of paradoxical movement, with normal airflow despite progressive desaturation, was observed in REM sleep. Patients were divided into desaturation (15 patients) and non-desaturation (11 patients) groups. Daytime arterial blood gas, lung function values, and 6-min walking distance did not differ. Awake, mode, maximum and minimum nocturnal SpO2 were lower in the desaturation group. SDB-induced desaturation events in the desaturation group were more frequent (9.2+/-3.5 vs. 1.8+/-2.2 times), a greater SpO2 decrease (11.4+/-7.1% vs. 5.2+/-2.1%) and longer duration (73.2+/-34.8 vs. 18.8+/-39.0 min). Patterns of SDB in the desaturation group were hypoventilation (74.4+/-23.4%), paradoxical movement (10.2+/-14.5%), periodic breathing (12.1+/-18.3%) and unclassified (5.8+/-11.2%). These results reveal that lower SpO2 and SDB influence nocturnal desaturation in stable COPD patients. PMID:17136951

  20. Molecular biology of Philadelphia-negative myeloproliferative neoplasms

    Directory of Open Access Journals (Sweden)

    Paulo Vidal Campregher

    2012-01-01

    Full Text Available Myeloproliferative neoplasms are clonal diseases of hematopoietic stem cells characterized by myeloid hyperplasia and increased risk of developing acute myeloid leukemia. Myeloproliferative neoplasms are caused, as any other malignancy, by genetic defects that culminate in the neoplastic phenotype. In the past six years, since the identification of JAK2V617F, we have experienced a substantial increase in our knowledge about the genetic mechanisms involved in the genesis of myeloproliferative neoplasms. Mutations described in several genes have revealed a considerable degree of molecular homogeneity between different subtypes of myeloproliferative neoplasms. At the same time, the molecular differences between each subtype have become clearer. While mutations in several genes, such as JAK2, myeloproliferative leukemia (MPL and LNK have been validated in functional assays or animal models as causative mutations, the roles of other recurring mutations in the development of disease, such as TET2 and ASXL1 remain to be elucidated. In this review we will examine the most prevalent recurring gene mutations found in myeloproliferative neoplasms and their molecular consequences.

  1. Effectiveness of family training and life skills on caring chronic mental disorders in an Iranian population

    Directory of Open Access Journals (Sweden)

    Mohsen Dareke

    2013-05-01

    Full Text Available This research is semi-empirical study which is done to examiningthe effectiveness of family trainings and life skills on Caring Chronic Mental Disorders in Mashhad (Iran. 300 participants has been chosen in randomly from the families of chronic mental disorders who has been admitted in round the clock centers in Mashhad and who had been in waiting list and these participants has been divided to two groups of experiment and control randomly and we introduce family trainings and life skills to experiment group. There was no training to control group. These two groups were tested period to the beginning of trainings and post training with instruments such as California Social Adjustment Questionnaire; Families’ Attitude and Awareness about Life Skills and Family Trainings; Rosenberg Self-Esteem; General Health Questionnaire;and Symptom Checklist-90-Revised. Results indicated that training had no effect on the attitude of families toward taking care of mental disorder, but family member،s attitudetoward mental disorder has been changed. This training has been influential on changing family member،s attitude toward life skills, social adjustment, self esteem, mental health increase and reducing mental disorders.

  2. Atypical Chronic Myeloid Leukaemia with Trisomy 13: a Case Report

    Institute of Scientific and Technical Information of China (English)

    Guo-yu Hu; Chao-hui Yuan; Kui Tan; Zhen-zhen Chen

    2011-01-01

    ATYPICAL chronic myeloid leukaemia (aCML),which shows both myeloproliferative and myeIodysplastic features,is a type of myeloproliferative/myelodysplastic disease as defined by the World Health Organisation (WHO) classification of the myeloid neoplasms.1 Because of the presence of neutrophilic leukocytosis,aCML may resemble chronic myeIogenous leukemia (CML).However,in contrast with CML,aCML does not have the Philadelphia chromosome or the bcr/abl fusion gene.

  3. Sleep-disordered breathing and oxidative stress in preclinical chronic mountain sickness (excessive erythrocytosis)

    OpenAIRE

    Julian, Colleen Glyde; Vargas, Enrique; Gonzales, Marcelino; Dávila, R. Daniela; Ladenburger, Anne; Reardon, Lindsay; Schoo, Caroline; Powers, Robert W.; Lee-Chiong, Teofilo; Moore, Lorna G.

    2013-01-01

    Chronic mountain sickness (CMS) is considered to be a loss of ventilatory acclimatization to high altitude (>2500 m) resulting in marked arterial hypoxemia and polycythemia. This case-control study explores the possibility that sleep-disordered breathing (SBD) and associated oxidative stress contribute to the etiology of CMS. Nocturnal respiratory and SaO2 patterns were measured using standard polysomnography techniques and compared between male high-altitude residents (aged 18–25) with precl...

  4. The ecology of suffering: developmental disorders of structured stress, emotion, and chronic inflammation

    OpenAIRE

    Wallace, Rodrick

    2003-01-01

    'Punctuated equilibrium' models of cognitive process, adapted from the Large Deviations Program of probability theory, are applied to the interaction between immune function and emotion in the context of culturally structured psychosocial stress. The analysis suggests: (1) Chronic inflammatory diseases should be comorbid and synergistic with characteristic emotional dysfunction, and may form a collection of joint disorders most effectively treated at the individual level using multifa...

  5. Neurobiologically informed treatment for adults with anorexia nervosa: a novel approach to a chronic disorder

    OpenAIRE

    Knatz, Stephanie; Wierenga, Christina E.; Murray, Stuart B.; Hill, Laura; Kaye, Walter H.

    2015-01-01

    Anorexia nervosa (AN) is a severe and debilitating disorder with significant medical and psychological sequelae. To date, there are no effective treatments for adults, resulting in high rates of chronicity, morbidity, and mortality. Recent advances in brain imaging research have led to an improved understanding of etiology and specific neurobiological mechanisms underlying symptoms. Despite this, there are no treatments focused on targeting symptoms using this empirically supported mechanisti...

  6. Imaging Chronic Pain and Inflammation : Positron Emission Tomography Studies of Whiplash Associated Disorder

    OpenAIRE

    Linnman, Clas

    2008-01-01

    This thesis is on chronic neck pain after a rear impact car injury, so called whiplash associated disorder (WAD). Three empirical studies using positron emission tomography (PET) with different radioligands have been performed. The first study evaluated resting state regional cerebral blood flow (rCBF) in WAD patients and in healthy, pain-free controls, by use of oxygen-15 labeled water. Patients had heightened resting rCBF bilaterally in the posterior parahippocampal and the posterior cingul...

  7. Elevated [11C]-D-Deprenyl Uptake in Chronic Whiplash Associated Disorder Suggests Persistent Musculoskeletal Inflammation

    OpenAIRE

    Linnman, Clas; Appel, Lieuwe; Fredrikson, Mats; Gordh, Torsten; Söderlund, Anne; Långström, Bengt; Engler, Henry

    2011-01-01

    There are few diagnostic tools for chronic musculoskeletal pain as structural imaging methods seldom reveal pathological alterations. This is especially true for Whiplash Associated Disorder, for which physical signs of persistent injuries to the neck have yet to be established. Here, we sought to visualize inflammatory processes in the neck region by means Positron Emission Tomography using the tracer 11C-D-deprenyl, a potential marker for inflammation. Twenty-two patients with enduring pain...

  8. Personality Features and Personality Disorders in Chronic Fatigue Syndrome: A Population-Based Study

    OpenAIRE

    Nater, Urs M.; Jones, James F; Lin, Jin-Mann S.; Maloney, Elizabeth; Reeves, William C; Heim, Christine

    2010-01-01

    Background Chronic fatigue syndrome (CFS) presents unique diagnostic and management challenges. Personality may be a risk factor for CFS and may contribute to the maintenance of the illness. Methods 501 study participants were identified from the general population of Georgia: 113 people with CFS, 264 with unexplained unwellness but not CFS (insufficient fatigue, ISF) and 124 well controls. We used the Personality Diagnostic Questionnaire, 4th edition, to evaluate DSM-IV personality disorders...

  9. Prevalence of psychiatric disorders in sick listed chronic low back pain patients

    OpenAIRE

    Reme, Silje Endresen; Tangen, Tone; Moe, Trygve; Eriksen, Hege Randi

    2011-01-01

    Background: Previous findings have shown a high degree of comorbid psychopathology in chronic low back pain (CLBP), but less is known about the broad range of comorbid psychiatric disorders. The prevalence is reported to be between 40%-100% depending on methods being used, sample or setting. Aims: To assess the prevalence of psychiatric comorbidity in a population of CLBP patients, using a psychiatric diagnostic interview. Methods: 565 patients sick listed between 2 and 10 m...

  10. IMPACT OF CHRONIC POSTTRAUMATIC STRESS DISORDER ON THE QUALITY OF LIFE OF WAR SURVIVORS

    OpenAIRE

    Bravo-Mehmedbašić, Alma; Kučukalić, Abdulah; Džubur Kulenović, Alma; Suljić, Enra

    2010-01-01

    Background: Research data from studies of functional neuroanatomy and neurochemistry indicate various dysfunctions in certain areas of the brain in individuals who suffer from chronic Posttraumatic Stress Disorder. These abnormalities are involved in the evolution of symptoms of PTSD, deterioration of cognitive functions and decreased quality of life of the survivors. The intensity of these symptoms is in direct correlation with the degree of dysfunction in the central nervous system...

  11. Probiotic foods: Can their increasing use in India ameliorate the burden of chronic lifestyle disorders?

    Directory of Open Access Journals (Sweden)

    Neerja Hajela

    2014-01-01

    Full Text Available Probiotics are defined as live microorganisms which, when ingested in adequate amounts, confer health benefits on the host. Chronic diseases such as diabetes, non-alcoholic fatty liver disease, coronary artery disease, a variety of chronic inflammatory disorders with an immune basis, and some forms of cancer are increasing in incidence around the world and in India, and may be attributable in part to rapid changes in our lifestyle. There is considerable public interest in India in the consumption of probiotic foods. This brief review summarizes the background of the gut microbiota, the immunological reactions induced by these, the evidence linking the microbiota to health outcomes, and the evidence linking the use of probiotics for amelioration of chronic lifestyle diseases.

  12. [sup 123]I-OIH renoscintigraphy in children with chronic disorder of glomerulus

    Energy Technology Data Exchange (ETDEWEB)

    Okada, Junichi; Oonishi, Hiroshi; Sanayama, Kazunori; Fukumoto, Yasuhiko (Narita Red Cross Hospital, Chiba (Japan))

    1994-01-01

    To evaluate the usefulness of [sup 123]I-orthoiodohippurate (OIH) renography, 31 patients with chronic disorder of glomerulus, from 5 years to 15 years old, were studied. In chronic glomerulonephritis and nephrotic syndrome with resistance to steroid therapy, T[sub max] and T[sub 1]/[sub 2] of the time-activity curve were statistically prolonged. Most of the renograms (time-activity curves) of the patients showed the hypofunction pattern. In most of the patients, other laboratory data, including BUN, PSP test and creatine clearance, didn't show abnormal values. [sup 123]I-OIH scan was considered to be a sensitive and useful method to evaluate the renal function in pediatric patients with chronic glomerulonephritis. (author).

  13. Individualizing Opioid Use Disorder (OUD Treatment: Time to Fully Embrace a Chronic Disease Model

    Directory of Open Access Journals (Sweden)

    Richard Gustin

    2015-02-01

    Full Text Available The current opioid epidemic in the United States is changing our perceptions of the face of addiction. Opioid Use Disorder (OUD has become pervasive and is affecting all ethnicities, races, socioeconomic classes, the young and the old. In 2015, 46 people will lose their life each day to a chronic brain disease that is going unnoticed and undertreated. Over the last five decades, numerous scientific and clinical breakthroughs have allowed for a better understanding of the mechanisms underlying addiction, and the development of medications that can help support a patient’s long-term recovery. All of those that have contributed to these advancements have aided in redefining addiction as a primary, chronic disease of the brain reward, motivation, memory and related circuitry; however, our treatment strategies have not necessarily advanced to the same extent as our current understanding of the disease. This commentary will explore how personal philosophies can bias treatments strategies and definitions of treatment success, and prevent adoption of chronic disease treatment models that would significantly improve the quality of life of those suffering with OUD. This is a challenge to consider how our views and stigma can impact a patient’s recovery. We are currently losing a battle with a disease that is taking the lives of 46 individuals daily; it is time to fully embrace a chronic disease model which comprises an integrated pharmacopsychosocial approach for treating the biopsychosocial disorder that is addiction to reverse these trends.

  14. The high prevalence of obsessive-compulsive disorder in patients with chronic pain.

    Directory of Open Access Journals (Sweden)

    Ali Mehraban

    2014-12-01

    Full Text Available Chronic pain is a common disorder with a high prevalence of psychiatric disorder that imposes a worse prognosis on both conditions. Obsessive compulsive disorder (OCD is estimated to be the fourth most prevalent life time psychiatric disorder, but yet has gained less attention in chronic pain comorbidity researches.Ninety three heterogeneous chronic pain patients who attended a pain clinic in Tehran (Iran in an outpatient setting during three months were included in this study. Diagnosis was made by Structured Clinical Interview for DSM-IV (SCID.The mean age of the patients was 46.37 (SD 15.005 years; of the patients, 66.7% were female and 33.3% were male. The mean duration of pain was 34.43 (SD 51.422 months. The mean pain severity on numerical pain scale was 5.82 (SD 1.950 from 10. The mean pain site number was 3.68 (SD 3.401 from the maximum of 27 places. Furthermore, 61.3% of the participants were diagnosed with lifetime OCD, 25.8% with subclinical OCD and 61.5 % with major depressive disorder (MDD. OCD diagnosis was not correlated with MDD or pain intensity. Female gender was associated with OCD (OD; 4.182, 95% CL (1.655-10.568. Pain intensity was correlated with MDD (P < 0.05.The high prevalence of OCD was comorbidity, independent of MDD and most pain characteristics. The high prevalence of OCD may be explained by the high rate of undiagnosed cause of pain as well as cultural and local factors. Using screening tests is suggested for tackling under diagnosis and under treatment of OCD and MDD.

  15. ACUTE STRESS DISORDER VERSUS CHRONIC POSTTRAUMATIC STRESS DISORDER: INHIBITION OF FEAR AS A FUNCTION OF TIME SINCE TRAUMA

    Science.gov (United States)

    Jovanovic, Tanja; Sakoman, Andrea Jambrošić; Kozarić-Kovačić, Dragica; Meštrović, Ana Havelka; Duncan, Erica J.; Davis, Michael; Norrholm, Seth D.

    2013-01-01

    Background Previous work has shown that inhibition of fear is impaired in posttraumatic stress disorder (PTSD) resulting from both civilian and combat trauma. The purpose of the present study was to investigate the inhibition of learned fear in traumatized individuals diagnosed with either acute stress disorder (ASD) or PTSD. This is the first study to use a conditioned inhibition paradigm with traumatized individuals within a month of trauma exposure. We hypothesized that impaired fear inhibition would be evident in PTSD, but not ASD. Method Using established translational, psychophysiological methods including fear-potentiated startle, and skin conductance, we examined fear acquisition, stimulus discrimination, and the transfer of learned safety in a Croatian population with ASD or PTSD. This cross-sectional study included three age-matched groups: healthy nontrauma controls (n = 27), a group with chronic PTSD (10 or more years since trauma exposure, n = 24), and a group with ASD (30 days or less since trauma exposure, n = 27). Results The presence of trauma-related psychopathology, whether acute or chronic, was associated with an impaired ability to transfer learned safety based on fear-potentiated startle measures, while healthy control subjects showed significant fear inhibition in the presence of the safety cue compared to the danger cue, F(1,26) = 12.64, P = .001. Conclusions These data expand our previously observed findings of PTSD-associated fear inhibition deficits by demonstrating that trauma-related impairments in safety learning are evident within 30 days of trauma exposure. PMID:22907890

  16. Sleep disorders and its related risk factors in patients undergoing chronic peritoneal dialysis

    Institute of Scientific and Technical Information of China (English)

    Li Han; Li Xiaobei; Feng Sujuan; Zhang Guizhi; Wang Wei; Wang Shixiang

    2014-01-01

    Background The prevalence of sleep disorders has been shown to be high in patients with chronic dialysis patients and may contribute to impaired quality of life and higher mortality in this population.However,there are few data on the relationship of sleep disorders and their risk factors in chronic dialysis patients.The aim of this study was to evaluate the relationship of sleep disorders and their risk factors in chronic dialysis patients.Methods A total of 42 continuous ambulatory peritoneal dialysis (CAPD) patients were involved in this cross-sectional study.Sleep quality was assessed by the Pittsburgh Sleep Quality Index (PSQI).Restless legs syndrome (RLS) was diagnosed according to the criteria of the International Restless Legs Syndrome Study Group.And depression was assessed by Hamilton depression scale.General information and laboratory data were collected.Results The prevalence of sleep disorders was 47.6% in the CAPD patients.According to the PSQI,the 42 CAPD patients were divided into sleep disturbance group and non-sleep disorders group.There were no significant differences in age,gender,dialysis duration,hemoglobin,serum creatinine,urea nitrogen,β2-microglobulin,parathyroid hormone,calcium,and phosphorus between CAPD patients with sleep disorders and those without sleep disorders.But the level of serum albumin (AIb) in CAPD patients with sleep disorders was significantly lower than that in CAPD patients without sleep disorders (31.3±1.4 vs.34.3±3.7,t=3.603,P=0.001).And the prevalence of RLS and depression was significantly higher than that in CAPD patients without sleep disorders (RLS:11/22 vs.1/20,x2=10.395,P=0.001; depression:7/22 vs.1/20,x2=4.886,P=0.027).In CAPD patients with RLS,the prevalence of sleep disorders was significantly higher than that in CAPD patients without RLS (11/22 vs.11/30,x2=10.395,P=0.001).And in CAPD patients with depression,the prevalence of sleep disorders was significantly higher than that in CAPD patients without

  17. Treatment Options for Childhood Acute Myeloid Leukemia, Childhood Chronic Myelogenous Leukemia, Juvenile Myelomonocytic ...

    Science.gov (United States)

    ... time, MDS may become AML. Transient myeloproliferative disorder (TMD) is a type of MDS. This disorder of ... of life. Infants who have Down syndrome and TMD have an increased chance of developing AML before ...

  18. Investigations of disorders of motility of the esophagus in chronic diseases. 14

    International Nuclear Information System (INIS)

    Chronic diseases of the esophagus impair the transport function of this organ. Esophageal scintigraphy investigates the transport function under physiological circumstances. Various test meals are radioactively labelled and swallowed by the patient. depending on the cause of a transport disorder the investigation lasts only 60s up to half an hour. Parametric imaging techniques like the so called condensed pictures give information about extent and peristalsis of transport disorders. The determination of transit times and/or percent of residuum in the eso-phagus allows for quantitating functional disorders. The use of multiple swallow technique or of various tracers during one investigation leads to high sensitivity in the detection of esophageal transport disorders, even in an early stage of a chronic disease, like morphea or sclero-derma. The documentation of successful therapeutical interventions is possible, for example in achalasia. In obscure complaints or in thoracic pain without coronary heart disease esophageal scintigraphy may confirm or exclude a disease of the esophagus. (author). 40 refs.; 5 figs

  19. Internet-based adherence interventions for treatment of chronic disorders in adolescents

    Directory of Open Access Journals (Sweden)

    Bass AM

    2015-05-01

    Full Text Available Alexandria M Bass,1 Michael E Farhangian,1 Steven R Feldman1–3 1Center for Dermatology Research, Department of Dermatology, Wake Forest School of Medicine, Winston-Salem, NC, USA; 2Department of Pathology, Wake Forest School of Medicine, Winston-Salem, NC, USA; 3Department of Public Health Sciences, Wake Forest School of Medicine, Winston-Salem, NC, USA Background: Treatment adherence is a ubiquitous challenge in medicine, particularly in the adolescent population with chronic disorders. Web-based adherence interventions may be particularly useful in adolescents, due to their familiarity with and frequent use of the Internet. Objective: To review web-based interventions used to improve adherence to medication in adolescent patients with chronic disorders. Methods: A PubMed search was performed for full-text, English, clinical trials in adolescents using keywords “adherence” or “compliance”, “Internet” or “web”, and “treatment” from inception until November 2014. Articles were selected if they involved using the Internet to provide support to adolescents to help improve their adherence to treatment, excluding those focused on solely providing medical services through the Internet and articles focusing on preventative care, rather than treatment of an illness. Results: Fourteen studies were found concentrating on chronic adolescent disorders. Interventions included online surveys, physician chat lines, monitoring programs, and interactive programs. All interventions experienced either greater improvement in adherence or another disease control measure or no statistically significant difference compared with the control group (in-clinic visits. Limitations: Few clinical trials studying web-based interventions to improve adherence in adolescents were found. Due to not having one standard outcome measured in all of the studies, it was also difficult comparing the effectiveness of the interventions. Conclusion: Web

  20. Association between pre- and perinatal exposures and Tourette syndrome or chronic tic disorder in the ALSPAC cohort†

    OpenAIRE

    Mathews, Carol A; Scharf, Jeremiah M.; Miller, Laura L; Macdonald-Wallis, Corrie; Lawlor, Debbie A.; Ben-Shlomo, Yoav

    2014-01-01

    Background Tourette syndrome and chronic tic disorder are heritable but aetiologically complex. Although environment plays a role in their development, existing studies of non-genetic risk factors are inconsistent. Aims To examine the association between pre- and perinatal exposures and Tourette syndrome/chronic tic disorder in the Avon Longitudinal Study of Parents and Children (ALSPAC) prospective longitudinal pre-birth cohort. Method Relationships between exposures and Tourette syndrome/ch...

  1. Disease characteristics as determinants of the labour market position of adolescents and young adults with chronic digestive disorders.

    OpenAIRE

    Calsbeek, H.; Rijken, M.; Dekker, J.; van Berge Henegouwen, G P

    2006-01-01

    BACKGROUND: Job prospects can be problematic for young patients with chronic digestive disorders. OBJECTIVES: To compare the employment status and disease burden in young adult patients with several chronic digestive disorders with healthy controls, and to determine whether labour participation depends on disease characteristics, such as type of diagnosis and burden of disease. PARTICIPANTS: In total 622 patients categorized into five diagnostic groups--inflammatory bowel disease (IBD) (n=274...

  2. Gastrointestinal Disorders Associated with Common Variable Immune Deficiency (CVID) and Chronic Granulomatous Disease (CGD).

    Science.gov (United States)

    Uzzan, Mathieu; Ko, Huaibin M; Mehandru, Saurabh; Cunningham-Rundles, Charlotte

    2016-04-01

    Common variable immune deficiency (CVID) and chronic granulomatous disease (CGD) are two of the well-characterized primary immune deficiencies with distinct pathologic defects. While CVID is predominantly a disorder of the adaptive immune system, in CGD, innate immunity is impaired. In both syndromes, the clinical manifestations include an increased susceptibility to infections and a number of non-infectious, inflammatory conditions including systemic autoimmunity, as well as organ-specific pathology. Among the organ-associated disorders, gastrointestinal (GI) manifestations are one of the most intractable. As such, non-infectious inflammatory disorders of the GI tract are clinically challenging as they have protean manifestations, often resembling inflammatory bowel disease (IBD) or celiac disease, are notoriously difficult to treat, and hence are associated with significant morbidity and mortality. Therefore, assessing the pathogenesis and defining appropriate therapeutic approaches for GI disease in patients with CVID and CGD is imperative. PMID:26951230

  3. Differences in maladaptive schemas between patients suffering from chronic and acute posttraumatic stress disorder and healthy controls

    OpenAIRE

    Ahmadian, Alireza; Mirzaee, Jafar; Omidbeygi, Maryam; Holsboer-Trachsler, Edith; Brand, Serge

    2015-01-01

    Background War, as a stressor event, has a variety of acute and chronic negative consequences, such as posttraumatic stress disorder (PTSD). In this context, early maladaptive schema-based problems in PTSD have recently become an important research area. The aim of this study was to assess early maladaptive schemas in patients with acute and chronic PTSD. Method Using available sampling methods and diagnostic criteria, 30 patients with chronic PTSD, 30 patients with acute PTSD, and 30 normal ...

  4. [Myeloproliferative neoplasms: pathophysiology and therapeutic strategy].

    Science.gov (United States)

    Kubuki, Yoko; Hidaka, Tomonori; Shimoda, Kazuya

    2015-10-01

    Myeloproliferative neoplasms (MPNs) arise from hematopoietic stem cells (HSCs) with genetic abnormalities in combination with mutations in JAK2, MPL or CALR, which induce autosomal JAK-STAT pathway activation, and mutations in epigenetic regulator genes such as TET2 or DNMT3A. The prognosis of patients with polycythemia vera (PV) or essential thrombocythemia (ET) is relatively good, and the therapeutic goal in cases with PV or ET is to prevent thrombohemorrhagic complications. PV or ET patients at least 60 years of age or with a history of thrombosis are in a high-risk category, and are managed with low dose aspirin and cytoreductive therapy. Phlebotomy to maintain Ht65 years, Hb<10 g/dl, the presence of constitutional symptoms, and the presence of blasts in blood were identified as being associated with shorter survival in MF patients. Those patients in the high-risk category are candidates for allogenic HSC transplantation (allo-HSCT), which is potentially curative but is also associated with higher therapy-related mortality. High-risk MF patients without indications for allo-HSCT are treated with JAK inhibitors, which can markedly ameliorate constitutional symptoms and splenomegaly, and might thereby lead to a degree of improvement in survival. PMID:26458438

  5. Family-Based Cognitive-Behavioral Treatment of Chronic Pediatric Headache and Anxiety Disorders: A Case Study

    Science.gov (United States)

    Drake, Kelly L.; Ginsburg, Golda S.

    2012-01-01

    Background: Chronic pediatric headache disorders are pervasive, debilitating, and associated with high rates of comorbid anxiety disorders. The combination of headaches and anxiety presents unique challenges for clinicians. Cognitive behavioral therapy (CBT) is a promising treatment for pediatric headache, however, available treatments fail to…

  6. The Efficacy of Supervised Home-based Pulmonary Rehabilitation in Patients with Chronic Respiratory Disorders

    Directory of Open Access Journals (Sweden)

    İpek Candemir

    2015-12-01

    Full Text Available Objective: Pulmonary rehabilitation (PR programs can be carried out in hospital or home basis with a different organizational aspect and program content. This study aimed to evaluate the efficacy of a multidisciplinary supervised home-based PR program in patients with chronic respiratory disorders. Methods: Forty patients with chronic respiratory disorders who admitted to our center between September 2007 and May 2012 were enrolled. In all patients before and after PR, dyspnea was assessed with Medical Research Council (MRC dyspnea scale, exercise capacity with Incremental Shuttle Walk Test (ISWT and Endurance Shuttle Walk Test (ESWT, health related quality of life with St. Geoerge Respiratory Questionnaire (SGRQ, psychosocial evaluation with hospital anxiety and depression scale (HAD, the body composition with bioelectrical impedance method. Ten patients did not complete home-based PR for various reasons. Results: In patients with Chronic Obstructive Pulmonary Disease (COPD, dyspnea sensation (p=0.026, exercise capacity (p=0.001, quality of life (p=0.001, body composition (p=0.012, anxiety and depression score (p=0.001 improvements were statistically significant. In all patients with COPD and non-COPD perception of dyspnea, exercise capacity, quality of life, anxiety and depression score improvements were above minimal clinically important differences. Conclusion: In this study supervised home-based pulmonary rehabilitation has been shown as an effective and safe modality when applied by an experiencied and multidisciplinary team in selected severe COPD or non-COPD patients.

  7. Improved cognitive, affective and anxiety measures in patients with chronic systemic disorders following structured physical activity.

    Science.gov (United States)

    Teixeira, Robson Bonoto; Marins, João Carlos Bouzas; de Sá Junior, Antonio Reis; de Carvalho, Cristiane Junqueira; da Silva Moura, Tiago Augusto; Lade, Carlos Gabriel; Rizvanov, Albert A; Kiyasov, Andrey P; Mukhamedyarov, Marat A; Zefirov, Andrey L; Palotás, András; Lima, Luciana Moreira

    2015-11-01

    Mental illnesses are frequent co-morbid conditions in chronic systemic diseases. High incidences of depression, anxiety and cognitive impairment complicate cardiovascular and metabolic disorders such as hypertension and diabetes mellitus. Lifestyle changes including regular exercise have been advocated to reduce blood pressure and improve glycaemic control. The purpose of this project was to evaluate the effect of physical training on the most prevalent corollary psychiatric problems in patients with chronic organic ailments. This longitudinal study assessed the mental health of hypertensive (age: 57 ± 8 years) and/or diabetic (age: 53 ± 8 years) patients using mini-mental state examination, Beck's depression inventory, Beck's anxiety inventory and self-reporting questionnaire-20 before and after a 3-month supervised resistance and aerobic exercise programme comprising structured physical activity three times a week. Clinically relevant improvement was observed in the Beck's depression inventory and Beck's anxiety inventory scores following the 12-week training (61%, p = 0.001, and 53%, p = 0.02, respectively). Even though statistically not significant (p = 0.398), the cognitive performance of this relatively young patient population also benefited from the programme. These results demonstrate positive effects of active lifestyle on non-psychotic mental disorders in patients with chronic systemic diseases, recommending exercise as an alternative treatment option. PMID:26410835

  8. [Mucolytics in acute and chronic respiratory tract disorders. II. Uses for treatment and antioxidant properties].

    Science.gov (United States)

    Kupczyk, Maciej; Kuna, Piotr

    2002-03-01

    In the first part of our editorial we reviewed the possible factors responsible for mucus hypersecretion in acute and chronic pulmonary diseases. The present paper presents the results of studies proving, that mucolytics are useful in adjunctive therapy of respiratory tract disorders. Mucolytic agents such as Ambroxol and N-acetylcysteine are able to alter the secretion of mucus and its physical properties which results in improvement of mucociliary clearance. Current evidence indicate, that these drugs are effective, especially in chronic obstructive pulmonary disease, asthma and acute bronchitis. They produce a modest improvement in symptom control and lung function. It has been demonstrated that there is a synergism between mucolytics and antibiotics in the treatment of exacerbation of chronic bronchitis. Moreover, they act as scavengers of reactive oxygen species. Ambroxol is able to inhibit mediator release involved in the pathogenesis of allergic inflammation. As mucolytics are cheap and well-tolerated they are beneficial in the therapy of patients suffering from respiratory tract disorders. PMID:12053601

  9. Anagrelide treatment in 52 patients with chronic myeloproliferative diseases

    DEFF Research Database (Denmark)

    Penninga, E; Jensen, B A; Hansen, P B;

    2004-01-01

    -term treatment with anagrelide. Furthermore, a total of 13 events were recorded. More than 25% of these events occurred in patients with platelet counts between 400 and 600 x 10(9)/l and almost 40% of all events occurred in patients with platelet counts above 400 x 10(9)/l. This observation supports the...

  10. Association of chronic hepatitis C with major depressive disorders: irrespective of interferon-alpha therapy

    Directory of Open Access Journals (Sweden)

    Chessa Luchino

    2007-10-01

    Full Text Available Abstract Background Mood and anxiety symptoms in chronic hepatitis C (CHC may be related to the patient awareness of the diagnosis and prognosis, to side effects induced by interferon (IFN-alpha treatment, as well as to substance abuse. However, the observation of metabolic alterations in patients with CHC has led to hypothesize a direct effect of hepatitis C virus (HCV on brain function. This study was aimed at elucidating whether CHC is associated with specific anxiety or mood disorders independently of confounding factors. Methods Patient cohort: consecutive patients, 135 with CHC and 76 with chronic hepatitis B (CHB. Exclusion criteria: previous treatment with IFN-alpha, co-infection with HCV and hepatitis B virus, infection with human immunodeficiency virus, drug or alcohol abuse, or malignancies. Controls: subjects without evidence of hepatitis randomly extracted from the database of a previous epidemiological study; they were divided into two groups of 540 (332 males and 304 (220 males as controls for patients with CHC and CHB, respectively. The psychiatric diagnosis was formulated by means of the Composite International Diagnostic Interview Simplified carried out by a physician according to DSM-IV criteria. Results A higher lifetime prevalence of major depressive disorder (MDD was observed among CHC compared to CHB or controls. The risk of MDD was not statistically different between CHB and controls. Both the CHC and CHB groups showed a significantly higher frequency of panic disorder when compared to controls. No statistical differences were observed in the prevalence of general anxiety disorder and social phobia when CHC or CHB were compared to controls. Conclusion The present study provides the first evidence of an association between CHC and MDD, diagnosed on the basis of well-defined international criteria. This association is independent of treatment with IFN-alpha and is not influenced by substance or alcohol abuse. By contrast

  11. The prevalence of Pneumocystis jirovecii among patients with different chronic pulmonary disorders in Ahvaz, Iran

    Directory of Open Access Journals (Sweden)

    Elham Aboualigalehdari

    2016-01-01

    Full Text Available Background and Objectives: Pneumocystis jirovecii pneumonia (PJP is a chronic fungal infection that caused by P. ji- rovecii. Disease is more prevalent among the HIV-infected patients. The colonization of pneumocystis in human respiratory system is associated with the airway inflammation and obstruction. The current study was conducted to identify the preva- lence of P. jirovecii among the patients with chronic pulmonary disorders in Ahvaz, Iran.Materials and Methods: In the present study, 115 bronchoalveolar lavage (BAL specimens were collected from patients. Samples were subjected to Nested-PCR with specific primers. The second PCR products were used for sequencing analysis.Results: Our findings demonstrated that 31(27.0% of samples were positive for P. jirovecii. Nine patients (29% have tuberculosis (TB followed by, 1(3.2% HIV positive and 21(67.7% miscellaneous pulmonary disorders. Our results show that there was no significant differences between sex (male:female ratio, 17:14, TB, HIV and P. jirovecii in BAL samples (P>0.05.Conclusion: The current study is the first report from Ahvaz and it showed a relatively high frequency (27% of P. jirovecii among patients with different pulmonary disorders. In addition Nested-PCR might be reliable technique for diagnosis of P. jirovecii, while the Grocott's methenamine silver (GMS have a low sensitivity, which only two positive patients were identified. Keywords: Pneumocystis jirovecii pneumonia; HIV-infected patients; Tuberculosis, Ahvaz-Iran

  12. Myeloid-derived suppressor cells in patients with myeloproliferative neoplasm.

    Science.gov (United States)

    Wang, Jen Chin; Kundra, Ajay; Andrei, Mirela; Baptiste, Stacey; Chen, Chi; Wong, Ching; Sindhu, Hemant

    2016-04-01

    Although BCR-ABL negative myeloproliferative neoplasms (MPN)--and especially myelofibrosis (MF)--are recognized to be associated with autoimmune phenomena, immune derangements in MPN have been much less studied. Myeloid-derived suppressor cells (MDSC) are one type of important immune modulator cell. Therefore, we studied MDSCs in MPN disease. MDSCs were studied in two cohorts: the first cohort was 55 patients including 16 primary myelofibrosis (PMF), 7 post-polycythemia vera (PV)-MF, 2 post-essential thrombocythemia (ET)-MF, 11 ET, 17 PV, 2 undefined MPN disorder, and 23 normal controls; the second cohort included 38 patients: 17 ET, 7 PMF, 3 ET-MF, 2 PV-MF, 9 PV patients, and 20 normal volunteers. The second cohort was studied using freshly collected specimens and a comparable age group as controls. CD11b(+), CD14(-), and CD33(+) cells were defined as MDSCs in both cohorts by flow cytometry. Since there are no differences in MDSC levels among different MPN categories, they were grouped as MPNs. The results showed that MDSCs were significantly elevated in MPNs compared with controls in both cohorts. We also performed RT-PCR and found that MPN patients have significantly elevated arginase-1 mRNA compared with controls, and sorted MDSCs were found to have suppressor T cell activity in MPNs, substantiating the hypothesis that levels of MDSCs are, in fact, deranged in MPNs. MDSC levels were not correlated with JAK2 status, white blood cells, Hb levels, platelet counts, splenomegaly, or the degree of bone marrow fibrosis (in MF). Further studies in immune therapy involving MDSC inhibitors or differentiation may be developed to treat MPN disease. PMID:26943702

  13. The expression of Death Inducer-Obliterator (DIDO) variants in Myeloproliferative Neoplasms.

    Science.gov (United States)

    Berzoti-Coelho, Maria Gabriela; Ferreira, Aline Fernanda; de Souza Nunes, Natalia; Pinto, Mariana Tomazini; Júnior, Maurício Cristiano Rocha; Simões, Belinda Pinto; Martínez-A, Carlos; Souto, Elizabeth Xisto; Panepucci, Rodrigo Alexandre; Covas, Dimas Tadeu; Kashima, Simone; Castro, Fabíola Attié

    2016-07-01

    Chronic Myeloid Leukemia (CML), Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF) are Myeloproliferative Neoplasms (MPN) characterized by clonal myeloproliferation without cell maturation impairment. CML pathogenesis is associated with the Ph chromosome leading to BCR-ABL tyrosine-kinase constitutive expression. The Ph negative MPN (PV, ET and PMF) are characterized by the mutation JAK2(V617F) of the JAK2 protein in the auto-inhibitory JH2 domain, which is found in most PV patients and in approximately half of ET and PMF patients. Considerable effort is being made to understand the role of JAK2(V617F) at the MPN initiation and to clarify the pathogenesis and apoptosis resistance in CML, PV, ET and PMF patients. In the present investigation, we evaluated the Death Inducer-Obliterator (DIDO) (variants DIDO 1, 2 and 3) levels in CML, PV, ET and PMF patients. Our data reported the DIDO 1, 2 and 3 differential expressions in Myeloproliferative Neoplasms. PMID:27282563

  14. Lymphocytic Bronchiolitis as Presenting Disorder in an Undiagnosed Adult Patient with Chronic Granulomatous Disease

    Directory of Open Access Journals (Sweden)

    Payam Tabarsi

    2007-12-01

    Full Text Available Chronic Granulomatous Disease (CGD is a rare primary immunodeficiency disease. Although the most affected patients are diagnosed in childhood, there are several reports of the disease presenting in adult patients. Here we present a 40 years old man who was admitted in hospital due to respiratory symptoms and ground glass pattern in high resolution computed tomography of lung. Open lung biopsy revealed lymphocytic bronchiolitis. Because of past medical history of granulomatous lesion in lung and recurrent abscesses of skin and soft tissue, NBT test was conducted which its result revealed that the disorder was compatible with CGD and then it was confirmed by fluorescent cytometry.

  15. The model of rat lipid metabolism disorder induced by chronic stress accompanying high-fat-diet

    OpenAIRE

    Shaodong Chen; Jing Li; Haihong Zhou; Manting Lin; Yihua Liu

    2011-01-01

    Abstract Objective To develop an animal model of Lipid Metabolism Disorder, which conforms to human clinical characteristic. Methods: There were 24 male Wistar rats that were randomly divided into 3 groups with 8 rats in each. They were group A (normal diet), group B (high-fat-diet), group C (chronic stress+ high-fat-diet). Group A was fed with normal diet, while group B and C were fed with high-fat-diet, going on for 55 days. From the 35th day, group B and C received one time of daily chroni...

  16. Complex radionuclide evaluation of structural and functional kidneys disorders of children with chronic obstructive pyelonephritis

    International Nuclear Information System (INIS)

    Children's chronic obstructive pyelonephritis (COPN) is the most important and difficult among microbes-inflammatory kidney's deceases. Kidney's COPN functional disorders are evaluated generally by clinical and laboratory criteria which not always have high informativity. Scintigraphic kidney's examinations with nephrotropic radiopharmaceuticals (NRP) are more informative. Conclusion. The most informative are 99mTc-EC and 99mTc-DTPA for initial examinations, 99mTc-DTPA and 99mTc-pyrophosphate for monitoring examinations

  17. Neurobiologically informed treatment for adults with anorexia nervosa: a novel approach to a chronic disorder

    Science.gov (United States)

    Knatz, Stephanie; Wierenga, Christina E.; Murray, Stuart B.; Hill, Laura; Kaye, Walter H.

