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1

Transcatheter therapy of Lutembacher syndrome.  

PubMed

Lutembacher syndrome is a combination of congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). The combination of these 2 diseases has hemodynamic influences on each other and the degree of MS may be underestimated. Traditionally, Lutembacher syndrome is corrected by surgical treatment. Nowadays, these 2 diseases are amenable to transcatheter treatment without the need for surgery. Here, we describe a 28-year-old female with Lutembacher syndrome who benefited from combined transcatheter therapy of balloon valvuloplasty for MS and device closure for ASD with an Amplatzer septal occluder. PMID:17591586

Ho, Chi-Lin; Liang, Kae-Woei; Fu, Yun-Ching; Jan, Sheng-Ling; Lin, Ming-Chi; Chi, Ching-Shiang; Hwang, Betau

2007-06-01

2

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

3

Automates cellulaires : des dynamiques aux calculs,  

E-print Network

;#12;#12;#12;Automates cellulaires Systèmes dynamiques Topologie sur AZd topologie produit de la topologie discrète #12;Automates cellulaires Systèmes dynamiques Topologie sur AZd topologie produit de la topologie discrète Topologie sur AZd topologie produit de la topologie discrète métrisable par (x, y) = 2- min{ z :xz =yz } 0 x

Theyssier, Guillaume

4

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

5

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

6

Pr-ordres de simulation et automates cellulaires  

E-print Network

mod 2 #12;#12;#12;#12;Automates cellulaires Systèmes dynamiques Topologie sur AZ topologie produit de la topologie discrète métrisable par d(x, y) = 2- min{ z :xz =yz } espace compact #12;Automates cellulaires Systèmes dynamiques Topologie sur AZ topologie produit de la topologie discrète métrisable par d

Theyssier, Guillaume

7

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.

Tomatsu, Shunji; Almciga-Daz, Carlos J; Barbosa, Hector; Montao, 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

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

Schfer, 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

Evaluation and therapy of polycystic ovarian syndrome.  

PubMed

The amenorrhea associated with bilateral polycystic ovaries, described by Stein and Leventhal, actually represents a syndrome involving various organs and systems. Clinically, this symptom complex commonly presents as menstrual disturbances, infertility, excessive body weight, and hirsutism. An understanding of the pathophysiology that underlies these symptoms provides a logical basis for evaluation and treatment of the syndrome. The diagnostic approach may involve biochemical determinations (baseline, stimulated, and suppressed) and radiologic testing. Therapy is directed at chronic anovulation, the hyperandrogenism responsible for hirsutism and acne, and the prophylaxis against endometrial and breast carcinomas. Ovulation can be induced with various agents, many of which have a risk of ovarian hyperstimulation in the PCOD patient. The use of GnRH agonists with HMG or FSH for ovulation induction will probably increase in the future. Although classic wedge resection has little place in modern management of PCOD, the recent laparoscopic ovarian cautery remains largely unstudied with respect to long-term postoperative plasma androgen levels and pelvic adhesions. It is too premature to evaluate this new surgical therapy. Hirsutism is effectively treated with estrogen-progestin combinations, medroxyprogesterone acetate, androgen receptor blockers (spironolactone, cimetidine, cyproterone acetate, and cyproheptadine), and glucocorticoids. To date, the available GnRH agonists have not been found selective enough to be used in the treatment of hirsutism, owing to possible long-term complications. Most medical approaches should include electrolysis for permanent hair removal. At present, gynecologic surgery seems to have little place in the management of hirsutism. PMID:3143568

Loy, R; Seibel, M M

1988-12-01

10

Short stature in Noonan syndrome: response to growth hormone therapy  

Microsoft Academic Search

BACKGROUNDGrowth hormone (GH) has been used to promote growth in both the short and long term in a number of dysmorphic syndromes, including Turner syndrome. As this condition shares many clinical features with Noonan syndrome, it would seem logical to treat the latter group with GH.AIMSTo assess the short and long term response to GH therapy in patients with Noonan

J M W Kirk; P R Betts; G E Butler; M D C Donaldson; D B Dunger; D I Johnston; C J H Kelnar; D A Price; P Wilton

2001-01-01

11

[Autoimmune hepatitis and overlap syndrome: therapy].  

PubMed

Autoimmune Hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) represent acute and chronic inflammatory liver diseases in which immune reactions against host antigens are found to be the major pathological mechanism. Only for AIH there is evidence of an autoimmune etiology and humoral and cellular immune reactions are found directed against various liver cell antigens. By diverse autoantibodies several subgroups of autoimmune hepatitis can be distinguished. A very important disease promoting factor seems to be the genetically determined background for autoimmunity characterized by the HLA haplotype A1, B8 and DR3, respectively DR4. Although the histopathology of AIH shows no pathognomonic features distinguishing this type of hepatitis from virus induced chronic hepatitis there are some distinct characteristic morphological lesions. If untreated the prognosis of AIH is unfavourable but the benefit from immunosuppressive therapy with prednisolone and azathioprin is well established. In the last years there was increasing evidence for an overlap syndrome between AIH and PBC and rarely AIH and PSC. These patients are characterized by PBC characteristic bileduct lesions and oftenly antimitochondrial antibodies (AMA). They also show AIH typical inflammatory hepatic lesions in the periportal areas and portal tracts and oftenly the typical genetical background, the HLA haplotype A1, B8, DR3 or DR4. Most of these patients respond probably to a combination therapy containing prednisolon, azathioprine and ursodesoxycholic acid that leads to the reduction of the inflammatory activity. PMID:12233265

Lhr, H F

2002-08-21

12

Intravenous immunoglobulin therapy for Stevens-Johnson syndrome.  

PubMed

Stevens-Johnson syndrome (SJS) is an acute mucocutaneous disorder that can be associated with considerable morbidity. Several previous reports, all involving either adults with acquired immunodeficiency syndrome or children, suggest that intravenous immunoglobulin may be an effective treatment for SJS. We report a case of SJS in an immunocompetent adult whose condition improved dramatically after therapy with intravenous immunoglobulin. PMID:11284525

Brett, A S; Philips, D; Lynn, A W

2001-03-01

13

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

14

Systemic therapy of Cushings syndrome  

PubMed Central

Cushings 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, Cushings 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 2050 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 24h-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

15

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).

16

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

17

Prostaglandins and Aspirin Therapy in Bartter's Syndrome.  

National Technical Information Service (NTIS)

a young patient with Bartter's syndrome was treated for three months with 100 mg/kg/day of aspirin to inhibit prostaglanin synthesis. Clinical symptoms resolved and serum potassium increased from 2.9 to 3.5 mEq/L. Urinary excretion and plasma concentratio...

L. Norby, R. Lentz, W. Flamenbaum, P. Ramwell

1976-01-01

18

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

19

PPAR ligands: Potential therapies for metabolic syndrome  

Microsoft Academic Search

Metabolic syndrome (MS), a condition characterized by multiple related clinical disorders including insulin resistance, central\\u000a obesity, hyperlipidemia, hypertension, and heart disease, is an increasingly prevalent disease in industrialized societies.\\u000a The intense research interest in the peroxisome proliferator-activated receptors (PPARs), by both the pharmaceutical industry and academia, stems largely from the well-documented therapeutic actions of their synthetic\\u000a agonists in alleviating several

Taro E. Akiyama; Peter T. Meinke; Joel P. Berger

2005-01-01

20

Pharmacologic therapies for complex regional pain syndrome  

Microsoft Academic Search

Complex regional pain syndrome (CRPS) remains a challenging condition to diagnose and treat. There are few large-scale, randomized\\u000a trials of pharmacologic agents, and most published studies are small, uncontrolled, or presented only in abstract form at\\u000a meetings. The most commonly used agents, such as anticonvulsants, antidepressants, and opiates, have been found to be useful\\u000a for other neuropathic pain conditions in

Sean Mackey; Steven Feinberg

2007-01-01

21

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

22

[Dyspeptic syndrome associated with antidiabetic therapy].  

PubMed

Dyspeptic syndrome is a common complication of treatment with antidiabetic drugs. This may be a trivial as well as a very serious complication. Nausea, vomiting, diarrhoea, abdominal pain, loss of appetite and taste disturbances are the most common symptoms of dyspeptic problems in patients treated with metformin. They rarely are a reason for treatment discontinuation. Dyspeptic syndrome is a common complication in patients treated with acarbose, this may be prevented by reduced intake of sucrose. Pneumatosis cystoides intestinalis is a rare complication in acarbose-treated patients. Antiobesity agent orlistat is frequently associated with dyspeptic symptoms, particularly if fat intake is not reduced. Treatment with drugs affecting the incretin system (dipeptidyl peptidase-4 inhibitors and glucagon-like peptide-1 receptor agonists) is very rarely complicated by acute pancreatitis. Glucagon-like peptide-1 receptor agonists may cause dyspeptic symptoms (nausea, vomiting, diarrhoea) at the beginning of treatment. These complaints usually cease and the treatment usually does not need to be discontinued. PMID:21612066

Smahelov, A

2011-04-01

23

[Antiplatelet therapy and anticoagulation in acute coronary syndrome : Current evidence].  

PubMed

In recent years, the prognosis of patients with an acute coronary syndrome (ACS) has significantly improved. This can mainly be attributed to the implementation of primary percutaneous coronary intervention (PCI). Apart from mechanical reperfusion, an optimal medical strategy is of great importance. Antiplatelet and antithrombotic therapies in particular play a crucial role in the management of patients with ACS. New options in antiplatelet therapy are more potent P2Y12 inhibitors in addition to acetylsalicylic acid and clopidogrel. Furthermore, anticoagulant therapy before, during and after PCI can be performed by the use of unfractionated heparin, low molecular weight heparins, such as enoxaparin, the synthetic pentasaccharide fondaparinux and the direct thrombin inhibitor bivalirudin with or without additional administration of glycoprotein IIb/IIIa inhibitors. In this article, data on antiplatelet and anticoagulant therapy are presented and the current evidence is discussed. PMID:25081127

de Waha, S; Thiele, H; Richardt, G

2014-09-01

24

Centrally acting therapies for irritable bowel syndrome.  

PubMed

Irritable bowel syndrome (IBS) and other functional gastrointestinal (GI) disorders typically defy traditional diagnostic methods based on structural abnormalities, and has led to the emergence of the discipline of neurogastroenterology or the study of the "brain-gut axis," which is based on dysregulation of neuroenteric pathways as a key pathophysiological feature of IBS. Centrally acting treatments can influence these pathways and improve the clinical manifestations of pain and bowel dysfunction associated with this disorder. To successfully implement these treatment strategies, it is important to recognize their dual effects on brain and gut, understanding the nature and severity of the GI symptoms and their psychosocial concomitants, and applying them within the context of the patient's understanding of their value. PMID:21333907

Grover, Madhusudan; Drossman, Douglas A

2011-03-01

25

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

26

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

27

[Antithrombotic therapy and atrial fibrillation : Dual or triple therapy after acute coronary syndrome and stent?].  

PubMed

Current guidelines recommend dual antiplatelet therapy preferably with prasugrel or ticagrelor for 12 months in patients after acute coronary syndrome with stent implantation. Problems occur in patients with a need for oral anticoagulation, such as patients with atrial fibrillation. In these patients a combination of oral anticoagulation and platelet inhibitors is necessary. Antithrombotic combination therapy is known to increase bleeding complications. In a small randomized trial the combination of a vitamin K antagonist (VKA) and clopidogrel decreased bleeding compared to triple therapy with VKA, clopidogrel and aspirin. The new oral anticoagulants have consistently been shown to decrease bleeding complications compared to VKAs regardless of additional antiplatelet therapy. Because of the lack of randomized trials the individual decision about the intensity and duration of antithrombotic combination therapy should be based on the bleeding and ischemic risk in the individual patient. However, in most patients in addition to oral anticoagulation, antiplatelet monotherapy preferably with clopidogrel seems necessary only for 3-6 months. PMID:25336242

Zeymer, U; Zahn, R

2014-11-01

28

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

29

Treating irritable bowel syndrome: overview, perspective and future therapies  

PubMed Central

This article summarizes the ongoing challenges in irritable bowel syndrome and the exciting opportunities for development of novel therapies for this common, enigmatic condition. The challenges include insufficient understanding of mechanisms, lack of specificity of symptoms, differentiation from other conditions, and lack of availability of noninvasive tests to identify dysfunctions. However, significant opportunities are reflected by the advances in clinical trial design and, particularly, clinically relevant end points for such trials, and the increasing understanding of basic neuroenteric science. The latter has delivered two new medications to the practice (alosetron and tegaserod), and other candidate therapies (other serotonergic, tachykininergic, opioid, cannabinoid modulators) are being carefully appraised as potential drugs for the future. PMID:15037521

Camilleri, Michael

2004-01-01

30

Body awareness therapy: A new strategy for relief of symptoms in irritable bowel syndrome patients  

Microsoft Academic Search

AIM: To compare irritable bowel syndrome (IBS) pa- tients with apparently healthy persons and to evaluate body awareness therapy, which is a physiotherapeutic remedy focusing on normalising tensions in the body, for the treatment of IBS with the hypothesis that altered body tension is associated with the syndrome. METHODS: Twenty-one IBS patients received body awareness therapy two hours weekly for

EM Eriksson; IE Mller; RH Sderberg; HT Eriksson; GK Kurlberg

31

Updates in medical and surgical therapies for Tourette syndrome.  

PubMed

Tourette syndrome is a complex neurobehavioral disorder defined by multiple motor and at least 1 vocal tic, persisting over 1year, waxing and waning in severity, and not explained by another condition. The condition may range from mild nuisance to debilitating and disabling in severity. Management includes counseling and reassurance, behavioral interventions, pharmacologic, and rarely, surgical interventions. Traditionally, alpha-2 agonists and dopamine receptor antagonists have been utilized. In addition, a number of different pharmacotherapies have been implemented in the search for improved management of tics with better tolerability. In rare, severely disabling cases, neuromodulation with deep brain stimulation may be indicated. Optimal brain targets and candidate selection are still in evolution. This article will review the evidence for current medical and surgical therapies with a focus on recent updates. PMID:24871966

Malaty, Irene A; Akbar, Umer

2014-07-01

32

Iron chelation therapy in myelodysplastic syndromes: where do we stand?  

PubMed Central

Anemia leading to transfusion dependency (TD) and iron overload (IO) is commonly observed in patients with myelodysplastic syndromes (MDS). In MDS, TD and IO have been retrospectively associated with inferior survival and worse clinical outcomes, including cardiac, hepatic and endocrine dysfunction, and, in some analyses, with leukemic progression and infectious complications. Although suggested by retrospective analyses, clear prospective documentation of the beneficial effects of iron chelation therapy (ICT) on organ function and survival in MDS patients with TD and IO is currently lacking. Consequently, the role of ICT in MDS patients with TD and IO remains a very controversial aspect in the management of MDS. In this review, the authors summarize the current knowledge regarding IO in MDS and the role of ICT. PMID:23991926

Mitchell, Mhairi; Gore, Steven D; Zeidan, Amer M

2014-01-01

33

Adherence to secondary prevention therapies in acute coronary syndrome.  

PubMed

Despite overwhelming evidence of the effectiveness of secondary prevention therapies, surveys indicate poor adherence to medical treatments and lifestyle recommendations after an acute coronary syndrome. The term adherence is preferred over compliance, as the former suggests a therapeutic alliance, whereas the latter reflects passive patient obedience. Poor adherence results from a complex interplay of multiple factors at patient, practitioner and system levels. Poor adherence among patients with stable coronary artery disease is associated with increased risk of cardiovascular admissions (10%-40%), coronary interventions (10%-30%) and cardiovascular mortality (50%-80%). Improving adherence is a complex process. A range of interventions that target modifiable factors influencing adherence have been explored, but there are no guidelines to guide the choice, and multidisciplinary efforts may be needed. Future research in the area should focus on comparative efficacy of interventions to enhance adherence. PMID:25390496

Thakkar, Jay B; Chow, Clara K

2014-11-17

34

Journees Automates Cellulaires 2008 (Uz`es), pp. 54-64 SOFIC ONE HEAD MACHINES  

E-print Network

Journ´ees Automates Cellulaires 2008 (Uz`es), pp. 54-64 SOFIC ONE HEAD MACHINES A. GAJARDO-dimensional Turing machines with only one head is adopted. A subshift is associated to each Turing machine, and its properties are studied. The subshift consists in the set of sequences of symbols that the machine reads

Paris-Sud XI, Université de

35

Metabolic syndrome - Removing roadblocks to therapy: Antigenic immunotherapies.  

PubMed

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

2014-06-01

36

Stevens-Johnson syndrome complicating adalimumab therapy in Crohn's disease  

PubMed Central

The anti-tumor necrosis factor (TNF)? medications demonstrate efficacy in the induction of remission and its maintenance in numerous chronic inflammatory conditions. With the increasing number of patients receiving anti-TNF? agents, however, less common adverse reactions will occur. Cutaneous eruptions complicating treatment with an anti-TNF? agent are not uncommon, occurring in around 20% of patients. Adalimumab, a fully humanized antibody against TNF?, may be expected to cause minimal immune-mediated skin reactions compared to the chimeric monoclonal antibody, infliximab. We, however, report a case of Stevens-Johnson syndrome that required hospitalization and cessation of adalimumab in a patient with Crohns disease (CD). In this case report, a 29-year-old male with colonic and perianal CD with associated erythema nodosum and large joint arthropathy developed severe mucositis, peripheral rash and desquamation, fevers and respiratory symptoms concomitant with a second dose of 40 mg adalimumab after a 2 mo break from adalimumab therapy. Skin biopsies of the abdominal wall confirmed erythema multiforme and the patient was on no other drugs and infective etiologies were excluded. The patient responded rapidly to IV hydrocortisone and was able to be commenced on infliximab without recurrence of the Stevens-Johnson syndrome. Desquamating skin reactions have now been described in three of the TNF? antagonists (infliximab, etanercept and adalimumab). These reactions can be serious and prescribers need to be aware of the potential mucocutaneous side effects of these agents, especially as Stevens-Johnson syndrome is associated with significant morbidity and mortality. PMID:19764100

Salama, Muna; Lawrance, Ian Craig

2009-01-01

37

Evaluation of low intensity laser therapy in myofascial pain syndrome.  

PubMed

Limited studies have demonstrated that low intensity laser therapy (LILT) may have a therapeutic effect on the treatment of myofascial pain syndrome (MPS). Sixty (60) patients with MPS and having one active trigger point in the anterior masseter and anterior temporal muscles were selected and assigned randomly to six groups (n=10): Groups I to Ill were treated with GaAIAS (780 nm) laser, applied in continuous mode and in a meticulous way, twice a week, for four weeks. Energy was set to 25 J/cm2, 60 J/cm2 and 105 J/cm2, respectively. Groups IV to VI were treated with placebo applications, simulating the same parameters as the treated groups. Pain scores were assessed just before, then immediately after the fourth application, immediately after the eighth application, at 15 days and one month following treatment. A significant pain reduction was observed over time (p<0.001). The analgesic effect of the LILT was similar to the placebo groups. Using the parameters described in this experiment, LILT was effective in reducing pain experienced by patients with myofascial pain syndrome. Thus, it was not possible to establish a treatment protocol. Analyzing the analgesic effect of LILT suggests it as a possible treatment of MPS and may help to establish a clinical protocol for this therapeutic modality. PMID:19891258

Carrasco, Thaise Graciele; Guerisoli, Laise Daniela Carrasco; Guerisoli, Danilo Mathias Zanello; Mazzetto, Marcelo Oliveira

2009-10-01

38

[Methylene blue in the therapy of vasoplegic syndrome after cardiac surgery procedure].  

PubMed

Vasoplegic syndrome after cardiac surgery with cardiopulmonary bypass is severe complication with high morbidity and mortality. Without appropriate therapy the syndrome advances to the shock state with subsequent multiorgan failure. Basic haemodynamic parameters of vasoplegic syndrome include low systemic vascular resistance with severe hypotension, tachycardia, and normal or increased cardiac output and low filling pressures. In therapy norepinephrine and vasopressin or its analogues are used. Methylene blue is other therapeutic option. The case of successful application of methylene blue for the treatment of vasoplegic syndrome is presented. PMID:16639934

Rha, H; Rhov, L; Pind'k, M; Brezina, A; Pirk, J

2006-01-01

39

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

PubMed Central

Le naevus bleu cellulaire atypique est une entit pathologique rare et sa localisation au niveau du poignet est exceptionnelle. Il est Considr comme une Variante des caractristiques intermdiaires entre le naevus bleu cellulaire typique et le naevus bleu malin, dont lvolution est incertaine. Le but de notre travail est d'attirer l'attention sur cette varit lsionnelle rare et de discuter les diagnostiques diffrentiels, ainsi que dcrire les aspects histologique et les options thrapeutiques possibles. PMID:25426206

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

2014-01-01

40

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

41

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 childrens functioning levels were assessed using

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

2006-01-01

42

The Use of Cognitive-Behavioural Therapy with People with Asperger SyndromeA Case Study  

Microsoft Academic Search

A young man with Asperger syndrome who presented with severe depression and self-injurious behaviour was treated with Ig sessions of cognitive-behavioural therapy. A decrease in the levels of measured depression and self-injury were found following the therapy. It remained unclear exactly which elements of the intervention brought about the improvements. The use of cognitive-behavioural treatments with people with Asperger syndrome

Dougal Julian Hare

1997-01-01

43

[Interference therapy and radon baths in the combined treatment of patients with reflex cervicobrachial syndromes].  

PubMed

Patients with cervicobrachialgic syndromes on interference therapy, exercise treatment, massage of the cervical collar region received balneotherapy. 42 of them took water baths, 39 took dry air radon baths. These complexes proved effective in cervicobrachialgic syndromes, the effect being slightly dependent on the clinical symptoms of the disease. PMID:9987978

Gorbunov, F E; Semenistaia, S V

1998-01-01

44

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

45

Subcutaneous immunoglobulin therapy in a patient with myopathic dropped head syndrome and common variable immunodeficiency.  

PubMed

Prominent neck extension weakness is an uncommon clinical entity, also termed dropped-head syndrome, that may be part of a generalized neuromuscular disorder. We report here the case of a woman with dropped-head syndrome and pulmonary arterial hypertension secondary to systemic sclerosis. Subsequently, she developed common variable immunodeficiency and subcutaneous immunoglobulin therapy was started. After two months from the start of therapy we did not observe any improvement in the degree of flexion of the head, although the clinical examination shows an improvement in neck extensor muscle strength. Subcutaneous immunoglobulin therapy could be a possible therapeutic option for the treatment of myopathic neck extensor weakness. PMID:22217999

Rosato, E; Molinaro, I; Pisarri, S; Salsano, F

2011-01-01

46

Interdisciplinary therapy in Cornelia de Lange syndrome - review of the literature.  

PubMed

Cornelia de Lange syndrome (CdLS, also called Brachmann-de Lange syndrome - BdLS) is a multisystem developmental disorder characterized by distinctive facial features, growth and mental retardation, microcephaly, and various malformations. According to their clinical status, patients with CdLS require individual interdisciplinary therapy. The therapy is difficult and mainly symptomatic. To optimize the therapy results, early diagnosis programs and appropriate developmental and therapeutic intervention are recommended. The interdisciplinary approach described in the article needs further clinical research and detailed guidelines. PMID:23986218

Miko?ajewska, Emilia

2013-01-01

47

Impact of immune reconstitution inflammatory syndrome on antiretroviral therapy adherence  

PubMed Central

Objective We determined the impact of immune reconstitution inflammatory syndrome (IRIS) on antiretroviral therapy (ART) adherence in a cohort of 274 human immunodeficiency virus (HIV)-infected South African adults initiating ART. Methods We carried out a secondary analysis of data from a randomized controlled trial of partially supervised ART in Cape Town, South Africa. Monthly pill count adherence, viral suppression (HIV viral load < 50 c/mL), and IRIS events were documented. Poisson regression was used to identify variables associated with ART adherence below the median in the first 6 months of ART. Results We enrolled 274 patients: 58% women, median age 34 years, median CD4 count 98 cells/?L, 46% World Health Organization clinical stage IV, and 40% on treatment for tuberculosis (TB). IRIS and TB-IRIS developed in 8.4% and 6.6% of patients, respectively. The median cumulative adherence at 6 months for those with an IRIS event vs no IRIS was 95.5% vs 98.2% (P = 0.04). Although not statistically significant, patients developing IRIS had a lower 6-month viral load suppression than those without IRIS (68% vs 80%, P = 0.32). ART adherence below the median of 98% was independently associated with alcohol abuse (relative risk [RR] 1.5; 95% confidence interval [CI] 1.21.9; P = 0.003) and IRIS events (RR 1.7; 95% CI 1.22.2; P = 0.001). Conclusion Although IRIS events were associated with slightly lower adherence rates, overall adherence to ART remained high in this study population. Concerns about IRIS should not deter clinicians from early ART initiation. PMID:23271897

Nachega, Jean B; Morroni, Chelsea; Chaisson, Richard E; Goliath, Rene; Efron, Anne; Ram, Malathi; Maartens, Gary

2012-01-01

48

Minimally Invasive Therapies for Chronic Pelvic Pain Syndrome  

Microsoft Academic Search

Chronic pelvic pain syndrome (CPPS) is a common problem among men and women worldwide. It is a symptoms-complex term for interstitial\\u000a cystitis\\/painful bladder syndrome in women and chronic prostatitis\\/chronic pelvic pain syndrome in men. Patients often present\\u000a with a combination of lower urinary tract symptoms with pelvic pain and sexual dysfunction. No gold standard exists for diagnosis\\u000a or treatment of

Salim A. Wehbe; Jennifer Y. Fariello; Kristene Whitmore

2010-01-01

49

Metabolic syndrome-related hepatocellular carcinoma treated by volumetric modulated arc therapy.  

PubMed

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

2014-04-01

50

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

51

Transfert d'nergie linique et radiosensibilit cellulaire  

NASA Astrophysics Data System (ADS)

The response of human tumour cell lines to in vitro irradiation by high LET particles depends on several factors. For charged particles, there is an increase in radio-sensitivity with LET up to LET values of about 200 KeV/m, then it decreases for higher values. In clinical practice, the increase in the average LET value with depth leads to a continuous increase in relative biological effectiveness (RBE). The probability of particle traversal through the nucleus producing lethal damage is almost 1 for particles with very high LET values such as 7 MeV Ar (LET = 1500 KeV/?m); it is only 0.02 for 400 MeV O ions (LET = 20 KeV/?m). RBE is inversely related with dose, this relation being more marked for cells with small ?/beta values after photon irradiation according to the linear-quadratic formula. Particles that are less efficient than photons in terms of average cell death induce heavy individual cell damage, as shown by the yield of multiple micronuclei (MN). With regard to RBE and intrinsic radiosensitivity, recent data using both the clonogenic method and the MN assay indicate that the higher the radioresistance to photon irradiation, the higher the RBE. La rponse des cellules tumorales humaines l'irradiation in vitro par des particules de TEL lev dpend de plusieurs facteurs. Pour les particules charges, la radiosensibilit augmente avec le TEL jusqu' des valeurs de TEL voisines de 200 KeV/?m, puis diminue pour des valeurs de TEL suprieures. En clinique, l'augmentation du TEL moyen de la particule charge en profondeur est responsable d'une efficacit biologique relative (EBR) croissante. La probabilit de mortalit de la cellule par une particule traversant le noyau est proche de 1 pour les particules de TEL trs lev comme l'Ar de 7 MeV par nuclon (TEL = 1500 KeV/?m) ; elle n'est que de 0,02 pour les ions d'O de 400 MeV (TEL = 20 KeV/?m). L'EBR est plus leve pour les petites doses, la relation EBR/dose tant plus marque dans les cellules ayant un rapport ?/beta plus faible aprs exposition aux photons, selon le modle linaire-quadratique. Les particules moins efficaces que les photons en terme de mortalit cellulaire moyenne induisent des dgts individuels importants, rvls par la production de micronoyaux (MN) multiples. Concernant le lien entre l'EBR et la radio-sensibilit intrinsque, les donnes rcentes utilisant la fois la technique des colonies et la mthode des MN, montrent que l'EBR est d'autant plus leve que les cellules sont radiorsistantes aux photons.

Courdi, A.; Pignol, J. P.; Iborra-Brassart, N.; Hrault, J.; Fares, G.; Hachem, A.; Chauvel, P.

1998-04-01

52

Serotonin syndrome in dextromethorphan ingestion responsive to propofol therapy.  

PubMed

An 18-year-old male developed a severe serotonin syndrome after recreational ingestion of Coricidin HBP (chlorpheniramine 4 mg and dextromethorphan hydrobromide 30 mg). Propofol infusion rapidly normalized his agitation, neuromuscular hyperactivity, and autonomic instability. Confirmatory analysis demonstrated a dextromethorphan serum concentration of 930 ng/mL. Dextromethorphan can produce serotonin syndrome in the absence of another serotonergic drug. PMID:18007217

Ganetsky, Michael; Babu, Kavita M; Boyer, Edward W

2007-11-01

53

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

54

The role of nutrition therapy and dietitians in the management of the metabolic syndrome  

Microsoft Academic Search

Nutrition therapy interventions for the metabolic syndrome include weight reduction or maintenance, physical activity, whole\\u000a grains and fiber, and type and amount of food fats. Interventions related to carbohydrateamount and typeand alcohol are\\u000a controversial. The role of the dietitian is to assist persons with the metabolic syndrome to make lifestyle changes that modify\\u000a the factors that increase risk of diabetes

Marion J. Franz

2007-01-01

55

Piracetam Therapy Does Not Enhance Cognitive Functioning in Children With Down Syndrome  

Microsoft Academic Search

Background: Piracetam is widely used as a purported means of improving cognitive function in children with Down syndrome. Its efficacy, however, has not been rig- orously assessed. Objective: To determine whether 4 months of pi- racetam therapy (80-100 mg\\/kg per day) enhances cog- nitive function in children with Down syndrome. Design: A randomized, double-blind, placebo-con- trolled crossover study. Participants and

Nancy J. Lobaugh; Vladimir Karaskov; Vicki Rombough; Joanne Rovet; Susan Bryson; Rachel Greenbaum; Robert H. Haslam; Gideon Koren

2001-01-01

56

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, HurlerScheie 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

57

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

58

Three cases of corticosteroid therapy triggering ventricular fibrillation in J-wave syndromes.  

PubMed

We describe three cases of J-wave syndrome in which ventricular fibrillation (VF) was probably induced by corticosteroid therapy. The patients involved were being treated with prednisolone for concomitant bronchial asthma. One of the three patients had only one episode of VF during her long follow-up period (14 years). Two patients had hypokalemia during their VF episodes. Corticosteroids have been shown to induce various types of arrhythmia and to modify cardiac potassium channels. We discuss the possible association between corticosteroid therapy and VF in J-wave syndrome based on the cases we have encountered. PMID:24281399

Sakamoto, Naka; Sato, Nobuyuki; Goto, Masahide; Kobayashi, Motoi; Takehara, Naofumi; Takeuchi, Toshiharu; Talib, Ahmed Karim; Sugiyama, Eitaro; Minoshima, Akiho; Tanabe, Yasuko; Akasaka, Kazumi; Kawabe, Junichi; Kawamura, Yuichiro; Doi, Atsushi; Hasebe, Naoyuki

2014-11-01

59

Maternal and neonatal factors impacting response to methadone therapy in infants treated for neonatal abstinence syndrome  

Microsoft Academic Search

Objective:To identify maternal and neonatal factors that impact response to methadone therapy for neonatal abstinence syndrome.Study Design:This is a retrospective review of 128 infants that received pharmacotherapy for opiate withdrawal to identify factors associated with favorable response to methadone therapy. Maternal and neonatal data were analyzed with univariate statistics and multivariate logistic regression.Result:Maternal methadone maintenance dose during pregnancy correlated with

B Isemann; J Meinzen-Derr; H Akinbi

2011-01-01

60

Investigation of the effect of GaAs laser therapy on cervical myofascial pain syndrome  

Microsoft Academic Search

Low-energy laser therapy has been applied in several rheumatoid and soft tissue disorders with varying rates of success. The objective of our study was to investigate the effect of laser therapy on cervical myofascial pain syndrome with a placebo-controlled double-blind prospective study model. It was performed with a total of 53 patients (35 females and 18 males) with cervical myofascial

Lale Altan; Umit Bingl; Mehtap Ayka; Merih Yurtkuran

2005-01-01

61

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

62

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

63

Music Therapy for Children with Down Syndrome: Perceptions of Caregivers in a Special School Setting  

ERIC Educational Resources Information Center

Down syndrome (DS) is a genetic disorder resulting from chromosome 21 having three copies (trisomy 21). Cognitive functioning and anatomical features cause speech and language development delay (Kumin, 2003). Children with DS generally enjoy communication (Schoenbrodt, 2004), and respond well to interaction and social scripts. Music therapy has

Pienaar, Dorothea

2012-01-01

64

Effect of psychiatric disorders on outcome of cognitive-behavioural therapy for chronic fatigue syndrome  

Microsoft Academic Search

Psychiatric disorders have been associated with poor outcome in individuals with chronic fatigue syndrome (CFS). This study examines the impact of psychiatric disorders on outcome of cognitive-behavioural therapy (CBT). Psychiatric diagnoses were assessed with a structured psychiatric interview in a CBT trial of 270 people with CFS. Lifetime and current psychiatric disorders were found in 50 and 32% respectively. No

J. Prins; G. Bleijenberg; E. K. Rouweler

2005-01-01

65

Is a Full Recovery Possible after Cognitive Behavioural Therapy for Chronic Fatigue Syndrome?  

Microsoft Academic Search

Background: Cognitive behavioural therapy (CBT) for chronic fatigue syndrome (CFS) leads to a decrease in symptoms and disabilities. There is controversy about the nature of the change following treatment; some suggest that patients improve by learning to adapt to a chronic condition, others think that recovery is possible. The objective of this study was to find out whether recovery from

Hans Knoop; Gijs Bleijenberg; Marieke F. M. Gielissen; Peter D. White

2007-01-01

66

Increase in prefrontal cortical volume following cognitive behavioural therapy in patients with chronic fatigue syndrome  

Microsoft Academic Search

Chronic fatigue syndrome (CFS) is a disabling disorder, characterized by persistent or relapsing fatigue. Recent studies have detected a decrease in cortical grey matter volume in patients with CFS, but it is unclear whether this cerebral atrophy constitutes a cause or a consequence of the disease. Cognitive behavioural therapy (CBT) is an effective behavioural intervention for CFS, which combines a

Floris P. de Lange; Anda Koers; Joke S. Kalkman; Gijs Bleijenberg; Peter Hagoort; Ivan Toni

2008-01-01

67

Effect of low level laser therapy in rheumatoid arthritis patients with carpal tunnel syndrome  

Microsoft Academic Search

Objective: the aim of the present study was to evaluate the efficacy of low level laser therapy (LLLT) in patients with rheumatoid arthritis (RA) with carpal tunnel syndrome (CTS). Material and methods: a total of 19 patients with the diagnosis of CTS in 19 hands were included and randomly assigned to two treatment groups; LLLT (Group 1) (10 hands) with

Cengiz Onera; Meric Colak

68

Efficacy of low level laser therapy in myofascial pain syndrome: An algometric and thermographic evaluation  

Microsoft Academic Search

Background and objectives: The efficacy of low level laser therapy (LLLT) in myofascial pain syndrome (MPS) seems controversial. Our aim was to clarify the effect of LLLT in MPS by using algometry and thermography. Study Design\\/Materials and Methods: Sixty-two patients with MPS having an active trigger point in the neck or upper back region were randomly divided into two equal

Murat Birtane; Fatma Nesrin Turan

2003-01-01

69

Primary therapy for small cell lung cancer reversing the Eaton-Lambert syndrome  

SciTech Connect

A case report is presented of a patient with small cell carcinoma of the lung associated with the classic Eaton-Lambert syndrome. He received intermittent anticholinesterase therapy, with minimal improvement. Combined radiotherapy and chemotherapy for the primary neoplasm produced considerable improvement, with normal EMG findings after complete remission was achieved. 7 references, 1 table.

Kalter, S.; Dhingra, H.M.; Farha, P.

1985-02-01

70

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

ERIC Educational Resources Information Center

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

Scheeres, Korine; Wensing, Michel; Knoop, Hans; Bleijenberg, Gijs

2008-01-01

71

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

72

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

73

Medicinal leech therapy in pain syndromes: a narrative review.  

PubMed

Medicinal leech therapy is used in a variety of conditions; most of which have pain as a major symptom. Its mode of action relies on the injection of leech saliva into patients' tissues during the process of blood withdrawal. Leech saliva contains active ingredients with anti-inflammatory, thrombolytic, anti-coagulant and blood- and lymph-circulation enhancing properties. A specific analgesic substance within the leech saliva is yet to be identified. Pain relief from leech therapy is rapid, effective and long-lasting in many conditions. This review compiles studies and case reports that provide clinical evidence for leech therapy's analgesic effects. PMID:24081747

Koeppen, Detlev; Aurich, Michael; Rampp, Thomas

2014-03-01

74

Music therapy as a tool for assessing hand use and communicativeness in children with Rett Syndrome.  

PubMed

A six-year-old girl with Rett syndrome was assessed in a multi-disciplinary specialist therapy clinic and aspects of her responsiveness and developmental potential were found in the music therapy assessment. Functional hand use, eye-referencing, motivated and intentional communication were observed and reported through video analysis of a 30 min session of music therapy employing improvisational methods. Absent or reduced hand clasping/plucking, interactive turn-taking, primary and secondary inter-subjectivity, and vocalisation with appropriate emotional expression were evident. Stable truncal positioning and occasional gentle restraint of either hand improved both spontaneous and prompted activity. PMID:16182499

Wigram, Tony; Lawrence, Margaret

2005-11-01

75

Injection therapy of lumbar facet syndrome: a prospective study  

Microsoft Academic Search

Summary\\u000a Background. Chronic low back pain remains a major health problem. Facet joint injection therapy is an easy to perform therapeutic option.\\u000a However, few prospective studies use a standardized protocol to investigate injection therapy. The aim of our study was to\\u000a evaluate quantity and duration of clinical improvement after this protocol, and to identify the best time for additional repetitive

T. L. Schulte; T. A. Pietil; J. Heidenreich; M. Brock; R. Stendel

2006-01-01

76

Intravenous immunoglobulin replacement therapy to prevent pulmonary infection in a patient with Good's syndrome.  

