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1

Therapy for the Brugada Syndrome  

PubMed Central

The Brugada syndrome is a congenital syndrome of sudden cardiac death first described as a new clinical entity in 1992. Electrocardiographically characterized by a distinct coved-type ST segment elevation in the right precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young and otherwise healthy adults, and less frequently in infants and children. The ECG manifestations of the Brugada syndrome are often dynamic or concealed and maybe revealed or modulated by sodium channel blockers. The syndrome may also be unmasked or precipitated by a febrile state, vagotonic agents, ?-adrenergic agonists, ?-adrenergic blockers, tricyclic ortetracyclic antidepressants, a combination of glucose and insulin, and hypokalemia, as well as by alcohol and cocaine toxicity. An implantable cardioverter-defibrillator (ICD) is the most widely accepted approach to therapy. Pharmacological therapy aimed at rebalancing the currents active during phase 1 of the right ventricular action potential is used to abort electrical storms, as an adjunct to device therapy, and as an alternative to device therapy when use of an ICD is not possible. Isoproterenol and cilostazol boost calcium channel current, and drugs like quinidine inhibit the transient outward current, acting to diminish the action potential notch and thus suppress the substrate and trigger for ventricular tachycardia/fibrillation (VT/VF).

Fish, J. M.

2006-01-01

2

Electroconvulsive therapy and Klinefelter syndrome.  

PubMed

Klinefelter syndrome is a common sex chromosome disorder characterized by the presence of 1 or more extra X chromosomes, and the most prevalent karyotype is 47,XXY. Epidemiological studies have showed that patients with Klinefelter syndrome had a significantly increased risk of psychosis. We presented a case of a patient with Klinefelter syndrome who was characterized by psychiatric symptoms. The patients had been refractory to clozapine and sodium valproate, but a remarkable improvement occurred after a cycle of 11 sessions of modified electroconvulsive therapy. PMID:23965608

Wei, Qiang; Xie, Xinhui; Chen, Yang; Tian, Yanghua; Wang, Honghao; Wang, Keyong; Wang, Kai

2013-09-01

3

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

4

Immunomodulatory therapy for myelodysplastic syndromes.  

PubMed

Thalidomide and lenalidomide belong to the proprietary group of immunomodulatory drugs (IMiDs) that display broad biologic and pharmacologic properties. Encouraging results of clinical studies that evaluated the efficacy of thalidomide in patients with myelodysplastic syndromes (MDSs) led to the investigation of its structural analogue, lenalidomide, in patients with lower-risk MDS. The cumulative results of studies that tested lenalidomide in patients with interstitial deletion of chromosome 5q, ie, del(5q), showed a high frequency of both erythroid and cytogenetic responses (approximately 75% of patients), which led to US Food and Drug Administration approval of this agent for this cytogenetically defined MDS subset. A multicenter phase III study (MDS-002) that investigated the frequency of transfusion response in lower-risk non-del(5q) MDS patients showed that lenalidomide had significant erythropoietic activity, albeit less robust in lower-risk MDS without del(5q). These studies established lenalidomide as an active erythropoietic-remitting agent with novel cytogenetic-remitting activity in lower-risk MDS patients who would not otherwise benefit from therapy with erythropoietic growth factors. The National Comprehensive Cancer Network Clinical Practice Guidelines recently added lenalidomide to the therapeutic algorithm for MDS as front-line therapy for lower-risk MDS patients with del(5q) and transfusion-dependent anemia. PMID:18055335

Sokol, Lubomir; List, Alan F

2007-11-01

5

Speech & Language Therapy for Children and Adolescents with Down Syndrome  

MedlinePLUS

... Adolescents with Down Syndrome Speech & Language Therapy for Children & Adolescents with Down Syndrome Children with Down syndrome have ... of communication. What Are the Language Characteristics of Children and Adolescents with Down Syndrome? Research and clinical ...

6

Therapy with azanucleosides for myelodysplastic syndromes  

Microsoft Academic Search

Azanucleosides constitute the core therapy in the management of myelodysplastic syndromes (MDS), and have altered the treatment paradigm of MDS, previously dominated by supportive care strategies. DNA methylation regulates gene transcription in MDS, and it is hypothesized that azanucleoside therapy induces DNA hypomethylation and re-expression of aberrantly silenced genes in patients with these disorders. A series of clinical trials conducted

Fabio P. S. Santos; Guillermo Garcia-Manero; Alfonso Quintás-Cardama

2010-01-01

7

Cognitive Therapy for Irritable Bowel Syndrome.  

ERIC Educational Resources Information Center

Randomly assigned 20 patients with irritable bowel syndrome to intensive, individualized cognitive therapy or to daily gastrointestinal symptom monitoring. Pre- to posttreatment evaluations showed significantly greater gastrointestinal symptom reduction for those receiving cognitive therapy than for those in symptom monitoring. At posttreatment,…

Greene, Barbara; Blanchard, Edward B.

1994-01-01

8

Response of Paraneoplastic Syndromes to Antineoplastic Therapy  

PubMed Central

The development of a clinically apparent paraneoplastic syndrome in a patient with malignant disease is cause for considerable concern as the symptoms can be as serious and difficult to deal with as those produced by the cancer itself. While the information in the medical literature concerning the response of paraneoplastic processes to specific antineoplastic therapy is limited, case reports and small series would suggest that a number of syndromes are alleviated with successful treatment of the underlying neoplasm. As treatment programs for advanced malignant disease improve, a larger percentage of patients suffering from the effects of paraneoplastic processes will benefit from therapy directed at their cancer.

Markman, Maurie

1986-01-01

9

Upper Plexus Thoracic Outlet Syndrome: Optimal Therapy  

Microsoft Academic Search

Background. Previously, transaxillary first rib resection alone was not considered adequate therapy for “upper plexus” (median nerve) thoracic outlet syndrome. It was thought that the “combined” approach with upper plexus dissection through a supraclavicular incision in addition to the transaxillary approach was necessary. However, with better understanding of anatomy—that the median nerve receives fibers from C8 and T1 as well

1997-01-01

10

Ganglioside Therapy and Guillain-Barré Syndrome  

Microsoft Academic Search

We investigated an alleged association between ganglioside therapy and the risk of developing the Guillain-Barré syndrome (GBS) in a historical cohort study. Using official medical records of the Local Health Unit No. 31 of Ferrara Province, northern Italy, we linked data on sales of gangliosides with information regarding hospital admissions and discharge diagnoses between January 1, 1988, and May 31,

Enrico Granieri; Ilaria Casetta; Vittorio Govoni; Maria R. Tola; Ezio Paolino; Walter A. Rocca

1991-01-01

11

Turner's syndrome: results of estrogen therapy.  

PubMed

Annual endometrial biopsy had been advocated for patients with Turner's syndrome. The practicality of using this procedure on patients with no symptoms of abnormal bleeding has been questioned; this study attempts to answer this issue. Charts of patients with Turner's syndrome from the Cleveland Clinic for the period 1951-75 were reviewed. A criteria was established for inclusion of patients in the study. 34 of 43 women who met the criteria returned for the follow-up and were given pelvic exam, Pap smear, and endometrial biopsy by Vabra aspiration. Of the 43 patients given substitution therapy, 13 were taking estrogen alone; 24 were on estrogen-progesterone therapy, and 6 had stopped taking estrogen (Table 1). The presenting symptom in all cases of endometrial adenocarcinoma was abnormal bleeding (menorrhagia or menometrorrhagia), suggesting a change from previous menstrual patterns. At high risk for developing endometrial carcinoma at an early age was patients with dysgenesis who were receiving estrogen replacement therapy. The results of this study suggest that annual endometrial sampling is not necessary for patients with Turner's syndrome. It is recommended, however, that an annual examination and a Pap smear of an endocervical specimen be performed. Should there be any sign of menstrual aberration, endometrial biopsy or dilatation and curettage should be done. The importance of careful follow-up should also be impressed on patients who are on estrogen therapy; not more than 1 year's supply of estrogen should be given to such patients. PMID:436270

Gidwani, G P; Seiler, J C; Ballard, L A

1979-01-01

12

[Differentiated antiplatelet therapy for acute coronary syndromes].  

PubMed

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

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

2014-01-01

13

[Replacement therapy for hepatic failure syndrome].  

PubMed

The results of treatment were studied in 33 patients with hepatic failure syndrome. The patients received MARS therapy or hemodiafiltration as part of intensive care. Albumin dialysis (MARS) may replace hepatic detoxifying function and it is highly effective in diminishing cytolysis in patients with hepatic failure syndrome. Hepatic encephalopathy rapidly regressed with MARS therapy, except for patients with irreversible cerebral changes. The major effect of albumin dialysis is a prompt reducing cytolosis of hepatocytes and liver function stabilization. The most likely mechanisms of action of MARS are the elimination of hydrophobic toxins and the deligandization of albumin molecules, suggesting that MARS is effective in correcting osmotic and colloid ostomic pressures, acid-base balance, microcirculation, and hemodynamics, resulting in a decrease in the concentration of proinflammatory cytokines. PMID:17288262

Iampol'ski?, A F; Eremeeva, L F

2006-01-01

14

New antiplatelet therapies for acute coronary syndromes  

Microsoft Academic Search

Platelets play a central role in the pathophysiology of acute coronary syndromes (ACS). Dual antiplatelet therapy has resulted\\u000a in significant advances in the treatment of ACS; however, ACS remains an important cause of morbidity and mortality. Important\\u000a limitations exist among the current antiplatelet agents and therefore a pressing need for the development of improved antiplatelet\\u000a agents exists. Three antiplatelet agents

Jonathan D. Rich; Stephen D. Wiviott

2007-01-01

15

Music Therapy: A Therapeutic Intervention for Girls with Rett Syndrome.  

ERIC Educational Resources Information Center

The paper reviews music therapy, the educational background of music therapists, music therapy's various settings, and its use as an intervention with girls with Rett Syndrome. Sample music therapy programs for three girls (aged 5, 14, and 20 years) with Rett Syndrome are presented. The sample programs provide: student descriptions; the girls'…

Coleman, Kathleen A.

16

[Incretin therapy and the metabolic syndrome].  

PubMed

Incretin therapy includes treatment with incretin analogues (exenatid and liraglutid) and so called incretin enhancers (gliptins and DPP-4 inhibitors respectively--sitagliptin, vildagliptin, saxagliptin, linagliptin). In patients with type 2 diabetes, this novel antidiabetic treatment usually leads to successful reduction in fasting as well as postprandial glycaemia and glycosylated haemoglobin. At the same time, it importantly improves all components of metabolic syndrome (dyslipidemia, hypertension, systemic inflammation). Incretin analogues also reduce body weight while DPP-4 inhibitors are weight-neutral. Both groups of drugs are expected to have positive cardiovascular effects, although it is not clear whether these are likely to be direct or indirect, i.e. facilitated by improved compensation of metabolic syndrome components. PMID:21612071

Svacina, S

2011-04-01

17

Rifaximin Therapy of Irritable Bowel Syndrome  

PubMed Central

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

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

2012-01-01

18

Review of pharmacological therapies in fibromyalgia syndrome  

PubMed Central

This review addresses the current status of drug therapy for the management of fibromyalgia syndrome (FMS) and is based on interdisciplinary FMS management guidelines, meta-analyses of drug trial data, and observational studies. In the absence of a single gold-standard medication, patients are treated with a variety of drugs from different categories, often with limited evidence. Drug therapy is not mandatory for the management of FMS. Pregabalin, duloxetine, milnacipran, and amitriptyline are the current first-line prescribed agents but have had a mostly modest effect. With only a minority of patients expected to experience substantial benefit, most will discontinue therapy because of either a lack of efficacy or tolerability problems. Many drug treatments have undergone limited study and have had negative results. It is unlikely that these failed pilot trials will undergo future study. However, medications, though imperfect, will continue to be a component of treatment strategy for these patients. Both the potential for medication therapy to relieve symptoms and the potential to cause harm should be carefully considered in their administration.

2014-01-01

19

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

20

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

21

Sweet's syndrome after adalimumab therapy for refractory relapsing polychondritis  

PubMed Central

Relapsing polychondritis and Sweet’s syndrome are rare systemic inflammatory conditions. The authors present a patient who developed Sweet’s syndrome 1 week after adalimumab therapy for refractory relapsing polychondritis. Coexistent relapsing polychondritis and Sweet’s syndrome is rare, however, is likely to represent a true disease association and signifies a high risk of myelodysplasia. Antitumour necrosis factor ? (TNF?) therapy is a treatment option in both relapsing polychondritis and Sweet’s syndrome, and switching anti-TNF? agents may be feasible in the event of adverse reaction.

Keidel, Sarah; McColl, Aubretia; Edmonds, Sally

2011-01-01

22

[Cardiorenal syndrome: limits of heart failure therapy].  

PubMed

The cardiorenal syndrome is an interdisciplinary challenge with increasing health economic relevance. Renal failure is a strong predictor for mortality in patients with severe congestive heart failure (CHF) and CHF is one of the fastest increasing morbidities in western countries. For successful therapy a close cooperation between cardiology und nephrology is required. Moreover, a good compliance of the patient is needed to improve symptoms and to reduce the frequency of cardiac decompensation. A broad cardiological and nephrological evaluation and consideration of optimal conservative options according to national and international guidelines are essential. However, a renal replacement therapy might be helpful in patients with refractory heart failure even if they are not dialysis-dependent. In cases of acute heart and renal failure an intensive care management might be necessary to reduce volume overload with the help of extracorporeal ultrafiltration or a dialysis modality. Nevertheless, in cases of chronic refractory CHF peritoneal dialysis should be preferred. The first analysis of the registry of the German Society of Nephrology (http://www.herz-niere.de) confirmed that there is a benefit for health-related quality of life in chronic CHF patients treated with peritoneal dialysis. PMID:23740085

Großekettler, L; Schmack, B; Schwenger, V

2013-09-01

23

[Pain therapy in irritable bowel syndrome].  

PubMed

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

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

2014-06-01

24

[Successful therapy of hepatorenal syndrome with norepinephrine].  

PubMed

A 39-year-old female with alcoholic cirrhosis was admitted with signs of an alcoholic hepatitis. Within one week a hepatorenal syndrome (HRS) (Creatinin 5.83 mg/100 ml, Harnstoff 235 mg/100 ml) evolved in the absence of additional causes. She had a diminished water (urine volume 31 ml/h) and sodium excretion (10 mmol/l). Urine flow was increased to 131 ml/h by plasma expansion with i.v. infusion of volume and albumin and with infusion of dopamine (3 micrograms/kg/min) and, as there was no diuretic pretreatment and thus, no HRS secondary to diuretic treatment, furosemide (500 mg/24 h). However, impairment of renal function remained unchanged with this therapy. Therefore, norepinephrine (NE) therapy was initiated. A dosage of 0.1-0.12 microgram/kg/min was necessary to achieve the desired increase in the mean arterial pressure of 10-20 mm Hg. During the NE infusion the urine volume increased further to 231 ml/h, the sodium excretion raised to 44 mmol/l, and serum levels of creatinine and urea decreased to 1.91 mg/100 ml and 141 mg/100 ml, respectively. With recovering liver function the NE infusions could be discontinued after 5 days without recurrence of a HRS until discharge after 3 weeks. Beside the vasopressin analogon ornipressin, the combination of norepinephrine and dopamine seems to be useful for the therapy of HRS. Norepinephrine has the advantage of an easy accessibility in ICUs and seems to exert less side effects. PMID:11194884

Pehl, C; Schepp, W

2000-12-01

25

Allo-SCT for  Philadelphia-negative myeloproliferative neoplasms in blast phase: a study from the Societe Française de Greffe de Moelle et de Therapie Cellulaire (SFGM-TC).  

PubMed

Progression of Philadelphia-negative myeloproliferative (MPN) or myelodysplastic/myeloproliferative neoplasms (MDS/MPN) to acute myeloid leukemia (AML) is an adverse event in the course of the disease. Although allogeneic hematopoietic SCT (allo-SCT) is considered as the only curative therapy, few data exist on the outcome of patients with Philadelphia-negative MPN or MDS/MPN in blast phase who received an allo-SCT. Sixty patients were included in this retrospective study. AML was secondary to an MPN in 43 cases, whereas AML evolved from an MDS/MPN in 17 cases. Patients received allo-SCT in CR or advanced disease in 26 cases and 34 cases, respectively. With a median follow-up of 31 months (range, 25-44), OS and leukemia-free survival (LFS) were, respectively, 18% and 9% at 3 years. CR at transplant was associated with an improved LFS in univariate and multivariate analysis. The 3-year LFS was 18% for patients undergoing allo-SCT in CR versus 3% in advanced disease (P=0.008). Absence of thrombosis and an intermediate or favorable AML karyotype were associated with an improved outcome for patients who received allo-SCT in CR. New strategies are needed to improve the outcome of patients with MPN-MDS/MPN in blast phase. PMID:24614840

Cahu, X; Chevallier, P; Clavert, A; Suarez, F; Michallet, M; Vincent, L; Vigouroux, S; Blaise, D; Mariette, C; Bilger, K; Robin, M; Yakoub-Agha, I; Peffault de Latour, R; Mohty, M

2014-06-01

26

Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome  

Microsoft Academic Search

Susac's syndrome is the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss (Susac 1994) [1]. It occurs predominantly in young females and is believed to be an immune-mediated endotheliopathy of small vessels of the brain, retina and cochlea (Neumayer et al. 2009) [2]. Early, aggressive, and sustained immunosuppressive therapy has been recommended for Susac's syndrome and

T. A. Hardy; R. J. Garsia; G. M. Halmagyi; S. J. G. Lewis; B. Harrisberg; M. J. Fulham; M. H. Barnett

2011-01-01

27

Art therapy applied to an adolescent with Asperger's syndrome  

Microsoft Academic Search

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

Deborah L. Elkis-Abuhoff

2008-01-01

28

Sick sinus syndrome as a complication of mediastinal radiation therapy  

Microsoft Academic Search

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

Sinikka Pohjola-Sintonen; K. J. K. Toetterman; Markku Kupari

1990-01-01

29

Pharmacologic Therapies for Complex Regional Pain Syndrome  

PubMed Central

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

Mackey, Sean; Feinberg, Steven

2010-01-01

30

Apixaban with antiplatelet therapy after acute coronary syndrome  

Microsoft Academic Search

BACKGROUND: Apixaban, an oral, direct factor Xa inhibitor, may reduce the risk of recurrent ischemic events when added to antiplatelet therapy after an acute coronary syndrome. METHODS: We conducted a randomized, double-blind, placebo-controlled clinical trial comparing apixaban, at a dose of 5 mg twice daily, with placebo, in addition to standard antiplatelet therapy, in patients with a recent acute coronary

J. H. Alexander; R. D. Lopes; S. James; R. Kilaru; Y. He; P. Mohan; D. L. Bhatt; S. Goodman; F. W. A. Verheugt; M. Flather; K. Huber; D. Liaw; S. E. Husted; J. Lopez-Sendon; R. de Caterina; P. Jansky; H. Darius; D. Vinereanu; J. H. Cornel; F. Cools; D. Atar; J. L. Leiva-Pons; M. Keltai; H. Ogawa; P. Pais; A. Parkhomenko; W. Ruzyllo; R. Diaz; H. White; M. Ruda; M. Geraldes; J. Lawrence; R. A. Harrington; L. Wallentin

2011-01-01

31

FAMILY THERAPY OF THE MODERATE TYPE OF PARENTAL ALIENATION SYNDROME  

Microsoft Academic Search

from The American Journal of Family Therapy. 27:195-212, 1999 Each of the three types of parental alienation syndrome (PAS) warrants a different therapeutic approach. Because PAS is a family problem, family therapy is usually warranted-separation, divorce, and even litigation notwithstanding Furthermore, formidable modifications of traditional family therapy approaches are warranted if there is to be any chance of success in

Richard A. Gardner

1999-01-01

32

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

33

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).

34

Tumour necrosis factor (TNF) inhibitor therapy in Susac's syndrome.  

PubMed

Susac's syndrome is the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss (Susac 1994) [1]. It occurs predominantly in young females and is believed to be an immune-mediated endotheliopathy of small vessels of the brain, retina and cochlea (Neumayer et al. 2009) [2]. Early, aggressive, and sustained immunosuppressive therapy has been recommended for Susac's syndrome and anecdotal evidence has suggested a therapeutic role for monoclonal antibodies (Rennebohm et al. 2008, Lee and Amezcua 2009) [3,4]. We report a case of Susac's syndrome in which the patient improved immediately after tumour necrosis factor (TNF) inhibition with the monoclonal antibody, infliximab. PMID:21167503

Hardy, T A; Garsia, R J; Halmagyi, G M; Lewis, S J G; Harrisberg, B; Fulham, M J; Barnett, M H

2011-03-15

35

Bortezomib therapy for nephrotic syndrome due to idiopathic membranous nephropathy.  

PubMed

Idiopathic membranous nephropathy is a common cause of nephrotic syndrome in adults. The nephrotic syndrome due to idiopathic membranous nephropathy is often resistant to glucocorticosteroids and requires an alkylating agent such as chlorambucil or cyclophosphamide to induce remission. Recent studies illustrate that antibodies against the autoantigen M-type phospholipase A2 receptor contribute to a vast majority but not all cases of idiopathic membranous nephropathy. Herein, we report a patient with nephrotic syndrome due to membranous nephropathy that was resistant to 6 months of therapy with ramipril and high-dose glucocorticosteroids but responded to a single cycle of bortezomib infusion. PMID:24430762

Hartono, Choli; Chung, Miriam; Kuo, Sheng F; Seshan, Surya V; Muthukumar, Thangamani

2014-02-01

36

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

37

Family Therapy of the Moderate Type of Parental Alienation Syndrome.  

ERIC Educational Resources Information Center

Modification of traditional family therapy approaches are warranted if there is to be any chance of success in the treatment of Parental Alienation Syndrome families. Especially important is the full support of the court. Describes the special family therapeutic techniques warranted in the treatment of families in which the Parental Alienation…

Gardner, Richard A.

1999-01-01

38

Play Therapy for Children With Fetal Alcohol Syndrome  

Microsoft Academic Search

Fetal alcohol syndrome (FAS) is the leading birth defect in the United States, yet few treatment options exist for children afflicted with this disorder. Although play therapy has demonstrated its efficacy with numerous special populations, as of today, no research has been conducted on its use with FAS. This article presents an overview of current data on FAS, an argument

Elisabeth E. Liles; Jill Packman

2009-01-01

39

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

40

Complementary Therapy in Polycystic Ovary Syndrome  

PubMed Central

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

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

2014-01-01

41

Probiotic Therapy for Irritable Bowel Syndrome  

PubMed Central

The etiology of irritable bowel syndrome (IBS) is thought to be multifactorial, with several factors (including alterations in gut motility, small-bowel bacterial overgrowth, microscopic inflammation, and visceral hypersensitivity) potentially playing a role. Recent studies have suggested that probiotics may be useful in the treatment of IBS. Although the exact mechanism for how probiotics may aid in the reduction of symptoms commonly found in IBS is unknown, the effects of probiotics on alterations in gut bacteria appear to play a part. This review focuses on recent studies examining the role of probiotics in the treatment of IBS.

Aragon, George; Graham, Deborah B.; Borum, Marie

2010-01-01

42

Corticosteroids therapy and peptic ulcer disease in nephrotic syndrome patients  

PubMed Central

AIMS Whether corticosteroids induce peptic ulcer disease (PUD) remains uncertain. The study evaluated and compared the occurrence of PUD between nephrotic patients receiving oral prednisolone therapy and nephritic patients without steroid therapy. METHODS The prospective case control study compared 60 nephrotic syndrome patients who received 60 mg daily prednisolone therapy for 3 months with 30 age-and sex-matched nephritic patients without steroid therapy. Each patient underwent endoscopic examination and tissue and blood sampling before and after the study. Examined parameters included Helicobacter pylori (H. pylori) infection, and gastric and serum prostaglandin (PG) E2 and thromboxane (TX) B2 concentrations. The primary endpoint was the occurrence of endoscopic peptic ulcers between the two groups, while the secondary end point was the occurrence of ulcer complications. RESULTS The two groups were comparable in sex, age, smoking habits, alcohol drinking, past history of PUD, H. pylori infection rate and serum creatinine. There were no differences in the occurrence of endoscopic peptic ulcers (1.6% vs. 3.3%) and ulcer complications (0% vs. 0%), pre-therapy gastric PGE2, and pre- and post-therapy gastric TXB2, serum PGE2 and serum TXB2 between the two groups. However, there was significantly lower post-therapy gastric PGE2 concentrations in the prednisolone group. CONCLUSIONS Three-month therapy with 60 mg daily prednisolone caused few endoscopic ulcers (1.6%) and no ulcer complications in nephrotic patients. Corticosteroids therapy did not increase PUD in nephrotic syndrome patients [odds ratio 0.492 with 95% confidence interval (CI) 0.03, 8.142, P= 0.620]. Further larger studies are needed to clarify the role of corticosteroids in PUD.

Hsiang, Kuo-Wei; Ng, Yee-Yung; Lu, Ching-Liang; Chen, Tseng-Shing; Lin, Hsiao-Yi; Luo, Jiing-Chyuan; Wu, Jia-Min; Lin, Han-Chieh; Chang, Full-Young; Lee, Shou-Dong

2010-01-01

43

Rituximab therapy in nephrotic syndrome due to AH amyloidosis.  

PubMed

We report a patient with AH amyloidosis associated with lymphoplasmacytic leukemia that has remained in a stable state with a nephrotic syndrome for 17 months since the commencement of cyclic rituximab therapy aimed at suppression of pathogenetic gamma heavy chains. Free light chains in serum and CD20-positive cells in peripheral blood were useful as hematological markers in the patient. Rituximab might be a potent therapeutic option for AH amyloidosis associated with a B-cell lymphoproliferative disorder. PMID:19590993

Katoh, Nagaaki; Matsuda, Masayuki; Miyazaki, Daigo; Gono, Takahisa; Yazaki, Masahide; Ikeda, Shu-Ichi

2009-01-01

44

Current gut-directed therapies for irritable bowel syndrome  

Microsoft Academic Search

Opinion statement  Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder that can present with a wide array of symptoms that\\u000a make treatment difficult. Current therapies are directed at relieving symptoms of abdominal pain or discomfort, bloating,\\u000a constipation, and diarrhea. Pharmacologic agents used to treat IBS-associated pain include myorelaxants, peppermint oil, and\\u000a peripherally acting opiates. Dicyclomine and hyoscyamine, the two myorelaxants

Howard Y. Chang; Eoin C. Kelly; Anthony J. Lembo

2006-01-01

45

Should we consider MMF therapy after rituximab for nephrotic syndrome?  

Microsoft Academic Search

The management of steroid-dependent nephrotic syndrome, especially in patients who have failed to respond to cytotoxic drugs,\\u000a such as cyclophosphamide, remains challenging. Rituximab represents a new (off-label) therapeutic option. In a significant\\u000a portion of patients, it has a short serum half-life following the recovery of CD20-positive cells. The addition of mycophenolate\\u000a mofetil (MMF) as a maintenance therapy is also an

Guido Filler; Shih-Han Susan Huang; Ajay P. Sharma

46

The development of Moyamoya syndrome after proton beam therapy.  

PubMed

The development of Moyamoya syndrome (MMS) after cranial irradiation for pediatric tumors has been well established. However, information on the development of MMS after proton beam radiotherapy is sparse. We present the case of a 2-year-old child who developed radiation-induced MMS after treatment with proton beam therapy. Pediatr Blood Cancer 2014; 61:1490-1492. © 2014 Wiley Periodicals, Inc. PMID:24501091

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

2014-08-01

47

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

48

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

SciTech Connect

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

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

2011-01-01

50

Biological therapies in the acute respiratory distress syndrome.  

PubMed

Introduction: The acute respiratory distress syndrome (ARDS) is characterised by life-threatening respiratory failure requiring mechanical ventilation, and multiple organ failure. It has a mortality of up to 30 - 45% and causes a long-term reduction in quality of life for survivors, with only approximately 50% of survivors able to return to work 12 months after hospital discharge. Areas covered: In this review we discuss the complex pathophysiology of ARDS, describe the mechanistic pathways implicated in the development of ARDS and how these are currently being targeted with novel biological therapies. These include therapies targeted against inflammatory cytokines, mechanisms mediating increased alveolar permeability and disordered coagulation, as well as the potential of growth factors, gene therapy and mesenchymal stem cells. Expert opinion: Although understanding of the pathophysiology of ARDS has improved, to date there are no effective pharmacological interventions that target a specific mechanism, with the only potentially effective therapies to date aiming to limit ventilator-associated lung injury. However, we believe that through this improved mechanistic insight and better clinical trial design, there is cautious optimism for the future of biological therapies in ARDS, and expect current and future biological compounds to provide treatment options to clinicians managing this devastating condition. PMID:24702248

Boyle, Andrew James; McNamee, James Joseph; McAuley, Daniel Francis

2014-07-01

51

Irritable bowel syndrome treatment: cognitive behavioral therapy versus medical treatment  

PubMed Central

Introduction The study aims to investigate two kinds of treatment in patients suffering from irritable bowel syndrome (IBS) and consequently compares its efficacy on improving the symptoms and mental health of patients; one with just medical treatment and another through a combination of psychotherapy and medical treatment. Material and methods Applying general sampling, 50 IBS patients were selected from among those who used to refer to a Gastroenterology Clinic. After physical and mental evaluations based on ROME-II scale and SCL-90-R questionnaires, the subjects were randomly superseded into: the control group with medical treatment and, the case group with a combination of medical and psychological treatments. The acquired data were then analyzed through t-test and Mann-Whitney U-test. Results The findings show that the mental health of patients receiving cognitive behavioral therapy along with the medical treatment was higher than those of the control group at post-test level. It was observed that the therapy reduces the disability caused by IBS. Comparatively, while the cognitive therapy and medical treatments cured 80% of the patients, those receiving cognitive therapy alone showed an extensive reduction of symptoms. Conclusions Considering the role of cognitive behavioral therapy, it is therefore recommend that such patients be managed by a combined team of gastroenterologists and psychologists.

Mahvi-Shirazi, Majid; Rasoolzade-Tabatabaei, Sayed-Kazem; Amini, Mohsen

2012-01-01

52

Fibrate therapy in patients with metabolic syndrome and diabetes mellitus  

Microsoft Academic Search

Patients with metabolic syndrome and type 2 diabetes mellitus are usually in moderately high-risk, high-risk, or very high-risk\\u000a cardiovascular categories and present major therapeutic challenges. The dyslipidemia in such patients is typically a disorder\\u000a of the triglyceride\\/high-density lipoprotein axis (TG\\/HDL axis) characterized by an excess of triglyceride-rich lipoproteins\\u000a and a reduction of HDL. Very often, lifestyle therapy and statin monotherapy

Thomas Dayspring; Gregory Pokrywka

2006-01-01

53

Affectation heuristique de cellules à des commutateurs dans les réseaux cellulaires pour mobiles  

Microsoft Academic Search

Résumé  Le problème ?affectation de cellules à des commutateurs dans les réseaux cellulaires est un problème difficile qui ne peut\\u000a pas se résoudre de manière exacte en des temps de calcul raisonnables. Dans cet article, on propose un compromis basé sur\\u000a la méta-heuristique de recherche avec tabous pour obtenir des solutions acceptables en des temps de calcul raisonnables. La\\u000a démarche consiste

F. Houéto; S. pierre

2001-01-01

54

Evaluation of some prophylactic therapies for the idiopathic headshaker syndrome.  

PubMed

Eighteen horses affected by the idiopathic headshaker syndrome were studied in an owner assessed trial to test the efficacy of some prophylactic therapies. Riding the affected animal with a veil over the nostrils gave varying degrees of temporary relief in three of 10 horses. Local (intra-nasal) corticosteroid therapy was reported to be slightly effective in three of nine horses, but treatment with sodium cromoglycate, systemic corticosteroid, flunixin meglumine and an antihistamine were generally ineffective. Bilateral infraorbital neurectomy provided sustained relief in three of seven horses, but in one of these cases, a reaction at the neurectomy site necessitated another surgical procedure after six months. A fourth horse was reported to be slightly improved after neurectomy. A period of nasal irritation resulting in self-inflicted trauma was a common complication of this surgery. PMID:9109952

Mair, T S; Howarth, S; Lane, J G

1992-02-01

55

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

56

Failure of hypomethylating agent-based therapy in myelodysplastic syndromes.  

PubMed

Hypomethylating agents such as 5-azacytidine or decitabine have been a major breakthrough in the treatment of patients with myelodysplastic syndromes (MDS). They have been shown to improve transfusion requirements and to change the natural history of the disease. However, with increasing cumulative clinical experience, it has become apparent that these agents are not curative and have their own shortcomings. There is a subgroup of patients who do not respond to frontline therapy and a large, growing cohort of patients that lose response or progress while on hypomethylating agent-based therapy. There are no standard treatment options in this arena and it is therefore a focus of significant research interest. Since the mechanisms of resistance to hypomethylating agents are not known, selection of therapy is largely empiric but must take into account the age, comorbidities, and performance status of the patient, as well as the characteristics of the disease at the time of treatment failure. Higher intensity approaches and allogeneic stem cell transplant can yield improved response rates and long-term disease control but should be limited to a selected cohort of patients who can tolerate the treatment-related morbidities. For the majority of patients who likely will be better candidates for lower intensity therapy, several novel, investigational approaches are becoming available. Among these are newer nucleoside analogues, inhibitors of protein tyrosine kinases, molecules that interact with redox signaling within the cell, immunotherapy approaches, and others. Patients with MDS whose disease has failed to respond to hypomethylating agent therapy should be referred for clinical trials when available. As we learn more about the patterns and mechanisms of failure, the next challenge will be to determine which therapies are suitable for each individual patient. PMID:21943675

Kadia, Tapan M; Jabbour, Elias; Kantarjian, Hagop

2011-10-01

57

FAILURE OF HYPOMETHYLATING AGENT-BASED THERAPY IN MYELODYSPLASTIC SYNDROMES  

PubMed Central

Hypomethylating agents such as 5-azacytidine or decitabine have been a major breakthrough in the treatment of patients with myelodysplastic syndromes (MDS). They have been shown to improve transfusion requirements and change the natural history of the disease. However, with increasing cumulative clinical experience, it has become apparent that these agents are not curative and have their own shortcomings. There are a subgroup of patients who do not respond to frontline therapy and a large, growing cohort of patients that lose response or progress while on hypomethylating agent-based therapy. There are no standard treatment options in this arena and is therefore a focus of significant research interest. Since the mechanisms of resistance to hypomethylating agents are not known, selection of therapy is largely empiric, but must take into account the age, comorbidities, and performance status of the patient as well as the characteristics of the disease at the time treatment failure. Higher intensity approaches and allogeneic stem cell transplant can yield high response rates and long term disease control, but should be limited to a selected cohort of patients who can tolerate the treatment related morbidities. For the majority of patients who will likely be better candidates for lower intensity therapy, several novel, investigational approaches are becoming available. Among these include newer nucleoside analogues, inhibitors of protein tyrosine kinases, molecules that interact with redox signaling within the cell, immunotherapy approaches, and others. Patients with MDS whose disease has failed hypomethylating agent therapy should be referred for clinical trials when available. As we learn more about the patterns and mechanisms of failure, the next challenge will be determining which therapies would be suitable for each individual patient.

