INT150476

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Context Info
Confidence 0.56
First Reported 2007
Last Reported 2010
Negated 1
Speculated 0
Reported most in Body
Documents 32
Total Number 32
Disease Relevance 13.61
Pain Relevance 2.39

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 5
ERt 2
plasma 1
leukocyte 1
lung 1
ELF3 (Homo sapiens)
Pain Link Frequency Relevance Heat
Morphine 8 99.20 Very High Very High Very High
Paracetamol 4 97.12 Very High Very High Very High
Codeine 128 94.48 High High
anesthesia 8 92.88 High High
Pain 130 89.76 High High
qutenza 16 89.08 High High
Central nervous system 48 85.40 High High
tolerance 14 85.36 High High
Inflammatory marker 2 81.32 Quite High
corticosteroid 3 80.36 Quite High
Disease Link Frequency Relevance Heat
Cough 409 100.00 Very High Very High Very High
Proteinuria 454 99.86 Very High Very High Very High
Renal Disease 260 99.60 Very High Very High Very High
Gauchers Disease 403 99.52 Very High Very High Very High
Apoptosis 8 99.38 Very High Very High Very High
Disease 378 99.04 Very High Very High Very High
Fabry Disease 590 98.52 Very High Very High Very High
Neurologic Manifestations 12 96.68 Very High Very High Very High
Chronic Renal Failure 129 96.32 Very High Very High Very High
Coronary Heart Disease 45 93.52 High High

Sentences Mentioned In

Key: Protein Mutation Event Anatomy Negation Speculation Pain term Disease term
There could be another form of kidney disease superimposed upon Fabry nephropathy that obviously would not respond to increased ERT, and in addition, if the biopsy demonstrates complete clearance of glomerular capillary endothelial GL-3 deposits, such as in Figure 7, then there does not appear to be any current justification for using a higher or more frequent dose of ERT.
Neg (not) Positive_regulation (increased) of ERT in kidney associated with renal disease
1) Confidence 0.56 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.46 Pain Relevance 0
CONCLUSION: If early intervention with ERT in females is to be advocated, it is necessary to demonstrate not only that females with Anderson-Fabry disease have clinical and biochemical features of alpha-galactosidase A deficiency and respond to ERT, but also that early intervention prevents the onset of the later manifestations of the disorder.
Positive_regulation (respond) of ERT associated with fabry disease
2) Confidence 0.45 Published 2008 Journal Acta Paediatr Suppl Section Abstract Doc Link 18339187 Disease Relevance 0.39 Pain Relevance 0.09
Despite these limitations of the phase IV study, Schiffmann concluded (Schiffmann 2007) that “…hemizygous male patients with the classic form of Fabry disease at possibly any age and symptomatic patients with milder variants should receive ERT with the particular goal to preserve renal function”.


Positive_regulation (receive) of ERT associated with fabry disease
3) Confidence 0.40 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.63 Pain Relevance 0.03
Rather than being a criticism to the effectiveness of ERT in Fabry nephropathy, it should be recognized that this limitation exists for all forms of CKD, emphasizing the importance of surveillance, early detection and close monitoring of burden of disease and progression of organ involvement as important contributors to the ultimate success of treating these diseases.


