Metabolism 101: Tutorial 4 - Emergency management of metabolic disease

For the non-metabolic | taught by Georgianne Arnold
  • 48 Videos
  • 5 Quizzes
  • 5 Surveys
  • 3 Texts
  • 1 PDF
  • 1.3 hrs

Course Curriculum

Tutorial 4 introduction and objectives
00:54
Tutorial 4 introduction and objectives
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Pre-course assessment
Pre-course assessment questions 1 and 2 chemical reaction
Pre-course assessment
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Chapter 1: The metabolic crisis: a review
01:48
Clinical signs/biochemical features
Components of an acute crisis
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Chapter 2: Reducing toxicity from accumulating compounds by decreasing toxin production
11:51
Methylmalonic acidemia
Decreasing toxin production
Application of principals to treatment
Use of dextrose
Take home point #1
Enzyme cofactors
Take home point #2
Chapter 2 example question
Chapter 2 example question - interactive
Chapter 2 example answer
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Chapter 3: Reducing toxicity from accumulating compounds by removing the toxin
08:20
Direct removal of toxin
Shunt toxin into alternative pathway
Take home point #3
Chapter 3 case study
Chapter 3 case study - interactive
Chapter 3 case study analysis
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Chapter 4: Problems caused by product deficiency
06:00
L-arginine and urea cycle disorders
Biotinidase deficiency
Long chain fatty acid oxidation defects
nAGS deficiency
Take home point #4 and chapter summary
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Chapter 5: Overall energy deficiency
06:36
Primary defects in energy production
Treatment of primary defects in energy production
Indirect defects of energy deficiency
Take home point #5 and chapter summary
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Chapter 6: Deleterious impact from acidosis, hypoglycemia, hypperammonemia
02:48
Acidosis
Hypoglycemia
Hyperammonemia
Tutorial 4 Chapter 6 Take Home 6 and Summary
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Chapter 7: Impact from triggers of the crisis
01:34
Common triggers of the crisis and treatment
Take home point #7 and chapter summary
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Chapter 8: Iatrogenic worsening of the crisis
03:14
Summary, causes and apparent treatment failure
Take home point #8 and caution
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Chapter 9: Application of treatment principles to case studies
03:06
Managing the patient
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Chapter 10: Case study 1
08:11
Case study 1: scenario
Case study 1 question - interactive
Case study 1 - answer and analysis
Case study 1 - writing orders
Case study 1 - writing orders exercise
Case study 1 - writing orders analysis
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Chapter 10: Case study 2
07:20
Case study 2 - scenario
Case study 2 question - interactive
Case study 2 - answer and analysis
Case study 2 - writing orders exercise
Case study 2 - writing orders analysis
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Chapter 10: Case study 3
04:59
Case study 3 - scenario
Case study 3 question - interactive
Case study 3 - answer and analysis
Case study 3 - writing orders exercise
Case study 3 - writing orders analysis
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Chapter 11: Tutorial 4 Summary
01:48
Tutorial 4 summary
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Patient-as-teacher video
04:18
Introduction to video
Tasia: VLCAD patient
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Essential slides for download
Tutorial 4 essential slides for download
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Post lecture quiz
Post-course quiz questions 1 and 2 chemical reaction
Post-course quiz
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eLearning course survey
Course survey link
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Course description

Course Title:

Metabolism 101: Emergency Management of Metabolic Disease

Program Overview:

The final tutorial in the “Metabolism 101” series reviews the physiologic and biochemical causes of a metabolic crisis since these also project opportunities for treatment. These include the toxicity from accumulating compounds, a deficiency of products, an overall deficiency of energy, associated metabolic disturbances, the role of the factors that trigger a crisis, and the morbidity and potential mortality that can occur because of iatrogenic complications. With this background, treatment strategies are proposed and the users are provided a number of cases for which to develop sample orders.

