Enzyme replacement therapy for infantile-onset Pompe disease.

Science.gov (United States)

Chen, Min; Zhang, Lingli; Quan, Shuyan

2017-11-20

Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency. To assess the effectiveness, safety and appropriate dose regimen of enzyme replacement therapy for treating infantile-onset Pompe disease. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register, which is compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the Cochrane Central Register of Controlled Trials (CENTRAL), Embase (Ovid), PubMed and LILACS, and CBM, CNKI, VIP, and WANFANG for literature published in Chinese. In addition, we searched three online registers: WHO International Clinical Trials Registry Platform ClinicalTrials.gov, and www.genzymeclinicalresearch.com. We also searched the reference lists of relevant articles and reviews.Date of last search of the Group's Inborn Errors of Metabolism Trials Register: 24 November 2016. Randomized and quasi-randomized controlled trials of enzyme replacement therapy in children with infantile-onset Pompe disease. Two authors independently selected relevant trials, assessed the risk of bias and extracted data. We contacted investigators to obtain important missing information. We found no trials comparing the effectiveness and safety of enzyme replacement therapy to another intervention, no intervention or placebo.We found one trial (18 participants

Long term enzyme replacement therapy for Fabry disease: effectiveness on kidney, heart and brain

NARCIS (Netherlands)

Rombach, Saskia M.; Smid, Bouwien E.; Bouwman, Machtelt G.; Linthorst, Gabor E.; Dijkgraaf, Marcel G. W.; Hollak, Carla E. M.

2013-01-01

Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase A deficiency leading to renal, cardiac, cerebrovascular disease and premature death. Treatment with α-galactosidase A (enzyme replacement therapy, ERT) stabilises disease in some patients, but long term effectiveness

Overweight, insulin resistance and type II diabetes in type I Gaucher disease patients in relation to enzyme replacement therapy

NARCIS (Netherlands)

Langeveld, M.; de Fost, M.; Aerts, J. M. F. G.; Sauerwein, H. P.; Hollak, C. E. M.

2008-01-01

Type I Gaucher disease, a lysosomal storage disorder is associated with metabolic abnormalities such as high resting energy expenditure, low circulating adiponectin and peripheral insulin resistance. Treatment with enzyme replacement therapy (enzyme therapy) leads to a decrease in resting energy

Partial resolution of bone lesions. A child with severe combined immunodeficiency disease and adenosine deaminase deficiency after enzyme-replacement therapy

International Nuclear Information System (INIS)

Yulish, B.S.; Stern, R.C.; Polmar, S.H.

1980-01-01

A child with severe combined immunodeficiency disease and adenosine deaminase deficiency, with characteristic bone dysplasia, was treated with transfusions of frozen irradiated RBCs as a means of enzyme replacement. This therapy resulted in restoration of immunologic competence and partial resolution of the bone lesions. Although the natural history of these lesions without therapy is not known, enzyme-replacement therapy may have played a role in the resolution of this patient's bone lesions

AB073. Classic infantile-onset Pompe disease: phenotypes and outcomes of 5 Vietnamese patients receiving enzyme replacement therapy

Science.gov (United States)

Nguyen, Khanh Ngoc; Do, Mai Thi Thanh; Can, Ngoc Thi Bich; Hwu, Wuh-Liang; Vu, Dung Chi

2017-01-01

Background Pompe disease (PD) or glycogen storage disease type II is a lysosomal storage disorder, caused by mutations of GAA gene which results in deficiency of acid alpha-glucosidase (GAA) enzyme that involves in metabolism of glycogen in the lysosomes. Its incidence is 1/14,000–1/100,000. PD is divided into three types: classic infantile onset, non-classic infantile onset, and late onset. Early enzyme replacement therapy (ERT) before developing respiratory distress may lead to good outcome. Since 2013, we have identified 16 cases with classic infantile-onset and 5 cases were treated with ERT. Herein, we describe phenotypes and outcomes of five infantile-onset PD patients who received ERT. Methods GAA enzyme assay was done at National Taiwan University Hospital. Results Ages of diagnosis were 12, 38 and 70 days, 5 and 9 months of age. Clinical presentations included macroglossia (5/5), hypertrophic cardiomyopathy (5/5), failure to thrive (5/5), facial weakness and hypotonia (3 patients diagnosed after 70 days of age), respiratory failure (1 patient diagnosed at 9 months of age). All patients had mildly elevated plasma CK (270–380 UI/L) and transaminase (60–260 UI/l). Ages at starting ERT were 28 and 58 days, 3, 6 and 10 months. The time intervals from diagnosis to starting ERT were between 14 days and 1 month. The durations of ERT were 4–22 months. The outcomes were good. All patients had improvement of cardiac functions shown on echocardiography, respiratory status, and motor development. The patient who first received ERT at 10 months of age was reportedly dead at home due to food obstruction at 18 months of age. Current ages of the survivors were 5–24 months. Conclusions Patients with classic infantile-onset PD will have good outcomes if ERT is started early. Newborn screening for this disease is necessary to yield an early diagnosis.

Effect of Palm Kernel Cake Replacement and Enzyme ...

African Journals Online (AJOL)

A feeding trial which lasted for twelve weeks was conducted to study the performance of finisher pigs fed five different levels of palm kernel cake replacement for maize (0%, 40%, 40%, 60%, 60%) in a maize-palm kernel cake based ration with or without enzyme supplementation. It was a completely randomized design ...

Pancreatic Enzyme Therapy and Coefficient of Fat Absorption in Children and AdolReplacement escents With Cystic Fibrosis

NARCIS (Netherlands)

Woestenenk, Janna W; van der Ent, Cornelis K.; Houwen, Roderick H J; van der Ent, CK

Objectives: Pancreatic enzyme replacement therapy (PERT) is the proven therapy to substantially reduce fat malabsorption in patients with cystic fibrosis (CF). Few details of the daily practice regarding PERT and the resulting coefficient of fat absorption (CFA) are known. We therefore recorded the

Clinical response to long term enzyme replacement treatment in children, adolescent and adult patients with Hunter syndrome.

Science.gov (United States)

Dalmau Serra, Jaime; Vitoria Miñana, Isidro; Calderón Fernández, Rafael; Cortell Aznar, Isidoro

2015-11-06

Since enzyme replacement treatment (ERT) with idursulfase is available for Hunter syndrome (HS; mucopolysaccharidosis type II), for the first time, disease progression can be limited and organ damage reduced or prevented. We described retrospectively the clinical evolution of eight HS males, treated with ERT and followed in routine clinical practice in Hospital Infantil La Fe (Valencia, Spain). We studied three children, three adolescents and two adults. Time from diagnosis to ERT ranged from 13.7 to 0.2 years, and duration of ERT ranged from 24 to 77.1 months. From the start of ERT, weight and height increased in children and adolescents and remained stable in adults. Glycosaminoglycans (GAG) decreased in all patients; in patient 5 (aged 23 years), we observed the highest reduction (86%) with recovery of carpal tunnel syndrome, splenomegaly and a decrease in nocturnal oxygen dependence. Our results show that ERT improve respiratory impairment and organomegalies and decrease GAGs levels in all patients including children, adolescent and adults. While cardiac manifestations and facial features stabilized, responses in other parameters were heterogeneous. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

Enzyme replacement therapy in Fabry disease, towards individualized treatment

NARCIS (Netherlands)

Arends, M.

2017-01-01

Fabry disease is a very heterogeneous disorder for which expensive enzyme replacement therapy is available since more than 15 years. Because of the variety of symptoms and disease course, individual choices need to be made to improve the appropriate use of therapy. Supported by ZONWM, we have been

Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network

Directory of Open Access Journals (Sweden)

Alícia Dorneles Dornelles

2014-01-01

Full Text Available Mucopolysaccharidosis type I (MPS I is a rare lysosomal disorder caused by deficiency of alph-L-iduronidase. Few clinical trials have assessed the effect of enzyme replacement therapy (ERT for this condition. We conducted an exploratory, open-label, non-randomized, multicenter cohort study of patients with MPS I. Data were collected from questionnaires completed by attending physicians at the time of diagnosis (T1; n = 34 and at a median time of 2.5 years later (T2; n = 24/34. The 24 patients for whom data were available at T2 were allocated into groups: A, no ERT (9 patients; median age at T1 = 36 months; 6 with severe phenotype; B, on ERT (15 patients; median age at T1 = 33 months; 4 with severe phenotype. For all variables in which there was no between-group difference at baseline, a delta of ;±20% was considered clinically relevant. The following clinically relevant differences were identified in group B in T2: lower rates of mortality and reported hospitalization for respiratory infection; lower frequency of hepatosplenomegaly; increased reported rates of obstructive sleep apnea syndrome and hearing loss; and stabilization of gibbus deformity. These changes could be due to the effect of ERT or of other therapies which have also been found more frequently in group B. Our findings suggest MPS I patients on ERT also receive a better overall care. ERT may have a positive effect on respiratory morbidity and overall mortality in patients with MPS I. Additional studies focusing on these outcomes and on other therapies should be performed.

Guanidinylated Neomycin Conjugation Enhances Intranasal Enzyme Replacement in the Brain.

Science.gov (United States)

Tong, Wenyong; Dwyer, Chrissa A; Thacker, Bryan E; Glass, Charles A; Brown, Jillian R; Hamill, Kristina; Moremen, Kelley W; Sarrazin, Stéphane; Gordts, Philip L S M; Dozier, Lara E; Patrick, Gentry N; Tor, Yitzhak; Esko, Jeffrey D

2017-12-06

Iduronidase (IDUA)-deficient mice accumulate glycosaminoglycans in cells and tissues and exhibit many of the same neuropathological symptoms of patients suffering from Mucopolysaccharidosis I. Intravenous enzyme-replacement therapy for Mucopolysaccharidosis I ameliorates glycosaminoglycan storage and many of the somatic aspects of the disease but fails to treat neurological symptoms due to poor transport across the blood-brain barrier. In this study, we examined the delivery of IDUA conjugated to guanidinoneomycin (GNeo), a molecular transporter. GNeo-IDUA and IDUA injected intravenously resulted in reduced hepatic glycosaminoglycan accumulation but had no effect in the brain due to fast clearance from the circulation. In contrast, intranasally administered GNeo-IDUA entered the brain rapidly. Repetitive intranasal treatment with GNeo-IDUA reduced glycosaminoglycan storage, lysosome size and number, and neurodegenerative astrogliosis in the olfactory bulb and primary somatosensory cortex, whereas IDUA was less effective. The enhanced efficacy of GNeo-IDUA was not the result of increased nose-to-brain delivery or enzyme stability, but rather due to more efficient uptake into neurons and astrocytes. GNeo conjugation also enhanced glycosaminoglycan clearance by intranasally delivered sulfamidase to the brain of sulfamidase-deficient mice, a model of Mucopolysaccharidosis IIIA. These findings suggest the general utility of the guanidinoglycoside-based delivery system for restoring missing lysosomal enzymes in the brain. Copyright © 2017 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.

The outcome of clinical parameters in adults with severe Type I Gaucher disease using very low dose enzyme replacement therapy.

Science.gov (United States)

Wilson, Callum; Spearing, Ruth; Teague, Lochie; Robertson, Patsy; Blacklock, Hilary

2007-01-01

Enzyme replacement therapy is now well established as the treatment of choice in Type I Gaucher disease. Historically higher dosage regimens have been used in preference to lower doses despite the little clinical evidence in the way of large controlled clinical trials to support this. Moreover, the extraordinary cost of therapy means that not all eligible patients are able to be treated at the higher dose. Twelve type I adult patients with relatively severe disease were commenced on a very low dose of 7.5U of alglucerase/imiglucerase per kg every two weeks (initially given thrice weekly and later weekly). Follow-up 5 year data reveal a good visceral and haematological response with outcomes consistent with recently published treatment guidelines. Satisfactory clinical and radiological skeletal improvement was also demonstrated in most patients. Three patients had an inadequate overall skeletal response to therapy. Biomarkers also steadily improved although perhaps not quite at the same rate as that seen in higher doses. Very low dose enzyme replacement therapy may be appropriate for adult type I Gaucher patients with mild-moderate skeletal disease.

Interruption of enzyme replacement therapy in Gaucher disease.

Science.gov (United States)

Goldblatt, J; Fletcher, J M; McGill, J; Szer, J; Wilson, M

2016-05-25

In Australia, 58 patients with Gaucher disease were managed by a Gaucher Disease Advisory Committee (GDAC) through a centrally adminis-tered national programme, the Life Savings Drug Program (LSDP). In June 2009, Genzyme Corporation, which manufactures imiglucerase (Cerezyme), the only enzyme replacement therapy (ERT) registered for the treatment of Gaucher disease in Australia at that time, announced that due to a viral contamination problem there would be no further shipments of Cerezyme to Australia prior to the end of 2009. The GDAC allocated available drug supplies in order to maintain treatment to those most in need on a hierarchal clinical severity basis. A cohort of 24 patients with Type 1 Gaucher disease was withdrawn from therapy, 22 of whom had no discernible clinically significant adverse effects when reviewed off therapy for up to 6 months. In this paper, we review the course of 20 of the patients who have been on imiglucerase for periods of at least 24 months after the end of their 'drug holiday'. No patient experienced a bone crisis nor clinical nor magnetic resonance imaging evidence of new avascular necrosis events during this period. Two years after recommencing ERT after a 6-month drug holiday, no patient had developed an overt irreversible complication of their Gaucher disease, with the majority returning to their previous clinical status.

Renal function predicts long-term outcome on enzyme replacement therapy in patients with Fabry disease.

Science.gov (United States)

Lenders, Malte; Schmitz, Boris; Stypmann, Jörg; Duning, Thomas; Brand, Stefan-Martin; Kurschat, Christine; Brand, Eva

2017-12-01

Renal and cardiac involvement is responsible for substantial morbidity and mortality in Fabry disease (FD). We analysed the incidence of FD-related renal, cardiac and neurologic end points in patients with FD on long-term enzyme replacement therapy (ERT). A retrospective analysis of prospectively collected data from two German FD centres was performed. The impact of renal and cardiac function at ERT-naïve baseline on end point development despite ERT was analysed. Fifty-four patients (28 females) receiving ERT (mean 81 ± 21 months) were investigated. Forty per cent of patients were diagnosed with clinical end points before ERT initiation and 50% of patients on ERT developed new clinical end points. In patients initially diagnosed with an end point before ERT initiation, the risk for an additional end point on ERT was increased {hazard ratio [HR] 3.83 [95% confidence interval (CI) 1.61-9.08]; P = 0.0023}. A decreased glomerular filtration rate (eGFR) ≤75 mL/min/1.73 m2 in ERT-naïve patients at baseline was associated with an increased risk for cardiovascular end points [HR 3.59 (95% CI 1.15-11.18); P = 0.0273] as well as for combined renal, cardiac and neurologic end points on ERT [HR 4.77 (95% CI 1.93-11.81); P = 0.0007]. In patients with normal kidney function, left ventricular hypertrophy at baseline predicted a decreased end point-free survival [HR 6.90 (95% CI 2.04-23.27); P = 0.0018]. The risk to develop an end point was independent of sex. In addition to age, even moderately impaired renal function determines FD progression on ERT. In patients with FD, renal and cardiac protection is warranted to prevent patients from deleterious manifestations of the disease. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Dose-response relationships for enzyme replacement therapy with imiglucerase/alglucerase in patients with Gaucher disease type 1

NARCIS (Netherlands)

Grabowski, Gregory A.; Kacena, Katherine; Cole, J. Alexander; Hollak, Carla E. M.; Zhang, Lin; Yee, John; Mistry, Pramod K.; Zimran, Ari; Charrow, Joel; vom Dahl, Stephan

2009-01-01

Purpose: To determine whether enzyme therapy with imiglucerase/ alglucerase demonstrates dose-response relationships with doses and disease parameters used in routine clinical practice for Gaucher disease type 1 patients. Methods: Analyses included all patients with Gaucher disease type 1 on enzyme

Parental experience of enzyme replacement therapy for Hunter syndrome.

Science.gov (United States)

Buraczewska, M; O'Leary, D; Walsh, O; Monavari, A; Crushell, E

2013-04-01

We aimed to establish the profile of Irish patients with Hunter Syndrome (Mucopolysaccharidosis type II, MPS II) receiving weekly intravenous Enzyme Replacement Therapy (ERT) with recombinant iduronate-2-sulfatase and to assess the social impact and parental opinion of ERT through the use of a parental questionnaire. Nine patients aged 3.5- 14 years have received a mean of 2 (range 0.5-3.5) years of ERT. Treatment was associated with clinical improvements from baseline in hepatosplenomegaly in 6/7 (85%) respiratory manifestations in 4/6 (67%) and a mean reduction in urinary glycosaminoglycan excretion of 62%. Changes noted by parents included increased energy 3/9 (33%) and softening of skin, hair and facial features 8/9 (89%). Parents report that seven hours weekly were spent on hospitalizations for ERT. Parental employment was adversely affected in 8 (89%) families. One day of school/preschool (20%) was lost every week for 8 (89%) children. All parents believed the benefits of ERT out-weigh the difficulties involved. All families would welcome the introduction of home based therapy. In conclusion the social and educational burden of hospital-based ERT on these children and their families is significant. The introduction of home-based therapy is likely to improve overall quality of life for MPSII patients and their families.

Enzyme replacement therapy for Fabry disease: some answers but more questions

Directory of Open Access Journals (Sweden)

Majid Alfadhel

2011-02-01

Full Text Available Majid Alfadhel1, Sandra Sirrs21Division of Biochemical Diseases, Department of Paediatrics, BC Children’s and Women’s Hospital, University of British Columbia, Vancouver, BC, Canada; 2Adult Metabolic Diseases Clinic, Division of Endocrinology, Department of Medicine, Vancouver Hospital and Health Sciences Centre, University of British Columbia, Vancouver, BC, CanadaAbstract: Fabry disease (FD is a multisystem, X-linked disorder of glycosphingolipid metabolism caused by enzyme deficiency of α-galactosidase A. Affected patients have symptoms including acroparesthesias, angiokeratomas, and hypohidrosis. More serious manifestations include debilitating pain and gastrointestinal symptoms, proteinuria and gradual deterioration of renal function leading to end-stage renal disease, hypertrophic cardiomyopathy, and stroke. Heterozygous females may have symptoms as severe as males with the classic phenotype. Before 2001, treatment of patients with FD was supportive. The successful development of enzyme replacement therapy (ERT has been a great advancement in the treatment of patients with FD and can stabilize renal function and cardiac size, as well as improve pain and quality of life of patients with FD. In this review, we have provided a critical appraisal of the literature on the effects of ERT for FD. This analysis shows that data available on the treatment of FD are often derived from studies which are not controlled, rely on surrogate markers, and are of insufficient power to detect differences on hard clinical endpoints. Further studies of higher quality are needed to answer the questions that remain concerning the efficacy of ERT for FD.Keywords: Fabry disease, agalsidase α, agalsidase β, Replagal, Fabrazyme, critical appraisal, evidence-based medicine

Elosulfase alfa enzyme replacement therapy attenuates disease progression in a non-ambulatory Japanese patient with Morquio A syndrome (case report

Directory of Open Access Journals (Sweden)

Misako Hiramatsu

2017-12-01

Full Text Available Enzyme replacement therapy (ERT with elosulfase alfa is the only approved therapy in Japan for patients with Morquio A syndrome, a lysosomal storage disorder inherited in an autosomal recessive fashion. The experience with ERT in severely affected, non-ambulatory patients has not been reported in previous studies. This case report describes clinical evidence for the 1-year efficacy and safety of ERT with elosulfase alfa in a severely affected, non-ambulatory, 47-year-old patient with Morquio A syndrome who needs intensive respiratory management. ERT with elosulfase alfa was well tolerated in this patient. Because of the possibility of potential hypersensitivity adverse events, special attention is needed when using ERT in patients with respiratory disorders. However, under the appropriate management of specialists, the patient in this case report showed significant respiratory improvement after starting ERT, and abdominal bloating was improved by gas evacuation. In addition, the patient was able to lift up her arms, reach behind her back, and move her legs slightly, and she recovered her grip strength. Her hearing loss improved and she could hear without a hearing aid. This report shows that ERT with elosulfase alfa can be used with appropriate respiratory care in patients with severe respiratory dysfunction.

Does early use of enzyme replacement therapy alter the natural history of mucopolysaccharidosis I? Experience in three siblings.

Science.gov (United States)

Laraway, Sarah; Breen, Catherine; Mercer, Jean; Jones, Simon; Wraith, James E

2013-07-01

Enzyme replacement therapy is widely used as treatment for mucopolysaccharidosis I (MPS I), and there is evidence that this produces improvement in certain clinical domains. There does appear to be variation in the response of clinical features to treatment once these are established. In a reported sibling pair, when enzyme replacement therapy was commenced pre-symptomatically in the younger child, the natural history of the condition appeared to be affected. We present data from three siblings treated with enzyme replacement therapy at different ages which supports this finding. Copyright © 2013 Elsevier Inc. All rights reserved.

Replacement of traditional seawater-soluble pigments by starch and hydrolytic enzymes in polishing antifouling coatings

DEFF Research Database (Denmark)

Olsen, Søren Martin; Pedersen, L. T.; Dam-Johansen, Kim

2010-01-01

The use of starch and hydrolytic enzymes as replacement for traditional polishing pigments (e.g., Cu2O and ZnO) in antifouling coatings has been investigated. The enzymes facilitate a slow conversion of water-insoluble starch into water-soluble glucose, and dissolution of glucose causes the devel......The use of starch and hydrolytic enzymes as replacement for traditional polishing pigments (e.g., Cu2O and ZnO) in antifouling coatings has been investigated. The enzymes facilitate a slow conversion of water-insoluble starch into water-soluble glucose, and dissolution of glucose causes...... the development of a leached (porous) layer in the wetted, outermost part of the coating. Subsequent water-binder interaction at the pore walls gives rise to polishing, in a manner similar to that of conventional antifouling coatings. Different starch types have been evaluated and classified as potential coating...

Oral pharmacological chaperone migalastat compared with enzyme replacement therapy in Fabry disease

DEFF Research Database (Denmark)

Hughes, Derralynn A.; Nicholls, Kathleen; Shankar, Suma P.

2017-01-01

Background Fabry disease is an X-linked lysosomal storage disorder caused by GLA mutations, resulting in α-galactosidase (α-Gal) deficiency and accumulation of lysosomal substrates. Migalastat, an oral pharmacological chaperone being developed as an alternative to intravenous enzyme replacement t...

Guidelines to start enzyme replacement therapy in classic Fabry Disease patients in Latin America

Directory of Open Access Journals (Sweden)

Juan Manuel Politei

2017-01-01

Full Text Available Introduction: Fabry disease is a rare inherited X-linked disorder resulting from the absence or deficient activity of the α-galactosidase A enzyme. Objetive: To provide the first guideline on the best time to start enzyme replacement therapy to treat classic Fabry disease, based on the knowledge and experience of experts from ten Latin American countries: Argentina, Brazil, Colombia, Costa Rica, Chile, Ecuador, Mexico, Peru, Uruguay and Venezuela. Methods: The project coordinator designed a survey based on the criteria for starting the treatment which are established in different international guidelines published to date. This document was later sent to all the participants for its evaluation. Results: Fifty experts responded to the survey, whose criteria was divided into 5 sections according to specialty, and they arrived at a consensus. Discussion: The criteria for an early treatment were defined given the growing evidence of a better response and prognosis associated with it. Conclusion: We believe that the importance of this guideline relies on the participation of experts from ten Latin American countries. However, as it deals with a systemic disease whose physiopathological mechanisms and complications are still being described, some manifestations have not been included in the criteria, making it necessary to revise this guideline in order to report any changes that may arise in the future.

Hair cortisol content in patients with adrenal insufficiency on hydrocortisone replacement therapy.

Science.gov (United States)

Gow, Rachel; Koren, Gideon; Rieder, Michael; Van Uum, Stan

2011-06-01

Patients with adrenal insufficiency (AI) require life-long replacement therapy with exogenous glucocorticoids. Several studies have shown impaired subjective health status in these patients as well as increased morbidity and mortality risk, which may be caused by glucocorticoid over-replacement. As a measure of long-term cortisol exposure, the usefulness of hair cortisol analysis in patients receiving glucocorticoid replacement therapy was investigated. Hair samples, demographics, medical history and perceived stress scale questionnaires were collected from 93 patients across North America diagnosed with primary or secondary AI. Sixty-two household partners served as a control group. Cortisol was measured in the proximal 2 cm of hair, representing the most recent 2 months of exposure. A modified enzyme immunoassay was used for the measurement of cortisol. The male patients had significantly higher hair cortisol levels than the male controls (P cortisol content correlated significantly with glucocorticoid dose (r = 0·3, P cortisol content correlates with hydrocortisone (HC) dose in patients with AI. Our results suggest that some AI patients may be over-treated and hence may be at risk for the adverse effects of cortisol. Measurement of HC in hair may become a useful monitoring tool for long-term cortisol exposure in patients treated with glucocorticoids. © 2011 Blackwell Publishing Ltd.

Effect of enzyme replacement therapy with alglucosidase alfa (Myozyme®) in 12 patients with advanced late-onset Pompe disease.

Science.gov (United States)

Papadopoulos, Constantinos; Orlikowski, David; Prigent, Hélène; Lacour, Arnaud; Tard, Céline; Furby, Alain; Praline, Julien; Solé, Guilhem; Hogrel, Jean-Yves; De Antonio, Marie; Semplicini, Claudio; Deibener-Kaminsky, Joelle; Kaminsky, Pierre; Eymard, Bruno; Taouagh, Nadjib; Perniconi, Barbara; Hamroun, Dalil; Laforêt, Pascal

2017-09-01

The efficacy of enzyme replacement therapy (ERT) in patients at an advanced stage of Pompe disease has only been addressed in a few studies. Our objective was to assess the long term effects of ERT in a cohort of patients with severe Pompe disease. We identified patients from the French Pompe Registry with severe respiratory failure and permanent wheelchair use (assisted walk for a few meters was allowed) when starting ERT. Patients' medical records were collected and reviewed and respiratory and motor functions, before ERT initiation and upon last evaluation were compared. Twelve patients (7 males) were identified. Median age at symptom onset was 24years [IQR=15.5; 36.0]. At baseline ventilation was invasive in 11 patients and noninvasive in one, with a median ventilation time of 24h [IQR=21.88; 24.00] (min 20; max 24). ERT was initiated at a median age of 52.5years [IQR=35.75; 66.50]. Median treatment duration was 55months [IQR=39.5; 81.0]. During observational period no adverse reaction to ERT was recorded, five patients (41.67%) died, three decreased their ventilation time by 30, 60 and 90min and two increased their assisted walking distance, by 80 and 20m. Some patients at a very advanced stage of Pompe disease may show a mild benefit from ERT, in terms of increased time of autonomous ventilation and of enlarged distance in assisted walk. ERT can be initiated in these patients in order to retain their current level of independence and ability to perform daily life activities. Copyright © 2017 Elsevier Inc. All rights reserved.

Consequence of patient substitution of nattokinase for warfarin after aortic valve replacement with a mechanical prosthesis.

Science.gov (United States)

Elahi, Maqsood M; Choi, Charles H; Konda, Subbareddy; Shake, Jay G

2015-01-01

This report describes a patient's self-substitution of nattokinase for the vitamin K antagonist warfarin after aortic valve replacement with a mechanical prosthesis. Nattokinase is an enzyme derived from a popular fermented soybean preparation in Japan (natto), which has fibrinolytic properties and is gaining popularity in nontraditional health journals and nonmedical health websites as an over-the-counter thrombolytic. After nearly a year of use of nattokinase without warfarin, the patient developed thrombus on the mechanical valve and underwent successful repeat valve replacement. We believe this is the first documented case of nattokinase being used as a substitute for warfarin after valve replacement, and we strongly discourage its use for this purpose.

Residual glycosaminoglycan accumulation in mitral and aortic valves of a patient with attenuated MPS I (Scheie syndrome after 6 years of enzyme replacement therapy: Implications for early diagnosis and therapy

Directory of Open Access Journals (Sweden)

Yohei Sato

2015-12-01

Full Text Available Mucopolysaccharidosis (MPS is an inherited metabolic disease caused by deficiency of the enzymes needed for glycosaminoglycan (GAG degradation. MPS type I is caused by the deficiency of the lysosomal enzyme alpha-l-iduronidase and is classified into Hurler syndrome, Scheie syndrome, and Hurler–Scheie syndrome based on disease severity and onset. Cardiac complications such as left ventricular hypertrophy, cardiac valve disease, and coronary artery disease are often observed in MPS type I. Enzyme replacement therapy (ERT has been available for MPS type I, but the efficacy of this treatment for cardiac valve disease is unknown. We report on a 56-year-old female patient with attenuated MPS I (Scheie syndrome who developed aortic and mitral stenosis and coronary artery narrowing. The cardiac valve disease progressed despite ERT and she finally underwent double valve replacement and coronary artery bypass grafting. The pathology of the cardiac valves revealed GAG accumulation and lysosomal enlargement in both the mitral and aortic valves. Zebra body formation was also confirmed using electron microscopy. Our results suggest that ERT had limited efficacy in previously established cardiac valve disease. Early diagnosis and initiation of ERT is crucial to avoid further cardiac complications in MPS type I.

Pancreatic Enzyme Replacement Therapy in Children with Severe Acute Malnutrition : A Randomized Controlled Trial

NARCIS (Netherlands)

Bartels, Rosalie H.; Bourdon, Celine; Potani, Isabel; Mhango, Brian; van den Brink, Deborah A.; Mponda, John S.; Kobold, Anneke C. Muller; Bandsma, Robert H.; van Hensbroek, Michael Boele; Voskuijl, Wieger P.

Objective: To assess the benefits of pancreatic enzyme replacement therapy (PERT) in children with complicated severe acute malnutrition. Study design: We conducted a randomized, controlled trial in 90 children aged 6-60 months with complicated severe acute malnutrition at the Queen Elizabeth

Pancreatic Enzyme Replacement Therapy in Children with Severe Acute Malnutrition: A Randomized Controlled Trial

NARCIS (Netherlands)

Bartels, Rosalie H.; Bourdon, Céline; Potani, Isabel; Mhango, Brian; van den Brink, Deborah A.; Mponda, John S.; Muller Kobold, Anneke C.; Bandsma, Robert H.; Boele van Hensbroek, Michael; Voskuijl, Wieger P.

2017-01-01

Objective To assess the benefits of pancreatic enzyme replacement therapy (PERT) in children with complicated severe acute malnutrition. Study design We conducted a randomized, controlled trial in 90 children aged 6-60 months with complicated severe acute malnutrition at the Queen Elizabeth Central

Children with Pompe disease: clinical characteristics, peculiar features and effects of enzyme replacement therapy

NARCIS (Netherlands)

C.I. van Capelle (Carine)

2014-01-01

markdownabstract__Abstract__ Pompe disease is a metabolic myopathy. Since the first description of the disease in 1932 by J.C. Pompe,1 tremendous progress has been made from discovering the biochemical and genetic basis of the disease to developing enzyme replacement therapy (ERT). With this

Patients Unicondylar Knee Replacement vs. Total Knee Replacement

OpenAIRE

Hedra Eskander

2017-01-01

The aim of this review article is to analyse the clinical effectiveness of total knee replacement (TKR) compared to unicondylar knee replacement (UKR) on patients. In terms of survival rates, revision rates and postoperative complications. The keywords used were: knee arthroplasty. Nearly three thousand articles were found on 25 August 2016. Of those, only twenty-five were selected and reviewed because they were strictly focused on the topic of this article. Compared with those who have TKR, ...

Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement

International Nuclear Information System (INIS)

Poll, L.W.; Koch, J.A.; Boerner, D.; Cohnen, M.; Jung, G.; Scherer, A.; Moedder, U.; Niederau, C.

2001-01-01

Purpose: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluating bone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. Materials and Methods: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T 1 /T 2 ) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. Results: Infiltration of bm by Gaucher cells is characterized by a reduction of Sl on both T 1 - and T 2 -weighted sequences. Bone marrow responses were seen in 19 patients (63%) during treatment. Focal bone lesions, surrounded by a rim of reduced Sl, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. (orig.) [de

Consequence of patient substitution of nattokinase for warfarin after aortic valve replacement with a mechanical prosthesis

OpenAIRE

Elahi, Maqsood M.; Choi, Charles H.; Konda, Subbareddy; Shake, Jay G.

2015-01-01

This report describes a patient's self-substitution of nattokinase for the vitamin K antagonist warfarin after aortic valve replacement with a mechanical prosthesis. Nattokinase is an enzyme derived from a popular fermented soybean preparation in Japan (natto), which has fibrinolytic properties and is gaining popularity in nontraditional health journals and nonmedical health websites as an over-the-counter thrombolytic. After nearly a year of use of nattokinase without warfarin, the patient d...

Fabry disease: the importance of the enzyme replacement therapy (TRE, treating quickly and efficiently

Directory of Open Access Journals (Sweden)

Juan Manuel Politei

2014-06-01

Full Text Available Fabry Disease is a lysosomal disorder due to the absence or deficiency of the Alpha galactosidase A enzyme that causes a pathological accumulation of glycosphingolipids mainly in the endothelial cells, vascular smooth muscle cells and podocytes among others. Enzyme replacement therapy is the only option for a specific treatment at present. Increasing knowledge of the physiopathological mechanisms has changed the management of the disease and above all, when treatment should begin. At present, beginning treatment at an early age seems to be a way of preventing and in some cases reverting some of the signs and symptoms of Fabry disease.

Extended interval between enzyme therapy infusions for adult patients with Gaucher′s disease type 1.

Directory of Open Access Journals (Sweden)

Pérez-Calvo J

2003-01-01

Full Text Available BACKGROUND: Enzyme replacement therapy (ERT for Gaucher′s disease with alglucerase or imiglucerase is efficacious, well-tolerated and safe. However, cost considerations, visits to medical facilities, potentially duration of theray for life, are issues of major concern to a proportion of treated patients and has, in some cases, led to the withdrawal of therapy. AIMS: To elucidate whether an extension of the interval between enzyme infusions to once every three weeks is as effective in maintaining the clinical responses achieved with the bi-monthly regimen. MATERIALS AND METHODS: Four patients with an optimal response to ERT (at 30 units/kg every two weeks for an average of 27 months, were subjected to enzyme dose/frequency changes that essentially constituted a reduction in cumulative dose over the treatment period. Patients were assessed every 6 months for alterations in haematological parameters, plasma chitotriosidase levels, liver and spleen size, and bone symptoms. RESULTS: All patients had to resume the previous infusion schedule of once every two weeks; one because of new bone marrow infiltrates, two because of visceral enlargement, and the fourth due to progressive anaemia. CONCLUSIONS: This limited experience suggests that a reduction in enzyme dose associated with an extended interval between infusions may lead to variable disease control, and underscores the need for individualization of enzyme therapy.

Efficacy of pancreatic enzyme replacement therapy in chronic pancreatitis: systematic review and meta-analysis.

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de la Iglesia-García, Daniel; Huang, Wei; Szatmary, Peter; Baston-Rey, Iria; Gonzalez-Lopez, Jaime; Prada-Ramallal, Guillermo; Mukherjee, Rajarshi; Nunes, Quentin M; Domínguez-Muñoz, J Enrique; Sutton, Robert

2017-08-01

The benefits of pancreatic enzyme replacement therapy (PERT) in chronic pancreatitis (CP) are inadequately defined. We have undertaken a systematic review and meta-analysis of randomised controlled trials of PERT to determine the efficacy of PERT in exocrine pancreatic insufficiency (EPI) from CP. Major databases were searched from 1966 to 2015 inclusive. The primary outcome was coefficient of fat absorption (CFA). Effects of PERT versus baseline and versus placebo, and of different doses, formulations and schedules were determined. A total of 17 studies (511 patients with CP) were included and assessed qualitatively (Jadad score). Quantitative data were synthesised from 14 studies. PERT improved CFA compared with baseline (83.7±6.0 vs 63.1±15.0, pregression analyses revealed that sample size, CP diagnostic criteria, study design and enzyme dose contributed to heterogeneity; data on health inequalities were lacking. PERT is indicated to correct EPI and malnutrition in CP and may be improved by higher doses, enteric coating, administration during food and acid suppression. Further studies are required to determine optimal regimens, the impact of health inequalities and long-term effects on nutrition. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Modelling Gaucher disease progression: long-term enzyme replacement therapy reduces the incidence of splenectomy and bone complications

NARCIS (Netherlands)

van Dussen, Laura; Biegstraaten, Marieke; Dijkgraaf, Marcel Gw; Hollak, Carla Em

2014-01-01

Long-term complications and associated conditions of type 1 Gaucher Disease (GD) can include splenectomy, bone complications, pulmonary hypertension, Parkinson disease and malignancies. Enzyme replacement therapy (ERT) reverses cytopenia and reduces organomegaly. To study the effects of ERT on

The influence of NSAIDs on coumarin sensitivity in patients with CYP2C9 polymorphism after total hip replacement surgery.

NARCIS (Netherlands)

Beinema, M.J.; Jong, P.H. de; Salden, H.J.; Wijnen, M.H.W.A.; Meer, J.W.M. van der; Brouwers, J.R.B.J.

2007-01-01

OBJECTIVE: To determine the influence of NSAIDs on the international normalized ratio (INR) in patients with cytochrome P450 (CYP)-2C9 enzyme variants starting acenocoumarol (an oral coumarin) therapy during the first 7 days after total hip replacement surgery. METHODS: In this prospective study, an

The influence of NSAIDs on coumarin sensitivity in patients with CYP2C9 polymorphism after total hip replacement surgery

NARCIS (Netherlands)

Beinema, Maarten J.; de Jong, Petra H.; Salden, Har J. M.; van Wijnen, Merel; van der Meer, Jan; Brouwers, Jacobus R. B. J.

2007-01-01

Objective: To determine the influence of NSAIDs on the international normalized ratio (INR) in patients with cytochrome P450 (CYP)-2C9 enzyme variants starting acenocoumarol (an oral coumarin) therapy during the first 7 days after total hip replacement surgery. Methods: In this prospective study, an

Insulin dependence and pancreatic enzyme replacement therapy are independent prognostic factors for long-term survival after operation for chronic pancreatitis.

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Winny, Markus; Paroglou, Vagia; Bektas, Hüseyin; Kaltenborn, Alexander; Reichert, Benedikt; Zachau, Lea; Kleine, Moritz; Klempnauer, Jürgen; Schrem, Harald

2014-02-01

This retrospective, single-center, observational study on postoperative long-term results aims to define yet unknown factors for long-term outcome after operation for chronic pancreatitis. We analyzed 147 consecutive patients operated for chronic pancreatitis from 2000 to 2011. Mean follow-up was 5.3 years (range, 1 month to 12.7 years). Complete long-term survival data were provided by the German citizen registration authorities for all patients. A quality-of-life questionnaire was sent to surviving patients after a mean follow-up of 5.7 years. Surgical principles were resection (n = 86; 59%), decompression (n = 29; 20%), and hybrid procedures (n = 32; 21%). No significant influences of different surgical principles and operative procedures on survival, long-term quality of life and pain control could be detected. Overall 30-day mortality was 2.7%, 1-year survival 95.9%, and 3-year survival 90.8%. Multivariate Cox regression analysis revealed that only postoperative insulin dependence at the time of hospital discharge (P = .027; Exp(B) = 2.111; 95% confidence interval [CI], 1.089-4.090) and the absence of pancreas enzyme replacement therapy at the time of hospital discharge (P = .039; Exp(B) = 2.102; 95% CI, 1.037-4.262) were significant, independent risk factors for survival with significant hazard ratios for long-term survival. Long-term improvement in quality of life was reported by 55 of 76 long-term survivors (73%). Pancreatic enzyme replacement should be standard treatment after surgery for chronic pancreatitis at the time of hospital discharge, even when no clinical signs of exocrine pancreatic failure exist. This study underlines the potential importance of early operative intervention in chronic pancreatitis before irreversible endocrine dysfunction is present. Copyright © 2014 Mosby, Inc. All rights reserved.

Potential efficacy of enzyme replacement and substrate reduction therapy in three siblings with Gaucher disease type III

NARCIS (Netherlands)

Cox-Brinkman, J.; van Breemen, M. J.; van Maldegem, B. T.; Bour, L.; Donker, W. E.; Hollak, C. E. M.; Wijburg, F. A.; Aerts, J. M. F. G.

2008-01-01

We report three siblings with Gaucher disease type III, born between 1992 and 2004. During this period, new developments resulted in different potential therapies, changing clinical practice. The two eldest siblings received enzyme replacement therapy (ERT) from the age of 24 and 5 months

[Valve-sparing Replacement in Patients with Aortic Root Dilatation].

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Yamazaki, Kazuhiro; Minatoya, Kenji; Ueda, Ryoma; Takehara, Masato; Sakamoto, Kazuhisa; Ide, Yujiro; Kanemitsu, Hideo; Ueyama, Koji; Ikeda, Tadashi

2018-01-01

Valve-sparing root replacement is increasingly used to overcome drawbacks associated with valvular prostheses. In our institution, 7 patients underwent valve-sparing root replacement from August 2016 to July 2017. The mean age was 45 years (range, 14~69 years). Three patients had Marfan syndrome and 1 had Loeys-Dietz syndrome with acute aortic dissection. All patients underwent surgery with reimplantation technique using a Valsalva graft. Two patients required repair of aortic valve leaflet prolapse. All patients had an excellent clinical course, with mild or no aortic regurgitation and a decrease in end-diastolic volume on echocardiography. These results support the continued use of valve-sparing root replacement in selected patients.

Pancreatic enzyme replacement therapy for pancreatic exocrine insufficiency in the 21(st) century.

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Trang, Tony; Chan, Johanna; Graham, David Y

2014-09-07

Restitution of normal fat absorption in exocrine pancreatic insufficiency remains an elusive goal. Although many patients achieve satisfactory clinical results with enzyme therapy, few experience normalization of fat absorption, and many, if not most, will require individualized therapy. Increasing the quantity of lipase administered rarely eliminates steatorrhea but increases the cost of therapy. Enteric coated enzyme microbead formulations tend to separate from nutrients in the stomach precluding coordinated emptying of enzymes and nutrients. Unprotected enzymes mix well and empty with nutrients but are inactivated at pH 4 or below. We describe approaches for improving the results of enzyme therapy including changing to, or adding, a different product, adding non-enteric coated enzymes, (e.g., giving unprotected enzymes at the start of the meal and acid-protected formulations later), use of antisecretory drugs and/or antacids, and changing the timing of enzyme administration. Because considerable lipid is emptied in the first postprandial hour, it is prudent to start therapy with enteric coated microbead prior to the meal so that some enzymes are available during that first hour. Patients with hyperacidity may benefit from adjuvant antisecretory therapy to reduce the duodenal acid load and possibly also sodium bicarbonate to prevent duodenal acidity. Comparative studies of clinical effectiveness of different formulations as well as the characteristics of dispersion, emptying, and dissolution of enteric-coated microspheres of different diameter and density are needed; many such studies have been completed but not yet made public. We discuss the history of pancreatic enzyme therapy and describe current use of modern preparations, approaches to overcoming unsatisfactory clinical responses, as well as studies needed to be able to provide reliably effective therapy.

Pancreatic enzyme replacement therapy for pancreatic exocrine insufficiency in the 21st century

Science.gov (United States)

Trang, Tony; Chan, Johanna; Graham, David Y

2014-01-01

Restitution of normal fat absorption in exocrine pancreatic insufficiency remains an elusive goal. Although many patients achieve satisfactory clinical results with enzyme therapy, few experience normalization of fat absorption, and many, if not most, will require individualized therapy. Increasing the quantity of lipase administered rarely eliminates steatorrhea but increases the cost of therapy. Enteric coated enzyme microbead formulations tend to separate from nutrients in the stomach precluding coordinated emptying of enzymes and nutrients. Unprotected enzymes mix well and empty with nutrients but are inactivated at pH 4 or below. We describe approaches for improving the results of enzyme therapy including changing to, or adding, a different product, adding non-enteric coated enzymes, (e.g., giving unprotected enzymes at the start of the meal and acid-protected formulations later), use of antisecretory drugs and/or antacids, and changing the timing of enzyme administration. Because considerable lipid is emptied in the first postprandial hour, it is prudent to start therapy with enteric coated microbead prior to the meal so that some enzymes are available during that first hour. Patients with hyperacidity may benefit from adjuvant antisecretory therapy to reduce the duodenal acid load and possibly also sodium bicarbonate to prevent duodenal acidity. Comparative studies of clinical effectiveness of different formulations as well as the characteristics of dispersion, emptying, and dissolution of enteric-coated microspheres of different diameter and density are needed; many such studies have been completed but not yet made public. We discuss the history of pancreatic enzyme therapy and describe current use of modern preparations, approaches to overcoming unsatisfactory clinical responses, as well as studies needed to be able to provide reliably effective therapy. PMID:25206255

Case report of unexpected gastrointestinal involvement in type 1 Gaucher disease: comparison of eliglustat tartrate treatment and enzyme replacement therapy.

Science.gov (United States)

Kim, Yoo-Mi; Shin, Dong Hoon; Park, Su Bum; Cheon, Chong Kun; Yoo, Han-Wook

2017-05-15

Gastrointestinal involvement in Gaucher disease is very rare, and appears to be unresponsive to enzyme replacement therapy (ERT). Here, we describe identical twin, splenectomized, non-neuronopathic Gaucher patients on long-term ERT for 9 years, who complained of epigastric discomfort due to Gaucher cell infiltration of the gastroduodenal mucosa. Rare compound heterozygous mutations (p.Arg48Trp and p.Arg257Gln) of the GBA gene were found in both. Improvement in the gastroduodenal infiltration and reduced chitotriosidase levels were observed in one who switched to eliglustat tartrate for 1 year, whereas the other one who maintained ERT showed no improvement of chitotriosidase level and persistent duodenal lesions. This shows that eliglustat might be an effective treatment for Gaucher disease patients having lesions resistant to ERT.

Towards the development of an enzyme replacement therapy for the metabolic disorder propionic acidemia

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Mahnaz Darvish-Damavandi

2016-09-01

Full Text Available Propionic acidemia (PA is a life-threatening disease caused by the deficiency of a mitochondrial biotin-dependent enzyme known as propionyl coenzyme-A carboxylase (PCC. This enzyme is responsible for degrading the metabolic intermediate, propionyl coenzyme-A (PP-CoA, derived from multiple metabolic pathways. Currently, except for drastic surgical and dietary intervention that can only provide partial symptomatic relief, no other form of therapeutic option is available for this genetic disorder. Here, we examine a novel approach in protein delivery by specifically targeting and localizing our protein candidate of interest into the mitochondrial matrix of the cells. In order to test this concept of delivery, we have utilized cell penetrating peptides (CPPs and mitochondria targeting sequences (MTS to form specific fusion PCC protein, capable of translocating and localizing across cell membranes. In vitro delivery of our candidate fusion proteins, evaluated by confocal images and enzymatic activity assay, indicated effectiveness of this strategy. Therefore, it holds immense potential in creating a new paradigm in site-specific protein delivery and enzyme replacement therapeutic for PA.

Evaluation of treatment response to enzyme replacement therapy with Velaglucerase alfa in patients with Gaucher disease using whole-body magnetic resonance imaging.

Science.gov (United States)

Laudemann, K; Moos, L; Mengel, E; Lollert, A; Hoffmann, C; Brixius-Huth, M; Wagner, D; Düber, C; Staatz, G

2016-03-01

This was a retrospective data analysis to evaluate the treatment response to enzyme replacement therapy (ERT) with Velaglucerase alfa using whole-body magnetic resonance imaging (MRI). A baseline and follow-up MRI were performed on 18 Gaucher Type 1 patients at an interval of 11.6 months. The MRI score systems determined the Bone-Marrow-Burden (BMB) score, the Düsseldorf-Gaucher score (DGS), and the Vertebra-Disc-Ratio (VDR). The Severity Score Index Type 1 (GD-DS3) was also assessed. The baseline MRI medians were: BMB, 7.00; DGS, 3.00; and VDR: 1.70; while, the follow-up MRI medians were: BMB, 7.00; DGS, 3.00; and VDR: 1.73. The baseline GD-DS3 median was 2.40 (BMB excl.: 0.50) and the follow-up median was 2.00 (BMB excl.: 0.50). There was weak statistical significance with the Wilcoxon signed-rank test for the DGS (p=0.034) and GD-DS3 (p=0.047) between both MRIs. Velaglucerase alfa therapy is a effective long-term treatment for Gaucher Type 1 patients who are newly diagnosed or switching therapies. Measurements with whole-body MRI and an objective scoring system were reliable tools for detecting early stage bone marrow activity. Further research is needed to evaluate the "Booster-Effect" of Velaglucerase alfa therapy in Gaucher skeletal disease. Copyright © 2015 Elsevier Inc. All rights reserved.

Enzyme replacement therapy for murine hypophosphatasia.

Science.gov (United States)

Millán, José Luis; Narisawa, Sonoko; Lemire, Isabelle; Loisel, Thomas P; Boileau, Guy; Leonard, Pierre; Gramatikova, Svetlana; Terkeltaub, Robert; Camacho, Nancy Pleshko; McKee, Marc D; Crine, Philippe; Whyte, Michael P

2008-06-01

Hypophosphatasia (HPP) is the inborn error of metabolism that features rickets or osteomalacia caused by loss-of-function mutation(s) within the gene that encodes the tissue-nonspecific isozyme of alkaline phosphatase (TNALP). Consequently, natural substrates for this ectoenzyme accumulate extracellulary including inorganic pyrophosphate (PPi), an inhibitor of mineralization, and pyridoxal 5'-phosphate (PLP), a co-factor form of vitamin B6. Babies with the infantile form of HPP often die with severe rickets and sometimes hypercalcemia and vitamin B6-dependent seizures. There is no established medical treatment. Human TNALP was bioengineered with the C terminus extended by the Fc region of human IgG for one-step purification and a deca-aspartate sequence (D10) for targeting to mineralizing tissue (sALP-FcD10). TNALP-null mice (Akp2-/-), an excellent model for infantile HPP, were treated from birth using sALP-FcD10. Short-term and long-term efficacy studies consisted of once daily subcutaneous injections of 1, 2, or 8.2 mg/kg sALP-FcD10 for 15, 19, and 15 or 52 days, respectively. We assessed survival and growth rates, circulating levels of sALP-FcD10 activity, calcium, PPi, and pyridoxal, as well as skeletal and dental manifestations using radiography, microCT, and histomorphometry. Akp2-/- mice receiving high-dose sALP-FcD10 grew normally and appeared well without skeletal or dental disease or epilepsy. Plasma calcium, PPi, and pyridoxal concentrations remained in their normal ranges. We found no evidence of significant skeletal or dental disease. Enzyme replacement using a bone-targeted, recombinant form of human TNALP prevents infantile HPP in Akp2-/- mice.

The Effect of Hypotensive Anesthesia on Hepatic Function in Hip Replacement

OpenAIRE

Zagrekov V.I.; Zhirova T.A.; Ezhov I.Y.; Taranyuk А.V.

2011-01-01

The objective of the work is to assess the influence of spinal and epidural anesthesia with controlled hypotensive effect on hepatic function in patients in total hip replacement. Materials and Methods. There has been studied the dynamics of hepatic enzymes and bilirubin indexes in 80 patients in hip replacement. Depending on the anesthesia method, three groups were considered: with spinal and epidural anesthesia with controlled hypotensive effect and normotensive spinal anesthesia using ...

Enzyme Replacement Therapy for Murine Hypophosphatasia*

Science.gov (United States)

Millán, José Luis; Narisawa, Sonoko; Lemire, Isabelle; Loisel, Thomas P; Boileau, Guy; Leonard, Pierre; Gramatikova, Svetlana; Terkeltaub, Robert; Camacho, Nancy Pleshko; McKee, Marc D; Crine, Philippe; Whyte, Michael P

2008-01-01

Introduction Hypophosphatasia (HPP) is the inborn error of metabolism that features rickets or osteomalacia caused by loss-of-function mutation(s) within the gene that encodes the tissue-nonspecific isozyme of alkaline phosphatase (TNALP). Consequently, natural substrates for this ectoenzyme accumulate extracellulary including inorganic pyrophosphate (PPi), an inhibitor of mineralization, and pyridoxal 5`-phosphate (PLP), a co-factor form of vitamin B6. Babies with the infantile form of HPP often die with severe rickets and sometimes hypercalcemia and vitamin B6-dependent seizures. There is no established medical treatment. Materials and Methods Human TNALP was bioengineered with the C terminus extended by the Fc region of human IgG for one-step purification and a deca-aspartate sequence (D10) for targeting to mineralizing tissue (sALP-FcD10). TNALP-null mice (Akp2−/−), an excellent model for infantile HPP, were treated from birth using sALP-FcD10. Short-term and long-term efficacy studies consisted of once daily subcutaneous injections of 1, 2, or 8.2 mg/kg sALP-FcD10 for 15, 19, and 15 or 52 days, respectively. We assessed survival and growth rates, circulating levels of sALP-FcD10 activity, calcium, PPi, and pyridoxal, as well as skeletal and dental manifestations using radiography, μCT, and histomorphometry. Results Akp2−/− mice receiving high-dose sALP-FcD10 grew normally and appeared well without skeletal or dental disease or epilepsy. Plasma calcium, PPi, and pyridoxal concentrations remained in their normal ranges. We found no evidence of significant skeletal or dental disease. Conclusions Enzyme replacement using a bone-targeted, recombinant form of human TNALP prevents infantile HPP in Akp2−/− mice. PMID:18086009

TOTAL KNEE REPLACEMENT IN PATIENTS WITH BELOW-KNEE AMPUTATION

Science.gov (United States)

Karam, Matthew D; Willey, Michael; Shurr, Donald G

2010-01-01

Total knee replacement (TKR) is reserved for patients with severe and disabling arthritis that is non-responsive to conservative measures. Based on existing data, total knee replacement is a safe and cost-effective treatment for alleviating pain and improving physical function in patients who do not respond to conservative therapy. Despite the large variation in health status of patients and types of prosthesis implanted, total knee replacement has proven to be a relatively low risk and successful operation. Each year in the United States surgeons perform approximately 300,000 TKR.1 Likewise, lower extremity amputation is commonly performed in the United States with an annual incidence of 110,000 per year.2 Nearly 70% of all lower extremity amputations are performed as the result of chronic vascular disease, followed by trauma (22%), congenital etiology and tumor (4% each).3 Approximately 50% of all lower extremity amputations are performed secondary to complications from Diabetes Mellitus. Norvell et al. demonstrated that patients who have previously undergone transtibial amputation and ambulate with a prosthesis are more likely to develop degenerative joint disease in the con-tralateral extremity than the ipsilateral extremity.4 Further, radiographic changes consistent with osteoporosis have been demonstrated in up to 88% of limbs that have undergone transtibial amputation.8 To our knowledge, there have been only three reported cases of total knee replacement in patients with ipsilateral transtibial amputation.5,7 The purpose of the present study is to review the existing data on total knee replacement in patients who have undergone transtibial amputation. Further we present a patient with a transtibial amputation who underwent contralateral total knee replacement. PMID:21045987

Pancreatic Enzyme Replacement Therapy in Children with Severe Acute Malnutrition: A Randomized Controlled Trial.

Science.gov (United States)

Bartels, Rosalie H; Bourdon, Céline; Potani, Isabel; Mhango, Brian; van den Brink, Deborah A; Mponda, John S; Muller Kobold, Anneke C; Bandsma, Robert H; Boele van Hensbroek, Michael; Voskuijl, Wieger P

2017-11-01

To assess the benefits of pancreatic enzyme replacement therapy (PERT) in children with complicated severe acute malnutrition. We conducted a randomized, controlled trial in 90 children aged 6-60 months with complicated severe acute malnutrition at the Queen Elizabeth Central Hospital in Malawi. All children received standard care; the intervention group also received PERT for 28 days. Children treated with PERT for 28 days did not gain more weight than controls (13.7 ± 9.0% in controls vs 15.3 ± 11.3% in PERT; P = .56). Exocrine pancreatic insufficiency was present in 83.1% of patients on admission and fecal elastase-1 levels increased during hospitalization mostly seen in children with nonedematous severe acute malnutrition (P Children who died had low fecal fatty acid split ratios at admission. Exocrine pancreatic insufficiency was not improved by PERT, but children receiving PERT were more likely to be discharged with every passing day (P = .02) compared with controls. PERT does not improve weight gain in severely malnourished children but does increase the rate of hospital discharge. Mortality was lower in patients on PERT, a finding that needs to be investigated in a larger cohort with stratification for edematous and nonedematous malnutrition. Mortality in severe acute malnutrition is associated with markers of poor digestive function. ISRCTN.com: 57423639. Copyright © 2017 Elsevier Inc. All rights reserved.

The influence of a polymorphism in the gene encoding angiotensin converting enzyme (ACE on treatment outcomes in late-onset Pompe patients receiving alglucosidase alfa

Directory of Open Access Journals (Sweden)

Rena C. Baek

2016-09-01

Full Text Available Correlations between angiotensin-converting enzyme (ACE genotype (I/I, I/D, D/D, disease severity at baseline and response to enzyme replacement therapy (ERT were assessed in the Pompe disease Late-Onset Treatment Study (LOTS. No correlations were observed between ACE genotype and disease severity at baseline. However, D/D patients appeared to have a reduced response to alglucosidase alfa treatment than I/I or I/D patients, suggesting that ACE polymorphisms may influence the response to alglucosidase alfa treatment and warrants further investigation.

Valve-sparing and valve-replacing techniques for aortic root replacement in patients with Marfan syndrome: Analysis of early outcome.

