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1

Current therapy of myelodysplastic syndromes?  

PubMed Central

After being a neglected and poorly-understood disorder for many years, there has been a recent explosion of data regarding the complex pathogenesis of myelodysplastic syndromes (MDS). On the therapeutic front, the approval of azacitidine, decitabine, and lenalidomide in the last decade was a major breakthrough. Nonetheless, the responses to these agents are limited and most patients progress within 2 years. Allogeneic stem cell transplantation remains the only potentially curative therapy, but it is associated with significant toxicity and limited efficacy. Lack or loss of response after standard therapies is associated with dismal outcomes. Many unanswered questions remain regarding the optimal use of current therapies including patient selection, response prediction, therapy sequencing and combinations, and management of resistance. It is hoped that the improved understanding of the underpinnings of the complex mechanisms of pathogenesis will be translated into novel therapeutic approaches and better prognostic/predictive tools that would facilitate accurate risk-adaptive therapy. PMID:23954262

Zeidan, Amer M.; Linhares, Yuliya; Gore, Steven D.

2014-01-01

2

Complementary and Alternative Therapies for Down Syndrome  

ERIC Educational Resources Information Center

In their role as committed advocates, parents of children with Down syndrome have always sought alternative therapies, mainly to enhance cognitive function but also to improve their appearance. Nutritional supplements have been the most frequent type of complementary and alternative therapy used. Cell therapy, plastic surgery, hormonal therapy,…

Roizen, Nancy J.

2005-01-01

3

Music therapy for children with Rett syndrome  

Microsoft Academic Search

The Rett syndrome is good reactivity for sound and music. We enforced active music therapy (MT) individual session to patients with Rett syndrome. The patients were 4, 5 and 6-year-old. The active music therapy and individual session of 30 min\\/week were performed. It was recorded in video and description, and using the original evaluation list, six items of fact. On

Akihiro Yasuhara; Yuriko Sugiyama

2001-01-01

4

Atypical Takotsubo syndrome during anagrelide therapy.  

PubMed

Anagrelide is a phosphodiesterase III inhibitor utilized in the treatment of essential thrombocythemia. Anagrelide can be responsible for positive inotropic and chonotropic activity of the cardiovascular system. Moreover, it can induce vasospam directly on the epicardial coronary arteries. In the literature, it is well reported that this inhibitor can determine serious cardiovascular side effects, including congestive heart failure, arrhythmia and acute coronary syndrome. We describe the case of a 75-year-old woman who developed a mid-ventricular Takotsubo syndrome while on anagrelide therapy. Takotsubo cardiomyopathy, also known as left ventricular ballooning syndrome, is characterized by a reversible ventricular contractile dysfunction with akinesis and expansion of apical segments and hyperkinesis of the basal segments. Recently, atypical cases with akinesia and dilation of mid-ventricular segment and hypercontraction of the apical segments, also called mid-ventricular and inverted Takotsubo syndrome, have been described. Even though the pathogenesis of Takotsubo syndrome is poorly understood, several mechanisms have been proposed, including catecholamine-induced myocardial stunning, and ischemia-mediated stunning due to multivessel epicardial or microvascular spasm. We think that in our case, the adverse response of anagrelide therapy was determined, by accumulated dosage of the drug, through an intensive inotropic stimulation and a sympathetic hyperactivation in a vulnerable myocardium. To our knowledge, this is one of the first reports of an association between anagrelide therapy and Takotsubo cardiomyopathy. PMID:19395976

Proietti, Riccardo; Rognoni, Andrea; Ardizzone, Fabio; Maccio, Sergio; Santagostino, Alberto; Rognoni, Giorgio

2009-07-01

5

Cognitive behaviour therapy in chronic fatigue syndrome  

Microsoft Academic Search

Fifty patients fulfilling operational criteria for the chronic fatigue syndrome (CFS), and who had been ill for a mean of five years, were offered cognitive behaviour therapy in an open trial. Those fulfilling operational criteria for depressive illness were also offered tricyclic antidepressants. The rationale was that a distinction be drawn between factors that precipitate the illness and those that

S Butler; T Chalder; M Ron; S Wessely

1991-01-01

6

Therapies of mucopolysaccharidosis IVA (Morquio A syndrome)  

PubMed Central

Introduction Morquio A syndrome (mucopolysaccharidosis type IVA, MPS IVA) is one of the lysosomal storage diseases and is caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Deficiency of this enzyme leads to accumulation of glycosaminoglycans (GAGs), keratan sulfate (KS) and chondroitin-6-sulfate (C6S). The majority of KS is produced by chondrocytes, and therefore, the undegraded substrates accumulate mainly in cells and extracelluar matrix (ECM) of cartilage. This has a direct impact on cartilage and bone development, leading to systemic skeletal dysplasia. In patients with Morquio A, cartilage cells are vacuolated, and this results in abnormal chondrogenesis and/or endochondral ossification. Areas covered This article describes the advanced therapies of Morquio A, focused on enzyme replacement therapy (ERT) and gene therapy to deliver the drug to avascular bone lesions. ERT and gene therapies for other types of MPS are also discussed, which provide therapeutic efficacy to bone lesions. Expert opinion ERT, gene therapy and hematopietic stem therapy are clinically and/or experimentally conducted. However, there is no effective curative therapy for bone lesion to date. One of the limitations for Morquio A therapy is that targeting avascular cartilage tissues remains an unmet challenge. ERT or gene therapy with bone-targeting system will improve the bone pathology and skeletal manifestations more efficiently. PMID:25419501

Tomatsu, Shunji; Alméciga-Díaz, Carlos J; Barbosa, Hector; Montańo, Adriana M; Barrera, Luis A; Shimada, Tsutomu; Yasuda, Eriko; Mackenzie, William G; Mason, Robert W; Suzuki, Yasuyuki; Orii, Kenji E; Orii, Tadao

2014-01-01

7

[Intensive therapy for patients with Guillian-Barré syndrome.  

PubMed

Guillain-Barré syndrome is the leading cause of acute flaccid paralysis in the industrialized world. Approximately 25% of the patients suffering from Guillain-Barré syndrome develop respiratory failure requiring mechanical ventilation and intensive therapy. We seek answers to when it is optimal to start respiratory supportive therapy and review various complications associated with Guillain-Barré syndrome. PMID:25316360

Buus, Lone; Třnnesen, Else K

2014-10-13

8

[Differentiated antiplatelet therapy for acute coronary syndromes].  

PubMed

Dual antiplatelet therapy is the cornerstone of maintenance medication following invasive treatment of patients with acute coronary syndromes (ST elevation myocardial infarction, non-ST elevation myocardial infarction, unstable angina). Over the last decade, P2Y12 inhibition in addition to low-dose acetylsalicylic acid has been intensively debated. The debate was enriched by the results of the large phase III clinical trials for prasugrel (TRITON) and ticagrelor (PLATO) compared to clopidogrel in patients with acute coronary syndromes. This article summarizes the critical details und subanalyses of both study programmes and highlights on clinical decision making when using the three P2Y12 blockers in acute coronary syndromes. A special focus is on higher risk patients such as those with ST elevation myocardial infarction and those with coexisting diabetes, but also on minimizing relevant bleedings, which are common during more intense platelet inhibition. PMID:24430955

Schäfer, A; Arntz, H R; Boudriot, E; Garlichs, C; Hoffmann, S; Ince, H; Klingenheben, T; Weil, J; Zugck, C; Helms, T M; Silber, S

2014-01-01

9

Severe dyshidrotic eczema after intravenous immunoglobulin therapy for Kawasaki syndrome.  

PubMed

Dyshidrotic eczema is one of the rare cutaneous adverse effects of intravenous immunoglobulin therapy, usually seen in adults. We herein report the first pediatric case of severe dyshidrotic eczema occurring after intravenous immunoglobulin therapy for Kawasaki syndrome. PMID:22304420

Shiraishi, Takahisa; Yamamoto, Toshiyuki

2013-01-01

10

Iron Chelation Therapy in Myelodysplastic Syndromes  

PubMed Central

Myelodysplastic syndromes (MDS) are a heterogeneous disorder of the hematopoietic stem cells, frequently characterized by anemia and transfusion dependency. In low-risk patients, transfusion dependency can be long lasting, leading to iron overload. Iron chelation therapy may be a therapeutic option for these patients, especially since the approval of oral iron chelators, which are easier to use and better accepted by the patients. The usefulness of iron chelation in MDS patients is still under debate, mainly because of the lack of solid prospective clinical trials that should take place in the future. This review aims to summarize what is currently known about the incidence and clinical consequences of iron overload in MDS patients and the state-of the-art of iron chelation therapy in this setting. We also give an overview of clinical guidelines for chelation in MDS published to date and some perspectives for the future. PMID:20672005

Messa, Emanuela; Cilloni, Daniela; Saglio, Giuseppe

2010-01-01

11

Extracorporeal Shock Wave Therapy for Treatment of Navicular Syndrome  

Microsoft Academic Search

Extracorporeal shock wave therapy was effective in decreasing the lameness associated with navic- ular syndrome in 81% of the horses as determined by an unmasked evaluator and in 56% of the horses with masked evaluators. Extracorporeal shock wave therapy provided a non-invasive, effective mechanism to decrease the lameness associated with navicular syndrome. There were no compli- cations associated with the

Scott McClure; Richard B. Evans; Kristina G. Miles; Eric L. Reinertson; Jan F. Hawkins; Clifford M. Honnas

12

Jellyfish envenoming syndromes: unknown toxic mechanisms and unproven therapies.  

PubMed

Interest in envenoming syndromes caused by Australian jellyfish has been intense since the deaths in early 2002 of two tourists in Queensland, attributed to the Irukandji syndrome. We review current knowledge of these envenoming syndromes, mechanisms of venom action and therapy, focusing on the deadly box jellyfish, Chironex fleckeri, and the array of jellyfish thought to cause the Irukandji syndrome. Current understanding of jellyfish venom activity is very limited, and many treatments are unproven and based on anecdote. PMID:12492389

Bailey, Paul M; Little, Mark; Jelinek, George A; Wilce, Jacqueline A

2003-01-01

13

Evolution des types cellulaires du lait de brebis (race Churra) en fonction des dnombrements cellulaires totaux  

E-print Network

Evolution des types cellulaires du lait de brebis (race Churra) en fonction des dénombrements) en fonction des dénombrements cellulaires totaux est étudiée dans le lait de 91 brebis de race Churra traite du matin, obtenu sur 91 brebis de race Churra : 42 appartenant à une exploitation avec traite

Paris-Sud XI, Université de

14

Asthma therapies and Churg-Strauss syndrome.  

PubMed

The pulmonary vasculitides are a group of rare but serious disorders that require early recognition, accurate diagnosis, and effective therapy. Churg-Strauss syndrome (CSS) is classified as small vessel vasculitis. Four different definitions for the diagnosis of CSS have been developed: (1) the pathologic criteria put forth by Churg and Strauss, (2) the criteria based on clinical grounds from Lanham and colleagues, (3) the criteria based on clinical grounds from the American College of Rheumatology, and (4) the criteria from the Chapel Hill Consensus Conference, which closely concur with the Churg and Strauss definition. It is apparent that cessation, diminution, or even a switch from low-dose systemic to inhaled corticosteroid therapy can precipitate the appearance of CSS. The term forme fruste has been used to indicate that the signs and symptoms of CSS were (inadvertently) suppressed by cortico-steroids. The clinical risk factors for CSS are moderately severe or severe asthma, chronic sinusitis, or reductions in systemic corticosteroid therapy. Differential diagnosis, treatment, and ongoing monitoring of CSS therapeutic responses are reviewed. The introduction of leukotriene modifiers and high-potency inhaled corticosteroids have allowed control of asthma symptoms, which results in avoidance or reduction in oral corticosteroid use. The advent of these agents has been associated with reports of CSS appearing in patients with asthma. The available data regarding the association of CSS and antiasthma agents are most consistent with the unmasking of a previously contained pathologic condition (forme-fruste CSS) or disease that progresses because systemic corticosteroids were avoided. Early recognition and immunosuppressive therapy are the keystones of successful treatment of this rare disorder. PMID:11799357

Lilly, Craig M; Churg, Andrew; Lazarovich, Mark; Pauwels, Romain; Hendeles, Leslie; Rosenwasser, Lanny J; Ledford, Dennis; Wechsler, Michael E

2002-01-01

15

Rifaximin Therapy of Irritable Bowel Syndrome  

PubMed Central

Irritable bowel syndrome (IBS) is a common gastrointestinal disorder characterized by abdominal pain and altered bowel habits in the absence of specific organic pathology. Although the underlying pathogenesis of IBS is not well-understood, small intestinal bacterial overgrowth (SIBO) or other abnormalities in the gut flora is believed to contribute to the development of a subset of IBS cases. Rifaximin is a poorly absorbed antimicrobial with activity against enteric pathogens. A number of studies have shown a significant improvement in IBS symptoms with antibiotic therapy including rifaximin. In this review, we discuss the pharmacokinetics, in vitro susceptibility profile, and efficacy and safety data from clinical trials of rifaximin treatment of IBS. PMID:24833932

Koo, Hoonmo L.; Sabounchi, Saman; Huang, David B.; DuPont, Herbert L.

2012-01-01

16

[Pain therapy in irritable bowel syndrome].  

PubMed

Irritable bowel syndrome (IBS) is one of the most common gastrointestinal diseases. It is characterized by chronic abdominal pain, typically associated with altered bowel habits that cannot be explained by structural abnormalities in routine diagnostic workup. Based on the predominant symptom, IBS can be divided into different subtypes: IBS with predominant constipation, diarrhea, bloating, or pain. Knowledge about the complex and multifactorial IBS pathophysiology has increased tremendously in recent years, e.g., IBS may be related to alterations in gastrointestinal motility, visceral sensitivity, and the mucosal immune system. It is important, both for the patient and the physician, that IBS diagnosis is made quickly and thoroughly based on the typical symptom complex and exclusion of relevant differential diagnoses and to reassure the patient that IBS is a chronic, but benign disease. These components are the fundamental basis for a good patient-physician relationship and for a successful long-term management of this potentially very compromising disorder. IBS therapy is based on general measures as well as symptom-oriented medical therapy, where improvement of abdominal pain is one of the main goals in treating IBS patients. Several pain treatment options are available, which may be used long-term or on demand and which may be combined with other therapies. General medical approaches include antispasmodics, improvement of bowel function, phytotherapy, and probiotics. Especially in patients with psychological comorbidities, antidepressants may be used. Modern drug treatments include the GC-C agonist linaclotide in IBS with predominant constipation, the locally acting antibiotic rifaximin in IBS with bloating, and 5-HT3 antagonists in IBS with predominant diarrhea. Psychotherapy should be included in an interdisciplinary approach in refractory cases or in psychological comorbidity. PMID:24903042

de Greck, M; Layer, P; Andresen, V

2014-06-01

17

Die logopädische Therapie bei Kindern mit Down-Syndrom.  

E-print Network

??In dieser Diplomarbeit werden terminologiewissenschaftliche Aspekte der logopädischen Therapie bei Kindern mit Down-Syndrom in der Sprachkombination Deutsch-Englisch systematisch untersucht. Dazu wird im Sachteil dieser deskriptiven… (more)

Schlesinger-Özelmacikli, Ute

2009-01-01

18

Art therapy applied to an adolescent with Asperger's syndrome  

Microsoft Academic Search

Asperger's syndrome is a neurodevelopmental disorder characterized by impairments in social interaction, restricted, repetitive and stereotyped patterns of behavior, interests, and activities. Adolescents with Asperger's syndrome have developed a compromised self-regulatory system, which leads to difficulty in many areas of functioning. Some of these areas include social, behavioral, emotional, and an increase in anxiety. Art therapy is an important activity

Deborah L. Elkis-Abuhoff

2008-01-01

19

Targeted therapy for hereditary cancer syndromes: hereditary breast and ovarian cancer syndrome, Lynch syndrome, familial adenomatous polyposis, and Li-Fraumeni syndrome.  

PubMed

Cancer genetics has rapidly evolved in the last two decades. Understanding and exploring the several genetic pathways in the cancer cell is the foundation of targeted therapy. Several genomic aberrations have been identified and their role in carcinogenesis is being explored. In contrast to most cancers where these mutations are acquired, patients with hereditary cancer syndromes have inherited genomic aberrations. The understanding of the molecular pathobiology in hereditary cancer syndromes has advanced dramatically. In addition, many molecularly targeted therapies have been developed that could have potential roles in the treatment of patients with hereditary cancer syndromes. In this review, we outline the presentation, molecular biology, and possible targeted therapies for two of the most widely recognized hereditary cancer syndromes -- hereditary breast and ovarian cancer syndrome and hereditary non-polyposis colorectal cancer syndrome (Lynch syndrome). We will also discuss other syndromes such as familial adenomatous polyposis and Li-Fraumeni syndrome (TP53). PMID:25549704

Agarwal, Rishi; Liebe, Sarah; Turski, Michelle L; Vidwans, Smruti J; Janku, Filip; Garrido-Laguna, Ignacio; Munoz, Javier; Schwab, Richard; Rodon, Jordi; Kurzrock, Razelle; Subbiah, Vivek

2014-12-01

20

Pharmacologic Therapies for Complex Regional Pain Syndrome  

PubMed Central

Complex regional pain syndrome (CRPS) remains a challenging condition to diagnose and treat. There are few large-scale, randomized trials of pharmacologic agents, and most published studies are small, uncontrolled, or presented only in abstract form at meetings. The most commonly used agents, such as anticonvulsants, anti-depressants, and opiates, have been found to be useful for other neuropathic pain conditions in large-scale trials but have not been adequately studied in CRPS. Systemic steroids delivered by multiple routes continue to be used, with some good evidence for short-term administration. N-methyl-D-aspartate antagonists have recently gained in popularity, without evidence from well-controlled trials. Bisphosphonates have been well studied and offer promise. In addition, there has been interest in thalidomide; however, we are still awaiting well-controlled trials. This article presents an overview of the available data regarding pharmacologic therapies for CRPS. These agents should be used in conjunction with a comprehensive interdisciplinary approach aimed at functional restoration and improved quality of life. PMID:17214920

Mackey, Sean; Feinberg, Steven

2010-01-01

21

Emerging pharmacologic therapies for irritable bowel syndrome.  

PubMed

New therapies are being developed for irritable bowel syndrome (IBS). These advances are based on understanding pathophysiology or the development of medications with greater selectivity in classes of agents with known efficacy. Prucalopride, the newest European Medicines Agency-approved 5-hydroxytryptamine receptor 4 (5-HT(4)) agonist, is effective in the treatment of chronic constipation with improved cardiovascular safety relative to older 5-HT(4) drugs; similarly, ramosetron, the 5-hydroxytryptamine receptor 3 (5-HT(3)) antagonist, appears efficacious in diarrhea-predominant IBS. Secretagogues with different mechanisms of action target apical domains in enterocytes that are involved in chloride secretion, such as chloride channels, the cystic fibrosis transmembrane regulator, and guanylate cyclase C. As a class, such secretagogues have high efficacy and safety for constipation. With more data obtained from phase 2 and 3 trials, we expect other classes of medications, including bile acid modulators, anti-inflammatory agents, visceral analgesics, and newer centrally acting agents to be efficacious and enter the armamentarium for the treatment of IBS in the future. PMID:20694841

Manabe, Noriaki; Rao, Archana S; Wong, Banny S; Camilleri, Michael

2010-10-01

22

Systemic therapy of Cushing’s syndrome  

PubMed Central

Cushing’s disease (CD) in a stricter sense derives from pathologic adrenocorticotropic hormone (ACTH) secretion usually triggered by micro- or macroadenoma of the pituitary gland. It is, thus, a form of secondary hypercortisolism. In contrast, Cushing’s syndrome (CS) describes the complexity of clinical consequences triggered by excessive cortisol blood levels over extended periods of time irrespective of their origin. CS is a rare disease according to the European orphan regulation affecting not more than 5/10,000 persons in Europe. CD most commonly affects adults aged 20–50 years with a marked female preponderance (1:5 ratio of male vs. female). Patient presentation and clinical symptoms substantially vary depending on duration and plasma levels of cortisol. In 80% of cases CS is ACTH-dependent and in 20% of cases it is ACTH-independent, respectively. Endogenous CS usually is a result of a pituitary tumor. Clinical manifestation of CS, apart from corticotropin-releasing hormone (CRH-), ACTH-, and cortisol-producing (malign and benign) tumors may also be by exogenous glucocorticoid intake. Diagnosis of hypercortisolism (irrespective of its origin) comprises the following: Complete blood count including serum electrolytes, blood sugar etc., urinary free cortisol (UFC) from 24 h-urine sampling and circadian profile of plasma cortisol, plasma ACTH, dehydroepiandrosterone, testosterone itself, and urine steroid profile, Low-Dose-Dexamethasone-Test, High-Dose-Dexamethasone-Test, after endocrine diagnostic tests: magnetic resonance imaging (MRI), ultra-sound, computer tomography (CT) and other localization diagnostics. First-line therapy is trans-sphenoidal surgery (TSS) of the pituitary adenoma (in case of ACTH-producing tumors). In patients not amenable for surgery radiotherapy remains an option. Pharmacological therapy applies when these two options are not amenable or refused. In cases when pharmacological therapy becomes necessary, Pasireotide should be used in first-line in CD. CS patients are at an overall 4-fold higher mortality rate than age- and gender-matched subjects in the general population. The following article describes the most prominent substances used for clinical management of CS and gives a systematic overview of safety profiles, pharmacokinetic (PK)-parameters, and regulatory framework. PMID:25091295

2014-01-01

23

Carpal tunnel syndrome symptoms are lessened following massage therapy  

Microsoft Academic Search

Objective. To determine the effectiveness of massage therapy for relieving the symptoms of carpal tunnel syndrome (CTS).Methods. Sixteen adults with CTS symptoms were randomized to a 4-week massage therapy or control group. Participants in the massage therapy group were taught a self-massage routine that was done daily at home. They were also massaged once a week by a therapist. The

Tiffany Field; Miguel Diego; Christy Cullen; Kristin Hartshorn; Alan Gruskin; Maria Hernandez-Reif; William Sunshine

2004-01-01

24

Automates cellulaires : deux mots `a la mode  

E-print Network

Cellulaires ´Etude de champ lexical... Google Conway's Game of Life Wolfram Self-reproduction Java applet Edge's Game of Life Wolfram Self-reproduction Java applet Edge of Chaos Cryptography Artificial life Reaction-Diffusion Membrane model (´echantillonnage sur les 10 premiers liens) DBLP "Urban Cellular Automata : an Evolutionary

Theyssier, Guillaume

25

ACMLautomates cellulaire en caml API tutorial  

E-print Network

ACMLautomates cellulaire en caml API tutorial how to define your favorite 'bistrouillet' in a few writting OCaml source code to define cellular automata using the ACML API. A reasonable knowledge of both title). It is a good idea to have a look at the API reference each time you discover a new function name

Theyssier, Guillaume

26

un lien entre la voie endolysosomale et la mort cellulaire  

E-print Network

Alix un lien entre la voie endolysosomale et la mort cellulaire Co-directeurs de thèse : Dr Chapitre 1. La mort cellulaire programmée : les différents acteurs et mécanismes moléculaires I. Caractérisation morphologiques des différents processus de mort cellulaire 1 II. De C.elegans aux mammifères 3 III

Paris-Sud XI, Université de

27

Combination Therapy for Patients with Myelodysplastic Syndromes  

Cancer.gov

In this trial, patients with myelodysplatic syndrome and symptomatic anemia who have a low or intermediate-1 risk of progression to leukemia will be treated with lenalidomide, and some will randomly assigned to receive epoetin alfa (Procrit).

28

The development of Moyamoya syndrome after proton beam therapy.  

PubMed

The development of Moyamoya syndrome (MMS) after cranial irradiation for pediatric tumors has been well established. However, information on the development of MMS after proton beam radiotherapy is sparse. We present the case of a 2-year-old child who developed radiation-induced MMS after treatment with proton beam therapy. PMID:24501091

Zwagerman, Nathan T; Foster, Kimberly; Jakacki, Regina; Khan, Fazal H; Yock, Torunn I; Greene, Stephanie

2014-08-01

29

Dance Therapy with Physical Therapy for Children with Down Syndrome.  

ERIC Educational Resources Information Center

This study sought to investigate effects of a dance program on bilateral toe-standing balance and single-point static balance skills of a group of children with Down Syndrome. Thirteen experimental and 10 control group students between the ages of 3 and 13 years were assessed on toe-standing balance and single-point standing balance on the right…

Dupont, Blanche Burt; Schulmann, Diana

30

Behçet syndrome: from pathogenesis to novel therapies.  

PubMed

Behçet syndrome is a chronic disease hallmarked by inflammation of the blood vessels that is related to an autoimmune reaction caused by inherited susceptibility due to specific genes and environmental factors, probably components of infectious microorganisms, which turn on or get going the disease in genetically susceptible subjects. The more common clinical expression of the disease is represented by a triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis, sometimes associated with inflammatory arthritis, phlebitis, iritis, as well as inflammation of the digestive tract, brain, and spinal cord. The treatment strategies used to manage the manifestations of Behçet syndrome have gradually progressed, and a number of new therapeutic resources have been implemented in recent years, allowing better control of pathogenic mechanisms, reducing symptoms and suffering, and ameliorating patient's outcome. PMID:25447032

Mazzoccoli, Gianluigi; Matarangolo, Angela; Rubino, Rosa; Inglese, Michele; De Cata, Angelo

2014-12-01

31

Complementary Therapy in Polycystic Ovary Syndrome  

PubMed Central

Polycystic Ovary Syndrome (PCOS) is an endocrine disease. PCOS afflicts 5 to 10 % of women of reproductive age. The symptoms are: amenorrhea, oligomenorrhea, hirsutism, obesity, infertility, chronic hyperandrogenic anovulation and acne. Other risk factors aggravate this condition: insulin resistance, obesity, hypertension, dyslipidemia, inflammation and subclinical cardiovascular disease. Anxiety, depression and reduced quality of life are also common. This review highlights the mechanisms and the beneficial effects of acupuncture, exercise and resveratrol on animal models and on humans affected by PCOS. PMID:24809037

Aquino, C. I.; Nori, S. L.

2014-01-01

32

[Combined therapy in pregnancy with primary antiphospholipid syndrome].  

PubMed

Patients with antiphospholipid syndrome have a high frequency of mid-pregnancy intrauterine growth retardation or fetal death. The authors report the case of a 28 year old pregnant woman with antiphospholipid syndrome with severe obstetrical complications (spontaneous abortion, missed abortion) in the past history. By means of the administration of combined immunomudulant (intravenous immunoglobulin, steroid), anticoagulant and antiplatelet therapy we could prevent a subsequent pregnancy loss of the patient. Apropos of this case the authors give an overview on the monitoring of the disease and the therapeutical recommendations of the literature. PMID:14725207

Szodoray, Péter; Bacskó, György; Lakos, Gabriella; Zeher, Margit

2003-12-01

33

Electroconvulsive therapy for lycanthropy and Cotard syndrome: a case report.  

PubMed

We present a case of psychotic depression presenting with lycanthropy (being converted to a pig) and Cotard syndrome simultaneously and treated with electroconvulsive therapy. A 37-year-old female patient developed psychotic depression after a stressor (a possibility of having a malignancy). As her depression worsened, she developed delusional belief of self being metamorphosed to a pig and her children also being metamorphosed into pig. In addition, she had the delusional belief that her own body and body of her children was rotting away. She was treated with electroconvulsive therapy along with venlafaxine and olanzapine, with which she improved completely. PMID:20562636

Grover, Sandeep; Shah, Ruchita; Ghosh, Abhishek

2010-12-01

34

Diagnostic test for prenatal identification of Down's syndrome and mental retardation and gene therapy therefor  

DOEpatents

A a diagnostic test useful for prenatal identification of Down syndrome and mental retardation. A method for gene therapy for correction and treatment of Down syndrome. DYRK gene involved in the ability to learn. A method for diagnosing Down's syndrome and mental retardation and an assay therefor. A pharmaceutical composition for treatment of Down's syndrome mental retardation.

Smith, Desmond J. (Oakland, CA); Rubin, Edward M. (Berkeley, CA)

2000-01-01

35

Acquired haemophilia syndrome: pathophysiology and therapy.  

PubMed

Acquired inhibitors against coagulation factor VIII (FVIII), also termed acquired haemophilia A, neutralize its procoagulant function and result in severe or often life-threatening bleeding. The antibodies arise in individuals with no prior history of clinical bleeding. Acquired haemophilia occurs rarely with the incidence of approximately 1 to 4 per million/year, with severe bleeds in up to 90% of affected patients, and high mortality between 8-22%. About 50% of diagnosed patients were previously healthy, while the remaining cases may be associated with postpartum period, autoimmune diseases, malignancy, infections, or medications. Most patients have spontaneous haemorrhages into the skin, muscles or soft tissues, and mucous membranes, or after trauma and surgery, whereas haemarthroses are uncommon. The diagnosis of acquired haemophilia A based on the prolongation of activated partial thromboplastin time which does not normalize after the addition of normal plasma, reduced FVIII, with evidence of FVIII inhibitor measured by the Bethesda assay (Nijmegen modification). The treatment of acute bleeding episodes and the long-term eradication of the autoantibodies in acquired haemophilia are the main therapeutic strategy. Two options are currently available for acute bleeding control: the use rFVIIa or FEIBA in patients with higher inhibitor titer (> 5 BU), or to raise the level of FVIII by administration of DDAVP or concentrates of FVIII in patients with low level of inhibitors (< 5 BU). Treatment with FEIBA (50-100 IU/ kg every 8-12 hours) has shown good haemostatic response in 76-89% of the bleeding episodes. Patients treated with rFVIIa (90 microg/kg every 2-6 hours) have achieved good response in 95-100% as a first-line, and 75-80% as a salvage therapy. Patients with low inhibitor titer and lower response can be treated with concentrate of FVIII in the recommended dose of 40 IU/kg plus 20 IU/kg for each BU of inhibitor. The treatment of non-life-threatening haemorrhages with desmopressin (DDAVP 0.3 microg/kg) may increase both FVIII and vWF. Sometimes inhibitors disappear spontaneously, but long-term management is necessary for eradication of inhibitors by immunosuppression (prednisone 1 mg/kg 3 weeks alone or in combination cyclophosphamide 2 mg/kg), immunomodulation, intravenous immunoglobulin (HD IgG 2 g/kg 2 or 5 d), physical removal of antibodies (plasmapheresis or immunoadsorption), or various combinations. Recently, a therapy with rituximab, an anti-CD20 monoclonal antibody, has shown to be effective in acquired haemophilia. PMID:20229686

Elezovi?, Ivo

2010-01-01

36

Low-Energy Extracorporeal Shock Wave Therapy as a Treatment for Greater Trochanteric Pain Syndrome  

Microsoft Academic Search

Background: Greater trochanteric pain syndrome is often a manifestation of underlying gluteal tendinopathy. Extracorporeal shock wave therapy is effective in numerous types of tendinopathies.Hypothesis: Shock wave therapy is an effective treatment for chronic greater trochanteric pain syndrome.Study Design: Case control study; Level of evidence, 3.Methods: Thirty-three patients with chronic greater trochanteric pain syndrome received low-energy shock wave therapy (2000 shocks;

John P. Furia; Jan D. Rompe; Nicola Maffulli

2009-01-01

37

Fibromyalgia Syndrome and Spa Therapy: Myth or Reality?  

PubMed Central

Fibromyalgia syndrome (FS) is a common musculoskeletal disorder characterized by otherwise unexplained chronic widespread pain, a lowered pain threshold, high tender point counts, sleep disturbances, fatigue, headache, irritable bowel syndrome, morning stiffness, paraesthesias in the extremities, often psychological distress and depressed mood. Consequently, FS has a negative impact on working capacity, family life, social functioning and quality of life. Because of unknown etiology and not clearly understood pathogenesis, there is no standard therapy regime for FS. A variety of medical treatments, including antidepressants, opioids, analgesic or non-steroidal anti-inflammatory drugs, sedatives, muscle relaxants and antiepileptics, have been used to treat FS. Currently, no pharmacological treatment for FS is consistently successful. According to recent guidelines, the optimal treatment of FS requires a multidisciplinary approach with a combination of non-pharmacological and pharmacological treatment modalities. Spa therapy is a popular treatment for FS in many European countries, as well as in Japan and Israel. However, despite their long history and popularity spa treatments are still the subject of debate and their role in modern medicine is still not clear. The objective of this review is to summarize the currently available information on clinical effects and mechanism of action of spa therapy in FS. We also provide some suggestions for further development in this area. PMID:22408369

Guidelli, Giacomo M.; Tenti, Sara; De Nobili, Emanuele; Fioravanti, Antonella

2012-01-01

38

Cervical Syndrome – the Effectiveness of Physical Therapy Interventions  

PubMed Central

ABSTRACT Introduction: The cervical syndrome refers to a set of disorders caused by the changes in the cervical spine and the soft-tissue surrounding it, with pain as the predominant symptom. Sore neck has been a common problem among a large section of today`s population. The factors contributing to this issue include the modern lifestyle, prolonged sitting and incorrect, fixed or constrained working postures. The root of these difficulties is found in the mechanical disorders of the cervical spine structures, poor body posture and jerky body movements. In the Scandinavian countries neck pain is considered to be a public health problem. Methods: The study evaluated 25 patients with an established diagnosis of cervical syndrome. The research was conducted at the PI Institute of Occupational and Sports Medicine of Zenica–Doboj Canton. Each patient received twenty physical therapy treatment sessions. Results and conclusions: The study included 25 patients suffering from the cervical syndrome. The statistical analysis of gender distribution indicated that 36% of the patients were male, while 64% were female. The mean age of study participants was 46.76±4,23. The patients ranged in age from 39 to 54 years, with no statistically significant difference in the mean age of male and female patients, p=0.691. Analysing the types of occupational activities performed by the patients, the study found a positive relation between neck pain and prolonged sitting at work. The patients who performed office work made up 76% of the total number. Each method of physical therapy applied in the treatment of neck pain patients proved useful. However, the combination of electrotherapy, kinesiotherapy and manual massage proved to be most effective. Conclusion: The cervical syndrome is a common medical condition primarily affecting adult population, with prevalence being higher among women and office workers. The condition places a considerable socioeconomic burden on the afflicted. Cervical pain ranges greatly in severity – from moderate to unbearable, thus leading to high levels of work absence as well as to a decrease in the quality of life. Proper physical therapy program can help the patients with neck pain return to their normal everyday activities, improve their quality of life, as well as reduce the absence from work.

Kasumovic, Mersija; Gorcevic, Emir; Gorcevic, Semir; Osmanovic, Jasna

2013-01-01

39

[Diagnosis and therapy of obstructive sleep apnea syndrome in children with premature craniosynostosis syndromes].  

PubMed

Sleep related breathing disorders are a common symptom in children with craniosynostosis syndromes, as upper airways may be narrowed by midfacial hypoplasia.To better characterize the sleep related apneas, 24 children with syndromal craniofacial dysplasia underwent 68 poly-somnographies. 9 patients had reexaminations after therapeutic procedures. 4 patients had severe obstructive sleep apnea syndrom (OSAS), 8 patients had moderate and 11 patients mild obstructive sleep apnea respectivly. Only one child had no obstructive sleep apnea. Children with Morbus Crouzon tended to have moderate to severe breathing disorders (9/14) whereas Apert patients mostly had no or light breathing disorders (6/7). Number of central apneas was increased as well. Sleep architecture was not significantly impaired. Apneas were more frequent during REM-sleep. Nasal CPAP, BiPAP and adenotonsillectomy improved respiratory parameters.Pulse oxymetry can be used as a screening method because of the good correla-tion of oxygen desaturation index with severity of OSAS. Frequent examinations and, if necessary, adaptation of therapy is indicated as OSAS in -these children may be rapidly changing. We suggest a guideline for diagnostics and therapy. PMID:22131155

Hein, A; Schweitzer, T; Strabburg, H-M; Wurm, M

2011-12-01

40

Low-Energy Extracorporeal Shock Wave Therapy as a Treatment for Medial Tibial Stress Syndrome  

Microsoft Academic Search

Background: Medial tibial stress syndrome (MTSS) is a pain syndrome along the tibial origin of the tibialis posterior or soleus muscle. Extracorporeal shock wave therapy (SWT) is effective in numerous types of insertional pain syndromes.Hypothesis: Shock wave therapy is an effective treatment for chronic MTSS.Study Design: Cohort study; Level of evidence, 3.Methods: Forty-seven consecutive subjects with chronic recalcitrant MTSS underwent

Jan D. Rompe; Angelo Cacchio; John P. Furia; Nicola Maffulli

2010-01-01

41

[Beneficial effect of continuous positive airway pressure therapy in obstructive sleep apnea syndrome].  

PubMed

Continuous positive airway pressure therapy is an evidence based therapy of obstructive sleep apnea syndrome. The effective treatment of obstructive sleep apnea can decrease sympaticotonia and, consequently, blood pressure. Furthermore, it can improve inflammatory and metabolic parameters resulting in a decreased cardiovascular risk. This article summarizes the positive effects of continuous positive airway pressure therapy on cardiovascular risk factors in obstructive sleep apnea syndrome. PMID:25403278

Magyar, Mária Tünde

2014-11-23

42

[Irritable Bowel Syndrome; gut microbiota and probiotic therapy].  

PubMed

Irritable bowel syndrome (IBS) is characterized by symptoms of abdominal pain and altered bowel habits. This common disorder is managed by varying clinical styles as no dominant therapeutic strategy has emerged. The pathophysiology of IBS remains unknown, but several lines of evidence link this disorder with the gut microbiota. Although controversy exists, gut microbiota is likely contributing to symptoms of IBS, at least in some patients, through an altered fermentation process, an impaired intestinal barrier function, a harmful modulation of enteric sensorimotor function, a promotion of low-grade inflammation without tissue damage, and a harmful modulation of the brain-gut axis. Probiotic therapy has a modest effect on IBS symptomatic relief, but the actual evidence is not strong enough to support a general recommendation of use. The best results are achieved, in children, with Lactobacillus rhamnusus GG, which moderately improves abdominal pain, while in adults the benefit appears to be greatest employing Bifidobacterium species. PMID:25659060

Tojo González, Rafael; Suarez Gonzalez, Adolfo; Rúas Madiedo, Patricia; Mancebo Mata, Alejo; Pipa Muńiz, María; Barreiro Alonso, Eva; Roman Llorente, Francisco Javier; Moro Villar, María Carmen; Arce González, Marta María; Villegas Diaz, María Francisca; Mosquera Sierra, Eugenia; Ruiz Ruiz, Mónica

2015-01-01

43

Metabolic syndrome – Removing roadblocks to therapy: Antigenic immunotherapies?  

PubMed Central

Up to 25 per cent of the world?s adult population may have the metabolic syndrome, a condition closely associated with central obesity. The metabolic syndrome is a major risk factor for cardiovascular disease and type 2 diabetes and therefore represents an important worldwide health problem. In addition to metabolic abnormalities such as raised fasting plasma glucose, high cholesterol and high blood pressure, there is consensus that obese subjects develop a state of low-grade chronic immune activation. This sustained pro-inflammatory response in fat tissue is thought to worsen insulin resistance and dyslipidemia. Likewise, the immune system contributes to the detrimental cascade of events leading to plaque formation in atherosclerosis. It has long been assumed that the innate arm of the immune system was the only key player, but emerging evidence suggests that there is in fact a sizeable adaptive immune component to obesity and cardiovascular disease. From a therapeutic perspective, it could be envisioned that immune modulation drugs such as cytokine inhibitors, co-stimulation blockers or anti-T cell agents could offer benefit. It is questionable, however, whether chronic treatment with for instance biologicals will have a favorable risk/benefit profile in a silent condition such as the metabolic syndrome. An attractive alternative could be the development of antigen-specific T cell therapies, not unlike those currently in various phases of development for type 1 diabetes. In this article, we will give an overview of antigen-specific treatment modalities in type 1 diabetes, followed by a review of the evidence for T cell involvement in obesity and atherosclerosis. PMID:24749057

Coppieters, Ken T.; von Herrath, Matthias G.

2014-01-01

44

Morquio A Syndrome: Diagnosis and Current and Future Therapies  

PubMed Central

Morquio A syndrome is an autosomal recessive disorder, one of 50 lysosomal storage diseases (LSDs), and is caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Deficiency of this enzyme causes specific glycosaminoglycan (GAG) accumulation: keratan sulfate (KS) and chondroitin-6-sulfate (C6S). The majority of KS is produced in the cartilage, therefore, the undegraded substrates accumulate mainly in cartilage and in its extracelluar matrix (ECM), causing direct leads to direct impact on cartilage and bone development and leading to the resultant systemic skeletal spondyloepiphyseal dysplasia. Chondrogenesis, the earliest phase of skeletal formation that leads to cartilage and bone formation is controlled by cellular interactions with the ECM, growth and differentiation factors and other molecules that affect signaling pathways and transcription factors in a temporal-spatial manner. In Morquio A patients, in early childhood or even at birth, the cartilage is disrupted presumably as a result of abnormal chondrogenesis and/or endochondral ossification. The unique clinical features are characterized by a marked short stature, odontoid hypoplasia, protrusion of the chest, kyphoscoliosis, platyspondyly, coxa valga, abnormal gait, and laxity of joints. In spite of many descriptions of the unique clinical manifestations, diagnosis delay still occurs. The pathogenesis of systemic skeletal dysplasia in Morquio A syndrome remains an enigmatic challenge. In this review article, screening, diagnosis, pathogenesis and current and future therapies of Morquio A are discussed. PMID:25345096

Tomatsu, Shunji; Yasuda, Eriko; Patel, Pravin; Ruhnke, Kristen; Shimada, Tsutomu; Mackenzie, William G.; Mason, Robert; Thacker, Mihir M.; Theroux, Mary; Montańo, Adriana M.; Alméciga-Díaz, Carlos J.; Barrera, Luis A.; Chinen, Yasutsugu; Sly, William S.; Rowan, Daniel; Suzuki, Yasuyuki; Orii, Tadao

2014-01-01

45

Morquio A syndrome: diagnosis and current and future therapies.  

PubMed

Morquio A syndrome is an autosomal recessive disorder, one of 50 lysosomal storage diseases (LSDs), and is caused by the deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Deficiency of this enzyme causes specific glycosaminoglycan (GAG) accumulation: keratan sulfate (KS) and chondroitin-6-sulfate (C6S). The majority of KS is produced in the cartilage, therefore, the undegraded substrates accumulate mainly in cartilage and in its extracelluar matrix (ECM), causing direct leads to direct impact on cartilage and bone development and leading to the resultant systemic skeletal spondyloepiphyseal dysplasia. Chondrogenesis ,the earliest phase of skeletal formation that leads to cartilage and bone formation is controlled by cellular interactions with the ECM, growth and differentiation factors and other molecules that affect signaling pathways and transcription factors in a temporal-spatial manner. In Morquio A patients, in early childhood or even at birth, the cartilage is disrupted presumably as a result of abnormal chondrogenesis and/ or endochondral ossification. The unique clinical features are characterized by a marked short stature, odontoid hypoplasia, protrusion of the chest, kyphoscoliosis, platyspondyly, coxa valga, abnormal gait, and laxity of joints. In spite of many descriptions of the unique clinical manifestations, diagnosis delay still occurs. The pathogenesis of systemic skeletal dysplasia in Morquio A syndrome remains an enigmatic challenge. In this review article, screening, diagnosis, pathogenesis and current and future therapies of Morquio A are discussed. PMID:25345096

Tomatsu, Shunji; Yasuda, Eriko; Patel, Pravin; Ruhnke, Kristen; Shimada, Tsutomu; Mackenzie, William G; Mason, Robert; Thacker, Mihir M; Theroux, Mary; Montańo, Adriana M; Alméciga-Díaz, Carlos J; Barrera, Luis A; Chinen, Yasutsugu; Sly, William S; Rowan, Daniel; Suzuki, Yasuyuki; Orii, Tado

2014-09-01

46

Metabolic Syndromes Associated with HIV: Mitigating the Side Effects of Drug Therapy.  

ERIC Educational Resources Information Center

HIV infection and highly active antiretroviral therapy (HAART) are associated with such metabolic disorders as AIDS wasting syndrome, metabolic dysregulation, and abnormalities of serum lipids. Adjunctive therapies (e.g., diet and antilipid therapy); risk factor modification (e.g., smoking cessation and blood pressure control); aerobic exercise;…

Stringer, William W.; Sattler, Fred R.

2001-01-01

47

Differentiation Therapy With Decitabine in Treating Patients With Myelodysplastic Syndrome  

ClinicalTrials.gov

Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Myelodysplastic Syndromes; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Ringed Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Thrombocytopenia

2013-02-25

48

Strategic Role of Nuclear Inositide Signalling in Myelodysplastic Syndromes Therapy.  

PubMed

Nuclear inositide signalling is implicated in normal and pathological cell proliferation and differentiation in several distinct models. Among the key molecules of nuclear inositide pathways, phosphoinositide-phospholipase (PI-PLC) C ?1 is essential for regulating hematopoiesis, particularly along myeloid and erythroid lineage. Moreover, Akt activation is associated with protein synthesis, via mTOR pathway, and with erythroid induction, through PI-PLC?1 activation. Myelodysplastic syndromes (MDS) are a series of heterogeneous diseases characterized by ineffective hemopoiesis, with a variable risk of evolution into acute myeloid leukemia (AML). Therapeutic approaches for MDS include demethylating agents, such as azacitidine, aiming at reducing cell proliferation, and erythropoietin, useful for sustaining a normal erythropoiesis. In the last few years, a role for nuclear inositide signalling as a therapeutic target in MDS has been disclosed, in that PI-PLC?1 increase is associated with azacitidine responsiveness, even when this drug is used in combination with other agents, and Akt is specifically activated in MDS at higher risk of AML evolution. On the other hand, recent data demonstrated that inositide signalling can also be involved in erythroid therapy, given the inhibitory effect of erythropoietin on PI-PLC?1 and the activation of Akt/PI-PLC?1 pathway, following the administration of erythropoietin. Here, we review the strategic role of nuclear inositide signalling in MDS, in pathogenesis and therapy. PMID:25307310

Manzoli, Lucia; Mongiorgi, Sara; Clissa, Cristina; Finelli, Carlo; Billi, Anna Maria; Poli, Alessandro; Quaranta, Marilisa; Cocco, Lucio; Follo, Matilde Y

2014-10-13

49

New Receptor Targets for Medical Therapy in Irritable Bowel Syndrome  

PubMed Central

Background Despite setbacks to the approval of new medications for the treatment of irritable bowel syndrome, interim guidelines on endpoints for IBS trials have enhanced interest as new targets for medical therapy are proposed based on novel mechanisms or chemical entities. Aim To review the approved lubiprostone, two targets that are not meeting expectations (tachykinins and corticotrophin-releasing hormone), the efficacy and safety of new 5-HT4 agonists, intestinal secretagogues (chloride channel activators, and guanylate cyclase-C agonists), bile acid modulation, anti-inflammatory agents and visceral analgesics. Methods Review of selected articles based on PubMed search and clinically relevant information on mechanism of action, safety, pharmacodynamics, and efficacy Conclusions The spectrum of peripheral targets of medical therapy address chiefly the bowel dysfunction of IBS, and these effects are associated with pain relief. There are less clear targets related to the abdominal pain or visceral sensation in IBS. The new 5-HT4 agonists are more specific than older agents, and show cardiovascular safety to date. Secretory agents have high specificity, low bioavailability, and efficacy. The potential risks of agents “borrowed” from other indications (like hyperlipidemia, inflammatory bowel disease or somatic pain) deserve further study. There is reason for optimism in medical treatment of IBS. PMID:19785622

Camilleri, Michael

2010-01-01

50

Children with Down syndrome improved in motor functioning and muscle tone following massage therapy  

Microsoft Academic Search

Twenty?one moderate to high functioning young children (mean age, two years) with Down syndrome receiving early intervention (physical therapy, occupational therapy and speech therapy) were randomly assigned to additionally receive two 0.5?hour massage therapy or reading sessions (control group) per week for two months. On the first and last day of the study, the children’s functioning levels were assessed using

Tiffany Field; Shay Largie; Dana Mora; Joan Bornstein; Ronnie Waldman

2006-01-01

51

Physical Therapy and Infants with Down's Syndrome: The Effects of Early Intervention.  

ERIC Educational Resources Information Center

The neuromotor development of Down's syndrome (DS) infants is reviewed, current physical therapy approaches are cited, a neurodevelopmental treatment (NDT) approach is described, and a study on the effects of NDT on motor performance in DS infants is reported. (SB)

Harris, Susan R.

1981-01-01

52

[Syndrome of inappropriate secretion of ADH following chemoradiation therapy].  

PubMed

We report a 69-year-old female patient with pulmonary adenocarcinoma complicated by the syndrome of inappropriate secretion of antidiuretic hormone(SIADH)following systemic chemotherapy with cisplatin(CDDP)and vinorelbine(VNR). She was admitted to our hospital for chemo-radiotherapy for advanced lung cancer, and became restless 4 hours after the administration of CDDP and VNR. Symptoms such as restlessness and incontinence were worsening despite the massive infusion that was completed. Laboratory examinations on day 6 after chemotherapy showed severe hyponatremia(107mEq/L)with decreased serum osmolarity(227mOsm/L)and increased urine osmolarity(452mOsm/L). The serum anti-diuretic hormone(ADH)level was elevated to 16. 7 pg/mL despite severe hyponatremia. She was diagnosed with SIADH and was treated with hypertonic saline infusion and fluid restriction. Her restlessness and other psychiatric symptoms were improved. The use of carboplatin and VNR in the subsequent course did not develop SIADH, indicating that the SIADH was induced by CDDP. Although SIADH following CDDP administration is rare, the electrolyte balance should be carefully monitored throughout the clinical course of chemo-radiation therapy, when psychiatric symptoms are found in patients with lung cancer. PMID:23152025

Kikuchi, Norihiro; Masuda, Michiko; Tamura, Tomohiro; Nakazawa, Kensuke; Kanemoto, Koji; Iijima, Hiroaki; Ishikawa, Hirokazu; Sato, Shinya; Ishii, Yukio

2012-11-01

53

Short bowel syndrome in children: current and potential therapies.  

PubMed

Short bowel syndrome (SBS) reflects a state of malabsorption that occurs due to loss of a significant portion of the small bowel. The pathophysiology of SBS is determined largely by the process of adaptation, which is the innate attempt by the remnant portions of the intestine to increase fluid and nutrient reabsorption. In recent years, emphasis has been placed on intestinal rehabilitation with multidisciplinary teams as a comprehensive approach to the management of patients with SBS. In our institution, the multidisciplinary team members include pediatric gastroenterologists, pediatric surgeons, pediatric dieticians, physical therapists, occupational therapists, neonatologists (especially for patients still under their care), transplant surgeons, transplant coordinators and social workers. Parenteral nutrition plays a significant role in the management of SBS, but its use is associated with many potential complications, including cholestatic liver disease. Fish oil-based lipid emulsions have shown promise in their ability to reverse and also prevent the development of cholestasis in these patients. Clinical trials have shown that growth factors and other trophic hormones facilitate the process of adaptation. The most significant impact has been shown with the use of glucagon-like peptide-2 and its analog (teduglutide). Surgical interventions remain an important part of the management of SBS to facilitate adaptation and treat complications. Intestinal transplantation is a last resort option when the process of adaptation is unsuccessful. This review article is intended to provide an overview of the conventional and emerging therapies for pediatric SBS. PMID:22452596

Uko, Victor; Radhakrishnan, Kadakkal; Alkhouri, Naim

2012-06-01

54

HYPOGONADISM AND METABOLIC SYNDROME: IMPLICATIONS FOR TESTOSTERONE THERAPY  

Microsoft Academic Search

Purpose: Metabolic syndrome, characterized by central obesity, insulin resistance, dyslipide- mia and hypertension, is highly prevalent in the United States. When left untreated, it signifi- cantly increases the risk of diabetes mellitus and cardiovascular disease. It has been suggested that hypogonadism may be an additional component of metabolic syndrome. This has potential implications for the treatment of metabolic syndrome with

NAWRAS MAKHSIDA; JAY SHAH; GRACE YAN; HARRY FISCH; RIDWAN SHABSIGH

2005-01-01

55

SMART syndrome (stroke-like migraine attacks after radiation therapy) in adult and pediatric patients.  

PubMed

SMART syndrome (stroke-like migraine attacks after radiation therapy) is a rare condition that involves complex migraines with focal neurologic findings in patients following cranial irradiation for central nervous system malignancies. Little is known about the mechanisms behind the disorder, making successful treatment challenging. We report 2 new cases of SMART syndrome in pediatric patients as well as review all documented cases of the syndrome. Each of our 2 pediatric patients suffered multiple episodes. Attacks were characterized by severe headache, visual disturbance, aphasia, and weakness. Recovery occurred over several days to weeks. The data from all documented reports of SMART syndrome indicate a greater prevalence for male gender. An age-dependent pattern of onset was also observed, with a greater variability of syndrome onset in patients who received cranial irradiation at a younger age. SMART appears to be a reversible, recurrent long-term complication of radiation therapy with possible age- and gender-related influences. PMID:23364656

Armstrong, Amy E; Gillan, Eileen; DiMario, Francis Joseph

2014-03-01

56

La conductivité thermique ‘ŕ sec’ des bétons cellulaires autoclavés: un modčle conceptuel  

Microsoft Academic Search

Résumé  Dans cet article, nous présentons un modčle de l'influence du taux de macroporosité cellulaire sur la conductivité thermique\\u000a ŕ sec des bétons cellulaires autoclavés qui procure un cadre conceptuel efficace dans la perspective d'une amélioation des\\u000a performances thermiques de ce matériau. Pour construire ce modčle, nous séparons micro et macroporosité et nous considérons\\u000a la structure interne du béton cellulaire comme

J.-P. Laurent

1991-01-01

57

Metabolic syndrome–related hepatocellular carcinoma treated by volumetric modulated arc therapy  

PubMed Central

Hepatocellular carcinoma (hcc) is a leading cause of cancer mortality, and its incidence is increasing in developed countries. Risk factors include cirrhosis from viral hepatitis or alcohol abuse. Metabolic syndrome is a newly recognized, but important, risk factor that is likely contributing to the increased incidence of hcc. Surgery is the therapy of choice for hcc, but local therapies are often contraindicated, usually because of advanced disease or comorbid conditions such as cardiac disease (which is associated with metabolic syndrome). Current radiation therapy techniques such as stereotactic body radiotherapy allow for treatment plans that highly conform to the target and provide excellent sparing of normal structures. Radiation therapy is emerging as a viable option in patients not eligible for surgery or other locoregional therapies. Here, we report a case of a large hcc presenting in a patient with metabolic syndrome without significant alcohol history or biochemical liver dysfunction. The patient was not a candidate for locoregional therapies because of cardiac and renal comorbidities typical of patients experiencing the long-term sequelae of metabolic syndrome. Treatment using an arc-based volumetric-modulated arc therapy technique allowed for the highest dose of radiation to be delivered to the tumour while the peripheral radiation dose was minimized. A complete local response was confirmed by computed tomography imaging 21 months after treatment completion. PMID:24764717

Klein, J.; Dawson, L.A.; Tran, T.H.; Adeyi, O.; Purdie, T.; Sherman, M.; Brade, A.

2014-01-01

58

Myelodysplastic syndrome evolving from aplastic anemia treated with immunosuppressive therapy: efficacy of hematopoietic stem cell transplantation.  

PubMed

A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including post-aplastic anemia myelodysplastic syndrome. Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic hematopoietic stem cell transplantation, and in a matched-pair analysis compared outcome to that in 393 patients with de novo myelodysplastic syndrome. There was no difference in overall survival. There were no significant differences with regard to 5-year probabilities of relapse, non-relapse mortality, relapse-free survival and overall survival; these were 14%, 40%, 46% and 49% for post-aplastic anemia myelodysplastic syndrome, and 20%, 33%, 47% and 49% for de novo myelodysplastic syndrome, respectively. In multivariate analysis, relapse (hazard ratio 0.71; P=0.18), non-relapse mortality (hazard ratio 1.28; P=0.18), relapse-free survival (hazard ratio 0.97; P=0.80) and overall survival (hazard ratio 1.02; P=0.88) of post-aplastic anemia myelodysplastic syndrome were similar to those of patients with de novo myelodysplastic syndrome. Cytogenetic risk was independently associated with overall survival in both groups. Thus, transplant success in patients with post-aplastic anemia myelodysplastic syndrome was similar to that in patients with de novo myelodysplastic syndrome, and cytogenetics was the only significant prognostic factor for post-aplastic anemia myelodysplastic syndrome patients. PMID:25107891

Kim, Sung-Yong; Le Rademacher, Jennifer; Antin, Joseph H; Anderlini, Paolo; Ayas, Mouhab; Battiwalla, Minoo; Carreras, Jeanette; Kurtzberg, Joanne; Nakamura, Ryotaro; Eapen, Mary; Deeg, H Joachim

2014-12-01

59

Myelodysplastic syndrome evolving from aplastic anemia treated with immunosuppressive therapy: efficacy of hematopoietic stem cell transplantation  

PubMed Central

A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including post-aplastic anemia myelodysplastic syndrome. Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic hematopoietic stem cell transplantation, and in a matched-pair analysis compared outcome to that in 393 patients with de novo myelodysplastic syndrome. There was no difference in overall survival. There were no significant differences with regard to 5-year probabilities of relapse, non-relapse mortality, relapse-free survival and overall survival; these were 14%, 40%, 46% and 49% for post-aplastic anemia myelodysplastic syndrome, and 20%, 33%, 47% and 49% for de novo myelodysplastic syndrome, respectively. In multivariate analysis, relapse (hazard ratio 0.71; P=0.18), non-relapse mortality (hazard ratio 1.28; P=0.18), relapse-free survival (hazard ratio 0.97; P=0.80) and overall survival (hazard ratio 1.02; P=0.88) of post-aplastic anemia myelodysplastic syndrome were similar to those of patients with de novo myelodysplastic syndrome. Cytogenetic risk was independently associated with overall survival in both groups. Thus, transplant success in patients with post-aplastic anemia myelodysplastic syndrome was similar to that in patients with de novo myelodysplastic syndrome, and cytogenetics was the only significant prognostic factor for post-aplastic anemia myelodysplastic syndrome patients. PMID:25107891

Kim, Sung-Yong; Le Rademacher, Jennifer; Antin, Joseph H.; Anderlini, Paolo; Ayas, Mouhab; Battiwalla, Minoo; Carreras, Jeanette; Kurtzberg, Joanne; Nakamura, Ryotaro; Eapen, Mary; Deeg, H. Joachim

2014-01-01

60

Growth Hormone Therapy and Bone Mineral Density in Turner Syndrome  

Microsoft Academic Search

In a previous report, preliminary data showed a significant reduction in cortical bone mineral density (BMD) in women with Turner syndrome that had been treated with GH com- pared with women with Turner syndrome that had not been treated. To clarify this point, we have investigated the effects of GH treatment at multiple sites in this case-control, cross- sectional study.

