Urea Cycle Disorders

Overview

No Reviews

Credits

1.25

Post Assesment Questions

8

Release Date

5 Oct 2020

Expiration Date

11 Aug 2022

Last Reviewed

11 Aug 2021

Estimated Time To Finish

75 Minutes

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Activity Description

Urea cycle disorders are inborn errors of metabolism resulting from defects in one of the enzymes or transporter molecules involved in the hepatic removal of ammonia from the bloodstream. Removal of ammonia from the bloodstream normally occurs via its conversion to urea, which is then excreted by the kidneys. Consequently, urea cycle disorders lead to an accumulation of ammonia. Ammonia is extremely toxic, particularly to the central nervous system. Newborns with severe mutations in any one of the first four enzymes of the urea cycle can become catastrophically ill within 36 to 48 hours of birth despite appearing normal at birth. It is therefore possible for a newborn to be discharged from the hospital before signs of urea cycle disorders develop. The newborn may subsequently develop signs that go unrecognized at home. Hyperammonemia is key to the diagnosis of urea cycle disorders and treatment should not be delayed while a definitive diagnosis is sought. This activity reviews the evaluation and management of urea cycle disorders and highlights the role of interprofessional team members in collaborating to provide well-coordinated care and enhance outcomes for affected patients and families.

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Target Audience

This activity has been designed to meet the educational needs of physicians.

Learning Objectives

At the conclusion of this activity, the learner will be better able to:

  • Explain why the potential for urea cycle disorders poses a risk to early discharge of seemingly healthy newborns.
  • Explain how high serum ammonia level should be managed in a newborn.
  • Explain the management of urea cycle disorders.
  • Explain the importance of improving coordination amongst the interprofessional team to enhance care for patients affected by urea cycle disorders.

Author(s) / Contributors Disclosure of Conflicts of Interest

Each author, editor, faculty and planning committee member have completed a disclosure form indicating that neither they nor their spouse/partner has a financial interest/arrangement or affiliation that could be perceived as a real or apparent conflict of interest related to the content of this activity.

COMMERCIAL SUPPORT: This activity has received NO commercial support.

Contributors

Hover over the contributor names to see details and disclosures of any financial relationships or relationships they or their spouse/life partner have with commercial interests related to the content of this continuing education activity.

Continuing Education Accreditation Information


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 the Quillen College of Medicine, East Tennessee State University, and StatPearls, LLC. The Quillen College of Medicine, East Tennessee State University is accredited by the ACCME to provide continuing medical education for physicians.

Quillen College of Medicine, East Tennessee State University designates this activity for a maximum of 1.25 AMA PRA Category 1 CreditsTM. Physicians should only claim credit commensurate with the extent of their participation in the activity.

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  • StatPearls and ETSU reserve the right to cancel any course due to unforeseen circumstances. StatPearls and ETSU will not be responsible for other expenses incurred by the participant in the unlikely event that the program is canceled.

Equal Opportunity

  • StatPearls and ETSU are Equal Opportunity / Affirmative Action / Equal Access Institutions. 

Medium or Media Used:

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Instructions for Credit

  1. Register for the activity and create a StatPearls login.     
  2. Review the required accreditation information:  Target audience, learning objectives, and disclosure information.
  3. Complete the entire self-study activity.
  4. Complete the post-test assessments.
  5. Successfully pass the post-test with a minimum score of 100%.
  6. Complete the evaluation survey. 
  7. Obtain a certificate.

StatPearls and ETSU adhere to ACCME Standards regarding commercial support of continuing medical education. It is the policy of StatPearls and ETSU that the faculty and planning committee disclose real or apparent conflicts of interest relating to the topics of this educational activity, that relevant conflict(s) of interest are resolved, and also that authors and editors will disclose any unlabeled/unapproved use of drug(s) or device(s) during their presentation. Detailed disclosure will be made prior to starting the activity.

The information provided at this CME/CE activity is for continuing education purposes only and is not meant to substitute for the independent medical/clinical judgment of a healthcare provider relative to diagnostic and treatment options of a specific patient’s medical condition.

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  • Name: 'UCDs_organic.jpg' Attribution: Contributed by William Stone, MD

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