Kadia, Tapan M.; Jabbour, Elias; Kantarjian, Hagop

2014-01-01

58

Final height in young women with Turner syndrome after GH therapy: an open controlled study  

Microsoft Academic Search

GH therapy has been applied to patients with Turner syndrome for over a decade, but small sample size, delayed initiation of therapy into adolescent age and comparison with historical control subjects limit the usefulness of these studies for appraisal of the effect of GH on final adult height. We report 49 young women with Turner syndrome who completed a clinical

Ze' ev Hochberg; Zvi Zadik

1999-01-01

59

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

PubMed

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

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

2011-12-01

60

[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

61

Compliance with CPAP therapy in patients with the sleep apnoea\\/hypopnoea syndrome  

Microsoft Academic Search

BACKGROUND--Continuous positive airway pressure (CPAP) therapy is the treatment of choice for the sleep apnoea\\/hypopnoea syndrome. Compliance with this relatively obtrusive therapy has not been well studied. METHODS--Usage of CPAP was investigated in 54 patients with sleep apnoea\\/hypopnoea syndrome (median 36 (range 7-129) apnoeas + hypopnoeas\\/hour slept) over the first 1-3 months after starting CPAP therapy. In all cases CPAP

H M Engleman; S E Martin; N J Douglas

1994-01-01

62

Combination therapy of dyslipidemia in non–insulin-dependent diabetes mellitus and the metabolic syndrome  

Microsoft Academic Search

Non–insulin-dependent diabetes mellitus (NIDDM) and the metabolic syndrome separately and additively increase the risk for\\u000a atherosclerotic cardiovascular disease. Considering the high cardiovascular risk associated with NIDDM and the metabolic syndrome,\\u000a aggressive therapy of dyslipidemia with tailored combination therapy should be considered given informed consent and discussion\\u000a of risks. In addition to statins, niacin, and fibrates, therapies shown to decrease the

Christopher M. Rembold

2004-01-01

63

Older boys benefit from higher initial prednisolone therapy for nephrotic syndrome  

Microsoft Academic Search

Older boys benefit from higher initial prednisolone therapy for nephrotic syndrome.BackgroundA long course of the initial prednisolone therapy has been shown to be more effective than standard-course therapy in reducing relapse rates in children with idiopathic nephrotic syndrome, but it is commonly accompanied by corticosteroid toxicities. There has been no study on prednisolone dosage for the effective treatment of nephrotic

Masahiro Hiraoka; Hirokazu Tsukahara; Shinichi Haruki; Shuhei Hayashi; Nobuaki Takeda; Kazuhiko Miyagawa; Kenji Okuhara; Fumihiko Suehiro; Yusei Ohshima; Mitsufumi Mayumi

2000-01-01

64

Updates in medical and surgical therapies for Tourette syndrome.  

PubMed

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

Malaty, Irene A; Akbar, Umer

2014-07-01

65

Methylprednisolone therapy for short-term prevention of SUNCT syndrome.  

PubMed

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is characterized by severe and frequent daily pain attacks causing transient physical disability for the patients during the headache period. Currently there is no option for abortive treatment of the attacks, mainly due to the short-lived nature and frequency of the repeated headaches, while highly efficacious therapy is also unavailable for short-term prevention. We report rapidly suppressed headache attacks with orally administered methylprednisolone in eight headache periods of three patients with idiopathic, episodic SUNCT syndrome. The remission was maintained until the period was over in all cases. Although the mechanism of methylprednisolone action is unclear, it is probably based on the anti-inflammatory effects of the drug. PMID:19673911

Trauninger, A; Alkonyi, B; Kovács, N; Komoly, S; Pfund, Z

2010-06-01

66

[Acute extremity compartment syndrome : Current concepts in diagnostics and therapy].  

PubMed

Acute compartment syndrome of the upper and lower limbs is observed following trauma, reperfusion or as an intraoperative complication caused by positioning. The pathophysiology of the disorder has been extensively described and is well known as a loss of perfusion due to rising compartmental pressures. It is a serious and potentially limb- and life-threatening complication. Early diagnosis is made primarily based on clinical findings. Early and focused therapy is crucial to prevent the devastating complications of this acute condition. However, diagnosis can be difficult, particularly in unconscious patients. Thus, in uncertain cases, pressure measurements are essential. Dermato-fasciotomy is the routine method to decompress the compartmental space. This review article examines the clinical findings, diagnostic techniques, and management options for the patient with musculoskeletal injuries. PMID:25030961

Sellei, R M; Hildebrand, F; Pape, H-C

2014-07-01

67

[Practical guidelines for diagnosis and therapy of restless legs syndrome].  

PubMed

Restless legs syndrome (RLS) is the most common neurological sleep disorder affecting 10?% of the Caucasian population. The disorder is characterized by painful sensations in the lower limbs, especially during the evening, at night and during rest, resulting in an urge to move the legs and insomnia. As a result the quality of life is significantly reduced. Dopaminergic agents, opioids and anticonvulsants have proven to be effective for RLS with only the former being currently licensed; however, affected patients have to be identified, which is not always the case, especially in outpatient settings. Possible impediments to the adequate management of patients with RLS may include a lack of awareness, comorbidities and other medical conditions mimicking RLS. To overcome some of these difficulties practical guidelines for the diagnosis and therapy of RLS are provided. PMID:24414246

Krenzer, M; Oertel, W; Trenkwalder, C

2014-01-01

68

Metabolic syndrome - Removing roadblocks to therapy: Antigenic immunotherapies?  

PubMed Central

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

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

2014-01-01

69

Laser therapy in metabolic syndrome-related kidney injury.  

PubMed

Metabolic syndrome is characterized by hyperglycemia, hypertension, dyslipidemia and obesity. Diabetes and hypertension are the main causes of chronic end-stage kidney disease in humans. Chronic kidney disease is characterized by kidney inflammation and eventual development of kidney fibrosis. Low-level laser (or light) therapy (LLLT) can be used to relieve pain associated with some inflammatory diseases due to photochemical effects. Despite the known contribution of inflammation to metabolic syndrome and kidney disease, there is scarce information on the potential therapeutic use of LLLT in renal disease. The aim of this randomized, placebo-controlled study was to test the hypothesis that LLLT could modulate chronic kidney injury. Rats with nephropathy, hypertension, hyperlipidemia and type II diabetes (strain ZSF1) were subjected to three different conditions of LLLT or sham treatment for 8 weeks, and then sacrificed 10 weeks later. The main findings of this study are that the LLLT-treated rats had lower blood pressure after treatment and a better preserved glomerular filtration rate with less interstitial fibrosis upon euthanasia at the end of follow-up. This initial proof-of-concept study suggests that LLLT may modulate chronic kidney disease progression, providing a painless, noninvasive, therapeutic strategy, which should be further evaluated. PMID:23360312

Ucero, Alvaro C; Sabban, Bettina; Benito-Martin, Alberto; Carrasco, Susana; Joeken, Stephan; Ortiz, Alberto

2013-01-01

70

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

71

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

72

Differentiation Therapy With Decitabine in Treating Patients With Myelodysplastic Syndrome  

ClinicalTrials.gov

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

2013-02-25

73

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

74

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

Microsoft Academic Search

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

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

2006-01-01

75

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

ERIC Educational Resources Information Center

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

Harris, Susan R.

1981-01-01

76

Yellow nail syndrome associated with thiol compound therapy for rheumatoid arthritis. Two case reports.  

PubMed

Yellow nail syndrome is characterized by ungual dystrophy, lower limb lymphedema, and pleural effusions or bronchiectasis. Rheumatoid arthritis is the autoimmune disorder most often associated with yellow nail syndrome. We report two new cases of yellow nail syndrome in patents receiving thiol compound therapy for rheumatoid arthritis. Eight similar cases have been reported since 1979, suggesting a possible causative effect of this class of drugs. PMID:12184439

Lehuédé, Gaëlle; Toussirot, Eric; Despaux, Jacques; Michel, Fabrice; Wendling, Daniel

2002-06-01

77

Plates-formes de microscopie et fluorescence par resonance de plasmons de surface appliquees a l'imagerie cellulaire  

NASA Astrophysics Data System (ADS)

L'elaboration de nouveaux medicaments repose sur les etudes pharmacologiques, dont le role est d'identifier de nouveaux composes actifs ou de nouvelles cibles pharmacologiques agissant entre autres au niveau cellulaire. Recemment, la detection basee sur la resonance des plasmons de surface (SPR) a ete appliquee a l'etude de reponses cellulaires. Cette methode de detection, permettant d'observer des variations d'indice de refraction associes a de faibles changements de masse a la surface d'un metal, a l'avantage de permettre l'etude d'une population de cellules vivantes en temps reel, sans necessiter l'introduction d'agents de marquage. Pour effectuer la detection au niveau de cellules individuelles, on peut employer la microscopie SPR, qui consiste a localiser spatialement la detection par un systeme d'imagerie. Cependant, la detection basee sur la SPR est une mesure sans marquage et les signaux mesures sont attribues a une reponse moyennee des differentes sources cellulaires. Afin de mieux comprendre et identifier les composantes cellulaires generant le signal mesure en SPR, il est pertinent de combiner la microscopie SPR avec une modalite complementaire, soit l'imagerie de fluorescence. C'est dans cette problematique que s'insere ce projet de these, consistant a concevoir deux plates-formes distinctes de microscopie SPR et de fluorescence optimisees pour l'etude cellulaire, de sorte a evaluer les possibilites d'integration de ces deux modalites en un seul systeme. Des substrats adaptes pour chaque plate-forme ont ete concus et realises. Ces substrats employaient une couche d'argent passivee par l'ajout d'une mince couche d'or. La stabilite et la biocompatibilite des substrats ont ete validees pour l'etude cellulaire. Deux configurations permettant d'ameliorer la sensibilite en sondant les cellules plus profondement ont ete evaluees, soit l'emploi de plasmons de surface a longue portee et de guides d'onde a gaine metallique. La sensibilite accrue de ces configurations a aussi ete demontree pour un usage en biodetection cellulaire. Une plate-forme permettant de mesurer la spectroscopie SPR simultanement avec l'acquisition d'images de fluorescence a ete realisee. Cette plate-forme a ensuite ete validee par l'etude de reponses cellulaires suite a une stimulation pharmacologique. Puis, un systeme base sur la microscopie SPR a ete concu et caracterise. Son emploi pour l'etude de reponses au niveau de cellules individuelles a ete demontre. Finalement, les forces et faiblesses des substrats et des plates-formes realisees au cours de la these ont ete evaluees. Des possibilites d'amelioration sont mises de l'avant et l'integration des modalites de microscopie SPR et de fluorescence suite aux travaux de la these est discutee. Les realisations au cours de cette etude ont donc permis d'identifier les composantes cellulaires impliquees dans la generation du signal mesure en biodetection SPR. Mots-cles : Resonance des plasmons de surface, microscopie SPR, plasmons de surface a longue portee LRSPR, guide d'onde a gaine metallique MCWG, fluorescence exaltee par plasmons de surface SPEF, biodetection cellulaire, imagerie SPR.

Chabot, Vincent

78

Cellules souches et thérapie cellulaire. Contribution au débat éthique  

Microsoft Academic Search

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

Denys Pellerin

2002-01-01

79

Testosterone Therapy in Adult Men with Androgen Deficiency Syndromes  

MedlinePLUS

... health. What are the signs and symptoms of AD syndromes in adult men? AD syndromes caused by low testosterone levels in the ... common in adult men. Signs and symptoms of AD include: • Drop in sex drive (libido) • Erectile dysfunction ( ...

80

Cognitive behavior therapy for night eating syndrome: a pilot study.  

PubMed

Because no studies of psychotherapy treatments for night eating syndrome (NES) have been published, we conducted a pilot study of a 10-session cognitive behavior therapy (CBT) for NES. Twenty-five patients (19 female, 6 male) were screened and comprehensively assessed before being enrolled. At each visit, patients completed the Night Eating Symptom Scale (NESS), were weighed, and number of awakenings and the number of nocturnal ingestions and daily caloric intake were calculated from weekly food and sleep records. Mixed model regression analyses [of the data] showed significant decreases in caloric intake after dinner (35.0% to 24.9%); number of nocturnal ingestions (8.7 to 2.6 per week); weight (82.5 to 79.4 kg); and NESS score (28.7 to 16.3; all p values <0.0001). Number of awakenings per week, depressed mood, and quality of life also improved significantly (p values <.02). This first clinical trial of CBT for NES shows significant improvements in the core aspects of NES and weight reduction, suggesting the need for a controlled treatment trial. PMID:20405767

Allison, Kelly C; Lundgren, Jennifer D; Moore, Reneé H; O'Reardon, John P; Stunkard, Albert J

2010-01-01

81

Investigational approaches to therapies for restless legs syndrome.  

PubMed

Introduction: Restless Legs Syndrome (RLS) is a common neurological disorder that impairs nocturnal rest, causing decreased alertness, depressed mood, reduced job performance and poor quality of life. In patients affected by severe RLS, pharmacological treatment is mandatory. Areas covered: The present review is based on an extensive Internet and PubMed search from 1994 - 2014. It focuses on drugs currently in development for the treatment of RLS. Expert opinion: The drugs currently available for treating RLS do not always allow the patient to obtain a dose capable of controlling the symptoms, particularly in the long term. There is still the need for effective and well-tolerated new drugs. Monoamine oxidase B inhibitors could be good candidates for the initial treatment of RLS, sparing stronger dopaminergic agents for later stages of the disease. Oxycodone-naloxone has demonstrated a significant and sustained effect on patients with severe RLS inadequately controlled with first-line drugs; it could be used as a long-term treatment option in severe cases of RLS for which alternative satisfactory drug regimens are unavailable. There is a paucity of data comparing medications in head-to-head trials to determine their relative effectiveness and adverse event profiles. Furthermore, there is also a need for further studies that evaluate nondopaminergic agonists and combination therapies for treating RLS. PMID:24708072

de Biase, Stefano; Merlino, Giovanni; Lorenzut, Simone; Valente, Mariarosaria; Gigli, Gian Luigi

2014-06-01

82

HYPOGONADISM AND METABOLIC SYNDROME: IMPLICATIONS FOR TESTOSTERONE THERAPY  

Microsoft Academic Search

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

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

2005-01-01

83

Association of asthma therapy and churg-strauss syndrome: An analysis of postmarketing surveillance data  

Microsoft Academic Search

Background: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis (AGA), is a rare vasculitis that occurs in patients with bronchial asthma. The nature of the association of CSS with various asthma therapies is unclear.Objective: This study investigated the associations of different multidrug asthma therapy regimens and the reporting of AGA (the preferred code for CSS in the coding dictionary

William DuMouchel; Eric T. Smith; Richard Beasley; Harold Nelson; Xionghu Yang; David Fram; June S. Almenoff

2004-01-01

84

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

PubMed

Stevens-Johnson syndrome (SJS) is a severe cutaneous eruption that most often appears as an adverse reaction to medication. In this report, we present two children with brain tumour who developed SJS while receiving cranial irradiation and anticonvulsant therapy with phenobarbital. Concomitant application of these two therapies may play an important role in the occurrence of the disease. PMID:17589805

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

2007-11-01

85

Physical therapy alters recruitment of the vasti in patellofemoral pain syndrome  

Microsoft Academic Search

COWAN, S. M., K. L. BENNELL, K. M. CROSSLEY, P. W. HODGES, and J. MCCONNELL. Physical therapy alters recruitment of the vasti in patellofemoral pain syndrome. Med. Sci. Sports Exerc., Vol. 34, No. 12, pp. 1879 -1885, 2002. Purpose: To investigate the effect of physical therapy treatment on the timing of electromyographic (EMG) activity of the vasti in individuals with

SALLIE M. COWAN; KIM L. BENNELL; KAY M. CROSSLEY; PAUL W. HODGES; JENNY MCCONNELL

2002-01-01

86

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

PubMed

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

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

2011-01-01

87

Current gut-directed therapies for irritable bowel syndrome.  

PubMed

Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder that can present with a wide array of symptoms that make treatment difficult. Current therapies are directed at relieving symptoms of abdominal pain or discomfort, bloating, constipation, and diarrhea. Pharmacologic agents used to treat IBS-associated pain include myorelaxants, peppermint oil, and peripherally acting opiates. Dicyclomine and hyoscyamine, the two myorelaxants available in the United States, have not been proven effective in reducing abdominal pain in patients with IBS. The efficacy of peppermint oil is debated, but methodological problems with existing studies preclude definitive judgment. Loperamide is ineffective for relief of abdominal pain. For IBS patients with excessive abdominal bloating, a small number of studies suggest that bacterial eradication with gut-directed antibiotics and bacterial reconstitution with nonpathogenic probiotics may reduce flatulence. For constipation-predominant (C-IBS) symptoms, current treatment options include fiber supplementation, polyethylene glycol, and tegaserod. Soluble fibers (ispaghula, calcium polycarbophil, psyllium) are more effective than insoluble fibers (wheat bran, corn fiber) in alleviating global symptoms and relieving constipation, although fiber in general has marginal benefit in treatment of overall IBS symptoms. Polyethylene glycol increases bowel frequency in chronic constipation, but its overall efficacy against IBS is unclear. Tegaserod, a 5-HT(4) agonist, demonstrates superiority over placebo in improving bowel frequency and stool consistency and alleviating abdominal pain and bloating in women with C-IBS. Overall global symptoms are modestly improved with tegaserod when compared with placebo. Additional agents under investigation for C-IBS include the ClC(2) chloride channel opener lubiprostone, mu-opioid receptor antagonist alvimopan, and 5-HT(4) agonist renzapride. For diarrhea-predominant (D-IBS) symptoms, available therapies include loperamide, alosetron, and clonidine. Alosetron, a 5-HT(3) antagonist, is superior to placebo for reducing bowel frequency, improving stool consistency, and relieving abdominal pain in women with D-IBS. However, alosetron is available under a restricted license because of concerns for ischemic colitis and severe constipation necessitating colectomy. Clonidine may be helpful in alleviating global symptoms for D-IBS patients. PMID:16836950

Chang, Howard Y; Kelly, Eoin C; Lembo, Anthony J

2006-07-01

88

[Prophylaxis and therapy of the acid aspiration syndrome].  

PubMed

The best prevention of the aspiration syndrome begins with early identification of any patient at risk. Reduction of gastric volume and elevation of gastric pH can be achieved by premedication with glycopyrrolate (0.3 mg i.m.) and preoperative administration of H2-receptor antagonists (150 mg ranitidine p.o. 6-8 h and 80 mg i.m./i.v. 60 min before induction). Transportation of stomach contents into the duodenum can further be accelerated by 10 mg metoclopramide i.v. 20-40 min before induction. Metoclopramide will also elevate lower esophageal sphincter tone. Rapid elevation of gastric pH can be achieved by oral administration of 15-30 ml 0.3 mol sodium citrate. Induction of anesthesia should be performed as a "rapid sequence induction". Manual compression of the esophagus (Sellick manoever) may prevent gastric regurgitation. In cases of pulmonary aspiration, consequent therapy has to be initiated as soon as possible to limit broncho-alveolar damage. After endotracheal intubation the upper respiratory tract should be cleared of stomach contents by suction. Further efforts should include artificial ventilation with a high FiO2 and low PEEP (5-10 cm H2O) as well as vigorous volume substitution to maintain cardiovascular stability. Corticosteroids (200 mg Hydrocortisone i.v. may have a beneficial effect if given within 5 min after aspiration. Severe bronchospasm may respond to aminophylline (5 mg/kg i.v. as an initial bolus) or inhalation of beta-adrenergics (terbutaline 0.75-1.5 mg/inh). Bronchial lavage will rather damage than improve broncho-alveolar integrity and cannot be recommended.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3322095

Ellmauer, S

1987-11-01

89

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

PubMed

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

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

2014-04-01

90

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

PubMed Central

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

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

2014-01-01

91

Secondary renal Fanconi syndrome caused by valproate therapy  

Microsoft Academic Search

Although renal Fanconi syndrome resulting from valproate (VPA) has occasionally been reported, the detailed clinical characteristics of this disease remain unclear. To clarify the clinical features of patients with VPA-induced Fanconi syndrome, we analyzed the clinical and laboratory data of seven affected patients. All patients were children, were severely disabled and required tube feeding. Five patients required treatment with multiple

Toru Watanabe; Hideto Yoshikawa; Sawako Yamazaki; Yuki Abe; Tokinari Abe

2005-01-01

92

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

Microsoft Academic Search

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

Tony Attwood

2004-01-01

93

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

94

Sick sinus syndrome aggravated by carbamazepine therapy for epilepsy  

Microsoft Academic Search

A case of sick sinus syndrome aggravated by carbamazepine prescribed for epilepsy is described. A 70 year old woman with previously stable psychomotor epilepsy experienced syncope of increased frequency after her anticonvulsant was changed to carbamazepine. ECG monitoring confirmed a severe sick sinus syndrome which improved on carbamazepine withdrawal. Failure to distinguish carbamazepine-induced cardiac syncope from epileptic attacks may lead

K. A. Hewetson; A. E. Ritch; R. D. Watson

1986-01-01

95

Chronic Fatigue Syndrome and Women: Can Therapy Help?  

ERIC Educational Resources Information Center

Presents current research on chronic fatigue syndrome, which currently afflicts mostly females between ages of 25 and 55. Notes that, because depression is common symptom of chronic fatigue syndrome, mental health practitioners are often involved with victims and must formulate appropriate treatment strategy that considers physiological,…

Burke, Susan G.

1992-01-01

96

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

2013-01-01

97

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

98

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

99

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

100

[Cushing syndrome: Physiopathology, etiology and principles of therapy].  

PubMed

The most frequent cause of Cushing's syndrome is iatrogenic, as Cushing's syndrome is the unavoidable consequence of long-term glucocorticoid treatment using more than 7.5 mg prednisone per day. The most frequent cause of endogenous Cushing's syndrome is Cushing's disease (CD), which is an ACTH dependent hypercortisolism linked to a pituitary corticotroph adenoma. This adenoma is often very small, its diagnosis may require bilateral inferior petrosal sinus sampling and the first line treatment of CD is transsphenoidal surgery by an expert neurosurgeon. The second line treatments include drugs that can act either on the pituitary adenoma or on adrenal steroidogenesis, pituitary radiotherapy or bilateral adrenalectomy. Ectopic ACTH dependent Cushing's syndrome is linked either to poorly differentiated endocrine tumors with a very poor prognosis, such as small cell lung cancer, or to well differentiated endocrine tumors, such as bronchial carcinoid tumors, which have a good prognosis when treated by surgery, but may be very difficult to localize. Adrenal Cushing's syndromes, which are independent of pituitary ACTH secretion, include adrenal cortex carcinoma, which requires abdominal surgery with extended adrenalectomy by an expert surgeon, adrenal adenoma which is treated by laparoscopic unilateral adrenalectomy and bilateral macronodular hyperplasia, whose surgical treatment may require unilateral or bilateral adrenalectomy. Treatment of Cushing's syndrome generally leads to spectacular clinical results, which must not hide the fact that the reversibility of some signs is actually incomplete. This underlines the need for a timely multidisciplinary management of the patients by an expert team. PMID:24656295

Chabre, Olivier

2014-04-01

101

Metastatic mediastinal carcinoid presenting as superior mediastinal syndrome and treated with peptide receptor radionuclide therapy.  

PubMed

A 43-year-old man with known superior mediastinal syndrome due to (99m)Tc HYNIC DOTA SPECT/CT and (18)F-FDG PET tracer avid inoperable metastatic carcinoid received peptide receptor based radionuclide therapy (PRRT) with (177)Lu-DOTATATE. Repeat evaluation at 3 months post-therapy demonstrated stable disease with alleviation of patient's symptoms and remarkable improvement in the quality of life. PRRT with (177)Lu-DOTATATE may be an option for inoperable mediastinal carcinoids with superior mediastinal syndrome. PMID:23455519

Malhotra, Gaurav; Asopa, Ramesh V

2014-01-01

102

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

103

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

Microsoft Academic Search

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

Lale Altan; Umit Bingöl; Mehtap Aykaç; Merih Yurtkuran

2005-01-01

104

Antithymocyte globulin (ATG)-based therapy in patients with myelodysplastic syndromes  

Microsoft Academic Search

The purpose of this study was to determine the efficacy of and tolerance to antithymocyte globulin (ATG)-based therapy in patients with myelodysplastic syndrome (MDS). Therapy consisted of ATG 40 mg\\/kg\\/day daily intravenously (i.v.) for 4 days; cyclosporine daily orally for 6 months with levels titrated between 200 and 400 mg\\/dl; and methylprednisone 1 mg\\/kg i.v. daily before each dose of

S Yazji; F J Giles; A-M Tsimberidou; E H Estey; H M Kantarjian; S A O'Brien; R Kurzrock

2003-01-01

105

Efficacy of methylprednisolone pulse therapy on neuroleptic malignant syndrome in Parkinson’s disease  

Microsoft Academic Search

Background: Neuroleptic malignant syndrome (NMS) is a dangerous complication in patients with Parkinson’s disease (PD).Aims: To evaluate the efficacy of methylprednisolone pulse therapy compared to placebo in PD patients with NMS.Methods: In a double blind, placebo controlled study, 20 PD patients with NMS received steroid pulse therapy for three days, and 20 PD patients received placebo. Both groups received levodopa,

Y Sato; T Asoh; N Metoki; K Satoh

2003-01-01

106

Transforming genes and chromosome aberrations in therapy-related leukemia and myelodysplastic syndrome  

Microsoft Academic Search

Summary The presence of activated transforming genes was investigated in four patients with therapy-related leukemia and in three with therapy-related myelodysplastic syndrome. DNA of bone marrow cells from six of the patients exhibited transforming activity in the tumorigenicity assay. Five of the six patients who were positive in the tumorigenicity assay contained activated N-ras oncogenes, and three contained activated K-ras

K. Inokuchi; N. Amuro; M. Futaki; K. Dan; T. Shinohara; S. Kuriya; T. Okazaki; T. Nomura

1991-01-01

107

Study on Treatment of polycystic ovarian syndrome with Infertility by combined therapy of Chinese herbal medicine and compound cyproterone acetate  

Microsoft Academic Search

Objective: To evaluate the effect of combined therapy of Chinese herbal medicine and compound cyproterone acetate (CPA) in treating\\u000a non-obesity polycystic ovarian syndrome (PCOS) and to explore its mechanism in improving withdrawal ovulation.Methods: Eighty-six patients of non-obesity PCOS, typed as Shen-deficiency with blood stasis Syndrome or Shen-deficiency with Phlegm-Dampness\\u000a Syndrome by Syndrome Differentiation in traditional Chines medicine, were randomly divided

Tao Li-li; Chen Xiao-ping; Gu Zheng-tian

2003-01-01

108

Exposure and mindfulness based therapy for irritable bowel syndrome – An open pilot study  

Microsoft Academic Search

We conducted a study of a group therapy based on exposure and mindfulness in the treatment of irritable bowel syndrome (IBS). Out of 49 outpatients, most of whom were referred from gastroenterological clinics, 34 entered into the 10-week treatment. Patients were assessed before, immediately after and 6 months after treatment. The assessments consisted of a gastrointestinal symptom diary, self-report questionnaires

Brjánn Ljótsson; Sergej Andréewitch; Erik Hedman; Christian Rück; Gerhard Andersson; Nils Lindefors

2010-01-01

109

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

ERIC Educational Resources Information Center

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

Pienaar, Dorothea

2012-01-01

110

Cognitive Behavior Therapy for Relatively Active and for Passive Chronic Fatigue Syndrome Patients  

ERIC Educational Resources Information Center

In chronic fatigue syndrome (CFS), facilitating, initiating, and perpetuating factors are distinguished. Although somatic factors might have initiated symptoms in CFS, they do not explain the persistence of fatigue. Cognitive behavior therapy (CBT) for CFS focuses on factors that perpetuate and prolong symptoms. Recently it has been shown that,…

Bazelmans, Ellen; Prins, Judith; Bleijenberg, Gijs

2006-01-01

111

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

112

Oral carnitine therapy in children with cystinosis and renal Fanconi syndrome  

SciTech Connect

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-(/sup 3/H)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.

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

1988-02-01

113

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

114

Alternative Therapies for Obstructive Sleep Apnea Syndrome: Behavioral and Pharmacological Options  

Microsoft Academic Search

Many people with obstructive sleep apnea syndrome (OSAS) are treated well with continuous positive airway pressure (CPAP) but an important number cannot tolerate it or have ongoing symptoms despite good compliance.Alternative therapies are needed for these patients.Weight loss should be encouraged in all obese subjects since this may reduce or even cure the OSAS and the risks from associated disorders

I. Smith

2006-01-01

115

Transient bone marrow oedema syndrome in an HIV-infected patient on highly active antiretroviral therapy.  

PubMed

Bone marrow oedema syndrome (BMES, also known as transient osteoporosis) is an uncommon, self-limiting condition characterized by disabling pain, reversible osteopaenia on X-rays and by bone marrow oedema pattern on magnetic resonance imaging (MRI). Here we describe the first reported case of BMES in an HIV-positive patient on highly active antiretroviral therapy. PMID:22581878

Vera, J H; Walker, A E; Kinderlerer, A; Cooke, G S

2012-03-01

116

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

ERIC Educational Resources Information Center

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

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

2008-01-01

117

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

118

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

119

Pirfenidone: a potential new therapy for restrictive allograft syndrome?  

PubMed

This case report describes the evolution of pulmonary function findings (FVC, FEV1 and TLC) and CT features with pirfenidone treatment for restrictive allograft syndrome following lung transplantation. Furthermore, we herein report hypermetabolic activity on (18) F-FDG PET imaging in this setting, which could indicate active fibroproliferation and pleuroparenchymal remodeling. These findings may warrant further investigation. PMID:24102752

Vos, R; Verleden, S E; Ruttens, D; Vandermeulen, E; Yserbyt, J; Dupont, L J; Van Raemdonck, D E; De Raedt, N; Gheysens, O; De Jong, P A; Verleden, G M; Vanaudenaerde, B M

2013-11-01

120

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

121

Drug resistant neuroleptic malignant syndrome and the role of electroconvulsive therapy.  

PubMed

Neuroleptic malignant syndrome is considered as a rare but potentially fatal complication of neuroleptic medications e.g.,antipsychotics, sedatives and anti emetics. It is characterized by hyperthermia, muscle rigidity, an elevated creatine kinase level and autonomic instability. The syndrome often develops after the start of antipsychotic or a sudden increase in dosage of the neuroleptic medication or in states of dehydration. Treatment is mainly supportive and includes withdrawal of the neuroleptic medication and, possibly, administration of drugs such as dantrolene and bromocriptine. In rare cases where drugs treatment remains ineffective a trial of electroconvulsive therapy is being given. The case presented is a drug resistant case of Neuroleptic Malignant Syndrome where finally electroconvulsive therapy was effective. PMID:24864649

Hashim, Husnain; Zeb-un-Nisa; Alrukn, Suhail Abdulla Mohammad; Al Madani, Abubaker Abdul Rahman Shaffi

2014-04-01

122

Fatty Acids in the Causation and Therapy of Metabolic Syndrome  

Microsoft Academic Search

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

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

123

Current recommendations for bladder instillation therapy in the treatment of interstitial cystitis/bladder pain syndrome.  

PubMed

Bladder instillation therapy refers to the direct introduction of medication into the bladder and is a common treatment modality for patients with interstitial cystitis/bladder pain syndrome (IC/BPS) who have failed conservative and oral therapies. The current American Urological Association (AUA) recommendations list three medications as options for IC/BPS instillation therapy: dimethyl sulfoxide, heparin, and lidocaine. The purpose of this review is to examine the evidence behind the recommendations for these medications. We also examine several historical or experimental therapies that do not hold recommendations but are still used on rare occasion. Finally, we discuss our bladder instillation strategies as well as potential future research and development in intravesicular therapy. PMID:24101384

Colaco, Marc A; Evans, Robert J

2013-10-01

124

Vitamin B status and response to replacement therapy in patients with burning mouth syndrome.  

PubMed

The aim of the study was to determine, in a group of patients with therapy-resistant burning mouth syndrome (BMS), the possible deficiency of vitamins B1, B2, and B6 and the effect of proper vitamin replacement therapy. Sixteen individuals, aged 47 to 81 years, participated in the study. All underwent a base-line examination comprising anamnestic information, subjective assessment of symptoms, dietary registration, salivary analysis, and serum analysis of thiamine (B1), riboflavine (B2), and pyridoxine (B6). Fifteen individuals had low thiamine and/or riboflavine levels in accordance with suggested levels in the literature and were given replacement therapy. No effect on BMS of vitamin replacement therapy or placebo therapy could be demonstrated. PMID:1776404

Hugoson, A; Thorstensson, B

1991-12-01

125

Oral antiplatelet therapy in acute coronary syndromes: update 2012  

Microsoft Academic Search

Dual antiplatelet therapy (DAPT), usually consisting of clopidogrel and acetylsalicylic acid (ASA), has come into discussion in recent years due to an increasing number of major adverse cardiac events based on insufficient ADP-mediated platelet inhibition with clopidogrel, mainly explained by drug interactions or genetic variants slowing or hindering the bioactivation of the prodrug clopidgrel into an active metabolite. Accordingly, new

Thomas Höchtl; Peter R Sinnaeve; Tom Adriaenssens; Kurt Huber

2012-01-01

126

Complement therapy in atypical haemolytic uraemic syndrome (aHUS).  

PubMed

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

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

2013-12-15

127

Fragile X syndrome neurobiology translates into rational therapy.  

PubMed

Causal genetic defects have been identified for various neurodevelopmental disorders. A key example in this respect is fragile X syndrome, one of the most frequent genetic causes of intellectual disability and autism. Since the discovery of the causal gene, insights into the underlying pathophysiological mechanisms have increased exponentially. Over the past years, defects were discovered in pathways that are potentially amendable by pharmacological treatment. These findings have inspired the initiation of clinical trials in patients. The targeted pathways converge in part with those of related neurodevelopmental disorders raising hopes that the treatments developed for this specific disorder might be more broadly applicable. PMID:24508819

Braat, Sien; Kooy, R Frank

2014-04-01

128

[Susac's syndrome: improvement with combined cyclophosphamide and intravenous immunoglobulin therapy].  