    2015-01-01

    Anorexia nervosa (AN) is a severe and debilitating disorder with significant medical and psychological sequelae. To date, there are no effective treatments for adults, resulting in high rates of chronicity, morbidity, and mortality. Recent advances in brain imaging research have led to an improved understanding of etiology and specific neurobiological mechanisms underlying symptoms. Despite this, there are no treatments focused on targeting symptoms using this empirically supported mechanistic understanding of the illness. Updated treatment approaches focused on targeting neurobiological mechanisms underlying core AN symptomatology are necessary to improve treatment out-comes for this population. Neurobiologically Enhanced With Family Eating Disorder Trait Response Treatment (NEW FED TR) is a neurobiologically informed treatment targeting key temperament constructs associated with the illness through the delivery of psychoeducation and skills training to patients and nominated carers. PMID:26246796

  18. Behavioral disorders and low quality of life in children and adolescents with chronic kidney disease.

    Science.gov (United States)

    Marciano, Renata C; Soares, Cristina M Bouissou; Diniz, José Silvério S; Lima, Eleonora M; Silva, Jose Maria P; Canhestro, Monica R; Gazzinelli, Andrea; Melo, Carla Cristina D; Dias, Cristiane S; Simões e Silva, Ana Cristina; Correa, Humberto; Oliveira, Eduardo A

    2011-02-01

    Recent years has seen an increasing interest in the quality of life (QOL) of children with chronic kidney disease (CKD). The objective of this cross-sectional study was to investigate the prevalence of behavioral disorders and to assess the health-related QOL (HRQOL) in 136 patients with CKD. To estimate the prevalence of behavior disorders and analyze HRQOL, we used the Strengths and Difficulties Questionnaire (SDQ) and Pediatric Inventory of Quality of Life (PedsQL) Core Scales as assessment tools for both the patients and caregivers. When compared to healthy controls, the CKD group had significantly lower scores in almost all PedsQL domains. After adjustment, only absence of religion/other religions remained significantly associated with a lower global HRQOL score [odds ratio (OR) 6.2, P=0.009]. Among the parents, two factors remained significantly associated with a lower global HRQOL score: patients' age >10 years (OR 5.4, P=0.033) and absence of religion/other religions (OR 3.2, P=0.038). The CKD group demonstrated a higher proportion of behavioral and emotional disorders in all SDQ domains. There was a negative correlation between the presence of behavior and emotional disorders and HRQOL score (r= -0.552, Plife quality of this pediatric population. PMID:21110044

  19. Development of paroxysmal nocturnal hemoglobinuria in CALR-positive myeloproliferative neoplasm

    Science.gov (United States)

    Fraiman, Yarden S; Cuka, Nathan; Batista, Denise; Vuica-Ross, Milena; Moliterno, Alison R

    2016-01-01

    Paroxysmal nocturnal hemoglobinuria (PNH), a disease characterized by intravascular hemolysis, thrombosis, and bone marrow failure, is associated with mutations in the PIG-A gene, resulting in a deficiency of glycosylphosphatidylinositol-anchored proteins. Many hypotheses have been posed as to whether PNH and PIG-A mutations result in an intrinsic survival benefit of CD55−/CD59− cells or an extrinsic permissive environment that allows for their clonal expansion within the bone marrow compartment. Recent data have identified the concurrence of PIG-A mutations with additional genetic mutations associated with myeloproliferative disorders, suggesting that some presentations of PNH are the result of a stepwise progression of genetic mutations similar to other myelodysplastic or myeloproliferative syndromes. We report for the first time in the literature the development of clinically significant PNH in a patient with JAK2V617F-negative, CALR-positive essential thrombocythemia, providing further support to the hypothesis that the development of PNH is associated with the accumulation of multiple genetic mutations that create an intrinsic survival benefit for clonal expansion. This case study additionally highlights the utility of genomic testing in diagnosis and the understanding of disease progression in the clinical setting. PMID:27313483

  20. Differences in 99mTc-HMPAO brain SPET perfusion imaging between Tourette's syndrome and chronic tic disorder in children

    International Nuclear Information System (INIS)

    Early differential diagnosis between Tourette's syndrome and chronic tic disorder is difficult but important because both the outcome and the treatment of these two childhood-onset diseases are distinct. We assessed the sensitivity and specificity of brain single-photon emission tomography (SPET) perfusion imaging in distinguishing the two diseases, and characterized their different cerebral perfusion patterns. Twenty-seven children with Tourette's syndrome and 11 with chronic tic disorder (mean age 9.5 and 8.6 years, respectively) underwent brain SPET with technetium-99m hexamethylpropylene amine oxime (HMPAO). Visual interpretation and semi-quantitative analysis of SPET images were performed. On visual interpretation, 22 of 27 (82%) of the Tourette's syndrome group had lesions characterized by decreased perfusion. The left hemisphere was more frequently involved. None of the children with chronic tic disorder had a visible abnormality. Semi-quantitative analysis showed that, compared with children with chronic tic disorder, children with Tourette's syndrome had significantly lower perfusion in the left lateral temporal area and asymmetric perfusion in the dorsolateral frontal, lateral and medial temporal areas. In conclusion, using the visual approach, brain SPET perfusion imaging is sensitive and specific in differentiating Tourette's syndrome and chronic tic disorder. The perfusion difference between the two groups, demonstrated by semi-quantitative analysis, may be related more to the co-morbidity in Tourette's syndrome than to tics per se. (orig.)

  1. Attitudes antecedent to transition to self-management of a chronic genetic disorder.

    Science.gov (United States)

    Giarelli, E; Bernhardt, B A; Pyeritz, R E

    2008-10-01

    Marfan syndrome (MFS) is the exemplar of chronic genetic disorders that require multiorgan system management by health care providers (HCPs) and lifelong self-management by affected individuals. This article describes the ways to facilitate transition to self-management (TSM) by the adolescent with MFS. This thematic content analysis uses data collected for a larger grounded theory investigation of TSM of a chronic genetic disorder in adolescents and focuses on the system issues related to transition. A total sample of 107 included three groups of participants: parents (n = 39), adolescents (n = 37, ages 14-21 years) and young adults (n = 16, ages 22-34 years) with MFS, and HCPs (n = 15), including physicians, genetic counselors, and nurses. Data were derived from 180 transcripts of audiotaped interviews and a sociodemographic survey. Frames of mind that are antecedent to transition were belief in the diagnosis, wanting to understand and appreciate the cause and effect of MFS, and willing to share responsibility in problem solving. These frames of mind occurred primarily within the context of the family relationship. Parents taught children self-surveillance as 'listening to one's body'. The parent's fears and need to stay involved in the child's health care slowed the child's independent work on self-management responsibilities. A systematic, institutionalized transition program for adolescents might involve parents and the child soon after diagnosis and incrementally build acknowledgment, understanding, and rapport. PMID:18616734

  2. Perspectives on chronic inflammation in essential thrombocythemia, polycythemia vera, and myelofibrosis

    DEFF Research Database (Denmark)

    Hasselbalch, Hans K

    2012-01-01

    The morbidity and mortality of patients with the chronic Philadelphia-negative myeloproliferative neoplasms (MPNs), essential thrombocythemia, polycythemia vera, and primary myelofibrosis are mainly caused by cardiovascular diseases, thrombohemorrhagic complications, and bone marrow failure because...

  3. Chronicity factors of temporomandibular disorders: a critical review of the literature

    Directory of Open Access Journals (Sweden)

    Maísa Soares GUI

    2015-01-01

    Full Text Available Facial pain often persists long after any identifiable organic pathology has healed. Moreover, in a subgroup of patients with temporomandibular disorder (TMD, no treatment is effective. Knowledge of factors associated with persistent pain in TMD could help identify personalized treatment approaches. Therefore, we conducted a critical review of the literature for the period from January 2000 to December 2013 to identify factors related to TMD development and persistence. The literature findings showed that chronic TMD is marked by psychological distress (somatization and depression, affective distress, fear of pain, fear of movement, and catastrophizing and characteristics of pain amplification (hyperalgesia and allodynia. Furthermore, these factors seem to interact in TMD development. In addition, our review demonstrates that upregulation of the serotonergic pathway, sleep problems, and gene polymorphisms influence the chronicity of TMD. We conclude that psychological distress and pain amplification contribute to chronic TMD development, and that interactions among these factors complicate pain management. These findings emphasize the importance of multidisciplinary assistance in TMD treatment.

  4. CASE REPORT: Adult Type – Chronic Myeloid Leukemia in Childhood: A Case Report

    Directory of Open Access Journals (Sweden)

    Dhiraj B. Nikumbh

    2012-01-01

    Full Text Available Background: In pediatric patients, chronic myeloid leukemia (CML accounts for 2 to 5% of all the leukemia’s but has an incidence ofless than 1 case per 1,00,000 population younger than 20 years of age per year. CML is a clonal hematopoietic stem cell disorder. Asper WHO classification, CML is included in Myelodysplastic/Myeloproliferative disorder. Adult type - CML is extremely rare in childhood. Case history: We report one such a case of Adult type of CML in an 11 year old male patient with chief complaints of abdominal distension since 1 month and cough with feversince 4-5 days. The clinical differential diagnosis was malaria, storage disorder or tropical splenomegaly. Though biologicalbehaviour and prognosis are identical to that of adult type, we are reporting this case because of its extremely uncommon incidence.

  5. INFLUENCE OF BRONCHOLYTIC THERAPY ON VENTRICULAR RHYTHM DISORDERS IN PATIENTS WITH CHRONIC OBSTRUCTIVE PULMONARY DISEASE

    Directory of Open Access Journals (Sweden)

    Kh. Kh. Shugushev

    2016-01-01

    Full Text Available Aim. To evaluate effects of broncholytic therapy on ventricular rhythm disorders and high resolution electrocardiogram indices in patients with chronic obstructive pulmonary disease (COPD.Material and methods. 144 patients (54 women and 90 men; aged 47,1±1,5 y.o. with COPD and 35 patients of control group without respiratory and cardiovascular diseases (23 men and 12 women; aged 42,4±2,8 y.o. were examined. 24-hour ECG monitoring and high resolution ECG with time and spectral-time mapping of ventricular ECG complex were performed in patients additionally to routine examination.Results. Reduction of ventricular rhythm disorders rate was detected during therapy with extended-release theophylline or salmeterol/fluticasone in patients with moderate and severe COPD. This effect was more significant for salmeterol/fluticasone treatment. Treatment with extended-release theophylline led to increase of a number of ventricular extra systoles in patients with severe COPD. Salmeterol/fluticasone treatment did not influence number of ventricular extrasystoles. Broncholytic therapy had positive influence on processes of ventricular depolarization. It was shown by high resolution ECG indicators improvement and by late ventricular potential rate reduction.Conclusion. Salmeterol/fluticasone combination is more reasonable than extended-release theophylline for broncholytic therapy in patients with severe COPD and ventricular rhythm disorders.

  6. Subjective health complaints in patients with chronic Whiplash Associated Disorders (WAD). Relationships with physical, psychological, and collision associated factors

    OpenAIRE

    Camilla Ihlebæk; Arit Ødegaard; John Vikne; Hege R. Eriksen; Even Lærum

    2009-01-01

     Aims: Investigate subjective health complaints (SHC) in chronic whiplash associated disorder (WAD, grade I & II) patients, and to identify physical, psychological, and collision associated factors that might be associated with high levels of comorbidity. Method: During the years 2000-2002 171 chronic WAD patients filled in questionnaires and underwent physical examination. The prevalence of SHC was recorded and compared with a representative sample of the Norwegian population (n=1014). Resul...

  7. The relationship between chronic whiplash-associated disorder and posttraumatic stress: attachment-anxiety may be a vulnerability factor

    OpenAIRE

    Andersen, Tonny Elmose; Elklit, Ask; Vase, Lene

    2011-01-01

    Background: In more than 90% of whiplash accidents a good explanation regarding the association between trauma mechanism, organic pathology, and persistent symptoms has failed to be provided. Objective: We predicted that the severity of chronic whiplash-associated disorder (WAD), measured as number of whiplash symptoms, pain duration, pain-related disability, and degree of somatisation would be associated with the number of post-traumatic stress disorder symptoms (PTSD). Secondly, we expected...

  8. BCR-ABL negative myeloproliferative neoplasia: a review of involved molecular mechanisms.

    Science.gov (United States)

    Koopmans, Suzanne M; Schouten, Harry C; van Marion, Ariënne M W

    2015-02-01

    The clonal bone marrow stem cell disorders essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) belong to the group of Philadelphia chromosome negative myeloproliferative neoplasia (Ph- MPN). In 2005 the JAK2(V617F) mutation was discovered which has generated more insight in the pathogenetic mechanism of the MPNs. More mutations have been detected in MPN patients since. However, the underlying cause of MPN has not been discovered so far. The mechanism of increased angiogenesis in MPNs and the development of fibrosis in the bone marrow in PMF patients and in some ET and PV patients is still not known. This review will focus on the most important molecular pathogenetic mechanisms in MPN patients. PMID:25196073

  9. Do SF-36 summary scores work as outcome measures in chronic functional disorders?

    DEFF Research Database (Denmark)

    Schröder, Andreas; Ørnbøl, Eva; Fink, Per

    Background SF-36 Physical and Mental Component Summaries (PCS and MCS) are increasingly used as main measures of outcome in clinical trials. Yet, studies in different patient populations have demonstrated serious problems in interpreting these summary scores. We are conducting a randomised contro...... with interacting physical and mental health problems. One may benefit from using other scoring methods such as the RAND-36 HSI to validate the results.......Background SF-36 Physical and Mental Component Summaries (PCS and MCS) are increasingly used as main measures of outcome in clinical trials. Yet, studies in different patient populations have demonstrated serious problems in interpreting these summary scores. We are conducting a randomised...... controlled trial on cognitive behavioural therapy in patients with severe and chronic functional disorders. Based on a pilot study and baseline data, we have assessed the performance of the summary scores. Aim To demonstrate problems in the orthogonal factor solution for PCS and MCS and to assess other...

  10. Diagnostic agreement of schizophrenia spectrum disorders among chronic patients with functional psychoses

    DEFF Research Database (Denmark)

    Jakobsen, K D; Nordgaard, Julie; Parnas, J; Werge, Thomas

    2006-01-01

    BACKGROUND: To investigate whether diagnostic agreement across different diagnostic systems improves in a sample of chronic patients suffering from functional psychosis compared to first-admitted patients. SAMPLING AND METHODS: Among 353 patients with a history of functional psychosis, a subset of...... 100 individuals (35 women and 65 men) were randomly sampled and assessed using the Operational Criteria Checklist for Psychotic Illness and Affective Illness (OPCRIT). Based on the OPCRIT diagnoses the subjects suffering from schizophrenia and schizophrenia spectrum disorders according to seven...... diagnostic systems were identified. Diagnostic agreement was assessed using unweighted kappa-statistics and pairwise concordance rates (CR). RESULTS: High diagnostic agreement of schizophrenia was observed across the ICD-10 and DSM systems (CR >0.70, kappa >0.70), which all had a significantly lower...

  11. [Mucolytics in acute and chronic respiratory tract disorders. I. Pathophysiology and mechanisms of action].

    Science.gov (United States)

    Kupczyk, Maciej; Kuna, Piotr

    2002-03-01

    Mucus hypersecretion is a cardinal sign of both acute and chronic pulmonary diseases. Normally, mucus protects respiratory tract, but its overproduction leads to airway obstruction and promotes bacterial colonization. In the first part of our review we outlined the possible factors responsible for mucus hypersecretion and clinical consequences of this process. Mucolytic agents such as Ambroxol and N-acetylcysteine are able to alter the secretion of mucus and its physical properties which results in improvement of mucociliary clearance. Mechanisms of action and indications for use of mucolytics are presented. Mucolytics have been shown to have a role in improving lung functions and patients' quality of life. Undoubtedly they are useful as an adjunctive therapy of respiratory tract disorders. PMID:12053600

  12. The Relationship Between Chronic Inflammation and Glucidic-Lipidic Profile Disorders in Kidney Transplant Recipients

    Directory of Open Access Journals (Sweden)

    Tarța I.D.

    2016-03-01

    Full Text Available Introduction: Chronic inflammation has a proven role in atherogenesis, lipid profile parameters being related to cytokine production. In kidney transplant recipients, interleukin 6 (IL-6 is significantly associated with graft-related outcomes and also alterations of cholesterol and triglyceride metabolism. The aim of this study was to investigate the relationship between chronic inflammation and glucidic-lipidic metabolism disorders in a group of patients with kidney transplantation as renal replacement therapy. Methods: A prospective observational study which enrolled thirtysix non-diabetic kidney transplant recipients was conducted in the Nephrology and Peritoneal Dialysis Department, County Clinic Hospital of Tirgu Mures. The study group was divided as following: recipients with serum IL-6 concentration higher than 3.8 pg/ml (group A and IL-6 within the normal range (group B. Results: Allograft recipients with higher serum IL-6 had significant higher erytrocyte sedimentation rate(ESR, p=0.0067. Patients with over-the-range levels of IL-6 had significant higher levels of serum cholesterol and LDL-cholesterol respectively (p=0.0242 and p=0.0081. Serum Apo-B was also significant higher in Group A than Group B. Protein excretion was significant higher in patients from group A (p=0.0013. No statistical significant relationship could be proven between elevated levels of IL-6 and hbA1c, insulin and glycosuria disturbances in the two groups. Also, we found no statistical significant association between resistivity and pulsatility indices (both hilum and intragraft or carotid intima media thickness. Conclusion: Serum interleukin 6 is related to lipid profile disorders and less to glucidic metabolism anomalies in non-diabetic kidney transplant recipients.

  13. Antihypertensive therapy in patients on chronic lithium treatment for bipolar disorders.

    Science.gov (United States)

    Bisogni, Valeria; Rossitto, Giacomo; Reghin, Francesco; Padrini, Roberto; Rossi, Gian Paolo

    2016-01-01

    Bipolar disorders are chronic conditions treated with lithium, which exerts deleterious effects on the kidney, among which nephrogenic diabetes insipidus, tubular acidosis and ultimately chronic kidney disease. Conversely, drugs that alter renal function can modify its serum levels and lead to the potentially fatal lithium intoxication. A search in the main library databases from 1975 to 2015 to identify interactions between antihypertensive drugs and lithium using the Population Intervention Comparison Outcome strategy provided only 30 reports of lithium intoxication. A regression analysis showed that the severity of lithium intoxication was significantly predicted by female, age, and use of certain classes of antihypertensive agents. A model including certain albeit not all diuretics and/or inhibitors of the renin-angiotensin system, but not age, serum lithium or creatinine levels at baseline and/or on admission to the hospital, predicted lithium toxicity. The true incidence of lithium intoxication is unknown but probably low, albeit underestimated. Nonetheless, in patients treated with lithium, monitoring of the serum lithium levels and clinical conditions is mandatory after the introduction of antihypertensive drugs, as diuretics and renin-aldosterone system inhibitors. PMID:26630207

  14. Effect of zolpidem in chronic disorders of consciousness: a prospective open-label study

    Science.gov (United States)

    Thonnard, Marie; Gosseries, Olivia; Demertzi, Athena; Lugo, Zulay; Vanhaudenhuyse, Audrey; Bruno, Marie-Aurélie; Chatelle, Camille; Thibaut, Aurore; Charland-Verville, Vanessa; Habbal, Dina; Schnakers, Caroline; Laureys, Steven

    2013-01-01

    Summary Zolpidem has been reported as an “awakening drug” in some patients with disorders of consciousness (DOC). We here present the results of a prospective open-label study in chronic DOC patients. Sixty patients (35±15 years; 18 females; mean time since insult ± SD: 4±5.5 years; 31 with traumatic etiology) with a diagnosis of vegetative state/unresponsive wakefulness syndrome (n=28) or minimally conscious state (n=32) were behaviorally assessed using the Coma Recovery Scale-Revised (CRS-R) before and one hour after administration of 10 mg of zolpidem. At the group level, the diagnosis did not change after intake of zolpidem (p=0.10) and CRS-R total scores decreased (p=0.01). Twelve patients (20%) showed improved behaviors and/or CRS-R total scores after zolpidem administration but in only one patient was the diagnosis after zolpidem intake found to show a significant improvement (functional object use), which suggested a change of diagnosis. However, in this patient, a double-blind placebo-controlled trial was performed in order to better specify the effects of zolpidem, but the patient, on this trial, failed to show any clinical improvements. The present open-label study therefore failed to show any clinically significant improvement (i.e., change of diagnosis) in any of the 60 studied chronic DOC patients. PMID:24598393

  15. Anatomical changes within the medullary dorsal horn in chronic temporomandibular disorder pain.

    Science.gov (United States)

    Wilcox, S L; Gustin, S M; Macey, P M; Peck, C C; Murray, G M; Henderson, L A

    2015-08-15

    Accumulated evidence from experimental animal models suggests that neuroplastic changes at the dorsal horn are critical for the maintenance of various chronic musculoskeletal pain conditions. However, to date, no study has specifically investigated whether neuroplastic changes also occur at this level in humans. Using brain imaging techniques, we sought to determine whether anatomical changes were present in the medullary dorsal horn (spinal trigeminal nucleus caudalis) in subjects with the chronic musculoskeletal pain. In twenty-two subjects with painful temporomandibular disorders (TMDs) and forty pain-free controls voxel based morphometry of T1-weighted anatomical images and diffusion tensor images were used to assess regional grey matter volume and microstructural changes within the brainstem and, in addition, the integrity of ascending pain pathways. Voxel based morphometry revealed significant regional grey matter volume decreases in the medullary dorsal horn, in conjunction with alterations in diffusivity properties, namely an increase in mean diffusivity, in TMD subjects. Volumetric and mean diffusivity changes also occurred in TMD subjects in regions of the descending pain modulation system, including the midbrain periaqueductal grey matter and nucleus raphe magnus. Finally, tractography revealed altered diffusivity properties, namely decreased fractional anisotropy, in the root entry zone of the trigeminal nerve, the spinal trigeminal tract and the ventral trigeminothalamic tracts of TMD subjects. These data reveal that chronic musculoskeletal pain in humans is associated with discrete alterations in the anatomy of the medullary dorsal horn, as well as its afferent and efferent projections. These neural changes may be critical for the maintenance of pathological pain. PMID:25979666

  16. Impairment and Coping in Children and Adolescents with Chronic Fatigue Syndrome: A Comparative Study with Other Paediatric Disorders

    Science.gov (United States)

    Garralda, M. Elena; Rangel, Luiza

    2004-01-01

    Background: Functional impairment is a key feature of chronic fatigue syndrome (CFS) of childhood. Aim: To compare impairment, illness attitudes and coping mechanisms in childhood CFS and in other paediatric disorders. Method: Participants were 28 children and adolescents with CFS, 30 with juvenile idiopathic arthritis (JIA) and 27 with emotional…

  17. Alcohol abuse and dependence criteria as predictors of a chronic course of alcohol use disorders in the general population

    NARCIS (Netherlands)

    De Bruijn, C; Van den Brink, W; De Graaf, R; Vollebergh, WAM

    2005-01-01

    Aims: To investigate whether DSM-IV abuse and dependence criteria and the ICD-10 criterion for craving differentially predict a chronic course of alcohol use disorders (AUD) in the general population. Methods: Data were derived from the Netherlands Mental Health Survey and Incidence Study, a large r

  18. The water maze paradigm in experimental studies of chronic cognitive disorders: Theory, protocols, analysis, and inference.

    Science.gov (United States)

    Kapadia, Minesh; Xu, Josie; Sakic, Boris

    2016-09-01

    An instrumental step in assessing the validity of animal models of chronic cognitive disorders is to document disease-related deficits in learning/memory capacity. The water maze (WM) is a popular paradigm because of its low cost, relatively simple protocol and short procedure time. Despite being broadly accepted as a spatial learning task, inference of generalized, bona fide "cognitive" dysfunction can be challenging because task accomplishment is also reliant on non-cognitive processes. We review theoretical background, testing procedures, confounding factors, as well as approaches to data analysis and interpretation. We also describe an extended protocol that has proven useful in detecting early performance deficits in murine models of neuropsychiatric lupus and Alzheimer's disease. Lastly, we highlight the need for standardization of inferential criteria on "cognitive" dysfunction in experimental rodents and exclusion of preparations of a limited scientific merit. A deeper appreciation for the multifactorial nature of performance in WM may also help to reveal other deficits that herald the onset of neurodegenerative brain disorders. PMID:27229758

  19. Pycnogenol® in chronic venous insufficiency and related venous disorders.

    Science.gov (United States)

    Gulati, Om P

    2014-03-01

    The present review provides an update of the biological profile of Pycnogenol in the light of its use in the treatment of chronic venous insufficiency (CVI) and related venous disorders such as deep vein thrombosis (DVT), post-thrombotic syndrome, long haul air-travel-related leg oedema, venous ulcers and acute haemorrhoids. Pycnogenol is a French maritime pine bark extract produced from the outer bark of Pinus pinaster Ait. subsp. atlantica. Its strong antioxidant, anti-inflammatory and vasodilator activities, antithrombotic effects and collagen stabilizing properties make it uniquely able to target the multi facet pathophysiology of CVI and related venous disorders. Clinical studies have shown that it can reduce oedema of the legs in CVI, reduce the incidence of deep venous thrombosis during long haul flights and enhance the healing of venous ulcers and haemorrhoidal episodes by topical application and/or oral administration. This review highlights clinical research findings on the safety, compliance and efficacy of Pycnogenol, including its use in combination products. PMID:23775628

  20. Elevated [11C]-D-deprenyl uptake in chronic Whiplash Associated Disorder suggests persistent musculoskeletal inflammation.

    Science.gov (United States)

    Linnman, Clas; Appel, Lieuwe; Fredrikson, Mats; Gordh, Torsten; Söderlund, Anne; Långström, Bengt; Engler, Henry

    2011-01-01

    There are few diagnostic tools for chronic musculoskeletal pain as structural imaging methods seldom reveal pathological alterations. This is especially true for Whiplash Associated Disorder, for which physical signs of persistent injuries to the neck have yet to be established. Here, we sought to visualize inflammatory processes in the neck region by means Positron Emission Tomography using the tracer (11)C-D-deprenyl, a potential marker for inflammation. Twenty-two patients with enduring pain after a rear impact car accident (Whiplash Associated Disorder grade II) and 14 healthy controls were investigated. Patients displayed significantly elevated tracer uptake in the neck, particularly in regions around the spineous process of the second cervical vertebra. This suggests that whiplash patients have signs of local persistent peripheral tissue inflammation, which may potentially serve as a diagnostic biomarker. The present investigation demonstrates that painful processes in the periphery can be objectively visualized and quantified with PET and that (11)C-D-deprenyl is a promising tracer for these purposes. PMID:21541010

  1. Analysis of Chronic Temporomandibular Disorders Based on the Latest Diagnostic Criteria

    Directory of Open Access Journals (Sweden)

    Svechtarov V.

    2015-05-01

    Full Text Available The objective of this study is to analyze the distribution of the most common diagnoses observed in patients with chronic temporomandibular disorders, based on the new diagnostic criteria (DC/TMD adopted in 2014. The previous Research Diagnostic Criteria (RDC/TMD adopted in 1992, consisted of three main groups of eight diagnostic subgroups and is currently transformed into two main groups and twelve subgroups, respectively. All subgroups correspond to the nomenclature of the ICD-10. The new clinical diagnostic indices are also modified. The analysis showed a prevalence of Pain-Related TMD compared with that of intra-articular disorders in ratio 57.89% to 42.10%. In Pain-Related TMD arthralgia was represented in 55% of cases; local myalgia - in 12%, myofascial pain - in 18%, myofascial pain with referral - in 14%, headache attributed to TMD - in 1%. In Intra-articular TMD disc displacement with reduction was found in 23% of the cases, disc displacement with reduction with intermittent locking - in 3%, disc displacement without reduction with limited opening - in 25%, disc displacement without reduction and without limited opening - in 8%. Degenerative diseases were found in 14.28%, and hypermobility and subluxations - in 26.98%. These analyzes differ and can only partly be compared with previous analyzes based on RDC system. The changes in the diagnostic criteria require new clinical studies in order to refine the picture of temporomandibular pathology in accordance with the modern views on the matter.

  2. Elevated [11C]-D-deprenyl uptake in chronic Whiplash Associated Disorder suggests persistent musculoskeletal inflammation.

    Directory of Open Access Journals (Sweden)

    Clas Linnman

    Full Text Available There are few diagnostic tools for chronic musculoskeletal pain as structural imaging methods seldom reveal pathological alterations. This is especially true for Whiplash Associated Disorder, for which physical signs of persistent injuries to the neck have yet to be established. Here, we sought to visualize inflammatory processes in the neck region by means Positron Emission Tomography using the tracer (11C-D-deprenyl, a potential marker for inflammation. Twenty-two patients with enduring pain after a rear impact car accident (Whiplash Associated Disorder grade II and 14 healthy controls were investigated. Patients displayed significantly elevated tracer uptake in the neck, particularly in regions around the spineous process of the second cervical vertebra. This suggests that whiplash patients have signs of local persistent peripheral tissue inflammation, which may potentially serve as a diagnostic biomarker. The present investigation demonstrates that painful processes in the periphery can be objectively visualized and quantified with PET and that (11C-D-deprenyl is a promising tracer for these purposes.

  3. Complex cytogenetic findings in the bone marrow of a chronic idiopathic myelofibrosis patient

    Directory of Open Access Journals (Sweden)

    Tuğçe Bulakbaşı Balcı

    2010-06-01

    Full Text Available Chronic idiopathic myelofibrosis is a myeloproliferative disorder characterized by splenomegaly, myeloid metaplasia and reactive bone marrow fibrosis. Karyotype analysis of the bone marrow is an integral part of the diagnosis, especially as a discriminative tool in ruling out reactive conditions. The frequency of clonal cytogenetic anomalies in this disease is the highest among its group, varying between 30 and 75%. Among these, trisomy 1q, 20q-, 13q- and +8 are the most common aberrations. Here we report a 66-year-old male patient whose bone marrow biopsy revealed signs of chronic myeloproliferative changes and dysmegakaryopoiesis. He was administered hydroxyurea treatment, splenic radiotherapy and multiple transfusions. The patient worsened in the following months and the second bone marrow biopsy revealed myelofibrosis. Cytogenetic analysis of this bone marrow sample revealed a complex karyotype reported to be 46,XY,del(9(q22q34,t(8;17;21(q22;q21;q22[23]/46,XY[2], with a previously undefined three-way translocation and deletion in chromosome 9. The patient died shortly thereafter.

  4. Prediction of chronic disability in work-related musculoskeletal disorders: a prospective, population-based study

    Directory of Open Access Journals (Sweden)

    Lymp James F

    2004-05-01

    Full Text Available Abstract Background Disability associated with work-related musculoskeletal disorders is an increasingly serious societal problem. Although most injured workers return quickly to work, a substantial number do not. The costs of chronic disability to the injured worker, his or her family, employers, and society are enormous. A means of accurate early identification of injured workers at risk for chronic disability could enable these individuals to be targeted for early intervention to promote return to work and normal functioning. The purpose of this study is to develop statistical models that accurately predict chronic work disability from data obtained from administrative databases and worker interviews soon after a work injury. Based on these models, we will develop a brief instrument that could be administered in medical or workers' compensation settings to screen injured workers for chronic disability risk. Methods This is a population-based, prospective study. The study population consists of workers who file claims for work-related back injuries or carpal tunnel syndrome (CTS in Washington State. The Washington State Department of Labor and Industries claims database is reviewed weekly to identify workers with new claims for work-related back injuries and CTS, and these workers are telephoned and invited to participate. Workers who enroll complete a computer-assisted telephone interview at baseline and one year later. The baseline interview assesses sociodemographic, employment-related, biomedical/health care, legal, and psychosocial risk factors. The follow-up interview assesses pain, disability, and work status. The primary outcome is duration of work disability over the year after claim submission, as assessed by administrative data. Secondary outcomes include work disability status at one year, as assessed by both self-report and work disability compensation status (administrative records. A sample size of 1,800 workers with back

  5. Chronic Unpredictable Stress (CUS)-Induced Anxiety and Related Mood Disorders in a Zebrafish Model: Altered Brain Proteome Profile Implicates Mitochondrial Dysfunction

    OpenAIRE

    Sumana Chakravarty; Bommana R Reddy; Sudhakar, Sreesha R.; Sandeep Saxena; Tapatee Das; Vuppalapaty Meghah; Cherukuvada V Brahmendra Swamy; Arvind Kumar; Idris, Mohammed M.

    2013-01-01

    Anxiety and depression are major chronic mood disorders, and the etiopathology for each appears to be repeated exposure to diverse unpredictable stress factors. Most of the studies on anxiety and related mood disorders are performed in rodents, and a good model is chronic unpredictable stress (CUS). In this study, we have attempted to understand the molecular basis of the neuroglial and behavioral changes underlying CUS-induced mood disorders in the simplest vertebrate model, the zebrafish, D...

  6. Evolution of karyotypes in Philadelphia (Ph/sup 1/) chromosome-negative chronic myelogenous leukemia

    Energy Technology Data Exchange (ETDEWEB)

    Mintz, U.; Vardiman, J.; Golomb, H.M.; Rowley, J.D.

    1979-02-01

    Ten of 55 patients with chronic myelogenous leukemia (CML) diagnosed between 1972 and 1977 were found to lack the Philadelphia (Ph/sup 1/) chromosome. Serial clinical, morphologic, and cytogenetic studies of patients with Ph/sup 1/-negative CML showed that 30% of them had chromosomal abnormalities. Two had an extra chromosome No. 8 at the time of blast crisis, with a morphological picture of myeloblasts in the bone marrow. A third patient had a 6:14 translocation initially. Abnormalities of chromosome No. 14 are frequently seen in lymphoproliferative disorders, and the bone marrow and peripheral blood contained a significant population of lymphoblasts as well as myeloblasts. The median survival for the 10 patients was 19 months. The exact nature of Ph/sup 1/-negative CML is not yet clear; the disease appears to be a distinct entity among the myeloproliferative disorders.

  7. Neoplasias mieloproliferativas: revisão dos critérios diagnósticos e dos aspectos clínicos Myeloproliferative neoplasms: a review of diagnostic criteria and clinical aspects

    Directory of Open Access Journals (Sweden)

    Maria de Lourdes L. F. Chauffaille

    2010-01-01

    Full Text Available As síndromes mieloproliferativas crônicas, atualmente denominadas neoplasias mieloproliferativas (NMP, de acordo com a 4ª. edição da classificação da Organização Mundial da Saúde (OMS, são doenças clonais de célula-tronco hematopoética, nas quais há a proliferação aumentada de uma ou mais das séries mieloides (granulocítica, eritrocítica, megacariocítica ou mastocítica com maturação eficaz. A progressão de todas é caracterizada por fibrose medular ou transformação leucêmica. Pela classificação da OMS, as NMP incluem: leucemia mieloide crônica (LMC, policitemia vera (PV, mielofibrose idiopática crônica (MF, trombocitemia essencial (TE, leucemia neutrofílica crônica (LNC, leucemia eosinofílica crônica não especificada(LEC, mastocitose (M e neoplasia mieloproliferativa inclassificável (NMI. É interessante notar que tanto a LMC (BCR/ABL1 como PV, MF e TE (JAK2 V617F e éxon 12, MPLW515L/K e M (KITD816V tiveram suas bases moleculares desvendadas e apresentam em comum a ativação constitutiva de tirosino-quinase graças às mutações adquiridas pela célula-tronco hematopoética. A mutação JAK2 V617F é observada em mais de 90% dos casos de PV, mas também em cerca de 50%-60% das MF e TE, levando ao questionamento de como uma única lesão molecular desencadeia três manifestações clínicas diversas. Já há evidências de que eventos genéticos e epigenéticos adicionais contribuem para a patogênese, tais como MPLW515L e MPLW515K. No presente manuscrito são apresentados os aspectos clínicos, a fisiopatologia e os critérios diagnósticos das diferentes NMP.Chronic myeloproliferative disorders, currently called myeloproliferative neoplasms (MPN, according to the 4th edition of the World Health Organization (WHO classification are clonal diseases of hematopoietic stem cells, in which there is increased proliferation of the myeloid series (granulocytic, erythrocytic, megakaryocytic series or mast cells

  8. Radioaerosol lung scanning in chronic obstructive pulmonary disease (COPD) and related disorders

    International Nuclear Information System (INIS)

    As a coordinated research project of the International Atomic Energy Agency (IAEA), a multicentre joint study on radioaerosol lung scan using the BARC nebulizer has prospectively been carried out during 1988-1992 with the participation of 10 member countries in Asia [Bangladesh, China, India, Indonesia, Japan, Korea, Pakistan, Philippines, Singapore and Thailand]. The study was designed so that it would primarily cover chronic obstructive pulmonary disease (COPD) and the other related and common pulmonary diseases. The study also included normal controls and asymptomatic smokers. The purposes of this presentation are three fold: firstly, to document the usefulness of the nebulizer and the validity of user's protocol in imaging COPD and other lung diseases; secondly, to discuss scan features of the individual COPD and other disorders studied and thirdly, to correlate scan alterations with radiographic findings. Before proceeding with a systematic analysis of aerosol scan patterns in the disease groups, we documented normal pattern. The next step was the assessment of scan features in those who had been smoking for more than several years but had no symptoms or signs referable to airways. The lung diseases we analyzed included COPD [emphysema, chronic bronchitis, asthma and bronchiectasis], bronchial obstruction, compensatory overinflation and other common lung diseases such as lobar pneumonia, tuberculosis, interstitial fibrosis, diffuse panbronchiolitis, lung edema and primary and metastatic lung cancers. Lung embolism, inhalation bums and glue-sniffer's lung are separately discussed by Dr. Sundram of Singapore elsewhere in this book. The larger portion of this chapter is allocated to the discussion of COPD with a special effort made in sorting out differential scan features. Diagnostic criteria in individual COPD were defined for each category of disease and basic clinical symptoms and signs and pertinent laboratory data as well as radiographic manifestations are

  9. Chronic orthostatic and antiorthostatic restraint induce neuroendocrine, immune and neurophysiological disorders in rats

    Science.gov (United States)

    Assenmacher, I.; Mekaouche, M.; Maurel, D.; Barbanel, G.; Givalois, L.; Boissin, J.; Malaval, F.; Ixart, G.

    rhythmicity of major physiological variables, the loss of normal correlations between ACTH and CORT, and inflammatory-immune hyperreactivity. These pathophysiological disorders may all be parts of a complex chronic stress syndrome.

  10. Gender Difference in Associations between Chronic Temporomandibular Disorders and General Quality of Life in Koreans: A Cross-Sectional Study.

    Directory of Open Access Journals (Sweden)

    Tae-Yoon Kim

    Full Text Available Chronic temporomandibular disorder (TMD is known to have strong correlations with psychological factors and to display gender disparity. However, while chronic TMD is known to affect quality of life, large-scale studies investigating the influence on quality of life by gender are scarce.This cross-sectional study assessed the data of 17,198 participants aged ≥19 years who completed chronic TMD and EuroQol-5 Dimension sections in the 4th Korean National Health and Nutrition Examination Survey (2007-2009. We adjusted for covariates (health behavior, sociodemographic factors in regression analysis for complex sampling design to calculate regression coefficients and 95% CIs for gender difference in the association between chronic TMD and quality of life. We also evaluated which covariates of somatic health, mental health, health behavior, and sociodemographic factors weakened the relationship between TMD and EQ-5D.Prevalence of chronic TMD was 1.6% (men 1.3%, women 1.8%, and chronic TMD persisted to negatively impact quality of life even after adjusting for confounding variables. Low sociodemographic factors and health behavior had a negative effect on quality of life. Somatic health and mental health were most affected by chronic TMD. As for quality of life, women were affected to a greater extent than men by TMD. Women were more affected by osteoarthritis and general mental health (stress, depressive symptoms, and thoughts of suicide, and men by employment.These results imply that chronic diseases and psychological factors are important in chronic TMD, and that there may be physiological and pathological gender differences in TMD.

  11. Usefulness of Low Cardiac Index to Predict Sleep-Disordered Breathing in Chronic Thromboembolic Pulmonary Hypertension.

