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1

Therapy of Marfan syndrome.  

PubMed

Marfan syndrome is a common inherited disorder of connective tissue caused by deficiency of the matrix protein fibrillin-1. Effective surgical therapy for the most life-threatening manifestation, aortic root aneurysm, has led to a nearly normal lifespan for affected individuals who are appropriately recognized and treated. Traditional medical therapies, such as beta-adrenergic receptor blockade, are used to slow pathologic aortic growth and decrease the risk of aortic dissection by decreasing hemodynamic stress. New insights regarding the pathogenesis of Marfan syndrome have developed from investigation of murine models of this disorder. Fibrillin-1 deficiency is associated with excess signaling by transforming growth factor beta (TGFbeta). TGFbeta antagonists have shown great success in improving or preventing several manifestations of Marfan syndrome in these mice, including aortic aneurysm. These results highlight the potential for development of targeted therapies based on discovery of disease genes and interrogation of pathogenesis in murine models. PMID:17845137

Judge, Daniel P; Dietz, Harry C

2008-01-01

2

Therapy-related preleukemic syndrome  

Microsoft Academic Search

Eight patients developed the preleukemic syndrome after having been exposed to cytotoxic drugs for other primary diseases. All eight subsequently developed acute nonlymphocytic leukemia (ANLL). Survival following the onset of the preleukemic syndrome ranged from 5-34 months with a median of 11 months. Once overt leukemia developed median survival was three months. No patient responded to conventional antileukemic therapy. A

Robert L. Anderson; Kathryn Richert-Boe; R. Ellen Magenis; Robert D. Koler

1981-01-01

3

Current therapy of myelodysplastic syndromes.  

PubMed

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 2years. 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

2013-07-27

4

Therapy of the burnout syndrome  

PubMed Central

Background The prevalence, diagnostics and therapy of the burnout syndrome are increasingly discussed in the public. The unclear definition and diagnostics of the burnout syndrome are scientifically criticized. There are several therapies with unclear evidence for the treatment of burnout in existence. Objectives The health technology assessment (HTA) report deals with the question of usage and efficacy of different burnout therapies. Methods For the years 2006 to 2011, a systematic literature research was done in 31 electronic databases (e.g. EMBASE, MEDLINE, PsycINFO). Important inclusion criteria are burnout, therapeutic intervention and treatment outcome. Results 17 studies meet the inclusion criteria and are regarded for the HTA report. The studies are very heterogeneous (sample size, type of intervention, measuring method, level of evidence). Due to their study design (e.g. four reviews, eight randomized controlled trials) the studies have a comparable high evidence: three times 1A, five times 1B, one time 2A, two times 2B and six times 4. 13 of the 17 studies are dealing with the efficacy of psychotherapy and psychosocial interventions for the reduction of burnout (partly in combination with other techniques). Cognitive behaviour therapy leads to the improvement of emotional exhaustion in the majority of the studies. The evidence is inconsistent for the efficacy of stress management and music therapy. Two studies regarding the efficacy of Qigong therapy do not deliver a distinct result. One study proves the efficacy of roots of Rhodiola rosea (evidence level 1B). Physical therapy is only in one study separately examined and does not show a better result than standard therapy. Discussion Despite the number of studies with high evidence the results for the efficacy of burnout therapies are preliminary and do have only limited reach. The authors of the studies complain about the low number of skilled studies for the therapy of burnout. Furthermore, they point to the insufficient evaluation of the therapy studies and the need for further research. Some authors report the effects of considerable natural recovering. Numerous limitations affect the quality of the results. Intervention contents and duration, study design and study size are very diverse and do not permit direct comparison. Most of the samples are small by size with low statistical power, long-term follow-ups are missing. Comorbidities and parallel utilized therapies are insufficient documented or controlled. Most of the studies use the Maslach Burnout Inventory (MBI) as diagnostic or outcome-tool, but with different cut-off-points. It should be noticed that the validity of the MBI as diagnostic tool is not proved. Ethical, juridical and social determining factors are not covered or discussed in the studies. Conclusion The efficacy of therapies for the treatment of the burnout syndrome is insufficient investigated. Only for cognitive behavioural therapy (CBT) exists an adequate number of studies which prove its efficacy. Big long-term experimental studies are missing which compare the efficacy of the single therapies and evaluate their evidence. The natural recovering without any therapy needs further research. Additionally, it has to be examined to what extent therapies and their possible effects are thwarted by the conditions of the working place and the working conditions.

Korczak, Dieter; Wastian, Monika; Schneider, Michael

2012-01-01

5

Directed Therapy for Exfoliation Syndrome  

PubMed Central

Exfoliation syndrome (XFS) is an age-related disorder of the extracellular matrix that leads the production of abnormal fibrillar material that leads to elevated intraocular pressure and a relatively severe glaucoma. Exfoliation material is deposited in numerous ocular tissues and extraocular organs. XFS is associated with ocular ischemia, cerebrovascular disease, neurodegenerative disease and cardiovascular disease. Current modalities of treatment include intraocular pressure lowering with topical antihypertensives, laser trabeculoplasty and filtration surgery. The disease paradigm for XFS should be expanded to include directed therapy designed specifically to target the underlying disease process. Potential targets include preventing the formation or promoting the depolymerization of exfoliation material. Novel therapies targeting trabecular meshwork may prove particularly useful in the care of exfoliative glaucoma. The systemic and ocular associations of XFS underscore the need for a comprehensive search for neuroprotective agents in its treatment.

Angelilli, Allison; Ritch, Robert

2009-01-01

6

Complementary and Alternative Therapies for Down Syndrome  

ERIC Educational Resources Information Center

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

Roizen, Nancy J.

2005-01-01

7

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

8

[Chronic and therapy refractory Fregoli syndrome].  

PubMed

We present a 46-year old lady suffering from chronic paranoid schizophrenia accompanied by a chronic Fregoli's syndrome. The patient feels persecuted by a male person and identifies this persecutor in different persons of different shape, different age and different sex. The symptoms are very refractory to treatment despite various neuroleptic therapies. Historical, psychodynamic and neuropsychological aspects of this delusional syndrome are discussed. PMID:10705602

Zänker, S

2000-01-01

9

Psychologic therapies for irritable bowel syndrome  

Microsoft Academic Search

Opinion statement  The treatment of patients with irritable bowel syndrome (IBS) is a difficult task, as the results from therapy with pharmacologic\\u000a agents have been disappointing. Psychologic treatments, in particular cognitive behavior therapy, hypnotherapy, and dynamic\\u000a psychotherapy have all shown to be effective in the treatment of patients with IBS. Underpinning all these treatments is a\\u000a clear understanding of a biopsychosocial

Philip Boyce

2001-01-01

10

Gene therapy: Sjögren’s syndrome  

Microsoft Academic Search

\\u000a Sjögrens syndrome (SS) is characterized by inflammation and dysfunction of the secretory organs. In the majority of patients\\u000a the salivary and lachrymal glands are predominantly affected, although systemic symptoms are common. The pathogenesis of the\\u000a disease is not well understood and to date there is no universally effective therapy available. The development of gene therapy\\u000a and in particular local gene

Nienke Roescher; Paul Peter Tak; John A. Chiorini

11

Immunomodulatory Therapy for Myelodysplastic Syndromes  

Microsoft Academic Search

Thalidomide and lenalidomide belong to the proprietary group of immunomodulatory drugs (IMiDs) that display broad biologic\\u000a and pharmacologic properties. Encouraging results of clinical studies that evaluated the efficacy of thalidomide in patients\\u000a with myelodysplastic syndromes (MDSs) led to the investigation of its structural analogue, lenalidomide, in patients with\\u000a lower-risk MDS. The cumulative results of studies that tested lenalidomide in patients

Lubomir Sokol; Alan F. List

2007-01-01

12

Targeted Therapy in Myelodysplastic Syndrome  

Microsoft Academic Search

Managing patients with myelodysplastic syndrome (MDS) is a highly challenging endeavor. MDS appears to arise from intrinsic\\u000a or acquired genetic defects in stem cells that confer a proliferative advantage to the malignant clone over normal stem cells.\\u000a Recurrent chromosomal abnormalities are present in 40% to 70% of patients at diagnosis and in 95% of patients with treatment-related\\u000a MDS. Until now,

Alfonso Quintás-Cardama; Hagop Kantarjian; Guillermo Garcia-Manero; Jorge Cortes

13

Simplified therapy for Asherman's syndrome  

Microsoft Academic Search

Objective: To evaluate a technique that converts a blind hysteroscopic procedure to a “septum” division.Design: Open noncomparative clinical study.Setting: Tertiary care center.Patient(s): Six women with Asherman's syndrome; five with complete and one with incomplete obliteration of the uterine cavity.Intervention(s): The patients underwent recreation of the uterine cavity by the hysteroscopic-laparoscopic technique described to establish the correct dissection plane.Main Outcome Measure(s):

Peter F McComb; Brenda L Wagner

1997-01-01

14

Emerging Pharmacologic Therapies for Irritable Bowel Syndrome  

Microsoft Academic Search

New therapies are being developed for irritable bowel syndrome (IBS). These advances are based on understanding pathophysiology\\u000a or the development of medications with greater selectivity in classes of agents with known efficacy. Prucalopride, the newest\\u000a European Medicines Agency-approved 5-hydroxytryptamine receptor 4 (5-HT4) agonist, is effective in the treatment of chronic constipation with improved cardiovascular safety relative to older 5-HT4 drugs;

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

2010-01-01

15

Stem Cell Therapies Benefit Alport Syndrome  

PubMed Central

Patients with Alport syndrome progressively lose renal function as a result of defective type IV collagen in their glomerular basement membrane. In mice lacking the ?3 chain of type IV collagen (Col4A3 knockout mice), a model for Alport syndrome, transplantation of wild-type bone marrow repairs the renal disease. It is unknown whether cell-based therapies that do not require transplantation have similar potential. Here, infusion of wild-type bone marrow-derived cells into unconditioned, nonirradiated Col4A3 knockout mice during the late stage of disease significantly improved renal histology and function. Furthermore, transfusion of unfractionated wild-type blood into unconditioned, nonirradiated Col4A3 knockout mice improved the renal phenotype and significantly improved survival. Injection of mouse and human embryonic stem cells into Col4A3 knockout mice produced similar results. Regardless of treatment modality, the improvement in the architecture of the glomerular basement membrane is associated with de novo expression of the ?3(IV) chain. These data provide further support for testing cell-based therapies for Alport syndrome.

LeBleu, Valerie; Sugimoto, Hikaru; Mundel, Thomas M.; Gerami-Naini, Behzad; Finan, Elizabeth; Miller, Caroline A.; Gattone, Vincent H.; Lu, Lingge; Shield, Charles F.; Folkman, Judah

2009-01-01

16

Statin therapy improves brachial artery endothelial function in nephrotic syndrome  

Microsoft Academic Search

Statin therapy improves brachial artery endothelial function in nephrotic syndrome.Background:Patients with nephrotic syndrome have impaired endothelial function probably related to dyslipidemia. This study evaluated the effects of statin therapy on dyslipidemia and endothelial function in patients with nephrotic syndrome.Methods:A sequential, open-label study of the effects of statins on endothelial dysfunction in 10 nephrotic patients treated with an angiotensin-converting enzyme (ACE)

Gursharan K Dogra; Gerald F Watts; Susan Herrmann; Mark A B Thomas; Ashley B Irish

2002-01-01

17

New approaches in Sjögren's syndrome therapy.  

PubMed

At present, there is no treatment capable of modifying the evolution of Sjögren's syndrome (SS) and the therapeutic approach is based mainly on symptomatic replacement or stimulation of glandular secretions, while extraglandular involvement requires an organ-specific therapy. New therapeutic approaches for sicca manifestations include oral muscarinic agonists (e.g., pilocarpine and cevimeline) for dry mouth, as well as topical agents (e.g., cyclosporine A and diquafosol) for dry eye. Turning to biological agents, recent studies have demonstrated the lack of efficacy of anti-tumor necrosis factor (TNF) agents in primary SS. By contrast, a promising treatment is rituximab (anti-CD20). Advances in our knowledge of etiopathogenic mechanisms will allow the development of future treatments for SS, including localized gene transfer and agents antagonizing B cell-activating factor from the TNF family and CD22-positive cells. PMID:20477108

Ramos-Casals, Manuel; Brito-Zerón, Pilar

2007-03-01

18

[Antibacterial therapy in patients with diabetic foot syndrome].  

PubMed

Character of microflora in patients with infection complications of diabetic foot syndrome was analyzed. The efficacy of modern antibacterial preparations for empirical and specific therapy was studied up. PMID:21510362

Palamarchuk, V I; Siriachenko, V H; Markov, K V; Muz', M I; Shuliarenko, O V; Shuliarenko, L V

2011-01-01

19

Intravenous Immunoglobulin Therapy for Stevens-Johnson Syndrome  

Microsoft Academic Search

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.

ALLAN S. BRETT; DYANNA PHILLIPS; ANNETTE W. LYNN

2001-01-01

20

Antiplatelet therapy in acute coronary syndromes: ticagrelor.  

PubMed

Ticagrelor is a direct-acting, oral, reversibly binding P2Y(12) receptor antagonist. As a cyclopentyltriazolopyrimidine, ticagrelor represents a new chemical class of agents that do not require metabolic activation and have consistent ability to inhibit platelet aggregation. The phase III PLATO study evaluated ticagrelor compared with clopidogrel in 18,624 patients with acute coronary syndromes, and demonstrated a significant reduction in the risk of death from vascular causes/myocardial infarction (MI)/stroke with ticagrelor (9.8 vs. 11.7% with clopidogrel; HR: 0.84; 95% CI: 0.77-0.92; p < 0.001) without a significant increase in PLATO-defined major bleeding (11.6 vs. 11.2%, respectively; p = 0.43). MI and death from vascular causes were separately significantly reduced, and death from any cause and stent thrombosis reductions achieved nominal statistical significance. Ticagrelor showed benefit over clopidogrel in almost all patient subgroups, including patients who had received clopidogrel at randomization, patients with both planned invasive or noninvasive treatment; patients with ST elevation myocardial infarction (STEMI) referred for primary percutaneous coronary intervention, patients with non-STEMI, and patients who underwent bypass surgery. Hence, the PLATO population reflected specifically those patients who would ordinarily receive thienopyridine-based antiplatelet therapy in a clinical setting. PMID:22906903

Husted, Steen

2012-08-09

21

Emerging pharmacologic therapies for irritable bowel syndrome.  

PubMed

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

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

2010-10-01

22

Rituximab therapy in nephrotic syndrome: implications for patients' management.  

PubMed

Rituximab offers an alternative to current immunosuppressive therapies for difficult-to-treat nephrotic syndrome. The best outcomes are seen in patients with steroid-dependent nephrotic syndrome who have failed to respond to multiple therapies. By contrast, the benefits of rituximab therapy are limited in patients with steroid-resistant nephrotic syndrome, particularly those with focal segmental glomerulosclerosis (FSGS). Therapy with plasma exchange and one or two doses of rituximab has shown success in patients with recurrent FSGS. Young patients and those with normal serum albumin at recurrence of nephrotic syndrome are most likely to respond to rituximab therapy. A substantial proportion of rituximab-treated patients with idiopathic membranous nephropathy show complete or partial remission of proteinuria, and reduced levels of phospholipase A(2) receptor autoantibodies, which are implicated in the pathogenesis of this disorder. Successful rituximab therapy induces prolonged remission and enables discontinuation of other medications without substantially increasing the risk of infections and other serious adverse events. However, the available evidence of efficacy of rituximab therapy is derived chiefly from small case series and requires confirmation in prospective, randomized, controlled studies that define the indications for use and predictors of response to this therapy. PMID:23338210

Sinha, Aditi; Bagga, Arvind

2013-01-22

23

[Gorlin syndrome: photodynamic therapy, as a useful adjunct to surgery].  

PubMed

Gorlin syndrome, also called nevoid basal cell carcinoma syndrome, is well known by dermatologists. Since its onset, 10 years ago, photodynamic therapy has found new applications and is now currently used to cure single or multiple basal cell carcinomas, with good results and without residual scars. We recall some of the basic principles of this technique, as well as its indications in Gorlin syndrome, which we illustrate with one case. Plastic surgeons must consider this relatively new technique, developed by dermatologists, as a useful adjunct to surgery in the management of Gorlin syndrome. PMID:21907481

Huguier, V; Wierzbicka-Hainaut, E; Fray, J; Guillet, G; Dagrégorio, G

2011-09-09

24

[Successful electroconvulsive therapy of Cotard syndrome with bitemporal hypoperfusion].  

PubMed

A case study is presented to illustrate a rare condition described by Cotard as "délire de négation". The central symptom is a nihilistic delusion with denial of one's own existence of oneself and that of the external world. In the present case, the syndrome became manifest as an escalation of a recurrent depressive disorder late in life. After initial resistance to therapy, the syndrome was successfully treated with electroconvulsive therapy. For the first time, we report the regional cerebral blood flow measured by 99mTc-HMPAO-SPECT before and after therapy. Before treatment, significant bitemporal hypoperfusion relative to the cerebellum was found, which was no longer demonstrable on remission. PMID:9005351

Lohmann, T; Nishimura, K; Sabri, O; Klosterkötter, J

1996-05-01

25

Withdrawal syndrome following cessation of antihypertensive drug therapy.  

PubMed

In this study, a review of the available information concerning abrupt withdrawal of antihypertensive drug therapy is presented. Abrupt withdrawal of these drugs can produce a syndrome of sympathetic overactivity that includes nervousness, tachycardia, headache, agitation and nausea 36-72 h after cessation of the drug. A withdrawal syndrome may occur after discontinuation of almost all types of antihypertensive drugs, but mostly occurs with clonidine, beta-blockers, methyldopa and guanabenz. Less commonly can produce a rapid increase of the blood pressure to pre-treatment levels or above, or both and/or myocardial ischaemia. Although the exact incidence of the syndrome is not known, it appears to be rare, at least in patients receiving standard doses of the above antihypertensive drugs. The best treatment is prevention. In this study regarding the withdrawal syndrome that follows cessation of antihypertensive drugs therapy, a reference to the abrupt discontinuation of the main categories of antihypertensive drugs is also attempted. PMID:15857353

Karachalios, G N; Charalabopoulos, A; Papalimneou, V; Kiortsis, D; Dimicco, P; Kostoula, O K; Charalabopoulos, K

2005-05-01

26

Sick sinus syndrome as a complication of mediastinal radiation therapy  

SciTech Connect

A 33-year-old man who had received mediastinal radiation therapy for Hodgkin's disease 12 years earlier developed a symptomatic sick sinus syndrome requiring the implantation of a permanent pacemaker. The sick sinus syndrome and a finding of an occult constrictive pericarditis were considered to be due to the previous mediastinal irradiation. A ventricular pacemaker was chosen because mediastinal radiotherapy also increases the risk of developing atrioventricular conduction defects.

Pohjola-Sintonen, S.; Toetterman, K.J.K.; Kupari, M. (Helsinki Univ. Central Hospital (Finland))

1990-06-01

27

Combination Therapy for Patients with Myelodysplastic Syndromes  

Cancer.gov

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

28

Transfert d'énergie linéique et radiosensibilité 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 réponse des cellules tumorales humaines à l'irradiation in vitro par des particules de TEL élevé dépend de plusieurs facteurs. Pour les particules chargées, la radiosensibilité augmente avec le TEL jusqu'à des valeurs de TEL voisines de 200 KeV/?m, puis diminue pour des valeurs de TEL supérieures. En clinique, l'augmentation du TEL moyen de la particule chargée en profondeur est responsable d'une efficacité biologique relative (EBR) croissante. La probabilité de mortalité de la cellule par une particule traversant le noyau est proche de 1 pour les particules de TEL très élevé comme l'Ar de 7 MeV par nucléon (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 élevée pour les petites doses, la relation EBR/dose étant plus marquée dans les cellules ayant un rapport ?/beta plus faible après exposition aux photons, selon le modèle linéaire-quadratique. Les particules moins efficaces que les photons en terme de mortalité cellulaire moyenne induisent des dégâts individuels importants, révélés par la production de micronoyaux (MN) multiples. Concernant le lien entre l'EBR et la radio-sensibilité intrinsèque, les données récentes utilisant à la fois la technique des colonies et la méthode des MN, montrent que l'EBR est d'autant plus élevée que les cellules sont radiorésistantes aux photons.

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

1998-04-01

29

Dance Therapy with Physical Therapy for Children with Down Syndrome.  

ERIC Educational Resources Information Center

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

Dupont, Blanche Burt; Schulmann, Diana

30

Pharmacologic Therapy for the Irritable Bowel Syndrome  

Microsoft Academic Search

The management of the irritable bowel syndrome (IBS) remains unsatisfactory. For abdominal pain, antispasmodics are, at best, of only modest efficacy. Tricyclic antidepressants in low dose are useful (with the number needed to treat being three), but side effects and patient concerns regarding use of a centrally acting agent for depression remain limitations. Selective serotonin reuptake inhibitors are of uncertain

Nicholas J. Talley

2003-01-01

31

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

32

Peripherally acting therapies for the treatment of irritable bowel syndrome.  

PubMed

Gut-acting therapies are common therapies for irritable bowel syndrome (IBS). Most of these peripheral acting agents are primarily targeted at individual symptoms. The evidence supporting the use of these agents in IBS is largely anecdotal. Serotonergic agents and the chloride channel activator lubiprostone have shown efficacy in treating symptoms of IBS. The clinical evidence supporting the use of these agents is based on data from high-quality clinical trials. The use of serotonergic agents for IBS in the United States is limited to the 5-hydroxytryptamine-3 antagonist alosetron in the treatment of women with severe IBS with diarrhea refractory to traditional therapy. PMID:21333906

Saad, Richard J

2011-03-01

33

Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood.  

PubMed

We examined seizure, cognitive, and motor outcomes in patients with Rasmussen syndrome or Rasmussen encephalitis (RS), after recent initiation of immunomodulatory therapies. Among 53 patients with a diagnosis of RS referred from all over Japan, 49 patients (male 22, female 27) with symptoms and findings characteristic of RS were evaluated. Regular intravenous immunoglobulin (IVIg) therapy was administered at a dose of 100mg/kg/day, etc. Regular steroid pulse therapy was conducted with methylprednisolone at a dose of 30mg/kg/day (children) or 1000mg/day (adults) for 3days. Tacrolimus was given at an initial dose of 0.1mg/kg/day (children). Mean onset age was 8.7±10.5years. Seizure-free rate was 71% after treatment by functional hemispherectomy (FH), and response rate for seizures was 81% by regular steroid pulse therapy, 42% by tacrolimus therapy, and 23% by regular IVIg therapy. Rate of patients with IQ higher than 80 (R80) was 50% by regular steroid pulse therapy, 43% by regular IVIg therapy, 29% by tacrolimus therapy, and 0% by FH. R80 after regular steroid pulse therapy was 100% in patients without MRI lesions, and 37% in those with advanced MRI lesions. Improvement of motor function (paresis) was observed only by immunomodulatory therapy. Motor function was aggravated in 100% of patients treated by FH, 62% by regular IVIg, and 10% by regular steroid pulse therapy. We suggest a new treatment strategy for RS using early immunomodulatory therapy: initiation of regular steroid pulse therapy after early diagnosis indicated by biomarkers, then switching to tacrolimus therapy after several months. PMID:23433490

Takahashi, Yukitoshi; Yamazaki, Etsuko; Mine, Jun; Kubota, Yuko; Imai, Katsumi; Mogami, Yuki; Baba, Koichi; Matsuda, Kazumi; Oguni, Hirokazu; Sugai, Kenji; Ohtsuka, Yoko; Fujiwara, Tateki; Inoue, Yushi

2013-02-19

34

Therapies for steroid-resistant nephrotic syndrome  

Microsoft Academic Search

Between 10 and 20% of children with primary nephrotic syndrome are steroid-resistant (SRNS). From earlier studies in children\\u000a with SRNS, we know that cyclosporin (with or without alternate-day prednisone) and cyclophosphamide (with pulse intravenous\\u000a corticosteroids) result in comparable complete or partial remission rates of about 60%. An evaluation of the relative effectiveness\\u000a of cyclophosphamide and cyclosporin has not been possible

Elisabeth M. Hodson; Jonathan C. Craig

2008-01-01

35

Effects of Qi-therapy on premenstrual syndrome.  

PubMed

This study investigated the effects of Qi-therapy (nine sessions over two menstrual cycles) on pain and other symptoms in women with premenstrual syndrome (PMS). Forty-six women who were attending college were randomly allocated to receive Qi-therapy (Qi-therapy group: n = 23) or placed on a waiting list as controls (n = 22: 1 dropped out). Qi-therapy had a significant effect on pain and water retention. In addition, there were significant short-term effects on pain, mental depression, and anxiety. These results suggested that Qi-therapy might be useful as a nursing intervention for women who suffer from PMS to maintain or restore a balance of Qi. PMID:15527198

Jang, Hye-Sook; Lee, Myeong Soo; Kim, Myung-Ja; Chong, Elizabeth S

2004-08-01

36

Cognitive behaviour therapy for adolescents with chronic fatigue syndrome: randomised controlled trial  

Microsoft Academic Search

Objective To evaluate the efficacy of cognitive behaviour therapy for adolescents aged 10-17 years with chronic fatigue syndrome. Design Randomised controlled trial. Setting Department of child psychology. Participants 69 consecutively referred patients with chronic fatigue syndrome; 36 were randomly assigned to immediate cognitive behaviour therapy and 35 to the waiting list for therapy. Intervention 10 sessions of therapy over five

Maja Stulemeijer; Lieke W A M de Jong; Theo J W Fiselier; Sigrid W B Hoogveld; Gijs Bleijenberg

2004-01-01

37

Cognitive behaviour therapy for adolescents with chronic fatigue syndrome: randomised controlled trial  

Microsoft Academic Search

OBJECTIVE: To evaluate the efficacy of cognitive behaviour therapy for adolescents aged 10-17 years with chronic fatigue syndrome. DESIGN: Randomised controlled trial. SETTING: Department of child psychology. PARTICIPANTS: 71 consecutively referred patients with chronic fatigue syndrome; 36 were randomly assigned to immediate cognitive behaviour therapy and 35 to the waiting list for therapy. INTERVENTION: 10 sessions of therapy over five

M. Stulemeijer; L. W. A. M. de Jong; T. J. W. Fiselier; S. W. B. Hoogveld; G. Bleijenberg

2005-01-01

38

THERAPIES IN AICARDI-GOUTIÈRES SYNDROME.  

PubMed

Aicardi-Goutières syndrome (AGS) is a genetically determined disorder, most particularly affecting the brain and the skin, characterized by the inappropriate induction of a type I interferon-mediated immune response. In most, but not all, cases the condition is severe, with a high associated morbidity and mortality. A number of important recent advances have helped to elucidate the biology of the AGS-related proteins, thus providing considerable insight into disease pathology. Here, we outline the clinical phenotype of AGS, paying particular attention to factors relevant to therapeutic intervention. We then discuss the pathogenesis of AGS from a molecular and cell biology perspective. Finally, we suggest possible treatment strategies in light of these emerging insights. PMID:23607857

Crow, Yanick J; Vanderver, Adeline; Orcesi, Simona; Kuijpers, Taco W; Rice, Gillian I

2013-04-01

39

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

40

Electropalatographic therapy for children and young people with Down's syndrome.  

PubMed

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 syndrome, aged 10:1 to 18:9, received 24 individualized therapy sessions using EPG. Simultaneous acoustic and EPG recordings were made pre- and post-intervention during 10 repetitions of a word list containing lingua-palatal consonants. Participants also completed the DEAP phonology sub-test at both time points. Post-treatment, all participants showed qualitative and quantifiable differences in EPG patterns and improvements in DEAP percentage consonants correct. EPG assessment and therapy appears a positive approach for identifying and improving articulatory patterns in children with DS. PMID:20001308

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

2009-12-01

41

[New aspects in diagnosis and therapy of Turner's syndrome].  

PubMed

In the last 35 years we noticed 91 cases of Turner's syndrome. To the obligatory symptoms of the syndrome belong primary amenorrhoea, sexual infantilism, elevated values only of FSH and very reduced levels of estrogens, chromosomal aberrations in karyotype, most often 45, X and presence of streak gonads. The facultative symptoms of the syndrome belong short stature (94.5% of patients), presence of congenital defects (short webbed neck in 44.4%) and sterility (98.9%). Therapy above 15 years excepting to cause the menstrual cycles have to development the second sexual symptoms by giving enough large doses of oestrogens and gestagens. The best effects we obtain nearly in 100% in the development of breasts, in slowly growth of sexual hair and in the growth of the stature which does not cross the height of 150 cm and only in six patients attained in average 152.5 cm. PMID:8001844

Baron, J; Warenik-Szymankiewicz, A; Miedzianowski, J; Baron, J J

1994-03-01

42

Classic and recent etiologies of Cushing's syndrome: diagnosis and therapy.  

PubMed

Endogenous Cushing's syndrome can result from excess adrenocorticotropic hormone (ACTH; corticotropin) production by a pituitary adenoma (Cushing's disease) or by ectopic tumors secreting ACTH or corticotro- pin-releasing hormone (CRH). ACTH-independent Cushing's syndrome is caused by adrenocortical tumors or hyperplasias. Initial diagnosis is performed using 24-hour urinary free cortisol, low-dose dexamethasone tests, salivary cortisol, or night-time plasma cortisol values. A dexamethasone CRH test can discriminate between Cushing's syndrome and pseudo-Cushing's syndrome. If ACTH is elevated, combinations of high-dose dexamethasone tests, CRH/desmopressin tests, and pituitary magnetic resonance imaging can indicate a pituitary source. Discrimination from an ectopic ACTH tumor often requires inferior petrosal sinus sampling to confirm the ACTH source. If ACTH is low, adrenal computed tomography scan will identify the adrenal lesion(s) implicated. Some cortisol-producing adrenal tumors or, more frequently, bilateral macronodular hyperplasias, are under the control of aberrant membrane hormone receptors, or altered activity of eutopic receptors. The initial therapy of choice for patients with Cushing's disease is the selective transsphenoidal removal of the corticotroph adenoma; this induces remission in approximately 80% of patients, but long-term relapse occurs in up to 30% of these cases. The choice of second-line therapy remains controversial. Repeat surgery can be successful when residual tumor is detectable on magnetic resonance imaging, but carries a high risk of hypopituitarism. Bilateral adrenalectomy may be a better choice in patients without visible residual tumors, particularly in women desiring fertility. Radiotherapy combined with ketoconazole or radiosurgery was recently found effective, but longer-term evaluation of hypopituitarism and brain function is required. Current studies do not support the systematic use of prophylactic radiotherapy after bilateral adrenalectomy to decrease the risk of Nelson's syndrome; however, as soon as the residual tumor progresses, surgery and radiotherapy should be initiated. Various drugs which inhibit steroid synthesis (ketoconazole, metyrapone, aminoglutethimide, mitotane) are often effective for rapidly controlling hypercortisolism either in preparation for surgery, after unsuccessful removal of the etiologic tumor, or while awaiting the full effect of radiotherapy or more definitive therapy. Surgery is usually the treatment of choice for removal of cortisol-secreting adrenal tumors or ectopic ACTH/CRH-secreting tumors. The identification of aberrant adrenal receptors has recently allowed normalization of cortisol secretion by specific ligand receptor antagonists in limited cases of Cushing's syndrome secondary to bilateral macronodular adrenal hyperplasia. The long-term follow-up of patients treated for Cushing's syndrome should include the adequate replacement of glucocorticoids and other hormones, treatment of osteoporosis, and detection of long-term relapse of Cushing's syndrome. PMID:15765624

Beauregard, Catherine; Dickstein, Gabriel; Lacroix, André

2002-01-01

43

Bromocriptine augmentation therapy in a patient with Cotard's syndrome  

Microsoft Academic Search

A 72-year-old female with bipolar I disorder developed Cotard's syndrome, i.e., various delusions of negation accompanied by severe depressive symptoms. She responded neither to the combination of antipsychotic drug and antidepressant nor to the lithium augmentation therapy. However, the delusions and depressive symptoms improved dramatically after the addition of bromocriptine 2.5–5 mg\\/day to the combination of clomipramine and lithium. This

Shuko Kondo; Hiroshi Hayashi; Takuya Eguchi; Takanobu Oyama; Tadashi Wada; Koichi Otani

2003-01-01

44

Cyclophosphamide Therapy in the Nephrotic Syndrome in Childhood  

Microsoft Academic Search

Forty-six children with the nephrotic syndrome whose renal biopsy specimens showed minimal changes and whose response to corticosteroid therapy was unsatisfactory were treated with cyclophosphamide. Three patients were completely steroid-resistant from the outset and the remainder were steroid-dependent. In several patients steroids controlled the condition less effectively with time. Most patients showed signs of steroid toxicity, and growth retardation was

M. W. Moncrieff; R. H. R. White; C. S. Ogg; J. S. Cameron

1969-01-01

45

Nutritional therapy of irritable bowel syndrome.  

PubMed

Nutritional factors relative to IBS include diagnostic and therapeutic considerations. Etiologically, foods do not cause IBS. A small percentage of patients with childhood allergic diatheses, usually in association with atopic dermatitis and asthma, may be intolerant to one or more of wheat, corn, dairy products, coffee, tea, or citrus fruits. Diagnostically, many patients labeled as IBS subjects are in fact intolerant to the ingestion of lactose-containing foods, sorbitol, fructose, or combinations of fructose and sorbitol. A precise dietary history will characterize this group. Taken in its broadest context, IBS involves the entire hollow tract inclusive of esophagus, stomach, small bowel, and colon. The symptomatic presentation relative to the hollow organ involved allows the selection of dietary manipulations that may help to reduce symptoms. Gastroesophageal reflux, a consequence of low LES pressure in some IBS patients, may be treated with the elimination of fatty foods, alcohol, chocolate, and peppermint. Delayed gastric emptying may be helped by the elimination of fatty foods and reduction of soluble fiber. Aberrant small bowel motor function may be ameliorated by reduction of lactose, sorbitol, and fructose and the addition of soluble fiber. Gas syndromes may be improved by reduced intake of beans, cabbage, lentils, legumes, apples, grapes, and raisins. Colonic motor dysfunction may be overcome by the gradual addition of combinations of soluble and insoluble fiber-containing foods and supplements. The selective use of activated charcoal and simethicone may be helpful. PMID:2553606

Friedman, G

1989-09-01

46

Epigenetics, fragile X syndrome and transcriptional therapy.  

PubMed

Epigenetics refers to the study of heritable changes in gene expression that occur without a change in DNA sequence. Epigenetic mechanisms therefore include all transcriptional controls that determine how genes are expressed during development and differentiation, but also in individual cells responding to environmental stimuli. The purpose of this review is to examine the basic principles of epigenetic mechanisms and their contribution to human disorders with a particular focus on fragile X syndrome (FXS), the most common monogenic form of developmental cognitive impairment. FXS represents a prototype of the so-called repeat expansion disorders due to "dynamic" mutations, namely the expansion (known as "full mutation") of a CGG repeat in the 5'UTR of the FMR1 gene. This genetic anomaly is accompanied by epigenetic modifications (mainly DNA methylation and histone deacetylation), resulting in the inactivation of the FMR1 gene. The presence of an intact FMR1 coding sequence allowed pharmacological reactivation of gene transcription, particularly through the use of the DNA demethylating agent 5'-aza-2'-deoxycytydine and/or inhibitors of histone deacetylases. These treatments suggested that DNA methylation is dominant over histone acetylation in silencing the FMR1 gene. The importance of DNA methylation in repressing FMR1 transcription is confirmed by the existence of rare unaffected males carrying unmethylated full mutations. Finally, we address the potential use of epigenetic approaches to targeted treatment of other genetic conditions. © 2013 Wiley Periodicals, Inc. PMID:24123753

Tabolacci, Elisabetta; Chiurazzi, Pietro

2013-10-03

47

Immunosuppressive therapy for myelodysplastic syndrome: efficacy of methylprednisolone pulse therapy with or without cyclosporin A  

Microsoft Academic Search

We investigated whether immunosuppressive therapy using methylprednisolone (mPSL) with or without cyclosporin A (CsA) could benefit patients with myelodysplastic syndrome (MDS). Eligibility criteria for this study were a clinical diagnosis of MDS with less than 5% blast in peripheral blood, less than 10% blast in bone marrow and advanced cytopenia. Among 73 patients with MDS, 18 eligible and consecutive patients

Toshiki Yamada; Hisashi Tsurumi; Senji Kasahara; Takeshi Hara; Michio Sawada; Hisataka Moriwaki

2003-01-01

48

Monozygotic Twins With Turner Syndrome Develop Slipped Capital Femoral Epiphysis on Growth Hormone Therapy  

Microsoft Academic Search

Monozygotic twins with Turner syndrome have rarely been reported. An increased incidence of slipped capital femoral epiphysis has been associated with growth hormone therapy, as well as with Turner syndrome, but has never been described in twins with Turner syndrome. We report the first case of monozygotic twins with Turner syndrome with a 46,Xi(Xq) karyotype, both of whom developed slipped

Zeina M. Nabhan; Erica A. Eugster

49

Anti-PL7 antisynthetase syndrome under interferon therapy.  

PubMed

A 62-year-old woman with a past history of chronic hepatitis C virus infection presented an atypical predominantly painful polymyositis following a flu-like syndrome, persisting despite the withdrawal of PEG-interferon alpha-2b therapy. Clinical assessment, then immunological, electrophysiological and iconographic investigations including myositis antibodies, electromyography, pulmonary functions assessment and thoracic CT-scan found respectively "mechanic hands", arthralgia, presence of antisynthetase anti-PL7 antibody, typical myographic features of hip and shoulder girdles involvement, pulmonary fibrosis and restrictive syndrome features. A deltoid muscle biopsy revealed an unclassified myositis getting closer to anti-J0-1 and connective tissue disease associated myositis featuring: some components of polymyositis, dermatomyositis, and the following peculiar pathological aspects: noncaseous granulomatous infiltrate, endomysial microangiopathy and vascular and sarcolemic deposition of complement membrane attack complex. The diagnosis of interferon induced anti-PL7 antisynthetase syndrome with microangiopathic and granulomatous overlap myositis was retained. A treatment associating intravenous immunoglobulin and moderate tapered oral corticosteroids allowed a complete long-term resolution of myositis and a clear improvement of pulmonary involvement. This case points out the wide range of interferon alpha-associated disorders and call for a precise and detailed immunopathological analysis of myositis, instead of the usual vague classification as idiopathic polymyositis or dermatomyositis about antisynthetase syndrome. PMID:20851661

Aouba, Achille; Georgin-Lavialle, Sophie; Terrier, Benjamin; Guillevin, Loïc; Authier, François-Jérome

2010-09-18

50

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.

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

2012-01-01

51

Use of maggot debridement therapy for tropical diabetic hand syndrome.  

PubMed

Diabetic patients with infectious hand ulceration, known as tropical diabetic hand syndrome (TDHS), are generally less well recognised than those with foot ulcers. Maggot debridement therapy (MDT) is usually used for the treatment of diabetic foot ulcers and, in these wounds, the remarkable wound cleansing properties can be of considerable value. However, it is less commonly used in TDHS. Here we present a case of TDHS in a 51-year-old man with type II diabetes, hypertension and dilated cardiomyopathy, in which conventional therapy had minimal effect, but significant wound debridement was achieved with MDT. This suggests that MDT may be a cost-effective alternative to conventional treatments for the debridement of TDHS. PMID:23702721

Jiang, K C; Luo, N; Chen, Y C; Wang, A P

2013-05-01

52

Vitamin Therapy and Children with Down's Syndrome: A Review of Research.  

ERIC Educational Resources Information Center

|Following a brief outline of vitamin therapy's effects on schizophrenia and learning disabilities, research is reviewed on vitamin therapy for children with Down's Syndrome, concluding with a discussion of critical responses to research endorsing vitamin therapy. It is concluded that vitamin therapy contributes nothing to the development of…

Pruess, James B.; And Others

1989-01-01

53

Children with Down Syndrome Improved in Motor Functioning and Muscle Tone Following Massage Therapy  

ERIC Educational Resources Information Center

|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…

Hernandez-Reif, Maria; Field, Tiffany; Largie, Shay; Mora, Dana; Bornstein, Joan; Waldman, Ronnie

2006-01-01

54

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 Möller; RH Söderberg; HT Eriksson; GK Kurlberg

55

Acute vanishing bile duct syndrome after ibuprofen therapy in a child  

Microsoft Academic Search

We report the case of a 10 year-old girl who had Stevens-Johnson syndrome and cholestasis after ibuprofen therapy. Liver histology was compatible with vanishing bile duct syndrome. She received ursodeoxycholic acid, and liver tests normalized within 7 months. This report confirms that ibuprofen may induce acute vanishing bile duct syndrome.

M. Taghian; T. A. Tran; S. Bresson-Hadni; A. Menget; S. Felix; E. Jacquemin

2004-01-01

56

Modelisation microstructurale en fatigue/fluage a froid des alliages de titane quasi alpha par le modele des automates cellulaires  

NASA Astrophysics Data System (ADS)

Les proprietes d'emploi des alliages de titane sont extremement dependantes a certains aspects des microstructures developpees lors de leur elaboration. Ces microstructures peuvent etre fortement heterogenes du point de vue de leur orientation cristallographique et de leur repartition spatiale. Leurs influences sur le comportement du materiau et son endommagement precoce sont des questions qui sont actuellement soulevees. Dans le present projet de doctorat on chercher a repondre a cette question mais aussi de presenter des solutions tangibles quant a l'utilisation securitaire de ces alliages. Un nouveau modele appele automate cellulaire a ete developpe pour simuler le comportement mecanique des alliages de titane en fatigue-fluage a froid. Ces modeles ont permet de mieux comprendre la correlation entre la microstructure et le comportement mecanique du materiau et surtout une analyse detaillee du comportement local du materiau. Mots-cles: Automate cellulaire, fatigue/fluage, alliage de titane, inclusion d'Eshelby, modelisation

Boutana, Mohammed Nabil

57

[Irritable bowel syndrome: diet and complementary medicine therapies?].  

PubMed

Irritable bowel syndrome (IBS) is a frequent and invalidating functional bowel disorder with entangled mechanisms. Its therapeutic approach is therefore complex. Classical therapies, prescribed alone or in combination in light of the predominant symptom, consist of antispasmodics, fibers, laxatives, antidiarrheals, and psychotropic agents. Other emerging pharmacological therapies, such as prokinetics, prosecretory or serotoninergic agents, bile acid modulators and antibiotics have been recently studied in clinical trials. Dietary measures can include reduction of short-chain poorly absorbed carbohydrates (FODMAPs) and gluten restriction. Assessment of food allergy can be proposed in a subgroup of IBS patients. Complementary and alternative medicine therapies, that are generally low cost and safe, appear to be appreciated by patients. Probiotics have demonstrated action on the gut microbiote modulation, and may be helpful in a subset of patients. Peppermint oil has an established visceral analgesic effect. Hypnotherapy represents an original, global and effective approach. Finally, education, reassurance and listening to the patient, leading to a solid therapeutic relationship, represents an essential backdrop of remedy or diet effectiveness. PMID:23091952

Gerkens, A

2012-09-01

58

Therapeutic approach in acute coronary syndrome focusing on oral therapy.  