VLADIMIR K. BAKALOV; PHILLIP L. VAN; JEFFREY BARON; JAMES C. REYNOLDS; CAROLYN A. BONDY

2010-01-01

61

Cognitive Behaviour Therapy for Children and Adults with Asperger's Syndrome  

Microsoft Academic Search

Asperger's syndrome is a relatively newly diagnosed developmental disorder within the autistic spectrum. Children and adults with Asperger's syndrome have an intellectual ability within the normal range but due to their unusual profile of cognitive, social and emotional abilities are vulnerable to the development of a secondary mood disorder. This paper provides an explanation of the abilities associated with Asperger's

Tony Attwood

2004-01-01

62

Outcome after three years of laronidase enzyme replacement therapy in a patient with Hurler syndrome  

Microsoft Academic Search

Summary   Enzyme replacement therapy (ERT) with laronidase, recombinant ?-l-iduronidase, for mucopolysaccharidosis type I (MPS I) has been clinically available since April 2003. Pre-approval studies\\u000a were performed on patients with the more attenuated forms of MPS I, Hurler–Scheie and Scheie syndromes. The clinical efficacy\\u000a of laronidase on the severe form of MPS I, Hurler syndrome, is not well known. We present

J. A. Thomas; S. Jacobs; J. Kierstein; J. Van Hove

2006-01-01

63

The Metabolic Syndrome and Mind-Body Therapies: A Systematic Review  

PubMed Central

The metabolic syndrome, affecting a substantial and increasing percentage of the worldwide population, is comprised of a cluster of symptoms associated with increased risk of type 2 diabetes, cardiovascular disease, and other chronic conditions. Mind-body modalities based on Eastern philosophy, such as yoga, tai chi, qigong, and meditation, have become increasingly popular worldwide. These complementary therapies have many reported benefits for improving symptoms and physiological measures associated with the metabolic syndrome. However, clinical trial data concerning the effectiveness of these practices on the syndrome as a whole have not been evaluated using a systematic and synthesizing approach. A systematic review was conducted to critically evaluate the data from clinical trials examining the efficacy of mind-body therapies as supportive care modalities for management of the metabolic syndrome. Three clinical trials addressing the use of mind-body therapies for management of the metabolic syndrome were identified. Findings from the studies reviewed support the potential clinical effectiveness of mind-body practices in improving indices of the metabolic syndrome. PMID:21773016

Anderson, Joel G.; Taylor, Ann Gill

2011-01-01

64

Advances in the therapy of the myelodysplastic syndromes  

Microsoft Academic Search

The myelodysplastic syndrome (MDS) has been a most challenging disease for hematologists for decades. In terms of diagnosis,\\u000a it was less than 20 years ago that the French-American-British (FAB) group developed criteria for the diagnosis and the classification\\u000a of the myelodysplastic syndromes [1]. The FAB classification has provided a common language for hematologists, morphologists, and pathologists in the investigation\\u000a of

Peter A. Kouides; John M. Bennett

65

Recommended Therapies for Metabolic Defects in Polycystic Ovary Syndrome  

Microsoft Academic Search

Polycystic ovary syndrome (PCOS) is not just a reproductive condition but has metabolic sequelae. The heterogeneity of the\\u000a condition is reflected in different emphases in patients on expression of these abnormalities. These conditions include effects\\u000a on lipids, glucose and insulin metabolism, cardiovascular system and weight control. The metabolic syndrome appears to be\\u000a more common in PCOS. Treatment of metabolic sequelae

Robert J. Norman; Anneloes E. Ruifrok; Lisa J. Moran; Rebecca L. Robker

66

Biology and therapy of fibromyalgia. Genetic aspects of fibromyalgia syndrome  

Microsoft Academic Search

Genetic and environmental factors may play a role in the etiopathology of fibromyalgia syndrome (FMS) and other related syndromes.\\u000a There is a high aggregation of FMS in families of FMS patients. The mode of inheritance is unknown but it is most probably\\u000a polygenic. There is evidence that polymorphisms of genes in the serotoninergic, dopaminergic and catecholaminergic systems\\u000a play a role

Dan Buskila; Piercarlo Sarzi-Puttini

2006-01-01

67

Bilateral carpal tunnel syndrome associated with interleukin 2 therapy.  

PubMed Central

We report the development of synchronous bilateral carpal tunnel syndrome in a woman with metastatic colorectal cancer, undergoing treatment with recombinant interleukin 2. A carpal tunnel decompression was carried out on the hand which was more severely affected, with a gradual recovery in median nerve function. To the best of our knowledge, this is the first reported case of carpal tunnel syndrome in association with recombinant interleukin 2. PMID:1437960

Heys, S. D.; Mills, K. L.; Eremin, O.

1992-01-01

68

Prospects for Gene Therapy in the Fragile X Syndrome  

ERIC Educational Resources Information Center

Gene therapy is unarguably the definitive way to treat, and possibly cure, genetic diseases. A straightforward concept in theory, in practice it has proven difficult to realize, even when directed to easily accessed somatic cell systems. Gene therapy for diseases in which the central nervous system (CNS) is the target organ presents even greater…

Rattazzi, Mario C.; LaFauci, Giuseppe; Brown, W. Ted

2004-01-01

69

Profound hypokalemia and hypochloremic metabolic alkalosis during thiazide therapy in a child with Pendred syndrome.  

PubMed

Pendred syndrome is a recessive autosomal disorder characterized by thyroid goiter and sensorineural hearing loss. The Pendred syndrome gene (SLC26A4) encodes a new anion exchanger named pendrin which mediates iodide transport by thyrocytes and regulates ion and fluid transport by the endolymphatic sac epithelium. Pendrin defects result in inner ear malformations, with enlargement of the endolymphatic sac and duct in association with a large vestibular aqueduct. Furthermore, patients may develop endolymphatic hydrops requiring diuretic therapy, mainly in the form of thiazides. Pendrin could also account for apical Cl(-)/ HCO3(-) exchange at level of intercalated cells of the cortical collecting duct in the kidneys, however, humans with Pendred syndrome have no symptoms attributable to renal pendrin abnormalities in basal conditions. We report the case of a child with Pendred syndrome and intercurrent endolymphatic hydrops, who developed profound hypokalemia and severe hypochloremic metabolic alkalosis (potassium 1.7, chloride 70, sodium 129, HCO3 43.8, base excess +17.8 mmol/l, pH 7.52) following thiazide therapy. In subjects with Pendred syndrome thiazide therapy seems to provoke more severe Cl(-) and extracellular volume depletion. A possible explanation could be the defective action of the disrupted pendrin, which exacerbates the effects of the inhibition of C1(-) reabsorption mediated by the thiazide-sensitive NaCl cotransporter (SLC12A3). PMID:18538122

Pela, I; Bigozzi, M; Bianchi, B

2008-06-01

70

Biology and therapy of fibromyalgia. Genetic aspects of fibromyalgia syndrome  

PubMed Central

Genetic and environmental factors may play a role in the etiopathology of fibromyalgia syndrome (FMS) and other related syndromes. There is a high aggregation of FMS in families of FMS patients. The mode of inheritance is unknown but it is most probably polygenic. There is evidence that polymorphisms of genes in the serotoninergic, dopaminergic and catecholaminergic systems play a role in the etiology of FMS. These polymorphisms are not specific for FMS and are associated with other functional somatic disorders and depression. Future genetic studies in the field of FMS and related conditions should be conducted in larger cohorts of patients and ethnically matched control groups. PMID:16887010

Buskila, Dan; Sarzi-Puttini, Piercarlo

2006-01-01

71

Biology and therapy of fibromyalgia. Genetic aspects of fibromyalgia syndrome.  

PubMed

Genetic and environmental factors may play a role in the etiopathology of fibromyalgia syndrome (FMS) and other related syndromes. There is a high aggregation of FMS in families of FMS patients. The mode of inheritance is unknown but it is most probably polygenic. There is evidence that polymorphisms of genes in the serotoninergic, dopaminergic and catecholaminergic systems play a role in the etiology of FMS. These polymorphisms are not specific for FMS and are associated with other functional somatic disorders and depression. Future genetic studies in the field of FMS and related conditions should be conducted in larger cohorts of patients and ethnically matched control groups. PMID:16887010

Buskila, Dan; Sarzi-Puttini, Piercarlo

2006-01-01

72

Implementing Cognitive Behavioral Therapy for Chronic Fatigue Syndrome in a Mental Health Center: A Benchmarking Evaluation  

Microsoft Academic Search

Objective: This study evaluated the success of implementing cognitive behavioral therapy (CBT) for chronic fatigue syndrome (CFS) in a representative clinical practice setting and compared the patient outcomes with those of previously published randomized controlled trials (RCTs) of CBT for CFS. Method: The implementation interventions were the following: spreading information about the new treatment setting to general practitioners and CFS

Korine Scheeres; Michel Wensing; Hans Knoop; Gijs Bleijenberg

2008-01-01

73

Role of Treadmill Training Versus Suspension Therapy On Balance In Children With Down Syndrome  

Microsoft Academic Search

Background and PurposeMaintaining balance is a subordinate but necessary requirement for most human actions. Most Down syndrome (DS) children, who constitute a large portion in our country, continue to evidence deficits in balance, co-ordination, and gait throughout childhood and adulthood. So, it is essential to seek an ideal physical therapy program to help in solving such widespread problem. The present

Gehan H. Elmeniway; Hebatallah M. Kamal; Samah A. Elshemy

74

Successful Treatment of Olfactory Reference Syndrome with Cognitive Behavioral Therapy: A Case Study  

ERIC Educational Resources Information Center

Olfactory reference syndrome (ORS) is characterized by a preoccupation with the belief that one's body emits a foul odor. Cognitive behavioral therapy (CBT) was used to treat a woman in her 50s who presented in our outpatient anxiety disorders specialty clinic with ORS, accompanied by embarrassment, shame, distress, avoidance behavior, and social…

Martin-Pichora, Andrea L.; Antony, Martin M.

2011-01-01

75

Preliminary experience with dance movement therapy in patients with chronic fatigue syndrome  

Microsoft Academic Search

To evaluate the influence of dance movement therapy (DMT) on the perception of well-being and functional capacity in women with chronic fatigue syndrome (CFS). Previous studies have analysed the effects of DMT in fibromyalgia but not specifically in CFS. Seven women diagnosed with CFS attended a 4-month DMT program. All of them performed a maximal physical test before and after

A. Blázquez; E. Guillamó; C. Javierre

2010-01-01

76

Hunter syndrome follow-up after 1?year of enzyme-replacement therapy  

PubMed Central

Mucopolysaccharidosis II (Hunter syndrome) is a rare x-linked disorder caused by a deficiency in the lysosomal enzyme iduronate-2-sulphatase, leading to an accumulation of the glycosaminoglycans (GAGs) dermatansulphate and heparan sulphate. The consequence of GAGs accumulation is progressive, multiorgan disease. Enzyme-replacement therapy is hypothesised to result in disease stabilisation and improved prognosis. We present a severe case of Hunter syndrome diagnosed at age 2?years and 4?months, who started enzyme-replacement therapy at the age of 3?years and 3?months. We report his evolution after 1?year of treatment. The treatment response was good and there was significant improvement in the quality of life. Owing to the rarity of Hunter syndrome, the multisystem nature and the heterogeneity of disease progression, patient care implies interdisciplinary consultations with a wide range of specialists. The best management can be provided in reference centres for metabolic diseases. PMID:23307460

Puiu, Maria; Chiri??-Emandi, Adela; Dumitriu, Simona; Arghirescu, Smaranda

2013-01-01

77

Electropalatographic Therapy for Children and Young People with Down's Syndrome  

ERIC Educational Resources Information Center

Articulation disorders in Down's syndrome (DS) are prevalent and often intractable. Individuals with DS generally prefer visual to auditory methods of learning and may therefore find it beneficial to be given a visual model during speech intervention, such as that provided by electropalatography (EPG). In this study, participants with Down's…

Cleland, Joanne; Timmins, Claire; Wood, Sara E.; Hardcastle, William J.; Wishart, Jennifer G.

2009-01-01

78

Therapy Insight: adipocytokines in metabolic syndrome and related cardiovascular disease  

Microsoft Academic Search

Abdominal fat accumulation has been shown to play crucial roles in the development of metabolic syndrome. Visceral fat accumulation particularly is closely correlated to the development of cardiovascular disease and obesity-related disorders such as diabetes mellitus, hyperlipidemia and hypertension. Given these clinical findings, the functions of adipocytes have been intensively investigated in the past 10 years, and have been revealed

Yuji Matsuzawa

2006-01-01

79

Complex regional pain syndrome – diagnostic, mechanisms, CNS involvement and therapy  

Microsoft Academic Search

Complex regional pain syndromes (CRPS, formerly reflex sympathetic dystrophy and causalgia) are neuropathic pain conditions of one extremity developing inadequately after a trauma. The initiating trauma affects primarily the extremity, but can also be a central lesion (e.g., spinal cord injury, stroke). CRPS is clinically characterized by sensory, autonomic and motor disturbances. Pathophysiologically there is evidence for functional changes within

G Wasner; J Schattschneider; A Binder; R Baron

2003-01-01

80

Electroconvulsive therapy in Parkinson's syndrome with “on-off” phenomenon  

Microsoft Academic Search

Summary During long-term treatment with L-dopa in Parkinson's syndrome “on-off” phenomenon develops in many cases, often entailing considerable therapeutic problems. Decreased sensitivity in postsynaptic striatal dopamine (DA) receptors has been shown to occur in parkinsonian patients during long-term treatment with L-dopa. This has been suggested as one possible mechanism for development of the “on-off” phenomenon. In contrast to L-dopa treatment

J. Balldin; S. Edén; A.-K. Granérus; K. Modigh; A. Svanborg; J. Wĺlinder; L. Wallin

1980-01-01

81

Fatty Acids in the Causation and Therapy of Metabolic Syndrome  

Microsoft Academic Search

The role of fatty acids in the prevention and pathogenesis of metabolic syndrome leading to cardiovascular diseases, type\\u000a 2 diabetes and insulin resistance are reviewed. We did Medline, PubMed search till March, 2007. Excess of linoleic acid, trans\\u000a fatty acids (TFA), saturated and total fat as well as refined starches and sugar are proinflammatory. Low dietary monounsaturated\\u000a fatty acids (MUFA)

Ram B. Singh; Fabien De Meester; Viola Mechirova; Daniel Pella; Kuniaki Otsuka

82

Menstrual Cyclicity After Metformin Therapy in Polycystic Ovary Syndrome  

Microsoft Academic Search

Objective: To assess the effect of insulin-lowering treatment on menstrual cyclicity in polycystic ovary syndrome (PCOS).Methods: Forty oligoamenorrheic women with PCOS were recruited in a prospective clinical study to receive metformin for a minimum period of 6 months. Twenty-two women completed the study. Serum LH, FSH, free testosterone, and glucose and insulin response to oral glucose load were measured both

Elsy Velázquez M; Arnaldo Acosta; Soaira G Mendoza

1997-01-01

83

[Recent advances of studies on the therapy-related myelodysplastic syndromes/acute myeloid leukemia].  

PubMed

Therapy-related myelodysplasia syndromes/acute myeloid leukemia (t-MDS/AML) is a clinical syndrome occurring as a late complication after chemotherapy and (or) radiotherapy, attracting much more attention owing to the improved treatment agents and longer survival of many treated patients. According to the WHO classification of 2008, t-MDS/AML is a serious complication of chemotherapy or radiotherapy given to a malignant or nonmalignant condition consisting of t-AML, t-MDS and t-MDS/myeloproliferative diseases (t-MDS/MPD). This review mainly focuses on the pathogenesis, relationship with primary tumour, treatment and prognosis of t-MDS/AML. PMID:25543511

Li, Chan-Juan; Zhang, Yi-Zhuo

2014-11-01

84

Carbohydrate-deficient glycoprotein syndrome type Ib. Phosphomannose isomerase deficiency and mannose therapy.  

PubMed Central

Phosphomannose isomerase (PMI) deficiency is the cause of a new type of carbohydrate-deficient glycoprotein syndrome (CDGS). The disorder is caused by mutations in the PMI1 gene. The clinical phenotype is characterized by protein-losing enteropathy, while neurological manifestations prevailing in other types of CDGS are absent. Using standard diagnostic procedures, the disorder is indistinguishable from CDGS type Ia (phosphomannomutase deficiency). Daily oral mannose administration is a successful therapy for this new type of CDG syndrome classified as CDGS type Ib. PMID:9525984

Niehues, R; Hasilik, M; Alton, G; Körner, C; Schiebe-Sukumar, M; Koch, H G; Zimmer, K P; Wu, R; Harms, E; Reiter, K; von Figura, K; Freeze, H H; Harms, H K; Marquardt, T

1998-01-01

85

Complement therapy in atypical haemolytic uraemic syndrome (aHUS)  

PubMed Central

Central to the pathogenesis of atypical haemolytic uraemic syndrome (aHUS) is over-activation of the alternative pathway of complement. Inherited defects in complement genes and autoantibodies against complement regulatory proteins have been described. The use of plasma exchange to replace non-functioning complement regulators and hyper-functional complement components in addition to the removal of CFH-autoantibodies made this the ‘gold-standard’ for management of aHUS. In the last 4 years the introduction of the complement inhibitor Eculizumab has revolutionised the management of aHUS. In this review we shall discuss the available literature on treatment strategies to date. PMID:23810412

Wong, Edwin K.S.; Goodship, Tim H.J.; Kavanagh, David

2013-01-01

86

FRAGILE X SYNDROME: PSYCHIATRIC MANIFESTATIONS, ASSESSMENT AND EMERGING THERAPIES  

PubMed Central

Fragile X Syndrome (FXS), the most common inherited cause of intellectual disabilities, is an X-linked dominant disorder caused by the amplification of a CGG repeat in the 5? untranslated region of the fragile X mental retardation gene 1 (FMR1). Prevalence estimates of the disorder are approximately 1/3600. Psychiatric manifestations of the disorder include anxiety, attention deficit hyperactivity disorder, autism, mood instability and aggression. In this article we review the above psychiatric manifestations and challenges to accurate assessment. We also discuss how the neurobiological underpinnings of these symptoms are beginning to be understood and can help guide treatment.

Wadell, Paula M.; Hagerman, Randi J.; Hessl, David R.

2015-01-01

87

[New perspectives on molecular and genic therapies in Down syndrome].  

PubMed

Trisomy 21 was first described as a syndrome in the middle of the nineteenth century and associated to a chromosomic anomaly one hundred years later: the most salient feature of this syndrome is a mental retardation of variable intensity. Molecular mapping and DNA sequencing have allowed identifying the gene content of chromosome 21. Molecular quantitative analyses indicated that trisomy is inducing an overexpression for a large part of the triplicated genes and deregulates also pathways involving non HSA21 genes. Together with the physiological description of murine models overexpressing orthologous genes, these data have allowed to elaborate hypotheses on the cause of cognitive impairment. From these hypotheses and using murine models it is now possible to assess the efficiency of various therapeutic strategies. This paper reviews these new perspectives starting from the strategies targeting the level of HSA21 RNAs or HSA21 proteins; then it describes methods targeting activities either of proteins involved in cell cycle pathways or of proteins controlling the synaptic plasticity. It is promising that strategies targeting specific genes or specific pathways are already giving positive results. PMID:20412741

Delabar, Jean Maurice

2010-04-01

88

[Neurobiology, clinical characteristics and therapy in Tourette's syndrome].  

PubMed

Tourette's syndrome (TS) is characterised by motor and vocal tics. The clinical manifestation during childhood and the frequent remission during early adulthood point to a dysregulation in the maturation of neuronal pathways. As a neurobiological correlate, the cortico-striato-thalamo-cortical circuit is the main focus of research. Two main features of a movement, the speed and direction of a movement, are controlled by the basal ganglia. Both features are dysregulated in TS.? Besides the motor symptoms, patients with TS suffer from comorbidities such as attention deficit hyperactivity syndrome, depression and obsessive compulsive disorders. Tics are modified in frequency and intensity by actions that require a high level of concentration such as reading or by distress, e.g., teasing by peers. The results of structural and functional imaging data support the hypothesis of altered signal transmission in the basal ganglia and a dysfunction in the limbic system. They also point to a complex interaction between cortical motor areas, the anterior cingulum, prefrontal regions and the basal ganglia. PMID:22161167

Neuner, I; Ludolph, A G

2011-12-01

89

Australian venomous jellyfish, envenomation syndromes, toxins and therapy.  

PubMed

The seas and oceans around Australia harbour numerous venomous jellyfish. Chironex fleckeri, the box jellyfish, is the most lethal causing rapid cardiorespiratory depression and although its venom has been characterised, its toxins remain to be identified. A moderately effective antivenom exists which is also partially effective against another chirodropid, Chiropsalmus sp. Numerous carybdeids, some unidentified, cause less severe illness, including Carybdea rastoni whose toxins CrTX-A and CrTX-B are large proteins. Carukia barnesi, another small carybdeid is one cause of the 'Irukandji' syndrome which includes delayed pain from severe muscle cramping, vomiting, anxiety, restlessness, sweating and prostration, and occasionally severe hypertension and acute cardiac failure. The syndrome is in part caused by release of catecholamines but the cause of heart failure is undefined. The venom contains a sodium channel modulator. Two species of Physalia are present and although one is potentially lethal, has not caused death in Australian waters. Other significant genera of jellyfish include Tamoya, Pelagia, Cyanea, Aurelia and Chyrosaora. PMID:16928389

Tibballs, James

2006-12-01

90

Electrical stimulation therapy improves sleep respiratory parameters in obstructive sleep apnea syndrome: a meta-analysis.  

PubMed

Recent clinical trials have shown that electrical stimulation has beneficial effects in obstructive sleep apnea syndrome (OSAS). The purpose of this study was to evaluate the efficacy of electrical stimulation therapy for OSAS with a meta-analysis. The meta-analysis of all relative studies was performed through searching international literature, including PUBMED, CNKI, and EMBASE databases. This literature analysis compared all patients undergoing electrical stimulation therapy with respect to the respiratory disturbance index (RDI) and changes in sleep structure. Six studies were selected involving a total of 91 patients. The meta-analysis indicated that electrical stimulation therapy reduced RDI, longest apnea time, and improved the minimum SaO2. Based on the evidence found, electrical stimulation may be a potential therapy for OSAS, warranting further clinical trials. PMID:24142709

Tan, Jie-wen; Qi, Wei-wei; Ye, Rui-xin; Wu, Yuan-yuan

2013-10-01

91

Iron overload and chelation therapy in myelodysplastic syndromes.  

PubMed

Iron overload remains a concern in MDS patients especially those requiring recurrent blood transfusions. The consequence of iron overload may be more relevant in patients with low and intermediate-1 risk MDS who may survive long enough to experience such manifestations. It is a matter of debate whether this overload has time to yield organ damage, but it is quite evident that cellular damage and DNA genotoxic effect are induced. Iron overload may play a critical role in exacerbating pre-existing morbidity or even unmask silent ones. Under these circumstances, iron chelation therapy could play an integral role in the management of these patients. This review entails an in depth analysis of iron overload in MDS patients; its pathophysiology, effect on survival, associated risks and diagnostic options. It also discusses management options in relation to chelation therapy used in MDS patients and the impact it has on survival, hematologic response and organ function. PMID:24529413

Temraz, Sally; Santini, Valeria; Musallam, Khaled; Taher, Ali

2014-07-01

92

Emerging Pharmacologic Therapies for Constipation-predominant Irritable Bowel Syndrome and Chronic Constipation  

PubMed Central

Irritable bowel syndrome with constipation and chronic functional constipation are common digestive disorders that negatively impact quality of life and account for billions of dollars in health care costs. Related to the heterogeneity of pathogenesis that underlie these disorders and the failure of symptoms to reliably predict underlying pathophysiology, traditional therapies provide relief to only a subset of affected individuals. The evidence surrounding new and emerging pharmacologic treatments, which include both luminally and systemically acting drugs, is discussed here. These include agents such as lubiprostone, bile acid modulations, guanylate cyclase-C receptor agonists, serotonin receptor modulators and herbal therapies. PMID:24840367

Eswaran, Shanti; Guentner, Amanda; Chey, William D

2014-01-01

93

[Extracorporeal shock wave therapy for chronic pelvic pain syndrome: an update].  

PubMed

Chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) is a common disease in males, which is characterized by persistent discomfort or pain in the pelvic region. As currently used drug therapies fail to produce satisfactory results, it is an urgent task to find new and effective methods for the treatment of CP/CPPS. In recent years, many reports are seen on the extracorporeal shock wave therapy (ESWT) for CP/CPPS. ESWT can significantly improve the symptoms of pelvic pain and urination disorders in CPPS patients, and its therapeutic effect is attributed to the improvement of angiogenesis and block of pain nerves. PMID:25195375

Guo, Qi; Hong, Mei; Wang, Zhi-Ping

2014-08-01

94

Metformin therapy decreases hyperandrogenism and hyperinsulinemia in women with polycystic ovary syndrome  

Microsoft Academic Search

Objective: To evaluate the effects of 12 weeks of metformin therapy on hormonal and clinical indices in polycystic ovary syndrome (PCOS).Design: Prospective study.Setting: University hospital.Patient(s): Thirty-nine women with PCOS and fasting hyperinsulinemia.Intervention(s): Twelve weeks of therapy with oral metformin (500 mg three times per day).Main Outcome Measure(s): Levels of insulin, T, DHEAS, insulin-like growth factor-I (IGF-I), gonadotropins, and sex hormone-binding

Beata Kolodziejczyk; Antoni J Duleba; Robert Z Spaczynski; Leszek Pawelczyk

2000-01-01

95

Successful treatment of resistant SAPHO syndrome with anti-TNF therapy  

PubMed Central

A 42-year-old Caucasian woman with SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) refractory to non-steroidal anti-inflammatory drugs, sulfasalzine, methotrexate, bisphosphonates and steroids was successfully treated with antitumour necrosis factor therapy (infliximab). This case was a diagnostic challenge leading to extensive investigations for infection and malignancy and delayed diagnosis for several years. We report the significant improvement in clinical, radiological and laboratory markers of disease activity on infliximab and the steroid sparing effect of such therapy. PMID:23355559

Hampton, Shayma Lamya; Youssef, Hazem

2013-01-01

96

Endoscopic therapy for esophageal hematoma with blue rubber bleb nevus syndrome  

PubMed Central

A 57-year-old woman previously diagnosed with blue rubber bleb nevus syndrome (BRBNS) reported hematemesis. BRBNS is a rare vascular anomaly syndrome consisting of multifocal hemangiomas of the skin and gastrointestinal (GI) tract but her GI tract had never been examined. An upper gastrointestinal endoscopy revealed a large bleeding esophageal hematoma positioned between the thoracic esophagus and the gastric cardia. An endoscopic injection of polidocanol was used to stop the hematoma from bleeding. The hematoma was incised using the injection needle to reduce the pressure within it. Finally, argon plasma coagulation (APC) was applied to the edge of the incision. The esophageal hematoma disappeared seven days later. Two months after the endoscopic therapy, the esophageal ulcer healed and the hemangioma did not relapse. This rare case of a large esophageal hematoma originating from a hemangioma with BRBNS was treated using a combination of endoscopic therapy with polidocanol injection, incision, and APC. PMID:25512774

Takasumi, Mika; Hikichi, Takuto; Takagi, Tadayuki; Sato, Masaki; Suzuki, Rei; Watanabe, Ko; Nakamura, Jun; Sugimoto, Mitsuru; Waragai, Yuichi; Kikuchi, Hitomi; Konno, Naoki; Watanabe, Hiroshi; Obara, Katsutoshi; Ohira, Hiromasa

2014-01-01

97

Significant fibrosis after radiation therapy in a patient with Marfan syndrome  

PubMed Central

Marfan syndrome is one of the collagen vascular diseases that theoretically predisposes patients to excessive radiation-induced fibrosis yet there is minimal published literature regarding this clinical scenario. We present a patient with a history of Marfan syndrome requiring radiation for a diagnosis of a right brachial plexus malignant nerve sheath tumor. It has been suggested that plasma transforming growth factor beta 1 (TGF-?1) can be monitored as a predictor of subsequent fibrosis in this population of high risk patients. We therefore monitored the patient's TGF-?1 level during and after treatment. Despite maintaining stable levels of plasma TGF-?1, our patient still developed extensive fibrosis resulting in impaired range of motion. Our case reports presents a review of the literature of patients with Marfan syndrome requiring radiation therapy and the limitations of serum markers on predicting long-term toxicity. PMID:25324993

Knackstedt, Rebecca J.; Jenrette, Joseph M.

2014-01-01

98

Post-polio syndrome: Pathophysiological hypotheses, diagnosis criteria, drug therapy.  

PubMed

Post-polio syndrome (PPS) refers to a clinical disorder affecting polio survivors with sequelae years after the initial polio attack. These patients report new musculoskeletal symptoms, loss of muscular strength or endurance. PPS patients are tired, in pain and experience new and unusual muscular deficits, on healthy muscles as well as deficient muscles initially affected by the Poliovirus. Once a clinical diagnosis is established, the therapeutic options can be discussed. Some pathophysiological mechanisms have been validated by research studies on PPS (inflammatory process in cerebrospinal fluid [CSF] and cytokines of the immune system). Several studies have been conducted to validate medications (pyridostigmine, immunoglobulin, coenzyme Q10) or physical exercises protocols. This article focuses on the relevance and efficacy that can be expected from these therapeutics. Very few studies reported some improvements. Medications combined to individual and supervised exercise training programs are promising therapeutic strategies for PPS patients care management. PMID:20093102

Boyer, F-C; Tiffreau, V; Rapin, A; Laffont, I; Percebois-Macadré, L; Supper, C; Novella, J-L; Yelnik, A-P

2010-02-01

99

Effectiveness and Limitations of b-Blocker Therapy in Congenital Long-QT Syndrome  

Microsoft Academic Search

Background— b-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness and limitations of b-blockers in this disorder have not been evaluated. Methods and Results—The study population comprised 869 LQTS patients treated with b-blockers. Effectiveness of b-blockers was analyzed during matched periods before and after starting b-blocker therapy, and by survivorship methods to determine factors associated with cardiac

Arthur J. Moss; Wojciech Zareba; Peter J. Schwartz; Richard S. Crampton; Jesaia Benhorin; G. Michael Vincent; Emanuela H. Locati; Silvia G. Priori; Carlo Napolitano; Aharon Medina; Li Zhang; Jennifer L. Robinson; Katherine W. Timothy; Jeffrey A. Towbin; Mark L. Andrews

2000-01-01

100

New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome  

Microsoft Academic Search

Although many children with idiopathic nephrotic syndrome (INS) respond initially to steroid therapy, repeated courses for\\u000a patients with relapses often cause significant steroid toxicity. Patients with frequent relapses who develop steroid dependency\\u000a thus require alternative treatment. The first such options have been considered to be cyclophosphamide or levamisole, although\\u000a the latter is no longer available in many countries. There is

Michael van Husen; Markus J. Kemper

2011-01-01

101

The Effect of Nutritional Therapy for Yeast Infection (Candidiasis) in Cases of Chronic Fatigue Syndrome  

Microsoft Academic Search

The objective of this study was to evalu- ate the effectiveness of nutritional therapy for yeast infection in cases of medically-diag- nosed Chronic Fatigue Syndrome\\/ME. Forty participants, each with medically- diagnosed CFS\\/ME, received individual nu- tritional advice for treating yeast infection (candidiasis) over the course of one year, fol- lowing an anti-candida protocol which had been used successfully in nutritional

Erica White; Caroline Sherlock

102

[Effectiveness of duspatalin therapy in the treatment and prevention of post-cholecystectomy syndrome].  

PubMed

Results of evaluation of the efficiency of myotropic spasmolytic Duspatalin during long-term therapy and preventive treatment of functional post-cholecystectomy syndrome are presented. The influence of the treatment on manifestations of clinical symptoms, quality of a life estimated based on a visual-analog scale, and intestinal microbiocenosis (changes in the activity of short-chain fatty acids) are discussed. PMID:21395089

Lipnitski?, E M; Isakova, O V; Movchun, V A; Apdatskaia, M D

2011-01-01

103

Mutational analysis of therapy-related myelodysplastic syndromes and acute myelogenous leukemia.  

PubMed

Therapy-related myelodysplastic syndromes and acute myelogenous leukemia comprise a poor-risk subset of myelodysplastic syndromes and acute myelogenous leukemia. Large-scale mutation profiling efforts in de novo myelodysplastic syndromes have identified mutations that correlate with clinical features, but such mutations have not been investigated in therapy-related myelodysplastic syndromes and acute myelogenous leukemia. Genomic DNA from 38 patient samples were subjected to high throughput polymerase chain reaction and sequenced for TP53, TET2, DNMT3A, ASXL1, IDH1, IDH2, EZH2, EED, SUZ12, RBBP4, SRSF2, U2AF35, and SF3B1. We identified somatic mutations in 16 of 38 (42%) patients. TP53 mutations were the most common lesion, detected in 8 of 38 (21%) patients, followed by TET2 in 4 of 38 (10.5%). Cases with a TP53 mutation or loss of the TP53 locus had a worse overall survival compared to those with wild-type TP53 (8.8 vs. 37.4 months; P=0.0035). PMID:23349305

Shih, Alan H; Chung, Stephen S; Dolezal, Emily K; Zhang, Su-Jiang; Abdel-Wahab, Omar I; Park, Christopher Y; Nimer, Stephen D; Levine, Ross L; Klimek, Virginia M

2013-06-01

104

Fragile X Syndrome: From molecular pathology to therapy.  

PubMed

Fragile X Syndrome (FXS) is the most common form of inherited intellectual disability due to the silencing of the FMR1 gene encoding FMRP (Fragile X Mental Retardation Protein), an RNA-binding protein involved in different steps of RNA metabolism. Of particular interest is the key role of FMRP in translational regulation. Since the first functional characterizations of FMRP, its role has been underlined by its association with actively translating polyribosomes. Furthermore, a plethora of mRNA targets of FMRP have been identified. In the absence of FMRP the deregulation of translation/transport/stability of these mRNAs has a cascade effect on many pathways, resulting into the final phenotype. We review here a set of targets of FMRP (mRNAs and proteins) that may have an impact on the FXS phenotype by deregulating some key cellular processes, such as translation, cytoskeleton remodeling and oxidative stress. The manipulation of these abnormal pathways by specific drugs may represent new therapeutic opportunities for FXS patients. PMID:24462888

Maurin, Thomas; Zongaro, Samantha; Bardoni, Barbara

2014-10-01

105

Drug therapy options for patients with irritable bowel syndrome.  

PubMed

Existing pharmacotherapeutic options for the treatment of patients with irritable bowel syndrome (IBS) are limited in treating the multiple symptoms associated with the disorder. There is much interest in the use of serotonin agents as new therapeutics. Acting primarily through 5-HT3 and 5-HT4 receptors, serotonin elicits changes in motor function and possibly visceral sensation. Two serotonin agents were developed specifically for IBS: tegaserod, a 5-HT4 receptor partial agonist, and alosetron, a 5-HT3 receptor antagonist (which is no longer available). Phase III clinical trial data show that during a 12-week treatment period with tegaserod, IBS patients with abdominal pain and discomfort, bloating, and constipation experienced significant global relief (i.e., improvement in overall well-being, abdominal pain, and bowel habit) compared with placebo. Improvement in bowel movement frequency and consistency was achieved and pain was relieved by 1 week. During 12 weeks of treatment, alosetron was shown to elicit significant relief of abdominal pain and discomfort compared with placebo or mebeverine in female IBS patients with diarrhea. Alosetron slowed colonic transit and treatment efficacy was apparent after a week of treatment. Another 5-HT4 receptor agonist, prucalopride, which is being developed for chronic constipation, accelerates colonic transit and increases stool frequency. Therefore, this agent may be of benefit in IBS patients with constipation. PMID:11474911

Talley, N J

2001-07-01

106

Traditional therapies for irritable bowel syndrome: an evidence-based appraisal.  

PubMed

Irritable bowel syndrome (IBS) is a common chronic disease that adversely effects quality of life and is associated with substantial direct and indirect health care costs. It is defined by a constellation of symptoms in conjunction with an alteration in bowel function and defecation, and its underlying pathophysiological basis remains unknown. Numerous therapies are available, but many relieve only one symptom of the syndrome, and their effectiveness has not been demonstrated with adequate evidence from high-quality studies. This article reviews the criteria for appropriate design of any treatment study as well as those criteria specific to studies of treatment for functional gastrointestinal diseases. Using these criteria, the author evaluates the published investigations of "traditional" IBS therapies (antidepressants, bulking agents/fiber, antispasmodics, antidiarrheals) and "alternative" IBS therapies (Chinese herbal supplements, peppermint oil, behavioral therapies). Based on this evaluation, the author concludes that the available evidence does not support the effectiveness of any of these treatments and, therefore, none of these treatments can be reliably recommended. PMID:12775999

Fennerty, M Brian

2003-01-01

107

The prospect of molecular therapy for Angelman syndrome and other monogenic neurologic disorders  

PubMed Central

Background Angelman syndrome is a monogenic neurologic disorder that affects 1 in 15,000 children, and is characterized by ataxia, intellectual disability, speech impairment, sleep disorders, and seizures. The disorder is caused by loss of central nervous system expression of UBE3A, a gene encoding a ubiquitin ligase. Current treatments focus on the management of symptoms, as there have not been therapies to treat the underlying molecular cause of the disease. However, this outlook is evolving with advances in molecular therapies, including artificial transcription factors a class of engineered DNA-binding proteins that have the potential to target a specific site in the genome. Results Here we review the recent progress and prospect of targeted gene expression therapies. Three main issues that must be addressed to advance toward human clinical trials are specificity, toxicity, and delivery. Conclusions Artificial transcription factors have the potential to address these concerns on a level that meets and in some cases exceeds current small molecule therapies. We examine the possibilities of such approaches in the context of Angelman syndrome, as a template for other single-gene, neurologic disorders. PMID:24946931

2014-01-01

108

The Effect of Polarized Polychromatic Noncoherent Light (Bioptron) Therapy on Patients with Carpal Tunnel Syndrome  

PubMed Central

Introduction: To study the effects of Polarized Polychromatic Noncoherent Light (Bioptron) therapy on patients with carpal tunnel syndrome (CTS). Methods: This study was designed as a randomized clinical trial. Forty four patients with mild or moderate CTS (confirmed by clinical and electrodiagnostic studies) were assigned randomly into two groups (intervention and control goups). At the beginning of the study, both groups received wrist splinting for 8 weeks. Bioptron light was applied for the intervention group (eight sessions, for 3/weeks). Bioptron was applied perpendicularly to the wrist from a 10 centimeter sdistance. Pain severity and electrodiagnostic measurements were compared from before to 8 weeks after initiating each treatment. Results: Eight weeks after starting the treatments, the mean of pain severity based on Visual Analogue Scale (VAS) scores decreased significantly in both groups. Median Sensory Nerve Action Potential (SNAP) latency decreased significantly in both groups. However, other electrophysiological findings (median Compound Motor Action Potential (CMAP) latency and amplitude, also SNAP amplitude) did not change after the therapy in both groups. There was no meaningful difference between two groups regarding the changes in the pain severity. Conclusion: Bioptron with the above mentioned parameters led to therapeutic effects equal to splinting alone in patients with carpal tunnel syndrome. However, applying Bioptron with different therapeutic protocols and light parameters other than used in this study, perhaps longer duration of therapy and long term assessment may reveal different results favoring Bioptron therapy. PMID:25606338

Raeissadat, Seyed Ahmad; Rayegani, Seyed Mansoor; Rezaei, Sajad; Bahrami, Mohammad Hasan; Eliaspour, Dariush; Karimzadeh, Afshin

2014-01-01

109

Combination therapy of ursodeoxycholic acid and budesonide for PBC–AIH overlap syndrome: a meta-analysis  

PubMed Central

In this study, a meta-analysis of randomized controlled trials comparing ursodeoxycholic acid (UDCA) monotherapy with combination therapies utilizing UDCA and budesonide was performed. We found that combination therapy with UDCA and budesonide was more effective than UDCA monotherapy for primary biliary cirrhosis–autoimmune hepatitis overlap syndrome. Moreover, compared to prednisone, budesonide has fewer side effects.

Zhang, Huawei; Yang, Jing; Zhu, Rong; Zheng, Yuanyuan; Zhou, Yuqing; Dai, Weiqi; Wang, Fan; Chen, Kan; Li, Jingjing; Wang, Chengfen; Li, Sainan; Liu, Tong; Abudumijiti, Huerxidan; Zhou, Zheng; Wang, Jianrong; Lu, Wenxia; Wang, Junshan; Xia, Yujing; Zhou, Yingqun; Lu, Jie; Guo, Chuanyong

2015-01-01

110

A randomized controlled trial of cognitive behavior therapy, relaxation training, and routine clinical care for the irritable bowel syndrome  

Microsoft Academic Search

OBJECTIVES:Psychological treatments are considered to be useful in the irritable bowel syndrome (IBS), although the evidence is based on small, often flawed trials. Although cognitive behavior therapy (CBT) and relaxation therapy have both been promising, we hypothesized that CBT would be superior to relaxation and standard care alone in IBS patients. The objective of this study was to test this

Philip M. Boyce; Nicholas J. Talley; Belinda Balaam; Natasha A. Koloski; George Truman

2003-01-01

111

Toward resolving the unsettled role of iron chelation therapy in myelodysplastic syndromes.  

PubMed

Transfusion dependent low risk myelodysplastic syndromes (MDS) patients, eventually develop iron overload. Iron toxicity, via oxidative stress, can damage cellular components and impact organ function. In thalassemia major patients, iron chelation therapy lowered iron levels with recovery of cardiac and liver functions and significant improvement in survival. Several noncontrolled studies show inferior survival in MDS patients with iron overload, including an increase in transplant-related mortality and infection risk while iron chelation appears to improve survival in both lower risk MDS patients and in stem cell transplant settings. Collated data are presented on the pathophysiological impact of iron overload; measuring techniques and chelating agents' therapy positive impact on hematological status and overall survival are discussed. Although suggested by retrospective analyses, the lack of clear prospective data of the beneficial effects of iron chelation on morbidity and survival, the role of iron chelation therapy in MDS patients remains controversial. PMID:24641787

Merkel, Drorit G; Nagler, Arnon

2014-07-01

112

Pharmacologic and Complementary and Alternative Medicine Therapies for Irritable Bowel Syndrome  

PubMed Central

Irritable bowel syndrome (IBS) is a chronic functional gastrointestinal disorder characterized by episodic abdominal pain or discomfort in association with altered bowel habits (diarrhea and/or constipation). Other gastrointestinal symptoms, such as bloating and flatulence, are also common. A variety of factors are believed to play a role in the development of IBS symptoms, including altered bowel motility, visceral hypersensitivity, psychosocial stressors, altered brain-gut interactions, immune activation/low grade inflammation, alterations in the gut microbiome, and genetic factors. In the absence of biomarkers that can distinguish between IBS subgroups on the basis of pathophysiology, treatment of this condition is predicated upon a patient's most bothersome symptoms. In clinical trials, effective therapies have only offered a therapeutic gain over placebos of 7-15%. Evidence based therapies for the global symptoms of constipation predominant IBS (IBS-C) include lubiprostone and tegaserod; evidence based therapies for the global symptoms of diarrhea predominant IBS (IBS-D) include the probiotic Bifidobacter infantis, the nonabsorbable antibiotic rifaximin, and alosetron. Additionally, there is persuasive evidence to suggest that selected antispasmodics and antidepressants are of benefit for the treatment of abdominal pain in IBS patients. Finally, several emerging therapies with novel mechanisms of action are in development. Complementary and alternative medicine therapies including probiotics, herbal therapies and acupuncture are gaining popularity among IBS sufferers, although concerns regarding manufacturing standards and the paucity of high quality efficacy and safety data remain. PMID:21927652

Maneerattaporn, Monthira; Saad, Richard

2011-01-01

113

The Effect of Extracorporeal Shock Wave Therapy on Myofascial Pain Syndrome  

PubMed Central

Objective To investigate the effect of extracorporeal shock wave therapy (ESWT) on myofascial pain syndrome (MPS). Method Thirty patients with MPS in trapezius muscle were randomly divided into two groups, ESWT group (n=15), and trigger point injections (TPI)+transcutaneous electrical nerve stimulation (TENS) group (n=15). For a total of 3 weeks, ESWT was undertaken with 1,500 pulse each time at one week interval totaling 4,500 pulse, TPI for once a week totaling three times and TENS for five times a week totaling three weeks. Results The changes in pain threshold (lb/cm2) showed the values of 6.86±1.35 before first therapy, 11.43±0.27 after first therapy, and 12.57±0.72 after third therapy, while TPI+TENS group showed the values of 6.20±1.92 before first therapy, 8.80±0.48 after first therapy, and 9.60±2.19 after third therapy, and the changes between the groups were significantly different (p=0.045). The changes in visual analog scale were estimated to be 6.86±0.90 before first therapy, 2.86±0.90 after first therapy, and 1.86±0.69 after third therapy in case of ESWT group, whereas the figures were estimated to be 7.20±1.30 before first therapy, 4.60±0.55 after first therapy, and 2.80±0.84 after third therapy in case of TPI+TENS group, and the changes between the groups were significantly different (p=0.010). The changes in McGill pain questionnaire (p=0.816) and pain rating scale (p=0.644) between the groups were not significantly different. The changes in neck ROM were also not significantly different between the groups (p>0.05). Conclusion The ESWT in patients with MPS in trapezius muscle are as effective as TPI and TENS for the purpose of pain relief and improving cervical range of motion. PMID:23185731

Jeon, Jong Hyun; Jung, Yun Jae; Lee, Ju Youn; Choi, Ji Soo; Mun, Jeong Hyeon; Park, Won Yong; Seo, Cheong Hoon

2012-01-01

114

Cardiac ryanodine receptor in metabolic syndrome: is JTV519 (K201) future therapy?  

PubMed Central

Metabolic syndrome is characterized by a combination of obesity, hypertension, insulin resistance, dyslipidemia, and impaired glucose tolerance. This multifaceted syndrome is often accompanied by a hyperdynamic circulatory state characterized by increased blood pressure, total blood volume, cardiac output, and metabolic tissue demand. Experimental, epidemiological, and clinical studies have demonstrated that patients with metabolic syndrome have significantly elevated cardiovascular morbidity and mortality rates. One of the main and frequent complications seen in metabolic syndrome is cardiovascular disease. The primary endpoints of cardiometabolic risk are coronary and peripheral arterial disease, myocardial infarction, congestive heart failure, arrhythmia, and stroke. Alterations in expression and/or functioning of several key proteins involved in regulating and maintaining ionic homeostasis can cause cardiac disturbances. One such group of proteins is known as ryanodine receptors (intracellular calcium release channels), which are the major channels through which Ca2+ ions leave the sarcoplasmic reticulum, leading to cardiac muscle contraction. The economic cost of metabolic syndrome and its associated complications has a significant effect on health care budgets. Improvements in body weight, blood lipid profile, and hyperglycemia can reduce cardiometabolic risk. However, constant hyperadrenergic stimulation still contributes to the burden of disease. Normalization of the hyperdynamic circulatory state with conventional therapies is the most reasonable therapeutic strategy to date. JTV519 (K201) is a newly developed 1,4-benzothiazepine drug with antiarrhythmic and cardioprotective properties. It appears to be very effective in not only preventing but also in reversing the characteristic myocardial changes and preventing lethal arrhythmias. It is also a unique candidate to improve diastolic heart failure in metabolic syndrome. PMID:22563249

Dincer, U Deniz

2012-01-01

115

The effect of biofeedback therapy on dyssynergic constipation in patients with or without Irritable Bowel Syndrome  

PubMed Central

Background: The Rome II and III diagnostic criteria for dyssynergic defecation recommended the exclusion of irritable bowel syndrome (IBS). This study determined the effect of biofeedback therapy on dyssynergic constipation in patients with or without IBS. Materials and Methods: This study was a nonrandomized, single blinded, semi experimental study. Dyssynergic defecation patients with and without IBS were asked to undergo biofeedback therapy 8 sessions. The defecation dynamics and balloon expulsion time were evaluated before, at the end and 1 month after the biofeedback therapy. IBS symptoms were graded using a 4-point Likert scale. Mann–Whitney U-test, Wilcoxon test and Friedman test were applied to analyze data using SPSS software package (SPSS Inc., Chicago, IL, USA). Results: After the biofeedback therapy, the IBS symptoms have been decreased significantly (the median of 2 before and 1 after therapy, P < 0.01). The biofeedback therapy significantly decreased the anismus index in IBS group by the mean of 0.75 ± 0.31, 0.28 ± 0.07 and 0.28 ± 0.06 in three phases, respectively. Similar results were found in non-IBS patients (the mean of 0.74 ± 0.32, 0.28 ± 0.08, 0.27 ± 0.08 in three phases, respectively). The symptoms of constipation (sensation of incomplete evacuation, difficult and painful defecation), defecation facilitative manual maneuver frequency, pelvic floor muscles resting amplitude and strain amplitude decreased and squeezing amplitude improved significantly after biofeedback therapy in both groups with and without IBS (P < 0.001). There were not significant differences between patients with and without IBS (P > 0.05) with respect to outcome. No complication was observed in treatment groups. Conclusion: Dyssynergic constipation patients with and without IBS will likely benefit from biofeedback therapy. PMID:25538778

Ahadi, Tannaz; Madjlesi, Faezeh; Mahjoubi, Bahar; Mirzaei, Rezvan; Forogh, Bijan; Daliri, Seyedeh Somayeh; Derakhshandeh, Seyed Majid; Behbahani, Roxana Bazaz; Raissi, G. Reza

2014-01-01

116

[Clinical nutrition therapy in patients with short bowel syndrome in line with principles of personalized medicine].  

PubMed

Home parenteral nutrition administered in selected care centres has been financed in Hungary since January, 2013. The authors discuss diagnostic issues, treatment and nutrition therapy of short bowel syndrome patients in line with the principles of personalised medicine. The most severe form of short bowel syndrome occurs in patients having jejunostomy, whose treatment is discussed separately. The authors give a detailed overview of home parenteral feeding, its possible complications, outcomes and adaptation of the remaining bowel. They describe how their own care centre operates where they administer home parenteral nutrition to 12 patients with short bowel syndrome (5 females and 7 males aged 51.25±14.4 years). The body mass index was 19.07±5.08 kg/m2 and 20.87±3.3 kg/m2, skeletal muscle mass was 25.7±6.3 kg and 26.45±5.38 kg, and body fat mass was 14.25±8.55 kg and 11.77±2.71 kg at the start of home parenteral nutrition and presently, respectively. The underlying conditions of short bowel syndrome were tumours in 4 patients, bowel ischaemia in four patients, surgical complications in three patients, Crohn's disease in one patient, and Crohn's disease plus tumour in one patient. Orv. Hetil., 2014, 155(51), 2054-2062. PMID:25497156

Sahin, Péter; Molnár, Andrea; Varga, Mária; Bíró, Ilona; K?míves, Csilla; Fejér, Csaba; Futó, Judit; Tomsits, Erika; Topa, Lajos

2014-12-01

117

Transient gene therapy to treat cutaneous radiation syndrome: development in a minipig model.  

PubMed

Cutaneous radiation syndrome is the delayed consequence of localized skin exposure to high doses of ionizing radiation. Adipocyte derived stem cells injection may improve tissue regeneration through secreted factors. Thus mesenchymal stem cells secretome optimization, using transient transfection, may represent a new strategy to treat this syndrome. Sonic hedgehog, a secreted protein involved in cell proliferation and angiogenesis, has been chosen as a first candidate. Here preliminary results are reported of the therapeutic potential of transient gene therapy to cure cutaneous radiation syndrome in a minipig model. Adipocyte derived stem cells were transiently transfected by electroporation with a plasmid coding for Sonic Hedgehog. Göttingen minipigs were locally irradiated using a (60)Co gamma source at the dose of 50 Gy and received Phosphate Buffer Salin (controls: n = 8), stem cells (50 × 10? each time, n = 5) or transfected stem cells (25±7 × 10? each time, n = 1). All controls exhibited a homogeneous clinical evolution of cutaneous radiation syndrome with final necrosis (day 91). In stem cell injected minipigs, an ultimate wound healing was observed in four out of five grafted animals (day 130 ± 28, complete in two of them) (historical results). The Sonic hedgehog animal, albeit injected with a lower number of transfected stem cells, presented a very similar evolution of skin healing without necrosis or uncontrolble pain. Globally this preliminary report suggests that local injection of Sonic Hedgehog transfected adipocyte derived stem cells may improve wound healing. Thus work is ongoing to evaluate this therapeutic strategy on a larger number of animals. PMID:24776904

Riccobono, Diane; Forcheron, Fabien; Agay, Diane; Scherthan, Harry; Meineke, Viktor; Drouet, Michel

2014-06-01

118

State of the art in restless legs syndrome therapy: practice recommendations for treating restless legs syndrome.  

PubMed

Dopaminergic agents are the best-studied agents and are considered first-line treatment of restless legs syndrome (RLS). Extensive data are available for levodopa, pramipexole, and ropinirole, which have approval for the indication RLS, and to a smaller extent for cabergoline, pergolide, and rotigotine. Apart from one recent study, comparing two active drugs (levodopa and cabergoline), no comparative studies have been published. The individual treatment regimen with the most appropriate agent concerning efficacy and side effects has to be selected by the treating physician. On the basis of these clinical trials and expert opinion of the authors, a treatment algorithm is proposed to support the search for the optimal individual treatment. Opioids and anticonvulsants such as gabapentine are second-line options in individual patients. Iron substitution is justified in people with iron deficiency related RLS (ferritin concentration lower than 50 microg/L). PMID:17516455

Oertel, Wolfgang H; Trenkwalder, Claudia; Zucconi, Marco; Benes, Heike; Borreguero, Diego Garcia; Bassetti, Claudio; Partinen, Markku; Ferini-Strambi, Luigi; Stiasny-Kolster, Karin

2007-01-01

119

Physical Therapy and Rehabilitation of Complex Regional Pain Syndrome in Shoulder Prosthesis  

PubMed Central

We report a 66-year-old woman with complex regional pain syndrome (CRPS) 1 treated with combined medical and active physical therapy. She was diagnosed with CRPS 1 following partial shoulder prosthesis due to proximal humerus fracture. Despite continuous medication and physical therapy, there was no improvement in her pain and functional outcome. Her overall pain was decresed by stellate ganglion block 3 times in two weeks conducted during the second month of the follow-up period. Following the ganglion blockades, pain and the other symptoms were decreased intermittently but range of motion (ROM) and functional status were not satisfied as much as expected. After the third month of follow-up, her passive and active ROM of the shoulder joint was increased after application of manipulation under general anesthesia. In conclusion, because CRPS 1 remains one of the most difficult pain syndromes, early diagnosis and treatment are important to have adequate functional results from physical therapy. Manipulation under general anesthesia may be an additional effective treatment tool to obtain functional improvement in some patients diagnosed with CRPS 1. PMID:21217890

Demirhan, Mehmet

2010-01-01

120

Managing Cytokine Release Syndrome Associated With Novel T Cell-Engaging Therapies  

PubMed Central

Chimeric antigen receptor (CAR)-modified T cells and bispecific T cell–engaging antibodies have demonstrated dramatic clinical responses in recent clinical trials. The hallmark of these novel highly active immunotherapies is nonphysiologic T cell activation, which has correlated not only with greatly increased efficacy but also with notable toxicity in some cases. We and others have observed a cytokine release syndrome (CRS), which correlates with both toxicity and efficacy in patients receiving T cell–engaging therapies. In addition to elevations in effector cytokines, such as interferon-?, cytokines associated with hemophagocytic lymphohistiocytosis or macrophage activation syndrome, such as interleukin (IL)-10 and IL-6, may also be markedly elevated. Whereas corticosteroids may control some of these toxicities, their potential to block T cell activation and abrogate clinical benefit is a concern. Detailed studies of T cell proliferation and the resultant immune activation produced by these novel therapies have led to more targeted approaches that have the potential to provide superior toxicity control without compromising efficacy. One approach we have developed targets IL-6, a prominent cytokine in CRS, using the IL-6R antagonist tocilizumab. We will review the pathophysiology and management options for CRS associated with T cell–engaging therapies. PMID:24667956

Maude, Shannon L.; Barrett, David; Teachey, David T.; Grupp, Stephan A.

2014-01-01

121

Plummer-Vinson syndrome and dilation therapy: a report of two cases.  