Science.gov (United States)

Volguina, Irina V; Miller, D Craig; LeMaire, Scott A; Palmero, Laura C; Wang, Xing Li; Connolly, Heidi M; Sundt, Thoralf M; Bavaria, Joseph E; Dietz, Harry C; Milewicz, Dianna M; Coselli, Joseph S

2009-05-01

A prospective, international registry study was initiated to provide contemporary comparative data on short-term clinical outcomes after aortic valve-sparing and aortic valve-replacing root operations in patients with Marfan syndrome. The purpose of this initial report is to describe the study design and to compare early outcomes in the first 151 enrolled patients. We assessed 30-day outcomes in 151 patients who met strict Ghent diagnostic criteria for Marfan syndrome and underwent aortic root replacement with either valve-replacing (n = 46) or valve-sparing techniques (n = 105) at one of 18 participating centers. In the valve replacement group, a mechanical composite valve graft was used in 39 (85%) patients and a bioprosthetic valve in 7 (15%). In the valve-sparing group, David V procedures were performed in 57 (54%) patients, David I in 38 (36%), David IV in 8 (8%), Florida sleeve in 1 (1%), and Yacoub remodeling in 1 (1%). No in-hospital or 30-day deaths occurred. Despite longer crossclamp and cardiopulmonary bypass times in the valve-sparing group, there were no significant between-group differences in postoperative complications. Thirty-day valve-related complications occurred in 2 (4%) patients undergoing valve replacement and in 3 (3%) undergoing valve-sparing procedures (P = .6). The analysis of early outcomes revealed that valve-sparing techniques were the most common approach to root replacement in patients with Marfan syndrome in these centers. The complexity of valve-sparing root replacement did not translate into any demonstrable adverse early outcomes. Subsequent analysis will compare the 3-year durability of these two surgical approaches.

Effects of replacing groundnut cake with rumen content supplemented with or without enzyme in the diet of weaner rabbits.

Science.gov (United States)

Adeniji, A A; Rumak, S; Oluwafemi, R A

2015-12-18

Rabbits are also herbivores which efficiently convert fodder to food. They are prolific and converter of plant proteins of little or no use to people as food into high-value animal protein. Rabbit meat is high in protein, low in calories and low in fat and cholesterol contents, being considered as a delicacy and a healthy food product. Feeding rabbits with concentrates is expensive and therefore in order to reduce cost of production, hence the use of rumen content in this study as alternative feedstuff without competition. A total of thirty six (36) weaner rabbits (oryctalagus cuniculus) of different body weight and age where use in this experiment to determine the effects of replacing rumen content with or without enzyme supplementation for groundnut cake. This feeding trial which lasted for 8 weeks was carried out in order to determine the replacement value of groundnut cake with rumen content with or without enzyme in the diet of weaner rabbit. A 3x2 factorial experiment was adopted such that there where three (3) replacement level of rumen content (0, 25 and 50 %) for groundnut cake by two supplemental level (no enzyme and enzyme supplement). The results showed that increased inclusion level of rumen content has significant effects (p  0.05) with weight gained value of 7.62,7.44 and 7.36 g respectively. Similarly there was a significant (p  0.05) of the treatment on urinary nitrogen. Significant (p  0.05) of the supplementation on the nitrogen intake. The interaction between the varying levels of rumen content supplementation had significant effects (p < 0.05) on the feacal nitrogen, feed intake and feed to gain ratio but no significant (p < 0.05) effects on interaction of nitrogen intake. In conclusion, since the results from this study showed no negative effects on the performance of the experimental animals, the test ingredient can be used as alternative feedstuff at a lower inclusion level so as to reduce production cost and expand

Bone marrow involvement in Gaucher disease at MRI: what long-term evolution can we expect under enzyme replacement therapy?

International Nuclear Information System (INIS)

Fedida, Benjamin; Touraine, Sebastien; Laredo, Jean-Denis; Stirnemann, Jerome; Belmatoug, Nadia; Petrover, David

2015-01-01

To study the long-term evolution of the bone marrow burden (BMB) score at MRI in patients with Gaucher disease (GD) under enzyme replacement therapy (ERT). Forty patients treated for GD were retrospectively studied in a referral centre. BMB scores were assessed on spine and femur MR examinations performed between January 2003 and June 2014. The long-term evolution of the BMB scores was analyzed using a linear mixed model. A total of 121 MRI examinations were performed during the study period with a mean follow-up of 7.1 years ± 5.6, an average rate of 3.1 MR examinations ± 1.7 per patient and an interval of 2.3 years ± 1.1 between examinations. Patients had received ERT during 12 years on average ± 6.7. The trend of BMB scores with time decreased significantly by 15 % (P = 0.008) during the total study period and 39 % (P = 0.01) during the first 5 years of treatment. No changes in BMB scores were observed after five years of treatment. In Gaucher patients, the trend of MRI BMB scores with time decreased significantly under ERT the first 5 years of treatment before a long-term stabilization. (orig.)

Bone marrow involvement in Gaucher disease at MRI: what long-term evolution can we expect under enzyme replacement therapy?

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Fedida, Benjamin; Touraine, Sebastien; Laredo, Jean-Denis [Hopital Lariboisiere, AP-HP, Department of Musculoskeletal Imaging, Paris (France); Stirnemann, Jerome [Universite Paris-Diderot Hopital Bichat, AP-HP, Department of Biostatistics and Medical Data Processing, INSERM UMR 738, Paris (France); Geneva University Hospital, Division of General Internal Medicine, Faculty of Medicine, Geneva (Switzerland); Belmatoug, Nadia [Hopital Beaujon, AP-HP, Referral Center for Lysosomal Diseases (RCLD), Clichy (France); Hopital Beaujon, AP-HP, Department of Internal Medicine, Clichy (France); Petrover, David [Hopital Lariboisiere, AP-HP, Department of Musculoskeletal Imaging, Paris (France); Hopital Beaujon, AP-HP, Referral Center for Lysosomal Diseases (RCLD), Clichy (France)

2015-10-15

To study the long-term evolution of the bone marrow burden (BMB) score at MRI in patients with Gaucher disease (GD) under enzyme replacement therapy (ERT). Forty patients treated for GD were retrospectively studied in a referral centre. BMB scores were assessed on spine and femur MR examinations performed between January 2003 and June 2014. The long-term evolution of the BMB scores was analyzed using a linear mixed model. A total of 121 MRI examinations were performed during the study period with a mean follow-up of 7.1 years ± 5.6, an average rate of 3.1 MR examinations ± 1.7 per patient and an interval of 2.3 years ± 1.1 between examinations. Patients had received ERT during 12 years on average ± 6.7. The trend of BMB scores with time decreased significantly by 15 % (P = 0.008) during the total study period and 39 % (P = 0.01) during the first 5 years of treatment. No changes in BMB scores were observed after five years of treatment. In Gaucher patients, the trend of MRI BMB scores with time decreased significantly under ERT the first 5 years of treatment before a long-term stabilization. (orig.)

Dose response of bone-targeted enzyme replacement for murine hypophosphatasia.

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Yadav, Manisha C; Lemire, Isabelle; Leonard, Pierre; Boileau, Guy; Blond, Laurent; Beliveau, Martin; Cory, Esther; Sah, Robert L; Whyte, Michael P; Crine, Philippe; Millán, José Luis

2011-08-01

Hypophosphatasia (HPP) features rickets or osteomalacia from tissue-nonspecific alkaline phosphatase (TNSALP) deficiency due to deactivating mutations within the ALPL gene. Enzyme replacement therapy with a bone-targeted, recombinant TNSALP (sALP-FcD(10), renamed ENB-0040) prevents manifestations of HPP when initiated at birth in TNSALP knockout (Akp2(-/-)) mice. Here, we evaluated the dose-response relationship of ENB-0040 to various phenotypic traits of Akp2(-/-) mice receiving daily subcutaneous (SC) injections of ENB-0040 from birth at 0.5, 2.0, or 8.2mg/kg for 43days. Radiographs, μCT, and histomorphometric analyses documented better bone mineralization with increasing doses of ENB-0040. We found a clear, positive correlation between ENB-0040 dose and prevention of mineralization defects of the feet, rib cage, lower limbs, and jaw bones. According to a dose-response model, the ED(80) (the dose that prevents bone defects in 80% of mice) was 3.2, 2.8 and 2.9mg/kg/day for these sites, respectively. Long bones seemed to respond to lower daily doses of ENB-0040. There was also a positive relationship between ENB-0040 dose and survival. Median survival, body weight, and bone length all improved with increasing doses of ENB-0040. Urinary PP(i) concentrations remained elevated in all treatment groups, indicating that while this parameter is a good biochemical marker for diagnosing HPP in patients, it may not be a good follow up marker for evaluating response to treatment when administering bone-targeted TNSALP to mice. These dose-response relationships strongly support the pharmacological efficacy of ENB-0040 for HPP, and provide the experimental basis for the therapeutic range of ENB-0040 chosen for clinical trials. Copyright © 2011 Elsevier Inc. All rights reserved.

The metabolic consequences of thyroxine replacement in adult hypopituitary patients

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Filipsson Nyström, Helena; Feldt-Rasmussen, Ulla; Kourides, Ione

2012-01-01

The metabolic consequences of thyroxine replacement in patients with central hypothyroidism (CH) need to be evaluated. The aim was to examine the outcome of thyroxine replacement in CH. Adult hypopituitary patients (n = 1595) with and without CH from KIMS (Pfizer International Metabolic Database...

Histological characterisation of visceral changes in a patient with type 2 Gaucher disease treated with enzyme replacement therapy.

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Tezuka, Yuko; Fukuda, Mitsumasa; Watanabe, Shohei; Nakano, Takeshi; Okamoto, Kentaro; Kuzume, Kazuyo; Yano, Yoshiaki; Eguchi, Mariko; Ishimae, Minenori; Ishii, Eiichi; Miyazaki, Tatsuhiko

2018-02-01

Gaucher disease is a lysosomal storage disease caused by deficiency of glucocerebrosidase and accumulation of glucocerebroside. Three major sub-types have been described, type 2 is an acute neurological form that exhibits serious general symptoms and poor prognosis, compared with the other types. This case was a girl diagnosed with type 2 Gaucher disease at 12months of age who presented with poor weight gain from infancy, stridor, hypertonia, hepatosplenomegaly, trismus and an eye movement disorder. Enzyme replacement therapy (ERT) was administered, but she had frequent myoclonus and developmental regression. She needed artificial ventilation because of respiratory failure. She died at 11years of age. An autopsy demonstrated infiltrating CD68-positive large cells containing abundant lipids in alveoli, while in the liver, kidney and bone marrow CD68-positive cells were small and round. In the bone marrow, myelodysplastic changes were present without Gaucher cells. The infiltration of Gaucher cells in alveoli was marked, suggesting that ERT was relatively ineffective in pulmonary involvement, particularly intra-alveolar. Additional treatments are necessary to improve the neurological and pulmonary prognosis of type 2Gaucher disease. Copyright © 2016 Elsevier Inc. All rights reserved.

Enzyme replacement prevents enamel defects in hypophosphatasia mice

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Yadav, Manisha C.; de Oliveira, Rodrigo Cardoso; Foster, Brian L.; Fong, Hanson; Cory, Esther; Narisawa, Sonoko; Sah, Robert L.; Somerman, Martha; Whyte, Michael P.; Millán, José Luis

2012-01-01

Hypophosphatasia (HPP) is the inborn error of metabolism characterized by deficiency of alkaline phosphatase activity leading to rickets or osteomalacia and to dental defects. HPP occurs from loss-of-function mutations within the gene that encodes the tissue-nonspecific isozyme of alkaline phosphatase (TNAP). TNAP knockout (Alpl−/−, a.k.a. Akp2−/−) mice closely phenocopy infantile HPP, including the rickets, vitamin B6-responsive seizures, improper dentin mineralization, and lack of acellular cementum. Here, we report that lack of TNAP in Alpl−/− mice also causes severe enamel defects, which are preventable by enzyme replacement with mineral-targeted TNAP (ENB-0040). Immunohistochemistry was used to map the spatiotemporal expression of TNAP in the tissues of the developing enamel organ of healthy mouse molars and incisors. We found strong, stage-specific expression of TNAP in ameloblasts. In the Alpl−/− mice, histological, μCT, and scanning electron microscopy analysis showed reduced mineralization and disrupted organization of the rods and inter-rod structures in enamel of both the molars and incisors. All of these abnormalities were prevented in mice receiving from birth daily subcutaneous injections of mineral-targeting, human TNAP (sALP-FcD10, a.k.a. ENB-0040) at 8.2 mg/kg/day for up to 44 days. These data reveal an important role for TNAP in enamel mineralization, and demonstrate the efficacy of mineral-targeted TNAP to prevent enamel defects in HPP. PMID:22461224

Antibody-mediated enzyme replacement therapy targeting both lysosomal and cytoplasmic glycogen in Pompe disease.

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Yi, Haiqing; Sun, Tao; Armstrong, Dustin; Borneman, Scott; Yang, Chunyu; Austin, Stephanie; Kishnani, Priya S; Sun, Baodong

2017-05-01

Pompe disease is characterized by accumulation of both lysosomal and cytoplasmic glycogen primarily in skeletal and cardiac muscles. Mannose-6-phosphate receptor-mediated enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (rhGAA) targets the enzyme to lysosomes and thus is unable to digest cytoplasmic glycogen. Studies have shown that anti-DNA antibody 3E10 penetrates living cells and delivers "cargo" proteins to the cytosol or nucleus via equilibrative nucleoside transporter ENT2. We speculate that 3E10-mediated ERT with GAA will target both lysosomal and cytoplasmic glycogen in Pompe disease. A fusion protein (FabGAA) containing a humanized Fab fragment derived from the murine 3E10 antibody and the 110 kDa human GAA precursor was constructed and produced in CHO cells. Immunostaining with an anti-Fab antibody revealed that the Fab signals did not co-localize with the lysosomal marker LAMP2 in cultured L6 myoblasts or Pompe patient fibroblasts after incubation with FabGAA. Western blot with an anti-GAA antibody showed presence of the 150 kDa full-length FabGAA in the cell lysates, in addition to the 95- and 76 kDa processed forms of GAA that were also seen in the rhGAA-treated cells. Blocking of mannose-6-phosphate receptor with mannose-6-phosphate markedly reduced the 95- and the 76 kDa forms but not the 150 kDa form. In GAA-KO mice, FabGAA achieved similar treatment efficacy as rhGAA at an equal molar dose in reducing tissue glycogen contents. Our data suggest that FabGAA retains the ability of rhGAA to treat lysosomal glycogen accumulation and has the beneficial potential over rhGAA to reduce cytoplasmic glycogen storage in Pompe disease. FabGAA can be delivered to both the cytoplasm and lysosomes in cultured cells. FabGAA equally reduced lysosomal glycogen accumulation as rhGAA in GAA-KO mice. FabGAA has the beneficial potential over rhGAA to clear cytoplasmic glycogen. This study suggests a novel antibody-enzyme fusion protein therapy

Counseling patients on facial volume replacement and adherence with posttreatment instructions

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Doris Day

2010-07-01

Full Text Available Doris DayDay Dermatology and Aesthetics New York, USAAbstract: Use of injectable volume replacement products has increased dramatically in the US in recent years. An optimal outcome with volume replacement depends on a thorough ­knowledge of the products on the part of the dermatologic/aesthetic physician specialist, identification of patients with a likelihood of benefiting from volume replacement procedures, selection of an appropriate product for the individual patient, and effective patient counseling to ensure adherence to posttreatment care instructions. Adherence to physician instructions in the field of dermatology appears limited, and there is very little published information on adherence to physician instructions following facial volume replacement procedures. The purpose of this review is to provide strategies for understanding and overcoming the barriers to adherence with the widely used dermal fillers. Strategies include using patient-centered techniques, such as a motivational interview encouraging the patient to follow postprocedure care instructions, eg, massage. In this case, demonstrating massage techniques while the patient is still in the office, with patient participation and detailed feedback, also contributes to good adherence with posttreatment care instructions. Telephone counseling, reminder postcards, and text messages may help improve clinic attendance for follow-up. Motivated patients who demonstrate good ­adherence to physician instructions generally respond well to volume replacement treatments, and usually experience fewer adverse events than patients who do not follow instructions. Although promoting adherence to pretreatment and posttreatment protocols remains a challenge, patient counseling throughout the treatment process can lead to successful results.Keywords: improving adherence, injectable volume replacement, product selection, ­rejuvenation procedure, soft tissue augmentation

The impact of the immune system on the safety and efficiency of enzyme replacement therapy in lysosomal storage disorders.

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Broomfield, A; Jones, S A; Hughes, S M; Bigger, B W

2016-07-01

In the light of clinical experience in infantile onset Pompe patients, the immunological impact on the tolerability and long-term efficacy of enzyme replacement therapy (ERT) for lysosomal storage disorders has come under renewed scrutiny. This article details the currently proposed immunological mechanisms involved in the development of anti-drug antibodies and the current therapies used in their treatment. Given the current understanding of the adaptive immune response, it focuses particularly on T cell dependent mechanisms and the paradigm of using lymphocytic negative selection as a predictor of antibody formation. This concept originally postulated in the 1970s, stipulated that the genotypically determined lack of production or production of a variant protein determines an individual's lymphocytic repertoire. This in turn is the key factor in determining the potential severity of an individual's immunological response to ERT. It also highlights the need for immunological assay standardization particularly those looking at describing the degree of functional impact, robust biochemical or clinical endpoints and detailed patient subgroup identification if the true evaluations of impact are to be realised.

Angiotensin Converting Enzyme Gene Insertion/Deletion Polymorphism in Migraine Patients

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Belgin Alaşehirli

2009-12-01

Full Text Available OBJECTIVE: The beneficial effects of angiotensin converting enzyme inhibitor drugs on migraine attack frequency have been shown. We aimed to study the relationship between the angiotensin converting enzyme gene and migraine pathophysiology. METHODS: In the present study, to assess whether the angiotensin converting enzyme insertion/deletion (I/D gene polymorphisms have an effect on migraine attacks, we studied the angiotensin converting enzyme genotypes of 102 migraine patients (35 cases of migraine with aura and 67 of migraine without aura and 75 age-and sex-matched normal volunteers. Frequency and age of onset of migraine attacks were also assessed according to angiotensin converting enzyme genotypes. RESULTS: Patients with migraine with and without aura were comparable with each other and the control group with respect to angiotensin converting enzyme genotypes (respectively; p= 0.88 and p= 0.76, p= 0.624. We could not determine a relationship between angiotensin converting enzyme genotypes and attack frequency (p= 0.125, but cases with angiotensin converting enzyme-II genotype showed a significantly younger age for onset of migraine attacks in comparison with the I/D genotype patients (p= 0.021. CONCLUSION: We believe that further angiotensin converting enzyme gene studies are warranted in younger age groups of patients with migraine and also in different populations

The effect of growth hormone replacement on the thyroid axis in patients with hypopituitarism: in vivo and ex vivo studies.

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Glynn, Nigel; Kenny, Helena; Quisenberry, Leah; Halsall, David J; Cook, Paul; Kyaw Tun, Tommy; McDermott, John H; Smith, Diarmuid; Thompson, Christopher J; O'Gorman, Donal J; Boelen, Anita; Lado-Abeal, Joaquin; Agha, Amar

2017-05-01

Alterations in the hypothalamic-pituitary-thyroid axis have been reported following growth hormone (GH) replacement. The aim was to examine the relationship between changes in serum concentration of thyroid hormones and deiodinase activity in subcutaneous adipose tissue, before and after GH replacement. A prospective, observational study of patients receiving GH replacement as part of routine clinical care. Twenty adult hypopituitary men. Serum TSH, thyroid hormones - free and total thyroxine (T4) and triiodothyronine (T3) and reverse T3, thyroglobulin and thyroid-binding globulin (TBG) levels were measured before and after GH substitution. Changes in serum hormone levels were compared to the activity of deiodinase isoenzymes (DIO1, DIO2 and DIO3) in subcutaneous adipose tissue. The mean daily dose of growth hormone (GH) was 0·34 ± 0·11 mg (range 0·15-0·5 mg). Following GH replacement, mean free T4 levels declined (-1·09 ± 1·99 pmol/l, P = 0·02). Reverse T3 levels also fell (-3·44 ± 1·42 ng/dl, P = 0·03) and free T3 levels increased significantly (+0·34 ± 0·15 pmol/l, P = 0·03). In subcutaneous fat, DIO2 enzyme activity declined; DIO1 and DIO3 activities remained unchanged following GH substitution. Serum TSH, thyroglobulin and TBG levels were unaltered by GH therapy. In vitro analysis of subcutaneous adipose tissue from hypopituitary human subjects demonstrates that GH replacement is associated with significant changes in deiodinase isoenzyme activity. However, the observed variation in enzyme activity does not explain the changes in the circulating concentration of thyroid hormones induced by GH replacement. It is possible that deiodinase isoenzymes are differentially regulated by GH in other tissues including liver and muscle. © 2016 John Wiley & Sons Ltd.

Should patients with Björk-Shiley valves undergo prophylactic replacement?

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Birkmeyer, J D; Marrin, C A; O'Connor, G T

1992-08-29

About 85,000 patients have undergone replacement of diseased heart valves with prosthetic Björk-Shiley convexo-concave (CC) valves. These valves are prone to fracture of the outlet strut, which leads to acute valve failure that is usually fatal. Should patients with these valves undergo prophylactic replacement to avoid fracture? The incidence of strut fracture varies between 0% and 1.5% per year, depending on valve opening angle (60 degrees or 70 degrees), diameter (less than 29 mm or greater than or equal to 29 mm), and location (aortic or mitral). Other factors include the patient's life expectancy and the expected morbidity and mortality associated with reoperation. We have used decision analysis to identify the patients most likely to benefit from prophylactic reoperation. The incidence of outlet strut fracture was estimated from the data of three large studies on CC valves, and stratified by opening angle, diameter, and location. A Markov decision analysis model was used to estimate life expectancy for patients undergoing prophylactic valve replacement and for those not undergoing reoperation. Prophylactic valve replacement does not benefit patients with CC valves that have low strut fracture risks (60 degrees aortic valves and less than 29 mm, 60 degrees mitral valves). For most patients with CC valves that have high strut fracture risks (greater than or equal to 29 mm, 70 degrees CC), prophylactic valve replacement increases life expectancy. However, elderly patients with such valves benefit from prophylactic reoperation only if the risk of operative mortality is low. Patient age and operative risk are most important in recommendations for patients with CC valves that have intermediate strut fracture risks (less than 29 mm, 70 degrees valves and greater than or equal to 29 mm, 60 degrees mitral valves). For all patients and their doctors facing the difficult decision on whether to replace CC valves, individual estimates of operative mortality risk that

Response of women with Fabry disease to enzyme replacement therapy: comparison with men, using data from FOS--the Fabry Outcome Survey

NARCIS (Netherlands)

Hughes, Derralynn A.; Barba Romero, Miguel-Ángel; Hollak, Carla E. M.; Giugliani, Roberto; Deegan, Patrick B.

2011-01-01

Fabry disease (α-galactosidase A deficiency) is an X-linked disorder. Women who are heterozygous for disease-causing mutations often manifest signs and symptoms of Fabry disease, but most studies of the effects of enzyme replacement therapy (ERT) have included only men. To date, no direct comparison

Biochemical Characterization of Porphobilinogen Deaminase–Deficient Mice During Phenobarbital Induction of Heme Synthesis and the Effect of Enzyme Replacement

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Johansson, Annika; Möller, Christer; Fogh, Jens; Harper, Pauline

2003-01-01

Acute intermittent porphyria (AIP) is a genetic disorder caused by a deficiency of porphobilinogen deaminase (PBGD), the 3rd enzyme in heme synthesis. It is clinically characterized by acute attacks of neuropsychiatric symptoms and biochemically by increased urinary excretion of the porphyrin precursors porphobilinogen (PBG) and 5-aminolevulinic acid (ALA). A mouse model that is partially deficient in PBGD and biochemically mimics AIP after induction of the hepatic ALA synthase by phenobarbital was used in this study to identify the site of formation of the presumably toxic porphyrin precursors and study the effect of enzyme-replacement therapy by using recombinant human PBGD (rhPBGD). After 4 d of phenobarbital administration, high levels of PBG and ALA were found in liver, kidney, plasma, and urine of the PBGD-deficient mice. The administration of rhPBGD intravenously or subcutaneously after a 4-d phenobarbital induction was shown to lower the PBG level in plasma in a dose-dependent manner with maximal effect seen after 30 min and 2 h, respectively. Injection of rhPBGD subcutaneously twice daily during a 4-d phenobarbital induction reduced urinary PBG excretion to 25% of the levels found in PBGD-deficient mice administered with only phenobarbital. This study points to the liver as the main producer of PBG and ALA in the phenobarbital-induced PBGD-deficient mice and demonstrates efficient removal of accumulated PBG in plasma and urine by enzyme-replacement therapy. PMID:15208740

Enzyme Replacement Therapy and/or Hematopoietic Stem Cell Transplantation at diagnosis in patients with Mucopolysaccharidosis type I: results of a European consensus procedure

LENUS (Irish Health Repository)

de Ru, Minke H

2011-08-10

Abstract Background Mucopolysaccharidosis type I (MPS I) is a lysosomal storage disorder that results in the accumulation of glycosaminoglycans causing progressive multi-organ dysfunction. Its clinical spectrum is very broad and varies from the severe Hurler phenotype (MPS I-H) which is characterized by early and progressive central nervous system (CNS) involvement to the attenuated Scheie phenotype (MPS I-S) with no CNS involvement. Indication, optimal timing, safety and efficacy of the two available treatment options for MPS I, enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT), are subject to continuing debate. A European consensus procedure was organized to reach consensus about the use of these two treatment strategies. Methods A panel of specialists, including 8 specialists for metabolic disorders and 7 bone marrow transplant physicians, all with acknowledged expertise in MPS I, participated in a modified Delphi process to develop consensus-based statements on MPS I treatment. Fifteen MPS I case histories were used to initiate the discussion and to anchor decisions around either treatment mode. Before and at the meeting all experts gave their opinion on the cases (YES\\/NO transplantation) and reasons for their decisions were collected. A set of draft statements on MPS I treatment options composed by a planning committee were discussed and revised during the meeting until full consensus. Results Full consensus was reached on several important issues, including the following: 1) The preferred treatment for patients with MPS I-H diagnosed before age 2.5 yrs is HSCT; 2) In individual patients with an intermediate phenotype HSCT may be considered if there is a suitable donor. However, there are no data on efficacy of HSCT in patients with this phenotype; 3) All MPS I patients including those who have not been transplanted or whose graft has failed may benefit significantly from ERT; 4) ERT should be started at diagnosis and may be

Skin symptoms in patients with atopic dermatitis using enzyme-containing detergents

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Andersen, Peter Hundevadt; Bindslev-Jensen, C; Mosbech, H

1998-01-01

Detergent enzymes may cause skin irritation and occasionally hypersensitivity reactions. The potential hazards of these enzymes have led some physicians to advise atopic dermatitis patients against the use of enzyme-enriched detergents. A three-phased randomised, double-blind, cross-over experiment...... was designed to question this recommendation. Each period was of 1 month's duration.In the first phase patients continued using their normal washing detergent. In phase II patients used trial detergent with or without added enzymes, and during phase III patients were given the opposite trial detergent. A total...... differences in any of the primary or secondary parameters comparing treatment and placebo periods. Our data therefore seem to exclude that atopic dermatitis may exacerbate during 1 month's exposure to enzyme-enriched detergents. Since no significant irritant capacity was detected in atopic dermatitis patients...

[Reoperative valve replacement in patients undergoing cardiac reoperation: a report of 104 cases].

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Zou, Liang-jian; Xu, Zhi-yun; Wang, Zhi-nong; Lang, Xi-long; Han, Lin; Lu, Fang-lin; Xu, Ji-bin; Tang, Hao; Ji, Guang-yu; Wang, Er-song; Wang, Jun; Qu, Yi

2010-08-15

To review the experience of reoperative valve replacement for 104 patients. From January 2002 to December 2009, 104 patients underwent heart valve replacement in reoperations, accounting for 2.92% of the total patient population (3557 cases) who had valve replacement during this period. In this group, 53 male and 51 female patients were included with a median age of 46 years (ranged from 13 to 72 years). The reasons of reoperation included 28 cases suffered from another valve lesion after valve replacement, 10 cases suffered from valve lesion after mitral valvuloplasty, 19 cases suffered from perivalvular leakage after valve replacement, 18 cases suffered from valve lesion after previous correction of congenital heart defect, 7 cases suffered from bioprosthetic valve decline, 10 cases suffered from prosthetic valve endocarditis, 9 cases suffered from dysfunction of machine valve, and 3 cases suffered from other causes. The re-operations were mitral and aortic valve replacement in 2 cases, mitral valve replacement in 59 cases, aortic valve replacement in 24 cases, tricuspid valve replacement in 16 cases, and Bentall's operation in 3 cases. The interval from first operation to next operation was 1 month-19 years. There were 8 early deaths from heart failure, renal failure and multiple organ failure (early mortality 7.69%). Major complications were intraoperative hemorrhage in 2 cases, re-exploration for mediastinal bleeding in 2 cases and sternotomy surgical site infection in 1 case. Complete follow-up (3 months-7 years and 2 months) was available for all patients. Two patients died, one patient died of intracranial hemorrhage, and another cause was unknown. Satisfactory short-term and long-term results can be obtained in reoperative valve replacement with appropriate timing of operation control, satisfactory myocardial protection, accurate surgical procedure and suitable perioperative treatment.

Total knee replacement in patients with diffuse villonodular synovitis

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Lucio Flávio Biondi Pinheiro Júnior

Full Text Available ABSTRACT This paper reports a case of diffuse pigmented villonodular synovitis (DPVNS, associated with advanced gonarthrosis, submitted to total knee replacement. The patient had progressive pain and swelling. She had two previous surgeries, firstly arthroscopic , synovectomy and subsequently open synovectomy associated with radiotherapy, with recurrence of the disease. Magnetic resonance imaging revealed diffuse synovitis, advanced arthrosis, and bone cysts. The patient was submitted to a total knee replacement and synovectomy. There was a good postoperative clinical course, with improvement of pain, function, and joint edema on examination. The patient will be followed regarding the possibility of disease recurrence and implant survival.,

Lysosomal enzyme delivery by ICAM-1-targeted nanocarriers bypassing glycosylation- and clathrin-dependent endocytosis.

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Muro, Silvia; Schuchman, Edward H; Muzykantov, Vladimir R

2006-01-01

Enzyme replacement therapy, a state-of-the-art treatment for many lysosomal storage disorders, relies on carbohydrate-mediated binding of recombinant enzymes to receptors that mediate lysosomal delivery via clathrin-dependent endocytosis. Suboptimal glycosylation of recombinant enzymes and deficiency of clathrin-mediated endocytosis in some lysosomal enzyme-deficient cells limit delivery and efficacy of enzyme replacement therapy for lysosomal disorders. We explored a novel delivery strategy utilizing nanocarriers targeted to a glycosylation- and clathrin-independent receptor, intercellular adhesion molecule (ICAM)-1, a glycoprotein expressed on diverse cell types, up-regulated and functionally involved in inflammation, a hallmark of many lysosomal disorders. We targeted recombinant human acid sphingomyelinase (ASM), deficient in types A and B Niemann-Pick disease, to ICAM-1 by loading this enzyme to nanocarriers coated with anti-ICAM. Anti-ICAM/ASM nanocarriers, but not control ASM or ASM nanocarriers, bound to ICAM-1-positive cells (activated endothelial cells and Niemann-Pick disease patient fibroblasts) via ICAM-1, in a glycosylation-independent manner. Anti-ICAM/ASM nanocarriers entered cells via CAM-mediated endocytosis, bypassing the clathrin-dependent pathway, and trafficked to lysosomes, where delivered ASM displayed stable activity and alleviated lysosomal lipid accumulation. Therefore, lysosomal enzyme targeting using nanocarriers targeted to ICAM-1 bypasses defunct pathways and may improve the efficacy of enzyme replacement therapy for lysosomal disorders, such as Niemann-Pick disease.

New protein involved in the replacement of cell molecules

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Poulsen, Jesper Buchhave

2011-01-01

In collaboration with colleagues from La Trobe University, Australia, scientists at Aarhus University have discovered and defined a novel enzyme involved in the replacement and renewal of cell molecules. The enzyme exerts its function within the so-called mitochondria - small “enclosed” compartme......In collaboration with colleagues from La Trobe University, Australia, scientists at Aarhus University have discovered and defined a novel enzyme involved in the replacement and renewal of cell molecules. The enzyme exerts its function within the so-called mitochondria - small “enclosed...

Patient and implant survival following joint replacement because of metastatic bone disease

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Sørensen, Michala S; Gregersen, Kristine G; Grum-Schwensen, Tomas

2013-01-01

Patients suffering from a pathological fracture or painful bony lesion because of metastatic bone disease often benefit from a total joint replacement. However, these are large operations in patients who are often weak. We examined the patient survival and complication rates after total joint...... replacement as the treatment for bone metastasis or hematological diseases of the extremities....

Timing of initiation of enzyme replacement therapy after diagnosis of type 1 Gaucher disease: effect on incidence of avascular necrosis

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Mistry, Pramod K; Deegan, Patrick; Vellodi, Ashok; Cole, J Alexander; Yeh, Michael; Weinreb, Neal J

2009-01-01

Data from the International Collaborative Gaucher Group Gaucher Registry were analysed to assess the relationship between enzyme replacement therapy with imiglucerase (ERT) and incidence of avascular necrosis (AVN) in type 1 Gaucher disease (GD1), and to determine whether the time interval between diagnosis and initiation of ERT influences the incidence rate of AVN. All patients with GD1 enrolled in the Gaucher Registry who received ERT and did not report AVN prior to starting therapy (n = 2700) were included. The incidence rate of AVN following initiation of ERT was determined. An incidence rate of AVN of 13·8 per 1000 person-years was observed in patients receiving ERT. Patients who initiated ERT within 2 years of diagnosis had an incidence rate of 8·1 per 1000 person-years; patients who started ERT ≥2 years after diagnosis had an incidence rate of 16·6 per 1000 person-years. The adjusted incidence rate ratio was 0·59 [95% confidence interval (CI) 0·36–0·96, P = 0·0343]. Splenectomy was an independent risk factor for AVN (adjusted incidence rate ratio 2·23, 95% CI 1·61–3·08, P < 0·0001). In conclusion, the risk of AVN was reduced among patients who initiated ERT within 2 years of diagnosis, compared to initiating treatment ≥2 years after diagnosis. A higher risk of AVN was observed among patients who had previously undergone splenectomy. PMID:19732054

Study of vitamin D serum level in patients with epilepsy treated with enzyme-inducing and non enzyme-inducing medications

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sima Hashemipour

2014-01-01

Full Text Available Background : Changes of serum minerals and vitamin D have been reported in anticonvulsant drugs user patients. The present study aimed at comparing the changes of serum minerals and vitamin D among two groups of enzyme-inducing and non enzyme-inducing anticonvulsant drug users. Methods: In this study 22 patients treated with enzyme-inducing drugs (carbamazepin, phenytoin, phenobarbital were compared to 25 patients of matched sex, age, and BMI treated with non enzyme-inducing drugs (sodium evaporate, lamotrigine. Serum calcium, phosphate, parathormone, and 25-hydroxy vitamin D were calculated in both groups. Calcium was measured by Calorimetery method. Parathormone and vitamin D were measured using ELISA method. Results: The mean serum vitamin D level was lower in enzyme-inducing than non enzyme-inducing drugs users (15.9±8.3 and 24.2±14.8, P=0.02. Frequency of vitamin D deficiency was higher in enzyme-inducing compared to non enzyme-inducing drugs users, 84% and 48% , respectively (P=0.016. The mean serum calcium level was significantly lower in enzyme-inducing drugs users. (8.7±0.2 vs. 9.0± 0.7, p= 0.05. Four percent in enzyme-inducing group compared to twenty four percent of non enzyme-inducing group had secondary hyperparathyroidism (P=0.016. Conclusion: While vitamin D deficiency is more frequent in enzyme-inducing drug users, secondary hyperparathyroidism is less frequent.

Coronary artery disease in patients undergoing valve replacement at a tertiary care cardiac centre

International Nuclear Information System (INIS)

Shaikh, A.H.; Hanif, B.; Hasan, K.; Hashmani, S.

2011-01-01

To determine the prevalence of coronary artery disease in patients undergoing valve surgery at a tertiary care cardiac centre. The medical records of 144 consecutive patients who underwent mitral, aortic or dual (mitral and aortic) valve replacement surgery at the Tabba Heart Institute between January 2006 to December 2008 were retrospectively reviewed. All patients underwent coronary angiogram. Significant coronary artery disease (CAD) is defined as coronary stenosis of > 50%. There were 74 (51.4%) males and 70 (48.6%) females in the study. The mean age was 51.64 +- 11 years. Of all, 73 (50.7%) underwent mitral valve replacement, 47 (32.6%) had aortic and 24 (16.7%) had dual valve replacement. Out of 144 patients, 99 (68.8%) had 50% stenosis. In patients who had undergone mitral valve replacement (MVR), significant coronary disease was found in 32.9%, whereas in patients who had undergone aortic valve replacement (AVR) and dual valve replacement (DVR) the prevalence of coronary disease was 31.9% and 25% respectively. Our results suggest that the overall prevalence of coronary artery disease in patients undergoing valve surgery in our population is comparable with prevalence reported in international data. (author)

Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

International Nuclear Information System (INIS)

Poll, L.W.; Koch, J.A.; Scherer, A.; Boerner, D.; Moedder, U.; Dahl, S. vom; Niederau, C.; Haeussinger, D.; Willers, R.

2001-01-01

Objective:. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Patients and design:. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Results:. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). Conclusions:. MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non- homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021). (orig.)

Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

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Poll, L.W.; Koch, J.A.; Scherer, A.; Boerner, D.; Moedder, U. [Duesseldorf Univ. (Germany). Inst. fuer Diagnostische Radiologie; Dahl, S. vom; Niederau, C.; Haeussinger, D. [Duesseldorf Univ. (Germany). Medizinische Fakultaet; Willers, R. [Duesseldorf Univ. (Germany). Rechenzentrum

2001-09-01

Objective:. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Patients and design:. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Results:. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). Conclusions:. MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non- homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021). (orig.)

Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

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Kunkala, Meghana R; Schaff, Hartzell V; Li, Zhuo; Volguina, Irina; Dietz, Harry C; LeMaire, Scott A; Coselli, Joseph S; Connolly, Heidi

2013-09-10

Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤ 3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31 ± 10 months). Echocardiography performed 21 ± 13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤ 2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤ 2 would benefit from prophylactic MV intervention.

Replacement of Mushroom Cage Gastrostomy Tube Using a Modified Technique to Allow Percutaneous Replacement with an Endoscopic Tube in Patients with Amyotrophic Lateral Sclerosis

International Nuclear Information System (INIS)

Ammar, Thoraya; Rio, Alan; Ampong, Mary Ann; Sidhu, Paul S.

2010-01-01

Radiologic inserted gastrostomy (RIG) is the preferred method in our institution for enteral feeding in amyotrophic lateral sclerosis (ALS). Skin-level primary-placed mushroom cage gastrostomy tubes become tight with weight gain. We describe a minimally invasive radiologic technique for replacing mushroom gastrostomy tubes with endoscopic mushroom cage tubes in ALS. All patients with ALS who underwent replacement of a RIG tube were included. Patients were selected for a modified replacement when the tube length of the primary placed RIG tube was insufficient to allow like-for-like replacement. Replacement was performed under local anesthetic and fluoroscopic guidance according to a preset technique, with modification of an endoscopic mushroom cage gastrostomy tube to allow percutaneous placement. Assessment of the success, safety, and durability of the modified technique was undertaken. Over a 60-month period, 104 primary placement mushroom cage tubes in ALS were performed. A total of 20 (19.2%) of 104 patients had a replacement tube positioned, 10 (9.6%) of 104 with the modified technique (male n = 4, female n = 6, mean age 65.5 years, range 48-85 years). All tubes were successfully replaced using this modified technique, with two minor complications (superficial wound infection and minor hemorrhage). The mean length of time of tube durability was 158.5 days (range 6-471 days), with all but one patient dying with a functional tube in place. We have devised a modification to allow percutaneous replacement of mushroom cage gastrostomy feeding tubes with minimal compromise to ALS patients. This technique allows tube replacement under local anesthetic, without the need for sedation, an important consideration in ALS.

[Shift of focus in the financing of Hungarian drugs. Reimbursement for orphan drugs for treating rare diseases: financing of enzyme replacement therapy in Hungary].

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Szegedi, Márta; Molnár, Mária Judit; Boncz, Imre; Kosztolányi, György

2014-11-02

Focusing on the benefits of patients with rare disease the authors analysed the aspects of orphan medicines financed in the frame of the Hungarian social insurance system in 2012 in order to make the consumption more rational, transparent and predictable. Most of the orphan drugs were financed in the frame of compassionate use by the reimbursement system. Consequently, a great deal of crucial problems occurred in relation to the unconventional subsidized method, especially in the case of the highest cost enzyme replacement therapies. On the base of the findings, proposals of the authors are presented for access to orphan drugs, fitting to the specific professional, economical and ethical aspects of this unique field of the health care system. The primary goal is to provide a suitable subsidized method for the treatment of rare disease patients with unmet medical needs. The financial modification of orphans became indispensible in Hungary. Professionals from numerous fields dealing with rare disease patients' care expressed agreement on the issue. Transforming the orphan medicines' financial structure has been initiated according to internationally shared principles.

Quantitative liver functions in Turner syndrome with and without hormone replacement therapy

DEFF Research Database (Denmark)

Gravholt, Claus Højbjerg; Poulsen, H.E.; Ott, Peter

2007-01-01

Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes.......Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes....

Respiratory muscle training with enzyme replacement therapy improves muscle strength in late - onset Pompe disease.

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Jevnikar, Mitja; Kodric, Metka; Cantarutti, Fabiana; Cifaldi, Rossella; Longo, Cinzia; Della Porta, Rossana; Bembi, Bruno; Confalonieri, Marco

2015-12-01

Pompe disease is an autosomal recessive metabolic disorder caused by the deficiency of the lysosomal enzyme acid α-glucosidase. This deficiency leads to glycogen accumulation in the lysosomes of muscle tissue causing progressive muscular weakness particularly of the respiratory system. Enzyme replacement therapy (ERT) has demonstrated efficacy in slowing down disease progression in infants. Despite the large number of studies describing the effects of physical training in juvenile and adult late onset Pompe disease (LOPD). There are very few reports that analyze the benefits of respiratory muscle rehabilitation or training. The effectiveness of respiratory muscle training was investigated using a specific appliance with adjustable resistance (Threshold). The primary endpoint was effect on respiratory muscular strength by measurements of MIP and MEP. Eight late-onset Pompe patients (aged 13 to 58 years; 4 female, 4 male) with respiratory muscle deficiency on functional respiratory tests were studied. All patients received ERT at the dosage of 20 mg/kg/every 2 weeks and underwent training with Threshold at specified pressures for 24 months. A significant increase in MIP was observed during the follow-up of 24 month: 39.6 cm H 2 O (+ 25.0%) at month 3; 39.5 cm H 2 O (+ 24.9%) at month 6; 39.1 cm H 2 O (+ 23.7%) at month 9; 37.3 cm H 2 O (+ 18.2%) at month 12; and 37.3 cm H 2 O (+ 17.8%) at month 24. Median MEP values also showed a significant increase during the first 9 months: 29.8 cm H 2 O, (+ 14.3%) at month 3; 31.0 cm H 2 O (+ 18.6) at month 6; and 29.5 cm H 2 O (+ 12.9) at month 9. MEP was then shown to be decreased at months 12 and 24; median MEP was 27.2 cm H 2 O (+ 4.3%) at 12 months and 26.6 cm H 2 O (+ 1.9%) at 24 months. The FVC remain stable throughout the study. An increase in respiratory muscular strength was demonstrated with Threshold training when used in combination with ERT.

Hunter syndrome in an 11-year old girl on enzyme replacement therapy with idursulfase: brain magnetic resonance imaging features and evolution.

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Manara, Renzo; Rampazzo, Angelica; Cananzi, Mara; Salviati, Leonardo; Mardari, Rodica; Drigo, Paola; Tomanin, Rosella; Gasparotto, Nicoletta; Priante, Elena; Scarpa, Maurizio

2010-12-01

Mucopolysaccharidosis type II (MPS-II, Hunter disease) is a X-linked recessive disorder. Affected females are extremely rare, mostly due to skewed X chromosome inactivation. A few papers outline MPS-II brain magnetic resonance imaging (MRI) "gestalt" in males, but neuroradiological reports on females are still lacking. We present an 11-year-old girl affected by the severe form of MPS-II who was followed up over a time span of 8 years, focusing on clinical and brain MRI evolution. In the last 2.5 years, the patient has been treated with enzyme replacement therapy (ERT) with idursulfase (Elaprase™, Shire Human Genetic Therapies AB, Sweden). On brain and cervical MRI examination, abnormalities in our patient did not differ from those detected in male patients: J-shaped pituitary sella, enlargement of perivascular spaces, brain atrophy, mild T2-hyperintensity in the paratrigonal white matter, diffuse platyspondylia, and mild odontoid dysplasia with odontoid cup. Brain atrophy progressed despite ERT introduction, whereas perivascular space enlargement did not change significantly before and after ERT. Cognitive impairment worsened independently from the course of white matter abnormality. Despite a profound knowledge of genetic and biochemical aspects in MPS-II, neuroradiology is still poorly characterized, especially in female patients. Spinal and brain involvement and its natural course and evolution after ERT introduction still need to be clarified.

Pancreatic enzyme replacement therapy in cystic fibrosis: dose, variability and coefficient of fat absorption

Directory of Open Access Journals (Sweden)

Joaquim Calvo-Lerma

Full Text Available Objectives: Pancreatic enzyme replacement therapy (PERT remains a backbone in the nutritional treatment of cystic fibrosis. Currently, there is a lack of an evidence-based tool that allows dose adjustment. To date, no studies have found an association between PERT dose and fat absorption. Therefore, the aim of the study was to assess the influence of both the PERT dose and the variability in this dose on the coefficient of fat absorption (CFA. Methods: This is a retrospective longitudinal study of 16 pediatric patients (192 food records with three consecutive visits to the hospital over a twelve-month period. Dietary fat intake and PERT were assessed via a four-day food record and fat content in stools was determined by means of a three-day stool sample collection. A beta regression model was built to explain the association between the CFA and the interaction between the PERT dose (lipase units [LU]/g dietary fat and the variability in the PERT dose (standard deviation [SD]. Results: The coefficient of fat absorption increased with the PERT dose when the variability in the dose was low. In contrast, even at the highest PERT dose values, the CFA decreased when the variability was high. The confidence interval suggested an association, although the analysis was not statistically significant. Conclusion: The variability in the PERT dose adjustment should be taken into consideration when performing studies on PERT efficiency. A clinical goal should be the maintenance of a constant PERT dose rather than trying to obtain an optimal value.

Pancreatic enzyme replacement therapy in cystic fibrosis: dose, variability and coefficient of fat absorption.

Science.gov (United States)

Calvo-Lerma, Joaquim; Martínez-Barona, Sandra; Masip, Etna; Fornés, Victoria; Ribes-Koninckx, Carmen

2017-10-01

Pancreatic enzyme replacement therapy (PERT) remains a backbone in the nutritional treatment of cystic fibrosis. Currently, there is a lack of an evidence-based tool that allows dose adjustment. To date, no studies have found an association between PERT dose and fat absorption. Therefore, the aim of the study was to assess the influence of both the PERT dose and the variability in this dose on the coefficient of fat absorption (CFA). This is a retrospective longitudinal study of 16 pediatric patients (192 food records) with three consecutive visits to the hospital over a twelve-month period. Dietary fat intake and PERT were assessed via a four-day food record and fat content in stools was determined by means of a three-day stool sample collection. A beta regression model was built to explain the association between the CFA and the interaction between the PERT dose (lipase units [LU]/g dietary fat) and the variability in the PERT dose (standard deviation [SD]). The coefficient of fat absorption increased with the PERT dose when the variability in the dose was low. In contrast, even at the highest PERT dose values, the CFA decreased when the variability was high. The confidence interval suggested an association, although the analysis was not statistically significant. The variability in the PERT dose adjustment should be taken into consideration when performing studies on PERT efficiency. A clinical goal should be the maintenance of a constant PERT dose rather than trying to obtain an optimal value.

Patient retention and replacement trends after saline breast implants: are deflations inflationary?

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Stevens, W Grant; Pacella, Salvatore J; Hirsch, Elliot; Stoker, David A

2009-01-01

This study aimed to examine serial operative trends with patients who have experienced surgical implant deflation. In addition, the economic impact of deflation on practice caseload was analyzed. A retrospective review was conducted to examine patients who experienced deflation from 2000 to 2007. Patient demographics, implant data, and the presence of secondary (performed at explantation) or tertiary (performed later) procedures were examined. Financial information was tabulated to determine the economic multiplier effect (i.e. the expected value of revenue from secondary and tertiary procedures divided by explantation cost) of taking on deflation cases in a practice. For this study, 285 patients with an average age of 38.4 years were identified. The average time to explantation was 50 months. Slightly more than half of the patients (55%) had both implants replaced at the time of explantation, whereas 59% switched to silicone implants and 41% continued with saline implants. A larger implant was chosen by 54% of the patients (average increase, 82 ml), whereas 18% underwent secondary procedures at the time of explantation including mastopexy (n = 22), facial rejuvenation (n = 8), liposuction (n = 7), or a combination of the two (n = 8). Tertiary procedures were performed for 31% of the patients after their explantation/reimplantation (average time frame, 13 months). The tertiary procedures included replacement with silicone (33.7%), liposuction (24.7%), abdominoplasty (11.2%), facial rejuvenation (13.5%), or nonsurgical rejuvenation using Botox, Restylane, or laser procedure (33.7%). Economic multiplier analysis showed that the financial impact of revenue derived from implant deflation on downstream practice revenue is 1.02. At the time of explantation, replacement with silicone after saline deflation is common (59% of patients). In this study, patients who chose replacement with saline had a significant tendency to replace with silicone (33%) as a tertiary procedure

Gene therapy/bone marrow transplantation in ADA-deficient mice: roles of enzyme-replacement therapy and cytoreduction.

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Carbonaro, Denise A; Jin, Xiangyang; Wang, Xingchao; Yu, Xiao-Jin; Rozengurt, Nora; Kaufman, Michael L; Wang, Xiaoyan; Gjertson, David; Zhou, Yang; Blackburn, Michael R; Kohn, Donald B

2012-11-01

Gene therapy (GT) for adenosine deaminase-deficient severe combined immune deficiency (ADA-SCID) can provide significant long-term benefit when patients are given nonmyeloablative conditioning and ADA enzyme-replacement therapy (ERT) is withheld before autologous transplantation of γ-retroviral vector-transduced BM CD34+ cells. To determine the contributions of conditioning and discontinuation of ERT to the therapeutic effects, we analyzed these factors in Ada gene knockout mice (Ada(-/-)). Mice were transplanted with ADA-deficient marrow transduced with an ADA-expressing γ-retroviral vector without preconditioning or after 200 cGy or 900 cGy total-body irradiation and evaluated after 4 months. In all tissues analyzed, vector copy numbers (VCNs) were 100- to 1000-fold greater in mice receiving 900 cGy compared with 200 cGy (P < .05). In mice receiving 200 cGy, VCN was similar whether ERT was stopped or given for 1 or 4 months after GT. In unconditioned mice, there was decreased survival with and without ERT, and VCN was very low to undetectable. When recipients were conditioned with 200 cGy and received transduced lineage-depleted marrow, only recipients receiving ERT (1 or 4 months) had detectable vector sequences in thymocytes. In conclusion, cytoreduction is important for the engraftment of gene-transduced HSC, and short-term ERT after GT did not diminish the capacity of gene-corrected cells to engraft and persist.

Surgical or Transcatheter Aortic-Valve Replacement in Intermediate-Risk Patients

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Reardon, Michael J; Van Mieghem, Nicolas M; Popma, Jeffrey J

2017-01-01

BACKGROUND: Although transcatheter aortic-valve replacement (TAVR) is an accepted alternative to surgery in patients with severe aortic stenosis who are at high surgical risk, less is known about comparative outcomes among patients with aortic stenosis who are at intermediate surgical risk. METHO...

Exercise therapy may postpone total hip replacement surgery in patients with hip osteoarthritis

DEFF Research Database (Denmark)

Svege, Ida; Nordsletten, Lars; Fernandes, Linda

2015-01-01

Exercise treatment is recommended for all patients with hip osteoarthritis (OA), but its effect on the long-term need for total hip replacement (THR) is unknown.......Exercise treatment is recommended for all patients with hip osteoarthritis (OA), but its effect on the long-term need for total hip replacement (THR) is unknown....

Digestibility, productive performance, and egg quality of laying hens as affected by dried cassava pulp replacement with corn and enzyme supplementation.

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Khempaka, Sutisa; Maliwan, Prapot; Okrathok, Supattra; Molee, Wittawat

2018-02-24

Two experiments were conducted to investigate the potential use of dried cassava pulp (DCP) supplemented with enzymes as an alternative feed ingredient in laying hen diets. In experiment 1, 45 laying hens (Isa Brown) aged 45 weeks were placed in individual cages to measure nutrient digestibility for 10 days. Nine dietary treatments were control and DCP as a replacement for corn at 20, 25, 30, and 35% supplemented with mixed enzymes (cellulase, glucanase, and xylanase) at 0.10 and 0.15%. Results showed that the use of DCP at 20-35% added with mixed enzymes had no negative effects on dry matter digestibility, while organic matter digestibility and nitrogen retention decreased with increased DCP up to 30-35% in diets. Both enzyme levels (0.10 and 0.15%) showed similar results on nutrient digestibility and retention. In experiment 2, a total of 336 laying hens aged 32 weeks were randomly allocated to seven dietary treatments (control and DCP-substituted diets at 20, 25, and 30%) supplemented with mixed enzymes (0.10 and 0.15%). Diets incorporated with 20-30% of DCP and supplemented with mixed enzymes at both levels had no significant effects on egg production, egg weight, feed intake, egg mass, feed conversion ratio, protein efficiency ratio, or egg quality, except for egg yolk color being decreased with an increase of DCP in diets (P digestibility, productive performance, or egg quality.

Enzyme replacement therapy prevents dental defects in a model of hypophosphatasia.

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McKee, M D; Nakano, Y; Masica, D L; Gray, J J; Lemire, I; Heft, R; Whyte, M P; Crine, P; Millán, J L

2011-04-01

Hypophosphatasia (HPP) occurs from loss-of-function mutation in the tissue-non-specific alkaline phosphatase (TNALP) gene, resulting in extracellular pyrophosphate accumulation that inhibits skeletal and dental mineralization. TNALP-null mice (Akp2(-/-)) phenocopy human infantile hypophosphatasia; they develop rickets at 1 week of age, and die before being weaned, having severe skeletal and dental hypomineralization and episodes of apnea and vitamin B(6)-responsive seizures. Delay and defects in dentin mineralization, together with a deficiency in acellular cementum, are characteristic. We report the prevention of these dental abnormalities in Akp2(-/-) mice receiving treatment from birth with daily injections of a mineral-targeting, human TNALP (sALP-FcD(10)). sALP-FcD(10) prevented hypomineralization of alveolar bone, dentin, and cementum as assessed by micro-computed tomography and histology. Osteopontin--a marker of acellular cementum--was immuno-localized along root surfaces, confirming that acellular cementum, typically missing or reduced in Akp2(-/-) mice, formed normally. Our findings provide insight concerning how acellular cementum is formed on tooth surfaces to effect periodontal ligament attachment to retain teeth in their osseous alveolar sockets. Furthermore, they provide evidence that this enzyme-replacement therapy, applied early in post-natal life--where the majority of tooth root development occurs, including acellular cementum formation--could prevent the accelerated tooth loss seen in individuals with HPP.

Effect of preoperative education of patients before hip or knee replacement surgery: A systematic review

DEFF Research Database (Denmark)

Aydin, Dogu; Klit, Jakob; Husted, Henrik

2013-01-01

Background Total hip (THA) and knee arthroplasty (TKA) are standard procedures for treatment of end stage osteoarthritis, and much is done to optimize results. It is usually hypothesized that education of patients before surgery reduces anxiety, secures more realistic patient expectations...... and enhances postoperative outcomes. Objectives To determine whether results reported in the literature proves an effect of preoperative education on postoperative outcomes in replacement surgery patients regarding anxiety, pain, length of hospital stay, patient satisfaction, postoperative complications...... visual) given by health professionals to patients were included. Results Seven studies involving 677 participants met the inclusion criteria. Two studies involved patients undergoing THA replacement, while five studies involved patients undergoing both THA and TKA replacement surgery. Mean number...

Somatic mosaicism caused by monoallelic reversion of a mutation in T cells of a patient with ADA-SCID and the effects of enzyme replacement therapy on the revertant phenotype.

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Moncada-Vélez, M; Vélez-Ortega, A; Orrego, J; Santisteban, I; Jagadeesh, J; Olivares, M; Olaya, N; Hershfield, M; Candotti, F; Franco, J

2011-11-01

Patients with adenosine deaminase (ADA) deficiency exhibit spontaneous and partial clinical remission associated with somatic reversion of inherited mutations. We report a child with severe combined immunodeficiency (T-B- SCID) due to ADA deficiency diagnosed at the age of 1 month, whose lymphocyte counts including CD4+ and CD8+ T and NK cells began to improve after several months with normalization of ADA activity in Peripheral blood lymphocytes (PBL), as a result of somatic mosaicism caused by monoallelic reversion of the causative mutation in the ADA gene. He was not eligible for haematopoietic stem cell transplantation (HSCT) or gene therapy (GT); therefore he was placed on enzyme replacement therapy (ERT) with bovine PEG-ADA. The follow-up of metabolic and immunologic responses to ERT included gradual improvement in ADA activity in erythrocytes and transient expansion of most lymphocyte subsets, followed by gradual stabilization of CD4+ and CD8+ T (with naïve phenotype) and NK cells, and sustained expansion of TCRγδ+ T cells. This was accompanied by the disappearance of the revertant T cells as shown by DNA sequencing from PBL. Although the patient's clinical condition improved marginally, he later developed a germinal cell tumour and eventually died at the age of 67 months from sepsis. This case adds to our current knowledge of spontaneous reversion of mutations in ADA deficiency and shows that the effects of the ERT may vary among these patients, suggesting that it could depend on the cell and type in which the somatic mosaicism is established upon reversion. © 2011 The Authors. Scandinavian Journal of Immunology © 2011 Blackwell Publishing Ltd.

Patients' perceptions of information and education for renal replacement therapy: an independent survey by the European Kidney Patients' Federation on information and support on renal replacement therapy

NARCIS (Netherlands)

van Biesen, Wim; van der Veer, Sabine N.; Murphey, Mark; Loblova, Olga; Davies, Simon

2014-01-01

Selection of an appropriate renal replacement modality is of utmost importance for patients with end stage renal disease. Previous studies showed provision of information to and free modality choice by patients to be suboptimal. Therefore, the European Kidney Patients' Federation (CEAPIR) explored

Dislocation of total hip replacement in patients with fractures of the femoral neck.