PubMed

In the light of some new information based on clinical evidence, current therapeutic approach to patients with acute coronary syndrome especially focusing on oral therapy is being considered. The initial stage of treatment does not differ greatly among patients with unstable angina pectoris (UA), non-ST-elevation myocardial infarction (NSTEMI), or ST-elevation myocardial infarction (STEMI). It is necessary to simultaneously resolve a series of problems within the first twenty minutes upon admission, i.e. risk assessment, selection of treatment strategy (conservative, invasive), relief of ischemic pain, determination of hemodynamic status and elimination of any undesired complications (hypertension, tachycardia, heart failure), and administration of antithrombotic therapy. Patients suffering from STEMI require reperfusion treatment, and the method of choice is primary percutaneous coronary intervention (PCI) where available. Fibrinolytic reperfusion therapy is limited exclusively to STEMI within the first three hours from the onset of pain. Unlike this, in patients suffering from UA/NSTEMI it is necessary to make risk assessment in the early stage of disease, and thus select the patients that will certainly benefit from invasive treatment through PCI. For pain relief, the patient should be immediately administered nitroglycerin along with oxygen. Beta-blockers that are reasonably used in the initial stage of treatment during the first 24 hours, if not contraindicated, are still underused. Clopidogrel becomes an obligatory drug not only in patients having undergone PCI, but also in those treated conservatively following fibrinolysis. PMID:20635590

Heitzler, Vjeran Nikoli?; Pavlov, Marin

2010-03-01

59

Iatrogenic diabetes mellitus during ACTH therapy in an infant with West syndrome  

Microsoft Academic Search

West syndrome is a rare epileptic disease of infancy, typified by an association of characteristic spasms, hypsarrhythmia\\u000a on electroencephalography and severe psychomotor retardation or deterioration. Adrenocorticotropic hormone (ACTH) is the current\\u000a first-line therapy for West syndrome despite the fact that ACTH therapy is associated with various adverse effects. We describe\\u000a a rare case of iatrogenic diabetes mellitus during ACTH therapy

Valeria Calcaterra; Andrea Bottazzi; Chrissoula Tzialla; Stefano D’Arrigo; Daniela Larizza

60

Emerging therapies to treat frailty syndrome in the elderly.  

PubMed

Frailty syndrome (FS) has become increasingly recognized as a major predictor of co-morbidities and mortality in older individuals. Interventions with the potential to benefit frail elders include nutritional supplementation (vitamins D, carotenoids, creatine, dehydroepiandrosterone (DHEA), and beta-hydroxy-beta-methylbutyrate) and exercise modalities (tai chi and cobblestone walking). While these have not been explicitly tested for their impact on FS, vitamin D supplementation appears to offer significant promise in enhancing long-term health of the elderly. Exercise modalities such as tai chi and cobblestone walking, because of probable low risk and ease of participation, may also confer benefit. Additional studies are needed to investigate interventions that directly prevent, delay, and/or ameliorate frailty. Successful therapies may well involve multi-component approaches utilizing a combination of medication, nutritional supplementation, and exercise. PMID:18072820

Cherniack, E Paul; Florez, Hermes J; Troen, Bruce R

2007-09-01

61

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

PubMed

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

2013-08-01

62

Epoxyeicosatrienoic acids--novel mechanism and pharmacological therapy of chronic renocardiac syndrome.  

PubMed

Cardiorenal syndromes were defined and classified recently, but the mechanism of chronic renocardiac syndrome remains disputed. Theories about chronic renocardiac syndrome cannot offer a convincing explanation for it. As a result, the current therapies of chronic renocardiac syndrome do not contribute to a satisfied prognosis. Epoxyeicosatrienoic acids, the products of arachidonic acid metabolized by cytochrome P450 enzymes, play an important role in the maintenance of renal hemodynamics, and regulation of renal, cardiac, and vascular function with antihypertensive and anti-inflammatory properties. It is well documented that down-regulation of epoxyeicosatrienoic acids might be involved in alterations in various pathophysiological states, including hypertension, uremia and hepatorenal syndrome. Likewise, epoxyeicosatrienoic acids were reduced in heart failure and renal dysfunction. This leads to the proposed hypothesis that epoxyeicosatrienoic acids down-regulation may be the novel mechanism of chronic renocardiac syndrome. These findings suggest that manipulation of epoxyeicosatrienoic acid levels could be a novel pharmacological therapy strategy for chronic renocardiac syndrome. PMID:21333454

Huang, Hui; Chen, Jie; Lin, Tianxin; Wang, Tong; Tang, Yi; Dong, Yanbing; Wang, Jingfeng

2011-02-18

63

Short Communication The effects of repeated thermal therapy for two patients with chronic fatigue syndrome  

Microsoft Academic Search

Objective: This paper describes the successful treatment of two patients with chronic fatigue syndrome (CFS) using repeated thermal therapy. Methods: Two patients with CFS underwent treatment with prednisolone (PSL), with no satisfactory effect. They were subjected to thermal therapy that consisted of a far-infrared ray dry sauna at 60 8C and postsauna warming. The therapy was performed once a day,

Akinori Masuda; Takashi Kihara; Tsuyoshi Fukudome; Takuro Shinsato; Shinichi Minagoe; Chuwa Teib

64

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

ERIC Educational Resources Information Center

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

Stringer, William W.; Sattler, Fred R.

2001-01-01

65

Statins as first-line therapy for acute coronary syndrome?  

PubMed Central

It has repeatedly been shown that statins decrease morbidity and mortality in patients with atherosclerosis, thus supporting their use for the primary and secondary prevention of ischemic heart disease. Different pathological pathways that are triggered in the setting of acute coronary syndrome (ACS), such as endothelial dysfunction, activation of inflammatory and coagulation cascades, and thrombus formation, are known to be inhibited by statins, thereby justifying the use of these agents in patients with ACS. Several recent prospective controlled clinical trials have demonstrated the safety and, in some cases, the efficacy of statins when administered early after ACS. An increasing number of publications have reported, however, that statins may confer a beneficial effect not only in early secondary prevention, but also in the direct treatment of ACS (ie, when statins are administered as first-line treatment in clinically unstable patients). This therapeutic option is supported by the following: numerous experimental studies demonstrating a protective effect of statins under conditions of acute ischemia; analysis of different registries and trials, which has demonstrated a more favourable prognosis for statin-treated patients at the time of acute myocardial ischemia; and small clinical trials reporting a lower periprocedural infarction rate during coronary intervention or lower levels of several prognostic biomarkers, in addition to a lower incidence of cardiovascular events associated with statin therapy. Nevertheless, confirmation of this hypothesis in large prospective controlled clinical trials will be necessary before the implementation of statins as first-line therapy in unstable patients with ACS, irrespective of blood cholesterol levels.

Ostadal, Petr

2012-01-01

66

New Receptor Targets for Medical Therapy in Irritable Bowel Syndrome  

PubMed Central

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

Camilleri, Michael

2010-01-01

67

Photodynamic therapy of subfoveal choroidal neovascularization with verteporfin in the ocular histoplasmosis syndrome  

Microsoft Academic Search

ObjectiveTo evaluate the safety and effect on visual acuity of photodynamic therapy with verteporfin (Visudyne, Novartis AG) in patients with subfoveal choroidal neovascularization (CNV) secondary to the ocular histoplasmosis syndrome (OHS).

David A Saperstein; Philip J Rosenfeld; Neil M Bressler; Robert H Rosa; Michel Sickenberg; Paul Sternberg; Thomas M Aaberg; Troy A Reaves

2002-01-01

68

Targeted therapies for diarrhea-predominant irritable bowel syndrome.  

PubMed

Irritable bowel syndrome (IBS) causes gastrointestinal symptoms such as abdominal pain, bloating, and bowel pattern abnormalities, which compromise patients' daily functioning. Common therapies address one or two IBS symptoms, while others offer wider symptom control, presumably by targeting pathophysiologic mechanisms of IBS. The aim of this targeted literature review was to capture clinical trial reports of agents receiving the highest recommendation (Grade 1) for treatment of IBS from the 2009 American College of Gastroenterology IBS Task Force, with an emphasis on diarrhea-predominant IBS. Literature searches in PubMed captured articles detailing randomized placebo-controlled trials in IBS/diarrhea-predominant IBS for agents receiving Grade I (strong) 2009 American College of Gastroenterology IBS Task Force recommendations: tricyclic antidepressants, nonabsorbable antibiotics, and the 5-HT(3) receptor antagonist alosetron. Studies specific for constipation-predominant IBS were excluded. Tricyclic antidepressants appear to improve global IBS symptoms but have variable effects on abdominal pain and uncertain tolerability; effects on stool consistency, frequency, and urgency were not adequately assessed. Nonabsorbable antibiotics show positive effects on global symptoms, abdominal pain, bloating, and stool consistency but may be most efficacious in patients with altered intestinal microbiota. Alosetron improves global symptoms and abdominal pain and normalizes bowel irregularities, including stool frequency, consistency, and fecal urgency. Both the nonabsorbable antibiotic rifaximin and the 5-HT(3) receptor antagonist alosetron improve quality of life. Targeted therapies provide more complete relief of IBS symptoms than conventional agents. Familiarization with the quantity and quality of evidence of effectiveness can facilitate more individualized treatment plans for patients with this heterogeneous disorder. PMID:22754282

Olden, Kevin W

2012-05-25

69

[SMART syndrome: case report of a rare complication after cerebral radiation therapy].  

PubMed

The authors report a 71-year-old woman case who developed, 7 years after a cerebral radiation therapy for a parietooccipital glioblastoma, a stroke-like migraine attacks after radiotherapy syndrome (SMART syndrome), a rare complication characterized by reversible neurologic deficits with migraine described after cerebral irradiation. Transient gyriform reversible enhancement is found on MRI during crises. This case report allows discussing the clinical, iconographic presentation and the clinical outcome of this syndrome at the light of the literature publication. PMID:22819470

Truntzer, P; Monjour, A; Gaultier, C; Ahle, G; Guillerme, F; Boutenbat, G; Stilhart, B; Salze, P; Atlani, D

2012-07-20

70

Association of statin therapy with outcomes of acute coronary syndromes: The GRACE study  

Microsoft Academic Search

BACKGROUND: Statins administered early in patients with acute coronary syndromes may lead to modest reductions in recurrent ischemic events. OBJECTIVE: To examine the association between previous and early in-hospital statin therapy and the presentation and outcomes of an acute coronary syndrome. DESIGN: Cohort study. SETTING: 94 hospitals in 14 countries participating in the Global Registry of Acute Coronary Events (GRACE).

Frederick A. Spencer; Jeanna Allegrone; Robert J. Goldberg; Joel M. Gore; Keith A. A. Fox; Christopher B. Granger; Rajendra H. Mehta; David Brieger

2004-01-01

71

Down's syndrome-associated Single Minded 2 gene as a pancreatic cancer drug therapy target  

Microsoft Academic Search

We report here a pancreatic cancer drug therapy utility of a gene involved in Down's syndrome. Single Minded 2 gene (SIM2) from Down's Syndrome Critical Region was expressed in pancreatic cancer-derived cell lines as well as in tumor tissues, but not in the normal pancreas. A related member of the SIM family, SIM1, did not show similar specificity. Inhibition by

Maurice Phil DeYoung; Matthew Tress; Ramaswamy Narayanan

2003-01-01

72

Animal Models of Alport Syndrome: Advancing the Prospects for Effective Human Gene Therapy  

Microsoft Academic Search

Several animal models for Alport syndrome have been described. These are available for studies on the pathogenetic mechanisms of the disease, as well as for the development of new technologies for gene therapy in this progressive hereditary kidney disease. This review summarizes current knowledge on the molecular basis of Alport syndrome, and on the animal models which all remarkably well

Pirkko Heikkilä; Karl Tryggvason; Paul Thorner

2000-01-01

73

Cerebrolysin therapy in Rett syndrome: clinical and EEG mapping study  

Microsoft Academic Search

Based on the suggestion that nerve growth factor plays a core role in the brain maturation process, which is altered in Rett syndrome, we investigate the influence of Cerebrolysin — the brain-derived peptidergic drug — on motor and higher cortical functions in Rett syndrome girls. The open pilot study was performed on nine Rett syndrome girls (aged from 2 years

Natalia Gorbachevskaya; Vera Bashina; Vitali Gratchev; Andrey Iznak

2001-01-01

74

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

75

Periradicular therapy in lumbar radicular syndromes: methodology and results.  

PubMed

Periradicular therapy (PRT) has become popular in the treatment of lumbar radicular complaints, both primary, due to disc herniations (Group 1), and postoperative, following disc surgery (Group 2). However, hitherto reported data on 'periradicular injections' are more of a technical nature. The present study was designed to evaluate the therapeutic success of CT- versus fluoroscope-guided periradicular injections of local anaesthetics and corticoids, and to investigate the impact of imaging procedures on the results. We report on 80 patients who underwent CT-guided (n = 40) or fluoroscope-guided (n = 40) PRT for lumbar radicular irritation since April 1993. Forty patients had primary nerve root irritation due to disc protrusion, and 40 suffered from postoperative epidural fibrosis. Follow-up consisted in a clinical examination and an interview one day after each injection as well as 1 and 6 months later. Assessment was based on a score taking into account the clinical findings, the patient's complaints, and the patient's evaluation. Six months following treatment there was a significant reduction of ischialgia in both Groups 1 and 2 (p < 0.001). However, the results were significantly poorer in Group 2 (postoperative). Among the patients not operated on, those who underwent CT-guided injections had a significantly better outcome (p < 0.001). PRT had no significant influence on low back pain or pseudoradicular syndromes. The improvement of sensory disturbances achieved by both CT- and fluoroscope-guided injections in patients not previously submitted to surgery is statistically significant (p < 0.05). As compared to Group 1 (no surgery), the positive effects in patients of Group 2 were of significantly shorter duration (p < 0.05). Motor deficits were not influenced by the treatment. Long-term follow-up shows that there is no positive effect in those patients in whom the first two PRT attempts had failed. Thus, PRT represents a useful long-term therapeutic alternative for lumbar radicular syndromes, particularly when due to primary discogenic compression. CT-guided injection is superior to fluoroscope-assisted treatment for both its visualization and its longer-lasting effect. PMID:9309286

Lutze, M; Stendel, R; Vesper, J; Brock, M

1997-01-01

76

Salivary cortisol output before and after cognitive behavioural therapy for chronic fatigue syndrome  

Microsoft Academic Search

BackgroundThere is evidence that patients with chronic fatigue syndrome (CFS) have mild hypocortisolism. One theory about the aetiology of this hypocortisolism is that it occurs late in the course of CFS via factors such as inactivity, sleep disturbance, chronic stress and deconditioning. We aimed to determine whether therapy aimed at reversing these factors – cognitive behavioural therapy for CFS –

Amanda D. L. Roberts; Andrew S. Papadopoulos; Simon Wessely; Trudie Chalder; Anthony J. Cleare

2009-01-01

77

Intravenous Ulinastatin Therapy for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Pediatric Patients  

Microsoft Academic Search

Background: More effective therapy is needed for the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The clinical efficacy of intravenous ulinastatin therapy was investigated in 3 Japanese pediatric patients with SJS or TEN. Methods: Ulinastatin was given to 1 pediatric SJS patient and 2 pediatric TEN patients within 7 days (patient 1; SJS), 6 days (patient 2;

Yasuji Inamo; Takahiro Okubo; Mika Wada; Sachiko Fuchigami; Kouji Hashimoto; Tatsuo Fuchigami; Shigeru Takahashi; Shigemasa Sawada; Kensuke Harada

2002-01-01

78

[Antiviral therapy in a patient with chronic hepatitis C, metabolic syndrome and interferon-induced depression].  

PubMed

A case of metabolic syndrome is reported in which its elimination before the onset of antiviral treatment of chronic hepatitis A and drug therapy of subsequent depression promoted successful outcome with a stable virological response. Modern approaches are discussed to enhancing efficiency of antiviral therapy by active correction of factors responsible for poor results of the treatment and its undesirable consequences. PMID:21395034

Ba?zhanova, Zh Zh; Ignatova, T M; Kinkul'kina, M A

2010-01-01

79

A randomized clinical trial of manual therapy for cervico-brachial pain syndrome – a pilot study  

Microsoft Academic Search

Cervico-brachial pain syndrome is an upper quarter pain condition in which mechanosensitive neural tissue is considered a primary feature. A single-blind randomized controlled trial was conducted to determine the clinical effect of two manual therapy interventions. Thirty subjects (20 females and 10 males) were randomly allocated to one of three groups — one of two manual therapy intervention groups or

G. T. Allison; B. M. Nagy; T. Hall

2002-01-01

80

A Pilot Study Comparing Two Manual Therapy Interventions for Carpal Tunnel Syndrome  

Microsoft Academic Search

ObjectiveThe purpose of this study was to determine the clinical efficacy of manual therapy interventions for relieving the signs and symptoms of carpal tunnel syndrome (CTS) by comparing 2 forms of manual therapy techniques: Graston Instrument–assisted soft tissue mobilization (GISTM) and STM administered with the clinician hands.

Jeanmarie Burke; Dale J. Buchberger; M. Terry Carey-Loghmani; Paul E. Dougherty; Douglas S. Greco; J. Donald Dishman

2007-01-01

81

Salivary cortisol output before and after cognitive behavioural therapy for chronic fatigue syndrome  

Microsoft Academic Search

Background: There is evidence that patients with chronic fatigue syndrome (CFS) have mild hypocortisolism. One theory about the aetiology of this hypocortisolism is that it occurs late in the course of CFS via factors such as inactivity, sleep disturbance, chronic stress and deconditioning. We aimed to determine whether therapy aimed at reversing these factors - cognitive behavioural therapy for CFS

Amanda D. L. Roberts; Andrew S. Papadopoulos; Simon Wessely; Trudie Chalder; Anthony J. Cleare

2008-01-01

82

A comparison of hormone therapy, coping skills training, and relaxation for the relief of premenstrual syndrome  

Microsoft Academic Search

Approximately 10% of women are severely affected by premenstrual syndrome (PMS) during their reproductive years. Several biological theories of causation have been proposed and each has provoked treatment attempts through medication to little sustained effect. As many of the reported complaints are psychological, a new treatment approach was considered using cognitive-behavioral therapy. A preliminary study which combined cognitive-behavioral therapy with

Carol A. Morse; Lorraine Dennerstein; Elizabeth Farrell; Katrina Varnavides

1991-01-01

83

SMART Syndrome (Stroke-Like Migraine Attacks After Radiation Therapy) in Adult and Pediatric Patients.  

PubMed

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

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

2013-01-29

84

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

85

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

86

Immunonutritional effects during synbiotics therapy in pediatric patients with short bowel syndrome  

Microsoft Academic Search

The aim of this study was to evaluate the effects of synbiotic therapy in patients with short bowel syndrome (SBS). Four pediatric\\u000a patients with SBS, who were receiving synbiotics therapy including Bifidobacterium breve, Lactobacillus casei and galactooligosaccharides, were enrolled in this study. We evaluated changes in immunonutritional parameters before and\\u000a after receiving synbiotics therapy. Four normal, healthy, age-matched children were

Keiichi Uchida; Takuya Takahashi; Mikihiro Inoue; Masami Morotomi; Kohei Otake; Makoto Nakazawa; Yoshihide Tsukamoto; Chikao Miki; Masato Kusunoki

2007-01-01

87

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

Microsoft Academic Search

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 30min session of music therapy employing improvisational methods. Absent or reduced hand clasping\\/plucking, interactive

Tony Wigram; Margaret Lawrence

2005-01-01

88

Growth Hormone Therapy and Bone Mineral Density in Turner Syndrome  

Microsoft Academic Search

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

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

2010-01-01

89

Intradural intraneural hemorrhagic cyst resulting in progressive cauda equina syndrome after anticoagulation therapy: case report.  

PubMed

Study Design: Case report.Objective: To report a case of lumbar intraneural hemorrhagic cyst after anticoagulation therapy that caused progressive radiculopathy and cauda equina syndrome. The possible pathogenic mechanism, associated diseases, and treatment options are discussed.Summary of Background Data: Various pathologic processes can cause progressive cauda equina syndrome. However, there have been no reports of progressive cauda equina syndrome due to compression from an intraneural hemorrhagic cyst after anticoagulation therapy.Methods: A case of lumbar intradural intraneural hemorrhagic cyst with progressive cauda equina syndrome after anticoagulation therapy is presented.Results: A 42-year-old female patient complained atpresentation of progressive bilateral lower extremity radiating pain, numbness and urinary difficulty during the previous 2 months. Lumbar magnetic resonance imaging (MRI) revealed an L1 cystic lesion with marked mass effect on the surrounding nerve roots. Complete drainage and excision of the lesion was performed which resulted in excellent postoperative symptoms relief. Pathologic examination revealed no definite neoplastic process except some nerve fibers with hemosiderin stain along the cyst wall. Based on a combination of intraoperative findings and pathology, an intradural intraneural hemorrhagic cyst that developed after systemic anticocagulation therapy was diagnosed.Conclusion: This is the first report of an intradural intraneural hemorrhagic cyst causing progressive cauda equina syndrome due to anticoagulation therapy. Surgical excision of the cyst is the definite treatment of choice. PMID:24042718

Hsieh, Po-Chuan; Jung, Shih-Ming; Wu, Chieh-Tsai; Chen, Jyi-Feng; Lee, Shih-Tseng

2013-09-15

90

SMART syndrome: a late reversible complication after radiation therapy for brain tumours.  

PubMed

With intensified treatment leading to longer survival, complications of therapy for brain tumours are more frequently observed. Regarding radiation therapy, progressive and irreversible white matter disease with cognitive decline is most feared. We report on four patients with reversible clinical and radiological features occurring years after radiation for brain tumours, suggestive for the so called SMART syndrome (stroke-like migraine attacks after radiation therapy). All four patients (males, age 36-60 years) had been treated with focal brain radiation for a primary brain tumour or with whole-brain radiation therapy for brain metastases. Ranging from 2 to 10 years following radiation therapy patients presented with headache and focal neurological deficits, suggestive for tumour recurrence. Two patients also presented with focal seizures. MRI demonstrated typical cortical swelling and contrast enhancement, primarily in the parieto-occipital region. On follow-up both clinical and MRI features improved spontaneously. Three patients eventually proved to have tumour recurrence. The clinical and radiological picture of these patients is compatible with the SMART syndrome, a rare complication of radiation therapy which is probably under recognized in brain tumour patients. The pathophysiology of the SMART syndrome is poorly understood but bears similarities with the posterior reversible encephalopathy syndrome (PRES). These four cases underline that the SMART syndrome should be considered in patients formerly treated with radiation therapy for brain tumours, who present with new neurologic deficits. Before the diagnosis of SMART syndrome can be established other causes, such as local tumour recurrence, leptomeningeal disease or ischemic disease should be ruled out. PMID:21373901

Kerklaan, Joost P; Lycklama á Nijeholt, Geert J; Wiggenraad, Ruud G J; Berghuis, Bianca; Postma, Tjeerd J; Taphoorn, Martin J B

2011-03-04

91

Family Therapy of Brain Injury: Syndromes, Strategies, and Solutions.  

ERIC Educational Resources Information Center

|Describes effects that brain-injured patient may have on family system and kinds of reactions typically seen in family members. Describes strategies of family therapy in brain injury, including education, supportive counseling, and more intensive family therapy modalities. Considers goals and outcomes of family therapy with this group and offers…

Miller, Laurence

1993-01-01

92

Effects of various drug therapies on Kleine-Levin syndrome: a case report.  

PubMed

Kleine-Levin syndrome (KLS) is a rare sleep disorder, predominantly affecting adolescent males, which presents as recurrent episodes of hypersomnia, behavioral and cognitive disturbances, hyperphagia and sometimes hypersexuality (Lisk, "Kleine-Levin syndrome." Pract Neurol 2009;9:42-45). Modafinil has been reported to show an effect in shortening the duration of symptomatic periods, but does not affect the recurrence rate (Huang et al., "Kleine-Levin syndrome: current status." Med Clin N Am 2010;94:557-562). However, no single drug therapy has been consistently successful, despite various psychotropic agents, including lithium, anticonvulsants and antidepressants, having been systematically tried (Arnulf et al., "Kleine-Levin syndrome: a systematic study of 108 patients." Ann Neurol 2008;63:482-492). This study reports a male adolescent with KLS who received several courses of drug therapy, providing a chance to compare differential drug effects over time. PMID:22520717

Yao, Chi-Chun; Lin, Ying; Liu, Hui-Ching; Lee, Chau-Shoun

2012-04-18

93

Cognitive behaviour therapy for the chronic fatigue syndrome: a randomised controlled trial  

Microsoft Academic Search

AbstractObjective: To evaluate the acceptability and efficacy of adding cognitive behaviour therapy to the medical care of patients presenting with the chronic fatigue syndrome.Design: Randomised controlled trial with final assessment at 12 months.Setting: An infectious diseases outpatient clinic.Subjects: 60 consecutively referred patients meeting consensus criteria for the chronic fatigue syndrome.Interventions: Medical care comprised assessment, advice, and follow up in general

Michael Sharpe; Keith Hawton; Sue Simkin; Christina Surawy; Ann Hackmann; Ivana Klimes; Tim Peto; David Warrell; Valerie Seagroatt

1996-01-01

94

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

95

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

Microsoft Academic Search

BackgroundRett 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 FindingsWe generated

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

2009-01-01

96

Pediatric patients with multi-organ dysfunction syndrome receiving continuous renal replacement therapy  

Microsoft Academic Search

Pediatric patients with multi-organ dysfunction syndrome receiving continuous renal replacement therapy.BackgroundCritical illness leading to multi-organ dysfunction syndrome (MODS) and associated acute renal failure (ARF) is less common in children compared to adult patients. As a result, many issues plague the pediatric ARF outcome literature, including a relative lack of prospective study, a lack of modality stratification in subject populations and

Stuart L. Goldstein; Michael J. G. Somers; Michelle A. Baum; Jordan M. Symons; Patrick D. Brophy; DOUGLAS BLOWEY; Timothy E. Bunchman; CHERYL BAKER; THERESA MOTTES; NANCY MCAFEE; JONI BARNETT; GLORIA MORRISON; KRISTINE ROGERS; James D. Fortenberry

2005-01-01

97

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

Microsoft Academic Search

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

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

2009-01-01

98

Abdominal Compartment Syndrome: Risk Factors, Diagnosis, and Current Therapy  

PubMed Central

Abdominal compartment syndrome's manifestations are difficult to definitively detect on physical examination alone. Therefore, objective criteria have been articulated that aid the bedside clinician in detecting intra-abdominal hypertension as well as the abdominal compartment syndrome to initiate prompt and potentially life-saving intervention. At-risk patient populations should be routinely monitored and tiered interventions should be undertaken as a team approach to management.

Luckianow, Gina M.; Ellis, Matthew; Governale, Deborah; Kaplan, Lewis J.

2012-01-01

99

Recommended Therapies for Metabolic Defects in Polycystic Ovary Syndrome  

Microsoft Academic Search

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

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

100

Castillo-Morales' orofacial therapy: treatment of 67 children with Down syndrome.  

PubMed

Infants with Down syndrome often present with a typical orofacial disorder, the features of which include hypotonicity of the perioral muscles, lips and chewing muscles, and a protruding tongue, later followed by active tongue protrusion, as well as problems with sucking, drooling, etc. This study presents the effects of Castillo-Morales' therapy with 67 Down syndrome children (average age at start of therapy 13.9 months), who wore the palatal plate intermittently for an average of 12.1 months. Significant positive results were obtained in spontaneous tongue position, upper and lower lip tonicity and position, mouth closure, drooling and sucking. PMID:1828445

Limbrock, G J; Fischer-Brandies, H; Avalle, C

1991-04-01

101

Regulation therapy by Castillo-Morales in children with Down syndrome: primary and secondary orofacial pathology.  

PubMed

Since Castillo-Morales developed the Orofacial Regulation Therapy for children with Down syndrome in the mid 1970s, close observation of orofacial symptoms in the growing child has led to new findings. Primary orofacial signs are present at birth through age one; secondary alterations develop with untreated school-age children. A synopsis of the most important disorders in children with Down syndrome is given. Findings that relate to malfunction are summed up; these findings can be influenced by Orofacial Regulation Therapy. PMID:2147925

Limbrock, G J; Hoyer, H; Scheying, H

102

Stevens-Johnson syndrome developing in a girl with minimal change nephrotic syndrome on deflazacort therapy  

Microsoft Academic Search

RationaleStevens-Johnson syndrome is a vesiculobullous disease of skin and mucosa. This generalized hypersensitivity reaction is well known to occur in association with certain drugs or viral infections. The disease is often treated with corticosteroids. However, corticosteroids induced Stevens-Johnson syndrome have been described. We present a case with nephrotic syndrome who developed cutaneous bullae and mucositis while being treated with oral

W. Kim; E. Kim; H. Lee; J. Jeong; S. Nahm

2004-01-01

103

Posterior Reversible Encephalopathy Syndrome (PRES\\/RPLS) During Pulse Steroid Therapy in Macrophage Activation Syndrome  

Microsoft Academic Search

Posterior reversible encephalopathy syndrome (PRES)or Reversible posterior leukoencephalopathy syndrome (RPLS) is a neurological\\u000a complication associated with various illnesses and medications(including rheumatological illnesses and their medications).\\u000a Cyclosporine is the drug which is most commonly implicated in the causation of this condition. The authors report a 6 year\\u000a old patient with systemic onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome who developed

Sharath Kumar; L. Rajam

104

Stevens-Johnson syndrome in children receiving phenobarbital therapy and cranial radiotherapy  

Microsoft Academic Search

Stevens-Johnson syndrome (SJS) is a severe cutaneous eruption that most often appears as an adverse reaction to medication.\\u000a In this report, we present two children with brain tumour who developed SJS while receiving cranial irradiation and anticonvulsant\\u000a therapy with phenobarbital. Concomitant application of these two therapies may play an important role in the occurrence of\\u000a the disease.

Antonio Ruggiero; Paola Sabrina Buonuomo; Palma Maurizi; Maria Pia Cefalo; Mirta Corsello; Riccardo Riccardi

2007-01-01

105

SMART syndrome: a late reversible complication after radiation therapy for brain tumours  

Microsoft Academic Search

With intensified treatment leading to longer survival, complications of therapy for brain tumours are more frequently observed.\\u000a Regarding radiation therapy, progressive and irreversible white matter disease with cognitive decline is most feared. We report\\u000a on four patients with reversible clinical and radiological features occurring years after radiation for brain tumours, suggestive\\u000a for the so called SMART syndrome (stroke-like migraine attacks

Joost P. KerklaanGeert; Geert J. Lycklama á Nijeholt; Ruud G. J. Wiggenraad; Bianca Berghuis; Tjeerd J. Postma; Martin J. B. Taphoorn

2011-01-01

106

Prospects for Gene Therapy in the Fragile X Syndrome  

ERIC Educational Resources Information Center

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

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

2004-01-01

107

Short and long-term beneficial effects of a multidisciplinary therapy for the control of metabolic syndrome in obese adolescents  

Microsoft Academic Search

Visceral fat is highly correlated with metabolic syndrome in obese adolescents. The aims of this study were to determine the prevalence of metabolic syndrome and to assess the effect of a long-term (1 year) intervention with multidisciplinary therapy in predicting metabolic syndrome among obese adolescents, as well as to compare short- with long-term therapy. Eighty-three postpuberty obese adolescents were recruited,

Danielle Arisa Caranti; Marco Túlio de Mello; Wagner L. Prado; Lian Tock; Kãli O. Siqueira; Aline de Piano; Mara C. Lofrano; Dejaldo M. J. Cristofalo; Henrique Lederman; Sérgio Tufik; Ana R. Dâmaso

2007-01-01

108

Endoscopic therapy of the buried bumper syndrome: a clinical algorithm  

Microsoft Academic Search

Background  Buried bumper syndrome is a rare complication of percutaneous endoscopic gastrostomy with an overgrowth of the inner flange\\u000a of the tube. Various therapeutic approaches in case studies in the literature are described.\\u000a \\u000a \\u000a \\u000a Methods  We present the prospective data collection and analysis of 18 cases of buried bumper syndrome treated in our institution between\\u000a 1 January 1998 and 31 December 2005.\\u000a \\u000a \\u000a \\u000a Results  The

Thomas Horbach; Viola Teske; Werner Hohenberger; Michael Siassi

2007-01-01

109

Papillary thyroid carcinoma after recombinant GH therapy for Turner syndrome  

Microsoft Academic Search

Turner syndrome (TS) has been included for several years among the indications for GH treatment, generally with satisfactory outcomes. Nevertheless, the long-term effects of this treatment in non-GH deficient patients are not fully known. The incidence of thyroid carcinoma is rare in patients during childhood, it is unusual to find this neoplasia in children under sixteen years old. This article

P Cabanas; T Garcia-Caballero; J Barreiro; L Castro-Feijoo; R Gallego; T Arevalo; R Canete; M Pombo

2005-01-01

110

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

111

Therapy Insight: aortic aneurysm and dissection in Marfan's syndrome  

Microsoft Academic Search

Aortic dissection and aneurysm are common clinical problems with life-threatening consequences; they are also the hallmark of several genetic diseases, including Marfan's syndrome (MFS). In spite of clinical and surgical advances that have increased life expectancy for affected patients, cardiovascular manifestations remain significant contributors to morbidity and mortality in MFS. Dissecting aortic aneurysm in this disorder is accounted for by

Harry C Dietz; Francesco Ramirez

2004-01-01

112

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

113

Prevention and therapy of the adult respiratory distress syndrome  

Microsoft Academic Search

The complex pathophysiology of adult respiratory distress syndrome (ARDS) makes preventive and therapeutic concepts difficult. Ample experimental evidence indicates that ARDS can be prevented by blocking systemic inflammatory agents. Clinically, only heparin, for inhibition of coagulation phenomena, is presently used among this array of approaches. Corticosteroids have not proven to be beneficial in ARDS. Alternative antiinflammatory agents are being proposed

B. Temmesfeld-Wollbrück; D. Walmrath; F. Grimminger; W. Seeger

1995-01-01

114

Fragile X syndrome: from molecular genetics to therapy  

Microsoft Academic Search

Fragile X syndrome, the main cause of inherited mental retardation, is caused by transcriptional silencing of the fragile X mental retardation gene, FMR1. Absence of the associated protein FMRP leads to the dysregulation of many genes creating a phenotype of ADHD, anxiety, epilepsy and autism. The core aim of this review is to summarise two decades of molecular research leading

C D’Hulst; R F Kooy

2009-01-01

115

Antibiotic Therapy in Chronic Prostatitis\\/Chronic Pelvic Pain Syndrome  

Microsoft Academic Search

\\u000a The use of antibiotics in chronic prostatitis\\/chronic pelvic pain syndrome is widespread and generally accepted. This chapter\\u000a explores the reasons for this acceptance, presents the evidence based information that would refute this practice, and delineates\\u000a the areas of confusion that still confound physicians endeavoring to care for these patients.

Russell Blair Egerdie

116

Anti–interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes  

Microsoft Academic Search

BackgroundIL-5 is a cytokine critically involved in regulating several aspects of eosinophils including their production, activation, and tissue recruitment. As such, IL-5 may be involved in the pathogenesis of hypereosinophilic syndromes, a group of poorly treated diverse disorders characterized by sustained peripheral blood and\\/or tissue eosinophilia.

Jennifer K Garrett; Sean C Jameson; Blythe Thomson; Margaret H Collins; Lynne E Wagoner; Debbie K Freese; Lisa A Beck; Joshua A Boyce; Alexandra H Filipovich; Joyce M Villanueva; Steven A Sutton; Amal H Assa'ad; Marc E Rothenberg

2004-01-01

117

Increase in Prefrontal Cortical Volume following Cognitive Behavioural Therapy in Patients with Chronic Fatigue Syndrome  

ERIC Educational Resources Information Center

|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…

de Lange, Floris P.; Koers, Anda; Kalkman, Joke S.; Bleijenberg, Gijs; Hagoort, Peter; van der Meer, Jos W. M.; Toni, Ivan

2008-01-01

118

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

ERIC Educational Resources Information Center

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

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

2011-01-01

119

High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome  

Microsoft Academic Search

We treated 5 patients with polyneurop- athy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome and multifo- cal bone lesions or diffuse bone marrow plasmacytic infiltration with high-dose therapy (HDT) and autologous blood stem cell transplantation. In all cases, the treat- ment produced remission of plasma cell proliferation associated with marked im- provement in the patients' performance status, neurologic

Arnaud Jaccard; Bruno Royer; Dominique Bordessoule; Jean-Claude Brouet; Jean-Paul Fermand

120

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

ERIC Educational Resources Information Center

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

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

2011-01-01

121

Stevens-Johnson syndrome limited to multiple sites of radiation therapy in a patient receiving phenobarbital  

Microsoft Academic Search

Stevens-Johnson syndrome (SJS) is a severe cutaneous eruption that most often appears as an adverse reaction to a medication. There have been 21 reported cases of atypical erythema multiforme, toxic epidermal necrolysis, and SJS arising in patients receiving radiation therapy in addition to phenytoin, phenobarbital, or carbamazepine. We report the second case of SJS resulting from concomitant phenobarbital and radiation

Karynne O. Duncan; Robert E. Tigelaar; Jean L. Bolognia

1999-01-01

122

Side Effects of Combined Therapy of Methylprednisolone and Intravenous Immunoglobulin in Guillain-Barré Syndrome  

Microsoft Academic Search

Side effects were compared in 9 patients with Guillain-Barré syndrome treated with standard intravenous immunoglobulin (IVIg) only and in 9 treated with combined methylprednisolone and IVIg therapy. Headache occurred in 2 in both groups, indicative that pre-infusion with steroids does not prevent headache. Transient liver function disturbances were present in 2 patients of the former group and in 6 of

M. Odaka; M. Tatsumoto; E. Hoshiyama; K. Hirata; N. Yuki

2005-01-01

123

New Carpal Ligament Traction Device for the Treatment of Carpal Tunnel Syndrome Unresponsive to Conservative Therapy  

Microsoft Academic Search

This study evaluated the treatment efficacy and patient satisfaction of a new hand traction device called C-TRAC in patients that failed conservative therapy for carpal tunnel syndrome (CTS). Patients were diagnosed with electromyography and nerve conduction studies. Only patients with a positive Phalens test and a Visual Analog Scale (VAS) of more than 5\\/10 were eligible for the study. The

Julian Sosner; A PORRATA

2007-01-01

124

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

125

Corticosteroid therapy for prevention of respiratory distress syndrome in severe preeclampsia  

Microsoft Academic Search

Objective: The objective of the study was to determine the efficacy and side effects of corticosteroid therapy for pregnant women with severe preeclampsia in the prevention of respiratory distress syndrome in their premature neonates. Study Design: A prospective double-blind randomized trial enrolled 218 pregnant women with severe preeclampsia and gestational age between 26 and 34 weeks. One hundred ten received

Melania M. R. Amorim; Luiz Carlos Santos; Anibal Faúndes

1999-01-01

126

Bronchiolitis Obliterans Organizing Pneumonia Syndrome Primed by Radiation Therapy to the Breast  

Microsoft Academic Search

Reports of bronchiolitis obliterans organizing pneumonia (BOOP) occurring in women after radiation therapy for breast cancer have suggested that radiation to the lung could participate in the develop- ment of BOOP. We now describe the clinical, radiographic, functional, and bronchoalveolar lavage characteristics of this syndrome in a series of 15 patients reported to the Groupe d'Etudes et de Re- cherche

BRUNO CRESTANI; DOMINIQUE VALEYRE; SERGE RODEN; BENOÎT WALLAERT; JEAN-CHARLES DALPHIN

127

The Intervention of Rehabilitation Therapy on the Treatment of Ramsay Hunt Syndrome  

Microsoft Academic Search

This report presents 2 cases of Ramsay Hunt Syndrome with different levels of severity treated effectively with integrated rehabilitative program. Case one was a 36-year-old female experiencing right earache, auricular eruption, and fuzzy oral pronunciation 5 days before admission. Although combination therapy of acyclovir and prednisone was given immediately after hospitalization, level of facial palsy only improved from grade III

Cheng-Wen Ho; Kao-Chung Tsai

128

Pharmacologic Therapy for the Cancer Anorexia\\/Weight Loss Syndrome: A Data-Driven, Practical Approach  

Microsoft Academic Search

The cancer anorexia\\/weight loss syndrome occurs in over 80% of patients with incurable cancer and is associated with a poor prognosis and negative effects on quality of life. Educating patients and families plays an important role in its management, and caregivers sometimes forget that in select patients who are candidates for it, antineoplastic therapy can occasionally reverse some aspects of

Aminah Jatoi

2006-01-01

129

Minimal Change Nephrotic Syndrome Developing during Postoperative Interferon-Beta Therapy for Malignant Melanoma  

Microsoft Academic Search

A 64-year-old man presented with proteinuria during postoperative interferon (IFN)-? therapy against malignant melanoma. Renal pathologic findings were consistent with minimal change nephrotic syndrome (MCNS) showing extensive foot process effacement of visceral glomerular epithelial cells (podocyte). Nephrotic range proteinuria gradually regressed after stoppage of local injection of IFN-? without glucocorticoid treatment. To our knowledge this is the first report that

Kazushi Nakao; Hitoshi Sugiyama; Eiichi Makino; Hironori Matsuura; Akiko Ohmoto; Taro Sugimoto; Haruo Ichikawa; Jun Wada; Yasushi Yamasaki; Hirofumi Makino

2002-01-01

130

Subacromial Impingement Syndrome as a Consequence of Botulinum Therapy to the Upper Trapezii: A Case Report  

Microsoft Academic Search

Abbott Z, Richardson JK. Subacromial impingement syndrome as a consequence of botulinum therapy to the upper trapezii: a case report.Scapular upward rotation is predominantly achieved via a force coupling involving the upper and lower trapezius and the serratus anterior. Although studies have shown a relationship between abnormal scapular motion and subacromial impingement, it has been unclear whether the altered scapular

Zachary Abbott; James K. Richardson

2007-01-01

131

Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.  