PubMed

Plummer-Vinson syndrome is known as the association of postcricoid dysphagia, upper esophageal web, and iron deficiency anemia. Although correction of iron deficiency may result in resolution of dysphagia and sometimes disappearance of the webs, dilation therapy is usually necessary to remove webs and relieve dysphagia. We report two cases of Plummer-Vinson syndrome. Both patients presented with significant and longstanding dysphagia, sideropenia, glossitis and koilonychia. Our two patients had occasional choking and aspiration episodes at eating and endoscope did not pass through at the level of the upper esophagus. Patients' esophagograms revealed the presence of webs in part of the post-cricoid region. Both patients were treated with esophageal bougienage or balloon dilation, and iron supplementation. The patients were examined periodically for two years after the initial treatment and found to be in good general condition. PMID:16547853

Demirci, Fikret; Sava?, M Cemil; Kepkep, Necip; Okan, Vahap; Yilmaz, Mehmet; Büyükberber, Mehmet; Gül?en, M Taner

2005-12-01

122

Use of Early Inhaled Nitric Oxide Therapy in Fat Embolism Syndrome to Prevent Right Heart Failure  

PubMed Central

Fat embolism syndrome (FES) is a life-threatening condition in which multiorgan dysfunction manifests 48–72 hours after long bone or pelvis fractures. Right ventricular (RV) failure, especially in the setting of pulmonary hypertension, is a frequent feature of FES. We report our experience treating 2 young, previously healthy trauma patients who developed severe hypoxemia in the setting of FES. Neither patient had evidence of RV dysfunction on echocardiogram. The patients were treated with inhaled nitric oxide (NO), and their oxygenation significantly improved over the subsequent few days. Neither patient developed any cardiovascular compromise. Patients with FES that have severe hypoxemia and evidence of adult respiratory distress syndrome (ARDS) are likely at risk for developing RV failure. We recommend that these patients with FES and severe refractory hypoxemia should be treated with inhaled NO therapy prior to the onset of RV dysfunction. PMID:25180103

Koyfman, Leonid; Kutz, Ruslan; Frenkel, Amit; Gruenbaum, Shaun E.; Zlotnik, Alexander; Klein, Moti

2014-01-01

123

Cognitive-behavioral therapy for the management of irritable bowel syndrome  

PubMed Central

Irritable bowel syndrome (IBS) is a common disorder, reported to be found in 5%-20% of the general population. Its management accounts for up to 25% of a gastroenterologist’s workload in the outpatient department, and the main symptoms are abdominal pain, bloating, and altered bowel habits. Despite a great amount of available pharmacological treatments aimed at a wide variety of gastrointestinal and brain targets, many patients have not shown adequate symptom relief. In recent years, there has been increasing evidence to suggest that psychological treatments, in particular cognitive-behavioral therapy (CBT), are effective for the management of IBS. This review discusses CBT for the management of IBS. CBT has proved to be effective in alleviating the physical and psychological symptoms of IBS and has thus been recommended as a treatment option for the syndrome. PMID:24379577

Tang, Qing-Lin; Lin, Guo-Yao; Zhang, Ming-Qing

2013-01-01

124

Eculizumab Therapy Leads to Rapid Resolution of Thrombocytopenia in Atypical Hemolytic Uremic Syndrome  

PubMed Central

Eculizumab is highly effective in controlling complement activation in patients with the atypical hemolytic uremic syndrome (aHUS). However, the course of responses to the treatment is not well understood. We reviewed the responses to eculizumab therapy for aHUS. The results show that, in patients with aHUS, eculizumab therapy, when not accompanied with concurrent plasma exchange therapy, led to steady increase in the platelet count and improvement in extra-renal complications within 3 days. By day 7, the platelet count was normal in 15 of 17 cases. The resolution of hemolytic anemia and improvement in renal function were less predictable and were not apparent for weeks to months in two patients. The swift response in the platelet counts was only observed in one of five cases who received concurrent plasma exchange therapy and was not observed in a case of TMA due to gemcitabine/carboplatin. In summary, eculizumab leads to rapid increase in the platelet counts and resolution of extrarenal symptoms in patients with aHUS. Concurrent plasma exchange greatly impedes the response of aHUS to eculizumab therapy. Eculizumab is ineffective for gemcitabine/carboplatin associated TMA. PMID:25400666

Kuo, Elizabeth

2014-01-01

125

Delayed Onset of Acute Limb Compartment Syndrome With Neuropathy After Venoarterial Extracorporeal Membrane Oxygenation Therapy  

PubMed Central

Acute limb compartment syndrome (ALCS) is defined as compound symptoms resulting from poor oxygenation and decreased nutrition supply to muscles and nerves in a tightly confined compartment. The most common cause of ALCS is tibia fracture, followed by blunt trauma to soft tissue. However, non-traumatic causes are rare. We report an iatrogenic, non-traumatic ALCS case after venoarterial extracorporeal membrane oxygen (VA-ECMO) therapy. A 14-year-old male received VA-ECMO therapy due to cardiorespiratory failure after drowning. Although he had no symptoms during therapy, leg swelling appeared 10 hours after ECMO treatment. Two days after the leg swelling, the patient underwent a fasciotomy. Unfortunately, nerve conduction studies and electromyography showed multiple neuropathies in the lower leg. Despite 2 weeks of rehabilitation with electrical stimulation, an exercise program, and physical therapy, there was no definite change in muscle strength. To our knowledge, this is the first reported case of non-traumatic ALCS after VA-ECMO therapy in Korea. PMID:25229039

Go, Jin Young; Min, Yu-Sun

2014-01-01

126

Analysis of Factors Associated With Radiation-Induced Bronchiolitis Obliterans Organizing Pneumonia Syndrome After Breast-Conserving Therapy  

SciTech Connect

Purpose: To evaluate factors associated with radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome after breast-conserving therapy. Methods and Materials: A total of 702 women with breast cancer who received radiotherapy after breast-conserving surgery at seven institutions between July 1995 and December 2006 were analyzed. In all patients, the whole breast was irradiated with two tangential photon beams. The criteria used for the diagnosis of radiation-induced BOOP syndrome were as follows: (1) radiotherapy to the breast within 12 months, (2) general and/or respiratory symptoms lasting for {>=}2 weeks, (3) radiographs showing lung infiltration outside the radiation port, and (4) no evidence of a specific cause. Results: Radiation-induced BOOP syndrome was seen in 16 patients (2.3%). Eleven patients (68.8%) were administered steroids. The duration of steroid administration ranged from 1 week to 3.7 years (median, 1.1 years). Multivariate analysis revealed that age ({>=}50 years; odds ratio [OR] 8.88; 95% confidence interval [CI] 1.16-67.76; p = 0.04) and concurrent endocrine therapy (OR 3.05; 95% CI 1.09-8.54; p = 0.03) were significantly associated with BOOP syndrome. Of the 161 patients whose age was {>=}50 years and who received concurrent endocrine therapy, 10 (6.2%) developed BOOP syndrome. Conclusions: Age ({>=}50 years) and concurrent endocrine therapy can promote the development of radiation-induced BOOP syndrome after breast-conserving therapy. Physicians should carefully follow patients who received breast-conserving therapy, especially those who are older than 50 years and received concurrent endocrine therapy during radiotherapy.

Katayama, Norihisa [Department of Radiology, Okayama University Hospital, Okayama (Japan)], E-mail: n-katayama@bea.hi-ho.ne.jp; Sato, Shuhei [Department of Radiology, Okayama University Hospital, Okayama (Japan); Katsui, Kuniaki [Department of Radiology, Fukuyama City Hospital, Fukuyama (Japan); Takemoto, Mitsuhiro [Department of Radiology, Okayama University Hospital, Okayama (Japan); Tsuda, Toshihide [Department of Environmental Epidemiology, Graduate School of Environmental Science, Okayama University Graduate School, Okayama (Japan); Yoshida, Atsushi [Department of Radiology, Kure Kyosai Hospital, Kure (Japan); Morito, Tsuneharu [Department of Radiology, Okayama Saiseikai General Hospital, Okayama (Japan); Nakagawa, Tomio [Department of Radiology, NHO Fukuyama Medical Center, Fukuyama (Japan); Mizuta, Akifumi [Department of Radiology, Chugoku Central Hospital, Fukuyama (Japan); Waki, Takahiro; Niiya, Harutaka [Department of Radiology, NHO Okayama Medical Center, Okayama (Japan); Kanazawa, Susumu [Department of Radiology, Okayama University Hospital, Okayama (Japan)

2009-03-15

127

Clonidine as an Adjunct Therapy to Opioids for Neonatal Abstinence Syndrome: A Randomized, Controlled Trial  

PubMed Central

Objective To determine if oral clonidine would reduce the duration of opioid detoxification for neonatal abstinence syndrome. Methods Infants with intrauterine exposure to methadone or heroin and neonatal abstinence syndrome (2 consecutive modified Finnegan scores of ?9) were enrolled at 2 hospitals during 2002–2005 and followed until final hospital discharge. All enrolled infants (80) received oral diluted tincture of opium according to a standardized algorithm and were randomly assigned to receive oral clonidine (1 ?g/kg every 4 hours) (40 infants) or placebo (40 infants). Primary outcome was duration of opioid therapy. Secondary outcomes included the amount of opium required to control symptoms, number of treatment failures, and differences in blood pressure, heart rate, and oxygen saturation. Results The median length of therapy was 27% shorter in the clonidine group (11 [95% confidence interval: 8–15 days]) than in the placebo group (15 days [95% confidence interval: 12–17 days]). In the clonidine group, 7 infants required restarting opium after initial discontinuation versus none in the placebo group, with the total length of treatment/observation remaining significantly less in the clonidine group. Higher dosages of opium were required by 40% of the infants in the placebo group versus 20% in the clonidine group. Treatment failures occurred in 12.5% of the infants in the placebo group versus none in the clonidine group. Hypertension, hypotension, bradycardia, or desaturations did not occur in either group. Three infants in the clonidine group died as a result of myocarditis, sudden infant death syndrome, and homicide, all after hospital discharge and before 6 months of age. Conclusions In this randomized, double-blind trial, adding clonidine to standard opioid therapy for detoxification from in utero exposure to methadone or heroin reduced the duration of pharmacotherapy for neonatal abstinence without causing short-term adverse cardiovascular outcomes. A larger trial is indicated to determine long-term safety. PMID:19398463

Agthe, Alexander G.; Kim, George R.; Mathias, Kay B.; Hendrix, Craig W.; Chavez-Valdez, Raul; Jansson, Lauren; Lewis, Tamorah R.; Yaster, Myron; Gauda, Estelle B.

2009-01-01

128

Long-Term Therapy with Lenalidomide in a Patient with POEMS Syndrome  

PubMed Central

Lenalidomide is an effective therapy against malignant plasma cells and a potent agent against proinflammatory and proangiogenic cytokines. The use of lenalidomide in POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein with plasma cells, skin changes) has been reported, but its benefit in long-term use is not well established. A 55-year-old man with POEMS and debilitating polyneuropathy was treated with lenalidomide and dexamethasone followed by maintenance lenalidomide. He remains in haematologic remission and in complete recovery of functional status 3.5 years after diagnosis. This case supports the long-term use of lenalidomide in patients with POEMS syndrome.

Chu, Benjamin F.; Shana’ah, Arwa; Hofmeister, Craig C.; Benson, Don M.; Sell, Megan; Tucker, Jill; Pichiorri, Flavia; Efebera, Yvonne A.

2014-01-01

129

Immune reconstitution inflammatory syndrome after highly active antiretroviral therapy: a review.  

PubMed

The use of highly active antiretroviral therapy (HAART) in the management of human immunodeficiency virus (HIV) restores immune responses against pathogens and has greatly decreased mortality. However, in about 25% to 35% of patients receiving HAART, the reconstituted immune system leads to a pathological inflammatory response, commonly known as immune reconstitution inflammatory syndrome (IRIS), which causes substantial short-term morbidity or even mortality. Although we have gleaned some knowledge on IRIS in the past few years, a number of unanswered questions remain. In this review, we discuss the definition, diagnostic criteria, pathogenesis, risk factors, clinical spectrum including oral manifestations, and management of IRIS. PMID:19758406

Tsang, C S P; Samaranayake, L P

2010-04-01

130

Treating metabolic syndrome's metaflammation with low level light therapy: preliminary results  

NASA Astrophysics Data System (ADS)

Metabolic syndrome comprises a constellation of morbidities such as insulin resistance, hyperinsulinemia, atherogenic dyslipidemia, dysglycemia and obesity (especially abdominal). Metabolic alterations are observed in major insulin target organs, increasing the risk of cardiovascular diseases, type-2 diabetes and therefore mortality. Tissue alterations are characterized by immune cells infiltrates (especially activated macrophages). Released inflammatory mediators such as TNF-? induce chronic inflammation in subjects with metabolic syndrome, since inflammatory pathways are activated in the neighboring cells. The intra-abdominal adipose tissue appears to be of particular importance in the onset of the inflammatory state, and strategies contributing to modulate the inflammatory process within this adipose tissue can mitigate the metabolic syndrome consequences. Considering the low level light therapy (LLLT) recognized benefits in inflammatory conditions, we hypothesized this therapeutic approach could promote positive effects in modulating the inflammatory state of metabolic syndrome. That being the scope of this study, male C57BL/6 mice were submitted to a high-fat/high-fructose diet among 8 weeks to induce metabolic syndrome. Animals were then irradiated on the abdominal region during 21 days using an 850 nm LED (6 sessions, 300 seconds per session, 60 mW output power, ~6 J/cm2 fluence, ~19 mW/cm2 fluence rate). Before and during treatment, blood was sampled either from the retroorbital plexus or from tail puncture for glucose, total cholesterol and triglycerides analysis. So far our results indicate no alterations on these metabolic parameters after LLLT. For further investigations, blood was collected for plasma inflammatory cytokine quantification and fresh ex vivo samples of liver and intra-abdominal adipose tissue were harvested for immunohistochemistry purposes.

Yoshimura, Tania M.; Kato, Ilka T.; Deana, Alessandro M.; Ribeiro, Martha S.

2014-02-01

131

Visceral fat as target of highly active antiretroviral therapy-associated metabolic syndrome.  

PubMed

HIV-associated lipodystrophy or lipoatrophy, unreported before the introduction of highly active antiretroviral therapy (HAART), was first described in 1998, and has a prevalence ranging from 18% to 83%. As in genetic lipodystrophy syndromes, fat redistribution may precede the development of metabolic complications (dyslipidemia, insulin resistance) in HIV-infected patients receiving HAART. The pathogenesis of HAART-associated lipodystrophy and metabolic syndrome is complex and a number of factors are involved, including direct effects of HAART on lipid metabolism, endothelial and adipocyte cell function, and mitochondria. Protease inhibitors are responsible for a decrease in cytoplasmic retinoic-acid protein-1, in low density lipoprotein-receptor-related protein and in peroxisome proliferator activated receptor type-gamma. Nucleoside reverse transcriptase inhibitors, and thymidine analogues, are responsible for mitochondrial dysfunction as demonstrated by a decrease in subcutaneous adipose tissue mitochondrial DNA content. Both phenomena are responsible for a decreased differentiation of adipocytes, increased levels of free fatty acids and lipoatrophy. The increased levels of proinflammatory cytokines, such as tumor necrosis factor (TNF)-alpha and interleukin-6 may further contribute in development of lipodystrophy. TNF-alfa activates 11-beta-hydroxysteroid dehydrogenase type-1, which converts inactive cortisone to active cortisol, resulting in increased lipid accumulation in adipocytes and insulin resistance. HAART drugs and inflammatory cytokines are associated with a decrease in adiponectin. The levels of adiponectin and adiponectin-to-leptin ratio correlate positively with insulin resistance in HIV-infected patients with lipodystrophy. HAART-associated metabolic syndrome is an increasingly recognized clinical entity. The atherogenic profile of this syndrome may increase the risk of cardiovascular disease even in young HIV-infected patients. A better understanding of the molecular mechanisms responsible for this syndrome will lead to the discovery of new drugs that will reduce the incidence of lipodystrophy and related metabolic complications in HIV-infected patients receiving HAART. PMID:17627554

Barbaro, Giuseppe

2007-01-01

132

NTOS symptoms and mobility: a case study on neurogenic thoracic outlet syndrome involving massage therapy.  

PubMed

Neurogenic thoracic outlet syndrome (NTOS) is a neuromuscular condition affecting brachial plexus functionality. NTOS is characterized by paresthesia, pain, muscle fatigue, and restricted mobility in the upper extremity. This study quantified massage therapy's possible contribution to treatment of NTOS. A 24-year-old female with NTOS received eight treatments over 35 days. Treatment included myofascial release, trigger point therapy, cross fiber friction, muscle stripping, and gentle passive stretching. Abduction and lateral rotation at the glenohumeral (GH joint) assessments measured range of motion (ROM). A resisted muscle test evaluated upper extremity strength. The client rated symptoms daily via a visual analog scale (VAS). Findings showed improvement in ROM at the GH joint. VAS ratings revealed a reduction in muscle weakness, pain, numbness, and 'paresthesia'. Results suggest massage may be useful as part of a broad approach to managing NTOS symptoms and improving mobility. PMID:24411148

Streit, Robin S

2014-01-01

133

Bridging therapy in antiphospholipid syndrome and antiphospholipid antibodies carriers: case series and review of the literature.  

PubMed

Peri-operative management of patients on warfarin involves assessing and balancing individual risks for thromboembolism and bleeding. The timing of warfarin withdrawal and a tailored pre/postoperative management (including the substitution of heparin in place of warfarin, the so-called bridging therapy) is critical in patients with prothrombotic conditions. The antiphospholipid syndrome (APS) is the most common cause of acquired thrombophilia. In this particular subset of patients, as the risk of thrombosis is higher than in general population, bridging therapy can represent a real challenge for treating physicians. Only few studies have been designed to address this topic. We aim to report our experience and to review the available literature in the peri-procedural management of APS and antiphospholipid antibody-positive patients, reporting adverse events and attempting to identify potential risk factor associated with thrombosis or bleeding complications. PMID:25242343

Raso, Samuele; Sciascia, Savino; Kuzenko, Anna; Castagno, Irene; Marozio, Luca; Bertero, Maria Tiziana

2015-01-01

134

Multimodal therapy for category III chronic prostatitis/chronic pelvic pain syndrome in UPOINTS phenotyped patients  

PubMed Central

The complex network of etiological factors, signals and tissue responses involved in chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) cannot be successfully targeted by a single therapeutic agent. Multimodal approaches to the therapy of CP/CPPS have been and are currently being tested, as in the frame of complex diagnostic-therapeutic phenotypic approaches such as the urinary, psychosocial, organ-specific, infection, neurological and muscle tenderness (UPOINTS) system. In this study, the effect of combination therapy on 914 patients diagnosed, phenotyped and treated in a single specialized prostatitis clinic was analyzed. Patients received ?-blockers, Serenoa repens (S. repens) extracts combined or not with supplements (lycopene and selenium) and, in the presence of documented or highly suspected infection, antibacterial agents. Combination treatment induced marked and significant improvements of National Institutes of Health Chronic Prostatitis Symptom Index (NIH-CPSI) prostatitis symptom scores, International Index of Erectile Function (IIEF) sexual dysfunction scores, urinary peak flow rates and bladder voiding efficiency. These improvements, assessed after a 6-month course of therapy, were sustained throughout a follow-up period of 18 months. A clinically appreciable reduction of ?6 points of the total NIH-CPSI score was achieved in 77.5% of patients subjected to combination therapy for a period of 6 months. When the patients were divided in two cohorts, depending on the diagnosis of CP/CPPS [inflammatory (IIIa) vs. non-inflammatory (IIIb) subtypes], significant improvements of all signs and symptoms of the syndrome were observed in both cohorts at the end of therapy. Intergroup comparison showed that patients affected by the IIIa sub-category of CP/CPPS showed more severe signs and symptoms (NIH-CPSI total, pain and quality of life impact scores, and Qmax) at baseline when compared with IIIb patients. However, the improvement of symptoms after therapy was significantly more pronounced in IIIa patients when compared with IIIb patients. In contrast to current opinion, the evidence emerging from the present investigation suggests that the inflammatory and non-inflammatory sub-categories of CP/CPPS may represent two distinct pathological conditions or, alternatively, two different stages of the same condition. In conclusion, a simple protocol based on ?-blockers, S. repens extracts and supplements and antibacterial agents, targeting the urinary, organ specific and infection domains of UPOINTS, may induce a clinically appreciable improvement of the signs and symptoms of CP/CPPS in a considerable percentage of patients. In patients not responding sufficiently to such therapy, second-line agents (antidepressants, anxiolytics, muscle relaxants, 5-phosphodiesterase inhibitors and others) may be administered in order to achieve a satisfactory therapeutic response.

MAGRI, VITTORIO; MARRAS, EMANUELA; RESTELLI, ANTONELLA; WAGENLEHNER, FLORIAN M.E.; PERLETTI, GIANPAOLO

2015-01-01

135

Nephrotic syndrome due to membranous nephropathy associated with metastatic prostate cancer: rapid remission after initial endocrine therapy.  

PubMed

A case of severe nephrotic syndrome (urinary protein excretion 12.9 g/day) due to membranous nephropathy associated with untreated prostate cancer and multiple bone metastases is described. A combination of initial endocrine treatment and steroid therapy resulted in normalization of prostate-specific antigen levels followed by a rapid decrease of urinary protein excretion within 4 months. No proteinuria was subsequently detected. Seven months after the initiation of therapy, the patient remained well with complete clinical remission from the nephrotic syndrome. This rapid achievement of remission may have been due to tumor shrinkage by androgen ablation in addition to steroid therapy of the membranous nephropathy. The nephrotic syndrome is a rare complication of prostate cancer, and, to the best of our knowledge, no previous cases have been reported of membranous nephropathy as one of the first disease manifestations. PMID:10644912

Matsuura, H; Sakurai, M; Arima, K

2000-01-01

136

Opsoclonus-myoclonus in an HIV-infected child on antiretroviral therapy—possible immune reconstitution inflammatory syndrome  

Microsoft Academic Search

The exact immunopathogenesis and neuroanatomical localization of opsoclonus-myoclonus ataxia syndrome remains unclear. We describe a 1 year 9 month old girl who, shortly after commencement of highly active antiretroviral therapy developed opsoclonus-myoclonus syndrome and subsequently died of disseminated cytomegalovirus infection. We postulate on the etiological factors that may have played a role in the disease pathogenesis of the patient's opsoclonus-myoclonus

Ronald van Toorn; Helena Rabie; James M. Warwick

2005-01-01

137

Case Report: The Effects of Massage Therapy on a Woman with Thoracic Outlet Syndrome  

PubMed Central

Introduction Thoracic outlet syndrome (TOS) refers to a group of conditions resulting from compression of the neurovascular structures of the thoracic outlet. The parameters for physical therapy include myofascial release (MFR), neuromuscular therapy (NMT), muscle strengthening, and stretching. This case study examined the effects of neuromuscular therapy, massage, and other manual therapies on a 56-year-old female presenting with bilateral numbness over the forearms and hands on waking. Numbness occurred most days, progressing to “dead rubbery” forearms and hands once or twice a month. Methods The treatment plan was implemented over eight weeks and consisted of six, 50-minute bodywork sessions. Several nonbodywork strategies were also employed to address potential contributing factors to the TOS symptomology experienced by the client. Objective measurements included posture analysis (PA), range of movement (ROM), and Roos and Adson’s tests. The Measure Your Own Medical Outcome Profile (MYMOP2), a client-generated measure of clinical outcome, was used to measure clinical change. Results MYMOP2 overall profile score results demonstrated an improvement of 2.25 from pretreatment to post-treatment measurement. Clinically meaningful change was measured by the individual and was indicative of substantial symptom improvement where a score change of over one was considered as meaningful. Conclusions A course of massage was effective for numbness symptoms in an individual with TOS, and results lasted over a year without additional treatments. Further research is needed to fully understand the effects of massage for TOS symptoms. PMID:25452819

Wakefield, Mary Lillias

2014-01-01

138

Effective prophylactic therapy for cyclic vomiting syndrome in children using valproate.  

PubMed

This trial sought to evaluate our experience using the antimigraine prophylactic drug, use of valproate for the prophylactic management of cyclic vomiting syndrome (CVS) in children. Thirteen children diagnosed with severe CVS were enrolled. Prophylactic therapy consisted of valproate administered at a dose of 10-40 mg/kg/day. Upon enrollment in the study, all patients underwent diagnostic tests to rule out organic causes of their symptoms. Vomiting was severe enough in all patients to cause dehydration requiring hospitalization for intravenous rehydration. Nine of 13 patients did not respond to numerous previous medical therapies like propranolol, amitriptyline, cyproheptadine, phenobarbital, phenytoin, and carbamazepine. Three of 13 patients required combination therapy with valproate and phenobarbital. Of the 13 patients, two showed complete resolution of their symptoms, nine had marked improvement in their symptoms, as evidenced by infrequent attacks of reduced severity, and two failed to respond to valproate therapy. Four patients experienced relapse with a decreased dosage of valproate. Side effects associated with long-term valproate administration were not observed. Valproate appears to be effective for the prophylactic management of severe CVS, with 85% of all patients achieving at least a reduction in the frequency of attacks. PMID:18752910

Hikita, Toshiyuki; Kodama, Hiroko; Nakamoto, Natsue; Kaga, Fumiaya; Amakata, Kaori; Ogita, Kaori; Kaneko, Sono; Fujii, Yasushi; Yanagawa, Yukishige

2009-06-01

139

Role of adjunctive medical therapy in the fetoscopic surgical treatment of twin-twin transfusion syndrome.  

PubMed

The underlying etiology of twin-twin transfusion syndrome (TTTS) is unknown, but our growing understanding of the cardiovascular features of TTTS suggests this may be a disease that could respond to transplacental medical therapy. Adjunctive medical therapy in TTTS with the calcium channel blocker nifedipine has been shown to improve recipient survival while having no effect on the donor. There is no significant difference in recipient survival from postoperative day 5 to birth suggesting that the survival benefit is confined to the effects of nifedipine in the perioperative period. Also, there is no significant effect of nifedipine on gestational age at delivery suggesting the survival benefit was unrelated to the tocolytic effects of nifedipine and more likely a result of hemodynamic effects in the recipient twins' cardiovascular system during the perioperative period. TTTS remains poorly understood but there appears to be good evidence suggesting twin-twin hypertensive cardiomyopathy is a large component of the pathophysiology in recipient twins. The initial findings of nifedipine's effectiveness as a targeted medical therapy to address TTTS cardiomyopathy and improve survival of recipient twins opens the door for further research for adjunctive medical therapies in TTTS. PMID:25077476

Zaretsky, Michael V; Somme, Stig; Crombleholme, Timothy M

2014-09-01

140

The acute cardiorenal syndrome type I: considerations on physiology, epidemiology, and therapy.  

PubMed

Acute cardiorenal syndrome, also known as cardiorenal syndrome type 1, is defined as an abrupt worsening of cardiac function that occurs in at least 30 % of patients with acute decompensated heart failure and can lead to the development of acute kidney injury. The changes in renal function that occur in this setting have variable prognostic implications, as both poorer and better outcomes have been reported when renal function worsens during treatment of heart failure decompensation. Furthermore, it remains unclear when worsening renal function is actually a manifestation of true acute kidney injury or simply an indicator of hemoconcentration. Given these gaps in the understanding of the significance of renal function changes in the setting of decompensated heart failure, it is not surprising that studies on the effects of available therapies, including diuretics, vasoactive drugs, and mechanical fluid removal have yielded inconsistent results. The purpose of this review is to analyze critically the current knowledge on the pathophysiology, epidemiology, prognosis, and treatment of acute cardiorenal syndrome. PMID:25224320

Valika, Ali A; Costanzo, Maria Rosa

2014-12-01

141

Ponatinib as targeted therapy for FGFR1 fusions associated with the 8p11 myeloproliferative syndrome  

PubMed Central

The 8p11 myeloproliferative syndrome is a rare, aggressive myeloproliferative neoplasm characterized by constitutively active FGFR1 fusion proteins that arise from specific chromosomal translocations and which drive aberrant proliferation. Although FGFR1 inhibitors have shown in vitro activity against FGFR1 fusions, none are in use clinically and there is a need to assess additional compounds as potential therapy. Here we use cell lines and primary cells to investigate ponatinib (AP24534). Ponatinib-treated Ba/F3 cells transformed by ZMYM2-FGFR1 and BCR-FGFR1 and the FGFR1OP2-FGFR1 positive KG1A cell line showed reduced proliferation and decreased survival when compared to control cells. Inhibition induced apoptosis and reduced phosphorylation of the FGFR1 fusion proteins and substrates. Ponatinib-treated cells from 8p11 myeloproliferative syndrome patients (n=5) showed reduced colony growth compared to controls. In one evaluable patient, ponatinib specifically reduced numbers of FGFR1-fusion gene positive colonies. Ponatinib, therefore, shows considerable promise for the treatment of patients with 8p11 myeloproliferative syndrome. PMID:22875613

Chase, Andrew; Bryant, Catherine; Score, Joannah; Cross, Nicholas C.P.

2013-01-01

142

Immuno-therapy of Acute Radiation Syndromes : Extracorporeal Immuno-Lympho-Plasmo-Sorption.  

NASA Astrophysics Data System (ADS)

Methods Results Summary and conclusions Introduction: Existing Medical Management of the Acute Radiation Syndromes (ARS) does not include methods of specific immunotherapy and active detoxication. Though the Acute Radiation Syndromes were defined as an acute toxic poisonous with development of pathological processes: Systemic Inflammatory Response Syndrome (SIRS), Toxic Multiple Organ Injury (TMOI), Toxic Multiple Organ Dysfunction Syndrome(TMODS), Toxic Multiple Organ Failure (TMOF). Radiation Toxins of SRD Group play an important role as the trigger mechanisms in development of the ARS clinical symptoms. Methods: Immuno-Lympho-Plasmo-Sorption is a type of Immuno-therapy which includes prin-ciples of immunochromato-graphy, plasmopheresis, and hemodialysis. Specific Antiradiation Antitoxic Antibodies are the active pharmacological agents of immunotherapy . Antiradia-tion Antitoxic Antibodies bind selectively to Radiation Neurotoxins, Cytotoxins, Hematotox-ins and neutralize their toxic activity. We have developed the highly sensitive method and system for extracorporeal-immune-lypmh-plasmo-sorption with antigen-specific IgG which is clinically important for treatment of the toxic and immunologic phases of the ARS. The method of extracorporeal-immune-lypmh-plasmo-sorption includes Antiradiation Antitoxic Antibodies (AAA) immobilized on microporous polymeric membranes with a pore size that is capable to provide diffusion of blood-lymph plasma. Plasma of blood or lymph of irradiated mammals contains Radiation Toxins (RT) that have toxic and antigenic properties. Radiation Toxins are Antigen-specific to Antitoxic blocking antibodies (Immunoglobulin G). Plasma diffuses through membranes with immobilized AAA and AA-antibodies bind to the polysaccharide chain of tox-ins molecules and complexes of AAA-RT that are captured on membrane surfaces. RT were removed from plasma. Re-transfusion of plasma of blood and lymph had been provided. We show a statistical significant reduction in postradiation lethality.

Popov, Dmitri; Maliev, Slava

143

Development of cognitive-behavioral therapy intervention for patients with Dhat syndrome  

PubMed Central

Dhat syndrome is a culture-bound syndrome prevalent in the natives of the Indian subcontinent characterized by excessive concern about harmful consequences of loss of semen (ICD-10). Treatment offered to the patients suffering from it continues to be esoteric, unstructured and without standardization. The present study aimed to develop and examine the feasibility of Cognitive – Behavior Therapy module for patients with Dhat syndrome. A draft module was developed based on existing theoretical knowledge and suggestions from five mental health professionals. This module was then applied on five patients with Dhat syndrome to assess and judge the suitability of the module. The pre and post-assessments were carried out using Sexual Knowledge and Attitude Questionnaire - II, Hamilton Depression Rating Scale, The Cognitive-Somatic Anxiety Scale, Screener for Somatoform Disorder, International Index for Erectile Function, Clinical Global Impressions, The World Health Organization Quality of Life Assessment - BREF. Experiences and insights gained from each patient were used to refine the module before applying on the next patient. The final module consisted of the following components was developed: Basic sex education, cognitive restructuring, relaxation training, imaginal desensitization, masturbatory training as homework and Kegel's exercises and ‘start-stop technique’ and ‘squeeze technique’ for sexual dysfunctions. Results of the study reveal that it is feasible to carry out the CBT module in clinical settings. Number of sessions ranged from 11 to 16 sessions. The duration of the session was 45 minutes on the average. Findings of the present study revealed improvement in sexual knowledge, anxiety, depressive and somatic symptoms. Implications and limitations of the study are highlighted and suggestions for future research offered. PMID:23372242

Salam, K. P. Abdul; Sharma, Mahendra P.; Prakash, Om

2012-01-01

144

Randomized Controlled Trial of Cognitive–Behavioral Group Therapy for Irritable Bowel Syndrome in a Medical Setting  

Microsoft Academic Search

Standard medical treatments have not been effective for irritable bowel syndrome (IBS) patients. Though individualized cognitive–behavior therapy is an empirically supported treatment option, cognitive–behavioral group therapy (CBGT) has yet to be established as an effective alternative in a randomized controlled trial. This study compared the efficacy of a 10-session CBGT with a home-based symptom monitoring with weekly telephone contact (SMTC)

Gregg A. Tkachuk; Lesley A. Graff; Garry L. Martin; Charles N. Bernstein

2003-01-01

145

Metformin therapy improves ovulatory rates, cervical scores, and pregnancy rates in clomiphene citrate-resistant women with polycystic ovary syndrome  

Microsoft Academic Search

Objective: To evaluate the effect of metformin therapy on hyperandrogenism, insulin resistance, cervical scores, ovulation, and pregnancy rates in clomiphene citrate-resistant women with polycystic ovary syndrome (PCOS).Design: Prospective, randomized, double-blind, placebo-controlled study.Setting: Infertility clinic of a tertiary referral center.Patient(s): Fifty-six women with clomiphene citrate-resistant PCOS.Intervention(s): Two cycles of oral metformin therapy (850 mg, twice daily) in group I and placebo

Müberra Kocak; Eray Caliskan; Coskun Simsir; Ali Haberal

2002-01-01

146

Kinesio taping compared to physical therapy modalities for the treatment of shoulder impingement syndrome.  

PubMed

The purpose of this study was to determine and compare the efficacy of kinesio tape and physical therapy modalities in patients with shoulder impingement syndrome. Patients (n?=?55) were treated with kinesio tape (n?=?30) three times by intervals of 3 days or a daily program of local modalities (n?=?25) for 2 weeks. Response to treatment was evaluated with the Disability of Arm, Shoulder, and Hand scale. Patients were questioned for the night pain, daily pain, and pain with motion. Outcome measures except for the Disability of Arm, Shoulder, and Hand scale were assessed at baseline, first, and second weeks of the treatment. Disability of Arm, Shoulder, and Hand scale was evaluated only before and after the treatment. Disability of Arm, Shoulder, and Hand scale and visual analog scale scores decreased significantly in both treatment groups as compared with the baseline levels. The rest, night, and movement median pain scores of the kinesio taping (20, 40, and 50, respectively) group were statistically significantly lower (p values were 0.001, 0.01, and 0.001, respectively) at the first week examination as compared with the physical therapy group (50, 70, and 70, respectively). However, there was no significant difference in the same parameters between two groups at the second week (0.109, 0.07, and 0.218 for rest, night, and movement median pain scores, respectively). Disability of Arm, Shoulder, and Hand scale scores of the kinesio taping group were significantly lower at the second week as compared with the physical therapy group. No side effects were observed. Kinesio tape has been found to be more effective than the local modalities at the first week and was similarly effective at the second week of the treatment. Kinesio taping may be an alternative treatment option in the treatment of shoulder impingement syndrome especially when an immediate effect is needed. PMID:20443039

Kaya, Erkan; Zinnuroglu, Murat; Tugcu, Ilknur

2011-02-01

147

MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy.  

PubMed

Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to a granulomatous inflammation in the cavernous sinus. Corticosteroid therapy dramatically resolves both the clinical and radiological findings of THS. We present MRI findings of six patients with a clinical history of at least one episode of unilateral or bilateral orbital-periorbital pain, clinical findings of associated paresis of one or more of 3rd, 4th, 5th or 6th cranial nerves. All of the patients revealed an enlargement of the symptomatic cavernous sinus on magnetic resonance imaging (MRI) scans. Five patients revealed total resolution of the clinical findings within 1-8 weeks, following systemic corticosteroid treatment. One patient revealed only minor regression of clinical findings within 2 weeks after the initiation of the treatment, so the cavernous sinus lesion was reevaluated as meningioma on MRI, and the patient underwent surgical resection of the mass with resultant histopathological finding of cavernous sinus meningioma. A follow-up MRI scan was performed for five patients at the end of 8-weeks of steroid therapy. Three of these five patients showed total resolution of the cavernous sinus lesions whereas two of them revealed a partial regression of the cavernous sinus lesions. MRI findings before and after systemic corticosteroid therapy are important diagnostic criteria to put the definitive diagnosis of THS and to differentiate it from other cavernous sinus lesions that simulate THS both clinically and radiologically. PMID:12536085

Cakirer, Sinan

2003-02-01

148

Synergistic GABA-Enhancing Therapy against Seizures in a Mouse Model of Dravet Syndrome  

PubMed Central

Seizures remain uncontrolled in 30% of patients with epilepsy, even with concurrent use of multiple drugs, and uncontrolled seizures result in increased morbidity and mortality. An extreme example is Dravet syndrome (DS), an infantile-onset severe epilepsy caused by heterozygous loss of function mutations in SCN1A, the gene encoding the brain type-I voltage-gated sodium channel NaV1.1. Studies in Scn1a heterozygous knockout mice demonstrate reduced excitability of GABAergic interneurons, suggesting that enhancement of GABA signaling may improve seizure control and comorbidities. We studied the efficacy of two GABA-enhancing drugs, clonazepam and tiagabine, alone and in combination, against thermally evoked myoclonic and generalized tonic-clonic seizures. Clonazepam, a positive allosteric modulator of GABA-A receptors, protected against myoclonic and generalized tonic-clonic seizures. Tiagabine, a presynaptic GABA reuptake inhibitor, was protective against generalized tonic-clonic seizures but only minimally protective against myoclonic seizures and enhanced myoclonic seizure susceptibility at high doses. Combined therapy with clonazepam and tiagabine was synergistic against generalized tonic-clonic seizures but was additive against myoclonic seizures. Toxicity determined by rotorod testing was additive for combination therapy. The synergistic actions of clonazepam and tiagabine gave enhanced seizure protection and reduced toxicity, suggesting that combination therapy may be well tolerated and effective for seizures in DS. PMID:23424217

Oakley, John C.; Cho, Alvin R.; Cheah, Christine S.; Scheuer, Todd

2013-01-01

149

Evidence for mast cell activation in patients with therapy-resistant irritable bowel syndrome.  

PubMed

Previous findings suggested an involvement of mast cells in the pathogenesis of irritable bowel syndrome (IBS). The pathophysiological significance of mast cells is defined both by their number in tissue and by their activity. In the present pilot study activity of mast cells in patients with therapy-resistant IBS was investigated for the first time systematically. Twenty patients with therapy-resistant IBS were investigated for the presence of a pathologically increased mast cell mediator release by means of a validated structured interview suitable to identify mast cell mediator-related symptoms and by determing selected surrogate parameters for mast cell activity. Nineteen of the 20 patients presented mast cell mediator-related symptoms. Pathologically increased mast cell activity-related coagulation and fibrinolysis parameters were detected in 11 of 12 patients investigated in that regard. One patient had an elevated level of methylhistamine in urine. The present data provide evidence that in patients with therapy-resistant IBS a pathologically increased systemic mast cell activity may occur with high prevalence. This finding fits to the idea of an assumed contribution of activated mast cells in the pathophysiology of IBS. PMID:21298604

Frieling, T; Meis, K; Kolck, U W; Homann, J; Hülsdonk, A; Haars, U; Hertfelder, H-J; Oldenburg, J; Seidel, H; Molderings, G J

2011-02-01

150

Controversies of oral antiplatelet therapy in acute coronary syndromes and percutaneous coronary intervention.  

PubMed

Platelet activation is a pivotal event in the pathophysiology of acute coronary syndromes (ACS) and percutaneous coronary intervention (PCI) and has a substantial impact on the outcomes in these settings. Aggressive implementation of antiplatelet therapy has significantly decreased adverse cardiovascular events, such as death, myocardial infarction (MI), stroke, and repeat revascularization. Although the widespread use of aspirin has contributed to this improvement, many patients continue to have a significant risk of recurrent events during the ensuing months to years. The advent of other antiplatelet agents, notably the thienopyridine clopidogrel bisulfate has heralded a new era of combined antiplatelet blockade, offering the hope of better outcomes. Recently, clinical trials have tested the use of dual oral antiplatelet blockade and have shown impressive results. Notably, the Clopidogrel in Unstable Angina to Prevent Recurrent Events (CURE) trial found that dual therapy with clopidogrel and aspirin in ACS reduced adverse cardiovascular events by 20% at 1 year (p < 0.001). The PCI-CURE substudy of CURE and the Clopidogrel for the Reduction of Events During Observation (CREDO) trial demonstrated that these benefits extend to patients undergoing both urgent and elective PCI. This article will explore the current role of and controversies in oral antiplatelet therapy after ACS and PCI. PMID:15630671

Rajagopal, Vivek; Bhatt, Deepak L

2004-12-01

151

ADAPTATION D'UN ISOLEMENT DE CORONAVIRUS ENTRIQUE BOVIN A LA CULTURE CELLULAIRE  

E-print Network

ADAPTATION D'UN ISOLEMENT DE CORONAVIRUS ENTĂ?RIQUE BOVIN A LA CULTURE CELLULAIRE ET CARACTĂ?RISATION Thiverval-Grignon, France. . Summary ADAPTATION OF AN ISOLATE OF BOVINE ENTERITIC CORONAVIRUS TO SERIAL GROWTH IN CELL CULTURE AND CHARACTERIZATION OF THE VIRUS. - Coronaviruses are known to behave

Paris-Sud XI, Université de

152

Journes Automates Cellulaires 2008 (Uzs), pp. 216-227 TOPOLOGICAL PROPERTIES OF SAND AUTOMATA  

E-print Network

Journées Automates Cellulaires 2008 (Uzès), pp. 216-227 TOPOLOGICAL PROPERTIES OF SAND AUTOMATA.masson@lif.univ-mrs.fr Abstract. In this paper, we exhibit a strong relation between the sand automata con- figuration space and the cellular automata configuration space. This relation induces a compact topology for sand automata

Paris-Sud XI, Université de

153

Journees Automates Cellulaires 2008 (Uz`es), pp. 102-118 UNIVERSALITIES IN CELLULAR AUTOMATA  

E-print Network

] on self-reproduction in the 1940s, using cellular automata under suggestions by Ulam. Following the workJourn´ees Automates Cellulaires 2008 (Uz`es), pp. 102-118 UNIVERSALITIES IN CELLULAR AUTOMATA in the field of cellular automata. To fulfill this goal, the approach taken here is organized in three parts

Boyer, Edmond

154

Increased Atherothrombotic Burden in Patients with Diabetes Mellitus and Acute Coronary Syndrome: A Review of Antiplatelet Therapy  

PubMed Central

Patients with diabetes mellitus presenting with acute coronary syndrome have a higher risk of cardiovascular complications and recurrent ischemic events when compared to nondiabetic counterparts. Different mechanisms including endothelial dysfunction, platelet hyperactivity, and abnormalities in coagulation and fibrinolysis have been implicated for this increased atherothrombotic risk. Platelets play an important role in atherogenesis and its thrombotic complications in diabetic patients with acute coronary syndrome. Hence, potent platelet inhibition is of paramount importance in order to optimise outcomes of diabetic patients with acute coronary syndrome. The aim of this paper is to provide an overview of the increased thrombotic burden in diabetes and acute coronary syndrome, the underlying pathophysiology focussing on endothelial and platelet abnormalities, currently available antiplatelet therapies, their benefits and limitations in diabetic patients, and to describe potential future therapeutic strategies to overcome these limitations. PMID:22347666

Balasubramaniam, Karthik; Viswanathan, Girish N.; Marshall, Sally M.; Zaman, Azfar G.

2012-01-01

155

Diagnostik und Therapie des Restless-Legs-Syndroms in der Arztpraxis  

Microsoft Academic Search

\\u000a Summary\\u000a Background  Restless legs syndrome (RLS) is a movement and sleep disorder with leg dysesthesias with a high prevalence (9–18% in Austria).\\u000a \\u000a \\u000a \\u000a Aim of the study  Determination of frequency, diagnosis and therapy of RLS in general practitioner and specialist offices.\\u000a \\u000a \\u000a \\u000a Methods  Telephone survey of a random sample of 120 general practitioners, 100 neurologists and 80 specialists in internal medicine.\\u000a \\u000a \\u000a \\u000a Results  69% of the whole

Michael Saletu; Martina Esberger-Chowdhury; Josef Zeitlhofer; Lüder Deecke

2004-01-01

156

Urosodeoxycholic Acid Therapy in a Child with Trimethoprim-Sulfamethoxazole-induced Vanishing Bile Duct Syndrome  

PubMed Central

We present a case of a 7-year-old boy who had cholestasis after trimethoprim-sulfamethoxazole combination therapy. Liver biopsy was performed 36 days after the onset of jaundice because of no evidence of improving cholestasis. Liver histology revealed portal inflammation, bile plug, and biliary stasis around the central vein with the loss of the interlobular bile ducts. Immunohistochemical stains for cytokeratin 7 and 19 were negative. These findings were consistent with those of vanishing bile duct syndrome (VBDS). Chlestasis was progressively improved with dose increment of urosodeoxycholic acid from conventional to high dose. This is the first case report of trimethoprime-sulfamethoxazole associated VBDS in Korean children. The case suggests that differential diagnosis of VBDS should be considered in case of progressive cholestatic hepatitis with elevation of alkaline phosphatase and gamma-glutamyl transpeptidase after or during taking medicine to treat nonhepatobiliary diseases illness. PMID:24511525

Cho, Hyun Jeong; Jwa, Hye Jeong; Kim, Kyu Seon; Gang, Dae Yong

2013-01-01

157

Randomized clinical trial of surgery versus conservative therapy for carpal tunnel syndrome [ISRCTN84286481  

PubMed Central

Background Conservative treatment remains the standard of care for treating mild to moderate carpal tunnel syndrome despite a small number of well-controlled studies and limited objective evidence to support current treatment options. There is an increasing interest in the usefulness of wrist magnetic resonance imaging could play in predicting who will benefit for various treatments. Method and design Two hundred patients with mild to moderate symptoms will be recruited over 3 1/2 years from neurological surgery, primary care, electrodiagnostic clinics. We will exclude patients with clinical or electrodiagnostic evidence of denervation or thenar muscle atrophy. We will randomly assign patients to either a well-defined conservative care protocol or surgery. The conservative care treatment will include visits with a hand therapist, exercises, a self-care booklet, work modification/ activity restriction, B6 therapy, ultrasound and possible steroid injections. The surgical care would be left up to the surgeon (endoscopic vs. open) with usual and customary follow-up. All patients will receive a wrist MRI at baseline. Patients will be contacted at 3, 6, 9 and 12 months after randomization to complete the Carpal Tunnel Syndrome Assessment Questionnaire (CTSAQ). In addition, we will compare disability (activity and work days lost) and general well being as measured by the SF-36 version II. We will control for demographics and use psychological measures (SCL-90 somatization and depression scales) as well as EDS and MRI predictors of outcomes. Discussion We have designed a randomized controlled trial which will assess the effectiveness of surgery for patients with mild to moderate carpal tunnel syndrome. An important secondary goal is to study the ability of MRI to predict patient outcomes. PMID:15656907

Martin, Brook I; Levenson, Linda M; Hollingworth, William; Kliot, Michel; Heagerty, Patrick J; Turner, Judith A; Jarvik, Jeffrey G

2005-01-01

158

Usher syndrome: animal models, retinal function of Usher proteins, and prospects for gene therapy  

PubMed Central

Usher syndrome is a deafness-blindness disorder. The blindness occurs from a progressive retinal degeneration that begins after deafness and after the retina has developed. Three clinical subtypes of Usher syndrome have been identified, with mutations in any one of six different genes giving rise to type 1, in any one of three different genes to type 2, and in one identified gene causing Usher type 3. Mutant mice for most of the genes have been studied; while they have clear inner ear defects, retinal phenotypes are relatively mild and have been difficult to characterize. The retinal functions of the Usher proteins are still largely unknown. Protein binding studies have suggested many interactions among the proteins, and a model of interaction among all the proteins in the photoreceptor synapse has been proposed. However this model is not supported by localization data from some laboratories, or the indication of any synaptic phenotype in mutant mice. An earlier suggestion, based on patient pathologies, of Usher protein function in the photoreceptor cilium continues to gain support from immunolocalization and mutant mouse studies, which are consistent with Usher protein interaction in the photoreceptor ciliary/periciliary region. So far, the most characterized Usher protein is myosin VIIa. It is present in the apical RPE and photoreceptor ciliary/periciliary region, where it is required for organelle transport and clearance of opsin from the connecting cilium, respectively. Usher syndrome is amenable to gene replacement therapy, but also has some specific challenges. Progress in this treatment approach has been achieved by correction of mutant phenotypes in Myo7a-null mouse retinas, following lentiviral delivery of MYO7A. PMID:17936325

Williams, David S.

2009-01-01

159

Molecular analysis, pathogenic mechanisms, and readthrough therapy on a large cohort of Kabuki syndrome patients.  

PubMed

Kabuki syndrome (KS) is a multiple congenital anomalies syndrome characterized by characteristic facial features and varying degrees of mental retardation, caused by mutations in KMT2D/MLL2 and KDM6A/UTX genes. In this study, we performed a mutational screening on 303 Kabuki patients by direct sequencing, MLPA, and quantitative PCR identifying 133 KMT2D, 62 never described before, and four KDM6A mutations, three of them are novel. We found that a number of KMT2D truncating mutations result in mRNA degradation through the nonsense-mediated mRNA decay, contributing to protein haploinsufficiency. Furthermore, we demonstrated that the reduction of KMT2D protein level in patients' lymphoblastoid and skin fibroblast cell lines carrying KMT2D-truncating mutations affects the expression levels of known KMT2D target genes. Finally, we hypothesized that the KS patients may benefit from a readthrough therapy to restore physiological levels of KMT2D and KDM6A proteins. To assess this, we performed a proof-of-principle study on 14 KMT2D and two KDM6A nonsense mutations using specific compounds that mediate translational readthrough and thereby stimulate the re-expression of full-length functional proteins. Our experimental data showed that both KMT2D and KDM6A nonsense mutations displayed high levels of readthrough in response to gentamicin treatment, paving the way to further studies aimed at eventually treating some Kabuki patients with readthrough inducers. PMID:24633898

Micale, Lucia; Augello, Bartolomeo; Maffeo, Claudia; Selicorni, Angelo; Zucchetti, Federica; Fusco, Carmela; De Nittis, Pasquelena; Pellico, Maria Teresa; Mandriani, Barbara; Fischetto, Rita; Boccone, Loredana; Silengo, Margherita; Biamino, Elisa; Perria, Chiara; Sotgiu, Stefano; Serra, Gigliola; Lapi, Elisabetta; Neri, Marcella; Ferlini, Alessandra; Cavaliere, Maria Luigia; Chiurazzi, Pietro; Monica, Matteo Della; Scarano, Gioacchino; Faravelli, Francesca; Ferrari, Paola; Mazzanti, Laura; Pilotta, Alba; Patricelli, Maria Grazia; Bedeschi, Maria Francesca; Benedicenti, Francesco; Prontera, Paolo; Toschi, Benedetta; Salviati, Leonardo; Melis, Daniela; Di Battista, Eliana; Vancini, Alessandra; Garavelli, Livia; Zelante, Leopoldo; Merla, Giuseppe

2014-07-01

160

Combination therapy with montelukast and ketotifen for arthritis and rash resulting from idiopathic hypereosinophilic syndrome.  

PubMed

Idiopathic hypereosinophilic syndrome (IHES) is a rare condition of uncertain etiology characterized by marked peripheral blood eosinophilia and organ system dysfunction that cannot be explained by any factor other than the presence of eosinophils or their potentially toxic products. Diagnostic criteria include 1) a sustained eosinophilia greater than 1500/mm for longer than 6 months, 2) absence of other causes of eosinophilia, including parasitic infections and allergic diseases, and 3) multiorgan involvement (ie, lungs, heart, central nervous system, skin, liver, joints). Steroids represent the initial therapeutic approach, although for those patients unresponsive to steroids, cytotoxic chemotherapy should be considered. We describe a case of IHES characterized by polyarticular inflammatory joint involvement, hypereosinophilia, and urticarioid skin manifestations without visceral involvement. Synovial fluid smears as well as pathology of skin lesions and knee synovial membrane confirmed the presence of numerous eosinophils. The patient was successfully treated with a combination therapy of a cysteinyl leukotriene receptor antagonist (montelukast) and ketotifen. Hypereosinophilic syndrome like in this patient is a rare cause of polyarthritis. PMID:17043546

Selvi, Enrico; Rubegni, Pietro; Manganelli, Stefania; De Stefano, Renato; Frati, Elena; Lorenzini, Sauro; Sbano, Paolo; Marcolongo, Roberto

2004-12-01

161

Clobazam as an adjunctive therapy in treating seizures associated with Lennox–Gastaut syndrome  

PubMed Central

Lennox–Gastaut syndrome (LGS) is a devastating childhood epilepsy syndrome characterized by the occurrence of multiple types of seizures and cognitive decline. Most children suffer from frequent seizures that are refractory to current medical management. Recent clinical trials have suggested that addition of clobazam may improve the clinical outcome for some LGS patients. Although clobazam has been available for over five decades, it has only recently been approved by the US Food and Drug Administration for this indication. As a 1,5-benzodiazepine, clobazam is structurally related to the widely used 1,4-benzodiazepines, which include diazepam. Clobazam has been shown to modulate GABAergic neurotransmission by positive allosteric modulation of GABAA receptors, and to increase expression of transporters for both GABA and glutamate. The active metabolite n-desmethylclobazam (norclobazam) also modulates GABAA receptors, and the relative importance of these two compounds in the clinical effectiveness of clobazam remains an open question. Clinical trials involving clobazam as an addon therapy in a variety of pediatric epilepsy populations have found a significant improvement in seizure control. In patients with LGS, clobazam may have greatest efficacy for drop seizures. Longstanding clinical experience suggests that clobazam is a safe and well tolerated antiepileptic drug with infrequent and mild adverse effects. These results suggest that adjunctive treatment with clobazam may be a reasonable option for LGS patients, particularly those who are treatment-resistant. PMID:22128252

Leahy, Jennifer T; Chu-Shore, Catherine J; Fisher, Janet L

2011-01-01

162

Molecular Analysis, Pathogenic Mechanisms, and Readthrough Therapy on a Large Cohort of Kabuki Syndrome Patients  

PubMed Central

Kabuki syndrome (KS) is a multiple congenital anomalies syndrome characterized by characteristic facial features and varying degrees of mental retardation, caused by mutations in KMT2D/MLL2 and KDM6A/UTX genes. In this study, we performed a mutational screening on 303 Kabuki patients by direct sequencing, MLPA, and quantitative PCR identifying 133 KMT2D, 62 never described before, and four KDM6A mutations, three of them are novel. We found that a number of KMT2D truncating mutations result in mRNA degradation through the nonsense-mediated mRNA decay, contributing to protein haploinsufficiency. Furthermore, we demonstrated that the reduction of KMT2D protein level in patients’ lymphoblastoid and skin fibroblast cell lines carrying KMT2D-truncating mutations affects the expression levels of known KMT2D target genes. Finally, we hypothesized that the KS patients may benefit from a readthrough therapy to restore physiological levels of KMT2D and KDM6A proteins. To assess this, we performed a proof-of-principle study on 14 KMT2D and two KDM6A nonsense mutations using specific compounds that mediate translational readthrough and thereby stimulate the re-expression of full-length functional proteins. Our experimental data showed that both KMT2D and KDM6A nonsense mutations displayed high levels of readthrough in response to gentamicin treatment, paving the way to further studies aimed at eventually treating some Kabuki patients with readthrough inducers. PMID:24633898

Micale, Lucia; Augello, Bartolomeo; Maffeo, Claudia; Selicorni, Angelo; Zucchetti, Federica; Fusco, Carmela; De Nittis, Pasquelena; Pellico, Maria Teresa; Mandriani, Barbara; Fischetto, Rita; Boccone, Loredana; Silengo, Margherita; Biamino, Elisa; Perria, Chiara; Sotgiu, Stefano; Serra, Gigliola; Lapi, Elisabetta; Neri, Marcella; Ferlini, Alessandra; Cavaliere, Maria Luigia; Chiurazzi, Pietro; Monica, Matteo Della; Scarano, Gioacchino; Faravelli, Francesca; Ferrari, Paola; Mazzanti, Laura; Pilotta, Alba; Patricelli, Maria Grazia; Bedeschi, Maria Francesca; Benedicenti, Francesco; Prontera, Paolo; Toschi, Benedetta; Salviati, Leonardo; Melis, Daniela; Di Battista, Eliana; Vancini, Alessandra; Garavelli, Livia; Zelante, Leopoldo; Merla, Giuseppe

2014-01-01

163

Cell and Gene Therapy for Genetic Diseases: Inherited Disorders Affecting the Lung and Those Mimicking Sudden Infant Death Syndrome  

PubMed Central

Abstract Some of the first human gene therapy trials targeted diseases of the lung and provided important information that will continue to help shape future trials. Here we describe both cell and gene therapies for lung diseases such as cystic fibrosis and alpha-1 antitrypsin disorder as well as fatty acid oxidation disorders that mimic sudden infant death syndrome (SIDS). Human clinical gene therapy trials for cystic fibrosis and alpha-1 antitrypsin have been performed using a variety of vectors including adenovirus, adeno-associated virus, and nonviral vectors. No human clinical gene therapy trials have been performed for disorders of fatty acid oxidation; however, important proof-of-principle studies have been completed for multiple fatty acid oxidation disorders. Important achievements have been made and have yet to come for cell and gene therapies for disorders of the lung and those mimicking SIDS. PMID:22642257

Keeler, Allison M.

2012-01-01

164

Comparison of long-term effects of cognitive-behavioral therapy versus mindfulness-based therapy on reduction of symptoms among patients suffering from irritable bowel syndrome  

PubMed Central

Aim The aim of this study was to compare the long-term effects of cognitive-behavioral treatment and mindfulness-based treatment on decreasing symptoms of patients suffering from irritable bowel syndrome (IBS). Background One of the most modern therapies put forward in therapy of IBS is mindfulness-based metacognitive therapy. Patients and methods In this quasi-experimental study, 36 people with mean age of 32 years old, including 24 patients with IBS and 12 healthy normal subjects as control group, were studied. Patients with IBS were randomly divided into two experimental groups of cognitive-behavioral treatment (n=12) and mindfulness-based treatment (n=12). Data were analysed by one-way covariance analysis. Results There was significant decrease of the symptoms of IBS among two treatment groups versus control group in long-term (p<0.05). Mindfulness-based therapy was the most effective technique in decreasing symptoms. Conclusion This study showed mindfulness-based therapy, as a modern psychotherapy technique, is an effective method to decrease symptoms of patients with irritable bowel syndrome, compared with old methods. Therefore, this technique is advised among these patients, especially those who have refractory symptoms. PMID:24834303

Zomorodi, Saeedeh; Abdi, Saeed; Tabatabaee, Seyed Kazem Rasulzadeh

2014-01-01

165

IMPACT OF CHRONIC ANTI-CHOLESTEROL THERAPY ON DEVELOPMENT OF MICROVASCULAR RAREFACTION IN THE METABOLIC SYNDROME  

PubMed Central

Object The obese Zucker rat (OZR) model of the metabolic syndrome is partly characterized by moderate hypercholesterolemia in addition to other contributing co-morbidities. Previous results suggest that vascular dysfunction in OZR is associated with chronic reduction in vascular nitric oxide (NO) bioavailability and chronic inflammation, both frequently associated with hypercholesterolemia. As such, we evaluated the impact of chronic cholesterol reducing therapy on the development of impaired skeletal muscle arteriolar reactivity and microvessel density in OZR and its impact on chronic inflammation and NO bioavailability. Materials and Methods Beginning at 7 weeks of age, male OZR were treated with gemfibrozil, probucol, atorvastatin or simvastatin (in chow) for 10 weeks. Subsequently, plasma and vascular samples were collected for biochemical/molecular analyses, while arteriolar reactivity and microvessel network structure were assessed using established methodologies after 3, 6 and 10 weeks of drug therapy Results All interventions were equally effective at reducing total cholesterol, although only the statins also blunted the progressive reductions to vascular NO bioavailability, evidenced by greater maintenance of acetylcholine-induced dilator responses, an attenuation of adrenergic constrictor reactivity, and an improvement in agonist-induced NO production. Comparably, while minimal improvements to arteriolar wall mechanics were identified with any of the interventions, chronic statin treatment reduced the rate of microvessel rarefaction in OZR. Associated with these improvements was a striking statin-induced reduction in inflammation in OZR, such that numerous markers of inflammation were correlated with improved microvascular reactivity and density. However, using multivariate discriminant analyses, plasma RANTES, IL-10, MCP-1 and TNF-? were determined to be the strongest contributors to differences between groups, although their relative importance varied with time. Conclusions While the positive impact of chronic statin treatment on vascular outcomes in the metabolic syndrome are independent of changes to total cholesterol, and are more strongly associated with improvements to vascular NO bioavailability and attenuated inflammation, these results provide both a spatial and temporal framework for targeted investigation into mechanistic determinants of vasculopathy in the metabolic syndrome. PMID:19905967

Goodwill, Adam G.; Frisbee, Stephanie J.; Stapleton, Phoebe A.; James, Milinda E.; Frisbee, Jefferson C.