PubMed

Good's syndrome is an acquired immunodeficiency state associated with thymoma and characterized by recurrent pulmonary infections. We describe a 67-year-old woman who presented with respiratory symptoms caused by concomitant disseminated cytomegalovirus infection and Pneumocystis jiroveci pneumonia 38 months after thymectomy for a thymoma. Immunologic analysis revealed hypogammaglobulinemia with absent B-cell population as demonstrated by flow cytometry, consistent with Good's syndrome. Following treatment with sulfamethoxazole/trimethoprim and ganciclovir, the patient improved with resolution of her respiratory symptoms. However, the patient subsequently experienced additional infections, necessitating additional subsequent hospital admissions. During the last admission, intravenous immunoglobulin (IVIG) replacement therapy was initiated and continued after discharge. Infection has been prevented for one year after beginning IVIG replacement therapy. This case reveals that in patients with combined humoral and cell-mediated immune deficiency, concomitant infection with different pathogens is not unusual, and immediate specific therapy is important. Periodic IVIG infusion, to maintain adequate Ig levels, is recommended. PMID:23200552

Wang, Ching-Hsun; Chan, Edward D; Perng, Cherng-Lih; Chian, Chih-Feng; Chen, Chien-Wen; Perng, Wann-Cherng; Su, Wen-Lin

2012-11-28

77

Menopause, the metabolic syndrome, and mind-body therapies  

PubMed Central

Cardiovascular disease risk rises sharply with menopause, likely due to the coincident increase in insulin resistance and related atherogenic changes that together comprise the metabolic or insulin resistance syndrome, a cluster of metabolic and hemodynamic abnormalities strongly implicated in the pathogenesis and progression of cardiovascular disease. A growing body of research suggests that traditional mind-body practices such as yoga, tai chi, and qigong may offer safe and cost-effective strategies for reducing insulin resistance syndrome-related risk factors for cardiovascular disease in older populations, including postmenopausal women. Current evidence suggests that these practices may reduce insulin resistance and related physiological risk factors for cardiovascular disease; improve mood, well-being, and sleep; decrease sympathetic activation; and enhance cardiovagal function. However, additional rigorous studies are needed to confirm existing findings and to examine long-term effects on cardiovascular health. PMID:18779682

Innes, Kim E.; Selfe, Terry Kit; Taylor, Ann Gill

2009-01-01

78

Diagnostik und Therapie beim Williams-Beuren-Syndrom (WBS)  

Microsoft Academic Search

Zusammenfassung Das Williams-Beuren-Syndrom gehrt zu den Mikrodeletionssyndromen und hat eine geschtzte Prvalenz von 1:7500. tiologisch liegt ihm eine hemizygote Deletion im langen Arm eines Chromosoms 7 (7q11.23) zugrunde. Die Symptomatik ist variabel und umfasst neben der typischen kraniofazialen Dysmorphie v.a. kardiovaskulre und renale Fehlbildungen, eine statomotorische und mentale Entwicklungsstrung sowie ein charakteristisches Persnlichkeitsprofil und Strungen von Wachstum und Pubertt. Altersabhngig

R. Pankau; A. Gosch; P. Meinecke; K. Sarimski; R. Schneppenheim; J. Weissenborn; A. Wessel; C.-J. Partsch

2005-01-01

79

Antiangiogenic therapy in myelodysplastic syndromes: Is there a role?  

Microsoft Academic Search

Angiogenesis has been shown to play a pivotal role in the growth and metastasis of solid tumors. Numerous in vitro and translational\\u000a research studies have implicated a role for angiogenesis in the pathogenesis of myelodysplastic syndrome (MDS). Although the\\u000a role of angiogenesis inhibitors in the treatment of solid tumors has evolved significantly over the past 5 years, their role\\u000a in

Stephen T. Oh; Jason Gotlib

2008-01-01

80

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 Velzquez M; Arnaldo Acosta; Soaira G Mendoza

1997-01-01

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

Cellules souches et thrapie cellulaire. Contribution au dbat thique  

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

83

A practical guide to the therapy of narcolepsy and hypersomnia syndromes.  

PubMed

Narcolepsy and other syndromes associated with excessive daytime sleepiness can be challenging to treat. New classifications now distinguish narcolepsy/hypocretin deficiency (also called type 1 narcolepsy), a lifelong disorder with well-established diagnostic procedures and etiology, from other syndromes with hypersomnolence of unknown causes. Klein-Levin Syndrome, a periodic hypersomnia associated with cognitive and behavioral abnormalities, is also considered a separate entity with separate therapeutic protocols. Non hypocretin-related hypersomnia syndromes are diagnoses of exclusion. These diagnoses are only made after eliminating sleep deprivation, sleep apnea, disturbed nocturnal sleep, and psychiatric comorbidities as the primary cause of daytime sleepiness. The treatment of narcolepsy/hypocretin deficiency is well-codified, and involves pharmacotherapies using sodium oxybate, stimulants, and/or antidepressants, plus behavioral modifications. These therapies are almost always needed, and the risk-to-benefit ratio is clear, notably in children. Detailed knowledge of the pharmacological profile of each compound is needed to optimize use. Treatment for other syndromes with hypersomnolence is more challenging and less codified. Preferably, therapy should be conservative (such as modafinil, atomoxetine, behavioral modifications), but it may have to be more aggressive (high-dose stimulants, sodium oxybate, etc.) on a case-by-case, empirical trial basis. As cause and evolution are unknown in these conditions, it is important to challenge diagnosis and therapy over time, keeping in mind the possibility of tolerance and the development of stimulant addiction. Kleine-Levin Syndrome is usually best left untreated, although lithium can be considered in severe cases with frequent episodes. Guidelines are provided based on the literature and personal experience of the author. PMID:23065655

Mignot, Emmanuel J M

2012-10-01

84

Acute coronary syndrome caused by coronary vasospasms associated with Churg-Strauss syndrome: effects of betamethasone therapy.  

PubMed

A 42-year-old woman with a history of aspirin-induced asthma was admitted with severe chest pain. Emergency coronary angiography revealed coronary artery spasms. The administration of vasodilators did not suppress the anginal symptoms, and the differential white blood cell count continued to show eosinophilia. The patient's symptoms of aspirin-induced asthma, eosinophilia and other allergic states led to the diagnosis of Churg-Strauss syndrome (CSS). After starting betamethasone therapy, the eosinophilia and cardiac symptoms rapidly disappeared. Although coronary vasospasms related to CSS are rare, the present case suggests that a differential white blood cell count should be obtained in patients with refractory coronary vasospasms. PMID:24694483

Suzuki, Yuji; Nishiyama, Osamu; Sakai, Toshiaki; Niiyama, Masanobu; Itoh, Tomonori; Nakamura, Motoyuki

2014-01-01

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

[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

87

Trajectory and outcomes of speech language therapy in the Prader-Willi syndrome (PWS): case report.  

PubMed

The aim of this study was to describe the trajectory and the outcomes of speech-language therapy in Prader-Willi syndrome through a longitudinal study of the case of an 8 year-old boy, along four years of speech-language therapy follow-up. The therapy sessions were filmed and documental analysis of information from the child's records regarding anamnesis, evaluation and speech-language therapy reports and multidisciplinary evaluations were carried out. The child presented typical characteristics of Prader-Willi syndrome, such as obesity, hyperfagia, anxiety, behavioral problems and self aggression episodes. Speech-language pathology evaluation showed orofacial hypotony, sialorrhea, hypernasal voice, cognitive deficits, oral comprehension difficulties, communication using gestures and unintelligible isolated words. Initially, speech-language therapy had the aim to promote the language development emphasizing social interaction through recreational activities. With the evolution of the case, the main focus became the development of conversation and narrative abilities. It were observed improvements in attention, symbolic play, social contact and behavior. Moreover, there was an increase in vocabulary, and evolution in oral comprehension and the development of narrative abilities. Hence, speech-language pathology intervention in the case described was effective in different linguistic levels, regarding phonological, syntactic, lexical and pragmatic abilities. PMID:21552737

Misquiatti, Andra Regina Nunes; Cristovo, Melina Pavini; Brito, Maria Claudia

2011-03-01

88

Genotype- and phenotype-directed antiplatelet therapy selection in patients with acute coronary syndromes.  

PubMed

Although dual antiplatelet therapy (DAPT) has been a standard treatment in patients with acute coronary syndrome (ACS) for over a decade, only recently have therapeutic options beyond aspirin and clopidogrel become available. Additional treatment options are particularly useful because of the documented history of variability in antiplatelet response. This article reviews the current treatment options for DAPT in ACS, and reviews both genotype- and phenotype-guided methods for determining optimal antiplatelet therapy for patients with ACS. Additionally, recommendations from current guidelines as well as expert commentary are provided for the use of available testing methods to determine optimal DAPT for ACS patients. PMID:25340283

Ismail, Sahar; Lee, Yee Ming; Patel, Meet; Duarte, Julio D; Ardati, Amer K

2014-11-01

89

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

90

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

91

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

92

The spectrum of monogenic autoinflammatory syndromes: understanding disease mechanisms and use of targeted therapies.  

PubMed

Monogenic autoinflammatory diseases encompass a distinct and growing clinical entity of multisystem inflammatory diseases with known genetic defects in the innate immune system. The diseases present clinically with episodes of seemingly unprovoked inflammation (fever, rashes, and elevation of acute phase reactants). Understanding the genetics has led to discovery of new molecules involved in recognizing exogenous and endogenous danger signals, and the inflammatory response to these stimuli. These advances have furthered understanding of innate inflammatory pathways and spurred collaborative research in rheumatology and infectious diseases. The pivotal roles of interleukin (IL)-1beta in cryopyrin-associated periodic syndromes, tumor necrosis factor (TNF) in TNF receptor-associated periodic syndrome, and links to inflammatory cytokine dysregulation in other monogenic autoinflammatory diseases have resulted in effective therapies targeting proinflammatory cytokines IL-1beta and TNF and uncovered other new potential targets for anti-inflammatory therapies. PMID:18606080

Glaser, Rachel L; Goldbach-Mansky, Raphaela

2008-07-01

93

Ephedrine therapy in eight patients with congenital myasthenic syndrome due to DOK7 mutations.  

PubMed

In congenital myasthenic syndrome with DOK7 mutations ephedrine was reported to be beneficial in single patients. We carried out a small, open and prospective cohort study in eight European patients manifesting from birth to 12 years. Five patients showed limb-girdle and facial weakness, three a floppy infant syndrome with bulbar symptoms and/or respiratory distress. Ephedrine was started with 25 mg/day and slowly increased to 75-100 mg/day. Within weeks after starting therapy an improvement was observed in all patients and clinical follow-up disclosed positive effects more pronounced on proximal muscle weakness and strength using MRC scale. Effects on facial weakness were less pronounced. Vital capacity measurements and repetitive stimulation tests did not improve in the same way as clinical symptoms did. These investigations are appropriate to confirm the diagnosis in case of pathological results, but they might not be appropriate means to monitor patients under ephedrine therapy. PMID:19837590

Schara, U; Barisic, N; Deschauer, M; Lindberg, C; Straub, V; Strigl-Pill, N; Wendt, M; Abicht, A; Mller, J S; Lochmller, H

2009-12-01

94

Cost-effectiveness of cognitive behaviour therapy for patients with chronic fatigue syndrome  

Microsoft Academic Search

BACKGROUND: There is some evidence that cognitive behaviour therapy (CBT) is efficacious in chronic fatigue syndrome (CFS), but little data on its cost-effectiveness. DESIGN: Prospective economic analysis alongside a randomized clinical trial. METHODS: CFS patients were randomly assigned to CBT, guided support groups (SG), or the 'natural course' (NC, no protocol-based interventions). Patients were treated for 8 months and followed-up

J. L. Severens; J. B. Prins; G. J. van der Wilt; G. Bleijenberg

2004-01-01

95

Antithrombotic Therapy in Acute Coronary Syndrome: How Far Up the Coagulation Cascade Will We Go?  

Microsoft Academic Search

The contribution of thrombosis to the natural history and clinical expression of advanced atherosclerotic coronary artery\\u000a disease is well established. Less well understood is the biochemical and pathobiological distinction between normal hemostasis\\u000a and thrombosis as the proximate cause of acute coronary syndrome. In this article, we summarize an evolving area of interest\\u000a within the field of antithrombotic therapythe contact system

Becky Woodruff; Bruce Sullenger; Richard C. Becker

2010-01-01

96

Novel multidrug therapy for children with cyclosporine-resistant or -intolerant nephrotic syndrome  

Microsoft Academic Search

An effective treatment for children with refractory nephrotic syndrome (NS), especially in those with cyclosporine (CsA)-resistant\\u000a or CsA-intolerant NS, has yet to be established. Recently, the efficacy of multidrug therapy consisting of tacrolimus (Tac),\\u000a mycophenolate mofetil (MMF) in combination with prednisolone (PDN) in adult patients with refractory NS has been reported.\\u000a We successfully treated 14 consecutive children with refractory CsA-resistant

Tomomi Aizawa-Yashiro; Kazushi Tsuruga; Shojiro Watanabe; Eishin Oki; Etsuro Ito; Hiroshi Tanaka

2011-01-01

97

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

Microsoft Academic Search

The purpose of this study was to determine and compare the efficacy of kinesio tape and physical therapy modalities in patients\\u000a with shoulder impingement syndrome. Patients (n?=?55) were treated with kinesio tape (n?=?30) three times by intervals of 3days or a daily program of local modalities (n?=?25) for 2weeks. Response to treatment was evaluated with the Disability of Arm, Shoulder,

Erkan Kaya; Murat Zinnuroglu; Ilknur Tugcu

2011-01-01

98

[Midocalm in complex therapy of chronic low back pain syndrome].  

PubMed

The aim of the study was to determine whether application of midocalm is appropriate in patients with chronic low back pain (LBP) from the point of view of quality of life (QL), efficacy and tolerance. The subjects were 50 patients with chronic LBP associated with spinal osteochondrosis, who underwent clinical examination and were questioned using four QL questionnaires: Health Assessment Questionnaire (HAQ), Womac osteoarthritis index Womac osteoarthritis index, Oswestry Low Back Pain Disability Questionnaire, and The 36-Item Short-Form Health Survey (SF-36). The subjects were divided into two groups. The 25 patients of Group I were administered nise in a dose of 100 mg twice a day during 10 days, the 25 patients of Group II--nise in a dose of 100 mg twice a day plus midocalm in a dose of 150 mg per day during the first two days and 450 mg per day from the third day through the tenth day. The study showed high efficacy of midocalm in complex therapy of patients with chronic LBP, as well as low rate of adverse reactions and high treatment tolerance. QL of the patients improved. Combining midocalm therapy with nise allows quicker positive effect in patients with chronic LBP and lowers need for long application of non-steroid antiinflammatory drugs. PMID:16404939

Chernysheva, T V; Bagirova, G G

2005-01-01

99

Combination of Steven-Johnson syndrome and neuroleptic malignant syndrome following carbamazepine therapy: a rare occurrence.  

PubMed

Stevens-Johnson syndrome (SJS) is a severe, episodic, acute mucocutaneous reaction that is most often elicited by drugs and occasionally by infections. The drugs commonly implicated as the cause of SJS are anticonvulsants, sulfonamides, non-steroidal anti-inflammatory drugs and antibiotics. Carbamazepine (CBZ) has been commonly implicated in SJS. Neuroleptic malignant syndrome (NMS) is a rare, life-threatening but potentially treatable condition. Among the neuroleptics, haloperidol (parenteral) is implicated as a most common drug for NMS. Though rare, association of NMS with CBZ and association of NMS with toxic epidermal necrolysis (TEN) in a single patient after administration of neuroleptics has been reported in the literature before. However, a combination of NMS and SJS in a single patient after administration of CBZ has not been reported so far. We present a patient with seizure who developed SJS and NMS following administration of CBZ. PMID:23761563

Sharma, Bhawna; Sannegowda, Raghavendra Bakki; Gandhi, Pankaj; Dubey, Parul; Panagariya, Ashok

2013-01-01

100

Inositol: history of an effective therapy for Polycystic Ovary Syndrome.  

PubMed

Inositol is a physiological compound belonging to the sugar family. The two inositol stereoisomers, myo-inositol and D-chiroinositol are the two main stereisomers present in our body. Myo-inositol is the precursor of inositol triphosphate, a second messenger regulating many hormones such as TSH, FSH and insulin. D-chiroinositol is synthetized by an insulin dependent epimerase that converts myo-inositol into D-chiro-inositol. Polycistic Ovary Syndrome (PCOS) is a metabolic and hormonal disorder and a common cause of infertility. Insulin resistance and the consequent hyperinsulinaemia contribute to hyperandrogenism development, typical marker of PCOS. In these patients myo and/or D-chiro-inositol administration improves insulin sensivity while only myo-inositol is a quality marker for oocytes evaluation. Myo-inositol produces second messengers for FSH and glucose uptake, while D-chiroinositol provides second messengers promoting glucose uptake and glycogen synthesis. The physiological ratio of these two isomers is 40:1 (MI/DCI) and seems to be an optimal approach for the treatment of PCOS disorders. PMID:25010620

Bizzarri, M; Carlomagno, G

2014-07-01

101

Antiplatelet therapy in acute coronary syndromes: focus on ticagrelor  

PubMed Central

The use of antiplatelet agents, specifically the thienopyridines, has become a standard of care in the approach to the patient presenting with an acute coronary syndrome. These drugs irreversibly inhibit the platelet by permanently binding to the surface P2Y12 receptor and blocking the downstream fibrinogen cross-linking between platelets, which leads to aggregation and thrombus. However, currently available therapeutic choices are limited by potential interaction with other medications, slow hepatic conversion to active metabolite, genetic resistance, and narrow therapeutic safety margin. In order to overcome these disadvantages, there has been an interest in developing alternatives to thienopyridines. Recent investigations have included ticagrelor, a reversible inhibitor of the P2Y12 platelet receptor, which appears to have overcome several drawbacks of the current thienopyridines. Its unique pharmacokinetic and pharmacodynamic profiles result in an inhibition of platelet aggregation that is rapid, high, consistent, and less susceptible to interpatient variability than currently available P2Y12 inhibitors. In addition, ticagrelor offers a potential mortality advantage not apparent with current agents. Although questions regarding the nature, magnitude, and clinical significance of several observed adverse effects (dyspnea and ventricular pauses) remain unanswered, it appears that ticagrelor may represent a significant advancement over currently available oral antiplatelet agents. PMID:22282698

Birkeland, Kade; Parra, David; Rosenstein, Robert

2010-01-01

102

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

103

How Much? How Frequent? How Long? A Clinical Guide to New Therapies in Myelodysplastic Syndromes  

PubMed Central

Advances in the treatment of myelodysplastic syndromes (MDSs) over the last decade have given patients and their hematologists a multitude of treatment options. Therapeutic options now exist that reduce disease-related symptoms, improve quality of life, and alter the natural history of the disease. Three drugs are now specifically Food and Drug Administration-approved for treatment of MDS: (1) azacitidine, (2) decitabine, and (3) lenalidomide. Clinical results with each of these agents, plus results with immunosuppressive therapy, are reviewed to guide clinical decision making. Although each therapy has made a substantial impact in improving the care of patients with MDS, unfortunately MDS treatment in 2010 ultimately fails in most patients, but these therapies provide a foundation on which we can build to further improve outcomes. PMID:21239812

Blum, William

2011-01-01

104

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

105

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

106

Additive effects of low-level laser therapy with exercise on subacromial syndrome: a randomised, double-blind, controlled trial  

Microsoft Academic Search

The subacromial syndrome is the most common source of shoulder pain. The mainstays of conservative treatment are non-steroidal\\u000a anti-inflammatory drugs and exercise therapy. Recently, low-level laser therapy (LLLT) has been popularized in the treatment\\u000a of various musculoskeletal disorders. The aim of this study is to evaluate the additive effects of LLLT with exercise in comparison\\u000a with exercise therapy alone in

Seyyed Mohammad Jalil Abrisham; Mohammad Kermani-Alghoraishi; Rahil Ghahramani; Latife Jabbari; Hossein Jomeh; Maryam Zare

107

The role of nutritional therapy in the treatment of equine Cushing's syndrome and laminitis.  

PubMed

Equine Cushing's syndrome, a relatively common and complex condition, is difficult to treat with conventional medicine. Cushing's syndrome involves a hyperplasia or adenoma of the anterior pituitary gland. Biochemical alterations include increased endogenous cortisol, insulin resistance, elevated adrenocorticotrophic hormone, and decreased thyroid hormone levels. Symptoms include hirsutism with no loss of the winter coat in summer, refractory laminitis, weight problems (over- or underweight), polyuria/polydipsia (Pu/Pd), frequent infections, lowered immunity to intestinal parasites, decreased intestinal wall integrity, and infertility. Laminitis (an inflammation of the laminae of the foot) is a common and often fatal complication of Cushing's syndrome that tends to be refractory to conventional treatment. One of the most common therapies is phenylbutazone, a non-steroidal anti-inflammatory drug (NSAID) known to cause significant changes in the permeability of the intestinal wall. Recent research has shown an intestinal bacterial exotoxin to be one of the triggering factors in laminitis. By removing phenylbutazone and healing the intestinal wall, laminitis becomes more responsive to treatment. Good hoof-care combined with nutritional management and the application of other modalities, including acupuncture and Chinese and Western herbs, can complete the healing process. The successful treatment of equine Cushing's syndrome is one of the best examples of treating a disease using the holistic approach. While each case requires different combinations of modalities, the outcome is usually positive with individually selected treatments. PMID:11591169

Harman, J; Ward, M

2001-09-01

108

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. Gttingen 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 (257 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

109

Oral antiplatelet therapy for the management of acute coronary syndromes: defining the role of prasugrel.  

PubMed

Antiplatelet therapy is important in the prevention of thrombosis, a significant cause of morbidity and mortality that is associated with acute coronary syndrome. Current evidence supports the general acceptance of aspirin and clopidogrel as the standard of care for the management of all patients experiencing an acute coronary syndrome with or without percutaneous coronary intervention. Thienopyridine antiplatelet agents such as clopidogrel, when used alone and in combination with aspirin, are effective in preventing myocardial infarction and death. Prasugrel, a potent antiplatelet agent that was recently approved by the Food and Drug Administration, may have some advantages over other antiplatelet agents, owing to its rapid onset of action, higher degree of platelet inhibition, and decreased interpatient variability in pharmacokinetics and pharmacodynamics as compared with clopidogrel. However, further studies are required to assess the potential for increased bleeding in high-risk patients. PMID:20592188

Devabhakthuni, Sandeep; Seybert, Amy L

2011-02-01

110

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 4872 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

111

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.

Kuo, Elizabeth

2014-01-01

112

Effects of Temperature on Chronic Trapezius Myofascial Pain Syndrome during Dry Needling Therapy  

PubMed Central

The purpose of this study was to investigate the effects of temperature on chronic trapezius myofascial pain syndrome during dry needling therapy. Sixty patients were randomized into two groups of dry needling (DN) alone (group A) and DN combined with heat therapy group (group B). Each patient was treated once and the therapeutic effect was assessed by the visual analogue scale (VAS), pressure pain threshold (PPT), and the 36-item short form health survey (SF-36) at seven days, one month, and three months after treatment. Evaluation based on VAS and PPT showed that the pain of patients in groups A and B was significantly (P < 0.05) relieved at seven days, one month, and three months after treatment Compared to before treatment. There was significantly (P < 0.05) less pain in group B than group A at one and three months after treatment. The SF-36 evaluation demonstrated that the physical condition of patients in both groups showed significant (P < 0.05) improvement at one month and three months after treatment than before treatment. Our study suggests that both DN and DN heating therapy were effective in the treatment of trapezius MPS, and that DN heating therapy had better long-term effects than DN therapy. PMID:25383083

2014-01-01

113

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

114

Cauda equina syndrome associated with multiple lumbar arachnoid cysts in ankylosing spondylitis: improvement following surgical therapy.  

PubMed Central

A case of cauda equina syndrome with multiple lumbar arachnoid cysts complicating ankylosing spondylitis (AS) is described. The value of computerised tomography (CT) and magnetic resonance imaging (MRI) as a non-invasive means of establishing the diagnosis is emphasised. In contrast to previously reported cases the patient showed neurological improvement following surgical therapy. Surgery may be indicated in some patients, particularly when there is nerve root compression by the arachnoid cysts and when the patient is seen early before irreversible damage to the cauda equina has occurred. Images PMID:2292702

Shaw, P J; Allcutt, D A; Bates, D; Crawford, P J

1990-01-01

115

Fulminant hepatic failure in nephrotic syndrome related to withdrawal of immunosuppressive therapy.  

PubMed Central

Two patients with nephrotic syndrome developed fatal fulminant hepatitis B following withdrawal of prednisolone or cyclophosphamide. Immunosuppressive therapy probably enhanced hepatitis B virus (HBV) replication and widespread infection of hepatocytes; its withdrawal permitted a return of immune competence resulting in massive destruction of infected hepatocytes. Prior screening of all patients for hepatitis B surface antigen, gradual withdrawal of immunosuppressive drugs with careful monitoring, and prompt intervention with corticosteroids at the first clinical or biochemical signs of liver cell damage may prevent this complication. PMID:3186580

Onwubalili, J. K.

1988-01-01

116

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

117

Simultaneous intrahepatic and subgaleal hemorrhage in antiphospholipid syndrome following anticoagulation therapy  

PubMed Central

Warfarin is a widely used anticoagulant. Interindividual differences in drug response, a narrow therapeutic range and the risk of bleeding render warfarin difficult to use clinically. An 18-year-old woman with antiphospholipid syndrome received long-term warfarin therapy for a recurrent deep vein thrombosis. Six years later, she developed right flank pain. We diagnosed intrahepatic and subgaleal hemorrhages secondary to anticoagulation therapy. After stopping oral anticoagulation, a follow-up computed tomography showed improvement in the hemorrhage. After restarting warfarin because of a recurrent thrombosis, the intrahepatic hemorrhage recurred. We decided to start clopidogrel and hydroxychloroquine instead of warfarin. The patient has not developed further recurrent thrombotic or bleeding episodes. Intrahepatic hemorrhage is a very rare complication of warfarin, and our patient experienced intrahepatic and subgaleal hemorrhage although she did not have any risk factors for bleeding or instability of the international normalized ratio control. PMID:24151371

Park, In-Chul; Baek, Yang-Hyun; Han, Sang-Young; Lee, Sung-Wook; Chung, Won-Tae; Lee, Sung-Won; Kang, Sang-Hyeon; Cho, Duk-Song

2013-01-01

118

Simultaneous intrahepatic and subgaleal hemorrhage in antiphospholipid syndrome following anticoagulation therapy.  

PubMed

Warfarin is a widely used anticoagulant. Interindividual differences in drug response, a narrow therapeutic range and the risk of bleeding render warfarin difficult to use clinically. An 18-year-old woman with antiphospholipid syndrome received long-term warfarin therapy for a recurrent deep vein thrombosis. Six years later, she developed right flank pain. We diagnosed intrahepatic and subgaleal hemorrhages secondary to anticoagulation therapy. After stopping oral anticoagulation, a follow-up computed tomography showed improvement in the hemorrhage. After restarting warfarin because of a recurrent thrombosis, the intrahepatic hemorrhage recurred. We decided to start clopidogrel and hydroxychloroquine instead of warfarin. The patient has not developed further recurrent thrombotic or bleeding episodes. Intrahepatic hemorrhage is a very rare complication of warfarin, and our patient experienced intrahepatic and subgaleal hemorrhage although she did not have any risk factors for bleeding or instability of the international normalized ratio control. PMID:24151371

Park, In-Chul; Baek, Yang-Hyun; Han, Sang-Young; Lee, Sung-Wook; Chung, Won-Tae; Lee, Sung-Won; Kang, Sang-Hyeon; Cho, Duk-Song

2013-10-14

119

Immobilization hypercalcemia associated with Landry-Guillain-Barr syndrome. Successful therapy with combined calcitonin and etidronate.  

PubMed

Two patients with immobilization hypercalcemia associated with Landry-Guillain-Barr syndrome had marked hypercalciuria (890 and 1136 mg/d [22.2 and 28.3 mmol/d]) and radiologic evidence of generalized osteopenia. Parathyroid hormone levels were either low or normal by C-terminal radioimmunoassay. Subtotal parathyroidectomy was performed in the one patient, with no improvement in serum or urinary calcium levels. A bone biopsy specimen revealed decreased cellular activity in the first patient and increased bone resorption in the second patient. Treatment with intravenous saline, furosemide, oral phosphate supplementation, mithramycin, and calcitonin alone was ineffective in lowering serum or urinary calcium levels. However, when subcutaneous calcitonin combined with oral etidronate disodium was used, a reduction in the serum calcium level was observed within two days of therapy. Within one week of the start of this combined therapy, the calcium level returned to normal and urinary calcium excretion was substantially reduced. PMID:3089187

Meythaler, J M; Korkor, A B; Nanda, T; Kumar, N A; Fallon, M

1986-08-01

120

Iatrogenic Cushing's syndrome and topical steroid therapy: case series and review of the literature.  

PubMed

Topical corticosteroids are considered first-line therapy in patients with chronic inflammatory oral mucosal diseases; among them, clobetasol propionate is one of the most widely used in oral medicine. Under physiological conditions, the transmucosal application is characterized by a significantly greater absorption than the skin application. Contrary to many publications about the side effects of topical corticosteroids in dermatology, few studies have investigated the systemic effects due to local application of these drugs on oral mucosa. Although topical steroid therapy for the management of oral diseases is generally associated with local adverse effects (candidiasis, stomatopyrosis, and hypogeusia), these drugs can also lead to systemic side effects, such as suppression of the hypothalamic-pituitary-adrenal axis and Cushing's syndrome. This review reports five cases of systemic adverse effects caused by clobetasol propionate topical treatment. PMID:23210698

Decani, Sem; Federighi, Veronica; Baruzzi, Elisa; Sardella, Andrea; Lodi, Giovanni

2014-12-01

121

Bronchiolitis obliterans organising pneumonia syndrome presenting with neutrophilia in bronchoalveolar lavage fluid after breast-conserving therapy  

PubMed Central

A 61-year-old female presented with a dry cough and fever 4 months after tangential radiation therapy (RT) following conserving surgery for breast cancer. Chest radiography and CT demonstrated consolidation with air bronchogram outside the irradiated area. Neutrophil granulocytes were abundant in bronchoalveolar lavage fluid (BALF) (39.6% of total cells), and transbronchial lung biopsy revealed organising pneumonia (OP) histologically. Antibiotic therapy had no effect, but corticosteroid therapy brought about clinical improvement. Her condition was diagnosed as bronchiolitis obliterans OP (BOOP) syndrome. Lymphocytic BALF has been identified as a characteristic of BOOP syndrome induced after RT for breast cancer. The BALF in this case, however, was neutrophilic. In our analysis of differential cell counts in the BALF of 24 patients with BOOP syndrome, the BALF was neutrophilic (>5%) in 16 (76%) cases, and the neutrophilia was severe in some of those patients. PMID:22605699

Chiba, Sahoko; Jinta, Torahiko; Chohnabayashi, Naohiko; Fujie, Toshihide; Sumi, Yuki; Inase, Naohiko

2012-01-01

122

Bronchiolitis obliterans organising pneumonia syndrome presenting with neutrophilia in bronchoalveolar lavage fluid after breast-conserving therapy.  

PubMed

A 61-year-old female presented with a dry cough and fever 4 months after tangential radiation therapy (RT) following conserving surgery for breast cancer. Chest radiography and CT demonstrated consolidation with air bronchogram outside the irradiated area. Neutrophil granulocytes were abundant in bronchoalveolar lavage fluid (BALF) (39.6% of total cells), and transbronchial lung biopsy revealed organising pneumonia (OP) histologically. Antibiotic therapy had no effect, but corticosteroid therapy brought about clinical improvement. Her condition was diagnosed as bronchiolitis obliterans OP (BOOP) syndrome. Lymphocytic BALF has been identified as a characteristic of BOOP syndrome induced after RT for breast cancer. The BALF in this case, however, was neutrophilic. In our analysis of differential cell counts in the BALF of 24 patients with BOOP syndrome, the BALF was neutrophilic (>5%) in 16 (76%) cases, and the neutrophilia was severe in some of those patients. PMID:22605699

Chiba, Sahoko; Jinta, Torahiko; Chohnabayashi, Naohiko; Fujie, Toshihide; Sumi, Yuki; Inase, Naohiko

2012-01-01

123

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

124

A case of multiple evanescent white dot syndrome treated by steroid pulse therapy.  

PubMed

Multiple evanescent white dot syndrome (MEWDS) is characterized by acute visual loss which is usually spontaneously restored after a few months. While occasional treatments with local or oral steroids have been reported, these are hardly effective. A 25-year-old man, a Sumo wrestler, was diagnosed with MEWDS, and as he wanted a quick recovery to compete in an upcoming tournament treatment with steroid pulse therapy was initiated 8 days after the symptoms appeared. Before treatment, visual acuity in his left eye was 20/400. Multiple white dots were seen at the level of the deep retina or retinal pigment epithelium, and the Mariotte's blind spot extended to the center of the visual field. Immediately after the end of steroid pulse therapy, left visual acuity increased to 20/25, and left visual field recovered remarkably. No white dots were seen funduscopically. Steroid pulse therapy might provide early improvement of visual functions, and we believe it could be a treatment option for initiating an early recovery from MEWDS. However, steroid pulse therapy may also result in lethal damage including disorders of the circulatory organs. Thus, it should only be applied in limited situations in which patients had rapid decline in visual function and needs for social return at an early stage like that of our patient. PMID:17330396

Takahashi, Yasuhiro; Ataka, Shinsuke; Wada, Sonomi; Kohno, Takeya; Nomura, Yuya; Shiraki, Kunihiko

2006-12-01

125

Exercise therapy and cognitive behavioural therapy to improve fatigue, daily activity performance and quality of life in Postpoliomyelitis Syndrome: the protocol of the FACTS2PPS trial  

Microsoft Academic Search

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

Fieke S Koopman; Anita Beelen; Karin H Gerrits; Gijs Bleijenberg; Tineke A Abma; Marianne de Visser; Frans Nollet

2010-01-01

126

Exacerbation of BMI after cessation of growth hormone therapy in patients with Prader-Willi syndrome.  

PubMed

Long-term treatment with growth hormone (GH) in patients with Prader-Willi syndrome (PWS) improves not only height velocity, height standard deviation score, and final height, but also the degree of obesity and body composition abnormalities. Anecdotally, PWS patients tend to suffer from severe obesity and its complications after cessation of GH therapy. However, there have been no studies to investigate changes in body mass index (BMI) and adipose tissue distribution after cessation of GH therapy in young PWS patients. Therefore, we investigated changes in the BMI-standard deviation score (SDS) and adipose tissue distribution after cessation of GH therapy in PWS patients. We evaluated 14 PWS patients. BMI-SDS was calculated at 0, 6, 12, 18, and 24 months before and after cessation of GH treatment. We also evaluated subcutaneous adipose tissue (SAT) (cm(2)) and visceral adipose tissue (VAT) (cm(2)) area in 8 of the 14 study patients with single slice abdominal computed tomography at the level of the umbilicus. The BMI-SDS significantly increased at 6, 12, 18, and 24 months after cessation of GH therapy (P = 0.039, P = 0.008, P = 0.003, P = 0.003, respectively). There was a tendency toward increases in VAT at 12 and 24 months after cessation of GH therapy, but the increases did not reach statistical significance (P = 0.062, P = 0.125, respectively). Therefore, cessation of GH therapy in PWS patients worsened BMI. To maintain good body composition and prevent complications of obesity, long-term use of GH in adult PWS patients may be advisable. PMID:24443368

Oto, Yuji; Tanaka, Yuriko; Abe, Yoshiko; Obata, Kazuo; Tsuchiya, Takayoshi; Yoshino, Atsunori; Murakami, Nobuyuki; Nagai, Toshiro

2014-03-01

127

Choroidal thickness changes with photodynamic therapy for a diffuse choroidal hemangioma in SturgeWeber syndrome.  

PubMed

The aim of this study was to evaluate the choroidal thickness (CT) changes associated with visual function following photodynamic therapy (PDT) for a diffuse choroidal hemangioma in SturgeWeber syndrome. We report a case of SturgeWeber syndrome and symptomatic serous retinal detachment (SRD) with diffuse choroidal hemangioma treated with PDT. Visual acuity (VA), macular sensitivity measured by means of MP1 microperimeter (Nidek Technologies, Padova, Italy), retinal and CT, measured by means of enhanced depth optical coherence tomography (EDIOCT, Spectralis, Heidelberg Engineering, Heidelberg, Germany) were analyzed at baseline, 3 and 12 months follow-up.After the PDT VA and macular sensitivity improved.The OCT examination showed the resolution of SRD. The choroid was measured after PDT using EDIOCT. At baseline, the subfoveal CT showed a progressive thickness reduction from 251 to 83 lm during follow-up. To our knowledge, this is the first report of CT changes after PDT for a diffuse choroidal hemangioma in SturgeWeber syndrome in a longterm follow-up. The CT measurement represents a potential parameter to better follow choroidal hemangiomas and their response to treatment. However,the long-term choroidal changes should be carefully taken into account. PMID:24658736

Cacciamani, Andrea; Scarinci, Fabio; Parravano, Mariacristina; Giorno, Paola; Varano, Monica

2014-10-01

128

Perioperative Endocrine Therapy for Patients with Cushing's Syndrome Undergoing Retroperitoneal Laparoscopic Adrenalectomy  

PubMed Central

Objectives. To investigate the efficacy and safety of perioperative endocrine therapy (PET) for patients with Cushing's syndrome (CS) undergoing retroperitoneal laparoscopic adrenalectomy (RLA). Methods. The novel, simplified PET modality of 82 patients who underwent RLA procedures for CS were studied. Clinical manifestations were observed for all patients on days 1 and 5 postoperatively, and clinical data, such as blood pressure (BP), levels of serum cortisol, adrenocorticotropin (ACTH), blood glucose, and electrolytes, were acquired and analyzed. Results. Supraphysiological doses of glucocorticoid were administered during the perioperative period, and the dosage was reduced gradually. In all 82 cases, the RLAs were performed successfully without any perioperative complication, such as steroid withdrawal symptoms. The patient's symptoms and signs were improved quickly and safely during the hospital days. The serum cortisol and potassium levels were rather stable on days 1 and 5 postoperatively, and most were within the normal range. The clinical manifestations, serum levels of cortisol, ACTH, and potassium in most patients restored to normal gradually after several months (mean, 6.7 1.2 months), except for one patient undergoing bilateral adrenalectomy. Conclusions. This perioperative endocrine therapy for patients with Cushing's syndrome (mainly for adrenocortical adenoma) undergoing retro-laparoscopic adrenalectomy is both effective and safe. PMID:23193401

Cui, Xiaobo; Yang, Lu; Li, Jianwei; Bu, Siyuan; Wei, Qiang; An, Zhenmei; Fan, Tianyong

2012-01-01

129

[Clinical description of effectiveness of pioglitazone in a complex therapy of patients with ischemic heart disease on background of metabolic syndrome].  

PubMed

The results suggest that addition of pioglitazone in a complex treatment therapy of patients with ischemic heart disease on background of metabolic syndrome was not associated with an increased ischemic heart disease (IHD) risk and improved the clinical course of IHD, increased effectiveness of standard therapy for patients with IHD and metabolic syndrome. PMID:21954640

Vinnik, N I; Ka?dashev, I P

2011-01-01

130

Polymorphic variants of MIF gene and prognosis in steroid therapy in children with idiopathic nephrotic syndrome.  

PubMed

Nephrotic syndrome (NS) is the most common reason of proteinuria in children and can be caused by the pathology of renal glomeruli. Steroid therapy is typically used in this disorder. It has been shown that MIF is a cytokine which counteracts the immunosuppressive properties of glucocorticoids. The aim of this study was looking for a correlation between MIF polymorphisms and genetic susceptibility to steroid resistance in children with INS (Idiopathic NS). Methods: The study was performed in 71 patients with INS including SRNS (steroid resistance nephrotic syndrome) (41) and SSNS (steroid sensitive nephrotic syndrome) (30) and in 30 control subjects. We employed Sanger sequencing and capillary electrophoresis. Linkage disequilibrium was made using Haploview and PHASE. Results: We didn't observe a statistical significance between SNPs detected in patients with INS and controls. Our studies revealed statistical significance for two polymorphisms: rs2070767C > T and rs2000466T > G between patients with SRNS and SSNS. The results for rs34383331T > A are close to being statistically significant. Statistical significance was revealed for CATT5/CATT6 genotype in SRNS group vs SSNS group (OR=4.604, 95%CI=1.356-15.632, p=0.0168). We found that the frequency of 5/X-CATT genotype compared with X/X-CATT genotype was significantly higher in SRNS patients vs SSNS (OR=3.167, 95%CI=1.046-9.585, p=0.0426). In linkage disequilibrium analysis we didn't show involvement in susceptibility to INS and steroid sensitive phenotype. Conclusions: Our results suggest that the role of MIF polymorphisms in the susceptibility to positive response to steroid therapy is still unresolved. It indicates that MIF may be involved in indirect and complex molecular mechanisms of steroid activity in hormone-dependent metabolic pathways in children with INS. Because of ambiguous findings, pleiotropic features of this cytokine require that more research should be undertaken. PMID:24644543

?wierczewska, Monika; Ostalska-Nowicka, Danuta; Kempisty, Bartosz; Szczepankiewicz, Aleksandra; Nowicki, Micha?