PubMed Central

11 children with either cystinosis or Lowe's syndrome had a reduced content of plasma and muscle carnitine due to renal Fanconi syndrome. After treatment with oral L-carnitine, 100 mg/kg per d divided every 6 h, plasma carnitine concentrations became normal in all subjects within 2 d. Initial plasma free fatty acid concentrations, inversely related to free carnitine concentrations, were reduced after 7-20 mo of carnitine therapy. Muscle lipid accumulation, which varied directly with duration of carnitine deficiency (r = 0.73), improved significantly in three of seven rebiopsied patients after carnitine therapy. One Lowe's syndrome patient achieved a normal muscle carnitine level after therapy. Muscle carnitine levels remained low in all cystinosis patients, even though cystinotic muscle cells in culture took up L-[3H]carnitine normally. The half-life of plasma carnitine for cystinotic children given a single oral dose approximated 6.3 h; 14% of ingested L-carnitine was excreted within 24 h. Studies in a uremic patient with cystinosis showed that her plasma carnitine was in equilibrium with some larger compartment and may have been maintained by release of carnitine from the muscle during dialysis. Because oral L-carnitine corrects plasma carnitine deficiency, lowers plasma free fatty acid concentrations, and reverses muscle lipid accumulation in some patients, its use as therapy in renal Fanconi syndrome should be considered. However, its efficacy in restoring muscle carnitine to normal, and the optimal dosage regimen, have yet to be determined. Images

Gahl, W A; Bernardini, I; Dalakas, M; Rizzo, W B; Harper, G S; Hoeg, J M; Hurko, O; Bernar, J

1988-01-01

132

Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome.  

PubMed

11 children with either cystinosis or Lowe's syndrome had a reduced content of plasma and muscle carnitine due to renal Fanconi syndrome. After treatment with oral L-carnitine, 100 mg/kg per d divided every 6 h, plasma carnitine concentrations became normal in all subjects within 2 d. Initial plasma free fatty acid concentrations, inversely related to free carnitine concentrations, were reduced after 7-20 mo of carnitine therapy. Muscle lipid accumulation, which varied directly with duration of carnitine deficiency (r = 0.73), improved significantly in three of seven rebiopsied patients after carnitine therapy. One Lowe's syndrome patient achieved a normal muscle carnitine level after therapy. Muscle carnitine levels remained low in all cystinosis patients, even though cystinotic muscle cells in culture took up L-[3H]carnitine normally. The half-life of plasma carnitine for cystinotic children given a single oral dose approximated 6.3 h; 14% of ingested L-carnitine was excreted within 24 h. Studies in a uremic patient with cystinosis showed that her plasma carnitine was in equilibrium with some larger compartment and may have been maintained by release of carnitine from the muscle during dialysis. Because oral L-carnitine corrects plasma carnitine deficiency, lowers plasma free fatty acid concentrations, and reverses muscle lipid accumulation in some patients, its use as therapy in renal Fanconi syndrome should be considered. However, its efficacy in restoring muscle carnitine to normal, and the optimal dosage regimen, have yet to be determined. PMID:3276734

Gahl, W A; Bernardini, I; Dalakas, M; Rizzo, W B; Harper, G S; Hoeg, J M; Hurko, O; Bernar, J

1988-02-01

133

Menopause, the metabolic syndrome, and mind-body therapies.  

PubMed

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

134

Menstrual Cyclicity After Metformin Therapy in Polycystic Ovary Syndrome  

Microsoft Academic Search

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

Elsy Velázquez M; Arnaldo Acosta; Soaira G Mendoza

1997-01-01

135

Management of hyperlipidemia in children with refractory nephrotic syndrome: the effect of statin therapy.  

PubMed

The efficacy and safety of hydroxymethylglutaric coenzyme A reductase inhibitor (statins) in the treatment of hyperlipidemia were evaluated in 12 infants and children with steroid-resistant nephrotic syndrome followed prospectively for 1 to 5 years. All patients experienced a hypolipidemic response with a marked reduction in their total cholesterol (40%), low-density lipoprotein cholesterol (44%), and triglyceride levels (33%), but no appreciable change in high-density lipoprotein cholesterol. Statin therapy was well tolerated without clinical or laboratory adverse effects. In spite of a significant hypolipidemic response to statin therapy there were no changes observed in the degree of proteinuria, hypoalbuminemia, or in the rate of progression to chronic renal failure. Long-term controlled studies with statin therapy are needed to further document or negate their renoprotective role in refractory nephrotic syndrome. PMID:9063427

Sanjad, S A; al-Abbad, A; al-Shorafa, S

1997-03-01

136

A Patient with Multiple Immune Reconstitution Inflammatory Syndrome (IRIS) Following Initiation of Antiretroviral Therapy  

PubMed Central

The Immune Reconstitution Inflammatory Syndrome (IRIS) is an exaggerated pathological inflammatory reaction which occurs after the initiation of the antiretroviral therapy, due to the exuberant immune responses to the occult or the apparent opportunistic infections. The hallmark of the syndrome is the paradoxical worsening of an existing infection or a disease process or the appearance of a new infection or a disease process soon after the initiation of the antiretroviral therapy. The most common forms of IRIS occur in association with tuberculosis and chronic viral and invasive fungal infections. Multiple IRIS in a patient is extremely rare. Our patient had multiple manifestations of IRIS, in the form of cryptococcal meningitis, toxoplasmosis and Cytomegalovirus (CMV) retinitis after the initiation of highly active antiretroviral therapy.

Achappa, Basavaprabhu; Madi, Deepak; Shetty, Nishitha; Mahalingam, Soundarya

2012-01-01

137

Fragile X syndrome: From protein function to therapy.  

PubMed

Fragile X syndrome (FXS) is the leading monogenic cause of intellectual disability and autism. The FMR1 gene contains a CGG repeat present in the 5'-untranslated region which can be unstable upon transmission to the next generation. The repeat is up to 55 CGGs long in the normal population. In patients with fragile X syndrome (FXS), a repeat length exceeding 200 CGGs generally leads to methylation of the repeat and the promoter region, which is accompanied by silencing of the FMR1 gene. The disease is a result of lack of expression of the fragile X mental retardation protein leading to severe symptoms, including intellectual disability, hyperactivity, and autistic-like behavior. The FMR1 protein (FMRP) has a number of functions. The translational dysregulation of a subset of mRNAs targeted by FMRP is probably the major contribution to FXS. FMRP is also involved in mRNA transport to synapses where protein synthesis occurs. For some FMRP-bound mRNAs, FMRP is a direct modulator of mRNA stability either by sustaining or preventing mRNA decay. Increased knowledge about the role of FMRP has led to the identification of potential treatments for fragile X syndrome that were often tested first in the different animal models. This review gives an overview about the present knowledge of the function of FMRP and the therapeutic strategies in mouse and man. © 2013 Wiley Periodicals, Inc. PMID:24115651

Bagni, Claudia; Oostra, Ben A

2013-09-24

138

The role of the therapeutic relationship in cognitive behaviour therapy for chronic fatigue syndrome.  

PubMed

Cognitive behaviour therapy (CBT) for chronic fatigue syndrome (CFS) can reduce fatigue and impairment. Recently, it was found that changes in fatigue-perpetuating factors, i.e. focusing on symptoms, control over fatigue, perceived activity and physical functioning, are associated with and explain up to half of the variance in fatigue during CBT for CFS. The therapy relationship, e.g. outcome expectations and working alliance, may also contribute to treatment outcome. We aimed to examine the role of the therapy relationship in CBT and determine whether it exerts its effect independently of changes in fatigue-perpetuating factors. We used a cohort of 217 CFS patients in which the pattern of change in fatigue-perpetuating factors was examined previously. Fatigue, therapy relationship and fatigue-perpetuating factors were measured at the start of therapy, three times during CBT and at the end of therapy. Baseline outcome expectations and agreement about the content of therapy predicted post therapy fatigue. A large part of the variance in post-treatment fatigue (25%) was jointly explained by outcome expectations, working alliance and changes in fatigue-perpetuating factors. From this, we conclude that positive outcome expectations and task agreement seem to facilitate changes in fatigue-perpetuating factors during CBT for CFS. It is therefore important to establish a positive therapy relationship early in therapy. PMID:23639303

Heins, Marianne J; Knoop, Hans; Bleijenberg, Gijs

2013-04-09

139

A case of calcineurin-inhibitor induced pain syndrome associated with tacrolimus therapy for ulcerative colitis.  

PubMed

A 23-year-old woman was admitted with a relapse of ulcerative colitis. Tacrolimus therapy was initiated following inadequate response to corticosteroid therapy. Although the symptoms partially improved, she suddenly developed severe pain localized to the lower limbs on day 16 of tacrolimus therapy. By day 17, she was unable to move. Magnetic resonance imaging revealed born marrow edema in the lower limbs. We suspected calcineurin-inhibitor induced pain syndrome (CIPS) due to tacrolimus therapy. The pain improved within approximately four weeks of tacrolimus cessation. CIPS that is not associated with organ transplantation is a rare occurrence. Here we report a rare case of CIPS that was caused by tacrolimus therapy in a patient with ulcerative colitis. PMID:24097149

Tadano, Toshihiro; Takahashi, Hiroki; Sugawara, Kaori; Sugimura, Mikako; Iwabuchi, Masahiro; Mano, Yutaka; Ukai, Katsuaki; Tadokoro, Keiichi; Mori, Yoshimasa

2013-10-01

140

Stevens-Johnson syndrome in a boy with nephrotic syndrome during prednisolone therapy  

Microsoft Academic Search

Stevens-Johnson syndrome (SJS) is a mucocutaneous disease that can be lethal. It can occur in association with altered immunological conditions and the administration of various drugs, including corticosteroids. We report a case of SJS in a 14-year-old male with nephrotic syndrome, who was treated with oral prednisolone for 6 weeks. He presented symptoms of fever, skin lesions consisting of multiple vesiculopapular

Dae Sun Jo; Jun-Won Yang; Pyoung Han Hwang; Dae-Yeol Lee

2003-01-01

141

Periradicular therapy in lumbar radicular syndromes: Methodology and results  

Microsoft Academic Search

Summary Periradicular therapy (PRT) has become popular in the treatment of lumbar radicular complaints, both primary, due to disc herniations (Group 1), and postoperative, following disc surgery (Group 2). However, hitherto repored data on ‘periradicular injections’ are more of a technical nature. The present study was designed to evaluate the therapeutic success of CT-versus fluoroscope-guided periradicular injections of local anaesthetics

M. Lutze; R. Stendel; J. Vesper; M. Brock

1997-01-01

142

Fetoscopic laser therapy for twin-to-twin transfusion syndrome.  

PubMed

Twin-to-twin transfusion syndrome (TTTS) is a unique and complicated phenomenon occurring in 10-15% of monochorionic gestations. The chronic unbalanced distribution of blood volume across placental anastomoses between the donor and recipient fetuses leads to multisystem organ impairment including maladaptive changes in both fetuses. Fetoscopic selective laser photocoagulation (SLPC) is now established as the primary treatment modality for advanced stages of TTTS. SLPC is also associated with a risk reduction in fetal demise and long-term neurological impairment. PMID:23395141

Khalek, Nahla; Johnson, Mark P; Bebbington, Michael W

2013-02-01

143

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

144

Cognitive behaviour therapy for the chronic fatigue syndrome: a randomized controlled trial.  

PubMed Central

OBJECTIVE--To evaluate the acceptability and efficacy of adding cognitive behaviour therapy to the medical care of patients presenting with the chronic fatigue syndrome. DESIGN--Randomised controlled trial with final assessment at 12 months. SETTING--An infectious diseases outpatient clinic. SUBJECTS--60 consecutively referred patients meeting consensus criteria for the chronic fatigue syndrome. INTERVENTIONS--Medical care comprised assessment, advice, and follow up in general practice. Patients who received cognitive behaviour therapy were offered 16 individual weekly sessions in addition to their medical care. MAIN OUTCOME MEASURES--The proportions of patients (a) who achieved normal daily functioning (Karnofsky score 80 or more) and (b) who achieved a clinically significant improvement in functioning (change in Karnofsky score 10 points or more) by 12 months after randomisation. RESULTS--Only two eligible patients refused to participate. All randomised patients completed treatment. An intention to treat analysis showed that 73% (22/30) of recipients of cognitive behaviour therapy achieved a satisfactory outcome as compared with 27% (8/30) of patients who were given only medical care (difference 47 percentage points; 95% confidence interval 24 to 69). Similar differences were observed in subsidiary outcome measures. The improvement in disability among patients given cognitive behaviour therapy continued after completion of therapy. Illness beliefs and coping behaviour previously associated with a poor outcome changed more with cognitive behaviour therapy than with medical care alone. CONCLUSION--Adding cognitive behaviour therapy to the medical care of patients with the chronic fatigue syndrome is acceptable to patients and leads to a sustained reduction in functional impairment.

Sharpe, M.; Hawton, K.; Simkin, S.; Surawy, C.; Hackmann, A.; Klimes, I.; Peto, T.; Warrell, D.; Seagroatt, V.

1996-01-01

145

May-Thurner syndrome: case report and review of the literature involving modern endovascular therapy.  

PubMed

May-Thurner syndrome is a rare clinical entity involving venous obstruction of the left lower extremity. Obstruction occurs secondary to compression of the left common iliac vein between the right common iliac artery and the underlying vertebral body. Current management largely involves endovascular therapy. A review was conducted of six studies containing at least five patients with May-Thurner syndrome treated by endovascular therapy. We compiled data on 113 patients, analyzing patient demographics, treatment details, and outcome. An 18-year-old female presented 1 week after the onset of left lower extremity pain and swelling. Duplex ultrasonography revealed extensive left-sided deep venous thrombosis (DVT). Thrombolysis followed by iliac vein stent placement restored patency to the venous system, with subsequent resolution of symptoms. Review of 113 patients revealed that the majority were females (72%) presenting with DVT (77%), most of which was acute in onset (73%). Therapy consisted of catheter-directed thrombolysis and subsequent stent placement in the majority of patients, resulting in a mean technical success of 95% and a mean 1-year patency of 96%. Endovascular therapy is the current mainstay of treatment for May-Thurner syndrome. Review of the current literature supports treatment via catheter-directed thrombolysis followed by stent placement with good early results. PMID:19909680

Moudgill, Neil; Hager, Eric; Gonsalves, Carin; Larson, Robert; Lombardi, Joseph; DiMuzio, Paul

146

Contrasting effects of subcutaneous pulsatile GnRH therapy in congenital adrenal hypoplasia and Kallmann's syndrome.  

PubMed

A patient with congenital adrenal hypoplasia (AH) and hypogonadotrophic hypogonadism was treated with pulsatile subcutaneous GnRH therapy for 16 weeks in an attempt to induce puberty. No rise in serum LH or FSH concentrations occurred despite increasing doses of GnRH (2.8 micrograms/pulse-22.4 micrograms/pulse). In contrast a similar programme of therapy successfully initiated the biochemical changes of puberty in a patient with Kallmann's syndrome. Both patients before therapy had low basal serum LH and FSH concentrations with blunted LH and FSH responses to GnRH stimulation. After 1 week, serum LH and FSH rose into the normal adult range in the patient with Kallmann's syndrome. This study fails to confirm a previous report which suggested that intermittent low dose GnRH therapy may be of value in inducing puberty in AH. The reasons for the difference of pituitary responsiveness to GnRH in AH and Kallmann's syndrome are unclear at present. PMID:6439437

Gordon, D; Cohen, H N; Beastall, G H; Hay, I D; Thomson, J A

1984-12-01

147

The combination of medical treatment plus multicomponent behavioral therapy is superior to medical treatment alone in the therapy of irritable bowel syndrome  

Microsoft Academic Search

OBJECTIVE:Although the standard treatments for the irritable bowel syndrome (IBS) are medical, growing evidence indicates the substantial therapeutic value of psychological therapy. However, it has not been investigated whether the combination of multicomponent behavioral therapy plus medical treatment is more effective than medical treatment alone. The aim of this study was to investigate this question in patients consulting a tertiary

Ingeborg Heymann-Mönnikes; Rudolf Arnold; Irmela Florin; Christoph Herda; Siebke Melfsen; Hubert Mönnikes

2000-01-01

148

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

PubMed

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

Tan, Jie-Wen; Qi, Wei-Wei; Ye, Rui-Xin; Wu, Yuan-Yuan

2013-10-20

149

Effect of cyclosporine therapy on idiopathic membranous nephropathy presented with refractory nephrotic syndrome  

Microsoft Academic Search

Background. Recent studies suggested the possible benefits of cyclosporine (CsA) therapy in patients with membranous nephropathy, although\\u000a most of these studies were short-term. An uncontrolled retrospective study was undertaken to evaluate the long-term effect\\u000a of CsA therapy on idiopathic membranous nephropathy presented with refractory nephrotic syndrome.\\u000a \\u000a \\u000a \\u000a \\u000a Methods. The subjects were eight patients with idiopathic membranous nephropathy presenting with refractory nephrotic

Hiroyuki Iida; Takero Naito; Norihiko Sakai; Shuichi Aoki

2000-01-01

150

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

151

Pachymeningeal involvement in POEMS syndrome: dramatic cerebral MRI improvement after lenalidomide therapy.  

PubMed

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystemic disease associated with plasma cell dyscrasia and increased serum or plasma vascular endothelial growth factor (VEGF) levels, the latter likely responsible for several POEMS syndrome manifestations. Whereas peripheral neuropathy is the main neurological feature and a mandatory diagnostic criterium, central nervous system involvement is less common except for papilledema and stroke. We recently reported the frequent occurrence at brain MRI of cranial pachymeningeal involvement ina series of POEMS syndrome patients. Meningeal histopathology revealed hyperplasia of meningothelial cells, neovascularization, and obstructive vessel remodeling without inflammatory signs pointing to a role of VEGF in the meningeal manifestations. Here, we report the dramatic pachymeningeal improvement in patients undergoing lenalidomide therapy. These findings support the therapeutic role of lenalidomide and might shed further light on the pathophysiology of the disease PMID:22389216

Briani, Chiara; Manara, Renzo; Lessi, Federica; Citton, Valentina; Zambello, Renato; Adami, Fausto

2012-03-03

152

Antithrombotic therapy in acute coronary syndromes: guidelines translated for the clinician  

Microsoft Academic Search

The use of anticoagulant and antiplatelet therapy during the management of acute coronary syndromes (ACS) has been associated\\u000a with improvements in short- and long-term clinical outcomes, regardless of whether patients are managed conservatively or\\u000a with acute coronary revascularization. Translating the existing evidence for selection of the most appropriate antithrombotic\\u000a strategy has been summarized in available guideline recommendations. Given the breadth

S. Michael Gharacholou; Renato D. Lopes; Jeffrey B. Washam; L. Kristin Newby; Stefan K. James; John H. Alexander

2010-01-01

153

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 3 days or a daily program of local modalities (n?=?25) for 2 weeks. Response to treatment was evaluated with the Disability of Arm, Shoulder,

Erkan Kaya; Murat Zinnuroglu; Ilknur Tugcu

2011-01-01

154

Treatment Options in Advanced Myelodysplastic Syndrome, with Emphasis on Epigenetic Therapy  

Microsoft Academic Search

Medical management of myelodysplastic syndrome (MDS) remains challenging, particularly in advanced stages where the risk of\\u000a developing acute leukemia is very high and the prospect of survival is generally poor. Over the past decade, epigenetic changes\\u000a such as alterations in DNA methylation and histone modifications have been well described in MDS and are now recognized as\\u000a targets of therapy (epigenetic

Yasuhiro Oki; Jean-Pierre J. Issa

2007-01-01

155

Maintenance therapy with mycophenolate mofetil after rituximab in pediatric patients with steroid-dependent nephrotic syndrome  

Microsoft Academic Search

Rituximab (RTX) has a significant steroid-sparing effect in children with steroid-dependent nephrotic syndrome (SDNS). However,\\u000a patients are likely to relapse with the recovery of CD20+ cells. We conducted a small prospective cohort study with a historical\\u000a control to evaluate the effect of RTX infusion followed by mycophenolate mofetil (MMF) as a maintenance therapy. Nine patients\\u000a with SDNS who stopped their

Shuichi Ito; Koichi Kamei; Masao Ogura; Mai Sato; Takuya Fujimaru; Tomoaki Ishikawa; Tomohiro Udagawa; Kazumoto Iijima

156

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

Microsoft Academic Search

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

Michael van Husen; Markus J. Kemper

2011-01-01

157

Do glutathione-S-transferase polymorphisms influence response to intravenous cyclophosphamide therapy in idiopathic nephrotic syndrome?  

Microsoft Academic Search

The response to cyclophosphamide (CP) is variable and difficult to predict in children with idiopathic nephrotic syndrome\\u000a (INS). The polymorphic expression of glutathione-S-transferase (GST) may affect the remission rate after CP therapy. In this\\u000a study, we evaluated the correlation of GST polymorphism and response to CP in INS. We studied GST polymorphism in 74 children\\u000a with steroid-sensitive (44) and steroid-resistant

Sheetal V. Sharda; Sanjeev Gulati; Gaurav Tripathi; Tabrez Jafar; Alok Kumar; Raj Kumar Sharma; Suraksha Agrawal

2008-01-01

158

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

Microsoft Academic Search

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

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

2000-01-01

159

Pathogenesis of lethal cardiac arrhythmias in Mecp2 mutant mice: implication for therapy in Rett syndrome.  

PubMed

Rett syndrome is a neurodevelopmental disorder typically caused by mutations in methyl-CpG-binding protein 2 (MECP2) in which 26% of deaths are sudden and of unknown cause. To explore the hypothesis that these deaths may be due to cardiac dysfunction, we characterized the electrocardiograms in 379 people with Rett syndrome and found that 18.5% show prolongation of the corrected QT interval (QTc), an indication of a repolarization abnormality that can predispose to the development of an unstable fatal cardiac rhythm. Male mice lacking MeCP2 function, Mecp2(Null/Y), also have prolonged QTc and show increased susceptibility to induced ventricular tachycardia. Female heterozygous null mice, Mecp2(Null/+), show an age-dependent prolongation of QTc associated with ventricular tachycardia and cardiac-related death. Genetic deletion of MeCP2 function in only the nervous system was sufficient to cause long QTc and ventricular tachycardia, implicating neuronally mediated changes to cardiac electrical conduction as a potential cause of ventricular tachycardia in Rett syndrome. The standard therapy for prolonged QTc in Rett syndrome, ?-adrenergic receptor blockers, did not prevent ventricular tachycardia in Mecp2(Null/Y) mice. To determine whether an alternative therapy would be more appropriate, we characterized cardiomyocytes from Mecp2(Null/Y) mice and found increased persistent sodium current, which was normalized when cells were treated with the sodium channel-blocking anti-seizure drug phenytoin. Treatment with phenytoin reduced both QTc and sustained ventricular tachycardia in Mecp2(Null/Y) mice. These results demonstrate that cardiac abnormalities in Rett syndrome are secondary to abnormal nervous system control, which leads to increased persistent sodium current. Our findings suggest that treatment in people with Rett syndrome would be more effective if it targeted the increased persistent sodium current to prevent lethal cardiac arrhythmias. PMID:22174313

McCauley, Mark D; Wang, Tiannan; Mike, Elise; Herrera, Jose; Beavers, David L; Huang, Teng-Wei; Ward, Christopher S; Skinner, Steven; Percy, Alan K; Glaze, Daniel G; Wehrens, Xander H T; Neul, Jeffrey L

2011-12-14

160

Pathogenesis of Lethal Cardiac Arrhythmias in Mecp2 Mutant Mice: Implication for Therapy in Rett Syndrome  

PubMed Central

Rett Syndrome is a neurodevelopmental disorder typically caused by mutations in Methyl-CpG-Binding Protein 2 (MECP2) in which 26% of deaths are sudden and of unknown cause. To explore the hypothesis that these deaths may be due to cardiac dysfunction, we characterized the electrocardiograms (ECGs) in 379 people with Rett syndrome and found that 18.5% show prolongation of the corrected QT interval (QTc), indicating a repolarization abnormality that can predispose to the development of an unstable fatal cardiac rhythm. Male mice lacking MeCP2 function, Mecp2Null/Y, also have prolonged QTc and show increased susceptibility to induced ventricular tachycardia. Female heterozygous null mice, Mecp2Null/+, show an age-dependent prolongation of QTc associated with ventricular tachycardia and cardiac-related death. Genetic deletion of MeCP2 function in only the nervous system was sufficient to cause long QTc and ventricular tachycardia, implicating neuronally-mediated changes to cardiac electrical conduction as a potential cause of ventricular tachycardia in Rett syndrome. The standard therapy for prolonged QTc in Rett syndrome, ?-adrenergic receptor blockers, did not prevent ventricular tachycardia in Mecp2Null/Y mice. To determine whether an alternative therapy would be more appropriate, we characterized cardiomyocytes from Mecp2Null/Y mice and found increased persistent sodium current, which was normalized when cells were treated with the sodium channel-blocking anti-seizure drug phenytoin. Treatment with phenytoin reduced both QTc and sustained ventricular tachycardia in Mecp2Null/Y mice. These results demonstrate that cardiac abnormalities in Rett syndrome are secondary to abnormal nervous system control, which leads to increased persistent sodium current. Our findings suggest that treatment in people with Rett syndrome would be more effective if it targeted the increased persistent sodium current in order to prevent lethal cardiac arrhythmias.

McCauley, Mark D.; Wang, Tiannan; Mike, Elise; Herrera, Jose; Beavers, David L.; Huang, Teng-Wei; Ward, Christopher S.; Skinner, Steven; Percy, Alan K.; Glaze, Daniel G.; Wehrens, Xander H. T.; Neul, Jeffrey L.

2013-01-01

161

Compartment Syndrome of the Arm: A Complication of Noninvasive Blood Pressure Monitoring during Thrombolytic Therapy for Myocardial Infarction  

Microsoft Academic Search

We report a rare case of tricep compartment syndrome caused by a hematoma which resulted from noninvasive blood pressure monitoring (NIBPM) during thrombolytic therapy. Clinicians administering thrombolytic agents should be aware of the risk of bleeding and compartment syndrome at the site of NIBPM. Appropriate preventative measures should be instituted when using automated pneumatic cuffs. An understanding of the pathophysiology

J. Winslow Alford; Mark A. Palumbo; Michael J. Barnum

2002-01-01

162

Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation.  

PubMed

Hepatopulmonary syndrome (HPS)--a pulmonary complication observed in patients who have chronic liver disease and/or portal hypertension--is attributed to intrapulmonary vascular dilatation and induces severe hypoxemia. HPS is mainly detected when patients are included on the waiting list for orthotopic liver transplantation (OLT) and can be diagnosed by blood gas analysis, transthoracic contrast-enhanced echocardiography or body scan with (99m)Tc-labeled macroaggregated albumin perfusion. When the partial pressure of arterial oxygen (PaO(2)) is >or=80 mmHg, it is unlikely that the patient has HPS. When the PaO(2) is <80 mmHg, imaging techniques should be used to confirm or exclude pulmonary vascular dilatation. When a diagnosis of HPS is confirmed, knowing the degree of hypoxemia is crucial for optimum patient management. Patients who have a PaO(2) >or=50 mmHg but <60 mmHg should be prioritized for OLT. This procedure is not indicated for patients with a PaO(2) between 60 mmHg and 80 mmHg, although follow-up every 3 months is recommended to detect any deterioration of the PaO(2). A PaO(2) of <50 mmHg might preclude OLT, because mortality and morbidity after OLT are greatly increased in these patients. PMID:17978818

Pastor, Catherine M; Schiffer, Eduardo

2007-11-01

163

[Low-energy laser therapy in medial tibial stress syndrome].  

PubMed

The effect of low-energy laser therapy on shin splints was examined in a randomized study with an unblinded design. Constripts from the Jutland Dragoon regiment with shin splints were given either active laser treatment (40 mW in 60 sec per cm tender tibia edge) or placebo laser. All patients were exempted from normal duty concerning activities like running and march. Forty-nine patients participated in the study, 23 in the laser group and 26 in the control group. From the start the study was designed to be double-blind, but by accident the code was broken towards the end of the study. We found no significant differences between the groups regarding pain visual analog score and readiness to return to active duty after 14 days. PMID:7801390

Nissen, L R; Astvad, K; Madsen, L

1994-12-01

164

Exercise therapy is evidence-based treatment of shoulder impingement syndrome. Current practice or recommendation only.  

PubMed

Background: Subacromial impingement syndrome is the most common indication for shoulder operation. However, exercise therapy for the conservative treatment is recommended in the first instance. Aim: To evaluate the implementation of exercise therapy in impingement syndrome. Design: Retrospective study using structured postal questionnaire and data collected from hospital archive. Methods: A total of 104 consecutive patients who had undergone shoulder surgery due to impingement syndrome. Patients were asked about therapy modalities that they had received before and after the operation as well as pain (VAS) and functional impairment (ASES) at one-year follow-up. Results: Before surgery 49% of patients had not received advice for shoulder muscle exercises. After operation all patients had received mobility exercises, but one quarter of patients still reported that they had not received instructions about shoulder strength exercises. At the follow-up the means of the ASES index was 85 and use of NSAID had decreased by 75%. However, 15% of patients had moderate functional impairment (ASES under 60). Conclusion: About half of patients reported that they had not received advice for rotator cuff exercise therapy before surgery even though with it surgery would probably have been avoided in many cases. Although symptoms in most patients had decreased after operation, several patients still suffered from pain and decreased function. Still several patients had not received advice for shoulder strengthening exercises that are important to recovery. Clinical Rehabilitation Impact: The adherence to the current recommendations about exercise therapy is insufficient in clinical practice. Thus we recommend that it should be monitored in all institutions in which shoulder pain is treated. PMID:23480979

Ylinen, J; Vuorenmaa, M; Paloneva, J; Kiviranta, I; Kautiainen, H; Oikari, M; Häkkinen, A

2013-03-13

165

GH Therapy and first final height data in Noonan-like syndrome with loose anagen hair (Mazzanti syndrome).  

PubMed

Noonan-like syndrome with loose anagen hair (NS/LAH or Mazzanti Syndrome) is caused by a single missense mutation in SHOC2 promoting tN-myristoylation of the encoded protein. Cardinal features include facial features resembling NS, short stature often associated with proven growth hormone deficiency (GHD), typical ectodermal anomalies, and distinctive behavior. Overall, the clinical features are more severe than those generally observed in NS, even though the phenotype improves with age. We report on growth and pubertal trend in seven patients heterozygous for a mutated SHOC2 allele, treated with long-term GH-therapy, and final height (FH) in three of them. They were approximately -3 SDS below the Italian general population standards, they had very low IGF1 levels at baseline and GHD at pharmacological tests. All patients were treated with GH (0.035?mg/kg/day) for a mean period of 8.49?±?5.72 years. After the 1st year of GH-therapy, IGF1 level and height velocity had increased. Three of 7 patients reached the FH (-2.34?±?0.12 SDS) at 18.25?±?0.73 years, after GH administration for 12.39?±?2.12 years. Pubertal development was variable, showing a prolonged and delayed puberty or rapid pubertal progression that could impair the FH. Overall, our data in this small cohort suggest that NS/LAH patients benefit from long-term GH-therapy, although they do not show the characteristic catch-up growth of isolated GHD. While the observed growth and pubertal behavior is consistent with a dysfunction of the hypothalamic-pituitary-gonadal axis, the functional link between SHOC2 and the GH/IGF signaling pathways remains to be clarified. © 2013 Wiley Periodicals, Inc. PMID:24124081

Mazzanti, Laura; Tamburrino, Federica; Scarano, Emanuela; Perri, Annamaria; Vestrucci, Benedetta; Guidetti, Monica; Rossi, Cesare; Tartaglia, Marco

2013-10-03

166

Emerging therapies for treatment of acute lung injury and acute respiratory distress syndrome.  

PubMed

Acute lung injury/acute respiratory distress syndrome (ALI/ARDS) is a life-threatening form of respiratory failure that affects a heterogeneous population of critically ill patients. Although overall mortality appears to be decreasing in recent years due to improvements in supportive care, there are presently no proven, effective pharmacological therapies to treat ARDS and prevent its associated complications. The most common cause of death in ARDS is not hypoxemia or pulmonary failure, but rather multiple organ dysfunction syndrome (MODS), suggesting that improving survival in patients with ARDS may be linked to decreasing the incidence or severity of MODS. The key to developing novel treatments depends, in part, on identifying and understanding the mechanisms by which ARDS leads to MODS, although the heterogeneity and complexity of this disorder certainly poses a challenge to investigators. Novel therapies in development for treatment of ALI/ARDS include exogenous surfactant, therapies aimed at modulating neutrophil activity, such as prostaglandin and complement inhibitors, and treatments targeting earlier resolution of ARDS, such as beta-agonists and granulocyte macrophage colony-stimulating factor. From a clinical perspective, identifying subpopulations of patients most likely to benefit from a particular therapy and recognising the appropriate stage of illness in which to initiate treatment could potentially lead to better outcomes in the short term. PMID:17874973

Bosma, Karen J; Lewis, James F

2007-09-01

167

Group cognitive behaviour therapy for adults with Asperger syndrome and anxiety or mood disorder: a case series.  

PubMed

Individuals with Asperger syndrome are at increased risk for mental health problems compared with the general population, especially with regard to mood and anxiety disorders. Generic mental health services are often ill-equipped to offer psychotherapeutic treatments to this population, and specialized supports are difficult to find. This case series used a manualized cognitive behaviour therapy group programme (Mind Over Mood) with three adults diagnosed with Asperger syndrome, who were each unable to access psychotherapy through mainstream mental health services. This review highlights the benefits of a cognitive behaviour therapy (CBT) group approach for adults with Asperger syndrome and suggests some potential modifications to traditional CBT provision.? PMID:20827741

Weiss, Jonathan A; Lunsky, Yona

168

Family cognitive behaviour therapy for chronic fatigue syndrome: an uncontrolled study  

PubMed Central

Aim: To examine the efficacy of family focused cognitive behaviour therapy for 11–18 year olds with chronic fatigue syndrome. Methods: Twenty three patients were offered family focused cognitive behaviour therapy. The main outcome was a fatigue score of less than 4 and attendance at school 75% of the time. Results: Twenty patients completed treatment. Eighteen had completed all measures at six months follow up; 15 of these (83%) improved according to our predetermined criterion. Substantial improvements in social adjustment, depression, and fear were noted. Conclusions: Family focused cognitive behaviour therapy was effective in improving functioning and reducing fatigue in 11–18 year olds. Gains were maintained at six months follow up.

Chalder, T; Tong, J; Deary, V

2002-01-01

169

Clinical review: Acute respiratory distress syndrome - clinical ventilator management and adjunct therapy.  

PubMed

Acute respiratory distress syndrome (ARDS) is a potentially devastating form of acute inflammatory lung injury with a high short-term mortality rate and significant long-term consequences among survivors. Supportive care, principally with mechanical ventilation, remains the cornerstone of therapy - although the goals of this support have changed in recent years - from maintaining normal physiological parameters to avoiding ventilator-induced lung injury while providing adequate gas exchange. In this article we discuss the current evidence base for ventilatory support and adjunctive therapies in patients with ARDS. Key components of such a strategy include avoiding lung overdistension by limiting tidal volumes and airway pressures, and the use of positive end-expiratory pressure with or without lung recruitment manoeuvres in patients with severe ARDS. Adjunctive therapies discussed include pharmacologic techniques (for example, vasodilators, diuretics, neuromuscular blockade) and nonpharmacologic techniques (for example, prone position, alternative modes of ventilation). PMID:23672857

Silversides, Jonathan A; Ferguson, Niall D

2012-04-29

170

Impact of massage therapy in the treatment of linked pathologies: Scoliosis, costovertebral dysfunction, and thoracic outlet syndrome  

Microsoft Academic Search

Summary Objectives: To investigate the efficacy of massage therapy in the concurrent treatment of three related, but discrete, disorders: scoliosis, costovertebral dysfunction, and thoracic outlet syndrome. Methods: A 34-year-old female subject reported steadily increasing pain in the right shoulder over the previous 8 months. Chiropractic diagnosis and assessment by the author's clinical supervisor had identified these three conditions. Massage therapy

Michael Hamm

2006-01-01

171

Long-term therapy with cytochrome c, flavin mononucleotide and thiamine diphosphate for a patient with Kearns-Sayre syndrome  

Microsoft Academic Search

Cardiocrome®, containing cytochrome c, flavin mononucleotide and thiamine diphosphate, was administered intravenously for 22 months to a patient with Kearns-Sayre syndrome. This combined therapy alleviated the patient's easy fatigability, motor disability, corneal edema and chilblains, but was not effective for his opthalmoplegia, blepharoptosis or hearing loss. Truncal ataxia, dysphagia and an atrioventricular block appeared even with this therapy. Although the

Eiji Nakagawa; Shin-ichi Osari; Hideo Yamanouchi; Hiroshi Matsuda; Yu-ichi Goto; Ikuya Nonaka

1996-01-01

172

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.

Maneerattaporn, Monthira; Saad, Richard

2011-01-01

173

Pharmacologic and complementary and alternative medicine therapies for irritable bowel syndrome.  

PubMed

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

Chey, William D; Maneerattaporn, Monthira; Saad, Richard

2011-08-18

174

Sweet's syndrome in a patient with metastatic melanoma after ipilimumab therapy.  

PubMed

Sweet's syndrome, a neutrophilic dermatosis, is a known paraneoplastic complication occurring with various malignancies. It has been infrequently reported in association with melanoma. Ipilimumab is an antibody against an inhibitory cytotoxic T-lymphocyte-associated antigen 4 receptor on T cells. It is associated with a range of immune-related toxicities. Sweet's syndrome in association with ipilimumab has been reported only briefly in the literature. However, neutrophilic infiltration has been seen in biopsies of patients with ipilimumab-associated enterocolitis. We report, in detail, the case of a woman with metastatic melanoma undergoing ipilimumab therapy. After the second cycle of immunotherapy, the patient presented with high-grade fever followed by a rash on her hands. No infectious etiology was elucidated after an extensive workup. Pathologic examination of the skin biopsy from the hands confirmed neutrophilic dermatosis. The patient was treated with systemic steroids achieving complete remission of the skin lesions. Physicians should be aware of Sweet's syndrome as a possible cutaneous side effect of ipilimumab therapy and be familiar with its management. PMID:24113862

Pintova, Sofya; Sidhu, Harleen; Friedlander, Philip A; Holcombe, Randall F

2013-12-01

175

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

176

Therapy of resistant systemic necrotizing vasculitis. Polyarteritis, Churg-Strauss syndrome, Wegener's granulomatosis, and hypersensitivity vasculitis group disorders.  

PubMed

The systemic necrotizing vasculitides represent a difficult group of diseases from a therapeutic perspective. Even with successful therapy patients often experience a high degree of disease and treatment morbidity. Refractory disease, or that which fails to respond to standard therapy, is particularly challenging. A clinical approach to several vasculitic syndromes is outlined in this article with emphasis given to therapies supported by controlled investigations and those supported by consensus opinion where available. PMID:7732173

Calabrese, L H; Hoffman, G S; Guillevin, L

1995-02-01

177

Insulin therapy in acute coronary syndromes: an appraisal of completed and ongoing randomised trials with important clinical end points  

PubMed Central

Insulin therapy was first proposed as an adjunctive therapy in patients with acute coronary syndromes (ACS) in the 1960s. Since then, numerous randomised clinical trials have been conducted to determine the efficacy and to define the role of insulin therapy in ACS. This review will discuss: 1) completed trials of insulin therapy in ACS, including both glucose-insulin-potassium (GIK) approaches and non-GIK approaches; 2) trials of insulin therapy in critically ill non-ACS patients and the lessons from these trials that can be applied to trials of insulin in ACS patients; and 3) a summary of ongoing and planned trials of insulin in ACS patients.

Goyal, Abhinav; Nerenberg, Kara; Gerstein, Hertzel C; Umpierrez, Guillermo; Wilson, Peter WF

2013-01-01

178

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

PubMed

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

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

2007-01-01

179

Evidence for early initiation of growth hormone and transdermal estradiol therapies in girls with Turner syndrome.  

PubMed

Results from the first randomized, controlled trial of growth hormone (GH) therapy in girls with Turner syndrome (TS) followed to final height firmly establish that GH increases final adult stature. It is widely believed that the efficacy of GH is dependent upon the duration of therapy and dosing (longer duration and higher dose give taller final height). In a recent observational study involving more than 1500 French girls with TS, multivariate analyses demonstrated that the age at initiation of GH therapy accounted for a large percentage of the variance (44%) in response. Age at initiation of estrogen therapy was the second most important factor in determining GH effect (later initiation, taller final height), accounting for 22% of the variance. Overall, 0.3 cm in adult height was gained for every year that estrogen therapy was delayed. However, analyses of the French data restricted to patients with induced puberty revealed that those treated with percutaneous estradiol attained a height 2.1cm taller than those using oral estradiol or other estrogen preparations. In another study, girls receiving GH therapy (n=14) who were randomized to receive intramuscular (IM) depot estradiol early (12.0-12.9 years) attained at least as much height as those who initiated it late (14.0-14.9 years). These results are consistent with the observations in adult women that oral estrogens decrease IGF-I serum levels and suppress the IGF-independent metabolic effects of GH, while transdermal estrogens do not. Taken together, these studies suggest that girls with TS should begin GH therapy as soon as growth failure is demonstrated and that puberty should be induced with transdermal or IM estradiol. Girls for whom height is normalized with GH therapy in early childhood have the opportunity to undergo puberty at an age-appropriate time and still achieve a normal adult stature. PMID:16735135

Davenport, Marsha L

2006-06-02

180

Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome.  

PubMed

Hepatic sinusoidal obstruction syndrome is frequently linked to high-dose chemotherapy/total-body irradiation in recipients of haematopoietic stem cell transplantation, long-term use of azathioprine after organ transplantation and other chemotherapeutic agents. The incidence of hepatic sinusoidal obstruction syndrome varies from 0% to 70%, and is decreasing. Disease risk is higher in patients with malignancies, hepatitis C virus infection, those who present late, when norethisterone is used to prevent menstruation, and when broad-spectrum antibiotics and antifungals are used during and after the conditioning therapy. Hepatic sinusoidal obstruction syndrome presents with tender hepatomegaly, hyperbilirubinaemia and ascites, and diagnosis is mainly clinical (Seattle and Baltimore Criteria). Imaging excludes biliary obstruction and malignancy, but cannot establish accurate diagnosis. Hepatic sinusoidal obstruction syndrome may be prevented by avoiding the highest risk regimens, using non-myelo-ablative regimens, and reducing total-body irradiation dose. Treatment is largely symptomatic and supportive, because 70-80% of patients recover spontaneously. Tissue plasminogen activator plus heparin improves outcome in <30% of cases. Defibrotide, a polydeoxyribonucleotide, is showing encouraging results. Transjugular intrahepatic porto-systemic shunt relieves ascites, but does not improve outcome. Liver transplantation may be an option in the absence of malignancy. Prognosis is variable and depends on disease severity, aetiology and associated conditions. Death is most commonly caused by renal or cardiopulmonary failure. PMID:16393276

Helmy, A

2006-01-01

181

Hematologic responses to deferasirox therapy in transfusion-dependent patients with myelodysplastic syndromes  

PubMed Central

Background Reductions in transfusion requirements/improvements in hematologic parameters have been associated with iron chelation therapy in transfusion-dependent patients, including those with myelodysplastic syndromes; data on there reductions/improvements have been limited to case reports and small studies. Design and Methods To explore this observation in a large population of patients, we report a post-hoc analysis evaluating hematologic response to deferasirox in a cohort of iron-overloaded patients with myelodysplastic syndromes enrolled in the Evaluation of Patients’ Iron Chelation with Exjade® (EPIC) study using International Working Group 2006 criteria. Results Two-hundred and forty-seven, 100 and 50 patients without concomitant medication for myelodysplastic syndromes were eligible for analysis of erythroid, platelet and neutrophil responses, respectively. Erythroid, platelet and neutrophil responses were observed in 21.5% (53/247), 13.0% (13/100) and 22.0% (11/50) of the patients after a median of 109, 169 and 226 days, respectively. Median serum ferritin reductions were greater in hematologic responders compared with non-responders at end of study, although these differences were not statistically significant. A reduction in labile plasma iron to less than 0.4 ?mol/L was observed from week 12 onwards; this change did not differ between hematologic responders and non-responders. Conclusions This analysis suggests that deferasirox treatment for up to 1 year could lead to improvement in hematologic parameters in some patients with myelodysplastic syndromes.