Faculty:

Georgianne Arnold, MD - Associate Medical Director - Genetic Metabolic Center for Education


Credit Hours:

1.0 AMA PRA Category 1 Credit™


Important Dates:

Valid Until: 12/01/2019
Date Released: 12/01/2016
Date Updated: 12/01/2016


Fee Statement:

This activity if FREE to all participants


Commercial Support:

This enduring internet activity has no commercial support


Learning Objectives:

1.Identify the various components of a metabolic crisis, including:

a.Toxicity from accumulating compounds

b.Product deficiency

c.Energy deficiency

d.Secondary metabolic disturbances

e.Trigger(s) of the crisis

f.Iatrogenic complications

2.Describe the treatment strategies for each component

3.Apply these treatment principles to case studies



Accreditation Statement:

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Nemours and the Genetic Metabolic Center for Education. Nemours is accredited by the ACCME to provide continuing medical education for physicians.

Nemours designates this internet enduring activity for a maximum number of 1.0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in this activity.


Disclosure Statement:

The information on this website is provided exclusively for educational purposes. Information about medicine is ever changing. As new research and clinical experience broadens knowledge, changes in treatment and procedures will be necessary. Nemours has consulted with sources believed to be reliable in order to provide information that is comprehensive and in accord with standards of care at the initial time of publication. However, Nemours disclaims any duty to update the information and encourages readers to do so. Nemours makes no warranties, either implied or expressed, that the information contained herein is accurate or complete. Nemours is not responsible for any errors or omissions with respect to the information or for the results obtained from the use of the information. Readers are encouraged to confirm the information with other sources.


Faculty Disclosure:

The listed speaker has no real or apparent conflict of interest to disclose.


Planning Committee Disclosure:

The members of the planning committee have no real or apparent conflict of interest to disclose.


Instruction for Completion:

To receive credit for this unit, view the video files and complete all survey questions. It will take approximately 60 minutes to complete. When you have finished, complete the post-test. Upon scoring at least 70% on the post-test and completion of the evaluation you will be able download your CME/CE certificate.

Clinicians who are not physicians will be provided a certificate of completion to submit for credit in accordance with their state nursing board, specialty society, or other professional association.


Additional Resources:

Schwartz IV, de Souza CF, Giugliani R. Treatment of inborn errors of metabolism. J Pediatr (Rio J). 2008;84(4 Suppl):S8-19.
The Online Metabolic and Molecular Bases of Inherited Disease, Scriver et al, eds. McGraw-Hill
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Instructor

Georgianne  Arnold
Georgianne Arnold
MD, Associate Medical Director

Dr. Georgianne Arnold is Associate Medical Director of the Genetic Metabolic Center for Education and is active in both the consultative servicesand educational programs.

Dr. Arnold previously served as Professor of Pediatrics as well as Clinical Director of the Division of Medical Genetics (2010 – 2016) at the University of Pittsburgh Medical Center. She brings 25 years of experience in the diagnosis and care of patients with inborn errors of metabolism. She has extensive experience in teaching including students, residents and faculty, and was a charter faculty member at the North American Metabolic Academy (the premier training conference for genetic trainees on this continent).

Dr. Arnold has published more than 50 peer reviewed articles and book chapters, has presented papers at national and international meetings, and was awarded the Shapira Award in 2010 for “A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency.” She is sought after as a teacher with a gift for making metabolism understandable for all audiences.

Dr. Arnold graduated from SUNY Upstate Medical University and and completed pediatric residency at Children’s Memorial Hospital at Nothwestern University. She completed her fellowship in genetics and metabolism in 1991 at the University of Colorado. She was Associate Professor of Pediatrics at the University of Rochester School of Medicine and Dentistry until 2010 where she directed care for patients with metabolic and genetic disorders.

Reviews (3)

by maryam bawazir

Very useful course!!!

by Tinatin Tkemaladze

OK

by Michael Schneider
No clear guidelines for hyperammonemic dialysis (coma versus NH3 level) Urine ketones may be lagging indicator of metabolic response in organic academia Role of MRI, saline bolus in organic acidemias unclear

by maryam bawazir

Very useful course!!!

by Tinatin Tkemaladze

OK

by Michael Schneider
No clear guidelines for hyperammonemic dialysis (coma versus NH3 level) Urine ketones may be lagging indicator of metabolic response in organic academia Role of MRI, saline bolus in organic acidemias unclear