Top Tweets for #ecneph
Follow the #ECneph hashtag to follow this case from beginning to end. And donβt forget to listen to the recordingπ
A suberb discussion by @anuja_java on the tricky subject that is #TMA
@anuja_java @ISNkidneycare @Dilushiwijay Thankyou for sharing your expertise and the wonderful insights regarding TMA #ECNeph
Had such a fruitful discussion today on the topic - TMA.
#ecneph.
So glad to have been able to attend the space session by @ISNeducation .
Thanks @ISNeducation for arranging a wonderful space session @myadla @anuja_java @Dilushiwijay @ISNkidneycare #TMA
This is not renal limited TMA . Favours systemic TMA presentation #ECNeph @anuja_java @Dilushiwijay @myadla
27/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Hope you had great learning!
Do check XSpace recording and continue conversation on X reg TMA !π
Most imp,share what you do in your centres π
Economics play a major role in management π°
Thankyou for joining us π
26/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Summing up our discussion
Lets check - TMA approach
Understanding pathogenesis
Interplay of genetics
and Triggers
Appropriate Drugs
Audits/Assessments
Affordability,accessibility
Quick recap of diverse aetiologies of TMA
Dont forget to Check the drug list /metabolic defects
Infographic by @krithicism on causes of TMA
@ISNkidneycare #ECNeph @Dilushiwijay
25/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Type /nature of mutation and Rx relevance
Do share your experience in management of such cases
24/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
How to approach a case of TMA ?
An algorithm for ease of practice
23/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
What are the salient points of interpretation of genetic testing ?
22/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Not ordering the test but interpretation of test is very crucial
Standard terminologies in reporting
What does it imply ?
21/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Why should genetic testing be done in TMA ?
20/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Similar case from Srilanka with 30 cycles of PLEX.. Pt had complete recovery
Sharing challenges in LMIC regarding rare diseases and Orphan drug availability
19/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Our Pt had renal limited TMA , persistence of TMA
Lets check what continued with investigations and Rx
IVMP +
PLEX :17 cycles
CFHR4 variant (pathogenic)
Given Eculizumab
Contd dialysis dependent
18/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Its very imp to know when to suspect CM-TMA ?
Kidney dominant
Persistence of TMA
Fam H/o
Recurrence
Normal ADAMTS 13
Insufficient trigger of TMA trajectory
17/n #ECNeph @ISNkidneycare @anuja_java @Dilushiwijay @myadla
Underlying genetics and superimposed triggers have a major role and intricate play for disease manifestation
15/n #ECNeph @ISNkidneycare @Dilushiwijay @myadla @anuja_java
Little more info about CFH/CFHR gene cluster
In India , many setups dont have support of genetic analysis /Mx strategies readily available
CoE for rare disease Mx are identified by NPRD
https://t.co/VYfxrojv5T
14/n #ECNeph @ISNkidneycare @Dilushiwijay @myadla @anuja_java
Lets look and understand what are we assessing in complement pathway? The Alternative pathway , Amplification loop ...
Whats feasibility in your settings to undertake these tests ?
13/n #ECNeph @ISNkidneycare @Dilushiwijay @myadla @anuja_java
Whats complement mediated TMA (CM-TMA)
Importance of and interpretation of genetics !
Changes in Management strategy
Not "one size fits all"
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