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Sample records for patients enzyme replacement

  1. Combined miglustat and enzyme replacement therapy in two patients with type 1 Gaucher disease: two case reports.

    Science.gov (United States)

    Amato, Dominick; Patterson, Mary Anne

    2018-01-27

    Intravenous enzyme replacement therapy is a first-line therapy for Gaucher disease type 1, and substrate reduction therapy represents an oral treatment alternative. Both enzyme replacement therapy and substrate reduction therapy are generally used as monotherapies in Gaucher disease. However, one randomized study and several case reports have described combination therapy over short time periods. We report two female Gaucher disease type 1 patients of mainly Anglo-Saxon descent, where combined enzyme replacement therapy and miglustat substrate reduction therapy were administered to overcome refractory clinical symptoms. The first patient was diagnosed at age 17 and developed Gaucher disease-related bone manifestations that worsened despite starting imiglucerase enzyme replacement therapy. After switching to miglustat substrate reduction therapy, her bone symptoms improved, but she developed tremors and eventually switched back to enzyme replacement therapy. Miglustat was later recommenced in combination with ongoing enzyme replacement therapy due to continued bone pain, and her bone symptoms improved along with maintained visceral manifestations. Enzyme replacement therapy was subsequently tapered off and the patient has since been successfully maintained on miglustat. The second patient was diagnosed aged 3, and commenced imiglucerase enzyme replacement therapy aged 15. After 9 years on enzyme replacement therapy she switched to miglustat substrate reduction therapy and her core symptoms were maintained/stable for 3 years. Imiglucerase enzyme replacement therapy was later added as a boost to therapy and her symptoms were subsequently maintained over a 2.3-year period. However, miglustat was discontinued due to her relocation, necessitating an increase in enzyme replacement therapy dose. Overall, both patients benefited from combination therapy. While the majority of Gaucher disease type 1 patients will not need treatment with both substrate reduction therapy

  2. Recommendations for initiation and cessation of enzyme replacement therapy in patients with Fabry disease

    DEFF Research Database (Denmark)

    Biegstraaten, Marieke; Arngrímsson, Reynir; Barbey, Frederic

    2015-01-01

    INTRODUCTION: Fabry disease (FD) is a lysosomal storage disorder resulting in progressive nervous system, kidney and heart disease. Enzyme replacement therapy (ERT) may halt or attenuate disease progression. Since administration is burdensome and expensive, appropriate use is mandatory. We aimed ...

  3. Histologic findings of femoral heads from patients with Gaucher disease treated with enzyme replacement.

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    Lebel, Ehud; Elstein, Deborah; Peleg, Ariel; Reinus, Constantine; Zimran, Ari; Amir, Gail

    2013-07-01

    To assess correlations of patient demographics, including enzyme replacement therapy (ERT) with bone histology, to facilitate decisions of whether and when to perform hip replacement surgery in patients with Gaucher disease. We examined the histology of surgically removed femoral heads and categorized findings by the presence or extent of osteonecrosis, Gaucher cell (GC) infiltration, and bone regeneration qualifiers using a tripartite histology-based scoring system. Twenty-two patients with 26 bone specimens were evaluated. Seventeen patients (77%) were splenectomized, 16 (73%) received ERT, and 12 (55%) had the putatively milder genotype (N370S/N370S), with the rest putatively at increased risk for skeletal disease (N370S/other). The 3 histology subscores were applicable to all specimens. Osteonecrotic bone was seen in 19 of 26 (73%); osteoarthritis was seen in all cartilage specimens. Gaucher cell infiltration was not correlated with demographics or disease severity. A trend was noted between reduced GC infiltration and ERT (ρ = 0.407), but regeneration qualifiers were not correlated with ERT or other features. Histologic findings of GC infiltration and bone regeneration qualifiers did not correlate with demographics or with exposure to ERT. Most specimens unexpectedly showed good regenerative responses to osteonecrosis despite heavy GC infiltration.

  4. COMPARING THE ENZYME REPLACEMENT THERAPY COST IN POST PANCREATECTOMY PATIENTS DUE TO PANCREATIC TUMOR AND CHRONIC PANCREATITIS.

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    Fragoso, Anna Victoria; Pedroso, Martha Regina; Herman, Paulo; Montagnini, André Luis

    2016-01-01

    Among late postoperative complications of pancreatectomy are the exocrine and endocrine pancreatic insufficiencies. The presence of exocrine pancreatic insufficiency imposes, as standard treatment, pancreatic enzyme replacement. Patients with chronic pancreatitis, with intractable pain or any complications with surgical treatment, are likely to present exocrine pancreatic insufficiency or have this condition worsened requiring adequate dose of pancreatic enzymes. The aim of this study is to compare the required dose of pancreatic enzyme and the enzyme replacement cost in post pancreatectomy patients with and without chronic pancreatitis. Observational cross-sectional study. In the first half of 2015 patients treated at the clinic of the Department of Gastrointestinal Surgery at Hospital das Clínicas, Universidade de São Paulo, Brazil, who underwent pancreatectomy for at least 6 months and in use of enzyme replacement therapy were included in this series. The study was approved by the Research Ethics Committee. The patients were divided into two groups according to the presence or absence of chronic pancreatitis prior to pancreatic surgery. For this study, Ptreatment was R$ 2150.5 ± 729.39; R$ 2118.18 ± 731.02 in patients without pancreatitis and R$ 2217.74 ± 736.30 in patients with pancreatitis. There was no statistically significant difference in the cost of treatment of enzyme replacement post pancreatectomy in patients with or without chronic pancreatitis prior to surgical indication.

  5. Resolution of Hydronephrosis in a Patient With Mucopolysaccharidosis Type II With Enzyme Replacement Therapy.

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    Nishiyama, Kei; Imai, Takashi; Ohkubo, Kazuhiro; Sanefuji, Masafumi; Takada, Hidetoshi

    2017-03-01

    Mucopolysaccharidosis type II (MPS II) is caused by deficiency of lysosomal enzyme iduronate-2-sulfatase. Insufficient activity of the enzyme results in accumulation of glycosaminoglycans leading to progressive multisystem pathologies. MPS II is less likely to be complicated by kidney and urinary tract problems. We report a boy with MPS II, who developed left hydronephrosis. His hydronephrosis improved after starting enzyme replacement therapy. It was suggested that MPS II was closely associated with the pathogenesis of hydronephrosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. Hearing loss in adult patients with Fabry disease treated with enzyme replacement therapy

    NARCIS (Netherlands)

    Suntjens, Eefje B.; Smid, Bouwien E.; Biegstraaten, Marieke; Dreschler, Wouter A.; Hollak, Carla E. M.; Linthorst, Gabor E.

    2015-01-01

    Data on prevalence, natural history, and effect of enzyme replacement therapy (ERT) on hearing loss (HL) in Fabry disease (FD) are scarce. This is a retrospective study with cross-sectional and longitudinal analyses. Low and high-frequency HL in the Dutch FD cohort was studied in four groups:

  7. Successful switch from enzyme replacement therapy to miglustat in an adult patient with type 1 Gaucher disease: a case report.

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    Giuffrida, Gaetano; Lombardo, Rita; Di Francesco, Ernesto; Parrinello, Laura; Di Raimondo, Francesco; Fiumara, Agata

    2016-11-08

    Gaucher disease is one of the most common lipid-storage disorders, affecting approximately 1 in 75,000 births. Enzyme replacement therapy with recombinant glucocerebrosidase is currently considered the first-line treatment choice for patients with symptomatic Gaucher disease type 1. Oral substrate reduction therapy is generally considered a second-line treatment option for adult patients with mild to moderate Gaucher disease type 1 who are unable or unwilling to receive lifelong intravenous enzyme infusions. The efficacy and safety of the oral substrate reduction therapy miglustat (Zavesca®) in patients with Gaucher disease type 1 have been established in both short-term clinical trials and long-term, open-label extension studies. Published data indicate that miglustat can be used as maintenance therapy in patients with stable Gaucher disease type 1 switched from previous enzyme replacement therapy. We report a case of a 44-year-old Caucasian man with Gaucher disease type 1 who was initially treated with enzyme replacement therapy but, owing to repeated cutaneous allergic reactions, had to be switched to miglustat after several attempts with enzyme replacement therapy. Despite many attempts, desensitization treatment did not result in improved toleration of imiglucerase infusions, and the patient became unwilling to continue with any intravenous enzyme replacement therapy. He subsequently agreed to switch to oral substrate reduction therapy with miglustat 100 mg twice daily titrated up to 100 mg three times daily over a short period. Long-term miglustat treatment maintained both hemoglobin and platelet levels within acceptable ranges over 8 years. The patient's spleen volume decreased, his plasma chitotriosidase levels stayed at reduced levels, and his bone mineral density findings have remained stable throughout follow-up. The patient's quality of life has remained satisfactory. Miglustat showed good gastrointestinal tolerability in this patient, and no

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

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    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.

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

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    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.

  10. COMPARING THE ENZYME REPLACEMENT THERAPY COST IN POST PANCREATECTOMY PATIENTS DUE TO PANCREATIC TUMOR AND CHRONIC PANCREATITIS

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    Anna Victoria FRAGOSO

    Full Text Available ABSTRACT Background - Among late postoperative complications of pancreatectomy are the exocrine and endocrine pancreatic insufficiencies. The presence of exocrine pancreatic insufficiency imposes, as standard treatment, pancreatic enzyme replacement. Patients with chronic pancreatitis, with intractable pain or any complications with surgical treatment, are likely to present exocrine pancreatic insufficiency or have this condition worsened requiring adequate dose of pancreatic enzymes. Objective - The aim of this study is to compare the required dose of pancreatic enzyme and the enzyme replacement cost in post pancreatectomy patients with and without chronic pancreatitis. Methods - Observational cross-sectional study. In the first half of 2015 patients treated at the clinic of the Department of Gastrointestinal Surgery at Hospital das Clínicas, Universidade de São Paulo, Brazil, who underwent pancreatectomy for at least 6 months and in use of enzyme replacement therapy were included in this series. The study was approved by the Research Ethics Committee. The patients were divided into two groups according to the presence or absence of chronic pancreatitis prior to pancreatic surgery. For this study, P<0.05 was considered statistically significant. Results - The annual cost of the treatment was R$ 2150.5 ± 729.39; R$ 2118.18 ± 731.02 in patients without pancreatitis and R$ 2217.74 ± 736.30 in patients with pancreatitis. Conclusion - There was no statistically significant difference in the cost of treatment of enzyme replacement post pancreatectomy in patients with or without chronic pancreatitis prior to surgical indication.

  11. Cost-effectiveness of enzyme replacement therapy with alglucosidase alfa in classic-infantile patients with Pompe disease

    NARCIS (Netherlands)

    T.A. Kanters (Tim A.); I Hoogenboom-Plug (Iris); M.P.M.H. Rutten-van Mölken (Maureen); W.K. Redekop (Ken); A.T. van der Ploeg (Ans); L. van Hakkaart-van Roijen (Leona)

    2014-01-01

    textabstractBackground: Infantile Pompe disease is a rare metabolic disease. Patients generally do not survive the first year of life. Enzyme replacement therapy (ERT) has proven to have substantial effects on survival in infantile Pompe disease. However, the costs of therapy are very high. In this

  12. 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.

  13. A distinct urinary biomarker pattern characteristic of female Fabry patients that mirrors response to enzyme replacement therapy.

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    Andreas D Kistler

    Full Text Available Female patients affected by Fabry disease, an X-linked lysosomal storage disorder, exhibit a wide spectrum of symptoms, which renders diagnosis, and treatment decisions challenging. No diagnostic test, other than sequencing of the alpha-galactosidase A gene, is available and no biomarker has been proven useful to screen for the disease, predict disease course and monitor response to enzyme replacement therapy. Here, we used urine proteomic analysis based on capillary electrophoresis coupled to mass spectrometry and identified a biomarker profile in adult female Fabry patients. Urine samples were taken from 35 treatment-naïve female Fabry patients and were compared to 89 age-matched healthy controls. We found a diagnostic biomarker pattern that exhibited 88.2% sensitivity and 97.8% specificity when tested in an independent validation cohort consisting of 17 treatment-naïve Fabry patients and 45 controls. The model remained highly specific when applied to additional control patients with a variety of other renal, metabolic and cardiovascular diseases. Several of the 64 identified diagnostic biomarkers showed correlations with measures of disease severity. Notably, most biomarkers responded to enzyme replacement therapy, and 8 of 11 treated patients scored negative for Fabry disease in the diagnostic model. In conclusion, we defined a urinary biomarker model that seems to be of diagnostic use for Fabry disease in female patients and may be used to monitor response to enzyme replacement therapy.

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

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    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.

  15. 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

  16. Enzyme Replacement Therapy for Fabry Disease

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    Maria Dolores Sanchez-Niño PhD

    2016-11-01

    Full Text Available Fabry disease is a rare X-linked disease caused by the deficiency of α-galactosidase that leads to the accumulation of abnormal glycolipid. Untreated patients develop potentially lethal complications by age 30 to 50 years. Enzyme replacement therapy is the current standard of therapy for Fabry disease. Two formulations of recombinant human α-galactosidase A (agalsidase are available in most markets: agalsidase-α and agalsidase-β, allowing a choice of therapy. However, the US Food and Drug Administration rejected the application for commercialization of agalsidase-α. The main difference between the 2 enzymes is the dose. The label dose for agalsidase-α is 0.2 mg/kg/2 weeks, while the dose for agalsidase-β is 1.0 mg/kg/2 weeks. Recent evidence suggests a dose-dependent effect of enzyme replacement therapy and agalsidase-β is 1.0 mg/kg/2 weeks, which has been shown to reduce the occurrence of hard end points (severe renal and cardiac events, stroke, and death. In addition, patients with Fabry disease who have developed tissue injury should receive coadjuvant tissue protective therapy, together with enzyme replacement therapy, to limit nonspecific progression of the tissue injury. It is likely that in the near future, additional oral drugs become available to treat Fabry disease, such as chaperones or substrate reduction therapy.

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

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    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.

  18. 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...

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

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    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.

  20. 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...

  1. 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

  2. The use of port-a-caths in adult patients with Lysosomal Storage Disorders receiving Enzyme Replacement Therapy-one centre experience

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    Mairead McLoughlin

    2017-12-01

    Full Text Available Port-a-cath is a widely used device in patients with long-term venous access demand such as frequent or continuous administration of medications such as Enzyme Replacement Therapy (ERT, chemotherapy delivery, blood transfusions, blood products, and fluids. Patients with Lysosomal Storage Diseases (LSDs often require recurrent courses of ERT. We reviewed our experience of using port-a-caths in patients with LSDs with the focus on challenges and complications associated with these catheters. Among 245 adult patients who were treated with ERT, twenty patients (8.2% had a port-a-cath inserted due to poor venous access. Six patients were using their first port whereas five other patients had their port-a-caths replaced at least once. The remaining six patients had inactive port-a-caths. The majority of patients with active port-a-caths never missed more than one consecutive infusion, although one patient missed 2 consecutive infusions whilst on holiday. We identified significant gaps in patients' and their families' understanding of the management of port-a-caths and risks associated with them. It resulted in producing a leaflet and designing an educational program for our LSD patients.

  3. 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.

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

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    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.

  5. Model for Quantitative Evaluation of Enzyme Replacement Treatment

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    Radeva B.

    2009-12-01

    Full Text Available Gaucher disease is the most frequent lysosomal disorder. Its enzyme replacement treatment was the new progress of modern biotechnology, successfully used in the last years. The evaluation of optimal dose of each patient is important due to health and economical reasons. The enzyme replacement is the most expensive treatment. It must be held continuously and without interruption. Since 2001, the enzyme replacement therapy with Cerezyme*Genzyme was formally introduced in Bulgaria, but after some time it was interrupted for 1-2 months. The dose of the patients was not optimal. The aim of our work is to find a mathematical model for quantitative evaluation of ERT of Gaucher disease. The model applies a kind of software called "Statistika 6" via the input of the individual data of 5-year-old children having the Gaucher disease treated with Cerezyme. The output results of the model gave possibilities for quantitative evaluation of the individual trends in the development of the disease of each child and its correlation. On the basis of this results, we might recommend suitable changes in ERT.

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

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    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.

  7. 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.

  8. 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

  9. Enzyme replacement and substrate reduction therapy for Gaucher disease.

    Science.gov (United States)

    Shemesh, Elad; Deroma, Laura; Bembi, Bruno; Deegan, Patrick; Hollak, Carla; Weinreb, Neal J; Cox, Timothy M

    2015-03-27

    adverse events were reported, nearly all deemed unrelated to treatment.There are, as yet, no randomized trials of substrate reduction therapy in treatment-naïve patients that can be evaluated. Miglustat monotherapy appeared as effective as continued enzyme replacement therapy for maintenance of hematological, organ and biomarker responses in people with type 1 Gaucher disease previously treated with imiglucerase for at least two years. In those with neuronopathic Gaucher disease, no significant improvements in haemoglobin concentration, platelet count or organ volumes occurred when enzyme replacement therapy was augmented with miglustat.One randomized controlled study assessing substrate reduction therapy was published immediately prior to producing the final version of this review, and this, along with a further ongoing study (expected to be published in the near future), will be assessed for eligibility in a future update of the review. The results reflect the limitations of analysing evidence restricted to prospective randomized controlled trials, especially when dealing with chronic rare diseases. This analysis suggests that, during the first year of treatment, different recombinant glucocerebrosidases are bio-similar and non-inferior in safety and efficacy for surrogate biological response parameters. Enzyme replacement therapy given at 30 to 45 units/kg body weight every two to four weeks was generally as effective as the 60 unit/kg dose for the assessed clinical outcomes. The analysis emphasise the need to determine whether it is realistic to carry out multi-decade prospective clinical trials for rare diseases such as type 1 Gaucher disease. With large treatment effects on the classical manifestations of the disorder, therapeutic investigations in Gaucher disease mandate innovative trial designs and methodology to secure decisive data concerning long-term efficacy and safety - with the realization that knowledge about disease-modifying actions that are sustained

  10. Ophthalmic experience over 10 years in an observational nationwide Danish cohort of Fabry patients with access to enzyme replacement

    DEFF Research Database (Denmark)

    Fledelius, Hans C.; Sandfeld, Lisbeth; Rasmussen, Åse Krogh

    2015-01-01

    , respectively, and phlebopathy in 22, although generally without evidence of loss of vessel wall integrity. Systemic vascular lesions with or without loss of function were recorded for kidney (n = 23), heart (n = 17) and brain (n = 7), and an association was suggested between nephropathy and abnormal morphology...... parameters in 37. Compared to other Fabry series, two of 39 patients with serious unilateral occlusive retinal disease may appear a high number. The presence of retinal tortuosity is discussed, possibly reflecting haemodynamic events related to vessel wall deposits, but could also be 'constitutional...

  11. Cerebral magnetic resonance findings during enzyme replacement therapy in mucopolysaccharidosis

    International Nuclear Information System (INIS)

    Matsubara, Yoshiko; Miyazaki, Osamu; Nosaka, Shunsuke; Kosuga, Motomichi; Okuyama, Torayuki

    2017-01-01

    Although enzyme replacement therapy (ERT) is an effective treatment for mucopolysaccharidosis (MPS) types I, II, IVA and VI, its effectiveness in children with central nervous system (CNS) disorders is said to be poor because the blood-brain barrier cannot be penetrated by ERT drugs. To assess CNS involvement in mucopolysaccharidosis at the start of enzyme replacement therapy and to investigate the time course of ERT in the central nervous system. We performed brain MRI in 17 children and young adults who underwent ERT. The clinical severity was classified as attenuated or severe by a specialist pediatrician, based on the clinical symptoms and genotypes. At the start of ERT, we scored nine parameters using two- or three-point scales based on the severity of the disease revealed on MRI scans. After the start of ERT, we compared the initial and follow-up MRI scans, and classified the findings as no change, improved or worse. We then compared the results with the changes in clinical findings. At the start of ERT, comparison of the clinical symptoms and image scores revealed differences between severe and attenuated mucopolysaccharidosis. The scores in patients with severe MPS ranged from 9 to 16 (mean 12.2); for patients with attenuated MPS, they ranged from 2 to 11 (mean 6.4). Images of the four patients with severe MPS showed ventricular dilation and brain atrophy. Such findings were made in only 2 of 13 patients with attenuated MPS. The results after the start of ERT showed that 11/17 (65%) patients manifested improvement or no change. All five patients with MPS I experienced improvement in some regions. There were no new lesions. One patient with MPS II experienced worsening of his CNS symptoms, and his MRI findings revealed more severe ventricular dilation, brain atrophy and white matter lesions. Ventricular dilation and brain atrophy on imaging studies might represent useful markers in predicting the severity of mucopolysaccharidosis and worsening of CNS

  12. Cerebral magnetic resonance findings during enzyme replacement therapy in mucopolysaccharidosis

    Energy Technology Data Exchange (ETDEWEB)

    Matsubara, Yoshiko [Division of Radiology, National Center for Child Health and Development, Tokyo (Japan); Hiroshima University, Department of Diagnostic Radiology, Hiroshima (Japan); Miyazaki, Osamu; Nosaka, Shunsuke [Division of Radiology, National Center for Child Health and Development, Tokyo (Japan); Kosuga, Motomichi [Division of Medical Genetics, National Center for Child Health and Development, Tokyo (Japan); Okuyama, Torayuki [Department of Clinical Laboratory Medicine, National Center for Child Health and Development, Tokyo (Japan)

    2017-11-15

    Although enzyme replacement therapy (ERT) is an effective treatment for mucopolysaccharidosis (MPS) types I, II, IVA and VI, its effectiveness in children with central nervous system (CNS) disorders is said to be poor because the blood-brain barrier cannot be penetrated by ERT drugs. To assess CNS involvement in mucopolysaccharidosis at the start of enzyme replacement therapy and to investigate the time course of ERT in the central nervous system. We performed brain MRI in 17 children and young adults who underwent ERT. The clinical severity was classified as attenuated or severe by a specialist pediatrician, based on the clinical symptoms and genotypes. At the start of ERT, we scored nine parameters using two- or three-point scales based on the severity of the disease revealed on MRI scans. After the start of ERT, we compared the initial and follow-up MRI scans, and classified the findings as no change, improved or worse. We then compared the results with the changes in clinical findings. At the start of ERT, comparison of the clinical symptoms and image scores revealed differences between severe and attenuated mucopolysaccharidosis. The scores in patients with severe MPS ranged from 9 to 16 (mean 12.2); for patients with attenuated MPS, they ranged from 2 to 11 (mean 6.4). Images of the four patients with severe MPS showed ventricular dilation and brain atrophy. Such findings were made in only 2 of 13 patients with attenuated MPS. The results after the start of ERT showed that 11/17 (65%) patients manifested improvement or no change. All five patients with MPS I experienced improvement in some regions. There were no new lesions. One patient with MPS II experienced worsening of his CNS symptoms, and his MRI findings revealed more severe ventricular dilation, brain atrophy and white matter lesions. Ventricular dilation and brain atrophy on imaging studies might represent useful markers in predicting the severity of mucopolysaccharidosis and worsening of CNS

  13. JCL Roundtable: enzyme replacement therapy for lipid storage disorders.

    Science.gov (United States)

    Brown, W Virgil; Desnick, Robert J; Grabowski, Gregory A

    2014-01-01

    There are several inherited disorders that involve abnormal storage of lipids in tissues leading to severe compromise of organs. Sadly, these are often accompanied by lifelong morbidity and early mortality. Disorders such as Gaucher, Fabry, and lysosomal acid lipase deficiencies (Wolman and cholesteryl ester storage diseases) have been known for many years, and provide a difficult and frustrating set of problems for patients, their families, and their physicians. With recombinant methods of protein synthesis, it is now possible to literally replace the defective enzymes that underlie the basic pathophysiology of many such disorders. The delivery of these enzymes into the affected cells is possible because of their location in the lysosomes where the natural degradation of their lipid substrates occurs. I have asked 2 well-known investigators to join us for this Roundtable. These are professors who have been involved with the research that has made this type of therapy possible and who have participated in the clinical trials that demonstrated the value of enzyme replacement therapy. They are Dr. Robert Desnick, dean of Genetic and Genomic Medicine and professor and chairman emeritus of the Department of Genetics and Genomic Sciences at the Icahn School of Medicine at Mount Sinai in New York City, and Dr. Gregory Grabowski, professor of Microbiology, Biochemistry, and Pediatrics, at the University of Cincinnati College of Medicine. Dr. Grabowski recently retired from that school to become the chief science officer of Synageva, a company involved in producing enzymes for this type of therapy. Copyright © 2014 National Lipid Association. Published by Elsevier Inc. All rights reserved.

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. Cognitive Development in Infantile-Onset Pompe Disease Under Very Early Enzyme Replacement Therapy.

    Science.gov (United States)

    Lai, Chih-Jou; Hsu, Ting-Rong; Yang, Chia-Feng; Chen, Shyi-Jou; Chuang, Ya-Chin; Niu, Dau-Ming

    2016-12-01

    Most patients with infantile-onset Pompe disease die in early infancy before beginning enzyme replacement therapy, which has made it difficult to evaluate the impact of Pompe disease on cognitive development. Patients with infantile-onset Pompe disease can survive with enzyme replacement therapy, and physicians can evaluate cognitive development in these patients. We established an effective newborn screening program with quick clinical diagnostic criteria. Cognitive and motor development were evaluated using the Bayley Scales of Infant and Toddler Development-Third Edition at 6, 12, and 24 months of age. The patients who were treated very early demonstrate normal cognitive development with no significant change in cognition during this period (P = .18 > .05). The cognitive development was positively correlated with motor development (r = 0.533, P = .011). The results indicated that very early enzyme replacement therapy could protect cognitive development in patients with infantile-onset Pompe disease up to 24 months of age. © The Author(s) 2016.

  19. 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.

  20. 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 ...

  1. 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

  2. Engineering of GlcNAc-1-Phosphotransferase for Production of Highly Phosphorylated Lysosomal Enzymes for Enzyme Replacement Therapy.

    Science.gov (United States)

    Liu, Lin; Lee, Wang-Sik; Doray, Balraj; Kornfeld, Stuart

    2017-06-16

    Several lysosomal enzymes currently used for enzyme replacement therapy in patients with lysosomal storage diseases contain very low levels of mannose 6-phosphate, limiting their uptake via mannose 6-phosphate receptors on the surface of the deficient cells. These enzymes are produced at high levels by mammalian cells and depend on endogenous GlcNAc-1-phosphotransferase α/β precursor to phosphorylate the mannose residues on their glycan chains. We show that co-expression of an engineered truncated GlcNAc-1-phosphotransferase α/β precursor and the lysosomal enzyme of interest in the producing cells resulted in markedly increased phosphorylation and cellular uptake of the secreted lysosomal enzyme. This method also results in the production of highly phosphorylated acid β-glucocerebrosidase, a lysosomal enzyme that normally has just trace amounts of this modification.

  3. Pancreatic enzyme replacement therapy for people with cystic fibrosis.

    Science.gov (United States)

    Somaraju, Usha Rani; Solis-Moya, Arturo

    2016-11-23

    Most people with cystic fibrosis (80% to 90%) need pancreatic enzyme replacement therapy to prevent malnutrition. Enzyme preparations need to be taken whenever food is taken, and the dose needs to be adjusted according to the food consumed. A systematic review on the efficacy and safety of pancreatic enzyme replacement therapy is needed to guide clinical practice, as there is variability between centres with respect to assessment of pancreatic function, time of commencing treatment, dose and choice of supplements. This is an updated version of a published review. To evaluate the efficacy and safety of pancreatic enzyme replacement therapy in children and adults with cystic fibrosis and to compare the efficacy and safety of different formulations of this therapy and their appropriateness in different age groups. Also, to compare the effects of pancreatic enzyme replacement therapy in cystic fibrosis according to different diagnostic subgroups (e.g. different ages at introduction of therapy and different categories of pancreatic function). We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Most recent search: 15 July 2016.We also searched an ongoing trials website and the websites of the pharmaceutical companies who manufacture pancreatic enzyme replacements for any additional trials. Most recent search: 22 July 2016. Randomised and quasi-randomised controlled trials in people of any age, with cystic fibrosis and receiving pancreatic enzyme replacement therapy, at any dosage and in any formulation, for a period of not less than four weeks, compared to placebo or other pancreatic enzyme replacement therapy preparations. Two authors independently assessed trials and extracted outcome data. They also assessed the risk of bias of the trials included in the review. One

  4. 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.

  5. 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

  6. 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

  7. 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, ...

