Estrogen Replacement Therapy in Ovariectomized Nonpregnant Ewes Stimulates Uterine Artery Hydrogen Sulfide Biosynthesis by Selectively Up-Regulating Cystathionine β-Synthase Expression

PubMed Central

Lechuga, Thomas J.; Zhang, Hong-hai; Sheibani, Lili; Karim, Muntarin; Jia, Jason; Magness, Ronald R.; Rosenfeld, Charles R.

2015-01-01

Estrogens dramatically dilate numerous vascular beds with the greatest response in the uterus. Endogenous hydrogen sulfide (H2S) is a potent vasodilator and proangiogenic second messenger, which is synthesized from L-cysteine by cystathionine β-synthase (CBS) and cystathionine γ-lyase (CSE). We hypothesized that estrogen replacement therapy (ERT) selectively stimulates H2S biosynthesis in uterine artery (UA) and other systemic arteries. Intact and endothelium-denuded UA, mesenteric artery (MA), and carotid artery (CA) were obtained from ovariectomized nonpregnant ewes (n = 5/group) receiving vehicle or estradiol-17β replacement therapy (ERT). Total RNA and protein were extracted for measuring CBS and CSE, and H2S production was determined by the methylene blue assay. Paraffin-embedded UA rings were used to localize CBS and CSE proteins by immunofluorescence microscopy. ERT significantly stimulated CBS mRNA and protein without altering CSE mRNA or protein in intact and denuded UA. Quantitative immunofluorescence microscopic analyses showed CBS and CSE protein localization in endothelium and smooth muscle and confirmed that ERT stimulated CBS but not CSE protein expression in UA endothelium and smooth muscle. ERT also stimulated CBS, but not CSE, mRNA and protein expression in intact and denuded MA but not CA in ovariectomized ewes. Concomitantly, ERT stimulated UA and MA but not CA H2S production. ERT-stimulated UA H2S production was completely blocked by a specific CBS but not CSE inhibitor. Thus, ERT selectively stimulates UA and MA but not CA H2S biosynthesis by specifically up-regulating CBS expression, implicating a role of H2S in estrogen-induced vasodilation and postmenopausal women's health. PMID:25825818

Estrogen Replacement Therapy in Ovariectomized Nonpregnant Ewes Stimulates Uterine Artery Hydrogen Sulfide Biosynthesis by Selectively Up-Regulating Cystathionine β-Synthase Expression.

PubMed

Lechuga, Thomas J; Zhang, Hong-hai; Sheibani, Lili; Karim, Muntarin; Jia, Jason; Magness, Ronald R; Rosenfeld, Charles R; Chen, Dong-bao

2015-06-01

Estrogens dramatically dilate numerous vascular beds with the greatest response in the uterus. Endogenous hydrogen sulfide (H2S) is a potent vasodilator and proangiogenic second messenger, which is synthesized from L-cysteine by cystathionine β-synthase (CBS) and cystathionine γ-lyase (CSE). We hypothesized that estrogen replacement therapy (ERT) selectively stimulates H2S biosynthesis in uterine artery (UA) and other systemic arteries. Intact and endothelium-denuded UA, mesenteric artery (MA), and carotid artery (CA) were obtained from ovariectomized nonpregnant ewes (n = 5/group) receiving vehicle or estradiol-17β replacement therapy (ERT). Total RNA and protein were extracted for measuring CBS and CSE, and H2S production was determined by the methylene blue assay. Paraffin-embedded UA rings were used to localize CBS and CSE proteins by immunofluorescence microscopy. ERT significantly stimulated CBS mRNA and protein without altering CSE mRNA or protein in intact and denuded UA. Quantitative immunofluorescence microscopic analyses showed CBS and CSE protein localization in endothelium and smooth muscle and confirmed that ERT stimulated CBS but not CSE protein expression in UA endothelium and smooth muscle. ERT also stimulated CBS, but not CSE, mRNA and protein expression in intact and denuded MA but not CA in ovariectomized ewes. Concomitantly, ERT stimulated UA and MA but not CA H2S production. ERT-stimulated UA H2S production was completely blocked by a specific CBS but not CSE inhibitor. Thus, ERT selectively stimulates UA and MA but not CA H2S biosynthesis by specifically up-regulating CBS expression, implicating a role of H2S in estrogen-induced vasodilation and postmenopausal women's health.

Enzyme replacement therapy in newborn mucopolysaccharidosis IVA mice: early treatment rescues bone lesions?

PubMed Central

Tomatsu, Shunji; Montaño, Adriana M.; Oikawa, Hirotaka; Dung, Vu Chi; Hashimoto, Amiko; Oguma, Toshihiro; Takahashi, Tatsuo; Shimada, Tsutomu; Orii, Tadao; Sly, William S.

2014-01-01

We treated mucopolysaccharidosis IVA (MPS IVA) mice to assess the effects of long-term enzyme replacement therapy (ERT) initiated at birth, since adult mice treated by ERT showed little improvement in bone pathology (1). To conduct ERT in newborn mice, we used recombinant human N-acetylgalactosamine-6-sulfate sulfatase (GALNS) produced in a CHO cell line. First, to observe the tissue distribution pattern, a dose of 250 units/g body weight was administered intravenously in MPS IVA mice at day 2 or 3. The infused enzyme was primarily recovered in liver and spleen, with detectable activity in bone and brain. Second, newborn ERT was conducted after tissue distribution study. The first injection of newborn ERT was performed intravenously, the second to fourth weekly injections were intraperitoneal, and the remaining injections from 5th to 14th week were intravenous into the tail vein. MPS IVA mice treated with GALNS showed clearance of lysosomal storage in liver, spleen, and sinus lining cells in bone marrow. The column structure of the growth plate was organized better than adult mice treated with ERT; however, hyaline and fibrous cartilage cells in femur, spine, ligaments, discs, synovium, and periosteum still had storage materials to some extent. Heart valves were refractory to the treatment. Levels of serum keratan sulfate were kept normal in newborn ERT mice. In conclusion, the enzyme, which enters the cartilage before the cartilage cell layer becomes mature, prevents disorganization of column structure. Early treatment from birth leads to partial remission of bone pathology in MPS IVA mouse. PMID:24953405

Safety and efficacy of enzyme replacement therapy in the nephropathy of Fabry disease

PubMed Central

Fervenza, Fernando C; Torra, Roser; Warnock, David G

2008-01-01

Kidney involvement with progressive loss of kidney function (Fabry nephropathy) is an important complication of Fabry disease, an X-linked lysosomal storage disorder arising from deficiency of α-galactosidase activity. Clinical trials have shown that enzyme replacement therapy (ERT) with recombinant human α-galactosidase clears globotriaosylceramide from kidney cells, and can stabilize kidney function in patients with mild to moderate Fabry nephropathy. Recent trials show that patients with more advanced Fabry nephropathy and overt proteinuria do not respond as well to ERT alone, but can benefit from anti-proteinuric therapy given in conjunction with ERT. This review focuses on the use of enzyme replacement therapy with agalsidase-alfa and agalsidase-beta in adults with Fabry nephropathy. The current results are reviewed and evaluated. The issues of dosing of enzyme replacement therapy, the use of adjunctive agents to control urinary protein excretion, and the individual factors that affect disease severity are reviewed. PMID:19707461

Autophagy in Natural History and After ERT in Glycogenosis Type II.

PubMed

Angelini, Corrado; Nascimbeni, Anna C; Fanin, Marina

2015-01-01

We studied the role of autophagy in a series of 10 infantile-, juvenile-, and adult-onset GSDII patients and investigated autophagy blockade in successive biopsies of adult cases during disease natural history. We also correlated the autophagosome accumulation and efficiency of enzyme replacement therapy (ERT) in four treated cases (two infantile and two juvenile-adult onsets).The autophagic flux was monitored by measuring the amount of p62-positive protein aggregates and compared, together with fibre vacuolisation, to fibre atrophy.A blocked autophagic flux resulted in p62 accumulation, increased vacuolisation, and progressive atrophy of muscle fibres in biopsies collected from patients during natural history. On the contrary, in the GSDII cases early treated with ERT, the autophagic flux improved and muscle fibre atrophy, fibre vacuolisation, and acid phosphatase activity decreased.The functionality of the autophagy-lysosome system is essential in GSDII muscle, which is characterised by the presence of swollen glycogen-filled lysosomes and autophagic build-up. Defining the role of autophagy and its relationship with muscle loss is critical for understanding the disease pathogenesis, for developing new therapies, and for improving ERT efficacy in GSDII.

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

PubMed Central

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

2017-01-01

Abstract 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. PMID:28569047

Late pubertal growth spurt in a girl with growth hormone deficiency: Is Kaufmann therapy effective in a girl with short stature who responds poorly to growth hormone therapy and estrogen-replacement therapy?

PubMed

Yasuda, Katsuhiko

2017-05-01

A Japanese senior high school girl aged 18 years and 5 months with growth hormone deficiency was referred for primary amenorrhea. Her height was 1.36 m, and her bodyweight was 23.5 kg. She had received daily growth hormone therapy from the age of 5 years. Growth hormone therapy was discontinued at the age of 16 years and 11 months, and estrogen-replacement therapy (ERT) was started to stimulate secondary sexual characteristics. Although ERT was performed until the age of 18 years and 11 months, genital bleeding did not occur. ERT was changed to Kaufmann therapy, and the first genital bleeding occurred 1 year and 4 months later. Finally, regular medically induced menses occurred at the age of 21 years and 10 months. Her height increased by 9 cm in 1 year after the initiation of menstrual bleeding. Kaufmann therapy was associated not only with menstrual bleeding but also with growth spurt. © 2017 Japan Society of Obstetrics and Gynecology.

Uneventful pregnancy outcome after enzyme replacement therapy with agalsidase beta in a heterozygous female with Fabry disease: A case report.

PubMed

Germain, Dominique P; Bruneval, Patrick; Tran, Thi-Chien; Balouet, Pierre; Richalet, Bernard; Benistan, Karelle

2010-01-01

No reproductive studies have been performed with enzyme replacement therapy (ERT) for Fabry disease (FD, OMIM 301500), a lysosomal storage disorder. Therefore, use during pregnancy is theoretically contraindicated. We report the first case of agalsidase beta treatment throughout pregnancy. High-range proteinuria remained stable and the patient gave birth to a healthy boy after an uncomplicated pregnancy. The decision to administer ERT during pregnancy should be made on an individual basis, considering the FD status and possible risks. Copyright 2009 Elsevier Masson SAS. All rights reserved.

Effect of enzyme therapy and prognostic factors in 69 adults with Pompe disease: an open-label single-center study

PubMed Central

2012-01-01

Background Enzyme replacement therapy (ERT) in adults with Pompe disease, a progressive neuromuscular disorder, is of promising but variable efficacy. We investigated whether it alters the course of disease, and also identified potential prognostic factors. Methods Patients in this open-label single-center study were treated biweekly with 20 mg/kg alglucosidase alfa. Muscle strength, muscle function, and pulmonary function were assessed every 3–6 months and analyzed using repeated-measures ANOVA. Results Sixty-nine patients (median age 52.1 years) were followed for a median of 23 months. Muscle strength increased after start of ERT (manual muscle testing 1.4 percentage points per year (pp/y); hand-held dynamometry 4.0 pp/y; both p < 0.001). Forced vital capacity (FVC) remained stable when measured in upright, but declined in supine position (−1.1 pp/y; p = 0.03). Muscle function did not improve in all patients (quick motor function test 0.7 pp/y; p = 0.14), but increased significantly in wheelchair-independent patients and those with mild and moderate muscle weakness. Relative to the pre-treatment period (49 patients with 14 months pre-ERT and 22 months ERT median follow-up), ERT affected muscle strength positively (manual muscle testing +3.3 pp/y, p < 0.001 and hand-held dynamometry +7.9 pp/y, p < 0.001). Its effect on upright FVC was +1.8 pp/y (p = 0.08) and on supine FVC +0.8 (p = 0.38). Favorable prognostic factors were female gender for muscle strength, and younger age and better clinical status for supine FVC. Conclusions We conclude that ERT positively alters the natural course of Pompe disease in adult patients; muscle strength increased and upright FVC stabilized. Functional outcome is probably best when ERT intervention is timely. PMID:23013746

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

PubMed Central

2014-01-01

Background 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 paper, we assess the cost-effectiveness of enzyme replacement therapy in infantile Pompe disease. Methods A patient simulation model was used to compare costs and effects of ERT with costs of effects of supportive therapy (ST). The model was filled with data on survival, quality of life and costs. For both arms of the model, data on survival were obtained from international literature. In addition, survival as observed among 20 classic-infantile Dutch patients, who all received ERT, was used. Quality of life was measured using the EQ-5D and assumed to be the same in both treatment groups. Costs included the costs of ERT (which depend on a child’s weight), infusions, costs of other health care utilization, and informal care. A lifetime time horizon was used, with 6-month time cycles. Results Life expectancy was significantly longer in the ERT group than in the ST group. On average, ST receiving patients were modelled not to survive the first half year of life; whereas the life expectancy in the ERT patients was modelled to be almost 14 years. Lifetime incremental QALYs were 6.8. Incremental costs were estimated to be € 7.0 million, which primarily consisted of treatment costs (95%). The incremental costs per QALY were estimated to be € 1.0 million (range sensitivity analyses: € 0.3 million - € 1.3 million). The incremental cost per life year gained was estimated to be € 0.5 million. Conclusions The incremental costs per QALY ratio is far above the conventional threshold values. Results from univariate and probabilistic sensitivity analyses showed the robustness of the results. PMID:24884717

Enzyme replacement therapy with agalsidase beta in kidney transplant patients with Fabry disease: a pilot study.

PubMed

Mignani, Renzo; Panichi, Vincenzo; Giudicissi, Antonio; Taccola, Daniele; Boscaro, Francesca; Feletti, Carlo; Moneti, Gloriano; Cagnoli, Leonardo

2004-04-01

We sought to assess the safety and efficacy of enzyme replacement therapy (ERT) with recombinant human-alpha-galactosidase A (rh-alpha-Gal A) in kidney transplant recipients with Fabry disease, a previously unstudied population. Three male kidney transplant recipients with biochemically, genetically, and histologically confirmed Fabry disease and documented Fabry myocardiopathy received the rh-alpha-Gal A, agalsidase beta, 1 mg/kg of body weight every 2 weeks by intravenous infusion and were monitored biochemically, clinically, and electrocardiographically and echocardiographically for 18 months. Patients showed biochemical, clinical/functional, and morphologic response to ERT. Plasma globotriaosylceramide decreased 23% to 50%. Extremity pain resolved within 2 months in the patient with this manifestation. On echocardiography, left ventricular mass, end diastolic diameter (EDD), and cardiac contractility, shown by ejection fraction (EF), improved in 2 of the 3 patients receiving essentially all planned infusions. EDD and EF remained basically stable, but cardiac morphologic abnormalities progressed in the other patient, who had a 5-month interruption in ERT after the initial month. Mild mitral insufficiency persisted in all patients, as did atrial fibrillation in the affected individual. After a combined total of 116 infusions, no treatment-related adverse event, intolerance, or seroconversion was seen. Renal function remained stable and the immunosuppression regimen unchanged in all patients. Our pilot study provides preliminary evidence that ERT with agalsidase beta, 1 mg/kg every 2 weeks, is safe and often effective against extra-renal manifestations in kidney transplant patients with Fabry disease. Studies with longer courses of this and higher doses of ERT are merited in this population.

Fabry disease in children: agalsidase-beta enzyme replacement therapy.

PubMed

Borgwardt, L; Feldt-Rasmussen, U; Rasmussen, A K; Ballegaard, M; Meldgaard Lund, A

2013-05-01

Fabry disease is a rare, multiorgan disease. The most serious complications involve the kidney, brain and heart. This study aims to assess the effect of enzyme replacement therapy (ERT) using agalsidase-beta in children with Fabry disease. We carried out a nationwide, descriptive and observational retrospective cohort study of 10 children (9-16 years at baseline), who underwent regular systematic investigations for 1-8 years after initiation of ERT with agalsidase-beta (Fabryzyme®, Genzyme). Ophthalmological, echocardiographic abnormalities and hypohidrosis were found at baseline and during the follow-up period. Serious kidney, heart or brain involvement had not developed at the last follow-up examination. For the majority of the patients improvements were found concerning headache, acroparaesthesias and gastrointestinal pain during the follow-up period. The level of energy and physical activity also increased. Treatment with agalsidase-beta was associated with a reduction of neuropathic and abdominal pain and headache. Although all aspects of the Fabry pain phenotype cannot be treated with ERT, the observed effects were clinically significant in the lives of the majority of Fabry children and together with the absence of serious Fabry manifestations at last follow-up, we argue that early initiation of ERT may be considered. © 2012 John Wiley & Sons A/S.

Long-term velaglucerase alfa treatment in children with Gaucher disease type 1 naïve to enzyme replacement therapy or previously treated with imiglucerase.

PubMed

Smith, Laurie; Rhead, William; Charrow, Joel; Shankar, Suma P; Bavdekar, Ashish; Longo, Nicola; Mardach, Rebecca; Harmatz, Paul; Hangartner, Thomas; Lee, Hak-Myung; Crombez, Eric; Pastores, Gregory M

2016-02-01

Gaucher Disease type 1 (GD1) often manifests in childhood. Early treatment with enzyme replacement therapy (ERT) may prevent disease complications. We report the assessment of velaglucerase alfa ERT in pediatric GD1 patients who participated in a long-term extension study (HGT-GCB-044, ClinicalTrials.gov Identifier NCT00635427). Safety and efficacy were evaluated in pediatric patients receiving velaglucerase alfa 30-60U/kg by intravenous infusion every other week. In addition to key hematological and visceral efficacy assessments, exploratory assessments conducted specifically in pediatric patients included evaluation of height, bone age, bone marrow burden, and Tanner stage of puberty. The study included 24 pediatric patients. Fifteen patients were naïve to ERT on entry into the preceding trials TKT032 (12-month trial) or HGT-GCB-039 (9-month trial): in the preceding trials, ten of these 15 patients received velaglucerase alfa and five patients received imiglucerase ERT. Nine patients in the study were previously treated with imiglucerase for >30months and were switched to velaglucerase alfa in the preceding trial TKT034 (12-month trial). Cumulative ERT exposure in the clinical studies ranged from 2.0 to 5.8years. Three serious adverse events, including a fatal convulsion, were reported; none were deemed related to velaglucerase alfa. One patient tested positive for anti-velaglucerase alfa antibodies. An efficacy assessment at 24months showed that velaglucerase alfa had positive effects on primary hematological and visceral parameters in treatment-naïve patients, which were maintained with longer-term treatment. Disease parameters were stable in patients switched from long-term imiglucerase ERT. Exploratory results may suggest benefits of early treatment to enable normal growth in pediatric patients. The safety profile and clinical response seen in pediatric patients are consistent with results reported in adults. Copyright © 2016 Shire Development LLC. Published by Elsevier Inc. All rights reserved.

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.

PubMed

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 for ERT for IOPD, e.g. modifying the enzyme to enhance uptake into skeletal muscle and/or to cross the blood brain barrier (BBB), will be required.

A prospective 10-year study of individualized, intensified enzyme replacement therapy in advanced Fabry disease.

PubMed

Schiffmann, Raphael; Swift, Caren; Wang, Xuan; Blankenship, Derek; Ries, Markus

2015-11-01

To test the hypothesis that more frequent enzyme replacement therapy (ERT) slows the decline in kidney function in adult patients with Fabry disease. A single center open label 10-year prospective clinical trial of 12 patients with advanced Fabry disease who, after having experienced an ongoing decline in renal function after 2-4 years of receiving ERT at the approved dose of 0.2 mg/kg agalsidase alfa every other week (EOW), were switched to weekly (EW) ERT at the same dose. We used linear regression to fit each individual patient's longitudinal estimated glomerular filtration rate (eGFR) record in order to compare the deterioration rates between EOW and EW ERT. For the entire group, mean slope on agalsidase alfa every 2 weeks was -7.92 ± 2.88 ml/min/1.73 m(2)/year and 3.84 ± 4.08 ml/min/1.73 m(2)/year on weekly enzyme infusions (p = 0.01, two-tailed paired t test). Three patients (25 %) completed the entire study with relatively preserved renal function while 50 % of patients reached end-stage renal disease (ESRD) during the 10 years of this study. The estimated average delay to ESRD was 13.8 years [n = 11; 95 % CI 0.66, 27]. One patient had a positive eGFR slope on weekly infusions while the patient with the highest antibody titer had a steeper slope after switching. Mean globotriaosylceramide concentrations in urine and plasma as well as urine protein excretion remained unchanged. Weekly enzyme infusions slow the decline of renal function in a subgroup of more severe patients thus showing that existing ERT can be further optimized.

ESTROGEN REPLACEMENT THERAPY INDUCES FUNCTIONAL ASYMMETRY ON AN ODOR MEMORY/DISCRIMINATION TEST

PubMed Central

Doty, Richard L.; Kisat, Mehreen; Tourbier, Isabelle

2008-01-01

The secondary afferents of the olfactory system largely project to the ipsilateral cortex without synapsing in the thalamus, making unilateral olfactory testing a useful probe of ipsilateral hemispheric activity. In light of evidence that lateralized performance on some perceptual tasks may be influenced by estrogen, we assessed left:right nostril differences in two measures of olfactory function in 14 post-menopausal women receiving estrogen replacement therapy (ERT) and 48 post-menopausal women receiving no such therapy. Relative to women not taking ERT, those receiving ERT exhibited better performance in the left nostril and poorer performance in the right nostril on an odor memory/discrimination test. Similar laterality effects were not observed for an odor detection threshold test employing phenyl ethyl alcohol. These results suggest that estrogen influences the lateralization of an odor memory/discrimination task and that hormone replacement therapy in the menopause may be an excellent paradigm for understanding lateralizing effects of hormones on some sensory processes. PMID:18466883

Activities of Daily Living in patients with Hunter syndrome: Impact of enzyme replacement therapy and hematopoietic stem cell transplantation

PubMed Central

Tanjuakio, Julian; Suzuki, Yasuyuki; Patel, Pravin; Yasuda, Eriko; Kubaski, Francyne; Tanaka, Akemi; Yabe, Hiromasa; Mason, Robert W.; Montaño, Adriana M.; Orii, Kenji E.; Orii, Koji O.; Fukao, Toshiyuki; Orii, Tadao; Tomatsu, Shunji

2014-01-01

The aim of this study was to assess the Activities of Daily Living (ADL) in patients with Hunter syndrome (mucopolysaccharidosis II; MPS II) using a newly designed ADL questionnaire. We applied the questionnaire to evaluate clinical phenotypes and therapeutic efficacies of enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT). We also explored early signs and symptoms to make early diagnosis feasible. We devised a new ADL questionnaire with three domains: “Movement,” “Movement with Cognition,” and “Cognition.” Each domain has four subcategories rated on a 5-point scale based on level of assistance. We also scored signs and symptoms unique to MPS by 12 subcategories (five points per category), providing 60 points in total. The questionnaire was first administered to 138 healthy Japanese controls (0.33 – 50 years), and successively, to 74 Japanese patients with Hunter syndrome (4 – 49 years). The patient cohort consisted of 51 severe and 23 attenuated phenotypes; 20 patients treated with HSCT, 23 patients treated early with ERT (≤ 8 years), and 25 patients treated late with ERT (> 8 years), and 4 untreated patients. Among 18 severe phenotypic patients treated by HSCT, 10 were designated as early HSCT (≤ 5 years), while 8 were designated as late HSCT (> 5 years). Scores from patients with severe phenotypes were lower than controls and attenuated phenotypes in all categories. Among patients with severe phenotypes, there was a trend that HSCT provides a higher ADL score than early ERT, and there was a significant difference in ADL scores between late ERT and HSCT groups. Early ERT and early HSCT provided a higher score than late ERT and late HSCT, respectively. In conclusion, we have evaluated the feasibility of a new questionnaire in control population and patients with Hunter syndrome, leading to a novel evaluation method for clinical phenotypes and therapeutic efficacy. Early treatment with HSCT provides a better consequence in ADL of patients. PMID:25468646

The cost-effectiveness of enzyme replacement therapy (ERT) for the infantile form of Pompe disease: comparing a high-income country's approach (England) to that of a middle-income one (Colombia).

PubMed

Castro-Jaramillo, Héctor E

2012-01-01

Determining the cost-effectiveness of enzyme replacement therapy (ERT) for the classical infantile form of Pompe disease (complete acid a-glucosidase deficiency-related) in two different settings: England and Colombia. Pompe disease is very rare (1:40,000 births incidence). A literature review was made and historic databases searched for National Health Service (NHS) reimbursed costs in England and by health insurers in Colombia; expert opinion was elicited. Two Markov models were constructed for comparing both countries; alive with symptoms and dead were the transition states used. Patients aged < 6 months receiving ERT were assumed to have 75 % survival rate and better health-related quality of life (HR-QoL) compared to those without treatment (0.700 HR- QoL using the EQ-5D scale). The incremental cost-effectiveness ratio (ICER) per quality-adjusted life year (QALY) gained was £234,307.7 for England and £109,991 for Colombia. Uncertainty about Anal HR-QoL with ERT, disease progression and cost from palliative care had the biggest impact on the ICER in both models. If ERT costs were reduced to 10,000 times per dose and HR-QoL was 0.750-0.820 ICER, then £165,000 could be attainable for England and £65,000 for Colombia. Transaction costs per case in Colombia were high. ERT was more effective than no ERT in treating infantile Pompe disease, but high levels of uncertainty still remain about survival and progression rates and QoL in the long-run. ICERs were high compared to CE thresholds. Manufacturers' ERT costs and monopoly had a major impact on Anal CEA results.

[Enzyme replacement therapy in patients with Fabry disease: state of the art and review of the literature].

PubMed

Riccio, Eleonora; Capuano, Ivana; Visciano, Bianca; Marchetiello, Cristina; Petrillo, Fortunato; Pisani, Antonio

2013-01-01

Anderson-Fabry disease is a hereditary X-linked lysosomal storage disorder caused by a deficiency of the lysosomal enzyme alpha galactosidase A. It results in the accumulation of the glycosphingolypid globotrioasoyl ceramide (Gb3 in different cells and organs, resulting in a multi-system pathology including end organ failure. Patients with Fabry disease present clinically with cardiac, renal and neurological involvement; both life expectancy and quality of life are severely compromised. The current causal treatment for Fabry disease is enzyme replacement therapy (ERT), available since 2001. The two recombinant preparations available for ERT are agalsidase alfa (Replagal) and agalsidase beta (Fabrazyme). They have both been showed to have positive effect on kidney and heart, on the symptoms of pain and quality of life. Few data to date are available on comparison of the two preparations of ERT. This article reviews evidence of the literature and shows our personal experience about the safety and efficacy of ERT.

Racial Disparities in Recurrence Among Patients with Early Stage Endometrial Cancer: Is Recurrence Increased in Black Patients on Estrogen Replacement Therapy?: A Gynecologic Oncology Group Study

PubMed Central

Maxwell, G. Larry; Tian, Chunqiao; Risinger, John I; Hamilton, Chad A.; Barakat, Richard R.

2008-01-01

Objective Population-based studies suggest that Black women with localized endometrial cancer have shorter survival compared to White patients because of inequalities in treatment. The purpose of this investigation was to determine if there is a racial disparity in outcome between Black and White patients with early stage endometrial cancer treated similarly in a clinical trial setting. Methods A retrospective review of 110 Black and 1049 White patients with stage I and II endometrial cancer was performed using data from a randomized, placebo controlled trial performed by the Gynecologic Oncology Group (GOG) that evaluated postoperative estrogen replacement therapy (ERT) and the risk of cancer recurrence. Demographic, pathologic, treatment and outcome related data were collected and analyzed using regression and survival analysis. Results Estimates of recurrence-free survival (RFS) suggested that Black patients may be more likely to have disease recurrence, particularly those on ERT. Within a median follow-up of three years, 5 of 56 Black endometrial cancer patients in the ERT group were identified with recurrent disease compared to only 8 of 521 White patients. Adjusted for age, BMI and tumor grade, the relative risk of recurrence among Blacks in the ERT group was 11.2 (95% CI: 2.86-43.59, p=0.0005). Conclusions Our findings suggest that RFS may be shorter among Black women with stage I endometrial cancer, even in a clinical trials setting in which patients receive similar treatment and followup. This increased risk of recurrence appears to be most evident in Black women with endometrial cancer who maintain ERT following primary treatment. PMID:18698590

Fabry disease, enzyme replacement therapy and the significance of antibody responses.

PubMed

Deegan, Patrick B

2012-03-01

Fabry disease is an X-linked disorder caused by a deficiency of α-galactosidase A. This leads to a progressive accumulation of globotriaosylceramide in tissues throughout the body. Cardiac, renal and neurological manifestations are common and life expectancy is significantly reduced relative to the general population. Management of Fabry disease involves the administration of intravenous enzyme replacement therapy (ERT). Two forms - agalsidase alfa and agalsidase beta - have been licensed in certain jurisdictions and are generally well tolerated; however, some patients develop antibodies to the infused enzyme, which may impair the efficacy and safety of treatment. Agalsidase alfa and agalsidase beta are produced in different systems; this leads to certain differences in post-translational modification that may affect immunogenicity. Immunoglobulin (Ig) G antibodies have frequently been reported in patients with Fabry disease receiving ERT; IgG responses are reported in a greater proportion of patients receiving agalsidase beta than in patients receiving agalsidase alfa. IgE antibodies are less common than IgG antibodies, and have not been observed in patients receiving agalsidase alfa. However, these data are difficult to interpret due to methodological differences in the assessment of seropositivity, and in the doses of enzyme used. The clinical impact of the development of IgG antibodies to ERT in patients with Fabry disease remains unclear, due to lack of data and to the marked heterogeneity of patients both in terms of disease manifestations and response to therapy. Further studies that examine the development of antibodies in patients with Fabry disease and the potential impact of such antibodies on the outcome of ERT are necessary.

The effectiveness and cost-effectiveness of enzyme and substrate replacement therapies: a longitudinal cohort study of people with lysosomal storage disorders.

PubMed

Wyatt, K; Henley, W; Anderson, L; Anderson, R; Nikolaou, V; Stein, K; Klinger, L; Hughes, D; Waldek, S; Lachmann, R; Mehta, A; Vellodi, A; Logan, S

2012-01-01

To determine natural history and estimate effectiveness and cost of enzyme replacement therapy (ERT) and substrate replacement therapy (SRT) for patients with Gaucher disease, Fabry disease, mucopolysaccharidosis type I (MPS I), mucopolysaccharidosis type II (MPS II), Pompe disease and Niemann-Pick type C (NPC) disease. Cohort study including prospective and retrospective clinical- and patient-reported data. Age- and gender-adjusted treatment effects were estimated using generalised linear mixed models. Treated patients contributed data before and during treatment. Untreated patients contributed natural history data. National Specialised Commissioning Group-designated lysosomal storage disorder (LSD) treatment centres in England. Consenting adults and children with a diagnosis of Gaucher disease (n = 272), Fabry disease (n = 499), MPS I (n = 126), MPS II (n = 58), NPC (n = 58) or Pompe disease (n = 93) who had attended a treatment centre in England. ERT and SRT. Clinical outcomes chosen by clinicians to reflect disease progression for each disorder; patient-reported quality-of-life (QoL) data; cost of treatment and patient-reported service-use data; numbers of hospitalisations, outpatient and general practitioner appointments; medication use; data pertaining to associated family/carer costs and QoL impacts. Seven hundred and eleven adults and children were recruited. In those with Gaucher disease (n = 175) ERT was associated with improved platelet count, haemoglobin, liver function and reduced risk of enlarged liver or spleen. No association was found between ERT and QoL. In patients with Fabry disease (n = 311) increased time on ERT was associated with small decreases in left ventricular mass and improved glomerular filtration rate, but not with changes in risk of stroke/transient ischaemic attacks or the need for a hearing aid. There was a statistically significant association between duration of ERT use and worsening QoL and fatigue scores. We found no statistical difference in estimates of treatment effectiveness between the two preparations, agalsidase beta (Fabrazyme(®), Genzyme) (n = 127) and agalsidase alpha (Replagal(®), Shire HGT) (n = 91), licensed for this condition. In Pompe disease (n = 77) our data provide some evidence of a beneficial effect on muscle strength and mobility as measured by a 6-minute walk test in adult-onset patients; there were insufficient data from infantile-onset Pompe patients to estimate associations between ERT and outcome. Among subjects with MPS I (n = 68), 42 of the 43 patients with MPS I subtype Hurler's disease had undergone a bone marrow transplant. No significant associations were found between ERT and any outcome measure for the MPS I subtype Scheie disease and heparan sulphate patients. An association between duration of ERT and growth in children was the only statistically significant finding among patients with MPS II (n = 39). There were insufficient data for patients with NPC disease to draw any conclusions regarding the effectiveness of SRT. The current annual cost to the NHS of the different ERTs means that between 3.6 and 17.9 discounted quality-adjusted life-years (QALYs) for adult patients and between 2.6 and 10.5 discounted QALYs for child patients would need to be generated for each year of being on treatment for ERTs to be considered cost-effective by conventional criteria. These data provide further evidence on the effectiveness of ERT in people with LSDs. However, the results need to be interpreted in light of the fact that the data are observational and the relative lack of power due to the small numbers of patients with MPS I, MPS II, Pompe disease and NPC disease. Future work should aim to effectively address the unanswered questions and this will require agreement on a common set of outcome measures and their consistent collection across all treatment centres. This project was funded by the NIHR Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 16, No. 39. See the HTA programme website for further project information.

Clinical observations on enzyme replacement therapy in patients with Fabry disease and the switch from agalsidase beta to agalsidase alfa.

PubMed

Lin, Hsiang-Yu; Huang, Yu-Hsiu; Liao, Hsuan-Chieh; Liu, Hao-Chuan; Hsu, Ting-Rong; Shen, Chia-I; Li, Shao-Tzu; Li, Cheng-Fang; Lee, Li-Hong; Lee, Pi-Chang; Huang, Chun-Kai; Chiang, Chuan-Chi; Lin, Shuan-Pei; Niu, Dau-Ming

2014-04-01

Fabry disease is an X-linked inherited lysosomal storage disease that can be treated with the enzymes of agalsidase beta (Fabrazyme) and agalsidase alfa (Replagal). Since June 2009, viral contamination of Genzyme's production facility has resulted in a worldwide shortage of agalsidase beta, leading to the switch to agalsidase alfa for patients with Fabry disease in Taiwan. The medical records were retrospectively reviewed for nine male patients with Fabry disease from the start of agalsidase beta treatment until the switch to agalsidase alfa for at least 1 year. After 12-112 months of enzyme replacement therapy (ERT), decreased plasma globotriaosylsphingosine (lyso-Gb3) was found in five out of seven patients, indicating improvement in disease severity. Among the six patients with available echocardiographic data at baseline and after ERT, all six experienced reductions of left ventricular mass index. Renal function, including microalbuminuria and estimated glomerular filtration rate, showed stability after ERT. Mainz Severity Score Index scores revealed that all nine patients remained stable at 12 months after switching to agalsidase alfa. ERT improved or stabilized cardiac status and stabilized renal function, while reducing plasma lyso-Gb3. ERT was well tolerated, even among the three patients who had hypersensitivity reactions. The switch of ERT from agalsidase beta to agalsidase alfa appears to be safe after 1 year of follow-up for Taiwanese patients with Fabry disease. Copyright © 2013. Published by Elsevier B.V.

CD77 levels over enzyme replacement treatment in Fabry Disease Family (V269M).

PubMed

Pereira, Ester Miranda; Silva, Adalberto Socorro da; Silva, Raimundo Nonato da; Monte Neto, José Tiburcio; Nascimento, Fernando F do; Sousa, Jackeline L M; Costa Filho, Henrique César Saraiva de Arêa Leão; Sales Filho, Herton Luiz Alves; Labilloy, Anatalia; Monte, Semiramis Jamil Hadad do

2018-06-04

Fabry disease (FD) is a disorder caused by mutations in the gene encoding for lysosomal enzyme α-galactosidase A (α-GAL). Reduced α-GAL activity leads to progressive accumulation of globotriaosylceramide (Gb3), also known as CD77. The recent report of increased expression of CD77 in blood cells of patients with FD indicated that this molecule can be used as a potential marker for monitoring enzyme replacement therapy (ERT). The purpose of this study was to evaluate the CD77 levels throughout ERT in FD patients (V269M mutation). We evaluated the fluctuations in PBMC (peripheral blood mononuclear cell) membrane CD77 expression in FD patients undergoing ERT and correlated these levels with those observed in different cell types. A greater CD77 expression was found in phagocytes of patients compared to controls at baseline. Interestingly, the variability in CD77 levels is larger in patients at baseline (340 - 1619 MIF) and after 12 months of ERT (240 - 530 MIF) compared with the control group (131 - 331 MFI). Furthermore, by analyzing the levels of CD77 in phagocytes from patients throughout ERT, we found a constant decrease in CD77 levels. The increased CD77 levels in the phagocytes of Fabry carriers together with the decrease in CD77 levels throughout ERT suggest that measuring CD77 levels in phagocytes is a promising tool for monitoring the response to ERT in FD.

[Development of an orphan drug to treat a genetic disease: the paradigm of agalsidase beta].

PubMed

Germain, Dominique P; Benistan, Karelle

2007-03-01

Preclinical and phase I/II studies gave the proof of principle of enzyme replacement therapy (ERT) with recombinant alpha-galactosidase A through the demonstration of the clearance of the accumulated subtrate from plasma and tissues. In a multicenter, randomized, placebo-controlled, double-blind phase Ill study, the biological efficacy of recombinant alpha-galactosidose A (agalsidase beta 1 mg/kg/714 days) was demonstrated on the basis of complete clearance of accumulated globotriaosylceramide from the endothelia of the kidney, heart and skin. The phase III extension study data gives additional results: kidney function appears to be stabilized after 54 to 60 months of treatment with agolsidase beta in most patients. Intent-to-treat analysis of a double-blind, randomized, placebo-controlled, phase IV study, showed that, adjusted for on imbalance in baseline proteinuria, agalsidase beta significantly reduces by 53% the risk of a first clinical event (renal, cardiac and cerebrovascular), compared with placebo. Clinical benefits of ERT depend on patients' clinical status at baseline, therefore prompting for onset of ERT before irreversible damage occur and underlying the need to stratify patients' populations to better understand the outcome of ERT.

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

PubMed

Alfadhel, Majid; Sirrs, Sandra

2011-01-01

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.

Progressive renal failure despite long-term biweekly enzyme replacement therapy in a patient with Fabry disease secondary to a new α-galactosidase mutation of Leu311Arg (L311R).

PubMed

Suzuki, Keisuke; Miura, Naoto; Kitagawa, Wataru; Suzuki, Shinkichi; Komatsuda, Atsushi; Nishikawa, Kazuhiro; Watanabe, Daisuke; Imai, Hirokazu

2011-12-01

A 37-year-old Japanese man affected by Fabry disease secondary to a novel mutation of Leu311Arg (L311R) in α-galactosidase demonstrated progressive renal failure despite biweekly enzyme replacement therapy (ERT) for approximately 10 years. Kidney biopsy revealed foamy glomerular epithelial cells, compatible with the typical pathologic features of Fabry disease. The patient entered a phase III study of Replagal (agalsidase alfa) in 2001, allowing him to continue ERT with biweekly dosing for almost 10 years. During 2 years of that period, he was continued on Fabrazyme (agalsidase beta) biweekly dosing. His estimated GFR was calculated to decrease by 9.9 mL/min/1.73 m(2) per year. Patients with Fabry disease have been reported to have a mean decrease in GFR of 12.2 ± 8.1 mL/min/1.73 m(2) per year. This result suggests that biweekly ERT is only mildly effective at preventing loss of kidney function.

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

PubMed Central

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.

Severe infusion reactions to fabry enzyme replacement therapy: rechallenge after tracheostomy.

PubMed

Nicholls, K; Bleasel, K; Becker, G

2012-01-01

A 34-year-old male patient with Fabry disease (OMIM 301500) commenced enzyme replacement therapy (ERT) with Agalsidase alfa, with positive clinical response. Infusion reactions, initially mild and easily managed, commenced during his 13th infusion, and continued over the next 3 years. Severity of reactions subsequently increased despite very slow infusion, extended prophylactic medication and attempted desensitisation, requiring regular intensive care unit (ICU) admissions. Facial oedema and flushing, throat tightness, headache and joint pain typically occurred 4-36 h after completion of most infusions, responding rapidly to subcutaneous adrenaline. Low titre specific IgG seroconversion was noted at 12 months, with subsequent reversion to negative after 5 years, despite persistence of infusion reactions. Specific IgE and skin testing was negative. Trial of ERT product switch to Agalsidase-beta resulted in no improvement in reactions. At 5 years, ERT was ceased in the face of recurrent ICU readmissions. In the face of progressive clinical deterioration, he underwent tracheostomy to allow recommencement of ERT. Two years later, he has clinically improved on regular attenuated dose Agalsidase-beta, administered by slow infusion in a local hospital setting.

Futile Interhospital Transfer for Endovascular Treatment in Acute Ischemic Stroke: The Madrid Stroke Network Experience.

PubMed

Fuentes, Blanca; Alonso de Leciñana, María; Ximénez-Carrillo, Alvaro; Martínez-Sánchez, Patricia; Cruz-Culebras, Antonio; Zapata-Wainberg, Gustavo; Ruiz-Ares, Gerardo; Frutos, Remedios; Fandiño, Eduardo; Caniego, Jose L; Fernández-Prieto, Andrés; Méndez, Jose C; Bárcena, Eduardo; Marín, Begoña; García-Pastor, Andrés; Díaz-Otero, Fernando; Gil-Núñez, Antonio; Masjuán, Jaime; Vivancos, Jose; Díez-Tejedor, Exuperio

2015-08-01

The complexity of endovascular revascularization treatment (ERT) in acute ischemic stroke and the small number of patients eligible for treatment justify the development of stroke center networks with interhospital patient transfers. However, this approach might result in futile transfers (ie, the transfer of patients who ultimately do not undergo ERT). Our aim was to analyze the frequency of these futile transfers and the reasons for discarding ERT and to identify the possible associated factors. We analyzed an observational prospective ERT registry from a stroke collaboration ERT network consisting of 3 hospitals. There were interhospital transfers from the first attending hospital to the on-call ERT center for the patients for whom this therapy was indicated, either primarily or after intravenous thrombolysis (drip and shift). The ERT protocol was activated for 199 patients, 129 of whom underwent ERT (64.8%). A total of 120 (60.3%) patients required a hospital transfer, 50 of whom (41%) ultimately did not undergo ERT. There were no differences in their baseline characteristics, the times from stroke onset, or in the delays in interhospital transfers between the transferred patients who were treated and those who were not treated. The main reasons for rejecting ERT after the interhospital transfer were clinical improvement/arterial recanalization (48%) and neuroimaging criteria (32%). Forty-one percent of the ERT transfers were futile, but none of the baseline patient characteristics predicted this result. Futility could be reduced if repetition of unnecessary diagnostic tests was avoided. © 2015 American Heart Association, Inc.

The beneficial effects of long-term enzyme replacement therapy on cardiac involvement in Japanese Fabry patients.

PubMed

Hongo, Kenichi; Ito, Keiichi; Date, Taro; Anan, Ikuko; Inoue, Yasunori; Morimoto, Satoshi; Ogawa, Kazuo; Kawai, Makoto; Kobayashi, Hiroshi; Kobayashi, Masahisa; Ida, Hiroyuki; Ohashi, Toya; Taniguchi, Ikuo; Yoshimura, Michihiro; Eto, Yoshikatsu

2018-06-01

Fabry disease is a hereditary disorder that occurs due to the reduction or absence of alpha-galactosidase A activity, which leads to cardiac involvement including left ventricular hypertrophy (LVH). Enzyme replacement therapy (ERT) provides better patient outcomes by preventing serious complications. However, there have been very few studies on the long-term effects of ERT on the cardiac manifestations in Japanese Fabry patients. We retrospectively analyzed the data from the medical records of 42 Fabry patients (male, n = 17; female, n = 25) who were followed at Jikei University Hospital, and in whom the long-term effects of ERT could be evaluated (median follow-up period: male, 11 years; female, 8 years). The slope of the left ventricular mass (LVM) increase was 3.02 ± 3.41 g/m 2 /year in males and 1.69 ± 2.73 g/m 2 /year in females. In a subgroup analysis, the slopes of males with and without LVH did not differ to a statistically significant extent; however, the slope in female patients without LVH was significantly smaller than that of female patients with LVH. We then compared our data to the natural historical data that have previously been reported. In comparison to the previously reported data, we found a significant reduction in the LVM changes (g/height 2.7 /year) of patients who received long-term ERT (male, 4.07 ± 1.03 to 1.25 ± 1.39; female, 2.31 ± 0.81 to 0.78 ± 1.23). Long-term ERT effectively prevents LVH in Fabry patients. This effect was also observed in the patients with LVH prior to the initiation of ERT. Copyright © 2018 Elsevier Inc. All rights reserved.

Cost-effectiveness of enzyme replacement therapy for Fabry disease.

PubMed

Rombach, Saskia M; Hollak, Carla E M; Linthorst, Gabor E; Dijkgraaf, Marcel G W

2013-02-19

The cost-effectiveness of enzyme replacement therapy (ERT) compared to standard medical care was evaluated in the Dutch cohort of patients with Fabry disease. Cost-effectiveness analysis was performed using a life-time state-transition model. Transition probabilities, effectiveness data and costs were derived from retrospective data and prospective follow-up of the Dutch study cohort consisting of males and females aged 5-78 years. Intervention with ERT (either agalsidase alfa or agalsidase beta) was compared to the standard medical care. The main outcome measures were years without end organ damage (renal, cardiac en cerebrovascular complications), quality adjusted life years (QALYs), and costs. Over a 70 year lifetime, an untreated Fabry patient will generate 55.0 years free of end-organ damage (53.5 years in males, 56.9 years in females) and 48.6 QALYs (47.8 in males, 49.7 in females). Starting ERT in a symptomatic patient increases the number of years free of end-organ damage by 1.5 year (1.6 in males, 1.3 in females), while the number of QALYs gained increases by a similar amount (1.7 in males, 1.4 in females). The costs of ERT starting in the symptomatic stage are between €9 - €10 million (£ 7.9 - £ 8.8 million, $13.0- $14.5 million) during a patient's lifetime. Consequently, the extra costs per additional year free of end-organ damage and the extra costs per additional QALY range from €5.5 - €7.5 million (£ 4.8 - £ 6.6 million, $ 8.0 - $ 10.8 million), undiscounted. In symptomatic patients with Fabry disease, ERT has limited effect on quality of life and progression to end organ damage. The pharmaco-economic evaluation shows that this modest effectiveness drives the costs per QALY and the costs per year free of end-organ damage to millions of euros. Differentiation of patients who may benefit from ERT should be improved to enhance cost-effectiveness.

Effects of enzyme replacement therapy for cardiac-type Fabry patients with a Chinese hotspot late-onset Fabry mutation (IVS4+919G>A)

PubMed Central

Lin, Hsiang-Yu; Liu, Hao-Chuan; Huang, Yu-Hsiu; Liao, Hsuan-Chieh; Hsu, Ting-Rong; Shen, Chia-I; Li, Shao-Tzu; Li, Cheng-Fang; Lee, Li-Hong; Lee, Pi-Chang; Huang, Chun-Kai; Chiang, Chuan-Chi; Lin, Ching-Yuang; Lin, Shuan-Pei; Niu, Dau-Ming

2013-01-01

Objective Current studies of newborn screening for Fabry disease in Taiwan have revealed a remarkably high prevalence of cardiac-type Fabry disease with a Chinese hotspot late-onset Fabry mutation (IVS4+919G>A). Design Retrospective cohort study. Setting Tertiary medical centre. Participants 21 patients with cardiac-type Fabry disease (15 men and 6 women) as well as 15 patients with classic Fabry disease (4 men and 11 women) treated with biweekly intravenous infusions of agalsidase β (1 mg/kg) or agalsidase α (0.2 mg/kg) for at least 6 months. Outcome measures These data were collected at the time before enzyme replacement therapy (ERT) began and followed up after ERT for at least 6 months, including patient demographics, medical history, parameter changes of cardiac status and renal functions, plasma globotriaosylsphingosine (lyso-Gb3) and Mainz Severity Score Index. Results After 6–39 months of ERT, plasma lyso-Gb3 was found to be reduced in 89% (17/19) and 93% (14/15) of patients with cardiac-type and classic Fabry disease, respectively, which indicated an improvement of disease severity. For patients with cardiac-type Fabry disease, echocardiography revealed the reduction or stabilisation of left ventricular mass index (LVMI), the thicknesses of intraventricular septum (IVS) and left posterior wall (LPW) in 83% (15/18), 83% (15/18) and 67% (12/18) of patients, respectively, as well as 77% (10/13), 73% (11/15) and 60% (9/15) for those with classic type. Most patients showed stable renal function after ERT. There were statistically significant improvements (p<0.05) between the data at baseline and those after ERT for values of plasma lyso-Gb3, LVMI, IVS, LPW and Mainz Severity Score Index. No severe clinical events were reported during the treatment. Conclusions ERT is beneficial and appears to be safe for Taiwanese patients with cardiac-type Fabry disease, as well as for those with the classic type. PMID:23864212

Enzyme replacement therapy on hypophosphatasia mouse model

PubMed Central

Montaño, Adriana M.; Shimada, Tsutomu; Sly, William S.

2013-01-01

Hypophosphatasia (HPP) is an inborn error of metabolism caused by deficiency of the tissue-nonspecific alkaline phosphatase (TNSALP), resulting in a defect of bone mineralization. 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. Enzyme replacement therapy (ERT) for HPP by functional TNSALP is one of the therapeutic options. The C-terminal-anchorless human recombinant TNSALP derived from Chinese hamster ovary cell lines was purified. TNSALP-null mice (Akp2−/−), an infantile model of HPP, were treated from birth using TNSALP and vitamin B6 diet. Long-term efficacy studies of ERT consisted of every 3 days subcutaneous or intravenous injections till 28 days old (dose 20 U/g) and subsequently every 3 days intravenous injections for 6 months (dose 10 U/g). We assessed therapeutic effect by growth and survival rates, fertility, skeletal manifestations, and radiographic and pathological finding. Treated Akp2−/− mice grew normally till 4 weeks and appeared well with a minimum skeletal abnormality as well as absence of epilepsy, compared with untreated mice which died by 3 weeks old. The prognosis of TNSALP-treated Akp2−/− mice was improved substantially: 1) prolonged life span over 6 months, 2) improvement of the growth, and 3) normal fertility. After 6 months of treatment, we found moderate hypomineralization with abnormal proliferative chondrocytes in growth plate and articular cartilage. In conclusion, ERT with human native TNSALP improves substantial clinical manifestations in Akp2−/− mice, suggesting that ERT with anchorless TNSALP is also a potential therapy for HPP. PMID:23978959

Enzyme replacement therapy improves joint motion and outcome of the 12-min walk test in a mucopolysaccharidosis type VI patient previously treated with bone marrow transplantation.

PubMed

Sohn, Young Bae; Park, Sung Won; Kim, Se-Hwa; Cho, Sung-Yoon; Ji, Sun-Tae; Kwon, Eun Kyung; Han, Sun Ju; Oh, Se Jung; Park, Yong Jae; Ko, Ah-Ra; Paik, Kyung-Hoon; Lee, Jeehun; Lee, Dong Hwan; Jin, Dong-Kyu

2012-05-01

Mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome, OMIM #253200) is a rare disorder involving multiple organs and manifested particularly by severe skeletal abnormalities. Bone marrow transplantation (BMT) improves cardiopulmonary function and facial features, but has limited success in ameliorating skeletal abnormalities and short stature. Here, we report the outcome of enzyme replacement therapy (ERT) with recombinant human arylsulfatase-B (ASB, Naglazyme, BioMarin, Novato, CA) in an MPS VI patient who received BMT 10 years prior to ERT induction. Administration of weekly Naglazyme for 18 months was effective in improving range of motion in several joints [shoulders (improvement of flexion (Right/Left): 40°/55°; improvement of extension 30°/40°; improvement of abduction 10°/10°), elbows (improvement of flexion 25°/25°; improvement of extension 10°/15°), hips (improvement of flexion 25°/10°), and knees (improvement of flexion 45°/40°; improvement of extension 50°/60°)]. Improvement in the outcome of the 12-min walk test (70% increase) and 3-min stair-climbing test (29% increase) was also noted after ERT. Because ERT improved clinical features in an MPS VI patient who had undergone prior BMT, the role of ERT post successful BMT in MPS VI needs further investigation. Copyright © 2012 Wiley Periodicals, Inc.

Successful management of enzyme replacement therapy in related fabry disease patients with severe adverse events by switching from agalsidase Beta (fabrazyme(®)) to agalsidase alfa (replagal (®)).

PubMed

Tsuboi, Kazuya; Yamamoto, Hiroshi; Somura, Fuji; Goto, Hiromi

2015-01-01

Enzyme replacement therapy (ERT) is the only approved therapy for Fabry disease. In June 2009, there was a worldwide shortage of agalsidase beta, necessitating dose reductions or switching to agalsidase alfa in some patients. We present two cases of Fabry disease (a parent and a child) who received agalsidase beta for 27 months at the licensed dose and 10 months at a reduced dose, followed by a switch to agalsidase alfa for 28 months. Case 1, a 26-year-old male had severe coughing and fatigue during ERT with agalsidase beta requiring antitussive and asthmatic drug therapy. After switching to agalsidase alfa, the coughing gradually resolved completely. Case 2, a 62-year-old female had advanced cardiac manifestations at the time of diagnosis. Despite receiving ERT with the approved dose of agalsidase beta, she experienced aggravation of congestive heart failure and was hospitalized. After switching to agalsidase alfa with standard care in heart disease, BNP level, echocardiographic parameters, eGFR rate and lyso-Gb3 levels were improved or stabilized. We report on two Fabry disease patients who experienced severe adverse events while on approved and/or reduced doses of agalsidase beta. Switching to agalsidase alfa associated with standard care in heart disease led to resolution or improvement in the cardiorespiratory status. And reduction in dose associated with standard care in respiratory disease was useful for decrease in cough and fatigue. Plasma BNP level was useful for monitoring heart failure and the effects of ERT.

Exocrine pancreatic insufficiency is not a cause of abdominal complaints in patients with Fabry disease.

PubMed

Vujasinovic, Miroslav; Tepes, Bojan; Vujkovac, Bojan; Cokan Vujkovac, Andreja; Tretjak, Martin; Korat, Vesna

2015-12-01

Fabry disease (FD), also called Anderson-Fabry disease, is the second most prevalent lysosomal storage disorder after Gaucher disease. Gastrointestinal (GI) symptoms are very common among male and female individuals, although the age of onset is later among female patients. To our best knowledge, exocrine pancreatic insufficiency (EPI) has not yet been studied in patients with FD as a possible cause of abdominal complaints. The aim of our study was to determine whether exocrine pancreatic function is impaired in patients with FD. We analysed medical records of patients with FD treated in Fabry Center in Slovenj Gradec General Hospital (Slovenian referral centre for FD) by the evaluation of the following features: gender, age, first symptoms before confirmation of FD diagnosis, time interval between first symptoms and diagnosis, therapy and current abdominal complaints. Diagnosis of FD was established by genetic analysis and confirmation of mutation in the α-galactosidase A gene. Faecal elastase-1 (FE-1) measurements were performed using enzyme-linked immunosorbent assay and the commercial kit ScheBo Biotech, Giessen, Germany. There were 28 adult patients (Slovene, Caucasians) with known FD included in the study: 12 male and 16 female; mean age, 45.6 ± 14.3 (range, 19-75) years. Seventeen patients (63%) were on enzyme replacement therapy (ERT). In seven (25.9%) patients, abdominal complaints (diarrhoea, bloating and feeling of satiety) were present before introduction of ERT. In three out of these seven patients, abdominal complaints resolved after ERT, and in four patients, they were still occasionally present. FE-1 was normal in all patients (547.9 ± 104.5 µg/g). Our results show that exocrine pancreatic function is normal in all patients with FD and is most likely not a cause of abdominal complaints in this group of patients. Nevertheless, EPI still could not be completely excluded as an aetiology factor for GI problems in patients with FD because all our patients with GI problems were treated with ERT. Therefore, a potential effect of ERT on EPI cannot be excluded. Further studies are necessary to determine the aetiology, especially in the group of naïve male patients.

Recommendations for initiation and cessation of enzyme replacement therapy in patients with Fabry disease: the European Fabry Working Group consensus document.

PubMed

Biegstraaten, Marieke; Arngrímsson, Reynir; Barbey, Frederic; Boks, Lut; Cecchi, Franco; Deegan, Patrick B; Feldt-Rasmussen, Ulla; Geberhiwot, Tarekegn; Germain, Dominique P; Hendriksz, Chris; Hughes, Derralynn A; Kantola, Ilkka; Karabul, Nesrin; Lavery, Christine; Linthorst, Gabor E; Mehta, Atul; van de Mheen, Erica; Oliveira, João P; Parini, Rossella; Ramaswami, Uma; Rudnicki, Michael; Serra, Andreas; Sommer, Claudia; Sunder-Plassmann, Gere; Svarstad, Einar; Sweeb, Annelies; Terryn, Wim; Tylki-Szymanska, Anna; Tøndel, Camilla; Vujkovac, Bojan; Weidemann, Frank; Wijburg, Frits A; Woolfson, Peter; Hollak, Carla E M

2015-03-27

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 to define European consensus recommendations for the initiation and cessation of ERT in patients with FD. A Delphi procedure was conducted with an online survey (n = 28) and a meeting (n = 15). Patient organization representatives were present at the meeting to give their views. Recommendations were accepted with ≥75% agreement and no disagreement. For classically affected males, consensus was achieved that ERT is recommended as soon as there are early clinical signs of kidney, heart or brain involvement, but may be considered in patients of ≥16 years in the absence of clinical signs or symptoms of organ involvement. Classically affected females and males with non-classical FD should be treated as soon as there are early clinical signs of kidney, heart or brain involvement, while treatment may be considered in females with non-classical FD with early clinical signs that are considered to be due to FD. Consensus was achieved that treatment should not be withheld from patients with severe renal insufficiency (GFR < 45 ml/min/1.73 m(2)) and from those on dialysis or with cognitive decline, but carefully considered on an individual basis. Stopping ERT may be considered in patients with end stage FD or other co-morbidities, leading to a life expectancy of <1 year. In those with cognitive decline of any cause, or lack of response for 1 year when the sole indication for ERT is neuropathic pain, stopping ERT may be considered. Also, in patients with end stage renal disease, without an option for renal transplantation, in combination with advanced heart failure (NYHA class IV), cessation of ERT should be considered. ERT in patients who are non-compliant or fail to attend regularly at visits should be stopped. The recommendations can be used as a benchmark for initiation and cessation of ERT, although final decisions should be made on an individual basis. Future collaborative efforts are needed for optimization of these recommendations.

Globotriaosylsphingosine (lyso-Gb3) might not be a reliable marker for monitoring the long-term therapeutic outcomes of enzyme replacement therapy for late-onset Fabry patients with the Chinese hotspot mutation (IVS4+919G>A)

PubMed Central

2014-01-01

Background In Taiwan, DNA-based newborn screening showed a surprisingly high incidence (1/875 in males and 1/399 in females) of a cardiac Fabry mutation (IVS4 + 919G > A). However, the natural course, long-term treatment outcomes and suitable biomarkers for monitoring the therapeutic outcomes of these patients are largely unknown. Methods Fabry disease (FD) patients who had received enzyme replacement therapy (ERT) for more than 1 year were enrolled in this study from December 2008 to April 2013. Periodic echocardiography and serum globotriaosylsphingosine (lyso-Gb3) analysis were carried out. Before and after ERT, left ventricular mass index (LVMI) and serum lyso-Gb3 level were compared and the correlation between the change of LVMI and the change of serum lyso-Gb3 were also analyzed. Results Thirty-six patients, in four patient groups, were enrolled: (1) 16 males with IVS4 + 919G > A mutation; (2) 7 females with IVS4 + 919G > A mutation; (3) 2 males with classical mutations; and (4) 11 females with classical mutations. The follow-up period was 13–46 months. There were significant LVMI reductions after ERT in all four groups after excluding confounding factors. However, interestingly, serum lyso-Gb3 decreased significantly in the early period after ERT in all groups, but increased gradually after an average of 11.1 months after ERT in late-onset male and female Fabry groups, even when their LVMI still decreased or remained stable. Furthermore, there was no correlation between the change of serum lyso-Gb3 and the change of LVMI in both classical and IVS4 + 919G > A FD patients. Conclusion Although lyso-Gb3 has a high diagnostic sensitivity in late-onset Fabry patients and has a good response to ERT during the early stages, it might not be a reliable marker for monitoring the long-term therapeutic outcomes of ERT for late-onset Fabry patients with the Chinese hotspot mutation (IVS4 + 919G > A). PMID:25047006

Sphingolipid metabolism diseases.

PubMed

Kolter, Thomas; Sandhoff, Konrad

2006-12-01

Human diseases caused by alterations in the metabolism of sphingolipids or glycosphingolipids are mainly disorders of the degradation of these compounds. The sphingolipidoses are a group of monogenic inherited diseases caused by defects in the system of lysosomal sphingolipid degradation, with subsequent accumulation of non-degradable storage material in one or more organs. Most sphingolipidoses are associated with high mortality. Both, the ratio of substrate influx into the lysosomes and the reduced degradative capacity can be addressed by therapeutic approaches. In addition to symptomatic treatments, the current strategies for restoration of the reduced substrate degradation within the lysosome are enzyme replacement therapy (ERT), cell-mediated therapy (CMT) including bone marrow transplantation (BMT) and cell-mediated "cross correction", gene therapy, and enzyme-enhancement therapy with chemical chaperones. The reduction of substrate influx into the lysosomes can be achieved by substrate reduction therapy. Patients suffering from the attenuated form (type 1) of Gaucher disease and from Fabry disease have been successfully treated with ERT.

Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study.

PubMed

Imbriaco, M; Pisani, A; Spinelli, L; Cuocolo, A; Messalli, G; Capuano, E; Marmo, M; Liuzzi, R; Visciano, B; Cianciaruso, B; Salvatore, M

2009-07-01

Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. This study aimed to assess the long-term effects of ERT with recombinant alpha-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson-Fabry disease. Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson-Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). At 45 months of treatment, LV mass and LV wall thickness had significantly reduced: 188 (SD 60) g versus 153 (47) g, and 16 (4) mm versus 14 (4) mm, respectively. Furthermore, a significant reduction in myocardial T2 relaxation times was noted in all myocardial regions, that is, interventricular septum 80 (5) ms versus 66 (8) ms, apex 79 (10) ms versus 64 (10) ms, and lateral wall 80 (8) ms versus 65 (16) ms. Changes in LV ejection fraction were not significant. Amelioration of clinical symptoms was observed in all patients. Long-term therapy with agalsidase beta at 1 mg/kg every 2 weeks was effective in significantly reducing LV hypertrophy, improving overall cardiac performance and ameliorating clinical symptoms in patients with Anderson-Fabry disease.

Variations in plasma and urinary lipids in response to enzyme replacement therapy for Fabry disease patients by nanoflow UPLC-ESI-MS/MS.

PubMed

Byeon, Seul Kee; Kim, Jin Yong; Lee, Jin-Sung; Moon, Myeong Hee

2016-03-01

A deficiency of α-galactosidase A causes Fabry disease (FD) by disrupting lipid metabolism, especially trihexosylceramide (THC). Enzyme replacement therapy (ERT) is clinically offered to FD patients in an attempt to lower the accumulated lipids. Studies on specific types of lipids that are directly or indirectly altered by FD are very scarce, even though they are crucial in understanding the biological process linked to the pathogenesis of FD. We performed a comprehensive lipid profiling of plasma and urinary lipids from FD patients with nanoflow liquid chromatography electrospray-ionization tandem mass spectrometry (nLC-ESI-MS/MS) and identified 129 plasma and 111 urinary lipids. Among these, lipids that exhibited alternations (>twofold) in patients were selected as targets for selected reaction monitoring (SRM)-based high-speed quantitation using nanoflow ultra-performance LC-ESI-MS/MS (nUPLC-ESI-MS/MS) and 31 plasma and 26 urinary lipids showed significant elevation among FD patients. Higher percentages of sphingolipids (SLs; 48% for plasma and 42% for urine) were highly elevated in patients; whereas, a smaller percentage of phospholipids (PLs; 15% for plasma and 13% for urine) were significantly affected. Even though α-galactosidase A is reported to affect THC only, the results show that other classes of lipids (especially SLs) are changed as well, indicating that FD not only alters metabolism of THC but various classes of lipids too. Most lipids showing significant increases in relative amounts before ERT decreased after ERT, but overall, ERT influenced plasma lipids more than urinary lipids.

Pulmonary involvement in Fabry disease: effect of plasma globotriaosylsphingosine and time to initiation of enzyme replacement therapy.

PubMed

Franzen, Daniel; Haile, Sarah R; Kasper, David C; Mechtler, Thomas P; Flammer, Andreas J; Krayenbühl, Pierre A; Nowak, Albina

2018-01-01

Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by mutations of GLA gene leading to reduced α-galactosidase activity and resulting in a progressive accumulation of globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3). Plasma Lyso-Gb3 levels serve as a disease severity and treatment monitoring marker during enzyme replacement therapy (ERT). Adult patients with AFD who had yearly pulmonary function tests between 1999 and 2015 were eligible for this observational study. Primary outcome measures were the change in z-score of forced expiratory volume in the first second (FEV 1 ) and FEV 1 /FVC over time. Plasma Lyso-Gb3 levels and the age of ERT initiation were investigated for their association with lung function decline. Fifty-three patients (42% male, median (range) age at diagnosis of AFD 34 (6-61) years in men, 34 (13-67) in women) were included. The greatest decrease of FEV 1 /FVC z-scores was observed in Classic men (-0.048 per year, 95% CI -0.081 to -0.014), compared with the Later-Onset men (+0.013,95% CI -0.055 to 0.082), Classic women (-0.008, 95% CI -0.035 to +0.020) and Later-Onset women (-0.013, 95% CI -0.084 to +0.058). Cigarette smoking (P=0.022) and late ERT initiation (P=0.041) were independently associated with faster FEV 1 decline. FEV 1 /FVC z-score decrease was significantly reduced after initiation of ERT initiation (-0.045 compared with -0.015, P=0.014). Furthermore, there was a trend towards a relevant influence of Lyso-Gb3 (P=0.098) on airflow limitation with age. Early ERT initiation seems to preserve pulmonary function. Plasma Lyso-Gb3 is maybe a useful predictor for airflow limitation. Classic men need a closer monitoring of the lung function.

Effects of long-term estrogen replacement therapy on bone turnover in periarticular tibial osteophytes in surgically postmenopausal cynomolgus monkeys

PubMed Central

Olson, Erik J.; Lindgren, Bruce R.; Carlson, Cathy S.

2008-01-01

The aims of the present study were to assess the effects of long-term estrogen replacement therapy (ERT) on size and indices of bone turnover in periarticular osteophytes in ovariectomized cynomolgus monkeys and to compare dynamic indices of bone turnover in osteophyte bone with those of subchondral bone (SCB) and epiphyseal/metaphyseal cancellous (EMC) bone. One hundred sixty-five adult female cynomolgus macaques were bilaterally ovariectomized and randomly divided into three age- and weight-matched treatment groups for a 36-month treatment period. Group 1 (OVX control) received no treatment, Group 2 (SPE) received soy phytoestrogens, and Group 3 (ERT) received conjugated equine estrogens in the diet; all monkeys were labeled with calcein before necropsy. A midcoronal, plastic-embedded section of the right proximal tibia from 20 randomly selected animals per treatment group was examined histologically. Forty-nine of the sections (OVX control, n=16; SPE, n=16; ERT, n=17) contained lateral abaxial osteophytes, and static and dynamic histomorphometry measurements were taken from osteophyte bone, SCB from the lateral tibial plateau, and EMC bone. Data were analyzed using the ANOVA and Kruskal-Wallis test, correlation and regression methods, and the Friedman and Wilcoxon signed rank test. There was no significant effect of long-term ERT on osteophyte area or on any static or dynamic histomorphometry parameters. The bone volume, trabecular number, and trabecular thickness in osteophyte bone were considerably higher than in EMC bone; whereas, trabecular separation was considerably lower in osteophyte bone. In all three treatment groups, BS/BV was significantly lower in osteophyte bone vs. EMC bone and significantly higher in osteophyte bone vs. lateral SCB. We conclude that osteophyte area and static and dynamic histomorphometry parameters within periarticular tibial osteophytes in ovariectomized cynomolgus monkeys are not significantly influenced by long-term ERT, but that site differences in static and dynamic bone histomorphometry parameters exist, particularly between EMC and osteophyte bone. PMID:18291743

Effects of long-term estrogen replacement therapy on bone turnover in periarticular tibial osteophytes in surgically postmenopausal cynomolgus monkeys.

PubMed

Olson, Erik J; Lindgren, Bruce R; Carlson, Cathy S

2008-05-01

The aims of the present study were to assess the effects of long-term estrogen replacement therapy (ERT) on size and indices of bone turnover in periarticular osteophytes in ovariectomized cynomolgus monkeys and to compare dynamic indices of bone turnover in osteophyte bone with those of subchondral bone (SCB) and epiphyseal/metaphyseal cancellous (EMC) bone. One hundred sixty-five adult female cynomolgus macaques were bilaterally ovariectomized and randomly divided into three age- and weight-matched treatment groups for a 36-month treatment period. Group 1 (OVX control) received no treatment, Group 2 (SPE) received soy phytoestrogens, and Group 3 (ERT) received conjugated equine estrogens in the diet; all monkeys were labeled with calcein before necropsy. A midcoronal, plastic-embedded section of the right proximal tibia from 20 randomly selected animals per treatment group was examined histologically. Forty-nine of the sections (OVX control, n=16; SPE, n=16; ERT, n=17) contained lateral abaxial osteophytes, and static and dynamic histomorphometry measurements were taken from osteophyte bone, SCB from the lateral tibial plateau, and EMC bone. Data were analyzed using the ANOVA and Kruskal-Wallis test, correlation and regression methods, and the Friedman and Wilcoxon signed rank test. There was no significant effect of long-term ERT on osteophyte area or on any static or dynamic histomorphometry parameters. The bone volume, trabecular number, and trabecular thickness in osteophyte bone were considerably higher than in EMC bone; whereas, trabecular separation was considerably lower in osteophyte bone. In all three treatment groups, BS/BV was significantly lower in osteophyte bone vs. EMC bone and significantly higher in osteophyte bone vs. lateral SCB. We conclude that osteophyte area and static and dynamic histomorphometry parameters within periarticular tibial osteophytes in ovariectomized cynomolgus monkeys are not significantly influenced by long-term ERT, but that site differences in static and dynamic bone histomorphometry parameters exist, particularly between EMC and osteophyte bone.