2014-01-01

131

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

132

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

133

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 3days or a daily program of local modalities (n?=?25) for 2weeks. 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

134

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

Mberra Kocak; Eray Caliskan; Coskun Simsir; Ali Haberal

2002-01-01

135

[Antiplatelet therapy in acute coronary syndrome : Prehospital phase: nothing, aspirin or what?].  

PubMed

In most cases of ST segment elevation myocardial infarction (STEMI) a major coronary vessel is occluded by a thrombus. This is why early and effective antiplatelet therapy plays a key role. The current guidelines recommend the administration of dual antiplatelet therapy as early as possible. Despite the lack of convincing clinical evidence, prehospital administration appears reasonable, primarily because of pharmacokinetic considerations. Ticagrelor should be preferentially administered because the largest amount of evidence is available and it appears to be safe. In high-risk patients undergoing transfer to a catheterization laboratory, upstream use of a glycoprotein (GP) IIb/IIIa receptor antagonist (tirofiban) may be considered. Acute coronary syndrome without ST segment elevation (NSTE-ACS) represents a clinically heterogeneous group. Current guidelines recommend that antiplatelet therapy should be initiated as early as possible when the diagnosis of NSTE-ACS is made. If there is high clinical suspicion of NSTE-ACS acetylsalicylic acid (ASA) should be given before hospital admission. In high-risk patients prehospital administration of ticagrelor may be considered. PMID:25315248

Bauer, T; Hamm, C

2014-11-01

136

Pompholyx of the hands after intravenous immunoglobulin therapy for clinically isolated syndrome: a paediatric case.  

PubMed

Pompholyx is a common eruption of small vesicles on the palms, soles, and/or lateral aspects of the fingers. It has a multifactorial etiology, including genetic determinants, allergy to metals, and id reaction; rarely it is a drug-related side effect. We report a paediatric case of pompholyx of the hands related to the intravenous immunoglobulin (IVIG) therapy for Clinically Isolated Syndrome (CIS). A 10-year-old boy, received an IVIG therapy (Venital, Kedrion Spa, Italy) at a dose of 400 mg/kg daily for five days. The fifth day of IVIG infusion, a symmetrical vesicular eruption appeared on the palms of the hands and on lateral aspects of the fingers. The lesions improved with application of topical steroids in few days. The mechanism of induction of pompholyx by IVIG therapy is unknown. A review of the Literature suggests the hypothesis that dyshidrotic eczematous reactions may be related not only to the type of IVIG, to the dose and the rates of infusion, but also to an allergic response to excipients and preservatives contained in the drug, probably elicited by an underlying neurological disease in some cases. PMID:24674688

Brazzelli, V; Grassi, S; Savasta, S; Ruffinazzi, G; Carugno, A; Barbaccia, V; Marseglia, G L; Borroni, G

2014-01-01

137

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

138

Iatrogenic Cushing syndrome after epidural triamcinolone injections in an HIV type 1-infected patient receiving therapy with ritonavir-lopinavir.  

PubMed

We report the first case of a human immunodeficiency virus type 1 (HIV-1)-infected individual receiving combination antiretroviral therapy, which included ritonavir, who developed Cushing syndrome with profound complications after epidural triamcinolone injections. This case highlights the potential of ritonavir interactions even with local injections of a corticosteroid. PMID:18991509

Ramanathan, Roshan; Pau, Alice K; Busse, Kristin H; Zemskova, Marina; Nieman, Lynnette; Kwan, Richard; Hammer, Jean H; Mican, JoAnn M; Maldarelli, Frank

2008-12-15

139

How does cognitive behaviour therapy reduce fatigue in patients with chronic fatigue syndrome? The role of physical activity  

Microsoft Academic Search

BACKGROUND: Cognitive behaviour therapy (CBT) is known to reduce fatigue severity in chronic fatigue syndrome (CFS). How this change in symptomatology is accomplished is not yet understood. The purpose of the present study was to determine whether the effect of CBT on fatigue is mediated by an increase in physical activity. METHOD: Three randomized controlled trials were reanalysed, previously conducted

J. F. Wiborg; H. Knoop; M. Stulemeijer; J. B. Prins; G. Bleijenberg

2010-01-01

140

Immune reconstitution inflammatory syndrome in an HIV/TB co-infected patient four years after starting antiretroviral therapy.  

PubMed

The Immune reconstitution inflammatory syndrome (IRIS) after starting antiretroviral therapy for HIV is well known. We describe an HIV seropositive woman, presenting 2 IRIS episodes associated with Mycobacterium tuberculosis. Exceptional was that the last episode occurred 4 years after initiating antiretroviral treatment, when her CD4+ lymphocyte count had been around 300 cells/mm3 for one year. PMID:17547295

Huyst, V; Lynen, L; Bottieau, E; Zolfo, M; Kestens, L; Colebunders, R

2007-01-01

141

The effect of cognitive behaviour therapy for chronic fatigue syndrome on self-reported cognitive impairments and neuropsychological test performance  

Microsoft Academic Search

Background: Patients with chronic fatigue syndrome (CFS) often have concentration and memory problems. Neuropsychological test performance is impaired in at least a subgroup of patients with CFS. Cognitive behavioural therapy (CBT) for CFS leads to a reduction in fatigue and disabilities.Aim: To test the hypothesis that CBT results in a reduction of self-reported cognitive impairment and in an improved neuropsychological

Hans Knoop; Judith B Prins; Maja Stulemeijer; Jos W M van der Meer; Gijs Bleijenberg

2007-01-01

142

Decision-Making, Reward-Seeking Behaviors and Dopamine Agonist Therapy in Restless Legs Syndrome  

PubMed Central

Study Objectives: To assess whether the frequency of impulse control disorders (ICDs), addictive behaviors, impulsivity, and impairment of decision-making task performance under ambiguous and risky conditions were present in patients with restless legs syndrome (RLS) and whether changes could be related to dopaminergic medications. Design: Case-control prospective study. Setting: Academic Sleep Disorders Center. Participants: Of the 149 participants, there were 39 who were drug free with primary RLS, 50 who were taking dopamine agonists (DA), and 60 control subjects. Participants were assessed with a clinical interview screening for ICDs, augmentation syndrome, impulsivity, depression, and addictive behaviors. All participants completed two decision-making tasks, one under an ambiguous condition (Iowa Gambling Task) and the other under a risky condition (Game of Dice Task). Drug-free patients with RLS underwent 1 night of polysomnography recording. Measurements and Results: Seventy percent of patients were treated with pramipexole (median dose, 0.36 mg), and 30% with ropinirole (median dose, 0.75 mg). Median duration of DA intake was 11 mo (range, 1-72 mo). No differences were found on impulsivity scores, ICDs, and substance addiction between drug-free patients or those taking DA, or control subjects. Patients with RLS reported more depressive symptoms than control subjects, but without differences between patients taking or not taking DA. Drug-free and treated patients demonstrated reduced performances on the Iowa Gambling Task but not on the Game of Dice Task compared to control subjects, with no differences between patients taking medications and those who were not. No association was found between decision-making task performances, or polysomnographic and clinical variables. Conclusion: Impulse control disorders, impulsivity, and substance addiction were infrequent in drug-free patients with restless legs syndrome or those treated with a low dose of dopamine agonists. However, patients with restless legs syndrome, either drug free or taking dopamine agonists, had preferences toward risky choices on the Iowa Gambling Task, which led to negative consequences in the long run, a condition potentially leading to further development of impulse control disorders. Citation: Bayard S; Langenier MC; Dauvilliers Y. Decision-making, reward-seeking behaviors and dopamine agonist therapy in restless legs syndrome. SLEEP 2013;36(10):1501-1507. PMID:24082309

Bayard, Sophie; Langenier, Muriel Croisier; Dauvilliers, Yves

2013-01-01

143

[Therapy strategies for acute coronary syndrome and after coronary interventions. Antiplatelet agents and anticoagulants].  

PubMed

There is ongoing development of new therapeutic regimens in the use of antithrombotic agents and anticoagulants focussing on acute coronary syndrome (ACS) with an increasing impact on current guidelines over the last years. This was especially accompanied by an increase in innovative percutaneous coronary interventional (PCI) methods in patients with ACS, non-ST-segment elevation myocardial infarction (NSTEMI) or ST-segment elevation myocardial infarction (STEMI) with a need for therapeutics with more sufficient and effective antiplatelet action. On the other hand, newer direct and indirect thrombin inhibitors with primary use in prevention and therapy of thromboembolic events have been shown to have beneficial and even superior effects in ACS with or without PCI. The current review aims to report on the evidence-based use of approved antithrombotic agents and anticoagulants in ACS with special focus on PCI according to the actualized European guidelines. PMID:21922225

Divchev, D; Nienaber, C; Ince, H

2011-11-01

144

Synthesis of ganglioside epitopes for oligosaccharide specific immunoadsorption therapy of Guillian-Barr syndrome.  

PubMed

Guillain-Barr syndrome is a postinfectious, autoimmune neuropathy resulting in neuromuscular paralysis. Auto-antibodies, often induced by bacterial infection, bind to human gangliosides possessing monosialoside and diasialoside epitopes and impair the function of nerve junctions, where these ganglioside structures are highly enriched. Truncated gangliosides representive of GD3, GQ1b and GM2 epitopes have been synthesized as methyl glycosides and as a glycosides of an eleven carbon tether. The synthetic oligosaccharide ligands are structural mimics of these highly complex ganglioside epitopes and via their ability to neutralize or remove auto-antibodies have the potential for therapy, either as soluble blocking ligands administered systemically, or as immuno-affinity ligands for use as extracorporeal immunoadsorbents. PMID:15064799

Andersen, Sren M; Ling, Chang-Chun; Zhang, Ping; Townson, Kate; Willison, Hugh J; Bundle, David R

2004-04-21

145

An update on the pathomechanisms and future therapies of Alport syndrome.  

PubMed

Alport Syndrome (AS) is an inherited progressive disease that is caused by mutations of the genes encoding the key collagen chains, ?3, ?4, and ?5, which are necessary for the composition of collagen type IV to form a robust glomerular basement membrane (GBM), capable of withstanding the significant biomechanical strain to which the glomerulus is subjected. Progressive loss of the filtration barrier allows excessive proteinuria, which ultimately leads to end-stage kidney disease (ESKD). The evidence for a beneficial renoprotective effect of renin-angiotensin-aldosterone system (RAAS) blockade by angiotensin-converting enzyme (ACE) inhibition and/or angiotensin receptor blockers (ARBs) is well established in AS and recent evidence has shown that it can significantly delay the time to onset of renal replacement therapy and ESKD. Future potential treatments of AS disease progression are evaluated in this review. PMID:22903660

Noone, Damien; Licht, Christoph

2013-07-01

146

MECP2 Isoform-Specific Vectors with Regulated Expression for Rett Syndrome Gene Therapy  

PubMed Central

Background Rett Syndrome (RTT) is an Autism Spectrum Disorder and the leading cause of mental retardation in females. RTT is caused by mutations in the Methyl CpG-Binding Protein-2 (MECP2) gene and has no treatment. Our objective is to develop viral vectors for MECP2 gene transfer into Neural Stem Cells (NSC) and neurons suitable for gene therapy of Rett Syndrome. Methodology/Principal Findings We generated self-inactivating (SIN) retroviral vectors with the ubiquitous EF1? promoter avoiding known silencer elements to escape stem-cell-specific viral silencing. High efficiency NSC infection resulted in long-term EGFP expression in transduced NSC and after differentiation into neurons. Infection with Myc-tagged MECP2-isoform-specific (E1 and E2) vectors directed MeCP2 to heterochromatin of transduced NSC and neurons. In contrast, vectors with an internal mouse Mecp2 promoter (MeP) directed restricted expression only in neurons and glia and not NSC, recapitulating the endogenous expression pattern required to avoid detrimental consequences of MECP2 ectopic expression. In differentiated NSC from adult heterozygous Mecp2tm1.1Bird+/? female mice, 48% of neurons expressed endogenous MeCP2 due to random inactivation of the X-linked Mecp2 gene. Retroviral MECP2 transduction with EF1? and MeP vectors rescued expression in 95100% of neurons resulting in increased dendrite branching function in vitro. Insulated MECP2 isoform-specific lentiviral vectors show long-term expression in NSC and their differentiated neuronal progeny, and directly infect dissociated murine cortical neurons with high efficiency. Conclusions/Significance MeP vectors recapitulate the endogenous expression pattern of MeCP2 in neurons and glia. They have utility to study MeCP2 isoform-specific functions in vitro, and are effective gene therapy vectors for rescuing dendritic maturation of neurons in an ex vivo model of RTT. PMID:19710912

Rastegar, Mojgan; Hotta, Akitsu; Pasceri, Peter; Makarem, Maisam; Cheung, Aaron Y. L.; Elliott, Shauna; Park, Katya J.; Adachi, Megumi; Jones, Frederick S.; Clarke, Ian D.; Dirks, Peter; Ellis, James

2009-01-01

147

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

148

Etude comparative de rcepteurs aux strognes : Aspects molculaire et cellulaire de la rponse aux strognes et anti-strognes impliqus dans les causes et thrapies du cancer du sein.  

E-print Network

??Les strognes (E2), via le rcepteur aux strognes (ER), contrlent l'expression de nombreux gnes impliqus dans la croissance, la diffrenciation cellulaire et les fonctions reproductrices. (more)

Le Grand, Adlade

2009-01-01

149

Correction of Murine Bernard-Soulier Syndrome by Lentivirus-mediated Gene Therapy  

PubMed Central

BernardSoulier 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

2012-01-01

150

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 14days of diloxanide furoate 500mg thrice daily, trimethoprim/sulfamethoxazole 160/80mg twice daily and secnidazole 400mg thrice daily. Faecal specimens were collected at baseline, day 15 and 4weeks 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 (MannWhitney, 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

151

Which place for stem cell therapy in the treatment of acute radiation syndrome?  

PubMed

Radiation-induced (RI) tissue injuries can be caused by radiation therapy, nuclear accidents or radiological terrorism. Notwithstanding the complexity of RI pathophysiology, there are some effective approaches to treatment of both acute and chronic radiation damages. Cytokine therapy is the main strategy capable of preventing or reducing the acute radiation syndrome (ARS), and hematopoietic growth factors (GF) are particularly effective in mitigating bone marrow (BM) aplasia and stimulating hematopoietic recovery. However, first, as a consequence of RI stem and progenitor cell death, use of cytokines should be restricted to a range of intermediate radiation doses (3 to 7 Gy total body irradiation). Second, ARS is a global illness that requires treatment of damages to other tissues (epithelial, endothelial, glial, etc.), which could be achieved using pleiotropic or tissue-specific cytokines. Stem cell therapy (SCT) is a promising approach developed in the laboratory that could expand the ability to treat severe radiation injuries. Allogeneic hematopoietic stem cell transplantation (BM, mobilized peripheral blood and cord blood) transplantation has been used in radiation casualties with variable success due to limiting toxicity related to the degree of graft histocompatibility and combined injuries. Ex vivo expansion should be used to augment cord blood graft size and/or promote very immature stem cells. Autologous SCT might also be applied to radiation casualties from residual hematopoietic stem and progenitor cells (HSPC). Stem cell plasticity of different tissues such as liver or skeletal muscle, may also be used as a source of hematopoietic stem cells. Finally, other types of stem cells such as mesenchymal, endothelial stem cells or other tissue committed stem cells (TCSC), could be used for treating damages to nonhematopoietic organs. PMID:16382889

Hrodin, Francis; Mayol, Jean-Franois; Mourcin, Frdric; Drouet, Michel

2005-01-01

152

Effect of Testosterone Replacement Therapy on Bone Mineral Density in Patients with Klinefelter Syndrome  

PubMed Central

Purpose Klinefelter syndrome (KS) is related to testicular insufficiency, which causes low testosterone levels in serum. Generally, sex hormone levels and bone mineral density (BMD) are lower in patients with KS than normal. We investigated the effects of testosterone replacement on serum testosterone levels and BMD in KS patients. Materials and Methods From December 2005 to March 2008, 18 KS patients with a 47, XXY karyotype were treated with initial intramuscular injections of long-acting testosterone undecanoate (Nebido, 1000 mg/4 mL) at baseline and second injections after six weeks. An additional four injections were administered at intervals of 12 weeks after the second injection. BMD was measured at the lumbar spine (L2-4), the left femoral neck and Ward's triangle, using dual energy X-ray absorptiometry. Medical histories, physical examinations and prostate specific antigen, hematology and serum chemistry were conducted for each patient. In addition, total testosterone and sex hormone-binding globulin levels were measured. Results Following testosterone replacement, mean serum total testosterone increased significantly from baseline (0.90 vs. 4.51 ng/mL, p<0.001), and total testosterone rose to normal levels after replacement in all patients. The mean BMD of the lumbar spine increased significantly (0.91 vs. 0.97 g/cm2, p<0.001). Similar increases of BMD were also observed at the femoral neck, but this increase was not significant. Conclusion These findings suggest that testosterone replacement therapy may be effective in treating BMD deficiency in men with testosterone deficiency, especially those with Klinefelter syndrome. PMID:24142635

Jo, Dae Gi; Lee, Hyo Serk; Joo, Young Min

2013-01-01

153

Sheehan's syndrome: baseline characteristics and effect of 2 years of growth hormone replacement therapy in 91 patients in KIMS - Pfizer International Metabolic Database  

Microsoft Academic Search

Objective: Sheehan's syndrome occurs as a result of ischaemic pituitary necrosis due to severe postpar- tum haemorrhage. It is one of the most important causes of hypopituitarism, and hence growth hor- mone deficiency (GHD), in developing countries. However, little is known about the effects of growth hormone (GH) replacement therapy in patients with Sheehan's syndrome. Design: The demographic background characteristics

Fahrettin Kelestimur; Peter Jonsson; Senay Molvalilar; Jose Manuel Gomez; Christoph J Auernhammer; Ramiz Colak; Maria Koltowska-Haggstrom; Miklos I Goth

2005-01-01

154

Effects of Growth Hormone Therapy on Cardiac Dimensions in Children and Adolescents with Prader-Willi Syndrome  

Microsoft Academic Search

Objectives: Growth hormone (GH) secretory status and GH therapy influence cardiac muscle mass and function. Most patients with Prader-Willi syndrome (PWS) are GH-deficient (GHD), and are at risk to develop obesity-related cardiac problems. Few data exist on the development of cardiac dimensions under GH treatment in PWS. Study Design: In a retrospective study, cardiac dimensions measured echocardiographically in 26 children

Berthold P. Hauffa; Kathrin Knaup; Nils Lehmann; Ulrich Neudorf; Bert Nagel

2011-01-01

155

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 46 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 46 months.Main Outcome Measure(s): Clinical symptoms, menstrual pattern,

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

1998-01-01

156

Growth hormone therapy in Silver Russell Syndrome: 5 years experience of the Australian and New Zealand Growth database (OZGROW)  

Microsoft Academic Search

Data were analysed on 33 children (22 males) with Silver Russell syndrome treated with growth hormone for periods up to 5 years. Baseline data (medians) at commencement of growth hormone (GH) therapy were age 6.7 years, bone age delay 1.7 years, height standard deviation score (SDS)-3.2, weight SDS 3.1, and growth velocity 5.7 cm\\/year. All were prepubertal. Median birth weight

Y. Rakover; S. Dietsch; G. R. Ambler; C. Chock; M. Thomsett; C. T. Cowell

1996-01-01

157

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 10-18years 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

158

A manual therapy intervention improves symptoms in patients with carpal tunnel syndrome: a pilot study.  

PubMed

In carpal tunnel syndrome (CTS), manual therapy interventions (MTI) reduce tissue adhesion and increase wrist mobility. We evaluated the efficacy of a MTI in relieving CTS signs and symptoms. Twenty-two CTS patients (pts) (41 hands) were treated with a MTI, consisting in 6 treatments (2/week for 3 weeks) of soft tissues of wrist and hands and of carpal bones. Pts were assessed for hand sensitivity, paresthesia, hand strength, hand and forearm pain, night awakening; Phalen test, thenar eminence hypotrophy and Boston Carpal Tunnel Questionnaire (BCTQ) Symptom Severity Scale (SSS) and Functional Status Scale (FSS). Median nerve was studied by sensory nerve conduction velocity (SNCV) and distal motor latency (DML). CTS was scored as minimal, mild, medium, severe and extreme. We considered as control group the same pts assessed before treatment: at baseline (T0a) and after 12 weeks (T0b). Pts were evaluated at the end of treatment (T1) and after 24-week (T2) follow-up. At T0b, versus T0a, forearm pain and Phalen test positivity were increased and hand strength reduced (p < 0.05). BCTQ-SSS and BCTQ-FSS scores improved at T1 versus T0b (p < 0.05) with the amelioration maintained at T2. At T1, the number of pts with paresthesia, night awakening, hypoesthesia, Phalen test, hand strength reduction and hand sensitivity was reduced with the lacking of symptoms maintained at T2 (p < 0.05). No changes in SNCV, DML and CTS scoring were shown. MTI improved CTS signs and symptoms, with benefits maintained at follow-up. Thus, it may be valid as a conservative therapy. PMID:23064542

Maddali Bongi, Susanna; Signorini, Massimo; Bassetti, Massimo; Del Rosso, Angela; Orlandi, Martina; De Scisciolo, Giuseppe

2013-05-01

159

Effect of various drugs used in conservative therapy of hepatorenal syndrome: A retrospective drug utilization study  

PubMed Central

Objectives: To analyze the impact of various drugs used in conservative therapy on renal failure and mortality in hepatorenal syndrome (HRS) at a tertiary care teaching hospital. Materials and Methods: Retrospective review of hospital admission records was conducted for case records with HRS as diagnosis. Demographic and clinical data and drug utilization pattern were collected in a pre-designed patient information sheet. Impact of various drugs especially hepatoprotector antioxidant, silymarin, on survival benefits in terms of number of patients alive, change in mean arterial pressure (MAP) and change in serum creatinine at the end of treatment period were estimated by univariate and followed by multivariate analysis. Results: Of the total 89 case records, 31 met the eligibility criteria and were included in the analysis. On multivariate analysis, a significant correlation between use of intravenous fluids (IVFs) and survival benefits was observed (P < 0.05); wherein patients treated with IVFs had an increase in log odds of survival by 2.42 (95% CI = 1.06 to 121.13) as compared to patient not treated with IVF. However, MAP was not affected by any of the treatment modalities. While change in serum creatinine level was not significantly (P = 0.06, regression correlation = ?0.3) correlated with duration of treatment with IVFs. Conclusion: Use of IVFs may be associated with better short-term survival benefits and favor HRS reversal. Use of silymarin as hepatoprotector antioxidant has no beneficial effects on HRS reversal or survival benefits. PMID:25298585

Hiremath, Sharanabasayyaswamy B.; Kamdod, Mohammed Ameeruddin

2014-01-01

160

Long-term outcome of immunosuppressive therapy for Japanese patients with lower-risk myelodysplastic syndromes.  

PubMed

To investigate the long-term usefulness of immunosuppressive therapy (IST) for Japanese patients with lower-risk myelodysplastic syndromes, we retrospectively analyzed 29 MDS patients who were treated with cyclosporine A alone or with anti-thymocyte globulin at a single institute in Japan. A total of 58.6% of patients showed hematological response to IST. Overall survival of all patients was 74.5% at 5years and 48.3% at 10years. The major adverse event was the elevation of creatinine level (grade 1 and 2). Eleven patients were still on IST at the time of analysis with, at least, some clinical benefits. Pneumonia was the most frequent cause of death (eight of 12 deaths), followed by bleeding (three of 12); most of the patients who died were non-responders. The presence of paroxysmal nocturnal hemoglobinuria-type cells was significantly associated with both response to IST and long-term survival by univariate analysis. The 10-year overall survival of responders (72.2%) was significantly superior to that of non-responders (15.6%, P<0.0001). These results suggest that IST using cyclosporine A provides long-term benefit for Japanese patients with lower-risk MDS. PMID:24254637

Hata, Tomoko; Tsushima, Hideki; Baba, Maki; Imaizumi, Yoshitaka; Taguchi, Jun; Imanishi, Daisuke; Nagai, Kazuhiro; Tomonaga, Masao; Miyazaki, Yasushi

2013-12-01

161

[Piriformis muscle syndrome: etiology, pathogenesis, clinical manifestations, diagnosis, differential diagnosis and therapy].  

PubMed

The term 'piriformis syndrome' (PS), introduced by Robinson in 1947, implies a group of signs and symptoms caused by piriformis muscle (PM) disorders. Since PM disorders lead to irritation/compression of the anatomic structures passing under its belly, the main clinical PS signs and symptoms are actually the clinical signs and symptoms of irritation/ compression of neural and vascular structures passing through the infrapiriform foramen: sciatic nerve/SN, inferior gluteal nerve, posterior femoral cutaneous nerve, pudendal nerve, inferior gluteal artery and vein and inferior pudendal artery and vein. The clinical picture is usually dominated by signs and symptoms of irritation/compression of SN (SN irritation --> low back and buttock pain, sciatica,paresthesias in distribution of SN; SN compression --> low back and buttock pain,sciatica, paresthesias and neurologic deficit in distribution of SN). Irritation/compression of other structures can result in the following signs and symptoms: inferior gluteal nerve --> atrophy of gluteal muscles; posterior femoral cutaneous nerve --> pain, paresthesias and sensory disturbances in the posterior thigh; pudendal nerve --> pudendal neuralgia, painful sexual intercourse (dyspareunia), sexual dysfunction, urination and defecation problems; inferior gluteal artery --> ischemic buttock pain; inferior pudendal artery --> ischemic pain in the area of external sex organs, perineum and rectum, sexual dysfunction, urination and defecation problems; inferior gluteal vein --> venous stasis in gluteal area; inferior pudendal vein --> venous stasis in external sex organs and rectum. Functional/non-organic and organic PM disorders can cause PS: spasm, shortening, hypertrophy, anatomic variations, edema, fibrosis, adhesions, hematoma, atrophy, cyst, bursitis, abscess, myositis ossificans, endometriosis, tumors (functional disorders: PM spasm and shortening). The most common causes for PS are PM spasm, shortening and hypertrophy and anatomic variations of PM and SN. In 5-6% of patients with low back pain and/or unilateral sciatica, the pain is caused by PM disorders. PS diagnosis can be made on the basis of anamnesis, clinical picture, clinical examination, EMNG, perisciatic anesthetic block of PM and radiological exams (pelvis/PM MRI; MR neurography of LS plexus and SN). PS therapy includes medicamentous therapy, physical therapy, kynesitherapy, acupuncture, therapeutic perisciatic blocks, botulinum toxin injections and surgical treatment (tenotomy of PM, neurolysis of SN). PMID:23607175

Grgi?, Vjekoslav

2013-01-01

162

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

163

Multimodal physical therapy management of a 48-year-old female with post-stroke complex regional pain syndrome.  

PubMed

This case report describes a 48-year-old female who presented with complaints of right shoulder pain, hyperesthesias and swelling of the hand along with added symptoms of pain centralization following a cerebrovascular accident. On clinical evaluation, the patient satisfied the Budapest diagnostic criteria for Complex Regional Pain Syndrome (CRPS) type-1. Physical therapy management (1st three sessions) was initially focused on pain neurophysiology education with an aim to reduce kinesiophobia and reconceptualise her pain perception. The patient had an immediate significant improvement in her pain and functional status. Following this, pain modulation in the form of transcutaneous electrical nerve stimulation, kinesio tape application, "pain exposure" physical therapy and exercise therapy was carried out for a period of 7 weeks. The patient had complete resolution of her symptoms which was maintained at a six-month follow-up. PMID:23879307

Anandkumar, Sudarshan; Manivasagam, Murugavel

2014-01-01

164

[Compliance with continuous positive airway pressure therapy in patients with sleep apnea/hypopnea syndrome].  

PubMed

Predictive factors and compliance level were evaluated in a group of patients with sleep apnea syndrome under CPAP treatment, assessing side effects and equipment condition: silicone interface (SI), mask-conectors (M-C), air tube (AT) and head strap (HS). Patients with >3mo treatment were included, clock counter reading was registered at the beginning, 2 and 4 mo. Patients were considered compliant (C+) when usage was >4h/day and >5day/week. Of 46 patients (male 34; age 62 +/- 9years; BMI 33 +/- 7kg/m2; AHI 38 +/- 18/h; time of therapy 2.1 +/- 1.7years; CPAP 9 +/- 1.4 cmH2O), 34 had a clock counter and 24 (71%) were C+. Initial symptoms included: somnolence (65%), snoring (39%), bed-partner witnessed apneas (28%). Comparing C+ and C- we didn't find significant difference in age, BMI, CPAP pressure, length of therapy, AHI and pre-treatment Epworth classification. Referred vs. measured time of use in C+ and C- were 6.6 +/- 1 vs. 6.1 +/- 1 h/d (p=0.02) and 5.6 +/- 1 vs. 2.4 +/- 1 h/d (p<0.005). Compliant patients reported more resolution of somnolence (p<0.005) and nocturia (p<0.05), lower post CPAP Epworth (p<0.05), more frequent somnolence as initial symptom (p<0.05) and a higher education level (p=0.01). Side effects (SE) (n=45): dry mouth 36%, nasal congestion 27%, sleep disruption 11%, CPAP noisy 9%, dry nose, rhinorrhea and skin irritation 7%. Twenty seven percent of patients reduced the CPAP use because of the SE. Correction strategies included: humidifier, nasal steroid, surgery or infiltration of turbinates. Comparing the condition of SI, M-C, AT and HS between < or =1 vs. >1year of use, we observed a lower percentage of fine elements (87 to 44%, 74 to 44%, 83 to 44%, 91 to 78%, respectively). Most common defects included stiffness of SI, cracks in SI, M-C and AT, loose conexions. The study confirms the importance of objective monitoring in patients with CPAP. Side effects and equipment condition require special attention because this could affect an effective treatment. PMID:15560539

Gallego, Claudio W; Dibur, Eduardo; Salomone, Csar; Di Bartolo, Carlos G

2004-01-01

165

[Increased incidence of ovarian hyperstimulation syndrome following combined GnRH agonist/hMG therapy].  

PubMed

In the present paper we examined, whether the combined GnRH-agonist/hMG therapy implies an increased risk of the ovarian hyperstimulation syndrome (OHS). In a retrospective analysis, 525 GnRH-a/hMG cycles were compared with 643 cycles of hMG stimulation, which were simultaneously performed at the Department of Gynecology and Obstetrics of the University of Hamburg. Two different GnRH-agonists were used: Buserelin (Hoechst) given intranasally (410 cycles) and Triptorelin (Ferring) intramuscularly (115 cycles). The clinical results of hMG "only"-therapy revealed an OHS incidence of 7% for grade II and 0.2% for grade III. In contrast, significantly higher incidences were observed after GnRH-a/hMG treatment. In Buserelin/hMG cycles in 23% OHS grade II and in 1.0% OHS grade III occurred, in Triptorelin/hMG cycles in 40% OHS II and in 5.2% OHS III, respectively. The increased incidence of OHS correlated with higher ovarian estrogen production as well as a higher number of follicles following the GnRH-a/hMG stimulation. Furthermore, in GnRH-a/hMG cycles a prolonged duration of follicular maturation occurred due to an increase of the active phase; in addition the amount of hMG-ampoules needed for ovarian stimulation was higher. After GnRH-a/hMG treatment, an endogenous LH-surge was not detected, whereas in 34% of hMG stimulated cycles irregular LH-fluctuations were observed. There was a higher pregnancy rate in GnRH-a/hMG cycles (15%/525 cycles), as compared to hMG stimulation (8%/643 cycles), but the abortion rate was similar (23%, GnRH-a/hMG, versus 13%, hMG). The demonstration of an increased ovarian response leading to better pregnancy rates but also higher risks of OHS is well known from earlier data of hMG stimulation in patients with hypogonadotropic amenorrhoea (WHO group I). This implies that GnRH-agonist pre-treatment shows similar endocrine conditions in normogonadotropic patients. PMID:2525500

Lindner, C; Braendle, W; Khler, S; Bettendorf, G

1989-04-01

166

The prevalence and correlates of mind-body therapy practices in patients with acute coronary syndrome  

PubMed Central

Summary Objectives While the benefits of mind-body therapy (MBT) for cardiac secondary prevention continues to be investigated, the prevalence of such practices by cardiac patients is not well known. The aim of this study was to quantitatively examine the prevalence of MBT practice and its sociodemographic, clinical, psychosocial and behavioral correlates among patients with acute coronary syndrome (ACS). Methods Six hundred and sixty-one ACS in-patients (75% response rate) recruited from three hospitals completed a demographic survey, and clinical data were extracted from charts. Four hundred and sixty five patients (81% retention rate; 110 (23.7%) female) responded to an 18-month post-discharge survey that queried about MBT use and its correlates. Results One hundred and sixty-three (35.1%) ACS patients practised MBT in their lifetime, and 118 (25.4%) were currently practising. MBT users were more often women (OR = 2.98), non-white (OR = 2.17), had higher levels of education (OR = 2.22), past smokers (OR = 3.33), reported poorer mental health (OR =2.15), and engaged in more exercise (OR = 1.65). Conclusion One-third of ACS patients practised some form of MBT. The greater MBT practice among female ACS patients is noteworthy, given their generally lower physical activity and lower receipt of evidence-based treatments including cardiac rehabilitation. In addition, there is some evidence that MBT can promote mental well-being, and thus such practice might reduce risk related to negative affect in cardiac patients. PMID:19186341

Leung, Yvonne W.; Tamim, Hala; Stewart, Donna E.; Arthur, Heather M.; Grace, Sherry L.

2010-01-01

167

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

168

Management of acute coronary syndrome in the hospital: a focus on ACCF/AHA guideline updates to oral antiplatelet therapy.  

PubMed

The 3 main clinical manifestations of acute coronary syndrome (ACS) are unstable angina, non-ST-segment myocardial infarction, and ST-segment myocardial infarction. Together they comprise a major cause of emergency care and hospitalization in the United States. Consequently, all hospital-based physicians should be familiar with current recommendations regarding the diagnosis and management of ACS. Effective inhibition of platelet activation and aggregation is central to the treatment of ACS, and dual antiplatelet therapy with aspirin and a P2Y12 inhibitor is recommended in all patients with ACS. Recently, the American Heart Association and American College of Cardiology Foundation published focused updates to their guidelines for the management of ACS patients. These updates included changes in antiplatelet therapy arising from the introduction of prasugrel and ticagrelor as alternatives to clopidogrel for the P2Y12 inhibitor component of dual antiplatelet therapy. Among the P2Y12 inhibitors recommended for each indication, the guidelines do not advocate any one P2Y12 inhibitor over another, but instead recommend that therapy is individualized based on each patient's demographic and clinical characteristics. This article presents a clinical case study to illustrate the hospital management of ST-segment myocardial infarction and unstable angina/non-ST-segment myocardial infarction with particular reference to the latest changes in antiplatelet therapy guidelines. This article outlines key differences in the indications and recommendations for P2Y12 inhibitors and summarizes clinical data from the pivotal studies of prasugrel and ticagrelor. PMID:25255405

Vengoechea, Fabian

2014-08-01

169

Linezolid-Induced Near-Fatal Serotonin Syndrome During Escitalopram Therapy: Case Report and Review of Literature  

PubMed Central

Linezolid is a synthetic antimicrobial agent of the oxazolidinone class with weak, nonspecific inhibitor of monoamine oxidase enzymes. Concomitant therapy with an adrenergic or serotonergic agent or consuming tyramine (>100 mg/day) may induce serotonin syndrome (SS). We present a case report of near-fatal adverse interaction between linezolid and escitalopram inducing SS in a 65-year-old woman with sepsis, under empirical antibiotic treatment. This report also summarizes the current relevant literature as identified via PubMed, EMBASE, and PsycINFO, supplemented with a manual search of cross references. PMID:24379509

Kulkarni, Ranganath R.; Kulkarni, Pratibha R.

2013-01-01

170

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

Microsoft Academic Search

BackgroundIrritable 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 MethodsParticipants (N = 85) with IBS were recruited through

Erik Andersson; Brjnn Ljtsson; Filip Smit; Bjrn Paxling; Erik Hedman; Nils Lindefors; Gerhard Andersson; Christian Rck

2011-01-01

171

The acute phase reactant, fibrinogen, as a guide to plasma exchange therapy for acute Guillain-Barr syndrome.  

PubMed

The Guillian Barr syndrome is an acute inflammatory disorder for which plasma exchange is effective treatment. Up to 10% relapse after plasma exchange suggesting that treatment sometimes finishes before disease activity has resolved. We studied whether plasma fibrinogen, an inflammatory marker, might be used to determine when to discontinue plasma exchange in patients with acute Guillain-Barr syndrome. We conducted a post-hoc analysis of apheresis database and hospital records of patients treated with plasma exchange for acute Guillain-Barr syndrome during 1999-2004. Data were analyzed from 28 patients who underwent a total of 29 courses of plasma exchange for acute Guillain-Barr syndrome. The mean (+/-SD) plasma fibrinogen concentration was 422.5 (+/-96.4) mg/dl at the time of presentation and, in 17 of the 29, it was above 400 mg/dl (reference range 200-400). Twenty of the 21 patients whose fibrinogen fell by more than 30% from baseline by the time of the final plasma exchange treatment had neurological improvement. There was improvement in only 3 of the 8 instances where fibrinogen decreased by less than 30% by the end of plasma exchange therapy. A > or =30% decrease in fibrinogen by the conclusion of plasma exchange was significantly associated with sustained neurological improvement (P = 0.0025). The plasma fibrinogen level appears to reflect disease activity in acute Guillain-Barr syndrome. A <30% fall in fibrinogen level despite plasma exchange may indicate the need to continue plasma exchange to maximize the benefit of treatment or minimize the risk of relapse. Therapeutic plasma exchange need not be extended when plasma fibrinogen remains > or =30% below its level at presentation by the time of the final planned plasma exchange procedure. PMID:16342192

Sanjay, Rashmi; Flanagan, Janice; Sodano, Donata; Gorson, Kenneth C; Ropper, Allan H; Weinstein, Robert

2006-07-01

172

Oral anticoagulant use in addition to antiplatelet therapy for secondary prevention in acute coronary syndrome: current perspectives.  