PubMed

We report a case of Susac's syndrome characterized by subacute encephalopathy, bilateral hearing loss and multiple bilateral branch retinal artery occlusions in a forty-year-old-white-woman. Brain Magnetic Resonance Imaging showed on T2-weighted images multiple, punctate areas of increased signal intensity in periventricular white matter, gray matter and brainstem most of them being enhanced by gadolinium. Cerebrospinal fluid was acellular but with an increased protein level (1.66 g/l). Treatment with cyclophosphamid and intravenous immmunoglobulin resulted in dramatic improvement of the clinical status over the following months and CSF normalization. PMID:10992124

Papeix, C; Laloum, L; Richet, A; Ayache, D; Moulignier, A; Héran, F; Bakouche, P; Gout, O

2000-09-01

129

Complement therapy in atypical haemolytic uraemic syndrome (aHUS)  

PubMed Central

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

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

2013-01-01

130

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

PubMed Central

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

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

1998-01-01

131

Early development of immune reconstitution inflammatory syndrome related to Pneumocystis pneumonia after antiretroviral therapy.  

PubMed

Immune reconstitution inflammatory syndrome is a recognized complication after the initiation of combination antiretroviral therapy (cART). We report a patient who developed life-threatening pulmonary immune reconstitution inflammatory syndrome (IRIS) three days after initiation of cART. We reviewed published cases of IRIS after Pneumocystis pneumonia (PCP), in particular the time from initiation of cART to IRIS event. The median duration from the initiation of cART to the onset of IRIS was 15 days in the 33 patients reviewed. This report alerts clinicians to the rapidity of the development of pulmonary IRIS following PCP after the initiation of cART. PMID:24122663

Mok, Hoi Ping; Hart, Elizabeth; Venkatesan, Pradhib

2014-04-01

132

Randomized Multicenter Feasibility Trial of Myofascial Physical Therapy for Treatment of Urologic Chronic Pelvic Pain Syndrome  

PubMed Central

Objectives To determine the feasibility of conducting a randomized clinical trial designed to compare two methods of manual therapy (myofascial physical therapy (MPT) and global therapeutic massage (GTM)) among patients with urologic chronic pelvic pain syndromes. Materials and Methods Our goal was to recruit 48 subjects with chronic prostatitis/chronic pelvic pain syndrome or interstitial cystitis/painful bladder syndrome at six clinical centers. Eligible patients were randomized to either MPT or GTM and were scheduled to receive up to 10 weekly treatments, each 1 hour in duration. Criteria to assess feasibility included adherence of therapists to prescribed therapeutic protocol as determined by records of treatment, adverse events which occurred during study treatment, and rate of response to therapy as assessed by the Patient Global Response Assessment (GRA). Primary outcome analysis compared response rates between treatment arms using Mantel-Haenszel methods. Results Twenty-three (49%) men and 24 (51%) women were randomized over a six month period. Twenty-four (51%) patients were randomized to GTM, 23 (49%) to MPT; 44 (94%) patients completed the study. Therapist adherence to the treatment protocols was excellent. The GRA response rate of 57% in the MPT group was significantly higher than the rate of 21% in the GTM treatment group (p=0.03). Conclusions The goals to judge feasibility of conducting a full-scale trial of physical therapy methods were met. The preliminary findings of a beneficial effect of MPT warrants further study.

FitzGerald, Mary P; Anderson, Rodney U; Potts, Jeannette; Payne, Christopher K; Peters, Kenneth M; Clemens, J Quentin; Kotarinos, Rhonda; Fraser, Laura; Cosby, Annamarie; Fortman, Carole; Neville, Cynthia; Badillo, Suzanne; Odabachian, Lisa; Sanfield, Anna; O'Dougherty, Betsy; Halle-Podell, Rick; Cen, Liyi; Chuai, Shannon; Landis, J Richard; Kusek, John W; Nyberg, Leroy M

2010-01-01

133

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

134

Therapy-related myelodysplastic syndrome with monosomy 5 and 7 following successful therapy for acute promyelocytic leukemia with anthracyclines.  

PubMed

Myelodysplastic syndrome (MDS) in patients treated for acute promyelocytic leukemia (APL) is a rare event. We describe a patient with APL who developed MDS 40 months after entering complete remission (CR). Karyotypic analysis revealed monosomy 5 and 7, which are cytogenetic changes usually occurring after the use of alkylating agents. The patient had received only anthracyclines as potential leukemogenic drugs. A review of the literature on t-AML/MDS occurring after successful therapy for APL showed three similar cases. These observations suggest that anthracyclines may cause t-AML/MDS similar to that induced by alkylating agents. PMID:12613533

Athanasiadou, A; Saloum, R; Zorbas, I; Tsompanakou, A; Batsis, I; Fassas, A; Anagnostopoulos, A

2002-12-01

135

From FMRP Function to Potential Therapies for Fragile X Syndrome.  

PubMed

Fragile X syndrome (FXS) is caused by mutations in the fragile X mental retardation 1 (FMR1) gene. Most FXS cases occur due to the expansion of the CGG trinucleotide repeats in the 5' un-translated region of FMR1, which leads to hypermethylation and in turn silences the expression of FMRP (fragile X mental retardation protein). Numerous studies have demonstrated that FMRP interacts with both coding and non-coding RNAs and represses protein synthesis at dendritic and synaptic locations. In the absence of FMRP, the basal protein translation is enhanced and not responsive to neuronal stimulation. The altered protein translation may contribute to functional abnormalities in certain aspects of synaptic plasticity and intracellular signaling triggered by Gq-coupled receptors. This review focuses on the current understanding of FMRP function and potential therapeutic strategies that are mainly based on the manipulation of FMRP targets and knowledge gained from FXS pathophysiology. PMID:24346713

Sethna, Ferzin; Moon, Changjong; Wang, Hongbing

2014-06-01

136

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

PubMed

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

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

2014-04-30

137

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

PubMed Central

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

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

2014-01-01

138

The Spectrum of Monogenic Autoinflammatory Syndromes: Understanding Disease Mechanisms and Use of Targeted Therapies  

PubMed Central

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

Glaser, Rachel L.; Goldbach-Mansky, Raphaela

2009-01-01

139

Group Therapy for Boys with Features of Asperger Syndrome and Concurrent Learning Disabilities: Finding a Peer Group  

Microsoft Academic Search

In this paper, we present a pilot group therapy model with modifications, for boys who meet the criteria of Asperger Syndrome as defined by the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV; American Psychiatric Association, 1994), and who have concurrent learning disabilities. It is recognized that learning disabilities are often a feature of Asperger Syndrome. Children and

Faye Mishna; Barbara Muskat

1998-01-01

140

Patient characteristics associated with the choice of triple antithrombotic therapy in acute coronary syndromes.  

PubMed

Evidence regarding the use of dual antiplatelet therapy and oral anticoagulation (i.e., triple therapy) in acute coronary syndromes (ACS) is limited. We evaluated the characteristics associated with the choice of triple therapy in ACS. Using the Get With The Guidelines (GWTG) Coronary Artery Disease national registry, we studied patients with ACS at 361 sites in the United States from 2004 to 2007. Both univariate analysis and multivariate logistic regression analysis were used to assess the factors associated using triple therapy on discharge. The Generalized Estimating Equation method was used to account for within-hospital clustering in modeling. A total of 86,304 patients presented with ACS during the study period. At discharge, 3,933 patients (4.6%) were prescribed triple therapy, 60,716 patients (70.4%) received dual antiplatelet therapy, 2,348 patients (2.7%) received single antiplatelet therapy plus oral anticoagulation, 19,065 patients (22.1%) received antiplatelet monotherapy, and 242 patients (0.3%) received oral anticoagulation alone. Patients with a history of atrial fibrillation (odds ratio 7.01, 95% confidence interval 6.06 to 8.12; p <0.001), documented new-onset atrial fibrillation (odds ratio 3.76, 95% confidence interval 2.87 to 4.93; p <0.001), or history of atrial flutter (odds ratio 3.38, 95% confidence interval 2.15 to 5.32; p <0.001) were more frequently discharged with triple therapy. In conclusion, for patients with ACS, atrial fibrillation and atrial flutter were most strongly associated with the use of triple therapy; however, this therapy was used less often than dual or single antiplatelet therapy. PMID:19840557

Depta, Jeremiah P; Cannon, Christopher P; Fonarow, Gregg C; Zhao, Xin; Peacock, W Frank; Bhatt, Deepak L

2009-11-01

141

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

142

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

PubMed Central

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

Onwubalili, J. K.

1988-01-01

143

Massive exudative retinal detachment following photodynamic therapy for retinal hemangioma in von Hippel-Lindau Syndrome.  

PubMed

Photodynamic therapy (PDT) is a common treatment on retinal capillary hemangioma. We applied PDT to a patient with von Hippel-Lindau(VHL) syndrome and she developed severe massive exudative retinal detachment the next day, which is a rare complication for PDT. After intraocular anti-VEGF agent and peribulbar dexamethasone several times to the patient, her subretinal fluid disappeared and hemangiomas atrophied. Treatment with Anti-VEGF agent and corticosteroid is effective for such complication. PMID:24632330

Chen, Yao; Liu, Hong; Zhang, Kun; Gao, Ling

2014-06-01

144

Autoimmune haemolytic anaemia and renal Fanconi syndrome caused by valproate therapy  

Microsoft Academic Search

A 2-year-old girl developed autoimmune haemolytic anaemia and renal Fanconi syndrome secondary to valproate therapy. Valproate (VPA) is the most common anti-epileptic drug used to treat patients with epilepsy. Although gastrointestinal disturbances, hepatic enzyme elevations, neurological disturbance, alopecia, weight gain, pancreatitis, thrombocytopenia and renal injury are welldocumented side-effects of VPA [2,6], autoimmune haemolytic anaemia (AIHA) has never been described. We

Toru Watanabe; Kosuke Nakayasu; Yoshihisa Nagayama

2005-01-01

145

Effective prophylactic therapy for cyclic vomiting syndrome in children using valproate  

Microsoft Academic Search

This trial sought to evaluate our experience using the antimigraine prophylactic drug, use of valproate for the prophylactic management of cyclic vomiting syndrome (CVS) in children. Thirteen children diagnosed with severe CVS were enrolled. Prophylactic therapy consisted of valproate administered at a dose of 10–40mg\\/kg\\/day. Upon enrollment in the study, all patients underwent diagnostic tests to rule out organic causes

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

2009-01-01

146

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

147

Highly Active Antiretroviral Therapy-Associated Metabolic Syndrome and Cardiovascular Risk  

Microsoft Academic Search

The introduction of highly active antiretroviral therapy (HAART) has significantly modified the course of human immunodeficiency virus (HIV) disease, with longer survival and improved quality of life of HIV-infected subjects. However, HAART regimens, especially those including protease inhibitors, have been shown to cause in a high proportion of HIV-infected patients a metabolic syndrome (lipodystrophy\\/lipoatrophy, dyslipidemia, type 2 diabetes mellitus, insulin

Giuseppe Barbaro; Giorgio Barbarini

2006-01-01

148

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

Microsoft Academic Search

We systematically reviewed empirical studies that investigated the use of cognitive-behavioral therapy (CBT) for premenstrual\\u000a syndrome (PMS) or premenstrual dysphoric disorder (PMDD). Our multi-database search identified seven published empirical reports.\\u000a Three were identified as randomized controlled trials (RCTs). The methods utilized to investigate therapeutic efficacy of\\u000a CBT in these studies varied widely from case reports to RCTs with pharmacotherapy comparison

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

2009-01-01

149

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

PubMed

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

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

2013-06-01

150

Serial retinal fluorescein angiography and immune therapy in Susac's syndrome.  

PubMed

We report a patient with Susac's disease presenting classically in a young female with an encephalopathy and visual disturbance with later deafness and tinnitus. Her encephalopathy settled, but subsequent serial fluorescein angiograms allowed sensitive monitoring of continuing sub-clinical disease activity, and provide evidence of a clear therapeutic response to immune suppression with tacrolimus (but not steroids alone)--and of a lack of efficacy of nimodipine and aspirin. We believe this single case study has both pathogenetic and useful practical implications: the apparently favourable response to immunosuppression lends support to the hypothesis that Susac's Syndrome is an immune-mediated disease; while the presence during symptomatic clinical remission of sporadic, multi-focal episodes of hyper-fluorescence, suggestive of breakthrough vasculopathy despite treatment, underlines the fact that the natural history of this rare condition is still not fully understood. Fluorescein angiography is proposed as a sensitive and important approach to the monitoring of sub-clinical disease activity, and so optimising immune suppressive treatment. PMID:19577774

Mallam, Beth; Damato, Erika M; Scolding, Neil J; Bailey, Clare

2009-10-15

151

Antiplatelet therapy in acute coronary syndromes: focus on ticagrelor  

PubMed Central

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

Birkeland, Kade; Parra, David; Rosenstein, Robert

2010-01-01

152

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

PubMed

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

Bizzarri, M; Carlomagno, G

2014-07-01

153

Prediction of Pubertal Growth at Start of Estrogen Replacement Therapy in Turner Syndrome  

PubMed Central

Abstract In estrogen replacement therapy in Turner syndrome, there is no report which recommends the timing of the start of estrogen therapy in relation to height or adult height prediction. We have established a prediction model for pubertal growth (height difference from the start of estrogen therapy until adult height) at the start of estrogen replacement therapy. Twenty-seven Turner girls without spontaneous puberty were divided into two groups according to birth years; Group I consisted of 16 patients born from 1980–1989 and Group II consisted of 11 patients born before 1980. Using clinical characteristics from Group I, stepwise multiple regression analysis taking pubertal growth as an independent factor, and chronological age, bone age (TW2 RUS method standardized for Japanese children), height and height SDS as dependent factors revealed following formula (p<0.001, R2=0.737): (pubertal growth) = – 1.01x (Chronological age at start of E) – 0.326x (height at start of E) – 1.779x (bone age at start of E) + 90.997. Predicted adult height was obtained by adding predicted pubertal growth to height at the start of estrogen therapy. The mean absolute difference between real adult height (tallest height after height velocity less than 1 cm/yr) and predicted adult height was 1.6 ± 0.9 cm (0.3–2.8 cm) in Group I. When this prediction model was applied to Group II, The mean absolute difference between real adult height and predicted adult height was 1.0 ± 0.7 cm (0.1–2.0 cm). A prediction model for pubertal growth at start of estrogen therapy in Turner syndrome was obtained. Using this prediction model, the timing of the start of estrogen therapy can be decided in consideration of the patient’s desired adult height.

Tanaka, Toshiaki; Horikawa, Reiko; Naiki, Yasuhiro; Yokoya, Susumu; Satoh, Mari

2008-01-01

154

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. PMID:24124081

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

2013-11-01

155

[Therapy of the orthostatic syndrome. Studies using dimepropion-HCI].  

PubMed

We treated 30 patients between 22 to 30 years of age, who showed static labile circulatory disturbances of blood pressure. We carried out a double blind study following a two week schedule using for treatment an "indirect" sympathomimeticum Dimepropion-HCI. Control of therapy was achieved by a testing program which consisted in measuring systolic and diastolic blood pressure as well as the pulse rate. Also, we employed a flickers-fusion-test. Circulation parameters were measured in one-minute-intervals during three periods of 7 minutes each, patients changing from rest to stress (standing up) to rest again. The pulse rate was measured during the fourth minute of each of the three periods. Success of treatment was achieved by reducing the cardiovascular disturbances during upright conditions. The amplitude was broadened, while the pulse rate went up only slightly. The level of flicker fusion rose, indicating heightened physical and mental shape. Side effects of disturbed orthostasis disappeared. PMID:7042502

Soholing, W E

1982-02-18

156

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

PubMed Central

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

2014-01-01

157

Lentiviral hematopoietic stem cell gene therapy in patients with Wiskott-Aldrich syndrome.  

PubMed

Wiskott-Aldrich syndrome (WAS) is an inherited immunodeficiency caused by mutations in the gene encoding WASP, a protein regulating the cytoskeleton. Hematopoietic stem/progenitor cell (HSPC) transplants can be curative, but, when matched donors are unavailable, infusion of autologous HSPCs modified ex vivo by gene therapy is an alternative approach. We used a lentiviral vector encoding functional WASP to genetically correct HSPCs from three WAS patients and reinfused the cells after a reduced-intensity conditioning regimen. All three patients showed stable engraftment of WASP-expressing cells and improvements in platelet counts, immune functions, and clinical scores. Vector integration analyses revealed highly polyclonal and multilineage haematopoiesis resulting from the gene-corrected HSPCs. Lentiviral gene therapy did not induce selection of integrations near oncogenes, and no aberrant clonal expansion was observed after 20 to 32 months. Although extended clinical observation is required to establish long-term safety, lentiviral gene therapy represents a promising treatment for WAS. PMID:23845947

Aiuti, Alessandro; Biasco, Luca; Scaramuzza, Samantha; Ferrua, Francesca; Cicalese, Maria Pia; Baricordi, Cristina; Dionisio, Francesca; Calabria, Andrea; Giannelli, Stefania; Castiello, Maria Carmina; Bosticardo, Marita; Evangelio, Costanza; Assanelli, Andrea; Casiraghi, Miriam; Di Nunzio, Sara; Callegaro, Luciano; Benati, Claudia; Rizzardi, Paolo; Pellin, Danilo; Di Serio, Clelia; Schmidt, Manfred; Von Kalle, Christof; Gardner, Jason; Mehta, Nalini; Neduva, Victor; Dow, David J; Galy, Anne; Miniero, Roberto; Finocchi, Andrea; Metin, Ayse; Banerjee, Pinaki P; Orange, Jordan S; Galimberti, Stefania; Valsecchi, Maria Grazia; Biffi, Alessandra; Montini, Eugenio; Villa, Anna; Ciceri, Fabio; Roncarolo, Maria Grazia; Naldini, Luigi

2013-08-23

158

Statins in the first-line therapy of acute coronary syndrome - similar to aspirin?  

PubMed Central

Statins are cholesterol-lowering drugs, highly effective in the primary and secondary prevention of coronary artery disease. It has been found, however, that statins also have nonlipid effects; they can influence different pathways, which have been described to participate in the pathogenesis of acute coronary syndrome (ACS). Inflammation or decreased production of nitric oxide are obvious targets for statin therapy. Recently, several large clinical trials have been published, showing safety and, in some areas, efficacy of administration of statins early after ACS. Furthermore, there is growing evidence from both experimental and small clinical studies that statin therapy may have favourable effects when started as soon as possible after the development of ACS. Confirmation of this approach by large randomized trials is needed; however, based on currently available data, statins have high chance of achieving a similar place in the first-line therapy of ACS as the pillar of contemporary therapeutic strategy, aspirin.

Ostadal, Petr; Alan, David; Vejvoda, Jiri

2005-01-01

159

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

PubMed

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

Merkel, Drorit G; Nagler, Arnon

2014-07-01

160

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

PubMed Central

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).

2013-01-01

161

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

PubMed Central

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

Blum, William

2011-01-01

162

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

163

High-dose enzyme replacement therapy in murine Hurler syndrome.  

PubMed

Mucopolysaccharidosis type I (MPS I) is an autosomal recessive disease that is systemic, including progressive neurodegeneration, mental retardation and death before the age of 10 years. MPS I results from deficiency of ?-L-iduronidase (IDUA) in lysosomes and subsequent accumulation of glycosaminoglycans (GAG). Clinical enzyme replacement therapy (ERT) with intravenous laronidase reverses some aspects of MPS I disease (e.g., hepatomegaly, splenomegaly, glycosaminoglycanuria) and ameliorates others (e.g., pulmonary function, cardiac disease, arthropathy, exercise tolerance). However, neurologic benefits are thought to be negligible because the blood-brain barrier (BBB) blocks enzyme from reaching the central nervous system (CNS). We considered the possibility that a very high dose of intravenous laronidase might be able to traverse the BBB in small quantities, and provide some metabolic correction in the brain. To address this question, high-dose laronidase was administered (11.6 mg/kg, once per week, 4 weeks) to adult MPS I mice. IDUA enzyme activity in the cortex of treated mice increased to 97% of that in wild type mice (p<0.01). GAG levels in cortex were reduced by 63% of that from untreated MPS I mice (p<0.05). Further, immunohistochemical analysis showed that treatment reduced secondary GM3-ganglioside accumulation in treated MPS I mice. Water T-maze tests showed that the learning abnormality in MPS I mice was reduced (p<0.0001). In summary, repeated, high-dose ERT facilitated laronidase transit across the BBB, reduced GAG accumulation within the CNS, and rescued cognitive impairment. PMID:24100243

Ou, Li; Herzog, Tyler; Koniar, Brenda L; Gunther, Roland; Whitley, Chester B

2014-02-01

164

[A case in which chromosome 5q deletion syndrome resistant to lenalidomide therapy transformed to refractory anemia with excess blasts].  

PubMed

An 80-year-old man was admitted to our hospital because of pancytopenia. Bone marrow examination revealed an increase in the number of dysplastic cells indicating trilineage dysplasia. A 5q13q31 deletion was the only genetic abnormality found, and consequently, 5q deletion syndrome was diagnosed. Although lenalidomide therapy was initiated, it had to be discontinued because of Stevens-Johnson syndrome, which occurred during the second course of treatment. There was no discernible hematological improvement, and bone marrow aspiration showed transformation to refractory anemia with excess blasts-2(RAEB-2)after lenalidomide therapy. However, by changing the therapy to azacitidine, cytogenetic remission was achieved. PMID:24335378

Yamada, Michiko; Kuroda, Hiroyuki; Jomen, Wataru; Yoshida, Masahiro; Miura, Shogo; Abe, Tomoyuki; Sakurai, Tamaki; Fujii, Shigeyuki; Maeda, Masahiro; Fujita, Miri; Nagashima, Kazuo; Arihara, Yohei; Hirako, Tasuku; Kato, Junji

2013-12-01

165

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

PubMed

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

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

2014-06-01

166

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

167

Cardiorenal syndrome: ultrafiltration therapy for heart failure--trials and tribulations.  

PubMed

Heart failure remains the leading cause of hospitalization in older patients and is considered a growing public health problem with a significant financial burden on the health care system. The suboptimal efficacy and safety profile of diuretic-based therapeutic regimens coupled with unsatisfactory results of the studies on novel pharmacologic agents have positioned ultrafiltration on the forefront as an appealing therapeutic option for patients with acute decompensated heart failure (ADHF). In recent years, substantial interest in the use of ultrafiltration has been generated due to the advent of dedicated portable devices and promising results of trials focusing both on mechanistic and clinical aspects of this therapeutic modality. This article briefly reviews the proposed benefits of ultrafiltration therapy in the setting of ADHF and summarizes the major findings of the currently available studies in this field. The results of more recent trials on cardiorenal syndrome that present a counterpoint to previous observations and highlight certain limitations of ultrafiltration therapy are then discussed, followed by identification of major challenges and unanswered questions that could potentially hinder its more widespread use. Future studies are warranted to shed light on less well characterized aspects of ultrafiltration therapy and to further define its role in ADHF and cardiorenal syndrome. PMID:23723339

Kazory, Amir

2013-10-01

168

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

PubMed Central

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

Demirhan, Mehmet

2010-01-01

169

Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.  

PubMed

Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset. PMID:11300147

Tashima, K; Uyama, E; Hashimoto, Y; Yonehara, T; Uchino, M

2001-02-01

170

Hypoxaemic rescue therapies in acute respiratory distress syndrome: Why, when, what and which one?  

PubMed

Acute respiratory distress syndrome (ARDS) is an inflammatory condition of the lungs which can result in refractory and life-threatening hypoxaemic respiratory failure. The risk factors for the development of ARDS are many but include trauma, multiple blood transfusions, burns and major surgery, therefore this condition is not uncommon in the severely injured patient. When ARDS is severe, high-inspired oxygen concentrations are frequently required to minimise hypoxaemia. In these situations clinicians commonly utilise interventions termed 'hypoxaemic rescue therapies' in an attempt to improve oxygenation, as without these, conventional mechanical ventilation can be associated with high mortality. However, their lack of efficacy on mortality when used prophylactically in generalised ARDS cohorts has resulted in their use being confined to clinical trials and the subset of ARDS patients with refractory hypoxaemia. First line hypoxaemic rescue therapies include inhaled nitric oxide, prone positioning, alveolar recruitment manoeuvres and high frequency oscillatory ventilation, which have all been shown to be effective in improving oxygenation. In situations where these first line rescue therapies are inadequate extra-corporeal membrane oxygenation has emerged as a lifesaving second line rescue therapy. Rescue therapies in critically ill patients with traumatic injuries presents specific challenges and requires careful assessment of both the short and longer term benefits, therapeutic limitations, and specific adverse effects before their use. PMID:23261071

Hodgson, Carol; Carteaux, Guillaume; Tuxen, David V; Davies, Andrew R; Pellegrino, Vin; Capellier, Gilles; Cooper, David J; Nichol, Alistair

2013-12-01

171

Therapy related myelodysplastic syndrome: a case report and review of literature.  

PubMed

Therapy related myeloid neoplasm is directly related to previous cytotoxic chemotherapy or radiation therapy. We present a 47-year-old lady who developed therapy related myelodysplastic syndrome (MDS) 2.5 years after she received four cycles of chemotherapy and local radiation therapy for carcinoma breast. She presented with bicytopenia with trilineage dyspoiesis in the peripheral blood, bone marrow aspirate and biopsy. Fluorescent in-situ hybridization studies did not reveal any of the common abnormalities associated with MDS. A diagnosis of therapy related MDS was rendered. Different studies have shown that patients treated with alkylating agents and ionizing radiation present as MDS with a latent period of 3-10 years. Our patient developed MDS within 2.5 years of starting chemotherapy and radiotherapy and did not reveal any of the conventional cytogenetic abnormalities. It highlights the importance of simple tests like a complete blood count and peripheral blood smear examination in follow-up of the patients treated with chemotherapy. PMID:21623094

Sonawane, Smita; Gadgil, Nitin; Margam, Sangita

2011-01-01

172

Treatment for Churg-Strauss syndrome: induction of remission and efficacy of intravenous immunoglobulin therapy.  

PubMed

Churg-Strauss syndrome (CSS) is characterized by the presence of asthma, eosinophilia, and small-vessel vasculitis with granuloma. It is a distinct entity, as determined from all classifications of systemic vasculitis. The poor prognostic factors in CSS are renal insufficiency, cardiomyopathy, severe gastrointestinal (GI) tract, and central nervous systems (CNS) involvement. The initial management of CSS should include a high dose of a corticosteroid: prednisone at 1 mg/kg/day or its equivalent for methylprednisolone with tapering over 6 months. In patients with severe or rapidly progressing CSS, the administration of methylprednisolone pulse at 1 g/body/day for 3 days is recommended. When corticosteroid therapy does not induce remission, or when patients have poor prognostic factors, immunosuppressive cytotoxic therapy is indicated. However, some patients with severe CSS often show resistance to conventional treatment. We think that IVIG therapy is a hopeful candidate for second-line treatment for CSS patients, particularly in the case of neuropathy and/or cardiomyopathy, which are resistant to conventional therapy. However, there is not much evidence supporting the effectiveness of IVIG in CSS, and the mechanisms underlying the action of IVIG remain unclear. Now we are performing clinical trials of IVIG therapy for CSS patients who are resistant to conventional treatment, through a nationwide double-blinded placebo-controlled study in Japan. PMID:17460439

Taniguchi, Masami; Tsurikisawa, Naomi; Higashi, Noritaka; Saito, Hiroshi; Mita, Haruhisa; Mori, Akio; Sakakibara, Hiroki; Akiyama, Kazuo

2007-06-01

173

The locked-in syndrome: a syndrome looking for a therapy.  

PubMed

The locked-in syndrome (LIS) is a very severe condition caused by a primary vascular or traumatic injury to the brainstem, normally corresponding to a ventral pons lesion due to an obstruction of the basilar artery, and characterized by upper motor neuron quadriplegia, paralysis of lower cranial nerves, bilateral paresis of horizontal gaze and anarthria, and with preserved consciousness. Patients who have suffered this pontine lesion generally have preserved vertical eye movements and movement of the eyelids (blinking), this being their only means of responding to the outside world. A survey was conducted of 44 people diagnosed with LIS, all of them belonging to the Association of Locked-in Syndrome (ALIS) of France. Results of this survey showed that LIS was equally frequent in men and women (51.2% vs. 48.1%) and had occurred at any age between 22-77 years of age (normally between 41-52 years, the mean age being 46.79 years). The average time that transpired post-insult was 71.35 months. The principal cause of LIS was stroke (86.4%), with traumatic brain injury (TBI) being a distant second cause with an incidence of only 13.6%. The diagnosis of LIS was usually made around the middle of the second month after onset (mean of 78.76 days). The principal treatments, when present, were pharmacological and physiotherapy. However, 47.1% of the patients were not receiving treatment of any kind at the time of the survey. Neuropsychologically, 86% had a good attentional level, 97.6% were temporally oriented and 76.7% could read; 18.6% reported memory problems and 24% showed visual deficit (found mainly in patients with LIS originated by TBI); 47.5% reported a good mood state and 12.5% reported feeling depressed; 61.1% reported having sexual desire, but only 30% maintained sexual relations; 78% were capable of emitting sounds and 65.8% could communicate without technical aid; 73.2% enjoyed going out and 81% met with friends at least twice a month. Only 14.3% participated in social activities and 23.8% watched television regularly. Nearly 100% of the patients reported being sensitive to touch to any part of their bodies. This survey suggests diagnostics and rehabilitation procedures. PMID:12119076

León-Carrión, José; van Eeckhout, Philippe; Domínguez-Morales, María Del Rosario; Pérez-Santamaría, Francisco Javier

2002-07-01

174

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

175

[Preventive and cessation therapy of mental disorders in patients with the acute coronary syndrome].  

PubMed

An open prospective trial of 93 inpatients divided into two main groups (61 patients) - with mental disorders (31) and without mental disorders (30) and a comparison group (32 patients) was carried out. All patients survived the acute coronary syndrome (myocardial infarction, unstable angina). The efficacy and safety of both preventive and cessation therapy with pantogam active in mean doses 1.8 and 1.2 g /daily, respectively, was demonstrated in respect of heterogeneous depressive, anxiety and somatoform disorders of the neurotic level. PMID:22810740

Medvedev, V E; Epifanov, A V; Zverev, K V

2012-01-01

176

DRAVET SYNDROME Insights into pathophysiology and therapy from a mouse model of Dravet syndrome  

PubMed Central

Summary Mutations in voltage-gated sodium channels are associated with epilepsy syndromes with a wide range of severity. Complete loss of function in the Nav1.1 channel encoded by the SCN1A gene is associated with severe myoclonic epilepsy in infancy (SMEI), a devastating infantile-onset epilepsy with ataxia, cognitive dysfunction, and febrile and afebrile seizures resistant to current medications. Genetic mouse models of SMEI have been created that strikingly recapitulate the SMEI phenotype including age and temperature dependence of seizures and ataxia. Loss-of-function in Nav1.1 channels results in severely impaired sodium current and action potential firing in hippocampal ?-aminobutyric acid (GABA)ergic interneurons without detectable changes in excitatory pyramidal neurons. The resulting imbalance between excitation and inhibition likely contributes to hyperexcitability and seizures. Reduced sodium current and action potential firing in cerebellar Purkinje neurons likely contributes to comorbid ataxia. A mechanistic understanding of hyperexcitability, seizures, and comorbidities such as ataxia has led to novel strategies for treatment.

Oakley, John C.; Kalume, Franck; Catterall, William A.

2012-01-01

177

[ANMCO/SICI-GISE document on antiplatelet therapy in patients with acute coronary syndrome].  

PubMed

Antiplatelet therapy is the cornerstone of the pharmacologic management of patients with acute coronary syndrome (ACS). Over the last years, several studies have evaluated old and new oral or intravenous antiplatelet agents in ACS patients. In particular, research was focused on assessing superiority of two novel platelet ADP P2Y12 receptor antagonists (i.e., prasugrel and ticagrelor) over clopidogrel. Several large randomized controlled trials have been undertaken in this setting and a wide variety of prespecified and post-hoc analyses are available that evaluated the potential benefits of novel antiplatelet therapies in different subsets of patients with ACS. The aim of this document is to review recent data on the use of current antiplatelet agents for in-hospital treatment of ACS patients. For each drug or class of drugs, strong evidence and/or areas of uncertainty that warrant further research are highlighted by examining 10 subgroups of patients with ACS. PMID:24336601

De Luca, Leonardo; Bolognese, Leonardo; Valgimigli, Marco; Ceravolo, Roberto; Danzi, Gian Battista; Piccaluga, Emanuela; Rakar, Serena; Cremonesi, Alberto; Bovenzi, Francesco Maria

2013-12-01

178

Intrathecal baclofen therapy after liver transplant in a patient with Crigler-Najjar syndrome.  

PubMed

Crigler-Najjar syndrome (CNS) is described as severe infantile, nonhemolytic, unconjugated hyperbilirubinemia and is divided into type I and type II according to the patient's response to phenobarbital treatment. Patients with type I CNS usually require a liver transplant. These patients often have spasticity and dystonia, both of which can be treated with intrathecal baclofen therapy. We present the case of a patient with CNS type I who underwent a liver transplant followed years later by intrathecal baclofen therapy. To our knowledge, this article provides the first report of a patient with CNS being treated for dystonia with an intrathecal baclofen pump. Despite his complicated history, this patient has remained medically stable after both interventions. PMID:24365780

Pratini, Napala R; Neufeld, Jacob A

2014-02-01

179

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

PubMed

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

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

2014-12-01

180

Hyperglycemic hyperosmolar syndrome caused by steroid therapy in a patient with lupus nephritis.  