    Science.gov (United States)

    Orr, Jeremy E; Auger, William R; DeYoung, Pamela N; Kim, Nick H; Malhotra, Atul; Owens, Robert L

    2016-03-15

    Patients with chronic thromboembolic pulmonary hypertension (CTEPH) often have substantial right ventricular dysfunction. The resulting low cardiac index might predispose to sleep disordered breathing (SDB) by increasing ventilatory instability. The prevalence of SDB and potential association with impaired cardiac index was examined in patients with CTEPH. Patients referred for evaluation for pulmonary thromboendarterectomy surgery were recruited. Subjects underwent a sleep study, unless already using positive airway pressure therapy. Hemodynamic data were obtained from contemporaneous right-sided cardiac catheterization. A total of 49 subjects were included. SDB-defined as ongoing positive airway pressure use or apnea-hypopnea index (AHI) ≥5/h-was found in 57% of subjects. SDB was generally mild in severity, with respiratory events mainly consisting of hypopneas. Cardiac index was found to be significantly lower in subjects with SDB than those without (2.19 vs 2.55 L/min/m(2); p = 0.024), whereas no differences were observed in other characteristics. Additionally, cardiac index was independently predictive of AHI. In a subgroup of subjects with an elevated percentage of central events, both cardiac index and lung to finger circulation time correlated with AHI. In conclusion, SDB is prevalent in patients with CTEPH and might decrease with treatments that improve cardiac index. PMID:26805659

  12. Calcimimetics in the chronic kidney disease-mineral and bone disorder.

    Science.gov (United States)

    Bover, Jordi; Aguilar, Armando; Baas, Juan; Reyes, Joselyne; Lloret, Maria-J; Farré, Neus; Olaya, Mayte; Canal, Cristina; Marco, Helena; Andrés, Enric; Trinidad, Pedro; Ballarin, José

    2009-02-01

    Mineral and bone disorders (MBD) are both an early and very common complication of chronic kidney disease (CKD). It is now accepted that they represent a significant risk factor, explaining the high cardiovascular morbidity and mortality in CKD patients. During the last decade, we have been witnessing many advances in the nomenclature, classification, pathophysiology, diagnosis, and treatment of CKD and some of its complications, such as CKD-MBD. The identification of the calcium-sensing receptor (CaSR) involvement in the pathogenesis of primary and secondary hyperparathyroidism (SHPT) and the availability of a new class of drugs called calcimimetics are two outstanding examples. Cinacalcet, the only available calcimimetic, has been shown to be a very effective therapeutic tool in CKD-MBD. Many clinical trials with cinacalcet in hemodialysis patients with SHPT have shown a reduction in parathyroid hormone, calcium (Ca), phosphate (P) and Ca x P product levels, allowing far greater success in reaching therapeutic goals as recommended by international guidelines. Additionally, some studies have shown that the use of cinacalcet may improve other aspects of CKD-MBD, reducing the risk of vascular calcification and parathyroidectomy, among others. Prospective studies on dialysis patients, with hard endpoint data, are currently underway. This review summarizes the most significant aspects of calcimimimetics based on both experimental and clinical results, underlining their possibilities not only for the treatment of isolated SHPT but also for other CKD-MBD related conditions. PMID:19363783

  13. Altered thermal sensitivity in facial skin in chronic whiplash-associated disorders

    Institute of Scientific and Technical Information of China (English)

    Birgitta Haggman-Henrikson; Ewa Lampa; Erik Nordh

    2013-01-01

    There is a close functional relationship between the jaw and neck regions and it has been suggested that trigeminal sensory impairment can follow whiplash injury. Inclusion of manageable routines for valid assessment of the facial sensory capacity is thus needed for comprehensive evaluations of patients exposed to such trauma. The present study investigated facial thermal thresholds in patients with chronic whiplash-associated disorders (WADs) with both a qualitative method and quantitative sensory testing (QST). Ten women with pain and dysfunction following a whiplash injury were compared to 10 healthy age-matched women. Thermal detection thresholds were assessed by qualitative chair-side testing and by QST according to the method-of-limits. Seven test sites in the facial skin (overlying each trigeminal branch bilaterally, and the midpoint of the chin) were examined. The detection warm and cold thresholds were defined as the mean values of 10 individual thresholds. For the WAD patients, the qualitative assessment demonstrated both reduced and increased sensitivity compared to the healthy, whereas QST systematically showed significantly higher detection thresholds (i.e., decreased sensitivity) for both cold and warm stimuli. For the individuals who were assessed as having increased sensitivity in the qualitative assessment, the QST displayed either normal or higher thresholds, i.e., decreased sensitivity. The results suggest that QST is more sensitive for detecting thermal sensory disturbances in the face than a qualitative method. The impaired thermal sensitivity among the patients corroborates the notion of altered thermal detection capacity induced by WAD-related pain.

  14. Mismatch negativity to the patient's own name in chronic disorders of consciousness.

    Science.gov (United States)

    Qin, Pengmin; Di, Haibo; Yan, Xiaodan; Yu, Senming; Yu, Dan; Laureys, Steven; Weng, Xuchu

    2008-12-19

    Previous studies implicated potential value of mismatch negativity (MMN) in predicting recovery of consciousness in patients with disorders of consciousness (DOC). We have adopted a novel MMN evoked by subject's own name (SON), a self-referential stimulus thought to be powerful in evoking residual brain activity, and examined the correlation between the MMN and recovery of consciousness in patients with chronic (>1 month) DOC. Twelve patients and 12 age-matched healthy controls were investigated. The patients were diagnosed as coma (n=4), vegetative state (VS, n=6), and minimally conscious state (MCS, n=2), mainly based on the JFK Coma Recovery Scale-Revised. The SON-evoked MMN (SON-MMN) was present in seven patients. Critically, the presence of SON-MMN was significantly correlated with recovery of consciousness. While four of the five patients (three VS and two coma) showing SON-MMN changed to MCS 3 months later, the rest of the patients (three VS and two coma) without SON-MMN failed to show any clinical improvement. Our study thus illustrates that the subject's own name is effective in evoking MMN in patients with DOC, and that SON-MMN has potential prognostic values in predicting recovery of consciousness. PMID:18938213

  15. Unravelling motor networks in patients with chronic disorders of consciousness: A promising minimally invasive approach.

    Science.gov (United States)

    Naro, Antonino; Leo, Antonino; Buda, Antonio; Manuli, Alfredo; Bramanti, Alessia; Bramanti, Placido; Calabrò, Rocco Salvatore

    2016-09-01

    Behavioral responsiveness and awareness levels correlate with the degree of functional connectivity within cortical-thalamocortical networks, whose breakdown accounts for chronic disorders of consciousness (DOC). Our study was aimed at assessing the role of the primary motor area (M1) and premotor-M1 circuitry dysfunction in motor output deterioration in minimally conscious state (MCS) and unresponsive wakefulness syndrome (UWS) patients. As a control group, we included a healthy subject (HC) sample in the study. We evaluated the effects of different types of transcranial magnetic stimuli over M1 by recording post-stimulus time histogram (PSTH), which includes a series of peaks of unit firing activity that match with D and I-waves, characterizing the descending corticospinal volleys evoked by transcranial magnetic stimuli. As compared to HC, DOC patients showed a dysfunction of intra-M1 and premotor-M1 circuits, which correlated with the Coma Recovery Scale-Revised scorings. Nonetheless, one UWS patient showed a partially preserved premotor-M1 circuitry, paralleled by a severe intra-M1 circuitry dysfunction. Our data suggest that motor unresponsiveness in some DOC patients may be due to a pure motor output failure, as in the functional locked-in syndrome (fLIS), rather than to a premotor-motor connectivity impairment, which instead characterizes MCS and UWS. PMID:27288702

  16. Association of microsatellite instability and chronic obstructive pulmonary disorder in isocyanate-exposed population of Bhopal

    Directory of Open Access Journals (Sweden)

    Protiti Bose

    2012-01-01

    Full Text Available Context: Survivors of the Bhopal gas disaster still suffer from various respiratory ailments. We examined the effects of exposures among a cross-section of current residents suffering from COPD by ISSR-PCR. Aims: Molecular screening of the gas-affected population of Bhopal with COPD for microsatellite instability due to exposure of MIC. Settings and Design: The isocyanate-exposed population of Bhopal city suffering from chronic obstructive pulmonary disorder. Materials and Methods: Inter-(SSR analysis was used to characterize microsatellite instability in 52 MIC victims of Bhopal, suffering from COPD using (CA 8 RG and (CA 8 R[Y-Q] primer. Statistical Analysis Used: Association analyses were performed using regression analysis. Results: The study on the MIC-affected population in Bhopal showed weak association between microsatellite instability and age (r = + 0.37; exposure distance from site (r = −0.44; and smoking status(r = + 0.12; while regression analysis of the above parameters displayed supporting evidence. Conclusions: The high prevalence of smoking coupled with aging and poor living habits threatens, to further increase COPD incidences among this population, highlighting the need for enhanced screening efforts.

  17. Heart rate increment analysis is not effective for sleep-disordered breathing screening in patients with chronic heart failure.

    OpenAIRE

    Damy, Thibaud; d'Ortho, Marie-Pia; Estrugo, Brigitte; Margarit, Laurent; Mouillet, Gauthier; Mahfoud, Mohannad; Roudot-Thoraval, Francoise; Vermes, Emmanuelle; Hittinger, Luc; Roche, Frederic; Macquin-Mavier, Isabelle

    2010-01-01

    Frequency domain analysis of heart rate variation has been suggested as an effective screening tool for sleep-disordered breathing (SDB) in the general population. The aim of this study was to assess this method in patients with chronic congestive heart failure (CHF). We included prospectively 84 patients with stable CHF, left ventricular ejection fraction (LVEF) or =15 h(-1) was diagnosed in 57.4% of patients. Percent VLFI was not correlated with AHI (r = 0.12). Receiver-operating character...

  18. Identification of clusters of individuals relevant to temporomandibular disorders and other chronic pain conditions: the OPPERA study.

    Science.gov (United States)

    Bair, Eric; Gaynor, Sheila; Slade, Gary D; Ohrbach, Richard; Fillingim, Roger B; Greenspan, Joel D; Dubner, Ronald; Smith, Shad B; Diatchenko, Luda; Maixner, William

    2016-06-01

    The classification of most chronic pain disorders gives emphasis to anatomical location of the pain to distinguish one disorder from the other (eg, back pain vs temporomandibular disorder [TMD]) or to define subtypes (eg, TMD myalgia vs arthralgia). However, anatomical criteria overlook etiology, potentially hampering treatment decisions. This study identified clusters of individuals using a comprehensive array of biopsychosocial measures. Data were collected from a case-control study of 1031 chronic TMD cases and 3247 TMD-free controls. Three subgroups were identified using supervised cluster analysis (referred to as the adaptive, pain-sensitive, and global symptoms clusters). Compared with the adaptive cluster, participants in the pain-sensitive cluster showed heightened sensitivity to experimental pain, and participants in the global symptoms cluster showed both greater pain sensitivity and greater psychological distress. Cluster membership was strongly associated with chronic TMD: 91.5% of TMD cases belonged to the pain-sensitive and global symptoms clusters, whereas 41.2% of controls belonged to the adaptive cluster. Temporomandibular disorder cases in the pain-sensitive and global symptoms clusters also showed greater pain intensity, jaw functional limitation, and more comorbid pain conditions. Similar results were obtained when the same methodology was applied to a smaller case-control study consisting of 199 chronic TMD cases and 201 TMD-free controls. During a median 3-year follow-up period of TMD-free individuals, participants in the global symptoms cluster had greater risk of developing first-onset TMD (hazard ratio = 2.8) compared with participants in the other 2 clusters. Cross-cohort predictive modeling was used to demonstrate the reliability of the clusters. PMID:26928952

  19. Occurrence of chronic lymphocytic leukemia in patients with chronic myelogenous leukemia

    OpenAIRE

    Bhattacharyya, Pritish K

    2013-01-01

    Chronic lymphocytic leukemia (CLL) is the most common leukemia of adults in the western world and constitutes about 33% of all leukemia′s. The incidence of CLL increases with age and are more common in older population. Chronic myeloid leukemia (CML) on the contrary occurs in both young adults and elderly and is a chronic myeloproliferative disease that originates from abnormal pluripotent stem cells and results in involvement of multiple hematopoietic lineages, but predominantly myeloid and ...

  20. Chronic widespread musculoskeletal pain, fatigue, depression and disordered sleep in chronic post-SARS syndrome; a case-controlled study

    Directory of Open Access Journals (Sweden)

    Patcai John

    2011-03-01

    Full Text Available Abstract Background The long term adverse effects of Severe Acute Respiratory Syndrome (SARS, a viral disease, are poorly understood. Methods Sleep physiology, somatic and mood symptoms of 22 Toronto subjects, 21 of whom were healthcare workers, (19 females, 3 males, mean age 46.29 yrs.+/- 11.02 who remained unable to return to their former occupation (mean 19.8 months, range: 13 to 36 months following SARS were compared to 7 healthy female subjects. Because of their clinical similarities to patients with fibromyalgia syndrome (FMS these post-SARS subjects were similarly compared to 21 drug free female patients, (mean age 42.4 +/- 11.8 yrs. who fulfilled criteria for fibromyalgia. Results Chronic post-SARS is characterized by persistent fatigue, diffuse myalgia, weakness, depression, and nonrestorative sleep with associated REM-related apneas/hypopneas, an elevated sleep EEG cyclical alternating pattern, and alpha EEG sleep anomaly. Post- SARS patients had symptoms of pre and post-sleep fatigue and post sleep sleepiness that were similar to the symptoms of patients with FMS, and similar to symptoms of patients with chronic fatigue syndrome. Both post-SARS and FMS groups had sleep instability as indicated by the high sleep EEG cyclical alternating pattern rate. The post-SARS group had a lower rating of the alpha EEG sleep anomaly as compared to the FMS patients. The post-SARS group also reported less pre-sleep and post-sleep musculoskeletal pain symptoms. Conclusions The clinical and sleep features of chronic post-SARS form a syndrome of chronic fatigue, pain, weakness, depression and sleep disturbance, which overlaps with the clinical and sleep features of FMS and chronic fatigue syndrome.

  1. Effect on health-related quality of life of a multimodal physiotherapy program in patients with chronic musculoskeletal disorders

    Directory of Open Access Journals (Sweden)

    Cuesta-Vargas Antonio Ignacio

    2013-02-01

    Full Text Available Abstract Background Musculoskeletal disorders are major causes of morbidity in the world, and these conditions have a strong negative influence in terms of health-related quality of life. The purpose of this study was to evaluate the effect of an 8-week multimodal physical therapy program on general health state and health-related quality of life in patients with chronic musculoskeletal disorders. Methods There were 244 participants in this prospective cohort analysis with 8-week follow-up. The primary outcome was general health state (physical and mental components, determined with the Short Form-12 Health Survey (SF-12. The secondary outcome was health related quality of life, determined with the EuroQoL-5D and VAS. The intervention was evaluated by comparing pre- and post-outcome measurements. T-tests were performed for paired data. Results Differences were statistically significant for physical health state: +1.68 (p  Conclusions Eight weeks of a Multimodal Physical Therapy Program seemed to moderately enhance the general health state and HRQoL of patients with chronic musculoskeletal diseases. This kind of therapeutic exercise can be recommended to patients with chronic low back pain, chronic neck pain and osteoarthritis, at least in the short term.

  2. Magnetic Acupressure in Reducing Pain in Cancer Patients Undergoing Bone Marrow Aspiration and Biopsy

    Science.gov (United States)

    2010-04-09

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  3. Opioid Titration Order Sheet or Standard Care in Treating Patients With Cancer Pain

    Science.gov (United States)

    2012-08-04

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  4. Personalized Information or Basic Information in Helping Patients Make Decisions About Participating in a Clinical Trial

    Science.gov (United States)

    2013-08-20

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Unspecified Adult Solid Tumor, Protocol Specific

  5. Music in Reducing Anxiety and Pain in Adult Patients Undergoing Bone Marrow Biopsy for Hematologic Cancers or Other Diseases

    Science.gov (United States)

    2012-07-12

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Psychosocial Effects of Cancer and Its Treatment

  6. Methadone, Morphine, or Oxycodone in Treating Pain in Patients With Cancer

    Science.gov (United States)

    2012-11-09

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Pain; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  7. Update on JAK2 Inhibitors in Myeloproliferative Neoplasm

    OpenAIRE

    Chan, Daniel; Koren-Michowitz, Maya

    2011-01-01

    Since the discovery of mutant Janus Kinase 2 (JAK2), JAK2 V617F, in a major proportion of myeloproliferative neoplasm (MPN) patients, there has been a flurry of activity in the development of JAK2 inhibitors. Pan-JAK, predominantly JAK2 and off-target JAK2 inhibitors have been developed in the short span of the past 5 years. These compounds have since been tested to varying success in both in vitro and in vivo settings with several proceeding on to advanced clinical trials. Although it was ho...

  8. Chronic idiopathic urticaria and post-traumatic stress disorder (PTSD): an under-recognized comorbidity.

    Science.gov (United States)

    Gupta, Madhulika A; Gupta, Aditya K

    2012-01-01

    A large body of literature supports the role of psychologic stress in urticaria; however, the comorbidity between chronic idiopathic urticaria (CIU) and post-traumatic stress disorder (PTSD), a classic stress-mediated syndrome, has received little attention. The underlying etiology of urticaria is not identifiable in about 70% of patients, possibly because of difficulties with identification of a direct cause-and-effect relationship between a potential causative factor and the onset of urticaria. The core features of PTSD (Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision [DSMIV-TR]) that are important in urticaria include (1) autonomic nervous system reactivity and state of sympathetic hyperarousal that can manifest as CIU, and (2) the persistent re-experiencing of the traumatic events in PTSD, which can manifest as urticaria or angioedema, or both, affecting a previously traumatized body region (eg, urticarial wheals affecting the body region where the patient had been stabbed years earlier). The following features of PTSD make it difficult to use the cause-and-effect model for the determination of causation: (1) PTSD may first emerge years after the initial trauma and is classified as PTSD with Delayed Onset (DSMIV-TR); and (2) the traumatic triggers that precipitate the PTSD symptoms may be unique and idiosyncratic to the patient and not even qualify as stressful or traumatic by standard criteria (eg, precipitating events for the PTSD may include smell of a certain cologne that was used by the perpetrator or witnessing a scene in a movie that was reminiscent of the location where the abuse occurred). Finally, in PTSD with Delayed Onset, patients may not make a conscious association between their recurrent urticaria and their earlier traumas because they can develop classically conditioned associations between stimuli that are reminiscent of the original abuse situation and their somatic reactions such as urticaria. The clinician

  9. Chronic fatigue syndrome and seasonal affective disorder: comorbidity, diagnostic overlap, and implications for treatment.

    Science.gov (United States)

    Terman, M; Levine, S M; Terman, J S; Doherty, S

    1998-09-28

    This study aimed to determine symptom patterns in patients with chronic fatigue syndrome (CFS), in summer and winter. Comparison data for patients with seasonal affective disorder (SAD) were used to evaluate seasonal variation in mood and behavior, atypical neurovegetative symptoms characteristic of SAD, and somatic symptoms characteristic of CFS. Rating scale questionnaires were mailed to patients previously diagnosed with CFS. Instruments included the Personal Inventory for Depression and SAD (PIDS) and the Systematic Assessment for Treatment Emergent Effects (SAFTEE), which catalogs the current severity of a wide range of somatic, behavioral, and affective symptoms. Data sets from 110 CFS patients matched across seasons were entered into the analysis. Symptoms that conform with the Centers for Disease Control and Prevention (CDC) case definition of CFS were rated as moderate to very severe during the winter months by varying proportions of patients (from 43% for lymph node pain or enlargement, to 79% for muscle, joint, or bone pain). Fatigue was reported by 92%. Prominent affective symptoms included irritability (55%), depressed mood (52%), and anxiety (51%). Retrospective monthly ratings of mood, social activity, energy, sleep duration, amount eaten, and weight change showed a coherent pattern of winter worsening. Of patients with consistent summer and winter ratings (n = 73), 37% showed high global seasonality scores (GSS) > or = 10. About half this group reported symptoms indicative of major depressive disorder, which was strongly associated with high seasonality. Hierarchical cluster analysis of wintertime symptoms revealed 2 distinct clinical profiles among CFS patients: (a) those with high seasonality, for whom depressed mood clustered with atypical neurovegetative symptoms of hypersomnia and hyperphagia, as is seen in SAD; and (b) those with low seasonality, who showed a primary clustering of classic CFS symptoms (fatigue, aches, cognitive disturbance

  10. The relation of hepcidin to iron disorders, inflammation and hemoglobin in chronic kidney disease.

    Directory of Open Access Journals (Sweden)

    Lucile Mercadal

    Full Text Available The metabolism of hepcidin is profoundly modified in chronic kidney disease (CKD. We investigated its relation to iron disorders, inflammation and hemoglobin (Hb level in 199 non-dialyzed, non-transplanted patients with CKD stages 1-5. All had their glomerular filtration rate measured by 51Cr-EDTA renal clearance (mGFR, as well as measurements of iron markers including hepcidin and of erythropoietin (EPO. Hepcidin varied from 0.2 to 193 ng/mL. The median increased from 23.3 ng/mL [8.8-28.7] to 36.1 ng/mL [14.1-92.3] when mGFR decreased from ≥60 to <15 mL/min/1.73 m2 (p = 0.02. Patients with absolute iron deficiency (transferrin saturation (TSAT <20% and ferritin <40 ng/mL had the lowest hepcidin levels (5.0 ng/mL [0.7-11.7], and those with a normal iron profile (TSAT ≥20% and ferritin ≥40, the highest (34.5 ng/mL [23.7-51.6]. In multivariate analysis, absolute iron deficiency was associated with lower hepcidin values, and inflammation combined with a normal or functional iron profile with higher values, independent of other determinants of hepcidin concentration, including EPO, mGFR, and albuminemia. The hepcidin level, although it rose overall when mGFR declined, collapsed in patients with absolute iron deficiency. There was a significant interaction with iron status in the association between Hb and hepcidin. Except in absolute iron deficiency, hepcidin's negative association with Hb level indicates that it is not down-regulated in CKD anemia.

  11. Pulmonary alveolar proteinosis with myeloproliferative syndrome with myelodysplasia: bronchoalveolar lavage reduces white blood cell count.

    Science.gov (United States)

    Pollack, Seth M; Gutierrez, Guillermo; Ascensao, Joao

    2006-08-01

    Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by surfactant component accumulation in the alveolar space. Primary PAP is likely an autoimmune disorder caused by antibodies to granulocyte-macrophage colony stimulating factor (GM-CSF). When an underlying disease causes PAP, this is called secondary PAP. Hematologic malignancies are an important cause of secondary PAP. As the pathogenesis of primary PAP has become more fully understood, improvements in diagnostic and therapeutic approaches have followed. However, when PAP is secondary to an underlying hematologic malignancy, much remains unclear. Here we describe for the first time a patient with hybrid myelodysplastic syndrome/myeloproliferative syndrome and PAP who had a marked decrease in her white blood cell count following a transbronchial biopsy accompanied by bronchoalveolar lavage (BAL). Similar significant decreases in WBC count accompanied clinical improvement following two unilateral BALs. Given that patients with pulmonary alveolar proteinosis frequently have elevated GM-CSF in bronchoalveolar fluid, this observation provides a unique vantage point to understand the pathophysiology of secondary PAP. PMID:16906593

  12. Neuropathological Changes and Clinical Features of Autism Spectrum Disorder Participants Are Similar to that Reported in Congenital and Chronic Cerebral Toxoplasmosis in Humans and Mice

    Science.gov (United States)

    Prandota, Joseph

    2010-01-01

    Anatomic, histopathologic, and MRI/SPET studies of autistic spectrum disorders (ASD) patients' brains confirm existence of very early developmental deficits. In congenital and chronic murine toxoplasmosis several cerebral anomalies also have been reported, and worldwide, approximately two billion people are chronically infected with T. "gondii"…

  13. Differences in maladaptive schemas between patients suffering from chronic and acute posttraumatic stress disorder and healthy controls

    Directory of Open Access Journals (Sweden)

    Ahmadian A

    2015-07-01

    Full Text Available Alireza Ahmadian,1,2 Jafar Mirzaee,1 Maryam Omidbeygi,1 Edith Holsboer-Trachsler,3 Serge Brand3,41Department of Psychology, Kharazmi University, 2Sadr Psychiatric Hospital, Janbazan Medical and Engineering Research Center (JMERC, Tehran, Iran; 3Psychiatric Clinics of the University of Basel, Center for Affective, Stress and Sleep Disorders, 4Department of Sport, Exercise and Health, Sport Science Section, University of Basel, Basel, Switzerland Background: War, as a stressor event, has a variety of acute and chronic negative consequences, such as posttraumatic stress disorder (PTSD. In this context, early maladaptive schema-based problems in PTSD have recently become an important research area. The aim of this study was to assess early maladaptive schemas in patients with acute and chronic PTSD.Method: Using available sampling methods and diagnostic criteria, 30 patients with chronic PTSD, 30 patients with acute PTSD, and 30 normal military personnel who were matched in terms of age and wartime experience were selected and assessed with the Young Schema Questionnaire-Long Form, Beck Depression Inventory second version (BDI-II, the Beck Anxiety Inventory (BAI, and the Impact of Events Scale (IES.Results: Both acute and chronic PTSD patients, when compared with normal military personnel, had higher scores for all early maladaptive schemas. Additionally, veterans suffering from chronic PTSD, as compared with veterans suffering from acute PTSD and veterans without PTSD, reported more impaired schemas related, for instance, to Self-Control, Social Isolation, and Vulnerability to Harm and Illness.Discussion: The results of the present study have significant preventative, diagnostic, clinical, research, and educational implications with respect to PTSD. Keywords: veterans, PTSD, depression, anxiety 

  14. Clofarabine, Cytarabine, and Filgrastim in Treating Patients With Newly Diagnosed Acute Myeloid Leukemia, Advanced Myelodysplastic Syndrome, and/or Advanced Myeloproliferative Neoplasm

    Science.gov (United States)

    2015-12-28

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Refractory Anemia With Excess Blasts; Untreated Adult Acute Myeloid Leukemia; Myeloproliferative Neoplasm With 10% Blasts or Higher

  15. Molecular mechanisms associated with leukemic transformation of MPL-mutant myeloproliferative neoplasms

    DEFF Research Database (Denmark)

    Beer, Philip A; Ortmann, Christina A; Stegelmann, Frank;

    2010-01-01

    Somatic activating mutations in MPL, the thrombopoietin receptor, occur in the myeloproliferative neoplasms, although virtually nothing is known about their role in evolution to acute myeloid leukemia. In this study, the MPL T487A mutation, identified in de novo acute myeloid leukemia......, was not detected in 172 patients with a myeloproliferative neoplasm. In patients with a prior MPL W515L-mutant myeloproliferative neoplasm, leukemic transformation was accompanied by MPL-mutant leukemic blasts, was seen in the absence of prior cytoreductive therapy and often involved loss of wild-type MPL...

  16. Vulnerability to chronic subordination stress-induced depression-like disorders in adult 129SvEv male mice.

    Science.gov (United States)

    Dadomo, Harold; Sanghez, Valentina; Di Cristo, Luisana; Lori, Andrea; Ceresini, Graziano; Malinge, Isabelle; Parmigiani, Stefano; Palanza, Paola; Sheardown, Malcolm; Bartolomucci, Alessandro

    2011-08-01

    Exposure to stressful life events is intimately linked with vulnerability to neuropsychiatric disorders such as major depression. Pre-clinical animal models offer an effective tool to disentangle the underlying molecular mechanisms. In particular, the 129SvEv strain is often used to develop transgenic mouse models but poorly characterized as far as behavior and neuroendocrine functions are concerned. Here we present a comprehensive characterization of 129SvEv male mice's vulnerability to social stress-induced depression-like disorders and physiological comorbidities. We employed a well characterized mouse model of chronic social stress based on social defeat and subordination. Subordinate 129SvEv mice showed body weight gain, hyperphagia, increased adipose fat pads weight and basal plasma corticosterone. Home cage phenotyping revealed a suppression of spontaneous locomotor activity and transient hyperthermia. Subordinate 129SvEv mice also showed marked fearfulness, anhedonic-like response toward a novel but palatable food, increased anxiety in the elevated plus maze and social avoidance of an unfamiliar male mouse. A direct measured effect of the stressfulness of the living environment, i.e. the amount of daily aggression received, predicted the degree of corticosterone level and locomotor activity but not of the other parameters. This is the first study validating a chronic subordination stress paradigm in 129SvEv male mice. Results demonstrated remarkable stress vulnerability and establish the validity to use this mouse strain as a model for depression-like disorders. PMID:21093519

  17. Impact of posttraumatic stress disorder on sinonasal symptoms and quality of life in patients with chronic rhinosinusitis

    Directory of Open Access Journals (Sweden)

    Shejbal D

    2012-11-01

    Full Text Available Dražen Shejbal,1 Davor Vagic, 2 Siniša Stevanovic, 3 Elvira Koic, 4 Livije Kalogjera21Division of Otorhinolaryngology, Pakrac City Hospital, Pakrac, Croatia; 2Department of Otorhinolaryngology and Head and Neck Surgery, Sisters of Mercy Hospital, Zagreb, Croatia; 3Division of Otorhinolaryngology, 4Division of Psychiatry, Virovitica General Hospital, Virovitica, CroatiaBackground: Severity of chronic rhinosinusitis (CRS, measured by disease-specific health-related quality-of-life questionnaires, is expected to increase in patients who also suffer from posttraumatic stress disorder (PTSD. Altered pain perception, sleep disorders, and fatigue may be associated with this comorbidity.Methods: Severity of CRS was compared between a group of 28 patients with CRS and a group of 28 patients with CRS and concomitant PTSD using different disease-specific and generic instruments, such as visual analog scale (VAS, Short Form-36 test (SF-36, and Sino-Nasal Outcome Test-22 (SNOT 22.Results: SNOT-22 test showed significantly higher CRS severity in patients with CRS and PTSD, compared to patients with CRS without PTSD.Conclusion: Patients with less severe CRS, measured by objective outcome measures, due to the impact of comorbid PTSP, are classified as having severe rhinosinusitis, and are exposed to the risk of unnecessary diagnostic and therapeutic procedures. In patients with difficult-to-treat rhinosinusitis, diagnosis should be revised, and one item that should be evaluated is whether they suffer from PTSD.Keywords: posttraumatic stress disorder, chronic rhinosinusitis, quality of life

  18. Comorbid mental disorders account for the role impairment of commonly occurring chronic physical disorders: Results from the national comorbidity survey

    NARCIS (Netherlands)

    Kessler, R.C.; Ormel, J.; Demler, O.; Stang, P.E.

    2003-01-01

    Most health and work productivity studies have focused on individual conditions without considering comorbidity. We illustrate the implication of this neglect by examining the effects of comorbid mental disorders on role impairment (number of sickness absence and work cut-back days in the past month

  19. Substance P at the Nexus of Mind and Body in Chronic Inflammation and Affective Disorders

    Science.gov (United States)

    Rosenkranz, Melissa A.

    2007-01-01

    For decades, research has demonstrated that chronic diseases characterized by dysregulation of inflammation are particularly susceptible to exacerbation by stress and emotion. Likewise, rates of depression and anxiety are overrepresented in individuals suffering from chronic inflammatory disease. In recent years, substance P has been implicated in…

  20. Peculiarities of coagulation hemostasis disorders in patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Yakovlieva V.H.

    2015-09-01

    Full Text Available The violation of blood coagulation properties is observed in many diseases of the respiratory system, including chronic obstructive pulmonary disease (COPD. It is known that in a stable COPD phase release of proinflammatory cytokines in blood is observed, it leads to disturbance of hemostasis parameters toward hypercoagulation. The aim of our study was to determine possibility of coagulation disorders formation in patients with COPD in a stable phase of pathological process at different stages of disease course and identify relationship between coagulation parameters levels and degree of violation of ventilation lung function. Materials and methods. We examined 30 patients with COPD in a stable disease phase, included in the main group (FEV1 =49,5±15,5% of the due, there were 27 men (90.0%, 3 (30.0% women, mean age was 61,8±7,9 years, level of pack /years index - 34,2±15,3. The control group consisted of 10 healthy subjects matched with the patients of the main group by age and sex. All patients were divided into 2 subgroups. Subgroup 1 included 16 patients with moderate COPD, that is the level of FEV1>50% (61,8±7,4% of predicted, and subgroup 2 - 14 COPD patients with severe COPD, that is the level of FEV1 <50% (35,3±8,2% of predicted. Patients received standard treatment according to the disease stage. Main indicators of coagulation levels: prothrombin index (PI, prothrombin ratio (PR, international normalized ratio (INR, activated partial thromboplastin time (APTT, thrombin time (TT and antithrombin III (AT III were identified in all patients. Results. The levels of PI, PR and INR in the subgroup 1 differed significantly from those of in the subgroup 2, control group (p<0,05 and pointed at hypercoagulation, whereas in the subgroup 2 all indicators were absolutely identical with control group. Correlation link between the level of INR levels and FEV1 (r=-0,73; p<0.01 in patients of the main group was determined. Levels of APTT, TT and AT

  1. Subjective health complaints in patients with chronic Whiplash Associated Disorders (WAD. Relationships with physical, psychological, and collision associated factors

    Directory of Open Access Journals (Sweden)

    Camilla Ihlebæk

    2009-10-01

    Full Text Available  Aims: Investigate subjective health complaints (SHC in chronic whiplash associated disorder (WAD, grade I & II patients, and to identify physical, psychological, and collision associated factors that might be associated with high levels of comorbidity. Method: During the years 2000-2002 171 chronic WAD patients filled in questionnaires and underwent physical examination. The prevalence of SHC was recorded and compared with a representative sample of the Norwegian population (n=1014. Results: The chronic WAD patients reported higher number of subjective health complaints (median: 9 than the general population (median: 5. They showed significantly higher risk of reporting all musculoskeletal complaints, palpitation, heat flushes, sleep problems, tiredness, dizziness, anxiety, depression, breathing difficulties, chest pain, coughing, heartburn, gas discomfort, and obstipation. The patients with the highest level of comorbid subjective health complaints also reported more function loss, reading difficulties, poorer quality of life, higher psychological distress, higher use of medication, and less optimism about their situation. There were no differences however, in any collision factors or physical meassures recorded by physiotherapists between the high, medium and low comorbidity groups. Conclusion: The high comorbidity of other complaints, the strong relationships between degree of comorbidity and psychological factors, and the lack of relationships between degree of comorbidity and collision factors and physical tests, suggest that chronic WAD is best understood as a syndrome and not simply as a neck injury. Sensitization is suggested as a possible psychobiological mechanism

  2. Chronic pain patients with possible co-morbid post-traumatic stress disorder admitted to multidisciplinary pain rehabilitation

    DEFF Research Database (Denmark)

    Andersen, Tonny Elmose; Andersen, Lou-Ann Christensen; Andersen, Per Grünwald

    2014-01-01

    BACKGROUND: Although post-traumatic stress disorder (PTSD) is a common co-morbidity in chronic pain, little is known about the association between PTSD and pain in the context of chronic pain rehabilitation. OBJECTIVE: The aim of the present study was two-fold: (1) to investigate the association of...... consecutively referred cohort of 194 patients completed a baseline questionnaire at admission covering post-traumatic stress, pain symptoms, physical and mental functioning, as well as self-reported sleep quality and cognitive difficulties. Medication use was calculated from their medical records. A total of 95...... well as inferior social functioning compared to patients without PTSD. Possible co-morbid PTSD did not result in higher use of opioids or sedatives. Surprisingly, possible co-morbid PTSD at admission was not associated with lower levels of symptom reduction from pre- to post-treatment. CONCLUSIONS...

  3. A Brief Historic Overview of Clinical Disorders Associated with Tryptophan: The Relevance to Chronic Fatigue Syndrome (CFS) and Fibromyalgia (FM).

    Science.gov (United States)

    Blankfield, Adele

    2012-01-01

    Last century there was a short burst of interest in the tryptophan related disorders of pellagra and related abnormalities that are usually presented in infancy.1,2 Nutritional physiologists recognized that a severe human dietary deficiency of either tryptophan or the B group vitamins could result in central nervous system (CNS) sequelae such as ataxia, cognitive dysfunction and dysphoria, accompanied by skin hyperpigmentation.3,4 The current paper will focus on the emerging role of tryptophan in chronic fatigue syndrome (CFS) and fibromyalgia (FM). PMID:23032646

  4. Immunoglobulin D multiple myeloma, plasma cell leukemia and chronic myelogenous leukemia in a single patient treated simultaneously with lenalidomide, bortezomib, dexamethasone and imatinib

    Directory of Open Access Journals (Sweden)

    Naveed Ali

    2016-03-01

    Full Text Available Multiple myeloma (MM is a neoplastic lymphoproliferative disorder characterized by uncontrolled monoclonal plasma cell proliferation. Among different isotypes of MM, immunoglobulin D (IgD MM is very rare, representing only 1 to 2% of all isotypes. Chronic myelogenous leukemia (CML is a neoplastic myeloproliferative disorder of pluripotent hematopoietic stem cell, which is characterized by the uncontrolled proliferation of myeloid cells. An 88-year-old male was diagnosed simultaneously with IgD kappa MM and CML. A distinctive feature in this patient was the progression to plasma cell leukemia without any symptomatic myeloma stage. He was treated simultaneously with lenalidomide, bortezomib and imatinib. Synchronous occurrence of these rare hematological malignancies in a single patient is an exceedingly rare event. Multiple hypotheses to explain co-occurrence of CML and MM have been proposed; however, the exact etiological molecular pathophysiology remains elusive.

  5. WHO-defined Classification of Myeloproliferative Neoplasms: Morphological Reproducibility and Clinical Correlations - The Danish Experience

    DEFF Research Database (Denmark)

    Madelung, AB; Stamp, IMH; Nielsen, SL;

    2013-01-01

    We examined inter- and intraobserver reproducibility and concordance between histological diagnosis and independently collected clinical findings in a large series of patients with the major subtypes of myeloproliferative neoplasms (MPNs) and controls. Seven hematopathologists reviewed 272 bone...

  6. The role of inflammatory cytokines and ERK1/2 signaling in chronic prostatitis/chronic pelvic pain syndrome with related mental health disorders.

    Science.gov (United States)

    Hu, Chao; Yang, Hualan; Zhao, Yanfang; Chen, Xiang; Dong, Yinying; Li, Long; Dong, Yehao; Cui, Jiefeng; Zhu, Tongyu; Zheng, Ping; Lin, Ching-Shwun; Dai, Jican

    2016-01-01

    Mental health disorders(MHD) in chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) have been widely studied. However, the underlying role of inflammatory cytokines and their associated signaling pathways have not been investigated. Here, we report the potential role of cytokines and associated signaling pathways in CP/CPPS patients with MHD and in a CP/CPPS animal model. CP/CPPS patients (n = 810) and control subjects (n = 992) were enrolled in this case-control multicenter study, and serum cytokine levels were measured. Male Sprague-Dawley rats received multiple intracutaneous injections of an immuno-agent along with a pertussis-diphtheria-tetanus triple vaccine for autoimmune CP/CPPS development. The results revealed that, in CP/CPPS patients with significant MHD, elevated IL-1α, IL-1β, IL-4, IL-13, and TNF-α serum levels were observed. The above five cytokines in CP/CPPS rats were significantly elevated in prostate tissue (p MHD. PMID:27334333

  7. Contribution of an aged microenvironment to aging-associated myeloproliferative disease.

    Directory of Open Access Journals (Sweden)

    Virag Vas

    Full Text Available The molecular and cellular mechanisms of the age-associated increase in the incidence of acute myeloid leukemia (AML remain poorly understood. Multiple studies support that the bone marrow (BM microenvironment has an important influence on leukemia progression. Given that the BM niche itself undergoes extensive functional changes during lifetime, we hypothesized that one mechanism for the age-associated increase in leukemia incidence might be that an aged niche promotes leukemia progression. The most frequent genetic alteration in AML is the t(8;21 translocation, resulting in the expression of the AML1-ETO fusion protein. Expression of the fusion protein in hematopoietic cells results in mice in a myeloproliferative disorder. Testing the role of the age of the niche on leukemia progression, we performed both transplantation and in vitro co-culture experiments. Aged animals transplanted with AML1-ETO positive HSCs presented with a significant increase in the frequency of AML-ETO positive early progenitor cells in BM as well as an increased immature myeloid cell load in blood compared to young recipients. These findings suggest that an aged BM microenvironment allows a relative better expansion of pre-leukemic stem and immature myeloid cells and thus imply that the aged microenvironment plays a role in the elevated incidence of age-associated leukemia.

  8. New Strategies in Myeloproliferative Neoplasms: The Evolving Genetic and Therapeutic Landscape.

    Science.gov (United States)

    Patel, Ami B; Vellore, Nadeem A; Deininger, Michael W

    2016-03-01

    The classical BCR-ABL1-negative myeloproliferative neoplasms (MPN) include essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF). Although these clonal disorders share certain clinical and genetic features, MF in particular is distinct for its complex mutational landscape, severe disease phenotype, and poor prognosis. The genetic complexity inherent to MF has made this disease extremely challenging to treat. Pharmacologic JAK inhibition has proven to be a transformative therapy in MPNs, alleviating symptom burden and improving survival, but has been hampered by off-target toxicities and, as monotherapy, has shown limited effects on mutant allele burden. In this review, we discuss the genetic heterogeneity contributing to the pathogenesis of MPNs, focusing on novel driver and epigenetic mutations and how they relate to combination therapeutic strategies. We discuss results from ongoing studies of new JAK inhibitors and report on new drugs and drug combinations that have demonstrated success in early preclinical and clinical trials, including type II JAK inhibitors, antifibrotic agents, and telomerase inhibitors. PMID:26933174

  9. Methylation of SOCS3 in Myeloproliferative Neoplasms and Secondary Erythrocytosis/Thrombocythemia

    Directory of Open Access Journals (Sweden)

    Deniz Torun

    2013-03-01

    Full Text Available OBJECTIVE: Myeloproliferative neoplasms (MPNs like essential thrombocythemia (ET, polycythemia vera (PV, and primary myelofibrosis (PMF are acquired clonal hematopoietic stem cell disorders and originate from a multipotent hematopoietic stem cell. The SOCS1 and SOCS3 genes are negative regulators of the JAK/STAT signal pathway. In this study we investigate the promoter methylation of these genes in the pathogenesis of MPNs and secondary erythrocytosis/thrombocythemia. METHODS: Promoter methylation of SOCS1 and SOCS3 genes was analyzed with methylation-specific PCR. PCR products were analyzed by agarose gel electrophoresis. RESULTS: No disease-specific CpG island methylation of SOCS1 was observed. Hypermethylation of the SOCS3 promoter was identified in 5 out of 19 (26.3% PV cases, 2 out of 21 (9.5% ET cases, 1 out of 5 (20% PMF cases, and 9 out of 42 (21.4% cases of secondary erythrocytosis/thrombocythemia. CONCLUSION: The results revealed that promoter methylation of the SOCS3 gene suggests a possible role for SOCS3 methylation in the pathogenesis of MPNs and secondary erythrocytosis/thrombocythemia.