  8. Enzyme replacement prevents enamel defects in hypophosphatasia mice

    Science.gov (United States)

    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

  9. Effect of enzyme replacement therapy on isokinetic strength for all major muscle groups in four patients with Pompe disease-a long-term follow-up

    DEFF Research Database (Denmark)

    Andreassen, Christer Swan; Schlütter, Jacob Mørup; Vissing, John

    2014-01-01

    Pompe disease is a rare, inherited metabolic myopathy characterized by progressive weakness of the proximal limb and respiratory muscles. We report the findings from four patients with late-onset Pompe disease treated with α-glucosidase (Myozyme) for 2 (n=2) and 6 (n=2) years, and monitored with ...

  10. 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.

    Science.gov (United States)

    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.

  11. 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

  12. 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

  13. 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...

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

    Science.gov (United States)

    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/.

  15. 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

  16. 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

  17. 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

  18. 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

  19. 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

  20. 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...

  1. 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.

  2. 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.

  3. 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.

  4. 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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  8. 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

  9. 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.

  10. 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.

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

    Science.gov (United States)

    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.

  12. 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

  13. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. The metabolic consequences of thyroxine replacement in adult hypopituitary patients

    DEFF Research Database (Denmark)

    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...

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

    Science.gov (United States)

    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

  17. 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...

  18. 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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  2. 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

  3. 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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  5. Replacement

    Directory of Open Access Journals (Sweden)

    S. Radhakrishnan

    2014-03-01

    Full Text Available The fishmeal replaced with Spirulina platensis, Chlorella vulgaris and Azolla pinnata and the formulated diet fed to Macrobrachium rosenbergii postlarvae to assess the enhancement ability of non-enzymatic antioxidants (vitamin C and E, enzymatic antioxidants (superoxide dismutase (SOD and catalase (CAT and lipid peroxidation (LPx were analysed. In the present study, the S. platensis, C. vulgaris and A. pinnata inclusion diet fed groups had significant (P < 0.05 improvement in the levels of vitamins C and E in the hepatopancreas and muscle tissue. Among all the diets, the replacement materials in 50% incorporated feed fed groups showed better performance when compared with the control group in non-enzymatic antioxidant activity. The 50% fishmeal replacement (best performance diet fed groups taken for enzymatic antioxidant study, in SOD, CAT and LPx showed no significant increases when compared with the control group. Hence, the present results revealed that the formulated feed enhanced the vitamins C and E, the result of decreased level of enzymatic antioxidants (SOD, CAT and LPx revealed that these feeds are non-toxic and do not produce any stress to postlarvae. These ingredients can be used as an alternative protein source for sustainable Macrobrachium culture.

  6. 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.

  7. Total knee replacement in patients with diffuse villonodular synovitis

    Directory of Open Access Journals (Sweden)

    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.,

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

    Science.gov (United States)

    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.

  9. 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

  10. 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

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

    Science.gov (United States)

    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

  12. 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

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

    Science.gov (United States)

    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.

  14. 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.

  15. Functional assessment of patients after total knee replacement

    Directory of Open Access Journals (Sweden)

    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.

  16. 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

  17. 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

  18. Agreement between the results of meta-analyses from case reports and from clinical studies regarding the efficacy of laronidase therapy in patients with mucopolysaccharidosis type I who initiated enzyme replacement therapy in adult age: An example of case reports meta-analyses as an useful tool for evidence-based medicine in rare diseases.

    Science.gov (United States)

    Sampayo-Cordero, Miguel; Miguel-Huguet, Bernat; Pardo-Mateos, Almudena; Moltó-Abad, Marc; Muñoz-Delgado, Cecilia; Pérez-López, Jordi

    2018-02-01

    Case reports might have a prominent role in the rare diseases field, due to the small number of patients affected by one such disease. A previous systematic review regarding the efficacy of laronidase therapy in patients with mucopolysaccharidosis type I (MPS-I) who initiated enzyme replacement therapy (ERT) in adult age has been published. The review included a meta-analysis of 19 clinical studies and the description of eleven case reports. It was of interest to perform a meta-analysis of those case reports to explore the role of such meta-analyses as a tool for evidence-based medicine in rare diseases. The study included all case reports with standard treatment regimen. Primary analysis was the percentage of case reports showing an improvement in a specific outcome. Only when that percentage was statistically higher than 5%, the improvement was confirmed as such. The outcomes that accomplished this criterion were ranked and compared to the GRADE criteria obtained by those same outcomes in the previous meta-analysis of clinical studies. There were three outcomes that had a significant improvement: Urine glycosaminoglycans, liver volume and 6-minute walking test. Positive and negative predictive values, sensitivity and specificity for the results of the meta-analysis of case reports as compared to that of clinical studies were 100%, 88.9%, 75% and 100%, respectively. Accordingly, absolute (Rho=0.82, 95%CI: 0.47 to 0.95) and relative agreement (Kappa=0.79, 95%CI: 0.593 to 0.99) between the number of case reports with improvement in a specific outcome and the GRADE evidence score for that outcome were good. Sensitivity analysis showed that agreement between the meta-analysis of case reports and that of the clinical studies were good only when using a strong confirmatory strategy for outcome improvement in case reports. We found an agreement between the results of meta-analyses from case reports and from clinical studies in the efficacy of laronidase therapy in

  19. 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.

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

    Science.gov (United States)

    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

  1. Enzyme

    Science.gov (United States)

    Enzymes are complex proteins that cause a specific chemical change in all parts of the body. For ... use them. Blood clotting is another example of enzymes at work. Enzymes are needed for all body ...

  2. Aortic valve replacement in a patient with severe nickel allergy.

    Science.gov (United States)

    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.

  3. Survival, Quality of Life and Effects of Enzyme Replacement Therapy in Adults with Pompe Disease

    NARCIS (Netherlands)

    D. Güngör (Deniz)

    2013-01-01

    textabstractPompe disease, or glycogen storage disorder type II, is a rare inherited metabolic disorder caused by deficiency of the lysosomal enzyme acid α-glucosidase. This results in accumulation of glycogen in cells throughout the body, particularly muscle cells. The disease presents

  4. 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.

  5. Avaliação de dois anos de tratamento da doença de Gaucher tipo 1 com terapia de reposição enzimática em pacientes do estado de São Paulo, Brasil Evaluation of two years of treatment with enzyme replacement therapy in type 1 Gaucher disease patients of São Paulo State, Brazil

    Directory of Open Access Journals (Sweden)

    Elisa A. P. Sobreira

    2008-01-01

    marrow. The symptoms are heterogeneous and include hepatosplenomegaly, anemia, thrombocytopenia, bone marrow infiltration, and skeletal lesions. We evaluated the effects of enzyme replacement therapy (ERT on anemia, thrombocytopenia, hepatosplenomegaly, growth retardation (children and adolescents and bone pain among 90 patients with type 1 Gaucher's disease treated during 24 months in Sao Paulo State. Baseline signs and symptoms were anemia (50%, thrombocytopenia (59%, hepatomegaly (97%, splenomegaly (96%, growth retardation (46% and bone pain (62%. The mean dose of ERT was 35 U/kg every two weeks. Reduction in anemia, thrombocytopenia and bone pain was greatest during the first six months of ERT and of hepatosplenomegaly and growth retardation within 18 months. Improvements were sustained by most of the patients during the follow-up. Within 24 months of ERT, therapeutic goals were achieved in at least 88% of patients with anemia, 80% with thrombocytopenia, 34% with hepatosplenomegaly, 77% with growth retardation and 76% with bone pain. Other studies are needed to evaluate the treatment efficacy over a longer period, mainly for skeletal lesions and growth retardation, which may present a slower recovery due to low penetration of imiglucerase in bones.

  6. Survival, Quality of Life and Effects of Enzyme Replacement Therapy in Adults with Pompe Disease

    OpenAIRE

    Güngör, Deniz

    2013-01-01

    textabstractPompe disease, or glycogen storage disorder type II, is a rare inherited metabolic disorder caused by deficiency of the lysosomal enzyme acid α-glucosidase. This results in accumulation of glycogen in cells throughout the body, particularly muscle cells. The disease presents with (progressive) muscle weakness and can hence be categorized as a lysosomal storage disorder, a glycogen storage disorder and also a neuromuscular disorder. Pompe disease was the first neuromuscular disorde...

  7. 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

  8. 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.

  9. 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.

  10. 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.

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

    Science.gov (United States)

    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.

  12. 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.

  13. 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

  14. 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.

  15. Enzyme replacement therapy in late-onset Pompe's disease : A three-year follow-up

    NARCIS (Netherlands)

    Winkel, LPF; Van den Hout, JMP; Kamphoven, JHJ; Disseldorp, JAM; Remmerswaal, M; Arts, WFM; Loonen, MCB; Vulto, AG; Van Doorn, PA; De Jong, G; Hop, W; Smit, GPA; Shapira, SK; Boer, MA; van Diggelen, OP; Reuser, AJJ; Van der Ploeg, AT

    Pompe's disease is an autosomal recessive myopathy. The characteristic lysosomal storage of glycogen is caused by acid et-glucosidase deficiency. Patients with late-onset Pompe's disease present with progressive muscle weakness also affecting pulmonary function. In search of a treatment, we

  16. Fabry disease under enzyme replacement therapy-new insights in efficacy of different dosages.

    Science.gov (United States)

    Krämer, Johannes; Lenders, Malte; Canaan-Kühl, Sima; Nordbeck, Peter; Üçeyler, Nurcan; Blaschke, Daniela; Duning, Thomas; Reiermann, Stefanie; Stypmann, Jörg; Brand, Stefan-Martin; Gottschling, Timo; Störk, Stefan; Wanner, Christoph; Sommer, Claudia; Brand, Eva; Weidemann, Frank

    2017-11-23

    Fabry patients on reduced dose of agalsidase-beta or after switch to agalsidase-alfa show a decline in estimated glomerular filtration rate (eGFR) and an increase of the Mainz Severity Score Index. In this prospective observational study, we assessed end-organ damage and clinical symptoms in 112 patients who had received agalsidase-beta (1.0 mg/kg) for >1 year, who were (i) non-randomly assigned to continue this treatment regime (regular-dose group, n = 37); (ii) received a reduced dose of agalsidase-beta and subsequent switch to agalsidase-alfa (0.2 mg/kg) or a direct switch to 0.2 mg/kg agalsidase-alfa (switch group, n = 38); or (iii) were re-switched to agalsidase-beta after receiving agalsidase-alfa for at least 12 months (re-switch group, n = 37) with a median follow-up of 53 (38-57) months. eGFR of patients in the regular-dose group remained stable. Patients in the switch group showed an annual eGFR loss of - 4.6  ±  9.1 mL/min/1.73 m2 (P risk 0.42; 95% confidence interval 0.19-0.93; P = 0.02). Lyso-Gb3 remained stable in the switch (P = 0.97) and the regular-dose (P = 0.48) groups, but decreased in the re-switch group after change of the therapy regimen (P < 0.05). After switch to agalsidase-alfa, Fabry patients experienced a continuous decline in eGFR, while this decline was attenuated in patients who were re-switched to agalsidase-beta. Decreasing lyso-Gb3 levels may indicate a better treatment response in the latter group. © The Author 2017. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

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

    Science.gov (United States)

    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.

  18. 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.

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

    Science.gov (United States)

    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.

  20. 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.

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

    Science.gov (United States)

    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.

  2. 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.

  3. 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

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

    DEFF Research Database (Denmark)

    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....

  5. 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

  6. 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....

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

    Science.gov (United States)

    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.

  8. 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

  9. 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.

    Science.gov (United States)

    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

  10. 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...

  11. 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....

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

    NARCIS (Netherlands)

    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

  13. 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.

  14. Joint replacement in human immunodeficiency virus-infected patients

    Directory of Open Access Journals (Sweden)

    Ta-I Wang

    2012-11-01

    Conclusion: HIV-positive patients can have excellent outcomes after undergoing various arthroplastic surgeries. This revelation, coupled with the advances in antiviral therapy that have helped to lengthen HIV patient lifespans, strongly suggests that these patients should receive arthroplastic surgery.

  15. 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...

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

    Science.gov (United States)

    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.

  17. 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).

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  20. Emergency heart valve replacement: an analysis of 170 patients.

    Science.gov (United States)

    Louw, J W; Kinsley, R H; Dion, R A; Colsen, P R; Girdwood, R W

    1980-05-01

    The results of 170 emergency heart valve procedures performed during a 4 1/2-year period were analyzed. Five pathological groups of patients were recognized: those with infective endocarditis (Group 1, 28 patients); acute rheumatic carditis (Group 2, 43 patients); previous valve operation (Group 3, 29 patients); acute-on-chronic cardiac disease (Group 4, 67 patients); and miscellaneous conditions (Group 5, 3 patients). Mitral, aortic, and multiple valve procedures were performed on 58, 65, and 44 patients, respectively. The most common functional lesion was regurgitation. Hospital mortality was highest in Groups 3 (34%) and 4 (31%). By contrast, among the hospital survivors, the highest rate of attrition was in Group 2. Myocardial failure was the predominat cause of death. In view of the hopeless prognosis without operation, the 52% overall 3-year actuarial survival is a gratifying salvage. Unnecessary procrastination can only jeopardize the prospects for surgical cure.

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

    Science.gov (United States)

    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

  2. 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)

  3. 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

  4. 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...

  5. 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...

  6. 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...

  7. Trends in Joint Replacement Surgery in Patients with Rheumatoid Arthritis.

    Science.gov (United States)

    Young, Bradley L; Watson, Shawna L; Perez, Jorge L; McGwin, Gerald; Singh, Jasvinder A; Ponce, Brent A

    2018-02-01

    This study analyzed trends in large total joint arthroplasties (TJA) and in the proportion of these procedures performed on patients with rheumatoid arthritis (RA). The US Nationwide Inpatient Sample (2002-2012) was used to identify the incidences of total shoulder (TSA), elbow (TEA), knee (TKA), hip (THA), and ankle (TAA) arthroplasty and the proportion of these performed with coexisting RA. The prevalence of RA among patients with TJA increased 3.0%. The prevalence of RA among cases of TEA and TSA decreased by 50% (p < 0.0001) and 18% (p = 0.0016), respectively; a 38.0% decrease occurred in the prevalence of RA among TAA (p = 0.06); and nonsignificant increases were seen among THA and TKA. The average age difference between RA and non-RA patients undergoing TJA narrowed by 2 years (p < 0.0001). There was a greater reduction in the proportion of TSA, TEA, and TAA groups among women with RA than men with RA. In the TSA and TEA groups, there was a reduction in the proportion of whites with RA, but not blacks. The proportion of privately insured TSA and TAA patients with RA decreased, while patients with RA undergoing TSA, TEA, or TAA who were receiving Medicaid (government medical insurance) remained relatively stable over time. The prevalence of RA has decreased among TSA and TEA patients. A nonsignificant decline occurred among TAA patients. The average age of TJA patients with RA is beginning to mirror those without RA. Sex ratios for TSA, TEA, and TAA patients are following a similar pattern. These results may be evidence of the success of modern RA treatment strategies.

  8. Pulmonary valve replacement in patients with corrected tetralogy of Fallot

    Directory of Open Access Journals (Sweden)

    Fotios M. Mitropoulos

    2017-06-01

    Results: There were 2 perioperative deaths (2%. One patient developed sternal dehiscence requiring rewiring. Median ICU and hospital stay was 1 and 7 days respectively. Postoperative echocardiography at 6 and 12 months showed excellent bioprosthetic valve performance, significant decrease in size of the right cardiac chambers and reduction of tricuspid regurgitation (TR in the majority of the patients. At mean follow-up of 3.6 ± 2 years, all surviving patients remain in excellent clinical condition. Conclusion: Probability of reoperation for pulmonary insufficiency in patients with surgically corrected TOF increases with time and timely PVR by preventing the development of right heart failure is crucial for long-term survival. Current bioprosthetic valve technology in combination with the beating heart technique provides excellent immediate and short-term results. Further follow-up is necessary to evaluate long-term outcome.

  9. 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...

  10. Should patients with Björk-Shiley valves undergo prophylactic replacement?

    Science.gov (United States)

    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

  11. Patient prosthesis mismatch after aortic valve replacement: An Indian perspective

    Directory of Open Access Journals (Sweden)

    Shreedhar S Joshi

    2016-01-01

    Full Text Available Context: Perioperative period. Aims: Occurrence of PPM after AVR, factors associated with PPM, impact on mortality. Settings and Design: Teritary Care Referral Cardiac Centre. Materials and Methods: A retrospective analysis of AVR procedures at a single centre over 4 years was conducted. Demographic, echocardiographic and outcome data were collected from institute database. Rahimtoola criteria of indexed effective orifice area (iEOA were used to stratify patients into PPM categories. Patients with and without PPM were compared for associated factors. Statistical Analysis Used: Independent t-test, chi-square test, logistic regression analysis, ROC-AUC, Youden index. Results: 606 patients with complete data were analysed for PPM. The incidence of mild, moderate and severe PPM was 6.1% (37, 2.5% (15 and 0.5% (3 respectively. There was no impact of PPM on all-cause in-hospital mortality. PPM was observed more with Aortic Stenosis (AS compared to Aortic Regurgitation (AR as etiology. Aortic annulus indexed to BSA (iAA had a very good predictive ability for PPM at <16mm/m 2 BSA. Conclusions: PPM has lower incidence after AVR in this Indian population and does not increase early mortality. Patients with AS and iAA<16mm/m2BSA should be cautiously dealt with to prevent PPM.

  12. 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.

  13. Counseling patients on facial volume replacement and adherence with posttreatment instructions

    Directory of Open Access Journals (Sweden)

    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

  14. 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)

  15. Testosterone Replacement Therapy and Polycythemia in HIV-infected Patients

    Science.gov (United States)

    Vorkas, Charles Kyriakos; Vaamonde, Carlos M.; Glesby, Marshall J.

    2013-01-01

    We conducted a case-control study to assess testosterone use as a primary risk factor for polycythemia in 21 HIV-infected men. Any testosterone use within two months of first elevated hemoglobin was associated with polycythemia (matched odds ratio 6.55; 95% CI 1.83-23.4; P=0.004) and intramuscular administration demonstrated a stronger association than topical use. No adverse cardiovascular or thrombotic events were observed. HIV-infected patients taking testosterone should undergo routine hematologic monitoring with adjustment of therapy when appropriate. PMID:22008652

  16. 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.

  17. Postoperative Recovery of Mechanical Muscle Function in Hip Replacement Patients

    DEFF Research Database (Denmark)

    Jensen, Carsten; Aagaard, Per; Overgaard, Søren

    2011-01-01

    the posterior-lateral approach. Prior to surgery no training program was initiated but the patients were encouraged to live as usual. Post surgery all patients were allowed fully weight-bearing and they were instructed to follow a conventional home-based rehabilitation, but were otherwise not engaged in any......-test for between group comparisons while ANOVA was used for repeated measures for comparisons over time (α=0.05)   RESULTS The results were calculated as deficits in percentages of the unaffected side (A-NA/NA)*100)) in order to evaluate degree of asymmetry. Our overall side-to-side deficits for peak torque ranged...... from 32.6 to 0.4% and hip flexion deficit being significantly more impacted then the other muscle groups (32.6%).  At baseline all muscle groups showed a significant torque deficit. At 8 weeks post surgery that asymmetry had increased for 4 out of 6 muscle groups. At 26 weeks the hip adduction and hip...

  18. 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.

  19. 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...

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

    Directory of Open Access Journals (Sweden)

    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.

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

    DEFF Research Database (Denmark)

    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...

  2. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  5. 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.

  6. 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.

  7. 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...

  8. 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.

  9. 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...

  10. 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.

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

    Science.gov (United States)

    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.

  12. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. 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.

  17. 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

  18. 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:

  19. 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...

  20. 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

  1. 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...

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

    Science.gov (United States)

    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.

  3. 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....

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

    DEFF Research Database (Denmark)

    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...

  5. 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.

  6. 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.

  7. 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...

  8. 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...

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

    Science.gov (United States)

    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.

  10. 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.

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

    Science.gov (United States)

    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.

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

    DEFF Research Database (Denmark)

    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....

  13. [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.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. Angiotensin Converting Enzyme Gene Insertion/Deletion Polymorphism in Migraine Patients

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

    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

  17. 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

  18. 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.

  19. 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.

  20. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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....

  3. 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.

  4. 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...

  5. 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...

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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.

  9. 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.

  10. Intra-articular enzyme replacement therapy with rhIDUA is safe, well-tolerated, and reduces articular GAG storage in the canine model of mucopolysaccharidosis type I.

    Science.gov (United States)

    Wang, Raymond Y; Aminian, Afshin; McEntee, Michael F; Kan, Shih-Hsin; Simonaro, Calogera M; Lamanna, William C; Lawrence, Roger; Ellinwood, N Matthew; Guerra, Catalina; Le, Steven Q; Dickson, Patricia I; Esko, Jeffrey D

    2014-08-01

    Treatment with intravenous enzyme replacement therapy and hematopoietic stem cell transplantation for mucopolysaccharidosis (MPS) type I does not address joint disease, resulting in persistent orthopedic complications and impaired quality of life. A proof-of-concept study was conducted to determine the safety, tolerability, and efficacy of intra-articular recombinant human iduronidase (IA-rhIDUA) enzyme replacement therapy in the canine MPS I model. Four MPS I dogs underwent monthly rhIDUA injections (0.58 mg/joint) into the right elbow and knee for 6 months. Contralateral elbows and knees concurrently received normal saline. No intravenous rhIDUA therapy was administered. Monthly blood counts, chemistries, anti-rhIDUA antibody titers, and synovial fluid cell counts were measured. Lysosomal storage of synoviocytes and chondrocytes, synovial macrophages and plasma cells were scored at baseline and 1 month following the final injection. All injections were well-tolerated without adverse reactions. One animal required prednisone for spinal cord compression. There were no clinically significant abnormalities in blood counts or chemistries. Circulating anti-rhIDUA antibody titers gradually increased in all dogs except the prednisone-treated dog; plasma cells, which were absent in all baseline synovial specimens, were predominantly found in synovium of rhIDUA-treated joints at study-end. Lysosomal storage in synoviocytes and chondrocytes following 6 months of IA-rhIDUA demonstrated significant reduction compared to tissues at baseline, and saline-treated tissues at study-end. Mean joint synovial GAG levels in IA-rhIDUA joints were 8.62 ± 5.86 μg/mg dry weight and 21.6 ± 10.4 μg/mg dry weight in control joints (60% reduction). Cartilage heparan sulfate was also reduced in the IA-rhIDUA joints (113 ± 39.5 ng/g wet weight) compared to saline-treated joints (142 ± 56.4 ng/g wet weight). Synovial macrophage infiltration, which was present in all joints at baseline, was

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

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.

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

    Science.gov (United States)

    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.

  14. 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.

  15. 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.

  16. 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.

  17. 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

  18. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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

  1. 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

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

    Science.gov (United States)

    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.

  3. 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.

  4. 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

  5. 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.

  6. 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

  7. 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

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

    Science.gov (United States)

    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.

  9. 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

  10. 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.

  11. 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)

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

    Directory of Open Access Journals (Sweden)

    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.

  13. 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...

  14. Effect of vitamin D replacement on immunological biomarkers in patients with multiple sclerosis.

    Science.gov (United States)

    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.

  15. Left bronchial artery arising from a replaced left hepatic artery in a patient with massive hemoptysis

    Energy Technology Data Exchange (ETDEWEB)

    Khil, Eun Kyung; Lee, Jae Myung [Dept. of Radiology, Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon (Korea, Republic of)

    2015-09-15

    A 70-year-old man with a 3-year history of bronchiectasis presented with massive hemoptysis that had lasted for 3 days. In our attempt to perform bronchial artery embolization, upper abdominal angiography was required to locate the left bronchial artery, which in this case was of anomalous origin, arising from a replaced left hepatic artery, which arose from the left gastric artery-a very unusual anatomical variant. We performed embolization with polyvinyl alcohol particles, and the patient's symptoms resolved completely, with no additional complications after conservative treatment.

  16. 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.

  17. Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

    Science.gov (United States)

    Cortez, Daniel; Barham, Waseem; Ruckdeschel, Emily; Sharma, Nandita; McCanta, Anthony C; von Alvensleben, Johannes; Sauer, William H; Collins, Kathryn K; Kay, Joseph; Patel, Sonali; Nguyen, Duy T

    2017-08-01

    Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias. A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort. We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015. History of AFL/IART was documented using electrocardiogram, Holter monitor, exercise stress test, implanted cardiac device, and electrophysiology study. P-R intervals, Pvm, QRS duration, and QRS vector magnitude were assessed from resting sinus-rhythm 12-lead electrocardiograms and identification of those with AFL/IART was determined. Fourteen patients (8.9%) were found to have AFL/IART. Pvm, QRS duration, and QRS vector magnitude significantly differentiated those with AFL/IART from those without on univariate analysis: 0.09 ± 0.04 vs 0.18 ± 0.07 mV, 161.3 ± 21.9 vs 137.7 ± 31.4 ms, and 1.2 (interquartile range, 1.0-1.2) vs 1.6 mV (1.0-2.3), respectively (P < 0.05 for each). The Pvm had the highest area under the ROC curve (0.88) and was the only significant predictor on multivariate analysis, with odds ratio of 0.02 (95% confidence interval: 0.01-0.53). P-R duration, MRI volumes, and right-heart hemodynamics did not significantly differentiate those with vs those without AFL/IART. In TOF patients undergoing pulmonary valve replacement, Pvm has significant value in predicting those with perioperative AFL/IART. These clinical features may help further evaluate TOF patients at risk for perioperative atrial arrhythmias. Prospective studies are warranted. © 2017 Wiley Periodicals, Inc.

  18. 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.

  19. Using continuous renal replacement therapy to manage patients of shock and acute renal failure

    Directory of Open Access Journals (Sweden)

    Soni Sachin

    2009-01-01

    Full Text Available Background: The incidence of acute renal failure (ARF in the hospital setting is increasing. It portends excessive morbidity and mortality and a considerable burden on hospital resources. Extracorporeal therapies show promise in the management of patients with shock and ARF. It is said that the potential of such therapy goes beyond just providing renal support. The aim of our study was to analyze the clinical setting and outcomes of critically ill ARF patients managed with continuous renal replacement therapy (CRRT. Patients and Methods: Ours was a retrospective study of 50 patients treated between January 2004 and November 2005. These 50 patients were in clinical shock and had concomitant ARF. All of these patients underwent CVVHDF (continuous veno-venous hemodiafiltration in the intensive care unit. For the purpose of this study, shock was defined as systolic BP < 100 mm Hg in spite of administration of one or more inotropic agents. SOFA (Sequential Organ Failure Assessment score before initiation of dialysis support was recorded in all cases. CVVHDF was performed using the Diapact ® (Braun CRRT machine. The vascular access used was as follows: femoral in 32, internal jugular in 8, arteriovenous fistula (AVF in 4, and subclavian in 6 patients. We used 0.9% or 0.45% (half-normal saline as a prefilter replacement, with addition of 10% calcium gluconate, magnesium sulphate, sodium bicarbonate, and potassium chloride in separate units, while maintaining careful monitoring of electrolytes. Anticoagulation of the extracorporeal circuit was achieved with systemic heparin in 26 patients; frequent saline flushes were used in the other 24 patients. Results: Of the 50 patients studied, 29 were males and 21 females (1.4:1. The average age was 52.88 years (range: 20-75 years. Causes of ARF included sepsis in 24 (48%, hemodynamically mediated renal failure (HMRF in 18 (36%, and acute over chronic kidney disease in 8 (16% patients. The overall mortality was

  20. [Application of enzymes for treatment of patients with hypertrophic cicatrices].

    Science.gov (United States)

    Paramonov, B A; Turkovskiĭ, I I; Bondarev, S V

    2007-01-01

    Treatment of 27 patients by electrophoresis with preparation "Polycollagenase K" was analyzed and it was shown that it gave positive results according to complex assessment by the scale developed by the authors.

  1. 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

  2. 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

  3. 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.

  4. Testosterone Replacement and Bone Mineral Density in Male Pituitary Tumor Patients

    Directory of Open Access Journals (Sweden)

    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.

  5. Replacing Escherichia coli NAD-dependent glyceraldehyde 3-phosphate dehydrogenase (GAPDH) with a NADP-dependent enzyme from Clostridium acetobutylicum facilitates NADPH dependent pathways.