PubMed

A 51-yr-old female was referred to our outpatient clinic for the evaluation of generalized edema. She had been diagnosed with idiopathic thrombocytopenic purpura (ITP). She had taken no medicine. Except for the ITP, she had no history of systemic disease. She was diagnosed with systemic lupus erythematosus. Immunosuppressions consisting of high-dose steroid were started. When preparing the patient for discharge, a generalized myoclonic seizure occurred at the 47th day of admission. At that time, the laboratory and neurology studies showed hyperglycemic hyperosmolar syndrome. Brain MRI and EEG showed brain atrophy without other lesion. The seizure stopped after the blood sugar and serum osmolarity declined below the upper normal limit. The patient became asymptomatic and she was discharged 10 weeks after admission under maintenance therapy with prednisolone, insulin glargine and nateglinide. The patient remained asymptomatic under maintenance therapy with deflazacort and without insulin or medication for blood sugar control. PMID:21394317

Kang, Seok-Hui; Lee, Ja-Young; Park, Hoon-Suk; Sun, In-O; Choi, Sun-Ryoung; Chung, Byung-Ha; Choi, Bum-Soon; Yang, Chul-Woo; Kim, Yong-Soo; Park, Cheol-Whee

2011-03-01

181

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

182

Anti-thymocyte Globulin plus Etanercept as Therapy for Myelodysplastic Syndromes (MDS): a Phase II Study  

PubMed Central

Summary Immunosuppressive therapies have proven valuable in treating patients with myelodysplastic syndromes (MDS). We evaluated the combination of equine anti-thymocyte globulin (ATGAM®) and the soluble TNF receptor etanercept (Enbrel®) in a phase II trial. Twenty-five patients with MDS (4-RA, 2-RARS, 15-RCMD, 3-RCMD-RS, 1-RAEB-1) in IPSS risk groups low (n=11) or intermediate-1 (n=14) were enrolled. All patients were platelet or red cell transfusion dependent. Nineteen patients completed therapy with ATG at 40 mg/kg/day for four consecutive days, followed by etanercept, 25 mg subcutaneous twice a week for 2 weeks, every month for 4 months. Thirteen patients had hematological improvement (HI)-erythroid, 2 HI-neutrophil, and 6 HI-platelet. One patient with a co-existing diagnosis of multiple sclerosis and rheumatoid arthritis had a complete remission. The overall response by intent to treat analysis among the 25 patients was 56% (95% CI 35–56%). Four patients did not complete their first course of therapy and one patient did not survive to the 8-week post-treatment assessment. Among patients who completed treatment and survived to the 8-week assessment, 70% had at least hematological responses lasting for at least 5 to more than 36 months. Thus, combination therapy with ATG and etanercept was active and safe in patients with MDS.

Scott, Bart L.; Ramakrishnan, Aravind; Fosdal, Mark; Storer, Barry; Becker, Pamela; Petersdorf, Steve; Deeg, H. Joachim

2010-01-01

183

Refining the role of antiplatelet therapy in medically managed patients with acute coronary syndrome.  

PubMed

Dual-antiplatelet therapy with aspirin plus a P2Y(12) receptor inhibitor is recommended for use as first-line therapy in patients with acute coronary syndromes (ACS) who undergo high-risk percutaneous coronary intervention. However, revascularization may not be a beneficial option for some subgroups of patients with ACS. This includes a broad spectrum of lower risk patients as well as high-risk patients with numerous previous revascularizations and those who are at high risk for complications, such as those with complex coronary anatomy and co-morbidities such as diabetes mellitus, chronic kidney disease, or advanced age and frailty. For such patients, there remains an unmet need for evaluation of alternatives to the currently recommended treatment options. Notably, there is a paucity of prospective data regarding management approaches to medically managed patients with ACS. Thus, this group of medically managed patients with ACS would benefit from inclusion in clinical trials investigating therapeutic options for patients not scheduled to undergo invasive procedures, such as those who are targeted for pharmacologic management only. In conclusion, in this review, the investigators revisit data from clinical studies of dual-antiplatelet therapy in ACS to highlight areas of unmet need in antiplatelet therapy in patients with ACS and to examine the use of newer agents in subgroups, such as medically managed patients with ACS, that would potentially benefit from more potent platelet inhibition after ACS. PMID:23168289

Boden, William E; Lansky, Alexandra; Angiolillo, Dominick J

2013-02-01

184

Cognitive behaviour therapy in addition to antispasmodic treatment for irritable bowel syndrome in primary care: randomised controlled trial  

PubMed Central

Objective To assess the efficacy of cognitive behaviour therapy delivered in primary care for treating irritable bowel syndrome. Design Randomised controlled trial. Setting 10 general practices in London. Participants 149 patients with moderate or severe irritable bowel syndrome resistant to the antispasmodic mebeverine. Interventions Cognitive behaviour therapy delivered by trained primary care nurses plus 270 mg mebeverine taken thrice daily compared with mebeverine treatment alone. Main outcome measures Primary measures were patients' scores on the irritable bowel syndrome symptom severity scale. Secondary measures were scores on the work and social adjustment scale and the hospital anxiety and depression scale. Results Of 334 referred patients, 72 were randomised to mebeverine plus cognitive behaviour therapy and 77 to mebeverine alone. Cognitive behaviour therapy had considerable initial benefit on symptom severity compared with mebeverine alone, with a mean reduction in score of 68 points (95% confidence interval 103 to 33), with the benefit persisting at three months and six months after therapy (mean reductions 71 points (109 to 32) and 11 points (20 to 3)) but not later. Cognitive behaviour therapy also showed significant benefit on the work and social adjustment scale that was still present 12 months after therapy (mean reduction 2.8 points (5.2 to 0.4)), but had an inconsistent effect on the hospital anxiety and depression scale. Conclusion Cognitive behaviour therapy delivered by primary care nurses offered additional benefit over mebeverine alone up to six months, although the effect had waned by 12 months. Such therapy may be useful for certain patients with irritable bowel syndrome in primary care.

Kennedy, Tom; Jones, Roger; Darnley, Simon; Seed, Paul; Wessely, Simon; Chalder, Trudie

2005-01-01

185

Successful revascularization to right coronary artery by percutaneous coronary intervention after endovascular therapy for leriche syndrome.  

PubMed

A 69-year-old man with effort angina was admitted to our institution. Echocardiography showed poor left ventricular systolic function with akinesis of the anterior wall and severe hypokinesis of the inferior wall. We performed coronary angiography, which revealed two diseased vessels including chronic total occlusion in the left anterior descending artery and severe stenosis in the right coronary artery (RCA). In addition, aortography revealed aortoiliac occlusive disease known as Leriche syndrome. As the patient's symptom was stable, we first planned to perform endovascular therapy (EVT) for Leriche syndrome to make a route for intra-aortic balloon pumping. We prepared a bi-directional approach from bi-femoral arteries and a left brachial artery. The guidewire was passed through the occlusive area using the retrograde approach. The self-expanding stents were deployed by a kissing technique. At one week after EVT, a 6Fr sheath was inserted from the right radial artery and an intra-aortic balloon pump was successfully inserted through the right femoral artery for percutaneous coronary intervention (PCI) to the RCA. Two drug-eluting stents were successfully deployed to RCA after using an atherectomy device (rotablator). We reported the case as a successfully performed PCI to the RCA after EVT for Leriche syndrome. PMID:24678244

Niizeki, Takeshi; Kaneko, Kazuyoshi; Sugawara, Shigeo; Sasaki, Toshiki; Tsunoda, Yuichi; Takeishi, Yasuchika; Kubota, Isao

2014-01-01

186

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

2013-01-01

187

Successful Revascularization to Right Coronary Artery by Percutaneous Coronary Intervention after Endovascular Therapy for Leriche Syndrome  

PubMed Central

A 69-year-old man with effort angina was admitted to our institution. Echocardiography showed poor left ventricular systolic function with akinesis of the anterior wall and severe hypokinesis of the inferior wall. We performed coronary angiography, which revealed two diseased vessels including chronic total occlusion in the left anterior descending artery and severe stenosis in the right coronary artery (RCA). In addition, aortography revealed aortoiliac occlusive disease known as Leriche syndrome. As the patient’s symptom was stable, we first planned to perform endovascular therapy (EVT) for Leriche syndrome to make a route for intra-aortic balloon pumping. We prepared a bi-directional approach from bi-femoral arteries and a left brachial artery. The guidewire was passed through the occlusive area using the retrograde approach. The self-expanding stents were deployed by a kissing technique. At one week after EVT, a 6Fr sheath was inserted from the right radial artery and an intra-aortic balloon pump was successfully inserted through the right femoral artery for percutaneous coronary intervention (PCI) to the RCA. Two drug-eluting stents were successfully deployed to RCA after using an atherectomy device (rotablator). We reported the case as a successfully performed PCI to the RCA after EVT for Leriche syndrome.

Niizeki, Takeshi; Kaneko, Kazuyoshi; Sugawara, Shigeo; Sasaki, Toshiki; Tsunoda, Yuichi; Takeishi, Yasuchika; Kubota, Isao

2014-01-01

188

Statin Therapy in Metabolic Syndrome and Hypertension Post-JUPITER: What is the Value of CRP?  

PubMed Central

Much evidence supports a pivotal role for inflammation in atherosclerosis. C-reactive protein (CRP), the prototypic marker of inflammation in humans, is a cardiovascular risk marker and may also promote atherogenesis. CRP levels are increased in metabolic syndrome and hypertension and confer increased risk of cardiovascular events in patients in these subgroups. Statins have been shown to lower low-density lipoproteins and CRP independently, and reduce cardiovascular events in subjects with and without metabolic syndrome and hypertension. In this review, we focus on the results from the primary prevention statin trial, Justification for the Use of statins in Primary prevention: an Intervention Trial Evaluating Rosuvastatin (JUPITER), which showed reductions in LDL, CRP, and cardiovascular events. Post-JUPITER, the new guidelines will now need to consider recommending high-sensitivity CRP testing to intermediate-risk metabolic syndrome patients and those with hypertension and intermediate risk so that we can better identify candidates at greater risk and reduce cardiovascular burden in these subjects with statin therapy.

Devaraj, Sridevi; Siegel, David

2010-01-01

189

[Devic syndrome--case report, current principles of diagnosis and therapy].  

PubMed

Neuromyelitis optica (NMO, Devic-syndrome) is a rare, relapsing autoimmune disease of the central nervous system, which is distinguished from other demyelinating disorders by a recently identified, specific autoantibody. By demonstrating the anti-aquaporin-4 IgG in the serum, a heterogenous group of syndromes can be defined, called NMO-spectrum. In the future, optical coherence tomography may support this diagnosis besides the clinical features, imaging examinations and presence of serum antibody. Early recognition and treatment can improve clinical outcome even in serious condition. Long-term immunosuppressive therapy is advised to prevent further relapses and to stabilize or improve clinical status. Hereby, we report a case of a 51-year-old woman, under treatment for one and a half years. We summarize the current knowledge about the pathomechanism, diagnostic strategy and therapy of neuromyelitis optica. We review recent findings and the diagnostic value of a new, non-invasive ophtalmological examination, the optical coherence tomography. According to the first results, this method may be helpful in the early differential diagnosis of optic neuritis. PMID:21033421

Iljicsov, Anna; Barsi, Péter; Várallyay, György; Tátrai, Erika; Somfai, Gábor Márk; Bereczki, Dánieli; Rudas, Gábor; Simó, Magdolna

2010-09-30

190

Comparision of Ultrasound Therapy of Various Durations in the Treatment of Subacromial Impingement Syndrome  

PubMed Central

[Purpose] A prospective, randomized, single-blind study was performed in order to compare the efficacy of ultrasound treatments of various durations for patients with subacromial impingement syndrome. [Subjects and Methods] One hundred patients who had been diagnosed with subacromial impingement syndrome by clinical examination and magnetic resonance imaging were included in this study. Patients were randomly divided into two groups of fifty patients per group. The first group received 15 sessions of therapeutic ultrasound (4 minutes), superficial heat and transcutaneous electrical stimulation therapy combined with exercise. The second group received the same treatment except that each of the 15 ultrasound sessions were eight minutes in length. The patients were evaluated before and after the treatment. A visual analog scale (VAS) was used to assess pain, the University of California at Los Angeles (UCLA) and Constant Scale were used to assess shoulder function and the Beck Depression Inventory (BDI) was used to quantify depressive symptoms. [Results] There were no statistically significant differences between the groups in age, time since the onset of pain, sex, education and depression levels prior to the treatment. The post-treatment evaluation of patients VAS, UCLA, Constant, and BDI scores showed statistically significant within group improvements. When the two groups were compared, we found no statistically significant differences in the Constant activities of daily living, Constant external rotation, Constant force and BDI scores. However, the second group scored better than the first group in all the remaining parameters. [Conclusion] Ultrasound therapy was found to have beneficial effects on pain and functional status in the treatment of subacromial impingement syndrome. Eight minutes of ultrasound treatment was shown to be more effective than 4 minutes of ultrasound treatment.

Yildirim, Mustafa Aziz; Ones, Kadriye; Celik, Evrim Coskun

2013-01-01

191

Chinese herbal extracts (SK0506) as a potential candidate for the therapy of the metabolic syndrome.  

PubMed

The metabolic syndrome has reached epidemic proportions worldwide, but currently there is a lack of effective therapies for this multifactorial endocrine disease. TCM (traditional Chinese medicine) has been utilized to treat a wide variety of diseases for centuries in the People's Republic of China, subsequently becoming a promising source for the development of new therapeutic agents. Chinese medicinal herbs Gynostemma pentaphyllum, Coptis chinensis and Salvia miltiorrhiza have been shown to have anti-atherosclerotic and antidiabetic properties. In this study, we have investigated the metabolic effects of a mixture of these three herbal extracts (SK0506) in a rodent model of the metabolic syndrome induced by an HFD (high-fat diet). SD (Sprague-Dawley) rats that were fed on an HFD for 4 weeks gained 33% more weight compared with chow-fed rats (P<0.05). Four weeks treatment with SK0506 prevented weight gain with decreased visceral fat (P<0.01 compared with vehicle treatment). SK0506 also significantly reduced plasma triacylglycerols (triglycerides), NEFAs (non-esterified fatty acids) and cholesterol. SK0506 exerted similar effects to RSG (rosiglitazone) on impaired glucose intolerance. SK0506 also significantly enhanced glucose uptake and glycogen synthesis in adipose tissue during hyperinsulinaemic-euglycaemic clamp. Western blotting analysis revealed that SK0506 enhanced GLUT4 (glucose transporter 4) expression in adipose tissue, and RSG markedly up-regulated GLUT4 translocation in skeletal muscle. Overall, the present study has discovered that SK0506 can reverse several components of the metabolic syndrome primarily through acting on hyperlipidaemia and visceral obesity. The results from the present study suggest that it is worthwhile to conduct a randomized clinical trial to confirm the potential that SK0506 may be a new oral agent for treating the metabolic syndrome and preventing Type 2 diabetes. PMID:20950275

Tan, Yi; Kamal, Mohammad A; Wang, Zheng-Zhong; Xiao, Wei; Seale, John P; Qu, Xianqin

2011-04-01

192

Comparison between single antiplatelet therapy and combination of antiplatelet and anticoagulation therapy for secondary prevention in ischemic stroke patients with antiphospholipid syndrome  

PubMed Central

Satisfactory results have not yet been obtained in therapy for secondary prevention in ischemic stroke patients with antiphospholipid syndrome (APS). We therefore compared single antiplatelet therapy and a combination of antiplatelet and anticoagulation therapy for secondary prevention in ischemic stroke patients with APS. The subjects were 20 ischemic stroke patients with antiphospholipid antibody, 13 with primary antiphospholipid syndrome and 7 with SLE-related antiphospholipid syndrome. Diagnosis of APS was based on the 2006 Sydney criteria. Eligible patients were randomly assigned to either single antiplatelet therapy (aspirin 100 mg) or a combination of antiplatelet and anticoagulation therapy (target INR: 2.0-3.0; mean 2.4±0.3) for the secondary prevention of stroke according to a double-blind protocol. There was no significant difference between the two groups in age, gender, NIH Stroke Scale on admission, mRS at discharge, or rate of hypertension, diabetes mellitus, hyperlipidemia, or cardiac disease. We obtained Kaplan-Meier survival curves for each treatment. The primary outcome was the occurrence of stroke. The mean follow-up time was 3.9±2.0 years. The cumulative incidence of stroke in patients with single antiplatelet treatment was statistically significantly higher than that in patients receiving the combination of antiplatelet and anticoagulation therapy (log-rank test, p-value=0.026). The incidence of hemorrhagic complications was similar in the two groups. The recent APASS study did not show any difference in effectiveness for secondary prevention between single antiplatelet (aspirin) and single anticoagulant (warfarin) therapy. Our results indicate that combination therapy may be more effective in APS-related ischemic stroke.

Okuma, Hirohisa; Kitagawa, Yasuhisa; Yasuda, Takashi; Tokuoka, Kentaro; Takagi, Shigeharu

2010-01-01

193

Systemic radionuclide therapy using indium-111-DTPA-D-Phe1-octreotide in midgut carcinoid syndrome.  

PubMed

A 55-yr-old woman with a midgut carcinoid syndrome due to metastatic spread of an ileal tumor to the liver, paraortic and mediastinal lymph nodes and to the skeleton was given systemic radionuclide therapy with 111In-DTPA-D-Phe1-octreotide. Before therapy, dosimetric calculations were performed on whole-body scintigraphs and 111In retention was shown to be long-lasting. Excretion was mainly seen during the first 24 hr after injection; thereafter whole-body retention remained stationary at 30%. Indium-111 activity in tumor biopsies and blood was measured using a gamma counter. Very high tumor-to-blood ratios were obtained: 150 for the primary tumor and 400-650 for liver metastases, which further justified radiation therapy. Indium-111-DTPA-D-Phe1-octreotide treatment was given on three separate occasions (3.0, 3.5 and 3.1 GBq) 8 and 4 wk apart. After each therapy, the patient experienced facial flush and pain over the skeletal lesions followed by symptomatic relief, even though no objective tumor regression was found radiologically after 5 mo. After initiation of octreotide treatment, there was a 14% reduction of the main tumor marker, urinary 5-HIAA. After three subsequent radionuclide therapies, there was a further 31% reduction of 5-HIAA levels. No adverse reactions, other than a slight decrease in leukocyte counts, were seen. The mean absorbed radiation dose after the three treatments was estimated to be about 10-12 Gy in liver metastases and 3-6 Gy in other tumors, depending on the size and location of the metastases. Assuming internalization of 111In into tumor cells and a radiobiological effect from short range Auger and conversion electrons, there might be a therapeutic effect on the tumor. PMID:8790206

Fjälling, M; Andersson, P; Forssell-Aronsson, E; Grétarsdóttir, J; Johansson, V; Tisell, L E; Wängberg, B; Nilsson, O; Berg, G; Michanek, A; Lindstedt, G; Ahlman, H

1996-09-01

194

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

2013-05-01

195

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

PubMed

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

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

2014-01-01

196

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

PubMed Central

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

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

2013-01-01

197

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

198

A Controlled Comparison of Cognitive Therapy and Self-Help Support Groups in the Treatment of Irritable Bowel Syndrome.  

ERIC Educational Resources Information Center

Patients with irritable bowel syndrome (n=34) were randomly assigned to 1 of 3 treatment conditions for 8 weeks: individualized cognitive treatment, support group, or control. Results indicated significantly greater reductions in gastrointestinal symptoms and amelioration of depression and anxiety for the cognitive therapy group, and these results…

Payne, Annette; Blanchard, Edward B.

1995-01-01

199

Unmasking cryptococcal meningitis immune reconstitution inflammatory syndrome in pregnancy induced by HIV antiretroviral therapy with postpartum paradoxical exacerbation  

PubMed Central

Cryptococcosis is the most common cause of meningitis in Africa due to the high burden of HIV. Immune reconstitution inflammatory syndrome (IRIS) is a frequent and deadly complication of cryptococcal meningitis. We report a fatal case of cryptococcal-IRIS in a pregnant woman that began after starting antiretroviral therapy (unmasking IRIS) and markedly worsened postpartum after delivery (paradoxical IRIS).

Kiggundu, Reuben; Rhein, Joshua; Meya, David B.; Boulware, David R.; Bahr, Nathan C.

2014-01-01

200

Unmasking cryptococcal meningitis immune reconstitution inflammatory syndrome in pregnancy induced by HIV antiretroviral therapy with postpartum paradoxical exacerbation.  

PubMed

Cryptococcosis is the most common cause of meningitis in Africa due to the high burden of HIV. Immune reconstitution inflammatory syndrome (IRIS) is a frequent and deadly complication of cryptococcal meningitis. We report a fatal case of cryptococcal-IRIS in a pregnant woman that began after starting antiretroviral therapy (unmasking IRIS) and markedly worsened postpartum after delivery (paradoxical IRIS). PMID:24944885

Kiggundu, Reuben; Rhein, Joshua; Meya, David B; Boulware, David R; Bahr, Nathan C

2014-07-01

201

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

202

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

PubMed Central

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

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

2013-01-01

203

Physicians compliance during maintenance therapy in children with Down syndrome and acute lymphoblastic leukemia.  

PubMed

Children with Down syndrome (DS) and acute lymphoblastic leukemia (ALL) have an inferior prognosis compared with non-DS ALL patients. We reviewed methotrexate (MTX)/mercaptopurine (6MP) maintenance therapy data for children with DS treated according to the Nordic Society of Pediatric Hematology and Oncology (NOPHO) ALL92 or the NOPHO ALL2000 protocols between 1992 and 2007. The 5-year event-free survival probability (pEFS(5?yr)) for the 66 DS patients was inferior to the 2602 non-DS patients (0.50 ± 0.07 vs 0.77 ± 0.01 (P<0.001)). The 48 DS patients in first remission at the beginning of maintenance therapy had pEFS(10?yr) below that of the 522 non-DS control patients (pEFS(10?yr): 0.58 (95% confidence interval (CI) 0.43-0.77) vs 0.83 (95% CI 0.80-0.86), respectively (P<0.0001)). The DS patients received lower median doses of MTX (median: 11.8 vs 15.4 (P<0.0001)) and 6MP (median: 43.6 vs 59.4 (P<0.0001)). In Cox regression analysis, male gender, presence of DS and high median maintenance therapy white blood cell levels (mWBC) were associated with increased risk for relapse. DS-ALL patients with mWBC above or below 3.5 × 10(9)/l (protocol target) had pEFS(10?yr) of 0.31 and 0.72 (P=0.02), and the mWBC hazard ratio for DS-ALL patients was 2.0 (P<0.0005). We conclude that insufficient treatment intensity during maintenance therapy of DS-ALL patients may contribute to their poor prognosis. PMID:23138181

Bohnstedt, C; Levinsen, M; Rosthøj, S; Zeller, B; Taskinen, M; Hafsteinsdottir, S; Björgvinsdóttir, H; Heyman, M; Schmiegelow, K

2013-04-01

204

Photodynamic Therapy for Diffuse Choroidal Hemangioma in Sturge-Weber Syndrome  

PubMed Central

Purpose. To report the treatment outcome of photodynamic therapy with verteporfin (PDT) for exudative retinal detachment (RD) associated with diffuse choroidal hemangioma in Sturge-Weber syndrome (SWS). Methods. An interventional case report of a 10-year-old girl with SWS who developed an exudative RD (visual acuity hand motions) that was treated with PDT. She was treated with a first session of multispot PDT. Posteriorly, a choroidotomy for drainage of subretinal fluid was created, combined with an intravitreal injection of gas (SF6) and cryoapplication. Finally, a second session of PDT was applied. Results. Subretinal fluid resolved over a period of one year and visual acuity increased to 20/125. Conclusions. PDT is an effective therapeutic option for exudative RD associated with diffuse choroidal hemangioma.

Monteiro, Silvia; Casal, Ines; Santos, Marinho

2014-01-01

205

Neurologic improvement without angiographic improvement after antithyroid therapy in a patient with Moyamoya syndrome.  

PubMed

Moyamoya disease with special complications, including Graves' disease, is called as moyamoya syndrome. A 22-year-old Japanese woman had left middle cerebral artery (MCA) territory infarction complicated with Graves' disease. She had right-sided hemiparesis that deteriorated on day 8 with the infarct growth and thyrotoxicosis. On angiogram, the left MCA was occluded at the origin without moyamoya vessels. Positron emission tomography (PET) revealed misery-perfusion phenomenon in the left MCA territory. After initiation of the antithyroid therapy, her hemiparesis became milder. Seventeen months later, her thyroid function was normalized and net-like collateral moyamoya vessels proliferated in the left MCA territory. Misery-perfusion phenomenon persisted on PET. This report is unique in the point of neurologic recovery of the moyamoya patient right after initiation of antithyroid medication without radiological improvement. PMID:24119625

Ishigami, Akiko; Toyoda, Kazunori; Suzuki, Rieko; Miyashita, Fumio; Iihara, Koji; Minematsu, Kazuo

2014-01-01

206

Anti-inflammatory therapy with a COX-2 inhibitor in Tourette's syndrome.  

PubMed

An infectious/inflammatory process plays a role in at least a subgroup of patients with tics and Tourette's syndrome (TS). Successful antibiotic therapy and prophylaxis was described repeatedly. We report the case of a patient suffering from chronic TS who was treated with celecoxib additionally to the antibiotic prophylaxis. This treatment was associated with a continuous improvement of tics and disturbed behaviour, such as aggression and social withdrawal. The withdrawal of celecoxib led to a marked deterioration in TS symptoms while the re-exposition had advantageous therapeutic effects. This result of the treatment with a COX-2 inhibitor supports the view that COX-2 inhibitors show therapeutic benefit in patients suffering from psychiatric disorders in which an inflammatory process is involved in the pathophysiology. PMID:15527551

Müller, Norbert

2004-01-01

207

Possible induction of West syndrome by oxcarbazepine therapy in a patient with complex partial seizures.  

PubMed

Oxcarbazepine has been reported to precipitate myoclonic, generalised tonic-clonic, absence, and complex partial seizures, and carbamazepine to precipitate absences, myoclonic seizures and spasms. Here, we report a one-year, six-month-old girl with complex partial seizures who developed infantile spasms, developmental regression, and hypsarrhythmia during the two weeks directly following initiation of oxcarbazepine (14?mg/kg/day). All of these resolved within a few days after discontinuation of this medication. Although we cannot rule out that the above association may have been coincidental, or that the improvement may have been due to concurrent therapy, this case raises the possibility that oxcarbazepine, like carbamazepine, may precipitate infantile spasms and West syndrome. PMID:22425652

Veerapandiyan, Aravindhan; Singh, Piyush; Mikati, Mohamad A

2012-03-01

208

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

PubMed Central

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

Williams, David S.

2009-01-01

209

Cost-Effectiveness of Latent Tuberculosis Screening Before Steroid Therapy for Idiopathic Nephrotic Syndrome in Children  

PubMed Central

Background Guidelines differ on screening recommendations for latent tuberculosis infection (LTBI) prior to immunosuppressive therapy. We aimed to determine the most cost-effective LTBI screening strategy before long-term steroid therapy in a child with new onset idiopathic nephrotic syndrome. Study Design Markov state-transition model. Setting and Population Five year old male with new onset idiopathic nephrotic syndrome. Model, Perspective, and Timeframe The Markov model took a societal perspective over a lifetime horizon. Intervention Three strategies were compared: universal tuberculin skin testing (TST), targeted screening using a risk-factor questionnaire, and no screening. A secondary model included the newer interferon-? release assays (IGRA), requiring only one visit and having greater specificity than TST. Outcomes Marginal cost-effectiveness ratios (2010 United States $) with effectiveness measured as quality-adjusted life years (QALYs). Results At a LTBI prevalence of 1.1% (the average United States childhood prevalence in our base-case), a no-screening strategy dominated ($2,201, 29.3356 QALYs) targeted screening ($2,218, 29.3356 QALYs) and universal TST ($2,481, 29.3347 QALYs). At a prevalence >10.3%, targeted screening with a risk-factor questionnaire was the most cost-effective option. Above a prevalence of 58.5%, universal TST was preferred. In the secondary model, targeted screening with a questionnaire followed by IGRA testing was cost-effective compared to no screening in the base case when the LTBI prevalence was >4.9%. Limitations There is no established gold standard for the diagnosis of LTBI. Results of any modeling task are limited by the accuracy of available data. Conclusions Prior to starting steroids, only patients in areas with a high prevalence of LTBI will benefit from universal TST. As more evidence becomes available on the use of IGRA testing in children, the assay may become a component of cost-effective screening protocols in populations with a higher burden of LTBI.

Laskin, Benjamin L; Goebel, Jens; Starke, Jeffrey R; Schauer, Daniel P; Eckman, Mark H

2013-01-01

210

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

PubMed Central

Background Rett Syndrome (RTT) is an Autism Spectrum Disorder and the leading cause of mental retardation in females. RTT is caused by mutations in the Methyl CpG-Binding Protein-2 (MECP2) gene and has no treatment. Our objective is to develop viral vectors for MECP2 gene transfer into Neural Stem Cells (NSC) and neurons suitable for gene therapy of Rett Syndrome. Methodology/Principal Findings We generated self-inactivating (SIN) retroviral vectors with the ubiquitous EF1? promoter avoiding known silencer elements to escape stem-cell-specific viral silencing. High efficiency NSC infection resulted in long-term EGFP expression in transduced NSC and after differentiation into neurons. Infection with Myc-tagged MECP2-isoform-specific (E1 and E2) vectors directed MeCP2 to heterochromatin of transduced NSC and neurons. In contrast, vectors with an internal mouse Mecp2 promoter (MeP) directed restricted expression only in neurons and glia and not NSC, recapitulating the endogenous expression pattern required to avoid detrimental consequences of MECP2 ectopic expression. In differentiated NSC from adult heterozygous Mecp2tm1.1Bird+/? female mice, 48% of neurons expressed endogenous MeCP2 due to random inactivation of the X-linked Mecp2 gene. Retroviral MECP2 transduction with EF1? and MeP vectors rescued expression in 95–100% of neurons resulting in increased dendrite branching function in vitro. Insulated MECP2 isoform-specific lentiviral vectors show long-term expression in NSC and their differentiated neuronal progeny, and directly infect dissociated murine cortical neurons with high efficiency. Conclusions/Significance MeP vectors recapitulate the endogenous expression pattern of MeCP2 in neurons and glia. They have utility to study MeCP2 isoform-specific functions in vitro, and are effective gene therapy vectors for rescuing dendritic maturation of neurons in an ex vivo model of RTT.

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

2009-01-01

211

Testosterone therapy improves the heart rate turbulence without effect on NT-proBNP level in men with metabolic syndrome.  

PubMed

It is now known that BNP and NT-proBNP levels are decreasing with increased BMI, regardless of other metabolic syndrome (MS) constituents. Additionally, testosterone deficiency may intensify frequency of ventricular rhythm disorders in obese individuals by inhibition of the parasympathetic system. Determination of heart rhythm turbulence (HRT) is a useful, noninvasive method used for evaluation of equilibrium of the vegetative system. The aim of the study was to evaluate effect of testosterone therapy on HRT and NT-proBNP levels in MS patients. Eighty males were qualified for the study. They were divided into 3 groups: I (n=30), males with testosterone deficiency syndrome and metabolic syndrome (MS+TDS+); II (n=25), males with MS+TDS-; III (n=25), healthy males. The patients with MS+TDS+ received Omnadrem 250 in the form of intramuscular injections for 9 weeks. Laboratory tests and 24-h Holter ECG were taken twice before the therapy and directly after completion of the therapy. Males with MS+TDS+ more often presented irregular HRT parameters and were characterised by lower NT-proBNP levels compared to the healthy individuals. Testosterone replacement therapy caused improvement of HRT and had no significant effect on the NT-proBNP level. Testosterone replacement therapy and body weight reduction may significantly decrease negative consequences of MS and TDS. PMID:24062090

Poliwczak, A R; Tyli?ska, M; Broncel, M

2014-02-01

212

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

PubMed

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

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

2014-07-01

213

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

PubMed Central

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

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

2011-01-01

214

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

PubMed Central

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

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

2014-01-01

215

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

PubMed

The subacromial syndrome is the most common source of shoulder pain. The mainstays of conservative treatment are non-steroidal anti-inflammatory drugs and exercise therapy. Recently, low-level laser therapy (LLLT) has been popularized in the treatment of various musculoskeletal disorders. The aim of this study is to evaluate the additive effects of LLLT with exercise in comparison with exercise therapy alone in treatment of the subacromial syndrome. We conducted a randomised clinical study of 80 patients who presented to clinic with subacromial syndrome (rotator cuff and biceps tendinitis). Patients were randomly allocated into two groups. In group I (n?=?40), patients were given laser treatment (pulsed infrared laser) and exercise therapy for ten sessions during a period of 2 weeks. In group II (n?=?40), placebo laser and the same exercise therapy were given for the same period. Patients were evaluated for the pain with visual analogue scale (VAS) and shoulder range of motion (ROM) in an active and passive movement of flexion, abduction and external rotation before and after treatment. In both groups, significant post-treatment improvements were achieved in all parameters (P?=?0.00). In comparison between the two groups, a significant improvement was noted in all movements in group I (P?=?0.00). Also, there was a substantial difference between the groups in VAS scores (P?=?0.00) which showed significant pain reduction in group I. This study indicates that LLLT combined exercise is more effective than exercise therapy alone in relieving pain and in improving the shoulder ROM in patients with subacromial syndrome. PMID:21538218

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

2011-10-01

216

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

217

Cell and gene therapy for genetic diseases: inherited disorders affecting the lung and those mimicking sudden infant death syndrome.  

PubMed

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

Keeler, Allison M; Flotte, Terence R

2012-06-01

218

Efficacy of antibiotic therapy for SAPHO syndrome is lost after its discontinuation: an interventional study  

PubMed Central

Introduction The acronym SAPHO was introduced in 1987 to unify the various descriptions of a seronegative arthritis associated with skin manifestations and to show synovitis, acne, pustulosis, hyperostosis, and osteitis with and without sterile multifocal osteomyelitis. The etiology of SAPHO syndrome is unknown, but an association with infection by semipathogenic bacteria like Propionibacterium acnes has been suggested. We conducted an interventional study of SAPHO patients receiving antibiotics. Methods Thirty-seven patients met the clinical criteria of SAPHO syndrome, 21 of them underwent a needle biopsy of the osteitis lesion, and 14 of them showed positive bacteriological cultures for P. acnes. Thirty patients (14 bacteriological positive and 16 without biopsy) were treated with antibiotics for 16 weeks. The activity of skin disease and osteitis were assessed by a physician using a scoring model (from 0 to 6). In addition, patients completed a Health Assessment Score (HAS, from 0 to 6). The erythrocyte sedimentation rate was determined and a MRI (of the osteitis lesion, radiologic activity score from 0 to 2) was performed in week 1 (W1), week 16 (W16), and week 28 (W28, 12 weeks after antibiotics). Results Twenty-seven patients continued the medication (azithromycin, n = 25, 500 mg twice a week; clindamycin, n = 1, 300 mg daily; or doxycycline, n = 1, 100 mg daily) for 16 weeks. After W16 the scores for MRI (1.5 to 1.1, P = 0.01), skin activity (3.2 to 1.2, P = 0.01), osteitis activity (4.0 to 2.1, P = 0.02), and HAS (3.3 to 2.1, P = 0.01) decreased significantly. However, this was followed by increasing values for MRI scores (1.2 to 1.4, P = 0.08), skin activity (1.2 to 1.7, P = 0.11), osteitis activity (1.9 to 2.7, P = 0.01), and HAS (2.2 to 3.3, P = 0.02) from W16 to W28. The comparison of the scores in W1 and W28 in these 12 patients showed no significant differences. Conclusions For the period of application, the antibiotic therapy seems to have controlled the disease. After antibiotic discontinuation, however, disease relapse was observed. SAPHO syndrome thus groups with other chronic inflammatory arthropathies with a need for permanent therapy.