PubMed

Patients with acute coronary syndrome (ACS) are typically managed with long-term dual antiplatelet therapy of acetylsalicylic acid plus a P2Y12 platelet receptor antagonist; however, although effective, the risk of another vascular event within 12 months remains at approximately 10%. Considerable efforts have been made to find improved therapeutic approaches to secondary prevention in ACS. The ATLAS ACS 2-TIMI 51 trial demonstrated that rivaroxaban (2.5 mg twice daily) significantly reduced recurrent vascular events, increased the risk of major bleeding but not the risk of fatal bleeding, and resulted in reduced rates of death from cardiovascular causes. These results formed the basis for approval in Europe of rivaroxaban (2.5 mg twice daily) in conjunction with standard antiplatelet therapy for the secondary prevention of ACS. PMID:25017622

Turpie, Alexander G G

2014-08-01

173

Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome1  

Microsoft Academic Search

Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome.BackgroundAlport syndrome (AS) is a common hereditary cause of end-stage renal failure in adolescence due to defects in type IV collagen genes. Molecular genetics allows early diagnosis, however, no preventive strategy can be offered. Using the COL4A3 ?\\/? mouse, an animal model for human AS,

Oliver Gross; Bogdan Beirowski; Marie-Louise Koepke; Jeannine Kuck; Michael Reiner; Klaus Addicks; Neil Smyth; Eckhard Schulze-Lohoff; Manfred Weber

2003-01-01

174

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

175

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

176

The Nephrotic Syndrome in an Elderly Woman. An Unusual Association with an Unusual Response to Therapy.  

National Technical Information Service (NTIS)

A 73-year-old woman with the nephrotic syndrome is presented. Percutaneous renal biopsy demonstrated changes compatible with chronic pyelonephritis. Significant reductions in proteinuria, edema, and weight were obtained with the combined use of a spironol...

P. J. Schneider, C. S. Eytel

1969-01-01

177

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

178

NPHS2 R229Q polymorphism in steroid resistant nephrotic syndrome: is it responsive to immunosuppressive therapy?  

PubMed

Steroid-resistant nephrotic syndrome (SRNS) patients with NPHS2 gene mutations have been reported as non-responsive to immunosuppressive therapy. Inter-ethnic differences can have influence over the frequency of mutations. The present study was undertaken to find out the incidence and treatment response. Mutational analysis of NPHS2 gene was performed in 20 sporadic idiopathic SRNS, 90 steroid-sensitive nephrotic syndrome (SSNS) and 50 normal controls. NPHS2 gene analysis showed R229Q polymorphism in six SRNS (30%), four SSNS (4.4%) and 13 controls (26%). The polymorphism (G?A) showed Hardy-Weinberg distribution and risk allele (G) had strong association with the disease (odds ratio 3.14, 95% CI 1.33-7.43) than controls. Five cases of SRNS having polymorphism showed partial remission to cyclosporine and prednisolone. Overall, partial remission was achieved in 14(70%), complete remission in four (20%), one(5%) patient had no response and one(5%) died. Thus, NPHS2 gene showed R229Q polymorphism and patients achieved partial remission to therapy. PMID:24519673

Mishra, Om P; Kakani, Neha; Singh, Arun K; Narayan, Gopeshwar; Abhinay, Abhishek; Prasad, Rajniti; Batra, Vineeta V

2014-06-01

179

Proton Beam Therapy for a Patient with a Giant Thymic Carcinoid Tumor and Severe Superior Vena Cava Syndrome  

PubMed Central

Surgical resection is the first choice for treatment of a thymic carcinoid tumor and radiotherapy is often performed as adjuvant therapy. Here, we report a case of an unresectable and chemoresistant thymic carcinoid tumor that was treated successfully using standalone proton beam therapy (PBT). The patient was a 66-year-old woman in whom surgical resection of the tumor was impossible because of cardiac invasion. Therefore, chemotherapy was administered. However, the tumor grew to 15 cm in diameter and she developed severe superior vena cava (SVC) syndrome. She was referred to our hospital and received PBT at a dose of 74 GyE in 37 fractions. PBT was conducted without severe early toxicities. After PBT, the tumor mildly shrunk to 13 cm in diameter and SVC syndrome almost disappeared. Subsequently, the tumor has continued to decrease in size slowly over the last 2 years and late toxicities have not been observed. Our experience with this case suggests that PBT may be effective for an unresectable thymic carcinoid tumor. PMID:25002943

Sugawara, Kaori; Mizumoto, Masashi; Numajiri, Haruko; Ohno, Toshiki; Ohnishi, Kayoko; Ishikawa, Hitoshi; Okumura, Toshiyuki; Sakurai, Hideyuki

2014-01-01

180

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

181

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

Microsoft Academic Search

LEGO therapy and the Social Use of Language Programme (SULP) were evaluated as social skills interventions for 611 year olds\\u000a with high functioning autism and Asperger Syndrome. Children were matched on CA, IQ, and autistic symptoms before being randomly\\u000a assigned to LEGO or SULP. Therapy occurred for 1 h\\/week over 18 weeks. A no-intervention control group was also assessed.\\u000a Results

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

2008-01-01

182

The effect of gallium arsenide aluminum laser therapy in the management of cervical myofascial pain syndrome: a double blind, placebo-controlled study  

Microsoft Academic Search

The efficacy of low-level laser therapy (LLLT) in myofascial pain syndrome (MPS) seems controversial. A prospective, double-blind,\\u000a randomized controlled trial was conducted in patients with chronic MPS in the neck to evaluate the effects of low-level 830-nm\\u000a gallium arsenide aluminum (GaAsAl) laser therapy. The study group consisted of 64 MPS patients. The patients were randomly\\u000a assigned into two groups. In

U. Dundar; D. Evcik; F. Samli; H. Pusak; V. Kavuncu

2007-01-01

183

Effects of recombinant human growth hormone therapy on carbohydrate, lipid and protein metabolisms of children with Turner syndrome  

PubMed Central

Objective: To study the effect of recombinant human growth hormone (rhGH) therapy on carbohydrate, lipid and protein metabolisms of Turner syndrome (TS). Metho d s: Total 45 patients with TS admitted between Jul. 2008 and Jun. 2011 were involved in this study. All patients received the clinical evaluation of body fat, plasma lipids, proteins and oral glucose tolerance test (OGTT) before and after rhGH therapy. Results : Our results indicated a significant decrease of body fat (FAT%) from 23.564.21 to 18.712.23 but no obvious change on the level of fat mass (FM) (p>0.05) was observed after rhGH therapy. We also detected significant changes on plasma high-density lipoprotein cholesterol (HDL-C) from (1.650.58 mmol/L) to (2.200.65 mmol/L) and low-density lipoprotein cholesterol (LDH-C) from (2.550.55 mmol/L) to (2.100.54 mmol/L) after rhGH exposure. However, no statistical significance was detected on the level of plasma triglyceride (TG), cholesterol (CHO). Interestingly, the levels of plasma retinol binding protein (RbP) (32.554.28mg/L), transferrin (TRF) (2.950.40 mg/L), serum albumin (PRE) (250.0045.50 mg/L) and albumin (propagated) (33.584.25 mg/L) were significantly increased. When it goes to the oral glucose tolerance test (OGTT) test, there were 10 impaired glucose tolerance (IGT) cases among all patients before and after rhGH therapy. No significant change was observed on homeostasis model assessment- insulin resistance (HOMA-IR) level during rhGH intervention. Conclusion : Abnormal lipid and protein metabolisms of the children with TS can be improved with rhGH therapy for 6 months. PMID:25097506

Qi, Weibin; Li, Shuxian; Shen, Qiong; Guo, Xiuxia; Rong, Huijuan

2014-01-01

184

DRUG-BASED THERAPIES FOR VASCULAR DISEASE IN MARFAN SYNDROME: FROM MOUSE MODELS TO HUMAN PATIENTS  

PubMed Central

Marfan syndrome is a congenital disorder of the connective tissue with a long history of clinical and basic science breakthroughs that have forged our understanding of vascular disease pathogenesis. The biomedical importance of Marfan syndrome was recently underscored by the discovery that the underlying genetic lesion impairs both tissue integrity and transforming growth factor-? regulation of cell behavior. This discovery has led to the successful implementation of the first pharmacological intervention in a connective tissue disorder otherwise incurable by either gene- or stem cell-based therapeutic strategies. More generally, information gathered from the study of Marfan syndrome pathogenesis has the potential to improve the clinical management of common acquired disorders of connective tissue degeneration. PMID:20687182

Cook, Jason R.; Nistala, Harikiran; Ramirez, Francesco

2010-01-01

185

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; Huser, Winfried

2013-01-01

186

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

187

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

188

Cognitive-behaviour therapy for chronic fatigue syndrome: Comparison of outcomes within and outside the confines of a randomised controlled trial  

Microsoft Academic Search

Outcomes for cognitive-behaviour therapy (CBT) in randomised controlled trials (RCTs) have rarely been compared to those in routine clinical practice. Taking the case of CBT for chronic fatigue syndrome (CFS), we evaluated the results of a successful RCT against those of the same treatment given in the same setting as part of routine practice. Fatigue and social adjustment scores were

Louise Quarmby; Katharine A. Rimes; Alicia Deale; Simon Wessely; Trudie Chalder

2007-01-01

189

CASE REPORT: SUCCESSFUL TREATMENT OF ACUTE EXERTIONAL PARASPINAL COMPARTMENT SYNDROME WITH HYPERBARIC OXYGEN THERAPY  

PubMed Central

An NCAA football player developed an acute paraspinal compartment syndrome after a weight-lifting strain. The patient presented with myone-crosis (CK up to 77,400 U/L), and myoglobinuria. Treatment consisted of forced diuresis and six sessions in the hyperbaric oxygen chamber. PMID:21045996

Karam, Mathew D.; Amendola, Annunziato; Mendoza-Lattes, Sergio

2010-01-01

190

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; Danille C. Cath; W. J. G. Hoogendijk

2006-01-01

191

Troglitazone Therapy Improves Endothelial Function to Near Normal Levels in Women with Polycystic Ovary Syndrome  

Microsoft Academic Search

Obese women with polycystic ovary syndrome (PCOS) exhibit impaired endothelial function, which is strongly and directly correlated with both testosterone levels and insulin resis- tance. Endothelial dysfunction is considered a potent risk factor for macrovascular disease. Because troglitazone (Tgz) improves both hormonal profiles and insulin sensitivity, we tested whether Tgz treatment ameliorates endothelial func- tion in these patients. We studied

GIANCARLO PARADISI; HELMUT O. STEINBERG; MARGUERITE K. SHEPARD; GINGER HOOK; ALAIN D. BARON

2010-01-01

192

Update on the classification, assessment of prognosis and therapy of BuddChiari syndrome  

Microsoft Academic Search

BuddChiari syndrome (BCS) occurs as a result of obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium. Diagnosis can be difficult because of the wide spectrum of presentation of the disease and the varying severity of liver damage. The traditional classification of BCSas fulminant,

Marco Senzolo; Evangelos C Cholongitas; David Patch; Andrew K Burroughs

2005-01-01

193

Reversed Twin-to-Twin Transfusion Syndrome following Successful Laser Therapy  

Microsoft Academic Search

We report a case of severe twin-to-twin transfusion syndrome (TTTS) in which fetoscopic laser ablation was successfully performed at 21 weeks gestation. Despite successful resolution of signs on ultrasound examination after a period of 7 weeks, the donor developed polyhydramnios whereas the initial recipient maintained normal liquor volume, features suggestive of reversed TTTS. Two live babies were delivered at 31

Caroline E. Fox; Ben C. Chan; Phillip Cox; Adam Gornall; Mark D. Kilby

2009-01-01

194

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

195

Cyclosporin A therapy for patients with myelodysplastic syndrome: multicenter pilot studies in Japan  

Microsoft Academic Search

We examined the efficacy of cyclosporin A (CsA) in 50 patients with myelodysplastic syndrome (MDS) consisting from 47 of RA, 1 of RARS, and 2 of RAEB. These patients showed various marrow cell types including hypo-, normo-, and hypercellularity. Patients belonged to the following International Prognostic Scoring System (IPSS) risk groups: 4 of low, 41 of intermediate-1, and 5 of

Takashi Shimamoto; Kaoru Tohyama; Takahiro Okamoto; Takashi Uchiyama; Hiroyuki Mori; Masao Tomonaga; Yoshinobu Asano; Yoshiyuki Niho; Masanao Teramura; Hideaki Mizoguchi; Mitsuhiro Omine; Kazuma Ohyashiki

2003-01-01

196

A Placebo-controlled Randomized Trial of Antithrombin Therapy in Neonatal Respiratory Distress Syndrome  

Microsoft Academic Search

Neonatal respiratory distress syndrome (RDS) is associated with decreased plasma activity of anti- thrombin (AT) and increased formation of thrombin. We tested whether AT reduces thrombin forma- tion, improves gas exchange, and decreases the duration of mechanical ventilation and supplemental oxygen. One hundred twenty-two infants were randomized to pasteurized AT concentrate or to pla- cebo. Two ml\\/kg (equivalent to 100

BARBARA SCHMIDT; PATRICE GILLIE; LESLEY MITCHELL; MAUREEN ANDREW; CHRIS CACO; ROBIN ROBERTS

1998-01-01

197

Tumor lysis syndrome in a chronic lymphocytic leukemia patient with pleural effusion after oral fludarabine and cyclophosphamide therapy.  

PubMed

Tumor lysis syndrome (TLS) is a rare complication of the treatment for chronic lymphocytic leukemia (CLL). Since the advent of new therapeutic agents with higher response rates, however, TLS has been observed with increasing frequency. An 84-year-old woman with a nine-year history of untreated CLL presented with exacerbating dyspnea due to pleural effusion. CLL cells without Richter transformation were observed in the pleural effusion at a high concentration, as well as in lymph nodes and bone marrow. After 5 days of oral fludarabine and cyclophosphamide (FC) therapy, the patient developed TLS, which necessitated rescue with hemodialysis. Although transient exacerbation of pleurisy occurred, the effusion cytology ameliorated, and she eventually achieved complete remission after additional two courses of FC and rituximab. Sequestration of fludarabine in the pleural effusion may be attributable to the development of TLS. PMID:24584911

Nakazawa, Hideyuki; Nishina, Sayaka; Mimura, Yuto; Kawakami, Toru; Senoo, Yasushi; Sakai, Kaoko; Nakazawa, Ko; Kitano, Kiyoshi

2014-06-01

198

Kaposi's sarcoma in pregnancy after initiation of highly active antiretroviral therapy: a manifestation of immune reconstitution syndrome.  

PubMed

A case of Kaposi's sarcoma (KS) presenting as an immune reconstitution inflammatory syndrome in pregnancy with conservative management is reported. Successful outcomes for mother and baby were achieved. HIV was diagnosed at antenatal booking and highly active antiretroviral therapy commenced at 20 weeks. Multiple lymphadenopathies developed two months later. Excision biopsy of a node confirmed KS. In the absence of advanced disease, she was managed conservatively until delivery. The placenta showed no evidence of KS or human herpes virus 8 (HHV-8). The baby had negative HIV and HHV-8 polymerase chain reaction tests at zero, six and 12 weeks of life. Six months postpartum, the KS had regressed and HHV-8 viral load was undetectable. PMID:23258835

Adeyemo, A; Wood, C; Govind, A

2012-12-01

199

Hyperbaric oxygen therapy prevents vascular derangement during zymosan-induced multiple-organ-failure syndrome  

Microsoft Academic Search

ObjectiveThis study investigated the effects of hyperbaric oxygen (HBO) therapy on the cardiovascular alteration (e.g. mean arterial pressure, vascular reactivity of thoracic aorta rings changes) caused by zymosan in rats.DesignRats.SettingUniversity research laboratory.Intervention and measurementsWe investigated the effects of HBO therapy (2 ATA at the fourth and eleventh hours after study onset) on the cardiovascular alteration caused by zymosan (500mg\\/kg, administered

Francesco Imperatore; Salvatore Cuzzocrea; Carlo Luongo; Giovanni Liguori; Antonella Scafuro; Antonella De Angelis; Francesco Rossi; Achille P. Caputi; Amelia Filippelli

2004-01-01

200

Paraneoplastic stiff person syndrome associated with colon cancer misdiagnosed as idiopathic Parkinsons disease worsened after capecitabine therapy  

PubMed Central

Objectives To refresh clinical diagnostic dilemmas in patients presenting with symptoms resembling to those of parkinsonism, to report rare association of colon cancer and paraneoplastic stiff person syndrome (SPS), and to draw attention on the possible correlation of capecitabine therapy with worsening of paraneoplastic SPS. Methods Case report of the patient with paraneoplastic SPS due to colon cancer that was misdiagnosed as idiopathic Parkinsons disease (iPD), whose symptoms worsened after beginning adjuvant capecitabine chemotherapy. Results We describe a 55-year-old woman with subacute onset of symmetrical stiffness and rigidity of the truncal and proximal lower limb muscles that caused lower body bradykinesia, gait difficulties, and postural instability. Diagnose of iPD was made and levodopa treatment was initiated but failed to provide beneficial effect. Six months later, colon cancer was discovered and the patient underwent surgical procedure and chemotherapy with capecitabine thereafter. Aggravation of stiffness, rigidity, and low back pain was observed after the first chemotherapy cycle and capecitabine was discontinued. Furthermore, levodopa was slowly discontinued and low dose of diazepam was administered which resulted in partial resolution of the patients symptoms. Conclusion Paraneoplastic SPS is rare disorder with clinical features resembling those of parkinsonian syndrome and making the correct diagnosis remains a challenge. The diagnosis of parkinsonian syndrome should be re-examined if subsequent examinations discover an associated malignant process. Although it remains unclear whether the patients with history of SPS are at the greater risk for symptoms deterioration after administration of capecitabine, clinicians should be aware of capecitabine side effects because recognition and appropriate management can prevent serious adverse outcomes. PMID:24028681

2013-01-01

201

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

202

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

203

17/09/09 18:50NetLogo User's Guide Page 1 sur 10file:///Users/jost/Documents/enseignement/lectures/Cours+TD%20AutomateCellulaire/NetLogo%20tutorial/firefly-tutorial.html  

E-print Network

17/09/09 18:50NetLogo User's Guide Page 1 sur 10file:///Users/jost/Documents/enseignement/lectures/Cours+TD%20AutomateCellulaire/NetLogo%20tutorial/firefly-tutorial.html NetLogo User's Guide Programming Tutorial for synchronizing fireflies (adapted from the official tutorial) NetLogo is a freeware program

Jost, Christian

204

Acquired Bartter-like syndrome association with netilmicin therapy in an extremely low birth weight infant.  

PubMed

Aminoglycosides are commonly used antibiotics with excellent renal parenchymal penetration. Their clinical effectiveness is counter balanced with the risk of renal toxicity, which develops in a dose-dependent fashion. Aminoglycoside-induced renal tubular dysfunction could result in diffuse damage or manifest as a Fanconi-like syndrome, Bartter-like syndrome (BLS), or distal renal tubular acidosis.(1-4) Although tubulopathy associated with amikacin and gentamicin was reported in adults and rarely children, to the best of our knowledge, netilmicin-associated BLS neither in adults nor in children has been reported in the literature. We here report a 30-week, 770?g male preterm infant who developed BLS just after netilmicin treatment for neonatal sepsis and recovered 6 weeks after the drug cessation. PMID:24059693

Sandal, Gonca; Akbay, ?enay; Ozen, Metehan

2014-02-01

205

Conservative therapy for Complex Regional Pain Syndrome Type I in a paediatric patient: a case study  

PubMed Central

Complex regional pain syndrome (CRPS) is a term that describes a variety of chronic pain conditions that are believed to result from dysfunction in the central or peripheral nervous systems. Typical features include dramatic changes in the colour and temperature of the skin over the affected limb or body part, accompanied by an intense pain which is out of proportion to the injury thought responsible. Skin sensitivity, sweating, and swelling are also commonly involved. This case study presents subjective reports of changes in pain and extremity weight bearing capacity in an 8 year-old child with Chronic Region Pain Syndrome Type I. The changes reported occurred over a 12 week conservative course of treatment which included manipulation, nutritional supplementation and rehabilitation. The patient was able to regain full control of her legs and full weight bearing after 3 weeks of treatment. PMID:19506699

Beck, Randy W.

2009-01-01

206

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

207

Deep brain stimulation therapy for treatment-refractory Tourettes syndrome  

Microsoft Academic Search

Tourettes syndrome is a chronic neurobehavioral disorder that can demonstrate refractoriness to conservative treatments,\\u000a or to invasive nonsurgical treatments such as botulinum toxin infiltration, or to psychobehavioral treatments. In these cases,\\u000a the surgical option is often proposed, either with lesional interventions, or more recently with deep brain stimulation (DBS).\\u000a This latter modality is currently preferred because of its reversibility and

Marco Sassi; Mauro Porta; Domenico Servello

2011-01-01

208

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

209

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

210

AMP kinase and malonyl-CoA: targets for therapy of the metabolic syndrome  

Microsoft Academic Search

Patients with the metabolic syndrome are characterized by insulin resistance, obesity and a predisposition to hypertension, dyslipidaemia, pancreatic ?-cell dysfunction, type 2 diabetes and premature atherosclerosis. Here we review the hypothesis that a common feature linking these multiple abnormalities is dysregulation of the AMP-activated protein kinase (AMPK)\\/malonyl-CoA fuel-sensing and signalling network. It is proposed that such dysregulation leads to alterations

Neil Ruderman; Marc Prentki

2004-01-01

211

Efficacy of salmon calcitonin in complex regional pain syndrome (type 1) in addition to physical therapy  

Microsoft Academic Search

The aim of the study was to assess the efficacy of salmon calcitonin, which was suggested as effective in the treatment of\\u000a complex regional pain syndrome type 1 (CRPS 1). Patients who had suffered trauma to their upper extremities and developed\\u000a CRPS 1 were included into this randomised, controlled single-blind study. The diagnosis was made according to the clinical\\u000a examination

Fusun Sahin; Figen Yilmaz; Nurdan Kotevoglu; Banu Kuran

2006-01-01

212

Effectiveness of aquatic therapy in the treatment of fibromyalgia syndrome: a randomized controlled open study  

Microsoft Academic Search

The aim of this study was to investigate the efficacy of aquatic exercises in fibromyalgia syndrome (FMS). A total of 63 patients\\u000a were included and allocated to two groups. Group I (n=33) received an aquatic exercise program and Group II (n=30) received a home-based exercise program for 60min, 3 a week, over 5weeks. Patients were evaluated for pain (visual\\u000a analogue

Deniz Evcik; Ilknur Yigit; Hasan Pusak; Vural Kavuncu

2008-01-01

213

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 Mller; Martin Hellmich; Martin Theisohn

2005-01-01

214

Use of Secondary Prevention Drug Therapy in Patients With Acute Coronary Syndrome After Hospital Discharge  

Microsoft Academic Search

syndrome were significantly less likely than those with acute myocardial infarction to receive any of the study drugs (P < 0.001). Those patients who had used ACE inhibitors\\/ARBs, beta-blockers, statins, and all 3 drug class- es during the 6 months prior to the index diagnosis of ACS were more likely than those without prior use (odds ratios of 12.2, 9.4,

Helen Y. Lee; Catherine E. Cooke; Teisha A. Robertson

2008-01-01

215

Down Syndrome  

MedlinePLUS

... help improve skills. They may include speech, physical, occupational, and/or educational therapy. With support and treatment, many people with Down syndrome live happy, productive lives. NIH: National Institute of Child Health and Human Development

216

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

PubMed Central

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 8th 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 Fourniers 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, Flavia Souza; Zangirolami, Carlos Eduardo Alves; Moreira, Alecsandro; Silva, Giovanni Faria; de Oliveira, Cassio Vieira

2014-01-01

217

Shockwave therapy in the management of complex regional pain syndrome in medial femoral condyle of the knee.  

PubMed

The aim of this prospective study was to assess the efficacy of shockwave (SW) therapy in the management of complex regional pain syndrome (CRPS). In this study, 30 patients (pts) who were affected by CRPS of the medial femoral condyle and unresponsive to previous standard physiotherapeutic and pharmacological treatment underwent 3 SW sessions at 72-h intervals, each consisting of 4000 shocks emitted by a MiniLith SL1 Storz electromagnetic generator. An energy flux density (EFD) of 0.035 or 0.09 mJ/mm(2) was used, depending on how well the patient endured the pain during the treatment. Satisfactory results were observed in 76.7% of the cases (23 pts) at the 2-month follow-up (FU) visit, and in 80% (24 pts) at the 6-month FU visit. The therapeutic effects of SW were caused by decreasing pain. The significant improvements we obtained bear witness to the potential value of SW therapy in the management of CRPS. PMID:20447749

Notarnicola, Angela; Moretti, Lorenzo; Tafuri, Silvio; Panella, Antonio; Filipponi, Marco; Casalino, Alessio; Panella, Michele; Moretti, Biagio

2010-06-01

218

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

219

[Phantom limb pain syndrome: therapeutic approach using mirror therapy in a Geriatric Department].  

PubMed

The clinical use of mirror visual feedback was initially introduced to alleviate phantom pain by restoring motor function through plastic changes in the human primary motor cortex. It is a promising novel technique that gives a new perspective to neurological rehabilitation. Using this therapy, the mirror neuron system is activated and decrease the activity of those systems that perceive protopathic pain, making somatosensory cortex reorganization possible. This paper reports the results of the mirror therapy in three patients with phantom limb pain after recent lower limb amputation, showing its analgesic effects and its benefits as a comprehensive rehabilitation instrument for lower limb amputee geriatric patients. PMID:23498652

Gonzlez Garca, Paloma; Manzano Hernndez, M Pilar; Muoz Toms, M Teresa; Martn Hernndez, Carlos; Forcano Garca, Mercedes

2013-01-01

220

Amlodipine/Atorvastatin single-pill therapy for blood pressure and lipid goals in African Americans: influence of the metabolic syndrome and type 2 diabetes mellitus.  

PubMed

African Americans with diabetes +/- the metabolic syndrome are at high risk for cardiovascular disease. This subanalysis of the Clinical Utility of Caduet in Simultaneously Achieving Blood Pressure and Lipid End Points (CAPABLE) trial studied attainment of the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure (JNC 7) and the National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III) blood pressure (BP) and low-density lipoprotein cholesterol (LDL-C) goals by 8 flexibly titrated doses (5/10-10/80 mg) of amlodipine/atorvastatin single pill in 494 African Americans with hypertension and dyslipidemia, according to the presence of diabetes +/- the metabolic syndrome. In 169 diabetic patients, the metabolic syndrome was associated with poorer BP goal attainment (38.5% vs 48.5% in diabetic patients without the metabolic syndrome). Among diabetic patients (+/- the metabolic syndrome) 61% to 62% reached LDL-C goal. More than 60% of patients with diabetes uncontrolled for LDL-C were maintained on suboptimal atorvastatin therapy (mean final dose: 29.9 mg vs maximum of 80 mg). Reluctance to intensify therapy to attain accepted targets in high-risk individuals suggests a degree of clinical inertia not explained by objective evidence of dose-dependent intolerance. PMID:19817942

Ferdinand, Keith C; Flack, John M; Saunders, Elijah; Victor, Ronald; Watson, Karol; Kursun, Attila; Jamieson, Michael J; Shi, Harry

2009-10-01

221

Maintenance oral pantoprazole therapy is effective for patients with Zollinger-Ellison syndrome and idiopathic hypersecretion  

Microsoft Academic Search

ObjectiveMaintenance proton pump inhibitor (PPI) therapy is effective for gastric acid hypersecretory states, although data with pantoprazole are limited. The aim of this study was to evaluate the safety and efficacy of long term p.o. pantoprazole in individuals with hypersecretion.

David C Metz; Elaine Soffer; Chris E Forsmark; Byron Cryer; William Chey; Wieslaw Bochenek; Joseph R Pisegna

2003-01-01

222

A randomised trial of adaptive pacing therapy, cognitive behaviour therapy, graded exercise, and specialist medical care for chronic fatigue syndrome (PACE): statistical analysis plan  

PubMed Central

Background The publication of protocols by medical journals is increasingly becoming an accepted means for promoting good quality research and maximising transparency. Recently, Finfer and Bellomo have suggested the publication of statistical analysis plans (SAPs).The aim of this paper is to make public and to report in detail the planned analyses that were approved by the Trial Steering Committee in May 2010 for the principal papers of the PACE (Pacing, graded Activity, and Cognitive behaviour therapy: a randomised Evaluation) trial, a treatment trial for chronic fatigue syndrome. It illustrates planned analyses of a complex intervention trial that allows for the impact of clustering by care providers, where multiple care-providers are present for each patient in some but not all arms of the trial. Results The trial design, objectives and data collection are reported. Considerations relating to blinding, samples, adherence to the protocol, stratification, centre and other clustering effects, missing data, multiplicity and compliance are described. Descriptive, interim and final analyses of the primary and secondary outcomes are then outlined. Conclusions This SAP maximises transparency, providing a record of all planned analyses, and it may be a resource for those who are developing SAPs, acting as an illustrative example for teaching and methodological research. It is not the sum of the statistical analysis sections of the principal papers, being completed well before individual papers were drafted. Trial registration ISRCTN54285094 assigned 22 May 2003; First participant was randomised on 18 March 2005. PMID:24225069

2013-01-01

223

Progressive neurodegenerative syndrome in a patient with X-linked agammaglobulinemia receiving intravenous immunoglobulin therapy.  

PubMed

A progressive encephalopathy of unknown etiology has been described in patients with primary immunodeficiency disorders. In this report, we characterize the clinical features of this progressive neurodegenerative dementing disorder in a young man with Bruton agammaglobulinemia, through neuropsychological tests and a video sequence. The clinical course of the encephalopathy seems rather uniform: Cognition, especially frontal lobe function, is affected in the early stages, and some patients develop movement disorders. The syndrome causes severe cognitive and physical disability, and can eventually be fatal. The autoimmunity results from dysregulated immune responses, but the underlying mechanism has not yet been fully explained. PMID:25237746

Sag, Aslihan Taskiran; Saka, Esen; Ozgur, Tuba Turul; Sanal, Ozden; Ayvaz, Deniz Cagdas; Elibol, Bulent; Kurne, Asli Tuncer

2014-09-01

224

New oral anticoagulants in addition to single or dual antiplatelet therapy after an acute coronary syndrome: a systematic review and meta-analysis  

PubMed Central

Background Oral anticoagulation in addition to antiplatelet treatment after an acute coronary syndrome might reduce ischaemic events but increase bleeding risk. We performed a meta-analysis to evaluate the efficacy and safety of adding direct thrombin or factor-Xa inhibition by any of the novel oral anticoagulants (apixaban, dabigatran, darexaban, rivaroxaban, and ximelagatran) to single (aspirin) or dual (aspirin and clopidogrel) antiplatelet therapy in this setting. Methods and results All seven published randomized, placebo-controlled phase II and III studies of novel oral anticoagulants in acute coronary syndromes were included. The database consisted of 30 866 patients, 4135 (13.4%) on single, and 26 731 (86.6%) on dual antiplatelet therapy, with a non-ST- or ST-elevation acute coronary syndrome within the last 714 days. We defined major adverse cardiovascular events (MACEs) as the composite of all-cause mortality, myocardial infarction, or stroke; and clinically significant bleeding as the composite of major and non-major bleeding requiring medical attention according to the study definitions. When compared with aspirin alone the combination of an oral anticoagulant and aspirin reduced the incidence of MACE [hazard ratio (HR) and 95% confidence interval 0.70; 0.590.84], but increased clinically significant bleeding (HR: 1.79; 1.542.09). Compared with dual antiplatelet therapy with aspirin and clopidogrel, adding an oral anticoagulant decreased the incidence of MACE modestly (HR: 0.87; 0.800.95), but more than doubled the bleeding (HR: 2.34; 2.062.66). Heterogeneity between studies was low, and results were similar when restricting the analysis to phase III studies. Conclusion In patients with a recent acute coronary syndrome, the addition of a new oral anticoagulant to antiplatelet therapy results in a modest reduction in cardiovascular events but a substantial increase in bleeding, most pronounced when new oral anticoagulants are combined with dual antiplatelet therapy. PMID:23470494

Oldgren, Jonas; Wallentin, Lars; Alexander, John H.; James, Stefan; Jonelid, Birgitta; Steg, Gabriel; Sundstrom, Johan

2013-01-01

225

Physical therapy management of pelvi\\/perineal and perianal pain syndromes  

Microsoft Academic Search

Pelvic floor physical therapy is considered to be effective in the management of functional urogenital and anorectal disorders.\\u000a A functioning pelvic floor is integral to increases in intra-abdominal pressure, provides rectal support during defecation,\\u000a has an inhibitory effect on bladder activity, helps support pelvic organs, and assists in lumbopelvic stability. Coordinated\\u000a release of the sphincters within a supporting extensible levator

S. J. Markwell

2001-01-01

226

Dexamethasone palmitate successfully attenuates hemophagocytic syndrome after allogeneic stem cell transplantation: macrophage-targeted steroid therapy.  

PubMed

Hemophagocytic syndrome (HPS) induced by uncontrolled macrophage activation and subsequent graft failure is a frequent and prominent complication after allogeneic stem cell transplantation (allo-SCT), a cause of severe morbidity and death, and a therapeutic challenge. Liposome-incorporated dexamethasone, dexamethasone palmitate (DP), shows greater efficacy against macrophages as compared to dexamethasone sodium phosphate (DSP). Based on our findings that DP achieves significantly larger decrease than DSP on the viability of primary human macrophages compared in vitro, we tested the effects of DP in patients with HPS. A decrease in number of macrophages in the bone marrow and prevention of engraftment failure were observed in all patients without any severe complications. In conclusion, these data provide a rationale for testing DP as a first-line treatment for patients with HPS after allo-SCT. PMID:22351164

Nishiwaki, Satoshi; Nakayama, Takayuki; Murata, Makoto; Nishida, Tetsuya; Sugimoto, Kyoko; Saito, Shigeki; Kato, Tomonori; Mizuno, Hiroki; Imahashi, Nobuhiko; Seto, Aika; Ozawa, Yukiyasu; Goto, Tatsunori; Koyama, Daisuke; Yokohata, Emi; Kubota, Naomi; Kamoshita, Sonoko; Miyamura, Koichi; Matsumoto, Kimikazu; Ito, Masafumi; Naoe, Tomoki

2012-04-01

227

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.

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

2014-01-01

228

Endovascular therapy for left main compression syndrome. Case report and literature review.  

PubMed

Extrinsic compression of the left main coronary artery (LMCA) can occur in patients with severe pulmonary hypertension and enlarged pulmonary artery trunk. It has been usually described in the setting of congenital defects such as atrial septal defect, ventricular septal defect, and, more rarely, isolated persistent ductus arteriosus. Functional and structural evaluation of such patients can currently be performed noninvasively with the use of cardiac CT scanning and/or MRI. The optimal management of symptomatic patients remains unknown. We report a case of extrinsic compression of the LMCA in a symptomatic patient with Eisenmenger syndrome who underwent unprotected LMCA stent implantation. We also performed a literature review of the reported cases concerning patients treated with LMCA stent implantation for the management of this condition. PMID:19497900

Caldera, Angel E; Cruz-Gonzalez, Ignacio; Bezerra, Hiram G; Cury, Ricardo C; Palacios, Igor F; Cockrill, Barbara A; Inglessis-Azuaje, Ignacio

2009-06-01

229

[Antiplatelet therapy after acute coronary syndrome : Therapeutic strategies and treatment duration].  

PubMed

Besides percutaneous coronary interventions, antiplatelet drugs are of overwhelming importance for patients with acute coronary syndrome (ACS). For ACS patients, the guidelines recommend treatment with acetylsalicylic acid and a P2Y12 receptor antagonist. The third generation P2Y12 receptor antagonists prasugrel and ticagrelor provide stronger platelet inhibition than clopidogrel and improve the clinical outcome in patients with ACS; however, it is still under discussion which P2Y12 antagonist fits best to which subgroup of ACS patients. This article summarizes current guidelines and antiplatelet treatment strategies for patients with non-ST-segment elevation (NSTE) ACS or ST-segment elevation myocardial infarction (STEMI). The information is mainly based on the recently published guidelines of the European Society of Cardiology on myocardial revascularization. PMID:25359405

Olivier, C B; Diehl, P; Bode, C; Moser, M

2014-11-01

230

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 todays 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

231

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 Schirmers test data showed that omega-3 fatty acid supplementation could significantly improve the Schirmers 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 Schirmers. 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

232

Induction and Subsequent Rupture of Aneurysms of the Circle of Willis after Radiation Therapy in Ehlers-Danlos Syndrome: A Plausible Hypothesis  

Microsoft Academic Search

Summary: Multiple aneurysms of the circle of Willis developed in a 62-year-old woman with Ehlers-Danlos syndrome (EDS) within 7 months of initiation of radiation therapy to the brain. A major response of the arterial wall to radiation damage is the formation of collagen. In patients with EDS, in whom there is a defect in collagen, this repair is incomplete. We

Andrei I. Holodny; Michael Deck; Carol K. Petito

233

The role of aspirin resistance on outcome in patients with acute coronary syndrome and the effect of clopidogrel therapy in the prevention of major cardiovascular events  

Microsoft Academic Search

Background: Aspirin resistance may increase up to more then threefold the risk of major cardiovascular events (MACE) in patients with\\u000a stable coronary artery disease.\\u000a \\u000a \\u000a Aim:The aim of our study was to determine; the prevalence of aspirin resistance in patients with acute coronary syndromes, the\\u000a role of aspirin resistance on outcome in the follow-up and the effect of clopidogrel therapy in

Burak Pamukcu; Huseyin Oflaz; Aytac Oncul; Berrin Umman; Fehmi Mercanoglu; Mustafa Ozcan; Mehmet Meric; Yilmaz Nisanci

2006-01-01

234

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

235

Central sensitization does not identify patients with carpal tunnel syndrome who are likely to achieve short-term success with physical therapy  

Microsoft Academic Search

The aim of the current study was to identify whether hyperexcitability of the central nervous system is a prognostic factor\\u000a for individuals with carpal tunnel syndrome (CTS) likely to experience rapid and clinical self-reported improvement following\\u000a a physical therapy program including soft tissue mobilization and nerve slider neurodynamic interventions. Women presenting\\u000a with clinical and electrophysiological findings of CTS were involved

Csar Fernndez-de-las-Peas; Joshua A. Cleland; Ricardo Ortega-Santiago; Ana Isabel de-la-Llave-Rincon; Almudena Martnez-Perez; Juan A. Pareja

2010-01-01

236

The Clinical Importance of Moderate/Severe Bone Marrow Fibrosis in Patients with Therapy-related Myelodysplastic Syndromes  

PubMed Central

Background The presence of moderate to severe bone marrow (BM) fibrosis has been shown to be an adverse feature in patients with primary myelodysplastic syndromes (MDS). However, the clinical importance of BM fibrosis is not clear in therapy-related MDS. Methods We retrieved all t-MDS cases (n=266) diagnosed at our hospital over a 10-year period (20032012). Reticulin and trichromestains were performed in cases in which BM fibrosis was suspected on initial evaluation of hematoxylin & eosin stained slide. BM fibrosis was graded according to European consensus guidelines, and a score of MF2/MF3 was defined as moderate/severe fibrosis. Result Moderate/severe BM fibrosis was found in 47 (17%) patients. Compared to 219 patients with no/mild BM fibrosis, the patients with moderate/severe fibrosis presented with severer thrombocytopenia (p=0.039), higher numbers of circulating blasts (p=0.051) but similar degrees of anemia and neutropenia, transfusion requirements, and similar incidences of hepatosplenomegaly and constitutional symptoms. Histological examination revealed a comparable BM cellularity and BM blast percentage, but markedly increased megakaryocytes (p<0.001) in the fibrotic group. Although the risk distribution of cytogenetic data was similar according to the New Comprehensive Cytogenetic Scoring criteria, ?5 and ?17 were more frequently observed in t-MDS with moderate/severe BM fibrosis (p=0.031 and p=0.043 respectively). With a median follow-up of 11.5 months, patients with moderate/severe BM fibrosis showed a similar risk of acute myeloid leukemia transformation, and a comparable overall survival in univariate and multivariate analyses. Conclusions Moderate/severe BM fibrosis in patients with t-MDS is associated with certain clinicopathological and genetic features. However, unlike the situation in patients with primary MDS, moderate/severe BM fibrosis does not add additional risk to patients with therapy-related MDS. PMID:23660629

Fu, Bin; OK, Chi Young; Goswami, Maitrayee; Xei, Wei; Jaso, Jesse M; Muzzafar, Tariq; Bueso-Ramos, Carlos; Verstovsek, Srdan; Garcia-Manero, Guillermo; Medeiros, L. Jeffrey; Wang, Sa A.