  10. Severity of Anxiety Disorders in Patients with Chronic Obstructive Pulmonary Disease

    OpenAIRE

    Mitra Safa; Saeed Fallah-Tafti; Firrouzeh Talischi; Fatemeh Ghassem-Boroujerdi

    2015-01-01

    Objective: Patients with chronic physical diseases sometimes show increased loss of function; such patients need more care. Anxiety is a well-known symptom that is prevalent among chronic obstructive pulmonary disease patients that can prolong and increase the risk of hospitalization. The purpose of this study was to evaluate the severity of anxiety in the mentioned patients and to examine the presence of symptoms and appropriate treatment strategies to understand the role of psychological fu...

  11. The Investigation of Nail Disorders in Patients with Chronic Renal Failure Undergoing Hemodialysis

    OpenAIRE

    Perihan Öztürk; Neslihan Dokur; Ergül Kurutaş; Ekrem Doğan; Tuğba Karakaş; Murat Kalender; Hasan Ekerbiçer

    2012-01-01

    Objective: Nail changes are often observed in patients with end-stage renal disease. These changes may occur due to chronic renal failure itself or to the treatment. This study aims to investigate the frequency of nail findings in patients undergoing hemodialysis therapy and to compare with healthy controls. Methods: One hundred and four patients with chronic renal failure treated with hemodialysis, and 104 healthy controls without any dermatological and sistemic diseases, were examined fo...

  12. Influenza hemagglutination inhibiting activity in respiratory mucus from horses with chronic obstructive pulmonary disorders (heaves syndrome).

    OpenAIRE

    Thorsen, J; Willoughby, R A; McDonell, W; Valli, V. E.; Viel, L; Bignell, W

    1983-01-01

    Samples of mucus from the lower trachea were collected from 53 horses with chronic obstructive pulmonary disease and from 24 clinically normal horses. Serum samples were collected from 35 of the horses with chronic obstructive pulmonary disease and from the 24 normal horses. Samples were tested for inhibition of hemagglutination by influenza A equine 1 and 2 viruses. There were high levels of hemagglutination inhibiting activity against influenza A equine 1 in mucus samples from horses with c...

  13. Autism Spectrum Disorders May Be Due to Cerebral Toxoplasmosis Associated with Chronic Neuroinflammation Causing Persistent Hypercytokinemia that Resulted in an Increased Lipid Peroxidation, Oxidative Stress, and Depressed Metabolism of Endogenous and Exogenous Substances

    Science.gov (United States)

    Prandota, Joseph

    2010-01-01

    Worldwide, approximately 2 billion people are chronically infected with "Toxoplasma gondii" with largely yet unknown consequences. Patients with autism spectrum disorders (ASD) similarly as mice with chronic toxoplasmosis have persistent neuroinflammation, hypercytokinemia with hypermetabolism associated with enhanced lipid peroxidation, and…

  14. Mouse models of myeloproliferative neoplasms: JAK of all grades

    Directory of Open Access Journals (Sweden)

    Juan Li

    2011-05-01

    Full Text Available In 2005, several groups identified a single gain-of-function point mutation in the JAK2 kinase that was present in the majority of patients with myeloproliferative neoplasms (MPNs. Since this discovery, much effort has been dedicated to understanding the molecular consequences of the JAK2V617F mutation in the haematopoietic system. Three waves of mouse models have been produced recently (bone marrow transplantation, transgenic and targeted knock-in, which have facilitated the understanding of the molecular pathogenesis of JAK2V617F-positive MPNs, providing potential platforms for designing and validating novel therapies in humans. This Commentary briefly summarises the first two types of mouse models and then focuses on the more recently generated knock-in models.

  15. Phase I Dose-Escalation Trial of Clofarabine Followed by Escalating Doses of Fractionated Cyclophosphamide in Children With Relapsed or Refractory Acute Leukemias

    Science.gov (United States)

    2010-09-21

    Myelodysplastic Syndrome; Acute Myeloid Leukemia; Myeloproliferative Disorders; Acute Lymphocytic Leukemia; Acute Promyelocytic Leukemia; Acute Leukemia; Chronic Myelogenous Leukemia; Myelofibrosis; Chronic Myelomonocytic Leukemia; Juvenile Myelomonocytic Leukemia

  16. T-Cell Depleted Allogeneic Stem Cell Transplantation for Patients With Hematologic Malignancies

    Science.gov (United States)

    2015-10-05

    Acute Myelogenous Leukemia; Lymphoid Leukemia; Chronic Myelogenous Leukemia; Malignant Lymphoma; Hodgkin's Disease; Chronic Lymphocytic Leukemia; Myeloproliferative Disorder; Anemia, Aplastic; Myelodysplastic Syndromes

  17. Differences in {sup 99m}Tc-HMPAO brain SPET perfusion imaging between Tourette's syndrome and chronic tic disorder in children

    Energy Technology Data Exchange (ETDEWEB)

    Chiu, N.-T.; Lee, B.-F. [Dept. of Nuclear Medicine, College of Medicine, National Cheng Kung University, Tainan, Taiwan (Taiwan); Chang, Y.-C. [Dept. of Pediatrics, Kaohsiung Chang Kang Children' s Hospital, Kaohsiung, Taiwan (Taiwan); Huang, C.-C. [Dept. of Pediatrics, College of Medicine, National Cheng Kung University, Tainan (Taiwan); Wang, S.-T. [Dept. of Public Health, College of Medicine, National Cheng Kung University, Tainan, Taiwan (Taiwan)

    2001-02-01

    Early differential diagnosis between Tourette's syndrome and chronic tic disorder is difficult but important because both the outcome and the treatment of these two childhood-onset diseases are distinct. We assessed the sensitivity and specificity of brain single-photon emission tomography (SPET) perfusion imaging in distinguishing the two diseases, and characterized their different cerebral perfusion patterns. Twenty-seven children with Tourette's syndrome and 11 with chronic tic disorder (mean age 9.5 and 8.6 years, respectively) underwent brain SPET with technetium-99m hexamethylpropylene amine oxime (HMPAO). Visual interpretation and semi-quantitative analysis of SPET images were performed. On visual interpretation, 22 of 27 (82%) of the Tourette's syndrome group had lesions characterized by decreased perfusion. The left hemisphere was more frequently involved. None of the children with chronic tic disorder had a visible abnormality. Semi-quantitative analysis showed that, compared with children with chronic tic disorder, children with Tourette's syndrome had significantly lower perfusion in the left lateral temporal area and asymmetric perfusion in the dorsolateral frontal, lateral and medial temporal areas. In conclusion, using the visual approach, brain SPET perfusion imaging is sensitive and specific in differentiating Tourette's syndrome and chronic tic disorder. The perfusion difference between the two groups, demonstrated by semi-quantitative analysis, may be related more to the co-morbidity in Tourette's syndrome than to tics per se. (orig.)

  18. Role of tyrosine-kinase inhibitors in myeloproliferative neoplasms: comparative lessons learned

    Science.gov (United States)

    Pinilla-Ibarz, Javier; Sweet, Kendra L; Corrales-Yepez, Gabriela M; Komrokji, Rami S

    2016-01-01

    An important pathogenetic distinction in the classification of myeloproliferative neoplasms (MPNs) is the presence or absence of the BCR–ABL fusion gene, which encodes a unique oncogenic tyrosine kinase. The BCR–ABL fusion, caused by the formation of the Philadelphia chromosome (Ph) through translocation, constitutes the disease-initiating event in chronic myeloid leukemia. The development of successive BCR–ABL-targeted tyrosine-kinase inhibitors has led to greatly improved outcomes in patients with chronic myeloid leukemia, including high rates of complete hematologic, cytogenetic, and molecular responses. Such levels of treatment success have long been elusive for patients with Ph-negative MPNs, because of the difficulties in identifying specific driver proteins suitable as drug targets. However, in recent years an improved understanding of the complex pathobiology of classic Ph-negative MPNs, characterized by variable, overlapping multimutation profiles, has prompted the development of better and more broadly targeted (to pathway rather than protein) treatment options, particularly JAK inhibitors. In classic Ph-negative MPNs, overactivation of JAK-dependent signaling pathways is a central pathogenic mechanism, and mutually exclusive mutations in JAK2, MPL, and CALR linked to aberrant JAK activation are now recognized as key drivers of disease progression in myelofibrosis (MF). In clinical trials, the JAK1/JAK2 inhibitor ruxolitinib – the first therapy approved for MF worldwide – improved disease-related splenomegaly and symptoms independent of JAK2V617F mutational status, and prolonged survival compared with placebo or standard therapy in patients with advanced MF. In separate trials, ruxolitinib also provided comprehensive hematologic control in patients with another Ph-negative MPN – polycythemia vera. However, complete cytogenetic or molecular responses with JAK inhibitors alone are normally not observed, underscoring the need for novel

  19. The pain drawing as an instrument for identifying cervical spine nerve involvement in chronic whiplash-associated disorders

    Science.gov (United States)

    Bernhoff, Gabriella; Landén Ludvigsson, Maria; Peterson, Gunnel; Bertilson, Bo Christer; Elf, Madeleine; Peolsson, Anneli

    2016-01-01

    Objective The aim of the study was to investigate the psychometric properties of a standardized assessment of pain drawing with regard to clinical signs of cervical spine nerve root involvement. Design This cross-sectional study included data collected in a randomized controlled study. Patients: Two hundred and sixteen patients with chronic (≥6 months) whiplash-associated disorders, grade 2 or 3, were included in this study. Methods The validity, sensitivity, and specificity of a standardized pain drawing assessment for determining nerve root involvement were analyzed, compared to the clinical assessment. In addition, we analyzed the interrater reliability with 50 pain drawings. Results Agreement was poor between the standardized pain drawing assessment and the clinical assessment (kappa =0.11, 95% CI: −0.03 to 0.20). Sensitivity was high (93%), but specificity was low (19%). Interrater reliability was good (kappa =0.64, 95% CI: 0.53 to 0.76). Conclusion: The standardized pain drawing assessment of nerve root involvement in chronic whiplash-associated disorders was not in agreement with the clinical assessment. Further research is warranted to optimize the utilization of a pain/discomfort drawing as a supportive instrument for identifying nerve involvement in cervical spinal injuries.

  20. Safety and efficacy of aripiprazole for the treatment of pediatric Tourette syndrome and other chronic tic disorders

    Directory of Open Access Journals (Sweden)

    Cox JH

    2016-06-01

    Full Text Available Joanna H Cox,1 Stefano Seri,2,3 Andrea E Cavanna,2,4,5 1Heart of England NHS Foundation Trust, 2School of Life and Health Sciences, Aston Brain Centre, Aston University, 3Children’s Epilepsy Surgery Programme, The Birmingham Children’s Hospital NHS Foundation Trust, 4Department of Neuropsychiatry, Birmingham and Solihull Mental Health NHS Foundation Trust, Birmingham, 5Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology and UCL, London, UK Abstract: Tourette syndrome is a childhood-onset chronic tic disorder characterized by multiple motor and vocal tics and often accompanied by specific behavioral symptoms ranging from obsessionality to impulsivity. A considerable proportion of patients report significant impairment in health-related quality of life caused by the severity of their tics and behavioral symptoms and require medical intervention. The most commonly used medications are antidopaminergic agents, which have been consistently shown to be effective for tic control, but are also associated with poor tolerability because of their adverse effects. The newer antipsychotic medication aripiprazole is characterized by a unique mechanism of action (D2 partial agonism, and over the last decade has increasingly been used for the treatment of tics. We conducted a systematic literature review to assess the available evidence on the efficacy and safety of aripiprazole in pediatric patients with Tourette syndrome and other chronic tic disorders (age range: 4–18 years. Our search identified two randomized controlled trials (involving 60 and 61 participants and ten open-label studies (involving between six and 81 participants. The majority of these studies used two validated clinician-rated instruments (Yale Global Tic Severity Scale and Clinical Global Impression scale as primary outcome measures. The combined results from randomized controlled trials and open-label studies showed that aripiprazole is an

  1. Cerebrospinal fluid volume depletion in chronic whiplash-associated disorders from motor vehicle-related spinal injuries

    International Nuclear Information System (INIS)

    To evaluate cerebrospinal fluid (CSF) volume depletion in chronic cases of whiplash-associated disorders, 111In radioisotope (RI) cisternography, brain magnetic resonance imaging (MRI) and lumbar MR myelography were consecutively conducted on 460 individuals with chronic whiplash-associated disorders resulting from motor vehicle collision (Group A, n=225) and other traumatic injuries (Group B, n=57), spontaneous intracranial hypotension syndromes and other miscellaneous disorders (Group C, n=155), iatrogenic intracranial hypotension syndrome (Group D, n=11), and communicating hydrocephalus (Group E, n=12). Movement of intrathecally administered RI via a lumbar puncture was sequentially scanned at 1, 2 or 3, 5 and 24 hours. A whole body neuroaxis scanned figure showing high spinal parathecal activity at any time was considered to be a CSF leak, if small enough meningeal diverticula evidenced by MR myelography were present. Retention rate (%) of intrathecal RI for each scan was calculated using the formula: (whole body count-urinary bladder count)/whole body count (cpm) at 1 h x 100. All CSF leaks, although having single to multiple poles, were located in the spinal canal. CSF leakage was observed in 99/225 (44%), 24/57 (42%), 61/155 (39%), 9/11 (82%), and 4/12 (33%), in Groups A, B, C, D and E respectively. All CSF leakages was involved with the lumbar spine in Group A, although 20 cases extended to mid-thoracic levels. In Group A, spinal vertebrae were concomitantly injured in 7 cases (1 cervical spine dislocation, 1 cervical spine fracture, 2 thoracic and 1 lumbar compression fracture (s), and 2 lumbar disc hernia). CSF leakage for 2 cervical spine injuries was not at the injured site but at the lumbar spinal canal. CSF leakage limited to the lumbar spine involved 22 and 43 cases in groups B and C, respectively. Of all CSF leaks, 24 h retention rates less than 30% accounted for 90% of cases. In Group A, early CSF excretion and less than a 30% retention rate at 24

  2. Acute and chronic gastric emptying disorders in rats after localized X-irradiation, and the therapy of these disorders

    International Nuclear Information System (INIS)

    After localized 300 kV X-irradiation of the rat stomach the stomach emptying time of a liquid and a solid test meal was examined with a non-invasive radiological method. In the acute period one to three weeks after irradiation with single doses between 10.7 and 21.3 Gy we observed a faster emptying of the liquid and a delayed emptying of the solid test meal. The faster emptying of the liquid test meal was treated successfully with atropin. In the chromic period we observed a delayed emptying of the liquid and of the solid test meal. These emptying disorders were treated partially successfully with the parasympathomimeticum carbachol and they were treated completeley successfully with the dopamine antagonist metoclopramide. (orig.)

  3. EFFECTIVE TREATMENT OF PATIENTS WITH CHRONIC HEART FAILURE AND DEPRESSIVE DISORDERS WITH NOOTROPICS DRUG PANTOGAM ACTIV

    OpenAIRE

    Baranov, A. P.; A. V. Strutynsky; O. Sh. Oynotkinova; Gorbacheva, E. V.; V. V. Trishina; A. S. Gusev-Scherbakov; J. J. Golubev

    2016-01-01

    We investigate the efficiency of the inclusion nootropic drug Pantogam Activ in the complex therapy of 82 patients with heart failure, ischemic heart diseases, anxiety and depressive disorders. It was shown that an 8-week treatment with Pantogam Activ in most patients is accompanied by a significant reduction of anxiety and depressive disorders, increase exercise tolerance, improved autonomic regulation of heart function and decrease the frequency of supraventricular and ventricular arrhythmi...

  4. Pathological Features of Bone Marrow in Commonly Seen Myeloproliferative Diseases%常见骨髓增殖性疾病骨髓病理学特征

    Institute of Scientific and Technical Information of China (English)

    陈辉树

    2009-01-01

    2008年WHO造血与淋巴组织肿瘤分类(简称2008年WHO分类)将慢性骨髓增殖性疾病(chronic myeloproliferative disease,CMPD)称为骨髓增殖性肿瘤(myeloproliferative neoplasm,MPN),并在2001年WHo分类的CMPD基础上增加了肥大细胞增生症(mastocytosis,MC),共8种亚型。慢性中性粒细胞白血病(chronic neutrophilic leukemia,CNL)、慢性嗜酸粒细胞白血病,非特指型(CEL,NOS)、MC和MPN,无法分类(MPN,U)均相对少见,故本文仅对其中常见的真性红细胞增多症(polycythemia vera,PV)、原发性骨髓纤维化(idiopathic myelfibrosis,IMF)和原发性血小板增多症(essential thrombocythemia,ET)的骨髓活检病理诊断与鉴别做一介绍。

  5. Amygdala, Hippocampus, and Ventral Medial Prefrontal Cortex Volumes Differ in Maltreated Youth with and without Chronic Posttraumatic Stress Disorder.

    Science.gov (United States)

    Morey, Rajendra A; Haswell, Courtney C; Hooper, Stephen R; De Bellis, Michael D

    2016-02-01

    Posttraumatic stress disorder (PTSD) is considered a disorder of recovery where individuals fail to learn and retain extinction of the traumatic fear response. In maltreated youth, PTSD is common, chronic, and associated with comorbidity. Studies of extinction-related structural volumes (amygdala, hippocampus, anterior cingulate cortex (ACC), and ventral medial prefrontal cortex (vmPFC)) and this stress diathesis, in maltreated youth were not previously investigated. In this cross-sectional study, neuroanatomical volumes associated with extinction in maltreated youth with PTSD (N=31), without PTSD (N=32), and in non-maltreated healthy volunteers (n=57) were examined using magnetic resonance imaging. Groups were sociodemographically similar. Participants underwent extensive assessments for strict inclusion/exclusion criteria and DSM-IV disorders. Maltreated youth with PTSD demonstrated decreased right vmPFC volumes compared with both maltreated youth without PTSD and non-maltreated controls. Maltreated youth without PTSD demonstrated larger left amygdala and right hippocampal volumes compared with maltreated youth with PTSD and non-maltreated control youth. PTSD symptoms inversely correlated with right and left hippocampal and left amygdala volumes. Confirmatory masked voxel base morphometry analyses demonstrated greater medial orbitofrontal cortex gray matter intensity in controls than maltreated youth with PTSD. Volumetric results were not influenced by psychopathology or maltreatment variables. We identified volumetric differences in extinction-related structures between maltreated youth with PTSD from those without PTSD. Alterations of the vmPFC may be one mechanism that mediates the pathway from PTSD to comorbidity. Further longitudinal work is needed to determine neurobiological factors related to chronic and persistent PTSD, and to PTSD resilience despite maltreatment. PMID:26171720

  6. Chronic subordination stress induces hyperphagia and disrupts eating behavior in mice modeling binge-eating-like disorder

    Directory of Open Access Journals (Sweden)

    Maria eRazzoli

    2015-01-01

    Full Text Available Background: Eating disorders are associated with physical morbidity and appear to have causal factors like stressful life events and negative affect. Binge eating disorder (BED is characterized by eating in a discrete period of time a larger than normal amount of food, a sense of lack of control over eating, and marked distress. There are still unmet needs for the identification of mechanisms regulating excessive eating, which is in part due to the lack of appropriate animal models. We developed a naturalistic murine model of subordination stress induced hyperphagia associated with the development of obesity. Here we tested the hypotheses that the eating responses of subordinate mice recapitulate the BED and that limiting hyperphagia could prevent stress-associated metabolic changes. Methods: Adult male mice were exposed to a model of chronic subordination stress associated with the automated acquisition of food intake and we performed a detailed meal pattern analysis. Additionally, using a pair-feeding protocol was test the hypothesis that the manifestation of obesity and the metabolic syndrome could be prevented by limiting hyperphagia. Results: The architecture of feeding of subordinate mice was disrupted during the stress protocol due to disproportionate amount of food ingested at higher rate and with shorter satiety ratio than control mice. Subordinate mice hyperphagia was further exacerbated in response to either hunger or to the acute application of a social defeat. Notably, the obese phenotype but not the fasting hyperglycemia of subordinate mice was abrogated by preventing hyperphagia in a pair feeding paradigm. Conclusion: Overall these results support the validity of our chronic subordination stress to model binge eating disorder allowing for the determination of the underlying molecular mechanisms and the generation of testable predictions for innovative therapies, based on the understanding of the regulation and the control of food

  7. The JAK2V617F activating mutation occurs in chronic myelomonocytic leukemia and acute myeloid leukemia, but not in acute lymphoblastic leukemia or chronic lymphocytic leukemia

    Science.gov (United States)

    Levine, Ross L.; Loriaux, Marc; Huntly, Brian J. P.; Loh, Mignon L.; Beran, Miroslav; Stoffregen, Eric; Berger, Roland; Clark, Jennifer J.; Willis, Stephanie G.; Nguyen, Kim T.; Flores, Nikki J.; Estey, Elihu; Gattermann, Norbert; Armstrong, Scott; Look, A. Thomas; Griffin, James D.; Bernard, Olivier A.; Heinrich, Michael C.; Gilliland, D. Gary; Druker, Brian; Deininger, Michael W. N.

    2005-01-01

    Activating mutations in tyrosine kinases have been identified in hematopoietic and nonhematopoietic malignancies. Recently, we and others identified a single recurrent somatic activating mutation (JAK2V617F) in the Janus kinase 2 (JAK2) tyrosine kinase in the myeloproliferative disorders (MPDs) polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. We used direct sequence analysis to determine if the JAK2V617F mutation was present in acute myeloid leukemia (AML), chronic myelomonocytic leukemia (CMML)/atypical chronic myelogenous leukemia (aCML), myelodysplastic syndrome (MDS), B-lineage acute lymphoblastic leukemia (ALL), T-cell ALL, and chronic lymphocytic leukemia (CLL). Analysis of 222 patients with AML identified JAK2V617F mutations in 4 patients with AML, 3 of whom had a preceding MPD. JAK2V617F mutations were identified in 9 (7.8%) of 116 CMML/a CML samples, and in 2 (4.2%) of 48 MDS samples. We did not identify the JAK2V617F disease allele in B-lineage ALL (n = 83), T-cell ALL (n = 93), or CLL (n = 45). These data indicate that the JAK2V617F allele is present in acute and chronic myeloid malignancies but not in lymphoid malignancies. PMID:16081687

  8. A prospective, descriptive study of hour-to-hour and day-to-day temperature variability of skin affected by chronic venous disorders.

    Science.gov (United States)

    Kelechi, Teresa J; McNeil, Rebecca B

    2008-04-01

    Evidence suggests that skin temperature is elevated in the lower legs of individuals with the most severe stages of chronic venous disorder-related skin inflammation. Fifteen (15) patients (average age 67.7 years) with several chronic health conditions, chronic venous disorders, and a history of leg ulcers volunteered to participate in a prospective, descriptive, two-part (hourly and daily) study to test two hypotheses: 1) that skin temperature variations of chronically inflamed skin of lower legs affected by chronic venous disorders exhibit no differences in hour-to-hour and day-to-day rhythmic patterns associated with sleep and activities such as walking, exercise, or compression stocking use among four selected skin sites (two per leg) or between the legs of individuals with chronic venous disorders; and 2) that the difference in temperature between sites is unequal between legs. All study participants were at high risk for developing venous ulcers (CEAP stage 4 and 5). Skin temperature was obtained at sites with highest temperatures and/or areas of healed ulcers and mapped hourly over a 2-day period with a data logger and daily for 30 days with an infrared thermometer. No consistent, visually detectable effects due to caffeine use, eating, activity, or other variables assessed were found; only sleeping resulted in a consistent increase in skin temperature. Difference in skin temperature between measurement sites was found to be dependent on the leg on which the sites were located (P=0.1127). Because skin temperature variability could not be explained by the variables assessed, a temperature change could suggest the presence of a pathological process such as an infection or increased inflammation. Future studies to determine whether variability of skin temperature over sites affected by chronic venous disorders heralds further skin impairment are warranted. PMID:18480503

  9. A Research Synthesis of Therapeutic Interventions for Whiplash-Associated Disorder (WAD: Part 4 – Noninvasive Interventions for Chronic WAD

    Directory of Open Access Journals (Sweden)

    Robert W Teasell

    2010-01-01

    Full Text Available Whiplash-associated disorder (WAD represents a significant public health problem, resulting in substantial social and economic costs throughout the industrialized world. While many treatments have been advocated for patients with WAD, scientific evidence supporting their effectiveness is often lacking. A systematic review was conducted to evaluate the strength of evidence for various WAD therapies. Multiple databases (including Web of Science, EMBASE and PubMed were searched to identify all studies published from January 1980 through March 2009 that evaluated the effectiveness of any clearly defined treatment for acute (less than two weeks, subacute (two to 12 weeks or chronic (longer than 12 weeks WAD. The present article, the fourth in a five-part series, evaluates the evidence for noninvasive interventions initiated during the chronic phase of WAD. Twenty-two studies that met the inclusion criteria were identified, 12 of which were randomized controlled trials with ‘good’ overall methodological quality (median Physiotherapy Evidence Database score of 6. For the treatment of chronic WAD, there is evidence to suggest that exercise programs are effective in relieving whiplash-related pain, at least over the short term. While the majority of a subset of nine studies supported the effectiveness of interdisciplinary interventions, the two randomized controlled trials provided conflicting results. Finally, there was limited evidence, consisting of one supportive case series each, that both manual joint manipulation and myofeedback training may provide some benefit. Based on the available research, exercise programs were the most effective noninvasive treatment for patients with chronic WAD, although many questions remain regarding the relative effectiveness of various exercise regimens.

  10. Maintaining a balance: a focus group study on living and coping with chronic whiplash-associated disorder

    Directory of Open Access Journals (Sweden)

    Ihlebæk Camilla

    2010-07-01

    Full Text Available Abstract Background There is little qualitative insight into how persons with chronic Whiplash-Associated Disorder cope on a day to day basis. This study seeks to identify the symptoms persons with Whiplash-Associated Disorder describe as dominating and explore their self-initiated coping strategies. Methods Qualitative study using focus groups interviews. Fourteen Norwegian men and women with Whiplash-Associated Disorder (I or II were recruited to participate in two focus groups. Data were analyzed according to a phenomenological approach, and discussed within the model of Cognitive Activation Theory of Stress (CATS. Results Participants reported neck and head pain, sensory hypersensitivity, and cognitive dysfunction following their whiplash injury. Based on the intensity of symptoms, participants divided everyday life into good and bad periods. In good periods the symptoms were perceived as manageable. In bad periods the symptoms intensified and took control of the individual. Participants expressed a constant notion of trying to balance their three main coping strategies; rest, exercise, and social withdrawal. In good periods participants experienced coping by expecting good results from the strategies they used. In bad periods they experienced no or negative relationships between their behavioral strategies and their complaints. Conclusions Neck and head pain, sensory hypersensitivity, and cognitive dysfunction were reported as participants' main complaints. A constant notion of balancing between their three main coping strategies; rest, exercise, and social withdrawal, was described.

  11. Genetic and environmental risk for chronic pain and the contribution of risk variants for major depressive disorder: a family-based mixed-model analysis

    OpenAIRE

    McIntosh, Andrew; Hall, Lynsey S.; Zeng, Yanni; Adams, Mark J; Gibson, Jude; Wigmore, Ella; Hagenaars, Saskia; Davies, Gail; Fernandez-Pujals, Ana Maria; Campbell, Archie; Clarke, Toni-Kim; Hayward, Caroline; Haley, Christopher; Porteous, David; Deary, Ian

    2016-01-01

    Background Chronic pain is highly prevalent and a significant source of disability, yet its genetic and environmental risk factors are poorly understood. Its relationship with major depressive disorder (MDD) is of particular importance. We sought to test the contribution of genetic factors and shared and unique environment to risk of chronic pain and its correlation with MDD in Generation Scotland: Scottish Family Health Study (GS:SFHS). We then sought to replicate any significant findings in...

  12. Moxifloxacin in Preventing Bacterial Infections in Patients Who Have Undergone Donor Stem Cell Transplant

    Science.gov (United States)

    2013-03-14

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Infection; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  13. Motexafin Gadolinium and Doxorubicin in Treating Patients With Advanced Cancer

    Science.gov (United States)

    2015-09-30

    Breast Cancer; Chronic Myeloproliferative Disorders; Colorectal Cancer; Head and Neck Cancer; Leukemia; Lung Cancer; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic/Myeloproliferative Diseases; Prostate Cancer; Small Intestine Cancer; Unspecified Adult Solid Tumor, Protocol Specific

  14. Moxifloxacin Compared With Ciprofloxacin/Amoxicillin in Treating Fever and Neutropenia in Patients With Cancer

    Science.gov (United States)

    2012-09-20

    Chronic Myeloproliferative Disorders; Fever, Sweats, and Hot Flashes; Infection; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neutropenia; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  15. Blood Samples From Patients With Cancer Treated on a Clinical Trial to Control Nausea and Vomiting During Donor Stem Cell Transplant

    Science.gov (United States)

    2016-02-11

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nausea and Vomiting; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  16. T Cells in Predicting Acute Graft-Versus-Host Disease in Patients Undergoing Donor Stem Cell Transplant

    Science.gov (United States)

    2016-06-22

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  17. Ravuconazole in Preventing Fungal Infections in Patients Undergoing Allogeneic Stem Cell Transplantation

    Science.gov (United States)

    2012-03-07

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Infection; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  18. Aprepitant, Granisetron, & Dexamethasone in Preventing Nausea & Vomiting in Pts. Receiving Cyclophosphamide Before a Stem Cell Transplant

    Science.gov (United States)

    2016-02-12

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nausea and Vomiting; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  19. 1H MRS study of brain metabolic disorder in patients with chronic liver cirrhosis

    International Nuclear Information System (INIS)

    Objective: To study the metabolic alterations in the brain of patients with chronic liver cirrhosis with 1H magnetic resonance spectroscopy (1H MRS) for better understanding the pathophysiology of chronic hepatic encephalopathy (CHE), which will help us in the diagnosis and treatment of this disease. Methods: STEAM 1H MRS and MRI were performed in 30 patients with chronic liver cirrhosis is and 15 healthy volunteers. The height of resonance peaks of different metabolites was measured and the ratios of the other metabolites to Cr were calculated. The authors also studied the correlation between metabolites and the association between globus pallidum signal intensity and the spectroscopic alterations. Results: In patients with severe cirrhosis (in decompensatory period) or CHE, the mean values of mI/Cr and Cho/Cr ratio were significantly lower than those in healthy volunteers or patients with minor cirrhosis (in compensatory period) (P0.05). There was negative correlation between mI/Cr and Glx-α/Cr(r -0.51, n = 44, P1-weighted images, the globus pallidum signal intensity was significantly higher in patients with severe cirrhosis or CHE. mI/Cr, Cho/Cr and Glx-α/Cr correlated significantly with MRI signal changes respectively. Conclusions: Proton MRS can demonstrate brain metabolic changes in patients with chronic liver cirrhosis in vivo noninvasively, thereby helping interpret the pathophysiology of CHE

  20. Diagnostic agreement of schizophrenia spectrum disorders among chronic patients with functional psychoses

    DEFF Research Database (Denmark)

    Jakobsen, K D; Frederiksen, JN; Parnas, J;

    2006-01-01

    BACKGROUND: To investigate whether diagnostic agreement across different diagnostic systems improves in a sample of chronic patients suffering from functional psychosis compared to first-admitted patients. SAMPLING AND METHODS: Among 353 patients with a history of functional psychosis, a subset o...

  1. Cardiovascular Risk in Chronic Kidney Disease (CKD), the CKD- Mineral Bone Disorder (CKD- MBD)

    OpenAIRE

    Hruska, Keith A; Choi, Eric T.; Memon, Imran; Davis, T. Keefe; Mathew, Suresh

    2009-01-01

    Recent advances in our understanding of the excess mortality of chronic kidney disease (CKD) due to cardiovascular complications demonstrate through observational studies that vascular calcification and hyperphosphatemia are major cardiovascular risk factors. Mechanistic studies demonstrate that these two risk factors are related, and that hyperphosphatemia directly stimulates vascular calcification. The role of hyperphosphatemia in stimulating vascular calcification in CKD is associated with...

  2. Complex Variant of Philadelphia Translocation Involving Chromosomes 9, 12, and 22 in a Case with Chronic Myeloid Leukaemia

    Directory of Open Access Journals (Sweden)

    F. Malvestiti

    2014-01-01

    Full Text Available Chronic myeloid leukemia (CML is a hematopoietic stem cell disorder included in the broader diagnostic category of myeloproliferative neoplasms, associated with fusion by BCR gene at chromosome 22q11 to ABL1 gene at chromosome 9q34 with the formation of the Philadelphia (Ph chromosome. In 2–10% of CML cases, the fusion gene arises in connection with a variant translocation, involving chromosomes 9, 22, and one or more different chromosomes; consequently, the Ph chromosome could be masked within a complex chromosome rearrangement. In cases with variant Ph translocation a deletion on der(9 may be more frequently observed than in cases with the classical one. Herein we describe a novel case of CML with complex variant Ph translocation involving chromosomes 9, 12, and 22. We present the hematologic response and cytogenetic response after Imatinib treatment. We also speculated the mechanism which had originated the chromosome rearrangement.

  3. Induction of Chronic Myeloid Leukemia in Mice.

    Science.gov (United States)

    Zhang, Haojian; Li, Shaoguang

    2016-01-01

    Chronic myeloid leukemia (CML) is a myeloproliferative disorder derived from a hematopoietic stem cell (HSC), harboring Philadelphia chromosome (Ph chromosome). Formation of the Ph chromosome is caused by a reciprocal translocation between the chromosomes 9 and 22 t(9;22)(q34;q11), resulting in a fusion protein known as BCR-ABL which has constitutive tyrosine kinase activity and promotes the proliferation of leukemia cells via multiple mechanisms. Studies on CML have led to the identification of the first cancer-associated chromosomal abnormality and the subsequent development of tyrosine kinase inhibitors (TKIs) that inhibit BCR-ABL kinase activity in CML. It has become clear that leukemia stem cells (LSCs) in CML are insensitive to inhibition by TKIs, and eradication of LSCs appears to be difficult. Therefore, some of the major issues in current CML therapy are to understand the biology of LSCs and to investigate why LSCs are insensitive to TKIs for developing curative therapeutic strategies. In this regard, application of mouse models recapitulating human CML disease will be critical. In this chapter, we describe methods for induction of CML in mice with BCR-ABL. PMID:27581135

  4. EFFECTIVE TREATMENT OF PATIENTS WITH CHRONIC HEART FAILURE AND DEPRESSIVE DISORDERS WITH NOOTROPICS DRUG PANTOGAM ACTIV

    Directory of Open Access Journals (Sweden)

    A. P. Baranov

    2016-01-01

    Full Text Available We investigate the efficiency of the inclusion nootropic drug Pantogam Activ in the complex therapy of 82 patients with heart failure, ischemic heart diseases, anxiety and depressive disorders. It was shown that an 8-week treatment with Pantogam Activ in most patients is accompanied by a significant reduction of anxiety and depressive disorders, increase exercise tolerance, improved autonomic regulation of heart function and decrease the frequency of supraventricular and ventricular arrhythmias, which is accompanied by a marked improvement in the quality of life. 

  5. A pilot study of a chiropractic intervention for management of chronic myofascial temporomandibular disorder

    DEFF Research Database (Denmark)

    DeVocht, James W; Goertz, Christine M; Hondras, Maria;

    2013-01-01

    BACKGROUND: Temporomandibular pain has multiple etiologies and a range of therapeutic options. In this pilot study, the authors assessed the feasibility of conducting a larger trial to evaluate chiropractic treatment of temporomandibular disorders (TMDs). METHODS: The authors assigned 80 particip......BACKGROUND: Temporomandibular pain has multiple etiologies and a range of therapeutic options. In this pilot study, the authors assessed the feasibility of conducting a larger trial to evaluate chiropractic treatment of temporomandibular disorders (TMDs). METHODS: The authors assigned 80...... that will provide clinicians with information that should be helpful when discussing treatment options for patients with TMD....

  6. Chronic pain patients with possible co-morbid post-traumatic stress disorder admitted to multidisciplinary pain rehabilitation—a 1-year cohort study

    OpenAIRE

    Andersen, Tonny Elmose; Andersen, Lou-Ann Christensen; Andersen, Per Grünwald

    2014-01-01

    Background: Although post-traumatic stress disorder (PTSD) is a common co-morbidity in chronic pain, little is known about the association between PTSD and pain in the context of chronic pain rehabilitation.Objective: The aim of the present study was two-fold: (1) to investigate the association of a possible PTSD diagnosis with symptoms of pain, physical and mental functioning, as well as the use of opioids, and (2) to compare the outcome of multidisciplinary chronic pain rehabilitation for p...

  7. [Chronic fatigue syndrome--a disease entity or an unspecified psychosomatic disorder?].

    Science.gov (United States)

    Albus, C

    1997-12-01

    In spite of its nature as an often severe and disabeling disease, it is still unclear, whether the Chronic Fatigue Syndrome (CFS) is an entire disease of its own right or not. Moreover, there is a growing evidence that patients with CFS belong to an inhomogeneous group with different etiologic constellations. Specific somatic factors, e.g. viruses, seem to be less important for onset than certain personality-traits like depressiveness and workaholism. These traits lead to an increased vulnerability to unspecific psychological or biological stressors that may cause chronic fatigue by complex psychosomatic interferences. Concerning diagnosis, there are no specific methods or results available, the same is true for pharmacological treatment. As a consequence, practitioners should be aware not to miss a somatic disease causing fatigue, and, parallel to this, start right from the beginning talking about the psychosomatic background of CFS. Furthermore, psychotherapy has shown to be effective in CFS. PMID:9487622

  8. Depression and Anxiety in Patients with Epilepsy, With or Without Other Chronic Disorders

    OpenAIRE

    Asadi-Pooya, A A; Sperling, M.R.

    2011-01-01

    Background Most people with epilepsy lead a normal emotional and cognitive life, however neurobehavioral problems can be found in a large number of patients. This study evaluates the prevalence of depression and anxiety among patients with epilepsy and determines whether having other chronic somatic illnesses increases the prevalence. Methods Adults with epilepsy were recruited in either the inpatient epilepsy monitoring unit or the Outpatient Epilepsy Clinic at Thomas Jefferson University in...

  9. Fourth revolution in psychiatry – Addressing comorbidity with chronic physical disorders

    OpenAIRE

    Gautam, Shiv

    2010-01-01

    The moral treatment of mental patients, Electro Convulsive therapy (ECT), and Psychotropic medications constitute the first, second, and third revolution in psychiatry, respectively. Addressing comorbidities of mental illnesses with chronic physical illnesses will be the fourth revolution in psychiatry. Mind and body are inseparable; there is a bidirectional relationship between psyche and soma, each influencing the other. Plausible biochemical explanations are appearing at an astonishing rat...

  10. Central role of carotid body chemoreceptors in disordered breathing and cardiorenal dysfunction in chronic heart failure

    OpenAIRE

    Marcus, Noah J.; Del Rio, Rodrigo; Schultz, Harold D.

    2014-01-01

    Oscillatory breathing (OB) patterns are observed in pre-term infants, patients with cardio-renal impairment, and in otherwise healthy humans exposed to high altitude. Enhanced carotid body (CB) chemoreflex sensitivity is common to all of these populations and is thought to contribute to these abnormal patterns by destabilizing the respiratory control system. OB patterns in chronic heart failure (CHF) patients are associated with greater levels of tonic and chemoreflex-evoked sympathetic nerve...

  11. What’s in a name? Compliance, adherence and concordance in chronic psychiatric disorders

    OpenAIRE

    Chakrabarti, Subho

    2014-01-01

    About half or more of the patients with chronic psychiatric illnesses, either do not take their medications correctly, or completely stop taking them. The problem of poor initial compliance or adherence is often compounded by a continued decline in compliance/adherence over time. The failure to take medicines, adversely affects the outcome of treatment, and places a huge burden of wasted resources on the society. Three terms have been used to describe medication-taking among patients with chr...

  12. Reliability of clinical ICD-10 diagnoses among electroconvulsive therapy patients with chronic affective disorders

    DEFF Research Database (Denmark)

    Jakobsen, Klaus Damgaard; Hansen, Thomas Folkmann; Dam, Henrik;

    2008-01-01

    investigated. A standardized schema for basic anamnesis and the Operational Criteria Checklist for Psychotic and Affective Illness (OPCRIT) were used. The sensitivity, specificity, positive and negative predictive values of clinical affective disorder ICD-10 diagnoses and the formal agreement between clinical...