Gattermann, Norbert; Finelli, Carlo; Della Porta, Matteo; Fenaux, Pierre; Stadler, Michael; Guerci-Bresler, Agnes; Schmid, Mathias; Taylor, Kerry; Vassilieff, Dominique; Habr, Dany; Marcellari, Andrea; Roubert, Bernard; Rose, Christian

2012-01-01

182

Surfactant therapy for maternal blood aspiration: an unusual cause of neonatal respiratory distress syndrome.  

PubMed

Surfactant replacement therapy is the main treatment of neonatal respiratory distress syndrome. However, surfactant therapy has been shown to be effective in the treatment of other diseases causing neonatal respiratory diseases such as pulmonary hemorrhage, meconium aspiration syndrome, pneumonia/sepsis, pulmonary edema or acute lung injury resulting a secondary surfactant deficiency (SSD). Rarely, as like as in the present patient, exogenous blood aspiration such as breast milk or formula aspiration may lead to SSD. Blood in alveolus leads to a significant biochemical and functional disturbance of the surfactant system and inhibits surfactant production. Here, the authors report a preterm infant of 33 wk gestational age with secondary surfactant deficiency due to maternal blood aspiration because of abruptio placentae. She was received two courses of beractant, a natural bovine surfactant, therapy in 24 h. She was extubated on second day and did not require oxygen on 4(th) day. To the authors' knowledge, this is the first reported case of SSD due to maternal blood aspiration treated with surfactant. In conditions such as abruptio placentae, infant should be protected from blood aspiration and if respiratory distress occurs, surfactant inhibition and need for surfactant administration should be considered. PMID:22120615

Celik, Istemi Han; Demirel, Gamze; Canpolat, Fuat Emre; Erdeve, Omer; Dilmen, Ugur

2011-11-26

183

Normal cerebrovascular reactivity in Stroke-like Migraine Attacks after Radiation Therapy syndrome.  

PubMed

Stroke-like Migraine Attacks after Radiation Therapy (SMART) syndrome is a rare complication of cranial irradiation. Radiation is well-known to impair vascular vessel architecture and function. We investigated the hypothesis of radiation-induced cerebral vascular reserve dysfunction as the underlying mechanism of SMART. Interictal cerebrovascular reactivity was investigated using Tc-99m hexamethylpropyleneamine oxime-SPECT and acetazolamide challenge in 3 patients. We found interictal hypoperfusion and normal cerebrovascular reactivity in all patients. Neither ictal restriction of the apparent diffusion coefficient nor MR angiography abnormalities were observed. These findings do not support a vascular mechanism in SMART syndrome. Postradiation neuronal dysfunction may be the underlying mechanism. Further investigations on larger series are needed to confirm this hypothesis. PMID:20631504

Farid, Karim; Meissner, Wassilios G; Samier-Foubert, Alexandra; Barret, Olivier; Menegon, Patrice; Rouanet, François; Fernandez, Philippe; Orgogozo, Jean Marc; Allard, Michèle; Tison, François; Sibon, Igor

2010-08-01

184

Family intervention therapy in alcohol dependence syndrome: One-year follow-up study  

PubMed Central

Background: Among the various treatment modalities, family intervention is the most notable current advance in the area of psychosocial treatment of alcoholism. Aim: To assess the impact of family intervention therapy as an adjuvant to pharmacotherapy in alcohol-dependent subjects in a case-control study design. Materials and Methods: Thirty patients who satisfied DSM-IV Criteria for alcohol dependence syndrome were given the right package of family intervention therapy. Thirty age-, sex- and ‘marital status’-matched patients who satisfied the same diagnostic criteria were given only brief supportive psychotherapy. Both groups were assessed at base line, six months and at one year using Michigan Alcohol Screening Test, Motivation Scale, Rotter's Locus of Control, Family Intervention Pattern Scale and Presumptive Stressful Events Scale. Primary efficacy variable was cumulative abstinence duration, and secondary efficacy variables were relapse rate and time to first drink. Results: Family intervention therapy significantly reduced the severity of alcohol intake, improved the motivation to stop alcohol and changed the locus of control from external to internal in the study group. Control group experienced more severe stressful life events than the study group during the follow-up periods. Drop-out rate was comparable in both groups. Conclusion: Combining pharmacological treatment with appropriate psychosocial therapies focusing on the specific problem of the patient provides better outcome than either of these therapies given alone.

Suresh Kumar, P. N.; Thomas, Biju

2007-01-01

185

Antithrombotic therapy in acute coronary syndrome: how far up the coagulation cascade will we go?  

PubMed

The contribution of thrombosis to the natural history and clinical expression of advanced atherosclerotic coronary artery disease is well established. Less well understood is the biochemical and pathobiological distinction between normal hemostasis and thrombosis as the proximate cause of acute coronary syndrome. In this article, we summarize an evolving area of interest within the field of antithrombotic therapy--the contact system and the intrinsic pathway of coagulation, focusing our discussion on factors XI, XII, and IX to include their biochemical properties, relationship to arterial thrombosis phenotypes, and rational for future investigation of targeted pharmacotherapy. PMID:20432072

Woodruff, Becky; Sullenger, Bruce; Becker, Richard C

2010-07-01

186

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

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

1990-01-01

187

[Therapy of Buried Bumper Syndrome via NOTES - A Case Report].  

PubMed

Mucosal overgrowth and migration of the inner bumper into or even through the gastric wall (= buried bumper syndrome) is a possible complication of percutaneous endoscopic gastrostomy (PEG). In this case report we describe the successful endoscopic recovery of an intraabdominally migrated inner bumper as well as reestablishment of PEG using NOTES (Natural Orifice Translumenal Endoscopic Surgery). This practical application of the NOTES technique via the preexisting transgastric approach (PEG) can therefore be a reasonable alternative to a surgical-laparoscopic therapy of the severe buried bumper. PMID:23955140

Nennstiel, S; Schlag, C; Meining, A

2013-08-16

188

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

189

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.

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

190

Long-term results of intravesical hyaluronan therapy in bladder pain syndrome\\/interstitial cystitis  

Microsoft Academic Search

Introduction and hypothesis  While the short-term efficacy of intravesical hyaluronan for bladder pain syndrome\\/interstitial cystitis (BPS\\/IC) has been\\u000a demonstrated, no data exist on the long-term outcome of this therapy.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  Seventy BPS\\/IC patients treated with intravesical hyaluronan therapy from 2001 to 2003 were asked to rate their present status\\u000a of bladder symptoms on a visual analog scale.\\u000a \\u000a \\u000a \\u000a \\u000a Results  Forty-eight of 70 patients responded

Paul F. Engelhardt; Nike Morakis; Lukas K. Daha; Britta Esterbauer; Claus R. Riedl

2011-01-01

191

Thrombotic microangiopathy secondary to steroid pulse therapy administered for refractory nephrotic syndrome.  

PubMed

A 79-year-old woman with familial hyperlipidemia was treated with low-density lipoprotein apheresis. She was hospitalized due to fatigue and edema, and massive proteinuria was discovered. Renal biopsy revealed no distinct abnormalities, thus suggesting a diagnosis of minimal change nephrotic syndrome. She developed acute kidney injury and hemodialysis was initiated. Two series of steroid pulse therapy were given, but the proteinuria did not decrease. Thereafter, she developed thrombocytopenia and fell into a stupor. Thrombotic microangiopathy (TMA) was the most likely diagnosis. Plasma exchange was initiated, resulting in improvements in platelet counts and in her level of consciousness. Clinicians should therefore be aware that TMA can occur as a result of steroid pulse therapy. PMID:24042520

Miyamoto, Tomotsune; Ishikawa, Yozo; Yamamoto, Junya; Yamamura, Tsuyoshi; Kawata, Tetsuya

2013-01-01

192

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

193

Modifications de l'expression des gènes GST-? et p53 dans des lignées tumorales cellulaires humaines O.R.L. après irradiation gamma : induction, études cellulaires et moléculaires  

NASA Astrophysics Data System (ADS)

Cell sub-populations surviving to high radiation doses were selected. The KBm survival part was obtained by exposure to a mutagenic agent and irradiation, FaDum results of a progressive irradiation of FaDu. A semi-quantitative RT-PCR analysis revealed a significant overexpression of GST-? and p53 genes for KBm and FaDum cell lines that remained stable for 18 months. The SF2, ?, ?, and MID parameters, determined by clonogenic assays, show no modifications of radiosensitivity. The variations of expression observed are not correlated to a radiosensitivity variation. The overexpression of GST-? and p53 does not seem to be a radiosensitivity marker. Nous avons isolé des sous-populations de 2 lignées cellulaires humaines (KB et FaDu) de carcinomes des voies aéro-digestives supérieures survivant à de fortes doses d'irradiation. La fraction survivante KBm a été obtenu après exposition à un agent mutagène et à une irradiation, FaDum résulte de l'irradiation progressive de FaDu. Une analyse par RT-PCR semi-quantitative nous a permis de mettre en évidence une surexpression significative des gènes GST-? et p53 pour les souches KBm et FaDum analysées après 18 mois de culture. Les paramètres, ?, ?, SF2, MID, déterminés par essais clonogéniques, n'indiquent pas de modification de la radiosensibilité. Les variations d'expression observées ne sont donc pas corrélées à une variation de radiosensibilité. La surexpression des gènes GST-? et p53 ne semble pas constituer un marqueur de radiosensibilité.

Dubessy, C.; Merlin, J. L.; Marchal, C.

1998-04-01

194

Compartment syndrome of the thigh. A case report with delayed onset after stable pelvic ring fracture and chronic anticoagulation therapie  

PubMed Central

Compartment syndrome of the thigh is a rare occurrence potentially leading to devastating functional restrictions. There is a wide spectrum of reported conditions leading to increased tissue pressure in the thigh possibly resulting in a compartment syndrome, ranging from deep venous thrombosis to blunt injuries and femoral fractures. We report a case of a delayed development of a compartment syndrome of the thigh secondary to an undisplaced anterior pelvic ring fracture and chronic anticoagulation therapy in a 94-year-old woman. Regarding anticoagulation therapy there are numerous reports about the spectrum of bleeding complications during therapy, however this severe complication has to our knowledge not been reported previously. Treatment consisted in immediate fasciotomy and subsequently secondary wound closure.

2010-01-01

195

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

196

The effect of cetylated fatty esters and physical therapy on myofascial pain syndrome of the neck.  

PubMed

Participants with Myofascial Pain Syndrome (MPS) of the neck were randomly assigned into 2 groups of the double-blinded study: topical cetylated fatty ester complex (CFEC) cream application plus physical therapy (CF-PT; n=37), and placebo cream application plus physical therapy (PL-PT; n=35). There were 3 visits during 4 weeks of treatment. Physical Therapy (PT), given twice/week, included Ischaemic Compression, Deep Pressure Trigger Point Massage and Myofascial Releases. Topical cream [CFEC cream (5.6%) and 1.5% menthol] or placebo cream [1.5% menthol, in a cream base] was applied twice/day. CF-PT provided the fastest and most effective study treatment modality. The addition of CFEC cream to PT resulted in statistically significant improvements, compared to PL-PT, for reduction of pain, neck disability and life quality indicators. Our results indicate that cetylated derivatives of fatty acids can effectively reduce pain and symptoms associated with neck MPS, when combined with physical therapy. PMID:21665114

Sharan, Deepak; Jacob, Biju Nirmal; Ajeesh, P S; Bookout, Jack B; Barathur, Raj R

2011-05-31

197

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

PubMed Central

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

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

2013-01-01

198

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

PubMed

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

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

2012-08-08

199

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.

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

2012-01-01

200

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

PubMed

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

2012-10-01

201

Comparison the efficacy of phonophoresis and ultrasound therapy in myofascial pain syndrome.  

PubMed

The aim of this study is to compare the effect of phonophoresis, ultrasound and placebo ultrasound therapies in the treatment of myofascial pain syndrome (MPS). This is a randomized, double-blind placebo controlled study. Sixty patients (48 women, 12 men, mean age 37.9 ± 12.2 years) with MPS were included in this study. Patients were allocated into three groups. Group 1(n = 20) was received diclofenac phonophoresis, group 2(n = 20) was received ultrasound and group 3(n = 20) was received placebo ultrasound therapies over trigger points, 10 min a day for 15 session during 3 weeks (1 MHz-1,5 watt/cm²). Additionally, all patients were given neck exercise program including isotonic, isometric and stretching. Patients were assessed by means of pain, range of motion (ROM) of neck, number of trigger points (NTP), algometric measurement and disability. Pain severity was measured by visual analog scale (VAS) and Likert scale. The neck pain disability index (NPDI) was used for assessing disability. Measurements were taken before and after treatment. After treatment, there were statistically significant improvements in pain severity, NTP, pressure pain threshold (PPT), ROM and NPDI scores both in phonophoresis and in ultrasound therapy groups (P < 0.05). Statistically significant increase in cervical lateral flexion and rotation was observed in the placebo US group. While there was no statistically significant improvement in the cervical flexion-extension joint movement, pain levels, number of trigger points and NPDI score, pressure pain threshold (P > 0.05), also there were no significant differences in all parameters between group 1 and 2 (P = 0.05). Both diclofenac phonophoresis and ultrasound therapy were effective in the treatment of patients with MPS. Phonophoresis was not found to be superior over ultrasound therapy. PMID:20354859

Ay, Saime; Do?an, Sebnem Kolda?; Evcik, Deniz; Ba?er, Ozgün Cakmak

2010-03-31

202

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

Microsoft Academic Search

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

Müberra Kocak; Eray Caliskan; Coskun Simsir; Ali Haberal

2002-01-01

203

Concentrated Ascites Reinfusion Therapy for Sinusoidal Obstructive Syndrome After Hematopoietic Stem Cell Transplantation.  

PubMed

Sinusoidal obstruction syndrome (SOS) is one of the severe complications of hematopoietic stem cell transplantation (HSCT). Systemic management including respiratory and circulatory support is necessary. In addition, abdominal paracentesis is often needed for pain relief and to reduce the pressure of tense ascites. Concentrated ascites reinfusion therapy (CART) involves the filtration, concentration, and reinfusion of drained ascites, which contributes to reuse of autologous proteins. CART has been reported as supportive therapy for patients with liver cirrhosis and cancer. We retrospectively reviewed the efficacy and safety of CART in three patients (two with acute myelogenous leukemia and one with chronic myeloid leukemia) who developed SOS after allo-HSCT. They all had symptomatic, tense, and diuretic-refractory ascites with right costal pain and marked weight gain. Two patients showed immediate improvement after CART. However, one patient experienced four CARTs with slow recovery. All patients are now alive and are being monitored as outpatients over 2 years with remission. No severe adverse event was observed related to CART, and 25.2-98.0 (median 30.2) grams of albumin was collected and reinfused. CART after paracentesis reduces protein loss in ascites by reinfusion of autologous protein instead of exogenous albumin preparations. Although transient fever is reported as a frequent adverse event, no events like severe bleeding or infection were observed. While its safety has not been fully established in patients with hematological disease after HSCT, CART may be a considerable supportive therapy for SOS with tense ascites. PMID:23692354

Takahashi, Hiroyuki; Sakai, Rika; Fujita, Atsuko; Kuwabara, Hideyuki; Hattori, Yukako; Matsuura, Shiro; Ohshima, Rika; Hagihara, Maki; Tomita, Naoto; Ishigatsubo, Yoshiaki; Fujisawa, Shin

2013-05-20

204

Synergistic GABA-enhancing therapy against seizures in a mouse model of Dravet syndrome.  

PubMed

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; Catterall, William A

2013-02-19

205

Progesterone - new therapy in mild carpal tunnel syndrome? Study design of a randomized clinical trial for local therapy  

PubMed Central

Background Local corticosteroid injection for carpal tunnel syndrome (CTS) provides greater clinical improvement in symptoms one month after injection compared to placebo but significant symptom relief beyond one month has not been demonstrated and the relapse of symptoms is possible. Neuroprotection and myelin repair actions of the progesterone was demonstrated in vivo and in vitro study. We report the design of a randomized controlled trial for the local injection of cortisone versus progesterone in "mild" idiopathic CTS. Methods Sixty women with age between 18 and 60 years affected by "mild" idiopathic CTS, diagnosed on the basis of clinical and electrodiagnostic tests, will be enrolled in one centre. The clinical, electrophysiological and ultasonographic findings of the patients will be evaluate at baseline, 1, 6 and 12 months after injection. The major outcome of this study is to determine whether locally-injected progesterone may be more beneficial than cortisone in CTS at clinical levels, tested with symptoms severity self-administered Boston Questionnaire and with visual analogue pain scale. Secondary outcome measures are: duration of experimental therapy; improvement of electrodiagnostic and ultrasonographic anomalies at various follow-up; comparison of the beneficial and harmful effects of the cortisone versus progesterone. Conclusion We have designed a randomized controlled study to show the clinical effectiveness of local progesterone in the most frequent human focal peripheral mononeuropathy and to demonstrate the neuroprotective effects of the progesterone at the level of the peripheral nervous system in humans.

2010-01-01

206

Directed Therapy: An Approach to the Improved Treatment of Exfoliation syndrome  

PubMed Central

Exfoliation syndrome (XFS) is an age-related, generalized disorder of the extracellular matrix characterized by the production and progressive accumulation of a fibrillar extracellular material in many ocular tissues and is the most common identifiable cause of open-angle glaucoma worldwide. Exfoliation syndrome plays an etiologic role in open-angle glaucoma, angle-closure glaucoma, cataract, and retinal vein occlusion. It is accompanied by an increase in serious complications at the time of cataract extraction, such as zonular dialysis, capsular rupture, and vitreous loss. It is associated systemically with an increasing number of vascular disorders, hearing loss, and Alzheimer's disease. Exfoliation syndrome appears to be a disease of elastic tissue microfibrils. Directed therapy simply means devising specific treatments for specific diseases. There was little incentive to attempt to distinguish between various open-angle glaucomas if the treatments were essentially the same. However, this view also prevented the application of directed therapy in those instances in which such was available and applicable. Pilocarpine has multiple beneficial actions in eyes with XFS. Not only does it lower IOP, but by increasing aqueous outflow, it should enable the trabecular meshwork to clear more rapidly, and by limiting pupillary movement, should slow the progression of the disease. Theoretically, miotics should be the first line of treatment. Pilocarpine 2% q.h.s. can provide sufficient limitation of pupillary mobility without causing these side effects. In 2007, two common single nucleotide polymorphisms in the coding region of the lysyl oxidase-like 1 (LOXL1) gene located on chromosome 15 were specifically associated with XFS and XFG. LOXL1 is a member of the lysyl oxidase family of enzymes, which are essential for the formation, stabilization, maintenance, and remodelling of elastic fibers and prevent age-related loss of elasticity of tissues. LOXL1 protein is a major component of exfoliation deposits and appears to play a role in its accumulation and in concomitant elastotic processes in intra- and extraocular tissues of XFS patients. This discovery should open the way to new approaches and directions of therapy for this protean disorder.

Angelilli, Allison; Ritch, Robert

2009-01-01

207

Directed Therapy: An Approach to the Improved Treatment of Exfoliation syndrome.  

PubMed

Exfoliation syndrome (XFS) is an age-related, generalized disorder of the extracellular matrix characterized by the production and progressive accumulation of a fibrillar extracellular material in many ocular tissues and is the most common identifiable cause of open-angle glaucoma worldwide. Exfoliation syndrome plays an etiologic role in open-angle glaucoma, angle-closure glaucoma, cataract, and retinal vein occlusion. It is accompanied by an increase in serious complications at the time of cataract extraction, such as zonular dialysis, capsular rupture, and vitreous loss. It is associated systemically with an increasing number of vascular disorders, hearing loss, and Alzheimer's disease. Exfoliation syndrome appears to be a disease of elastic tissue microfibrils. Directed therapy simply means devising specific treatments for specific diseases. There was little incentive to attempt to distinguish between various open-angle glaucomas if the treatments were essentially the same. However, this view also prevented the application of directed therapy in those instances in which such was available and applicable. Pilocarpine has multiple beneficial actions in eyes with XFS. Not only does it lower IOP, but by increasing aqueous outflow, it should enable the trabecular meshwork to clear more rapidly, and by limiting pupillary movement, should slow the progression of the disease. Theoretically, miotics should be the first line of treatment. Pilocarpine 2% q.h.s. can provide sufficient limitation of pupillary mobility without causing these side effects. In 2007, two common single nucleotide polymorphisms in the coding region of the lysyl oxidase-like 1 (LOXL1) gene located on chromosome 15 were specifically associated with XFS and XFG. LOXL1 is a member of the lysyl oxidase family of enzymes, which are essential for the formation, stabilization, maintenance, and remodelling of elastic fibers and prevent age-related loss of elasticity of tissues. LOXL1 protein is a major component of exfoliation deposits and appears to play a role in its accumulation and in concomitant elastotic processes in intra- and extraocular tissues of XFS patients. This discovery should open the way to new approaches and directions of therapy for this protean disorder. PMID:20142958

Angelilli, Allison; Ritch, Robert

2009-01-01

208

Comparison of adaptive pacing therapy, cognitive behaviour therapy, graded exercise therapy, and specialist medical care for chronic fatigue syndrome (PACE): a randomised trial  

PubMed Central

Summary Background Trial findings show cognitive behaviour therapy (CBT) and graded exercise therapy (GET) can be effective treatments for chronic fatigue syndrome, but patients' organisations have reported that these treatments can be harmful and favour pacing and specialist health care. We aimed to assess effectiveness and safety of all four treatments. Methods In our parallel-group randomised trial, patients meeting Oxford criteria for chronic fatigue syndrome were recruited from six secondary-care clinics in the UK and randomly allocated by computer-generated sequence to receive specialist medical care (SMC) alone or with adaptive pacing therapy (APT), CBT, or GET. Primary outcomes were fatigue (measured by Chalder fatigue questionnaire score) and physical function (measured by short form-36 subscale score) up to 52 weeks after randomisation, and safety was assessed primarily by recording all serious adverse events, including serious adverse reactions to trial treatments. Primary outcomes were rated by participants, who were necessarily unmasked to treatment assignment; the statistician was masked to treatment assignment for the analysis of primary outcomes. We used longitudinal regression models to compare SMC alone with other treatments, APT with CBT, and APT with GET. The final analysis included all participants for whom we had data for primary outcomes. This trial is registered at http://isrctn.org, number ISRCTN54285094. Findings We recruited 641 eligible patients, of whom 160 were assigned to the APT group, 161 to the CBT group, 160 to the GET group, and 160 to the SMC-alone group. Compared with SMC alone, mean fatigue scores at 52 weeks were 3·4 (95% CI 1·8 to 5·0) points lower for CBT (p=0·0001) and 3·2 (1·7 to 4·8) points lower for GET (p=0·0003), but did not differ for APT (0·7 [?0·9 to 2·3] points lower; p=0·38). Compared with SMC alone, mean physical function scores were 7·1 (2·0 to 12·1) points higher for CBT (p=0·0068) and 9·4 (4·4 to 14·4) points higher for GET (p=0·0005), but did not differ for APT (3·4 [?1·6 to 8·4] points lower; p=0·18). Compared with APT, CBT and GET were associated with less fatigue (CBT p=0·0027; GET p=0·0059) and better physical function (CBT p=0·0002; GET p<0·0001). Subgroup analysis of 427 participants meeting international criteria for chronic fatigue syndrome and 329 participants meeting London criteria for myalgic encephalomyelitis yielded equivalent results. Serious adverse reactions were recorded in two (1%) of 159 participants in the APT group, three (2%) of 161 in the CBT group, two (1%) of 160 in the GET group, and two (1%) of 160 in the SMC-alone group. Interpretation CBT and GET can safely be added to SMC to moderately improve outcomes for chronic fatigue syndrome, but APT is not an effective addition. Funding UK Medical Research Council, Department of Health for England, Scottish Chief Scientist Office, Department for Work and Pensions.

White, PD; Goldsmith, KA; Johnson, AL; Potts, L; Walwyn, R; DeCesare, JC; Baber, HL; Burgess, M; Clark, LV; Cox, DL; Bavinton, J; Angus, BJ; Murphy, G; Murphy, M; O'Dowd, H; Wilks, D; McCrone, P; Chalder, T; Sharpe, M

2011-01-01

209

Acute respiratory distress syndrome due to miliary tuberculosis in a patient with rheumatoid arthritis under corticosteroid therapy  

Microsoft Academic Search

We report the case of a 65-year-old woman with rheumatoid arthritis (RA) under corticosteroid therapy who presented with high fever and dyspnea and who, within 48 h, developed acute respiratory distress syndrome (ARDS) due to Mycobacterium tuberculosis (MTB). Miliary tuberculosis is a rare cause of ARDS; however, because of its ominous outcome, it should be included in the differential diagnosis

S. Pavlatos; M. Psallas; K. Makrilakis; J. Doupis; C. Stathi; E. Nikolakopoulou; N. Katsilambros

2004-01-01

210

Massage therapy decreases frequency and intensity of symptoms related to temporomandibular joint syndrome in one case study  

Microsoft Academic Search

Summary Objective: This study investigated the ability of massage therapy to mitigate the frequency and intensity of headaches, jaw clicking and masticatory pain associated with temporomandibular joint syndrome (TMJ). Methods: The subject reported 3 years of masticatory pain, clicking, teeth grinding, reduced jaw opening and headaches prior to the study. A log was kept documenting frequency, intensity and type of

Lindsay Phipps Eisensmith

2007-01-01

211

Does the Heterogeneity of Chronic Fatigue Syndrome Moderate the Response to Cognitive Behaviour Therapy? An Exploratory Study  

Microsoft Academic Search

Background: Chronic fatigue syndrome (CFS) is a heterogeneous condition. A few studies have shown that some independent factors predict outcomes after cognitive behaviour therapy (CBT). Two recent systematic reviews suggest that heterogeneity may moderate treatment outcomes. However, no study has explored whether subgroups of CFS predict response to treatment. Methods: We used both latent class analysis (LCA) and latent class

Matteo Cella; Trudie Chalder; Peter D. White

2011-01-01

212

Rocuronium and sugammadex: An alternative to succinylcholine for electro convulsive therapy in patients with suspected neuroleptic malignant syndrome.  

PubMed

We report a case of presumptive neuroleptic malignant syndrome requiring muscle relaxation for electro-convulsive therapy. short acting muscle relaxation without the use of succinylcholine was achieved using rocvronivm reversed with the novel reversal agent sugammadex. We suggest that this combination is a safe and effective alternative to succinylcholine in such cases. PMID:21897513

Ramamoorthy, Karthik G; Downey, H; Hawthorne, P

2011-07-01

213

Rocuronium and sugammadex: An alternative to succinylcholine for electro convulsive therapy in patients with suspected neuroleptic malignant syndrome  

PubMed Central

We report a case of presumptive neuroleptic malignant syndrome requiring muscle relaxation for electro-convulsive therapy. short acting muscle relaxation without the use of succinylcholine was achieved using rocvronivm reversed with the novel reversal agent sugammadex. We suggest that this combination is a safe and effective alternative to succinylcholine in such cases.

Ramamoorthy, Karthik G.; Downey, H; Hawthorne, P

2011-01-01

214

Therapy-related myelodysplastic syndromes: FAB classification, bone marrow histology, and immunohistology in the prognostic assessment.  

PubMed

In this study we describe the morphologic and immunohistochemical evaluation of bone marrow biopsies from 14 patients with therapy-related myelodysplastic syndromes (t-MDS). We employed CD34, anti-HLA-Dr, anti-elastase, CD68, anti-glycophorin, CD61 monoclonal antibodies immunostaining, and enzyme histochemistry for chloroacetate esterase. Moreover, we used PC10, a MAb raised against the proliferating cell nuclear antigen, to study the proliferative capacity of these marrows. Our data suggest that diagnosis of refractory anemia with excess of blasts (versus chronic myelomonocytic leukemia), the abnormal localization of immature precursors, marrow fibrosis, and augmented CD34 expression in the bone marrow biopsy are ominous prognostic factors at a statistically significant level (p < 0.0005). A combined morpho-immunohistochemical analysis of bone marrow biopsy correctly classifies t-MDS cases according to the biologic and clinical aggressiveness. PMID:7684797

Orazi, A; Cattoretti, G; Soligo, D; Luksch, R; Lambertenghi-Deliliers, G

1993-06-01

215

[Use of ECMO as a salvage therapy for refractory hypoxia secondary to a Lemierre's syndrome].  

PubMed

We reported the case of a young adult with a Lemierre syndrome, which was complicated by septic shock and ARDS. Because of rapid onset of a life-threatening hypoxemia (PaO(2)/FiO(2)=60) in the course of the ARDS despite mechanical ventilation, ECMO was started with jugular and femoral cannulas, as rescue therapy. Good control of hypoxemia was obtained and ECMO was set up for six days. No significant complication was observed. The course was nevertheless complicated by acute renal failure and a pleural fistula that necessitated separate-lung ventilation during 24 hours. After 48 days left in the ICU, the patient was sent to a rehabilitation unit. PMID:21493033

Carre, A-C; David, J-S; Mahr, A; Flamens, C; Fuster, P; Bastien, O

2011-04-13

216

[Defibrotide therapy for patients with sinusoidal obstruction syndrome after hematopoietic stem cell transplantation].  

PubMed

Sinusoidal obstruction syndrome (SOS) is one of the life-threatening complications caused by endothelial damage to the hepatic sinusoids after hematopoietic stem cell transplantation. However, a satisfactory treatment for SOS has not yet been established. Defibrotide has anti-thrombotic, anti-ischemic, anti-inflammatory, and thrombolytic properties without systemic anticoagulant effects. We treated eight post-transplant SOS patients with defibrotide. Three patients responded to the therapy and the initial response was observed within a week. In addition to the improvement of liver function, rapid recovery of response to diuretic drugs followed by the improvement of renal function was observed. All of the five patients with respiratory dysfunction died despite administration of defibrotide, suggesting that early treatment might lead to better outcomes. There were no severe adverse effects directly due to defibrotide administration. Defibrotide seems to be a promising treatment for SOS, and the initiation of a clinical study in Japan would be important. PMID:19225222

Yakushijin, Kimikazu; Okamura, Atsuo; Ono, Kanako; Kawano, Yuko; Kawano, Hiroki; Funakoshi, Yohei; Kawamori, Yuriko; Nishikawa, Shinichiro; Minagawa, Kentaro; Sada, Akiko; Shimoyama, Manabu; Yamamoto, Katsuya; Katayama, Yoshio; Matsui, Toshimitsu

2009-01-01

217

MEDNIK syndrome: a novel defect of copper metabolism treatable by zinc acetate therapy.  

PubMed

MEDNIK syndrome-acronym for mental retardation, enteropathy, deafness, neuropathy, ichthyosis, keratodermia-is caused by AP1S1 gene mutations, encoding ?1A, the small subunit of the adaptor protein 1 complex, which plays a crucial role in clathrin coat assembly and mediates trafficking between trans-Golgi network, endosomes and the plasma membrane. MEDNIK syndrome was first reported in a few French-Canadian families sharing common ancestors, presenting a complex neurocutaneous phenotype, but its pathogenesis is not completely understood. A Sephardic-Jewish patient, carrying a new AP1S1 homozygous mutation, showed severe perturbations of copper metabolism with hypocupremia, hypoceruloplasminemia and liver copper accumulation, along with intrahepatic cholestasis. Zinc acetate treatment strikingly improved clinical conditions, as well as liver copper and bile-acid overload. We evaluated copper-related metabolites and liver function retrospectively in the original French-Canadian patient series. Intracellular copper metabolism and subcellular localization and function of copper pump ATP7A were investigated in patient fibroblasts. Copper metabolism perturbation and hepatopathy were confirmed in all patients. Studies in mutant fibroblasts showed abnormal copper incorporation and retention, reduced expression of copper-dependent enzymes cytochrome-c-oxidase and Cu/Zn superoxide dismutase, and aberrant intracellular trafficking of Menkes protein ATP7A, which normalized after rescue experiments expressing wild-type AP1S1 gene. We solved the pathogenetic mechanism of MEDNIK syndrome, demonstrating that AP1S1 regulates intracellular copper machinery mediated by copper-pump proteins. This multisystem disease is characterized by a unique picture, combining clinical and biochemical signs of both Menkes and Wilson's diseases, in which liver copper overload is treatable by zinc acetate therapy, and can now be listed as a copper metabolism defect in humans. Our results may also contribute to understand the mechanism(s) of intracellular trafficking of copper pumps. PMID:23423674

Martinelli, Diego; Travaglini, Lorena; Drouin, Christian A; Ceballos-Picot, Irene; Rizza, Teresa; Bertini, Enrico; Carrozzo, Rosalba; Petrini, Stefania; de Lonlay, Pascale; El Hachem, Maya; Hubert, Laurence; Montpetit, Alexandre; Torre, Giuliano; Dionisi-Vici, Carlo

2013-02-18

218

Cefepime compared with ceftazidime as initial therapy for serious bacterial infections and sepsis syndrome.  

PubMed Central

In an open randomized multicenter comparative study, we evaluated the safety and efficacy of cefepime (CP; 2.0 g given intravenously every 12 h) and ceftazidime (CZ; 2.0 g given intravenously every 8 h) as initial treatment for adult patients with suspected serious bacterial infections. A total of 133 patients entered the study, of whom 114 were evaluable for clinical and microbiological response assessment: 56 received CP and 58 received CZ. About 50% (30 who received CP and 25 who received CZ) fulfilled the criteria of the sepsis syndrome. The treatment groups were comparable with respect to sex distribution, mean age, underlying diseases, treatment duration, APACHE II score, and type of infection. The most commonly cultured microorganisms were members of the family Enterobacteriaceae, Streptococcus pneumoniae, and Staphylococcus aureus. The causative microorganisms were eradicated from 92% (37 of 40) of patients with a microbiologically documented infection who underwent treatment with CP; they were eradicated from 86% (42 to 49) of patients who received CZ. The responses of only clinically documented infections in the CP group were 90% (27 of 30 patients); in the CZ group they were 87% (26 of 30 patients). When patients fulfilled the criteria of the sepsis syndrome (septic shock excluded), the causative microorganisms were eradicated from 89% (16 of 18) of CP-treated patients and 86% (12 of 14) of CZ-treated patients. None of these differences was statistically significant. Mortality was the same in both groups (four patients in each group) and was not attributable to the study medication. In conclusion, CP is at least as effective and as safe as CZ, as initial antimicrobial therapy for suspected serious bacterial infections in nonneutropenic patients with or without the sepsis syndrome. CP has the additional advantage in that it can be given twice daily, which may lead to a decrease in hospital costs.

Kieft, H; Hoepelman, A I; Rozenberg-Arska, M; Branger, J M; Voskuil, J H; Geers, A B; Kluyver, M; Hart, H C; Poest-Clement, E; van Beugen, L

1994-01-01

219

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

PubMed

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

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

2011-11-11

220

Double-blind randomized controlled trial of low-level laser therapy in carpal tunnel syndrome.  

PubMed

Several studies have suggested that low-level laser therapy (LLLT) is effective in patients with carpal tunnel syndrome (CTS). In a double-blind randomized controlled trial of LLLT, 15 CTS patients, 34 to 67 years of age, were randomly assigned to either the control group (n = 8) or treatment group (n =7). Both groups were treated three times per week for 5 weeks. Those in the treatment group received 860 nm galium/aluminum/arsenide laser at a dosage of 6 J/cm2 over the carpal tunnel, whereas those in the control group were treated with sham laser. The primary outcome measure was the Levine Carpal Tunnel Syndrome Questionnaire, and the secondary outcome measures were electrophysiological data and the Purdue pegboard test. All patients completed the study without adverse effects. There was a significant symptomatic improvement in both the control (P = 0.034) and treatment (P =0.043) groups. However, there was no significant difference in any of the outcome measures between the two groups. Thus, LLLT is no more effective in the reduction of symptoms of CTS than is sham treatment. PMID:15266633

Irvine, Jamie; Chong, Su L; Amirjani, Nasim; Chan, K Ming

2004-08-01

221

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.

Keeler, Allison M.

2012-01-01

222

Shoulder functionality after manual therapy in subjects with shoulder impingement syndrome: a case series.  

PubMed

The aim of the study was to identify the differences in functionality of the upper limb in subjects suffering from shoulder impingement syndrome after intervention by two manual therapy protocols. Randomized, single-blind study with a sample of 22 subjects (58 ± 10.86 years old) divided into two groups. The conventional-group (n = 11) received mobilizations of the shoulder and the experimental-group (n = 11) was treated with soft tissue techniques in the cervical and upper thoracic regions. These two groups received electrotherapy and postural advices. The treatment lasted three weeks (15 daily sessions of 1 h and 30 min). Both active and passive range of motion (ROM) and self-perceived functionality of the upper limb (DASH questionnaire) were measured. The experimental group showed a significant improvement in the DASH scores and both groups improved mobility in the intra-group comparison pre-intervention versus post-intervention (p < .05), but not statistically significant differences were found in the between-group comparison (p > .05). Our results suggest that a combined treatment with electrotherapy, postural hygiene and manual therapy, regardless of the protocol, improves shoulder mobility and functionality. PMID:23561869

Heredia-Rizo, Alberto Marcos; López-Hervás, Antonia; Herrera-Monge, Patricia; Gutiérrez-Leonard, Ana; Piña-Pozo, Fernando

2012-08-04

223

Etanercept therapy for late-onset idiopathic pneumonia syndrome after nonmyeloablative allogeneic hematopoietic stem cell transplantation.  

PubMed

Idiopathic pneumonia syndrome (IPS) is a noninfectious pulmonary complication of allogeneic hematopoietic stem cell transplantation (AHSCT), which usually develops within the first 100 days after transplantation. Donor T-cell-derived tumor necrosis factor-alpha (TNF-alpha) may play a crucial role in the pathogenesis of IPS, and inhibition of TNF-alpha has been used as a therapeutic option. We report two patients who had late-onset IPS about day 150 after nonmyeloablative AHSCT (NMA-AHSCT). They responded well to etanercept in combination with standard immunosuppressive drugs. Both patients had relapses and responded to retreatment with etanercept-based therapy. One patient was alive at 30 months after the initial diagnosis on long-term maintenance therapy with etanercept. The second patient was lost to follow-up at our institution but died 13 months after the onset of IPS. Our two cases showed that IPS could develop late after NMA-AHSCT and inhibition of TNF-alpha activity can be therapeutically effective. PMID:16387152

Tun, H W; Wallace, K H; Grinton, S F; Khoor, A; Burger, C D

2005-12-01

224

Cognitive-behavioral therapy for premenstrual syndrome and premenstrual dysphoric disorder: a systematic review.  

PubMed

We systematically reviewed empirical studies that investigated the use of cognitive-behavioral therapy (CBT) for premenstrual syndrome (PMS) or premenstrual dysphoric disorder (PMDD). Our multi-database search identified seven published empirical reports. Three were identified as randomized controlled trials (RCTs). The methods utilized to investigate therapeutic efficacy of CBT in these studies varied widely from case reports to RCTs with pharmacotherapy comparison groups. Initially we provide a brief overview of CBT and justifications for its potential use to treat PMS/PMDD. Next, we provide critical evaluations of the analyses used in each study focusing on the detection of intervention effects assessed by statistically significant time by group interactions. When possible we calculate effect sizes to elucidate the clinical significance of results. Our review revealed a dearth of evidence providing statistically significant CBT intervention effects. Issues such as overall time investment, latency to treatment effects, and complementary and combined therapies are considered. We present a theoretical argument for applying mindfulness- and acceptance-based CBT interventions to PMS/PMDD and suggest future research in this area. In conclusion, to produce the necessary evidence-base support for PMS/PMDD given the limited empirical evidence reported here, researchers are called on to produce methodologically rigorous investigations of psychosocial interventions for PMS/PMDD. PMID:19247573

Lustyk, M Kathleen B; Gerrish, Winslow G; Shaver, Shelley; Keys, Shaunie L

2009-02-27

225

Injury to the lung from cancer therapy: Clinical syndromes, measurable endpoints, and potential scoring systems  

SciTech Connect

Toxicity of the respiratory system is a common side effect and complication of anticancer therapy that can result in significant morbidity. The range of respiratory compromise can extend from acute lethal events to degrees of chronic pulmonary decompensation, manifesting years after the initial cancer therapy. This review examines the anatomic-histologic background of the lung and the normal functional anatomic unit. The pathophysiology of radiation and chemotherapy induced lung injury is discussed as well as the associated clinical syndromes. Radiation tolerance doses and volumes are assessed in addition to chemotherapy tolerance and risk factors and radiation-chemotherapy interactions. There are a variety of measurable endpoints for detection and screening. Because of the wide range of available quantitative tests, it would seem that the measurement of impaired lung function is possible. The development of staging systems for acute and late toxicity is discussed an a new staging system for Late Effects in Normal Tissues :(LENT) is proposed. 115 refs., 2 figs., 9 tabs.

McDonald, S.; Rubin, P. [Univ. of Rochester Cancer Center, NY (United States); Phillips, T.L. [Univ. of California, San Francisco, CA (United States)] [and other

1995-03-30

226

Practical issues in the management of the long QT syndrome: focus on diagnosis and therapy.  

PubMed

The long QT syndrome (LQTS) is a leading cause of sudden death in the young. It is not as rare as previously assumed, given its established prevalence of 1:2,000 live births. It is characterised by prolongation of the QT interval and by the occurrence of syncope, due to torsades-des-pointes ventricular tachycardia, cardiac arrest and sudden death; these life-threatening cardiac events are usually, but not always, associated with physical or emotional stress. It is a genetic disorder, and knowledge of the genotype impacts significantly on management. Extremely effective therapies are available, which makes the existence of undiagnosed affected and symptomatic patients inexcusable. Indeed, mortality for properly treated patients has now declined to around 1% over a 10-year period. This review, aimed at the clinical cardiologist, discusses briefly the essential genetic information and focuses primarily on the main issues of diagnosis and therapy. One special point of interest is in the impact of genetics on clinical management and the potential medicolegal consequences of not pursuing genetic screening in the proband and hence in the family members. PMID:24089242

Schwartz, P

2013-10-02

227

Medical therapy in women with polycystic ovarian syndrome before and during pregnancy and lactation.  