2009-01-01

219

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, Peter J

2013-01-01

220

Effect of atorvastatin therapy on borderline vulnerable lesions in patients with acute coronary syndrome  

PubMed Central

Introduction It is still controversial whether borderline lesions with a vulnerable plaque should be stented early or simply treated pharmacologically. No data exist concerning the potential effects of statin therapy on borderline vulnerable lesions in patients with acute coronary syndrome (ACS). Material and methods Fifty patients with ACS whose culprit lesions were classified as “borderline lesions” were enrolled. All patients were treated with atorvastatin (20 mg) for 12 months. Intravascular ultrasound (IVUS) was performed and matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase-1 (TIMP-1), and high-sensitive C-reactive protein (hsCRP) levels were measured at baseline and 12-month follow-up. Results At 12-month follow-up, we found: 1) IVUS revealed that minimal lumen cross-sectional area (CSA) increased but plaque/media (P&M) area and plaque burden decreased. A total of 25 soft plaques (50%) were transformed into fibrous plaques. 2) ApoB, MMP-9 and hsCRP levels decreased, but TIMP-1 level increased. 3) Stepwise multivariate linear regression analysis showed that the independent predictors for changes in P&M area/year were the decrease in MMP-9 and hsCRP levels. Conclusions Atorvastatin therapy stabilized borderline vulnerable plaques and reversed atherosclerosis progression in patients with ACS. Reversal of this progression was accompanied by a decrease in the levels of plasma MMP-9 and hsCRP. Changes in MMP-9 and hsCRP could predict vulnerable plaque stabilization.

Yu, Dan-Qing; Lin, Shu-Guang; Chen, Ji-Yan; Xue, Ling; Li, Guang; Dong, Hao-Jian; Zhou, Ying-Ling

2011-01-01

221

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

2013-04-01

222

Low-level laser therapy of myofascial pain syndromes of patients with osteoarthritis of knee and hip joints  

NASA Astrophysics Data System (ADS)

The purpose of the given research is the comparison of efficiency of conventional treatment of myofascial pain syndromes of patients with osteoarthritis (OA) of hip and knee joints and therapy with additional application of low level laser therapy (LLLT) under dynamic control of clinical picture, rheovasographic, electromyographic examinations, and parameters of peroxide lipid oxidation. The investigation was made on 143 patients with OA of hip and knee joints. Patients were randomized in 2 groups: basic group included 91 patients, receiving conventional therapy with a course of LLLT, control group included 52 patients, receiving conventional treatment only. Transcutaneous ((lambda) equals 890 nm, output peak power 5 W, frequency 80 - 3000 Hz) and intravenous ((lambda) equals 633 nm, output 2 mW in the vein) laser irradiation were used for LLLT. Studied showed, that clinical efficiency of LLLT in the complex with conventional treatment of myofascial pain syndromes at the patients with OA is connected with attenuation of pain syndrome, normalization of parameters of myofascial syndrome, normalization of the vascular tension and parameters of rheographic curves, as well as with activation of antioxidant protection system.

Gasparyan, Levon V.

2001-04-01

223

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

PubMed

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

Hérodin, Francis; Mayol, Jean-François; Mourcin, Frédéric; Drouet, Michel

2005-01-01

224

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

PubMed Central

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

Ilkun, Olesya; Boudina, Sihem

2013-01-01

225

[Effect of estrogen therapy on the growth of Turner syndrome in girls treated with growth hormone].  

PubMed

Two groups of patients with Turner Syndrome (TS) were studied to analyse the effect of estrogens on their growth. Group 1 (G1): 14 patients treated with growth hormone (GH) who started estrogens (SO) (Premarín) at 15.3 +/- 0.9 years old. Group 2 (G2): 10 girls not treated with GH who started Premarín at 14.3 +/- 2.3 years old. Height SDS improvement in periods of time of GH and GH plus estrogen treatment was evaluated and compared between both groups. The gain in stature during estrogen therapy (final height-projected height at SO) was calculated. In G1 multiple regression was used to examine factors influencing the gain in stature during GH treatment (final height-projected height at the start of GH): bone age at the SO, chronological age at the SO and the time of GH treatment prior to SO. In G1 the height SDS improvement was 0.43 +/- 0.11 cm during GH treatment and 0.59 +/- 0.18 cm during GH plus oestrogens (p = 0.064). The height SDS improvement during estrogen therapy in G2 was 0.14 +/- 0.19 cm, smaller than in G1 (p < 0.001). Gain in stature during estrogen therapy was 5.3 +/- 1.8 cm in G1 and -0.6 +/- 4.2 cm in G2 (p = 0.001). In G1 the time of GH treatment prior to SO was the strongest predictor of the gain in stature during GH treatment (r = 0.89). PMID:11474872

Morin, A; Guimarey, L; Apezteguia, M; Santucci, Z

2001-01-01

226

Combination Systemic and Intravitreal Antiviral Therapy in The Management of Acute Retinal Necrosis Syndrome (An American Ophthalmological Society Thesis)  

PubMed Central

Purpose: To compare the outcomes of combination systemic and intravitreal antiviral therapy vs systemic antiviral therapy alone for treating acute retinal necrosis syndrome (ARN). We hypothesize that combination therapy might result in superior visual acuity (VA) and retinal detachment (RD) outcomes vs traditional systemic antiviral therapy alone. Methods: A retrospective, interventional, comparative single-center study of patients with ARN. We reviewed demographic data, herpesvirus diagnoses, polymerase chain reaction (PCR) results, VA, RD, and the use of systemic and intravitreal antiviral therapy. Outcome measures included VA improvement by 2 or more lines, severe visual loss, VA ?20/200, and RD. Results: We studied 29 eyes of 24 patients, treated from 1987 through 2009. Mean age was 42.6 years and mean follow-up was 44.0 months. Twelve patients (14 eyes) were treated with combined systemic and intravitreal antiviral therapy and 12 patients (15 eyes) with systemic therapy alone. Kaplan-Meier survival analysis revealed that patients receiving combination intravitreal and systemic antiviral therapy were more likely to have VA improved by 2 lines or greater (P=.006). Patients receiving combination therapy also showed a decreased incidence of progression to severe visual loss (0.13/patient-years [PY]) compared to patients receiving systemic therapy alone (0.54/PY, P=.02) and had decreased incidence of RD (0.29/PY vs 0.74/PY, P=.03). Conclusions: Combination oral and intravitreal antiviral therapy may improve visual and functional outcomes in patients with ARN. Clinicians should consider prompt administration of combination systemic and intravitreal antiviral therapy as first-line treatment for patients with clinical features of ARN.

Flaxel, Christina J.; Yeh, Steven; Lauer, Andreas K.

2013-01-01

227

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

228

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

229

Clinical, psychiatric and manometric profile of cyclic vomiting syndrome in adults and response to tricyclic therapy.  

PubMed

Our goal was to investigate 31 adult patients (mean age 29 years, range 18-62 years) meeting Rome II criteria for cyclic vomiting syndrome (CVS). All subjects completed a clinical questionnaire, a Hamilton Rating Scale for Anxiety (HAM-A) and Zung Depression Inventory. Gastric emptying time was assessed in 30 subjects and electrogastrogram (EGG) in 11 between acute attacks. Twenty-seven patients treated with amitriptyline completed a follow-up questionnaire. The mean age of onset of the patients was 30 years (range 14-53 years) and cycles of nausea and vomiting were accompanied by often-severe epigastric and diffuse abdominal pain. A typical attack ranged from 1 to 14 days, with the majority being 4-6 days. The HAM-A revealed that 84% had an anxiety disorder, and based on Zung Depression Inventory 78% suffered from mild-to-severe depression. Only 4 (13%) patients reported migraine, but 14 had a family history of migraine. Gastric emptying time was rapid in 23 (77%), normal in 4 and delayed in 3. The EGG was abnormal in 7 of 11 patients, with 4 having tachygastria. Of 13 patients using marijuana, 7 had symptom relief, while 2 had resolution of CVS after stopping use. The overall treatment experience in the 24 patients receiving amitriptyline up to 1 mg kg(-1) day(-1) for at least 3 months indicated that 93% had decreased symptoms and 26% achieved full remission. Cyclic vomiting syndrome in adults has the following hallmarks: prominence of accompanying abdominal pain and increased prevalence of anxiety and depression, rapid gastric emptying and tachygastric EGG, and successful suppression of attacks by chronic amitriptyline therapy. PMID:17300289

Namin, F; Patel, J; Lin, Z; Sarosiek, I; Foran, P; Esmaeili, P; McCallum, R

2007-03-01

230

Prolonged remission of a demyelinating neuropathy in a patient with lymphoma and Sjögren's syndrome after Rituximab therapy.  

PubMed

Chronic acquired demyelinating polyneuropathies may be refractory to conventional therapy including corticosteroids, plasma exchange, and intravenous immunoglobulin (Ig) or require long-term immunotherapy to maintain remission. Use of alternative approaches such as Rituximab, an anti-CD20 antibody, in the treatment of demyelinating polyneuropathy, unrelated to IgM gammopathy and myelin-associated glycoprotein antibodies, has been the subject of only a few case reports. We report the case of a 79-year-old woman with a distal acquired demyelinating polyneuropathy in the context of Sjögren's syndrome, IgG paraproteinemia, and occult lymphoma who has had an excellent and durable response to Rituximab therapy. PMID:20215986

Botez, Stephan A; Herrmann, David N

2010-03-01

231

Complete Remission of del(5q) Myelodysplastic Syndrome after 7 Days of Lenalidomide Therapy Gives an Alert!  

PubMed Central

Myelodysplastic syndrome (MDS) refers to a group of haematological, monoclonal disorders. A 50-year-old woman was diagnosed with MDS 5q deletion syndrome [del(5q)], becoming dependent on blood transfusion after long-term treatment with cytotoxic drugs for chronic scleritis. Lenalidomide therapy (10 mg/day) led to profound pancytopaenia, followed by recovery of her blood cell counts. A cytogenetic study, repeated 4 months after lenalidomide treatment, revealed complete remission after only 1 week of lenalidomide therapy. Lenalidomide was approved for low- and intermediate-1-risk MDS, where it normalises platelet counts and induces haematological and cytogenetic remission. This patient has remained transfusion independent for 3 years by continuing on a minimal maintenance dose of lenalidomide. Starting MDS patients on lenalidomide has to be done cautiously or with only 5 mg/day because of the potentially high sensitivity of the stem cells to this immunomodulatory agent in MDS patients.

Al-Jafar, Hassan A.; Al-Azmi, Salih; Qassem, J.A.; Hasan, Eman A.; Alduaij, Arwa

2012-01-01

232

Hepatorenal syndrome and type 1 and 2 cardiorenal syndromes: distinct competing medical therapies applied to a similar background of vasomotor reactive nephropathy.  

PubMed

The authors summarize some current views regarding the pharmacologic therapies of hepatorenal and cardiorenal syndromes, respectively. A common pathogenetic background of the two edematous disorders is outlined, consisting in reduced effective blood arterial volume ? due to the splanchnic vasodilation in the hepatorenal syndrome (HRS) and to the fall in cardiac output and the rise in central venous pressure in cardiorenal syndrome (CRS). In both diseases, arterial underfilling elicits multiple water- and sodium- retentive mechanisms, by activating sympathetic nervous system and stimulating both rennin-angiotensin-aldosterone and vasopressin systems. These neurohormonal adjustments subsequently concur to a vasomotor nephropathy which originates - as a same kind of vasoconstrictor reflex renal response ? from the splanchnic vasodilation, in the case of liver cirrhosis, or from the fall in renal perfusion and filtration gradients in the case of cardiorenal syndrome. Despite these pathogenetic similarities, the renal insufficiency of HRS compared to that of CRS is treated using diametrically opposite approaches: actually withdrawal of diuretics and administration of vasoconstrictor agents is the first choice in the case of HRS, while CRS is tackled by forcing diuretic regimen and by continuing vasodilator treatment with ACE-inhibitors. The pros and cons of these strategies ? which are still matter of debate among the physicians and researchers ? are then succinctly presented and discussed. PMID:24500221

De Vecchis, R; Esposito, C; Di Biase, G; Ariano, C

2014-02-01

233

Adjunctive use of tetracycline fibers with nonsurgical periodontal therapy in an adult with Down syndrome: a case report.  

PubMed

Individuals with Down syndrome (DS) are susceptible to severe periodontal disease, due to immune alterations related to functional defects of polymorphonuclear leukocytes and monocytes. The adjunctive use of locally delivered antimicrobials has been proven to be beneficial, especially in areas where mechanical therapy might fail. This article describes the management of a patient with DS who had aggressive periodontitis. The treatment used a local drug delivery system with tetracycline fibers as an adjunct to scaling and root planing. PMID:22416988

Gautami, Penmetsa Subadhra; Ramaraju, Alluri Venkata; Gunashekhar, Madiraju

2012-03-01

234

Double-blind, placebo-controlled, hormonal, syndromal and EEG mapping studies with transdermal oestradiol therapy in menopausal depression  

Microsoft Academic Search

In a double-blind, placebo-controlled study, the antidepressant and vigilance-promoting properties of transdermal oestrogen in post-menopausal depression were investigated utilizing hormonal, syndromal and EEG mapping evaluations. Sixty-nine menopausal women, aged 45–60 years without previous hormonal replacement therapy, diagnosed as major depression without psychotic or suicidal symptoms (DSM-III-R criteria), were randomly assigned to a 3-month treatment with transdermal oestradiol [Estraderm TTS (ETTS)

B. Saletu; N. Brandstätter; M. Stamenkovic; P. Anderer; H. V. Semlitsch; J. Grünberger; L. Linzmayer; K. Decker; G. Binder; M. Metka; G. Heytmanek; J. Huber; Ch. Kurz; W. Knogler; B. Koll

1995-01-01

235

Frequent p53 overexpression in therapy related myelodysplastic syndromes and acute myeloid leukemias: an immunohistochemical study of bone marrow biopsies.  

PubMed

p53 overexpression was studied immunohistochemically in paraffin-embedded bone marrow biopsies using a recently described technique for antigen retrieval based on microwave oven heating of paraffin sections. Using a monoclonal antibody (PAb1801) that reacts with human cellular p53, nuclear staining was detected in 7/11 (63%) therapy-related myelodysplastic syndromes and in 3/4 (75%) therapy-related acute myeloid leukemias. Conversely, staining for p53 was seen only in 9/40 (22%) cases of "primary" hematologic conditions (P < 0.007); these included myelodysplastic syndromes [#2], acute myeloid leukemia [#4], and chronic granulocytic leukemia in accelerated phase or blast crisis [#3]. Biopsies of normal controls and of chronic granulocytic leukemia in stable phase were consistently p53(-). Nine of the 10 karyotyped p53(+) acute myeloid leukemia/myelodysplastic syndrome cases showed complex cytogenetic findings with frequent involvement of chromosome 5 and/or 7. Only four of the 33 karyotyped p53(-) cases showed similar cytogenetic changes. Chromosome 17 involvement was present in four of 13 (31%) cytogenetically assessed p53+ cases, but in none of the p53(-). In univariate analysis, p53 expression in both MDS and AML was significantly associated with shorter survival. The frequent overexpression of p53 in therapy-related myelodysplastic syndromes, therapy-related acute myeloid leukemias and in accelerated phase/blast crisis, chronic granulocytic leukemia and its strong association with complex karyotypes suggests an important role of this gene in the pathogenesis of these leukemic conditions. PMID:8248107

Orazi, A; Cattoretti, G; Heerema, N A; Sozzi, G; John, K; Neiman, R S

1993-09-01

236

58. Rett Syndrome Is Reversible and Treatable by MeCP2 Gene Therapy into the Striatum in Mice  

Microsoft Academic Search

Introduction and aims: Mutations in the methyl-CpG-binding protein 2 (MeCP2), which binds methylated DNA and regulates gene expression, cause Rett syndrome (RTT). RTT is a neurodevelopmental disorder characterized by normal early psychomotor development, followed by the loss of psychomotor skills, and by the onset of stereotyped hand movement and of gait disturbances; no effective therapy has been developed for the

Ken-ichiro Kosai; Akira Kusaga; Takeo Isagai; Koji Hirata; Satoshi Nagano; Yoshiteru Murofushi; Tomoyuki Takahashi; Sachio Takashima; Toyojiro Matsuishi

2005-01-01

237

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

Microsoft Academic Search

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

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

1996-01-01

238

Concentrated ascites re-infusion therapy for pseudo-Meigs' syndrome complicated by massive ascites in large pedunculated uterine leiomyoma.  

PubMed

Pseudo-Meigs' syndrome accompanied by massive ascites in uterine leiomyoma is rare. We encountered a rare case of a 37-year-old, nulliparous woman with a lower abdominal tumor and severe abdominal distention due to massive ascites. Serum cancer antigen 125 and vascular endothelial growth factor levels were elevated to 1007.9?U/mL and 103?pg/mL, respectively. She was tentatively diagnosed with ovarian cancer. Emergency concentrated ascites re-infusion therapy was performed to improve dyspnea, abdominal pain, and her preoperative respiratory condition. Concentrated ascites re-infusion therapy eliminated dyspnea and abdominal discomfort without decreasing serum albumin levels. The patient underwent laparotomy, which revealed a fist-sized pedunculated uterine leiomyoma arising from the right uterine fundus. Myomectomy was performed. Pseudo-Meigs' syndrome mimics advanced ovarian cancer due to massive ascites and markedly elevated serum cancer antigen 125 and vascular endothelial growth factor levels. Concentrated ascites re-infusion therapy was effective in improving the subjective symptoms of pseudo-Meigs' syndrome and the patient's preoperative condition. PMID:25056475

Yonehara, Yukie; Yanazume, Shintaro; Kamio, Masaki; Togami, Shinichi; Tasaki, Takashi; Douchi, Tsutomu

2014-07-01

239

Plasma therapy in atypical haemolytic uremic syndrome: lessons from a family with a factor H mutation  

PubMed Central

Whilst randomised control trials are undoubtedly the best way to demonstrate whether plasma exchange or infusion alone is the best first-line treatment for patients with atypical haemolytic uremic syndrome (aHUS), individual case reports can provide valuable information. To that effect, we have had the unique opportunity to follow over a 10-year period three sisters with aHUS associated with a factor H mutation (CFH). Two of the sisters are monozygotic twins. A similar natural evolution and response to treatment would be expected for the three patients, as they all presented with the same at-risk polymorphisms for CFH and CD46 and no identifiable mutation in either CD46 or CFI. Our report of different modalities of treatment of the initial episode and of three transplantations and relapses in the transplant in two of them, strongly suggest that intensive plasma exchange, both acutely and prophylactically, can maintain the long-term function of both native kidneys and allografts. In our experience, the success of plasma therapy is dependent on the use of plasma exchange as opposed to plasma infusion alone, the prolongation of daily plasma exchange after normalisation of haematological parameters followed by prophylactic plasma exchange, the use of prophylactic plasma exchange prior to transplantation and the use of prophylactic plasma exchange at least once a week posttransplant with immediate intensification of treatment if there are any signs of recurrence.

Strain, Lisa; Goodship, Tim H. J.

2008-01-01

240

Glucocorticoid levels in maternal and cord serum after prenatal betamethasone therapy to prevent respiratory distress syndrome.  

PubMed Central

Serum glucocorticoid levels were determined in 20 mothers and 43 premature infants who received prenatal betamethasone therapy for prevention of respiratory distress syndrome (RDS). Maternal betamethasone peaked at 75 microg cortisol equivalents per 100 ml 1 h after injection of 12 mg steroid and declined to half by 6 h. Betamethasone in cord blood was 14.3 microg cortisol equivalents per 100 ml at 1 h, decreased to a level of 4.7 at 20 h, and was not detected 2 days after a second dose at 24 h. After the second dose, the mean level of cortisol in cord blood was 5.9 microg per 100 ml compared with 13.05 microg per 100 ml (p less than 0.001) in untreated premature infants. The unbound glucocorticoid activity in treated infants delivered 1-10 h after the second dose (mean, 8.4 microg per 100 ml) is similar to the unbound cortisol level after birth in untreated premature infants who develop RDS. These findings indicate that (a) serum glucocorticoid levels in the physiologic stress range can induce lung maturation in the human and (b) antenatal treatment with this dose of betamethasone does not expose the human fetus to potentially harmful pharmacologic levels of steroid.

Ballard, P L; Granberg, P; Ballard, R A

1975-01-01

241

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

PubMed Central

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.

HADJ, JAMAL OULAD; BRAVEN, ROGIER DEN; TILLIER, CORRINE; SCHRIJVER, HANS M.; VERHEUL, HENK M.W.; VAN DER VLIET, HANS J.

2012-01-01

242

[Efficacy of complex therapy with metformin and ramipril combination for patients with metabolic syndrome].  

PubMed

We have studied efficiency of a complex therapy with metformin and ramipril combination (1000 mg and 5 mg per day) respectively in patients with metabolic syndrome (MS). The group of patients with MS which answered the basic criteria IDF (2005) was determined. Carbohydrate and Lipidic metabolism were studied. Patients were characterized with raised weight index (WI), arterial hypertension, increased concentration of triglycerides in blood serum, of glucose, of HbAlc level and S-peptide, and also high level of endotelin (1-38) and CD32+CD40+circulating particles of endothelium. Three months treatment lead to decrease in WI, arterial pressure, triglycerides concentration, HbAlc, glucose, except CD32+CD40+. Six months treatment lead to more expressed positive dynamics. Thus, metformin and ramipril combination in patients with MS leads to decrease in insulin resistancy, carbohydrate and lipid metabolism normalization, to restoration of endothelium functions that is possible to consider as prophylaxis of the development of type 2 diabetes melitus and its cardiovascular complications. PMID:21714292

Ka?dashev, I P; Savchenko, L H; Ka?dasheva, E I; Kutsenko, N L; Kutsenko, L O; Solokhina, I L; Mamontova, T V

2010-01-01

243

[The role of testosterone drugs in combined therapy of erectile dysfunction in patients with metabolic syndrome].  

PubMed

The aim of the trial was assessment of efficacy of combined therapy with testosterone drug (androgel) and wardenafil in patients with erectile dysfunction (ED) and metabolic syndrome (MS). The trial included 16 males with organic ED, laboratory and clinical symptoms of hypogonadism and MS (mean age 63.8 +/- 8.4) who had failed monotherapy with phosphodiesterase of type 5 (FDE-5). Both routine methods and special methods of ED and MS diagnosis with application of International Index of Erectile Function were used in examination. MS was diagnosed according to the criteria of the National Cholesterol Educational Program. Wardenafil was given in a dose 20 mg 1 hour before coitus, but not less than 4 tablets a month. Androgel applications were given in a daily dose 50 mg. The treatment lasted for 3 months. The combined treatment significantly reduced body mass index, waist circumference, improved erectile function and libido. Erectile function normalized in 3 (18.75%) patients. Carbohydrate and lipid metabolism improved, the levels of total and free testosterone normalized. Elevation of sexual hormones concentration eliminated clinical hypogonadism symptoms in 68.75% patients. Thus, combined treatment with FDE-5 inhibitor wardenafil and testosterone drug androgel is effective, safe, improves erectile function, hormonal, lipid and carbohydrate blood profile and can be used in patients with MS, ED and hypogonadism. PMID:17915451

Mazo, E B; Gamidov, S I; Iremashvili, V V; Sotnikova, E M

2007-01-01

244

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

PubMed

The efficacy of longer initial course of prednisolone with standard regimen was compared in children with idiopathic nephrotic syndrome (INS). It was a randomized controlled trial in which patients with INS who responded to initial course of prednisolone, either long or standard regimen were included and followed for one year after completion of treatment of first attack. The long regimen consisted of prednisolone 60mg/m²/day for six weeks followed by 40mg/m² every alternate day single morning dose for further six weeks. The standard regimen consisted of prednisolone 60mg/m²/day for four weeks followed by 40mg/m² every alternate day single morning dose for further four weeks. There were 93 children who fulfilled the criteria of the study, 47 from long group and 46 from standard group though ultimately 72 completed the study (41 from long and 31 from standard group). The primary outcome measure was relapse within next one year. Relapse within one year was noted in 73.2% of long and 64.5% of standard group. The odds ratio for relapse within one year was 0.80 (95% confidence interval 0.22, 2.05). This did not reach statistical significance (p=0.696). Adverse effects of prednisolone between two groups were also not statistically significant (p>0.05). Prolongation of prednisolone therapy for initial episode of steroid-sensitive INS does not have a beneficial effect on the outcome in next one year. PMID:24858152

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

2014-04-01

245

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

PubMed

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

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

2013-12-01

246

Insulin resistance is increased by transdermal estrogen therapy in postmenopausal women with cardiac syndrome X.  

PubMed

Estrogen has been reported to have both short- and long-term effects on the cardiovascular system. However, it remains to be examined how short-term transdermal estrogen therapy (TET) affects insulin sensitivity (SI) in patients with cardiac syndrome X (CSX), who are characterized by elevated insulin resistance. SI was assessed in a randomized, double-blind, placebo-controlled crossover study by minimal model analysis in seven postmenopausal women with CSX treated by TET. SI decreased by 32 +/- 8.3%, from 5.94 +/- 1.14 at baseline to 3.61 +/- 0.40 [(10(-4) x min(-1))/(microU/ml)] during TET (p = 0.03). Time to the onset of symptoms increased from 414.2 +/- 51.0 s at baseline to 450.0 +/- 53.2 s (p = 0.04). We conclude that TET increases SI in postmenopausal women with CSX. This effect is unrelated to the beneficial anti-ischemic effects on exercise duration. PMID:11385189

Assali, A R; Jabara, Z; Shafer, Z; Solodky, A; Herz, I; Sclarovsky, E; Strasberg, B; Sclarovsky, S; Fainaru, M

2001-01-01

247

[The therapy of non-traumatic vegetative state syndrome in children].  

PubMed

Twenty nine non-traumatic appalic syndrome (AS) cases of various etiology were observed in children aged from 3 to 14 years. The clinical picture study was carried out, along with brain structure visualization in vivo (CT and MRT), EEG and cerebral blood flow detection with transcranial ultrasonic dopplerography. All the cases were studied in dynamics in relation to influence of complex course therapy developed by the authors, including vasoactive drugs, nootropics, craniopuncture, acupuncture and electrostimulation of craniopuncture zones. Five patients (17.2%) had a distinct positive dynamics with complete consciousness rehabilitation on the background of moderate neurological deficit regress. In general, CT-data and changes of EEG and cerebral blood flow in AS were not found to have any prognostic value and to be etiologically specific. But changes of EEG and cerebral blood flow in response to the treatment appear to be positive prognostic sign. The prognosis for AS due to meningoencephalitis is worse than one for AS due to hypoxic-ischemic encephalopathy. The non-traumatic AS duration for more than 2 months corresponds to unfavorable outcome. PMID:11765607

Evtushenko, S K; Omel'ianenko, A A

2001-01-01

248

Energy expenditure during synthetic surfactant replacement therapy for neonatal respiratory distress syndrome.  

PubMed

Little information is available on the energy expenditure of infants with increased work of breathing from respiratory distress syndrome (RDS). A study was carried out to determine whether surfactant replacement therapy modifies respiratory gas exchange in newborn infants with RDS and an arterial-alveolar oxygen tension ratio of less than 0.22. In a double-blind, placebo-controlled, rescue trial, infants received either two 5 ml/kg doses of a synthetic surfactant, Exosurf Neonatal, or air placebo. Of 23 infants ventilated for RDS, 11 were randomly assigned to receive air and 12 to receive surfactant. Oxygen consumption, carbon dioxide production, respiratory quotient, and metabolic rate were measured by computerized, closed-circuit, indirect calorimetry. Concomitantly, transcutaneous oxygen and carbon dioxide tension were continuously recorded. Oxygen consumption and carbon dioxide production remained constant during the period infants received surfactant. In patients randomly assigned to surfactant, a decrease in respiratory quotient was observed after the first (p less than 0.025) but not the second dose. This decrease was possibly related to a change in substrate utilization. The improved clinical outcomes reported among infants receiving surfactant were not accompanied by changes in energy expenditure. PMID:1735848

Hazan, J; Chessex, P; Piedboeuf, B; Bourgeois, M; Bard, H; Long, W

1992-02-01

249

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

PubMed

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

Grgi?, Vjekoslav

2013-01-01

250

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

PubMed Central

Background Approximately 70% of all patients with myelodysplastic syndrome (MDS) present with lower-risk disease. Some of these patients will initially respond to treatment with growth factors to improve anemia but will eventually cease to respond, while others will be resistant to growth factor therapy. Eventually, all lower-risk MDS patients require multiple transfusions and long-term therapy. While some patients may respond briefly to hypomethylating agents or lenalidomide, the majority will not, and new therapeutic options are needed for these lower-risk patients. Our previous clinical trials with ezatiostat (ezatiostat hydrochloride, Telentra®, TLK199), a glutathione S-transferase P1-1 inhibitor in clinical development for the treatment of low- to intermediate-risk MDS, have shown significant clinical activity, including multilineage responses as well as durable red-blood-cell transfusion independence. It would be of significant clinical benefit to be able to identify patients most likely to respond to ezatiostat before therapy is initiated. We have previously shown that by using gene expression profiling and grouping by response, it is possible to construct a predictive score that indicates the likelihood that patients without deletion 5q will respond to lenalidomide. The success of that study was based in part on the fact that the profile for response was linked to the biology of the disease. Methods RNA was available on 30 patients enrolled in the trial and analyzed for gene expression on the Illumina HT12v4 whole genome array according to the manufacturer’s protocol. Gene marker analysis was performed. The selection of genes associated with the responders (R) vs. non-responders (NR) phenotype was obtained using a normalized and rescaled mutual information score (NMI). Conclusions We have shown that an ezatiostat response profile contains two miRNAs that regulate expression of genes known to be implicated in MDS disease pathology. Remarkably, pathway analysis of the response profile revealed that the genes comprising the jun-N-terminal kinase/c-Jun molecular pathway, which is known to be activated by ezatiostat, are under-expressed in patients who respond and over-expressed in patients who were non-responders to the drug, suggesting that both the biology of the disease and the molecular mechanism of action of the drug are positively correlated.

2012-01-01

251

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

252

Rituximab Therapy for Primary Sj?gren's Syndrome: An Open-Label Clinical Trial and Mechanistic Analysis  

PubMed Central

Objective To study the safety and clinical efficacy of rituximab therapy for primary Sjögren’s syndrome, as well as investigate its mechanisms. Methods Patients with primary Sjögren’s syndrome were enrolled in an open-label trial and received rituximab (1 g) on days 1 and 15 and followed through week 52. The primary endpoint was safety, with secondary endpoints evaluating clinical and biologic efficacy. Blood was obtained for enumeration of lymphocyte subsets, measurement of serum autoantibodies and BAFF levels, and analysis of gene expression. Results Twelve female subjects with primary Sjögren’s syndrome were administered rituximab. They had a median (range) age of 51 (34–69) years and a median (range) disease duration of 8.0 (2–18) years. We observed no unexpected toxicities from rituximab therapy. Modest improvements were observed at week 26 in patient-reported symptoms of fatigue and oral dryness, with no significant improvement in the objective measures of lacrimal and salivary gland function. The recovery of blood B cells following the nadir from rituximab therapy was characterized by a predominance of transitional B cells and a lack of memory B cells. While blood B cell depletion was associated with an increase in serum BAFF levels, no significant changes were observed in the levels of serum anti-Ro/SSA, anti-La/SSB, and anti-muscarinic receptor 3 autoantibodies or in the blood IFN signature. Conclusion In primary Sjögren’s syndrome, a single treatment course of rituximab was not associated with any unexpected toxicities and led to only modest clinical benefits despite effective depletion of blood B cells.

St Clair, E. William; Levesque, Marc C.; Luning Prak, Eline T.; Vivino, Frederick B.; Alappatt, Chacko J.; Spychala, Meagan E.; Wedgwood, Josiah; McNamara, James; Moser Sivils, Kathy L.; Fisher, Lytia; Cohen, Philip

2013-01-01

253

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

254

Effect of metformin therapy on 2-h post-glucose insulin levels in patients of polycystic ovarian syndrome  

PubMed Central

AIMS: To evaluate if 2-h post glucose insulin level is an effective tool to monitor insulin resistance in response to metformin therapy, in infertile women with polycystic ovarian syndrome (PCOS). SETTINGS AND DESIGN: This prospective observational study was carried out in a tertiary care infertility clinic. MATERIALS AND METHODS: 40 women with PCOS were categorized as having insulin resistance if fasting or 2-h post glucose insulin levels were >25 or >41?U/ml respectively. Post glucose insulin was compared before and after six months of metformin therapy along with other clinical, hormonal and metabolic parameters by using McNemar and the Student’s t-test. RESULTS: Fasting insulin was elevated in 4 (10%) and post-load insulin in 34 (85%) patients; after metformin therapy respective values were 2 (5%) and 16 (40%). Metformin therapy reduced post glucose insulin levels (P<0.001), improved the regularity of periods (P<0.001) and resulted in reduction of LH levels (P<0.001), total testosterone (P<0.001) and mean Body mass index (BMI) (P=0.047). Metformin therapy did not alter waist-hip ratio and fasting insulin levels. CONCLUSION: 2-h post glucose insulin level is an effective tool to monitor insulin resistance in PCOS patients and improves significantly after metformin therapy, similar to improvements observed in clinical, hormonal and metabolic parameters.

Saxena, Pikee; Prakash, Anupam; Nigam, Aruna

2010-01-01

255

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

2013-10-01

256

[Case of tuberculosis-associated hemophagocytic syndrome in a hemodialysis patient under steroid therapy].  