Science.gov (United States)

Enocson, Anders; Hedbeck, Carl-Johan; Tidermark, Jan; Pettersson, Hans; Ponzer, Sari; Lapidus, Lasse J

2009-04-01

Total hip replacement is increasingly used in active, relatively healthy elderly patients with fractures of the femoral neck. Dislocation of the prosthesis is a severe complication, and there is still controversy regarding the optimal surgical approach and its influence on stability. We analyzed factors influencing the stability of the total hip replacement, paying special attention to the surgical approach. We included 713 consecutive hips in a series of 698 patients (573 females) who had undergone a primary total hip replacement (n = 311) for a non-pathological, displaced femoral neck fracture (Garden III or IV) or a secondary total hip replacement (n = 402) due to a fracture-healing complication after a femoral neck fracture. We used Cox regression to evaluate factors associated with prosthetic dislocation after the operation. Age, sex, indication for surgery, the surgeon's experience, femoral head size, and surgical approach were tested as independent factors in the model. The overall dislocation rate was 6%. The anterolateral surgical approach was associated with a lower risk of dislocation than the posterolateral approach with or without posterior repair (2%, 12%, and 14%, respectively (p replacement in patients with femoral neck fractures.

Mucopolysaccharidosis type VI on enzyme replacement therapy since infancy: Six years follow-up of four children

Directory of Open Access Journals (Sweden)

Dafne D.G. Horovitz

2015-12-01

Full Text Available Clinical and biochemical improvements are reported on Mucopolysaccharidosis type VI (MPS VI patients on Enzyme Replacement Therapy (ERT with rhASB (galsulfase, Naglazyme®, and preclinical and clinical studies have shown clinical benefits of early initiation. We report four unrelated MPS VI children who began ERT as infants (ages 5 days–10 months. The three older patients showed the first clinical signs of MPS VI at baseline, also presenting different degrees of dysostosis multiplex, and two had mild heart disease. The two oldest also had mild facial coarseness, one had hearing conduction deficit and sleep disorder and the other corneal clouding at baseline. After six years on ERT, all four patients have normal urinary GAG values. Although they all showed normal motor and mental development, brain and cervical spine MRI images available from two of the older patients showed abnormalities, while the youngest child continues having normal images. The four patients presented slower progression of bone and joint disease when compared to their affected older siblings. It should be noticed that only two patients in this sample are currently below the 3rd percentile for height: the youngest who has a constitutional factor associated and the eldest who already presented frank dysostosis at 10 months of age. These findings confirm previous studies that report that skeletal features of the disease cannot be completely prevented despite early ERT. Heart disease already present in two of the four infants at baseline got worse over time and appeared in another patient, but the youngest child on ERT introduction still has a normal echocardiogram at six years of age; he also is the only one without corneal clouding after six years follow-up. Our results also suggest that early ERT prevented storage in spleen and liver and may also have improved or prevented progression of facial dysmorphic features, corroborating similar findings seen in previous studies

Gene therapy/bone marrow transplantation in ADA-deficient mice: roles of enzyme-replacement therapy and cytoreduction

Science.gov (United States)

Jin, Xiangyang; Wang, Xingchao; Yu, Xiao-Jin; Rozengurt, Nora; Kaufman, Michael L.; Wang, Xiaoyan; Gjertson, David; Zhou, Yang; Blackburn, Michael R.; Kohn, Donald B.

2012-01-01

Gene therapy (GT) for adenosine deaminase–deficient severe combined immune deficiency (ADA-SCID) can provide significant long-term benefit when patients are given nonmyeloablative conditioning and ADA enzyme-replacement therapy (ERT) is withheld before autologous transplantation of γ-retroviral vector-transduced BM CD34+ cells. To determine the contributions of conditioning and discontinuation of ERT to the therapeutic effects, we analyzed these factors in Ada gene knockout mice (Ada−/−). Mice were transplanted with ADA-deficient marrow transduced with an ADA-expressing γ-retroviral vector without preconditioning or after 200 cGy or 900 cGy total-body irradiation and evaluated after 4 months. In all tissues analyzed, vector copy numbers (VCNs) were 100- to 1000-fold greater in mice receiving 900 cGy compared with 200 cGy (P < .05). In mice receiving 200 cGy, VCN was similar whether ERT was stopped or given for 1 or 4 months after GT. In unconditioned mice, there was decreased survival with and without ERT, and VCN was very low to undetectable. When recipients were conditioned with 200 cGy and received transduced lineage-depleted marrow, only recipients receiving ERT (1 or 4 months) had detectable vector sequences in thymocytes. In conclusion, cytoreduction is important for the engraftment of gene-transduced HSC, and short-term ERT after GT did not diminish the capacity of gene-corrected cells to engraft and persist. PMID:22833548

Predictors of length of stay and patient satisfaction after hip and knee replacement surgery: fast-track experience in 712 patients

DEFF Research Database (Denmark)

Husted, Henrik; Holm, Gitte; Jacobsen, Steffen

2008-01-01

BACKGROUND AND PURPOSE: Very few studies have focused on patient characteristics that influence length of stay (LOS) in fast-track total hip (THR) and knee arthroplasty (TKR). The aim of this prospective study was to identify patient characteristics associated with LOS and patient satisfaction...... after total hip and knee replacement surgery. PATIENTS AND METHODS: Between September 2003 and December 2005, 712 consecutive, unselected patients (440 women) with a mean age of 69 (31-91) years were admitted for hip and knee replacement surgery at our specialized fast-track joint replacement unit....... Epidemiological, physical, and perioperative parameters were registered and correlated to LOS and patient satisfaction. RESULTS: 92% of the patients were discharged directly to their homes within 5 days, and 41% were discharged within 3 days. Age, sex, marital status, co-morbidity, preoperative use of walking...

Combination therapy in a patient with chronic neuronopathic Gaucher disease: a case report.

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Ceravolo, Ferdinando; Grisolia, Michele; Sestito, Simona; Falvo, Francesca; Moricca, Maria Teresa; Concolino, Daniela

2017-01-20

The variants of neuronopathic Gaucher disease may be viewed as a clinical phenotypic continuum divided into acute and chronic forms. The chronic neuronopathic form of Gaucher disease is characterized by a later onset of neurological symptoms and protracted neurological and visceral involvement. The first-choice treatment for nonneuronopathic Gaucher disease is enzyme replacement therapy with recombinant analogues of the deficient human enzyme glucocerebrosidase. Enzyme replacement therapy has been shown to improve hematological and bone manifestations associated with Gaucher disease, but, as with most proteins, recombinant enzymes cannot cross the blood-brain barrier, which prevents effects on neurological manifestations. Substrate reduction therapy with miglustat (N-butyldeoxynojirimycin) inhibits glucosylceramide synthase, which catalyzes the first step in glycosphingolipid synthesis. Because miglustat can cross the blood-brain barrier, it has been suggested that, combined with enzyme replacement therapy, it might be effective in treating neurological symptoms in patients with neuronopathic Gaucher disease. We report observed effects of combined enzyme replacement therapy and substrate reduction therapy in a 7-year-old Caucasian boy with neuronopathic Gaucher disease who was homozygous for L444P mutations. He had received enzyme replacement therapy from the age of 18 months, and concomitant miglustat treatment was commenced, with dosing according to body surface area uptitrated over 1 month with dietary modifications when he reached the age of 30 months. He experienced mild diarrhea after commencing miglustat therapy, which decreased in frequency/severity over time. His splenomegaly was reduced, and his hematological values and plasma angiotensin-converting enzyme activity normalized. Plasma chitotriosidase also showed substantial and sustained decreases. After 5 years of combination therapy, the patient showed no signs of neurological impairment. This case

Understanding cost of care for patients on renal replacement therapy: looking beyond fixed tariffs.

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Li, Bernadette; Cairns, John A; Fotheringham, James; Tomson, Charles R; Forsythe, John L; Watson, Christopher; Metcalfe, Wendy; Fogarty, Damian G; Draper, Heather; Oniscu, Gabriel C; Dudley, Christopher; Johnson, Rachel J; Roderick, Paul; Leydon, Geraldine; Bradley, J Andrew; Ravanan, Rommel

2015-10-01

In a number of countries, reimbursement to hospitals providing renal dialysis services is set according to a fixed tariff. While the cost of maintenance dialysis and transplant surgery are amenable to a system of fixed tariffs, patients with established renal failure commonly present with comorbid conditions that can lead to variations in the need for hospitalization beyond the provision of renal replacement therapy. Patient-level cost data for incident renal replacement therapy patients in England were obtained as a result of linkage of the Hospital Episodes Statistics dataset to UK Renal Registry data. Regression models were developed to explore variations in hospital costs in relation to treatment modality, number of years on treatment and factors such as age and comorbidities. The final models were then used to predict annual costs for patients with different sets of characteristics. Excluding the cost of renal replacement therapy itself, inpatient costs generally decreased with number of years on treatment for haemodialysis and transplant patients, whereas costs for patients receiving peritoneal dialysis remained constant. Diabetes was associated with higher mean annual costs for all patients irrespective of treatment modality and hospital setting. Age did not have a consistent effect on costs. Combining predicted hospital costs with the fixed costs of renal replacement therapy showed that the total cost differential for a patient continuing on dialysis rather than receiving a transplant is considerable following the first year of renal replacement therapy, thus reinforcing the longer-term economic advantage of transplantation over dialysis for the health service. © The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Limited diagnostic value of enzyme analysis in patients with mitochondrial tRNA mutations

DEFF Research Database (Denmark)

Wibrand, Flemming; Jeppesen, Tina Dysgaard; Frederiksen, Anja L

2010-01-01

We evaluated the diagnostic value of respiratory chain (RC) enzyme analysis of muscle in adult patients with mitochondrial myopathy (MM). RC enzyme activity was measured in muscle biopsies from 39 patients who carry either the 3243A>G mutation, other tRNA point mutations, or single, large......, respectively, in these three groups. The results indicate that RC enzyme analysis in muscle is not a sensitive test for MM in adults. In these patients, abnormal muscle histochemistry appears to be a better predictor ofMM....

Technique and Patient Selection Criteria of Right Anterior Mini-Thoracotomy for Minimal Access Aortic Valve Replacement.

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Tavakoli, Reza; Leprince, Pascal; Gassmann, Max; Jamshidi, Peiman; Yamani, Nassrin; Amour, Julien; Lebreton, Guillaume

2018-03-26

Aortic valve stenosis has become the most prevalent valvular heart disease in developed countries, and is due to the aging of these populations. The incidence of the pathology increases with growing age after 65 years. Conventional surgical aortic valve replacement through median sternotomy has been the gold standard of patient care for symptomatic aortic valve stenosis. However, as the risk profile of patients worsens, other therapeutic strategies have been introduced in an attempt to maintain the excellent results obtained by the established surgical treatment. One of these approaches is represented by transcatheter aortic valve implantation. Although the outcomes of high-risk patients undergoing treatment for symptomatic aortic valve stenosis have improved with transcatheter aortic valve replacement, many patients with this condition remain candidates for surgical aortic valve replacement. In order to reduce the surgical trauma in patients who are candidates for surgical aortic valve replacement, minimally invasive approaches have garnered interest during the past decade. Since the introduction of right anterior thoracotomy for aortic valve replacement in 1993, right anterior mini-thoracotomy and upper hemi-sternotomy have become the predominant incisional approaches among cardiac surgeons performing minimal access aortic valve replacement. Beside the location of the incision, the arterial cannulation site represents the second major landmark of minimal access techniques for aortic valve replacement. The two most frequently used arterial cannulation sites include central aortic and peripheral femoral approaches. With the purpose of reducing surgical trauma in these patients, we have opted for a right anterior mini-thoracotomy approach with a central aortic cannulation site. This protocol describes in detail a technique for minimally invasive aortic valve replacement and provides recommendations for patient selection criteria, including cardiac computer

Divergent clinical outcomes of alpha-glucosidase enzyme replacement therapy in two siblings with infantile-onset Pompe disease treated in the symptomatic or pre-symptomatic state.

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Matsuoka, Takashi; Miwa, Yoshiyuki; Tajika, Makiko; Sawada, Madoka; Fujimaki, Koichiro; Soga, Takashi; Tomita, Hideshi; Uemura, Shigeru; Nishino, Ichizo; Fukuda, Tokiko; Sugie, Hideo; Kosuga, Motomichi; Okuyama, Torayuki; Umeda, Yoh

2016-12-01

Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid α-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectancy of patients with IOPD. However, the efficacy of ERT in patients with IOPD is affected by the presence of symptoms and cross-reactive immunologic material (CRIM) status. We have treated two siblings with IOPD with ERT at different ages: the first was symptomatic and the second was asymptomatic. The female proband (Patient 1) was diagnosed with IOPD and initiated ERT at 4 months of age. Her younger sister (Patient 2) was diagnosed with IOPD at 10 days of age and initiated ERT at Day 12. Patient 1, now 6 years old, is alive but bedridden, and requires 24-hour invasive ventilation due to gradually progressive muscle weakness. In Patient 2, typical symptoms of IOPD, including cardiac failure, respiratory distress, progressive muscle weakness, hepatomegaly and myopathic facial features were largely absent during the first 12 months of ERT. Her cardiac function and mobility were well-maintained for the first 3 years, and she had normal motor development. However, she developed progressive hearing impairment and muscle weakness after 3 years of ERT. Both siblings have had low anti-rhGAA immunoglobulin G (IgG) antibody titers during ERT and have tolerated the treatment well. These results suggest that initiation of ERT during the pre-symptomatic period can prevent and/or attenuate the progression of IOPD, including cardiomyopathy, respiratory distress, and muscle weakness for first several years of ERT. However, to improve the long-term efficacy of ERT for IOPD, new strategies

Functional assessment of patients after total knee replacement

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Matla Joanna

2017-06-01

Full Text Available Introduction: In the society of the 21st century, osteoarthritis is considered one of the primary causes of the occurrence of pain and disability. Arthroplasty is the treatment of choice for advanced degenerative changes. The aim of the study was to carry out a functional assessment of patients at early stages of rehabilitation after total knee replacement.

Aorto-right atrial fistula after ascending aortic replacement or aortic value replacement

International Nuclear Information System (INIS)

Zhi Aihua; Dai Ruping; Jiang Shiliang; Lu Bin

2012-01-01

Objective: To evaluate the CT features of aorto-right atrial fistula after aortic valve replacement (AVR) or ascending aortic replacement. Methods: Eighty-seven patients with aortic-right atrial fistula underwent CT after operation. The CT features were retrospectively analyzed. Fistula was measured according to maximum width of the shunt. Results: Aorto-right atrial fistula was detected in 87 patients after aortic valve replacement or ascending aortic replacement by CT scan. Among them, 25 patients were diagnosed as mild aorto-right atrial fistula, 47 patients as moderate, and 15 patients as severe. Thirty-seven patients underwent follow-up CT.Among them, 10 patients with mild to moderate aorto-right atrial fistula were considered to have complete regression, 8 patients with mild aorto-right atrial fistula considered to have incomplete regression, 14 patients with mild to moderate aorto-right atrial fistula considered to have stable condition, and 5 patients with moderate aorto-right atrial fistula considered to have progression at the 3-month follow-up. Conclusion: CT is a useful tool for defining aorto-right atrial fistula after AVR or ascending aortic replacement and for evaluating it in follow-up. (authors)

Subgroup analysis of continuous renal replacement therapy in severely burned patients.

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Jaechul Yoon

Full Text Available Continuous renal replacement therapy (CRRT is administered to critically ill patients with renal injuries as renal replacement or renal support. We aimed to identify predictors of mortality among burn patients receiving CRRT, and to investigate clinical differences according to acute kidney injury (AKI status. This retrospective observational study evaluated 216 Korean burn patients who received CRRT at a burn intensive care unit. Patients were categorized by AKI status. Data were collected regarding arterial pH, laboratory results, ratio of arterial oxygen partial pressure to fractional inspired oxygen (PF ratio, and urine production. Among surviving patients, CRRT duration and the sequential organ failure assessment score were 6.5 days and 4.7 in the non-AKI group and 23.4 days and 7.4 in the AKI group, respectively (p = 0.003 and p = 0.008. On logistic regression analyses, mortality was significantly associated with a pH of 5.0 mEg/L (p = 0.045, creatinine levels of >2.0 mg/dL (p = 0.011, lactate levels of >2 mmol/L (p2 mmol/L, and a platelet count of 2 mg/dL. In the non-AKI group, poor outcomes were associated with lactate levels of >1.5 mmol/L, a PF ratio of 1.2 mg/dL. Duration of the CRRT application and the requirement for either renal replacement or renal support at the initiation of CRRT application are important considerations depending on its application.

Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement; Morbus Gaucher: Analyse der Knochenmarkveraenderungen in der MRT waehrend Enzymersatztherapie

Energy Technology Data Exchange (ETDEWEB)

Poll, L.W.; Koch, J.A.; Boerner, D.; Cohnen, M.; Jung, G.; Scherer, A.; Moedder, U. [Duesseldorf Univ. (DF). Inst. fuer Diagnostische Radiologie; Dahl, S. vom; Haeussinger, D. [Duesseldorf Univ. (Germany). Klinik fuer Gastroenterologie, Hepatologie und Infektiologie; Willers, R. [Rechenzentrum, Heinrich-Heine-Univ. Duesseldorf (Germany); Niederau, C. [Innere Abt., St. Josef-Hospital Oberhausen, Akademisches Lehrkrankenhaus der Univ. Essen (Germany)

2001-10-01

Purpose: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluating bone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. Materials and Methods: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T{sub 1}/T{sub 2}) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. Results: Infiltration of bm by Gaucher cells is characterized by a reduction of Sl on both T{sub 1}- and T{sub 2}-weighted sequences. Bone marrow responses were seen in 19 patients (63%) during treatment. Focal bone lesions, surrounded by a rim of reduced Sl, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. (orig.) [German] Ziel: Unter Enzymersatztherapie (enzyme replacement therapy = ERT) zeigen Patienten mit adulter Form des Morbus Gaucher, der haeufigsten lysosomalen Speicherkrankheit, eine deutliche Besserung der haematologischen und visceralen Symptome. Bislang liegen nur wenige Untersuchungen zur Analyse der Knochenveraenderungen waehrend der ERT vor. Ziel war es, die Knochenmarkveraenderungen bei Gaucher-Patienten waehrend der Enzymersatztherapie mit Alglucerase/Imiglucerase in der Magnetresonanztomographie (MRT) zu evaluieren. Material und Methoden: In einer prospektiven Untersuchung wurden 30 adulte Patienten mit gesichertem Morbus Gaucher vor und waehrend der ERT in der MRT

Dislocation of total hip replacement in patients with fractures of the femoral neck

OpenAIRE

Enocson, Anders; Hedbeck, Carl-Johan; Tidermark, Jan; Pettersson, Hans; Ponzer, Sari; Lapidus, Lasse J

2009-01-01

Background Total hip replacement is increasingly used in active, relatively healthy elderly patients with fractures of the femoral neck. Dislocation of the prosthesis is a severe complication, and there is still controversy regarding the optimal surgical approach and its influence on stability. We analyzed factors influencing the stability of the total hip replacement, paying special attention to the surgical approach. Patients and methods We included 713 consecutive hips in a series of 698 p...

Sciatic nerve block causing heel ulcer after total knee replacement in 36 patients.

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Todkar, Manoj

2005-12-01

Femoral and sciatic nerve blocks are often used for postoperative analgesia following total knee replacement surgery. In this report, we focus on cases of heel ulcers which occurred following the implementation of peripheral nerve block in concert with knee replacement surgery. In some instances, heel ulcers have resulted in delayed rehabilitation and prolonged hospital stays in this group of patients, which makes this phenomenon a potential burden on the healthcare system. Pressure points in the foot should be protected after the implementation of nerve blocks to prevent pressure sores. An awareness of this unusual complication related to knee replacement surgery is necessary to prevent its occurrence and avoid delays in patient rehabilitation and recovery.

High endothelin-converting enzyme-1 expression independently predicts poor survival of patients with esophageal squamous cell carcinoma.

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Wu, Ching-Fang; Lee, Ching-Tai; Kuo, Yao-Hung; Chen, Tzu-Haw; Chang, Chi-Yang; Chang, I-Wei; Wang, Wen-Lun

2017-09-01

Patients with esophageal squamous cell carcinoma have poor survival and high recurrence rate, thus an effective prognostic biomarker is needed. Endothelin-converting enzyme-1 is responsible for biosynthesis of endothelin-1, which promotes growth and invasion of human cancers. The role of endothelin-converting enzyme-1 in esophageal squamous cell carcinoma is still unknown. Therefore, this study investigated the significance of endothelin-converting enzyme-1 expression in esophageal squamous cell carcinoma clinically. We enrolled patients with esophageal squamous cell carcinoma who provided pretreated tumor tissues. Tumor endothelin-converting enzyme-1 expression was evaluated by immunohistochemistry and was defined as either low or high expression. Then we evaluated whether tumor endothelin-converting enzyme-1 expression had any association with clinicopathological findings or predicted survival of patients with esophageal squamous cell carcinoma. Overall, 54 of 99 patients with esophageal squamous cell carcinoma had high tumor endothelin-converting enzyme-1 expression, which was significantly associated with lymph node metastasis ( p = 0.04). In addition, tumor endothelin-converting enzyme-1 expression independently predicted survival of patients with esophageal squamous cell carcinoma, and the 5-year survival was poorer in patients with high tumor endothelin-converting enzyme-1 expression ( p = 0.016). Among patients with locally advanced and potentially resectable esophageal squamous cell carcinoma (stage II and III), 5-year survival was poorer with high tumor endothelin-converting enzyme-1 expression ( p = 0.003). High tumor endothelin-converting enzyme-1 expression also significantly predicted poorer survival of patients in this population. In patients with esophageal squamous cell carcinoma, high tumor endothelin-converting enzyme-1 expression might indicate high tumor invasive property. Therefore, tumor endothelin-converting enzyme-1 expression

Single-Dose Lignocaine-Based Blood Cardioplegia in Single Valve Replacement Patients

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Jaydip Ramani

Full Text Available Abstract OBJECTIVE: Myocardial protection is the most important in cardiac surgery. We compared our modified single-dose long-acting lignocaine-based blood cardioplegia with short-acting St Thomas 1 blood cardioplegia in patients undergoing single valve replacement. METHODS: A total of 110 patients who underwent single (aortic or mitral valve replacement surgery were enrolled. Patients were divided in two groups based on the cardioplegia solution used. In group 1 (56 patients, long-acting lignocaine based-blood cardioplegia solution was administered as a single dose while in group 2 (54 patients, standard St Thomas IB (short-acting blood-based cardioplegia solution was administered and repeated every 20 minutes. All the patients were compared for preoperative baseline parameters, intraoperative and all the postoperative parameters. RESULTS: We did not find any statistically significant difference in preoperative baseline parameters. Cardiopulmonary bypass time were 73.8±16.5 and 76.4±16.9 minutes (P=0.43 and cross clamp time were 58.9±10.3 and 66.3±11.2 minutes (P=0.23 in group 1 and group 2, respectively. Mean of maximum inotrope score was 6.3±2.52 and 6.1±2.13 (P=0.65 in group 1 and group 2, respectively. We also did not find any statistically significant difference in creatine-phosphokinase-MB (CPK-MB, Troponin-I levels, lactate level and cardiac functions postoperatively. CONCLUSION: This study proves the safety and efficacy of long-acting lignocaine-based single-dose blood cardioplegia compared to the standard short-acting multi-dose blood cardioplegia in patients requiring the single valve replacement. Further studies need to be undertaken to establish this non-inferiority in situations of complex cardiac procedures especially in compromised patients.

Long-Term Survival of Dialysis Patients with Bacterial Endocarditis Undergoing Valvular Replacement Surgery in the United States

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Leither, Maxwell D.; Shroff, Gautam R.; Ding, Shu; Gilbertson, David T.; Herzog, Charles A.

2013-01-01

Background Bacterial endocarditis in dialysis patients is associated with high mortality rates. The literature is limited regarding long-term outcomes of valvular replacement surgery and choice of prosthesis in dialysis patients with bacterial endocarditis. Methods and Results Dialysis patients hospitalized for bacterial endocarditis, 2004-2007, were studied retrospectively using data from the US Renal Data System. Long-term survival of patients undergoing valve replacement surgery with tissue or non-tissue valves was compared using the Kaplan-Meier method. A Cox proportional hazards model was used to identify independent predictors of mortality in patients undergoing valvular replacement surgery. During the study period, 11,156 dialysis patients were hospitalized for bacterial endocarditis and 1267 (11.4%) underwent valvular replacement surgery (tissue valve 44.3%, non-tissue valve 55.7%). In the valve replacement cohort, 60% were men, 50% white, 54% aged 45-64 years, and 36% diabetic. Estimated survival with tissue and non-tissue valves, respectively, at 0.5, 1, 2, and 3 years was 59% and 60%, 48% and 50%, 35% and 37%, and 25% and 30% (log rank P = 0.42). Staphylococcus was the predominant organism (66% of identified organisms). Independent predictors of mortality in patients undergoing valve replacement surgery included older age, diabetes as cause of end-stage renal disease, surgery during index hospitalization, staphylococcus as the causative organism, and dysrhythmias as a comorbid condition. Conclusions Valve replacement surgery is appropriate for well-selected dialysis patients with bacterial endocarditis, but is associated with high mortality rates. Survival does not differ with tissue or non-tissue prosthesis. PMID:23785002

Serum levels of polyamine synthesis enzymes increase in diabetic patients with breast cancer

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V Kenan Çelik

2017-09-01

Full Text Available In this study, it was aimed to investigate the relationship between diabetes and breast cancer and the detection of enzymes and ornithine levels in polyamine synthesis pathway in diabetes, breast cancer and diabetic breast cancer patients. Methods: Ornithine, arginine decarboxylase, ornithine decarboxylase and agmatinase levels have been measured in serum of all groups. Ornithine levels were measured spectrophotometrically. Arginine decarboxylase, ornithine decarboxylase and agmatinase levels were determined by ELISA kits. Results: Except for the diabetic group, the levels of enzymes in the polyamine synthesis pathway were increased in all and statistically significant (P < 0.05. The increase in the levels of agmatinase was very important among the enzymes (P < 0.001. Conclusions: Decreased levels of polyamine synthase enzymes in diabetes mellitus were found to be increased patients with breast cancer. Whether and how diabetes-based breast cancer development relates to increase activity of enzymes responsible for polyamine synthesis requires further mechanistic and prospective monitoring studies in larger patient cohorts.

Long term clinical history of an Italian cohort of infantile onset Pompe disease treated with enzyme replacement therapy.

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Parini, Rossella; De Lorenzo, Paola; Dardis, Andrea; Burlina, Alberto; Cassio, Alessandra; Cavarzere, Paolo; Concolino, Daniela; Della Casa, Roberto; Deodato, Federica; Donati, Maria Alice; Fiumara, Agata; Gasperini, Serena; Menni, Francesca; Pagliardini, Veronica; Sacchini, Michele; Spada, Marco; Taurisano, Roberta; Valsecchi, Maria Grazia; Di Rocco, Maja; Bembi, Bruno

2018-02-08

Enzyme replacement therapy (ERT) has deeply modified the clinical history of Infantile Onset Pompe Disease (IOPD). However, its long-term effectiveness is still not completely defined. Available data shows a close relationship between clinical outcome and patients' cross-reactive immunological status (CRIM), being CRIM-negative status a negative prognostic factor. At the same time limited data are available on the long-term treatment in CRIM-positive infants. A retrospective multicentre observational study was designed to analyse the long-term effectiveness of ERT in IOPD. Thirteen Italian centres spread throughout the country were involved and a cohort of 28 patients (15 females, 13 males, born in the period: February 2002-January 2013) was enrolled. IOPD diagnosis was based on clinical symptoms, enzymatic and molecular analysis. All patients received ERT within the first year of life. Clinical, laboratory, and functional data (motor, cardiac and respiratory) were collected and followed for a median period of 71 months (5 years 11 months). Median age at onset, diagnosis and start of ERT were 2, 3 and 4 months, respectively. CRIM status was available for 24/28 patients: 17/24 (71%) were CRIM-positive. Nineteen patients (67%) survived > 2 years: 4 were CRIM-negative, 14 CRIM-positive and one unknown. Six patients (5 CRIM-positive and one unknown) never needed ventilation support (21,4%) and seven (6 CRIM-positive and one unknown: 25%) developed independent ambulation although one subsequently lost this function. Brain imaging study was performed in 6 patients and showed peri-ventricular white matter abnormalities in all of them. Clinical follow-up confirmed the better prognosis for CRIM-positive patients, though a slow, progressive worsening of motor and/or respiratory functions was detected in 8 patients. These data are the result of the longest independent retrospective study on ERT in IOPD reported so far outside clinical trials. The data obtained

Earlier Pulmonary Valve Replacement in Down Syndrome Patients Following Tetralogy of Fallot Repair.

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Sullivan, Rachel T; Frommelt, Peter C; Hill, Garick D

2017-08-01

The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis. Those with Down syndrome were compared to those without. The primary outcome of interest was time from repair to pulmonary valve replacement. Secondary outcomes included pulmonary regurgitation and indexed right ventricular volume on cardiac magnetic resonance imaging. The cohort of 284 patients included 35 (12%) with Down syndrome. Transannular patch repair was performed in 210 (74%). Down syndrome showed greater degree of pulmonary regurgitation (55 ± 14 vs. 37 ± 16%, p = 0.01) without a significantly greater rate of right ventricular dilation (p = 0.09). In multivariable analysis, Down syndrome (HR 2.3, 95% CI 1.2-4.5, p = 0.02) and transannular patch repair (HR 5.5, 95% CI 1.7-17.6, p = 0.004) were significant risk factors for valve replacement. Those with Down syndrome had significantly lower freedom from valve replacement (p = 0.03). Down syndrome is associated with an increased degree of pulmonary regurgitation and earlier pulmonary valve replacement after tetralogy of Fallot repair. These patients require earlier assessment by cardiac magnetic resonance imaging to determine timing of pulmonary valve replacement and evaluation for and treatment of preventable causes of pulmonary hypertension.

Case Study of a physiotherapy treatment of a patient after total hip replacement

OpenAIRE

Havlíčková, Barbora

2013-01-01

The bachelor's thesis consists of two parts. The general part includes anatomy and kinesiology of the hip joint and also the topic of coxarthrosis as the most common reason for the total hip replacement surgery. An integral part of this part is the elaboration of a rehabilitation plan for patients undergoing such procedure. The practical part of the thesis is conceived a case study of a patient after total hip replacement because of coxarthrosis. Finally, the thesis includes the therapeutic o...

Pancreatic enzyme replacement therapy.

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Layer, P; Keller, J; Lankisch, P G

2001-04-01

Malabsorption due to severe pancreatic exocrine insufficiency is one of the most important late features of chronic pancreatitis. Generally, steatorrhea is more severe and occurs several years prior to malabsorption of other nutrients because synthesis and secretion of lipase are impaired more rapidly, its intraluminal survival is shorter, and the lack of pancreatic lipase activity is not compensated for by nonpancreatic mechanisms. Patients suffer not only from nutritional deficiencies but also from increased nutrient delivery to distal intestinal sites, causing symptoms by profound alteration of upper gastrointestinal secretory and motor functions. Adequate nutrient absorption requires delivery of sufficient enzymatic activity into the duodenal lumen simultaneously with meal nutrients. The following recommendations are based on modern therapeutic concepts: 25,000 to 40,000 units of lipase per meal using pH-sensitive pancreatin microspheres, with dosage increases, compliance checks, and differential diagnosis in case of treatment failure. Still, in most patients, lipid digestion cannot be completely normalized by current standard therapy, and future developments are needed to optimize treatment.

Enzyme replacement therapy rescues weakness and improves muscle pathology in mice with X-linked myotubular myopathy.

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Lawlor, Michael W; Armstrong, Dustin; Viola, Marissa G; Widrick, Jeffrey J; Meng, Hui; Grange, Robert W; Childers, Martin K; Hsu, Cynthia P; O'Callaghan, Michael; Pierson, Christopher R; Buj-Bello, Anna; Beggs, Alan H

2013-04-15

No effective treatment exists for patients with X-linked myotubular myopathy (XLMTM), a fatal congenital muscle disease caused by deficiency of the lipid phosphatase, myotubularin. The Mtm1δ4 and Mtm1 p.R69C mice model severely and moderately symptomatic XLMTM, respectively, due to differences in the degree of myotubularin deficiency. Contractile function of intact extensor digitorum longus (EDL) and soleus muscles from Mtm1δ4 mice, which produce no myotubularin, is markedly impaired. Contractile forces generated by chemically skinned single fiber preparations from Mtm1δ4 muscle were largely preserved, indicating that weakness was largely due to impaired excitation contraction coupling. Mtm1 p.R69C mice, which produce small amounts of myotubularin, showed impaired contractile function only in EDL muscles. Short-term replacement of myotubularin with a prototypical targeted protein replacement agent (3E10Fv-MTM1) in Mtm1δ4 mice improved contractile function and muscle pathology. These promising findings suggest that even low levels of myotubularin protein replacement can improve the muscle weakness and reverse the pathology that characterizes XLMTM.

Perioperative management of patient with Bombay blood group undergoing mitral valve replacement

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Shio Priye

2015-01-01

Full Text Available Bombay red blood cell phenotype is an extremely rare blood type for which patients can receive only autologous or Bombay phenotype red blood cells. We report a case of stenotic mitral valve with Bombay phenotype who underwent minimal invasive right lateral thoracotomy for the replacement of the mitral valve. A male patient from Bangladesh presented to the hospital with New York Heart Association III symptoms. His medical evaluation revealed severe mitral valve stenosis and mild aortic valve regurgitation. The patient received erythropoietin, intravenous iron succinate and folic acid tablets. Autologous blood transfusion was carried out. The mitral valve was replaced with a prosthetic valve successfully. After weaning off from cardiopulmonary bypass, heparinisation was corrected with protamine. Post-operatively, the patient received autologous red blood cells. The patient recovered after 1-day of inotropic support with adrenaline and milrinone, and diuretics and was discharged on the 5 th post-operative day.

Efficient rehabilitation care for joint replacement patients: skilled nursing facility or inpatient rehabilitation facility?

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Tian, Wenqiang; DeJong, Gerben; Horn, Susan D; Putman, Koen; Hsieh, Ching-Hui; DaVanzo, Joan E

2012-01-01

There has been lengthy debate as to which setting, skilled nursing facility (SNF) or inpatient rehabilitation facility (IRF), is more efficient in treating joint replacement patients. This study aims to determine the efficiency of rehabilitation care provided by SNF and IRF to joint replacement patients with respect to both payment and length of stay (LOS). This study used a prospective multisite observational cohort design. Tobit models were used to examine the association between setting of care and efficiency. The study enrolled 948 knee replacement patients and 618 hip replacement patients from 11 IRFs and 7 SNFs between February 2006 and February 2007. Output was measured by motor functional independence measure (FIM) score at discharge. Efficiency was measured in 3 ways: payment efficiency, LOS efficiency, and stochastic frontier analysis efficiency. IRF patients incurred higher expenditures per case but also achieved larger motor FIM gains in shorter LOS than did SNF patients. Setting of care was not a strong predictor of overall efficiency of rehabilitation care. Great variation in characteristics existed within IRFs or SNFs and severity groups. Medium-volume facilities among both SNFs and IRFs were most efficient. Early rehabilitation was consistently predictive of efficient treatment. The advantage of either setting is not clear-cut. Definition of efficiency depends in part on preference between cost and time. SNFs are more payment efficient; IRFs are more LOS efficient. Variation within SNFs and IRFs blurred setting differences; a simple comparison between SNF and IRF may not be appropriate.

Meal replacements as a strategy for weight loss in obese hemodialysis patients.

Science.gov (United States)

Lassemillante, Annie-Claude M; Oliver, Veronica; Hickman, Ingrid; Murray, Eryn; Campbell, Katrina L

2016-10-01

Introduction There is currently limited evidence on the use or safety of meal replacements as part of a low- or very-low-calorie diet in patients with renal insufficiency; however, these are occasionally used under dietetic supervision in clinical practice to achieve the desired weight loss for kidney transplant. This case series reports on the safety and efficacy of a weight loss practice utilizing meal replacements among hemodialysis patients, who needed to lose weight for kidney transplant. Methods Five hemodialysis patients were prescribed a modified low-calorie diet (950 kcal and 100 g protein per day) comprising three meal replacements (Optifast ® ), one main meal, and two low-potassium fruits per day. Dietary requirements and restrictions were met for all participants. Dialysis prescriptions, weight (predialysis and postdialysis), interdialytic weight gain, biochemistry, and medications were monitored during the study period for up to 12 months. Findings Participants were aged between 46 and 61 years, and the median time on the low-calorie diet was 364 days. Phosphate binders were temporarily ceased for one participant for reasons unrelated to this program and no other safety concerns were recorded. The low-calorie diet resulted in energy deficits ranging from 1170 kcal to 2160 kcal, and all participants lost weight (median 7% [range 5.2%-11.4%]). The most dramatic weight change appeared to occur by week 12, and declining adherence led to erratic weight change thereafter. Discussion This modified low-calorie diet was safe and effective to use in this population. Meal replacements are a useful weight loss strategy in hemodialysis patients, therefore, offering an alternative to usual weight loss protocols. © 2016 International Society for Hemodialysis.

Effect of enzyme inducing anticonvulsants on ethosuximide pharmacokinetics in epileptic patients

Science.gov (United States)

GIACCONE, M.; BARTOLI, A.; GATTI, G.; MARCHISELLI, R.; PISANI, F.; LATELLA, M.A.; PERUCCA, E.

1996-01-01

1To assess the effect of enzyme inducing anticonvulsants on ethosuximide pharmacokinetics, plasma ethosuximide concentrations after a single oral dose (500 mg) of the drug were compared in 12 healthy control subjects and 10 epileptic patients receiving chronic therapy with phenobarbitone, phenytoin and/or carbamazepine. 2Compared with controls, epileptic patients showed markedly shorter ethosuximide half-lives (29.0±7.8 vs 53.7±14.3 h, means±s.d., Panticonvulsants, the effect probably being mediated by stimulation of cytochrome CYP3A activity. 4The enhancement of ethosuximide clearance in patients comedicated with enzyme inducing anticonvulsants is likely to be clinically relevant. Higher ethosuximide dosages will be required to achieve therapeutic drug concentrations in these patients. PMID:8799524

Orthodontic space closure versus prosthetic replacement of missing upper lateral incisors in patients with bilateral cleft lip and palate.

Science.gov (United States)

Oosterkamp, Barbara C M; Dijkstra, Pieter U; Remmelink, Hendrik J; van Oort, Robert P; Sandham, Andrew

2010-11-01

To compare dental aesthetics and function of orthodontic space closure versus prosthetic replacement of upper lateral incisors in patients with bilateral cleft lip and palate. The predominant mode of prosthetic replacement was resin-bonded bridges. The retrospective study group consisted of 17 patients treated with orthodontic space closure (13 men, four women; median age, 27.1 years; interquartile range, 20.6 to 33.3 years) and 10 patients treated with prosthetic replacement (five men, five women; median age, 27.7 years; interquartile range, 20.9 to 39.7 years). Dental aesthetics were evaluated by the patients and by a professional panel. Mandibular function was evaluated by means of the mandibular function impairment questionnaire. The level of mandibular impairment was calculated using the Function Impairment Rating Scale. With respect to dental aesthetics, no significant differences between patients treated with orthodontic space closure and prosthetic replacement were found. With respect to function, the level of mandibular impairment was significantly higher in patients treated with prosthetic replacement compared with patients treated with orthodontic space closure, as indicated by high scores on specific masticatory functions. Orthodontic space closure and prosthetic replacement in bilateral cleft lip and palate patients produce similar results in terms of aesthetics. In terms of function, prosthetic replacement results in significantly more impairment of specific masticatory functions.

The impact of changes in LVEF and renal function on the prognosis of ICD patients after elective device replacement.

Science.gov (United States)

Vandenberk, Bert; Robyns, Tomas; Garweg, Christophe; Floré, Vincent; Foulon, Stefaan; Voros, Gabor; Ector, Joris; Willems, Rik

2017-10-01

A proportion of patients with an implantable cardioverter-defibrillator (ICD) in prevention of sudden cardiac death will only receive their first appropriate ICD therapy (AT) after device replacement. Clinical reassessment at the time of replacement could be helpful to guide the decision to replace or not in the future. All patients with an ICD for primary or secondary prevention in ischemic (ICM) or nonischemic cardiomyopathy were included in a single-center retrospective registry. The association of changes in left ventricular ejection fraction (LVEF; cut-off at 35%), worsening renal function (decrease in estimated glomerular filtration rate > 15 mL/min), and worsening New York Heart Association class at elective device replacement with mortality and AT was analyzed using adjusted Cox regression analysis. A total of 238 (33%) out of 727 patients received elective device replacement (86.1% male, 74.4% ICM, 42.9% primary prevention). During this replacement 20.2% received a device upgrade. The mean time to replacement was 6.4 ± 2.0 years and mean follow-up after replacement was 3.4 ± 3.0 years. Of patients who did not receive AT before replacement 23.1% received their first AT after replacement. Worsening renal function (hazard ratio [HR] 2.79, 95% confidence interval [CI] 1.50-5.18) and a consistently LVEF ≤35% compared to a consistently LVEF >35% (HR 2.15, 95% CI 1.10-4.19) at the time of replacement were independent predictors of mortality. Independent predictors of first AT after replacement could not be identified. Although reassessment of LVEF and renal function at replacement can be helpful in predicting total mortality, the clinical utility to guide reimplantation seemed limited. Our experience indicates that approximately 25% of patients received their first AT only after replacement. © 2017 Wiley Periodicals, Inc.

Patient-Controlled Oral Analgesia for Postoperative Pain Management Following Total Knee Replacement

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Patti Kastanias

2010-01-01

Full Text Available PURPOSE: To investigate whether patient-controlled oral analgesia (PCOA used by individuals receiving a total knee replacement could reduce pain, increase patient satisfaction, reduce opioid use and/or reduce opioid side effects when compared with traditional nurse (RN-administered oral analgesia.

Minimally invasive aortic valve replacement

DEFF Research Database (Denmark)

Foghsgaard, Signe; Schmidt, Thomas Andersen; Kjaergard, Henrik K

2009-01-01

In this descriptive prospective study, we evaluate the outcomes of surgery in 98 patients who were scheduled to undergo minimally invasive aortic valve replacement. These patients were compared with a group of 50 patients who underwent scheduled aortic valve replacement through a full sternotomy...... operations were completed as mini-sternotomies, 4 died later of noncardiac causes. The aortic cross-clamp and perfusion times were significantly different across all groups (P replacement...... is an excellent operation in selected patients, but its true advantages over conventional aortic valve replacement (other than a smaller scar) await evaluation by means of randomized clinical trial. The "extended mini-aortic valve replacement" operation, on the other hand, is a risky procedure that should...

The Effect of Replacing Fish Meal in the Diet with Enzyme-Treated Soybean Meal (HP310) on Growth and Body Composition of Rainbow Trout Fry.

Science.gov (United States)

Haghbayan, Samira; Shamsaie Mehrgan, Mehdi

2015-11-26

The potential of enzyme-treated soybean meal powder (HP310) as fish meal alternative in diets for rainbow trout weighing 1.17 ± 0.3 g was evaluated for 60 days. Fish meal was replaced with HP310 at 25%, 50%, 75% and 100% of experimental diets. A control group was also considered. The results showed that diets containing 75% and 100% HP310 had significantly higher feed conversion ratio and lower feed intake, weight gain and specific growth rate compared to fish feed diets containing higher levels of fish protein ingredients (p replacement levels of diet (p > 0.05). However increasing in level of HP310 in the diet caused a significant increase of the white blood cells (p replaced by HP310 showed the highest values of ash and moisture content among the diets and showed significantly different levels when compared with the control and other feeding treatments (p < 0.05).

HPRT Enzyme Activity of Blood Cells From Patients With Downs Syndrome

International Nuclear Information System (INIS)

Sbubber, E.K.; Abdul-Rahman, M.H.; Sultan, A.F.; Hamamy, H.A.

1998-01-01

Hypoxanthine phosphoribosyl transferase (HPRT) enzyme activity was determined in erythrocytes from 16 children (aged below one year to 11 year) with down s syndrome using 8-C 14 Hypoxanthine and radioeleelrophorsis techniques. Significant (P<0.01) reduction in HPRT enzyme activity was seen in D S children compared to that of 18 (age and sex matched) healthy children. Pure 21 - trisomic erythrocytes expressed lower enzyme activity than mosaic cell. Mothers of D S children showed significantly (P<0.01) lower enzyme activity than mothers of normal children . Reduced activity of HPRT enzyme was also observed in PHA-stimulated lymphocytes of DS children and their mothers. These results indicated that deficiency of HPRT in D S patients may contribute to the abnormal purine metabolism associated with the symptomatology of this syndrome

A Water Rehabilitation Program in Patients with Hip Osteoarthritis Before and After Total Hip Replacement.

Science.gov (United States)

Łyp, Marek; Kaczor, Ryszard; Cabak, Anna; Tederko, Piotr; Włostowska, Ewa; Stanisławska, Iwona; Szypuła, Jan; Tomaszewski, Wiesław

2016-07-25

BACKGROUND Pain associated with coxarthrosis, typically occurring in middle-aged and elderly patients, very commonly causes considerable limitation of motor fitness and dependence on pharmacotherapy. This article provides an assessment of a rehabilitation program with tailored water exercises in patients with osteoarthritis before and after total hip replacement. MATERIAL AND METHODS A total of 192 patients (the mean age 61.03±10.89) suffering from hip osteoarthritis (OA) were evaluated before and after total hip replacement (THR). The clinical study covered measurements of hip active ranges of motion (HAROM) and the forces generated by pelvis stabilizer muscles. Pain intensity was assessed according to analogue-visual scale of pain (VAS) and according to the Modified Laitinen Questionnaire. The patients were divided into 6 groups (4 treatment and 2 control). We compared 2 rehabilitation programs using kinesitherapy and low-frequency magnetic field. One of them also had specially designed exercises in the water. Statistical analysis was carried out at the significance level α=0.05. This was a cross-sectional study. RESULTS A positive effect of water exercises on a number of parameters was found in patients with OA both before and after total hip replacement surgery. We noted a significant reduction of pain (pwater exercises most significantly reduced pain in patients with OA before and after total hip replacement surgery. 2. Inclusion of water exercises in a rehabilitation program can reduce the use of medicines in patient with OA and after THR.

Assessment of 105 Patients with Angiotensin Converting Enzyme-Inhibitor Induced Angioedema

DEFF Research Database (Denmark)

Rasmussen, Eva Rye; von Buchwald, Christian; Wadelius, Mia

2017-01-01

Objective. To asses a cohort of 105 consecutive patients with angiotensin converting enzyme-inhibitor induced angioedema with regard to demographics, risk factors, family history of angioedema, hospitalization, airway management, outcome, and use of diagnostic codes used for the condition. Study...... gender was associated with a significantly higher risk of angiotensin converting enzyme-inhibitor induced angioedema. 6.7% had a positive family history of angioedema. Diabetes seemed to be a protective factor with regard to angioedema. 95% experienced angioedema of the head and neck. 4.7% needed...... Design. Cohort study. Methods. This was a retrospective cohort study of 105 patients with angiotensin converting enzyme-inhibitor induced angioedema in the period 1995-2014. Results. The cohort consisted of 67 females and 38 males (F : M ratio 1.8), with a mean age of 63 [range 26-86] years. Female...

Concerns of patients actively contemplating total knee replacement: differences by race and gender.

Science.gov (United States)

Chang, Huan J; Mehta, Priya S; Rosenberg, Aaron; Scrimshaw, Susan C

2004-02-15

To examine differences by race/ethnicity and gender in patients' concerns regarding total knee replacement (TKR). Focus groups of patients actively considering TKR were conducted. Discussion included patients' questions and concerns regarding TKR. The software ATLAS.ti was used to tabulate themes by race/ethnicity and gender. Concerns raised by focus group participants were compared with thematic content from patient joint replacement information materials. This comparison used patient literature from 3 high-volume academic TKR centers, the Arthritis Foundation, and the American Academy of Orthopedic Surgeons. All groups shared similar concerns. However, some issues were more prevalent among certain gender and racial groups. For instance, concerns regarding anesthesia were more important to white Americans and concerns regarding recovery were more important to women. Some of these concerns were not addressed in the available patient literature. Different gender and racial subgroups focus on different concerns when considering TKR. These differences may contribute to gender and race/ethnicity disparity seen in TKR use.

Divergent clinical outcomes of alpha-glucosidase enzyme replacement therapy in two siblings with infantile-onset Pompe disease treated in the symptomatic or pre-symptomatic state

OpenAIRE

Matsuoka, Takashi; Miwa, Yoshiyuki; Tajika, Makiko; Sawada, Madoka; Fujimaki, Koichiro; Soga, Takashi; Tomita, Hideshi; Uemura, Shigeru; Nishino, Ichizo; Fukuda, Tokiko; Sugie, Hideo; Kosuga, Motomichi; Okuyama, Torayuki; Umeda, Yoh

2016-01-01

Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid ?-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectan...

Late results of total shoulder replacement in patients with rheumatoid arthritis

DEFF Research Database (Denmark)

Søjbjerg, J.O.; Frich, Lars Henrik; Johannsen, H.V.

1999-01-01

at the authors' institution. Total shoulder replacement yields satisfactory short and long term results even in patients with severely destructed joints. Pain relief is reliable and significant as reported in short and long term studies. In most patients the functional result is good or acceptable. Although...... range of motion is only slightly increased, a satisfactory overall range of motion is achieved by most patients because of the unaffected scapulothoracic motion. However, deteriorating results, emphasizing the complexity of shoulder arthroplasty, were seen with increasing observation time in patients...

Arthroscopic treatment of patients with moderate arthrofibrosis after total knee replacement.

Science.gov (United States)

Jerosch, Joerg; Aldawoudy, Akram M

2007-01-01

The purpose of this study was to document the effect of arthroscopic management in patients with knee stiffness after total knee replacement. We present a case series study, in which 32 patients have been treated for moderate arthrofibrosis of the knee after total knee replacement, with the same regimen. We have excluded all cases of stiffness, because of infection, mechanical mal-alignment, loosening of the implants and other obvious reasons of stiffness of the knee, rather than pure arthrofibrosis. All patients first underwent a trial of conservative treatment before going for arthroscopic management. A pain catheter for femoral nerve block was inserted just before anesthesia for post-operative pain management. Arthroscopic arthrolysis of the intra-articular pathology was performed in a standardized technique with release of all fibrous bands in the suprapatellar pouch, reestablishing the medial and lateral gutter, release of the patella, resection of the remaining meniscal tissue or an anterior cyclops, if needed. Intensive physiotherapy and continuous passive motion were to start immediately post-operatively. All the patients were available for the follow up and they were evaluated using the knee society rating system. A total of 25 of the 32 procedures resulted in an improvement of the patients knee score. All the knees operated upon had intra-articular fibrous bands, hypertrophic synovitis and peri-patellar adhesions. A total of eight patients suffered from an anterior cyclops lesion and six patients showed pseudomenicus. In 19 cases a medial and lateral relapse of the patella was performed; only 5 patients got an isolated lateral release. The mean knee flexion was 119 degrees (100-130) at the end of arthroscopy and was 97 degrees (75-115) at the last follow up. The eight patients with extension lags decreased from 27 degrees (10 degrees-35 degrees) pre-operatively to 4 degrees (0-10) at time of follow up. The average knee society ratings increased from 70

After total knee replacement younger patients demonstrate superior balance control compared to older patients when recovering from a forward fall.

Science.gov (United States)

Street, Brian D; Gage, William

2017-05-01

National joint replacement registries have reported a substantial growth in younger knee osteoarthritic patients (controlled perturbation have shown age-related differences between younger and older healthy adults, whether similar age-related differences exist among total knee replacement patients is unknown. A total of 59 participants, including 29 unilateral total knee replacement patients (six-months post-surgery) made up the four experimental groups: 1) younger patient (54.3 (SD 7.9) years), 2) younger control (55.2 (SD 4.0) years), 3) older patient (76.9 (SD 4.7) years), and 4) older control (77.7 (SD 4.1) years). Using a tether-release method to perturb balance and simulate a forward fall, center of mass and stepping characteristics were analyzed. Younger patients recovered following the perturbation with a significantly smaller center of mass displacement compared to the older patients (14.85 (SD 0.01) v. 18.13 (SD 0.02) %ht, p=0.02); utilizing a longer (0.43 (SD 0.02) v. 0.39 (SD 0.03) m, pcontrols in center of mass displacement or recovery step characteristics (p>0.05). The younger patients demonstrated superior center of mass control in response to a forward perturbation, suggesting that younger patients would be at a reduced risk of falling when recovering from a forward-directed postural perturbation compared to older patients. Copyright © 2017 Elsevier Ltd. All rights reserved.

Posterior Slope of the Tibia Plateau in Malaysian Patients Undergoing Total Knee Replacement

Directory of Open Access Journals (Sweden)

R Yoga

2009-05-01

Full Text Available The posterior slope of the tibial plateau is an important feature to preserve during knee replacement. The correct slope aids in the amount of flexion and determines if the knee will be loose on flexion. This is a study on the posterior tibial plateau slope based on preoperative and postoperative radiographs of 100 consecutive patients who had total knee replacements. The average posterior slope of the tibia plateau was 10.1 degrees. There is a tendency for patients with higher pre-operative posterior tibial plateau slope to have higher post-operative posterior tibial plate slope.

Better prevention than cure: optimal patient preparation for renal replacement therapy.

Science.gov (United States)

Huang, Xiaoyan; Carrero, Juan Jesús

2014-03-01

A generous proportion of end-stage renal disease patients may not be adequately prepared for initiation of renal replacement therapy (RRT). Here we review potential benefits of early patient referral to nephrologists and optimal preparation for RRT. We place this evidence in the context of the epidemiological study by Kurella Tamura et al., which shows that voluntary community kidney disease screening and education is associated with better patient preparation and, importantly, improved survival upon initiation of RRT.

Late complications in patients with Björk-Shiley and St. Jude Medical heart valve replacement.

Science.gov (United States)

Horstkotte, D; Körfer, R; Seipel, L; Bircks, W; Loogen, F

1983-09-01

Valve-related complications after Björk-Shiley mitral valve implantation (n = 475), aortic valve implantation (n = 424), or mitral-aortic valve implantation (n = 119) were compared with those after St. Jude Medical mitral valve replacement (n = 173), aortic valve replacement (n = 152), or mitral-aortic valve replacement (n = 69). All patients were placed on anticoagulant therapy with phenprocoumon early after operation. All patients had a comparable follow-up time of approximately 23 months, which showed that cumulative thromboembolic rates were significantly higher after St. Jude valve implantation than after Björk-Shiley valve implantation. Reoperations were necessary because of valve thrombosis (0.46%), perivalvular leakage (2.2%), or prosthetic valve endocarditis with perivalvular regurgitation (0.46%). One Björk-Shiley mitral valve prosthesis had to be replaced because of fracture of the outlet strut. Without significant intergroup differences, hemorrhage due to anticoagulant treatment was the most frequent complication. Thromboembolic complications were significantly more frequent after Björk-Shiley mitral, aortic, and double valve replacements than after St. Jude valve implantation. This may lead to consideration of changes in the prophylaxis of thrombus formations in the St. Jude valve, especially in aortic valve replacements, in patients with sinus rhythm.

Stabilized incidence of diabetic patients referred for renal replacement therapy in Denmark

DEFF Research Database (Denmark)

Sørensen, V R; Hansen, P M; Heaf, J

2006-01-01

Despite an improvement in diabetes care during the last 20 years, the number of diabetic patients starting renal replacement therapy (RRT) has continued to increase in the Western world. The aim was to study the incidence of patients starting RRT in Denmark from 1990 to 2004. Data were obtained f...

Diagnosis, Treatment and Long-Term Follow Up of Patients with ADA Deficiency: a Single-Center Experience.

Science.gov (United States)

Baffelli, Renata; Notarangelo, Lucia D; Imberti, Luisa; Hershfield, Michael S; Serana, Federico; Santisteban, Ines; Bolda, Federica; Porta, Fulvio; Lanfranchi, Arnalda

2015-10-01

We carried out a retrospective analysis of 27 patients with Adenosine Deaminase (ADA) deficiency diagnosed in a single center from 1997 to the 2013, for evaluating whether data regarding types of disease-inducing mutations, biochemical and immunological features as well as clinical outcomes of patients treated with enzyme replacement or transplantation, were comparable to those obtained in multicenter studies. The ADA deficiency diagnosis was performed with biochemical, immunological and molecular techniques. Ten patients treated with hematopoietic stem cell transplantation and three in treatment with enzyme replacement were followed up in our center. Twenty-four different mutations were identified and five were not previously reported. Identical mutations were found among patients from the same Romani ethnic group or from the same geographical region. A more rapid recovery was observed in enzyme replacement treated patients in comparison with those transplanted that, however, showed a continuous and long-lasting improvement both in terms of immune and metabolic recovery. The data obtained in our single center are comparable with those that have been reported in multicenter surveys.

Rehabilitation of patients aged 65 and over after total hip replacement

DEFF Research Database (Denmark)

Hørdam, Britta

by a randomised clinical trial by using telephone interviews and counseling postoperatively, and testing improvement in patients´health status after THR. The last study involved testing the effect of nursing intervention by providing a measurement of patients´healthrelated quality of life.......The overall aim of this thesis was to investigate improvement in health status of patients aged 65 and over after total hip replacement (THR) as a result of nursing rehabilitation. The projects conssits of three studies, first a description of patients´health status after THR, followed...

Delirium After Transcatheter Aortic Valve Replacement.

Science.gov (United States)

Giuseffi, Jennifer L; Borges, Nyal E; Boehm, Leanne M; Wang, Li; McPherson, John A; Fredi, Joseph L; Ahmad, Rashid M; Ely, E Wesley; Pandharipande, Pratik P

2017-07-01

Postoperative delirium is associated with increased mortality. Patients undergoing transcatheter aortic valve replacement are at risk for delirium because of comorbid conditions. To compare the incidence, odds, and mortality implications of delirium between patients undergoing transcatheter replacement and patients undergoing surgical replacement. The Richmond Agitation-Sedation Scale and the Confusion Assessment Method for the Intensive Care Unit were used to assess arousal level and delirium prospectively in all patients with severe aortic stenosis who had transcatheter or surgical aortic valve replacement at an academic medical center. Multivariable logistic regression was used to determine the relationship between procedure type and occurrence of delirium. Cox regression was used to assess the association between postoperative delirium and 6-month mortality. A total of 105 patients had transcatheter replacement and 121 had surgical replacement. Patients in the transcatheter group were older (median age, 81 vs 68 years; P replacement. Delirium is less likely to develop in the transcatheter group but is associated with higher mortality in both groups. ©2017 American Association of Critical-Care Nurses.