PubMed

Polycystic ovary syndrome (PCOS) is probably the most common endocrinopathy in women of childbearing age, and is particularly common in African-American and Hispanic ethnic groups. It is characterized by oligo-amenorrhea, clinical and/or biochemical hyperandrogenism, polycystic ovaries, and, often, morbid obesity. PCOS is associated with infertility and frequent 1st trimester miscarriage, and with an increased risk of gestational diabetes. Insulin resistance with compensatory hyperinsulinemia plays an important role in the pathogenesis of PCOS. Reduction of hyperinsulinemia with metformin-diet is associated not only with improvement of the biochemical endocrinopathy, but, commonly, with restoration of menstrual cycles and fertility. The combination of metformin and clomiphene citrate (CC) in CC resistant patients provides additional benefit to a subset of patients, not responsive to metformin alone. Metformin appears to be safe for mothers and neonates (non-teratogenic) during pregnancy, though the results of double-blinded placebo-controlled studies are not yet available. Benefits from metformin therapy during pregnancy include reduction of miscarriage, reduction in likelihood of developing gestational diabetes, reduction in fetal macrosomia, and prevention of excessive maternal weight gain during pregnancy. Rosiglitazone and pioglitazone are effective therapy for ovulation induction, but pregnancy class C and should not be used during pregnancy. PMID:18277353

Goldenberg, N; Glueck, C

2008-02-01

228

Russell Silver syndrome: a perspective on growth and the influence of growth hormone therapy  

PubMed Central

A 6 years male child was referred to our Endocrinology clinic with complaints of failure to thrive and he displayed the characteristic features of Russell Silver Syndrome which included short stature, relative macrocephaly, triangular facies and bilateral clinodactyly. He had a birth weight of 2.14 kg and an expected target height of 170 cm. He was subjected to a hormonal analysis which revealed a normal thyroid profile, but low serum markers of growth namely IGF-1=68 ng/ml (52-297 ng/ml) and basal growth hormone (GH) (1.5 ?g/l). No defects were detected on MRI of the sella. Therefore a growth hormone stimulation test with Clonidine was performed which confirmed complete GH deficiency (at 0 min=0.16 ?g/l, 60 min=0.27 ?g/l, 120 min=4.73 ?g/l). He was commenced on rhGH therapy at 8 years of age (height=102 cm, SDS=-4.53), due to financial restraints. Following initiation of GH therapy (1.5 IU/day) for 19 months, a height gain of 15 cm was obtained (Height=117 cm, SDS=-3.05). Bone age at 9 yr. was between 7-8 years.

Mascarenhas, J. V.; Ayyar, Vageesh S.

2012-01-01

229

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

PubMed Central

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

2009-01-01

230

Static Magnetic Field Therapy for Carpal Tunnel Syndrome: A Feasibility Study  

PubMed Central

Objectives To assess the feasibility of conducting trials of static magnetic field (SMF) therapy for carpal tunnel syndrome (CTS), to collect preliminary data on the effectiveness of two SMF dosages and to explore the influence of a SMF on median nerve conduction. Design Randomized, double blind, sham controlled trial with 6-week intervention and 12-week follow-up. Setting University hospital outpatient clinics Participants Women and men (N=60), ages 21–65, with electrophysiologically-confirmed CTS diagnosis, recruited from the general population. Interventions Participants wore nightly either neodymium magnets that delivered either 15 or 45mTesla (mT) to the contents of the carpal canal, or a non-magnetic disk. Main Outcome Measures Symptom Severity Scale (SSS) and Function Severity Scale (FSS) of the Boston Carpal Tunnel Questionnaire (BCTQ) and 4 median nerve parameters: sensory distal latency, sensory nerve action potential amplitude, motor distal latency and compound motor action potential amplitude). Results 58 of 60 randomized participants completed the study. There were no significant between-group differences for change in the primary endpoint SSS or for FSS or median nerve conduction parameters. For the SSS and the FSS each group showed a reduction at 6-weeks indicating improvement in symptoms. Conclusions This study demonstrated the feasibility and safety of testing SMF therapy for CTS. There were no between-group differences observed for the BCTQ or median nerve parameters following 6 weeks of SMF therapy. Significant within-group, symptomatic improvements of the same magnitude were experienced by participants in both active and sham magnet groups. Future studies are needed to optimize SMF dosimetry and resolve issues related to the use of sham controls in SMF trials.

Colbert, Agatha P.; Markov, Marko S.; Carlson, Nels; Gregory, William L.; Carlson, Hans; Elmer, Patricia J.

2010-01-01

231

[A trial of titration in oral appliance therapy for obstructive sleep apnea syndrome].  

PubMed

This study evaluated the effect of titration in oral appliance therapy for obstructive sleep apnea syndrome (OSAS), and examined problems with this test. However, the method of predicting the appropriate mandible position has not yet been established. In this study, titration was attempted in order to predict appropriate mandible position prior to wearing an oral appliance. Twenty-three male patients diagnosed as OSAS by a physician participated in this study. The mandible was protruded by a titratable splint (TS) until apneic and hypopneic signs had disappeared. Moreover, polisomnography (PSG) was used to monitor brain wave patterns, eye movement, muscle tone, body movement and breathing. Sleep study was performed by a portable sleep monitoring device before and after examination wearing titrated oral appliance (OA), and the effect of therapy was evaluated. The results obtained were as follows. 1. In the Apnea Hypopnea Index (AHI), Apnea Hypopnea density (AH density), lowest SpO2, Oxygen Desaturation Index (ODI), there was a statistically significant improvement. The mean AHI reduced from 13.8 to 4.7 (p<0.001). The mean AH density reduced from 12.0 to 3.5 (p<0.001). The mean lowest SpO2 increased from 78.7 to 84.7 (p<0.0001). The mean ODI reduced from 15.7 to 6.1 (p<0.001). 2.. The average proportion of protrusive distance for movable distance was 71.7%. 3. Only one patient complained of discomfort in the maxillofacial region, however, this discomfort disappeared after adjustment of OA. Therefore, it is suggested that titration for OA is a very useful examination for OSAS therapy. PMID:15301278

Watanabe, Kazunari

2004-06-01

232

Acute compartment syndrome of the thigh following minor trauma in a patient on dual anti-platelet therapy.  

PubMed

Acute compartment syndrome of the thigh is a rare surgical emergency. It is usually associated with high energy trauma and concomitant femoral fracture. We present the case of a 54 year old man who developed acute compartment syndrome of the thigh following a low energy traumatic hamstring tear. Background anti-platelet therapy and hospital prescribed anticoagulants were likely contributors to this pathology. We describe these factors and discuss the issues associated with assessment of minor trauma in the anti-coagulated patient. PMID:21284819

Calabro, Lorenzo J; Dick, Charles G; Lutz, Michael J

2011-02-01

233

Effect of testosterone replacement therapy on bone mineral density in patients with klinefelter syndrome.  

PubMed

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; Seo, Ju Tae

2013-11-01

234

Update on the role of pacemaker therapy in vasovagal syncope and carotid sinus syndrome.  

PubMed

Vasovagal syncope (VVS) and carotid sinus syndrome (CSS) are related, neurally mediated disorders with different clinical features and treatment implications. Pacemaker intervention studies in both syndromes are hampered by a dearth of randomized, controlled (particularly with placebo) studies, unfortunate premature termination of studies, and variation in both the clinical presentation and cardiovascular profile of patients enrolled. Given this relatively limited evidence base, pacing therapy in VVS should be reserved for older sufferers with relatively frequent symptoms and spontaneous asystole documented during real-life monitoring. The long term course of patients paced for vasovagal syncope needs clarification, both in terms of symptom and device-related burden, while a comprehensive health economic evaluation of the costs and benefits of such an invasive approach for this usually benign condition is essential. It will also be important to determine the efficacy of closed loop stimulation pacing in an adequately powered study, including a comparison with standard pacing. Pacing should be considered in those with unexplained syncope with reproduction of symptoms during a cardio-inhibitory or mixed response to carotid sinus massage (asystole >3 s), though the evidence base for this recommendation is firmly in the consensus rather than pragmatic randomized controlled trial camp. Patients presenting with unexplained falls and drop attacks, particularly where injuries are prominent and where patients cannot give a clear account of their fall ("I just ended up on the floor, didn't slip or trip"), should also be considered for pacing. Pacing is not recommended for the vasodepressor sub-type of CSS. PMID:23472782

Parry, Steve W; Matthews, Iain G

235

Association of statin therapy with ventricular arrhythmias among patients with acute coronary syndrome  

PubMed Central

Background In addition to lowering cholesterol, statins stabilise atherosclerotic plaques and can potentially reduce the incidence of ventricular arrhythmias. We tested the hypothesis that prior statin therapy is associated with a lower incidence of inhospital ventricular arrhythmias among patients with acute coronary syndrome (ACS). Methods The study population consisted of 2007 patients (mean age 64?years, 67.5% male) enrolled in the Thai Registry of Acute Coronary Syndrome, a prospective, multicentre, nationwide, observational study of patients with ACS. Patients were categorised as either statin users or non-users according to their reports of statin use before enrolment at their initial presentation. The primary endpoint was inhospital ventricular arrhythmias. The secondary endpoint was a composite endpoint of inhospital ventricular arrhythmias or inhospital cardiac death. A propensity-adjusted multivariate model was developed to assess the effects of statin use on the primary and secondary endpoints. Results During a mean hospital stay of 7?days, a total of 96 patients (4.8%) died; 82 (4.1%) of the deaths were due to cardiac causes. The primary and secondary endpoints were reached in 163 patients (8.1%) and 194 patients (9.7%), respectively. A total of 525 patients (26.2%) had used statins prior to hospitalisation. After adjusting for the propensity scores and other relevant covariates, statin use was associated with lower risks of the primary (adjusted OR 0.505, 95% CI 0.276 to 0.923) and secondary endpoints (adjusted OR 0.498, 95% CI 0.276 to 0.897). Conclusions The use of statins is associated with a reduced incidence of ventricular arrhythmias among patients with ACS.

Apiyasawat, Sirin; Sritara, Piyamitr; Ngarmukos, Tachapong; Sriratanasathavorn, Charn; Kasemsuwan, Piya

2013-01-01

236

Frequency of impulse control behaviours associated with dopaminergic therapy in restless legs syndrome  

PubMed Central

Background Low doses of dopamine agonists (DA) and levodopa are effective in the treatment of restless legs syndrome (RLS). A range of impulse control and compulsive behaviours (ICBs) have been reported following the use of DAs and levodopa in patients with Parkinson's disease. With this study we sought to assess the cross-sectional prevalence of impulse control behaviours (ICBs) in restless legs syndrome (RLS) and to determine factors associated with ICBs in a population cohort in Germany. Methods Several questionnaires based on validated and previously used instruments for assessment of ICBs were mailed out to patients being treated for RLS. Final diagnoses of ICBs were based on stringent diagnostic criteria after psychiatric interviews were performed. Results 10/140 RLS patients of a clinical cohort (7.1%) were finally diagnosed with ICBs, 8 of 10 on dopamine agonist (DA) therapy, 2 of 10 on levodopa. 8 of the 10 affected patients showed more than one type of abnormal behaviour. Among those who responded to the questionnaires 6/140 [4.3%] revealed binge eating, 5/140 [3.6%] compulsive shopping, 3/140 [2.1%] pathological gambling, 3/140 [2.1%] punding, and 2/140 [1.4%] hypersexuality in psychiatric assessments. Among those who did not respond to questionnaires, 32 were randomly selected and interviewed: only 1 patient showed positive criteria of ICBs with compulsive shopping and binge eating. ICBs were associated with higher DA dose (p = 0.001), younger RLS onset (p = 0.04), history of experimental drug use (p = 0.002), female gender (p = 0.04) and a family history of gambling disorders (p = 0.02), which accounted for 52% of the risk variance. Conclusion RLS patients treated with dopaminergic agents and dopamine agonists in particular, should be forewarned of potential side effects. A careful history of risk factors should be taken.

2011-01-01

237

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.

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

2013-01-01

238

Immediate effect of intensive atorvastatin therapy on lipid parameters in patients with acute coronary syndrome  

PubMed Central

Background Intensive statin therapy decreases mortality and incidence of coronary events in patients after acute coronary syndrome (ACS). Recently it has been reported that spontaneous lipid levels remain clinically stable during ACS. The immediate influence of lipid levels by high-dose statin therapy initiated at admission in ACS patients is, however, not clear. Methods We have analyzed a group of 114 patients with ACS (mean age 63.7; females 25.4%). Atorvastatin 80 mg was administered at admission and then once daily for the rest of hospitalization. The levels of total cholesterol (TC), LDL-cholesterol (LDL), HDL-cholesterol (HDL), and triglycerides (TG) were measured at admission (D0), and then every morning of hospitalization (D1, D2). Results The mean entry values (D0) of TC, LDL, HDL and TG (in mmol/L) were 5.24, 3.26, 1.07 and 1.31, respectively. The therapy with atorvastatin 80 mg resulted in a decrease of TC levels in the first morning (D1) by 6.1% and in the second morning (D2) by 13.2% (p < 0.001 for all comparisons with the entry value D0); LDL was decreased by 5.8% (D1) and 15.6% (D2) (p < 0.001 vs. D0); the level of HDL was decreased by 7.5% (D1) and 12.1% (D2) (p < 0.001 vs. D0). In contrast, the TG level was higher in the first morning (D1) by 20.6% and in the following morning (D2) by 25.5% (p < 0.05 vs. D0). Conclusions We have shown that intensive statin therapy started at admission in ACS patients has a highly significant, immediate effect on all monitored lipid levels. Since TC and LDL levels were decreased as predicted, reduction in HDL and increase in TG levels suggest a different acute effect of high-dose statin on lipid levels in comparison with long-term treatment of ACS patients.

2010-01-01

239

Complementary and alternative medicine and mind-body therapies for treatment of irritable bowel syndrome in women.  

PubMed

Irritable bowel syndrome (IBS) is a common gastrointestinal disorder, characterized by chronic or recurrent abdominal pain with constipation, diarrhea and/or an alternation of the two, and often bloating. Complementary and alternative medicine (CAM) consists of a group of medical treatments that are not commonly considered to be a part of traditional medicine. CAM is commonly used for difficult-to-treat chronic medical conditions. Many patients choose CAM because there are only a limited number of treatments available for IBS or because they would like to have a 'natural therapy'. Mind-body therapies for IBS have proven efficacy, but have not been well accepted by patients or practitioners for treatment. This article reviews the use of CAM and mind-body therapies in IBS, with a focus on probiotics, acupuncture, herbal medicines and psychological therapies. PMID:24161308

Magge, Suma S; Wolf, Jacqueline L

2013-11-01

240

A randomized, 48-week, placebo-controlled trial of intensive lifestyle modification and\\/or metformin therapy in overweight women with polycystic ovary syndrome: A pilot study  

Microsoft Academic Search

ObjectiveTo obtain data from a pilot randomized trial on the effect of metformin therapy and lifestyle modification on ovulation and androgen concentrations in women with polycystic ovary syndrome (PCOS).

Kathleen M Hoeger; Lynda Kochman; Nellie Wixom; Kimberly Craig; Richard K Miller; David S Guzick

2004-01-01

241

Modafinil zur Behandlung exzessiver Tagesschläfrigkeit trotz nCPAP-Therapie beim obstruktiven Schlafapnoe-Syndrom (OSAS) – Ergebnisse einer multizentrischen Beobachtungsstudie  

Microsoft Academic Search

\\u000a \\u000a Summary\\u000a \\u000a \\u000a Introduction\\u000a   Despite optimal therapy of obstructions and exclusion of other causes approximately 10% of patients with obstructive sleep\\u000a apnea syndrome (OSAS) suffer from residual sleepiness. Efficacy and tolerability of a symptomatic therapy with modafinil in\\u000a patients with OSAS and persisting daytime sleepiness despite optimised nasal continuous positive airway pressure (nCPAP) treatment\\u000a were documented in a non-interventional multicentre study.\\u000a \\u000a \\u000a \\u000a \\u000a Patients

Martin Konermann; Hartmut Grüger; Ralf Warmuth

2007-01-01

242

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

2012-10-11

243

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

PubMed

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

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

2012-10-25

244

Effect of oestrogen\\/gestagen replacement therapy on liver enzymes in patients with Ullrich-Turner Syndrome  

Microsoft Academic Search

The absence of breast development and the prevention of osteoporosis in Ullrich-Turner syndrome (UTS) require oestrogen\\/gestagen substitution therapy. In 8 out of 35 (23%) patients with UTS treated with conjugated equine oestrogens and cyclically with norethisterone acetate, the serum liver enzymes increased to conspicuous levels (AST 35; 20–73 U\\/l, ALT 92; 37–141 U\\/l, GGT 77; 25–227 U\\/l, [median; min-max]). These

H. Wemme; J. Pohlenz; W. Schönberger

1995-01-01

245

Tropical Splenomegaly Syndrome: Long-term Proguanil Therapy Correlated with Spleen Size, Serum IgM, and Lymphocyte Transformation  

Microsoft Academic Search

Forty-three patients with an initial diagnosis of tropical splenomegaly syndrome were placed on long-term proguanil therapy. All patients who failed to respond to proguanil and who were adequately followed up developed identifiable disease, usually malignant lymphoma or chronic lymphatic leukaemia. In patients who responded to proguanil IgM values were always very high and phytohaemagglutinin (P.H.A.)-lymphocyte-transformation scores were always normal before

Aba-Segua Sagoe

1970-01-01

246

Diagnosis and therapy of polycystic ovarian syndrome: results of a survey among German gynecologists with a review on literature  

Microsoft Academic Search

Objective  Polycystic ovarian syndrome is one of the most frequent endocrine dysfunctions in women in their fertile age. To date, a clear\\u000a definition of the disease remains controversial among experts, partly because study results concerning diagnostics and therapy\\u000a are incoherent. In this survey, we intend to give an insight into the diagnostics and treatment regimes of PCOS in Germany\\u000a and subsequently

Ines Doll; Richard Doll; Kai J. Buhling

247

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

Microsoft Academic Search

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

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

1998-01-01

248

Consensus recommendations for the treatment of basal cell carcinomas in Gorlin syndrome with topical methylaminolaevulinate-photodynamic therapy.  

PubMed

BACKGROUND: Patients with Gorlin syndrome develop multiple basal cell carcinomas (BCC), for which treatment is often difficult. Methylaminolevulinate-photodynamic therapy (MAL-PDT) is approved for the treatment of superficial and nodular BCCs in Canada and several European countries. OBJECTIVES: To establish consensus recommendations for the use of MAL-PDT in patients with Gorlin syndrome. METHODS: The Gorlin consensus panel was comprised of 7 dermatologists who had treated a total of 83 patients with Gorlin syndrome using MAL-PDT. Consensus was developed based on the personal experience of the expert and results of literature review (on PUBMED using the keywords 'MAL' and 'PDT' and 'Gorlin' or 'naevoid basal cell carcinoma syndrome'). RESULTS: Consensus was reached among the experts and the literature review identified 9 relevant reports. The experts considered MAL-PDT a generally effective and safe therapy for treatment of BCC in Gorlin syndrome. For superficial BCC (sBCC), all sizes can be treated, and in nodular BCC (nBCC), better efficacy can be achieved in thinner lesions (<2 mm in thickness). MAL-PDT treatment schedule should be performed according to labelling although in individual cases, it may be adapted and performed on a monthly basis based on clinical assessment. Follow-up should be related to frequency of recurrence, and severity, number and location of lesions. Multiple lesions and large areas may be treated during the same session; however, adequate pain management should be considered. CONCLUSIONS: MAL-PDT is safe and effective in patients with Gorlin syndrome. Utilization of these recommendations may improve efficacy and clearance rates in this population. PMID:23581795

Basset-Seguin, N; Bissonnette, R; Girard, C; Haedersdal, M; Lear, J T; Paul, C; Piaserico, S

2013-04-13

249

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

250

Advances in pathogenesis and therapy of hemolytic uremic syndrome caused by shiga toxin-2.  

PubMed

Shiga toxin (Stx) producing Escherichia coli (STEC) is responsible to bloody diarrhea (hemorrhagic colitis) and the hemolytic uremic syndrome (HUS). STEC strains carry inducible lambda phages integrated into their genomes that encode Stx 1 and/or 2, with several allelic variants each one. O157:H7 is the serotype that was documented in the vast majority of HUS cases although non-O157 serotypes have been increasingly reported to account for HUS cases. However, the outbreak that occurred in central Europe during late spring of 2011 showed that the pathogen was E. coli O104:H4. More than 4,000 persons were infected mainly in Germany, and it produced more than 900 cases of HUS resulting in 54 deaths. E. coli O104:H4 is a hybrid organism that combines some of the virulence genes of STEC and enteroaggregative E. coli specially production of Stx2 and the adherence mechanisms to intestinal epithelium. The differences in the epidemiology and presentation of E. coli pathogen meant a challenge for public health and scientific research to increase the knowledge of HUS-pathophysiology and to improve available therapies to treat HUS. © 2013 IUBMB Life, 65(10):827-835, 2013. PMID:24014500

Ibarra, Cristina; Amaral, María Marta; Palermo, Marina S

2013-09-06

251

[Bacterial overgrowth syndrome in the small intestine: pathogenesis, clinical significance and therapy tactics].  

PubMed

The redundant bacterial growth syndrome in the small intestine is associated with the increased total semination of over 10(5) CFU/ml presented by enterobacteria, bacteroids, clostridia, fusobacteria, etc. It is developed at the dysfunction of the gastrointestinal tract, insufficient bacteria inhibition at the time when they come from the large intestine (atony, stasis, bypasses) and is accompanied by the enhanced intestinal barrier permeability along with chronic diarrhea and intoxication. Intestinal absorption disorders cause B12-deficiency anemia, hypovitaminosis and protein deficiency. The redundant growth is diagnosed based on the hydrogen concentration in the expired air or bacterial inoculation of the small intestine aspirate. Tetracycline, Vancomycin, Metronidazole and aminoglycoside are used for the therapy; Amoxicillin/clavunate and cephalosporins of the second generation are also applied with success. Decontamination of the small intestine is more successful when probiotics are prescribed (both after antibiotics and independently), which suppress the opportunistic flora, protect the mucous coat, improve digestion and arrest diarrhea. Probifor or Bifidumbacterin forte in the complex with probiotics comprising lactobacteria can also have a therapeutic effect. PMID:17378388

Lykova, E A; Bondarenko, V M; Parfenov, A I; Matsulevich, T V

2005-01-01

252

The role of intrathecal baclofen therapy in the upper motor neuron syndrome.  

PubMed

This review article will discuss the application of intrathecal baclofen (ITB) therapy in the upper motor neuron syndrome (UMNS). While the UMNS consists of a variety of signs and symptoms, spasticity appears to be the most widely discussed in research and clinical practice. Thus, while a variety of motor disorders result from spasticity and the other components of the UMNS, such as dystonia, rigidity, and co-contraction of agonists and antagonists, we will refer to spasticity as the representative pathology of the UMNS. The term spasticity will be used in this discussion as if it were synonymous to the UMNS, because it is the term used in most published research and papers. This does not necessarily mean that the other features of the UMNS are less important. Publications in the use of ITB in the pediatric population, especially cerebral palsy, abound, but this paper will focus on UMNS in adults. In addition to reviewing the process of ITB management, from patient selection to rehabilitation, topics of practical interest to clinicians will be discussed. PMID:16046935

Francisco, G E

2004-06-01

253

Atypical hemolytic uremic syndrome: From the rediscovery of complement to targeted therapy.  

PubMed

Atypical hemolytic uremic syndrome (aHUS) is a devastating form of renal thrombotic microangiopathy. In the last five years, we have finally witnessed a dramatic improvement in the management of aHUS patients, and three breakthroughs in our understanding of aHUS have led to such an improvement. The first breakthrough was the emergence of a new clinical picture of aHUS (frequency of adult cases, and overall poor renal prognosis despite plasma therapy). The second breakthrough was the identification of complement alternative pathway dysregulation as a major risk factor for aHUS. The third breakthrough was the availability in clinical practice of the first complement inhibitor, the anti-C5 monoclonal antibody, eculizumab. Available data from case series and prospective studies indicate that eculizumab use has dramatically improved the renal prognosis of aHUS. These breakthroughs have prompted the French working group on aHUS to propose a new algorithm for the management of aHUS in children and in adults. This algorithm will evolve as we gain new insights in the pathogenesis and evolution of aHUS in the eculizumab era. PMID:23756030

Fakhouri, Fadi; Frémeaux-Bacchi, Véronique; Loirat, Chantal

2013-06-05

254

A comparison of immunomodulation therapies in mechanically ventilated patients with Guillain Barré syndrome.  

PubMed

A comparison of the effectiveness of immunomodulatory therapies in patients with Guillain Barré syndrome (GBS) who require mechanical ventilation (MV) is important for patient treatment and cost. We aimed to compare the effectiveness of three modes of intervention on the outcome of patients with GBS receiving MV: intravenous immunoglobulin (IVIgG); small volume plasmapheresis (SVP) and large volume plasmapheresis (LVP). Patients with GBS satisfying National Institute of Neurological and Communicative Disorders and Stroke 1990 criteria and requiring MV between 1997 between 2007 were analyzed. The primary outcome parameters evaluated were mortality, duration of MV, hospital stay and Hughes scale at discharge from hospital. Of the 173 (Male: Female, 118:55) patients who required MV during the study, 106 patients received single modality treatment (IVIgG 31, LVP 45, SVP 30) based on availability, affordability and feasibility. Patients receiving IVIgG had a higher incidence of severe weakness and bulbar involvement. The mean duration of MV (p=0.61), total hospital stay (p=0.44) and Hughes scale at discharge (p=0.31) did not differ among the three groups. Complications were similar in the three treatment groups except for hypoalbuminemia and anemia, which were more common in patients in the LVP group. In conclusion, the outcome of patients treated with these three immunomodulatory treatment modalities did not vary. The beneficial effects of SVP in our study warrant further randomized control trials especially in resource-constrained settings. PMID:23036171

Netto, Archana B; Kulkarni, Girish Baburao; Taly, Arun B; Rao, Gs Umamaheshwara; Periyavan, Sunder; Rao, Shivaji

2012-10-02

255

Interleukin-targeted therapy for metabolic syndrome and type 2 diabetes.  

PubMed

Interleukin-1? Interleukin-1? (IL-1?) is a key regulator of the body's inflammatory response and is produced after infection, injury, and an antigenic challenge. Cloned in 1984, the single polypeptide IL-1? has been shown to exert numerous biological effects. It plays a role in various diseases, including autoimmune diseases such as rheumatoid arthritis, inflammatory bowel diseases, and Type 1 diabetes, as well as in diseases associated with metabolic syndrome such as atherosclerosis, chronic heart failure, and Type 2 diabetes. The macrophage is the primary source of IL-1?, but epidermal, epithelial, lymphoid, and vascular tissues also synthesize IL-1. Recently, IL-1? production and secretion have also been reported from pancreatic islets. Insulin-producing ?-cells ?-cells within the pancreatic islets are specifically prone to IL-?-induced destruction and loss of function. Macrophage-derived IL-1? production in insulin-sensitive organs leads to the progression of inflammation inflammation and induction of insulin resistance in obesity. This chapter explains the mechanisms involved in the inflammatory response during diabetes progression with specific attention to the IL-1? signal effects influencing insulin action and insulin secretion insulin secretion . We highlight recent clinical studies, rodent and in vitro experiments with isolated islets using IL-1? as a potential target for the therapy of Type 2 diabetes. PMID:21484575

Maedler, Kathrin; Dharmadhikari, Gitanjali; Schumann, Desiree M; Størling, Joachim

2011-01-01

256

Reversible posterior leukoencephalopathy syndrome during sunitinib therapy for metastatic renal cell carcinoma.  

PubMed

Sunitinib is an oral receptor tyrosine kinase inhibitor with potent antiangiogenic and antitumor activity that is approved for the treatment of advanced renal cell carcinoma (RCC), malignant gastrointestinal stromal tumors and pancreatic neuroendocrine tumors. Well-known side effects of sunitinib include hypertension, fatigue, thyroid dysfunction, cardiotoxicity, gastrointestinal toxicity and skin toxicity. In this study, we report the case of a 61-year-old male with papillary metastatic RCC who responded to sunitinib but developed generalized tonic-clonic seizures during the third cycle. Magnetic resonance imaging (MRI) was compatible with reversible posterior leukoencephalopathy syndrome (RPLS). After the administration of anti-epileptic drugs and the withdrawal of sunitinib there was rapid clinical improvement. Notably, radiological characteristics of RPLS persisted during second-line therapy with the mammalian target of rapamycin (mTOR) inhibitor everolimus and only resolved when everolimus was terminated due to disease progression. Although sunitinib-induced RPLS has been reported previously, our case is the first to additionally suggest that everolimus may sustain and therefore potentially contribute to the occurrence of RPLS. PMID:22783436

Hadj, Jamal Oulad; Braven, Rogier DEN; Tillier, Corrine; Schrijver, Hans M; Verheul, Henk M W; VAN DER Vliet, Hans J

2012-03-16

257

Hepatopathy-thrombocytopenia syndrome after actinomycin-d therapy: treatment with defibrotide.  

PubMed

We report a case of administration compassionate use defibrotide in a 13-year-old girl with Sinusoidal Obstructive Syndrome and thrombocytopenia, also known as Hepatopathy--Thrombocytopenia Syndrome (HTS) during chemotherapy for Wilms' tumor. PMID:23216129

Martín-Lázaro, Juan F; Palanca, Daniel; Garcia-Iñiguez, Juan P; Madurga, Paula; Carboné, Ana

2012-12-05

258

[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

259

Failure of antioxidant therapy to attenuate interstitial disease in rats with reversible nephrotic syndrome.  

PubMed

The present two studies were designed to determine whether oxidized LDL contributes to the tubulointerstitial changes seen in rats during the acute phase of acute puromycin aminonucleoside nephrosis (PAN). In the single-dose study, rats were given one injection of puromycin aminonucleoside (PA; 15 mg/100 g body wt) and killed 1, 2, or 3 wk thereafter. The four animal groups were saline controls, PAN controls, PAN plus probucol, and PAN plus lovastatin. This study showed that the addition of probucol significantly reduced the mean levels of serum cholesterol and renal lipid-peroxidation products, an effect not seen with lovastatin therapy. Compared with saline controls, PAN controls had a significant increase in total kidney collagen (7.9 +/- 1.2 versus 5.9 +/- 0.6 mg/kidney at 3 wk). Neither probucol nor lovastatin therapy attenuated the interstitial inflammation or fibrosis. In the multidose study, rats were given the same initial PA dose and were uninephrectomized on day 12. They were killed on day 35 after two smaller PA doses were given on days 16 and 23. Animal groups were saline controls, PAN controls, PAN plus probucol, and PAN plus vitamin E. Hepatic lipid-peroxidation products were significantly lower in the probucol-treated, but not in the vitamin E-treated, PAN groups when compared with the PAN controls. Neither probucol nor vitamin E prevented the increase in total kidney collagen that was observed in the PAN control group (7.4 +/- 0.7, 10.1 +/- 2.6, and 9.3 +/- 1.8 mg of collagen/kidney, respectively, versus 5.4 +/- 0.5 mg/kidney for the saline controls). Renal cortical mRNA levels for matrix-encoding genes and protease inhibitors were similar in the three nephrotic groups. Transforming growth factor-beta1 mRNA levels were highly variable within each group and not significantly different at day 35, but showed a significant positive correlation with the degree of albuminuria (r = 0.70). The present results demonstrate that the treatment of acutely nephrotic rats with antioxidant therapy did not attenuate interstitial inflammation or fibrosis. We speculate that other factors, possibly a consequence of proteinuria itself, are the predominant pathogenetic mediators of the tubulointerstitial damage in acute nephrotic syndrome. PMID:9527400

Drukker, A; Eddy, A A

1998-02-01

260

Anticonvulsant hypersensitivity syndrome associated with bellamine s, a therapy for menopausal symptoms  

Microsoft Academic Search

Anticonvulsant hypersensitivity syndrome (AHS) is a rare, potentially fatal, idiosyncratic drug reaction characterized by fever, morbilliform rash, lymphadenopathy, hepatitis, and hematologic abnormalities. Aromatic antiepileptic agents, such as phenytoin, carbamazepine, and phenobarbital are the most frequent causes of this syndrome. We report a case of a previously healthy, postmenopausal woman who developed anticonvulsant hypersensitivity syndrome while taking Bellamine S (belladonna alkaloids;

Ryan B Turner; Caroline Choi Kim; Keliegh Culpepper; Harley A Haynes

2004-01-01

261

[Double antiaggregant therapy in patients with acute coronary syndrome undergoing coronary angioplasty revascularization. Pending clinical problems and effects of therapy non-compliance].  

PubMed

Acute coronary syndromes (ACS) are the most frequent cause of hospitalization in intensive cardiac care units and are associated with a high risk of early complications. As a consequence, medical attention and healthcare are mainly directed towards the treatment of the acute phase of ACS, when the risk is higher. However, the risk associated with ACS is not simply confined to the hospitalization period, but may persist for months or years because of the frequent recurrence of ischemic events and related outcomes. Although more aggressive invasive strategies and powerful therapies with anticoagulants and antiplatelet agents have strongly improved the acute prognosis of ACS, the prognosis still remains unfavorable. Notwithstanding this, cardiologists mostly focus on the acute phase of ACS, while adopting a less aggressive approach after patient discharge and during outpatient follow-up. However, secondary prevention measures are as important as acute therapy and should be largely applied, including accurate estimation of the residual risk for the individual patient, appropriate implementation of secondary prevention, and close monitoring of adherence to suggested treatments, such as dual antiplatelet therapy. In conclusion, adherence to therapy is a clinically relevant factor, and efforts towards its improvement should be actively pursued. Moreover, appropriate prescriptions may stimulate therapy adherence with favorable effects, resulting in better clinical outcomes and healthcare cost containment. This holds particularly true for patients with ACS undergoing percutaneous revascularization. PMID:21563433

Casella, Gianni; De Servi, Stefano; Tubaro, Marco; Cavallini, Claudio; Andreotti, Felicita; Olivari, Zoran; Bolognese, Leonardo; Di Pasquale, Giuseppe

2011-02-01

262

Potential of Advexin: a p53 gene-replacement therapy in Li-Fraumeni syndrome.  

PubMed

Li-Fraumeni syndrome is a rare autosomal dominant cancer predisposition syndrome. The majority of families fulfilling definition of Li-Fraumeni syndrome demonstrate inherited abnormalities involving the p53 gene. Cells with dysfunctional p53 are predisposed to the development of cancer phenotype. Advexin (Introgen Therapeutics Inc., TX, USA) is an adenoviral-based experimental therapeutic that provides delivery of wild-type p53 to cancer cells and demonstrates anticancer activity following adequate expression of p53. Theoretically, correction of p53 function in cancer developing in patients with Li-Fraumeni syndrome through treatment with Advexin will provide anti-tumor activity. One patient with Li-Fraumeni syndrome has been reported to have responded to Advexin. This review will summarize background knowledge of Li-Fraumeni syndrome, mechanisms of Advexin and clinical response of cancer to Advexin with a focus on Li-Fraumeni syndrome. PMID:19086841

Nemunaitis, Jackie M; Nemunaitis, John

2008-12-01

263

A case of chronic hepatitis C developing insulin-dependent diabetes, thyroid autoimmunity and stiff-person syndrome as complications of interferon therapy  

Microsoft Academic Search

We describe the case of a 66-year-old man with chronic hepatitis C who developed type 1 diabetes mellitus (T1DM) and thyroid autoimmunity during Interferon ? (INF?) therapy and then stiff-person syndrome (SPS). This is the first reported case in which SPS has appeared as complication of IFN? therapy.

G. Scavone; F. Zaccardi; A. Manto; S. Caputo; D. Pitocco; G. Ghirlanda

2010-01-01

264

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

265

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

PubMed

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

Fey, K; Jonigkeit, E; Moritz, A

1998-02-01

266

The long QT syndrome: a transatlantic clinical approach to diagnosis and therapy.  

PubMed

The mind-boggling progress in the understanding of the molecular mechanisms underlying the long QT syndrome (LQTS) has been the subject of many articles and reviews. Still, when it comes to the management of the patients affected by this life-threatening disorder, too many errors still take place, both in the diagnostic process and in the therapeutic choices. The price of these errors is paid by the patients and their families. This review is not directed to the relatively small number of LQTS experts who know what to do. It does not deal with genetics, with epidemiology, or with the well-known clinical manifestations. We have focused solely on the approach to diagnosis and therapy and we have directed this review to the average clinical cardiologist who, in his/her practice, sees occasionally patients affected or suspected to be affected by LQTS; the cardiologist who may know enough to manage them but not enough to be completely confident on his/her most critical choices. We have provided our personal views without making any attempt to blend differences whenever present. On most issues we agree fully but where we do not, we make it clear to the reader by indicating who is thinking what. The result may be unconventional, but it mirrors the challenges, often severe, that we all face in managing and protecting these patients from sudden death while also helping them live and thrive despite their diagnosis. We trust that this unabashed presentation of our clinical approach will be useful for both cardiologists and patients. PMID:23509228

Schwartz, Peter J; Ackerman, Michael J

2013-03-18

267

Guideline adherence for antithrombotic therapy in acute coronary syndrome: an overview in Dutch hospitals  

PubMed Central

Objective To assess current Dutch antithrombotic treatment strategies for acute coronary syndrome (ACS) in light of the current European Society of Cardiology (ESC) guidelines. Methods For every Dutch hospital with a coronary care unit (CCU) (n = 93) a single cardiologist was interviewed concerning heparin, thienopyridine and GP IIb/IIIa inhibitor (GPI) treatment. In each hospital, we randomly approached one cardiologist assuming equal policy among physicians employed at the same hospital. Results The response rate was 90%. In 59% of hospitals, treatment of ST-elevation myocardial infarction (STEMI) occurred according to the 2008 ESC STEMI guideline, with unfractionated heparin. In contrast, although not recommended, low-molecular-weight heparin (LMWH) was used in 39% (enoxaparin 19%, dalteparin 12%, nadroparin 8%). In non-STEMI, low-molecular-weight-heparins (LMWHs) were used in 97% of all hospitals. Fondaparinux, agent of choice in a noninvasive strategy for the treatment of non-STEMI, was applied in only 2% of hospitals. Although recommended by the ESC, dose adjustment of LMWH therapy for patients with renal failure is not applied in 71% of hospitals. Likewise, LMWH dose adjustment is not applied for patients aged over 75 years in 92% of hospitals. Conclusion To a great extent treatment of ACS in the Netherlands occurs according to ESC guidelines. Additional benefit may be achieved by routine dose adjustment of LMWH for patients with renal insufficiency and aged >75 years, since these patients are at high risk of bleeding complications secondary to antithrombotic treatment. Periodical evaluation of real-life practice may improve guideline adherence and potentially improve clinical outcome. (Neth Heart J 2010;18:291–9.)

Kikkert, W.J.; Piek, J.J.; de Winter, R.J.; Peters, R.J.; Henriques, J.P.S.

2010-01-01

268

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.

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

2010-01-01

269

Effect of nicotine replacement therapy on cardiovascular outcomes after acute coronary syndromes.  

PubMed

The optimal approach to encourage smoking cessation after acute coronary syndrome (ACS) remains unclear. The safety of nicotine replacement therapy (NRT) after ACS is not well established. The aim of the present study was to determine the relationship between NRT use and adverse cardiovascular outcomes after ACS. Using a pre-existing database, 663 smokers with ACS were identified. The patients were separated into the NRT (n = 184) or control (n = 479) groups according to whether NRT was prescribed on hospital discharge. Multivariate logistic regression analysis was used to account for the baseline differences between the 2 groups. Of the 663 patients, 202 had adverse events in the first year after ACS. No significant differences were seen with NRT use for the 1-year combined end point of death, myocardial infarction), repeat revascularization, or rehospitalization for angina, congestive heart failure or arrhythmia (odds ratio [OR] 0.89, 95% confidence interval [CI] 0.61 to 1.30, p = 0.54). There were no differences in the individual 1-year end points of death (odds ratio 0.80, 95% confidence interval 0.33 to 1.91, p = 0.61), myocardial infarction (odds ratio 0.90, 95% confidence interval 0.40 to 2.06, p = 0.80), repeat revascularization (odds ratio 0.77, 95% confidence interval 0.44 to 1.36, p = 0.37), or rehospitalization for angina, congestive heart failure, or arrhythmia (odds ratio 1.01, 95% confidence interval 0.66 to 1.53, p = 0.97). In conclusion, NRT use was not associated with an increased risk of adverse cardiovascular events in the first year after ACS. PMID:22727182

Woolf, Kevin J; Zabad, Mohammed Nour; Post, Jennifer M; McNitt, Scott; Williams, Geoffrey C; Bisognano, John D

2012-06-20

270

MeCP2 and Rett syndrome: reversibility and potential avenues for therapy.  