    Science.gov (United States)

    Martínez, Irene; Zhu, Jiangfeng; Lin, Henry; Bennett, George N; San, Ka-Yiu

    2008-11-01

    Reactions requiring reducing equivalents, NAD(P)H, are of enormous importance for the synthesis of industrially valuable compounds such as carotenoids, polymers, antibiotics and chiral alcohols among others. The use of whole-cell biocatalysis can reduce process cost by acting as catalyst and cofactor regenerator at the same time; however, product yields might be limited by cofactor availability within the cell. Thus, our study focussed on the genetic manipulation of a whole-cell system by modifying metabolic pathways and enzymes to improve the overall production process. In the present work, we genetically engineered an Escherichia coli strain to increase NADPH availability to improve the productivity of products that require NADPH in its biosynthesis. The approach involved an alteration of the glycolysis step where glyceraldehyde-3-phosphate (GAP) is oxidized to 1,3 bisphophoglycerate (1,3-BPG). This reaction is catalyzed by NAD-dependent endogenous glyceraldehyde-3-phosphate dehydrogenase (GAPDH) encoded by the gapA gene. We constructed a recombinant E. coli strain by replacing the native NAD-dependent gapA gene with a NADP-dependent GAPDH from Clostridium acetobutylicum, encoded by the gene gapC. The beauty of this approach is that the recombinant E. coli strain produces 2 mol of NADPH, instead of NADH, per mole of glucose consumed. Metabolic flux analysis showed that the flux through the pentose phosphate (PP) pathway, one of the main pathways that produce NADPH, was reduced significantly in the recombinant strain when compared to that of the parent strain. The effectiveness of the NADPH enhancing system was tested using the production of lycopene and epsilon-caprolactone as model systems using two different background strains. The recombinant strains, with increased NADPH availability, consistently showed significant higher productivity than the parent strains.

  6. High prevalence of abnormal liver enzymes in South African patients ...

    African Journals Online (AJOL)

    Objective: To determine the prevalence of liver function test abnormalities in South African black and Indian adult patients with type 2 diabetes mellitus attending a tertiary diabetes clinic. iabetes clinic. Recorded data included the past medical and drug history, history of alcohol abuse, anthropometry, lipid profile and liver ...

  7. 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.

  8. Pharmaceutical intervention in menopausal patients with hormone replacement therapy in a community pharmacy from Antofagasta

    Directory of Open Access Journals (Sweden)

    Alejandrina Alucema

    2015-02-01

    Full Text Available Context: Hormone replacement therapy (HRT is the most widely used treatment for controlling the effects of menopause. This type of therapy causes some drug-related problems (DRP, which requires monitoring to control the negative effects and ensure patient adherence to therapy. Aims: Perform a pharmacotherapeutic monitoring and educate to menopausal patients in HRT of a community pharmacy from the city of Antofagasta. Methods: A 98-menopausal patients underwent a pharmaceutical intervention to identify the PRM and its resolution. It was applied to them a survey before and after educational activities about this disease and HRT to determine the knowledge on the subject. Results: During the pharmacotherapeutic monitoring was determined that 55% of patients using combined HRT. 62 DRPs were detected, of which 43 were resolved (69%; the most were Patient-Pharmacist (73%. The better resolution DRP were DRP 4(b “frequency of inadequate administration” and DRP 2(a “no medical indication”. At baseline, 90% had an inadequate level of knowledge about the disease and THR, 8% intermediate, and only 2% adequate. After the implementation of the education strategy, the level of knowledge increased, achieving at the end of the study only intermediate (10% and adequate (90% levels. Conclusions: The results confirm the importance of pharmaceutical intervention for the identification and resolution of DRP and the requirement to establish educational strategies to increase the knowledge about menopause and HRT in menopausal patients.

  9. 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.

  10. 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...

  11. Quality-adjusted life years gained in patients aged over 65 years after total hip replacement

    DEFF Research Database (Denmark)

    Hørdam, Britta; Pedersen, P.U.; Søballe, Kjeld

    2011-01-01

    Background: Total hip replacement (THR) is an effective, but also cost-intensive health care procedure for the elderly. Because of demographic changes in Western Europe, THR-associated financial investment for health care has become a question of priorities in society. To provide a quantitative...... rationale for a discussion within Western European health care systems, we undertook a prospective assessment of the benefit of THR from the patients´ perspective and as measured by quality-adjusted life years (QALYs). Aim: To measure the difference in health related quality of life between an intervention...... treatment and telephone intervention. QALYs were calculated from measures of health-related quality of life using questionnaire SF-36. These scores were transformed to QALYs using a formula based on the method developed by Brazier (Brazier 1998). Results: Both the control and the intervention patients...

  12. The dislocating hip replacement - revision with a dual mobility cup in 56 consecutive patients

    DEFF Research Database (Denmark)

    Jakobsen, Thomas; Kappel, Andreas; Hansen, Flemming

    2014-01-01

    INTRODUCTION: Recurrent dislocations of hip replacements are a difficult challenge. One treatment option for recurrent dislocations is the use of a dual mobility cup. The aim of this study was to retrospective investigate the effect of dual mobility cups as a treatment for recurrent dislocations...... with a Saturne dual mobility cup (Amplitude, Neyron, France). The mean follow-up period was 44 months (SD 30, range 0.1-119). RESULTS: One patient (1.8%) experienced a re-dislocation. Three patients (5.3%) had to be revised. One due to disintegration between the femoral head and inner shell, one due to loosening...... of the acetabular component, and one due to infection. Harris Hip Score improved from a mean of 76 before index surgery to 87 within one year after index surgery. CONCLUSION: This study advocates the use of a dual mobility cup for treatment of recurrent dislocations of THR. However, studies with a longer follow up...

  13. 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

  14. 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...... affective relations emerge and underexplored aspects of professional engagements appear. Through theories of affectivity, I propose the notion of touchability as a theoretical device which focuses attention on the body and the sensory, affective and embodied vicissitudes that traverse it, while nonetheless...... attending to the difference between feeling patients and plastic. Sensuous ethnography is the method used for exploring these professional engagements. By engaging through bodily senses, I actively use bodies as tools of inquiry and sources of knowledge within nursing. I argue that the notion...

  15. 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

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

    Science.gov (United States)

    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

  17. 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.

  18. 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.

  19. Postoperative cognitive dysfunction and its relationship to cognitive reserve in elderly total joint replacement patients.

    Science.gov (United States)

    Scott, J E; Mathias, J L; Kneebone, A C; Krishnan, J

    2017-06-01

    Whether total joint replacement (TJR) patients are susceptible to postoperative cognitive dysfunction (POCD) remains unclear due to inconsistencies in research methodologies. Moreover, cognitive reserve may moderate the development of POCD after TJR, but has not been investigated in this context. The current study investigated POCD after TJR, and its relationship with cognitive reserve, using a more rigorous methodology than has previously been utilized. Fifty-three older adults (aged 50+) scheduled for TJR were assessed pre and post surgery (6 months). Forty-five healthy controls matched for age, gender, and premorbid IQ were re-assessed after an equivalent interval. Cognition, cognitive reserve, and physical and mental health were all measured. Standardized regression-based methods were used to assess cognitive changes, while controlling for the confounding effect of repeated cognitive testing. TJR patients only demonstrated a significant decline in Trail Making Test Part B (TMT B) performance, compared to controls. Cognitive reserve only predicted change in TMT B scores among a subset of TJR patients. Specifically, patients who showed the most improvement pre to post surgery had significantly higher reserve than those who showed the greatest decline. The current study provides limited evidence of POCD after TJR when examined using a rigorous methodology, which controlled for practice effects. Cognitive reserve only predicted performance within a subset of the TJR sample. However, the role of reserve in more cognitively compromised patients remains to be determined.

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

    Directory of Open Access Journals (Sweden)

    Megan E. Kunka

    2015-01-01

    Full Text Available 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.

  1. SYNDROME-PATHOGENETIC APPROACH TO MEDICAL REHABILITATION OF PATIENTS AFTER THE TOTAL HIP AND KNEE REPLACEMENT

    Directory of Open Access Journals (Sweden)

    Y. Y. Udartsev

    2011-01-01

    Full Text Available The results of treatment 103 patients in term of 4-6 months after total hip and knee replacement were examined. Established that in the operated limb disorders of regional blood flow in the form of chronic lympho-venous insufficiency and changes in the functional state of neuromuscular apparatus in the form of muscle dysfunction, autonomic dysfunction occured. According to detected characteristics the medical rehabilitation for patients of the 1st group (52 patients was based on the combination of therapeutic factors complex usage, each of which exerts a specific effect on pathogenesis of a certain syndrome or their different combinations, and developing therapeutic factors are summarized and potentiated. That is why balneo-radon-kinesiotherapy, intermittent pneumocompression, low-frequency magnetotherapy, transcranial electroanalgesia, electrical myostimulation, pelotherapy, taking of central myorelexanat tizanidine were included in the treatment program. Among the patients of the 2nd group medical rehabilitation was based exclusively on motion state and bodily exercises. The analysis of treatment showed the advantage of syndrome-pathogenetic approach, applied for the 1st group of patients: joint range of motions increased by 33,1%, venous drainage increased by 24,4%, neuromuscular apparatus duty improved by 51,8%, involuntary nervous system balance normalized, functional outcome of the medical rehabilitation improved by 73,1%.

  2. Evaluation of Marfan patients status post valve-sparing aortic root replacement with 4D flow.

    Science.gov (United States)

    Hope, Thomas A; Kvitting, John-Peder Escobar; Hope, Michael D; Miller, D Craig; Markl, Michael; Herfkens, Robert J

    2013-11-01

    Over the past two decades elective valve-sparing aortic root replacement (V-SARR) has become more common in the treatment of patients with aortic root and ascending aortic aneurysms. Currently there are little data available to predict complications in the post-operative population. The study goal was to determine if altered flow patterns in the thoracic aorta, as measured by MRI, are associated with complications after V-SARR. Time-resolved three-dimensional phase-contrast MRI (4D flow) was used to image 12 patients with Marfan syndrome after V-SARR. The patients were followed up for an average of 5.8 years after imaging and 8.2 years after surgery. Additionally 5 volunteers were imaged for comparison. Flow profiles were visualized during peak systole using streamlines. Wall shear stress estimates and normalized flow displacement were evaluated at multiple planes in the thoracic aorta. During the follow-up period, a single patient developed a Stanford Type B aortic dissection. At initial imaging, prior to the development of the dissection, the patient had altered flow patterns, wall shear stress estimates, and increased normalized flow displacement in the thoracic aorta in comparison to the remaining V-SARR patients and volunteers. This is the first follow-up study of patients after 4D flow imaging. An aortic dissection developed in one patient with altered flow patterns and hemodynamic stresses in the thoracic aorta. These results suggest that flow and altered hemodynamics may play a role in the development of post-operative intramural hematomas and dissections. Copyright © 2013 Elsevier Inc. All rights reserved.

  3. Effects of music on psychophysiological responses and opioid dosage in patients undergoing total knee replacement surgery.

    Science.gov (United States)

    Chen, Hsin-Ji; Chen, Tsung-Ying; Huang, Chiung-Yu; Hsieh, Yuan-Mei; Lai, Hui-Ling

    2015-10-01

    The present authors examined the effects of listening to music on psychophysiological parameters (blood pressure, heart rate, and respiratory rate) during preoperative and postoperative days and determined whether listening to music could lower pain intensity and opioid dosage during postoperative days in patients who underwent total knee replacements. This was a two group repeated measures design for 30 subjects aged 53-85 years who were scheduled for total knee replacement. Subjects were randomly assigned to either a music group or a control group. Psychophysiological parameters were obtained from patients' monitors. A visual analog scale was used to assess postoperative pain. Opioid dosage was recorded and converted to standardized units. Mann-Whitney U-test and generalized estimating equation analysis were used to compare groups. Respiratory rates while in the surgical waiting area were lower for the music group than for the control group (P = 0.02). There was no significant difference between these groups for blood pressure, heart rate, pain intensity, or opioid dosage. However, a within-group comparison showed that systolic blood pressure in the music group was significantly and consistently decreased during postoperative recovery (Wald = 9.21, P = 0.007). These results suggest that listening to music stabilized systolic blood pressure in patients during postoperative recovery. However, the effects of music on psychophysiological parameters, pain intensity, and opioid dosage in a surgical setting require further research. © 2015 The Authors. Japan Journal of Nursing Science © 2015 Japan Academy of Nursing Science.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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

  6. 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.

  7. Risk stratification using lean body mass in patients undergoing transcatheter aortic valve replacement.

    Science.gov (United States)

    Hioki, Hirofumi; Watanabe, Yusuke; Kozuma, Ken; Yamamoto, Masanori; Naganuma, Toru; Araki, Motoharu; Tada, Norio; Shirai, Shinichi; Yamanaka, Futoshi; Higashimori, Akihiro; Mizutani, Kazuki; Tabata, Minoru; Takagi, Kensuke; Ueno, Hiroshi; Hayashida, Kentaro

    2018-02-22

    The prognostic impact of skeletal muscle mass, assessed using lean body mass (LBM), remain unclear in patients who underwent transcatheter aortic valve replacement (TAVR). The aim of this study to assess prognostic impact of LBM on mortality after TAVR. We assessed 1,613 patients (median age 85 years, 70% female) who underwent TAVI from October 2013 to April 2016 using OCEAN (Optimized transCathEter vAlvular interveNtion)-TAVI registry data. LBM was calculated using the James formula. The primary endpoint was all-cause death after TAVR. Median follow-up period was 287 days (interquartile range 110-462). The Kaplan-Meier analysis demonstrated that patients with low LBM had significantly higher incidence of all-cause death than those with high LBM in male (32.3% vs. 9.9%, log rank P LBM was an independent predictor of all-cause death in male (Hazard ratio [HR] 0.93; 95% confidence interval [CI] 0.89-0.98) and female (HR 0.94; 95% CI 0.89-0.99). Inversely, the assessment using BMI could not identify the high-risk population in a female. The patients with low LBM had the higher incidence of all-cause death after TAVR than those with high LBM, regardless of gender. Thus, the risk stratification using LBM might provide further insight to identify the high-risk TAVR population, compared to conventional risk stratification using BMI. © 2018 Wiley Periodicals, Inc.

  8. Mitral valve repair versus replacement in elderly patients: a systematic review and meta-analysis.

    Science.gov (United States)

    Shang, Xiaoke; Lu, Rong; Liu, Mei; Xiao, Shuna; Dong, Nianguo

    2017-09-01

    Although mitral valve repair (MVP) is generally accepted as the standard treatment for mitral valve disease, in older patients, there is increasing debate about whether MVP is superior to mitral valve replacement (MVR). We, therefore, performed a meta-analysis to compare MVP vs. MVR in the elderly population. We systematically searched PubMed, the Cochrane Library, and Scopus up to February 2017 and scrutinized the references of relevant literatures. Only studies of MVP vs. MVR in the elderly patients (aged 70 years or older) that were published after 2000 were included. The retrieval process yielded seven observational clinical studies with 1,809 patients. Compared with MVR, MVP was associated with a significantly reduced 30-day mortality [risk ratio (RR): 0.40, 95% confidence interval (CI): 0.25-0.64], with shorter duration of postoperative hospital stay (days) (weighted mean difference: -1.47, 95% CI: -2.47--0.48) and less postoperative complications (RR: 0.69, 95% CI: 0.56-0.86). In addition, our study also demonstrated improved 1-year (RR: 1.16, 95% CI: 1.08-1.24) and 5-year (RR: 1.26, 95% CI: 1.13-1.41) survival rates following MVP. There was no difference in reoperations between these two surgery approaches. The present meta-analysis indicates that elderly patients who receive MVP have better early and late outcomes than those undergoing MVR. MVP may be the preferred strategy for mitral valve surgery in the elderly population.

  9. Neurological, psychological, and cognitive disorders in patients with chronic kidney disease on conservative and replacement therapy.

    Science.gov (United States)

    Lai, Silvia; Mecarelli, Oriano; Pulitano, Patrizia; Romanello, Roberto; Davi, Leonardo; Zarabla, Alessia; Mariotti, Amalia; Carta, Maria; Tasso, Giorgia; Poli, Luca; Mitterhofer, Anna Paola; Testorio, Massimo; Frassetti, Nicla; Aceto, Paola; Galani, Alessandro; Lai, Carlo

    2016-11-01

    Chronic kidney disease (CKD) is a highly prevalent condition in the world. Neurological, psychological, and cognitive disorders, related to CKD, could contribute to the morbidity, mortality, and poor quality of life of these patients. The aim of this study was to assess the neurological, psychological, and cognitive imbalance in patients with CKD on conservative and replacement therapy.Seventy-four clinically stable patients affected by CKD on conservative therapy, replacement therapy (hemodialysis (HD), peritoneal dialysis (PD)), or with kidney transplantation (KT) and 25 healthy controls (HC), matched for age and sex were enrolled. Clinical, laboratory, and instrumental examinations, as renal function, inflammation and mineral metabolism indexes, electroencephalogram (EEG), psychological (MMPI-2, Sat P), and cognitive tests (neuropsychological tests, NPZ5) were carried out.The results showed a significant differences in the absolute and relative power of delta band and relative power of theta band of EEG (P = 0.008, P therapy, and Grade 2-3 in KT patients. The scales of MMPI-2 hysteria and paranoia, are significantly correlated with creatinine, eGFR, serum nitrogen, CRP, 1,25-(OH)2D3, intact parathyroid hormone (iPTH), phosphorus, and cynical and hysterical personality, are correlated with higher relative power of delta (P = 0.016) and theta band (P = 0.016). Moreover, all NPZ5 scores showed a significant difference between the means of nephropathic patients and the means of the HC, and a positive correlation with eGFR, serum nitrogen, CRP, iPTH, and vitamin D.In CKD patients, simple and noninvasive instruments, as EEG, and cognitive-psychological tests, should be performed and careful and constant monitoring of renal risk factors, probably involved in neuropsychological complications (inflammation, disorders of mineral metabolism, electrolyte disorders, etc.), should be carried out. Early identification and adequate therapy of neuropsychological

  10. Outcomes of Patients With Severe Chronic Lung Disease Who Are Undergoing Transcatheter Aortic Valve Replacement.

    Science.gov (United States)

    Suri, Rakesh M; Gulack, Brian C; Brennan, J Matthew; Thourani, Vinod H; Dai, Dadi; Zajarias, Alan; Greason, Kevin L; Vassileva, Christina M; Mathew, Verghese; Nkomo, Vuyisile T; Mack, Michael J; Rihal, Charanjit S; Svensson, Lars G; Nishimura, Rick A; O'Gara, Patrick T; Holmes, David R

    2015-12-01

    In this study, we sought to determine the clinical outcomes after transcatheter aortic valve replacement (TAVR) among patients with chronic lung disease (CLD) and to evaluate the safety of transaortic versus transapical alternate access approaches in patients with varying severities of CLD. Clinical records for patients undergoing TAVR from 2011 to 2014 in The Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry were linked to Medicare hospital claims (n = 11,656). Clinical outcomes were evaluated across strata of CLD severity, and the risk-adjusted association between access route and post-TAVR mortality was determined among patients with severe CLD. In this cohort (median age, 84 years; 51.7% female), moderate to severe CLD was present in 27.7% (14.3%, moderate; 13.4%, severe). Compared with patients with no or mild CLD, patients with severe CLD had a higher rate of post-TAVR mortality to 1-year (32.3% versus 21.0%; adjusted hazard ratio [HR], 1.48; 95% confidence interval [CI], 1.31 to 1.66), as did those with moderate CLD (25.5%; adjusted HR, 1.16; 95% CI, 1.03 to 1.30). The adjusted rate of mortality was similar for transapical versus transaortic approaches to 1 year (adjusted HR, 1.17; 95% CI, 0.83 to 1.65). Moderate or severe CLD is associated with an increased risk of death to 1-year after TAVR, and among patients with severe CLD, the risk of death appears to be similar with either transapical or transaortic alternate-access approaches. Further study is necessary to understand strategies to mitigate risk associated with CLD and the long-term implications of these findings. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  11. Indications for MARS-MRI in Patients Treated With Articular Surface Replacement XL Total Hip Arthroplasty.

    Science.gov (United States)

    Connelly, James W; Galea, Vincent P; Laaksonen, Inari; Matuszak, Sean J; Madanat, Rami; Muratoglu, Orhun; Malchau, Henrik

    2018-04-19

    The purpose of this study was to identify which patient and clinical factors are predictive of adverse local tissue reaction (ALTR) and to use these factors to create a highly sensitive algorithm for indicating metal artifact reduction sequence magnetic resonance imaging (MARS-MRI) in Articular Surface Replacement (ASR) XL total hip arthroplasty patients. Our secondary aim was to compare our algorithm to existing national guidelines on when to take MARS-MRI in metal-on-metal total hip arthroplasty patients. The study consisted of 137 patients treated with unilateral ASR XL implants from a prospective, multicenter study. Patients underwent MARS-MRI regardless of clinical presentation at a mean of 6.2 (range, 3.3-10.4) years from surgery. Univariate and multivariate analyses were conducted to determine which variables were predictive of ALTR. Predictors were used to create an algorithm to indicate MARS-MRI. Finally, we compared our algorithm's ability to detect ALTR to existing guidelines. We found a visual analog scale pain score ≥2 (odds ratio [OR] = 2.53; P = .023), high blood cobalt (OR = 1.05; P = .023), and male gender (OR = 2.37; P = .034) to be significant predictors of ALTR presence in our cohort. The resultant algorithm achieved 86.4% sensitivity and 60.2% specificity in detecting ALTR within our cohort. Our algorithm had the highest area under the curve and was the only guideline that was significantly predictive of ALTR (P = .014). Our algorithm including patient-reported pain and sex-specific cutoffs for blood cobalt levels could predict ALTR and indicate MARS-MRI in our cohort of ASR XL metal-on-metal patients with high sensitivity. Level II, diagnostic study. Copyright © 2018 Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. Detublarized Sigmoid Colon for Total Urinary Bladder Replacement: Clinical Outcome in 51 Patients

    International Nuclear Information System (INIS)

    Bassiouny, M.; El-Sherbiny, M. M.

    2003-01-01

    The technique and results of a detublarized sigmoid neo bladder for total urinary bladder substitution after radical cystectomy is described. Methods: Fifty one patients (44 men and 7 women) having a mean age of 46 (range, 32-61 years). Radical cysto prostatectomy was performed for the male patients and anterior pelvic exenteration for the female patients. This was followed by total replacement of the bladder with a detubularized sigmoid colon. They were selected to have invasive bladder cancer away from the bladder neck. The mean follow-up period was 38.8 months (range 8-96). Blood chemistry was analyzed periodically every month during the follow-up period. Early and late complications, continence and voiding pattern were recorded. Urodynamic studies were performed every 6 months. Early postoperative complications included, urinary leakage in 7 patients (14%); wound infection in II patients (22%), deep venous thrombosis of the lower extremities in one patient (2%). Late complications included stenosis of the ureterointestinal anastomosis in one patient (2%), stenosis of urethral anastomosis in one patient (2%). A reservoir stone occurred in one patient (2%). No metabolic acidosis nor vitamin B 12 deficiency was reported. The upper urinary tract function was either improved or stable in 96% of cases. However, deterioration occurred in only 4% of cases. All patients could void voluntery without catheterization. Patient satisfaction for continence, by daytime and nighttime (nocturnal continence), was 86% and 72%, respectively. The mean neo bladder capacity and mean residual urine volume was 463.8 ml (range, 275-603) and 93.7 ml (range 50-189), respectively. Pressure flow analysis showed that the mean basal pressure was 5.1 cm H 2 O (range, 1-18) and the mean pressure at maximal capacity was 37.4 cm H 2 O (range, 1-73). The mean maximal flow rate was 17.8 ml/sec (range 3.1-24.5), the mean average flow rate was 11.4 ml/sec. (range 1.9-14.6). The mean first sensation

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

    Science.gov (United States)

    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

  15. The Concordance between Patients' Renal Replacement Therapy Choice and Definitive Modality: Is It a Utopia?

    Science.gov (United States)

    Prieto-Velasco, Mario; Quiros, Pedro; Remon, Cesar

    2015-01-01

    It is desirable for patients to play active roles in the choice of renal replacement therapy (RRT). Patient decision aid tools (PDAs) have been developed to allow the patients to choose the option best suited to their individual needs. An observational, prospective registry was conducted in 26 Spanish hospitals between September 2010 and May 2012. The results of the patients' choice and the definitive RRT modality were registered through the progressive implementation of an Education Process (EP) with PDAs designed to help Chronic Kidney Disease (CKD) patients choose RRT. Patients included in this study: 1044. Of these, 569 patients used PDAs and had made a definitive choice by the end of registration. A total of 88.4% of patients chose dialysis [43% hemodialysis (HD) and 45% peritoneal dialysis (PD)] 3.2% preemptive living-donor transplant (TX), and 8.4% conservative treatment (CT). A total of 399 patients began RRT during this period. The distribution was 93.4% dialysis (53.6% HD; 40% PD), 1.3% preemptive TX and 5.3% CT. The patients who followed the EP changed their mind significantly less often [kappa value of 0.91 (95% CI, 0.86-0.95)] than those who did not follow it, despite starting unplanned treatment [kappa value of 0.85 (95% CI, 0.75-0.95]. A higher agreement between the final choice and a definitive treatment was achieved by the EP and planned patients [kappa value of 0.93 (95% CI, 0.89-0.98)]. Those who did not go through the EP had a much lower index of choosing PD and changed their decision more frequently when starting definitive treatment [kappa value of 0.73 (95% CI, 0.55-0.91)]. Free choice, assisted by PDAs, leads to a 50/50 distribution of PD and HD choice and an increase in TX choice. The use of PDAs, even with an unplanned start, achieved a high level of concordance between the chosen and definitive modality.

  16. Using continuous renal replacement therapy to manage patients of shock and acute renal failure

    Science.gov (United States)

    Soni, Sachin S; Nagarik, Amit P; Adikey, Gopal Kishan; Raman, Anuradha

    2009-01-01

    Background: The incidence of acute renal failure (ARF) in the hospital setting is increasing. It portends excessive morbidity and mortality and a considerable burden on hospital resources. Extracorporeal therapies show promise in the management of patients with shock and ARF. It is said that the potential of such therapy goes beyond just providing renal support. The aim of our study was to analyze the clinical setting and outcomes of critically ill ARF patients managed with continuous renal replacement therapy (CRRT). Patients and Methods: Ours was a retrospective study of 50 patients treated between January 2004 and November 2005. These 50 patients were in clinical shock and had concomitant ARF. All of these patients underwent CVVHDF (continuous veno-venous hemodiafiltration) in the intensive care unit. For the purpose of this study, shock was defined as systolic BP magnesium sulphate, sodium bicarbonate, and potassium chloride in separate units, while maintaining careful monitoring of electrolytes. Anticoagulation of the extracorporeal circuit was achieved with systemic heparin in 26 patients; frequent saline flushes were used in the other 24 patients. Results: Of the 50 patients studied, 29 were males and 21 females (1.4:1). The average age was 52.88 years (range: 20–75 years). Causes of ARF included sepsis in 24 (48%), hemodynamically mediated renal failure (HMRF) in 18 (36%), and acute over chronic kidney disease in 8 (16%) patients. The overall mortality was 74%. The average SOFA score was 14.31. The variables influencing mortality on multivariate analysis were: age [odds ratio (OR):1.65; 95% CI: 1.35 to 1.92; P = 0.04], serum creatinine (OR:1.68; 95% CI: 1.44 to 1.86; P = 0.03), and serum bicarbonate (OR: 0.76; 95% CI: 0.55 to 0.94; P = 0.01). On univariate analysis the SOFA score was found to be a useful predictor of mortality. Conclusions: Despite advances in treating critically ill patients with newer extracorporeal therapies, mortality is dismally high

  17. Prevalence of patients receiving renal replacement therapy in El Salvador in 2014.

    Science.gov (United States)

    García-Trabanino, Ramón; Trujillo, Zulma; Colorado, Ana Verónica; Magaña Mercado, Salvador; Henríquez, Carlos Atilio

    El Salvador has the highest renal failure mortality rate in the Americas. Five healthcare providers offer renal replacement therapy (RRT) in the country. The national RRT prevalence has never been reported. To determine the RRT prevalence in El Salvador and some basic characteristics. The association of nephrology coordinated a nationwide cross-sectional survey during the third quarter of 2014. 31 renal centres participated in the survey, covering 99.5% of patients. National RRT prevalence: 595 per million population (pmp), N=3807, average age 50.4 years, 67.5% male. By modality: peritoneal dialysis (PD) 289 pmp, haemodialysis (HD) 233 pmp, with functioning kidney transplantation 74 pmp (living donor only). Social security covers 25% of the population but treats 49.7% of RRT patients. Generally, higher prevalence was observed in municipalities with renal centres or located on the coast or lowlands. Ninety-five percent of HD patients receive fewer than 3 weekly sessions. Of PD patients, 59% do not belong to a continuous outpatient or automated programme, and 25% still use rigid catheter. Aetiology of chronic kidney disease: unavailable/undetermined 50%, hypertension 21.1%, diabetes 18.9%, glomerulonephritis 6.7%, obstructive causes 1.2%, tubulointerstitial 0.9%, polycystic 0.4% and other 0.7%. Despite the increase in RRT services, the prevalence is lower than the Latin American average (660 pmp). Three quarters of HD and PD patients are under-dialysed. Obsolete RRT techniques are still used. The presence of Mesoamerican nephropathy influences the demographic characteristics (many young patients, two-thirds male, high prevalence in lowlands and coastlands). Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  18. DIFFICULTIeS OF TOTAL HIP REPLACEMENT IN PATIENTS WITH ANKYLOSING SPONDYLITIS (case report

    Directory of Open Access Journals (Sweden)

    R. M. Tikhilov

    2016-01-01

    Full Text Available A distinctive feature of patients with ankylosing spondylitis is the formation of hip ankylosis in an extremely unfavorable functional position combined with upset of sagittal balance of the body along with a thoracolumbar kyphosis. Treatment of these patients poses considerable technical difficulties and is often associated with complications. The authors report a clinical case of a female 40 years old patient with confirmed rhizomelic spondylitis. The patient mainly complained of fixed malposition of the right lower extremity (hip ankylosis in extreme 1450 flexion and 1500 abduction combined with a severe fixed spine deformity (thoracic kyphosis 920, lumbar lordosis 170. Considering significant sagittal balance disorder it was decided to go for a two-stage procedure. Total hip arthroplasty of the right joint was performed at the first stage. At the second stage the authors corrected thoracolumbar spinal deformity by Th12 (type PSO 4 and L2 (type PSO 3 wedge resections and converging resected vertebral bodies by a multilevel fixation system with transpedicular support elements. The interval between the stages was 11 months. Two-stage treatment of this patient al-lowed to avoid adverse postoperative complications and to achieve a significant functional improvement in one year after treatment started. The sum of points before and after the treat-ment amounted respectively to 46 and 79 on Harris Hip Score, 17 and 38 points on Oxford Hip Score (OHS. To summarize, comprehensive treatment with planning of all subsequent steps prior to hip replacement is the method of choice for avoidance of postoperative complications in patients with ankylosing spondylitis accompanied by a significant upset of sagittal balance.