PubMed

A 70-year-old woman was referred and admitted to our hospital with fever of unknown etiology. She had a past medical history of pulmonary tuberculosis. Ten weeks before admission she was diagnosed with acute renal failure caused by crescentic glomerulonephritis. Oral steroid therapy was not effective and she required dialysis. On admission, she was started on empiric antibiotic treatment, with the suspicion of bacterial infection. On the 3rd hospital day, she developed sudden hypotension and underwent direct hemoperfusion with a polymyxin B immobilized fiber. Soon after, her blood pressure normalised. Her inflammatory level apparently then improved in terms of white blood cell count and C-reactive protein, although severe fatigue and liver dysfunction persisted. On the 17th hospital day, her blood pressure went down again, accompanied by progressive pancytopenia and significant increase in serum vitamin B12, lactate dehydrogenase and uric acid. The patient was transmitted to the intensive care unit where she received bone marrow aspiration. The result revealed marked hemophagocytosis. Suspecting lymphoma-associated hemophagocytic syndrome (HPS), we administered high-dose steroid and combination chemotherapy. The treatment had no effect, and the patient died on the 21st hospital day. The autopsy demonstrated a large number of tuberculous bacilli, marked hemophagocytosis and necrosis without granuloma formation in multiple organs, leading to the pathological diagnosis of tuberculosis-associated HPS. Tuberculosis in one of the major causes for morbidity and mortality in hemodialyzed patients. It often shows atypical clinical manifestation and is difficult to diagnose. HPS in general runs a mild course unless it is lymphoma or EB virus-associated. This case seemed like bacterial infection improved with antibiotics but turned out to be a rapidly progressive tuberculosis-associated HPS. A careful examination and extensive laboratory workup is necessary to rule out tuberculosis, particularly in patients undergoing hemodialysis. PMID:19999590

Hori, Mayuko; Yoshida, Reiko; Aoyama, Isao; Ichida, Sizunori

2009-01-01

257

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

258

Placebo-controlled investigation of low-level laser therapy to treat carpal tunnel syndrome.  

PubMed

Abstract Objective: This study investigated the short-term efficacy of low-level laser therapy (LLLT) in patients with mild to moderate carpal tunnel syndrome (CTS), lasting for <1 year. Methods: Seventy-nine patients with CTS were included in this double-blind, placebo-controlled study, and randomly divided in two treatment groups: Experimental group (EG), active laser group (40 patients); and control group (CG), placebo (sham) laser group (39 patients). A GaAlAs diode laser [780?nm, 30?mW continuous wave (CW), 0.785?cm(2), 38.2 mW/cm(2)] was applied in contact with four points perpendicularly to the skin over the carpal tunnel area for 90?sec per point (2.7?J, 3.4?J/cm(2)/point). Both groups were treated five times per week, once a day over 2 weeks, followed by 10 treatments every other day for 3 weeks, that is, for a total of 20 treatments. Clinical assessment, including visual analogue scale (VAS) pain rating, Tinel's sign, and median nerve conduction studies (NCSs) were evaluated before, and 3 weeks after, the last LLLT treatment. Results: Significant reduction in pain, reduction in the percentage of patients with a positive Tinel's sign, and shortening of sensory and motor latency time in the NCS examination was observed in the experimental LLLT group (but not in the control group). Conclusions: This study has observed and documented the statistically significant short-term effects of LLLT on CTS patients in comparison with a placebo group. The results support this conclusion, especially if the LLLT is applied in the earlier stages of CTS, and with mild to moderate cases. PMID:24905929

Lazovic, Milica; Ilic-Stojanovic, Olivera; Kocic, Mirjana; Zivkovic, Vesna; Hrkovic, Marija; Radosavljevic, Natasa

2014-06-01

259

Metformin therapy decreases hyperandrogenism and ovarian volume in women with polycystic ovary syndrome.  

PubMed

Background: It is well known that there is a close relationship between elevated androgen plasma levels and the ultrasound findings of stromal hypertrophy in polycystic ovary syndrome (PCOS). The objective of this study was to investigate the effects metformin on the hyperandrogenism and ovarian volume in PCOS.Methods: The study is an unrandomized clinical trial with before-after design. Twenty eight patients with infertility (male or female factor) meeting the Rotterdam ESHRE/ASRM criteria for PCOS were studied during the 2008-2009. The anthropometric characteristics of the patients, mean bilateral ovarian volume, and morphology by trans vaginal sonography as well as the plasma levels of leutinizing hormone, follicle stimulating hormone, estradiol, testosterone, 17-?-hydroxyprogesterone, and dehydroepianderosterone sulfate were obtained before and after treatment with metformin (500 mg three times a day) for three months. Paired t, Pearson's Correlation Coefficient, or Partial Correlation test was used to analyze the findings. Results: The patients had a mean age of 25.67 years. A significant reduction in mean ovarian volume (11.70±4.31 ml vs 8.27±3.71 ml P=0.001), body mass index (BMI, 28.11±4.55 kg/m(2) vs 26.84±4.55 kg/m(2) P=0.000) and serum androgen levels was seen after three months of treatment with metformin. There was positive correlations between the ovarian volume and serum testosterone level (r=0.589, P=0.001) or BMI (r=0.663, P=0.000).Conclusion: Metformin therapy may lead to a reduction in ovarian volume. It is likely that the reduction of ovarian volume reflect a decrease in the mass of androgen producing tissues.Trial Registration Number: IRCT138903244176N1. PMID:23358726

Farimani Sanoee, Marzieh; Neghab, Nosrat; Rabiee, Soghra; Amiri, Iraj

2011-06-01

260

Translating molecular advances in fragile X syndrome into therapy: a review.  

PubMed

Fragile X syndrome is an inherited disease with cognitive, behavioral, and neurologic manifestations, resulting from a single genetic mutation. A variety of treatments that target individual symptoms of fragile X syndrome are currently utilized with limited efficacy. Research in animal models has resulted in the development of potential novel pharmacologic treatments that target the underlying molecular defect in fragile X syndrome, rather than the resultant symptoms. This review describes recent advances in our understanding of the molecular basis of fragile X syndrome and summarizes the ongoing clinical research programs. PMID:24813413

Hagerman, Randi J; Des-Portes, Vincent; Gasparini, Fabrizio; Jacquemont, Sébastien; Gomez-Mancilla, Baltazar

2014-04-01

261

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

PubMed

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. PMID:3081708

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

1986-02-01

262

Azacitidine in the Treatment of Pediatric Therapy-related Myelodysplastic Syndrome After Allogeneic Hematopoietic Stem Cell Transplantation.  

PubMed

We herein present a case of pediatric therapy-related myelodysplastic syndrome (t-MDS) with complex karyotype who was treated with azacitidine (AZA) for AML1-EVI1 fusion transcript as minimal residual disease after allogeneic hematopoietic stem cell transplantation (HSCT). The patient was started on AZA 41 days after the HSCT without having achieved complete remission. After 9 cycles of AZA, the AML1-EVI1 fusion transcript disappeared, and there was no manifestation of graft versus host disease during AZA treatment. Preemptive AZA treatment for minimal residual disease has an acceptable safety profile and appears to be an effective strategy for preventing or substantially delaying hematological relapse in pediatric patients with high-risk myelodysplastic syndrome after HSCT. PMID:24608072

Inoue, Akiko; Kawakami, Chihiro; Takitani, Kimitaka; Tamai, Hiroshi

2014-07-01

263

Veno-occlusive disease in a male patient with Marfan syndrome and common acute lymphoblastic leukemia during induction therapy.  

PubMed

Veno-occlusive disease (VOD) of the liver is characterized by jaundice, painful hepatomegaly, and retention of fluids. VOD is a severe complication in allogeneic stem cell or bone marrow transplantation. Additionally, the disease has been described in children suffering from nephroblastoma or rhabdomyosarcoma, treated with intense chemotherapy. Recently, VOD has been shown to be a complication in the treatment of myeloid leukemia with anti-CD33 linked to calicheamicin. We report the unusual case of a 21-year-old male patient with Marfan syndrome, diagnosed of acute lymphoblastic leukemia, who developed severe VOD during induction therapy after a single application of 2 mg vincristine. We speculate that coincidence of Marfan syndrome and application of induction chemotherapy might favor the disease in our patient. PMID:12761649

Kraemer, D M; Waschke, J; Kunzmann, V; Wilhelm, M

2003-07-01

264

Functional and EMG responses to a physical therapy treatment in patellofemoral syndrome patients  

Microsoft Academic Search

There are several pathologies related to the patellofemoral joint, in which the patellofemoral syndrome is one of the most com- mon and challenging to treat. The patellofemoral syndrome results from a malalignment of the knee extensor mechanism. The pur- pose of our study was to describe and compare EMG responses of the vastus medialis and vastus lateralis muscles while walking

C. N. Sacco; Gil Kiyoshi Konno; Guilherme Benetti Rojas; Antonio Carlos Arnone; Anice de Campos Passaro; Amelia Pasqual Marques; Cristina Maria Nunes Cabral

265

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

266

Acute respiratory distress syndrome as an initial presentation of hemophagocytic lymphohistiocytosis after induction therapy for acute myeloid leukemia.  

PubMed

A 7-month-old girl with acute myeloid leukemia (AML) developed acute respiratory distress syndrome (ARDS) during the pancytopenic period after induction chemotherapy. Respiratory failure did not improve despite intensive treatments. Eventually, hemophagocytic lymphohistiocytosis (HLH) was diagnosed based on hemophagocytosis in bone marrow, and high soluble interleukin-2 receptor (sIL-2R) and ferritin levels. Even after cyclosporin A was started against HLH, she did not recover. Autopsy showed macrophage proliferation in bone marrow and lymph nodes. HLH should be considered, even in the pancytopenic period after chemotherapy, when patients develop ARDS that does not respond to supportive therapies. PMID:21083362

Nishikawa, Takuro; Okamoto, Yasuhiro; Tanabe, Takayuki; Shinkoda, Yuichi; Kodama, Yuichi; Kakihana, Yasuyuki; Goto, Masamichi; Kawano, Yoshifumi

2011-04-01

267

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

PubMed Central

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

Kulkarni, Ranganath R.; Kulkarni, Pratibha R.

2013-01-01

268

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

PubMed Central

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

2010-01-01

269

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

PubMed

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

Turpie, Alexander Gg

2014-08-01

270

Salvage therapy with high dose Intravenous Immunoglobulins in acquired Von Willebrand Syndrome and unresponsive severe intestinal bleeding  

PubMed Central

A 91-year-old woman affected with acquired Von Willebrand (VW) syndrome and intestinal angiodysplasias presented with severe gastrointestinal bleeding (hemoglobin 5 g/dl). Despite replacement therapy with VW factor/factor VIII concentrate qid, bleeding did not stop (eleven packed red blood cell units were transfused over three days). High circulating levels of anti-VW factor immunoglobulin M were documented immunoenzimatically. Heart ultrasound showed abnormalities of the mitral and aortic valves with severe flow alterations. When intravenous immunoglobulins were added to therapy, prompt clinical and laboratory responses occurred: complete cessation of bleeding, raise in hemoglobin, VW factor antigen, VW ristocetin cofactor and factor VIII levels as well as progressive reduction of the anti-VWF autoantibody levels.

2014-01-01

271

Tuberculosis complicated by immune reconstitution inflammatory syndrome in a patient on anti-TNF? therapy for Crohn's disease  

PubMed Central

A 28-year-old man treated with the antitumour necrosis factor ? (TNF?) monoclonal antibody infliximab for Crohn's disease developed pulmonary tuberculosis (TB), despite testing negative for latent TB prior to treatment. On starting anti-TB treatment and withdrawal of the anti-TNF? therapy, he deteriorated both clinically and radiologically. He was diagnosed with a flare of Crohn's disease, and immune reconstitution inflammatory syndrome (IRIS) in his right upper lobe and mediastinal lymph nodes, and commenced on oral prednisolone. Anti-TNF? therapy was re-introduced, and prednisolone weaned, following 4 months of anti-TB treatment without complication. He made a full recovery from TB, although his Crohn's symptoms continue to be troublesome. There has been no reactivation of TB to date, after 2 years follow-up.

O'Dowd, Caroline; Kewin, Peter; Morris, John; Cotton, Mark

2011-01-01

272

The low level laser therapy in the management of neurological burning mouth syndrome. A pilot study  

PubMed Central

Summary Background and objective. Burning Mouth Syndrome (BMS) is a common disease but still a diagnostic and therapeutic challenge for clinicians. Despite many studies its nature remains obscure and controversial; nowadays there is no consensus about definition, diagnosis and classification. BMS is characterized clinically by burning sensations in the tongue or other oral sites, often without clinical and laboratory findings. According to the etiology, BMS cases should be subdivided into three subtypes: BMS by local factors (lfBMS), BMS by systemic factors (sfBMS) and neurological BMS (nBMS), the most frequent, in which the symptom is caused by central or peripheral neurological malfunctions affecting in particular the taste pathway. To establish the type of BMS, both anamnesis and clinical examination, including laboratory tests, are necessary; nBMS cases will be recognized by exclusion of any other type. In case of lfBMS or sfBMS, the treatment of the main pathology will be resolutive; in nBMS cases many Authors proposed different pharmacological trials without satisfactory results and the current opinion is that a multidisciplinary approach is required to keep the condition under control. This pilot study aimed to investigate whether the biostimulative effect of Low Level Laser Therapy (LLLT) could enhance the symptoms of nBMS cases, improving patients’ quality of life. Study design/materials and methods. Among 160 patients affected by oral burning sensation attending to the Oral Pathology Complex Operative Unit of the Department of Stomatological Sciences of Sapienza University of Rome, 77 resulted affected by nBMS. Twenty-five of these patients, 16 females and 9 males, were randomly selected for low level laser applications. All the patients were irradiated with a double diode laser (Lumix 2 Prodent, Italy) emitting contemporarily at 650 nm and 910 nm, with a fluence of 0.53 J/cm2 for 15 minutes twice a week for 4 weeks. The areas of irradiation were the sides of the tongue on the path of taste fibers. A NRS (numerical rating scale) evaluation of maximum and minimum pain was registered before and after the treatment. In each case to the total value of NRS rates registered before the treatment was deducted the total NRS rate registered after the treatment. The difference was estimated effective if over two points. The Kruskall-Wallis test revealed the significance of the study (p<0.0001) and the Dunn’s Multiple Comparison test, applied to compare NRS rates before and after the treatment, showed that there is not a statistically relevant difference between min NRS ratings before and after treatment, while there are statistically significant differences between max NRS ratings (p<0.05). Results All the patients agreed the treatment confirming the general good compliance related to laser treatments. No side effects were registered and all the patients completed the therapy without interruption. Seventeen patients (68%) had relevant benefits from the treatment with valid reduction of NRS ratings. In 8 cases the differences of NRS rates were not relevant being under the limit of reliability established in study design. In no case there was a worsening of the symptoms. Conclusions: According to the results of this pilot study it is reasonable to suppose that LLLT may play an important role in the management of nBMS cases, more investigations are needed to clarify, by a greater number of cases and a placebo control group, the real effectiveness of this innovative LLLT application.

Romeo, Umberto; Del Vecchio, Alessandro; Capocci, Mauro; Maggiore, Claudia; Ripari, Maurizio

2010-01-01

273

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

274

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

275

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

PubMed Central

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

2012-01-01

276

Duration of dual antiplatelet therapy following percutaneous coronary intervention on re-hospitalization for acute coronary syndrome  

PubMed Central

Background The optimal duration of dual antiplatelet therapy after percutaneous coronary intervention (PCI) remains uncertain. The objective of this study was to examine the association between duration of dual antiplatelet therapy and re-hospitalization for acute coronary syndrome (ACS) in ACS patients who underwent PCI. Methods We identified 975 newly diagnosed ACS patients who underwent PCI between July, 2007 and June, 2009, at a medical center in Taiwan. Cox proportional hazard models were used to examine the association between duration of dual antiplatelet therapy (9 months, 12 months and 15 months) and risks of re-hospitalization for ACS. Results At a mean follow-up of 2.3 years, we found that use of clopidogrel for???12 months was associated with a decreased risk of re-hospitalization for ACS (adjusted HR 0.59, 95% CI 0.36-0.95; p?=?0.03). However, use of clopidogrel for???15 months was not associated with a decreased risk of re-hospitalization for ACS (adjusted HR 0.57, 95% CI 0.29-1.13; p?=?0.11). Similar results were found in patients who implanted drug-eluting stents (DES), for whom at least 12 months of clopidogrel therapy is especially critical. Conclusion The benefit of???12 months of clopidogrel use in reducing the risk of re-hospitalization for ACS was significant among ACS patients who underwent PCI and was especially critical for those who implanted DES.

2014-01-01

277

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

PubMed

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

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

2013-06-27

278

[The new possibility of therapy for medical personnel with metabolic syndrome].  

PubMed

The possibility of magnesium deficiency correction was investigated in medical personnel with metabolic syndrome. It was shown that treatment with drugs containing magnesium leads to a decrease the cholesterol level. PMID:23785828

Bakumov, P A; Zerniukova, E A; Grechkina, E R

2013-01-01

279

Reflex therapy and UHF-acupuncture in the treatment of chronic fatigue syndrome  

Microsoft Academic Search

In the present-day social and economic environment a growing number of people suffer from chronic fatigue. The paper presents the results of treating the chronic fatigue syndrome (CFS) with the aid of mm waves.

N. V. Lyan; V. N. Lyan; S. D. Votoropin

2003-01-01

280

Churg-Strauss syndrome with pachymeningitis refractory to steroid therapy atone--a case report.  

PubMed

Churg-Strauss syndrome (CSS) is a rare syndrome of unknown cause, which is characterized by bronchial asthma and hypereosinophilia followed by systemic symptoms of angiitis. In 1951, Churg and Strauss first distinguished this disease from periarteritis nodosa. Our patient in particular was also complicated with otorhinological symptoms, which have less frequently been reported in CSS cases. We have detailed her clinical symptoms. PMID:11683330

Lio, M; Fukuda, S; Maguchi, S; Kawanami, M; Inuyama, Y

2001-05-01

281

Susac's syndrome: intratympanic therapy for hearing loss and a review of the literature.  

PubMed

Two cases of Susac's Syndrome are presented with a discussion of treatment modalities for the associated severe sensorineural deafness. Patients were managed with high dose oral steroids, anticoagulants and immunosuppressive drugs. The addition of intratympanic methylprednisolone injections were added to one patient's regimen with initial improvement but subsequent relapse and progression to profound deafness. The management of Susac's syndrome is difficult and still in need of innovative methods as the standard treatment continues to be ineffective in the long term. PMID:19117315

Crawley, Brianna K; Close, Abby; Canto, Cecilia; Harris, Jeffrey P

2009-01-01

282

Long-term results of growth hormone therapy in turner syndrome  

Microsoft Academic Search

Short stature is one of the main features of Turner syndrome. Today, most patients are treated with growth hormone (GH) to\\u000a improve adult height. We have reviewed the literature reporting adult height in patients with Turner syndrome treated with\\u000a GH alone or in combination with oxandrolone. The reported adult heights as well as the height gain over projected or predicted

J. H. Bramswig

2001-01-01

283

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

284

Proton beam therapy for a patient with a giant thymic carcinoid tumor and severe superior vena cava syndrome.  

PubMed

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

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

2014-05-13

285

NPHS2 R229Q Polymorphism in Steroid Resistant Nephrotic Syndrome: Is it Responsive to Immunosuppressive Therapy?  

PubMed

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

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

2014-06-01

286

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

PubMed Central

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

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

2014-01-01

287

Efficacy of a short course of complex lymphedema therapy or graduated compression stocking therapy in the treatment of post-thrombotic syndrome.  

PubMed

Treatment options for established post-thrombotic syndrome (PTS) are limited. Complex lymphedema therapy (CLT), a non-invasive treatment that improves lymphatic flow, may have the potential to improve PTS. We conducted a single-center, investigator-blind, randomized controlled trial of 31 patients with a clinically established diagnosis of PTS and compared the efficacy of graduated compression stockings alone (30-40 mmHg) with CLT, a treatment that includes compression stockings, exercise, patient education, skin care and lymphatic drainage. Primary outcomes were the 1- and 3-month changes in PTS severity by the Villalta score and disease-specific quality of life using the VEINES-QOL (Venous Insufficiency Epidemiological and Economic Study Quality of Life) questionnaire. Analysis was by intent-to-treat. We found from a baseline average score of 9.9 points, CLT reduced mean PTS severity scores by -2.4 points (p=0.02) at the 1-month and -2.3 points (p=0.05) at the 3-month follow-up. Score reductions with stockings only were similar at -2.1 (p=0.03) and -3.3 points (p=0.03) at 1 and 3 months. The differences in score between treatments were not significant. Neither treatment significantly changed the VEINES-QOL score except in patients with severe disease. Patients with moderate to severe PTS derived the greatest benefit from either therapy and the two therapies differentially impacted PTS signs and symptoms. We found a short course of lymphedema therapy and compression stockings offer similar benefit in patients with PTS; however, larger studies are needed to further explore the potential use of CLT in PTS, particularly in patients with more severe disease. ClinicalTrials.gov Identifier: NCT00633971. PMID:24558028

Holmes, Chris E; Bambace, Nadia M; Lewis, Patricia; Callas, Peter W; Cushman, Mary

2014-02-01

288

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

289

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

290

Bone scan findings of chest wall pain syndrome after stereotactic body radiation therapy: implications for the pathophysiology of the syndrome  

PubMed Central

We present a case of a 72-year-old woman treated with stereotactic body radiation therapy (SBRT) for peripherally located stage I non-small cell lung cancer (NSCLC). After treatment she developed ipsilateral grade II chest wall pain. A bone scan showed nonspecific and heterogeneous increased radiotracer uptake in the volume of ribs receiving 30% of the prescription dose of radiation (V30). We present a color wash image demonstrating excellent concordance between the V30 and the area of scintigraphic uptake on bone scan. We present a discussion of the current state of knowledge of dose volume parameters for chest wall toxicity following SBRT. This is the first case in the literature demonstrating bone scan findings corresponding to chest wall pain from SBRT. To explain these findings we propose a mechanism whereby SBRT results in bone degeneration, which prompts an increase in bone repair and an increased inflammatory state.

Lloyd, Shane; Decker, Roy H.

2013-01-01

291

Bone scan findings of chest wall pain syndrome after stereotactic body radiation therapy: implications for the pathophysiology of the syndrome.  

PubMed

We present a case of a 72-year-old woman treated with stereotactic body radiation therapy (SBRT) for peripherally located stage I non-small cell lung cancer (NSCLC). After treatment she developed ipsilateral grade II chest wall pain. A bone scan showed nonspecific and heterogeneous increased radiotracer uptake in the volume of ribs receiving 30% of the prescription dose of radiation (V30). We present a color wash image demonstrating excellent concordance between the V30 and the area of scintigraphic uptake on bone scan. We present a discussion of the current state of knowledge of dose volume parameters for chest wall toxicity following SBRT. This is the first case in the literature demonstrating bone scan findings corresponding to chest wall pain from SBRT. To explain these findings we propose a mechanism whereby SBRT results in bone degeneration, which prompts an increase in bone repair and an increased inflammatory state. PMID:23585956

Lloyd, Shane; Decker, Roy H; Evans, Suzanne B

2013-04-01

292

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

PubMed Central

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

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

2010-01-01

293

[The otilonium bromide-benzodiazepine combination in the therapy of the irritable colon syndrome].  

PubMed

The irritable bowel syndrome is classified ad "disturbance of intestinal motility without an identifiable anatomic substrate". However, the clear etiopathogenetic implications of a psychosomatic nature complicate the search for an adequate therapeutic strategy. Based on this clinical experience, we set out to check the importance of a spasmolytic with a benzodiazepine and the tolerability of this type of combination. We therefore compared the results in 60 patients with irritable bowel syndrome of 8 weeks' treatment with tablets containing octylonium bromide (OB) 20 mg plus diazepam (DZ) 2 mg or OB 40 mg + 2 mg DZ. The doubling of the spasmolytic without increasing the daily dose of anxiolytic appeared to be useful for reducing the symptoms typical for the irritable bowel syndrome. In addition, the combination was found to be perfectly tolerated. PMID:1395454

Capurso, L; Del Sette, F; Ferrario, F; Tarquini, M

1992-08-01

294

Adverse events and deterioration reported by participants in the PACE trial of therapies for chronic fatigue syndrome  

PubMed Central

Objective Adverse events (AEs) are health related events, reported by participants in clinical trials. We describe AEs in the PACE trial of treatments for chronic fatigue syndrome (CFS) and baseline characteristics associated with them. Methods AEs were recorded on three occasions over one year in 641 participants. We compared the numbers and nature of AEs between treatment arms of specialist medical care (SMC) alone, or SMC supplemented by adaptive pacing therapy (APT), cognitive behaviour therapy (CBT) or graded exercise therapy (GET). We examined associations with baseline measures by binary logistic regression analyses, and compared the proportions of participants who deteriorated by clinically important amounts. Results Serious adverse events and reactions were infrequent. Non-serious adverse events were common; the median (quartiles) number was 4 (2, 8) per participant, with no significant differences between treatments (P = .47). A greater number of NSAEs were associated with recruitment centre, and baseline physical symptom count, body mass index, and depressive disorder. Physical function deteriorated in 39 (25%) participants after APT, 15 (9%) after CBT, 18 (11%) after GET, and 28 (18%) after SMC (P < .001), with no significant differences in worsening fatigue. Conclusions The numbers of adverse events did not differ significantly between trial treatments, but physical deterioration occurred most often after APT. The reporting of non-serious adverse events may reflect the nature of the illness rather than the effect of treatments. Differences between centres suggest that both standardisation of ascertainment methods and training are important when collecting adverse event data.

Dougall, Dominic; Johnson, Anthony; Goldsmith, Kimberley; Sharpe, Michael; Angus, Brian; Chalder, Trudie; White, Peter

2014-01-01

295

The immune reconstitution inflammatory syndrome with tuberculosis: a common problem in Ethiopian HIV-infected patients beginning antiretroviral therapy.  

PubMed

The Immune Reconstitution Inflammatory Syndrome (IRIS) in Ethiopian HIV-infected patients coinfected with tuberculosis (TB) was studied. HIV-infected outpatients initiating antiretroviral therapy (ART) at an HIV clinic in northern Ethiopia from January 2007 through September 2008 were identified (n = 1977). Patients with TB-IRIS occurring within 6 months of starting ART (n = 143) were compared with a control group of patients with HIV who began ART but did not develop TB-IRIS (n = 277). ART was not interrupted in any patient. Eleven (8%) patients with TB-IRIS died. New or "unmasked" TB with accompanying IRIS occurred in 132 or 92% of the cases. Worsening or "paradoxical" TB (ie, already known to be present and treated) was accompanied by IRIS in 11 (8%) patients. There was no significant difference between "unmasked" and "paradoxical" cases with respect to presentation of disease and outcome. Only a low baseline CD4 count (mean: 102 cells/?L) and a past history of World Health Organization (WHO) Clinical Stage 3 or 4 were associated with TB-IRIS (P < .05). The clinical manifestations of TB-IRIS were diverse, requiring a high index of suspicion. For example, pleural disease occurred in 13 patients, TB lymphadenitis in 17, intracranial TB in 9 patients, and disseminated TB in 15 patients. The majority of patients (88%) responded to continuation of ART and TB therapy. Thus, TB-IRIS is common in Ethiopian patients beginning ART, occurring in 7% of patients initiating antiretroviral therapy. PMID:21521804

Ali, Kedir; Klotz, Stephen A

2012-01-01

296

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

297

A PROSPECTIVE, RANDOMIZED, PLACEBO CONTROLLED, DOUBLE-BLIND STUDY OF PELVIC ELECTROMAGNETIC THERAPY FOR THE TREATMENT OF CHRONIC PELVIC PAIN SYNDROME WITH 1 YEAR OF FOLLOWUP  

Microsoft Academic Search

Purpose:Male chronic pelvic pain syndrome is a condition of uncertain etiology and treatment is often unsatisfactory. There is evidence that the symptom complex may result from pelvic floor muscular dysfunction and\\/or neural hypersensitivity\\/inflammation. We hypothesized that the application of electromagnetic therapy may have a neuromodulating effect on pelvic floor spasm and neural hypersensitivity.

E. ROWE; C. SMITH; L. LAVERICK; J. ELKABIR; R. O’N WITHEROW; A. PATEL

2005-01-01

298

The failure of parenteral copper therapy in Menkes Kinky Hair Syndrome  

Microsoft Academic Search

In the untreated infant with Menkes Kinky Hair Syndrome, copper concentrations in brain and liver are deficient, while excessive copper accumulates in other tissues. The observed serum ceruloplasmin response after parenteral copper administration is suggestive of an impairment in the incorporation of copper into this metalloprotein. These findings, together with increased urinary copper excretion and the absence of clinical improvement,

A. D. Garnica

1984-01-01

299

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

300

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

301

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

Microsoft Academic Search

BACKGROUND: Conservative treatment remains the standard of care for treating mild to moderate carpal tunnel syndrome despite a small number of well-controlled studies and limited objective evidence to support current treatment options. There is an increasing interest in the usefulness of wrist magnetic resonance imaging could play in predicting who will benefit for various treatments. METHOD AND DESIGN: Two hundred

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

2005-01-01

302

Extreme efficacy of intravenous immunoglobulin therapy for severe burning pain in a patient with small fiber neuropathy associated with primary Sjögren's syndrome.  

PubMed

Neurological involvement occurs in approximately 20% of patients with primary Sjögren's syndrome. Although neurological symptoms can affect the peripheral nervous system and the central nervous system, the most frequent symptom is polyneuropathy. Small fiber neuropathy (SFN) is a form of painful peripheral polyneuropathy that is common in patients with diabetic neuropathy, but may also occur in toxic, infectious, or immune-mediated neuropathy. We show here a patient with Sjögren's syndrome who developed SFN and was treated with intravenous immunoglobulin (IVIG) therapy, which was immediately and extremely effective. Because of the efficacy of IVIG therapy, we propose that direct immune-mediated mechanisms may be involved in the pathogenesis of SFN complicated by Sjögren's syndrome. PMID:19458906

Wakasugi, Daisuke; Kato, Takashi; Gono, Takahisa; Ito, Eiichi; Nodera, Hiroyuki; Kawaguchi, Yasushi; Yamanaka, Hisashi; Hara, Masako

2009-01-01

303

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

304

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

Microsoft Academic Search

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

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

2004-01-01

305

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

PubMed Central

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

2013-01-01

306

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

SciTech Connect

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

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

2011-11-01

307

Temporal patterns of lipid testing and statin therapy in acute coronary syndrome patients (from the Canadian GRACE Experience).  

PubMed

Current guidelines recommend the measurement of fasting lipid profile and use of statins in all patients with acute coronary syndrome (ACS). However, the temporal trends of lipid testing and statin therapy in "real-world" patients with ACS are unclear. From January 1999 through December 2008, the prospective, multicenter, Global Registry of Acute Coronary Events (GRACE/GRACE(2)/CANRACE) enrolled 13,947 patients with ACS in Canada. We stratified the study population based on year of presentation into 3 groups (1999 to 2004, 2005 to 2006, and 2007 to 2008) and compared the use of lipid testing and use of statin therapy in hospital. Overall, 70.8% of patients underwent lipid testing and 79.4% received in-hospital statin therapy; these patients were younger and had lower GRACE risk scores (p <0.001 for the 2 comparisons) compared to those who did not. Over time there was a significant increase in rates of in-hospital statin therapy (70% in 1999 to 2004 to 84.5% in 2007 to 2008, p for trend < 0.001) but only a minor increase in rates of lipid testing (69.4% in 1999 to 2004 to 72.4% in 2007 to 2008, p for trend = 0.003). After adjusting for confounders, this increasing temporal trend remained statistically significant for statin therapy (p <0.001) but not for lipid testing. Lipid testing was independently associated with in-hospital statin use (adjusted odds ratio 1.62, 95% confidence interval 1.27 to 2.08, p <0.001). In patients who did have lipid testing, those with low-density lipoprotein cholesterol level >130 mg/dl (3.4 mmol/L) were more likely to be treated with in-hospital statins. In conclusion, there has been a significant temporal increase in the use of in-hospital statin therapy but only a minor increase in lipid testing. Lipid testing was strongly associated with in-hospital statin use. A substantial proportion of patients with ACS, especially those at higher risk, still do not receive these guideline-recommended interventions in contemporary practice. PMID:22381155

Elbarouni, Basem; Banihashemi, S Behnam; Yan, Raymond T; Welsh, Robert C; Kornder, Jan M; Wong, Graham C; Anderson, Frederick A; Spencer, Frederick A; Grondin, François R; Goodman, Shaun G; Yan, Andrew T

2012-05-15

308

[Ulna impaction syndrome therapy: decompressive surgical procedures of the head of the ulna].  

PubMed

The ulnar impaction syndrome can be defined as the impaction of the ulnar head against the triangular fibrocartilage complex and ulnar carpus. As a result of this pattern, painful degeneration of the TFCC occurs. Wrist arthroscopy offers a certain diagnostic tool. In addition, arthroscopic debridement of the TFCC shows a high success rate of 70 to 80 percent. After failed arthroscopic debridement, ulnar shortening osteotomy reduces ulnar load significantly. Malunion of the radius resulting in posttraumatic ulna-plus variance should be treated by correction osteotomy with lengthening of the radius. If arthrosis of the distal radioulnar joint is combined with ulnar impaction syndrome, the hemiresection procedure of Bowers or the method of Kapandji-Sauvé are the methods of choice. PMID:9888007

Krimmer, H; Tränkle, M; Schober, F; van Schoonhoven, J

1998-11-01

309

Corticosteroid replacement therapy in hepatoadrenal syndrome: case report with a review of the literature.  

PubMed

In liver cirrhosis, hepatoadrenal syndrome has been described recently as a progressive impairment in the adrenocortical reserve, with deficient production or action of glucocorticoids resulting in adrenal insufficiency. Data on the treatment of this syndrome are scarce. We report a case of a 60-year-old male patient referred to our hospital because of rectal bleeding and bilateral leg swelling. He complained of marked weakness, bilateral leg swelling, and shortness of breath with exertion for the last 2 months. Biochemistry and imaging indicated liver cirrhosis. Because of the weakness and persistent hypotension, we performed a low-dose synacthen test, which indicated adrenal insufficiency (baseline cortisol level 1.8 ?g/dl, increasing to 3.5 and 3.7 ?g/dl at 20 and 30 min, respectively). The patient's general condition improved promptly after corticosteroid supplementation. PMID:22850193

Tomaselli, Tania; Privitera, Graziella; Fede, Giuseppe; Spadaro, Luisa; Scicali, Roberto; Di Vita, Rossella; Compagnone, Domenico; Purrello, Francesco

2012-11-01

310

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

311

Inhaled Ribavirin Therapy in Adult Respiratory Syncytial Virus-Induced Acute Respiratory Distress Syndrome  

Microsoft Academic Search

Respiratory syncytial virus (RSV) infection-induced acute respiratory distress syndrome (ARDS) in previously healthy adults is rare, but the overall mortality rate is 40%-60%. Inhaled ribavirin is approved for the treatment of hospitalized infants and young children with severe lower respiratory tract infections due to RSV. We present the case of an adult female with RSV pneumonia-induced ARDS who was successfully

Yung-Hung Luo; Chu-Yun Huang; Kuang-Yao Yang; Yu-Chin Lee

2011-01-01

312

Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome  

Microsoft Academic Search

This guideline for the investigation and initial treatment of atypical hemolytic uremic syndrome (HUS) is intended to offer\\u000a an approach based on opinion, as evidence is lacking. It builds on the current ability to identify the etiology of specific\\u000a diagnostic sub-groups of HUS. HUS in children is mostly due to infection, enterohemorrhagic Escherichia coli (EHEC), Shigella dysenteriae type 1 in

Gema Ariceta; Nesrin Besbas; Sally Johnson; Diana Karpman; Daniel Landau; Christoph Licht; Chantal Loirat; Carmine Pecoraro; C. Mark Taylor; Nicole Van de Kar; Johan VandeWalle; Lothar B. Zimmerhackl

2009-01-01

313

[The application of combined physical therapy for the treatment of women with pelvic pain syndrome].  