PubMed

Mutations in the X-linked gene MECP2 (methyl CpG-binding protein 2) are the primary cause of the neurodevelopmental disorder RTT (Rett syndrome), and are also implicated in other neurological conditions. The expression product of this gene, MeCP2, is a widely expressed nuclear protein, especially abundant in mature neurons of the CNS (central nervous system). The major recognized consequences of MECP2 mutation occur in the CNS, but there is growing awareness of peripheral effects contributing to the full RTT phenotype. MeCP2 is classically considered to act as a DNA methylation-dependent transcriptional repressor, but may have additional roles in regulating gene expression and chromatin structure. Knocking out Mecp2 function in mice recapitulates many of the overt neurological features seen in RTT patients, and the characteristic postnatally delayed onset of symptoms is accompanied by aberrant neuronal morphology and deficits in synaptic physiology. Evidence that reactivation of endogenous Mecp2 in mutant mice, even at adult stages, can reverse aspects of RTT-like pathology and result in apparently functionally mature neurons has provided renewed hope for patients, but has also provoked discussion about traditional boundaries between neurodevelopmental disorders and those involving dysfunction at later stages. In the present paper we review the neurobiology of MeCP2 and consider the various genetic (including gene therapy), pharmacological and environmental interventions that have been, and could be, developed to attempt phenotypic rescue in RTT. Such approaches are already providing valuable insights into the potential tractability of RTT and related conditions, and are useful pointers for the development of future therapeutic strategies. PMID:21916843

Gadalla, Kamal K E; Bailey, Mark E S; Cobb, Stuart R

2011-10-01

271

Progesterone - new therapy in mild carpal tunnel syndrome? Study design of a randomized clinical trial for local therapy  

Microsoft Academic Search

BACKGROUND: Local corticosteroid injection for carpal tunnel syndrome (CTS) provides greater clinical improvement in symptoms one month after injection compared to placebo but significant symptom relief beyond one month has not been demonstrated and the relapse of symptoms is possible. Neuroprotection and myelin repair actions of the progesterone was demonstrated in vivo and in vitro study. We report the design

Paolo Milani; Mauro Mondelli; Federica Ginanneschi; Riccardo Mazzocchio; Alessandro Rossi

2010-01-01

272

Wiskott-Aldrich Syndrome, A Genetically Determined Cellular Immunologic Deficiency: Clinical and Laboratory Responses to Therapy with Transfer Factor*  

PubMed Central

Patients with diseases associated with defects in cellular immunity, such as the Wiskott-Aldrich syndrome, characteristically have severe recurrent infections and usually succumb to overwhelming infection at an early age. This communication describes a patient with this syndrome, defective delayed hypersensitivity by skin tests and by in vitro lymphocyte response, who was treated with dialysate of peripheral blood leukocytes (transfer factor). After treatment, the clinical status of the patient improved dramatically, concomitant with the development of delayed hypersensitivity to antigens to which the donor was sensitive. In vitro tests after transfer indicated that the patient's lymphocytes, when stimulated by specific antigen, produced migration inhibitory factor without concomitant DNA synthesis. These observations dissociate skin test sensitivity and activity of migration inhibitory factor from in vitro blastogenesis. Further, the response to phytohemagglutinin remained diminished before and after therapy. While these findings represent only an individual case, the climical results suggest that investigation of the use of transfer factor appears warranted in the therapy of Wiskott-Aldrich syndrome and other genetically-determined diseases associated with impaired cellular immunity. Images

Levin, A. S.; Spitler, L. E.; Stites, D. P.; Fudenberg, H. H.

1970-01-01

273

Adjuvant physical therapy versus occupational therapy in patients with reflex sympathetic dystrophy\\/complex regional pain syndrome type I  

Microsoft Academic Search

Objective: To investigate the effectiveness and cost of physical therapy (PT) or occupational therapy (OT) in patients with reflex sympathetic dystrophy (RSD).Design: Prospective randomized controlled trial, with 1 year follow-up.Setting: Two university hospitals.Patients: One hundred thirty-five patients who had been suffering from RSD of one upper extremity for less than I year.Interventions: Patients were assigned to PT, OT, or a

H. Margreet Oerlemans; Rob A. B. Oostendorp; Theo de Boo; Lyckle van der Laan; Johan L. Severens; R. Jan A. Goris

2000-01-01

274

Physiological responses to psychological challenge under hypnosis in patients considered to have the hyperventilation syndrome: implications for diagnosis and therapy.  

PubMed Central

Thirty patients who were considered to have the hyperventilation syndrome on clinical grounds (history and observation) were referred for testing: 29 patients completed a forced hyperventilation provocation test, and 28 underwent hypnosis during which time a psychological challenge was introduced which was meaningful to each individual patient. In 19/27 of these patients the PetCO2 fell by an average of 18.2 mmHg and persisted spontaneously for more than three minutes. In 10 normal controls studied in a similar fashion there was an average fall of 5 mmHg. The difference in response between responders and controls/non-responders was highly significant (P less than 0.001). A review of the literature is presented for comparison. It is considered that a psychological challenge under hypnosis may have important implications for diagnosis and therapy in some patients considered to have the hyperventilation syndrome. Images Figure 2. Figure 3. Figure 4. Figure 5.

Freeman, L J; Conway, A; Nixon, P G

1986-01-01

275

[Successful treatment with intensive immunosuppressive therapy and mechanical ventilation for idiopathic pneumonia syndrome following allogeneic bone marrow transplantation].  

PubMed

A 45-year-old man with acute myelogenous leukemia (WHO classification, AML with multilineage dysplasia) received allogeneic bone marrow transplantation from an HLA-identical brother in first remission. He became febrile on day 7, and pulmonary failure and multi-organ failure developed subsequently, requiring mechanical ventilation. Chest X-ray and CT scan demonstrated diffuse interstitial shadows, suggesting the development of idiopathic pneumonia syndrome. Administration of methylprednisolone and tacrolimus was effective, but respiratory failure exacerbated along with a decrease in the dose of steroids. Lung biopsy revealed organizing pneumonia with CMV pneumonia. Methylprednisolone and mycophenolate mofetil were instituted, which led to an improvement of lung injury. Intensive immunosuppressive therapy with mechanical ventilation should be considered for the treatment of idiopathic pneumonia syndrome after allogeneic bone marrow transplantation. PMID:19638724

Hino, Haruhiro; Kawatani, Eri; Kuwahara, Nobuo; Tominaga, Masaki; Matsuishi, Eijyo; Masuda, Masanori; Mori, Daisuke; Gondo, Hisashi

2009-07-01

276

Orofacial regulation therapy in children with Down syndrome, using the methods and appliances of Castillo-Morales.  

PubMed

The varying hypotonia in Down syndrome is one of its most dramatic signs. In particular, the facial expression and oral dysfunctions of these handicapped persons are uniquely characteristic. The more-or-less permanently open mouth; the prolapse of the tongue, exposed on the everted lower lip; and a lack of mastication, deglutition and speech are primarily caused by the hypotonic orofacial muscles. Breathing through the mouth leads to a dehydration of bacteria and plaque on gums and teeth, and ultimately to premature destruction of the dentition. This developmental syndrome indicates the need for early functional training of the orofacial muscles. Oral Regulation Therapy as described by Castillo-Morales was applied to seventy-four children here, with encouraging results. PMID:2147926

Hoyer, H; Limbrock, G J

277

Elektroconvulsie-Therapie (Electroconvulsive Therapy).  

National Technical Information Service (NTIS)

Electroconvulsive therapy is used in the Netherlands almost exclusively for the treatment of vital depressive syndrome, and therefore the methods of diagnosing and treating this syndrome are discussed in the report. The discussion of electroconvulsive the...

L. B. J. Stuyt

1983-01-01

278

The additional effect of orthotic devices on exercise therapy for patients with patellofemoral pain syndrome: a systematic review.  

PubMed

The aim of the study is to determine "the additional effect of... function" for patellofemoral pain syndrome (PFPS). The additional effect of orthotic devices over exercise therapy on pain and function. A systematic literature search was conducted in MEDLINE, CINAHL, EMBASE, Cochrane and PEDro. Randomised controlled trials and controlled clinical trials of patients diagnosed with PFPS evaluating a clinically relevant outcome were included. Treatment had to include exercise therapy combined with orthotics, compared with an identical exercise programme with or without sham orthotics. Data were summarised using a best evidence synthesis. Eight trials fulfilled the inclusion criteria, of which three had a low risk of bias. There is moderate evidence for no additive effectiveness of knee braces to exercise therapy on pain (effect sizes (ES) varied from -0.14 to 0.04) and conflicting evidence on function (ES -0.33). There is moderate evidence for no difference between knee braces and exercise therapy versus placebo knee braces and exercise therapy on pain and function (ES -0.1-0.10). More studies of high methodological quality are needed to draw definitive conclusions. PMID:21402565

Swart, Nynke M; van Linschoten, Robbart; Bierma-Zeinstra, Sita M A; van Middelkoop, Marienke

2011-03-14

279

Scheie syndrome: enzyme replacement therapy does not prevent progression of cervical myelopathy due to spinal cord compression.  

PubMed

Hurler-Scheie syndrome is caused by alpha-l-iduronidase deficiency. Enzyme replacement therapy (ERT) can improve physical capacity and reduces organomegaly. However, the effect on bradytrophic connective tissue is limited. As intravenously administered enzyme cannot cross the blood-brain barrier, the therapy of choice for the more severe Hurler syndrome is haematopoietic stem cell transplantation (HCT). In the more attenuated Scheie syndrome, neurological impairment is less severe; therefore, ERT may be appropriate to treat these patients. Information on long-term outcome in Scheie patients undergoing ERT is scarce. We report a 38-year-old female Scheie patient who has been on ERT for 8 years. While non-neurological symptoms improved, she developed paresthesias in her hands and feet and progressive pain in her legs. Somatosensory evoked potentials were abnormal, suggesting dysfunction of the dorsal funiculus and lemniscus medialis. After 6 years of ERT, a spinal MRI showed dural thickening at the upper cervical spine. These soft-tissue deposits are presumably due to the accumulation of mucopolysaccharides. Intramedullary hyperintensities at the level of C1/2 revealed cervical myelopathy. An MRI before the start of ERT had shown milder spinal lesions. Cystic lesions in the white matter of the centrum semiovale due to dilated Virchow-Robin spaces were essentially unchanged compared with the MRI scan before ERT. Decompression of the spinal cord resulted in clinical improvement. In an adult patient with Scheie syndrome, ERT failed to prevent progression of cervical myelopathy. Clinical significance of cerebral changes is unclear. Whether early HCT or intrathecal ERT could have prevented these lesions remains speculative. PMID:19894140

Illsinger, S; Lücke, T; Hartmann, H; Mengel, E; Müller-Forell, W; Donnerstag, F; Das, A M

2009-11-04

280

Stevens-Johnson syndrome after erythromycin therapy while deployed at sea.  

PubMed

Stevens-Johnson syndrome is a cutaneous reaction pattern that represents the progression of symptoms of erythema multiforme. These reactions can range from mild (EM minor) to severe (EM major) and even life-threatening (Stevens-Johnson syndrome or toxic epidermal necrolysis). The difference between Stevens-Johnson syndrome and toxic epidermal necrolysis is the percentage of body surface area involved; toxic epidermal necrolysis involves widespread skin necrosis and bullous formation with epidermal detachment resembling scalded skin. The three most common triggers for Stevens-Johnson syndrome are herpes simplex infection, Mycoplasma infection, and drug reactions. This is a case of Stevens-Johnson syndrome occurring after erythromycin treatment aboard an aircraft carrier while deployed at sea in the Persian Gulf. PMID:10957862

Williams, D A

2000-08-01

281

Compliance to continuous positive airway pressure therapy in a group of Portuguese patients with obstructive sleep apnea syndrome  

Microsoft Academic Search

Introduction  Obstructive sleep apnea–hypopnea syndrome (OSAHS) is an emerging public health concern. Although different treatments for\\u000a OSAHS had been proposed, continuous positive airway pressure (CPAP) is the first-line treatment in moderate to serious OSAHS\\u000a in which success can be achieved by increasing compliance to CPAP.\\u000a \\u000a \\u000a \\u000a \\u000a Materials and methods  This study analyzes long-term CPAP compliance in patients with OSAHS on CPAP therapy for

Claudia Alves; José Manuel Pinto Chaves Caminha; Antonio Martins da Silva; Denisa Mendonça

282

Adherence to CPAP therapy improves quality of life and reduces symptoms among obstructive sleep apnea syndrome patients  

Microsoft Academic Search

Purpose  The aim of the study was to asses quality of life and symptoms of obstructive sleep apnea syndrome (OSAS) patients after adhering\\u000a to 6 months of continuous positive airway pressure (CPAP) treatment.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  A group of 50 patients (41 men and 9 women) were diagnosed by polysomnography and treated with CPAP therapy for 6 months.\\u000a Their symptoms and health-related quality of life were

Eirini Avlonitou; Fotis Kapsimalis; George Varouchakis; Constantine I. Vardavas; Panagiotis Behrakis

283

Atraumatic osteonecrosis after estrogen replacement therapy associated with low protein S level in a patient with Turner syndrome.  

PubMed

Atraumatic osteonecrosis has been associated with a variety of clinical conditions including corticosteroid usage, alcoholism, infections, hyperbaric events, storage disorders, marrow-infiltrating diseases, coagulation defects, and some autoimmune diseases. Osteonecrosis due to thrombophilia is an extremely rare condition with only few cases reported previously in the literature. Hormone-replacement therapies cause increased risk of venous thrombosis, probably by causing a synergistic effect with inherited clotting defects. In this article, we report a young female with Turner syndrome, who developed avascular necrosis of the femoral head during treatment with oral estrogen, which was associated with low protein S levels. PMID:19648147

Ureten, Kemal; Ozturk, M Akif; Bostanci, Ahmet; Ceneli, Ozcan; Ozbek, Mustafa; Haznedaroglu, Ibrahim C

2009-07-31

284

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

Microsoft Academic Search

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

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

2011-01-01

285

[Clinical effectiveness of a combination of metformin and ramipril in complex therapy of patients with metabolic syndrome].  

PubMed

6 monthly application of the combination of metformin with ramipril (1000 and 5 mg per day respectively) in complex treatment of metabolic syndrome (MS) leads to improvement of the clinical course of ischemic heart disease (decrease of quantity and duration of painful cardial attacks, depression of the functional class of an angina of exertion, decrease of displays of chronic heart failure), normalization of arterial pressure and decrease of an abdominal obesity which can be considered as prophylaxis of development of diabetes mellitus 2 types and its cardiovascular complications. Combination of metformin with ramipril in complex treatment of a MS is effective and safe option of therapy of MS. PMID:23951921

286

Rationale and Design of a Randomized Clinical Trial of Beta Blocker Therapy (Atenolol) vs. Angiotensin II Receptor Blocker Therapy (Losartan) in Individuals with Marfan Syndrome  

PubMed Central

Background Cardiovascular pathology, including aortic root dilation, dissection, and rupture, is the leading cause of mortality in patients with Marfan syndrome (MFS). The maximal aortic root diameter at the sinuses of Valsalva is considered the best predictor of adverse cardiovascular outcome. Although advances in therapy have improved life expectancy, affected individuals continue to suffer cardiovascular morbidity and mortality. Recent studies in a FBN1-targeted mouse model of MFS with aortic pathology similar to that seen in humans showed that treatment with losartan normalized aortic root growth and aortic wall architecture. Methods The Pediatric Heart Network designed a randomized clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in MFS subjects receiving atenolol or losartan. Individuals 6 months to 25 years of age with a body surface area-adjusted aortic root Z-score > 3.0 will be eligible for inclusion. The primary aim is to compare the effect of atenolol therapy to that of losartan therapy on the rate of aortic root growth over 3 years. Secondary endpoints include progression of aortic regurgitation; incidence of aortic dissection, aortic root surgery, and death; progression of mitral regurgitation; left ventricular size and function; echocardiographically-derived measures of central aortic stiffness; skeletal and somatic growth; and incidence of adverse drug reactions. Conclusion This randomized trial should make a substantial contribution to the management of individuals with MFS and expand our understanding of the mechanisms responsible for the aortic manifestations of this disorder.

Lacro, Ronald V.; Dietz, Harry C.; Wruck, Lisa M.; Bradley, Timothy J.; Colan, Steven D.; Devereux, Richard B.; Klein, Gloria L.; Li, Jennifer S.; Minich, L. LuAnn; Paridon, Stephen M.; Pearson, Gail D.; Printz, Beth F.; Pyeritz, Reed E.; Radojewski, Elizabeth; Roman, Mary J.; Saul, J. Philip; Stylianou, Mario P.; Mahony, Lynn

2009-01-01

287

Acceleration of the Meckel Syndrome by Near-Infrared Light Therapy  

PubMed Central

Background/Aims Phototherapy using a narrow-band, near-infrared (NIR) light (using a light-emitting diode, LED) is being used to treat certain medical conditions. This narrow-band red light has been shown to stimulate cytochrome c oxidase (CCO) in mitochondria that would stimulate ATP production and has the ability to stimulate wound healing. LED treatment also decreases chemical-induced oxidative stress in tested systems. As renal cystic diseases are known to have evidence of oxidative stress with reduced antioxidant protection, we hypothesized that NIR light therapy might ameliorate the renal pathology in renal cystic disease. Methods Wistar-Wpk/Wpk rats with Meckel syndrome (MKS) were treated with light therapy on days 10–18 at which time disease severity was evaluated. Wpk rats were either treated daily for 80 s with narrow-band red light (640–690 nm wavelength) or sham treated. At termination, renal and cerebral pathology was evaluated, and renal expression and activity of enzymes were assessed to evaluate oxidative stress. Blood was collected for blood urea nitrogen (BUN) determination, the left kidney frozen for biochemical evaluation, and the right kidney and head fixed for morphological evaluation. Results There were no significant effects of LED treatment on body weight (BW) or total kidney weight in non-cystic rats. Total kidney weight was increased and anephric BW was decreased in cystic versus non-cystic controls. LED reduced BW and total kidney weight in cystic rats compared to non-light-treated cystic (control) rats. BUN was already increased almost 6-fold in cystic rats compared to control rats. BUN was further increased almost 2-fold with NIR treatment in both non-cystic and cystic rats compared to cystic and control rats. The hydrocephalus associated with Wpk/Wpk (ventricular volume expressed as total volume and as percent of anephric BW) was also more severe in NIR-treated cystic rats compared to the normal control rats. Renal glutathione peroxidase and catalase (CAT) were reduced in the cystic kidney while superoxide dismutase and CCO were increased. NIR increased CAT and CCO, marginally decreased glutathione S-transferase and slightly decreased glutathione reductase in cystic rats compared to the normal control rats. The detrimental effects of NIR may be related to reduced renal blood flow associated with progression of cystic pathology. Compression by cysts may not allow sufficient oxygen or nutrient supply necessary to support the increased oxidative phosphorylation-associated cellular activity, and the increased demand induced by NIR-increased CCO may have created further oxidative stress. Conclusion LED phototherapy initiated after the onset of symptoms was detrimental to MKS-induced pathology. NIR stimulates CCO thereby increasing the kidney's need for oxygen. We hypothesize that cystic compression of the vasculature impairs oxygen availability and the enhanced CCO activity produces more radicals, which are not sufficiently detoxified by the increased CAT activity.

Lim, Jinhwan; Gattone, Vincent H.; Sinders, Rachel; Miller, Caroline A.; Liang, Yun; Harris, Peter; Watkins, John B.; Henshel, Diane S.

2011-01-01

288

Low Areal Bone Mineral Density Values in Adolescents and Young Adult Turner Syndrome Patients Increase after Long-Term Transdermal Estradiol Therapy  

Microsoft Academic Search

Objective: To study the effects of long-term estradiol therapy on areal bone mineral density (aBMD) values in young adult Turner syndrome patients. Methods: The effects of 2-year transdermal estradiol administration on lumbar, L2–L4, aBMD values were evaluated in 12 Turner syndrome patients, 15.41–21.85 years old, who had reached adult height and had low aBMD values. Puberty was induced in all

M. Gussinyé; P. Terrades; D. Yeste; E. Vicens-Calvet; A. Carrascosa

2000-01-01

289

Comparison of conservative treatment with and without manual physical therapy for patients with shoulder impingement syndrome: a prospective, randomized clinical trial  

Microsoft Academic Search

The aim of this prospective, randomized clinical study was to compare the effectiveness of two physical therapy treatment\\u000a approaches for impingement syndrome, either by joint and soft tissue mobilization techniques or by a self-training program.\\u000a Thirty patients (Group 1, n = 15; Group 2, n = 15) with the diagnosis of an outlet impingement syndrome of the shoulder were treated either by strengthening the

Gamze Senbursa; Gul Baltac?; Ahmet Atay

2007-01-01

290

Mycophenolate mofetil therapy in frequently relapsing steroid-dependent and steroid-resistant nephrotic syndrome of childhood: current status and future directions  

Microsoft Academic Search

Clinicians are often faced with therapeutic dilemmas and challenges while treating children with frequently relapsing steroid-dependent nephrotic syndrome (SDNS) and steroid-resistant nephrotic syndrome (SRNS). In the past, children with SDNS have been treated with long-term alternate day steroids cyclophosphamide, cyclosporine (CSA), chlorambucil, levamisole, and azathioprine. The essential aim of these therapies is to maintain remission while limiting exposure to steroids.

Asha Moudgil; Arvind Bagga; Stanley C. Jordan

2005-01-01

291

Impact of Combination Evidence-Based Medical Therapy on Mortality in Patients With Acute Coronary Syndromes  

Microsoft Academic Search

Background—Several individual pharmacological agents, such as antiplatelet drugs, -blockers, ACE inhibitors, and lipid-lowering agents, have proven efficacy in reducing mortality in patients with acute coronary syndromes. However, the impact of the combination of these agents on clinical outcomes has not been studied before. Methods and Results—A total of 1358 consecutive patients presenting with acute coronary syndromes between January 1999 and

Debabrata Mukherjee; Jianming Fang; Stanley Chetcuti; Mauro Moscucci; Eva Kline-Rogers; Kim A. Eagle

2010-01-01

292

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

Microsoft Academic Search

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

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

2005-01-01

293

Simultaneous Improvement of Minimal-Change Nephrotic Syndrome and Anemia with Steroid Therapy  

Microsoft Academic Search

A 56-year-old man presented with transient anemia in minimal-change nephrotic syndrome. Following nephrotic syndrome, anemia suddenly appeared without renal dysfunction. The anemia might be attributable to hemodilution because of significant correlations between the values of hemoglobin concentration and serum total protein or blood urea nitrogen during the clinical course. A low serum level and a low urinary excretion of erythropoietin

Machiko Yabana; Minoru Kihara; Yoshiyuki Toya; Kouichi Tamura; Katsumi Matsumoto; Nobuyoshi Takagi; Seiko Kamijo; Masao Ishii; Satoshi Umemura

1999-01-01

294

Improved Identification and Antihypertension Pharmacotherapy in Cardiorenal Metabolic Syndrome: Focus on Racial/Ethnic Minorities, Olmesartan Medoxomil, and Combination Therapy  

PubMed Central

Cardiorenal metabolic syndrome (CRS) is a global health care concern in view of aging in certain populations, increased obesity, changing lifestyles, and its close association with type 2 diabetes mellitus and cardiovascular morbidity and mortality. Determining the appropriate criteria for CRS has been somewhat controversial, and efforts to fully describe and define the syndrome are still ongoing. Nonetheless, improving knowledge of the syndrome among health care professionals will help to identify patients who may require pharmacological and therapeutic lifestyle intervention, particularly with regards to addressing high-normal blood pressure and hypertension. This article reviews current clinical guidelines with a focus on the identification, especially in racial/ethnic minorities, treatment, and associated cardiovascular morbidity and mortality of high blood pressure and hypertension in patients with CRS. Efficacy and outcomes studies that provide insight into the selection of an initial antihypertensive regimen in this population will be discussed. Finally, a brief review of the benefits of olmesartan medoxomil and combination therapy and patient factors in the management of hypertension with CRS will be addressed.

Ferdinand, Keith C.

2012-01-01

295

Effectiveness of low-level laser therapy for patients with carpal tunnel syndrome: design of a randomized single-blinded controlled trial  

PubMed Central

Background Carpal tunnel syndrome is the most common neuropathy in the upper extremity, resulting from the compression of the median nerve at wrist level. Clinical studies are essentials to present evidence on therapeutic resources use at early restoration on peripheral nerve functionality. Low-level laser therapy has been widely investigated in researches related to nerve regeneration. Therefore, it is suggested that the effect of low-level laser therapy associated with other conservative rehabilitation techniques may positively affect symptoms and overall hand function in compressive neuropathies such as carpal tunnel syndrome. The aim of this study is to evaluate the effectiveness of low-level laser therapy in addition to orthoses therapy and home orientations in patients with carpal tunnel syndrome. Methods/Design Patients older than 18?years old will be included, with clinical diagnosis of carpal tunnel syndrome, excluding comorbidies. A physiotherapist will conduct intervention, with a blinding evaluator. Randomization will be applied to allocate the patients in each group: with association or not to low-level laser therapy. All of them will be submitted to orthoses therapy and home orientations. Outcome will be assessed through: pain visual analogic scale, Semmes Weinstein monofilaments™ threshold sensibility test, Pinch Gauge™, Boston Carpal Tunnel Questionnaire and two point discrimination test. Discussion This paper describes the design of a randomized controlled trial, which aim to assess the effectiveness of conservative treatment added to low-level laser therapy for patients with carpal tunnel syndrome. Trial registration Brazilian Clinical Trials Registry (ReBec) - 75ddtf / Universal Trial Number: U1111-1121-5184

2012-01-01

296

Fanconi's Syndrome Associated with Prolonged Adefovir Dipivoxil Therapy in a Hepatitis B Virus Patient  

PubMed Central

Adefovir dipivoxil (ADV) is commonly used as an antiviral agent in the treatment of chronic hepatitis B or human immunodeficiency virus infection. Nephrotoxicity has been shown to occur at daily dosages of 60-120 mg. Fanconi's syndrome is a generalized dysfunction of the renal proximal tubular cells, which is usually accompanied by complications. Here we report a case of Fanconi's syndrome in a chronic hepatitis B patient who had been treated with a prolonged regimen of ADV at 10 mg/day. A 47-year-old man complained of severe back and chest-wall pain. He had chronic hepatitis B and had been treated with ADV at a daily dose of 10 mg for 38 months. He was hospitalized because of severe bone pain, and laboratory and radiologic findings suggested a diagnosis of Fanconi's syndrome with osteomalacia. After discontinuation of the ADV, he recovered and was discharged from hospital. His laboratory findings had normalized within 2 weeks. This case indicates that Fanconi's syndrome can be acquired by a chronic hepatitis B patient taking ADV at a conventional dosage of 10 mg/day. Therefore, patients treated with long-term ADV should be checked regularly for the occurrence of ADV-induced Fanconi's syndrome.

Jung, Young Kul; Choi, Jong Hwan; Kim, Chung Ho; Jung, Eun Suk; Kim, Ji Hoon; Park, Jong Jae; Kim, Jae Seon; Bak, Young-Tae; Byun, Kwan Soo

2010-01-01

297

Low frequency of FLT3 gene internal tandem duplication and activating loop mutation in therapy-related acute myelocyticleukemia and myelodysplastic syndrome  

Microsoft Academic Search

FLT3 gene internal tandem duplication (ITD) and activating loop mutations (D835) were determined in 22 cases of therapy-related acute myelocytic leukemia\\/myelodysplastic syndrome (t-AML\\/MDS) and 102 cases of de novo AML\\/MDS. In t-AML\\/MDS, FLT3 ITD was absent, and D835 was found in only one case of therapy-related acute promyelocytic leukemia (APL). In de novo AML\\/MDS, however, FLT3 ITD and D835 were

Wing Y. Au; Alvin T. Fung; Edmond S. Ma; Raymond H. Liang; Yok L. Kwong

2004-01-01

298

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 6–11 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

299

Drug-based therapies for vascular disease in Marfan syndrome: from mouse models to human patients.  

PubMed

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-beta 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-based 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

300

Ehlers-Danlos syndrome type IV: diagnosis and therapy of associated bowel perforation.  

PubMed

Ehlers-Danlos syndrome type IV is a heritable disease of type III collagen metabolism. This diagnosis is suspected in a patient with a combination of clinical manifestations and family history, but it is confirmed only by culture of the patient's skin fibroblasts and demonstration of a defect in type III collagen metabolism. The disease may rarely present with spontaneous colonic perforation, a complication traditionally treated by primary closure of the perforated segment and creation of an end colostomy. Attempts at bowel reanastomosis have often resulted in repeated colon perforations. We present the first patient with Ehlers-Danlos type IV syndrome to develop a colon perforation proximal to an end colostomy, and discuss the surgical strategy to prevent recurrences of this and other postoperative complications associated with the syndrome. PMID:1998319

Stillman, A E; Painter, R; Hollister, D W

1991-03-01

301

Shigatoxin-associated hemolytic uremic syndrome: current molecular mechanisms and future therapies  

PubMed Central

Hemolytic uremic syndrome is the leading cause of acute kidney injury in childhood. Ninety percent of cases are secondary to gastrointestinal infection with shigatoxin-producing bacteria. In this review, we discuss the molecular mechanisms of shigatoxin leading to hemolytic uremic syndrome and the emerging role of the complement system and vascular endothelial growth factor in its pathogenesis. We also review the evidence for treatment options to date, in particular antibiotics, plasma exchange, and immunoadsorption, and link this to the molecular pathology. Finally, we discuss future avenues of treatment, including shigatoxin-binding agents and complement inhibitors, such as eculizumab.

Keir, Lindsay S; Marks, Stephen D; Kim, Jon Jin

2012-01-01

302

Dapsone hypersensitivity syndrome: A rare life threatening complication of dapsone therapy  

PubMed Central

Dapsone can cause several adverse effects, the most serious being dapsone hypersensitivity syndrome (DHS), which is potentially fatal. Here we report a case of severe, life threatening dapsone systemic hypersensitivity syndrome in a 17-year-old male who presented with high grade fever, eosinophilia, lymphadenopathy, skin rash, hepatitis and encephalopathy, which was managed successfully with oral steroids. The case is being reported to emphasize the need for timely diagnosis and prompt treatment of this rare complication for successful outcomes. DHS is also reviewed in brief.

Vinod, Kolar Vishwanath; Arun, Karyampudi; Dutta, Tarun Kumar

2013-01-01

303

Mycophenolate mofetil therapy for children with steroid-resistant nephrotic syndrome  

Microsoft Academic Search

Treating children with steroid-resistant nephrotic syndrome (SRNS) has been a clinical challenge for pediatricians. We recruited\\u000a 24 children (18 boys and six girls) with steroid-resistant idiopathic nephrotic syndrome (SRINS) who were <2 years. All patients\\u000a were administered prednisone 2 mg\\/kg per day prior to mycophenolate mofetil (MMF). By the end of the eighth week, MMF was\\u000a initiated at 25–30 mg\\/kg daily for 6??12 months.

Zhihui Li; Cuirong Duan; Jinhua He; Tianhui Wu; Mai Xun; Yi Zhang; Yan Yin

2010-01-01

304

Long-term remission of pulmonary veno-occlusive disease associated with primary Sjögren's syndrome following immunosuppressive therapy.  

PubMed

The patient described here is a 21-year-old Japanese woman with primary Sjögren's syndrome (pSS) presenting with worsening of dyspnea, palpitation, recurrent parotitis, and arthritis. Chest computed tomography showed diffuse interlobular septal thickening and ground-glass opacities. Right heart catheterization demonstrated pulmonary hypertension, right-sided heart failure, normal pulmonary capillary wedge pressure, and no evidence of arterio-venous shunt. Transbronchial lung biopsy showed luminal obliteration of pulmonary venules by intimal cellular proliferations, without abnormalities in the small pulmonary arteries. These findings were consistent with pulmonary veno-occlusive disease (PVOD). Immunosuppressive therapy, starting with prednisolone 20 mg/day and subsequently combined with azathioprine, resulted in the disappearance of the signs and symptoms, including exertional dyspnea and abnormal pulmonary parenchymal shadows on computed tomography, and the normalization of pulmonary artery pressure. So far, there have been no reported cases of PVOD associated with pSS. Of interest, immunosuppressive therapy without vasodilator therapy almost completely resolved the pulmonary hypertension in this patient. PMID:21394665

Naniwa, Taio; Takeda, Yutaka

2011-03-11

305

Development of novel efficient SIN vectors with improved safety features for Wiskott-Aldrich syndrome stem cell based gene therapy.  

PubMed

Gene therapy is a promising therapeutic approach to treat primary immunodeficiencies. Indeed, the clinical trial for the Wiskott-Aldrich Syndrome (WAS) that is currently ongoing at the Hannover Medical School (Germany) has recently reported the correction of all affected cell lineages of the hematopoietic system in the first treated patients. However, an extensive study of the clonal inventory of those patients reveals that LMO2, CCND2 and MDS1/EVI1 were preferentially prevalent. Moreover, a first leukemia case was observed in this study, thus reinforcing the need of developing safer vectors for gene transfer into HSC in general. Here we present a novel self-inactivating (SIN) vector for the gene therapy of WAS that combines improved safety features. We used the elongation factor 1 alpha (EFS) promoter, which has been extensively evaluated in terms of safety profile, to drive a codon-optimized human WASP cDNA. To test vector performance in a more clinically relevant setting, we transduced murine HSPC as well as human CD34+ cells and also analyzed vector efficacy in their differentiated myeloid progeny. Our results show that our novel vector generates comparable WAS protein levels and is as effective as the clinically used LTR-driven vector. Therefore, the described SIN vectors appear to be good candidates for potential use in a safer new gene therapy protocol for WAS, with decreased risk of insertional mutagenesis. PMID:21851067

Avedillo Díez, Inés; Zychlinski, Daniela; Coci, Emanuele G; Galla, Melanie; Modlich, Ute; Dewey, Ricardo A; Schwarzer, Adrian; Maetzig, Tobias; Mpofu, Nonsikelelo; Jaeckel, Elmar; Boztug, Kaan; Baum, Christopher; Klein, Christoph; Schambach, Axel

2011-08-31

306

Evaluation of risk markers fluctuation during an initial therapy with rosiglitazon in patients suffering from metabolic syndrome.  

PubMed

The aim of this study was to examine the effects of hypoglycaemic drug-agonists of PPAR-gama receptors-rosiglitazone (Avandia,4 mg - Glaxo Smith Kline) on values of wide-spread risk - markers-fibrinogen, C-reactive protein and uric acid and glicolysated haemoglobin HbA1C as parameter of metabolic control .We included fourty patients with criteria for metabolic syndrome and evaluated results into groups of diabetic and prediabetic patients according to criteria of IDF (International Diabetic Federation)These risk markers and glicolysated haemoglobin HbA1C were observed at the start of therapy, then after four, eight and twelve weeks and results were compared and statistically calculated. Three months initial therapy with rosiglitazone significantly reduced values of HbA1C, fibrinogen and CRP but not uric acid in prediabetic patients.Rosiglitazone initial three months therapy significantly reduced HbA1C, fibrinogen and uric acid, but not CRP in diabetic patients. PMID:20001999

Maci?-Dzankovi?, Amra; Dzankovi?, Fuad; Pojski?, Belma; Velija-Asimi, Zelija

2009-11-01

307

Management of hyperlipidemia in children with refractory nephrotic syndrome: The effect of statin therapy  

Microsoft Academic Search

The efficacy and safety of hydroxymethylglutaric coenzyme A reductase inhibitor (statins) in the treatment of hyperlipidemia were evaluated in 12 infants and children with steroid-resistant nephrotic syndrome followed prospectively for 1 to 5 years. All patients experienced a hypolipidemic response with a marked reduction in their total cholesterol (40%), low-density lipoprotein cholesterol (44%), and triglyceride levels (33%), but no appreciable

Sami A. Sanjad; Abbas Al-Abbad; Saleh Al-Shorafa

1997-01-01

308

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

Microsoft Academic Search

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

Mine Uyanik; Hulya Kayihan

309

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

Microsoft Academic Search

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

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

2006-01-01

310

The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report  

Microsoft Academic Search

The Churg-Strauss syndrome (CSS) is a distinct form of vasculitis that is notable for its eosinophilia and frequent associations with asthma and sinusitis. Because there has been an increasing recognition that CSS can develop in patients with asthma and that CSS might be associated with specific asthma treatments, the National Heart, Lung, and Blood Institute, the National Institute of Allergy

Peter F. Weller; Marshall Plaut; Virginia Taggart; Anne Trontell

2001-01-01

311

Insulin-sensitizing agents as primary therapy for patients with polycystic ovarian syndrome  

Microsoft Academic Search

BACKGROUND: This paper is a systematic review of metformin versus clomiphene citrate (CC) in women with polycystic ovary syndrome (PCOS). METHODS: Meta-analysis Of Observational Studies in Epidemiology (MOOSE) and QUality Of Reporting Of Meta-analyses (QUOROM) guidelines were followed. A systematic computerized literature search was done of seven bibliographic databases. Inclusion criteria included cohort and randomized controlled trials (RCT) of women

Sonya Kashyap; George A. Wells; Zev Rosenwaks

2004-01-01

312

Therapy-related myelodysplastic syndrome in children with medulloblastoma following MOPP chemotherapy  

Microsoft Academic Search

Between 1978 and 1988, 20 children with medulloblastoma (MB) of the brain were treated postoperatively with MOPP (nitrogen mustard, vincristine, prednisone, and procarbazine). All but one received post-operative radiation prior to MOPP. Eight of 20 patients remained in continuous complete remission from MB, two of whom eventually developed myelodysplastic syndrome (MDS). Following resection of MB at age 12 months, one

Ammar Hayani; Donald H. Mahoney Jr; Linda D. Taylor

1992-01-01

313

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

314

Approach to Treatment of the Patient with Metabolic Syndrome: Lifestyle Therapy  

Microsoft Academic Search

The National Cholesterol Education Program's Adult Treatment Panel III definition of the metabolic syndrome identifies those at high risk for diabetes mellitus and\\/or a cardiac event by clustering a number of easily measured clinical findings, including abdominal obesity, elevated plasma levels of triglycerides, low plasma levels of high-density lipoprotein cholesterol, elevated fasting blood glucose, and elevated blood pressure. The presence

Neil J. Stone; David Saxon

315

Brief Behavioral Therapies Reduce Early Positive Airway Pressure Discontinuation Rates in Sleep Apnea Syndrome: Preliminary Findings  

Microsoft Academic Search

Sleep apnea syndrome (SAS) is a serious disorder with significant daytime consequences. Treatment for SAS most commonly takes the form of positive airway pressure (PAP). Although effective, PAP adherence is often below expectations. Previous studies have suggested that the provision of information on the importance of PAP use can enhance adherence. In this study, we compare 2 brief behavioral approaches—traditional

Mark S. Aloia; Kevin Smith; J. Todd Arnedt; Richard P. Millman; Michael Stanchina; Carol Carlisle; Jacki Hecht; Belinda Borrelli

2007-01-01

316

A European multicenter randomized controlled trial of single dose surfactant therapy for idiopathic respiratory distress syndrome  

Microsoft Academic Search

We performed a multicenter prospective randomized controlled trial to determine the efficacy and safety of the surfactant preparation, Survanta (Abbott Laboratories, Chicago, USA), for 750–1750 g infants with idiopathic respiratory distress syndrome, (IRDS) receiving assisted ventilation with 40% or more oxygen. One hundred and six eligible infants from the eight participating centers were randomly assigned between March 1986 and June

J. D. Horbar; R. F. Soll; H. Schachinger; G. Kewitz; H. T. Versmold; W. Lindner; G. Duc; D. Mieth; O. Linderkamp; E. P. Zilow; P. Lemburg; V. Von Loewenich; M. Brand; I. Minoli; G. Moro; K. P. Riegel; R. Roos; L. Weiss; J. F. Lucey

1990-01-01

317

The Role of Active Release Manual Therapy for Upper Extremity Overuse Syndromes—A Preliminary Report  

Microsoft Academic Search

The study was carried out to evaluate the efficacy of a specific protocol for treatment of overuse syndromes known as Active Release. This treatment protocol was taught to an athletic trainer who had six months experience before initiating a prospective study. Most of the 28 patients who were in the study had failed previous medical treatment for epicondylitis, tendonitis, and

Berit Schiottz-Christensen; Vert Mooney; Shadi Azad; Dan Selstad; Jennifer Gulick; Mark Bracker

1999-01-01

318

Treatment of Cannabis Withdrawal Syndrome Using Cognitive-Behavioral Therapy and Relapse Prevention for Cannabis Dependence  

Microsoft Academic Search

Cannabis is the most frequently used illegal substance in the United States and Europe. There is a dramatic increase in the demand for treatment for cannabis dependence. The majority of marijuana-dependent individuals who enter treatment have difficulty in achieving and maintaining abstinence from cannabis partly due to the cannabis withdrawal syndrome. Onset of most symptoms occurs during the 1st week

Aviv Weinstein; Hila Miller; Eti Tal; Irit Ben Avi; Isachar Herman; Rachel Bar-Hamburger; Miki Bloch

2010-01-01

319

The Epidemiology of Meconium Aspiration Syndrome: Incidence, Risk Factors, Therapies, and Outcome  

Microsoft Academic Search

OBJECTIVE.We sought to examine, in a large cohort of infants within a definable population of live births, the incidence, risk factors, treatments, complications, and outcomes of meconium aspiration syndrome (MAS). DESIGN.Data were gathered on all of the infants in Australia and New Zealand who were intubated and mechanically ventilated with a primary diagnosis of MAS (MASINT) between 1995 and 2002,

Peter A. Dargaville; MBBS FRACP; Beverley Copnell

320

Modified Atkins diet therapy for a case with glucose transporter type 1 deficiency syndrome  

Microsoft Academic Search

Glucose transporter type 1 deficiency syndrome (GLUT-1 DS), giving rise to impaired glucose transport across the blood–brain barrier, is characterized by infantile seizures, complex motor disorders, global developmental delay, acquired microcephaly, and hypoglycorrhachia. GLUT-1 DS can be treated effectively with a ketogenic diet because it can provide an alternative fuel for brain metabolism; however, the excessive restriction of food intake

Susumu Ito; Hirokazu Oguni; Yasushi Ito; Keiko Ishigaki; Junko Ohinata; Makiko Osawa

2008-01-01

321

Personality Characteristics of Patients with Myofascial Pain-Dysfunction (MPD) Syndrome Unresponsive to Conventional Therapy  

Microsoft Academic Search

The composite MMPI profiles of 42 successfully-treated and 42 unsuccessfully-treated female patients with MPD syndrome were compared statistically. The profiles did not differ in configuration, but that of the unsuccessful patients was significantly higher than that of the successful group, indicating a greater degree of emotional distress. The pattern of deviation from normal for both groups was diagnostic of a

Ronald A. Schwartz; Charles S. Greene; Daniel M. Laskin

1979-01-01

322

The use of Simvastatin Plus Metformin Therapy in Patients With Polycystic Ovarian Syndrome  

Microsoft Academic Search

Background: Polycystic ovarian syndrome (PCOS) is a common endocrine disorder that affects the fertility of reproductive-aged women due to high levels of testosterone. Metformin is currently used to treat patients with PCOS in order to improve the biochemical markers of the disease. New studies show that statin medications like simvastatin may prove efficacious with reduction of testosterone levels, and therefore,

Erin Carrick

2012-01-01

323

Towards Liver-Directed Gene Therapy for Crigler-Najjar Syndrome  

Microsoft Academic Search

Crigler-Najjar (CN) syndrome is a recessive inherited disorder caused by deficiency of uridine diphosphoglucuronosyl transferase 1A1. This hepatic enzyme catalyzes the glucuronidation of bilirubin, an essential step in excretion into bile of this neurotoxic compound. As a result, CN patients suffer from severe unconjugated hyperbilirubinemia and are at risk of bilirubin encephalopathy. Over the last decades ex vivo and in

Paula S. Montenegro Miranda; Piter J. Bosma

2009-01-01

324

Angiogenesis Research to Improve Therapies for Vascular Leak Syndromes, Intra-Abdominal Adhesions, and Arterial Injuries.  

National Technical Information Service (NTIS)

The three goals of this project are: (i) to discover and develop novel drugs which could prevent or reverse the vascular leak syndrome; (ii) to develop angiogenesis inhibitors which would inhibit post-operative abdominal adhesions; and, (iii) to isolate e...