  19. Left atrial appendage obliteration in atrial fibrillation patients undergoing bioprosthetic mitral valve replacement.

    Science.gov (United States)

    Min, X P; Zhu, T Y; Han, J; Li, Y; Meng, X

    2016-02-01

    Left atrial appendage (LAA) obliteration is a proven stroke-preventive measure for patients with nonvalvular atrial fibrillation (AF). However, the efficacy of LAA obliteration for patients with AF after bioprosthetic mitral valve replacement (MVR) remains unclear. This study aimed to estimate the efficacy of LAA obliteration in preventing embolism and to investigate the predictors of thromboembolism after bioprosthetic MVR. We retrospectively studied 173 AF subjects with bioprosthetic MVR; among them, 81 subjects underwent LAA obliteration using an endocardial running suture method. The main outcome measure was the occurrence of thrombosis events (TEs). The mean follow-up time was 40 ± 17 months. AF rhythm was observed in 136 patients postoperatively. The incidence rate of TEs was 13.97 % for postoperative AF subjects; a dilated left atrium (LA; > 49.5 mm) was identified as an independent risk factor of TEs (OR = 10.619, 95 % CI = 2.754-40.94, p = 0.001). For postoperative AF patients with or without LAA, the incidence rate of TEs was 15.8 % (9/57) and 12.7 % (10/79; p = 0.603), respectively. The incidence rate of TEs was 2.7 % (1/36) and 4.2 % (2/48) for the subgroup patients with a left atrial diameter of  49.5 mm (p = 0.346). Surgical LAA obliteration in patients with valvular AF undergoing bioprosthetic MVR did not reduce TEs, even when the CHA2DS2-VASc score (a score for estimating the risk of stroke in AF) was ≥ 2 points.

  20. 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...

  1. 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.

  2. Comparative analysis of pain in patients who underwent total knee replacement regarding the tourniquet pressure

    Directory of Open Access Journals (Sweden)

    Marcos George de Souza Leão

    Full Text Available ABSTRACT OBJECTIVES: To evaluate through the visual analog scale (VAS the pain in patients undergoing total knee replacement (TKR with different pressures of the pneumatic tourniquet. METHODS: An observational, randomized, descriptive study on an analytical basis, with 60 patients who underwent TKR, divided into two groups, which were matched: a group where TKR was performed with tourniquet pressures of 350 mmHg (standard and the other with systolic blood pressure plus 100 mmHg (P + 100. These patients had their pain assessed by VAS at 48 h, and at the 5th and 15th days after procedure. Secondarily, the following were also measured: range of motion (ROM, complications, and blood drainage volume in each group; the data were subjected to statistical analysis. RESULTS: After data analysis, there was no statistical difference regarding the incidence of complications (p = 0.612, ROM (p = 0.202, bleeding after 24 and 48 h (p = 0.432 and p = 0.254 or in relation to VAS. No correlation was observed between time of ischemia compared to VAS and bleeding. CONCLUSIONS: The use of the pneumatic tourniquet pressure at 350 mmHg or systolic blood pressure plus 100 mmHg did not influence the pain, blood loss, ROM, and complications. Therefore the pressures at these levels are safe and do not change the surgery outcomes; the time of ischemia must be closely observed to avoid major complications.

  3. [Echinocandins: searching for differences. The example of their use in patients requiring continuous renal replacement therapy].

    Science.gov (United States)

    de la Llama-Celis, Natalia; Huarte-Lacunza, Rafael; Gómez-Baraza, Cristina; Cañamares-Orbis, Iciar; Sebastián-Aldeanueva, Manuel; Arrieta-Navarro, Raquel

    2012-12-01

    The echinocandins have a growing role in the treatment of fungal infections because of their novel mechanism of action. This is reflected in recently published management guidelines, but available in vitro data, animal studies, and clinical studies do not clearly differentiate the three agents in class. Comparative clinical efficacy among agents within the class, pharmacokinetic profiles in special populations, pharmacoeconomics justifications, and place in therapy have been largely unanswered. They share many common properties but marketing strategies of drug manufacturers are engaged in product differentiation. Although exist similarities in the pharmacokinetic (PK) profiles of the echinocandins, limited data have been published regarding their pharmacokinetics in continuous renal replacement therapy (CRRT) patients. The pharmacokinetics of drug removal in critically ill patients receiving CRRT is very complex, with multiple variables affecting clearance. This review outlines the basic principles that determine whether a dose adjustment is required. Two studies with data on PK parameters of micafungin and anidulafungin in CRRT patients have been published and are compared following that basic principles in the review.

  4. Effectiveness of Music Listening in Patients With Total Knee Replacement During CPM Rehabilitation.

    Science.gov (United States)

    Hsu, Chih-Chung; Chen, Wei-Ming; Chen, Su-Ru; Tseng, Yen-Ting; Lin, Pi-Chu

    2016-01-01

    This study investigated the effects of music listening on the anxiety, heart rate variability (HRV), and joint range of motion (ROM) of patients undergoing continuous passive motion (CPM) after total knee replacement surgery. An experimental design was used. Participants in the experimental group (n = 49) listened to music from 10 min before receiving CPM until the end of the session (25 min in total) on the first and second day following surgery, whereas participants in the control group (n = 42) did not listen to music but rested quietly in bed starting 10 min before and throughout CPM. Compared with the control group, the experimental group exhibited significantly lower anxiety levels (p CPM angles (p < .05) during treatment and increased active flexion ROM (p < .05) upon discharge. The low-frequency (LF)/high-frequency (HF) power ratio, normalized LF HRV, and normalized HF HRV of the two groups differed significantly, indicating that the patients in the experimental group had greater parasympathetic activity compared with those in the control group. Music listening can effectively reduce patient anxiety and enhance the ROM of their joints during postoperative rehabilitation. Health-care practitioners should consider including music listening as a routine practice for postoperative rehabilitation following orthopedic surgery. © The Author(s) 2015.

  5. 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

  6. 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

  7. Position controlled Knee Rehabilitation Orthotic Device for Patients after Total Knee Replacement Arthroplasty

    Science.gov (United States)

    Wannaphan, Patsiri; Chanthasopeephan, Teeranoot

    2016-11-01

    Knee rehabilitation after total knee replacement arthroplasty is essential for patients during their post-surgery recovery period. This study is about designing one degree of freedom knee rehabilitation equipment to assist patients for their post-surgery exercise. The equipment is designed to be used in sitting position with flexion/extension of knee in sagittal plane. The range of knee joint motion is starting from 0 to 90 degrees angle for knee rehabilitation motion. The feature includes adjustable link for different human proportions and the torque feedback control at knee joint during rehabilitation and the control of flexion/extension speed. The motion of the rehabilitation equipment was set to move at low speed (18 degrees/sec) for knee rehabilitation. The rehabilitation link without additional load took one second to move from vertical hanging up to 90° while the corresponding torque increased from 0 Nm to 2 Nm at 90°. When extra load is added, the link took 1.5 seconds to move to 90° The torque is then increased from 0 Nm to 4 Nm. After a period of time, the speed of the motion can be varied. User can adjust the motion to 40 degrees/sec during recovery activity of the knee and users can increase the level of exercise or motion up to 60 degrees/sec to strengthen the muscles during throughout their rehabilitation program depends on each patient. Torque control is included to prevent injury. Patients can use the equipment for home exercise to help reduce the number of hospital visit while the patients can receive an appropriate therapy for their knee recovery program.

  8. Multidetector CT predictors of prosthesis-patient mismatch in transcatheter aortic valve replacement.

    Science.gov (United States)

    Freeman, Melanie; Webb, John G; Willson, Alexander B; Wheeler, Miriam; Blanke, Philipp; Moss, Robert R; Thompson, Christopher R; Munt, Brad; Norgaard, Bjarne L; Yang, Tae-Hyun; Min, James K; Poulsen, Steen; Hansson, Nicolaj C; Binder, Ronald K; Toggweiler, Stefan; Hague, Cameron; Wood, David A; Pibarot, Philippe; Leipsic, Jonathon

    2013-01-01

    Prosthesis-patient mismatch (PPM) is a predictor of mortality after aortic valve replacement (AVR). We examined whether accurate 3-dimensional annular sizing with multidetector CT (MDCT) is predictive of PPM after transcatheter AVR (TAVR). One hundred twenty-eight patients underwent MDCT then TAVR. Moderate PPM was defined as an indexed effective orifice area ≤0.85 cm²/m² and severe ≤0.65 cm²/m². MDCT annular measurements (area, short and long axis) were compared with the size of the selected transcatheter heart valve (THV) to obtain (1) the difference between prosthesis size and CT-measured mean annular diameter and (2) the percentage of undersizing or oversizing (calculated as 100 × [MDCT annular area--THV nominal area]/THV nominal area). In addition, the MDCT annular area was indexed to body surface area. These measures were evaluated as potential PPM predictors. We found that 42.2% of patients had moderate PPM and 9.4% had severe PPM. Procedural characteristics and in-hospital outcomes were similar between patients with or without PPM. THV undersizing of the mean aortic annulus diameter was not predictive of PPM (odds ratio [OR], 0.84; 95% CI, 0.65-1.07; P = .16; area under the receiver-operating characteristic curve [AUC], 0.58). THV undersizing of annular area was not predictive of PPM (OR, 0.96; 95% CI, 0.80-1.16; P = .69; AUC, 0.52). Indexed MDCT annular area was, however, predictive of PPM (OR, 0.24; 95% CI, 0.10-0.59; P < .001; AUC, 0.66). PPM is frequent after TAVR. Appropriate annular oversizing does not reduce the rate or severity of PPM. Patient annulus size mismatch, identified by indexed MDCT annular area, is a significant predictor of PPM. Copyright © 2013 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

  9. 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

  10. Infection imaging with 99mTc-biotin in patients with prosthetic hip replacement

    International Nuclear Information System (INIS)

    Villa, G.; Mariani, G.; Augeri, C.; Pipino, F.; Paganelli, G.; Chinol, M.

    2002-01-01

    Full text: Although the incidence of infection in prosthetic hip joint replacements has decreased from about 10-15 % to about 0.5-2 % over the last 20 years, the total number of infections has actually increased because of the large number of patients undergoing the procedure. The most frequent clinical presentation of this complication is functional impairment and pain, with or without fever and other signs and/or symptoms of infection. The main is differentiating true infection from simple loosening with inflammation of the implanted stem. Scintigraphy with radiolabeled autologous leukocytes (WBC) represents the 'gold standard' nuclear medicine procedure for imaging infection. However, this procedure is time-consuming, expensive, and involves some biological hazard. Preliminary data, obtained during validation of the avidin/111In-biotin approach, have suggested some potential of 111ln-biotin per se to accumulate at sites of infection. In this pilot study we explored the potential of 99mTc-biotin as an infection imaging agent in pts with orthopedic infections. N4-lys-biotin was labeled with 1110 MBq. Sixteen pts bearing a total of 20 prosthetic hip replacements were enrolled in the study (9 women and 7 men, mean age 73.2 yrs). Eight pts had previously undergone removal of their hip prosthesis because of infection, while infection was suspected in the remaining 8 pts. Scintigraphy was recorded 20 min, then 1, 4 and 24 hr after the i.v. injection of 99mTc-biotin. Within 48 hrs of the 99mTc-biotin study, all pts also underwent scintigraphy with 99mTc-HMPAO-WBC. Out of the 20 hips evaluated, 15 turned out to be infected while in the remaining 5 cases pain was only caused by bone-prosthetic loosening and/or conditions other than infection. In 12/15 infected sites scintigraphy was concordantly positive with both procedures, 99mTc-biotin yielding higher target-to-nontarget ratios than 99mTc-HMPAO-WBC in 4 cases and similar values in the other cases. Discordant patterns

  11. 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)

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

    Directory of Open Access Journals (Sweden)

    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.

  13. Optimising glucocorticoid replacement therapy in severely adrenocorticotropin (ACTH) deficient hypopituitary male patients.

    LENUS (Irish Health Repository)

    Behan, Lucy-Ann

    2011-04-18

    Context:  The optimal replacement regimen of hydrocortisone in adults with severe ACTH deficiency remains unknown. Management strategies vary from treatment with 15mg to 30mg or higher in daily divided doses, reflecting the paucity of prospective data on the adequacy of different glucocorticoid regimens. Objective:  Primarily to define the hydrocortisone regimen which results in a 24hour cortisol profile that most closely resembles that of healthy controls and secondarily to assess the impact on quality of life (QoL). Design:  10 male hypopituitary patients with severe ACTH deficiency (basal cortisol <100nM and peak response to stimulation <400nM) were enrolled in a prospective, randomised, crossover study of 3 hydrocortisone dose regimens. Following 6 weeks of each regimen patients underwent 24hour serum cortisol sampling and QoL assessment with the Short Form 36 and the Nottingham Health Profile questionnaires. Free cortisol was calculated using Coolen\\'s equation. All results were compared to those of healthy, matched controls. Results:  CBG was significantly lower across all dose regimens compared to controls (p<0.05). The lower dose regimen C(10mg mane\\/5mg tarde) produced a 24hour free cortisol profile which most closely resembled that of controls. Both regimen A(20mg mane\\/10mg tarde) and B(10mg mane\\/10mg tarde) produced supraphysiological post-absorption peaks. There was no significant difference in QoL in patients between the three regimens, however energy level was significantly lower across all dose regimens compared to controls (p<0.001). Conclusions:  The lower dose of HC(10mg\\/5mg) produces a more physiological cortisol profile, without compromising quality of life, compared to higher doses still used in clinical practice. This may have important implications in these patients, known to have excess cardiovascular mortality.

  14. Optimizing glucocorticoid replacement therapy in severely adrenocorticotropin-deficient hypopituitary male patients.

    LENUS (Irish Health Repository)

    Behan, Lucy-Ann

    2012-02-01

    BACKGROUND: The optimal replacement regimen of hydrocortisone in adults with severe ACTH deficiency remains unknown. Management strategies vary from treatment with 15-30 mg or higher in daily divided doses, reflecting the paucity of prospective data on the adequacy of different glucocorticoid regimens. OBJECTIVE: Primarily to define the hydrocortisone regimen which results in a 24 h cortisol profile that most closely resembles that of healthy controls and secondarily to assess the impact on quality of life (QoL). DESIGN: Ten male hypopituitary patients with severe ACTH deficiency (basal cortisol <100 nm and peak response to stimulation <400 nm) were enrolled in a prospective, randomized, crossover study of 3 hydrocortisone dose regimens. Following 6 weeks of each regimen patients underwent 24 h serum cortisol sampling and QoL assessment with the Short Form 36 (SF36) and the Nottingham Health Profile (NHP) questionnaires. Free cortisol was calculated using Coolen\\'s equation. All results were compared to those of healthy, matched controls. RESULTS: Corticosteroid binding globulin (CBG) was significantly lower across all dose regimens compared to controls (P < 0.05). The lower dose regimen C (10 mg mane\\/5 mg tarde) produced a 24 h free cortisol profile (FCP) which most closely resembled that of controls. Both regimen A(20 mg mane\\/10 mg tarde) and B(10 mg mane\\/10 mg tarde) produced supraphysiological post-absorption peaks. There was no significant difference in QoL in patients between the three regimens, however energy level was significantly lower across all dose regimens compared to controls (P < 0.001). CONCLUSIONS: The lower dose of hydrocortisone (10 mg\\/5 mg) produces a more physiological cortisol profile, without compromising QoL, compared to higher doses still used in clinical practice. This may have important implications in these patients, known to have excess cardiovascular mortality.

  15. 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

  16. The Influence of Obesity on Patient Reported Outcomes following Total Knee Replacement

    Directory of Open Access Journals (Sweden)

    Vandana Ayyar

    2012-01-01

    Full Text Available This study retrospectively analysed the effects of obesity as described by Body Mass Index (BMI on patient reported outcomes following total knee replacement. Participants (105 females and 66 males who had undergone surgery under the care of a single surgeon were included in the review and were grouped according to their preoperative BMI into nonobese ( kg/m2, ( obese ( kg/m2 (. Oxford Knee Score (OKS and Short Form 12 scores (SF12 were taken preoperatively and 6 and 12 months after surgery to analyse differences between groups in the absolute scores as well as changes from before to after surgery. Preoperatively, the obese group had a significantly poorer OKS compared to non obese (44.7 versus 41.2, . There were no statistically significant group effects on follow-up or change scores of the OKS and SF12. Correlations coefficients between BMI and follow-up and change scores were low (. There were no significant differences in the number of complications and revisions (local wound infection, 6.7% non obese, 11% obese, postoperative systemic complication, 8% non obese, 12% obese, revision, 4% nonobese, 3% obese. In conclusion, our findings indicate similar degrees of benefits from the surgery irrespective of patient BMI.

  17. Cast Partial Denture versus Acrylic Partial Denture for Replacement of Missing Teeth in Partially Edentulous Patients

    Directory of Open Access Journals (Sweden)

    Pramita Suwal

    2017-03-01

    Full Text Available Aim: To compare the effects of cast partial denture with conventional all acrylic denture in respect to retention, stability, masticatory efficiency, comfort and periodontal health of abutments. Methods: 50 adult partially edentulous patient seeking for replacement of missing teeth having Kennedy class I and II arches with or without modification areas were selected for the study. Group-A was treated with cast partial denture and Group-B with acrylic partial denture. Data collected during follow-up visit of 3 months, 6 months, and 1 year by evaluating retention, stability, masticatory efficiency, comfort, periodontal health of abutment. Results: Chi-square test was applied to find out differences between the groups at 95% confidence interval where p = 0.05. One year comparison shows that cast partial denture maintained retention and stability better than acrylic partial denture (p< 0.05. The masticatory efficiency was significantly compromising from 3rd month to 1 year in all acrylic partial denture groups (p< 0.05. The comfort of patient with cast partial denture was maintained better during the observation period (p< 0.05. Periodontal health of abutment was gradually deteriorated in all acrylic denture group (p

  18. Knee Joint Loads and Surrounding Muscle Forces during Stair Ascent in Patients with Total Knee Replacement.

    Directory of Open Access Journals (Sweden)

    Robert Rasnick

    Full Text Available Total knee replacement (TKR is commonly used to correct end-stage knee osteoarthritis. Unfortunately, difficulty with stair climbing often persists and prolongs the challenges of TKR patents. Complete understanding of loading at the knee is of great interest in order to aid patient populations, implant manufacturers, rehabilitation, and future healthcare research. Musculoskeletal modeling and simulation approximates joint loading and corresponding muscle forces during a movement. The purpose of this study was to determine if knee joint loadings following TKR are recovered to the level of healthy individuals, and determine the differences in muscle forces causing those loadings. Data from five healthy and five TKR patients were selected for musculoskeletal simulation. Variables of interest included knee joint reaction forces (JRF and the corresponding muscle forces. A paired samples t-test was used to detect differences between groups for each variable of interest (p<0.05. No differences were observed for peak joint compressive forces between groups. Some muscle force compensatory strategies appear to be present in both the loading and push-off phases. Evidence from knee extension moment and muscle forces during the loading response phase indicates the presence of deficits in TKR in quadriceps muscle force production during stair ascent. This result combined with greater flexor muscle forces resulted in similar compressive JRF during loading response between groups.

  19. Knee Joint Loads and Surrounding Muscle Forces during Stair Ascent in Patients with Total Knee Replacement

    Science.gov (United States)

    Rasnick, Robert; Standifird, Tyler; Reinbolt, Jeffrey A.; Cates, Harold E.

    2016-01-01

    Total knee replacement (TKR) is commonly used to correct end-stage knee osteoarthritis. Unfortunately, difficulty with stair climbing often persists and prolongs the challenges of TKR patents. Complete understanding of loading at the knee is of great interest in order to aid patient populations, implant manufacturers, rehabilitation, and future healthcare research. Musculoskeletal modeling and simulation approximates joint loading and corresponding muscle forces during a movement. The purpose of this study was to determine if knee joint loadings following TKR are recovered to the level of healthy individuals, and determine the differences in muscle forces causing those loadings. Data from five healthy and five TKR patients were selected for musculoskeletal simulation. Variables of interest included knee joint reaction forces (JRF) and the corresponding muscle forces. A paired samples t-test was used to detect differences between groups for each variable of interest (pknee extension moment and muscle forces during the loading response phase indicates the presence of deficits in TKR in quadriceps muscle force production during stair ascent. This result combined with greater flexor muscle forces resulted in similar compressive JRF during loading response between groups. PMID:27258086

  20. 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...

  1. 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.

  2. Early versus late pulmonary valve replacement in patients with transannular patch-repaired tetralogy of Fallot.

    Science.gov (United States)

    Dobbels, Bieke; Herregods, Marie-Christine; Troost, Els; Van De Bruaene, Alexander; Rega, Filip; Budts, Werner; De Meester, Pieter

    2017-09-01

    Although the effects of pulmonary regurgitation after tetralogy of Fallot repair are detrimental, timing of pulmonary valve replacement (PVR) is unclear. Our goal was to evaluate the midterm efficacy and safety of early PVR. Patients with tetralogy of Fallot who underwent repair from 1962 to 2015 were included from the local database. Statistical analyses compared patients who underwent early PVR (age ≤16 years), late PVR and no PVR. The timing of the intervention was compared for efficacy-all-cause mortality and the combined end-point of all-cause mortality, ventricular tachycardia and defibrillator implantation-and for safety-the combined end-point of 1-year postoperative mortality after PVR, endocarditis and reintervention. Echocardiographic and electrocardiographic data at the last follow-up examination were compared across the 3 groups. Two hundred seventy-three patients (age 21 ± 5 years; 52% female) were included. The mean follow-up was 24 (95% confidence interval 22.7-26.2) years; the observed median was 21 years (interquartile range 11-31). No significant difference in survival was found between the early PVR (n = 106; 39%), the late PVR (n = 47; 17%) and the no PVR groups (n = 120; 44%) (P = 0.990). No significant difference in the combined efficacy end-point was noted between patients who underwent early PVR compared with patients who underwent late PVR (P = 0.247). Worse event-free survival for the 3-point safety end-point was observed after early PVR (P < 0.001). Right ventricular morphology (P < 0.001) and function (P < 0.001) were better preserved in the patient group that underwent PVR before the age of 16 years. As expected, PVR-related morbidity was higher in patients who underwent early PVR but the midterm outcome was similar. Nevertheless, better preservation of right ventricular morphology and function in the early PVR group might result in better long-term survival. © The Author 2017. Published by Oxford

  3. 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

  4. 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.

  5. Quality indicators of continuous renal replacement therapy (CRRT) care in critically ill patients: a systematic review.

    Science.gov (United States)

    Rewa, Oleksa G; Villeneuve, Pierre-Marc; Lachance, Philippe; Eurich, Dean T; Stelfox, Henry T; Gibney, R T Noel; Hartling, Lisa; Featherstone, Robin; Bagshaw, Sean M

    2017-06-01

    Renal replacement therapy is increasingly utilized in the intensive care unit (ICU), of which continuous renal replacement therapy (CRRT) is most common. Despite CRRT being a relatively invasive and resource intensive technology, there remains wide practice variation in its application. This systematic review appraised the evidence for quality indicators (QIs) of CRRT care in critically ill patients. A comprehensive search strategy was developed and performed in five citation databases (Medline, Embase, CINAHL, Cochrane Library, and PubMed) and select grey literature sources. Two reviewers independently screened, selected, and extracted data using standardized forms. Each retrieved citation was appraised for quality using the Newcastle-Ottawa Scale (NOS) and Cochrane risk of bias tool. Data were summarized narratively. Our search yielded 8374 citations, of which 133 fulfilled eligibility. This included 97 cohort studies, 24 randomized controlled trials, 10 case-control studies, and 2 retrospective medical audits. The quality of retrieved studies was generally good. In total, 18 QIs were identified that were mentioned in 238 instances. Identified QIs were classified as related to structure (n = 4, 22.2 %), care processes (n = 9, 50.0 %), and outcomes (n = 5, 27.8 %). The most commonly mentioned QIs focused on filter lifespan (n = 98), small solute clearance (n = 46), bleeding (n = 30), delivered dose (n = 19), and treatment interruption (n = 5). Across studies, the definitions used for QIs evaluating similar constructs varied considerably. When identified, QIs were most commonly described as important (n = 144, 48.3 %), scientifically acceptable (n = 32, 10.7 %), and useable and/or feasible (n = 17, 5.7 %) by their primary study authors. We identified numerous potential QIs of CRRT care, characterized by heterogeneous definitions, varying quality of derivation, and limited evaluation. Further study is needed to prioritize a concise

  6. 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....

  7. The effect of tourniquet deflation on hemodynamics and regional cerebral oxygen saturation in aged patients undergoing total knee replacement surgery

    OpenAIRE

    Song, Inkyung; Kim, Dong Yeon; Kim, Youn Jin

    2012-01-01

    Background 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 tot...

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  11. Telemedicine consulting in the patient preparation and planning of prosthetic tooth replacement

    Directory of Open Access Journals (Sweden)

    Mladenović Dragan

    2013-01-01

    Full Text Available Introduction. In the management of edentulous spaces, there is a permanent need of a dentist-prosthetician in charge to consult other specialists. Modern telemedicine, based on powerful computer and telecomunication systems, offers an adequate answer to these challenges, being able to transfer and obtain clinical data and consultation information over large distances. Using smartphone or a computer, the teleconsultant acces the system, downloads and review the data and photographs and gave suggestions. The system then enables direct, real time contact with the consultant, chat, or directs them to contact each other by phone. Case report. We presented telemedicine consulting in the patient preparation and planning of prosthetic tooth replacement in 3 cases with different teleconsultation requirements: the first case for prosthetic rehabilitation of his upper teeth, the second one for prosthetic management of his partial edentulousness and “a growth on his gums” in the vestibular region of the frontal teeth and the third one for prosthetic management of total edentulousness of her upper jaw. We used the system of telemedicine in dentistry, established at the Faculty of Medicine in Kosovska Mitrovica. The operation was based on the computer application system XPA3 Online, computer networking and mobile smartphone network. All consultations were succefull with no need for further procedures in regional center. Conclusion. The use of a mobile smartphone has brought about the mobility and availability of teleconsultant specialists in an extent never seen before. Prostheticians are thus able to offer better service to their patients and improve the quality of management of partially or totally edentulous patients, especially in rural areas.