2011-01-01

166

A severe course of Cronkhite-Canada syndrome and the review of clinical features and therapy in 49 Chinese patients.  

PubMed

We describe a severe case of Cronkhite-Canada syndrome and review the clinical features and therapy in 49 Chinese patients. A 67-year-old man who underwent severe chronic diarrhea had typical clinical manifestations of hyperpigmentation, hair loss, and dystrophic changes in the fingernails. Although sufficient nutrition support and other therapies reported in the literature were provided, the patient died of systemic failure one year later. Cronkhite-Canada syndrome is characterized by generalized gastrointestinal polyps associated with hyperpigmentation, hair loss, and onycholysis. Anemia, positive stool occult blood, serum electrolyte disturbances, and low serum proteins are the main clinical features of patients with Cronkhite-Canada syndrome. Most patients were diagnosed by esophagogastroduodenoscopy and/or colonoscopy, and polyps were found in the entire alimentary tract, except the notable exception of the esophagus. The polyp-like samples of mucosa, hyperplasia, and adenoma were characterized by acute/chronic inflammation. Four cases were complicated with cancer. The treatment of Cronkhite-Canada syndrome includes symptomatic and support therapy, administration of corticosteroids, antibiotics and acid inhibitors, therapeutic endoscopy, and surgery. While the mortality rate was reported as 47.3% (9/19), some patients may live a long life with controlled symptoms. PMID:24226722

She, Qiang; Jiang, Jian-Xia; Si, Xin-Min; Tian, Xue-Ying; Shi, Rui-Hua; Zhang, Guo-Xin

2013-01-01

167

Correction of murine Bernard-Soulier syndrome by lentivirus-mediated gene therapy.  

PubMed

Bernard-Soulier syndrome (BSS) is an inherited bleeding disorder caused by a defect in the platelet glycoprotein (GP) Ib-IX-V complex. The main treatment for BSS is platelet transfusion but it is often limited to severe bleeding episodes or surgical interventions due to the risk of alloimmunization. We have previously reported successful expression of human GPIb? (hGPIb?) in human megakaryocytes using a lentiviral vector (LV) encoding human GP1BA under control of the platelet-specific integrin ?IIb promoter (2bIb?). In this study, we examined the efficacy of this strategy for the gene therapy of BSS using GPIb?(null) as a murine model of BSS. GPIb?(null) hematopoietic stem cells (HSC) transduced with 2bIb? LV were transplanted into lethally irradiated GPIb?(null) littermates. Therapeutic levels of hGPIb? expression were achieved that corrected the tail bleeding time and improved the macrothrombocytopenia. Sequential bone marrow (BM) transplants showed sustained expression of hGPIb? with similar phenotypic correction. Antibody response to hGPIb? was documented in 1 of 17 total recipient mice but was tolerated without any further treatment. These results demonstrate that lentivirus-mediated gene transfer can provide sustained phenotypic correction of murine BSS, indicating that this approach may be a promising strategy for gene therapy of BSS patients. PMID:22044935

Kanaji, Sachiko; Kuether, Erin L; Fahs, Scot A; Schroeder, Jocelyn A; Ware, Jerry; Montgomery, Robert R; Shi, Qizhen

2012-03-01

168

Antithrombotic Therapy in Patients with Acute Coronary Syndrome in the Intermountain Heart Collaborative Study  

PubMed Central

Objective. To determine factors associated with single antiplatelet (SAP) or dual antiplatelet (DAP) therapy and anticoagulants (AC) use in hospital and after discharge among patients with acute coronary syndrome (ACS). Methods. We evaluated 5,294 ACS patients in the Intermountain Heart Collaborative Study from 2004 to 2009. Multivariable logistic regressions were used to determine predictors of AC or AP use. Results. In hospital, 99% received an AC, 79% DAP, and 19% SAP; 78% had DAP + AC. Coronary stents were the strongest predictors of DAP use in hospital compared to SAP (P < 0.001). After discharge, 77% received DAP, 20% SAP, and 9% AC; 5% had DAP + AC. DAP compared to SAP was less likely for patients on AC (odds ratio [OR] = 0.30, P < 0.0001) after discharge. Placement of a stent increased the likelihood of DAP (bare metal: OR = 54.8, P < 0.0001; drug eluting: OR = 59.4, P < 0.0001). 923 had atrial fibrillation and 337 had a history of venous thromboembolism; these patients had increased use of AC (29% and 40%, resp.). Conclusion. While in-hospital use of AC was nearly universal, postdischarge AC use was rare. Concern for providing the best antithrombotic therapy, while maintaining an acceptable bleeding risk, may explain the selection decisions. PMID:25632367

Klaskala, Winslow; Woller, Scott C.; Horne, Benjamin D.; Bunch, T. Jared; Le, Viet T.; Mills, Roger M.; Muhlestein, Joseph B.

2015-01-01

169

Supervised exercise therapy versus usual care for patellofemoral pain syndrome: an open label randomised controlled trial  

PubMed Central

Objective To assess the effectiveness of supervised exercise therapy compared with usual care with respect to recovery, pain, and function in patients with patellofemoral pain syndrome. Design Open label randomised controlled trial. Setting General practice and sport physician practice. Participants Patients with a new episode of patellofemoral pain syndrome recruited by their general practitioner or sport physician. Interventions The intervention group received a standardised exercise programme for 6 weeks tailored to individual performance and supervised by a physical therapist, and were instructed to practise the tailored exercises at home for 3 months. The control group were assigned usual care, which comprised a “wait and see” approach of rest during periods of pain and refraining from pain provoking activities. Both the intervention group and the control group received written information about patellofemoral pain syndrome and general instructions for home exercises. Main outcome measures The primary outcomes were self reported recovery (7 point Likert scale), pain at rest and pain on activity (0-10 point numerical rating scale), and function (0-100 point Kujala patellofemoral score) at 3 months and 12 months follow-up. Results A total of 131 participants were included in the study: 65 in the intervention group and 66 in the control group. After 3 months, the intervention group showed better outcomes than the control group with regard to pain at rest (adjusted difference ?1.07, 95% confidence interval ?1.92 to ?0.22; effect size 0.47), pain on activity (?1.00, ?1.91 to ?0.08; 0.45), and function (4.92, 0.14 to 9.72; 0.34). At 12 months, the intervention group continued to show better outcomes than the control group with regard to pain (adjusted difference in pain at rest ?1.29, ?2.16 to ?0.42; effect size 0.56; pain on activity ?1.19, ?2.22 to ?0.16; effect size 0.54), but not function (4.52, ?0.73 to 9.76). A higher proportion of patients in the exercise group than in the control group reported recovery (41.9% v 35.0% at 3 months and 62.1% v 50.8% at 12 months), although the differences in self reported recovery between the two groups were not statistically significant. Predefined subgroup analyses revealed that patients recruited by sport physicians (n=30) did not benefit from the intervention, whereas those recruited by general practitioners (n=101) showed significant and clinically relevant differences in pain and function in favour of the intervention group. Conclusion Supervised exercise therapy resulted in less pain and better function at short term and long term follow-up compared with usual care in patients with patellofemoral pain syndrome in general practice. Exercise therapy did not produce a significant difference in the rate of self reported recovery. Trial registration ISRCTN83938749. PMID:19843565

2009-01-01

170

Brice MarcetChercheur en biologie cellulaire Texte:LaureCailloce.Photo:CNRSDR20-JohnPusceddu  

E-print Network

'Institut de pharmacologie moléculaire et cellulaire (IPMC), le chercheur y mène des recherches au plus près de scientifique engagé, qui s'inscrivent dans ceux menés à l'IPMC depuis les années 1990, ont reçu depuis leur moléculaire et cellulaire (IPMC), CNRS / Université de Nice Sophia-Antipolis, Nice |www.ipmc

van Tiggelen, Bart

171

Le naevus bleu cellulaire atypique du poignet: ŕ propos d'un cas et revue de la literature  

PubMed Central

Le naevus bleu cellulaire atypique est une entité pathologique rare et sa localisation au niveau du poignet est exceptionnelle. Il est Considéré comme une Variante ŕ des caractéristiques intermédiaires entre le naevus bleu cellulaire typique et le naevus bleu malin, dont l’évolution est incertaine. Le but de notre travail est d'attirer l'attention sur cette variété lésionnelle rare et de discuter les diagnostiques différentiels, ainsi que décrire les aspects histologique et les options thérapeutiques possibles. PMID:25426206

Boussakri, Hassan; Roux, Jean Luc; Durand, Luc; Elibrahimi, Abdelhalim; Elmrini, Abdelmajid

2014-01-01

172

Clinical pilot study: efficacy of triple antibiotic therapy in Blastocystis positive irritable bowel syndrome patients  

PubMed Central

Background Blastocystis species are common human enteric parasites. Carriage has been linked to Irritable Bowel Syndrome (IBS). Treatment of Blastocystis spp. with antimicrobials is problematic and insensitive diagnostic methods and re-infection complicate assessment of eradication. We investigated whether triple antibiotic therapy comprising diloxanide furoate, trimethoprim/sulfamethoxazole and secnidazole (TAB) given to diarrhoea-predominant IBS (D-IBS) patients positive for Blastocystis would achieve eradication. Methods In a longitudinal, prospective case study 10 D-IBS Blastocystis-positive patients took 14 days of diloxanide furoate 500 mg thrice daily, trimethoprim/sulfamethoxazole 160/80 mg twice daily and secnidazole 400 mg thrice daily. Faecal specimens were collected at baseline, day 15 and 4 weeks after completion of TAB. Specimens were analysed using faecal smear, culture and polymerase chain reaction (PCR) of the 16 SSU rRNA. Patients kept a concurrent clinical diary. Results Six (60%) patients cleared Blastocystis spp. after TAB, including three who had failed previous therapy. Subtypes detected were ST3 (60%), ST4 (40%), ST1 (20%) and ST7, 8 (10%); four patients had mixed ST infections. Serum immunoglobulin A (IgA) levels were low in 40% of patients. Higher rates of Blastocystis clearance were observed in patients symptomatic for less than a year (Mann–Whitney, p?=?0.032, 95% confidence) with no associations found with age, previous antibiotic therapy, faecal parasite load, ST, IgA level or clinical improvement. Conclusions Clearance of Blastocystis spp. was achieved with TAB in 60% of D-IBS patients, an improvement over conventional monotherapy. Higher clearance rates are needed to facilitate investigation of the relevance of this parasite in clinically heterogenous IBS. PMID:25349629

2014-01-01

173

Cardiac Dysfunction and Oxidative Stress in the Metabolic Syndrome: an Update on Antioxidant Therapies  

PubMed Central

The metabolic syndrome (MetS) is a cluster of risk factors including obesity, insulin resistance, dyslipidemia, elevated blood pressure and glucose intolerance. The MetS increases the risk for cardiovascular disease (CVD) and type 2 diabetes. Each component of the MetS causes cardiac dysfunction and their combination carries additional risk. The mechanisms underlying cardiac dysfunction in the MetS are complex and might include lipid accumulation, increased fibrosis and stiffness, altered calcium homeostasis, abnormal autophagy, altered substrate utilization, mitochondrial dysfunction and increased oxidative stress. Mitochondrial and extra-mitochondrial sources of reactive oxygen species (ROS) and reduced antioxidant defense mechanisms characterize the myocardium of humans and animals with the MetS. The mechanisms for increased cardiac oxidative stress in the MetS are not fully understood but include increased fatty acid oxidation, mitochondrial dysfunction and enhanced NADPH oxidase activity. Therapies aimed to reduce oxidative stress and enhance antioxidant defense have been employed to reduce cardiac dysfunction in the MetS in animals. In contrast, large scale clinical trials using antioxidants therapies for the treatment of CVD have been disappointing because of the lack of efficacy and undesired side effects. The focus of this review is to summarize the current knowledge about the mechanisms underlying cardiac dysfunction in the MetS with a special interest in the role of oxidative stress. Finally, we will update the reader on the results obtained with natural antioxidant and mitochondria-targeted antioxidant therapies for the treatment of CVD in the MetS. PMID:23323621

Ilkun, Olesya; Boudina, Sihem

2013-01-01

174

Rufinamide: a pharmacoeconomic profile of its use as adjunctive therapy in Lennox-Gastaut syndrome.  

PubMed

Rufinamide (Inovelon®), a triazole derivative, is an oral antiepileptic drug approved in the EU as adjunctive therapy in the treatment of seizures associated with Lennox-Gastaut syndrome (LGS) in patients aged ?4 years. The efficacy of oral rufinamide as adjunctive therapy in patients with LGS uncontrolled on one to three concomitant antiepileptic drugs was demonstrated in a pivotal, 12-week, randomized, double-blind trial. Rufinamide significantly reduced the 28-day frequency of both drop attacks and total seizures compared with placebo, and significantly increased the proportions of patients experiencing a ?50% reduction in each seizure frequency. A significantly higher proportion of rufinamide than placebo recipients recorded an improvement in seizure severity at the end of treatment. Reductions in the frequency of drop attacks and total seizures were maintained in a long-term (up to 3 years), open-label extension study. Oral rufinamide was generally well tolerated in patients with LGS. Somnolence and vomiting were the most common adverse events occurring more frequently with rufinamide than with placebo. Two pharmacoeconomic analyses, using decision-analysis models with 3-month cycles over a time horizon of 3 years, assessed the cost effectiveness and cost utility, respectively, of rufinamide compared with topiramate and lamotrigine as adjunctive therapy in patients with LGS from the perspective of the UK NHS. The cost-effectiveness analysis suggested that rufinamide would be associated with incremental costs of Ł62 (drop attacks) or Ł2151 (total seizures) per 1% increase in the number of patients achieving a >50% reduction in seizure frequency over 3 years. The cost-utility analysis predicted that the incremental cost per QALY gained for rufinamide compared with the next less-costly and undominated therapy would be more than 5-fold higher than the commonly accepted willingness-to-pay threshold range in the UK. In conclusion, the available pharmacoeconomic data indicate that rufinamide is more effective, but more expensive, than alternative adjunctive therapies approved for use in patients with LGS in the UK. Rufinamide would appear to be a cost-effective alternative to topiramate. Although rufinamide exceeds conventional cost-effectiveness thresholds when compared with lamotrigine, it may still be considered a valuable treatment option for a devastating orphan disease such as LGS. PMID:22332960

McCormack, Paul L

2012-03-01

175

Tuberculosis Immune Reconstitution Inflammatory Syndrome in Children Initiating Antiretroviral Therapy for HIV Infection  

PubMed Central

Background People with HIV initiating combination antiretroviral therapy are at risk for tuberculosis-associated immune reconstitution inflammatory syndrome (TB-IRIS). While this syndrome has been well researched in adults, little is known about the incidence, case fatality, underlying immunopathology and treatment approaches in children. Methods Major databases were searched for articles related to TB-IRIS in children. Data were abstracted using standardized forms. Results Thirteen studies were identified: 6 retrospective and 2 prospective cohort studies, 1 cross-sectional study, 3 case reports and 1 case series. In total, 303 cases of TB-IRIS were described, of which 270 were unmasking TB-IRIS, 12 paradoxical TB-IRIS and 21 were not classifiable due to lack of key information. None of the cohort studies had investigation of TB-IRIS as its primary aim. Nine studies were from Africa, 3 from Asia and 1 from Latin America. Age at cART initiation (reported by 12 studies) ranged from 1 month to 16 years. Median time from start of cART to IRIS diagnosis (reported by 8 studies) ranged from 8 days to 16 weeks. Few deaths attributable to TB-IRIS were recorded. Treatment was only discussed in 2 case studies, both of which reported children receiving corticosteroids. No studies evaluated risk factors for, or immunopathogenesis of, pediatric TB-IRIS. Conclusions There is a paucity of information available on TB-IRIS in children. Future research assessing incidence, risk factors, case fatality and optimal treatment or prevention strategies of TB-IRIS is needed. PMID:24736441

Link-Gelles, Ruth; Moultrie, Harry; Sawry, Shobna; Murdoch, David; Van Rie, Annelies

2014-01-01

176

The effectiveness of low laser therapy in subacromial impingement syndrome: a randomized placebo controlled double?blind prospective study  

PubMed Central

OBJECTIVES: Conflicting results were reported about the effectiveness of Low level laser therapy on musculoskeletal disorders. The aim of this study was to investigate the effectiveness of 850?nm gallium arsenide aluminum (Ga?As?Al) laser therapy on pain, range of motion and disability in subacromial impingement syndrome. METHODS: A total of 52 patients (33 females and 19 males with a mean age of 53.59±11.34 years) with subacromial impingement syndrome were included. The patients were randomly assigned into two groups. Group I (n?=?30, laser group) received laser therapy (5 joule/cm2 at each point over maximum 5?6 painful points for 1 minute). Group II (n?=?22, placebo laser group) received placebo laser therapy. Initially cold pack (10 minutes) was applied to all of the patients. Also patients were given an exercise program including range of motion, stretching and progressive resistive exercises. The therapy program was applied 5 times a week for 14 sessions. Pain severity was assessed by using visual analogue scale. Range of motion was measured by goniometer. Disability was evaluated by using Shoulder Pain and Disability Index. RESULTS: In group I, statistically significant improvements in pain severity, range of motion except internal and external rotation and SPADI scores were observed compared to baseline scores after the therapy (p<0.05). In Group II, all parameters except range of motion of external rotation were improved (p<0.05). However, no significant differences were recorded between the groups (p>0.05). CONCLUSIONS: The Low level laser therapy seems to have no superiority over placebo laser therapy in reducing pain severity, range of motion and functional disability. PMID:21120304

Dogan, Sebnem Koldas; AY, Saime; Evcik, Deniz

2010-01-01

177

Recurrent Stent Thrombosis in a Patient with Antiphospholipid Syndrome and Dual Anti-Platelet Therapy Non-Responsiveness  

PubMed Central

Antiphospholipid syndrome (APS), the most common acquired hypercoagulable condition, is diagnosed by persistent presence of antiphospholipid antibodies and episodes of vascular thrombosis. It may be an important predisposing factor for stent thrombosis, resulting in poor outcomes. Also, anti-platelet therapy non-responsiveness is associated with stent thrombosis. We report a case of a 39-year-old man who after undergoing successful percutaneous coronary intervention for significant coronary artery disease suffered repeated stent thrombosis events leading to ST-segment elevation myocardial infarction. Eventually, he underwent coronary artery bypass surgery because of uncontrolled thrombosis and was diagnosed as having APS and dual antiplatelet therapy non-responsiveness. PMID:25653707

Lee, You-Hong; Tahk, Seung-Jea; Hong, You-Sun; Park, Jin-Sun; Seo, Kyoung-Woo; Choi, Yong-Woo; Noh, Choong-Kyun

2015-01-01

178

Metformin therapy improves the menstrual pattern with minimal endocrine and metabolic effects in women with polycystic ovary syndrome  

Microsoft Academic Search

Objective: To determine the clinical, hormonal, and biochemical effects of 4–6 months of metformin therapy in obese patients with polycystic ovary syndrome (PCOS).Design: Prospective study.Setting: The Gynecological Endocrine Unit of University Central Hospital, Oulu, Finland.Patient(s): Twenty obese patients with PCOS.Intervention(s): Patients were treated with 0.5 g of metformin three times daily for 4–6 months.Main Outcome Measure(s): Clinical symptoms, menstrual pattern,

Laure C. Morin-Papunen; Riitta M. Koivunen; Aimo Ruokonen; Hannu K. Martikainen

1998-01-01

179

Autologous stem cell transplantation in refractory Asherman's syndrome: A novel cell based therapy  

PubMed Central

BACKGROUND: There is substantial evidence that adult stem cell populations exist in human endometrium, and hence it is suggested that either endogenous endometrial stem/progenitor cells can be activated or bone marrow derived stem cells can be transplanted in the uterine cavity for endometrial regeneration in Asherman's syndrome (AS). AIMS AND OBJECTIVES: The objective was to evaluate the role of sub-endometrial autologous stem cell implantation in women with refractory AS in attaining menstruation and fertility. SETTING: Tertiary care referral center. DESIGN: Prospective case series. MATERIALS AND METHODS: Six cases of refractory AS with failed standard treatment option of hysteroscopic adhesiolysis in the past were included. Mononuclear stem cells (MNCs) were implanted in sub-endometrial zone followed by exogenous oral estrogen therapy. Endometrial thickness (ET) was assessed at 3, 6, and 9 months. RESULTS: Descriptive statistics and statistical analysis of study variables was carried out using STATA version 9.0. The mean MNC count was 103.3 × 106 (±20.45) with mean CD34+ count being 203,642 (±269,274). Mean of ET (mm) at 3 months (4.05 ± 1.40), 6 months (5.46 ± 1.36) and 9 months (5.48 ± 1.14) were significantly (P < 0.05) increased from pretreatment level (1.38 ± 0.39). Five out of six patients resumed menstruation. CONCLUSION: The autologous stem cell implantation leads to endometrial regeneration reflected by restoration of menstruation in five out of six cases. Autologous stem cell implantation is a promising novel cell based therapy for refractory AS. PMID:25191021

Singh, Neeta; Mohanty, Sujata; Seth, Tulika; Shankar, Meenakshi; Bhaskaran, Sruthi; Dharmendra, Sona

2014-01-01

180

Multidisciplinary therapy reduces risk factors for metabolic syndrome in obese adolescents.  

PubMed

This study aims to assess the effects of a 16-week multidisciplinary program of obesity treatment on the control of metabolic syndrome (MS) and dyslipidemia in obese adolescents. Eighty-six adolescents aged 10-18 years were allocated in either the intervention group (IG; n?=?44) or control group (CG; n?=?42). IG was submitted to a multidisciplinary intervention based on cognitive behavioral therapy that aimed to modify eating habits and exercise behavior. We analyzed, before and after the intervention period, anthropometric parameters, body composition, bone mineral density, cardiorespiratory fitness, blood pressure, glucose, insulin, and lipid profile of the subjects. MS was classified according to International Diabetes Federation (2007) and the presence of dyslipidemia according to Back et al. (Arq Bras Cardiol 85:4-36, 2005). In the beginning of the intervention, the median number (range) of risk factors for MS present was 2.0 (0.0-5.0) in the IG and 2.0 (0.0-4.0) in the CG. After the intervention, this parameter reduced significantly in the IG (1.0 (0.0-5.0); p?=?0.004) while no change was observed in the CG (2.0 (0.0-4.0); p?=?0.349). In addition, we observed improvements in body mass index, waist circumference, hip circumference, maximal oxygen uptake, absolute and relative body fat, systolic blood pressure, diastolic blood pressure, and total cholesterol in the IG which was not identified in the CG. Conclusio n: We suggest that a 16-week multidisciplinary intervention based on cognitive behavioral therapy was adequate to reduce risk factors for MS in obese adolescents. PMID:23097084

Bianchini, Josiane Aparecida Alves; da Silva, Danilo Fernandes; Nardo, Claudia Christina Sanchez; Carolino, Idalina Diair Regla; Hernandes, Florencio; Nardo, Nelson

2013-02-01

181

N-acetylcysteine advancement of surfactant therapy in experimental meconium aspiration syndrome: possible mechanisms.  

PubMed

Meconium aspiration syndrome (MAS) is meconium-induced respiratory failure of newborns associated with activation of inflammatory and oxidative pathways. For severe MAS, exogenous surfactant treatment is used which improves respiratory functions but does not treat the inflammation. Oxidative process can lead to later surfactant inactivation; hence, surfactant combination with antioxidative agent may enhance the therapeutic effect. Young New Zealand rabbits were instilled by meconium suspension and treated by surfactant alone, N-acetylcysteine (NAC) alone or by their combination and oxygen-ventilated for 5 h. Blood samples were taken before and 30 min after meconium application and 30 min, 1, 3 and 5 h after the treatment for evaluating of oxidative damage, total leukocyte count, leukocyte differential count and respiratory parameters. Leukocyte differential was assessed also in bronchoalveolar lavage fluid. NAC alone had only mild therapeutic effect on MAS. However, the combination of NAC and surfactant facilitated rapid onset of therapeutic effect in respiratory parameters (oxygenation index, PaO(2)/FiO(2)) compared to surfactant alone and was the only treatment which prevented neutrophil migration into the lungs, oxidative damage and lung edema. Moreover, NAC suppressed IL-8 and IL-beta formation and thus seems to be favorable agent for improving surfactant therapy in MAS. PMID:25669694

Kopincová, J; Mokrá, D; Mikolka, P; Kolomazník, M; ?alkovská, A

2015-02-10

182

Comparison of efficacy of kinesiological taping and subacromial injection therapy in subacromial impingement syndrome.  

PubMed

The aim of the study was to compare the efficacy of kinesiological taping and subacromial injection therapy in patients with subacromial impingement syndrome (SIS). Seventy patients diagnosed with SIS were randomly assigned to group 1 (n?=?35, injection group) or group 2 (n?=?35, kinesiological taping group). Betamethasone plus prilocaine was injected to subacromial space in the patients in group 1. In group 2, tape was applied three times for a period of five consecutive days with a 2-day recovery interval. A 3-month exercise program was prescribed for both groups including stretching and strengthening exercises. All patients were assessed at baseline and at 1 and 3 months post-intervention. Assessments were made by visual analog scale (VAS) for pain, range of motion (ROM) measurements, specific tests, and Shoulder Pain and Disability Index (SPADI). Significant differences were detected in VAS and SPADI scores as well as ROM measurements in both groups when compared to baseline (p?>?0.05). No significant differences were detected between the groups except for active flexion degree in favor of group 1 (p?=?0.004). Both kinesiological taping and steroid injection in conjunction with an exercise program were found to be effective in the treatment of SIS. Kinesio taping may be an alternative treatment option in the rehabilitation of SIS especially when a non-invasive technique is needed. PMID:25403253

Suba??, Volkan; Cak?r, Tuncay; Ar?ca, Zuhal; Sar?er, Rahime Nur; Bilgilisoy Filiz, Meral; Kolda? Do?an, Sebnem; Toraman, Naciye Füsun

2014-11-18

183

Results of topotecan-based combination therapy in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia.  

PubMed

The efficacy and safety of topotecan and high-dose cytarabine were evaluated in 59 patients with advanced myelodysplastic syndromes (MDS) (n = 38) or chronic myelomonocytic leukemia (CMML) (n = 21). Topotecan 1.25 mg/m2/d was administered by continuous Intravenous infusion daily for 5 days, and cytarabine 1.0g/m2/d by Infusion over 2 hours for 5 days. At a median follow-up duration of 7 months, 59 patients were evaluable for response and toxicity. Complete remission (CR) was achieved in a significantly larger proportion of MDS patients than CMML patients (66% v 48%, P < or = .05). CR rates for good-risk and poor-risk MDS patients were similar (79% and 58%, respectively). Treatment was particularly effective in patients with poor-prognosis karyotypes and secondary MDS, producing CR rates of 63% and 69%, respectively. Medlan duration of CR was 32 weeks (41 weeks for MDS; 33 weeks for CMML). Median survival was 60 weeks for MDS patients and 41 weeks for CMML patients. Prospective analysis of response by International Prognostic Scoring System (IPSS) risk classification showed comparable CR rates among intermediate 1 (60%), Intermediate 2 (83%), and high-risk (56%) categories. Fever of undetermined origin and Infections occurred in 72% and 59% of patients, respectively. Six patients (10%) died during Induction therapy. This regimen was well tolerated and was associated with a low mortality rate. PMID:10622223

Beran, M; Kantarjian, H

1999-10-01

184

Conservative treatment of carpal tunnel syndrome: Comparison between laser therapy and fascial manipulation(®).  

PubMed

The etiopathogenesis of Carpal Tunnel Syndrome (CTS) is multifactorial and most cases are classified as idiopathic (Thurston 2013). A randomized controlled trial was performed to compare the effectiveness of Fascial Manipulation(®) (FM) and Low-Level Laser Therapy (LLLT) for CTS. This prospective trial included 42 patients (70 hands with symptoms) with clinical and electroneuromyographic diagnosis of CTS. The patients were randomly assigned to receive multiple sessions of FM or multiple session of LLLT. The Visual Analogic Scale (VAS) and Boston Carpal Tunnel Questionnaire (BCTQ) were performed at baseline, end of treatment and after three months. The group that received FM showed a significant reduction in subjective pain perception and an increased function assessed by BCTQ at the end of the treatment and follow-up. The group that received LLLT showed an improvement in the BCTQ at the end of the treatment but the improvement level was not sustained at the three month follow-up. FM is a valid alternative treatment for CTS. PMID:25603750

Pratelli, Elisa; Pintucci, Marco; Cultrera, Pina; Baldini, Enrico; Stecco, Antonio; Petrocelli, Antonio; Pasquetti, Pietro

2015-01-01

185

Effectiveness of extracorporeal shock wave therapy in bone marrow edema syndrome of the hip.  

PubMed

There is no gold standard for treatment of bone marrow edema syndrome of the hip (BMESH). Usually, treatment is conservative, owing to the favorable and self limiting prognosis. In musculoskeletal disorders, the effectiveness of extracorporeal shock wave therapy (ESWT) has been widely recognized and recent research supports its use in the treatment of the first stages of avascular osteonecrosis of the proximal femur and in other conditions where bone marrow edema is present. On this basis, we performed a prospective study to evaluate the effectiveness of ESWT in normalizing the symptoms and imaging features of BMESH. Twenty consecutive symptomatic patients underwent two treatments of high-energy ESWT and were followed-up at 2, 3 and 6 months, with a final clinical follow-up at mean 15.52 ± 1.91 months. Patients underwent magnetic resonance imaging of the hip and were evaluated according to the Harris hip score. The mean improvement in HHS over the course of the study was of 58.5 ± 14.9 points (p < 0.0001), and the mean edema area reduced from 981.9 ± 453.2 mm(2) pre-treatment to 107.8 ± 248.1 mm(2) at 6 months. ESWT seems to be a powerful, non-pharmacological tool that produces rapid pain relief and functional improvement and aids the normalization of the vascular and metabolic impairments which characterize BMESH. PMID:24658812

d'Agostino, Cristina; Romeo, Pietro; Lavanga, Vito; Pisani, Salvatore; Sansone, Valerio

2014-11-01

186

Paraoxonase responses to exercise and niacin therapy in men with metabolic syndrome.  

PubMed

Our purpose was to characterize changes in paraoxonase 1 (PON1) activity and concentration after single aerobic exercise sessions conducted before and after 6 weeks of niacin therapy in men with metabolic syndrome (MetS). Twelve men with MetS expended 500 kcal by walking at 65% of VO2max before and after a 6-week regimen of niacin. Niacin doses were titrated by 500 mg/week from 500 to 1500 mg/day and maintained at 1500 mg/day for the last 4 weeks. Fasting blood samples were collected before and 24 hours after each exercise session and analyzed for PON1 activity, PON1 concentration, myeloperoxidase (MPO), apolipoprotein A1, oxidized low-density lipoprotein (oLDL), lipoprotein particle sizes and concentrations. PON1 activity, PON1 concentration, MPO, and oLDL were unaltered following the independent effects of exercise and niacin (P > 0.05 for all). High-density lipoprotein particle size decreased by 3% (P = 0.040) and concentrations of small very low-density lipoprotein increased (P = 0.016) following exercise. PON1 activity increased 6.1% (P = 0.037) and PON1 concentrations increased 11.3% (P = 0.015) with the combination of exercise and niacin. Exercise and niacin works synergistically to increase PON1 activity and concentration with little or no changes in lipoproteins or markers of lipid oxidation. PMID:25180827

Taylor, James Kyle; Plaisance, Eric P; Mahurin, A Jack; Mestek, Michael L; Moncada-Jimenez, Jose; Grandjean, Peter W

2015-01-01

187

Gene therapy for Wiskott-Aldrich syndrome--long-term efficacy and genotoxicity.  

PubMed

Wiskott-Aldrich syndrome (WAS) is characterized by microthrombocytopenia, immunodeficiency, autoimmunity, and susceptibility to malignancies. In our hematopoietic stem cell gene therapy (GT) trial using a ?-retroviral vector, 9 of 10 patients showed sustained engraftment and correction of WAS protein (WASP) expression in lymphoid and myeloid cells and platelets. GT resulted in partial or complete resolution of immunodeficiency, autoimmunity, and bleeding diathesis. Analysis of retroviral insertion sites revealed >140,000 unambiguous integration sites and a polyclonal pattern of hematopoiesis in all patients early after GT. Seven patients developed acute leukemia [one acute myeloid leukemia (AML), four T cell acute lymphoblastic leukemia (T-ALL), and two primary T-ALL with secondary AML associated with a dominant clone with vector integration at the LMO2 (six T-ALL), MDS1 (two AML), or MN1 (one AML) locus]. Cytogenetic analysis revealed additional genetic alterations such as chromosomal translocations. This study shows that hematopoietic stem cell GT for WAS is feasible and effective, but the use of ?-retroviral vectors is associated with a substantial risk of leukemogenesis. PMID:24622513

Braun, Christian Jörg; Boztug, Kaan; Paruzynski, Anna; Witzel, Maximilian; Schwarzer, Adrian; Rothe, Michael; Modlich, Ute; Beier, Rita; Göhring, Gudrun; Steinemann, Doris; Fronza, Raffaele; Ball, Claudia Regina; Haemmerle, Reinhard; Naundorf, Sonja; Kühlcke, Klaus; Rose, Martina; Fraser, Chris; Mathias, Liesl; Ferrari, Rudolf; Abboud, Miguel R; Al-Herz, Waleed; Kondratenko, Irina; Maródi, László; Glimm, Hanno; Schlegelberger, Brigitte; Schambach, Axel; Albert, Michael Heinrich; Schmidt, Manfred; von Kalle, Christof; Klein, Christoph

2014-03-12

188

Long versus standard initial prednisolone therapy in children with idiopathic nephrotic syndrome.  

PubMed

The efficacy of longer initial course of prednisolone with standard regimen was compared in children with idiopathic nephrotic syndrome (INS). It was a randomized controlled trial in which patients with INS who responded to initial course of prednisolone, either long or standard regimen were included and followed for one year after completion of treatment of first attack. The long regimen consisted of prednisolone 60mg/m˛/day for six weeks followed by 40mg/m˛ every alternate day single morning dose for further six weeks. The standard regimen consisted of prednisolone 60mg/m˛/day for four weeks followed by 40mg/m˛ every alternate day single morning dose for further four weeks. There were 93 children who fulfilled the criteria of the study, 47 from long group and 46 from standard group though ultimately 72 completed the study (41 from long and 31 from standard group). The primary outcome measure was relapse within next one year. Relapse within one year was noted in 73.2% of long and 64.5% of standard group. The odds ratio for relapse within one year was 0.80 (95% confidence interval 0.22, 2.05). This did not reach statistical significance (p=0.696). Adverse effects of prednisolone between two groups were also not statistically significant (p>0.05). Prolongation of prednisolone therapy for initial episode of steroid-sensitive INS does not have a beneficial effect on the outcome in next one year. PMID:24858152

Paul, S K; Muinuddin, G; Jahan, S; Begum, A; Rahman, M H; Hossain, M M

2014-04-01

189

ALERTEES PAR LA MORT CELLULAIRE, NOS DEFENSES ANTIVIRALES METTENT LE TURBO  

E-print Network

MORT CELLULAIRE, NOS DEFENSES ANTIVIRALES METTENT LE TURBO Genève, le 9 février 2012 SOUS EMBARGO JUSQU marche après que certaines cellules ont été détruites par l'ennemi viral. A leur mort, elles relâchent associated molecular pattern). Ces DAMP se distinguent du reste des débris de la cellule morte par leur

Loewith, Robbie

190

LEGO[R] Therapy and the Social Use of Language Programme: An Evaluation of Two Social Skills Interventions for Children with High Functioning Autism and Asperger Syndrome  

ERIC Educational Resources Information Center

LEGO[R] therapy and the Social Use of Language Programme (SULP) were evaluated as social skills interventions for 6-11 year olds with high functioning autism and Asperger Syndrome. Children were matched on CA, IQ, and autistic symptoms before being randomly assigned to LEGO or SULP. Therapy occurred for 1 h/week over 18 weeks. A no-intervention…

Owens, Gina; Granader, Yael; Humphrey, Ayla; Baron-Cohen, Simon

2008-01-01

191

Sjogrens syndrome as failed local immunohomeostasis: prospects for cell-based therapy.  

PubMed

Sjogrens syndrome has been estimated to affect between 0.2% and 2% or more of the population. It is an autoimmune disease with the hallmark histopathology of focal, periductal, and perivascular CD4(+) cell infiltration of the lacrimal and salivary glands. The immunohistopathology is typically associated with severe lacrimal and salivary dysfunctions, which contribute to debilitating ocular surface and oral symptoms. The quality of life of patients with Sjogrens syndrome often is degraded further by serious, multisystemic manifestations, and they are subject to a forty-fold increased risk of developing B cell lymphomas. In normal lacrimal glands, secretory epithelial cells, autoimmune effector lymphocytes, and regulatory lymphocytes can be seen as collaborating to maintain a local immunohomeostasis. The epithelium contributes by secreting immunomodulatory paracrine factors and also by continuously exposing autoantigens, which thereby become available for uptake by professional antigen presenting cells (APCs). Local or systemic perturbations may initiate autoimmune pathophysiology by impairing the replacement of normally-turning-over regulatory cells, by altering epithelial production of immunomodulatory paracrine factors, by inducing intact epithelial cells to begin secreting previously cryptic epitopes (epitopes that previously were not available to bind to major histocompatibility complex (MHC) molecules and so could not be recognized by T cell antigen receptors), and by inducing epithelial cells to begin expressing MHC Class II molecules and presenting formerly cryptic epitopes directly to CD4(+) cells. This process has been modeled ex vivo with mixed cell reactions comprised of isolated epithelial cells and autologous lymphocytes. This development has occurred as studies of anterior chamber-associated immune deviation (ACAID) and other immunoregulatory phenomena have elucidated the origins and functions of several different kinds of regulatory lymphocytes and shown that regulatory lymphocytes can be generated ex vivo. It now is possible to envision strategies for exploiting each possible mode of epithelial autoantigen exposure to produce therapeutic regulatory cells that might be capable of re-establishing normal immunohomeostasis. Consideration of the hypothetical therapies identifies a number of basic questions that warrant investigation. PMID:17075648

Mircheff, Austin K

2003-10-01

192

[Equine Cushing syndrome (ECS). Case report, review of its diagnosis and therapy and substantial differences from Cushing syndrome in dogs].  

PubMed

Equine and canine Cushing's syndrome, both of which are the result of elevated cortisol levels, show some different pathogenetical and clinical features and require different therapeutical approaches. In older horses the equine Cushing's syndrome (ECS) is not uncommon. Nearly all cases result from excessive hormone production in cells of the pars intermedia of the pituitary. Besides elevated levels of adrenocorticotrope hormone (ACTH), high peripheral levels of pro-opiomelanocortin, beta-endorphines and alpha-melanocyte-stimulating hormone can be measured. In middle-aged and geriatric dogs, Cushing's syndrome is the most frequently diagnosed endocrinologic abnormality. 80-85% of cases are pituitary-dependent and 15-20% are caused by cortisol producing tumors of the adrenals. 90% of pituitary lesions can be identified as adenomas, which are localised in most cases in the pars distalis of the gland, but may occur rarely in the pars intermedia, too. Clinical symptoms in both species are characterised by wasting despite good appetite or polyphagia, reduction of muscle mass with altered fat deposition and lethargy. Whereas polydipsia/polyuria is a very common feature in dogs with Cushing's syndrome, in horses it is almost invariably a sign of concurrent secondary diabetes mellitus. A typical symptom in ECS is a continuously growing haircoat (hirsutism), whereas in canine Cushing's syndrome generalised alopecia may bring the owner to consult a veterinarian. The symptoms and diagnostic procedures in a 33-year-old mare are described. Useful diagnostic tests are reviewed with special attention to species differences in reacting to them. The therapeutic approach with dopamine-agonists such as bromocriptine and pergolide as well as cyproheptadine to ECS is reviewed. PMID:9626747

Fey, K; Jonigkeit, E; Moritz, A

1998-02-01

193

Targeted therapy of short-bowel syndrome with teduglutide: the new kid on the block  

PubMed Central

Extensive intestinal resection impairs the absorptive capacity and results in short-bowel syndrome-associated intestinal failure (SBS-IF), when fluid, electrolyte, acid-base, micro-, and macronutrient homeostasis cannot be maintained on a conventional oral diet. Several factors, including the length and site of the resected intestine, anatomical conformation of the remnant bowel, and the degree of postresection intestinal adaptation determine the disease severity. While mild SBS patients achieve nutritional autonomy with dietary modification (eg, hyperphagia, small frequent meals, and oral rehydration fluids), those with moderate-to-severe disease may develop SBS-IF and become dependent on parenteral support (PS) in the form of intravenous fluids and/or nutrition for sustenance of life. SBS-IF is a chronic debilitating disease associated with a poor quality of life, and carries significant morbidity and health care costs. Medical management of SBS-IF is primarily focused on individually tailored symptomatic treatment strategies, such as antisecretory and antidiarrheal agents to mitigate fluid losses, and PS. However, PS administration is associated with potentially life-threatening complications, such as central venous thromboses, bloodstream infections, and liver disease. In pursuit of a targeted therapy to augment intestinal adaptation, research over the past 2 decades has identified glucagon-like peptide, an intestinotrophic gut peptide that has been shown to enhance intestinal absorptive capacity by causing an increase in the villus length, crypt depth, and mesenteric blood flow and by decreasing gastrointestinal motility and secretions. Teduglutide, a recombinant analog of glucagon-like peptide-2, is the first targeted therapeutic agent to gain approval for use in adult SBS-IF. Teduglutide was shown to result in significant (20%–100%) reduction in PS-volume requirement and have a satisfactory safety profile in three randomized control trials. Further research is warranted to see if reduction in PS dependency translates to improved quality of life and reduced PS-associated complications. PMID:25525380

Vipperla, Kishore; O’Keefe, Stephen J

2014-01-01

194

Successful treatment of myelodysplastic syndrome and chronic hepatitis C using combined peginterferon-?-2b and ribavirin therapy.  

PubMed

We report a patient with myelodysplastic syndrome (MDS) and hepatitis C virus (HCV) infection who was successfully treated with a combination of peginterferon and ribavirin therapy. A 65-year-old man was referred to our hospital for treatment of chronic hepatitis C and close examination of pancytopenia. MDS of "refractory cytopenia with multilineage dysplasia" was diagnosed on the basis of bone marrow findings. Although the patient was not a good candidate for interferon (IFN) therapy because of his pancytopenia, we decided to proceed with IFN therapy for the following reasons: his elevated transaminases could not be controlled; he had a high possibility of recovery from chronic hepatitis C in consideration of his HCV genotype 2a and relatively low RNA titer; and his pancytopenia was expected to worsen in the future. After combination peginterferon/ribavirin therapy, the patient achieved sustained viral response, and the bone marrow findings showed neutrophils with normal granulation and megakaryocytes with normal morphological features. Additionally, the normal 46, XY karyotype converted from 45, X0 which was found before IFN therapy. This suggested that the patient's MDS was completely resolved. PMID:24224981

Maruyama, Shigeo; Koda, Masahiko; Oi, Shinnji; Murawaki, Yoshikazu

2014-10-01

195

Optimal In-Hospital and Discharge Medical Therapy in Acute Coronary Syndromes in Kerala: Results from the Kerala ACS Registry  

PubMed Central

Background In-hospital and post-discharge treatment rates for acute coronary syndrome (ACS) remain low in India. However, little is known about the prevalence and predictors of the package of optimal ACS medical care in India. Our objective was to define the prevalence, predictors, and impact of optimal in-hospital and discharge medical therapy in the Kerala ACS Registry of 25,718 admissions. Methods and Results We defined optimal in-hospital ACS medical therapy as receiving the following five medications: aspirin, clopidogrel, heparin, beta-blocker, and statin. We defined optimal discharge ACS medical therapy as receiving all of the above therapies except heparin. Comparisons by optimal vs. non-optimal ACS care were made via Student’s t test for continuous variables and chi-square test for categorical variables. We created random effects logistic regression models to evaluate the association between GRACE risk score variables and optimal in-hospital or discharge medical therapy. Optimal in-hospital and discharge medical care was delivered in 40% and 46% of admissions, respectively. Wide variability in both in-hospital and discharge medical care was present with few hospitals reaching consistently high (>90%) levels. Patients receiving optimal in-hospital medical therapy had an adjusted OR (95%CI)=0.93 (0.71, 1.22) for in-hospital death and an adjusted OR (95%CI)=0.79 (0.63, 0.99) for MACE. Patients who received optimal in-hospital medical care were far more likely to receive optimal discharge care (adjusted OR [95%CI]=10.48 [9.37, 11.72]). Conclusions Strategies to improve in-hospital and discharge medical therapy are needed to improve local process-of-care measures and improve ACS outcomes in Kerala. PMID:23800985

Huffman, Mark D; Prabhakaran, Dorairaj; Abraham, AK; Krishnan, Mangalath Narayanan; Nambiar, C. Asokan; Mohanan, Padinhare Purayil

2013-01-01

196

Cellules souches et thérapie cellulaire. Contribution au débat éthique  

Microsoft Academic Search

Divergent and sometimes conflicting positions with respect to human stem cells and cell therapy do not merely reflect disagreement among scientists and conflicts of interest. They attest the ethical tension resulting from recent progress in understanding the earliest stages of development of the human being that can be observed in vitro. Can the extremely potent notion of the human person

Denys Pellerin

2002-01-01

197

Association cellules–matériaux pour thérapies cellulaires osseuses  

Microsoft Academic Search

Although autologous or allogenous bone grafting materials are widely used to repair skeletal tissue, their limitations induce surgeons to look for more advanced therapies such as tissue engineering. However, bone tissue engineering still lacks proofs of clinical efficacy, possibly due to the inadequate design of the tissue-engineered constructs in relation to the natural tissue. Our aim is therefore to design

G. Daculsi; S. Baroth; L. Sensebé; P. Rosset; M. Durand; O. Boisteau; P. Layrolle

2011-01-01

198

The antiphospholipid syndrome as a disorder initiated by inflammation: implications for the therapy of pregnant patients  

Microsoft Academic Search

Arterial thrombosis, venous thrombosis and morbidity during pregnancy, or a combination of these events, are clinical outcomes associated with antiphospholipid antibodies produced by patients with antiphospholipid syndrome (APS). Our understanding of the etiology and pathogenesis of the syndrome is limited, but it has generally been considered a thrombophilic disease and treatment has focused on anticoagulation. Agents such as aspirin and

Guillermina Girardi; Michael D Lockshin; Jane E Salmon

2007-01-01

199

von Hippel-Lindau Syndrome: Target for Anti-Vascular Endothelial Growth Factor (VEGF) Receptor Therapy  

Microsoft Academic Search

von Hippel-Lindau (VHL) syndrome is a familial cancer syndrome caused by germline mutations in the VHL tumor suppressor gene. Mutations in the VHL gene result in the constitutive stabilization of transcrip- tion factors hypoxia-inducible factors 1? and 2?, which bind to specific enhancer elements in the vascular endothelial growth factor (VEGF) gene and stimulate angiogenesis. This increase in angiogenesis under

ADRIAN L. HARRIS

200

Cost-effectiveness of internet-based cognitive behavior therapy for irritable bowel syndrome: results from a randomized controlled trial  

Microsoft Academic Search

Background  Irritable Bowel Syndrome (IBS) is highly prevalent and is associated with a substantial economic burden. Cognitive behavior\\u000a therapy (CBT) has been shown to be effective in treating IBS. The aim of this study was to evaluate the cost-effectiveness\\u000a of a new treatment alternative, internet-delivered CBT based on exposure and mindfulness exercises.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  Participants (N = 85) with IBS were recruited through

Erik Andersson; Brjánn Ljótsson; Filip Smit; Björn Paxling; Erik Hedman; Nils Lindefors; Gerhard Andersson; Christian Rück

2011-01-01

201

Exercise therapy and cognitive behavioural therapy to improve fatigue, daily activity performance and quality of life in Postpoliomyelitis Syndrome: the protocol of the FACTS-2-PPS trial  

PubMed Central

Background Postpoliomyelitis Syndrome (PPS) is a complex of late onset neuromuscular symptoms with new or increased muscle weakness and muscle fatigability as key symptoms. Main clinical complaints are severe fatigue, deterioration in functional abilities and health related quality of life. Rehabilitation management is the mainstay of treatment. Two different therapeutic interventions may be prescribed (1) exercise therapy or (2) cognitive behavioural therapy (CBT). However, the evidence on the effectiveness of both interventions is limited. The primary aim of the FACTS-2-PPS trial is to study the efficacy of exercise therapy and CBT for reducing fatigue and improving activities and quality of life in patients with PPS. Additionally, the working mechanisms, patients' and therapists' expectations of and experiences with both interventions and cost-effectiveness will be evaluated. Methods/Design A multi-centre, single-blinded, randomized controlled trial will be conducted. A sample of 81 severely fatigued patients with PPS will be recruited from 3 different university hospitals and their affiliate rehabilitation centres. Patients will be randomized to one of three groups i.e. (1) exercise therapy + usual care, (2) CBT + usual care, (3) usual care. At baseline, immediately post-intervention and at 3- and 6-months follow-up, fatigue, activities, quality of life and secondary outcomes will be assessed. Costs will be based on a cost questionnaire, and statistical analyses on GEE (generalized estimated equations). Analysis will also consider mechanisms of change during therapy. A responsive evaluation will be conducted to monitor the implementation process and to investigate the perspectives of patients and therapists on both interventions. Discussion A major strength of the FACTS-2-PPS study is the use of a mixed methods design in which a responsive and economic evaluation runs parallel to the trial. The results of this study will generate new evidence for the rehabilitation treatment of persons with PPS. Trial registration Dutch Trial Register NTR1371. PMID:20082714

2010-01-01

202

The effectiveness and cost evaluation of pain exposure physical therapy and conventional therapy in patients with complex regional pain syndrome type 1. Rationale and design of a randomized controlled trial  

PubMed Central

Background Pain Exposure Physical Therapy is a new treatment option for patients with Complex Regional Pain Syndrome type 1. It has been evaluated in retrospective as well as in prospective studies and proven to be safe and possibly effective. This indicates that Pain Exposure Physical Therapy is now ready for clinical evaluation. The results of an earlier performed pilot study with an n?=?1 design, in which 20 patients with Complex Regional Pain Syndrome type 1 were treated with Pain Exposure Physical Therapy, were used for the design and power calculation of the present study. After completion and evaluation of this phase III study, a multi-centre implementation study will be conducted. The aim of this study is to determine whether Pain Exposure Physical Therapy can improve functional outcomes in patients with Complex Regional Pain Syndrome type 1. Methods/design This study is designed as a single-blinded, randomized clinical trial. 62 patients will be randomized with a follow-up of 9?months to demonstrate the expected treatment effect. Complex Regional Pain Syndrome type 1 is diagnosed in accordance with the Bruehl/International Association for the Study of Pain criteria. Conventional therapy in accordance with the Dutch guideline will be compared with Pain Exposure Physical Therapy. Primary outcome measure is the Impairment level SumScore, restricted version. Discussion This is the first randomized controlled study with single blinding that has ever been planned in patients with Complex Regional Pain Syndrome type 1 and does not focus on a single aspect of the pain syndrome but compares treatment strategies based on completely different pathophysiological and cognitive theories. Trial registration Clinical trials NCT00817128; National Trial Register NTR2090 PMID:22515496

2012-01-01

203

Alport syndrome from bench to bedside: the potential of current treatment beyond RAAS blockade and the horizon of future therapies.  

PubMed

The hereditary type IV collagen disease Alport syndrome (AS) always leads to end-stage renal failure. Yesterday, for the past 90 years, this course was described as 'inevitable'. Today, RAAS blockade has changed the 'inevitable' course to a treatable disease. Tomorrow, researchers hope to erase the 'always' from 'always leads to renal failure' in the textbooks. This review elucidates therapeutic targets that evolve from research: (i) kidney embryogenesis and pathogenesis; (ii) phenotype-genotype correlation and the role of collagen receptors and podocytes; (iii) the malfunctioning Alport-GBM; (iv) tubulointerstitial fibrosis; (v) the role of proteinuria in pathogenesis and prognosis; and (vi) secondary events such as infections, hyperparathyroidism and hypercholesterolaemia. Therefore, moderate lifestyle, therapy of bacterial infections, Paricalcitol in adult patients with hyperparathyroidism and HMG-CoA-reductase inhibitors in adult patients with dyslipoproteinemia might contribute to a slower progression of AS and less cardiovascular events. In the future, upcoming treatments including stem cells, chaperon therapy, collagen receptor blockade and anti-microRNA therapy will expand our perspective in protecting the kidneys of Alport patients from further damage. This perspective on current and future therapies is naturally limited by our personal focus in research, but aims to motivate young scientists and clinicians to find a multimodal cure for AS. PMID:25165179

Gross, Oliver; Perin, Laura; Deltas, Constantinos

2014-09-01

204

Cytokine release syndrome after blinatumomab treatment related to abnormal macrophage activation and ameliorated with cytokine-directed therapy  

PubMed Central

Blinatumomab is a CD19/CD3-bispecific T-cell receptor-engaging (BiTE) antibody with efficacy in refractory B-precursor acute lymphoblastic leukemia. Some patients treated with blinatumomab and other T cell-activating therapies develop cytokine release syndrome (CRS). We hypothesized that patients with more severe toxicity may experience abnormal macrophage activation triggered by the release of cytokines by T-cell receptor–activated cytotoxic T cells engaged by BiTE antibodies and leading to hemophagocytic lymphohistiocytosis (HLH). We prospectively monitored a patient during blinatumomab treatment and observed that he developed HLH. He became ill 36 hours into the infusion with fever, respiratory failure, and circulatory collapse. He developed hyperferritinemia, cytopenias, hypofibrinogenemia, and a cytokine profile diagnostic for HLH. The HLH continued to progress after discontinuation of blinatumomab; however, he had rapid improvement after IL-6 receptor-directed therapy with tocilizumab. Patients treated with T cell-activating therapies, including blinatumomab, should be monitored for HLH, and cytokine-directed therapy may be considered in cases of life-threatening CRS. This trial was registered at www.clinicaltrials.gov as #NCT00103285. PMID:23678006

Rheingold, Susan R.; Maude, Shannon L.; Zugmaier, Gerhard; Barrett, David M.; Seif, Alix E.; Nichols, Kim E.; Suppa, Erica K.; Kalos, Michael; Berg, Robert A.; Fitzgerald, Julie C.; Aplenc, Richard; Gore, Lia; Grupp, Stephan A.

2013-01-01

205

Combination therapy for segmental craniocervical dystonia (Meige syndrome) with aripiprazole, trihexyphenidyl, and botulinum toxin: three cases reports.  

PubMed

Segmental craniocervical dystonia is characterized by blephalospasm and oromandibular dystonia and is also called Meige syndrome. The current treatment strategy including botulinum toxin (BTX) injections has not yet attained an acceptable level. We describe a long-term favorable response of a novel combination therapy with aripiprazole (ARP), trihexyphenidyl (THP), and BTX in three patients with segmental craniocervical dystonia. The symptoms of three patients responded promptly to the combination therapy with ARP 3-6 mg daily, THP 2-8 mg daily, and BTX. Although the patients were required to receive a BTX 50-100 IU injection every 3-6 months, their symptoms were kept in a satisfactory condition for up to 2 years without any adverse effects. ARP possesses the potential for dramatically improving dystonia. THP has the possibility to enhance the efficacy of ARP and prolong the effective period of BTX. It may be an important requisite to give all three agents together for a successful treatment. The combination therapy with ARP, THP, and BTX was well-tolerated and useful in controlling the symptoms of segmental craniocervical dystonia, however, the reason why this combination therapy succeeded is unknown. A further long-term follow-up is required to monitor the delayed neurological adverse effects. PMID:25151364

Saito, Tsukasa; Katayama, Takayuki; Sawada, Jun; Kano, Kohei; Asanome, Asuka; Takahashi, Kae; Sato, Nobuyuki; Hasebe, Naoyuki

2015-02-01

206

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is not always completely reversible: a case series.  

PubMed

We retrospectively reviewed clinical and imaging findings in 11 patients with stroke-like migraine attacks after radiation therapy (SMART) syndrome to better understand this disorder previously thought to be reversible. Six men and 5 women had complex bouts of neurologic impairment beginning, on average, 20 years after cerebral irradiation. All had characteristic, unilateral gyriform enhancement on MR imaging that developed within 2-7 days and typically resolved in 2-5 weeks. Unlike prior reports, 45% had incomplete neurologic recovery manifesting as dysphasia, cognitive impairment, or hemiparesis. The remaining 55% recovered completely over an average of 2 months. Three of 11 patients developed cortical laminar necrosis. Brain biopsies in 4 of 11 did not demonstrate a specific pathologic substrate. These additional 11 patients contribute to the understanding of variability in stroke-like migraine attacks after radiation therapy syndrome, which often but not uniformly manifests with headaches and seizures, demonstrates a typical evolution of imaging findings, and may result in permanent neurologic and imaging sequelae. PMID:23788601

Black, D F; Morris, J M; Lindell, E P; Krecke, K N; Worrell, G A; Bartleson, J D; Lachance, D H

2013-12-01

207

Prediction of response to therapy with ezatiostat in lower risk myelodysplastic syndrome  

E-print Network

Background: Approximately 70% of all patients with myelodysplastic syndrome (MDS) present with lower-risk disease. Some of these patients will initially respond to treatment with growth factors to improve anemia but will ...

Galili, Naomi

208

Therapy Insight: cancer anorexia–cachexia syndrome—when all you can eat is yourself  

Microsoft Academic Search

Tumor growth is associated with profound metabolic and neurochemical alterations, which can lead to the onset of anorexia–cachexia syndrome. Anorexia is defined as the loss of the desire to eat, while cachexia results from progressive wasting of skeletal muscle mass—and to a lesser extent adipose tissue—occurring even before weight loss becomes apparent. Cancer anorexia–cachexia syndrome is highly prevalent among cancer

Michael M Meguid; Akio Inui; Maurizio Muscaritoli; Filippo Rossi-Fanelli; Alessandro Laviano

2005-01-01

209

Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy  

PubMed Central

We present a rare case of Stewart-Treves syndrome characterized by a diffuse angiosarcoma of the leg in a 22-year-old man with a history of chronic lymphedema due to Klippel-Trénaunay-Weber syndrome. He underwent limb disarticulation and medical treatment with cycles of doxorubicin, oral thalidomide and sunitinib with a very good response after 12 months of follow-up. PMID:20740188

Simas, Ângela; Matos, Catarina; Lopes da Silva, Rodrigo; Brotas, Vítor; Teófilo, Eugénio; Albino, José Pereira

2010-01-01

210

Treatment of Fibromyalgia Syndrome: Recommendations of Recent Evidence-Based Interdisciplinary Guidelines with Special Emphasis on Complementary and Alternative Therapies  

PubMed Central

Objective. Current evidence indicates that there is no single ideal treatment for fibromyalgia syndrome (FMS). First choice treatment options remain debatable, especially concerning the importance of complementary and alternative medicine (CAM) treatments. Methods. Three evidence-based interdisciplinary guidelines on FMS in Canada, Germany, and Israel were compared for their first choice and CAM-recommendations. Results. All three guidelines emphasized a patient-tailored approach according to the key symptoms. Aerobic exercise, cognitive behavioral therapy, and multicomponent therapy were first choice treatments. The guidelines differed in the grade of recommendation for drug treatment. Anticonvulsants (gabapentin, pregabalin) and serotonin noradrenaline reuptake inhibitors (duloxetine, milnacipran) were strongly recommended by the Canadian and the Israeli guidelines. These drugs received only a weak recommendation by the German guideline. In consideration of CAM-treatments, acupuncture, hypnosis/guided imagery, and Tai Chi were recommended by the German and Israeli guidelines. The Canadian guidelines did not recommend any CAM therapy. Discussion. Recent evidence-based interdisciplinary guidelines concur on the importance of treatment tailored to the individual patient and further emphasize the need of self-management strategies (exercise, and psychological techniques). PMID:24348701

Fitzcharles, Mary-Ann; Buskila, Dan; Shir, Yoram; Sommer, Claudia

2013-01-01

211

Treatment of fibromyalgia syndrome: recommendations of recent evidence-based interdisciplinary guidelines with special emphasis on complementary and alternative therapies.  