J. Bischoff J. Folkman M. Puder

2007-01-01

325

[Application of extracorporeal membrane oxygenation therapy (ECMO) in the treatment of severe adult respiratory distress syndrome].  

PubMed

Extracorporeal oxygenator is used in severe respiratory and/or circulatory failure that is intractable to other therapies. In ECMO therapy, poorly oxygenated blood is pumped through an extracorporeal oxygenator and directed back to the patient's circulation. The therapy can be utilized to maintain the homeostasis of the organ system during circulatory or respiratory failure resulting from a disease. Due to risk of complications, ECMO should be used with caution on carefully selected patients. For a severely ill patient ECMO can be life-saving when started early. PMID:22073537

Mildh, Leena; Lemström, Karl B; Jokinen, Janne J; Raivio, Peter; Suojaranta-Ylinen, Raili; Hämmäinen, Pekka

2011-01-01

326

[Apparative aversive therapy in combination with verbal suggestions in special obsessional syndromes (initial investigation)].  

PubMed

The author restricts the use of aversion therapy by means of deliberate production of pain to obsessional, especially therapy-resistant disturbances of a permanent nature, with consideration being, of course, given to ethical factors. Experiences worthy of generalization are derived from methodically varied courses of treatment, bringing out suggestive moments subliminally involved in any therapeutical situation and also specifically used by the therapist. In addition, the author emphasizes the need for simultaneously developing, besides aversion therapy, positive attitudes and behavior patterns. PMID:609650

Dummer, W

1977-12-01

327

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

Microsoft Academic Search

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.

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

2011-01-01

328

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

329

Changes in regional cerebral blood flow in the thalamus after electroconvulsive therapy for patients with complex regional pain syndrome type 1 (preliminary case series)  

Microsoft Academic Search

Background and Objectives: The aim of the present case series was to examine whether changes in regional cerebral blood flow (rCBF) induced by electroconvulsive therapy (ECT) in the thalamus are related to the efficacy of ECT. Four chronic pain patients with complex regional pain syndrome (CRPS) type-1 (age, 33 to 58 years) who had failed to respond to standard pain

Sei Fukui; Shino Shigemori; Shuichi Nosaka

2002-01-01

330

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

PubMed Central

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

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

2012-01-01

331

Role of Continuous Positive Airway Pressure Therapy on the Pathogenesis of Sleep-Related Frontal Lobe Epilepsy in a Child With Obstructive Sleep Apnea Syndrome  

Microsoft Academic Search

We describe an obese child with severe obstructive sleep apnea syndrome in whom nocturnal frontal lobe seizures developed within a week after therapy was started with continuous positive airway pressure. The video polysomnographic study after the onset of nocturnal episodes showed 3 seizures: 2 starting from slow-wave sleep when he was sleeping with continuous positive airway pressure, and 1 from

Silvia Miano; Andrea Pelliccia; Melania Evangelisti; Jacopo Pagani; Maria Pia Villa

2008-01-01

332

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

PubMed

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

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

2013-10-23

333

Filgrastim as a Rescue Therapy for Persistent Neutropenia in a Case of Dengue Hemorrhagic Fever with Acute Respiratory Distress Syndrome and Myocarditis  

PubMed Central

Pathogenesis of dengue involves suppression of immune system leading to development of characteristic presentation of haematological picture of thrombocytopenia and leucopenia. Sometimes, this suppression in immune response is responsible for deterioration in clinical status of the patient in spite of all specific and supportive therapy. Certain drugs like steroids are used for rescue therapy in conditions like sepsis. We present a novel use of filgrastim as a rescue therapy in a patient with dengue hemorrhagic fever (DHF) with acute respiratory distress syndrome (ARDS), myocarditis, and febrile neutropenia and not responding to standard management.

Deepak, Desh; Garg, Rakesh; Pawar, Mridula; Banerjee, Neerja; Solanki, Rakesh; Maurya, Indubala

2011-01-01

334

Stevens-Johnson syndrome after lenalidomide therapy for multiple myeloma: a case report and a review of treatment options.  

PubMed

Stevens- Johnson syndrome (SJS) is a severe and life-threatening condition. Although allopurinol, an antihyperuricemia drug, is the drug most commonly associated with SJS, more than 100 different causative drugs have been reported. Among hematologic drugs recently introduced into the market, drugs such as rituximab, imatinib, and bortezomib are reported. Here, we describe a patient with SJS while receiving lenalidomide in combination with prednisolone for treatment-naïve multiple myeloma. Although SJS has been reported rarely as an adverse reaction to Lenalidomide, this drug should be considered in the etiology of SJS, and the increased number of prescriptions of Lenalidomide for the therapy of multiple myeloma has to stress the awareness of its potentially serious side-effects. PMID:21702057

Allegra, Alessandro; Alonci, Andrea; Penna, Giuseppa; Russo, Sabina; Gerace, Demetrio; Greve, Bruna; D'Angelo, Arianna; Catena, Sara; Musolino, Caterina

2011-06-23

335

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

336

The clinical practice of Cognitive Behavioural Therapy for children and young people with a diagnosis of Asperger's Syndrome.  

PubMed

Children and young people diagnosed with Asperger's Syndrome (AS) have significant social-communication difficulties and impaired empathy and theory of mind skills. These difficulties place them at risk of developing mental health problems, particularly anxiety, depression and obsessive compulsive disorder. Although Cognitive Behavioural Therapy (CBT) is recognised as an effective intervention for these problems in both child and adult populations, little research has specifically looked at the use of CBT with children and young people with an AS diagnosis. However, limited evidence suggests that CBT, if suitably adapted, is a feasible and potentially helpful treatment option. This paper focuses on the clinical practice of CBT and explores how the underpinning therapeutic relationship can be modified to meet the cognitive needs of this particular group of young clients. PMID:20516059

Donoghue, Kate; Stallard, Paul; Kucia, Joanna

2010-06-01

337

POEMS syndrome treated with melphalan high-dose therapy and autologous blood stem cell transplantation: a single-institution experience.  

PubMed

The acronym POEMS syndrome stands for a rare multi-system disorder, comprised of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Here, we present a single-center report of a series of five POEMS patients treated with melphalan high-dose therapy (HDT) with subsequent autologous blood stem cell transplantation (ABSCT). After a median follow-up of 52 months from time of diagnosis (range, 15-192) and a median follow-up of 18 months after ABSCT (range, 11-120), all patients were alive. Overall, no severe transplantation-associated complications such as engraftment syndrome or peri- or post-transplant death were noted. In two cases, HDT followed by ABSCT resulted in a complete hematologic response; in the additional three cases, partial responses (PR) were achieved including one very good hematologic PR. Only one patient with initial PR developed progressive disease nearly 2.5 years after transplantation. Consequently, a second HDT with ABSCT was successfully applied resulting in clinical improvement and hematologic PR. In line with previous single-center reports, melphalan HDT followed by ABSCT proved to be a first-line treatment option with tolerable side effects in severely affected POEMS patients with progressing symptoms. PMID:22543828

Thoennissen, Gabriela B; Thoennissen, Nils H; Fritz, Fleur; Hilbig, Andreas; Kerkhoff, Andrea; Liersch, Ruediger; Krug, Utz; Koschmieder, Steffen; Müller-Tidow, Carsten; Mesters, Rolf; Kropff, Martin; Berdel, Wolfgang E

2012-04-29

338

[Congenital myasthenic syndromes: difficulties in the diagnosis, course and prognosis, and therapy--The French National Congenital Myasthenic Syndrome Network experience].  

PubMed

Congenital myasthenic syndromes (CMS) are a heterogeneous group of disorders caused by genetic defects affecting neuromuscular transmission and leading to muscle weakness accentuated by exertion. Three different aspects have been investigated by members of the national French CMS Network: the difficulties in making a proper diagnosis; the course and long-term prognosis; and the response to therapy, especially for CMS that do not respond to cholinesterase inhibitors. CMS diagnosis is late in most cases because of confusion with other entities such as: congenital myopathies, due to the frequent presentation in patients of myopathies such as permanent muscle weakness, atrophy and scoliosis, and the abnormalities of internal structure, diameter and distribution of fibers (type I predominance, type II atrophy) seen on biopsy; seronegative autoimmune myasthenia gravis, when CMS is of late onset; and metabolic myopathy, with the presence of lipidosis in muscle. The long-term prognosis of CMS was studied in a series of 79 patients recruited with the following gene mutations: CHRNA; CHRNE; DOK7; COLQ; RAPSN; AGRN; and MUSK. Disease-course patterns (progressive worsening, exacerbation, stability, improvement) could be variable throughout life in a given patient. DOK7 patients had the most severe disease course with progressive worsening: of the eight wheelchair-bound and ventilated patients, six had mutations of this gene. Pregnancy was a frequent cause of exacerbation. Anticholinesterase agents are the first-line therapy for CMS patients, except for cases of slow-channel CMS, COLQ and DOK7. In our experience, 3,4-DAP was a useful complement for several patients harboring CMS with AChR loss or RAPSN gene mutations. Ephedrine was given to 18 patients (eight DOK7, five COLQ, four AGRN and one RAPSN). Tolerability was good. Therapeutic responses were encouraging even in the most severely affected patients, particularly with DOK7 and COLQ. Salbutamol was a good alternative in one patient who was allergic to ephedrine. PMID:23452772

Eymard, B; Stojkovic, T; Sternberg, D; Richard, P; Nicole, S; Fournier, E; Béhin, A; Laforêt, P; Servais, L; Romero, N; Fardeau, M; Hantaï, D

2013-02-01

339

Growth hormone deficiency and growth hormone therapy in Ullrich-Turner-Syndrome  

Microsoft Academic Search

Zusammenfassung Bei einer 7jährigen Patientin mit dem Chromosomensatz 45 XO wurde mit Hilfe von Stimulationstesten ein Mangel an Wachstumshormon nachgewiesen. Die Wachstumsrate von 2 cm\\/Jahr ließ sich mit. Hilfe von humanem Wachstumshormon auf 4,1 cm\\/Jahr (2 Behandlungsjahre) und unter zusätzlicher Gabe von Anabolica auf 5,7 bzw, 5,4 cm\\/Jahr (3. bzw. 4. Behandlungsjahr) steigern. Bei vier Patientinnen mit Ullrich-Turner-Syndrom (2mal 45X\\/,

O. Butenandt

1980-01-01

340

Effects of Long-Term Growth Hormone Therapy on Adrenal Steroidogenesis in Turner Syndrome  

Microsoft Academic Search

It has been shown that growth hormone (GH) and insulin-like growth factor-1 (IGF1) enhance steroidogenesis responsiveness to ACTH in cultured adrenal cells. To investigate the GH effect on adrenal steroidogenesis in non-GH-deficient subjects, we studied 9 girls with Turner syndrome (chronological age 5.5–7.2 years; bone age 5–7 years). In all subjects an ACTH test (Synacthen depot, 0.25 mg i.v. with

R. Balducci; V. Toscano; D. Larizza; A. Mangiantini; C. Galasso; G. Municchi; A. Di Rito; S. Picone; B. Boscherini

1998-01-01

341

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

Microsoft Academic Search

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

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

2000-01-01

342

An Unusual Combination of Phototoxicity and Stevens-Johnson Syndrome due to Antimalarial Therapy  

Microsoft Academic Search

A 12-year-old boy developed a phototoxic rash with subsequent progression to Stevens-Johnson syndrome due to prophylactic ingestion of antimalarials (chloroquine and sulfadoxine-pyrimethamine; Fansidar®). The patient recovered from his skin symptoms after 4 weeks during which he received systemic corticosteroids and antibiotics. This unusual combination of two different patterns of adverse cutaneous drug reactions was most probably caused by the sulfonamide

B. Ordel; A. Sivayathorn; H. Hönigsmann

1989-01-01

343

The Rumination Syndrome (Clinical and Manometric Profile, Therapy, and Long-Term Outcome)  

Microsoft Academic Search

The aims of this study were to investigate thediagnostic studies necessary to identify ruminationsyndrome and the long-term therapeutic outcomes ofpatients with rumination syndrome. Sixteen patients with rumination were evaluated between 1989 and1995. Esophageal motility, gastric emptying, uppergastrointestinal motility, and electrogastrography ofall patients were reviewed; follow-up information about their current status was available from10 of the 16 patients. Duration of symptoms

Irfan Soykan; Jiande Chen; Bradley J. Kendall; Richard W. Mccallum

1997-01-01

344

Complex regional pain syndrome related movement disorders: studies on pathophysiology and therapy  

Microsoft Academic Search

Complex regional pain syndrome (CRPS) may occur after trauma, usually to one limb, and is characterised by pain and disturbed blood flow, temperature regulation and motor control. Knowledge on CRPS and its movement disorders is scarce.\\u000aDysfunction in small nerve fiber processing was found in CRPS patients with dystonia. Furthermore, a dominant pattern of fixed dystonia was observed in two

Alexander Gerard Munts

2011-01-01

345

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

346

[Anaesthesia and pain therapy: perioperative management of patients with complex regional pain syndrome].  

PubMed

The complex regional pain syndrome (CRPS) is indeed a very complex pain situation. The treatment of CRPS is challenging. Patients with an acute CRPS or a CRPS in the past, who have to undergo surgery, provide a special situation. Recommendations for the management of such patients will be described in the article. High degree of evidence exists for high doses of vitamin C to prevent the development of a CRPS in trauma patients. PMID:23235899

Pouskoulas, Christos Dimitrios; Aeschbach, Armin; Ruppen, Wilhelm

2012-12-12

347

Etanercept Therapy for Late-Onset Idiopathic Pneumonia Syndrome After Nonmyeloablative Allogeneic Hematopoietic Stem Cell Transplantation  

Microsoft Academic Search

Idiopathic pneumonia syndrome (IPS) is a noninfectious pulmonary complication of allogeneic hematopoietic stem cell transplantation (AHSCT), which usually develops within the first 100 days after transplantation. Donor T-cell-derived tumor necrosis factor-? (TNF-?) may play a crucial role in the pathogenesis of IPS, and inhibition of TNF-? has been used as a therapeutic option. We report two patients who had late-onset

H. W. Tun; K. H. Wallace; S. F. Grinton; A. Khoor; C. D. Burger

2005-01-01

348

Deep brain stimulation therapy for treatment-refractory Tourette’s syndrome  

Microsoft Academic Search

Tourette’s 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

349

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

Microsoft Academic Search

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

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

2011-01-01

350

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

351

Effect of intensive lipid-lowering therapy on mortality after acute coronary syndrome (a patient-level analysis of the Aggrastat to Zocor and Pravastatin or Atorvastatin Evaluation and Infection Therapy-Thrombolysis in Myocardial Infarction 22 trials).  

PubMed

Compared with moderate lipid lowering with standard-dose statin therapy, intensive lipid lowering with high-dose statin therapy after acute coronary syndromes (ACS) significantly reduces cardiovascular events. However, the 2 trials of high-dose versus standard-dose statin therapy in patients with ACS, Aggrastat to Zocor (A to Z) and Pravastatin or Atorvastatin Evaluation and Infection Therapy-Thrombolysis In Myocardial Infarction 22 (PROVE-IT-TIMI 22), were not individually powered to evaluate the impact on mortality alone. In this study, a pooled, patient-level analysis of these trials of 8,658 post-ACS patients was performed to provide a more robust estimate of the impact of intensive statin therapy on mortality. By 8 months, achieved low-density lipoprotein levels were lower in the group with intensive statin therapy (median 64 mg/dl, interquartile range 51 to 81) than in the group with moderate statin therapy (median 87 mg/dl, interquartile range 71 to 107) (p <0.001). All-cause mortality was significantly reduced in the group with intensive statin therapy compared with the group with moderate statin therapy (3.6% vs 4.9%, hazard ratio 0.77, 95% confidence interval 0.63 to 0.95, p = 0.015), without significant interaction by trial (interaction p = 0.63). The reduction in all-cause mortality with intensive statin therapy was consistent across key subgroups. In conclusion, in this analysis of >8,600 patients, intensive lipid lowering with high-dose statin therapy after ACS was associated with reduced mortality compared with moderate lipid lowering with standard-dose statin therapy. On the basis of these findings, 1 death was prevented for every 95 patients treated with high-dose statin therapy for 2 years. The results of this pooled analysis provide further evidence for early intensive statin therapy after ACS. PMID:17884359

Murphy, Sabina A; Cannon, Christopher P; Wiviott, Stephen D; de Lemos, James A; Blazing, Michael A; McCabe, Carolyn H; Califf, Robert M; Braunwald, Eugene

2007-07-18

352

[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

González García, Paloma; Manzano Hernández, M Pilar; Muñoz Tomás, M Teresa; Martín Hernández, Carlos; Forcano García, Mercedes

2013-03-14

353

Efficacy of extracorporeal shock wave therapy for the treatment of chronic pelvic pain syndrome: a randomized, controlled trial.  

PubMed

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; Alizadeh, Farshid; Moayednia, Amir; Emadi, Masoud; Khorami, Mohammad Hatef; Haghdani, Saeid

2013-08-28

354

Ex-vivo Gene Therapy Restores LEKTI Activity and Corrects the Architecture of Netherton Syndrome-derived Skin Grafts  

PubMed Central

Netherton syndrome (NS) is a debilitating congenital skin disorder caused by mutations in the SPINK5 gene encoding the lymphoepithelial Kazal-type-related inhibitor (LEKTI). It is characterized by defective keratinization, recurrent infections, and hypernatraemic dehydration with a mortality rate of about 10% in the first year of life. Currently, there are no curative treatments for NS. We have developed a HIV-1 based, self-inactivating lentiviral vector to express SPINK5 in keratinocytes as part of an ex-vivo gene therapy strategy for NS. High transduction efficiency was achieved in NS keratinocytes and reconstitution of LEKTI expression was confirmed in previously deficient cells. These genetically corrected keratinocytes were further tested in an in vitro organotypic culture (OTC) system and in vivo mouse/human skin engraftment model. Results showed correction of epidermal architecture in both OTCs and regenerated skin grafts. Importantly, the results from corrected skin grafts indicated that even where detectable LEKTI expression was restored to a limited numbers of cells, a wider bystander benefit occurred around these small populations. As LEKTI is a secreted protein, the genetically modified graft may provide not only an immediate local protective barrier, but also act as a source of secreted LEKTI providing a generalized benefit following ex-vivo gene therapy.

Di, Wei-Li; Larcher, Fernado; Semenova, Ekaterina; Talbot, Gill E; Harper, John I; Del Rio, Marcela; Thrasher, Adrian J; Qasim, Waseem

2011-01-01

355

Surfactant replacement therapy for non-respiratory distress syndrome neonatal respiratory disease —research or clinical application?  

Microsoft Academic Search

Research studies have highlighted both physiological and pathological evidence to incriminate surfactant abnormality and\\/or deficiency in many neonatal respiratory diseases. Data from animal models and clinical studies support the concept that surfactant replacement therapy (SRT) may have a role to play in such problems. There is now, therefore, a need to perform further randomized controlled trials to assess the appropriate

Anne Greenough

1995-01-01

356

Cognitive-Behavioral Therapy for Somatization and Symptom Syndromes: A Critical Review of Controlled Clinical Trials  

Microsoft Academic Search

Objective: Few treatments for somatization have been proven effective. In the past decade, however, clinical trials of cognitive-behavioral therapy (CBT) have been promising. Our aim was to critically review and synthesize the evidence from these trials. Methods: A search of the Medline database from 1966 through July 1999 was conducted to identify controlled trials designed to evaluate the efficacy of

Kurt Kroenke; Ralph Swindle

2000-01-01

357

Effective intravenous immunoglobulin therapy for Churg-Strauss syndrome (allergic granulomatous angiitis) complicated by neuropathy of the eighth cranial nerve: a case report  

PubMed Central

Introduction We report the case of a patient with Churg-Strauss syndrome with eighth cranial nerve palsy. Vestibulocochlear nerve palsy is extremely rare in Churg-Strauss syndrome. To the best of our knowledge, only one case of complicated neuropathy of the eighth cranial nerve has been described in a previous report presenting an aggregate calculation, but no differentiation between polyarteritis nodosa and Churg-Strauss syndrome was made. High-dose immunoglobulin was administered to our patient, and her neuropathy of the eighth cranial nerve showed improvement. Case presentation At the age of 46, a Japanese woman developed Churg-Strauss syndrome that later became stable with low-dose prednisolone treatment. At the age of 52, she developed sudden difficulty of hearing in her left ear, persistent severe rotary vertigo, and mononeuritis multiplex. At admission, bilateral perceptive deafness of about 80dB and eosinophilia of 4123/?L in peripheral blood were found. A diagnosis of cranial neuropathy of the eighth cranial nerve associated with exacerbated Churg-Strauss syndrome was made. Although high doses of steroid therapy alleviated the inflammatory symptoms and markers, the vertigo and bilateral hearing loss remained. Addition of a high-dose immunoglobulin finally resulted in marked alleviation of the symptoms associated with neuropathy of the eighth cranial nerve. Conclusions A high dose of immunoglobulin therapy shows favorable effects in neuropathy of the eighth cranial nerve, but no reports regarding its efficacy in cranial neuropathy have been published.

2012-01-01

358

Persistence and adherence in the treatment of overactive bladder syndrome with anticholinergic therapy: a systematic review of the literature.  

PubMed

Overactive bladder syndrome (OAB) is a chronic condition that has an impact on patients' daily activities and health-related quality of life (HRQL). Anticholinergic therapy is often prescribed following insufficient results with behaviour modification alone; however, rates of treatment discontinuation are consistently high. This study systematically reviewed persistence and adherence data in patients with OAB treated with anticholinergic therapy. A search focused on the intersection of OAB, persistence/adherence, and anticholinergic therapy was conducted in MEDLINE and EMBASE. Articles published after 1998 were reviewed and selected for inclusion based on prespecified criteria. A total of 147 articles and two abstracts were included in the review. Results from 12-week clinical trials showed high rates of discontinuation, ranging from 4% to 31% and 5% to 20% in treatment and placebo groups, respectively. Unsurprisingly, rates of discontinuation found in medical claims studies were substantially higher, with 43% to 83% of patients discontinuing medication within the first 30 days and rates continuing to rise over time. Findings from medical claims studies also suggest that over half of patients never refill their initial prescription and that adherence levels tend to be low, with mean/median medication possession ratio (MPR) values ranging from 0.30 to 0.83. The low levels of persistence and adherence documented in this review reveal cause for concern about the balance between the efficacy and tolerability of anticholinergic agents. Strategies should be identified to increase persistence and adherence. New agents and non-pharmacologic alternatives with good efficacy and minimal side effects should be explored. PMID:21489081

Sexton, C C; Notte, S M; Maroulis, C; Dmochowski, R R; Cardozo, L; Subramanian, D; Coyne, K S

2011-05-01

359

Lipid emulsion therapy in cardiodepressive syndrome after diltiazem overdose--case report.  

PubMed

We present a case of diltiazem overdose in which the patient ingested 5.6 g in an apparent suicide attempt. She was admitted in the emergency department 2 hours postingestion with cardiodepressive syndrome. She was treated with gastric lavage, activated charcoal, intravenous fluids, calcium, and epinephrine, without improvement in vital signs. We gave her an infusion of 20% intralipid, leading to a favorable evolution. The patient was stable hemodynamically and metabolic in the following 24 hours. She was alert and oriented and was extubated in the second day. She was discharged after 4 days in a good state and without any neurologic deficits. PMID:23685061

Bologa, Cristina; Lionte, Catalina; Coman, Adorata; Sorodoc, Laurentiu

2013-05-16

360

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

PubMed Central

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

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

2006-01-01

361

Pharmacoeconomics of therapy for Guillain-Barré syndrome: plasma exchange and intravenous immunoglobulin.  

PubMed

Guillain-Barré syndrome (GBS) is an acute neuropathy and a clinical syndrome that includes a number of pathological and electrophysiological subtypes. Intravenous immunoglobulin (IVIG) and plasma exchange (PE) are both equally efficacious for the treatment of GBS; however, the cost of IVIG may be lower for both the patient and the healthcare system. To compare the pharmacoeconomics of PE and IVIG in GBS, a retrospective study was done from 1999 to 2004, which included a total of 24 patients with GBS who were admitted to Taipei Veterans General Hospital. This showed that except for the costs of the drugs used in IVIG, treatment of GBS with IVIG was more cost-effective (p=0.057) than that with PE in total length of hospitalization and the cost of procedures and hospitalization. The study also showed that the total costs were higher for patients on ventilators than those not requiring ventilators (p=0.008, t-test) and the length of hospitalization showed a very strong linear relationship to total costs (Pearson correlation coefficient=0.907). The regression analysis showed that each additional day of hospitalization increased the hospitalization costs by an average of 5599 New Taiwan Dollars (NT) (US$1.00=NT$33.50 in 2005). PMID:17532498

Tsai, Ching-Piao; Wang, Kai-Chen; Liu, Chih-Yang; Sheng, Wen-Yung; Lee, Tzu-Chi

2007-07-01

362

[Critical state medicine. Surfactant therapy of adult respiratory distress syndrome (results of multicenter studies)].  

PubMed

The paper provides evidence for the pathogenetic approach to treating acute lung lesion (ALL) and adult respiratory distress syndrome (ARDS). An algorithm of the use of Russian lung surfactant preparations: CT-HL and CT-BL has been developed. In involves earlier (the first days following the onset of respiratory failure) use of surfactant, its combined bolus intratracheal or intrabronchial administration in doses of 200-400 mg/m2, followed by continuous (5-day) aerosol inhalation in doses of 20-30 mg/h for children and 30-75 mg/h for adults until pronounced clinical and X-ray effects are shown. Fifty three patients were found to develop ALL and ARDS in the presence of severe pneumonia, postperfusion lung disorders, reperfusion syndrome, pulmonary embolism, long-term artificial ventilation, combined car accident injury and gunshot wounds of the chest, heroine intoxication, septic shock, sepsis, postoperative sequels in cancer patients, and after hepatic transplantation or massive aspiration of gastric contents. Fifty patients were overcome their critical status, 44 survived. The duration of artificial ventilation (AV) ranged from 1 to 6 days. Earlier use of the drugs made it possible to transfer patients to safe AV regimens and to eliminate ALL and ARDS rapidly and to significantly reduce mortality due to critical states. PMID:11510148

Granov, A M; Rozenberg, O A; Tsybul'kin, E K; Erokhin, V V; Khubulava, G G; Likhvantsev, V V; Osovskikh, V V; Bautin, A E; Gavrilin, S V; Kazennov, V V; Zhilin, Iu N; Nabokova, T S; Shefer, S P; Vashkevich, S M; Nefedov, A V; Se?liev, A A; Volchkov, V A

2001-01-01

363

Case report of exercise and statin-fibrate combination therapy-caused myopathy in a patient with metabolic syndrome: contradictions between the two main therapeutic pathways  

PubMed Central

Background Lifestyle modifications including exercise are beneficial and fundamentally part of the therapy of metabolic syndrome, although in most of the cases medical interventions are also required to reach the target values in the laboratory parameters. Statin and fibrate combination therapy is considered to be safe and effective in dyslipidaemia and metabolic syndrome. However, increased physical activity can enhance the statin and fibrate-associated myopathy. Myositis and the rare but life-threatening rhabdomyolysis are causing a conflict between exercise and statin-fibrate therapy, which is yet to be resolved. Case presentation We present a case of a 43-year-old Caucasian man with metabolic syndrome who had the side-effect of exercise and drug-associated myositis. The patient had only transient moderate complaints and rhabdomyolysis could be avoided with the one-month creatine kinase control, a test which is not recommended routinely by the new guidelines. Conclusions We would like to turn the spotlight on the possible complications of statin-fibrate therapy and exercise, when strict follow-up is recommended. In this condition high number of patients can be affected and the responsibility of general practitioners is accentuated.

2013-01-01

364

Rett Syndrome  

MedlinePLUS

... problems Breathing problems Behavior problems Learning problems or intellectual disability Rett syndrome has no cure. You can treat some of the symptoms with medicines, surgery, and physical and speech therapy. Most people with ...

365

Enzyme replacement therapy in a murine model of Morquio A syndrome  

Microsoft Academic Search

Mucopolysaccharidosis IVA (MPS IVA) is an autosomal recessive disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS), leading to accumulation of keratan sulfate (KS) and chrondroitin-6-sulfate. The pharmacokinetics and biodistributions were determined for two recombinant human GALNSs produced in CHO cell lines: native GALNS and sulfatase-modifier-factor 1 (SUMF1) modified GALNS. Preclinical studies of enzyme replacement therapy (ERT) by using two

Shunji Tomatsu; Adriana M. Montano; Amiko Ohashi; Monica A. Gutierrez; Hirotaka Oikawa; Toshihiro Oguma; Vu Chi Dung; Tatsuo Nishioka; Tadao Orii; William S. Sly

2008-01-01

366

[Is there a drug treatment approach for prevention and therapy of fat embolism syndrome?].  

PubMed

The pathogenesis of fat embolism has been subject to considerable debate ever since it was first described by Zenker in 1864. The clinical course, management and pathophysiology of a typical case are described. As no single causative factor has been implied in the development of fat embolism, its therapy and prevention primarily involves the adequate treatment of shock and of the underlying traumatic lesions. PMID:1813724

Schröder, D; Buttenschön, K; Herrmann, F; Brede, S

1991-01-01

367

Die Therapie absetzen? — Anmerkungen zu einem ethischen Problem bei der Begleitung von Menschen mit apallischem Syndrom  

Microsoft Academic Search

\\u000a Seit einigen Jahren wird auf dem Hintergrund unterschiedlicher ethischer Vorstellungen äußerst kontrovers und emotional diskutiert,\\u000a was für Menschen, die sich im apallischen Sydrom befinden, das „Richtige“ sei: Ist es die Weiterführung der medizinischen (Maximal-) Therapie und bedeutet diese nicht für die Betroffenen ein „zeitlich maximales“ Weiterleben mit schwerster Mehrfachbehinderung? Oder\\u000a ist es im Gegenteil der medizinische Therapieverzicht? Aber unterstellt dieser

K. Kahlmann

368

Serious air leak syndrome complicating high-flow nasal cannula therapy: a report of 3 cases.  

PubMed

Despite the absence of clinical safety data, heated, humidified high-flow nasal cannula (HHFNC) therapy is increasingly being used as an alternative to positive-pressure ventilation in pediatrics. This use of HHFNC is "off label" because the US Food and Drug Administration's approval for these devices was only for air humidification and not as a modality to provide positive distending pressure. For the first time we describe 3 cases who developed serious air leaks related to HHFNC therapy. The first child was a previously healthy 2-month-old male infant with respiratory syncytial virus bronchiolitis who developed a right pneumothorax on day 5 of his illness at 8 liters per minute (lpm). He subsequently required intubation and ventilation for 14 days. The second case involved an otherwise healthy 16-year-old boy with cerebral palsy who developed pneumomediastinum and died of its complications. He was receiving 20 lpm HHFNC therapy when he developed pneumomediastinum. The third case involved a 22-month-old, previously healthy boy who developed subdural hematoma secondary to abuse. He developed a right pneumothorax while receiving HHFNC at a flow of 6 lpm, requiring chest tube placement. These cases emphasize the need for extreme caution while using HHFNC for the off-label indication of providing positive distending pressure in children, especially at flows higher than the patient's minute ventilation. A more detailed study to specifically look at the serious adverse events related to HHFNC is urgently needed. PMID:23382446

Hegde, Satyanarayan; Prodhan, Parthak

2013-02-04

369

Monte-Carlo simulation of primary stochastic effects induced at the cellular level in boron neutron capture therapy  

NASA Astrophysics Data System (ADS)

A Monte Carlo code is developed to study the action of particles in Boron Neutron Capture Therapy (BNCT). Our aim is to calculate the probability of dissipating a lethal dose in cell nuclei. Cytoplasmic and nuclear membranes are considered as non-concentric ellipsoids. All geometrical parameters may be adjusted to fit actual configurations. The reactions 10B(n,??)^7Li and 14N(n,p)14C create heavy ions which slow down losing their energy. Their trajectories can be simulated taking into account path length straggling. The contribution of each reaction to the deposited dose in different cellular compartments can be studied and analysed for any distribution of 10B. Un code de simulation Monte-Carlo est développé pour étudier les modalités d'action de la Thérapie par Capture de Neutrons (TCN) sur le {10}B. L'objectif est le calcul de la probabilité de dépôt d'une dose létale dans les noyaux cellulaires. Les membranes cytoplasmique et nucléaire sont schématisées par des ellipsoïdes non concentriques dont tous les paramètres sont ajustables à des configurations réelles. Les réactions considérées, 10B(n,??)7 et 14N(n,p)14, produisent des ions dont les trajectoires peuvent être simulées en considérant les fluctuations sur les longueurs de parcours. Les contributions respectives de chaque réaction aux doses déposées dans les divers compartiments cellulaires peuvent être étudiées et analysées en fonction des distributions de 10B.

Cirioni, L.; Patau, J. P.; Nepveu, F.

1998-04-01

370

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

PubMed

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

Reusch, C; Hähnle, B

1991-02-01

371

Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy.  

PubMed

A 55-year-old Caribbean woman with a 6-year history of smoldering adult T-cell leukemia/lymphoma presented with clinical and biological symptoms of hemophagocytic syndrome. An extensive search for infectious diseases was negative. A lymph node biopsy showing large T-cell lymphoma (CD4-, CD25+) and findings of high LDH count and severe lymphocytosis led to the diagnosis of acute adult T-cell leukemia/lymphoma. Anti-retroviral therapy combining zidovudine, lamivudine, and interferon-alpha was started, resulting in rapid control of both hemophagocytic syndrome and symptoms of acute adult T-cell leukemia/lymphoma. Thus, we propose that adult T-cell leukemia/lymphoma must be added to the spectrum of etiologies of hemophagocytic syndrome. PMID:15164389

Aouba, Achille; Lambotte, Olivier; Vasiliu, Viorel; Divine, Marine; Valensi, Françoise; Varet, Bruno; Bazarbachi, Ali; Hermine, Olivier

2004-06-01

372

Early Changes in Clinical Characteristics after Emergency Department Therapy for Acute Heart Failure Syndromes: Identifying Patients who Do Not Respond to Standard Therapy  

PubMed Central

Aims Clinical trials for acute heart failure syndromes (AHFS) have traditionally enrolled patients well after emergency department (ED) presentation. We hypothesized a large proportion of patients would undergo changes in clinical profiles during the first 24 hours of hospitalization and these changes would be associated with adverse events. Methods We evaluated a prospective cohort of patients with clinical data available at ED presentation and 12–24 hours after ED treatment for AHFS. Patients were categorized into distinct clinical profiles at these time points based on: 1) systolic blood pressure- a. hypertensive [>160 mmHg]; b. normotensive [100–159 mmHg]; or c. hypotensive [<100 mmHg]; 2) moderate to severe renal dysfunction [GFR ? 60 ml/min/1.73 m2]; and 3) presence of troponin positivity. A composite outcome of 30-day cardiovascular events was determined by phone follow-up. Results In the 370 patients still hospitalized with data available at the 12–24 hour time point, 196 (53.0%) had changed their clinical profiles, with 117 (59.7%) improving and 79 (40.3%) worsening. The composite 30-day event rate was 16.9%. Patients whose clinical profile started and stayed abnormal had a significantly greater proportion of events than those who started and stayed normal (26.1% vs. 11.3%; p=0.03). Conclusion Patients with abnormal clinical profiles at presentation that remain abnormal throughout the first 12–24 hours of hospitalization are at increased risk of 30-day adverse events. Future clinical trials may need to consider targeting these patients, as they may be the most likely to benefit from experimental therapy.

Collins, Sean P.; Storrow, Alan B.; Fermann, Gregory J.; Peacock, W. Frank; Pang, Peter S.; Weintraub, Neal; Levy, Phillip D.; Sawyer, Douglas B.; Lindsell, Christopher J.; Gheorghiade, Mihai

2012-01-01

373

Neurodevelopmental outcome at 6 years of age after intrauterine laser therapy for twin-twin transfusion syndrome.  

PubMed

This study was undertaken to evaluate neurodevelopmental outcome of children at 6 years of age after intrauterine laser therapy for Twin-twin transfusion syndrome (TTTS). This is part of a longitudinal study in children after intrauterine laser therapy for TTTS; 190 of 254 (74.8%) children, previously investigated at a median age of 2 years 10 months, were re-evaluated at 6 years 5 months (range 4 years 11 months -10 years 4 months). Sixty-four patients were not examined due to loss of contact. The median gestational age at birth was 34 + 3 weeks. The study included a physical/neurological examination, a standardized neurodevelopmental test (Kaufman-ABC) and/or results from the national screening programme for children as well as questionnaires. Patients were grouped in three outcome categories: group I: normal examination and test result; group II: minor neurological deficiencies and normal test results; group III: major neurological deficiencies and/or test results below minus two standard deviations. The following results were obtained at 6 years 5 months (for comparison, results of the same patients at 2 years 10 months in brackets). Group I: 79.5% (84.2%); group II: 11.6% (8.9%); group III: 8.9% (6.8%). Twenty-one (11%) patients had a worse and 8 (4.2%) an improved classification at 6 years 5 months as compared to 2 years 10 months. Overall, the results with 6 years did not significantly differ from the results with 2 years. Neurodevelopmental outcome at 6 years 5 months was not statistically, significantly different from outcome at 2 years 10 months. PMID:22946904

Graeve, Pauline; Banek, Corinna; Stegmann-Woessner, Gaby; Maschke, Cornelia; Hecher, Kurt; Bartmann, Peter

2012-09-29

374

Increased Serum Adipokines Implicate Chronic Inflammation in the Pathogenesis of Overactive Bladder Syndrome Refractory to Antimuscarinic Therapy  

PubMed Central

Objectives Recent studies have shown that chronic inflammation is involved in overactive bladder (OAB) syndrome. OAB could be a subtype of neurogenic inflammation. This pilot study investigated serum adipokine levels in patients with OAB refractory to antimuscarinic therapy. Methods Thirty consecutive patients with OAB-dry (n?=?16) or OAB-wet (n?=?14) refractory to previous antimuscarinic treatment were prospectively enrolled in this study, a group of 26 normal subjects without lower urinary tract symptoms served as controls. Concentrations of serum C-reactive protein (CRP), nerve growth factor (NGF), and adipokines including interleukins ([IL], IL-1?, IL-6, IL-8), tumor necrosis factor (TNF)-?, monocyte chemotactic protein (MCP)-1, insulin, and leptin were quantified using a bead-based human serum adipokine panel B kit. Data were analyzed using the LX 200 platform. Patients were further classified as having dry or wet OAB and having medical diseases or not. The serum CRP, NGF, and adipokine levels were compared between OAB patients and the controls, and between OAB subgroups. Results The serum concentrations of CRP, NGF, IL-1?, IL-6, IL-8, and TNF-? in OAB-dry and OAB-wet patients were significantly higher than among the controls. There was no significant difference in adipokine levels between OAB-dry and OAB-wet, or between OAB patients with and without medical diseases. Serum CRP and NGF levels were significantly higher only in OAB-wet or OAB patients with medical diseases than among controls. The MCP-1 levels, on the other hand, were significantly higher in OAB-dry or OAB patients with disease, than the controls. Conclusions Both OAB-dry and OAB-wet patients showed increased serum CRP, NGF, and adipokine levels compared with the controls, suggesting chronic inflammation of the bladder involving both peripheral and central mechanisms in all OAB patients refractory to antimuscarinic therapy. The increased serum adipokine levels were not relevant to medical diseases.

Liu, Hsin-Tzu; Jiang, Yuan-Hong; Kuo, Hann-Chorng

2013-01-01

375

Use of thromboelastography PlateletMapping to monitor antithrombotic therapy in a patient with Budd-Chiari syndrome.  

PubMed

Budd-Chiari syndrome (BCS) is the end result of a number of disease states resulting in hepatic venous outflow obstruction. We report a Janus kinase 2-homozygous patient with BCS who thrombosed a transjugular intrahepatic portosystemic shunt (TIPS) despite treatment with warfarin (international normalized ratio = 3.0), aspirin, and clopidogrel. PlateletMapping (Haemonetics Corp.) is a novel point-of-care assay of platelet function based on thromboelastography (TEG) that has the ability to detect platelet inhibition (%) by antiplatelet therapy. Initial PlateletMapping traces showed no platelet inhibition by aspirin or clopidogrel but demonstrated adequate suppression of plasmatic coagulation. On this basis, the aspirin dose was doubled, and this resulted in a significant increase in platelet inhibition (45%). To further suppress platelet activity, the patient was started on tirofiban, a glycoprotein IIb/IIIa inhibitor. Repeat PlateletMapping revealed 100% inhibition of platelets by both pathways, and this coincided with angiographic evidence of TIPS blood flow. Subsequently, the patient developed bleeding from the venous access sites. TEG demonstrated poor underlying plasmatic coagulation with a prolonged R time of 9.2 minutes (normal = 2-8 minutes), and the international normalized ratio was found to be supratherapeutic (>4). Treatment with fresh frozen plasma stopped the bleeding without compromising the platelet inhibition. This case demonstrates that increased platelet activation may contribute to the development of thromboses in BCS. Despite the standard dose of dual antiplatelet therapy, there was minimal inhibition in platelet function, and anticoagulation with warfarin alone was not adequate to prevent thrombotic events. PlateletMapping was used to assess and then optimize the antiplatelet treatment while facilitating the management of complications without an increased risk of thrombosis. PMID:20035517

James, Katherine; Bertoja, Elisa; O'Beirne, James; Mallett, Susan

2010-01-01

376

330. Lentiviral Vector Mediated Hematopoietic Stem Cell Gene Therapy Combined with Non-Lethal Conditioning Restores T Cell Function in the Murine Model of Wiskott-Aldrich Syndrome  

Microsoft Academic Search

Wiskott-Aldrich syndrome (WAS) is a severe X-linked immunodeficiency characterized by recurrent infections, thrombocytopenia, eczema and increased risk of autoimmune disorders and lymphomas. The present therapeutic strategy is based on transplantation of bone marrow from HLA-identical sibling donors. Because of the limited availability of such donors, the development of alternative approaches, such as hematopoietic stem cells (HSC) gene therapy is highly

Francesco Marangoni; Loïc Dupré; Samantha Scaramuzza; Sara Trifari; Raisa Jofra Hernández; Adrian Thrasher; Anne Galy; Alessandro Aiuti; Luigi Naldini; Maria Grazia Roncarolo

2005-01-01

377

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

378

A possible chemopreventive role for photodynamic therapy in Gorlin syndrome: a report of basal cell carcinoma reduction and review of literature.  