  12. Telemedicine consulting in the patient preparation and planning of prosthetic tooth replacement.

    Science.gov (United States)

    Mladenović, Dragan; Tosić, Goran; Zivković, Dusan; Djindjić, Natasa; Mladenović, Lidija; Mladenović, Sanja; Marković, Ivana

    2013-09-01

    In the management of edentulous spaces, there is a permanent need of a dentist-prosthetician in charge to consult other specialists. Modern telemedicine, based on powerful computer and telecomunication systems, offers an adequate answer to these challenges, being able to transfer and obtain clinical data and consultation information over large distances. Using smartphone or a computer, the teleconsultant acces the system, downloads and review the data and photographs and gave suggestions. The system then enables direct, real time contact with the consultant, chat, or directs them to contact each other by phone. We presented telemedicine consulting in the patient preparation and planning of prosthetic tooth replacement in 3 cases with different teleconsultation requirements: the first case for prosthetic rehabilitation of his upper teeth, the second one for prosthetic management of his partial edentulousness and "a growth on his gums" in the vestibular region of the frontal teeth and the third one for prosthetic management of total edentulousness of her upper jaw. We used the system of telemedicine in dentistry, established at the Faculty of Medicine in Kosovska Mitrovica. The operation was based on the computer application system XPA3 Online, computer networking and mobile smartphone network. All consultations were succefull with no need for further procedures in regional center. The use of a mobile smartphone has brought about the mobility and availability of teleconsultant specialists in an extent never seen before. Prostheticians are thus able to offer better service to their patients and improve the quality of management of partially or totally edentulous patients, especially in rural areas.

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

    Science.gov (United States)

    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

  14. The anteroposterior axis of the tibia in Korean patients undergoing total knee replacement.

    Science.gov (United States)

    Kim, C W; Seo, S S; Kim, J H; Roh, S M; Lee, C R

    2014-11-01

    The aim of this study was to find anatomical landmarks for rotational alignment of the tibial component in total knee replacement (TKR) in a CT-based study. Pre-operative CT scanning was performed on 94 South Korean patients (nine men, 85 women, 188 knees) with osteoarthritis of the knee joint prior to TKR. The tibial anteroposterior (AP) axis was defined as a line perpendicular to the femoral surgical transepicondylar axis and passing through the centre of the posterior cruciate ligament (PCL). The angles between the defined tibial AP axis and anatomical landmarks at various levels of the tibia were measured. The mean values of the angles between the defined tibial AP axis and the line connecting the anterior border of the proximal third of the tibia to the centre of the PCL was -0.2° (-17 to 14.1, sd 4.1). This was very close to the defined tibial axis, and remained so regardless of lower limb alignment and the degree of tibial bowing. Therefore, AP axis defined as described, is a reliable anatomical landmark for rotational alignment of tibial components. ©2014 The British Editorial Society of Bone & Joint Surgery.

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

    Science.gov (United States)

    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.

  16. 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

  17. 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

  18. 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.

  19. 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

  20. 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 ...

  1. Reducing Prosthesis-Patient Mismatch With Edwards Magna Prosthesis for Aortic Valve Replacement.

    Science.gov (United States)

    Kume, Yuta; Fujita, Tomoyuki; Fukushima, Satsuki; Hata, Hiroki; Shimahara, Yusuke; Matsumoto, Yorihiko; Yamashita, Kizuku; Kobayashi, Junjiro

    2017-03-24

    Prosthesis-patient mismatch (PPM) is associated with increased mid-term and long-term mortality rates after aortic valve replacement (AVR). This study aimed to evaluate the efficacy of the Carpentier-Edwards Perimount Magna and Magna Ease (CEPMs) aortic bioprostheses to reduce the incidence of PPM.Methods and Results:Altogether, 282 consecutive patients (113 women, mean age 69.9±9.9 years) underwent AVR with a CEPMs between 2008 and 2015. They were divided into 3 groups based on the risk of PPM as a result of their body surface area and aortic annular diameter (BSA/AnnD ratio): low-risk (LR) group: 0.64±0.05 m 2 /cm (n=94); medium-risk (MR) group: 0.73±0.02 m 2 /cm (n=94); high-risk (HR) group: 0.83±0.05 m 2 /cm (n=94). The 30-day mortality rate was 0.4%. The 5-year actuarial survival rates were 93.2%, 92.3%, and 94.8% for groups LR, MR, and HR, respectively. No explants as a result of structural valve deterioration occurred. No patients showed severe PPM, defined as a measured effective orifice area index (EOAI) <0.65 cm 2 /m 2 . Although there were significant (P<0.05) differences in EOAI (0.98±0.2, 0.90±0.21, and 0.88±0.1 cm 2 /m 2 among the LR, MR, and HR groups, respectively), the corresponding transvalvular mean pressure gradients (13.0±5.5, 12.3±4.0, 12.7±5.3 mmHg) and regression rates of the left ventricular mass (29.8%, 28.7%, 28.9%) were similar among groups. CEPMs provide low surgical risk and reduce the risks of PPM, even in HR patients, with excellent hemodynamics.

  2. 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

  3. Effects of Reiki on Pain, Anxiety, and Blood Pressure in Patients Undergoing Knee Replacement: A Pilot Study.

    Science.gov (United States)

    Baldwin, Ann Linda; Vitale, Anne; Brownell, Elise; Kryak, Elizabeth; Rand, William

    This blinded, controlled pilot study investigated the effects of Reiki on 46 patients undergoing knee replacement surgery. Of the 3 groups, Reiki, Sham Reiki, and Standard of Care, only the Reiki group showed significant reductions in pain, blood pressure, respiration rate, and state anxiety, which provides evidence for a full-scale clinical study.

  4. 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

  5. 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

  6. A comparison of early versus late initiation of renal replacement therapy in critically ill patients with acute kidney injury

    DEFF Research Database (Denmark)

    Karvellas, Constantine J; Farhat, Maha R; Sajjad, Imran

    2011-01-01

    Introduction: Our aim was to investigate the impact of early versus late initiation of renal replacement therapy (RRT) on clinical outcomes in critically ill patients with acute kidney injury (AKI). Methods: Systematic review and meta-analysis were used in this study. PUBMED, EMBASE, SCOPUS, Web ...

  7. 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.

  8. Reversal of atherogenic lipoprotein profile in HIV-1 infected patients with lipodystrophy after replacing protease inhibitors by nevirapine

    NARCIS (Netherlands)

    Negredo, Eugenia; Ribalta, Josep; Paredes, Roger; Ferré, Raimón; Sirera, Guillem; Ruiz, Lidia; Salazar, Juliana; Reiss, Peter; Masana, Lluís; Clotet, Bonaventura

    2002-01-01

    Background: The widespread use of protease inhibitors (PI) has been associated with abnormalities in the lipid profile of HIV-1-infected patients. Treatment simplification approaches in which PI are replaced by nevirapine (NVP) have been shown to improve PI-related toxicity. Objective: To assess the

  9. Predictors of clinical outcome in total hip and knee replacement : a methodological appraisal of implants and patient factors

    NARCIS (Netherlands)

    Keurentjes, Johan Christiaan

    2014-01-01

    In this thesis, we studied both implants and patient and surgeon factors as predictors of clinical outcome after total hip and knee replacement. Additionally, we studied a number of methodological aspects of orthopaedic research, such as competing risks in estimating the probability of revision

  10. 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.

  11. 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

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

    Science.gov (United States)

    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.

  13. 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

  14. Urea kinetics during sustained low-efficiency dialysis in critically ill patients requiring renal replacement therapy.

    Science.gov (United States)

    Marshall, Mark R; Golper, Thomas A; Shaver, Mary J; Alam, Muhammad G; Chatoth, Dinesh K

    2002-03-01

    Continuous renal replacement therapies have practical and theoretical advantages compared with conventional intermittent hemodialysis in hemodynamically unstable or severely catabolic patients with acute renal failure (ARF). Sustained low-efficiency dialysis (SLED) is a hybrid modality introduced July 1998 at the University of Arkansas for Medical Sciences that involves the application of a conventional hemodialysis machine with reduced dialysate and blood flow rates for 12-hour nocturnal treatments. Nine critically ill patients with ARF were studied during a single SLED treatment to determine delivered dialysis dose and the most appropriate model for the description of urea kinetics during treatment. Five patients were men, mean Acute Physiology and Chronic Health Evaluation (APACHE) II score was 28.9 and mean weight was 92.5 kg. Kt/V was determined by the reference method of direct dialysate quantification (DDQ) combined with an equilibrated postdialysis plasma water urea nitrogen (PUN) concentration and four other methods that were either blood or dialysate based, single or double pool, or model independent (whole-body kinetic method). Solute removal indices (SRIs) were determined from net urea removal and urea distribution volume supplied from DDQ (reference method) and by mass balance using variables supplied from blood-based formal variable-volume single-pool (VVSP) urea kinetic modeling. Equivalent renal urea clearances (EKRs) were calculated from urea generation rates and time-averaged concentrations for PUN based on weekly mass balance with kinetic variables supplied by either DDQ (reference method) or formal blood-based VVSP modeling. Mean Kt/V determined by the reference method was 1.40 and not significantly different when determined by formal VVSP modeling, DDQ using an immediate postdialysis PUN, or the whole-body kinetic method. Correction of single-pool Kt/V by a Daugirdas rate equation did not yield plausible results. Mean SRI and EKR by the

  15. 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.

  16. Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement.

    Science.gov (United States)

    Lee, Cheul; Kim, Yang Min; Lee, Chang-Ha; Kwak, Jae Gun; Park, Chun Soo; Song, Jin Young; Shim, Woo-Sup; Choi, Eun Young; Lee, Sang Yun; Baek, Jae Suk

    2012-09-11

    The objectives of this study were to evaluate outcomes of pulmonary valve replacement (PVR) in patients with chronic pulmonary regurgitation (PR) and to better define the optimal timing of PVR. Although PVR is effective in reducing right ventricular (RV) volume overload in patients with chronic PR, the optimal timing of PVR is not well defined. A total of 170 patients who underwent PVR between January 1998 and March 2011 for chronic PR were retrospectively analyzed. To define the optimal timing of PVR, pre-operative and post-operative cardiac magnetic resonance imaging (MRI) data (n = 67) were analyzed. The median age at the time of PVR was 16.7 years. Follow-up completeness was 95%, and the median follow-up duration was 5.9 years. Overall and event-free survival at 10 years was 98% and 70%, respectively. Post-operative MRI showed significant reduction in RV volumes and significant improvement in biventricular function. Receiver-operating characteristic curve analysis revealed a cutoff value of 168 ml/m(2) for non-normalization of RV end-diastolic volume index (EDVI) and 80 ml/m(2) for RV end-systolic volume index (ESVI). Cutoff values for optimal outcome (normalized RV volumes and function) were 163 ml/m(2) for RV EDVI and 80 ml/m(2) for RV ESVI. Higher pre-operative RV ESVI was identified as a sole independent risk factor for suboptimal outcome. Midterm outcomes of PVR in patients with chronic PR were acceptable. PVR should be considered before RV EDVI exceeds 163 ml/m(2) or RV ESVI exceeds 80 ml/m(2), with more attention to RV ESVI. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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

    Science.gov (United States)

    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.

  18. Physiologic Growth Hormone-Replacement Therapy and Craniopharyngioma Recurrence in Pediatric Patients: A Meta-Analysis.

    Science.gov (United States)

    Alotaibi, Nawaf M; Noormohamed, Nadia; Cote, David J; Alharthi, Salman; Doucette, Joanne; Zaidi, Hasan A; Mekary, Rania A; Smith, Timothy R

    2018-01-01

    A systematic review and meta-analysis were conducted to examine the effect of growth hormone-replacement therapy (GHRT) on the recurrence of craniopharyngioma in children. PubMed, Embase, and Cochrane databases were searched through April 2017 for studies that evaluated the effect of GHRT on the recurrence of pediatric craniopharyngioma. Pooled effect estimates were calculated with fixed- and random-effects models. Ten studies (n = 3487 patients) met all inclusion criteria, including 2 retrospective cohorts and 8 case series. Overall, 3436 pediatric patients were treated with GHRT after surgery and 51 were not. Using the fixed effect model, we found that the overall craniopharyngioma recurrence rate was lower among children who were treated by GHRT (10.9%; 95% confidence interval 9.80%-12.1%; I 2  = 89.1%; P for heterogeneity <0.01; n = 10 groups) compared with those who were not (35.2%; 95% confidence interval 23.1%-49.6%; I 2  = 61.7%; P for heterogeneity = 0.11; n = 3); the P value comparing the 2 groups was <0.01. Among patients who were treated with GHRT, subgroup analysis revealed that there was a greater prevalence of craniopharyngioma recurrence among studies conducted outside the United States (P < 0.01), single-center studies (P < 0.01), lower impact factor studies (P = 0.03), or studies with a lower quality rating (P = 0.01). Using the random-effects model, we found that the results were not materially different except for when stratifying by GHRT, impact factor, or study quality; this led to nonsignificant differences. Both Begg's rank correlation test (P = 0.7) and Egger's linear regression test (P = 0.06) indicated no publication bias. This meta-analysis demonstrated a lower recurrence rate of craniopharyngioma among children treated with GHRT than those who were not. Copyright © 2017 Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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.

  2. Salivary Cortisol Can Replace Free Serum Cortisol Measurements in Patients With Septic Shock

    Science.gov (United States)

    Orlander, Philip R.

    2011-01-01

    Background: There is a renewed interest in adrenal function during severe sepsis. Most studies have used total serum cortisol levels; however, only free serum cortisol is biologically active. The aim of this study was to determine the validity of salivary cortisol levels as a surrogate for free serum cortisol levels during septic shock. Methods: Fifty-seven patients with septic shock were studied to determine the correlation between total serum cortisol and salivary cortisol to free serum cortisol levels. Thirty-eight patients were included in the salivary to free serum cortisol correlation. Salivary cortisol level was tested by enzyme immunoassay. Serum total cortisol, free cortisol, and cortisol-binding globulin (CBG) levels were determined by liquid chromatography-mass spectrometry, equilibrium analysis, and radioimmunoassay, respectively. Results: The mean ± SD age was 56.6 ± 18.5 years. Fifty-seven percent were women. APACHE (Acute Physiology and Chronic Health Evaluation) II score median was 26, Simplified Acute Physiology Score II median was 61, and Sequential Organ Failure Assessment median was 13. The correlation between salivary and free serum cortisol levels was 0.79 (95% CI, 0.63-0.89; P cortisol and total serum cortisol levels was 0.86 (95% CI, 0.78-0.92; P cortisol level was 2.27 ± 1.64 μg/dL. The mean ± SD salivary cortisol level was 2.60 ± 2.69 μg/dL. The mean ± SD total serum cortisol level was 21.56 ± 8.71 μg/dL. The mean ± SD CBG level was 23.54 ± 8.33 mg/dL. Conclusions: Salivary cortisol level can be used as a surrogate of free serum cortisol level in patients with septic shock with very good correlation. Salivary cortisol testing is noninvasive, easy to perform, and can be conducted daily. Trial registry: ClinicalTrials.gov; No.: NCT00523198; URL: www.clinicaltrials.gov PMID:21816912

  3. Feasibility of replacing patient specific cutouts with a computer-controlled electron multileaf collimator

    International Nuclear Information System (INIS)

    Eldib, Ahmed; Jin Lihui; Li Jinsheng; Ma, C-M Charlie

    2013-01-01

    target was showing a mean percentage difference of 1.57%±1.65, while the difference in the dose received by 99% of the volume was showing a mean percentage difference of 1.08%±0.78. The mean percentage volume of Lung receiving a percentage dose equal to or greater than 20% of the prescribed dose was found to be 8.55%±7.3 and 8.67%±7 for the eMLC and applicator/cutout combination delivery methods respectively. Results have shown that target coverage and critical structure sparing can be effectively achieved by electron beams collimated with the eMLC. Positioning the eMLC leaves in such a way to avoids shielding any part of the projected treatment volume is most conservative and would be the recommended method to define the actual leaf position for the eMLC defined field. More optimal leaf positions can be achieved in shaping the same treatment field through the interplay of different leaf positioning strategies. We concluded that the eMLC represents an effective time saving and pollution-free device that can completely replace patient specific cutouts. (paper)

  4. Feasibility of replacing patient specific cutouts with a computer-controlled electron multileaf collimator

    Science.gov (United States)

    Eldib, Ahmed; Jin, Lihui; Li, Jinsheng; Ma, C.-M. Charlie

    2013-08-01

    target was showing a mean percentage difference of 1.57%±1.65, while the difference in the dose received by 99% of the volume was showing a mean percentage difference of 1.08%±0.78. The mean percentage volume of Lung receiving a percentage dose equal to or greater than 20% of the prescribed dose was found to be 8.55%±7.3 and 8.67%±7 for the eMLC and applicator/cutout combination delivery methods respectively. Results have shown that target coverage and critical structure sparing can be effectively achieved by electron beams collimated with the eMLC. Positioning the eMLC leaves in such a way to avoids shielding any part of the projected treatment volume is most conservative and would be the recommended method to define the actual leaf position for the eMLC defined field. More optimal leaf positions can be achieved in shaping the same treatment field through the interplay of different leaf positioning strategies. We concluded that the eMLC represents an effective time saving and pollution-free device that can completely replace patient specific cutouts.

  5. 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.

  6. 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.

  7. 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

  8. 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.

  9. 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.

  10. Effects of brown fish meal replacement with fermented soybean meal on growth performance, feed efficiency and enzyme activities of Chinese soft-shelled turtle, Pelodiscus sinensis

    Science.gov (United States)

    Zou, Yurong; Ai, Qinghui; Mai, Kangsen; Zhang, Wenbing; Zhang, Yanjiao; Xu, Wei

    2012-06-01

    A 120-day feeding experiment was conducted to investigate the effects of partial replacement of brown fish meal (BFM) by fermented soybean meal (FSBM) in diets of Chinese soft-shelled turtle ( Pelodiscus sinensis). The turtles (initial mean body weight, (115.52 ± 1.05) g) were fed with three experimental diets, in which 0%, 4.72% and 9.44% BFM protein was replaced by 0%, 3% and 6% FSBM, respectively. Results showed that the feeding rate (FR), specific growth rate (SGR) and feed efficiency ratio (FER) of turtles fed with the diet containing 3% FSBM were not significantly different from the control group (0% FSBM) ( P > 0.05). However, FR, SGR and FER of turtles fed with the diet containing 6% FSBM were significantly lower than those of the control group ( P 0.05). However, the uric acid concentration in turtles fed with the diet containing 3% or 6% FSBM was significantly lower than that in the control group ( P 0.05). The results suggested that FSBM could replace 4.72% BFM protein in turtle diets without exerting adverse effects on turtle growth, feed utilization and measured immune parameters.

  11. Angiotensin-converting enzyme inhibition improves cardiac fatty acid metabolism in patients with congestive heart failure.

    Science.gov (United States)

    Yamauchi, S; Takeishi, Y; Minamihaba, O; Arimoto, T; Hirono, O; Takahashi, H; Miyamoto, T; Nitobe, J; Nozaki, N; Tachibana, H; Watanabe, T; Fukui, A; Kubota, I

    2003-08-01

    This study aimed to examine whether angiotensin-converting enzyme (ACE) inhibition improved cardiac fatty acid metabolism in patients with congestive heart failure (CHF). Myocardial 123I-beta-methyl-iodophenylpentadecanoic acid (123I-BMIPP) imaging was performed in 25 patients with CHF and in 10 control subjects. Myocardial 123I-BMIPP images were obtained 30 min and 4 h after tracer injection. The heart-to-mediastinum (H/M) ratio of 123I-BMIPP uptake and the washout rate of 123I-BMIPP from the myocardium were calculated. Patients were given enalapril for 6 months, and 123I-BMIPP imaging was repeated. H/M ratios on early and delayed images were lower in CHF patients than in normal controls (Pacid metabolism by ACE inhibition may represent a new mechanism for the beneficial effect of this therapy in heart failure.

  12. The influence of a balanced volume replacement concept on inflammation, endothelial activation, and kidney integrity in elderly cardiac surgery patients.

    Science.gov (United States)

    Boldt, Joachim; Suttner, Stephan; Brosch, Christian; Lehmann, Andreas; Röhm, Kerstin; Mengistu, Andinet

    2009-03-01

    A balanced fluid replacement strategy appears to be promising for correcting hypovolemia. The benefits of a balanced fluid replacement regimen were studied in elderly cardiac surgery patients. In a randomized clinical trial, 50 patients aged >75 years undergoing cardiac surgery received a balanced 6% HES 130/0.42 plus a balanced crystalloid solution (n = 25) or a non-balanced HES in saline plus saline solution (n = 25) to keep pulmonary capillary wedge pressure/central venous pressure between 12-14 mmHg. Acid-base status, inflammation, endothelial activation (soluble intercellular adhesion molecule-1, kidney integrity (kidney-specific proteins glutathione transferase-alpha; neutrophil gelatinase-associated lipocalin) were studied after induction of anesthesia, 5 h after surgery, 1 and 2 days thereafter. Serum creatinine (sCr) was measured approximately 60 days after discharge. A total of 2,750 +/- 640 mL of balanced and 2,820 +/- 550 mL of unbalanced HES were given until the second POD. Base excess (BE) was significantly reduced in the unbalanced (from +1.21 +/- 0.3 to -4.39 +/- 1.0 mmol L(-1) 5 h after surgery; P volume replacement strategy including a balanced HES and a balanced crystalloid solution resulted in moderate beneficial effects on acid-base status, inflammation, endothelial activation, and kidney integrity compared to a conventional unbalanced volume replacement regimen.

  13. Effects of dietary fish oil replacement by vegetable oil on the digestive enzymes activity and intestinal morphology in Meagre, Argyrosomus regius (Asso, 1801

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    Fernando Antunes Magalhães

    2014-07-01

    The results were analyzed by three way factorial. Amylase activity was bigger in FO when compared with VO (Table 1. The same result was observed in chymotrypsin activity. On the other hand, lipase activity was higher in VO. Regarding the levels of lipids, diets with 17% had higher amylase activity than diets with 12%. The inverse was observed in chymotrypsin activity. In relation to lipase activity, no differences were observed on the two levels of lipids studied. No differences in digestive enzymes activities were observed when diets were supplemented with selenium. Epithelium architecture of the posterior intestine was slightly affected by dietary treatments. Higher levels of lipids seem to induce enterocyte vacuolization, and vacuoles seem to be larger when a blend of vegetable oils was used instead of fish oil. No clear role can be attributed to selenium regarding intestinal morphology. In conclusion, our study showed that the source and levels of lipid in diets for meagre have influence in activity of digestible enzymes like amylase, lipase and chymotrypsin. Furthermore, levels of selenium do not cause an alteration in studied digestible enzymes.

  14. 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.

  15. Ankle replacement

    Science.gov (United States)

    Ankle arthroplasty - total; Total ankle arthroplasty; Endoprosthetic ankle replacement; Ankle surgery ... Ankle replacement surgery is most often done while you are under general anesthesia. This means you will ...

  16. 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.

  17. Patient Recall of Informed Consent at 4 Weeks After Total Hip Replacement With Standardized Versus Procedure-Specific Consent Forms.

    Science.gov (United States)

    Pomeroy, Eoghan; Shaarani, Shahril; Kenyon, Robert; Cashman, James

    2017-08-25

    Informed consent plays a pivotal role in the operative process, and surgeons have an ethical and legal obligation to provide patients with information to allow for shared decision-making. Unfortunately, patient recall after the consent process is frequently poor. This study aims to evaluate the effect of procedure-specific consent forms on patient's recall four weeks after total hip replacement (THR). This is a prospective study using a posttest-only control group design. Sixty adult patients undergoing total hip replacement were allocated to be consented using either the generic or the surgery-specific consent form. Four weeks after surgery, a phone interview was conducted to assess patient's recall of risk of surgical complications. Patient demographic characteristics and educational attainment were similar in both groups. There was a statistically significant increase in the mean number of risks recalled in the study group at 1.43 compared with 0.67 in the control group (P = 0.0131). Consent is a complex process, and obtaining informed consent is far from straightforward. A statistically significant improvement in patient's recall with the use of procedure-specific consent forms was identified, and based on this, we would advocate their use. However, overall patient recall in both groups was poor. We believe that improving the quality of informed consent may require the sum of small gains, and the use of procedure-specific consent forms may aid in this regard.

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

    Science.gov (United States)

    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

  19. 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.

  20. 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.

  1. 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

  2. Systematic review of the outcome of aortic valve replacement in patients with aortic stenosis

    NARCIS (Netherlands)

    Sharma, Umesh C.; Barenbrug, Paul; Pokharel, Saraswati; Dassen, Willem R. M.; Pinto, Yigal M.; Maessen, Jos G.

    2004-01-01

    BACKGROUND: After the establishment of aortic valve replacement procedure for aortic stenosis, there are heterogeneous studies and varying reports on outcome. An analysis that compares individual studies to summarize the overall effect is still lacking. This study systematically analyzes the change

  3. The use of clinical analysis of movements in evaluation of motor functional status of patients after total hip replacement

    Directory of Open Access Journals (Sweden)

    Romakina N.A.

    2016-06-01

    Full Text Available Aim: to estimate functional status of coxarthrosis in patients requiring total hip replacement of the two hip joints. Material and methods. The biomechanical examination of 94 patients with bilateral primary coxarthrosis before and after total hip replacement was performed using clinical stabilometric software complex. The ability to perceive the mechanical load during standing and walking was evaluated at different stages of the treatment. The difference between the samples was estimated with the use of Mann — Whitney U-test. The rank correlation of biomechanical parameters was measured by Spearman coefficient. Results. It was revealed that the most responsive indicators are the transfer period, the first and second periods of double support and the deviation of the center of pressure relative to the average position in the frontal plane. Conclusion. After surgical treatment there was observed some improvement: the-left-and-right-step asymmetry decrease, rhythm rate increase, improved ability to maintain body balance, jog reactions increase.

  4. Measurement and purification of Alanine aminotransferase (ALT enzyme activity in patients with celiac disease

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    Taghreed U. Mohammed

    2017-09-01

    Full Text Available Celiac disease (CD is the most common genetically - based disease in correlation with food intolerance. The aim of this study is to measure the activity of ALT enzyme and purify enzyme from sera women with celiac disease. Alanine aminotransferase (ALT activity has been assayed in (30 women serum samples with celiac disease, age range between (20-40 year and (30 serum of healthy women as control group, age range between (22-38 year. In the present study, the mean value of ALT activity was significantly higher in patients with celiac disease than healthy group (p<0.01. The ALT enzyme was partial purified from sera women with celiac disease by dialysis, gel filtration using Sephadex G- 50 and ion exchange chromatography using DEAE- cellulose A-50 . The results showed a single peak by using gel filtration and the activity reached 31-15 U/L .Two isoenzymes were obtained by using ion exchange chromatography and the purity degree of isoenzymse (I, II were (5.7 and (5.53 fold respectively

  5. 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.

  6. Liver enzymes serum levels in patients with chronic kidney disease on hemodialysis: a comprehensive review

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    Luís Henrique Bezerra Cavalcanti Sette

    2014-04-01

    Full Text Available We reviewed the literature regarding the serum levels of the enzymes aspartate aminotransferase, alanine aminotransferase, and gamma-glutamyl transferase in patients with chronic kidney disease on hemodialysis with and without viral hepatitis. Original articles published up to January 2013 on adult patients with chronic kidney disease on hemodialysis were selected. These articles contained the words “transaminases” “aspartate aminotransferase” “alanine aminotransferase” “gamma glutamyl transferase,” “liver enzymes”, AND “dialysis” OR “hemodialysis”. A total of 823 articles were retrieved. After applying the inclusion and exclusion criteria, 49 articles were selected. The patients with chronic kidney disease on hemodialysis had reduced serum levels of aminotransferases due to hemodilution, lower pyridoxine levels, or elevated homocysteine levels. The chronic kidney disease patients on hemodialysis infected with the hepatitis C virus also had lower aminotransferase levels compared with the infected patients without chronic kidney disease. This reduction is in part due to decreased viremia caused by the dialysis method, the production of a hepatocyte growth factor and endogenous interferon-α, and lymphocyte activation, which decreases viral action on hepatocytes. Few studies were retrieved on gamma-glutamyl transferase serum levels; those found reported that there were no differences between the patients with or without chronic kidney disease. The serum aminotransferase levels were lower in the patients with chronic kidney disease on hemodialysis (with or without viral hepatitis than in the patients with normal renal function; this reduction has a multifactorial origin.

  7. Pre- and postoperative ventilation-perfusion scan findings in patients undergoing total hip replacement or knee arthroplasty

    International Nuclear Information System (INIS)

    Kim, S.M.; Park, C.H.; Intenzo, C.M.