[Valve-sparing aortic root replacement for young female patients with Marfan syndrome].

Science.gov (United States)

Nawata, Kan; Morota, T

2009-10-01

Annuloaortic ectasia is frequently related with Marfan syndrome, and Bentall procedure or aortic root replacement with valved conduit has been the conventional standard operation for this disease. Recently, some institutes have adopted valve-sparing aortic root replacement (VSARR) instead of Bentall procedure. Young female patients with Marfan syndrome who wishes for childbearing seem to be a group of good candidates of this type of operation, because it let them free from morbidities after artificial valve implantation. Valve-sparing operation should be taken into consideration when the size of Valsalva sinus reaches 45 mm for patients with Marfan syndrome and when it reaches 40 mm for patients with past histories or family histories of aortic dissection or aortic rupture. Since pregnancy is one of the most serious risk factors for aortic events, Valsalva sinus of 40 mm or larger could be the new standard for surgical indication if VSARR is applicable.

High patient satisfaction in 445 patients who underwent fast-track hip or knee replacement

DEFF Research Database (Denmark)

Specht, Kirsten; Kjaersgaard-Andersen, Per; Kehlet, Henrik

2015-01-01

BACKGROUND AND PURPOSE: Patient satisfaction is important in fast-track total hip and knee replacement (THR, TKR). We assessed: (1) how satisfied patients were with the treatment; (2) factors related to overall satisfaction; and (3) whether there was a difference between THR and TKR regarding...... length of stay (LOS) and patient satisfaction. PATIENTS AND METHODS: In this follow-up study, a consecutive series of 445 patients undergoing THR and TKR completed a questionnaire 2 weeks after discharge. LOS and short-term patient satisfaction with the fast-track management were measured. Patient...... satisfaction was measured using a numerical rating scale (NRS; 0-10). RESULTS: For THR, the median satisfaction score was 9-10 and for TKR it was 8.5-10 in all parameters. Older THR patients had higher overall satisfaction. No association was found between overall satisfaction following THR or TKR and sex...

Recombinant human acid alpha-glucosidase: high level production in mouse milk, biochemical characteristics, correction of enzyme deficiency in GSDII KO mice

NARCIS (Netherlands)

A.G.A. Bijvoet (Agnes); M.A. Kroos (Marian); F.R. Pieper (Frank); M. Van der Vliet (Martin); H.A. de Boer (Herman); A.T. van der Ploeg (Ans); M.Ph. Verbeet (Martin); A.J.J. Reuser (Arnold)

1998-01-01

textabstractGlycogen storage disease type II (GSDII) is caused by lysosomal acid alpha-glucosidase deficiency. Patients have a rapidly fatal or slowly progressive impairment of muscle function. Enzyme replacement therapy is under investigation. For large-scale, cost-effective

Comparative impact of AAV and enzyme replacement therapy on respiratory and cardiac function in adult Pompe mice

Directory of Open Access Journals (Sweden)

Darin J Falk

Full Text Available Pompe disease is an autosomal recessive genetic disorder characterized by a deficiency of the enzyme responsible for degradation of lysosomal glycogen (acid α-glucosidase (GAA. Cardiac dysfunction and respiratory muscle weakness are primary features of this disorder. To attenuate the progressive and rapid accumulation of glycogen resulting in cardiorespiratory dysfunction, adult Gaa−/− mice were administered a single systemic injection of rAAV2/9-DES-hGAA (AAV9-DES or bimonthly injections of recombinant human GAA (enzyme replacement therapy (ERT. Assessment of cardiac function and morphology was measured 1 and 3 months after initiation of treatment while whole-body plethysmography and diaphragmatic contractile function was evaluated at 3 months post-treatment in all groups. Gaa−/− animals receiving either AAV9-DES or ERT demonstrated a significant improvement in cardiac function and diaphragmatic contractile function as compared to control animals. AAV9-DES treatment resulted in a significant reduction in cardiac dimension (end diastolic left ventricular mass/gram wet weight; EDMc at 3 months postinjection. Neither AAV nor ERT therapy altered minute ventilation during quiet breathing (eupnea. However, breathing frequency and expiratory time were significantly improved in AAV9-DES animals. These results indicate systemic delivery of either strategy improves cardiac function but AAV9-DES alone improves respiratory parameters at 3 months post-treatment in a murine model of Pompe disease.

Quality of life: patient-reported outcomes after total replacement of the temporomandibular joint.

Science.gov (United States)

Kunjur, J; Niziol, R; Matthews, N S

2016-09-01

Since publication of the UK guidelines on total replacement of the temporomandibular joint (TMJ) in 2008 by the British Association of Oral and Maxillofacial Surgeons (BAOMS), pain scores, mouth opening, and diet have been used as markers of success. We have looked at quality of life (QoL) as another. We analysed the data from a single surgeon on patients who had had joints replaced and devised a questionnaire to find out about the subjective, functional, psychological, and social aspects of TMJ disease. A total of 18 patients who had the same operation were included (mean (range) age 50 (33 - 73) years, mean (range) follow up 30 (18 - 48) months). Jaw function and facial aesthetics had improved, and patients needed less analgesia. Overall, they reported a better QoL with improvements in mood and social interaction, and the activities of daily life were easier. The NHS uses QoL questionnaires to measure success in fields such as orthopaedic surgery, but currently we know of no nationally accepted questionnaire that measures success after total replacement of the TMJ. Copyright © 2016 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Anesthetic implication of tricuspid valve replacement in a patient with acute intermittent porphyria

Directory of Open Access Journals (Sweden)

Kianoush Saberi

2016-01-01

Full Text Available Facing a patient with acute intermittent porphyria (AIP, there is narrow safety margin which circumscribe all the therapeutic actions including choice of drugs. This would become even more complicated when it comes to a stressful and drug-dependent process like a cardiopulmonary bypass. According to author′s researches, no specific AIP case of tricuspid valve (TV replacement is reported recently. Furthermore, fast-track anesthesia was safely used in this 37-year-old male known the case of AIP, who was a candidate for TV replacement and removing the port catheter. The patient was extubated subsequently, only 3 h after entering the Intensive Care Unit.

Influence of Total Knee Arthroplasty on Gait Mechanics of the Replaced and Non-Replaced Limb During Stair Negotiation.

Science.gov (United States)

Standifird, Tyler W; Saxton, Arnold M; Coe, Dawn P; Cates, Harold E; Reinbolt, Jeffrey A; Zhang, Songning

2016-01-01

This study compared biomechanics during stair ascent in replaced and non-replaced limbs of total knee arthroplasty (TKA) patients with control limbs of healthy participants. Thirteen TKA patients and fifteen controls performed stair ascent. Replaced and non-replaced knees of TKA patients were less flexed at contact compared to controls. The loading response peak knee extension moment was greater in control and non-replaced knees compared with replaced. The push-off peak knee abduction moment was elevated in replaced limbs compared to controls. Loading and push-off peak hip abduction moments were greater in replaced limbs compared to controls. The push-off peak hip abduction moment was greater in non-replaced limbs compared to controls. Future rehabilitation protocols should consider the replaced knee and also the non-replaced knee and surrounding joints. Copyright © 2016 Elsevier Inc. All rights reserved.

Ranking hospitals for outcomes in total hip replacement - administrative data with or without patient surveys? - Part 2: Patient survey and administrative data

Directory of Open Access Journals (Sweden)

Schäfer, Thomas

2007-03-01

Full Text Available Background: Many hospital rankings rely on the frequency of adverse outcomes and are based on administrative data. In the study presented here, we tried to find out, to what extent available administrative data of German Sickness Funds allow for an adequate hospital ranking and compared this with rankings based on additional information derived from a patient survey. Total hip replacement was chosen as an example procedure. In part II of the publication, we present the results of the approach based on administrative and patient-derived data. Methods: We used administrative data from a large health insurance (AOK-Lower Saxony of the year 2002 and from a patient survey. The study population comprised mainly beneficiaries, who received primary total hip replacement in the year 2002, were mailed a survey 6 month post-operatively and participated in the survey. Performance indicators used where “Revision”, “Complications” and “Change of functional impairment”. Hospitals were ranked if they performed at least 20 procedures on AOK-beneficiaries. Multivariate modelling (logistic regression and generalized linear models was used to estimate the performance indicators by case-mix variables (a.o. age, sex, co-morbidity, medical history and hospital characteristics (hospital size, surgical volume. The actual ranking was based on these multivariate models, excluding hospital variables and adding dummy-variables for each hospital. Hospitals were ranked by their case-mix adjusted odds ratio or Standardized Difference (SDR with respect to a pre-selected reference hospital. The resulting rankings were compared with each other and with regard to the impact of case-mix variables. Results: 4089 beneficiaries received primary total hip replacement in 2002. 3293 patients participated in the survey (80.5%. The ranking included 60 hospitals. The agreement of rankings based on different performance indicators in the same year was low to high (a correlation

Changes in Liver Function Enzymes of HIV/AIDS Patients Treated ...

African Journals Online (AJOL)

user

on liver enzyme markers (Aspartate aminotransferase, Alanine aminotransferase ... the diagnosis and advanced infection of the liver cells by HIV. ... recommended guideline for the treatment of HIV ... HIV-positive patients not on treatment and.

Assessment of the Patient-Centered and Family-Centered Care Experience of Total Joint Replacement Patients Using a Shadowing Technique.

Science.gov (United States)

Marcus-Aiyeku, Ulanda; DeBari, Margaret; Salmond, Susan

2015-01-01

In 2030, when baby boomers reach 65 years of age and represent 18% of the population, it is anticipated that 67 million adults will have a diagnosis of arthritis increasing the demand for total hip and knee arthroplasty. With the growing emphasis on patient- and family-centered care, the aim of this project was to assess the patient experience of patients and families throughout the entire spectrum of the total joint replacement service line care at a university regional trauma hospital. A shadowing methodology as defined by the Institute for Health Improvement was utilized. Eight patient/family groups undergoing total joint replacements were shadowed. The mapped care experience included time, caregiver, activity, shadower observations, and impressions. Findings revealed inconsistencies in the delivery of patient- and family-centered care. Communication and interactions were predominantly provider-centric, with a focus on care routines versus the patient and family, and anticipation that care would be medically directed.

Artificial Disc Replacement

Science.gov (United States)

... Spondylolisthesis BLOG FIND A SPECIALIST Treatments Artificial Disc Replacement (ADR) Patient Education Committee Jamie Baisden The disc ... Disc An artificial disc (also called a disc replacement, disc prosthesis or spine arthroplasty device) is a ...

TOTAL JOINT REPLACEMENT OF THE LOWER EXTREMITY IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS

Directory of Open Access Journals (Sweden)

Стюарт Б. Гудмэн

2014-03-01

Full Text Available Joint replacement of the lower extremity in Juvenile Idiopathic Arthritis (JIA is becoming more commonly performed worldwide. These young adults experience severe pain and disability from end-stage arthritis, and require joint replacement of the hip or knee to alleviate pain, and restore ambulation and function. These procedures are very challenging from the anesthesia and surgical point of view, due to small overall proportions, numerous bony and other deformities and soft tissue contractures. Joint replacement operations for JIA are best performed by experienced teams, where pre-operative and peri-operative care, and post-operative rehabilitation can be optimized in a collaborative, patient-centered environment.

Knee injury and obesity in patients undergoing total knee replacement: a retrospective study in 115 patients

DEFF Research Database (Denmark)

Jensen, Claus Hjorth; Rofail, S

1999-01-01

The prevalence of obesity and previous knee injury was assessed in a retrospective study of 115 patients under-going total knee replacement due to osteoarthritis. Obesity was considered a contributing factor in the development of osteoarthritis in 37% of the patients, and 33% of the patients had...... had an injury to the knee in question. Unilateral osteoarthritis was significantly more frequent than bilateral osteoarthritis among patients with a history of previous knee injury. The association of previous injury to the knee and unilateral osteoarthritis was stronger in men than women. Aggressive...... treatment of patients with knee injuries seems warranted....

Aortic valve replacement in a patient with severe nickel allergy.

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Lusini, Mario; Barbato, Raffaele; Spadaccio, Cristiano; Chello, Massimo

2011-11-01

Nickel allergy can raise clinical problems in patients undergoing cardiac surgery who require sternal closure with stainless steel wire. We describe the case of a 51-year-old woman with severe nickel allergy who underwent aortic valve replacement with a nickel-free ON-X prosthesis and sternal closure by Fiberwire # 2 suture without complications. Considering its biocompatibility and its mechanical characteristics including optimal strength and knot resistance, this suture might be a viable alternative in patients in which the use of stainless steel wire is contraindicated. © 2011 Wiley Periodicals, Inc.

[Efficacy of health education on patients with hip replacement based on the Internet].

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Zhou, Yang; Yang, Tubao; Li, Yinglan; Yu, Jie; Zeng, Biyun

2015-03-01

To evaluate the efficacy of health education on patients with hip replacement based on the Internet, and to establish a new health education model through modern technology. A total of 300 patients with hip replacement from March to August, 2015 were enrolled in this study. The participants were divided into a control group and an experimental group according to months surgeries performed. Traditional education was applied in the control group while the multimedia source material plus the Internet platform of Joint Registration System were applied in the experimental group. Levels of anxiety, degree of satisfaction, and postoperative complications were analyzed. The levels of knowledge, attitude and behavior compliance in the patients of the experimental group were significantly improved, while the levels of postoperative anxiety were decreased compared with those in the control group (PEducation based on the Internet platform of Joint Registration System and the computer video could improve patients' knowledge, attitude, and behavior, which is worthy of clinical spread.

The stress response and anesthetic potency of unilateral spinal anesthesia for total Hip Replacement in geriatric patients.

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Zhu, Li; Tian, Chun; Li, Min; Peng, Ming-Qing; Ma, Kun-Long; Wang, Zhong-Lin; Ding, Jia-Hui; Cai, Yi

2014-11-01

Recently, some scholars suggested that it is important to keep a stablehemodynamic state and prevent the stress responses in geriatric patients undergoing total hip replacement (THR). We conducted this randomized prospective study to observe anesthetic potency of unilateral spinal anesthesia and stress response to it in geriatric patients during THR. We compared the effect of unilateral spinal and bilateral spinal on inhibition of stress response through measuring Norepinephrine (NE), epinephrine (E) and cortisol (CORT). Plasma concentrations of NE, E and CORT were determined in blood samples using ELISA (enzyme-linked immunosorbent assays) at three time points: To (prior to anesthesia) T1 (at the time point of skin closure), T2 (twenty-four hours after the operation). Sixty patients were randomly divided into two groups: group A (unilateral spinal anesthesia) and group B (conventional bilateral spinal anesthesia). 7.5tymg of hypobaric bupivacaine were injected into subarachnoid cavity at group A and 12mg hypobaric bupivacaine were given at group B. The onset time of sensory and motor block, loss of pinprick sensation, degree of motor block, regression of sensory and motor blocks and hemodynamic changes were also recorded. These data were used to evaluate anesthetic potency of spinal anesthesia. The results of this experiment show that unilateral spinal anesthesia can provide restriction of sensory and motor block, minimize the incidence of hypotension and prevent the stress responses undergoing THR. It is optimal anesthesia procedure for geriatric patients by rapid subarachnoid injection of small doses of bupivacaine.

Tissue Expressions of Soluble Human Epoxide Hydrolase-2 Enzyme in Patients with Temporal Lobe Epilepsy.

Science.gov (United States)

Ahmedov, Merdin Lyutviev; Kemerdere, Rahsan; Baran, Oguz; Inal, Berrin Bercik; Gumus, Alper; Coskun, Cihan; Yeni, Seher Naz; Eren, Bulent; Uzan, Mustafa; Tanriverdi, Taner

2017-10-01

We sought to simply demonstrate how levels of soluble human epoxide hydrolase-2 show changes in both temporal the cortex and hippocampal complex in patients with temporal lobe epilepsy. A total of 20 patients underwent anterior temporal lobe resection due to temporal lobe epilepsy. The control group comprised 15 people who died in traffic accidents or by falling from a height, and their autopsy findings were included. Adequately sized temporal cortex and hippocampal samples were removed from each patient during surgery, and the same anatomic structures were removed from the control subjects during the autopsy procedures. Each sample was stored at -80°C as rapidly as possible until the enzyme assay. The temporal cortex in the epilepsy patients had a significantly higher enzyme level than did the temporal cortex of the control group (P = 0.03). Correlation analysis showed that as the enzyme level increases in the temporal cortex, it also increases in the hippocampal complex (r 2  = 0.06, P = 0.00001). More important, enzyme tissue levels showed positive correlations with seizure frequency in both the temporal cortex and hippocampal complex in patients (r 2  = 0.7, P = 0.00001 and r 2  = 0.4, P = 0.003, respectively). The duration of epilepsy was also positively correlated with the hippocampal enzyme level (r 2  = 0.06, P = 0.00001). Soluble human epoxy hydrolase enzyme-2 is increased in both lateral and medial temporal tissues in temporal lobe epilepsy. Further studies should be conducted as inhibition of this enzyme has resulted in a significant decrease in or stopping of seizures and attenuated neuroinflammation in experimental epilepsy models in the current literature. Copyright © 2017 Elsevier Inc. All rights reserved.

Managing infection in the revision total hip replacement patient.

Science.gov (United States)

Halley, D K

1993-01-01

Deep infection is one of the most devastating complications in total hip replacement. This dreaded complication is presented in considerable detail with special emphasis on prevention, diagnosis, and various methods of surgical and antibiotic treatment. Basic fundamentals of antibiotic therapy are reviewed. In addition, guidelines are given for the indications of surgical intervention, the type and staging of operative procedures, with detailed techniques of the various operative procedures used to treat the infected total hip implant patient.

Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years.

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Cameron, Duke E; Alejo, Diane E; Patel, Nishant D; Nwakanma, Lois U; Weiss, Eric S; Vricella, Luca A; Dietz, Harry C; Spevak, Philip J; Williams, Jason A; Bethea, Brian T; Fitton, Torin P; Gott, Vincent L

2009-05-01

We reviewed the evolution of practice and late results of aortic root replacement (ARR) in Marfan syndrome patients at our institution. A retrospective clinical review of Marfan patients undergoing ARR at our institution was performed. Follow-up data were obtained from hospital and office records and from telephone contact with patients or their physicians. Between September 1976 and September 2006, 372 Marfan syndrome patients underwent ARR: 269 had a Bentall composite graft, 85 had valve-sparing ARR, 16 had ARR with homografts, and 2 had ARR with porcine xenografts. In the first 24 years of the study, 85% received a Bentall graft; during the last 8 years, 61% had a valve-sparing procedure. There was no operative or hospital mortality among the 327 patients who underwent elective repair; there were 2 deaths among the 45 patients (4.4%) who underwent emergent or urgent operative repair. There were 74 late deaths (70 Bentalls, 2 homograft, and 2 valve-sparing ARRs). The most frequent causes of late death were dissection or rupture of the residual aorta (10 of 74) and arrhythmia (9 of 74). Of the 85 patients who had a valve-sparing procedure, 40 had a David II remodeling operation; there was 1 late death in this group, and 5 patients required late aortic valve replacement for aortic insufficiency. A David I reimplantation procedure using the De Paulis Valsalva graft has been used exclusively since May 2002. All 44 patients in this last group have 0 to 1+ aortic insufficiency. Prophylactic surgical replacement of the ascending aorta in patients with Marfan syndrome has low operative risk and can prevent aortic catastrophe in most patients. Valve-sparing procedures, particularly using the reimplantation technique with the Valsalva graft, show promise but have not yet proven as durable as the Bentall.

Preoperative information provided to Swedish and immigrant patients before total hip replacement.

Science.gov (United States)

Krupic, Ferid; Määttä, Sylvia; Garellick, Göran; Lyckhage, Elisabeth Dahlborg; Kärrholm, Johan

2012-01-01

Total hip replacement is an operation that usually leads to pain relief and improved health related quality of life (HRQoL). Previous studies have demonstrated the importance of information about upcoming surgery. Therefore, it was of interest to study how both immigrants, whose first language was not Swedish, and Swedish patients described pre-operative information. Individual interviews were conducted with 10 immigrants and 10 Swedish participants. The data were analysed using qualitative content analysis. The study was carried out in western Sweden from March to November 2010. The findings revealed that pre-operative information for all patients undergoing elective total hip replacement was limited. Patients from both groups expressed concern about inadequate preoperative information pertaining to the surgery, implant selection, pain relief, choice of anaesthesia, no or too short a time to put questions to the surgeon and an overall stressful situation. Adequate preoperative information is important for optimising pain relief and shortening the hospital stay. The fact that the patients overwhelmingly rated the preoperative information as inadequate may be due to several reasons. Mental distress and the two-week interval between the time when the patient received the information and the operation might have contributed to the low degree of retention.

Can shared care deliver better outcomes for patients undergoing total hip replacement?

NARCIS (Netherlands)

Rosendal, H.; Beekum, W.T. van; Nijhof, P.; Witte, L.P. de; Schrijvers, A.J.P.

2000-01-01

Objectives: To assess whether shared care for patients undergoing total hip replacement delivers better outcomes compared to care as usual. Design: Prospective, observational cohort study. Setting: Two regions in the Netherlands where different organisational health care models have been

Transformation in pretreatment manifestations of Gaucher disease type 1 during two decades of alglucerase/imiglucerase enzyme replacement therapy in the International Collaborative Gaucher Group (ICGG) Gaucher Registry.

Science.gov (United States)

Mistry, Pramod K; Batista, Julie L; Andersson, Hans C; Balwani, Manisha; Burrow, Thomas Andrew; Charrow, Joel; Kaplan, Paige; Khan, Aneal; Kishnani, Priya S; Kolodny, Edwin H; Rosenbloom, Barry; Scott, C Ronald; Weinreb, Neal

2017-09-01

This study tests the hypothesis that the prevalence of severe clinical manifestations in Gaucher disease type 1 (GD1) patients at the time of treatment initiation has changed since alglucerase/imiglucerase enzyme replacement therapy (ERT) was approved in the United States (US) in 1991. US alglucerase/imiglucerase-treated GD1 patients from the International Collaborative Gaucher Group Gaucher Registry clinicaltrials.gov NCT00358943 were stratified by age at ERT initiation (<18, 18 to <50, ≥50 years), era of ERT initiation (1991-1995, 1996-2000, 2001-2005, 2006-2009), and splenectomy status pre-ERT. Prevalence of splenectomy decreased dramatically across the eras among all age groups. Bone manifestations were more prevalent in splenectomized patients than non-splenectomized patients in all age groups. Prevalence of bone manifestations differed across eras in certain age groups: non-splenectomized patients had a lower prevalence of ischemic bone events (pediatric patients) and bone crisis (pediatric patients and adults 18 to <50 years) in later eras; splenectomized adult (18 to <50 years) patients had a lower prevalence of ischemic bone events and bone crisis in later eras. Over two decades after the introduction of ERT, the prevalence of splenectomy and associated skeletal complications has declined dramatically. Concomitantly, the interval between diagnosis and initiation of ERT has decreased, most strikingly in pediatric patients who have the most severe disease. Together, these findings suggest that since the introduction of alglucerase/imiglucerase ERT, optimal standard of care has become established in the US to prevent destructive complications of GD1. © 2017 The Authors American Journal of Hematology Published by Wiley Periodicals, Inc.

A stochastic model of enzyme kinetics

Science.gov (United States)

Stefanini, Marianne; Newman, Timothy; McKane, Alan

2003-10-01

Enzyme kinetics is generally modeled by deterministic rate equations, and in the simplest case leads to the well-known Michaelis-Menten equation. It is plausible that stochastic effects will play an important role at low enzyme concentrations. We have addressed this by constructing a simple stochastic model which can be exactly solved in the steady-state. Throughout a wide range of parameter values Michaelis-Menten dynamics is replaced by a new and simple theoretical result.

Orthodontic Space Closure Versus Prosthetic Replacement of Missing Upper Lateral Incisors in Patients With Bilateral Cleft Lip and Palate

NARCIS (Netherlands)

Oosterkamp, Barbara C. M.; Dijkstra, Pieter U.; Remmelink, Hendrik J.; van Oort, Robert P.; Sandham, John

2010-01-01

Objective To compare dental aesthetics and function of orthodontic space closure versus prosthetic replacement of upper lateral incisors in patients with bilateral cleft lip and palate The predominant mode of prosthetic replacement was resin-bonded bridges Patients and Methods The retrospective

Effect of corn replacement with graded levels of wheat screening and enzyme supplementation on performance, blood lipids, viscosity and jejunal histomorphology of finisher broilers

Energy Technology Data Exchange (ETDEWEB)

Mazhari, M.; Golian, A.; Kermanshahi, H.

2015-07-01

An experiment was carried out to study the effect of corn replacement with five levels of wheat screening (0, 150, 300, 450 and 600 g/kg of diet) with (0.5 g/kg of diet) or without xylanase-glucanase enzyme on performance, blood lipids, viscosity and jejunal histomorphology of finisher broilers (25-42 days of age). Five hundred day-old Ross-308 male broiler chicks were fed by a standard commercial diet up to 24 days of age, then randomly assigned to 10 diets. Each diet was fed to five groups of ten chicks each. There was not significant differences in body weight gain (BWG), feed intake, and feed conversion ratio of birds fed with different levels of wheat screening (WS), whereas enzyme increased (p<0.05) BWG. Different levels of WS and enzyme did not have a significant effect on relative weights of carcass, breast, thigh, and abdominal fat of broilers. Relative weights of gizzard, pancreas, small and large intestine, and relative length of jejunum and jejunal and ileal viscosity were increased (p<0.05) by WS, while were decreased (p<0.05) by enzyme. The serum cholesterol level decreased (p<0.05) by increasing levels of WS. Jejunal histomorphological observations showed (p<0.05) shorter and thicker villus and lower crypt depth by increasing levels of WS, while addition of enzyme to the diets, affected (p<0.05) reversely to these parameters. The results showed that the addition of wheat screening up to an inclusion level of 600 g/kg of diet had no adverse effect on broiler performance in the finisher (25-42 d) phases whereas decreased serum cholesterol levels, increased viscosity and villus atrophy. The dietary administration of exogenous enzyme improved performance parameters and decreased viscosity and villus atrophy of broiler jejunum. (Author)

Lean body mass-based levothyroxine replacement in young athyrotic patients with differentiated carcinoma of thyroid

Directory of Open Access Journals (Sweden)

Fatima Begum

2013-01-01

Full Text Available Objective: The objective of this study was to optimize dose of levothyroxine (LT4 based on lean body mass (LBM in young athyrotic patients with differentiated carcinoma of thyroid (DCT which has not been properly addressed in Bangladesh before. Materials and Methods: Sixty patients with DCT (age, range: 20-39 years having total thyroidectomy followed by radioiodine ablative therapy (RIT and 23 euthyroid volunteers were recruited. Clinical, biochemical parameters were obtained from all patients after 2 months of RIT and on LT4 replacement at a dose of 200 μg/day as first follow up visit and also from control subjects. Then 60 patients were divided into two groups consisting of 30 patients each. Patients of Group-I received LT4 replacement based on LBM measured by dual energy X-ray absorptiometry (DXA and Group-II continued LT4 replacement in conventional dose. Patients of both groups were assessed again for same parameters at 6 to 12 months at the second visit. Results: Optimized dose of LT4 based on LBM by DXA (131 ±23 μg/day significantly reduced thyroid hormones and kept thyroid stimulating hormone (TSH in expected levels in patients of Group-I at the second visit compared to patients of Group-II who continued conventional LT4 dose (200 μg/day. Hyperthyroid symptom scale (HSS was significantly reduced to 2 ± 1 in patients of Group-I but not in patients of Group-II, HSS, 8 ±1 ( P < 0.001. Conclusion: Optimization of LT4 dose based on LBM can avoid chronic exposure of mild excess of thyroid hormone in young patients with low risk DCT.

Predictors and Outcomes of Prosthesis-Patient Mismatch After Aortic Valve Replacement.

Science.gov (United States)

Dayan, Victor; Vignolo, Gustavo; Soca, Gerardo; Paganini, Juan Jose; Brusich, Daniel; Pibarot, Philippe

2016-08-01

This study sought to evaluate predictors of prosthesis-patient mismatch (PPM) and its association with the risk of perioperative and overall mortality. PPM is associated with increased mid- and long-term mortality after surgical aortic valve replacement. Conflicting results have been reported with regard to its association with perioperative mortality. Databases were searched for studies published between 1965 and 2014. Main outcomes of interest were perioperative mortality and overall mortality. The search yielded 382 studies for inclusion. Of these, 58 articles were analyzed and their data extracted. The total number of patients included was 40,381 (39,568 surgical aortic valve replacement and 813 transcatheter aortic valve replacement). Perioperative (odds ratio: 1.54; 95% confidence interval: 1.25 to 1.91) and overall (i.e., perioperative and post-operative) mortality (hazard ratio: 1.26; 95% confidence interval: 1.16 to 1.36) was increased in patients with PPM. The impact of PPM on mortality was higher in those studies in which the mean age of the patients was body mass index (>28 kg/m(2)) compared with those with lower index. Predictors of PPM were older age, female sex, hypertension, diabetes, renal failure, larger body surface area, larger body mass index, and the utilization of a bioprosthesis. PPM increases perioperative and overall mortality proportionally to its severity. The identification of predictors for PPM may be useful to identify patients who are at higher risk for PPM. The findings of this study support the implementation of strategies to prevent PPM especially in patients <70 years of age and/or with concomitant coronary artery bypass graft. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Increased Hair Cortisol Concentrations and BMI in Patients With Pituitary-Adrenal Disease on Hydrocortisone Replacement.

Science.gov (United States)

Staufenbiel, Sabine M; Andela, Cornelie D; Manenschijn, Laura; Pereira, Alberto M; van Rossum, Elisabeth F C; Biermasz, Nienke R

2015-06-01

Intrinsic imperfections and lack of reliable biomarkers preclude optimal individual dosing of hydrocortisone replacement in adrenal insufficiency (AI). However, the clinical relevance of optimal dosing is exemplified by frequently occurring side effects of overreplacement and the dangers of underreplacement. Cortisol in scalp hair has been identified as a retrospective biomarker for long-term cortisol exposure. We compared hair cortisol concentrations (CORT(hair)) of patients with primary or secondary AI on replacement therapy with those of patient controls with a pituitary disease without AI (PCs) and of healthy controls (HCs). In this cross-sectional study, hair samples and anthropometric data were collected in 132 AI patients (52 males), 42 PCs (11 males), and 195 HCs (90 males). The proximal 3 cm of hair were used. CORT(hair) were measured using an ELISA. CORT(hair) were higher in AI patients than in HCs and PCs (P AI patients demonstrated higher CORT(hair) than female patients (P AI patients had higher body mass index (BMI) than HCs (P AI.

Permanent Quadriplegia Following Replacement of Voice Prosthesis.

Science.gov (United States)

Ozturk, Kayhan; Erdur, Omer; Kibar, Ertugrul

2016-11-01

The authors presented a patient with quadriplegia caused by cervical spine abscess following voice prosthesis replacement. The authors present the first reported permanent quadriplegia patient caused by voice prosthesis replacement. The authors wanted to emphasize that life-threatening complications may be faced during the replacement of voice prosthesis. Care should be taken during the replacement of voice prosthesis and if some problems have been faced during the procedure patients must be followed closely.

Postoperative radiation therapy after hip replacement in high-risk patients for development of heterotopic bone formation

International Nuclear Information System (INIS)

Hashem, R.; Rene, N.; Souhami, L.; Tanzer, M.; Evans, M.

2011-01-01

Purpose. - To report the results of postoperative radiation therapy in preventing the development of heterotopic bone formation after hip replacement surgery in high-risk patients. Patients and methods. - Between 1991 and 2007, 44 patients were preventively treated with postoperative RT after total hip replacement. In total, 47 hips were treated. All patients were considered at high risk for developing heterotopic bone formation. Most patients (63.5%) were treated because of a history of severe osteoarthritis or ankylosing spondylitis. All patients were treated with shaped parallel-opposed fields with a single fraction of 7 Gy using 6 or 18 MV photons. Most patients (94%) received radiation therapy within 72 hours postoperative and in only three patients radiation therapy was delivered after 72 hours post-surgery (5-8 days). Results. - Minimum follow-up was 1 year. There were 18 females and 26 males. Median age was 63 years (range: 18-80). Treatments were well tolerated and no acute toxicity was seen post-radiation therapy. Only one of the 47 hips (2%) developed heterotopic bone formation. This patient received postoperative radiation therapy to both hips but only developed heterotopic bone formation in one of them. None of the three patients treated beyond 72 hours failed. To date no late toxicity has been observed. Conclusion. - The use of postoperative radiation therapy was an effective and safe treatment in the prevention of heterotopic bone formation in a high-risk group of patients undergoing total hip replacement. (authors)

Is Total Femur Replacement a Reliable Treatment Option for Patients With Metastatic Carcinoma of the Femur?

Science.gov (United States)

Sevelda, Florian; Waldstein, Wenzel; Panotopoulos, Joannis; Kaider, Alexandra; Funovics, Philipp Theodor; Windhager, Reinhard

2018-05-01

The majority of metastatic bone lesions to the femoral bone can be treated without surgery or with minimally invasive intramedullary nailing. In rare patients with extensive metastatic disease to the femur, total femur replacement may be the only surgical alternative to amputation; however, little is known about this approach. In a highly selected small group of patients with metastatic carcinoma of the femur, we asked: (1) What was the patient survivorship after this treatment? (2) What was the implant survivorship free from all-cause revision and amputation, and what complications were associated with this treatment? (3) What functional outcomes were achieved by patients after total femur replacement for this indication? Eleven patients (three men, eight women) with a mean age of 64 years (range, 41-78 years) received total femur replacements between 1986 and 2016; none were lost to followup. The most common primary disease was breast cancer. In general, during this period, our indications for this procedure were extensive metastatic disease precluding internal fixation or isolated proximal or distal femur replacement, and an anticipated lifespan exceeding 6 months. Our contraindication for this procedure during this time was expected lifespan less than 6 months. Patient survival was assessed by Kaplan-Meier analysis; implant survival free from revision surgery and amputation were assessed by competing risk analysis. Function was determined preoperatively and 6 to 12 weeks postoperatively with the Musculoskeletal Tumor Society (MSTS) score normalized to a 100-point scale, with higher scores representing better function from a longitudinally maintained institutional database. Eleven patients died at a median of 5 months (range, 1-31 months) after surgery. One-year revision-free and limb survival were 82% (95% CI, 51%-98%) and 91% (95% CI, 61%-99%), respectively. Reasons for reoperation were hip dislocation, infection and local recurrence in one patient each. The

Valve Repair Is Superior to Replacement in Most Patients With Coexisting Degenerative Mitral Valve and Coronary Artery Diseases.

Science.gov (United States)

Javadikasgari, Hoda; Gillinov, A Marc; Idrees, Jay J; Mihaljevic, Tomislav; Suri, Rakesh M; Raza, Sajjad; Houghtaling, Penny L; Svensson, Lars G; Navia, José L; Mick, Stephanie L; Desai, Milind Y; Sabik, Joseph F; Blackstone, Eugene H

2017-06-01

For mitral regurgitation (MR) from degenerative mitral disease in patients with coexisting coronary artery disease, the appropriate surgical strategy remains controversial. From 1985 to 2011, 1,071 adults (age 70 ± 9.3 years, 77% men) underwent combined coronary artery bypass grafting and either mitral valve repair (n = 872, 81%) or replacement (n=199, 19%) for degenerative MR. Propensity matching (177 patient pairs, 89% of possible matches) was used to compare early outcomes and time-related recurrence of MR after mitral valve repair, mitral valve reoperation, and mortality. Risk factors for death were identified with multivariable, multiphase hazard-function analysis. Patients undergoing valve replacement were older, with more valve calcification and a higher prevalence of preoperative atrial fibrillation and heart failure (all p < .0001). Among matched pairs, mitral replacement versus repair was associated with higher hospital mortality (5.0% vs 1.0%, p = .0001) and more postoperative renal failure (7.0% vs 3.2%, p = .01), reexplorations for bleeding (6.0% vs 3.1%, p = .05), and respiratory failure (14% vs 4.7%, p < .0001). Of matched patients undergoing repair, 18% had MR above 3+ by 5 years. Mitral valve durability was similar between matched groups, but survival at 15 years was 18% after replacement versus 52% after repair. Nomograms from the multivariable equation revealed that in 94% of cases, 10-year survival was calculated to be higher after repair than after replacement. In patients with coexisting degenerative mitral valve and coronary artery diseases, mitral valve repair is expected to confer a long-term survival advantage over replacement despite some recurrence of MR. When feasible, it is the procedure of choice for these patients. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Salivary cortisol day curves in Addison's disease in patients on hydrocortisone replacement.

Science.gov (United States)

Ross, I L; Levitt, N S; Van der Walt, J S; Schatz, D A; Johannsson, G; Haarburger, D H; Pillay, T S

2013-01-01

Using salivary cortisol (SC) measurements, cortisol exposure in Addison's disease patients on hydrocortisone replacement was determined and compared with healthy controls. Cortisol pharmacokinetics was assessed in 31 patients with Addison's disease on replacement hydrocortisone doses (median daily dose 20 mg; range 5-50 mg) and 30 healthy control subjects. Saliva samples (n=16) were collected between 08:00 and 00:00 h in 1 day, using a passive drool technique. Cortisol exposure was evaluated by noncompartmental approach. In the patients, cortisol exposure was significantly higher than in controls: median inter-quartile range (IQR) peak cortisol (C(max)) 174.5 (59.3-837.0) vs. 6.50 (4.7-19.3) nmol/l, p=0.0001; area under the curve (AUC) 390.1 (177.1-928.9) vs. 21.4 (14.6-28.4) minutes*nmol/l, p=0.0001, trough cortisol level (C(min)) 0.49 (0.49-0.96) vs. 0.49 (0.49-0.49) nmol/l, p=0.02, occurring at 480.0 (0.1-660.0) vs. 405.0 (180.0-570.0) min, p=0.56. First peak cortisol was 174.5 (53.0-754.7) vs. 6.27 (3.90-8.47) nmol/l, p=0.0001 and second peak cortisol 18.90 (5.22-76.9) vs. 3.12 (1.76-4.79) nmol/l, p=0.0001. The time to first peak cortisol differed between the 2 groups, 30 (30-75) vs. 0.1 (0.1-30) minutes; p=0.0001. At doses studied, hydrocortisone replacement therapy results in cortisol pharmacokinetics being markedly different from endogenous cortisol profiles in healthy control subjects. Addison's disease patients had significantly higher SC levels compared to healthy control subjects. © Georg Thieme Verlag KG Stuttgart · New York.

Three-year hemodynamic performance, left ventricular mass regression, and prosthetic-patient mismatch after rapid deployment aortic valve replacement in 287 patients.

Science.gov (United States)

Haverich, Axel; Wahlers, Thorsten C; Borger, Michael A; Shrestha, Malakh; Kocher, Alfred A; Walther, Thomas; Roth, Matthias; Misfeld, Martin; Mohr, Friedrich W; Kempfert, Joerg; Dohmen, Pascal M; Schmitz, Christoph; Rahmanian, Parwis; Wiedemann, Dominik; Duhay, Francis G; Laufer, Günther

2014-12-01

Superior aortic valve hemodynamic performance can accelerate left ventricular mass regression and enhance survival and functional status after surgical aortic valve replacement. This can be achieved by rapid deployment aortic valve replacement using a subannular balloon-expandable stent frame, which functionally widens and reshapes the left ventricular outflow tract, to ensure a larger effective orifice area compared with conventional surgical valves. We report the intermediate-term follow-up data from a large series of patients enrolled in the Surgical Treatment of Aortic Stenosis With a Next Generation Surgical Aortic Valve (TRITON) trial. In a prospective, multicenter (6 European hospitals), single-arm study, 287 patients with aortic stenosis underwent rapid deployment aortic valve replacement using a stented trileaflet bovine pericardial bioprosthesis. Core laboratory echocardiography was performed at baseline, discharge, and 3 months, 1 year, and 3 years after rapid deployment aortic valve replacement. The mean patient age was 75.7 ± 6.7 years (range, 45-93; 49.1% women). The mean aortic valve gradient significantly decreased from discharge to 3 years of follow-up. The mean effective orifice area remained stable from discharge to 3 years. At 1 year, the left ventricular mass index had decreased by 14% (P replacement using a subannular balloon-expandable stent frame demonstrated excellent hemodynamic performance and significant left ventricular mass regression. With continued follow-up, future studies will establish whether these favorable structural changes correlate with improvement in long-term survival and functional status. Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Comparison of Warfarin Requirements in Post-cardiac Surgery Patients: Valve Replacement Versus Non-valve Replacement.

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Olson, Logan M; Nei, Andrea M; Joyce, David L; Ou, Narith N; Dierkhising, Ross A; Nei, Scott D

2018-01-11

Anticoagulation with warfarin affects approximately 140,000 post-cardiac surgery patients every year, yet there remains limited published data in this patient population. Dosing remains highly variable due to intrinsic risk factors that plague cardiac surgery candidates and a lack of diverse literature that can be applied to those who have undergone a cardiac surgery alternative to heart valve replacement (HVR). In the present study, our aim was to compare the warfarin requirements between HVR and non-HVR patients. This was a single-center, retrospective study of post-cardiac surgery patients initiated on warfarin at Mayo Clinic Hospital, Rochester, from January 1st, 2013 to October 31st, 2016. The primary outcome was the maintenance warfarin dose at the earliest of discharge or warfarin day 10 between patients with HVR and non-HVR cardiac surgeries. A total of 683 patients were assessed during the study period: 408 in the HVR group and 275 in the non-HVR group. The mean warfarin maintenance doses in the HVR and non-HVR groups were 2.55 mg [standard deviation (SD) 1.52] and 2.43 mg (SD 1.21), respectively (adjusted p = 0.65). A multivariable analysis was performed to adjust for gender, age, body mass index and drug interactions. This was the largest study to evaluate warfarin dose requirements in post-cardiac surgery patients and is the first to compare warfarin requirements between HVR and non-HVR patients during the immediate post-operative period. Both groups had similar warfarin requirements, which supports expanding the initial warfarin dosing recommendations of the 9th edition Chest guideline to include non-HVR patients as well as HVR patients.

Telerehabilitation Feasibility in Total Joint Replacement

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Mark J. Nelson

2017-11-01

Full Text Available Despite documented benefits, many Total Joint Replacement (TJR patients find it difficult to access rehabilitation following discharge from hospital. One solution to improve access for TJR patients is telerehabilitation. This study aimed to assess the feasibility of introducing a telerehabilitation program for TJR patients.   TJR patients at QEII Jubilee Hospital were invited to complete a questionnaire regarding their access, feelings towards and preferences in using technology. Seventy-five patients were recruited. Most patients had computer access (72% and internet (69% at home. Sixty-five percent of participants were willing to participate in telerehabilitation. A significant difference was found between older and younger patients. Watching videos on an electronic device was the preferred method for a technology-based home exercise program and phone call the preferred method of communication.  Results indicate telerehabilitation in the TJR population is feasible from the perspective of access to, feelings toward, and preferences for technology. Keywords: Hip replacement, Knee replacement, Telerehabilitation, Telemedicine, Total joint replacement

Patient outcomes using Wii-enhanced rehabilitation after total knee replacement - the TKR-POWER study.

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Negus, J J; Cawthorne, D P; Chen, J S; Scholes, C J; Parker, D A; March, L M

2015-01-01

Home-based rehabilitation following total knee replacement surgery can be as effective as clinic-based or in-patient rehabilitation. The use of the Nintendo Wii has been postulated as a novel rehabilitation tool that adds an additional focus on balance and proprioception into the recovery protocol. The aim of the proposed clinical trial is to investigate the effectiveness of this novel rehabilitation tool, used at home for three months after total knee replacement surgery and to assess any lasting improvements in functional outcome at one year. This will be a randomised controlled trial of 128 patients undergoing primary total knee replacement. The participants will be recruited preoperatively from three surgeons at a single centre. There will be no change to the usual care provided until 6 weeks after the operation. Then participants will be randomised to either the Wii-Fit group or usual rehabilitative care group. Outcomes will be assessed preoperatively, a 6-week post surgery baseline and then at 18 weeks, 6 months and 1 year. The primary outcome is the change in self-reported WOMAC total score from week 6 to 18 weeks. Secondary outcomes include objective measures of strength, function and satisfaction scores. The results of this clinical trial will be directly relevant for implementation into clinical practice. If beneficial, this affordable technology could be used by many patients to rehabilitate at home. Not only could it optimize the outcomes from their total knee replacement surgery but decrease the need for clinic-based or outpatient therapy for the majority. (ACTRN12611000291987). Copyright © 2014 Elsevier Inc. All rights reserved.

Incidence and factors associated with infective endocarditis in patients undergoing left-sided heart valve replacement

DEFF Research Database (Denmark)

Østergaard, Lauge; Valeur, Nana; Ihlemann, Nikolaj

2018-01-01

Aims: Patients with left-sided heart valve replacement are considered at high-risk of infective endocarditis (IE). However, data on the incidence and risk factors associated with IE are sparse. Methods and results: Through Danish administrative registries, we identified patients who underwent left.......35-2.15), and cardiac implantable electronic device (CIED) (HR = 1.57, 95% CI 1.19-2.06) were among factors associated with an increased risk of IE. Conclusion: Infective endocarditis after left-sided heart valve replacement is not uncommon and occurs in about 1/20 over 10 years. Male, bioprosthetic valve, and heart...

Do intra-articular hyaluronic acid injections delay total knee replacement in patients with osteoarthritis - A Cox model analysis.

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Delbarre, Angélique; Amor, Bernard; Bardoulat, Isabelle; Tetafort, Aymeric; Pelletier-Fleury, Nathalie

2017-01-01

This study aimed to describe patients treated for knee osteoarthritis between 2006 and 2013 in France and to compare the delay from diagnosis to total knee replacement between patients who received intra-articular hyaluronic acid injections and those who did not receive the injections. A second objective was to compare direct medical costs for ambulatory care between treatment groups. Patients were selected from a representative sample of the real world administrative claims database using an algorithm developed by experts from the scientific committee of the study. Data were matched with the medico-administrative database for hospital care. A Cox proportional hazards model was stratified for the treatment group and adjusted for available socio-demographic and medical covariates to compare restricted mean survival times at different time points (1, 3, 5 and 7.5 years) between groups. Costs were expressed in 2013 euros. A total of 14,782 patients were treated for knee osteoarthritis (67% women; mean age = 68 years). Among this population, 1,662 patients had total knee replacement (11.2%). At each time point, restricted mean survival time without total knee replacement was significantly higher (p-valueshyaluronic acid group, from +51 to +217 days at 1 and 7.5 years, respectively. For the year preceding total knee replacement, the means for total direct medical costs were similar between groups, €744 vs €805 for treatment and control groups, respectively, (p-value = 0.104). Intra-articular injections accounted for less than 10% of the total costs. This is the first retrospective longitudinal study involving knee osteoarthritis patients using medico-administrative databases in France. The results support the effectiveness of hyaluronic acid injections in delaying total knee replacement and show that patients treated with hyaluronic acid have similar direct medical costs for ambulatory care compared to patients treated with corticosteroids only.

Angiotensin-converting enzyme inhibitor use and protection against pneumonia in patients with diabetes

NARCIS (Netherlands)

van de Garde, Ewoudt M W; Souverein, Patrick C; Hak, Eelko; Deneer, Vera H M; van den Bosch, Jules M M; Leufkens, Hubert G M

OBJECTIVES: Because of the high risk of pneumonia in patients with diabetes, we aimed to assess the effect of angiotensin-converting enzyme (ACE) inhibitor use on the occurrence of pneumonia in a general population of patients with diabetes. METHODS: The study population comprised all patients in

Therapeutic Enzymes: Applications and Approaches to Pharmacological Improvement.

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Yari, Maryam; Ghoshoon, Mohammad B; Vakili, Bahareh; Ghasemi, Younes

2017-01-01

Among therapeutic proteins, enzymes represent small and of course profitable market. They can be used to treat important, rare, and deadly diseases. Enzyme therapy is the only available treatment for certain disorders. Here, pharmaceutical enzymes are reviewed. They are categorized in four main groups, enzymes in replacement therapy, enzymes in cancer treatment, enzymes for fibrinolysis, and finally enzymes that are used topically for various treatments. Furthermore, enzyme gene therapy and future perspective of therapeutic enzymes are mentioned in brief. There are many important approved enzymes in pharmaceutical market. Several approaches such as point mutation, fusion protein designing, glycoengineering, and PEGylation were used to achieve improved enzymes. Although sometimes enzymes were engineered to facilitate production and purification process, appropriate delivery to target sites, extending half-life, and reducing immunogenicity are among the main goals of engineering approaches. Overall, enzymes play a critical role in treatment of common and rare diseases. Evaluation of new enzymes as well as improvement of approved enzymes are of the most important challenges in biotechnology. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Decreased plasma cholesterol esterification and cholesteryl ester transfer in hypopituitary patients on glucocorticoid replacement therapy

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Beentjes, JAM; Van Tol, A; Sluiter, WJ; Dullaart, RPF

Cardiovascular risk is increased in hypopituitary patients. No data are available with respect to the effect of glucocorticoid replacement therapy on high density lipoproteins (HDL) metabolism in such patients. Plasma lecithin:choresterol acyl transferase (LCAT), cholesteryl ester transfer protein

Serum Antioxidative Enzymes Levels and Oxidative Stress Products in Age-Related Cataract Patients

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Dong Chang

2013-01-01

Full Text Available Purpose. To investigate the activity of antioxidative enzymes and the products of oxidative stress in patients with age-related cataracts and compare the findings with those in healthy control subjects. Method. Sixty patients with age-related cataract and sixty healthy controls of matched age and gender were included in this study. Serum samples were obtained to detect the antioxidative enzymes of superoxide dismutase (SOD, catalase (CAT, and glutathione peroxidase (GSH-Px, and oxidation degradation products of malondialdehyde (MDA, 4-hydroxynonenal (4-HNE, conjugated diene (CD, advanced oxidation protein products (AOPP, protein carbonyl (PC, and 8-hydroxydeoxyguanosine (8-OHdG. Results. Serum SOD, GSH-Px, and CAT activities in cataract group were significantly decreased as compared to the control subjects (P<0.05. The levels of MDA, 4-HNE, and CD in cataract patients were significantly higher than those in the control subjects (P<0.05, P<0.01. Cataract patients had higher levels of 8-OHdG, AOPP, and PC with respect to the comparative group of normal subjects (P<0.01. And there was no statistical significance in concentration of antioxidative enzymes and oxidative stress products in patients with different subtype cataract. Conclusions. Oxidative stress is an important risk factor in the development of age-related cataract, and augmentation of the antioxidant defence systems may be of benefit to prevent or delay cataractogenesis.

Prolonged Intermittent Renal Replacement Therapy.

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Edrees, Fahad; Li, Tingting; Vijayan, Anitha

2016-05-01

Prolonged intermittent renal replacement therapy (PIRRT) is becoming an increasingly popular alternative to continuous renal replacement therapy in critically ill patients with acute kidney injury. There are significant practice variations in the provision of PIRRT across institutions, with respect to prescription, technology, and delivery of therapy. Clinical trials have generally demonstrated that PIRRT is non-inferior to continuous renal replacement therapy regarding patient outcomes. PIRRT offers cost-effective renal replacement therapy along with other advantages such as early patient mobilization and decreased nursing time. However, due to lack of standardization of the procedure, PIRRT still poses significant challenges, especially pertaining to appropriate drug dosing. Future guidelines and clinical trials should work toward developing consensus definitions for PIRRT and ensure optimal delivery of therapy. Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

Enzymes: The possibility of production and applications

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Petronijević Živomir B.

2003-01-01

Full Text Available Enzymes are biological catalysts with increasing application in the food pharmaceutical, cosmetic, textile and chemical industry. They are also important as reagents in chemical analysis, leather fabrications and as targets for the design of new drugs. Keeping in mind the growing need to replace classical chemical processes by alternative ones, because of ever growing environmental pollution, it is important that enzyme and other biotechnological processes are economical. Therefore, price decrease and stability and enzyme preparation efficiency increase are required more and more. This paper presents a short review of methods for yield increase and the improvement of the quality of enzyme products as commercial products, as well as a review of the possibilities of their application.

Testosterone Replacement and Bone Mineral Density in Male Pituitary Tumor Patients

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Min Jeong Lee

2014-03-01

Full Text Available BackgroundHypopituitarism is associated with osteoporosis and osteopenia especially when hypogonadotropic hypogonadism is present. Despite hypopituitarism being an important cause of secondary osteoporosis, osteoporosis in patients receiving surgery for pituitary tumors in Korea has not been studied. In this study, we evaluated the effects of testosterone replacement therapy (TRT on bone mineral density (BMD in postoperative hypogonadal patients with pituitary tumors.MethodsTo examine the effect of TRT on BMD, we performed a retrospective observational study in 21 postoperative male patients who underwent pituitary tumor surgery between 2003 and 2012 at the Ajou University Hospital. Testosterone was replaced in postoperative hypogonadal patients by regular intramuscular injection, daily oral medication, or application of transdermal gel. BMD (g/cm2 measurements of central skeletal sites (lumbar spine, femoral neck, and total femur were obtained using dual-energy X-ray absorptiometry (GE Lunar. For lumbar spine BMD, L1 to L4 values were chosen for analysis. Femur neck and total femur were also analyzed.ResultsDuring the follow-up period (mean, 56 months; range, 12 to 99 months serum testosterone levels increased with the administration of TRT (P=0.007. There was significant improvement (4.56%±9.81% in the lumbar spine BMD compared to baseline BMD. There were no significant changes in the femur neck BMD or total femur BMD. We did not find any statistically significant relationships between changes in testosterone levels and BMD using Spearman correlation analysis.ConclusionOur results indicated that TRT used in the postoperative period for hypogonadal pituitary tumor surgery patients may have beneficial effects on the BMD of the spine.

Long-term enzyme replacement therapy is associated with reduced proteinuria and preserved proximal tubular function in women with Fabry disease

DEFF Research Database (Denmark)

Prabakaran, Thaneas; Birn, Henrik; Bibby, Bo M

2014-01-01

dysfunction in women with Fabry disease treated with ERT. METHODS: A retrospective, single centre, cohort study evaluated the long-term association between ERT, albuminuria and eGFR in 13 women with Fabry disease and mild renal involvement. In particular, we analysed the changes in the proteinuric profile...... to end-stage renal failure. In women with Fabry disease, accumulation of GL-3 in the glomerular podocytes and other renal cells induces progressive, proteinuric nephropathy, but not as severe as in men. Enzyme replacement therapy (ERT) with recombinant α-Gal A reduces cellular GL-3 deposits in podocytes...... in albuminuria was paralleled by a decrease in both glomerular and tubular urine protein markers. CONCLUSIONS: The data indicate that long-term ERT is associated with a reduction in albuminuria and glomerular and tubular urinary protein markers in women with Fabry disease and mild renal manifestations....

Levels of muscle enzymes in the serum after esophageal pneumatic dilation in patients with achalasia.

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Kimchi, N A; Ron, Y; Abramowich, D; Shirin, H; Scapa, E; Avni, Y

2005-01-01

The success rate of pneumatic dilation of the esophagus in patients with achalasia is variable. We aim to assess whether levels of muscle enzymes in the serum are useful for predicting the efficacy of this procedure. Consecutive adults with symptomatic achalasia treated with pneumatic dilation were included. Blood samples were taken immediately before the procedure and after 12, 24 and 32 h. Clinical efficacy of the pneumatic dilation was evaluated on the basis of a symptom score defined prior to, and 2 months after the procedure. Eleven patients underwent 13 pneumatic dilations. In nine patients this was the first dilation attempt. Ten dilations were clinically effective. The study was discontinued after enzyme levels did not show a trend of increase in any of our patients. Moreover, a statistically significant unexpected decrease in creatine phosphokinase values was found 12 h after the procedure, among the 10 successful dilations. We believe that levels of muscle enzymes in the serum cannot predict the efficacy of pneumatic dilation in patients with achalasia.

Pharmacogenetic Risk Stratification in Angiotensin-Converting Enzyme Inhibitor-Treated Patients with Congestive Heart Failure

DEFF Research Database (Denmark)

Nelveg-Kristensen, Karl Emil; Busk Madsen, Majbritt; Torp-Pedersen, Christian

2015-01-01

BACKGROUND: Evidence for pharmacogenetic risk stratification of angiotensin-converting enzyme inhibitor (ACEI) treatment is limited. Therefore, in a cohort of ACEI-treated patients with congestive heart failure (CHF), we investigated the predictive value of two pharmacogenetic scores...... SNPs of the angiotensin-converting enzyme gene (rs4343) and ABO blood group genes (rs495828 and rs8176746). METHODS: Danish patients with CHF enrolled in the previously reported Echocardiography and Heart Outcome Study were included. Subjects were genotyped and categorized according to pharmacogenetic.......05 [95% CI 0.79-1.40]), respectively. CONCLUSIONS: We found no association between either of the analyzed pharmacogenetic scores and fatal outcomes in ACEI-treated patients with CHF....

Enzymes - important players in green chemistry

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Agata Tarczykowska

2017-09-01

Full Text Available Green chemistry has become a worldwide approach that leads to sustainable growth through application and development of its principles. A lot of work has to be put into designing new processes comprising of materials which do not emit pollutants to the atmosphere. Inventing new safer methods and finding less harmful products can be challenging. Enzymes are a great hope of scientists in the field of green chemistry. Enzymes as catalysts require mild conditions therefore it is a great way of saving resources such as energy or water. Processes with the use of enzymes have become more feasible by being more cost effective and eco friendly. Taking into account the benefits of green chemistry, enzyme biocatalysis has quickly replaced traditional chemical processes in several fields, and this substitution is going to reach even more areas because of new emerging technologies in enzyme engineering.

Use of cardiac magnetic resonance imaging to evaluate cardiac structure, function and fibrosis in children with infantile Pompe disease on enzyme replacement therapy.