PubMed

Multiple basal cell carcinomas (BCC) are a significant cause of morbidity and disfigurement in patients with naevoid basal cell carcinoma syndrome (NBCCS). Chemopreventive strategies are needed to reduce the formation of new BCC in these patients. Appropriate evidence-based guidelines for photodynamic therapy chemoprevention in NBCCS do not exist. We herein report one patient treated with methyl aminolevulinate PDT with red light (630?nm) activation to continue sustained chemoprevention following other BCC chemopreventive modalities and a relevant literature review. PMID:22780558

Wolfe, Christopher M; Green, W Harris; Cognetta, Armand B; Hatfield, H Keith

2012-07-11

379

First experience of enzyme replacement therapy with idursulfase in Spanish patients with Hunter syndrome under 5 years of age: Case observations from the Hunter Outcome Survey (HOS)  

Microsoft Academic Search

Hunter syndrome (mucopolysaccharidosis type II [MPS II], OMIM309900) is a rare X-linked lysosomal storage disorder caused by deficiency of the enzyme iduronate-2-sulphatase, resulting in accumulation of glycosaminoglycans (GAGs), multisystem organ failure and early death. Enzyme replacement therapy (ERT) with idursulfase is commercially available since 2007. Early access programs were established since 2005. However, limited information on the effects of ERT

C. Alcalde-Martín; J. M. Muro-Tudelilla; R. Cancho-Candela; L. González Gutiérrez-Solana; G. Pintos-Morell; M. Martí-Herrero; P. Munguira-Aguado; E. Galán-Gómez

2010-01-01

380

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

381

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

César Fernández-de-las-Peñas; Joshua A. Cleland; Ricardo Ortega-Santiago; Ana Isabel de-la-Llave-Rincon; Almudena Martínez-Perez; Juan A. Pareja

2010-01-01

382

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

2013-04-03

383

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

384

A-type lamins and Hutchinson-Gilford progeria syndrome: pathogenesis and therapy.  

PubMed

Lamin A and lamin C (A-type lamins, both encoded by the LMNA gene) are major components of the mammalian nuclear lamina, a complex proteinaceous structure that acts as a scaffold for protein complexes that regulate nuclear structure and function. Abnormal accumulation of farnesylated-progerin, a mutant form of prelamin A, plays a key role in the pathogenesis of the Hutchinson-Gilford progeria syndrome (HGPS), a devastating disorder that causes the death of affected children at an average age of 13.5 years, predominantly from premature atherosclerosis and myocardial infarction or stroke. Remarkably, progerin is also present in normal cells and appears to progressively accumulate during aging of non-HGPS cells. Therefore, understanding how this mutant form of lamin A provokes HGPS may shed significant insight into physiological aging. In this review, we discuss recent advances into the pathogenic mechanisms underlying HGPS, the main murine models of the disease, and the therapeutic strategies developed in cellular and animal models with the aim of reducing the accumulation of farnesylated-progerin, as well as their use in clinical trials of HGPS. PMID:21622261

Gonzalez, Jose M; Pla, Davinia; Perez-Sala, Dolores; Andres, Vicente

2011-06-01

385

The role of platelet activation in determining response to therapy in patients with primary nephrotic syndrome.  

PubMed

To test the role of platelet activation in the prognosis of nephrotic syndrome (NS), we evaluated the mean platelet volume (MPV) in patients with NS undergoing treatment. In this prospective, multicenter clinical study 156 patients with primary NS under treatment were assigned and followed for one year. Patients were divided into three groups for complete remission, partial remission, and resistance. Biochemical parameters, estimated glomerular filtration rate, proteinuria level, and MPV levels were compared at baseline and 12 months after treatment. MPV, proteinuria, total cholesterol, triglyceride, LDL cholesterol, HDL cholesterol, total protein, albumin, and hs-CRP levels significantly decreased in partial and complete remission group after 12 months compared to the baseline (p?

Kocyigit, Ismail; Yilmaz, Mahmut Ilker; Sim?ek, Yasin; Unal, Aydin; Sipahioglu, Murat Hayri; Eroglu, Eray; Dede, Fatih; Tokgoz, Bulent; Oymak, Oktay; Utas, Cengiz

2012-11-13

386

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.

Hernandez-Yero, Arturo; Santana Perez, Felipe; Ovies Carballo, Gisel; Cabrera-Rode, Eduardo

2012-01-01

387

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; Ró?d?y?ska-?wi?tkowska, Agnieszka; Tylki-Szyma?ska, Anna

2012-12-01

388

Quality of life among people living with acquired immune deficiency syndrome receiving anti-retroviral therapy: a study from Nepal  

PubMed Central

Purpose The present study was undertaken to determine the impact of acquired immune deficiency syndrome (AIDS) on the quality of life of affected individuals in Nepal. Patients and methods A cross sectional study was done among 70 individuals attending the Anti-Retroviral Therapy clinic of the University Hospital in Nepal. Quality of life (QOL) was evaluated using World Health Organization Quality of life questionnaire (WHO QOL-BREF) instrument. Statistical analysis was done using SPSS Version 17.0. Results The median scores with interquartile range (IQR) in four domains of QOL in descending order were physical (61; IQR 22), social (58; IQR 33), environmental (56; IQR 13), and psychological (54; IQR 8). Older age was associated with lower perceived overall QOL. Females were more likely to have lower QOL scores in the social and psychological domains. Higher CD4 counts and a married status were significant predictors of higher QOL scores in the environmental domain. Conclusion Being older, female, single, and having advanced clinical stage is associated with lower QOL scores in people living with AIDS. Lowest QOL scores were seen in the psychological domain suggesting the need of psychological interventions.

Giri, Smith; Neupane, Maniraj; Pant, Sushil; Timalsina, Utsav; Koirala, Sagar; Timalsina, Santosh; Sharma, Sashi

2013-01-01

389

Effects of nCPAP therapy on cardiorespiratory outcomes in obstructive sleep apnea syndrome: compliance and technological advancements.  

PubMed

Obstructive sleep apnea syndrome (OSAS) is an independent and modifiable risk factor for cardiovascular diseases; however, the pathophysiological mechanisms underlying this association are not yet fully understood. Intermittent hypoxemia, one of the physiological markers of OSAS, is characterized by transient periods of oxygen desaturation followed by reoxygenation. The hypoxia-reoxygenation cycles are associated with oxidative stress that, in turn, triggers the activation of pathways that lead to cardiovascular damage. The results of several studies show that OSAS causes oxidative stress and that nasal continuous positive airway pressure therapy normalizes these biological abnormalities. In conclusion, treatment of OSAS with continuous positive airway pressure may lower cardiovascular risk by reducing sympathetic nerve activity, ambulatory blood pressure and arterial stiffness, and by increasing sensitivity of the arterial baroreflex. Newer modalities such as C-Flex and A-Flex also show promise as treatment options in the future. However, the evidence supporting the use of these alternative modalities remains scant, in particular with regards to long-term cardiovascular outcomes. PMID:21348585

Toraldo, Domenico M; De Nuccio, Francesco; Nicolardi, Giuseppe

2011-02-01

390

Directed Therapy for Patients with Myelodysplastic Syndromes (MDS) by Suppression of Cyclin D1 with ON 01910.Na  

PubMed Central

Background We previously demonstrated upregulation of c-myc, survivin, and cyclin D1 in CD34+ bone marrow mononuclear cells (BMMNCs) of patients with trisomy 8 and monosomy 7 myelodysplastic syndromes (MDS). “Knockdown” of cyclin D1 by RNA interference decreased trisomy 8 cell growth, suggesting that this might be a therapeutic target in MDS. Experimental Design We performed preclinical studies using BMMNCs from patients with MDS and AML to examine the effects of the styryl sulfone ON 01910.Na on cyclin D1 accumulation, aneuploidy, and CD34+ blast percentage. We next treated twelve patients with higher risk MDS and two trisomy 8 AML patients with ON01910.Na on a phase I clinical protocol (NCT00533416). Results ON 01910.Na inhibited cyclin D1 expression, and was selectively toxic to trisomy 8 cells in vitro. Flow cytometry studies demonstrated increased mature CD15+ myeloid cells and decreased CD34+ blasts. Three patients treated with ON01910.Na on a clinical had decreased bone marrow blasts by ?50%, and three patients had hematologic improvements, one of which was sustained for 33 months. Patients with hematologic responses to ON 01910.Na had decreased cyclin D1 expression in their CD34+ cells. Conclusions The preclinical results and responses of patients on a clinical trial warrant further investigation of ON 01910.Na as a potential novel targeted therapy for higher risk MDS patients.

Olnes, Matthew J.; Shenoy, Aarthie; Weinstein, Barbara; Pfannes, Loretta; Loeliger, Kelsey; Tucker, Zachary; Tian, Xin; Kwak, Minjung; Wilhelm, Francois; Yong, Agnes S.M.; Maric, Irina; Maniar, Manoj; Scheinberg, Phillip; Groopman, Jerome; Young, Neal S.; Sloand, Elaine M.

2012-01-01

391

Disease correction by combined neonatal intracranial AAV and systemic lentiviral gene therapy in Sanfilippo Syndrome type B mice.  

PubMed

Mucopolysaccharidosis type IIIB (MPS IIIB) or Sanfilippo Syndrome type B is a lysosomal storage disease resulting from the deficiency of N-acetyl glucosaminidase (NAGLU) activity. We previously showed that intracranial adeno-associated virus (AAV)-based gene therapy results in partial improvements of several aspects of the disease. In an attempt to further correct the disease, MPS IIIB mice were treated at 2-4 days of age with intracranial AAV2/5-NAGLU (IC-AAV), intravenous lentiviral-NAGLU (IV-LENTI) or the combination of both (BOTH). The BOTH group had the most complete biochemical and histological improvements of any treatment group. Compared with untreated MPS IIIB animals, all treatments resulted in significant improvements in motor function (rotarod) and hearing (auditory-evoked brainstem response). In addition, each treatment group had a significantly increased median life span compared with the untreated group (322 days). The combination arm had the greatest increase (612 days), followed by IC-AAV (463 days) and IV-LENTI (358 days). Finally, the BOTH group had nearly normal circadian rhythm measures with improvement in time to activity onset. In summary, targeting both the systemic and central nervous system disease of MPS IIIB early in life appears to be the most efficacious approach for this inherited metabolic disorder. PMID:23535899

Heldermon, C D; Qin, E Y; Ohlemiller, K K; Herzog, E D; Brown, J R; Vogler, C; Hou, W; Orrock, J L; Crawford, B E; Sands, M S

2013-03-28

392

Growth and Growth Hormone Therapy in Turner Syndrome (Groei en Groeihormoon Behandeling bij Patienten met het Syndroom van Turner).  

National Technical Information Service (NTIS)

Turner syndrome is one of the most common chromosomal disorders. Short stature is, in addition to gonadal dysgenesis and several dysmorphic features, one of the main characteristics of Turner syndrome. The growth pattern of Dutch patients with Turner synd...

C. Rongen-Westerlaken

1992-01-01

393

Mode of onset of ventricular fibrillation in patients with Brugada syndrome detected by implantable cardioverter defibrillator therapy  

Microsoft Academic Search

OBJECTIVESWe sought to demonstrate the mode of spontaneous onset of ventricular fibrillation (VF) in patients with Brugada syndrome.BACKGROUNDThe electrophysiologic mechanisms of VF in Brugada syndrome have not been fully investigated.METHODSNineteen patients (all male, mean age 47 ± 12 years) with Brugada syndrome were treated with an implantable cardioverter defibrillator (ICD). The implanted devices were capable of storing electrograms during an

Mikio Kakishita; Takashi Kurita; Kiyotaka Matsuo; Atsushi Taguchi; Kazuhiro Suyama; Wataru Shimizu; Naohiko Aihara; Shiro Kamakura; Fumio Yamamoto; Junjiro Kobayashi; Yoshio Kosakai; Tohru Ohe

2000-01-01

394

Compartment syndrome of the thigh. A case report with delayed onset after stable pelvic ring fracture and chronic anticoagulation therapie  

Microsoft Academic Search

Compartment syndrome of the thigh is a rare occurrence potentially leading to devastating functional restrictions. There is a wide spectrum of reported conditions leading to increased tissue pressure in the thigh possibly resulting in a compartment syndrome, ranging from deep venous thrombosis to blunt injuries and femoral fractures. We report a case of a delayed development of a compartment syndrome

Rolf D Burghardt; Sebastian Siebenlist; Stefan Döbele; Martin Lucke; Ulrich Stöckle

2010-01-01

395

Novel therapies for the management of short bowel syndrome in children.  

PubMed

Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. It is defined as the inability to maintain adequate nutrition enterally as a result of a major loss of the small intestine. SBS is a life-threatening entity associated with potential significant morbidity and mortality. The etiology in the pediatric age group includes necrotizing enterocolitis (32 %), atresia (20 %), volvulus (18 %), gastroschisis (17 %), and aganglionosis (6 %). It is characterized by substrate malabsorption, electrolyte imbalance, intestinal bacterial overgrowth, steatorrhea, and weight loss. Current medical management includes parenteral nutrition, progressive feeds as tolerated, various medications, and surgical manipulations. However, frequently this management is not successful in achieving the goal of attaining normal growth and development without parenteral nutrition. It has been known for decades that there is a normal physiologic response of the residual intestine to massive bowel resection referred to as intestinal adaptation. The mechanisms that control this process are unknown. Unfortunately, intestinal adaptation and the current management are not always successful. As a result of new knowledge regarding the pathophysiology of SBS over the past two decades, several novel strategies have been developed in experimental animal models as well as limited clinical trials in infants and children. They can be divided into several categories that potentially influence intestinal (1) absorption, (2) secretion, (3) motility, and (4) adaptation. More recently, newer modalities have been studied including small intestine transplantation, and the use of specific intestinal growth factors. Ultimately, tissue and organ engineering will become the treatment for infants and children with SBS. PMID:23989526

Schwartz, Marshall Z

2013-10-01

396

Fragile X Syndrome.  

ERIC Educational Resources Information Center

|Physical, psychological, and cytogenic characteristics of individuals with the Fragile X syndrome are reviewed. Prospects for therapy with folic acid, prenatal diagnosis, phenotype of heterozygote for the marker X, and unresolved issues about the syndrome are discussed. (CL)|

de la Cruz, Felix F.

1985-01-01

397

Thrombolytic therapy for cerebral vein thrombosis in antiphospholipid syndrome secondary to systemic lupus erythematosus.  

PubMed

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

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

2012-03-01

398

Role of continuous positive airway pressure therapy on the pathogenesis of sleep-related frontal lobe epilepsy in a child with obstructive sleep apnea syndrome.  

PubMed

We describe an obese child with severe obstructive sleep apnea syndrome in whom nocturnal frontal lobe seizures developed within a week after therapy was started with continuous positive airway pressure. The video polysomnographic study after the onset of nocturnal episodes showed 3 seizures: 2 starting from slow-wave sleep when he was sleeping with continuous positive airway pressure, and 1 from stage 2 non-rapid eye movement sleep when he was sleeping without continuous positive airway pressure. Cyclic alternating pattern analysis during the video polysomnography recorded after the onset of nocturnal seizures disclosed a high cyclic alternating pattern rate during slow-wave sleep, and the recording obtained after antiepileptic therapy began showed a low cyclic pattern analysis rate. In this child, we describe the non-rapid eye movement sleep instability induced by continuous positive airway pressure therapy might have had a role in triggering the nocturnal seizures. PMID:18079319

Miano, Silvia; Pelliccia, Andrea; Evangelisti, Melania; Pagani, Jacopo; Villa, Maria Pia

2007-12-13

399

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

PubMed Central

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

2012-01-01

400

Enzyme replacement therapy in a murine model of Morquio A syndrome.  

PubMed

Mucopolysaccharidosis IVA (MPS IVA) is an autosomal recessive disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS), leading to accumulation of keratan sulfate (KS) and chrondroitin-6-sulfate. The pharmacokinetics and biodistributions were determined for two recombinant human GALNSs produced in CHO cell lines: native GALNS and sulfatase-modifier-factor 1 (SUMF1) modified GALNS. Preclinical studies of enzyme replacement therapy (ERT) by using two GALNS enzymes were performed on MPS IVA mice. The half-lives in blood circulation of two phosphorylated GALNS enzymes were similar (native, 2.4 min; SUMF1, 3.3 min). After intravenous doses of 250 units/g body weight were administered, each enzyme was primarily recovered in liver and spleen, with detectable activity in other tissues including bone and bone marrow. At 4 h post-injection, enzyme activity was retained in the liver, spleen, bone and bone marrow at levels that were 20-850% of enzyme activity in the wild-type mice. After intravenous doses of 250 units/g of native GALNS, and 250, 600 or 1000 units/g of SUMF1-GALNS were administered weekly for 12 weeks, MPS IVA mice showed marked reduction of storage in visceral organs, sinus lining cells in bone marrow, heart valves, ligaments and connective tissues. A dose-dependent clearance of storage material was observed in brain. The blood KS level assayed by tandem mass spectrometry was reduced nearly to normal level. These preclinical studies demonstrate the clearance of tissue and blood KS by administered GALNS, providing the in vivo rationale for the design of ERT trials in MPS IVA. PMID:18056156

Tomatsu, Shunji; Montaño, Adriana M; Ohashi, Amiko; Gutierrez, Monica A; Oikawa, Hirotaka; Oguma, Toshihiro; Dung, Vu Chi; Nishioka, Tatsuo; Orii, Tadao; Sly, William S

2007-12-03

401

The evolution of antiplatelet therapy in the treatment of acute coronary syndromes: from aspirin to the present day.  

PubMed

Our knowledge of the mechanisms of platelet-mediated thrombosis has increased dramatically over the last 40 years. This increased understanding has identified treatment strategies for acute coronary syndromes (ACS) by targeting key mediators of platelet activation and aggregation processes. Aspirin (acetylsalicylic acid) monotherapy improves patient outcomes by irreversibly inhibiting the cyclooxygenase (COX)-1 enzyme in the arachidonic acid pathway. The later-developed thienopyridines, prodrugs that irreversibly inhibit the P2Y(12) receptor, and therefore adenosine diphosphate (ADP) binding, further enhance platelet inhibition and patient outcomes. The thienopyridine clopidogrel has been the standard of care, but it is limited by variable response and treatment failure. A more potent thienopyridine, prasugrel, requires fewer hepatic metabolic steps for activation, and elicits significantly improved outcomes for patients with ACS. The increased potency of prasugrel is associated with an increase in Thrombolysis in Myocardial Infarction (TIMI)-defined major bleeding compared with clopidogrel. Ticagrelor represents a new chemical class of agents called the cyclopentyltriazolopyrimidines. It interacts reversibly with the platelet P2Y(12) receptor, and does not require metabolic bioactivation for activity. Data show a significant improvement in ischaemic outcomes, including mortality, for ticagrelor compared with clopidogrel, without an increase in overall major bleeding, although non-coronary artery bypass graft bleeding is increased. Glycoprotein IIb/IIIa targeted agents (abciximab, tirofiban and eptifibatide) are also used in ACS patients undergoing percutaneous coronary interventions. These inhibitors utilize a different mechanism of action by preventing fibrinogen-mediated platelet aggregation. Other therapeutic strategies for platelet inhibition are being evaluated, including the investigative protease-activated receptor (PAR)-1 and thromboxane A(2) antagonists. This review highlights the mechanisms of action of these agents, and the continuing evolution of ACS therapy. PMID:23083110

Angiolillo, Dominick J

2012-11-12

402

Efficacy and safety of oral tolvaptan therapy in patients with the syndrome of inappropriate antidiuretic hormone secretion  

PubMed Central

Objective Tolvaptan, an oral antagonist of the vasopressin V2 receptor, has been found to improve hyponatremia in patients with mixed etiologies. This study analyzed a subgroup of patients with the syndrome of inappropriate antidiuretic hormone secretion (SIADH) to evaluate the efficacy and safety of tolvaptan in this group. Design and patients Hyponatremic patients in the SALT-1 and SALT-2 studies with a diagnosis of SIADH were identified based on clinical diagnosis by individual study investigators. Subjects were randomized to receive oral placebo (n=52) or tolvaptan 15 mg daily, with further titration to 30 and 60 mg daily, if necessary, based on the response of serum [Na+] (n=58). Results In patients with SIADH, improvement in serum [Na+] was significantly greater (P<0.0001) with tolvaptan than placebo over the first 4 days of therapy as well as the entire 30-day study, with minimal side effects of increased thirst, dry mouth, and urination. Only 5.9% of tolvaptan-treated patients had overly rapid correction of hyponatremia as defined by current guidelines. After discontinuation of tolvaptan, serum [Na+] declined to values similar to placebo. A significant positive treatment effect favoring tolvaptan on the physical component, and a near-significant trend on the mental component, was found using the SF-12 Health Survey. Tolvaptan was associated with a significantly reduced incidence of fluid restriction. Conclusions Results for the SIADH subgroup were analogous to those of the combined SALT population regarding efficacy and safety but demonstrated a greater improvement in the physical component of the SF-12 Health Survey than in the full mixed etiology SALT patient group.

Verbalis, Joseph G; Adler, Suzanne; Schrier, Robert W; Berl, Tomas; Zhao, Qiong; Czerwiec, Frank S

2013-01-01

403

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.

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

404

Adherence to Nasal Positive Airway Pressure Therapy Among School-aged Children and Adolescents With Obstructive Sleep Apnea Syndrome  

Microsoft Academic Search

OBJECTIVES.Although many children with obstructive sleep apnea syndrome have complete resolution of obstructive sleep apnea syndrome after adenotonsillec- tomy, some patients have persistent obstructive sleep apnea syndrome requiring positive airway pressure treatment. Little is known about positive airway pressure adherence among school-aged children and adolescents. PATIENTS AND METHODS.We retrospectively reviewed records from January 2000 through December 2004 to assess positive

Elizabeth C. Uong; Mary Epperson; Sharon A. Bathon; Donna B. Jeffe

2010-01-01

405

Hospital Performance and Differences by Kidney Function in the Use of Recommended Therapies After Non-ST-Elevation Acute Coronary Syndromes  

PubMed Central

Background Chronic kidney disease (CKD) is associated with an increased risk of cardiac events and death, yet underuse of guideline-recommended therapies is widespread. The extent to which hospital performance affects the care of patients with CKD and non–ST-segment elevation acute coronary syndromes (NSTE ACS) is unknown. Study Design Observational cohort. Setting & Participants 81,374 patients with NSTE ACS treated at 327 US hospitals. Predictor Hospital performance, as measured by quartiles of composite adherence to American Heart Association class I guidelines for therapy acutely (aspirin, beta-blockers, clopidogrel, heparin, glycoprotein IIb/IIIa inhibitors) and at discharge (aspirin, clopidogrel, angiotensin-converting enzyme inhibitors, lipid-lowering agents) in eligible patients. Outcomes & Measurements Use of each American Heart Association class I acute and discharge therapy, stratified by continuous estimated glomerular filtration rate (eGFR). Multivariable models were adjusted for demographics, clinical factors, and hospital features. Results Better performing hospitals had lower prescribing rates for most therapies (5 of 9) with lower levels of kidney function while lower performing hospitals were more likely to have similar prescribing rates across the eGFR spectrum, suggesting that prescribing patterns at these hospitals were insensitive to differences in eGFR. Limitations Observational design, selection bias of study cohort. Conclusion Patients with lower levels of kidney function admitted with NSTE ACS are less likely to receive evidence-based therapies. Treatment disparities related to CKD are most evident at top-performing hospitals.

Patel, Uptal D.; Ou, Fang-Shu; Ohman, E. Magnus; Gibler, W. Brian; Pollack, Charles V.; Peterson, Eric D.; Roe, Matthew T.

2009-01-01

406

An economic evaluation of laparoscopic ovarian diathermy versus gonadotrophin therapy for women with clomiphene citrate resistant polycystic ovary syndrome  

Microsoft Academic Search

BACKGROUND: Laparoscopic ovarian diathermy and gonadotrophin ovulation induction for women with clomiphene citrate resistant polycystic ovary syndrome have been shown to result in similar pregnancy rates, but their relative cost-effectiveness has not been evaluated. METHODS: A cost-minimization study was undertaken alongside a randomized controlled trial in women with anovulatory infertility secondary to clomiphene resistant polycystic ovary syndrome. Inclusion criteria were

Cynthia M. Farquhar; Karen Williamson; Paul M. Brown; Jules Garland

2004-01-01

407

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.

Stolinsky, David C.

1980-01-01

408

Lemierre syndrome.  

PubMed

Lemierre's syndrome is an uncommon complication of pharyngitis in the United States and caused most commonly by the bacterium Fusobacterium necrophorum. The syndrome is characterized by a history of recent pharyngitis followed by ipsilateral internal jugular vein thrombosis and metastatic pulmonary abscesses and is a disease for which patients will seek medical care and advice. As most patients are admitted to the hospital under internal medicine, practitioners should be familiar with the usual signs and symptoms of Lemierre's syndrome along with its diagnosis and treatment. Controversy involves the choice and duration of antimicrobial therapy used for treatment and anticoagulation therapy for internal jugular vein thrombosis. As the diagnosis and management of this syndrome has generated controversy, an updated review of the literature and treatment recommendations may be helpful for providing optimal care for patients with this often unrecognized and confusing infection. PMID:22561543

Wright, William F; Shiner, Christine N; Ribes, Julie A

2012-05-01

409

Hyperbaric oxygen therapy for painful bladder syndrome\\/interstitial cystitis resistant to conventional treatments: long-term results of a case series in Japan  

Microsoft Academic Search

Background  There is no confirmed strategy for treating painful bladder syndrome\\/interstitial cystitis (PBS\\/IC) with unclear etiology.\\u000a Therefore, a pilot study was carried out to evaluate the efficacy and safety of hyperbaric oxygen (HBO) therapy in treatment-resistant\\u000a PBS\\/IC patients.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  HBO treatment (2.0 ATA for 60 minutes\\/day × 5 days\\/week for 2 or 4 weeks) was performed on 11 patients with severe symptoms

Tomoaki Tanaka; Yujiro Nitta; Kazuya Morimoto; Noriaki Nishikawa; Chikako Nishihara; Satoshi Tamada; Hidenori Kawashima; Tatsuya Nakatani

2011-01-01

410

Lemierre syndrome complicating multiple brain abscesses caused by extended-spectrum ?-lactamase-producing Klebsiella pneumoniae cured by fosfomycin and meropenem combination therapy.  

PubMed

A woman aged 56 years of age had a community-acquired left neck abscess and internal jugular vein thrombosis with septicemia due to extended-spectrum ?-lactamase (ESBL)-producing Klebsiella pneumoniae. Even though she was treated with intravenous meropenem, the bacteremia persisted. She was complicated with multiple brain abscesses, seizure, and leucopenia. After a combination of intravenous fosfomycin and meropenem, her clinical condition became stable. Combination treatment was continued for 2 months and she recovered. In individual cases of Lemierre syndrome with brain abscess caused by ESBL-producing Enterobacteriaceae, fosfomycin combination therapy may be the alternative choice. PMID:22154996

Lee, Wen-Sen; Wang, Fu-Der; Shieh, Ying-Hua; Teng, Sing-On; Ou, Tsong-Yih

2011-12-11

411

Metformin therapy in a hyperandrogenic anovulatory mutant murine model with polycystic ovarian syndrome characteristics improves oocyte maturity during superovulation  

PubMed Central

Background Metformin, an oral biguanide traditionally used for the treatment of type 2 diabetes, is widely used for the management of polycystic ovary syndrome (PCOS)-related anovulation. Because of the significant prevalence of insulin resistance and glucose intolerance in PCOS patients, and their putative role in ovulatory dysfunction, the use of metformin was touted as a means to improve ovulatory function and reproductive outcomes in PCOS patients. To date, there has been inconsistent evidence to demonstrate a favorable effect of metformin on oocyte quality and competence in women with PCOS. Given the heterogeneous nature of this disorder, we hypothesized that metformin may be beneficial in mice with aberrant metabolic characteristics similar to a significant number of PCOS patients. The aim of this study was to gain insight into the in vitro and in vivo effects of metformin on oocyte development and ovulatory function. Methods We utilized metformin treatment in the transgenic ob/ob and db/db mutant murine models which demonstrate metabolic and reproductive characteristics similar to women with PCOS. Results: Metformin did not improve in vitro oocyte maturation nor did it have an appreciable effect on in vitro granulosa cell luteinization (progesterone production) in any genotype studied. Although both mutant strains have evidence of hyperandrogenemia, anovulation, and hyperinsulinemia, only db/db mice treated with metformin had a greater number of mature oocytes and total overall oocytes compared to control. There was no observed impact on body mass, or serum glucose and androgens in any genotype. Conclusions Our data provide evidence to suggest that metformin may optimize ovulatory performance in mice with a specific reproductive and metabolic phenotype shared by women with PCOS. The only obvious difference between the mutant murine models is that the db/db mice have elevated leptin levels raising the questions of whether their response to metformin is related to elevated leptin levels and/or if a subset of PCOS women with hyperleptinemia may be responsive to metformin therapy. Further study is needed to better define a subset of women with PCOS that may be responsive to metformin.

2011-01-01

412

Therapeutic Options for Middle East Respiratory Syndrome Coronavirus (MERS-CoV) - possible lessons from a systematic review of SARS-CoV therapy.  

PubMed

The Middle East Respiratory Syndrome coronavirus (MERS-CoV) has been detected in a number of countries in the Middle East and Europe with an apparently high mortality rate. It is phylogenetically related to the SARS coronavirus and has also been associated with severe respiratory illness as well as nosocomial transmission in healthcare settings. Current international recommendations do not support any specific therapies; however, there are a number of agents, which were used during the SARS epidemic of 2003. It is possible that these might be active against the related MERS coronavirus. We have reviewed the literature on the safety and efficacy of therapies used in patients with SARS with a view to their potential use in patients with MERS-CoV infections. PMID:23993766

Momattin, Hisham; Mohammed, Khurram; Zumla, Alimuddin; Memish, Ziad A; Al-Tawfiq, Jaffar A

2013-08-29

413

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

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

414

A progressive, fatal dystonia-Parkinsonism syndrome in a patient with primary immunodeficiency receiving chronic IVIG therapy.  

PubMed

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by a mutation in the Bruton agammaglobulinemia tyrosine kinase gene that results in severe B-cell deficiency. So far, neurological complications of XLA have been primarily related to acute and/or chronic central nervous system enteroviral infections. In the last few years a progressive neurodegenerative syndrome of unknown etiology has been described in XLA patients. We describe and present a video of an XLA patient who developed a fatal dementing, dystonia-Parkinsonism syndrome 14 years into his immune disorder. Physician awareness of this rare syndrome may lead to its better characterization and management. PMID:17588239

Papapetropoulos, Spiridon; Friedman, Jennifer; Blackstone, Craig; Kleiner, Gary I; Bowen, Brian C; Singer, Carlos

2007-08-15

415

Heightened pain sensitivity in individuals with signs and symptoms of carpal tunnel syndrome and the relationship to clinical outcomes following a manual therapy intervention.  

PubMed

Neurophysiological responses related to lessening of pain sensitivity are a suggested mechanism of manual therapy. Prior studies have observed generalized lower pain thresholds associated with carpal tunnel syndrome (CTS) in comparison to healthy controls. The present study sought to determine whether similar findings were present in suprathreshold measures and measures specific to central integration of pain (temporal summation and after sensations). Additionally, we wished to determine whether measures of pain sensitivity were related to clinical outcomes in participants with signs and symptoms of CTS receiving a manual therapy intervention. Individuals with signs and symptoms of CTS reported greater pain sensitivity to suprathreshold measures of mechanical pain, temporal summation, and after sensation in comparison to healthy controls. Immediate lessening of mechanical pain sensitivity and after sensations in response to a manual therapy intervention and 3-week attenuation of temporal summation following a 3-week course of manual therapy were associated with 3-week changes in clinical pain intensity in participants with signs and symptoms of CTS. These findings suggest heightened pain sensitivity across several parameters may be associated with CTS. Furthermore, changes in mechanical pain, after sensation, and temporal summation may be related to improvements in clinical outcomes. PMID:21764354

Bialosky, Joel E; Bishop, Mark D; Robinson, Michael E; Price, Donald D; George, Steven Z

2011-07-20

416

Randomized, Double-Blind Trial to Evaluate the Safety of Apixaban With Antiplatelet Therapy After Acute Coronary Syndrome in Japanese Patients (APPRAISE-J).  

PubMed

Background:?Concomitant anticoagulant therapy may further reduce the risk of thrombotic events in patients with acute coronary syndrome (ACS) when given in addition to current standard antiplatelet therapies. This Phase II, randomized, double-blind, placebo-controlled study in Japanese patients with ACS assessed the bleeding risk of apixaban compared with placebo when given in combination with standard antiplatelet therapy, and followed a similar design to APPRAISE-1, the larger global Phase II study. Methods and Results:?Patients with recently diagnosed ACS were randomized to receive apixaban 2.5mg twice daily (BID; n=49), apixaban 5mg BID (n=50), or placebo (n=52) in addition to standard antiplatelet therapy for 24 weeks. The composite primary endpoint of major or clinically relevant nonmajor bleeding occurred in 2 patients (4.1%) in each apixaban treatment group and 1 patient (2.0%) in the placebo group, and a dose-dependent increase was seen in all bleeding events. No hemorrhagic strokes occurred in either apixaban treatment group. This study was terminated before completion because the APPRAISE-2 global Phase III trial was stopped based on the recommendation of the Data Monitoring Committee, following an increase in bleeding events without a counterbalancing reduction in ischemic events. Conclusions:?The bleeding profile of apixaban in Japanese patients with ACS was similar to that found in the global APPRAISE-1 study, supporting the safety of apixaban in Japanese patients.??(Circ J?2013; 77: 2341-2348). PMID:23748920

Ogawa, Hisao; Goto, Shinya; Matsuzaki, Masunori; Hiro, Shintaro; Shima, Daisuke

2013-06-07

417

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

1998-01-01

418

Effect of continuous positive airway pressure therapy on a large hemangioma complicated with obstructive sleep apnea syndrome: a case report  

Microsoft Academic Search

INTRODUCTION: Hemangiomas involving the upper airway can be an uncommon cause of obstructive sleep apnea syndrome. CASE PRESENTATION: A 26-year-old Caucasian man with a known history of a large hemangioma of his head and neck presented with sleep-disordered breathing to the sleep unit of our hospital. Severe obstructive sleep apnea syndrome was revealed on polysomnography. Nasal continuous positive airway pressure

Maria Antoniadou; Paschalis Steiropoulos; Evangelia Serasli; Venetia Tsara

2010-01-01

419

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

PubMed Central

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

Simas, Angela; Matos, Catarina; Lopes da Silva, Rodrigo; Brotas, Vitor; Teofilo, Eugenio; Albino, Jose Pereira

2010-01-01

420

[Cardiorenal syndrome].  

PubMed

The cardiorenal syndrome describes the frequent clinical situation in which therapy of a patient with acute cardiac decompensation is limited by a decline of renal function. This is frequently associated with preexisting chronic renal insufficiency. It is important to be aware of this potentially fatal constellation and perform diuretic therapy slowly and carefully in order to avoid inadequate volume depletion. This may require surveillance in an intensive care unit. Ultrafiltration is an alternative to diuretic therapy which may permit greater fluid loss when beginning therapy and better control of volume reduction once heart failure symptoms improve. The individual treatment of patients with cardiorenal syndrome requires a close cooperation between cardiologists and nephrologists. PMID:22274302

Alscher, M D; Sechtem, U

2012-03-01

421

Is there a need for "triple therapy"? Role of anticoagulation with dual antiplatelet therapy in acute coronary syndromes (ATLAS Study & TRAP Study).  

PubMed

In the past two decades there has been a succession of advances in the development of anticoagulant and antiplatelet therapies to be used in the treatment of ACS. Despite optimal dual antiplatelet therapy, nearly 10-12 % of patients still face a risk of death or myocardial infarction one year following PCI. This large residual risk provides the impetus for the development of more effective strategies. Dual pathway regimens that combine antiplatelets (aspirin and a thienopyridine), along with an anticoagulant such as rivaroxaban may prove to be a therapeutic option in patients with ACS. PMID:24022544

Patrick, William L; Patel, Charmaine; Guddeti, Raviteja; Narayan, Aarti; Maddaleni, Michael; Kumar, Shankar; Dolma, Kalsang; Serla, Vishnu Vardhan; Sangha, Maheep; Govindavarjhulla, Aditya; Saddala, Prashanth; Singh, Priyamvada; Halaby, Rim; Gibson, C Michael

2013-10-01

422

Health-related quality of life in patients with chronic fatigue syndrome: group cognitive behavioural therapy and graded exercise versus usual treatment. A randomised controlled trial with 1 year of follow-up  

Microsoft Academic Search

Chronic fatigue syndrome (CFS) produces physical and neurocognitive disability that significantly affects health-related quality\\u000a of life (HRQL). Multidisciplinary treatment combining graded exercise therapy (GET) cognitive behavioural therapy (CBT) and\\u000a pharmacological treatment has shown only short-term improvements. To compare the effects on HRQL of (1) multidisciplinary\\u000a treatment combining CBT, GET, and pharmacological treatment, and (2) usual treatment (exercise counselling and pharmacological

Montserrat Núñez; Joaquim Fernández-Solà; Esther Nuñez; José-Manuel Fernández-Huerta; Teresa Godás-Sieso; Esther Gomez-Gil

2011-01-01

423

Long-term compliance with nasal continuous positive airway pressure therapy for sleep apnea syndrome in an otorhinolaryngological office.  

PubMed

Continuous positive airway pressure (CPAP) is effective for patients with SAS. CPAP therapy requires long-term usage to prevent recurrence of symptoms. It is, thus, important to examine the level of long-term CPAP use and the factors influencing compliance with CPAP therapy for SAS. Compliance with CPAP therapy was examined in 204 patients in whom such therapy was started between 2003 and 2009. The median follow-up duration was 19 months (IQR = 6.8-37.5). Although the subjective and objective curative effects were significant, 18 patients (8.9%) refused CPAP therapy. Survival analysis showed that the patients' adherence to CPAP after 5 years was 89.8%. Multivariate analysis, including gender, age, BMI, AHI, arousal index, minSpO?, ESS, sleep stage, and LMI, indicated that the degree of improvement of AHI, percentage of deep sleep stage, and LMI were clinical variables independently associated with long-term adherence to CPAP. Furthermore, use of appropriate drugs for the patients with nasal congestion resulted in better satisfaction and adherence to CPAP therapy. We have shown that the rate of compliance and the subjective and objective curative effects of CPAP therapy were high, and detected the independent clinical factors associated with continued CPAP therapy. PMID:23568045

Tokunaga, Takahiro; Ninomiya, Takahiro; Kato, Yukinori; Ito, Yumi; Takabayashi, Tetsuji; Tokuriki, Masaharu; Sunaga, Hiroshi; Fujieda, Shigeharu

2013-04-09

424

Autoimmune Polyendocrine Syndrome Type 1 in North-Western France: AIRE Gene Mutation Specificities and Severe Forms Needing Immunosuppressive Therapies  

Microsoft Academic Search

Background: Autoimmune polyendocrine syndrome type 1 (APS1) has been poorly evaluated in France. We focused on the north-western part of the country to describe clinical phenotypes, especially severe forms of the disease, and AIRE gene mutations. Methods: Clinical and immunological data were collected, and pathological mutations were identified by DNA sequencing. Results: Nineteen patients were identified with APS1. Clinical manifestations

E. Proust-Lemoine; P. Saugier-Véber; D. Lefranc; S. Dubucquoi; A. Ryndak; D. Buob; J. D. Lalau; R. Desailloud; J. Weill; L. Prin; H. Lefebvre; J. L. Wémeau

2010-01-01

425

PTPN11 (Protein Tyrosine Phosphatase, Nonreceptor Type 11) Mutations and Response to Growth Hormone Therapy in Children with Noonan Syndrome  

Microsoft Academic Search

Context: The cause of growth impairment in Noonan syndrome (NS) remains unclear. Mutations in PTPN11 (protein tyrosine phospha- tase, nonreceptor type 11) that codify constitutively activated Src homology protein tyrosine phosphatase-2 tyrosine phosphatase and may interfere with GH and IGF-I signaling were identified in ap- proximately 40% of patients with NS. Objective: The objective of this study was to evaluate

Lize V. Ferreira; Silvia A. L. Souza; Ivo J. P. Arnhold; Berenice B. Mendonca; Alexander A. L. Jorge

426

Internal Jugular Vein Thrombosis, Lemierre's Syndrome; Oropharyngeal Infection with Antibiotic and Anticoagulation TherapyA Case Report  

Microsoft Academic Search

The authors present a case of Lemierre's syndrome that is an uncommon septic throm bophlebitis of the internal jugular vein. A 31-year-old man developed pharyngeal pain one month before hospital admission when he suffered from a severe headache and painful swelling of the left side of his neck. He was diagnosed with tonsillitis. Contrast- enhanced computed tomography and magnetic resonance

Shin Nakamura; Seizo Sadoshima; Yasufumi Doi; Maki Yoshioka; Shigeru Yamashita; Hiroki Gotoh; Kaoru Onoyama

2000-01-01

427

Study to Evaluate the Efficacy of Antenatal Steroid Therapy in the Prevention of Neonatal Respiratory Distress Syndrome.  

National Technical Information Service (NTIS)

A collaborative double blind clinical trial to assess the benefits and risks associated with antenatal steroid therapy in the prevention of RDS was designed. The institution sited above is one of the collaborative institutions in the study. A rigid protoc...

R. D. Zachman L. B. Curet

1979-01-01

428

Long term effects of the palatal plate therapy for the orofacial regulation in children with Down syndrome.  

PubMed

The objective of this study was to evaluate long term effects of orofacial regulation therapy with modified Castillo-Morales palatal plate on 68 Down children that attended our Unit from 1992 to 2001. Corrections obtained with palatal plate therapy were evaluated according to the following parameters: spontaneous lingual protrusion based on three level scale, position "open mouth", labial hypotonia and sialorrhea. The results showed distinct improvement in nearly all of the parameters compared to initial conditions. PMID:14604150

Zavaglia, V; Nori, A; Mansour, N M

2003-01-01

429

New recurrent chromosome change in pediatric therapy-related myelodysplastic syndrome: unbalanced translocation 1/6 with cryptic duplication of short arm of chromosome 6.  

PubMed

The incidence of therapy-related myelodysplastic syndrome (t-MDS) in pediatric patients is increasing in parallel with the more successful management of the primary tumor, but scant information is available on clinical and cytogenetic characteristics. We report here two children affected by t-MDS after chemo/radiotherapy for a primary solid tumor, both with an unbalanced translocation 1/6 in their bone marrow. Characterization by array comparative genomic hybridization of the imbalances showed an almost identical pattern: almost complete trisomy of the long arm of chromosome 1, and a terminal deletion and interstitial duplication of the short arm of chromosome 6. The gain of chromosome 6 short arm encompasses regions already highlighted as possibly relevant for t-MDS in adults, and we suggest that the unbalanced translocation reported here be considered a new recurrent, non-random chromosomal abnormality in pediatric patients with t-MDS. PMID:22616618

Tassano, Elisa; Tavella, Elisa; Valli, Roberto; Micalizzi, Concetta; Cuoco, Cristina; Maserati, Emanuela; Pasquali, Francesco; Morerio, Cristina

2012-06-13