This document summarizes a case discussion of a 10-year-old female patient presenting with acute liver failure and hemolysis who was potentially in need of a liver transplant. The patient had symptoms for 4 weeks and was initially evaluated at a nearby hospital before being referred for further evaluation. On examination, she had pallor, icterus, periorbital puffiness, distended abdomen, and enlarged liver and spleen. Investigations showed abnormal liver function tests and signs of hemolysis. Based on further testing, she was diagnosed with Wilson's disease. Due to developing grade 2 encephalopathy, she underwent living-related liver transplantation with her mother as the donor. She recovered well after the transplant.
Update on Fabry disease findings and the heart.
Update on cardiac predictors in Fabry disease.
Update on imaging in Fabry disease.
Update on comprehensive cardiovascular care in Fabry disease.
- Recorded videos of this lecture:
English Language version of this lecture is available at:
https://youtu.be/Zb6WISbvE2k
Arabic Language version of this lecture is available at:
https://youtu.be/4IvvrbC31Q4
- Visit our website for more lectures: www.NephroTube.com
- Subscribe to our YouTube channel: www.youtube.com/NephroTube
- Join our facebook group: www.facebook.com/groups/NephroTube
- Like our facebook page: www.facebook.com/NephroTube
- Follow us on twitter: www.twitter.com/NephroTube
Kidney transplantation, if not contraindicated, is the most preferred renal replacement therapy for patients with end stage renal disease. Generally, live related transplantation is associated with longer term survival of the transplantated kidney as well as the patient. However, it is associated with great physical and psychological challenges for the donor. Therefore, an exhaustive physical workup as well comprehensive psychological counselling go a long way for a happy donor as well as recipient. Laparoscopic donor surgery has helped reduce surgical morbidity and improve acceptance. Moreover, to avoid medicolegal issues, exhaustive documentation is necessary.
Update on Fabry disease findings and the heart.
Update on cardiac predictors in Fabry disease.
Update on imaging in Fabry disease.
Update on comprehensive cardiovascular care in Fabry disease.
- Recorded videos of this lecture:
English Language version of this lecture is available at:
https://youtu.be/Zb6WISbvE2k
Arabic Language version of this lecture is available at:
https://youtu.be/4IvvrbC31Q4
- Visit our website for more lectures: www.NephroTube.com
- Subscribe to our YouTube channel: www.youtube.com/NephroTube
- Join our facebook group: www.facebook.com/groups/NephroTube
- Like our facebook page: www.facebook.com/NephroTube
- Follow us on twitter: www.twitter.com/NephroTube
Kidney transplantation, if not contraindicated, is the most preferred renal replacement therapy for patients with end stage renal disease. Generally, live related transplantation is associated with longer term survival of the transplantated kidney as well as the patient. However, it is associated with great physical and psychological challenges for the donor. Therefore, an exhaustive physical workup as well comprehensive psychological counselling go a long way for a happy donor as well as recipient. Laparoscopic donor surgery has helped reduce surgical morbidity and improve acceptance. Moreover, to avoid medicolegal issues, exhaustive documentation is necessary.
A slide on Chronic kidney disease. At the beginning of the presentation is a case study, a patient admitted and treated for chronic kidney disease. Other parts covered include relevant anatomy and physiology, aetiopathogenesis and pathophysiology of the condition, as well as management and prevention.
Wilsons disease and hepatitis dr. abhamoni baroSanjeev Kumar
Case: Prolonged acute hepatitis – is there more to it?
Presenter: Abhamoni Baro
Moderator: Ashish Bavdekar
Panelists: Prakash Vaidya, Harshad Devarbhavi, Seema Alam
Hepatomegaly with seizures and hepatitis in a family dr. rajesh kumar meenaSanjeev Kumar
Case: Hepatomegaly with seizures and hepatitis in a family
Presenter: Rajesh Kumar Meena
Moderator: Vidyut Bhatia
Panelists: Seema Alam, Alka Jadhav, Anshu Srivastava
Acetabularia Information For Class 9 .docxvaibhavrinwa19
Acetabularia acetabulum is a single-celled green alga that in its vegetative state is morphologically differentiated into a basal rhizoid and an axially elongated stalk, which bears whorls of branching hairs. The single diploid nucleus resides in the rhizoid.