  13. Hemolytic anemia following high dose intravenous immunoglobulin in patients with chronic neurological disorders

    DEFF Research Database (Denmark)

    Markvardsen, L H; Christiansen, Ingelise; Harbo, T;

    2014-01-01

    High dose intravenous immunoglobulin (IVIG) is an established treatment for various neuromuscular disorders. Recently, cases of hemolytic anemia following IVIG have been observed. The objective of this study was to determine the extent of anemia and hemolysis after IVIG and its relationship...

  14. Conceptual and Perceptual Priming and Dissociation in Chronic Posttraumatic Stress Disorder

    NARCIS (Netherlands)

    Lyttle, Nigel; Dorahy, Martin J.; Hanna, Donncha; Huntjens, Rafaele J. C.

    2010-01-01

    Cognitive models of posttraumatic stress disorder (PTSD) assert that memory processes play a significant role in PTSD (see e.g., Ehlers & Clark, 2000). Intrusive reexperiencing in PTSD has been linked to perceptual processing of trauma-related material with a corresponding hypothesized lack of conce

  15. Alcohol use, cigarette consumption and chronic post-traumatic stress disorder

    NARCIS (Netherlands)

    Op den Velde, W; Aarts, PGH; Falger, PRJ; Hovens, JE; van Duijn, H; de Groen, JHM; van Duijn, MAJ

    2002-01-01

    Aims: The relationship between alcohol consumption, cigarette smoking and post-traumatic stress disorder (PTSD) was studied in 147 male former members of the civilian resistance against the Nazi occupation of Holland during World War II. Methods: The subjects were interviewed at home. Measures inclu

  16. Generation of functional neutrophils from a mouse model of X-linked chronic granulomatous disorder using induced pluripotent stem cells.

    Directory of Open Access Journals (Sweden)

    Sayandip Mukherjee

    Full Text Available Murine models of human genetic disorders provide a valuable tool for investigating the scope for application of induced pluripotent stem cells (iPSC. Here we present a proof-of-concept study to demonstrate generation of iPSC from a mouse model of X-linked chronic granulomatous disease (X-CGD, and their successful differentiation into haematopoietic progenitors of the myeloid lineage. We further demonstrate that additive gene transfer using lentiviral vectors encoding gp91(phox is capable of restoring NADPH-oxidase activity in mature neutrophils derived from X-CGD iPSC. In the longer term, correction of iPSC from human patients with CGD has therapeutic potential not only through generation of transplantable haematopoietic stem cells, but also through production of large numbers of autologous functional neutrophils.

  17. Genome profiling of chronic myelomonocytic leukemia: frequent alterations of RAS and RUNX1 genes

    International Nuclear Information System (INIS)

    Chronic myelomonocytic leukemia (CMML) is a hematological disease close to, but separate from both myeloproliferative disorders (MPD) and myelodysplastic syndromes and may show either myeloproliferative (MP-CMML) or myelodysplastic (MD-CMML) features. Not much is known about the molecular biology of this disease. We studied a series of 30 CMML samples (13 MP- and 11 MD-CMMLs, and 6 acutely transformed cases) from 29 patients by using Agilent high density array-comparative genomic hybridization (aCGH) and sequencing of 12 candidate genes. Two-thirds of samples did not show any obvious alteration of aCGH profiles. In one-third we observed chromosome abnormalities (e.g. trisomy 8, del20q) and gain or loss of genes (e.g. NF1, RB1 and CDK6). RAS mutations were detected in 4 cases (including an uncommon codon 146 mutation in KRAS) and PTPN11 mutations in 3 cases. We detected 11 RUNX1 alterations (9 mutations and 2 rearrangements). The rearrangements were a new, cryptic inversion of chromosomal region 21q21-22 leading to break and fusion of RUNX1 to USP16. RAS and RUNX1 alterations were not mutually exclusive. RAS pathway mutations occurred in MP-CMMLs (~46%) but not in MD-CMMLs. RUNX1 alterations (mutations and cryptic rearrangement) occurred in both MP and MD classes (~38%). We detected RAS pathway mutations and RUNX1 alterations. The latter included a new cryptic USP16-RUNX1 fusion. In some samples, two alterations coexisted already at this early chronic stage

  18. Dry-needling and exercise for chronic whiplash-associated disorders: a randomized single-blind placebo-controlled trial.

    Science.gov (United States)

    Sterling, Michele; Vicenzino, Bill; Souvlis, Tina; Connelly, Luke B

    2015-04-01

    This randomized controlled trial investigated the effectiveness and cost-effectiveness of dry-needling and exercise compared with sham dry-needling and exercise for chronic whiplash-associated disorders (WAD). The setting was a single university centre and 4 physiotherapy practices in Queensland, Australia. Eighty patients with chronic WAD (>3 months) were enrolled between June 2009 and August 2012 with 1-year follow-up completed in August 2013. The interventions were 6 weeks of dry-needling to posterior neck muscles (n = 40) and exercise or sham dry-needling and exercise (n = 40). The primary outcomes of the Neck Disability Index (NDI) and self-rated recovery were measured at baseline, 6 and 12 weeks, 6 and 12 months by a blinded assessor. Analysis was intention to treat. An economic evaluation was planned but missing data deemed further analysis unwarranted. Seventy-nine patients (99%) were followed up at 6 weeks, 78 (98%) at 12 weeks, 74 (93%) at 6 months, and 73 (91%) at 12 months. The dry-needling and exercise intervention was more effective than sham dry-needling and exercise in reducing disability at 6 and 12 months but not at 6 and 12 weeks. The treatment effects were small and not clinically worthwhile. At 6 weeks, the treatment effect on the 0-100 NDI was -0.3 (95% confidence interval -5.4 to 4.7), 12 weeks -0.3 (-5.2 to 4.9), 6 months -4.4 (-9.6 to -0.74), and 12 months -3.8 (-9.1 to -0.5). There was no effect for self-rated recovery. In patients with chronic WAD, dry-needling and exercise has no clinically worthwhile effects over sham dry-needling and exercise. PMID:25790454

  19. Cognitive-behaviour therapy and skilled motor performance in adults with chronic tic disorder

    OpenAIRE

    O’Connor, Kieron P.; Lavoie, Marc E.; Stip, Emmanuel; Borgeat, François; Laverdure, Anick

    2008-01-01

    The first aim of the present study was to compare performance of people with tic disorders (TD) and controls on executive function and a range of skilled motor tests requiring complex performance, guided movements, hand co-ordination, and fine control of steadiness. The second aim was to investigate the effect of cognitive behaviour therapy (CBT) on motor performance. A total of 55 patients with TD were recruited at baseline from participants in a behavioural management programme. A compariso...

  20. Quantitative MRI Measures of Orbitofrontal Cortex in Patients with Chronic Schizophrenia or Schizoaffective Disorder

    OpenAIRE

    Hoptman, Matthew J.; Volavka, Jan; Weiss, Elisabeth M.; Czobor, Pál; Szeszko, Philip R.; Gerig, Guido; Chakos, Miranda; Blocher, Joseph; Citrome, Leslie L; Lindenmayer, Jean-Pierre; Sheitman, Brian; Lieberman, Jeffrey A.; Bilder, Robert M.

    2005-01-01

    The relationship between orbitofrontal cortex (OFC) volumes and functional domains in treatment-resistant patients with schizophrenia or schizoaffective disorder is poorly undestood. OFC dysfunction is implicated in several of the behaviors that are abnormal in schizophrenia. However, little is known about the relationship between these behaviors and OFC volumes. Forty-nine (49) patients received magnetic resonance imaging scanning as part of a double-blind treatment study in which psychiatri...

  1. Targeted treatment of chronic myeloid leukemia: role of imatinib

    Directory of Open Access Journals (Sweden)

    Ila Tamascar

    2009-03-01

    Full Text Available Ila Tamascar, Jeyanthi RamanarayananDepartment of Medical Oncology, Roswell Park Cancer Institute, Elm and Carlton Streets, Buffalo, NY, USAAbstract: Chronic myeloid leukemia (CML is a myeloproliferative disorder characterized by clonal expansion of pleuripotent hematopoetic stem cells. The incidence of CML is 1 to 2 cases per 100,000 people per year; in the Western Hemisphere, CML accounts for 15% of leukemias in adults. Discovery of the specific karyotypic abnormality of the Philadelphia (Ph chromosome in the pathogenesis of CML has led to a better understanding of the disease and hence to an advancement of targeted therapeutics. Availability of imatinib as an accepted targeted therapy in newly diagnosed patients has changed the treatment paradigm in CML. The majority of CML patients in chronic phase achieve excellent and durable responses with standard-dose imatinib. Mechanisms of primary and secondary resistance to imatinib in CML have been extensively studied and newer tyrosine kinase inhibitors are now being evaluated for clinical use. It is important that at any time the CML treatment and response remain optimal and thus patients on imatinib require continuous monitoring for early detection of resistance. This review will discuss the treatment and guidelines for monitoring CML patients in the imatinib era.Keywords: BCR-ABL, imatinib, tyrosine kinase inhibitors, leukemia, CML

  2. BCR-ABL DERIVED PEPTIDE VACCINES FOR CHRONIC MYELOID LEUKAEMIA

    Directory of Open Access Journals (Sweden)

    M. Bocchia

    2012-01-01

    Full Text Available Chronic Myeloid Leukemia (CML is a myeloproliferative pluripotent stem cell disorder characterized by the presence of a cytogenetic hallmark, the Philadelphia (Ph chromosome, and accounts for 15% of adult leukemias. The disease progresses from a chronic phase through an accelerated phase to a blast phase and its natural course accounts for a median 4 years survival1. The Ph chromosome is derived by a reciprocal translocation termed t(9;22 in which the c-abl oncogene has moved from chromosome 9 into the breakpoint cluster region (bcr, within the bcr gene on chromosome 22, resulting in a chimeric bcr-abl fusion gene that encodes a 210 KD protein (p210 with constitutive tyrosine kinase activity. Two major alternative chimeric p210 can result from this fusion gene: p210-b2a2 where the junction occurs between bcr exon 2 (b2 and abl exon 2 (a2 and p210-b3a2 where the the junction occurs between bcr exon 3 (b3 and abl exon 2 (a2. About 40% of CML patients harbor the p210-b2a2 and about 60% of them show the p210-b3a2.

  3. Mood and Anxiety Disorders in Chronic Fatigue Syndrome, Fibromyalgia, and Irritable Bowel Syndrome : Results From the LifeLines Cohort Study

    NARCIS (Netherlands)

    Janssens, Karin A. M.; Zijlema, Wilma L.; Joustra, Monica L.; Rosmalen, Judith G. M.

    2015-01-01

    Objective: Functional somatic syndromes (FSSs) have often been linked to psychopathology. The aim of the current study was to compare prevalence rates of psychiatric disorders among individuals with chronic fatigue syndrome (CFS), fibromyalgia (FM), and irritable bowel syndrome (IBS). Methods: This

  4. Differences in Depression, Posttraumatic Stress Disorder, and Lifetime Trauma Exposure in Formerly Abused Women with Mild versus Moderate to Severe Chronic Pain

    Science.gov (United States)

    Humphreys, Janice; Cooper, Bruce A.; Miaskowski, Christine

    2010-01-01

    Although associations between intimate partner violence, chronic pain, depression, posttraumatic stress disorder (PTSD), and lifetime trauma exposure are well known, previous studies are limited by their recruitment of women from shelters. These relationships were explored with a community-based sample of formerly abused women ( N = 84).…

  5. Chronic toxicity risk after radiotherapy for patients with systemic sclerosis (systemic scleroderma) or systemic lupus erythematosus: Association with connective tissue disorder severity

    International Nuclear Information System (INIS)

    No method reliably identifies which patients with connective tissue disorders are at greatest risk of radiotherapy-related complications. Building on our prior experience, we postulated that disease severity, as measured by the number of organ systems involved, may predict chronic radiation toxicity risk

  6. Review article: the potential role of nitric oxide in chronic inflammatory bowel disorders

    DEFF Research Database (Denmark)

    Perner, Anders; Rask-Madsen, J

    1999-01-01

    The aetiology of the chronic inflammatory bowel diseases-ulcerative colitis and Crohn's disease-as well as 'microscopic colitis'-both collagenous (COC) and lymphocytic colitis (LC)-remains unknown. Autoimmune mechanisms, cytokine polymorphism, commensal bacteria, infectious agents and vascular...... phagocytes to the venular endothelium and extravasation of these cells into the colonic mucosa. In addition to large amounts of nitric oxide (NO), injurious peroxynitrite may be formed in the epithelium by the inducible nitric oxide synthase (iNOS), which is considered to elicit cytotoxicity by the...

  7. Chronic stress and brain plasticity: Mechanisms underlying adaptive and maladaptive changes and implications for stress-related CNS disorders.

    Science.gov (United States)

    Radley, Jason; Morilak, David; Viau, Victor; Campeau, Serge

    2015-11-01

    Stress responses entail neuroendocrine, autonomic, and behavioral changes to promote effective coping with real or perceived threats to one's safety. While these responses are critical for the survival of the individual, adverse effects of repeated exposure to stress are widely known to have deleterious effects on health. Thus, a considerable effort in the search for treatments to stress-related CNS disorders necessitates unraveling the brain mechanisms responsible for adaptation under acute conditions and their perturbations following chronic stress exposure. This paper is based upon a symposium from the 2014 International Behavioral Neuroscience Meeting, summarizing some recent advances in understanding the effects of stress on adaptive and maladaptive responses subserved by limbic forebrain networks. An important theme highlighted in this review is that the same networks mediating neuroendocrine, autonomic, and behavioral processes during adaptive coping also comprise targets of the effects of repeated stress exposure in the development of maladaptive states. Where possible, reference is made to the similarity of neurobiological substrates and effects observed following repeated exposure to stress in laboratory animals and the clinical features of stress-related disorders in humans. PMID:26116544

  8. Child abuse and neglect in complex dissociative disorder, abuse-related chronic PTSD, and mixed psychiatric samples.

    Science.gov (United States)

    Dorahy, Martin J; Middleton, Warwick; Seager, Lenaire; Williams, Mary; Chambers, Ron

    2016-01-01

    Only a select number of studies have examined different forms of child maltreatment in complex dissociative disorders (DDs) in comparison to other groups. Few of these have used child abuse-related chronic posttraumatic stress disorder (C-PTSD) and mixed psychiatric (MP) patients with maltreatment as comparison groups. This study examined child sexual, physical, and emotional abuse as well as physical and emotional neglect in DD (n = 39), C-PTSD (n = 13), and MP (n = 21) samples, all with abuse and neglect histories. The predictive capacity of these different forms of maltreatment across the 3 groups was assessed for pathological dissociation, shame, guilt, relationship esteem, relationship anxiety, relationship depression, and fear of relationships. All forms of maltreatment differentiated the DD from the MP group, and sexual abuse differentiated the DD sample from the C-PTSD group. Childhood sexual abuse was the only predictor of pathological dissociation. Emotional abuse predicted shame, guilt, relationship anxiety, and fear of relationships. Emotional neglect predicted relationship anxiety and relationship depression. Physical neglect was associated with less relationship anxiety. Different forms of abuse and neglect are associated with different symptom clusters in psychiatric patients with maltreatment histories. PMID:26275087

  9. A Case of ChroniC Mania in a Patient with A Double Diagnosis of Bipolar I and Delusional Disorders

    Directory of Open Access Journals (Sweden)

    Marina Teles Martins

    2013-12-01

    Full Text Available The authors describe the case of a 62 year old woman without any significant personal or family psychiatric history prior to being 52, when after a minor head trauma occurring during worktime, she started showing delusional ideas of hypochondri- ac and somatic content believing to have developed a “problem in the head”. Two years later she was admitted to a Psychiatric inpatient unit and diagnosed with a delusional disorder of the somatic subtype. At discharge she maintained the delusional ideas, which, however, were encapsulated from her personality and quiescent, while exhibiting no insight into her psychopatho- logical state. Very shortly thereafter, at follow-up in the outpatient clinic, she stopped all drug therapy (oral antipsychotic drugs. One year later, she was readmitted to the inpatient unit upon worsening of the hypochondriac and somatic delusional ideas. The prescribed medication was switched to depot injection, which she also stopped shortly thereafter. Three years later, being 58 years of age, she began to show manic symptoms of crescendo severity (grandiose delusion-like ideas, elated mood, overactivity, disinhibition, acceleration of thinking, reduced need for sleep and increased pres- sure of speech. This clinical condition gets worse, with persecutory delusional ideas and complex auditory hallucinations and she was admitted to the inpatient unit once more. This time she presents a full manic episode and a Bipolar I affective disorder diagnosis was made. She had a hyperthymic pre-morbid temperament. For the next 4 years, the patient remained somewhat stable with elation of mood, grandiose ideas, increased pressure of speech, eccen- tric clothing and lack of insight to her psychopathological state. Since the beginning of follow up, the patient always kept poor treatment compliance. The authors discuss the evolution and clinical significance of a particular and infrequent type of Bipolar Disorder, chronic mania.

  10. Analysis of genomic aberrations and gene expression profiling identifies novel lesions and pathways in myeloproliferative neoplasms

    International Nuclear Information System (INIS)

    Polycythemia vera (PV), essential thrombocythemia and primary myelofibrosis, are myeloproliferative neoplasms (MPNs) with distinct clinical features and are associated with the JAK2V617F mutation. To identify genomic anomalies involved in the pathogenesis of these disorders, we profiled 87 MPN patients using Affymetrix 250K single-nucleotide polymorphism (SNP) arrays. Aberrations affecting chr9 were the most frequently observed and included 9pLOH (n=16), trisomy 9 (n=6) and amplifications of 9p13.3–23.3 (n=1), 9q33.1–34.13 (n=1) and 9q34.13 (n=6). Patients with trisomy 9 were associated with elevated JAK2V617F mutant allele burden, suggesting that gain of chr9 represents an alternative mechanism for increasing JAK2V617F dosage. Gene expression profiling of patients with and without chr9 abnormalities (+9, 9pLOH), identified genes potentially involved in disease pathogenesis including JAK2, STAT5B and MAPK14. We also observed recurrent gains of 1p36.31–36.33 (n=6), 17q21.2–q21.31 (n=5) and 17q25.1–25.3 (n=5) and deletions affecting 18p11.31–11.32 (n=8). Combined SNP and gene expression analysis identified aberrations affecting components of a non-canonical PRC2 complex (EZH1, SUZ12 and JARID2) and genes comprising a ‘HSC signature' (MLLT3, SMARCA2 and PBX1). We show that NFIB, which is amplified in 7/87 MPN patients and upregulated in PV CD34+ cells, protects cells from apoptosis induced by cytokine withdrawal

  11. Myeloproliferative neoplasms (BCR-ABL1 negative) and myelodysplastic/myeloproliferative neoplasms: current diagnostic principles and upcoming updates.

    Science.gov (United States)

    Geyer, J T; Orazi, A

    2016-05-01

    Since the publication of the latest World Health Organization (WHO) classification in 2008, there has been a significant effort for clarification of unresolved questions, especially with the help of the rapidly developing field of molecular genetic studies, next-generation sequencing in particular. Numerous entities within the WHO categories of myeloproliferative neoplasms (MPNs) and myelodysplastic (MDS)/MPNs have been extensively studied, with large published series attempting to characterize and better define their morphologic and molecular genetic features. This emerging genetic landscape maintains a robust correlation with the various disease entities recognized by the WHO classification scheme based on a careful integration of detailed clinical information, bone marrow and peripheral blood morphology, immunohistology, and genomics. This brief review summarizes the current guidelines as they apply to diagnosing both the classical BCR-ABL1 negative MPN (polycythemia vera, essential thrombocythemia, and primary myelofibrosis) and the more common subtypes of MDS/MPN overlap syndromes. The more important recent molecular updates as well as the upcoming changes to the current WHO classification, expected to be published in late 2016, will also be briefly reviewed. PMID:27161873

  12. Impairments in Object Relations and Chronicity of Suicidal Behavior in Individuals With Borderline Personality Disorder.

    Science.gov (United States)

    Lewis, Katie C; Meehan, Kevin B; Cain, Nicole M; Wong, Philip S; Clemence, A Jill; Stevens, Jennifer; Tillman, Jane G

    2016-02-01

    While studies have demonstrated connections between impairments in object relations and self-destructive behaviors in individuals with borderline personality disorder (BPD), few have investigated whether these impairments relate to actual suicidal behaviors. The current study utilized the Social Cognition and Object Relations Scale-Global Method to investigate object relational functioning and suicidal behaviors in 131 residential treatment patients. Cognitive but not affective aspects of internalized representations predicted past suicidal behavior in BPD subjects; no relationships were found between quality of object representations and suicide in other-PD subjects. Implications of these findings for research, theory, and treatment of suicidal individuals are discussed. PMID:25710732

  13. Biodegradable Magnesium (Mg) Implantation Does Not Impose Related Metabolic Disorders in Rats with Chronic Renal Failure

    Science.gov (United States)

    Wang, Jiali; Xu, Jiankun; Liu, Waiching; Li, Yangde; Qin, Ling

    2016-05-01

    Mg and its alloys have been considered as one of the most promising biodegradable medical devices, but it was still unclear whether hypermagnesemia involved health risks would occur in persons with kidney disease due to their deteriorated kidney function for Mg ions excretion from their body. In this study, we established a chronic renal failure (CRF) model in rats induced by adenine administration prior to Mg implantation, aiming to predict if CRF patients are suitable for the use of Mg implants. The results showed that Mg levels in serum, urine, feces and internal organs had no significant changes after Mg implantation for both normal and CRF rats. Biochemical indices detection and histopathological analysis in kidney, liver and heart tissue confirmed that Mg implants did not induce any extra damage in animals even with renal failure. Our study indicates that Mg based orthopaedic medical device may be considered for use in CRF patients without biosafety concerns.

  14. Review article: the potential role of nitric oxide in chronic inflammatory bowel disorders

    DEFF Research Database (Denmark)

    Perner, Anders; Rask-Madsen, J

    1999-01-01

    The aetiology of the chronic inflammatory bowel diseases-ulcerative colitis and Crohn's disease-as well as 'microscopic colitis'-both collagenous (COC) and lymphocytic colitis (LC)-remains unknown. Autoimmune mechanisms, cytokine polymorphism, commensal bacteria, infectious agents and vascular...... impairment have all been proposed as playing important roles in the pathogenesis of this spectrum of diseases. A variety of proinflammatory mediators, including tumour necrosis factor alpha, interleukin-1beta, interferon gamma, leukotriene B4 and platelet activating factor, promote the adherence of...... generation of superoxide with reduced L-arginine availability. In active ulcerative colitis, and to a lesser extent in Crohn's disease, a greatly increased production of NO has been demonstrated by indirect and direct measurements. Surprisingly, even higher rates of production have been observed in COC...

  15. The Investigation of Nail Disorders in Patients with Chronic Renal Failure Undergoing Hemodialysis

    Directory of Open Access Journals (Sweden)

    Perihan Öztürk

    2012-06-01

    Full Text Available Objective: Nail changes are often observed in patients with end-stage renal disease. These changes may occur due to chronic renal failure itself or to the treatment. This study aims to investigate the frequency of nail findings in patients undergoing hemodialysis therapy and to compare with healthy controls. Methods: One hundred and four patients with chronic renal failure treated with hemodialysis, and 104 healthy controls without any dermatological and sistemic diseases, were examined for nail signs. Groups were compared for the incidence of nail findings. Results: 74.4% of hemodialysis patients, and 51.9% of controls had at least one nail finding. The most common signs in hemodialysis patients were (58.7% absence of lunula, (40.5% streaking, (15.7% terry nail and (14.9% half and half nail. In the control group, the most common signs were vertical streaking (36.5%, absence of lunula (8.7% and coilonichi (2.9%. In hemodialysis patients, absence of lunula, beau lines, onycomycosis, terry nail, half and half nail and splinter hemorrhages were found to be significantly higher (p<0.05. Conclusion: The frequency of nail diseases in hemodialysis patients is higher than in the healthy control group. In our study, absence of lunula is the most frequently observed finding in hemodialysis patients. Although the second most common nail change was vertical streaking, it was not different from the control group statistically. We recommend that, when hemodialysis patients are examined, nail examination (as a part of physical examination should be performed. (Turk J Dermatol 2012; 6: 35-8

  16. Leptin and Adiponectin Levels in Patients with Chronic Hepatitis C with Carbohydrate and Lipid Metabolism Disorders

    Directory of Open Access Journals (Sweden)

    T. V. Antonova

    2014-01-01

    Full Text Available Aim: to analyze leptin and adiponectin serum levels in patients with chronic hepatitis C in comparison with metabolic syndrome components, biochemical features and stage of hepatitis.Materials and methods: In 93 patients with chronic HCV in age 20-55 with a few symptomatic HCV-infection and minimal liver fibrosis stage serum leptin and adiponectin was measured. Associations between leptin, adiponectin and metabolic abnormalities, biochemical features, and hepatic fibrosis were determined.Results: Abdominal obesity was revealed at 40% patients, overweight – at 41%, insulin resistance – at 36,6% cases. The leptin and adiponectin levels were within normal limits range at most patients. Patients with minimal liver fibrosis had higher index of leptin by comparison to patients with moderate and severe fibrosis (r= – 0,402, р= 0,018. In patients with HCV genotype 3a the adiponectin level was below, than in HCV genotype 1b. Patients with abdominal obesity and overweight had higher leptin and lower adiponectin indexes by comparison to patients without these metabolic abnormalities. Direct cross-correlation between the leptin level and body mass index (r=0,358, p=0,001, waist circumference (r=0,292, p=0,01; negative cross-correlation between the adiponectin level and body mass index (r=- 0,435, р <0,021, waist circumference (r=- 0,386, р =0,001 were displayed.Conclusion: Leptin and adiponectin blood levels in HCVpatientis associated with abdominal obesity and overweight. The connection of leptin level and liver fibrosis stage was revealed. Difference of adiponectin level in HCV-patients with 3a and 1b genotypes of virus was found.

  17. HLA class II genes in chronic hepatitis C virus-infection and associated immunological disorders.

    Science.gov (United States)

    Congia, M; Clemente, M G; Dessi, C; Cucca, F; Mazzoleni, A P; Frau, F; Lampis, R; Cao, A; Lai, M E; De Virgiliis, S

    1996-12-01

    To investigate the factors that may confer susceptibility or protection to hepatitis C virus (HCV) infection and to HCV-associated immunological disorders, we designed two studies on 420 Sardinian transfusion-dependent thalassemia patients followed in our department in Cagliari since 1974. The first one was an epidemiological survey aimed to evaluate the prevalence of HCV infection and HCV-associated immunological disorders. In the second study, the distribution of different HLA class II genes was examined by DNA analysis in 116 HCV positive patients, 30 HCV negative patients, and 606 healthy controls. Three hundred fourteen patients became infected with HCV (74.7%) after 5.6 +/- 2.8 years of regular transfusion program. Mixed cryoglobulinemia, purpura, arthritis, proteinuria, decreased complement levels, rheumatoid factor and anti-GOR, smooth muscle antibody (SMA), anti-nuclear antibody (ANA), and liver, kidney microsome (LKM) autoantibodies were significantly more represented in HCV positive patients than in negative ones (P patients who despite 10.3 +/- 2.2 years in a regular blood transfusion program did not show any evidence of HCV infection (Pc < .0092). Our results represent clear evidence for a relationship between HCV infection and immune extrahepatic abnormalities. A gene(s) located in the human major histocompatibility complex (MHC) region may play an important role in conferring protection against HCV infection. PMID:8938157

  18. [Reactive anxiety crisis and chronic adjustment disorder: a unique case of work injury and suspected occupational disease].

    Science.gov (United States)

    Taino, Giuseppe; Pizzuto, Cristina; Pezzuto, Cristina; Pucci, Ennio; Imbriani, Marcello

    2014-01-01

    The present study aims to describe a case of work injury and occupational disease which is unique for the type of disease diagnosed, conditions of onset and mode of management by INAIL (Italian National Institute of Insurance for Injuries at Work and Occupational Diseases). A worker, after a verbal animated dispute with some collegues and superiors, had an acute psychiatric agitation attack and went to the nearest emergency room, where he was subjected to clinical exams. No neuropsychiatric alteration was found, but the physicians diagnosed an anxiety crisis reactive to the work environment. Consequently, the medical certificate for work injury was edited and sent to INAIL. The worker has been off work for 110 days because of a anxious and depressive syndrome, due to the verbal conflict. In a later assessment, INAIL recognized only the first 30 days of the employee's time off as injury at work, while judging the following period off work as related to affectivity disturbance due to common disease, not related to work environment. The following year, "anxious-depressive syndrome" is worsened and attributed by the same worker to the recurrence of acts of persecution and discrimination against him at work. For this reason he applied for recognition of occupational disease diagnosed as "Chronic Adjustment Disorder with prolonged depressive reaction and somatic anxiety, which developed into a protracted conflict marked the employment situation". INAIL rejected that request, but in the same year the employee has submitted the complaint for "mobbing". Even this request was rejected. Literature shows many examples of traumatic events during working activities which cause psychiatric disturbances. These events include industrial disasters, explosions, transport and mining accidents, accidents in psychiatric units with high risks of assaults, armed conflicts, war, assault and sexual assault, natural disasters. Victims show symptoms of acute stress disorder (ASD) or post

  19. Predicting chronic posttraumatic stress disorder in bereaved relatives: a 6-month follow-up study.

    Science.gov (United States)

    Kristensen, Tina E; Elklit, Ask; Karstoft, Karen-Inge; Palic, Sabina

    2014-06-01

    The objectives of the present study were to examine the prevalence of posttraumatic stress disorder (PTSD) and to identify predicative risk factors for PTSD in bereaved people after a terminal illness. Fifty-four persons (mean age 60 years) participated in the study. Demographic, peritraumatic, and psychosocial factors were assessed in order to identify variables that affected PTSD severity. Six months after the loss, 21.6 % of the subjects had PTSD, an 8.6 % decrease from PTSD measured one month after the loss. Intake of medicine after the loss, place of death, not having a close intimate, negative affectivity, and the A2 criterion predicted 65 % of PTSD severity. A considerable number of the bereaved were still at great risk for developing PTSD six months after loss. PMID:23687214

  20. The effect of theophylline on sleep-disordered breathing in patients with stable chronic congestive heart failure

    Institute of Scientific and Technical Information of China (English)

    胡克; 李清泉; 杨炯; 胡苏萍; 陈喜兰

    2003-01-01

    Objective To determine the prevalence of sleep-disordered breathing in patients with stable, optimally treated chronic congestive heart failure and the effect of short-term oral theophylline therapy on periodic breathing in these patients.Methods Patients with stable, optimally treated chronic congestive heart failure were monitored by polysomnography during nocturnal sleep. The effects of theophylline therapy on periodic breathing associated with stable heart failure were observed before and after treatment.Results Patients were divided into two groups. GroupⅠ(n=21) consisted of individuals with 15 episodes of apnea and hypopnea [as determined by the apnea-hypopnea index (AHI)] per hour or less; Group Ⅱ (n=15, 41.7%) individuals had an index of more than 15 episodes per hour. In group Ⅱ, the AHI varied from 16.8 to 78.8 (42.6±15.5) in which the obstructive AHI was 11.1±8.4 and the central AHI was 31.5±9.6. Group Ⅱ had significantly more arousals (36.8±21.3 compared with 19.4±11.2 in group Ⅰ) that were directly attributable to episodes of apnea and hypopnea, lower arterial oxyhemoglobin saturation (76.7%±4.6% compared with 86.5%±2.8%) and lower left ventricular ejection fraction (24.2%±8.8% compared with 31.5%±10.6%). Thirteen patients with compensated heart failure and periodic breathing received theophylline orally (at an average dose of 4.3 mg/kg) for five to seven days. After treatment, the mean plasma theophylline concentration was (11.3±2.5) μg/ml. Theophylline therapy resulted in significant decreases in the number of AHI (20.8±13.2 vs. 42.6±15.5; P<0.001) and the number of episodes of central apnea-hypopnea per hour (10.1±7.6 vs. 31.5±9.6; P<0.001). Furthermore, the percentage of total sleep time during which arterial oxyhemoglobin saturation (SaO2) was less than 90 percent (8.8%±8.6% vs. 23.4%±24.1%; P<0.05) and the arousals per hour (18.7±21.2 vs. 36.8±21.3; P<0.05) were also lower. There were no significant differences in the

  1. Effects of oral motor exercises and laser therapy on chronic temporomandibular disorders: a randomized study with follow-up.

    Science.gov (United States)

    Machado, Barbara Cristina Zanandréa; Mazzetto, Marcelo Oliveira; Da Silva, Marco Antonio M Rodrigues; de Felício, Cláudia Maria

    2016-07-01

    This study investigated the efficacy of combining low-level laser therapy (LLLT) with oral motor exercises (OM-exercises) for rehabilitation of patients with chronic temporomandibular disorders (TMDs). Eighty-two patients with chronic TMD and 20 healthy subjects (control group) participated in the study. Patients were randomly assigned to treatment groups: GI (LLLT + OM exercises), GII (orofacial myofunctional therapy-OMT-which contains pain relief strategies and OM-exercises), and GIII (LLLT placebo + OM-exercises) and GIV (LLLT). LLLT (AsGaAl; 780-nm wavelength; average power of 60 mW, 40 s, and 60 ± 1.0 J/cm²) was used to promote analgesia, while OM-exercises were used to reestablish the orofacial functions. Evaluations at baseline (T1), after treatment immediate (T2), and at follow-up (T3) were muscle and joint tenderness to palpation, TMD severity, and orofacial myofunctional status. There was a significant improvement in outcome measures in all treated groups with stability at follow-up (Friedman test, P  0.05). Intergroup comparisons showed that all treated groups had no difference in tenderness to palpation of temporal muscle compared to GC at follow-up (Kruskal-Wallis test, P < 0.01). Moreover, GI, GII, and GIII showed no difference from GC in orofacial functional condition (T2 and T3) while they differed significantly from GIV (P < 0.01). In conclusion, LLLT combined with OM-exercises was more effective in promoting TMD rehabilitation than LLLT alone was. Similar treatment results were verified with the OMT protocol. PMID:27085322

  2. Transcranial direct current stimulation and exercises for treatment of chronic temporomandibular disorders: a blind randomised-controlled trial.

    Science.gov (United States)

    Oliveira, L B; Lopes, T S; Soares, C; Maluf, R; Goes, B T; Sá, K N; Baptista, A F

    2015-10-01

    To evaluate the effect of adding transcranial direct current stimulation (tDCS) to exercises for chronic pain, dysfunction and quality of life in subjects with temporomandibular disorders (TMD). Participants were selected based on the RDC/TMD criteria and assessed for pain intensity, pressure pain threshold over temporomandibular joint and cervical muscles and quality of life. After initial assessment, all individuals underwent a 4-week protocol of exercises and manual therapy, together with active or sham primary motor cortex tDCS. Stimulation was delivered through sponge electrodes, with 2 mA amplitude, for 20 min daily, over the first 5 days of the trial. A total of 32 subjects (mean age 24.7 ± 6.8 years) participated in the evaluations and treatment protocol. Mean pain intensity pre-treatment was 5.5 ± 1.4 for active tDCS group, and 6.3 ± 1.2 for sham tDCS. Both groups showed a decrease in pain intensity scores during the trial period (time factor--F(4.5,137.5) = 28.7, P 0.05). Pressure pain thresholds decrease and improvement in quality of life were also noticeable in both groups, but again without significant differences between them. Absolute benefit increase was 37.5% (CI 95%: -15.9% to 90.9%), and number needed to treat was 2.66. This study suggests that there is no additional benefit in adding tDCS to exercises for the treatment of chronic TMD in young adults. PMID:25891021

  3. The pharmacology of neurotrophic treatment with Cerebrolysin: brain protection and repair to counteract pathologies of acute and chronic neurological disorders.

    Science.gov (United States)

    Masliah, E; Díez-Tejedor, E

    2012-04-01

    Neurotrophic factors are considered as part of the therapeutic strategy for neurological disorders like dementia, stroke and traumatic brain injury. Cerebrolysin is a neuropeptide preparation which mimics the action of endogenous neurotrophic factors on brain protection and repair. In dementia models, Cerebrolysin decreases β-amyloid deposition and microtubule-associated protein tau phosphorylation by regulating glycogen synthase kinase-3β and cyclin-dependent kinase 5 activity, increases synaptic density and restores neuronal cytoarchitecture. These effects protect integrity of the neuronal circuits and thus result in improved cognitive and behavioral performance. Furthermore, Cerebrolysin enhances neurogenesis in the dentate gyrus, the basis for neuronal replacement therapy in neurodegenerative diseases. Experimental studies in stroke animal models have shown that Cerebrolysin stabilizes the structural integrity of cells by inhibition of calpain and reduces the number of apoptotic cells after ischemic lesion. Cerebrolysin induces restorative processes, decreases infarct volume and edema formation and promotes functional recovery. Stroke-induced neurogenesis in the subventricular zone was also promoted by Cerebrolysin, thus supporting the brain's self-repair after stroke. Both, traumatic brain and spinal cord injury conditions stimulate the expression of natural neurotrophic factors to promote repair and regeneration processes -axonal regeneration, neuronal plasticity and neurogenesis- that is considered to be crucial for the future recovery. Neuroprotective effects of Cerebrolysin on experimentally induced traumatic spinal cord injury have shown that Cerebrolysin prevents apoptosis of lesioned motoneurons and promotes functional recovery. This section summarizes the most relevant data on the pharmacology of Cerebrolysin obtained from in vitro assays (biochemical and cell cultures) and in vivo animal models of acute and chronic neurological disorders. PMID

  4. The traumatised chronic pain patient—Prevalence of posttraumatic stress disorder - PTSD and pain sensitisation in two Scandinavian samples referred for pain rehabilitation

    DEFF Research Database (Denmark)

    Andersen, Tonny Elmose; Andersen, Per Grünwald; Vakkala, Merja Annika;

    2012-01-01

    Introduction: Correctly identifying chronic pain patients with posttraumatic stress disorder (PTSD) is important because the comorbidity of a chronic pain condition and PTSD is found to compromise treatment success. In addition, the existence of PTSD is associated with pain sensitisation, elevated...... gender differences in PTSD. The three most reported traumatic events: traffic accidents, serious illness personally or in the family, and the actual loss of someone, were reported as the primary traumatic events by almost 50% of those with PTSD. No particular pain diagnosis was significantly related to...

  5. Chronic fatigue syndrome in middle-aged women: the role of disorders of glucose metabolism

    Directory of Open Access Journals (Sweden)

    Anastasiya V Pleshcheva

    2014-12-01

    Full Text Available Objectives: To determine the prevalence of chronic fatigue syndrome (CFS among middle-aged women and to assess the role of glucose metabolism disturbances in the development of this pathology. Materials and Methods: The study included 231 women from 40 to 60 years old (mean age 52.3 ± 5 years, observed at urban polyclinic in Moscow, who was referred to or was observed by an endocrinologist (n = 142, group 1, therapist (n = 56, group 2 or had a prophylactic medical examinations (n = 33, group 3. We recorded demographic and anthropometric data, accessed levels of glucose, glycated hemoglobin, insulin and calculated BMI and HOMA indexes. Results: The prevalence of CFS in the whole group of patients studied was 27%. The highest frequency of CFS is registered in the group of patients observed by the endocrinologist – 35%, and at dispensary examination – 21%, which was significantly different from the prevalence of CFS in patients seeking an appointment with a therapist – 13% (p = 0.002 and p = 0.03 for Fisher's exact test, respectively. Higher BMI, blood glucose, glycosylated hemoglobin, and HOMA insulin resistance index as naturally expected were observed in group 1, but after further division for the presence or absence of CFS, significant differences for patients with CFS were only higher fasting glucose levels in group 2. Conclusions: We determined the prevalence of CFS in the examined groups of patients and showed only a weak correlation of CFS and disturbances of glucose metabolism.

  6. Progressive or degressive compression pressure profile in patients with chronic venous disorders of the lower limb

    Directory of Open Access Journals (Sweden)

    Giovanni Mosti

    2014-03-01

    Full Text Available Graduated compression devices are considered the standard care for management of venous and lymphatic disorders. Recently compression devices exerting a pressure over the calf higher than over the ankle have been proved to be more effective than traditional graduated devices in increasing the impaired ejection fraction (EF from the lower leg in patients with venous disease. Aim of this work is presenting an overview of the new concept on progressive compression, its potential benefits and limits. In different series of tests, the EF from the lower leg was assessed in 70 patients with severe reflux in the great saphenous vein (GSV. EF was measured by strain gauge plethysmography, in baseline conditions and after applying graduated compression devices or the new inversely graduated or progressive compression (PC devices. The interface pressure was recorded, simultaneously with the EF, both in the gaiter area (B1 point and at the calf (C point in order to assess the compression pressure profile. EF, severely impaired in patients with GSV reflux, was increased by compression. So called PC devices (both PC elastic stocking and PC inelastic bandages were significantly more effective than graduated compression in increasing the ejection fraction. The higher the pressure on the calf the higher the EF improvement. Maintaining the same strong pressure over the calf by means of two progressive stockings and increasing the pressure only over the calf to restore a graduated compression didn’t improve the EF. To improve venous pumping function in the ambulant patient stronger compression of the calf is more effective than graduated compression. This can be explained by the higher amount of blood volume pooled in the calf veins.