Enzyme replacement therapy for mucopolysaccharidosis VI: Growth and pubertal development in patients treated with recombinant human N-acetylgalactosamine 4-sulfatase

PubMed Central

Decker, Celeste; Yu, Zi-Fan; Giugliani, Roberto; Schwartz, Ida Vanessa D.; Guffon, Nathalie; Teles, Elisa Leão; Miranda, M. Clara Sá; Wraith, J. Edmond; Beck, Michael; Arash, Laila; Scarpa, Maurizio; Ketteridge, David; Hopwood, John J.; Plecko, Barbara; Steiner, Robert; Whitley, Chester B.; Kaplan, Paige; Swiedler, Stuart J.; Conrad, Susan; Harmatz, Paul

2010-01-01

Background and Methods Growth failure is characteristic of untreated mucopolysaccharidosis type VI (MPS VI: Maroteaux-Lamy syndrome). Growth was studied in fifty-six MPS VI patients (5 to 29 years old) prior to and for up to 240 weeks of weekly infusions of recombinant human arylsulfatase B (rhASB) at 1 mg/kg during Phase 1/2, Phase 2, Phase 3 or Phase 3 Extension clinical trials. Height, weight, and Tanner stage data were collected. Pooled data were analyzed to determine mean height increase by treatment week, growth impacts of pubertal status, baseline urinary GAG, and age at treatment initiation. Growth rate for approximately 2 years prior to and following treatment initiation was analyzed using longitudinal modeling. Results Mean height increased by 2.9 cm after 48 weeks and 4.3 cm after 96 weeks on enzyme replacement therapy (ERT). Growth on ERT was not correlated with baseline urinary GAG. Patients under 16 years of age showed greatest increases in height on treatment. Model results based on pooled data showed significant improvement in growth rate during 96 weeks of ERT when compared to the equivalent pretreatment time period. Delayed pubertal onset or progression was noted in 10 patients entering the clinical trials; all of whom showed progression of at least one Tanner stage during 2 years on ERT, and 6 of whom (60%) completed puberty. Conclusion Analysis of mean height by treatment week and longitudinal modeling demonstrate significant increase in height and growth rate in MPS VI patients receiving long-term ERT. This impact was greatest in patients aged below 16 years. Height increase may result from bone growth and/or reduction in joint contractures. Bone growth and resolution of delayed puberty may be related to improvements in general health, bone cell health, nutrition, endocrine gland function and reduced inflammation. PMID:20634905

Epidemiology of estrogen and dementia in women with Down syndrome.

PubMed

Schupf, Nicole; Lee, Joseph H; Pang, Deborah; Zigman, Warren B; Tycko, Benjamin; Krinsky-McHale, Sharon; Silverman, Wayne

2018-01-01

Several lines of investigation have shown a protective role for estrogen in Alzheimer's disease through a number of biological actions. This review examines studies of the role of estrogen-related factors in age at onset and risk for Alzheimer's disease in women with Down syndrome, a population at high risk for early onset of dementia. The studies are consistent in showing that early age at menopause and that low levels of endogenous bioavailable estradiol in postmenopausal women with Down syndrome are associated with earlier age at onset and overall risk for dementia. Polymorphisms in genes associated with estrogen receptor activity and in genes for estrogen biosynthesis affecting endogenous estrogen are related to age at onset and cumulative incidence of dementia, and may serve as biomarkers of risk. To date, no clinical trials of estrogen or hormone replacement therapy (ERT/HRT) have been published for women with Down syndrome. While findings from clinical trials of ERT or HRT for dementia have generally been negative among women in the neurotypical population, the short interval between menopause and onset of cognitive decline, together with a more positive balance between potential benefits and risks, suggests an opportunity to evaluate the efficacy of ERT/HRT for delaying or preventing dementia in this high risk population, although questions concerning the optimal formulation and timing of the hormone therapy are not yet resolved. Copyright © 2017 Elsevier Inc. All rights reserved.

Cardiac energy metabolism is disturbed in Fabry disease and improves with enzyme replacement therapy using recombinant human galactosidase A.

PubMed

Machann, Wolfram; Breunig, Frank; Weidemann, Frank; Sandstede, Jörn; Hahn, Dietbert; Köstler, Herbert; Neubauer, Stefan; Wanner, Christoph; Beer, Meinrad

2011-03-01

In vitro studies have shown impairment of energy metabolism in cardiac fibroblasts from Fabry patients. A recent in vivo study reported an association between cardiac energy metabolism and increased myocardial mass in Fabry patients. We therefore assessed possible disturbances of cardiac energy metabolism in Fabry patients by in vivo (31)P-MR-spectroscopy. Additionally, the effect of enzyme replacement therapy (ERT) on cardiac energetics was tested. Twenty-three patients (41 ± 9 years; 10 females) with genetically proven Fabry disease were examined with a 1.5 T Scanner, and compared with an age-matched healthy control group. Eight patients underwent ERT and had follow-up examinations after 3 and 14 months. The high-energy phosphate molecules phosphocreatine (PCr) and adenosine triphosphate (ATP) were quantified in localized 31P-spectra by SLOOP (spectral localization with optimum point spread function). Cine- and late gadolinium enhancement (LGE) studies were also performed. When compared with healthy controls, Fabry patients demonstrated reduced PCr- (6.1 ± 1.9 vs. 8.8 ± 2.6 mmol/kg; P = 0.003) and ATP concentrations (3.9 ± 1.5 vs. 4.6 ± 1.0 mmol/kg; P = 0.048). During ERT, PCr concentrations increased (7.1 ± 1.5 mmol/kg vs. 6.1 ± 1.9; P < 0.05) and left ventricular mass decreased (215 ± 55 vs. 185 ± 45 g; P = 0.012). Disturbances in cardiac energetics were not correlated to the presence or absence of cardiac fibrosis on LGE. Cardiac energy metabolism is disturbed in Fabry disease; this may play an important role in the pathogenesis of Fabry cardiomyopathy. Enzyme replacement therapy ameliorates energetic depression.

Clinical efficacy of gene-modified stem cells in adenosine deaminase-deficient immunodeficiency.

PubMed

Shaw, Kit L; Garabedian, Elizabeth; Mishra, Suparna; Barman, Provaboti; Davila, Alejandra; Carbonaro, Denise; Shupien, Sally; Silvin, Christopher; Geiger, Sabine; Nowicki, Barbara; Smogorzewska, E Monika; Brown, Berkley; Wang, Xiaoyan; de Oliveira, Satiro; Choi, Yeong; Ikeda, Alan; Terrazas, Dayna; Fu, Pei-Yu; Yu, Allen; Fernandez, Beatriz Campo; Cooper, Aaron R; Engel, Barbara; Podsakoff, Greg; Balamurugan, Arumugam; Anderson, Stacie; Muul, Linda; Jagadeesh, G Jayashree; Kapoor, Neena; Tse, John; Moore, Theodore B; Purdy, Ken; Rishi, Radha; Mohan, Kathey; Skoda-Smith, Suzanne; Buchbinder, David; Abraham, Roshini S; Scharenberg, Andrew; Yang, Otto O; Cornetta, Kenneth; Gjertson, David; Hershfield, Michael; Sokolic, Rob; Candotti, Fabio; Kohn, Donald B

2017-05-01

Autologous hematopoietic stem cell transplantation (HSCT) of gene-modified cells is an alternative to enzyme replacement therapy (ERT) and allogeneic HSCT that has shown clinical benefit for adenosine deaminase-deficient (ADA-deficient) SCID when combined with reduced intensity conditioning (RIC) and ERT cessation. Clinical safety and therapeutic efficacy were evaluated in a phase II study. Ten subjects with confirmed ADA-deficient SCID and no available matched sibling or family donor were enrolled between 2009 and 2012 and received transplantation with autologous hematopoietic CD34+ cells that were modified with the human ADA cDNA (MND-ADA) γ-retroviral vector after conditioning with busulfan (90 mg/m2) and ERT cessation. Subjects were followed from 33 to 84 months at the time of data analysis. Safety of the procedure was assessed by recording the number of adverse events. Efficacy was assessed by measuring engraftment of gene-modified hematopoietic stem/progenitor cells, ADA gene expression, and immune reconstitution. With the exception of the oldest subject (15 years old at enrollment), all subjects remained off ERT with normalized peripheral blood mononuclear cell (PBMC) ADA activity, improved lymphocyte numbers, and normal proliferative responses to mitogens. Three of nine subjects were able to discontinue intravenous immunoglobulin replacement therapy. The MND-ADA vector was persistently detected in PBMCs (vector copy number [VCN] = 0.1-2.6) and granulocytes (VCN = 0.01-0.3) through the most recent visits at the time of this writing. No patient has developed a leukoproliferative disorder or other vector-related clinical complication since transplant. These results demonstrate clinical therapeutic efficacy from gene therapy for ADA-deficient SCID, with an excellent clinical safety profile. ClinicalTrials.gov NCT00794508. Food and Drug Administration Office of Orphan Product Development award, RO1 FD003005; NHLBI awards, PO1 HL73104 and Z01 HG000122; UCLA Clinical and Translational Science Institute awards, UL1RR033176 and UL1TR000124.

Clinical efficacy of gene-modified stem cells in adenosine deaminase–deficient immunodeficiency

PubMed Central

Shaw, Kit L.; Garabedian, Elizabeth; Mishra, Suparna; Barman, Provaboti; Davila, Alejandra; Carbonaro, Denise; Shupien, Sally; Silvin, Christopher; Geiger, Sabine; Nowicki, Barbara; Smogorzewska, E. Monika; Brown, Berkley; Wang, Xiaoyan; de Oliveira, Satiro; Choi, Yeong; Ikeda, Alan; Terrazas, Dayna; Fu, Pei-Yu; Yu, Allen; Fernandez, Beatriz Campo; Cooper, Aaron R.; Engel, Barbara; Podsakoff, Greg; Balamurugan, Arumugam; Anderson, Stacie; Muul, Linda; Jagadeesh, G. Jayashree; Kapoor, Neena; Tse, John; Moore, Theodore B.; Purdy, Ken; Rishi, Radha; Mohan, Kathey; Skoda-Smith, Suzanne; Buchbinder, David; Abraham, Roshini S.; Scharenberg, Andrew; Yang, Otto O.; Cornetta, Kenneth; Gjertson, David; Hershfield, Michael; Sokolic, Rob; Candotti, Fabio

2017-01-01

BACKGROUND. Autologous hematopoietic stem cell transplantation (HSCT) of gene-modified cells is an alternative to enzyme replacement therapy (ERT) and allogeneic HSCT that has shown clinical benefit for adenosine deaminase–deficient (ADA-deficient) SCID when combined with reduced intensity conditioning (RIC) and ERT cessation. Clinical safety and therapeutic efficacy were evaluated in a phase II study. METHODS. Ten subjects with confirmed ADA-deficient SCID and no available matched sibling or family donor were enrolled between 2009 and 2012 and received transplantation with autologous hematopoietic CD34+ cells that were modified with the human ADA cDNA (MND-ADA) γ-retroviral vector after conditioning with busulfan (90 mg/m2) and ERT cessation. Subjects were followed from 33 to 84 months at the time of data analysis. Safety of the procedure was assessed by recording the number of adverse events. Efficacy was assessed by measuring engraftment of gene-modified hematopoietic stem/progenitor cells, ADA gene expression, and immune reconstitution. RESULTS. With the exception of the oldest subject (15 years old at enrollment), all subjects remained off ERT with normalized peripheral blood mononuclear cell (PBMC) ADA activity, improved lymphocyte numbers, and normal proliferative responses to mitogens. Three of nine subjects were able to discontinue intravenous immunoglobulin replacement therapy. The MND-ADA vector was persistently detected in PBMCs (vector copy number [VCN] = 0.1–2.6) and granulocytes (VCN = 0.01–0.3) through the most recent visits at the time of this writing. No patient has developed a leukoproliferative disorder or other vector-related clinical complication since transplant. CONCLUSION. These results demonstrate clinical therapeutic efficacy from gene therapy for ADA-deficient SCID, with an excellent clinical safety profile. TRIAL REGISTRATION. ClinicalTrials.gov NCT00794508. FUNDING. Food and Drug Administration Office of Orphan Product Development award, RO1 FD003005; NHLBI awards, PO1 HL73104 and Z01 HG000122; UCLA Clinical and Translational Science Institute awards, UL1RR033176 and UL1TR000124. PMID:28346229

Gauchers disease--a reappraisal of hematopoietic stem cell transplantation.

PubMed

Ito, Sawa; Barrett, A John

2013-03-01

Hematopoietic stem cell transplantation (HSCT), first performed in 1984, was the first treatment approach for Gaucher's disease (GD) which had curative intent. The early successes in HSCT were soon eclipsed by the introduction of a highly effective enzyme replacement therapy (ERT), which has remained the single most widely used treatment. Experience with HSCT is limited to about 50 reported cases, mainly performed in the last century, with an overall survival around 85%. HSCT typically achieves complete correction of visceral and bony changes and can fully stabilize neurological features in otherwise progressive type II and III GD. ERT, in contrast, is completely safe and effective, but is limited by cost, incomplete resolution of visceral, hematological, and bony features in some patients, and lack of neurological correction in type II and III disease. In this review, we summarize and compare HSCT and ERT. With 20 years of experience of ERT, its limitations as well as its advantages are now well delineated. Meanwhile progress in HSCT over the last decade suggests that transplantation would today represent a very safe curative approach for GD offering one time complete correction of the disease, contrasting with the lifelong need for ERT with its associated expense and dependence on sophisticated drug manufacture. Additionally, unlike ERT, HSCT can be beneficial for neurological forms of GD. We conclude that the time has come to re-evaluate HSCT in selected patients with GD where ERT is less likely to fully eradicate symptoms of the disease.

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

PubMed

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 confirmed the better outcome of the CRIM-positive patients but at the same time, showed the inability of the current therapeutic approach to reverse or stabilize the disease progression. The results also evidenced the involvement of central nervous system in Pompe disease. To better understand the disease clinical history and to improve treatment efficacy larger multicentre studies are needed as well as the development of new therapeutic approaches.

Enzyme replacement therapy in classical infantile pompe disease: results of a ten-month follow-up study.

PubMed

Klinge, L; Straub, V; Neudorf, U; Voit, T

2005-02-01

Infantile Pompe disease (IPD) is a fatal, autosomal recessive muscle-wasting disorder. Due to a deficiency of the lysosomal enzyme acid alpha-glucosidase patients develop a generalized myopathy, diaphragmatic weakness, and cardiomyopathy leading to death usually within the first year of life. So far there is no therapy available. We report on the safety and efficacy of transgenically derived recombinant human precursor acid alpha-glucosidase (rhGAA) in a 10-month follow-up study in two children with IPD who previously completed a 48-week course of enzyme replacement therapy (ERT) with the same medication at the same dose in a phase II clinical trial. Under this therapy cardiac status and muscle strength had improved, leading to survival beyond the age of one year. These results, together with data from two other phase II clinical trials encouraged further evaluation of the long-term safety and efficacy of enzyme replacement therapy in patients with infantile-onset Pompe disease. During the 10-month follow-up period, ERT was well-tolerated and neither patient experienced a single infusion-associated reaction. The initial improvements in cardiac size and function, as measured by left ventricular mass index and the fractional shortening, were maintained in both patients, and a continued improvement of motor function, as measured by the Alberta infant motor scale, was observed.

Reduction of elevated plasma globotriaosylsphingosine in patients with classic Fabry disease following enzyme replacement therapy.

PubMed

van Breemen, Mariëlle J; Rombach, Saskia M; Dekker, Nick; Poorthuis, Ben J; Linthorst, Gabor E; Zwinderman, Aeilko H; Breunig, Frank; Wanner, Christoph; Aerts, Johannes M; Hollak, Carla E

2011-01-01

Fabry disease is treated by two-weekly infusions with α-galactosidase A, which is deficient in this X-linked globotriaosylceramide (Gb3) storage disorder. Elevated plasma globotriaosylsphingosine (lysoGb3) is a hallmark of classical Fabry disease. We investigated effects of enzyme replacement therapy (ERT) on plasma levels of lysoGb3 and Gb3 in patients with classical Fabry disease treated with agalsidase alfa at 0.2mg/kg, agalsidase beta at 0.2mg/kg or at 1.0mg/kg bodyweight. Each treatment regimen led to prominent reductions of plasma lysoGb3 in Fabry males within 3 months (P=0.0313), followed by relative stability later on. Many males developed antibodies against α-galactosidase A, particularly those treated with agalsidase beta. Patients with antibodies tended towards smaller correction in plasma lysoGb3 concentration, whereas treatment with high dose agalsidase beta allowed a reduction comparable to patients without antibodies. Pre-treatment plasma lysoGb3 concentrations of Fabry females were relatively low. In all females and with each treatment regimen, ERT gave reduction or stabilisation of plasma lysoGb3. Our investigation revealed that ERT of Fabry patients reduces plasma lysoGb3, regardless of the recombinant enzyme used. This finding shows that ERT can correct a characteristic biochemical abnormality in Fabry patients. Copyright © 2010 Elsevier B.V. All rights reserved.

Experimental Model for Successful Liver Cell Therapy by Lenti TTR-YapERT2 Transduced Hepatocytes with Tamoxifen Control of Yap Subcellular Location

PubMed Central

Yovchev, Mladen; Jaber, Fadi L.; Lu, Zhonglei; Patel, Shachi; Locker, Joseph; Rogler, Leslie E.; Murray, John W.; Sudol, Marius; Dabeva, Mariana D.; Zhu, Liang; Shafritz, David A.

2016-01-01

Liver repopulation by transplanted hepatocytes has not been achieved previously in a normal liver microenvironment. Here we report that adult rat hepatocytes transduced ex vivo with a lentivirus expressing a human YapERT2 fusion protein (hYapERT2) under control of the hepatocyte-specific transthyretin (TTR) promoter repopulate normal rat liver in a tamoxifen-dependent manner. Transplanted hepatocytes expand very slowly but progressively to produce 10% repopulation at 6 months, showing clusters of mature hepatocytes that are fully integrated into hepatic parenchyma, with no evidence for dedifferentiation, dysplasia or malignant transformation. Thus, we have developed the first vector designed to regulate the growth control properties of Yap that renders it capable of producing effective cell therapy. The level of liver repopulation achieved has significant translational implications, as it is 2-3x the level required to cure many monogenic disorders of liver function that have no underlying hepatic pathology and is potentially applicable to diseases of other tissues and organs. PMID:26763940

Glucocerebroside: an evolutionary advantage for patients with Gaucher disease and a new immunomodulatory agent.

PubMed

Ilan, Yaron; Elstein, Deborah; Zimran, Ari

2009-10-01

Gaucher disease (GD) is caused by the reduced activity of a lysosomal enzyme, glucocerebrosidase, leading to the accumulation of glucocerebroside (GC). The relatively high prevalence of this disease within an ethnic group is believed to reflect a selective advantage. Treatment with enzyme replacement therapy (ERT) is safe and effective in ameliorating the primary symptoms of the disease, yet there have been reports that some patients on ERT have developed type 2 diabetes or metabolic syndrome, malignancies and central nervous system disorders. A series of animal studies suggest that these complications may be related to the reduction of GC levels by the enzyme administered. GC has been shown to have an immunomodulatory effect through the promotion of dendritic cells, natural killer T cells, and regulatory T cells. The break down of GC to ceramide can underline part of these findings. Clinical trials suggested a beneficial effect of GC in type 2 diabetes or nonalcoholic steatohepatitis. This review of the data from animal models and humans proposes that the increased level of GC may provide an evolutionary advantage for patients with GD. Indirectly, these data support treating symptomatic patients with mild/moderate GD with low-dose ERT and re-evaluating the use of ERT in asymptomatic patients.

Deleterious effects of interruption followed by reintroduction of enzyme replacement therapy on a lysosomal storage disorder.

PubMed

Schneider, Ana Paula; Matte, Ursula; Pasqualim, Gabriela; Tavares, Angela Maria Vicente; Mayer, Fabiana Quoos; Martinelli, Barbara; Ribas, Graziela; Vargas, Carmen Regla; Giugliani, Roberto; Baldo, Guilherme

2016-10-01

Temporary interruption of enzyme replacement therapy (ERT) in patients with different lysosomal storage disorders may happen for different reasons (adverse reactions, issues with reimbursement, logistic difficulties, and so forth), and the impact of the interruption is still uncertain. In the present work, we studied the effects of the interruption of intravenous ERT (Laronidase, Genzyme) followed by its reintroduction in mice with the prototypical lysosomal storage disorder mucopolysaccharidosis type I, comparing to mice receiving continuous treatment, untreated mucopolysaccharidosis type I mice, and normal mice. In the animals which treatment was temporarily interrupted, we observed clear benefits of treatment in several organs (liver, lung, heart, kidney, and testis) after reintroduction, but a worsening in the thickness of the aortic wall was detected. Furthermore, these mice had just partial improvements in behavioral tests, suggesting some deterioration in the brain function. Despite worsening is some disease aspects, urinary glycosaminoglycans levels did not increase during interruption, which indicates that this biomarker commonly used to monitor treatment in patients should not be used alone to assess treatment efficacy. The deterioration observed was not caused by the development of serum antienzyme antibodies. All together our results suggest that temporary ERT interruption leads to deterioration of function in some organs and should be avoided whenever possible. Copyright © 2016 Elsevier Inc. All rights reserved.

[Clinical and histological findings in Fabry nephropathy].

PubMed

Pieruzzi, Federico; Salerno, Fabio; Di Giacomo, Antonella; Torti, Giacomo; Ferrario, Franco; Pagni, Fabio; Stella, Andrea

2013-01-01

Fabry disease is a complex pathology, requiring a multidisciplinar approach both in the diagnostic workout and in the management of therapy. Clinical criteria able to predict its morbidity have not yet been found. The wide variability of clinical signs and symptoms requires an individual approach based on the single patient, in order to achieve an optimal management. Enzyme replacement therapy (ERT) has been introduced in the clinical setting for over ten years, but its ability to change the course of the disease has not yet been clearly proved. Recently the hypothesis that ERT may be ineffective in patients with severe organ involvement has emerged. The clinical course of Fabry disease is usually slower in eterozygous women than emizygous men, but can be frequently associated to severe organ failure and premature death in both cases. In this review we discuss the histological aspects of Fabry nephropathy in relation to diagnosis, prognosis, therapy and its effectiveness.

Long-term outcome of enzyme-replacement therapy in advanced Fabry disease: evidence for disease progression towards serious complications

PubMed Central

Weidemann, F; Niemann, M; Störk, S; Breunig, F; Beer, M; Sommer, C; Herrmann, S; Ertl, G; Wanner, C

2013-01-01

Objective The long-term effects of enzyme-replacement therapy (ERT) in Fabry disease are unknown. Thus, the aim of this study was to determine whether ERT in patients with advanced Fabry disease affects progression towards ‘hard’ clinical end-points in comparison with the natural course of the disease. Methods A total of 40 patients with genetically proven Fabry disease (mean age 40 ± 9 years; n = 9 women) were treated prospectively with ERT for 6 years. In addition, 40 subjects from the Fabry Registry, matched for age, sex, chronic kidney disease stage and previous transient ischaemic attack (TIA), served as a comparison group. The main outcome was a composite of stroke, end-stage renal disease (ESRD) and death. Secondary outcomes included changes in myocardial left ventricular (LV) wall thickness and replacement fibrosis, change in glomerular filtration rate (GFR), new TIA and change in neuropathic pain. Results During a median follow-up of 6.0 years (bottom and top quartiles: 5.1, 7.2), 15 events occurred in 13 patients (n = 7 deaths, n = 4 cases of ESRD and n = 4 strokes). Sudden death occurred (n = 6) only in patients with documented ventricular tachycardia and myocardial replacement fibrosis. The annual progression of myocardial LV fibrosis in the entire cohort was 0.6 ± 0.7%. As a result, posterior end-diastolic wall thinning was observed (baseline, 13.2 ± 2.0 mm; follow-up, 11.4 ± 2.1 mm; P < 0.01). GFR decreased by 2.3 ± 4.6 mL min−1 per year. Three patients experienced a TIA. The major clinical symptom was neuropathic pain (n = 37), and this symptom improved in 25 patients. The event rate was not different between the ERT group and the untreated (natural history) group of the Fabry Registry. Conclusion Despite ERT, clinically meaningful events including sudden cardiac death continue to develop in patients with advanced Fabry disease. PMID:23586858

Early treatment with laronidase improves clinical outcomes in patients with attenuated MPS I: a retrospective case series analysis of nine sibships.

PubMed

Al-Sannaa, Nouriya A; Bay, Luisa; Barbouth, Deborah S; Benhayoun, Youssef; Goizet, Cyril; Guelbert, Norberto; Jones, Simon A; Kyosen, Sandra Obikawa; Martins, Ana Maria; Phornphutkul, Chanika; Reig, Celia; Pleat, Rebecca; Fallet, Shari; Ivanovska Holder, Iva

2015-10-07

Enzyme replacement therapy (ERT) with laronidase, (recombinant human α-L-iduronidase; Aldurazyme) is the primary treatment option for patients with attenuated mucopolysaccharidosis type I (MPS I). This study examined the effect of early ERT on clinical manifestations. This multinational, retrospective case series abstracted data from records of 20 patients with Hurler-Scheie syndrome within nine sibships that included older siblings treated with laronidase after the development of significant clinical symptoms, and younger siblings treated before significant symptomatology. Median age at diagnosis was 5.6 and 0.5 years for older and younger siblings, respectively. Median age at ERT initiation was 7.9 and 1.9 years for older and younger siblings, respectively. Improvement or stabilization of somatic signs and symptoms was more notable in younger siblings. Organomegaly present at onset of ERT improved in the majority of both older and younger siblings. Analysis of physician-rated symptom severity demonstrated that cardiac, musculoskeletal, and cognitive symptoms, when absent or mild in younger siblings at ERT initiation, generally did not develop or progress. The majority of older siblings had height/length Z-scores greater than two standard deviations below the mean (less than -2) at both time points. In general, Z-scores for younger siblings were closer to the sex- and age-matched means at follow-up. These findings suggest early initiation of laronidase, prior to the onset of symptoms in patients with attenuated MPS I, can slow or prevent the development of severe clinical manifestations.

Antibody Epitope of Human α-Galactosidase A Revealed by Affinity Mass Spectrometry: A Basis for Reversing Immunoreactivity in Enzyme Replacement Therapy of Fabry Disease.

PubMed

Kukacka, Zdenek; Iurascu, Marius; Lupu, Loredana; Rusche, Hendrik; Murphy, Mary; Altamore, Lorenzo; Borri, Fabio; Maeser, Stefan; Papini, Anna Maria; Hennermann, Julia; Przybylski, Michael

2018-05-08

α-Galactosidase (αGal) is a lysosomal enzyme that hydrolyses the terminal α-galactosyl moiety from glycosphingolipids. Mutations in the encoding genes for αGal lead to defective or misfolded enzyme, which results in substrate accumulation and subsequent organ dysfunction. The metabolic disease caused by a deficiency of human α-galactosidase A is known as Fabry disease or Fabry-Anderson disease, and it belongs to a larger group known as lysosomal storage diseases. An effective treatment for Fabry disease has been developed by enzyme replacement therapy (ERT), which involves infusions of purified recombinant enzyme in order to increase enzyme levels and decrease the amounts of accumulated substrate. However, immunoreactivity and IgG antibody formation are major, therapy-limiting, and eventually life-threatening complications of ERT. The present study focused on the epitope determination of human α-galactosidase A against its antibody formed. Here we report the identification of the epitope of human αGal(309-332) recognized by a human monoclonal anti-αGal antibody, using a combination of proteolytic excision of the immobilized immune complex and surface plasmon resonance biosensing mass spectrometry. The epitope peptide, αGal(309-332), was synthesized by solid-phase peptide synthesis. Determination of its affinity by surface plasmon resonance analysis revealed a high binding affinity for the antibody (K D =39×10 -9  m), which is nearly identical to that of the full-length enzyme (K D =16×10 -9  m). The proteolytic excision affinity mass spectrometry method is shown here to be an efficient tool for epitope identification of an immunogenic lysosomal enzyme. Because the full-length αGal and the antibody epitope showed similar binding affinities, this provides a basis for reversing immunogenicity upon ERT by: 1) treatment of patients with the epitope peptide to neutralize antibodies, or 2) removal of antibodies by apheresis, and thus significantly improving the response to ERT. © 2018 Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim.

Treatment-naïve Gaucher disease patients achieve therapeutic goals and normalization with velaglucerase alfa by 4years in phase 3 trials.

PubMed

Zimran, Ari; Elstein, Deborah; Gonzalez, Derlis E; Lukina, Elena A; Qin, Yulin; Dinh, Quinn; Turkia, Hadhami Ben

2018-02-01

Gaucher disease is an inherited metabolic disease characterized by β-glucocerebrosidase deficiency and commonly treated with enzyme replacement therapy (ERT). The efficacy of ERT with velaglucerase alfa was assessed based on the achievement of published therapeutic goals and the normalization of disease parameters in 39 treatment-naïve patients with type 1 Gaucher disease, 6 to 62years of age, enrolled in phase 3 clinical trials. After 4years of ERT, therapeutic goals for thrombocytopenia and splenomegaly had been achieved in 100% of patients; goals for anemia and hepatomegaly had been achieved in 95% and 94% of patients, respectively. Consistent with the goal for bone mineral density, lumbar spine bone density improved in 87% of patients ≥18years of age. At year 4, compared with clinical ranges for healthy individuals, 86% of patients with a low baseline hemoglobin concentration had normalized, 60% with a low baseline platelet count had normalized, 67% with baseline splenomegaly had normalized, 58% with hepatomegaly had normalized, and lumbar spine bone density had normalized in 53% of adults. The decade-old therapeutic goals do not reflect the potential for normalization of clinical parameters in ERT-treated patients. Goals consistent with normalization or near-normalization should be considered. ClinicalTrials.gov identifiers: NCT00430625, NCT00553631, NCT00635427. Copyright © 2016 Shire Human Genetic Therapies, Inc. Published by Elsevier Inc. All rights reserved.

Enzyme replacement therapy with agalsidase beta improves cardiac involvement in Fabry's disease.

PubMed

Spinelli, L; Pisani, A; Sabbatini, M; Petretta, M; Andreucci, M V; Procaccini, D; Lo Surdo, N; Federico, S; Cianciaruso, B

2004-08-01

Fabry's disease is an X-linked lysosomal storage disease caused by a deficiency of alpha-galactosidase that results in an accumulation of neutral glycosphingolipids throughout the body, including the cardiovascular system. Fabry cardiomyopathy, characterized by progressive severe concentric left ventricular (LV) hypertrophy, is very frequent and is the most important cause of death in affected patients. Enzyme replacement therapy (ERT) allows a specific treatment for this disease, however, there are very few data on the effectiveness of therapy on cardiac involvement. Nine patients with Fabry cardiac disease were studied on basal condition and after 6 and 12 months of treatment with algasidase beta (Fabrazyme). A complete clinical, electrocardiographic and echocardiographic evaluation was performed in all patients. Interpretable Doppler recordings of transmitral flow and pulmonary flow velocity curves were also acquired. At baseline, the patients with Fabry's disease had increased LV septum and posterior wall thickness, normal LV fractional shortening, LV ejection fraction, normal Doppler parameters of mitral inflow but a duration of pulmonary vein flow velocity wave exceeding that of the mitral wave at atrial systole. ERT did not affect heart rate and arterial pressure. LV internal diameters did not change, there was a slight but not significant decrease in the LV posterior wall thickening and a progressive decrease in the interventricular septum thickening (p < 0.025) and in LV mass (p < 0.001) The difference in duration between pulmonary vein flow velocity wave and mitral wave at atrial systole significantly decreased (p < 0.001). These results suggest that ERT in patients with Fabry cardiomyopathy is able to reduce the LV mass and ameliorate the LV stiffness. Copyright 2004 Blackwell Munksgaard

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

PubMed

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

2012-05-01

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

Adaptive pathway development for Fabry disease: a clinical approach.

PubMed

Schuller, Yvonne; Arends, Maarten; Körver, Simon; Langeveld, Mirjam; Hollak, Carla E M

2018-06-01

Fabry disease (FD) is a rare X-chromosome-linked lysosomal storage disorder. Although initial expectations of enzyme replacement therapy (ERT) were high, it is now clear that real-world effectiveness is disappointing and evidence gathering has been inadequate. In retrospect, development of ERT for FD had several shortcomings. Little convincing evidence on the effectiveness existed at time of authorization. Also, post-marketing evaluation failed to generate sufficient and relevant data for adequate evaluation on effectiveness. Adaptive pathways might have benefitted ERT development by: (i) involving healthcare professionals, patients, health technology assessment bodies and payers in the development process; (ii) iterative development, starting with initial authorization in classical males; (iii) a clear real-world data collection plan; (iv) an independent disease registry; and (v) prescription control. Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

Distinct Functional Connectivities Predict Clinical Response with Emotion Regulation Therapy

PubMed Central

Fresco, David M.; Roy, Amy K.; Adelsberg, Samantha; Seeley, Saren; García-Lesy, Emmanuel; Liston, Conor; Mennin, Douglas S.

2017-01-01

Despite the success of available medical and psychosocial treatments, a sizable subgroup of individuals with commonly co-occurring disorders, generalized anxiety disorder (GAD) and major depressive disorder (MDD), fail to make sufficient treatment gains thereby prolonging their deficits in life functioning and satisfaction. Clinically, these patients often display temperamental features reflecting heightened sensitivity to underlying motivational systems related to threat/safety and reward/loss (e.g., somatic anxiety) as well as inordinate negative self-referential processing (e.g., worry, rumination). This profile may reflect disruption in two important neural networks associated with emotional/motivational salience (e.g., salience network) and self-referentiality (e.g., default network, DN). Emotion Regulation Therapy (ERT) was developed to target this hypothesized profile and its neurobehavioral markers. In the present study, 22 GAD patients (with and without MDD) completed resting state MRI scans before receiving 16 sessions of ERT. To test study these hypotheses, we examined the associations between baseline patterns of intrinsic functional connectivity (iFC) of the insula and of hubs within the DN (anterior and dorsal medial prefrontal cortex [MPFC] and posterior cingulate cortex [PCC]) and treatment-related changes in worry, somatic anxiety symptoms and decentering. Results suggest that greater treatment linked reductions in worry were associated with iFC clusters in both the insular and parietal cortices. Greater treatment linked gains in decentering, a metacognitive process that involves the capacity to observe items that arise in the mind with healthy psychological distance that is targeted by ERT, was associated with iFC clusters in the anterior and posterior DN. The current study adds to the growing body of research implicating disruptions in the default and salience networks as promising targets of treatment for GAD with and without co-occurring MDD. PMID:28316567

Impact of enzyme replacement therapy and hematopoietic stem cell transplantation in patients with Morquio A syndrome

PubMed Central

Tomatsu, Shunji; Sawamoto, Kazuki; Alméciga-Díaz, Carlos J; Shimada, Tsutomu; Bober, Michael B; Chinen, Yasutsugu; Yabe, Hiromasa; Montaño, Adriana M; Giugliani, Roberto; Kubaski, Francyne; Yasuda, Eriko; Rodríguez-López, Alexander; Espejo-Mojica, Angela J; Sánchez, Oscar F; Mason, Robert W; Barrera, Luis A; Mackenzie, William G; Orii, Tadao

2015-01-01

Patients with mucopolysaccharidosis IVA (MPS IVA) can present with systemic skeletal dysplasia, leading to a need for multiple orthopedic surgical procedures, and often become wheelchair bound in their teenage years. Studies on patients with MPS IVA treated by enzyme replacement therapy (ERT) showed a sharp reduction on urinary keratan sulfate, but only modest improvement based on a 6-minute walk test and no significant improvement on a 3-minute climb-up test and lung function test compared with the placebo group, at least in the short-term. Surgical remnants from ERT-treated patients did not show reduction of storage materials in chondrocytes. The impact of ERT on bone lesions in patients with MPS IVA remains limited. ERT seems to be enhanced in a mouse model of MPS IVA by a novel form of the enzyme tagged with a bone-targeting moiety. The tagged enzyme remained in the circulation much longer than untagged native enzyme and was delivered to and retained in bone. Three-month-old MPS IVA mice treated with 23 weekly infusions of tagged enzyme showed marked clearance of the storage materials in bone, bone marrow, and heart valves. When treatment was initiated at birth, reduction of storage materials in tissues was even greater. These findings indicate that specific targeting of the enzyme to bone at an early stage may improve efficacy of ERT for MPS IVA. Recombinant N-acetylgalactosamine-6-sulfate sulfatase (GALNS) in Escherichia coli BL21 (DE3) (erGALNS) and in the methylotrophic yeast Pichia pastoris (prGALNS) has been produced as an alternative to the conventional production in Chinese hamster ovary cells. Recombinant GALNS produced in microorganisms may help to reduce the high cost of ERT and the introduction of modifications to enhance targeting. Although only a limited number of patients with MPS IVA have been treated with hematopoietic stem cell transplantation (HSCT), beneficial effects have been reported. A wheelchair-bound patient with a severe form of MPS IVA was treated with HSCT at 15 years of age and followed up for 10 years. Radiographs showed that the figures of major and minor trochanter appeared. Loud snoring and apnea disappeared. In all, 1 year after bone marrow transplantation, bone mineral density at L2–L4 was increased from 0.372 g/cm2 to 0.548 g/cm2 and was maintained at a level of 0.48±0.054 for the following 9 years. Pulmonary vital capacity increased approximately 20% from a baseline of 1.08 L to around 1.31 L over the first 2 years and was maintained thereafter. Activity of daily living was improved similar to the normal control group. After bilateral osteotomies, a patient can walk over 400 m using hip–knee–ankle–foot orthoses. This long-term observation of a patient shows that this treatment can produce clinical improvements although bone deformity remained unchanged. In conclusion, ERT is a therapeutic option for MPS IVA patients, and there are some indications that HSCT may be an alternative to treat this disease. However, as neither seems to be a curative therapy, at least for the skeletal dysplasia in MPS IVA patients, new approaches are investigated to enhance efficacy and reduce costs to benefit MPS IVA patients. PMID:25897204

Modelling the resource implications of managing adults with Fabry disease in Italy.

PubMed

Guest, Julian F; Concolino, Daniela; Di Vito, Raffaele; Feliciani, Claudio; Parini, Rossella; Zampetti, Anna

2011-07-01

This study estimated the resource implications and budget impact of managing adults with Fabry disease in Italy, from the perspective of the Servizio Sanitario Nazionale (SSN). A decision model was constructed using published clinical outcomes and clinician-derived resource utilisation estimates depicting the management of adults with Fabry disease in Italy. The expected annual cost of managing 220 existing and 20 new Fabry patients in Italy was estimated to be €28·3 million. In an average year, patients receiving enzyme replacement therapy (ERT) with 0·2 mg kg(-1) agalsidase alfa (Replagal; Shire Human Genetic Therapies, Basingstoke, Hampshire, UK) or 1·0 mg kg(-1) agalsidase beta (Fabrazyme; Genzyme Europe BV, Naarden, The Netherlands) are collectively expected to make 4500 hospital attendances to a day ward for infusions, which equates to 2000 eight-h days on the day ward associated with ERT. If all ERT-treated patients received their infusions at home, there would be a marginal reduction in the annual health care cost to manage these patients, and the total annual number of days on the day ward associated with ERT in the second year could potentially be reduced from a mean 2000 to zero, thereby releasing substantial hospital resources for use by non-Fabry patients. Currently, only agalsidase alfa is licensed for home treatment in Italy; hence, only patients receiving this enzyme could be offered home treatment. Use of agalsidase alfa (0·2 mg kg(-1) ) instead of agalsidase beta (1·0 mg kg(-1)) has the potential to reduce health care costs and release hospital resources in different specialities for alternative use by non-Fabry patients, thereby improving the efficiency of the public health care system in Italy. © 2011 The Authors. European Journal of Clinical Investigation © 2011 Stichting European Society for Clinical Investigation Journal Foundation.

Therapies for the bone in mucopolysaccharidoses

PubMed Central

Tomatsu, Shunji; Alméciga-Díaz, Carlos J.; Montaño, Adriana M.; Yabe, Hiromasa; Tanaka, Akemi; Dung, Vu Chi; Giugliani, Roberto; Kubaski, Francyne; Mason, Robert W.; Yasuda, Eriko; Sawamoto, Kazuki; Mackenzie, William; Suzuki, Yasuyuki; Orii, Kenji E.; Barrera, Luis A.; Sly, William S.; Orii, Tadao

2014-01-01

Patients with mucopolysaccharidoses (MPS) have accumulation of glycosaminoglycans in multiple tissues which may cause coarse facial features, mental retardation, recurrent ear and nose infections, inguinal and umbilical hernias, hepatosplenomegaly, and skeletal deformities. Clinical features related to bone lesions may include marked short stature, cervical stenosis, pectus carinatum, small lungs, joint rigidity (but laxity for MPS IV), kyphoscoliosis, lumbar gibbus, and genu valgum. Patients with MPS are often wheelchair-bound and physical handicaps increase with age as a result of progressive skeletal dysplasia, abnormal joint mobility, and osteoarthritis, leading to 1) stenosis of the upper cervical region, 2) restrictive small lung, 3) hip dysplasia, 4) restriction of joint movement, and 5) surgical complications. Patients often need multiple orthopedic procedures including cervical decompression and fusion, carpal tunnel release, hip reconstruction and replacement, and femoral or tibial osteotomy through their lifetime. Current measures to intervene in bone disease progression are not perfect and palliative, and improved therapies are urgently required. Enzyme replacement therapy (ERT), hematopoietic stem cell transplantation (HSCT), and gene therapy are available or in development for some types of MPS. Delivery of sufficient enzyme to bone, especially avascular cartilage, to prevent or ameliorate the devastating skeletal dysplasias remains an unmet challenge. The use of an anti-inflammatory drug is also under clinical study. Therapies should start at a very early stage prior to irreversible bone lesion, and damage since the severity of skeletal dysplasia is associated with level of activity during daily life. This review illustrates a current overview of therapies and their impact for bone lesions in MPS including ERT, HSCT, gene therapy, and anti-inflammatory drugs. PMID:25537451

Significant improvement in Fabry disease podocytopathy after 3 years of treatment with agalsidase beta.

PubMed

Ito, Shuichi; Ogura, Masao; Kamei, Koichi; Matsuoka, Kentaro; Warnock, David G

2016-08-01

Fabry disease is an X-linked lysosomal disorder caused by decreased activity of α-galactosidase A (GLA). Consequent accumulation of globotriaosylceramide (GL-3) in lysosomes results in damage to a variety of organs, including the kidneys. Enzyme replacement therapy (ERT) is an effective treatment, but whether it should be started before organ damage is evident is a matter of debate. A 10-year-old boy who complained of severe sole pain for 3 years had been misdiagnosed with juvenile idiopathic arthritis. Further investigations revealed decreased GLA activity and a M1T mutation in the GLA gene causing protein truncation, suggestive of Fabry disease. Despite normal renal function and urinalysis, renal biopsy showed abnormal structure, with marked accumulation of GL-3 in podocytes, partial effacement of foot processes and irregularly reduced expression of nephrin in the slit diaphragm. After 1 year of ERT with 1 mg/kg agalsidase beta once every 2 weeks, his pain had resolved with ERT combined with carbamazepine and pregabalin. After 3 years of the ERT, repeat biopsy showed little renal GL-3 deposition, resolution of foot process effacement, and a dramatic improvement in nephrin expression. There may be a window of opportunity in which pain and renal injury can be addressed in the early stages of Fabry disease. Early initiation of ERT should therefore be considered for children with Fabry disease.

Enzyme replacement therapy in patients with Fabry disease: state of the art and review of the literature.

PubMed

Pisani, Antonio; Visciano, Bianca; Roux, Graciana Diez; Sabbatini, Massimo; Porto, Caterina; Parenti, Giancarlo; Imbriaco, Massimo

2012-11-01

Anderson-Fabry disease is an X-linked lysosomal storage disorder resulting from the deficiency of the hydrolytic enzyme alpha galactosidase A, with consequent accumulation of globotrioasoyl ceramide in cells and tissues of the body, resulting in a multi-system pathology including end organ failure. In the classical phenotype, cardiac failure, renal failure and stroke result in a reduced median life expectancy. The current causal treatment for Fabry disease is the enzyme replacement therapy (ERT): two different products, Replagal (agalsidase alfa) and Fabrazyme (agalsidase beta), have been commercially available in Europe for almost 10 years and they are both indicated for long-term treatment. In fact, clinical trials, observational studies and registry data have provided many evidences for safety and efficacy of ERT in improving symptoms of pain, gastrointestinal disturbances, hypohidrosis, left ventricular mass index, glomerular filtration rate and quality of life. Few data are available on comparison of the two treatments and on the clinical course of the disease. This article reviews the published evidence for clinical efficacy of the two available enzyme preparations. Copyright © 2012 Elsevier Inc. All rights reserved.

Towards an operational ERTS - requirements for implementing cartographic applications of an operational ERTS type satellite

NASA Technical Reports Server (NTRS)

Colvocoresses, A. P.

1974-01-01

After nearly 18 months of successful operation of the first Earth Resources Technology Satellite (ERTS-1), a careful look at the future in order. Judging from the results of ERTS-1 experiments, public sales of ERTS-1 products and overall worldwide response it is believed that ERTS-1 has demonstrated an earth sensing mode that should become operational. It is recognized that several studies leading to the definition of an operational ERTS have been made. However cartographic requirements are generally more basic and demanding than those of the earth science disciplines and are therefore treated separately in this report. One assumption made is that the configuration of ERTS, particularly with respect to the multispectral scanner and data transmission rates cannot be materially altered.

Current therapies for Morquio A syndrome and their clinical outcomes

PubMed Central

Sawamoto, Kazuki; Suzuki, Yasuyuki; Mackenzie, William G.; Theroux, Mary C.; Pizarro, Christian; Yabe, Hiromasa; Orii, Kenji E.; Mason, Robert W.; Orii, Tadao; Tomatsu, Shunji

2016-01-01

Introduction Morquio A syndrome is characterized by a unique skeletal dysplasia, leading to short neck and trunk, pectus carinatum, laxity of joints, kyphoscoliosis, and tracheal obstruction. Cervical spinal cord compression/inability, a restrictive and obstructive airway, and/or bone deformity and imbalance of growth, are life-threatening to Morquio A patients, leading to a high morbidity and mortality. It is critical to review the current therapeutic approaches with respect to their efficacy and limitations. Areas covered Patients with progressive skeletal dysplasia often need to undergo orthopedic surgical interventions in the first two decades of life. Recently, we have treated four patients with a new surgery to correct progressive tracheal obstruction. Enzyme replacement therapy (ERT) has been approved clinically. Cell-based therapies such as hematopoietic stem cell therapy (HSCT) and gene therapy are typically one-time, permanent treatments for enzyme deficiencies. We report here on four Morquio A patients treated with HSCT approved in Japan and followed for at least ten years after treatment. Gene therapy is under investigation on mouse models but not yet available as a therapeutic option. Expert opinion ERT and HSCT in combination with surgical intervention(s) are a therapeutic option for Morquio A; however, the approach for bone and cartilage lesion remains an unmet challenge. PMID:28217429

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

PubMed

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.

Fibrosis: a key feature of Fabry disease with potential therapeutic implications

PubMed Central

2013-01-01

Fabry disease is a rare X-linked hereditary disease caused by mutations in the AGAL gene encoding the lysosomal enzyme alpha-galactosidase A. Enzyme replacement therapy (ERT) is the current cornerstone of Fabry disease management. Involvement of kidney, heart and the central nervous system shortens life span, and fibrosis of these organs is a hallmark of the disease. Fibrosis was initially thought to result from tissue ischemia secondary to endothelial accumulation of glycosphingolipids in the microvasculature. However, despite ready clearance of endothelial deposits, ERT is less effective in patients who have already developed fibrosis. Several potential explanations of this clinical observation may impact on the future management of Fabry disease. Alternative molecular pathways linking glycosphingolipids and fibrosis may be operative; tissue injury may recruit secondary molecular mediators of fibrosis that are unresponsive to ERT, or fibrosis may represent irreversible tissue injury that limits the therapeutic response to ERT. We provide an overview of Fabry disease, with a focus on the assessment of fibrosis, the clinical consequences of fibrosis, and recent advances in understanding the cellular and molecular mechanisms of fibrosis that may suggest novel therapeutic approaches to Fabry disease. PMID:23915644

Sea truth and environmental characterization studies of Mobile Bay, Alabama, utilizing ERTS-1, data collection platforms

NASA Technical Reports Server (NTRS)

Schroeder, W. W.

1977-01-01

The paper reports on the scientific results obtained during a feasibility study that evaluated the potential of using ERTS data collection platforms (DCPs) in the coastal environment of Mobile Bay, Alabama. The utility of instrumented buoys operated in a coastal marine environment as ERTS DCPs is demonstrated. It is shown that these platforms are capable of providing both sea-truth data for ERTS imagery studies and time-series data for event monitoring and/or environmental characterization studies.

Isolation of Novel CreERT2-Driver Lines in Zebrafish Using an Unbiased Gene Trap Approach

PubMed Central

Jungke, Peggy; Hammer, Juliane; Hans, Stefan; Brand, Michael

2015-01-01

Gene manipulation using the Cre/loxP-recombinase system has been successfully employed in zebrafish to study gene functions and lineage relationships. Recently, gene trapping approaches have been applied to produce large collections of transgenic fish expressing conditional alleles in various tissues. However, the limited number of available cell- and tissue-specific Cre/CreERT2-driver lines still constrains widespread application in this model organism. To enlarge the pool of existing CreERT2-driver lines, we performed a genome-wide gene trap screen using a Tol2-based mCherry-T2a-CreERT2 (mCT2aC) gene trap vector. This cassette consists of a splice acceptor and a mCherry-tagged variant of CreERT2 which enables simultaneous labeling of the trapping event, as well as CreERT2 expression from the endogenous promoter. Using this strategy, we generated 27 novel functional CreERT2-driver lines expressing in a cell- and tissue-specific manner during development and adulthood. This study summarizes the analysis of the generated CreERT2-driver lines with respect to functionality, expression, integration, as well as associated phenotypes. Our results significantly enlarge the existing pool of CreERT2-driver lines in zebrafish and combined with Cre–dependent effector lines, the new CreERT2-driver lines will be important tools to manipulate the zebrafish genome. PMID:26083735

Application of ERTS imagery to environmental studies of Lake Champlain

NASA Technical Reports Server (NTRS)

Lind, A. O.

1974-01-01

ERTS Imagery has provided data relating to a number of environmental and limnological concerns such as water quality, lake flooding and lake ice formation. Pollution plume data provided by ERTS was recently used in the Supreme Court case involving the States of Vermont and New York and a paper company. Flooding of lowland tracts has been a major concern due to a repetitive pattern of high lake levels over the past three years, and ERTS imagery is being used to construct the first series of flood maps of the affected areas. Lake ice development and turbidity patterns have also been studied from ERTS, since these have significance for shore erosion studies.

Estrogen replacement, vascular distensibility, and blood pressures in postmenopausal women.

PubMed

De Meersman, R E; Zion, A S; Giardina, E G; Weir, J P; Lieberman, J S; Downey, J A

1998-05-01

The pathogenesis of blood pressure (BP) rise in aging women remains unexplained, and one of the many incriminating factors may include abnormalities in arteriolar resistance vessels. The aim of this study was to determine the effects of unopposed estrogen on arteriolar distensibility, baroreceptor sensitivity (BRS), BP changes, and rate-pressure product (RPP). We tested the hypotheses that estrogen replacement therapy (ERT) enhances arteriolar distensibility and ameliorates BRS, which leads to decreases in BP and RPP. Postmenopausal women participated in a single-blind crossover study; the participants of this study, after baseline measurements, were randomly assigned to receive estrogen (ERT) or a drug-free treatment with a 6-wk washout period between treatments. The single-blind design was instituted because subjects become unblinded due to physiological changes (i.e., fluid shifts, weight gain, and secretory changes) associated with estrogen intake. However, investigators and technicians involved in data collection and analyses remained blind. After each treatment, subjects performed identical autonomic tests, during which electrocardiograms, beat-by-beat BPs, and respiration were recorded. The area under the dicrotic notch of the BP wave was used as an index of arteriolar distensibility. The magnitude of the reflex bradycardia after a precipitous rise in BP was used to determine BRS. Power spectral analysis of heart rate variability was used to assess autonomic activity. BPs were recorded from resistance vessels in the finger using a beat-by-beat photoplethysmographic device. RPP, a noninvasive marker of myocardial oxygen consumption, was calculated. Repeated-measures analyses of variance revealed a significantly enhanced arteriolar distensibility and BRS after ERT (P < 0.05). A trend of a lower sympathovagal balance at rest was observed after ERT, however, this trend did not reach statistical significance (P = 0.061) compared with the other treatments. The above autonomic changes produced significantly lower systolic and diastolic BP changes and RPPs (P < 0.05) at rest and during isometric exercise. We conclude that short-term unopposed ERT favorably enhances arteriolar distensibility, BRS, and hemodynamic parameters in postmenopausal women. These findings have clinical implications in the goals for treating cardiovascular risk factors in aging women.

The management of Gaucher disease in developing countries: a successful experience in Southern Brazil.

PubMed

Krug, B C; Schwartz, I V; Lopes de Oliveira, F; Alegra, T; Campos Martins, N L; Todeschini, L A; Picon, P D

2010-01-01

Gaucher disease (GD) is a genetic disease caused by glucocerebrosidase deficiency. GD is treated by enzyme replacement therapy (ERT) with imiglucerase, a high-cost drug provided by the Brazilian Ministry of Health (BMH). This study reports the implementation of the BMH guidelines for GD in the southernmost state of the country. We review the clinical and laboratorial data for patients seen at the reference center for GD from Rio Grande do Sul, Brazil (July 2003 to June 2006). Twenty-five patients were included in this study. At baseline, 19/20 were on ERT (mean dosage of imiglucerase = 51.8 U/kg/infusion), 3/17 presented anemia, and 5/16 thrombocytopenia. The amount of imiglucerase prescribed to these patients was adjusted according to the guidelines in July 2003; out of them, 18 were receiving ERT in the reference center at month 36 (mean dosage of imiglucerase = 27.5 U/kg/infusion), 2/18 presented anemia, and 4/18 presented thrombocytopenia. The analysis of the liver, spleen, and bone data presented some limitations, but the available information suggests that patients did not deteriorate. GD patients who initiated ERT after July 2003 (n = 5) received lower dosage of imiglucerase since the beginning of the treatment; most of them demonstrated clinical and laboratorial response. From baseline to month 36, the consumption of imiglucerase by the reference center showed a significant reduction, which represented savings of USD 3 million to the public health system. The model of care of GD patients suggested by the BMH guidelines appears to be cost-effective and could be an example for management of rare diseases in underdeveloped countries. Copyright © 2009 S. Karger AG, Basel.

Cardiovascular testing in Fabry disease: exercise capacity reduction, chronotropic incompetence and improved anaerobic threshold after enzyme replacement.

PubMed

Lobo, T; Morgan, J; Bjorksten, A; Nicholls, K; Grigg, L; Centra, E; Becker, G

2008-06-01

The aim of this study was to document exercise capacity and serial electrocardiogram and echocardiograph findings in a cohort of Australian patients with Fabry disease, in relation to their history of enzyme replacement therapy (ERT). Fabry disease has multifactorial effects on the cardiovascular system. Most previous studies have focused on electrocardiographic and echocardiographic parameters. Exercise capacity can be used as an integrated measure of cardiovascular function and allows the effects of treatment to be monitored. A total of 38 patients (30 men and 8 women) with Fabry disease were monitored by 12-lead electrocardiograms every 6-12 months, and by annual standardized-protocol echocardiograms. Bicycle stress tests with VO(2) max measurement and once-only 6 minutes' walk tests were also carried out in subsets of patients whose general health status allowed testing. Seventy per cent of patients met electrocardiogram criteria for left ventricular hypertrophy. Left ventricular hypertrophy on echocardiograph was present in 64% of patients (80% of men). Exercise capacity was reduced in patients with Fabry disease compared with that predicted from normative population data. Mild improvement in anaerobic threshold was seen in the first year of ERT (14.1 +/- 3.0 to 15.8 +/- 3.0, P = 0.02), but no consistent further increase was seen beyond the first year. Most patients had resting bradycardia, with impaired ability to increase heart rate during exercise. Serial testing on ERT showed an improvement in anaerobic threshold but no significant change in VO(2) max. Male patients with Fabry disease were unable to attain predicted maximal heart rate on exercise or to achieve normal exercise levels. ERT was associated with a small improvement in anaerobic threshold over the first year.

Prevention of Lethal Murine Hypophosphatasia by Neonatal Ex Vivo Gene Therapy Using Lentivirally Transduced Bone Marrow Cells.

PubMed

Iijima, Osamu; Miyake, Koichi; Watanabe, Atsushi; Miyake, Noriko; Igarashi, Tsutomu; Kanokoda, Chizu; Nakamura-Takahashi, Aki; Kinoshita, Hideaki; Noguchi, Taku; Abe, Shinichi; Narisawa, Sonoko; Millán, José Luis; Okada, Takashi; Shimada, Takashi

2015-12-01

Hypophosphatasia (HPP) is an inherited skeletal and dental disease caused by loss-of-function mutations in the gene that encodes tissue-nonspecific alkaline phosphatase (TNALP). The major symptoms of severe forms of the disease are bone defects, respiratory insufficiency, and epileptic seizures. In 2015, enzyme replacement therapy (ERT) using recombinant bone-targeted TNALP with deca-aspartate (D10) motif was approved to treat pediatric HPP patients in Japan, Canada, and Europe. However, the ERT requires repeated subcutaneous administration of the enzyme because of the short half-life in serum. In the present study, we evaluated the feasibility of neonatal ex vivo gene therapy in TNALP knockout (Akp2(-/-)) HPP mice using lentivirally transduced bone marrow cells (BMC) expressing bone-targeted TNALP in which a D10 sequence was linked to the C-terminus of soluble TNALP (TNALP-D10). The Akp2(-/-) mice usually die within 20 days because of growth failure, epileptic seizures, and hypomineralization. However, an intravenous transplantation of BMC expressing TNALP-D10 (ALP-BMC) into neonatal Akp2(-/-) mice prolonged survival of the mice with improved bone mineralization compared with untransduced BMC-transplanted Akp2(-/-) mice. The treated Akp2(-/-) mice were normal in appearance and experienced no seizures during the experimental period. The lentivirally transduced BMC were efficiently engrafted in the recipient mice and supplied TNALP-D10 continuously at a therapeutic level for at least 3 months. Moreover, TNALP-D10 overexpression did not affect multilineage reconstitution in the recipient mice. The plasma ALP activity was sustained at high levels in the treated mice, and tissue ALP activity was selectively detected on bone surfaces, not in the kidneys or other organs. No ectopic calcification was observed in the ALP-BMC-treated mice. These results indicate that lentivirally transduced BMC can serve as a reservoir for stem cell-based ERT to rescue the Akp2(-/-) phenotype. Neonatal ex vivo gene therapy thus appears to be a possible treatment option for treating severe HPP.

Prevention of Lethal Murine Hypophosphatasia by Neonatal Ex Vivo Gene Therapy Using Lentivirally Transduced Bone Marrow Cells

PubMed Central

Iijima, Osamu; Miyake, Koichi; Watanabe, Atsushi; Miyake, Noriko; Igarashi, Tsutomu; Kanokoda, Chizu; Nakamura-Takahashi, Aki; Kinoshita, Hideaki; Noguchi, Taku; Abe, Shinichi; Narisawa, Sonoko; Millán, José Luis; Okada, Takashi; Shimada, Takashi

2015-01-01

Hypophosphatasia (HPP) is an inherited skeletal and dental disease caused by loss-of-function mutations in the gene that encodes tissue-nonspecific alkaline phosphatase (TNALP). The major symptoms of severe forms of the disease are bone defects, respiratory insufficiency, and epileptic seizures. In 2015, enzyme replacement therapy (ERT) using recombinant bone-targeted TNALP with deca-aspartate (D10) motif was approved to treat pediatric HPP patients in Japan, Canada, and Europe. However, the ERT requires repeated subcutaneous administration of the enzyme because of the short half-life in serum. In the present study, we evaluated the feasibility of neonatal ex vivo gene therapy in TNALP knockout (Akp2−/−) HPP mice using lentivirally transduced bone marrow cells (BMC) expressing bone-targeted TNALP in which a D10 sequence was linked to the C-terminus of soluble TNALP (TNALP-D10). The Akp2−/− mice usually die within 20 days because of growth failure, epileptic seizures, and hypomineralization. However, an intravenous transplantation of BMC expressing TNALP-D10 (ALP-BMC) into neonatal Akp2−/− mice prolonged survival of the mice with improved bone mineralization compared with untransduced BMC-transplanted Akp2−/− mice. The treated Akp2−/− mice were normal in appearance and experienced no seizures during the experimental period. The lentivirally transduced BMC were efficiently engrafted in the recipient mice and supplied TNALP-D10 continuously at a therapeutic level for at least 3 months. Moreover, TNALP-D10 overexpression did not affect multilineage reconstitution in the recipient mice. The plasma ALP activity was sustained at high levels in the treated mice, and tissue ALP activity was selectively detected on bone surfaces, not in the kidneys or other organs. No ectopic calcification was observed in the ALP-BMC-treated mice. These results indicate that lentivirally transduced BMC can serve as a reservoir for stem cell-based ERT to rescue the Akp2−/− phenotype. Neonatal ex vivo gene therapy thus appears to be a possible treatment option for treating severe HPP. PMID:26467745

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

PubMed

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.

A multicenter, open-label extension study of velaglucerase alfa in Japanese patients with Gaucher disease: Results after a cumulative treatment period of 24months.

PubMed

Ida, Hiroyuki; Tanaka, Akemi; Matsubayashi, Tomoko; Murayama, Kei; Hongo, Teruaki; Lee, Hak-Myung; Mellgard, Björn

2016-07-01

Enzyme replacement therapy (ERT) with exogenous glucocerebrosidase is indicated to treat symptomatic Gaucher disease (GD), a rare, inherited metabolic disorder. ERT with velaglucerase alfa, which is produced in a human cell line using gene activation technology, was studied in a 12-month phase III trial in Japanese patients with type 1 or 3 GD who were switched from imiglucerase ERT (n=6); the current, open-label, 12-month extension study was designed to assess longer-term safety and efficacy. Two adult and three pediatric patients (aged <18years) were enrolled into the extension study. Every-other-week intravenous infusions were administered for 63-78weeks at average doses between 51.5 and 60.7units/kg. Three non-serious adverse events were considered related to velaglucerase alfa treatment, but no patient discontinued from the study. Six serious but non-drug-related adverse events were reported. No patient tested positive for anti-velaglucerase alfa antibodies. Hemoglobin concentrations, platelet counts, and liver and spleen volumes (normalized to body weight) in these patients were generally stable over a cumulative 24-month period from the baseline of the parent trial. The data suggest that velaglucerase alfa was well tolerated and maintained clinical stability in Japanese GD patients over 2years after switching from imiglucerase. ClinicalTrials.gov identifier NCT01842841. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

A study of the utilization of ERTS-1 data from the Wabash River Basin

NASA Technical Reports Server (NTRS)

Landgrebe, D. A. (Principal Investigator)

1974-01-01

The author has identified the following significant results. The identification and area estimation of crops experiment tested the usefulness of ERTS data for crop survey and produced results indicating that crop statistics could be obtained from ERTS imagery. Soil association mapping results showed that strong relationships exist between ERTS data derived maps and conventional soil maps. Urban land use analysis experiment results indicate potential for accurate gross land use mapping. Water resources mapping demonstrated the feasibility of mapping water bodies using ERTS imagery.

Expensive drugs for rare disorders: to treat or not to treat? The case of enzyme replacement therapy for mucopolysaccharidosis VI.

PubMed

Schlander, M; Beck, M

2009-05-01

Mucopolysaccharidosis VI (MPS VI) is a very rare, chronically debilitating lysosomal storage disorder that develops in people with an enzyme deficiency. Clinical characteristics and progression rates vary widely between patients. The recent introduction of enzyme replacement therapy (ERT) has improved considerably the lives of patients with MPS VI, at an annual cost of treatment between euro 150,000 and euro 450,000 per patient. This Commentary article addresses the controversial topic of granting reimbursement for expensive treatment options for orphan diseases, such as MPS VI. The discussion reflects clinical, economic and ethical aspects and incorporates insights from the relevant literature (based on a Medline search to September 2008) on MPS VI, efficacy of ERT, orphan drugs, and the economics and ethics of health-care prioritisation. Although ERT for MPS VI received marketing authorisation in the European Union in January 2006, patients' access to this therapy varies geographically due to differences between national reimbursement schemes for orphan drugs. Some inclusion and exclusion criteria for treatment of MPS VI patients with ERT appear arbitrary and may contribute to the exclusion from treatment of patients who could benefit in the long term. Reimbursement schemes which rely on proof of short-term treatment effectiveness may discriminate against slowly progressive patients, as health gain can often not be confirmed over a short period of time in these patients. Conventional cost-effectiveness analysis remains silent on crucial issues related to budgetary impact, i.e. opportunity cost from a system perspective, and fair access to treatment. To prevent patients from being deprived of effective treatment, it is suggested that inclusion and exclusion criteria for treatment should be primarily based on a careful individual assessment of expected long-term clinical benefits. Once treatment has been agreed to as the correct option on clinical grounds, it is further argued that the conventional cost-effectiveness criterion currently in widespread use does not offer a sufficient basis for rejecting reimbursement of expensive treatments for exceptionally rare disorders, providing that decisions on reimbursement are intended to reflect public preferences.

A study on the erosion of Niigata Beach from ERTS-A imagery

NASA Technical Reports Server (NTRS)

Maruyasu, T.

1973-01-01

Coastal erosion of Niigata Beach, Japan as a result of construction works is discussed. The application of ERTS-1 imagery for defining and monitoring the extent of the erosion is described. The contribution of ERTS-1 data to studies leading to effective erosion control methods are reported.

[The switch of enzyme therapy in Fabry disease].

PubMed

Riccio, Eleonora; Pisani, Antonio

2014-01-01

Fabry disease (FD) is a multiorgan X-linked lysosomal storage disorder resulted from the deficiency of the lysosomal enzyme alpha galactosidase A. It particularly affects the heart, kidneys and cerebrovascular system. The treatment options for FD patients include long-term enzyme replacement therapy (ERT). Two recombinant enzyme formulations for the ERT of FD are available on European market: agalsidase alfa and agalsidase beta. Numerous evidences in the literature have confirmed the safety and efficacy of ERT. However, to date, there have been limited comparisons between the two agents, and no firm conclusion can be drawn regarding their specific efficacy and safety. In June 2009, a viral contamination in the manufacturing process of Fabrazyme led to a global shortage of agalsidase beta. Recommendations to shift patients to the recommended dose of Replagal were published by the European Medicines Agency (EMA) for all patients receiving Fabrazyme. This offered the unique opportunity to compare, although indirectly, the two drugs evaluating any clinical modification or adverse events that occurred after the switch. Moreover, with the increased availability of agalsidase beta in the last 3 months of 2012, some of the patients who previously switched to agalsidase alfa, were switched-back and returned to full-dose agalsidase beta. This article reviews the published evidence for the clinical efficacy of the two available enzyme preparations and compare it with the experience of our center.

ADA Deficiency: Evaluation of the Clinical and Laboratory Features and the Outcome.

PubMed

Cagdas, Deniz; Gur Cetinkaya, Pınar; Karaatmaca, Betül; Esenboga, Saliha; Tan, Cagman; Yılmaz, Togay; Gümüş, Ersin; Barış, Safa; Kuşkonmaz, Barış; Ozgur, Tuba Turul; Bali, Pawan; Santisteban, Ines; Orhan, Diclehan; Yüce, Aysel; Cetinkaya, Duygu; Boztug, Kaan; Hershfield, Michael; Sanal, Ozden; Tezcan, İlhan

2018-05-09

Adenosine deaminase (ADA) deficiency is an autosomal recessive primary immunodeficiency. It results in the intracellular accumulation of toxic metabolites which have effects particularly on lymphocytes and the brain. The aim of this study was to evaluate the outcome of 13 ADA-deficient patients. We planned to evaluate their clinical and laboratory findings before and after enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplantation (aHSCT), and hematopoietic stem cell gene therapy (HSCGT). Measurement of ADA enzyme activity and metabolites and sequencing of the ADA gene were performed in most of the patients with ADA deficiency. One of the patients with late-onset ADA deficiency was diagnosed by the help of primary immunodeficiency panel screening. Ten out of 13 patients were diagnosed as SCID, while 3 out of 13 were diagnosed as delayed-/late-onset ADA deficiency. Late-onset ADA deficiency patients had clinical and laboratory findings of combined immunodeficiency (CID). Eight patients with ADA-SCID were found to have higher levels of ADA metabolite (dAXP%) (62.1% (34.6-71.9)) than 3 patients with delayed-/late-onset ADA deficiency (6.9% (2.1-8.9). All but one patient with SCID had T-B-NK- phenotype, one had T-B-NK+ phenotype. Genetic defect was documented in 11 patients. Four out of 11 patients had compound heterozygous defects. Three out of 4 patients with compound heterozygous defects had delayed-onset/late-onset ADA deficiency. Seven out of 11 patients with SCID had homozygous defects. Five out of 7 had the same homozygous indel frameshift mutation (c.955-959delGAAGA) showing a founder effect. There were two novel splice site defects: one (IVS10+2T>C) was heterozygous in a patient with late-onset ADA deficiency, and the other was homozygous (IVS2delT+2) in a SCID patient. Other defects were missense defects. Nine out of 13 patients were put on pegylated ADA ERT. Four out of six patients were transplanted without using a conditioning regimen. HSCGT was performed to one of the patients. The genetic diagnosis of SCID is utmost important. There is a chance to give ERT before the definitive therapy if the patient with SCID/CID has ADA deficiency. Although ERT was insufficient to restore a normal immune function in ADA-SCID patients, it was useful to improve and stabilize the clinical status before curative therapy (aHSCT/HSCGT). Enzyme replacement therapy was successful in patients with late-/delayed-onset ADA deficiency who presented with the features of combined immunodeficiency. Gastrointestinal polyposis in a patient with late-onset ADA deficiency may be an association or a coincidental finding. Intermittent neurodevelopmental evaluation especially for hearing impairment should be performed in most of the ADA-deficient patients. This may alleviate the speech delay and cognitive abnormalities which may be observed in the follow-up.

Delineation of contaminant plume for an inorganic contaminated site using electrical resistivity tomography: comparison with direct-push technique.

PubMed

Liao, Qing; Deng, Yaping; Shi, Xiaoqing; Sun, Yuanyuan; Duan, Weidong; Wu, Jichun

2018-03-03

Precise delineation of contaminant plume distribution is essential for effective remediation of contaminated sites. Traditional in situ investigation methods like direct-push (DP) sampling are accurate, but are usually intrusive and costly. Electrical resistivity tomography (ERT) method, as a non-invasive geophysical technique to map spatiotemporal changes in resistivity of the subsurface, is becoming increasingly popular in environmental science. However, the resolution of ERT for delineation of contaminant plumes still remains controversial. In this study, ERT and DP technique were both conducted at a real inorganic contaminated site. The reliability of the ERT method was validated by the direct comparisons of their investigation results that the resistivity acquired by ERT method is in accordance with the total dissolved solid concentration in groundwater and the overall variation of the total iron content in soil obtained by DP technique. After testifying the applicability of ERT method for contaminant identification, the extension of contaminant plume at the study site was revealed by supplementary ERT surveys conducted subsequently in the surrounding area of the contaminant source zone.

Contribution of 3-D time-lapse ERT to the study of leachate recirculation in a landfill

DOE Office of Scientific and Technical Information (OSTI.GOV)

Clement, R., E-mail: remi.clement@hmg.inpg.fr; Grenoble Universite, B.P. 53, 38041 Grenoble Cedex 9; Oxarango, L.