June 3, 2024 Anti-Semitism Letter Sent to MIT President Kornbluth and MIT Cor...Levi Shapiro
Letter from the Congress of the United States regarding Anti-Semitism sent June 3rd to MIT President Sally Kornbluth, MIT Corp Chair, Mark Gorenberg
Dear Dr. Kornbluth and Mr. Gorenberg,
The US House of Representatives is deeply concerned by ongoing and pervasive acts of antisemitic
harassment and intimidation at the Massachusetts Institute of Technology (MIT). Failing to act decisively to ensure a safe learning environment for all students would be a grave dereliction of your responsibilities as President of MIT and Chair of the MIT Corporation.
This Congress will not stand idly by and allow an environment hostile to Jewish students to persist. The House believes that your institution is in violation of Title VI of the Civil Rights Act, and the inability or
unwillingness to rectify this violation through action requires accountability.
Postsecondary education is a unique opportunity for students to learn and have their ideas and beliefs challenged. However, universities receiving hundreds of millions of federal funds annually have denied
students that opportunity and have been hijacked to become venues for the promotion of terrorism, antisemitic harassment and intimidation, unlawful encampments, and in some cases, assaults and riots.
The House of Representatives will not countenance the use of federal funds to indoctrinate students into hateful, antisemitic, anti-American supporters of terrorism. Investigations into campus antisemitism by the Committee on Education and the Workforce and the Committee on Ways and Means have been expanded into a Congress-wide probe across all relevant jurisdictions to address this national crisis. The undersigned Committees will conduct oversight into the use of federal funds at MIT and its learning environment under authorities granted to each Committee.
• The Committee on Education and the Workforce has been investigating your institution since December 7, 2023. The Committee has broad jurisdiction over postsecondary education, including its compliance with Title VI of the Civil Rights Act, campus safety concerns over disruptions to the learning environment, and the awarding of federal student aid under the Higher Education Act.
• The Committee on Oversight and Accountability is investigating the sources of funding and other support flowing to groups espousing pro-Hamas propaganda and engaged in antisemitic harassment and intimidation of students. The Committee on Oversight and Accountability is the principal oversight committee of the US House of Representatives and has broad authority to investigate “any matter” at “any time” under House Rule X.
• The Committee on Ways and Means has been investigating several universities since November 15, 2023, when the Committee held a hearing entitled From Ivory Towers to Dark Corners: Investigating the Nexus Between Antisemitism, Tax-Exempt Universities, and Terror Financing. The Committee followed the hearing with letters to those institutions on January 10, 202
How to Make a Field invisible in Odoo 17Celine George
It is possible to hide or invisible some fields in odoo. Commonly using “invisible” attribute in the field definition to invisible the fields. This slide will show how to make a field invisible in odoo 17.
Biological screening of herbal drugs: Introduction and Need for
Phyto-Pharmacological Screening, New Strategies for evaluating
Natural Products, In vitro evaluation techniques for Antioxidants, Antimicrobial and Anticancer drugs. In vivo evaluation techniques
for Anti-inflammatory, Antiulcer, Anticancer, Wound healing, Antidiabetic, Hepatoprotective, Cardio protective, Diuretics and
Antifertility, Toxicity studies as per OECD guidelines
Synthetic Fiber Construction in lab .pptxPavel ( NSTU)
Synthetic fiber production is a fascinating and complex field that blends chemistry, engineering, and environmental science. By understanding these aspects, students can gain a comprehensive view of synthetic fiber production, its impact on society and the environment, and the potential for future innovations. Synthetic fibers play a crucial role in modern society, impacting various aspects of daily life, industry, and the environment. ynthetic fibers are integral to modern life, offering a range of benefits from cost-effectiveness and versatility to innovative applications and performance characteristics. While they pose environmental challenges, ongoing research and development aim to create more sustainable and eco-friendly alternatives. Understanding the importance of synthetic fibers helps in appreciating their role in the economy, industry, and daily life, while also emphasizing the need for sustainable practices and innovation.