PubMed

Objective. Current evidence indicates that there is no single ideal treatment for fibromyalgia syndrome (FMS). First choice treatment options remain debatable, especially concerning the importance of complementary and alternative medicine (CAM) treatments. Methods. Three evidence-based interdisciplinary guidelines on FMS in Canada, Germany, and Israel were compared for their first choice and CAM-recommendations. Results. All three guidelines emphasized a patient-tailored approach according to the key symptoms. Aerobic exercise, cognitive behavioral therapy, and multicomponent therapy were first choice treatments. The guidelines differed in the grade of recommendation for drug treatment. Anticonvulsants (gabapentin, pregabalin) and serotonin noradrenaline reuptake inhibitors (duloxetine, milnacipran) were strongly recommended by the Canadian and the Israeli guidelines. These drugs received only a weak recommendation by the German guideline. In consideration of CAM-treatments, acupuncture, hypnosis/guided imagery, and Tai Chi were recommended by the German and Israeli guidelines. The Canadian guidelines did not recommend any CAM therapy. Discussion. Recent evidence-based interdisciplinary guidelines concur on the importance of treatment tailored to the individual patient and further emphasize the need of self-management strategies (exercise, and psychological techniques). PMID:24348701

Ablin, Jacob; Fitzcharles, Mary-Ann; Buskila, Dan; Shir, Yoram; Sommer, Claudia; Häuser, Winfried

2013-01-01

212

Outcome predictors affecting the efficacy of clonazepam therapy for the management of burning mouth syndrome (BMS)  

Microsoft Academic Search

BMS is a common condition characterized by chronic oral mucosal pain condition and primarily affects elderly women. Although clonazepam therapy has been widely used due to its efficacy, it is not always effective because of the complexity of BMS pathogenesis. In this study, we have investigated outcome predictors of clonazepam therapy in patients with BMS. One hundred patients with BMS

Ji-Young Ko; Moon-Jong Kim; Sang-Goo Lee; Hong-Seop Kho

213

Role of dynamic intraventricular obstruction and protective effect of beta-blocking therapy in left ventricular apical ballooning syndrome: a case report.  

PubMed

This paper reports on a 58-year old woman with left ventricular apical ballooning syndrome (LVABS), who after interruption of beta-blocking therapy had a recurrence of symptoms and developed a severe dynamic left ventricular obstruction (LV-DYN-O) during sympathetic stimulation with dobutamine. PMID:18036677

Panigada, Stefania; Camporotondo, Rita; Repetto, Alessandra; Previtali, Mario

2009-02-01

214

Diagnosis of Men-I Syndrome on (68)Ga-DOTANOC PET-CT and Role of Peptide Receptor Radionuclide Therapy With (177)Lu-DOTATATE.  

PubMed

MEN-I is a rare genetic disorder classically characterized by a predisposition to tumors of the parathyroid glands, anterior pituitary gland, and pancreatic islet cells. We present a case of MEN-I syndrome diagnosed using predominantly nuclear medicine imaging followed by radionuclide therapy, thus emphasizing the role of nuclear imaging in diagnosing and treating MEN-I. PMID:23843835

Kumar Gupta, Santosh; Singla, Suhas; Damle, Nishikant A; Agarwal, Krishankant; Bal, Chandersekhar

2012-01-01

215

Diagnosis of Men-I Syndrome on 68Ga-DOTANOC PET-CT and Role of Peptide Receptor Radionuclide Therapy With 177Lu-DOTATATE  

PubMed Central

Abstract MEN-I is a rare genetic disorder classically characterized by a predisposition to tumors of the parathyroid glands, anterior pituitary gland, and pancreatic islet cells. We present a case of MEN-I syndrome diagnosed using predominantly nuclear medicine imaging followed by radionuclide therapy, thus emphasizing the role of nuclear imaging in diagnosing and treating MEN-I. PMID:23843835

kumar Gupta, Santosh; Singla, Suhas; Damle, Nishikant A; Agarwal, Krishankant; Bal, Chandersekhar

2012-01-01

216

Randomised placebo controlled trial of daytime function after continuous positive airway pressure (CPAP) therapy for the sleep apnoea\\/hypopnoea syndrome  

Microsoft Academic Search

BACKGROUNDPatients with the sleep apnoea\\/hypopnoea syndrome (SAHS) report improved sleepiness, cognitive function, and psychological well being after continuous positive airway pressure (CPAP) therapy, and it is for these daytime features that CPAP is usually given. However, few randomised or controlled studies exist on the effects of CPAP on daytime function.METHODSA prospective, randomised, single blind, placebo controlled, crossover trial of daytime

Heather M Engleman; Sascha E Martin; Ruth N Kingshott; Thomas W Mackay; Ian J Deary; Neil J Douglas

1998-01-01

217

Investigation of the Syndrome of Apotemnophilia and Course of a Cognitive-Behavioural Therapy  

Microsoft Academic Search

Background: The syndrome of apotemnophilia, body integrity or amputee identity disorder, is defined as the desire for amputation of a healthy limb, and may be accompanied by behaviour of pretending to be an amputee and sometimes, but not necessarily, by sexual arousal. Sampling and Methods: A case history is presented of a 35-year-old man who was referred because of his

Arjan W. Braam; Sako Visser; Daniëlle C. Cath; W. J. G. Hoogendijk

2006-01-01

218

Down Syndrome: Sensory Integration, Vestibular Stimulation and Neurodevelopmental Therapy Ap proaches for Children  

Microsoft Academic Search

Down Syndrome is a disability characterized by significant limitations both in intellectual functioning and in adaptive behavior as expressed in conceptual, social, and practical adap- tive skills. There are various degrees of sensory integration dysfunctions in children with an intellectual disability. Sensory integration is the organization of sensory input for use. Func- tion of learning depends on the child's ability

Mine Uyanik; Hulya Kayihan

219

Specific Factors Predict the Response to Pulsatile Gonadotropin-Releasing Hormone Therapy in Polycystic Ovarian Syndrome  

Microsoft Academic Search

Ovulation induction is particularly challenging in patients with polycystic ovarian syndrome (PCOS) and may be complicated by mul- tifollicular development. Pulsatile GnRH stimulates monofollicular development in women with anovulatory infertility; however, ovula- tion rates are considerably lower in the subgroup of patients with PCOS. The aim of this retrospective study was to determine specific hormonal, metabolic, and ovarian morphological characteristics

SABRINA GILL; ANN E. TAYLOR; KATHRYN A. MARTIN; CORRINE K. WELT; JUDITH M. ADAMS; JANET E. HALL

2010-01-01

220

Growth hormone therapy of Turner's syndrome: Beneficial effect on adult height  

Microsoft Academic Search

Objective: To carry out a multicenter, prospective, randomized trial of human growth hormone (GH), alone or in combination with oxandrolone (OX), in patients with Turner's syndrome (TS). Methods: In an initial phase lasting 12 to 24 months, 70 girls with TS, verified by karyotype, were randomly assigned to one of four groups: (1) observation, (2) OX, (3) GH, or (4)

Ron G. Rosenfeld; Kenneth M. Attie; James Frane; Anne Brasel; Stephen Burstein; Jose F. Cara; Steven Chernausek; Ronald W. Gotlin; Joyce Kuntze; Barbara M. Lippe; C. Patrick Mahoney; Wayne V. Moore; Paul Saenger; Ann J. Johanson

1998-01-01

221

Towards a therapy for Angelman syndrome by targeting a long non-coding RNA.  

PubMed

Angelman syndrome is a single-gene disorder characterized by intellectual disability, developmental delay, behavioural uniqueness, speech impairment, seizures and ataxia. It is caused by maternal deficiency of the imprinted gene UBE3A, encoding an E3 ubiquitin ligase. All patients carry at least one copy of paternal UBE3A, which is intact but silenced by a nuclear-localized long non-coding RNA, UBE3A antisense transcript (UBE3A-ATS). Murine Ube3a-ATS reduction by either transcription termination or topoisomerase I inhibition has been shown to increase paternal Ube3a expression. Despite a clear understanding of the disease-causing event in Angelman syndrome and the potential to harness the intact paternal allele to correct the disease, no gene-specific treatment exists for patients. Here we developed a potential therapeutic intervention for Angelman syndrome by reducing Ube3a-ATS with antisense oligonucleotides (ASOs). ASO treatment achieved specific reduction of Ube3a-ATS and sustained unsilencing of paternal Ube3a in neurons in vitro and in vivo. Partial restoration of UBE3A protein in an Angelman syndrome mouse model ameliorated some cognitive deficits associated with the disease. Although additional studies of phenotypic correction are needed, we have developed a sequence-specific and clinically feasible method to activate expression of the paternal Ube3a allele. PMID:25470045

Meng, Linyan; Ward, Amanda J; Chun, Seung; Bennett, C Frank; Beaudet, Arthur L; Rigo, Frank

2015-02-19

222

Increased uptake of guideline-recommended oral antiplatelet therapy: insights from the canadian acute coronary syndrome reflective.  

PubMed

Current guideline-based recommendations for oral dual-antiplatelet therapy in an acute coronary syndrome (ACS) include the use of newer adenosine diphosphate receptor inhibitor (ADPri) regimens and agents. The Canadian ACS Reflective Program is a multicenter observational quality-enhancement project that compared the use of ADPri therapy in 2 phases (November 2011-March 2013 and April 2013-November 2013) and also compared ADPri use with previous national data from the Canadian Global Registry of Acute Coronary Events (2000-2008). Of 3099 patients with ACS, 30.6% had ST-segment elevation myocardial infarction (STEMI), 52.3% had non-STEMI, and 17% had unstable angina. There was high use of dual-antiplatelet therapy for ? 24 hours, with important increases noted when compared with previous national experience (P for trend, < 0.0001). Clopidogrel was the most commonly used ADPri (82.2%), with lower use of the newer agents ticagrelor (9.0%) and prasugrel (3.1%). Ticagrelor and prasugrel use was most frequent in patients with STEMI undergoing percutaneous coronary intervention PCI (34.3%). There was relatively lower use of ADPri therapy at discharge; it was given mainly to patients who did not undergo PCI (68.2%) and to those with non-ST-elevation ACS (82%). When comparing the 2 consecutive phases of data collection in the ACS Reflective, there was an approximate 3- and 2-fold increase in the early and discharge use of the newer ADPri agents, respectively. In conclusion, there has been a temporal increase in ADPri use compared with previous national experience and an increased uptake of newer ADPri agents. Additional work is needed to identify and address barriers limiting optimal implementation of these newer guideline-recommended agents into routine Canadian practice. PMID:25475475

Gandhi, Sumeet; Zile, Brigita; Tan, Mary K; Saranu, Jhansi; Bucci, Claudia; Yan, Andrew T; Robertson, Patrick; Quantz, Mackenzie A; Letovsky, Eric; Tanguay, Jean-Francois; Dery, Jean-Pierre; Fitchett, David; Madan, Mina; Cantor, Warren J; Heffernan, Michael; Natarajan, Madhu K; Wong, Graham C; Welsh, Robert C; Goodman, Shaun G

2014-12-01

223

Therapy-related myelodysplastic syndrome/acute leukemia after multiple myeloma in the era of novel agents.  

PubMed

Survival for patients with multiple myeloma has increased. Both melphalan and lenalidomide are associated with subsequent development of myelodysplasia. We reviewed the cases of all patients with multiple myeloma who had subsequent development of myelodysplastic syndrome (MDS) or acute non-lymphoblastic leukemia (ANLL) during a 12-year period in three centers. Of 55 patients identified, two received only lenalidomide before myelodysplasia developed. The median time between the diagnoses of multiple myeloma and MDS/ANLL was 52.7 months. Median survival after the diagnosis of MDS or ANLL was 6.7 months. Treatment of MDS comprised allogeneic stem cell transplant in eight patients (median survival, 219 days; one patient alive at 624 days) and a hypomethylating agent in 21 patients (response of stable or better in five patients). Myelodysplasia remains a devastating complication of therapy for multiple myeloma, with short survival and poor response rates to available modalities. PMID:25284489

Gertz, Morie A; Terpos, Evangelos; Dispenzieri, Angela; Kumar, Shaji; Shah, Rupin A; Orlowski, Robert; Kastritis, Efstathios; Dimopoulos, Meletios A; Shah, Jatin

2014-11-01

224

Cyclophosphamide and methotrexate in Susac's Syndrome: a successful sequential therapy in a case with involvement of the cerebellum.  

PubMed

Susac's Syndrome (SS) is a rare disease with unknown aetiology due to a microangiopathy affecting the precapillary arterioles of the brain, retina, cochlea and semicircular canals. Neurological manifestations, visual dysfunction and hearing loss represent the classical clinical triad of SS. Diagnosis is confirmed by laboratory investigations, neuroimaging findings, fluoroangiography and inner-ear studies. An early treatment with steroids and immunosuppressors limits the sequelae of disease. We report a case of SS in which the clinical triad occurred in a very short period of time. Brain MRI showed the involvement of cerebellum, this representing a rare neuroradiological finding in SS. A full remission of disease was obtained by using corticosteroids and cyclophosphamide in the acute-subacute phase and methotrexate as maintenance therapy. This latter has never been used before in SS. PMID:24794491

Catarsi, Eleonora; Pelliccia, Veronica; Pizzanelli, Chiara; Pesaresi, Ilaria; Cosottini, Mirco; Migliorini, Paola; Tavoni, Antonio

2014-05-01

225

Therapy for mucopolysaccharidosis VI: (Maroteaux-Lamy syndrome) present status and prospects.  

PubMed

Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disorder caused by deficient activity of Arylsulphatase B (ARSB). The disease is progressive and multisystemic, usually leading to death in the first decades of life. In addition to supportive management, specific treatments for MPS VI are the hematopoietic stem cell transplantation (HSCT) and enzyme replacement therapy (ERT). Both are effective for some aspects of the disease, but fail in correcting important clinical features, such as bone deformities and heart valve thickening. Based on that, new treatments are currently being tested to be used alone or in combination with the current therapies. Here we summarize some of these new approaches and the preliminary results obtained, reporting their limitations and indicating possible future trends in MPS VI treatment. We discuss intrathecal ERT, gene therapy and therapies based on anti-inflammatory molecules, among other approaches. Finally, we highlight the importance of early treatment and diagnosis for a better outcome in these patients. PMID:25345097

Giugliani, Roberto; Herber, Silvani; Lapagesse, Louise; de Pinto, Camargo; Baldo, Guilherme

2014-09-01

226

Refractory postsurgical pyoderma gangrenosum in a patient with Beckwith Wiedemann syndrome: response to multimodal therapy.  

PubMed

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that may be difficult to diagnose and treat. We presented a 41-year-old woman who required skin grafting following third-degree burns to her left breast. She suffered recurrent graft dehiscence and infections over many years, prompting elective bilateral reduction mammoplasty. She subsequently developed suture margin ulcerations unresponsive to topical therapies and antibiotics. Skin biopsies were non-specific, and a clinical diagnosis of PG was established. Although initially responsive to corticosteroids, wounds promptly recurred following steroid taper. She was treated unsuccessfully with various immunomodulatory agents and underwent elective bilateral mastectomy. Following a mastectomy, she developed progressive deep chest wall ulcerations. She failed numerous immunomodulatory treatments, surgical wound closure and negative pressure wound therapy. Ultimately, treatment with adalimumab, mycophenolate mofetil and prednisone, in addition to hyperbaric oxygen therapy facilitated progressive healing. Our case highlights the role of collaborative multimodal therapy for the treatment of refractory PG. PMID:24154999

Fakhar, Faiza; Memon, Sehrish; Deitz, Diane; Abramowitz, Richard; Alpert, Deborah R

2013-01-01

227

Radiation Therapy for Gorham-Stout Syndrome: Results of a National Patterns-of-Care Study and Literature Review  

SciTech Connect

Purpose: The German Cooperative Group on Radiotherapy for Benign Diseases conducted a national patterns-of-care study to investigate the value of radiation therapy (RT) in the management of Gorham-Stout syndrome. Methods and Materials: In 2009 a structured questionnaire was circulated to 230 German RT institutions to assess information about the number of patients, the RT indication and technique, and the target volume definition, as well as accompanying treatments, outcome data, and early or late radiation toxicity. Results: In November 2009 responses were available from 197 departments (85.6%): 29 university hospitals (14.7%), 89 community hospitals (45.2%), and 79 private RT offices (40.1%). Of these institutions, 8 (4.0%) had experience using RT, for a total of 10 cases in various anatomic sites. Four patients underwent irradiation postoperatively, and six patients received primary RT. The total doses applied after computed tomography-based treatment planning ranged from 30 to 45 Gy. After a median follow-up period of 42 months, local disease progression was avoided in 8 cases (80.0%). In 2 of these cases a progression occurred beyond the target volume. Acute and late toxicity was mild; in 4 patients RT was associated with Grade I side effects according to Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer criteria. The literature analysis of 38 previously published articles providing results after the use of RT in 44 patients showed stable or regressive disease in 77.3%. Conclusions: RT may prevent disease progression effectively in Gorham-Stout syndrome in 77% to 80% of cases. Total doses ranging from 30 to 45 Gy applied after computed tomography-based treatment planning are recommended.

Heyd, Reinhard, E-mail: reiniheyd@aol.com [Department of Radiotherapy, Offenbach Hospital, Offenbach (Germany); Micke, Oliver [Department of Radiotherapy and Radiation Oncology, Franziskus Hospital, Bielefeld (Germany); Surholt, Christine [Department of Radiotherapy, Ansbach Hospital, Ansbach (Germany); Berger, Bernhard [Department of Radiation Oncology, University Hospital Tuebingen, Tuebingen (Germany); Martini, Carmen [Department of Radiotherapy, University Hospital Freiburg, Freiburg (Germany); Fueller, Juergen [Department of Radiotherapy and Radiation Oncology, Hospital of the Friedrich Schiller University, Jena (Germany); Schimpke, Thomas [Department of Radiotherapy Aschaffenburg Hospital, Aschaffenburg (Germany); Seegenschmiedt, M. Heinrich [Radiation Therapy Center, Hamburg (Germany)

2011-11-01

228

Safety and efficacy of enzyme replacement therapy with idursulfase beta in children aged younger than 6years with Hunter syndrome.  

PubMed

Idursulfase beta (Hunterase®) has been used for enzyme replacement therapy (ERT) of patients with mucopolysaccharidosis II (MPS II, Hunter syndrome) aged 6years or older since 2012 in Korea. The objective of this study was to evaluate the safety and efficacy of ERT with idursulfase beta in Hunter syndrome children younger than 6years. This study was a 52-week, single center, single arm, open-label clinical trial (NCT01645189). Idursulfase beta (0.5mg/kg/week) was administered intravenously for 52weeks. The primary endpoint was safety assessed by adverse events (AEs). Secondary endpoints included vital signs, physical examination, ECG, laboratory tests, anti-idursulfase antibodies, and efficacy represented by changes in urinary glycosaminoglycan (GAG) at week 53 from baseline. In addition, growth indices and developmental milestones (Denver II test) were evaluated as exploratory variables. All six patients experienced at least one AE. A total of 109 AEs were reported. One patient experienced a serious AE (hospitalization due to gastroenteritis) that was considered not to be treatment related. One patient (16.7%) experienced infusion-related adverse drug reactions (ADRs), developing urticaria six times and a cough five times. There were no serious ADRs and no clinically significant changes in vital signs, physical exam, laboratory parameters, or ECG. Of the six patients, four (66.7%) showed anti-idursulfase antibodies and neutralizing antibodies on at least one occasion during the study. At week 53, urinary GAG was significantly reduced by -35.1±30.6mgGAG/g creatine from baseline (P=0.038). This study indicates that the safety and efficacy of idursulfase beta are similar to those reported in Hunter syndrome patients aged 6years or older. PMID:25219292

Sohn, Young Bae; Cho, Sung Yoon; Lee, Jieun; Kwun, Yonghee; Huh, Rimm; Jin, Dong-Kyu

2014-08-30

229

Activities of daily living in patients with Hunter syndrome: Impact of enzyme replacement therapy and hematopoietic stem cell transplantation.  

PubMed

The aim of this study was to assess the activities of daily living (ADL) in patients with Hunter syndrome (mucopolysaccharidosis II; MPS II) using a newly designed ADL questionnaire. We applied the questionnaire to evaluate clinical phenotypes and therapeutic efficacies of enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT). We also explored early signs and symptoms to make early diagnosis feasible. We devised a new ADL questionnaire with three domains: "movement," "movement with cognition," and "cognition." Each domain has four subcategories rated on a 5-point scale based on level of assistance. We also scored signs and symptoms unique to MPS by 12 subcategories (five points per category), providing 60 points in total. The questionnaire was first administered to 138 healthy Japanese controls (0.33-50years), and successively, to 74 Japanese patients with Hunter syndrome (4-49years). The patient cohort consisted of 51 severe and 23 attenuated phenotypes; 20 patients treated with HSCT, 23 patients treated early with ERT (?8years), 25 patients treated late with ERT (>8years), and 4 untreated patients. Among 18 severe phenotypic patients treated by HSCT, 10 were designated as early HSCT (?5years), while 8 were designated as late HSCT (>5years). Scores from patients with severe phenotypes were lower than controls and attenuated phenotypes in all categories. Among patients with severe phenotypes, there was a trend that HSCT provides a higher ADL score than early ERT, and there was a significant difference in ADL scores between late ERT and HSCT groups. Early ERT and early HSCT provided a higher score than late ERT and late HSCT, respectively. In conclusion, we have evaluated the feasibility of a new questionnaire in control population and patients with Hunter syndrome, leading to a novel evaluation method for clinical phenotypes and therapeutic efficacy. Early treatment with HSCT provides a better consequence in ADL of patients. PMID:25468646

Tanjuakio, Julian; Suzuki, Yasuyuki; Patel, Pravin; Yasuda, Eriko; Kubaski, Francyne; Tanaka, Akemi; Yabe, Hiromasa; Mason, Robert W; Montańo, Adriana M; Orii, Kenji E; Orii, Koji O; Fukao, Toshiyuki; Orii, Tadao; Tomatsu, Shunji

2015-02-01

230

Updates on the myo-inositol plus D-chiro-inositol combined therapy in polycystic ovary syndrome.  

PubMed

Polycystic ovary syndrome (PCOS) is one of the most common endocrine disorders affecting women of reproductive age. It is characterized by chronic anovulation, hyperandrogenism, and insulin resistance. It is the main cause of infertility due to the menstrual dysfunction and metabolic disorders. Women with PCOS also have an increased cardiovascular risk because of dyslipidemia and insulin resistance. So far, we have a lot of information about the etiology of PCOS, and many steps forward have been made about the diagnosis of this syndrome, but there is still no certainty about the therapy. Myo-inositol (MI) and D-chiro-inositol, two inositol stereoisomers, have been proven to be effective in PCOS treatment. However, only MI has been shown to have beneficial effects on reproductive function, whereas the administration of MI/D-chiro-inositol, in the physiological plasma ratio (i.e., 40:1) ensures better clinical results, such as the reduction of insulin resistance, androgens' blood levels, cardiovascular risk and regularization of menstrual cycle with spontaneous ovulation. PMID:24898153

Unfer, Vittorio; Porcaro, Giuseppina

2014-09-01

231

Androgen receptor roles in insulin resistance and obesity in males: the linkage of androgen-deprivation therapy to metabolic syndrome.  

PubMed

Prostate cancer (PCa) is one of the most frequently diagnosed malignancies in men. Androgen-deprivation therapy (ADT) is the first-line treatment and fundamental management for men with advanced PCa to suppress functions of androgen/androgen receptor (AR) signaling. ADT is effective at improving cancer symptoms and prolonging survival. However, epidemiological and clinical studies support the notion that testosterone deficiency in men leads to the development of metabolic syndrome that increases cardiovascular disease risk. The underlying mechanisms by which androgen/AR signaling regulates metabolic homeostasis in men are complex, and in this review, we discuss molecular mechanisms mediated by AR signaling that link ADT to metabolic syndrome. Results derived from various AR knockout mouse models reveal tissue-specific AR signaling that is involved in regulation of metabolism. These data suggest that steps be taken early to manage metabolic complications associated with PCa patients receiving ADT, which could be accomplished using tissue-selective modulation of AR signaling and by treatment with insulin-sensitizing agents. PMID:25249645

Yu, I-Chen; Lin, Hung-Yun; Sparks, Janet D; Yeh, Shuyuan; Chang, Chawnshang

2014-10-01

232

Improvement of hypercortisolism by ?-blocker therapy in subclinical Cushing's syndrome associated with ACTH-independent macronodular adrenocortical hyperplasia.  

PubMed

A 61-year-old man with hypertension and diabetes was referred for the evaluation of multiple bilateral adrenal tumors. While Cushingoid features were not apparent, an elevated cortisol level in response to a low-dose dexamethasone suppression test (187.7 nmol/l), an elevated urinary cortisol level (170.9 nmol/day), and a weak response to a cosyntropin-releasing hormone (CRH) provocation test were observed. Furthermore, the serum cortisol level increased in response to a posture test or isoproterenol infusion. Accordingly, the patient was diagnosed as having ACTH-independent macronodular adrenal hyperplasia (AIMAH) with subclinical Cushing's syndrome associated with the aberrant expression of ?-adrenergic receptors. After 2 months of propranolol therapy, the serum cortisol responses to a posture test and isoproterenol infusion, the cortisol level in response to a low-dose dexamethasone suppression test (102.1 nmol/l), and the urinary cortisol level (165.9 nmol/day) all normalized. While the suppression of cortisol secretion was sustained for 24 months, glucose metabolism and adrenal size were unaffected. To our knowledge, this is the first report of AIMAH accompanied by subclinical Cushing's syndrome associated with the aberrant expression of ?-adrenergic receptors. Furthermore, propranolol inhibited cortisol hypersecretion in the present case. Additional cases or controlled studies are needed to determine the potential effect of propranolol on metabolic disorders and adrenal size in patients with AIMAH. PMID:19813002

Oki, Kenji; Yamane, Kiminori; Nakanishi, Shuhei; Nakashima, Reiko; Jitsuiki, Kuniaki; Kohno, Nobuoki

2009-12-01

233

Metformin therapy throughout pregnancy reduces the development of gestational diabetes in women with polycystic ovary syndrome  

Microsoft Academic Search

Objective: To assess whether metformin safely reduced development of gestational diabetes in women with the polycystic ovary syndrome (PCOS).Design: Prospective and retrospective study.Setting: Outpatient clinical research center.Patient(s): The prospective study included 33 nondiabetic women with PCOS who conceived while taking metformin and had live births; of these, 28 were taking metformin through delivery. The retrospective study included 39 nondiabetic women

C. J Glueck; Ping Wang; Suichi Kobayashi; Harvey Phillips; Luann Sieve-Smith

2002-01-01

234

Therapy of the neonatal abstinence syndrome with tincture of opium or morphine drops  

Microsoft Academic Search

Background:Treating opioid-addicted women with methadone in pregnancy increased the number of newborns suffering from neonatal abstinence syndrome (NAS). High-pitch crying, insomnia, tremor, myoclonic jerks, vomiting, diarrhoea and poor weight gain were reported symptoms, which were evaluated using the Finnegan (F)-score. Earlier phenobarbital or paregoric had been used to suppress symptoms. We surveyed the administration of pure ?-agonist morphine (MO) in

Stefan Langenfeld; Larissa Birkenfeld; Peter Herkenrath; Carsten Müller; Martin Hellmich; Martin Theisohn

2005-01-01

235

Habit Reversal Training and Acceptance and Commitment Therapy for Tourette Syndrome: A Pilot Project  

Microsoft Academic Search

Chronic tic disorders (CTDs), including Tourette Syndrome, affect approximately 1 in 100 individuals, and pediatric onset\\u000a is the norm. Pharmacotherapy has traditionally been the first line of treatment for CTD. However, given that partial response\\u000a to pharmacotherapy is typical and that the side effect profile for efficacious medications warrants consideration before initiating\\u000a treatment, there is a clear need to develop

Martin E. Franklin; Stephanie H. Best; Michelle A. Wilson; Benjamin Loew; Scott N. Compton

2011-01-01

236

A Biopsychosocial Therapy Model for Chronic Prostatitis\\/Chronic Pelvic Pain Syndrome  

Microsoft Academic Search

\\u000a Chronic prostatitis\\/chronic pelvic pain syndrome (CP\\/CPPS) has long been considered a frustrating and prevalent condition\\u000a by patients and treating physicians alike. Pain is noted as the cardinal symptom in CP\\/CPPS and is strongly associated with\\u000a a host of intra and interpersonal difficulties reported by patients in domains such as pain, disability, relations, and overall\\u000a quality of life. With no tenable

Dean A. Tripp

237

Myelodysplasia syndrome and acute myeloid leukemia in patients with congenital neutropenia receiving G-CSF therapy  

Microsoft Academic Search

Granulocyte colony-stimulating factor (G- CSF) has had a major impact on manage- ment of ''severe chronic neutropenia,'' a collective term referring to congenital, idiopathic, or cyclic neutropenia. Almost all patients respond to G-CSF with in- creased neutrophils, reduced infections, and improved survival. Some responders with congenital neutropenia have devel- oped myelodysplastic syndrome and acute myeloblastic leukemia (MDS\\/AML), which raises the

Melvin H. Freedman; Mary Ann Bonilla; Carol Fier; Audrey Anna Bolyard; Debra Scarlata; Laurence A. Boxer; Sherri Brown; Bonnie Cham; George Kannourakis; Sally E. Kinsey; Pier Georgio; Tammy Cottle; Karl Welte; David C. Dale

2000-01-01

238

Adult Asperger Syndrome and the Utility of Cognitive-Behavioral Therapy  

Microsoft Academic Search

Asperger syndrome (AS) is a developmental disorder that was first recognized in the United States in 1994 with the publication\\u000a of DSM-IV (American Psychiatric Association, Diagnostic and statistical manual of mental disorders, 1994). As a relatively new concept to the United States, both within the professional community and public awareness, the diagnosis\\u000a has most often applied to children, but there

Valerie L. Gaus

2011-01-01

239

Overweight, obesity and features of metabolic syndrome in children with diabetes treated with insulin pump therapy  

Microsoft Academic Search

There has been no specific evaluation of atherogenic risk factors in children with type 1 diabetes mellitus (T1DM) treated\\u000a with continuous subcutaneous insulin infusion (CSII). We, therefore, studied the prevalence of overweight\\/obesity and metabolic\\u000a syndrome among these patients. Five hundred children with T1DM treated with CSII and multiple daily insulin (MDI) regimen\\u000a were included in the study. Anthropometric data\\/physical examination,

W?odzimierz ?uczy?ski; Agnieszka Szypowska; Barbara G?owi?ska-Olszewska; Artur Bossowski

2011-01-01

240

Grade 4 febrile neutropenia and Fournier's Syndrome associated with triple therapy for hepatitis C virus: A case report.  

PubMed

The use of triple therapy for hepatitis C not only increases the rate of sustained virological responses compared with the use of only interferon and ribavirin (RBV) but also leads to an increased number of side effects. The subject of this study was a 53-year-old male who was cirrhotic with hepatitis C virus genotype 1 A and was a previous null non-responder. We initially attempted retreatment with boceprevir (BOC), Peg-interferon and RBV, and a decrease in viral load was observed in the 8(th) week. In week 12, he presented with disorientation, flapping, fever, tachypnea, arterial hypotension and tachycardia. He also exhibited leucopenia with neutropenia. Cefepime and filgrastim were initiated, and treatment for hepatitis C was suspended. A myelogram revealed hypoplasia, cytotoxicity and maturational retardation. After 48 h, he developed bilateral inguinal erythema that evolved throughout the perineal area to the root of the thighs, with exulcerations and an outflow of seropurulent secretions. Because we hypothesized that he was suffering from Fournier's Syndrome, treatment was replaced with the antibiotics imipenem, linezolid and clindamycin. After this new treatment paradigm was initiated, his lesions regressed without requiring surgical debridement. Triple therapy requires knowledge regarding the management of adverse effects and drug interactions; it also requires an understanding of the importance of respecting the guidelines for the withdrawal of treatment. In this case report, we observed an adverse event that had not been previously reported in the literature with the use of BOC. PMID:25018856

Oliveira, Kelly Cristhian Lima; Cardoso, Emili de Oliveira Bortolon; de Souza, Suzana Carla Pereira; Machado, Flávia Souza; Zangirolami, Carlos Eduardo Alves; Moreira, Alecsandro; Silva, Giovanni Faria; de Oliveira, Cássio Vieira

2014-06-27

241

Efficacy of Extracorporeal Shock Wave Therapy for the Treatment of Chronic Pelvic Pain Syndrome: A Randomized, Controlled Trial  

PubMed Central

Objectives. To investigate the effectiveness of extracorporeal shock wave therapy (ESWT) for symptoms alleviation in chronic pelvic pain syndrome (CPPS). Materials and Methods. 40 patients with CPPS were randomly allocated into either the treatment or sham group. In the first group, patients were treated by ESWT once a week for 4 weeks by a defined protocol. In the sham group, the same protocol was applied but with the probe being turned off. The follow-up assessments were done at 1, 2, 3, and 12 weeks by Visual Analogue Scale (VAS) for pain and NIH-developed Chronic Prostatitis Symptom Index (NIH-CPSI). Results. Pain domain scores at follow-up points in both treatment and sham groups were reduced, more so in the treatment group, which were significant at weeks 2, 3, and 12. Urinary scores became significantly different at weeks 3 and 12. Also, quality of life (QOL) and total NIH-CPSI scores at all four follow-up time points reduced more significantly in the treatment group as compared to the sham group. Noticeably, at week 12 a slight deterioration in all variables was observed compared to the first 3 weeks of the treatment period. Conclusions. our findings confirmed ESWT therapy as a safe and effective method in CPPS in short term. PMID:24000311

Vahdatpour, Babak; Moayednia, Amir; Emadi, Masoud; Khorami, Mohammad Hatef; Haghdani, Saeid

2013-01-01

242

The PEX study – Exercise therapy for patellofemoral pain syndrome: design of a randomized clinical trial in general practice and sports medicine [ISRCTN83938749  

PubMed Central

Background Patellofemoral complaints are frequently seen in younger and active patients. Clinical strategy is usually based on decreasing provoking activities as sports and demanding knee activities during work and leisure and reassuring the patient on the presumed good outcome. Exercise therapy is also often prescribed although evidence on effectiveness is lacking. The objective of this article is to present the design of a randomized clinical trial that examines the outcome of exercise therapy supervised by a physical therapist versus a clinically accepted "wait and see" approach (information and advice about the complaints only). The research will address to both effectiveness and cost effectiveness of supervised exercise therapy in patients with patellofemoral pain syndrome (PFPS). Methods/design 136 patients (adolescents and young adults) with patellofemoral pain syndrome are recruited in general practices and sport medicine centers. They will be randomly allocated receiving either 3 months of exercise therapy (or usual care. The primary outcome measures are pain, knee function and perception of recovery after 3 months and 12 months of follow up and will be measured by self reporting. Measurements will take place at baseline, 6 weeks, and 3 monthly until 1 year after inclusion in the study. Secondary outcome measurements include an economic evaluation. A cost-utility analysis will be performed that expresses health improvements in Quality Adjusted Life Years (QALYs) and incorporates direct medical costs and productivity costs Discussion This study has been designed after reviewing the literature on exercise therapy for patellofemoral pain syndrome. It was concluded that to merit the effect of exercise therapy a trial based on correct methodological concept needed to be executed. The PEX study is a randomized clinical trial where exercise therapy is compared to usual care. This trial started in April 2005 and will finish in June 2007. The first results will be available around December 2007. PMID:16545120

van Linschoten, Robbart; van Middelkoop, Marienke; Berger, Marjolein Y; Heintjes, Edith M; Koopmanschap, Mark A; Verhaar, Jan AN; Koes, Bart W; Bierma-Zeinstra, Sita MA

2006-01-01

243

Valorisation nutritive d'un grignon d'olive trait la soude. Utilisation digestive des constituants des parois cellulaires  

E-print Network

Valorisation nutritive d'un grignon d'olive traité à la soude. Utilisation digestive des'utilisation digestive et la contribution des constituants des parois cellulaires à la valeur nutritive d'un grignon d'olive décrit l'effet du traitement à la soude sur la valeur nutritive du grignon d'olive et nous avons aussi

Paris-Sud XI, Université de

244

Antiplatelet Therapy in Acute Coronary Syndromes without Persistent ST-Segment Elevation  

Microsoft Academic Search

The treatment of ACS without persistent ST-segment elevation is evolving. Antiplatelet and antithrombin therapy forms the mainstay of medical management. The antiplatelet agents studied can be pharmacologically classified as GP IIb\\/IIIa receptor antagonists, ADP receptor antagonists, thromboxane inhibitors, and cyclo-oxgenase inhibitors. While aspirin, a cyclo-oxygenase inhibitor, is well entrenched in the treatment (and thus will not be reviewed here), other

Deepak Jain; Hugo A. Katus; Gert Richardt

2001-01-01

245

Klinefelter Syndrome  

MedlinePLUS

... testosterone. That doesn't make a guy less male, but it can affect things like penis and testicle growth. Boys with Klinefelter syndrome may ... bigger muscles, a deeper voice, growth of the penis, and facial and body ... or reverse infertility. Physical therapy, occupational therapy, and ...

246

Transformation of Sézary Syndrome Into CD30+ Anaplastic Large T-Cell Lymphoma After Alemtuzumab Therapy With Evidence of Clonal Unity.  

PubMed

: Alemtuzumab is a humanized mouse antibody targeting the CD52 cell surface, which has been effective in patients with advanced stage mycosis fungoides (MF) including erythrodermic MF and Sézary syndrome. There are a few descriptions of large cell transformation after its administration. A young patient with an acute onset of Sézary syndrome treated initially unsuccessfully with fludarabine and cyclophosphamide and later on successfully with alemtuzumab has been described. Three weeks after the beginning of therapy, however, she developed transformed T-cell lymphoma indistinguishable from CD30 anaplastic large-cell lymphoma. After bone marrow transplantation, the transformed CD30 cutaneous T-cell lymphoma recurred as a transformed CD30 plaque MF. All 3 types of lesions showed the same T-cell receptor clonal gene rearrangement, which supports the notion that Sézary syndrome, CD30 anaplastic large-cell lymphoma, and MF are interrelated. PMID:25548993

Nevet, Mariela Judith; Zuckerman, Tsila; Sahar, Dvora; Bergman, Reuven

2015-01-01

247

Omega-3 Essential Fatty Acids Therapy for Dry Eye Syndrome: A Meta-Analysis of Randomized Controlled Studies  

PubMed Central

Background Dry eye is a common, complex condition that can reduce ocular comfort and visual performance. The impact on quality of life has been rated as similar to the effect of moderate angina and, in more severe cases, dialysis and severe angina. This study aimed to use meta-analysis to compare omega-3 fatty acid and placebo fatty acid in the management of dry eye syndrome. Material/Methods Comparative studies published until 1 June 2014 were searched through a comprehensive search of the Medline, Embase, Web of Science, and the Cochrane Library electronic databases. A systematic review and cumulative analysis of comparative studies reporting the effect of omega-3 fatty acid on dry eye syndrome was conducted. All analyses were performed using the Review Manager (RevMan) v.5 software (Nordic Cochrane Centre, Copenhagen, Denmark). Results The trials involved a total of 790 participants in 7 independent studies. All the studies are published between 2007 and 2013. Meta-analysis of the 5 studies that reported data in mean SD values revealed that the tear break-up time (TBUT) was significantly greater by 1.58 s (WMD=1.58, 95% CI=0.60 to 2.55; P=0.007). Combination of all the Schirmer’s test data showed that omega-3 fatty acid supplementation could significantly improve the Schirmer’s test (WMD=0.74, 95% CI=0.29 to 1.19; P=0.001). However, the combination of all the OSDI test data showed that omega-3 fatty acid supplementation did not significantly improve the OSDI test results (WMD=?4.54, 95% CI=?9.85 to 0.78; P=0.09). Conclusions Based on the data included in our meta-analysis, omega-3 fatty acid was associated with better TBUT and Schirmer’s. No significant differences were detected in OSDI test results. Consequently, our findings suggest that omega-3 fatty acid offers is an effective therapy for dry eye syndrome. PMID:25193932

Liu, Aihua; Ji, Jian

2014-01-01

248

Insulin autoimmune syndrome in a health supplement user: the effectiveness of cornstarch therapy for treating hypoglycemia.  

PubMed

A 70-year-old woman with no history of diabetes was admitted to the hospital for the management of hypoglycemia. Her fasting plasma glucose level was 54 mg/dL with an extremely high serum immunoreactive insulin level (1210 ?U/mL). She had high titers of anti-insulin antibodies and exhibited the DRB1*0406 genotype for HLA-DR4, leading to a diagnosis of insulin autoimmune syndrome. She had been taking several health preparations for approximately 10 years; however, all were thiol group-free. Due to frequent episodes of nocturnal hypoglycemia, the health preparations were discontinued and the patient was treated with cornstarch. This protocol successfully ameliorated the hypoglycemic episodes and normalized the patient's laboratory and serological test results. PMID:23370747

Deguchi, Arichika; Okauchi, Yukiyoshi; Suehara, Setsuyo; Mineo, Ikuo

2013-01-01

249

Diagnosis of Atypical Hemolytic Uremic Syndrome and Response to Eculizumab Therapy  

PubMed Central

Atypical hemolytic uremic syndrome (aHUS) has a high mortality rate if not detected and treated early. While in the past, it was associated with renal failure in children, today, it has become increasingly identified among adults. Due to recent advances in the pathogenesis of aHUS and other major thrombotic microangiopathies (TMA), diagnosing it has become a lot easier. We present a case of a 62-year-old man who was initially thought to have thrombotic thrombocytopenic purpura (TTP), but after further evaluation was diagnosed with aHUS. We will discuss how to distinguish aHUS from other major TMA and the role of eculizumab in the management of aHUS. PMID:25285252

Mathew, Jacob J; Denunzio, Troy M; Carmichael, Mark G

2014-01-01

250

Antiplatelet therapy in acute coronary syndromes: current agents and impact on patient outcomes  

PubMed Central

Platelets play a central role in atherothrombosis and subsequent development of acute coronary syndromes (ACS). The understanding of this process has driven a large body of evidence demonstrating the mortality and morbidity benefits of antiplatelet agents in the ACS population. As expected, however, these agents come with an intrinsically increased risk of bleeding which underlies the vast majority of their complications and adverse effects. In today’s setting of compounding comorbidities and broadening indications, finding the balance between thrombosis prevention and bleeding risk remains the challenge for all clinicians considering these medications. This article reviews the current main antiplatelet agents that are available for clinical use and outlines their impact on ACS outcome. We also outline factors which affect the response to these agents and discuss strategies to optimize clinical outcomes. PMID:22915965

Tayeb, Hussam M; Nelson, Adam J; Willoughby, Scott R; Worthley, Matthew I

2011-01-01

251

Metformin therapy is associated with a decrease in plasma plasminogen activator inhibitor-1, lipoprotein(a), and immunoreactive insulin levels in patients with the polycystic ovary syndrome  

Microsoft Academic Search

Sixteen nondiabetic women with polycystic ovary syndrome (PCOS) aged 18 to 33 years were studied before and after 8 weeks on metformin (1.5 g\\/d) therapy to assess whether reducing hyperinsulinemia would reduce the levels of the major inhibitor of fibrinolysis, antigenic plasminogen activator inhibitor type 1 (PAI-1). Compared with six normal control women, PCOS women had a higher body mass

Elsy M. Velazquez; Soaira G. Mendoza; Ping Wang; Charles J. Glueck

1997-01-01

252

The Effect Of A Music Therapy Intervention Employing Peer Models On The Social Skills Development Of Young Adults With Asperger's Syndrome  

Microsoft Academic Search

The purpose of this study was to determine the effectiveness of the use of music therapy interventions on selected social skills of young adults with Asperger’s Syndrome (AS). Six dyads were formed with one young adult with AS (mean age = 24.5 years) and one neuro-typical, non-Asperger’s peer (mean age = 24.8 years). The study was conducted in 12 sessions

James Ryley Maxson

2009-01-01

253

[Laboratory parameters for the control of the course of therapy of canine Cushing's syndrome].  

PubMed

We compared the results of the ACTH stimulation tests with measurements of alkaline phosphatase and serum cholesterol during Lysodren therapy in 23 dogs with pituitary-dependent hyperadrenocorticism. The ACTH stimulation test proved to be a very sensitive parameter, by which the extent of Lysodren under- or overdosage could be reliably estimated. On the other hand, regulation of the individual Lysodren requirement was not possible by measuring AP and serum cholesterol only. However, it is highly probable that those two parameters can be used to evaluate the general state of metabolism, and they appear to be of prognostic value when greatly elevated. PMID:1646492

Reusch, C; Hähnle, B

1991-02-01

254

A non-complement-fixing antibody to ?2 glycoprotein I as a novel therapy for antiphospholipid syndrome.  

PubMed

A single-chain fragment variable (scFv) recognizing ?2-glycoprotein 1 (?2GPI) from humans and other species was isolated from a human phage display library and engineered to contain an IgG1 hinge-CH2-CH3 domain. The scFv-Fc directed against ?2GPI domain I-induced thrombosis and fetal loss, thus mimicking the effect of antibodies from patients with antiphospholipid syndrome (APS). Complement is involved in the biological effect of anti-?2GPI scFv-Fc, as demonstrated by its ability to promote in vitro and in vivo complement deposition and the failure to induce vascular thrombosis in C6-deficient rats and fetal loss in C5-depleted mice. A critical role for complement was also supported by the inability of the CH2-deleted scFv-Fc to cause vessel occlusion and pregnancy failure. This antibody prevented the pathological effects of anti-?2GPI antibodies from APS patients and displaced ?2GPI-bound patient antibodies. The CH2-deleted antibody represents an innovative approach potentially useful to treat APS patients refractory to standard therapy. PMID:24642748

Agostinis, Chiara; Durigutto, Paolo; Sblattero, Daniele; Borghi, Maria O; Grossi, Claudia; Guida, Filomena; Bulla, Roberta; Macor, Paolo; Pregnolato, Francesca; Meroni, Pier Luigi; Tedesco, Francesco

2014-05-29

255

Acute Renal Replacement Therapy in Children with Diarrhea-Associated Hemolytic Uremic Syndrome: A Single Center 16 Years of Experience  

PubMed Central

Acute kidney injury (AKI) is becoming more prevalent among hospitalized children, its etiologies are shifting, and new treatment modalities are evolving; however, diarrhea-associated hemolytic uremic syndrome (D+HUS) remains the most common primary disease causing AKI in young children. Little has been published about acute renal replacement therapy (ARRT) and its challenges in this population. We describe our single center's experience managing 134 pediatric patients with D+HUS out of whom 58 (43%) required ARRT over the past 16 years. In our cohort, all but one patient were started on peritoneal dialysis (PD). Most patients, 47 (81%), received acute PD on a pediatric inpatient ward. The most common recorded complications in our cohort were peritoneal fluid leaks 13 (22%), peritonitis 11 (20%), and catheter malfunction 5 (9%). Nine patients (16%) needed surgical revision of their PD catheters. There were no bleeding events related to PD despite a mean platelets count of 40.9 (±23.5)?×?103/mm3 and rare use of platelets infusions. Despite its methodological limitations, this paper adds to the limited body of evidence supporting the use of acute PD as the primary ARRT modality in children with D+HUS. PMID:21716936

Grisaru, Silviu; Morgunov, Melissa A.; Samuel, Susan M.; Midgley, Julian P.; Wade, Andrew W.; Tee, James B.; Hamiwka, Lorraine A.

2011-01-01

256

Cost-effectiveness of internet-based cognitive behavior therapy for irritable bowel syndrome: results from a randomized controlled trial  

PubMed Central

Background Irritable Bowel Syndrome (IBS) is highly prevalent and is associated with a substantial economic burden. Cognitive behavior therapy (CBT) has been shown to be effective in treating IBS. The aim of this study was to evaluate the cost-effectiveness of a new treatment alternative, internet-delivered CBT based on exposure and mindfulness exercises. Methods Participants (N = 85) with IBS were recruited through self-referral and were assessed via a telephone interview and self-report measures on the internet. Participants were randomized to internet-delivered CBT or to a discussion forum. Economic data was assessed at pre-, post- and at 3-month and 1 year follow-up. Results Significant cost reductions were found for the treatment group at $16,806 per successfully treated case. The cost reductions were mainly driven by reduced work loss in the treatment group. Results were sustained at 3-month and 1 year follow-up. Conclusions Internet-delivered CBT appears to generate health gains in IBS treatment and is associated with cost-savings from a societal perspective. PMID:21473754

2011-01-01

257

Hedgehog agonist therapy corrects structural and cognitive deficits in a Down syndrome mouse model  

PubMed Central

Down syndrome (DS) is among the most frequent genetic causes of intellectual disability, and ameliorating this deficit is a major goal in support of people with trisomy 21. The Ts65Dn mouse recapitulates some major brain structural and behavioral phenotypes of DS, including reduced size and cellularity of the cerebellum and learning deficits associated with the hippocampus. We show that a single treatment of newborn mice with the sonic hedgehog pathway agonist, SAG1.1 (SAG), results in normal cerebellar morphology in adults. Further, SAG treatment at birth rescued phenotypes associated with hippocampal deficits that occur in untreated adult Ts65Dn mice. This treatment resulted in behavioral improvements and normalized performance in the Morris Water Maze task for learning and memory. SAG treatment also produced physiological effects and partially rescued both NMDA receptor dependent synaptic plasticity and NMDA/AMPA receptor ratio, physiological measures associated with memory. These outcomes confirm an important role for the hedgehog pathway in cerebellar development and raise the possibility for its direct influence in hippocampal function. The positive results from this approach suggest a possible direction for therapeutic intervention to improve cognitive function for this population. PMID:24005160

Das, Ishita; Park, Joo-Min; Shin, Jung H.; Jeon, Soo Kyeong; Lorenzi, Hernan; Linden, David J.; Worley, Paul F.; Reeves, Roger H.

2014-01-01

258

Constipation-predominant irritable bowel syndrome: A review of current and emerging drug therapies  

PubMed Central

Irritable bowel syndrome (IBS) is a highly prevalent medical condition that adversely affects patient quality of life and constitutes a significant economic burden on healthcare resources. A large proportion of patients suffer from the constipation subtype of IBS (IBS-C), most commonly afflicting older individuals and those with a lower socioeconomic status. Conventional pharmacologic and nonpharmacologic treatment options have limited efficacies and/or significant adverse events, which lead to increased long-term health care expenditures. Failure to effectively treat IBS-C patients over the past decades has largely been due to a poor understanding of disease pathophysiology, lack of a global view of the patient, and an inappropriate selection of patients and treatment endpoints in clinical trials. In recent years, however, more effective and safer drugs have been developed for the treatment of IBS-C. The advancement in the area of pharmacologic treatment is based on new knowledge of the pathophysiologic basis of IBS-C and the development of drugs with increased selectivity within pharmacologic classes with recognized efficacies. This narrative review covers the spectrum of available drugs and their mechanisms of action, as well as the efficacy and safety profiles of each as determined in relevant clinical trials that have investigated treatment options for IBS-C and chronic constipation. A brief summary of laxative-based treatment options is presented, followed by up-to-date assessments for three classes of drugs: prokinetics, prosecretory agents, and bile acid modulators. PMID:25083062

Jadallah, Khaled A; Kullab, Susan M; Sanders, David S

2014-01-01

259

Efficacy of Topical Mesenchymal Stem Cell Therapy in the Treatment of Experimental Dry Eye Syndrome Model  

PubMed Central

Purpose. The current study was set out to address the therapeutic efficacy of topically applied mesenchymal stem cells (MSCs) on dry eye syndrome (DES) induced by benzalkonium chloride (BAC) in rats. Methods. Rats were divided into two groups just after establishment of DES. Eye drops containing either bromodeoxyuridine labeled MSCs (n = 9) or phosphate buffer solution (n = 7) were topically applied once daily for one week. Schirmer test, break-up time score, ocular surface evaluation tests, and corneal inflammatory index scoring tests were applied to all rats at baseline and after treatment. All rats were sacrificed after one week for histological and electron microscopic analysis. Results. Mean aqueous tear volume and tear film stability were significantly increased in rats treated with MSCs (P < 0.05). Infiltration of bromodeoxyuridine labeled MSCs into the meibomian glands and conjunctival epithelium was observed in MSCs treated rats. Increased number of secretory granules and number of goblet cells were observed in MSCs treated rats. Conclusion. Topical application of MSCs could be a safe and effective method for the treatment of DES and could potentially be used for further clinical research studies. PMID:25136370

Beyazy?ld?z, Emrullah; P?narl?, Ferda Alpaslan; Beyazy?ld?z, Özlem; Hekimo?lu, Emine Rümeysa; Acar, U?ur; Demir, Muhammed Necati; Albayrak, Aynur; Kaymaz, Figen; Sobac?, Güngör; Deliba??, Tuncay

2014-01-01

260

Pathogenesis of Macrophage Activation Syndrome and Potential for Cytokine-Directed Therapies.  

PubMed

Macrophage activation syndrome (MAS) is an acute episode of overwhelming inflammation characterized by activation and expansion of T lymphocytes and hemophagocytic macrophages. In rheumatology, it occurs most frequently in patients with systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus. The main clinical manifestations include cytopenias, liver dysfunction, coagulopathy resembling disseminated intravascular coagulation, and extreme hyperferritinemia. Clinically and pathologically, MAS bears strong similarity to hemophagocytic lymphohistiocytosis (HLH), and some authors prefer the term secondary HLH to describe it. Central to its pathogenesis is a cytokine storm, with markedly increased levels of numerous proinflammatory cytokines including IL-1, IL-6, IL-18, TNF?, and IFN?. Although there is evidence that IFN? may play a central role in the pathogenesis ofMAS, the role of other cytokines is still not clear. There are several reports of SJIA-associated MAS dramatically benefiting from anakinra, a recombinant IL-1 receptor antagonist, but the utility of other biologics in MAS is not clear. The mainstay of treatment remains corticosteroids; other medications, including cyclosporine, are used in patients who fail to respond. Expected final online publication date for the Annual Review of Medicine Volume 66 is January 14, 2015. Please see http://www.annualreviews.org/catalog/pubdates.aspx for revised estimates. PMID:25386930

Schulert, Grant S; Grom, Alexei A

2014-11-01

261

Outcome of Patients With Myelodysplastic Syndrome After Failure of Decitabine Therapy  

PubMed Central

BACKGROUND The prognosis of patients with myelodysplastic syndrome (MDS) after decitabine failure is not known. METHODS Data from 87 patients with MDS (n = 67) and chronic myelomonocytic leukemia (n = .20) after failure of decitabine regimens were reviewed. RESULTS After a median follow-up of 21 months from decitabine failure, 13 (15%) patients remained alive; the median survival was 4.3 months, and the estimated 12-month survival rate was 28%. The estimated 12-month survival rates were 27%, 33%, and 33%, respectively, for patients with high-risk, intermediate-2-risk, and intermediate-1-risk disease (P = .99) by the International Prognostic Scoring System. The estimated 12-month survival rates were 100%, 54%, 41%, and 18%, respectively, for patients with low-risk, intermediate-1-risk, intermediate-2-risk, and high-risk disease according to The University of Texas M. D. Anderson Cancer Center risk model (P = .01). CONCLUSIONS The outcome of patients after decitabine failure is poor and appears to be predictable after decitabine failure. PMID:20564137

Jabbour, Elias; Garcia-Manero, Guillermo; Batty, Nicolas; Shan, Jenny; O’Brien, Susan; Cortes, Jorge; Ravandi, Farhad; Issa, Jean-Pierre; Kantarjian, Hagop

2015-01-01

262

Therapy in endocrine disease: treatment of hirsutism in the polycystic ovary syndrome.  