  7. Associations among temporomandibular disorders, chronic neck pain and neck pain disability in computer office workers: a pilot study.

    Science.gov (United States)

    Bragatto, M M; Bevilaqua-Grossi, D; Regalo, S C H; Sousa, J D; Chaves, T C

    2016-05-01

    Neck pain is the most common musculoskeletal complaint among computer office workers. There are several reports about the coexistence of neck pain and temporomandibular disorders (TMD). However, there are no studies investigating this association in the context of work involving computers. The purpose of this study was to verify the association between TMD and neck pain in computer office workers. Fifty-two female computer workers who were divided into two groups: (i) those with self-reported chronic neck pain and disability (WNP) (n = 26) and (ii) those without self-reported neck pain (WONP) (n = 26), and a control group (CG) consisting of 26 women who did not work with computers participated in this study. Clinical assessments were performed to establish a diagnosis of TMD, and craniocervical mechanical pain was assessed using manual palpation and pressure pain threshold (PPT). The results of this study showed that the WNP group had a higher percentage of participants with TMD than the WONP group (42·30% vs. 23·07%, χ(2) = 5·70, P = 0·02). PPTs in all cervical sites were significantly lower in the groups WNP and WONP compared to the CG. Regression analysis revealed TMD, neck pain and work-related factors to be good predictors of disability (R(2) = 0·93, P workplace conditions. Consequently, this study attempted to emphasise the importance of considering work activity for minimising neck pain-related disability. PMID:26732204

  8. Transcranial Alternating Current Stimulation in Patients with Chronic Disorder of Consciousness: A Possible Way to Cut the Diagnostic Gordian Knot?

    Science.gov (United States)

    Naro, Antonino; Bramanti, Placido; Leo, Antonino; Russo, Margherita; Calabrò, Rocco Salvatore

    2016-07-01

    Unresponsive wakefulness syndrome (UWS) is a chronic disorder of consciousness (DOC) characterized by a lack of awareness and purposeful motor behaviors, owing to an extensive brain connectivity impairment. Nevertheless, some UWS patients may retain residual brain connectivity patterns, which may sustain a covert awareness, namely functional locked-in syndrome (fLIS). We evaluated the possibility of bringing to light such residual neural networks using a non-invasive neurostimulation protocol. To this end, we enrolled 15 healthy individuals and 26 DOC patients (minimally conscious state-MCS- and UWS), who underwent a γ-band transcranial alternating current stimulation (tACS) over the right dorsolateral prefrontal cortex. We measured the effects of tACS on power and partial-directed coherence within local and long-range cortical networks, before and after the protocol application. tACS was able to specifically modulate large-scale cortical effective connectivity and excitability in all the MCS participants and some UWS patients, who could be, therefore, considered as suffering from fLIS. Hence, tACS could be a useful approach in supporting a DOC differential diagnosis, depending on the level of preservation of the cortical large-scale effective connectivity. PMID:27062669

  9. [Transition to adult care for children with chronic neurological disorders; which is the best way to make it?].

    Science.gov (United States)

    Moreno Villares, José Manuel

    2014-01-01

    Chronic neurological disorders in children have significant effects on adult medical and social function. Transition from pediatric to adult services is a complex process. No objective data are available to inform physicians about the most effective approach. Nevertheless the most recommended approach is a joint pediatric/adult transition clinic. Malnutrition, either under or overnutrition, is a common condition among neurologically impaired children. Undernutrition is most prevalent, and its causes are diverse: insufficient caloric intake, excessive nutrient losses and abnormal energy metabolism. Malnutrition is associated with significant morbidity, while nutritional rehabilitation improves overall health as well as quality of life. It is not easy to determine which the nutritional needs in these patients are. Besides, they often present difficulties for oral feeding, mainly due to oromotor dysfunction. Gastrointestinal symptoms, gastro esophageal reflux and constipation, as well as spasticity, scoliosis and joint deformities contribute to these difficulties. Because of that, an assessment of nutritional status should be performed periodically, and to assess efficacy and security of oral intake. If modifying oral diet we cannot confirm an adequate support, a nasogastric tube or a gastrostomy need to be considered. Often, a fundoplication is associated to the placement of a gastrostomy. Although the outcomes in a better nutritional status and quality of life are often obtained, it is not an easy decision for families. PMID:25077342

  10. Renal thrombotic microangiopathy and FIP1L1/PDGFRα-associated myeloproliferative variant of hypereosinophilic syndrome

    OpenAIRE

    Langlois, Anne Lyse; Shehwaro, Nathalie; Rondet, Claire; Benbrik, Youssef; Maloum, Karim; Gueutin, Victor; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    We report a case of renal thrombotic microangiopathy (TMA) in a myeloproliferative variant of hypereosinophilic syndrome (HES) in a 24-year-old man which resolved with imatinib therapy. This is one of a few cases in the literature to date describing TMA in HES, suggesting that the pathogenesis of thrombosis is at least in part related to damage from activated eosinophils.

  11. Renal thrombotic microangiopathy and FIP1L1/PDGFRα-associated myeloproliferative variant of hypereosinophilic syndrome

    Science.gov (United States)

    Langlois, Anne Lyse; Shehwaro, Nathalie; Rondet, Claire; Benbrik, Youssef; Maloum, Karim; Gueutin, Victor; Rouvier, Philippe; Izzedine, Hassane

    2013-01-01

    We report a case of renal thrombotic microangiopathy (TMA) in a myeloproliferative variant of hypereosinophilic syndrome (HES) in a 24-year-old man which resolved with imatinib therapy. This is one of a few cases in the literature to date describing TMA in HES, suggesting that the pathogenesis of thrombosis is at least in part related to damage from activated eosinophils. PMID:27293571

  12. 2012478 Biological characteristics of bone marrow mesenchymal stem cells and JAK2 mutation in myeloproliferative neoplasms

    Institute of Scientific and Technical Information of China (English)

    田竑

    2012-01-01

    Objective To study the biological characteristics of bone marrow mesenchymal stem cells(BMSCs) and detect JAK2 mutation in BMSCs from myeloproliferative neoplasms(MPN) patients. Methods JAK2 V617F mutation and exon 12 mutation in 70 MPN patients’ blood or bone marrow samples were detected.

  13. JAK2 exon 12 mutations in patients with Philadelphia(Ph) chromosome-negative myeloproliferative neoplasms

    Institute of Scientific and Technical Information of China (English)

    王婕妤

    2012-01-01

    Objective To investigate JAK2 exon 12 mutations in patients with Philadelphia (Ph) chromosome-negative myeloproliferative neoplasms (MPN) and the clinical characteristics of patients with JAK2 exon 12 mutants. Methods Allele-specific PCR(AS-PCR) was applied to identify JAK2 V617F mutation.

  14. Chronic inflammation as a promotor of mutagenesis in essential thrombocythemia, polycythemia vera and myelofibrosis. A human inflammation model for cancer development?

    DEFF Research Database (Denmark)

    Hasselbalch, Hans K

    2013-01-01

    The Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) are acquired stem cell neoplasms, in which a stem cell lesion induces an autonomous proliferative advantage. In addition to the JAK2V617 mutation several other mutations have been described. Recently chronic inflammation has be...

  15. G-CSF priming, clofarabine, and high dose cytarabine (GCLAC) for upfront treatment of acute myeloid leukemia, advanced myelodysplastic syndrome or advanced myeloproliferative neoplasm.

    Science.gov (United States)

    Becker, Pamela S; Medeiros, Bruno C; Stein, Anthony S; Othus, Megan; Appelbaum, Frederick R; Forman, Stephen J; Scott, Bart L; Hendrie, Paul C; Gardner, Kelda M; Pagel, John M; Walter, Roland B; Parks, Cynthia; Wood, Brent L; Abkowitz, Janis L; Estey, Elihu H

    2015-04-01

    Prior study of the combination of clofarabine and high dose cytarabine with granulocyte colony-stimulating factor (G-CSF) priming (GCLAC) in relapsed or refractory acute myeloid leukemia resulted in a 46% rate of complete remission despite unfavorable risk cytogenetics. A multivariate analysis demonstrated that the remission rate and survival with GCLAC were superior to FLAG (fludarabine, cytarabine, G-CSF) in the relapsed setting. We therefore initiated a study of the GCLAC regimen in the upfront setting in a multicenter trial. The objectives were to evaluate the rates of complete remission (CR), overall and relapse-free survival (OS and RFS), and toxicity of GCLAC. Clofarabine was administered at 30 mg m(-2) day(-1) × 5 and cytarabine at 2 g m(-2) day(-1) × 5 after G-CSF priming in 50 newly-diagnosed patients ages 18-64 with AML or advanced myelodysplastic syndrome (MDS) or advanced myeloproliferative neoplasm (MPN). Responses were assessed in the different cytogenetic risk groups and in patients with antecedent hematologic disorder. The overall CR rate was 76% (95% confidence interval [CI] 64-88%) and the CR + CRp (CR with incomplete platelet count recovery) was 82% (95% CI 71-93%). The CR rate was 100% for patients with favorable, 84% for those with intermediate, and 62% for those with unfavorable risk cytogenetics. For patients with an antecedent hematologic disorder (AHD), the CR rate was 65%, compared to 85% for those without an AHD. The 60 day mortality was 2%. Thus, front line GCLAC is a well-tolerated, effective induction regimen for AML and advanced myelodysplastic or myeloproliferative disorders. PMID:25545153

  16. Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management.

    Science.gov (United States)

    Elliott, Michelle A; Tefferi, Ayalew

    2016-03-01

    Chronic neutrophilic leukemia (CNL) is a potentially aggressive myeloproliferative neoplasm, for which current WHO diagnostic criteria include leukocytosis of ≥25 × 10(9) /L (of which >80% are neutrophils) and with strategies, but the foundations for these are strengthening. Am. J. Hematol. 91:342-349, 2016. © 2015 Wiley Periodicals, Inc. PMID:26700908

  17. Tourette syndrome and chronic tic disorder are associated with lower socio-economic status: Findings from the Avon Longitudinal Study of Parents and Children cohort

    OpenAIRE

    Miller, LL; Scharf, JM; Mathews, CA; Ben-Shlomo, Y.

    2013-01-01

    Aim: Only a few studies have examined the relationship between Tourette syndrome or chronic tic disorder and socio-economic status (SES). Existing studies are primarily cross-sectional, arise from specialty clinics, and use single measures of SES. In this study we examine this relationship in a longitudinal, population-based sample. Method: Data are from 7152 children born during 1991 and 1992 in the county of Avon, UK, from the Avon Longitudinal Study of Parents and Children, who were follow...

  18. Tourette syndrome and chronic tic disorder are associated with lower socio-economic status: findings from the Avon Longitudinal Study of Parents and Children cohort

    OpenAIRE

    Miller, Laura L.; Scharf, Jeremiah M.; Mathews, Carol A; Ben-Shlomo, Yoav

    2013-01-01

    Aim Only a few studies have examined the relationship between Tourette syndrome or chronic tic disorder and socio-economic status (SES). Existing studies are primarily cross-sectional, arise from specialty clinics, and use single measures of SES. In this study we examine this relationship in a longitudinal, population-based sample. Method Data are from 7152 children born during 1991 and 1992 in the county of Avon, UK, from the Avon Longitudinal Study of Parents and Children, who were followed...

  19. Diagnostic Workup for Disorders of Bone and Mineral Metabolism in Patients with Chronic Kidney Disease in the Era of KDIGO Guidelines

    OpenAIRE

    Luigi Francesco Morrone; Domenico Russo; Biagio Di Iorio

    2011-01-01

    KDIGO (Kidney Disease: Improving Global Outcomes) is an international nonprofit organization devoted to “improve the care and outcomes of kidney disease patients worldwide through promoting coordination, collaboration, and integration of initiatives to develop and implement clinical practice guidelines.” The mineral and bone disorder (MBD) in patients with chronic kidney disease (CKD) has been the first area of interest of KDIGO international initiative. KDIGO guidelines on CKD-MBD were publi...

  20. Eysenck's Two Big Personality Factors and Their Relationship to Depression in Patients with Chronic Idiopathic Pain Disorder: A Clinimetric Validation Analysis

    OpenAIRE

    Marianne Lunde; Per Bech; Stine Bjerrum Møller

    2012-01-01

    Aim. The clinimetric aspects of Eysenck’s two big personality factors (neuroticism and extraversion) were originally identified by principal component analysis but have been insufficiently analysed with item response theory models. Their relationship to states of melancholia and anxiety was subsequently analysed. Method. Patients with chronic idiopathic pain disorder were included in the study. The nonparametric item response model (Mokken) was compared to the coefficient alpha to validate th...

  1. Self-awareness of depression and life events in three groups of patients: Psychotic depression, obsessive–compulsive disorder and chronic medical illness in North India

    OpenAIRE

    Gupta, Anjali; Bahadur, Indu; Gupta, K.R.; Bhugra, Dinesh

    2006-01-01

    Background: Depression is a common experience across cultures although not all languages have words describing depression. Aim: To identify patients' perception and awareness of depression as an illness. Methods: Sixty psychiatric patients (each with depression or obsessive–compulsive disorder [OCD]) were compared with 30 medical patients with chronic physical illness and assessed on levels of awareness of depression in relation to life events. Results: Life events were more in patients with ...

  2. Biological Therapy Following Chemotherapy and Peripheral Stem Cell Transplantation in Treating Patients With Cancer

    Science.gov (United States)

    2013-03-25

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Kidney Cancer; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Neuroblastoma; Ovarian Cancer; Sarcoma; Testicular Germ Cell Tumor

  3. St. John's Wort in Relieving Fatigue in Patients Undergoing Chemotherapy or Hormone Therapy for Cancer

    Science.gov (United States)

    2013-01-17

    Chronic Myeloproliferative Disorders; Fatigue; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  4. Interleukin-12 in Treating Patients With Hematologic Cancers or Solid Tumors

    Science.gov (United States)

    2014-09-09

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Kidney Cancer; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Neuroblastoma; Ovarian Cancer; Testicular Germ Cell Tumor

  5. Lymphocyte Infusion in Treating Patients With Relapsed Cancer After Bone Marrow or Peripheral Stem Cell Transplantation

    Science.gov (United States)

    2011-11-28

    Breast Cancer; Chronic Myeloproliferative Disorders; Gestational Trophoblastic Tumor; Kidney Cancer; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Neuroblastoma; Ovarian Cancer; Sarcoma; Testicular Germ Cell Tumor

  6. The effects of Gilles de la Tourette syndrome and other chronic tic disorders on quality of life across the lifespan: a systematic review.

    Science.gov (United States)

    Evans, Joel; Seri, Stefano; Cavanna, Andrea E

    2016-09-01

    Gilles de la Tourette syndrome (GTS) and other chronic tic disorders are neurodevelopmental conditions characterized by the presence of tics and associated behavioral problems. Whilst converging evidence indicates that these conditions can affect patients' quality of life (QoL), the extent of this impairment across the lifespan is not well understood. We conducted a systematic literature review of published QoL studies in GTS and other chronic tic disorders to comprehensively assess the effects of these conditions on QoL in different age groups. We found that QoL can be perceived differently by child and adult patients, especially with regard to the reciprocal contributions of tics and behavioral problems to the different domains of QoL. Specifically, QoL profiles in children often reflect the impact of co-morbid attention-deficit and hyperactivity symptoms, which tend to improve with age, whereas adults' perception of QoL seems to be more strongly affected by the presence of depression and anxiety. Management strategies should take into account differences in age-related QoL needs between children and adults with GTS or other chronic tic disorders. PMID:26880181

  7. The Polymorphisms in LNK Gene Correlated to the Clinical Type of Myeloproliferative Neoplasms

    Science.gov (United States)

    Hu, Yang; Liu, Qian; Bu, Dingfang; Tan, Mei; Wu, Liusong; Zhu, Ping

    2016-01-01

    Objective LNK is an adapter protein negatively regulating the JAK/STAT cell signaling pathway. In this study, we observed the correlation between variation in LNK gene and the clinical type of myeloproliferative neoplasms (MPN). Methods A total of 285 MPN cases were recruited, including essential thrombocythemia (ET) 154 cases, polycythemia vera (PV) 76 cases, primary myelofibrosis (PMF) 19 cases, and chronic myeloid leukemia (CML) 36 cases. Ninety-three healthy individuals were used as normal controls. V617F mutation in JAK2 was identified by allele-specific PCR method, RT-PCR was used for the detection of BCR/ABL1 fusion gene, and mutations and variations in coding exons and their flanking sequences of LNK gene were examined by PCR-sequencing. Results Missense mutations of A300V, V402M, and R415H in LNK were found in 8 patients including ET (4 cases, all combined with JAK2-V617F mutation), PV (2 cases, one combined with JAK2-V617F mutation), PMF (one case, combined with JAK2-V617F mutation) and CML (one case, combined with BCR/ABL1 fusion gene). The genotype and allele frequencies of the three SNPs (rs3184504, rs111340708 and rs78894077) in LNK were significantly different between MPN patients and controls. For rs3184504 (T/C, in exon2), the T allele (p.262W) and TT genotype were frequently seen in ET, PV and PMF (P<0.01), and C allele (p.262R) and CC genotype were frequently seen in CML (P<0.01). For rs78894077 (T/C, in exon1), the T allele (p.242S) was frequently found in ET (P<0.05). For rs111340708 (TGGGGx5/TGGGGx4, in intron 5), the TGGGG x4 allele was infrequently found in ET, PMF and CML(P<0.01). Conclusion Mutations in LNK could be found in some of MPN patients in the presence or absence of JAK2-V617F mutation. Several polymorphisms in LNK gene may affect the clinical type or the genetic predisposition of MPN. PMID:27111338

  8. The Efficacy of Syzygium aromaticum Essential Oil in Cognitive Disorders against Manganese Chronic Exposure in Rats during Development

    Directory of Open Access Journals (Sweden)

    Djallal Eddine Houari ADLI

    2014-06-01

    Full Text Available The essential oil of Syzygium aromaticum has been widely used in traditional medicine to treat a variety of diseases, including some neurological disorders. This study aims at testing, in vivo, the possible anxiolytic and antidepressant effects, of the Syzygium aromaticum essential oil against chronic manganese chloride (4.79 mg/l intoxication during the gestation and lactation period, in Wistar rat pups. Wistar rat pups were exposed to manganese via their dams’ drinking water from postnatal day (PND 1 to (PND 21. After their weaning, the rats exposed to manganese received injections of essential oil of Syzygium aromaticum (0.1 ml/kg for 18 days. The level of anxiety, depression and locomotor activity were studied. Locomotor activity (open field test, anxiety (elevated plus maze tests, and depression (forced swimming test were evaluated. The results of the present study indicate that Manganese exposure induces, on the one hand, impairments of body (p<0.001 and of brain weight (p<0.05. On the other hand, it increases level of anxiety (p<0.05, depression (p<0.001 and locomotor hyporactivity (p<0.001, when compared to control rats. Administration of essential oil of Syzygium aromaticum leads to a reduction in the level of anxiety (p<0.05, of depression (p<0.001 and corrects locomotor hyporactivity (p<0.05 in rats exposed to manganese beforehand. These results suggest that essential oil of Syzygium aromaticum can employ as a natural, protective agent against neuro-toxicity induced by manganese chloride during the gestation and lactation periods.

  9. Genetic and Environmental Risk for Chronic Pain and the Contribution of Risk Variants for Major Depressive Disorder: A Family-Based Mixed-Model Analysis

    Science.gov (United States)

    McIntosh, Andrew M.; Zeng, Yanni; Davies, Gail; Clarke, Toni-Kim; Hayward, Caroline; Haley, Chris S.; Porteous, David J.; Deary, Ian J.; Smith, Daniel J.; Hinds, David A.; Jones, Amy V.; Scollen, Serena; Meng, Weihua; Hocking, Lynne J.

    2016-01-01

    Background Chronic pain is highly prevalent and a significant source of disability, yet its genetic and environmental risk factors are poorly understood. Its relationship with major depressive disorder (MDD) is of particular importance. We sought to test the contribution of genetic factors and shared and unique environment to risk of chronic pain and its correlation with MDD in Generation Scotland: Scottish Family Health Study (GS:SFHS). We then sought to replicate any significant findings in the United Kingdom Biobank study. Methods and Findings Using family-based mixed-model analyses, we examined the contribution of genetics and shared family environment to chronic pain by spouse, sibling, and household relationships. These analyses were conducted in GS:SFHS (n = 23,960), a family- and population-based study of individuals recruited from the Scottish population through their general practitioners. We then examined and partitioned the correlation between chronic pain and MDD and estimated the contribution of genetic factors and shared environment in GS:SFHS. Finally, we used data from two independent genome-wide association studies to test whether chronic pain has a polygenic architecture and examine whether genomic risk of psychiatric disorder predicted chronic pain and whether genomic risk of chronic pain predicted MDD. These analyses were conducted in GS:SFHS and repeated in UK Biobank, a study of 500,000 from the UK population, of whom 112,151 had genotyping and phenotypic data. Chronic pain is a moderately heritable trait (heritability = 38.4%, 95% CI 33.6% to 43.9%) that is significantly concordant in spouses (variance explained 18.7%, 95% CI 9.5% to 25.1%). Chronic pain is positively correlated with depression (ρ = 0.13, 95% CI 0.11 to 0.15, p = 2.72x10-68) and shows a tendency to cluster within families for genetic reasons (genetic correlation = 0.51, 95%CI 0.40 to 0.62, p = 8.24x10-19). Polygenic risk profiles for pain, generated using independent GWAS

  10. Shorter Course Tacro After NMA, Related Donor PBSCT With High-dose Posttransplant Cy for Hard-to-Engraft Malignancies

    Science.gov (United States)

    2016-08-24

    Myelodysplastic Syndrome; Chronic Myelomonocytic Leukemia; Small Lymphocytic Lymphoma; Chronic Lymphocytic Leukemia; Prolymphocytic Leukemia; Chronic Myeloid Leukemia; Chronic Myeloproliferative Disorders; Multiple Myeloma; Plasma Cell Neoplasm; Plasma Cell Dyscrasia; Myelofibrosis; Polycythemia Vera; Essential Thrombocythemia; Plasma Cell Leukemia

  11. Chronic granulomatous disease

    Science.gov (United States)

    CGD; Fatal granulomatosis of childhood; Chronic granulomatous disease of childhood; Progressive septic granulomatosis ... In chronic granulomatous disease (CGD), immune system cells called ... some types of bacteria and fungi. This disorder leads to long- ...

  12. Effects of conventional versus multimodal vestibular rehabilitation on functional capacity and balance control in older people with chronic dizziness from vestibular disorders: design of a randomized clinical trial

    Directory of Open Access Journals (Sweden)

    Ricci Natalia

    2012-12-01

    Full Text Available Abstract Background There are several protocols designed to treat vestibular disorders that focus on habituation, substitution, adaptation, and compensation exercises. However, protocols that contemplate not only vestibular stimulation but also other components that are essential to the body balance control in older people are rare. This study aims to compare the effectiveness of two vestibular rehabilitation protocols (conventional versus multimodal on the functional capacity and body balance control of older people with chronic dizziness due to vestibular disorders. Methods/design A randomized, single-blind, controlled clinical trial with a 3 months follow-up period will be performed. The sample will be composed of older individuals with a clinical diagnosis of chronic dizziness resulting from vestibular disorders. The subjects will be evaluated at baseline, post-treatment and follow-up. Primary outcomes will be determined in accordance with the Dizziness Handicap Inventory (functional capacity and the Dynamic Gait Index (body balance. Secondary outcomes include dizziness features, functional records, body balance control tests, and psychological information. The older individuals (minimum sample n = 68 will be randomized to either the conventional or multimodal Cawthorne&Cooksey protocols. The protocols will be performed during individual 50-minute sessions, twice a week, for 2 months (a total of 16 sessions. The outcomes of both protocols will be compared according to the intention-to-treat analysis. Discussion Vestibular rehabilitation through the Cawthorne&Cooksey protocol has already proved to be effective. However, the addition of other components related to body balance control has been proposed to improve the rehabilitation of older people with chronic dizziness from vestibular disorders. Trial registration ACTRN12610000018011

  13. Blood responses under chronic low daily dose gamma irradiation: Pt.1

    International Nuclear Information System (INIS)

    Male beagles chronically exposed to low daily doses of 60Co γ rays show one of three hematopoietic patterns, which reflect three different distinctly responding subgroups: (1) low radioresistance with progressing aplastic anemia and shortened survival (-S-AA subgroup); (2) high radioresistance with a complex of progressing myeloproliferative disorders (+R-MPD group); or (3) high radioresistance with other nonMPD syndromes (+R-nonMPD group). Blood cell levels (granulocytes, monocytes, erythrocytes, lymphocytes, and platelets) were assessed and fitted to a flexible polynomial spline model. Results showed that relative to the overall magnitude of blood cell loss as well as to the maximum rate of suppression during the initial phase, the subgroups were generally ranked -S-AA >> +R-MPD > +R-nonMPD. Relative to the overall strength of the recovery response, the subgroups were generally ranked +R-MPD > +R-nonMPD >>> -S-AA. In terms of overall maintenance levels of circulating blood cells during the recovery phase, however, the +R-nonMPD subgroup consistently exhibited stronger responses than the +R-MPD subgroup. These results support our contention that selected subgroups of dogs have strong propensities to specific hematopathologies (i.e. aplastic anemia and myeloid leukemia) under chronic irradiation and that these pathology-prone animals exhibit a series of marked differential hematopoietic responses during early preclinical phases, which serve effectively to prognosticate subsequent pathological progression. (author)

  14. Bruceine D induces apoptosis in human chronic myeloid leukemia K562 cells via mitochondrial pathway

    Science.gov (United States)

    Zhang, Jian-Ye; Lin, Min-Ting; Tung, Ho-Yi; Tang, Si-Li; Yi, Tao; Zhang, Ya-Zhou; Tang, Yi-Na; Zhao, Zhong-Zhen; Chen, Hu-Biao

    2016-01-01

    Chronic myeloid leukemia (CML), an acquired malignant myeloproliferative disorder of hematopoietic stem cells, is one of the three most common forms of leukemia. In this study, we investigated the effects of bruceine D, which have been isolated from Brucea javanica (L.) Merr. on human chronic myeloid leukemia K562 cells. MTT assay was used to evaluate cell growth inhibition. Flow cytometry was performed to analyze mitochondrial membrane potential (ΔΨm). Western blot was applied to detect expression of cytochrome c, caspases-9, -3, PARP and other proteins. Bruceine D exhibited potent cytotoxicity to K562 cells with IC50 of 6.37 ± 0.39 μM. It led to loss of ΔΨm, release of cytochrome c, activation of caspases-9, -3 and cleavage of PARP, which suggested that bruceine D induced apoptosis of K562 cells through mitochondrial pathway. In addition, bruceine D inhibited the phosphorylation of AKT and ERK. It’s indicative that the potent anticancer activity of bruceine D be related to MAPK and PI3K pathways.

  15. Chronic Granulomatous Disease (CGD)

    Science.gov (United States)

    ... Share this: Main Content Area Chronic Granulomatous Disease (CGD) Phagocyte (purple) engulfing Staphylococcus aureus bacteria (yellow). Credit: NIAID CGD is a genetic disorder in which white blood ...

  16. MPL W515L/K Mutations in Chronic Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Timur Selçuk Akpınar

    2013-03-01

    Full Text Available OBJECTIVE: The MPL gene encodes the thrombopoietin receptor. Recently MPL mutations (MPL W515L or MPL W515K were described in patients with essential thrombocythemia (ET and primary (idiopathic myelofibrosis (PMF. The prevalence and the clinical importance of these mutations are not clear. In the present study, we aimed to investigate the frequency and clinical significance of MPL W515L/K mutations in our patients with ET and PMF. METHODS: A total of 77 patients (66 were diagnosed with ET and 11 with PMF and 42 healthy controls were included in the study. Using peripheral blood samples, the presence of MPL W515L/K mutations and JAK-2 V617F mutation were analyzed by real-time polymerase chain reaction. RESULTS: In our study, MPL W515L/K or JAK-2 V617F mutations were not observed in healthy controls. JAK-2 V617F mutation was present in 35 patients, of whom 29 had ET (43.9%, 29/66 and 6 had PMF (54.5%, 6/11. In the patient group, MPL W515L/K mutations were found in only 2 PMF cases, and these cases were negative for JAK-2 V617F mutation. The prevalence of MPL W515L/K mutations in the patient group was 2.6%, and the prevalence of MPL W515L/K mutations among the cases negative for the JAK-2 V617F mutation was found to be 4.8%. The 2 cases with MPL W515L/K mutations had long follow-up times (124 months and 71 months, respectively, had no thrombotic or hemorrhagic complications, and had no additional cytogenetic anomalies. CONCLUSION: MPL W515L/K mutations may be helpful for identifying clonal disease in MPN patients with no established Ph chromosome or JAK-2 V617F mutation.

  17. MPL W515L/K Mutations in Chronic Myeloproliferative Neoplasms

    Science.gov (United States)

    Akpınar, Timur Selçuk; Hançer, Veysel Sabri; Nalçacı, Meliha; Diz-Küçükkaya, Reyhan

    2013-01-01

    Objective: The MPL gene encodes the thrombopoietin receptor. Recently MPL mutations (MPL W515L or MPL W515K) were described in patients with essential thrombocythemia (ET) and primary (idiopathic) myelofibrosis (PMF). The prevalence and the clinical importance of these mutations are not clear. In the present study, we aimed to investigate the frequency and clinical significance of MPL W515L/K mutations in our patients with ET and PMF. Materials and Methods: A total of 77 patients (66 were diagnosed with ET and 11 with PMF) and 42 healthy controls were included in the study. Using peripheral blood samples, the presence of MPL W515L/K mutations and JAK-2 V617F mutation were analyzed by real-time polymerase chain reaction. Results: In our study, MPL W515L/K or JAK-2 V617F mutations were not observed in healthy controls. JAK-2 V617F mutation was present in 35 patients, of whom 29 had ET (43.9%, 29/66) and 6 had PMF (54.5%, 6/11). In the patient group, MPL W515L/K mutations were found in only 2 PMF cases, and these cases were negative for JAK-2 V617F mutation. The prevalence of MPL W515L/K mutations in the patient group was 2.6%, and the prevalence of MPL W515L/K mutations among the cases negative for the JAK-2 V617F mutation was found to be 4.8%. The 2 cases with MPL W515L/K mutations had long follow-up times (124 months and 71 months, respectively), had no thrombotic or hemorrhagic complications, and had no additional cytogenetic anomalies. Conclusion: MPL W515L/K mutations may be helpful for identifying clonal disease in MPN patients with no established Ph chromosome or JAK-2 V617F mutation. Conflict of interest:None declared. PMID:24385746

  18. Interferon-alpha in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms. Status and perspectives

    DEFF Research Database (Denmark)

    Hasselbalch, H.C.; Larsen, T.S.; Riley, C.H.; Jensen, M.K.; Kiladjian, J.-J.

    2011-01-01

    shown that interferon-alpha (IFN-alpha) induces complete hematological remissions in a large proportion of the patients. However, its use in clinical practice has unfortunately been limited due to side effects with high drop-out rates in most studies. Recently, IFN-alpha2 has been shown to induce deep...... hyperproliferative phase of PMF. This paper reviews the history of IFN -in principle IFN-alpha2 -and its present status in the treatment of PV and related diseases. The role of IFN-alpha2 as immune therapy in the future treatment of CMPNs is highlighted and the rationale for the concept of minimal residual disease...... and potentially cure after long-term immune therapy with IFN-alpha2 is discussed and foreseen as an achievable goal in the future. © 2011 Bentham Science Publishers Ltd....

  19. Interferon-alpha in the treatment of Philadelphia-negative chronic myeloproliferative neoplasms. Status and perspectives

    DEFF Research Database (Denmark)

    Hasselbalch, Hans Carl; Larsen, Thomas Stauffer; Riley, Caroline Hasselbalch; Jensen, Morten Krogh; Kiladjian, Jean Jeaques

    2011-01-01

    shown that interferon-alpha (IFN-alpha) induces complete haematological remissions in a large proportion of the patients. However, its use in clinical practice has unfortunately been limited due to side effects with high drop-out rates in most studies. Recently, IFN-alpha2 has been shown to induce deep...... hyperproliferative phase of PMF. This paper reviews the history of IFN - in principle IFN-alpha2 - and its present status in the treatment of PV and related diseases. The role of IFN-alpha2 as immune therapy in the future treatment of CMPNs is highlighted and the rationale for the concept of minimal residual disease...... and potentially cure after long-term immune therapy with IFN-alpha2 is discussed and foreseen as an achievable goal in the future....

  20. Use of a synthetic cannabinoid in a correctional population for posttraumatic stress disorder-related insomnia and nightmares, chronic pain, harm reduction, and other indications: a retrospective evaluation.

    Science.gov (United States)

    Cameron, Colin; Watson, Diane; Robinson, Jeffrey

    2014-10-01

    Nabilone is a synthetic cannabinoid that has shown promise for the treatment of posttraumatic stress disorder (PTSD)-related insomnia and nightmares as well as efficacy in the management of chronic pain. It has also been proposed for harm reduction in cannabis dependence. Its effectiveness for management of concurrent disorders in seriously mentally ill correctional populations has not been evaluated. This retrospective study of 104 male inmates with serious mental illness prescribed nabilone analyzes the indications, efficacy, and safety of its use. Medications discontinued with the initiation of nabilone were also reviewed. The results showed nabilone targeting a mean of 3.5 indications per patient, thus likely reducing polypharmacy risk. The mean final dosage was 4.0 mg. Results indicated significant improvement in PTSD-associated insomnia, nightmares, PTSD symptoms, and Global Assessment of Functioning and subjective improvement in chronic pain. Medications associated with greater risk for adverse effects or abuse than nabilone were often able to be discontinued with the initiation of nabilone, most often antipsychotics and sedative/hypnotics. There was no evidence of abuse within this high-risk population or reduction of efficacy when nabilone was given in powder form with water rather than as a capsule. This study supports the promise of nabilone as a safe, effective treatment for concurrent disorders in seriously mentally ill correctional populations. Prospective, randomized controlled trials are required to confirm our preliminary results. Follow-up in the community will be required to confirm effectiveness in harm reduction. PMID:24987795

  1. Chronic effects of ionizing radiation on animals and humans

    International Nuclear Information System (INIS)

    Numerous experimental radiobiological studies and medical observations were conducted after the Chernobyl disaster based on the published results; patterns and characteristics influence of the fission products of nuclear materials on the body of mammals, including humans were analyzed. Chronic exposure to low doses leads to the changes in the hemopoietic system was founded and increases the risk of myeloproliferative diseases. The consequence of radiation-related immunodeficiency is the autoimmune disease and chronic fatigue syndrome. Radiation damage leads to endocrine system disruption of the body and of polipatologycal states. High radiosensitivity of the central nervous system was founded. Genotoxic chronic radiation exposure threatens the stability of the genome

  2. HSP90 is a therapeutic target in JAK2-dependent myeloproliferative neoplasms in mice and humans

    OpenAIRE

    Marubayashi, Sachie; Koppikar, Priya; Taldone, Tony; Abdel-Wahab, Omar; West, Nathan; Bhagwat, Neha; Caldas-Lopes, Eloisi; Ross, Kenneth N.; Gönen, Mithat; Gozman, Alex; Ahn, James H.; Rodina, Anna; Ouerfelli, Ouathek; Yang, Guangbin; Hedvat, Cyrus

    2010-01-01

    JAK2 kinase inhibitors were developed for the treatment of myeloproliferative neoplasms (MPNs), following the discovery of activating JAK2 mutations in the majority of patients with MPN. However, to date JAK2 inhibitor treatment has shown limited efficacy and apparent toxicities in clinical trials. We report here that an HSP90 inhibitor, PU-H71, demonstrated efficacy in cell line and mouse models of the ...

  3. Combination treatment for myeloproliferative neoplasms using JAK and pan-class I PI3K inhibitors

    OpenAIRE

    Choong, Meng Ling; Pecquet, Christian; Pendharkar, Vishal; Diaconu, Carmen C.; Yong, Jacklyn Wei Yan; Tai, Shi Jing; Wang, Si Fang; Defour, Jean-Philippe; Sangthongpitag, Kanda; Villeval, Jean-Luc; Vainchenker, William; Constantinescu, Stefan N.; Lee, May Ann

    2013-01-01

    Current JAK2 inhibitors used for myeloproliferative neoplasms (MPN) treatment are not specific enough to selectively suppress aberrant JAK2 signalling and preserve physiological JAK2 signalling. We tested whether combining a JAK2 inhibitor with a series of serine threonine kinase inhibitors, targeting nine signalling pathways and already used in clinical trials, synergized in inhibiting growth of haematopoietic cells expressing mutant and wild-type forms of JAK2 (V617F) or thrombopoietin rece...

  4. JAK2 Inhibition: Reviewing a New Therapeutical Option in Myeloproliferative Neoplasms

    OpenAIRE

    Mar Bellido; Te Boekhorst, Peter A. W.

    2012-01-01

    JAK2 is a tyrosine kinase gene that plays an essential role in the development of normal haematopoiesis. Hyperactivation of JAK2 occurs in myeloproliferative neoplasms by different mechanisms. As a consequence, JAK2 inhibitors have been designed to suppress the cytokine signalling cascade caused by the constitutive activation of JAK2. In clinical trials, JAK2 inhibitors are efficient in decreasing spleen size, controlling clinical symptoms, and improving quality of life in patients with myelo...

  5. JAK Inhibitors and other Novel Agents in Myeloproliferative Neoplasms: Are We Hitting the Target?

    OpenAIRE

    Kucine, Nicole; Levine, Ross L.

    2011-01-01

    The discovery of somatic mutations in the JAK-STAT signaling pathway was a major breakthrough in our understanding of the molecular pathogenesis of the myeloproliferative neoplasms (MPNs) polycythemia vera, essential thrombocytosis, and primary myelofibrosis. This finding led to the development of small molecule inhibitors targeting Janus kinase (JAK) 2 and other JAK family members. Currently, there are a number of research and clinical trials ongoing with JAK inhibitors. While the appeal of ...

  6. D-cycloserine for treatment of numbing and avoidance in chronic post traumatic stress disorder: A randomized, double blind, clinical trial

    Directory of Open Access Journals (Sweden)

    Abbas Attari

    2014-01-01

    Full Text Available Background: Posttraumatic stress disorder (PTSD tends to follow a chronic and treatment resistant course. Avoidance and numbing are symptoms associated with chronicity and impaired life quality. As D-cycloserine (DCS can facilitate extinction of conditioned fear, we aimed to investigate the efficacy and tolerability of DCS for the treatment of numbing and avoidance in chronic PTSD. Materials and Methods: This was an 11-week, double-blind, cross-over trial conducted in 2012 and 2013, in out-patient University psychiatry clinics. The studied population was selected randomly among outpatients with chronic combat-related PTSD (based on DSM-IV-TR criteria for chronic PTSD, who were males over 18 and <65 years of age (n = 319. Seventy six eligible patients were randomly assigned to two groups. Patients entered a 1-week run-in period. The groups received either an add-on treatment of DCS (50 mg daily, or placebo (4-week. After a 2-week washout, the groups received cross-over treatments (4-week. Clinical, paraclinical assessments, and clinician administered PTSD scale (CAPS were performed at baseline, and at the end of the 1 st , 5 th , and 11 th week. Side-effects were also evaluated. The overall number of avoidance and numbing symptoms, symptom frequency, and symptom intensity were measured separately. Results: Neither frequency nor number of symptoms was significantly influenced. However, DCS treatment demonstrates a significant decrease in intensity of avoidance/numbing symptoms, and improvement in function (mean [standard error] = −4.2 [1.5], P = 0.008. Side-effects were not statistically remarkable. Conclusion: D-cycloserine can help as an adjunctive treatment to alleviate numbing and avoidance in combat-related chronic PTSD.