PubMed

A total of 98 women presenting with pelvic pain syndrome of different etiology (inflammatory diseases of small pelvic organs following surgical interventions, dysmenorrhoea, ovulatory syndrome) were enrolled in this study with an object of estimating the efficacy of the combined central and local application of physiotherapeutic techniques. An AVIM-1 apparatus was used to cause vibration in and apply a magnetic field to the perineal region. Transcranial magnetotherapy and electrostimulation were applied centrally using an AMO-ATOS-E device. The study has demonstrated that the combined central and local application of physiotherapeutic techniques supplemented by medicamental treatment according to the indications made it possible to attenuate pain syndrome by 64.6% and improve the vegetative nervous function and cerebral blood circulation by 30% and 18% respectively compared with the women in the control group. Moreover, the 1.5-2-fold improvement of the main characteristics of microcirculation was achieved. The overall resistance increased under the influence of physiotherapy in 71% of the women which promoted their reproductive potential and enhanced the probability of pregnancy. PMID:22403954

Tkachenko, L V; Ra?gorodski?, Iu M; Tarasenko, Iu N; Tikhaeva, K Iu; Kurushina, O V

2011-01-01

314

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

PubMed Central

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

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

2014-01-01

315

[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

2013-01-01

316

Myelodysplastic Syndromes  

MedlinePLUS

... Pinpoint spots under the skin caused by bleeding Fever or frequent infections Myelodysplastic syndromes are rare. People at higher risk are over 60, have had chemotherapy or radiation therapy, or have been exposed to certain chemicals. Treatment ...

317

The clinical importance of moderate/severe bone marrow fibrosis in patients with therapy-related myelodysplastic syndromes.  

PubMed

The presence of moderate to severe bone marrow (BM) fibrosis has been shown to be an adverse feature in patients with primary myelodysplastic syndromes (MDS). However, the clinical importance of BM fibrosis is not clear in therapy-related MDS. We retrieved all therapy-related MDS (t-MDS) cases (n = 266) diagnosed at our hospital over a 10-year period (2003-2012). Reticulin and trichrome stains were performed in cases in which BM fibrosis was suspected on initial evaluation of hematoxylin and eosin-stained slide. BM fibrosis was graded according to European consensus guidelines, and a score of MF2/MF3 was defined as moderate/severe fibrosis. Moderate/severe BM fibrosis was found in 47 (17%) patients. Compared to 219 patients with no/mild BM fibrosis, the patients with moderate/severe fibrosis presented with severer thrombocytopenia (p = 0.039) and higher numbers of circulating blasts (p = 0.051) but with similar degrees of anemia and neutropenia, transfusion requirements, and similar incidences of hepatosplenomegaly and constitutional symptoms. Histological examination revealed a comparable BM cellularity and BM blast percentage, but markedly increased megakaryocytes (p < 0.001) in the fibrotic group. Although the risk distribution of cytogenetic data was similar according to the New Comprehensive Cytogenetic Scoring criteria, -5 and -17 were more frequently observed in t-MDS with moderate/severe BM fibrosis (p = 0.031 and p = 0.043, respectively). With a median follow-up of 11.5 months, patients with moderate/severe BM fibrosis showed a similar risk of acute myeloid leukemia transformation and a comparable overall survival in univariate and multivariate analyses. Moderate/severe BM fibrosis in patients with t-MDS is associated with certain clinicopathological and genetic features. However, unlike the situation in patients with primary MDS, moderate/severe BM fibrosis does not add additional risk to patients with therapy-related MDS. PMID:23660629

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

2013-10-01

318

Reduction in glutamine/glutamate levels in the cerebral cortex after adrenocorticotropic hormone therapy in patients with west syndrome.  

PubMed

West syndrome (WS), an intractable epileptic encephalopathy of infancy, is refractory to many antiepileptic drugs; however, adrenocorticotropic hormone (ACTH) is an effective treatment for WS. The mechanism behind the efficacy of ACTH is mediated by biochemical processes that remain unknown. We examined the effects of ACTH therapy with tetracosactide (TCS), a synthetic ACTH analogue, on brain metabolism in patients with WS, using (1)H magnetic resonance spectroscopy ((1)H-MRS). In six patients with cryptogenic WS, we performed single-voxel (1)H-MRS at the occipital lobe cortex. Measurements were taken prior to TCS treatment, a few days after therapy, and several months after therapy. Data were also compared with subjects having only mild psychomotor delays. The metabolites measured were glutamine plus glutamate (Glx), N-acetylaspartate (NAA), choline (Cho), and myoinositol (mI); each was expressed as a ratio with creatine plus phosphocreatine (total creatine: tCr). The Glx/tCr ratio was significantly reduced after the TCS treatment. The NAA/tCr ratio was also significantly reduced after the treatment compared with the control group, although the change in NAA signal was heterogeneous among patients, correlating with respective outcomes. The Cho/tCr and mI/tCr ratios were not affected by TCS treatment. The reduction in Glx suggests a decrease in the glutamate-glutamine cycle, which plays a pivotal role in synthesizing neurotransmitters such as glutamate and GABA. TCS-induced Glx reduction may induce changes in synaptic signal transduction, thereby accounting for the effect of TCS on WS. The change in NAA indicates altered neuronal activity, which may be correlated with outcome in WS patients. PMID:24705707

Munakata, Mitsutoshi; Togashi, Noriko; Sakamoto, Osamu; Haginoya, Kazuhiro; Kobayashi, Yasuko; Onuma, Akira; Iinuma, Kazuie; Kure, Shigeo

2014-01-01

319

Recognition of a dopamine replacement therapy dependence syndrome in Parkinson's disease: a pilot study  

Microsoft Academic Search

Patients with Parkinson's disease may use Dopamine Replacement Therapy (DRT) in excess of therapeutic need. We investigate whether a group of 10 patients with Parkinson's disease, provisionally diagnosed with “Hedonistic Homeostatic Dysregulation” because of their excessive use of DRT, met established operational psychiatric criteria for substance dependence, compared with 10 patients with Parkinson's disease compliant with prescribed DRT. Using a

Jennifer Bearn; Andrew Evans; Michael Kelleher; Kirsten Turner; Andrew Lees

2004-01-01

320

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

321

Growth Hormone Therapy of Turner Syndrome: The Impact of Age of Estrogen Replacement on Final Height  

Microsoft Academic Search

Clinical trials of recombinant human GH therapy in Turner syn- drome that began more than a decade ago show that GH accelerates the linear growth rate. Several studies indicate that final height is also improved, although the magnitude of the increase has been debated. The age at which feminization is induced could be an im- portant factor in determining the

STEVEN D. CHERNAUSEK; KENNETH M. ATTIE; JOSE F. CARA; RON G. ROSENFELD; JAMES FRANE

322

[Metabolic therapy with Actovegin in case of ischemic syndrome of limbs].  

PubMed

Actovegin was used in complex conservative and surgical treatment of lower limb chronic ischemia and diabetic foot syndrome in 200 patients. In 120 patients Actovegin was used in combination with reconstructive operations, angioplasty and stenting for preparation for surgery. In 80 patients Actovegin was used as independent method of treatment. In severe cases the medicine was injected intravenously 1000-2000 mg ?10-15, and in mild degrees (IIA-IIB) - 400-800 mg intramuscularly with conversion on the tablet form in the future. Clinical picture, dopplerographic and microcirculatory data evidence about absolute safety, efficiency and comparative cheapness of the parenteral and enteral forms of Actovegine. PMID:24736538

2014-01-01

323

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

2013-07-01

324

Crescentic post-streptococcal glomerulonephritis with nephrotic syndrome in the adult: is aggressive therapy warranted?  

PubMed

The prognosis for adults with acute post-streptococcal glomerulonephritis (PSGN) who present with crescentic glomerulonephritis and nephrotic proteinuria is not known. We report a patient with rapidly progressive glomerulonephritis and nephrotic-range proteinuria following acute pharyngitis, in whom serologic and kidney biopsy findings led to a diagnosis of PSGN. The patient was treated with corticosteroids and anti-hypertensive medications resulting in improvement in renal function and decrease in proteinuria. These results suggest that aggressive treatment of crescentic PSGN with nephrotic syndrome can result in a favorable outcome. PMID:15909597

Raff, A; Hebert, T; Pullman, J; Coco, M

2005-05-01

325

Linaclotide: a novel therapy for chronic constipation and constipation-predominant irritable bowel syndrome.  

PubMed

Chronic constipation and irritable bowel syndrome (IBS) are functional gastrointestinal disorders that significantly affect patients' quality of life. Chronic constipation and IBS are prevalent-1 2% of the US population meet the diagnostic criteria for IBS, and 1 5% meet the criteria for chronic constipation- and these conditions negatively impact the healthcare system from an economic perspective. Despite attempts at dietary modification, exercise, or use of over-the-counter medications, many patients have persistent symptoms. Alternative treatment options are limited. This article describes linaclotide (Linzess, Ironwood Pharmaceuticals/Forest Pharmaceuticals), a new, first-in-class medication for the treatment of chronic constipation and constipation-predominant IBS. PMID:24683372

Lacy, Brian E; Levenick, John M; Crowell, Michael D

2012-10-01

326

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

327

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

PubMed Central

Background Oral anticoagulation in addition to antiplatelet treatment after an acute coronary syndrome might reduce ischaemic events but increase bleeding risk. We performed a meta-analysis to evaluate the efficacy and safety of adding direct thrombin or factor-Xa inhibition by any of the novel oral anticoagulants (apixaban, dabigatran, darexaban, rivaroxaban, and ximelagatran) to single (aspirin) or dual (aspirin and clopidogrel) antiplatelet therapy in this setting. Methods and results All seven published randomized, placebo-controlled phase II and III studies of novel oral anticoagulants in acute coronary syndromes were included. The database consisted of 30 866 patients, 4135 (13.4%) on single, and 26 731 (86.6%) on dual antiplatelet therapy, with a non-ST- or ST-elevation acute coronary syndrome within the last 7–14 days. We defined major adverse cardiovascular events (MACEs) as the composite of all-cause mortality, myocardial infarction, or stroke; and clinically significant bleeding as the composite of major and non-major bleeding requiring medical attention according to the study definitions. When compared with aspirin alone the combination of an oral anticoagulant and aspirin reduced the incidence of MACE [hazard ratio (HR) and 95% confidence interval 0.70; 0.59–0.84], but increased clinically significant bleeding (HR: 1.79; 1.54–2.09). Compared with dual antiplatelet therapy with aspirin and clopidogrel, adding an oral anticoagulant decreased the incidence of MACE modestly (HR: 0.87; 0.80–0.95), but more than doubled the bleeding (HR: 2.34; 2.06–2.66). Heterogeneity between studies was low, and results were similar when restricting the analysis to phase III studies. Conclusion In patients with a recent acute coronary syndrome, the addition of a new oral anticoagulant to antiplatelet therapy results in a modest reduction in cardiovascular events but a substantial increase in bleeding, most pronounced when new oral anticoagulants are combined with dual antiplatelet therapy.

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

2013-01-01

328

Alzheimer's Disease and Down Syndrome  

MedlinePLUS

... Toddlers & Young Children Speech & Language Therapy for Children & Adolescents with Down Syndrome Toilet Training Children with Down Syndrome Wellness Nutrition Healthy Eating Habits in Children with Down Syndrome Weight Management for ...

329

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

PubMed

BMS is a common condition characterized by chronic oral mucosal pain condition and primarily affects elderly women. Although clonazepam therapy has been widely used due to its efficacy, it is not always effective because of the complexity of BMS pathogenesis. In this study, we have investigated outcome predictors of clonazepam therapy in patients with BMS. One hundred patients with BMS (7 men and 93 women, mean age 58.5 ± 10.8 years) were instructed to take 0.5mg of clonazepam once or twice daily for 4 weeks. The patients were sub-grouped according to psychological status, salivary flow rate, presence of psychiatric medications, symptom area and duration, symptom severity, presence of oral parafunctions, and accompanying oral complaints. The changes in symptoms were analyzed and compared between the sub-groups. Subjects with T-scores ?50 for each psychological symptom dimension, a greater degree of initial symptoms (visual analog scale (VAS)?5), and accompanying oral complaints, such as xerostomia and taste disturbance, displayed greater decreases in symptoms compared with their counterparts. In conclusion, psychological status, initial symptom severity, and the presence of xerostomia and/or taste disturbance can serve as outcome predictors of clonazepam therapy for patients with BMS. PMID:22040716

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

2012-01-01

330

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

PubMed Central

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

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

2011-01-01

331

Recurrent strokes under anticoagulation therapy: Sticky platelet syndrome combined with a patent foramen ovale  

PubMed Central

The sticky platelet syndrome (SPS) is a congenital disorder characterized by platelet hyperaggregability to epinephrine and/or adenosine diphosphate; this predisposes affected individuals to acute myocardial infarction, ischemic optic neuropathy, recurrent venous thromboembolism, and transient ischemic cerebral attacks and strokes. Here, we describe an unusual case with recurrent cerebrovascular accidents due to SPS, in the presence of a patent foramen ovale (PFO). We report an unusual case of a 56-year-old female patient with a PFO, who suffered from recurrent strokes despite long-term medication with clopidogrel for SPS. The patient underwent successful transcatheter closure of the PFO, and, in addition, she has been placed on low-dose acetylsalicylic acid. After 18-month follow-up, she demonstrated an intact atrial septum without any vegetations on the percutaneous device until today. She has had no further thromboembolic events.

Gehoff, A.; Kluge, J. G.; Gehoff, P.; Jurisch, D.; Pfeifer, D.; Hinz, J.; Popov, A. F.

2011-01-01

332

Recurrent strokes under anticoagulation therapy: Sticky platelet syndrome combined with a patent foramen ovale.  

PubMed

The sticky platelet syndrome (SPS) is a congenital disorder characterized by platelet hyperaggregability to epinephrine and/or adenosine diphosphate; this predisposes affected individuals to acute myocardial infarction, ischemic optic neuropathy, recurrent venous thromboembolism, and transient ischemic cerebral attacks and strokes. Here, we describe an unusual case with recurrent cerebrovascular accidents due to SPS, in the presence of a patent foramen ovale (PFO). We report an unusual case of a 56-year-old female patient with a PFO, who suffered from recurrent strokes despite long-term medication with clopidogrel for SPS. The patient underwent successful transcatheter closure of the PFO, and, in addition, she has been placed on low-dose acetylsalicylic acid. After 18-month follow-up, she demonstrated an intact atrial septum without any vegetations on the percutaneous device until today. She has had no further thromboembolic events. PMID:21716756

Gehoff, A; Kluge, J G; Gehoff, P; Jurisch, D; Pfeifer, D; Hinz, J; Popov, A F

2011-01-01

333

Hemispheric integrative therapy in Landau-Kleffner syndrome: applications for rehabilitation sciences.  

PubMed

A case study is presented of a 14-year-old right-handed Caucasian female diagnosed with the Landau-Kleffner Syndrome (LKS) at the age 3 1/2 years. Her LKS symptoms presented with abrupt disruption in language after normal development, electroencephalogram (EEG) brain-wave abnormality, seizure activity, inability to read, and impairment in her motor skills. After 11 years of pharmacological and special education interventions with no significant improvement in any measurable area of function, a multimodal approach using techniques purportedly aimed at facilitating inter-hemispheric communication was provided. At completion of the program, EEG was controlled, reading, language, and auditory processing improved and objective behavioral-social measures improved significantly. PMID:16040364

Pedro, Victor M; Leisman, Gerry

2005-08-01

334

[The low FODMAP diet as a therapy for irritable bowel syndrome].  

PubMed

Irritable bowel syndrome (IBS) is a very common, sometimes disabling disorder for which there is a lack of effective treatments. Many patients report that the make-up of the diet has an effect on their symptoms. The western diet consists of many different molecules. Some of them are small, fermentable and cannot be absorbed (FODMAPs). The low FODMAP diet was recently developed in Australia. In randomised controlled trials 37-45% of IBS patients experience symptom reduction with this diet. The low FODMAP diet starts with a 6-week elimination phase. If symptoms decrease substantially, it is followed by a structured reintroduction phase of the various FODMAPs over several weeks, after which patients are left with a diet that is simpler to follow. PMID:24823855

van der Waaij, Laurens A; Stevens, Janneke

2014-01-01

335

Effect of chronic continuous positive airway pressure (CPAP) therapy on upper airway size in patients with sleep apnoea/hypopnoea syndrome.  

PubMed Central

BACKGROUND: There is evidence to suggest that chronic continuous positive airway pressure (CPAP) therapy may produce reversible changes in upper airway morphology and function in patients with sleep apnoea/hypopnoea. This study was designed to examine the effect of chronic CPAP therapy on upper airway calibre. METHODS: Twenty four men with the sleep apnoea/hypopnoea syndrome (mean (SE) apnoea/hypopnoea index 37 (5)) underwent lateral cephalometry with measurement of posterior airway space performed before and at least three months after initiation of CPAP therapy. RESULTS: There was no weight change between the two assessments and mean CPAP use was 4.8 (0.4) hours per night. Posterior airway space (PAS) was measured in erect and supine postures. PAS supine increased with CPAP therapy from a mean (SE) of 11.8 (0.8) mm to 13.4 (0.8) mm, but PAS erect did not. Correlation of the change in PAS (dPAS) before and after CPAP therapy showed an increase with increasing CPAP compliance measured as machine run time both for dPAS supine (r = 0.68) and dPAS erect (r = 0.47). CONCLUSIONS: Patients with the sleep apnoea/hypopnoea syndrome regularly using CPAP for more than four hours per night all showed an increase in dPAS supine. The use of chronic CPAP increases PAS supine probably by a reduction in upper airway oedema, and the change in size is dependent on CPAP use.

Mortimore, I. L.; Kochhar, P.; Douglas, N. J.

1996-01-01

336

Transient effect of anti-CD20 therapy in a child with 22q11.2 deletion syndrome and severe steroid refractory cytopenias: a case report.  

PubMed

We report on the development of steroid-refractory recurrent cytopenias in a child with 22q11.2 deletion syndrome. His first hematological complication was autoimmune hemolytic anemia at 3 months of age. Thereafter, he developed severe autoimmune cytopenias of all 3 hematological lineages with poor response to steroids and intravenous immunoglobulin. At the age of 2½ years, a course of anti-CD20 therapy (Rituximab) was given with transient hematological recovery. Because of persistent symptoms, bone marrow transplantation from a matched unrelated donor was performed. Although the data in the use of anti-CD20 therapy in children with 22q11.2 deletion syndrome and autoimmune cytopenias are limited, our experience suggests its potential benefit. PMID:23612383

Soldatou, Alexandra; Anastassiou, Theodora; Vougiouka, Olga; Goussetis, Evgenios; Kossiva, Lydia

2013-05-01

337

Guillain Barre syndrome in an HIV-1-infected patient after the beginning of combined antiretroviral therapy: an immune reconstitution inflammatory syndrome?  

PubMed

HIV-1-associated Guillan-Barre syndrome (hGBS) is an ascendant progressive polyradiculoneuropathy described throughout the course of the viral disease, mainly associated with the acute retroviral syndrome. HGBS is occasionally described in severely immunocompromised subjects in the context of the immune reconstitution inflammatory syndrome. The case described occurred soon after the start of a combined antiretroviral treatment in an HIV-1 infected patient with ulcerative colitis in the absence of severe immunosuppression. This manifestation may be interpreted as an uncommon appearance of an immune reconstitution syndrome in the presence of a predisposing autoimmune pathology. PMID:24531178

Fantauzzi, Alessandra; Digiulio, Maria Anna; Cavallari, Eugenio Nelson; d'Ettorre, Gabriella; Vullo, Vincenzo; Mezzaroma, Ivano

2014-01-01

338

Recurrent spleen enlargement during cyclic granulocyte-macrophage colony-stimulating factor therapy for myelodysplastic syndrome  

SciTech Connect

A 65-year-old woman with refractory anemia with excess of blasts received sequential courses of granulocyte-macrophage colony-stimulating factor therapy (GM-CSF) and low-dose cytosine arabinoside. Each course of GM-CSF induced a rapid and tremendous increase in leukocyte count as well as in spleen size, 111-indium chloride scanning suggested a myeloid metaplasia of the spleen. This observation suggests that in some patients the granulopoietic response to the myeloid growth factor stimulation may be predominant in the spleen.

Delmer, A.; Karmochkine, M.; Cadiou, M.; Gerhartz, H.; Zittoun, R. (Service d'Hematologie, Paris (France))

1990-05-01

339

Improved multilineage response of hematopoiesis in patients with myelodysplastic syndromes to a combination therapy with all- trans -retinoic acid, granulocyte colony-stimulating factor, erythropoietin and ?-tocopherol  

Microsoft Academic Search

Differentiation induction therapy is being tested in myelodysplastic syndromes to ameliorate maturation defects and to restore\\u000a normal hematopoietic function. To this end, 17 patients (eight with refractory anemia, two with refractory anemia and ring\\u000a sideroblasts, and seven with refractory anemia and excess of blast cells) were treated with a combination of all-trans-retinoic acid (ATRA), granulocyte colony-stimulating factor (G-CSF), erythropoietin (EPO),

A. Ganser; A. Maurer; C. Contzen; G. Seipelt; O. G. Ottmann; C. Schadeck-Gressel; K. Kolbe; R. Haas; C. Zander; R. Reutzel; D. Hoelzer

1996-01-01

340

Air-leak syndrome following allo-SCT in adult patients: report from the Kanto Study Group for Cell Therapy in Japan  

Microsoft Academic Search

We retrospectively investigated air-leak syndrome (ALS), including pneumothorax and mediastinal\\/s.c. emphysema, following allogeneic hematopoietic SCT. Eighteen patients (1.2%) developed ALS among 1515 undergoing SCT between 1994 and 2005 at the nine hospitals participating in the Kanto Study Group on Cell Therapy. The median onset of ALS was at 575 days (range: 105–1766) after SCT and 14 patients (77.8%) had experienced

R Sakai; H Kanamori; C Nakaseko; F Yoshiba; K Fujimaki; T Sakura; S Fujisawa; N Kawai; M Onoda; T Matsushima; A Maruta; H Sakamaki; S Okamoto

2011-01-01

341

The Effect of an Occupational Therapy Role-Playing Intervention on the Social Skills of Adolescents With Asperger's Syndrome: A Pilot Study  

Microsoft Academic Search

The purpose of the authors in this pilot study was to assess the effects of an occupational therapy role-playing intervention for adolescents with Asperger's syndrome. An ABA multiple baseline single-subject design across participants was used with three adolescents to determine if a six-week intervention could increase targeted social behaviors. Data were collected over baseline, intervention, and probe phases. Three methods

Sharon A. Gutman; Emily I. Raphael-Greenfield; Sabrina Salvant

2012-01-01

342

Ovulation induction in women with polycystic ovary syndrome: randomized trial of clomiphene citrate versus low-dose recombinant FSH as first line therapy  

Microsoft Academic Search

This single centre randomized controlled trial was undertaken to compare the efficacy and safety of clomiphene citrate and lowdose recombinant FSH as first line pharmacological therapy for anovulatory infertility associated with polycystic ovary syndrome (PCOS). Seventy-six infertile patients with PCOS were randomized to receive clomiphene citrate (50–150 mg\\/day for 5 days) (clomiphene citrate group, n = 38) or recombinant human

Eugenio López; Gunby Joanne; Salim Daya; Juan J Parrilla; Lorenzo Abad; Juan Balasch

2004-01-01

343

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

344

The Prevalence of Hypomagnesaemia in Pediatric Patients with Mitral Valve Prolapse Syndrome and the Effect of Mg Therapy  

PubMed Central

Background A paucity of data exists regarding the prevalence and relationship of hypomagnesaemia with clinical symptoms of mitral valve prolapse (MVP) in pediatric patients. Objective In this study we evaluated the prevalence of magnesium (Mg) deficiency in pediatric patients with MVP syndrome and attempted to clarify the effect of Mg therapy on alleviating their symptoms. Methods The present study was conducted from April 2010 to January 2012, and included 230 patients (90 males and 140 females) with symptoms of mitral valve prolapse and mean age of 11.6±3.66. Serum magnesium (Mg) level less than 1.5 mg/dl was defined as hypomagnesaemia. Patients with 2 mm leaflet displacement and maximum leaflet thickness of 5 mm in echocardiography were considered to have classic MVP, while those with leaflet thickness less than 5 mm were considered as non-classic MVP. Patients with hypomagnesaemia were orally treated with 4.5 mg/kg/day Mg chloride for 5 weeks followed by re-evaluation of symptoms of chest pain, palpitation, fatigue and dyspnea. Results Hypomagnesaemia was found in 19 (8.2 %) of 230 patients with mitral valve prolapse. The re-evaluation of patients with Hypomagnesaemia after 5 weeks of Mg therapy, showed statistically significant relief of chest pain (P=0.01). However, no significant changes was detected in regard to palpitation (P=0.06), fatigue (P= 0.5) and dyspnea (P=0.99). Conclusion This study revealed that the prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse is relatively low compared to adults, but treatment with oral Mg in patient with hypomagnesaemia decreases chest pain.

Amoozgar, Hamid; Rafizadeh, Hashem; Ajami, Gholamhossein; Borzoee, Mohammad

2012-01-01

345

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

346

The effect of vitamin D replacement therapy on insulin resistance and androgen levels in women with polycystic ovary syndrome.  

PubMed

Insulin resistance (IR) is one of the common features of the polycystic ovary syndrome (PCOS), and recent studies indicate the possible role of vitamin D in the pathogenesis of IR and glucose metabolism. Aim of this study was aimed to determine the effect of vitamin D replacement therapy on glucose metabolism, insulin, and androgen levels in obese, insulin-resistant women with PCOS. Eleven women with PCOS were included in the study. Mean age of the patients was 23.6+/-5.7 yr, body mass index 33.9+/-5.1 kg/m(2). Six patients (54.5%) had acantosis nigricans and 10 (90.9%) oligoamenorrhea. The mean Ferriman Gallwey score was 14.1+/-4.6. Only 2 women were within the normal limits of vitamin D levels as >20 ng/ml. Three weeks after the administration of the single dose of 300,000 units of vitamin D3 orally, 25-hydroxyvitamin D3 significantly increased from 16.9+/-16 ng/ml to 37.1+/-14.6 ng/ml (p: 0.027) and only 2 women were detected to have vitamin D3 levels <20 ng/ml. Although glucose and insulin levels were decreased non-significantly, homeostasis model assessment (HOMA)-IR significantly decreased from 4.41+/-1.38 to 3.67+/-1.48 (p: 0.043). No significant alterations were witnessed at the levels of DHEAS, total and free testosterone, androstenedione. No correlation was found between vitamin D with HOMA and other hormonal parameters. In conclusion, women with PCOS have mostly insufficient vitamin D levels, and vitamin D replacement therapy may have a beneficial effect on IR in obese women with PCOS. PMID:19820295

Selimoglu, H; Duran, C; Kiyici, S; Ersoy, C; Guclu, M; Ozkaya, G; Tuncel, E; Erturk, E; Imamoglu, S

2010-04-01

347

Effect of Combination Therapy on Cardiovascular Risk in the Pit Miners with Hypertension, Metabolic Syndrome and Depression  

PubMed Central

Background: Higher cardiovascular (CV) morbidity and mortality in depressed patient has demonstrated repeatedly. Aim: Determine the degree of occurrence of hypertension, metabolic syndrome (MetS) and depression in the pit miners and the impact of the six-month holistic treatment of all CV risk factors and depression in the overall CV risk in miners with hypertension, MetS and depression. Methods: From 492 pit miners was taken smoking status, measured blood pressure, waist circumference and body mass index. Analysis was done using the concentration of sugar in blood, triglycerides, total cholesterol, HDL, LDL cholesterol and determined total CV risk. All respondents filled self-assessment Beck's depression scale. Prevalence MetS hypertension and depression were determined. Group of 67 patients with joint hypertension, MetS and depression that is treated six months with psychotropic and somatotropin medication, was singled out. After six months, the effect of therapy on the risk factors and total CV was assessed. Results: Among 492 miners 67 (13,61%) of them had hypertension, MetS and depression. After six months treatment, it showed statistically significant reduction in blood pressure (p=0,0001), waist circumference (p=0,0001) ,total (p=0,002), HDL (p=0,007) and LDL cholesterol (p=0,003), smoking (p=0,002), Beck's scale results (p=0,007) and reduction in total CV risk. Conclusion: After six month of combine therapy in respondents has led to reduced CV risk and level of all factors, except BMI and triglycerides.

Becarevic, Munevera; Barakovic, Fahir; Batic-Mujanovic, Olivera; Beganlic, Azijada

2014-01-01

348

Domaines d’application et enjeux de l’ingénierie et de la thérapie cellulaires et tissulaires  

Microsoft Academic Search

A new medical field, known as regeneration medicine is developing and attracting more and more researchers and practitioners. Whereas hematopoietic cell-based therapies have already proven their efficacy in numerous – malignant or not – diseases, non-hematopoietic cell-based therapies have not. They can be useful to dozens, if not hundreds, of patients with various disorders, such as cardiopathy, diabetes, some types

Patrick Hervé

2007-01-01

349

National Down Syndrome Society  

MedlinePLUS

... for Spanish speakers, including About Down Syndrome, Healthcare, Early Intervention, Therapies & Development, Education and more! Read More » My ... thinking about people with Down syndrome. Read More » Early Intervention The first years of life are a critical ...

350

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

351

Prone Position Ventilation Used during a Transfer as a Bridge to Ecmo Therapy in Hantavirus-Induced Severe Cardiopulmonary Syndrome  

PubMed Central

Background. Transport of critically ill patients is a complex issue. We present a case using prone positioning as a bridge to extracorporeal membrane oxygenation (ECMO), performed by a critical retrieval team from a university hospital. Case Report. A 28-year-old male developed fever, progressive respiratory failure, and shock. He was admitted to ICU from a public hospital, and mechanical ventilation was begun, but clinical response was not adequate. ECMO was deemed necessary due to severe respiratory failure and severe shock. A critical retrieval team of our center was assembled to attempt transfer. Prone positioning was employed to stabilize and transfer the patient, after risk-benefit assessment. Once in our hospital, ECMO was useful to resolve shock and pulmonary edema secondary to Hantavirus cardiopulmonary syndrome. Finally, he was discharged with normal functioning. Conclusion. This case exemplifies the relevance of a retrieval team and bridge therapy. Prone positioning improves oxygenation and is safe to perform as transport if performed by a trained team as in this case. Preparation and organization is necessary to improve outcomes, using teams and organized networks. Catastrophic respiratory failure and shock should not be contraindications to transferring patients, but it must be done with an experienced team.

Cornejo, R.; Ugalde, D.; Llanos, O.; Bisbal, P.; De la Barrera, L.; Romero, C.; Neira, R.; Gonzalez, Roberto; Gajardo, J.

2013-01-01

352

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

PubMed Central

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

2011-01-01

353

Prone Position Ventilation Used during a Transfer as a Bridge to Ecmo Therapy in Hantavirus-Induced Severe Cardiopulmonary Syndrome.  

PubMed

Background. Transport of critically ill patients is a complex issue. We present a case using prone positioning as a bridge to extracorporeal membrane oxygenation (ECMO), performed by a critical retrieval team from a university hospital. Case Report. A 28-year-old male developed fever, progressive respiratory failure, and shock. He was admitted to ICU from a public hospital, and mechanical ventilation was begun, but clinical response was not adequate. ECMO was deemed necessary due to severe respiratory failure and severe shock. A critical retrieval team of our center was assembled to attempt transfer. Prone positioning was employed to stabilize and transfer the patient, after risk-benefit assessment. Once in our hospital, ECMO was useful to resolve shock and pulmonary edema secondary to Hantavirus cardiopulmonary syndrome. Finally, he was discharged with normal functioning. Conclusion. This case exemplifies the relevance of a retrieval team and bridge therapy. Prone positioning improves oxygenation and is safe to perform as transport if performed by a trained team as in this case. Preparation and organization is necessary to improve outcomes, using teams and organized networks. Catastrophic respiratory failure and shock should not be contraindications to transferring patients, but it must be done with an experienced team. PMID:24829824

Cornejo, R; Ugalde, D; Llanos, O; Bisbal, P; De la Barrera, L; Romero, C; Neira, R; González, Roberto; Gajardo, J

2013-01-01

354

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

PubMed

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

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

2014-05-29

355

Pulsed vs. CW low level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome)  

NASA Astrophysics Data System (ADS)

Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias. The transforming growth factor beta (TGF-?) has been identified has a major player in the pathogenic process, while low level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940nm using microsecond domain pulsing and continuous wave mode (CW) on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks, using a sequential pulsing mode on one elbow, and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, and health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Significant functional and morphologic improvements were observed after LLLT, with best results seen with the pulsing mode. No significant adverse effects were noted. Two mechanisms of action may be at play. The 940nm wavelength provides inside-out heating possibly vasodilating capillaries which in turn increases catabolic processes leading to a reduction of in situ calcinosis. LLLT may also improve symptoms by triggering a cascade of cellular reactions, including the modulation of inflammatory mediators.

Barolet, Daniel

2012-02-01

356

A combined intravesical therapy with hyaluronic acid and chondroitin for refractory painful bladder syndrome/interstitial cystitis.  

PubMed

The aims of this study were to evaluate the efficacy and tolerability of intravesical instillations of high-molecular-weight hyaluronic acid (HA) 1.6% and chondroitin sulfate (CS) 2.0% in patients with refractory painful bladder syndrome/interstitial cystitis (PBS/IC) and to observe their impact on Quality of Life. Twenty-three women were enrolled. They received bladder instillations with HA and CS weekly for 20 weeks and then monthly for 3 months. Mean follow-up after completion of therapy was 5 months. We observed a significant improvement in urinary symptoms on voiding diaries and Visual Analogue Scale for frequency (p = 0.045), urgency (p = 0.005), and pain (p = 0.001). The O'Leary-Sant Interstitial Cystitis Symptom Index and Interstitial Cystitis Problem Index resulted in a significant improvement in both scores (p = 0.004 and 0.01, respectively). The Pelvic Pain and Urgency/Frequency Symptom Scale only showed significant improvement in the symptom score (p = 0.001). This promising experience seems to offer an additional therapeutic option in patients with refractory PBS/IC. PMID:18338095

Cervigni, M; Natale, F; Nasta, L; Padoa, A; Voi, R Lo; Porru, D

2008-07-01

357

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

PubMed Central

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

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

2014-01-01

358

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

2011-01-01

359

Implications of melatonin therapy in irritable bowel syndrome: a systematic review.  