2011-03-15

Leachate recirculation is a key process in the operation of municipal waste landfills as bioreactors. It aims at increasing the moisture content to optimise the biodegradation. Because waste is a very heterogeneous and anisotropic porous media, the geometry of the leachate plume recirculation is difficult to delineate from the surface at the scale of the bioreactor site. In this study, 3-D time-lapse electrical resistivity tomography (ERT) was used to obtain useful information for understanding leachate recirculation hydrodynamics. The ERT inversion methodology and the electrode arrays were optimised using numerical modelling simulating a 3-D leachate injection scenario. Time-lapse ERT was subsequentlymore » applied at the field scale during an experimental injection. We compared ERT images with injected volumes to evaluate the sensitivity of time-lapse ERT to delineate the plume migration. The results show that time-lapse ERT can accomplish the following: (i) accurately locate the injection plume, delineating its depth and lateral extension; (ii) be used to estimate some hydraulic properties of waste.« less

Enzyme replacement therapy stabilized white matter lesion progression in Fabry disease.

PubMed

Fellgiebel, Andreas; Gartenschläger, Martin; Wildberger, Kerstin; Scheurich, Armin; Desnick, Robert J; Sims, Katherine

2014-01-01

The central nervous system manifestations in Fabry disease (FD) include progressive white matter lesions (WMLs) and stroke. Due to progressive microvascular involvement, men and women with FD over 35 years of age develop WMLs. Moreover, the prevalence of stroke has been estimated to be 12 times higher in FD compared with the general population. Enzyme replacement therapy (ERT) is available and has shown beneficial effects on renal, cardiac, and peripheral nerve function in FD, but the ERT effect on the progression of WMLs, or the reduction in cerebrovascular events, remains unknown. The WML burden and the effect of agalsidase beta 1 mg/kg biweekly on WML progression were assessed longitudinally in a Phase 4 agalsidase-beta placebo-controlled analysis of untreated and treated FD patients with mild-to-moderate renal involvement (serum creatinine measurements of ≥1.2 mg/dl and <3.0 mg/dl). The primary end point was the difference in the number of patients with increased WML burden between the agalsidase beta and placebo groups at the end of treatment. The diameters of the WMLs were determined manually using axial flow-attenuated-inversion-recovery-weighted magnetic resonance imaging (MRI) scans taken at baseline and follow-up. MRI scans from 41 FD patients (mean age 43.9, age range 20-68, 3 females; n=25 on ERT, n=16 on placebo) were analyzed. WML burden was present in 63% of patients at baseline, increased over a mean of 27 months (range 12-33 months) follow-up, and correlated with left ventricular hypertrophy (LVPW). Patients with previous or recent strokes (n=11, 39-68 years) showed an increase in the number of WMLs (p=0.005). A greater proportion of younger patients (≤50 years) on ERT (n=18) had stable WML burden compared with younger patients in the placebo group (n=13): 44% (8 of 18) versus 31% (4 of 13), p=0.014. The number needed to treat was 8. This FD patient cohort, with mild-to-moderate renal involvement, had a significant WML burden and high inter-individual variability associated with the degree of LVPW but not the degree of kidney dysfunction. These advanced patients with increased LVPW and stroke evidence may have had a higher cerebrovascular risk. The WML burden in patients on ERT was more likely to remain stable, compared with patients on placebo. Thus, ERT may reduce the progression of vascular disease, even in advanced FD patients, suggesting that early treatment may stabilize WML progression and stroke risk. © 2014 S. Karger AG, Basel.

Atypical immunologic response in a patient with CRIM-negative Pompe disease

PubMed Central

Abbott, Mary-Alice; Prater, Sean N.; Banugaria, Suhrad G.; Richards, Susan M.; Young, Sarah P.; Rosenberg, Amy S.; Kishnani, Priya S.

2013-01-01

We report the clinical course of a patient with severe infantile onset Pompe disease [cross-reactive immunologic material (CRIM) negative, R854X/R854X] who was diagnosed prenatally and received standard dosing of alglucosidase alfa (Myozyme®) enzyme replacement therapy (ERT) from day 10 of life until she passed away at the age of 3 years 9 months. In the immediate neonatal period there was cardiomegaly on chest X-ray, cardiac hypertrophy by echocardiogram, and development of a wide complex tachycardia. CRIM negative (CN) status was suspected based on her family history, and the available data at the time indicated that CN patients had limited survival even with ERT. However, given the opportunity for very early treatment, the treating provider and family elected to initiate treatment with ERT, without immune modulation. By 9 months of age echocardiogram was normal. Early motor development was within normal limits but by 2 years of age her developmental progress had slowed. She seroconverted by the 4th month of ERT, and anti-rhGAA antibody titers peaked at 25,600 in the 27th month. Immunomodulatory therapy was considered but declined by family. She acquired Influenza A at 2 years 6 months, which led to a prolonged hospitalization with invasive respiratory support, and placement of tracheostomy and gastrostomy tube. Her developmental progress ceased, and she died suddenly at home from a presumed cardiac event at age 3 years 9 months. The poor outcomes observed in CN patients have been attributed to the development of high sustained antibody titers. Although this CN patient’s anti-rhGAA response was elevated and sustained, it is unlike any of the 3 patterns that have been previously described: high titer CN, high titer CRIM positive (HTCP), and low titer CP (LTCP) patients. This patient’s clinical course, with achievement of 24 months of motor gains, 30 months of ventilator-free survival and 45 month survival, is like that of only a fraction of ERT treated CN patients, yet it is identical to other reported CN patients in its relentless progression and early fatality. The immunologic response (moderate sustained antibody titers) described here has not been previously reported and may have played a role in the overall pattern of developmental decline. In light of proposed universal newborn screening for Pompe disease, there is an urgent need for improved understanding of the interplay between immunologic responses to the only available treatment, ERT, and the relentless nature of this disease in CN patients. PMID:21889385

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.

PubMed

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.

Somatic mosaicism due to monoallelic reversion of a mutation in T cells of an ADA-SCID patient and the effects of enzyme replacement therapy on the revertant phenotype

PubMed Central

Moncada-Vélez, Marcela; Vélez-Ortega, Alejandra C.; Orrego, Julio C.; Santisteban, Inés; Jagadeesh, Jayashree; Olivares, Margarita; Olaya, Natalia; Hershfield, Michael S.; Candotti, Fabio; Franco, Jose L.

2011-01-01

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-NK- 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 PBL, as a result of somatic mosaicism due to monoallelic reversion of the causative mutation in the ADA gene. Our patient was not eligible for hematopoietic stem cell transplantation (HSCT) or gene therapy (GT); therefore enzyme replacement therapy (ERT) with bovine PEG-ADA was initiated. 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, with sustained expansion of TCRγδ+ T cells. This was accompanied by 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 tumor 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. PMID:21671975

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

PubMed

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/m(2) (range 43.8-334) vs. CMR median at most recent study 44.5 g/m(2) (range 34-303), p=0.44). In 5 patients with serial CMR scans over time, LVMI decreased in 2, was similar in 2, and increased in 1 patient with high sustained antibodies to exogenous enzyme. Delayed enhancement was noted in only l separate patient who also had high sustained antibodies to exogenous enzyme. CMR is an imaging tool that is feasible to use to serially follow LVMI and EF in children with Pompe disease on ERT. Real-time imaging is adequate for quantification purposes in these patients and minimizes the need for sedation. Quantitative CMR LVMI is generally lower than echo derived LVMI. Delayed enhancement appears to be a rare finding by CMR in Pompe disease. A further follow-up is necessary to better understand the long term effects of ERT in infantile Pompe survivors, especially those with high sustained antibody titers or advanced cardiac disease at treatment outset. Copyright © 2010 Elsevier Inc. All rights reserved.

Use of Cardiac Magnetic Resonance Imaging to Evaluate Cardiac Structure, Function and Fibrosis in Children with Infantile Pompe Disease on Enzyme Replacement Therapy

PubMed Central

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.

2010-01-01

Background 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 better tissue definition and characterization of myocardial fibrosis. However, CMR for Pompe disease is not frequently performed due to the high risk of sedation. We report the first use of CMR in a feasible protocol to quantify left ventricular (LV) mass, function, and presence of myocardial fibrosis in the Pompe population. Methods 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 with CMR by delayed enhancement imaging 5-10 min after gadolinium contrast using single-shot inversion recovery sequences with inversion time set to null the myocardium. Results Seventeen CMR scans were successfully performed in 10 subjects with Pompe disease (median age at first CMR 9 months, range 1-38 months, 80% male), with sedation only performed for 4 studies. There was a median interval of 5 months (range 0-34 months) from start of ERT to first CMR (baseline). At baseline, median indexed LVMI by CMR (140.0 g/m2, range 43.8-334.0) tended to be lower than that assessed by echo (median 204.0 g/m2, 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 not a significant decrease in CMR measured LVMI over time (CMR median LVMI at baseline 94 g/m2 (range 43.8-334) vs. CMR median at most recent study 44.5 g/m2 (range 34-303), p=0.44). In 5 patients with serial CMR scans over time, LVMI decreased in 2, was similar in 2, and increased in 1 patient with high sustained antibodies to exogenous enzyme. Delayed enhancement was noted in only l separate patient who also had high sustained antibodies to exogenous enzyme. Conclusion CMR is a useful imaging tool that is feasible to use to serially follow LVMI and EF in children with Pompe disease on ERT. Real-time imaging is adequate for quantification purposes in these patients and minimizes the need for sedation. Quantitative CMR LVMI is generally lower than echo derived LVMI. Delayed enhancement appears to be a rare finding by CMR in Pompe Disease. Further follow-up is necessary to better understand the long term effects of ERT in infantile Pompe survivors, especially those with high sustained antibody titers or advanced cardiac disease at treatment outset. PMID:20875764

Monitoring CO2 sequestration into deep saline aquifer and associated salt intrusion using coupled multiphase flow modeling and time-lapse electrical resistivity tomography

NASA Astrophysics Data System (ADS)

Lu, C.; Zhang, C.; Huang, H.; Johnson, T.

2012-12-01

Geological sequestration of carbon dioxide (CO2) into the subsurface has been considered as one solution to reduce greenhouse emission to the atmosphere. Successful sequestration process requires efficient and adequate monitoring of injected fluids as they migrate into the aquifer to evaluate flow path, leakage, and geochemical interactions between CO2 and geologic media. In this synthetic field scale study, we have integrated 3D multiphase flow modeling code PFLOTRAN with 3D time-laps electrical resistivity tomography (ERT) to gain insight into the supercritical (SC) CO2 plumes movement in the deep saline aquifer and associated brine intrusion into shallower fresh water aquifer. A parallel ERT forward and inverse modeling package was introduced, and related algorithms are briefly described. The capabilities and limitations of ERT in monitoring CO2 migration are assessed by comparing the results from PFLOTRAN simulations with the ERT inversion results. In general, our study shows the ERT inversion results compare well with PFLOTRAN with reasonable discrepancies, indicating that the ERT can capture the actual CO2 plume dynamics and brine intrusion. Detailed comparisons on the location, size and volume of CO2 plume show the ERT method underestimated area review and overestimated total plume volume in the predictions of SC CO2 movements. These comparisons also show the ERT method constantly overestimate salt intrusion area and underestimated total solute amount in the predictions of brine filtration. Our study shows that together with other geochemical and geophysical methods, ERT is a potentially useful monitoring tool in detecting the SC CO2 and formation fluid migrations.

Use of areal snow cover measurements from ERTS-1 imagery in snowmelt-runoff relationships in Arizona

NASA Technical Reports Server (NTRS)

Aul, J. S.; Ffolliott, P. F.

1975-01-01

Methods of interpreting ERTS-1 imagery to measure areal snow cover were analyzed. Relationship of areal snow cover and runoff were among the objectives in this study of ERTS-1 imagery use for forecasting snowmelt-runoff relationships.

Preliminary results of ERTS-investigations by W-German investigations. [multidisciplinary geoscientific experiments in central Germany and hydrogeology of Argentina Pampas

NASA Technical Reports Server (NTRS)

Muehlfeld, R.

1974-01-01

Results are presented of West German investigations into multidisciplinary geoscientific experiments in central Germany and the Alps, and hydrogeological investigations in the Pampa of Argentina based on ERTS-1 data. The main goals of the investigation were achieved. The studies have given a good idea of the possibilities and limitations of ERTS imagery depending on the objectives in question and on the geographical conditions of the areas under investigation. Even in the well known region of central Europe, ERTS has proven its ability of improving present knowledge. In fields such as pollution monitoring and regional planning the satellite techniques should have distinct practical value. For any regional study of less known areas, the value of ERTS imagery can hardly be overestimated.

Design, baseline characteristics, and early findings of the MPS VI (mucopolysaccharidosis VI) Clinical Surveillance Program (CSP).

PubMed

Hendriksz, Christian J; Giugliani, Roberto; Harmatz, Paul; Lampe, Christina; Martins, Ana Maria; Pastores, Gregory M; Steiner, Robert D; Leão Teles, Elisa; Valayannopoulos, Vassili

2013-03-01

To outline the design, baseline data, and 5-year follow-up data of patients with mucopolysaccharidosis (MPS) VI enrolled in the Clinical Surveillance Program (CSP), a voluntary, multinational, observational program. The MPS VI CSP was opened in 2005 to collect, for at least 15 years, observational data from standard clinical and laboratory assessments of patients with MPS VI. Baseline and follow-up data are documented by participating physicians in electronic case report forms. Between September 2005 and March 2010 the CSP enrolled 132 patients, including 123 who received enzyme replacement therapy (ERT) with galsulfase. Median age at enrolment was 13 years (range 1-59). Mean baseline data showed impaired growth, hepatosplenomegaly, and reduced endurance and pulmonary function. The most common findings were heart valve disease (90%), reduced visual acuity (79%), impaired hearing (59%), and hepatosplenomegaly (54%). Follow-up data up to 5 years in patients with pre- and post-ERT measurements showed a decrease in urinary glycosaminoglycans and increases in height and weight in patients <16 years and suggested reductions in liver and spleen size and improvements in endurance and pulmonary function after ERT was started. Vision, hearing, and cardiac function were unchanged. Safety data were in line with previous reports. The CSP represents the largest cross-sectional study of MPS VI to date. This first report provides information on the design and implementation of the program and population statistics for several clinical variables in patients with MPS VI. Data collected over 5 years suggest that ERT provides clinical benefit and is well-tolerated with no new safety concerns.

Inner ear involvement in Fabry disease: Clinical and audiometric evaluation of a large cohort of patients followed in a reference centre.

PubMed

Rodrigues, Jorge; Azevedo, Olga; Sousa, Nuno; Cunha, Damião; Mexedo, Alexandre; Fonseca, Rui

2018-06-01

Fabry disease (FD) is a lysosomal storage disorder (LSD) that involves the cochleovestibular system. Tinnitus and progressive sensorineural hearing loss are frequent complains. A stabilization of hearing function has been reported with enzyme replacement therapy (ERT). This study aims to characterize the inner ear involvement, identify factors associated to hearing loss and evaluate the effect of ERT on the hearing function of FD patients. We reviewed the clinical records of patients with confirmed diagnosis of FD followed in a Reference Centre on LSD in the North of Portugal. We included a total of 122 patients with a mean age of 47.1 ± 17.6 years and 48.3% males. Hearing loss was reported by 26.2% of the patients and 23.0% mentioned tinnitus. Pure tone audiometry revealed sensorineural hearing loss in 36.9% of the cases. FD patients presented worse age-adjusted hearing thresholds in all analysed frequencies compared to the normal population (p = .001). Patients with hearing loss presented a significantly higher value of microalbuminuria (p = .001) and a higher frequency of acroparesthesias (p = .032). Patients presented a comparable hearing level one year after starting ERT (p = .384). In FD, hearing loss is common and age-matched hearing thresholds by frequency are worse than in the general population. Hearing loss was associated to the presence of acroparesthesias and higher values of microalbuminuria. Hearing loss stabilized in patients under ERT. A careful cochleo-vestibular evaluation should be part of the clinical assessment of FD. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

Enzyme replacement therapy with galsulfase in 34 children younger than five years of age with MPS VI.

PubMed

Horovitz, Dafne D G; Magalhães, Tatiana S P C; Acosta, Angelina; Ribeiro, Erlane M; Giuliani, Liane R; Palhares, Durval B; Kim, Chong A; de Paula, Ana Carolina; Kerstenestzy, Marcelo; Pianovski, Mara A D; Costa, Maria Ione F; Santos, Francisca C; Martins, Ana Maria; Aranda, Carolina S; Correa Neto, Jordão; Holanda, Gervina Brady Moreira; Cardoso, Laércio; da Silva, Carlos A B; Bonatti, Renata C F; Ribeiro, Bethania F R; Rodrigues, Maria do Carmo S; Llerena, Juan C

2013-05-01

Mucopolysaccharidosis type VI (MPS VI) is a progressive, chronic and multisystem lysosomal storage disease with a wide disease spectrum. Clinical and biochemical improvements have been reported for MPS VI patients on enzyme replacement therapy (ERT) with rhASB (recombinant human arylsulfatase B; galsulfase, Naglazyme®, BioMarin Pharmaceutical Inc.), making early diagnosis and intervention imperative for optimal patient outcomes. Few studies have included children younger than five years of age. This report describes 34 MPS VI patients that started treatment with galsulfase before five years of age. Data from patients who initiated treatment at <5 years of age were collected from patients' medical records. Baseline and follow-up assessments of common symptoms that led to diagnosis and that were used to evaluate disease progression and treatment efficacy were evaluated. A significant negative correlation was seen with treatment with ERT and urinary GAG levels. Of those with baseline and follow-up growth data, 47% remained on their pre-treatment growth curve or moved to a higher percentile after treatment. Of the 9 patients with baseline and follow-up sleep studies, 5 remained unaffected and 1 patient initially with mild sleep apnea showed improvement. Data regarding cardiac, ophthalmic, central nervous system, hearing, surgical interventions and development are also reported. No patient discontinued treatment due to an adverse event and all that were treatment-emergent resolved. The prescribed dosage of 1mg/kg IV weekly with galsulfase ERT is shown to be safe and effective in slowing and/or improving certain aspects of the disease, although patients should be closely monitored for complications associated with the natural history of the disease, especially cardiac valve involvement and spinal cord compression. A long-term follow-up investigation of this group of children will provide further information on the benefits of early treatment as well as disease progression and treatment efficacy and safety in this young patient population. Copyright © 2013 Elsevier Inc. All rights reserved.

Exploration for fossil and nuclear fuels from orbital altitudes. [results of ERTS program for oil exploration

NASA Technical Reports Server (NTRS)

Short, N. M.

1974-01-01

Results from the ERTS program pertinent to exploration for oil, gas, and uranium are discussed. A review of achievements in relevant geological studies from ERTS, and a survey of accomplishments oriented towards exploration for energy sources are presented along with an evaluation of the prospects and limitations of the space platform approach to fuel exploration, and an examination of continuing programs designed to prove out the use of ERTS and other space system in exploring for fuel resources.

An integrated study of earth resources in the state of California based on ERTS-1 and supporting aircraft data

NASA Technical Reports Server (NTRS)

Colwell, R. N.; Thorley, G. A.; Burgy, R. H.; Schubert, G.; Estes, J. E.; Bowden, L. W.; Algazi, V. R.; Wildman, W. E.; Huntington, G. L. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. Results of an integrated study of earth resources in the state of California using ERTS-1 and supporting aircraft data are presented. Areas of investigation cover (1) regional agricultural surveys; (2) solving water resource management problems; (3) resource management in Northern California using ERTS-1 data; (4) analysis of river meanders; (5) assessment and monitoring change in west side of the San Joaquin Valley and central coastal zone of state; (6) assessment and monitoring of changes in Southern California environment; (7) digital handling and processing of ERTS-1 data; (8) use of ERTS-1 data in educational and applied research programs of the Agricultural Extension Service; and (9) identification, classification, and mapping of salt affected soils.

Cerebellar Alterations and Gait Defects as Therapeutic Outcome Measures for Enzyme Replacement Therapy in α-Mannosidosis

PubMed Central

Damme, Markus; Stroobants, Stijn; Walkley, Steven U.; Lüllmann-Rauch, Renate; D`Hooge, Rudi; Fogh, Jens; Saftig, Paul; Lübke, Torben; Blanz, Judith

2011-01-01

α-Mannosidosis is a rare lysosomal storage disease with accumulation of undegraded mannosyl-linked oligosaccharides in cells throughout the body, most notably in the CNS. This leads to a broad spectrum of neurological manifestations, including progressive intellectual impairment, disturbed motor functions and cerebellar atrophy. To develop therapeutic outcome measures for enzyme replacement therapy (ERT) that could be used for human patients, a gene knockout model of α-mannosidosis in mice was analyzed for CNS pathology and motor deficits. In the cerebellar molecular layer, α-mannosidosis mice display clusters of activated Bergman glia, infiltration of phagocytic macrophages and accumulation of free cholesterol and gangliosides (GM1), notably in regions lacking Purkinje cells. α-mannosidosis brain lysates also displayed increased expression of Lamp1 and hyperglycosylation of the cholesterol binding protein NPC2. Detailed assessment of motor function revealed age-dependent gait defects in the mice that resemble the disturbed motor function in human patients. Short-term ERT partially reversed the observed cerebellar pathology with fewer activated macrophages and astrocytes but unchanged levels of hyperglycosylated NPC2, gangliosides and cholesterol. The present study demonstrates cerebellar alterations in α-mannosidosis mice that relate to the motor deficits and pathological changes seen in human patients and can be used as therapeutic outcome measures. PMID:21157375

A Coupled model for ERT monitoring of contaminated sites

NASA Astrophysics Data System (ADS)

Wang, Yuling; Zhang, Bo; Gong, Shulan; Xu, Ya

2018-02-01

The performance of electrical resistivity tomography (ERT) system is usually investigated using a fixed resistivity distribution model in numerical simulation study. In this paper, a method to construct a time-varying resistivity model by coupling water transport, solute transport and constant current field is proposed for ERT monitoring of contaminated sites. Using the proposed method, a monitoring model is constructed for a contaminated site with a pollution region on the surface and ERT monitoring results at different time is calculated by the finite element method. The results show that ERT monitoring profiles can effectively reflect the increase of the pollution area caused by the diffusion of pollutants, but the extent of the pollution is not exactly the same as the actual situation. The model can be extended to any other case and can be used to scheme design and results analysis for ERT monitoring.

Molecular cloning of Kuruma shrimp Marsupenaeus japonicus endonuclease-reverse transcriptase and its positive role in white spot syndrome virus and Vibrio alginolyticus infection.

PubMed

Ma, Xiongchao; Sun, Baozhen; Zhu, Fei

2018-02-01

This study investigated the function of endonuclease-reverse transcriptase (mjERT) in Marsupenaeus japonicus. The 1129 bp cDNA sequence of mjERT was cloned from M. japonicus using rapid amplification of cDNA ends (RACE) PCR, and RT-qPCR analysis indicated that mjERT was highly expressed in the gills and hepatopancreas of M. japonicus. We also found that white spot syndrome virus (WSSV) or Vibrio alginolyticus challenge could enhance the expression of mjERT. When mjERT was inhibited, immune genes such as toll, p53, hemocyanin and tumor necrosis factor-α (TNF-α) were significantly down-regulated (P < .01) in the hemocytes of shrimp, while myosin was significantly up-regulated (P < .01). We demonstrated that mjERT is very important for the progression of WSSV infection and that the cumulative mortality of WSSV-infected and V. alginolyticus-infected shrimps was significantly increased following mjERT RNA interfere (RNAi). Apoptosis data provided information to suggest that mjERT-dsRNA challenge caused less apoptosis in hemocytes in both the disease-free and viral group. We also revealed that mjERT-dsRNA treatment resulted in a lower phagocytosis rate in the hemocytes of V. alginolyticus-challenged shrimp. Finally, we found that the absence of mjERT had an significantly negative impact upon shrimp phenoloxidase (PO) activity, superoxide dismutase (SOD) activity and total hemocyte count (THC) following WSSV or V. alginolyticus infection, indicating a regulative role for mjERT in the innate immunity of shrimp in response to pathogenic infection. In summary, we concluded that mjERT might promote the anti-WSSV immune response of shrimp by regulating apoptosis, PO activity, THC and SOD activity, and also exert a positive role in the immune response against V. alginolyticus by regulating phagocytosis, SOD activity, PO activity and THC. Copyright © 2017 Elsevier Ltd. All rights reserved.

ERTS imagery for ground-water investigations

USGS Publications Warehouse

Moore, Gerald K.; Deutsch, Morris

1975-01-01

ERTS imagery offers the first opportunity to apply moderately high-resolution satellite data to the nationwide study of water resources. This imagery is both a tool and a form of basic data. Like other tools and basic data, it should be considered for use in ground-water investigations. The main advantage of its use will be to reduce the need for field work. In addition, however, broad regional features may be seen easily on ERTS imagery, whereas they would be difficult or impossible to see on the ground or on low-altitude aerial photographs. Some present and potential uses of ERTS imagery are to locate new aquifers, to study aquifer recharge and discharge, to estimate ground-water pumpage for irrigation, to predict the location and type of aquifer management problems, and to locate and monitor strip mines which commonly are sources for acid mine drainage. In many cases, boundaries which are gradational on the ground appear to be sharp on ERTS imagery. Initial results indicate that the accuracy of maps produced from ERTS imagery is completely adequate for some purposes.

Analysis of ERTS-1 imagery and its application to evaluation of Wyoming's natural resources

NASA Technical Reports Server (NTRS)

Marrs, R. W.

1973-01-01

The author has identified the following significant results. A summary of the significant results of the studies completed during the July-August, 1973 period includes: (1) ERTS-1 image brightness contrasts can be related to important contrasts in rangeland and forest vegetation communities of the Laramie Basin. (2) Stereoscopic viewing is essential for correct structural interpretation in outcrop patterns in some areas. (3) Complex fracture patterns which may have exerted a controlling influence on intrusive activity in the Absaroka Mountains can be mapped from ERTS. (4) Volcanic lithologies of the Yellowstone region are often differentiated on the basis of their textures, and cannot be successfully mapped by photogeologic interpretation of ERTS-1 imagery. Ground spectral readings confirm a general lack of contrast between these lithologies in the four ERTS-1 MSS bands. (5) Major dune fields can be recognized and defined from ERTS-1 image interpretations and recognition of differences in stabilizing plant communities (some of which may be mappable from ERTS-1) yields information about migration history of the dune fields.

A summary of selected early results from the ERTS-1 menhaden experiment

NASA Technical Reports Server (NTRS)

Stevenson, W. H. (Principal Investigator); Kemmerer, A. J.; Benigno, J. A.; Reese, G. B.; Minkler, F. C.

1973-01-01

The author has identified the following significant results. Imagery from ERTS-1 satellite was used in conjunction with aerial photographically-sensed menhaden distribution information, sea truth oceanographic measurements, and commercial fishing information from a 8685 square kilometer study area in the north-central portion of the Gulf of Mexico to demonstrate relationships between selected oceanographic parameters and menhaden distribution, ERTS-1 imagery and menhaden distribution, and ERTS-1 imagery and oceanographic parameters. ERTS-1, MSS band 5 imagery density levels correlated with photographically detected menhaden distribution patterns and could be explained based on sea truth Secchi disc transparency and water depth measurements. These two parameters, together with surface salinity, Forel-Ule color, and chlorophyll-a also were found to correlate significantly with menhaden distribution. Eight empirical models were developed which provided menhaden distribution predictions for the study area on combinations of Secchi disc transparency, water depth, surface salinity, and Forel-Ule color measurements.

Assessment of Southern California environment from ERTS-1

NASA Technical Reports Server (NTRS)

Bowden, L. W.; Viellenave, J. H.

1973-01-01

ERTS-1 imagery is a useful source of data for evaluation of earth resources in Southern California. The improving quality of ERTS-1 imagery, and our increasing ability to enhance the imagery has resulted in studies of a variety of phenomena in several Southern California environments. These investigations have produced several significant results of varying detail. They include the detection and identification of macro-scale tectonic and vegetational patterns, as well as detailed analysis of urban and agricultural processes. The sequential nature of ERTS-1 imagery has allowed these studies to monitor significant changes in the environment. In addiation, some preliminary work has begun directed toward assessing the impact of expanding recreation, agriculture and urbanization into the fragile desert environment. Refinement of enhancement and mapping techniques and more intensive analysis of ERTS-1 imagery should lead to a greater capability to extract detailed information for more precise evaluations and more accurate monitoring of earth resources in Southern California.

Inducible targeting of CNS astrocytes in Aldh1l1-CreERT2 BAC transgenic mice

PubMed Central

Winchenbach, Jan; Düking, Tim; Berghoff, Stefan A.; Stumpf, Sina K.; Hülsmann, Swen; Nave, Klaus-Armin; Saher, Gesine

2016-01-01

Background: Studying astrocytes in higher brain functions has been hampered by the lack of genetic tools for the efficient expression of inducible Cre recombinase throughout the CNS, including the neocortex. Methods: Therefore, we generated BAC transgenic mice, in which CreERT2 is expressed under control of the Aldh1l1 regulatory region. Results: When crossbred to Cre reporter mice, adult Aldh1l1-CreERT2 mice show efficient gene targeting in astrocytes. No such Cre-mediated recombination was detectable in CNS neurons, oligodendrocytes, and microglia. As expected, Aldh1l1-CreERT2 expression was evident in several peripheral organs, including liver and kidney. Conclusions: Taken together, Aldh1l1-CreERT2 mice are a useful tool for studying astrocytes in neurovascular coupling, brain metabolism, synaptic plasticity and other aspects of neuron-glia interactions. PMID:28149504

Inducible targeting of CNS astrocytes in Aldh1l1-CreERT2 BAC transgenic mice.

PubMed

Winchenbach, Jan; Düking, Tim; Berghoff, Stefan A; Stumpf, Sina K; Hülsmann, Swen; Nave, Klaus-Armin; Saher, Gesine

2016-01-01

Background: Studying astrocytes in higher brain functions has been hampered by the lack of genetic tools for the efficient expression of inducible Cre recombinase throughout the CNS, including the neocortex. Methods: Therefore, we generated BAC transgenic mice, in which CreERT2 is expressed under control of the Aldh1l1 regulatory region. Results: When crossbred to Cre reporter mice, adult Aldh1l1-CreERT2 mice show efficient gene targeting in astrocytes. No such Cre-mediated recombination was detectable in CNS neurons, oligodendrocytes, and microglia. As expected, Aldh1l1-CreERT2 expression was evident in several peripheral organs, including liver and kidney. Conclusions: Taken together, Aldh1l1-CreERT2 mice are a useful tool for studying astrocytes in neurovascular coupling, brain metabolism, synaptic plasticity and other aspects of neuron-glia interactions.

The utilization of ERTS-1 data for the study of the French Atlantic Littoral. [coastal water and geomorphology

NASA Technical Reports Server (NTRS)

Demathieu, P. G.; Verger, F. H.

1974-01-01

The French Atlantic Littoral (FRALIT) program uses ERTS-1 data to study coastal geomorphology and waters. ERTS-1 gives an overall picture of the phenomena for the first time due mainly to channel 4 data, but the other channels also contribute valuable complementary data on superficial waters. These studies have already resulted in accurate maps of the mud transported south-westwards from the mouth of the River Loire.

Flood hazards studies in the Mississippi River basin using remote sensing

NASA Technical Reports Server (NTRS)

Rango, A.; Anderson, A. T.

1974-01-01

The Spring 1973 Mississippi River flood was investigated using remotely sensed data from ERTS-1. Both manual and automatic analyses of the data indicated that ERTS-1 is extremely useful as a regional tool for flood mamagement. Quantitative estimates of area flooded were made in St. Charles County, Missouri and Arkansas. Flood hazard mapping was conducted in three study areas along the Mississippi River using pre-flood ERTS-1 imagery enlarged to 1:250,000 and 1:100,000 scale. Initial results indicate that ERTS-1 digital mapping of flood prone areas can be performed at 1:62,500 which is comparable to some conventional flood hazard map scales.

Evaluating time-lapse ERT for monitoring DNAPL remediation via numerical simulation

NASA Astrophysics Data System (ADS)

Power, C.; Karaoulis, M.; Gerhard, J.; Tsourlos, P.; Giannopoulos, A.

2012-12-01

Dense non-aqueous phase liquids (DNAPLs) remain a challenging geoenvironmental problem in the near subsurface. Numerous thermal, chemical, and biological treatment methods are being applied at sites but without a non-destructive, rapid technique to map the evolution of DNAPL mass in space and time, the degree of remedial success is difficult to quantify. Electrical resistivity tomography (ERT) has long been presented as highly promising in this context but has not yet become a practitioner's tool due to challenges in interpreting the survey results at real sites where the initial condition (DNAPL mass, DNAPL distribution, subsurface heterogeneity) is typically unknown. Recently, a new numerical model was presented that couples DNAPL and ERT simulation at the field scale, providing a tool for optimizing ERT application and interpretation at DNAPL sites (Power et al., 2011, Fall AGU, H31D-1191). The objective of this study is to employ this tool to evaluate the effectiveness of time-lapse ERT to monitor DNAPL source zone remediation, taking advantage of new inversion methodologies that exploit the differences in the target over time. Several three-dimensional releases of chlorinated solvent DNAPLs into heterogeneous clayey sand at the field scale were generated, varying in the depth and complexity of the source zone (target). Over time, dissolution of the DNAPL in groundwater was simulated with simultaneous mapping via periodic ERT surveys. Both surface and borehole ERT surveys were conducted for comparison purposes. The latest four-dimensional ERT inversion algorithms were employed to generate time-lapse isosurfaces of the DNAPL source zone for all cases. This methodology provided a qualitative assessment of the ability of ERT to track DNAPL mass removal for complex source zones in realistically heterogeneous environments. In addition, it provided a quantitative comparison between the actual DNAPL mass removed and that interpreted by ERT as a function of depth below the water table, as well as an estimate of the minimum DNAPL saturation changes necessary for an observable response from ERT.

Detection of Old Mine Tunnels in Mexico City Highlands by Electric Resistivity Image Methods

NASA Astrophysics Data System (ADS)

Chavez, R. E.; Tejero, A.; Cifuentes-Nava, G.; HernaNdez-Quintero, J.

2013-12-01

Electrical Resistivity Tomography (ERT) methods have been applied to study cavities or subsurface subsidence threatening urbanized areas. Unfortunately, ERT-3D techniques carried out on heavily urbanized areas become a difficult task, since parallel ERT arrays cannot be deployed. Then, a conventional regular grid cannot be carried out. We present a subsidence problem located in a densely populated portion of Mexico City highlands. Since the damaged houses are in the middle of a highly populated low-class neighborhood, an unconventional ERT array had to be applied. At first, a ';T'-array formed by two perpendicular transects was applied, deployed within a small alley, that stretched from the house entrance. This study determined a tubular structure beneath the houses following an irregular path at depth. Finally, houses were demolished due to the extensive damaged in their foundations. This made possible to carry out a second ERT-3D study, which included a dipolar array called ';L' and ';Corner' arrays. Such a new work defined a similar tubular structure. The cavity entrance was discovered, when excavations were made, although its precise shape could not be defined. The ERT-3D interpretation contributed to locate and accurately determine the geometrical characteristics of the geological feature that caused the collapse of dwellings.

A study of the utilization of ERTS-1 data from the Wabash River Basin. [crop identification, water resources, urban land use, soil mapping, and atmospheric modeling

NASA Technical Reports Server (NTRS)

Landgrebe, D. A. (Principal Investigator)

1974-01-01

The author has identified the following significant results. The most significant results were obtained in the water resources research, urban land use mapping, and soil association mapping projects. ERTS-1 data was used to classify water bodies to determine acreages and high agreement was obtained with USGS figures. Quantitative evaluation was achieved of urban land use classifications from ERTS-1 data and an overall test accuracy of 90.3% was observed. ERTS-1 data classifications of soil test sites were compared with soil association maps scaled to match the computer produced map and good agreement was observed. In some cases the ERTS-1 results proved to be more accurate than the soil association map.

Electrical resistance tomography to monitor unsaturated moisture flow in cementitious materials

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hallaji, Milad; Seppänen, Aku; Pour-Ghaz, Mohammad, E-mail: mpourghaz@ncsu.edu

2015-03-15

Traditionally the electrically-based assessment of the moisture flow in cement-based materials relies on two- or four-point measurements. In this paper, imaging of moisture distribution with electrical resistance tomography (ERT) is considered. Especially, the aim is to study whether ERT could give information on unsaturated moisture flows in cases where the flow is non-uniform. In the experiment, the specimens are monitored with ERT during the water ingress. The ERT reconstructions are compared with neutron radiographs, which provide high resolution information on the 2D distribution of the moisture. The results indicate that ERT is able to detect the moisture movement and tomore » show approximately the shape and position of the water front even if the flow is nonuniform.« less

Analysis of ERTS imagery of Wyoming and its application to evaluation of Wyoming's natural resources

NASA Technical Reports Server (NTRS)

Houston, R. S. (Principal Investigator); Marrs, R. W.

1972-01-01

The author has identified the following significant results. The Wyoming ERTS investigation has been hindered only slightly by incomplete ERTS data sets and lack of coverage. Efforts to map cultural development, vegetation distributions, and various geomorphologic features are underway. Tectonic analysis of the Rock Springs area has isolated two linear features that may be very significant with regard to the regional structure of central Wyoming. Studies of the fracture systems of the Wind River Mountains are being completed. The fracture map, constructed from ERTS-1 interpretations, contains a great deal of structural information which was previously unavailable. Mapping of the Precambrian metasedimentary and metavolcanic terrain of the Granite Mountains is nearing completion, and interpretation of ERTS supporting aircraft data has revealed deposits of iron formation.

Geographic applications of ERTS-1 imagery to landscape change. [Mississippi River and Great Smoky Mountains of Tennessee and North Carolina

NASA Technical Reports Server (NTRS)

Rehder, J. B. (Principal Investigator)

1973-01-01

The author has identified the following significant results. ERTS-1 has proven to be an effective earth-orbiting monitor of landscape change. Its regional coverage for large areal monitoring has been effective for the detection and mapping of agricultural plowing regions, for general forest cover mapping, for flood mapping, for strip mine mapping, and for short-lived precipitation mapping patterns. Paramount to the entire study has been the temporal coverage provided by ERTS. Without the cyclic coverage on an 18 day basis, temporal coverage would have been inadequate for the detection and mapping of strip mining landscape change, the analysis of agricultural landscape change based on plowing patterns, the analysis of urban-suburban growth changes, and the mapping of the Mississippi River floods. Cost benefits from ERTS are unquestionably superior to aircraft systems in regard to large regional coverage and cyclic temporal parameters. For the analysis of landscape change in large regions such as statewide areas or even areas of 10,000 square miles, ERTS is of cost benefit consideration. Not only does the cost of imagery favor ERTS but the reduction of man-hours using ERTS has been in the magnitude of 1:10.

How We Manage Adenosine Deaminase-Deficient Severe Combined Immune Deficiency (ADA SCID).

PubMed

Kohn, Donald B; Gaspar, H Bobby

2017-05-01

Adenosine deaminase-deficient severe combined immune deficiency (ADA SCID) accounts for 10-15% of cases of human SCID. From what was once a uniformly fatal disease, the prognosis for infants with ADA SCID has improved greatly based on the development of multiple therapeutic options, coupled with more frequent early diagnosis due to implementation of newborn screening for SCID. We review the various treatment approaches for ADA SCID including allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen-matched sibling or family member or from a matched unrelated donor or a haplo-identical donor, autologous HSCT with gene correction of the hematopoietic stem cells (gene therapy-GT), and enzyme replacement therapy (ERT) with polyethylene glycol-conjugated adenosine deaminase. Based on growing evidence of safety and efficacy from GT, we propose a treatment algorithm for patients with ADA SCID that recommends HSCT from a matched family donor, when available, as a first choice, followed by GT as the next option, with allogeneic HSCT from an unrelated or haplo-identical donor or long-term ERT as other options.

Fabry nephropathy: a review - how can we optimize the management of Fabry nephropathy?

PubMed

Waldek, Stephen; Feriozzi, Sandro

2014-05-06

Fabry disease is a rare, X-linked, lysosomal storage disease caused by mutations in the gene encoding the enzyme alpha-galactosidase A. Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in many cell types throughout the body, including the kidney. Progressive accumulation of Gb3 in podocytes, epithelial cells and the tubular cells of the distal tubule and loop of Henle contribute to the renal symptoms of Fabry disease, which manifest as proteinuria and reduced glomerular filtration rate leading to chronic kidney disease and progression to end-stage renal disease. Early diagnosis and timely initiation of treatment of Fabry renal disease is an important facet of disease management. Initiating treatment with enzyme replacement therapy (ERT; agalsidase alfa, Replagal®, Shire; agalsidase beta, Fabrazyme®, Genzyme) as part of a comprehensive strategy to prevent complications of the disease, may be beneficial in stabilizing renal function or slowing its decline. Early initiation of ERT may also be more effective than initiating therapy in patients with more advanced disease. Several strategies are required to complement the use of ERT and treat the myriad of associated symptoms and organ involvements. In particular, patients with renal Fabry disease are at risk of cardiovascular events, such as high blood pressure, cardiac arrhythmias and stroke. This review discusses the management of renal involvement in Fabry disease, including diagnosis, treatments, and follow-up, and explores recent advances in the use of biomarkers to assist with diagnosis, monitoring disease progression and response to treatment.

Fabry nephropathy: a review – how can we optimize the management of Fabry nephropathy?

PubMed Central

2014-01-01

Fabry disease is a rare, X-linked, lysosomal storage disease caused by mutations in the gene encoding the enzyme alpha-galactosidase A. Complete or partial deficiency in this enzyme leads to intracellular accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in many cell types throughout the body, including the kidney. Progressive accumulation of Gb3 in podocytes, epithelial cells and the tubular cells of the distal tubule and loop of Henle contribute to the renal symptoms of Fabry disease, which manifest as proteinuria and reduced glomerular filtration rate leading to chronic kidney disease and progression to end-stage renal disease. Early diagnosis and timely initiation of treatment of Fabry renal disease is an important facet of disease management. Initiating treatment with enzyme replacement therapy (ERT; agalsidase alfa, Replagal®, Shire; agalsidase beta, Fabrazyme®, Genzyme) as part of a comprehensive strategy to prevent complications of the disease, may be beneficial in stabilizing renal function or slowing its decline. Early initiation of ERT may also be more effective than initiating therapy in patients with more advanced disease. Several strategies are required to complement the use of ERT and treat the myriad of associated symptoms and organ involvements. In particular, patients with renal Fabry disease are at risk of cardiovascular events, such as high blood pressure, cardiac arrhythmias and stroke. This review discusses the management of renal involvement in Fabry disease, including diagnosis, treatments, and follow-up, and explores recent advances in the use of biomarkers to assist with diagnosis, monitoring disease progression and response to treatment. PMID:24886109

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

PubMed Central

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

2009-01-01

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

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

PubMed Central

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

2017-01-01

Background 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. Objectives To evaluate the efficacy and safety of ERT for AFD. Materials and methods 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. Results 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%, p<0.0001]. Effect differences favored agalsidase beta compared to untreated patients; b) for cardiovascular complications, agalsidase alfa 28% [95% CI 0.07, 0.55; I2 = 96.7%, p<0.0001]; agalsidase beta 7% [95% CI 0.05, 0.08; I2 = not applicable]; and untreated patients 26.2% [95% CI 0.149, 0.394; I2 = 98.8%, p<0.0001]. Effect differences favored agalsidase beta compared to untreated patients; and c) for cerebrovascular complications, agalsidase alfa 11.1% [95% CI 0.058, 0.179; I2 = 70.5%, p = 0.0024]; agalsidase beta 3.5% [95% CI 0.024, 0.046; I2 = 0%, p = 0.4209]; and untreated patients 18.3% [95% CI 0.129, 0.245; I2 = 95% p < 0.0001]. Effect differences favored agalsidase beta over agalsidase alfa or untreated patients. A linear regression showed that Fabry patients receiving agalsidase alfa are more likely to have higher rates of composite endpoints compared to those receiving agalsidase beta. Conclusions 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. PMID:28296917

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.

PubMed

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%, p<0.0001]. Effect differences favored agalsidase beta compared to untreated patients; b) for cardiovascular complications, agalsidase alfa 28% [95% CI 0.07, 0.55; I2 = 96.7%, p<0.0001]; agalsidase beta 7% [95% CI 0.05, 0.08; I2 = not applicable]; and untreated patients 26.2% [95% CI 0.149, 0.394; I2 = 98.8%, p<0.0001]. Effect differences favored agalsidase beta compared to untreated patients; and c) for cerebrovascular complications, agalsidase alfa 11.1% [95% CI 0.058, 0.179; I2 = 70.5%, p = 0.0024]; agalsidase beta 3.5% [95% CI 0.024, 0.046; I2 = 0%, p = 0.4209]; and untreated patients 18.3% [95% CI 0.129, 0.245; I2 = 95% p < 0.0001]. Effect differences favored agalsidase beta over agalsidase alfa or untreated patients. A linear 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.

Enzyme replacement therapy with velmanase alfa (human recombinant alpha-mannosidase): Novel global treatment response model and outcomes in patients with alpha-mannosidosis.

PubMed

Harmatz, Paul; Cattaneo, Federica; Ardigò, Diego; Geraci, Silvia; Hennermann, Julia B; Guffon, Nathalie; Lund, Allan; Hendriksz, Christian J; Borgwardt, Line

2018-06-01

Alpha-mannosidosis is an ultra-rare monogenic disorder resulting from a deficiency in the lysosomal enzyme alpha-mannosidase, with a prevalence estimated to be as low as 1:1,000,000 live births. The resulting accumulation of mannose-rich oligosaccharides in all tissues leads to a very heterogeneous disorder with a continuum of clinical manifestations with no distinctive phenotypes. Long-term enzyme replacement therapy (ERT) with velmanase alfa is approved in Europe for the treatment of non-neurological manifestations in patients with mild to moderate alpha-mannosidosis. The clinical heterogeneity and rarity of the disease limit the sensitivity of single parameters to detect clinically relevant treatment effects. Thus, we propose a novel multiple variable responder analysis to evaluate the efficacy of ERT for alpha-mannosidosis and present efficacy analyses for velmanase alfa using this method. Global treatment response to velmanase alfa (defined by response to ≥2 domains comprising pharmacodynamic, functional, and quality of life outcomes) was applied post hoc to data from the pivotal placebo-controlled rhLAMAN-05 study and to the longer-term integrated data from all patients in the clinical development program (rhLAMAN-10). After 12 months of treatment, a global treatment response was achieved by 87% of patients receiving velmanase alfa (n = 15) compared with 30% of patients receiving placebo (n = 10). Longer-term data from all patients in the clinical program (n = 33) showed 88% of patients were global responders, including all (100%) pediatric patients (n = 19) and the majority (71%) of adult patients (n = 14). The responder analysis model demonstrates a clinically meaningful treatment effect with velmanase alfa and supports the early initiation and continued benefit of longer-term treatment of all patients with alpha-mannosidosis with this ERT. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

Forward problem studies of electrical resistance tomography system on concrete materials

NASA Astrophysics Data System (ADS)

Ang, Vernoon; Rahiman, M. H. F.; Rahim, R. A.; Aw, S. R.; Wahab, Y. A.; Thomas W. K., T.; Siow, L. T.

2017-03-01

Electrical resistance tomography (ERT) is well known as non-invasive imaging technique, inexpensive, radiation free, visualization measurements of the multiphase flows and frequently applied in geophysical, medical and Industrial Process Tomography (IPT) applications. Application of ERT in concrete is a new exploration field, which can be used in monitoring and detecting the health and condition of concrete without destroying it. In this paper, ERT model under the condition of concrete is studied in which the sensitivity field model is produced and simulated by using COMSOL software. The affects brought by different current injection values with different concrete conductivity are studied in detail. This study able to provide the important direction for the further study of inverse problem in ERT system. Besides, the results of this technique hopefully can open a new exploration in inspection method of concrete structures in order to maintain the health of the concrete structure for civilian safety.

Long-term cognitive follow-up in children treated for Maroteaux-Lamy syndrome.

PubMed

Ebbink, Berendine J; Brands, Marion M G; van den Hout, Johanna M P; Lequin, Maarten H; Coebergh van den Braak, Robert R J; van de Weitgraven, Rianne L; Plug, Iris; Aarsen, Femke K; van der Ploeg, Ans T

2016-03-01

It remains unclear to what extent the brain is affected by Maroteaux-Lamy syndrome (MPS VI), a progressive lysosomal storage disorder. While enzyme replacement therapy (ERT) elicits positive effects, the drug cannot cross the blood-brain barrier. We therefore studied cognitive development and brain abnormalities in the Dutch MPS VI patient population treated with ERT. In a series of 11 children with MPS VI (age 2 to 20 years), we assessed cognitive functioning and brain magnetic resonance imaging prospectively at the start of ERT and at regular times thereafter up to 4.8 years. We also assessed the children's clinical characteristics, their siblings' cognitive development, and their parents' educational levels. The patients' intelligence scores ranged from normal to mentally delayed (range test scores 52-131). In 90%, their scores remained fairly stable during follow-up, generally lying in the same range as their siblings' test scores (median for patients = 104, median for siblings = 88) and comparing well with the parental educational levels. Native-speaking patients had higher intelligence test scores than non-native-speaking patients. Two patients, both with high baseline glycosaminoglycan levels in their urine and severe mutations in the arylsulfatase B gene, scored clearly lower than expected. Patients with pY210C performed best. Brain abnormalities were aspecific, occurring more in patients with severe symptoms. Our study shows that cognitive development in MPS VI patients is determined not only by familial and social-background factors, but, in patients with a severe form of the disease, also by the disease itself. Therefore in patients with severe disease presentation cognition should be monitored carefully.

Stochastic Representation and Uncertainty Assessment of a Deep Geothermal Reservoir Using Cross-Borehole ERT: A 3D Synthetic Case

NASA Astrophysics Data System (ADS)

Brunet, P.; Gloaguen, E.

2014-12-01

Designing and monitoring of geothermal systems is a complex task which requires a multidisciplinary approach. Deep geothermal reservoir models are prone to greater uncertainty, with a lack of direct data and lower resolution of surface geophysical methods. However, recent technical advances have enabled the potential use of permanent downhole vertical resistivity arrays for monitoring fluid injection. As electrical resistivity is sensitive to temperature changes, such data could provide valuable information for deep geothermal reservoir characterization. The objective of this study is to assess the potential of time-lapse cross-borehole ERT to constrain 3D realizations of geothermal reservoir properties. The synthetic case of a permeable geothermal reservoir in a sedimentary basin was set up, as a confined deep and saline sandstone aquifer with intermediate reservoir temperatures (150ºC), depth (1 km) and 30m thickness. The reservoir permeability distribution is heterogeneous, as the result of a fluvial depositional environment. The ERT monitoring system design is a triangular arrangement of 3 wells at 150 m spacing, including 1 injection and 1 extraction well. The optimal number and spacing of electrodes of the ERT array design is site-specific and has been assessed through a sensibility study. Dipole-dipole and pole-pole electrode configurations were used. The study workflow was the following: 1) Generation of a reference reservoir model and 100 stochastic realizations of permeability; 2) Simulation of saturated single-phase flow and heat transport of reinjection of cooled formation fluid (50ºC) with TOUGH2 software; 3) Time-lapse forward ERT modeling on the reference model and all realizations (observed and simulated apparent resistivity change); 4) heuristic optimization on ERT computed and calculated data. Preliminary results show significant reduction of parameter uncertainty, hence realization space, with assimilation of cross-borehole ERT data. Loss in sensitivity of ERT between boreholes is compensated here by the stochastic modeling approach, rather than using a deterministic inversion scheme. Our results suggest stochastic reservoir simulations, together with assimilation of cross-borehole ERT data, could be useful tools for design and monitoring of deep geothermal systems.

A study of the utilization of ERTS-1 data from the Wabash River Basin

NASA Technical Reports Server (NTRS)

Landgrebe, D. A. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Nine projects are defined, five ERTS data applications experiments and four supporting technology tasks. The most significant applications results were achieved in the soil association mapping, earth surface feature identification, and urban land use mapping efforts. Four soil association boundaries were accurately delineated from ERTS-1 imagery. A data bank has been developed to test surface feature classifications obtained from ERTS-1 data. Preliminary forest cover classifications indicated that the number of acres estimated tended to be greater than actually existed by 25%. Urban land use analysis of ERTS-1 data indicated highly accurate classification could be obtained for many urban catagories. The wooded residential category tended to be misclassified as woods or agricultural land. Further statistical analysis revealed that these classes could be separated using sample variance.

Effect of reduced agalsidase Beta dosage in fabry patients: the Australian experience.

PubMed

Ghali, Joanna; Nicholls, Kathy; Denaro, Charles; Sillence, David; Chapman, Ian; Goldblatt, Jack; Thomas, Mark; Fletcher, Janice

2012-01-01

In Australia, enzyme replacement therapy (ERT) for Fabry Disease (FD), both Agalsidase alfa (Replagal, Shire HGT) and beta (Fabrazyme, Genzyme), is funded and monitored through a specific government program. Agalsidase beta supply has been rationed by Genzyme since 2009 due to manufacturing issues. Consequently, the Australian Fabry Disease Advisory Committee has treated patients on Agalsidase beta at 50% of their usual dose from mid-2009, with a further reduction to 30% for some patients from late 2009. To determine the clinical effect of Agalsidase beta dose reduction in the Australian FD patient cohort. A questionnaire assessing FD symptoms was administered to 40 patients on long-term ERT. Clinical data from The Fabry Registry for patients receiving Agalsidase alfa or beta, for at least 2 years prior to the time of enforced Agalsidase beta dose reduction, were reviewed. Disease burden and quality of life (QOL) were graded using the Disease Severity Scoring System, Mainz Severity Score Index, Brief Pain Inventory and Short Form 36 Health Survey at 2 years before dose reduction, at the time of dose reduction and at the most recent clinical review following dose reduction. Disease severity and QOL scores did not change between the ERT groups. Males on Agalsidase beta reported lower energy levels after dose reduction, while no change was reported by females on either product or by males on a stable dose of Agalsidase alfa. This study suggests that energy levels in male patients worsen after dose reduction of Agalsidase beta.

Determine utility of ERTS-1 to detect and monitor area strip mining and reclamation. [southeastern Ohio

NASA Technical Reports Server (NTRS)

Rogers, R. H. (Principal Investigator); Pettyjohn, W. A.

1975-01-01

The author has identified the following significant results. Computer techniques were applied to process ERTS tapes acquired over coal mining operations in southeastern Ohio on 21 August 1972 and 3 September 1973. ERTS products obtained included geometrically correct map overlays showing stripped earth, partially reclaimed earth, water, and natural vegetation. Computer-generated tables listing the area covered by each land-water category in square kilometers and acres were produced. By comparing these mapping products, the study demonstrates the capability of ERTS to monitor changes in the extent of stripping, success of reclamation, and the secondary effects of mining on the environment.

All-digital precision processing of ERTS images

NASA Technical Reports Server (NTRS)

Bernstein, R. (Principal Investigator)

1975-01-01

The author has identified the following significant results. Digital techniques have been developed and used to apply precision-grade radiometric and geometric corrections to ERTS MSS and RBV scenes. Geometric accuracies sufficient for mapping at 1:250,000 scale have been demonstrated. Radiometric quality has been superior to ERTS NDPF precision products. A configuration analysis has shown that feasible, cost-effective all-digital systems for correcting ERTS data are easily obtainable. This report contains a summary of all results obtained during this study and includes: (1) radiometric and geometric correction techniques, (2) reseau detection, (3) GCP location, (4) resampling, (5) alternative configuration evaluations, and (6) error analysis.

Plant-based oral delivery of β-glucocerebrosidase as an enzyme replacement therapy for Gaucher's disease.

PubMed

Shaaltiel, Yoseph; Gingis-Velitski, Svetlana; Tzaban, Salit; Fiks, Nadia; Tekoah, Yoram; Aviezer, David

2015-10-01

Gaucher's disease (GD), a lysosomal storage disorder caused by mutations in the gene encoding glucocerebrosidase (GCD), is currently treated by enzyme replacement therapy (ERT) using recombinant GCD that is administered intravenously every 2 weeks. However, intravenous administration includes discomfort or pain and might cause local and systemic infections that may lead to low patient compliance. An orally administered drug has the potential to alleviate these problems. In this study, we describe the potential use of plant cells as a vehicle for the oral delivery of recombinant human GCD (prGCD) expressed in carrot cells. The in vitro results demonstrate that the plant cells protect the recombinant protein in the gastric fluids and may enable absorption into the blood. Feeding experiments, with rat and pig as model animals, using carrot cells containing prGCD, show that active recombinant prGCD was found in the digestive tract and blood system and reached both, liver and spleen, the target organs in GD. These results demonstrate that the oral administration of proteins encapsulated in plant cells is feasible. Specifically, carrot cells containing recombinant human prGCD can be used as an oral delivery system and are a feasible alternative to intravenous administration of ERT for GD. © 2015 Society for Experimental Biology, Association of Applied Biologists and John Wiley & Sons Ltd.

Skylab S192 data evaluation: Comparisons with ERTS-1 results. [classification results using ERTS-1 and Skylab MSS data over Holt County, Nebraska agricultural area

NASA Technical Reports Server (NTRS)

Chang, C. Y.

1974-01-01

The author has identified the following significant results. The Skylab S192 data was evaluated by: (1) comparing the classification results using S192 and ERTS-1 data over the Holt County, Nebraska agricultural study area, and (2) investigating the impact of signal-to-noise ratio on classification accuracies using registered S192 and ERTS-1 data. Results indicate: (1) The classification accuracy obtained on S192 data using its best subset of four bands can be expected to be as high as that on ERTS-1 data. (2) When a subset of four S192 bands that are spectrally similar to the ERTS-1 bands was used for classification, an obvious deterioration in the classification accuracy was observed with respect to the ERTS-1 results. (3) The thermal bands 13 and 14 as well as the near IR bands were found to be relatively important in the classification of agricultural data. Although bands 11 and 12 were highly correlated, both were invariably included in the best subsets of the band sizes, four and beyond, according to the divergence criterion. (4) The differentiation of corn from popcorn was difficult on both S192 and ERTS-1 data acquired at an early summer date. (5) The results on both sets of data indicate that it was relatively easy to differentiate grass from any other class.

Application of ERTS-1 satellite imagery for land use mapping and resource inventories in the central coastal region of California

NASA Technical Reports Server (NTRS)

Estes, J. E.; Thaman, R. R.; Senger, L. W.

1974-01-01

ERTS-1 satellite imagery has proved a valuable data source for land use as well as natural and cultural resource studies on a regional basis. ERTS-1 data also provide an excellent base for mapping resource related features and phenomena. These investigations are focused on a number of potential applications which are already showing promise of having operational utility.

Utilization of ERTS-1 data in North Carolina. [forested wetlands, water management, and land use

NASA Technical Reports Server (NTRS)

Welby, C. W. (Principal Investigator); Lammi, J. O.; Carson, R. J., III

1973-01-01

The author has identified the following significant results. ERTS-1 imagery has been used to study forested wetlands, dynamic processes off Coastal North Carolina, and land use patterns in the Wilmington, North Carolina area. The thrust of the investigation is still involvement of state and regional agencies in the use of ERTS-1 imagery in solving some of their day-to-day problems.

Fracture trends identified by ERTS-1 imagery in Utah and Nevada

NASA Technical Reports Server (NTRS)

Jensen, M. L. (Principal Investigator); Erickson, M. P.; Smith, M. R.

1973-01-01

The author has identified the following significant results. In the Utah-Nevada area, linear structural trends recorded on ERTS-1 imagery conform in part to previously recognized structures. In addition, the ERTS-1 imagery reveals cryptic structures not previously identified and not readily apparent in other imagery. These structures are illustrated by prominent east-west trending structures which appear to be concentrated in pre-volcanic rocks. This suggests that the structures are older than many of those with other trends which are equally prominent in volcanic and non-volcanic terrain. Since the older east-west structures may have controlled early Tertiary emplacement of magma or the ascent of mineralizing fluids, their recognition is important in minerial exploration. Soil-gas sampling and analysis for mercury content is being continued over structures, and projected trends of buried structures which appear, from studies of ERTS-1 imagery, to be favorable to mineralization. Comparison of ERTS-1 and Skylab imagery indicated that ERTS-1 imagery records more previously unrecognized linear structures than the Skylab imagery. In differentiating and identifying different rock types, the Skylab imagery appears to be more effective.

A reconnaissance space sensing investigation of crustal structure for a strip from the eastern Sierra Nevada to the Colorado Plateau

NASA Technical Reports Server (NTRS)

Liggett, M. A. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Research progress in an investigation using ERTS-1 MSS imagery to study regional tectonics and related natural resources is summarized. Field reconnaissance guided by analysis of ERTS-1 imagery has resulted in development of a tectonic model relating strike-slip faulting to crustal extension in the southern Basin Range Province. The tectonics of the northern Death Valley-Furnace Creek Fault Zone and spacially associated volcanism and mercury mineralization were also investigated. Field work in the southern Sierra Nevada has confirmed the existence of faults and diabase dike swarms aligned along several major lineaments first recognized in ERTS-1 imagery. Various image enhancement and analysis techniques employed in the study of ERTS-1 data are summarized.

Applicability of ERTS for surveying Antarctic iceberg resources

NASA Technical Reports Server (NTRS)

Hult, J. L. (Principal Investigator); Ostrander, N. C.

1973-01-01

The author has identified the following significant results. This investigation explores the applicability of ERTS to (1) determine the Antarctic sea ice and environmental behavior that may influence the harvesting of icebergs, and (2) monitor iceberg locations, characteristics, and evolution. From image sampling, it is found that the potential applicability of ERTS to the research, planning, and harvesting operations can contribute importantly to the promise derived from broader scope studies for the use of Antarctic iceberg to relieve fresh Thermal sensor bands will provide coverage in daylight and darkness. Several years of comprehensive monitoring will be necessary to characterize sea ice and environmental behavior and iceberg evolution. Live ERTS services will assist harvesting control and claming operations and offer a means for harmonizing entitlements to iceberg resources. The valuable ERTS services will be more cost effective than other means and will be easily justified and borne by the iceberg harvesting operation.

Evaluation of ERTS data for certain oceanographic uses. [sunglint, algal bloom, water temperature, upwelling, and turbidity of Great Lakes waters

NASA Technical Reports Server (NTRS)

Strong, A. E. (Principal Investigator)

1973-01-01

The author has identified the following significant results. (1) Sunglint effects over water can be expected in ERTS-1 images whenever solar elevations exceed 55 deg. (2) Upwellings were viewed coincidently by ERTS-1 and NOAA-2 in Lake Michigan on two occasions during August 1973. (3) A large oil slick was identified 100 km off the Maryland coast in the Atlantic Ocean. Volume of the oil was estimated to be least 200,000 liters (50,000 gallons). (4) ERTS-1 observations of turbidity patterns in Lake St. Clair provide circulation information that correlates well with physical model studies made 10 years ago. (5) Good correlation has been established between ERTS-1 water color densities and NOAA-2 thermal infrared surface temperature measurements. Initial comparisons have been made in Lake Erie during March 1973.

Applicability of ERTS to Antarctic iceberg resources. [harvesting sea ice for fresh water

NASA Technical Reports Server (NTRS)

Hult, J. L. (Principal Investigator); Ostrander, N. C.

1973-01-01

The author has identified the following significant results. This investigation explorers the applicability of ERTS to (1) determine the Antarctic sea ice and environmental behavior that may influence the harvesting of icebergs, and (2) monitor iceberg locations, characteristics, and evolution. Imagery has shown that the potential applicability of ERTS to the research, planning, and harvesting operations can contribute importantly to the glowing promise derived from broader scope studies for the use of Antarctic icebergs to relieve a growing global thirst for fresh water. Several years of comprehensive monitoring will be necessary to characterize sea ice and environmental behavior and iceberg evolution. Live ERTS services will assist harvesting control and claiming operations and offer a means of harmonizing entitlements of iceberg resources. The valuable ERTS services will be more cost effective than other means will be easily justified and borne by the iceberg harvesting operations.

Automatic mapping of strip mine operations from spacecraft data. [Ohio

NASA Technical Reports Server (NTRS)

Rogers, R. H. (Principal Investigator); Reed, L. E.; Pettyjohn, W. A.

1974-01-01

The author has identified the following significant results. Computer techniques were applied to process ERTS tapes acquired over coal mining operations in southeastern Ohio on 21 August 1972 and 3 September 1973. ERTS products obtained included geometrically-correct map overlays, at scales from 1:24,000 to 1:250,000, showing stripped earth, partially reclaimed earth, water, and natural vegetation. Computer-generated tables listing the area covered by each land-water category in square kilometers were also produced. By comparing these mapping products, the study demonstrates the capability of ERTS to monitor changes in the extent of stripping and reclamation. NASA C-130 photography acquired on 7 September 1973 when compared with the ERTS products generated from the 3 September 1973 tape established the categorization accuracy to be better than 90%. It is estimated that the stripping and reclamation maps and data were produced from the ERTS CCTs at a tenth of the cost of conventional techniques.

Monitoring CO 2 sequestration into deep saline aquifer and associated salt intrusion using coupled multiphase flow modeling and time lapse electrical resistivity tomography

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chuan Lu; CHI Zhang; Hai Hanag

2014-04-01

Successful geological storage and sequestration of carbon dioxide (CO2) require efficient monitoring of the migration of CO2 plume during and after large-scale injection in order to verify the containment of the injected CO2 within the target formation and to evaluate potential leakage risk. Field studies have shown that surface and cross-borehole electrical resistivity tomography (ERT) can be a useful tool in imaging and characterizing solute transport in heterogeneous subsurface. In this synthetic study, we have coupled a 3-D multiphase flow model with a parallel 3-D time-lapse ERT inversion code to explore the feasibility of using time-lapse ERT for simultaneously monitoringmore » the migration of CO2 plume in deep saline formation and potential brine intrusion into shallow fresh water aquifer. Direct comparisons of the inverted CO2 plumes resulting from ERT with multiphase flow simulation results indicate the ERT could be used to delineate the migration of CO2 plume. Detailed comparisons on the locations, sizes and shapes of CO2 plume and intruded brine plumes suggest that ERT inversion tends to underestimate the area review of the CO2 plume, but overestimate the thickness and total volume of the CO2 plume. The total volume of intruded brine plumes is overestimated as well. However, all discrepancies remain within reasonable ranges. Our study suggests that time-lapse ERT is a useful monitoring tool in characterizing the movement of injected CO2 into deep saline aquifer and detecting potential brine intrusion under large-scale field injection conditions.« less

Fabry disease: multidisciplinary evaluation after 10 years of treatment with agalsidase Beta.

PubMed

Juan, Politei; Hernan, Amartino; Beatriz, Schenone Andrea; Gustavo, Cabrera; Antonio, Michref; Eduardo, Tanus; Raul, Dominguez; Margarita, Larralde; Mariana, Blanco; Daniela, Gaggioli; Marina, Szlago

2014-01-01

Fabry disease is an X linked disorder of metabolism due to deficient α-galactosidase A activity. Enzyme replacement therapy (ERT) with agalsidase Beta was approved by EMA in 2001 and FDA in 2003. Six patients were enrolled. Baseline data was measured for renal, cardiac, and cerebrovascular functioning. We compared baseline quality of life scales with the current results. These parameters were assessed during the 10 years of follow-up period. Before ERT four patients showed normal eGFR, one stage 2 of CKD, and one hyperfiltration stage. All presented microalbuminuria and just two cases showed proteinuria. After 10 years of ERT, no patient showed decrease in renal functioning. One patient decreased from proteinuria to microalbuminuria range. Before treatment one case showed left ventricular (LV) hypertrophy and LV Mass Index was abnormal in two female patients. After 10 years echocardiographic values did not present progression to LVH and one female showed regression to normal values of LV posterior wall and interventricular septum. Brain MRI showed ischemic lesions in one female and vertebrobasilar dolichoectasia in one male. From baseline and during the follow-up period MRI did not progress to new ischemic lesions and there were no clinical signs of cerebrovascular damage. After 10 years quality of life showed improvement in all domains measured. Early treatment of agalsidase Beta is related to a better outcome regarding stability and regression of signs and symptoms in Fabry disease. Our results in patients with mild organ involvement showed good outcomes and support an early and continuous ERT.

Nanoparticulation improves bioavailability of Erlotinib.

PubMed

Yang, Kyung Mi; Shin, In Chul; Park, Joo Won; Kim, Kab-Sig; Kim, Dae Kyong; Park, Kyungmoon; Kim, Kunhong

2017-09-01

Nanoparticulation using fat and supercritical fluid (NUFS TM ) is a drug delivery platform technology enabling efficient and effective formulation of poorly soluble drugs. We performed experiments to examine whether NUFS™ could improve poor bioavailability and reduce fed-fasted bioavailability variances of erlotinib (Ert). NUFS-Ert was prepared using NUFS™ technology; its physical properties were characterized, and drug release was measured. Furthermore, in vitro and in vivo efficacy tests and pharmacokinetic analysis were performed. NUFS-Ert nanoparticles had an average size of 250 nm and were stable for 2 months at 40 °C, 4 °C, and room temperature. The dissolution rate of NUFS-Ert increased in bio-relevant dissolution media. NUFS-Ert was more potent in inhibiting EGF signaling and in suppressing the proliferation of A549, a human non-small cell lung cancer cell line. Furthermore, A549 xenografts in BALB/c nude mice treated with NUFS-Ert regressed more efficiently than those in the mice treated with vehicle or Tarceva ® . In addition, experimental lung metastasis was more efficiently inhibited by NUFS-Ert than by Tarceva ® . The relative bioavailability of NUFS-Ert compared with that of Tarceva ® was 550% and the ratio of the area under the concentration-time curve (AUC) of fed state to the AUC of fasted state was 1.8 for NUFS-Ert and 5.8 for Tarceva ® . NUFS-Ert could improve poor bioavailability and reduce fed-fasted bioavailability variances of Ert. NUFS-Ert was more efficacious than Tarceva ® .

The use of ERTS imagery for lake classification. [turbidity due to phytoplankton

NASA Technical Reports Server (NTRS)

Scarpace, F. L.; Wade, R. E.; Fisher, L. T.

1975-01-01

The feasibility of using photographic representations of the ERTS imagery to classify lakes in the State of Wisconsin as to their trophic level was studied. Densitometric readings in band 5 of ERTS 70 mm imagery were taken for all the lakes in Wisconsin greater than 100 acres (approximately 1000 lakes). An algorithm has been developed from ground truth measurements to predict from satellite imagery an indicator of trophic status.

Smoking Cessation for Pregnant Smokers: Development and Pilot Test of an Emotion Regulation Treatment Supplement to Standard Smoking Cessation for Negative Affect Smokers.