    1988-01-01

    Venous thrombolembolism is one of the major postoperative complications in patients undergoing total hip replacement (THR) or knee anthroplasty (TKA). The reported incidence of pulmonary embolism in this group is as high as 20%. The purpose of this report was to evaluate the value of preoperative and 7th-day postpoperative ventilation-perfusion (V/Q) lung scans in the management of patients undergoing elective reconstructive surgery of the hips or knees. Routine preoperative and 7th-day postoperative V/Q lung scans were obtained in 34 patients who underwent THR (17 patients) or TKA (17 patients). There were 15 male and 19 female patients, with an age distribution ranging from 56 to 80 years. Chest radiographs were obtained within 1 day of the pre- or postoperative lungs scan. Lung scans were interpreted by two experienced nuclear physicians

  8. PRIMARY ENDOPROSTHETIC REPLACEMENT OF THE ANOPHTHALMIC ORBIT IN PATIENTS WITH UVEAL MELANOMA: SIX-YEAR FOLLOW-UP RESULTS

    Directory of Open Access Journals (Sweden)

    A. A. Yarovoy

    2012-01-01

    Full Text Available A locomotor stump was formed in 36 patients (28 women and 8 men, by implanting an endoprosthesis for enucleation of the eyeball with uveal melanoma (UM. The indication for endoprosthesis implantation was no signs of extrabulbar growth. A modified 17–19 mm silicone implant covered with strips from a dura mater graft and medical mesh fabric was used as an orbital implant. The follow-up was 3 to 72 months (mean 32.5 months. All the patients achieved a satisfactory cosmetic effect. None patient was found to have a recurrent orbital tumor. Out of the complications, anterior implant surface denudation was noted in 4 patients. Two patients developed metastases. The absence of recurrent orbital UM at a 6-year follow-up enables primary endoprosthetic replacement of the orbit for UM to be regarded as a safe and reasonable method for patient cosmetic rehabilitation. 

  9. Successful Tricuspid Valve Replacement in a Patient with Severe Pulmonary Arterial Hypertension and Preserved Right Ventricular Systolic Function

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    Jamil A. Aboulhosn

    2009-01-01

    Full Text Available A 56-year-old patient with severe pulmonary hypertension developed severe tricuspid regurgitation, right-sided heart failure, and congestive hepatopathy. She was transferred for possible lung transplant and/or tricuspid valve surgery. Clinical and echocardiographic assessment provided confidence that acute tricuspid valve failure was responsible for the decompensation and that tricuspid valve replacement despite pulmonary hypertension could be performed.

  10. Valve-sparing aortic root replacement and aortic valve repair in a patient with acromegaly and aortic root dilatation

    Directory of Open Access Journals (Sweden)

    Karel Van Praet

    2015-07-01

    Full Text Available Aortic regurgitation and dilatation of the aortic root and ascending aorta are severe complications of acromegaly. The current trend for management of an aortic root aneurysm is valve-sparing root replacement as well as restoring the diameter of the aortic sinotubular junction (STJ and annulus. Our case report supports the recommendation that in patients with acromegaly, severe aortic root involvement may indicate the need for surgery.

  11. Identification of Genes Coding Aminoglycoside Modifying Enzymes in E. coli of UTI Patients in India

    Directory of Open Access Journals (Sweden)

    Abdul Rouf Mir

    2016-01-01

    Full Text Available This study is to probe the pattern of antibiotic resistance against aminoglycosides and its mechanism in E. coli obtained from patients from Chennai, India. Isolation and identification of pathogens were done on MacConkey agar. Antimicrobial sensitivity testing was done by disc diffusion test. The identification of genes encoding aminoglycoside modifying enzymes was done by Polymerase Chain Reaction (PCR. Out of 98 isolates, 71 (72.45% isolates were identified as E. coli and the remaining 27 (27.55% as other bacteria. Disc diffusion method results showed a resistance level of 72.15% for streptomycin, 73.4% for gentamicin, 63.26% for neomycin, 57.14% for tobramycin, 47.9% for netilmicin, and 8.16% for amikacin in E. coli. PCR screening showed the presence of four genes, namely, rrs, aacC2, aacA-aphD, and aphA3, in their plasmid DNA. The results point towards the novel mechanism of drug resistance in E. coli from UTI patients in India as they confirm the presence of genes encoding enzymes that cause resistance to aminoglycoside drugs. This could be an alarm for drug prescription to UTI patients.

  12. Identification of Genes Coding Aminoglycoside Modifying Enzymes in E. coli of UTI Patients in India.

    Science.gov (United States)

    Mir, Abdul Rouf; Bashir, Yasir; Dar, Firdous Ahmad; Sekhar, M

    This study is to probe the pattern of antibiotic resistance against aminoglycosides and its mechanism in E. coli obtained from patients from Chennai, India. Isolation and identification of pathogens were done on MacConkey agar. Antimicrobial sensitivity testing was done by disc diffusion test. The identification of genes encoding aminoglycoside modifying enzymes was done by Polymerase Chain Reaction (PCR). Out of 98 isolates, 71 (72.45%) isolates were identified as E. coli and the remaining 27 (27.55%) as other bacteria. Disc diffusion method results showed a resistance level of 72.15% for streptomycin, 73.4% for gentamicin, 63.26% for neomycin, 57.14% for tobramycin, 47.9% for netilmicin, and 8.16% for amikacin in E. coli. PCR screening showed the presence of four genes, namely, rrs, aacC2, aacA-aphD, and aphA3, in their plasmid DNA. The results point towards the novel mechanism of drug resistance in E. coli from UTI patients in India as they confirm the presence of genes encoding enzymes that cause resistance to aminoglycoside drugs. This could be an alarm for drug prescription to UTI patients.

  13. 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.

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. 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.

  19. Clinical efficacy of transcatheter aortic valve replacement for severe aortic stenosis in high-risk patients: the PREVAIL JAPAN trial.

    Science.gov (United States)

    Sawa, Yoshiki; Takayama, Morimasa; Mitsudo, Kazuaki; Nanto, Shinsuke; Takanashi, Shuichiro; Komiya, Tatsuhiko; Kuratani, Toru; Tobaru, Tetsuya; Goto, Tsuyoshi

    2015-01-01

    Transcatheter aortic valve replacement (TAVR) is suggested to be less invasive and/or equally effective in comparison to conventional aortic valve replacement for high-risk symptomatic aortic stenosis patients. We herein report the initial results of a pivotal clinical trial of TAVR in Japan (the PREVAIL JAPAN). Sixty-four aortic stenosis patients (mean age 84.3 ± 6.1 years) not suitable for surgery were enrolled at three centers in Japan, with a primary composite endpoint of the 6-month post-procedure improvements in the aortic valve area and New York Heart Association (NYHA) functional classification. A transfemoral approach was used in 37 patients and a transapical approach was used in 27. The device success rate was 91.9 %. After 30 days and 6 months, the rates of mortality from any cause were 8.1 and 11.3 %, respectively. At 6 months, symptomatic stroke was found in 3.1 % of the patients, and silent infarction in 7.8 %. The aortic valve area and mean pressure gradient were significantly improved over time with both approaches (p < 0.001). At 6 months, the NYHA functional classification based on a conventional physician's assessment was improved in 87.9 % of the patients. We found results that were equivalent to those in other major TAVR trials, such as an acceptable 30-day survival (91.9 %), suggesting that balloon-expandable TAVR is effective for small Japanese AS patients classified as high-risk or inoperable.

  20. 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.

  1. Outcome and prognostic factors of critically ill patients with acute renal failure requiring continuous renal replacement therapy

    Directory of Open Access Journals (Sweden)

    Aldawood Abdulaziz

    2010-01-01

    Full Text Available Continuous renal replacement therapy (CRRT has proved to be beneficial for the treatment of critically ill patients with acute renal failure (ARF. The aim of this study is to determine the outcome and identify the predictors of mortality of critically ill patients treated with CRRT for ARF in the intensive care unit (ICU. This prospective cohort study of critically ill patients with ARF requiring CRRT admitted to the ICU was carried out at a tertiary care hospital in Saudi Arabia from 2002 to 2008. A total of 644 of 7173 patients with ARF required CRRT were studied. About 9% of the ARF patients required CRRT and comprised mainly those with medical causes, carrying a mortality of 64%. Multivariate analysis found high serum creatinine as an independent factor for better outcome and requirement of mechanical ventilation (MV as an independent factor for worse outcome. In our cohort study, ARF requiring CRRT in the ICU was associated with a high mortality.

  2. 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”?

  3. 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"?

  4. [Effect of Transcutaneous Acupoint Electrical Stimulation on Hemodynamic Fluctuation Caused by Loosing Tourniquet in Elderly Patients Undergoing Knee Joint Replacement].

    Science.gov (United States)

    Liang, Han-Sheng; Feng, Yi

    2017-12-25

    To observe the effect of transcutaneous acupoint electrical stimulation (TAES) on hemodynamic fluctuation caused by loosing tourniquet in the elderly patients undergoing knee joint replacement. A total of 60 ASA (America Society Anesthesiologist) I or II elderly patients for elective knee joint replacement surgery were randomly divided into control group (30 cases) and TAES group (30 cases). Patients of both groups were treated by intravenous anesthesia, and monitored with bispectral index (BIS, between 45-60) for anesthesia depth, stroke volume variation (SVV) for fluid management, mean arterial pressure (MAP) and cardiac index (CI) for hemodynamic fluctuation evaluation, and with analgesia nociception index (ANI, between 50-70) for remifentanil dosage adjustment. TAES (2 Hz/100 Hz, 8-20 mA) was applied to bilateral Xinshu (BL 15), Feishu (BL 13), Neiguan (PC 6) and Hegu (LI 4) acupoints for 30 min first (followed by anesthesia induction and operation), and given continuously until 15 min after tourniquet loosing. Patients of the control group were only given with electrodes attachment without electrical stimulation. The levels of MAP, CI, and arterial blood pH, PaCO 2 , PaO 2 , base excess (BE) and lactic acid (Lac) 1 min before, and 5 and 15 min after tourniquet loosing, and the dosages of remifentanil and ephedrine after tourniquet loosing were recorded. The changed levels of MAP, CI and blood Lac at 5 min after tourniquet loosing (relevant to the baseline levels), and blood Lac content at 15 min after tourniquet loosing (relevant to 5 min after tourniquet loosing) were significantly lower in the TAES group than in the control group ( P 0.05). TAES has a positive effect on hemodynamics fluctuation caused by loosing tourniquet in the aged patients undergoing knee joint replacement.

  5. 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.

  6. Clinical predictors of prosthesis-patient mismatch after aortic valve replacement for aortic stenosis

    Directory of Open Access Journals (Sweden)

    Luis M Astudillo

    2012-01-01

    Full Text Available OBJECTIVE: We sought to ascertain predictors of Patient Prosthesis Mismatch, an independent predictor of mortality, in patients with aortic stenosis using bioprosthetic valves. METHOD: We analyzed 2,107 sequential surgeries. Patient Prosthesis Mismatch was calculated using the effective orifice area of the prosthesis divided by the patient's body surface area. We defined nonsignificant, moderate, and severe Patient Prosthesis Mismatch as effective orifice area indexes of .0.85 cm²/m, 0.85-0.66 cm²/m², and <0.65 cm²/m², respectively. RESULTS: A total of 311 bioprosthetic patients were identified. The incidence of nonsignificant, moderate, and severe Patient Prosthesis Mismatch was 41%, 42, and 16%, respectively. Severe Patient Prosthesis Mismatch was significantly more prevalent in females (82%. In severe Patient Prosthesis Mismatch, the perfusion and the crossclamp times were considerably lower when compared with nonsignificant Patient Prosthesis Mismatch and moderate Patient Prosthesis Mismatch. Patients with severe Patient Prosthesis Mismatch had a significantly higher likelihood of spending time in the intensive care unit and a significantly longer length of stay in the hospital. Body surface area was not different in severe Patient Prosthesis Mismatch when compared with nonsignificant Patient Prosthesis Mismatch. In-hospital mortality in patients with nonsignificant, moderate, and severe Patient Prosthesis Mismatch was 2.3%, 6.1%, and 8%, respectively. Minimally invasive surgery was significantly associated with moderate Patient Prosthesis Mismatch in 49% of the patients, but not with severe Patient Prosthesis Mismatch. CONCLUSION: Severe Patient Prosthesis Mismatch is more common in females, but not in those with minimal available body surface area. Though operative times were shorter in these patients, intensive care unit and hospital lengths of stay were longer. Surgeons and cardiologists should be cognizant of these clinical

  7. Onco plastic volume replacement with latissimus dorsi myocutaneous flap in patients with large ptotic breasts. Is it feasible?

    International Nuclear Information System (INIS)

    El-Marakby, H.H.; Kotb, M.H.

    2011-01-01

    Onco plastic breast conservative surgery has evolved as a safe alternative to the standard mastectomy in the treatment of early breast cancer. The procedure involves tumour resection with an adequate safety margin and either breast reshaping with volume displacement procedures (large or pt otic breasts) or volume replacement with latissimus dorsi myocutaneous flap (LDF) (small to medium sized non-ptotic breasts). A contra lateral mastopexy procedure is usually necessary with the volume displacement Onco plastic surgery, a procedure that is often rejected by a significant number of patients. This limits the choice of the reconstruction of breast defects in such patients to autologous tissues i.e. LDF. Aim: Aim is to evaluate the feasibility of volume replacement onco plastic breast conservative surgery with lemmatise dorsi myocutaneous flaps for patients with large ptotic breasts. This involves testing the oncologic safety in terms of adequate safety margin, the complications rate and the final cosmetic outcome. The loco regional recurrence rate will be recorded and compared with oncoplastic volume displacement for similar sized breast defects. Patients and methods: A group of 50 female patients with early breast cancers (T2) who presented to the department of surgery at the National Cancer Institute, Cairo, Egypt in the period between carried out in all patient groups and was used to annually follow up the patients. All patients were detected with T2 NO breast cancer by both clinical and radiological examinations. All patients underwent partial mastectomy was and reconstruction with LDFs. Results: The average age at presentation was 46.5 ± 9 years and the range was 26-65 years. Most of the patients were subjected to partial mastectomy in 30 patients (60%), excision of a single quadrant from the four major quadrants was carried out in 15 patients (30%) where skin sparing wide local excision was carried out in only five patients (10%). The safety margin ranged from

  8. Determinants of anxiety in patients with advanced somatic disease: differences and similarities between patients undergoing renal replacement therapies and patients suffering from cancer.

    Science.gov (United States)

    Janiszewska, Justyna; Lichodziejewska-Niemierko, Monika; Gołębiewska, Justyna; Majkowicz, Mikołaj; Rutkowski, Bolesław

    2013-10-01

    Anxiety is the most frequent emotional reaction to the chronic somatic disease. However, little is known about anxiety and coping strategies in patients with end-stage renal disease (ESRD) undergoing renal replacement therapies (RRTs). The purpose of the study was to assess the intensity and determinants of anxiety in patients treated with different RRTs in comparison with end-stage breast cancer patients and healthy controls. The study involved (1) ESRD patients undergoing different RRTs: 32 renal transplant recipients, 31 maintenance haemodialysis and 21 chronic peritoneal dialysis patients, (2) women with end-stage breast cancer (n = 25) and (3) healthy persons (n = 55). We used State-Trait Anxiety Inventory, Scale of Personal Religiousness, Mental Adjustment to Cancer Scale, Rotterdam Symptom Checklist with reference to medical history. The data thus obtained were analysed using the analysis of variance, the Tukey's HSD post hoc test and Spearman's rank correlation coefficient. Both ESRD and breast cancer patients revealed higher level of anxiety state and trait than healthy controls; however, there was no statistically significant difference found between both findings. There was a tendency towards higher levels of anxiety state in breast cancer patients when compared to ESRD patients undergoing the RRT treatment and for both groups non-constructive coping strategies correlated with the levels of anxiety state. With ESRD patients undergoing RRTs, the intensity of anxiety state did not depend on the mode of treatment but on the correlation between the levels of anxiety and the general quality of their life, psychological condition and social activity. In patients with advanced somatic disease (ESRD and end-stage breast cancer), non-constructive strategies of coping with the disease require further evaluation and possibly psychological support.

  9. Effect of Different Stages of Chronic Kidney Disease and Renal Replacement Therapies on Oxidant-Antioxidant Balance in Uremic Patients

    Directory of Open Access Journals (Sweden)

    Hadja Fatima Tbahriti

    2013-01-01

    Full Text Available Oxidative stress seems to be involved in the path physiology of cardiovascular complications of chronic kidney disease (CKD. In this study, we determined the effect of different stages of CKD and substitutive therapies on oxidative stress. One hundred sixty-seven patients (age: 44±06 years; male/female: 76/91 with CKD were divided into 6 groups according to the National Kidney Foundation classification. Prooxidant status was assessed by assaying thiobarbituric acid reactive substances, hydroperoxides, and protein carbonyls. Antioxidant defence was performed by analysis of superoxide dismutase, catalase, glutathione peroxidase, glutathione reductase, vitamin E, Iron, and bilirubin. TBARS and LPO were higher in HD patients compared to other groups (P<0.001, while protein carbonyls were more increased in PD patients. The antioxidant enzymes were declined already at severe stage of CKD and they were declined notably in HD patients (P<0.001. Similar observation was found for vitamin E, Fe, and bilirubin where we observed a significant decrease in the majority of study groups, especially in HD patients (P<0.001. The evolution of CKD was associated with elevated OS. HD accentuates lipid, while PD aggravates protein oxidation. However, the activity of antioxidant enzymes was altered by impaired renal function and by both dialysis treatments.

  10. Ventricular arrhythmia risk stratification in patients with tetralogy of Fallot at the time of pulmonary valve replacement.

    Science.gov (United States)

    Sabate Rotes, Anna; Connolly, Heidi M; Warnes, Carole A; Ammash, Naser M; Phillips, Sabrina D; Dearani, Joseph A; Schaff, Hartzell V; Burkhart, Harold M; Hodge, David O; Asirvatham, Samuel J; McLeod, Christopher J

    2015-02-01

    Most patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR), but the evaluation for and management of ventricular arrhythmia remain unclear. This study is aimed at clarifying the optimal approach to this potentially life-threatening issue at the time of PVR. A retrospective analysis was performed on 205 patients with repaired tetralogy of Fallot undergoing PVR at our institution between 1988 and 2010. Median age was 32.9 (range, 25.6) years. Previous ventricular tachycardia occurred in 16 patients (8%) and 37 (16%) had left ventricular dysfunction, defined as left ventricular ejection fraction tetralogy of Fallot undergoing PVR with history of ventricular tachycardia or left ventricular dysfunction appear to be associated with a higher risk of arrhythmic events after operation. Events in the first year after PVR are rare, and in select high-risk patients, surgical cryoablation does not seem to increase arrhythmic events and may be protective. © 2014 American Heart Association, Inc.

  11. Relatives in older patients' fast-track treatment programme during total hip or knee replacement. A grounded theory study

    DEFF Research Database (Denmark)

    Berthelsen, Connie Bøttcher

    and considerate support in concern for the patients; Substituting mode, with practical and cognitive support; and Adapting mode, by trying to fit in with the patients’ and health professionals’ requirements. Study II: 16 patients aged 70 to 94 were included and data was collected through 15 non......The aim of this Ph.D.-dissertation was to generate grounded theories of relatives, patients, and health professionals’ pattern of behaviour, respectively, in relation to the relatives of older patients’ fast-track treatment programmes during total hip or knee replacement. The dissertation includes...... to the older patients’ self-determination of being autonomous and how they adapt and are perceived in the health professionals’ environment. This may be useful to the health professionals in orthopaedic fast-track treatment programmes and their future collaboration with older patients and their relatives....

  12. PERIPROSTHETIC INFECTION AFTER ENDOPROSTHETIC REPLACEMENT OF THE HIP JOINT IN PATIENTS WITH RHEUMATOID DISEASES

    Directory of Open Access Journals (Sweden)

    Vadim Petrovich Pavlov

    2013-01-01

    Full Text Available Periprosthetic infection (PI in patients with rheumatoid diseases (RD after total hip joint endoprosthesis (THJE is a relevant problem of rheumoorphopedics that has not been solved yet. The relative assessment of PI incidence rate after THJE and treatment outcomes in patients with this complication is expected to be carried out. A total of 1201 THJE performed in 1069 patients with RD are considered. The female : male ratio was 3.6 : 1; the mean age was 49.6 years (range: 16 to 83 years. 323 patients had rheumatoid arthritis (RA; 124 patients had juvenile rheumatoid arthritis (JRA; 80 patients had ankylosing spondylitis (AS; 79 patients had systemic lupus erythematosus (SLE; 11 patients had systemic sclerodermatitis (SSD; 18 patients had psoriatic arthropathy (PsA; and 353 patients had osteoarthritis (OA. Other RD were revealed in 81 patients. PI were detected in 9 (0.84% of 1069 patients with RD, including 7 (2.17% of 323 patients with RA; 1 (0.8% of 124 patients with JRA; and 1 (0.28% of 353 patients with OA. No PI were detected in 81 patients who had other RD. An eightfold (in patients with RA and a threefold (in patients with JRA increase in PI compared to that in patients with OA attests to the high risk of this complication in patients with inflammatory as compared to the patients with degenerative RD. After integrated treatment (revision surgery, sanation, continuous irrigation with antibiotics/lavasept, the graft was preserved in OA patients; the complication was prevented in RA patients. Six patients will be subjected to repeated revision surgery; one patient died of diabetes complications after the graft had been removed. A significant increase in PI incidence rate after THJE in patients with RA and JRA as compared to that in OA patients allows one to regard inflammatory RD as risk factors of this complication. The low effectiveness of the procedure for integrated therapy for PI in RD patients indicates that it needs to

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

    Science.gov (United States)

    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.

  14. 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...

  15. 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.

  16. How best to use partial meal replacement in managing overweight or obese patients with poorly controlled type 2 diabetes.

    Science.gov (United States)

    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.

  17. 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...

  18. Associated liver enzymes with hyperlipidemic profile in type 2 diabetes patients.

    Science.gov (United States)

    Al-Jameil, Noura; Khan, Farah A; Arjumand, Sadia; Khan, Mohammad F; Tabassum, Hajera

    2014-01-01

    Type 2 diabetes mellitus (T2DM) is characterized by hyperglycemia and is associated with dyslipidemia and disturbed liver function. Aim of the present work is to assess the liver enzymes and to find its association with hyperlipidemic profile in T2DM. Total of 157 subjects were studied and divided into two groups; diabetes (n=81) and non-diabetes (n=76). Various biochemical parameters like fasting glucose, post prandial glucose, HbA1c, total cholesterol (TC), triglycerides (Tg), high density lipoprotein cholesterol (HDL-C), alanine amino transferase (ALT), aspartate amino transferase (AST) and gamma-glutamyl transferase (GGT) were analyzed by ROCHE module Cobas 6000 (C501 & C601) analyzer, kits were procured by ROCHE diagnostics. Low density lipoprotein cholesterol (LDL-C) was estimated by Freidwald's formula. Statistical analysis was performed by applying student t test and Pearson's correlation coefficient, at 0.0001 and 0.05 level of significance, respectively. All the glycemic control parameters, lipid profile parameters except HDL-C and liver enzymes were found increased in diabetes group and significantly differ from non-diabetes group (p>0.0001). ALT showed significant positive correlation with fasting glucose, post prandial glucose, HbA1c, TC, Tg, LDL-C and GGT at p>0.05. AST showed very weak relation with all parameters while GGT was positively associated with fasting glucose, post prandial glucose, HbA1c, TC, Tg, LDL-C and ALT at p>0.05. In conclusion, T2DM incline to elevate liver enzymes, especially ALT and GGT were of significance. Routine screening of ALT and GGT in T2DM patients may assists early detection of liver abnormalities and to arrest the progress of disease.

  19. Knee Replacement

    Science.gov (United States)

    Knee replacement is surgery for people with severe knee damage. Knee replacement can relieve pain and allow you to ... Your doctor may recommend it if you have knee pain and medicine and other treatments are not ...

  20. Patient Self-Assessed Passive Range of Motion of the Knee Cannot Replace Health Professional Measurements

    DEFF Research Database (Denmark)

    Borgbjerg, Jens; Madsen, Frank; Odgaard, Anders

    2017-01-01

    The purpose of this study was to investigate whether patients can accurately self-assess their knee passive range of motion (PROM). A picture-based questionnaire for patient self-assessment of knee PROM was developed and posted to patients. The self-assessed PROM from 58 patients was compared...... (≥ 10-degree flexion contracture). Surgeon- and patient-assessed knee PROM showed a mean difference (95% limits of agreement) of -2.1 degrees (-42.5 to 38.3 degrees) for flexion and -8.1 degrees (-28.8 to 12.7 degrees) for extension. The sensitivity of patient self-assessed PROM in identifying knee...

  1. Peripheral nerve blocks versus general anesthesia for total knee replacement in elderly patients on the postoperative quality of recovery

    Directory of Open Access Journals (Sweden)

    Liu JL

    2014-02-01

    Full Text Available JunLe Liu,1,* WeiXiu Yuan,1,* XiaoLin Wang,1,* Colin F Royse,2,3 MaoWei Gong,1 Ying Zhao,1 Hong Zhang1 1Anesthesia and Operation Center, Chinese People's Liberation Army General Hospital and Medical School of Chinese People's Liberation Army, Beijing, People's Republic of China; 2Anesthesia and Pain Management Unit, Department of Surgery, University of Melbourne, Melbourne, Victoria, Australia; 3Department of Anesthesia and Pain Management, The Royal Melbourne Hospital, Melbourne, Victoria, Australia *These authors contributed equally to this work Background: Both peripheral nerve blocks with sedation or general anesthesia can be used for total knee replacement surgery. Objectives: We compared these anesthetic techniques on the postoperative quality of recovery early in elderly patients. Materials and methods: In our study, 213 patients who were ≥65 years old and undergoing total knee replacement were randomized to peripheral nerve blocks (PNBs – lumbar plexus and sciatic – with propofol sedation, or general anesthesia with combined propofol and remifentanil. Blocks were performed using nerve stimulation and 0.35% ropivacaine. All patients received postoperative multimodal analgesia. Postoperative recovery was assessed at 15 minutes, 40 minutes, 1 day, 3 days, and 7 days after surgery, with the Postoperative Quality of Recovery Scale, in physiological, nociceptive, emotive, modified activities of daily living, modified cognitive, and overall patient perspective domains. Results: Intraoperative blood pressure and heart rate were more stable with PNBs (P<0.001. The recovery was better with PNBs in physiological (P<0.001, emotive (depression and anxiety (P<0.001, nociceptive (pain and nausea (P<0.001, modified cognitive (P<0.001, and all domains recovery (P<0.001, but not in activities of daily living (P=0.181. Intraoperative drugs and the postoperative sulfentanil requirement of the PNBs group were lower (all P<0.001. Differences were

  2. 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.

  3. Exercise Training positively modulates the Ectonucleotidase Enzymes in Lymphocytes of Metabolic Syndrome Patients.

    Science.gov (United States)

    Martins, C C; Bagatini, M D; Cardoso, A M; Zanini, D; Abdalla, F H; Baldissarelli, J; Dalenogare, D P; Dos Santos, D L; Schetinger, M R C; Morsch, V M M

    2016-11-01

    In this study, we investigated the cardiovascular risk factors as well as ectonucleotidase activities in lymphocytes of metabolic syndrome (MetS) patients before and after an exercise intervention. 20 MetS patients, who performed regular concurrent exercise training for 30 weeks, 3 times/week, were studied. Anthropometric, biochemical, inflammatory and hepatic parameters and hydrolysis of adenine nucleotides and nucleoside in lymphocytes were collected from patients before and after 15 and 30 weeks of the exercise intervention as well as from participants of the control group. An increase in the hydrolysis of ATP and ADP, and a decrease in adenosine deamination in lymphocytes of MetS patients before the exercise intervention were observed (Pexercise training after 30 weeks of intervention. Additionally, exercise training reduced the inflammatory and hepatic markers to baseline levels after 30 weeks of exercise. Our results clearly indicated alteration in ectonucleotidase enzymes in lymphocytes in the MetS, whereas regular exercise training had a protective effect on the enzymatic alterations and on inflammatory and hepatic parameters, especially if it is performed regularly and for a long period. © Georg Thieme Verlag KG Stuttgart · New York.

  4. Can the epirubicin cardiotoxicity in cancer patients be prevented by angiotensin converting enzyme inhibitors?

    Science.gov (United States)

    Radulescu, D; Buzdugan, E; Ciuleanu, T E; Todor, N; Stoicescu, L

    2013-01-01

    The aim of this study was to assess whether treatment with angiotensin converting enzyme inhibitors (ACEI) can prevent the alteration of left ventricular systolic and diastolic performance in cancer patients treated with different chemotherapy regimens containing epirubicin. In this prospective study , 68 patients with different malignant tumors treated with epirubicin and perindopril in different chemotherapy protocols (study group), and a gender- and age-matched group of 68 patients with different malignant tumors treated with epirubicin without perindopril in different chemotherapy protocols (control group), were assessed by Doppler echocardiography. Left ventricular systolic function was assessed by measuring left ventricular ejection fraction (EF). Left ventricular diastolic function was assessed by Doppler ultrasound by evaluating the transmitral flow. We also assessed the QTc on the 12 lead electrocardiograms. At the end of chemotherapy the left ventricular systolic function was less altered in the study group compared to the control group and was superior in the study group (epirubicin+ACEI) compared to the control group (epirubicin alone). We documented a significantly deteriorated left ventricular diastolic function in both groups at the completion of chemotherapy. QTc time in both arms was also significantly prolonged. In the present echo-Doppler study we documented a preserved left ventricular systolic performance in patients with various malignancies treated with epirubicin plus perindopril. Although co-treatment with ACEI prevented the alteration of systolic performance, it failed to prevent the deterioration of the left ventricular diastolic performance impairment due to poor left ventricular compliance.