Operation “Blue Star” is the only event in the history of Independent India where the state went into war with its own people. Even after about 40 years it is not clear if it was culmination of states anger over people of the region, a political game of power or start of dictatorial chapter in the democratic setup.
The people of Punjab felt alienated from main stream due to denial of their just demands during a long democratic struggle since independence. As it happen all over the word, it led to militant struggle with great loss of lives of military, police and civilian personnel. Killing of Indira Gandhi and massacre of innocent Sikhs in Delhi and other India cities was also associated with this movement.
2024.06.01 Introducing a competency framework for languag learning materials ...Sandy Millin
http://sandymillin.wordpress.com/iateflwebinar2024
Published classroom materials form the basis of syllabuses, drive teacher professional development, and have a potentially huge influence on learners, teachers and education systems. All teachers also create their own materials, whether a few sentences on a blackboard, a highly-structured fully-realised online course, or anything in between. Despite this, the knowledge and skills needed to create effective language learning materials are rarely part of teacher training, and are mostly learnt by trial and error.
Knowledge and skills frameworks, generally called competency frameworks, for ELT teachers, trainers and managers have existed for a few years now. However, until I created one for my MA dissertation, there wasn’t one drawing together what we need to know and do to be able to effectively produce language learning materials.
This webinar will introduce you to my framework, highlighting the key competencies I identified from my research. It will also show how anybody involved in language teaching (any language, not just English!), teacher training, managing schools or developing language learning materials can benefit from using the framework.
Francesca Gottschalk - How can education support child empowerment.pptxEduSkills OECD
Francesca Gottschalk from the OECD’s Centre for Educational Research and Innovation presents at the Ask an Expert Webinar: How can education support child empowerment?
Palestine last event orientationfvgnh .pptxRaedMohamed3
An EFL lesson about the current events in Palestine. It is intended to be for intermediate students who wish to increase their listening skills through a short lesson in power point.
Read| The latest issue of The Challenger is here! We are thrilled to announce that our school paper has qualified for the NATIONAL SCHOOLS PRESS CONFERENCE (NSPC) 2024. Thank you for your unwavering support and trust. Dive into the stories that made us stand out!
1. Presenter : Ravi Bhardwaj
Moderator: Anupam Sibal
Panelists : BR Thapa, Harshad Devarbhavi,
RK Dhiman, Srinivas Sankaranarayanan
Case Discussion
Acute liver failure with hemolysis
– needing a transplant
2. Dr Ravi Bharadwaj
FNB Pediatric Gastroenterology
Apollo Center For Advanced Pediatrics
Indraprastha Apollo Hospital
3. Presenting complaints
10 year old
Female
Presented in June 2016 with c/o:
Poor appetite with nausea
Fatigability
Progressive abdominal distension
Symptoms for 4 weeks
Took medicines from nearby practitioner
4. 5th week of illness
Two episode of cola colored urine
painless
A day later parents noticed yellowish discoloration
of eyes
Decreased urine output
5. No documented fever
No diarrhea/abdominal pain/vomiting
No skin lesions/joint pain/joint swelling/chest pain
No dysuria/edema/pustules
No significant drug history
No bleeding from any site (skin, GI)
No seizures/alteration in sensorium/abnormal
movements/behavioural changes
7. Past History
No history of blood transfusion
No similar history in past
No history of prior admission for any illness
8. Developmentally normal
Vaccinated for age
Family history
One younger male sibling – 7 years, well
no history of similar illness in family
No h/o consanguinity
Dietary history: Calories 90 Cal/kg/day
Protein 1.5 g/kg/day
9. Admitted in nearby hospital
Evaluated and referred for further evaluation
10. On examination
RR: 26/min
Pulse: 96/min
Temp: 98.3 F
BP: 116/70mm Hg
SPO2: 96%
Weight: 25 kg (-1 to -2 SD)
Height: 128 cms ( -1 to -2 SD )
15. Investigations
Anti-HAV IgM and total NR
HBsAg NR
Anti-HCV NR
Ceruloplasmin 8 mg/dl
24 Hr Urinary Copper (without
challenge)
413 mcg/day
KF ring positive
ANA negative
16.