PubMed

Bradizza, Clara M; Stasiewicz, Paul R; Zhuo, Yue; Ruszczyk, Melanie; Maisto, Stephen A; Lucke, Joseph F; Brandon, Thomas H; Eiden, Rina D; Slosman, Kim S; Giarratano, Paulette

2017-05-01

Negative affect has been identified as a factor influencing continued smoking during pregnancy. In this study, a multi-component emotion regulation intervention was developed to address negative emotional smoking triggers and pilot-tested among low-income pregnant smokers. Treatment feasibility and acceptability, cotinine-verified rates of smoking cessation, and self-report of mean cigarettes smoked were assessed. Pregnant smokers who self-reported smoking in response to negative affect (N = 70) were randomly assigned to receive one of two 8-session interventions: (1) emotion regulation treatment combined with standard cognitive-behavioral smoking cessation (ERT + CBT) or (2) a health and lifestyle plus standard smoking cessation active control (HLS + CBT). Outcomes for the 4-month period following the quit date are reported. Treatment attendance and subjective ratings provide evidence for the feasibility and acceptability of the ERT + CBT intervention. Compared with the HLS + CBT control condition, the ERT + CBT condition demonstrated higher abstinence rates at 2 months (ERT + CBT = 23% vs. HLS + CBT = 0%, OR = 13.51; 95% CI = 0.70-261.59) and 4 months (ERT = 18% vs. HLS = 5%; OR = 2.98; 95% CI = 0.39-22.72) post-quit. Mean number of cigarettes per day was significantly lower in ERT + CBT at 2 months (ERT + CBT = 2.73 (3.35) vs. HLS + CBT = 5.84 (6.24); p = .05) but not at 4 months (ERT + CBT = 2.15 (3.17) vs. HLS + CBT = 5.18 (2.88); p = .06) post-quit. The development and initial test of the ERT + CBT intervention supports its feasibility and acceptability in this difficult-to-treat population. Further development and testing in a Stage II randomized clinical trial are warranted. Negative affect has been identified as a motivator for continued smoking during pregnancy. To date, smoking cessation interventions for pregnant smokers have not specifically addressed the role of negative affect as a smoking trigger. This treatment development pilot study provides support for the feasibility and acceptability of a multi-component ERT + CBT for low-income pregnant smokers who self-report smoking in response to negative affect. Study findings support further testing in a fully-powered Stage II efficacy trial powered to assess mediators and moderators of treatment effects. © The Author 2017. Published by Oxford University Press on behalf of the Society for Research on Nicotine and Tobacco. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Use of ERTS data for a multidisciplinary analysis of Michigan resources. [forests, agriculture, soils, and landforms

NASA Technical Reports Server (NTRS)

Andersen, A. L.; Myers, W. L.; Safir, G.; Whiteside, E. P. (Principal Investigator)

1974-01-01

The author has identified the following significant results. The results of this investigation of ratioing simulated ERTS spectral bands and several non-ERTS bands (all collected by an airborne multispectral scanner) indicate that significant terrain information is available from band-ratio images. Ratio images, which are based on the relative spectral changes which occur from one band to another, are useful for enhancing differences and aiding the image interpreter in identifying and mapping the distribution of such terrain elements as seedling crops, all bare soil, organic soil, mineral soil, forest and woodlots, and marsh areas. In addition, the ratio technique may be useful for computer processing to obtain recognition images of large areas at lower costs than with statistical decision rules. The results of this study of ratio processing of aircraft MSS data will be useful for future processing and evaluation of ERTS-1 data for soil and landform studies. Additionally, the results of ratioing spectral bands other than those currently collected by ERTS-1 suggests that some other bands (particularly a thermal band) would be useful in future satellites.

Environmental study of ERTS-1 imagery: Lake Champlain and Vermont

NASA Technical Reports Server (NTRS)

Lind, A. O.; Henson, E. B.; Pelton, J. O.

1973-01-01

Environmental concerns of the State of Vermont currently being stressed include water quality in Lake Champlain and a state-wide land use and capability plan. Significant results obtained from ERTS-1 relate directly to the above concerns. Industrial water pollution and turbidity in Lake Champlain have been identified and mapped and the ERTS pollution data will be used in the developing court suit which Vermont has initiated against the polluters. ERTS imagery has also provided a foundation for updating and revising land use inventories. Major classes of land use have been identified and mapped, and substantial progress has been made toward the mapping of such land use divisions as crop and forest type, and wetlands.

Identification of irrigated crop types from ERTS-1 density contour maps and color infrared aerial photography. [Wyoming

NASA Technical Reports Server (NTRS)

Marrs, R. W.; Evans, M. A.

1974-01-01

The author has identified the following significant results. The crop types of a Great Plains study area were mapped from color infrared aerial photography. Each field was positively identified from field checks in the area. Enlarged (50x) density contour maps were constructed from three ERTS-1 images taken in the summer of 1973. The map interpreted from the aerial photography was compared to the density contour maps and the accuracy of the ERTS-1 density contour map interpretations were determined. Changes in the vegetation during the growing season and harvest periods were detectable on the ERTS-1 imagery. Density contouring aids in the detection of such charges.

A study of the break-up characteristics of Chena River Basin using ERTS imagery

NASA Technical Reports Server (NTRS)

Carlson, R. F. (Principal Investigator); Kane, D. L.; Wendler, G.

1973-01-01

The author has identified the following significant results. The Chena River Basin was selected because of the availability of ground truth data for comparison. Very good agreement for snow distribution and rates of ablation was found between the ERTS-1 imagery, the snowmelt model, and field measurements. Monitoring snowmelt rates for relatively small basins appears to be practical. The main limitation of the ERTS-1 imagery is the interval of coverage. More frequent overflights providing coverage are needed for the study of transient hydrologic events. ERTS-1 data is most useful when used in conjunction with snowmelt prediction models and existing snow course data. These results should prove very useful in preliminary assessment of hydrologic conditions in ungaged watersheds and will provide a tool for month-to-month volume forecasting.

Characterizing root system characteristics with Electrical resistivity Tomography: a virtual rhizotron simulation

NASA Astrophysics Data System (ADS)

Rao, Sathyanarayan; Ehosioke, Solomon; Lesparre, Nolwenn; Nguyen, Frédéric; Javaux, Mathieu

2017-04-01

Electrical Resistivity Tomography (ERT) is more and more used for monitoring soil water content in a cropped soil. Yet, the impact of roots on the signal is often neglected and a topic of controversy. In several studies related to soil-root system, it has been showed that the measured root mass density statistically correlates with the electrical conductivity (EC) data obtained from ERT. In addition, some studies suggest that some roots are more electrically conductive than soil for most water content. Thus, higher EC of roots suggest that it might have a measurable impact on ERT signals. In this work, virtual rhizotrons are simulated using the software package called R-SWMS that solves water and solute transport in plant root-soil system, including root growth. The distribution of water content obtained from R-SWMS simulation is converted into EC data using pedo-physical models. The electrical properties of roots and rhizosphere are explicitly included in the EC data to form a conductivity map (CM) with a very detailed spatial resolution. Forward ERT simulations is then carried out for CM generated for various root architectures and soil conditions to study the impact of roots on ERT forward (current and voltage patterns) and inverse solutions. It is demonstrated that under typical injection schemes with lateral electrodes, root system is hardly measurable. However, it is showed that adding electrodes and constraints on the ERT inversion based on root architecture help quantifying root system mass and extent.

Geologic and mineral and water resources investigations in western Colorado using ERTS-1 data

NASA Technical Reports Server (NTRS)

Knepper, D. H., Jr. (Compiler)

1973-01-01

The author has identified the following significant results. Geologic interpretation of ERTS-1 imagery is dependent on recognition of the distribution, continuity, trend, and geometry of key surface features. In the examination of ERTS-1 imagery, lithology must be interpreted largely from the geomorphic expression of the terrain. ERTS-1 imagery is extremely useful in detecting local structures. Most mapped structures are topographically-expressed. Consequently, ERTS-1 imagery acquired during mid-winter, when the solar illumination angle is low, provides the largest amount of structural information. Stereoscopic analyses of ERTS-1 images significantly aid geologic interpretation. Positive transparencies of ERTS-1 images (1:1,000,000) commonly contain more geologic information than can be adequately annotated during geologic interpretation.

Use of ERTS-1 data: Summary report of work on ten tasks. [solving natural resources and environmental quality problems using ERTS-1 MSS data

NASA Technical Reports Server (NTRS)

Thomson, F. J.; Polcyn, F. C.; Bryan, M. L.; Sattinger, I. J.; Malila, W. A.; Nalepka, R. F.; Wezernak, C. T.; Horvath, R.; Vincent, R. K. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Depth mapping's for a portion of Lake Michigan and at the Little Bahama Bank test site have been verified by use of navigation charts and on-site visits. A thirteen category recognition map of Yellowstone Park has been prepared. Model calculation of atmospheric effects for various altitudes have been prepared. Radar, SLAR, and ERTS-1 data for flooded areas of Monroe County, Michigan are being studied. Water bodies can be reliably recognized and mapped using maximum likelihood processing of ERTS-1 digital data. Wetland mapping has been accomplished by slicing of single band and/or ratio processing of two bands for a single observation date. Both analog and digital processing have been used to map the Lake Ontario basin using ERTS-1 data. Operating characteristic curves were developed for the proportion estimation algorithm to determine its performance in the measurement of surface water area. The signal in band MSS-5 was related to sediment content of waters by modelling approach and by relating surface measurements of water to processed ERTS data. Radiance anomalies in ERTS-1 data could be associated with the presence of oil on water in San Francisco Bay, but the anomalies were of the same order as those caused by variations in sediment concentration and tidal flushing.

Kansas environmental and resource study: A Great Plains model, tasks 1-6

NASA Technical Reports Server (NTRS)

Haralick, R. M.; Kanemasu, E. T.; Morain, S. A.; Yarger, H. L. (Principal Investigator); Ulaby, F. T.; Shanmugam, K. S.; Williams, D. L.; Mccauley, J. R.; Mcnaughton, J. L.

1972-01-01

There are no author identified significant results in this report. Environmental and resources investigations in Kansas utilizing ERTS-1 imagery are summarized for the following areas: (1) use of feature extraction techniqued for texture context information in ERTS imagery; (2) interpretation and automatic image enhancement; (3) water use, production, and disease detection and predictions for wheat; (4) ERTS-1 agricultural statistics; (5) monitoring fresh water resources; and (6) ground pattern analysis in the Great Plains.

Study of application of ERTS-A imagery to fracture-related mine safety hazards in the coal mining industry. [Indiana

NASA Technical Reports Server (NTRS)

Wier, C. E.; Wobber, F. J. (Principal Investigator); Russell, O. R.; Amato, R. V.; Leshendok, T.

1973-01-01

The author has identified the following significant results. The Mined Land Inventory map of Pike, Gibson, and Warrick Counties, Indiana, prepared from ERTS-1 imagery, was included in the 1973 Annual Report of the President's Council on Environmental Quality as an example of ERTS applications to mined lands. Increasing numbers of inquiries have been received from coal producing states and coal companies interested in the Indiana Program.

Evaluating four-dimensional time-lapse electrical resistivity tomography for monitoring DNAPL source zone remediation.

PubMed

Power, Christopher; Gerhard, Jason I; Karaoulis, Marios; Tsourlos, Panagiotis; Giannopoulos, Antonios

2014-07-01

Practical, non-invasive tools do not currently exist for mapping the remediation of dense non-aqueous phase liquids (DNAPLs). Electrical resistivity tomography (ERT) exhibits significant potential but has not yet become a practitioner's tool due to challenges in interpreting the survey results at real sites. This study explores the effectiveness of recently developed four-dimensional (4D, i.e., 3D space plus time) time-lapse surface ERT to monitor DNAPL source zone remediation. A laboratory experiment demonstrated the approach for mapping a changing NAPL distribution over time. A recently developed DNAPL-ERT numerical model was then employed to independently simulate the experiment, providing confidence that the DNAPL-ERT model is a reliable tool for simulating real systems. The numerical model was then used to evaluate the potential for this approach at the field scale. Four DNAPL source zones, exhibiting a range of complexity, were initially simulated, followed by modeled time-lapse ERT monitoring of complete DNAPL remediation by enhanced dissolution. 4D ERT inversion provided estimates of the regions of the source zone experiencing mass reduction with time. Results show that 4D time-lapse ERT has significant potential to map both the outline and the center of mass of the evolving treated portion of the source zone to within a few meters in each direction. In addition, the technique can provide a reasonable, albeit conservative, estimate of the DNAPL volume remediated with time: 25% underestimation in the upper 2m and up to 50% underestimation at late time between 2 and 4m depth. The technique is less reliable for identifying cleanup of DNAPL stringers outside the main DNAPL body. Overall, this study demonstrates that 4D time-lapse ERT has potential for mapping where and how quickly DNAPL mass changes in real time during site remediation. Copyright © 2014 Elsevier B.V. All rights reserved.

CNS-directed gene therapy for the treatment of neurologic and somatic mucopolysaccharidosis type II (Hunter syndrome).

PubMed

Motas, Sandra; Haurigot, Virginia; Garcia, Miguel; Marcó, Sara; Ribera, Albert; Roca, Carles; Sánchez, Xavier; Sánchez, Víctor; Molas, Maria; Bertolin, Joan; Maggioni, Luca; León, Xavier; Ruberte, Jesús; Bosch, Fatima

2016-06-16

Mucopolysaccharidosis type II (MPSII) is an X-linked lysosomal storage disease characterized by severe neurologic and somatic disease caused by deficiency of iduronate-2-sulfatase (IDS), an enzyme that catabolizes the glycosaminoglycans heparan and dermatan sulphate. Intravenous enzyme replacement therapy (ERT) currently constitutes the only approved therapeutic option for MPSII. However, the inability of recombinant IDS to efficiently cross the blood-brain barrier (BBB) limits ERT efficacy in treating neurological symptoms. Here, we report a gene therapy approach for MPSII through direct delivery of vectors to the CNS. Through a minimally invasive procedure, we administered adeno-associated virus vectors encoding IDS (AAV9- Ids ) to the cerebrospinal fluid of MPSII mice with already established disease. Treated mice showed a significant increase in IDS activity throughout the encephalon, with full resolution of lysosomal storage lesions, reversal of lysosomal dysfunction, normalization of brain transcriptomic signature, and disappearance of neuroinflammation. Moreover, our vector also transduced the liver, providing a peripheral source of therapeutic protein that corrected storage pathology in visceral organs, with evidence of cross-correction of nontransduced organs by circulating enzyme. Importantly, AAV9- Ids -treated MPSII mice showed normalization of behavioral deficits and considerably prolonged survival. These results provide a strong proof of concept for the clinical translation of our approach for the treatment of Hunter syndrome patients with cognitive impairment.

CNS-directed gene therapy for the treatment of neurologic and somatic mucopolysaccharidosis type II (Hunter syndrome)

PubMed Central

Motas, Sandra; Haurigot, Virginia; Garcia, Miguel; Marcó, Sara; Ribera, Albert; Roca, Carles; Sánchez, Víctor; Molas, Maria; Bertolin, Joan; Maggioni, Luca; León, Xavier; Ruberte, Jesús; Bosch, Fatima

2016-01-01

Mucopolysaccharidosis type II (MPSII) is an X-linked lysosomal storage disease characterized by severe neurologic and somatic disease caused by deficiency of iduronate-2-sulfatase (IDS), an enzyme that catabolizes the glycosaminoglycans heparan and dermatan sulphate. Intravenous enzyme replacement therapy (ERT) currently constitutes the only approved therapeutic option for MPSII. However, the inability of recombinant IDS to efficiently cross the blood-brain barrier (BBB) limits ERT efficacy in treating neurological symptoms. Here, we report a gene therapy approach for MPSII through direct delivery of vectors to the CNS. Through a minimally invasive procedure, we administered adeno-associated virus vectors encoding IDS (AAV9-Ids) to the cerebrospinal fluid of MPSII mice with already established disease. Treated mice showed a significant increase in IDS activity throughout the encephalon, with full resolution of lysosomal storage lesions, reversal of lysosomal dysfunction, normalization of brain transcriptomic signature, and disappearance of neuroinflammation. Moreover, our vector also transduced the liver, providing a peripheral source of therapeutic protein that corrected storage pathology in visceral organs, with evidence of cross-correction of nontransduced organs by circulating enzyme. Importantly, AAV9-Ids-treated MPSII mice showed normalization of behavioral deficits and considerably prolonged survival. These results provide a strong proof of concept for the clinical translation of our approach for the treatment of Hunter syndrome patients with cognitive impairment. PMID:27699273

Comparative evaluation of ERTS imagery for resource inventory in land use planning

NASA Technical Reports Server (NTRS)

Simonson, G. H. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Numerous previously unmapped faults in central Oregon have been distinguished on ERTS-1 imagery. Tectonic mapping of fault-controlled linears demonstrates the utility of ERTS-1 imagery as a mean of illustrating and studying the regional tectonics of the state. Soil colors observed on ERTS-1 frame 1075-18150-5 at the eastern end of the Columbia basin correlate very well with those from descriptions of soils from that area. Digital output from frame 1021-18151 has shown the enhanced ability to interpret such features as joint patterns, shadowed landslide blocks, bottomlands, and drainage patterns. Widespread use of wheat-fallow rotation in northern Umatilla County, Oregon, insures that nearly one-half of the cultivated soil is devoid of vegetation much of the time. On ERTS-1 imagery, fallow fields are only slightly darker than fields of wheat stubble at the western end of the transect. Similar climate-related contrasts in soil color are visible on ERTS-1 Imagery from several other portions of the Columbia Basin. Absence of steep topography in the area mentioned, however, minimizes the disturbing effect caused by shadows.

A study to explore the use of orbital remote sensing to determine native arid plant distribution

NASA Technical Reports Server (NTRS)

Mcginnies, W. G. (Principal Investigator); Haase, E. F.; Musick, H. B. (Compiler)

1973-01-01

The author has identified the following significant results. A theory has been developed of a method for determining the reflectivities of natural areas from ERTS-1 data. This method requires the following measurements: (1) ground truth reflectivity data from two different calibration areas; (2) radiance data from ERTS-1 MSS imagery for the same two calibration areas; and (3) radiance data from ERTS-1 MSS imagery for the area(s) in which reflectivity is to be determined. The method takes into account sun angle effects and atmospheric effects on the radiance seen by the space sensor. If certain assumptions are made, the ground truth data collection need not be simultaneous with the ERTS-1 overflight. The method allows the calculation of a conversion factor for converting ERTS-1 MSS radiance measurements of a given overflight to reflectivity values. This conversion factor can be used to determine the reflectivity of any area in the general vicinity of the calibration areas which has a relatively similar overlying atmosphere. This method, or some modification of it, may be useful in ERTS investigations which require the determination of spectral signatures of areas from spacecraft data.

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

PubMed

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.

Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID).

PubMed

Booth, Claire; Gaspar, H Bobby

2009-01-01

Adenosine deaminase deficiency (ADA) is a rare, inherited disorder of purine metabolism characterized by immunodeficiency, failure to thrive and metabolic abnormalities. A lack of the enzyme ADA allows accumulation of toxic metabolites causing defects of both cell mediated and humoral immunity leading to ADA severe combined immune deficiency (SCID), a condition that can be fatal in early infancy if left untreated. Hematopoietic stem cell transplant is curative but is dependent on a good donor match. Other therapeutic options include enzyme replacement therapy (ERT) with pegademase bovine (PEG-ADA) and more recently gene therapy. PEG-ADA has been used in over 150 patients worldwide and has allowed stabilization of patients awaiting more definitive treatment with hematopoietic stem cell transplant. It affords both metabolic detoxification and protective immune function with patients remaining clinically well, but immune reconstitution is often suboptimal and may not be long lived. We discuss the pharmacokinetics, immune reconstitution, effects on systemic disease and side effects of treatment with PEG-ADA. We also review the long-term outcome of patients receiving ERT and discuss the role of PEG-ADA in the management of infants and children with ADA-SCID, alongside other therapeutic options.

The New Biology of Estrogen-induced Apoptosis Applied to Treat and Prevent Breast Cancer

PubMed Central

Jordan, V Craig

2014-01-01

The successful use of high dose synthetic estrogens to treat post-menopausal metastatic breast cancer, is the first effective “chemical therapy” proven in clinical trial to treat any cancer. This review documents the clinical use of estrogen for breast cancer treatment or estrogen replacement therapy (ERT) for postmenopausal hysterectomized women which can either result in breast cancer cell growth or breast cancer regression. This has remained a paradox since the 1950s until the discovery of the new biology of estrogen induced apoptosis at the end of the 20th century. The key to triggering apoptosis with estrogen is the selection of breast cancer cell populations that are resistant to long term estrogen deprivation. However, through trial and error estrogen independent growth occurs. At the cellular level, estrogen induced apoptosis is dependent upon the presence of the estrogen receptor (ER) which can be blocked by non-steroidal or steroidal anti-estrogens. The shape of an estrogenic ligand programs the conformation of the ER complex which in turn can modulate estrogen induced apoptosis: class I planar estrogens (eg: estradiol) trigger apoptosis after 24 hours whereas class II angular estrogens (eg: bisphenol triphenylethylene) delay the process until after 72 hours. This contrasts with paclitaxel that causes G2 blockade with immediate apoptosis. The process is complete within 24 hours. Estrogen induced apoptosis is modulated by glucocorticoids and cSrc inhibitors but the target mechanism for estrogen action is genomic and not through a non-genomic pathway. The process is step wise through the creation of endoplasmic reticulum stress and, inflammatory responses that then initiate an unfolded protein response. This in turn initiates apoptosis through the intrinsic pathway (mitochondrial) with subsequent recruitment of the extrinsic pathway (death receptor) to complete the process. The symmetry of the clinical and laboratory studies now permits the creation of rules for the future clinical application of ERT or phytoestrogen supplements: a five year gap is necessary after menopause to permit the selection of estrogen deprived breast cancer cell populations to become vulnerable to apoptotic cell death. Earlier treatment with estrogen around the menopause encourages ER positive tumor cell growth, as the cells are still dependent on estrogen to maintain replication within the expanding population. An awareness of the evidence that the molecular events associated with estrogen induced apoptosis can be orchestrated in the laboratory in estrogen deprived breast cancers, now support the clinical findings for the treatment of metastatic breast cancer following estrogen deprivation, decreases in mortality following long term antihormonal adjuvant therapy, and the results of ERT and ERT plus progestin in the Women’s Health Initiative for women over the age of 60. Principles have emerged to understand and apply physiologic estrogen therapy appropriately by targeting the correct patient populations. PMID:25339261

Equine-related treatments for mental disorders lack empirical support: a systematic review of empirical investigations.

PubMed

Anestis, Michael D; Anestis, Joye C; Zawilinski, Laci L; Hopkins, Tiffany A; Lilienfeld, Scott O

2014-12-01

Equine-related treatments (ERT) for mental disorders are becoming increasingly popular for a variety of diagnoses; however, they have been subjected only to limited systematic investigation. To examine the quality of and results from peer-reviewed research on ERT for mental disorders and related outcomes. Peer-reviewed studies (k = 14) examining treatments for mental disorders or closely related outcomes were identified from databases and article reference sections. All studies were compromised by a substantial number of threats to validity, calling into question the meaning and clinical significance of their findings. Additionally, studies failed to provide consistent evidence that ERT is superior to the mere passage of time in the treatment of any mental disorder. The current evidence base does not justify the marketing and utilization of ERT for mental disorders. Such services should not be offered to the public unless and until well-designed studies provide evidence that justify different conclusions. © 2014 Wiley Periodicals, Inc.

Artic and subarctic environmental analyses utilizing ERTS-1 imagery. Cold regions environmental analysis based on ERTS-1 imagery (preprint)

NASA Technical Reports Server (NTRS)

Anderson, D. M. (Principal Investigator); Haugen, R. K.; Gatto, L. W.; Slaughter, C. W.; Marlar, T. L.; Mckim, H. L.

1972-01-01

There are no author-identified significant results in this report. An overriding problem in arctic and subarctic environmental research has been the absence of long-term observational data and the sparseness of geographical coverage of existing data. A first look report is presented on the use of ERTS-1 imagery as a major tool in two large area environmental studies: (1) investigation of sedimentation and other nearshore marine processes in Cook Inlet, Alaska; and (2) a regional study of permafrost regimes in the discontinuous permafrost zone of Alaska. These studies incorporate ground truth acquisition techniques that are probably similar to most ERTS investigations. Studies of oceanographic processes in Cook Inlet will be focused on seasonal changes in nearshore bathymetry, tidal and major current circulation patterns, and coastal sedimentation processes, applicable to navigation, construction, and maintenance of harbors. Analyses will be made of the regional permafrost distribution and regimes in the Upper Koyukuk-Kobuk River area located in NW Alaska.

Monitoring coastal water properties and current circulation with ERTS-1. [Delaware Bay

NASA Technical Reports Server (NTRS)

Klemas, V.; Otley, M.; Wethe, C.; Rogers, R.

1974-01-01

Imagery and digital tapes from nine successful ERTS-1 passes over Delaware Bay during different portions of the tidal cycle have been analyzed with special emphasis on turbidity, current circulation, waste disposal plumes and convergent boundaries between different water masses. ERTS-1 image radiance correlated well with Secchi depth and suspended sediment concentration. Circulation patterns observed by ERTS-1 during different parts of the tidal cycle, agreed well with predicted and measured currents throughout Delaware Bay. Convergent shear boundaries between different water masses were observed from ERTS-1. In several ERTS-1 frames, waste disposal plumes have been detected 36 miles off Delaware's Atlantic coast. The ERTS-1 results are being used to extend and verify hydrodynamic models of the bay, developed for predicting oil slick movement and estimating sediment transport.

The ERTS-1 investigation (ER-600): A compendium of analysis results of the utility of ERTS-1 data for land resources management

NASA Technical Reports Server (NTRS)

Erb, R. B.

1974-01-01

The results of the ERTS-1 investigations conducted by the Earth Observations Division at the NASA Lyndon B. Johnson Space Center are summarized in this report, which is an overview of documents detailing individual investigations. Conventional image interpretation and computer-aided classification procedures were the two basic techniques used in analyzing the data for detecting, identifying, locating, and measuring surface features related to earth resources. Data from the ERTS-1 multispectral scanner system were useful for all applications studied, which included agriculture, coastal and estuarine analysis, forestry, range, land use and urban land use, and signature extension. Percentage classification accuracies are cited for the conventional and computer-aided techniques.

Evaluation of ERTS-1 data for certain hydrological uses

NASA Technical Reports Server (NTRS)

Wiesnet, D. R. (Principal Investigator); Mcginnis, D. F.; Mcmillan, M. C.

1974-01-01

The author has identified the following significant results. ERTS-1 MSS data have been used in a variety of hydrologic research including snow-extent mapping; studies of snowmelt, snowmelt runoff, spectral reflectance of snow for assessing snowpack conditions, and snow albedo; lake ice formation, breakup, and migration; lake current measurements; multispectral studies of lake ice; and flood studies. MSS sensing of soil moisture over a well-vegetated test site was unsuccessfully attempted. Although a powerful research tool, ERTS-1 has very limited use as an operational system for hydrologic communities because of its 18-day revisit cycle and its lack of a quick look capability.

Effects of switching from agalsidase Beta to agalsidase alfa in 10 patients with anderson-fabry disease.

PubMed

Pisani, A; Spinelli, L; Visciano, B; Capuano, I; Sabbatini, M; Riccio, E; Messalli, G; Imbriaco, M

2013-01-01

Anderson-Fabry disease (AFD) is a multiorgan X-linked lysosomal storage disease that particularly affects the heart, kidneys, and cerebrovascular system. Current treatment is enzyme replacement therapy (ERT) with agalsidase beta (Fabrazyme(®), Genzyme Corporation, Cambridge, MA, USA) or agalsidase alfa (Replagal(®), Shire Human Genetic Therapies AB, Lund, Sweden). It was recommended that patients switch to agalsidase alfa due to a manufacturing shortage of agalsidase beta beginning in June 2009. This study assessed the effect of switching to agalsidase alfa on clinical outcomes in patients with AFD previously treated with agalsidase beta. Ten patients (seven male, three female) with genetically confirmed AFD and at least 48 months' continuous data collected during treatment with agalsidase beta 1 mg/kg every other week were switched to agalsidase alfa 0.2 mg/kg every other week for at least 20 months, with prospective clinical evaluations every 6 months. Pre-switch data was collected retrospectively from patient charts. Cardiac functional parameters were assessed using magnetic resonance imaging. Results showed that renal function was normal (estimated glomerular filtration rate ≥90 mL/min/1.73 m(2)) in 8 of 10 patients prior to agalsidase alfa and generally remained stable after the switch. Cardiac mass decreased significantly (p < 0.05 vs pre-ERT) after agalsidase beta and remained unchanged after switching to agalsidase alfa. Symptoms of pain and health status scores did not deteriorate during agalsidase alfa therapy. Adverse events were mostly mild and infusion related. In conclusion, switching to agalsidase alfa was relatively well tolerated and associated with stable clinical status and preserved renal and cardiac function.

The Effect of Neonatal Gene Therapy with a Gamma Retroviral Vector on Cardiac Valve Disease in Mucopolysaccharidosis VII Dogs after a Decade

PubMed Central

Bigg, Paul W.; Sleeper, Meg M.; O’Donnell, Patricia A.; Liu, Yuli; Wu, Susan; Casal, Margret L.; Haskins, Mark E.; Ponder, Katherine P.

2013-01-01

Mucopolysaccharidosis VII (MPS VII) is due to deficient activity of the lysosomal enzyme β-glucuronidase (GUSB) and results in the accumulation of glycosaminoglycans (GAGs). This study determined the long-term effect of neonatal intravenous injection of a gamma retroviral vector (RV) on cardiac valve disease in MPS VII dogs. Transduced hepatocytes secreted GUSB into blood for up to 11 years at levels similar to or greater than those achieved with enzyme replacement therapy (ERT). Valve regurgitation and thickening were scored from 0 (normal) to +4 (severely abnormal). At 1 year, untreated MPS VII dogs had mitral regurgitation, mitral valve thickening, aortic regurgitation, and aortic valve thickening scores of 2.3±0.7, 2.3±0.6, 1.8±0.5, and 1.6±0.7, respectively, which were higher than the values of 0.6±0.1, 0.1±0.4, 0.3±0.8, and 0.1±0.4, respectively, in treated MPS VII dogs. Treated MPS VII dogs maintained low aortic regurgitation and aortic valve thickening scores for their lifetime. Although mitral regurgitation and mitral valve thickening scores increased to 2.0 at ≥8 years of age in the treated MPS VII dogs, older normal dogs from the colony had similar scores, making it difficult to assess mitral valve disease. Older treated dogs had calcification within the mitral and aortic valve annulus, while GUSB staining demonstrated enzyme activity within the mitral valve. We conclude that neonatal RV-mediated gene therapy reduced cardiac valve disease in MPS VII dogs for up to 11 years, and propose that neonatal initiation of ERT should have a similar effect. PMID:23860311

The effect of neonatal gene therapy with a gamma retroviral vector on cardiac valve disease in mucopolysaccharidosis VII dogs after a decade.

PubMed

Bigg, Paul W; Sleeper, Meg M; O'Donnell, Patricia A; Liu, Yuli; Wu, Susan; Casal, Margret L; Haskins, Mark E; Ponder, Katherine P

2013-11-01

Mucopolysaccharidosis VII (MPS VII) is due to deficient activity of the lysosomal enzyme β-glucuronidase (GUSB) and results in the accumulation of glycosaminoglycans (GAGs). This study determined the long-term effect of neonatal intravenous injection of a gamma retroviral vector (RV) on cardiac valve disease in MPS VII dogs. Transduced hepatocytes secreted GUSB into the blood for up to 11 years at levels similar to or greater than those achieved with enzyme replacement therapy (ERT). Valve regurgitation and thickening were scored from 0 (normal) to +4 (severely abnormal). At 1 year, untreated MPS VII dogs had mitral regurgitation, mitral valve thickening, aortic regurgitation, and aortic valve thickening scores of 2.3 ± 0.7, 2.3 ± 0.6, 1.8 ± 0.5, and 1.6 ± 0.7, respectively, which were higher than the values of 0.6 ± 0.1, 0.1 ± 0.4, 0.3 ± 0.8, and 0.1 ± 0.4, respectively, in treated MPS VII dogs. Treated MPS VII dogs maintained low aortic regurgitation and aortic valve thickening scores in their lifetime. Although mitral regurgitation and mitral valve thickening scores increased to 2.0 at ≥ 8 years of age in the treated MPS VII dogs, older normal dogs from the colony had similar scores, making it difficult to assess mitral valve disease. Older treated dogs had calcification within the mitral and the aortic valve annulus, while GUSB staining demonstrated enzyme activity within the mitral valve. We conclude that neonatal RV-mediated gene therapy reduced cardiac valve disease in MPS VII dogs for up to 11 years, and propose that neonatal initiation of ERT should have a similar effect. © 2013.

Task 1: Correlation of satellite and ground data in air pollution studies. Task 2: Investigation to relate the chlorophyll and suspended sediment content in the waters of the lower Chesapeake Bay to ERTS-1 imagery. Task 3: The use of ERTS-1 to more fully utilize and apply marine station data to the study of productivity along the Eastern Shelf expanded waters of the United States

NASA Technical Reports Server (NTRS)

Copeland, G. E. (Principal Investigator); Bandy, A. R.; Fleischer, P.; Ludwick, J. C. (Principal Investigator); Hanna, W. J.; Gosink, T. A.; Bowker, D. W.; Marshall, H. G. (Principal Investigator)

1972-01-01

The author has identified the following significant results. Analysis of U-2 imagery of CARETS site indicates smoke plumes can be easily detected. First look at selected ERTS-1 color composites demonstrates plumes from forest fires can be detected.

ERTS-1 data applied to strip mining

NASA Technical Reports Server (NTRS)

Anderson, A. T.; Schubert, J.

1976-01-01

Two coal basins within the western region of the Potomac River Basin contain the largest strip-mining operations in western Maryland and West Virginia. The disturbed strip-mine areas were delineated along with the surrounding geological and vegetation features by using ERTS-1 data in both analog and digital form. The two digital systems employed were (1) the ERTS analysis system, a point-by-point digital analysis of spectral signatures based on known spectral values and (2) the LARS automatic data processing system. These two systems aided in efforts to determine the extent and state of strip mining in this region. Aircraft data, ground-verification information, and geological field studies also aided in the application of ERTS-1 imagery to perform an integrated analysis that assessed the adverse effects of strip mining. The results indicated that ERTS can both monitor and map the extent of strip mining to determine immediately the acreage affected and to indicate where future reclamation and revegetation may be necessary.

The use of ERTS-1 to more fully utilize and apply marine station data to the study and productivity along the eastern shelf waters of the United States

NASA Technical Reports Server (NTRS)

Marshall, H. G. (Principal Investigator); Bowker, D. E.; Witte, W. G.

1976-01-01

The author has identified the following significant results. Sea truth data were obtained during two ERTS overpasses in waters near the entrance of the Chesapeake Bay. Correlations were made between total phytoplankton and chlorophyll values in these waters to radiance detected by ERTS in an effort to map areas of similar productivity levels. Band 4 radiance had the highest correlation to all parameters with bands 5 and 6 showing decreasing correlations in each case. The radiance values were apparently influenced by one or more factors, most likely including the sediment content of the water. Data have shown that ERTS MSS is not suitable for monitoring chlorophyll in near-shore waters where sediment loads are high. It is suggested that in more seaward or pelagic locations, that ERTS MSS would be more efficient in monitoring surface chlorophyll values and establishing direct relationships to phytoplankton concentrations.

Principles of cost-benefit analysis for ERTS experiments, volumes 1 and 2

NASA Technical Reports Server (NTRS)

1973-01-01

The basic elements of a cost-benefit study are discussed along with special considerations for ERTS experiments. Elements required for a complete economic analysis of ERTS are considered to be: statement of objectives, specification of assumptions, enumeration of system alternatives, benefit analysis, cost analysis nonefficiency considerations, and final system selection. A hypothetical cost-benefit example is presented with the assumed objective of an increase in remote sensing surveys of grazing lands to better utilize available forage to lower meat prices.

The interdependence of lake ice and climate in central North America

NASA Technical Reports Server (NTRS)

Jelacic, A. J. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. This investigation is to identify any correlations between the freeze/ thaw cycles of lakes and regional weather variations. ERTS-1 imagery of central Canada and north central United States is examined on a seasonal basis. The ice conditions of certain major study lakes are noted and recorded on magnetic tape, from which the movement of a freeze/thaw transition zone may be deduced. Weather maps and tables are used to establish any obvious correlations. The process of selecting major study lakes is discussed, and a complete lake directory is presented. Various routines of the software support library are described, accompanied by output samples. Procedures used for ERTS imagery processing are presented along with the data analysis plan. Application of these procedures to selected ERTS imagery has demonstrated their utility. Preliminary results show that the freeze/thaw transition zone can be monitored from ERTS.

Summary of an integrated ERTS-1 project and its results at the Missouri Geological Survey

NASA Technical Reports Server (NTRS)

Martin, J. A.; Allen, W. H.; Rath, D. L.; Rueff, A.

1974-01-01

Use of the ERTS imagery involved the recognition and interpretation of various ground patterns. Analysis and application are tied to ongoing programs. Specific studies utilizing the imagery and NASA aircraft photography are: a statewide lake and dam inventory; assessment of flooding and floodprone areas along the Missouri portion of the Mississippi and Missouri Rivers; land-use classification for several counties; structural features in selected areas; and Pleistocene features in northern Missouri. Though it has been suggested that repetitive coverage is not necessary for geologic studies, it is this specific feature along with the synoptic view of large portions of the State that provided the potential for the utilization of the ERTS imagery in Missouri. Other State agencies, Departments of Conservation, Agriculture, and Community Affairs, have expressed interest in the potential application of ERTS data in their respective fields.

An operational application of ERTS-1 imagery to the environmental inventory process. [environmental impact statement for Atchafalaya basin of Louisiana and Mississippi

NASA Technical Reports Server (NTRS)

Oneal, J. D.; Bwins, J. R.

1974-01-01

A discussion is presented of how The Engineer Agency for Resources Inventories utilized ERTS remotely sensed imagery as a/supplement to airphotos and collateral data for an environmental impact study of the Atchafalaya Basin for the New Orleans engineer district. This single overall inventory permitted a systematic approach and substantial economy for a number of engineering projects for which environmental impact statements would be required. The study area covered approximately 25,000 square miles and included all or part of 36 parishes in Louisiana and 8 counties in Mississippi. It was concluded that as a supplementary source, used in conjunction with airphotos, ERTS had proven itself as a significant means of economy. As a primary source, the Engineer Agency is not yet ready to accept ERTS for their own particular applications.

Single and Concurrent Effects of Endurance and Resistance Training on Pulmonary Function

PubMed Central

Khosravi, Maryam; Tayebi, Seyed Morteza; Safari, Hamed

2013-01-01

Objective(s): As not only few evidences but also contradictory results exist with regard to the effects of resistance training (RT) and resistance plus endurance training (ERT) on respiratory system, so the purpose of this research was therefore to study single and concurrent effects of endurance and resistance training on pulmonary function. Materials and Methods: Thirty seven volunteer healthy inactive women were randomly divided into 4 groups: without training as control (C), Endurance Training (ET), RT, and ERT. A spirometry test was taken 24 hrs before and after the training course. The training period (8 weeks, 3 sessions/week) for ET was 20-26 min/session running with 60-80% maximum heart rate (HR max); for RT two circuits/session, 40-60s for each exercise with 60-80% one repetition maximum (1RM), and 1 and 3 minutes active rest between exercises and circuits respectively; and for ERT was in agreement with either ET or RT protocols, but the times of running and circuits were half of ET and RT. Results: ANCOVA showed that ET and ERT increased significantly (P< 0.05) vital capacity (VC), forced vital capacity (FVC), and forced expiratory flows to 25%-75%; ET, RT and ERT increased significantly (P< 0.05) maximum voluntary ventilation (MVV); and only ET increased significantly (P<0.05) peak expiratory flows (PEF); but ET, RT and ERT had no significant effect (P>0.05) on forced expiratory volume in one second (FEV1) and FEV1/FVC ratio. Conclusion: In conclusion, ET combined with RT (ERT) has greater effect on VC, FVC, FEF rating at25%-75%, and also on PEF except MVV, rather than RT, and just ET has greater effect rather than ERT. PMID:24250940

Analysis of ERTS-1 imagery of Wyoming and its application to evaluation of Wyoming's natural resources

NASA Technical Reports Server (NTRS)

Houston, R. S. (Principal Investigator); Marrs, R. W.; Borgman, L. E.; Agard, S. S.; Barton, R.; Blackstone, D. L.; Breckenridge, R. M.; Decker, E. R.; Earle, J.; Evans, M. A.

1975-01-01

The author has identified the following significant results. The Earth Resources Technology Satellite data included the following successful applications: (1) general geologic mapping, (2) structural and tectonic studies, (3) landforms and surface processes, (4) mineral exploration, (5) land use inventories, (6) hydrologic studies, (7) investigations in agriculture and forestry, and (8) environmental quality and ecology. The chief advantages of ERTS-1 data for geologic studies are synoptic view, spectral information, and seasonal coverage. The spectral data and repetitive aspect are also important for land use and vegetation studies. Low resolution and lack of steoscopic coverage were found to be the main limitations of ERTS data.

Evaluation of ERTS-1 imagery for mapping Quaternary deposits and landforms in the Great Plains and Midwest

NASA Technical Reports Server (NTRS)

Morrison, R. B. (Principal Investigator); Hallberg, G. R.

1973-01-01

The author has identified the following significant results. The main landform associations and larger landforms are readily identifiable on the better images and commonly the gross associations of surficial Quaternary deposits also can be differentiated, primarily by information on landforms and soils. Maps showing the Quaternary geologic-terrain units that can be differentiated from the ERTS-1 images are being prepared for study areas in Illinois, Iowa, Missouri, Kansas, Nebraska, and South Dakota. Preliminary maps at 1:1 million scale are given of two of the study areas, the Peoria and Decatur, Illinois, 1 deg x 2 quadrangles. These maps exemplify the first phase of investigations, which consists of identifying and mapping landform and land use characteristics and geologic-surficial materials directly from ERTS-1 images alone, without input of additional data. These maps shown that commonly the boundaries of geologic-terrain units can be identified more accurately on ERTS-1 images than on topographic maps of 1:250,000 scale. From analysis of drainage patterns, stream-divide relations, and tone and textural variations on the ERTS-1 images, the trends of numerous moraines of Wisconsinan and possibly some of Illinoian age were mapped. In the Peoria study area the trend of a buried valley of the Mississippi River is revealed.

Integrated electromagnetic (EM) and Electrical Resistivity Tomography (ERT) geophysical studies of environmental impact of Awotan dumpsite in Ibadan, southwestern Nigeria

NASA Astrophysics Data System (ADS)

Osinowo, Olawale Olakunle; Falufosi, Michael Oluseyi; Omiyale, Eniola Oluwatosin

2018-04-01

This study attempts to establish the level of contamination caused by the decomposition of wastes by defining the lateral distribution and the vertical limit of leachate induced zone of anomalous conductivity distribution within the subsurface through the analyses of Electromagnetic (EM) and Electrical Resistivity Tomography (ERT) data, generated from the integrated geophysical survey over Awotan landfill dumpsite, in Ibadan, southwest Nigeria. Nine (9) EM and ERT profiles each were established within and around the Awotan landfill site. EM data were acquire at 5 m station interval using 10 m, 20 m and 40 m inter-coil spacings, while ERT stations were occupied at 2 m electrode spacing using dipole-dipole electrode configuration. The near perfect agreement between the two sets of data generated from the EM and ERT surveys over the Awotan landfill site as well as the subsurface imaging ability of these geophysical methods to delineate the region of elevated contamination presented in the form of anomalously high apparent ground conductivity and low subsurface resistivity distribution, suggest the importance of integrating electromagnetic and electrical resistivity investigation techniques for environmental studies and more importantly for selecting appropriate landfill dump site location such with ability to retain the generated contaminants and thus prevent environmental pollution.

Baseline characteristics and outcome in Romanian patients with Gaucher disease type 1.

PubMed

Grigorescu-Sido, Paula; Drugan, Cristina; Alkhzouz, Camelia; Zimmermann, Anca; Coldea, Cristina; Denes, Carmen; Grigorescu, Mircea Dan; Cret, Victoria; Bucerzan, Simona

2010-04-01

To present clinical and genetic characteristics of all Romanian patients with Gaucher disease type 1, in whom specific diagnosis has been confirmed by enzymatic and molecular methods and to analyze their outcome with and without enzymatic replacement therapy (ERT). There are fifty patients (F/M - 1.63/1) with Gaucher disease type 1. Clinical status, haemoglobin, thrombocytes, hepatic/splenic volume, bone mineral density and severity score were assessed at baseline and every six months thereafter. Thirty-nine patients (78%) received imiglucerase (44.4+/-13.6 U/kg/2 weeks) for 3.1+/-1.4 years. Based on general prevalence data, our group represents 22.7% of the expected total number of patients with Gaucher disease type 1 in Romania. Mean age was 15.5 years at clinical onset and 28.9 years at confirmation of diagnosis. The genotype N370S/L444P was frequent in our group (35.9% of alleles). Anaemia, thrombocytopenia, splenomegaly and bone disease were present at 38%, 70%, 100% and 84%, respectively. Mean values for haemoglobin, thrombocytes, hepatic volume and chitotriosidase normalized after 0.5, 1.5, 2.5 and 3 years of ERT, respectively. Splenomegaly regressed from 14.4 x N (normal) to 3.06 x N over four years of treatment. Bone disease was ameliorated under ERT, yet bone mineral density worsened in patients treated with 30 U/kg/2 weeks. The genotype N370S/L444P is frequent in our patients, in line with the severe phenotypes. ERT improved haematological parameters and visceromegaly, without a clear benefit for bone mineral density. To attain therapeutic goals, an early treatment start with optimal dosage is mandatory. Copyright 2009 Elsevier B.V. All rights reserved.

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

PubMed

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.

Imaging of Ground Ice with Surface-Based Geophysics

DTIC Science & Technology

2015-10-01

terrains. Electrical Resistivity Tomography (ERT), in particular, has been effective for imaging ground ice. ERT measures the ability of materials to...13 2.2.1 Electrical resistivity tomography (ERT...Engineer Research and Development Center ERT Electrical Resistivity Tomography GPS Global Positioning System LiDAR Light Detection and Ranging SIPRE

ERTS-1 applied for structural and morphological investigtions case studies. 1: Los Angeles, California. 2: Coastal plain, New Jersey

NASA Technical Reports Server (NTRS)

Kedar, E. Y.

1973-01-01

Two major earth's resources management problems, the application of ERTS-1 imagery for geomorphotectonics, and subsequently seismic-risk, earthquake, and mineral exploration applications are discussed. Case studies are presented for Los Angeles, California, and New Jersey coastal plain.

Utilization of remotely-sensed data in the management of inland wetlands

NASA Technical Reports Server (NTRS)

Carter, V.; Smith, D. G.

1973-01-01

The author has identified the following significant results. ERTS-1 data and aerial photography are proving to be a useful tool for the inventory and management of inland wetlands. Two examples of the application of remotely-sensed data to specific wetland management needs or requirements are discussed. Studies of the Great Dismal Swamp are utilizing ERTS-1 imagery and color IR photography in: (1) study area selection; (2) field inspection; (3) vegetation mapping; (4) identification of drainage characteristics and moisture regime; (5) location of intensive study areas; and (6) detection of change. Thematic extractions of ERTS-1 data made using the United States Geological Survey's Autographic Theme Extraction System are aiding analyses of swamp hydrologic regime and providing information pertinent to quick recognition and inventory of wetlands from ERTS-1. DCP'S in south Florida wetlands provide near-real time data for water resources managers. Data relayed by satellite can be entered into models to provide predictive data and water storage information for long-term and short-term decision making.

Copackaged AAV9 Vectors Promote Simultaneous Immune Tolerance and Phenotypic Correction of Pompe Disease

PubMed Central

Doerfler, Phillip A.; Todd, Adrian G.; Clément, Nathalie; Falk, Darin J.; Nayak, Sushrusha; Herzog, Roland W.; Byrne, Barry J.

2016-01-01

Pompe disease is a progressive neuromuscular disorder caused by lysosomal accumulation of glycogen from a deficiency in acid alpha-glucosidase (GAA). Replacement of the missing enzyme is available by repeated protein infusions; however, efficacy is limited by immune response and inability to restore enzymatic function in the central nervous system. An alternative therapeutic option is adeno-associated virus (AAV)-mediated gene therapy, which results in widespread gene transfer and prolonged transgene expression. Both enzyme replacement therapy (ERT) and gene therapy can elicit anti-GAA immune reactions that dampen their effectiveness and pose life-threatening risks to patient safety. To modulate the immune responses related to gene therapy, we show that a human codon-optimized GAA (coGAA) driven by a liver-specific promoter (LSP) using AAV9 is capable of promoting immune tolerance in a Gaa−/− mouse model. Copackaging AAV9-LSP-coGAA with the tissue-restricted desmin promoter (AAV9-DES-coGAA) demonstrates the necessary cell autonomous expression in cardiac muscle, skeletal muscle, peripheral nerve, and the spinal cord. Simultaneous high-level expression in liver led to the expansion of GAA-specific regulatory T-cells (Tregs) and induction of immune tolerance. Transfer of Tregs into naïve recipients prevented pathogenic allergic reactions after repeated ERT challenges. Copackaged AAV9 also attenuated preexisting humoral and cellular immune responses, which enhanced the biochemical correction. Our data present a therapeutic design in which simultaneous administration of two copackaged AAV constructs may provide therapeutic benefit and resolve immune reactions in the treatment of multisystem disorders. PMID:26603344

Toward reducing immunogenicity of enzyme replacement therapy: altering the specificity of human β-glucuronidase to compensate for α-iduronidase deficiency.

PubMed

Chuang, Huai-Yao; Suen, Ching-Shu; Hwang, Ming-Jing; Roffler, Steve R

2015-11-01

Enzyme replacement therapy (ERT) is an effective treatment for many patients with lysosomal storage disorders caused by deficiency in enzymes involved in cell metabolism. However, immune responses that develop against the administered enzyme in some patients can hinder therapeutic efficacy and cause serious side effects. Here we investigated the feasibility of a general approach to decrease ERT immunogenicity by altering the specificity of a normal endogenous enzyme to compensate for a defective enzyme. We sought to identify human β-glucuronidase variants that display α-iduronidase activity, which is defective in mucopolysaccharidosis (MPS) type I patients. A human β-glucuronidase library was screened by the Enzyme Cleavable Surface-Tethered All-purpose Screen sYstem to isolate variants that exhibited 100-290-fold greater activity against the α-iduronidase substrate 4-methylumbelliferyl α-l-iduronide and 7900-24 500-fold enzymatic specificity shift when compared with wild-type β-glucuronidase. In vitro treatment of MPS I cells with the β-glucuronidase variants significantly restored lysosome appearance similar to treatment with α-iduronidase. Our study suggests that β-glucuronidase variants can be isolated to display α-iduronidase activity and produce significant phenotype improvement of MPS I cells. This strategy may represent a possible approach to produce enzymes that display therapeutic benefits with potentially less immunogenicity. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

Joint inversion of lake-floor electrical resistivity tomography and boat-towed radio-magnetotelluric data illustrated on synthetic data and an application from the Äspö Hard Rock Laboratory site, Sweden

NASA Astrophysics Data System (ADS)

Wang, Shunguo; Kalscheuer, Thomas; Bastani, Mehrdad; Malehmir, Alireza; Pedersen, Laust B.; Dahlin, Torleif; Meqbel, Naser

2018-04-01

The electrical resistivity tomography (ERT) method provides moderately good constraints for both conductive and resistive structures, while the radio-magnetotelluric (RMT) method is well suited to constrain conductive structures. Additionally, RMT and ERT data may have different target coverage and are differently affected by various types of noise. Hence, joint inversion of RMT and ERT data sets may provide a better constrained model as compared to individual inversions. In this study, joint inversion of boat-towed RMT and lake-floor ERT data has for the first time been formulated and implemented. The implementation was tested on both synthetic and field data sets incorporating RMT transverse electrical mode and ERT data. Results from synthetic data demonstrate that the joint inversion yields models with better resolution compared with individual inversions. A case study from an area adjacent to the Äspö Hard Rock Laboratory (HRL) in southeastern Sweden was used to demonstrate the implementation of the method. A 790-m-long profile comprising lake-floor ERT and boat-towed RMT data combined with partial land data was used for this purpose. Joint inversions with and without weighting (applied to different data sets, vertical and horizontal model smoothness) as well as constrained joint inversions incorporating bathymetry data and water resistivity measurements were performed. The resulting models delineate subsurface structures such as a major northeasterly directed fracture system, which is observed in the HRL facility underground and confirmed by boreholes. A previously uncertain weakness zone, likely a fracture system in the northern part of the profile, is inferred in this study. The fractures are highly saturated with saline water, which make them good targets of resistivity-based geophysical methods. Nevertheless, conductive sediments overlain by the lake water add further difficulty to resolve these deep fracture zones. Therefore, the joint inversion of RMT and ERT data particularly helps to improve the resolution of the resistivity models in areas where the profile traverses shallow water and land sections. Our modification of the joint inversion of RMT and ERT data improves the study of geological units underneath shallow water bodies where underground infrastructures are planned. Thus, it allows better planning and mitigating the risks and costs associated with conductive weakness zones.

ERTS-1 imagery use in reconnaissance prospecting: Evaluation of commercial utility of ERTS-1 imagery in structural reconnaissance for minerals and petroleum

NASA Technical Reports Server (NTRS)

Saunders, D. F.; Thomas, G. E. (Principal Investigator); Kinsman, F. E.; Beatty, D. F.

1973-01-01

The author has identified the following significant results. This study was performed to investigate applications of ERTS-1 imagery in commercial reconnaissance for mineral and hydrocarbon resources. ERTS-1 imagery collected over five areas in North America (Montana; Colorado; New Mexico-West Texas; Superior Province, Canada; and North Slope, Alaska) has been analyzed for data content including linears, lineaments, and curvilinear anomalies. Locations of these features were mapped and compared with known locations of mineral and hydrocarbon accumulations. Results were analyzed in the context of a simple-shear, block-coupling model. Data analyses have resulted in detection of new lineaments, some of which may be continental in extent, detection of many curvilinear patterns not generally seen on aerial photos, strong evidence of continental regmatic fracture patterns, and realization that geological features can be explained in terms of a simple-shear, block-coupling model. The conculsions are that ERTS-1 imagery is of great value in photogeologic/geomorphic interpretations of regional features, and the simple-shear, block-coupling model provides a means of relating data from ERTS imagery to structures that have controlled emplacement of ore deposits and hydrocarbon accumulations, thus providing a basis for a new approach for reconnaissance for mineral, uranium, gas, and oil deposits and structures.

The application of ERTS imagery to monitoring Arctic sea ice. [mapping ice in Bering Sea, Beaufort Sea, Canadian Archipelago, and Greenland Sea

NASA Technical Reports Server (NTRS)

Barnes, J. C. (Principal Investigator); Bowley, C. J.

1974-01-01

The author has identified the following significant results. Because of the effect of sea ice on the heat balance of the Arctic and because of the expanding economic interest in arctic oil and minerals, extensive monitoring and further study of sea ice is required. The application of ERTS data for mapping ice is evaluated for several arctic areas, including the Bering Sea, the eastern Beaufort Sea, parts of the Canadian Archipelago, and the Greenland Sea. Interpretive techniques are discussed, and the scales and types of ice features that can be detected are described. For the Bering Sea, a sample of ERTS-1 imagery is compared with visual ice reports and aerial photography from the NASA CV-990 aircraft. The results of the investigation demonstrate that ERTS-1 imagery has substantial practical application for monitoring arctic sea ice. Ice features as small as 80-100 m in width can be detected, and the combined use of the visible and near-IR imagery is a powerful tool for identifying ice types. Sequential ERTS-1 observations at high latitudes enable ice deformations and movements to be mapped. Ice conditions in the Bering Sea during early March depicted in ERTS-1 images are in close agreement with aerial ice observations and photographs.

Geologic and mineral and water resources investigations in western Colorado using ERTS-1 data

NASA Technical Reports Server (NTRS)

Knepper, D. H., Jr. (Principal Investigator); Hutchinson, R. M.; Sawatzky, D. L.; Trexler, D. W.; Bruns, D. L.; Nicolais, S. M.

1973-01-01

The author has identified the following significant results. Topography was found to be the most important factor defining folds on ERTS-1 imagery of northwestern Colorado; tonal variations caused by rock reflectance and vegetation type and density are the next most important factors. Photo-linears mapped on ERTS-1 imagery of central Colorado correlate well with ground-measured joint and fracture trends. In addition, photo-linears have been successfully used to determine the location and distribution of metallic mineral deposits in the Colorado Mineral Belt. True color composites are best for general geologic analysis and false color composites prepared with positive/negative masks are useful for enhancing local geologic phenomena. During geologic analysis of any given area, ERTS-1 imagery from several different dates should be studied.

Applicability of ERTS to Antarctic iceberg resources. [harvesting icebergs for fresh water

NASA Technical Reports Server (NTRS)

Hult, J. L.; Ostrander, N. C.

1974-01-01

This investigation explores the applicability of ERTS to: (1) determine the Antarctic sea ice and environmental behavior that may influence the harvesting of icebergs, and (2) monitor iceberg locations, characteristics, and evolution. Imagery sampling in the western Antarctic between the Peninsula and the Ross Sea is used in the analysis. It is found that the potential applicability of ERTS to the research, planning, and harvesting operations can contribute importantly to the glowing promise derived from broader scope studies for the use of Antarctic icebergs to relieve a growing global thirst for fresh water. Several years of comprehensive monitoring will be necessary to characterize sea-ice and environmental behavior and iceberg evolution. Live ERTS services will assist harvesting control and claiming operations and offer a means for harmonizing entitlements to iceberg resources.

The trophic classification of lakes using ERTS multispectral scanner data

NASA Technical Reports Server (NTRS)

Blackwell, R. J.; Boland, D. H.

1975-01-01

Lake classification methods based on the use of ERTS data are described. Preliminary classification results obtained by multispectral and digital image processing techniques indicate satisfactory correlation between ERTS data and EPA-supplied water analysis. Techniques for determining lake trophic levels using ERTS data are examined, and data obtained for 20 lakes are discussed.

Diagnosis, quality of life, and treatment of patients with Hunter syndrome in the French healthcare system: a retrospective observational study.

PubMed

Guffon, Nathalie; Heron, Bénédicte; Chabrol, Brigitte; Feillet, François; Montauban, Vincent; Valayannopoulos, Vassili

2015-04-12

Mucopolysaccharidosis II (MPS II) is associated with a broad spectrum of chronic and progressive, life-limiting symptoms. Idursulfase is approved for MPS II enzyme replacement therapy (ERT) in over 50 countries. This retrospective study evaluated the MPS II burden, organization of clinical care, and effects of idursulfase treatment on the disease in France. MPS II patients who had received idursulfase ERT in the French healthcare system were enrolled. In addition to clinician and patient questionnaires, the Clinical Global Impression-Improvement (CGI-I); Patient Global Impression-Improvement (PGI-I); KIDSCREEN-27, and EuroQoL-5D for adult patients scales were used to assess quality of life (QoL) and efficacy. Fifty-two patients were enrolled from 5 sites in France. The majority of patients (69.2%) presented a severe MPS II phenotype with progressive neurocognitive impairment. Major impacts on QoL were apparent, with at least 1 member of the family having to reorganize working hours (45.5%) or to stop working (22.7%). KIDSCREEN-27 and EuroQoL-5D scale scores were well below those for referent (control) populations. Most families (70.0%) experienced a diagnostic delay of at least 3 years after the initial observation of symptoms. The MPS II diagnosis was often delivered without adequate sensitivity, psychological support, or comprehensive information about the disease. The study population had received a mean of 3.8 ± 1.3 years ERT. Forty-four percent of patients with the attenuated phenotype (without progressive neurocognitive impairment) showed symptom improvement during both the first year (Period 1) and from the end of the first year of treatment to "the present" (Period 2), as measured by CGI-I/PGI-I. 30.3% and 9.1% of severe patients experienced symptom improvement during Periods 1 and 2, respectively, while 63.6% and 51.5% displayed no change. The most common adverse reactions reported were skin rash and other infusion-associated reactions. MPS II adversely affects multiple domains of QoL for patients and families, requiring multiple healthcare services and social aid programs. The majority of patients with either phenotype experienced either improvement or stability in their symptoms during the first year of ERT, but this was clearly less so for patients with the severe phenotype after the first year of treatment.

ERTS-1 imagery as an aid to the definition of the geotectonic domains of the southern African crystalline shield

NASA Technical Reports Server (NTRS)

Viljoen, M. J.; Viljoen, R. P.

1973-01-01

Portions of two geotectonic environments in South Africa have been studied on ERTS-1 imagery. It was found that the broad synoptic view provided by this imagery is ideally suited to a study of the diagnostic macro-structures, and that the different geotectonic styles are clearly recorded. ERTS-1 imagery thus allows a more accurate definition than exists at present of the contact zones and internal structures of the two domains. The importance of this investigation as an aid to gaining on insight into the relevance of plate tectonics in Precambrian times is briefly discussed.

The economic value of remote sensing by satellite: An ERTS overview and the value of continuity of service. Volume 2: Source document

NASA Technical Reports Server (NTRS)

Andrews, J.; Donziger, A.; Hazelrigg, G. A., Jr.; Heiss, K. P.; Sand, F.; Stevenson, P.

1974-01-01

The economic value of an ERS system with a technical capability similar to ERTS, allowing for increased coverage obtained through the use of multiple active satellites in orbit is presented. A detailed breakdown of the benefits achievable from an ERS system is given and a methodology for their estimation is established. The ECON case studies in agriculture, water use, and land cover are described along with the current ERTS system. The cost for a projected ERS system is given.

Application of ERTS-1 imagery to the study of caribou movements and winter dispersal in relation to prevailing snowcover

NASA Technical Reports Server (NTRS)

Lent, P. C. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Step-wise discriminate analysis has demonstrated the feasibility of feature identification using linear discriminate functions of ERTS-1 MSS band densities and their ratios. The analysis indicated that features such as small streams can be detected even when they are in dark mountain shadow. The potential utility of this and similar analytic techniques appears considerable, and the limits it can be applied to analysis of ERTS-1 imagery are not yet fully known.

Widening ERTS applications

NASA Technical Reports Server (NTRS)

Mercanti, E. P.

1974-01-01

In less than two years of operation ERTS-1 is shown to have successfully completed its experimental mission and to be delivering an ever-increasing roster of benefits. The widening ERTS applications reviewed include air quality and weather modification, aid to oil exploration, ore-deposit exploration, short-lived event observation, flood area assessment and flood-plain mapping, land and water quality assessment, soil association mapping, crop production measurements, wildlife resources, drought and desertification studies, ground-water exploration, watershed surveys, snow and ice monitoring, surface water mapping, and iceberg surveys. Future projects and developments are also briefly reviewed.

Evaluation of ERTS-1 data for acquiring land use data of northern Megalopolis. [New England

NASA Technical Reports Server (NTRS)

Simpson, R. B.; Lindgren, D. T.; Goldstein, W. D.

1974-01-01

State planners are increasingly becoming interested in ERTS as a possible method for acquiring land use data. An important consideration to them is whether ERTS can provide such data at a savings in both time and money over alternative systems. A preliminary evaluation of ERTS as a planning tool is given.

Land use classification and change analysis using ERTS-1 imagery in CARETS

NASA Technical Reports Server (NTRS)

Alexander, R. H.

1973-01-01

Land use detail in the CARETS area obtainable from ERTS exceeds the expectations of the Interagency Steering Committee and the USGS proposed standardized classification, which presents Level 1 categories for ERTS and Level 2 for high altitude aircraft data. Some Levels 2 and 3, in addition to Level 1, categories were identified on ERTS data. Significant land use changes totaling 39.2 sq km in the Norfolk-Portsmouth SMSA were identified and mapped at Level 2 detail using a combination of procedures employing ERTS and high altitude aircraft data.

Reported outcomes of 453 pregnancies in patients with Gaucher disease: An analysis from the Gaucher outcome survey.

PubMed

Lau, Heather; Belmatoug, Nadia; Deegan, Patrick; Goker-Alpan, Ozlem; Schwartz, Ida Vanessa D; Shankar, Suma P; Panahloo, Zoya; Zimran, Ari

2018-02-01

Gaucher disease (GD) may worsen during pregnancy, leading to the discussion of continuing treatment during pregnancy. We examined fetal outcomes of pregnancies reported in the Gaucher Outcome Survey, an international GD-specific registry established in 2010. A total of 453 pregnancies were reported. Most pregnancies (336/453, 74.2%) were in women who did not receive GD-specific treatment during pregnancy, while enzyme replacement therapy (ERT) was received during 117/453 (25.8%) pregnancies. No pregnancies exposed to substrate reduction therapy were reported. The percentage of normal outcomes (live birth delivered at term with no congenital abnormalities) was similar in untreated and treated pregnancies (92.9% vs. 91.4%). The percentage of spontaneous abortions in untreated pregnancies was 3.6% (95% CI, 1.9%- 6.2%) compared with 6.9% (95% CI, 3.0%-13.1%) in treated pregnancies (p=0.1866). In women who received velaglucerase alfa <1month prior to conception and/or during pregnancy, 34/36 (94.4%) pregnancies had normal outcomes and 2 (5.6%) ended in spontaneous abortion. Normal outcomes were observed in the 20 pregnancies with velaglucerase alfa exposure starting <1month prior to conception and continuing through all trimesters. These observations, in addition to information in the literature, suggest that continuation of ERT during pregnancy may be appropriate for GD patients. Copyright © 2016 Shire Human Genetic Therapies, Inc. Published by Elsevier Inc. All rights reserved.

Satellite geological and geophysical remote sensing of Iceland: Preliminary results of geologic, hydrologic, oceanographic, and agricultural studies with ERTS-1 imagery

NASA Technical Reports Server (NTRS)

Williams, R. S., Jr. (Principal Investigator); Boeovarsson, A.; Frioriksson, S.; Palmason, G.; Rist, S.; Sigtryggsson, H.; Saemundsson, K.; Thorarinsson, S.; Thorsteinsson, I.

1973-01-01

The author has identified the following significant results. The wide variety of geological and geophysical phenomena which can be observed in Iceland, and particularly their very direct relation to the management of the country's natural resources, has provided great impetus to the use of ERTS-1 imagery to measure and map the dynamic natural phenomena in Iceland. MSS imagery is being used to study a large variety of geological and geophysical eruptive products, geologic structure, volcanic geomorphology, hydrologic, oceanographic, and agricultural phenomena of Iceland. Some of the preliminary results from this research projects are: (1) a large number of geological and volcanic features can be studied from ERTS-1 imagery, particularly imagery acquired at low sun angle, which had not previously been recognized; (2) under optimum conditions the ERTS-1 satellite can discern geothermal areas by their snow melt pattern or warm spring discharge into frozen lakes; (3) various maps at scales of 1:1 million and 1:500,000 can be updated and made more accurate with ERTS-1 imagery; (4) the correlation of water reserves with snowcover can improve the basis for planning electrical production in the management of water resources; (5) false-color composites (MSS) permitted the mapping of four types of vegetation: forested; grasslands, reclaimed, and cultivated areas, and the seasonal change of the vegetation, all of high value to rangeland management.

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.

PubMed

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 patients with MPS-I who initiated ERT in adult age. This agreement suggests that combining case reports quantitatively, rather than analyzing them separately or qualitatively, may improve conclusions in the field of rare diseases. Copyright © 2018 Elsevier Inc. All rights reserved.

Correlation between urinary GAG and anti-idursulfase ERT neutralizing antibodies during treatment with NICIT immune tolerance regimen: A case report☆,☆☆

PubMed Central

Kim, Sarah; Whitley, Chester B.; Jarnes Utz, Jeanine R.

2018-01-01

Introduction Antibodies to intravenous idursulfase enzyme replacement therapy (ERT) for patients with Hunter syndrome (mucopolysaccharidosis type II, MPS II) can have a harmful clinical impact, including both increasing risk of infusion reactions and inhibiting therapeutic activity. Thus, failure to monitor anti-idursulfase antibodies and neutralizing antibodies, and delays in reporting results, may postpone critical clinical decisions. Hypothesis Urinary glycosaminoglycan (GAG) levels may be used as a biomarker for anti-idursulfase antibodies and neutralizing antibodies to improve timeliness in monitoring and managing ERT. Methods This is a case report describing a patient with MPS II with high levels of neutralizing antibodies and worsened clinical status who was treated for five years with a non-immunosuppressive and non-cytotoxic immune tolerance (NICIT) regimen, consisting of intravenous immune globulin and frequent infusions of idursulfase. Neutralizing antibodies and total anti-idursulfase antibodies were measured by two different methods, the direct 1,9-dimethylmethylene blue (DMB) assay and cetylpyridinium chloride carbazole-borate (CPC) assay. Results Neutralizing antibodies, measured as percent inhibition of enzyme activity and also by total neutralizing antibody titer, were correlated with quantitative urinary GAG measured by DMB assay (p = 0.026, p = 0.0067), and quantitative urinary GAG by CPC assay with percent inhibition of enzyme activity by neutralizing antibodies (p = 0.0475). The NICIT regimen showed a sustained immune tolerance after five years and was well-tolerated. Conclusions Urinary GAG, measured by DMB assay, may be a biomarker for anti-idursulfase neutralizing antibodies and is useful for managing immune tolerance regimens for patients with MPS II who have high levels of anti-idursulfase neutralizing antibodies. This study highlights the importance of regular and frequent monitoring of urinary GAG in patients with MPS II who are receiving ERT. The NICIT regimen, with less drug toxicities, may be preferred in patients with MPS who have a high risk of infections and whose disease progresses less rapidly than some other lysosomal storage diseases, such as infantile Pompe disease. PMID:28610913

A study of the utilization of ERTS-1 data from the Wabash River Basin. [soil mapping, crop identification, water resources

NASA Technical Reports Server (NTRS)

Landgrebe, D. A. (Principal Investigator)

1973-01-01

The author has identified the following significant results. In soil association mapping, computerized analysis of ERTS-1 MSS data has yielded images which will prove useful in the ongoing Cooperative Soil Survey program, involving the Soil Conservation Service of USDA and other state and local agencies. In the present mode of operation, a soil survey for a county may take up to 5 years to be completed. Results indicate that a great deal of soils information can be extracted from ERTS-1 data by computer analysis. This information is expected to be very valuable in the premapping conference phase of a soil survey, resulting in more efficient field operations during the actual mapping. In the earth surface features mapping effort it was found that temporal data improved the classification accuracy of forest classification in Tippecanoe County, Indiana. In water resources study a severe scanner look angle effect was observed in the aircraft scanner data of a test lake which was not present in ERTS-1 data of the same site. This effect was greatly accentuated by surface roughness caused by strong winds. Quantitative evaluation of urban features classification in ERTS-1 data was obtained. An 87.1% test accuracy was obtained for eight categories in Marion County, Indiana.

Monitoring the dispersion of ocean waste disposal plumes from ERTS-1 and Skylab. [Delaware coastal waters

NASA Technical Reports Server (NTRS)

Klemas, V. (Principal Investigator); Davis, G.; Myers, T.

1974-01-01

The author has identified the following significant results. About forty miles off the Delaware coast is located the disposal site for waste discharged from a plant processing titanium dioxide. The discharge is a greenish-brown; 15-20% acid liquid which consists primarily of iron chlorides and sulfates. The barge which transports this waste has a 1,000,000 gallon capacity and makes approximately three trips to the disposal site per week. ERTS-1 MSS digital tapes are being used to study the dispersion patterns and drift velocities of the iron-acid plume. Careful examination of ERTS-1 imagery disclosed a fishhook-shaped plume about 40 miles east of Cape Henlopen caused by a barge disposing acid wastes. The plume shows up more strongly in the green band than in the red band. Since some acids have a strong green component during dumping and turn slowly more brownish-reddish with age, the ratio of radiance signatures between the green and red bands may give an indication of how long before the satellite overpass the acid was dumped. Enlarged enhancements of the acid waste plumes, prepared from the ERTS-1 MSS digital tapes aided considerably in studies of the dispersion of the waste plume. Currently acid dumps are being coordinated with ERTS-1 overpasses.

Using Contaminant Transport Simulations to Optimize Electrical Resistivity Tomography Survey Design for Improved Contaminant Detection at Lined Ponds

NASA Astrophysics Data System (ADS)

Herring, T.; Pidlisecky, A.

2015-12-01

The saline flowback water produced during hydraulic fracturing is often stored in lined surface ponds. Leakage from these ponds poses a significant environmental threat and there is a need for a reliable and economical long term monitoring strategy. Electrical resistivity tomography (ERT), being sensitive to changes in groundwater salinity, is therefore well suited to such a problem. The goal of this work is to compare the leak detection capabilities of a surface ERT array and a downhole ERT array. In this study several plausible 3D electrical conductivity models were created that simulated a contaminant plume evolving over time, using realistic contaminant concentrations, plume geometries, water saturation profiles, and seasonal temperature profiles. The forward modeled data were used to identify the advantages and drawbacks of using each ERT array orientation.