PubMed

Hirsutism is a common medical complaint among women of reproductive age, and it affects the majority of women with the polycystic ovary syndrome (PCOS). Increased rate of androgen production and its availability in tissue represent the main pathophysiological mechanisms responsible for hirsutism. In addition, androgens may be generated de novo in the hair follicle; therefore, circulating androgen levels do not quantify the real exposure of the hair follicle to androgens, as a quota is locally generated. Hirsutism is a clinical sign and not a disease in itself; its presence does not therefore necessarily require treatment, particularly in mild-to-moderate forms, and when an affected woman does not worry about it. Physicians should decide whether hirsutism is to be treated or not by evaluating not only the severity of the phenomenon but also the subjective perception of the patient, which does not necessarily correspond to the true extent of hair growth. In any case, a physician should manage a woman with hirsutism only on the basis of a diagnosis of the underlying cause, and after a clear explanation of the efficacy of each therapeutic choice. Cosmetic procedures and pharmacological intervention are commonly used in the treatment of hirsutism and are discussed in this paper. Importantly, there are different phenotypes of women with hirsutism and PCOS that may require specific attention in the choice of treatment. In particular, when obesity is present, lifestyle intervention should be always considered, and if necessary combined with pharmacotherapy. PMID:24272197

Pasquali, Renato; Gambineri, Alessandra

2014-02-01

263

Intradermal Therapy (Mesotherapy) for the Treatment of Acute Pain in Carpal Tunnel Syndrome: A Preliminary Study  

PubMed Central

Background The carpal tunnel syndrome (CTS) is the most common cause of severe hand pain. In this study we treated acute pain in CTS patients by means of local intradermal injections of anti-inflammatory drugs (mesotherapy). Methods In twenty-five patients (forty-five hands), CTS diagnosis was confirmed by clinical and neurophysiological examination prior to mesotherapy. A mixture containing lidocaine 10 mg, ketoprophen lysine-acetylsalycilate 80 mg, xantinol nicotinate 100 mg, cyanocobalamine 1,000 mcg plus injectable water was used. Sites of injection were three parallel lines above the transverse carpal ligament and two v-shaped lines, one at the base of the thenar eminence, and the other at the base of the hypothenar eminence. Results The day after the treatment, all but four patients reported a significant reduction in pain and paresthesias. After 12 months, 17 patients had a complete pain relief, eight patients reported recurrence of pain and sensory symptoms and four out of them underwent surgical treatment. Conclusions With the obvious limits of a small-size open-label study, our results suggest that mesotherapy can temporary relieve pain and paresthesias in most CTS patients and in some cases its effect seems to be long-lasting. Further controlled studies are needed to confirm our preliminary findings and to compare mesotherapy to conventional approaches for the treatment of CTS. PMID:24478901

Conforti, Giorgio; Capone, Loredana

2014-01-01

264

Posterior Reversible Encephalopathy Syndrome (PRES) as a Complication of Immunosuppressive Therapy in Renal Transplantation in Children  

PubMed Central

Although kidney transplantation is by far the best method of renal replacement therapy, organ receiver is still not spared of eventual toxic consequences of drugs that are in charge of keeping the transplanted kidney functional. Both calcineurin inhibitors, of which tacrolimus more often, occasionally lead to neurotoxic side effects, mostly mild and reversible and dose-dependent in nature, but they can also be very severe or even fatal. It is very important to be aware of possible neurotoxic effects, to confirm them radiologically, and to prevent or reduce drug effects on nervous system. Sometimes the reduction of dose or substitution with another drug with similar mechanism effect is sufficient to terminate the neurotoxic effects of the drug and still not jeopardize the function of transplanted organ. PMID:25568539

Hodzic, Emir; Brcic, Majda; Atic, Mirza; Halilcevic, Alma; Jasarevic, Amila; Aleckovic-Halilovic, Mirna; Trojak, Davor; Atic, Nedima; Zulic, Snezana; Mehmedovic, Zlatan; Iveljic, Ivana

2014-01-01

265

Combination of thrombolytic therapy and angioplastic stent insertion in a patient with Budd-Chiari syndrome  

PubMed Central

A 31-year-old female who had well-established polycythemia vera one year before, presented with the sudden onset. She had severe ascites and hepatic encephalopathy 12 d prior to admission. Real-time ultrasonography revealed a supra hepatic thrombosis extending toward the inferior vena cava (lVC). Thrombolytic therapy with systemic streptokinase (250?000 IU loading + 100?000 IU/h infusion) was started. At the end of 72 h infusion, the patient's general condition improved. A color Doppler ultrasonography then showed complete and partial resolution of the thrombosis in the supra hepatic vein and IVC, respectively. Despite this good response, 12 d later, the symptoms recurred. Venography detected complete obstruction of the lVC. Percutanous balloon angioplasty with stent insertion was performed successfully and the patient was discharged without any evidence of liver disease. A combination of systemic streptokinase and radiological intervention was effective in our patient. PMID:17659745

Reza, Fatemi; Naser, Daryani E; Hossein, Ganaati; Mehrdad, Zahmatkesh

2007-01-01

266

Astrocyte glutamine synthetase: importance in hyperammonemic syndromes and potential target for therapy.  

PubMed

Many theories have been advanced to explain the encephalopathy associated with chronic liver disease and with the less common acute form. A major factor contributing to hepatic encephalopathy is hyperammonemia resulting from portacaval shunting and/or liver damage. However, an increasing number of causes of hyperammonemic encephalopathy have been discovered that present with the same clinical and laboratory features found in acute liver failure, but without liver failure. Here, we critically review the physiology, pathology, and biochemistry of ammonia (i.e., NH3 plus NH4+) and show how these elements interact to constitute a syndrome that clinicians refer to as hyperammonemic encephalopathy (i.e., acute liver failure, fulminant hepatic failure, chronic liver disease). Included will be a brief history of the status of ammonia and the centrality of the astrocyte in brain nitrogen metabolism. Ammonia is normally detoxified in the liver and extrahepatic tissues by conversion to urea and glutamine, respectively. In the brain, glutamine synthesis is largely confined to astrocytes, and it is generally accepted that in hyperammonemia excess glutamine compromises astrocyte morphology and function. Mechanisms postulated to account for this toxicity will be examined with emphasis on the osmotic effects of excess glutamine (the osmotic gliopathy theory). Because hyperammonemia causes osmotic stress and encephalopathy in patients with normal or abnormal liver function alike, the term "hyperammonemic encephalopathy" can be broadly applied to encephalopathy resulting from liver disease and from various other diseases that produce hyperammonemia. Finally, the possibility that a brain glutamine synthetase inhibitor may be of therapeutic benefit, especially in the acute form of liver disease, is discussed. PMID:20880508

Brusilow, Saul W; Koehler, Raymond C; Traystman, Richard J; Cooper, Arthur J L

2010-10-01

267

Irritable bowel syndrome: a disease still searching for pathogenesis, diagnosis and therapy.  

PubMed

Irritable bowel syndrome (IBS) is the most frequently diagnosed functional gastrointestinal disorder in primary and secondary care. It is characterised by abdominal discomfort, pain and changes in bowel habits that can have a serious impact on the patient's quality of life. The pathophysiology of IBS is not yet completely clear. Genetic, immune, environmental, inflammatory, neurological and psychological factors, in addition to visceral hypersensitivity, can all play an important role, one that most likely involves the complex interactions between the gut and the brain (gut-brain axis). The diagnosis of IBS can only be made on the basis of the symptoms of the Rome III criteria. Because the probability of organic disease in patients fulfilling the IBS criteria is very low, a careful medical history is critical and should pay particular attention to the possible comorbidities. Nevertheless, the severity of the patient's symptoms or concerns sometimes compels the physician to perform useless and/or expensive diagnostic tests, transforming IBS into a diagnosis of exclusion. The presence of alarming symptoms (fever, weight loss, rectal bleeding, significant changes in blood chemistry), the presence of palpable abdominal masses, any recent onset of symptoms in patient aged over 50 years, the presence of symptoms at night, and a familial history of celiac disease, colorectal cancer and/or inflammatory bowel diseases all warrant investigation. Treatment strategies are based on the nature and severity of the symptoms, the degree of functional impairment of the bowel habits, and the presence of psychosocial disorders. This review examines and discusses the pathophysiological aspects and the diagnostic and therapeutic approaches available for patients with symptoms possibly related to IBS, pointing out controversial issues and the strengths and weaknesses of the current knowledge. PMID:25083055

Bellini, Massimo; Gambaccini, Dario; Stasi, Cristina; Urbano, Maria Teresa; Marchi, Santino; Usai-Satta, Paolo

2014-07-21

268

Astrocyte Glutamine Synthetase: Importance in Hyperammonemic Syndromes and Potential Target for Therapy  

PubMed Central

Summary Many theories have been advanced to explain the encephalopathy associated with chronic liver disease and with the less common acute form. A major factor contributing to hepatic encephalopathy is hyperammonemia resulting from portacaval shunting and/or liver damage. However, an increasing number of causes of hyperammonemic encephalopathy have been discovered that present with the same clinical and laboratory features found in acute liver failure, but without liver failure. Here, we critically review the physiology, pathology, and biochemistry of ammonia (i.e., NH3 plus NH4+) and show how these elements interact to constitute a syndrome that clinicians refer to as hyperammonemic encephalopathy (i.e., acute liver failure, fulminant hepatic failure, chronic liver disease). Included will be a brief history of the status of ammonia and the centrality of the astrocyte in brain nitrogen metabolism. Ammonia is normally detoxified in the liver and extrahepatic tissues by conversion to urea and glutamine, respectively. In the brain, glutamine synthesis is largely confined to astrocytes, and it is generally accepted that in hyperammonemia excess glutamine compromises astrocyte morphology and function. Mechanisms postulated to account for this toxicity will be examined with emphasis on the osmotic effects of excess glutamine (the osmotic gliopathy theory). Because hyperammonemia causes osmotic stress and encephalopathy in patients with normal or abnormal liver function alike, the term “hyperammonemic encephalopathy” can be broadly applied to encephalopathy resulting from liver disease and from various other diseases that produce hyperammonemia. Finally, the possibility that a brain glutamine synthetase inhibitor may be of therapeutic benefit, especially in the acute form of liver disease, is discussed. PMID:20880508

Brusilow, Saul W.; Koehler, Raymond C.; Traystman, Richard J.; Cooper, Arthur J. L.

2010-01-01

269

Cognitive behavioural therapy versus multidisciplinary rehabilitation treatment for patients with chronic fatigue syndrome: study protocol for a randomised controlled trial (FatiGo)  

PubMed Central

Background Patients with chronic fatigue syndrome experience extreme fatigue, which often leads to substantial limitations of occupational, educational, social and personal activities. Currently, there is no consensus regarding the treatment. Patients try many different therapies to overcome their fatigue. Although there is no consensus, cognitive behavioural therapy is seen as one of the most effective treatments. Little is known about multidisciplinary rehabilitation treatment, a combination of cognitive behavioural therapy with principles of mindfulness, gradual increase of activities, body awareness therapy and pacing. The difference in effectiveness and cost-effectiveness between multidisciplinary rehabilitation treatment and cognitive behavioural therapy is as yet unknown. The FatiGo (Fatigue-Go) trial aims to compare the effects of both treatment approaches in outpatient rehabilitation on fatigue severity and quality of life in patients with chronic fatigue syndrome. Methods One hundred twenty patients who meet the criteria of chronic fatigue syndrome, fulfil the inclusion criteria and sign the informed consent form will be recruited. Both treatments take 6 months to complete. The outcome will be assessed at 6 and 12 months after the start of treatment. Two weeks after the start of treatment, expectancy and credibility will be measured, and patients will be asked to write down their personal goals and score their current performance on these goals on a visual analogue scale. At 6 and 14 weeks after the start of treatment, the primary outcome and three potential mediators—self-efficacy, causal attributions and present-centred attention-awareness—will be measured. Primary outcomes are fatigue severity and quality of life. Secondary outcomes are physical activity, psychological symptoms, self-efficacy, causal attributions, impact of disease on emotional and physical functioning, present-centred attention-awareness, life satisfaction, patient personal goals, self-rated improvement and economic costs. The primary analysis will be based on intention to treat, and longitudinal analysis of covariance will be used to compare treatments. Discussion The results of the trial will provide information on the effects of cognitive behavioural therapy and multidisciplinary rehabilitation treatment at 6 and 12 months follow-up, mediators of the outcome, cost-effectiveness, cost-utility, and the influence of treatment expectancy and credibility on the effectiveness of both treatments in patients with chronic fatigue syndrome. Trial registration Current Controlled Trials ISRCTN77567702. PMID:22647321

2012-01-01

270

Continuous renal replacement therapy in children with multiple organ dysfunction syndrome: A case series.  

PubMed

There is a lack of definitive information regarding the precise indications, implementation, and outcomes of continuous renal replacement therapy (CRRT) for the treatment of critically ill children. Six children (three boys, three girls) aged from 3 days to 8 years, all of whom had multiple organ failure, were submitted to bedside CRRT using M60 filter membranes. Modified Port carbonate formula was used and clotting time was maintained between 20 and 30 minutes. Activated partial thromboplastin time was 1.5- to 2-fold normal. One patient discontinued treatment due to family decision. Marked improvements were seen in the remaining five patients, including normalization of blood urea nitrogen and creatinine levels, stabilization of electrolytes, and improvements in markers of organ function. Of note, one patient (a six-year-old male) underwent the treatment for 241 hours. All five patients were subsequently discharged and recovered uneventfully. CRRT is effective for the management of children who are critically ill due to multiple organ failure. PMID:25615255

Zhang, Yan-Lin; Hu, Wei-Ping; Zhou, Ling-Hui; Wang, Yin; Cheng, Ao; Shao, Si-Nan; Hong, Ling-Ling; Chen, Qiu-Yue

2014-01-01

271

Thrombolytic Therapy for Cerebral Vein Thrombosis in Antiphospholipid Syndrome Secondary to Systemic Lupus Erythematosus  

PubMed Central

A 20-year-old woman was admitted to a Gynecology Hospital in her 6th month of pregnancy for high blood pressure and tonic-clonic seizure. Primary diagnosis was eclampsia, and for that reason she underwent cesarean section. She also had headache on frontal and parietal areas without nausea or vomiting. There was not a focal neurological sign. Rheumatology consultation was requested. Systemic lupus erythematosus and secondary antiphospholipid (APS) was confirmed. The patient had headache that continued several days after cesarean section, therefore, brain magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) were performed, and cerebral vein thrombosis was documented. Distal segment of right lateral sinus and sigmoid sinus were not appeared in brain MRV. Abnormal hypersignal intensity of right lateral sinus/coronal T2 was detected. Thrombolytic therapy with 20 mg tissue plasminogen activator on right sigmoid and transverse sinus was performed by an interventional neurologist. After this procedure, the patient's headache healed and she was discharged in a good condition. PMID:23115432

Hajialilo, Mehrzad; Noshad, Hamid; Mohammadian, Reza; Khabbazi, Ali Reza; Daghighi, Mohammad Hossein; Saleh, Parviz

2012-01-01

272

[Molecular mechanisms of ischemic-reperfusion syndrome and its personalized therapy].  

PubMed

Cardiovascular pathologies are the major causes of morbidity and mortality in the world. Cessation of the blood flow in large vessels, supplying tissues with oxygen and substrates, leads to ischemic conditions accompanied by unwanted shifts of oxidative metabolism and rise of the reactive oxygen species (ROS) generation. Small amounts of ROS are essential elements of the cell metabolism, however pathological elevation of ROS jeopardizes the survival of cells, organs and even organisms. Paradoxically, blood flow restoration during prolonged ischemia leads to oxidative stress that is often fatal for a live system. Oxygen paradox appears to be a limiting factor in clinical practice that intuitively seeks for immediate and complete restoration of a damaged blood flow. Mitochondrion is a major ROS source and a key element of pro-apoptotic signaling, however it is clear, that mitochondria are the main target for anti-ischemic treatment. In the present review we consider two ways of such anti-ischemic strategy, bringing ischemic tolerance to the organ through mitochondrial involvement, such as intrinsic, biological, or artificial, pharmacological adaptive systems (preconditioning). The latter is aimed to simulate elements and high efficiency of intrinsic protective system. The role of antioxidants in anti-ischemic therapy and their effects on preconditioning signaling are discussed in the review. PMID:25306686

Grebenchikov, O A; Likhvantsev, V V; Plotnikov, E Iu; Silachev, D N; Pevzner, I B; Zorova, L D; Zorov, D B

2014-01-01

273

Advances in the development of novel antioxidant therapies as an approach for fetal alcohol syndrome prevention.  

PubMed

Ethanol is the most common human teratogen, and its consumption during pregnancy can produce a wide range of abnormalities in infants known as fetal alcohol spectrum disorder (FASD). The major characteristics of FASD can be divided into: (i) growth retardation, (ii) craniofacial abnormalities, and (iii) central nervous system (CNS) dysfunction. FASD is the most common cause of nongenetic mental retardation in Western countries. Although the underlying molecular mechanisms of ethanol neurotoxicity are not completely determined, the induction of oxidative stress is believed to be one central process linked to the development of the disease. Currently, there is no known effective strategy for prevention (other than alcohol avoidance) or treatment. In the present review we will provide the state of art in the evidence for the use of antioxidants as a potential therapeutic strategy for the treatment using whole-embryo and culture cells models of FASD. We conclude that the imbalance of the intracellular redox state contributes to the pathogenesis observed in FASD models, and we suggest that antioxidant therapy can be considered a new efficient strategy to mitigate the effects of prenatal ethanol exposure. Birth Defects Research (Part A), 2014. © 2014 Wiley Periodicals, Inc. PMID:25131946

Joya, Xavier; Garcia-Algar, Oscar; Salat-Batlle, Judith; Pujades, Cristina; Vall, Oriol

2014-08-18

274

Hyperbaric Oxygen Therapy Can Improve Post Concussion Syndrome Years after Mild Traumatic Brain Injury - Randomized Prospective Trial  

PubMed Central

Background Traumatic brain injury (TBI) is the leading cause of death and disability in the US. Approximately 70-90% of the TBI cases are classified as mild, and up to 25% of them will not recover and suffer chronic neurocognitive impairments. The main pathology in these cases involves diffuse brain injuries, which are hard to detect by anatomical imaging yet noticeable in metabolic imaging. The current study tested the effectiveness of Hyperbaric Oxygen Therapy (HBOT) in improving brain function and quality of life in mTBI patients suffering chronic neurocognitive impairments. Methods and Findings The trial population included 56 mTBI patients 1–5 years after injury with prolonged post-concussion syndrome (PCS). The HBOT effect was evaluated by means of prospective, randomized, crossover controlled trial: the patients were randomly assigned to treated or crossover groups. Patients in the treated group were evaluated at baseline and following 40 HBOT sessions; patients in the crossover group were evaluated three times: at baseline, following a 2-month control period of no treatment, and following subsequent 2-months of 40 HBOT sessions. The HBOT protocol included 40 treatment sessions (5 days/week), 60 minutes each, with 100% oxygen at 1.5 ATA. “Mindstreams” was used for cognitive evaluations, quality of life (QOL) was evaluated by the EQ-5D, and changes in brain activity were assessed by SPECT imaging. Significant improvements were demonstrated in cognitive function and QOL in both groups following HBOT but no significant improvement was observed following the control period. SPECT imaging revealed elevated brain activity in good agreement with the cognitive improvements. Conclusions HBOT can induce neuroplasticity leading to repair of chronically impaired brain functions and improved quality of life in mTBI patients with prolonged PCS at late chronic stage. Trial Registration ClinicalTrials.gov NCT00715052 PMID:24260334

Fishlev, Gregori; Bechor, Yair; Volkov, Olga; Bergan, Jacob; Friedman, Mony; Hoofien, Dan; Shlamkovitch, Nathan; Ben-Jacob, Eshel; Efrati, Shai

2013-01-01

275

The KDM2B- Let-7b -EZH2 Axis in Myelodysplastic Syndromes as a Target for Combined Epigenetic Therapy  

PubMed Central

Both DNA and histone methylation are dysregulated in the myelodysplastic syndromes (MDS). Based on preliminary data we hypothesized that dysregulated interactions of KDM2B, let-7b and EZH2 signals lead to an aberrant epigenetic landscape. Gene expression in CD34+ cells from MDS marrows was analyzed by NanoString miR array and validated by real-time polymerase chain reaction (PCR). The functions of KDM2B, let-7b and EZH2 were characterized in myeloid cell lines and in primary MDS cells. Let-7b levels were significantly higher, and KDM2B and EZH2 expression was lower in primary CD34+ MDS marrow cells (n?=?44) than in healthy controls (n?=?21; p<0.013, and p<0.0001, respectively). Overexpression of let-7b reduced EZH2 and KDM2B protein levels, and decreased cells in S-phase while increasing G0/G1 cells (p?=?0.0005), accompanied by decreased H3K27me3 and cyclin D1. Silencing of KDM2B increased let-7b expression. Treatment with the cyclopentanyl analog of 3-deazaadenosine, DZNep, combined with the DNA hypomethylating agent 5-azacitidine, decreased levels of EZH2, suppressed methylation of di- and tri-methylated H3K27, and increased p16 expression, associated with cell proliferation. Thus, KDM2B, via let-7b/EZH2, promotes transcriptional repression. DZNep bypassed the inhibitory KDM2B/let-7b/EZH2 axis by preventing H3K27 methylation and reducing cell proliferation. DZNep might be able to enhance the therapeutic effects of DNA hypomethylating agents such as 5-azacitidine, currently considered standard therapy for patients with MDS. PMID:25225797

Karoopongse, Ekapun; Yeung, Cecilia; Byon, John; Ramakrishnan, Aravind; Holman, Zaneta J.; Jiang, Peter Y. Z.; Yu, Qiang; Deeg, H. Joachim; Marcondes, A. Mario

2014-01-01

276

Non-surgical therapy for bilateral glossopharyngeal neuralgia caused by Eagle's syndrome, diagnosed by three-dimensional computed tomography: a case report.  

PubMed

Eagle's syndrome is an uncommon sequela of elongation of the styloid process. Symptoms include recurrent throat pain and anterolateral neck pain, with referred pain to the ear. We report a 65-year-old man who presented with bilateral glossopharyngeal neuralgia. We performed three-dimensional computed tomography which revealed that the right styloid process was 35.1 mm and the left process was 29.6 mm, leading to diagnosis of an elongated styloid process, i.e. Eagle's syndrome. Because the patient refused surgical treatment, conservative therapy was selected. Oral gabapentin, stellate ganglion block, and 8 % lidocaine spray on the tonsillar branches of the glossopharyngeal nerve resulted in complete resolution of the paroxysms of pain in approximately 3 weeks. PMID:22760524

Kawasaki, Masanori; Hatashima, Sunao; Matsuda, Tomio

2012-12-01

277

Olmsted Syndrome  

PubMed Central

Olmsted syndrome is a rare congenital, sharply circumscribed transgredient palmoplantar keratoderma. It was first described by Olmsted in 1927. The diagnosis of this rare disease depends on clinical features like symmetrical involvement of keratoderma of the palms and soles and the symmetrical hyperkeratotic plaques around the body orifices. It starts in the neonatal period or in childhood. The disease has a slow but progressive and extremely disabling course. Treatment of Olmsted syndrome is often based on topical therapy with retinoic acid, corticosteroid, emollients, and keratolytics. The present paper describes a case of Olmsted syndrome and its treatment. PMID:23320205

Elise Tonoli, Renata; De Villa, Damię; Hübner Frainer, Renata; Pizzarro Meneghello, Luana; Ricachnevsky, Nelson; de Quadros, Maurício

2012-01-01

278

Learning about Turner Syndrome  

MedlinePLUS

... hormone replacement therapy. Top of page How is Turner syndrome diagnosed? A diagnosis of Turner syndrome may be suspected when there are a number ... slow growth rate and other features are identified. Diagnosis sometimes takes place later ... syndrome may be suspected in pregnancy during an ultrasound ...

279

Morbidity in Turner Syndrome  

Microsoft Academic Search

Turner syndrome afflicts approximately 50 per 100,000 females and is characterized by retarded growth, gonadal dysgenesis, and infertility. Much attention has been focused on growth and growth promoting therapies, while less is known about the natural course of the syndrome, especially in adulthood. We undertook this study to assess the incidence of diseases relevant in the study of Turner syndrome.

Claus Hřjbjerg Gravholt; Svend Juul; Rune Weis Naeraa; Jan Hansen

1998-01-01

280

Effects of immunosuppressive therapy in a patient with aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome during ongoing eculizumab treatment.  

PubMed

A 65-year-old woman experienced a hemolytic attack triggered by sepsis. She presented with markedly increased CD55(-) CD59(-) erythrocytes and the signs of bone marrow failure, which led to a diagnosis of aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome. There was a possibility of increasing hemolysis, as large PNH clones remained after immunosuppressive therapy (IST). Accordingly, eculizumab was first used to control the hemolytic attack followed by IST with antithymocyte globulin and cyclosporine A. The patient was successfully weaned from blood transfusions and has been followed up without any recurrence of hemolytic attacks. PMID:24429452

Asano, Jin; Ueda, Ryosuke; Tanaka, Yasuhiro; Shinzato, Isaku; Takafuta, Toshiro

2014-01-01

281

[Effects of mitotane therapy in dogs with pituitary dependent Cushing syndrome on the adrenal gland size--an ultrasonographic study].  

PubMed

The effect of mitotane therapy on adrenal gland size was evaluated in 13 dogs with pituitary dependent hyperadrenocorticism. Ultrasonographic measurements were obtained before and during mitotane therapy. During therapy both adrenal glands were shorter and thinner (median during therapy: left adrenal gland 19.4 mm long, 5.4 mm thick, right adrenal gland 18.1 mm long, 6.1 mm thick) than before mitotane therapy (median before therapy: left adrenal gland 23.6 mm long, 8.3 mm thick, right adrenal gland 21.6 mm long, 8.1 mm thick). Statistical evaluation showed a significant reduction in size. But ultrasonographic measurement of adrenal gland size is not useful in the evaluation of adrenal reserve during mitotane therapy. Inadequate adrenal reserve was not identified and adrenal size measurement by ultrasonography was not helpful to differentiate adequate and inadequate control of adrenal cortisol secretion during mitotane therapy. PMID:10354742

Hörauf, A; Reusch, C

1999-01-01

282

Cost-utility analysis of genotype-guided antiplatelet therapy in patients with moderate-to-high risk acute coronary syndrome and planned percutaneous coronary intervention  

PubMed Central

Background Prasugrel is recommended over clopidogrel in poor/intermediate CYP2C19 metabolizers with acute coronary syndrome (ACS) and planned percutaneous coronary intervention (PCI), reducing the risk of ischemic events. CYP2C19 genetic testing can guide antiplatelet therapy in ACS patients. Objective The purpose of this study was to evaluate the cost-utility of genotype-guided treatment, compared with prasugrel or generic clopidogrel treatment without genotyping, from the US healthcare provider’s perspective. Methods A decision model was developed to project lifetime economic and humanistic burden associated with clinical outcomes (myocardial infarction [MI], stroke and major bleeding) for the three strategies in patients with ACS. Probabilities, costs and age-adjusted quality of life were identified through systematic literature review. Incremental cost-utility ratios (ICURs) were calculated for the treatment strategies, with quality-adjusted life years (QALYs) as the primary effectiveness outcome. Relative risk of developing myocardial infarction and stroke between patients with and without variant CYP2C19 when receiving clopidogrel were estimated to be 1.34 and 3.66, respectively. One-way and probabilistic sensitivity analyses were performed. Results Clopidogrel cost USD19,147 and provided 10.03 QALYs versus prasugrel (USD21,425, 10.04 QALYs) and genotype-guided therapy (USD19,231, 10.05 QALYs). The ICUR of genotype-guided therapy compared with clopidogrel was USD4,200. Genotype-guided therapy provided more QALYs at lower costs compared with prasugrel. Results were sensitive to the cost of clopidogrel and relative risk of myocardial infarction and stroke between CYP2C19 variant vs. non-variant. Net monetary benefit curves showed that genotype-guided therapy had at least 70% likelihood of being the most cost-effective alternative at a willingness-to-pay of USD100,000/QALY. In comparison with clopidogrel, prasugrel therapy was more cost-effective with <21% certainty at willingness-to-pay of >USD170,000/QALY. Conclusions Our modeling analyses suggest that genotype-guided therapy is a cost-effective strategy in patients with acute coronary syndrome undergoing planned percutaneous coronary intervention. PMID:25243032

Patel, Vardhaman; Lin, Fang-Ju; Ojo, Olaitan; Rao, Sapna; Yu, Shengsheng; Zhan, Lin; Touchette, Daniel R.

2014-01-01

283

Statin therapy in patients with acute coronary syndrome: low-density lipoprotein cholesterol goal attainment and effect of statin potency  

PubMed Central

Background Elevated low-density lipoprotein cholesterol (LDL-C) is associated with an increased risk of coronary artery disease. Current guidelines recommend an LDL-C target of <70 mg/dL (<1.8 mmol/L) for acute coronary syndrome (ACS) patients, and the first-line treatment to lower lipids is statin therapy. Despite current guidelines and the efficacious lipid-lowering agents available, about half of patients at very high risk, including ACS patients, fail to achieve their LDL-C goal. This study assessed LDL-C goal attainment according to use of high and low potency statins in routine practice in Thailand. Methods A retrospective cohort study was performed by retrieving data from medical records and the electronic hospital database for a tertiary care hospital in Thailand between 2009 and 2011. Included were ACS patients treated with statins at baseline and with follow-up of LDL-C levels. Patients were divided into high or low potency statin users, and the proportion reaching the LDL-C goal of <70 mg/dL was determined. A Cox proportional hazard model was applied to determine the relationship between statin potency and LDL-C goal attainment. Propensity score adjustment was used to control for confounding by indication. Results Of 396 ACS patients (60% males, mean age 64.3±11.6 years), 229 (58%) were treated with high potency statins and 167 (42%) with low potency statins. A quarter reached their target LDL-C goal (25% for patients on high potency statins and 23% on low potency statins). High potency statins were not associated with increased LDL-C goal attainment (adjusted hazards ratio 1.22, 95% confidence interval 0.79–1.88; P=0.363). Conclusion There was no significant effect of high potency statins on LDL-C goal attainment. Moreover, this study showed low LDL-C goal attainment for patients on either low or high potency statins. The reasons for the low LDL-C goal attainment rate warrants further investigation.

Chinwong, Dujrudee; Patumanond, Jayanton; Chinwong, Surarong; Siriwattana, Khanchai; Gunaparn, Siriluck; Hall, John Joseph; Phrommintikul, Arintaya

2015-01-01

284

5-HT3 receptor antagonists. 3. Quinoline derivatives which may be effective in the therapy of irritable bowel syndrome.  

PubMed

A series of quinolinecarboxylic acid derivatives has been previously described as a new class of 5-HT3 receptor antagonists due to deviation of a carbonyl moiety from the place of an aromatic ring in their minimum-energy conformations. These derivatives were evaluated in a wrap-restraint stress-induced defecation model in rats. Reference compounds, ondansetron (1), granisetron (2), and YM060 (4), potently inhibited a stress-induced increase in stools excreted from fed rats (ID50 = 0.27, 0.12, and 0.0052 mg/kg, po, respectively). However, quinoline derivatives exhibited different activities depending on structural class. 4-Hydroxyquinoline-3-carboxylic acid derivatives 5 and 6a possess high affinity for the 5-HT3 receptor (Ki = 6.1 and 1.5 nM, respectively) and exhibit potent activity in the Bezold-Jarisch (B-J) reflex test (ED50 = 0.0017 and 0.000 10 mg/kg, i.v., respectively), but they did not effectively inhibit the increase in fecal pellet output at the dose of 1 mg/kg, po. On the other hand, most of 1-substituted 2-oxoquinoline-4-carboxylates 10 showed less potent activity in the B-J reflex test than 1 or 2 but inhibited restraint stress-induced defecation more potently than 1 or 2. The ID50 value of endo-8-methyl-8- azabicyclo[3.2.1]oct-3-yl 1-isobutyl-2-oxo-1,2-dihydro-4- quinolinecarboxylate 10e was 0.013 mg/kg, po. With respect to the selected compounds 6a and 10e, effects of 5-HT- and thyrotropin-releasing hormone (TRH)-induced defecation, castor oil-induced diarrhea and wrap-restraint stress-induced colonic propulsion in rats were examined. These 5-HT3 receptor antagonists did not effectively inhibit castor oil-induced diarrhea, which has been reported not to be mediated via the 5-HT3 receptor. Although 10e showed 800-fold decreased potency compared with 4 in the B-J reflex test, 10e exhibited activity as potent as 4 in 5-HT- and TRH-induced defecation assays; 10e exhibited 7-fold increased potency compared with 4 in wrap-restraint stress-induced colonic propulsions. From these results, 10e appears to interact selectively with 5-HT3 receptors in the gastrointestinal system and might be effective in the therapy of irritable bowel syndrome (IBS). PMID:8230119

Kishibayashi, N; Miwa, Y; Hayashi, H; Ishii, A; Ichikawa, S; Nonaka, H; Yokoyama, T; Suzuki, F

1993-10-29

285

Immune Reconstitution Inflammatory Syndrome following Antiretroviral Therapy Initiation in Tuberculosis Patients: Findings from the SAPiT Trial  

PubMed Central

Background Concerns about immune reconstitution inflammatory syndrome (IRIS) remain a barrier to antiretroviral therapy (ART) initiation during anti-tuberculosis treatment in co-infected patients. Objective We assessed IRIS incidence, severity, and outcomes relative to timing of ART initiation in patients with HIV-related tuberculosis (HIV-TB). Setting An outpatient clinic in Durban, South Africa Patients 642 HIV-TB co-infected patients Design In a secondary analysis of the SAPiT trial, IRIS was assessed in patients randomized to initiate ART either within four weeks of tuberculosis treatment initiation (early integrated-treatment arm), within four weeks of completion of the intensive phase of tuberculosis treatment (late integrated-treatment arm) or within four weeks after tuberculosis therapy completion (sequential-treatment arm). IRIS was defined as new onset or worsening symptoms, signs or radiographic manifestations temporally related to treatment initiation accompanied by a treatment response. IRIS severity, hospitalization and time to resolution were monitored. Results IRIS incidence was 19.5 (n=43), 7.5 (n=18) and 8.1 (n=19) per 100 person-years in the early integrated-, late integrated-, and sequential-treatment arms, respectively; P < 0.001, and 45.5, 9.7 and 19.7 per 100 person-years in patients with baseline CD4+ counts <50 cells/mm3, P = 0.004. IRIS incidence was higher in the early integrated- compared to the late integrated- (incidence rate ratio (IRR) = 2.6, 95%confidence interval (CI): 1.5 to 4.8; P < 0.001) or sequential-treatment arm (IRR=2.4, 95%CI: 1.4 to 4.4; P < 0.001). IRIS cases in the early integrated-treatment arm were more severe (34.9% vs. 18.9%, P = 0.18); had significantly higher hospitalization rates (18/43 vs. 5/37; P = 0.01), and longer time to resolution (70.5 vs. 29.0 days; P = 0.001) compared to IRIS cases in the other two arms. Limitation IRIS could not be assessed, due to LTFU, withdrawal or death within 6 months of scheduled ART initiation, in more patients from the sequential treatment arm (n=74) than in the late integrated treatment arm (n=50) and in the early integrated treatment arm (n=32). This study did not assess IRIS risk in non-ambulant patients and in patients with extra-pulmonary and smear negative pulmonary tuberculosis. Conclusion Initiation of ART early during tuberculosis treatment resulted in significantly higher IRIS rates, with longer time to resolution, and more severe cases of IRIS requiring hospitalization. These findings, particularly relevant to patients initiating ART with CD4+ counts < 50 cells/mm3, need to be considered together with the increased survival benefit of early ART initiation in this group. Clintrials.gov: NCT00398996 PMID:22944873

Naidoo, Kogieleum; Yende-Zuma, Nonhlanhla; Padayatachi, Nesri; Naidoo, Kasavan; Jithoo, Niraksha; Nair, Gonasagrie; Bamber, Sheila; Gengiah, Santhana; El-Sadr, Wafaa M.; Friedland, Gerald; Karim, Salim Abdool

2012-01-01

286

Using a digital game for training desirable behavior in cognitive-behavioral therapy of burnout syndrome: a controlled study.  

PubMed

Burnout is a globally increasing illness, and as a result, many forms of burnout therapy have arisen. The use of digital games can be psychotherapeutically effective because they can transform exercises that are by themselves unattractive into intrinsically motivated action. This pilot study aims to test whether a specially designed game contributes to patients learning desired behavior and achieving other specific therapeutic goals in an online cognitive-behavioral therapy (CBT)-based burnout treatment context. In total, 101 participants took part in the experiment, under four conditions: (a) Game+Therapy, (b) Therapy Only, (c) Game Only, and (d) No Game+No Therapy. Pre- and postmeasures were taken online. Results showed that the two therapy conditions (Game+Therapy and Therapy Only) showed a greater decrease in complaints and disengagement, and a stronger increase in coping skills than the nontherapy conditions (Game Only and No Game+No Therapy). As expected, the Game+Therapy condition outperformed the Therapy Only condition on combined improvement measures of burnout symptoms. However, analyses of individual measures showed no effects. It can be cautiously concluded that the therapeutic digital game may be a useful tool when embedded in a therapeutic burnout treatment program and is probably more efficient than CBT, as it is used in current practice. PMID:25684611

Zielhorst, Thomas; van den Brule, Daphne; Visch, Valentijn; Melles, Marijke; van Tienhoven, Sam; Sinkbaek, Helle; Schrieken, Bart; Tan, Eduard S-H; Lange, Alfred

2015-02-01

287

Selective compartmental dominance: an explanation for a noninfectious, multifactorial etiology for acquired immune deficiency syndrome (AIDS), and a rationale for ozone therapy and other immune modulating therapies  

Microsoft Academic Search

The most widely accepted etiological explanation for acquired immune deficiency syndrome (AIDS) currently invokes an infectious model involving the human immunodeficiency virus (HIV). Because this infectious model has failed to meet any conventional criteria for establishing microbial causation, this theory still relies on the high, though not perfect, statistical correlation linking presence of HIV antibodies with patients diagnosed with, and

F. Shallenberger

1998-01-01

288

A Survey of Radiation-Induced Bronchiolitis Obliterans Organizing Pneumonia Syndrome After Breast-Conserving Therapy in Japan  

SciTech Connect

Purpose: We observed a rare and unique occurrence of radiation-induced pulmonary injury outside the tangential field for early breast cancer treatment. The findings appeared to be idiopathic and were called radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome. We surveyed major hospitals in Japan to review their findings of radiation-induced BOOP, in particular the clinical and pictorial characteristics of the entity. Methods and Materials: We reviewed surveys completed and returned by 20 institutions. The survey responses were based on a total of 37 cases of BOOP syndrome. We also reviewed X-ray and computed tomography scans provided by these institutions. We discussed the information derived from the questionnaire and analyzed patients' characteristics, methods used in the treatment of BOOP syndrome, and prognosis. Results: The incidence of the radiation-induced BOOP syndrome was about 1.8% (37 of 2,056). We did not find a relationship between the characteristics of patients and the occurrence of radiation-induced BOOP syndrome. The pulmonary findings were classified into four patterns on chest computed tomography scans. Progression of the pulmonary lesions observed on chest X-ray was classified into three patterns. Pneumonitis appeared within 6 months after radiotherapy was completed and disappeared within 6-12 months after its onset. At 5-year follow-up, 2 patients had died, 1 of breast cancer and the other of interstitial pneumonitis, which seemed to be idiopathic and unrelated to the radiation-induced BOOP syndrome. Conclusions: Although the incidence of BOOP syndrome and its associated prognosis are not significant, the patients' clinical condition must be carefully followed.

Ogo, Etsuyo [Department of Radiology, Kurume University School of Medicine, Kurume (Japan)], E-mail: etsuogo@med.kurume-u.ac.jp; Komaki, Ritsuko [Department of Radiation Oncology, The University of Texas, M.D. Anderson Cancer Center, Houston, TX (United States); Fujimoto, Kiminori; Uchida, Masafumi; Abe, Toshi [Department of Radiology, Kurume University School of Medicine, Kurume (Japan); Nakamura, Katsumasa [Department of Radiology, Faculty of Medicine, Fukuoka University, Fukuoka (Japan); Mitsumori, Michihide [Department of Radiology, Faculty of Medicine, Kyoto University, Kyoto (Japan); Sekiguchi, Kenji [Department of Radiation Oncology, St. Luke's International Hospital, Tokyo (Japan); Kaneyasu, Yuko [Department of Radiology, Faculty of Medicine, Hiroshima University, Hiroshima (Japan); Hayabuchi, Naofumi [Department of Radiology, Kurume University School of Medicine, Kurume (Japan)

2008-05-01

289

Long-term effect of extracorporeal shock wave therapy on the treatment of chronic pelvic pain syndrome due to non bacterial prostatitis  

PubMed Central

Background: There is limited evidence about the chronic pelvic pain syndrome (CPPS) treatment by shockwave therapy, and the aim of this study was to evaluate the effect of extracorporeal shockwave therapy on CPPS due to non bacterial prostatitis in a long-term period. Materials and Methods: In a follow-up survey, 40 patients with CPPS (that were randomly distributed into the treatment or sham groups were evaluated at 16, 20, and 24 weeks. In the treatment group, patients were treated by extracorporeal shock wave therapy (ESWT) once a week for 4 weeks by a protocol of 3000 impulses, 0.25 mJ/m2 and 3 Hz of frequency. 0.05 mJ/m2 were added in each week. In the sham group, the same protocol was applied, but with the probe being turned off. The follow-up assessments were done by visual analog scale for pain and National Institutes of Health-developed Chronic Prostatitis Symptom Index (NIH-CPSI). Data were compared using independent t-test or analysis of variences. Results: Three patients did not complete the study protocol, 37 patients were evlauated (19 patients in treatment and 18 patients in the sham group). At week 24, the mean of pain score, urinary score, quality-of-life and NIH-CPSI score between two groups were not statistically different. Conclusion: Although, ESWT therapy as a safe and effective therapy in CPPS in short-term follow-up has been established, its long-term efficacy was not supported by this study. PMID:25097599

Moayednia, Amir; Haghdani, Saeid; Khosrawi, Saeid; Yousefi, Elham; Vahdatpour, Babak

2014-01-01

290

Efficacy and safety of individually tailored antiplatelet therapy in patients with acute coronary syndrome after coronary stenting: a single center, randomized, feasibility study  

PubMed Central

Background Low responsiveness to clopidogrel (LRC) is associated with increased risk of ischemic events. This study was aimed to explore the feasibility of tailored antiplatelet therapy according to the responsiveness to clopidogrel. Methods A total of 305 clopidogrel naďve patients with acute coronary syndromes (ACS) undergoing coronary stenting were randomly assigned to receive standard (n = 151) or tailored (n = 154) antiplatelet therapy. The ADP-induced platelet aggregation tests by light transmission aggregometry were performed to identify LRC patients assigned to the tailored group. The standard antiplatelet regimen was dual antiplatelet therapy with aspirin and clopidogrel. The tailored antiplatelet therapy was standard regimen for non-LRC patients and an additional 6-month cilostazol treatment for LRC patients. The primary efficacy outcome was the composite of cardiovascular death, myocardial infarction or stroke at one year. Results LCR was present in 26.6% (41/154) of patients in the tailored group. The percentage platelet aggregation for LCR patients was significantly decreased at three days after adjunctive cilostazol treatment (77.5% ± 12.1% vs. 64.5% ± 12.1%, P < 0.001). At one year follow-up, a non-significant 37% relative risk reduction of primary events were observed in the tailored group as compared to the standard group (5.8% vs. 9.3%, P = 0.257). There were no differences in the rates of stent thrombosis and hemorrhagic events between the two groups. Conclusions Tailored antiplatelet therapy for ACS patients after coronary stenting according to responsiveness to clopidogrel is feasible. However, its efficacy and safety need further confirmation by clinical trials with larger sample sizes.

Zhu, Hong-Chang; Li, Yi; Guan, Shao-Yi; Li, Jing; Wang, Xiao-Zeng; Jing, Quan-Min; Wang, Zu-Lu; Han, Ya-Ling

2015-01-01

291

Therapy-related patterns of cytogenetic abnormalities in acute myeloid leukemia and myelodysplastic syndrome post polycythemia vera: single center experience and review of literature.  

PubMed

A minor fraction of patients with polycythemia vera (PV) develop a terminal acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). Analysis of the cytogenetic abnormalities during AML or MDS may help in understanding if this development is part of the natural course of the disease or induced by myelosuppressive therapy. Thirty-six cases with AML or MDS post PV, collected in a single Swedish institution during a 33-year period, are described with special regard to time to development of AML or MDS, therapy given during active PV, and cytogenetic findings during AML or MDS. A further 118 cases of AML or MDS post PV, in whom type of therapy during active PV and cytogenetic findings during AML or MDS were reported, were collected from the literature. AML or MDS developed in our own series after 1-30 years with a fairly constant rate (two cases per year). The most frequent cytogenetic abnormalities were +1q, -5, 5q-, -7, 7q-, +8, +9, 11q-, 13q-, and 20q-. When patients in the total material (n = 154) were divided with regard to treatment during active PV, marked differences were observed. The highest frequency of abnormalities was found in patients given multiple lines of therapy (n = 61), dominating features being -5/5q- in 28 patients (46%), -7/7q- in 19 patients (31%), numerous translocations in 24 patients (39%), and unidentified markers in 22 patients (36%). Half of the patients treated with hydroxyurea alone showed a -5 or 5q- abnormality. In patients treated with phlebotomy alone, +8 and +9 were the most frequent findings. The type of therapy given during active PV influences the type of chromosome abnormalities present during terminal AML or MDS and can also be instrumental in the development of leukemia. PMID:18351338

Swolin, Birgitta; Rödjer, Stig; Westin, Jan

2008-06-01

292

Effect of high-intensity laser therapy in the management of myofascial pain syndrome of the trapezius: a double-blind, placebo-controlled study.  

PubMed

Myofascial pain syndrome (MPS) of the trapezius muscle is one of the main causes of neck pain. In this randomized, double-blind study, we evaluated the effects of high-intensity laser therapy (HILT) in female patients with chronic MPS of the trapezius muscle. The patients were assigned to two groups. The HILT group was treated with HILT and exercise, and the sham therapy group was treated with placebo HILT and exercise. The patients were assessed for pain, cervical active range of motion, disability, and quality of life. Evaluations were performed before treatment (week 0) and after treatment (weeks 4 and 12). Both groups showed significant improvement in all parameters at weeks 4 and 12. However, in a comparison of the percentage changes in the parameters at weeks 4 and 12 relative to pretreatment values, the HILT group showed greater improvement in pain scores, the neck disability index, and several subparts of the short-form 36 health survey (SF-36) (physical functioning, role limitations due to physical functioning, bodily pain, general health perceptions, social functioning, and role limitations due to emotional problems) than did the sham therapy group. We conclude that HILT is an effective therapeutic method in the treatment of patients with chronic MPS of the trapezius muscle. PMID:25274197

Dundar, Umit; Turkmen, Utku; Toktas, Hasan; Solak, Ozlem; Ulasli, Alper Murat

2015-01-01

293

A Case of Plummer-Vinson Syndrome Showing Rapid Improvement of Dysphagia and Esophageal Web after Two Weeks of Iron Therapy  

PubMed Central

Plummer-Vinson syndrome (PVS) is a rare entity characterized by upper esophageal webs and iron deficiency anemia. We report a case of PVS whose esophageal web was rapidly improved by iron therapy. A 77-year-old woman was admitted to our hospital with complaints of dysphagia, vomiting, shortness of breath and weight loss for 1 month. Physical examination revealed conjunctival pallor, koilonychia, angular cheilitis and smooth tongue, and laboratory findings were consistent with microcytic hypochromic anemia with iron deficiency. Gastrointestinal endoscopy and barium-swallow esophagography detected a web that prevented passage of the endoscope into the upper portion of the esophagus. The patient received oral iron therapy daily; the hemoglobin concentration rose to 8.9 g/dl and the complaints of dysphagia were dramatically improved after 2 weeks, with improvement of luminal stenosis confirmed by gastrointestinal endoscopy and barium-swallow esophagography. The PVS described in this report had a distinct clinical course, showing very rapid improvement of dysphagia and esophageal web after 2 weeks of oral iron therapy. PMID:25028578

Tahara, Tomomitsu; Shibata, Tomoyuki; Okubo, Masaaki; Yoshioka, Daisuke; Ishizuka, Takamitsu; Sumi, Kazuya; Kawamura, Tomohiko; Nagasaka, Mitsuo; Nakagawa, Yoshihito; Nakamura, Masakatsu; Arisawa, Tomiyasu; Ohmiya, Naoki; Hirata, Ichiro

2014-01-01

294

A case of plummer-vinson syndrome showing rapid improvement of Dysphagia and esophageal web after two weeks of iron therapy.  

PubMed

Plummer-Vinson syndrome (PVS) is a rare entity characterized by upper esophageal webs and iron deficiency anemia. We report a case of PVS whose esophageal web was rapidly improved by iron therapy. A 77-year-old woman was admitted to our hospital with complaints of dysphagia, vomiting, shortness of breath and weight loss for 1 month. Physical examination revealed conjunctival pallor, koilonychia, angular cheilitis and smooth tongue, and laboratory findings were consistent with microcytic hypochromic anemia with iron deficiency. Gastrointestinal endoscopy and barium-swallow esophagography detected a web that prevented passage of the endoscope into the upper portion of the esophagus. The patient received oral iron therapy daily; the hemoglobin concentration rose to 8.9 g/dl and the complaints of dysphagia were dramatically improved after 2 weeks, with improvement of luminal stenosis confirmed by gastrointestinal endoscopy and barium-swallow esophagography. The PVS described in this report had a distinct clinical course, showing very rapid improvement of dysphagia and esophageal web after 2 weeks of oral iron therapy. PMID:25028578

Tahara, Tomomitsu; Shibata, Tomoyuki; Okubo, Masaaki; Yoshioka, Daisuke; Ishizuka, Takamitsu; Sumi, Kazuya; Kawamura, Tomohiko; Nagasaka, Mitsuo; Nakagawa, Yoshihito; Nakamura, Masakatsu; Arisawa, Tomiyasu; Ohmiya, Naoki; Hirata, Ichiro

2014-05-01

295

[A case of Stevens-Johnson syndrome (SJS) progressive toxic epidermal necrolysis (TEN) onset during hyposensitization therapy for pulmonary tuberculosis complicated with dermatomyositis].  

PubMed

A 58-year-old female with a history of dermatomyositis was receiving large oral doses of steroids. She had pulmonary tuberculosis and developed a fever, systemic exudative erythema, exanthema, and epidermolysis covering 30% of her body surface area while being treated with four agents, including isoniazid (INH) and rifampicin (RFP). Histopathologically, eosinophilic necrosis was observed in all layers of the epidermis and a diagnosis of Stevens-Johnson syndrome (SJS) progressive toxic epidermal necrolysis (TEN) was made. The drugs suspected in the drug-induced lymphocyte stimulation test (DLST) re-testing were INH and RFP, and the DLST was considered to be important during the recovery period as well as in the acute phase. Early treatment with plasma exchange therapy and large quantities of intravenous immunoglobulin (IVIG) was successful. Plasma exchange therapy and IVIG are extremely effective when SJS and TEN occur in a patient already on high-dose steroid therapy. Note that the incidence of SJS and TEN is believed to be higher in patients with collagen disease, such as in our case, as compared to the general population. PMID:22991850

Yamashita, Hiroyuki; Ueda, Yo; Takahashi, Yuko; Mimori, Akio

2012-07-01

296

Cost-effectiveness of long-term intrathecal morphine therapy for pain associated with failed back surgery syndrome  

Microsoft Academic Search

A decision analytic study was conducted using computer simulation to project the outcomes in a simulated cohort of patients whose treatment for back surgery had failed. The objective of this study was to estimate the direct cost of intrathecal morphine therapy (IMT) delivered via an implantable pump relative to alternative therapy (medical management) over a 60-month course of treatment. IMT

Gregory de Lissovoy; Ruth E. Brown; Michael Halpern; Samuel J. Hassenbusch; Edgar Ross

1997-01-01

297

Cognitive Behavior Therapy for Social Anxiety Disorder in the Context of Asperger's Syndrome: A Single-Subject Report  

ERIC Educational Resources Information Center

Asperger's Syndrome (AS) is a developmental disorder characterized by social impairment, highly circumscribed interests, repetitive behaviors, and motor clumsiness. The social impairment features of AS are similar to characteristics of social anxiety disorder (SAD). However, little is known about the comorbidity of these disorders or the treatment…

Cardaciotto, LeeAnn; Herbert, James D.

2004-01-01

298

The growing teratoma syndrome: results of therapy and long-term follow-up of 33 patients  

Microsoft Academic Search

Growing teratoma syndrome (GTS) is defined as an increase in tumour size during or after chemotherapy for germ cell tumour (GCT), and only mature teratoma at histological analysis of the resected tumour specimen. Between 1985 and 1997, 30 male patients fulfilling GTS criteria were included in the present study. 3 female patients were also included but analysed separately. A mature

F André; K Fizazi; S Culine; J.-P Droz; P Taupin; C Lhommé; M.-J Terrier-Lacombe; C Théodore

2000-01-01

299

Mycoplasma-associated stevens-johnson syndrome in children: retrospective review of patients managed with or without intravenous immunoglobulin, systemic corticosteroids, or a combination of therapies.  

PubMed

Administration of intravenous immunoglobulin (IVIG) to patients with Stevens-Johnson syndrome (SJS) has been controversial. The objective of this study was to evaluate the effectiveness of IVIG, systemic corticosteroids, or both in treating Mycoplasma pneumoniae-associated SJS (mpSJS). Retrospective series of 10 pediatric mpSJS cases were stratified into four treatment groups: IVIG alone, IVIG and systemic corticosteroids together, systemic corticosteroids alone, and supportive care. The efficacy of therapy was evaluated on the basis of several proxies of disease severity, including hospital length of stay (LOSt ) and number of febrile days (Febt ) after initiation of therapy. Patients treated with IVIG alone had a longer LOSt and more Febt , despite different baseline characteristics, than patients treated with supportive therapy. Of patients who received IVIG, 50% were treated with corticosteroids concurrently and had similar characteristics of disease severity but showed a non-statistically significant trend toward shorter LOSt and fewer Febt than those who received IVIG alone. A patient treated with corticosteroids alone had the shortest LOSt in this series. Therefore treatment with IVIG alone was associated with a more severe disease course than supportive therapy, although causality cannot be inferred given possible confounding by indication. When systemic corticosteroids were used alone or in conjunction with IVIG, hospital LOSt and Febt trended lower than with the use of IVIG alone, although disease severity at baseline was similar between those treated with IVIG and corticosteroids concurrently and those treated with IVIG alone. It was thus concluded that treatment with systemic corticosteroids as monotherapy or in combination with IVIG may be preferable to IVIG alone. Further large-scale studies are warranted to evaluate this hypothesis. PMID:25424206

Ahluwalia, Jusleen; Wan, Joy; Lee, Diana H; Treat, James; Yan, Albert C

2014-11-01

300

The effects of scapular stabilization based exercise therapy on pain, posture, flexibility and shoulder mobility in patients with shoulder impingement syndrome: a controlled randomized clinical trial  

PubMed Central

Background: Dysfunction in the kinetic chain caused by poor scapula stabilization can contribute to shoulder injuries and Shoulder Impingement Syndrome (SIS). The purpose of this study was to compare the effectiveness of two treatment approaches scapular stabilization based exercise therapy and physical therapy in patients with SIS. Methods: The study is a randomized clinical trial in which 68 patients with SIS were randomly assigned in two groups of exercise therapy (ET) and physical therapy (PT) and received 18 sessions of treatment. Pain, shoulders' range of abduction and external rotation, shoulder protraction, scapular rotation and symmetry as well as postural assessment and Pectoralis minor length were evaluated pre and post intervention. The paired-sample t test and the independent sample t test were applied respectively to determine the differences in each group and between two groups. Results: Our findings indicated significant differences in abduction and external rotation range, improvement of forward shoulder translation and increase in the flexibility of the involved shoulder between the two groups (respectively ; p=0.024, p=0.001, p<0/0001, p<0/0001). No significant difference was detected in pain reduction between the groups (p=0.576). Protraction of the shoulder (p<0.0001), forward head posture (p<0/0001) and mid thoracic curvature (p<0.0001) revealed a significant improvement in the ET group. Apparent changes occurred in scapular rotation and symmetry in both groups but no significant differences were observed between the two groups (respectively; p=0.183, p=0.578). Conclusion: The scapular stabilization based exercise intervention was successful in increasing shoulder range, decreasing forward head and shoulder postures and Pectoralis minor flexibility.

Moezy, Azar; Sepehrifar, Saeed; Solaymani Dodaran, Masoud

2014-01-01

301

Severe Guillain-Barré syndrome in a patient receiving anti-TNF therapy. Consequence or coincidence. A case-based review.  

PubMed

The adverse effects of anti-tumour necrosis factor alpha (TNF?) drugs include an increase in the risk of infections, congestive heart failure, lupus-like syndrome, and the onset or worsening of various demyelinating diseases such as, multiple sclerosis, optic neuritis, and Guillain-Barrč syndrome (GBS), among others. We describe the case of a patient who developed GBS while she was on treatment with adalimumab. A 50-year-old woman with rheumatoid arthritis (RA) was admitted to the hospital due to progressive severe bilateral symmetric weakness of the legs, which quickly extended to the upper limbs and to the respiratory muscles. Adalimumab was started 13 months before. GBS was diagnosed and the anti-TNF? therapy discontinued. The serological test for Campylobacter jejuni was positive. She required invasive mechanical ventilatory support for 9 months. Twelve months later, the patient was using a wheelchair following a rehabilitation programme, and at 24 months she was walking a few steps with assistive devices. The relevant literature on the relationship between GBS and anti-TNF? is reviewed. Twenty three cases of GBS occurring during anti-TNF? therapy have been reported so far in the literature. In several cases, there was no clear temporal association, more than half had a possible previous infection, and in two cases the drug was reintroduced without recurrence of GBS. Our case, which is best explained by C. jejuni infection, as well as some of the cases described are probably not a direct result of anti-TNF? treatment, but an accidental coincidence. We also discuss the potential therapeutic options after anti-TNF? discontinuation. PMID:23666317

Alvarez-Lario, Bonifacio; Prieto-Tejedo, Rosa; Colazo-Burlato, María; Macarrón-Vicente, Jesús

2013-09-01

302

Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy  

PubMed Central

Background The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects. Methods and results Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area–adjusted aortic root diameter z-score >3.0. The mean age at study entry was 11.2 years, 60% were male, and 25% were older teenagers and young adults. The median aortic root diameter z-score was 4.0. Aortic root diameter z-score did not vary with age. Mitral valve prolapse and mitral regurgitation were more common in females. Among those with a positive family history, 56% had a family member with aortic surgery, and 32% had a family member with a history of aortic dissection. Conclusions Baseline demographic, clinical, and anthropometric characteristics of the randomized cohort are representative of patients in this population with moderate to severe aortic root dilation. The high percentage of young subjects with relatives who have had aortic dissection or surgery illustrates the need for more definitive therapy; we expect that the results of the study and the wealth of systematic data collected will make an important contribution to the management of individuals with Marfan syndrome. PMID:23622922

Lacro, Ronald V.; Guey, Lin T.; Dietz, Harry C.; Pearson, Gail D.; Yetman, Anji T.; Gelb, Bruce D.; Loeys, Bart L.; Benson, D. Woodrow; Bradley, Timothy J.; De Backer, Julie; Forbus, Geoffrey A.; Klein, Gloria L.; Lai, Wyman W.; Levine, Jami C.; Lewin, Mark B.; Markham, Larry W.; Paridon, Stephen M.; Pierpont, Mary Ella; Radojewski, Elizabeth; Selamet Tierney, Elif Seda; Sharkey, Angela M.; Wechsler, Stephanie Burns; Mahony, Lynn

2013-01-01

303

Pulsed versus continuous wave low-level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome): a case report  

NASA Astrophysics Data System (ADS)

Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. The transforming growth factor beta has been identified as a major player in the pathogenic process, where low-level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940 nm using millisecond pulsing and continuous wave (CW) modes on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks using a sequential pulsing mode on one elbow and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Considerable functional and morphologic improvements were observed after LLLT, with the best results seen with the pulsing mode. No adverse effects were noted. Pulsed LLLT represents a treatment alternative for osteoarticular signs and symptoms in limited scleroderma (CREST syndrome).

