Adrenoleukodystrophy

Overview

4.5 out of 5 (93 Reviews)

Credits

1.50

Post Assessment Questions

8

Start Date

1 Sep 2023

Last Review Date

2 Aug 2024

Expiration Date

31 Aug 2026

Estimated Time To Finish

90 Minutes


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Activity Description

Adrenoleukodystrophy is a rare genetic peroxisomal disorder characterized by the abnormal metabolism of very long-chain fatty acids (VLCFAs) due to mutations in the ABCD1 gene. This leads to the accumulation of VLCFAs, particularly affecting the brain, spinal cord, adrenal glands, and testes. The condition is classified into various subtypes based on inheritance patterns, clinical presentation, age of onset, and affected organs. Diagnosis typically involves clinical suspicion prompted by neurological symptoms and biochemical testing showing elevated VLCFAs. Genetic confirmation through DNA analysis of ABCD1 gene mutations is crucial for a definitive diagnosis.

Management focuses on early detection through newborn screening programs in some regions, followed by regular monitoring of VLCFA levels. Symptoms typically include progressive neurological dysfunction and adrenal insufficiency, which is managed with glucocorticoid and mineralocorticoid replacement therapies. Hematopoietic stem cell transplantation (HSCT) remains the primary therapeutic option for early-stage adrenoleukodystrophy, offering the best chance to halt disease progression and improve outcomes. Despite these interventions, the prognosis is generally poor, with most patients developing severe neurological disabilities and a shortened lifespan. This activity reviews the pathophysiology, clinical manifestations, and genetic basis of adrenoleukodystrophy and provides clinicians with a comprehensive understanding of the condition. This activity also highlights the crucial role of the multidisciplinary healthcare team in recognizing the symptoms of progressive neurological dysfunction and adrenal insufficiency through early diagnosis and in becoming familiar with current management strategies. Additionally, this activity will offer clinicians insights into the therapeutic potential of HSCT for managing early-stage adrenoleukodystrophy.


Target Audience

This activity has been designed to meet the educational needs of physicians, physician associates, nurses, pharmacists, and nurse practitioners.

Learning Objectives

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

  • Identify early signs and symptoms of adrenoleukodystrophy to facilitate timely diagnosis and intervention. 

  • Implement appropriate diagnostic tests, including very long-chain fatty acids measurement and genetic analysis, to confirm adrenoleukodystrophy and guide treatment.

  • Apply current evidence-based guidelines for the management of adrenoleukodystrophy, including glucocorticoid and mineralocorticoid replacement therapies.

  • Collaborate with a multidisciplinary healthcare team, including neurologists, endocrinologists, and geneticists, to provide comprehensive care for adrenoleukodystrophy patients.

Disclosures

StatPearls, LLC requires everyone who influences the content of an educational activity to disclose relevant financial relationships with ineligible companies that have occurred within the past 24 months. Ineligible companies are organizations whose primary business is producing, marketing, selling, re-selling, or distributing healthcare products used by or on patients. All relevant conflict(s) of interest have been mitigated. Hover over contributor names for financial disclosures. Others involved in planning this educational activity have no relevant financial relationships to disclose.

Commercial Support: This activity has received NO commercial support.

Continuing Education Accreditation Information

In support of improving patient care, StatPearls, LLC is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC) to provide continuing education for the healthcare team.

 

Nurses: StatPearls designates this activity for 1.50 ANCC contact hour(s). Nurses should only claim credit commensurate with the extent of their participation in the activity.

Please consult your professional licensing board for information on the applicability and acceptance of continuing education credit for this activity.

Method of Participation and Credit

  1. Register for the activity.    
  2. Review the target audience, learning objectives, and disclosure information.
  3. Study the educational content of the enduring material.
  4. Choose the best answer to each activity test question. To receive credit and a certificate, you must pass the test questions with a minimum score of 100%.
  5. Complete the post-activity assessment survey.

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Disclaimer

This educational activity was planned and produced in accordance with the ACCME Standards for Integrity and Independence in Accredited Continuing Education. Faculty may discuss investigational products or off-label uses of products regulated by the US Food and Drug Administration. Readers should verify all information before employing any therapies described in this educational activity.

The information provided for this 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. The information presented does not necessarily reflect the views of StatPearls or any commercial supporters of educational activities on statpearls.com. StatPearls expressly disclaims responsibility for any adverse consequences resulting directly or indirectly from information in the course, for undetected error, or through a participant's misunderstanding of the content.

Unapproved Uses of Drugs/Devices: In accordance with FDA requirements, the audience is advised that information presented in this continuing education activity may contain references to unlabeled or unapproved uses of drugs or devices. Please refer to the FDA-approved package insert for each drug/device for full prescribing/utilization information.

Cancellation Policy: Please see the cancellation policy. StatPearls, LLC reserves the right to cancel any course due to unforeseen circumstances.

 

 
 

Reviews

Donna M. on 5/24/2021

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mode of inheritance of PEX genes not priovided

louis v. on 10/25/2023

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Brian M. on 12/20/2023

There is a sentence that does not make sense in one of the first parts of describing the natural history for a patient with X-linked ALD. The sentence has a duplicated phrase that reappears at the end of the sentence and does not make sense in that order in the sentence. For example, the phrase says something like "death, followed by" other complications of the illness.

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Unlimited Nurse CE

Stay up to date on the latest medical knowledge with 6631 CE activities. In these online self-assessment activities, read our reference articles and test your knowledge with more than 7875.5 hours of CE.

Single Activity

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Access to all the Unlimited Nurse CE activities in all specialties.

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