2014-01-01

237

Enzyme replacement therapy in an attenuated case of mucopolysaccharidosis type I (Scheie syndrome): a 6.5-year detailed follow-up.  

PubMed

We present the 6.5-year follow-up of a boy with Scheie syndrome whose therapy was initiated at age 2.5 years. Detailed anthropometric features, echocardiography, ophthalmologic and audiologic examinations, psychologic tests, joint range of motion, skeletal radiographs, ultrasound studies of liver and spleen volumes, urinary glycosaminoglycans, and antibodies were documented. After 6.5 years of enzyme replacement therapy, the patient experienced a decline in overall status, and the disease progressed significantly despite treatment. The patient's height at age 9 was equal to that at age 6. The patient developed heart insufficiency and a deterioration of airway flow. The patient's intelligence quotient remained unchanged, i.e., at the average level of 86 on the Terman-Merill Scale. Magnetic resonance imaging revealed cervical spinal canal stenosis and marked spinal cord compression with myelopathy. A worsening of carpal tunnel syndrome was also evident. Ophthalmologic evaluation revealed increased central corneal thickness and intraocular pressure. In audiologic assessment, the patient's results were similar to those after 3 years of treatment. The only benefit involved temporarily improved passive and active shoulder flexion. Overall, the benefit of enzyme replacement therapy with laronidase on Scheie syndrome appeared minimal. PMID:23127271

Jurecka, Agnieszka; Marucha, Jolanta; Jurkiewicz, El?bieta; R?d?y?ska-?wi?tkowska, Agnieszka; Tylki-Szyma?ska, Anna

2012-12-01

238

Intravenous tissue plasminogen activator therapy for an acute ischemic stroke patient with later diagnosed unilateral moyamoya syndrome.  

PubMed

We report the use of intravenous tissue plasminogen activator (t-PA) therapy in a 38-year-old patient who was later diagnosed with unilateral moyamoya syndrome. The patient had a sudden onset of unconsciousness, vomiting, dysarthria, and tetraparesis. A neurologic examination revealed consciousness disturbance, right central facial nerve palsy, dysarthria, and tetraparesis with bilateral exotropia and horizontal gaze palsy. A magnetic resonance imaging scan on admission did not reveal fresh cerebral infarction or hemorrhage, but magnetic resonance angiography revealed severe stenosis at the terminal portion of left internal carotid artery, the anterior cerebral arteries, and the right vertebral artery. We suspected infarction of brain stem. The patient was treated with intravenous t-PA approximately 2.5 hours after onset, and the patient demonstrated a remarkable recovery 1 day after onset and had only a minimal deficit at discharge (12 days after onset). Cerebral angiography 7 days after onset confirmed the diagnosis of moyamoya disease. The present case suggests that therapeutic intravenous t-PA may be applicable for an acute ischemic stroke patient coexisting with moyamoya disease after careful evaluation and discussion with patient and family. PMID:22832175

Yokoyama, Shota; Manabe, Yasuhiro; Fujii, Daiki; Ikeda-Sakai, Yasuko; Narai, Hisashi; Omori, Nobuhiko; Abe, Koji

2013-10-01

239

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

240

Dendritic cell functional improvement in a preclinical model of lentiviral-mediated gene therapy for Wiskott-Aldrich syndrome  

PubMed Central

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency caused by the defective expression of the WAS protein (WASP) in hematopoietic cells. It has been shown that dendritic cells (DCs) are functionally impaired in WAS patients and was?/? mice. We have previously demonstrated the efficacy and safety of a murine model of WAS gene therapy (GT), using stem cells transduced with a lentiviral vector. The aim of this study was to investigate whether GT can correct DC defects in was?/? mice. As DCs expressing WASP were detected in the secondary lymphoid organs of the treated mice, we tested the in vitro and in vivo function of bone marrow-derived DCs (BMDCs). The BMDCs showed efficient in vitro uptake of latex beads and Salmonella typhimurium. When BMDCs from the treated mice (GT BMDCs) and the was?/? mice were injected into wild type hosts, we found a higher number of cells that had migrated to the draining lymph nodes compared to mice injected with was?/? BMDCs. Finally, we found that OVA-pulsed GT BMDCs or vaccination with anti-DEC205 OVA fusion protein can efficiently induce antigen-specific T cell activation in vivo. These findings show that WAS GT significantly improves DC function, thus adding new evidence of the preclinical efficacy of lentiviral vector-mediated WAS GT. PMID:22189416

Catucci, Marco; Prete, Francesca; Bosticardo, Marita; Castiello, Maria Carmina; Draghici, Elena; Locci, Michela; Roncarolo, Maria Grazia; Aiuti, Alessandro; Benvenuti, Federica; Villa, Anna

2011-01-01

241

Dendritic cell functional improvement in a preclinical model of lentiviral-mediated gene therapy for Wiskott-Aldrich syndrome.  

PubMed

Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency caused by the defective expression of the WAS protein (WASP) in hematopoietic cells. It has been shown that dendritic cells (DCs) are functionally impaired in WAS patients and was(-/-) mice. We have previously demonstrated the efficacy and safety of a murine model of WAS gene therapy (GT), using stem cells transduced with a lentiviral vector (LV). The aim of this study was to investigate whether GT can correct DC defects in was(-/-) mice. As DCs expressing WASP were detected in the secondary lymphoid organs of the treated mice, we tested the in vitro and in vivo function of bone marrow-derived DCs (BMDCs). The BMDCs showed efficient in vitro uptake of latex beads and Salmonella typhimurium. When BMDCs from the treated mice (GT BMDCs) and the was(-/-) mice were injected into wild-type hosts, we found a higher number of cells that had migrated to the draining lymph nodes compared with mice injected with was(-/-) BMDCs. Finally, we found that ovalbumin (OVA)-pulsed GT BMDCs or vaccination of GT mice with anti-DEC205 OVA fusion protein can efficiently induce antigen-specific T-cell activation in vivo. These findings show that WAS GT significantly improves DC function, thus adding new evidence of the preclinical efficacy of LV-mediated WAS GT. PMID:22189416

Catucci, M; Prete, F; Bosticardo, M; Castiello, M C; Draghici, E; Locci, M; Roncarolo, M G; Aiuti, A; Benvenuti, F; Villa, A

2012-12-01

242

Diamel Therapy in Polycystic Ovary Syndrome Reduces Hyperinsulinaemia, Insulin Resistance, and Hyperandrogenaemia  

PubMed Central

For to determine the effect of Diamel on the insulin resistance, insulin sensitivity, and sexual hormones results in women with polycystic ovary syndrome (PCOS). A study was carried out on 37 patients with this disorder. A triple-blind clinical trial was designed in which the Diamel food supplement was compared with a placebo. The women with reproductive ages were randomly distributed in two groups, with 18 and 19 women respectively, and they took Diamel or placebo and were followed up during 6 months with clinical and biochemical evaluation. A significant decrease in the HOMA-IR from the initial value at six months was observed in the group with Diamel. The insulin sensitivity improved considerably in this group. The rate of menstrual recovery was higher in the group with Diamel, and two patients from this group obtained pregnancy. The hormone levels shows a significant decrease in testosterone at 3 months in the group with Diamel compared with the control group. The LH also decreases in the same group when comparing the start with 6 months.We concluded that the Diamel decreases insulin resistance and improves sensitivity to this hormone in women with PCOS, with improvement in the levels of LH and testosterone. PMID:22778733

Hernndez-Yero, Arturo; Santana Prez, Felipe; Ovies Carballo, Gisel; Cabrera-Rode, Eduardo

2012-01-01

243

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

244

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

PubMed

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 SAG 1.1 (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 N-methyl-d-aspartate (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

2013-09-01

245

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, Ozlem; Hekimoglu, Emine Rumeysa; Acar, Ugur; Demir, Muhammed Necati; Albayrak, Aynur; Kaymaz, Figen; Sobac?, Gungor; Delibas?, Tuncay

2014-01-01

246

Oxygen therapy in acute coronary syndrome: are the benefits worth the risk?  

PubMed

Oxygen supplementation is a standard treatment for all patients who present with acute coronary syndrome, regardless of oxygen saturation levels. Most of the data regarding the function of oxygen in myocardial infarction is based on a limited number of basic and clinical studies. We performed a systematic literature review that explores the basic and clinical data on the function of oxygen in ischaemic heart disease and myocardial infarction. This review discusses many aspects of oxygen treatment: (i) basic studies on the effects of oxygen in ischaemia and the potential cardiovascular effects of oxygen metabolites; (ii) clinical trials that have assessed the value of inhaled oxygen, supersaturated oxygen, and intracoronary injection of hyperoxaemic solutions in myocardial infarction; and (iii) the haemodynamic effects of oxygen in various clinical scenarios and its direct effects on the coronary vasculature. Our findings suggest that there are conflicting data on the effects of oxygen treatment. Further, the potential harmful effects of oxygen must be considered, particularly in myocardial infarction. These findings question the current guidelines and recommendations and emphasize the need for large clinical trials. PMID:23554440

Shuvy, Mony; Atar, Dan; Gabriel Steg, Philippe; Halvorsen, Sigrun; Jolly, Sanjit; Yusuf, Salim; Lotan, Chaim

2013-06-01

247

What is the value of growth hormone therapy in Prader Willi syndrome?  

PubMed

Prader Willi syndrome (PWS) is a genetic condition caused by loss of the paternal copy of a region of imprinted genes on chromosome 15. There is severe muscular hypotonia in the neonatal period, with the onset of hyperphagia and food-seeking behaviour in childhood. All individuals with PWS have developmental delay. Without careful control of food intake and the food environment, individuals with PWS become morbidly obese and are likely to die as young adults from the complications of obesity. The aims of growth hormone (GH) treatment in PWS are distinct from the use of GH in other conditions-although GH does increase final height in PWS, the main benefits of treatment are improved body composition and better exercise capacity, which can help with the aim of preventing obesity. GH trials in PWS have demonstrated improved muscle bulk, reduced fat mass and increased levels of physical activity. GH has also been demonstrated to improve attainment of developmental and cognitive milestones in children with PWS. GH treatment appears to change respiratory status in PWS, possibly because of growth of lymphoid tissue at the start of treatment. Respiratory assessment is recommended prior to, and just after starting GH treatment. Ideal age for starting GH is not clear, although there has been a trend towards starting at younger ages. It may be that GH treatment in childhood confers benefits into adult life. There are less data to support continuing GH treatment into adult life. PMID:24162007

Bridges, Nicola

2014-02-01

248

Hypertonic mannitol in the therapy of the acute respiratory distress syndrome.  

PubMed Central

Increased pulmonary artery pressure, an increase in pulmonary vascular resistance and an increase in physiologic dead space are consistent findings in patients with post-traumatic respiratory distress. Since mannitol has been shown to decrease renal vascular resistance following trauma, the effect of a bolus injection of 100 ml of 25% solution of this drug on pulmonary hemodynamics and physiologic dead space was investigated in 11 patients who had suffered multiple trauma. Five minutes after the injection, pulmonary vascular resistance fell (p less than .01), cardiac index increased (p less than .001) and physiologic dead space decreased (p less than .05). In contrast, the administration of 40 mg of furosemide produced no significant change in any of these parameters. Mannitol rapidly equilibrates in the extracellular space and exerts an osmotic effect across cell membranes. We postulate that the beneficial response to mannitol on the pulmonary vascular resistance and the improved perfusion of ventilated regions of the lung is due to a reduction in cell swelling and is not explainable by its diuretic effect. Improvement in the distribution of perfusion of pulmonary blood flow by mannitol may be a useful aid in the treatment of the post-traumatic form of the respiratory distress syndrome. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. PMID:324415

Powers, S R; Shah, D; Ryon, D; Newell, J; Ralph, C; Scovill, W; Dutton, R

1977-01-01

249

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

PubMed

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

2014-07-21

250

Oxidative stress and Down syndrome. Do antioxidants play a role in therapy?  

PubMed

Oxidative stress is a phenomenon associated with imbalance between production of free radicals and reactive metabolites (e.g. superoxide and hydrogen peroxide) and the antioxidant defences. Oxidative stress in individuals with Down syndrome (DS) has been associated with trisomy of the 21(st) chromosome resulting in DS phenotype as well as with various morphological abnormalities, immune disorders, intellectual disability, premature aging and other biochemical abnormalities. Trisomy 21 in patients with DS results in increased activity of an important antioxidant enzyme Cu/Zn superoxide dismutase (SOD) which gene is located on the 21(st) chromosome along with other proteins such as transcription factor Ets-2, stress inducing factors (DSCR1) and precursor of beta-amyloid protein responsible for the formation of amyloid plaques in Alzheimer disease. Mentioned proteins are involved in the management of mitochondrial function, thereby promoting mitochondrial theory of aging also in people with DS. In defence against toxic effects of free radicals and their metabolites organism has built antioxidant defence systems. Their lack and reduced function increases oxidative stress resulting in disruption of the structure of important biomolecules, such as proteins, lipids and nucleic acids. This leads to their dysfunctions affecting pathophysiology of organs and the whole organism. This paper examines the impact of antioxidant interventions as well as positive effect of physical exercise on cognitive and learning disabilities of individuals with DS. Potential therapeutic targets on the molecular level (oxidative stress markers, gene for DYRK1A, neutrophic factor BDNF) after intervention of natural polyphenols are also discussed. PMID:24908086

Muchov, J; Zit?anov, I; Dura?kov, Z

2014-11-27

251

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

252

Autonomic modulation and antiarrhythmic therapy in a model of long QT syndrome type 3  

PubMed Central

Aims Clinical observations in patients with long QT syndrome carrying sodium channel mutations (LQT3) suggest that bradycardia caused by parasympathetic stimulation may provoke torsades de pointes (TdP). ?-Adrenoceptor blockers appear less effective in LQT3 than in other forms of the disease. Methods and results We studied effects of autonomic modulation on arrhythmias in vivo and in vitro and quantified sympathetic innervation by autoradiography in heterozygous mice with a knock-in deletion (?KPQ) in the Scn5a gene coding for the cardiac sodium channel and increased late sodium current (LQT3 mice). Cholinergic stimulation by carbachol provoked bigemini and TdP in freely roaming LQT3 mice. No arrhythmias were provoked by physical stress, mental stress, isoproterenol, or atropine. In isolated, beating hearts, carbachol did not prolong action potentials per se, but caused bradycardia and rate-dependent action potential prolongation. The muscarinic inhibitor AFDX116 prevented effects of carbachol on heart rate and arrhythmias. ?-Adrenoceptor stimulation suppressed arrhythmias, shortened rate-corrected action potential duration, increased rate, and minimized difference in late sodium current between genotypes. ?-Adrenoceptor density was reduced in LQT3 hearts. Acute ?-adrenoceptor blockade by esmolol, propranolol or chronic propranolol in vivo did not suppress arrhythmias. Chronic flecainide pre-treatment prevented arrhythmias (all P < 0.05). Conclusion Cholinergic stimulation provokes arrhythmias in this model of LQT3 by triggering bradycardia. ?-Adrenoceptor density is reduced, and ?-adrenoceptor blockade does not prevent arrhythmias. Sodium channel blockade and ?-adrenoceptor stimulation suppress arrhythmias by shortening repolarization and minimizing difference in late sodium current. PMID:20110334

Fabritz, Larissa; Damke, Dierk; Emmerich, Markus; Kaufmann, Susann G.; Theis, Kathrin; Blana, Andreas; Fortmuller, Lisa; Laakmann, Sandra; Hermann, Sven; Aleynichenko, Elena; Steinfurt, Johannes; Volkery, Daniela; Riemann, Burkhard; Kirchhefer, Uwe; Franz, Michael R.; Breithardt, Gunter; Carmeliet, Edward; Schafers, Michael; Maier, Sebastian K.G.; Carmeliet, Peter; Kirchhof, Paulus

2010-01-01

253

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

254

Subchronic administration and combination metabotropic glutamate and GABAB receptor drug therapy in fragile X syndrome.  

PubMed

The most common cause of inherited mental retardation, fragile X syndrome, results from a triplet repeat expansion in the FMR1 gene and loss of the mRNA binding protein, fragile X mental retardation protein (FMRP). In the absence of FMRP, signaling through group I metabotropic glutamate receptors (mGluRs) is enhanced. We previously proposed a mechanism whereby the audiogenic seizures exhibited by FMR1 null mice result from an imbalance in excitatory mGluR and inhibitory GABA(B) receptor (GABA(B)R) signaling (Mol Pharmacol 76:18-24, 2009). Here, we tested the mGluR5-positive allosteric modulator 3-cyano-N-(1,3-diphenyl-1H-pyrazol-5-yl)benzamide (CDPPB), the mGluR5 inverse agonist 2-methyl-6-(phenylethynyl)pyridine (MPEP), and GABA(B) receptor agonists, alone and in combination on receptor protein expression and audiogenic seizures in FMR1 mice. Single doses of MPEP (30 mg/kg), the GABA(B)R orthosteric agonist R-baclofen (1 mg/kg), or the GABA(B)R-positive allosteric modulator N,N'-dicyclopentyl-2-(methylthio)-5-nitro-4,6-pyrimidine diamine (GS-39783) (30 mg/kg), reduced the incidence of seizures. However, when administered subchronically (daily injections for 6 days), MPEP retained its anticonvulsant activity, whereas R-baclofen and GS-39783 did not. When administered at lower doses that had no effect when given alone, a single injection of MPEP plus R-baclofen also reduced seizures, but the effect was lost after subchronic administration. We were surprised to find that subchronic treatment with R-baclofen also induced tolerance to a single high dose of MPEP. These data demonstrate that tolerance develops rapidly to the antiseizure properties of R-baclofen alone and R-baclofen coadministered with MPEP, but not with MPEP alone. Our findings suggest that cross-talk between the G-protein signaling pathways of these receptors affects drug efficacy after repeated treatment. PMID:21636656

Pacey, Laura K K; Tharmalingam, Sujeenthar; Hampson, David R

2011-09-01

255

Paraneoplastic Syndromes  

PubMed Central

Neoplasms can produce a variety of remote effects on the host; these are referred to as paraneoplastic syndromes. The syndromes may affect any of the systems of the body, may precede or follow the diagnosis of the underlying neoplasm, and may or may not parallel the course of the neoplasm in severity. The diagnosis of and therapy for these syndromes can be challenging to a physician, but successful therapy may bring about worthwhile relief for the patient. In addition, the syndromes and the substances that cause them are sometimes useful in diagnosing and in following the course of certain neoplasms. Perhaps of greater importance, study of these remote effects of neoplasia may shed light on the nature of the neoplastic process itself. PMID:6990627

Stolinsky, David C.

1980-01-01

256

The General Weakness Syndrome Therapy (GymNAST) study: protocol for a cohort study on recovery on walking function  

PubMed Central

Introduction Critical illness myopathy (CIM) and polyneuropathy (CIP) are common complications of critical illness that frequently occur together. Both cause so called intensive care unit (ICU)-acquired muscle weakness. This weakness of limb muscles increases morbidity and delay rehabilitation and recovery of walking ability. Although full recovery has been reported people with severe weakness may take months to improve walking. Focused physical rehabilitation of people with ICU-acquired muscle weakness is therefore of great importance. However, although physical rehabilitation is common, detailed knowledge about the pattern and the time course of recovery of walking function are not well understood. Therefore, the aim of the General Weakness Syndrome Therapy (GymNAST) study is to describe the time course of recovery of walking function and other activities of daily living in these patients. Methods and analysis We conduct a prospective cohort study of people with ICU-acquired muscle weakness with defined diagnosis of CIM or CIP. Based on our sample size calculation, approximately 150 patients will be recruited from the ICU of our hospital in Germany. Amount and content of physical rehabilitation, clinical tests for example, muscle strength and motor function and neuropsychological assessments will be used as independent variables. The primary outcomes will include recovery of walking function and mobility. Secondary outcomes will include global motor function, activities in daily life and participation. Ethics and dissemination The study is being carried out in agreement with the Declaration of Helsinki and conducted with the approval of the local medical Ethics Committee (Landesrztekammer Sachsen, Germany, reference number EK-BR-32/13-1) and with the understanding and written consent of each patient's guardian. The results of this study will be published in peer-reviewed journals and disseminated to the medical society and general public. PMID:25344484

Mehrholz, Jan; Muckel, Simone; Oehmichen, Frank; Pohl, Marcus

2014-01-01

257

Combined intravesical sodium hyaluronate/chondroitin sulfate therapy for interstitial cystitis/bladder pain syndrome: a prospective study  

PubMed Central

Objectives: The aim of this study was to verify the efficacy and safety of intravesical treatment combining sodium hyaluronate (HA) and chondroitin sulfate (CS) in patients with interstitial cystitis/bladder pain syndrome (IC/BPS). Methods: Between February 2010 and May 2011, 20 consecutive women with IC/BPS were treated with intravesical instillations containing sodium HA (1.6%; 800 mg/50 ml) and sodium CS (2%; 1 g/50 ml) weekly for the first month, biweekly for the second month, and then monthly for at least 3 months. Before and after treatment, all patients filled in the Interstitial Cystitis Symptom Index and Problem Index (ICSI/ICPI), the Patient Health Questionnaire 9 and the Pelvic Pain and Urgency/Frequency Patient Symptom Scale (PUF). Treatment efficacy was assessed by comparing the pre- and post-treatment mean scores of the three questionnaires using Students t test (p value <0.05 was considered significant). Results: Statistically significant mean decreases in ICSI (from 13.0 to 9.3; p = 0.0003), ICPI (from 11.35 to 8.85; p = 0.0078) and PUF (from 20.0 to 15.75; p = 0.0007) questionnaire scores were seen. No cases of side effects or complications were observed. The mean follow up was 5 months. Conclusions: Despite the limitations of this study, the outcomes confirmed the role of combination therapy with HA and CS as a safe and effective option for the treatment of IC/BPS. Further randomized controlled studies with a higher number of patients and a longer follow-up period are needed to confirm these results. PMID:23904856

Giberti, Claudio; Cortese, Pierluigi; Schenone, Maurizio

2013-01-01

258

Utilization of evidence-based therapy for acute coronary syndrome in high-income and low/middle-income countries.  

PubMed

Limited data exist regarding the management of patients with acute coronary syndrome (ACS) in high-income countries compared with low/middle-income countries. We aimed to compare in-hospital trends of revascularization and prescription of medications at discharge in patients with ACS from high-income (Canada and United States) and low/middle-income (India, Iran, Pakistan, and Tunisia) countries. Data from a double-blind, placebo-controlled, randomized trial investigating the effect of bupropion on smoking cessation in patients after an enzyme-positive ACS was used for our study. A total of 392 patients, 265 and 127 from high-income and from low/middle-income countries, respectively, were enrolled. Patients from high-income countries were older, and were more likely to have diagnosed hypertension and dyslipidemia. During the index hospitalization, patients from high-income countries were more likely to be treated by percutaneous coronary intervention (odds ratio [OR] 19.7, 95% confidence interval [CI] 10.5 to 37.0). Patients with ST elevation myocardial infarction from high-income countries were more often treated by primary percutaneous coronary intervention (OR 16.3, 95% CI 6.3 to 42.3) in contrast with thrombolytic therapy (OR 0.24, 95% CI 0.14 to 0.41). Patients from high-income countries were also more likely to receive evidence-based medications at discharge (OR 2.32, 95% CI 1.19 to 4.52, a composite of aspirin, clopidogrel, and statin). In conclusion, patients with ACS in low/middle-income countries were less likely to be revascularized and to receive evidence-based medications at discharge. Further studies are needed to understand the underutilization of procedures and evidence-based medications in low/middle-income countries. PMID:24440324

Shimony, Avi; Grandi, Sonia M; Pilote, Louise; Joseph, Lawrence; O'Loughlin, Jennifer; Paradis, Gilles; Rinfret, Stphane; Sarrafzadegan, Nizal; Adamjee, Nasreen; Yadav, Rakesh; Gamra, Habib; Diodati, Jean G; Eisenberg, Mark J

2014-03-01

259

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 15 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

260

Growth Hormone Research Society Workshop Summary: Consensus Guidelines for Recombinant Human Growth Hormone Therapy in Prader-Willi Syndrome  

PubMed Central

Context: Recombinant human GH (rhGH) therapy in Prader-Willi syndrome (PWS) has been used by the medical community and advocated by parental support groups since its approval in the United States in 2000 and in Europe in 2001. Its use in PWS represents a unique therapeutic challenge that includes treating individuals with cognitive disability, varied therapeutic goals that are not focused exclusively on increased height, and concerns about potential life-threatening adverse events. Objective: The aim of the study was to formulate recommendations for the use of rhGH in children and adult patients with PWS. Evidence: We performed a systematic review of the clinical evidence in the pediatric population, including randomized controlled trials, comparative observational studies, and long-term studies (>3.5 y). Adult studies included randomized controlled trials of rhGH treatment for ? 6 months and uncontrolled trials. Safety data were obtained from case reports, clinical trials, and pharmaceutical registries. Methodology: Forty-three international experts and stakeholders followed clinical practice guideline development recommendations outlined by the AGREE Collaboration (www.agreetrust.org). Evidence was synthesized and graded using a comprehensive multicriteria methodology (EVIDEM) (http://bit.ly.PWGHIN). Conclusions: Following a multidisciplinary evaluation, preferably by experts, rhGH treatment should be considered for patients with genetically confirmed PWS in conjunction with dietary, environmental, and lifestyle interventions. Cognitive impairment should not be a barrier to treatment, and informed consent/assent should include benefit/risk information. Exclusion criteria should include severe obesity, uncontrolled diabetes mellitus, untreated severe obstructive sleep apnea, active cancer, or psychosis. Clinical outcome priorities should vary depending upon age and the presence of physical, mental, and social disability, and treatment should be continued for as long as demonstrated benefits outweigh the risks. PMID:23543664

Tony, Michele; Hoybye, Charlotte; Allen, David B.; Tauber, Maithe; Christiansen, Jens Sandahl; Ambler, Geoffrey R.; Battista, Renaldo; Beauloye, Veronique; Berall, Glenn; Biller, Beverly M. K.; Butler, Merlin G.; Cassidy, Suzanne B.; Chihara, Kazuo; Cohen, Pinchas; Craig, Maria; Farholt, Stense; Goetghebeur, Mireille; Goldstone, Anthony P.; Greggi, Tiziana; Grugni, Graziano; Hokken-Koelega, Anita C.; Johannsson, Gudmundur; Johnson, Keegan; Kemper, Alex; Kopchick, John J.; Malozowski, Saul; Miller, Jennifer; Mogul, Harriette R.; Muscatelli, Francoise; Nergardh, Ricard; Nicholls, Robert D.; Radovick, Sally; Rosenthal, M. Sara; Sipila, Ilkka; Tarride, Jean-Eric; Vogels, Annick; Waters, Michael J.

2013-01-01

261

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 Hjbjerg Gravholt; Svend Juul; Rune Weis Naeraa; Jan Hansen

1998-01-01

262

Rationale and design of the Anti-Xa Therapy to Lower cardiovascular events in Addition to standard therapy in Subjects with Acute Coronary SyndromeThrombolysis in Myocardial Infarction 51 (ATLAS-ACS 2 TIMI 51) trial: A randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of rivaroxaban in subjects with acute coronary syndrome  

Microsoft Academic Search

BACKGROUND: Although therapy with aspirin or aspirin plus a thienopyridine reduces the incidence of long-term adverse cardiovascular events among patients with acute coronary syndrome (ACS), there remains a significant residual risk of cardiovascular death, recurrent myocardial infarction (MI), and stroke. In a phase 2 trial (ClinicalTrials.gov NCT00402597) in which the addition of the factor Xa inhibitor rivaroxaban was compared with

C. Michael Gibson; Jessica L. Mega; Paul Burton; Shinya Goto; Freek Verheugt; Christoph Bode; Alexei Plotnikov; Xiang Sun; Nancy Cook-Bruns; Eugene Braunwald

2011-01-01

263

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

264

Health care utilization and risk of infection and bleeding among patients with myelodysplastic syndromes with/without transfusions, and with/without active therapy.  

PubMed

This study utilized claims data from a national US commercial health insurer to examine rates of cytopenia-related complications (significant bleeding, infection) and health care utilization (emergency room visits, inpatient hospitalizations) among patients with myelodysplastic syndromes (MDS) within predefined periods of transfusion activity and active therapy. Periods with no transfusions, regardless of relationship to treatment intervention, were associated with lower rates of cytopenia-related complications. These data suggest that eliminating or reducing the need for transfusions may help to reduce MDS-related medical problems, and treatment toward that goal should be considered in patients with MDS needing transfusions. PMID:23841504

Smith, B Douglas; Mahmoud, Dalia; Dacosta-Byfield, Stacey; Rosen, Virginia M

2014-05-01

265

Health care utilization and risk of infection and bleeding among patients with myelodysplastic syndromes with/without transfusions, and with/without active therapy  

PubMed Central

This study utilized claims data from a national US commercial health insurer to examine rates of cytopenia-related complications (significant bleeding, infection) and health care utilization (emergency room visits, inpatient hospitalizations) among patients with myelodysplastic syndromes (MDS) within predefined periods of transfusion activity and active therapy. Periods with no transfusions, regardless of relationship to treatment intervention, were associated with lower rates of cytopenia-related complications. These data suggest that eliminating or reducing the need for transfusions may help to reduce MDS-related medical problems, and treatment toward that goal should be considered in patients with MDS needing transfusions. PMID:23841504

Smith, B. Douglas; Mahmoud, Dalia; Dacosta-Byfield, Stacey; Rosen, Virginia M.

2014-01-01

266

Paroxysmal Nocturnal Hemoglobinuria Following Alemtuzumab Immunosuppressive Therapy for Myelodysplastic Syndrome and Complicated by Recurrent Life-Threatening Thrombosis Despite Anticoagulation: Successful Intervention with Eculizumab and Fondaparinux  

PubMed Central

The pathogenesis of paroxysmal nocturnal hemoglobinuria (PNH) is not fully understood. We report a patient with myelodysplastic syndrome who developed symptomatic PNH following treatment with alemtuzumab. A small PNH clone, identified prior to alemtuzumab, expanded resulting in hemolytic anemia and recurrent CNS thromboses despite anticoagulation. Remission was achieved with eculizumab and fondaparinux therapy. Alemtuzumab has been associated with the development of glycosylphosphotidylinositol negative cells, but its clinical significance has been unclear. Our case emphasizes its potential clinical importance. Future studies are necessary to expand our understanding of this rare disease entity and improve its management. PMID:19836075

Cheng, Kit L.; Brody, Judith; Warshall, Craig E.; Sloand, Elaine M.; Allen, Steven L.

2009-01-01

267

Renal replacement therapy in infants and children with hepatorenal syndrome awaiting liver transplantation: A case-control study.  

PubMed

Limited data on short- and long-term outcomes of renal replacement therapy (RRT) in pediatric liver transplantation (LT) patients exist. We evaluated risk factors for RRT in pediatric LT recipients with hepatorenal syndrome (HRS) and described the outcomes. We performed a single-center, case-control study of LT recipients who required RRT for HRS from 1999 to 2011. Three controls who did not receive RRT were matched with each case on the basis of age, diagnosis, and LT date. We identified 8 recipients among 133 recipients of 152 LT cases [6%, 95% confidence interval?=?2%-10%; mean age?=?7.7 years, range?=?0.5-19.8 years) who required RRT before LT for HRS. Four patients were <1 year old and weighed 5.6 to 6.6 kg. Biliary atresia was the most common LT indication. Cases had higher Model for (Pediatric) End-Stage Liver Disease scores at listing (26 versus 16, P?=?0.01) and lower glomerular filtration rates (GFRs; 15 versus 102 mL/minute/1.73 m(2) , P??0.99; and 97 versus 114 mL/minute/1.73 m(2) at the most recent follow-up, P?=?0.09). The case survival rates were 75% and 63% at 1 month and 1 year, respectively; 4 cases required antihypertensives and diuretics 1 month after LT, but at the last follow-up, only 1 case required antihypertensive therapy, and none required diuretics. In conclusion, pediatric patients with HRS, including infants, benefit from RRT. Although HRS decreases survival, patients with HRS who undergo LT generally recover renal function within 1 month that persists during long-term follow-up. Liver Transpl 20:1468-1474, 2014. 2014 AASLD. PMID:25179803

Elizabeth Parsons, C; Nelson, Raoul; Book, Linda S; Kyle Jensen, M

2014-12-01

268

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

269

Protocol for the PACE trial: A randomised controlled trial of adaptive pacing, cognitive behaviour therapy, and graded exercise as supplements to standardised specialist medical care versus standardised specialist medical care alone for patients with the chronic fatigue syndrome\\/myalgic encephalomyelitis or encephalopathy  

Microsoft Academic Search

BACKGROUND: Chronic fatigue syndrome (CFS, also called myalgic encephalomyelitis\\/encephalopathy or ME) is a debilitating condition with no known cause or cure. Improvement may occur with medical care and additional therapies of pacing, cognitive behavioural therapy and graded exercise therapy. The latter two therapies have been found to be efficacious in small trials, but patient organisations' surveys have reported adverse effects.

Peter D White; Michael C Sharpe; Trudie Chalder; Julia C DeCesare; Rebecca Walwyn

2007-01-01

270

Vaccine Therapy Plus Immune Adjuvant in Treating Patients With Chronic Myeloid Leukemia, Acute Myeloid Leukemia, or Myelodysplastic Syndrome  

ClinicalTrials.gov

Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Myeloid Leukemia in Remission; Chronic Phase Chronic Myelogenous Leukemia; Previously Treated Myelodysplastic Syndromes; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Relapsing Chronic Myelogenous Leukemia

2013-01-04

271

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

272

Fragile X Syndrome What is fragile X syndrome?  

E-print Network

be present. Can fragile X syndrome be treated? There is no cure for fragile X syndrome. However, early. These therapies may also be useful in addressing issues of shyness, depression, and worry in individuals

Bordenstein, Seth

273

Effect of combining therapy with traditional chinese medicine-based psychotherapy and herbal medicines in women with menopausal syndrome: a randomized controlled clinical trial.  

PubMed

This multicenter, randomized, controlled clinical study was designed to address the effectiveness of combined traditional-Chinese-medicine- (TCM-) based psychotherapy and Chinese herbal medicine (CHM) in the treatment of menopausal syndrome. Altogether 424 eligible women diagnosed as menopausal syndrome and categorized as Kidney-Yin/Kidney-Yang deficiency pattern in TCM were randomly assigned into 4 groups and accepted TCM-based psychotherapy (PSY), CHM, PSY + CHM, or placebo therapies, respectively, for 12 weeks, and another 12 weeks were taken as the followup. Kupperman Index (KI) and the Menopause-Specific Quality of Life (MENQOL) with its four subscales (vasomotor, physical, psychosocial, and sexual) were employed for efficacy assessment. Results showed that 400 participants completed 12-week treatment, of which 380 finished the record of KI and MENQOF at week 24. The average adjusted number of KI score decreased between baseline and 12 weeks in all groups. Statistically significant differences were detected in the average adjusted change between the PSY + CHM group and placebo at overall time points (P < 0.05). No severe adverse events occurred in each group and no significant differences were indicated between any of the three groups and placebo in adverse event proportion. We concluded that TCM psychotherapy combined with CHM has a favorable outcome in treating menopausal syndrome. PMID:23304198

Yang, Hongyan; Yang, Jing; Wen, Zehuai; Zha, Qinglin; Nie, Guangning; Huang, Xuchun; Zhang, Chunlin; Lu, Aiping; Jiang, Miao; Wang, Xiaoyun

2012-01-01

274

Effect of Combining Therapy with Traditional Chinese Medicine-Based Psychotherapy and Herbal Medicines in Women with Menopausal Syndrome: A Randomized Controlled Clinical Trial  

PubMed Central

This multicenter, randomized, controlled clinical study was designed to address the effectiveness of combined traditional-Chinese-medicine- (TCM-) based psychotherapy and Chinese herbal medicine (CHM) in the treatment of menopausal syndrome. Altogether 424 eligible women diagnosed as menopausal syndrome and categorized as Kidney-Yin/Kidney-Yang deficiency pattern in TCM were randomly assigned into 4 groups and accepted TCM-based psychotherapy (PSY), CHM, PSY + CHM, or placebo therapies, respectively, for 12 weeks, and another 12 weeks were taken as the followup. Kupperman Index (KI) and the Menopause-Specific Quality of Life (MENQOL) with its four subscales (vasomotor, physical, psychosocial, and sexual) were employed for efficacy assessment. Results showed that 400 participants completed 12-week treatment, of which 380 finished the record of KI and MENQOF at week 24. The average adjusted number of KI score decreased between baseline and 12 weeks in all groups. Statistically significant differences were detected in the average adjusted change between the PSY + CHM group and placebo at overall time points (P < 0.05). No severe adverse events occurred in each group and no significant differences were indicated between any of the three groups and placebo in adverse event proportion. We concluded that TCM psychotherapy combined with CHM has a favorable outcome in treating menopausal syndrome. PMID:23304198

Yang, Hongyan; Yang, Jing; Wen, Zehuai; Zha, Qinglin; Nie, Guangning; Huang, Xuchun; Zhang, Chunlin; Lu, Aiping; Jiang, Miao; Wang, Xiaoyun

2012-01-01

275

Detection of the Metabolic Syndrome in Schizophrenia and Implications for Antipsychotic Therapy: Is There a Role for Folate?  

PubMed Central

In general, presence of the metabolic syndrome is associated with significant cardiovascular mortality and represents a growing public health concern in the United States. Patients with a schizophrenia have a three times greater risk of death compared to the general population, with cardiovascular disease being the most common cause of this mortality. Use of the atypical antipsychotics (AAPs) to treat schizophrenia contributes significantly to this cardiovascular disease risk. While currently several different clinical guidelines exist to monitor for the metabolic consequences of AAP use, implementation is lacking. Due to the under monitoring of side effects and the lack of alternative treatment choices in schizophrenia, research has focused on the identification of various biomarkers and pharmacogenomic targets to focus on those at greatest risk for metabolic syndrome, thus aiming to increase the efficacy and minimize the side effects of the AAPs. This has led to several different lines of research. This manuscript focuses on summarizing the differing metabolic syndrome criteria, monitoring guidelines for AAPs and the role of folic acid as it relates to metabolic syndrome within the schizophrenia population. It will concentrate not only on the pharmacogenomics of folic acid metabolism, but also its epigenetic interaction with the environment. From this work, genetic variation within both the methylenetetrahydrofolate reductase (MTHFR) and catechol-o-methyl transferase (COMT) genes has been associated with increased metabolic syndrome risk in schizophrenia patients treated with AAPs. Furthermore, the combination of folate pharmacogenetics and epigenetics has uncovered relationships between methylation, schizophrenia disease, treatment type and metabolic syndrome. Despite the several areas of biomarker research for schizophrenia related metabolic syndrome, translation to the clinical setting is still lacking and further studies are needed to bridge this gap. Future folate supplementation research may prove to be an easy and effective clinical tool for the prevention and/or treatment of metabolic syndrome associated with AAP treatment, but clearly more work needs to be done in this area. PMID:23341251

Burghardt, Kyle J; Ellingrod, Vicki L

2014-01-01

276

Acute Respiratory Distress Syndrome in Lemierre's Syndrome  

PubMed Central

Lemierre's syndrome is an infectious disease defined by the presence of septic thrombophlebitis with associated embolic phenomenon, most commonly to the lungs. Here we present two cases from a single institution of acute respiratory distress syndrome (ARDS) developing as a result of Lemierre's syndrome in previously healthy young adult men. ARDS can occur as a consequence of pulmonary septic emboli and sepsis, both of which are well-described consequences of Lemierre's syndrome. We describe important diagnostic and management considerations in the care of patients with hypoxemic respiratory failure and Lemierre's syndrome. Essential components of management include prompt antibiotic therapy, lung-protective ventilation strategies, and supportive care. PMID:25143837

Hein, Paul N.; Soghikian, Maida V.; Bhangoo, Munveer S.