PubMed

Irritable bowel syndrome (IBS) is a highly prevalent chronic functional gastrointestinal (GI) disorder associated with abdominal pain and change in bowel habits that its etiology is not known yet. In the recent years, melatonin has been proposed as a possible candidate. In the present work, all clinical or non-clinical data about effects of melatonin in GI tract and IBS obtained from literature without time limit up to August 2010 have been studied and reviewed. Eight clinical trials were reviewed for efficacy and disturbance of melatonin in IBS and other GI disorders. The results showed disturbances in endogenous melatonin concentration in IBS patients and significant benefits of exogenous melatonin in these patients by decreasing abdominal pain and improvement of overall IBS symptom scores. The results of seventeen non-clinical studies showed anxiolytic, anti-inflammatory, anti oxidative and motility regulatory effects of melatonin on GI tract. In conclusion melatonin can be a target of interest in IBS because of its potentials to regulate GI motility. PMID:21128901

Mozaffari, Shilan; Rahimi, Roja; Abdollahi, Mohammad

2010-01-01

360

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

PubMed

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

Pasquali, Renato; Gambineri, Alessandra

2014-02-01

361

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

PubMed Central

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

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

2014-01-01

362

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

PubMed Central

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

Conforti, Giorgio; Capone, Loredana

2014-01-01

363

Effect of lifestyle intervention plus rosiglitazone or placebo therapy on left ventricular mass assessed with cardiovascular magnetic resonance in the metabolic syndrome  

PubMed Central

Background To evaluate the effect of lifestyle intervention in conjunction with rosiglitazone or placebo therapy on left ventricular (LV) mass, using cardiovascular magnetic resonance (CMR) in the metabolic syndrome. Methods The present study was a pre-specified substudy of a double-blind randomized controlled trial evaluating the effect of lifestyle intervention in conjunction with rosiglitazone or placebo therapy on carotid artery atherosclerosis in the metabolic syndrome. From this original study population, 10 subjects from the placebo group and 10 from the rosiglitazone group were randomly selected. At baseline and follow-up (52 weeks), clinical and laboratory measurements were assessed and a CMR-examination was performed to evaluate LV mass indexed for body surface area (LV mass-I). Subsequently, the effect of therapy (rosiglitazone vs. placebo) and clinical and laboratory variables on LV mass-I was evaluated. Results In both groups, body mass index, waist circumference, systolic and diastolic blood pressure significantly decreased during follow-up. Interestingly, LV mass-I significantly decreased in the placebo group (48.9 ± 5.3 g/m2 vs. 44.3 ± 5.6 g/m2, p < 0.001) indicating reverse remodeling, whereas LV mass-I remained unchanged in the rosiglitazone group (54.7 ± 9.9 g/m2 vs. 53.7 ± 9.2 g/m2, p = 0.3). After correction for systolic and diastolic blood pressure and triglyceride, the kind of therapy (rosiglitazone vs. placebo) remained the only significant predictor of LV mass-I reduction. Conclusions Lifestyle intervention resulted in a reduction of LV mass-I in the metabolic syndrome, indicating reverse remodeling. However, rosiglitazone therapy may have inhibited this positive reverse remodeling. Trial registration Current Controlled Trials ISRCTN54951661.

2011-01-01

364

Possibility of Treating Basal Cell Carcinomas of Nevoid Basal Cell Carcinoma Syndrome with Superficial X-Ray Therapy  

Microsoft Academic Search

Background: The nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin-Goltz syndrome, is a rare dermatological disease inherited according to an autosomal dominant pattern. From the dermatological point of view, the most evident characteristic of the syndrome is the early onset of multiple basal cell carcinomas (BCCs). An ideal treatment of BCCs of the NBCCS does not exist. Objective:

Massimo Caccialanza; Serena Percivalle; Roberta Piccinno

2004-01-01

365

Citalopram is not Effective Therapy for Non-Depressed Patients with Irritable Bowel Syndrome  

PubMed Central

Background & Aims Data are conflicting on the benefit of selective serotonin reuptake inhibitors (SSRIs) for patients with irritable bowel syndrome (IBS); the role of visceral sensitivity in IBS pathophysiology is unclear. We assessed the effects of citalopram and the relationships between, symptoms, and quality of life (QOL), and rectal sensitivity in non-depressed patients with IBS. Methods Patients from primary, secondary and tertiary care centers were randomly assigned to groups given citalopram (20 mg/day for 4 weeks, then 40 mg/day for 4 weeks) or placebo. The study was double masked with concealed allocation. Symptoms were assessed weekly; IBS-QOL and rectal sensation were determined from barostat measurements made at the beginning and end of the study. Results Patients that received citalopram did not have a higher rate of adequate relief from IBS symptoms than subjects that received placebo (12/27, 44% vs 15/27, 56% respectively; P=0.59), regardless of IBS subtype. The odds ratio for weekly response to citalopram vs placebo was 0.80 (95% confidence interval [CI] 0.61–1.04). Citalopram did not reduce specific symptoms or increase IBS-QOL scores; it had no effect on rectal compliance and a minimal effect on sensation. Changes in IBS-QOL score and pressure-eliciting pain were correlated (r=0.33, 95% CI 0.03–0.57); changes in symptoms and rectal sensitivity or IBS-QOL scores were not correlated. Conclusions Citalopram was not superior to placebo in treating non-depressed IBS patients. Changes in symptoms were not correlated with changes in rectal sensation assessed by barostat; Any benefit of citalopram in non-depressed IBS patients is likely to be modest.

Ladabaum, Uri; Sharabidze, Annie; Levin, Theodore R.; Zhao, Wei K.; Chung, Elaine; Bacchetti, Peter; Jin, Chengshi; Grimes, Barbara; Pepin, Craig J.

2009-01-01

366

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

PubMed Central

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

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

2014-01-01

367

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

PubMed Central

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

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

2010-01-01

368

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

369

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.  

PubMed

LEGO 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 control group was also assessed. Results showed that the LEGO therapy group improved more than the other groups on autism-specific social interaction scores (Gilliam Autism Rating Scale). Maladaptive behaviour decreased significantly more in the LEGO and SULP groups compared to the control group. There was a non-significant trend for SULP and LEGO groups to improve more than the no-intervention group in communication and socialisation skills. PMID:18566882

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

2008-11-01

370

Cerebral hemodynamics in patients with obstructive sleep apnea syndrome monitored with near-infrared spectroscopy (NIRS) during positive airways pressure (CPAP) therapy: a pilot study  

NASA Astrophysics Data System (ADS)

In obstructive sleep apnea syndrome (OSA) the periodic reduction or cessation of breathing due to narrowing or occlusion of the upper airway during sleep leads to daytime symptoms and increased cardiovascular risk, including stroke. The higher risk of stroke is related to the impairment in cerebral vascular autoregulation. Continuous positive airways pressure (CPAP) therapy at night is the most effective treatment for OSA. However, there is no suitable bedside monitoring method evaluating the treatment efficacy of CPAP therapy, especially to monitor the recovery of cerebral hemodynamics. NIRS is ideally suited for non-invasive monitoring the cerebral hemodynamics during sleep. In this study, we will for first time assess dynamic changes of cerebral hemodynamics during nocturnal CPAP therapy in 3 patients with OSA using NIRS. We found periodic oscillations in HbO2, HHb, tissue oxygenation index (TOI) and blood volume associated with periodic apnea events without CPAP in all OSA patients. These oscillations were gradually attenuated and finally eliminated with the stepwise increments of CPAP pressures. The oscillations were totally eliminated in blood volume earlier than in other hemodynamic parameters. These results suggested that 1) the cerebral hemodynamic oscillations induced by OSA events can effectively be attenuated by CPAP therapy, and 2) blood flow and blood volume recovered first during CPAP therapy, followed by the recovery of oxygen consumption. Our study suggested that NIRS is a useful tool to evaluate the efficacy of CPAP therapy in patients with OSA bedside and in real time.

Zhang, Zhongxing; Schneider, Maja; Laures, Marco; Fritschi, Ursula; Lehner, Isabella; Qi, Ming; Khatami, Ramin

2014-03-01

371

Anti-M?llerian hormone - a prognostic marker for metformin therapy efficiency in the treatment of women with infertility and polycystic ovary syndrome  

PubMed Central

Background: The anti- M?llerian hormone (AMH) is secreted in women exclusively by the granulosa cells of the ovarian follicles. The serum level of AMH is a precise marker of follicle pool size. In recent clinical studies of polycystic ovary syndrome (PCOS), the serum levels of AMH were elevated about two to threefold. The use of metformin in women with infertility and PCOS has proved to be efficient: restoring ovulation and reducing metabolic dysfunctions. The aim of our study is to assess AMH as a prognostic marker for metformin therapy efficiency in the treatment of women with infertility and polycystic ovary syndrome (PCOS). Methods: Eleven patients with infertility and PCOS were enrolled; PCOS was diagnosed according to the criteria of Androgen Excess and Polycystic Ovarian Syndrome Society 2006 (AE/PCOS). All patients have received metformin therapy. Serum AMH was recorded before and after 2 months of treatment; the normal laboratory values were 2.0-6.8 ng/ml. Results: The primary serum AMH level of all women in study was very high: 8.99±0.99 ng/ml. After 2 months of treatment with metformin ovulation was restored in all the patients and the serum AMH levels were significantly decreased. Conclusions: In clinical practice, serum AMH levels of women with infertility and PCOS receiving metformin are a useful predictive marker for the treatment efficiency.

Neagu, M; Cristescu, C

2012-01-01

372

11p15 translocations involving the NUP98 gene in childhood therapy-related acute myeloid leukemia/myelodysplastic syndrome.  

PubMed

In a survey of childhood therapy-related acute myeloid leukemia/myelodysplastic syndrome (t-AML/MDS) in Japan, we found 11p15 translocations in 5 (6%) of 81 children with t-AML/MDS. t(11;17)(p15;q21), t(11;12)(p15;q13), t(7;11)(p15;p15), inv(11)(p15q22), and add(11)(p15) were each found in one patient. Southern blotting and/or RT-PCR analyses revealed rearrangements of the NUP98 gene in tumor samples of all five patients. Rearrangements of DDX10 were detected in t-AML/MDS cells with inv(11), and rearrangements of HOXA9 were detected in t-AML cells with t(7;11). The 17q21 breakpoint of t(11;17) and the 12q13 breakpoint of t(11;12)(p15;q13) coincided with the loci of the HOXB and HOXC gene families, respectively. Therefore, it is reasonable to speculate that one of the HOXB genes and one of the HOXC genes were fused to NUP98 by t(11;17) and t(11;12), respectively, in t-AML/MDS cells. We propose that NUP98 may be a target gene for t-AML/MDS, and that t-AML/MDS with a fusion of NUP98 and HOX or DDX10 genes may be more frequent in children than in patients of other age groups. PMID:10502319

Nishiyama, M; Arai, Y; Tsunematsu, Y; Kobayashi, H; Asami, K; Yabe, M; Kato, S; Oda, M; Eguchi, H; Ohki, M; Kaneko, Y

1999-11-01

373

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

PubMed

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

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

2014-03-01

374

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

PubMed Central

Objectives: The aim of this study was to verify the efficacy and safety of intravesical treatment combining sodium hyaluronate (HA) and chondroitin sulfate (CS) in patients with interstitial cystitis/bladder pain syndrome (IC/BPS). Methods: Between February 2010 and May 2011, 20 consecutive women with IC/BPS were treated with intravesical instillations containing sodium HA (1.6%; 800 mg/50 ml) and sodium CS (2%; 1 g/50 ml) weekly for the first month, biweekly for the second month, and then monthly for at least 3 months. Before and after treatment, all patients filled in the Interstitial Cystitis Symptom Index and Problem Index (ICSI/ICPI), the Patient Health Questionnaire 9 and the Pelvic Pain and Urgency/Frequency Patient Symptom Scale (PUF). Treatment efficacy was assessed by comparing the pre- and post-treatment mean scores of the three questionnaires using Student’s t test (p value <0.05 was considered significant). Results: Statistically significant mean decreases in ICSI (from 13.0 to 9.3; p = 0.0003), ICPI (from 11.35 to 8.85; p = 0.0078) and PUF (from 20.0 to 15.75; p = 0.0007) questionnaire scores were seen. No cases of side effects or complications were observed. The mean follow up was 5 months. Conclusions: Despite the limitations of this study, the outcomes confirmed the role of combination therapy with HA and CS as a safe and effective option for the treatment of IC/BPS. Further randomized controlled studies with a higher number of patients and a longer follow-up period are needed to confirm these results.

Giberti, Claudio; Cortese, Pierluigi; Schenone, Maurizio

2013-01-01

375

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

376

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

Microsoft Academic Search

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

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

2011-01-01

377

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

PubMed

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

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

2014-01-01

378

Reduced-Intensity Allogeneic Stem Cell Transplantation for Co-Emergence of Chemotherapy-Refractory Follicular Lymphoma and Therapy-Related Myelodysplastic Syndrome  

PubMed Central

A 54-year-old male was diagnosed with follicular lymphoma in September 2003. Despite multiple chemotherapies, including autologous hematopoietic stem cell transplantation (HSCT) with high-dose chemotherapy, the disease eventually relapsed. Additionally, bone marrow analysis revealed the co-emergence of therapy-related myelodysplastic syndrome (t-MDS) in February 2012. In March 2012, we performed related allogeneic HSCT for the treatment of both malignancies. This strategy was successful and the patient has remained free from both malignancies for 23 months. Allogeneic HSCT is a potent curative therapeutic option for both t-MDS and refractory follicular lymphoma.

Shimura, Yuji; Kuroda, Junya; Sasaki, Nana; Uchiyama, Hitoji; Ohshiro, Muneo; Matsumura, Yayoi; Nagoshi, Hisao; Mizutani, Shinsuke; Kobayashi, Tsutomu; Matsumoto, Yosuke; Horiike, Shigeo; Taniwaki, Masafumi

2014-01-01

379

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

PubMed Central

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

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

2009-01-01

380

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

381

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

Microsoft Academic Search

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

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

2007-01-01

382

Humoral and cellular immune parameters before and during immunosuppressive therapy of a patient with stiff-man syndrome and insulin dependent diabetes mellitus  

PubMed Central

OBJECTIVES—Humoral and cellular immune reactivity are reported for two neuroendocrine autoantigens—glutamic acid decarboxylase (GAD) and the protein tyrosine phosphatase IA-2—in a patient with the autoimmune type of stiff-man syndrome and insulin dependent diabetes (IDDM).?METHODS—Antibodies and T cell proliferation against GAD and IA-2 and cytokine release of antigen stimulated T cells (IFN-?) were determined before and several times during immunosuppressive therapy with prednisolone.?RESULTS—Raised GAD antibodies against full length GAD65 or chimeric constructs were detected before therapy and they remained at a high concentration despite a marked clinical improvement during cortisone treatment. Antibodies to IA-2 were undetectable, but weak T cell responses to both GAD and IA-2 were seen before therapy and once on reduction of high cortisone dosages when the patient showed signs of clinical deterioration. Cytokine profiles showed increased IFN-? production after stimulation with GAD or IA-2 suggesting increased activation of TH1 cells.?CONCLUSION—Immunosuppressive therapy —even with extremely high doses of 500 mg a day—does not lead to the reduction of antibody concentrations in the periphery nor to a switch in epitope recognition of such antibodies despite clinical improvement. The amount of T cell reactivity to various antigens, however, may be a useful marker to monitor the effectiveness of immunotherapy.??

Hummel, M; Durinovic-Bello, I; Bonifacio, E; Lampasona, V; Endl, J; Fessele, S; Bergh, F; Trenkwalder, C; Standl, E; Ziegler, A

1998-01-01

383

Growth Promotion and Turner-Specific Bone Age after Therapy with Growth Hormone and in Combination with Oxandrolone: When Should Therapy Be Started in Turner Syndrome?  

Microsoft Academic Search

The aims of this comparative multicenter study of 67 girls with Turner syndrome (TS) on three different therapeutical regimens were, first, to evaluate the effect of either recombinant human growth hormone (GH) alone or in combination with the anabolic steroid oxandrolone (Oxa) on height velocity and on Turner-specific bone age (BA’TS) and, second, to estimate the gain in final height

E. E. Joss; R. E. Mullis; E. A. Werder; C. J. Partsch; W. G. Sippell

1997-01-01

384

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

ClinicalTrials.gov

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

2013-01-04

385

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

PubMed Central

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

Burghardt, Kyle J; Ellingrod, Vicki L

2014-01-01

386

Detection of metabolic syndrome in schizophrenia and implications for antipsychotic therapy : is there a role for folate?  

PubMed

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

Burghardt, Kyle J; Ellingrod, Vicki L

2013-02-01

387

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

PubMed Central

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

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

2012-01-01

388

Short communication: Paraoxonase 1 (PON1) in French HIV-infected patients under antiretroviral therapy: relationship with the metabolic syndrome and inflammation.  

PubMed

Our goal was to determine if paraoxonase 1 (PON1) activity relates to the presence of metabolic syndrome (MS) and inflammation in HIV patients treated with highly active antiretroviral therapy (HAART). This was a prospective, multicenter study including 269 patients receiving HAART for at least 1 year and a maximum of 4 years. PON1 and inflammatory markers [C reactive protein (CRP), interleukin-6 (IL-6), serum amyloid A (SAA), and soluble tumor necrosis factor receptors 2 (sTNF-R2)] were compared between patients with or without MS and the association between inflammatory markers and PON1 was assessed by logistic regression analyses. MS was found in 18.2% of the patients. Inflammatory markers, with the exception of sTNF-R2, were significantly higher, while PON1 activity was significantly lower in the presence of metabolic syndrome. PON1 activity was significantly related to apolipoprotein C3, CD4 count, and sTNF-R2. It may be concluded that PON1 appears to be a marker for the metabolic syndrome in HIV-infected subjects. PON1 activity is related to dyslipidemia and the immunological status of the patients but is not fully determined by inflammation. PMID:23837895

Bobin-Dubigeon, Christine; Biron, Charlotte; Volteau, Christelle; Piroth, Lionel; Biron, Antoine; Perré, Philippe; Leport, Catherine; Prazuck, Thierry; Sébille-Rivain, Veronique; Raffi, François; Bard, Jean Marie

2013-12-01

389

The "New Deadly Quartet" for cardiovascular disease in the 21st century: obesity, metabolic syndrome, inflammation and climate change: how does statin therapy fit into this equation?  

PubMed

Despite population-based improvements in cardiovascular risk factors, such as blood pressure, cholesterol and smoking, cardiovascular disease still remains the number-one cause of mortality in the United States. In 1989, Kaplan coined the term "Deadly Quartet" to represent a combination of risk factors that included upper body obesity, glucose intolerance, hypertriglyceridemia and hypertension [Kaplan in Arch Int Med 7:1514-1520, 1989]. In 2002, the third report of the National Cholesterol Education Program Adult Treatment Panel (NCEP-ATP III) essentially added low HDL-C criteria and renamed this the "metabolic syndrome." [The National Cholesterol Education Program (NCEP) in JAMA 285:2486-2497, 2001] However, often forgotten was that a pro-inflammatory state and pro-thrombotic state were also considered components of the syndrome, albeit the panel did not find enough evidence at the time to recommend routine screening for these risk factors [The National Cholesterol Education Program (NCEP) in JAMA 285:2486-2497, 2001]. Now over a decade later, it may be time to reconsider this deadly quartet by reevaluating the roles of obesity and subclinical inflammation as they relate to the metabolic syndrome. To complete this new quartet, the addition of increased exposure to elevated levels of particulate matter in the atmosphere may help elucidate why this cardiovascular pandemic continues, despite our concerted efforts. In this article, we will summarize the evidence, focusing on how statin therapy may further impact this new version of the "deadly quartet". PMID:24338517

Clearfield, Michael; Pearce, Melissa; Nibbe, Yasmin; Crotty, David; Wagner, Alesia

2014-01-01

390

Rare t(1;11)(q23;p15) in therapy-related myelodysplastic syndrome evolving into acute myelomonocytic leukemia: a case report and review of the literature.  

PubMed

Balanced chromosome rearrangements are the hallmark of therapy-related leukemia that develops in patients treated with topoisomerase II inhibitors. Many of these rearrangements involve recurrent chromosomal sites and associated genes (11q23/MLL, 21q22.3/AML1, and 11p15/NUP98), which can interact with a variety of partner genes. One such rearrangement is the rare t(1;11)(q23;p15), which involves juxtaposition of the homeobox gene PMX1 (PRRX1) and NUP98. We report on an additional patient with t(1;11) who presented with myelodysplastic syndrome (MDS) subsequent to treatment for a pleomorphic liposarcoma. With time, the patient's disorder progressed to acute myelomonocytic leukemia with cytogenetic evidence of clonal evolution. To our knowledge, this is the first report of a patient presenting with a myelodysplastic syndrome with isolated t(1;11) (q23;p15), which evolved into therapy-related acute myeloid leukemia (t-AML). This patient is the third reported with this cytogenetic rearrangement and t-AML, and is compared with the other two reports of t(1;11)(q23;p15). PMID:17889707

Zhang, Ling; Alsabeh, Randa; Mecucci, Cristina; La Starza, Roberta; Gorello, Paolo; Lee, Stephen; Lill, Michael; Schreck, Rhona

2007-10-01

391

Lemierre's syndrome  

PubMed Central

Lemierre’s syndrome is a condition characterized by thrombophlebitis of the internal jugular vein and bacteremia caused by primarily anaerobic organisms, following a recent oropharyngeal infection. This has been an uncommon illness in the era of antibiotic therapy, though it has been reported with increasing frequency in the past 15 years. Lemierre’s syndrome should be suspected in young healthy patients with prolonged symptoms of pharyngitis followed by symptoms of septicemia or pneumonia, or an atypical lateral neck pain. Diagnosis is often confirmed by identification of thrombophlebitis of the internal jugular vein and growth of anaerobic bacteria on blood culture. Treatment involves prolonged antibiotic therapy occasionally combined with anticoagulation. We review the literature and a case of a child with Lemierre’s syndrome.

2013-01-01

392

Treatment Options for Myelodysplastic Syndromes  

MedlinePLUS

... myelodysplastic syndromes includes supportive care, drug therapy, and stem cell transplantation. Patients with a myelodysplastic syndrome who have ... in patients with acute myeloid leukemia. Chemotherapy with stem cell transplant Stem cell transplant is a method of ...

393

Treatment Option Overview (Myelodysplastic Syndromes)  

MedlinePLUS

... myelodysplastic syndromes includes supportive care, drug therapy, and stem cell transplantation. Patients with a myelodysplastic syndrome who have ... in patients with acute myeloid leukemia. Chemotherapy with stem cell transplant Stem cell transplant is a method of ...

394

A diffuse T lymphocytic gastrointestinal mucosal infiltration associated with Sjögren's syndrome resulting in a watery diarrhea syndrome and responsive to immunosuppressive therapy.  

PubMed

We report the case of a 45-yr-old white man, investigated for chronic diarrhea, malabsorption and weight loss associated with sicca syndrome. Endoscopic and x-ray examinations showed normal macroscopic mucosa in gastrointestinal tract (GIT). Immunohistochemistry showed diffuse polyclonal T cell lymphocytes infiltrating either epithelium and lamina propria in GIT. There was no villous atrophy in the jejunum and ileum. Corticosteroids, azathioprine, and cyclosporine failed to improve symptoms. Monthly intravenous cyclophosphamide administered over 1 yr, stopped the diarrhea and weight loss. The patient is free of symptoms up to a 5-yr follow-up. PMID:9860433

Sobhani, I; Brousse, N; Vissuzaine, C; Mcintyre, E; Marmuse, J P; Kahn, M F; Potet, F; Mignon, M

1998-12-01

395

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

Microsoft Academic Search

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

Yong D. Park

2003-01-01

396

Risk factors for evolution of acquired aplastic anemia into myelodysplastic syndrome and acute myeloid leukemia after immunosuppressive therapy in children  

Microsoft Academic Search

Long-term survivors of acquired aplas- tic anemia (AA) have an increased risk of developing myelodysplastic syn- drome (MDS) and acute myeloid leuke- mia (AML) after immunosuppressive therapy (IST). It is uncertain whether the increased survival time simply dis- closes the natural history of AA as a premalignant disease or whether sec- ondary disease is related to the therapy itself. Between

Seiji Kojima; Akira Ohara; Masahiro Tsuchida; Toru Kudoh; Ryoji Hanada; Yuri Okimoto; Takashi Kaneko; Toshikuni Takano; Koichiro Ikuta; Ichiro Tsukimoto

2002-01-01

397

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

2013-08-01

398

Bone marrow fibrosis in patients with primary myelodysplastic syndromes has prognostic value using current therapies and new risk stratification systems.  

PubMed

Bone marrow fibrosis has recently been recognized as an adverse histological feature in patients with primary myelodysplastic syndromes. In this study, we assessed the prognostic impact of bone marrow fibrosis in patients with primary myelodysplastic syndromes under the recently revised new risk stratification systems: the New Comprehensive Cytogenetic Scoring System and the Revised International Prognostic Scoring System. From 2002 to 2012, a total of 79 (13%) patients with primary myelodysplastic syndromes and moderate/severe bone marrow fibrosis were identified; and these patients were compared with a control group of 166 patients with myelodysplastic syndromes but no significant fibrosis. Bone marrow fibrosis predicted an inferior overall survival and leukemia event-free survival for patients who received no hematopoietic stem cell transplant in univariate and multivariate analysis. Eleven patients with bone marrow fibrosis and 32 control group patients underwent hematopoietic stem cell transplant; and bone marrow fibrosis was an independent risk for an inferior overall survival but not leukemia-free survival. In addition, 17 (4%) patients developed bone marrow fibrosis during the course of myelodysplastic syndromes, which was accompanied by clinical and cytogenetic evidence of disease progression. JAK2 V617F mutations were detected in 6 of the 28 patients with bone marrow fibrosis presenting at the time of diagnosis and 2 of the 7 patients with bone marrow fibrosis developing in the course of disease, significantly higher than the control group patients. We conclude that bone marrow fibrosis is an adverse risk feature in primary myelodysplastic syndromes in the current therapeutic era, and this risk feature is not captured by newly revised risk stratification systems. Inclusion of bone marrow fibrosis in patient assessment may further aid in risk-adapted therapeutic decisions. PMID:24186132

Fu, Bin; Jaso, Jesse M; Sargent, Rachel L; Goswami, Maitrayee; Verstovsek, Srdan; Medeiros, L Jeffrey; Wang, Sa A

2014-05-01

399

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

PubMed

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

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

2014-05-01

400

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

PubMed Central

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

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

2014-01-01

401

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

PubMed

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

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

2013-02-01

402

Gentle thawing of the frozen shoulder: A prospective study of supervised neglect versus intensive physical therapy in seventy-seven patients with frozen shoulder syndrome followed up for two years  

Microsoft Academic Search

Seventy-seven patients with idiopathic frozen shoulder syndrome were included in a prospective study to compare the effect of intensive physical rehabilitation treatment, including passive stretching and manual mobilization (stretching group) versus supportive therapy and exercises within the pain limits (supervised neglect group). There were no significant differences in age, sex, time elapsed since onset, and disease severity at inclusion. All

Ronald L. Diercks; Martin Stevens

2004-01-01

403

De novo nephrotic syndrome following pegylated interferon alfa 2b\\/ribavirin therapy for chronic hepatitis C infection  

Microsoft Academic Search

This paper describes the case of a patient with HCV, without previous evidence of nephropathy, who following two well-tolerated\\u000a cycles of treatment with interferon alone developed nephrotic syndrome after a third attempt with ribavirin associated with\\u000a peginterferon alfa 2b. The patient exhibited a total remission when the antiviral treatment was discontinued.

Jose L. Tovar; Maria Buti; Alfonso Segarra; Joaquim Majó; Rafael Esteban

2008-01-01

404

Ticagrelor versus Genotype-Driven Antiplatelet Therapy for Secondary Prevention after Acute Coronary Syndrome: A Cost-Effectiveness Analysis  

Microsoft Academic Search

BackgroundClopidogrel's effectiveness is likely reduced significantly for prevention of thrombotic events after acute coronary syndrome (ACS) in patients exhibiting a decreased ability to metabolize clopidogrel into its active form. A genetic mutation responsible for this reduced effectiveness is detectable by genotyping. Ticagrelor is not dependent on gene-based metabolic activation and demonstrated greater clinical efficacy than clopidogrel in a recent secondary

Daniel J. Crespin; Jerome J. Federspiel; Andrea K. Biddle; Daniel E. Jonas; Joseph S. Rossi

2011-01-01

405

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

ERIC Educational Resources Information Center

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

Cardaciotto, LeeAnn; Herbert, James D.

2004-01-01

406

Childhood Moyamoya Syndrome  

Microsoft Academic Search

The development of typical clinical and radiological features of moyamoya syndrome is described in 3 children following radiation therapy for a gliomatous tumor at the base of the brain. The effect of radiation therapy on the medium-sized arteries is discussed with a review of the literature. Radiation therapy as the causative factor for the narrowing or occlusion of the carotid

Kandavanam Rajakulasingam; Leonard J. Cerullo; Anthony J. Raimondi

1979-01-01

407

[A case of acute respiratory distress syndrome associated with pandemic influenza A (H1N1) pneumonia which was aggravated by the cessation of corticosteroid therapy].  

PubMed

A 72-year-old man was admitted to the intensive care unit of our hospital with acute respiratory distress syndrome (ARDS). A chest CT scan showed diffuse consolidations and ground-glass opacities in both lungs. We diagnosed ARDS secondary to community-acquired pneumonia. We then started mechanical ventilation with airway pressure release ventilation and treated him with antibiotics, peramivir, and corticosteroids, despite negative results for a rapid antigen test for influenza. Bronchial lavage on day 10 showed blood-tinged fluid and hemosiderin-laden macrophages, but no bacteria or fungi. Real-time reverse-transcriptase polymerase chain reaction testing yielded a positive result for pandemic influenza A (H1N1). The mechanical ventilator was removed on day 15, corticosteroid administration was discontinued on day 22 and antibiotics were discontinued on day 23. However, he had a fever on day 28, pleural pain and dyspnea on day 29, and exacerbation of the infiltration as demonstrated on chest CT on day 30. On day 31, repeat bronchoalveolar lavage showed an increase in the number of total cells which were lymphocyte-predominant, but there were no pathogens. We believed that this clinical exacerbation might have occurred due to the re-exacerbation of pandemic influenza A (H1N1) pneumonia due to the cessation of corticosteroids. The re-administration of corticosteroids was effective, and were eventually tapered. Currently, the use of corticosteroid therapy for severe pandemic influenza A (H1N1) pneumonia is controversial, but even the late administration of corticosteroid therapy may be effective. However, this case also suggests that the early administration of corticosteroid therapy as immunomodulation might be effective in selected cases, and that cessation of such therapy during the early phase of ARDS may cause exacerbation of clinical symptoms. PMID:22352059

Hibino, Makoto; Akazawa, Kenichiro; Hikino, Koji; Oe, Motoki

2011-12-01

408

Patterns of infection in patients with myelodysplastic syndromes and acute myeloid leukemia receiving azacitidine as salvage therapy. Implications for primary antifungal prophylaxis.  

PubMed

Incidence, etiology, and outcome of infectious episodes in patients with myeloid neoplasms receiving azacitidine are uncertain, with no prospective data available in this group of patients. The aim of the current study was to analyze the incidence and factors related to the probability of infection in a cohort of patients with myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) treated with azacitidine who did not receive any type of antimicrobial prophylaxis. Significantly, the group of patients who received prior intensive chemotherapy had more infectious episodes (P = 10(-4)), and particularly, invasive aspergillosis (P = .015), than patients who received frontline azacitidine. Primary antifungal prophylaxis might be recommended in MDS and AML patients receiving azacitidine as salvage therapy after intensive regimens. PMID:24220615

Falantes, Jose F; Calderón, Cristina; Márquez-Malaver, Francisco J; Aguilar-Guisado, Manuela; Martín-Peña, Almudena; Martino, María L; Montero, Isabel; González, Jose; Parody, Rocío; Pérez-Simón, Jose A; Espigado, Ildefonso

2014-02-01

409

Rehabilitation of patients with thoracic outlet syndrome  

Microsoft Academic Search

A series of physical therapy protocols is proposed for patients with thoracic outlet syndrome. The anatomic findings dictating certain physical therapeutic approaches are outlined. General principles of physical therapy that stem from these findings are suggested, and a specific protocol for the physical therapy regimen is given. An appropriate physical therapy program for thoracic outlet syndrome patients with symptoms of

Christian Aligne; Xavier Barral

1992-01-01

410

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

PubMed Central

Histoplasma capsulatum var. capsulatum infection is rare outside disease-endemic areas. Clinical presentation and outcome of acquired immunodeficiency syndrome–related histoplasmosis are unknown in non-endemic areas with wide access to highly active anti-retroviral therapy (HAART). Retrospective analysis of cases recorded at the French National Reference Center for Mycoses and Antifungals during two decades: pre-HAART (1985–1994) and HAART (1997–2006). Clinical features and outcome of all adults with proven acquired immunodeficiency syndrome–related histoplasmosis were compared between the two periods. One hundred four patients were included (40 during the pre-HAART era and 64 during the HAART era). Diagnosis was established a mean of 62 days after onset of symptoms. One-year overall mortality rates decreased from 53% (pre-HAART era) to 22% (HAART era). Diagnosis during the pre-HAART era and an older age were the only independent factors associated with death. Histoplasmosis is a rare invasive fungal infection outside disease-endemic areas. Its prognosis improved significantly during the HAART era.

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

2011-01-01

411

Oxygen therapies.  

PubMed

Oxygen therapies are unproven alternatives promoted as a cure for cancer, acquired immune deficiency syndrome (AIDS), and other degenerative diseases. These "therapies" are offered at clinics in Mexico, the United States, and Europe. Proponents claim that many diseases, including cancer, are caused by oxygen deficiency and that oxygenation can restore health by destroying cancer cells, eliminating pathogens, stimulating metabolism, and by producing "oxidative detoxification." There is no scientific evidence to support any of these claims. Oxygen