Vegetative and geologic mapping of the western Seward Peninsula, Alaska, based on ERTS-1 imagery

NASA Technical Reports Server (NTRS)

Anderson, J. H.; Shapiro, L. H.; Belon, A. E.

1973-01-01

ERTS-1 scene 1009-22095 (Western Seward Peninsula, Alaska) has been studied, partly as a training exercise, to evaluate whether direct visual examination of individual and custom color-composite prints can provide new information on the vegetation and geology of this relatively well known area of Alaska. The vegetation analysis reveals seven major vegetation types, only four of which are described on existing vegetation maps. In addition, the ERTS analysis provides greater detail than the existing maps on the areal distribution of vegetation types. The geologic analysis demonstrates that most of the major rock units and geomorphic boundaries shown on the available geologic maps could also be identified on the ERTS data. Several major high-angle faults were observed, but the zones of thrust faults which are much less obvious.

ERTS-B (Earth Resources Technology Satellite). [spacecraft design remote sensor description, and technology utilization

NASA Technical Reports Server (NTRS)

1975-01-01

Mission plans and objectives of the ERTS 2 Satellite are presented. ERTS 2 follow-on investigations in various scientific disciplines including agriculture, meteorology, land-use, geology, water resources, oceanography, and environment are discussed. Spacecraft design and its sensors are described along with the Delta launch vehicle and launch operations. Applications identified from ERTS 1 investigations are summarized.

Editing ERTS-1 data to exclude land aids cluster analysis of water targets

NASA Technical Reports Server (NTRS)

Erb, R. B. (Principal Investigator)

1973-01-01

The author has identified the following significant results. It has been determined that an increase in the number of spectrally distinct coastal water types is achieved when data values over the adjacent land areas are excluded from the processing routine. This finding resulted from an automatic clustering analysis of ERTS-1 system corrected MSS scene 1002-18134 of 25 July 1972 over Monterey Bay, California. When the entire study area data set was submitted to the clustering only two distinct water classes were extracted. However, when the land area data points were removed from the data set and resubmitted to the clustering routine, four distinct groupings of water features were identified. Additionally, unlike the previous separation, the four types could be correlated to features observable in the associated ERTS-1 imagery. This exercise demonstrates that by proper selection of data submitted to the processing routine, based upon the specific application of study, additional information may be extracted from the ERTS-1 MSS data.

Progress and plans of a remote sensing program for the International Field Year for the Great Lakes (IFYGL)

NASA Technical Reports Server (NTRS)

Polcyn, F. C.; Wagner, T. W. (Principal Investigator)

1972-01-01

The author has identified the following significant results. ERTS-1 coverage of the 32,000 square mile Lake Ontario Basin is being used to study short term and seasonal changes which affect many aspects of water problems in the Great Lakes. As part of the International Field Year for the Great Lakes (IFYGL), a coordinated, synoptic study of the Lake Ontario Basin, processed ERTS-1 imagery will contribute to the data base of synchronized observations being made by investigators from many U.S. and Canadian government agencies and universities. The first set of ERTS data has been received and will be processed shortly for parameters of hydrological and limnological significance such as land use, terrain features, and water quality. When complete, nine ERTS-1 frames recorded during a substantially clear period will provide coverage of the entire Basin. Seven frames show all but a small portion of the southern and eastern end of the Basin. Many drainage basin characteristics are clearly identifiable on the imagery.

The uses of ERTS-1 imagery in the analysis of landscape change. [agriculture, strip mining forests, urban-suburban growth, and flooding in Tennessee, Kentucky, Mississippi, and Alabama

NASA Technical Reports Server (NTRS)

Rehder, J. B. (Principal Investigator)

1974-01-01

The author has identified the following significant results. The analysis of strip mining from ERTS-1 data has resulted in the mapping of landscape changes for the Cumberland Plateau Test Site. Several mapping experiments utilizing ERTS-1 data have been established for the mapping of state-wide land use regions. The first incorporates 12 frames of ERTS-1 imagery for the generalized thematic mapping of forest cover for the state of Tennessee. In another mapping effort, 14 ERTS-1 images have been analyzed for plowed ground signatures to produce a map of agricultural regions for Tennessee, Kentucky, and the northern portions of Mississippi and Alabama. Generalized urban land use categories and transportation networks have been determined from ERTS-1 imagery for the Knoxville Test Site. Finally, through the analysis of ERTS-1 imagery, short-lived phenomena such as the 1973 spring floods on the Mississippi River in western Tennessee, have been detected, monitored, and mapped.

PFLOTRAN-E4D: A parallel open source PFLOTRAN module for simulating time-lapse electrical resistivity data

DOE Office of Scientific and Technical Information (OSTI.GOV)

Johnson, Timothy C.; Hammond, Glenn E.; Chen, Xingyuan

Time-lapse electrical resistivity tomography (ERT) is finding increased application for remotely monitoring processes occurring in the near subsurface in three-dimensions (i.e. 4D monitoring). However, there are few codes capable of simulating the evolution of subsurface resistivity and corresponding tomographic measurements arising from a particular process, particularly in parallel and with an open source license. Herein we describe and demonstrate an electrical resistivity tomography module for the PFLOTRAN subsurface flow and reactive transport simulation code, named PFLOTRAN-E4D. The PFLOTRAN-E4D module operates in parallel using a dedicated set of compute cores in a master-slave configuration. At each time step, the master processesmore » receives subsurface states from PFLOTRAN, converts those states to bulk electrical conductivity, and instructs the slave processes to simulate a tomographic data set. The resulting multi-physics simulation capability enables accurate feasibility studies for ERT imaging, the identification of the ERT signatures that are unique to a given process, and facilitates the joint inversion of ERT data with hydrogeological data for subsurface characterization. PFLOTRAN-E4D is demonstrated herein using a field study of stage-driven groundwater/river water interaction ERT monitoring along the Columbia River, Washington, USA. Results demonstrate the complex nature of subsurface electrical conductivity changes, in both the saturated and unsaturated zones, arising from river stage fluctuations and associated river water intrusion into the aquifer. Furthermore, the results also demonstrate the sensitivity of surface based ERT measurements to those changes over time.« less

Joint Use of ERT, Tracer, and Numerical Techniques to Image Preferential Flow Paths in a Fractured Granite Aquifer

NASA Astrophysics Data System (ADS)

Sanaga, S.; Vijay, S.; Kbvn, P.; Peddinti, S. R.; P S L, S.

2017-12-01

Fractured geologic media poses formidable challenges to hydrogeologists due of the strenuous mapping of fracture-matrix system and quantification of flow and transport processes. In this research, we demonstrated the efficacy of tracer-ERT studies coupled with numerical simulations to delineate preferential flow paths in a fractured granite aquifer of Deccan traps in India. A series of natural gradient saline tracer experiments were conducted from a depth window of 18 to 22 m in an injection well located inside the IIT Hyderabad campus. Tracer migration was monitored in a time-lapse mode using two cross-sectional surface ERT profiles placed in the direction of flow gradient. Dynamic changes in sub-surface electrical properties inferred via resistivity anomalies were used to highlight preferential flow paths of the study area. ERT-derived tracer breakthrough curves were in agreement with geochemical sample measurements (R2=0.74). Fracture geometry and hydraulic properties derived from ERT and pumping tests were then used to evaluate two mathematical conceptualizations that are relevant to fractured aquifers. Results of numerical analysis conclude that a dual continuum model that combines matrix and fracture systems through a flow exchange term has outperformed equivalent continuum model in reproducing tracer concentrations at the monitoring wells (evident by decrease in RMSE from 199 mg/l to 65 mg/l). A sensitivity analysis of the model parameters reveals that spatial variability in hydraulic conductivity, local-scale dispersion, and flow exchange at fracture-matrix interface have a profound effect on model simulations. Keywords: saline tracer, ERT, fractured granite, groundwater, preferential flow, numerical simulation

PFLOTRAN-E4D: A parallel open source PFLOTRAN module for simulating time-lapse electrical resistivity data

DOE PAGES

Johnson, Timothy C.; Hammond, Glenn E.; Chen, Xingyuan

2016-09-22

Time-lapse electrical resistivity tomography (ERT) is finding increased application for remotely monitoring processes occurring in the near subsurface in three-dimensions (i.e. 4D monitoring). However, there are few codes capable of simulating the evolution of subsurface resistivity and corresponding tomographic measurements arising from a particular process, particularly in parallel and with an open source license. Herein we describe and demonstrate an electrical resistivity tomography module for the PFLOTRAN subsurface flow and reactive transport simulation code, named PFLOTRAN-E4D. The PFLOTRAN-E4D module operates in parallel using a dedicated set of compute cores in a master-slave configuration. At each time step, the master processesmore » receives subsurface states from PFLOTRAN, converts those states to bulk electrical conductivity, and instructs the slave processes to simulate a tomographic data set. The resulting multi-physics simulation capability enables accurate feasibility studies for ERT imaging, the identification of the ERT signatures that are unique to a given process, and facilitates the joint inversion of ERT data with hydrogeological data for subsurface characterization. PFLOTRAN-E4D is demonstrated herein using a field study of stage-driven groundwater/river water interaction ERT monitoring along the Columbia River, Washington, USA. Results demonstrate the complex nature of subsurface electrical conductivity changes, in both the saturated and unsaturated zones, arising from river stage fluctuations and associated river water intrusion into the aquifer. Furthermore, the results also demonstrate the sensitivity of surface based ERT measurements to those changes over time.« less

Machine processing of ERTS and ground truth data

NASA Technical Reports Server (NTRS)

Rogers, R. H. (Principal Investigator); Peacock, K.

1973-01-01

The author has identified the following significant results. Results achieved by ERTS-Atmospheric Experiment PR303, whose objective is to establish a radiometric calibration technique, are reported. This technique, which determines and removes solar and atmospheric parameters that degrade the radiometric fidelity of ERTS-1 data, transforms the ERTS-1 sensor radiance measurements to absolute target reflectance signatures. A radiant power measuring instrument and its use in determining atmospheric parameters needed for ground truth are discussed. The procedures used and results achieved in machine processing ERTS-1 computer -compatible tapes and atmospheric parameters to obtain target reflectance are reviewed.

Taliglucerase alfa: safety and efficacy across 6 clinical studies in adults and children with Gaucher disease.

PubMed

Zimran, Ari; Wajnrajch, Michael; Hernandez, Betina; Pastores, Gregory M

2018-02-23

Taliglucerase alfa is an enzyme replacement therapy (ERT) approved for treatment of adult and paediatric patients with Type 1 Gaucher disease (GD) in several countries and the first plant cell-expressed recombinant therapeutic protein approved by the US Food and Drug Administration for humans. Here, we review the findings across six key taliglucerase alfa clinical studies. A total of 33 treatment-naïve adult patients were randomized to taliglucerase alfa 30 U/kg or 60 U/kg in a 9-month, multicentre, randomized, double-blind, parallel-group, dose-comparison pivotal study, after which eligible patients continued into two consecutive extension studies; 17 treatment-naïve adult patients completed 5 total years of treatment with taliglucerase alfa. In the only ERT study focused on exclusively paediatric patients with GD, 11 treatment-naïve children were randomized to taliglucerase alfa 30 U/kg or 60 U/kg in a 12-month, multicentre, double-blind study; nine completed 3 total years of treatment in a dedicated paediatric extension study. The effect of switching patients from imiglucerase to taliglucerase alfa was also investigated in a separate 9-month study that included 26 adults and five children; 10 adults completed a total of 3 years and two children completed a total of 2.75 years of taliglucerase alfa treatment in the extension studies. All studies evaluated safety and spleen volume, liver volume, platelet count, haemoglobin concentration, and biomarkers as measures of efficacy. Detailed results from baseline through the end of these studies are presented. Taliglucerase alfa was well tolerated, and adverse events were generally mild/moderate in severity and transient. Treatment with taliglucerase alfa resulted in improvements (treatment-naïve patients) or stability (patients switched from imiglucerase) in visceral, haematologic, and biomarker parameters. Together, this comprehensive data set supports the treatment of adult and paediatric patients with GD who are naïve to ERT or who have previously been treated with imiglucerase.

ERTS-B imagery interpretation techniques in the Tennessee Valley

NASA Technical Reports Server (NTRS)

Gonzalez, R. C. (Principal Investigator)

1973-01-01

There are no author-identified significant results in this report. The proposed investigation is a continuation of an ERTS-1 project. The principal missions are to serve as the principal supporter on computer and image processing problems for the multidisciplinary ERTS effort of the University of Tennessee, and to carry out research in improved methods for the computer processing, enhancement, and recognition of ERTS imagery.

Activity of daily living for Morquio A syndrome.

PubMed

Yasuda, Eriko; Suzuki, Yasuyuki; Shimada, Tsutomu; Sawamoto, Kazuki; Mackenzie, William G; Theroux, Mary C; Pizarro, Christian; Xie, Li; Miller, Freeman; Rahman, Tariq; Kecskemethy, Heidi H; Nagao, Kyoko; Morlet, Thierry; Shaffer, Thomas H; Chinen, Yasutsugu; Yabe, Hiromasa; Tanaka, Akemi; Shintaku, Haruo; Orii, Kenji E; Orii, Koji O; Mason, Robert W; Montaño, Adriana M; Fukao, Toshiyuki; Orii, Tadao; Tomatsu, Shunji

2016-06-01

The aim of this study was to evaluate the activity of daily living (ADL) and surgical interventions in patients with mucopolysaccharidosis IVA (MPS IVA). The factor(s) that affect ADL are age, clinical phenotypes, surgical interventions, therapeutic effect, and body mass index. The ADL questionnaire comprises three domains: "Movement," "Movement with cognition," and "Cognition." Each domain has four subcategories rated on a 5-point scale based on the level of assistance. The questionnaire was collected from 145 healthy controls and 82 patients with MPS IVA. The patient cohort consisted of 63 severe and 17 attenuated phenotypes (2 were undefined); 4 patients treated with hematopoietic stem cell transplantation (HSCT), 33 patients treated with enzyme replacement therapy (ERT) for more than a year, and 45 untreated patients. MPS IVA patients show a decline in ADL scores after 10years of age. Patients with a severe phenotype have a lower ADL score than healthy control subjects, and lower scores than patients with an attenuated phenotype in domains of "Movement" and "Movement with cognition." Patients, who underwent HSCT and were followed up for over 10years, had higher ADL scores and fewer surgical interventions than untreated patients. ADL scores for ERT patients (2.5years follow-up on average) were similar with the-age-matched controls below 10years of age, but declined in older patients. Surgical frequency was higher for severe phenotypic patients than attenuated ones. Surgical frequency for patients treated with ERT was not decreased compared to untreated patients. In conclusion, we have shown the utility of the proposed ADL questionnaire and frequency of surgical interventions in patients with MPS IVA to evaluate the clinical severity and therapeutic efficacy compared with age-matched controls. Copyright © 2016. Published by Elsevier Inc.

Endomyocardial biopsies in patients with left ventricular hypertrophy and a common Chinese later-onset fabry mutation (IVS4 + 919G > A)

PubMed Central

2014-01-01

Background In Taiwan, DNA-based newborn screening showed a surprisingly high incidence of a cardiac Fabry mutation (IVS4 + 919G > A). The prevalence of this mutation is too high to be believed that it is a real pathogenic mutation. The purpose of this study is to identify the cardiac pathologic characteristics in patients with left ventricular hypertrophy and this mutation Methods and results Endomyocardial biopsies were obtained in 22 patients (Median age: 61, males: 17; females: 5) with left ventricular hypertrophy and the IVS4 + 919G > A mutation; five patients had not received enzyme replacement therapy (ERT) before biopsy, while the other 17 patients had received ERT from 8 months to 51 months. Except for three patients who had received ERT for more than 3 years, all other patients showed significant pathological change and globotriaosylceramide (Gb3) accumulation in their cardiomyocytes. In contrast to classical Fabry patients, no Gb3 accumulation was found in the capillary endothelial cells of any of our patients. Fourteen patients (63.6%) were found to have myofibrillolysis. Conclusions All of the untreated and most of the treated IVS4 + 919G > A patients showed typical pathological changes of Fabry disease in their cardiomyocytes. No endothelial accumulation of Gb3 was found, which is similar to the findings of several previous reports regarding later-onset Fabry disease. This result highly suggests that the IVS4 + 919G > A is a real pathogenic later-onset Fabry mutation. PMID:24980630

Identification, definition and mapping of terrestrial ecosystems in interior Alaska

NASA Technical Reports Server (NTRS)

Anderson, J. H. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The primary objective is to identify and analyze vegetation types in as great of detail as possible on ERTS-1 imagery and to classify and delineate them through mapping. This is basic to the identification, definition, and mapping of ecosystems. Major conclusions are: (1) the ERTS-1 system is useful for regional scale studies of broadly defined Alaskan vegetation types; (2) the resolution and spectral capabilities of ERTS-1 MSS imagery in photographic formats is adequate for certain phytecenologic purposes; and (3) preparation of an improved State vegetation map will be feasible.

Fiber type conversion by PGC-1α activates lysosomal and autophagosomal biogenesis in both unaffected and Pompe skeletal muscle.

PubMed

Takikita, Shoichi; Schreiner, Cynthia; Baum, Rebecca; Xie, Tao; Ralston, Evelyn; Plotz, Paul H; Raben, Nina

2010-12-13

PGC-1α is a transcriptional co-activator that plays a central role in the regulation of energy metabolism. Our interest in this protein was driven by its ability to promote muscle remodeling. Conversion from fast glycolytic to slow oxidative fibers seemed a promising therapeutic approach in Pompe disease, a severe myopathy caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA) which is responsible for the degradation of glycogen. The recently approved enzyme replacement therapy (ERT) has only a partial effect in skeletal muscle. In our Pompe mouse model (KO), the poor muscle response is seen in fast but not in slow muscle and is associated with massive accumulation of autophagic debris and ineffective autophagy. In an attempt to turn the therapy-resistant fibers into fibers amenable to therapy, we made transgenic KO mice expressing PGC-1α in muscle (tgKO). The successful switch from fast to slow fibers prevented the formation of autophagic buildup in the converted fibers, but PGC-1α failed to improve the clearance of glycogen by ERT. This outcome is likely explained by an unexpected dramatic increase in muscle glycogen load to levels much closer to those observed in patients, in particular infants, with the disease. We have also found a remarkable rise in the number of lysosomes and autophagosomes in the tgKO compared to the KO. These data point to the role of PGC-1α in muscle glucose metabolism and its possible role as a master regulator for organelle biogenesis - not only for mitochondria but also for lysosomes and autophagosomes. These findings may have implications for therapy of lysosomal diseases and other disorders with altered autophagy.

Land use mapping and change detection using ERTS imagery in Montgomery County, Alabama

NASA Technical Reports Server (NTRS)

Wilms, R. P.

1973-01-01

The feasibility of using remotely sensed data from ERTS-1 for mapping land use and detecting land use change was investigated. Land use information was gathered from 1964 air photo mosaics and from 1972 ERTS data. The 1964 data provided the basis for comparison with ERTS-1 imagery. From this comparison, urban sprawl was quite evident for the city of Montgomery. A significant trend from forestland to agricultural was also discovered. The development of main traffic arteries between 1964 and 1972 was a vital factor in the development of some of the urban centers. Even though certain problems in interpreting and correlating land use data from ERTS imagery were encountered, it has been demonstrated that remotely sensed data from ERTS is useful for inventorying land use and detecting land use change.

Enzyme replacement therapy in Fabry disease: influence on cardiac manifestations.

PubMed

Caballero, L; Climent, V; Hernández-Romero, D; Quintanilla, M A; de la Morena, G; Marín, F

2010-01-01

Fabry disease (FD) is an X-linked glycosphingolipid storage disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. This leads to a progressive accumulation of globotriaosylceramide (Gb3) in the lysosomes of different cells and tissues, causing principally ventricular hypertrophy, renal failure and cerebrovascular accidents, reducing lifespan both in hemizygous males and heterozygous females. Residual enzyme activity might lead to slow progression of the disease and result in the so-called cardiac or renal variants with delayed presentation. Two different forms of alpha-galactosidase A enzyme replacement therapies (ERT) are available for the treatment of FD, one genetically engineered in human cell line (agalsidase alfa, Replagal, Shire) and the other produced in a Chinese hamster ovary cell line (agalsidase beta, Fabrazyme, Genzyme). Although both proteins are structurally and functionally very similar, with the same amino acid sequence as the native human enzyme, they differ in the pattern of glycosilation of the protein depending on the originating cell line. Studies with both preparations have described a reduction in plasma, urinary sediment and tissue levels of Gb3, a decrease in the frequency of pain crisis and a reduction in left ventricular mass and improvement or stabilization of renal function. Studies have generally shown the greatest benefit when treatment is started at an early stage of the disease before extensive fibrosis or other irreversible tissue damage takes place. However, more data are needed to document long-term treatment outcomes. The aim of the present review is to provide an update overview of the two different forms of ERT for FD, their clinical effects in cardiac manifestations and their possible differences in terms of efficacy, side effects and safety profiles.

[Fabry nephropathy in a female with superposed IgA glomerulonephritis].

PubMed

Pisani, A; Sessa, A; Sabbatini, M; Andreucci, M V; Fusco, C; Balletta, M; Cianciaruso, B

2005-01-01

In Anderson-Fabry disease (AFd), the kidney is affected in all hemizygous males and in some heterozygous females. Female carriers can present subtle renal abnormalities due to glycosphingolipid (GSL) accumulation within renal cells. Renal biopsy is rarely performed in female Fabry patients because clinical renal manifestations are usually lacking. However, female carriers can accumulate GSL in their renal cells despite the absence of clinically evident kidney disease. We performed a kidney biopsy in a 52-year-old female patient, a Fabry disease carrier. The patient showed normal glomerular filtration rate, persistent microhematuria and proteinuria (about 1.7 g/24 hr), cornea "verticillata", and evident left ventricular hypertrophy. The molecular study documented a missense mutation R227Q in exon 5 of the alpha-galactosidase A gene. Optical microscopy showed electron-dense mesangial deposits due IgA glomerulonephritis, as confirmed by immunofluorescence. We decided to start therapy with angiotensin-converting enzyme inhibitors (ACE-I). After 8 months of treatment, the patient demonstrated proteinuria of 0.9 g/24 hr. To decide when to start treatment using enzyme replacement therapy (ERT) with human recombinant GAL A (Fabrazyme), we decided to perform an electron microscopy study of the renal biopsy. The renal ultrastructural findings were typical GSL inclusions in all kinds of glomerular cells, in tubular epithelial cells and in endothelial cells of interstitial capillaries, confirming the hypothesis of Fabry nephropathy. Consequently, Fabrazyme was given at a standard dose of 1 mg/kg every 2 weeks. After 24 months of combined treatment (ACE-I-Fabrazyme), proteinuria decreased to 0.2 g/24 hr. The importance of performing the ultrastructural examination of the kidney biopsy is stressed, especially in heterozygous Fabry patients to evaluate the need to treat them with ERT and to evaluate the degree of renal involvement.

The switch from fermentation to respiration in Saccharomyces cerevisiae is regulated by the Ert1 transcriptional activator/repressor.

PubMed

Gasmi, Najla; Jacques, Pierre-Etienne; Klimova, Natalia; Guo, Xiao; Ricciardi, Alessandra; Robert, François; Turcotte, Bernard

2014-10-01

In the yeast Saccharomyces cerevisiae, fermentation is the major pathway for energy production, even under aerobic conditions. However, when glucose becomes scarce, ethanol produced during fermentation is used as a carbon source, requiring a shift to respiration. This adaptation results in massive reprogramming of gene expression. Increased expression of genes for gluconeogenesis and the glyoxylate cycle is observed upon a shift to ethanol and, conversely, expression of some fermentation genes is reduced. The zinc cluster proteins Cat8, Sip4, and Rds2, as well as Adr1, have been shown to mediate this reprogramming of gene expression. In this study, we have characterized the gene YBR239C encoding a putative zinc cluster protein and it was named ERT1 (ethanol regulated transcription factor 1). ChIP-chip analysis showed that Ert1 binds to a limited number of targets in the presence of glucose. The strongest enrichment was observed at the promoter of PCK1 encoding an important gluconeogenic enzyme. With ethanol as the carbon source, enrichment was observed with many additional genes involved in gluconeogenesis and mitochondrial function. Use of lacZ reporters and quantitative RT-PCR analyses demonstrated that Ert1 regulates expression of its target genes in a manner that is highly redundant with other regulators of gluconeogenesis. Interestingly, in the presence of ethanol, Ert1 is a repressor of PDC1 encoding an important enzyme for fermentation. We also show that Ert1 binds directly to the PCK1 and PDC1 promoters. In summary, Ert1 is a novel factor involved in the regulation of gluconeogenesis as well as a key fermentation gene. Copyright © 2014 by the Genetics Society of America.

Relevance of ERTS-1 to the state of Ohio

NASA Technical Reports Server (NTRS)

Sweet, D. C. (Principal Investigator); Wells, T. L.; Wukelic, G. E.

1973-01-01

The author has identified the following significant results. To date, only one significant result has been reported for the Ohio ERTS program. This result relates to the proven usefulness of ERTS-1 imagery for mapping and inventorying strip-mined areas in southeastern Ohio. ERTS provides a tool for rapidly and economically acquiring an up-to-date inventory of strip-mined lands for state planning purposes which was not previously possible.

Techniques for computer-aided analysis of ERTS-1 data, useful in geologic, forest and water resource surveys. [Colorado Rocky Mountains

NASA Technical Reports Server (NTRS)

Hoffer, R. M.

1974-01-01

Forestry, geology, and water resource applications were the focus of this study, which involved the use of computer-implemented pattern-recognition techniques to analyze ERTS-1 data. The results have proven the value of computer-aided analysis techniques, even in areas of mountainous terrain. Several analysis capabilities have been developed during these ERTS-1 investigations. A procedure to rotate, deskew, and geometrically scale the MSS data results in 1:24,000 scale printouts that can be directly overlayed on 7 1/2 minutes U.S.G.S. topographic maps. Several scales of computer-enhanced "false color-infrared" composites of MSS data can be obtained from a digital display unit, and emphasize the tremendous detail present in the ERTS-1 data. A grid can also be superimposed on the displayed data to aid in specifying areas of interest.

The first Earth Resources Technology Satellite (ERTS-1)

NASA Technical Reports Server (NTRS)

Nordberg, W.

1973-01-01

The first Earth Resources Technology Satellite (ERTS-1) makes images of the earth's surface in four portions of the electromagnetic spectrum with sufficient spatial resolution and with a minimum of geometric distortions, so that these images may be applied experimentally to the study of geophysical processes relating to earth resources, to the exploration and conservation of these resources, and to the assessments of environmental stresses. During the first six months of operation, ERTS-1 has imaged 6.5 million square kilometers of the earth's surface every day, covering most major land masses and coastal zones as well as both polar regions of this planet. These images as well as the results of their analyses are available to all people throughout the world. Scientific investigators of all countries have been invited to participate in the utilization of ERTS-1 observations. Many of them have already demonstrated the great efficiency, economy, and reliability of making earth surveys from space.

Use of ERTS imagery in air pollution and marine biology studies, tasks 1 through 3

NASA Technical Reports Server (NTRS)

Copeland, G. E.; Ludwick, J. C.; Marshall, H. G. (Principal Investigator); Bandy, A. R.; Fleischer, P.; Hanna, W. J.; Gosink, T. A.; Bowker, D. W.

1972-01-01

There are no author-identified significant results in this report. The general suitability of ERTS imagery in detecting ground originated air pollution has proved to be excellent. The quality and resolution exceeded expectations and has permitted in some instances location of point sources to within a thousand feet. Suitable techniques have not yet been developed for determining or measuring area and line sources of air pollution. A major problem has been cloud cover that has persisted over the area of primary interest, the Chesapeake Bay. Work has been completed on mounting the shipboard transmissometer which will be used for investigations to relate the chlorophyll and suspended sediment content in the waters of the Lower Chesapeake Bay to ERTS-1 imagery. Water sampling, plankton analysis, and preparations for sea collection of water truth along the eastern continental shelf of the U.S. have been completed for use in comparisons with ERTS-1 data.

Cartographic quality of ERTS-1 images

NASA Technical Reports Server (NTRS)

Welch, R. I.

1973-01-01

Analyses of simulated and operational ERTS images have provided initial estimates of resolution, ground resolution, detectability thresholds and other measures of image quality of interest to earth scientists and cartographers. Based on these values, including an approximate ground resolution of 250 meters for both RBV and MSS systems, the ERTS-1 images appear suited to the production and/or revision of planimetric and photo maps of 1:500,000 scale and smaller for which map accuracy standards are compatible with the imaged detail. Thematic mapping, although less constrained by map accuracy standards, will be influenced by measurement thresholds and errors which have yet to be accurately determined for ERTS images. This study also indicates the desirability of establishing a quantitative relationship between image quality values and map products which will permit both engineers and cartographers/earth scientists to contribute to the design requirements of future satellite imaging systems.

Evaluation of surface water resources from machine-processing of ERTS multispectral data

NASA Technical Reports Server (NTRS)

Mausel, P. W.; Todd, W. J.; Baumgardner, M. F.; Mitchell, R. A.; Cook, J. P.

1976-01-01

The surface water resources of a large metropolitan area, Marion County (Indianapolis), Indiana, are studied in order to assess the potential value of ERTS spectral analysis to water resources problems. The results of the research indicate that all surface water bodies over 0.5 ha were identified accurately from ERTS multispectral analysis. Five distinct classes of water were identified and correlated with parameters which included: degree of water siltiness; depth of water; presence of macro and micro biotic forms in the water; and presence of various chemical concentrations in the water. The machine processing of ERTS spectral data used alone or in conjunction with conventional sources of hydrological information can lead to the monitoring of area of surface water bodies; estimated volume of selected surface water bodies; differences in degree of silt and clay suspended in water and degree of water eutrophication related to chemical concentrations.

The first Earth Resources Technology Satellite - Nearly two years of operation

NASA Technical Reports Server (NTRS)

Nordberg, W.

1974-01-01

A brief status report is given of the ERTS-1 satellite system as of June, 1974, and some applications of the ERTS-1 images are discussed. The multispectral images make it possible to identify or measure the quality and composition of water, the potential water content of snow, the moisture and possible composition of soils, the types and state of vegetation cover, and factors relating to stresses on the environment. The orthographic view of the earth provided by the satellite makes it possible to rapidly produce thematic maps, on a scale of 1:250,000, of most areas of the world. The regular, repetitive coverage provided by ERTS-1 every 18 days is important in areas such as water-supply and flood-damage studies. The use of ERTS-1 imagery for land-use planning, wetlands surveying, assessing marine resources, and observing processes such as desertification in the African Sahel is discussed.

Effects of vegetation canopy structure on remotely sensed canopy temperatures. [inferring plant water stress and yield

NASA Technical Reports Server (NTRS)

Kimes, D. S.

1979-01-01

The effects of vegetation canopy structure on thermal infrared sensor response must be understood before vegetation surface temperatures of canopies with low percent ground cover can be accurately inferred. The response of a sensor is a function of vegetation geometric structure, the vertical surface temperature distribution of the canopy components, and sensor view angle. Large deviations between the nadir sensor effective radiant temperature (ERT) and vegetation ERT for a soybean canopy were observed throughout the growing season. The nadir sensor ERT of a soybean canopy with 35 percent ground cover deviated from the vegetation ERT by as much as 11 C during the mid-day. These deviations were quantitatively explained as a function of canopy structure and soil temperature. Remote sensing techniques which determine the vegetation canopy temperature(s) from the sensor response need to be studied.

Natural history and galsulfase treatment in mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome)--10-year follow-up of patients who previously participated in an MPS VI Survey Study.

PubMed

Giugliani, Roberto; Lampe, Christina; Guffon, Nathalie; Ketteridge, David; Leão-Teles, Elisa; Wraith, James E; Jones, Simon A; Piscia-Nichols, Cheri; Lin, Ping; Quartel, Adrian; Harmatz, Paul

2014-08-01

Mucopolysaccharidosis VI (MPS VI) is a clinically heterogeneous and progressive disorder with multiorgan manifestations caused by deficient N-acetylgalactosamine-4-sulfatase activity. A cross-sectional Survey Study in individuals (n = 121) affected with MPS VI was conducted between 2001 and 2002 to establish demographics, urinary glycosaminoglycan (GAG) levels, and clinical progression of disease. We conducted a Resurvey Study (ClinicalTrials.gov: NCT01387854) to obtain 10-year follow-up data, including medical histories and clinical assessments (n = 59), and survival status over 12 years (n = 117). Patients received a mean (SD) of 6.8 (2.2) years of galsulfase ERT between baseline (Survey Study) and follow-up. ERT patients increased in height by 20.4 cm in the 4-7-year-old baseline age group and by 16.8 cm in the 8-12-year-old baseline age group. ERT patients <13 years-old demonstrated improvement in forced vital capacity (FVC) by 68% and forced expiratory volume in 1 sec (FEV1) by 55%, and those ≥13 years-old increased FVC by 12.8% and maintained FEV1. Patients with >200 µg/mg baseline uGAG levels increased FVC by 48% in the <13-year-old baseline age group and by 15% in the ≥13-year-old baseline age group. ERT patients who completed the 6-min walk test demonstrated a mean (SD) increase of 65.7 (100.6) m. Cardiac outcomes did not significantly improve or worsen. Observed mortality rate among naïve patients was 50% (7/14) and 16.5% (17/103) in the ERT group (unadjusted hazard ratio, 0.24; 95% CI, 0.10-0.59). Long-term galsulfase ERT was associated with improvements in pulmonary function and endurance, stabilized cardiac function and increased survival. © 2014 Wiley Periodicals, Inc.

Correlation of ERTS MSS data and earth coordinate systems

NASA Technical Reports Server (NTRS)

Malila, W. A. (Principal Investigator); Hieber, R. H.; Mccleer, A. P.

1973-01-01

The author has identified the following significant results. Experience has revealed a problem in the analysis and interpretation of ERTS-1 multispectral scanner (MSS) data. The problem is one of accurately correlating ERTS-1 MSS pixels with analysis areas specified on aerial photographs or topographic maps for training recognition computers and/or evaluating recognition results. It is difficult for an analyst to accurately identify which ERTS-1 pixels on a digital image display belong to specific areas and test plots, especially when they are small. A computer-aided procedure to correlate coordinates from topographic maps and/or aerial photographs with ERTS-1 data coordinates has been developed. In the procedure, a map transformation from earth coordinates to ERTS-1 scan line and point numbers is calculated using selected ground control points nad the method of least squares. The map transformation is then applied to the earth coordinates of selected areas to obtain the corresponding ERTS-1 point and line numbers. An optional provision allows moving the boundaries of the plots inward by variable distances so the selected pixels will not overlap adjacent features.

Facilitating the exploitation of ERTS imagery using snow enhancement techniques

NASA Technical Reports Server (NTRS)

Wobber, F. J. (Principal Investigator); Martin, K. R.; Sheffield, C.; Russell, O.; Amato, R. V.

1972-01-01

The author has identified the following significant results. Analysis of all available (Gemini, Apollo, Nimbus, NASA aircraft) small scale snow covered imagery has been conducted to develop and refine snow enhancement techniques. A detailed photographic interpretation of ERTS-simulation imagery covering the Feather River/Lake Tahoe area was completed and the 580-680nm. band was determined to be the optimum band for fracture detection. ERTS-1 MSS bands 5 and 7 are best suited for detailed fracture mapping. The two bands should provide more complete fracture detail when utilized in combination. Analysis of early ERTS-1 data along with U-2 ERTS simulation imagery indicates that snow enhancement is a viable technique for geological fracture mapping. A wealth of fracture detail on snow-free terrain was noted during preliminary analysis of ERTS-1 images 1077-15005-6 and 7, 1077-15011-5 and 7, and 1079-15124-5 and 7. A direct comparison of data yield on snow-free versus snow-covered terrain will be conducted within these areas following receipt of snow-covered ERTS-1 imagery.

Influence of female and male sex steroids on body composition in the rabbit model.

PubMed

Alexandersen, P; Hassager, C; Christiansen, C

2001-09-01

To study the influence on body composition of estrogen replacement therapy (ERT) in female rabbits and of replacement therapy with testosterone (TRT) in male rabbits using dual-energy X-ray absorptiometry (DEXA). Cholesterol-fed female and male rabbits receiving a weight-restricted diet (100 g/day) were used. Total lean tissue mass (LTM), total body fat tissue mass (FTM) and total tissue mass (TTM) were determined by DEXA at baseline, after which the animals were gonadectomized and treated with sex steroids. Soft body composition was then determined again after 30-31 weeks of treatment. Relative to controls, ERT with estradiol (E2) doses of 2 and 4 mg/day significantly increased LTM (p < 0.001), whereas E2 0.5 and 1 mg/day had a neutral effect on LTM. The change in fat mass, however, was not statistically significant between groups. In male rabbits, compared with castrated control rabbits, LTM decreased in testosterone-treated animals (by 7-12%; p < 0.001) but FTM decreased relatively more (by 66-79%; p < 0.0001). In both genders, body weight correlated with TTM as determined by DEXA (r = 0.89-0.91, p < 0.0001). In this in vivo model of growing rabbits, estrogen replacement significantly increased LTM in female animals, whereas testosterone replacement significantly decreased FTM in males, suggesting that soft body composition of both genders is significantly affected by replacement with sex steroids. Until comparable human data are available, it is speculated that similar changes in soft body composition may occur in humans treated with sex steroids.

Land use mapping and modelling for the Phoenix Quadrangle

NASA Technical Reports Server (NTRS)

Place, J. L. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Changes in the land use in the Phoenix (1:250,000 scale) Quadrangle in Arizona have been mapped using only the images from ERTS-1, tending to verify the utility of a land use classification system proposed for use with ERTS images. Seasonal changes were studied on successive ERTS-1 images, particularly large scale color composite transparencies for August, October, February, and May, and this seasonal variation aided delineation of land use boundaries. Types of equipment used to aid interpretation included color additive viewer, a twenty-power magnifier, a density slicer, and a diazo copy machine. A Zoom Transfer Scope was used for scale and photogrammetric adjustments. Types of changes detected have been: (1) cropland or rangeland developed as new residential areas; (2) rangeland converted to new cropland or to new reservoirs; and (3) possibly new activity by the mining industries. A map of land use previously compiled from air photos was updated in this manner. ERTS-1 images complemented air photos: the photos gave detail on a one-shot basis; the ERTS-1 images provided currency and revealed seasonal variation in vegetation which aided interpretation of land use.

Application of ERTS-A imagery to fracture related mine safety hazards in the coal mining industry

NASA Technical Reports Server (NTRS)

Wier, C. E.; Wobber, F. J. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The most important result to date is the demonstration of the special value of repetitive ERTS-1 multiband coverage for detecting previously unknown fracture lineaments despite the presence of a deep glacial overburden. The Illinois Basin is largely covered with glacial drift and few rock outcrops are present. A contribution to the geological understanding of Illinois and Indiana has been made. Analysis of ERTS-1 imagery has provided useful information to the State of Indiana concerning the surface mined lands. The contrast between healthy vegetation and bare ground as imaged by Band 7 is sharp and substantial detail can be obtained concerning the extent of disturbed lands, associated water bodies, large haul roads, and extent of mined lands revegetation. Preliminary results of analysis suggest a reasonable correlation between image-detected fractures and mine roof fall accidents for a few areas investigated. ERTS-1 applications to surface mining operations appear probable, but further investigations are required. The likelihood of applying ERTS-1 derived fracture data to improve coal mine safety in the entire Illinois Basin is suggested from studies conducted in Indiana.

Skylab study of water quality. [Kansas reservoirs

NASA Technical Reports Server (NTRS)

Yarger, H. L. (Principal Investigator); Mccauley, J. R.

1974-01-01

The author has identified the following significant results. Analysis of S-190A imagery from 1 EREP pass over 3 reservoirs in Kansas establishes a strong linear correlation between the red/green radiance ratio and suspended solids. This result compares quite favorably to ERTS MSS CCT results. The linear fits RMS for Skylab is 6 ppm as compared to 12 ppm for ERTS. All of the ERTS satellite passes yielded fairly linear results with typical RMS values of 12 ppm. However, a few of the individual passes did yield RMS values of 5 or 6 ppm which is comparable to the one Skylab pass analyzed. In view of the cloudy conditions in the Skylab photos, yet good results, the indications are that S-190A may do somewhat better than the ERTS MSS in determining suspended load. More S-190A data is needed to confirm this. As was the case with the ERTS MSS, the Skylab S-190A showed no strong correlation with other water quality parameters. S-190B photos because of their high resolution can provide much first look information regarding relative degrees of turbidity within various parts of large lakes and among smaller bodies of water.

A demonstration of ERTS-1 analog and digital techniques applied to strip mining in Maryland and West Virginia

NASA Technical Reports Server (NTRS)

Anderson, A. T.; Schubert, J.

1974-01-01

The largest contour strip mining operations in western Maryland and West Virginia are located within the Georges Creek and the Upper Potomac Basins. These two coal basins lie within the Georges Creek (Wellersburg) syncline. The disturbed strip mine areas were delineated with the surrounding geological and vegetation features using ERTS-1 data in both analog (imagery) and digital form. The two digital systems used were: (1) the ERTS-Analysis system, a point-by-point digital analysis of spectral signatures based on known spectral values, and (2) the LARS Automatic Data Processing System. The digital techniques being developed will later be incorporated into a data base for land use planning. These two systems aided in efforts to determine the extent and state of strip mining in this region. Aircraft data, ground verification information, and geological field studies also aided in the application of ERTS-1 imagery to perform an integrated analysis that assessed the adverse effects of strip mining. The results indicated that ERTS can both monitor and map the extent of strip mining to determine immediately the acreage affected and indicate where future reclamation and revegetation may be necessary.

PFLOTRAN-E4D: A parallel open source PFLOTRAN module for simulating time-lapse electrical resistivity data

DOE Office of Scientific and Technical Information (OSTI.GOV)

Johnson, Timothy C.; Hammond, Glenn E.; Chen, Xingyuan

Time-lapse electrical resistivity tomography (ERT) is finding increased application for remotely monitoring processes occurring in the near subsurface in three-dimensions (i.e. 4D monitoring). However, there are few codes capable of simulating the evolution of subsurface resistivity and corresponding tomographic measurements arising from a particular process, particularly in parallel and with an open source license. Herein we describe and demonstrate an electrical resistivity tomography module for the PFLOTRAN subsurface simulation code, named PFLOTRAN-E4D. The PFLOTRAN-E4D module operates in parallel using a dedicated set of compute cores in a master-slave configuration. At each time step, the master processes receives subsurface states frommore » PFLOTRAN, converts those states to bulk electrical conductivity, and instructs the slave processes to simulate a tomographic data set. The resulting multi-physics simulation capability enables accurate feasibility studies for ERT imaging, the identification of the ERT signatures that are unique to a given process, and facilitates the joint inversion of ERT data with hydrogeological data for subsurface characterization. PFLOTRAN-E4D is demonstrated herein using a field study of stage-driven groundwater/river water interaction ERT monitoring along the Columbia River, Washington, USA. Results demonstrate the complex nature of changes subsurface electrical conductivity, in both the saturated and unsaturated zones, arising from water table changes and from river water intrusion into the aquifer. The results also demonstrate the sensitivity of surface based ERT measurements to those changes over time. PFLOTRAN-E4D is available with the PFLOTRAN development version with an open-source license at https://bitbucket.org/pflotran/pflotran-dev .« less

PFLOTRAN-E4D: A parallel open source PFLOTRAN module for simulating time-lapse electrical resistivity data

NASA Astrophysics Data System (ADS)

Johnson, Timothy C.; Hammond, Glenn E.; Chen, Xingyuan

2017-02-01

Time-lapse electrical resistivity tomography (ERT) is finding increased application for remotely monitoring processes occurring in the near subsurface in three-dimensions (i.e. 4D monitoring). However, there are few codes capable of simulating the evolution of subsurface resistivity and corresponding tomographic measurements arising from a particular process, particularly in parallel and with an open source license. Herein we describe and demonstrate an electrical resistivity tomography module for the PFLOTRAN subsurface flow and reactive transport simulation code, named PFLOTRAN-E4D. The PFLOTRAN-E4D module operates in parallel using a dedicated set of compute cores in a master-slave configuration. At each time step, the master processes receives subsurface states from PFLOTRAN, converts those states to bulk electrical conductivity, and instructs the slave processes to simulate a tomographic data set. The resulting multi-physics simulation capability enables accurate feasibility studies for ERT imaging, the identification of the ERT signatures that are unique to a given process, and facilitates the joint inversion of ERT data with hydrogeological data for subsurface characterization. PFLOTRAN-E4D is demonstrated herein using a field study of stage-driven groundwater/river water interaction ERT monitoring along the Columbia River, Washington, USA. Results demonstrate the complex nature of subsurface electrical conductivity changes, in both the saturated and unsaturated zones, arising from river stage fluctuations and associated river water intrusion into the aquifer. The results also demonstrate the sensitivity of surface based ERT measurements to those changes over time. PFLOTRAN-E4D is available with the PFLOTRAN development version with an open-source license at https://bitbucket.org/pflotran/pflotran-dev.

Geologic hypotheses of Lake Tanganyika region, Zaire, drawn from ERTS imagery

NASA Technical Reports Server (NTRS)

Wolyce, U.; Ilunga, S.

1974-01-01

Based on initial work in the Lake Tanganyika area of eastern Zaire, it has been concluded that ERTS imagery is extremely useful for reconnaissance level geologic mapping and analysis in this region of the humid tropics. In particular, ERTS imagery has proven useful for recognizing and mapping regional structural units, for recognizing major structural features, and for arriving at some preliminary hypotheses about the mineral potential of the area. Results so far indicate that ERTS imagery can make a major contribution to the development of the mineral resources of the country. Research has concentrated on applications of ERTS imagery in the field of cartography, geology, forestry, hydrology and agriculture. For the work in geology, a test site was chosen in eastern Zaire on the shore of Lake Tanganyika in the vicinity of the Lukuga River. This area was selected because of its varied geology and the existence of two frames of cloud-free ERTS imagery.

Relevance of ERTS-1 to the State of Ohio. [agriculture, forestry, land use, mining, and environmental quality management

NASA Technical Reports Server (NTRS)

Sweet, D. C.; Pincura, P. G.; Wukelic, G. E. (Principal Investigator)

1974-01-01

The author has identified the following significant results. During the first year of project effort the ability of ERTS-1 imagery to be used for mapping and inventorying strip-mined areas in south eastern Ohio, the potential of using ERTS-1 imagery in water quality and coastal zone management in the Lake Erie region, and the extent that ERTS-1 imagery could contribute to localized (metropolitan/urban), multicounty, and overall state land use needs were experimentally demonstrated and reported as significant project results. Significant research accomplishments were achieved in the technological development of manual and computerized methods to extract multi-feature information as well as singular feature information from ERTS-1 data as is exemplified by the forestry transparency overlay. Fabrication of an image transfer device to superimpose ERTS-1 data onto existing maps and other data sources was also a significant analytical accomplishment.

Evaluation and comparison of ERTS measurements of major crops and soil associations for selected test sites in the central United States. [Texas, Indiana, Kansas, Iowa, Nebraska, and North Dakota

NASA Technical Reports Server (NTRS)

Baumgardner, M. F. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Multispectral scanner data obtained by ERTS-1 over six test sites in the Central United States were analyzed and interpreted. ERTS-1 data for some of the test sites were geometrically corrected and temporally overlayed. Computer-implemented pattern recognition techniques were used in the analysis of all multispectral data. These techniques were used to evaluate ERTS-1 data as a tool for soil survey. Geology maps and land use inventories were prepared by digital analysis of multispectral data. Identification and mapping of crop species and rangelands were achieved throught the analysis of 1972 and 1973 ERTS-1 data. Multiple dates of ERTS-1 data were examined to determine the variation with time of the areal extent of surface water resources on the Southern Great Plain.

Exploitation of ERTS-1 imagery utilizing snow enhancement techniques

NASA Technical Reports Server (NTRS)

Wobber, F. J.; Martin, K. R.

1973-01-01

Photogeological analysis of ERTS-simulation and ERTS-1 imagery of snowcovered terrain within the ERAP Feather River site and within the New England (ERTS) test area provided new fracture detail which does not appear on available geological maps. Comparative analysis of snowfree ERTS-1 images has demonstrated that MSS Bands 5 and 7 supply the greatest amount of geological fracture detail. Interpretation of the first snow-covered ERTS-1 images in correlation with ground snow depth data indicates that a heavy blanket of snow (more than 9 inches) accentuates major structural features while a light "dusting", (less than 1 inch) accentuates more subtle topographic expressions. An effective mail-based method for acquiring timely ground-truth (snowdepth) information was established and provides a ready correlation of fracture detail with snow depth so as to establish the working limits of the technique. The method is both efficient and inexpensive compared with the cost of similarly scaled direct field observations.

Mapping coastal vegetation, land use and environmental impact from ERTS-1. [Delaware Bay area

NASA Technical Reports Server (NTRS)

Klemas, V. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Vegetation map overlays at a scale of 1:24,000 compiled by multispectral analysis from NASA aircraft imagery for all of Delaware's wetlands are being used as ground truth for ERTS-1 mapping and by state agencies for wetlands management. Six major vegetation species were discriminated and mapped, including percentages of minor species. Analogue enhancements of wetlands vegetation and dredge-fill operations have been produced using General Electric's GEMS data processing and ERTS-1 false color composites. Digital, thematic land use, and vegetation mapping of entire Delaware Bay area is in progress using Bendix Corporation's Earth Resources Data System and ERTS-1 digital tapes. Statistical evaluation of target-group selection reliability has been completed. Three papers have been published on ERTS-1 coastal vegetation and land use. Local and state officials are participating in the ERTS-1 program as co-investigators.

Effective use of ERTS multisensor data in the Great Plains

NASA Technical Reports Server (NTRS)

Myers, V. I. (Principal Investigator)

1972-01-01

The author has identified the following significant results. One unique advantage of ERTS imagery for delineating soil associations is the large area that can be scanned with one photo. Although soil associations usually are published at scales of 1:500,000 or 1:1,000,000, the delineations are drawn on much larger scale maps covering small pieces of the scene and then pieced together. Alluvial areas are usually swollen out of proportion to other soil areas. ERTS imagery puts alluvial areas into their proper size. A second feature of ERTS imagery is that a soil association map constructed with its aid assures that the cartographic level of the associations is more nearly the same. Another advantage of ERTS imagery is that the actual shape and configuration of soil associations are apparent. Also with ERTS imagery significant new delineations may become apparent which were missed when constructing soil association maps from conventional large scale photos.

Ratio maps of iron ore deposits Atlantic City district, Wyoming

NASA Technical Reports Server (NTRS)

Vincent, R. K.

1973-01-01

Preliminary results of a spectral rationing technique are shown for a region at the southern end of the Wind River Range, Wyoming. Digital ratio graymaps and analog ratio images have been produced for the test site, but ground truth is not yet available for thorough interpretation of these products. ERTS analog ratio images were found generally better than either ERTS single-channel images or high altitude aerial photos for the discrimination of vegetation from non-vegetation in the test site region. Some linear geological features smaller than the ERTS spatial resolution are seen as well in ERTS ratio and single-channel images as in high altitude aerial photography. Geochemical information appears to be extractable from ERTS data. Good preliminary quantitative agreement between ERTS-derived ratios and laboratory-derived reflectance ratios of rocks and minerals encourage plans to use lab data as training sets for a simple ratio gating logic approach to automatic recognition maps.

A high-fat diet activates oncogenic Kras and COX2 to induce development of pancreatic ductal adenocarcinoma in mice.

PubMed

Philip, Bincy; Roland, Christina L; Daniluk, Jaroslaw; Liu, Yan; Chatterjee, Deyali; Gomez, Sobeyda B; Ji, Baoan; Huang, Haojie; Wang, Huamin; Fleming, Jason B; Logsdon, Craig D; Cruz-Monserrate, Zobeida

2013-12-01

Obesity is a risk factor for pancreatic ductal adenocarcinoma (PDAC), but it is not clear how obesity contributes to pancreatic carcinogenesis. The oncogenic form of KRAS is expressed during early stages of PDAC development and is detected in almost all of these tumors. However, there is evidence that mutant KRAS requires an additional stimulus to activate its full oncogenic activity and that this stimulus involves the inflammatory response. We investigated whether the inflammation induced by a high-fat diet, and the accompanying up-regulation of cyclooxygenase-2 (COX2), increases Kras activity during pancreatic carcinogenesis in mice. We studied mice with acinar cell-specific expression of KrasG12D (LSL-Kras/Ela-CreERT mice) alone or crossed with COX2 conditional knockout mice (COXKO/LSL-Kras/Ela-CreERT). We also studied LSL-Kras/PDX1-Cre mice. All mice were fed isocaloric diets with different amounts of fat, and a COX2 inhibitor was administered to some LSL-Kras/Ela-CreERT mice. Pancreata were collected from mice and analyzed for Kras activity, levels of phosphorylated extracellular-regulated kinase, inflammation, fibrosis, pancreatic intraepithelial neoplasia (PanIN), and PDACs. Pancreatic tissues from LSL-Kras/Ela-CreERT mice fed high-fat diets (HFDs) had increased Kras activity, fibrotic stroma, and numbers of PanINs and PDACs than LSL-Kras/Ela-CreERT mice fed control diets; the mice fed the HFDs also had shorter survival times than mice fed control diets. Administration of a COX2 inhibitor to LSL-Kras/Ela-CreERT mice prevented these effects of HFDs. We also observed a significant reduction in survival times of mice fed HFDs. COXKO/LSL-Kras/Ela-CreERT mice fed HFDs had no evidence for increased numbers of PanIN lesions, inflammation, or fibrosis, as opposed to the increases observed in LSL-Kras/Ela-CreERT mice fed HFDs. In mice, an HFD can activate oncogenic Kras via COX2, leading to pancreatic inflammation and fibrosis and development of PanINs and PDAC. This mechanism might be involved in the association between risk for PDAC and HFDs. Copyright © 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.

Coupling of hydrogeological models with hydrogeophysical data to characterize seawater intrusion and shallow geothermal systems

NASA Astrophysics Data System (ADS)

Beaujean, J.; Kemna, A.; Engesgaard, P. K.; Hermans, T.; Vandenbohede, A.; Nguyen, F.

2013-12-01

While coastal aquifers are being stressed due to climate changes and excessive groundwater withdrawals require characterizing efficiently seawater intrusion (SWI) dynamics, production of geothermal energy is increasingly being used to hinder global warming. To study these issues, we need both robust measuring technologies and reliable predictions based on numerical models. SWI models are currently calibrated using borehole observations. Similarly, geothermal models depend mainly on the temperature field at few locations. Electrical resistivity tomography (ERT) can be used to improve these models given its high sensitivity to TDS and temperature and its relatively high lateral resolution. Inherent geophysical limitations, such as the resolution loss, can affect the overall quality of the ERT images and also prevent the correct recovery of the desired hydrochemical property. We present an uncoupled and coupled hydrogeophysical inversion to calibrate SWI and thermohydrogeologic models using ERT. In the SWI models, we demonstrate with two synthetic benchmarks (homogeneous and heterogeneous coastal aquifers) the ability of cumulative sensitivity-filtered ERT images using surface-only data to recover the hydraulic conductivity. Filtering of ERT-derived data at depth, where resolution is poorer, and the model errors make the dispersivity more difficult to estimate. In the coupled approach, we showed that parameter estimation is significantly improved because regularization bias is replaced by forward modeling only. Our efforts are currently focusing on applying the uncoupled/coupled approaches on a real life case study using field data from the site of Almeria, SE Spain. In the thermohydrogeologic models, the most sensitive hydrologic parameters responsible for heat transport are estimated from surface ERT-derived temperatures and ERT resistance data. A real life geothermal experiment that took place on the Campus De Sterre of Ghent University, Belgium and a synthetic case are tested. They consist in a thermal injection and storage of water in a shallow sandy aquifer. The use of a physically-based constraint accounting for the difference in conductivity between the formation and the tap injected water and based on the hydrogeological model calibrated first on temperatures is necessary to improve the parameter estimation. Results suggest that time-lapse ERT data may be limited but useful information for estimating groundwater flow and transport parameters for both the convection and conduction phases.

2-D Electrical Resistivity Tomography (ERT) Assessment of Ground Failure in Urban Area

NASA Astrophysics Data System (ADS)

Nordiana, M. M.; Bery, A. A.; Taqiuddin, Z. M.; Jinmin, M.; Abir, I. A.

2018-04-01

This study was carried out to assess the foundation defects around an urban area in Selangor, Malaysia using 2-D electrical resistivity tomography (ERT). The affected structure is a three storey houses and having severe foundation-based cracks. Six 2-D ERT survey lines with 5 m minimum electrode spacing using Pole-dipole array were executed parallel to building’s wall. Four boreholes were conducted to identify the depth to competent layer to verify the 2-D ERT results. Inversion model of 2-D resistivity show that the study area consists of two main zones. The first zone is a low resistivity value (<100 Ωm), which appears to be a zone that is fully saturated with sandy silt and this could be an influence factor the increasing water level because sandy silt is highly permeable in nature and alluvium (silt, sand and clay), boulder (1200-3500 Ωm) or highly weathered with the resistivity values of 100-1000 Ωm at 20-70 m depth. The second zone is the granite bedrock of more than 3500 Ωm with depth greater than 70 m. These results were complimented and confirmed by borehole records. The ERT and borehole record suggest that the clay, sand, saturated zone, highly weathered zone and boulders at foundation depths may lead to ground movements which affected the stability of the building.

ERTS-A data as a teaching and research tool in the Department of Geology

NASA Technical Reports Server (NTRS)

Grybeck, D. (Principal Investigator)

1973-01-01

The author has identified the following significant results. ERTS-1 prints have been used extensively in a geology of Alaska class to give a basic framework of the geology of the state. In addition, they have been intermittantly used in such diverse classes as: (1) Economic Geology (e.g. the Sn-bearing granites of the Seward Peninsula are particularly noticeable due to their wide contact metamorphic aureoles.) (2) A canned geology of Alaska lecture which has been given to two different introductory geology courses. (3) Structural Geology (e.g. the Fairweather and Denali faults are striking obvious). It was found most convenient for larger classes to prepare 35mm slides of the ERTS-1 prints that are used in conjunction with slides of the topographic and geologic maps at about the same scale. Thus the emphasis has been in integration of the ERTS-1 material into existing courses. As such, the ERTS-1 data has provided a unique and striking viewpoint that never fails to initiate favorable comment. In addition, prints have been examined by numerous researchers to develop a regional, integrated overview of such varied topics as regional geology to a background for local geologic mapping to studies of ore deposits and to the definition of a formation to be studied in detail at its type locality.

The ERTS-1 investigation (ER-600). Volume 2: ERTS-1 coastal/estuarine analysis. [Galveston Bay, Texas

NASA Technical Reports Server (NTRS)

Erb, R. B.

1974-01-01

The Coastal Analysis Team of the Johnson Space Center conducted a 1-year investigation of ERTS-1 MSS data to determine its usefulness in coastal zone management. Galveston Bay, Texas, was the study area for evaluating both conventional image interpretation and computer-aided techniques. There was limited success in detecting, identifying and measuring areal extent of water bodies, turbidity zones, phytoplankton blooms, salt marshes, grasslands, swamps, and low wetlands using image interpretation techniques. Computer-aided techniques were generally successful in identifying these features. Aerial measurement of salt marshes accuracies ranged from 89 to 99 percent. Overall classification accuracy of all study sites was 89 percent for Level 1 and 75 percent for Level 2.

Long-term electrical resistivity monitoring of recharge-induced contaminant plume behavior.

PubMed

Gasperikova, Erika; Hubbard, Susan S; Watson, David B; Baker, Gregory S; Peterson, John E; Kowalsky, Michael B; Smith, Meagan; Brooks, Scott

2012-11-01

Geophysical measurements, and electrical resistivity tomography (ERT) data in particular, are sensitive to properties that are related (directly or indirectly) to hydrological processes. The challenge is in extracting information from geophysical data at a relevant scale that can be used to gain insight about subsurface behavior and to parameterize or validate flow and transport models. Here, we consider the use of ERT data for examining the impact of recharge on subsurface contamination at the S-3 ponds of the Oak Ridge Integrated Field Research Challenge (IFRC) site in Tennessee. A large dataset of time-lapse cross-well and surface ERT data, collected at the site over a period of 12 months, is used to study time variations in resistivity due to changes in total dissolved solids (primarily nitrate). The electrical resistivity distributions recovered from cross-well and surface ERT data agrees well, and both of these datasets can be used to interpret spatiotemporal variations in subsurface nitrate concentrations due to rainfall, although the sensitivity of the electrical resistivity response to dilution varies with nitrate concentration. Using the time-lapse surface ERT data interpreted in terms of nitrate concentrations, we find that the subsurface nitrate concentration at this site varies as a function of spatial position, episodic heavy rainstorms (versus seasonal and annual fluctuations), and antecedent rainfall history. These results suggest that the surface ERT monitoring approach is potentially useful for examining subsurface plume responses to recharge over field-relevant scales. Published by Elsevier B.V.

Influence of bedrock topography on the runoff generation under use of ERT data

NASA Astrophysics Data System (ADS)

Kiese, Nina; Loritz, Ralf; Allroggen, Niklas; Zehe, Erwin

2017-04-01

Subsurface topography has been identified to play a major role for the runoff generation in different hydrological landscapes. Sinks and ridges in the bedrock can control how water is stored and transported to the stream. Detecting the subsurface structure is difficult and laborious and frequently done by auger measurements. Recently, the geophysical imaging of the subsurface by Electrical Resistivity Tomography (ERT) gained much interest in the field of hydrology, as it is a non-invasive method to collect information on the subsurface characteristics and particularly bedrock topography. As it is impossible to characterize the subsurface of an entire hydrological landscape using ERT, it is of key interest to identify the bedrock characteristics which dominate runoff generation to adapt and optimize the sampling design to the question of interest. For this study, we used 2D ERT images and auger measurements, collected on different sites in the Attert basin in Luxembourg, to characterize bedrock topography using geostatistics and shed light on those aspects which dominate runoff generation. Based on ERT images, we generated stochastic bedrock topographies and implemented them in a physically-based 2D hillslope model. With this approach, we were able to test the influence of different subsurface structures on the runoff generation. Our results highlight that ERT images can be useful for hydrological modelling. Especially the connection from the hillslope to the stream could be identified as important feature in the subsurface for the runoff generation whereas the microtopography of the bedrock seemed to be less relevant.

Uncertainty in training image-based inversion of hydraulic head data constrained to ERT data: Workflow and case study

NASA Astrophysics Data System (ADS)

Hermans, Thomas; Nguyen, Frédéric; Caers, Jef

2015-07-01

In inverse problems, investigating uncertainty in the posterior distribution of model parameters is as important as matching data. In recent years, most efforts have focused on techniques to sample the posterior distribution with reasonable computational costs. Within a Bayesian context, this posterior depends on the prior distribution. However, most of the studies ignore modeling the prior with realistic geological uncertainty. In this paper, we propose a workflow inspired by a Popper-Bayes philosophy that data should first be used to falsify models, then only be considered for matching. We propose a workflow consisting of three steps: (1) in defining the prior, we interpret multiple alternative geological scenarios from literature (architecture of facies) and site-specific data (proportions of facies). Prior spatial uncertainty is modeled using multiple-point geostatistics, where each scenario is defined using a training image. (2) We validate these prior geological scenarios by simulating electrical resistivity tomography (ERT) data on realizations of each scenario and comparing them to field ERT in a lower dimensional space. In this second step, the idea is to probabilistically falsify scenarios with ERT, meaning that scenarios which are incompatible receive an updated probability of zero while compatible scenarios receive a nonzero updated belief. (3) We constrain the hydrogeological model with hydraulic head and ERT using a stochastic search method. The workflow is applied to a synthetic and a field case studies in an alluvial aquifer. This study highlights the importance of considering and estimating prior uncertainty (without data) through a process of probabilistic falsification.

40 CFR 60.2795 - In what form can I submit my reports?

Code of Federal Regulations, 2011 CFR

2011-07-01

... using the Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert_tool.html). ... or Before November 30, 1999 Model Rule-Recordkeeping and Reporting § 60.2795 In what form can I...

The effect of tigecycline and ertapenem against clinical isolates of Brucella melitensis detected by E-test on different media.

PubMed

Tanyel, E; Coban, A Y; Fisgin, N Tasdelen; Tulek, N

2010-01-01

In this study, in vitro activity of tigecycline (TIG) and ertapenem (ERT) against clinical isolates of Brucella melitensis and the effect of different media on in vitro test results were investigated. The in vitro effects of TIG and ERT to 38 B. melitensis isolates were comparatively investigated in brucella agar and 5% sheep blood agar. MIC value of ERT was 0.032 μg/mL in 23 of 38 and 20 of 38 isolates on blood and brucella agar, respectively. Minimum inhibitory concentration values of TIG were substantially different ranging between 0.064-0.25 μg/mL on blood agar. However, MIC values of TIG were similar on brucella agar with 0.25 μg/mL in 15 of 38 isolates and 0.5 μg/mL in 10 of 38 isolates. In conclusion, although ERT and TIG were effective against B. melitensis isolates in vitro, further studies are needed in order to determine the use of these novel drugs in treatment of brucellosis.

Evaluation of the application of ERTS-1 data to the regional land use planning process. [Northeast Wisconsin

NASA Technical Reports Server (NTRS)

Clapp, J. L. (Principal Investigator); Green, T., III; Hanson, G. F.; Kiefer, R. W.; Niemann, B. J., Jr.

1974-01-01

The author has identified the following significant results. Employing simple and economical extraction methods, ERTS can provide valuable data to the planners at the state or regional level with a frequency never before possible. Interactive computer methods of working directly with ERTS digital information show much promise for providing land use information at a more specific level, since the data format production rate of ERTS justifies improved methods of analysis.

Utilization of ERTS-1 data in geological evaluation, regional planning, forest management, and water management in North Carolina

NASA Technical Reports Server (NTRS)

Welby, C. W. (Principal Investigator); Lammi, J. O.; Carson, R. J., III

1974-01-01

The author has identified the following significant results. ERTS-1 imagery has been evaluated for use in resource planning and management in North Carolina, and found to be useful for general reconnaissance purposes in forestry, geology, and water resources work. It has also been used for studying large-scale transient phenomena such as river plumes and movement of sediment in the sounds. ERTS-1 imagery has been an aid to geologic and land-use mapping. Stereoscopes, projectors of various kinds, and microscopes have proved useful instruments for the kinds of data acquisition needed by resource planners and managers.

40 CFR 60.2795 - In what form can I submit my reports?

Code of Federal Regulations, 2012 CFR

2012-07-01

... (CDX) by using the Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert_tool.html). ... Reporting § 60.2795 In what form can I submit my reports? Submit initial, annual, and deviation reports...

ERTS imagery applied to Alaskan coastal problems. [surface water circulation

NASA Technical Reports Server (NTRS)

Wright, F. F.; Sharma, G. D.; Burbank, D. C.; Burns, J. J.

1974-01-01

Along the Alaska coast, surface water circulation is relatively easy to study with ERTS imagery. Highly turbid river water, sea ice, and fluvial ice have proven to be excellent tracers of the surface waters. Sea truth studies in the Gulf of Alaska, Cook Inlet, Bristol Bay, and the Bering Strait area have established the reliability of these tracers. ERTS imagery in the MSS 4 and 5 bands is particularly useful for observing lower concentrations of suspended sediment, while MSS 6 data is best for the most concentrated plumes. Ice features are most clearly seen on MSS 7 imagery; fracture patterns and the movement of specific floes can be used to map circulation in the winter when runoff is restricted, if appropriate allowance is made for wind influence. Current patterns interpreted from satellite data are only two-dimensional, but since most biological activity and pollution are concentrated near the surface, the information developed can be of direct utility. Details of Alaska inshore circulation of importance to coastal engineering, navigation, pollution studies, and fisheries development have been clarified with satellite data. ERTS has made possible the analysis of circulation in many parts of the Alaskan coast.

Application of ecological, geological and oceanographic ERTS-1 imagery to Delaware's coastal resources planning

NASA Technical Reports Server (NTRS)

Klemas, V. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Communities containing five different coastal vegetation species, developed marshlands, and fresh water impoundments have been identified in ERTS-1 images. Suspended sediment and circulation patterns in imagery from five ERTS-1 passes over Delaware Bay have been enhanced and correlated with predicted current patterns. Conclusions reached are: (1) ERTS-1 is suitable platform for observing suspended sediment patterns and water masses synoptically over large areas. (2) Suspended sediment acts as a natural tracer allowing photointerpreters to deduce gross current circulation patterns from ERTS-1 imagery. (3) Under atmospheric conditions encountered along the East Coast of the United States MSS band 5 seems to give the best representation of sediment load in upper one meter of water column. (4) In the ERTS-1 imagery the sediment patterns are delineated by three to four neighboring shades of grey. (5) Negative transparencies of the ERTS-1 images give better contrast whenever the suspended sediment tones fall within the first few steps of the grey scale. (6) Color density slicing helps delineate the suspended sediment patterns more clearly and differentiate turbidity levels.

Reliable Genetic Labeling of Adult-Born Dentate Granule Cells Using Ascl1 CreERT2 and Glast CreERT2 Murine Lines.

PubMed

Yang, Sung M; Alvarez, Diego D; Schinder, Alejandro F

2015-11-18

Newly generated dentate granule cells (GCs) are relevant for input discrimination in the adult hippocampus. Yet, their precise contribution to information processing remains unclear. To address this question, it is essential to develop approaches to precisely label entire cohorts of adult-born GCs. In this work, we used genetically modified mice to allow conditional expression of tdTomato (Tom) in adult-born GCs and characterized their development and functional integration. Ascl1(CreERT2);CAG(floxStopTom) and Glast(CreERT2);CAG(floxStopTom) mice resulted in indelible expression of Tom in adult neural stem cells and their lineage upon tamoxifen induction. Whole-cell recordings were performed to measure intrinsic excitability, firing behavior, and afferent excitatory connectivity. Developing GCs were also staged by the expression of early and late neuronal markers. The slow development of adult-born GCs characterized here is consistent with previous reports using retroviral approaches that have revealed that a mature phenotype is typically achieved after 6-8 weeks. Our findings demonstrate that Ascl1(CreERT2) and Glast(CreERT2) mouse lines enable simple and reliable labeling of adult-born GC lineages within restricted time windows. Therefore, these mice greatly facilitate tagging new neurons and manipulating their activity, required for understanding adult neurogenesis in the context of network remodeling, learning, and behavior. Our study shows that Ascl1(CreERT2) and Glast(CreERT2) mice lines can be used to label large cohorts of adult-born dentate granule cells with excellent time resolution. Neurons labeled in this manner display developmental and functional profiles that are in full agreement with previous findings using thymidine analogs and retroviral labeling, thus providing an alternative approach to tackle fundamental questions on circuit remodeling. Because of the massive neuronal targeting and the simplicity of this method, genetic labeling will contribute to expand research on adult neurogenesis. Copyright © 2015 the authors 0270-6474/15/3515379-12$15.00/0.

Interpretation of ERTS-MSS images of a Savanna area in eastern Colombia

NASA Technical Reports Server (NTRS)

Elberson, G. W. W.

1973-01-01

The application of ERTS-1 imagery for extrapolating existing soil maps into unmapped areas of the Llanos Orientales of Colombia, South America is discussed. Interpretations of ERTS-1 data were made according to conventional photointerpretation techniques. Most units delineated in the existing reconnaissance soil map at a scale of 1:250,000 could be recognized and delineated in the ERTS image. The methods of interpretation are described and the results obtained for specific areas are analyzed.