Positive_regulation (effectiveness) of ERT associated with chronic renal failure, renal disease and disease
4) Confidence 0.40 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.85 Pain Relevance 0
If there is any subsequent indication of decreasing eGFR, or increasing proteinuria, then increasing the dose of ERT to 1 mg/kg every other week could be considered.
Positive_regulation (increasing) of ERT associated with proteinuria
5) Confidence 0.40 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0.21 Pain Relevance 0.04
Splenectomized patients' platelet counts rose slightly under ERT, with no significant slope change, while nonsplenectomized patients' counts increased significantly from baseline under ERT but returned to normal after 6.5 years of treatment.
Positive_regulation (increased) of ERT in platelet
6) Confidence 0.37 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.06 Pain Relevance 0
In vitro studies in mice with sera from anti-agalsidase antibody-positive patients (Ohashi et al 2008; Vedder et al 2008). and in vivo studies in patients (Vedder et al 2008) suggest that the potential neutralizing effects of anti-agalsidase antibodies may be overcome by increasing the dose or ERT from 0.2 to 1.0 mg/kg.
Positive_regulation (increasing) of ERT
7) Confidence 0.37 Published 2008 Journal Biologics : Targets & Therapy Section Body Doc Link PMC2727881 Disease Relevance 0 Pain Relevance 0
In summary, this study demonstrates that the clinical effects of ERT were associated with a significant reduction in the serum and urine GL-3 levels, and clearance of the accumulated GL-3 from the vascular endothelium of biopsied kidney.
Positive_regulation (effects) of ERT in urine
8) Confidence 0.32 Published 2008 Journal Journal of Korean Medical Science Section Body Doc Link PMC2526436 Disease Relevance 0.29 Pain Relevance 0.18
During ERT, platelet counts increased (+7,035 platelets/year), while all other biomarkers declined.
Positive_regulation (increased) of ERT in platelets
9) Confidence 0.27 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.30 Pain Relevance 0.08
Before ERT, significantly more BE occurred in patients diagnosed with GD after 15 years of age (HR, 2.6 (95% CI, 1.0 to 6.7); P = 0.048), but no significant differences were found for the other covariates, and none had an effect during ERT.
Positive_regulation (effect) of ERT associated with gauchers disease
10) Confidence 0.27 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.10 Pain Relevance 0
Estimated individual slopes of the biomarkers had no significant influence on developing BE before or during ERT but, at diagnosis, ferritin concentration (HR, 1.18 (95% CI, 1.03 to 1.35); P = 0.019) and platelet count (HR, 0.69 (95% CI, 0.49 to 0.98); P = 0.039) increased the risk of BE during ERT in this univariate model.
Positive_regulation (increased) of ERT in platelet
11) Confidence 0.27 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.09 Pain Relevance 0
Platelet counts in nonsplenectomized patients were stable before ERT (+190 platelets/year), while chitotriosidase and TRAP decreased slightly, and ferritin and ACE increased slightly.
Positive_regulation (stable) of ERT in Platelet
12) Confidence 0.27 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.33 Pain Relevance 0.09
Data were censored if no BE occurred before the patient started ERT for the first analysis and until the closing date for the second analysis.
Positive_regulation (started) of ERT
13) Confidence 0.27 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.09 Pain Relevance 0
Overall, 21 (34%) patients were splenectomized: 5 before diagnosis, 16 between diagnosis and ERT onset; none were splenectomized after starting ERT.
Positive_regulation (starting) of ERT
14) Confidence 0.27 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.25 Pain Relevance 0
Although the authors did not specifically identify ERt in their paper, inspection of May and Widdicombe [33] data suggests that morphine inhibited cough more than mechanically induced ERt.
Positive_regulation (induced) of ERt associated with cough and morphine
15) Confidence 0.26 Published 2008 Journal Cough Section Body Doc Link PMC2405785 Disease Relevance 0.87 Pain Relevance 0.52
The proportion of ERt to coughs was increased in anesthetized cats compared with awake animals [15].
Positive_regulation (increased) of ERt associated with cough
16) Confidence 0.26 Published 2008 Journal Cough Section Body Doc Link PMC2405785 Disease Relevance 0.78 Pain Relevance 0.45
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).
Positive_regulation (increased) of ERT in platelet
17) Confidence 0.25 Published 2010 Journal Arthritis Res Ther Section Body Doc Link PMC2945057 Disease Relevance 0.26 Pain Relevance 0
Noteworthy, this upregulation did not change after institution of ERT, whereas apoptosis inducing factor appears to be upregulated under ERT.
Positive_regulation (upregulated) of ERT associated with apoptosis
18) Confidence 0.23 Published 2009 Journal Orphanet J Rare Dis Section Body Doc Link PMC2768700 Disease Relevance 1.19 Pain Relevance 0.07
These changes were observed in an early stage of the disease, and the authors speculated on its value as an indicator for ERT efficacy.
Positive_regulation (indicator) of ERT associated with disease
19) Confidence 0.23 Published 2009 Journal Orphanet J Rare Dis Section Body Doc Link PMC2768700 Disease Relevance 0.94 Pain Relevance 0
Early response to ERT
Positive_regulation (response) of ERT
20) Confidence 0.22 Published 2007 Journal Eur J Pediatr Section Body Doc Link PMC2234442 Disease Relevance 0.05 Pain Relevance 0.04

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