  5. The effect of growth hormone (GH) replacement on muscle strength in patients with GH-deficiency: a meta-analysis.

    LENUS (Irish Health Repository)

    Widdowson, W Matthew

    2012-02-01

    CONTEXT\\/OBJECTIVES: GH replacement increases muscle mass and reduces body fat in growth hormone deficiency (GHD) adults. A recent meta-analysis has demonstrated that this improvement in body composition is associated with improved exercise performance. The current meta-analysis was carried out to determine whether high-quality evidence exists to support a beneficial effect of GH replacement on strength. DESIGN\\/METHODS: An extensive Medline search\\/literature review identified eight studies with utilizable, robust data, involving 231 patients in nine cohorts. Previously unpublished data were sought from authors and obtained in two cases. All studies included were randomized, double-blind, placebo-controlled, of parallel or cross-over design and of an average 6.7 months duration. Information was retrieved in uniform format, with data pertaining to patient numbers, study-design, GH-dose, mean age, IGF-I levels and muscle strength measurements (isometric or isokinetic quadriceps strength) recorded. Data were analysed using a fixed-effects model, utilizing continuous data measured on different scales. A summary effect measure (d(s)) was derived for individual strength variables, whereas an overall summary effect was derived from the sum of all studies incorporating different variables; 95% CIs were calculated from the weighted variances of individual study effects. RESULTS: Analysis revealed no significant improvement, neither when all studies were combined (d(s) = +0.01 +\\/- 0.26) nor when measured individually (isometric quadriceps strength, d(s) = +0.02 +\\/- 0.32 and isokinetic quadriceps strength, d(s) = 0.00 +\\/- 0.45). CONCLUSIONS: Evidence from short-term controlled studies fails to support a benefit on muscle strength of GH replacement in GHD patients, which is likely to occur over a longer time-course, as seen in open-label studies.

  6. In situ replacement of infected vascular prosthesis with fresh arterial homograft: Early and long-term results in 18 patients

    Directory of Open Access Journals (Sweden)

    Pejkić Siniša

    2013-01-01

    Full Text Available Introduction. Graft infection is rightly considered one of the severest complications of vascular reconstruction. Treatment is non­standardized and associated with high mortality and morbidity rates. The choice of therapeutic modality depends upon variety of factors. One increasingly used option is in situ replacement of the infected prosthesis with the arterial allograft. Objective. The aim of this prospective nonrandomized study was to evaluate the effectiveness and durability of fresh arterial allograft as in situ substitute for the infected vascular prosthesis. Methods. During period of 2002-2005, 18 patients with the synthetic vascular graft infection underwent partial or complete prosthesis removal and secondary in situ reconstruction using the fresh arterial allograft, preserved under hypothermic conditions in buffered saline solution with an addition of antibiotics. Results. In 14 male and 4 female patients, meanaged 62 years, 8 aortic and 10 peripheral arterial infected prostheses were partially or completely replaced with the allograft. Operative mortality was 27.8% and amputation rate was 22.2%. Systemic sepsis at initial presentation and highly virulent nature of causative microorganisms were identified as significant negative prognostic factors (χ² test, p<0.05. During the long­term follow­up (mean 47 months, allograft aneurysm developed in three patients, requiring allograft explantation, followed in two cases by tertiary prosthetic reconstruction. Conclusion. Substitution of the infected prosthesis with the arterial allograft could be successful if used selectively - for less virulent and localized infections of extracavitary grafts. Close follow­up is mandatory for timely diagnosis of late homograft lesions and its eventual replacement with more durable prosthetic material.

  7. Verapamil and angiotensin-converting enzyme inhibitors in patients with coronary artery disease and reduced left ventricular ejection fraction

    DEFF Research Database (Denmark)

    Hansen, J F; Tingsted, L; Rasmussen, Verner

    1996-01-01

    Verapamil is effective as antianginal medication but contraindicated in patients with congestive heart failure. Angiotensin-converting enzyme (ACE) inhibitors improve survival in patients with congestive heart failure but have limited effect on patients with angina pectoris. No studies have been.......4 to 2.5 +/- 0.6 (p attacks were both significantly reduced after 3 months of treatment. These findings support the hypothesis that the combination of verapamil and trandolapril is useful in patients with attenuated left ventricular function...

  8. The Symmetry of Adverse Local Tissue Reactions in Patients with Bilateral Simultaneous and Sequential ASR Hip Replacement.

    Science.gov (United States)

    Madanat, Rami; Hussey, Daniel K; Donahue, Gabrielle S; Potter, Hollis G; Wallace, Robert; Bragdon, Charles R; Muratoglu, Orhun K; Malchau, Henrik

    2015-10-01

    The purpose of this study was to evaluate whether patients with bilateral metal-on-metal (MoM) hip replacements have symmetric adverse local tissue reactions (ALTRs) at follow-up. An MRI of both hips was performed at a mean time of six years after surgery in 43 patients. The prevalence and severity of ALTRs were found to be similar in simultaneous hips but differences were observed in sequential hips. The order and timing of sequential hip arthroplasties did not affect the severity of ALTRs. Thus, in addition to metal ion exposure from an earlier MoM implant other factors may also play a role in the progression of ALTRs. Bilateral implants should be given special consideration in risk stratification algorithms for management of patients with MoM hip arthroplasty. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Incidence of hip and knee replacement in patients with rheumatoid arthritis following the introduction of biological DMARDs

    DEFF Research Database (Denmark)

    Cordtz, René Lindholm; Hawley, Samuel; Prieto-Alhambra, Daniel

    2018-01-01

    person-years were calculated for patients with RA and GPCs in 6-month periods. Levels and trends in the pre-bDMARD (1996-2001) were compared with the bDMARD era (2003-2016) using segmented linear regression interrupted by a 1-year lag period (2002). RESULTS: We identified 30 404 patients with incident RA......OBJECTIVES: To study the impact of the introduction of biological disease-modifying anti-rheumatic drugs (bDMARDs) and associated rheumatoid arthritis (RA) management guidelines on the incidence of total hip (THR) and knee replacements (TKR) in Denmark. METHODS: Nationwide register-based cohort...... in 1996. In patients with RA, introduction of bDMARDs was associated with a decreasing incidence rate of TKR, whereas the incidence of THR had started to decrease before bDMARD introduction....

  10. The relationship between HbA(1c) and fasting plasma glucose in patients with increased plasma liver enzyme measurements

    DEFF Research Database (Denmark)

    Christiansen, R; Rasmussen, L Melholt; Nybo, H

    2012-01-01

    levels of increased liver enzyme concentrations. Methods:  Data from 10 065 patients with simultaneous measurement of HbA(1c) , venous fasting plasma glucose, alanine aminotransferase and γ-glutamyl transferase were extracted from our laboratory database. Correlations were investigated in four patient...

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

    DEFF Research Database (Denmark)

    Gilliland, Thomas; Dufour, Sylvie; Shulman, Gerald I.

    2016-01-01

    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...... 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...

  12. 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.

  13. Aortic valve replacement with 17-mm St. Jude Medical prostheses for a small aortic root in elderly patients.

    Science.gov (United States)

    Takaseya, Tohru; Kawara, Takemi; Tokunaga, Shigehiko; Kohno, Michitaka; Oishi, Yasuhisa; Morita, Shigeki

    2007-06-01

    There are few reports about the optimal prosthesis for elderly patients who have small aortic roots with an aortic annular size of less than 19 mm. From October 2004 to October 2006, 11 women aged 70 years or older (mean age, 74.9 +/- 3.5 years) underwent aortic valve replacement with a 17-mm Regent prosthesis (St. Jude Medical, St. Paul, MN), with the size determined using the manufacturer's sizer. Clinical status and the results of preoperative and postoperative echocardiography were evaluated. The patients had a mean body surface area of 1.33 +/- 0.13 m2. Preoperative average New York Heart Association (NYHA) functional class was 2.5 +/- 0.7. Preoperative echocardiography showed a mean indexed effective orifice area of 0.33 +/- 0.14 cm2/m2 and a left ventricular mass index of 175 +/- 63 g/m2. Seven patients underwent associated procedures. Postoperative ventilation time was 14 +/- 5.6 hours, and the intensive care unit stay was 1.0 +/- 0.4 days. No patients died perioperatively. The NYHA functional class improved to class I in 9 patients. No obstruction of valve opening was observed. A significant increase in the mean indexed effective orifice area (0.87 +/- 0.10 cm2/m2) and regression of left ventricular mass index were found (114 +/- 46 cm2/m2) on postoperative echocardiography. Aortic valve replacement in elderly patients with small aortic roots (less than 19 mm) using a 17-mm Regent prosthesis showed satisfactory clinical and hemodynamic results.

  14. 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....

  15. Serum and tissue angiotensin-converting enzyme in patients with alopecia areata.

    Science.gov (United States)

    Fahim, Shabnam; Montazer, Fatemeh; Tohidinik, Hamid Reza; Naraghi, Zahra Safaei; Abedini, Robabeh; Nasimi, Maryam; Ghandi, Narges

    2018-03-27

    Alopecia areata is an immune-dependent disorder characterized by the interaction of T-lymphocytes with follicular antigens. Recent studies have shown the existence of a local renin-angiotensin system in the skin, where angiotensin-converting enzyme (ACE) plays a role in autoimmunity and inflammation. The objective of this study was to evaluate serum and tissue ACE activity in patients with alopecia areata. This case-control study was conducted on patients with alopecia areata and healthy controls. Serum and tissue ACE activity were assessed and compared between the two groups. Twenty-five alopecia areata patients (60% male, mean age 32.1 ± 9.9 years) and 24 controls (50% male, mean age 37.4 ± 8.8 years) were included. Mean serum ACE activity was 52.1 ± 9 U/L in cases and 55.3 ± 14.7 U/L in controls (P = 0.37). Tissue ACE activity was significantly lower in cases in all parts of the skin i.e. epidermis (P = 0.016), follicular epithelium (P = 0.004), and endothelium (P = 0.037). Among cases, serum ACE activity was significantly higher in patients with more severe disease (P = 0.030), nonpatchy alopecia areata (alopecia universalis; ophiasis, patchy and ophiasis, diffuse) (P = 0.029), and with nail involvement (P = 0.027). The sample size was too small to draw definite conclusions. Further, most of the patients had only mild or moderate alopecia areata. Unlike in some other inflammatory diseases, the tissue level of ACE seems to be significantly lower in alopecia areata compared to normal controls. Serum ACE was significantly higher in patients with more severe disease.

  16. Effect of renal replacement therapy on viscosity in end-stage renal disease patients.

    Science.gov (United States)

    Feriani, M; Kimmel, P L; Kurantsin-Mills, J; Bosch, J P

    1992-02-01

    Viscosity, an important determinant of microcirculatory hemodynamics, is related to hematocrit (HCT), and may be altered by renal failure or its treatment. To assess these factors, we studied the effect of dialysis on the viscosity of whole blood, plasma, and reconstituted 70% HCT blood of eight continuous ambulatory peritoneal dialysis (CAPD) and nine hemodialysis (HD) patients under steady shear flow conditions at different shear rates, before and after dialysis, compared with nine normal subjects. The density of the red blood cells (RBCs), a marker of cell hydration, was measured in HD patients by a nonaqueous differential floatation technique. Whole blood viscosity was higher in controls than patients, and correlated with HCT before treatment (P less than 0.05) at shear rates of 11.5 to 230 s-1) in HD patients, and 23 to 230 s-1 in all end-stage renal disease (ESRD) patients. In contrast, whole blood viscosity correlated with HCT in CAPD patients only at the lowest shear rates (2.3 and 5.75 s-1, P less than 0.05). Plasma viscosity was higher in CAPD patients than both HD patients before treatment and controls (P less than 0.05, analysis of variance [ANOVA]), despite lower plasma total protein, albumin, and similar fibrinogen concentration compared with HD patients. When all samples were reconstituted to 70% HCT, CAPD patients had higher whole blood viscosity than control subjects'. The high HCT blood viscosity of the ESRD patients was higher than control subjects' at capillary shear rates, suggesting increased RBC aggregation and decreased RBC deformability in patients with renal disease.(ABSTRACT TRUNCATED AT 250 WORDS)

  17. Psychosocial issues and quality of life in patients on renal replacement therapy

    International Nuclear Information System (INIS)

    Panagopoulou, Alkioni; Hardalias, Andreas; Fourtounas, Costas; Berati, Stavroula

    2009-01-01

    The aim of the present study was to investigate psychosocial variables related to objective and subjective indicators of quality of life in a single center cohort study of patients undergoing in-center hemodialysis (Hd), Continuous Ambulatory Peritoneal Dialysis (CAPD) and renal transplant recipients (RTx). We studied 40 HD patients, 36 CAPD, and 48 RTx patients by a special questionnaire examining demographics, functional status, employment status, and impact of therapy on psychosocial issues such as anxiety and depression. The RTx patients disclosed a better functional and employment status than the CAPD and the HD patients. They were also more compliant and satisfied with their therapy and their relationship with the medical and nursing personnel. The CAPD patients were also more satisfied, more compliant, better motivated, and less anxious and depressed compared with the HD patients who scored low in every aspect studied. Successful renal transplantation is a superior modality of therapy than HD or CAPD regarding psychosocial and quality of life issues. However these results can partially be explained by some selection bias, as RTx patients are usually younger and CAPD patients are selected for this modality after examining functional and social status. (author)

  18. 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

  19. Impact of secondary hyperparathyroidism on ventricular mass regression after aortic valve replacement for aortic stenosis in hemodialysis-dependent patients.

    Science.gov (United States)

    Takami, Yoshiyuki; Tajima, Kazuyoshi

    2015-07-01

    In hemodialysis (HD)-dependent patients, secondary hyperparathyroidism induces cardiac hypertrophy. This study investigated whether parathyroid hormone (PTH) levels affect the degree of left ventricular (LV) mass regression in HD patients after aortic valve replacement (AVR) for aortic stenosis (AS). We retrospectively obtained preoperative and 2-year postoperative echocardiography and intact PTH measurements in 88 HD patients who underwent AVR, with bioprostheses (n = 35, 40%) and mechanical valves (n = 53, 60%) of effective orifice area >0.80 cm2/m2, between January 1997 and December 2010. The LV mass decreased significantly from 308 ± 88 to 217 ± 68 g at follow-up of 28 ± 4 months after AVR (p regression at follow-up was inversely related to preoperative PTH values (R = 0.44, p = 0.001). The LV mass regression at follow-up was significantly smaller in the patients (n = 47) with PTH ≥100 pg/mL than in those (n = 41) with PTH regression at 2-year follow-up (β = 0.23, r2 = 0.24, p = 0.02). In conclusion, the HD patients with high levels of PTH presented with less LV mass regression after AVR for AS without patient-prosthesis mismatch. Secondary hyperparathyroidism may impair regression of cardiac hypertrophy after AVR in HD patients with AS.

  20. Auricular Acupressure for Managing Postoperative Pain and Knee Motion in Patients with Total Knee Replacement: A Randomized Sham Control Study

    Directory of Open Access Journals (Sweden)

    Ling-hua Chang

    2012-01-01

    Full Text Available Background. Postoperative pain management remains a significant challenge for all healthcare providers. A randomized controlled trial was conducted to examine the adjuvant effects of auricular acupressure on relieving postoperative pain and improving the passive range of motion in patients with total knee replacement (TKR. Method. Sixty-two patients who had undergone a TKR were randomly assigned to the acupressure group and the sham control group. The intervention was delivered three times a day for 3 days. A visual analog scale (VAS and the Short-Form McGill Pain Questionnaire were used to assess pain intensity. Pain medication consumption was recorded, and the knee motion was measured using a goniometer. Results. The patients experienced a moderately severe level of pain postoperatively (VAS 58.66 ± 20.35 while being on the routine PCA. No differences were found in pain scores between the groups at all points. However, analgesic drug usage in the acupressure group patients was significantly lower than in the sham control group (<0.05, controlling for BMI, age, and pain score. On the 3rd day after surgery, the passive knee motion in the acupressure group patients was significantly better than in the sham control group patients (<0.05, controlling for BMI. Conclusion. The application of auricular acupressure at specific therapeutic points significantly reduces the opioid analgesia requirement and improves the knee motion in patients with TKR.

  1. The flaws in the detail of an observational study on transcatheter aortic valve implantation versus surgical aortic valve replacement in intermediate-risks patients

    NARCIS (Netherlands)

    Barili, Fabio; Freemantle, Nick; Folliguet, Thierry; Muneretto, Claudio; de Bonis, Michele; Czerny, Martin; Obadia, Jean Francois; Al-Attar, Nawwar; Bonaros, Nikolaos; Kluin, Jolanda; Lorusso, Roberto; Punjabi, Prakash; Sadaba, Rafael; Suwalski, Piotr; Benedetto, Umberto; Böning, Andreas; Falk, Volkmar; Sousa-Uva, Miguel; Kappetein, Pieter A.; Menicanti, Lorenzo

    2017-01-01

    The PARTNER group recently published a comparison between the latest generation SAPIEN 3 transcatheter aortic valve implantation (TAVI) system (Edwards Lifesciences, Irvine, CA, USA) and surgical aortic valve replacement (SAVR) in intermediate-risk patients, apparently demonstrating superiority of

  2. Patient experiences of recovery after heart valve replacement: suffering weakness, struggling to resume normality

    DEFF Research Database (Denmark)

    Kikkenborg Berg, Selina; Zwisler, Ann-Dorthe; Pedersen, Birthe D.

    2013-01-01

    Heart valve disease is becoming a public health problem due to increasing life expectancy and new treatment methods. Patients are at risk of developing depression, anxiety or post-traumatic stress disorder after heart valve surgery. To better plan proper care, describing and understanding patients...

  3. High prevalence of thyroid FDG uptake on PET study in patients with thyroid hormone replacement

    International Nuclear Information System (INIS)

    Yu, J.-Q.; Kumar, R.; Xiu, Y.; Dadparvar, S.; Kung, J.W.; Kunjunmen, B.D.; Feng, Q.; Alavi, A.; Zhuang, H.

    2004-01-01

    Thyroid uptake is commonly seen on whole-body FDG-PET images. One well-known cause for this uptake is hyperthyroidism. The purpose of this study was to determine whether hypothyroidism also affects FDG uptake by the thyroid gland. Hospital records of 2765 patients who had undergone whole-body FDG-PET imaging for malignancies were retrospectively reviewed. Among them, those who had thyroid cancer, history of thyroid ablation for hyperthyroidism, neck lymphoma, and other types of head and neck cancer or recent neck surgery were excluded from analysis. The prevalence and level of thyroid FDG uptake in the remaining 1939 patients was compared with the state of patients' thyroid function. There were 141 hypothyroid subjects and the rest (n=1798) were euthyroid. The prevalence of thyroid tissue uptake in euthyroid subjects was 2.34% (42 of 1798), while it was 22% (31 out of 141) in hypothyroid patients. This was statistically significant. Similarly, the prevalence of hypothyroidism in patients in patients showing thyroid uptake was 42.5% (31 of 73), while it was 5.9% (110 of 1866) in patients without thyroid uptake. This was again statistically significant (p<0.001). Based on this retrospective analysis it was concluded that the prevalence of increased thyroid FDG uptake is significantly higher in patients with hypothyroidism than those who are euthyroid. (author)

  4. Can duplex Doppler ultrasound replace computerized tomography in staging patients with renal cell carcinoma?

    NARCIS (Netherlands)

    Bos, SD; Mensink, HJA

    The purpose of this study was to evaluate the accuracy and reliability of duplex Doppler ultrasound (US) and computerized tomography (CT) in staging patients with renal cell carcinoma (RCC). Sixty-six patients were evaluated pre-operatively with duplex Doppler ultrasound and CT. The results were

  5. Clinical evolution of chronic renal patients with HIV infection in replacement therapy

    Directory of Open Access Journals (Sweden)

    Ramón Saracho

    2015-09-01

    Despite the use of HAART, the incidence of HIV+ patients on dialysis has increased; their mortality still exceeds non-HIV patients, and they have a very low rate of transplantation. It is necessary to further our knowledge of this disease in order to improve results.

  6. Predictors of low self-rated health in patients aged 65+ after total hip replacement (THA)

    DEFF Research Database (Denmark)

    Hørdam, Britta; Hemmingsen, Lars

    2013-01-01

    predicting low self-rated health after surgery. Material and method: A cross-sectional study including 287 patients aged 65+, who had had THR within 12-months were performed. Patients from five Danish counties received a mailed questionnaire assessing health status and demographic data. Short Form-36...

  7. 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.

  8. 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

  9. 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...

  10. Transcatheter aortic valve replacement in patients with severe mitral or tricuspid regurgitation at extreme risk for surgery.

    Science.gov (United States)

    Little, Stephen H; Popma, Jeffrey J; Kleiman, Neal S; Deeb, G Michael; Gleason, Thomas G; Yakubov, Steven J; Checuti, Stan; O'Hair, Daniel; Bajwa, Tanvir; Mumtaz, Mubashir; Maini, Brijeshwar; Hartman, Alan; Katz, Stanley; Robinson, Newell; Petrossian, George; Heiser, John; Merhi, William; Moore, B Jane; Li, Shuzhen; Adams, David H; Reardon, Michael J

    2018-05-01

    Patients with symptomatic severe aortic stenosis and severe mitral regurgitation or severe tricuspid regurgitation were excluded from the major transcatheter aortic valve replacement trials. We studied these 2 subgroups in patients at extreme risk for surgery in the prospective, nonrandomized, single-arm CoreValve US Expanded Use Study. The primary end point was all-cause mortality or major stroke at 1 year. A favorable medical benefit was defined as a Kansas City Cardiomyopathy Questionnaire overall summary score greater than 45 at 6 months and greater than 60 at 1 year and with a less than 10-point decrease from baseline. There were 53 patients in each group. Baseline characteristics for the severe mitral regurgitation and severe tricuspid regurgitation cohorts were age 84.2 ± 6.4 years and 84.9 ± 6.5 years; male, 29 (54.7%) and 22 (41.5%), and mean Society of Thoracic Surgeons score 9.9% ± 5.0% and 9.2% ± 4.0%, respectively. Improvement in valve regurgitation from baseline to 1 year occurred in 72.7% of the patients with severe mitral regurgitation and in 61.8% of patients with severe tricuspid regurgitation. A favorable medical benefit occurred in 31 of 47 patients (66.0%) with severe mitral regurgitation and 33 of 47 patients (70.2%) with severe tricuspid regurgitation at 6 months, and in 25 of 44 patients (56.8%) with severe mitral regurgitation and 24 of 45 patients (53.3%) with severe tricuspid regurgitation at 1 year. All-cause mortality or major stroke for the severe mitral regurgitation and severe tricuspid regurgitation cohorts were 11.3% and 3.8% at 30 days and 21.0% and 19.2% at 1 year, respectively. There were no major strokes in either group at 1 year. Transcatheter aortic valve replacement in patients with severe mitral regurgitation or severe tricuspid regurgitation is reasonable and safe and leads to improvement in atrioventricular valve regurgitation. Copyright © 2018 The American Association for Thoracic Surgery

  11. 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

  12. Lean Six Sigma: a new approach to the management of patients undergoing prosthetic hip replacement surgery.

    Science.gov (United States)

    Improta, Giovanni; Balato, Giovanni; Romano, Maria; Carpentieri, Francesco; Bifulco, Paolo; Alessandro Russo, Mario; Rosa, Donato; Triassi, Maria; Cesarelli, Mario

    2015-08-01

    In 2012, health care spending in Italy reached €114.5 billion, accounting for 7.2% of the Gross Domestic Product (GDP) and 14.2% of total public spending. Therefore, reducing waste in health facilities could generate substantial cost savings. The objective of this study is to show that Lean Six Sigma represents an appropriate methodology for the development of a clinical pathway which allows to improve quality and to reduce costs in prosthetic hip replacement surgery. The methodology used for the development of a new clinical pathway was Lean Six Sigma. Problem solving in Lean Six Sigma is the DMAIC (Define, Measure, Analyse, Improve, Control) roadmap, characterized by five operational phases which make possible to reach fixed goals through a rigorous process of defining, measuring, analysing, improving and controlling business problems. The following project indicated several variables influencing the inappropriate prolongation of the length of stay for inpatient treatment and corrective actions were performed to improve the effectiveness and efficiency of the process of care. The average length of stay was reduced from 18.9 to 10.6 days (-44%). This article shows there is no trade-off between quality and costs: Lean Six Sigma improves quality and, at the same time, reduces costs. © 2015 John Wiley & Sons, Ltd.

  13. Patient participation in postoperative care activities in patients undergoing total knee replacement surgery: Multimedia Intervention for Managing patient Experience (MIME). Study protocol for a cluster randomised crossover trial.

    Science.gov (United States)

    McDonall, Jo; de Steiger, Richard; Reynolds, John; Redley, Bernice; Livingston, Patricia; Botti, Mari

    2016-07-18

    Patient participation is an important indicator of quality care. Currently, there is little evidence to support the belief that participation in care is possible for patients during the acute postoperative period. Previous work indicates that there is very little opportunity for patients to participate in care in the acute context. Patients require both capability, in terms of having the required knowledge and understanding of how they can be involved in their care, and the opportunity, facilitated by clinicians, to engage in their acute postoperative care. This cluster randomised crossover trial aims to test whether a multimedia intervention improves patient participation in the acute postoperative context, as determined by pain intensity and recovery outcomes. A total of 240 patients admitted for primary total knee replacement surgery will be invited to participate in a cluster randomised, crossover trial and concurrent process evaluation in at least two wards at a major non-profit private hospital in Melbourne, Australia. Patients admitted to the intervention ward will receive the multimedia intervention daily from Day 1 to Day 5 (or day of discharge, if prior). The intervention will be delivered by nurses via an iPad™, comprising information on the goals of care for each day following surgery. Patients admitted to the control ward will receive usual care as determined by care pathways currently in use across the organization. The primary endpoint is the "worst pain experienced in the past 24 h" on Day 3 following TKR surgery. Pain intensity will be measured using the numerical rating scale. Secondary outcomes are interference of pain on activities of daily living, length of stay in hospital, function and pain following TKR surgery, overall satisfaction with hospitalisation, postoperative complications and hospital readmission. The results of this study will contribute to our understanding of the effectiveness of interventions that provide knowledge and

  14. Sutureless versus Conventional Aortic Valve Replacement: Outcomes in 70 High-Risk Patients Undergoing Concomitant Cardiac Procedures.

    Science.gov (United States)

    Hanedan, Muhammet Onur; Yuruk, Mehmet Ali; Parlar, Ali Ihsan; Ziyrek, Ugur; Arslan, Ali Kemal; Sayar, Ufuk; Mataraci, Ilker

    2018-02-01

    In elderly, high-risk surgical patients, sutureless aortic valve replacement (AVR) can often be an alternative to conventional AVR; shorter aortic cross-clamp and cardiopulmonary bypass times are the chief advantages. We compared the outcomes of sutureless AVR with those of conventional AVR in 70 elderly patients who underwent concomitant cardiac surgical procedures. We retrospectively analyzed the cases of 42 men and 28 women (mean age, 70.4 ± 10.3 yr; range, 34-93 yr) who underwent cardiac operations plus AVR with either a sutureless valve (group 1, n=38) or a conventional bioprosthetic or mechanical valve (group 2, n=32). Baseline patient characteristics were similar except for worse New York Heart Association functional status and the prevalence of diabetes mellitus in group 1. In group 1, the operative, cross-clamp, and cardiopulmonary bypass times were shorter (all P =0.001), postoperative drainage amounts were lower ( P =0.009), hospital stays were shorter ( P =0.004), and less red blood cell transfusion was needed ( P =0.037). Echocardiograms before patients' discharge from the hospital showed lower peak and mean aortic gradients in group 1 (mean transvalvular gradient, 8.4 ± 2.8 vs 12.2 ± 5.2 mmHg; P =0.012). We found that elderly, high-risk patients who underwent multiple cardiac surgical procedures and sutureless AVR had better hemodynamic outcomes and shorter ischemic times than did patients who underwent conventional AVR.