Impact of ERTS-1 images on management of New Jersey's coastal zone

NASA Technical Reports Server (NTRS)

Feinberg, E. B.; Yunghans, R. S.; Stitt, J. A.; Mairs, R. L.

1974-01-01

The thrust of New Jersey's ERTS investigation is development of procedures for operational use of ERTS-1 data by the Department of Environmental Protection in the management of the State's coastal zone. Four major areas of concern were investigated: detection of land use changes in the coastal zone; monitoring of offshore waste disposal; siting of ocean outfalls; and allocation of funds for shore protection. ERTS imagery was not useful for shore protection purposes; it was of limited practical value in the evaluation of offshore waste disposal and ocean outfall siting. However, ERTS imagery shows great promise for operational detection of land use changes in the coastal zone. Some constraints for practical change detection have been identified.

Earth Resources Technology Satellite data collection project, ERTS - Bolivia. [thematic mapping

NASA Technical Reports Server (NTRS)

Brockmann, C. E.

1974-01-01

The Earth Resources Technology Satellite program of Bolivia has developed a multidisciplinary project to carry out investigations in cartography and to prepare various thematic maps. In cartography, investigations are being carried out with the ERTS-1 images and with existing maps, to determine their application to the preparation of new cartographic products on one hand and on the other to map those regions where the cartography is still deficient. The application of the MSS images to the geological mapping has given more than satisfactory results. Working with conventional photointerpretation, it has been possible to prepare regional geological maps, tectonic maps, studies relative to mining, geomorphological maps, studies relative to petroleum exploration, volcanological maps and maps of hydrologic basins. In agriculture, the ERTS images are used to study land classification and forest and soils mapping.

Phase I/II Trial of Adeno-Associated Virus–Mediated Alpha-Glucosidase Gene Therapy to the Diaphragm for Chronic Respiratory Failure in Pompe Disease: Initial Safety and Ventilatory Outcomes

PubMed Central

Smith, Barbara K.; Collins, Shelley W.; Conlon, Thomas J.; Mah, Cathryn S.; Lawson, Lee Ann; Martin, Anatole D.; Fuller, David D.; Cleaver, Brian D.; Clément, Nathalie; Phillips, Dawn; Islam, Saleem; Dobjia, Nicole

2013-01-01

Abstract Pompe disease is an inherited neuromuscular disease caused by deficiency of lysosomal acid alpha-glucosidase (GAA) leading to glycogen accumulation in muscle and motoneurons. Cardiopulmonary failure in infancy leads to early mortality, and GAA enzyme replacement therapy (ERT) results in improved survival, reduction of cardiac hypertrophy, and developmental gains. However, many children have progressive ventilatory insufficiency and need additional support. Preclinical work shows that gene transfer restores phrenic neural activity and corrects ventilatory deficits. Here we present 180-day safety and ventilatory outcomes for five ventilator-dependent children in a phase I/II clinical trial of AAV-mediated GAA gene therapy (rAAV1-hGAA) following intradiaphragmatic delivery. We assessed whether rAAV1-hGAA results in acceptable safety outcomes and detectable functional changes, using general safety measures, immunological studies, and pulmonary functional testing. All subjects required chronic, full-time mechanical ventilation because of respiratory failure that was unresponsive to both ERT and preoperative muscle-conditioning exercises. After receiving a dose of either 1×1012 vg (n=3) or 5×1012 vg (n=2) of rAAV1-hGAA, the subjects' unassisted tidal volume was significantly larger (median [interquartile range] 28.8% increase [15.2–35.2], p<0.05). Further, most patients tolerated appreciably longer periods of unassisted breathing (425% increase [103–851], p=0.08). Gene transfer did not improve maximal inspiratory pressure. Expected levels of circulating antibodies and no T-cell-mediated immune responses to the vector (capsids) were observed. One subject demonstrated a slight increase in anti-GAA antibody that was not considered clinically significant. These results indicate that rAAV1-hGAA was safe and may lead to modest improvements in volitional ventilatory performance measures. Evaluation of the next five patients will determine whether earlier intervention can further enhance the functional benefit. PMID:23570273

Gingival overgrowth in Pompe disease: a case report.

PubMed

de Gijt, J Pieter; van Capelle, Carine I; Oosterhuis, J Wolter; van der Ploeg, Ans T; van der Wal, Karel G H

2011-08-01

Pompe disease, or glycogen storage disease type 2, is a rare inheritable metabolic disease caused by a deficiency of the lysosomal enzyme acid α-glucosidase. Patients with the classic infantile form of Pompe disease present with symptoms during the first 3 months after birth, and most will die within their first year. Recently, enzyme replacement therapy (ERT) with recombinant human α-glucosidase became commercially available for Pompe disease. This is a case report of an 8-year-old girl with the infantile form of Pompe disease who is one of the longest survivors through ERT. The patient was tetraplegic when she started ERT. At age 3 years, she developed massive gingival overgrowth and could not close her mouth, prompting a reduction of the gingival overgrowth surgically. We expected that massive accumulation of glycogen would explain the gingival overgrowth. However, histopathology of the gingiva tissue showed marked glycogen accumulation in smooth muscle cells of the arteries, but the glycogen content in fibroblasts did not exceed that of control individuals. Further, there was an increase of immature collagen in the connective tissue, and signs of a mild chronic inflammation. We concluded that glycogen storage is not a direct causative factor of gingival overgrowth in our patient. Chronic inflammation, dryness of the gingiva, or even the minimal glycogen accumulation in the fibroblasts may have played a role. Copyright © 2011 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

Lower Urinary Tract Symptoms and Incontinence in Children with Pompe Disease.

PubMed

Ajay, Divya; McNamara, Erin R; Austin, Stephanie; Wiener, John S; Kishnani, Priya

2016-01-01

Pompe disease (PD) is a disorder of lysosomal glycogen storage. The introduction of enzyme replacement therapy (ERT) has shifted the focus of care from survival to quality of life. The presence of lower urinary tract symptoms (LUTS) and incontinence has not been previously described in children with PD. Children with PD followed in the Duke Lysosomal Storage Disease Clinic completed a validated bladder control symptom score (BCSS) and additional questions regarding urinary tract infections (UTIs), giggle, and stress incontinence. Descriptive statistics were used to discriminate urinary symptoms between gender, age, and different types of PD. Sixteen of 23 children (aged 4-14 years) seen in our clinic participated. Seven were girls; ten had classic infantile PD, two atypical infantile PD, and four childhood presentation late-onset PD (LOPD). When stratified by PD subtype, median BCSS was worst for the classic PD subtype followed by atypical PD and LOPD. Daytime urinary incontinence accompanied by constipation was noted in six. Eight reported urinary incontinence with laughing: giggle incontinence in six and stress incontinence in two. Four girls reported a history of UTI. Longitudinal follow-up in 11 patients showed stable BCSS in six, improvement in three, and worsening in two. Worsening corresponded with changes in bowel function and improvement with increase in ERT dose or treatment of constipation. LUTS and incontinence are common in children with PD with greater symptoms noted with infantile-type PD. Improved bowel function and increase in ERT dose may lead to improvements in BCSS.

Application of ERTS-1 data to the protection and management of New Jersey's coastal environment

NASA Technical Reports Server (NTRS)

Yunghans, R. S. (Principal Investigator); Feinberg, E. B.; Stitt, J. A.; Mairs, R. L.; Wobber, F. J.; Macomber, R. T.; Stanczuk, D. T.; Thibult, D.

1974-01-01

The author has identified the following significant results. Quasi-operational information products for coastal zone management have been prepared using ERTS-1 imagery and collateral aerial photography. These products were applied to the practical regulation, protection, and management of New Jersey's coastal environment. Procedures were developed for the operational use of ERTS-1 data products within New Jersey's Department of Environmental Protection. Successful analysis and product preparation for operational needs centered on four major coastal resource problem areas: (1) detection of environmental changes in coastal areas, (2) siting of ocean outfalls, (3) monitoring of offshore waste disposal, and (4) calculation of recession rates along the Atlantic Shore. The utility and monetary benefits derived from ERTS and aircraft imagery for each problem area have been determined. The NJDEP estimates the possibility of $620,000 yearly savings through the use of an operational ERTS system and a one-time savings of $2.8 million on current or planned projects if a truly operational ERTS type satellite were available.

Coastal applications of the ERTS-1 satellite imagery

NASA Technical Reports Server (NTRS)

Magoon, O. T. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. Samples are given of the possible applications of ERTS-1 imagery to coastal and nearshore studies. Briefly discussed are: (1) obtaining regional views of extended coastal areas; (2) distribution of sediments; (3) coastal configurations and changes; (4) barrier islands; (5) underwater penetration, and (6) coastal waves.

Sea King Mk. 50 Helicopter Sonar Dynamics Study. A Simplified Control Systems Mathematical Model

DTIC Science & Technology

1979-02-01

cable mode signal (CAB P) comprises: (i) The propotional . trimmed, longitudinal cable angle error signal, THE ERT. THE ERT itself comprises: (a) The...used for body axes in the aircraft. (vi) Because the model has not yet been validated, the behaviour shown still has to be confirmed as an accurate

The Dark Side of Emotion in the Classroom: Emotional Processes as Mediators of Teacher Communication Behaviors and Student Negative Emotions

ERIC Educational Resources Information Center

Mazer, Joseph P.; McKenna-Buchanan, Timothy P.; Quinlan, Margaret M.; Titsworth, Scott

2014-01-01

Based on emotional response theory (ERT), recent researchers have observed connections between teachers' communication behaviors and students' emotional reactions. In the present study, we further elaborated ERT by exploring the effects of teacher communication behaviors and emotional processes on discrete negative emotions, including anger,…

Extra-Role Time, Burnout, and Commitment: The Power of Promises Kept

ERIC Educational Resources Information Center

Brown, Lori A.; Roloff, Michael E.

2011-01-01

This study examines the relationships of extra-role time (ERT) behavior with burnout and occupational commitment among teachers through the lens of conservation of resources (COR) theory. Results reveal that teachers who invest in more ERT are also more likely to experience burnout and decreased commitment to teaching than those who invest less.…

Monitoring ocean dumping with ERTS-1 data

NASA Technical Reports Server (NTRS)

Wezernak, C. T.; Roller, N.

1973-01-01

The results of an analysis of ERTS-1 data for the New York Bight collected on 16 August 1972 are described. Results are presented which show acid-iron wastes, sewage sludge, suspended solids, and major water mass boundary features in the study area. The potential of satellite remote sensing for monitoring large scale events such as ocean dumping is discussed.

A reconnaissance space sensing investigation of crustal structure for a strip from the eastern Sierra Nevada to the Colorado Plateau

NASA Technical Reports Server (NTRS)

Liggett, M. A. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Studies were conducted in key field areas in the Sierra Nevada, the Basin Range Province and the Colorado Plateau to evaluate the origins and significance of geologic and structural anomalies expressed in the ERTS-1 data. The investigation included development of image enhancement and analysis techniques and comparison of remote sensing data available over the test site. The ERTS-1 MSS imagery has proven to be an effective tool for studying the interrelationsships between Cenozoic tectonic patterns and the distributions of Cenozoic plutonism and volcanism, seismic activity, geologic hazards, and known mineral, geothermal and ground water resources. Recommendations are made for applications of ERTS-1 data to natural and resource exploration and management.

The FRALIT teledetection program, using the ERTS-A satellite, for the oceanic littoral of France

NASA Technical Reports Server (NTRS)

Verger, F. (Principal Investigator); Cazabat, C.; Demathieu, P.; Dupuis, J.

1972-01-01

There are no author-identified significant results in this report. The French Atlantic Littoral program, utilizing data from the ERTS-1 satellite, is considered. It involves teledetection of the French shoreline along the Atlantic Ocean and English Channel. A description is given of the ERTS-1 orbit and the satellite itself, including the attitude control system, and the data acquisition and transmission equipment. The geographic extent of the area covered by the program is delineated and the subjects studied are enumerated. These include the geomorphology, pedology, hydrology, and vegetation of the maritime marshes; sedimentology, morphology, and hydrology of the intertidal zones; and transport of material in suspension to the mouths of the Seine, the Loire, and the Gironde as a part of the coastal waters study.

Evaluate ERTS imagery for mapping and detection of changes of snowcover on land and on glaciers. [Washington, Alaska, British Columbia, and U.S.S.R.

NASA Technical Reports Server (NTRS)

Meier, M. F. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The standard error of measurement of snow covered areas in major drainage basins in the Cascade Range, Washington, using single measurements of ERTS-1 images, was found to range from 11% to 7% during a typical melt season, but was as high as 32% in midwinter. Many dangerous glacier situations in Alaska, Yukon, and British Columbia were observed on ERTS-1 imagery. Glacier dammed lakes in Alaska are being monitored by ERTS-1. Embayments in tidal glaciers show changes detectable by ERTS-1. Surges of Russell and Tweedsmuir Glaciers, now in progress, are clearly visible. The Tweedsmuir surge is likely to dam the large Alsek River by mid-November, producing major floods down-river next summer. An ERTS-1 image of the Pamir Mountains, Tadjik S.S.R., shows the surging Medvezhii (Bear) Glacier just after its surge of early summer which dammed the Abdukagor Valley creating a huge lake and later a flood in the populous Vanch River Valley. A map was compiled from an ERTS-1 image of the Lowell Glacier after its recent surge, compared with an earlier map compiled from pain-stakingly compiled from a mosaic of many aerial photographs, in a total elapsed time of 1.5 hours. This demonstrates the value of ERTS-1 for rapid mapping of large features.

Marine resources. [coastal processes, ice, oceanography, and living marine resources

NASA Technical Reports Server (NTRS)

Tilton, E. L., III

1974-01-01

Techniques have been developed for defining coastal circulation patterns using sediment as a natural tracer, allowing the formulation of new circulation concepts in some geographical areas and, in general, a better capability for defining the seasonal characteristics of coastal circulation. An analytical technique for measurement of absolute water depth based upon the ratios of two MSS channels has been developed. Suspended sediment has found wide use as a tracer, but a few investigators have reported limited success in measuring the type and amount of sediment quantitatively from ERTS-1 digital data. Significant progress has been made in developing techniques for using ERTS-1 data to locate, identify, and monitor sea and lake ice. Ice features greater than 70 meters in width can be detected, and both arctic and antarctic icebergs have been identified. In the application area of living marine resources, the use of ERTS-1 image-density patterns as a potential indicator of fish school location has been demonstrated for one coastal commercial resource, menhaden. ERTS-1 data have been used to locate ocean current boundaries using ERTS-1 image-density enhancement, and some techniques are under development for measurement of suspended particle concentration and chlorophyll concentration. The interrelationship of water color and surface characteristics (sea state) are also being studied to improve spectral and spatial interpretive techniques.

Study of application of ERTS-A imagery to fracture-related mine safety hazards in the coal mining industry

NASA Technical Reports Server (NTRS)

Wier, C. E.; Wobber, F. J.; Russell, O. R.; Martin, K. R. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Mined land reclamation analysis procedures developed within the Indiana portion of the Illinois Coal Basin were independently tested in Ohio utilizing 1:80,000 scale enlargements of ERTS-1 image 1029-15361-7 (dated August 21, 1972). An area in Belmont County was selected for analysis due to the extensive surface mining and the different degrees of reclamation occurring in this area. Contour mining in this area provided the opportunity to extend techniques developed for analysis of relatively flat mining areas in Indiana to areas of rolling topography in Ohio. The analysts had no previous experience in the area. Field investigations largely confirmed office analysis results although in a few areas estimates of vegetation percentages were found to be too high. In one area this error approximated 25%. These results suggest that systematic ERTS-1 analysis in combination with selective field sampling can provide reliable vegetation percentage estimates in excess of 25% accuracy with minimum equipment investment and training. The utility of ERTS-1 for practical and reasonably reliable update of mined lands information for groups with budget limitations is suggested. Many states can benefit from low cost updates using ERTS-1 imagery from public sources.

An interdisciplinary analysis of ERTS data for Colorado mountain environments using ADP techniques. An early analysis of ERTS-1 data

NASA Technical Reports Server (NTRS)

Hoffer, R. M.; Landgrebe, D. A. (Principal Investigator); Goodrick, F. E.

1972-01-01

There are no author-identified significant results in this report. The principal problem encountered has been the lack of good quality, small scale baseline photography for the test areas. Analysis of the ERTS-1 data for the San Juan Site will emphasize development of a preliminary spectral classification defining grass cover categories, and then selection of subframes for intensive investigation of the forestry, geologic, and hydrologic properties of the area. Primary work has been devoted to the selection and digitization of areas for topographic modeling, and compilation of ground based data maps necessary for computer analysis. Study effort has emphasized: geomorphic features; macro-vegetation; micro-vegetation; snow-hydrology; insect/disease damage; and blow-down. Analysis of a frame of the Lake Texoma area indicates a great deal of potential in the analysis and interpretation of ERTS imagery. Preliminary results of investigations of geologic, forest, range, cropland, and water resources of the area are summarized.

Change in land use in the Phoenix (1:250,000) Quadrangle, Arizona between 1970 and 1972: Successful use of proposed land use classification system

NASA Technical Reports Server (NTRS)

Place, J. L.

1973-01-01

Changes in land use in the Phoenix (1:250,000 scale) Quadrangle in Arizona have been mapped using only the images from ERTS-1, tending to verify the utility of a land use classification system proposed for use with ERTS images. The period of change investigated was from November 1970 to late summer or early fall, 1972. Seasonal changes also were studied using successive ERTS images. Types of equipment used to aid interpretation included a color additive viewer, a twenty-power magnifier, a density slicer, and a diazo copy machine for making ERTS color composites in hard copy. Types of changes detected have been: (1) cropland or rangeland developed for new residential areas; (2) rangeland converted to new cropland; and (3) possibly new areas of industrial or commercial development. A map of land use previously compiled from air photos was updated in this manner.

Natural resource inventories and management applications in the Great Basin. [Nevada vegetation and wildlands

NASA Technical Reports Server (NTRS)

Tueller, P. T.; Lorain, G.; Halvorson, R. M.

1974-01-01

ERTS-1 resolution capabilities and repetitive coverage have allowed the acquisition of several statewide inventories of natural resource features not previously completed or that could not be completed in any other way. Familiarity with landform, tone, pattern and other converging factors, along with multidate imagery, has been required. Nevada's vegetation has been mapped from ERTS-1. Dynamic characteristics of the landscape have been studied. Sequential ERTS-1 imagery has proved its usefulness for mapping vegetation, following vegetation phenology changes, monitoring changes in lakes and reservoirs (including water quality), determining changes in surface mining use, making fire fuel estimates and determining potential hazard, mapping the distribution of rain and snow events, making range readiness determinations, monitoring marshland management practices and other uses. Feasibility has been determined, but details of incorporating the data in management systems awaits further research and development. The need is to accurately define the steps necessary to extract required or usable information from ERTS imagery and fit it into on-going management programs.

Monitoring California's forage resource using ERTS-1 and supporting aircraft data

NASA Technical Reports Server (NTRS)

Carneggie, D. M.; Degloria, S. D.

1973-01-01

NASA's Earth Resource Technology Satellite (ERTS-1) launched July 23, 1972, offers for the first time the unique capabilities for regional monitoring of forage plant conditions. The repetitive coverage every 18 days, the synoptic view and the real-time recovery of the imagery for immediate analysis, combine to make the ERTS satellite a valuable tool for improving the evaluation of our rangeland resources. Studies presently underway at the University of California, Berkeley (sponsored jointly by NASA and the Bureau of Land Management), seek to determine if imagery obtained from high altitude aircraft and spacecraft (ERTS) can provide: (1) a means for monitoring the growth and development of annual and perennial range plants in California, and for determining the time and the rate of initial plant growth (germination) and terminal plant growth (maturation and senescence); (2) a means for determining or predicting the relative amount of forage that is produced; and (3) a means for mapping rangeland areas having different forage producing capabilities.

California coast nearshore processes study. [nearshore currents, sediment transport, estuaries, and river discharge

NASA Technical Reports Server (NTRS)

Pirie, D. M.; Steller, D. D. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Large scale sediment plumes from intermittent streams and rivers form detectable seasonal patterns on ERTS-1 imagery. The ocean current systems, as plotted from three California coast ERTS mosaics, were identified. Offshore patterns of sediment in areas such as the Santa Barbara Channel are traceable. These patterns extend offshore to heretofore unanticipated ranges as shown on the ERTS-1 imagery. Flying spot scanner enhancements of NASA tapes resulted in details of subtle and often invisible (to the eye) nearshore features. The suspended sediments off San Francisco and in Monterey Bay are emphasized in detail. These are areas of extremely changeable offshore sediment transport patterns. Computer generated contouring of radiance levels resulted in maps that can be used in determining surface and nearsurface suspended sediment distribution. Tentative calibrations of ERTS-1 spectral brightness against sediment load have been made using shipboard measurements. Information from the combined enhancement and interpretation techniques is applicable to operational coastal engineering programs.

BOREAS Landsat MSS Imagery: Digital Counts

NASA Technical Reports Server (NTRS)

Hall, Forrest G. (Editor); Nickeson, Jaime (Editor); Strub, Richard; Newcomer, Jeffrey A.

2000-01-01

The Boreal Ecosystem-Atmospheric Study (BOREAS) Staff Science Satellite Data Acquisition Program focused on providing the research teams with the remotely sensed satellite data products they needed to compare and spatially extend point results. The Earth Resources Technology Satellite (ERTS) Program launched the first of a series of satellites (ERTS-1) in 1972. Part of the NASA Earth Resources Survey Program, the ERTS Program and the ERTS satellites were later renamed Landsat to better represent the civil satellite program's prime emphasis on remote sensing of land resources. Landsat satellites 1 through 5 carry the Multispectral Scanner (MSS) sensor. Canada for Remote Sensing (CCRS) and BOREAS personnel gathered a set of MSS images of the BOREAS region from Landsat satellites 1, 2, 4, and 5 covering the dates of 21 Aug 1972 to 05 Sep 1988. The data are provided in binary image format files of various formats. The Landsat MSS imagery is available from the Earth Observing System Data and Information System (EOSDIS) Oak Ridge National Laboratory (ORNL) Distributed Active Archive Center (DAAC).

Loss of TGF-β signaling in osteoblasts increases basic-FGF and promotes prostate cancer bone metastasis.

PubMed

Meng, Xiangqi; Vander Ark, Alexandra; Daft, Paul; Woodford, Erica; Wang, Jie; Madaj, Zachary; Li, Xiaohong

2018-04-01

TGF-β plays a central role in prostate cancer (PCa) bone metastasis, and it is crucial to understand the bone cell-specific role of TGF-β signaling in this process. Thus, we used knockout (KO) mouse models having deletion of the Tgfbr2 gene specifically in osteoblasts (Tgfbr2 Col1CreERT KO) or in osteoclasts (Tgfbr2 LysMCre KO). We found that PCa-induced bone lesion development was promoted in the Tgfbr2 Col1CreERT KO mice, but was inhibited in the Tgfbr2 LysMCre KO mice, relative to their respective control Tgfbr2 FloxE2 littermates. Since metastatic PCa cells attach to osteoblasts when colonized in the bone microenvironment, we focused on the mechanistic studies using the Tgfbr2 Col1CreERT KO mouse model. We found that bFGF was upregulated in osteoblasts from PC3-injected tibiae of Tgfbr2 Col1CreERT KO mice and correlated with increased tumor cell proliferation, angiogenesis, amounts of cancer-associated fibroblasts and osteoclasts. In vitro studies showed that osteoblastogenesis was inhibited, osteoclastogenesis was stimulated, but PC3 viability was not affected, by bFGF treatments. Lastly, the increased PC3-induced bone lesions in Tgfbr2 Col1CreERT KO mice were significantly attenuated by blocking bFGF using neutralizing antibody, suggesting bFGF is a promising target inhibiting bone metastasis. Copyright © 2018 Elsevier B.V. All rights reserved.

A Retrospective, Multicenter Study of the Tolerance of Induction Chemotherapy With Docetaxel, Cisplatin, and 5-Fluorouracil Followed by Radiotherapy With Concomitant Cetuximab in 46 Cases of Squamous Cell Carcinoma of the Head and Neck

DOE Office of Scientific and Technical Information (OSTI.GOV)

Buiret, Guillaume, E-mail: guillaume.buiret@laposte.ne; Service de biostatistique, Hospices Civils de Lyon, Lyon; Combe, Claire

2010-06-01

Purpose: To investigate, in a multicenter study, the tolerance of induction chemotherapy (ICT) and external radiotherapy (ERT) with concomitant cetuximab in the treatment of patients with squamous cell carcinoma of the head and neck (SCCHN). Patients and Methods: Clinical data from 46 patients with Stage III or IV nonmetastatic SCCHN who received docetaxel, cisplatin, and 5-fluorouracil as ICT, followed by ERT with concomitant cetuximab, were retrospectively analyzed. Clinical safety (weight, allergy, mucositis, and dermatitis) and paraclinical safety (levels of hemoglobin, polynuclear neutrophils, and creatinine clearance) were studied. The primary objective was the proportion of patients who completed the protocol. Results:more » The percentage of patients completing ICT was 73.9%, ERT 93.5%, and cetuximab 69.6%. Induction chemotherapy was better tolerated than that previously reported. The rates of temporary suspensions of radiation (39.1%, mean duration of 13 days) and hospitalization (26.1%) during ERT with concomitant cetuximab were high. Weight loss during treatment (21.4% of patients lost >10% of their body weight), radiodermatitis, and radiomucositis were the main causes of temporary suspension of treatment, although Grade 4 dermatitis was not experienced. There were no allergic reactions to cetuximab. Conclusion: The completed protocol rate for SCCHN patients receiving ICT and ERT with concomitant cetuximab is high and the toxicity acceptable. Future improvements to protocol will be possible through early action and systematic implementation of nutritional support coupled with antibiotic treatment upon the first signs of radiodermatitis. These data could be useful for prospective studies on the safety and efficacy of this protocol.« less

Combined application of GPR and ERT for the assessment of a wall structure at the Heptapyrgion fortress (Thessaloniki, Greece)

NASA Astrophysics Data System (ADS)

Angelis, Dimitrios; Tsourlos, Panagiotis; Tsokas, Gregory; Vargemezis, George; Zacharopoulou, Georgia; Power, Christopher

2018-05-01

Non-destructive investigation of monuments can be an extremely valuable tool to evaluate potential structural defects and assist in developing any restoration plans. In this work, both Ground Penetrating Radar (GPR) and Electrical Resistivity Tomography (ERT) techniques were applied to a tower wall of the Heptapyrgion fortress located in Thessaloniki, Greece, which was facing significant moisture problems. GPR cross sections, mainly obtained with a 500 MHz centre frequency antenna, and ERT profiles were collected along the same survey grid on the tower wall. The gprMax numerical solver was used for the GPR forward modelling. In addition, an auxiliary program was used to design and import into gprMax complicated structures and this allowed to simulate more realistically the wall defects and moisture. The GPR simulator was used to assess and optimize the field data acquisition and processing parameters, and to assist in interpreting the GPR cross sections. The ERT sections were inverted as individual 2D lines and also, as a full 3D dataset. The final GPR and ERT data were jointly interpreted in view of the studied problem as results of both methods are highly correlated. A high moisture content area at the eastern part of the wall was identified in both GPR and ERT data, along with the interface between different phases of construction. Through the GPR data we were also able to delineate possible structural defects (cracks, small voids) which was not possible with just using the ERT data. Furthermore, a very good matching was evident between the simulated GPR modelling results incorporating field-interpreted features, and the actual field GPR results, thereby validating the proposed data interpretation. The overall survey and modelling approach produces results that are in a very good agreement between them and proved very useful in accessing the wall structure.

Mapping Site Remediation with Electrical Resistivity Tomography Explored via Coupled-Model Simulations

NASA Astrophysics Data System (ADS)

Power, C.; Gerhard, J. I.; Tsourlos, P.; Giannopoulos, A.

2011-12-01

Remediation programs for sites contaminated with dense non-aqueous phase liquids (DNAPLs) would benefit from an ability to non-intrusively map the evolving volume and extent of the DNAPL source zone. Electrical resistivity tomography (ERT) is a well-established geophysical tool, widely used outside the remediation industry, that has significant potential for mapping DNAPL source zones. However, that potential has not been realized due to challenges in data interpretation from contaminated sites - in either a qualitative or quantitative way. The objective of this study is to evaluate the potential of ERT to map realistic, evolving DNAPL source zones within complex subsurface environments during remedial efforts. For this purpose, a novel coupled model was developed that integrates a multiphase flow model (DNAPL3D-MT), which generates realistic DNAPL release scenarios, with 3DINV, an ERT model which calculates the corresponding resistivity response. This presentation will describe the developed model coupling methodology, which integrates published petrophysical relationships to generate an electrical resistivity field that accounts for both the spatial heterogeneity of subsurface soils and the evolving spatial distribution of fluids (including permeability, porosity, clay content and air/water/DNAPL saturation). It will also present an example in which the coupled model was employed to explore the ability of ERT to track the remediation of a DNAPL source zone. A field-scale, three-dimensional release of chlorinated solvent DNAPL into heterogeneous clayey sand was simulated, including the subsurface migration and subsequent removal of the DNAPL source zone via dissolution in groundwater. Periodic surveys of this site via ERT applied at the surface were then simulated and inversion programs were used to calculate the subsurface distribution of electrical properties. This presentation will summarize this approach and its potential as a research tool exploring the range of site conditions under which ERT may prove useful in aiding DNAPL site remediation. Moreover, it is expected to provide a cost-effective avenue to test optimum ERT data acquisition, inversion and interpretative tools at contaminated sites.

Monitoring Arctic Sea ice using ERTS imagery. [Bering Sea, Beaufort Sea, Canadian Archipelago, and Greenland Sea

NASA Technical Reports Server (NTRS)

Barnes, J. C.; Bowley, C. J.

1974-01-01

Because of the effect of sea ice on the heat balance of the Arctic and because of the expanding economic interest in arctic oil and other minerals, extensive monitoring and further study of sea ice is required. The application of ERTS data for mapping ice is evaluated for several arctic areas, including the Bering Sea, the eastern Beaufort Sea, parts of the Canadian Archipelago, and the Greenland Sea. Interpretive techniques are discussed, and the scales and types of ice features that can be detected are described. For the Bering Sea, a sample of ERTS imagery is compared with visual ice reports and aerial photography from the NASA CV-990 aircraft.

Application of NASA ERTS-1 satellite imagery in coastal studies

NASA Technical Reports Server (NTRS)

Magoon, O. T.; Berg, D. W. (Principal Investigator); Hallermeier, R. J.

1973-01-01

There are no author-identified significant results in this report. Review of ERTS-1 imagery indicates that it contains information of great value in coastal engineering studies. A brief introduction is given to the methods by which imagery is generated, and examples of its application to coastal engineering. Specific applications discussed include study of the movement of coastal and nearshore sediment-laden water masses and information for planning and construction in remote areas of the world.

Develop a land use-peak runoff classification system for highway engineering purposes

NASA Technical Reports Server (NTRS)

Stoeckeler, E. G. (Principal Investigator); Farrell, R. S.; Woodman, R. G.

1974-01-01

The author has identified the following significant results. Based on the detail study of the Sunkhaze Stream Watershed, it is believed that good detailed drainage studies can be derived from repetitive ERTS imagery. Land use maps tailored to hydrologic study can be prepared from ERTS imagery. Significant changes in the Sunkhaze Stream and Otter Stream Watersheds at spring flood conditions have given important information on the causes for flooding in the town of Bradley.

40 CFR 60.5235 - What reports must I submit?

Code of Federal Regulations, 2013 CFR

2013-07-01

... Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert_tool.html/) or other compatible... Existing Sewage Sludge Incineration Units Model Rule-Recordkeeping and Reporting § 60.5235 What reports... reporting period specified in paragraph (c)(3) of this section. You must submit your first annual compliance...

Fracture mapping and strip mine inventory in the Midwest by using ERTS-1 imagery

NASA Technical Reports Server (NTRS)

Wier, C. W.; Wobber, F. J.; Russell, O. R.; Amato, R. V.

1973-01-01

Analysis of the ERTS-1 imagery and high-altitude infrared photography indicates that useful fracture data can be obtained in Indiana and Illinois despite a glacial till cover. ERTS MSS bands 5 and 7 have proven most useful for fracture mapping in coal-bearing rocks in this region. Preliminary results suggest a reasonable correlation between image-detected fractures and mine roof-fall accidents. Information related to surface mined land, such as disturbed area, water bodies, and kind of reclamation, has been derived from the analysis of ERTS imagery.

Terrain type recognition using ERTS-1 MSS images

NASA Technical Reports Server (NTRS)

Gramenopoulos, N.

1973-01-01

For the automatic recognition of earth resources from ERTS-1 digital tapes, both multispectral and spatial pattern recognition techniques are important. Recognition of terrain types is based on spatial signatures that become evident by processing small portions of an image through selected algorithms. An investigation of spatial signatures that are applicable to ERTS-1 MSS images is described. Artifacts in the spatial signatures seem to be related to the multispectral scanner. A method for suppressing such artifacts is presented. Finally, results of terrain type recognition for one ERTS-1 image are presented.

Forestry, geology and hydrological investigations from ERTS-1 imagery in two areas of Ecuador, South America

NASA Technical Reports Server (NTRS)

Moreno, N. V. (Principal Investigator)

1973-01-01

The author has identified the following significant results. In the Oriente area, well-drained forests containing commercially valuable hardwoods can be recognized confidently and delineated quickly on the ERTS imagery. In the tropical rainforest, ERTS can provide an abundance of inferential information about large scale geologic structures. ERTS imagery is better than normal aerial photography for recognizing linears. The imagery is particularly useful for updating maps of the distributary system of the Guagas River Basin and of any other river with a similarly rapid changing channel pattern.

Relevance of ERTS-1 to the State of Ohio. [environmental monitoring and resources management

NASA Technical Reports Server (NTRS)

Sweet, D. C.; Pincura, P. G.; Wukelic, G. E. (Principal Investigator)

1974-01-01

The author has identified the following significant results. During the first year of project effort the ability of ERTS-1 imagery to be used for mapping and inventorying strip-mined areas in southeastern Ohio, the potential of using ERTS-1 imagery in water quality and coastal zone management in the Lake Erie region, and the extent that ERTS-1 imagery could contribute to localized (metropolitan/urban), multicounty, and overall state land use needs were experimentally demonstrated and reported as significant project results.

Relevance of ERTS-1 to the state of Ohio

NASA Technical Reports Server (NTRS)

Sweet, D. C.; Wells, T. L.; Wukelic, G. E. (Principal Investigator)

1973-01-01

The author has identified the following significant results. During the first six months of project effort the ability of ERTS-1 imagery to be used for mapping and inventorying strip-mined areas in southeastern Ohio was reported as a significant project result. During this reporting period, the potential of using ERTS-1 imagery in water quality and coastal zone management of Lake Erie became apparent and the extent that ERTS-1 imagery could contribute to localized (metropolitan/urban), multicounty, and overall state land use needs was experimentally demonstrated.

Application of ERTS-1 imagery in the Vermont-New York dispute over pollution of Lake Champlain

NASA Technical Reports Server (NTRS)

Lind, A. O. (Principal Investigator)

1973-01-01

The author has identified the following significant results. ERTS-1 imagery and a composite map derived from ERTS-1 imagery were presented as evidence in a U.S. Supreme Court case involving the pollution of an interstate water body (Lake Champlain). A pollution problem generated by a large paper mill forms the basis of the suit (Vermont vs. International Paper Co. and State of New York) and ERTS-1 imagery shows the effluent pattern on the lake surface as extending into Vermont during three different times.

Coordination and establishment of centralized facilities and services of the University of Alaska ERTS survey of the Alaskan environment

NASA Technical Reports Server (NTRS)

Belon, A. E. (Principal Investigator); Miller, J. M.

1973-01-01

The author has identified the following significant results. The objective of this project is to provide a focus for the entire University of Alaska ERTS-1 effort (12 projects covering 10 disciplines and involving 8 research institutes and science departments). Activities have been concentrated on the implementation of the project's three primary functions: (1) coordination and management of the U of A ERTS-1 program, including management of the flow of data and data products; (2) acquisition, installation, test, operation, and maintanence of centralized facilities for processing ERTS-1, aircraft, and ground truth data; and (3) development of photographic and digital techniques for processing and interpreting ERTS-1 and aircraft data. With minor exceptions these three functions are now well-established and working smoothly.

Conditions for Productive Learning in Networked Learning Environments: A Case Study from the VO@NET Project

ERIC Educational Resources Information Center

Ryberg, Thomas; Koottatep, Suporn; Pengchai, Petch; Dirckinck-Holmfeld, Lone

2006-01-01

In this article we bring together experiences from two international research projects: the Kaleidoscope ERT research collaboration and the VO@NET project. We do this by using a shared framework identified for cross-case analyses within the Kaleidoscope ERT to analyse a particular case in the VO@NET project, a training course called "Green…

Evaluation of ERTS-1 imagery for mapping Quaternary deposits and landforms in the Great Plains and Midwest

NASA Technical Reports Server (NTRS)

Morrison, R. B. (Principal Investigator); Hallberg, G. R.

1973-01-01

The author has identified the following significant results. The main landform associations and larger landforms are readily identifiable on the better images and commonly the gross associations of surficial Quaternary deposits also can be determined primarily by information on landforms and soils (obtained by analysis of stream dissection and drainage and stream-divide patterns, land use patterns, etc.). Maps showing the Quaternary geologic-terrain units that can be distinguished on the ERTS-1 images are being prepared for study areas in Illinois, Iowa, Missouri, Kansas, Nebraska, and South Dakota. Preliminary maps of 1:1,000,000 scale are included for three of the study areas: the Grand Island and Fremont, Nebraska, and the Davenport, Iowa-Illinois, 1 deg x 2 deg quadrangles. These maps exemplify the first phase of investigations, which consists of identifying and mapping landform and land use characteristics and geologic-surficial materials directly from the ERTS-1 images alone, with no additional information. These maps show that commonly the boundaries of geologic-terrain units can be delineated more accurately on ERTS-1 images than on topographic maps at 1:250,000 scale.

A study to explore the use of orbital remote sensing to determine native arid plant distribution. [Arizona Regional Ecological Test Site

NASA Technical Reports Server (NTRS)

Mcginnies, W. G. (Principal Investigator); Lepley, L. K.; Haase, E. F.; Conn, J. S.; Musick, H. B.; Foster, K. E.

1974-01-01

The author has identified the following significant results. It is possible to determine, from ERTS imagery, native arid plant distribution. Using techniques of multispectral masking and extensive fieldwork, three native vegetation communities were defined and mapped in the Avra Valley study area. A map was made of the Yuma area with the aid of ground truth correlations between areas of desert pavement visible on ERTS images and unique vegetation types. With the exception of the Yuma soil-vegetation correlation phenomena, only very gross differentiations of desert vegetation communities can be made from ERTS data. Vegetation communities with obvious vegetation density differences such as saguaro-paloverde, creosote bush, and riparian vegetation can be separated on the Avra Valley imagery while more similar communities such as creosote bush and saltbush could not be differentiated. It is suggested that large differences in vegetation density are needed before the signatures of two different vegetation types can be differentiated on ERTS imagery. This is due to the relatively insignificant contribution of vegetation to the total radiometric signature of a given desert scene. Where more detailed information concerning the vegetation of arid regions is required, large scale imagery is appropriate.

Interactive analysis and evaluation of ERTS data for regional planning and urban development: A Los Angeles Basin case study

NASA Technical Reports Server (NTRS)

Raje, S.; Economy, R.; Willoughby, G.; Mcknight, J.

1974-01-01

The progression endemic to the ERTS Data Use Experiment SR 124 in data quality, analysis sophistication and applications responsiveness is reviewed. The roles of the variety of ERTS products, including the supporting underflight aircraft imagery at various scales, are discussed in the context of this investigation. The versatility of interpretation techniques and outputs developed and implemented via the General Electric Multispectral Information Extraction Systems is described and exemplified by both system-expository and applications-explanatory products. The wide-ranging and in-depth applications studied in the course of this experiment can be characterized as community-oriented and agency-directed. In the former, generic category, which is primarily data-contextual, problems analyzed dealt with agricultural systems, surface water bodies, snow cover, brush fire burns, forestry, grass growth, parks - golf courses - cemeteries, dust storms, grading sites, geological features and coastal water structure. The ERTS MSS band selectivity and measurements thresholds were of primary interest here. The agency-directed application areas have been user-evaluational in nature. Beginning with overall urbanized regional analysis of land cover density-development intensity, residential areas were analyzed for ascertaining if housing types could be aggregated with any degree of reliability.

A retrospective analysis of the potential impact of IgG antibodies to agalsidase beta on efficacy during enzyme replacement therapy for Fabry disease.

PubMed

Bénichou, Bernard; Goyal, Sunita; Sung, Crystal; Norfleet, Andrea M; O'Brien, Fanny

2009-01-01

Fabry disease results from a genetic deficiency of alpha-galactosidase A (alpha GAL) and the impaired catabolism of globotriasoylceramide (GL-3) and other glycosphingolipid substrates, which then accumulate pathogenically within most cells. Enzyme replacement therapy (ERT) with agalsidase beta (Fabrazyme), one of two available forms of recombinant human alpha GAL, involves regular intravenous infusions of the therapeutic protein. Immunoglobulin G (IgG) antibodies to recombinant alpha GAL develop in the majority of patients upon repeated infusion. To explore whether anti-alpha GAL IgG interferes with therapeutic efficacy, retrospective analyses were conducted using data obtained from a total of 134 adult male and female patients with Fabry disease who were treated with agalsidase beta at 1mg/kg every 2 weeks for up to 5 years during placebo-controlled trials and the corresponding open-label extension studies. The analyses did not reveal a correlation between anti-alpha GAL IgG titers and the onset of clinical events or the rate of change in estimated GFR during treatment, and no statistically significant association was found between anti-alpha GAL IgG titers and abnormal elevations in plasma GL-3 during treatment. However, a statistically significant association was found between anti-alpha GAL IgG titers and observation of some GL-3 deposition in the dermal capillary endothelial cells of skin during treatment, suggesting that GL-3 clearance may be partially impaired in some patients with high antibody titers. Determination of the long-term impact of circulating anti-alpha GAL IgG antibodies on clinical outcomes will require continued monitoring, and serology testing is recommended as part of the routine care of Fabry disease patients during ERT.

Application of ERT, Saline Tracer and Numerical Studies to Delineate Preferential Paths in Fractured Granites.

PubMed

Sreeparvathy, Vijay; Kambhammettu, B V N P; Peddinti, Srinivasa Rao; Sarada, P S L

2018-03-22

Accurate quantification of in situ heterogeneity and flow processes through fractured geologic media remains elusive for hydrogeologists due to the complexity in fracture characterization and its multiscale behavior. In this research, we demonstrated the efficacy of tracer-electrical resistivity tomography (ERT) experiments combined with numerical simulations to characterize heterogeneity and delineate preferential flow paths in a fractured granite aquifer. A series of natural gradient saline tracer experiments were conducted from a depth window of 18 to 22 m in an injection well (IW) located inside the Indian Institute of Technology Hyderabad campus. Tracer migration was monitored in a time-lapse mode using two cross-sectional surface ERT profiles placed in the direction of flow gradient. ERT data quality was improved by considering stacking, reciprocal measurements, resolution indicators, and geophysical logs. Dynamic changes in subsurface electrical properties inferred via resistivity anomalies were used to highlight preferential flow paths of the study area. Temporal changes in electrical resistivity and tracer concentration were monitored along the vertical in an observation well located at 48 m to the east of the IW. ERT-derived tracer breakthrough curves were in agreement with geochemical sample measurements. Fracture geometry and hydraulic properties derived from ERT and pumping tests were further used to evaluate two mathematical conceptualizations that are relevant to fractured aquifers. Results of numerical analysis conclude that dual continuum model that combines matrix and fracture systems through a flow exchange term has outperformed equivalent continuum model in reproducing tracer concentrations at the monitoring wells (evident by a decrease in RMSE from 199 to 65 mg/L). A sensitivity analysis on model simulations conclude that spatial variability in hydraulic conductivity, local-scale dispersion, and flow exchange at fracture-matrix interface have a profound effect on model simulations. © 2018, National Ground Water Association.

40 CFR 60.4915 - What reports must I submit?

Code of Federal Regulations, 2013 CFR

2013-07-01

... Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert_tool.html/) or other compatible... Sludge Incineration Units Recordkeeping and Reporting § 60.4915 What reports must I submit? You must... reporting period specified in paragraph (d)(3) of this section. You must submit your first annual compliance...

40 CFR 60.2235 - In what form can I submit my reports?

Code of Federal Regulations, 2012 CFR

2012-07-01

... opacity data, electronically to EPA's Central Data Exchange (CDX) by using the Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert tool.html/) or other compatible electronic spreadsheet... Recordkeeping and Reporting § 60.2235 In what form can I submit my reports? Submit initial, annual, and...

40 CFR 63.11225 - What are my notification, reporting, and recordkeeping requirements?

Code of Federal Regulations, 2012 CFR

2012-07-01

... Exchange (CDX) by using the Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert tool... 40 Protection of Environment 15 2012-07-01 2012-07-01 false What are my notification, reporting... What are my notification, reporting, and recordkeeping requirements? (a) You must submit the...

40 CFR 60.2235 - In what form can I submit my reports?

Code of Federal Regulations, 2011 CFR

2011-07-01

... opacity data, electronically to EPA's Central Data Exchange (CDX) by using the Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert tool.html/) or other compatible electronic spreadsheet... Recordkeeping and Reporting § 60.2235 In what form can I submit my reports? Submit initial, annual, and...

40 CFR 60.4915 - What reports must I submit?

Code of Federal Regulations, 2014 CFR

2014-07-01

... Electronic Reporting Tool (ERT) (see http://www.epa.gov/ttn/chief/ert/ert_tool.html/) or other compatible... Sludge Incineration Units Recordkeeping and Reporting § 60.4915 What reports must I submit? You must... reporting period specified in paragraph (d)(3) of this section. You must submit your first annual compliance...

Sketch on the structural geology and vulcanism in the Central High Plateau of the Bolivian Andes

NASA Technical Reports Server (NTRS)

Brockmann, C. E. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The Earth Resources Technology Satellite Program has as an objective the development of tectonic maps for Bolivia. Maps were prepared using the images of ERTS-1 in a preliminary study of alignments observed and rapidly interpreted in images 1010-14033-3-4-5-6-7 on a scale of 1:1,000,000, and later verified on the ground with corresponding fault zones. This information was not shown on existing geologial maps. The ERTS-1 imagery was used in volcanology research for drawing the regional limits of volcanic formations as soon as the alignment and the extent of the volcanoes could be determined. The extensive coverage of ERTS-1 images provides an excellent opportunity for developing studies of regional structures.

An ERTS multispectral scanner experiment for mapping iron compounds

NASA Technical Reports Server (NTRS)

Vincent, R. K. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. An experimental plan for enhancing spectral features related to the chemical composition of geological targets in ERTS multispectral scanner data is described. The experiment is designed to produce visible-reflective infrared ratio images from ERTS-1 data. Iron compounds are promising remote sensing targets because they display prominent spectral features in the visible-reflective infrared wavelength region and are geologically significant. The region selected for this ERTS experiment is the southern end of the Wind River Range in Wyoming. If this method proves successful it should prove useful for regional geologic mapping, mineralogical exploration, and soil mapping. It may also be helpful to ERTS users in scientific disciplines other than geology, especially to those concerned with targets composed of mixtures of live vegetation and soil or rock.

The use of ERTS-1 MSS data for mapping strip mines and acid mine drainage in Pennsyvania

NASA Technical Reports Server (NTRS)

Alexander, S. S.; Dein, J. L.; Gold, D. P.

1973-01-01

Digital processing of ERTS-I MSS data for areas around the west branch of the Susquehanna River permits identification of stripped areas including ones that are not discernible from visual analysis of ERTS imagery. Underflight data and ground-based observations are used for ground-truth and as a basis for designing more refined operators to make sub-classifications of stripped areas, particularly with regard to manifestations of acid mine drainage; because of associated diagnostic effects on vegetation, seasonal changes in classifiction criteria are being documented as repeated, cloud-free ERTS-I coverage of the same area becomes available. Preliminary results indicate that ERTS data can be used to moniter not only the total extent of stripping in given areas but also the effectiveness of reclamation and pollution abatement procedures.

Evaluate ERTS imagery for mapping and detection of changes of snowcover on land and on glaciers

NASA Technical Reports Server (NTRS)

Meier, M. F. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The percentage of snow cover area on specific drainage basins was measured from ERTS-1 imagery by video density slicing with a repeatability of 4 percent of the snow covered area. Data from ERTS-1 images of the melt season snow cover in the Thunder Creek drainage basin in the North Cascades were combined with existing hydrologic and meteorologic observations to enable calculations of the time distribution of the water stored in this mountain snowpack. Similar data could be used for frequent updating of expected inflow to reservoirs. Equivalent snowline altitudes were determined from area measurements. Snowline altitudes were also determined by combining enlarged ERTS-1 images with maps. ERTS-1 imagery was also successfully used to measure glacier accumulation area ratios for a small test basin.

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

PubMed

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 for Pompe disease.

Using electrical resistivity tomography to differentiate sapwood from heartwood: application to conifers.

PubMed

Guyot, Adrien; Ostergaard, Kasper T; Lenkopane, Mothei; Fan, Junliang; Lockington, David A

2013-02-01

Estimating sapwood area is one of the main sources of error when upscaling point scale sap flow measurements to whole-tree water use. In this study, the potential use of electrical resistivity tomography (ERT) to determine the sapwood-heartwood (SW-HW) boundary is investigated for Pinus elliottii Engelm var. elliottii × Pinus caribaea Morelet var. hondurensis growing in a subtropical climate. Specifically, this study investigates: (i) how electrical resistivity is correlated to either wood moisture content, or electrolyte concentration, or both, and (ii) how the SW-HW boundary is defined in terms of electrical resistivity. Tree cross-sections at breast height are analysed using ERT before being felled and the cross-section surface sampled for analysis of major electrolyte concentrations, wood moisture content and density. Electrical resistivity tomography results show patterns with high resistivities occurring in the inner part of the cross-section, with much lower values towards the outside. The high-resistivity areas were generally smaller than the low-resistivity areas. A comparison between ERT and actual SW area measured after felling shows a slope of the linear regression close to unity (=0.96) with a large spread of values (R(2) = 0.56) mostly due to uncertainties in ERT. Electrolyte concentrations along sampled radial transects (cardinal directions) generally showed no trend from the centre of the tree to the bark. Wood moisture content and density show comparable trends that could explain the resistivity patterns. While this study indicates the potential for application of ERT for estimating SW area, it shows that there remains a need for refinement in locating the SW-HW boundary (e.g., by improvement of the inversion method, or perhaps electrode density) in order to increase the robustness of the method.

Earth Resources Technology Satellite-1 (ERTS-1) data and anthropology: Use of these data in carrying capacity estimates for sites in Upper Volta and Niger

NASA Technical Reports Server (NTRS)

Reining, P. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Repetitively derived multispectral band imagery from ERTS-1 is now available for many parts of the earth's land surface and represents major new data sources for anthropological work in habitat, land use, and settlement patterns. A completed first step test of ERTS-1 data is available in carrying capacity estimates for Mossi, Hausa, and Sonrai sites derived from: (1) field work; (2) aerial photography; and (3) ERTS-1. Data can test more than one carrying capacity formula.

An interdisciplinary analysis of ERTS data for Colorado mountain environments using ADP techniques

NASA Technical Reports Server (NTRS)

Hoffer, R. M. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. Research efforts have been placed on: (1) location, acquisition, and preparation of baseline information necessary for the computer analysis, and (2) refinement of techniques for analysis of MSS data obtained from ERTS-1. Analysis of the first frame of data collected by the ERTS-1 multispectral scanner system over the Lake Texoma area has proven very valuable for determining the best procedures to follow in working with and analyzing ERTS data. Progress on the following projects is described: (1) cover type mapping; (2) geomorphology; and hydrologic feature surveys.

Correlation of coastal water turbidity and current circulation with ERTS-1 and Skylab imagery

NASA Technical Reports Server (NTRS)

Klemas, V.; Otley, M.; Philpot, W.; Wethe, C.; Rogers, R.; Shah, N.

1974-01-01

The article reviews investigations of current circulation patterns, suspended sediment concentration, coastal frontal systems, and waste disposal plumes based on visual interpretation and digital analysis of ERTS-1 and Skylab/EREP imagery. Data on conditions in the Delaware Bay area were obtained from 10 ERTS-1 passes and one Skylab pass, with simultaneous surface and airborne sensing. The current patterns and sediments observed by ERTS-1 correlated well with ground-based observations. Methods are suggested which would make it possible to identify certain pollutants and sediment types from multispectral scanner data.

Monitoring hillslope moisture dynamics with surface ERT for enhancing spatial significance of hydrometric point measurements

NASA Astrophysics Data System (ADS)

Hübner, R.; Heller, K.; Günther, T.; Kleber, A.

2015-01-01

Besides floodplains, hillslopes are basic units that mainly control water movement and flow pathways within catchments of subdued mountain ranges. The structure of their shallow subsurface affects water balance, e.g. infiltration, retention, and runoff. Nevertheless, there is still a gap in the knowledge of the hydrological dynamics on hillslopes, notably due to the lack of generalization and transferability. This study presents a robust multi-method framework of electrical resistivity tomography (ERT) in addition to hydrometric point measurements, transferring hydrometric data into higher spatial scales to obtain additional patterns of distribution and dynamics of soil moisture on a hillslope. A geoelectrical monitoring in a small catchment in the eastern Ore Mountains was carried out at weekly intervals from May to December 2008 to image seasonal moisture dynamics on the hillslope scale. To link water content and electrical resistivity, the parameters of Archie's law were determined using different core samples. To optimize inversion parameters and methods, the derived spatial and temporal water content distribution was compared to tensiometer data. The results from ERT measurements show a strong correlation with the hydrometric data. The response is congruent to the soil tension data. Water content calculated from the ERT profile shows similar variations as that of water content from soil moisture sensors. Consequently, soil moisture dynamics on the hillslope scale may be determined not only by expensive invasive punctual hydrometric measurements, but also by minimally invasive time-lapse ERT, provided that pedo-/petrophysical relationships are known. Since ERT integrates larger spatial scales, a combination with hydrometric point measurements improves the understanding of the ongoing hydrological processes and better suits identification of heterogeneities.

Improved characterisation of measurement errors in electrical resistivity tomography (ERT) surveys

NASA Astrophysics Data System (ADS)

Tso, C. H. M.; Binley, A. M.; Kuras, O.; Graham, J.

2016-12-01

Measurement errors can play a pivotal role in geophysical inversion. Most inverse models require users to prescribe a statistical model of data errors before inversion. Wrongly prescribed error levels can lead to over- or under-fitting of data, yet commonly used models of measurement error are relatively simplistic. With the heightening interests in uncertainty estimation across hydrogeophysics, better characterisation and treatment of measurement errors is needed to provide more reliable estimates of uncertainty. We have analysed two time-lapse electrical resistivity tomography (ERT) datasets; one contains 96 sets of direct and reciprocal data collected from a surface ERT line within a 24h timeframe, while the other is a year-long cross-borehole survey at a UK nuclear site with over 50,000 daily measurements. Our study included the characterisation of the spatial and temporal behaviour of measurement errors using autocorrelation and covariance analysis. We find that, in addition to well-known proportionality effects, ERT measurements can also be sensitive to the combination of electrodes used. This agrees with reported speculation in previous literature that ERT errors could be somewhat correlated. Based on these findings, we develop a new error model that allows grouping based on electrode number in additional to fitting a linear model to transfer resistance. The new model fits the observed measurement errors better and shows superior inversion and uncertainty estimates in synthetic examples. It is robust, because it groups errors together based on the number of the four electrodes used to make each measurement. The new model can be readily applied to the diagonal data weighting matrix commonly used in classical inversion methods, as well as to the data covariance matrix in the Bayesian inversion framework. We demonstrate its application using extensive ERT monitoring datasets from the two aforementioned sites.

Use of small scale electrical resistivity tomography to identify soil-root interactions during deficit irrigation

NASA Astrophysics Data System (ADS)

Vanella, D.; Cassiani, G.; Busato, L.; Boaga, J.; Barbagallo, S.; Binley, A.; Consoli, S.

2018-01-01

Plant roots activity affect the exchanges of mass and energy between the soil and atmosphere. However, it is challenging to monitor the activity of the root-zone because roots are not visible from the soil surface, and root systems undergo spatial and temporal variations in response to internal and external conditions. Therefore, measurements of the activity of root systems are interesting to ecohydrologists in general, and are especially important for specific applications, such as irrigation water management. This study demonstrates the use of small scale three-dimensional (3-D) electrical resistivity tomography (ERT) to monitor the root-zone of orange trees irrigated by two different regimes: (i) full rate, in which 100% of the crop evapotranspiration (ETc) is provided; and (ii) partial root-zone drying (PRD), in which 50% of ETc is supplied to alternate sides of the tree. We performed time-lapse 3-D ERT measurements on these trees from 5 June to 24 September 2015, and compared the long-term and short-term changes before, during, and after irrigation events. Given the small changes in soil temperature and pore water electrical conductivity, we interpreted changes of soil electrical resistivity from 3-D ERT data as proxies for changes in soil water content. The ERT results are consistent with measurements of transpiration flux and soil temperature. The changes in electrical resistivity obtained from ERT measurements in this case study indicate that root water uptake (RWU) processes occur at the 0.1 m scale, and highlight the impact of different irrigation schemes.

Monitoring of olive oil mills' wastes using electrical resistivity tomography techniques

NASA Astrophysics Data System (ADS)

Simyrdanis, Kleanthis; Papadopoulos, Nikos; Kirkou, Stella; Sarris, Apostolos; Tsourlos, Panagiotis

2014-08-01

Olive oil mills' wastes (OOMW) are one of the byproducts of the oil production that can lead to serious environmental pollution when they are deposited in ponds dug on the ground surface. Electrical Resistivity Tomography (ERT) method can provide a valuable tool in order to monitor through time the physical flow of the wastes into the subsurface. ERT could potentially locate the electrical signature due to lower resistivity values resulting from the leakage of OOMW to the subsurface. For this purpose, two vertical boreholes were installed (12m depth, 9 m apart) in the vicinity of an existing pond which is filled with OOMW during the oil production period. The test site is situated in Saint Andreas village about 15km south of the city of Rethymno (Crete, Greece). Surface ERT measurements were collected along multiple lines in order to reconstruct the subsurface resistivity models. Data acquisition was performed with standard and optimized electrode configuration protocols. The monitoring survey includes the ERT data collection for a period of time. The study was initiated before the OOMW were deposited in the pond, so resistivity fluctuations are expected due to the flow of OOMW in the porous subsurface media through time. Preliminary results show the good correlation of the ERT images with the drilled geological formations and the identification of low resistivity subsurface zone that could be attributed to the flow of the wastes within the porous layers.

Comparative evaluation of ERTS-A imagery for resource inventory in land-use planning

NASA Technical Reports Server (NTRS)

Simonson, G. H. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. The overall objectives of this program are: (1) use of multidiscipline team approach to determine features that can be successfully monitored by ERTS-1 imagery for resource inventory, planning, land use zoning, and resource development; and (2) using carefully selected sample areas, develop a comprehensive resource inventory mapping system for use in planning, zoning, and resource development. Progress has included compilation and organization of ground truth data and observations in the primary study area of Crook County; resource inventory legend development; assembly and testing of color enhancement equipment; development and adaption of programs for digital data processing; and quick-look evaluations of initial ERTS-1 imagery for Oregon.

Thailand national programme of the Earth Resources Technology Satellite

NASA Technical Reports Server (NTRS)

Cheosakul, P. (Principal Investigator); Sabhasri, S.

1974-01-01

The author has identified the following significant results. Intensive interpretation of ERTS-1 scenes covering agricultural regions near Bangkok, backed up by ground surveys and low altitude reconnaissance flights, has established that the major agricultural crops of Thailand can be positively differentiated, and in most cases identified, after some experience has been gained, by examination of ERTS-1 imagery. A country-wide survey of remaining forest cover will be completed during 1974. MSS band 5 and band 7 and white positive transparencies were found to be the most desirable medium for identification of geological structure. The Royal Irrigation Department conducted a pilot study to examine the possibility of determining water reservoir capacity from surface area measurements derived from ERTS-1 images.

Mapping of soil banks using ERTS-1 pictures

NASA Technical Reports Server (NTRS)

Ahmad, M. U.; Kantner, D. A.; Antalovich, J. W.

1973-01-01

Earth Resources Technology Satellite (ERTS-1) pictures of different wavelengths (MSS 4,5,6,7) were used in the study of two strip mine areas in southeastern Ohio. The first area was near Piedmont Lake and the second area was near New Lexington. Prints were examined under a binocular microscope and the gray tone was correlated with the actual ground conditions at several sites. For the New Lexington area, color infrared pictures taken at an elevation of 18,000 feet were also used for correlation with the ERTS-1 imagery. The results indicate that MSS 5 and 7 are most useful in defining the stripped land and show that the hydrological and soil characteristics are remarkably different than the surrounding lands.

Approach/avoidance motives, test emotions, and emotional regulation related to testing.

PubMed

Schutz, Paul A; Benson, Jeri; Decuir-Gunby, Jessica T

2008-07-01

This research stems from our program of work that focuses on understanding how students regulated their emotions related to testing. The primary goal for this study was to incorporate the approach/ avoidance motives into a model of emotional regulation related to testing. In addition, a secondary goal was to report on efforts at construct validation of the scores obtained during the refinement of the Emotional Regulation Related to Testing (ERT) Scale. Our results suggest that underlying beliefs, such as approach/avoid motives and the cognitive-appraisal process, of the ERT had both direct and indirect effects to both pleasant and unpleasant emotions related to testing. In addition, the ERT accounted for 56% of the variance in Pleasant and 87% of Unpleasant Test Emotions.

Cost analysis of cetuximab (Erbitux) plus radiotherapy (ERT) versus concomitant cisplatin plus radiotherapy (CRT) within an NHS oncology unit (single institution): a pilot study.

PubMed

Caroline, Brammer; Sundus, Yahya; Dawn, Dawson; Carol, Glaister; Susan, Merrick

2016-12-01

The aim of this feasibility study is to define the resource effectiveness of cetuximab vs cisplatin given concomitantly with radiotherapy for squamous cell carcinoma within a National Health Service clinical oncology unit. 20 patients with Stage 3 or 4 head and neck squamous cell cancers were randomized to receive either cetuximab with radiotherapy (ERT) or cisplatin with radiotherapy concurrent with external beam radiotherapy 70 Gy in 35 fractions on a 1 : 1 basis over a 12-month duration. The study compared the resource utilization of ERT vs cisplatin with radiotherapy taking into account drug costs, clinical management and the costs of managing treatment-related toxicity from first fraction of radiotherapy to 6 months after the completion of therapy. Outcome measures were quality of life (recorded at the entry, end of radiotherapy, 6 weeks post treatment and 6 months post treatment), admissions to hospital, delays to radiotherapy, locoregional control and survival. Total drug costs including cost of nutritional supplements for patients treated with cetuximab were £7407.45 compared with £3959.07 for patients treated with cisplatin. Unscheduled admissions for toxicity management were significantly more common in the ERT arm. Healthcare personnel spent significantly more time delivering unscheduled outpatient care for patients receiving cisplatin than for those receiving cetuximab (p = 0.01). No significant difference in the quality of life was suggested at baseline, 6 weeks and 6 months. The mean time to removal of percutaneous gastrostomy (PEG) after completion of radiotherapy was 49.7 weeks in the cisplatin arm and 18.5 weeks in the cetuximab arm (p = 0.04). There was a statistically significant difference in patient-reported use of PEG between the cisplatin and cetuximab arms at 6 months following completion of treatment (p = 0.04). At 21 months, overall survival was 80% in the cisplatin arm vs 50% in the cetuximab (p = 0.332), with disease-free survival being 80% in the cisplatin arm vs 40% in the cetuximab (p = 0.097). Cetuximab is still more expensive in simple drug cost terms than cisplatin when delivered with radiotherapy taking into account costs of drugs for toxicity management and nutritional supplements but other resource implications such as inpatient admission, time spent delivering unscheduled care and cost of additional investigations to manage toxicity for patients treated with cisplatin significantly reduce differential. The study suggested significant differences in patient-reported PEG use at 6 months and in time to PEG removal in favour of the cetuximab arm. Advances in knowledge: There is paucity of randomized data on cost analysis for cisplatin vs cetuximab radiotherapy; this trial informs on the cost analysis between the two approaches.

Air quality indices from ERTS-1 MSS information

NASA Technical Reports Server (NTRS)

Riley, E. L.; Stryker, S.; Ward, E. A.

1973-01-01

Comparison between ground based atmospheric turbidity network measurements and the average scene grayness from MSS Channel 4 data is in progress. Correlation between these two sources is promising. If continued correlation occurs for other ERTS-1 overflight dates and ground test sites, a new operational use of ERTS-1 useful to Federal, state, and international organizations will become available.

Geologic and mineral and water resources investigations in western Colorado using ERTS-1 data

NASA Technical Reports Server (NTRS)

Knepper, D. H. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Most of the geologic information in ERTS-1 imagery can be extracted from bulk processed black and white transparencies by a skilled interpreter using standard photogeologic techniques. In central and western Colorado, the detectability of lithologic contacts on ERTS-1 imagery is closely related to the time of year the imagery was acquired. Geologic structures are the most readily extractable type of geologic information contained in ERTS images. Major tectonic features and associated minor structures can be rapidly mapped, allowing the geologic setting of a large region to be quickly accessed. Trends of geologic structures in younger sedimentary appear to strongly parallel linear trends in older metamorphic and igneous basement terrain. Linears and color anomalies mapped from ERTS imagery are closely related to loci of known mineralization in the Colorado mineral belt.

Interdisciplinary applications and interpretations of ERTS data within the Susquehanna River Basin; resources inventory, land use and pollution

NASA Technical Reports Server (NTRS)

Mcmurtry, G. J. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Identification and mapping of three major kinds of coal refuse targets based on spectral signatures in channels four through seven of the ERTS-1 MSS were conducted. Correlation of the placement of the coal refuse targets with an existing map of their location was accomplished. Digital processing of ERTS-1 data permitted identification of stripped areas including ones that are not discernible by visual analysis of ERTS imagery. Combined visual and digital techniques of analyzing ERTS-1 data for geologic formations have been tried on selected areas of Pennsylvania. Mapping of two major agriculture counties to show land forms, drainage patterns, water, and urban areas were made using positive transparencies of MSS data. Two frames of the same central Pennsylvania area were brought into registration by translation and then merged even though the frames were obtained 71 days apart.

Change in land use in the Phoenix (1:250,000) Quadrangle, Arizona between 1970 and 1973: ERTS as an aid in a nationwide program for mapping general land use. [Phoenix Quadrangle, Arizona

NASA Technical Reports Server (NTRS)

Place, J. L.

1974-01-01

Changes in land use between 1970 and 1973 in the Phoenix (1:250,000 scale) Quadrangle in Arizona have been mapped using only the images from ERTS-1, tending to verify the utility of a standard land use classification system proposed for use with ERTS images. Types of changes detected have been: (1) new residential development of former cropland and rangeland; (2) new cropland from the desert; and (3) new reservoir fill-up. The seasonal changing of vegetation patterns in ERTS has complemented air photos in delimiting the boundaries of some land use types. ERTS images, in combination with other sources of information, can assist in mapping the generalized land use of the fifty states by the standard 1:250,000 quadrangles. Several states are already working cooperatively in this type of mapping.

Correlation and registration of ERTS multispectral imagery. [by a digital processing technique

NASA Technical Reports Server (NTRS)

Bonrud, L. O.; Henrikson, P. J.

1974-01-01

Examples of automatic digital processing demonstrate the feasibility of registering one ERTS multispectral scanner (MSS) image with another obtained on a subsequent orbit, and automatic matching, correlation, and registration of MSS imagery with aerial photography (multisensor correlation) is demonstrated. Excellent correlation was obtained with patch sizes exceeding 16 pixels square. Qualities which lead to effective control point selection are distinctive features, good contrast, and constant feature characteristics. Results of the study indicate that more than 300 degrees of freedom are required to register two standard ERTS-1 MSS frames covering 100 by 100 nautical miles to an accuracy of 0.6 pixel mean radial displacement error. An automatic strip processing technique demonstrates 600 to 1200 degrees of freedom over a quater frame of ERTS imagery. Registration accuracies in the range of 0.3 pixel to 0.5 pixel mean radial error were confirmed by independent error analysis. Accuracies in the range of 0.5 pixel to 1.4 pixel mean radial error were demonstrated by semi-automatic registration over small geographic areas.

Effects of copper oxide nanomaterials (CuONMs) are life stage dependent - full life cycle in Enchytraeus crypticus.

PubMed

Bicho, Rita C; Santos, Fátima C F; Scott-Fordsmand, Janeck J; Amorim, Mónica J B

2017-05-01

Copper oxide nanomaterials (CuONMs) have various applications in industry and enter the terrestrial environment, e.g. via sewage sludge. The effects of CuONMs and copper chloride (CuCl 2 ) were studied comparing the standard enchytraeid reproduction test (ERT) and the full life cycle test (FLCt) with Enchytraeus crypticus. CuONMs mainly affected growth or juveniles' development, whereas CuCl 2 mainly affected embryo development and/or hatching success and adults survival. Compared to the ERT, the FLCt allowed discrimination of effects between life stages and provided indication of the underlying mechanisms; further, the FLCt showed increased sensitivity, e.g. reproductive effects for CuONMs: EC 10  = 8 mg Cu/kg and EC 10  = 421 mg Cu/kg for the FLCt and the ERT respectively. The performance of the FLCt is preferred to the ERT and we recommend it as a good alternative to assess hazard of NMs. Effects of CuONMs and CuCl 2 are life stage dependent and are different between Cu forms. Copyright © 2017 Elsevier Ltd. All rights reserved.

Survey of the seasonal snow cover in Alaska

NASA Technical Reports Server (NTRS)

Weller, G. E. (Principal Investigator)

1973-01-01

The author has identified the following significant results. ERTS-1 data are used together with synoptic-climatological data to describe the buildup of the seasonal snow and ice covers in a north-south transect of a total length of about 1250 km across Alaska. It has been demonstrated that the ERTS-1 data may, under favorable conditions, be used for accurate mapping of snow lines in high mountain regions. The analysis shows that especially in the Brooks Range and on the Arctic Slope where snow covers generally are relatively thin, the ERTS-1 scenes can be useful for qualitative descriptions of the snow and ice covers over wide expanses. The onset and retreat of the seasonal snow cover are sensitive indicators of climatic fluctuations and the ERTS-1 data offers a possibility to record variations of the snow and ice buildup from year to year in a practical and informative way, which should be especially useful for studies of climatic trends. This is particularly true in Alaska where the density of the station network is too low to permit interpolations between the stations.