2014-01-01

277

A randomized open-label comparative study of conventional therapy versus mizoribine onlay therapy in patients with steroid-resistant nephrotic syndrome (postmarketing survey)  

Microsoft Academic Search

Background A previous double-blind 24-week clinical trial of mizoribine (MZ) vs placebo in steroid-resistant primary nephrotic syndrome (SRPNS) showed that MZ was more effective than placebo in reducing the rate of deterioration of renal function. The present study was conducted to evaluate the efficacy and safety of MZ in patients with SRPNS after 2 years treatment. Methods A multicenter randomized

Toshiaki Shibasaki; Akio Koyama; Akira Hishida; Eri Muso; Gengo Osawa; Hideaki Yamabe; Hideo Shiiki; Hirofumi Makino; Hiroshi Sato; Isao Ishikawa; Kenji Maeda; Kimio Tomita; Masaaki Arakawa; Masashi Ishida; Masashi Sato; Mitsumasa Nagase; Naoki Kashihara; Noriaki Yorioka; Takao Koike; Takao Saito; Takashi Harada; Tetsuya Mitarai; Tetsuzo Sugisaki; Toshihiko Nagasawa; Yasuhiko Tomino; Yoshihisa Nojima; Yutaka Kobayashi; Osamu Sakai

2004-01-01

278

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

279

Efficacy of clopidogrel reloading in patients with acute coronary syndrome undergoing percutaneous coronary intervention during chronic clopidogrel therapy (from the Antiplatelet therapy for Reduction of MYocardial Damage during Angioplasty [ARMYDA-8 RELOAD-ACS] trial).  

PubMed

Whether an additional clopidogrel load in patients receiving chronic clopidogrel therapy and undergoing percutaneous coronary intervention (PCI) for acute coronary syndrome (ACS) is associated with clinical benefit has not been well characterized. The aim of the present study was to evaluate, in a randomized protocol, the safety and effectiveness of clopidogrel reload for patients with ACS undergoing PCI in the background of chronic clopidogrel therapy. A total of 242 patients with non-ST-segment elevation ACS with >10days of clopidogrel therapy randomly received a 600-mg loading dose of clopidogrel 4to 8 hours before PCI (n= 122) or placebo (n= 120). The primary end point was the 30-day incidence of major adverse cardiac events (death, myocardial infarction, target vessel revascularization). The primary end point occurred in 4.1% of patients in the reload arm versus 14.1% in the placebo arm (odds ratio 0.26, 95% confidence interval 0.10 to 0.73, p= 0.013). This benefit in the reload arm was mainly from the prevention of periprocedural myocardial infarction (4.1% vs 13%, p= 0.02) and was paralleled by lower periprocedural platelet reactivity. The aggregometry data were consistent with the clinical outcome. No difference was found in the bleeding outcomes between the 2 groups. In conclusion, the results from the Antiplatelet therapy for Reduction of MYocardial Damage during Angioplasty (ARMYDA-8 RELOAD-ACS) trial have shown a significant clinical benefit from reloading patients with ACS receiving chronic clopidogrel therapy before PCI. These data might be relevant in clinical practice, given the large number of patients with ACS who are still currently treated with clopidogrel during PCI. PMID:23601577

Patti, Giuseppe; Pasceri, Vincenzo; Mangiacapra, Fabio; Colonna, Giuseppe; Vizzi, Vincenzo; Ricottini, Elisabetta; Montinaro, Antonio; D'Ambrosio, Andrea; Wijns, William; Barbato, Emanuele; Di Sciascio, Germano

2013-07-15

280

Pacemaker twiddler's syndrome (rotation of the pacemaker around the electrode cable, a rare complication of pacemaker therapy).  

PubMed

Rotation of the pacemaker generator around the electrode cable (i.e. twiddler's syndrome) was observed by the authors in six cases during the implantation of 4250 pacemakers. Twiddler's syndrome developed in three cases following implantation and in three cases after the replacement of the pacemaker. As a result of the rotation of the device, displacement of the electrode occurred in all cases. The factors predisposing to rotation of the device were as follows: (i) a loose, dilated pocket in 5 cases; (ii) seroma formation around the device in 2 cases; (iii) manipulation with the pacemaker in one case. For treating twiddler's syndrome, reimplantation was performed, fashioning a small and tight pocket for the device and fixing it by transfixing sutures. After reimplantation, the patients became complaint free, no recurrences occurred. PMID:2596244

Solti, F; Moravcsik, E; Rnyi-Vmos, F; Szab, Z

1989-01-01

281

The effects of vagus nerve stimulation therapy on patients with intractable seizures and either LandauKleffner syndrome or autism  

Microsoft Academic Search

Acquired and developmental comorbid conditions, including language and behavioral disorders, are often associated with epilepsy. Although the relationship between these disorders is not fully understood, their close association may indicate that they share common features, suggesting that these conditions may respond to the same therapies. Not only has vagus nerve stimulation (VNS) therapy been proven to reduce the frequency of

Yong D. Park

2003-01-01

282

The impact of initial fludarabine therapy on transformation to Richter syndrome or prolymphocytic leukemia in patients with chronic lymphocytic leukemia: analysis of an intergroup trial (CALGB 9011).  

PubMed

The impact of initial fludarabine therapy on transformation to Richter syndrome (RS) or prolymphocytic leukemia (PLL) in patients with chronic lymphocytic leukemia (CLL) is uncertain. We studied the outcomes of 521 patients with CLL who were randomized to initial fludarabine (F), chlorambucil (C) or F + C therapy on an intergroup trial (CALGB 9011). RS developed in 34 (7%) patients and PLL in 10 (2%). RS and PLL occurred in 14 (7%) and three (2%) of 188 patients randomized to F; nine (5%) and four (2%) of 191 patients treated with C; and 11 (8%) and three (2%) of 142 receiving F + C, respectively. Four percent of the 206 patients with Rai stage III/IV developed PLL, compared to only 1% of the 315 patients with Rai stage I/II (p = 0.02). Initial fludarabine therapy in patients with CLL did not impact transformation to RS or PLL, nor were any other baseline characteristics predictive for such transformation in this series. PMID:22897733

Solh, Melhem; Rai, Kanti R; Peterson, Bercedis L; Kolitz, Jonathan E; Appelbaum, Frederick R; Tallman, Martin S; Belch, Andrew; Larson, Richard A; Morrison, Vicki A

2013-02-01

283

The impact of a multidisciplinary approach on response rate of mandibular advancing device therapy in patients with obstructive sleep apnoea syndrome.  

PubMed

The aim of the present study was to evaluate the importance of a multidisciplinary approach on increasing the response ratio expectation to mandibular advancing device (MAD) therapy in patients with obstructive sleep apnoea syndrome, especially in severe cases. Forty-two mild-to-severe OSAS patients were selected, after comprehensive evaluation by neurologists, otorhinolaryngologists and orthodontists, and treated with a Somnodent device. Six months later, a polysomnographic exam with the MAD in situ was performed. The paired t-test evaluated the effectiveness of therapy and the results were compared with data from systematic reviews. The average treatment response was statistically significant for the apnoea/hypopnea index (AHI) and oxygen desaturation index and was higher than the outcomes presented in literature. An optimum therapy response (AHI < 5) was observed in 53% of patients (40% in severe OSAS) and a good response (AHI < 10) in 73% of patients (50% in severe OSAS). The Somnodent device was effective and the multidisciplinary patient selection improved the response ratio compared to that reported by previous systematic reviews. PMID:24227900

Milano, F; Mondini, S; Billi, M C; Gobbi, R; Gracco, A; Sorrenti, G

2013-10-01

284

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

285

Elevated reticulocyte count a clue to the diagnosis of haemolytic-uraemic syndrome (HUS) associated with gemcitabine therapy for metastatic duodenal papillary carcinoma: a case report  

PubMed Central

In adults, the haemolytic-uraemic syndrome (HUS) is associated with probable causative factors in the minority of all cases. Cytotoxic drugs are one of these potential causative agents. Although metastatic cancer by itself is a recognized risk-factor for the development of HUS, therapy with mitomycin-C, with cis-platinum, and with bleomycin carries a significant, albeit extremely small, risk for the development of HUS, compared with all other cytotoxic drugs. Gemcitabine is a novel cytotoxic drug with promising activity against pancreatic adenocarcinoma. We are reporting on one patient with metastatic duodenal papillary carcinoma developing HUS while on weekly gemcitabine therapy. The presenting features in this patient were non-cardiac pulmonary oedema, renal failure, thrombocytopenia and haemolytic anaemia. The diagnosis of HUS was made on the day of admission of the patient to this institution. Upon aggressive therapy, including one single haemodialysis and five plasmaphereses, the patient recovered uneventfully, with modestly elevated creatinine-values as a remnant of the acute illness. Re-exposure to gemcitabine 6 months after the episode of HUS instituted for progressive carcinoma, thus far has not caused another episode of HUS. 1999 Cancer Research Campaign PMID:10188900

Serke, S; Riess, H; Oettle, H; Huhn, D

1999-01-01

286

Multidisciplinary treatment of disability in ehlers-danlos syndrome hypermobility type/hypermobility syndrome: A pilot study using a combination of physical and cognitive-behavioral therapy on 12 women.  

PubMed

Ehlers-Danlos Syndrome hypermobility type (EDS-HT) and joint hypermobility syndrome (JHS) are two overlapping heritable connective tissue disorders. Patients with these conditions have many and various complaints; limitations in performing daily activities, reduced muscle strength and proprioception, kinesiophobia, and pain. There is a lack of evidence-based treatment approaches; a few studies have shown effect of physiotherapy. Many authors propose multidisciplinary treatment, but this has neither been described nor evaluated for this patient group. The aim of this pilot study was to investigate if a multidisciplinary rehabilitation program combining physical and cognitive-behavioral therapy was feasible, safe and effective for 12 women with EDS-HT/JHS. Intervention was offered as a group program and consisted of three parts: (1) Two and a half week in a rehabilitation unit with testing, physical training, group discussions and lectures. (2) Individual home exercises for three months with weekly guidance by local physiotherapist. (3) Readmission four days for retesting and further training advice. All participants completed the intervention. We found significant changes in perceived performance of daily activities, significant increase of muscle strength and endurance and a significant reduction of kinesiophobia. There were smaller changes in self-perceived pain. The participants also reported increased participation in daily life. PMID:23913726

Bathen, Trine; Hngmann, Anett Bjrndegrd; Hoff, Marie; Andersen, Liv inaes; Rand-Hendriksen, Svend

2013-12-01

287

Increased Endothelin1 Levels in Women with Polycystic Ovary Syndrome and the Beneficial Effect of Metformin Therapy  

Microsoft Academic Search

Women with polycystic ovary syndrome who present with hyperandrogenemia, hyperinsulinemia, and insulin resis- tance appear to be at high risk of cardiovascular disease. Elevated levels of endothelin-1, a marker of vasculopathy, have been reported in insulin-resistant subjects with endo- thelial dysfunction. Male gender also seems to be an aggra- vating factor for cardiovascular disease. In this study we investigated endothelin-1

EVANTHIA DIAMANTI-KANDARAKIS; GIOVANNA SPINA; CHRYSSA KOULI; ILIAS MIGDALIS

2010-01-01

288

Morbihan syndrome  

PubMed Central

We report a case of severe Morbihan syndrome (chronic erythematous edema of the upper portion of the face) in a 60-year-old man. The syndrome was characterized clinically by erythematous edema involving the forehead, glabella, and both eyelids, because of which the patient was not able to open completely his eyes. Furthermore, erythema and telangiectasiae were visible on the nose and cheeks. Laboratory and instrumental examinations were within normal ranges or negative. Histopathological examination showed dermal edema, perivascular and periadnexal lympho-histiocytic infiltrate, and sebaceous gland hyperplasia. Oral isotretinoin was ineffective despite the relatively long duration of the therapy (26 weeks). PMID:23741671

Veraldi, Stefano; Persico, Maria Chiara; Francia, Claudia

2013-01-01

289

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 areaadjusted 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

290

Imported Acquired Immunodeficiency Syndrome-Related Histoplasmosis in Metropolitan France: A Comparison of Pre-Highly Active Anti-Retroviral Therapy and Highly Active Anti-Retroviral Therapy Eras  

PubMed Central

Histoplasma capsulatum var. capsulatum infection is rare outside disease-endemic areas. Clinical presentation and outcome of acquired immunodeficiency syndromerelated histoplasmosis are unknown in non-endemic areas with wide access to highly active anti-retroviral therapy (HAART). Retrospective analysis of cases recorded at the French National Reference Center for Mycoses and Antifungals during two decades: pre-HAART (19851994) and HAART (19972006). Clinical features and outcome of all adults with proven acquired immunodeficiency syndromerelated histoplasmosis were compared between the two periods. One hundred four patients were included (40 during the pre-HAART era and 64 during the HAART era). Diagnosis was established a mean of 62 days after onset of symptoms. One-year overall mortality rates decreased from 53% (pre-HAART era) to 22% (HAART era). Diagnosis during the pre-HAART era and an older age were the only independent factors associated with death. Histoplasmosis is a rare invasive fungal infection outside disease-endemic areas. Its prognosis improved significantly during the HAART era. PMID:22049053

Peigne, Vincent; Dromer, Francoise; Elie, Caroline; Lidove, Olivier; Lortholary, Olivier

2011-01-01

291

Neuroacanthocytosis Syndromes  

PubMed Central

Neuroacanthocytosis (NA) syndromes are a group of genetically defined diseases characterized by the association of red blood cell acanthocytosis and progressive degeneration of the basal ganglia. NA syndromes are exceptionally rare with an estimated prevalence of less than 1 to 5 per 1'000'000 inhabitants for each disorder. The core NA syndromes include autosomal recessive chorea-acanthocytosis and X-linked McLeod syndrome which have a Huntingtons disease-like phenotype consisting of a choreatic movement disorder, psychiatric manifestations and cognitive decline, and additional multi-system features including myopathy and axonal neuropathy. In addition, cardiomyopathy may occur in McLeod syndrome. Acanthocytes are also found in a proportion of patients with autosomal dominant Huntington's disease-like 2, autosomal recessive pantothenate kinase-associated neurodegeneration and several inherited disorders of lipoprotein metabolism, namely abetalipoproteinemia (Bassen-Kornzweig syndrome) and hypobetalipoproteinemia leading to vitamin E malabsorption. The latter disorders are characterized by a peripheral neuropathy and sensory ataxia due to dorsal column degeneration, but movement disorders and cognitive impairment are not present. NA syndromes are caused by disease-specific genetic mutations. The mechanism by which these mutations cause neurodegeneration is not known. The association of the acanthocytic membrane abnormality with selective degeneration of the basal ganglia, however, suggests a common pathogenetic pathway. Laboratory tests include blood smears to detect acanthocytosis and determination of serum creatine kinase. Cerebral magnetic resonance imaging may demonstrate striatal atrophy. Kell and Kx blood group antigens are reduced or absent in McLeod syndrome. Western blot for chorein demonstrates absence of this protein in red blood cells of chorea-acanthocytosis patients. Specific genetic testing is possible in all NA syndromes. Differential diagnoses include Huntington disease and other causes of progressive hyperkinetic movement disorders. There are no curative therapies for NA syndromes. Regular cardiologic studies and avoidance of transfusion complications are mandatory in McLeod syndrome. The hyperkinetic movement disorder may be treated as in Huntington disease. Other symptoms including psychiatric manifestations should be managed in a symptom-oriented manner. NA syndromes have a relentlessly progressive course usually over two to three decades. PMID:22027213

2011-01-01

292

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 Frster; Eva-B. Brcker; Jean-Hilaire Saurat; Lars E. French

2003-01-01

293

Genistin-rich soy isoflavone extract in substrate reduction therapy for Sanfilippo syndrome: An open-label, pilot study in 10 pediatric patients  

PubMed Central

Background: Mucopolysaccharidoses (MPSs) are a group of severe metabolic disorders caused by deficiencies in enzymes involved in the degradation of glycosaminoglycans (GAGs)long chains of sugar carbohydrates in cells that help build bone, cartilage, tendons, corneas, skin, and connective tissue. Although enzyme replacement therapy has become available for the treatment of some types of MPS, effective treatment of neurodegenerative forms of MPS has yet to be determined. Recently, genistein (4',5,7-trihydroxyisoflavone), a specific inhibitor of protein tyrosine kinase, has been found to inhibit GAG synthesis and to reduce GAG concentrations in cultures of fibroblasts of MPS patients. Therefore, a potential substrate reduction therapy has been proposed. Objective: The aim of this study was to examine urinary GAG concentration, hair morphology, and cognitive function in patients receiving genistin treatment for Sanfilippo syndrome (MPS type III). Methods:Patients aged 3 to 14 years with a biochemically confirmed diagnosis of MPS IIIA or MPS IIIB were eligible to enroll in this open-label, pilot study. Genistin-rich soy isoflavone extract 5 mg/kg/d was administered PO for 12 months. Urinary GAG concentration, hair morphology,and cognitive function (measured using a modified version of the Brief Assessment Examination [BAE] and parent observations)were measured at baseline and after 12 months of treatment. Results: Ten patients (6 girls, 4 boys; mean age, 8 years [range,3\\2-14 years];mean weight, 28 kg [range, 17\\2-43 kg]) were included in the study. All patients had Sanfilippo syndrome; 5 patients had MPS IIIA and 5 had MPS IIIB. After 1 year, statistically significant improvement was found in urinary GAG concentration, hair morphology, and cognitive function. Urinary GAG concentration decreased significantly in all 5 patients with MPS IIIA and in 2 patients with MPS IIIB (P = 0.028). Hair morphology improved significantly in all 5 MPS IIIA patients and in 3 MPS IIIB patients (P = 0.012). A significant increase in the BAE score (by 2-6 points) was noted in 8 patients, while the scores of 2 patients did not change after 12 months of treatment (P = 0.012). No adverse events (AEs) considered related to treatment were reported. Moreover, no AEs not related to the treatment (apart from classical symptoms of MPS III) were noted. Conclusions: This pilot study found some improvements in GAG concentration, hair morphology, and cognitive function in these pediatric patients with Sanfilippo syndrome treated with genistin-rich soy isoflavone extract for 1 year. Clinical trials are needed to evaluate the efficacy and safety of this potential treatment. PMID:24692796

Piotrowska, Ewa; Jakbkiewicz-Banecka, Joanna; Tylki-Szymanska, Anna; Liberek, Anna; Maryniak, Agnieszka; Malinowska, Marcelina; Czartoryska, Barbara; Puk, Ewa; Kloska, Anna; Liberek, Tomasz; Baranska, Sylwia; Wegrzyn, Alicja; Wegrzyn, Grzegorz

2008-01-01

294

[Use of local injections of tolperisone (midocalm) in combination with tractional therapy in the treatment of vertebral static syndrome].  

PubMed

A randomized double-blind study of the efficacy of midocalm combined with tractional therapy has been carried out in 24 patients (indexed group) comparing to the tractional therapy without midocalm (the drug has been substituted with placebo) in 25 patients (comparison group). The effective number of injections was 3-6 in the indexed group and 7 in the comparison group. A quantitative assessment revealed that in the indexed group treatment efficacy was 1,56 times higher and the effect achieved sooner than in the comparison group. PMID:18379519

Zakharov, Ia Iu; Kutarev, R V; Shelkov, S N; Suslov, S A

2007-01-01

295

Hepatorenal Syndrome  

PubMed Central

Hepatorenal syndrome (HRS) is a serious complication of liver cirrhosis with critically poor prognosis. The pathophysiological hallmark is severe renal vasoconstriction, resulting from complex changes in splanchnic and general circulations as well as systemic and renal vasoconstrictors and vasodilators. Rapid diagnosis and management are important, since recent treatment modalities including vasoconstrictor therapy can improve short-term outcome and buy time for liver transplantation, which can result in complete recovery. PMID:17603637

Ng, Charles KF; Chan, Michael HM; Tai, Morris HL; Lam, Christopher WK

2007-01-01

296

Low-level laser therapy with a wrist splint to treat carpal tunnel syndrome: a double-blinded randomized controlled trial.  

PubMed

The efficacy of low-level laser therapy (LLLT) was evaluated in a total of 66 patients with mild to moderate carpal tunnel syndrome (CTS) with a double-blinded randomized controlled study. The patients were randomly assigned into two groups. Group I received 15 sessions of a gallium-aluminum-arsenide laser treatment at a dosage of 18 J per session over the carpal tunnel area with neutral wrist splint. Group II received placebo laser therapy with neutral wrist splint. The patients were evaluated with the following parameters: (1) clinical parameters which consisted of visual analog scale, symptom severity scale, functional status scale, and pinch strength and grip strength before the treatment and at 5- and 12-week follow-ups and (2) electroneurophysiological parameters from nerve conduction study which were evaluated before the treatment and at 12-week follow-up. Fifty nine patients (112 hands: unilateral CTS = 6 hands and bilateral CTS = 106 hands) completed the study. Both groups I and II had n = 56 hands. Improvements were significantly more pronounced in the LLLT-treated group than the placebo group especially for grip strength at 5- and 12-week follow-ups. At 12-week follow-up, distal motor latency of the median nerve was significantly improved in the LLLT group than the placebo group (p < 0.05). LLLT therapy, as an alternative for a conservative treatment, is effective for treating mild to moderate CTS patients. It can improve hand grip strength and electroneurophysiological parameter with a carry-over effect up to 3 months after treatment for grip strength of the affected hands. PMID:24477392

Fusakul, Yupadee; Aranyavalai, Thanyaporn; Saensri, Phongphitch; Thiengwittayaporn, Satit

2014-05-01

297

The Othello Syndrome  

PubMed Central

A case of the Othello syndrome is presented. In its classical form the syndrome is rare, but as with other allied paranoid states, its medicosocial implications are great. Rational management should include pharmacotherapy, conjoint family therapy after symptom remission, and long-term individual psychotherapy. PMID:6827614

Famuyiwa, Oluwole O.; Ekpo, Micheal

1983-01-01

298

The Othello syndrome.  

PubMed

A case of the Othello syndrome is presented. In its classical form the syndrome is rare, but as with other allied paranoid states, its medicosocial implications are great. Rational management should include pharmacotherapy, conjoint family therapy after symptom remission, and long-term individual psychotherapy. PMID:6827614

Famuyiwa, O O; Ekpo, M

1983-02-01

299

Trousseau's syndrome.  

PubMed Central

We report 4 cases of Trousseau's syndrome, in which spontaneous recurrent or migratory venous thromboses, arterial emboli caused by nonbacterial thrombotic endocarditis, or both, develop in a patient with a recognized or occult malignant tumor. The clinical course of 3 of the patients emphasizes a key point: The occurrence for no known reason of thromboses preventable by anticoagulation therapy with heparin but not with warfarin sodium should alert a physician to focus diagnostic efforts on uncovering an underlying malignant lesion. Thromboses may occur months to years before the tumor is discovered, and a thorough negative initial examination does not obviate the need for a continuing search. Patients with Trousseau's syndrome have persistent low-grade intravascular coagulation, and therapy with heparin should be continued indefinitely. Stopping heparin therapy for even a day may permit a new thrombosis to develop. Immunostaining a biopsy specimen from 1 patient provided evidence that 2 properties of a neoplastic lesion are required for the syndrome to develop: The malignant cells express surface membrane tissue factor, and structural features of the tumor permit the malignant cells or vesicles it sheds to be exposed to circulating blood. Images PMID:8317122

Callander, N; Rapaport, S I

1993-01-01

300

Stevens-Johnson syndrome developing in a girl with systemic lupus erythematosus on high-dose corticosteroid therapy.  

PubMed

Stevens-Johnson syndrome (SJS) is a vesiculobullous disease of the skin and mucosa. This generalized hypersensitivity reaction is well known to occur in association with certain drugs, particularly sulfonamides, nonsteroidal anti-inflammatory agents (NSAIDs), and anticonvulsants. The disease is usually seen in children and young adults and is often treated with corticosteroids. We present a 9-year-old girl with lupus nephritis who developed cutaneous bullae and mucositis while being treated with intravenous methylprednisolone. The initial differential diagnosis included bullous lupus, but skin biopsy specimen findings supported a diagnosis of SJS. She was treated with intravenous immunoglobulin (IVIg). PMID:11860572

Samimi, Sarah S; Siegfried, Elaine

2002-01-01

301

Lifestyle intervention and anti-obesity therapies in the polycystic ovary syndrome: impact on metabolism and fertility.  

PubMed

Obesity is frequently present in patients with polycystic ovary syndrome (PCOS) and plays an important role in the pathogenesis of the metabolic, endocrine, and reproductive abnormalities associated with this syndrome. We aimed to summarize the effects of lifestyle changes and anti-obesity pharmacotherapy in patients with PCOS. We reviewed the literature regarding the effects of lifestyle changes and anti-obesity agents on the metabolic and endocrine abnormalities of PCOS. Lifestyle changes, including diet, exercise, and behavioral modification, appear to improve the metabolic and reproductive abnormalities of overweight and obese patients with PCOS. Therefore, lifestyle changes appear to represent the first-line management for all overweight and obese patients with PCOS. However, the optimal composition of diet and the optimal type of exercise in these patients are unknown. Anti-obesity agents that have been studied in PCOS include orlistat, sibutramine, and rimonabant. However, the latter two agents have been withdrawn from the market because of side effects. Long-term studies with orlistat in overweight and obese diabetic patients showed greater weight loss and metabolic and cardiovascular benefits than those achieved with lifestyle changes alone. However, there are limited data on the efficacy of orlistat in women with PCOS. In conclusion, lifestyle changes (diet, exercise and behavioral modification), particularly when combined with anti-obesity agents, exert beneficial effects on the endocrine abnormalities of obese patients with PCOS and improve metabolic parameters. PMID:23625194

Panidis, Dimitrios; Tziomalos, Konstantinos; Papadakis, Efstathios; Vosnakis, Christos; Chatzis, Panagiotis; Katsikis, Ilias

2013-12-01

302

Analysis of Copper and Zinc Plasma Concentration and the Efficacy of Zinc Therapy in Individuals with Asperger's Syndrome, Pervasive Developmental Disorder Not Otherwise Specified (PDD-NOS) and Autism  

PubMed Central

Aim To assess plasma zinc and copper concentration in individuals with Aspergers Syndrome, Pervasive Developmental Disorder-Not Otherwise Specified (PDD-NOS) and autistic disorder, and to analyze the efficacy of zinc therapy on the normalization of zinc and copper levels and symptom severity in these disorders. Subjects and methods Plasma from 79 autistic individuals, 52 individuals with PDD-NOS, 21 individuals with Aspergers Syndrome (all meeting DSM-IV diagnostic criteria), and 18 age and gender similar neurotypical controls, were tested for plasma zinc and copper using inductively-coupled plasma-mass spectrometry. Results Autistic and PDD-NOS individuals had significantly elevated plasma levels of copper. None of the groups (autism, Aspergers or PDD-NOS) had significantly lower plasma zinc concentrations. Post zinc and B-6 therapy, individuals with autism and PDD-NOS had significantly lower levels of copper, but individuals with Aspergers did not have significantly lower copper. Individuals with autism, PDD-NOS and Aspergers all had significantly higher zinc levels. Severity of symptoms decreased in autistic individuals following zinc and B-6 therapy with respect to awareness, receptive language, focus and attention, hyperactivity, tip toeing, eye contact, sound sensitivity, tactile sensitivity and seizures. None of the measured symptoms worsened after therapy. None of the symptoms in the Aspergers patients improved after therapy. Discussion These results suggest an association between copper and zinc plasma levels and individuals with autism, PDD-NOS and Aspergers Syndrome. The data also indicates that copper levels normalize (decrease to levels of controls) in individuals with autism and PDD-NOS, but not in individuals with Aspergers. These same Aspergers patients do not improve with respect to symptoms after therapy, whereas many symptoms improved in the autism group. This may indicate an association between copper levels and symptom severity. PMID:22174567

Russo, A.J.; deVito, Robert

2011-01-01

303

Persistence and selection of an expanded B-cell clone in the setting of rituximab therapy for Sjgrens syndrome  

PubMed Central

Introduction Subjects with primary Sjgrens syndrome (SjS) have an increased risk of developing B-cell lymphoma and may harbor monoclonal B-cell expansions in the peripheral blood. Expanded B-cell clones could be pathogenic, and their persistence could exacerbate disease or predispose toward the development of lymphoma. Therapy with anti-CD20 (rituximab) has the potential to eliminate expanded B-cell clones and thereby potentially ameliorate disease. This study was undertaken to identify and track expanded B-cell clones in the blood of subjects with primary SjS who were treated with rituximab. Methods To determine whether circulating B-cell clones in subjects with primary SjS emerge or remain after B cell-depleting therapy with rituximab, we studied the antibody heavy-chain repertoire. We performed single-memory B-cell and plasmablast sorting and antibody heavy-chain sequencing in six rituximab-treated SjS subjects over the course of a 1-year follow-up period. Results Expanded B-cell clones were identified in four out of the six rituximab-treated SjS subjects, based upon the independent amplification of sequences with identical or highly similar VH, DH, and JH gene segments. We identified one SjS subject with a large expanded B-cell clone that was present prior to therapy and persisted after therapy. Somatic mutations in the clone were numerous but did not increase in frequency over the course of the 1-year follow-up, suggesting that the clone had been present for a long period of time. Intriguingly, a majority of the somatic mutations in the clone were silent, suggesting that the clone was under chronic negative selection. Conclusions For some subjects with primary SjS, these data show that (a) expanded B-cell clones are readily identified in the peripheral blood, (b) some clones are not eliminated by rituximab, and (c) persistent clones may be under chronic negative selection or may not be antigen-driven. The analysis of sequence variation among members of an expanded clone may provide a novel means of measuring the chronicity and selection of expanded B-cell populations in humans. PMID:24517398

2014-01-01

304

Increased leptin supply to hypothalamus by gene therapy confers life-long benefits on energy homeostasis, disease cluster of metabolic syndrome- diabetes type 1 and 2, dyslipidemia and cardiovascular ailments- and bone remodeling  

Microsoft Academic Search

Leptin insufficiency in the hypothalamus is causally linked to increased fat accrual, diseases of the metabolic syndrome,\\u000a skeletal abnormalities and shortened life-span. We show that leptin sufficiency attained with hypothalamic leptin gene therapy\\u000a (i) suppressed food intake, the agerelated and energy enriched diet-induced fat accrual, and dyslipidemia, (ii) attenuated\\u000a episodic insulin secretion and enhanced insulin sensitivity, (iii) enhanced glucose tolerance

Satya P. Kalra

305

Fatigue In Teenagers on the interNET - The FITNET Trial. A randomized clinical trial of web-based cognitive behavioural therapy for adolescents with chronic fatigue syndrome: study protocol. [ISRCTN59878666  

Microsoft Academic Search

BackgroundChronic Fatigue Syndrome (CFS) is increasingly recognized as a cause of disability and inactivity in adolescents in the Netherlands.\\u000a CFS is characterized by unexplained fatigue lasting more than 6 months. Cognitive Behavioural Therapy (CBT) has proven to\\u000a be effective. However, CBT availability for adolescents with CFS is limited and requires special therapeutic skills not always\\u000a readily available. An alternative to

Sanne L Nijhof; Gijs Bleijenberg; Cuno SPM Uiterwaal; Jan LL Kimpen; Elise M van de Putte

2011-01-01

306

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; Huser, Winfried

2013-01-01

307

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 participants 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

308

Early Surfactant Therapy With Nasal Continuous Positive Airway Pressure or Continued Mechanical Ventilation in Very Low Birth Weight Neonates With Respiratory Distress Syndrome  

PubMed Central

Background: Various strategies have been suggested for the treatment of respiratory distress syndrome (RDS). Objectives: The aim of this study was to compare the efficacies of two common methods of RDS management among neonates with low birth weight. Patients and Methods: A cohort study was conducted on 98 neonates with definite diagnosis of RDS during 2008-2009. The neonates were divided into two groups by a blinded supervisor using simple randomization (odd and even numbers). Forty-five cases in the first group were treated with intubation, surfactant therapy, extubation (INSURE method) followed by nasal continuous positive airway pressure (N.CPAP) and 53 cases in the second group underwent intubation, surfactant therapy followed by mechanical ventilation (MV). Results: Five (11.1%) cases in the first group and 23 (43%) cases in the second group expired during the study. The rates of MV dependency among cases with INSURE failure and cases in the MV group were 37% and 83%, respectively (P < 0.001). Birth weight (BW) (P = 0.017), presence of retinopathy of prematurity (P = 0.022), C/S delivery (P = 0.029) and presence of lung bleeding (P = 0.010) could significantly predict mortality in the second group, although only BW (P = 0.029) had a significant impact on the mortality rate in the first group. Moreover, BW was significantly related to the success rate in the first group (P = 0.001). Conclusions: Our findings demonstrated that INSURE plus NCPAP was more effective than the routine method (permanent intubation after surfactant prescription). In addition, the lower rates of mortality, MV dependency, duration of hospitalization, and complications were observed in cases treated with the INSURE method compared to the routine one. PMID:24910785

Najafian, Bita; Fakhraie, Seyed Hasan; Afjeh, Seyed Abulfazl; Kazemian, Mohammad; Shohrati, Majid; Saburi, Amin

2014-01-01

309

A preliminary prospective study of nutritional, psychological and combined therapies for myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) in a private care setting  

PubMed Central

Background Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a condition characterised by severe and persistent fatigue, neurological disturbances, autonomic and endocrine dysfunctions and sleep difficulties that have a pronounced and significant impact on individuals lives. Current National Institute for Health and Clinical Excellence guidelines within the UK suggest that this condition should be treated with cognitive behavioural therapy and/or graded exercise therapy, where appropriate. There is currently a lack of an evidence base concerning alternative techniques that may be beneficial to those with ME/CFS. Objectives This study aimed to investigate whether three modalities of psychology, nutrition and combined treatment influenced symptom report measures in those with ME/CFS over a 3-month time period and whether there were significant differences in these changes between groups. Design and setting This is a preliminary prospective study with one follow-up point conducted at a private secondary healthcare facility in London, UK. Participants 138 individuals (110 females, 79.7%; 42 participants in psychology, 44 in nutrition and 52 in combined) participated at baseline and 72 participants completed the battery of measures at follow-up (52.17% response rate; 14, 27 and 31 participants in each group, respectively). Outcome measures Self-reported measures of ME/CFS symptoms, functional ability, multidimensional fatigue and perceived control. Results Baseline comparisons showed those in the combined group had higher levels of fatigue. At follow-up, all groups saw improvements in fatigue, functional ability and symptomatology; those within the psychology group also experienced a shift in perceived control over time. Conclusions This study provides early evidence that psychological, nutritional and combined techniques for the treatment of ME/CFS may influence symptomatology, fatigue, function and perceived control. However, these results must be viewed with caution as the allocation to groups was not randomised, there was no control group and the study suffered from high drop-out rates. PMID:23166120

Arroll, Megan Anne; Howard, Alex

2012-01-01

310

Phase I-II Clinical Trial of Oxaliplatin, Fludarabine, Cytarabine, and Rituximab Therapy in Aggressive, Relapsed/Refractory Chronic Lymphocytic Leukemia or Richter's Syndrome  

PubMed Central

Introduction To improve outcomes of patients with Richter's syndrome (RS) and relapsed/refractory chronic lymphocytic leukemia (CLL), we modified the oxaliplatin and cytarabine doses of the oxaliplatin, fludarabine, cytarabine, and rituximab (OFAR1) regimen for this phase I-II study (OFAR2). Patients and Methods OFAR2 consisted of oxaliplatin at 30 mg/m2 on days (D) 14, fludarabine at 30 mg/m2, cytarabine at 0.5 g/m2, rituximab at 375 mg/m2 (D3), and pelfigrastim at 6 mg (D6). Fludarabine and cytarabine were given on D23 (cohort 1), D24 (cohort 2), or D25 (cohort 3) every 4 weeks. Phase II followed the "3+3" design of phase I. Results The 102 patients (CLL, 67; RS, 35) treated had heavily pretreated, high-risk disease. Twelve patients were treated in phase I; cohort 2 was the phase II recommended dose. The most common toxicities were hematologic. Response rates (phase II) were 38.7% for RS (CR, 6.5%) and 50.8% for relapsed/refractory CLL (CR, 4.6%). The median survival durations were 6.6 (RS) and 20.6 (CLL) months. Among 9 patients who underwent allogeneic stem cell transplantation (SCT) as post-remission therapy, none has died (median follow-up, 15.9 months). Conclusion OFAR2 had significant antileukemic activity in RS and relapsed/refractory CLL. Patients undergoing SCT as post-remission therapy had favorable outcomes PMID:23810245

Tsimberidou, Apostolia M.; Wierda, William G.; Wen, Sijin; Plunkett, William; O'Brien, Susan; Kipps, Thomas J.; Jones, Jeffrey A.; Badoux, Xavier; Kantarjian, Hagop; Keating, Michael J.

2014-01-01

311

Lumbar disc herniation and cauda equina syndrome following spinal manipulative therapy: a review of six court decisions in Canada.  

PubMed

The purpose of this review is to expand practitioners' knowledge on areas of liability when treating low back pain patients. Six cases where chiropractors in Canada were sued for allegedly causing or aggravating lumbar disc herniation after spinal manipulative therapy were retrieved using the CANLII search database. The case series involves 4 men and 2 women with an average age of 37.3 years (range, 31-48 years). Trial courts' decisions were rendered between 2000 and 2011. This study highlights the following conclusions from Canadian courts: 1) informed consent is an ongoing process that cannot be entirely delegated to office personnel; 2) when the patient's history reveals risk factors for lumbar disc herniation the chiropractor has the duty to rule out disc pathology as an etiology for the symptoms presented by the patients before beginning anything but conservative palliative treatment; 3) lumbar disc herniation may be triggered by spinal manipulative therapy on vertebral segments distant from the involved herniated disc such as the thoracic spine. PMID:24485443

Boucher, Pierre; Robidoux, Sbastien

2014-02-01

312

Fluticasone, azithromycin and montelukast therapy in reducing corticosteroid exposure in bronchiolitis obliterans syndrome after allogeneic hematopoietic SCT: a case series of eight patients.  