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Barker, Piers C A; Pasquali, Sara K; Darty, Stephen; Ing, Richard J; Li, Jennifer S; Kim, Raymond J; DeArmey, Stephanie; Kishnani, Priya S; Campbell, Michael J

2010-12-01

Pompe disease (acid α-glucosidase deficiency) is one of several lysosomal storage diseases amenable to treatment with enzyme replacement therapy (ERT). While echocardiography (echo) has been the standard method to evaluate the cardiac response to ERT, cardiac magnetic resonance imaging (CMR) has the advantage of a better tissue definition and characterization of myocardial fibrosis. However, CMR for Pompe disease is not frequently performed due to a high risk of sedation. We report the first use of CMR in a feasible protocol to quantify left ventricular (LV) mass, function, and the presence of myocardial fibrosis in the Pompe population. Children with Pompe disease on ERT were assessed with transthoracic echo and CMR over a 3 year period at a single institution. Echocardiography was performed using standard techniques without sedation. CMR was performed using retrospectively gated and real-time imaging, with and without sedation. LV mass indexed to body surface area (LVMI) and ejection fraction (EF) were measured by both echo and CMR, and evaluated for change over time. Myocardial fibrosis was assessed by CMR with delayed enhancement imaging 5-10 min after gadolinium contrast using single shot inversion recovery sequences with inversion time set to null the signal from normal myocardium. Seventeen CMR scans were successfully performed in 10 subjects with Pompe disease (median age at first CMR is 9 months, range 1-38 months, 80% male), with sedation only performed in 4 studies. There was a median interval of 5 months (range 0-34 months) from the start of ERT to first CMR (baseline). At baseline, the median indexed LVMI by CMR (140.0 g/m(2), range 43.8-334.0) tended to be lower than that assessed by echo (median 204.0 g/m(2), range 52.0-385.0), but did not reach statistical significance. At baseline, CMR EF was similar to that assessed by echo (55% vs. 55%). Overall, there was no significant decrease in CMR measured LVMI over time (CMR median LVMI at baseline 94 g

The patient's experience of temporary paralysis from spinal anaesthesia, a part of total knee replacement

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Bager, Louise; Konradsen, Hanne; Dreyer, Pia Sander

2015-01-01

AIMS AND OBJECTIVES: The aim of this study was to describe the meaning of being temporary paralysed from spinal anaesthesia when undergoing total knee replacement. BACKGROUND: Total knee arthroplasty is a common procedure, and regional anaesthesia is used as a method for anaesthetising the patient...... of the experience of spinal anaesthesia, as a part of having a total knee replacement. METHODS: Twelve patients were interviewed in March 2014 after undergoing an elective total knee arthroplasty under spinal anaesthesia. The interviews were analysed with a Ricoeur-inspired interpretation method. RESULTS: Three...... TO CLINICAL PRACTICE: The results of this study contribute to insights and deeper knowledge that can enhance staff's ability to provide care for patients undergoing total knee arthroplasty in spinal anaesthesia. The results provide perspectives that argue for care in accordance to individual needs....

Identification of bacteriology and risk factor analysis of asymptomatic bacterial colonization in pacemaker replacement patients.

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Xian-Ming Chu

Full Text Available Recent researches revealed that asymptomatic bacterial colonization on PMs might be ubiquitous and increase the risk of clinical PM infection. Early diagnosis of patients with asymptomatic bacterial colonization could provide opportunity for targeted preventive measures.The present study explores the incidence of bacterial colonization of generator pockets in pacemaker replacement patients without signs of infection, and to analyze risk factors for asymptomatic bacterial colonization.From June 2011 to December 2013, 118 patients underwent pacemaker replacement or upgrade. Identification of bacteria was carried out by bacterial culture and 16S rRNA sequencing. Clinical risk characteristics were analyzed.The total bacterial positive rate was 37.3% (44 cases, and the coagulase-negative Staphylococcus aureus detection rate was the highest. Twenty two (18.6% patients had positive bacterial culture results, of which 50% had coagulase-negative staphylococcus. The bacterial DNA detection rate was 36.4 % (43 cases. Positive bacterial DNA results from pocket tissues and the surface of the devices were 22.0% and 29.7%, respectively. During follow-up (median, 27.0 months, three patients (6.8%, 3/44 became symptomatic with the same genus of microorganism, S. aureus (n=2 and S. epidermidis (n=1. Multivariable logistic regression analysis showed that history of bacterial infection, use of antibiotics, application of antiplatelet drugs, replacement frequency were independent risk factors for asymptomatic bacterial colonization.There was a high incidence of asymptomatic bacterial colonization in pacemaker patients with independent risk factors. Bacterial culture combined genetic testing could improve the detection rate.

Breakfast replacement with a low-glycaemic response liquid formula in patients with type 2 diabetes: a randomised clinical trial.

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Stenvers, Dirk J; Schouten, Lydia J; Jurgens, Jordy; Endert, Erik; Kalsbeek, Andries; Fliers, Eric; Bisschop, Peter H

2014-08-28

Low-glycaemic index diets reduce glycated Hb (HbA1c) in patients with type 2 diabetes, but require intensive dietary support. Using a liquid meal replacement with a low glycaemic response (GR) may be an alternative dietary approach. In the present study, we investigated whether breakfast replacement with a low-GR liquid meal would reduce postprandial glycaemia and/or improve long-term glycaemia. In the present randomised, controlled, cross-over design, twenty patients with type 2 diabetes consumed either a breakfast replacement consisting of an isoenergetic amount of Glucerna SR or a free-choice breakfast for 3 months. Postprandial AUC levels were measured using continuous glucose measurement at home. After the 3-month dietary period, meal profiles and oral glucose tolerance were assessed in the clinical setting. The low-GR liquid meal replacement reduced the AUC of postprandial glucose excursions at home compared with a free-choice control breakfast (estimated marginal mean 141 (95 % CI 114, 174) v. estimated marginal mean 259 (95 % CI 211, 318) mmol × min/l; P= 0·0002). The low-GR liquid meal replacement also reduced glucose AUC levels in the clinical setting compared with an isoenergetic control breakfast (low GR: median 97 (interquartile range (IQR) 60-188) mmol × min/l; control: median 253 (IQR 162-386) mmol × min/l; Pmeal replacement did not affect fasting plasma glucose, HbA1c or lipid levels, and even slightly reduced oral glucose tolerance. In conclusion, the low-GR liquid meal replacement is a potential dietary approach to reduce postprandial glycaemia in patients with type 2 diabetes. However, clinical trials into the effects of replacing multiple meals on long-term glycaemia in poorly controlled patients are required before a low-GR liquid meal replacement can be adopted as a dietary approach to the treatment of type 2 diabetes.

Left Ventricular Function Improves after Pulmonary Valve Replacement in Patients with Previous Right Ventricular Outflow Tract Reconstruction and Biventricular Dysfunction

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Kane, Colin; Kogon, Brian; Pernetz, Maria; McConnell, Michael; Kirshbom, Paul; Rodby, Katherine; Book, Wendy M.

2011-01-01

Congenital heart defects that have a component of right ventricular outflow tract obstruction, such as tetralogy of Fallot, are frequently palliated in childhood by disruption of the pulmonary valve. Although this can provide an initial improvement in quality of life, these patients are often left with severe pulmonary valve insufficiency. Over time, this insufficiency can lead to enlargement of the right ventricle and to the deterioration of right ventricular systolic and diastolic function. Pulmonary valve replacement in these patients decreases right ventricular volume overload and improves right ventricular performance. To date, few studies have examined the effects of pulmonary valve replacement on left ventricular function in patients with biventricular dysfunction. We sought to perform such an evaluation. Records of adult patients who had undergone pulmonary valve replacement from January 2003 through November 2006 were analyzed retrospectively. We reviewed preoperative and postoperative echocardiograms and calculated left ventricular function in 38 patients. In the entire cohort, the mean left ventricular ejection fraction increased by a mean of 0.07 after pulmonary valve replacement, which was a statistically significant change (P < 0.01). In patients with preoperative ejection fractions of less than 0.50, mean ejection fractions increased by 0.10. We conclude that pulmonary valve replacement in patients with biventricular dysfunction arising from severe pulmonary insufficiency and right ventricular enlargement can improve left ventricular function. Prospective studies are needed to verify this finding. PMID:21720459

Simulation of Post-Thyroidectomy Treatment Alternatives for Triiodothyronine or Thyroxine Replacement in Pediatric Thyroid Cancer Patients

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Ben-Shachar, Rotem; Huang, Stephen A.; DiStefano, Joseph J.

2012-01-01

Background As in adults, thyroidectomy in pediatric patients with differentiated thyroid cancer is often followed by 131I remnant ablation. A standard protocol is to give normalizing oral thyroxine (T4) or triiodothyronine (T3) after surgery and then withdraw it for 2 to 6 weeks. Thyroid remnants or metastases are treated most effectively when serum thyrotropin (TSH) is high, but prolonged withdrawals should be avoided to minimize hypothyroid morbidity. Methods A published feedback control system model of adult human thyroid hormone regulation was modified for children using pediatric T4 kinetic data. The child model was developed from data for patients ranging from 3 to 9 years old. We simulated a range of T4 and T3 replacement protocols for children, exploring alternative regimens for minimizing the withdrawal period, while maintaining normal or suppressed TSH during replacement. The results are presented with the intent of providing a quantitative basis to guide further studies of pediatric treatment options. Replacement was simulated for up to 3 weeks post-thyroidectomy, followed by various withdrawal periods. T4 vs. T3 replacement, remnant size, dose size, and dose frequency were tested for effects on the time for TSH to reach 25 mU/L (withdrawal period). Results For both T3 and T4 replacement, higher doses were associated with longer withdrawal periods. T3 replacement yielded shorter withdrawal periods than T4 replacement (up to 3.5 days versus 7–10 days). Higher than normal serum T3 concentrations were required to normalize or suppress TSH during T3 monotherapy, but not T4 monotherapy. Larger remnant sizes resulted in longer withdrawal periods if T4 replacement was used, but had little effect for T3 replacement. Conclusions T3 replacement yielded withdrawal periods about half those for T4 replacement. Higher than normal hormone levels under T3 monotherapy can be partially alleviated by more frequent, smaller doses (e.g., twice a day). LT4 may be the

David I reimplantation procedure for aortic root replacement in Marfan patients: medium-term outcome.

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Kari, Fabian A; Beyersdorf, Friedhelm; Rylski, Bartosz; Stephens, Elizabeth H; Russe, Maximilian; Siepe, Matthias

2014-11-01

Technical variations of the David reimplantation valve-sparing aortic root replacement (V-SARR) procedure have been proposed to be advantageous in patients with connective tissue disease, such as the Marfan syndrome (MFS). We report results of a Marfan cohort treated exclusively with the non-modified David I procedure. Forty-eight Marfan patients (25 males, mean age 33 ± 12 years, range 15-62 years) underwent the original variant of the David V-SARR (David I) between 1997 and 2013. Forty-two operations (88%) were performed as elective procedures for aortic root aneurysms and six for acute dissections (12%). Seventeen had aortic regurgitation (AR) grades ≥2+ preoperatively, and 3 had AR >2+. No patients with severe AR (4+) were selected for V-SARR. Three full or hemi-arch replacements were performed. Patients who were operated on using a variation of the David I or David II procedure were excluded. Mean prosthesis size was 28 ± 3 mm (18-30 mm). Mean clinical and echocardiographic follow-up (98% complete) was 3.8 ± 3.7 years with a cumulative follow-up of 178 patient-years. The early mortality rate was 2% (one hospital death). The survival rate was 98% (95% confidence 84-99%) at 4 years and 90% (57-98%) at 8 years with 5 patients at risk at 10 years. The rate of freedom from root or valve reoperation was 97% (79-99%) and 97% (79-99%) at 4 and 8 years, respectively. Only one patient required mechanical aortic valve replacement for progression of AR. Despite potential theoretical drawbacks of the David I V-SARR technique without neo-sinuses or a neo-sinotubular junction, it results in a favourable mid-term outcome in Marfan patients and compares well with reported results of different modifications of David V-SARR. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Penile Prosthesis First and Replacement Surgeries: Analysis of Patient and Partner Satisfaction.

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Lledó-García, Enrique; Jara-Rascón, José; Moncada Iribarren, Ignacio; Piñero-Sánchez, Javier; Aragón-Chamizo, Juan; Hernández-Fernández, Carlos

2015-07-01

Among the many treatments for erectile dysfunction, implantation of a penile prosthesis has been associated with high patient satisfaction rates. Prosthesis replacement has become an accepted procedure in the event of device malfunction or complications, but to our knowledge, there are no data regarding the impact of implant replacement on patients and partner satisfaction. The aim of our study was to assess and to compare the level of satisfaction, with a first or second penile prosthesis implantation (PPI), in men with refractory erectile dysfunction and their partners. A survey study based on a five-item questionnaire was carried out at our center between January 1999 and January 2012. The main outcome measure used was the level of patient and partner satisfaction with sexual intercourse after PPI. Of the 190 eligible patients, 149 (78%) completed the survey (110 underwent a first implant and 39 a reimplant). Seventy-nine percent of first-time implanted patients and 80% of the reimplanted patients (P > 0.05; not significant [ns]) reported satisfactory sexual intercourse (very or moderately satisfied), while 74% and 80% of their partners reported satisfactory intercourses, respectively (P > 0.05; ns). Overall, 73.7% of first implants and 70% of second implants reported that they would undergo the procedure again if the PPI failed (P > 0.05; ns). With regards to cosmetic aspects, 13% of the first implants' and 15% of second implants' partners reported either penile shortness or soft glans as the main causes of their dissatisfaction. Only 2.4% of first implants and 1% of reimplanted patients expressed difficulty in manipulating the device. PPI is successful in returning the ability for satisfactory sexual intercourse to both first implant and reimplanted patients and their respective partners. © 2015 International Society for Sexual Medicine.

Cardiac implantable electronic device and associated risk of infective endocarditis in patients undergoing aortic valve replacement

DEFF Research Database (Denmark)

Østergaard, Lauge; Valeur, Nana; Bundgaard, Henning

2017-01-01

Aims: Patients undergoing aortic valve replacement (AVR) are at increased risk of infective endocarditis (IE) as are patients with a cardiac implantable electronic device (CIED). However, few data exist on the IE risk after AVR surgery in patients with a CIED. Methods and results: Using the Danish...

Replacing and representing patients: Professional feelings and plastic body replicas in nursing education

DEFF Research Database (Denmark)

Soffer, Ann Katrine Bønnelykke

2015-01-01

This article examines the intimate profession of nursing and the affective practices that bind bodies together into engagements that appear stable. However, once the bodies of patients are replaced with ‘plastic replicas’ for the purpose of clinical training within an educational setting, new...

Time-based analysis of total cost of patient episodes: a case study of hip replacement.

Science.gov (United States)

Peltokorpi, Antti; Kujala, Jaakko

2006-01-01

Healthcare in the public and private sectors is facing increasing pressure to become more cost-effective. Time-based competition and work-in-progress have been used successfully to measure and improve the efficiency of industrial manufacturing. Seeks to address this issue. Presents a framework for time based management of the total cost of a patient episode and apply it to the six sigma DMAIC-process development approach. The framework is used to analyse hip replacement patient episodes in Päijät-Häme Hospital District in Finland, which has a catchment area of 210,000 inhabitants and performs an average of 230 hip replacements per year. The work-in-progress concept is applicable to healthcare--notably that the DMAIC-process development approach can be used to analyse the total cost of patient episodes. Concludes that a framework, which combines the patient-in-process and the DMAIC development approach, can be used not only to analyse the total cost of patient episode but also to improve patient process efficiency. Presents a framework that combines patient-in-process and DMAIC-process development approaches, which can be used to analyse the total cost of a patient episode in order to improve patient process efficiency.

[Evaluation of the antithrombotic strategy in low thrombotic risk patients who underwent aortic valve replacement with a bioprosthesis].

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Aceves-Velázquez, Eduardo; Vieyra-Herrera, Gerardo; Rodríguez-Chávez, Laura; Herrera-Alarcón, Valentín

2017-07-16

According to current guidelines, in patients without additional risk factors who have undergone aortic valve replacement with a bioprosthesis, anticoagulation in the first 3 months after surgery is still a matter of debate. According to current evidence, aspirin in low doses is a reasonable alternative to vitamin K antagonists (VKA). A comparison is made between the incidence of thrombotic and haemorrhagic complications in patients with low thrombotic risk who underwent aortic valve replacement with a bioprosthesis in the National Institute of Cardiology of Ignacio Chávez of Mexico. The hypothesis: aspirin as monotherapy has a beneficial effect compared to VKA. The studied patients were the low thrombotic risk patients who underwent aortic valve replacement with a bioprosthesis in the National Institute of Cardiology of Ignacio Chávez of Mexico from 2011 to 2015. The groups studied were: aspirin only, VKA only, and the combination of VKA plus aspirin. The patients were retrospectively followed-up for 12 months, and the thrombotic and haemorrhagic complications were documented. Of the 231 patients included in the study, only one patient in the VKA only group presented with a haemorrhagic complication. No thrombotic complications were observed. In the present study no thrombotic complications were observed in patients who did not receive anticoagulation in the first 3 months after an aortic valve replacement with a bioprosthesis after a follow up period of 12 months. This suggests that the use of aspirin only is safe during this period. Copyright © 2017 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

The use of transcatheter aortic valve replacement vs surgical aortic valve replacement for the treatment of aortic stenosis

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Jensen HA

2015-08-01

Full Text Available Hanna A Jensen, Lillian L Tsai, Vinod H Thourani Division of Cardiothoracic Surgery, Joseph B Whitehead Department of Surgery, Structural Heart and Valve Center, Emory University School of Medicine, Atlanta, GA, USA Abstract: Severe aortic stenosis (AS is associated with considerable morbidity and mortality and is increasing in prevalence as the global population increases. Since AS primarily affects the elderly, many of these patients have comorbidities that make them poor candidates for the gold standard treatment for AS, surgical aortic valve replacement. Transcatheter aortic valve replacement has emerged as a novel technology for the management of AS in higher risk patients over the past decade. Randomized trials have established the safety and efficacy of transcatheter aortic valve replacement, and the medical community has rallied to identify the patients who are most suitable for this transformative treatment. This review focuses on outlining the key procedural differences, describing the unique challenges of both operations, and finally assessing and comparing outcomes both on a general level and in challenging patient subgroups. Keywords: aortic valve replacement, transcatheter aortic valve replacement, surgical aortic valve replacement 

Echocardiographic and clinical findings in patients with Fabry disease during long-term enzyme replacement therapy

DEFF Research Database (Denmark)

Madsen, Christoffer Valdorff; Bundgaard, Henning; Rasmussen, Åse Krogh

2017-01-01

from baseline to follow-up; 30 mm [15-53] vs. 25 mm [3-44], p vs. 1520 mm·ms [550-5740], p within the non-ERT group. During follow-up, cardiac symptoms and use of cardiovascular procedures and -medication increased...... significantly in the ERT group, whereas no differences were observed within the non-ERT group. DISCUSSION: We raise concerns regarding the efficacy and benefit of ERT on cardiac involvement in Fabry disease and stress the need for further research....... and Holter-monitoring. RESULTS: We included 66 patients; 47 patients (27 women) received ERT (ERT group) and 19 patients (15 women) did not (non-ERT group). The groups were followed for a median of 8 [0-12] years and 6 [0-13] years, respectively. Comparison between ERT and non-ERT receiving patients by left...

Liquid L-thyroxine versus tablet L-thyroxine in patients on L- thyroxine replacement or suppressive therapy: a meta-analysis.

Science.gov (United States)

Laurent, Irakoze; Tang, Siying; Astère, Manirakiza; Wang, Kan Ran; Deng, Shuhua; Xiao, Ling; Li, Qi Fu

2018-03-23

To compare the effectiveness of liquid L-T4 (L-thyroxine) and tablet L-T4 in patients on L-T4 replacement or suppressive therapy. The Cochrane Library, PubMed, Embase, and Web of Science databases were searched to identify relevant articles. All prospective or randomized controlled studies (RCTs) comparing liquid L-T4 and tablet L-T4 in patients on L-T4 replacement or suppressive therapy were included in the analysis. Overall, the initial search of the four databases identified 1278 published studies; of these, eight studies were ultimately included in the meta-analysis. TSH (thyroid stimulating hormone) levels were significantly suppressed in patients on liquid L-T4 compared with those on tablet L-T4, in patients on L-T4 suppressive therapy with L-T4 malabsorption (Mean Difference (MD) = -2.26, 95% Confidence Interval (CI): -3.59, -0.93; P = 0.0009)). However, liquid L-T4 and tablet L-T4 did not show a statistically significant difference in patients on L-T4 suppressive therapy without malabsorption (MD = 0.08, 95% CI: -0.31, 0.47; P = 0.69). TSH levels were significantly normalized in patients on liquid L-T4 compared with those on tablet L-T4, in Patients on L-T4 replacement therapy with L-T4 malabsorption (MD = -3.20, 95% CI: -5.08, -1.32; P = 0.0009). However, liquid L-T4 and tablet L-T4 did not show a statistically significant difference in patients on L-T4 replacement therapy without malabsorption (MD = 0.91, 95% CI: -0.03, 1.86; P = 0.06). Liquid L-T4 is more efficient than tablet L-T4 in patients on L-T4 replacement or suppressive therapy with malabsorption. No significant differences were observed in patients without malabsorption. Further studies should be conducted to verify these findings.

Early and late results of graft replacement for dissecting aneurysm of thoracoabdominal aorta in patients with Marfan syndrome.

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Omura, Atsushi; Tanaka, Akiko; Miyahara, Shunsuke; Sakamoto, Toshihito; Nomura, Yoshikatsu; Inoue, Takeshi; Oka, Takanori; Minami, Hitoshi; Okada, Kenji; Okita, Yutaka

2012-09-01

When treating dissecting aneurysm of the thoracoabdominal aorta surgically in patients with Marfan syndrome, we have usually performed graft replacement- including the entire thoracoabdominal aorta and reconstruction of all visceral branches, even if dilatation is mild in some segments-to avoid further aortic operations in the follow-up period. From October 1999 through July 2011, 20 consecutive patients with Marfan syndrome underwent repair of dissecting aneurysm of the thoracoabdominal aorta (median age, 45 years; range, 19-65 years). All patients underwent surgical intervention with cerebrospinal fluid (CSF) drainage and distal aortic and selective organ perfusion. Deep hypothermia was used in 13 patients for spinal cord protection. No in-hospital mortality was observed. One patient had temporary spinal cord ischemia but was fully recovered by discharge. Other complications included exploration for bleeding (n=1), prolonged ventilation (n=1), and graft infection (n=1). At a mean follow-up of 54 months (range, 9-129 months), 1 patient had died of interstitial pneumonia at 38 months postoperatively. Survival at 8% years was 91.2±9.0%. Two patients required additional aortic procedures (total arch replacement and aortic valve-sparing surgery). Actuarial rate of freedom from aortic operations at 8 years was 83.9%±10.5%, but no patient needed required repeated thoracotomy for an aortic procedure. Neither false nor patch aneurysms were observed using computed tomography (CT) during follow-up surveillance. Graft replacement for dissecting aneurysm of the thoracoabdominal aorta in Marfan syndrome offers good early and long-term results. We believe total aortic replacement including the entire thoracoabdominal aorta and reconstruction of all visceral arteries should be recommended for selected patients with Marfan syndrome. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Hepatic Enzyme Alterations in HIV Patients on Antiretroviral Therapy: A Case-Control Study in a Hospital Setting in Ghana.

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Osakunor, Derick Nii Mensah; Obirikorang, Christian; Fianu, Vincent; Asare, Isaac; Dakorah, Mavis

2015-01-01

Diagnosing hepatic injury in HIV infection can be a herculean task for clinicians as several factors may be involved. In this study, we sought to determine the effects of antiretroviral therapy (ART) and disease progression on hepatic enzymes in HIV patients. A case-control study conducted from January to May 2014 at the Akwatia Government Hospital, Eastern region, Ghana, The study included 209 HIV patients on ART (designated HIV-ART) and 132 ART-naive HIV patients (designated HIV-Controls). Data gathered included demography, clinical history and results of blood tests for hepatic enzymes. We employed the Fisher's, Chi-square, unpaired t-test and Pearson's correlation in analysis, using GraphPad Prism and SPSS. A P value 0.05). There was a significant positive correlation between hepatic enzymes (ALP, ALT, AST and GGT) for both groups (p enzymes for both groups was small. Antiretroviral therapy amongst this population has minimal effects on hepatic enzymes and does not suggest modifications in therapy. Hepatic injury may occur in HIV, even in the absence of ART and other traditional factors. Monitoring of hepatic enzymes is still important in HIV patients.

Improved left ventricular function after growth hormone replacement in patients with hypopituitarism: assessment with radionuclide angiography

International Nuclear Information System (INIS)

Cuocolo, A.; Nicolai, E.; Colao, A.; Longobardi, S.; Cardei, S.; Fazio, S.; Merola, B.; Lombardi, G.; Sacca, L.; Salvatore, M.

1996-01-01

Prolonged growth hormone deficiency (GHD) leads to marked cardiac dysfunction; however, whether reversal of this abnormality may be achieved after specific replacement therapy has not yet been completely clarified. Fourteen patients with childhood-onset GHD (nine men and five women, mean age 27±4 years) and 12 normal control subjects underwent equilibrium radionuclide angiography under control conditions at rest. Patients with GHD were also studied 6 months after recombinant human (rh) GH treatment (0.05 IU/kg per day). Normal control subjects and patients with GHD did not differ with respect to age, gender and heart rate. In contrast, left ventricular ejection fraction (53%±9% vs 66%±6%, P 2 , P 2 , P 2 , P 2 , P <0.01) was observed in GHD patients. In conclusion, prolonged lack of GH leads to impaired left ventricular function at rest. Reversal of this abnormality may be observed after 6 months of specific replacement therapy in patients with childhood-onset GHD. (orig.). With 4 figs., 1 tab

Genotype of metabolic enzymes and the benefit of tamoxifen in postmenopausal breast cancer patients

International Nuclear Information System (INIS)

Wegman, Pia; Vainikka, Linda; Stål, Olle; Nordenskjöld, Bo; Skoog, Lambert; Rutqvist, Lars-Erik; Wingren, Sten

2005-01-01

Tamoxifen is widely used as endocrine therapy for oestrogen-receptor-positive breast cancer. However, many of these patients experience recurrence despite tamoxifen therapy by incompletely understood mechanisms. In the present report we propose that tamoxifen resistance may be due to differences in activity of metabolic enzymes as a result of genetic polymorphism. Cytochrome P450 2D6 (CYP2D6) and sulfotransferase 1A1 (SULT1A1) are polymorphic and are involved in the metabolism of tamoxifen. The CYP2D6*4 and SULT1A1*2 genotypes result in decreased enzyme activity. We therefore investigated the genotypes of CYP2D6 and SULT1A1 in 226 breast cancer patients participating in a trial of adjuvant tamoxifen treatment in order to validate the benefit from the therapy. The patients were genotyped using PCR followed by cleavage with restriction enzymes. Carriers of the CYP2D6*4 allele demonstrated a decreased risk of recurrence when treated with tamoxifen (relative risk = 0.28, 95% confidence interval = 0.11–0.74, P = 0.0089). A similar pattern was seen among the SULT1A1*1 homozygotes (relative risk = 0.48, 95% confidence interval = 0.21–1.12, P = 0.074). The combination of CYP2D6*4 and/or SULT1A1*1/*1 genotypes comprised 60% of the patients and showed a 62% decreased risk of distant recurrence with tamoxifen (relative risk = 0.38, 95% confidence interval = 0.19–0.74, P = 0.0041). The present study suggests that genotype of metabolic enzymes might be useful as a guide for adjuvant endocrine treatment of postmenopausal breast cancer patients. However, results are in contradiction to prior hypotheses and the present sample size is relatively small. Findings therefore need to be confirmed in a larger cohort

Minimally invasive unicompartmental knee replacement: retrospective clinical and radiographic evaluation of 83 patients.

Science.gov (United States)

Bruni, Danilo; Iacono, Francesco; Russo, Alessandro; Zaffagnini, Stefano; Marcheggiani Muccioli, Giulio Maria; Bignozzi, Simone; Bragonzoni, Laura; Marcacci, Maurilio

2010-06-01

We performed a retrospective clinical and radiographic evaluation of 83 nonconsecutive patients operated in our institute between February 1996 and March 2003 with a mean follow-up of 60 months to assess the efficiency of unicompartmental knee replacement (UKR) performed with a minimally invasive technique. The aim of this study was to correlate the clinical outcome with the pre- and post-operative alignment and with implant positioning on coronal and sagittal plane. Eighty-three nonconsecutive patients (60 males, 23 females) underwent cemented UKR (De Puy Preservation Uni with all-poly tibial component), for both medial OA (80 patients) and AVN of the medial femoral condyle (3 patients). All patients were available at final follow-up evaluation, and they all presented an evident varus alignment at pre-operative clinical and radiographic evaluation. At radiographic measurement, we considered a knee with femoro-tibial angle (FTA) > 175 degrees as varus knee, 170 degrees FTA FTA 90 degrees for valgus knee and a TPA clinical result presented a mean varus deformity of 7.2 degrees (3.6 degrees-10.8 degrees) pre-operatively. According to literature, we demonstrated that a small amount of undercorrection with a small amount of residual varus deformity of 3 degrees-5 degrees is the goal to be reached in order to avoid both rapid degeneration of the nonreplaced compartment and the premature loosening of the replaced compartment. We performed a mean axial correction of 5 degrees (SD 3.9 degrees), leaving a mean axial varus deformity of 2.2 degrees in the excellent group. In our series, the group with excellent results also showed a post-operative PTS of 7 degrees (2.4 degrees-11.6 degrees), while mean pre-operative PTS was 6.5 degrees (2.7 degrees-10.3 degrees). In this study, results have shown that minimally invasive UKR producing a small amount of varus undercorrection in selected patients with medial tibio-femoral osteoarthritis or moderate avascular necrosis of the

Mechanical stress is associated with right ventricular response to pulmonary valve replacement in patients with repaired tetralogy of Fallot.

Science.gov (United States)

Tang, Dalin; Yang, Chun; Del Nido, Pedro J; Zuo, Heng; Rathod, Rahul H; Huang, Xueying; Gooty, Vasu; Tang, Alexander; Billiar, Kristen L; Wu, Zheyang; Geva, Tal

2016-03-01

Patients with repaired tetralogy of Fallot account for a substantial proportion of cases with late-onset right ventricular failure. The current surgical approach, which includes pulmonary valve replacement/insertion, has yielded mixed results. Therefore, it may be clinically useful to identify parameters that can be used to predict right ventricular function response to pulmonary valve replacement. Cardiac magnetic resonance data before and 6 months after pulmonary valve replacement were obtained from 16 patients with repaired tetralogy of Fallot (8 male, 8 female; median age, 42.75 years). Right ventricular ejection fraction change from pre- to postpulmonary valve replacement was used as the outcome. The patients were divided into group 1 (n = 8, better outcome) and group 2 (n = 8, worst outcome). Cardiac magnetic resonance-based patient-specific computational right ventricular/left ventricular models were constructed, and right ventricular mechanical stress and strain, wall thickness, curvature, and volumes were obtained for analysis. Our results indicated that right ventricular wall stress was the best single predictor for postpulmonary valve replacement outcome with an area under the receiver operating characteristic curve of 0.819. Mean values of stress, strain, wall thickness, and longitudinal curvature differed significantly between the 2 groups with right ventricular wall stress showing the largest difference. Mean right ventricular stress in group 2 was 103% higher than in group 1. Computational modeling and right ventricular stress may be used as tools to identify right ventricular function response to pulmonary valve replacement. Large-scale clinical studies are needed to validate these preliminary findings. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Enzymes of industrial purpose - review of the market of enzyme preparations and prospects for its development

Directory of Open Access Journals (Sweden)

A. A. Tolkacheva

2017-01-01

Full Text Available Microbial enzyme preparations are increasingly replacing conventional chemical catalysts in a number of industrial processes. Such drugs, in addition to environmental friendliness and high activity, have a number of advantages over enzyme preparations of vegetable and animal origin, namely: the production of microbial enzymes in bioreactors is easily controlled and predictable; excreted microbiological enzymes are more stable than intracellular animals and plant enzymes; the genetic diversity of microorganisms makes it possible to produce enzyme preparations with a wide range of specificity; microbiological enzymes can be synthesized year-round, in contrast to the production of plant enzymes, which is often seasonal. The leaders of the world market of enzymes are proteases and amylases, which account for 25% and 15%, respectively. Over the past five years, the world market for carbohydrases, including mainly amylases, cellulases and xylanases, has been the fastest growing segment of the enzyme market with an aggregate annual growth rate of more than 7.0%. Another major product of the industrial enzyme market, which has a great potential for growth, is lipases. From the point of view of designation, the main part is represented by food and food enzymes. The Russian market continues to be unsaturated - the current supply is not able to meet the needs of the Russian feed and food industry in enzyme preparations. Enzyme preparations of domestic producers are in demand in forage production, while food industrial enterprises prefer imported products. The most significant enterprises in the enzymatic industry in Russia at the moment are Sibbiofarm, AgroSistema, Agroferment. In the light of the Russian policy of increasing food security, the development of the domestic enzyme industry is an extremely topical task.

Mechanical Aortic Valve Replacement in Octogenarian

Directory of Open Access Journals (Sweden)

Irfan Tasoglu

2013-10-01

Full Text Available Aim: This study analyzes the long-term outcomes of mechanical aortic valve replacement in octogenarian patients. Material and Method: A retrospective review was performed on 23 octogenarian patients who underwent mechanical aortic valve replacement. Hospital mortality, postoperative intensive care unit stay, hospital stay and long-term results was examined. Estimates of the cumulative event mortality rate were calculated by the Kaplan-Meier method. Results: The mean age of all patients was 82.9±2.3 years and most were men (65.22%. The median ejection fraction was 45%. 73.91% of patients were in New York Heart Association class III-IV. Thirteen patients (56.52% in this study underwent combined procedure, the remaining 10 (43.48% patients underwent isolated aortic valve replacement. The most common valve size was 23 mm. The mean intensive care unit stay was 1.76±1.14 days. The mean hospital stay was 9.33±5.06 days. No complications were observed in 56.52% patients during their hospital stay. The overall hospital mortality was 8.7%. Follow-up was completed for all 23 patients. Median follow-up time was 33 months (1-108 months. Actuarial survival among discharged from hospital was 59% at 5 years. Discussion: Mechanical aortic valve replacement is a safe procedure in octogenarian patients and can be performed safely even in combined procedure.

Anemia in chronic renal failure. Ferrokinetic, erythrocytes-survival time and enzymes of erythrocytes on chronic dialysed patients

Energy Technology Data Exchange (ETDEWEB)

Junkers, K; Jontofson, R; Meuret, G; Blume, K G; Heinze, V [Freie Univ. Berlin (F.R. Germany). Nuklearmedizinische Abt.; Freiburg Univ. (F.R. Germany). Nuklearmedizinische Abt.; Freiburg Univ. (F.R. Germany). Medizinische Poliklinik)

1975-12-01

Radio-iron utilization was nearly normal in these patients, only bilateral nephrectomized patients showed a reduced radio-iron utilization. Red blood half-life span was shortened in all patients, well corresponding to the degree of anemia. Parameters of erythropoesis like plasma iron clearance, bone marrow transit time, erythron iron turnover, non-erythron iron turnover and hemolysis iron turnover failed to quantitate disorders of red blood cell regeneration in these patients. No defect in red blood cell enzyme activity could be demonstrated. Enzymes of glycolysis were increased corresponding to the reduced erythrocyte half-life span.

Trends in Transfusion Transmitted Infections Among Replacement Blood Donors in Karachi, Pakistan

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Syed Mohammad Irfan

2013-06-01

Full Text Available OBJECTIVE: To determine the prevalence of Hepatitis-B, Hepatitis-C and Human Immunodeficiency infections in replacement blood donors. METHODS: From January 2004 to December 2011, 108,598 apparently healthy donors donated blood at our Blood Bank. Screening was done by Microparticle Enzyme Immuno Assay (MEIA method on Axsym System (Abbott Diagnostic, USA and in year 2011 by Chemiluminescent Immunoassay (CIA method on Architect i2000 (Abbott Diagnostic, USA. From 2010 onward, HIV reactive donors were advised for confirmatory tests and reported back with the results. RESULTS: Of the 108,598 total donors, 108,393 (99.8% were replacement donors with a mean age of 28.92 (17-55 years. Of this, only 164 (0.15% were females. Among the replacement donors, 4,906 (4.5% were found to be reactive for Hepatitis-B, C and Human Immunodeficiency Virus. All the reactive patients, except one, were males. HbsAg was positive in 2,068 (1.90% and anti-HCV in 2832 (2.61% donors, while 111 (0.10% were positive for Human Immunodeficiency Virus. Co-infectivity was observed in 103 (0.09% cases. The prevalence appeared to be higher in younger age group (17-30 yrs. Only 16.6% cases should be patients returned with results of the confirmatory tests for HIV and were found positive. CONCLUSION: Hepatitis-B and C sero-prevalence in our series of replacement donors appears high compared to most studies from neighboring countries and relatively low in comparison to earlier studies from Pakistan. Prevalence of HIV, however, appears low and turn out of HIV positive cases for confirmatory tests is low.

A Theoretical Approach to Engineering a New Enzyme

International Nuclear Information System (INIS)

Anderson, Greg; Gomatam, Ravi; Behera, Raghu N.

2016-01-01

Density function theory, a subfield of quantum mechanics (QM), in combination with molecular mechanics (MM) has opened the way to engineer new artificial enzymes. Herein, we report theoretical calculations done using QM/MM to examine whether the regioselectivity and rate of chlorination of the enzyme chloroperoxidase can be improved by replacing the vanadium of this enzyme with niobium through dialysis. Our calculations show that a niobium substituted chloroperoxidase will be able to enter the initial steps of the catalytic cycle for chlorination. Although the protonation state of the niobium substituted enzyme is calculated to be different from than that of the natural vanadium substituted enzyme, our calculations show that the catalytic cycle can still proceed forward. Using natural bond orbitals, we analyse the electronic differences between the niobium substituted enzyme and the natural enzyme. We conclude by briefly examining how good of a model QM/MM provides for understanding the mechanism of catalysis of chloroperoxidase. (paper)

Comparison of survival analysis and palliative care involvement in patients aged over 70 years choosing conservative management or renal replacement therapy in advanced chronic kidney disease.

Science.gov (United States)

Hussain, Jamilla A; Mooney, Andrew; Russon, Lynne

2013-10-01

There are limited data on the outcomes of elderly patients with chronic kidney disease undergoing renal replacement therapy or conservative management. We aimed to compare survival, hospital admissions and palliative care access of patients aged over 70 years with chronic kidney disease stage 5 according to whether they chose renal replacement therapy or conservative management. Retrospective observational study. Patients aged over 70 years attending pre-dialysis clinic. In total, 172 patients chose conservative management and 269 chose renal replacement therapy. The renal replacement therapy group survived for longer when survival was taken from the time estimated glomerular filtration rate management, in patients older than 80 years or with a World Health Organization performance score of 3 or more. There was also a significant reduction in the effect of renal replacement therapy on survival in patients with high Charlson's Comorbidity Index scores. The relative risk of an acute hospital admission (renal replacement therapy vs conservative management) was 1.6 (p management patients died in hospital, compared to 69% undergoing renal replacement therapy (Renal Registry data). Seventy-six percent of the conservative management group accessed community palliative care services compared to 0% of renal replacement therapy patients. For patients aged over 80 years, with a poor performance status or high co-morbidity scores, the survival advantage of renal replacement therapy over conservative management was lost at all levels of disease severity. Those accessing a conservative management pathway had greater access to palliative care services and were less likely to be admitted to or die in hospital.

Reoperative Aortic Root Replacement in Patients with Previous Aortic Root or Aortic Valve Procedures

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Byung Kwon Chong

2016-08-01

Full Text Available Background: Generalization of standardized surgical techniques to treat aortic valve (AV and aortic root diseases has benefited large numbers of patients. As a consequence of the proliferation of patients receiving aortic root surgeries, surgeons are more frequently challenged by reoperative aortic root procedures. The aim of this study was to evaluate the outcomes of redo-aortic root replacement (ARR. Methods: We retrospectively reviewed 66 patients (36 male; mean age, 44.5±9.5 years who underwent redo-ARR following AV or aortic root procedures between April 1995 and June 2015. Results: Emergency surgeries comprised 43.9% (n=29. Indications for the redo-ARR were aneurysm (n=12, pseudoaneurysm (n=1, or dissection (n=6 of the residual native aortic sinus in 19 patients (28.8%, native AV dysfunction in 8 patients (12.1%, structural dysfunction of an implanted bioprosthetic AV in 19 patients (28.8%, and infection of previously replaced AV or proximal aortic grafts in 30 patients (45.5%. There were 3 early deaths (4.5%. During follow- up (median, 54.65 months; quartile 1–3, 17.93 to 95.71 months, there were 14 late deaths (21.2%, and 9 valve-related complications including reoperation of the aortic root in 1 patient, infective endocarditis in 3 patients, and hemorrhagic events in 5 patients. Overall survival and event-free survival rates at 5 years were 81.5%±5.1% and 76.4%±5.4%, respectively. Conclusion: Despite technical challenges and a high rate of emergency conditions in patients requiring redo-ARR, early and late outcomes were acceptable in these patients.

Mechanical versus bioprosthetic mitral valve replacement in patients <65 years old.

Science.gov (United States)

Kaneko, Tsuyoshi; Aranki, Sary; Javed, Quratulain; McGurk, Siobhan; Shekar, Prem; Davidson, Michael; Cohn, Lawrence

2014-01-01

Because of its durability, the mechanical valve is typically chosen for young patients undergoing mitral valve replacement (MVR). However, a bioprosthetic valve might have the benefit of valve-in-valve transcatheter valve replacement when valve failure occurs. We examined the outcomes in patients who had undergone mechanical valve MVR (MVRm) versus bioprosthetic valve MVR (MVRb) in patients aged Security Death Index. The postoperative and long-term outcomes of interest included combined stroke and embolic events, reoperations, and mortality. Of 768 consecutive patients, 627 were in the MVRm and 141 in the MVRb group. Propensity score matching yielded a cohort of 125 MVRb (89%) and 125 control MVRm patients with similar etiology mixes. The groups were similar in age (MVRm, 53.2 ± 9.0 years; MVRb, 53.8 ± 10.6 years; P = .617) and other preoperative characteristics. The postoperative outcomes were also similar between the 2 groups, including reoperation for bleeding, stroke, deep sternal infection, sepsis, and length of hospital stay. The operative mortality was also similar (MVRm, 5.6%; MVRb, 8.0%; P = .617). However, Kaplan-Meier analysis showed the MVRb group had a greater reoperation rate (P = .001) and shorter estimated survival (11.3 vs 13.5 years, P = .004). The incidence of bleeding and stroke or embolic events between the 2 groups was similar. In the present report, MVRb for patients safety of mechanical valves in this group. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Do intra-articular hyaluronic acid injections delay total knee replacement in patients with osteoarthritis – A Cox model analysis

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Amor, Bernard; Bardoulat, Isabelle; Tetafort, Aymeric; Pelletier-Fleury, Nathalie

2017-01-01

Due to the growing worldwide prevalence of knee osteoarthritis, the optimal management of this issue is critical for reducing its burden. Objectives This study aimed to describe patients treated for knee osteoarthritis between 2006 and 2013 in France and to compare the delay from diagnosis to total knee replacement between patients who received intra-articular hyaluronic acid injections and those who did not receive the injections. A second objective was to compare direct medical costs for ambulatory care between treatment groups. Materials and methods Patients were selected from a representative sample of the real world administrative claims database using an algorithm developed by experts from the scientific committee of the study. Data were matched with the medico-administrative database for hospital care. A Cox proportional hazards model was stratified for the treatment group and adjusted for available socio-demographic and medical covariates to compare restricted mean survival times at different time points (1, 3, 5 and 7.5 years) between groups. Costs were expressed in 2013 euros. Results A total of 14,782 patients were treated for knee osteoarthritis (67% women; mean age = 68 years). Among this population, 1,662 patients had total knee replacement (11.2%). At each time point, restricted mean survival time without total knee replacement was significantly higher (p-valueshyaluronic acid group, from +51 to +217 days at 1 and 7.5 years, respectively. For the year preceding total knee replacement, the means for total direct medical costs were similar between groups, €744 vs €805 for treatment and control groups, respectively, (p-value = 0.104). Intra-articular injections accounted for less than 10% of the total costs. Conclusion This is the first retrospective longitudinal study involving knee osteoarthritis patients using medico-administrative databases in France. The results support the effectiveness of hyaluronic acid injections in delaying total knee

Verification of pharmacogenetics-based warfarin dosing algorithms in Han-Chinese patients undertaking mechanic heart valve replacement.

Science.gov (United States)

Zhao, Li; Chen, Chunxia; Li, Bei; Dong, Li; Guo, Yingqiang; Xiao, Xijun; Zhang, Eryong; Qin, Li

2014-01-01

To study the performance of pharmacogenetics-based warfarin dosing algorithms in the initial and the stable warfarin treatment phases in a cohort of Han-Chinese patients undertaking mechanic heart valve replacement. We searched PubMed, Chinese National Knowledge Infrastructure and Wanfang databases for selecting pharmacogenetics-based warfarin dosing models. Patients with mechanic heart valve replacement were consecutively recruited between March 2012 and July 2012. The predicted warfarin dose of each patient was calculated and compared with the observed initial and stable warfarin doses. The percentage of patients whose predicted dose fell within 20% of their actual therapeutic dose (percentage within 20%), and the mean absolute error (MAE) were utilized to evaluate the predictive accuracy of all the selected algorithms. A total of 8 algorithms including Du, Huang, Miao, Wei, Zhang, Lou, Gage, and International Warfarin Pharmacogenetics Consortium (IWPC) model, were tested in 181 patients. The MAE of the Gage, IWPC and 6 Han-Chinese pharmacogenetics-based warfarin dosing algorithms was less than 0.6 mg/day in accuracy and the percentage within 20% exceeded 45% in all of the selected models in both the initial and the stable treatment stages. When patients were stratified according to the warfarin dose range, all of the equations demonstrated better performance in the ideal-dose range (1.88-4.38 mg/day) than the low-dose range (pharmacogenetics-based warfarin dosing regimens performed similarly in our cohort. However, the algorithms of Wei, Huang, and Miao showed a better potential for warfarin prediction in the initial and the stable treatment phases in Han-Chinese patients undertaking mechanic heart valve replacement.

A longitudinal study of quality of life and functional status in total hip and total knee replacement patients.

Science.gov (United States)

Mandzuk, Lynda L; McMillan, Diana E; Bohm, Eric R

2015-05-01

Primary total hip and primary total knee surgeries are commonly performed to improve patients' quality of life and functional status. This longitudinal retrospective study (N = 851) examined self-reported quality of life and functional status over the preoperative and postoperative periods: 12 months prior to surgery, one month prior to surgery and 12 months following surgery. A linear mixed effects model was used to analyze the changes in quality of life and functional status over the sampling period. Patients in the convenience sample reported improvements in quality of life and functional status utilizing the SF-12 and Oxford Hip and Oxford Knee, although differences were noted by procedure and gender. Total hip patients tended to demonstrate greater improvement than total knee patients and males reported higher levels of physical and mental quality of life as well as functional status when compared to females. Of particular note was that mental health scores were consistently lower in both total hip and total knee replacement patients across the perioperative period and up to one year postoperative. This study identifies an opportunity for health care providers to proactively address the mental health of total hip and total knee replacement patients throughout their joint replacement trajectory. Copyright © 2014 Elsevier Ltd. All rights reserved.

The effect of growth hormone replacement in patients with hypopituitarism on pituitary tumor recurrence, secondary cancer, and stroke.

Science.gov (United States)

Jasim, Sina; Alahdab, Fares; Ahmed, Ahmed T; Tamhane, Shrikant U; Sharma, Anu; Donegan, Diane; Nippoldt, Todd B; Murad, M Hassan

2017-05-01

Growth hormone replacement therapy has benefits for patients with hypopituitarism. The safety profile in regard to tumor recurrence or progression, development of secondary malignancies, or cerebrovascular stroke is still an area of debate. A comprehensive search of multiple databases-MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, and Scopus was conducted through August 2015. Eligible studies that evaluated long-term adverse events in adult patients with hypopituitarism treated with growth hormone replacement therapy and reported development of pituitary tumor recurrence or progression, secondary malignancies, or cerebrovascular stroke were selected following a predefined protocol. Reviewers, independently and in duplicate, extracted data and assessed the risk of bias. Random-effects meta-analysis was used to pool relative risks and 95 % confidence intervals. We included 15 studies (published 1995-2015) that reported on 46,148 patients. Compared to non-replacement, growth hormone replacement therapy in adults with hypopituitarism was not associated with statistically significant change in pituitary tumor progression or recurrence (relative risk, 0.77; 95 % confidence interval, 0.53-1.13) or development of secondary malignancy (relative risk, 0.99; 95 % confidence interval, 0.70-1.39). In two retrospective studies, there was higher risk of stroke in patients who did not receive replacement (relative risk, 2.07; 95 % confidence interval, 1.51-2.83). The quality of evidence is low due to study limitations and imprecision. This systematic review and meta-analysis supports the overall safety of growth hormone therapeutic use in adults with hypopituitarism with no clear evidence of increased risk of pituitary tumor recurrence, malignancy, or stroke.

A Water Rehabilitation Program in Patients with Hip Osteoarthritis Before and After Total Hip Replacement

OpenAIRE

?yp, Marek; Kaczor, Ryszard; Cabak, Anna; Tederko, Piotr; W?ostowska, Ewa; Stanis?awska, Iwona; Szypu?a, Jan; Tomaszewski, Wies?aw

2016-01-01

Background Pain associated with coxarthrosis, typically occurring in middle-aged and elderly patients, very commonly causes considerable limitation of motor fitness and dependence on pharmacotherapy. This article provides an assessment of a rehabilitation program with tailored water exercises in patients with osteoarthritis before and after total hip replacement. Material/Methods A total of 192 patients (the mean age 61.03?10.89) suffering from hip osteoarthritis (OA) were evaluated before an...

Valve Sparing Aortic Root Replacement in Patients with Marfan Syndrome Enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions

Science.gov (United States)

Song, Howard K.; Preiss, Liliana R.; Maslen, Cheryl L.; Kroner, Barbara; Devereux, Richard B.; Roman, Mary J.; Holmes, Kathryn W.; Tolunay, H. Eser; Desvigne-Nickens, Patrice; Asch, Federico M.; Milewski, Rita K.; Bavaria, Joseph; LeMaire, Scott A.

2016-01-01

Background The long-term outcomes of aortic valve sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. We sought to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). Methods At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 have undergone aortic root replacement. Patients who have undergone AVS procedures were compared to those who have undergone aortic valve replacing (AVR) procedures. Results AVS root replacement was performed in 43.5% of MFS patients and the frequency of AVS increased over the past 5 years. AVS patients were younger at the time of surgery (31.0 vs. 36.3 years, p=0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients. AVR patients were more likely to have had aortic valve dysfunction and aortic dissection as a primary indication for surgery. After mean follow-up of 6.2 (SD=3.6) years, none of the 87 AVS patients have required reoperation; in contrast, after mean follow up of 10.5 (SD=7.6) years, 11.5% of AVR patients have required aortic root reoperation. Aortic valve function has been durable with 95.8% of AVS patients with aortic insufficiency graded as mild or less. Conclusions AVS root replacement is performed commonly in the MFS population. The durability of the aortic repair and aortic valve function have been excellent to date. These results justify the continued use of the procedure in the elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future. PMID:25296451

Double Valve Replacement (Mitral and Aortic for Rheumatic Heart Disease: A 20-year experience with 300 patients.

Directory of Open Access Journals (Sweden)

Prashant Mishra

2016-09-01

Full Text Available Introduction: Rheumatic heart disease still remains one of the leading causes of congestive heart failure and death owing to valvular pathologies, in developing countries. Valve replacement still remains the treatment of choice in such patients.The aim of this study wasto analyze the postoperative outcome of  double valve replacement (Mitral and Aortic in patients of rheumatic heart disease. Materials and Methods: Between 1988 and 2008, 300 patients of rheumatic heart disease underwent double (Mitral and Aortic valve replacement with Starr Edwards valve or St Jude mechanical valve prosthesis were implanted. These patients were studied retrospectively for preoperative data and postoperative outcome including causes of early and late deaths and the data was analyzed statistically. Results: The 30-day hospital death rate was 11.3% andlate death occurred in 11.6%. Anticoagulant regimen was followed to maintain the target pro-thrombin time at 1.5 times the control value. The actuarial survival (exclusive of hospital mortality was 92.4%, 84.6%, and 84.4%, per year at 5, 10, and 20 years, respectively Conclusions: In view of the acknowledged advantageof superior durability, increased thromboresistance in our patient population, and its cost effectiveness the Starr-Edwards ball valve or St. Jude valve is the mechanical prosthesis of choice for advanced combined valvular disease. The low-intensity anticoagulant regimen has offered suffcient protection against thromboembolism as well as hemorrhage.

Outcomes of Reoperative Valve Replacement in Patients with Prosthetic Valve Endocarditis: A 20-Year Experience

Directory of Open Access Journals (Sweden)

Young Woong Kim

2018-02-01

Full Text Available Background: Prosthetic valve endocarditis (PVE is a serious complication of cardiac valve replacement, and many p atients with P VE r equire r eoperation. The aim of t his study was to r eview our institutional 2 0 -year experience of surgical reoperative valve replacement in patients with PVE. Methods: A retrospective study was performed on 84 patients (mean age, 54.8±12.7 years; 51 males who were diagnosed with PVE and underwent reoperative valve replacement from January 1995 to December 2016. Results: PVE was found in 1 valve in 61 cases (72.6%, and in 2 or more valves in 23 cases (27.4%. The median follow-up duration was 47.3 months (range, 0 to 250 months. Postoperative complications occurred in 39 patients (46.4%. Reinfection occurred in 6 cases, all within 1 year. The freedom from reinfection rate at 5 years was 91.0%±3.5%. The overall survival rates at 5 and 10 years were 64.4%±5.8% and 54.3%±7.3%, respectively. In stepwise multivariable Cox proportional hazard models, older age (hazard ratio [HR], 1.48; 95% confidence interval [CI], 1.05 to 2.10; p=0.027 and cardiopulmonary bypass (CPB time (HR, 1.03; 95% CI, 1.00 to 1.0 1; p =0 . 0 33 e merged a s independent risk f actors f or d eath. Conclusion: Older age and a longer CPB time were associated with an increased risk of overall mortality in PVE patients.

Assimilation of wheat starch in patients with chronic pancreatitis. Positive effect of enzyme replacement

DEFF Research Database (Denmark)

Nordgaard, I; Rumessen, J J; Gudmand-Høyer, E

1992-01-01

Pancreatic insufficiency due to chronic pancreatitis may lead to symptomatic malabsorption of both starch and fat. The absorption capacity of wheat starch has not been studied previously in patients with chronic pancreatitis, although this carbohydrate is a quantitatively important component of t...

Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions.

Science.gov (United States)

Song, Howard K; Preiss, Liliana R; Maslen, Cheryl L; Kroner, Barbara; Devereux, Richard B; Roman, Mary J; Holmes, Kathryn W; Tolunay, H Eser; Desvigne-Nickens, Patrice; Asch, Federico M; Milewski, Rita K; Bavaria, Joseph; LeMaire, Scott A

2014-05-01

The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR). AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2 +/- 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 +/- 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less. AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.

Higher glucocorticoid replacement doses are associated with increased mortality in patients with pituitary adenoma.

Science.gov (United States)

Hammarstrand, Casper; Ragnarsson, Oskar; Hallén, Tobias; Andersson, Eva; Skoglund, Thomas; Nilsson, Anna G; Johannsson, Gudmundur; Olsson, Daniel S

2017-09-01

Patients with secondary adrenal insufficiency (AI) have an excess mortality. The objective was to investigate the impact of the daily glucocorticoid replacement dose on mortality in patients with hypopituitarism due to non-functioning pituitary adenoma (NFPA). Patients with NFPA were followed between years 1997 and 2014 and cross-referenced with the National Swedish Death Register. Standardized mortality ratio (SMR) was calculated with the general population as reference and Cox-regression was used to analyse the mortality. The analysis included 392 patients (140 women) with NFPA. Mean ± s.d. age at diagnosis was 58.7 ± 14.6 years and mean follow-up was 12.7 ± 7.2 years. AI was present in 193 patients, receiving a mean daily hydrocortisone equivalent (HCeq) dose of 20 ± 6 mg. SMR (95% confidence interval (CI)) for patients with AI was similar to that for patients without, 0.88 (0.68-1.12) and 0.87 (0.63-1.18) respectively. SMR was higher for patients with a daily HCeq dose of >20 mg (1.42 (0.88-2.17)) than that in patients with a daily HCeq dose of 20 mg (0.71 (0.49-0.99)), P  = 0.017. In a Cox-regression analysis, a daily HCeq dose of >20 mg was independently associated with a higher mortality (HR: 1.88 (1.06-3.33)). Patients with daily HCeq doses of ≤20 mg had a mortality risk comparable to patients without glucocorticoid replacement and to the general population. Patients with NFPA and AI receiving more than 20 mg HCeq per day have an increased mortality. Our data also show that mortality in patients substituted with 20 mg HCeq per day or less is not increased. © 2017 European Society of Endocrinology.

Intrathecal enzyme replacement therapy reduces lysosomal storage in the brain and meninges of the canine model of MPS I.

Science.gov (United States)

Kakkis, E; McEntee, M; Vogler, C; Le, S; Levy, B; Belichenko, P; Mobley, W; Dickson, P; Hanson, S; Passage, M

2004-01-01

Enzyme replacement therapy (ERT) has been developed for several lysosomal storage disorders, including mucopolysaccharidosis I (MPS I), and is effective at reducing lysosomal storage in many tissues and in ameliorating clinical disease. However, intravenous ERT does not adequately treat storage disease in the central nervous system (CNS), presumably due to effects of the blood-brain barrier on enzyme distribution. To circumvent this barrier, we studied whether intrathecal (IT) recombinant human alpha-L-iduronidase (rhIDU) could penetrate and treat the brain and meninges. An initial dose-response study showed that doses of 0.46-4.14 mg of IT rhIDU successfully penetrated the brain of normal dogs and reached tissue levels 5.6 to 18.9-fold normal overall and 2.7 to 5.9-fold normal in deep brain sections lacking CSF contact. To assess the efficacy and safety in treating lysosomal storage disease, four weekly doses of approximately 1 mg of IT rhIDU were administered to MPS I-affected dogs resulting in a mean 23- and 300-fold normal levels of iduronidase in total brain and meninges, respectively. Quantitative glycosaminoglycan (GAG) analysis showed that the IT treatment reduced mean total brain GAG to normal levels and achieved a 57% reduction in meningeal GAG levels accompanied by histologic improvement in lysosomal storage in all cell types. The dogs did develop a dose-dependent immune response against the recombinant human protein and a meningeal lymphocytic/plasmacytic infiltrate. The IT route of ERT administration may be an effective way to treat the CNS disease in MPS I and could be applicable to other lysosomal storage disorders.

