INT223091

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Context Info
Confidence 0.62
First Reported 2007
Last Reported 2010
Negated 3
Speculated 0
Reported most in Body
Documents 45
Total Number 45
Disease Relevance 17.90
Pain Relevance 1.56

This is a graph with borders and nodes. Maybe there is an Imagemap used so the nodes may be linking to some Pages.

Golgi apparatus (ELF3) nucleolus (ELF3) nucleus (ELF3)
extracellular matrix organization (ELF3) embryo development (ELF3) cytoplasm (ELF3)
Anatomy Link Frequency
platelet 3
urine 2
skin 2
plasma 1
autonomic 1
ELF3 (Homo sapiens)
Pain Link Frequency Relevance Heat
Pain 135 94.44 High High
Pain score 13 90.80 High High
qutenza 8 89.08 High High
cva 28 85.20 High High
Central nervous system 74 82.40 Quite High
Codeine 64 77.36 Quite High
Dermatone 6 77.12 Quite High
Neuropathic pain 22 71.28 Quite High
anesthesia 12 62.48 Quite High
peripheral neuropathy 19 43.60 Quite Low
Disease Link Frequency Relevance Heat
Hypertension 25 99.92 Very High Very High Very High
Proteinuria 633 99.84 Very High Very High Very High
Sensorineural Hearing Loss 25 99.82 Very High Very High Very High
Fabry Disease 733 99.80 Very High Very High Very High
Cough 205 99.28 Very High Very High Very High
Urticaria 5 99.16 Very High Very High Very High
Chronic Renal Failure 167 98.96 Very High Very High Very High
Angiokeratoma 49 98.84 Very High Very High Very High
Renal Disease 336 98.28 Very High Very High Very High
Disease 523 97.44 Very High Very High Very High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
Placebo-controlled trials of ERT
Gene_expression (trials) of ERT
1) Confidence 0.62 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.59 Pain Relevance 0.14
Randomized, placebo-controlled trials and long-term, open-label extension studies of both products have consistently demonstrated that ERT reduces GL-3 levels in plasma and urine as well as glycosphingolipids accumulation in capillary endothelial cells, renal glomerular cells, and tubular epithelial cells (Schiffmann et al 2001; Thurberg et al 2002), although the extent of clearance may be greater with the larger doses of ERT.
Gene_expression (reduces) of ERT in endothelial cells
2) Confidence 0.62 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.34 Pain Relevance 0
Endothelial GL-3 clearance and the effects of ERT
Gene_expression (effects) of ERT
3) Confidence 0.62 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.25 Pain Relevance 0
Open label studies of ERT dose and frequency of dosing
Gene_expression (dose) of ERT
4) Confidence 0.54 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.28 Pain Relevance 0
C) Are short-term changes in urinary GL-3 excretion of any utility is assessing response to ERT therapy?
Gene_expression (therapy) of ERT
5) Confidence 0.54 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.20 Pain Relevance 0.03
ERT alone will not reduce overt proteinuria in Fabry disease (Wilcox et al 2004; Banikazemi et al 2007; Germain et al 2007; Schiffmann 2007; Schiffmann et al 2007; Warnock 2007), but maintenance of urine protein excretion at ?
Neg (not) Gene_expression (reduce) of ERT in urine associated with proteinuria and fabry disease
6) Confidence 0.54 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 1.06 Pain Relevance 0
Four patients from the Fabry Outcomes Survey Registry have been described with similar degrees of proteinuria during ERT (222.8 ± 60 [SEM] mg/day) who progressed at ?
Gene_expression (progressed) of ERT associated with proteinuria
7) Confidence 0.54 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.78 Pain Relevance 0
During ERT, GL-3 levels were measured in plasma and urine every 3 months.


Gene_expression (measured) of ERT in urine
8) Confidence 0.50 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 0 Pain Relevance 0
Thus, ERT is expected to provide the greatest clinical benefit when initiated before permanent tissue damage has ensued and attention has been drawn to patients with Scheie syndrome whose disease may not be recognized.
Gene_expression (expected) of ERT associated with mucopolysaccharidosis i and disease
9) Confidence 0.43 Published 2010 Journal J Inherit Metab Dis Section Body Doc Link PMC2903709 Disease Relevance 1.03 Pain Relevance 0
In this report, corneal clouding, angiokeratoma, acroparesthesia, and sensorineural hearing loss did not change during ERT.
Neg (not) Gene_expression (change) of ERT associated with sensorineural hearing loss and angiokeratoma
10) Confidence 0.43 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 1.39 Pain Relevance 0.38
Urinary excretion of GL-3 has not been reduced except in patient 4, although his renal function has remained stable during ERT.
Gene_expression (stable) of ERT
11) Confidence 0.43 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 0.99 Pain Relevance 0.30
ERT was safe and effective in clearing of GL-3 and improvement of autonomic function in children with Fabry disease (29).
Gene_expression (effective) of ERT in autonomic associated with fabry disease
12) Confidence 0.43 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 0.67 Pain Relevance 0.13
But unlike previous studies, this study failed to show an acute improvement in skin-moisture in post-ERT Fabry patients who had received ERT over three years periodically.
Gene_expression (received) of ERT in skin
13) Confidence 0.39 Published 2008 Journal BMC Neurol Section Body Doc Link PMC2585087 Disease Relevance 0.17 Pain Relevance 0.04
But unlike previous studies, this study failed to show an acute improvement in skin-moisture in post-ERT Fabry patients who had received ERT over three years periodically.
Gene_expression (received) of ERT in skin
14) Confidence 0.39 Published 2008 Journal BMC Neurol Section Body Doc Link PMC2585087 Disease Relevance 0.17 Pain Relevance 0.04
The first ERT prescribed was alglucerase for 18 (29%) patients and imiglucerase for 44 (71%); ERT was started at a median of 14 (range, 0 to 61) years after diagnosis; median age (range) at ERT onset was 31.6 (4.4 to 65.9) years.
Gene_expression (prescribed) of ERT
15) Confidence 0.36 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.44 Pain Relevance 0
However, only the ferritin and platelet slopes differed significantly between before and during ERT (Table 3).
Gene_expression (during) of ERT in platelet
16) Confidence 0.36 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.05 Pain Relevance 0
During ERT, clinical abnormality rates decreased (except for neurological involvement) and the biological data improved overall during ERT (increased hemoglobin, leukocyte and platelet levels; decreased chitotriosidase, ACE, TRAP, ferritin and gammaglobulin levels).
Gene_expression (improved) of ERT in platelet
17) Confidence 0.36 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.26 Pain Relevance 0
For patients under ERT, a multivariate regression model including age at diagnosis and ferritin and platelet levels found only the baseline platelet count (P = 0.032) to have a significant impact.


Gene_expression (diagnosis) of ERT in platelet
18) Confidence 0.36 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.08 Pain Relevance 0
Approximately half the patients had urticaria during IDUA infusion, but in general the ERT was tolerated well.
Gene_expression (tolerated) of ERT associated with urticaria
19) Confidence 0.33 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727896 Disease Relevance 0.36 Pain Relevance 0.03
The median duration of ERT was 12 weeks before HCT and 7 weeks after HCT.
Gene_expression (duration) of ERT
20) Confidence 0.33 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727896 Disease Relevance 0.28 Pain Relevance 0.08

General Comments

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