PubMed

Bronchiolitis obliterans syndrome (BOS) is a devastating pulmonary complication affecting long-term survivors of allogeneic hematopoietic cell transplantation. Treatment of BOS with prolonged courses of high dose corticosteroids is often associated with significant morbidity. Reducing the exposure to corticosteroids may reduce treatment-related morbidity. Our institution has recently begun to treat patients with emerging therapies in an effort to diminish corticosteroid exposure. We retrospectively reviewed the 6-month corticosteroid exposure, lung function and failure rates in eight patients with newly diagnosed BOS who were treated with a combination of fluticasone, azithromycin and montelukast (FAM) and a rapid corticosteroid taper. These patients were compared with 14 matched historical patients who received high-dose corticosteroids, followed by a standard taper. The median 6-month prednisone exposure in FAM-treated patients was 1819?mg (0-4036?mg) compared with 7163?mg (6551-7829?mg) in the control group (P=0.002). The median forced expiratory volume in 1?s (FEV(1)) change in FAM-treated patients was 2% (-3 to 4%] compared with 1% (-4 to 5%) in the control group (P=1.0). Prednisone exposure in FAM patients was one quarter that of a retrospective-matched group of patients, with minimal change in median FEV(1), suggesting that BOS may be spared of the morbidities associated with long-term corticosteroid use by using alternative agents with less side effects. PMID:21132024

Norman, B C; Jacobsohn, D A; Williams, K M; Au, B K C; Au, M A; Lee, S J; Moravec, C K; Chien, J W

2011-10-01

313

Fluticasone, Azithromycin, And Montelukast (FAM) Therapy In Reducing Corticosteroid Exposure In Bronchiolitis Obliterans Syndrome After Allogeneic Hematopoietic Stem Cell Transplant - A Case Series Of Eight Patients  

PubMed Central

Backround Bronchiolitis obliterans syndrome (BOS) is a devastating pulmonary complication affecting long term survivors of allogeneic hematopoietic cell transplantation. Treatment of BOS with prolonged courses of high dose corticosteroids is often associated with significant morbidity. Reducing the exposure to corticosteroids may reduce treatment related morbidity. Our institution has recently begun to treat patients with emerging therapies in an effort to diminish steroid exposure. Methods We retrospectively reviewed the 6-month corticosteroid exposure, lung function, and failure rates in 8 patients with newly diagnosed BOS who were treated with a combination of fluticasone, azithromycin and montelukast (FAM) and a rapid corticosteroid taper. These patients were compared to 14 matched historical patients who received high dose corticosteroids followed by a standard taper. Results The median 6-month prednisone exposure in FAM-treated patients was 1819 mg [0 mg to 4036 mg] compared to 7163 mg [6551 mg to 7829 mg] in the control group (p = 0.002). The median FEV1 change in FAM-treated patients was 2% [?3% to 4%] compared to 1% [?4 to 5%] in the control group (p = 1.0). Discussion Prednisone exposure in FAM patients was one quarter that of a retrospective matched group of patients, with minimal change in median FEV1, suggesting that BOS may be spared of the morbidities associated with long-term corticosteroid use by using alternative agents with less side effects. PMID:21132024

Norman, Brett C.; Jacobsohn, David A.; Williams, Kirsten M.; Au, Brandon; Au, Margaret A.; Lee, Stephanie J.; Moravec, Carina K.; Chien, Jason W.

2014-01-01

314

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; Bll, Boris; von Tresckow, Bastian; Borchmann, Peter; Engert, Andreas

2014-03-13

315

Proinflammatory cytokine levels in saliva in patients with burning mouth syndrome before and after treatment with low-level laser therapy.  

PubMed

The aim of this study was to determine the levels of proinflammatory tumor necrosis factor-alpha (TNF-?) and interleukin-6 (IL-6) cytokines in whole unstimulated saliva in subjects with burning mouth syndrome (BMS) before and after treatment with low-level laser therapy (LLLT). BMS is characterized by a continuous, painful burning sensation in a clinically normal-appearing oral mucosa. A sample consisting of 40 consecutive subjects was selected on a voluntary basis from the pool of patients who presented for diagnosis and treatment of BMS at the Oral Medicine Unit of the Faculty of Medicine of the University of Rijeka. For determination of salivary levels of TNF-? and IL-6, ELISA (Sigma Immunochemicals, St. Louis, MO, USA) was performed to determine the salivary levels of TNF-? and IL-6. After 4 weeks of LLLT, the salivary levels of TNF-? and IL-6 in the experimental group decreased significantly (p < 0.001). There was no significant difference in the experimental group regarding visual analogue scale. PMID:22773117

Pezelj-Ribari?, Sonja; Kqiku, Lumnije; Brumini, Gordana; Urek, Miranda Muhvi?; Antoni?, Robert; Kui, Davor; Glaar, Irena; Stdtler, Peter

2013-01-01

316

In-depth analysis of hyaline fibromatosis syndrome frameshift mutations at the same site reveal the necessity of personalized therapy.  

PubMed

Hyaline fibromatosis syndrome is an autosomal recessive disease caused by mutations in ANTXR2, a gene involved in extracellular matrix homeostasis. Sixty percent of patients carry frameshift mutations at a mutational hotspot in exon 13. We show in patient cells that these mutations lead to low ANTXR2 mRNA and undetectable protein levels. Ectopic expression of the proteins encoded by the mutated genes reveals that a two base insertion leads to the synthesis of a protein that is rapidly targeted to the ER-associated degradation pathway due to the modified structure of the cytosolic tail, which instead of being hydrophilic and highly disordered as in wild type ANTXR2, is folded and exposes hydrophobic patches. In contrast, one base insertion leads to a truncated protein that properly localizes to the plasma membrane and retains partial function. We next show that targeting the nonsense mediated mRNA decay pathway in patient cells leads to a rescue of ANTXR2 protein in patients carrying one base insertion but not in those carrying two base insertions. This study highlights the importance of in-depth analysis of the molecular consequences of specific patient mutations, which even when they occur at the same site can have drastically different consequences. PMID:23554269

Yan, Shixu E; Lemmin, Thomas; Salvi, Suzanne; Lausch, Ekkehart; Superti-Furga, Andrea; Rokicki, Dariusz; Dal Peraro, Matteo; van der Goot, F Gisou

2013-07-01

317

Novel Anti-idiotype Antibody Therapy for Lipooligosaccharide-Induced Experimental Autoimmune Neuritis: Use Relevant to Guillain-Barr? Syndrome  

PubMed Central

Campylobacteriosis is a frequent antecedent event in Guillain-Barr syndrome (GBS), inducing high-titer serum antibodies for ganglioside antigens in the peripheral nervous system (PNS). Molecular mimicry between the lipooligosaccharide (LOS) component of Campylobacter jejuni and human peripheral nerve gangliosides is believed to play an important role in the pathogenesis of GBS. Conventional treatment strategies for patients with GBS include plasmapheresis, intravenous immunoglobulin (IVIG), and immunosuppression, which are invasive or relatively ineffective. In this study, we used our animal model of GBS, in which Lewis rats were immunized with GD3-like LOS isolated from C. jejuni. The animals developed anti-GD3 ganglioside antibodies and manifested neuromuscular dysfunction. To develop novel therapeutic strategies, we treated the animals by intraperitoneal administration of an anti-GD3 antiidiotype monoclonal antibody (BEC2) that specifically interacts with the pathogenic antibody. The treated animals had a remarkable reduction of anti-GD3 antibody titers and improvement of motor nerve functions. The results suggest that ganglioside mimics, such as antiidiotype antibodies, may be powerful reagents for therapeutic intervention in GBS by neutralizing specific pathogenic antiganglioside antibodies. PMID:20077429

Usuki, S.; Taguchi, K.; Thompson, S.A.; Chapman, P.B.; Yu, R.K.

2010-01-01

318

Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome  

PubMed Central

Reduced bone mineral density (osteopenia) is a poorly characterized manifestation of pediatric and adult patients afflicted with Marfan syndrome (MFS), a multisystem disorder caused by structural or quantitative defects in fibrillin-1 that perturb tissue integrity and TGF? bioavailability. Here we report that mice with progressively severe MFS (Fbn1mgR/mgR mice) develop osteopenia associated with normal osteoblast differentiation and bone formation. In vivo and ex vivo experiments, respectively, revealed that adult Fbn1mgR/mgR mice respond more strongly to locally induced osteolysis and that Fbn1mgR/mgR osteoblasts stimulate pre-osteoclast differentiation more than wild-type cells. Greater osteoclastogenic potential of mutant osteoblasts was largely attributed to Rankl up-regulation secondary to improper TGF? activation and signaling. Losartan treatment, which lowers TGF? signaling and restores aortic wall integrity in mice with mild MFS, did not mitigate bone loss in Fbn1mgR/mgR mice even though it ameliorated vascular disease. Conversely, alendronate treatment, which restricts osteoclast activity, improved bone quality but not aneurysm progression in Fbn1mgR/mgR mice. Taken together, our findings shed new light on the pathogenesis of osteopenia in MFS, in addition to arguing for a multifaceted treatment strategy in this congenital disorder of the connective tissue. PMID:20871099

Nistala, Harikiran; Lee-Arteaga, Sui; Carta, Luca; Cook, Jason R.; Smaldone, Silvia; Siciliano, Gabriella; Rifkin, Aaron N.; Dietz, Harry C.; Rifkin, Daniel B.; Ramirez, Francesco

2010-01-01

319

Epidemiology and Clinical Parameters of Adult Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome at the Initiation of Antiretroviral Therapy in South Eastern Nigeria  

PubMed Central

Background: Human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) has continued to ravage the teeming populations in Nigeria, with disastrous consequences. Despite many studies and progress on HIV/AIDS in Africa, the data on the status of the patients at the commencement of therapy is lacking. Aim: The aim of this study is to determine the demographic, clinical and some laboratory features of adult HIV/AIDS patients, seen at the commencement of antiretroviral therapy (ART) in Nnamdi Azikiwe University Teaching Hospital, Nnewi, south-east Nigeria between July 2002 and October 2004. Subjects and Methods: The study was a cross-sectional, descriptive study. Adult patients living with HIV/AIDS were studied using an interview administered questionnaire. Data was analyzed using Epi Info 2008 version 3.5.1. Results: A total of 400 respondents participated in this study. The mean age was 36.8 (8.8) years. Almost 60% patients were married and the HIV concordance rate was 53.3% (136/255). Nearly 30% of the families had at least one child positive for HIV. The most common associated risky behavior was injection administered in patent medicine stores 74.5%(302/400) and the most common clinical symptom was respiratory. Of the 400 patients recruited in this study, 19 (4.8%) were lost to follow-up on the 6 months visit, giving a follow-up rate of 95.2% (381/400). There was statistically significant difference in the mean body weight (P = 0.02), mean total white blood cell count (P < 0.001) and mean CD4+ count (P < 0.001) at presentation and after 6 months of ART therapy. Conclusion: HIV/AIDS patients present late and body weight, CD4+ count and total white blood cell count seemed to recover quickly on commencement of ART. The prevalence of concordance among couples and mother to child transmission rates tended to be high. Administration of injectable at patent medicine stores and multiple sexual partners are the most significant risk factors. PMID:24761241

Eleje, GU; Ele, PU; Okocha, EC; Iloduba, UC

2014-01-01

320

Sensory syndromes.  

PubMed

Somatosensory deficit syndromes represent a common impairment following stroke and have a prevalence rate of around 80% in stroke survivors. These deficits restrict the ability of survivors to explore and manipulate their environment and are generally associated with a negative impact on quality of life and personal safety. Sensory impairments affect different sensory modalities in diverse locations at varying degrees, ranging from complete hemianesthesia of multiple modalities to dissociated impairment of somatosensory submodalities within a particular region of the body. Sensory impairments induce typical syndromal patterns which can be differentiated by means of a careful neurological examination, allowing the investigator to deduce location and size of the underlying stroke. In particular, a stroke located in the brainstem, thalamus, and the corticoparietal cortex result in well-differentiable sensory syndromes. Sensory function following stroke can be regained during rehabilitation even without specific sensory training. However, there is emerging evidence that specialized sensory interventions can result in improvement of somatosensory and motor function. Herein, we summarize the clinical presentations, examination, differential diagnoses, and therapy of sensory syndromes in stroke. PMID:22377851

Klingner, Carsten M; Witte, Otto W; Gnther, Albrecht

2012-01-01

321

Fibromyalgia syndrome.  

PubMed

Fibromyalgia syndrome (FMS) is a more common a condition than previously estimated. The most recent estimates are that 3 to 6 million patients have been diagnosed with FMS. The ACR criteria, established in 1990, provide the primary care provider with definitive subjective and objective findings that have shown to be 88% accurate in their ability to diagnose patients with the syndrome. There is no cure for FMS. It is a chronic condition, but patients quality of life can be improved when fatigue and pain are reduced. The institution of a plan that is developed collaboratively by the patient and the provider is the essence of successful symptom management. The hallmarks of the management plan include: improving the quality of sleep through the judicious use of medications that boost the body's level of serotonin (therefore reducing fatigue), and reducing pain through complimentary modalities such as exercise, physical therapy, relaxation techniques, massage, and biofeedback. PMID:9769357

Smith, W A

1998-12-01

322

Therapy-related Acute Myelogenous Leukemia and Myelodysplastic Syndrome in Patients With Acute Lymphoblastic Leukemia Treated With the Hyperfractionated Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone Regimens  

PubMed Central

Background Secondary malignancies including myeloid neoplasms occur infrequently in acute lymphoblastic leukemia (ALL) and to the authors' knowledge have not been as well documented in adults as in children. Methods A total of 641 patients with de novo ALL who were treated with the hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) regimen or its variants were analyzed. Results Sixteen patients (2.49%) developed secondary acute myelogenous leukemia (AML) (6 patients) or myelodysplastic syndrome (MDS) (10 patients). At the time of ALL diagnosis, the median age was 53 years; cytogenetics were normal in 11 patients, pseudo-diploidy with del(2) in 1 patient, t(9;22) in 1 patient, and unavailable in 3 patients. Frontline therapy included hyper-CVAD in 7 patients, hyper-CVAD with rituximab in 8 patients, and hyper-CVAD with imatinib in 1 patient. Karyotype at time of AML/MDS diagnosis was ?5, ?7 in 9 patients, normal in 1 patient, complex in 1 patient, inv(11) in 1 patient, t(4;11) in 1 patient, del(20) in 1 patient, and unavailable in 2 patients. Secondary AML/MDS developed at a median of 32 months after ALL diagnosis. Cytarabine plus anthracycline-based treatment was given to 12 patients with AML and high-risk MDS. One patient with MDS received arsenic trioxide, 1 received clofarabine, and 2 received decitabine. Response to treatment was complete remission in 3 patients, partial remission in 6 patients, and no response in 6 patients; 1 patient was untreated. Eight patients (1 with AML and 7 with MDS) underwent allogeneic stem cell transplantation, and all but 2 died at a median of 3 months (range, 0.5-11 months) after transplantation. The median overall survival after a diagnosis of secondary AML and MDS was 9.25 months (range, 1+ to 26+ months). Conclusions Secondary AML and MDS occur infrequently in adult patients with de novo ALL treated with the hyper-CVAD regimens, and response to therapy is poor. PMID:19090005

Verma, Dushyant; O'Brien, Susan; Thomas, Deborah; Faderl, Stefan; Koller, Charles; Pierce, Sherry; Kebriaei, Partow; Garcia-Manero, Guillermo; Cortes, Jorge; Kantarjian, Hagop; Ravandi, Farhad

2014-01-01

323

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).

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

324

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

325

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

326

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

327

[Paraneoplastic neurological syndromes].  

PubMed

Paraneoplastic neurological syndromes (PNS) are uncommon and defined by an acute or subacute neurological syndrome associated with a cancer. These syndromes often antedate the diagnostis of the underlying neoplasia that is usually not clinically evident. An early diagnosis is the main condition for a better neurological and carcinologic outcome. Subacute cerebellar ataxia, subacute sensitive neuropathy, limbic encephalomyelitis, Lambert-Eaton myasthenic syndrome, opsoclonus myoclonus, dermatopolymyositis and intestinal pseudo-obstruction belong to the well-characterized PNS and their presence must lead to onconeuronal antibodies (ONA) detection. Treatment of the underlying neoplasia is the mainstay of treatment to obtain a better outcome. However, recent findings lead to consider immunological specific treatment according to the subtype of associated ONA. PNS associated with ONA targeting membrane antigens are thus usually responsive to immunomodulator therapies. Conversely, PNS associated with ONA targeting intracellular antigens are of poor outcome despite mild improvement under immunosuppressive therapies in some patients. PMID:21036427

Didelot, A; Honnorat, J

2011-10-01

328

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 ...

329

Fanconi syndrome  

MedlinePLUS

De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

330

[Dietary therapy of epilepsy].  

PubMed

Reappraisal of ketogenic diets (KD) were delayed in Japan compared to USA and Korea. The reasons are unknown, but possible explanations are (1) Japanese food culture prefers rice and less fat and (2) ACTH therapy is preferred for West syndrome in Japan. Since Japanese child neurologists were surprised at dramatic effects on glucose transporter 1 deficiency syndrome (Glut-1DS) in 2003, KD have been slowly accepted for treatment of epilepsy in Japan. New generation KD including modified Atkins diet (mAD) are preferred to classical KD. KD can be causal therapy in Glut-1DS and some of mitochondrial disorders, though anti-epileptic drugs are symptomatic therapy. KD can alleviate intractable seizures in epilepsies with brain malformation in addition to West syndrome and Dravet syndrome, etc. KD may work for brain tumor, cancer, neurodegenerative disorders including Alzheimer's disease. C7-8 triglycerides or fatty acid esters are under development as medicines replacing KD. PMID:24912289

Imai, Katsumi; Ishihara, Eiko; Ikeda, Hiroko

2014-05-01

331

Parkinsonian Syndromes  

PubMed Central

Purpose of Review The different parkinsonian conditions can be challenging to separate clinically. This review highlights the important clinical features that guide the diagnosis of Parkinson disease (PD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD). Strategies for treatment and disease management are also discussed. Recent Findings Over the past decade there has been an increasing recognition of the broad clinical presentations of the neurodegenerative forms of parkinsonism. Nonmotor symptoms in these diseases, including psychiatric, cognitive, autonomic, and gastrointestinal dysfunction, appear to have a major impact on quality of life and disability. PSP and CBD are now considered pathologic diagnoses, with several different and varied clinical phenotypes, that overlap and share features with PDand frontotemporal dementia syndromes. PD is distinguished by its excellent response to dopaminergic medications that is maintained over many years, in contrast to the response seen in patients with MSA and PSP. New diagnostic criteria have been proposed for CBD. No new therapeutic interventions have emerged for PSP, MSA, or CBD. Infusional therapies and deep brain stimulation surgery are established therapies for advanced PD. Summary The parkinsonian syndromes encompass a number of nosologic entities that are grouped together on the basis of their shared clinical features but are separated on the basis of their different pathologies. Overall, the consideration of clinical signs, mode of disease onset, and nature of disease progression are all important to make a timely and definitive diagnosis. PMID:24092286

Williams, David R.; Litvan, Irene

2013-01-01

332

Combination Therapy with Hydrocortisone and Fludrocortisone Does Not Improve Symptoms in Chronic Fatigue Syndrome: A Randomized, Placebo-Controlled, Double-blind, Crossover Study  

Microsoft Academic Search

PURPOSE: Chronic fatigue syndrome has been associated with decreased function of the hypothalamic-pituitary-adrenal axis. Although neurally mediated hypotension occurs more fre- quently in patients with chronic fatigue syndrome than in con- trols, attempts to alleviate symptoms by administration of hy- drocortisone or fludrocortisone have not been successful. The purpose of this study was to investigate the effect of combina- tion

Daniel Blockmans; Philippe Persoons; Boudewijn Van Houdenhove; Marina Lejeune; Herman Bobbaers

333

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.

Gulp?nar, Omer; Kay?s, Aytac; Suer, Evren; Gokce, Mehmet Ilker; Guclu, Adil Gucal; Ar?kan, Nihat

2014-01-01

334

Turner's syndrome.  

PubMed

Turner syndrome (TS) is the most common sex-chromosome abnormality in females. Short stature and hypogonadism are the classical clinical findings. The spontaneous final height (FH) ranges between 139 and 147 cm, representing a growth deficit of about 20 cm with respect to the unaffected population. GH therapy improves FH and should be started during childhood at a high dose of about 1 IU/kg/week (range 0.6-2 IU/kg/week). Some authors advocate combined therapy with an anabolic steroid at various doses (e.g. oxandrolone 0.05-0.1 mg/kg/day). This treatment results in a significantly increased FH, a large proportion of treated girls reaching a FH of more than 150 cm. Gonadal function is compromised during adolescence in about 80% of girls with TS, whilst in about 20% pubertal development occurs spontaneously. Oestrogen therapy should be started at the age of 13-14 years in hypogonadic patients; early onset of treatment (before 12 years) seems to compromise FH. Other concerns in these patients are fertility and osteopenia. PMID:11529402

Guarneri, M P; Abusrewil, S A; Bernasconi, S; Bona, G; Cavallo, L; Cicognani, A; Di Battista, E; Salvatoni, A

2001-07-01

335

Platelet count doubling after the first cycle of azacitidine therapy predicts eventual response and survival in patients with myelodysplastic syndromes and oligoblastic acute myeloid leukaemia but does not add to prognostic utility of the revised IPSS.  

PubMed

Reliable clinical or molecular predictors of benefit from azacitidine therapy in patients with myelodysplastic syndromes (MDS) are not defined. Doubling of platelet count at start of second cycle of azacitidine therapy compared to baseline was associated with achieving response and survival advantage in a Dutch cohort. To validate this observation, we analysed a larger cohort of North American patients, whose data was collected in a prospective clinical trial with a longer median follow-up. We found a significant association between platelet count doubling after first cycle of azacitidine therapy and probability of achieving objective response. Among patients with MDS or oligoblastic acute myeloid leukaemia (<30% bone marrow blasts, n=102), there was a statistically significant reduction in risk of death for patients who achieved platelet count doubling (n=23, median OS, 210months) compared to those who did not (n=79, median OS, 167months, adjusted hazard ratio (no/yes)=188, 95% confidence interval, 103-340, P=004). Nonetheless, the addition of this platelet count doubling variable did not improve the survival prediction provided by the revised International Prognostic Scoring System or the French Prognostic Scoring System. Identification of reliable and consistent predictors for clinical benefit for azacitidine therapy remains an unmet medical need and a top research priority. PMID:24995683

Zeidan, Amer M; Lee, Ju-Whei; Prebet, Thomas; Greenberg, Peter; Sun, Zhuoxin; Juckett, Mark; Smith, Mitchell R; Paietta, Elisabeth; Gabrilove, Janice; Erba, Harry P; Katterling, Rhett P; Tallman, Martin S; Gore, Steven D

2014-10-01

336

Locked-In Syndrome  

MedlinePLUS

... for locked-in syndrome, nor is there a standard course of treatment. A therapy called functional neuromuscular stimulation, which uses ... endorsement by or an official position of the National Institute of Neurological ... Advice on the treatment or care of an individual patient should be ...

337

[Marfan syndrome].  

PubMed

Marfan's syndrome is a monogenetic disease with an autosomal dominant transmission generally accompanied by type I fibrillin abnormality. This widely-distributed molecule participates in the structure of connective tissues so that any aberration may result in disease of many systems: skeletal morphology, dislocation of the lens, neurological or cutaneous signs and dilatation of the aorta predisposing to dissection, mitral valve prolapse being a common association. The diagnosis, clinical because of the size of the culprit gene and the multiplicity of the possible mutations, is sometimes difficult, and diagnostic criteria have been proposed. It is important to make the diagnosis because treatment is based on the restriction of violent exercise, betablocker therapy and regular echocardiographic monitoring of the ascending aorta, the region at highest risk of dilatation and dissection. A family enquiry is essential to make the diagnosis before the onset of complications in pauci-symptomatic patients (great intra-familial variability). Pregnancy poses special problems in these patients. PMID:14694784

Jondeau, G; Boileau, C; Chevallier, B; Delorme, G; Digne, F; Guiti, C; Milleron, O; de SaintJean, M; Le Parc, J M; Moura, B

2003-11-01

338

What's New in Myelodysplastic Syndrome Research and Treatment?  

MedlinePLUS

... Next Topic Additional resources for myelodysplastic syndromes What`s new in myelodysplastic syndrome research and treatment? Genetics and ... research unfolds, it may be used to design new drugs or eventually in developing gene therapy. This ...

339

Turner Syndrome  

PubMed Central

Turner syndrome (TS) is a neurogenetic disorder characterized by partial or complete monosomy-X. TS is associated with certain physical and medical features including estrogen deficiency, short stature and increased risk for several diseases with cardiac conditions being among the most serious. Girls with TS are typically treated with growth hormone and estrogen replacement therapies to address short stature and estrogen deficiency. The cognitive-behavioral phenotype associated with TS includes strengths in verbal domains with impairments in visual-spatial, executive function and emotion processing. Genetic analyses have identified the short stature homeobox (SHOX) gene as being a candidate gene for short stature and other skeletal abnormalities associated with TS but currently the gene or genes associated with cognitive impairments remain unknown. However, significant progress has been made in describing neurodevelopmental and neurobiologic factors underlying these impairments and potential interventions are on the horizon. Less is known regarding psychosocial and psychiatric functioning in TS but essential aspects of psychotherapeutic treatment plans are suggested in this report. Future investigations of TS should include continued genetic studies such as microarray analyses and determination of candidate genes for both physical and cognitive features. Multimodal, interdisciplinary studies will be essential for identifying optimal, syndrome-specific interventions for improving the lives of individuals with TS. PMID:17562588

Kesler, SR

2007-01-01

340

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 ...

341

Down Syndrome  

MedlinePLUS

... Information Clinical Trials Resources and Publications En Espaol 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 ...

342

Dravet Syndrome  

MedlinePLUS

NINDS Dravet Syndrome Information Page Synonym(s): Severe Myoclonic Epilepsy of Infancy (SMEI) Table of Contents (click to ... Dravet Syndrome? Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of ...

343

Piriformis Syndrome  

MedlinePLUS

... Awards Enhancing Diversity Find People About NINDS NINDS Piriformis Syndrome Information Page Table of Contents (click to ... is being done? Clinical Trials Organizations What is Piriformis Syndrome? Piriformis syndrome is a rare neuromuscular disorder ...

344

Radiolabeled Monoclonal Antibody Therapy, Fludarabine Phosphate, and Low-Dose Total-Body Irradiation Followed By Donor Stem Cell Transplantation and Immunosuppression Therapy in Treating Older Patients With Advanced Acute Myeloid Leukemia or High-Risk Myelodysplastic Syndrome  

ClinicalTrials.gov

Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndromes; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Excess Blasts in Transformation; Refractory Anemia With Ringed Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Secondary Myelodysplastic Syndromes; Untreated Adult Acute Myeloid Leukemia

2014-03-03

345

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; Krolicki, Andrzej; Maleszka, Romuald; Bielecka-Grzela, Stanislawa

2013-01-01

346

[Irritable bowel syndrome].  

PubMed

The prevalence of irritable bowel syndrome (IBS) ranges in all countries of the world between 6 and 25%, in Germany between 15 and 22%. The divergent rates of prevalence are mainly due to different definitions of IBS in epidemiological studies. In Germany, 20-50% of persons with IBS symptoms seek medical help. IBS patients produce high direct and indirect costs. The following psychophysiological mechanisms of IBS are presumed to be empirically validated: visceral hypersensitivity, postinfectious sequelae, psychiatric disorders, and psychosocial stress. Tricyclic antidepressant agents and psychotherapy (hypnosis, cognitive behavioral therapy, and psychodynamic therapy) are effective for treatment of IBS forms dominated by pain. In one controlled study, the combination of pharmacological therapy and cognitive behavioral therapy was superior to pharmacological therapy alone. PMID:15067533

Huser, W; Lempa, M

2004-04-01

347

Occupational Therapy Occupational Therapy  

E-print Network

Occupational Therapy Occupational Therapy When you work as an Occupational Therapist, you use and possibilities are endless! Program The UWM OT program has a rich history in Occupational Therapy education Therapy Association (AOTA). U.S. News and World Report has ranked the UWM Occupational Therapy Graduate

Saldin, Dilano

348

Treatment Approaches in Down's Syndrome: A Review.  

ERIC Educational Resources Information Center

The paper reviews research into treatment approaches in Down's Syndrome. Pharmacological treatments reviewed include thyroid therapy, 5-hydroxytryptophan, vitamin therapy, and cell therapy. Other treatments considered are movement patterning, early intervention, and facial surgery. Early educational intervention is seen as the most effective

Foreman, Philip J.; Ward, James

1986-01-01

349

Blood Pressure in Children with Minimal Change Nephrotic Syndrome during Oedema and after Steroid Therapy: The Influence of Familial Essential Hypertension  

Microsoft Academic Search

Aims: To verify the variations of blood pressure in children with minimal change nephrotic syndrome and to correlate the blood pressure with familial history of essential hypertension. Methods: We measured blood pressure in 49 prepubertal nephrotic children, 17 females and 32 males, in the first week of oedema, and after 4 weeks of ISKDC (International Study of Kidney Disease in

Laure Monthe Kontchou; Giulia Liccioli; Ivana Pela

2009-01-01

350

Estrogen therapy enhances calcium absorption and retention and diminishes bone turnover in young girls with turner's syndrome: A calcium kinetic study  

Microsoft Academic Search

Using stable tracers of calcium, we have previously shown a significant increase in calcium absorption and retention in prepubertal boys treated with exogenous testosterone. To investigate the effects of estrogen replacement on measures of calcium absorption, retention, and bone turnover, we studied a group of seven hypogonadal girls with Turner's syndrome (mean SE age, 12.5 0.7 years). At baseline,

Nelly Mauras; Nancy E. Vieira; Alfred L. Yergey

1997-01-01

351

The Need for Self-Report Data in the Assessment of Stuttering Therapy Efficacy: Repetitions and Prolongations of Speech. The Stuttering Syndrome  

ERIC Educational Resources Information Center

Background: Bloodstein reviewed hundreds of studies that investigated the efficacy of therapeutic protocols for ameliorating the stuttering syndrome. Surprisingly, almost all were effective in significantly reducing overtly perceptible behaviours such as repetitions and prolongations of speech sounds. These results seem highly improbable

Guntupalli, Vijaya K.; Kalinowski, Joseph; Saltuklaroglu, Tim

2006-01-01

352

Pilot study of combination transcriptional modulation therapy with sodium phenylbutyrate and 5-azacytidine in patients with acute myeloid leukemia or myelodysplastic syndrome  

Microsoft Academic Search

Epigenetic mechanisms underlying tumorigenesis have recently received much attention as potential therapeutic targets of human cancer. We designed a pilot study to target DNA methylation and histone deacetylation through the sequential administration of 5-azacytidine followed by sodium phenylbutyrate (PB) in patients with acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS). Ten evaluable patients (eight AML, two MDS) were treated with

P Maslak; S Chanel; L H Camacho; S Soignet; P P Pandolfi; I Guernah; R Warrell; S Nimer

2006-01-01

353

ETUDE DE LA DYSMORPHOSE CRANIOFACIALE CHEZ LE RAT DUMBO.  

E-print Network

??RESUME Le rat Dumbo prsente un aspect malformatif voquant certains syndromes crnio-faciaux humains. La comprhension du phnotype Dumbo pourrait expliquer les vnements cellulaires et molculaires (more)

Katerji, Suhair

2009-01-01

354

Neonatal opioid withdrawal syndrome.  

PubMed

Neonatal opioid withdrawal syndrome is common due to the current opioid addiction epidemic. Infants born to women covertly abusing prescription opioids may not be identified as at risk until withdrawal signs present. Buprenorphine is a newer treatment for maternal opioid addiction and appears to result in a milder withdrawal syndrome than methadone. Initial treatment is with nonpharmacological measures including decreasing stimuli, however pharmacological treatment is commonly required. Opioid monotherapy is preferred, with phenobarbital or clonidine uncommonly needed as adjunctive therapy. Rooming-in and breastfeeding may decease the severity of withdrawal. Limited evidence is available regarding long-term effects of perinatal opioid exposure. PMID:24845493

Sutter, Mary Beth; Leeman, Lawrence; Hsi, Andrew

2014-06-01

355

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

356

Goldenhar's syndrome.  

PubMed

We present a report on 16 patients with Goldenhar's syndrome. The criteria we required for the diagnosis of Goldenhar's syndrome consisted of an eye abnormality (lipoma, lipodermoid, epibulbar dermoid, or upper eyelid coloboma) associated with ear, mandibular, or vertebral anomalies (two of the three). Although Treacher Collins' syndrome can be easily differentiated from Goldenhar's syndrome, the differences between Goldenhar's syndrome and hemifacial microsomia are more difficult to delineate. PMID:626178

Feingold, M; Baum, J

1978-02-01

357

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

358

Marfan's syndrome: an overview.  

PubMed

Marfan's syndrome is an autosomal dominant condition with an estimated prevalence of one in 10,000 to 20,000 individuals. This rare hereditary connective tissue disorder affects many parts of the body. The diagnosis of Marfan's syndrome is established in accordance with a review of the diagnostic criteria, known as the Ghent nosology, through a comprehensive assessment largely based on a combination of major and minor clinical manifestations in various organ systems and the family history. Aortic root dilation and mitral valve prolapse are the main presentations among the cardiovascular malformations of Marfan's syndrome. The pathogenesis of Marfan's syndrome has not been fully elucidated. However, fibrillin-1 gene mutations are believed to exert a dominant negative effect. Therefore, Marfan's syndrome is termed a fibrillinopathy, along with other connective tissue disorders with subtle differences in clinical manifestations. The treatment may include prophylactic ?-blockers and angiotensin II-receptor blockers in order to slow down the dilation of the ascending aorta, and prophylactic aortic surgery. Importantly, ?-blocker therapy may reduce TGF-? activation, which has been recognized as a contributory factor in Marfan's syndrome. The present article aims to provide an overview of this rare hereditary disorder. PMID:21308160

Yuan, Shi-Min; Jing, Hua

2010-12-01

359

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

360

Growth Hormone Normalises Height, Prediction of Final Height and Hand Length in Children with Prader-Willi Syndrome after 4 Years of Therapy  

Microsoft Academic Search

Background: Based on the reported favourable effects of growth hormone (GH) treatment on growth and body composition in Prader-Labhart-Willi syndrome, we studied age dependency and the long-term effects on growth dynamics to elucidate the assumed hypothalamic GH deficiency. Methods: We examined 23 children treated with hGH (24 U\\/m2\\/week) during a median of 4 (range 1.55.5) years; group 1: 10 young

Urs Eiholzer; Dagmar lAllemand

2000-01-01

361

Mucocutaneous autoimmune syndrome following fludarabine therapy for low-grade non-Hodgkin's lymphoma of B-cell type (B-NHL)  

Microsoft Academic Search

A 40-year-old patient with low-grade B-NHL developed a generalized macular-papular rash following the first cycle of fludarabine\\u000a treatment which progressed to a complete epidermal necrolysis following the second cycle. Clinical symptoms and the results\\u000a of the direct and indirect immunofluorescence were consistent with a mucocutaneous autoimmune syndrome (pemphigus). Immunohistochemical\\u000a analysis demonstrated a dense epidermal infiltration of CD8+ lymphocytes associated with

J. Braess; K. Reich; S. Willert; F. Strutz; C. Neumann; W. Hiddemann; B. Wrmann

1997-01-01

362

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

363

Interventional approaches to the management of myofascial pain syndrome  

Microsoft Academic Search

Interventional therapies are a valuable addition to our armamentarium when treating myofascial pain syndromes. When combined\\u000a with other therapies, interventional techniques can be an effective adjunct in the multidisciplinary management of pain. This\\u000a article describes current interventional therapies that are employed in treating myofascial pain syndromes. The mainstay of\\u000a injection therapies, the myofascial trigger point injection, is emphasized. More recent

Christopher M. Criscuolo

2001-01-01

364

The Brugada syndrome revisited.  

PubMed

The Brugada syndrome is a rare but well-defined cause of sudden cardiac death. The key underlying abnormality is a decrease in net depolarising current due to a genetic defect, though recent evidence also implicates structural abnormalities in some patients. Diagnosis requires a Brugada-type ECG as well as typical clinical features: such clinical considerations are currently key in guiding risk stratification and hence management. Whilst pharmacological therapies are under investigation, the only intervention with a robust evidence base remains insertion of an implantable cardioverter defibrillator. Further research will be required to allow more effective risk stratification and hence more rational therapy. PMID:24332084

Mashar, Meghavi; Kwok, Andrew J; Pinder, Richard; Sabir, Ian

2014-07-01

365

[Economy class syndrome].  

PubMed

Economy class syndrome is venous thromboembolism following air travel. This syndrome was firstly reported in 1946, and many cases have been reported since 1990s. Low air pressure and low humidity in the aircraft cabin may contribute to the mechanism of this syndrome. Risk factors for venous thrombosis in the plane were old age, small height, obesity, hormonal therapy, malignancy, smoking, pregnancy or recent parturition, recent trauma or operation, chronic disease and history of venous thrombosis. In Japan, the feminine gender is also risk factor though reason was not well known. For prophylaxis, adequate fluid intake and leg exercise are recommended to all passengers. For passengers with high risk, prophylactic measures such as compression stockings, aspirin or low molecular weight heparin should be considered. PMID:14577308

Morio, Hiroshi

2003-10-01

366

Isaacs' syndrome in pregnancy.  

PubMed

Isaacs' syndrome is a rare neuromuscular disorder of continuous muscle fibre activity resulting from peripheral nerve hyperexcitability. Symptoms commonly include myokymia (muscle twitching at rest), pseudomyotonia (delayed muscle relaxation), muscle cramps and stiffness. It is caused by voltage-gated potassium channel dysfunction and may be inherited or acquired. Treatment commonly includes anticonvulsants, immunosuppressive therapy and plasma exchange. To date only two cases of Isaacs' syndrome in pregnancy have been reported. We present a case of maternal Isaacs' along with a review of the literature. There are few reports of Isaacs' syndrome in pregnancy, but all are associated with favourable outcomes. Given the autosomal dominant inheritance pattern, genetic counselling of the gravida is recommended. Anticonvulsant may have to be used in pregnancy, and given the potential teratogenicity with several of these agents; preference should be given to newer drugs such as lamotrigine. PMID:25301428

Lide, Brianna; Singh, Jasbir; Haeri, Sina

2014-01-01

367

[History of nephrotic syndrome].  