Multidisciplinary study (CO2 flux, ERT, self-potential, permeability and structural surveys) in Fondi di Baia, Astroni and Agnano volcanoes: insights for the structural architecture of the Campi Flegrei caldera (southern Italy)

NASA Astrophysics Data System (ADS)

Isaia, Roberto; Carapezza, Maria Luisa; Conti, Eric; Giulia Di Giuseppe, Maria; Lucchetti, Carlo; Prinzi, Ernesto; Ranaldi, Massimo; Tarchini, Luca; Tramparulo, Francesco; Troiano, Antonio; Vitale, Stefano; Cascella, Enrico; Castello, Nicola; Cicatiello, Alessandro; Maiolino, Marco; Puzio, Domenico; Tazza, Lucia; Villani, Roberto

2017-04-01

Recent volcanism at Campi Flegrei caldera produced more than 70 eruptions in the last 15 ka formed different volcanic edifices. The vent distribution was related to the main volcano-tectonic structure active in the caldera along which also concentrated part of the present hydrothermal and fumarolic activity, such as in the Solfatara area. In order to define the role of major faults in the Campi Flegrei Caldera, we analyzed some volcanic craters (Fondi di Baia and Astroni) and the Agnano caldera, by means of different geochemical and geophysical technics including CO2 flux, electrical resistivity (ERT), self-potential and permeability surveys. We provided some ERT profiles and different maps of geochemical and geophysical features. Major fault planes were identified comparing ERT imaging with alignments of anomalies in maps. The results can improve the knowledge on the present state of these volcanoes actually not fully monitored though included in the area with high probability of future vent opening within the Campi Flegrei caldera.

NASA to Survey Earth's Resources

NASA Technical Reports Server (NTRS)

Mittauer, R. T.

1971-01-01

A wide variety of the natural resources of earth and man's management of them will be studied by an initial group of foreign and domestic scientists tentatively chosen by the National Aeronautics and Space Administration to analyze data to be gathered by two earth-orbiting spacecraft. The spacecraft are the first Earth Resources Technology Satellite (ERTS-A) and the manned Skylab which will carry an Earth Resources Experiment Package (EREP). In the United States, the initial experiments will study the feasibility of remote sensing from a satellite in gathering information on ecological problems. The objective of both ERTS and EREP aboard Skylab is to obtain multispectral images of the surface of the earth with high resolution remote sensors and to process and distribute the images to scientific users in a wide variety of disciplines. The ERTS-A, EREP, and Skylab systems are described and their operation is discussed.

Utilizing ERTS imagery to detect plant diseases and nutrient deficiencies, soil types and soil moisture levels

NASA Technical Reports Server (NTRS)

Parks, W. L. (Principal Investigator); Sewell, J. I.; Hilty, J. W.; Rennie, J. C.

1973-01-01

The author has identified the following significant results. The delineation of soil associations and detection of drainage patterns, erosion and sedimentation through the use of ERTS-1 imagery are shown. Corn blight and corn virus could not be detected from ERTS-1 and detection of forest composition was at a very low probability.

ERTS-1 applications to California resource inventory

NASA Technical Reports Server (NTRS)

Colwell, R. N. (Principal Investigator)

1972-01-01

There are no author-identified significant results in this report. ERTS-1 information will be utilized by resource management groups working in the fields of forestry, hydrology, range management, and agriculture to develop resource inventories of the state of California. Five examples are given of the use of ERTS-1 imagery and aerial photography in identifying different crops and field conditions.

Radiant Power Measuring Instrument (RPMI)

NASA Technical Reports Server (NTRS)

Rogers, R. H. (Principal Investigator)

1973-01-01

There are no author-identified significant results in this report. The radiant power measuring instrument is a rugged, hand-carried instrument which provides an ERTS investigator with a capability of obtaining radiometric measurements needed to determine solar and atmospheric parameters that affect the ERTS radiance measurements. With these parameters, ERTS data can be transformed into absolute target reflectance signatures, making accurate unambiguous interpretations possible.

Geographic applications of ERTS-1 imagery to rural landscape change in eastern Tennessee

NASA Technical Reports Server (NTRS)

Rehder, J. B. (Principal Investigator); Omalley, J. R.

1973-01-01

There are no author-identified significant results in this report. A multistage sampling experiment was conducted using low (10,000') and high (60,000') altitude aircraft imagery in comparison with orbital (560 miles) ERTS imagery. Although the aircraft data provide detailed landscape observations similar to ground truth data, they cover relatively small areas per image frame for irregular static slices of time. By comparison, ERTS provides repetitive observations in a regional perspective for broad areal coverage. Microdensitometric and computer techniques are being used to analyze the ERTS imagery for gray tone signatures, comparisons, and ultimately for landscape change detection.

ERTS-1 Virgin Islands experiment 589: Determine boundaries of ERTS and aircraft data within which useful water quality information can be obtained. [water pollution in St. Thomas harbor, Virgin Islands

NASA Technical Reports Server (NTRS)

Coulbourn, W. C.; Egan, W. G.; Olsen, D. A. (Principal Investigator); Heaslip, G. B.

1973-01-01

The author has identified the following significant results. The boundaries of application of ERTS-1 and aircraft data are established for St. Thomas harbor within which useful water quality information can be obtained. In situ physical, chemical, and biological water quality and benthic data were collected. Moored current meters were employed. Optical measurements of solar irradiance, color test panel radiance and water absorption were taken. Procedures for correlating in situ optical, biological, and chemical data with underflight aircraft I2S data and ERTS-1 MSS scanner data are presented. Comparison of bulk and precision CCT computer printout data for this application is made, and a simple method for geometrically locating bulk data individual pixels based on land-water interface is described. ERTS spacecraft data and I2S aircraft imagery are correlated with optical in situ measurements of the harbor water, with the aircraft green photographic and ERTS-1 MSS-4 bands being the most useful. The biological pigments correlate inversely with the optical data for inshore areas and directly further seaward. Automated computer data processing facilitated analysis.

Land use mapping and modelling for the Phoenix Quadrangle

NASA Technical Reports Server (NTRS)

Place, J. L. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The land use of the Phoenix Quadrangle in Arizona had been mapped previously from aerial photographs and recorded in a computer data bank. During the ERTS experiment, changes in land use were detected, first with the ERTS-simulation photographs, then with the ERTS-1 images when they became available. In each case, the I2S color additive viewer was used as the primary image enhancement tool, operated in a multispectral mode. A search was made for a method of creating hard copy color composite images of the best combinations of multiband composites from ERTS-1, mostly by photographic and diazo processes. The I2S viewer was also used to enhance changes between successive images by quick flip techniques or by registering with different color filters. Improved interpretation of land use change resulted, and a map of changes in the Phoenix Quadrangle was compiled using magnified ERTS-1 images alone. The first level of a standard land use classification system was successfully used. Between the ERTS-1 images for August and November, some differences were detected that could be caused by seasonal characteristics of vegetation or by change in use.

Monitoring coastal water properties and circulation from ERTS-1. [Delaware Bay

NASA Technical Reports Server (NTRS)

Klemas, V. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Imagery and digital tapes from nine successful ERTS-1 passes over Delaware Bay during different portions of the tidal cycle have been analyzed with special emphasis on turbidity, current circulation, waste disposal plumes, and convergent boundaries between different water masses. ERTS-1 image radiance correlated well with Secchi depth and suspended sediment concentration. MSS band 5 seemed to give the best representation of sediment load in the upper one meter of the water column. Circulation patterns observed by ERTS-1 during different parts of the tidal cycle, agreed well with predicted and measured currents throughout Delaware Bay. During flood tide the suspended sediment as visible from ERTS-1 also correlated well with the depth profile. Convergent shear boundaries between different water masses were observed from ERTS-1, with foam lines containing high concentrations of lead, mercury, and other toxic substances. Several fronts have been seen. Those near the mouth of the bay are associated with the tidal intrusion of shelf water. Fronts in the interior of the bay on the Delaware side appear to be associated with velocity shears induced by differences in bottom topography. Waste disposal plumes have benn detected 36 miles offshore.

Land use mapping and modelling for the Phoenix Quadrangle

NASA Technical Reports Server (NTRS)

Place, J. L. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The land use of the Phoenix Quadrangle in Arizona had been mapped previously from aerial photographs and recorded in a computer data bank. During the ERTS-1 experiment, changes in land use were detected using only the ERTS-1 images. The I2S color additive viewer was used as the principal image enhancement tool, operated in a multispectral mode. Hard copy color composite images of the best multiband combinations from ERTS-1 were made by photographic and diazo processes. The I2S viewer was also used to enhance changes between successive images by quick flip techniques or by registering with different color filters. More recently, a Bausch and Lomb zoom transferscope has been used for the same purpose. Improved interpretation of land use change resulted, and a map of changes within the Phoenix Quadrangle was compiled. The first level of a proposed standard land use classification system was sucessfully used. ERTS-1 underflight photography was used to check the accuracy of the ERTS-1 image interpretation. It was found that the total areas of change detected in the photos were comparable with the total areas of change detected in the ERTS-1 images.

Expanding our understanding of lower urinary tract symptoms and incontinence in adults with pompe disease.

PubMed

McNamara, Erin R; Austin, Stephanie; Case, Laura; Wiener, John S; Peterson, Andrew C; Kishnani, Priya S

2015-01-01

To study the prevalence of lower urinary tract symptoms (LUTS) and incontinence in late-onset Pompe disease (LOPD) Methods: Adult LOPD patients seen at the Duke Pompe Clinic were prospectively recruited and asked to complete validated questionnaires on LUTS and incontinence as part of an IRB-approved study. Patient demographics as well as previous urologic history were reviewed. 35 patients with LOPD were included in the study (17 males and 18 females). The median age was 51.8 (range 18-72 years of age). Of these patients, 27/35 were receiving enzyme replacement therapy (ERT) with median duration of 54 months (range 5-88 months). In the male patients, 9/17 (53%) described their stream as dribbling, weak, or intermittent, and 9/17 (53%) complained of post-void dribbling. In addition 38% of the men were unable to stop their urination midstream. In the female patients, the most common complaint was urinary incontinence, reported in 14/18 (78%). In addition, 7/18 (39%) complained of post-void dribbling, and 47% were unable to stop their urination midstream. Bowel incontinence was reported in 45% of patients. There was a significant association between urinary symptoms and lower extremity function scores and duration of ERT (p = 0.005 and p = 0.04, respectively) This is the first study in a large cohort of LOPD patients that demonstrates LUTS and incontinence occur at a high rate. This study emphasizes the spectrum of LOPD is beyond isolated gross motor and pulmonary involvement and has a significant effect on the lower urinary tract.

Stereo study as an aid to visual analysis of ERTS and Skylab images

NASA Technical Reports Server (NTRS)

Vangenderen, J. L. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The parallax on ERTS and Skylab images is sufficiently large for exploitation by human photointerpreters. The ability to view the imagery stereoscopically reduces the signal-to-noise ratio. Stereoscopic examination of orbital data can contribute to studies of spatial, spectral, and temporal variations on the imagery. The combination of true stereo parallax, plus shadow parallax offer many possibilities to human interpreters for making meaningful analyses of orbital imagery.

Study of application of ERTS-A imagery to fracture related mine safety hazards in the coal mining industry

NASA Technical Reports Server (NTRS)

Wier, C. E.; Wobber, F. J. (Principal Investigator); Russell, O. R.; Amato, R. V.

1973-01-01

The author has identified the following significant results. The utility of ERTS-1/high altitude aircraft imagery to detect underground mine hazards is strongly suggested. A 1:250,000 scale mined lands map of the Vincennes Quadrangle, Indiana has been prepared. This map is a prototype for a national mined lands inventory and will be distributed to State and Federal offices.

Geoelectrical mapping of the Soil and Groundwater Contaminated Site: Case Study from Taiwan

NASA Astrophysics Data System (ADS)

Liu, H. C.; Lin, C. P.; Wang, T. P.

2016-12-01

In recent years, geophysical technology has been widely used in soil and groundwater investigation and remediation of contaminated sites assessments in Taiwan, such technology can securely work in either small or large sampler areas, and collect data from the traditional one-dimensional data to two-dimensional and three-dimensional data. In other words, geophysical technology helps provide more information to assist the data interpretation, and improves the overall effectiveness of soil and groundwater contamination surveys. Electrical Resistivity Tomography (ERT) is one of useful geophysical technology to the soil and groundwater contaminated sites. By estimating the groundwater flow direction and distribution of contaminations, we could establish monitoring or sampling wells in potential pollution areas. ERT survey could delineate the contaminated areas with high concentrations in relatively simple sites. Even in the seriously DNAPL leakage cases, it is possible to directly detect the DNAPL pool. In this study, we presented the investigation outcomes of electrical resistivity tomography (ERT) and ground-penetrating radar (GPR) at the DNAPLs-impacted site. Evaluation of ERT/GPR technique deployment in detecting buried DNAPLs and assessment of remediation efforts are also discussed. Results indicated zones with anomalously high resistivity to be associated with contaminated DNAPLs presence. Resistivity maps clearly outlined the subsurface distribution and the possible migration path of DNAPLs.

Waste disposal mapping with electrical resistivity tomography case: Leuwigajah landfill

NASA Astrophysics Data System (ADS)

Aryanti, Erisha; Ardi, Ahmad Puji; Almunziri, Muaz; Xanggam, Zael Yahd; Eleazar, Adino; Widodo

2017-07-01

Leuwigajah landfill as administrative is located between district of Bandung and Cimahi citythat has an environmental and social problem that caused aquifer contamination due to the big amount of waste from Bandung city, Cimahi and Bandung regency. It is occupied in abandoned andesite mine site with an area of about 25 hectare. The aim of this research is to map the geology structure and to study the leachate towards aquifer layer below Leuwigajah landfill. Here, we present the study of Leuwigajah landfill subsurface using Electrical Resistivity Tomography (ERT). ERT is one of the most promising prospecting techniques mainly concerning its effective contribution to resolve several environmental problems, was applied for the geophysical modeling. ERT is a robust imaging method the theory and implementation of which are well documented in geophysical research literature. The geological setting comprises clayed weathered layer, fractured andesitic dike. Due to the above-mentioned geological singularity and in the light of the requirement for an environmentally safe construction of the landfill, an ERT survey was carried out with dipole-dipole array, 78 m of acquisition line and 6 m of electrode spacing. The model consists of 4 layers below the Leuwigajah landfill and andesitic fracture until depth of 18.7 m below the surface.

Remote sensing applied to land-use studies in Wyoming

NASA Technical Reports Server (NTRS)

Breckenridge, R. M.; Marrs, R. W.; Murphy, D. J.

1973-01-01

Impending development of Wyoming's vast fuel resources requires a quick and efficient method of land use inventory and evaluation. Preliminary evaluations of ERTS-1 imagery have shown that physiographic and land use inventory maps can be compiled by using a combination of visual and automated interpretation techniques. Test studies in the Powder River Basin showed that ERTS image interpretations can provide much of the needed physiographic and land use information. Water impoundments as small as one acre were detected and water bodies larger than five acres could be mapped and their acreage estimated. Flood plains and irrigated lands were successfully mapped, and some individual crops were identified and mapped. Coniferous and deciduous trees were mapped separately using color additive analysis on the ERTS multispectral imagery. Gross soil distinctions were made with the ERTS imagery, and were found to be closely related to the bedrock geology. Several broad unstable areas were identified. These were related to specific geologic and slope conditions and generally extended through large regions. Some new oil fields and all large open-cut coal mines were mapped. The most difficult task accomplished was that of mapping urban areas. Work in the urban areas provides a striking example of snow enhancement and the detail available from a snow enhanced image.

First ERTS-1 results in southeastern France: Geology, sedimentology, pollution at sea

NASA Technical Reports Server (NTRS)

Fontanel, A.; Guillemot, J.; Guy, M.

1973-01-01

Results obtained by four ERTS projects in southeastern France are summarized. With regard to geology, ERTS photos of Western Alps are very useful for tectonic interpretation because large features are clearly visible on these photographs even though they are often hidden by small complicated structures if studied on large scale documents. The 18-day repetition coverage was not obtained, and time-varying sedimentological surveys were impossible. Nevertheless, it was possible to delineate the variations of the shorelines in the Rhone Delta for a period covering the least 8,000 years. Some instances of industries discharging pollutant products at sea were detected, as well as very large anomalies of unknown origin. Some examples of coherent optical processing have been made in order to bring out tectonic features in the Alps mountains.

Quality and use of ERTS radiometric information in geologic applications

NASA Technical Reports Server (NTRS)

Goetz, A. F. H.; Billingsley, F. C.

1974-01-01

Some techniques are described for making full use of the data contained in an ERTS MSS image. Only about one-fourth of the data in a single band can be displayed at one time on a black and white image; therefore, when all four bands are considered, only about 7% of the available data can be used by the interpreter. Selecting the proper subset of information for the photointerpreter is therefore a necessity. Ratio methods exclude the brightness information from the display. A field study in one area using a portable spectrometer has shown only fair correlation with ERTS radiometry after one normalization procedure. Plots of brightness of test areas with sun angle show discrepancies. Plots of ratios show discrepancies of lesser magnitude, although the error limits are large.

Satellite studies of turbidity and circulation patterns in Delaware Bay

NASA Technical Reports Server (NTRS)

Klemas, V.; Srna, R.; Treasure, W. M.; Rogers, R.

1973-01-01

Satellite imagery from four successful ERTS-1 passes over Delaware Bay during different portions of the tidal cycle are interpreted with special emphasis on visibility of suspended sediment and its use as a natural tracer for gross circulation patters. The MSS red band (band 5) appears to give the best contrast, although the sediment patterns are represented by only a few neighboring shades of grey. Color density slicing improves the differentiation of turbidity levels. However, color additive enhancements are of limited value since most of the information is in a single color band. The ability of ERTS-1 to present a synoptic view of the surface circulation over the entire bay is shown to be a valuable and unique contribution of ERTS-1 to coastal oceanography.

Application of ERTS-1 imagery to fracture related mine safety hazards in the coal mining industry. [Indiana

NASA Technical Reports Server (NTRS)

Wier, C. E.; Wobber, F. J. (Principal Investigator); Russell, O. R.; Amato, R. V.; Leshendok, T. V.

1974-01-01

The author has identified the following significant results. New fracture detail of Indiana has been observed and mapped from ERTS-1 imagery. Studies so far indicate a close relationship between the directions of fracture traces mapped from the imagery, fractures measured on bedrock outcrops, and fractures measured in the underground mines. First hand observations and discussions with underground mine operators indicate good correlation of mine hazard maps prepared from ERTS-1/aircraft imagery and actual roof falls. The inventory of refuse piles/slurry ponds of the coal field of Indiana has identified over 225 such sites from past mining operations. These data will serve the State Legislature in making tax decisions on coal mining which take on increased importance because of the energy crisis.

Combining geomorphological mapping and near surface geophysics (GPR and ERT) to study piping systems

NASA Astrophysics Data System (ADS)

Bernatek-Jakiel, Anita; Kondracka, Marta

2016-12-01

This paper aims to provide a more comprehensive characterization of piping systems in mountainous areas under a temperate climate using geomorphological mapping and geophysical methods (electrical resistivity tomography - ERT and ground penetrating radar - GPR). The significance of piping in gully formation and hillslope hydrology has been discussed for many years, and most of the studies are based on surface investigations. However, it seems that most surface investigations underestimate this subsurface process. Therefore, our purpose was to estimate the scale of piping activity based on both surface and subsurface investigations. We used geophysical methods to detect the boundary of lateral water movement fostering pipe development and recognize the internal structure of the underlying materials. The survey was carried out in the Bereźnica Wyżna catchment, in the Bieszczady Mountains. (Eastern Carpathians, Poland), where pipes develop in Cambisols at a mean depth of about 0.7-0.8 m. The geophysical techniques that were used are shown to be successful in identifying pipes. GPR data suggest that the density of piping systems is much larger than that detectible from surface observations alone. Pipe length can be > 6.5-9.2% (maximum = 49%) higher than what surface mapping suggests. Thus, the significance of piping in hillslope hydrology and gully formation can be greater than previously assumed. These results also draw attention to the scale of piping activity in the Carpathians, where this process has been neglected for many years. The ERT profiles reveal areas affected by piping as places of higher resistivity values, which are an effect of a higher content of air-filled pores (due to higher soil porosity, intense biological activity, and well-developed soil structure). In addition, the ERT profiles show that the pipes in the study area develop at the soil-bedrock interface, probably above the layers of shales or mudstones which create a water restrictive layer. Our results illustrate the suitability and limitations of GPR and ERT to study soil piping. In general, geophysical surveying is useful for gathering more information on pipe density, potential pipe detection, and recognition of the internal structure of materials underlying the pipes. However, the interpretation of radargrams and ERT profiles should be always accompanied by detailed terrain mapping due to potential disturbances affecting geophysical profiles.

To assess the value of satellite photographs in the resource evaluation on a national scale

NASA Technical Reports Server (NTRS)

Hepworth, J. V. (Principal Investigator); Akehurst, S. M.

1973-01-01

The author has identified the following significant results. Some observations have been made on ERTS-1 color imagery and comparison of imagery. Results of geophysical work are correlated with ERTS-1 imagery and new lineaments are postuated in the northern Kalahari Desert. ERTS-1 imagery reveals complex structural trends in the Basement Complex in the Selebi-Pikwe area.

Electrical Resistivity Tomography monitoring reveals groundwater storage in a karst vadose zone

NASA Astrophysics Data System (ADS)

Watlet, A.; Kaufmann, O.; Van Camp, M. J.; Triantafyllou, A.; Cisse, M. F.; Quinif, Y.; Meldrum, P.; Wilkinson, P. B.; Chambers, J. E.

2016-12-01

Karst systems are among the most difficult aquifers to characterize, due to their high heterogeneity. In particular, temporary groundwater storage that occurs in the unsaturated zone and the discharge to deeper layers are difficult processes to identify and estimate with in-situ measurements. Electrical Resistivity Tomography (ERT) monitoring is meant to track changes in the electrical properties of the subsurface and has proved to be applicable to evidence and quantify hydrological processes in several types of environments. Applied to karst systems, it has particularly highlighted the challenges in linking electrical resistivity changes to groundwater content with usual approaches of petrophysical relationships, given the high heterogeneity of the subsurface. However, taking up the challenge, we undertook an ERT monitoring at the Rochefort Cave Laboratory (Belgium) lasting from Spring 2014 to Winter 2016. This includes 3 main periods of several months with daily measurements, from which seasonal groundwater content changes in the first meters of the vadose zone were successfully imaged. The monitoring concentrates on a 48 electrodes profile that goes from a limestone plateau to the bottom of a sinkhole. 3D UAV photoscans of the surveyed sinkhole and of the main chamber of the nearby cave were performed. Combined with lithological observations from a borehole drilled next to the ERT profile, the 3D information made it possible to project karstified layers visible in the cave to the surface and assess their potential locations along the ERT profile. Overall, this helped determining more realistic local petrophysical properties in the surveyed area, and improving the ERT data inversion by adding structural constraints. Given a strong air temperature gradient in the sinkhole, we also developed a new approach of temperature correction of the raw ERT data. This goes through the solving (using pyGIMLI package) of the 2D ground temperature field and its temporal evolution, calibrated with data from in-situ temperature probes installed along the ERT profile. Results from a 3D ERT monitoring of a sprinkling experiment, those of a gravimetric monitoring and an in-cave flow discharges monitoring were also of interest to verify interpretations of the permanent ERT monitoring in terms of groundwater content changes.

Why 1D electrical resistivity techniques can result in inaccurate siting of boreholes in hard rock aquifers and why electrical resistivity tomography must be preferred: the example of Benin, West Africa

NASA Astrophysics Data System (ADS)

Alle, Iboukoun Christian; Descloitres, Marc; Vouillamoz, Jean-Michel; Yalo, Nicaise; Lawson, Fabrice Messan Amen; Adihou, Akonfa Consolas

2018-03-01

Hard rock aquifers are of particular importance for supplying people with drinking water in Africa and in the world. Although the common use of one-dimensional (1D) electrical resistivity techniques to locate drilling site, the failure rate of boreholes is usually high. For instance, about 40% of boreholes drilled in hard rock aquifers in Benin are unsuccessful. This study investigates why the current use of 1D techniques (e.g. electrical profiling and electrical sounding) can result in inaccurate siting of boreholes, and checks the interest and the limitations of the use of two-dimensional (2D) Electrical Resistivity Tomography (ERT). Geophysical numerical modeling and comprehensive 1D and 2D resistivity surveys were carried out in hard rock aquifers in Benin. The experiments carried out at 7 sites located in different hard rock groups confirmed the results of the numerical modeling: the current use of 1D techniques can frequently leads to inaccurate siting, and ERT better reveals hydrogeological targets such as thick weathered zone (e.g. stratiform fractured layer and preferential weathering associated with subvertical fractured zone). Moreover, a cost analysis demonstrates that the use of ERT can save money at the scale of a drilling programme if ERT improves the success rate by only 5% as compared to the success rate obtained with 1D techniques. Finally, this study demonstrates, using the example of Benin, that the use of electrical resistivity profiling and sounding for siting boreholes in weathered hard rocks of western Africa should be discarded and replaced by the use of ERT technique, more efficient.

Applications of ERTS-1 data to landscape change in eastern Tennessee

NASA Technical Reports Server (NTRS)

Rehder, J. B. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The analysis of landscape change in eastern Tennessee from ERTS-1 data is being derived from three avenues of experimentation and analysis: (1) a multi-stage sampling procedure utilizing ground and aircraft imagery for ground truth and control; (2) a densitometric and computer analytical experiment for the analysis of gray tone signatures and comparisons for landscape change detection and monitoring; and (3) an ERTS image enhancement procedure for the detection and analysis of photomorphic regions. Significant results include: maps of strip mining changes and forest inventory, watershed identification and delimitation, and agricultural regions derived from spring plowing patterns appearing on the ERTS-1 imagery.

Re-evaluation of bone pain in patients with type 1 Gaucher disease suggests that bone crises occur in small bones as well as long bones.

PubMed

Baris, Hagit N; Weisz Hubshman, Monika; Bar-Sever, Zvi; Kornreich, Liora; Shkalim Zemer, Vered; Cohen, Ian J

2016-09-01

Bone crises in type 1 Gaucher disease are reported in long bones and occasionally in weight bearing bones and other bones, but rarely in small bones of the hands and feet. We retrospectively examined the incidence of bone pain in patients followed at the Rabin Medical Center, Israel, before and following the initiation of enzyme replacement therapy (ERT) and evaluated them for bone crises. Of 100 type I Gaucher disease patients, 30 (30%) experienced one or more bone crises. Small bone crises represented 31.5% of all bone crises and were always preceded by crises in other bones. While the incidence of long bone crises reduced after the initiation of ERT, small bone crises increased. Almost 60% of patients with bone crises were of the N370S/84GG genotype suggesting a greater susceptibility of N370S/84GG patients to severe bone complications. These patients also underwent the greatest number of splenectomies (70.6% of splenectomised patients). Splenectomised patients showed a trend towards increased long and small bone crises after surgery. Active investigation of acute pain in the hands and feet in patients in our cohort has revealed a high incidence of small bone crises. Physicians should consider imaging studies to investigate unexplained pain in these areas. Copyright © 2015 Elsevier Inc. All rights reserved.

Recombinant human N-acetylgalactosamine-6-sulfate sulfatase (GALNS) produced in the methylotrophic yeast Pichia pastoris

PubMed Central

Rodríguez-López, Alexander; Alméciga-Díaz, Carlos J.; Sánchez, Jhonnathan; Moreno, Jefferson; Beltran, Laura; Díaz, Dennis; Pardo, Andrea; Ramírez, Aura María; Espejo-Mojica, Angela J.; Pimentel, Luisa; Barrera, Luis A.

2016-01-01

Mucopolysaccharidosis IV A (MPS IV A, Morquio A disease) is a lysosomal storage disease (LSD) produced by mutations on N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Recently an enzyme replacement therapy (ERT) for this disease was approved using a recombinant enzyme produced in CHO cells. Previously, we reported the production of an active GALNS enzyme in Escherichia coli that showed similar stability properties to that of a recombinant mammalian enzyme though it was not taken-up by culture cells. In this study, we showed the production of the human recombinant GALNS in the methylotrophic yeast Pichia pastoris GS115 (prGALNS). We observed that removal of native signal peptide and co-expression with human formylglycine-generating enzyme (SUMF1) allowed an improvement of 4.5-fold in the specific GALNS activity. prGALNS enzyme showed a high stability at 4 °C, while the activity was markedly reduced at 37 and 45 °C. It was noteworthy that prGALNS was taken-up by HEK293 cells and human skin fibroblasts in a dose-dependent manner through a process potentially mediated by an endocytic pathway, without any additional protein or host modification. The results show the potential of P. pastoris in the production of a human recombinant GALNS for the development of an ERT for Morquio A. PMID:27378276

Facilitating the exploitation of ERTS-1 imagery using snow enhancement techniques. [geological fault maps of Massachusetts and Connecticut

NASA Technical Reports Server (NTRS)

Wobber, F. J. (Principal Investigator); Martin, K. R.; Amato, R. V.; Leshendok, T.

1973-01-01

The author has identified the following significant results. The applications of ERTS-1 imagery for geological fracture mapping regardless of season has been repeatedly confirmed. The enhancement provided by a differential cover of snow increases the number and length of fracture-lineaments which can be detected with ERTS-1 data and accelerates the fracture mapping process for a variety of practical applications. The geological mapping benefits of the program will be realized in geographic areas where data are most needed - complex glaciated terrain and areas of deep residual soils. ERTS-1 derived fracture-lineament maps which provide detail well in excess of existing geological maps are not available in the Massachusetts-Connecticut area. The large quantity of new data provided by ERTS-1 may accelerate and improve field mapping now in progress in the area. Numerous other user groups have requested data on the techniques. This represents a major change in operating philosophy for groups who to data judged that snow obscured geological detail.

Application of ERTS-1 imagery to the harvest model of the US Menhaden fishery

NASA Technical Reports Server (NTRS)

Maughan, P. M.; Marmelstein, A. D.; Temple, O. R.

1973-01-01

Preliminary results of an experiment to demonstrate the utility of ERTS-1 imagery for providing significant information to the harvest model of the menhaden industry are reported. Fisheries and related environmental data were obtained discontinuously throughout the 1973 menhaden (a surface schooling, coastal species) fishing season in Mississippi Sound. The unexpected complexity of the physical environment in Mississippi Sound precluded simplistic analysis of fish/environment relationships. Preliminary indications are that an association does exist between fish availability and differences in water transparency (turbidity) within the Sound. A clearer relationship is developing between major turbid features, imaged by ERTS-1 and location of successful fishing attempts. On all occasions where relatively cloudfree ERTS-1 overflight days coincided with fishery activity, overlays of catch location of ERTS-1 images show an association of school position with interfaces between imaged turbid features. Analysis is currently underway to determine persistence of such associations in an attempt to define minimum satellite return time necessary to maintain continuity of associations.

Remote sensing in Iowa agriculture: Identification and classification of Iowa crop lands using ERTS-1 and complimentary underflight imagery

NASA Technical Reports Server (NTRS)

Mahlstede, J. P.; Carlson, R. E.; Thomson, G. W. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Results of the continuing analysis of ERTS-1 imagery covering Iowa during 1972 and periods during 1973 are covered. Emphasis is placed on the identification and classification of major crop types at two test sites in Iowa. Standard photointerpretive methods were used in this analysis including the direct enlargement of black and white single-band products and additive color multi-band procedures using a miniadcol system. The use of sequential coverage during the crop growing season is emphasized as a means to improve the effectiveness of ERTS-1 photointerpretations of crop land acreage estimates in Iowa. Illustrative black and white and color prints of both ERTS-1 and underflight imagery are included. In addition, forest land inventories at one test site are reported. A new method for the inventory of forest lands using ERTS-1 imagery is reported and compared with estimates obtained using earlier underflight imagery.

ERTS-1, a new window on our planet

USGS Publications Warehouse

Williams, Richard S.; Carter, William Douglas

1976-01-01

The launch, on July 23, 1972, of the first Earth Resources Technology Satellite (ERTS-1) by the National Aeronautics and Space Administration was a major step forward in extending man 's ability to inventory the Earth 's resources and to evaluate objectively his impact upon the environment. ERTS spacecraft represent the first step in merging space and remote-sensing technologies into a system for inventorying and managing the Earth 's resources. Examples presented in this book demonstrate ERTS ' vast potential for inventorying resources, monitoring environmental conditions, and measuring changes. Such information is essential for the full evaluation of the Federal lands and determining their future use, as well as for improved planning of overall land use throughout the United States and the world. Ten bureaus of the U.S. Department of Interior have roles in the ERTS project. Nearly all of these participating bureaus are represented in almost 100 papers included in this book. Chapter 3 is entitled ' Applications to Water Resources ' and contains 23 separate sections. (Woodard-USGS)

ERTS-1 anomalous dark patches

NASA Technical Reports Server (NTRS)

Strong, A. E. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Through combined use of imagery from ERTS-1 and NOAA-2 satellites was found that when the sun elevation exceeds 55 degrees, the ERTS-1 imagery is subject to considerable contamination by sunlight even though the actual specular point is nearly 300 nautical miles from nadir. Based on sea surface wave slope information, a wind speed of 10 knots will theoretically provide approximately 0.5 percent incident solar reflectance under observed ERTS multispectral scanner detectors. This reflectance nearly doubles under the influence of a 20 knot wind. The most pronounced effect occurs in areas of calm water where anomalous dark patches are observed. Calm water at distances from the specular point found in ERTS scenes will reflect no solar energy to the multispectral scanner, making these regions stand out as dark areas in all bands in an ocean scene otherwise comprosed by a general diffuse sunlight from rougher ocean surfaces. Anomalous dark patches in the outer parts of the glitter zones may explain the unusual appearance of some scenes.

ERTS data user no. 119: Effective use of ERTS multisensor data in the Great Plains. ERTS-1 MSS imagery: A tool for identifying soil associations

NASA Technical Reports Server (NTRS)

Myers, V. I. (Principal Investigator); Westin, F. C.

1973-01-01

The author has identified the following significant results. Soil association maps show the spatial relationships of land units developed in unique climatic, geologic, and topographic environments, and having characteristic slopes, soil depths, textures, available water capacities, permeabilities, and the like. ERTS-1 imagery was found to be a useful tool in the identification of soil associations since it provides a synoptic view of an 8 million acre scene, which is large enough so that the effect can be seen on soils of climate, topography, and geology. A regional view also allows soil associations to be observed over most, if not all, of their extent. ERTS-1 MSS imagery also provides four spectral bands taken every 18 days which give data on relief, hydrology, and vegetation, all of which bear on the delineation and interpretation of soil associations. Enlarged prints derived from the individual spectral bands and shown in gray tones were useful for identifying soil associations.

Interpretation and mapping of gypsy moth defoilation from ERTS (LANDSAT)-1 temporal composites

NASA Technical Reports Server (NTRS)

Mcmurtry, G. J.; Petersen, G. W. (Principal Investigator); Kowalik, W. S.

1975-01-01

The author has identified the following significant results. Photointerpretation of temporally composited color Diazo transparencies of ERTS(LANDSAT) images is a practical method for detecting and locating levels of widespread defoliation. ERTS 9 x 9 inch images are essentially orthographic and are produced at a nearly constant 1:1,000,000 scale. This allows direct superposition of scenes for temporal composites. ERTS coverage provides a sweeping 180 km (110 mile) wide view, permitting one interpreter to rapidly delineate defoliation in an area requiring days and weeks of work by aerial surveys or computerized processing. Defoliation boundaries can be located on the images within maximum errors on the order of hundreds of meters. The enhancement process is much less expensive than aerial surveys or computerized processing. Maps produced directly from interpretation are manageable working products. The 18 day periodic coverage of ERTS is not frequent enough to replace aerial survey mapping because defoliation and refoliation move as waves.

Earth resources applications of the Synchronous Earth Observatory Satellite (SEOS)

NASA Technical Reports Server (NTRS)

Lowe, D. S.; Cook, J. J.

1973-01-01

The results are presented of a four month study to define earth resource applications which are uniquely suited to data collection by a geosynchronous satellite. While such a satellite could also perform many of the functions of ERTS, or its low orbiting successors, those applications were considered in those situations where requirements for timely observation limit the capability of ERTS or EOS. Thus, the application presented could be used to justify a SEOS.

Use of ERTS-1 to utlitize and apply marine station data to study productivity along eastern shelf expanded waters of the United States

NASA Technical Reports Server (NTRS)

Marshall, H. G. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Water samples taken in offshore waters between Cap Cod, Massachusetts, and Charleston, South Carolina have been used with other sea truth information as a basis to correlate productivity values with ERTS-1 sensory data. Positive correlations were established on January 26, 1973 regarding chlorophyll concentrations and optical density values.

Management of natural resources through automatic cartographic inventory

NASA Technical Reports Server (NTRS)

Rey, P. A.; Gourinard, Y.; Cambou, F. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Significant correspondence codes relating ERTS imagery to ground truth from vegetation and geology maps have been established. The use of color equidensity and color composite methods for selecting zones of equal densitometric value on ERTS imagery was perfected. Primary interest of temporal color composite is stressed. A chain of transfer operations from ERTS imagery to the automatic mapping of natural resources was developed.

First look analyses of five cycles of ERTS-1 imagery over County of Los Angeles: Assessment of data utility for urban development and regional planning

NASA Technical Reports Server (NTRS)

Raje, S.; Economy, R.; Mcknight, J. S.

1973-01-01

Significant results have been obtained from the analyses of ERTS-1 imagery from five cycles over Test Site SR 124 by classical photointerpretation and by an interactive hybrid multispectral information extraction system (GEMS). The synopticity, periodicity and multispectrality of ERTS coverage, available for the first time to LA County planners, have opened up both a new dimensionality in data and offer new capability in preparation of planning inputs. Photointerpretation of ERTS images has produced over 25 overlays at 1:1,000,000 scale depicting regional relations and urban structure in terms of several hundred linear and areal features. To mention only one such result, a possible new fault lineament has been discovered on the northern slope of the Santa Monica mountains in the scene 1144-18015, composited of MSS bands 4, 5, 6,. GEMS analysis of the ERTS products has provided new or improved information in the following planning data categories: urban vegetation; land cover segregation; man-made and natural impact monitoring; urban design; and suitability. ERTS data analysis has allowed planners to establish trends that directly impact planning policies. This new source of information will not only assist current methods to be more efficient, but permits entirely new planning methodologies to be employed.

Principal sources and dispersal patterns of suspended particulate matter in nearshore surface waters of the northeast Pacific Ocean and seasonal variation in snow cover in the Sierra Nevada

NASA Technical Reports Server (NTRS)

Carlson, P. R. (Principal Investigator); Harden, D. R.

1973-01-01

The author has identified the following significant results. ERTS-1 imagery used in conjunction with the surface-drift cards indicated a southerly flow direction of the central California near surface coastal currents during mid-June 1973. The near-surface currents off northern California and southern Oregon were more complex. Some drift cards were recovered north and some south of their release points; however, the prevalent direction of flow was northerly. General agreement in flow direction of coastal currents obtained from ERTS-1 imagery and drift card data reinforces the image interpretation. Complete seasonal coverage of nearshore circulation interpreted from ERTS-1 imagery will provide information necessary for proper coastal zone management. Extent of snow cover can be readily delimited on ERTS-1 band 5. In the central Sierra Nevada Mountains this past winter season, the snow line, as recorded by ERTS-1, reached an elevation of less than 1500 meters in January but had melted back to between 2500 and 3000 meters by the end of May. ERTS-1 imagery seems to provide sufficient resolution to make it a useful tool for monitoring changes in snow cover in the Sierra Nevada Mountains.

ERIM progress report on use of ERTS-1 data: Summary report of work on ten tasks

NASA Technical Reports Server (NTRS)

Thomson, F. J. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Several of the tasks have produced significant results which are summarized: (1) Absolute water depth can be calculated from a ratio of signals from bands MSS 4 and MSS 5. (2) A 13 category terrain feature classification map of Yellowstone National Park has been produced using supervised pattern recognition techniques. (3) ERTS-1 data has been shown to provide a detection and monitoring capability for a number of water quality problems associated with off-shore ocean dumping sites and inland lakes. (4) A corrected ratio of bands MSS-5 and MSS-7 signals has been formed. (5) A concise format has been devised for storing the ratio signatures of geologic rock and mineral materials determined from laboratory reflectance spectra. (6) Results of work in information extraction demonstrate: signal variability exists among ERTS-1 detectors in any one spectral band that will impact users doing quantitative analysis on successive ERTS-1 images; a newly developed computer-aided procedure for correlating ERTS-1 pixels to ground features; the strong influence of atmospheric effects in ERTS-1 data; and area estimation accuracies are better using the ERIM proportion estimation algorithm than for conventional recognition techniques.

Evaluation of ERTS-1 data for inventory of forest and rangeland and detection of forest stress. [Atlanta, Georgia, Manitou, Colorado, and Black Hills

NASA Technical Reports Server (NTRS)

Heller, R. C. (Principal Investigator); Aldrich, R. C.; Driscoll, R. S.; Francis, R. E.; Weber, F. P.

1974-01-01

The author has identified the following significant results. Results of photointerpretation indicated that ERTS is a good classifier of forest and nonforest lands (90 to 95 percent accurate). Photointerpreters could make this separation as accurately as signature analysis of the computer compatible tapes. Further breakdowns of cover types at each site could not be accurately classified by interpreters (60 percent) or computer analysts (74 percent). Exceptions were water, wet meadow, and coniferous stands. At no time could the large bark beetle infestations (many over 300 meters in size) be detected on ERTS images. The ERTS wavebands are too broad to distinguish the yellow, yellow-red, and red colors of the dying pine foliage from healthy green-yellow foliage. Forest disturbances could be detected on ERTS color composites about 90 percent of the time when compared with six-year-old photo index mosaics. ERTS enlargements (1:125,000 scale, preferably color prints) would be useful to forest managers of large ownerships over 5,000 hectares (12,500 acres) for broad area planning. Black-and-white enlargements can be used effectively as aerial navigation aids for precision aerial photography where maps are old or not available.

Evaluate ERTS imagery for mapping and detection of changes of snowcover on land and on glaciers. [Cascade Mountains

NASA Technical Reports Server (NTRS)

Meier, M. F. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Snowlines on a small drainage basin were accurately identified on bulk ERTS-1 images without use of digital processing, and results checked with high altitude and ground-based photography. The area and approximate shape of snow patches as small as 20,000 sq m could be correctly identified with a magnifying scanning densitometer. The resolution of ERTS is more than ample for most snow mapping needs. Mount Baker, Washington, has a large crater south of the summit and an area north of the summit which emit considerable geothermal heat in the form of fumaroles and hot ground. Temperatures are being monitored using an ERTS DCS. Debris flows are occassionally released from the crater due to water saturation at the base of a heavy snowpack lying on hydrothermally altered hot ground. These debris flows present a possible hazard to life and property, as they are discharged down the Boulder Glacier toward Baker Lake, the upper of two major hydroelectric power reservoirs which are situated above the populated Skagit River Valley. ERTS-1 images show that the most recent debris flow (20-21 August 1973) can be clearly discerned and mapped. ERTS images provide another important tool for monitoring this potential hazard.

Correlation of ERTS-1 and aircraft optical data with water quality parameters of Charlotte Amalie Harbor, St. Thomas, Virgin Islands

NASA Technical Reports Server (NTRS)

Coulbourn, W. C.; Egan, W. G. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Attempts to correlate optical aircraft remote sensing of water quality with the optical data from the ERTS-1 satellite using calibrated imagery of Charlotte Amalie harbor, St. Thomas, Virgin Islands are reported. The harbor at Charlotte Amalie has a concentration of a number of factors affecting water quality: untreated sewage, land runoff, and sediment from navigation and dredging operations. Calibration procedures have been originated and applied to ERTS-1 and I2S camera imagery. The results indicate that the ERTS-1 and I2S imagery are correlated with optical in situ measurements of the harbor water. The aircraft green photographic and ERTS-1 MSS-4 bands have been found most suitable for monitoring the scattered light levels under the conditions of the investigation. The chemical parameters of the harbor water were found to be correlated to the optical properties for two stations investigated in detail. The biological properties of the harbor water (chlorophyll and carotenoids), correlate inversely with the optical data near the pollution sources compared to further away. Calibration procedures developed in this investigation were essential to the interpretation of the photographic and ERTS-1 photometric responses.

4D ERT-based calibration and prediction of biostimulant induced changes in fluid conductivity

NASA Astrophysics Data System (ADS)

Johnson, T. C.; Versteeg, R. J.; Day-Lewis, F. D.; Major, W. R.; Wright, K. E.

2008-12-01

In-situ bioremediation is an emerging and cost-effective method of removing organic contaminants from groundwater. The performance of bioremedial systems depends on the adequate delivery and distribution of biostimulants to contaminated zones. Monitoring the distribution of biostimulants using monitoring wells is expensive, time consuming, and provides inadequate information between sampling wells. We discuss a Hydrogeophysical Performance Monitoring System (HPMS) deployed to monitor bioremediation efforts at a TCE-contaminated Superfund site in Brandywine MD. The HPMS enables autonomous electrical geophysical data acquisition, processing, quality-assurance/quality-control, and inversion. Our objective is to demonstrate the feasibility and cost effectiveness of the HPMS to provide near real-time information on the spatiotemporal behavior of injected biostimulants. As a first step, we use time-lapse electrical resistivity tomography (ERT) to estimate changes in bulk conductivity caused by the injectate. We demonstrate how ERT-based bulk conductivity estimates can be calibrated with a small number of fluid conductivity measurements to produce ERT-based estimates of fluid conductivity. The calibration procedure addresses the spatially variable resolution of the ERT tomograms. To test the validity of these estimates, we used the ERT results to predict the fluid conductivity at tens of points prior to field sampling of fluid conductivity at the same points. The comparison of ERT-predicted vs. observed fluid conductivity displays a high degree of correlation (correlation coefficient over 0.8), and demonstrates the ability of the HPMS to estimate the four-dimensional (4D) distribution of fluid conductivity caused by the biostimulant injection.

Monitoring a pilot CO2 injection experiment in a shallow aquifer using 3D cross-well electrical resistance tomography

NASA Astrophysics Data System (ADS)

Yang, X.; Lassen, R. N.; Looms, M. C.; Jensen, K. H.

2014-12-01

Three dimensional electrical resistance tomography (ERT) was used to monitor a pilot CO2 injection experiment at Vrøgum, Denmark. The purpose was to evaluate the effectiveness of the ERT method for monitoring the two opposing effects from gas-phase and dissolved CO2 in a shallow unconfined siliciclastic aquifer. Dissolved CO2 increases water electrical conductivity (EC) while gas phase CO2 reduce EC. We injected 45kg of CO2 into a shallow aquifer for 48 hours. ERT data were collected for 50 hours following CO2 injection. Four ERT monitoring boreholes were installed on a 5m by 5m square grid and each borehole had 24 electrodes at 0.5 m electrode spacing at depths from 1.5 m to 13 m. ERT data were inverted using a difference inversion algorithm for bulk EC. 3D ERT successfully detected the CO2 plume distribution and growth in the shallow aquifer. We found that the changes of bulk EC were dominantly positive following CO2 injection, indicating that the effect of dissolved CO2 overwhelmed that of gas phase CO2. The pre-injection baseline resistivity model clearly showed a three-layer structure of the site. The electrically more conductive glacial sand layer in the northeast region are likely more permeable than the overburden and underburden and CO2 plumes were actually confined in this layer. Temporal bulk EC increase from ERT agreed well with water EC and cross-borehole ground penetrating radar data. ERT monitoring offers a competitive advantage over water sampling and GPR methods because it provides 3D high-resolution temporal tomographic images of CO2 distribution and it can also be automated for unattended operation. This work was performed under the auspices of the U.S. Department of Energy by Lawrence Livermore National Laboratory under contract DE-AC52-07NA27344. Lawrence Livermore National Security, LLC. LLNL IM release#: LLNL-PROC-657944.

Small scale monitoring of a bioremediation barrier using miniature electrical resistivity tomography

NASA Astrophysics Data System (ADS)

Sentenac, Philippe; Hogson, Tom; Keenan, Helen; Kulessa, Bernd

2015-04-01

The aim of this study was to assess, in the laboratory, the efficiency of a barrier of oxygen release compound (ORC) to block and divert a diesel plume migration in a scaled aquifer model using miniature electrical resistivity tomography (ERT) as the monitoring system. Two plumes of contaminant (diesel) were injected in a soil model made of local sand and clay. The diesel plumes migration was imaged and monitored using a miniature resistivity array system that has proved to be accurate in soil resistivity variations in small-scaled models of soil. ERT results reflected the lateral spreading and diversion of the diesel plumes in the unsaturated zone. One of the contaminant plumes was partially blocked by the ORC barrier and a diversion and reorganisation of the diesel in the soil matrix was observed. The technique of time-lapse ERT imaging showed that a dense non-aqueous phase liquid (DNAPL) contaminant like diesel can be monitored through a bioremediation barrier and the technique is well suited to monitor the efficiency of the barrier. Therefore, miniature ERT as a small-scale modelling tool could complement conventional techniques, which require more expensive and intrusive site investigation prior to remediation.

Hydrogeophysical imaging of deposit heterogeneity and groundwater chemistry changes during DNAPL source zone bioremediation.

PubMed

Chambers, J E; Wilkinson, P B; Wealthall, G P; Loke, M H; Dearden, R; Wilson, R; Allen, D; Ogilvy, R D

2010-10-21

Robust characterization and monitoring of dense nonaqueous phase liquid (DNAPL) source zones is essential for designing effective remediation strategies, and for assessing the efficacy of treatment. In this study high-resolution cross-hole electrical resistivity tomography (ERT) was evaluated as a means of monitoring a field-scale in-situ bioremediation experiment, in which emulsified vegetable oil (EVO) electron donor was injected into a trichloroethene source zone. Baseline ERT scans delineated the geometry of the interface between the contaminated alluvial aquifer and the underlying mudstone bedrock, and also the extent of drilling-induced physical heterogeneity. Time-lapse ERT images revealed major preferential flow pathways in the source and plume zones, which were corroborated by multiple lines of evidence, including geochemical monitoring and hydraulic testing using high density multilevel sampler arrays within the geophysical imaging planes. These pathways were shown to control the spatial distribution of the injected EVO, and a bicarbonate buffer introduced into the cell for pH control. Resistivity signatures were observed within the preferential flow pathways that were consistent with elevated chloride levels, providing tentative evidence from ERT of the biodegradation of chlorinated solvents. Copyright © 2010 S. Yamamoto. Published by Elsevier B.V. All rights reserved.

A reconnaissance space sensing investigation of crustal structure for a strip from the eastern Sierra Nevada to the Colorado Plateau

NASA Technical Reports Server (NTRS)

Bechtold, I. C. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Analysis of ERTS-1 MSS imagery over the sourthern Basin-Range Province of California, Nevada, and Arizona has led to recognition of regional tectonic control of volcanism, plutonism, mineralization, and fault patterns. This conclusion is the result of geologic reconnaissance of anomalies observed in ERTS-1 and Apollo-9 data, guided by intermediate scale U-2 photography, SLAR, and relevant geologic literature. In addition to regional tectonic studies, the ERTS-1 imagery provides a basis for detailed research of relatively small geologic features. Interpretation of ERTS-1 and Apollo-9 space imagery and intermediate scale X-15 and U-2 photography indicates the presence of a major fault zone along the California-Nevada state line, here named the Pahrump fault zone. Field mapping confirms previously unreported evidence of fault breaks in bedrock, along range fronts and in Quaternary alluvium and lake sediments. Regional gravity lows and fault traces within the Pahrump fault zone from a general left stepping en echelon pattern. The trend and postulated diplacement for this fault are similar to other major strike slip fault zones in the southern Basin-Range Province.

ERTS-1 imagery use in reconnaissance prospecting: Evaluation of commercial utility of ERTS-1 imagery in structural reconnaissance for minerals and petroleum

NASA Technical Reports Server (NTRS)

Saunders, D. F. (Principal Investigator); Thomas, G. L.; Kinsman, F. E.

1973-01-01

The author has identified the following significant results. Five areas in North America (North Slope-Alaska, Superior Province-Canada, Williston Basin-Montana, Colorado, and New Mexico-West Texas) are being studied for discernibility of geological evidence on ERTS-1 imagery. Evidence mapped is compared with known mineral/hydrocarbon accumulations to determine the value of the imagery in commercial exploration programs. Evaluation has proceeded in the New Mexico-Texas area, and to date, results have been better than expected. Clearly discernible structural lineaments in this area are evident on the photographs. Comparison of this evidence with known major mining localities in New Mexico indicates a clear pattern of coincidence between the lineaments and mining localities. In West Texas, lineament and geomorphological evidence obtainable from the photographs define the petroleum-productive Central Basin Platform. Based on evaluation of results in the New Mexico-West Texas area and on cursory results in the other four areas of North America, it is concluded that ERTS-1 imagery will be extremely valuable in defining the regional and local structure in any commercial exploration program.

Concepts of integrated satellite surveys. [thematic mapping of land use in Ethiopia, Sudan, and Morocco

NASA Technical Reports Server (NTRS)

Howard, J. A.

1974-01-01

The United Nations initially contracted with NASA to carry out investigations in three countries; but now as the result of rapidly increasing interest, ERTS imagery has been/is being used in 7 additional projects related to agriculture, forestry, land-use, soils, landforms and hydrology. Initially the ERTS frames were simply used to provide a synoptic view of a large area of a developing country as a basis to regional surveys. From this, interest has extended to using reconstituted false color imagery and latterly, in co-operation with Purdue University, the use of computer generated false color mosaics and computer generated large scale maps. As many developing countries are inadequately mapped and frequently rely on outdated maps, the ERTS imagery is considered to provide a very wide spectrum of valuable data. Thematic maps can be readily prepared at a scale of 1:250,000 using standard NASA imagery. These provide coverage of areas not previously mapped and provide supplementary information and enable existing maps to be up-dated. There is also increasing evidence that ERTS imagery is useful for temporal studies and for providing a new dimension in integrated surveys.

Land use, forest density, soil mapping, erosion, drainage, salinity limitations

NASA Technical Reports Server (NTRS)

Yassoglou, N. J. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The results of analyses show that it is possible to obtain information of practical significance as follows: (1) A quick and accurate estimate of the proper use of the valuable land can be made on the basis of temporal and spectral characteristics of the land features. (2) A rather accurate delineation of the major forest formations in the test areas was achieved on the basis of spatial and spectral characteristics of the studied areas. The forest stands were separated into two density classes; dense forest, and broken forest. On the basis of ERTS-1 data and the existing ground truth information a rather accurate mapping of the major vegetational forms of the mountain ranges can be made. (3) Major soil formations are mapable from ERTS-1 data: recent alluvial soils; soil on quarternary deposits; severely eroded soil and lithosol; and wet soils. (4) An estimation of cost benefits cannot be made accurately at this stage of the investigation. However, a rough estimate of the ratio of the cost for obtaining the same amount information from ERTS-1 data and from conventional operations would be approximately 1:6 to 1:10, in favor of the ERTS-1.

Quantifying potential recharge in mantled sinkholes using ERT.

PubMed

Schwartz, Benjamin F; Schreiber, Madeline E

2009-01-01

Potential recharge through thick soils in mantled sinkholes was quantified using differential electrical resistivity tomography (ERT). Conversion of time series two-dimensional (2D) ERT profiles into 2D volumetric water content profiles using a numerically optimized form of Archie's law allowed us to monitor temporal changes in water content in soil profiles up to 9 m in depth. Combining Penman-Monteith daily potential evapotranspiration (PET) and daily precipitation data with potential recharge calculations for three sinkhole transects indicates that potential recharge occurred only during brief intervals over the study period and ranged from 19% to 31% of cumulative precipitation. Spatial analysis of ERT-derived water content showed that infiltration occurred both on sinkhole flanks and in sinkhole bottoms. Results also demonstrate that mantled sinkholes can act as regions of both rapid and slow recharge. Rapid recharge is likely the result of flow through macropores (such as root casts and thin gravel layers), while slow recharge is the result of unsaturated flow through fine-grained sediments. In addition to developing a new method for quantifying potential recharge at the field scale in unsaturated conditions, we show that mantled sinkholes are an important component of storage in a karst system.

Skylab and ERTS-1 investigations of coastal land use and water properties. [Delaware Bay

NASA Technical Reports Server (NTRS)

Klemas, V. (Principal Investigator); Bartlett, D.; Rogers, R.

1974-01-01

The author has identified the following significant results. ERTS-1 multispectral scanner and Skylab's S190A, S190B, and S192 data products were evaluated for their utility in studying current circulation, suspended sediment concentrations and pollution dispersal in Delaware Bay and in mapping coastal vegetation and land use. Imagery from the ERTS-1 MSS, S190A and S190B cameras shows considerable detail in water structure, circulation, suspended sediment distribution and within waste disposal plumes in shelf waters. These data products were also used in differentiating and mapping twelve coastal vegetation and land use classes. The spatial resolution of the S190A multispectral facility appears to be about 30 to 70 meters while that of the S190B earth terrain camera is about 10 to 30 meters. Such resolution, along with good cartographic quality, indicates a considerable potential for mapping coastal land use and monitoring water properties in estuaries and on the continental shelf. The ERTS-1 MSS has a resolution of about 70-100 meters. Moreover, its regular 18-day cycle permits observation of important changes, including the environmental impact of coastal zone development on coastal vegetation and ecology.

Time-lapse 3D electrical resistivity tomography to monitor soil-plant interactions

NASA Astrophysics Data System (ADS)

Boaga, Jacopo; Rossi, Matteo; Cassiani, Giorgio; Putti, Mario

2013-04-01

In this work we present the application of time-lapse non-invasive 3D micro- electrical tomography (ERT) to monitor soil-plant interactions in the root zone in the framework of the FP7 Project CLIMB (Climate Induced Changes on the Hydrology of Mediterranean Basins). The goal of the study is to gain a better understanding of the soil-vegetation interactions by the use of non-invasive techniques. We designed, built and installed a 3D electrical tomography apparatus for the monitoring of the root zone of a single apple tree in an orchard located in the Trentino region, Northern Italy. The micro-ERT apparatus consists of 48 buried electrodes on 4 instrumented micro boreholes plus 24 mini-electrodes on the surface spaced 0.1 m on a square grid. We collected repeated ERT and TDR soil moisture measurements for one year and performed two different controlled irrigation tests: one during a very dry Summer and one during a very wet and highly dynamic plant growing Spring period. We also ran laboratory analyses on soil specimens, in order to evaluate the electrical response at different saturation steps. The results demonstrate that 3D micro-ERT is capable of characterizing subsoil conditions and monitoring root zone activities, especially in terms of root zone suction regions. In particular, we note that in very dry conditions, 3D micro ERT can image water plumes in the shallow subsoil produced by a drip irrigation system. In the very dynamic growing season, under abundant irrigation, micro 3D ERT can detect the main suction zones caused by the tree root activity. Even though the quantitative use of this technique for moisture content balance suffers from well-known inversion difficulties, even the pure imaging of the active root zone is a valuable contribution. However the integration of the measurements in a fully coupled hydrogeophysical inversion is the way forward for a better understanding of subsoil interactions between biomass, hydrosphere and atmosphere.

Improved characterisation and modelling of measurement errors in electrical resistivity tomography (ERT) surveys

NASA Astrophysics Data System (ADS)

Tso, Chak-Hau Michael; Kuras, Oliver; Wilkinson, Paul B.; Uhlemann, Sebastian; Chambers, Jonathan E.; Meldrum, Philip I.; Graham, James; Sherlock, Emma F.; Binley, Andrew

2017-11-01

Measurement errors can play a pivotal role in geophysical inversion. Most inverse models require users to prescribe or assume a statistical model of data errors before inversion. Wrongly prescribed errors can lead to over- or under-fitting of data; however, the derivation of models of data errors is often neglected. With the heightening interest in uncertainty estimation within hydrogeophysics, better characterisation and treatment of measurement errors is needed to provide improved image appraisal. Here we focus on the role of measurement errors in electrical resistivity tomography (ERT). We have analysed two time-lapse ERT datasets: one contains 96 sets of direct and reciprocal data collected from a surface ERT line within a 24 h timeframe; the other is a two-year-long cross-borehole survey at a UK nuclear site with 246 sets of over 50,000 measurements. Our study includes the characterisation of the spatial and temporal behaviour of measurement errors using autocorrelation and correlation coefficient analysis. We find that, in addition to well-known proportionality effects, ERT measurements can also be sensitive to the combination of electrodes used, i.e. errors may not be uncorrelated as often assumed. Based on these findings, we develop a new error model that allows grouping based on electrode number in addition to fitting a linear model to transfer resistance. The new model explains the observed measurement errors better and shows superior inversion results and uncertainty estimates in synthetic examples. It is robust, because it groups errors together based on the electrodes used to make the measurements. The new model can be readily applied to the diagonal data weighting matrix widely used in common inversion methods, as well as to the data covariance matrix in a Bayesian inversion framework. We demonstrate its application using extensive ERT monitoring datasets from the two aforementioned sites.

Improvement of electrical resistivity tomography for leachate injection monitoring.

PubMed

Clément, R; Descloitres, M; Günther, T; Oxarango, L; Morra, C; Laurent, J-P; Gourc, J-P

2010-03-01

Leachate recirculation is a key process in the scope of operating municipal waste landfills as bioreactors, which aims to increase the moisture content to optimize the biodegradation in landfills. Given that liquid flows exhibit a complex behaviour in very heterogeneous porous media, in situ monitoring methods are required. Surface time-lapse electrical resistivity tomography (ERT) is usually proposed. Using numerical modelling with typical 2D and 3D injection plume patterns and 2D and 3D inversion codes, we show that wrong changes of resistivity can be calculated at depth if standard parameters are used for time-lapse ERT inversion. Major artefacts typically exhibit significant increases of resistivity (more than +30%) which can be misinterpreted as gas migration within the waste. In order to eliminate these artefacts, we tested an advanced time-lapse ERT procedure that includes (i) two advanced inversion tools and (ii) two alternative array geometries. The first advanced tool uses invariant regions in the model. The second advanced tool uses an inversion with a "minimum length" constraint. The alternative arrays focus on (i) a pole-dipole array (2D case), and (ii) a star array (3D case). The results show that these two advanced inversion tools and the two alternative arrays remove almost completely the artefacts within +/-5% both for 2D and 3D situations. As a field application, time-lapse ERT is applied using the star array during a 3D leachate injection in a non-hazardous municipal waste landfill. To evaluate the robustness of the two advanced tools, a synthetic model including both true decrease and increase of resistivity is built. The advanced time-lapse ERT procedure eliminates unwanted artefacts, while keeping a satisfactory image of true resistivity variations. This study demonstrates that significant and robust improvements can be obtained for time-lapse ERT monitoring of leachate recirculation in waste landfills. Copyright 2009 Elsevier Ltd. All rights reserved.

Improvement of electrical resistivity tomography for leachate injection monitoring

DOE Office of Scientific and Technical Information (OSTI.GOV)

Clement, R., E-mail: remi.clement@hmg.inpg.f; Descloitres, M.; Guenther, T., E-mail: Thomas.Guenther@liag-hannover.d

2010-03-15

Leachate recirculation is a key process in the scope of operating municipal waste landfills as bioreactors, which aims to increase the moisture content to optimize the biodegradation in landfills. Given that liquid flows exhibit a complex behaviour in very heterogeneous porous media, in situ monitoring methods are required. Surface time-lapse electrical resistivity tomography (ERT) is usually proposed. Using numerical modelling with typical 2D and 3D injection plume patterns and 2D and 3D inversion codes, we show that wrong changes of resistivity can be calculated at depth if standard parameters are used for time-lapse ERT inversion. Major artefacts typically exhibit significantmore » increases of resistivity (more than +30%) which can be misinterpreted as gas migration within the waste. In order to eliminate these artefacts, we tested an advanced time-lapse ERT procedure that includes (i) two advanced inversion tools and (ii) two alternative array geometries. The first advanced tool uses invariant regions in the model. The second advanced tool uses an inversion with a 'minimum length' constraint. The alternative arrays focus on (i) a pole-dipole array (2D case), and (ii) a star array (3D case). The results show that these two advanced inversion tools and the two alternative arrays remove almost completely the artefacts within +/-5% both for 2D and 3D situations. As a field application, time-lapse ERT is applied using the star array during a 3D leachate injection in a non-hazardous municipal waste landfill. To evaluate the robustness of the two advanced tools, a synthetic model including both true decrease and increase of resistivity is built. The advanced time-lapse ERT procedure eliminates unwanted artefacts, while keeping a satisfactory image of true resistivity variations. This study demonstrates that significant and robust improvements can be obtained for time-lapse ERT monitoring of leachate recirculation in waste landfills.« less

An Inducible Transgenic Mouse Model for Immune Mediated Hepatitis Showing Clearance of Antigen Expressing Hepatocytes by CD8+ T Cells

PubMed Central

Cebula, Marcin; Ochel, Aaron; Hillebrand, Upneet; Pils, Marina C.; Schirmbeck, Reinhold; Hauser, Hansjörg; Wirth, Dagmar

2013-01-01

The liver has the ability to prime immune responses against neo antigens provided upon infections. However, T cell immunity in liver is uniquely modulated by the complex tolerogenic property of this organ that has to also cope with foreign agents such as endotoxins or food antigens. In this respect, the nature of intrahepatic T cell responses remains to be fully characterized. To gain deeper insight into the mechanisms that regulate the CD8+ T cell responses in the liver, we established a novel OVA_X_CreERT2 mouse model. Upon tamoxifen administration OVA antigen expression is observed in a fraction of hepatocytes, resulting in a mosaic expression pattern. To elucidate the cross-talk of CD8+ T cells with antigen-expressing hepatocytes, we adoptively transferred Kb/OVA257-264-specific OT-I T cells to OVA_X_CreERT2 mice or generated triple transgenic OVA_X CreERT2_X_OT-I mice. OT-I T cells become activated in OVA_X_CreERT2 mice and induce an acute and transient hepatitis accompanied by liver damage. In OVA_X_CreERT2_X_OT-I mice, OVA induction triggers an OT-I T cell mediated, fulminant hepatitis resulting in 50% mortality. Surviving mice manifest a long lasting hepatitis, and recover after 9 weeks. In these experimental settings, recovery from hepatitis correlates with a complete loss of OVA expression indicating efficient clearance of the antigen-expressing hepatocytes. Moreover, a relapse of hepatitis can be induced upon re-induction of cured OVA_X_CreERT2_X_OT-I mice indicating absence of tolerogenic mechanisms. This pathogen-free, conditional mouse model has the advantage of tamoxifen inducible tissue specific antigen expression that reflects the heterogeneity of viral antigen expression and enables the study of intrahepatic immune responses to both de novo and persistent antigen. It allows following the course of intrahepatic immune responses: initiation, the acute phase and antigen clearance. PMID:23869228

Combined GPR and ERT exploratory geophysical survey of the Medieval Village of Pancorbo Castle (Burgos, Spain)

NASA Astrophysics Data System (ADS)

Fernández-Álvarez, José-Paulino; Rubio-Melendi, David; Quirós Castillo, Juan Antonio; González-Quirós, Andrés; Cimadevilla-Fuente, David

2017-09-01

Ground-penetrating Radar (GPR) and Electrical Resistivity Tomography (ERT) have been fruitfully employed for archaeological purposes. An area at the Pancorbo medieval site in Burgos (Spain) has been jointly explored by GPR and ERT in the search for the buried remains of the Pancorbo medieval village. After data collection, quality control and merging, a shallow depth of interest was identified and studied in detail. 3D resistivity simulation, considering sensible geometrical structures of the targets helped discover anomalies present in the area. On the other hand, visual GPR inspection was considerably enhanced by trace energy attribute analysis which provided a plan view of the existing anomalies. Two posterior archaeological excavations have a very good correlation between the identified anomalies and the excavated remains. The survey also provides hints for the continuation of the excavation.

Interpretation of Pennsylvania agricultural land use from ERTS-1 data

NASA Technical Reports Server (NTRS)

Mcmurtry, G. J.; Petersen, G. W. (Principal Investigator); Wilson, A. D.

1974-01-01

The author has identified the following significant results. To study the complex agricultural patterns in Pennsylvania, a portion of an ERTS scene was selected for detailed analysis. Various photographic products were made and were found to be only of limited value. This necessitated the digital processing of the ERTS data. Using an unsupervised classification procedure, it was possible to delineate the following categories: (1) forest land with a northern aspect, (2) forest land with a southern aspect, (3) valley trees, (4) wheat, (5) corn, (6) alfalfa, grass, pasture, (7) disturbed land, (8) builtup land, (9) strip mines, and (10) water. These land use categories were delineated at a scale of approximately 1:20,000 on the line printer output. Land use delineations were also made using the General Electric IMAGE 100 interactive analysis system.

An analysis of Milwaukee county land use

NASA Technical Reports Server (NTRS)

Todd, W. J.; Mausel, P. E.

1973-01-01

The identification and classification of urban and suburban phenomena through analysis of remotely-acquired sensor data can provide information of great potential value to many regional analysts. Such classifications, particularly those using spectral data obtained from satellites such as the first Earth Resources Technology Satellite (ERTS-1) orbited by NASA, allow rapid frequent and accurate general land use inventories that are of value in many types of spatial analyses. In this study, Milwaukee County, Wisconsin was classified into several broad land use categories on the basis of computer analysis of four bands of ERTS spectral data (ERTS Frame Number E1017-16093). Categories identified were: (1) road-central business district, (2) grass (green vegetation), (3) suburban, (4) wooded suburb, (5) heavy industry, (6) inner city, and (7) water. Overall, 90 percent accuracy was attained in classification of these urban land use categories.

Phenology satellite experiment. [detection of brown wave and green wave in north-south corridors of United States

NASA Technical Reports Server (NTRS)

Dethier, B. E.; Ashley, M. D.; Blair, B. O.; Caprio, J. M.; Hopp, R. J.; Rouse, J., Jr. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The 1972 Brown Wave and 1973 Green Wave were detected at 24 sites located in four north-south corridors across the United States through analysis of ERTS-1 imagery and multispectral scanner digital tapes. Ground observations from these sites were correlated with ERTS data. These two phenological events were documented by observations from more than 3200 sites across the United States. The problem of changing atmospheric and illumination conditions were studied and corrections to ERTS data suggested. Band-to-band ratios were developed and correlated with the fall and spring phenological changes in field crops and forests. The results to date show the feasibility of developing and refining phenoclimatic models for use in characterizing crop status and as an aid to yield prediction.

A timer inventory based upon manual and automated analysis of ERTS-1 and supporting aircraft data using multistage probability sampling. [Plumas National Forest, California

NASA Technical Reports Server (NTRS)

Nichols, J. D.; Gialdini, M.; Jaakkola, S.

1974-01-01

A quasi-operational study demonstrating that a timber inventory based on manual and automated analysis of ERTS-1, supporting aircraft data and ground data was made using multistage sampling techniques. The inventory proved to be a timely, cost effective alternative to conventional timber inventory techniques. The timber volume on the Quincy Ranger District of the Plumas National Forest was estimated to be 2.44 billion board feet with a sampling error of 8.2 percent. Costs per acre for the inventory procedure at 1.1 cent/acre compared favorably with the costs of a conventional inventory at 25 cents/acre. A point-by-point comparison of CALSCAN-classified ERTS data with human-interpreted low altitude photo plots indicated no significant differences in the overall classification accuracies.

Remote Monitoring of Near-Surface Soil Moisture Dynamics In Unstable Slopes Using a Low-Power Autonomous Resistivity Imaging System

NASA Astrophysics Data System (ADS)

Chambers, J. E.; Meldrum, P.; Gunn, D.; Wilkinson, P. B.; Uhlemann, S.; Swift, R. T.; Kuras, O.; Inauen, C.; Hutchinson, D.; Butler, S.