  7. Cost-effectiveness of collaborative care for chronically ill patients with comorbid depressive disorder in the general hospital setting, a randomised controlled trial

    Directory of Open Access Journals (Sweden)

    Beekman Aartjan TF

    2007-02-01

    Full Text Available Abstract Background Depressive disorder is one of the most common disorders, and is highly prevalent in chronically ill patients. The presence of comorbid depression has a negative influence on quality of life, health care costs, self-care, morbidity, and mortality. Early diagnosis and well-organized treatment of depression has a positive influence on these aspects. Earlier research in the USA has reported good results with regard to the treatment of depression with a collaborative care approach and an antidepressant algorithm. In the UK 'Problem Solving Treatment' has proved to be feasible. However, in the general hospital setting this approach has not yet been evaluated. Methods/Design CC: DIM (Collaborative Care: Depression Initiative in the Medical setting is a two-armed randomised controlled trial with randomisation at patient level. The aim of the trial is to evaluate the treatment of depressive disorder in general hospitals in the Netherlands based on a collaborative care framework, including contracting, 'Problem Solving Treatment', antidepressant algorithm, and manual-guided self-help. 126 outpatients with diabetes mellitus, chronic obstructive pulmonary disease, or cardiovascular diseases will be randomised to either the intervention group or the control group. Patients will be included if they have been diagnosed with moderate to severe depression, based on the DSM-IV criteria in a two-step screening method. The intervention group will receive treatment based on the collaborative care approach; the control group will receive 'care as usual'. Baseline and follow-up measurements (after 3, 6, 9, and 12 months will be performed by means of questionnaires. The primary outcome measure is severity of depressive symptoms, as measured with the PHQ-9. The secondary outcome measure is the cost-effectiveness of these treatments according to the TiC-P, the EuroQol and the SF-36. Discussion Earlier research has indicated that depressive disorder is

  8. Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview

    Directory of Open Access Journals (Sweden)

    Renata Mendes de Freitas

    2015-10-01

    Full Text Available ABSTRACTMyeloproliferative neoplasms are caused by a clonal proliferation of a hematopoietic progenitor. First described in 1951 as 'Myeloproliferative Diseases' and reevaluated by the World Health Organization classification system in 2011, myeloproliferative neoplasms include polycythemia vera, essential thrombocythemia and primary myelofibrosis in a subgroup called breakpoint cluster region-Abelson fusion oncogene-negative neoplasms. According to World Health Organization regarding diagnosis criteria for myeloproliferative neoplasms, the presence of the JAK2 V617F mutation is considered the most important criterion in the diagnosis of breakpoint cluster region-Abelson fusion oncogene-negative neoplasms and is thus used as a clonal marker. The V617F mutation in the Janus kinase 2(JAK2 gene produces an altered protein that constitutively activates the Janus kinase/signal transducers and activators of transcription pathway and other pathways downstream as a result of signal transducers and activators of transcription which are subsequently phosphorylated. This affects the expression of genes involved in the regulation of apoptosis and regulatory proteins and modifies the proliferation rate of hematopoietic stem cells.

  9. Disease: H00012 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available ongs to the family of chronic myeloproliferative disorders (MPD), which includes hematological diseases that...types of hypereosinophilic syndrome (HES), systemic mast cell disease (SMD) and other rare disorders. Recent...nisms in myeloproliferative disorders. Cell Mol Life Sci 63:2939-53 (2006) PMID:16919893 (marker) Michiels J...J, Bernema Z, Van Bockstaele D, De Raeve H, Schroyens W. Current diagnostic criteria for the chronic myeloproliferative disorders...lippel S, Pahl HL. Molecular markers for the diagnosis of Philadelphia chromosome negative myeloproliferative disorders

  10. Chronic migraine.

    Science.gov (United States)

    Schwedt, Todd J

    2014-01-01

    Chronic migraine is a disabling neurologic condition that affects 2% of the general population. Patients with chronic migraine have headaches on at least 15 days a month, with at least eight days a month on which their headaches and associated symptoms meet diagnostic criteria for migraine. Chronic migraine places an enormous burden on patients owing to frequent headaches; hypersensitivity to visual, auditory, and olfactory stimuli; nausea; and vomiting. It also affects society through direct and indirect medical costs. Chronic migraine typically develops after a slow increase in headache frequency over months to years. Several factors are associated with an increased risk of transforming to chronic migraine. The diagnosis requires a carefully performed patient interview and neurologic examination, sometimes combined with additional diagnostic tests, to differentiate chronic migraine from secondary headache disorders and other primary chronic headaches of long duration. Treatment takes a multifaceted approach that may include risk factor modification, avoidance of migraine triggers, drug and non-drug based prophylaxis, and abortive migraine treatment, the frequency of which is limited to avoid drug overuse. This article provides an overview of current knowledge regarding chronic migraine, including epidemiology, risk factors for its development, pathophysiology, diagnosis, management, and guidelines. The future of chronic migraine treatment and research is also discussed. PMID:24662044

  11. Diagnosing Depression in Chronic Pain Patients: DSM-IV Major Depressive Disorder vs. Beck Depression Inventory (BDI)

    OpenAIRE

    Knaster, Peter; Estlander, Ann-Mari; Karlsson, Hasse; Kaprio, Jaakko; Kalso, Eija

    2016-01-01

    Background Diagnosing depression in chronic pain is challenging due to overlapping somatic symptoms. In questionnaires, such as the Beck Depression Inventory (BDI), responses may be influenced more by pain than by the severity of depression. In addition, previous studies have suggested that symptoms of negative self-image, a key element in depression, are uncommon in chronic pain-related depression. The object of this study is to assess the relationship of the somatic and cognitive-emotional ...

  12. Are smooth pursuit eye movements altered in chronic whiplash-associated disorders? A cross-sectional study

    DEFF Research Database (Denmark)

    Kongsted, Alice; Jørgensen, L V; Bendix, T;

    2007-01-01

    To evaluate whether smooth pursuit eye movements differed between patients with long-lasting whiplash-associated disorders and controls when using a purely computerized method for the eye movement analysis.......To evaluate whether smooth pursuit eye movements differed between patients with long-lasting whiplash-associated disorders and controls when using a purely computerized method for the eye movement analysis....

  13. Preparatory catheter-directed thrombolysis together with assisted endovascular angioplasty for the treatment of chronic occlusive arterial disorders of lower extremities

    International Nuclear Information System (INIS)

    Objective: To evaluate the safety and efficacy of preparatory catheter-directed thrombolysis together with assisted endovascular angioplasty in treating chronic occlusive arterial disorders of lower extremities. Methods: From January 2008 to December 2009, preparatory catheter-directed thrombolysis together with assisted endovascular angioplasty was performed in 12 patients with chronic occlusive arterial disorders of lower extremities, including 8 males and 4 females with an average age of 56.3 years (within a range of 38-71 years). All 12 patients had a history of chronic ischemia of lower limb,the mean ill duration was 19.3 months (3-48 months). All patients complained of intermittent claudication with a mean distance of 125 m (50-200 m). Rest pain occurred in 5 patients (42%), toe necrosis was seen in 3 patients (25%) and critically ischemic limb in 4 patients (33%). Ankle-brachial index (ABI) was 0.00 0.65 with a mean of 0.33. In all 12 patients catheter-directed thrombolysis with rt-PA or urokinase was initially carried out, which was followed by endovascular angioplasty (balloon dilatation or stent placement) in two days. The clinical data and the therapeutic results were analyzed. Results: Technical success was achieved in all 12 patients. The mean time of thrombolysis was 48 hours. Of 12 patients, rt-PA was employed in 4 and urokinase in 8. The occluded length of the diseased arteries before the treatment was 60-150 mm, with a mean of 80 mm. After catheter-directed thrombolysis,the occluded length decreased to 10-50 mm (mean of 30 mm). Endovascular angioplasty was successfully completed in all patients after thrombolysis therapy. Postoperative ABI was 0.64-1.0 (mean of 0.86), which was increased by 0.53 when compared to the preoperative figure. During the perioperative period neither complications needed to be surgically treated nor death occurred. All patients were followed up, and the arteries remained open after one year in all cases. Conclusion

  14. PORTAL-HYPERTENSION AS PRESENTING FEATURE OF A MYELOPROLIFERATIVE DISORDER - DIAGNOSIS AND THERAPEUTIC DILEMMAS

    NARCIS (Netherlands)

    MULLER, EW; DEWOLF, JTM; HAAGSMA, EB

    1993-01-01

    In some patients presenting with complications of portal hypertension, thrombosis of hepatic or portal veins is identified as the cause. Hepatic or portal vein thrombosis may be secondary to recognized etiologies like infection or malignancy. When no etiology for the thrombosis is found, it is likel

  15. Sustained expression of microRNA-155 in hematopoietic stem cells causes a myeloproliferative disorder.

    Science.gov (United States)

    O'Connell, Ryan M; Rao, Dinesh S; Chaudhuri, Aadel A; Boldin, Mark P; Taganov, Konstantin D; Nicoll, John; Paquette, Ronald L; Baltimore, David

    2008-03-17

    Mammalian microRNAs are emerging as key regulators of the development and function of the immune system. Here, we report a strong but transient induction of miR-155 in mouse bone marrow after injection of bacterial lipopolysaccharide (LPS) correlated with granulocyte/monocyte (GM) expansion. Demonstrating the sufficiency of miR-155 to drive GM expansion, enforced expression in mouse bone marrow cells caused GM proliferation in a manner reminiscent of LPS treatment. However, the miR-155-induced GM populations displayed pathological features characteristic of myeloid neoplasia. Of possible relevance to human disease, miR-155 was found to be overexpressed in the bone marrow of patients with certain subtypes of acute myeloid leukemia (AML). Furthermore, miR-155 repressed a subset of genes implicated in hematopoietic development and disease. These data implicate miR-155 as a contributor to physiological GM expansion during inflammation and to certain pathological features associated with AML, emphasizing the importance of proper miR-155 regulation in developing myeloid cells during times of inflammatory stress. PMID:18299402

  16. Sustained expression of microRNA-155 in hematopoietic stem cells causes a myeloproliferative disorder

    OpenAIRE

    O'Connell, Ryan M.; Rao, Dinesh S.; Chaudhuri, Aadel A.; Boldin, Mark P.; Taganov, Konstantin D.; Nicoll, John; Paquette, Ronald L.; Baltimore, David

    2008-01-01

    Mammalian microRNAs are emerging as key regulators of the development and function of the immune system. Here, we report a strong but transient induction of miR-155 in mouse bone marrow after injection of bacterial lipopolysaccharide (LPS) correlated with granulocyte/monocyte (GM) expansion. Demonstrating the sufficiency of miR-155 to drive GM expansion, enforced expression in mouse bone marrow cells caused GM proliferation in a manner reminiscent of LPS treatment. However, the miR-155–induce...

  17. Stem cell expression of the AML1/ETO fusion protein induces a myeloproliferative disorder in mice

    OpenAIRE

    Fenske, Timothy S; Pengue, Gina; Mathews, Vikram; Hanson, Piia T.; Hamm, Sarah E.; Riaz, Noor; Graubert, Timothy A

    2004-01-01

    The t(8;21)(q22;q22) translocation, present in 10-15% of acute myeloid leukemia (AML) cases, generates the AML1/ETO fusion protein. To study the role of AML1/ETO in the pathogenesis of AML, we used the Ly6A locus that encodes the well characterized hematopoietic stem cell marker, Sca1, to target expression of AML1/ETO to the hematopoietic stem cell compartment in mice. Whereas germ-line expression of AML1/ETO from the AML1 promoter results in embryonic lethality, heterozygous Sca1+/AML1-ETO i...

  18. Chronic pruritic skin disorders and sleep%睡眠不足在慢性瘙痒性皮肤病中的表现及处理

    Institute of Scientific and Technical Information of China (English)

    黄鸿达; 邓列华

    2012-01-01

    慢性瘙痒性皮肤病与睡眠不足有一定的联系.已有文献报导睡眠不足对患者的行为、生理及心理健康有不良的影响,降低了患者的生活质量.该文论述了慢性瘙痒性皮肤病引起睡眠不足的病因,对睡眠不足的常规治疗方案进行了概括,对如何治疗受睡眠不足困扰的皮肤病患者给予建议.正确的治疗不仅能改善皮肤病症状且能提高患者的整体生活质量.%Chronic pruritic skin disorder are variably associated with sleep deprivation. Sleep deprivation has been shown to have detrimental effects on behavioural, physiological and psychological health. This review focuses on the etiology of sleep disoder associated with pruritic skin conditions and also the commonly-seen chronic pruritic skin diseases such as atopic dermatitis, psoriasis, infestations and chronic idiopathic urticaria. Suggestions and advice on the management of sleep deprivation in patients with skin conditions are also included. Appropriate treatment will not only improve symptoms but also patient's quality of life.

  19. Chronic subordination stress induces hyperphagia and disrupts eating behavior in mice modeling binge-eating-like disorder

    OpenAIRE

    Maria eRazzoli; Valentina eSanghez; Alessandro eBartolomucci

    2015-01-01

    Background: Eating disorders are associated with physical morbidity and appear to have causal factors like stressful life events and negative affect. Binge eating disorder (BED) is characterized by eating in a discrete period of time a larger than normal amount of food, a sense of lack of control over eating, and marked distress. There are still unmet needs for the identification of mechanisms regulating excessive eating, which is in part due to the lack of appropriate animal models. We devel...

  20. Chronic Subordination Stress Induces Hyperphagia and Disrupts Eating Behavior in Mice Modeling Binge-Eating-Like Disorder

    OpenAIRE

    Razzoli, Maria; Sanghez, Valentina; Bartolomucci, Alessandro

    2015-01-01

    Background: Eating disorders are associated with physical morbidity and appear to have causal factors like stressful life events and negative affect. Binge-eating disorder (BED) is characterized by eating in a discrete period of time a larger than normal amount of food, a sense of lack of control over eating, and marked distress. There are still unmet needs for the identification of mechanisms regulating excessive eating, which is in part due to the lack of appropriate animal models. We devel...

  1. The effect of forearm posture on wrist flexion in computer workers with chronic upper extremity musculoskeletal disorders

    OpenAIRE

    Thompson R Terry; Burgess Ronald A; Rollman Gary B

    2008-01-01

    Abstract Background Occupational computer use has been associated with upper extremity musculoskeletal disorders (UEMSDs), but the etiology and pathophysiology of some of these disorders are poorly understood. Various theories attribute the symptoms to biomechanical and/or psychosocial stressors. The results of several clinical studies suggest that elevated antagonist muscle tension may be a biomechanical stress factor. Affected computer users often exhibit limited wrist range of motion, part...

  2. Chronic Inflammatory Demyelinating Polyneuropathy

    OpenAIRE

    Dimachkie, Mazen M.; Barohn, Richard J.

    2013-01-01

    Chronic Inflammatory polyneuropathies are an important group of neuromuscular disorders that present chronically and progress over more than 8 weeks, being referred to as chronic inflammatory demyelinating polyneuropathy (CIDP). Despite tremendous progress in elucidating disease pathogenesis, the exact triggering event remains unknown. Our knowledge regarding diagnosis and management of CIDP and its variants continues to expand, resulting in improved opportunities for identification and treat...

  3. Satisfaction With the Outcome of Physical Therapist-Prescribed Exercise in Chronic Whiplash-Associated Disorders: Secondary Analysis of a Randomized Clinical Trial.

    Science.gov (United States)

    Ardern, Clare L; Peterson, Gunnel; Ludvigsson, Maria Landén; Peolsson, Anneli

    2016-08-01

    Study Design Secondary analysis of a randomized clinical trial. Background Patient perception of the benefits gained from treatment is important, yet satisfaction with the outcome of treatment for chronic whiplash-associated disorders (WADs) has not been investigated. Objectives To investigate whether satisfaction with the outcome of treatment for chronic WAD changed over time, and whether there were group differences. Methods Two hundred sixteen people with chronic WAD (66% women; mean age, 40.4 years) participated in a 3-month program of physical therapist-led neck-specific exercises with or without a behavioral approach, or received a prescription of general physical activity. The main outcome was satisfaction with the outcome of treatment, assessed at baseline and 3, 6, and 12 months later. Additional outcomes were enablement and expectation fulfillment. Results Satisfaction improved over time in the 3 groups (odds ratio = 1.15; 95% confidence interval: 1.10, 1.20; P<.001). There was a significant group-by-time interaction (P<.001), with increased odds of being satisfied in the groups receiving neck-specific exercises compared to general physical activity. Enablement increased after completion of the intervention in all groups (P<.001). People who received neck-specific exercises reported greater enablement and expectation fulfillment than people prescribed general physical activity (P<.01). Conclusion Exercise interventions for chronic WAD led to increased satisfaction for 12 months following treatment that was unrelated to the type of exercise intervention received. Level of Evidence Therapy, level 1b. Registered January 22, 2012 at www.ClinicalTrials.gov (NCT01528579). J Orthop Sports Phys Ther 2016;46(8):640-649. Epub 3 Jul 2016. doi:10.2519/jospt.2016.6136. PMID:27374016

  4. Molecular cloning of human T-cell lymphotrophic virus type I-like proviral genome from the peripheral lymphocyte DNA of a patient with chronic neurologic disorders

    International Nuclear Information System (INIS)

    Human T-cell lymphotropic virus type 1 (HTLV-I), the etiologic agent of human T-cell leukemia, has recently been shown to be associated with neurologic disorders such as tropical spastic paraparesis, HTLV-associated myelopathy, and possibly with multiple sclerosis. In this communication, the authors have examined one specific case of neurologic disorder that can be classified as multiple sclerosis or tropical spastic paraparesis. The patient suffering from chronic neurologic disorder was found to contain antibodies to HTLV-I envelope and gag proteins in his serum and cerebrospinal fluid. Lymphocytes from peripheral blood and cerebrospinal fluid of the patient were shown to express viral RNA sequences by in situ hybridization. Southern blot analysis of the patient lymphocyte DNA revealed the presence of HTLV-I-related sequences. Blot-hybridization analysis of the RNA from fresh peripheral lymphocytes stimulated with interleukin 2 revealed the presence of abundant amounts of genomic viral RNA with little or no subgenomic RNA. They have clones the proviral genome from the DNA of the peripheral lymphocytes and determined its restriction map. This analysis shows that this proviral genome is very similar if not identical to that of the prototype HTLV-I genome

  5. Coverage and accuracy of myeloproliferative and myelodysplastic neoplasms in the Finnish Cancer Registry.

    Science.gov (United States)

    Leinonen, Maarit K; Rantanen, Matti; Pitkäniemi, Janne; Malila, Nea

    2016-06-01

    Background Registration of haematological malignancies presents specific challenges, and a wide range of data is required to ensure case ascertainment and proper classification of these diseases. We studied the data quality of myeloproliferative and myelodysplastic neoplasms in the Finnish Cancer Registry (FCR), comparing information with hospital discharges. Material and methods Hospital discharges (HILMO) in 2007-2013 including diagnostic codes of myeloproliferative and myelodysplastic neoplasms were extracted. Patients were individually linked to the FCR database for all haematological malignancies registered in 1953-2013. Coverage and accuracy of the FCR and agreement between registers was estimated. Results In total 5289 individuals were retrieved from two registers. Of these, 1406 were common, 1080 only found in the FCR and 2803 only in the HILMO. Coverage of myeloproliferative and myelodysplastic neoplasms in the FCR was 47.0% (95% CI 45.7-48.4%). Almost one quarter of the registrations in the FCR was based on a death certificate only. The accuracy of diagnosis was 51.4% (95% CI 49.4-53.3%), but it varied substantially by disease category. Kappa statistic for agreement between registers was excellent (0.83, 95% CI 0.80-0.85) for common cases. 7.6% of cases in the HILMO was registered as leukaemias in the FCR. Conclusions More than half of the patients found in the HILMO were entirely missing from the FCR. However, some of the diagnoses in HILMO may be preliminary and this represents the maximal number of missing cases. Cancer registers benefit from supplementary data sources, such as hospital discharges, to increase coverage and accuracy of register data on haematological malignancies. PMID:26767306

  6. Short Review of Our Work - “Chronic Metabolic Acidosis Destroys Pancreas” with Focus on the Functional Exocrine Pancreatic Disorders

    Directory of Open Access Journals (Sweden)

    linic of San Fran

    2015-07-01

    Full Text Available We deeply appreciate your publishing of our work - “Chronic metabolic acidosis destroys pancreas” in JOP (2014 [1]. We feel that our work can give the food for thought to many young researchers and health practitioners. A short review of our work may generate various questions and ideas for further investigations. In our work, we have focused on negative affects of the chronic metabolic acidosis on pancreatic function including: • Premature activation of the proteases within the pancreas • Diminishing the antimicrobial activity of the pancreatic juice • Suppressing of the flushing out zymogens from the pancreas • Precipitation of the aggressive bile acids • Calcification Authors believe that further research may provide more details of how the acidification destroys the pancreas and causes chronic pancreatitis. We would like to share some of our thoughts on this subject as follows: Descriptions of symptoms of chronic pancreatitis such as pain, malabsorption syndrome, steatorrhea, and weight loss are found in almost all medical books, textbooks, and articles. The medical literature refers to these conditions as “pancreatic insufficiency”. It is known that these symptoms occur when only 10 % of the exocrine pancreatic function is left intact. This is not an “insufficiency.” It is a pancreatic “failure” when the therapeutic opportunities are very limited.

  7. Chronic fatigue syndrome defies the mind-body-schism of medicine. New perspectives on a multiple realisable developmental systems disorder.

    Science.gov (United States)

    Ulvestad, Elling

    2008-09-01

    The article maintains that chronic fatigue syndrome can be properly understood only by taking an integrated perspective in which evolutionary, developmental and ecological aspects are considered. The integrative approach, supplemented by a complexity theory and psychoneuroimmunological research, is capable of explaining why there are so few structural aberrations to be found in chronic fatigue syndrome and why specific treatment is so difficult to establish. A major outcome of the investigation, that all individuals with chronic fatigue syndrome are diseased in their own way, emphasises the need to study the development of personalised life histories. It also highlights an ethical dimension; personalised disease defies essentialist thinking on patient management. Another major outcome, which follows from the developmental systems perspective, is the dissolution of ontological mind-body dualism. This in turn allows for a methodological complementation of the biological and phenomenological approaches to knowledge. New research strategies that may help to resolve chronic fatigue syndrome, grounded in the revised perspective on individual development, are suggested. PMID:18288588

  8. Pain Expectancies, Pain, and Functional Self-Efficacy Expectancies as Determinants of Disability in Patients with Chronic Low Back Disorders.

    Science.gov (United States)

    Lackner, Jeffrey M.; And Others

    1996-01-01

    Tested the predictive power of self-efficacy expectations of physical capabilities, expectations of pain, and expectations of reinjury on physical function in chronic back pain patients. Before assessment of function, patients rated their abilities to perform essential job tasks--functional self-efficacy (FSE)--and the likelihood working would…

  9. Evolving Therapeutic Strategies for the Classic Philadelphia-Negative Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Jason B. Kaplan

    2016-01-01

    Full Text Available Despite the emergence of JAK inhibitors, there is a need for disease-modifying treatments for Philadelphia-negative myeloproliferative neoplasms (MPNs. JAK inhibitors ameliorate symptoms and address splenomegaly, but because of the heterogeneous contributors to the disease process, JAK inhibitor monotherapy incompletely addresses the burden of disease. The ever-growing understanding of MPN pathogenesis has provided the rationale for testing novel and targeted therapeutic agents, as monotherapies or in combination, in preclinical and clinical settings. A number of intriguing options have emerged, and it is hoped that further progress will lead to significant changes in the natural history of MPNs.

  10. A pilot study on community-based outpatient treatment for patients with chronic psychotic disorders in Somalia: Change in symptoms, functioning and co-morbid khat use

    Directory of Open Access Journals (Sweden)

    Odenwald Michael

    2012-07-01

    Full Text Available Abstract Background In Low and Middle Income Countries, mental health services are often poorly developed due to the lack of resources and trained personnel. In order to overcome these challenges, new ways of care have been suggested such as a focus on community-based services. In Somalia, the consumption of the natural stimulant khat is highly prevalent, aggravating mental illness. At the same time, mental health care is largely unavailable to the vast majority of the population. In a pilot project, we tested possibilities for effective measures in community-based out-patient mental health care. Methods Thirty-five male patients with chronic psychotic disorders and their carers were involved in a 10-months follow-up study. All of them abused khat. Seventeen outpatients experiencing acute psychotic episodes were recruited from the community and received an intensive six week home-based treatment package. Additionally eighteen patients with chronic psychotic disorders in remission were recruited either following hospital discharge or from the community. In a second phase of the study, both groups received community-based relapse prevention that differed in the degree of the family’s responsibility for the treatment. The treatment package was comprised of psycho-education, low-dose neuroleptic treatment, monthly home visits and counseling. The Brief Psychiatric Rating Scale (BPRS was applied three times. Additionally, we assessed functioning, khat use and other outcomes. Results Of the 35 patients enrolled in the study, 33 participated in the 10-month follow-up. Outpatients improved significantly in the first six weeks of treatment and did not differ from remitted patients at the start of the second treatment phase. In the preventive treatment phase, we find heterogeneous outcomes that diverge between symptom and functioning domains. With the exception of depressive symptoms, symptoms in all patients tended to worsen. The outpatient group had

  11. Effectiveness and cost-effectiveness of a self-management training for patients with chronic and treatment resistant anxiety or depressive disorders: design of a multicenter randomized controlled trial

    OpenAIRE

    Zoun, Maringa H. H.; Koekkoek, Bauke; Sinnema, Henny; Muntingh, Anna D. T.; Anton J. L. M. van Balkom; Schene, Aart H; Smit, Filip; Spijker, Jan

    2016-01-01

    Background Many patients with anxiety or depressive disorders achieve no remission of their symptoms after evidence-based treatment algorithms. They develop a chronic course of the disorder. Current care for these patients usually consists of long-term supportive contacts with a community psychiatric nurse and pharmacological management by a psychiatrist. Data on the effectiveness of these treatments is lacking. A psychosocial rehabilitation approach, where self-management is an increasingly ...

  12. Sensitivity of the prefrontal GABAergic system to chronic stress in male and female mice: Relevance for sex differences in stress-related disorders.

    Science.gov (United States)

    Shepard, Ryan; Page, Chloe E; Coutellier, Laurence

    2016-09-22

    Stress-induced modifications of the prefrontal cortex (PFC) are believed to contribute to the onset of mood disorders, such as depression and anxiety, which are more prevalent in women. In depression, the PFC is hypoactive; however the origin of this hypoactivity remains unclear. Possibly, stress could impact the prefrontal GABAergic inhibitory system that, as a result, impairs the functioning of downstream limbic structures controlling emotions. Preclinical evidence indicates that the female PFC is more sensitive to the effects of stress. These findings suggest that exposure to stress could lead to sex-specific alterations in prefrontal GABAergic signaling, which contribute to sex-specific abnormal functioning of limbic regions. These limbic changes could promote the onset of depressive and anxiety behaviors in a sex-specific manner, providing a possible mechanism mediating sex differences in the clinical presentation of stress-related mood disorders. We addressed this hypothesis using a mouse model of stress-induced depressive-like behaviors: the unpredictable chronic mild stress (UCMS) paradigm. We observed changes in prefrontal GABAergic signaling after exposure to UCMS most predominantly in females. Increased parvalbumin (PV) expression and decreased prefrontal neuronal activity were correlated in females with severe emotionality deficit following UCMS, and with altered activity of the amygdala. In males, small changes in emotionality following UCMS were associated with minor changes in prefrontal PV expression, and with hypoactivity of the nucleus accumbens. Our data suggest that prefrontal hypoactivity observed in stress-related mood disorders could result from stress-induced increases in PV expression, particularly in females. This increased vulnerability of the female prefrontal PV system to stress could underlie sex differences in the prevalence and symptomatology of stress-related mood disorders. PMID:27365172

  13. An Overview of Multiple Sclerosis: Medical, Psychosocial, and Vocational Aspects of a Chronic and Unpredictable Neurological Disorder

    Science.gov (United States)

    Rumrill, Phillip D., Jr.; Roessler, Richard T.

    2015-01-01

    This article presents an overview of multiple sclerosis (MS), one of the most common neurological disorders in the western hemisphere. Medical and psychosocial aspects of the disease such as causes and risk factors, diagnosis, incidence and prevalence, symptoms, courses, and treatment are described. Existing research regarding the employment…

  14. Delayed Circadian Rhythm in Adults with Attention-Deficit/Hyperactivity Disorder and Chronic Sleep-Onset Insomnia

    NARCIS (Netherlands)

    Kooij, J. J. Sandra; Boonstra, A. Marije; Gordijn, Marijke C. M.; Van Someren, Eus J. W.

    2010-01-01

    Background: Previous studies suggest circadian rhythm disturbances in children with attention-deficit/hyperactivity disorder (ADHD) and sleep-onset insomnia (SOI). We investigate here sleep and rhythms in activity and melatonin in adults with ADHD. Methods: Sleep logs and actigraphy data were collec

  15. Jak2 Inhibitors are the solution for Myelo Proliferative Disorders

    OpenAIRE

    Siva jyothi Bugganaa; Safuara Ayesha mujeeb; Priyanka kuthadi; Swetha Padigela

    2014-01-01

    The recent discovery of JAK2 –activating mutations as a casual event in the majority of patients with Philadelphia chromosome negative (ph-)myeloproliferative disorders (MPDs) prompted many pharmaceutical companies to develop JAK2- selective inhibitors for the treatment of MPD’S . JAK2 inhibitors effectively reduce JAK2 driver phosphorylation of signal-transducer and activator of transcription 5,and cell proliferation and cell survival in JAK 2 activated cells in vitro and invivo. The results...

  16. Activation of the thrombopoietin receptor by mutant calreticulin in CALR-mutant myeloproliferative neoplasms.

    Science.gov (United States)

    Araki, Marito; Yang, Yinjie; Masubuchi, Nami; Hironaka, Yumi; Takei, Hiraku; Morishita, Soji; Mizukami, Yoshihisa; Kan, Shin; Shirane, Shuichi; Edahiro, Yoko; Sunami, Yoshitaka; Ohsaka, Akimichi; Komatsu, Norio

    2016-03-10

    Recurrent somatic mutations of calreticulin (CALR) have been identified in patients harboring myeloproliferative neoplasms; however, their role in tumorigenesis remains elusive. Here, we found that the expression of mutant but not wild-type CALR induces the thrombopoietin (TPO)-independent growth of UT-7/TPO cells. We demonstrated that c-MPL, the TPO receptor, is required for this cytokine-independent growth of UT-7/TPO cells. Mutant CALR preferentially associates with c-MPL that is bound to Janus kinase 2 (JAK2) over the wild-type protein. Furthermore, we demonstrated that the mutant-specific carboxyl terminus portion of CALR interferes with the P-domain of CALR to allow the N-domain to interact with c-MPL, providing an explanation for the gain-of-function property of mutant CALR. We showed that mutant CALR induces the phosphorylation of JAK2 and its downstream signaling molecules in UT-7/TPO cells and that this induction was blocked by JAK2 inhibitor treatment. Finally, we demonstrated that c-MPL is required for TPO-independent megakaryopoiesis in induced pluripotent stem cell-derived hematopoietic stem cells harboring the CALR mutation. These findings imply that mutant CALR activates the JAK2 downstream pathway via its association with c-MPL. Considering these results, we propose that mutant CALR promotes myeloproliferative neoplasm development by activating c-MPL and its downstream pathway. PMID:26817954

  17. Early diagnosis of interferon-induced myocardial disorder in patients with chronic hepatitis C. Evaluation by myocardial imaging with 123I-BMIPP

    International Nuclear Information System (INIS)

    Interferon (IFN) therapy for chronic hepatitis C is sometimes associated with cardiac complications. In the present study, we performed myocardial imaging with 123I-labeled β-methyl-p-iodophenylpentadecanoic acid (123I-BMIPP) in order to evaluate myocardial disorders caused by IFN. We studied 40 healthy subjects (H group) and 25 patients with chronic hepatitis C who had been treated with IFN (IFN group). A Holter electrocardiogram (ECG) was performed and the autonomic nervous function was assessed by analyzing the spectral variability and 1/f fluctuation of heart rate. Myocardial planner imaging with 123I-BMIPP was performed to obtain the time activity curve for 20 min immediately after administration of 123I-BMIPP (dynamic study). Early and delayed myocardial single photon emission computed tomography (SPECT) images were expressed as Bull's eyes and the myocardium was divided into four segments to calculate the washout rate for each segment on early and late SPECT images (early and late SPECT study). No significant differences in autonomic nervous function were observed between the two groups in heart rate variability. In a dynamic study, the reduction rate from the time activity curve was significantly higher in the IFN group compared with the H group (reduction rate, IFN group, 5.3±3.7% vs H group, 1.2±3.3%; P123I-BMIPP myocardial scintigraphy examination several months after IFN therapy. These results indicate that metabolic disorders of fatty acids caused by IFN therapy can be detected before abnormalities are observed by Holter-ECG or echocardiography. (author)

  18. Frequent deletions of JARID2 in leukemic transformation of chronic myeloid malignancies.

    Science.gov (United States)

    Puda, Ana; Milosevic, Jelena D; Berg, Tiina; Klampfl, Thorsten; Harutyunyan, Ashot S; Gisslinger, Bettina; Rumi, Elisa; Pietra, Daniela; Malcovati, Luca; Elena, Chiara; Doubek, Michael; Steurer, Michael; Tosic, Natasa; Pavlovic, Sonja; Guglielmelli, Paola; Pieri, Lisa; Vannucchi, Alessandro M; Gisslinger, Heinz; Cazzola, Mario; Kralovics, Robert

    2012-03-01

    Chronic myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS) have an inherent tendency to progress to acute myeloid leukemia (AML). Using high-resolution SNP microarrays, we studied a total of 517 MPN and MDS patients in different disease stages, including 77 AML cases with previous history of MPN (N = 46) or MDS (N = 31). Frequent chromosomal deletions of variable sizes were detected, allowing the mapping of putative tumor suppressor genes involved in the leukemic transformation process. We detected frequent deletions on the short arm of chromosome 6 (del6p). The common deleted region on 6p mapped to a 1.1-Mb region and contained only the JARID2 gene--member of the polycomb repressive complex 2 (PRC2). When we compared the frequency of del6p between chronic and leukemic phase, we observed a strong association of del6p with leukemic transformation (P = 0.0033). Subsequently, analysis of deletion profiles of other PRC2 members revealed frequent losses of genes such as EZH2, AEBP2, and SUZ12; however, the deletions targeting these genes were large. We also identified two patients with homozygous losses of JARID2 and AEBP2. We observed frequent codeletion of AEBP2 and ETV6, and similarly, SUZ12 and NF1. Using next generation exome sequencing of 40 patients, we identified only one somatic mutation in the PRC2 complex member SUZ12. As the frequency of point mutations in PRC2 members was found to be low, deletions were the main type of lesions targeting PRC2 complex members. Our study suggests an essential role of the PRC2 complex in the leukemic transformation of chronic myeloid disorders. PMID:22190018

  19. Neuroimaging and treatment evidence for clinical staging in psychotic disorders: from the at-risk mental state to chronic schizophrenia.

    Science.gov (United States)

    Wood, Stephen J; Yung, Alison R; McGorry, Patrick D; Pantelis, Christos

    2011-10-01

    A new approach to understanding severe mental disorders such as schizophrenia is to adopt a clinical staging model. Such a model defines the extent of the illness such that earlier and milder phenomena are distinguished from later, more impairing features. Specifically, a clinical staging model makes three key predictions. First, pathologic measures should be more abnormal in more severe stages. Second, patients who progress between the stages should show change in these same pathologic measures. Finally, treatment should be more effective in the earlier stages, as well as more benign. In this article, we review the evidence for these three predictions from studies of psychotic disorders, with a focus on neuroimaging data. For all three, the balance of evidence supports the predictions of the staging model. However, there are a number of alternative explanations for these findings, including the effects of medication and symptom heterogeneity. PMID:21762875

  20. Acute and chronic effects of cannabinoids on human brain: gene-environment interactions related to psychiatric disorders

    OpenAIRE

    Batalla Cases, Albert

    2014-01-01

    Tesi realitzada a l'Institut Clínic de Neurociències / Hospital Clínic 1) Introduction Cannabis use has been associated to mental health problems and worsened outcome of established psychiatric disorders. Disturbances of the endocannabinoid system may be responsible for long-lasting effects, such as neuropsychological deficits and morphological brain alterations. As not all the exposed individuals are equally affected, proneness to cannabis induced impairment may rely on key factors su...

  1. Differences in maladaptive schemas between patients suffering from chronic and acute posttraumatic stress disorder and healthy controls

    OpenAIRE

    Ahmadian A; Mirzaee J; Omidbeygi M; Holsboer-Trachsler E; Br; De, S.

    2015-01-01

    Alireza Ahmadian,1,2 Jafar Mirzaee,1 Maryam Omidbeygi,1 Edith Holsboer-Trachsler,3 Serge Brand3,41Department of Psychology, Kharazmi University, 2Sadr Psychiatric Hospital, Janbazan Medical and Engineering Research Center (JMERC), Tehran, Iran; 3Psychiatric Clinics of the University of Basel, Center for Affective, Stress and Sleep Disorders, 4Department of Sport, Exercise and Health, Sport Science Section, University of Basel, Basel, Switzerland Background: War, as a stressor event, has ...

  2. Differences in maladaptive schemas between patients suffering from chronic and acute posttraumatic stress disorder and healthy controls

    OpenAIRE

    Brand, Serge

    2015-01-01

    Alireza Ahmadian,1,2 Jafar Mirzaee,1 Maryam Omidbeygi,1 Edith Holsboer-Trachsler,3 Serge Brand3,41Department of Psychology, Kharazmi University, 2Sadr Psychiatric Hospital, Janbazan Medical and Engineering Research Center (JMERC), Tehran, Iran; 3Psychiatric Clinics of the University of Basel, Center for Affective, Stress and Sleep Disorders, 4Department of Sport, Exercise and Health, Sport Science Section, University of Basel, Basel, Switzerland Background: War, as a stressor event, ...

  3. Personality disorders do not affect treatment outcomes for chronic HCV infection in Spanish prisoners: the Perseo study

    OpenAIRE

    Marco, Andrés; Antón, José J.; Trujols, Joan; Saíz de la Hoya, Pablo; Juan, José; Faraco, Inmaculada; Caylà, Joan A; ,

    2015-01-01

    Background The link between infection with hepatitis C virus (HCV) and personality disorders (PD) has not been investigated in detail. The aim of this study was to compare the effectiveness of HCV treatment in prisoners with and without PD. Methods We performed a prospective multicentre study in inmates from 25 Spanish prisons who had been treated with pegylated interferon alfa-2a plus ribavirin in 2011. PD diagnosis was based on the Personality Diagnostic Questionnaire-4+. We calculated adju...