17.
18. Score 7 on WD criteria (Leipzig score) by Ferenci et al
Serum Cp <10 mg/dl +2
Urine Cu > 2 ULN +2
KF ring +2
Coomb’s negative hemolytic anemia +1
Wilson's disease ( score > 4)
19. Acute Liver Failure in WD
Modest rises in serum aminotransferases (<< 2000 IU/L)
Normal or markedly subnormal SAP
AST/ALT >2.2 and ALP/Bil <4
Coombs (–) hemolytic anemia and hemolysis
Rapid progression to renal failure
Korman J et al. Hepatology 2008
Ferenci et al. Aliment Pharmacol Ther 2004
Roberts et al. AASLD. Hepatology 2008
EASL. J Hepatol 2012
26. Score Bilirubin
ɥmol/L
INR AST
IU/L
WCC
x 109/L
Albumin
g/L
0 0-100 0-1.29 0-100 0-6.7 >45
1 101-150 1.3-1.6 101-150 6.8-8.3 34-44
2 151-200 1.7-1.9 151-300 8.4-10.3 25-33
3 201-300 2.0-2.4 301-400 10.4-15.3 21-24
4 >301 >2.5 >401 >15.4 <20
Modified King’s score (New Wilson Index)
A score ≥ 11 urgent need for transplantation
Our patient had a score of 16
Dhawan et al. Liver Transpl 2005
27.
28. Multivariate analysis
Unadjusted hazard 95% Confidence P
Ratio Interval Value
Enc 2.88 1.11 – 7.45 .03
T Bil 1.05 1.02 – 1.09 .002
Only encepaholopaty and total bilirubin emerged as
independent predictors of mortality
29. Devarbhavi H. J Gastro Hepatol 2014
Score = 2.87 x encephalopathy + 1.07 x t bilirubin
30. ALF with encephalopathy
High mortality 80% (90% to 100% in some series)
Liver transplantation is lifesaving
Berman et al. Gastroenterology 1991
Roberts et al. AASLD. Hepatology 2008
EASL. J Hepatol 2012
Devarbhavi H et al. J Gastro Hepatol 2014
34. Sibling evaluation
First-degree relatives of any patient newly diagnosed
with WD must be screened
Chance of a sibling being a homozygote and therefore
developing clinical disease – is 25%
Analysis of the ATP7B gene for mutations in the
children
Roberts et al. AASLD. Hepatology 2008
EASL. J Hepatol 2012
35. POD # 1
post LT on POD#1
sensorium improved over the next 72 hours
39. Neuro Wilson
Less common in children
< 10 years old: neuro-psychiatric disorders 17%
Average age of neurological dysfunction is 18.9 years
In adults neurological dysfunction constitutes initial
clinical manifestation in 40–60%
Pfeiffer et al. Semin Neurol. 2007
40. LT in treatment of progressive neurological
deterioration is controversial
Pfeiffer et al. Semin Neurol. 2007
42. Post LT outcome
Indication N/% Survival
@1 year
5 year 10 year 15 year
EHBA 66.1% 91.3 89.5 86.9 84.8
ALF 72.6 69 67 67
WD: Japan 2.6% 98.3 96.5 94.4 73.4
UNOS 90 89
SPLIT 96 91.4
France 89% 87% 87% 87%
Arnon et al. Clin Transplant. 2011
Kasahara et al. Am J of Transpl 2013
Guillaud et al. J Hepatol. 2014
43. LT experience
Pediatric 220
BA 81
Metabolic liver diseases 54
Cryptogenic 34
ALF 19
BCS 08
NNH 06
AIH 03
Hep B 03
Hyper oxaluria 02*
Poisoning 02
Hepatoblastoma 02
PVT 01**
Hep C 01
HCC 01
Chronic rejection 01
* combined LK
** re transplant