The caudal septum replacement graft.

Science.gov (United States)

Foda, Hossam M T

2008-01-01

To describe a technique for reconstructing the lost tip support in cases involving caudal septal and premaxillary deficiencies. The study included 120 patients with aesthetic and functional nasal problems resulting from the loss of caudal septal and premaxillary support. An external rhinoplasty approach was performed to reconstruct the lost support using a cartilaginous caudal septum replacement graft and premaxillary augmentation with Mersilene mesh. The majority of cases (75%) involved revisions in patients who had previously undergone 1 or more nasal surgical procedures. A caudal septum replacement graft was combined with premaxillary augmentation in 93 patients (77.5%). The mean follow-up period was 3 years (range, 1-12 years). The technique succeeded in correcting the external nasal deformities in all patients and resulted in a significant improvement in breathing in 74 patients (86%) with preoperative nasal obstruction. There were no cases of infection, displacement, or extrusion. The caudal septum replacement graft proved to be very effective in restoring the lost tip support in patients with caudal septal deficiency. Combining the graft with premaxillary augmentation using Mersilene mesh helped increase support and stability over long-term follow-up.

Study on changes of serum lipid profile after withdrawl of levo-thyroxine replacement therapy in patients with radical thyroidectomy for cancer

International Nuclear Information System (INIS)

Yang Shumin; Yu Lili; Dong Lin; Zhu Gaohong; Zhang Jijian

2010-01-01

Objective: To study the changes of serum lipid profile after withdrawl of levo-thyroxine replacement for 4 weeks in patient with radical thyroidectomy for cancer. Methods: Serum thyroid hormones (FT 3 , FT 4 , sTSH, with RIA) levels and lipid profile (Total cholesterol, triglycerides, LDL, HDL, apolipoprotein A-I, apolipoprotein B and Lp (a), with biochemistry) were determined in 46 patients with thyroid carcinoma after operation both during and after 4 weeks' withdrawl of levo-thyroxine replacement treatment. Results: Serum FT 3 , FT 4 levels decreased significantly and serum sTSH, TC, TG, LDL, apo-B, Lp (a) levels increased significantly after 4 weeks' withdrawl of levo-thyroxine replacement (vs during treatment, P 0.05). Conclusion: Hyperlipidaemia did develop in the hypothyroid patients after withdrawl of levo-thyroxine replacement but the degree of which did not not necessarily correlate with the increment of sTSH levels. (authors)

Midterm Results of Aortic Valve Replacement with Cryopreserved Homografts

Directory of Open Access Journals (Sweden)

Emre Özker

2012-06-01

Full Text Available Objective: The aim of this study was to analyze the midterm clinical results of aortic valve replacement with cryopreserved homografts.Materials and Methods: Aortic valve replacement was performed in 40 patients with cryopreserved homograft. The indications were aortic valve endocarditis in 20 patients (50%, truncus arteriosus in 6 patients (15%, and re-stenosis or regurtitation after aortic valve reconstruction in 14 (35% patients. The valve sizes ranged from 10 to 27mm. A full root replacement technique was used for homograft replacement in all patients.Results: The 30-day postoperative mortality rate was 12.5% (5 patients. There were four late deaths. Only one of them was related to cardiac events. Overall mortality was 22.5%. Thirty-three patients were followed up for 67±26 months. Two patients needed reoperation due to aortic aneurysm caused by endocarditis. The mean transvalvular gradient significantly decreased after valve replacement (p<0.003. The last follow up showed that the 27 (82% patients had a normal left ventricular function.Conclusion: Cryopreserved homografts are safe alternatives to mechanical valves that can be used when there are proper indications. Although it has a high perioperative mortality rate, cryopreserved homograft implantation is an alternative for valve replacement, particularly in younger patients and for complex surgical problems such as endocarditis that must be minimalized.

Reoperative aortic root replacement: Outcome in a contemporary series.

Science.gov (United States)

Esaki, Jiro; Leshnower, Bradley G; Binongo, Jose N; Lasanajak, Yi; McPherson, LaRonica; Thourani, Vinod H; Chen, Edward P

2017-09-01

Reoperative aortic root replacement is a challenging procedure associated with significant mortality and morbidity. The purpose of this study was to investigate the outcomes of reoperative aortic root replacement when performed in a number of complex clinical settings and to identify risk factors for operative mortality and long-term survival. From 2006 to 2015, 280 consecutive patients at an academic center underwent reoperative aortic root replacement after a variety of previous aortic or cardiac operations. Logistic regression and extended Cox proportional hazards regression analyses were used to determine risk factors for operative mortality and long-term survival, respectively. The mean age of patients was 52.5 ± 14.1 years. Prior operations included proximal aortic replacement in 113 patients, valve surgery in 162 patients, and coronary artery bypass grafting in 46 patients. Concomitant procedures included arch replacement in 135 patients, coronary artery bypass grafting in 68 patients, and mitral valve repair/replacement in 18 patients. Operative mortality was 14.3%. Five-year survival was 74.0%. Univariable analysis did not find previous root replacement, prior proximal aortic surgery, and concomitant arch replacement to be risk factors for operative mortality. In the multivariable analysis, chronic lung disease, prior myocardial infarction, and concomitant mitral valve surgery were risk factors for operative mortality. Age, peripheral artery disease, emergency, and concomitant mitral valve surgery were risk factors for mortality in the late phase. Reoperative aortic root replacement represents complex procedures carrying significant morbidity and mortality. Chronic lung disease, prior myocardial infarction, and concomitant mitral valve surgery were risk factors for operative mortality. Age, peripheral artery disease, emergency, and concomitant mitral valve surgery were risk factors for long-term mortality. Copyright © 2017 The American Association for

Verification of Pharmacogenetics-Based Warfarin Dosing Algorithms in Han-Chinese Patients Undertaking Mechanic Heart Valve Replacement

Science.gov (United States)

Zhao, Li; Chen, Chunxia; Li, Bei; Dong, Li; Guo, Yingqiang; Xiao, Xijun; Zhang, Eryong; Qin, Li

2014-01-01

Objective To study the performance of pharmacogenetics-based warfarin dosing algorithms in the initial and the stable warfarin treatment phases in a cohort of Han-Chinese patients undertaking mechanic heart valve replacement. Methods We searched PubMed, Chinese National Knowledge Infrastructure and Wanfang databases for selecting pharmacogenetics-based warfarin dosing models. Patients with mechanic heart valve replacement were consecutively recruited between March 2012 and July 2012. The predicted warfarin dose of each patient was calculated and compared with the observed initial and stable warfarin doses. The percentage of patients whose predicted dose fell within 20% of their actual therapeutic dose (percentage within 20%), and the mean absolute error (MAE) were utilized to evaluate the predictive accuracy of all the selected algorithms. Results A total of 8 algorithms including Du, Huang, Miao, Wei, Zhang, Lou, Gage, and International Warfarin Pharmacogenetics Consortium (IWPC) model, were tested in 181 patients. The MAE of the Gage, IWPC and 6 Han-Chinese pharmacogenetics-based warfarin dosing algorithms was less than 0.6 mg/day in accuracy and the percentage within 20% exceeded 45% in all of the selected models in both the initial and the stable treatment stages. When patients were stratified according to the warfarin dose range, all of the equations demonstrated better performance in the ideal-dose range (1.88–4.38 mg/day) than the low-dose range (warfarin dose prediction and in the low-dose and the ideal-dose ranges. Conclusions All of the selected pharmacogenetics-based warfarin dosing regimens performed similarly in our cohort. However, the algorithms of Wei, Huang, and Miao showed a better potential for warfarin prediction in the initial and the stable treatment phases in Han-Chinese patients undertaking mechanic heart valve replacement. PMID:24728385

Clinical study on postoperative steroid hormon replacement for preclinical Cushing's syndrome

International Nuclear Information System (INIS)

Furuta, Nozomu; Koide, Haruhisa; Sasaki, Hiroshi; Miki, Jun; Kimura, Takahiro; Egawa, Shin

2009-01-01

Diagnostic criteria for preclinical Cushing's syndrome (PCS) were reported in 1996. However, requirement of postoperative steroid hormone replacement is still controversial issue. In this study, we observed recent surgical cases retrospectively and evaluate the use of postoperative steroid hormone replacement. Eighteen patients with PCS underwent surgery from 1997 to 2007 in Jikei University Hospital. Thirteen of them received postoperative steroid hormone replacement. We investigated preoperative hormone activity by 131 I-adosterol scintigraphy and suppression of adrenocorticotropic hormone (ACTH) and evaluated the requirement of postoperative steroid hormone replacement. Preoperative serum cortisol was normal range in all patients. Serum ACTH was suppressed in 10 of them (56%). In 131 I-adosterol scintigraphy, accumulation in ipsilateral side was observed in all patients. Accumulation in contralateral side was observed in 13 patients whose serum ACTH had tendency to be suppressed. Mean period of steroid hormone replacement was 19.8 weeks. Patients with lower preoperative ACTH tended to require longer period until withdrawal of steroid hormone replacement. In addition, patients received steroid hormone replacement with higher starting dose significantly required longer period. Three of them had complications during tapering of steroid hormone. Postoperative adrenal insufficiency is important issue as postoperative management of PCS patients whose function of contralateral adrenal or pituitary gland is suppressed. 131 I-adosterol scintigraphy and preoperative serum ACTH were important factors to evaluate the requirement of postoperative steroid hormone replacement. Especially, patients with low preoperative serum ACTH tended to require long duration of postoperative steroid hormone replacement. On the other hand, patients with accumulation of contralateral side in 131 I-adosterol scintigraphy and without suppression of serum ACTH may not require steroid hormone

Patient-controlled oral analgesia for postoperative pain management following total knee replacement.

Science.gov (United States)

Kastanias, Patti; Gowans, Sue; Tumber, Paul S; Snaith, Kianda; Robinson, Sandra

2010-01-01

To investigate whether patient-controlled oral analgesia (PCOA) used by individuals receiving a total knee replacement could reduce pain, increase patient satisfaction, reduce opioid use and/or reduce opioid side effects when compared with traditional nurse (RN)-administered oral analgesia. Patients who underwent an elective total knee replacement at a quaternary care centre (Toronto Western Hospital, Toronto, Ontario) were randomly assigned to either PCOA or RN-administered short-acting oral opioids on postoperative day 2. Subjects in the RN group called the RN to receive their prescribed short-acting opioid. Subjects in the PCOA group kept a single dose of their prescribed oral opioid at their bedside and took this dose when they felt they needed it, to a maximum of one dose every 2 h. Study outcomes, collected on postoperative day 2, included pain (measured by the Brief Pain Inventory - Short Form), patient satisfaction (measured by the Pain Outcome Questionnaire Satisfaction subscale - component II), opioid use (oral morphine equivalents), opioid side effects (nausea, pruritus and/or constipation) and knee measures (maximum passive knee flexion and pain at maximum passive knee flexion, performed on the operative knee). Study outcomes were analyzed twice. First, for a subset of 73 subjects who remained in their randomly assigned group (PCOA group, n=36; RN group, n=37), randomized analyses were performed. Second, for the larger sample of 88 subjects who were categorized by their actual method of receiving oral opioids (PCOA group, n=41; RN group, n=47), as-treated analyses were performed. There were no differences in study outcomes between the PCOA and RN groups in either analysis. PCOA was not superior to RN administration on study outcomes. However, PCOA did not increase opioid use or pain. PCOA remains an important element in the patient-centred care facility.

Impact of prosthesis-patient mismatch on early and late mortality after aortic valve replacement

NARCIS (Netherlands)

Koene, Bart M.; Hamad, Mohamed A. Soliman; Bouma, Wobbe; Mariani, Massimo A.; Peels, Kathinka C.; van Dantzig, Jan-Melle; van Straten, Albert H.

2013-01-01

Background: The influence of prosthesis-patient mismatch (PPM) on survival after aortic valve replacement (AVR) remains controversial. In this study, we sought to determine the effect of PPM on early (30 days) after AVR or AVR combined with coronary artery bypass grafting (AVR with CABG). Methods:

Resolution of non-alcoholic steatohepatitis after growth hormone replacement in a pediatric liver transplant patient with panhypopituitarism.

Science.gov (United States)

Gilliland, Thomas; Dufour, Sylvie; Shulman, Gerald I; Petersen, Kitt Falk; Emre, Sukru H

2016-12-01

NAFLD is a common condition linked to obesity, type 2 diabetes, and metabolic syndrome. Simple hepatic steatosis is a risk factor for inflammatory reactions in the liver (NASH), which may lead to cirrhosis. While the mechanism is unclear, NAFLD and NASH are associated with panhypopituitarism, which in the pediatric population often results from craniopharyngioma or pituitary adenoma and the sequelae of treatment, causing hypothyroidism, adrenal insufficiency, hypogonadotropic hypogonadism, and GH deficiency. Refractory NAFLD in panhypopituitarism may be amenable to GH replacement. Here, we report a pediatric case of NASH secondary to panhypopituitarism from craniopharyngioma, which recurred by 11 months after LDLT. Despite low-dose GH replacement, the patient remained GH deficient. Pubertal dosed GH therapy led to rapid and complete resolution of hepatic steatosis, which we tracked using serial 1 H MRS. Pediatric patients with NASH cirrhosis secondary to panhypopituitarism can be good candidates for liver transplantation, but hormone deficiencies predispose to recurrence after transplant. High-dose GH replacement should be considered in pediatric patients with GH deficiency and recurrent disease. A multidisciplinary team approach is essential for successful outcomes. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Patient perspectives on the optimal start of renal replacement therapy.

Science.gov (United States)

Henry, Shayna L; Munoz-Plaza, Corrine; Garcia Delgadillo, Jazmine; Mihara, Nichole K; Rutkowski, Mark P

2017-09-01

Healthcare systems and providers are encouraged to prepare their patients with advanced chronic kidney disease (CKD) for a planned start to renal replacement therapies (RRT). Less well understood are the socioemotional experiences surrounding the optimal start of RRT versus suboptimal haemodialysis (HD) starts with a central catheter. To characterise the experiences of patients beginning RRT. Qualitative, semi-structured phone interviews. A total of 168 patients with stage 5 CKD initiating RRT in an integrated, capitated learning healthcare system. Qualitative data from patients were collected as part of a quality improvement initiative to better understand patient-reported themes concerning preparation for RRT, patients' perceptions of their transition to dialysis and why sub-optimal starts for RRT occur within our healthcare system. Dual review and verification was used to identify key phrases and themes within and across each domain, using both deductive a priori codes generated by the interview guide and grounded discovery of emergent themes. From the patient perspective, preparing for RRT is an experience rooted in deep feelings of fear. In addition, a number of key factors contributed to patients' preparation (or failure to prepare) for RRT. While the education provided by our system was viewed as adequate overall, patients often felt that their emotional and psychosocial needs went unmet, regardless of whether or not, they experienced an optimal dialysis start. Future efforts should incorporate additional strategies for helping patients with advanced CKD achieve emotional and psychological safety while preparing for RRT. © 2017 European Dialysis and Transplant Nurses Association/European Renal Care Association.

Binding affinity and adhesion force of organophosphate hydrolase enzyme with soil particles related to the isoelectric point of the enzyme.

Science.gov (United States)

Islam, Shah Md Asraful; Yeasmin, Shabina; Islam, Md Saiful; Islam, Md Shariful

2017-07-01

The binding affinity of organophosphate hydrolase enzyme (OphB) with soil particles in relation to the isoelectric point (pI) was studied. Immobilization of OphB with soil particles was observed by confocal microscopy, Fourier transform infrared spectroscopy (FT-IR), and Atomic force microscopy (AFM). The calculated pI of OphB enzyme was increased from 8.69 to 8.89, 9.04 and 9.16 by the single, double and triple mutant of OphB enzyme, respectively through the replacement of negatively charged aspartate with positively charged histidine. Practically, the binding affinity was increased to 5.30%, 11.50%, and 16.80% for single, double and triple mutants, respectively. In contrast, enzyme activity of OphB did not change by the mutation of the enzyme. On the other hand, adhesion forces were gradually increased for wild type OphB enzyme (90 pN) to 96, 100 and 104 pN for single, double and triple mutants of OphB enzyme, respectively. There was an increasing trend of binding affinity and adhesion force by the increase of isoelectric point (pI) of OphB enzyme. Copyright © 2017 Elsevier Inc. All rights reserved.

Human Mitochondrial HMG-CoA Synthase Deficiency: Role of Enzyme Dimerization Surface and Characterization of Three New Patients

Directory of Open Access Journals (Sweden)

Beatriz Puisac

2018-03-01

Full Text Available Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency (mitochondrial HMG-CoA synthase deficiency or mHS deficiency, OMIM #605911 is an inborn error of metabolism that affects ketone body synthesis. Acute episodes include vomiting, lethargy, hepatomegaly, hypoglycemia and dicarboxylic aciduria. The diagnosis is difficult due to the relatively unspecific clinical and biochemical presentation, and fewer than 30 patients have been described. This work describes three new patients with mHS deficiency and two missense mutations c.334C>T (p.R112W and c.430G>T (p.V144L previously not reported. We developed a new method to express and measure the activity of the enzyme and in this work the study is extended to ten new missense variants including those of our patients. Enzymatic assays showed that three of the mutant proteins retained some but seven completely lacked activity. The identification of a patient homozygous for a mutation that retains 70% of enzyme activity opens the door to a new interpretation of the disease by demonstrating that a modest impairment of enzyme function can actually produce symptoms. This is also the first study employing molecular dynamics modelling of the enzyme mutations. We show that the correct maintenance of the dimerization surface is crucial for retaining the structure of the active center and therefore the activity of the enzyme.

Survival and freedom from aortic valve-related reoperation after valve-sparing aortic root replacement in 1015 patients.

Science.gov (United States)

Kari, Fabian A; Doll, Kai-Nicolas; Hemmer, Wolfgang; Liebrich, Markus; Sievers, Hans-Hinrich; Richardt, Doreen; Reichenspurner, Hermann; Detter, Christian; Siepe, Matthias; Czerny, Martin; Beyersdorf, Friedhelm

2016-04-01

The aim of this study was to characterize mortality and aortic valve replacement after valve-sparing aortic root replacement (V-SARR) in a multicentre cohort. Between 1994 and 2014, 1015 patients had V-SARR with (n = 288, 28%) or without cusp/commissure repair (n = 727, 72%) at the centres of Lübeck (n = 343, 34%), Stuttgart (n = 346, 34%), Hamburg (n = 109, 11%) and Freiburg (n = 217, 21%), Germany. Comparative survival of an age- and gender-matched general population was calculated. Log-rank tests and multiple logistic regression were used to identify risk factors. The mean follow-up was 5.2 ± 3.9 years. Cumulative follow-up comprised 2933 patient-years. Early survival was 98%. NYHA status and aneurysm size were predictive of death during mid-term follow-up (P = 0.025). Freedom from aortic valve replacement was 90% at 8 years, with the type of V-SARR (root remodelling, David II) being a risk factor (P = 0.015). Bicuspid aortic valve (P = 0.26) and initial valve function (P = 0.4) did not impact reoperation. The need of additional valve repair (cusps/commissures) was not linked to reoperation: freedom from aortic valve replacement at 8 years was 84% if cusp repair was performed versus 90% if V-SARR alone was performed (P = 0.218). Marfan syndrome had no impact on survival or on aortic valve replacement. Mid-term survival of patients after V-SARR is comparable with that of a matched general population. The regurgitant bicuspid aortic valve is a favourable substrate for V-SARR. Prophylactic surgery should be performed before symptoms or large aneurysms are present to achieve optimal mid-term outcomes. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Predictors of length of stay and patient satisfaction after hip and knee replacement surgery: fast-track experience in 712 patients

DEFF Research Database (Denmark)

Husted, Henrik; Holm, Gitte; Jacobsen, Steffen

2008-01-01

BACKGROUND AND PURPOSE: Very few studies have focused on patient characteristics that influence length of stay (LOS) in fast-track total hip (THR) and knee arthroplasty (TKR). The aim of this prospective study was to identify patient characteristics associated with LOS and patient satisfaction...... aids, pre- and postoperative hemoglobin levels, the need for blood transfusion, ASA score, and time between surgery and mobilization, were all found to influence postoperative outcome in general, and LOS and patient satisfaction in particular. INTERPRETATION: We identified several patient...... characteristics that influence postoperative outcome, LOS, and patient satisfaction in our series of consecutive fast-track joint replacement patients, enabling further attention to be paid to certain aspects of surgery and rehabilitation....

Awareness, attitudes, need and demand on replacement of missing teeth among a group of partially dentate patients attending a University Dental Hospital.

Science.gov (United States)

Jayasinghe, Rasika Manori; Perera, Janana; Jayasinghe, Vajira; Thilakumara, Indika P; Rasnayaka, Sumudu; Shiraz, Muhammad Hanafi Muhammad; Ranabahu, Indra; Kularatna, Sanjeewa

2017-07-27

Our objective was to assess awareness, attitudes, need and demand on replacement of missing teeth according to edentulous space, age, gender, ethnicity, educational level and socio-economical status of the patient. 76.2% of the study group was opined that the missing teeth should be replaced by prosthetic means. Majority were keen in getting them replaced mainly for the comfort in mastication. Although 77.9 and 32.9% were aware of the removable prostheses and implants respectively, only 25.2% knew about tooth supported bridges as an option of replacement of missing teeth. Participants' awareness on tooth and implant supported prostheses is at a higher level. Participants' opinion on need of regular dental visit was statistically significant when gender, ethnicity and education level were considered. The highest demand for replacement of missing teeth was observed in Kennedy class I and II situations in both upper and lower arches. Demand for fixed prostheses was significantly highest in Kennedy class II in upper and lower arches. In conclusion, although removable prosthodontic options are known to most of the patients, their awareness on tooth and implant supported prostheses is also at a higher level. The highest demand for replacement of missing teeth is by patients with Kennedy class I and II situations whereas Kennedy class II being the category with highest demand for fixed prostheses. We recommend that the location of missing teeth to be considered as a priority when educating patients on the most appropriate prosthetic treatment options. Dentists' involvement in educating patients on prosthetic options needs to be improved.

Revisiting Sex Equality With Transcatheter Aortic Valve Replacement Outcomes: A Collaborative, Patient-Level Meta-Analysis of 11,310 Patients.

Science.gov (United States)

O'Connor, Stephen A; Morice, Marie-Claude; Gilard, Martine; Leon, Martin B; Webb, John G; Dvir, Danny; Rodés-Cabau, Josep; Tamburino, Corrado; Capodanno, Davide; D'Ascenzo, Fabrizio; Garot, Philippe; Chevalier, Bernard; Mikhail, Ghada W; Ludman, Peter F

2015-07-21

There has been conflicting clinical evidence as to the influence of female sex on outcomes after transcatheter aortic valve replacement. The aim of this study was to evaluate the impact of sex on early and late mortality and safety end points after transcatheter aortic valve replacement using a collaborative meta-analysis of patient-level data. From the MEDLINE, Embase, and the Cochrane Library databases, data were obtained from 5 studies, and a database containing individual patient-level time-to-event data was generated from the registry of each selected study. The primary outcome of interest was all-cause mortality. The safety end point was the combined 30-day safety end points of major vascular complications, bleeding events, and stroke, as defined by the Valve Academic Research Consortium when available. Five studies and their ongoing registry data, comprising 11,310 patients, were included. Women constituted 48.6% of the cohort and had fewer comorbidities than men. Women had a higher rate of major vascular complications (6.3% vs. 3.4%; p women and men (2.6 % vs. 2.2% [p = 0.24] and 6.5% vs. 6.5% [p = 0.93], respectively), but female sex was independently associated with improved survival at median follow-up of 387 days (interquartile range: 192 to 730 days) from the index procedure (adjusted hazard ratio: 0.79; 95% confidence interval: 0.73 to 0.86; p = 0.001). Although women experience more bleeding events, as well as vascular and stroke complications, female sex is an independent predictor of late survival after transcatheter aortic valve replacement. This should be taken into account during patient selection for this procedure. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Differential effects of valproic acid and enzyme-inducing anticonvulsants on nimodipine pharmacokinetics in epileptic patients

Science.gov (United States)

Tartara, A.; Galimberti, C.A.; Manni, R.; Parietti, L.; Zucca, C.; Baasch, H.; Caresia, L.; Mück, W.; Barzaghi, N.; Gatti, G.; Perucca, E.

1991-01-01

1 The single dose pharmacokinetics of orally administered nimodipine (60 mg) were investigated in normal subjects and in two groups of epileptic patients receiving chronic treatment with hepatic microsomal enzyme-inducing anticonvulsants (carbamazepine, phenobarbitone or phenytoin) and sodium valproate, respectively. 2 Compared with the values found in the control group, mean areas under the plasma nimodipine concentration curve were lowered by about seven-fold (P anticonvulsants and increased by about 50% (P < 0.05) in patients taking sodium valproate. 3 Nimodipine half-lives were shorter in enzyme-induced patients than in controls (3.9 ± 2.0 h vs 9.1 ± 3.4 h, means ± s.d., P < 0.01), but this difference could be artifactual since in the patients drug concentrations declined rapidly below the limit of assay, thus preventing identification of a possible slower terminal phase. In valproate-treated patients, half-lives (8.2 ± 1.8 h) were similar to those found in controls. PMID:1777370

Breakfast replacement with a low-glycaemic response liquid formula in patients with type 2 diabetes: a randomised clinical trial

NARCIS (Netherlands)

Stenvers, Dirk J.; Schouten, Lydia J.; Jurgens, Jordy; Endert, Erik; Kalsbeek, Andries; Fliers, Eric; Bisschop, Peter H.

2014-01-01

Low-glycaemic index diets reduce glycated Hb (HbA1c) in patients with type 2 diabetes, but require intensive dietary support. Using a liquid meal replacement with a low glycaemic response (GR) may be an alternative dietary approach. In the present study, we investigated whether breakfast replacement

Breakfast replacement with a low-glycaemic response liquid formula in patients with type 2 diabetes : a randomised clinical trial

NARCIS (Netherlands)

Stenvers, Dirk J; Schouten, Lydia J; Jurgens, Jordy; Endert, Erik; Kalsbeek, A.; Fliers, Eric; Bisschop, Peter H

2014-01-01

Low-glycaemic index diets reduce glycated Hb (HbA1c) in patients with type 2 diabetes, but require intensive dietary support. Using a liquid meal replacement with a low glycaemic response (GR) may be an alternative dietary approach. In the present study, we investigated whether breakfast replacement

Enzyme replacement therapy for Anderson-Fabry disease: A complementary overview of a Cochrane publication through a linear regression and a pooled analysis of proportions from cohort studies.

Science.gov (United States)

El Dib, Regina; Gomaa, Huda; Ortiz, Alberto; Politei, Juan; Kapoor, Anil; Barreto, Fellype

2017-01-01

Anderson-Fabry disease (AFD) is an X-linked recessive inborn error of glycosphingolipid metabolism caused by a deficiency of alpha-galactosidase A. Renal failure, heart and cerebrovascular involvement reduce survival. A Cochrane review provided little evidence on the use of enzyme replacement therapy (ERT). We now complement this review through a linear regression and a pooled analysis of proportions from cohort studies. To evaluate the efficacy and safety of ERT for AFD. For the systematic review, a literature search was performed, from inception to March 2016, using Medline, EMBASE and LILACS. Inclusion criteria were cohort studies, patients with AFD on ERT or natural history, and at least one patient-important outcome (all-cause mortality, renal, cardiovascular or cerebrovascular events, and adverse events) reported. The pooled proportion and the confidence interval (CI) are shown for each outcome. Simple linear regressions for composite endpoints were performed. 77 cohort studies involving 15,305 participants proved eligible. The pooled proportions were as follows: a) for renal complications, agalsidase alfa 15.3% [95% CI 0.048, 0.303; I2 = 77.2%, p = 0.0005]; agalsidase beta 6% [95% CI 0.04, 0.07; I2 = not applicable]; and untreated patients 21.4% [95% CI 0.1522, 0.2835; I2 = 89.6%, plinear regression showed that Fabry patients receiving agalsidase alfa are more likely to have higher rates of composite endpoints compared to those receiving agalsidase beta. Agalsidase beta is associated to a significantly lower incidence of renal, cardiovascular and cerebrovascular events than no ERT, and to a significantly lower incidence of cerebrovascular events than agalsidase alfa. In view of these results, the use of agalsidase beta for preventing major organ complications related to AFD can be recommended.

Abnormal serum pancreatic enzymes, but not pancreatitis, are associated with an increased risk of malignancy in patients with intraductal papillary mucinous neoplasms.

Science.gov (United States)

Roch, Alexandra M; Parikh, Janak A; Al-Haddad, Mohammad A; DeWitt, John M; Ceppa, Eugene P; House, Michael G; Nakeeb, Attila; Schmidt, C Max

2014-10-01

Pancreatitis is associated with intraductal papillary mucinous neoplasm (IPMN). This association is in part due to inflammation from pancreatic ductal obstruction. Although the correlation between pancreatitis and the malignant potential of IPMN is unclear, the 2012 International Consensus Guidelines (ICG) consider pancreatitis a "worrisome feature." We hypothesized that serum pancreatic enzymes, markers of inflammation, are a better predictor of malignancy than pancreatitis in patients with IPMN. Between 1992 and 2012, 364 patients underwent resection for IPMN at a single university hospital. In the past decade, serum amylase and lipase were collected prospectively as an inflammatory marker in 203 patients with IPMN at initial surveillance and "cyst clinic" visits. The latest serum pancreatic enzyme values within 3 months preoperatively were studied. Pancreatitis was defined according to the 2012 revision of the Atlanta Consensus. Of the 203 eligible patients, there were 76 with pancreatitis. Pancreatitis was not associated with an increased rate of malignancy (P = .51) or invasiveness (P = .08). Serum pancreatic enzymes categorically outside of normal range (high or low) were also not associated with malignancy or invasiveness. In contrast, as a continuous variable, the higher the serum pancreatic enzymes were, the greater the rate of invasive IPMN. Of the 127 remaining patients without pancreatitis, serum pancreatic enzymes outside of normal range (low and high) were each associated with a greater rate of malignancy (P enzyme levels above normal range (high) were associated with a greater rate of invasiveness (P = .02). In patients with IPMN without a history of pancreatitis, serum pancreatic enzymes outside of the normal range are associated with a greater risk of malignancy. In patients with a history of pancreatitis, there is a positive correlation between the levels of serum pancreatic enzymes and the presence of invasive IPMN. These data suggest

Facial-muscle weakness, speech disorders and dysphagia are common in patients with classic infantile Pompe disease treated with enzyme therapy.

Science.gov (United States)

van Gelder, C M; van Capelle, C I; Ebbink, B J; Moor-van Nugteren, I; van den Hout, J M P; Hakkesteegt, M M; van Doorn, P A; de Coo, I F M; Reuser, A J J; de Gier, H H W; van der Ploeg, A T

2012-05-01

Classic infantile Pompe disease is an inherited generalized glycogen storage disorder caused by deficiency of lysosomal acid α-glucosidase. If left untreated, patients die before one year of age. Although enzyme-replacement therapy (ERT) has significantly prolonged lifespan, it has also revealed new aspects of the disease. For up to 11 years, we investigated the frequency and consequences of facial-muscle weakness, speech disorders and dysphagia in long-term survivors. Sequential photographs were used to determine the timing and severity of facial-muscle weakness. Using standardized articulation tests and fibreoptic endoscopic evaluation of swallowing, we investigated speech and swallowing function in a subset of patients. This study included 11 patients with classic infantile Pompe disease. Median age at the start of ERT was 2.4 months (range 0.1-8.3 months), and median age at the end of the study was 4.3 years (range 7.7 months -12.2 years). All patients developed facial-muscle weakness before the age of 15 months. Speech was studied in four patients. Articulation was disordered, with hypernasal resonance and reduced speech intelligibility in all four. Swallowing function was studied in six patients, the most important findings being ineffective swallowing with residues of food (5/6), penetration or aspiration (3/6), and reduced pharyngeal and/or laryngeal sensibility (2/6). We conclude that facial-muscle weakness, speech disorders and dysphagia are common in long-term survivors receiving ERT for classic infantile Pompe disease. To improve speech and reduce the risk for aspiration, early treatment by a speech therapist and regular swallowing assessments are recommended.

Fate of remnant sinuses of Valsalva in patients with bicuspid and trileaflet valves undergoing aortic valve, ascending aorta, and aortic arch replacement.

Science.gov (United States)

Milewski, Rita Karianna; Habertheuer, Andreas; Bavaria, Joseph E; Siki, Mary; Szeto, Wilson Y; Krause, Eric; Korutla, Varun; Desai, Nimesh D; Vallabhajosyula, Prashanth

2017-08-01

In patients presenting with aortic valvulopathy with concomitant ascending aortic aneurysm, surgical management of the sinus of Valsalva segment remains undefined, especially for moderately dilated aortic roots. In patients with this pathology undergoing aortic valve replacement with supracoronary ascending aorta replacement, we assessed the fate of the remnant preserved sinus of Valsalva segment stratified by aortic valve morphology and pathology. From 2002 to 2015, 428 patients underwent elective aortic valve replacement with supracoronary ascending aorta replacement. Patients were stratified on the basis of valvular morphology (bicuspid aortic valve [n = 254] and tricuspid aortic valve [n = 174]), valvular pathology (bicuspid aortic valve with aortic stenosis [n = 178], bicuspid aortic valve with aortic insufficiency [n = 76], tricuspid aortic valve with aortic stenosis [n = 61], tricuspid aortic valve with aortic insufficiency [n = 113]), and preoperative sinus of Valsalva dimensions (45 mm). Kaplan-Meier analysis revealed no significant difference in freedom from reoperation in tricuspid aortic valve versus bicuspid aortic valve (P = .576). Multivariable Cox regression model performed with sinus of Valsalva dimensions at baseline and follow-up as time-varying covariates did not adversely affect survival. A repeated-measure, mixed-effects model constructed to assess longitudinal sinus of Valsalva trends revealed that the retained sinus of Valsalva dimensions remain stable over long-term follow-up (discharge to ≥10 years), irrespective of valvular morphology/pathology (bicuspid aortic valve with aortic insufficiency, tricuspid aortic valve with aortic insufficiency, tricuspid aortic valve with aortic stenosis) and preoperative sinus of Valsalva groups (45 mm). In patients with nonaneurysmal sinuses of Valsalva undergoing aortic valve replacement with supracoronary ascending aorta replacement, the sinus segment can be preserved irrespective of

Acute gastrointestinal bleeding following aortic valve replacement in a patient with Heyde's sindrome. Case report.

Science.gov (United States)

De Palma, G D; Salvatori, F; Masone, S; Simeoli, I; Rega, M; Celiento, M; Persico, G

2007-09-01

A 58-year old man was admitted to the hospital because of melena. He had a 1-year history of mechanical aortic valve replacement and coronary stent placement because of myocardial infarction and he was taking warfarin and clopidogrel. Esophagogastroduodenoscopy and colonoscopy were negative for bleeding. Capsule endoscopy showed bleeding diffuse angiodysplasia of the small bowel. The patient was treated with octreotide 20 mg, at monthly interval. After 25 months there had been no recurrence of gastrointestinal bleeding. The case suggests that mechanical valve replacement may not prevent gastrointestinal bleeding in Heyde syndrome and that octreotide treatment should be considered in these cases.

Replacement of Antibodies in Pseudo-ELISAs: Molecularly Imprinted Nanoparticles for Vancomycin Detection.

Science.gov (United States)

Canfarotta, Francesco; Smolinska-Kempisty, Katarzyna; Piletsky, Sergey

2017-01-01

The enzyme-linked immunosorbent assay (ELISA) is a widely employed analytical test used to quantify a given molecule. It relies on the use of specific antibodies, linked to an enzyme, to target the desired molecule. The reaction between the enzyme and its substrate gives rise to the analytical signal that can be quantified. Thanks to their robustness and low cost, molecularly imprinted polymer nanoparticles (nanoMIPs) are a viable alternative to antibodies. Herein, we describe the synthesis of nanoMIPs imprinted for vancomycin and their subsequent application in an ELISA-like format for direct replacement of antibodies.

Pain and senzitisation after total knee replacement or nonsurgical treatment in patients with knee osteoarthritis

DEFF Research Database (Denmark)

Arendt-Nielsen, Lars; Simonsen, Ole; Laursen, Mogens Berg

2018-01-01

BACKGROUND: This study is a secondary analysis of 12-month follow-ups from two parallel, randomised controlled trials (RCT) in painful knee osteoarthritis patients. RCT1: Total knee replacement (TKR) followed by non-surgical treatment compared with non-surgical treatment. RCT2: Non...

Long-term outcome on renal replacement therapy in patients who previously received a keto acid-supplemented very-low-protein diet.

Science.gov (United States)

Chauveau, Philippe; Couzi, Lionel; Vendrely, Benoit; de Précigout, Valérie; Combe, Christian; Fouque, Denis; Aparicio, Michel

2009-10-01

The consequences of a supplemented very-low-protein diet remain a matter of debate with regard to patient outcome before or after the onset of renal replacement therapy. We evaluated the long-term clinical outcome during maintenance dialysis and/or transplantation in patients who previously received a supplemented very-low-protein diet. We assessed the outcome of 203 patients who received a supplemented very-low-protein diet for >3 mo (inclusion period: 1985-2000) and started dialysis after a mean diet duration of 33.1 mo (4-230 mo). The survival rate in the whole cohort was 79% and 63% at 5 and 10 y, respectively. One hundred two patients continued with chronic dialysis during the entire follow-up, and 101 patients were grafted at least once. Patient outcomes were similar to those of the French Dialysis Registry patients for the dialysis group and similar to the 865 patients who were transplanted in Bordeaux during the same period for the transplant group. There was no correlation between death rate and duration of diet. The lack of correlation between death rate and duration of diet and the moderate mortality rate observed during the first 10 y of renal replacement therapy confirm that a supplemented very-low-protein diet has no detrimental effect on the outcome of patients with chronic kidney disease who receive renal replacement therapy.

The role of spiral CT in patients with intermediate probability V/Q scans: can spiral CT replace pulmonary angiography?

International Nuclear Information System (INIS)

Vu, T.; Glenn, D.; Lovett, I.; Moses, J.; Wadhwa, S.S.; Nour, R.

2000-01-01

Full text: Spiral CT (SCT) has been advocated as a replacement for pulmonary angiography (PA)in patients with intermediate probability (IP) ventilation-perfusion lung scans (V/Q). More generally it has been proposed as a replacement for V/Q to detect Pulmonary Embolism. This study investigates the accuracy of SCT in the IP patient group 31 patients with IP scans (Modified PIOPED criteria) who were not at high risk of contrast nephrotoxicity were enrolled to have both SCT and PA within the 24 hours following their V/Q. Patients were classified as IP due to a single segmental mismatch (n=7) or a matched V/Q abnormality corresponding to CXR opacity (n=21), or both (n=3). PA is the gold standard for the detection of PE. SCT was read by an experienced radiologist blinded to the PA results. SCT was performed according to standard protocol. All SCT were technically satisfactory for interpretation. Pulmonary embolism was present in 9/31 patients (29%). Of the patients with PE detected by PA, SCT was positive in 4 (44% sensitivity). Of the 22 patients who did not have PE, SCT was negative in 21 and positive in one (96% specificity). In conclusion SCT has limited sensitivity for the detection of PE in patients with IP lung scans. SCT may not be an adequate replacement for PA. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

Sexual activity after total hip replacement in Korean patients: how they do, what they want, and how to improve.

Science.gov (United States)

Yoon, Byung-Ho; Lee, Kyung-Hag; Noh, Serae; Ha, Yong-Chan; Lee, Young-Kyun; Koo, Kyung-Hoi

2013-12-01

Concerns of patients on sexual activity after total hip arthroplasty have not been well studied in Asian patients. This study aimed to determine the following: (1) what are the concerns of patients related to sexual activity after total hip arthroplasty? (2) what are the changes in sexual activity after total hip replacement in Korean patients? Details of sexual activity and concerns were obtained using a questionnaire designed specifically for the study. The questionnaire was administered to 64 patients in a face-to-face interview at an outpatient clinic. Preoperatively, 53.1% of patients experienced difficulties, primarily due to hip pathology and limitations of motion. The median time to the resumption of sexual activity was 3 months postoperatively, and most patients had no increase in the frequency of sexual activity after the total hip replacement. In 39.1% of patients were seen having difficulties with leg positioning following total hip replacement, and they were likely to change coital positions. The most common concern regarding sexual activity of patients was the fear of dislocation. Furthermore, patients with a higher stress level had lower satisfaction rates. Most patients were unable to obtain information on sexual activity following the total hip arthroplasty, and they did not consult with a physician due to the private nature of the topic. Dislocation was the most common concern of patients during sexual activity following a total hip arthroplasty, and a higher stress level was found to be associated with a lower satisfaction rate. Because most patients were unprepared to consult a physician, the provision of appropriate information before a consultation might be beneficial.

Extended replacement of the thoracic aorta.

Science.gov (United States)

Hino, Yutaka; Okada, Kenji; Oka, Takanori; Inoue, Takeshi; Tanaka, Akiko; Omura, Atsushi; Kano, Hiroya; Okita, Yutaka

2013-01-01

We present our experience of total aortic arch replacement. Twenty-nine patients (21 males and 8 females; mean age 63.3 ± 13.3 years) with extended thoracic aortic aneurysms underwent graft replacement. The pathology of the diseased aorta was non-dissecting aneurysm in 11 patients, including one aortitis and aortic dissection in 18 patients (acute type A: one, chronic type A: 11, chronic type B: six). Five patients had Marfan syndrome. In their previous operation, two patients had undergone the Bentall procedure, three had endovascular stenting, one had aortic root replacement with valve sparing and 12 had hemi-arch replacement for acute type A dissection. Approaches to the aneurysm were as follows: posterolateral thoracotomy with rib-cross incision in 16, posterolateral thoracotomy extended to the retroperitoneal abdominal aorta in seven, mid-sternotomy and left pleurotomy in three, anterolateral thoracotomy with partial lower sternotomy in two and clam-shell incision in one patient. Extension of aortic replacement was performed from the aortic root to the descending aorta in 4, from the ascending aorta to the descending aorta in 17 and from the ascending to the abdominal aorta in eight patients. Arterial inflow for cardiopulmonary bypass consisted of the femoral artery in 15 patients, ascending aorta and femoral artery in seven, descending or abdominal aorta in five and ascending aorta in two. Venous drainage site was the femoral vein in 10, pulmonary artery in eight, right atrium in five, femoral artery with right atrium/pulmonary artery in four and pulmonary artery with right atrium in two patients. The operative mortality, 30-day mortality and hospital mortality was one (cardiac arrest due to aneurysm rupture), one (rupture of infected aneurysm) and one (brain contusion), respectively. Late mortality occurred in three patients due to pneumonia, ruptured residual aneurysm and intracranial bleeding. Actuarial survival at 5 years after the operations was 80.6 �

Treatment Opportunities in Patients With Metabolic Myopathies

DEFF Research Database (Denmark)

Ørngreen, Mette Cathrine; Vissing, John

2017-01-01

the development of new therapeutic options. Enzyme replacement therapy with rGAA has revolutionized treatment of early onset Pompe disease. Supplements of riboflavin, carnitine, and sucrose show promise in patients with respectively riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency, primary...... carnitine deficiency, and McArdle disease. Treatment with citric acid cycle intermediates supply by triheptanoin seems promising in patients with glucogenoses, and studies are ongoing in patients with McArdle disease. Summary Treatment of metabolic myopathies primarily relies on avoiding precipitating...

Cardioprotection of Electroacupuncture for Enhanced Recovery after Surgery on Patients Undergoing Heart Valve Replacement with Cardiopulmonary Bypass: A Randomized Control Clinical Trial

Directory of Open Access Journals (Sweden)

Fangxiang Zhang

2017-01-01

Full Text Available We attempted to investigate cardioprotection of electroacupuncture (EA for enhanced recovery after surgery on patients undergoing heart valve replacement with cardiopulmonary bypass. Forty-four patients with acquired heart valve replacement were randomly allocated to the EA group or the control group. Patients in the EA group received EA stimulus at bilateral Neiguan (PC6, Ximen (PC4, Shenting (GV24, and Baihui (GV20 acupoints twenty minutes before anesthesia induction to the end of surgery. The primary end point was cardioprotection effect of electroacupuncture postoperatively and the secondary endpoints were quality of recovery and cognitive functioning postoperatively. The present study demonstrated that electroacupuncture reduced the occurrence of complications and played a role of cardioprotective effect on patients after heart valve replacement surgery with cardiopulmonary bypass, and it benefits patients more comfortable and contributes to recovery after surgery. This trial is registered with ChiCTR-IOC-16009123.

Effects of Estrogen Replacement Therapy on Lipid Peroxidation and Antioxidant Enzyme Activities of Ovariectomized and Ovariectomized-Diabetic Rats

Directory of Open Access Journals (Sweden)

Aslı F. Ceylan-Işık

2007-01-01

Full Text Available Menopause and diabetes are conditions producing free radicals independently from each other. Estrogen replacement therapy which widely used in postmenopausal period has beneficial effects because of its antioxidant property. The study groups were as follows: ovariectomy (n=8, ovariectomy+17-östradiol (n=8, ovariectomy+diabetes (n=10 and ovariectomy+diabetes+17-östradiol (n=8. Diabetes was induced by streptozotocin (45 mg/kg i.p. and the treatment with 17-östradiol (0.1 mg/kg/day was started a week after ovariectomy. After–week long experimental period aortic and uterine tissues were collected from the animals and the malondialdehyde concentration, glutathione peroxidase and catalase activities were quantified. The treatment did not effect blood glucose concentrations, but increased plasma estradiol concentrations. Increased malondialdehyde concentrations were reduced by the treatment in aorta from diabetics and nondiabetics, but the treatment increased malondialdehyde concentrations in nondiabetic uterine while were reducing in diabetic uterine. The treatment also reduced the increased activities of catalase and glutathione peroxidase in aorta from diabetics and nondiabetics, on the other hand the treatment increased the activities of those enzymes in uterine from diabetics and nondiabetics. Our results suggested that estrogen acts as an antioxidant or prooxidant depending on the tissues.

A comparison of minimally invasive and standard aortic valve replacement.

Science.gov (United States)

Stoliński, Jarosław; Plicner, Dariusz; Grudzień, Grzegorz; Wąsowicz, Marcin; Musiał, Robert; Andres, Janusz; Kapelak, Bogusław

2016-10-01

The study objective was to compare aortic valve replacement through a right anterior minithoracotomy with aortic valve replacement through a median sternotomy. With propensity score matching, we selected 211 patients after aortic valve replacement through a right anterior minithoracotomy and 211 patients after aortic valve replacement who underwent operation between January 2010 and December 2013. Perioperative outcomes were analyzed, and multivariable logistic regression analysis of risk factors of postoperative morbidity was performed. For propensity score-matched patients, hospital mortality was 1.0% in the aortic valve replacement through a right anterior minithoracotomy group and 1.4% in the aortic valve replacement group (P = 1.000). Stroke occurred in 0.5% versus 1.4% (P = .615), myocardial infarction occurred in 1.4% versus 1.9% (P = 1.000), and new onset of atrial fibrillation occurred in 12.8% versus 24.2% (P = .003) of patients in the aortic valve replacement through a right anterior minithoracotomy and aortic valve replacement groups, respectively. Postoperative drainage was 353.5 ± 248.6 mL versus 544.3 ± 324.5 mL (P replacement through a right anterior minithoracotomy and aortic valve replacement groups, respectively. Mediastinitis occurred in 2.8% of patients after aortic valve replacement and in 0.0% of patients after aortic valve replacement through a right anterior minithoracotomy surgery (P = .040). Intensive care unit stay (1.3 ± 1.2 days vs 2.6 ± 2.6 days) and hospital stay (5.7 ± 1.6 days vs 8.7 ± 4.4 days) were statistically significantly shorter in the aortic valve replacement through a right anterior minithoracotomy group. Aortic valve replacement through a right anterior minithoracotomy surgery resulted in reduced postoperative morbidity (odds ratio, 0.4; P replacement through a right anterior minithoracotomy surgery resulted in a reduced infection rate, diminished postoperative bleeding and blood

Lipid peroxidation and antioxidant enzymes activity in Plasmodium vivax malaria patients evolving with cholestatic jaundice

Science.gov (United States)

2013-01-01

Background Plasmodium vivax infection has been considered a benign and self-limiting disease, however, recent studies highlight the association between vivax malaria and life-threatening manifestations. Increase in reactive oxygen species has already been described in vivax malaria, as a result of the increased metabolic rate triggered by the multiplying parasite, and large quantities of toxic redox-active byproducts generated. The present study aimed to study the oxidative stress responses in patients infected with P. vivax, who developed jaundice (hyperbilirubinaemia) in the course of the disease, a common clinical complication related to this species. Methods An evaluation of the lipid peroxidation and antioxidant enzymes profile was performed in 28 healthy individuals and compared with P. vivax infected patients with jaundice, i.e., bilirubin jaundice (34 patients), on day 1 (D1) and day 14 (D14) after anti-malarial therapy. Results Hyperbilirubinaemia was more frequent among women and patients experiencing their first malarial infection, and lower haemoglobin and higher lactate dehydrogenase levels were observed in this group. Malondialdehyde levels and activity of celuroplasmin and glutathione reductase were increased in the plasma from patients with P. vivax with jaundice compared to the control group on D1. However, the activity of thioredoxin reductase was decreased. The enzymes glutathione reductase, thioredoxin reductase, thiols and malondialdehyde also differed between jaundiced versus non-jaundiced patients. On D14 jaundice and parasitaemia had resolved and oxidative stress biomarkers were very similar to the control group. Conclusion Cholestatic hyperbilirubinaemia in vivax malaria cannot be totally disassociated from malaria-related haemolysis. However, significant increase of lipid peroxidation markers and changes in antioxidant enzymes in patients with P. vivax-related jaundice was observed. These results suggest oxidative processes contributing

Metabolic Syndrome and Serum Liver Enzymes Level at Patients with Type 2 Diabetes Mellitus

Science.gov (United States)

Music, Miralem; Dervisevic, Amela; Pepic, Esad; Lepara, Orhan; Fajkic, Almir; Ascic-Buturovic, Belma; Tuna, Enes

2015-01-01

Objectives: The aim of this study was to evaluate liver function in patients with type 2 diabetes mellitus (T2DM) with and without metabolic syndrome (MS) by determining serum levels of gamma glutamyltransferase (GGT), alanine aminotransferase (ALT) and aspartate aminotransferase (AST). We also investigated correlation between levels of liver enzymes and some components of MS in both groups of patients. Methods: This cross-sectional study included 96 patients (age 47–83 years) with T2DM. All patients were divided according to the criteria of the National Cholesterol Education Program (NCEP) in two groups: 50 patients with T2 DM and MS (T2DM-MS) and 46 patients with T2DM without MS (T2DM-Non MS). The analysis included blood pressure monitoring and laboratory tests: fasting blood glucose (FBG), total lipoprotein cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), triglyceride (TG), fibrinogen and liver enzymes: GGT, ALT and AST. T2DM-MS group included patients which had FBG ≥ 6,1 mmol/L, TG ≥ 1,7 mmol/L and blood pressure ≥ 130/85 mm Hg. Results: T2DM-MS patients had significant higher values of systolic blood pressure, diastolic blood pressure and medium arterial pressure compared to T2DM-Non MS patients. Serum levels of TC, TG, LDL-C, VLDL-C and FBG were significantly higher in the T2DM-MS group compared to the T2DM-Non MS group. Serum fibrinogen level and GGT level were significantly higher in patients with T2DM-MS compared to the serum fibrinogen level and GGT level in T2DM-Non MS patients. Mean serum AST and ALT level were higher, but not significantly, in patients with T2DM and MS compared to the patients with T2DM without MS. Significant negative correlations were observed between TC and AST (r= -0,28, p<0,05), as well as between TC and ALT level (r= -0,29, p<0,05) in T2DM-MS group of patients. Conclusion: These results suggest that patients with T2DM and MS have markedly elevated liver enzymes. T2DM and MS probably play a role in

A Korean Multi-Center Survey about Warfarin Management before Gastroenterological Endoscopy in Patients with a History of Mechanical Valve Replacement Surgery

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Kuk Hui Son

2016-10-01

Full Text Available Background: Guidelines for esophagogastroduodenoscopy (EGD in the West allow the continued use of warfarin under therapeutic international normalized ratio (INR level. In Korea, no guidelines have been issued regarding warfarin treatment before EGD. The authors surveyed Korean cardiac surgeons about how Korean cardiac surgeons handle warfarin therapy before EGD using a questionnaire. Participants were requested to make decisions regarding the continuation of warfarin therapy in two hypothetical cases. Methods: The questionnaire was administered to cardiac surgeons and consisted of eight questions, including two case scenarios. Results: Thirty- six cardiac surgeons at 28 hospitals participated in the survey, and 52.7% of the participants chose to stop warfarin before EGD in aortic valve replacement patients without risk factors for thromboembolism. When the patient’s INR level was 2, 31% of the participants indicated that they would choose to continue warfarin therapy. For EGD with biopsy, 72.2% of the participants chose warfarin withdrawal, and 25% of the participants chose heparin replacement. In mitral valve replacement patients, 47.2% of the participants chose to discontinue warfarin, and 22.2% of the participants chose heparin replacement. For EGD with biopsy in patients with a mitral valve replacement, 58.3% of the participants chose to stop warfarin, and 41.7% of the participants chose heparin replacement. Conclusion: This study demonstrated that attitudes regarding warfarin treatment for EGD are very different among Korean surgeons. Guidelines specific to the Korean population are required.

Replacing corn with pearl millet (raw and sprouted) with and without enzyme in chickens' diet.

Science.gov (United States)

Afsharmanesh, M; Ghorbani, N; Mehdipour, Z

2016-04-01

An experiment was conducted to compare a commercial corn-soya bean meal diet with a pearl millet (raw and sprouted) diet containing less soya bean meal, alone or in combination with exogenous enzyme, on growth performance and ileal villus development of chicks. Two-hundred-and-forty-one-day-old male broilers (10/pen) were randomly allocated to one of the following dietary treatments: (i) a standard corn-soya bean meal control diet (CTL); (ii) a raw pearl millet-soya bean meal diet (PM); (iii) a sprouted pearl millet-soya bean meal diet (SPM); (iv) CTL + exogenous enzymes (CE); (v) PM + exogenous enzymes (PE); and (vi) SPM + exogenous enzymes (SPE) with four replicate pens/treatment. Body weight of birds at day 21 did not differ between those fed the CTL, and SPM and PE diets. In comparison with feeding broilers the CTL diet, feeding the PE and SPM diets caused significant decrease in feed intake, but with equivalent growth and feed efficiency. However, at day 21, feed conversion ratio did not differ between birds fed the CTL diet and those fed the PM, PE and SPM diets. At day 21, broilers fed the PM and PE diets had longer villi (p diet. At day 21, villi width was reduced (p diet. It is concluded that, in comparison with corn, broiler diets formulated with sprouted pearl millet or pearl millet with enzyme require less soya bean meal and can be used to improve growth performance traits and villus development. Journal of Animal Physiology and Animal Nutrition © 2015 Blackwell Verlag GmbH.

Efficacy and safety of transcatheter aortic valve replacement in aortic stenosis patients at low to moderate surgical risk: a comprehensive meta-analysis

OpenAIRE

Elmaraezy, Ahmed; Ismail, Ammar; Abushouk, Abdelrahman Ibrahim; Eltoomy, Moutaz; Saad, Soha; Negida, Ahmed; Abdelaty, Osama Mahmoud; Abdallah, Ahmed Ramadan; Aboelfotoh, Ahmed Magdy; Hassan, Hossam Mahmoud; Elmaraezy, Aya Gamal; Morsi, Mahmoud; Althaher, Farah; Althaher, Moath; AlSafadi, Ammar M.

2017-01-01

Background Recently, transcatheter aortic valve replacement (TAVR) has become the procedure of choice in high surgical risk patients with aortic stenosis (AS). However, its value is still debated in operable AS cases. We performed this meta-analysis to compare the safety and efficacy of TAVR to surgical aortic valve replacement (SAVR) in low-to-moderate surgical risk patients with AS. Methods A systematic search of five authentic databases retrieved 11 eligible studies (20,056 patients). Rele...

Autophagy and Mis-targeting of Therapeutic Enzyme in Skeletal Muscle in Pompe Disease

Science.gov (United States)

Fukuda, Tokiko; Ahearn, Meghan; Roberts, Ashley; Mattaliano, Robert J.; Zaal, Kristien; Ralston, Evelyn; Plotz, Paul H.; Raben, Nina

2009-01-01

Enzyme replacement therapy (ERT) became a reality for patients with Pompe disease, a fatal cardiomyopathy and skeletal muscle myopathy caused by a deficiency of glycogen-degrading lysosomal enzyme acid alpha-glucosidase (GAA). The therapy, which relies on receptor-mediated endocytosis of recombinant human GAA (rhGAA), appears to be effective in cardiac muscle, but less so in skeletal muscle. We have previously shown a profound disturbance of the lysosomal degradative pathway (autophagy) in therapy-resistant muscle of GAA knockout mice (KO). Our findings here demonstrate a progressive age-dependent autophagic build-up in addition to enlargement of glycogen-filled lysosomes in multiple muscle groups in the KO. Trafficking and processing of the therapeutic enzyme along the endocytic pathway appear to be affected by the autophagy. Confocal microscopy of live single muscle fibers exposed to fluorescently labeled rhGAA indicates that a significant portion of the endocytosed enzyme in the KO was trapped as a partially processed form in the autophagic areas instead of reaching its target – the lysosomes. A fluid-phase endocytic marker was similarly mis-targeted and accumulated in vesicular structures within the autophagic areas. These findings may explain why ERT often falls short of reversing the disease process, and point to new avenues for the development of pharmacological intervention. PMID:17008131

Mortality from infections and malignancies in patients treated with renal replacement therapy: data from the ERA-EDTA registry

NARCIS (Netherlands)

Vogelzang, Judith L.; van Stralen, Karlijn J.; Noordzij, Marlies; Diez, Jose Abad; Carrero, Juan J.; Couchoud, Cecile; Dekker, Friedo W.; Finne, Patrik; Fouque, Denis; Heaf, James G.; Hoitsma, Andries; Leivestad, Torbjørn; de Meester, Johan; Metcalfe, Wendy; Palsson, Runolfur; Postorino, Maurizio; Ravani, Pietro; Vanholder, Raymond; Wallner, Manfred; Wanner, Christoph; Groothoff, Jaap W.; Jager, Kitty J.