2016-12-01

ERT monitoring has been demonstrated in numerous studies as an effective means of imaging near surface processes for applications as diverse as permafrost studies and contaminated land assessment. A limiting factor in applying time-lapse ERT for long-term studies in remote locations has been the availability of cost-effective ERT measurement systems designed specifically for monitoring applications. Typically, monitoring is undertaken using repeated manual data collection, or by building conventional survey instruments into a monitoring setup. The latter often requires high power and is therefore difficult to operate remotely without access to mains electricity. We describe the development of a low-power resistivity imaging system designed specifically for remote monitoring, taking advantage of, e.g., solar power and data telemetry. Here, we present the results of two field deployments. The system has been installed on an active railway cutting to provide insights into the effect of vegetation on the moisture dynamics in unstable infrastructure slopes and to gather subsurface information for pro-active remediation measures. The system, comprising 255 electrodes, acquires 4596 reciprocal measurement pairs twice daily during standard operation. In case of severe weather events, the measurement schedule is reactively changed, to gather high temporal resolution data to image rainfall infiltration processes. The system has also been installed along a leaking and marginally stable canal embankment; a less favourable location for remote monitoring, with limited solar power and poor mobile reception. Nevertheless, the acquired data indicated the effectiveness of remedial actions on the canal. The ERT results showed that one leak was caused by the canal and fixed during remediation, while two other "leaks" were shown to be effects of groundwater dynamics. The availability of cost-effective, low-power ERT monitoring instrumentation, combined with an automated workflow of data processing and visualisation, has the potential to contribute to a step-change in the management and early warning of slope instability.

Time-lapse ERT and DTS for seasonal and short-term monitoring of an alpine river hyporheic zone

NASA Astrophysics Data System (ADS)

Boaga, Jacopo; Laura, Busato; Mariateresa, Perri; Giorgio, Cassiani

2016-04-01

The hyporheic zone (HZ) is the area located beneath and adjacent to rivers and streams, where the interactions between surface water and groundwater take place. This complex physical domain allows the transport of several substances from a stream to the unconfined aquifer below, and vice versa, thus playing a fundamental role in the river ecosystem. The importance of the hyporheic zone makes its characterization a goal shared by several disciplines, which range from applied geophysics to biogeochemistry, from hydraulics to ecology. The frontier field of HZ characterization stays in applied non-invasive methodologies as Electrical Resistivity Tomography - ERT - and Distributed Temperature Sensing - DTS. ERT is commonly applied in cross-well configuration or with a superficial electrodes deployment while DTS is used in hydro-geophysics in the last decade, revealing a wide applicability to the typical issues of this field of study. DTS for hydro-geophysics studies is based on Raman scattering and employs heat as tracer and uses a fiber-optic cable to acquire temperature values. We applied both techniques for an alpine river case studies located in Val di Sole, TN, Italy. The collected measurements allow high-resolution characterization of the hyporheic zone, overcoming the critical problem of invasive measurements under riverbeds. In this work, we present the preliminary results regarding the characterization of the hyporheic zone of the alpine river obtained combining ERT and DTS time-lapse measurements. The data collection benefits from an innovative instrumentation deployment, which consists of both an ERT multicore cable and a DTS fiber-optic located in two separated boreholes drilled 5m under the watercourse and perpendicular to it. In particular we present the first year monitoring results and a short time-lapse monitoring experiment conducted during summer 2015. The site and the results here described are part of the EU FP7 CLIMB (Climate Induced Changes on the Hydrology of Mediterranean Basins) project.

Interdisciplinary study of atmospheric processes and constituents of the mid-Atlantic coastal region.. [air pollution control studies in Virginia

NASA Technical Reports Server (NTRS)

Kindle, E. C.; Bandy, E. C.; Copeland, G.; Blais, R.; Levy, G.; Sonenshine, D.

1975-01-01

Past research projects for the year 1974-1975 are listed along with future research programs in the area of air pollution control, remote sensor analysis of smoke plumes, the biosphere component, and field experiments. A detailed budget analysis is presented. Attachments are included on the following topics: mapping forest vegetation with ERTS-1 MSS data and automatic data processing techniques, and use of LARS system for the quantitative determination of smoke plume lateral diffusion coefficients from ERTS images of Virginia.

ERTS-1 imagery and native plant distributions

NASA Technical Reports Server (NTRS)

Musick, H. B.; Mcginnies, W.; Haase, E.; Lepley, L. K.

1974-01-01

A method is developed for using ERTS spectral signature data to determine plant community distribution and phenology without resolving individual plants. An Exotech ERTS radiometer was used near ground level to obtain spectral signatures for a desert plant community, including two shrub species, ground covered with live annuals in April and dead ones in June, and bare ground. It is shown that comparisons of scene types can be made when spectral signatures are expressed as a ratio of red reflectivity to IR reflectivity or when they are plotted as red reflectivity vs. IR reflectivity, in which case the signature clusters of each component are more distinct. A method for correcting and converting the ERTS radiance values to reflectivity values for comparison with ground truth data is appended.

Photographic techniques for enhancing ERTS MSS data for geologic information

NASA Technical Reports Server (NTRS)

Yost, E.; Geluso, W.; Anderson, R.

1974-01-01

Satellite multispectral black-and-white photographic negatives of Luna County, New Mexico, obtained by ERTS on 15 August and 2 September 1973, were precisely reprocessed into positive images and analyzed in an additive color viewer. In addition, an isoluminous (uniform brightness) color rendition of the image was constructed. The isoluminous technique emphasizes subtle differences between multispectral bands by greatly enhancing the color of the superimposed composite of all bands and eliminating the effects of brightness caused by sloping terrain. Basaltic lava flows were more accurately displayed in the precision processed multispectral additive color ERTS renditions than on existing state geological maps. Malpais lava flows and small basaltic occurrences not appearing on existing geological maps were identified in ERTS multispectral color images.

The results of an agricultural analysis of the ERTS-1 MSS data at the Johnson Space Center

NASA Technical Reports Server (NTRS)

Bizzell, R. M.; Wade, L. C.; Prior, H. L.; Spiers, B.

1973-01-01

The initial analysis of the ERTS-1 multispectral scanner (MSS) data at the Johnson Space Center (JSC), Houston, Texas is discussed. The primary data set utilized was the scene over Monterey Bay, California, on July 25, 1972, NASA ERTS ID No. 1002-18134. It was submitted to both computerized and image interpretative processing. An area in the San Joaquin Valley was submitted to an intensive evaluation of the ability of the data to (1) discriminate between crop types and (2) to provide a reasonably accurate area measurement of agricultural features of interest. The results indicate that the ERTS-1 MSS data is capable of providing the identifications and area extent of agricultural lands and field crop types.

Some findings on the applications of ERTS and Skylab imagery for metropolitan land use analysis

NASA Technical Reports Server (NTRS)

Alexander, R. H. (Principal Investigator); Milazzo, V. A.

1974-01-01

The author has identified the following significant results. Work undertaken on a three-sensor land use data evaluation for a portion of the Phoenix area is reported. Analyses between land use data generated from 1970 high altitude photography and that detectable from ERTS and Skylab, especially in terms of changes in land use indicate that ERTS and Skylab imagery can be used effectively to detect and identify areas of post-1970 land use change, especially those documenting urban expansion at the rural-urban fringe. Significant preliminary findings on the utility of ERTS and Skylab data for metropolitan land use analysis, substantiated by evaluation with 1970 and 1972 ground control land use data are reported.

Modelling orange tree root water uptake active area by minimally invasive ERT data and transpiration measurements

NASA Astrophysics Data System (ADS)

Vanella, Daniela; Boaga, Jacopo; Perri, Maria Teresa; Consoli, Simona; Cassiani, Giorgio

2015-04-01

The comprehension of the hydrological processes involving plant root dynamics is crucial for implementing water saving measures in agriculture. This is particular urgent in areas, like those Mediterranean, characterized by scarce water availability. The study of root water dynamics should not be separated from a more general analysis of the mass and energy fluxes transferred in the soil-plant-atmosphere continuum. In our study, in order to carry this inclusive approach, minimal invasive 3D time-lapse electrical resistivity tomography (ERT) for soil moisture estimation was combined with plant transpiration fluxes directly measured with Sap Flow (SF) techniques and Eddy Covariance methods, and volumetric soil moisture measurements by TDR probes. The main objective of this inclusive approach was to accurately define root-zone water dynamics and individuate the root-area effectively active for water and nutrient uptake process. The monitoring was carried out in Eastern Sicily (south Italy) in summers 2013 and 2014, within an experimental orange orchard farm. During the first year of experiment (October 2013), ERT measurements were carried out around the pertinent volume of one fully irrigated tree, characterized by a vegetation ground cover of 70%; in the second year (June 2014), ERT monitoring was conducted considering a cutting plant, thus to evaluate soil water dynamics without the significant plant transpiration contribution. In order to explore the hydrological dynamics of the root zone volume surrounded by the monitored tree, the resistivity data acquired during the ERT monitoring were converted into soil moisture content distribution by a laboratory calibration based on the soil electrical properties as a function of moisture content and pore water electrical conductivity. By using ERT data in conjunction with the agro-meteorological information (i.e. irrigation rates, rainfall, evapotranspiration by Eddy Covariance, transpiration by Sap Flow and soil moisture content by TRD) of the test area, a spatially distributed one-dimensional (1D) model that solves the Richards' equation was applied; in the model the van Genuchten parameters were obtained by laboratory analysis of soil water retention and soil permeability at saturation. Results of the 1D model were successfully compared with both ERT-based soil moisture dynamics and TDR measurements of soil moisture. The modelling allows to defining the soil volume interested by root water uptake process and its extent. In particular, this volume results significantly smaller (i.e. surface area of 1.75 m2, with 0.4 m cm thickness) than expected, considering the design of the drip irrigation scheme adopted in the farm. The obtained results confirm that ERT is a technique that (i) can provide a lot of information on small scale and vegetation related processes; (ii) the integration with physical modelling is essential to capture the meaning of space-time signal changes; (iii) in the case of the orange orchard, this approach shows that about half of the irrigated water is wasted.

Role of the cardio-pulmonary exercise test and six-minute walking test in the evaluation of exercise performance in patients with late-onset Pompe disease.

PubMed

Crescimanno, G; Modica, R; Lo Mauro, R; Musumeci, O; Toscano, A; Marrone, O

2015-07-01

In patients with late-onset Pompe disease, we explored the role of the Cardiopulmonary Exercise Test (CPET) and the Six-Minute Walking Test (6MWT) in the assessment of exercise capacity and in the evaluation of the effects of enzyme replacement therapy (ERT). Eight patients affected by late-onset Pompe disease, followed up at the Centre for Neuromuscular Diseases and treated with ERT, underwent a baseline evaluation with a spirometry, a CPET and a 6MWT. Four of them were restudied after 36 months of treatment. Three patients showed a reduction in exercise capacity as evaluated by peak oxygen uptake (VO2) measured at the CPET and Distance Walked (DW) measured at the 6MWT (median % predicted: 67.1 [range 54.3-99.6] and 67.3 [56.6-82.6], respectively). Cardiac and respiratory limitations revealed by the CPET were correlated to peak VO2, but not to the DW. Nevertheless, percent of predicted values of peak VO2 and DW were strongly correlated (rho = 0.85, p = 0.006), and close to identity. In the longitudinal evaluation forced vital capacity decreased, while peak VO2 and DW showed a trend to a parallel improvement. We concluded that although only the CPET revealed causes of exercise limitation, which partially differed among patients, CPET and 6MWT showed a similar overall degree of exercise impairment. That held true in the longitudinal assessment during ERT, where both tests demonstrated similar small improvements, occurring despite deterioration in forced vital capacity. Copyright © 2015 Elsevier B.V. All rights reserved.

Evaluation of neopterin as a biomarker for the monitoring of Gaucher disease patients.

PubMed

Drugan, Cristina; Drugan, Tudor C; Miron, Nicolae; Grigorescu-Sido, Paula; Naşcu, Ioana; Cătană, Cristina

2016-07-01

Biomarker research is an important area of investigation in Gaucher disease, caused by an inherited deficiency of a lysosomal enzyme, glucocerebrosidase. We evaluated the usefulness of neopterin, as a novel biomarker reflecting chronic inflammation and immune system activation in Gaucher disease and analysed its evolution in response to enzyme replacement therapy (ERT). Circulating plasma neopterin levels in 31 patients with non-neuronopathic Gaucher disease were measured before and after the onset of ERT and were compared with those of 18 healthy controls. Plasma chitotriosidase activity was also monitored, as a reference biomarker, against which we evaluated the evolution of neopterin. Neopterin levels were significantly increased in treatment-naïve patients (mean 11.90 ± 5.82 nM) compared with controls (6.63 ± 5.59 nM, Mann-Whitney U test P = 0.001), but returned to normal levels (6.92 ± 4.66 nM) following ERT. Investigating the diagnostic value of neopterin by receiver operating characteristic analysis, we found a cut-off value of 7.613 nM that corresponds to an area under the curve of 0.780 and indicates a good discrimination capacity, with a sensitivity of 0.774 and a specificity of 0.778. Our results suggest that measurement of circulating neopterin may be considered as a novel test for the confirmation of diagnosis and monitoring of the efficacy of therapeutic intervention in Gaucher disease. Plasma neopterin levels reflect the global accumulation and activation of Gaucher cells and the extent of chronic immune activation in this disorder. Neopterin may be an alternative storage cell biomarker in Gaucher disease, especially in chitotriosidase-deficient patients.

Spotlight on taliglucerase alfa in the treatment of pediatric patients with type 1 Gaucher disease.

PubMed

Gupta, Punita; Pastores, Gregory M

2017-01-01

Gaucher disease (GD) is a heritable storage disorder caused by functional defects of the lysosomal acid β-glucosidase and the accumulation of glucosylceramide within macrophages, resulting in multiple organ dysfunction. There are three commercially available enzyme replacement therapy (ERT) products for the treatment of GD type 1 (GD1): imiglucerase, velaglucerase alfa, and taliglucerase alfa. Imiglucerase and velaglucerase alfa are produced in different mammalian cell systems; imiglucerase requires postproduction deglycosylation to expose terminal α-mannose residues, which are required for mannose receptor-mediated uptake by target macrophages. These steps are critical to the success of ERT for the treatment of visceral and hematologic manifestations of GD. Taliglucerase alfa is the first US Food and Drug Administration-approved plant-cell-expressed recombinant human protein, using carrot root cell cultures. Furthermore, it does not require postproduction glycosidic modifications. It is indicated for treatment of adults with GD1 in the US, Israel, Australia, Canada, Chile, Brazil, and other countries, and it is additionally approved for the treatment of pediatric patients in the US, Australia, and Canada and for the treatment of hematologic manifestations in pediatric patients with Type 3 GD in Canada and other countries. Our review focuses on the role of taliglucerase alfa in the pediatric population. A literature search through PubMed (from 1995 up till November 2016) of English language articles was performed with the following terms: Gaucher disease, lysosomal storage disease, taliglucerase. Secondary and tertiary references were obtained by reviewing related articles as well as the website www.Clinicaltrials.gov. It has been demonstrated that taliglucerase alfa is efficacious, with a well-established safety profile in pediatric, ERT-naïve patients with symptomatic GD1, as well as for those patients previously treated with imiglucerase.

Urban street patterns detectable from ERTS-1

NASA Technical Reports Server (NTRS)

Morain, S. A. (Principal Investigator); Williams, D. L.

1973-01-01

The author has identified the following significant results. The major street patterns in Lincoln, Nebraska, are detectable on the January 24, 1973 ERTS MSS-4 image. To further study and identify the street patterns, a 3x Polaroid enlargement was made of the city from the image. An overlay of the enlargement was used to map the street patterns, with reference to the original image for clarity. The technique seems to be adaptable for updating standard road maps.

Assessment of integrated electrical resistivity data on complex aquifer structures in NE Nuba Mountains - Sudan

NASA Astrophysics Data System (ADS)

Mohamed, N. E.; Yaramanci, U.; Kheiralla, K. M.; Abdelgalil, M. Y.

2011-07-01

Two geophysical techniques were integrated to map the groundwater aquifers on complex geological settings, in the crystalline basement terrain in northeast Nuba Mountains. The water flow is structurally controlled by the northwest-southeast extensional faults as one of several in-situ deformational patterns that are attributed to the collision of the Pan-African oceanic assemblage of the Nubian shield against the pre-Pan African continental crust to the west. The structural lineaments and drainage systems have been enhanced by the remote sensing technique. The geophysical techniques used are: vertical electrical soundings (VES) and electrical resistivity tomography (ERT), in addition to hydraulic conductivity measurements. These measurements were designed to overlap in order to improve the producibility of the geophysical data and to provide a better interpretation of the hydrogeological setting of the aquifer complex structure. Smooth and Block inversion schemes were attempted for the observed ERT data to study their reliability in mapping the different geometries in the complex subsurface. The VES data was conducted where ERT survey was not accessible, and inverted smoothly and merged with the ERT in the 3D resistivity grid. The hydraulic conductivity was measured for 42 water samples collected from the distributed dug wells in the study area; where extremely high saline zones were recorded and have been compared to the resistivity values in the 3D model.

ERTS & EROS

ERIC Educational Resources Information Center

Geotimes, 1972

1972-01-01

Describes the proposed investigations to be conducted with ERTS (Earth Resources Technology Satellite), the first experimental satellite for systematically surveying earth resources by remote sensing. Launching set for June, 1972. (PR)

Multidisciplinary geoscientific experiments in central Europe

NASA Technical Reports Server (NTRS)

Bannert, D. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Studies were carried out in the fields of geology-pedology, coastal dynamics, geodesy-cartography, geography, and data processing. In geology-pedology, a comparison of ERTS image studies with extensive ground data led to a better understanding of the relationship between vegetation, soil, bedrock, and other geologic features. Findings in linear tectonics gave better insight in orogeny and ore deposit development for prospecting. Coastal studies proved the value of ERTS images for the updating of nautical charts, as well as small scale topographic maps. A plotter for large scale high speed image generation from CCT was developed.

Seawater intrusion mapping using electrical resistivity tomography and hydrochemical data. An application in the coastal area of eastern Thermaikos Gulf, Greece.

PubMed

Kazakis, N; Pavlou, A; Vargemezis, G; Voudouris, K S; Soulios, G; Pliakas, F; Tsokas, G

2016-02-01

The aim of this study was to determine the extent and geometrical characteristics of seawater intrusion in the coastal aquifer of the eastern Thermaikos Gulf, Greece. Hydrochemical data and geoelectrical measurements were combined and supplemented to determine the hydrochemical regime of the study site in regard to seawater phenomena. Chemical analysis of groundwater was performed in 126 boreholes and fifteen electrical resistivity tomographies (ERT) were measured, whereas in two sites the ERT measurements were repeated following the wet season. The Cl(-) concentrations recorded reached 2240 mg/L indicating seawater intrusion which was also verified by ionic ratios. The ionic ratios were overlapped and a seawater intrusion map (SWIM) was produced. A significant part of the coastal aquifer (up to 150 km(2)) is influenced by seawater intrusion. The areas with the most intensive salinization are located between Nea Kallikratia-Epanomi and Aggelochori-Peraia. According to the ERTs, in the influenced areas the salinization of the aquifer exceeds 1 km toward the mainland and its depth reaches 200 m. In the area surrounding Thessaloniki airport, the ERTs revealed salinization of the upper aquifer to depths of up to 40 m, whereas the lower aquifer is uninfluenced. This abnormal distribution of seawater intrusion demonstrates the value of geoelectrical methods in the study of seawater intrusion especially in areas with limited available hydrochemical data. Copyright © 2015 Elsevier B.V. All rights reserved.

On the Salt Water Intrusion into the Durusu Lake, Istanbul: A Joint Central Loop TEM And Multi-Electrode ERT Field Survey

NASA Astrophysics Data System (ADS)

Ardali, Ayça Sultan; Tezkan, Bülent; Gürer, Aysan

2018-02-01

Durusu Lake is the biggest and most important freshwater source supplying drinking water to the European side of Istanbul. In this study, electrical resistivity tomography (ERT) and transient electromagnetic (TEM) measurements were applied to detect a possible salt water intrusion into the lake and to delineate the subsurface structure in the north of Durusu Lake. The ERT and TEM measurements were carried out along six parallel profiles extending from the sea coast to the lake shore on the dune barrier. TEM data were interpreted using different 1-D inversion methods such as Occam, Marquardt, and laterally constrained inversion (LCI). ERT data were interpreted using 2-D inversion techniques. The inversion results of ERT and TEM data were shown as resistivity depth sections including topography. The sand layer spreading over the basin has a resistivity of 150-400 Ωm with a thickness of 5-10 m. The sandy layer with clay, silt, and gravel has a resistivity of 15-100 Ωm and a thickness of 10-40 m followed by a clay layer of a resistivity below 10 Ωm. When the inversion of these data is interpreted along with the hydrogeology of the area, it is concluded that the salt water intrusion along the dune barrier is not common and occurs at a particular area where the distance between lake and sea is very close. Using information from boreholes around the lake, it was verified that the common conductive region at depths of 30 m or more consists of clay layers and clay lenses.

Time-lapse electrical geophysical monitoring of amendment-based biostimulation

USGS Publications Warehouse

Johnson, Timothy C.; Versteeg, Roelof J.; Day-Lewis, Frederick D.; Major, William; Lane, John W.

2015-01-01

Biostimulation is increasingly used to accelerate microbial remediation of recalcitrant groundwater contaminants. Effective application of biostimulation requires successful emplacement of amendment in the contaminant target zone. Verification of remediation performance requires postemplacement assessment and contaminant monitoring. Sampling-based approaches are expensive and provide low-density spatial and temporal information. Time-lapse electrical resistivity tomography (ERT) is an effective geophysical method for determining temporal changes in subsurface electrical conductivity. Because remedial amendments and biostimulation-related biogeochemical processes often change subsurface electrical conductivity, ERT can complement and enhance sampling-based approaches for assessing emplacement and monitoring biostimulation-based remediation.Field studies demonstrating the ability of time-lapse ERT to monitor amendment emplacement and behavior were performed during a biostimulation remediation effort conducted at the Department of Defense Reutilization and Marketing Office (DRMO) Yard, in Brandywine, Maryland, United States. Geochemical fluid sampling was used to calibrate a petrophysical relation in order to predict groundwater indicators of amendment distribution. The petrophysical relations were field validated by comparing predictions to sequestered fluid sample results, thus demonstrating the potential of electrical geophysics for quantitative assessment of amendment-related geochemical properties. Crosshole radar zero-offset profile and borehole geophysical logging were also performed to augment the data set and validate interpretation.In addition to delineating amendment transport in the first 10 months after emplacement, the time-lapse ERT results show later changes in bulk electrical properties interpreted as mineral precipitation. Results support the use of more cost-effective surface-based ERT in conjunction with limited field sampling to improve spatial and temporal monitoring of amendment emplacement and remediation performance.

Patterns in Soil Electrical Resistivity Across Land Uses in the Calhoun Critical Zone Observatory Landscape

NASA Astrophysics Data System (ADS)

Markewitz, D.; Sutter, L.; Richter, D. D., Jr.

2017-12-01

Soil Electrical Resistivity Tomography (ERT) was measured across the Calhoun Critical Zone Observatory in relation to land use cover. ERT can help identify patterns in soil and saprolite physical attributes and moisture content through multiple meters. ERT data were generated with an AGI Supersting R8 with a 28 probe dipole-dipole array on a 1.5 meter spacing providing information through the upper 9 m. In Nov/Dec 2016 ten soil pits were dug to 3m depth in agricultural fields, pine forests, and hardwood forests across the CCZO and ERT measures were taken centered on these pits. ERT values ranged from 200 to 2500 Ohm-m. ERT patterns in the agricultural field demonstrated a limited resistivity gradient (200-700 Ohm-m) appearing moist throughout. In contrast, research areas under pine and hardwood forest had stronger resistivity gradients reflecting both moisture and physical attributes (i.e., texture or rock content). For example, research area 2 under pine had an area of higher resistivity that correlated with a band of saprolite that was readily visible in the exposed profile. In research area 7 and 8 that included both pine and hardwood forest resistivity gradients had contradictory patterns of high to low resistivity from top to bottom. In research area 7 resistivity was highest at the surface and decreased with depth, a common pattern when water table is at depth. In research area 8 the inverse was observed with low resistivity above and resistivity increasing with depth, a pattern observed in upper landscape positions on ridges with moist clay above dry saprolite. ERT patterns did reflect a large difference in the measured agricultural fields compared to forest while other difference appeared to reflect landscape position.

New Electrical Resistivity Tomography approach for karst cave characterization: Castello di Lepre karst cave (Marsico Nuovo, Southern Italy).

NASA Astrophysics Data System (ADS)

Guerriero, Merilisa; Capozzoli, Luigi; De Martino, Gregory; Perciante, Felice; Gueguen, Erwan; Rizzo, Enzo

2017-04-01

Geophysical methods are commonly applied to characterize karst cave. Several geophysical method are used such as electrical resistivity tomography (ERT), gravimetric prospecting (G), ground penetrating radar (GPR) and seismic methods (S), in order to provide information on cave geometry and subsurface geological structure. In detail, in some complex karst systems, each geophysical method can only give partial information if used in normal way due to a low resolution for deep target. In order to reduce uncertainty and avoid misinterpretations based on a normal use of the electrical resistivity tomography method, a new ERT approach has been applied in karst cave Castello di Lepre (Marsico Nuovo, Basilicata region, Italy) located in the Mezo-Cenozoic carbonate substratum of the Monti della Maddalena ridge (Southern Appenines). In detail, a cross-ERT acquisition system was applied in order to improve the resolution on the electrical resistivity distribution on the surrounding geological structure of a karst cave. The cross-ERT system provides a more uniform model resolution vertically, increasing the resolution of the surface resistivity imaging. The usual cross-ERT is made by electrode setting in two or more borehole in order to acquire the resistivity data distribution. In this work the cross-ERT was made between the electrodes located on surface and along a karst cave, in order to obtain an high resolution of the electrical resistivity distributed between the cave and the surface topography. Finally, the acquired cross-ERT is potentially well-suited for imaging fracture zones since electrical current flow in fractured rock is primarily electrolytic via the secondary porosity associated with the fractures.

ERTS-A data as a teaching and research tool in the Department of Geology

NASA Technical Reports Server (NTRS)

Grybeck, D. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The ERTS-1 materials continue to be used in a number of courses including Geology of Alaska, Economic Geology, and Structural Geology. In addition, specific talks about the ERTS-1 material were given at a seminar at the Geophysical Institute, to the Geology Department, to numerous individuals, and were extensively used in a popularized talk on the Geology of Alaska to the local Historical Society.

ERTS-1 applications to Minnesota land use mapping

NASA Technical Reports Server (NTRS)

Brown, D.; Gamble, J.; Prestin, S.; Trippler, D.; Meyer, M. P.; Ulliman, J. J.; Eller, R. G.

1973-01-01

Land use class definitions that can be operationally employed with ERTS-1 imagery are being developed with the cooperation of personnel from several state, regional, and federal agencies with land management responsibilities within the state and the University of Minnesota. Investigations of urban, extractive, forest, and wetlands areas indicate that it is feasible to subdivide each of these classes into several sub-classes with the use of ERTS-1 images from one or more time periods.

Effects of spatially variable resolution on field-scale estimates of tracer concentration from electrical inversions using Archie's law

USGS Publications Warehouse

Singha, Kamini; Gorelick, Steven M.

2006-01-01

Two important mechanisms affect our ability to estimate solute concentrations quantitatively from the inversion of field-scale electrical resistivity tomography (ERT) data: (1) the spatially variable physical processes that govern the flow of current as well as the variation of physical properties in space and (2) the overparameterization of inverse models, which requires the imposition of a smoothing constraint (regularization) to facilitate convergence of the inverse solution. Based on analyses of field and synthetic data, we find that the ability of ERT to recover the 3D shape and magnitudes of a migrating conductive target is spatially variable. Additionally, the application of Archie's law to tomograms from field ERT data produced solute concentrations that are consistently less than 10% of point measurements collected in the field and estimated from transport modeling. Estimates of concentration from ERT using Archie's law only fit measured solute concentrations if the apparent formation factor is varied with space and time and allowed to take on unreasonably high values. Our analysis suggests that the inability to find a single petrophysical relation in space and time between concentration and electrical resistivity is largely an effect of two properties of ERT surveys: (1) decreased sensitivity of ERT to detect the target plume with increasing distance from the electrodes and (2) the smoothing imprint of regularization used in inversion.

Central Atlantic regional ecological test site: A prototype regional environmental information system

NASA Technical Reports Server (NTRS)

Alexander, R. H. (Principal Investigator)

1973-01-01

The author has identified the following significant results. A comparison of photomorphic regions from an uncontrolled ERTS-1 mosaic of CARETS to land use areas on a map published in the National Atlas revealed close correlations in non-urban regions. Such regional scale analysis of ERTS-1 data has the potential for providing an economical sampling strategy for selecting sites for more detailed field measurements if other environmental variables can be correlated with patterns on ERTS-1 imagery. ERTS-1 imagery has also revealed for the first time the appearance of CARETS during the winter months. Investigators have identified extensive areas of conifers, which have previously been indistinguishable from deciduous vegetation. Imagery has also shown very clearly the extent of snow cover at a particular time over the region. The evaluation of ERTS-1 imagery used for the land use mapping of the shore zone of CARETS, has shown that the presence or absence of elements of an hierarchal system of shoreline landforms can help identify areas of potential rapid change. Changes in land use class distributions on the Barrier Islands signify the environmental response to natural and man-caused processes. Both environmental vulnerability and sensitivity can be estimated from the repetitive ERTS-1 coverage of long reaches of the CARETS coast. Results indicate potential applications to land use planning, management, and regional environmental quality analysis.

Cost analysis of cetuximab (Erbitux) plus radiotherapy (ERT) versus concomitant cisplatin plus radiotherapy (CRT) within an NHS oncology unit (single institution): a pilot study

PubMed Central

Sundus, Yahya; Dawn, Dawson; Carol, Glaister; Susan, Merrick

2016-01-01

Objective: The aim of this feasibility study is to define the resource effectiveness of cetuximab vs cisplatin given concomitantly with radiotherapy for squamous cell carcinoma within a National Health Service clinical oncology unit. Methods: 20 patients with Stage 3 or 4 head and neck squamous cell cancers were randomized to receive either cetuximab with radiotherapy (ERT) or cisplatin with radiotherapy concurrent with external beam radiotherapy 70 Gy in 35 fractions on a 1 : 1 basis over a 12-month duration. The study compared the resource utilization of ERT vs cisplatin with radiotherapy taking into account drug costs, clinical management and the costs of managing treatment-related toxicity from first fraction of radiotherapy to 6 months after the completion of therapy. Outcome measures were quality of life (recorded at the entry, end of radiotherapy, 6 weeks post treatment and 6 months post treatment), admissions to hospital, delays to radiotherapy, locoregional control and survival. Results: Total drug costs including cost of nutritional supplements for patients treated with cetuximab were £7407.45 compared with £3959.07 for patients treated with cisplatin. Unscheduled admissions for toxicity management were significantly more common in the ERT arm. Healthcare personnel spent significantly more time delivering unscheduled outpatient care for patients receiving cisplatin than for those receiving cetuximab (p = 0.01). No significant difference in the quality of life was suggested at baseline, 6 weeks and 6 months. The mean time to removal of percutaneous gastrostomy (PEG) after completion of radiotherapy was 49.7 weeks in the cisplatin arm and 18.5 weeks in the cetuximab arm (p = 0.04). There was a statistically significant difference in patient-reported use of PEG between the cisplatin and cetuximab arms at 6 months following completion of treatment (p = 0.04). At 21 months, overall survival was 80% in the cisplatin arm vs 50% in the cetuximab (p = 0.332), with disease-free survival being 80% in the cisplatin arm vs 40% in the cetuximab (p = 0.097). Conclusion: Cetuximab is still more expensive in simple drug cost terms than cisplatin when delivered with radiotherapy taking into account costs of drugs for toxicity management and nutritional supplements but other resource implications such as inpatient admission, time spent delivering unscheduled care and cost of additional investigations to manage toxicity for patients treated with cisplatin significantly reduce differential. The study suggested significant differences in patient-reported PEG use at 6 months and in time to PEG removal in favour of the cetuximab arm. Advances in knowledge: There is paucity of randomized data on cost analysis for cisplatin vs cetuximab radiotherapy; this trial informs on the cost analysis between the two approaches. PMID:27734748

Application of ERTS-1 data to the protection and management of New Jersey's coastal environment

NASA Technical Reports Server (NTRS)

Yunghans, R. S. (Principal Investigator); Feinberg, E. B.; Stitt, J. A.; Mairs, R. L.; Macomber, R. T.; Stanczuk, D.; Wobber, F. J.

1974-01-01

The author has identified the following significant results. The principal thrust of this ERTS-1 experiment is to develop quasi-operational information products from analysis of ERTS-1 imagery and collateral aerial photography and to apply these products to the practical regulation, protection, and management of New Jersey's coastal environment. Incorporated into this goal is the development of procedures for the operational use of ERTS-1 data products within New Jersey's Department of Environmental Protection. Analysis and product preparation for operational needs has centered on four major coastal resource problem areas: detection of land use changes in the coastal zone; siting of ocean outfalls; monitoring of offshore waste disposal; and calculation of recession rates along the Atlantic Shore. The relative utility and estimated monetary benefits derived from ERTS and aircraft imagery for each problem area have been determined. Of equal importance is the development to a capability within the State to use and understand remote sensor-derived information, and the application of this information to meet the requirements of current and anticipated coastal zone legislation.

Evaluation and comparison of ERTS measurements of major crops and soil associations for selected sites in the central United States

NASA Technical Reports Server (NTRS)

Baumgardner, M. F. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The most significant result was the use of the temporal overlay technique where the computer was used to overlay ERTS-1 data from three different dates (9 Oct., 14 Nov., 2 Dec.). The registration of MSS digital data from different dates was estimated to be accurate within one half resolution element. The temporal overlay capability provides a significant advance in machine-processing of MSS data. It is no longer essential to go through the tedious exercise of locating ground observation sites on the digital data from each ERTS-1 overpass. Once the address of a ground observation site has been located on a digital tape from any ERTS-1 overpass, the overlay technique can be used to locate the same address on a digital tape of MSS data from any other ERTS-1 pass over the same area. The temporal overlay technique also adds a valuable dimension for identifying and mapping changes in vegetation, water, and other dynamic surface features.

Evaluation of the application of ERTS-1 data to the regional land use planning process. [Wisconsin

NASA Technical Reports Server (NTRS)

Clapp, J. L. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Investigators have concentrated on efforts toward assessing the achievements of the current ERTS-1 research so that significant results could be incorporated into the updated version of their ERTS-B research proposal. Significant progress was made in the development of operational mechanisms, linking the research community at the University and the operation agencies of the State of Wisconsin. Efforts have been made to finalize a report which is being prepared for the State of Wisconsin Department of Administration, evaluating the applicability of ERTS-1 to the planning needs of the State of Wisconsin. This document is intended to assess the degree of utility of the satellite for providing information which has been designated by state planners as essential to their needs. This effort is considered highly significant to the overall research program since a situation has been created in which a prime potential user of ERTS type data has been given the opportunity to determine the direction of the research.

An interdisciplinary analysis of ERTS data for Colorado mountain environments using ADP Techniques

NASA Technical Reports Server (NTRS)

Hoffer, R. M. (Principal Investigator)

1972-01-01

Author identified significant preliminary results from the Ouachita portion of the Texoma frame of data indicate many potentials in the analysis and interpretation of ERTS data. It is believed that one of the more significant aspects of this analysis sequence has been the investigation of a technique to relate ERTS analysis and surface observation analysis. At present a sequence involving (1) preliminary analysis based solely upon the spectral characteristics of the data, followed by (2) a surface observation mission to obtain visual information and oblique photography to particular points of interest in the test site area, appears to provide an extremely efficient technique for obtaining particularly meaningful surface observation data. Following such a procedure permits concentration on particular points of interest in the entire ERTS frame and thereby makes the surface observation data obtained to be particularly significant and meaningful. The analysis of the Texoma frame has also been significant from the standpoint of demonstrating a fast turn around analysis capability. Additionally, the analysis has shown the potential accuracy and degree of complexity of features that can be identified and mapped using ERTS data.

Vegetation analysis in the Laramie Basin, Wyoming from ERTS-1 imagery

NASA Technical Reports Server (NTRS)

Evans, M. A.; Redfern, F. R.

1973-01-01

The author has identified the following significant results. The application of ERTS-1 imagery to vegetation mapping and identification was tested and confirmed by field checking. ERTS-1 imagery interpretation and density contour mapping allows definition of minute vegetation features and estimation of vegetative biomass and species composition. Large- and small-scale vegetation maps were constructed for test areas in the Laramie Basin and Laramie mountains of Wyoming. Vegetative features reflecting grazing intensity, moisture availability, changes within the growing season, cutting of hay crops, and plant community constituents in forest and grassland are discussed and illustrated. Theoretical considerations of scattering, sun angle, slope, and instrument aperture upon image and map resolution were investigated. Future suggestions for applications of ERTS-1 data to vegetative analysis are included.

ERTS-1 DCS technical support provided by Wallops Station. [ground truth stations and DCP repair depot

NASA Technical Reports Server (NTRS)

Smith, R.

1975-01-01

Wallops Station accepted the tasks of providing ground truth to several ERTS investigators, operating a DCP repair depot, designing and building an airborne DCP Data Acquisition System, and providing aircraft underflight support for several other investigators. Additionally, the data bank is generally available for use by ERTS and other investigators that have a scientific interest in data pertaining to the Chesapeake Bay area. Working with DCS has provided a means of evaluating the system as a data collection device possibly applicable to ongoing Earth Resources Program activities in the Chesapeake Bay area as well as providing useful data and services to other ERTS investigators. The two areas of technical support provided by Wallops, ground truth stations and repair for DCPs, are briefly discussed.

Film/chemistry selection for the earth resources technology satellite /ERTS/ ground data handling system

NASA Technical Reports Server (NTRS)

Shaffer, R. M.

1973-01-01

A detailed description is given of the methods of choose the duplication film and chemistry currently used in the NASA-ERTS Ground Data Handling System. The major ERTS photographic duplication goals are given as background information to justify the specifications for the desirable film/chemistry combination. Once these specifications were defined, a quantitative evaluation program was designed and implemented to determine if any recommended combinations could meet the ERTS laboratory specifications. The specifications include tone reproduction, granularity, MTF and cosmetic effects. A complete description of the techniques used to measure the test response variables is given. It is anticipated that similar quantitative techniques could be used on other programs to determine the optimum film/chemistry consistent with the engineering goals of the program.

Adenosine Deaminase (ADA)-Deficient Severe Combined Immune Deficiency (SCID): Molecular Pathogenesis and Clinical Manifestations.

PubMed

Bradford, Kathryn L; Moretti, Federico A; Carbonaro-Sarracino, Denise A; Gaspar, Hubert B; Kohn, Donald B

2017-10-01

Deficiency of adenosine deaminase (ADA, EC3.5.4.4), a housekeeping enzyme of purine metabolism encoded by the Ada gene, is a cause of human severe combined immune deficiency (SCID). Numerous deleterious mutations occurring in the ADA gene have been found in patients with profound lymphopenia (T - B - NK - ), thus underscoring the importance of functional purine metabolism for the development of the immune defense. While untreated ADA SCID is a fatal disorder, there are multiple life-saving therapeutic modalities to restore ADA activity and reconstitute protective immunity, including enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplantation (HSCT) and gene therapy (GT) with autologous gene-corrected hematopoietic stem cells (HSC). We review the pathogenic mechanisms and clinical manifestations of ADA SCID.

ERTS-1 imagery and high flight photographs as aids to fire hazard appraisal at the NASA San Pablo Reservoir test site

NASA Technical Reports Server (NTRS)

Colwell, R. N.

1973-01-01

The identification of fire hazards at the San Pablo Reservoir Test Site in California using ERTS-1 data is discussed. It is stated that the two primary fire hazards in the area are caused by wild oat plants and eucalyptus trees. The types of imagery used in conducting the study are reported. Aerial photographs of specific areas are included to show the extent of the fire hazards.

Automatic photointerpretation for land use management in Minnesota

NASA Technical Reports Server (NTRS)

Swanlund, G. D. (Principal Investigator); Pile, D. R.

1973-01-01

The author has identified the following significant results. Primary conclusions from the lake acreage study are: (1) The ERTS-1 band 7 density range of 0-5 reliably indicates open water down to 2 acre size. (2) The density range 6-9 identifies swamps. (3) The depth of the water could not be determined. (4) Cloud shadows can be misread as lakes unless the clouds are detected. (5) ERTS-1 data would provide the information for classifying lakes and for monitoring fluctuations in lake area.

A study to explore the use of orbital remote sensing to determine native arid plant distribution. [Arizona

NASA Technical Reports Server (NTRS)

Mcginnies, W. G. (Principal Investigator); Conn, J. S.; Haase, E. F.; Lepley, L. K.; Musick, H. B.; Foster, K. E.

1975-01-01

The author has identified the following significant results. Research results include a method for determining the reflectivities of natural areas from ERTS data taking into account sun angle and atmospheric effects on the radiance seen by the satellite sensor. Ground truth spectral signature data for various types of scenes, including ground with and without annuals, and various shrubs were collected. Large areas of varnished desert pavement are visible and mappable on ERTS and high altitude aircraft imagery. A large scale and a small scale vegetation pattern were found to be correlated with presence of desert pavement. A comparison of radiometric data with video recordings shows quantitatively that for most areas of desert vegetation, soils are the most influential factor in determining the signature of a scene. Additive and subtractive image processing techniques were applied in the dark room to enhance vegetational aspects of ERTS.

Mapping forest vegetation with ERTS-1 MSS data and automatic data processing techniques

NASA Technical Reports Server (NTRS)

Messmore, J.; Copeland, G. E.; Levy, G. F.

1975-01-01

This study was undertaken with the intent of elucidating the forest mapping capabilities of ERTS-1 MSS data when analyzed with the aid of LARS' automatic data processing techniques. The site for this investigation was the Great Dismal Swamp, a 210,000 acre wilderness area located on the Middle Atlantic coastal plain. Due to inadequate ground truth information on the distribution of vegetation within the swamp, an unsupervised classification scheme was utilized. Initially pictureprints, resembling low resolution photographs, were generated in each of the four ERTS-1 channels. Data found within rectangular training fields was then clustered into 13 spectral groups and defined statistically. Using a maximum likelihood classification scheme, the unknown data points were subsequently classified into one of the designated training classes. Training field data was classified with a high degree of accuracy (greater than 95%), and progress is being made towards identifying the mapped spectral classes.

Mapping forest vegetation with ERTS-1 MSS data and automatic data processing techniques

NASA Technical Reports Server (NTRS)

Messmore, J.; Copeland, G. E.; Levy, G. F.

1975-01-01

This study was undertaken with the intent of elucidating the forest mapping capabilities of ERTS-1 MSS data when analyzed with the aid of LARS' automatic data processing techniques. The site for this investigation was the Great Dismal Swamp, a 210,000 acre wilderness area located on the Middle Atlantic coastal plain. Due to inadequate ground truth information on the distribution of vegetation within the swamp, an unsupervised classification scheme was utilized. Initially pictureprints, resembling low resolution photographs, were generated in each of the four ERTS-1 channels. Data found within rectangular training fields was then clustered into 13 spectral groups and defined statistically. Using a maximum likelihood classification scheme, the unknown data points were subsequently classified into one of the designated training classes. Training field data was classified with a high degree of accuracy (greater than 95 percent), and progress is being made towards identifying the mapped spectral classes.

Regional agriculture surveys using ERTS-1 data

NASA Technical Reports Server (NTRS)

Draeger, W. C.; Nichols, J. D.; Benson, A. S.; Larrabee, D. G.; Jenkus, W. M.; Hay, C. M.

1974-01-01

The Center for Remote Sensing Research has conducted studies designed to evaluate the potential application of ERTS data in performing agricultural inventories, and to develop efficient methods of data handling and analysis useful in the operational context for performing large area surveys. This work has resulted in the development of an integrated system utilizing both human and computer analysis of ground, aerial, and space imagery, which has been shown to be very efficient for regional crop acreage inventories. The technique involves: (1) the delineation of ERTS images into relatively homogeneous strata by human interpreters, (2) the point-by-point classification of the area within each strata on the basis of crop type using a human/machine interactive digital image processing system; and (3) a multistage sampling procedure for the collection of supporting aerial and ground data used in the adjustment and verification of the classification results.

Mineral exploration potential of ERTS-1 data

NASA Technical Reports Server (NTRS)

Brewer, W. A. (Principal Investigator); Erskine, M. C., Jr.; Prindle, R. O.

1973-01-01

The author has identified the following significant results. Further analysis of ERTS-1 MSS imagery of Arizona has led to division of the earlier reported three major regional fault/fracture systems into eight subdivisions. These are: System A - N40E to N50E; System B - N50W; System C - N35W; System D - N65E to N75E; System E - N-E; System F - N25W average; System G - N75W average; and System H - close to E-W. Their individual significance to known porphyry coppers and correlation to the Wasatch-Jerome and Front Orogens, the Texas Zone and Basin Range topography is postulated in the report. In this study area of Arizona the ERTS-1 mapped structural trends confirm and greatly extend some concepts of porphyry copper distribution based on prior geologic knowledge of major structural trends.

Study of the application of ERTS-A imagery to fracture-related mine safety hazards in the coal mining industry

NASA Technical Reports Server (NTRS)

Wier, C. E.; Wobber, F. J. (Principal Investigator); Russell, O. R.; Amato, R. V.

1972-01-01

The author has identified the following significant results. Various data compilation and analysis activities in support of ERTS-1 imagery interpretation are in progress or are completed. These include the compilation of mine accident data, areas of mine roof instability and the analysis of high altitude color infrared photography and low altitude color and color infrared photography which was acquired by NASA in support of the project. The photography reveals that many fracture lineaments are detectable through a varied thickness of glacial till. These data will be compiled on a series of 1:250,000 scale base maps and evaluated for a correlation between fracture zones and mine accidents and rooffalls. Due to high occurrence of cloud cover in the project area and to the delay in imagery shipments, little progress has been made in the analysis of ERTS-1 imagery.

The ERTS-1 investigation (ER-600). Volume 1: ERTS-1 agricultural analysis

NASA Technical Reports Server (NTRS)

Erb, R. B.

1974-01-01

The Agriculture Analysis Team of the Johnson Space Center conducted a 1-year-long investigation of ERTS-1 multispectral data to evaluate how well features of agricultural importance could be detected, identified, and located; and their areal extent measured. Six study areas were selected in cooperation with the U.S. Department of Agriculture. Two basic analytical approaches were used to meet the objectives. The conventional image interpretation technique revealed that a particular color was an indication of the density of vegetative cover, not an indication of crop classification. Computer-aided techniques were used to classify crop types (i.e., small grains, truck farm crops, grasses, summer fallow) to accuracies as high as 95 percent on large (12 hectares or more) well-defined fields. A further breakdown into crop species (wheat, barley, soybeans, oats, corn) reduced the accuracy to 70 to 80 percent for single-date observations.

Interdisciplinary applications and interpretations of ERTS data within the Susquehanna River Basin (resource inventory, land use, and pollution)

NASA Technical Reports Server (NTRS)

Mcmurtry, G. J.; Petersen, G. W. (Principal Investigator)

1973-01-01

The author has identified the following significant results. An interdisciplinary group at Penn State University is analyzing ERTS-1 data. The geographical area of interest is that of the Susquehanna River Basin in Pennsylvania. The objectives of the work have been to ascertain the usefulness of ERTS-1 data in the areas of natural resources and land use inventory, geology and hydrology, and environmental quality. Specific results include a study of land use in the Harrisburg area, discrimination between types of forest resources and vegetation, detection of previously unknown geologic faults and correlation of these with known mineral deposits and ground water, mapping of mine spoils in the anthracite region of eastern Pennsylvania, and mapping of strip mines and acid mine drainage in central Pennsylvania. Both photointerpretive techniques and automatic computer processing methods have been developed and used, separately and in a combined approach.

The use of ERTS-1 satellite data in Great Lakes mesometeorological studies

NASA Technical Reports Server (NTRS)

Lyons, W. A. (Principal Investigator)

1972-01-01

The author has identified the following significant results. In the original proposal, it was hoped that ERTS could, with its extremely high resolution and multispectral capability, detect many meteorological phenomena occurring at the low end of the mesoscale motion spectrum (1 - 100 km). This included convective cloud phenomena, internal wave patterns, air pollution, snow squalls, etc. For meteorologists, ERTS-1 has more than lived up to initial hopes. First-look inspection of images has produced a large number of truly remarkable finds. Some of the most significant are: (1) Images of Lake Ontario during late summer have revealed several extremely good examples of lake breeze frontal cloud patterns. (2) Detection of suspended particulates from Chicago-Gary industrial complex in the 50,000 to 150,000 tons/year category. (3) Inadvertant weather modification due to anthropogenic condensation and ice nuclei from urban areas.

A technique for correcting ERTS data for solar and atmospheric effects

NASA Technical Reports Server (NTRS)

Rogers, R. H.; Peacock, K.; Shah, N. J.

1974-01-01

A technique is described by which ERTS investigators can obtain and utilize solar and atmospheric parameters to transform spacecraft radiance measurements to absolute target reflectance signatures. A radiant power measuring instrument (RPMI) and its use in determining atmospheric paramaters needed for ground truth are discussed. The procedures used and results achieved in processing ERTS CCTs to correct for atmospheric parameters to obtain imagery are reviewed. Examples are given which demonstrate the nature and magnitude of atmospheric effects on computer classification programs.

Preliminary evaluation of the 15 October 1972 ERTS-1 imagery of east central Ohio (scene 1034-15415)

NASA Technical Reports Server (NTRS)

Pettyjohn, W. A. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Results of a general, physical interpretation of ERTS-1 imagery of east central Ohio are presented. Special emphasis is placed upon geologic features, such as linear features and hydrologic features. Man-made features are included as a matter of interest and image location. The interpretation is compared to available maps of the area and from this an assessment that ERTS-1 is potentially useful for updating and producing geological maps.

The use of ERTS imagery in reservoir management and operation

NASA Technical Reports Server (NTRS)

Cooper, S. (Principal Investigator)

1973-01-01

There are no author-identified significant results in this report. Preliminary analysis of ERTS-1 imagery suggests that the configuration and areal coverage of surface waters, as well as other hydrologically related terrain features, may be obtained from ERTS-1 imagery to an extent that would be useful. Computer-oriented pattern recognition techniques are being developed to help automate the identification and analysis of hydrologic features. Considerable man-machine interaction is required while training the computer for these tasks.

Regional geology subprogram: Geological interpretation of ERTS imagery of the occidental region of Bolivia

NASA Technical Reports Server (NTRS)

Brockmann, C. E. (Principal Investigator); Ayllon, R. B.

1973-01-01

The author has identified the following significant results. Using ERTS-1 imagery, it is possible to delimit great lithological units, folds, lineaments, faults, and in lesser degree unconformities. In the morphological aspect, the images show clearly the relief necessary for geological interpretation. The ERTS-1 images are important for the preparation of the geological and tectonic map of Bolivia, on a 1:1 million scale, if conventional methods of work are used as a base.

Evaluation of commercial utility of ERTS-A imagery in structural reconnaissance for minerals and petroleum

NASA Technical Reports Server (NTRS)

Saunders, D. F. (Principal Investigator)

1973-01-01

The author has identified the following significant results. The only area that has been analyzed to date is Area 3 where results have already been reported. However, work progressing in Area 1 and 2 seem to indicate a good correlation between lineament zones previously reported, mineralized areas and lineaments currently being picked from ERTS-1 imagery. There also appear to be many lineaments on ERTS-1 imagery in these areas which have not been reported in any other literature.

Analysis of state of vehicular scars on Arctic Tundra, Alaska

NASA Technical Reports Server (NTRS)

Lathram, E. H.

1974-01-01

Identification on ERTS images of severe vehicular scars in the northern Alaska tundra suggests that, if such scars are of an intensity or have spread to a dimension such that they can be resolved by ERTS sensors (20 meters), they can be identified and their state monitored by the use of ERTS images. Field review of the state of vehicular scars in the Umiat area indicates that all are revegetating at varying rates and are approaching a stable state.

An evaluation of the utility of ERTS-1 data for mapping and developing natural resources of Iran

NASA Technical Reports Server (NTRS)

Ebtehadj, K. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Significant results are reported in the creation of an Iranian photomosaic from ERTS-1 imagery; in tectonic and structural mapping and interpretation, including discovery of significant new fault patterns in Iran; in river and lake mapping; in wetlands and fisheries nursery delineation and mapping; in range and agricultural surveys and inventories using multi-stage sample methods; and in the computer analysis of ERTS-1 digital tapes for urban land use.

Mineral resources, geologic structure, and landform surveys

NASA Technical Reports Server (NTRS)

Lattman, L. H.

1973-01-01

The use of ERTS-1 imagery for mineral resources, geologic structure, and landform surveys is discussed. Four categories of ERTS imagery application are defined and explained. The types of information obtained by the various multispectral band scanners are analyzed. Samples of land use maps and tectoning and metallogenic models are developed. It is stated that the most striking features visible on ERTS imagery are regional lineaments, or linear patterns in the topography, which reflect major fracture zones extending upward from the basement of the earth.

Precision annotation of predetermined primary sampling units on ERTS-1 MSS images

NASA Technical Reports Server (NTRS)

Vanroessel, J. W.; Langley, P. G.

1973-01-01

Resectioning programs were developed for projecting the boundary corners of sample units, management units, and counties into U2 RC-10 and ERTS-1 MSS images. The technique used includes corrections for earth curvature, terrain elevation, and MSS distortions. The minimum standard error obtained was about 0.15 mm or 150 meters on the ground. This technique now makes it possible to include land ownership as an integral part of forest resource sampling plans using ERTS imagery.

Diazo Printing of ERTS Color Composites

NASA Technical Reports Server (NTRS)

Mcmurtry, G. J.; Petersen, G. W. (Principal Investigator); Kowalik, W. S.

1975-01-01

The author has identified the following significant results. ERTS-1 color composites were made with the help of a Diazo developer and printer. Five single channel, density standards were established, using typical ERTS images, in order to determine exposure time. These standards were used to develop a graph from which the exposure time for any transparency can be estimated. Exposure times varied from 3 to 30 minutes, and clear colored polyester sheets from two manufactures were used with slightly different, but equally successful, results.

Characterizing Uncertainty In Electrical Resistivity Tomography Images Due To Subzero Temperature Variability

NASA Astrophysics Data System (ADS)

Herring, T.; Cey, E. E.; Pidlisecky, A.

2017-12-01

Time-lapse electrical resistivity tomography (ERT) is used to image changes in subsurface electrical conductivity (EC), e.g. due to a saline contaminant plume. Temperature variation also produces an EC response, which interferes with the signal of interest. Temperature compensation requires the temperature distribution and the relationship between EC and temperature, but this relationship at subzero temperatures is not well defined. The goal of this study is to examine how uncertainty in the subzero EC/temperature relationship manifests in temperature corrected ERT images, especially with respect to relevant plume parameters (location, contaminant mass, etc.). First, a lab experiment was performed to determine the EC of fine-grained glass beads over a range of temperatures (-20° to 20° C) and saturations. The measured EC/temperature relationship was then used to add temperature effects to a hypothetical EC model of a conductive plume. Forward simulations yielded synthetic field data to which temperature corrections were applied. Varying the temperature/EC relationship used in the temperature correction and comparing the temperature corrected ERT results to the synthetic model enabled a quantitative analysis of the error of plume parameters associated with temperature variability. Modeling possible scenarios in this way helps to establish the feasibility of different time-lapse ERT applications by quantifying the uncertainty associated with parameter(s) of interest.

Inventory of forest and rangeland and detection of forest stress. [Colorado and California

NASA Technical Reports Server (NTRS)

Heller, R. C.; Aldrich, R. C.; Weber, F. P.; Driscoll, R. S. (Principal Investigator)

1974-01-01

The author has identified the following significant results. Disturbances in a forest environment that cause reductions in forest area, timber volume, and timber growth can be detected on ERTS-1 combined color composites. However, detection depends on comparing a conventional aerial photograph taken at some base year with an ERTS-1 image taken in some subsequent year. In a test made on the Atlanta site, 1:63,360 scale aerial photo index sheets made in 1966 were compared with ERTS-1 image 1264-15445 (April 1973). Five factors were found important to detection reliability: (1) the quality of the imagery; (2) the season of the imagery; (3) the size of the disturbed area; (4) the number of years since the disturbances; and (5) the type of cutting treatment. Of 209 disturbances verified on aerial photography, 165 (or approximately 80%) were detected on the ERTS-1 image by one independent interpreter. Improved training and additional experience in using this low resolution imagery should improve detection. Of the two seasons of data studies (fall and early spring), early spring is the best for detecting land use changes. Generally speaking, winter, early spring, and early summer are the best times of year for detecting forest disturbances.

A reconnaissance space sensing investigation of crustal structure for a strip from the eastern Sierra Nevada to the Colorado Plateau

NASA Technical Reports Server (NTRS)

Bechtold, I. C. (Principal Investigator); Liggett, M. L.; Childs, J. F.

1973-01-01

There are no author-identified significant results in this report. Research progress in applications of ERTS-1 MSS imagery in study of Basin-Range tectonics is summarized. Field reconnaissance of ERTS-1 image anomalies has resulted in recognition of previously unreported fault zones and regional structural control of volcanic and plutonic activity. NIMBUS, Apollo 9, X-15, U-2, and SLAR imagery are discussed with specific applications, and methods of image enhancement and analysis employed in the research are summarized. Areas studied and methods employed in geologic field work are outlined.

A reconnaissance space sensing investigation of crustal structure for a strip from the eastern Sierra Nevada to the Colorado Plateau

NASA Technical Reports Server (NTRS)

Liggett, M. A.; Childs, J. F.

1973-01-01

The author has identified the following significant results. Research progress in applications of ERTS-1 MSS imagery to study of Basin-Range tectonics is summarized. Field reconnaissance of ERTS-1 image anomalies has resulted in recognition of previously unreported fault zones and regional structural control of volcanic and plutonic activity. Nimbus, Apollo 9, X-15, U-2, and SIAR imagery are discussed with specific applications, and methods of image enhancement and analysis employed in the research are summarized. Field areas studied and methods employed in geologic field work are outlined.

Study of the marine environment of the northern Gulf of California. [seasonal variations in oceanography

NASA Technical Reports Server (NTRS)

Hendrickson, J. R. (Principal Investigator)

1973-01-01

The author has identified the following significant results. Results of studies of the oceanography of the northern Gulf of California (Mexico) are reported. A remote, instrumented buoy measuring and telemetering oceanographic data by ERTS-1 satellite was designed, constructed, deployed, and tested. Regular cruises by a research ship on a pattern of 47 oceanographic stations collected data which are analyzed and referenced to analysis of ERTS-1 satellite imagery. A thermal dynamic model of current patterns in the northern Gulf of California is proposed. Findings are examined in relation to the model.

Nicotiana benthamiana α-galactosidase A1.1 can functionally complement human α-galactosidase A deficiency associated with Fabry disease.

PubMed

Kytidou, Kassiani; Beekwilder, Jules; Artola, Marta; van Meel, Eline; Wilbers, Ruud H P; Moolenaar, Geri F; Goosen, Nora; Ferraz, Maria J; Katzy, Rebecca; Voskamp, Patrick; Florea, Bogdan I; Hokke, Cornelis H; Overkleeft, Herman S; Schots, Arjen; Bosch, Dirk; Pannu, Navraj; Aerts, Johannes M F G

2018-04-19

α-Galactosidases (EC 3.2.1.22) are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. α-Galactosidases take part in the turnover of cell wall-associated galactomannans in plants and in the lysosomal degradation of glycosphingolipids in animals. Deficiency of human α-galactosidase A (α-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lysoGb3). Current management of FD involves enzyme-replacement therapy (ERT). An activity-based probe (ABP) covalently labeling the catalytic nucleophile of α-Gal A has been previously designed to study α-galactosidases for use in FD therapy. Here, we report that this ABP labels proteins in Nicotiana benthamiana leaf extracts, enabling the identification and biochemical characterization of an N. benthamiana α-galactosidase we name here A1.1 (gene accession GJZM-1660). The transiently overexpressed and purified enzyme was a monomer lacking N-glycans and was active toward 4-methylumbelliferyl-α-D-galactopyranoside substrate (Km = 0.17 mM) over a broad pH range. A1.1 structural analysis by X-ray crystallography revealed marked similarities with human α-Gal A, even including A1.1's ability to hydrolyze Gb3 and lysoGb3, which are not endogenous in plants. Of note, A1.1 uptake into FD fibroblasts reduced the elevated lysoGb3 levels in these cells, consistent with A1.1 delivery to lysosomes as revealed by confocal microscopy. The ease of production and the features of A1.1, such as stability over a broad pH range, combined with its capacity to degrade glycosphingolipid substrates, warrant further examination of its value as a potential therapeutic agent for ERT-based FD management. Published under license by The American Society for Biochemistry and Molecular Biology, Inc.

Clinical benefit in Fabry patients given enzyme replacement therapy--a case series.

PubMed

Guffon, N; Fouilhoux, A

2004-01-01

Fabry disease is a rare lysosomal storage disorder resulting from deficient activity of alpha-galactosidase A and subsequent pathological accumulation of glycosphingolipids throughout the body. Traditionally, Fabry disease was managed symptomatically, but the introduction of enzyme replacement therapies (ERTs) (agalsidase beta (Fabrazyme); agalsidase alfa (Replagal)) has transformed treatment of this disorder. Clinical studies of both compounds have demonstrated clearance of glycosphingolipds from key tissues. To explore whether substrate clearance translates into clinical benefit, a retrospective survey of 17 patients (mean age 34.7 years) treated with agalsidase beta (1 mg/kg every 2 weeks) was undertaken, using an eight-item retrospective questionnaire developed specifically to assess the effect of ERT on the symptoms of Fabry disease. Pain severity, heat tolerance, physical activity, fatigue and psychological status were scored using a 10-point visual analogue scale (e.g. for pain severity: 1=none, 10=strong). Answers to all other questions were quantitative. Changes in mean scores were 4.69 to 2.25 (p =0.012) for pain severity; 4.38 to 2.21 (p =0.019) for number of pain crises per month; 8.69 to 2.98 (p =0.097) for duration of pain crises in hours; 2.76 to 5.76 (p =0.002) for heat tolerance; 3.28 to 2.51 (p =0.058) for bowel movements per day; 2.47 to 4.47 (p =0.007) for frequency of physical activity; 5.53 to 3.71 (p =0.046) for fatigue, and 5.82 to 8.12 (p =0.005) for psychological status. All patients improved in at least one aspect, although the degree of improvement across patients and aspects varied widely; reasons for this remain unclear. Despite the inherent bias involved in retrospective questionnaires, we believe that the findings are encouraging. A prospective version of the questionnaire is currently under validation.

A quasi-randomized feasibility pilot study of specific treatments to improve emotion recognition and mental-state reasoning impairments in schizophrenia.

PubMed

Marsh, Pamela Jane; Polito, Vince; Singh, Subba; Coltheart, Max; Langdon, Robyn; Harris, Anthony W

2016-10-24

Impaired ability to make inferences about what another person might think or feel (i.e., social cognition impairment) is recognised as a core feature of schizophrenia and a key determinant of the poor social functioning that characterizes this illness. The development of treatments to target social cognitive impairments as a causal factor of impaired functioning in schizophrenia is of high priority. In this study, we investigated the acceptability, feasibility, and limited efficacy of 2 programs targeted at specific domains of social cognition in schizophrenia: "SoCog" Mental-State Reasoning Training (SoCog-MSRT) and "SoCog" Emotion Recognition Training (SoCog-ERT). Thirty-one participants with schizophrenia or schizoaffective disorder were allocated to either SoCog-MSRT (n = 19) or SoCog-ERT (n = 12). Treatment comprised 12 twice-weekly sessions for 6 weeks. Participants underwent assessments of social cognition, neurocognition and symptoms at baseline, post-training and 3-months after completing training. Attendance at training sessions was high with an average of 89.29 % attendance in the SoCog-MSRT groups and 85.42 % in the SoCog-ERT groups. Participants also reported the 2 programs as enjoyable and beneficial. Both SoCog-MSRT and SoCog-ERT groups showed increased scores on a false belief reasoning task and the Reading the Mind in the Eyes test. The SoCog-MSRT group also showed reduced personalising attributional biases in a small number of participants, while the SoCog-ERT group showed improved emotion recognition. The results are promising and support the feasibility and acceptability of the 2 SoCog programs as well as limited efficacy to improve social cognitive abilities in schizophrenia. There is also some evidence that skills for the recognition of basic facial expressions need specific training. Australian New Zealand Clinical Trials Registry ACTRN12613000978763 . Retrospectively registered 3/09/2013.

Electrical resisitivity of mechancially stablized earth wall backfill

NASA Astrophysics Data System (ADS)

Snapp, Michael; Tucker-Kulesza, Stacey; Koehn, Weston

2017-06-01

Mechanically stabilized earth (MSE) retaining walls utilized in transportation projects are typically backfilled with coarse aggregate. One of the current testing procedures to select backfill material for construction of MSE walls is the American Association of State Highway and Transportation Officials standard T 288: ;Standard Method of Test for Determining Minimum Laboratory Soil Resistivity.; T 288 is designed to test a soil sample's electrical resistivity which correlates to its corrosive potential. The test is run on soil material passing the No. 10 sieve and believed to be inappropriate for coarse aggregate. Therefore, researchers have proposed new methods to measure the electrical resistivity of coarse aggregate samples in the laboratory. There is a need to verify that the proposed methods yield results representative of the in situ conditions; however, no in situ measurement of the electrical resistivity of MSE wall backfill is established. Electrical resistivity tomography (ERT) provides a two-dimensional (2D) profile of the bulk resistivity of backfill material in situ. The objective of this study was to characterize bulk resistivity of in-place MSE wall backfill aggregate using ERT. Five MSE walls were tested via ERT to determine the bulk resistivity of the backfill. Three of the walls were reinforced with polymeric geogrid, one wall was reinforced with metallic strips, and one wall was a gravity retaining wall with no reinforcement. Variability of the measured resistivity distribution within the backfill may be a result of non-uniform particle sizes, thoroughness of compaction, and the presence of water. A quantitative post processing algorithm was developed to calculate mean bulk resistivity of in-situ backfill. Recommendations of the study were that the ERT data be used to verify proposed testing methods for coarse aggregate that are designed to yield data representative of in situ conditions. A preliminary analysis suggests that ERT may be utilized as construction quality assurance for thoroughness of compaction in MSE construction; however more data are needed at this time.

Monitoring an Induced Permafrost Warming Experiment Using ERT, Temperature, and NMR in Fairbanks, Alaska

NASA Astrophysics Data System (ADS)

Ulrich, C.; Ajo Franklin, J. B.; Ekblaw, I.; Lindsey, N.; Wagner, A. M.; Saari, S.; Daley, T. M.; Freifeld, B. M.

2016-12-01

As global temperatures continue to rise, permafrost landscapes will experience more rapid changes than other global climate zones. Permafrost thaw is a result of increased temperatures in arctic settings resulting in surface deformation and subsurface hydrology changes. From an engineering perspective, surface deformation poses a threat to the stability of existing infrastructure such as roads, utility piping, and building structures. Preemptively detecting or monitoring subsurface thaw dynamics presents a difficult challenge due to the long time scales as deformation occurs. Increased subsurface moisture content results from permafrost thaw of which electrical resistivity tomography (ERT), soil temperature, and nuclear magnetic resonance (NMR) are directly sensitive. In this experiment we evaluate spatial and temporal changes in subsurface permafrost conditions (moisture content and temperature) at a experimental heating plot in Fairbanks, AK. This study focuses on monitoring thaw signatures using multiple collocated electrical resistivity (ERT), borehole temperature, and borehole nuclear magnetic resonance (NMR) measurements. Timelapse ERT (sensitive to changes in moisture content) was inverted using collocated temperature and NMR to constrain ERT inversions. Subsurface thermal state was monitored with timelapse thermistors, sensitive to soil ice content. NMR was collected in multiple boreholes and is sensitive to changes in moisture content and pore scale distribution. As permafrost thaws more hydrogen, in the form of water, is available resulting in a changing NMR response. NMR requires the availability of liquid water in order to induce spin of the hydrogen molecule, hence, if frozen water molecules will be undetectable. In this study, the permafrost is poised close to 0oC and is mainly silt with small pore dimensions; this combination makes NMR particularly useful due to the possibility of sub-zero thaw conditions within the soil column. Overall this experiment presents a complementary suite of methods that provides feedback on subsurface permafrost state even in cases where soil texture might control unfrozen water content.

Thermal surveillance of Cascade Range volcanoes using ERTS-1 multispectral scanner, aircraft imaging systems, and ground-based data communication platforms

NASA Technical Reports Server (NTRS)

Friedman, J. D.; Frank, D. G.; Preble, D.; Painter, J. E.

1973-01-01

A combination of infrared images depicting areas of thermal emission and ground calibration points have proved to be particularly useful in plotting time-dependent changes in surface temperatures and radiance and in delimiting areas of predominantly convective heat flow to the earth's surface in the Cascade Range and on Surtsey Volcano, Iceland. In an integrated experiment group using ERTS-1 multispectral scanner (MSS) and aircraft infrared imaging systems in conjunction with multiple thermistor arrays, volcano surface temperatures are relayed daily to Washington via data communication platform (DCP) transmitters and ERTS-1. ERTS-1 MSS imagery has revealed curvilinear structures at Lassen, the full extent of which have not been previously mapped. Interestingly, the major surface thermal manifestations at Lassen are aligned along these structures, particularly in the Warner Valley.

KSC-2013-4388

NASA Image and Video Library

2013-12-13

CAPE CANAVERAL, Fla. – At a training location near Launch Complex 39 at NASA’s Kennedy Space Center in Florida, members of the Emergency Response Team, or ERT, participate in specialized training simulations in order to keep their skills current. They are wearing full protective gear and carrying non-lethal firearms, which are denoted in blue, for the training exercises. Often, ERT leadership serves as simulated suspects to add realism to the training exercises. Recently, eight members of the ERT competed in the 31st Annual SWAT Roundup International competition in Orlando, Fla., and received recognition by placing in the top five overall. In keeping with NASA’s commitment to safety and security of workforce and assets, the ERT is part of Kennedy’s security team and is trained to respond in the event of an emergency at the center. Photo credit: NASA/Daniel Casper

A comparison of Gemini and ERTS imagery obtained over southern Morocco

NASA Technical Reports Server (NTRS)

Blodget, H. W.; Anderson, A. T.

1973-01-01

A mosaic constructed from three ERTS MSS band 5 images enlarged to 1:500,000 compares favorably with a similar scale geologic map of southern Morocco, and a near-similar scale Gemini 5 photo pair. A comparative plot of lineations and generalized geology on the three formats show that a significantly greater number of probable fractures are visible on the ERTS imagery than on the Gemini photography, and that both orbital formats show several times more lineaments than were previously mapped. A plot of mineral occurrences on the structural overlays indicates that definite structure-mineralization relationships exist; this finding is used to define underdeveloped areas which are prospective for mineralization. More detailed mapping is possible using MSS imagery than on Gemini 5 photographs, and in addition, the ERTS format is not restricted to limited coverage.

ERTS and EROS

USGS Publications Warehouse

Staff, EROS Program

1972-01-01

In June the National Aeronautics & Space Administration is to launch its first experimental satellite designed to view the Earth systematically with remote-sensing instruments that will provide new information about our resources and environment. The launching will culminate more than 8 years of planning and research by resource agencies of the Federal Government in cooperation with NASA, state and local governments, universities, and industry. The first Earth Resources Technology Satellite, ERTS-A, will be followed a year later by ERTS-B. Analyses of data from them, it is hoped, will lead to design of operational satellites for Earth resources investigations in the future. In the belief that satellite systems will be of significant assistance in meeting its responsibilities to map, monitor, and manage the vast resources and the public lands of the United States, the Department of the Interior assumed a major role in the ERTS-A Experiment.