  4. Bipolar disorder.

    Science.gov (United States)

    Grande, Iria; Berk, Michael; Birmaher, Boris; Vieta, Eduard

    2016-04-01

    Bipolar disorder is a recurrent chronic disorder characterised by fluctuations in mood state and energy. It affects more than 1% of the world's population irrespective of nationality, ethnic origin, or socioeconomic status. Bipolar disorder is one of the main causes of disability among young people, leading to cognitive and functional impairment and raised mortality, particularly death by suicide. A high prevalence of psychiatric and medical comorbidities is typical in affected individuals. Accurate diagnosis of bipolar disorder is difficult in clinical practice because onset is most commonly a depressive episode and looks similar to unipolar depression. Moreover, there are currently no valid biomarkers for the disorder. Therefore, the role of clinical assessment remains key. Detection of hypomanic periods and longitudinal assessment are crucial to differentiate bipolar disorder from other conditions. Current knowledge of the evolving pharmacological and psychological strategies in bipolar disorder is of utmost importance. PMID:26388529

  5. Treatments for chronic myeloid leukemia: a qualitative systematic review

    Directory of Open Access Journals (Sweden)

    Ferdin

    2012-08-01

    Full Text Available Roxanne Ferdinand,1 Stephen A Mitchell,2 Sarah Batson,2 Indra Tumur11Pfizer, Tadworth, UK; 2Abacus International, Bicester, UKBackground: Chronic myeloid leukemia (CML is a myeloproliferative disorder of blood stem cells. The tyrosine kinase inhibitor (TKI imatinib was the first targeted therapy licensed for patients with chronic-phase CML, and its introduction was associated with substantial improvements in response and survival compared with previous therapies. Clinical trial data are now available for the second-generation TKIs (nilotinib, dasatinib, and bosutinib in the first-, second-, and third-line settings. A qualitative systematic review was conducted to qualitatively compare the clinical effectiveness, safety, and effect on quality of life of TKIs for the management of chronic-, accelerated-, or blast-phase CML patients.Methods: Included studies were identified through a search of electronic databases in September 2011, relevant conference proceedings and the grey literature.Results: In the first-line setting, the long-term efficacy (up to 8 years of imatinib has been confirmed in a single randomized controlled trial (International Randomized Study of Interferon [IRIS]. All second-generation TKIs reported lower rates of transformation, and comparable or superior complete cytogenetic response (CCyR, major molecular response (MMR, and complete molecular response rates compared with imatinib by 2-year follow-up. Each of the second-generation TKIs was associated with a distinct adverse-event profile. Bosutinib was the only second-generation TKI to report quality-of-life data (no significant difference compared with imatinib treatment. Data in the second- and third-line setting confirmed the efficacy of the second-generation TKIs in either imatinib-resistant or -intolerant patients, as measured by CCyR and MMR rates.Conclusion: Data from first-line randomized controlled trials reporting up to 2-year follow-up indicate superior response

  6. The Impact of Chronic Kidney Disease - Mineral and Bone Disorder on the Locomotor System and Quality of Life in Hemodialysis Patients

    Directory of Open Access Journals (Sweden)

    Mohamed FOUAD

    2015-12-01

    Full Text Available OBJECTIVE: Chronic kidney disease - mineral and bone disorder (CKD -BMD is a worldwide challenge in hemodialysis patients (HD. Widespread use and improved methods of HD may have changed the spectrum of locomotor system disorders in this population. Locomotor system disorders have an impact on health-related quality of life (QOL. The objective of this study was to assess the effect of CKD-BMD on the locomotor system (bone, joint, muscle, tendon and bursa and document the prevalence of locomotor system disorders in HD populations and its impact in QOL. MATERIAL and METHODS: 550 HD patients were enrolled in this study. Each HD patient received complete locomotor system examination and specific diagnostic investigation. iPTH level classified study population into three groups. Group 1. (149 patients iPTH level 300 pg/ mL, high turnover bone disease. Patients were offered a self-administered QOL questionnaire, which assessed various QOL variables. RESULTS: 75% of hemodialysis patients suffered from one or more locomotor system disorders and the commonest was bone pain 60%, followed by muscle cramps 36%, proximal muscle weakness 30%, osteoarthritis 25%, osteoporosis 16%, rotator cuff syndrome15%, gout pre-HD 12.5%, carpal tunnel syndrome 12%, bone fracture 7%, fibromyalgia 7%, tenosynovitis 6%, periarticular calcification 5%, Dupuytren’s contracture 2%, septic arthritis 0.9% and osteomyelitis 0.9%. The three studied groups were represented by 27%, 23% and 50% respectively. The prevalence of osteoarthritis, muscle cramps, bone pain, spontaneous bone fracture and osteoporosis were higher in the third group. 30% of our HD patients completed the QOL questionnaire without assistance and their mean functional status, psychological status, pain scale, fatigue scale, global assessment and joint count were 3.24±2.24, 3.13 ±1.67, 4.07 ±1.7, 4.95 ±1. 8, 3.97 ±1.55 and 9.65±9.95 respectively. QOL variables pronouncedly worsen in HD patients, however the

  7. Pay Attention to the Diagnosis and Treatment of Myeloproliferative Disease%重视骨髓增殖性疾病的诊治与研究

    Institute of Scientific and Technical Information of China (English)

    沈悌

    2009-01-01

    1951年美国著名的血液病学家Dameshek提出:真性红细胞增多症(polycythemia vera,PV)、慢性髓细胞白血病(chronic myelocytic leukemia,CML)、原发性血小板增多症(essential thrombaythemia,ET)和原发性骨髓纤维化(primary myelofibrosis,PMF)这4种慢性血液病可统称为“骨髓增殖性疾病(myeloproliferative disease,MPD)”,因为它们有类似的临床表现和病理基础。此后,随着细胞遗传学技术的进步,确定MPD是一组多能造血干细胞的克隆性疾病。MPD在欧美国家中并不少见,总的发生率为6~10/(10万人口·年。好发于50岁以上的中、老年人,男性多见。在中国尚无可靠的流行病学调查。

  8. Mutations with epigenetic effects in myeloproliferative neoplasms and recent progress in treatment: Proceedings from the 5th International Post-ASH Symposium

    Science.gov (United States)

    Tefferi, A; Abdel-Wahab, O; Cervantes, F; Crispino, J D; Finazzi, G; Girodon, F; Gisslinger, H; Gotlib, J; Kiladjian, J-J; Levine, R L; Licht, J D; Mullally, A; Odenike, O; Pardanani, A; Silver, R T; Solary, E; Mughal, T

    2011-01-01

    Immediately following the 2010 annual American Society of Hematology (ASH) meeting, the 5th International Post-ASH Symposium on Chronic Myelogenous Leukemia and BCR-ABL1-Negative Myeloproliferative Neoplasms (MPNs) took place on 7–8 December 2010 in Orlando, Florida, USA. During this meeting, the most recent advances in laboratory research and clinical practice, including those that were presented at the 2010 ASH meeting, were discussed among recognized authorities in the field. The current paper summarizes the proceedings of this meeting in BCR-ABL1-negative MPN. We provide a detailed overview of new mutations with putative epigenetic effects (TET oncogene family member 2 (TET2), additional sex comb-like 1 (ASXL1), isocitrate dehydrogenase (IDH) and enhancer of zeste homolog 2 (EZH2)) and an update on treatment with Janus kinase (JAK) inhibitors, pomalidomide, everolimus, interferon-α, midostaurin and cladribine. In addition, the new ‘Dynamic International Prognostic Scoring System (DIPSS)-plus' prognostic model for primary myelofibrosis (PMF) and the clinical relevance of distinguishing essential thrombocythemia from prefibrotic PMF are discussed. PMID:23471017

  9. Mutations with epigenetic effects in myeloproliferative neoplasms and recent progress in treatment: Proceedings from the 5th International Post-ASH Symposium

    International Nuclear Information System (INIS)

    Immediately following the 2010 annual American Society of Hematology (ASH) meeting, the 5th International Post-ASH Symposium on Chronic Myelogenous Leukemia and BCR-ABL1-Negative Myeloproliferative Neoplasms (MPNs) took place on 7–8 December 2010 in Orlando, Florida, USA. During this meeting, the most recent advances in laboratory research and clinical practice, including those that were presented at the 2010 ASH meeting, were discussed among recognized authorities in the field. The current paper summarizes the proceedings of this meeting in BCR-ABL1-negative MPN. We provide a detailed overview of new mutations with putative epigenetic effects (TET oncogene family member 2 (TET2), additional sex comb-like 1 (ASXL1), isocitrate dehydrogenase (IDH) and enhancer of zeste homolog 2 (EZH2)) and an update on treatment with Janus kinase (JAK) inhibitors, pomalidomide, everolimus, interferon-α, midostaurin and cladribine. In addition, the new ‘Dynamic International Prognostic Scoring System (DIPSS)-plus' prognostic model for primary myelofibrosis (PMF) and the clinical relevance of distinguishing essential thrombocythemia from prefibrotic PMF are discussed

  10. A Case of ChroniC Mania in a Patient with A Double Diagnosis of Bipolar I and Delusional Disorders

    OpenAIRE

    Marina Teles Martins; Maria João Gracias; João Carlos Melo

    2013-01-01

    The authors describe the case of a 62 year old woman without any significant personal or family psychiatric history prior to being 52, when after a minor head trauma occurring during worktime, she started showing delusional ideas of hypochondri- ac and somatic content believing to have developed a “problem in the head”. Two years later she was admitted to a Psychiatric inpatient unit and diagnosed with a delusional disorder of the somatic subtype. At discharge she maintained the delusional id...

  11. The JAK2V617F activating mutation occurs in chronic myelomonocytic leukemia and acute myeloid leukemia, but not in acute lymphoblastic leukemia or chronic lymphocytic leukemia

    OpenAIRE

    Levine, Ross L; Loriaux, Marc; Huntly, Brian J.P.; Loh, Mignon L.; Beran, Miroslav; Stoffregen, Eric; Berger, Roland; Clark, Jennifer J; Willis, Stephanie G; Kim T. Nguyen; Flores, Nikki J.; Estey, Elihu; Gattermann, Norbert; Armstrong, Scott; Look, A. Thomas

    2005-01-01

    Activating mutations in tyrosine kinases have been identified in hematopoietic and nonhematopoietic malignancies. Recently, we and others identified a single recurrent somatic activating mutation (JAK2V617F) in the Janus kinase 2 (JAK2) tyrosine kinase in the myeloproliferative disorders (MPDs) polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. We used direct sequence analysis to determine if the JAK2V617F mutation was present in acute myeloid leukemia (A...

  12. mTOR inhibitors alone and in combination with JAK2 inhibitors effectively inhibit cells of myeloproliferative neoplasms.

    Directory of Open Access Journals (Sweden)

    Costanza Bogani

    Full Text Available BACKGROUND: Dysregulated signaling of the JAK/STAT pathway is a common feature of chronic myeloproliferative neoplasms (MPN, usually associated with JAK2V617F mutation. Recent clinical trials with JAK2 inhibitors showed significant improvements in splenomegaly and constitutional symptoms in patients with myelofibrosis but meaningful molecular responses were not documented. Accordingly, there remains a need for exploring new treatment strategies of MPN. A potential additional target for treatment is represented by the PI3K/AKT/mammalian target of rapamycin (mTOR pathway that has been found constitutively activated in MPN cells; proof-of-evidence of efficacy of the mTOR inhibitor RAD001 has been obtained recently in a Phase I/II trial in patients with myelofibrosis. The aim of the study was to characterize the effects in vitro of mTOR inhibitors, used alone and in combination with JAK2 inhibitors, against MPN cells. FINDINGS: Mouse and human JAK2V617F mutated cell lines and primary hematopoietic progenitors from MPN patients were challenged with an allosteric (RAD001 and an ATP-competitive (PP242 mTOR inhibitor and two JAK2 inhibitors (AZD1480 and ruxolitinib. mTOR inhibitors effectively reduced proliferation and colony formation of cell lines through a slowed cell division mediated by changes in cell cycle transition to the S-phase. mTOR inhibitors also impaired the proliferation and prevented colony formation from MPN hematopoietic progenitors at doses significantly lower than healthy controls. JAK2 inhibitors produced similar antiproliferative effects in MPN cell lines and primary cells but were more potent inducers of apoptosis, as also supported by differential effects on cyclinD1, PIM1 and BcLxL expression levels. Co-treatment of mTOR inhibitor with JAK2 inhibitor resulted in synergistic activity against the proliferation of JAK2V617F mutated cell lines and significantly reduced erythropoietin-independent colony growth in patients with

  13. Occurrence of chronic lymphocytic leukemia in patients with chronic myelogenous leukemia

    Directory of Open Access Journals (Sweden)

    Pritish K Bhattacharyya

    2013-01-01

    Full Text Available Chronic lymphocytic leukemia (CLL is the most common leukemia of adults in the western world and constitutes about 33% of all leukemia′s. The incidence of CLL increases with age and are more common in older population. Chronic myeloid leukemia (CML on the contrary occurs in both young adults and elderly and is a chronic myeloproliferative disease that originates from abnormal pluripotent stem cells and results in involvement of multiple hematopoietic lineages, but predominantly myeloid and less commonly lymphoid. Association between CLL and myeloid malignancies (CML, acute myeloid leukemia and MDS, myelodysplastic syndrome is rare. In literature documenting CLL and CML in same patients, occur either simultaneously or CML is preceded by CLL.

  14. Ultra-sensitive molecular MRI of cerebrovascular cell activation enables early detection of chronic central nervous system disorders

    International Nuclear Information System (INIS)

    Since endothelial cells can be targeted by large contrast-carrying particles, molecular imaging of cerebrovascular cell activation is highly promising to evaluate the underlying inflammation of the central nervous system (CNS). In this study, we aimed to demonstrate that molecular magnetic resonance imaging (MRI) of cerebrovascular cell activation can reveal CNS disorders in the absence of visible lesions and symptoms. To this aim, we optimized contrast carrying particles targeting vascular cell adhesion molecule-1 and MRI protocols through both in vitro and in vivo experiments. Although, pre-contrast MRI images failed to reveal the ongoing pathology, contrast-enhanced MRI revealed hypoperfusion-triggered CNS injury in vascular dementia, unmasked amyloid-induced cerebrovascular activation in Alzheimer's disease and allowed monitoring of disease activity during experimental autoimmune encephalomyelitis. Moreover, contrast-enhanced MRI revealed the cerebrovascular cell activation associated with known risk factors of CNS disorders such as peripheral inflammation, ethanol consumption, hyperglycemia and aging. By providing a dramatically higher sensitivity than previously reported methods and molecular contrast agents, the technology described in the present study opens new avenues of investigation in the field of neuro-inflammation. (authors)

  15. Chronic Myeloid Leukemia (CML) Mouse Model in Translational Research.

    Science.gov (United States)

    Peng, Cong; Li, Shaoguang

    2016-01-01

    Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by increased proliferation of granulocytic cells without the loss of their capability to differentiate. CML is a clonal disease, originated at the level of Hematopoietic Stem Cells with the Philadelphia chromosome resulting from a reciprocal translocation between the chromosomes 9 and 22t(9;22)-(q34;q11). This translocation produces a fusion gene known as BCR-ABL which acquires uncontrolled tyrosine kinase activity, constantly turning on its downstream signaling molecules/pathways, and promoting proliferation of leukemia cell through anti-apoptosis and acquisition of additional mutations. To evaluate the role of each critical downstream signaling molecule of BCR-ABL and test therapeutic drugs in vivo, it is important to use physiological mouse disease models. Here, we describe a mouse model of CML induced by BCR-ABL retrovirus (MSCV-BCR-ABL-GFP; MIG-BCR-ABL) and how to use this model in translational research.Moreover, to expand the application of this retrovirus induced CML model in a lot of conditional knockout mouse strain, we modified this vector to a triple gene coexpression vector in which we can co-express BCR-ABL, GFP, and a third gene which will be tested in different systems. To apply this triple gene system in conditional gene knockout strains, we can validate the CML development in the knockout mice and trace the leukemia cell following the GFP marker. In this protocol, we also describe how we utilize this triple gene system to prove the function of Pten as a tumor suppressor in leukemogenesis. Overall, this triple gene system expands our research spectrum in current conditional gene knockout strains and benefits our CML translational research. PMID:27150093

  16. Conditional Expression of E2A-HLF Induces B-Cell Precursor Death and Myeloproliferative-Like Disease in Knock-In Mice.

    Directory of Open Access Journals (Sweden)

    Jesús Duque-Afonso

    Full Text Available Chromosomal translocations are driver mutations of human cancers, particularly leukemias. They define disease subtypes and are used as prognostic markers, for minimal residual disease monitoring and therapeutic targets. Due to their low incidence, several translocations and their biological consequences remain poorly characterized. To address this, we engineered mouse strains that conditionally express E2A-HLF, a fusion oncogene from the translocation t(17;19 associated with 1% of pediatric B-cell precursor ALL. Conditional oncogene activation and expression were directed to the B-cell compartment by the Cre driver promoters CD19 or Mb1 (Igα, CD79a, or to the hematopoietic stem cell compartment by the Mx1 promoter. E2A-HLF expression in B-cell progenitors induced hyposplenia and lymphopenia, whereas expression in hematopoietic stem/progenitor cells was embryonic lethal. Increased cell death was detected in E2A-HLF expressing cells, suggesting the need for cooperating genetic events that suppress cell death for B-cell oncogenic transformation. E2A-HLF/Mb1.Cre aged mice developed a fatal myeloproliferative-like disorder with low frequency characterized by leukocytosis, anemia, hepatosplenomegaly and organ-infiltration by mature myelocytes. In conclusion, we have developed conditional E2A-HLF knock-in mice, which provide an experimental platform to study cooperating genetic events and further elucidate translational biology in cross-species comparative studies.

  17. In vitro megakaryocyte differentiation and proplatelet formation in Ph-negative classical myeloproliferative neoplasms: distinct patterns in the different clinical phenotypes.

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    Alessandra Balduini

    Full Text Available BACKGROUND: Ph-negative myeloproliferative neoplasms (MPNs are clonal disorders that include primary myelofibrosis (PMF, polycythemia vera (PV and essential thrombocythemia (ET. Although the pathogenesis of MPNs is still incompletely understood, an involvement of the megakaryocyte lineage is a distinctive feature. METHODOLOGY/PRINCIPAL FINDINGS: We analyzed the in vitro megakaryocyte differentiation and proplatelet formation in 30 PMF, 8 ET, 8 PV patients, and 17 healthy controls (CTRL. Megakaryocytes were differentiated from peripheral blood CD34(+ or CD45(+ cells in the presence of thrombopoietin. Megakaryocyte output was higher in MPN patients than in CTRL with no correlation with the JAK2 V617F mutation. PMF-derived megakaryocytes displayed nuclei with a bulbous appearance, were smaller than ET- or PV-derived megakaryocytes and formed proplatelets that presented several structural alterations. In contrast, ET- and PV-derived megakaryocytes produced more proplatelets with a striking increase in bifurcations and tips compared to both control and PMF. Proplatelets formation was correlated with platelet counts in patient peripheral blood. Patients with pre-fibrotic PMF had a pattern of megakaryocyte proliferation and proplatelet formation that was similar to that of fibrotic PMF and different from that of ET. CONCLUSIONS/SIGNIFICANCE: In conclusion, MPNs are associated with high megakaryocyte proliferative potential. Profound differences in megakaryocyte morphology and proplatelet formation distinguish PMF, both fibrotic and prefibrotic, from ET and PV.

  18. The Clinical Significance of Single Features of Borderline Personality Disorder: Anger, Affective Instability, Impulsivity, and Chronic Emptiness in Psychiatric Outpatients.

    Science.gov (United States)

    Ellison, William D; Rosenstein, Lia; Chelminski, Iwona; Dalrymple, Kristy; Zimmerman, Mark

    2016-04-01

    Although dimensional models of borderline personality disorder (BPD) are consistent with findings showing that minimal levels of pathology are associated with substantial increases in psychosocial impairment, it is still unclear whether different individual BPD criteria are each clinically significant on their own. The current study uses semistructured interview data from 1,870 adults presenting for outpatient psychiatric treatment to investigate whether the BPD criteria of impulsivity, affective instability, emptiness, and anger are each related to psychosocial morbidity when met in the absence of the other eight criteria. Analyses showed that each of these criteria was associated with dysfunction in comparison with a control group meeting zero BPD criteria, but only the emptiness criterion was a marker of impairment on all indices of psychosocial morbidity: suicidality, history of suicide attempts and psychiatric hospitalizations, social and work dysfunction, Axis I comorbidity, and global functioning. Implications for the study of borderline pathology are discussed. PMID:25893552

  19. Circulating Cytokine Levels as Markers of Inflammation in Philadelphia Negative Myeloproliferative Neoplasms: Diagnostic and Prognostic Interest

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    Julie Mondet

    2015-01-01

    Full Text Available Cytokines are well known mediators of numerous physiological and pathological processes. They contribute to the regulation of normal hematopoiesis but increasing data suggest that they also have a clinical impact in some hematopoietic malignancies. In particular, there is evidence that cytokines are implicated in the functional symptoms of Philadelphia negative myeloproliferative neoplasms (Ph− MPNs, suggesting that evaluation of circulating levels of cytokines could be of clinical interest for the characterization of patients at the time of diagnosis and for disease prognosis. In this review, we present the current knowledge on alteration of circulating cytokine profiles in MPNs and their role in myelofibrosis pathogenesis. Phenotypic correlation, prognostic value of cytokines, and impact of JAK inhibitors are also discussed.

  20. Post-therapeutic acute malignant myeloproliferative syndrome and acute nonlymphocytic leukemia in non-Hodgkin's lymphoma

    International Nuclear Information System (INIS)

    In a prospective randomized study of treatment with radiation therapy (RT) or RT + chemotherapy (CT) for patients with non-Hodgkin's lymphoma Stages I-III, one patient developed an acute malignant myeloproliferative syndrome (AMMS) and four others acute nonlymphocytic leukemia (ANLL). There was correlation between the intensity of treatment and development of this complication: Among patients treated with local radiation with or without chemotherapy no cases of AMMS or ANLL were observed. However, patients treated with total lymphoid irradiation alone (TLI) had an observed to expected ratio of 162. Among patients treated with TLI plus CT this ratio increased to over 1000. The cytogenetic, clinical, and hematologic abnormalities of these patients are discussed

  1. Gender and Vascular Complications in the JAK2 V617F-Positive Myeloproliferative Neoplasms

    Directory of Open Access Journals (Sweden)

    Brady L. Stein

    2011-01-01

    Full Text Available We previously found that gender influenced the JAK2 V617F allele burden, but it is unknown whether this gender difference in molecular epidemiology influences complications in the myeloproliferative neoplasms (MPNs. Historically, vascular complications represented the most common cause of mortality in polycythemia vera and essential thrombocytosis and contributed to morbidity in primary myelofibrosis. To determine the influence of gender on vascular complications, we retrospectively analyzed associations between gender and vascular complications. Despite their younger age, less prevalent dyslipidemia or smoking history, lower white blood counts, and lower JAK2 V617F allele burden, women had higher rates of abdominal venous thrombosis and comparable rates of all vascular complications. Vascular risk is currently not easily stratified by MPN-disease burden or traditional risk factors. Our analysis contributes to growing literature emphasizing gender differences in the MPN and further supports the important impact of individual and host variation on MPN clinical manifestations, and especially vascular risk.

  2. Anemia of chronic disease

    Science.gov (United States)

    Anemia of inflammation; AOCD; ACD ... Anemia is a lower-than-normal number of red blood cells in the blood. Some conditions can lead to anemia of chronic disease include: Autoimmune disorders , such as ...

  3. Sleep and Chronic Disease

    Science.gov (United States)

    ... message, please visit this page: About CDC.gov . Sleep About Us About Sleep Key Sleep Disorders Sleep ... Sheets Data & Statistics Projects and Partners Resources Events Sleep and Chronic Disease Recommend on Facebook Tweet Share ...

  4. Chronic Fatigue Syndrome

    Science.gov (United States)

    Chronic fatigue syndrome (CFS) is a disorder that causes extreme fatigue. This fatigue is not the kind of tired feeling that ... activities. The main symptom of CFS is severe fatigue that lasts for 6 months or more. You ...

  5. Eating disorders

    OpenAIRE

    Kontić Olga; Vasiljević Nadja; Trišović Marija; Jorga Jagoda; Lakić Aneta; Jašović-Gašić Miroslava

    2012-01-01

    Eating disorders are considered chronic diseases of civilization. The most studied and well known are anorexia and bulimia nervosa. Anorexia is considered one of the most common psychiatric problems of girls in puberty and adolescence. Due to high mortality and morbidity as well as the increasing expansion of these diseases, it is clear why the amount of research on these diseases is growing worldwide. Eating disorders lead to numerous medical complications, mostly due to late diagnosis...

  6. Soluble forms of leukocyte differentiation antigens in chronic obstructive pulmonary disease and their relation with pulmonary ventilation disorders

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    Makarova Е.М.

    2015-12-01

    Full Text Available Objective: to study the characteristics of soluble forms of leukocyte membrane molecules and their relationship with disease progression in chronic obstructive pulmonary disease (COPD. Materials and Methods. The study involved 50 patients with COPD. All the patients underwent physical examination, spirometry; serum concentration of antigens sCD54, sCD50, sCD38, sCD25, sCD8, sCD95, sHLA I, sHLA-DR was assessed by ELISA, and the results were compared with serum samples of 30 healthy donors. Results. The level of all soluble forms of membrane molecules of leukocytes decreased in patients with severe COPD. We have found the increased content of the differentiation molecules in patients with moderate COPD, indicating the activation of immune cells during the immune response. Conclusion. There was a correlation between the soluble forms of membrane molecules and impaired pulmonary ventilation, which confirmed the role of immune cells in the pathogenesis of COPD.

  7. Comorbid anxiety and depression disorders in patients with chronic pain Transtornos ansiosos e depressivos em pacientes com dor crônica

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    Martha Castro

    2009-12-01

    Full Text Available OBJECTIVE: To evaluate the frequency of anxiety and depression disorders in patients with chronic pain. METHOD: Patients receiving care at the pain clinic of the Federal University of Bahia between February 2003 and November 2006. The MINI PLUS - Mini International Neuropsychiatric Interview was used to evaluate the patients and establish psychiatric diagnoses. RESULTS: 400 patients were evaluated mean age was 45.6±11.37 years; 82.8% were female, 17.3% male; 48.5% were married; 55.1% were Catholics; and 40.5% had only high school education. Of these 29.9% reported intense pain and 70.8% reported suffering pain daily. The most frequent medical diagnosis was herniated disc (24.5%, and 48.5% of patients had been undergoing treatment at the pain clinic for less than 3 months. Comorbidities found were depressive episodes (42%, dysthymia (54%, social phobia (36.5%, agoraphobia (8.5% and panic disorder (7.3%. CONCLUSION: Psychiatric comorbidities are prevalent in patients suffering chronic pain.OBJETIVO: Avaliar a freqüência de transtornos ansiosos e depressivos em pacientes com dor crônica. MÉTODO: Os pacientes foram avaliados na clínica da dor da Universidade Federal da Bahia entre fevereiro 2003 e novembro 2006. O Mini-plus - entrevista neuropsiquiátrica internacional foi usado para estabelecer diagnósticos psiquiátricos nos pacientes. RESULTADOS: Foram avaliados 400 pacientes com idade média de 45,6±11,37 anos; 82,8% eram mulheres, homens 17,3%; 48,5% eram casados; 55,1% católicos; e 40,5% concluíram o segundo grau, 29,9% relataram dor intensa e 70,8% relataram sentir dor diariamente. O diagnóstico médico o mais freqüente foi hérnia de disco (24,5%, e 48,5% dos pacientes submeteu-se ao tratamento na clínica da dor por menos de 3 meses. Comorbidades encontradas foram episódios depressivos (42%, distimia (54%, fobia social (36,5%, agorafobia (8,5% e transtorno de pânico (7,3%. CONCLUSÃO: Comorbidades psiquiátricas s

  8. Early trauma-focused cognitive-behavioural therapy to prevent chronic post-traumatic stress disorder and related symptoms: A systematic review and meta-analysis

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    Kirkehei Ingvild

    2008-09-01

    Full Text Available Abstract Background Early trauma-focused cognitive-behavioural therapy (TFCBT holds promise as a preventive intervention for people at risk of developing chronic post-traumatic stress disorder (PTSD. The aim of this review was to provide an updated evaluation of the effectiveness of early TFCBT on the prevention of PTSD in high risk populations. Methods We performed a systematic literature search in international electronic databases (MEDLINE, EMBASE, PsycINFO, CENTRAL, CINAHL, ISI and PILOTS and included randomised controlled trials comparing TFCBT delivered within 3 months of trauma, to alternative interventions. All included studies were critically appraised using a standardised checklist. Two independent reviewers selected studies for inclusion and assessed study quality. Data extraction was performed by one reviewer and controlled by another. Where appropriate, we entered study results into meta-analyses. Results Seven articles reporting the results of five RCTs were included. All compared TFCBT to supportive counselling (SC. The study population was patients with acute stress disorder (ASD in four trials, and with a PTSD diagnosis disregarding the duration criterion in the fifth trial. The overall relative risk (RR for a PTSD diagnosis was 0.56 (95% CI 0.42 to 0.76, 1.09 (95% CI 0.46 to 2.61 and 0.73 (95% CI 0.51 to 1.04 at 3–6 months, 9 months and 3–4 years post treatment, respectively. A subgroup analysis of the four ASD studies only resulted in RR = 0.36 (95% CI 0.17 to 0.78 for PTSD at 3–6 months. Anxiety and depression scores were generally lower in the TFCBT groups than in the SC groups. Conclusion There is evidence for the effectiveness of TFCBT compared to SC in preventing chronic PTSD in patients with an initial ASD diagnosis. As this evidence originates from one research team replications are necessary to assess generalisability. The evidence about the effectiveness of TFCBT in traumatised populations without an ASD

  9. Chronic pancreatitis

    Science.gov (United States)

    Chronic pancreatitis - chronic; Pancreatitis - chronic - discharge; Pancreatic insufficiency - chronic; Acute pancreatitis - chronic ... abuse over many years. Repeated episodes of acute pancreatitis can lead to chronic pancreatitis. Genetics may be ...

  10. The effect of forearm posture on wrist flexion in computer workers with chronic upper extremity musculoskeletal disorders

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    Thompson R Terry

    2008-04-01

    Full Text Available Abstract Background Occupational computer use has been associated with upper extremity musculoskeletal disorders (UEMSDs, but the etiology and pathophysiology of some of these disorders are poorly understood. Various theories attribute the symptoms to biomechanical and/or psychosocial stressors. The results of several clinical studies suggest that elevated antagonist muscle tension may be a biomechanical stress factor. Affected computer users often exhibit limited wrist range of motion, particularly wrist flexion, which has been attributed to increased extensor muscle tension, rather than to pain symptoms. Recreational or domestic activities requiring extremes of wrist flexion may produce injurious stress on the wrist joint and muscles, the symptoms of which are then exacerbated by computer use. As these activities may involve a variety of forearm postures, we examined whether changes in forearm posture have an effect on pain reports during wrist flexion, or whether pain would have a limiting effect on flexion angle. Methods We measured maximum active wrist flexion using a goniometer with the forearm supported in the prone, neutral, and supine postures. Data was obtained from 5 subjects with UEMSDs attributed to computer use and from 13 control subjects. Results The UEMSD group exhibited significantly restricted wrist flexion compared to the control group in both wrists at all forearm postures with the exception of the non-dominant wrist with the forearm prone. In both groups, maximum active wrist flexion decreased at the supine forearm posture compared to the prone posture. No UEMSD subjects reported an increase in pain symptoms during testing. Conclusion The UEMSD group exhibited reduced wrist flexion compared to controls that did not appear to be pain related. A supine forearm posture reduced wrist flexion in both groups, but the reduction was approximately 100% greater in the UEMSD group. The effect of a supine forearm posture on wrist

  11. Association of pain intensity, pain-related disability, and depression with hypothalamus-pituitary-adrenal axis function in female patients with chronic temporomandibular disorders.

    Science.gov (United States)

    Jo, Kyung B; Lee, Young J; Lee, Il G; Lee, Sang C; Park, Jai Y; Ahn, Ryun S

    2016-07-01

    Patients with temporomandibular disorders (TMD) commonly experience myofascial and joint pain, pain-related disability, and other pain conditions including depression. The present study was carried out to explore the function of the hypothalamus-pituitary-adrenal (HPA) axis in relation to variables of the Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD) Axis II and comorbid depression in female patients with TMD. Cortisol and dehydroepiandrosterone (DHEA) levels were determined in saliva samples that had been collected at various periods after waking (0, 30, and 60min) and at nighttime (2100-2200h) from 52 female patients with chronic TMD pain and age- and gender-matched controls (n=54, 20-40 years old). There were no significant differences in the levels and diurnal patterns of cortisol and DHEA secretion between groups of patients with TMD and controls. In patients, the cortisol awakening response (CAR) or diurnal cortisol rhythm were not associated with any variables of the RDC/TMD Axis II or the Beck Depression Inventory (BDI)-II total scores. However, the ratio of overall cortisol secretion within the first hour after waking (CARauc) to overall DHEA secretion during the post-waking period (Daucawk), defined as CARauc/Daucawk, was significantly associated with pain-related RDC/TMD variables (pain intensity and pain-related disability) and BDI-II total scores. Pain intensity and pain-related disability scores were also significantly associated with BDI-II total scores. These results indicated that an increase in molar cortisol/DHEA ratio due to the dissociation between cortisol and DHEA secretion was associated with pain intensity, pain-related disability, and depression in female patients with TMD. PMID:27082645

  12. The relationship between chronic whiplash-associated disorder and post-traumatic stress: attachment-anxiety may be a vulnerability factor

    Directory of Open Access Journals (Sweden)

    Tonny Elmose Andersen

    2011-01-01

    Full Text Available Background: In more than 90% of whiplash accidents a good explanation regarding the association between trauma mechanism, organic pathology, and persistent symptoms has failed to be provided. Objective: We predicted that the severity of chronic whiplash-associated disorder (WAD, measured as number of whiplash symptoms, pain duration, pain-related disability, and degree of somatisation would be associated with the number of post-traumatic stress disorder symptoms (PTSD. Secondly, we expected attachment-anxiety to be a vulnerability factor in relation to both PTSD and WAD. Design: Data were collected from 1,349 women and 360 men suffering from WAD from the Danish Society for Polio, Traffic, and Accident Victims. The PTSD symptoms were measured by the Harvard Trauma Questionnaire. All three core PTSD clusters were included: re-experiencing, avoidance, and hyperarousal. Attachment security was measured along the two dimensions, attachment-anxiety and attachment-avoidance, by the Revised Adult Attachment Scale. Results: PTSD symptoms were significantly related to the severity of WAD. In particular, the PTSD clusters of avoidance and hyperarousal were associated with the number of whiplash symptoms, disability, and somatisation. Attachment-anxiety was significantly related to PTSD symptoms and somatisation but not to pain and disability. A co-morbidity of 38.8% was found between the PTSD diagnosis and WAD, and about 20% of the sample could be characterised as securely attached. Conclusions: The PTSD clusters of avoidance and hyperarousal were significantly associated with severity of WAD. The study emphasises the importance of assessing PTSD symptomatology after whiplash injury. Furthermore, it highlights that attachment theory may facilitate the understanding of why some people are more prone to develop PTSD and WAD than others.For abstract or full text in other languages, please see Supplementary files under Reading Tools online

  13. Chronic diseases in adolescence

    Directory of Open Access Journals (Sweden)

    Rončević Nevenka

    2006-01-01

    Full Text Available Introduction. The prevalence of chronic diseases in adolescence is constantly increasing, especially in the last two decades. Adolescence is a period of important changes: body growth and development, sexual development, development of cognitive abilities, change in family relations and between peers, formation of personal identity and personal system of values, making decisions on future occupation etc. Chronic diseases in adolescence. Chronic disorders affect all development issues and represent an additional burden for adolescents. The interaction between chronic disorders and various development issues is complex and two-way: the disease may affect development, and development may affect the disease. Developmental, psychosocial and family factors are of great importance in the treatment of adolescents with chronic disorders. Chronic disorders affect all aspects of adolescent life, including relations with peers, school, nutrition, learning, traveling, entertainment, choice of occupation, plans for the future. Physicians should keep in mind that chronic diseases and their treatment represent only one aspect of person's life. Adolescents with chronic diseases have other needs as well, personal priorities, social roles and they expect these needs to be recognized and respected. Adolescent health care should be adjusted to the life style of adolescents.

  14. Pemetrexed Disodium in the Cerebrospinal Fluid of Patients With Leptomeningeal Metastases

    Science.gov (United States)

    2015-04-09

    Brain and Central Nervous System Tumors; Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Metastatic Cancer; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Precancerous Condition; Secondary Myelofibrosis; Unspecified Adult Solid Tumor, Protocol Specific

  15. Chronic diseases in adolescence

    OpenAIRE

    Rončević Nevenka; Stojadinović Aleksandra; Odri Irena

    2006-01-01

    Introduction. The prevalence of chronic diseases in adolescence is constantly increasing, especially in the last two decades. Adolescence is a period of important changes: body growth and development, sexual development, development of cognitive abilities, change in family relations and between peers, formation of personal identity and personal system of values, making decisions on future occupation etc. Chronic diseases in adolescence. Chronic disorders affect all development issues and repr...

  16. Prognostic significance of cellular vascular endothelial growth factor (VEGF expression in the course of chronic myeloid leukaemia

    Directory of Open Access Journals (Sweden)

    Vidović Ana

    2009-01-01

    Full Text Available Introduction. Increased angiogenesis in bone marrow is one of the characteristics of chronic myeloid leukaemia (CML, a clonal myeloproliferative disorder that expresses a chimeric bcr/abl protein. Vascular endothelial growth factor (VEGF is one of the most potent and a specific regulator of angiogenesis which principally targets endothelial cells and regulates several of their functions, including mitogenesis, permeability and migration. The impact of elevated VEGF expression on the course of chronic myeloid leukaemia is unknown. Objective. The aim of this study was the follow-up of VEGF expression during the course of CML. Methods. We studied VEGF expression of 85 CML patients (median age 50 years, range 16-75 years. At the commencement of the study, 29 patients were in chronic phase (CP, 25 in an accelerated phase (AP, and 31 in the blast crisis (BC. The temporal expression (percentage positivity per 1000 analysed cells VEGF proteins over the course of CML were studied using the immunohistochemical technique utilizing relevant monoclonal antibodies. It was correlated with the laboratory (Hb, WBC and platelet counts, and the percentage of blasts and clinical parameters (organomegaly, duration of CP, AP, and BC of disease progression. Results. The expression of VEGF protein was most pronounced in AP (ANOVA, p=0.033. The level of VEGF expression correlated inversely with the degree of splenomegaly (Pearson, r=-0.400, p=0.011. High expression of VEGF correlated with a shorter overall survival (log rank, p=0.042. Conclusion. Immunohistochemically confirmed significance of the expression of VEGF in dependence of the CML stage could be of clinical importance in deciding on the timing therapy. These data suggest that VEGF plays a role in the biology of CML and that VEGF inhibitors should be investigated in CML.

  17. Egg white hydrolysate promotes neuroprotection for neuropathic disorders induced by chronic exposure to low concentrations of mercury.

    Science.gov (United States)

    Rizzetti, Danize Aparecida; Fernandez, Francisca; Moreno, Silvia; Uranga Ocio, José Antonio; Peçanha, Franck Maciel; Vera, Gema; Vassallo, Dalton Valentim; Castro, Marta Miguel; Wiggers, Giulia Alessandra

    2016-09-01

    This study aims to investigate whether the egg white hydrolysate (EWH) acts on the neuropathic disorders associated with long-term Mercury (Hg) exposure in rats. 8- week-old male Wistar rats were treated for 60 days with: a) Control - saline solution (i.m.); b) Mercury - HgCl2 (1st dose 4.6μg/kg, subsequent doses 0.07μg/kg/day, i.m.); c) Hydrolysate - EWH (1g/kg/day, gavage); d) Mercury and Hydrolysate. Mechanical allodynia was assessed using Von Frey Hairs test; heat hyperalgesia by the plantar test; catalepsy by a modification of the "ring test" and spontaneous locomotor activity by a photocell activity chambers. Analyses were performed at 0, 30 and 60 days of treatment. Brain and plasma MDA, plasma NPSH and TNF-α determination and skin immunohistochemistry were performed at 60 days. Hg induced a reduction in mechanical sensitivity threshold at 30 and 60 days and in thermal sensitivity threshold at 60 days. At the end of treatment catalepsy was developed, but there was not significant alteration in spontaneous locomotor activity. Hg also increased brain and plasma MDA, plasma NPSH and TNF-α levels and the number of Merkel cell-neurite complex in the skin. EWH prevented the development of mechanical allodynia, thermal hyperalgesia and catalepsy induced by Hg and the increase in MDA concentration in brain and plasma and in the number of Merkel cell-neurite complex in the skin. In conclusion, EWH promotes neuroprotection against the toxic effects caused by Hg, demonstrating a beneficial therapeutic potential. PMID:27350078

  18. Chronic whiplash-associated disorder and traumatic cerebrospinal fluid leak. Analysis of cases with radioisotope cisternography, epidural blood patch, and cervical facet joint blocks

    International Nuclear Information System (INIS)

    This paper describes RI cisternographic (RIC) examinations of whiplash-associated disorder (WAD) and results of their treatment with nerve block and epidural blood patch (EBP) conducted in authors' facilities. Subjects were 40 chronic (av. symptomatic period of 3.1 y) WAD patients (av. age 34 y) with traffic (28 cases), sports (7) and falling (5) causes with complication of suspicious cerebrospinal fluid (CSF) leak. RIC was done 2.5-24 hr after injection of 37 MBq of 111In-diethylenetriamine pentaacetic acid (DTPA) in the medullary space through epidural puncturing needle. Positive finding of clear leak or early accumulation of RI in the bladder was seen in 21 cases and negative, in 19. Positive patients had significantly higher rates of headache, abnormal vision and fatigue than negative ones. EBP was conducted through X-ray to all positive patients and to negative ones with strongly suspicious leak complication (7 cases), which resulted in improvement of symptoms like headache and vision in the former, but no improvement in the latter cases. Repeated RIC of the patients with poor improvement in the former was suggested effective for judgment of repetition of EBP treatment. Cervical facet joint blocks were found effective in cases with posterior cervical pain. Symptoms in WAD accompanying headache should be differentially diagnosed whether it is derived from posttraumatic CSF leak or from pain due to cervical facet arthritis. (R.T.)

  19. Combination of PIM and JAK2 inhibitors synergistically suppresses cell proliferation and overcomes drug resistance of myeloproliferative neoplasms

    OpenAIRE

    Huang, Shih-Min A.; Wang, Anlai; Greco, Rita; LI, Mrs Zhifang; Sun, Fangxian; Barberis, Claude; Tabart, Michel; Patel, Vinod; Schio, Laurent; Hurley, Raelene; Chen, Bo; Cheng, Hong; Lengauer, Christoph; Pollard, Jack; Watters, James

    2014-01-01

    Inhibitors of JAK2 kinase are emerging as an important treatment modality for myeloproliferative neoplasms (MPN). However, similar to other kinase inhibitors, resistance to JAK2 inhibitors may eventually emerge through a variety of mechanisms. Effective drug combination is one way to enhance therapeutic efficacy and combat resistance against JAK2 inhibitors. To identify potential combination partners for JAK2 compounds in MPN cell lines, we performed pooled shRNA screen targeting 5,000 genes ...

  20. Kinase domain mutations confer resistance to novel inhibitors targeting JAK2V617F in myeloproliferative neoplasms

    OpenAIRE

    Deshpande, Anagha; Reddy, Mamatha M.; Schade, Georg O.M.; Ray, Arghya; Chowdary, Tirumala K.; Griffin, James D.; Sattler, Martin

    2011-01-01

    The transforming JAK2V617F kinase is frequently associated with myeloproliferative neoplasms (MPNs) and thought to be instrumental for the overproduction of myeloid lineage cells. Several small molecule drugs targeting JAK2 are currently in clinical development for treatment in these diseases. We performed a high-throughput in vitro screen to identify point mutations in JAK2V617F that would be predicted to have potential clinical relevance and associated with drug resistance to the JAK2 inhib...