  15. Analysis of Outcomes of the Nutritional Status in Patients Qualified for Aortic Valve Replacement in Comparison to Healthy Elderly

    Directory of Open Access Journals (Sweden)

    Edyta Wernio

    2018-03-01

    Full Text Available Severe aortic stenosis (AS is associated with the reduction of muscle mass and may be associated with deterioration of nutritional status. Furthermore, malnourished cardiac patients are characterized by a higher risk of postoperative complications and mortality. The aim of this study was the evaluation and comparison of nutritional status, appetite and body composition in older people with severe aortic stenosis before aortic valve replacement and healthy elderly volunteers. One hundred and one patients, aged >65 years old with severe AS were included in the study. Nutritional status was assessed. Body composition was estimated using bioelectrical impedance analysis. Concentrations of albumin, prealbumin, triglycerides, total cholesterol and C-reactive protein were measured, and a complete blood count was done. About 40% of AS patients were at risk of malnutrition. They had decreased hand grip strength and they lost more body mass than the control group. Malnourished AS patients were older, had lower body mass indexes (BMIs and lower aortic valve areas in comparison to well-nourished patients. Older AS patients, like their peers, show excessive body mass and, at the same time, the features of malnutrition. They have additional factors such as unintentional weight lost and decreased muscle strength which may be associated with worse outcomes.

  16. 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.

  17. Anesthetic management for combined mitral valve replacement and aortic valve repair in a patient with osteogenesis imperfecta

    Directory of Open Access Journals (Sweden)

    Huang Jiapeng

    2011-01-01

    Full Text Available Osteogenesis imperfecta is a rare disorder of connective tissues and presents multiple challenges, including difficult airway, hyperthermia, coagulopathy and respiratory dysfunction, for anesthesiologists, especially during cardiac surgery. We present anesthetic management of a patient with osteogenesis impertecta during double valve surgery. Dexmedetomidine infusion minimized the risks of malignant hyperthermia. Glidescope and in-line stabilization facilitated endotracheal intubation and protected his oral structures and cervical spine. Transesophageal echocardiography (TEE diagnosed a flail A3 segment and redundant left coronary cusp causing mitral and aortic regurgitation. The mitral valve was replaced and the aortic valve repaired. Coagulopathy was corrected according to comprehensive coagulation analysis. Glidescope, dexmedetomidine, coagulation analysis and TEE could facilitate anesthetic management in these patients.

  18. The efficacy of 12 weeks non-surgical treatment for patients not eligible for total knee replacement

    DEFF Research Database (Denmark)

    Skou, Søren Thorgaard; Rasmussen, Sten; Laursen, Mogens Berg

    2015-01-01

    OBJECTIVE: To compare the efficacy of a 12-week non-surgical treatment program with usual care in patients with knee osteoarthritis (OA) not eligible for total knee replacement (TKR). METHOD: This two-arm parallel group assessor-blinded randomized controlled trial (RCT) included 100 adults from...... secondary care with knee OA, confirmed by radiography (Kellgren-Lawrence grade ≥1), but not eligible for a TKR. The 12-week non-surgical treatment program consisted of individualized progressed neuromuscular exercise, patient education, insoles, dietary advice and prescription of pain medication...... if indicated, while usual care comprised two leaflets with information and advice on knee OA and recommended treatments. The primary outcome was the change from baseline to 12 months in the Knee injury and Osteoarthritis Outcome Score (KOOS)4 defined as the average score for the KOOS subscales of pain...

  19. Use of locking plates for fixation of the greater trochanter in patients with hip replacement

    Directory of Open Access Journals (Sweden)

    Allison K. Tetreault, BA

    2016-12-01

    Conclusions: Locking plate technology is a successful method of fixation of the greater trochanter in patients with THA. Postoperative trochanteric pain and reoperation for hardware-related issues remain a challenge.

  20. Patient-reported outcome and risk of revision after shoulder replacement for osteoarthritis

    DEFF Research Database (Denmark)

    Rasmussen, Jeppe V; Polk, Anne; Brorson, Stig

    2014-01-01

    PURPOSE: We used patient-reported outcome and risk of revision to compare hemiarthroplasty (HA) with total shoulder arthroplasty (TSA) and stemmed hemiarthroplasty (SHA) with resurfacing hemiarthroplasty (RHA) in patients with glenohumeral osteoarthritis. PATIENTS AND METHODS: We included all...... of presentation, the raw scores were converted to a percentage of the maximum score. Revision rates were calculated by checking reported revisions to the DSR until December 2011. WOOS and risk of revision were adjusted for age, sex, previous surgery, and type of osteoarthritis. RESULTS: There were 113 TSAs...... and 1096 HAs (837 RHAs and 259 SHAs). Patients treated with TSA generally had a better WOOS, exceeding the predefined minimal clinically important difference, at 1 year (mean difference 10, p

  1. Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome.

    Science.gov (United States)

    Itagaki, Shinobu; Chikwe, Joanna P; Chiang, Yuting P; Egorova, Natalia N; Adams, David H

    2015-06-09

    Bicuspid aortic valves are associated with valve dysfunction, ascending aortic aneurysm and dissection. Management of the ascending aorta at the time of aortic valve replacement (AVR) in these patients is controversial and has been extrapolated from experience with Marfan syndrome, despite the absence of comparative long-term outcome data. This study sought to assess whether the natural history of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease is substantially different from that seen in patients with Marfan syndrome. In this retrospective comparison, outcomes of 13,205 adults (2,079 with bicuspid aortic valves, 73 with Marfan syndrome, and 11,053 control patients with acquired aortic valve disease) who underwent primary AVR without replacement of the ascending aorta in New York State between 1995 and 2010 were compared. The median follow-up time was 6.6 years. The long-term incidence of thoracic aortic dissection was significantly higher in patients with Marfan syndrome (5.5 ± 2.7%) compared with those with bicuspid valves (0.55 ± 0.21%) and control group patients (0.41 ± 0.08%, p Marfan syndrome (10.8 ± 4.4%) compared with those with bicuspid valves (4.8 ± 0.8%) and control group patients (1.4 ± 0.2%) (p Marfan syndrome were significantly more likely to undergo thoracic aortic surgery in late follow-up (10.4 ± 4.3%) compared with those with bicuspid valves (2.5 ± 0.6%) and control group patients (0.50 ± 0.09%) (p Marfan syndrome compared with those with bicuspid aortic valves confirm that operative management of patients with bicuspid aortic valves should not be extrapolated from Marfan syndrome and support discrete treatment algorithms for these different clinical entities. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  2. Mid-term shoulder functional and quality of life outcomes after shoulder replacement in obese patients.

    Science.gov (United States)

    Vincent, Heather K; Struk, Aimee M; Reed, Austin; Wright, Thomas W

    2016-01-01

    Shoulder pain and loss of function are directly associated with obesity. We hypothesized that significant interactions would exist between total shoulder arthroplasty (TSA) and reverse total shoulder arthroplasty (RSA) and obesity status on functional and quality of life (QOL) outcomes over the long term. Clinical and QOL outcomes (American Shoulder and Elbow Surgeons Evaluation form, Shoulder Pain and Disability Index, University of California at Los Angeles Shoulder Rating scale, Medical Outcomes Short Form 12 (SF-12), range of motion (ROM), and strength) were longitudinally compared in patients with low and high body mass index (BMI) after a TSA or a RSA. Prospectively collected data of patients with a TSA or RSA were reviewed (N = 310). Preoperative, 2-year, and final follow-up visits were included (range 3-17 years; mean 5.0 ± 2.5 years). Patient data were stratified for analysis using BMI. Morbidly obese patients had worse preoperative functional scores and QOL compared to the other groups. There were no significant interactions of BMI group by surgery type for any of the outcome variables except for active external rotation ROM. Morbidly obese patients attained lower SF-12 scores compared to the remaining groups at each time point. Both TSA and RSA can be expected to impart positive functional outcomes in patients irrespective of BMI. Morbidly obese patients do not attain the same gains in Medical Outcomes SF-12 scores as the non-morbidly obese patients. The lower improvements in active external ROM may be due to morphological limitations of excessive adiposity. This is a level II study.

  3. Evaluation of the efficacy and safety of three dosing regimens of agalsidase alfa enzyme replacement therapy in adults with Fabry disease

    Directory of Open Access Journals (Sweden)

    Goláň L

    2015-07-01

    Full Text Available Lubor Goláň,1 Ozlem Goker-Alpan,2 Myrl Holida,3 Ikka Kantola,4 Mariusz Klopotowski,5 Johanna Kuusisto,6 Aleš Linhart,1 Jacek Musial,7 Kathleen Nicholls,8 Derlis Gonzalez-Rodriguez,9 Reena Sharma,10 Bojan Vujkovac,11 Peter Chang,12 Anna Wijatyk12 1First Faculty of Medicine, Department of Cardiovascular Medicine, Charles University, Prague, Czech Republic; 2Lysosomal Research and Treatment Unit, Fairfax, VA, USA; 3Stead Family Department of Pediatrics, Division of Medical Genetics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA; 4Division of Medicine, Turku University Hospital, Turku, Finland; 5Institute of Cardiology, Warsaw, Poland; 6Department of Medicine, Center for Medicine and Clinical Research, University of Eastern Finland and Kuopio University Hospital, Kuopio, Finland; 7Department of Internal Medicine, Jagiellonian University Medical College, Krakow, Poland; 8Department of Nephrology, Royal Melbourne Hospital and the University of Melbourne, VIC, Australia; 9Instituto Privado de Hematologia E Investigacion Clinica (IPHIC, Asuncion, Paraguay; 10Salford Royal NHS Foundation Trust, Salford, UK; 11General Hospital Slovenj Gradec, Slovenj Gradec, Slovenia; 12Shire, Lexington, MA, USA Purpose: Efficacy and safety of agalsidase alfa at 0.2 mg/kg weekly were compared with 0.2 mg/kg every other week (EOW. Exploratory analyses were performed for 0.4 mg/kg weekly.Patients and methods: This was a 53-week, Phase III/IV, multicenter, open-label study (NCT01124643 in treatment-naïve adults (≥18 years with Fabry disease. Inclusion criteria were left ventricular hypertrophy at baseline, defined as left ventricular mass indexed to height >50 g/m2.7 for males and >47 g/m2.7 for females. Primary endpoint was reduction of left ventricular mass indexed to height as assessed by echocardiography. Secondary endpoints included cardiac (peak oxygen consumption, 6-minute walk test, Minnesota Living with Heart Failure Questionnaire, New

  4. Personality, function and satisfaction in patients undergoing total hip or knee replacement.

    Science.gov (United States)

    Ramaesh, Rishikesan; Jenkins, Paul; Lane, Judith V; Knight, Sara; Macdonald, Deborah; Howie, Colin

    2014-03-01

    The aim of this study was to investigate the relationships between personality and joint-specific function, general physical and general mental health in patients undergoing total hip (THA) and knee arthroplasty (TKA). One hundred and eighty-four patients undergoing THA and 205 undergoing TKA were assessed using the Eysneck Personality Questionnaire, brief version (EPQ-BV). General physical and mental health was measured using the Short-Form 12 (SF-12) questionnaire and the EuroQol (EQ-5D). Joint-specific function was measured using the Oxford hip or knee score. The "unstable introvert" personality type was associated with poorer pre-operative function and health in patients with hip arthrosis. In patients with knee arthrosis, there was poorer general health in those with "stable extrovert" and "unstable introvert" types. Personality was not an independent predictor of outcome following TKA or THA. The main predictor was pre-operative function and health. Comorbidity was an important covariate of both pre-operative and postoperative function. Personality may play a role in the interaction of these disease processes with function and health perception. It may also affect the response and interpretation of psychometric and patient-reported outcome measures. It may be important to characterise and identify these traits in potential arthroplasty patients as it may help deliver targeted education and management to improve outcomes in certain groups.

  5. Familial LCAT deficiency: from renal replacement to enzyme replacement

    NARCIS (Netherlands)

    Stoekenbroek, R. M.; van den Bergh Weerman, M. A.; Hovingh, G. K.; Potter van Loon, B. J.; Siegert, C. E. H.; Holleboom, A. G.

    2013-01-01

    Familial LCAT deficiency (FLD) is a recessive lipid disorder ultimately leading to end-stage renal disease (ESRD). We present two brothers with considerable variation in the age at which they developed ESRD. Kidney biopsies revealed both tubular and glomerular pathology. To date, no causal therapy

  6. Relative Expression of PBMC MicroRNA-133a Analysis in Patients Receiving Warfarin After Mechanical Heart Valve Replacement

    Science.gov (United States)

    Kabiri Rad, Hamid; Mazaheri, Mahta; Dehghani Firozabadi, Ali

    Background: MicroRNAs (miRNAs) are implicated in various biological processes including anticoagulation. However, the modulation of miRNA by pharmacological intervention such as warfarin treatment in patients receiving warfarin has not been disclosed yet. The aim of this study work was to assess the effect of warfarin drug on expression level of mir-133a-3p in patients with mechanical heart valve replacement. Methods: In this research, the expression level of miRNA-133a-3p was analyzed in Peripheral Blood Mononuclear Cells (PBMCs) from mechanical valve replacement patients who had received warfarin for at least 3 months continuously. Quantitative RT-PCR method was used for this assay. Results: Our findings indicated a significant diffrence between the rate of miR-133a-3p expression in individuals receiving warfarin and the control group (p<0.01). There was also a statistically significant difference in miR-133a-3p expression in patients with different ages (p<0.05) suggesting that the rate of miR-133a-3p expression in persons receiving warfarin is related to age. However, other variables like warfarin dose, International Normalized Ratio (INR), gender, and Body Mass Index (BMI) were not significantly effective on the miR-133a-3p experssion rate in individuals receving warfarin. Conclusion: Based on our results, it can be concluded that miR-133a-3p is involved in the coagulation pathway. The recent result indicates that warfarin affects the expression of miR-133a. This expression may be potentially important for treatment by anticoagulants. Awareness of the time course of miRNA expression profile can improve efficiency of response to warfarin. PMID:29296264

  7. [The hardware techniques for the restoration of the gait stereotype in the patients following total hip replacement: the personalized approach].

    Science.gov (United States)

    Koneva, E S; Lyadov, K V; Shapovalenko, T V; Zhukova, E V; Polushkin, V G

    2018-04-09

    total hip replacement has long ago become the «golden standard» for the treatment of dysplastic coxarthrosis in thousands of the patients receiving it every year. In the meantime, the analysis of the specialized literature gives evidence of the lack of a systematic and personified approach to the rehabilitation treatment. The objective of the present study was to improve medical rehabilitation of the patients following the total hip replacement and to develop the personalized programs for walking modality reconstruction taking into consideration the age and the body weight of the patients. A total of 240 patients were available for the observation including 184 women and 56 men. They were divided into three study groups and one control group, with the differentiation into the following three subgroups: one comprised of the patients of moderate acerage age and body weight, the other containing the obese patients (BMI>35), and the third one involving the elderly patients (age >70 years); each subgroup consisted of 20 patients. All the patients received the early basic rehabilitation treatment, those in the study groups had to perform in addition the robotic training based on the use of hardware techniques supplemented by passive mechanotherapy and electromyostimulation designed to restore the walking stereotype with three types of devices: body weight unloading, video-reconstruction associated with biological feedback and robototherapy. The comparative analysis of the effectiveness of various methods of gait reconstruction has demonstrated the high effectiveness of the application of the hardware technique in the patients of moderate acerage age and body weight. At the same time, the elderly patients had a significantly higher rate of successful walking reconstruction efficiency under the influence of the video-associated training with biological feedback (3 times that achieved with training using the device for unloading the body weight and 4 times compared with

  8. 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

  9. Total knee replacement with tibial tubercle osteotomy in rheumatoid patients with stiff knee.

    Science.gov (United States)

    Eid, Ahmed Salem; Nassar, Wael Ahmed Mohamed; Fayyad, Tamer Abdelmeguid Mohamed

    2016-11-01

    Total knee arthroplasty (TKA) is a well-proven modality that can provide pain relief and restore mobility for rheumatoid arthritis (RA) patients with advanced joint destruction. Patellar ligament avulsion, especially in presence of poor bone quality and knee stiffness, is one of the special considerations that must be addressed in this unique population of patients. This study aimed to determine the functional results in a series of rheumatoid patients with stiff knee and end-stage joint destruction who underwent tibial tubercle osteotomy during TKA. Twenty-three knees in 20 patients (16 women; four men) at a mean age of 54 years with end-stage arthritis and knee stiffness due to RA were operated upon for TKA using tibial tubercle osteotomy as a step during the operation. Patients were reviewed clinically and radiographically with a minimum follow-up of two years. Complications were noted. Hospital for Special Surgery (HSS) score was recorded pre-operatively and at six and 12 months postoperatively. Union occurred at the osteotomy site in 21 of 23 cases. One case had deep venous thrombosis (DVT). There was no infection or periprosthetic fracture, and at last follow-up, no patient required revision. HSS score improved from 46 (15-60) pre-operatively to 85 (71-96) post-operatively. Tibial tubercle osteotomy during TKA in patients with RA and stiff knee is technically demanding yet proved to be effective in improving post-operative range of movement and minimising the complication of patellar ligament avulsion.

  10. 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)

  11. Ochronosis of the knee with secondary osteoarthritis requiring total knee replacement in a patient with cryptogenic organising pneumonia.

    Science.gov (United States)

    Jasper, Jorrit; Metsaars, Wieneke; Jansen, Joris

    2016-05-20

    Ochronosis is a rare autosomal recessive metabolic disease caused by homogentisic acid oxidase enzyme deficiency. High homogentisic acid levels will eventually result in black deposits in skin, sclerae, connective tissues and urine (alkaptonuria). It can lead to early degeneration of connective tissues and cartilage. Ochronosis can damage normal cartilage, leading to secondary osteoarthritis. The diagnosis is often delayed because of its low prevalence and non-specific early symptoms. In our patient, the secondary osteoarthritis due to ochronosis deposits in the cartilage was treated by total knee arthroplasty, with good clinical outcome. This article reports the first case of ochronosis with secondary osteoarthritis of the knee in a patient previously diagnosed with cryptogenic organising pneumonia (COP). 2016 BMJ Publishing Group Ltd.

  12. BIOMECHANICAL INDICES OF STANDING AND GAIT IN PATIENTS AFTER TOTAL KNEE REPLACEMENT USING COMPUTER NAVIGATION

    Directory of Open Access Journals (Sweden)

    Y. A. Bezgodkov

    2011-01-01

    Full Text Available Several biomechanical parameters of standing and walking in 50 patients with osteoarthrosis after total knee arthroplasty were evaluated. The patients were randomly divided in two equal groups: in the first group the surgery was performed with computer navigation system and in the second - with traditional instruments. After TKA with computer navigation centers of common body pressure and legs pressure during standing phase improved significantly better than in traditional group. Walking parameters like step length, ground contact time and rhythm coefficient improved in both groups of patients but without significant difference. Thereby more precise orientation of implant that achieved during computer assisted TKA leads to better functional performance at 6 and 12 month after surgery.

  13. Validity of a simple Internet-based outcome-prediction tool in patients with total hip replacement: a pilot study.

    Science.gov (United States)

    Stöckli, Cornel; Theiler, Robert; Sidelnikov, Eduard; Balsiger, Maria; Ferrari, Stephen M; Buchzig, Beatus; Uehlinger, Kurt; Riniker, Christoph; Bischoff-Ferrari, Heike A

    2014-04-01

    We developed a user-friendly Internet-based tool for patients undergoing total hip replacement (THR) due to osteoarthritis to predict their pain and function after surgery. In the first step, the key questions were identified by statistical modelling in a data set of 375 patients undergoing THR. Based on multiple regression, we identified the two most predictive WOMAC questions for pain and the three most predictive WOMAC questions for functional outcome, while controlling for comorbidity, body mass index, age, gender and specific comorbidities relevant to the outcome. In the second step, a pilot study was performed to validate the resulting tool against the full WOMAC questionnaire among 108 patients undergoing THR. The mean difference between observed (WOMAC) and model-predicted value was -1.1 points (95% confidence interval, CI -3.8, 1.5) for pain and -2.5 points (95% CI -5.3, 0.3) for function. The model-predicted value was within 20% of the observed value in 48% of cases for pain and in 57% of cases for function. The tool demonstrated moderate validity, but performed weakly for patients with extreme levels of pain and extreme functional limitations at 3 months post surgery. This may have been partly due to early complications after surgery. However, the outcome-prediction tool may be useful in helping patients to become better informed about the realistic outcome of their THR.

  14. Energy Homeostasis and Body Weight before and after Cessation of Block and Replacement Therapy in Euthyroid Patients with Graves' Disease

    Directory of Open Access Journals (Sweden)

    Lars P. Klieverik

    2011-01-01

    Full Text Available Patients with Graves' hyperthyroidism (GH treated with a combination of thyrostatic drugs and T4, that is, block and replacement therapy (BRT, often report body weight (BW gain. We aimed to determine changes in BW and energy metabolism upon cessation of BRT in these patients, and to identify possible endocrine determinants. We analysed 22 patients with GH (i during BRT, and (ii 12 weeks after BRT cessation. Patients were euthyroid at both visits. There were no differences in BW or resting energy expenditure (REE between visits. At visit 1, after 13.5 (9.5–48.0 months of BRT, serum free (FT4 correlated positively with REE (=0.433, =0.044 and negatively with body fat % (=−0.450, =0.035. Plasma FT3 and FT3/FT4 ratio showed an increase 12 w after cessation of BRT (20%, <0.0001 and 16%, =0.007, resp.. Moreover, the relative change in FT3/FT4 ratio showed a significant, positive correlation with the relative change in REE between the 2 visits (=0.465, =0.029. In conclusion, serum FT4 determines REE in euthyroid patients with GH treated with BRT. Twelve weeks after BRT cessation, BW and energy homeostasis are unaltered. However, as serum FT3/FT4 ratio increases after cessation of BRT, which is a positive determinant of changes in REE, a longer term BW decrease is likely to occur.

  15. Anesthesia for the patient undergoing total knee replacement: current status and future prospects

    Directory of Open Access Journals (Sweden)

    Turnbull ZA

    2017-03-01

    Full Text Available Zachary A Turnbull, Dahniel Sastow, Gregory P Giambrone, Tiffany Tedore Department of Anesthesiology, Weill Cornell Medical College, New York, NY, USA Abstract: Total knee arthroplasty (TKA has become one of the most common orthopedic surgical procedures performed nationally. As the population and surgical techniques for TKAs have evolved over time, so have the anesthesia and analgesia used for these procedures. General anesthesia has been the dominant form of anesthesia utilized for TKA in the past, but regional anesthetic techniques are on the rise. Multiple studies have shown the potential for regional anesthesia to improve patient outcomes, such as a decrease in intraoperative blood loss, length of stay, and patient mortality. Anesthesiologists are also moving toward multimodal analgesia, which includes peripheral nerve blockade, periarticular injection, and preemptive analgesia. The goal of multimodal analgesia is to improve perioperative pain control while minimizing systemic narcotic consumption. With improved postoperative pain management and rapid patient rehabilitation, new clinical pathways have been engineered to fast track patient recovery after orthopedic procedures. The aim of these clinical pathways was to improve quality of care, minimize unnecessary variations in care, and reduce cost by using streamlined procedures and protocols. The future of TKA care will be formalized clinical pathways and tracks to better optimize perioperative algorithms with regard to pain control and perioperative rehabilitation. Keywords: TKA, regional anesthesia, analgesia

  16. Predictive value of NGAL for use of renal replacement therapy in patients with severe sepsis

    DEFF Research Database (Denmark)

    Hjortrup, P B; Haase, N; Treschow, F

    2015-01-01

    intensive care units (ICUs) in adult ICU patients with severe sepsis needing fluid resuscitation and a sub-study of the 6S trial. Plasma and urine were sampled at baseline and NGAL was measured using particle-enhanced turbidimetric immunoassay (The NGAL Test). Outcome measures were use of RRT in ICU...

  17. Mortality from infections and malignancies in patients treated with renal replacement therapy

    DEFF Research Database (Denmark)

    Vogelzang, Judith L; van Stralen, Karlijn J; Noordzij, Marlies

    2015-01-01

    .9 times higher in dialysis patients and 1.7 times higher in transplant recipients than in the general population. Cancer and multisystem disease as primary causes of end-stage renal disease were associated with higher mortality from malignancies. CONCLUSION: Infection-related mortality is highly increased...

  18. Optimal sizing for SAPIEN 3 transcatheter aortic valve replacement in patients with or without left ventricular outflow tract calcification.

    Science.gov (United States)

    Maeno, Yoshio; Abramowitz, Yigal; Jilaihawi, Hasan; Israr, Sharjeel; Yoon, Sunghan; Sharma, Rahul P; Kazuno, Yoshio; Kawamori, Hiroyuki; Miyasaka, Masaki; Rami, Tanya; Mangat, Geeteshwar; Takahashi, Nobuyuki; Okuyama, Kazuaki; Kashif, Mohammad; Chakravarty, Tarun; Nakamura, Mamoo; Cheng, Wen; Makkar, Raj R

    2017-04-07

    The impact of left ventricular outflow tract calcification (LVOT-CA) on SAPIEN 3 transcatheter aortic valve replacement (S3-TAVR) is not well understood. The aims of the present study were to determine optimal device sizing for S3-TAVR in patients with or without LVOT-CA and to evaluate the influence of residual paravalvular leak (PVL) on survival after S3-TAVR in these patients. This study analysed 280 patients (LVOT-CA=144, no LVOT-CA=136) undergoing S3-TAVR. Optimal annular area sizing was defined as % annular area sizing related to lower rates of ≥mild PVL. Annular area sizing was determined as follows: (prosthesis area/CT annulus area-1)×100. Overall, ≥mild PVL was present in 25.7%. Receiver operating characteristic curve analysis for prediction of ≥mild PVL in patients with LVOT-CA showed that 7.2% annular area sizing was identified as the optimal threshold (area under the curve [AUC] 0.71). Conversely, annular area sizing for no LVOT-CA appeared unrelated to PVL (AUC 0.58). Aortic annular injury was seen in four patients (average 15.5% annular area oversizing), three of whom had LVOT-CA. Although there was no difference in one-year survival between patients with ≥mild PVL and without PVL (log-rank p=0.91), subgroup analysis demonstrated that patients with ≥moderate LVOT-CA who had ≥mild PVL had lower survival compared to patients with ≥mild PVL and none or mild LVOT-CA (log-rank p=0.010). In the setting of LVOT-CA, an optimally sized S3 valve is required to reduce PVL and to increase survival following TAVR.

  19. Seasonal variations in TSH serum levels in athyreotic patients under L-thyroxine replacement monotherapy.

    Science.gov (United States)

    Gullo, Damiano; Latina, Adele; Frasca, Francesco; Squatrito, Sebastiano; Belfiore, Antonino; Vigneri, Riccardo

    2017-08-01

    Whether serum TSH undergoes seasonal fluctuations in euthyroid and hypothyroid residents of temperate climates is controversial. Monthly TSH and thyroid hormone levels were cross-sectionally analysed in a large cohort of euthyroid subjects (n=11 806) and L-thyroxine (L-T4)-treated athyreotic patients (n=3 934). Moreover, in a small group (n=119) of athyreotic patients treated with an unchanged dosage of L-T4 monotherapy, hormones were measured both in the coldest and in the hottest seasons of the same year (longitudinal study). No seasonal hormone change was observed in the euthyroid subjects except for a small FT3 increase in winter (+2.9%, P<.001). In contrast, the L-T4-treated athyreotic patients had significantly higher serum TSH values in the cold season when the FT4 values were significantly lower. The differences were more notable in the longitudinal series (TSH, 0.80 vs. 0.20 mU/L and FT4, 16.3 vs. 17.8 pmol/L in December-March vs. June-September, respectively). In these patients also serum FT3 values significantly decreased in winter (in the longitudinal series, 3.80 in winter vs 4.07 pmol/L in summer). Regression analysis showed that in athyreotic subjects, a greater FT4 change is required to obtain a TSH change similar to that of euthyroid controls and that this effect is more pronounced in the summer. Athyreotic patients undergoing L-T4 monotherapy have abnormal seasonal variations in TSH. These changes are secondary to the FT4 and FT3 serum decreases in winter, which occur in spite of the constant treatment. The underlying mechanisms are unclear, but in some cases, these changes may be clinically relevant. © 2017 John Wiley & Sons Ltd.

  20. Aortic valve replacement and prosthesis-patient mismatch in the era of trans-catheter aortic valve implantation.

    Science.gov (United States)

    Morita, Shigeki

    2016-08-01

    The treatment strategy for aortic stenosis (AS) has been changing due to newly deve