2015-01-01

Infections and malignancies are the most common non-cardiovascular causes of death in patients on chronic renal replacement therapy (RRT). Here, we aimed to quantify the mortality risk attributed to infections and malignancies in dialysis patients and kidney transplant recipients when compared with

Mortality from infections and malignancies in patients treated with renal replacement therapy: data from the ERA-EDTA registry

NARCIS (Netherlands)

Vogelzang, J.L.; Stralen, K.J. van; Noordzij, M.; Diez, J.A.; Carrero, J.J.; Couchoud, C.; Dekker, F.W.; Finne, P.; Fouque, D.; Heaf, J.G.; Hoitsma, A.J.; Leivestad, T.; Meester, J. de; Metcalfe, W.; Palsson, R.; Postorino, M.; Ravani, P.; Vanholder, R.; Wallner, M.; Wanner, C.; Groothoff, J.W.; Jager, K.J.

2015-01-01

BACKGROUND: Infections and malignancies are the most common non-cardiovascular causes of death in patients on chronic renal replacement therapy (RRT). Here, we aimed to quantify the mortality risk attributed to infections and malignancies in dialysis patients and kidney transplant recipients when

Treatable Bedridden Elderly―Recovery from Flexion Contracture after Cortisol Replacement in a Patient with Isolated Adrenocorticotropic Hormone Deficiency

Science.gov (United States)

Tanaka, Takamasa; Terada, Norihiko; Fujikawa, Yoshiki; Fujimoto, Takushi

2016-01-01

Isolated adrenocorticotropic hormone deficiency (IAD) is a rare disorder with diverse clinical presentations. A 79-year-old man was bedridden for six months due to flexion contractures of the bilateral hips and knees, along with hyponatremia. He was diagnosed with IAD based on the results of endocrine tests. After one month of corticosteroid replacement, he recovered and was able to stand up by himself. Although flexion contracture is a rare symptom of IAD, steroid replacement therapy may be effective, even for seemingly irreversibly bedridden elderly patients. In bedridden elderly patients with flexion contractures, we should consider and look for any signs of adrenal insufficiency. PMID:27746435

Treatable Bedridden Elderly -Recovery from Flexion Contracture after Cortisol Replacement in a Patient with Isolated Adrenocorticotropic Hormone Deficiency.

Science.gov (United States)

Tanaka, Takamasa; Terada, Norihiko; Fujikawa, Yoshiki; Fujimoto, Takushi

Isolated adrenocorticotropic hormone deficiency (IAD) is a rare disorder with diverse clinical presentations. A 79-year-old man was bedridden for six months due to flexion contractures of the bilateral hips and knees, along with hyponatremia. He was diagnosed with IAD based on the results of endocrine tests. After one month of corticosteroid replacement, he recovered and was able to stand up by himself. Although flexion contracture is a rare symptom of IAD, steroid replacement therapy may be effective, even for seemingly irreversibly bedridden elderly patients. In bedridden elderly patients with flexion contractures, we should consider and look for any signs of adrenal insufficiency.

Patient-prosthesis mismatch has no influence on in-hospital mortality after aortic valve replacement.

Science.gov (United States)

Yottasurodom, Chaiwut; Namthaisong, Kriengkrai; Porapakkham, Pramote; Kasemsarn, Choosak; Chotivatanapong, Taweesak; Chaiseri, Pradistchai; Wongdit, Suwannee; Yasotarin, Suwanna

2012-08-01

To analyze the relationship between prosthetic aortic valve orifice and body surface area (Effective Orifice Area Index, EOAI) and in-hospital mortality after aortic valve replacement. A prospective study was conducted between October 2007 to September 2010, 536 patients underwent isolated aortic valve replacement (AVR) was recorded on preoperative, operative and postoperative data. Patient Prosthesis Mismatch (PPM) was classified by Effective Orifice Area Indexed (EOAI) by prosthetic valve area divided by body surface area as mild or no significance if the EOAI is greater than 0.85 cm2/m2, moderate if between 0.65 cm2/m2 and 0.85 cm2/m2, and severe if less than 0.65 cm2/m2. Statistical differences were analyzed by Chi-square and student t-test with p-value less than 0.05 considered significant. There were 304 men, mean age was 60.98 years, mean valve orifice area 1.69 cm2, body surface area 1.60 m2, cross clamp time 1.13 hrs., bypass time 1.67 hrs. Mechanical valves were used in 274 patients (51.2%) and Bioprosthesis were used in 181 patients (48.8%). PPM was found in 33.7%, 6.7% was severe PPM, 27% was moderate PPM and 66.3% has no significant PPM Over all in-hospital mortality was 1.5%. There was no significant difference in hospital mortality between no PPM group, moderate PPM and severe PPM group (1.4% vs. 1.4% vs. 5.4%, p-value = 0.86). In a large aortic valve surgery population, moderate and severe patient prosthesis mismatch occurred in 35.6% of patients but had no influence on in-hospital mortality.

Human triose-phosphate isomerase deficiency: a single amino acid substitution results in a thermolabile enzyme.

Science.gov (United States)

Daar, I O; Artymiuk, P J; Phillips, D C; Maquat, L E

1986-10-01

Triose-phosphate isomerase (TPI; D-glyceraldehyde-3-phosphate ketol-isomerase, EC 5.3.1.1) deficiency is a recessive disorder that results in hemolytic anemia and neuromuscular dysfunction. To determine the molecular basis of this disorder, a TPI allele from two unrelated patients homozygous for TPI deficiency was compared with an allele from a normal individual. Each disease-associated sequence harbors a G X C----C X G transversion in the codon for amino acid-104 and specifies a structurally altered protein in which a glutamate residue is replaced by an aspartate residue. The importance of glutamate-104 to enzyme structure and function is implicated by its conservation in the TPI protein of all species that have been characterized to date. The glutamate-to-aspartate substitution results in a thermolabile enzyme as demonstrated by assays of TPI activity in cultured fibroblasts of each patient and cultured Chinese hamster ovary (CHO) cells that were stably transformed with the mutant alleles. Although this substitution conserves the overall charge of amino acid-104, the x-ray crystal structure of chicken TPI indicates that the loss of a side-chain methylene group (-CH2CH2COO- ---- -CH2COO-) is sufficient to disrupt the counterbalancing of charges that normally exists within a hydrophobic pocket of the native enzyme.

Imaging techniques in transcatheter aortic valve replacement

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Quaife RA

2013-11-01

Full Text Available Robert A Quaife, Jennifer Dorosz, John C Messenger, Ernesto E Salcedo Division of Cardiology, University of Colorado, Aurora, CO, USA Abstract: Calcific aortic stenosis is now understood as a complex valvular degenerative process sharing many risk factors with atherosclerosis. Once patients develop symptomatic calcific aortic stenosis, the only effective treatment is aortic valve replacement. In the past decade, transcatheter aortic valve replacement (TAVR has been developed as an alternative to surgery to treat severe calcific aortic stenosis. Cardiac imaging plays a pivotal role in the contemporary management of patients with calcific aortic stenosis, and particularly in patients being considered for TAVR, who demand detailed imaging of the aortic valve apparatus. In this review, we highlight the role of cardiac imaging for patient selection, procedural guidance, and evaluation of results of TAVR. Keywords: aortic stenosis, cardiovascular imaging, transcutaneous aortic valve replacement

Nursing intervention by telephone interviews of patients aged over 65 years after total hip replacement improves health status: a randomised clinical trial Nursing intervention by telephone interviews of patients aged over 65 years after total hip replacement improves health status: a randomised

DEFF Research Database (Denmark)

Hørdam, Britta

2010-01-01

and over by using telephone support and counselling 2 and 10 weeks after surgery compared with a control group receiving conventional care and treatment. Design: A randomised clinical trial focusing on patients' health status by using short-form 36 at 4 weeks preoperatively and 3 and 9 months...... postoperatively was carried out. Sample: 180 patients aged 65 years and over were randomised 4 weeks preoperatively to either control or intervention groups. Measurements: both groups received conventional surgical treatment, but the intervention group was interviewed by telephone 2 and 10 weeks after surgery......Nursing intervention by telephone interviews of patients aged over 65 years after total hip replacement improves health status: a randomised clinical trial Objective: We hypothesised that all areas of health status after total hip replacement could be improved in patients aged over 65 years...

Therapeutic response to intravenous infusions of glucocerebrosidase in a patient with Gaucher disease

International Nuclear Information System (INIS)

Barton, N.W.; Furbish, F.S.; Murray, G.J.; Garfield, M.; Brady, R.O.

1990-01-01

Enzyme replacement has been under consideration as a therapeutic strategy for patients with Gaucher disease for more than two decades. Previous studies indicated that single injections of purified glucocerebrosidase reduced the amount of storage material in the liver. It was important to determine whether administration of exogenous enzyme on a regular basis would be of clinical benefit. The authors weekly i.v. infusions of a macrophage-targeted preparation of human placental glucocerebrosidase in a child with type 1 Gaucher disease increased hemoglobin over a 20-week period. The platelet count also increased. Phagocytic activity in the spleen decreased during the period of enzyme administration, and there was radiographic evidence of skeletal improvement. These observations document objective clinical responses to enzyme supplementation in a patient with a sphingolipid storage disorder

Angiotensin-converting enzyme gene 2350 G/A polymorphism and susceptibility to atrial fibrillation in Han Chinese patients with essential hypertension

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Min-Hui Jiang

2013-11-01

Full Text Available OBJECTIVE: The angiotensin-converting enzyme gene is one of the most studied candidate genes related to atrial fibrillation. Among the polymorphisms of the angiotensin-converting enzyme gene, the 2350 G/A polymorphism (rs4343 is known to have the most significant effects on the plasma angiotensin-converting enzyme concentration. The aim of the present study was to investigate the association of the angiotensin-converting enzyme 2350 G/A polymorphism with atrial fibrillation in Han Chinese patients with essential hypertension. METHODS: A total of 169 hypertensive patients were eligible for this study. Patients with atrial fibrillation (n = 75 were allocated to the atrial fibrillation group, and 94 subjects without atrial fibrillation were allocated to the control group. The PCR-based restriction fragment length polymorphism technique was used to assess the genotype frequencies. RESULTS: The distributions of the angiotensin-converting enzyme 2350 G/A genotypes (GG, GA, and AA, respectively were 40.43%, 41.49%, and 18.08% in the controls and 18.67%, 46.67%, and 34.66% in the atrial fibrillation subjects (p = 0.037. The frequency of the A allele in the atrial fibrillation group was significantly greater than in the control group (58.00% vs. 38.83%, p = 0.0007. Compared with the wild-type GG genotype, the GA and AA genotypes had an increased risk for atrial fibrillation. Additionally, atrial fibrillation patients with the AA genotype had greater left atrial dimensions than the patients with the GG or GA genotypes (p<0.01 and p<0.05, respectively. CONCLUSIONS: The results obtained in this study indicate that the angiotensin-converting enzyme 2350 G/A polymorphism is associated with atrial fibrillation and that the A allele shows an increased risk for atrial fibrillation in Han Chinese patients with essential hypertension.

Liver enzymes in patients with chronic kidney disease undergoing peritoneal dialysis and hemodialysis

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Isabella Ramos de Oliveira Liberato

2012-01-01

Full Text Available OBJECTIVE: The present study was designed to analyze the serum levels of aspartate and alanine aminotransferases, gamma-glutamyl transferase, and the hematocrit in patients with chronic kidney disease who were undergoing peritoneal dialysis or hemodialysis. PATIENTS AND METHODS: Twenty patients on peritoneal dialysis and 40 on hemodialysis were assessed, and the patients were matched according to the length of time that they had been on dialysis. Blood samples were collected (both before and after the session for those on hemodialysis to measure the enzymes and the hematocrit. RESULTS: In the samples from the patients who were undergoing peritoneal dialysis, the aspartate and alanine aminotransferase levels were slightly higher compared with the samples collected from the patients before the hemodialysis session and slightly lower compared with the samples collected after the hemodialysis session. The levels of gamma-glutamyl transferase in the hemodialysis patients were slightly higher than the levels in the patients who were undergoing peritoneal dialysis. In addition, the levels of aminotransferases and gamma-glutamyl transferase that were collected before the hemodialysis session were significantly lower than the values collected after the session. The hematocrit levels were significantly lower in the patients who were on peritoneal dialysis compared with the patients on hemodialysis (both before and after the hemodialysis session, and the levels were also significantly lower before hemodialysis compared with after hemodialysis. CONCLUSION: The aminotransferase levels in the patients who were undergoing peritoneal dialysis were slightly higher compared with the samples collected before the hemodialysis session, whereas the aminotransferase levels were slightly lower compared with the samples collected after the session. The hematocrits and the aminotransferase and gamma-glutamyl transferase levels of the samples collected after the

Effect of regional citrate anticoagulation on critical patients with continuous renal replacement therapy

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Li-Li You

2016-12-01

Full Text Available Objective: To investigate the efficacy and safety of regional citrate anticoagulation (RCA in continuous renal replacement therapy (CRRT for critical patients. Methods: A total of 83 critical patients need CRRT in the intensive care units of our hospital from July 2012 to June 2016 were recruited in the study, and the patients were divided into two groups randomly, the patients in observation group received the RCA treatment, and the patients in control group received traditional low molecular heparin anticoagulation. The difference of safety indicators, biochemical indicators, extracorporeal circulation blood coagulation condition and complications in patients were determined between two groups. Results: Compared with control group, the patients in observation group had an elevated level of iCa2+, the level of chloride ion reduced, the use time of filter increased, the bleeding cases reduced, the concentrations of urea nitrogen, creatinine TNF-α , IL-1β, IL-8 and NO were all significantly downregulated, the data have a significant difference (P < 0.05. Conclusions: RCA is a safe and effective method for CRRT in patients with a high risk of bleeding.

Can shared care deliver better outcomes for patients undergoing total hip replacement? A prospective assessment of patient outcomes and associated service use

NARCIS (Netherlands)

Rosendal, H.; Beekum, W.T. van; Nijhof, P.; Witte, L.P. de; Schrijvers, A.J.P.

2000-01-01

Objectives: To assess whether shared care for patients undergoing total hip replacement delivers better outcomes compared to care as usual. Design: Prospective, observational cohort study. Setting: Two regions in the Netherlands where different organisational health care models have been

Impact of Prosthesis-Patient Mismatch on Long-term Functional Capacity After Mechanical Aortic Valve Replacement

NARCIS (Netherlands)

Petit-Eisenmann, H.; Epailly, E.; Velten, M.; Radojevic, J.; Eisenmann, B.; Kremer, H.; Kindo, M.

2016-01-01

BACKGROUND: The impact of prosthesis-patient mismatch (PPM) after aortic valve replacement (AVR) for aortic stenosis on exercise capacity remains controversial. The aim of this study was to analyze the long-term impact of PPM after mechanical AVR on maximal oxygen uptake (VO2max). METHODS: The study

Faecal excretion of brush border membrane enzymes in patients with clostridium difficile diarrhoea

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Katyal R

2002-01-01

Full Text Available PURPOSE: To look for the presence of intestinal brush border membrane (BBM enzymes in the faecal samples of patients with Clostridium difficile association. METHODS: One hundred faecal samples were investigated for C.difficile toxin (CDT. Simultaneous assays for faecal excretion of intestinal BBM enzymes viz., disaccharidases, alkaline phosphatase (AP and leucine aminopeptidase (LAP were also done. RESULTS: C.difficile toxin was detected in 25 (25% of the samples with a titre ranging from 10 to 160. No significant difference (p>0.05 was seen between the CDT positive and negative groups with any of the disaccharidases studied. However, significant increase (pC.difficile diarrhoea.

Pitfalls when implementing nurse-led annual telephone calls to replace outpatient visits for Inflammatory Bowel Disease patients

DEFF Research Database (Denmark)

Jakobsen, Dorrit; Bager, Palle; Hentze, Runa

Background: In Denmark, patients with Inflammatory Bowel Disease (IBD) and disease in remission are mainly seen on a regularly basis in outpatient clinics, by gastroenterologists. Replacing the regular visits with annual telephone calls performed by IBD nurses seems beneficial, both for the patie......Background: In Denmark, patients with Inflammatory Bowel Disease (IBD) and disease in remission are mainly seen on a regularly basis in outpatient clinics, by gastroenterologists. Replacing the regular visits with annual telephone calls performed by IBD nurses seems beneficial, both...... for the patients and for the outpatient clinic. The purpose of this study was to identify potential barriers and pitfalls in the planning and implementation phase of a nurse-led telephone service. Methods: Preparations prior to introducing the annual telephone calls were done in 2010. From January 2011 all...... eligible IBD patients were shifted from regularly visits to annual telephone calls performed by an IBD nurse. As the intervention contained of several sub-elements and the elements were subject to adjustment during implementation, the intervention was regarded as a Complex Intervention. All the elements...

Patient factors in referral choice for total joint replacement surgery.

Science.gov (United States)

Conner-Spady, Barbara L; Marshall, Deborah A; Bohm, Eric; Dunbar, Michael J; Loucks, Lynda; Hennigar, Allan W; Frank, Cy; Noseworthy, Tom W

2014-04-01

Although the option of next available surgeon can be found on surgeon referral forms for total joint replacement surgery, its selection varies across surgical practices. Objectives are to assess the determinants of (a) a patient's request for a particular surgeon; and (b) the actual referral to a specific versus the next available surgeon. Questionnaires were mailed to 306 consecutive patients referred to orthopedic surgeons. We assessed quality of life (Oxford Hip and Knee scores, Short Form-12, EuroQol 5D, Pain Visual Analogue Scale), referral experience, and the importance of surgeon choice, surgeon reputation, and wait time. We used logistic regression to build models for the 2 objectives. We obtained 176 respondents (response rate, 58%), 60% female, 65% knee patients, mean age of 65 years, with no significant differences between responders versus nonresponders. Forty-three percent requested a particular surgeon. Seventy-one percent were referred to a specific surgeon. Patients who rated surgeon choice as very/extremely important [adjusted odds ratio (OR), 6.54; 95% confidence interval (CI), 2.57-16.64] and with household incomes of $90,000+ versus <$30,000 (OR, 5.74; 95% CI, 1.56-21.03) were more likely to request a particular surgeon. Hip patients (OR, 3.03; 95% CI, 1.18-7.78), better Physical Component Summary-12 (OR, 1.29; 95% CI, 1.02-1.63), and patients who rated surgeon choice as very/extremely important (OR, 3.88; 95% CI, 1.56-9.70) were more likely to be referred to a specific surgeon. Most patients want some choice in the referral decision. Providing sufficient information is important, so that patients are aware of their choices and can make an informed choice. Some patients prefer a particular surgeon despite longer wait times.

Arteriovenous thrombosis in chronic renal failure patients receving renal replacement therapy

International Nuclear Information System (INIS)

Shoaib, M.; Naz, A.

2008-01-01

To determine the frequency of thrombotic complications and to identify factors associated with arteriovenous thrombosis in patients of chronic renal failure receiving renal replacement therapy. Of the 3000 patients evaluated, 61 End Stage Renal Disease (ESRD) patients on regular dialysis, having recent renal transplant, were selected for the study after informed consent. These patients had arteriovenous thrombosis with temporary central lines thrombosis and vascular access problems. Cases of congenital or acquired thrombotic disorders, e.g. with malignancy, DIC, liver disease, systemic lupus erythematosus or other immunologic diseases, pregnancy or women using oral contraceptives, were excluded. Similarly, patients taking any type of anticoagulant therapy during the preceding one week were not included in the study. Findings were recorded in a structured questionnaire. Laboratory analysis was done after clinical and radiological evaluation. Thrombophilia screening included antithrombin, protein C, protein S deficiencies and lupus anticoagulant. Forty-seven out of 61 patients selected were positive for thrombophilia screening with protein C deficiency in 26.2%, protein S deficiency in 16.3%, antithrombin in 5%, lupus anticoagulant in 13.1% and combined deficiency was observed in 16.3%. Of the 3000 patients, 61 with frequency of 2% were found to be deficient in one or had combined deficiency of these. Thus, the study of ESRD patients presenting with arteriovenous thromboembolism emphasizes the need to reconsider the perception that this clinical entity is rare and requires further studies. (author)

The effect of posterior and lateral approach on patient-reported outcome measures and physical function in patients with osteoarthritis, undergoing total hip replacement

DEFF Research Database (Denmark)

Rosenlund, Signe; Broeng, Leif; Jensen, Carsten

2014-01-01

BACKGROUND: Total hip replacement provides pain relief and improves physical function and quality of life in patients with end-stage hip osteoarthritis. The incidence of hip replacement operations is expected to increase due to the growing elderly population. Overall, the posterior approach...... Outcome Score, subscale of "Physical function Short form" (HOOS-PS) Secondary outcome measures include two other subscales of HOOS ("Pain" and "Hip related Quality of Life"), physical activity level (UCLA activity score), limping (HHS) and general health status (EQ-5D-3L). Explorative outcomes include...... physical function and pain; however, this has not been investigated in a randomised controlled trial with a twelve-month follow-up. We hypothesized that the lateral approach has an inferior outcome in patient-reported outcome compared with the posterior approach after one year. METHODS/DESIGN: The trial...

Clinical and Radiographic Assessment of Reasons for Replacement of Metal- Ceramic Fixed Dental Prostheses in Patients Referring to Dental School.

Science.gov (United States)

Al Refai, Roa'a; Saker, Samah

2018-01-01

The expected length of service and reasons for fixed dental prostheses (FDPs) replacement are a frequent inquiry by patients while the answers were mainly based on studies reports that was conducted outside the middle east region. This clinical and radiographic survey was constructed to assess and survey clinically and radiographically the reasons of replacement of metal-ceramic fixed dental prostheses, amongst patients reporting at dental school in Taibah University. Between January and May 2016, 151 patients were recruited for this study. Interview (include questions pertained to the length of service of the prosthesis, the nature of complaint as told by patient in her own words), clinical examination, intra-oral photographs, and periapical radiographs, were done by the researchers. The parameters assessed were secondary caries, open margins, loss of retention, failure of endodontic treatment of the abutment and periodontal diseases. A total number of 249 failed fixed dental prostheses were evaluated. Of which 180 (39.7%) were single crowns, 159 (35.0%) were retainers and 117 (25.8%) were pontics in 69 fixed partial denture. The most common reason for replacement of fixed restorations was periodontal diseases affecting 92.8% of all types' restorations, followed by defective margin in 90.4% of examined restoration, poor aesthetic in 88% of restorations, while periapical involvement was found in 85.5% of fixed dental prosthesis. The survival rates of fixed prostheses were not predictable, and no association was found between number of years in service and the number of restorations. The most common reasons for replacing single unit fixed dental prostheses are periodontal diseases and periapical involvement, while defective margins and poor aesthetic mainly associated with multi-unit fixed dental prostheses. Key words: Failure, Fixed dental prosthesis, Survival, Replacement.

Performance of broilers fed enzyme-supplemented tigernut ...

African Journals Online (AJOL)

A feeding trial was set up to study the effects of replacing maize with tigernut meal (TGN) at 0, 33.33, 66.67 and 100 per cent levels, with 0.10 per cent enzyme supplementation of all levels, on performance characteristics and carcass yield in broiler chicken for 8 weeks (56 days). A total of 200 Anak-2000 breed of broilers ...

No Effect of Acupuncture as Adjunctive Therapy for Patients with Total Knee Replacement

DEFF Research Database (Denmark)

Petersen, Tom; Hautopp, Holger; Duus, Benn

2018-01-01

Objective: Acupuncture is a low risk option in pain management following total knee replacement as an alternative to opioid analgesics. Therefore, the benefit of acupuncture as adjunct to an exercise program was investigated. Furthermore, the modifying effect of previous benefit from acupuncture...... was explored. Design: Three weeks postoperatively, eligible patients were randomized to acupuncture and exercises or exercises alone. Setting: An outpatient rehabilitation centre in the municipality of Copenhagen, Denmark. Subjects: A total of 172 patients were included. Methods: Main outcome was proportion...... of the course of treatment were assessed. Results: No additional benefit of acupuncture was found on any of the main outcomes. Between-group differences were non-significant in proportions of patients with a clinically important reduction of night pain (Relative Risk: 0.98; 95% Confidence Interval (CI): 0...

Role of Liver Function Enzymes in Diagnosis of Choledocholithiasis in Biliary Colic Patients

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Mohammad Hussein Mirshamsi

2011-10-01

Full Text Available Liver functional tests due to inflammatory process which induced by cholecystitis might changed and some clinicians suggested that these changes might help us to stone prediction in common bile ducts and decrease hazards of performing ERCP and other invasive procedures. Present study was performed for assessment of role of liver functional test in diagnosis of common bile duct stone in patients with cholecystitis and help in their management. Present prospective study was performed between April 2010 and March 2011 on 350 patients who come to our hospital with cholecystitis or biliary colic diagnosis. Patients with cholesistitis diagnosis were underwent operation for removing gall bladder stone and retrograde cholangiopancreatography (ERCP was performed for patients with suspicious to biliary colic and common bile duct (CBD stones. Ultrasonography, Aspartate Aminotransferases (AST, Alanine Aminotransferases (ALT, Alkaline Phosphatase (ALP and direct and total serum bilirubin were measured for all of participated patients. Mean of AST. ALT, ALP and total and direct bilirubin were had no significant differences between two study groups. In logistic regression analysis, after entering into the model only CBD diameter (OR: 20; P=0.00 and elevated serum level of ALT (OR: 2; P=0.04 were remained into the model and were known as independent predictor of cholelithiasis. Elevated level of liver enzymes had not main role in CBD diagnosis and ERCP had no to perform for suspicious CBD stone only with elevated liver enzyme and even with normal ultrasonography findings. Endosonography as non invasive procedure recommend for patients before ERCP.

Hydrogen sulfide synthesis enzymes reduced in lower esophageal sphincter of patients with achalasia.

Science.gov (United States)

Zhang, L; Zhao, W; Zheng, Z; Wang, T; Zhao, C; Zhou, G; Jin, H; Wang, B

2016-10-01

The etiology of achalasia remains largely unknown. Considerable evidence reveals that the lower esophageal sphincter dysfunction is due to the lack of inhibitory neurotransmitter, secondary to esophageal neuronal inflammation or loss. Recent studies suggest hydrogen sulfide may act as an inhibitory transmitter in gastrointestinal tract, but study about hydrogen sulfide in human esophagus still lack. The aim of the study was to investigate if hydrogen sulfide synthesis enzymes could be detected in human esophagus and if the synthesis of the endogenous hydrogen sulfide could be affected in achalasia patients. Tissue samples in cardia, lower esophageal sphincter, 2 cm and 4 cm above lower esophageal sphincter were obtained from achalasia patients undergoing peroral endoscopic myotomy. Control tissues in lower esophageal sphincter were obtained from esophageal carcinoma patients. Expression of cystathionine-β-synthase and cystathionine-γ-lyase in lower esophageal sphincter of achalasia patients and control were detected by immunohistochemical staining. In addition, expression of cystathionine-β-synthase and cystathionine-γ-lyase were compared among different parts of esophagus in achalasia patients. Compared with control, the expression of cystathionine-β-synthase and cystathionine-γ-lyase in lower esophageal sphincter of achalasia patients was significantly reduced (χ 2 = 11.429, P = 0.010). The expression of cystathionine-β-synthase and cystathionine-γ-lyase were lower in lower esophageal sphincter than that in 2 cm and 4 cm above lower esophageal sphincter, respectively (all P achalasia, which implicates the involvement of the two hydrogen sulfide synthesis enzymes in the pathophysiology of achalasia. © 2015 International Society for Diseases of the Esophagus.

Gait analysis of patients with an off-the-shelf total knee replacement versus customized bi-compartmental knee replacement.

Science.gov (United States)

Wang, Henry; Foster, Jonathan; Franksen, Natasha; Estes, Jill; Rolston, Lindsey

2018-04-01

Newer TKR designs have been introduced to the market with the aim of overcoming common sizing problems with older TKR designs. Furthermore, since a sizable percentage of patients with OA present with disease limited to the medial/lateral knee compartment in addition to the patellofemoral joint, for whom, a customized bi-compartmental knee replacement (BKR) is available as a treatment option. To date, there is very little information regarding knee strength and mechanics during gait for patients implanted with these modern TKR and BKR designs. The purpose of the study was to evaluate knee strength and mechanics during walking for patients with either a modern off the shelf TKR or a customized BKR and compare these findings to a cohort of healthy controls. Twelve healthy controls, eight BKR, and nine TKR patients participated in the study. Maximal isometric knee strength was evaluated. 3D kinematic and kinetic analyses were conducted for level walking. The TKR knee exhibited less peak extensor torque when compared to, both the BKR and control limbs (p < 0.05). The TKR knee had less extensor moment at stance than both the BKR and control knees (p < 0.05). Both the BKR and control knees displayed larger internal rotation at stance than that of the TKR knee (p < 0.05). This study suggests that, for patients that exhibit isolated OA of the tibiofemoral joint, using a customized BKR implant is a viable treatment option and may contribute to superior mechanical advantages.

Cost feasibility of a pre-checking medical tourism system for U.S. patients undertaking joint replacement surgery in Taiwan.

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Haung, Ching-Ying; Wang, Sheng-Pen; Chiang, Chih-Wei

2010-01-01

Medical tourism is a relatively recent global economic and political phenomenon that has assumed increasing importance for developing countries, particularly in Asia. In fact, Taiwan possesses a niche for developing medical tourism because many hospitals provide state-of-the-art medicine in all disciplines and many doctors are trained in the United States (US). Among the most common medical procedures outsourced, joint replacements such as total knee replacement (TKR) and total hip replacement (THR) are two surgeries offered to US patients at a lower cost and shorter waiting time than in the US. This paper proposed a pre-checking medical tourism system (PCMTS) and evaluated the cost feasibility of recruiting American clients traveling to Taiwan for joint replacement surgery. Cost analysis was used to estimate the prime costs for each stage in the proposed PCMTS. Sensitivity analysis was implemented to examine how different pricings for medical checking and a surgical operation (MC&SO) and recovery, can influence the surplus per patient considering the PCMTS. Finally, the break-even method was adopted to test the tradeoff between the sunk costs of investment in the PCMTS and the annual surplus for participating hospitals. A novel business plan was built showing that pre-checking stations in medical tourism can provide post-operative care and recovery follow-up. Adjustable pricing for hospital administrators engaged in the PCMTS consisted of two main costs: US$3,700 for MC&SO and US$120 for the hospital stay. Guidelines for pricing were provided to maximize the annual surplus from this plan with different number of patients participating in PCMTS. The maximal profit margin from each American patient undertaking joint surgery is about US$24,315. Using cost analysis, this article might be the first to evaluate the feasibility of PCMTS for joint replacement surgeries. The research framework in this article is applicable when hospital administrators evaluate the

Shift from intravenous or 16% subcutaneous replacement therapy to 20% subcutaneous immunoglobulin in patients with primary antibody deficiencies.

Science.gov (United States)

Canessa, Clementina; Iacopelli, Jessica; Pecoraro, Antonio; Spadaro, Giuseppe; Matucci, Andrea; Milito, Cinzia; Vultaggio, Alessandra; Agostini, Carlo; Cinetto, Francesco; Danieli, Maria Giovanna; Gambini, Simona; Marasco, Carolina; Trizzino, Antonino; Vacca, Angelo; De Mattia, Domenico; Martire, Baldassarre; Plebani, Alessandro; Di Gioacchino, Mario; Gatta, Alessia; Finocchi, Andrea; Licciardi, Francesco; Martino, Silvana; De Carli, Marco; Moschese, Viviana; Azzari, Chiara

2017-03-01

In patients with primary antibody deficiencies, subcutaneous administration of IgG (SCIG) replacement is effective, safe, well-tolerated, and can be self-administered at home. A new SCIG replacement at 20% concentration (Hizentra ® ) has been developed and has replaced Vivaglobin ® (SCIG 16%). An observational prospective multi-centric open-label study, with retrospective comparison was conducted in 15 Italian centers, in order to investigate whether and to what extent switching to Hizentra ® would affect frequency of infusions, number of infusion sites, patients' satisfaction, and tolerability in patients previously treated with Vivaglobin ® or intravenous immunoglobulins (IVIG). Any variations of dosage, frequency and duration of the infusions, and of number of infusion sites induced by Hizentra ® with respect to the former treatment were recorded. Practical advantages and disadvantages of Hizentra ® , with respect to the medicinal product formerly used, and the variations in patients' therapy-related satisfaction were monitored by means of the TSQM (Treatment Satisfaction Questionnaire for Medication); number, frequency, and duration of infectious events and adverse effects were recorded. Eighty-two patients switched to Hizentra ® : 19 (23.2%) from IVIG and 63 (76.8%) from Vivaglobin ® . The mean interval between infusions was not affected by the shift (7.0 ± 2.0 days with previous treatment versus 7.1 ± 1.2 during Hizentra ® ). A decrease in the number of infusion sites with Hizentra ® was recorded in 12 out of 56 patients for whom these data were available. At 6 months, 89.7% of patients were satisfied with Hizentra ® ; no difference in terms of effectiveness, side effects, convenience, and global satisfaction was observed. No difference in the incidence of adverse events was reported.

Intra-operative Vector Flow Imaging Using Ultrasound of the Ascending Aorta among 40 Patients with Normal, Stenotic and Replaced Aortic Valves

DEFF Research Database (Denmark)

Hansen, Kristoffer Lindskov; Møller-Sørensen, Hasse; Kjaergaard, Jesper

2016-01-01

Stenosis of the aortic valve gives rise to more complex blood flows with increased velocities. The angleindependent vector flow ultrasound technique transverse oscillation was employed intra-operatively on the ascending aorta of (I) 20 patients with a healthy aortic valve and 20 patients with aor...... replacement corrects some of these changes. Transverse oscillation may be useful for assessment of aortic stenosis and optimization of valve surgery. (E-mail: lindskov@gmail.com) 2016 World Federation for Ultrasound in Medicine & Biology...... with aortic stenosis before (IIa) and after (IIb) valve replacement. The results indicate that aortic stenosis increased flow complexity (p , 0.0001), induced systolic backflow (p , 0.003) and reduced systolic jet width (p , 0.0001). After valve replacement, the systolic backflow and jet width were normalized...

Microbial keratinases: industrial enzymes with waste management potential.

Science.gov (United States)

Verma, Amit; Singh, Hukum; Anwar, Shahbaz; Chattopadhyay, Anirudha; Tiwari, Kapil K; Kaur, Surinder; Dhilon, Gurpreet Singh

2017-06-01

Proteases are ubiquitous enzymes that occur in various biological systems ranging from microorganisms to higher organisms. Microbial proteases are largely utilized in various established industrial processes. Despite their numerous industrial applications, they are not efficient in hydrolysis of recalcitrant, protein-rich keratinous wastes which result in environmental pollution and health hazards. This paved the way for the search of keratinolytic microorganisms having the ability to hydrolyze "hard to degrade" keratinous wastes. This new class of proteases is known as "keratinases". Due to their specificity, keratinases have an advantage over normal proteases and have replaced them in many industrial applications, such as nematicidal agents, nitrogenous fertilizer production from keratinous waste, animal feed and biofuel production. Keratinases have also replaced the normal proteases in the leather industry and detergent additive application due to their better performance. They have also been proved efficient in prion protein degradation. Above all, one of the major hurdles of enzyme industrial applications (cost effective production) can be achieved by using keratinous waste biomass, such as chicken feathers and hairs as fermentation substrate. Use of these low cost waste materials serves dual purposes: to reduce the fermentation cost for enzyme production as well as reducing the environmental waste load. The advent of keratinases has given new direction for waste management with industrial applications giving rise to green technology for sustainable development.

Increased risk of revision for infection in rheumatoid arthritis patients with total hip replacements

DEFF Research Database (Denmark)

Schrama, Johannes Cornelis; Fenstad, Anne M; Dale, Håvard

2015-01-01

Background and purpose-Medical treatment of rheumatoid arthritis (RA) has changed dramatically over the last 15 years, including immune modulation. We investigated the risk of revision for infection after primary total hip replacement (THR) in patients with rheumatoid arthritis over a 16-year...... period, and compared it with that in THR patients with osteoarthritis (OA).Patients and methods-We identified 13,384 THRs in RA patients and 377,287 THRs in OA patients from 1995 through 2010 in a dataset from the Nordic Arthroplasty Register Association (NARA). Kaplan-Meier survival curves......, with revision for infection as the endpoint, were constructed. Cox regression analyses were performed to calculate the relative risk (RR) of revision for infection adjusted for age, sex, fixation technique, and year of primary surgery.Results-RA patients had a 1.3 times (95% CI 1.0-1.6) higher risk of revision...

Effects of early changes in organ dysfunctions on the outcomes of critically ill patients in need of renal replacement therapy

Directory of Open Access Journals (Sweden)

Elizabeth Maccariello

2008-01-01

Full Text Available INTRODUCTION: Acute kidney injury usually develops in critically ill patients in the context of multiple organ dysfunctions. OBJECTIVE: To evaluate the effect of changes in associated organ dysfunctions over the first three days of renal replacement therapy on the outcomes of patients with acute kidney injury. METHODS: Over a 19-month period, we evaluated 260 patients admitted to the intensive care units of three tertiary-care hospitals who required renal replacement therapy for > 48 h. Organ dysfunctions were evaluated by SOFA score (excluding renal points on the first (D1 and third (D3 days of renal replacement therapy. Absolute (A-SOFA and relative (D-SOFA changes in SOFA scores were also calculated. RESULTS: Hospital mortality rate was 75%. Organ dysfunctions worsened (A-SOFA>0 in 53%, remained unchanged (A-SOFA=0 in 17% and improved (A-SOFA<0 in 30% of patients; and mortality was lower in the last group (80% vs. 84% vs. 61%, p=0.003. SOFA on D1 (p<0.001, SOFA on D3 (p<0.001, A-SOFA (p=0.019 and D-SOFA (p=0.016 were higher in non-survivors. However, neither A-SOFA nor D-SOFA discriminated survivors from non-survivors on an individual basis. Adjusting for other covariates (including SOFA on D1, A-SOFA and D-SOFA were associated with increased mortality, and patients in whom SOFA scores worsened or remained unchanged had poorer outcomes. CONCLUSIONS: In addition to baseline values, early changes in SOFA score after the start of renal replacement therapy were associated with hospital mortality. However, no prognostic score should be used as the only parameter to predict individual outcomes.

Special features of total knee replacement in hemophilia.

Science.gov (United States)

Rodriguez-Merchan, Emerito Carlos

2013-12-01

Total knee replacement is an operation frequently needed by hemophilia patients, which greatly improves their quality of life. This operation, however, carries a higher risk of bleeding and infection for hemophiliacs than it does for osteoarthritis sufferers. It is advisable to implant prosthetic components using antibiotic-loaded cement. It is essential to maintain a level of 100% of the replacement clotting factor for 2 weeks. Hematological treatment must be established, depending on the patient's factor levels and other pharmacokinetic parameters such as recovery and half-life, optimal doses and treatment time. It is preferable to use general anesthesia due to the risk of spinal bleeding. The lifespan of total knee replacement in hemophilic patients is shorter than in patients with osteoarthritis because of the increased risk of infection.

Asymptomatic and persistent elevation of pancreatic enzymes in an ulcerative colitis patient.

Science.gov (United States)

Liverani, Elisa; Leonardi, Filippo; Castellani, Lucia; Cardamone, Carla; Belluzzi, Andrea

2013-01-01

Azathioprine has been extensively used in the management of inflammatory bowel diseases. It might cause pancreatic damage in the form of either asymptomatic elevation in serum amylase/lipase or overt acute pancreatitis. Here we report the case of a 61-year-old patient with ulcerative colitis who had been treated with azathioprine for three years, achieving clinical remission. During treatment he presented an asymptomatic elevation of serum pancreatic enzymes, without any signs of pancreatitis at imaging. This evidence brought us to reassess the drug dosage, without achieving a normalization of biochemical analysis. Autoimmune pancreatitis was excluded. One year after the suspension of azathioprine, we still face persistent high levels of amylase/lipase. Normalization of enzymatic values in patients who develop intolerance to azathioprine, in the form of either asymptomatic elevation in serum amylase/lipase or overt acute pancreatitis, is usually achieved in about two months after stopping drug intake. Asymptomatic elevation in serum pancreatic enzymes in the absence of pancreatic disease is reported in the literature and defined as "Gullo's syndrome," but nobody of the subjects studied had been treated in the past with pancreatotoxic drugs. Might this case be defined as "benign pancreatic hyperenzymemia"?

Results of Austin Moore replacement.

Directory of Open Access Journals (Sweden)

Jadhav A

1996-04-01

Full Text Available Forty cases of Austin Moore Replacement done for transcervical fractures of the femur in patients were reviewed after a period of 12 to 48 months postoperatively (mean 26 mth. 30 cases (75% had mild to severe pain of non-infective origin, starting as early as 6 months postoperatively. This was irrespective of the make, size or position (varus/valgus of the prosthesis. Though the Aufranc and Sweet clinical scoring was satisfactory in 65% cases, radiological evidence of complications like sinking, protrusion, etc. were seen in majority of the cases. Calcar resorption was seen in 34 cases (85% as early as 4 months postoperatively. Results of THR and bipolar replacement done for transcervical fractures in recent literature show 85% pain-free cases at 5 years. We feel that Austin Moore Replacement should be reserved for patients more than 65 years of age and those who are less active or debilitated because of other factors, because of increased acetabular wear with time in the younger individual. This is corroborated by unsatisfactory results in patients less than 65 years of age (p < 0.05.

Heart valve replacement with the Sorin tilting-disc prosthesis. A 10-year experience.

Science.gov (United States)

Milano, A; Bortolotti, U; Mazzucco, A; Mossuto, E; Testolin, L; Thiene, G; Gallucci, V

1992-02-01

From 1978 to 1988, 697 patients with a mean age of 48 +/- 11 years (range 5 to 75 years) received a Sorin tilting-disc prosthesis; 358 had had aortic valve replacement, 247 mitral valve replacement, and 92 mitral and aortic valve replacement. Operative mortality rates were 7.8%, 11.3%, and 10.8%, respectively, in the three groups. Cumulative duration of follow-up is 1650 patient-years for aortic valve replacement (maximum follow-up 11.4 years), 963 patient-years for mitral valve replacement (maximum follow-up 9.9 years) and 328 patient-years for mitral and aortic valve replacement (maximum follow-up 9.4 years). Actuarial survival at 9 years is 72% +/- 4% after mitral valve replacement, 70% +/- 3% after aortic valve replacement, and 50% +/- 12% after mitral and aortic valve replacement, and actuarial freedom from valve-related deaths is 97% +/- 2% after mitral valve replacement, 92% +/- 2% after aortic valve replacement, and 62% +/- 15% after mitral and aortic valve replacement. Thromboembolic events occurred in 21 patients with aortic valve replacement (1.3% +/- 0.2%/pt-yr), in 12 with mitral valve replacement (1.2% +/- 0.3% pt-yr), and in seven with mitral and aortic valve replacement (2.1% +/- 0.8%), with one case of prosthetic thrombosis in each group; actuarial freedom from thromboembolism at 9 years is 92% +/- 3% after mitral valve replacement, 91% +/- 3% after aortic valve replacement, and 74% +/- 16% after mitral and aortic valve replacement. Anticoagulant-related hemorrhage was observed in 15 patients after aortic valve replacement (0.9% +/- 0.2%/pt-yr), in 9 after mitral valve replacement (0.9% +/- 0.3%/pt-yr), and in 6 with mitral and aortic valve replacement (0.9% +/- 0.5%/pt-yr); actuarial freedom from this complication at 9 years is 94% +/- 2% after aortic valve replacement, 91% +/- 4% after mitral valve replacement, and 68% +/- 16% after mitral and aortic valve replacement. Actuarial freedom from reoperation at 9 years is 97% +/- 2% after mitral and

A 5 year prospective study of patient-relevant outcomes after total knee replacement

DEFF Research Database (Denmark)

Nilsdotter, A-K; Toksvig-Larsen, S; Roos, E M

2008-01-01

men, mean age 71 (51-86) assigned for TKR at the Department of Orthopaedics at Lund University Hospital were included in the study. The self-administered questionnaires Knee injury and Osteoarthritis Outcome Score (KOOS) and SF-36 were mailed preoperatively and 6 months, 12 months and at 5 years......OBJECTIVE: To prospectively describe self-reported outcomes up to 5 years after total knee replacement (TKR) in Osteoarthritis (OA) and to study which patient-relevant factors may predict outcomes for pain and physical function (PF). METHODS: 102 consecutive patients with knee OA, 63 women and 39...... postoperatively. RESULTS: Response rate at 5 years was 86%. At 6 months significant improvement was seen in all KOOS and SF-36 scores (P

Usability Test of Exercise Games Designed for Rehabilitation of Elderly Patients After Hip Replacement Surgery: Pilot Study

NARCIS (Netherlands)

Y. Ling (Yun); L.P.D.M. ter Meer (Louis); Z. Yumak (Zerrin); R.C. Veltkamp (Remco)

2017-01-01

markdownabstractBACKGROUND: Patients who receive rehabilitation after hip replacement surgery are shown to have increased muscle strength and better functional performance. However, traditional physiotherapy is often tedious and leads to poor adherence. Exercise games, provide ways for increasing

Flucytosine Pharmacokinetics in a Critically Ill Patient Receiving Continuous Renal Replacement Therapy.

Science.gov (United States)

Kunka, Megan E; Cady, Elizabeth A; Woo, Heejung C; Thompson Bastin, Melissa L

2015-01-01

Purpose. A case report evaluating flucytosine dosing in a critically ill patient receiving continuous renal replacement therapy. Summary. This case report outlines an 81-year-old male who was receiving continuous venovenous hemofiltration (CVVH) for acute renal failure and was being treated with flucytosine for the treatment of disseminated Cryptococcus neoformans infection. Due to patient specific factors, flucytosine was empirically dose adjusted approximately 50% lower than intermittent hemodialysis (iHD) recommendations and approximately 33% lower than CRRT recommendations. Peak and trough levels were obtained, which were supratherapeutic, and pharmacokinetic parameters were calculated. The patient experienced thrombocytopenia, likely due to elevated flucytosine levels, and flucytosine was ultimately discontinued. Conclusion. Despite conservative flucytosine dosing for a patient receiving CVVH, peak and trough serum flucytosine levels were supratherapeutic (120 μg/mL at 2 hours and 81 μg/mL at 11.5 hours), which increased drug-related adverse effects. The results indicate that this conservative dosing regimen utilizing the patient's actual body weight was too aggressive. This case report provides insight into flucytosine dosing in CVVH, a topic that has not been investigated previously. Further pharmacokinetic studies of flucytosine dosing in critically ill patients receiving CVVH are needed in order to optimize pharmacokinetic and pharmacodynamic parameters while avoiding toxic flucytosine exposure.

Nursing procedures during continuous renal replacement therapies: a national survey.

Science.gov (United States)

Ricci, Zaccaria; Benelli, Sonia; Barbarigo, Fabio; Cocozza, Giulia; Pettinelli, Noemi; Di Luca, Emanuela; Mettifogo, Mariangela; Toniolo, Andrea; Ronco, Claudio

2015-01-01

The current role of nurses in the management of critically ill patients needing continuous renal replacement therapies is clearly fundamental. The care of these complex patients is typically shared by critical care and dialysis nurses: their precise duties may vary from country to country. To clarify this issue we conducted a national-level survey at a recent Italian course on nursing practices during continuous renal replacement therapies. A total of 119 questionnaires were analysed. The participants, who were equally divided between critical care and dialysis nurses, came from 44 different hospitals and 35 Italian cities. Overall, 23% of participants answered that "the dialysis staff" were responsible for continuous renal replacement therapies in the Intensive Care Unit, while 39% answered "the critical care nurse", and 38% "a shared organization". Interestingly, less than the half of participants claimed specific continuous renal replacement therapies training was provided to employees before handling an acute dialysis machine. Finally, about 60% of participants had experience of extra-corporeal membrane oxygenation machines used in conjunction with continuous renal replacement therapies. Workload coordination and management of critically ill patients undergoing continuous renal replacement therapies in Italy is not standardized. At present, the duties of critical care and dialysis nurses vary significantly across the country. They frequently overlap or leave gaps in the assistance received by patients. The role of nurses involved in the care of continuous renal replacement therapies patients in Italy currently requires better organization, possibly starting with intensive standardized training and educational programs.

Patients' journeys through total joint replacement: patterns of medication use.

Science.gov (United States)

Johnson, Emma C; Horwood, Jeremy; Gooberman-Hill, Rachael

2014-06-01

Medication is used to manage pain that results from both osteoarthritis and total joint replacement (TJR). Research has provided insight into how people living with osteoarthritis use pain relief medication. However, it is not known whether elective TJR affects existing attitudes and behaviours with regard to pain medications. Using qualitative methods, the present study explored patterns of pain relief use around the time of TJR. In-depth face-to-face qualitative interviews were carried out with 24 patients two to four weeks after they had undergone TJR for hip or knee osteoarthritis. Participants were asked to reflect on their use of pain medication pre-surgery, while in hospital and while recovering from their operation at home. Transcripts of the audio-recorded interviews were imported into Atlas.ti® and thematic analysis was used. Attitudes to pain relief medication and their use are not static. Many participants change their use of pain medication around the time of surgery. This shift was influenced by interactions with health professionals and changing views on the acceptability, necessity and value of pain relief in helping to manage an altered pain experience. Understanding reasons for medication-taking behaviour during the journey through joint replacement may be helpful to health professionals. Health professionals have a fundamental role to play in challenging or reinforcing different treatment beliefs, which is the basis for effective use of pain relief over the pre- to postoperative period. © 2013 John Wiley & Sons, Ltd.

Aortic valve replacement with sutureless prosthesis: better than root enlargement to avoid patient-prosthesis mismatch?

Science.gov (United States)

Beckmann, Erik; Martens, Andreas; Alhadi, Firas; Hoeffler, Klaus; Umminger, Julia; Kaufeld, Tim; Sarikouch, Samir; Koigeldiev, Nurbol; Cebotari, Serghei; Schmitto, Jan Dieter; Haverich, Axel; Shrestha, Malakh

2016-06-01

Aortic valve replacement in patients with a small aortic annulus may result in patient-prosthesis mismatch (PPM). Aortic root enlargement (ARE) can reduce PPM, but leads to extended cardiac ischaemia times. Sutureless valves have the potential to prevent PPM while reducing cardiac ischaemia times. Between January 2007 and December 2011, a total of 128 patients with a small aortic annulus underwent surgery for aortic valve stenosis at our centre. Thirty-six (17% male, n = 6) patients received conventional valve replacement with ARE and 92 (16% male, n = 18) subjects received sutureless valve implantation (Sorin Perceval). We conducted a comparative, retrospective study with follow-up. The sutureless group showed a significantly higher age (79 years) than the ARE patients (62 years, P body surface area was 0.91 ± 0.2 cm(2)/m(2) in ARE patients and 0.83 ± 0.14 cm(2)/m(2) in sutureless patients (P = 0.040). The rate of patients with severe PPM was 6% (n = 2) in ARE patients and 11% (n = 8%) in sutureless patients (not significant, n.s.). The 30-day mortality rates were 2% (n = 2) in sutureless patients and 6% (n = 2) in ARE patients (n.s.). The 1- and 5-year survival rates of the sutureless group were 92 and 54% years, respectively, whereas the 1- and 5-year survival rates of the ARE group were 76% (n.s.). Although the sutureless valve patients received significantly more concomitant procedures, all operation-associated times were significantly shorter. Despite sutureless valve patients being older, the 30-day mortality and survival rates were comparable in the two groups. Since the indexed EOA was only slightly lower and the incidence of severe PPM was not significantly higher in the sutureless valve patients, we conclude that sutureless valve implantation is an alternative to conventional ARE to treat a small aortic annulus and avoid PPM, especially in geriatric patients who benefit from the quick implantation process. © The Author 2016. Published by Oxford

Enzyme alterations in mediastine during and after radiotherapy. 2

International Nuclear Information System (INIS)

Alheit, H.D.; Alheit, C.; Herrmann, T.

1986-01-01

Results are presented estimating the serum activity of transaminases (ASAT and ALAT) in 72 patients after mediastinal irradiation. During and after mediastinal irradiation both enzymes showed essentially a parallel reaction. One day after irradiation a decrease of enzymes in patients who were irradiated with high single dosis (5 Gy) was observed, while patients irradiated with low or middle single dosis showed an increase of enzyme activity. A different temporal enzyme reaction is discussed to be the cause for this reaction in dependence on the applied single dose so that in patients with high single doses an initial enzyme increase caused by the radiation insult has changed into a following decrease under the starting level at the first control 24 hours later. Because patients without mediastinal tumors react in the same manner, the normal tissue surrounding the tumor is discussed to be the original place of enzyme secretion. Up to the end of irradiation a decrease of enzymes was observed in patients with high single dose or with high total dose (60 Gy) which is interpreted as an enzyme deficiency in tissue in consequence of destruction in formation places. In patients with middle total and low single doses an enzyme increase is registered with a still sufficient restoration capacity of the tissue discussed to be the cause of it. An enzyme increase, observed from the end of irradiation to the control date 3 to 6 months after irradiation, is mainly caused by a tumor progression (increased rate of liver metastases, especially in bronchial carcinoma) and can still be intensified by occurrence of pulmonal or cardiac radioreactions. (author)

The importance of patient expectations as a determinant of satisfaction with waiting times for hip and knee replacement surgery.

Science.gov (United States)

Conner-Spady, Barbara L; Sanmartin, Claudia; Johnston, Geoffrey H; McGurran, John J; Kehler, Melissa; Noseworthy, Tom W

2011-08-01

The disconfirmation model hypothesizes that satisfaction is a function of a perceived discrepancy from an initial expectation. Our objectives were: (1) to test the disconfirmation model as it applies to patient satisfaction with waiting time (WT) and (2) to build an explanatory model of the determinants of satisfaction with WT for hip and knee replacement. We mailed 1000 questionnaires to 2 random samples: patients waiting or those who had received a joint replacement within the preceding 3-12 months. We used ordinal logistic regression analysis to build an explanatory model of the determinants of satisfaction. Of the 1330 returned surveys, 1240 contained patient satisfaction data. The sample was 57% female; mean age was 70 years (SD 11). Consistent with the disconfirmation model, when their WTs were longer than expected, both waiting (OR 5.77, 95% CI 3.57-9.32) and post-surgery patients (OR 6.57, 95% CI 4.21-10.26) had greater odds of dissatisfaction, adjusting for the other variables in the model. Compared to those who waited 3 months or less, post-surgery patients who waited 6 to 12 months (OR 2.59, 95% CI 1.27-5.27) and over 12 months (OR 3.30, 95% CI 1.65-6.58) had greater odds of being dissatisfied with their waiting time. Patients who felt they were treated unfairly had greater odds of being dissatisfied (OR 4.74, 95% CI 2.60-8.62). In patients on waiting lists and post-surgery for hip and knee replacement, satisfaction with waiting times is related to fulfillment of expectations about waiting, as well as a perception of fairness. Measures to modify expectations and increase perceived fairness, such as informing patients of a realistic WT and communication during the waiting period, may increase satisfaction with WTs. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

16-Detector row computed tomographic coronary angiography in patients undergoing evaluation for aortic valve replacement: comparison with catheter angiography

International Nuclear Information System (INIS)

Manghat, N.E.; Morgan-Hughes, G.J.; Broadley, A.J.; Undy, M.B.; Wright, D.; Marshall, A.J.; Roobottom, C.A.

2006-01-01

Aim: To evaluate the diagnostic accuracy of 16-detector row computed tomography (CT) in assessing haemodynamically significant coronary artery stenoses in patients under evaluation for aortic stenosis pre-aortic valve replacement. Subjects and methods: Forty consecutive patients under evaluation for severe aortic stenosis and listed for cardiac catheterization before potential aortic valve replacement underwent coronary artery calcium (CAC) scoring and retrospective electrocardiogram (ECG)-gated multi-detector row computed tomographic coronary angiography (MDCTA) using a GE Lightspeed 16-detector row CT within 1 month of invasive coronary angiography (ICA) for comparative purposes. All 13 major coronary artery segments of the American Heart Association model were evaluated for the presence of ≥50% stenosis and compared to the reference standard. Data were analysed on a segment-by-segment basis and also in 'whole patient' terms. Results: A total of 412/450 segments from 35 patients were suitable for analysis. The overall accuracy of MDCTA for detection of segments with ≥50% stenosis was high, with a sensitivity of 81.3%, specificity 95.0%, positive predictive value (PPV) 57.8%, and negative predictive value (NPV) 98.4%. On a 'whole-patient' basis, 100% (19/19) of patients with significant coronary disease were correctly identified and there were no false-negatives. Excluding patients with CAC >1000 from the analysis improved the accuracy of MDCTA to: sensitivity 90%, specificity 98.1%, PPV 60%, NPV 99.7%. Conclusion: Non-invasive 16-detector row MDCTA accurately excludes significant coronary disease in patients with severe aortic stenosis undergoing evaluation before aortic valve replacement and in whom ICA can therefore be avoided. Its segment-by-segment accuracy is improved further if CAC > 1000 is used as a gatekeeper to MDCTA

Comparison of aortic root replacement in patients with Marfan syndrome.