Barolet, Daniel

2014-11-01

304

Single strand conformation polymorphism analysis of androgen receptor gene mutations in patients with androgen insensitivity syndromes: Application for diagnosis, genetic counseling, and therapy  

SciTech Connect

Recent studies indicate that mutations in the androgen receptor gene are associated with androgen insensitivity syndromes, a heterogeneous group of related disorders involving defective sexual differentiation in karyotypic males. In this report, the authors address the possibility of rapid mutational analysis of the androgen receptor gene for initial diagnosis, genetic counseling, and molecular subclassification of affected patients and their families. DNA from peripheral blood leukocytes of six patients from five families with various degrees of androgen insensitivity was studied. Exons 2 to 8 of the androgen receptor gene were analyzed using a combination of single strand conformation polymorphism analysis and direct DNA sequencing. Female family members were also studied to identify heterozygote carriers. Point mutations in the AR gene were identified in all six patients, and all mutations caused amino acid substitutions. One patient with incomplete androgen insensitivity was a mosaic for the mutation. Four of the five mothers, as well as a young sister of one patient, were carriers of the mutation present in the affected child. The data show that new mutations may occur in the androgen receptor gene leading to sporadic androgen insensitivity syndrome. Molecular genetic characterization of the variant allele can serve as a primary tool for diagnosis and subsequent therapy, and can provide a basis for distinguishing heterozygous carriers in familial androgen resistance. The identification of carriers is of substantial clinical importance for genetic counseling. 29 refs., 2 figs., 1 tab.

Hiort, O. (Medizinische Universitaet zu Luebeck (Germany) Tufts-New England Medical Center, Boston, MA (United States)); Huang, Q. (Massachusetts Eye and Ear Infirmary, Boston, MA (United States)); Sinnecker, G.H.G.; Kruse, K. (Medizinische Universitaet zu Luebeck (Germany)); Sadeghi-Nejad, A.; Wolfe, H.J. (Tufts-New England Medical Center, Boston, MA (United States)); Yandell, D.W. (Massachusetts Eye and Ear Infirmary, Boston, MA (United States))(Harvard Medical School, Boston, MA (United States) Harvard School of Public Health, Boston, MA (United States))

1993-07-01

305

Prognostic value of the clinical and laboratory evaluation in patients with nonmosaic Klinefelter syndrome who are receiving assisted reproductive therapy  

Microsoft Academic Search

Objective: To characterize clinical and laboratory findings in nonmosaic 47,XXY patients that may help to predict spermatogenetic activity in their testicles.Design: Prospective study.Setting: Assisted reproductive technology program.Patient(s): Twenty patients with nonmosaic Klinefelter syndrome who underwent testicular sperm retrieval for IVF.Main Outcome Measure(s): The correlation between basal FSH, LH and testosterone levels, mean testicular volume, and results of the hCG test

Igael Madgar; Jehoshua Dor; Ruth Weissenberg; Gil Raviv; Yehezkel Menashe; Jacob Levron

2002-01-01

306

Effect of High-Dose Intravenous Immunoglobulin Therapy in Stevens-Johnson Syndrome: A Retrospective, Multicenter Study  

Microsoft Academic Search

Background:Stevens-Johnson syndrome (SJS) is a severe cutaneous drug reaction associated with considerable morbidity, possible transition to toxic epidermal necrolysis (TEN) and death in certain cases. Objective: To determine whether treatment with high-dose IVIG in SJS patients may improve outcome. Methods: Data from 12 patients (collected between January 1997 and November 2000 from7 university dermatology centers in Europe and North America)

Christa Prins; Carmela Vittorio; R. Steven Padilla; Thomas Hunziker; Peter Itin; John Förster; Eva-B. Bröcker; Jean-Hilaire Saurat; Lars E. French

2003-01-01

307

Long-Term Effectiveness and Compliance of Positional Therapy with the Sleep Position Trainer in the Treatment of Positional Obstructive Sleep Apnea Syndrome  

PubMed Central

Study Objectives: To investigate effectiveness, long-term compliance, and effects on subjective sleep of the Sleep Position Trainer (SPT) in patients with position-dependent obstructive sleep apnea syndrome (POSAS). Design: Prospective, multicenter cohort study. Patients or Participants: Adult patients with mild and moderate POSAS were included. Interventions: Patients asked to use the SPT for 6 mo. At baseline and after 1, 3, and 6 mo, questionnaires would be completed: Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), Functional Outcomes of Sleep Questionnaire (FOSQ), and questions related to SPT use. Measurements and Results: One hundred forty-five patients were included. SPT use and SPT data could not be retrieved in 39 patients. In the remaining 106 patients, median percentage of supine sleep decreased rapidly during SPT's training phase (day 3 to 9) to near-total avoidance of supine sleep. This decrease was maintained during the following months of treatment (21% at baseline versus 3% at 6 mo). SPT compliance, defined as more than 4 h of nightly use, was 64.4%. Regular use, defined as more than 4 h of usage over 5 nights/w, was 71.2%. Subjective compliance and regular use were 59.8% and 74.4%, respectively. Median ESS (11 to 8), PSQI (8 to 6), and FOSQ (87 to 103) values significantly improved compared with baseline. Conclusions: Positional therapy using the Sleep Position Trainer (SPT) effectively diminished the percentage of supine sleep and subjective sleepiness and improved sleep related quality of life in patients with mild to moderate position-dependent obstructive sleep apnea syndrome. SPT treatment appeared to have sustained effects over 6 months. SPT compliance and regular use rate were relatively good. Subjective and objective compliance data corresponded well. The lack of a placebo-controlled group limited the efficacy of conclusions. Citation: van Maamen JP, de Vries N. Long-term effectiveness and compliance of positional therapy with the Sleep Position Trainer in the treatment of positional obstructive sleep apnea syndrome. SLEEP 2014;37(7):1209-1215. PMID:25061249

van Maanen, J. Peter; de Vries, Nico

2014-01-01

308

Successful Management of Insulin Allergy and Autoimmune Polyendocrine Syndrome Type 4 with Desensitization Therapy and Glucocorticoid Treatment: A Case Report and Review of the Literature  

PubMed Central

Introduction. Insulin allergy is a rare complication of insulin therapy, especially in type 1 diabetes mellitus (T1DM). Key manifestations are hypersensitivity-related symptoms and poor metabolic control. T1DM, as well as insulin allergy, may develop in the context of autoimmune polyendocrine syndrome (APS), further complicating management. Case Report. A 17-year-old male patient, diagnosed with T1DM, was treated with various insulin therapy schemes over several months, which resulted in recurrent anaphylactoid reactions and poor glycemic control, after which he was referred to our Endocrinology and Immunology Department. A prick test was carried out for all commercially available insulin presentations and another insulin scheme was designed but proved unsuccessful. A desensitization protocol was started with Glargine alongside administration of Prednisone, which successfully induced tolerance. Observation of skin lesions typical of vitiligo prompted laboratory workup for other autoimmune disorders, which returned positive for autoimmune gastritis/pernicious anemia. These findings are compatible with APS type 4. Discussion. To our knowledge, this is the first documented case of insulin allergy in type 4 APS, as well as this particular combination in APS. Etiopathogenic components shared by insulin allergy and APS beg for further research in immunogenetics to further comprehend pathophysiologic aspects of these diseases.

Villalobos, Marjorie; Martínez, María Sofía; Chávez-Castillo, Mervin; Mejías, José Carlos; Miquilena, Edgar; Bermúdez, Valmore

2014-01-01

309

The TRACTISS Protocol: a randomised double blind placebo controlled clinical TRial of Anti-B-Cell Therapy In patients with primary Sjögren’s Syndrome  

PubMed Central

Background Primary Sjögren’s Syndrome (PSS) mainly affects women (9:1 female:male ratio) and is one of the commonest autoimmune diseases with a prevalence of 0.1 – 0.6% of adult women. For patients with PSS there is currently no effective therapy that can alter the progression of the disease. The aim of the TRACTISS study is to establish whether in patients with PSS, treatment with rituximab improves clinical outcomes. Methods/design TRACTISS is a UK multi-centre, double-blind, randomised, controlled, parallel group trial of 110 patients with PSS. Patients will be randomised on a 1:1 basis to receive two courses of either rituximab or placebo infusion in addition to standard therapy, and will be followed up for up to 48 weeks. The primary objective is to assess the extent to which rituximab improves symptoms of fatigue and oral dryness. Secondary outcomes include ocular dryness, salivary flow rates, lacrimal flow, patient quality of life, measures of disease damage and disease activity, serological and peripheral blood biomarkers, and glandular histology and composition. Discussion The TRACTISS trial will provide direct evidence as to whether rituximab in patients with PSS leads to an improvement in patient symptoms and a reduction in disease damage and activity. Trial registration UKCRN Portfolio ID: 9809 ISRCTN65360827. PMID:24438039

2014-01-01

310

[Lemierre's syndrome].  

PubMed

Lemierre's syndrome is a rare disease, mostly affecting young adults in good health, which can lead to significant morbidity and mortality. We report one case with favourable outcome and clinical features stereotypics: angina, septic thrombosis of the internal jugular vein, pulmonary septic metastasis, Fusobacterium necrophorum on blood cultures. Antibiotic therapy targeting anaerobes has been rapidly initiated and maintained 4-6 weeks. The anticoagulation has been maintained 4 weeks. PMID:21035996

Courtin, P; Toro, A; Gazagnes, M; Berrouba, A; Gallardo, M; Dembele, A

2010-11-01

311

Gut-directed hypnotherapy in children with irritable bowel syndrome or functional abdominal pain (syndrome): a randomized controlled trial on self exercises at home using CD versus individual therapy by qualified therapists  

PubMed Central

Background Irritable bowel syndrome (IBS) and functional abdominal pain (syndrome) (FAP(S)) are common pediatric disorders, characterized by chronic or recurrent abdominal pain. Treatment is challenging, especially in children with persisting symptoms. Gut-directed hypnotherapy (HT) performed by a therapist has been shown to be effective in these children, but is still unavailable to many children due to costs, a lack of qualified child-hypnotherapists and because it requires a significant investment of time by child and parent(s). Home-based hypnotherapy by means of exercises on CD has been shown effective as well, and has potential benefits, such as lower costs and less time investment. The aim of this randomized controlled trial (RCT) is to compare cost-effectiveness of individual HT performed by a qualified therapist with HT by means of CD recorded self-exercises at home in children with IBS or FAP(S). Methods/Design 260 children, aged 8-18 years with IBS or FAP(S) according to Rome III criteria are included in this currently conducted RCT with a follow-up period of one year. Children are randomized to either 6 sessions of individual HT given by a qualified therapist over a 3-month period or HT through self-exercises at home with CD for 3 months. The primary outcome is the proportion of patients in which treatment is successful at the end of treatment and after one year follow-up. Treatment success is defined as at least 50% reduction in both abdominal pain frequency and intensity scores. Secondary outcomes include adequate relief, cost-effectiveness and effects of both therapies on depression and anxiety scores, somatization scores, QoL, pain beliefs and coping strategies. Discussion If the effectiveness of home-based HT with CD is comparable to, or only slightly lower, than HT by a therapist, this treatment may become an attractive form of therapy in children with IBS or FAP(S), because of its low costs and direct availability. Trial registration Dutch Trial Register number NTR2725 (date of registration: 1 February 2011) PMID:24894077

2014-01-01

312

[Clinical signs, diagnostic approach and therapy for the so-called ovarian remnant syndrome in the bitch].  

PubMed

The ovarian remnant syndrome arises as a consequence of incomplete ovariectomy or ovariohysterectomy. Remnant ovarian tissue which has been left mostly unintentionally in the bitch may show endocrine activity a few weeks to several years after surgery, provoking a variety of clinical signs. The majority of affected bitches return to heat, in other cases signs of pseudopregnancy and endometritis may be observed. Occasionally, bitches with unclear clinical signs are presented with the suspicion of an inactive ovarian remnant. The following article intends to place the origin of the ovarian remnant syndrome into a factual context regarding the responsibility of the veterinarian and to demonstrate a reasonable diagnostic procedure according to the respective clinical signs. In this regard, the clinical-gynaecological examination, including vaginal cytology, must receive high priority, with the addition of progesterone analysis in peripheral blood plasma or serum if required. Using these combined diagnostic tools, ovarian remnants in stages of endocrine activity (follicular and luteal phases as well as cystic or tumourous ovarian tissue) can be easily unequivocally diagnosed. The application of a GnRH-stimulation test is only reasonable in bitches in which clinical signs are missing. In this context, the usefulness of semi-quantitative LH-assays is also discussed. PMID:22331327

Günzel-Apel, A-R; Buschhaus, J; Urhausen, C; Masal, C; Wolf, K; Meyer-Lindenberg, A; Piechotta, M; Beyerbach, M; Schoon, H-A

2012-01-01

313

Syndromic Disorders with Short Stature  

PubMed Central

Short stature is one of the major components of many dysmorphic syndromes. Growth failure may be due to a wide variety of mechanisms, either related to the growth hormone (GH)/insulin-like growth factor axis or to underlying unknown pathologies. In this review, the relatively more frequently seen syndromes with short stature (Noonan syndrome, Prader-Willi syndrome, Silver-Russell syndrome and Aarskog-Scott syndrome) were discussed. These disorders are associated with a number of endocrinopathies, as well as with developmental, systemic and behavioral issues. At present, GH therapy is used in most syndromic disorders, although long-term studies evaluating this treatment are insufficient and some controversies exist with regard to GH dose, optimal age to begin therapy and adverse effects. Before starting GH treatment, patients with syndromic disorders should be evaluated extensively. PMID:24637303

??klar, Zeynep; Berbero?lu, Merih

2014-01-01

314

Long-term testosterone therapy in hypogonadal men ameliorates elements of the metabolic syndrome: an observational, long-term registry study  

PubMed Central

Aim The goal of this study was to determine if long-term testosterone (T) therapy in men with hypogonadism, henceforth referred to as testosterone deficiency (TD), ameliorates or improves metabolic syndrome (MetS) components. Methods We performed a cumulative registry study of 255 men, aged between 33 and 69 years (mean 58.02 ± 6.30) with subnormal plasma total T levels (mean: 9.93 ± 1.38; range: 5.89–12.13 nmol/l) as well as at least mild symptoms of TD assessed by the Aging Males' symptoms scale. All men received treatment with parenteral T undecanoate 1000 mg (Nebido®, Bayer Pharma, Berlin, Germany), administered at baseline and 6 weeks and thereafter every 12 weeks for up to 60 months. Lipids, glucose, liver enzymes and haemoglobin A1c analyses were carried out in a commercial laboratory. Anthropometric measurements were also made throughout the study period. Results Testosterone therapy restored physiological T levels and resulted in reductions in total cholesterol (TC) [7.29 ± 1.03 to 4.87 ± 0.29 mmol/l (281.58 ± 39.8 to 188.12 ± 11.31 mg/dl)], low-density lipoprotein cholesterol [4.24 ± 1.07 to 2.84 ± 0.92 mmol/l (163.79 ± 41.44 to 109.84 ± 35.41 mg/dl)], triglycerides [3.14 ± 0.58 to 2.16 ± 0.13 mmol/l (276.16 ± 51.32 to 189.78 ± 11.33 mg/dl)] and increased high-density lipoprotein levels [1.45 ± 0.46 to 1.52 ± 0.45 mmol/l (56.17 ± 17.79 to 58.85 ± 17.51 mg/dl)] (p < 0.0001 for all). There were marked reductions in systolic and diastolic blood pressure, blood glucose, haemoglobin A1c, C-reactive protein (6.29 ± 7.96 to 1.03 ± 1.87 U/l), alanine aminotransferase and aspartate aminotransferase (p < 0.0001 for all). Conclusions Long-term T therapy, at physiological levels, ameliorates MetS components. These findings strongly suggest that T therapy in hypogonadal men may prove useful in reducing the risk of cardiometabolic diseases. PMID:24127736

Traish, A M; Haider, A; Doros, G; Saad, F

2014-01-01

315

Improvement of cardiac function by short-term enzyme replacement therapy in a murine model of cardiomyopathy associated with Hunter syndrome evaluated by serial echocardiography with speckle tracking 2-D strain analysis.  

PubMed

Cardiac systolic function is significantly decreased in a proportion of patients with Hunter syndrome. This study was performed to evaluate the change in myocardial function associated with enzyme replacement therapy (ERT) in a mouse model of cardiomyopathy associated with Hunter syndrome. Thirty 9-week-old iduronate-2-sulfatase (IDS) knockout mice received either intravenous injection of human recombinant IDS (ERT group, N=15) or saline (control group, N=15) for 5 weeks. Echocardiography was performed at baseline and after treatment. Echocardiographic parameters of left ventricular (LV) systolic function and 2-dimensional radial and circumferential strain were assessed. At follow-up, there was a significant increase in LV fractional shortening and radial and circumferential strain in the ERT group only. Notable myocardial fibrosis was observed in the control group only. In the murine model of Hunter syndrome, ERT exerts beneficial effects on cardiac function, which can be evaluated by serial echocardiographic evaluation including 2-dimensional strain analysis. PMID:24836711

Lee, Sang-Chol; Lee, Jieun; Jin, Dong-Kyu; Kim, Jung-Sun; Jeon, Eun-Seok; Kwun, Young Hee; Chang, Mi Sun; Ko, Ah-ra; Yook, Yeon Joo; Sohn, Young Bae

2014-07-01

316

Anticomplement C5 therapy with eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome.  

PubMed

The complement inhibitor eculizumab is a humanized monoclonal antibody against C5. It was developed to specifically target cleavage of C5 thus preventing release of C5a and activation of the terminal pathway. Paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS) are 2 diseases with distinctly different underlying molecular mechanisms. In PNH, progeny of hematopoietic stem cells that harbor somatic mutations lead to a population of peripheral blood cells that are deficient in complement regulators resulting in hemolysis and thrombosis. In aHUS, germline mutations in complement proteins or their regulators fail to protect the glomerular endothelium from complement activation resulting in thrombotic microangiopathy and renal failure. Critical to the development of either disease is activation of the terminal complement pathway. Understanding this step has led to the study of eculizumab as a treatment for these diseases. In clinical trials, eculizumab is proven to be effective and safe in PNH and aHUS. PMID:25468487

Wong, Edwin K S; Kavanagh, David

2014-10-20

317

Revisiting plummer vinson syndrome.  

PubMed

Plummer Vinson syndrome is a rare association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. Iron deficiency state has been hypothesized to play an etiological role. While literature review elucidates the resolution of dysphagia in most cases with iron therapy, we discuss our case where the dysphagia was resistant to such therapy and necessitated a mechanical dilatation. PMID:23634346

Gude, D; Bansal, Dp; Malu, A

2013-01-01

318

Revisiting Plummer Vinson Syndrome  

PubMed Central

Plummer Vinson syndrome is a rare association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia. Iron deficiency state has been hypothesized to play an etiological role. While literature review elucidates the resolution of dysphagia in most cases with iron therapy, we discuss our case where the dysphagia was resistant to such therapy and necessitated a mechanical dilatation. PMID:23634346

Gude, D; Bansal, DP; Malu, A

2013-01-01

319

Systemic lupus erythematosus and Klinefelter's syndrome  

Microsoft Academic Search

A case of Klinefelter's syndrome presenting with systemic lupus erythematosus while receiving androgen replacement therapy is described. The association of systemic lupus erythematosus with Klinefelter's syndrome is discussed, particularly in terms of the effect of sex hormones.

M A French; P Hughes

1983-01-01

320

Randomized Multicenter Clinical Trial of Myofascial Physical Therapy in Women with Interstitial Cystitis/Painful Bladder Syndrome (IC/PBS) and Pelvic Floor Tenderness  

PubMed Central

Objectives To determine the efficacy and safety of pelvic floor Myofascial Physical Therapy (MPT) in women with newly-symptomatic IC/PBS, as compared to Global Therapeutic Massage (GTM). Materials and Methods A randomized controlled trial of 10 scheduled treatments of MPT vs. GTM was performed at 11 clinical centers located in North America. We recruited women with IC/PBS with demonstrable pelvic floor tenderness on physical examination and a limitation of no more than 3 years symptom duration. The primary outcome was the proportion of responders defined as ‘moderately improved’ or ‘markedly improved’ in overall symptoms compared to baseline on a 7-point scale Global Response Assessment (GRA). Secondary outcomes included ratings for pain, urgency, frequency; the O'Leary-Sant IC Symptom and Problem Index (ICSI/ICPI) and reports of adverse events. We compared response rates between treatment arms using the exact conditional version of the Mantel-Haenszel test to control for clustering by clinical center. For secondary efficacy outcomes, cross-sectional descriptive statistics and changes from baseline were calculated. Results Eighty-one women randomized to the two treatment groups had similar symptoms at baseline. The GRA response rate was 26% in the GTM group and 59% in the MPT group (p=0.0012). Pain, urgency, and frequency ratings and in ICSI/ICPI decreased in both groups during follow-up and were not significantly different between the groups. Pain was the most common adverse event, occurring at similar rates in both groups. There were no serious adverse events reported. Conclusions A significantly higher proportion of women with IC/PBS reponded to treatment with MPT than with GTM. MPT may be a beneficial therapy in women with this syndrome. PMID:22503015

FitzGerald, MP; Payne, CK; Lukacz, ES; Yang, CC; Peters, KM; Chai, TC; Nickel, JC; Hanno, PM; Kreder, KJ; Burks, DA; Mayer, R; Kotarinos, R; Fortman, C; Allen, TM; Fraser, L; Mason-Cover, M; Furey, C; Odabachian, L; Sanfield, A; Chu, J; Huestis, K; Tata, GE; Dugan, N; Sheth, H; Bewyer, K; Anaeme, A; Newton, K; Featherstone, W; Halle-Podell, R; Cen, L; Landis, JR; Propert, KJ; Foster, HE; Kusek, JW; Nyberg, *LM

2012-01-01

321

Efficacy and safety of meditative movement therapies in fibromyalgia syndrome: a systematic review and meta-analysis of randomized controlled trials.  

PubMed

A systematic review with meta-analysis of the efficacy and safety of meditative movement therapies (Qigong, Tai Chi and Yoga) in fibromyalgia syndrome (FMS) was carried out. We screened Clinicaltrials.Gov, Cochrane Library, PsycINFO, PubMed and Scopus (through December 2010) and the reference sections of original studies for meditative movement therapies (MMT) in FMS. Randomized controlled trials (RCT) comparing MMT to controls were analysed. Outcomes of efficacy were pain, sleep, fatigue, depression and health-related quality of life (HRQOL). Effects were summarized using standardized mean differences (SMD [95% confidence interval]). Outcomes of safety were drop out because of adverse events and serious adverse events. A total of 7 out of 117 studies with 362 subjects and a median of 12 sessions (range 8-24) were included. MMT reduced sleep disturbances (-0.61 [-0.95, -0.27]; 0.0004), fatigue (-0.66 [-0.99, -0.34]; <0.0001), depression (-0.49 [-0.76, -0.22]; 0.0004) and limitations of HRQOL (-0.59 [-0.93, -0.24]; 0.0009), but not pain (-0.35 [-0.80, 0.11]; 0.14) compared to controls at final treatment. The significant effects on sleep disturbances (-0.52 [-0.97, -0.07]; 0.02) and HRQOL (-0.66 [-1.31, -0.01]; 0.05) could be maintained after a median of 4.5 (range 3-6) months. In subgroup analyses, only Yoga yielded significant effects on pain, fatigue, depression and HRQOL at final treatment. Drop out rate because of adverse events was 3.1%. No serious adverse events were reported. MMT are safe. Yoga had short-term beneficial effects on some key domains of FMS. There is a need for high-quality studies with larger sample sizes to confirm the results. PMID:22350253

Langhorst, Jost; Klose, Petra; Dobos, Gustav J; Bernardy, Kathrin; Häuser, Winfried

2013-01-01

322

Exploring Patients' Views of a Cognitive Behavioral Therapy-Based Website for the Self-Management of Irritable Bowel Syndrome Symptoms  

PubMed Central

Background Cognitive behavioral therapy (CBT) has been shown to have positive effects on the management of irritable bowel syndrome (IBS) symptoms. A factorial pilot randomized placebo-controlled trial (called MIBS) tested the potential effectiveness of a self-management CBT-based website alongside two medications: methylcellulose and mebeverine, and a placebo. The results showed no significant differences in quality of life or symptom severity measures, but enablement and participant’s global assessment of relief was higher in the website groups. Objective To conduct a qualitative study nested within this trial, in order to explore patients’ views and experiences of using the CBT-based website to facilitate self-management of IBS. Methods Semistructured interviews were carried out with patients who had used the website with one session of nurse support (n=16) or the website alone (n=15) while participating in the MIBS trial. An inductive thematic analysis was conducted. Results We identified three types of engagement with the CBT-based website. One group of participants, mostly in the website-only condition, had limited or no engagement with the website. One group engaged with the content and advice on practical lifestyle changes. The final group of participants engaged with the content and advice on psychological aspects related to IBS. Similarities and differences between these three groups are explored. Conclusions Teaching self-management techniques through a Web intervention was received positively by most of the participants. Concepts linked to cognitive aspects of CBT appeared to be harder for participants to engage with. Participants who received nurse support rated the cognitive aspects more positively, suggesting that some therapy support alongside the website should be considered. However, the Web format was preferred by some who favored anonymity as well as those who appreciated the accessibility and ease of use of this type of management. Suggestions on how to encourage engagement with Web interventions are discussed. PMID:24001787

Bishop, Felicity L; Ellis, Matthew; Moss-Morris, Rona; Everitt, Hazel

2013-01-01

323

Hypertension due to antiangiogenic cancer therapy with vascular endothelial growth factor inhibitors: understanding and managing a new syndrome.  

PubMed

Novel antiangiogenic cancer therapies, particularly agents that block vascular endothelial growth factor (VEGF) signalling, have improved outcomes in patients with cancers and are now used as first-line therapies for some tumours. However, with VEGF inhibitors (VEGFIs) are new complications, particularly hypertension. VEGFI-induced hypertension is a dose-dependent phenomenon due to on-target effects rather than off-target effects. Increased blood pressure occurs in almost 100% of patients who take VEGFIs, with a subset who develop severe hypertension. Molecular mechanisms underlying VEGFI-induced hypertension are unclear, but endothelial dysfunction and increased vascular resistance, due to impaired nitric oxide signalling, reduced prostacyclin production, endothelin-1 (ET-1) upregulation, oxidative stress, and rarefaction have been implicated. Treatment of hypertension should be aimed at reducing the risk of short-term morbidity associated with hypertension while maintaining effective dosing of antiangiogenic therapy for optimal cancer treatment. Although specific guidelines are not yet available for the management of VEGFI-induced hypertension, angiotensin-converting enzyme inhibitors and dihydropyridine calcium channel blockers are commonly used. Severe hypertension might require reduction of VEGFI dosing, or in some cases, interruption of treatment. As more potent VEGFIs are developed and as more cancer patients are treated with VEGFIs, the burden of hypertension toxicity will increase. This will be further compounded as the use of antiangiogenic drugs broadens to include older patients and those with pre-existing cardiovascular disease. Here we focus on VEGF as a target for antiangiogenesis and how this affects increased blood pressure. Putative mechanisms underlying VEGFI-induced hypertension are highlighted and therapeutic strategies to manage such hypertension are discussed. PMID:24786444

Small, Heather Yvonne; Montezano, Augusto C; Rios, Francisco J; Savoia, Carmine; Touyz, Rhian M

2014-05-01

324

Therapy-related acute myeloid leukemia and myelodysplastic syndromes in patients with Hodgkin lymphoma: a report from the German Hodgkin Study Group.  

PubMed

Therapy-related acute myeloid leukemia and myelodysplastic syndromes (t-AML/MDS) represent severe late effects in patients treated for Hodgkin lymphoma (HL). Because more recent data are scarce, we retrospectively analyzed incidence, outcome, and risk factors for the development of t-AML/MDS after HL. A total of 11,952 patients treated for newly diagnosed HL within German Hodgkin Study Group trials between 1993 and 2009 were considered. At a median follow-up of 72 months, t-AML/MDS was diagnosed in 106/11,952 patients (0.9%). Median time from HL treatment to t-AML/MDS was 31 months. The median age of patients with t-AML/MDS was higher than in the whole patient group (43 vs 34 years, P < .0001). Patients who received 4 or more cycles of BEACOPP(escalated) had an increased risk to develop t-AML/MDS when compared with patients treated with less than 4 cycles of BEACOPP(escalated) or no BEACOPP chemotherapy (1.7% vs 0.7% vs 0.3%, P < .0001). The median overall survival (OS) for all t-AML/MDS patients was 7.2 months. However, t-AML/MDS patients proceeding to allogeneic stem cell transplantation had a significantly better outcome with a median OS not reached after a median follow-up of 41 months (P < .001). PMID:24478403

Eichenauer, Dennis A; Thielen, Indra; Haverkamp, Heinz; Franklin, Jeremy; Behringer, Karolin; Halbsguth, Teresa; Klimm, Beate; Diehl, Volker; Sasse, Stephanie; Rothe, Achim; Fuchs, Michael; Böll, Boris; von Tresckow, Bastian; Borchmann, Peter; Engert, Andreas

2014-03-13

325

Analysis of the cost-effectiveness of surfactant treatment (Curosurf®) in respiratory distress syndrome therapy in preterm infants: early treatment compared to late treatment  

PubMed Central

Background The best criteria for surfactant treatment in the perinatal period are unknown and this makes it of interest to consider the possible economic implications of lessening the use of more restrictive criteria. Objective The objective of this study is the evaluation of the costs of respiratory care for preterm infants with Respiratory Distress Syndrome (RDS) treated with "early rescue" surfactant compared to a "late rescue" strategy. Methods The study was carried out applying the costs of materials used, of staff and pharmacological therapy calculated in the Neonatal Intensive Care Unit (NICU) of an Italian hospital to the Verder et al. study (Pediatrics 1999) clinical data. Results The cost for patients treated with early strategy was slightly lower than for patients treated with late strategy (Euro 4,901.70 vs. Euro 4,960.07). The cost of treatment with surfactant was greater in the early group (Euro 458.49 vs. Euro 311.74), but this was compensated by the greater cost of treatment with Mechanical Ventilation (MV) in the late group (respectively Euro 108.85 vs. Euro 259.25). Conclusions The cost-effectiveness analysis performed in this study shows how early treatment with surfactant in preterm infants with RDS, as well as being clinically more effective, is associated with a slightly lower cost. PMID:24886906

2014-01-01

326

Acupuncture for climacteric syndrome  

Microsoft Academic Search

This paper reviews and analyzes 50 articles concerning the treatment of climacteric syndrome by acupuncture and moxibustion\\u000a therapries. The therapies were single acupuncture, acupuncture combining other methods, auricular-plaster therapy, and some\\u000a other therapies. Clear diagnostic criteria were found in 39 articles, and the most common-used criteria wereCombination of TCM and Western Medicine Diagnostics and Therapeutics, Kupperman Index Scoring andCriterion of

Cui Xue-jun; Huang Qin-feng

2004-01-01

327

Short course daily prednisolone therapy during an upper respiratory tract infection in children with relapsing steroid-sensitive nephrotic syndrome (PREDNOS 2): protocol for a randomised controlled trial  

PubMed Central

Background Relapses of childhood steroid-sensitive nephrotic syndrome (SSNS) are treated with a 4- to 8-week course of high-dose oral prednisolone, which may be associated with significant adverse effects. There is a clear association between upper respiratory tract infection (URTI) and relapse development. Previous studies in developing nations have suggested that introducing a 5- to 7-day course of daily prednisolone during an URTI may prevent a relapse developing and the need for a treatment course of high-dose prednisolone. The aim of PREDNOS 2 is to evaluate the effectiveness of a 6-day course of daily prednisolone therapy during an URTI in reducing the development of a subsequent relapse in a developed nation. Methods/design The subjects will be 300 children with relapsing SSNS (?2 relapses in preceding year), who will be randomised to receive either a 6-day course of daily prednisolone or no change to their current therapy (with the use of placebo to double blind) each time they develop an URTI over 12 months. A strict definition for URTI will be used. Subjects will be reviewed at 3, 6, 9 and 12 months to capture data regarding relapse history, ongoing therapy and adverse effect profile, including behavioural problems and quality of life. A formal health economic analysis will also be performed. The primary end point of the study will be the incidence of URTI-related relapse (3 days of Albustix +++) following the first infection during the 12-month follow-up period. DNA and RNA samples will be collected to identify a potential genetic cause for the disease. Subjects will be recruited from over 100 UK centres with the assistance of the Medicines for Children Research Network. PREDNOS 2 is funded by the National Institute for Health Research Health Technology Assessment Programme (11/129/261). Discussion We propose that PREDNOS 2 will be a pivotal study that will inform the future standard of care for children with SSNS. If it is possible to reduce the disease relapse rate effectively and safely, this will reduce the morbidity and cost associated with drug treatment, notwithstanding hospital admission and parental absence from employment. Trial registration Current Controlled Trials (ISRCTN10900733). PMID:24767719

2014-01-01

328

Fibrillary glomerulonephritis with small fibrils in a patient with the antiphospholipid antibody syndrome successfully treated with immunosuppressive therapy  

PubMed Central

Background Fibrillary glomerulonephritis is a rare cause of progressive renal dysfunction, often leading to the need for dialysis within a few years. The role of immunosuppressive treatment is still uncertain although this has been tried with variable success. Case presentation A 56 year old woman with the antiphospholipid antibody syndrome (IgM anticardiolipin antibodies) was seen in the nephrology clinic with haematuria, proteinuria, and worsening renal function. A renal biopsy demonstrated a mesangial proliferative glomerulonephritis on light microscopy and smaller fibrils (10.6–13.8 nm in diameter) than is usual for fibrillary glomerulonephritis (typically 18–22 nm) on electron microscopy. Amyloidosis was excluded following detailed evaluation. On account of rapidly worsening renal failure she was started on cyclophosphamide and prednisolone which led to the partial recovery and stabilization of her renal function. Conclusion This case highlights the need for routine electron microscopy in native renal biopsies, where the differential diagnosis is wide and varied and the light and immunofluorescence microscopic findings may be non specific. PMID:17490479

Javaid, Muhammad M; Denley, Helen; Tagboto, Senyo

2007-01-01

329

Evolving Insights in the Pathogenesis and Therapy of Cutaneous T-cell lymphoma (Mycosis Fungoides and Sezary Syndrome)  

PubMed Central

Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of malignancies derived from skin-homing T cells. The most common forms of CTCL are Mycosis Fungoides (MF) and Sezary Syndrome (SS). Accurate diagnosis remains a challenge due to the heterogeneity of presentation and the lack of highly characteristic immunophenotypical and genetic markers. Over the past decade molecular studies have improved our understanding of the biology of CTCL. The identification of gene expression differences between normal and malignant T-cells has led to promising new diagnostic and prognostic biomarkers that now need validation to be incorporated into clinical practice. These biomarkers may also provide insight into the mechanism of development of CTCL. Additionally, treatment options have expanded with the approval of new agents, such as histone deacetylase inhibitors. A better understanding of the cell biology, immunology and genetics underlying the development and progression of CTCL will allow the design of more rational treatment strategies for these malignancies. This review summarizes the clinical epidemiology, staging and natural history of MF and SS; discusses the immunopathogenesis of MF and the functional role of the malignant T-cells; and reviews the latest advances in MF and SS treatment. PMID:21883142

Wong, Henry K.; Mishra, Anjali; Hake, Timothy; Porcu, Pierluigi

2015-01-01

330

[The effects of physical therapy in prevention of deep vein thrombosis (DVT) in the "syndrome of economy class"].  

PubMed

"Economy class syndrome" made headline news in Australia when young girl died from blood clot caused by deep vein thrombosis shortly after getting off a Sydney to London flight. Although medical research is yet to prove a link between long distance travelling and DVT, consumers sholud take precautions. Pain and swelling in one leg is usually the first sign of a DTV, although sometimes there won, t be any symptoms. In one to two percent of cases the blood clot can break away from the vein and travel to other major organs. If it s big enough it may cause breathing problems (pulmonary embolism), and in rare cases--around one in a thousand people with a DVT-death. During the flight: Change your position regularly and be aware of positions which might block your circulation. Regular excercise will help restore blood flow to your legs if you've been sitting for long period of time or in an awkward position. Dehydratation can cause the blood to thiceken. Alcohol and caffeine are known diuretics which may accentuate the effect of dry cabin air and immobility on blood flow. Drink 200 mls (a standards glass) of non-alcoholic fluids every hour and use the need to go to the toilet as an opportunity to change your posture. PMID:16528931

Vavra-Hadziahmetovi?, Naricsa; Hadziahmetovi?, Zoran

2006-01-01

331

[Thrombolytic therapy].  

PubMed

Thrombolytic therapy in patients with acute coronary syndrome is not so widely used compared to coronary intervention, such as PTCA or stenting in Japan. This is probably because of the facts that the narrowing of the coronary artery is not completely restored after the thrombolytic therapy and that the coronary intervention is frequently necessary inspite of the administration of the thrombolytic agent. However, the thrombolytic therapy can be easily done in the peracute stage of myocardial infarction, and improvement of the prognosis of the affected patient may be possible. In this paper, the concepts as well as the effect of thrombolytic therapy and the characteristics of newly developed thrombolytic drugs are discussed. PMID:9796322

Matsuda, T

1998-10-01

332

[Menopause and metabolic syndrome].  

PubMed

The incidence of cardiovascular disease increases considerably after the menopause. One reason for the increased cardiovascular risk seems to be determined by metabolic syndrome, in which all components (visceral obesity, dyslipidemia, hypertension, and glucose metabolism disorder) are associated with higher incidence of coronary artery disease. After menopause, metabolic syndrome is more prevalent than in premenopausal women, and may plays an important role in the occurrence of myocardial infarction and other atherosclerotic and cardiovascular morbidities. Obesity, an essential component of the metabolic syndrome, is also associated with increased incidence of breast, endometrial, bowel, esophagus, and kidney cancer. The treatment of metabolic syndrome is based on the change in lifestyle and, when necessary, the use of medication directed to its components. In the presence of symptoms of the climacteric syndrome, hormonal therapy, when indicated, will also contribute to the improvement of the metabolic syndrome. PMID:24830585

Meirelles, Ricardo M R

2014-03-01

333

Health economic analysis of ticagrelor in patients with acute coronary syndromes intended for non-invasive therapy  

PubMed Central

Objective To investigate the cost effectiveness of ticagrelor versus clopidogrel in patients with acute coronary syndromes (ACS) in the Platelet Inhibition and Patient Outcomes (PLATO) study who were scheduled for non-invasive management. Methods A previously developed cost effectiveness model was used to estimate long-term costs and outcomes for patients scheduled for non-invasive management. Healthcare costs, event rates and health-related quality of life under treatment with either ticagrelor or clopidogrel over 12?months were estimated from the PLATO study. Long-term costs and health outcomes were estimated based on data from PLATO and published literature sources. To investigate the importance of different healthcare cost structures and life expectancy for the results, the analysis was carried out from the perspectives of the Swedish, UK, German and Brazilian public healthcare systems. Results Ticagrelor was associated with lifetime quality-adjusted life-year (QALY) gains of 0.17 in Sweden, 0.16 in the UK, 0.17 in Germany and 0.13 in Brazil compared with generic clopidogrel, with increased healthcare costs of €467, €551, €739 and €574, respectively. The cost per QALY gained with ticagrelor was €2747, €3395, €4419 and €4471 from a Swedish, UK, German and Brazilian public healthcare system perspective, respectively. Probabilistic sensitivity analyses indicated that the cost per QALY gained with ticagrelor was below conventional threshold values of cost effectiveness with a high probability. Conclusions Treatment of patients with ACS scheduled for 12?months’ non-invasive management with ticagrelor is associated with a cost per QALY gained below conventional threshold values of cost effectiveness compared with generic clopidogrel. Trial registration number NCT000391872. PMID:25227704

Janzon, M; James, S; Cannon, C P; Storey, R F; Mellström, C; Nicolau, J C; Wallentin, L; Henriksson, M

2015-01-01

334

Alport's syndrome.  

PubMed Central

Alport's syndrome (AS) is a progressive glomerulonephritis which is associated with high tone sensorineural deafness and characteristic eye signs. It accounts for 0.6% of all patients who start renal replacement therapy in Europe, and is most commonly inherited as an X linked disorder with a gene frequency of 1 in 5000. During the last six years several type IV collagen genes have been implicated in the aetiology of AS, and mutation detection studies are enabling genotype/phenotype correlations to be made, as well as facilitating carrier detection and prenatal diagnosis. Images PMID:9138159

Flinter, F

1997-01-01

335

Griscelli syndrome.  

PubMed

We report a case of Griscelli Syndrome (GS). Our patient initially presented with a diagnosis of haemophagocytic lymphistiocytosis (HLH). Subsequent microscopic analysis of the patient's hair follicle revealed abnormal distribution of melanosomes in the shaft, which is a hallmark for GS. Analysis of RAB27A gene in this patient revealed a homozygous mutation in exon 6, c.550C>T, p.R184X . This nonsense mutation causes premature truncation of the protein resulting in a dysfunctional RAB27A. Recognition of GS allows appropriate institution of therapy namely chemotherapy for HLH and curative haemotopoeitic stem cell transplantation. PMID:25500851

Ariffin, H; Geikowski, A; Chin, T F; Chau, D; Arshad, A; Abu Bakar, K; Krishnan, S

2014-08-01

336

An open-label pilot study of pulsed electromagnetic field therapy in the treatment of failed back surgery syndrome pain  

PubMed Central

Persistent pain following back surgery remains a major treatment challenge. The primary objective of this open-label exploratory study was to investigate the analgesic effectiveness of pulsed electromagnetic field therapy administered twice daily over a 45-day period in 34 subjects (68% female) with persistent or recurrent pain following back surgery. A secondary goal was to guide the design of future randomized controlled trials that could target responsive subpopulations. All predefined primary and secondary outcomes, including change in pain intensity (PI), physical function (Oswestry Disability Index), analgesic consumption, and overall well-being (Patient Global Impression of Change), are reported. A responder analysis (?30% reduction in PI versus baseline) was added as a post hoc evaluation. Safety outcomes, as well as results of a cost-avoidance survey, are also summarized. Of the 30 per-protocol subjects who completed the study, 33% reported a clinically meaningful (?30%) reduction in PI. A higher response rate (60%) was reported for subjects who had undergone discectomy prior to the trial compared to subjects who had undergone other types of surgical interventions (decompression or fusion) without discectomy. Improvements in PI were paralleled by improvements in secondary outcomes. Relative to baseline, responders reported an average 44% and 55% reduction in back PI and leg PI (respectively), and an average 13% improvement in Oswestry Disability Index scores. In the per-protocol population, 50% of responders and 12% of nonresponders reported less analgesia consumption at the end of treatment versus baseline. Sixty-seven percent of per-protocol responders and 0% of nonresponders reported clinically meaningful improvement in overall well-being on the Patient Global Impression of Change scale.

Harper, Wayne L; Schmidt, William K; Kubat, Nicole J; Isenberg, Richard A

2015-01-01

337

No influence of parental origin of intact X chromosome and/or Y chromosome sequences on three-year height response to growth hormone therapy in Turner syndrome  

PubMed Central

Purpose Whether parental origin of the intact X chromosome and/or the presence of Y chromosome sequences (Yseq) play a role in three-year height response to growth hormone (GH) were investigated. Methods Paternal (Xp) or maternal (Xm) origin of X chromosome was assessed by microsatellite marker analysis and the presence of hidden Yseq was analyzed. The first-, second-, and third-year GH response was measured as a change in height z-score (Z_Ht) in Turner syndrome (TS) patients with 45,Xp (n=10), 45,Xm (n=15), and 45,X/46,X,+mar(Y) (Xm_Yseq) (n=8). Results The mean baseline Z_Ht did not differ according to Xp or Xm origin, however the mean baseline Z_Ht was higher in the Xm_Yseq group than in Xm group, after adjusting for bone age delay and midparental Z_Ht (P=0.04). There was no difference in the height response to GH between the 3 groups. The height response to GH decreased progressively each year (P<0.001), such that the third-year increase in Z_Ht was not significant. This third-year decrease in treatment response was unaffected by Xp, Xm, and Xm_Yseq groups. Increasing GH dosage from the second to third-year of treatment positively correlated with the increase in Z_Ht (P=0.017). Conclusion There was no evidence of X-linked imprinted genes and/or Yseq affecting height response to 3 years of GH therapy. Increasing GH dosages may help attenuate the decrease in third-year GH response in TS patients with 45,X and/or 46,X/+mar(Y). PMID:25346916

Lee, Hye Jin; Jung, Hae Woon; Lee, Gyung Min; Kim, Hwa Young; Kim, Jae Hyun; Lee, Sun Hee; Kim, Ji Hyun; Shin, Choong Ho; Yang, Sei Won

2014-01-01

338

Characterization of placental cholesterol transport: ABCA1 is a potential target for in utero therapy of Smith–Lemli–Opitz syndrome  

PubMed Central

Patients with Smith–Lemli–Opitz syndrome (SLOS) are born with multiple congenital abnormalities. Postnatal cholesterol supplementation is provided; however, it cannot correct developmental malformations due to in utero cholesterol deficit. Increased transport of cholesterol from maternal to fetal circulation might attenuate congenital malformations. The cholesterol transporters Abca1, Abcg1, and Sr-b1 are present in placenta; however, their potential role in placental transport remains undetermined. In mice, expression analyses showed that Abca1 and Abcg1 transcripts increased 2–3-fold between embryonic days 13.5 and 18.5 in placental tissue; whereas, Sr-b1 expression decreased. To examine the functional role of Abca1, Abcg1 and Sr-b1 we measured the maternal–fetal transfer of 14C-cholesterol in corresponding mutant embryos. Disruption of either Abca1 or Sr-b1 decreased cholesterol transfer by ?30%. In contrast, disruption of the Abcg1 had no effect. Treatment of pregnant C57Bl/6 female mice with TO901317, an LXR-agonist, increased both Abca1 expression and maternal–fetal cholesterol transfer to the fetus. In an SLOS mouse model (Dhcr7?/?), which is incapable of de novo synthesis of cholesterol, in utero treatment with TO901317 resulted in increased cholesterol content in Dhcr7?/? embryos. Our data support the hypothesis that Abca1, and possibly Sr-b1, contributes to transport maternal cholesterol to the developing fetus. Furthermore, we show, as a proof of principle, that modulating maternal–fetal cholesterol transport has potential for in utero therapy of SLOS. PMID:18775956

Lindegaard, Marie L.; Wassif, Christopher A.; Vaisman, Boris; Amar, Marcelo; Wasmuth, Elizabeth V.; Shamburek, Robert; Nielsen, Lars B.; Remaley, Alan T.; Porter, Forbes D.

2008-01-01

339

A phase I study of low-pressure hyperbaric oxygen therapy for blast-induced post-concussion syndrome and post-traumatic stress disorder.  

PubMed

This is a preliminary report on the safety and efficacy of 1.5 ATA hyperbaric oxygen therapy (HBOT) in military subjects with chronic blast-induced mild to moderate traumatic brain injury (TBI)/post-concussion syndrome (PCS) and post-traumatic stress disorder (PTSD). Sixteen military subjects received 40 1.5 ATA/60 min HBOT sessions in 30 days. Symptoms, physical and neurological exams, SPECT brain imaging, and neuropsychological and psychological testing were completed before and within 1 week after treatment. Subjects experienced reversible middle ear barotrauma (5), transient deterioration in symptoms (4), and reversible bronchospasm (1); one subject withdrew. Post-treatment testing demonstrated significant improvement in: symptoms, neurological exam, full-scale IQ (+14.8 points; p<0.001), WMS IV Delayed Memory (p=0.026), WMS-IV Working Memory (p=0.003), Stroop Test (p<0.001), TOVA Impulsivity (p=0.041), TOVA Variability (p=0.045), Grooved Pegboard (p=0.028), PCS symptoms (Rivermead PCSQ: p=0.0002), PTSD symptoms (PCL-M: p<0.001), depression (PHQ-9: p<0.001), anxiety (GAD-7: p=0.007), quality of life (MPQoL: p=0.003), and self-report of percent of normal (p<0.001), SPECT coefficient of variation in all white matter and some gray matter ROIs after the first HBOT, and in half of white matter ROIs after 40 HBOT sessions, and SPECT statistical parametric mapping analysis (diffuse improvements in regional cerebral blood flow after 1 and 40 HBOT sessions). Forty 1.5 ATA HBOT sessions in 1 month was safe in a military cohort with chronic blast-induced PCS and PTSD. Significant improvements occurred in symptoms, abnormal physical exam findings, cognitive testing, and quality-of-life measurements, with concomitant significant improvements in SPECT. PMID:22026588

Harch, Paul G; Andrews, Susan R; Fogarty, Edward F; Amen, Daniel; Pezzullo, John C; Lucarini, Juliette; Aubrey, Claire; Taylor, Derek V; Staab, Paul K; Van Meter, Keith W

2012-01-01

340

Practical Considerations in the Selection and Use of Pulmonary Surfactant Therapy for Neonatal Respiratory Distress Syndrome in the Intensive Care Setting  

PubMed Central

Pulmonary surfactant is the treatment of choice for neonatal respiratory distress syndrome, as it significantly reduces infant morbidity and mortality. Extensive clinical trials compare the surfactant products and their optimal usage, but often the practical administration issues are less frequently discussed. Herein, a panel of respiratory therapists and neonatal nurse practitioners share their experience regarding surfactant usage. According to the panelists, the primary criteria for surfactant selection are the ability to rapidly decrease ventilatory requirements toward extubation, a low incidence of adverse effects, cost-effectiveness, and ease of use. In most cases, surfactant is most efficacious when given as early as possible where indicated. The surfactant products differ in their storage, handling, preparation, and administration traits, and this may affect rapid dosing of the surfactant during acute treatment. During and after administration, optimal response to therapy depends on efficient management of ventilator settings, which requires vigilant monitoring of the infant. Common adverse effects include endotracheal tube reflux, bradycardia, and desaturation. Using a surfactant which requires a small dosing volume may decrease the incidence of these adverse effects. An emerging trend in clinical practice is the quick extubation of the infant to nasal continuous positive airway pressure after surfactant administration. This practice can reduce the need for ventilation and reduce the risk of ventilator-related lung damage. Nebulization of surfactant may be a future avenue of delivery, but further research is required to determine its precise role. The practical considerations summarized in this discussion may be useful for other clinicians in their own practice. PMID:23118653

Corff, Karen E.; Greubel, Steve; McCann, Debra L.; Williams, Richard; Varner, Dwight L.

2006-01-01

341

A mixed methods feasibility study to evaluate the use of a low-intensity, nurse-delivered cognitive behavioural therapy for the treatment of irritable bowel syndrome  

PubMed Central

Introduction Irritable bowel syndrome (IBS) is characterised by symptoms such as abdominal pain, constipation, diarrhoea and bloating. These symptoms impact on health-related quality of life, result in excess service utilisation and are a significant burden to healthcare systems. Certain mechanisms which underpin IBS can be explained by a biopsychosocial model which is amenable to psychological treatment using techniques such as cognitive behavioural therapy (CBT). While current evidence supports CBT interventions for this group of patients, access to these treatments within the UK healthcare system remains problematic. Methods and analysis A mixed methods feasibility randomised controlled trial will be used to assess the feasibility of a low-intensity, nurse-delivered guided self-help intervention within secondary care gastrointestinal clinics. A total of 60 participants will be allocated across four treatment conditions consisting of: high-intensity CBT delivered by a fully qualified cognitive behavioural therapist, low-intensity guided self-help delivered by a registered nurse, self-help only without therapist support and a treatment as usual control condition. Participants from each of the intervention arms of the study will be interviewed in order to identify potential barriers and facilitators to the implementation of CBT interventions within clinical practice settings. Quantitative data will be analysed using descriptive statistics only. Qualitative data will be analysed using a group thematic analysis. Ethics and dissemination This study will provide essential information regarding the feasibility of nurse-delivered CBT interventions within secondary care gastrointestinal clinics. The data gathered during this study would also provide useful information when planning a substantive trial and will assist funding bodies when considering investment in substantive trial funding. A favourable opinion for this research was granted by the Nottingham 2 Research Ethics Committee. Trial registration number ISRCTN: 83683687 (http://www.controlled-trials.com/ISRCTN83683687). PMID:24939813

Dainty, Andrew David; Fox, Mark; Lewis, Nina; Hunt, Melissa; Holtham, Elizabeth; Timmons, Stephen; Kinsella, Philip; Wragg, Andrew; Callaghan, Patrick

2014-01-01

342

Health economics perspective of fesoterodine, tolterodine or solifenacin as first-time therapy for overactive bladder syndrome in the primary care setting in Spain  

PubMed Central

Background Overactive bladder (OAB) is associated with high healthcare costs, which may be partially driven by drug treatment. There is little comparative data on antimuscarinic drugs with respect to resource use and costs. This study was conducted to address this gap and the growing need for naturalistic studies comparing health economics outcomes in adult patients with OAB syndrome initiating treatment with different antimuscarinic drugs in a primary care setting in Spain. Methods Medical records from the databases of primary healthcare centres in three locations in Spain were assessed retrospectively. Men and women ?18 years of age who initiated treatment with fesoterodine, tolterodine or solifenacin for OAB between 2008 and 2010 were followed for 52 weeks. Healthcare resource utilization and related costs in the Spanish National Health System were compared. Comparisons among drugs were made using multivariate general linear models adjusted for location, age, sex, time since diagnosis, Charlson comorbidity index, and medication possession ratio. Results A total of 1,971 medical records of patients (58.3% women; mean age, 70.1 [SD:10.6] years) initiating treatment with fesoterodine (n?=?302), solifenacin (n?=?952) or tolterodine (n?=?717) were examined. Annual mean cost per patient was €1798 (95% CI: €1745; €1848). Adjusted mean (95% bootstrap CI) healthcare costs were significantly lower in patients receiving fesoterodine (€1639 [1542; 1725]) compared with solifenacin (€1780 [€1699; €1854], P?=?0.022) or tolterodine (€1893 [€1815; €1969], P?=?0.001). Cost differences occurred because of significantly fewer medical visits, and less use of absorbent products and OAB-related concomitant medication in the fesoterodine group. Conclusions Compared with solifenacin and tolterodine, fesoterodine was a cost-saving therapy for treatment of OAB in the primary care setting in Spain. PMID:24144225

2013-01-01

343

[Acute colonic pseudoobstruction: Ogilvie's syndrome].  

PubMed

Based on literature and own original clinical data authors conclude that Ogilvie's syndrome is the form of dynamic obstruction of colon due to lesion of retroperitoneal neural nodes, heart failure and intoxication. Ogilvie's syndrome complicates therapeutic and surgical diseases. This syndrome can be manifested with acute abdomen symptoms and at 22% cases may be the cause of surgical treatment. Ogilvie's syndrome is successfully treated with evacuation of intestinal contents, but the risk of recurrence after this treatment is high. Ethiotropic therapy, correction of water-electrolytic balance and tissues oxygenation, administration of acetylcholinesterase inhibitors are the more effective treatment of this syndrome. PMID:17690630

Trenin, S O; Shishkov, A V; Maslennikov, V A; Keropian, O K

2007-01-01

344

LA COOPERATION D'ONCOGENES DANS LA TRANSFORMATION CELLULAIRE Samarut J. 1.(J!J(Jrll{)in! (Ir liinloyicMol(!clJlain! el C(!Julairc, [cole Normale 5lJpl!ricLJrc  

E-print Network

LA COOPERATION D'ONCOGENES DANS LA TRANSFORMATION CELLULAIRE Samarut J. 1.(J!J(JrĂŤll{)in! (Ir'HEPATOCYTES DE RAT PAR TRANSFECTION IN VITRO OU IN VIVO A L'AIDE DE PLASMIDES ONCOGENES (Ha-ras ou BPV) VEHICULES l'AR DES LIPOSOMES : EXPRESSION D'UN ONCOGENE ET DE PHENOTYPES METABOLIQUES DU FOIE. Fischbach M

Paris-Sud XI, Université de

345

The efficacy of interferential current therapy and chiropractic adjustments for the treatment of quadratus lumborum trigger points found in acute sacroiliac joint syndrome .  

E-print Network

??Objectives: To investigate the effects of a combined therapeutic approach from the chiropractic perspective for the treatment of acute sacroiliac joint syndrome. Chiropractic adjustments combined… (more)

Von Hörsten, Marilize

2012-01-01

346

Paraneoplastic syndromes  

SciTech Connect

Paraneoplastic syndromes (PNS) comprise a diverse group of disorders that are associated with cancer but unrelated to the size, location, metastases, or physiologic activities of the mature tissue of origin. They are remote effects of tumors that may appear as signs, symptoms or syndromes which can mimic other disease conditions encountered in veterinary medicine. Various types of PNS, singly or in multiples, may be associated with either benign or malignant tumors and may involve almost every organ system, directly or indirectly. These disorders can precede the discovery of the tumor by weeks, months, or even years, and many are good diagnostic and prognostic indicators. The true incidence of PNS in animal cancer patients is unknown, although approximately 75% of all human cancer patients, at some time during the tumor-bearing part of their lives, suffer from one or more of these disorders. Recognition of PNS is valuable because the observed abnormalities may represent tumor cell markers and facilitate early diagnosis of the tumor, because they may allow assessment of premalignant states, because they may aid in the search for metastases, because they may help quantify and monitor response to therapy, and because they may provide insight into the study of malignant transformations and oncogene expression. Recognition of these syndromes is relevant to the diagnosis and treatment of many problems in veterinary cancer medicine. 22 refs., 2 tabs.

Weller, R.E.

1986-10-01

347

Tourette Syndrome  

MedlinePLUS

NINDS Tourette Syndrome Information Page Condensed from Tourette Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Trials Organizations Additional resources from MedlinePlus What is Tourette Syndrome? Tourette syndrome (TS) is a neurological disorder ...

348

Tourette Syndrome  

MedlinePLUS

What Is Tourette Syndrome? Tourette syndrome is a condition that affects a person's central nervous system and causes tics. Tics are ... few months or a year. Continue Who Gets Tourette Syndrome? Tourette syndrome can affect people of all ...

349

Wallenberg's Syndrome  

MedlinePLUS

NINDS Wallenberg's Syndrome Information Page Synonym(s): Lateral Medullary Syndrome Table of Contents (click to jump to sections) What is ... is being done? Clinical Trials Organizations What is Wallenberg's Syndrome? Wallenberg’s syndrome is a neurological condition caused ...

350

[Post-polyiomyelitis syndrome].  

PubMed

Postpoliomyelitis syndrome is a clinical syndrome characterized by late progression of symptoms, neuromuscular weakness, fatigue and pain, several (more than 20) Years after acute anterior poliomyelitis. In the United States, where it has been mainly described, frequency is estimated between 20 and 30p.100 in patients with sequelae of poliomyelitis. Although the cause is still unknown, postpoliomyelitis syndrome is likely due to degeneration and dysfunction of terminal axons of enlarged post-polio units, with a possible role of inflammatory reaction driven by persistence of the polio virus. Due to lack of specific therapy, rational therapeutic approaches are symptomatic, including exercise, reassurance and life-strategies for fatigue. PMID:15034482

Clavelou, P

2004-02-01

351

Pulmonary-renal syndromes.  