PubMed

The history of nephrotic syndrome can go back to beginning of the era. In old Alexandria, urine was conceived to be produced in kidneys. In 1827, Bright classified diseases with proteinuria into three categories, and lipoid nephrosis seemed to be included in group 1. Mechanisms of urinary protein leakage have been in great controvesies, and nowadays defects of slit-membrane protein are supposed to play important roles. In the middle of the 20th centuries, electron microscopy and renal biopsy technique were adopted in practical use. Since then, the treatment and research of nephrotic syndrome have been remarkably progressed. Clinical strategies including diet therapy, immuno-suppressive and antihypertensive drugs and other modalities have improved the prognosis of nephrotic syndrome. PMID:15500117

Nihei, Hiroshi; Nitta, Kousaku; Yumura, Wako

2004-10-01

368

Lipid Replacement and Antioxidant Nutritional Therapy for Restoring Mitochondrial Function and Reducing Fatigue in Chronic Fatigue Syndrome and Other Fatiguing Illnesses  

Microsoft Academic Search

Evidence in the literature indicates that diminished mitochondrial function through loss of efficiency in the electron transport chain caused by oxidation occurs during aging and in fatiguing illnesses. Lipid Replacement Therapy (LRT) administered as a nutritional supplement with antioxidants can prevent oxidative membrane damage, and LRT can be used to restore mitochondrial and other cellular membrane functions via delivery of

Garth L. Nicolson; Rita Ellithorpe

2006-01-01

369

Facilitating Optimal Care of Acute Coronary, Cerebrovascular and Peripheral Vascular Syndromes in the Emergency Department: The Role of Oral Antiplatelet Therapy  

Microsoft Academic Search

The benefits of aspirin use in the emergent care of MI and stroke have been well established. Recent studies have further demonstrated the importance of antiplatelet therapy in the acute setting, primarily with the use of intravenous glycoprotein IIb\\/IIIa receptor inhibitors. Aspirin and the thienopyridines (ticlopidine and clopidogrel) are oral antiplatelet agents that interfere with platelet activation in complementary, but

Gregory A. Volturo; Richard V. Aghababian

2004-01-01

370

Genistin-Rich Soy Isoflavone Extract in Substrate Reduction Therapy for Sanfilippo Syndrome: An Open-Label, Pilot Study in 10 Pediatric Patients  

Microsoft Academic Search

Background: Mucopolysaccharidoses (MPSs) are a group of severe metabolic disorders caused by deficiencies in enzymes involved in the degradation of glycosamino- glycans (GAGs) long chains of sugar carbohydrates in cells that help build bone, cartilage, tendons, corneas, skin, and connective tissue. Although enzyme replacement therapy has become available for the treatment of some types of MPS, effective treat- ment

Ewa Piotrowska; Joanna Jakbkiewicz-Baneck; Anna Tylki-Szymanska; Anna Liberek; Agnieszka Maryniak; Marcelina Malinowska; Barbara Czartoryska; Ewa Puk; Anna Kloska; Tomasz Liberek; Sylwia Baranska; Alicja Wegrzyn; Grzegorz Wegrzyn

2008-01-01

371

Genistin-rich soy isoflavone extract in substrate reduction therapy for Sanfilippo syndrome: An open-label, pilot study in 10 pediatric patients  

Microsoft Academic Search

Background: Mucopolysaccharidoses (MPSs) are a group of severe metabolic disorders caused by deficiencies in enzymes involved in the degradation of glycosaminoglycans (GAGs)long chains of sugar carbohydrates in cells that help build bone, cartilage, tendons, corneas, skin, and connective tissue. Although enzyme replacement therapy has become available for the treatment of some types of MPS, effective treatment of neurodegenerative forms of

Ewa Piotrowska; Joanna Jakbkiewicz-Banecka; Anna Tylki-Szymanska; Anna Liberek; Agnieszka Maryniak; Marcelina Malinowska; Barbara Czartoryska; Ewa Puk; Anna Kloska; Tomasz Liberek; Sylwia Baranska; Alicja Wegrzyn; Grzegorz Wegrzyn

2008-01-01

372

ADCK4 "reenergizes" nephrotic syndrome.  

PubMed

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

2013-12-01

373

Atrioventricular junction ablation and pacemaker implantation for heart failure associated with atrial fibrillation: potential issues and therapies in the setting of acute heart failure syndrome.  

PubMed

Atrial fibrillation is the most common arrhythmia and is especially clinically important in patients with heart failure. Prolonged atrial fibrillation with high ventricular rate response may lead to development or worsening of left ventricular function. If adequate heart rate control cannot be obtained medically, often patients will undergo pacemaker implant and catheter ablation of the atrioventricular junction. This intervention can have profound effects on the course of heart failure. This article reviews the technique, complications, outcome data, and alternatives to this management strategy. The potential role of this therapeutic modality in those hospitalized with acute heart failure syndromes is discussed. PMID:21424742

Rubenstein, Jason C; Roth, James A

2011-09-01

374

Monoclonal Antibody Therapy in Treating Patients With Ovarian Epithelial Cancer, Melanoma, Acute Myeloid Leukemia, Myelodysplastic Syndrome, or Non-Small Cell Lung Cancer  

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); Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Recurrent Melanoma; Recurrent Non-small Cell Lung Cancer; Recurrent Ovarian Epithelial Cancer; Stage IV Melanoma; Stage IV Non-small Cell Lung Cancer

2013-01-09

375

Klinefelter syndrome: Case report  

PubMed Central

SUMMARY Objectives. The aim of this work is to describe the problems of child dentistry affected by Klinefelter's syndrome. Methodos. Through the presentation of a clinical case that was followed by a Pediatric Dentistry, University Sapienza of Rome. Result. Interceptive therapy aims to eliminate the functional interference that occurs during growth. Conclusion. For this reason its a must for the dentist to carefully monitor the oral health of the small patient immediately. PMID:23285366

CAPASSO, F.; PANETTA, F.; IERARDO, G.; PARISELLA, V.; POLIMENI, A.

2010-01-01

376

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. ...

377

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 ...

378

Sjogren's Syndrome  

MedlinePLUS

... the prognosis? What research is being done? Clinical Trials Organizations Additional resources from MedlinePlus What is Sjgren's Syndrome? Sjgren's syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce ...

379

Paraneoplastic Syndromes  

MedlinePLUS

... the prognosis? What research is being done? Clinical Trials Organizations What are Paraneoplastic Syndromes? Paraneoplastic syndromes are a group of rare disorders that are triggered by an abnormal immune system response to a cancerous tumor known as ...

380

Sjgren's Syndrome  

MedlinePLUS

... syndrome sometimes develops as a complication of another autoimmune disorder. Symptoms vary in type and intensity, but many ... syndrome is not known, but it is an autoimmune disorder. People with this disease have abnormal proteins in ...

381

Asperger Syndrome  

MedlinePLUS

... and symptoms of Asperger syndrome is given the diagnosis of Autism Spectrum Disorder (ASD). If a person was diagnosed with Asperger syndrome before May 2013, his or her diagnosis stays the same, but can be considered ASD ...

382

Asperger Syndrome  

MedlinePLUS

... is Asperger syndrome? Asperger syndrome (AS) is an autism spectrum disorder (ASD), one of a distinct group ... and stereotyped patterns of behavior. Other ASDs include autistic disorder, childhood disintegrative disorder, and pervasive developmental disorder not ...

383

Fat embolism syndrome in delirium tremens.  

PubMed

Delirium tremens in a common feature in the alcoholic population. The Fat Embolism Syndrome (FES) is characterized by fever, encephalopathy, respiratory failure and skin petechiae. Fat embolism has been associated with alcoholics but the diagnosis was apparent only at autopsy. We present an alcoholic male who developed delirium tremens unresponsive to therapy, followed by features of the FES. Asterixis and Korsakoff's psychosis are newly described features of this syndrome. Corticosteroids were a definitive therapy in this case. PMID:607798

Horowitz, I; Klingenstein, R J; Levy, R; Zimmerman, M J

1977-11-01

384

Transient Fanconi syndrome in Quarter horses.  

PubMed

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

2014-02-01

385

Developmental syndromes: growth hormone deficiency and treatment.  

PubMed

Developmental syndromes are characterized by numerous phenotypical signs and malformations. In most of them such as Turner, Noonan, Prader-Willi, Silver-Russel, Williams, Kabuki, Leri-Weill syndrome and skeletal dysplasias, short stature is a common feature. Growth defect is very often related to a defect in cellular growth, but some unknown abnormality in GH action is possible. Recently, the greater availability of recombinant GH has expanded the interest towards GH secretion and therapy also in developmental syndromes. We recognize syndromes associated with GH deficiency (GHD), showing a developmental midline defect such as Pallister-Hall syndrome, septo-optic dysplasia, but many of these conditions do not have a convincing link with GHD. Moreover, some conditions, in particular the well-studied Turner syndrome, that do not have a real GHD, have proven to benefit from GH therapy at supra-physiological doses obtaining a higher final height than the expected one according to the natural history. This has expanded the indications for GH therapy. The aim of our paper is to review the literature on GH secretion, on the effects and costs-benefits of GH therapy in many dysmorphic syndromes, presenting some results of GH secretion and therapy obtained in our experience. PMID:19293579

Mazzanti, Laura; Tamburrino, Federica; Bergamaschi, Rosalba; Scarano, Emanuela; Montanari, Francesca; Torella, Michele; Ballarini, Elisa; Cicognani, Alessandro

2009-01-01

386

A review on cognitive behavorial therapy (CBT) and graded exercise therapy (GET) in myalgic encephalomyelitis (ME) / chronic fatigue syndrome (CFS): CBT/GET is not only ineffective and not evidence-based, but also potentially harmful for many patients with ME/CFS.  

PubMed

Benign Myalgic Encephalomyelitis (ME) / Chronic Fatigue Syndrome (CFS) is a debilitating disease which, despite numerous biological abnormalities has remained highly controversial. Notwithstanding the medical pathogenesis of ME/CFS, the (bio)psychosocial model is adopted by many governmental organizations and medical profes-sio-nals to legitimize the combination of Cognitive Behavioral Therapy (CBT) and Graded Exercise Therapy (GET) for ME/CFS. Justified by this model CBT and GET aim at eliminating presumed psychogenic and socially induced maintaining factors and reversing deconditioning, respectively. In this review we invalidate the (bio)psychosocial model for ME/CFS and demonstrate that the success claim for CBT/GET to treat ME/CFS is unjust. CBT/GET is not only hardly more effective than non-interventions or standard medical care, but many patients report that the therapy had affected them adversely, the majority of them even reporting substantial deterioration. Moreover, this review shows that exertion and thus GET most likely have a negative impact on many ME/CFS patients. Exertion induces post-exertional malaise with a decreased physical performan-ce/aerobic capacity, increased muscoskeletal pain, neurocognitive impairment, "fatigue", and weakness, and a long lasting "recovery" time. This can be explained by findings that exertion may amplify pre-existing pa-thophysiological abnormalities underpinning ME/CFS, such as inflammation, immune dysfunction, oxidative and nitrosative stress, channelopathy, defec-tive stress response mechanisms and a hypoactive hypothalamic-pituitary-adrenal axis. We conclude that it is unethical to treat patients with ME/CFS with ineffective, non-evidence-based and potentially harmful "rehabilitation therapies", such as CBT/GET. PMID:19855350

Twisk, Frank N M; Maes, Michael

2009-01-01

387

Sweet syndrome as a manifestation of azathioprine hypersensitivity.  

PubMed

Sweet syndrome is a reactive, sterile, pustular dermatosis that occurs in association with infection, malignancy, or connective tissue disease or in response to the use of certain medications. Sweet syndrome secondary to azathioprine therapy is rarely reported. We describe 3 patients, 2 with inflammatory bowel disease and 1 with myasthenia gravis, who developed febrile pustulosis consistent with Sweet syndrome 1 to 2 weeks after treatment with azathioprine. Antibiotic therapy failed in all patients, but Sweet syndrome resolved with drug withdrawal and prednisone therapy. Because azathioprine is widely used, clinicians should be aware of this adverse reaction. PMID:18775203

El-Azhary, Rokea A; Brunner, Kelly L; Gibson, Lawrence E

2008-09-01

388

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

389

Metabolic Syndrome  

MedlinePLUS

... metabolic syndrome fact sheet hOW is the metAbOlic syndrOme treAted? Increasing physical activity and losing weight are the best ways to begin to manage your condition. Medications can also treat risk factors such as ... factors for the metabolic syndrome, talk with your doctor. Your doctor can run ...

390

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 ...

391

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. ...

392

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

393

Nijmegen breakage syndrome (NBS).  

PubMed

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive syndrome of chromosomal instability mainly characterized by microcephaly at birth, combined immunodeficiency and predisposition to malignancies. Due to a founder mutation in the underlying NBN gene (c.657_661del5) the disease is encountered most frequently among Slavic populations. The principal clinical manifestations of the syndrome are: microcephaly, present at birth and progressive with age, dysmorphic facial features, mild growth retardation, mild-to-moderate intellectual disability, and, in females, hypergonadotropic hypogonadism. Combined cellular and humoral immunodeficiency with recurrent sinopulmonary infections, a strong predisposition to develop malignancies (predominantly of lymphoid origin) and radiosensitivity are other integral manifestations of the syndrome. The NBN gene codes for nibrin which, as part of a DNA repair complex, plays a critical nuclear role wherever double-stranded DNA ends occur, either physiologically or as a result of mutagenic exposure. Laboratory findings include: (1) spontaneous chromosomal breakage in peripheral T lymphocytes with rearrangements preferentially involving chromosomes 7 and 14, (2) sensitivity to ionizing radiation or radiomimetics as demonstrated in vitro by cytogenetic methods or by colony survival assay, (3) radioresistant DNA synthesis, (4) biallelic hypomorphic mutations in the NBN gene, and (5) absence of full-length nibrin protein. Microcephaly and immunodeficiency are common to DNA ligase IV deficiency (LIG4 syndrome) and severe combined immunodeficiency with microcephaly, growth retardation, and sensitivity to ionizing radiation due to NHEJ1 deficiency (NHEJ1 syndrome). In fact, NBS was most commonly confused with Fanconi anaemia and LIG4 syndrome. Genetic counselling should inform parents of an affected child of the 25% risk for further children to be affected. Prenatal molecular genetic diagnosis is possible if disease-causing mutations in both alleles of the NBN gene are known. No specific therapy is available for NBS, however, hematopoietic stem cell transplantation may be one option for some patients. Prognosis is generally poor due to the extremely high rate of malignancies. PMID:22373003

Chrzanowska, Krystyna H; Gregorek, Hanna; Dembowska-Bagi?ska, Bo?enna; Kalina, Maria A; Digweed, Martin

2012-01-01

394

Mitochondrial Gene Therapy Improves Respiration, Biogenesis, and Transcription in G11778A Leber's Hereditary Optic Neuropathy and T8993G Leigh's Syndrome Cells  

PubMed Central

Abstract Many incurable mitochondrial disorders result from mutant mitochondrial DNA (mtDNA) and impaired respiration. Leigh's syndrome (LS) is a fatal neurodegenerative disorder of infants, and Leber's hereditary optic neuropathy (LHON) causes blindness in young adults. Treatment of LHON and LS cells harboring G11778A and T8993G mutant mtDNA, respectively, by >90%, with healthy donor mtDNA complexed with recombinant human mitochondrial transcription factor A (rhTFAM), improved mitochondrial respiration by ?1.2-fold in LHON cells and restored >50% ATP synthase function in LS cells. Mitochondrial replication, transcription, and translation of key respiratory genes and proteins were increased in the short term. Increased NRF1, TFAMB1, and TFAMA expression alluded to the activation of mitochondrial biogenesis as a mechanism for improving mitochondrial respiration. These results represent the development of a therapeutic approach for LHON and LS patients in the near future. PMID:22390282

Bergquist, Kristen; Young, Kisha; Gnaiger, Erich; Rao, Raj R.

2012-01-01

395

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

396

Rett Syndrome  

MedlinePLUS

... therapy can help children develop skills needed for performing self-directed activities (such as dressing, feeding, and practicing arts and crafts), while physical therapy and hydrotherapy may ...

397

Management of neonatal abstinence syndrome from opioids.  

PubMed

Most infants at risk for neonatal abstinence syndrome have opioid plus another drug exposure; polypharmacy is the rule rather than the exception. Scales for evaluation of neonatal abstinence syndrome are primarily based for opioid withdrawal. A standard protocol to treat neonatal abstinence syndrome has not been developed. Institute nonpharmacologic strategies for all neonates at risk. The American Academy of Pediatrics recommends mechanism-directed therapy (treat opioid withdrawal with an opioid) as the first-line therapy. Second-line medications are currently under evaluation. PMID:23972754

Grim, Kendra; Harrison, Tracy E; Wilder, Robert T

2013-09-01

398

Serotonin Syndrome  

PubMed Central

Background Serotonin syndrome is a potentially life-threatening syndrome that is precipitated by the use of serotonergic drugs and overactivation of both the peripheral and central postsynaptic 5HT-1A and, most notably, 5HT-2A receptors. This syndrome consists of a combination of mental status changes, neuromuscular hyperactivity, and autonomic hyperactivity. Serotonin syndrome can occur via the therapeutic use of serotonergic drugs alone, an intentional overdose of serotonergic drugs, or classically, as a result of a complex drug interaction between two serotonergic drugs that work by different mechanisms. A multitude of drug combinations can result in serotonin syndrome. Methods This review describes the presentation and management of serotonin syndrome and discusses the drugs and interactions that can precipitate this syndrome with the goal of making physicians more alert and aware of this potentially fatal yet preventable syndrome. Conclusion Many commonly used medications have proven to be the culprits of serotonin syndrome. Proper education and awareness about serotonin syndrome will improve the accuracy of diagnosis and promote the institution of the appropriate treatment that may prevent significant morbidity and mortality. PMID:24358002

Volpi-Abadie, Jacqueline; Kaye, Adam M.; Kaye, Alan David

2013-01-01

399

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

400

The physical activity levels among people living with human immunodeficiency virus/acquired immunodeficiency syndrome receiving high active antiretroviral therapy in Rwanda  

PubMed Central

The accessibility of high active antiretroviral therapy (HAART) for local human immunodeficiency virus (HIV) patients is improving in Rwanda. It is well known that this therapy is associated with serious adverse effects, such as metabolic and morphologic changes. One of the recommended preventive modalities for these complications is participation in physical activity. The current study aims to determine the anthropometric profile and physical activity levels among people living with HIV and receiving HAART in Kigali, Rwanda. The study was a cross-sectional, descriptive quantitative survey. The participant's levels of physical activity participation and their association with anthropometric profiles were measured, using a structured self-administered questionnaire for 407 clients passing through the clinics. Of the participants, approximately 70% were inactive and in addition, 40% were obese and 43% overweight. Obesity was found to be strongly associated with inactivity. Lack of motivation, and time as well as fear of worsening the disease were found to be barriers to participation in physical activity. PMID:24521093

Frantz, J.M.; Murenzi, A.

2014-01-01

401

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

402

Barth syndrome  

PubMed Central

First described in 1983, Barth syndrome (BTHS) is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM), skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA). Fewer than 200 living males are known worldwide, but evidence is accumulating that the disorder is substantially under-diagnosed. Clinical features include variable combinations of the following wide spectrum: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), endocardial fibroelastosis (EFE), left ventricular non-compaction (LVNC), ventricular arrhythmia, sudden cardiac death, prolonged QTc interval, delayed motor milestones, proximal myopathy, lethargy and fatigue, neutropenia (absent to severe; persistent, intermittent or perfectly cyclical), compensatory monocytosis, recurrent bacterial infection, hypoglycaemia, lactic acidosis, growth and pubertal delay, feeding problems, failure to thrive, episodic diarrhoea, characteristic facies, and X-linked family history. Historically regarded as a cardiac disease, BTHS is now considered a multi-system disorder which may be first seen by many different specialists or generalists. Phenotypic breadth and variability present a major challenge to the diagnostician: some children with BTHS have never been neutropenic, whereas others lack increased 3-MGCA and a minority has occult or absent CM. Furthermore, BTHS was first described in 2010 as an unrecognised cause of fetal death. Disabling mutations or deletions of the tafazzin (TAZ) gene, located at Xq28, cause the disorder by reducing remodeling of cardiolipin, a principal phospholipid of the inner mitochondrial membrane. A definitive biochemical test, based on detecting abnormal ratios of different cardiolipin species, was first described in 2008. Key areas of differential diagnosis include metabolic and viral cardiomyopathies, mitochondrial diseases, and many causes of neutropenia and recurrent male miscarriage and stillbirth. Cardiolipin testing and TAZ sequencing now provide relatively rapid diagnostic testing, both prospectively and retrospectively, from a range of fresh or stored tissues, blood or neonatal bloodspots. TAZ sequencing also allows female carrier detection and antenatal screening. Management of BTHS includes medical therapy of CM, cardiac transplantation (in 14% of patients), antibiotic prophylaxis and granulocyte colony-stimulating factor (G-CSF) therapy. Multidisciplinary teams/clinics are essential for minimising hospital attendances and allowing many more individuals with BTHS to live into adulthood. PMID:23398819

2013-01-01

403

Barth syndrome.  

PubMed

First described in 1983, Barth syndrome (BTHS) is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM), skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA). Fewer than 200 living males are known worldwide, but evidence is accumulating that the disorder is substantially under-diagnosed. Clinical features include variable combinations of the following wide spectrum: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), endocardial fibroelastosis (EFE), left ventricular non-compaction (LVNC), ventricular arrhythmia, sudden cardiac death, prolonged QTc interval, delayed motor milestones, proximal myopathy, lethargy and fatigue, neutropenia (absent to severe; persistent, intermittent or perfectly cyclical), compensatory monocytosis, recurrent bacterial infection, hypoglycaemia, lactic acidosis, growth and pubertal delay, feeding problems, failure to thrive, episodic diarrhoea, characteristic facies, and X-linked family history. Historically regarded as a cardiac disease, BTHS is now considered a multi-system disorder which may be first seen by many different specialists or generalists. Phenotypic breadth and variability present a major challenge to the diagnostician: some children with BTHS have never been neutropenic, whereas others lack increased 3-MGCA and a minority has occult or absent CM. Furthermore, BTHS was first described in 2010 as an unrecognised cause of fetal death. Disabling mutations or deletions of the tafazzin (TAZ) gene, located at Xq28, cause the disorder by reducing remodeling of cardiolipin, a principal phospholipid of the inner mitochondrial membrane. A definitive biochemical test, based on detecting abnormal ratios of different cardiolipin species, was first described in 2008. Key areas of differential diagnosis include metabolic and viral cardiomyopathies, mitochondrial diseases, and many causes of neutropenia and recurrent male miscarriage and stillbirth. Cardiolipin testing and TAZ sequencing now provide relatively rapid diagnostic testing, both prospectively and retrospectively, from a range of fresh or stored tissues, blood or neonatal bloodspots. TAZ sequencing also allows female carrier detection and antenatal screening. Management of BTHS includes medical therapy of CM, cardiac transplantation (in 14% of patients), antibiotic prophylaxis and granulocyte colony-stimulating factor (G-CSF) therapy. Multidisciplinary teams/clinics are essential for minimising hospital attendances and allowing many more individuals with BTHS to live into adulthood. PMID:23398819

Clarke, Sarah L N; Bowron, Ann; Gonzalez, Iris L; Groves, Sarah J; Newbury-Ecob, Ruth; Clayton, Nicol; Martin, Robin P; Tsai-Goodman, Beverly; Garratt, Vanessa; Ashworth, Michael; Bowen, Valerie M; McCurdy, Katherine R; Damin, Michaela K; Spencer, Carolyn T; Toth, Matthew J; Kelley, Richard I; Steward, Colin G

2013-01-01

404

Behcet's Syndrome.  

PubMed

Behcet's syndrome (BS) is a vasculitis, seen more commonly around the Mediterranean and the Far East, and manifests with oral and genital ulcerations, skin lesions, uveitis, and vascular, central nervous system and gastrointestinal involvement. Its natural history of getting less severe over time, more severe disease in males and lack of specific diagnostic testing separates it from other commonly seen conditions in rheumatology. Most of the serious manifestations respond well to immunosuppression, and these are the mainstays of treatment for BS. BS is more prevalent in regions along the Silk Road, from the Mediterranean to the Far East. The genetic risk factor most strongly associated with BS is the human leukocyte antigen (HLA)-B51 allele. While genetic factors seem to play a role in the development of certain features of BS, there is general consensus that as yet unidentified environmental stimuli are necessary for initiation of disease. Proposed exogenous triggers include both bacterial and viral infections, which may then lead to dysregulation of the immune system, ultimately leading to the phenotypic expression of disease. The clinical manifestations of BS are protean in nature. While most patients develop mucocutaneous and genital ulcers along with eye disease, other patients may also present with arthritis, frank vasculitis, thrombophlebitis and CNS disease. Interestingly, the manifestations of this illness vary considerably based on gender and ethnicity. As the phenotypic expression among patients with BS is quite heterogeneous, pharmacological therapy is variable and dependent upon the severity of the disease as well as organ involvement. Treatment for BS overlaps considerably with therapies for other autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis and the vasculitides. Pharmacological agents utilized for treatment of BS include corticosteroids, colchicine, azathioprine, and tumour necrosis factor (TNF).? inhibitors, among others. In this article, we review the salient clinical studies for each drug class along with important side effects as well as drug toxicity monitoring. Management of the patient with BS is complex and oftentimes requires a multidisciplinary approach. We discuss strategies to assess and stratify patients based on clinical manifestations and disease severity. A summary of drug toxicities as they relate to the aforementioned pharmacological agents, as well as guidelines regarding vaccinations in this patient population, are offered. Finally, we conclude with treatment strategies for the common manifestations of BS along with a discussion of the management of thrombotic disease in these patients. PMID:23153327

Dalvi, Sam R; Yildirim, Resit; Yazici, Yusuf

2012-12-01

405

pain syndrome: a randomised controlled trial  

Microsoft Academic Search

Complex regional pain syndrome type I (CRPSI) involves cortical abnormalities similar to those observed in phantom pain and after stroke. In those groups. treatment is aimed at activation of cortical networks that subserve the affected limb, for example mirror therapy. However, mirror therapy is not effective for chronic CRPSI, possibly because movement of the limb evokes intolerable pain. It was

G. L. Moseley

406

Radionuclide Therapy of Bone Metastases  

Microsoft Academic Search

Summary The skeleton is a potential metastatic target of many malignant tumors. Up to 85% of prostate and breast cancer patients may develop bone metastases causing severe pain syndromes in many of them. In patients suffering from multilocular, mainly osteoblastic lesions and pain syndrome, radionuclide therapy is recommended for pain palliation. Low-energy beta-emitting radionuclides (153samarium-ethylenediaminetetramethylenephosphonate (EDTMP) and 89strontium) deliver high

Manfred Fischer; Willm U. Kampen

2012-01-01

407

[Nerve compression syndromes of the upper extremity].  

PubMed

The nerve compression syndromes of the upper extremity, the common carpal and cubital tunnel syndromes are described, but also the less common entrapments such as thoracic outlet syndrome,suprascapular-, interosseus-posterior- and anterior entrapments and the Loge-de-Guyon and Wartenberg syndromes are mentioned. Besides history, symptoms and physical examination electrodiagnostic and imaging are necessary for establishing the correct diagnosis. The conservative treatment is less successful than the operative therapy. Surgical procedures and techniques are discussed as well as complications. The prognosis is in most cases good--provided that indication and technique were correct. PMID:20936778

Assmus, H; Martini, A-K

2010-09-01

408

Marine peptides for preventing metabolic syndrome.  

PubMed

Recently, there has been a growing in alternative prevents and therapies of marine organisms for metabolic syndrome. Marine-derived bioactive peptides attracted extensive interest due to their numerous health beneficial effects including antioxidant, antihypertensive, anti-diabetes, anti-obesity. The bioactive peptides may provide novel therapeutic applications for the prevention of metabolic syndrome. The facts summarized here may provide improvement of metabolic syndrome by peptides from marine organisms. However, further insightful researches on the prevention of metabolic syndrome by marine peptides in in vivo and clinical studies are needed to firmly establish their therapeutic potency. PMID:23721312

Ko, Seok-Chun; Jeon, You-Jin

2013-05-01

409

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 patients 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 disease. Given that the disease affects mostly younger patients, new therapeutic strategies are necessary in order to avoid potentially irreversible joint and bone lesions. PMID:22670011

Henriques, Celia Coelho; Sousa, Mnica; Panarra, Antnio; Riso, Nuno

2011-01-01

410

Late onset imatinib-induced Stevens-Johnson syndrome.  

PubMed

Imatinib, a BCR-ABL tyrosine kinase inhibitor, is known to cause mild to moderate cutaneous reactions in up to approximately 20% of patients. It rarely causes severe reactions, such as Stevens-Johnson syndrome, which can be life threatening. Typically, these rashes occur within two months of initiating therapy. We report a case of Stevens-Johnson syndrome induced by imatinib after several years of therapy, but later successfully was treated with nilotinib therapy. PMID:24399835

Bois, Evan; Holle, Lisa M; Farooq, Umar

2014-12-01

411

Colonic smooth muscle cells and colonic motility patterns as a target for irritable bowel syndrome therapy: mechanisms of action of otilonium bromide  

PubMed Central

Otilonium bromide (OB) is a spasmolytic compound of the family of quaternary ammonium derivatives and has been successfully used in the treatment of patients with irritable bowel syndrome (IBS) due to its specific pharmacodynamic effects on motility patterns in the human colon and the contractility of colonic smooth muscle cells. This article examines how. OB inhibits the main patterns of human sigmoid motility in vitro, which are spontaneous rhythmic phasic contractions, smooth muscle tone, contractions induced by stimulation of excitatory motor neurons and contractions induced by direct effect of excitatory neurotransmitters. It does this mainly by blocking calcium influx through L-type calcium channels and interfering with mobilization of cellular calcium required for smooth muscle contraction, thereby limiting excessive intestinal contractility and abdominal cramping. OB also inhibits T-type calcium channels and muscarinic responses. Finally, OB inhibits tachykinin receptors on smooth muscle and primary afferent neurons which may have the joint effect of reducing motility and abdominal pain. All these mechanisms mediate the therapeutic effects of OB in patients with IBS and might be useful in patients with other spastic colonic motility disorders such as diverticular disease. PMID:25057296

Rychter, Jakub; Espn, Francisco; Gallego, Diana; Vergara, Patri; Jimnez, Marcel

2014-01-01

412

Down's syndrome.  

PubMed

The sequencing of chromosome 21 and the use of models of Down's syndrome in mice have allowed us to relate genes and sets of genes to the neuropathogenesis of this syndrome, and to better understand its phenotype. Research in prenatal screening and diagnosis aims to find methods to identify fetuses with Down's syndrome, and reduce or eliminate the need for amniocentesis. Other areas of active research and clinical interest include the association of Down's syndrome with coeliac disease and Alzheimer's disease, and improved median age of death. Medical management of the syndrome requires an organised approach of assessment, monitoring, prevention, and vigilance. Improvements in quality of life of individuals with Down's syndrome have resulted from improvements in medical care, identification and treatment of psychiatric disorders (such as depression, disruptive behaviour disorders, and autism), and early educational interventions with support in typical educational settings. Approaches and outcomes differ throughout the world. PMID:12699967

Roizen, Nancy J; Patterson, David

2003-04-12

413

Aicardi syndrome.  

PubMed

Aicardi syndrome is a rare neurodevelopmental disease characterised by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral callosum, chorioretinopathy and electroencephalogram abnormality. We present a case of Aicardi syndrome with callosal hypogenesis in a 4.5-month-old baby who presented with infantile spasms. Ophthalmoscopy revealed chorioretinal lacunae. The clinical and magnetic resonance imaging features were diagnostic of Aicardi syndrome. PMID:22815034

Singh, Paramdeep; Goraya, Jatinder Singh; Saggar, Kavita; Ahluwalia, Archana

2012-07-01

414

Establishing evidence for practice in medical nutrition therapy: a case study of the impact of a high amylose resistant starch diet on clinical indicators of the insulin resistant syndrome  

Microsoft Academic Search

In the context where current strategies employed by health professionals are not able to slow the increasing rates of obesity, diabetes mellitus and insulin resistance, collectively known as the insulin resistance syndrome (metabolic syndrome, syndrome X), evidence-based practice is central to implementing appropriate and targeted guidelines to the individual. The central hypothesis argued here is that for evidence based practice

Vanessa Brenninger

2005-01-01

415

Thoracic Outlet Syndromes.  

PubMed

The clinical presentation of thoracic outlet syndrome depends on which anatomic structure is compressed in the area of the thoracic outlet (eg, the axillary-subclavian artery, vein, brachial plexus, or the sympathetic nerves). The clinical syndrome may be isolated to one or a mixture of these compressed anatomic structures. Although there are multiple compressive forces, the first rib is the common denominator, and extirpation of this structure is the "gold" standard for therapy. The various syndromes are discussed in their clinical order of frequency. For severe nerve compression, whether upper or lower brachial plexus, the best surgical procedure is the transaxillary first rib resection, anterior scalenectomy, and neurovascular decompression. Axillary-subclavian vein occlusion (ie, Paget-Schroetter syndrome) is best treated with early diagnosis, intravenous thrombolysis of the clot, transaxillary first rib resection anterior scalenectomy, and resection of the costoclavicular ligament. Arterial compression, occlusion, or aneurysm may occur with or without peripheral emboli and require a supraclavicular approach with arterial ligation, endarterectomy, or bypass grafts from the supraclavicular subclavian to the infraclavicular brachial artery. Compression of the sympathetic nerves produces vasospasm, Raynaud's phenomenon, or Raynaud's disease, requiring dorsal sympathectomy as well as first rib extirpation occasionally. This may be performed through the transaxillary, supraclavicular posterior, or thoracoscopic approach. Recurrent thoracic outlet syndrome may involve any or all of the above anatomic syndromes. The surgical approach is best from the posterior, high thoracoplasty, muscle-splitting incision, performing neurolysis of the C7, C8, and T1 nerve roots and the brachial plexus as well as release of any vascular constriction. PMID:12686013

Urschel, Harold C.; Patel, Amit

2003-04-01

416

[Hereditary syndromes of neuroendocrine tumours].  

PubMed

Diffuse localised neuroendocrinal cells represent the largest population of endocrinally active cells and can degenerate to malignant neuroendocrine tumours (NET). In this review the most important hereditary syndromes that predispose for endocrine and neuroendocrine tumours are presented and discussed. NET occur mainly as sporadic tumours. Current investigations on the pathogenesis of sporadic neuroendocrine tumours have revealed a close relationship between hereditary and sporadic neuroendocrine tumours. In the course of hereditary syndromes, such as multiple endocrine neoplasia, endocrine and neuroendocrine tumours as well as non-endocrine neoplasias can occur. In order to recognise these syndromes in good time a knowledge of the predisposing syndromes and their cardinal symptoms is essential. In this way not only individualised diagnosis and therapy can be planned but also an appropriate early management of first degree relatives can be initiated. PMID:24327486

Kaemmerer, D; Posorski, N; Hommann, M; Baum, R P; Hrsch, D

2014-08-01

417

[Kounis syndrome].  

PubMed

Kounis syndrome was described in 1991 by Kounis and Zavras as the coincidental occurrence of acute coronary syndromes with allergic reactions (anaphylactic or anaphylactoid). Today, allergic angina and allergic myocardial infarction are referred to as Kounis syndrome, and the latter has been reported in association with a variety of drugs, insect stings, food, environmental exposures and medical conditions, among other factors. The incidence is not known, as most of the available information comes from case reports or small case series. In this article, the clinical aspects, diagnosis, pathogenesis, related conditions and therapeutic management of the syndrome are discussed. PMID:22154226

Rico Cepeda, P; Palencia Herrejn, E; Rodrguez Aguirregabiria, M M

2012-01-01

418

[Successful treatment of the backward-bending attack due to generalized spasm in stiff-person syndrome with intravenous immune globulin therapy].  

PubMed

We report a 63-year-old man with stiff-person syndrome, who dramatically responded to the treatment with high dose intravenous immunoglobulin (IVIG). He developed stiffness of the right leg and low back five years ago. He was treated with oral diazepam 6 mg/day and showed a marked improvement. He had been maintained on the same dose since then. In 2000, he began to have episodic generalized spasm and painful spasm of his left leg as well as persistent stiffness of his legs and low back. Findings on the physical examination were normal except for a prominent hyperlordosis with co-contracture of the lumbar paraspinal and abdominal muscles. Neurologic examination revealed stiffness in the lower limbs, more marked on the left side, and lower truncal muscles. The left leg had painful spasm, which was provoked by tactile stimuli. There was severe generalized spasm which made him suddenly bend backward. These backward-bending attacks were provoked spontaneously or reflexively by sudden tactile stimuli. He was unable to arise from a chair or stand without assistance. His deep tendon reflexes on both legs were brisk and Babinski sign was negative. He had no diabetes mellitus and thyroid function was normal. Antibodies against glutamic acid decarboxylase(GAD), antinuclear antibody, thyroid peroxidase autoantibody and antithyroglobulin autoantibody in the serum were present. His painful spasm was disappeared and muscle stiffness was moderately improved by treatment with oral diazepam and clonazepam, but backward-bending attacks due to generalized spasms were not controlled. He received IVIG. Three days after the administration of IVIG, these attacks disappeared completely. Subsequently muscle stiffness improved. One week after, he was able to walk without assistance. IVIG may be useful for treatment of generalized spasm, which had no response to treatment with diazepam or clonazepam. PMID:12684995

Wada, Yuko; Suenaga, Toshihiko; Hashimoto, Shuji

2003-02-01

419

Laparoscopic ovarian electrocautery versus gonadotropin therapy in infertile women with clomiphene citrate-resistant polycystic ovary syndrome: A systematic review and meta-analysis  

PubMed Central

Background: Some trials have compared laparoscopic ovarian drilling (LOD) with gonadotropins but, because of variations in study design and small sample size, the results are inconsistent and definitive conclusions about the relative efficacy of LOD and gonadotropins cannot be extracted from the individual studies. Objective: To evaluate the relative efficacy of LOD and gonadotropins for infertile women with clomiphene citrate- resistant poly cystic ovary syndrome (PCOS). Materials and Methods: A complete electronic literature search in databases including EMBASE, MEDLINE, Cochrane Library and Google scholar for some specific keywords was accomplished. We contained randomized clinical trials comparing outcomes between LOD, without medical ovulation induction, and gonadotropins. Results: Six trials, covering 499 women, reported on the primary outcome of pregnancy rate. There was no evidence of a difference in pregnancy rate when LOD compared with gonadotropins (OR: 0.534; 95% CI: 0.242-1.176, p=0.119, 6 trials, 499 women, I2=73.201%). There was evidence of significantly fewer live births following LOD compared with gonadotropin (OR: 0.446; 95% CI: 0.269-0.74, p=0.02, 3 trials, 318 women, I2=3.353%). The rate of multiple pregnancies was significantly lower in the LOD arm compared to the gonadotropins arm (OR: 0.127; 95% CI: 0.028-0.579, p=0.008, 3 trials, 307 women, I2=0%). Conclusion: Our result revealed that there was no evidence of a significant difference in rates of clinical pregnancy and miscarriage in women with clomiphene citrate-resistant PCOS undergoing LOD compared to the gonadotropin arm. The decrease in multiple pregnancies rate in women undergoing LOD makes this option attractive. The increase in live birth rate in the gonadotropin group may be because of the higher rate of multiple pregnancies in these women. However, more focus on the long-term effects of LOD on ovarian function is suggested. PMID:25408702

Moazami Goudarzi, Zahra; Fallahzadeh, Hossein; Aflatoonian, Abbas; Mirzaei, Masoud

2014-01-01

420

[Chronic relapsing compartment syndrome].  

PubMed

Chronic relapsing exertional compartment syndrome is not only found in young athletic subjects. Non-athletic patients can also suffer from exercise dependent pain in the lower limb. The syndrome is defined by increased tissue pressure in between a closed osteo-fibrous space resulting in disturbed microcirculation and finally irreversible neuroischemic damage. Pain increases during running and disappears gradually at rest. The diagnosis is based on the typical symptoms and so far on the pre-, 1 and 5 min post exercise intramuscular pressure measurement. We first describe sonographic criteria induced by increased compartment pressure, especially for the most frequent anterior compartment syndrome with compression of the deep veins, increased movement of arterial wall and finally enddiastolic closure of the anterior tibial artery. Duplex ultrasound also provides pathogenetic factors like popliteal and anterior entrapment, stenosis or hypoplasia of the anterior tibial artery or thrombosis of the deep muscle veins. Many patients are reporting a pain history over years, resulting in complete reduction of their sporting activity. The knowledge about pathogenesis, symptoms, diagnosis and therapy should help shorten the negative impact of the syndrome on their quality of life. PMID:19347380

Stiegler, H; Brandl, R; Krettek, C

2009-04-01