Science.gov (United States)

Bernhardt, Alexander M J; Treede, Hendrik; Rybczynski, Meike; Sheikzadeh, Sara; Kersten, Jan F; Meinertz, Thomas; von Kodolitsch, Yskert; Reichenspurner, Hermann

2011-11-01

Although the aortic-valve-sparing (AVS) reimplantation technique according to David has shown favorable durability results in mid-term and long-term studies, composite valve grafting (CVG) according to Bentall is still considered the standard procedure. Retrospectively, we evaluated the results of aortic root replacement of patients with Marfan syndrome (MFS) who underwent surgery between January 1995 and January 2010. MFS was diagnosed using the Ghent criteria. AVS was used in 58 patients and CVG in 30 patients with MFS. AVS was done for aortic-root aneurysm (n=48) or aortic dissection type A (n=10). CVG was used for aortic-root aneurysm in 14 patients or aortic dissection type A in 16 patients. The mean follow-up was 3.2 (95% CI: 2.4-4.2) years. In both groups, 30-day mortality was 0%. Three patients (10.0%) in the CVG group required resternotomy for postoperative bleeding versus two patients (3.4%) in the AVS group (p=0.3). At follow-up, mortality was 10% in the CVG group versus 3.4% in the AVS group (p=0.3). Re-operation was required in two patients (3.4%) after AVS and in three patients after CVG (10%) (p=0.3). Three patients (10.0%) who underwent CVG had endocarditis and two patients (6.7%) had a stroke during follow-up, whereas no endocarditis and stroke occurred after AVS. After 14 years, stratified event-free survival was better in the AVS group (event-free survival was 82.3% vs 58.6%, log-rank test p=0.086), especially after aneurysm (p=0.057). After 10 years, freedom from aortic regurgitation ≥II° in the AVS group was 80% for aneurysm and 50% after dissection (p=0.524). The reimplantation technique according to David was associated with excellent survival, good valve function and a low rate of re-operation, endocarditis, and stroke. There was a trend to better event-free survival for AVS patients making it the procedure of choice in MFS patients. Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights

Continuous renal replacement therapy for acute renal failure in patients with cancer: a well-tolerated adjunct treatment

Directory of Open Access Journals (Sweden)

Rebecca Fischler

2016-08-01

Full Text Available Abstract Introduction – Acute renal failure (ARF has a poor prognosis in patients with cancer requiring intensive care unit (ICU admission. Our aim is finding prognostic factors for hospital mortality in patients with cancer with ARF requiring renal replacement therapy (RRT. Methods – In this retrospective study, all patients with cancer with ARF treated with continuous venovenous filtration (CVVHDF in the ICU of the Institut Jules Bordet, between January 1st 2003 and December 31st 2012, were included in the study.Results – 103 patients are assessed: men/women 69/34, median age 62 years, solid/haematologic tumours 68/35, median SAPS II 56. Mortality rate was 63%. Seven patients required chronic renal dialysis. After multivariate analysis, two variables were statistically associated with hospital mortality : more than one organ failure (including kidney (OR 5.918 ; 95% CI 2.184 – 16.038 ; p<0,001 and low albumin level (OR 3.341; 95% CI 1.229 – 9.077; p=0,02. Only minor complications related to CVVHDF have been documented.Conclusions – Despite the poor prognosis associated with ARF, CVVHDF is an effective and tolerable renal replacement technique in patients with cancer admitted to the ICU. Multiple organ failure and hypoalbuminemia, two independent prognostic factors for hospital mortality have to be considered when deciding for introducing RRT.

Perioperative plasmatic presepsin levels in patients undergoing total hip or knee replacement: a preliminary study.

Science.gov (United States)

Vicenti, G; Pesce, V; Bizzoca, D; Nappi, V; Palmiotto, F; Carrozzo, M; Moretti, B

2017-01-01

Presepsin (sCD14-ST) is an emerging biomarker in the diagnosis of sepsis. In the field of orthopaedics, it could be useful in the diagnosis and management of periprosthetic joint infections (PJI). The aim of this study is to define the normal perioperative plasmatic levels of presepsin in patients undergoing primary cementless total hip replacement (THR) or primary cemented total knee replacement (TKR). For this purpose, 50 patients (19 male, 31 female, mean age= 64.04±8.88) were recruited. The patients were divided into two groups: Group A patients underwent cementless THR, whereas Group B patients underwent cemented TKR. On recruitment, anthropometric data, smocking status, osteoarthritis stage according to Kellgren and Lawrence, Harris Hip Score (HHS) for Group A patients and Knee Society Score (KSS) for Group B patients, drugs assumption and comorbidities were recorded. All the patients underwent serial blood tests, including complete blood count, presepsin (PS), C-reactive protein (CRP) and procalcitonin (PCT) 24 hours before arthroplasty (T0) and at 24 (T1), 48 (T2), 72 (T3) and 96 (T4) hours postoperatively. Body temperature (θ) was recorded every six hours in the time lapse T0-T4. Presepsin plasmatic concentration was comparable at baseline in both groups. After surgery, however, a significant increase of presepsin was observed in Group A, whereas in Group B no significant changes of presepsin were recorded. A comparable trend of this biomarker was found in the two groups, i.e. presepsin increased from T0 to T3, when it reached its maximum value, and its decrease started at T4. Finally, presepsin resulted more accurate than CRP in the evaluation of perioperative inflammatory response in patients undergoing THR or TKR. These data will be helpful in defining a reference interval for presepsin in patients with prosthetic joint implants, and a cut-off of this biomarker for the diagnosis of PJI.

High titers of autoantibodies to glutamate decarboxylase in Type 1 Diabetes Patients: Epitope Analysis and Inhibition of Enzyme Activity

Science.gov (United States)

Hampe, Christiane S.; Maitland, Murray E.; Gilliam, Lisa K.; Thi Phan, Thanh-H.; Sweet, Ian R.; Radtke, Jared R.; Bota, Vasile; Ransom, Bruce R.; Hirsch, Irl B.

2014-01-01

Objective Autoantibodies to glutamate decarboxylase (GAD65Ab) are found in patients with autoimmune neurological disorders and patients with type 1 diabetes. The correct diagnosis of GAD65Ab-associated neurological disorders is often delayed by the variability of symptoms and a lack of diagnostic markers. We hypothesize that the frequency of neurological disorders with high GAD65Ab titers is significantly higher than currently recognized. Methods We analyzed GAD65Ab titer, inhibition of GAD65 enzyme activity, and pattern of GAD65Ab epitopes in a cohort of type 1 diabetes patients (n=100) and correlated our findings with neurological symptoms and diseases. Results Fourty-three percent (43/100) of the patients had detectable GAD65Ab titers (median=400 U/ml, range: 142–250,000U/ml). The GAD65Ab titers in 10 type 1 diabetes patients exceeded the 90th percentile of the cohort (2,000–250,000 U/ml). Sera of these 10 patients were analyzed for their GAD65Ab epitope specificity and their ability to inhibit GAD65 enzyme activity in vitro. GAD65Ab of five patients inhibited the enzyme activity significantly (by 34–55%). Three of these patients complained of muscle stiffness and pain, which was documented in two of these patients. Conclusions Based on our findings we suggest that neurological disorders with high GAD65Ab titers are more frequent in type 1 diabetes patients than currently recognized. PMID:23512385

The safety of peri-articular local anaesthetic injection for patients undergoing total knee replacement with autologous blood transfusion: a randomised trial.

Science.gov (United States)

Wallace, D F; Emmett, S R; Kang, K K; Chahal, G S; Hiskens, R; Balasubramanian, S; McGuinness, K; Parsons, H; Achten, J; Costa, M L

2012-12-01

Intra-operative, peri-articular injection of local anaesthesia is an increasingly popular way of controlling pain following total knee replacement. At the same time, the problems associated with allogenic blood transfusion have led to interest in alternative methods for managing blood loss after total knee replacement, including the use of auto-transfusion of fluid from the patient's surgical drain. It is safe to combine peri-articular infiltration with auto-transfusion from the drain. We performed a randomised clinical trial to compare the concentration of local anaesthetic in the blood and in the fluid collected in the knee drain in patients having either a peri-articular injection or a femoral nerve block. Clinically relevant concentrations of local anaesthetic were found in the fluid from the drains of patients having peri-articular injections (4.92 μg/ml (sd 3.151)). However, none of the patients having femoral nerve blockade had detectable levels. None of the patients in either group had clinically relevant concentrations of local anaesthetic in their blood after re-transfusion. The evidence from this study suggests that it is safe to use peri-articular injection in combination with auto-transfusion of blood from peri-articular drains during knee replacement surgery.

Vascular complications associated with transcatheter aortic valve replacement.

Science.gov (United States)

Sardar, M Rizwan; Goldsweig, Andrew M; Abbott, J Dawn; Sharaf, Barry L; Gordon, Paul C; Ehsan, Afshin; Aronow, Herbert D

2017-06-01

Transcatheter aortic valve replacement (TAVR) is now an accepted pathway for aortic valve replacement for patients who are at prohibitive, severe and intermediate risk for traditional aortic valve surgery. However, with this rising uptrend and adaptation of this new technology, vascular complications and their management remain an Achilles heel for percutaneous aortic valve replacement. The vascular complications are an independent predictor of mortality for patients undergoing TAVR. Early recognition of these complications and appropriate management is paramount. In this article, we review the most commonly encountered vascular complications associated with currently approved TAVR devices and their optimal percutaneous management techniques.

Joint replacement in Zambia: A review of Hip & Knee Replacement ...

African Journals Online (AJOL)

Methods: Data captured by the different variables entered into the Joint Register covering the pre-op, intra-op and post-op period of all total hip and knee replacement surgery done at the ZIOH from 1998 to 2010 was entered into a spreadsheet after verification with individual patient medical records. This was then imported ...

Generator replacement is associated with an increased rate of ICD lead alerts.

Science.gov (United States)

Lovelock, Joshua D; Cruz, Cesar; Hoskins, Michael H; Jones, Paul; El-Chami, Mikhael F; Lloyd, Michael S; Leon, Angel; DeLurgio, David B; Langberg, Jonathan J

2014-10-01

Lead malfunction is an important cause of morbidity and mortality in patients with an implantable cardioverter-defibrillator (ICD). We have shown that the failure of recalled high-voltage leads significantly increases after ICD generator replacement. However, generator replacement has not been recognized as a predictor of lead failure in general. The purpose of this study is to assess the effect of ICD generator exchange on the rate of ICD lead alerts. A time-dependent Cox proportional hazards model was used to analyze a database of remotely monitored ICDs. The model assessed the impact of generator exchange on the rate of lead alerts after ICD generator replacement. The analysis included 60,219 patients followed for 37 ± 19 months. The 5-year lead survival was 99.3% (95% confidence interval 99.2%-99.4%). Of 60,219 patients, 7458 patients (12.9%) underwent ICD generator exchange without lead replacement. After generator replacement, the rate of lead alerts was more than 5-fold higher than in controls with leads of the same age without generator replacement (hazard ratio 5.19; 95% confidence interval 3.45-7.84). A large number of lead alerted within 3 months of generator replacement. Lead alerts were more common in patients with single- vs dual-chamber ICDs and in younger patients. Sex was not associated with lead alerts. Routine generator replacement is associated with a 5-fold higher risk of lead alert compared to age-matched leads without generator replacement. This suggests the need for intense surveillance after generator replacement and the development of techniques to minimize the risk of lead damage during generator replacement. Copyright © 2014 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

Enzyme based soil stabilization for unpaved road construction

Directory of Open Access Journals (Sweden)

Renjith Rintu

2017-01-01

Full Text Available Enzymes as soil stabilizers have been successfully used in road construction in several countries for the past 30 years. However, research has shown that the successful application of these enzymes is case specific, emphasizing that enzyme performance is dependent on subgrade soil type, condition and the type of enzyme used as the stabilizer. A universal standard or a tool for road engineers to assess the performance of stabilized unbound pavements using well-established enzymes is not available to date. The research aims to produce a validated assessment tool which can be used to predict strength enhancement within a generalized statistical framework. The objective of the present study is to identify new materials for developing the assessment tool which supports enzyme based stabilization, as well as to identify the correct construction sequence for such new materials. A series of characterization tests were conducted on several soil types obtained from proposed construction sites. Having identified the suitable soil type to mix with the enzyme, a trial road construction has been performed to investigate the efficiency of the enzyme stabilization along with the correct construction sequence. The enzyme stabilization has showed significant improvement of the road performance as was evidenced from the test results which were based on site soil obtained before and after stabilization. The research will substantially benefit the road construction industry by not only replacing traditional construction methods with economical/reliable approaches, but also eliminating site specific tests required in current practice of enzyme based road construction.

Enhanced left ventricular mass regression after aortic valve replacement in patients with aortic stenosis is associated with improved long-term survival.

Science.gov (United States)

Ali, Ayyaz; Patel, Amit; Ali, Ziad; Abu-Omar, Yasir; Saeed, Amber; Athanasiou, Thanos; Pepper, John

2011-08-01

Aortic valve replacement in patients with aortic stenosis is usually followed by regression of left ventricular hypertrophy. More complete resolution of left ventricular hypertrophy is suggested to be associated with superior clinical outcomes; however, its translational impact on long-term survival after aortic valve replacement has not been investigated. Demographic, operative, and clinical data were obtained retrospectively through case note review. Transthoracic echocardiography was used to measure left ventricular mass preoperatively and at annual follow-up visits. Patients were classified according to their reduction in left ventricular mass at 1 year after the operation: group 1, less than 25 g; group 2, 25 to 150 g; and group 3, more than 150 g. Kaplan-Meier and multivariable Cox regression were used. A total of 147 patients were discharged from the hospital after aortic valve replacement for aortic stenosis between 1991 and 2001. Preoperative left ventricular mass was 279 ± 98 g in group 1 (n = 47), 347 ± 104 g in group 2 (n = 62), and 491 ± 183 g in group 3 (n = 38) (P regression such as ischemic heart disease or hypertension, valve type, or valve size used. Ten-year actuarial survival was not statistically different in patients with enhanced left ventricular mass regression when compared with the log-rank test (group 1, 51% ± 9%; group 2, 54% ± 8%; and group 3, 72% ± 10%) (P = .26). After adjustment, left ventricular mass reduction of more than 150 g was demonstrated as an independent predictor of improved long-term survival on multivariate analysis (P = .02). Our study is the first to suggest that enhanced postoperative left ventricular mass regression, specifically in patients undergoing aortic valve replacement for aortic stenosis, may be associated with improved long-term survival. In view of these findings, strategies purported to be associated with superior left ventricular mass regression should be considered when undertaking

The epidemic of aging in renal replacement therapy: an update on elderly patients and their outcomes

NARCIS (Netherlands)

Jager, K. J.; van Dijk, P. C. W.; Dekker, F. W.; Stengel, B.; Simpson, K.; Briggs, J. D.

2003-01-01

Background: In the past 2 decades, a rapid growth has occurred in the number of patients over 65 years of age accepted for renal replacement therapy (RRT) with an increasing need for dialysis resources as a consequence. The aim of this study is to describe the trends in incidence, treatment and

Body mass index and risk of perioperative cardiovascular adverse events and mortality in 34,744 Danish patients undergoing hip or knee replacement

DEFF Research Database (Denmark)

Thornqvist, Catharina; Gislason, Gunnar H; Køber, Lars

2014-01-01

underwent elective primary hip or knee replacement surgery between 2005 and 2011. We used multivariable Cox regression models to calculate the 30-day risks of MACE and mortality associated with 5 BMI groups (underweight (BMI ...BACKGROUND AND PURPOSE: Obesity is a risk factor for osteoarthritis in the lower limb, yet the cardiovascular risks associated with obesity in hip or knee replacement surgery are unknown. We examined associations between body mass index (BMI) and the risk of a major adverse cardiovascular event...... (MACE: ischemic stroke, acute myocardial infarction, or cardiovascular death) or the risk of all-cause mortality in a nationwide Danish cohort of patients who underwent primary hip or knee replacement surgery. METHODS: Using Danish nationwide registries, we identified 34,744 patients aged ≥ 20 years who...

D-dimer to guide the intensity of anticoagulation in Chinese patients after mechanical heart valve replacement: a randomized controlled trial.

Science.gov (United States)

Zhang, L; Zheng, X; Long, Y; Wu, M; Chen, Y; Yang, J; Liu, Z; Zhang, Z

2017-10-01

Essentials Low anticoagulation intensity reduces bleeding but increases thrombosis during warfarin therapy. Elevated D-dimer level is associated with increased thrombosis events. D-dimer can be used to find potential thrombosis in those receiving low intensity therapy. D-dimer-guided therapy may be the optimal strategy for those with mechanical heart valve replacement. Background Controversies remain regarding the optimal anticoagulation intensity for Chinese patients after mechanical heart valve replacement despite guidelines having recommended a standard anticoagulation intensity. Objectives To investigate whether D-dimer could be used to determine the optimal anticoagulation intensity in Chinese patients after mechanical heart valve replacement. Patients/Methods This was a prospective, randomized controlled clinical study. A total of 748 patients following mechanical heart valve replacement in Wuhan Asia Heart Hospital were randomized to three groups at a ratio of 1 : 1 : 1. Patients in two control groups received warfarin therapy based on constant standard intensity (international normalized ratio [INR], 2.5-3.5; n = 250) and low intensity (INR, 1.8-2.6; n = 248), respectively. In the experimental group (n = 250), warfarin therapy was initiated at low intensity, then those with elevated D-dimer levels were adjusted to standard intensity. All patients were followed-up for 24 months until the occurrence of endpoints, including bleeding events, thrombotic events and all-cause mortality. Results A total of 718 patients were included in the analysis. Fifty-three events occurred during follow-up. There was less hemorrhage (3/240 vs. 16/241; hazard ratio [HR], 0.18; 95% confidence interval [CI], 0.07-0.45) and all-cause mortality (4/240 vs. 12/241; HR, 0.33; 95% CI, 0.12-0.87) observed in the D-dimer-guided group than in the standard-intensity group. A lower incidence of thrombotic events was also observed in the D-dimer-guided group when compared with the

Effect of radiotherapy on the activity of lysosomal enzymes in lymphocytes and immunoglobulins in the serum in patients with laryngeal carcinoma

International Nuclear Information System (INIS)

Gierek, T.; Lisiewicz, J.; Kusnierczyk, W.; Plich, J.; Sasiadek, U.; Namyslowski, G.

1980-01-01

In 30 male patients aged 40 to 70 years treated with radiotherapy for laryngeal carcinoma presence of the lysosomal apparatus of the lymphocytes was observed after 6-9 years, with diffusion of the enzymes (especially beta-glucuronidase and N-acetyl-beta-glucosaminidase, and in a lower degree of acid phosphatase) from the lysosomes into the cytoplasm, and disappearance of normal lysosomal granules. The increased immunological reactivity of the patients was manifested frequently by a rise in the level of immunoglobulins, especially IgA in the serum, and a rise in the number of enzyme-positive lymphocytes (with the above-mentioned enzymes). (author)

Growth hormone replacement normalizes impaired fibrinolysis: new insights into endothelial dysfunction in patients with hypopituitarism and growth hormone deficiency.

Science.gov (United States)

Miljic, D; Miljic, P; Doknic, M; Pekic, S; Stojanovic, M; Cvijovic, G; Micic, D; Popovic, V

2013-12-01

Cardiovascular morbidity in adult patients with growth hormone deficiency (GHD) and hypopituitarism is increased. Clustering of cardiovascular risk factors leading to endothelial dysfunction and impaired fibrinolysis has also been reported and may account for progression to overt vascular changes in these patients. However, effect of long lasting GH replacement therapy on fibrinolytic capacity in GH deficient patients has not been investigated so far. To investigate fibrinolysis before and after challenge with venous occlusion in GHD patients with hypopituitarism before and during one year of growth hormone replacement. Hospital based, interventional, prospective study. Twenty one patient with GHD and fourteen healthy control subjects matched for age, sex and body mass index (BMI). Anthropometric, metabolic and fibrinolytic parameters were measured at the start and after three, six and twelve months of treatment with human recombinant GH. At baseline GHD patients had significantly impaired fibrinolysis compared to healthy persons. During treatment with GH, significant changes were observed in insulin like growth factor 1(IGF-1) [from baseline 6.9(2.4-13.5) to 22.0(9.0-33.0) nmol/l after one month of treatment; p<0.01] and fibrinolysis. Improvement in fibrinolysis was mostly attributed to improvement of stimulated endothelial tissue plasminogen activator (t-PA) release in response to venous occlusion [from baseline 1.1(0.4-2.6) to 1.9(0.5-8.8) after one year of treatment; p<0.01]. Growth hormone replacement therapy has favorable effects on t-PA release from endothelium and net fibrinolytic capacity in GHD adults, which may contribute to decrease their risk of vascular complications. © 2013.

Impact of patient-prosthesis mismatch following aortic valve replacement on short-term survival: a retrospective single center analysis of 632 consecutive patients with isolated stented biological aortic valve replacement.

Science.gov (United States)

Hoffmann, Grischa; Ogbamicael, Selam Abraham; Jochens, Arne; Frank, Derk; Lutter, Georg; Cremer, Jochen; Petzina, Rainer

2014-09-01

The impact of patient-prosthesis mismatch (PPM) after aortic valve replacement (AVR) on short-term and long-term mortality remains controversial. The objective of this study was to evaluate the incidence and severity of PPM and its impact on short-term survival in a large cohort of patients treated with isolated stented biological AVR in a single institution. We analyzed retrospectively data of 632 consecutive patients with aortic stenosis undergoing isolated stented biological AVR between January 2007 and February 2012 at our institution. PPM was defined as an indexed effective orifice area ≤ 0.85 cm(2)/m(2). Statistical analyses were performed to identify influencing variables on valve size implanted. Of the 632 patients investigated, 46% were females and mean age was 71.9 ± 10.4 years. PPM was observed in 93.8% (593 of 632 patients). In 71% of the patients, moderate (0.65-0.85 cm(2)/m(2)) PPM was present and in 22.8% severe (body mass index, and body surface area as simultaneous predictors of the valve size implanted (R(2)= 0.39). PPM had no discernable impact on short-term survival, although it was present in 93.8% of our patients following isolated stented biological AVR. Georg Thieme Verlag KG Stuttgart · New York.

A comparison of conventional surgery, transcatheter aortic valve replacement, and sutureless valves in "real-world" patients with aortic stenosis and intermediate- to high-risk profile.

Science.gov (United States)

Muneretto, Claudio; Alfieri, Ottavio; Cesana, Bruno Mario; Bisleri, Gianluigi; De Bonis, Michele; Di Bartolomeo, Roberto; Savini, Carlo; Folesani, Gianluca; Di Bacco, Lorenzo; Rambaldini, Manfredo; Maureira, Juan Pablo; Laborde, Francois; Tespili, Maurizio; Repossini, Alberto; Folliguet, Thierry

2015-12-01

We sought to investigate the clinical outcomes of patients with isolated severe aortic stenosis and an intermediate- to high-risk profile treated by means of conventional surgery (surgical aortic valve replacement), sutureless valve implantation, or transcatheter aortic valve replacement in a multicenter evaluation. Among 991 consecutive patients with isolated severe aortic stenosis and an intermediate- to high-risk profile (Society of Thoracic Surgeons score >4 and logistic European System for Cardiac Operative Risk Evaluation I >10), a propensity score analysis was performed on the basis of the therapeutic strategy: surgical aortic valve replacement (n = 204), sutureless valve implantation (n = 204), and transcatheter aortic valve replacement (n = 204). Primary end points were 30-day mortality and overall survival at 24-month follow-up; the secondary end point was survival free from a composite end point of major adverse cardiac events (defined as cardiac-related mortality, myocardial infarction, cerebrovascular accidents, and major hemorrhagic events) and periprosthetic regurgitation greater than 2. Thirty-day mortality was significantly higher in the transcatheter aortic valve replacement group (surgical aortic valve replacement = 3.4% vs sutureless = 5.8% vs transcatheter aortic valve replacement = 9.8%; P = .005). The incidence of postprocedural was 3.9% in asurgical aortic valve replacement vs 9.8% in sutureless vs 14.7% in transcatheter aortic valve replacement (Prisk factor for overall mortality hazard ratio (hazard ratio, 2.5; confidence interval, 1.1-4.2; P = .018). The use of transcatheter aortic valve replacement in patients with an intermediate- to high-risk profile was associated with a significantly higher incidence of perioperative complications and decreased survival at short- and mid-term when compared with conventional surgery and sutureless valve implantation. Copyright © 2015 The American Association for Thoracic Surgery. Published by

Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance.

NARCIS (Netherlands)

Oosterhof, T.; Straten, A. van; Vliegen, H.W.; Meijboom, F.J.; Dijk, A.P.J. van; Spijkerboer, A.M.; Bouma, B.J.; Zwinderman, A.H.; Hazekamp, M.G.; Roos, A.; Mulder, B.J.M.

2007-01-01

BACKGROUND: To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular (RV) volumes above which no decrease or normalization of RV size takes place after surgery. METHODS AND RESULTS: Between 1993 and 2006, 71 adult patients with

Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance

NARCIS (Netherlands)

Oosterhof, Thomas; van Straten, Alexander; Vliegen, Hubert W.; Meijboom, Folkert J.; van Dijk, Arie P. J.; Spijkerboer, Anje M.; Bouma, Berto J.; Zwinderman, Aeilko H.; Hazekamp, Mark G.; de Roos, Albert; Mulder, Barbara J. M.

2007-01-01

Background - To facilitate the optimal timing of pulmonary valve replacement, we analyzed preoperative thresholds of right ventricular ( RV) volumes above which no decrease or normalization of RV size takes place after surgery. Methods and Results - Between 1993 and 2006, 71 adult patients with

Early identification of risk factors for refractory secondary hyperparathyroidism in patients with long-term renal replacement therapy

NARCIS (Netherlands)

Jorna, Francisca Hillegonda; Tobe, TJM; Huisman, RM; de Jong, PE; Plukker, JTM; Stegeman, CA

Background. Secondary hyperparathyroidism can complicate renal replacement therapy (RRT) in patients with end-stage renal disease. Current medical therapies often result in hypercalcaemia and fail to correct hyperparathyroidism, but might be more effective at an early stage of disease. The aim of

Predictors of renal function recovery among patients undergoing renal replacement therapy following orthotopic liver transplantation.

Science.gov (United States)

Andreoli, Maria Claudia Cruz; Souza, Nádia Karina Guimarães de; Ammirati, Adriano Luiz; Matsui, Thais Nemoto; Carneiro, Fabiana Dias; Ramos, Ana Claudia Mallet de Souza; Iizuca, Ilson Jorge; Coelho, Maria Paula Vilela; Afonso, Rogério Carballo; Ferraz-Neto, Ben-Hur; Almeida, Marcio Dias de; Durão, Marcelino; Batista, Marcelo Costa; Monte, Julio Cesar; Pereira, Virgílio Gonçalves; Santos, Oscar Pavão Dos; Santos, Bento Cardoso Dos

2017-01-01

Renal dysfunction frequently occurs during the periods preceding and following orthotopic liver transplantation (OLT), and in many cases, renal replacement therapy (RRT) is required. Information regarding the duration of RRT and the rate of kidney function recovery after OLT is crucial for transplant program management. We evaluated a sample of 155 stable patients undergoing post-intensive care hemodialysis (HD) from a patient population of 908 adults who underwent OLT. We investigated the average time to renal function recovery (duration of RRT required) and determined the risk factors for remaining on dialysis > 90 days after OLT. Log-rank tests were used for univariate analysis, and Cox proportional hazards models were used to identify factors associated with the risk of remaining on HD. The results of our analysis showed that of the 155 patients, 28% had pre-OLT diabetes mellitus, 21% had pre-OLT hypertension, and 40% had viral hepatitis. Among the patients, the median MELD (Model for End-Stage Liver Disease) score was 27 (interquartile range [IQR] 22-35). When they were listed for liver transplantation, 32% of the patients had serum creatinine (Scr) levels > 1.5 mg/dL or were on HD, and 50% had serum creatinine (Scr) levels > 1.5 mg/dL or were on HD at the time of OLT. Of the transplanted patients, 25% underwent pre-OLT intermittent HD, and 14% and 41% underwent continuous renal replacement therapy (CRRT) pre-OLT and post-OLT, respectively. At 90 days post-OLT, 118 (76%) patients had been taken off dialysis, and 16 (10%) patients had died while undergoing HD. The median recovery time of these post-OLT patients was 33 (IQR 27-39) days. In the multivariate analysis, fulminant hepatic failure as the cause of liver disease (prenal function after OLT, and those who were diagnosed with fulminant hepatic failure, had no pre-OLT hypertension, received a lower transfused volume of intraoperative FFP and did not undergo pre-OLT intermittent HD had a higher probability

Basal longitudinal strain predicts future aortic valve replacement in asymptomatic patients with aortic stenosis

DEFF Research Database (Denmark)

Carstensen, Helle Gervig; Larsen, Linnea Hornbech; Hassager, Christian

2016-01-01

analysis and coronary angiography by MDCT. The combined endpoint was indication for aortic valve replacement (AVR) and sudden cardiac death. During a median follow-up of 2.3 years (interquartile range 1.7-3.6) 43 patients (41%) met the endpoint of indication for AVR. The basal (13.4 ± 3.1% vs. 15.7 ± 3.......1%) and mid-ventricular segments (14.9 ± 2.7% vs. 16.2 ± 2.9%) were significantly reduced, but with sparing of the apical segments, in patients who later underwent AVR. In various multivariable Cox regression models, including only BLS, but not GLS, remained an independent predictor of AVR. CONCLUSION...

Correlation of C-Reactive Protein and Cardiac Enzymes with Angiographic Severity of Coronary Artery Disease in Pakistani Patients with Acute Coronary Syndrome.

Science.gov (United States)

Saleem, Ayesha; Ali, Azmat

2017-02-01

To determine the correlation of C-reactive protein (CRP) levels with the severity of coronary stenosis on angiography and the association of cardiac enzymes with the degree of stenosis in acute coronary syndrome (ACS) patients. Secondly, to compare association of angiographic severity of vascular stenosis with CRP in patients with ST segment elevation myocardial infarction (STEMI) and non-STEMI / Unstable angina (UA). Prospective, descriptive study. Khan Research Laboratories (KRL) Hospital, from October 2014 to March 2015. CRP was measured on diagnosis of ACS in 70 patients. Cardiac enzymes were measured 6 hours after the onset of chest pain. Angiographic scoring for degree of stenosis and number of culprit vessels was done. Two groups consisting of patients with STEMI (group 1) and with NSTEMI/UA (group 2) were made. No correlation was found between CRP levels and angiographic stenosis in patients with ACS (r=0.162, p>0.05). No association was found between eosinophil count and severity of stenosis (p=0.88). Rise of cardiac enzymes and degree of coronary stenosis showed a positive correlation (p Rise in cardiac enzymes still grade high in predicting severity of vascular stenosis than eosinophil count or CRP levels.

Asymptomatic and Persistent Elevation of Pancreatic Enzymes in an Ulcerative Colitis Patient

Directory of Open Access Journals (Sweden)

Elisa Liverani

2013-01-01

Full Text Available Azathioprine has been extensively used in the management of inflammatory bowel diseases. It might cause pancreatic damage in the form of either asymptomatic elevation in serum amylase/lipase or overt acute pancreatitis. Here we report the case of a 61-year-old patient with ulcerative colitis who had been treated with azathioprine for three years, achieving clinical remission. During treatment he presented an asymptomatic elevation of serum pancreatic enzymes, without any signs of pancreatitis at imaging. This evidence brought us to reassess the drug dosage, without achieving a normalization of biochemical analysis. Autoimmune pancreatitis was excluded. One year after the suspension of azathioprine, we still face persistent high levels of amylase/lipase. Normalization of enzymatic values in patients who develop intolerance to azathioprine, in the form of either asymptomatic elevation in serum amylase/lipase or overt acute pancreatitis, is usually achieved in about two months after stopping drug intake. Asymptomatic elevation in serum pancreatic enzymes in the absence of pancreatic disease is reported in the literature and defined as “Gullo’s syndrome,” but nobody of the subjects studied had been treated in the past with pancreatotoxic drugs. Might this case be defined as “benign pancreatic hyperenzymemia”?

Aquatic exercises versus land based exercises for elderly patients after a total hip replacement

OpenAIRE

Miroljub Jakovljevič; Renata Vauhnik

2011-01-01

Background: Aquatic therapy allows secure, active exercise with pain reduction using a combination of the water’s buoyancy, hydrostatic pressure, resistance and warmth. By aquatic therapy, elderly patients after total hip replacement can achieve more positive effects than by land-based exercise. The aim of the study was to investigate the use of aquatic-based exercises in the rehabilitation programme after a hip fracture surgery in elderly adults. Results: Both groups, regardless of the ty...

Avascular necrosis of bone complicating corticosteroid replacement therapy.

OpenAIRE

Williams, P L; Corbett, M

1983-01-01

Two patients who developed widespread severe avascular necrosis of bone while on steroid replacement therapy are described. One, a diabetic, underwent yttrium-90 pituitary ablation for retinopathy and developed avascular necrosis within 18 months of starting prednisolone. The other, who had Addison's disease, developed avascular necrosis within 14 months of starting cortisol replacement therapy. Both cases came to bilateral total hip replacement.

Alkali replacement raises urinary citrate excretion in patients with topiramate-induced hypocitraturia.

Science.gov (United States)

Jhagroo, R Allan; Wertheim, Margaret L; Penniston, Kristina L

2016-01-01

The aims of this study were to assess (1) the magnitude and temporality of decreased urinary citrate excretion in patients just starting topiramate and (2) the effect of alkali replacement on topiramate-induced hypocitraturia. Study 1 was a prospective, non-intervention study in which patients starting topiramate for headache remediation provided pre- and post-topiramate 24 h urine collections for measurement of urine citrate. Study 2 was a clinical comparative effectiveness study in which patients reporting to our stone clinic for kidney stones and who were treated with topiramate were prescribed alkali therapy. Pre- and post-alkali 24 h urinary citrate excretion was compared. Data for 12 and 22 patients (studies 1 and 2 respectively) were evaluated. After starting topiramate, urinary citrate excretion dropped significantly by 30 days (P = 0.016) and 62% of patients had hypocitraturia (citrate alkali, urine citrate increased in stone-forming patients on topiramate (198 ± 120 to 408 ± 274 mg day(-1) ; P = 0.042 for difference). 85% of patients were hypocitraturic on topiramate alone vs. 40% after adding alkali. The increase in urinary citrate was greater in patients provided ≥ 90 mEq potassium citrate. Our study is the first to provide clinical evidence that alkali therapy can raise urinary citrate excretion in patients who form kidney stones while being treated with topiramate. Clinicians should consider alkali therapy for reducing the kidney stone risk of patients benefitting from topiramate treatment for migraine headaches or other conditions. © 2015 The British Pharmacological Society.

Flow-Based Systems for Rapid and High-Precision Enzyme Kinetics Studies

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Supaporn Kradtap Hartwell

2012-01-01

Full Text Available Enzyme kinetics studies normally focus on the initial rate of enzymatic reaction. However, the manual operation of steps of the conventional enzyme kinetics method has some drawbacks. Errors can result from the imprecise time control and time necessary for manual changing the reaction cuvettes into and out of the detector. By using the automatic flow-based analytical systems, enzyme kinetics studies can be carried out at real-time initial rate avoiding the potential errors inherent in manual operation. Flow-based systems have been developed to provide rapid, low-volume, and high-precision analyses that effectively replace the many tedious and high volume requirements of conventional wet chemistry analyses. This article presents various arrangements of flow-based techniques and their potential use in future enzyme kinetics applications.

Timing of renal replacement therapy and clinical outcomes in critically ill patients with severe acute kidney injury

NARCIS (Netherlands)

Bagshaw, Sean M.; Uchino, Shigehiko; Bellomo, Rinaldo; Morimatsu, Hiroshi; Morgera, Stanislao; Schetz, Miet; Tan, Ian; Bouman, Catherine; Macedo, Ettiene; Gibney, Noel; Tolwani, Ashita; Oudemans-van Straaten, Heleen M.; Ronco, Claudio; Kellum, John A.; French, Craig; Mulder, John; Pinder, Mary; Roberts, Brigit; Botha, John; Mudholkar, Pradeen; Holt, Andrew; Hunt, Tamara; Honoré, Patrick Maurice; Clerbaux, Gaetan; Schetz, Miet Maria; Wilmer, Alexander; Yu, Luis; Macedo, Ettiene V.; Laranja, Sandra Maria; Rodrigues, Cassio José; Suassuna, José Hermógenes Rocco; Ruzany, Frederico; Campos, Bruno; Leblanc, Martine; Senécal, Lynne; Gibney, R. T. Noel; Johnston, Curtis; Brindley, Peter; Tan, Ian K. S.; Chen, Hui De; Wan, Li; Rokyta, Richard; Krouzecky, Ales; Neumayer, Hans-Helmut; Detlef, Kindgen-Milles; Mueller, Eckhard; Tsiora, Vicky; Sombolos, Kostas; Mustafa, Iqbal; Suranadi, Iwayan; Bar-Lavie, Yaron; Nakhoul, Farid; Ceriani, Roberto; Bortone, Franco; Zamperetti, Nereo; Pappalardo, Federico; Marino, Giovanni; Calabrese, Prospero; Monaco, Francesco; Liverani, Chiara; Clementi, Stefano; Coltrinari, Rosanna; Marini, Benedetto; Fuke, Nobuo; Miyazawa, Masaaki; Katayama, Hiroshi; Kurasako, Toshiaki; Hirasaw, Hiroyuki; Oda, Shigeto; Tanigawa, Koichi; Tanaka, Keiichi; Oudemans-van Straaten, Helena Maria; de Pont, Anne-Cornelie J. M.; Bugge, Jan Frederik; Riddervold, Fridtjov; Nilsen, Paul Age; Julsrud, Joar; Teixeira e Costa, Fernando; Marcelino, Paulo; Serra, Isabel Maria; Yaroustovsky, Mike; Grigoriyanc, Rachik; Lee, Kang Hoe; Loo, Shi; Singh, Kulgit; Barrachina, Ferran; Llorens, Julio; Sanchez-Izquierdo-Riera, Jose Angel; Toral-Vazquez, Darío; Wizelius, Ivar; Hermansson, Dan; Gaspert, Tomislav; Maggiorini, Marco; Davenport, Andrew; Lombardi, Raúl; Llopart, Teresita; Venkataraman, Ramesh; Kellum, John; Murray, Patrick; Trevino, Sharon; Benjamin, Ernest; Hufanda, Jerry; Paganini, Emil; Warnock, David; Guirguis, Nabil

2009-01-01

The aim of this study is to evaluate the relationship between timing of renal replacement therapy (RRT) in severe acute kidney injury and clinical outcomes. This was a prospective multicenter observational study conducted at 54 intensive care units (ICUs) in 23 countries enrolling 1238 patients.

The effect of tourniquet deflation on hemodynamics and regional cerebral oxygen saturation in aged patients undergoing total knee replacement surgery.

Science.gov (United States)

Song, Inkyung; Kim, Dong Yeon; Kim, Youn Jin

2012-11-01

Inflation and deflation of a pneumatic tourniquet used in total knee replacement surgery induces various changes in patient's hemodynamic and metabolic status, which may result in serious complications, especially in aged patients. Near-infrared spectroscopy (NIRS) is a monitoring device designed to estimate the regional cerebral oxygen saturation. We evaluated the effect of tourniquet deflation on hemodynamics and regional cerebral oxygen saturation in aged patients undergoing total knee replacement surgery, using NIRS. Twenty-eight American Society of Anesthesiologists physical status I or II patients, over the age of sixty-five years undergoing total knee replacement surgery, were included. Under general anesthesia, the mean arterial pressure (MAP), heart rate (HR), cardiac output (CO), stroke volume (SV), and regional cerebral oxygen saturation (rSO(2)) were recorded before induction of anesthesia and every 2 min after tourniquet deflation for 20 min. Arterial blood gas analysis was performed 5 min before, in addition to 0, and 10 min after tourniquet deflation. The decrease of rSO(2) was not significant during 20-min deflation period. MAP, CO and SV showed significant decrease during 2 to 12, 4 to 6 and 2 to 6-min period after tourniquet deflation, respectively (P deflation caused significant changes in hemodynamic and metabolic status, but not in regional cerebral oxygen saturation. It is recommended to monitor neurologic status, as well as hemodynamic and metabolic status to avoid serious complications, especially in aged patients.

Patients' perceptions of information and education for renal replacement therapy: an independent survey by the European Kidney Patients' Federation on information and support on renal replacement therapy.

Directory of Open Access Journals (Sweden)

Wim Van Biesen

Full Text Available Selection of an appropriate renal replacement modality is of utmost importance for patients with end stage renal disease. Previous studies showed provision of information to and free modality choice by patients to be suboptimal. Therefore, the European Kidney Patients' Federation (CEAPIR explored European patients' perceptions regarding information, education and involvement on the modality selection process.CEAPIR developed a survey, which was disseminated by the national kidney patient organisations in Europe.In total, 3867 patients from 36 countries completed the survey. Respondents were either on in-centre haemodialysis (53% or had a functioning graft (38% at the time of survey. The majority (78% evaluated the general information about kidney disease and treatment as helpful, but 39% did not recall being told about alternative treatment options than their current one. Respondents were more often satisfied with information provided on in-centre haemodialysis (90% and transplantation (87% than with information provided on peritoneal dialysis (79% or home haemodialysis (61%, and were more satisfied with information from health care professionals vs other sources such as social media. Most (75% felt they had been involved in treatment selection, 29% perceived they had no free choice. Involvement in modality selection was associated with enhanced satisfaction with treatment (OR 3.13; 95% CI 2.72-3.60. Many respondents (64% could not remember receiving education on how to manage their kidney disease in daily life. Perceptions on information seem to differ between countries.Kidney patients reported to be overall satisfied with the information they received on their disease and treatment, although information seemed mostly to have been focused on one modality. Patients involved in modality selection were more satisfied with their treatment. However, in the perception of the patients, the freedom to choose an alternative modality showed room for

Circadian hormone profiles and insulin sensitivity in patients with Addison's disease: a comparison of continuous subcutaneous hydrocortisone infusion with conventional glucocorticoid replacement therapy.

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Björnsdottir, Sigridur; Øksnes, Marianne; Isaksson, Magnus; Methlie, Paal; Nilsen, Roy M; Hustad, Steinar; Kämpe, Olle; Hulting, Anna-Lena; Husebye, Eystein S; Løvås, Kristian; Nyström, Thomas; Bensing, Sophie

2015-07-01

Conventional glucocorticoid replacement therapy in patients with Addison's disease (AD) is unphysiological with possible adverse effects on mortality, morbidity and quality of life. The diurnal cortisol profile can likely be restored by continuous subcutaneous hydrocortisone infusion (CSHI). The aim of this study was to compare circadian hormone rhythms and insulin sensitivity in conventional thrice-daily regimen of glucocorticoid replacement therapy with CSHI treatment in patients with AD. An open, randomized, two-period, 12-week crossover multicentre trial in Norway and Sweden. Ten Norwegian patients were admitted for 24-h sampling of hormone profiles. Fifteen Swedish patients underwent euglycaemic-hyperinsulinaemic clamp. Thrice-daily regimen of oral hydrocortisone (OHC) and CSHI treatment. We measured the circadian rhythm of cortisol, adrenocorticotropic hormone (ACTH), growth hormone (GH), insulin-like growth factor-1, (IGF-1), IGF-binding protein-3 (IGFBP-3), glucose, insulin and triglycerides during OHC and CSHI treatment. Euglycaemic-hyperinsulinaemic clamp was used to assess insulin sensitivity. Continuous subcutaneous hydrocortisone infusion provided a more physiological circadian cortisol curve including a late-night cortisol surge. ACTH levels showed a near normal circadian variation for CSHI. CSHI prevented a continuous decrease in glucose during the night. No difference in insulin sensitivity was observed between the two treatment arms. Continuous subcutaneous hydrocortisone infusion replacement re-established a circadian cortisol rhythm and normalized the ACTH levels. Patients with CSHI replacement had a more stable night-time glucose level compared with OHC without compromising insulin sensitivity. Thus, restoring night-time cortisol levels might be advantageous for patients with AD. © 2015 John Wiley & Sons Ltd.

Changing of the guard: reducing infection when replacing neural pacemakers.

Science.gov (United States)

Pepper, Joshua; Meliak, Lara; Akram, Harith; Hyam, Jonathan; Milabo, Catherine; Candelario, Joseph; Foltynie, Thomas; Limousin, Patricia; Curtis, Carmel; Hariz, Marwan; Zrinzo, Ludvic

2017-04-01

OBJECTIVE Infection of deep brain stimulation (DBS) hardware has a significant impact on patient morbidity. Previous experience suggests that infection rates appear to be higher after implantable pulse generator (IPG) replacement surgery than after the de novo DBS procedure. In this study the authors examine the effect of a change in practice during DBS IPG replacements at their institution. METHODS Starting in January 2012, patient screening for methicillin-resistant Staphylococcus aureus (MRSA) and, and where necessary, eradication was performed prior to elective DBS IPG change. Moreover, topical vancomycin was placed in the IPG pocket during surgery. The authors then prospectively examined the infection rate in patients undergoing DBS IPG replacement at their center over a 3-year period with at least 9 months of follow-up. RESULTS The total incidence of infection in this prospective consecutive series of 101 IPG replacement procedures was 0%, with a mean follow-up duration of 24 ± 11 months. This was significantly lower than the authors' previously published historical control group, prior to implementing the change in practice, where the infection rate for IPG replacement was 8.5% (8/94 procedures; p = 0.003). CONCLUSIONS This study suggests that a change in clinical practice can significantly lower infection rates in patients undergoing DBS IPG replacement. These simple measures can minimize unnecessary surgery, loss of benefit from chronic stimulation, and costly hardware replacement, further improving the cost efficacy of DBS therapies.

Effect of Patient-Prosthesis Mismatch in Aortic Position on Late-Onset Tricuspid Regurgitation and Clinical Outcomes after Double Valve Replacement.

Science.gov (United States)

Lee, Seung Hyun; Youn, Young Nam; Chang, Byung Chul; Joo, Hyun Chel; Lee, Sak; Yoo, Kyung Jong

2017-09-01

Significant late-onset tricuspid regurgitation (TR) is unfortunately common after double valve replacement (DVR); however, its underlying factors remain undefined. We evaluated the effect of aortic patient-prosthesis mismatch (PPM) on late-onset TR and clinical outcomes after DVR. Of the 2392 consecutive patients who underwent aortic valve replacement between January 1990 and May 2014 at our institution, we retrospectively studied 462 patients who underwent DVR (excluding concomitant tricuspid valvular annuloplasty or replacement). Survival and freedom from grade >3 TR were compared between PPM (n=152) and non-PPM (n=310) groups using the Kaplan-Meier method. Although the overall survival rates were similar between the two groups at 5 and 10 years (95%, 91% vs. 96%, 93%, p=0.412), grade >3 TR-free survival was significantly lower in the PPM group (98%, 91% vs. 99%, 95%, p=0.014). Small body-surface area, atrial fibrillation, PPM, and subaortic pannus were risk factors for TR progression. However, aortic prosthesis size and trans-valvular pressure gradient were not significant factors for either TR progression or overall survival. Aortic PPM in DVR, regardless of mitral prosthesis size, was associated with late TR progression, but was not significantly correlated with overall survival. Therefore, we recommend careful echocardiographic follow-up for the early detection of TR progression in patients with aortic PPM in DVR. © Copyright: Yonsei University College of Medicine 2017

Study of wettability of calcite surfaces using oil-brine-enzyme systems for enhanced oil recovery applications

DEFF Research Database (Denmark)

Khusainova, Alsu; Nielsen, Sidsel Marie; Pedersen, Hanne Høst

2015-01-01

and adhesion behaviour tests. Comparative studies with a surfactant, protein, purified enzyme, enzyme stabiliser using n-decane (as a model for the oil) have also been carried out in order to verify experimental results. The enzymes that have the highest effect on the wettability have been identified. Those...... action has been found to be replacement of oil at the solid surface by the enzyme. Other mechanisms (modification of the surface tension or catalytic modification of hydrocarbons resulting in reducing the oil viscosity) have shown to be much less pronounced from the measurements reported here....

Delay in onset of metabolic alkalosis during regional citrate anti-coagulation in continous renal replacement therapy with calcium-free replacement solution

Directory of Open Access Journals (Sweden)

See Kay

2009-01-01

Full Text Available Regional citrate anti-coagulation for continuous renal replacement therapy chelates calcium to produce the anti- coagulation effect. We hypothesise that a calcium-free replacement solution will require less citrate and produce fewer metabolic side effects. Fifty patients, in a Medical Intensive Care Unit of a tertiary teaching hospital (25 in each group, received continuous venovenous hemofiltration using either calcium-containing or calcium-free replacement solutions. Both groups had no significant differences in filter life, metabolic alkalosis, hypernatremia, hypocalcemia, and hypercalcemia. However, patients using calcium-containing solution developed metabolic alkalosis earlier, compared to patients using calcium-free solution (mean 24.6 hours,CI 0.8-48.4 vs. 37.2 hours, CI 9.4-65, P = 0.020. When calcium-containing replacement solution was used, more citrate was required (mean 280ml/h, CI 227.2-332.8 vs. 265ml/h, CI 203.4-326.6, P = 0.069, but less calcium was infused (mean 21.2 ml/h, CI 1.2-21.2 vs 51.6ml/h, CI 26.8-76.4, P ≤ 0.0001.

Sleeve gastrectomy leads to easy management of hormone replacement therapy and good weight loss in patients treated for craniopharyngioma.

Science.gov (United States)

Trotta, Manuela; Da Broi, Joël; Salerno, Angelo; Testa, Rosa M; Marinari, Giuseppe M

2017-03-01

The aim of this study is to investigate the effects of sleeve gastrectomy on hormone replacement therapy and on hypothalamic obesity in patients affected by craniopharyngioma with post-surgical pan-hypopituitarism. A retrospective review of three patients, treated for hypothalamic obesity with laparoscopic sleeve gastrectomy, who have previously undergone surgery for craniopharyngioma in their childhood, was done. Patients' mean age and BMI were 22.3 years (range 21-24) and 49.2 kg/m 2 (range 41.6-58.1), respectively. The mean time of delay between neurosurgery and bariatric surgery was 12.3 years (range 6-16). There were no major complications or deaths. At 24 months follow-up, the mean BMI was 35.3 kg/m 2 (range 31.2-40.6). No hydrocortisone and sex steroids dose changes were observed, while levothyroxine was decreased in two patients. Growth hormone replacement therapy was increased in two patients, whereas it was started in one patient. Desmopressin was significantly decreased in all of them. Patients with surgically induced pan-hypopituitarism after craniopharyngioma who become obese, can expect good results from sleeve gastrectomy: this procedure does not have significant negative effects on hormone substitution and leads to a good stabilization of body weight in a mid-term follow-up.

Relationship between angiotensin-converting enzyme gene polymorphism and cardio-brain complications in patients with NIDDM (type 2 diabetes mellitus)

International Nuclear Information System (INIS)

Xu Qinfang; Zhu Yan; Ding Mingwei

2002-01-01

Objective: To investigate the relationship between angiotensin-converting enzyme gene polymorphism and cardio-brain complications in patients with NIDDM. Methods: The angiotensin-converting enzyme (ACE) gene insertion/deletion polymorphism in 174 patients with NIDDM and 62 controls were examined with PCR. Results: ACE gene I/D polymorphism was closely related to coronary heart disease (angina, cardiac infarction) and cerebral infarction in diabetic patients but not with hypertension. Plasma renin activity and plasma angiotensin II levels in complicated diabetic patients with ACE D/D gene were significantly higher than those in the controls (p < 0.01). Their aldosterone and endothelin contents were not significantly different. Conclusion: Examination of ACE gene I/D polymorphism was useful for the primary prevention of cardio-brain complications in diabetic patients and helpful in the early diagnosis and therapy of coronary heart disease and cerebral infarction

Optimal timing of aortic valve replacement in elderly patients with severe aortic stenosis.

Science.gov (United States)

Marumoto, Akira; Nakamura, Yoshinobu; Kishimoto, Yuichiro; Saiki, Munehiro; Nishimura, Motonobu

2014-01-01

The elderly population with severe aortic stenosis (AS) requiring aortic valve replacement (AVR) is increasing. The optimal timing of AVR in these patients has been under discussion. We retrospectively reviewed the data from severe AS patients (n = 84) who underwent AVR with/without concomitant procedures from 2005 to 2010. The symptom status, preoperative data, operative outcome, late survival and freedom from cardiac events were compared between elderly patients (age ≥80 years [n = 31]) and younger patients (age <80 years [n = 53]). The operative mortality in elderly patients (3.2 %) and younger patients (3.8 %) was comparable. The symptoms in elderly patients were more severe and hospitalized heart failure (HF) was more frequently noted as the primary symptom (p = 0.017). Patients with and without hospitalized HF differed significantly in late survival and freedom from cardiac events (p = 0.001), but advanced age had no significant effect. The results of a Cox proportional hazards analysis revealed that hospitalized HF was a significant predictor for cardiac events after AVR, irrespective of age (hazard ratio 6.93, 95 % confidence interval 1.83-26.26, p < 0.004). In elderly patients with severe AS, surgery should be recommended even in the presence of minimal symptoms and should be performed before the onset of life-threatening HF.

Ultra Low-Dose Naloxone and Tramadol/Acetaminophen in Elderly Patients Undergoing Joint Replacement Surgery: A Pilot Study

Directory of Open Access Journals (Sweden)

Ngozi N Imasogie

2009-01-01

Full Text Available OBJECTIVE: A pilot study was conducted to assess whether both the rationale and feasibility exist for future randomized clinical trials to evaluate the combined use of naloxone infusion and tramadol/acetaminophen as opioid-sparing drugs in elderly patients undergoing lower extremity joint replacement surgery.

We Offer Renal Replacement Therapy to Patients Who Are Not Benefitted by It.

Science.gov (United States)

Holley, Jean L

2016-07-01

Nephrologists offer renal replacement therapy (RRT) to patients who are unlikely to benefit in part because of our discomfort discussing goals of care in the setting of an uncertain prognosis for a given individual. Permanent neurological impairment, terminal illness (life expectancy <6 months), medical conditions precluding the safe delivery of dialysis, elderly patients with poor prognosis, and those who begin "early" RRT are categories of patients for whom dialysis may not be beneficial. Successful use of time-limited trials of dialysis may reduce the number of patients who are started on RRT without significant benefit. However, clear achievable milestones and goals need to be incorporated into plans for time-limited trials to ensure that continuing RRT beyond the trial period is appropriate. The lack of information on outcomes and symptom management using a "palliative approach" to dialysis suggests this should not be a clinical option until additional study is done and efficacy data available. Clinical practice guidelines are available to assist nephrologists in the appropriate withholding of RRT. © 2016 Wiley Periodicals, Inc.

CUSTOM TRIFLANGE ACETABULAR COMPONENTS IN REVISION HIP REPLACEMENT (EXPERIENCE REVIEW

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A. A. Korytkin

2017-01-01

Full Text Available Extensive defects of acetabulum especially accompanied by pelvis discontinuity at the level of acetabulum pose a serious challenge in revision hip replacement and create additional complexity in fixing the acetabular component. One of the perspective options to solve the above issue is the use of custom triflange acetabular components (CTAC designed based on preoperative computer tomography given the specifics of bone defects of the patient. Purpose of the study — to evaluate the outcomes of CTAC use in revision hip replacement.Materials and methods. The authors analyzed treatment outcomes of 12 patients after revision hip replacement using additive techniques of computer simulation and 3D printing CTAC. Follow up period after the surgery averaged 7±3 months (from one to ten months. 7 out of 12 patients had acetabular defects of Paprosky 3B type, 4 patients had defects of Paprosky 3A and in one patient — of Paprosky 2C.Results. Two out of twelve patients had prosthesis dislocations that required revision hip surgery, one of those patients underwent open reduction of dislocation with wound debridement, another patient underwent replacement of articulating couple of acetabular component. Total scores under Harris Hip Score and paint VAS score prior to treatment was 28±7 and 7±1 points respectively, postoperative scores were 76±9 and 3±1 respectively.Conclusion. The application of additive techniques for revision hip replacement in patients with extensive acetabular and pelvic defects allows to make a precise preoperative planning, to restore joint rotation center, to reconstruct bone defects and to securely fix triflange acetabular component that altogether significantly improve treatment outcomes and patients satisfaction with the surgery.

Effect of vitamin D replacement on immunological biomarkers in patients with multiple sclerosis.

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Mrad, May F; El Ayoubi, Nabil K; Esmerian, Maria O; Kazan, Jalal M; Khoury, Samia J

2017-08-01

We aimed to investigate the immunologic effects of vitamin D replacement in RRMS patients. In a controlled single center study, patients deficient in 25-hydroxyvitamin D (serum levelvitamin D patients (serum level>35ng/ml) were followed for the same period. Assessments were performed at baseline and at 3months. 25-hydroxyvitamin D levels increased significantly from baseline to month-3 in the deficient group after treatment and remained stable in the sufficient group. We observed a decreased interferon-γ (IFNγ) secretion by CD4 + T cells in vitamin D deficient group but not in the sufficient group, and a negative correlation between baseline serum vitamin D and IFNγ production. There was no change in the frequency of T helper or regulatory T cell subsets in either group. Increasing serum levels of 25-hydroxyvitamin D are associated with decreased production of IFNγ by CD4 + T cells. Copyright © 2017 Elsevier Inc. All rights reserved.

How best to use partial meal replacement in managing overweight or obese patients with poorly controlled type 2 diabetes.

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Leader, Natasha J; Ryan, Lynne; Molyneaux, Lynda; Yue, Dennis K

2013-02-01

To compare patient compliance and benefits, over 12 months, of 1 versus 2 partial meal replacement (PMR) for the management of overweight/obese subjects with inadequately controlled type 2 diabetes. Thirty-six overweight patients with inadequately controlled type 2 diabetes (BMI > 27 kg/m(2) and HbA1c > 7.5% [58 mmol/mol]) were randomized to receive 1 or 2 PMR/day, while maintaining usual lifestyle. Subjects were seen monthly and adjustment of medications was made to prevent hypoglycemia. Compliance was assessed by counting unused sachets. Patients on 2 PMR/day lost almost 4 kg compared with only 0.5 kg in the 1 PMR/day group. This difference was statistically significant (P meal replacement. Reductions in weight, waist, and HbA1c were better in the 2 PMR/day group while patient dropout and compliance were not worse over a 12-month period. PMR provides a further management option for overweight/obese individuals with type 2 diabetes. The initial recommendation should be 2 PMR/day. Copyright © 2012 The Obesity Society.

[Effects of growth hormone replacement therapy on bone metabolism].

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Yamamoto, Masahiro; Sugimoto, Toshitsugu

2014-06-01