PubMed

Pulmonary-renal syndromes or lung-kidney syndromes are clinical syndromes defined by a combination of diffuse alveolar haemorrhage (DAH) and glomerulonephritis. Pulmonary-renal syndromes are not a single entity, but are caused by a wide variety of diseases, including various forms of primary systemic vasculitis (especially Wegener's granulomatosis and microscopic polyangiitis), Goodpasture's syndrome (associated with autoantibodies to the alveolar and glomerular basement membrane) and systemic lupus erythematosus. The diagnosis rests on the identification of particular patterns of clinical, radiologic, pathologic and laboratory features. Serologic testing is important in the diagnostic work-up of patients presenting with a pulmonary-renal syndrome. The majority of cases of pulmonary-renal syndrome are associated with ANCAs, either c-ANCA or p-ANCA, due to autoantibodies against the target antigens proteinase-3 and myeloperoxidase respectively. The antigen target in Goodpasture's syndrome is type IV collagen, the major component of basement membranes. Diffuse alveolar haemorrhage is characterized by the presence of a haemorrhagic bronchoalveolar lavage (BAL) in serial BAL samples. In the clinical setting of an acute nephritis syndrome, percutaneous renal biopsy is commonly performed for histopathology and immunofluorescence studies. Treatment of generalized ANCA-associated vasculitis consists of corticosteroids and immunosuppressive agents such as cyclophosphamide (as induction therapy) or azathioprine (as maintenance therapy once remission has been achieved). The combination of plasmapheresis with these cytotoxic agents and steroids is effective in patients with Goodpasture's syndrome, especially if instituted early in the course of the disease. Recent evidence suggests that patients with severe ANCA-associated vasculitis, defined by the presence of diffuse alveolar haemorrhage and/or severe renal involvement (creatinine concentration > 5.7 mg/dl), might benefit from plasma exchange in combination with cyclophosphamide and corticosteroids. PMID:17547289

Brusselle, G G

2007-01-01

352

Clinical comparision of intravesical hyaluronic acid and hyaluronic acid-chondroitin sulphate therapy for patients with bladder pain syndrome/interstitital cystitis  

PubMed Central

Introduction: Patients with a history of bladder pain syndrome/interstitial cystitis (BPS/IC) and who responded poorly or unsatisfactorily with previous treatment were compared taking intravesical hyaluronic acid (HA) or hyaluronic acid-chondroitin sulphate (HA-CS). Methods: Patients were treated with intravesical instillation with 50 mL sterile sodium hyalurinic acid (Hyacyst, Syner-Med, Surrey, UK) (n = 32) and sodium hyaluronate 1.6% sodium chondroitin sulphate 2% (Ialuril, Aspire Pharma, UK) (n = 33). Intravesical instillations were performed weekly in first month, every 15 days in the second month and monthly in third and fourth months, for a total of 8 doses. Patients were evaluated using a visual analog pain scale (VAS), interstitial cystitis symptom index (ICSI), interstitial cystitis problem index (ICPI), voiding diary for frequency/nocturia, cystometric bladder capacity and voided volume at the beginning and at 6 months. All patients had a potassium sensitivity test (PST) initially. Wilcoxon and Mann-Whitney U tests were used for statistical analysis. Results: In total, 53 patients met the study criteria. There were 30 patients in the HA-CS group (mean age: 48.47) and 23 patients in the HA group (mean age: 49.61) (p > 0.05). The initial PST was positive in 71.7% patients (38/53) overall with no difference between groups (p > 0.05). Responses for VAS, ICCS, ICPS, 24-hour frequency/nocturia statistically improved in both groups at 6 months. There was no significant difference in symptomatic improvement (p > 0.05). Eight patients had mild adverse events. Conclusion: HA and HA/CS instillation can be effective in BPS/IC patients who do not respond to conservative treatment. An important limitation of our study is that the HA dosage of the 2 treatment arms were different. It would be more appropriate with same HA dosage in both groups; however, there was no commercially available glycosaminoglycan (GAG) substance with same HA dosage for single and combination therapy. Large, long-term randomized studies are required to determine if there is a difference between these treatments. PMID:25295131

Gülp?nar, Ömer; Kay??, Aytaç; Süer, Evren; Gökçe, Mehmet ?lker; Güçlü, Adil Güçal; Ar?kan, Nihat

2014-01-01

353

The effect of interleukin-1 receptor antagonist therapy on markers of inflammation in non-ST elevation acute coronary syndromes: the MRC-ILA Heart Study  

PubMed Central

Aims Acute coronary syndromes (ACSs) are driven by inflammation within coronary plaque. Interleukin-1 (IL-1) has an established role in atherogenesis and the vessel-response to injury. ACS patients have raised serum markers of inflammation. We hypothesized that if IL-1 is a driving influence of inflammation in non-ST elevation ACS (NSTE-ACS), IL-1 inhibition would reduce the inflammatory response at the time of ACS. Methods and results A phase II, double-blinded, randomized, placebo-controlled, study recruited 182 patients with NSTE-ACS, presenting <48 h from onset of chest pain. Treatment was 1:1 allocation to daily, subcutaneous IL-1receptor antagonist (IL-1ra) or placebo for 14 days. Baseline characteristics were well matched. Treatment compliance was 85% at 7 days. The primary endpoint (area-under-the-curve for C-reactive protein over the first 7 days) was: IL-1ra group, 21.98 mg day/L (95%CI 16.31–29.64); placebo group, 43.5 mg day/L (31.15–60.75) (geometric mean ratio = 0.51 mg/L; 95%CI 0.32–0.79; P = 0.0028). In the IL-1ra group, 14-day achieved high-sensitive C-reactive protein (P < 0.0001) and IL-6 levels (P = 0.02) were lower than Day 1. Sixteen days after discontinuation of treatment (Day 30) high-sensitive C-reactive protein levels had risen again in the IL-1ra group [IL-1ra; 3.50 mg/L (2.65–4.62): placebo; 2.21 mg/L (1.67–2.92), P = 0.022]. MACE at Day 30 and 3 months was similar but at 1 year there was a significant excess of events in the IL-1ra group. Conclusion IL-1 drives C-reactive protein elevation at the time of NSTE-ACS. Following 14 days IL-1ra treatment inflammatory markers were reduced. These results show the importance of IL-1 as a target in ACS, but also indicate the need for additional studies with anti-IL-1 therapy in ACS to assess duration and safety. Clinical Trial Registration EUCTR: 2006-001767-31-GB: www.clinicaltrialsregister.eu/ctr-search/trial/2006-001767-31/GB. PMID:25079365

Morton, Allison C.; Rothman, Alexander M. K.; Greenwood, John P.; Gunn, Julian; Chase, Alex; Clarke, Bernard; Hall, Alistair S.; Fox, Keith; Foley, Claire; Banya, Winston; Wang, Duolao; Flather, Marcus D.; Crossman, David C.

2015-01-01

354

Meta-analysis of time-related benefits of statin therapy in patients with acute coronary syndrome undergoing percutaneous coronary intervention.  

PubMed

Patients with acute coronary syndromes (ACSs) still experience high rates of recurrent coronary events, particularly, early in their presentation. Statins yield substantial cardiovascular benefits, but the optimal timing of their administration, before or after percutaneous coronary intervention (PCI), remains unclear. We aimed to perform a meta-analysis of randomized controlled trials of statin administration before or after PCI versus no statin or low-dose statin in patients with ACS. Primary end points were 30-day all-cause mortality and 30-day myocardial infarction (MI) stratified by pre- and post-PCI statin administration. Secondary end points were major adverse cardiac events (MACEs) or major adverse cardiac and cerebrovascular events (MACCEs). Long-term analysis beyond 30 days was also performed. Twenty randomized controlled trials enrolling 8,750 patients were included. At 30 days, the rate of MI was significantly lower in the statin group (odds ratio [OR] 0.67, 95% confidence interval [CI] 0.53 to 0.84, p = 0.0007) with a trend toward reduced mortality (p = 0.06) and significant reductions in MACE and MACCE compared with no or low-dose statin. The 30-day incidence of MI was markedly lower when statins were administered before PCI (OR 0.38, 95% CI 0.24 to 0.59, p <0.0001) rather than after PCI (p = 0.28). The direction and magnitude of the estimates for before and after PCI versus no statin or low-dose statin were sustained at long term, not reaching statistical significance for MI (OR 0.81, 95% CI 0.65 to 1.01, p = 0.06) but with significant reductions in MACE (p = 0.0002). By meta-regression, earlier statin administration correlated significantly with lower risk of MI, MACE, and MACCE at 30 days. In conclusion, the present meta-analysis indicates a time-related impact of statin therapy on clinical outcomes of patients with ACS undergoing PCI: the earlier the administration before PCI, the greater the benefits. PMID:24792742

Navarese, Eliano Pio; Kowalewski, Mariusz; Andreotti, Felicita; van Wely, Marleen; Camaro, Cyril; Kolodziejczak, Michalina; Gorny, Bartosz; Wirianta, Jeffrey; Kubica, Jacek; Kelm, Malte; de Boer, Menko-Jan; Suryapranata, Harry

2014-05-15

355

Ogilvie's Syndrome following Cardioversion for Atrial Fibrillation  

PubMed Central

Acute colonic pseudoobstruction, also known as Ogilvie's syndrome, is characterized by distension of the colon in the absence of a mechanical obstruction as evident by abdominal radiography. This syndrome is usually treated conservatively; however, medical or surgical therapies can be employed in refractory cases. Ogilvie's syndrome has been reported following cardiac events, such as myocardial infarction, heart failure, and cardiac bypass surgeries. We report the first case of Ogilvie's syndrome following synchronized electric cardioversion for atrial fibrillation. PMID:25214851

Al-Halawani, Moh'd; Thawabi, Mohammad; Abdeen, Yazan; Miller, Richard A.; Fedida, Andre A.

2014-01-01

356

Dangerous triplet: Polycystic ovary syndrome, oral contraceptives and Kounis syndrome  

PubMed Central

Polycystic ovary syndrome is characterized by ovulatory dysfunction, androgen excess and polycystic ovaries and is associated with hypertension, diabetes, metabolic syndrome and cardiovascular events. Oral contraceptives constitute ?rst-line treatment, particularly when symptomatic hyperandrogenism is present. However, these drugs are associated with cardiovascular events and hypersensitivity reactions that pose problem in differential diagnosis and therapy. We present a 14 year-old female with polycystic ovary syndrome taking oral contraceptive and suffering from recurrent coronary ischemic attacks with increased eosinophils, and troponin levels suggesting Kounis syndrome. PMID:25548619

Erol, Nurdan; Karaagac, Aysu Turkmen; Kounis, Nicholas G

2014-01-01

357

Burning Mouth Syndrome  

MedlinePLUS

... problem) ? Allergies to dental products, dental materials (usually metals), or foods ? Dry mouth, which can be caused by certain disorders (such as Sjögren’s syndrome) and treatments (such as certain drugs and radiation therapy) ? Certain medicines, such as those that reduce blood pressure ? Nutritional ...

358

Learning about WAGR Syndrome  

MedlinePLUS

... who have WAGR syndrome develop a type of kidney cancer called Wilms' tumor. In the early stages of Wilms' tumor there ... and is related to the features of the tumor, and the stage of the disease. Treatment ... radiation therapy and chemotherapy. Aniridia : The treatment of ...

359

Complex Regional Pain Syndrome  

MedlinePLUS

... pain reliever) into certain nerves to block the pain signals. If the injection works, it may be repeated. Physical therapy and psychological counseling are also helpful. However, a treatment that works for ... pain syndrome from getting worse. Sometimes the condition improves. ...

360

Lemierre’s syndrome  

PubMed Central

Lemierre’s syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic organisms, following a recent oropharyngeal infection. This has been an uncommon illness in the era of antibiotic therapy, though it has been reported with increasing frequency in the past 15 years. Lemierre’s syndrome should be suspected in young healthy patients with prolonged symptoms of pharyngitis followed by symptoms of septicemia or pneumonia, or an atypical lateral neck pain. Diagnosis is often confirmed by identification of thrombophlebitis of the internal jugular vein and growth of anaerobic bacteria on blood culture. Treatment involves prolonged antibiotic therapy occasionally combined with anticoagulation. We review the literature and a case of a child with Lemierre’s syndrome. PMID:24152679

2013-01-01

361

Dry eye and Meige's syndrome  

PubMed Central

AIMS—To determine the relation between dry eye and Meige's syndrome.?METHODS—325 patients with dry eye were divided into those responsive to topical and other forms of treatment (n=276) and those who were not (n=49). A neuropsychiatric examination was performed to check for Meige's syndrome in the latter group.?RESULTS—Twenty eight (57%) of the treatment unresponsive patients were diagnosed with Meige's syndrome.?CONCLUSIONS—There is a subgroup of patients with dry eye who do not respond to simple therapy. More than half of these patients have Meige's syndrome and need psychiatric, as well as ophthalmic, care.?? PMID:9274405

Tsubota, K.; Fujihara, T.; Kaido, M.; Mori, A.; Mimura, M.; Kato, M.

1997-01-01

362

[Hyponatremic syndrome].  

PubMed

Sodium, the most important extracellular fluid electrolyte, is the focus of several homeostatic mechanisms that regulate fluid and electrolyte balance. Hyponatremia is a common electrolyte abnormality caused by an actual sodium deficiency or extracellular compartment fluid excess. Clinical symptoms are related with acuity and speed with which this abnormality is established. The symptoms are mainly neurological and neuromuscular disorders (headache, confusion, stupor, seizures, coma) due to brain cells edema. Hyponatremia due to sodium deficiency is caused by sodium loss from kidney (nephritis, diuretics, mineralocorticoid deficiency) and / or extrarenal (vomiting, diarrhea, burns). Hyponatremia due to water excess seems to be the most common and it is attributable to cirrhosis, nephrotic syndrome, heart failure, infusion 5% glucose solutions and drugs that stimulate ADH secretion. It was recently highlighted the role of inflammation and IL-6 in the non-osmotic ADH release. Hyponatremia is considered also marker of phlogosis. Acute (<48 h) and severe (<125 mEq/ L) hyponatremia is a medical emergency that requires prompt correction. Patients with chronic hyponatremia have a high risk of osmotic demyelination syndrome if rapid correction of the plasmatic sodium occurs. In combination with conventional therapy, a new class of drugs, vasopressin receptors antagonists (AVP-R antagonists) would be able to increase the excretion of electrolyte-free water and the serum sodium concentration. PMID:22362242

Urso, C; Caimi, G

2012-01-01

363

Down Syndrome  

MedlinePLUS

... Information Clinical Trials Resources and Publications En Espańol Down Syndrome: Condition Information Skip sharing on social media links Share this: Page Content What is Down syndrome? Down syndrome describes a set of cognitive and ...

364

Brown Syndrome  

MedlinePLUS

... Does Brown syndrome cause eye problems besides abnormal eye movements? Some children with Brown syndrome have poor binocular ... In the congenital form of Brown syndrome, the eye movement problem is usually constant and unlikely to resolve ...

365

Metabolic Syndrome  

MedlinePLUS

... is a signal that someone could be on the road to serious health problems. Diagnosing metabolic syndrome helps ... like heart disease or type 2 diabetes down the road. What Exactly Is Metabolic Syndrome? Metabolic syndrome is ...

366

Williams syndrome  

MedlinePLUS

Williams-Beuren syndrome ... Williams syndrome is a rare condition caused by missing a copy of several genes. Parents may not have ... history of the condition. However, a person with Williams syndrome has a 50% chance of passing the disorder ...

367

Angelman Syndrome  

MedlinePLUS

... being done? Clinical Trials Organizations What is Angelman Syndrome? Angelman syndrome is a genetic disorder that causes developmental ... and ultimately cure them. NIH Patient Recruitment for Angelman Syndrome Clinical Trials At NIH Clinical Center Throughout the ...

368

[Secondary myelodysplastic syndrome].  

PubMed

Secondary or therapy related myelodysplastic syndrome may develop following treatment with alkylating agents and radiotherapy. The condition may also follow high dose therapy for malignant lymphomas. We describe two patients who developed secondary myelodysplasia. The first is a 76-year old female with a low grade lymphoma who developed pancytopenia with monosomy 7. Secondary myelodysplasia was diagnosed 24 months after treatment with chlorambucil (total dose of 900 mg) and 12 months after treatment with trophosphamide (total dose of 24 g). The second patient was a 48-year old male who underwent autologous bone marrow transplantation for a relapsed low grade lymphoma. Following autografting he had persistent anemia and trombocytopenia. Secondary myelodysplastic syndrome with complex karyotype was diagnosed 54 months after high dose therapy. He died nine months later of bone marrow failure. PMID:9441464

Shammas, F V; Heikkilä, R

1997-11-30

369

Resolution of Hypoprothrombinemia-Lupus Anticoagulant Syndrome (HLAS) after multidrug therapy with rituximab: a case report and review of the literature.  

PubMed

Hypoprothrombinemia associated with a lupus anticoagulant (LA) was first reported in the literature over 50 years ago. The hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare bleeding diathesis that has been associated with LAs in adult and paediatric patients with systemic lupus erythematosus (SLE) and with transient LAs due to other causes. There are no standard recommendations for treating haemorrhage associated with this syndrome. Herein, we report a patient with SLE and HLAS who achieved a durable remission following treatment with intravenous immune globulin (IVIG), prednisone and rituximab. PMID:22989209

Paschal, R D; Neff, A T

2013-03-01

370

Therapeutic effects of CO2 laser therapy of linear nevus sebaceous in the course of the Schimmelpenning-Feuerstein-Mims syndrome  

PubMed Central

The Schimmelpenning-Feuerstein-Mims (SFM) syndrome is a rare phakomatosis which comprises a nevus sebaceous of Jadassohn, seizures and developmental delay associated with a wide spectrum of extracutaneous abnormalities including neurological, skeletal, ocular, cardiovascular and urogenital defects. We are presenting a case of an 18-year-old patient with systemic features of the SFM syndrome and an extensive linear nevus sebaceous partially removed with a carbon dioxide (CO2) laser. The treatment options of skin lesions in patients with SFM are discussed. PMID:24353494

Kacalak-Rzepka, Anna; Królicki, Andrzej; Maleszka, Romuald; Bielecka-Grzela, Stanis?awa

2013-01-01

371

Bone Age Progression during the First Year of Growth Hormone Therapy in Pre-Pubertal Children with Idiopathic Growth Hormone Deficiency, Turner Syndrome or Idiopathic Short Stature, and in Short Children Born Small for Gestational Age: Analysis of Data from KIGS (Pfizer International Growth Database)  

Microsoft Academic Search

Background\\/Aims: The beneficial effects of growth hormone (GH) therapy on statural growth in children are well established, but the effects on skeletal maturation are less clear. The progression of bone age (BA) was therefore studied during the first year of GH treatment in pre-pubertal children with idiopathic GH deficiency (GHD), Turner syndrome (TS) or idiopathic short stature (ISS), and in

Feyza Darendeliler; Michael B. Ranke; Bert Bakker; Anders Lindberg; Christopher T. Cowell; Kerstin Albertsson-Wikland; Edward O. Reiter; David A. Price

2005-01-01

372

Tourette's syndrome.  

PubMed

Tourette's syndrome (TS) is a chronic disorder characterized by motor and vocal tics and a variety of associated behaviour disorders. Because current therapy is often unsatisfactory, there is expanding interest in new therapeutic strategies that are more effective, cause less side effects and ameliorate not only tics but also behavioural problems. From anecdotal reports and preliminary controlled studies it is suggested that - at least in a subgroup of patients - cannabinoids are effective in the treatment of TS. While most patients report beneficial effects when smoking marijuana (Cannabis sativa L.), available clinical trials have been performed using oral ??-tetrahydrocannabinol (THC). In otherwise treatment-resistant TS patients, therefore, therapy with THC should not be left unattempted. To date, it is unknown whether other drugs that interact with the endocannabinoid receptor system might be more effective in the treatment of TS than smoked marijuana or pure THC. Since it has been suggested that abnormalities within the endocannabinoid receptor system might underlie TS pathophysiology, it would be of interest to investigate the effect of substances that for example bind more selectively to the central cannabinoid receptor or inhibit the uptake or the degradation of different endocannabinoids. PMID:21104394

Müller-Vahl, Kirsten R

2009-01-01

373

Role of cyclooxygenase-2 in hyperprostaglandin E syndrome\\/antenatal Bartter syndrome  

Microsoft Academic Search

Role of cyclooxygenase-2 in hyperprostaglandin E syndrome\\/antenatal Bartter syndrome.BackgroundHyperprostaglandin E syndrome\\/antenatal Bartter syndrome (HPS\\/aBS) is a congenital salt-losing tubulopathy with an induced expression of cyclooxygenase-2 (COX-2) in the macula densa probably leading to hyperreninemia. Inhibition of stimulated prostaglandin E2 (PGE2) formation with indomethacin results in a significant improvement of clinical symptoms and is therefore standard therapy. Using the COX-2 selective

Stephan C Reinalter; Nikola Jeck; Christoph Brochhausen; Bernhard Watzer; Rolf M Nüsing; Hannsjörg W Seyberth; Martin Kömhoff

2002-01-01

374

Antiretroviral therapy associated mastopathy.  

PubMed

Reports about bilateral breast enlargement in patients on antiretroviral therapy are rare. It forms a constituent of the human immunodeficiency virus lipodystrophy syndrome. Often clinical suspicion followed by appropriate imaging evaluation is confirmative.A case of antiretroviral therapy associated mastopathy is therefore presented here so that increased awareness would obviate the need of mastectomy in such cases. We also emphasise the role of adequate counselling in this scenario in alleviating patients' anxiety. PMID:23024714

Sankaye, Smita; Kachewar, Sushil

2012-01-01

375

Changes in Intra-Abdominal Visceral Fat and Serum Leptin Levels in Patients With Obstructive Sleep Apnea Syndrome Following Nasal Continuous Positive Airway Pressure Therapy  

Microsoft Academic Search

Background—Obstructive sleep apnea syndrome (OSAS) is a common disorder in obese subjects. Visceral fat accumulation (VFA) is a better predictor of coronary heart disease than body mass index. Leptin is a hormone involved in the control of body weight and fat distribution. The effect of nasal continuous positive airway pressure (NCPAP) treatment on VFA and serum leptin levels in OSAS

Kazuo Chin; Kouichi Shimizu; Takaya Nakamura; Noboru Narai; Hiroaki Masuzaki; Yoshihiro Ogawa; Michiaki Mishima; Takashi Nakamura; Kazuwa Nakao; Motoharu Ohi

2010-01-01

376

Management of Antiphospholipid Syndrome  

PubMed Central

The antiphospholipid syndrome (APS) is an autoimmune disorder presenting with tissue injury in various organs related to large- or small-vessel thrombosis associated with antiphospholipid and antiprotein/phospholipid complex antibodies. Although the pathophysiology, diagnosis, and clinical scenario may seem clear and straightforward, a more detailed examination reveals a more complex and uncertain picture related to the management of APS. This article reviews the current situation relating to APS therapy by evaluating the different clinical features of the syndrome ranging from thrombosis to pregnancy complications together with new strategies and pharmacological approaches. PMID:22870449

Del Papa, Nicoletta; Vaso, Nikoleta

2010-01-01

377

Understanding Thoracic Outlet Syndrome  

PubMed Central

The diagnosis of thoracic outlet syndrome was once debated in the world of vascular surgery. Today, it is more understood and surprisingly less infrequent than once thought. Thoracic outlet syndrome (TOS) is composed of three types: neurogenic, venous, and arterial. Each type is in distinction to the others when considering patient presentation and diagnosis. Remarkable advances have been made in surgical approach, physical therapy, and rehabilitation of these patients. Dedicated centers of excellence with multidisciplinary teams have been developed and continue to lead the way in future research. PMID:25140278

Freischlag, Julie

2014-01-01

378

Understanding thoracic outlet syndrome.  

PubMed

The diagnosis of thoracic outlet syndrome was once debated in the world of vascular surgery. Today, it is more understood and surprisingly less infrequent than once thought. Thoracic outlet syndrome (TOS) is composed of three types: neurogenic, venous, and arterial. Each type is in distinction to the others when considering patient presentation and diagnosis. Remarkable advances have been made in surgical approach, physical therapy, and rehabilitation of these patients. Dedicated centers of excellence with multidisciplinary teams have been developed and continue to lead the way in future research. PMID:25140278

Freischlag, Julie; Orion, Kristine

2014-01-01

379

Antiphospholipid syndrome: an overview.  

PubMed

Antiphospholipid antibodies are a heterogeneous group of autoantibodies that are detected by immunoassays and functional coagulation tests. The antigenic targets are negatively charged phospholipids and serum phospholipid-binding proteins. The latter antibodies are frequently associated with thrombosis, fetal loss and other clinical manifestations of the antiphospholipid syndrome. These antibodies are felt to be etiologically important in the syndrome, although the precise pathogenic mechanisms are still being determined. Proposed mechanisms include antibody-mediated interference with coagulation homeostasis, activation of platelets and endothelial cells and a T-cell immune response to serum phospholipid-binding proteins. The mainstay of therapy is anticoagulation, whereas immunosuppression is ineffective. PMID:12821621

Hanly, John G

2003-06-24

380

Lemierre Syndrome: A Case of Postanginal Sepsis  

PubMed Central

Lemierre syndrome is a rare disease that's characterized by internal jugular vein thrombosis and septic emboli. These symptoms typically develop after acute oropharyngeal infection by Fusobacterium necrophorum1). Although this syndrome is less frequently seen in modern times due to the availability of antibiotics, physicians must be aware of the syndrome in order to initiate prompt antibiotics therapy, including coverage of the anerobic organisms. We discuss here the case of an 18-year-old female with Lemierre syndrome and we review the relevant literature on this syndrome. PMID:17939341

Seo, Young Tak; Kim, Ji Hoon; Ha, Byung Wook; Choi, Hyo Sun; Kim, Yong Tai; Ham, Young Hwan

2007-01-01

381

Sono-Electro-Magnetic Therapy for Treating Chronic Pelvic Pain Syndrome in Men: A Randomized, Placebo-Controlled, Double-Blind Trial  

PubMed Central

Objective To assess the efficacy and safety of sono-electro-magnetic therapy compared to placebo in men with refractory CPPS. Patients and Methods In a randomized, placebo-controlled, double-blind single center trial, we assessed the effect of sono-electro-magnetic therapy in men with treatment refractory CPPS. Sixty male patients were randomly assigned to treatment with either sono-electro-magnetic (n?=?30) or placebo therapy (n?=?30) for 12 weeks. The primary outcome was a change in the National Institutes of Health Chronic Prostatitis Symptom Index (NIH-CPSI) from baseline to 12 weeks. Results The 12-week difference between sono-electro-magnetic and placebo therapy in changes of the NIH-CPSI total score was ?3.1 points (95% CI ?6.8 to 0.6, p?=?0.11). In secondary comparisons of NIH-CPSI sub-scores, we found differences between groups most pronounced for the quality-of-life sub-score (difference at 12 weeks ?1.6, 95% CI ?2.8 to ?0.4, p?=?0.015). In stratified analyses, the benefit of sono-electro-magnetic therapy appeared more pronounced among patients who had a symptom duration of 12 months or less (difference in NIH-CPSI total score ?8.3, 95% CI ?14.5 to 2.6) than in patients with a longer symptom duration (?0.8, 95% CI ?4.6 to 3.1; p for interaction?=?0.023). Conclusions Sono-electro-magnetic therapy did not result in a significant improvement of symptoms in the overall cohort of treatment refractory CPPS patients compared to placebo treatment. Subgroup analysis indicates, however, that patients with a symptom-duration of 12 months or less may benefit from sono-electro-magnetic therapy, warranting larger randomized controlled trials in this subpopulation. Trial Registration ClinicalTrials.gov NCT00688506 PMID:25546177

Kessler, Thomas M.; Mordasini, Livio; Weisstanner, Christian; Jüni, Peter; da Costa, Bruno R.; Wiest, Roland; Thalmann, George N.

2014-01-01

382

Biological Therapy in Treating Patients With Advanced Myelodysplastic Syndrome, Acute or Chronic Myeloid Leukemia, or Acute Lymphoblastic Leukemia Who Are Undergoing Stem Cell Transplantation  

ClinicalTrials.gov

Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; 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); B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; Childhood Chronic Myelogenous Leukemia; Childhood Myelodysplastic Syndromes; Chronic Myelomonocytic Leukemia; Essential Thrombocythemia; Polycythemia Vera; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; T-cell Adult Acute Lymphoblastic Leukemia; T-cell Childhood Acute Lymphoblastic Leukemia

2013-07-03

383

Extensive cytogenetic studies of clonality following interferon-? therapy in chronic myeloid leukemia occurring in monosomic cells in a patient with Turner syndrome mosaic  

Microsoft Academic Search

In a 27-year-old female with Turner syndrome mosaic, Philadelphia (Ph) chromosome-positive chronic myeloid leukemia (CML) occurred only in the monosomic cells (45, Xc). Extensive cytogenetic studies, including triple-color fluorescence in situ hybridization (FISH), revealed that Ph-positive monosomic cells (45, Xc), Ph-negative monosomic cells and normal diploid cells (46, XX) were present in her bone marrow at diagnosis. After successful interferon

A Takeshita; K Shinjo; M Yamashita; S Fujisawa; K Naito; K Ohnishi; K Takahashi; H Matsui; R Ohno

1999-01-01

384

Severe Pulmonary Suppuration with Infection-Induced Systemic Inflammatory Response Syndrome following Tongue Cancer Surgery in a Patient Undergoing Tocilizumab Therapy for Rheumatoid Arthritis  

PubMed Central

A 65-year-old woman with rheumatoid arthritis treated by tocilizumab (TCZ) presented with tongue squamous cell carcinoma. While surgery was performed without any complications the aspiration pneumonia rapidly worsened by postoperative day 2 and severe pulmonary suppuration in the right lung field with infection-induced systemic inflammatory response syndrome (SIRS) was diagnosed. Antibiotic and respirator treatment improved her condition. The anti-inflammatory effect of TCZ may mask the symptoms and signs of severe infection with SIRS. PMID:24872899

Yamagata, Kenji; Shimojo, Nobutake; Ito, Hiroyuki; Ijima, Junya; Hasegawa, Shogo; Yanagawa, Toru; Mizutani, Taro; Bukawa, Hiroki

2014-01-01

385

Chromosomal abnormalities in women with breast cancer after autologous stem cell transplantation are infrequent and may not predict development of therapy-related leukemia or myelodysplastic syndrome  

Microsoft Academic Search

We determined prospectively the incidence of chromosomal abnormalities in patients with high-risk breast cancer (HRBC) after high-dose chemotherapy (HDCT) and autologous stem cell transplantation (ASCT), and correlated the cytogenetic abnormalities with the development of post-transplant myelodysplastic syndrome or acute myeloid leukemia (MDS\\/AML). From 1990 to 1999, 229 women with HRBC underwent ASCT. Cytogenetic analysis of bone marrow (BM) cells was

JA Martínez-Climent; AM Comes; E Vizcarra; I Benet; C Arbona; F Prósper; C Solano; B García Clavel; I Marugán; A Lluch; J García-Conde

2000-01-01

386

Noonan syndrome  

PubMed Central

Noonan Syndrome (NS) is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set posteriorly rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency and lymphatic dysplasias. The syndrome is transmitted as an autosomal dominant trait. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene on chromosome 12, resulting in a gain of function of the non-receptor protein tyrosine phosphatase SHP-2 protein. Recently, mutations in the KRAS gene have been identified in a small proportion of patients with NS. A DNA test for mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. NS should be considered in all foetuses with polyhydramnion, pleural effusions, oedema and increased nuchal fluid with a normal karyotype. With special care and counselling, the majority of children with NS will grow up and function normally in the adult world. Management should address feeding problems in early childhood, evaluation of cardiac function and assessment of growth and motor development. Physiotherapy and/or speech therapy should be offered if indicated. A complete eye examination and hearing evaluation should be performed during the first few years of schooling. Preoperative coagulation studies are indicated. Signs and symptoms lessen with age and most adults with NS do not require special medical care. PMID:17222357

van der Burgt, Ineke

2007-01-01

387

Fragile-X Syndrome and Education.  

ERIC Educational Resources Information Center

This article summarizes the major cognitive, behavioral, and language characteristics of the Fragile X syndrome and considers their educational implications. This common heritable cause of mental disability has implications for teaching style, curriculum, family counseling, and speech therapy. (DB)

Gibb, Charles; Holliday, Judith

1992-01-01

388

ADCK4 “reenergizes” nephrotic syndrome  

PubMed Central

Steroid-resistant nephrotic syndrome has a poor prognosis and often leads to end-stage renal disease development. In this issue of the JCI, Ashraf and colleagues used exome sequencing to identify mutations in the aarF domain containing kinase 4 (ADCK4) gene that cause steroid-resistant nephrotic syndrome. Patients with ADCK4 mutations had lower coenzyme Q10 levels, and coenzyme Q10 supplementation ameliorated renal disease in a patient with this particular mutation, suggesting a potential therapy for patients with steroid-resistant nephrotic syndrome with ADCK4 mutations. PMID:24270414

Malaga-Dieguez, Laura; Susztak, Katalin

2013-01-01

389

Eagle's syndrome: signs and symptoms.  

PubMed

Eagle's syndrome, an uncommon sequela of an elongated styloid process, can manifest itself as a sensation of a foreign body in the throat and a retrogoniac or anterolateral neck pain often referred to the TMJ and the ear. When treating patients affected by temporomandibular disorders (TMD), complaining of atypical orofacial pain, and with a concomitant radiographic finding of a bilaterally elongated styloid, the possible coexistence of Eagle's syndrome should always be considered. The aim of this review is to present an overview of the epidemiology, pathophysiology, diagnosis, and therapy for Eagle's syndrome and to help the clinician in differential diagnosis with other conditions that can provide similar signs and symptoms. PMID:23461263

Costantinides, Fulvia; Vidoni, Gabriele; Bodin, Christiane; Di Lenarda, Roberto

2013-01-01

390

Multi-Family Psychoeducational Support Group Therapy for Families with a Member Afflicted with Irreversible Brain Syndrome (Alzheimer's Disease): Report of a Pilot Study.  

ERIC Educational Resources Information Center

Alzheimers Disease (AD), an incurable disability which afflicts older adults, can have devastating emotional consequences for the victim and the family. In an attempt to determine the effectiveness of multifamily psychoeducational support, group therapy (MFPSGT), 22 persons (13 families) from the Alzheimer's Disease and Related Disorders…

Paley, Evelyn S.; And Others

391

Adrenal Cushing's syndrome due to bilateral macronodular adrenal hyperplasia: prediction of the efficacy of beta-blockade therapy and interest of unilateral adrenalectomy.  

PubMed

Bilateral adrenalectomy is the standard treatment for Cushing's syndrome (CS) related to ACTH-independent bilateral macronodular hyperplasia (AIMAH), although it imposes life-long adrenal insufficiency. This study reports a clinical case in order to discuss the clinical interest of pharmacological beta-blockade of illegitimate membrane receptors and unilateral adrenalectomy as alternatives to bilateral adrenalectomy for treatment of CS due to AIMAH. Evidence for cortisol stimulation by upright posture and insulin-induced hypoglycemia in a patient with CS related to AIMAH led us to try beta-blockers as a therapeutic test and then as a first line treatment. Thus, a 3-day beta-blocker test (320 mg/d propranolol) induced normalization of cortisol secretion, with return of hypercortisolism at the end of the test. A long term treatment with 320 mg/d propranolol allowed sustained normalization of cortisol secretion and progressive disappearance of Cushingoid features but after 8 months the patient complained of Raynaud's syndrome and fatigue. Lowering propranolol dosage or switching to atenolol was less efficient to reduce cortisol levels. Unilateral adrenalectomy was then performed as a second line treatment, leading to normalisation of the 24h urinary cortisol without adrenal insufficiency. Long term control of blood pressure and glycemia were observed during a 7-year follow-up without beta-blocker. In conclusion, a 3-day propranolol test may identify patients with AIMAH who can benefit from a long term beta-blocker treatment. In case of intolerance to beta-blocking agents, unilateral adrenalectomy may allow for long term control of Cushing's syndrome related to AIMAH without adrenal insufficiency. PMID:19564707

Mazzuco, Tânia L; Chaffanjon, Philippe; Martinie, Monique; Sturm, Nathalie; Chabre, Olivier

2009-01-01

392

Transient Fanconi syndrome in Quarter horses  

PubMed Central

Two Quarter horses with weight loss had glucosuria, euglycemia, and a mild metabolic acidosis suggesting a proximal renal tubular defect. Further testing revealed transient generalized aminoaciduria, lactic aciduria, and glucosuria, indicating Fanconi syndrome. Both horses recovered with supportive therapy. This is the first report of acquired Fanconi syndrome in horses. PMID:24489393

Ohmes, Cameon M.; Davis, Elizabeth G.; Beard, Laurie A.; Vander Werf, Karie A.; Bianco, Alex W.; Giger, Urs

2014-01-01

393

Learning about Down Syndrome  

MedlinePLUS

... genetic terms used on this page Learning About Down Syndrome What is Down syndrome? What are the symptoms ... syndrome Additional Resources for Down Syndrome What is Down syndrome? Down syndrome is a chromosomal condition related to ...

394

Kawasaki Syndrome  

PubMed Central

Kawasaki syndrome (KS) is an acute, sometimes fatal vasculitis of young children. KS has replaced acute rheumatic fever as the most common cause of acquired heart disease in children in the United States. The illness is manifested by prolonged fever, conjunctival injection, enanthem, exanthem, erythema and swelling of the hands and feet, and cervical adenopathy. These acute features of illness are self-limiting, but coronary artery abnormalities occur in 20% of untreated patients. The etiology of the illness is unknown, but its clinical and epidemiologic features are most consistent with an infectious cause. Common cardiovascular manifestations of the illness include myocarditis, pericardial effusion, and coronary artery aneurysm formation. Treatment with intravenous gamma globulin (IVGG) and aspirin within the first 10 days of illness reduces the prevalence of coronary artery abnormalities from 20% in those treated with aspirin alone to 4%. Patients who develop coronary artery aneurysms, particularly those who develop giant coronary artery aneurysms, may suffer myocardial infarction secondary to thrombosis or stenosis in the abnormal vessel. Additional research to determine the cause of KS is urgently needed to allow for improved diagnosis, more specific therapy, and prevention of the disorder. PMID:9665974

Rowley, Anne H.; Shulman, Stanford T.

1998-01-01

395

Asherman's syndrome.  

PubMed

Asherman's syndrome is being diagnosed with increasing frequency. Although it usually occurs following curettage of the pregnant or recently pregnant uterus, any uterine surgery can lead to intrauterine adhesions (IUA). Most women with IUA have amenorrhea or hypomenorrhea, but up to a fourth have painless menses of normal flow and duration. Those who have amenorrhea may also have cyclic pelvic pain caused by outflow obstruction. The accompanying retrograde menstruation may lead to endometriosis. In addition to abnormal menses, infertility and recurrent spontaneous abortion are common complaints. Hysteroscopy is the standard method to both diagnose and treat this condition. Various techniques for adhesiolysis and for prevention of scar reformation have been advocated. The most efficacious appears to be the use of miniature scissors for adhesiolysis and the placement of a balloon stent inside the uterus immediately after surgery. Postoperative estrogen therapy is prescribed to stimulate endometrial regrowth. Follow-up studies to assure resolution of the scarring are mandatory before the patient attempts to conceive as is careful monitoring of pregnancies for cervical incompetence, placenta accreta, and intrauterine growth retardation. PMID:21437822

March, Charles M

2011-03-01

396

Aicardi Syndrome  

MedlinePLUS

... males normally have an X and a Y chromosome.) The precise gene or genetic mechanism causing Aicardi syndrome is not yet known. Originally, Aicardi syndrome was characterized by three main features: 1) partial or complete absence of ...

397

Tourette Syndrome  

MedlinePLUS

If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics ... words, spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. ...

398

Marfan Syndrome  

MedlinePLUS

... syndrome is a condition in which your body's connective tissue is abnormal. Connective tissue helps support all parts of your body. It ... and develops. Marfan syndrome most often affects the connective tissue of the heart and blood vessels, eyes, bones, ...

399

Down Syndrome  

MedlinePLUS

... Body Works Main Page The Pink Locker Society Down Syndrome KidsHealth > Kids > Health Problems > Birth Defects & Genetic Problems > ... skills. Continue Do a Lot of People Have Down Syndrome? About 1 out of every 800 babies born ...

400

Turner Syndrome  

MedlinePLUS

Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

401

Klinefelter syndrome  

MedlinePLUS

47 X-X-Y syndrome ... have two XX chromosomes. Boys normally have an X and a Y chromosome. Klinefelter syndrome is when ... boy is born with at least one extra X chromosome. Usually, this occurs due to one extra ...

402

[Styloid syndrome; Sluder's syndrome; Charlin's syndrome].  

PubMed

The styloid syndrome is caused by an irritation of the glossopharyngeal nerve from an excessive development of the styloid apophysis. Treatment consists of the resection of the same. The Sluder syndrome represents a nevralgy with origin in the sphenopalatine ganglion and a dysfunction of the parasympathetic system. The Charlin syndrome, much less frequent, manifests itself by pains in the territory of the nasociliar nerve and the ciliar ganglion. PMID:293019

Strupler, W

1979-02-01

403

Nephrotic syndrome.  

PubMed

Nephrotic syndrome refers to excessive proteinuria, with associated hypoalbuminemia, edema, and hyperlipidemia. A diverse spectrum of disorders has been associated with nephrotic syndrome and related neurologic complications, although the relative infrequency of these cases limits conclusive associations. Neurologic manifestations of nephrotic syndrome may result from hypoproteinemia, hypercoagulability, hyperlipidemia, hypertension, amyloid deposition, hormonal changes, or electrolyte disorders. Neurologic diagnosis hinges on prompt recognition of this syndrome and rational therapeutic strategies are aimed at the underlying systemic disorder. PMID:24365309

Liebeskind, David S

2014-01-01

404

HELLP syndrome  

MedlinePLUS

HELLP syndrome is a group of symptoms that occur in pregnant women who have: H -- hemolysis (the breakdown ... The cause of HELLP syndrome has not been found. HELLP syndrome occurs in about 1 to 2 out of 1,000 pregnancies, and in ...

405

Down syndrome  

MedlinePLUS

Down syndrome is a genetic condition in which a person has 47 chromosomes instead of the usual 46. ... In most cases, Down syndrome occurs when there is an extra copy of chromosome 21. This form of Down syndrome is called Trisomy 21. ...

406

Usher Syndrome  

MedlinePLUS

Home Health Info Hearing, Ear Infections, and Deafness Usher Syndrome Usher Syndrome On this page What is Usher syndrome? Who is affected by Usher ... legally blind. Type 1 Type 2 Type 3 Hearing Profound deafness in both ears from birth Moderate to severe hearing loss from ...

407

Aase syndrome  

MedlinePLUS

Aase-Smith syndrome; Hypoplastic anemia/Triphalangeal thumb syndrome ... Jones KL, ed. Aase syndrome. In: Smith's Recognizable Patterns Of Human Malformation. 6th ed. Saunders. 2005. Clinton C, Gazda HT. Diamond-Blackfan Anemia. 2009 Jun 25 [Updated 2013 Jul ...

408

Rowell syndrome  

PubMed Central

Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine. PMID:25506561

Bhat, Ramesh Y; Varma, Chaitanya; Bhatt, Sonia; Balachandran, C

2014-01-01

409

Metabolic Syndrome  

MedlinePLUS

... to occur together. You must have at least three metabolic risk factors to be diagnosed with metabolic syndrome. A large ... syndrome may overtake smoking as the leading risk factor for heart disease. It is possible to prevent or ... Metabolic Syndrome Clinical Trials Clinical trials are ...

410

Velocardiofacial Syndrome  

ERIC Educational Resources Information Center

Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

2009-01-01

411

Rowell syndrome.  

PubMed

Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine. PMID:25506561

Bhat, Ramesh Y; Varma, Chaitanya; Bhatt, Sonia; Balachandran, C

2014-11-01

412

Alport's Syndrome in Pregnancy  

PubMed Central

Background. Alport's syndrome is an X-linked hereditary disorder affecting the glomerular basement membrane associated with ocular and hearing defects. In women, the disease is much less severe compared to that in men. However, women with Alport's syndrome can have an accelerated form of their disease during pregnancy with worsening of kidney function and can also develop preeclampsia. There are only four described cases of Alport's syndrome in pregnancy. Case Presentation. 20-year-old woman with a history of Alport's syndrome, which during pregnancy worsened resulting in hypertension, proteinuria, and acute kidney injury. Fortunately, there was complete resolution of the proteinuria and kidney injury with delivery, and the patient did not require any renal replacement therapy. Conclusion. One of the four reported cases had an accelerated form of the disease during pregnancy with rapid progression of kidney injury and end-stage renal disease. There are no definite guidelines to monitor these patients during pregnancy. Further studies are required to understand the exact pathophysiology of kidney damage that occurs in pregnant women with Alport's syndrome. This may give us some insight into the prognostic predictors, so that we can monitor these women more thoroughly and prevent adverse outcomes. PMID:23861686

Mehta, Suchita; Saifan, Chadi; Abdellah, Marie; Choueiry, Rita; Nasr, Rabih; El-Sayegh, Suzanne

2013-01-01

413

[Oto-vertebral syndrome].  

PubMed

The oto-vertebral syndrome is supposed to be caused by an early embryonic exogenous damage which at the same time affects the development of the ear and vertebral column and possibly also causes cardiac anomalies. Animal studies showed that the malformation syndrome originates in the 6th-7th week of embryonic development. The extent of the malformations of the ear and the vertebral column varies. Frequently seen are dysplasia of the external ear and dystopia and atresia of the external ear canal as well as vertebral malformations, mostly involving the thoracic region. Only 4 case reports have been published in the literature. The differential diagnosis includes Goldenhar-syndrome, oculo-vertebral syndrome. Thalidomide-syndrome and chromosomal aberrations. Therapy depends on the extent of the malformations. In case of atresia of the ear canal hearing is first of all improved with a hearing aid, operative procedures are to be planned later on. Child development is promoted with acustic stimulation. PMID:1225802

Böggering, B

1975-10-23

414

Utility of P300 auditory event related potential latency in detecting cognitive dysfunction in growth hormone (GH) deficient patients with Sheehan's syndrome and effects of GH replacement therapy  

Microsoft Academic Search

Objective: Impaired cognitive function has been demonstrated in adults with growth hormone (GH) deficiency (GHD) by using different neuropsychological tests. Despite several studies, present knowl- edge about the impact of GHD and GH replacement therapy (GHRT) on cognitive function is limited. P300 event-related potential (ERP) application is a well-established neurophysiological approach in the assessment of cognitive functions including the updating

A Golgeli; F Tanriverdi; C Suer; C Gokce; C Ozesmi; F Bayram; F Kelestimur

2004-01-01

415

Challenging pain syndromes: Parsonage-Turner syndrome.  

PubMed

Parsonage-Turner syndrome (PTS) is a rare disorder typically characterized by an abrupt onset of upper extremity pain followed by progressive neurologic deficits, including weakness, atrophy, and occasionally sensory abnormalities. The exact cause and pathophysiology of PTS are complex and incompletely understood. Autoimmune, genetic, infectious, and mechanical processes have all been implicated. No specific treatments have been proven to reduce neurologic impairment or improve the prognosis of PTS. Most patients with PTS are treated with a multidisciplinary approach that includes both physical therapy and pharmacologic treatment, often with multiple agents. Further research is needed. PMID:24787332

Smith, Clark C; Bevelaqua, Anna-Christina

2014-05-01

416

Refeeding syndrome.  

PubMed

Refeeding syndrome is a complex syndrome that occurs as a result of reintroducing nutrition (oral, enteral or parenteral) to patients who are starved or malnourished. Patients can develop fluid-balance abnormalities, electrolyte disorders (hypophosphataemia, hypokalaemia and hypomagnesaemia), abnormal glucose metabolism and certain vitamin deficiencies. Refeeding syndrome encompasses abnormalities affecting multiple organ systems, including neurological, pulmonary, cardiac, neuromuscular and haematological functions. Pathogenic mechanisms involved in the refeeding syndrome and clinical manifestations have been reviewed. We provide suggestions for the prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk, reintroduce nutrition cautiously and correct electrolyte and vitamin deficiencies properly. PMID:19712606

Fernández López, M T; López Otero, M J; Alvarez Vázquez, P; Arias Delgado, J; Varela Correa, J J

2009-01-01

417

[Bartter's syndrome].  

PubMed

Bartter syndrome is an uncommon tubular disorder inherited as an autosomal recessive entity. It is associated with hypokalemic metabolic alkalosis with high renin and aldosterone plasma concentration with low or normal blood pressure. Recent studies have demonstrated genetic heterogeneity in Bartter syndrome. Mutations of two genes encoding the Na/K/2Cl cotransporter and potassium channel ROMK are responsible for clinical features of neonatal Bartter syndrome. Mutations of gen encoding the chloride channel ClC-Kb is identified as being causative for the classic Bartter syndrome. And dysfunction of Na/Cl cotransporter in the distal convoluted renal tubule is described as Gitelman syndrome. PMID:15518434

Daniluk, Urszula; Kaczmarski, Maciej; Wasilewska, Jolanta; Matuszewska, Elzbieta; Semeniuk, Janusz; Sidor, Katarzyna; Krasnow, Aleksander

2004-05-01

418

Amniotic Membrane Transplantation as a New Therapy for the Acute Ocular Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis  

PubMed Central

Stevens-Johnson syndrome and its more severe variant, toxic epidermal necrolysis, have relatively low overall incidence; however, this disease presents with high morbidity and mortality. The majority of patients develop ocular inflammation and ulceration at the acute stage. Due to the hidden nature of these ocular lesions and the concentration of effort toward life-threatening issues, current acute management has not devised a strategy to preclude blinding cicatricial complications. This review summarizes recent literature data, showing how sight-threatening corneal complications can progressively develop from cicatricial pathologies of lid margin, tarsus, and fornix at the chronic stage. It illustrates how such pathologies can be prevented with the early intervention of cryopreserved amniotic membrane transplantation to suppress inflammation and promote epithelial healing at the acute stage. Significant dry eye problems and photophobia can also be avoided with this intervention. This new therapeutic strategy can avert the catastrophic ophthalmic sequelae of this rare but devastating disease. PMID:19699503

Shay, Elizabeth; Kheirkhah, Ahmad; Liang, Lingyi; Sheha, Hossam; Gregory, Darren G.; Tseng, Scheffer C.G.

2010-01-01

419

Endoscopic sphenoid sinus drainage in Lemierre syndrome.  

PubMed

Lemierre syndrome is a rare condition arising from an invasive oropharyngeal infection, which leads to septic thrombophlebitis of the internal jugular vein and multi-organ septic embolization. Intracranial complications are rare but serious, including subdural empyema, cavernous sinus thrombosis, and internal carotid artery aneurysms. We report a patient with Lemierre syndrome with multiple intracranial complications despite aggressive antimicrobial therapy. The patient eventually required transsphenoidal endoscopic drainage of the sphenoid sinus to help eradicate the infectious source. We postulate that in patients with Lemierre syndrome with evidence of infection in the paranasal sinuses, endoscopic sinus drainage can be an adjunct to antimicrobial therapy in achieving infection control. PMID:24060623

Golan, E; Wong, K; Alahmadi, H; Agid, R F; Morris, A; Sharkawy, A; Zadeh, G

2014-02-01

420

Amyloidosis and POEMS Syndrome  

PubMed Central

Importance of the field Treatment options for amyloidosis and POEMS have rapidly increased in the past years, but many patients are diagnosed late in the disease course and do not receive state of the art therapy Areas covered in this review Stem cell transplantation and novel agents have widened the chemotherapy alternatives available in these disorders and combinations of novel agents with high dose therapy further improve treatment opotions. This review covers the main areas of debate in the optimal treatment amyloidosis and POEMS patients, focusing on the implications for everyday clinical practice and management strategies published in the past 36 months. What the reader will gain Insights into treatment strategies are provided in the review. Keys to early recognition of the syndromes are reviewed Take home message With early diagnosis most patients are therapy candidates. New agents and new application of stem cell transplantation have dramatically improved outcomes for these previously uniformly poor prognosis disorders. PMID:20426710

Chee, Cheng E. M.D.; Dispenzieri, Angela M.D.; Gertz, Morie A. M.D

2010-01-01

421

TNF-Receptor Inhibitor Therapy for the Treatment of Children with Idiopathic Pneumonia Syndrome. A Joint Pediatric Blood and Marrow Transplant Consortium and Children's Oncology Group Study (ASCT0521).  

PubMed

Idiopathic pneumonia syndrome (IPS) is an acute, noninfectious lung disorder associated with high morbidity and mortality after hematopoietic cell transplantation. Previous studies have suggested a role for TNF? in the pathogenesis of IPS. We report a multicenter phase II trial investigating a soluble TNF-binding protein, etanercept (Enbrel, Amgen, Thousand Oaks, CA), for the treatment of pediatric patients with IPS. Eligible patients were < 18 years old, within 120 days after transplantation, and with radiographic evidence of a diffuse pneumonitis. All patients underwent a pretherapy broncho-alveolor lavage (BAL) to establish the diagnosis of IPS. Systemic corticosteroids (2.0 mg/kg/day) plus etanercept (.4 mg/kg twice weekly × 8 doses) were administered. Response was defined as survival and discontinuation of supplemental oxygen support by day 28 of study. Thirty-nine patients (median age, 11 years; range, 1 to 17) were enrolled, with 11 of 39 patients nonevaluable because of identification of pathogens from their pretherapy BAL. In the remaining 28 patients, the median fraction of inspired oxygen at study entry was 45%, with 17 of 28 requiring mechanical ventilation. Complete responses were seen in 20 (71%) patients, with a median time to response of 10 days (range, 1 to 24). Response rates were higher for patients not requiring mechanical ventilation at study entry (100% versus 53%, P = .01). Overall survival at 28 days and 1 year after therapy were 89% (95% confidence interval [CI], 70% to 96%) and 63% (95% CI, 42% to 79%), respectively. Plasma levels of proinflammatory cytokines were significantly increased at onset of therapy, subsequently decreasing in responding patients. The addition of etanercept to high-dose corticosteroids was associated with high response rates and survival in children with IPS. PMID:25270958

Yanik, Gregory A; Grupp, Stephan A; Pulsipher, Michael A; Levine, John E; Schultz, Kirk R; Wall, Donna A; Langholz, Bryan; Dvorak, Christopher C; Alangaden, Keith; Goyal, Rakesh K; White, Eric S; Collura, Jennifer M; Skeens, Micah A; Eid, Saada; Pierce, Elizabeth M; Cooke, Kenneth R

2015-01-01

422

Fanconi Syndrome in a Basenji  

PubMed Central

Renal tubular dysfunction resembling canine Fanconi syndrome in a Basenji dog is described. The signs and laboratory results were similar to other reports of this disease. Therapy to correct some of the clinical signs and serum chemistry abnormalities was attempted. PMID:17422579

Mainka, Susan A.

1985-01-01

423

Molecular Mechanisms of Myelodysplastic Syndrome  

Microsoft Academic Search

Myelodysplastic syndrome (MDS) is a family of clonal disorders of hematopoietic stem cells that are characterized by ineffective hematopoiesis and susceptibility to acute myelogenous leukemias and are shown to be strikingly refractory to current therapeutic modalities. A sub- stantial proportion of these complex diseases arises in the setting of exposures to environmen- tal or occupational toxins, including cytotoxic therapy for

Hisamaru Hirai

2003-01-01

424

State of the Art Review: Emerging Therapies: The Use of Insulin Sensitizers in the Treatment of Adolescents with Polycystic Ovary Syndrome (PCOS)  

PubMed Central

PCOS, a heterogeneous disorder characterized by cystic ovarian morphology, androgen excess, and/or irregular periods, emerges during or shortly after puberty. Peri- and post-pubertal obesity, insulin resistance and consequent hyperinsulinemia are highly prevalent co-morbidities of PCOS and promote an ongoing state of excess androgen. Given the relationship of insulin to androgen excess, reduction of insulin secretion and/or improvement of its action at target tissues offer the possibility of improving the physical stigmata of androgen excess by correction of the reproductive dysfunction and preventing metabolic derangements from becoming entrenched. While lifestyle changes that concentrate on behavioral, dietary and exercise regimens should be considered as first line therapy for weight reduction and normalization of insulin levels in adolescents with PCOS, several therapeutic options are available and in wide use, including oral contraceptives, metformin, thiazolidenediones and spironolactone. Overwhelmingly, the data on the safety and efficacy of these medications derive from the adult PCOS literature. Despite the paucity of randomized control trials to adequately evaluate these modalities in adolescents, their use, particularly that of metformin, has gained popularity in the pediatric endocrine community. In this article, we present an overview of the use of insulin sensitizing medications in PCOS and review both the adult and (where available) adolescent literature, focusing specifically on the use of metformin in both mono- and combination therapy. PMID:21899727

2011-01-01

425

Neurogenetics and Nutrigenomics of Neuro-Nutrient Therapy for Reward Deficiency Syndrome (RDS): Clinical Ramifications as a Function of Molecular Neurobiological Mechanisms  

PubMed Central

In accord with the new definition of addiction published by American Society of Addiction Medicine (ASAM) it is well-known that individuals who present to a treatment center involved in chemical dependency or other documented reward dependence behaviors have impaired brain reward circuitry. They have hypodopaminergic function due to genetic and/or environmental negative pressures upon the reward neuro-circuitry. This impairment leads to aberrant craving behavior and other behaviors such as Substance Use Disorder (SUD). Neurogenetic research in both animal and humans revealed that there is a well-defined cascade in the reward site of the brain that leads to normal dopamine release. This cascade has been termed the “Brain Reward Cascade” (BRC). Any impairment due to either genetics or environmental influences on this cascade will result in a reduced amount of dopamine release in the brain reward site. Manipulation of the BRC has been successfully achieved with neuro-nutrient therapy utilizing nutrigenomic principles. After over four decades of development, neuro-nutrient therapy has provided important clinical benefits when appropriately utilized. This is a review, with some illustrative case histories from a number of addiction professionals, of certain molecular neurobiological mechanisms which if ignored may lead to clinical complications. PMID:23926462

Blum, Kenneth; Oscar-Berman, Marlene; Stuller, Elizabeth; Miller, David; Giordano, John; Morse, Siobhan; McCormick, Lee; Downs, William B; Waite, Roger L; Barh, Debmalya; Neal, Dennis; Braverman, Eric R; Lohmann, Raquel; Borsten, Joan; Hauser, Mary; Han, David; Liu, Yijun; Helman, Manya; Simpatico, Thomas

2013-01-01

426

The dark side of SAPHO syndrome  

PubMed Central

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is a relatively rare entity. The therapeutic approach of patients with SAPHO syndrome has included multiple drugs with varying success and incoherence responses. The therapy is still empirical today. SAPHO syndrome is commonly treated with non-steroidal anti-inflammatory drugs, bisphophonates and non-biologic disease modifying antirheumatic drugs. Recent reports showed successful treatment with tumour necrosis factor ? (TNF ?) antagonists, but there is still a dark side of SAPHO syndrome including a subgroup of patient’s refractory to all the treatments that have been empirically experienced. A clinical report of a patient with SAPHO syndrome with 12 years of evolution is described. All the therapeutic approaches, including anti TNF ? therapy, have not prevented the clinical and radiographic progression of the di