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CME: Acute hepatic porphyria: optimizing pharmacotherapeutic management strategies

Activity Description / Statement of Need:

In this online, self-learning activity:

Acute hepatic porphyria (AHP) is an umbrella term for four types of acute porphyria, the most severe of which is acute intermittent porphyria (AIP). An estimated 80% of AHP cases are AIP, which is an inherited autosomal dominant condition that results from mutations of the third enzyme of heme synthesis, porphobilinogen deaminase. In the Western countries, it is estimated that approximately 1 in 2000 individuals are carriers of the relevant mutated genotype, although the majority have latent AIP and are clinically asymptomatic. Acute attacks occur in less than 10% of the at-risk population, reflecting the role of environmental factors, such as alcohol use, infections, and hormonal changes, among others. AHP symptoms are believed to be caused by ALAS1-mediated accumulation of ALA and PBG in the liver and bloodstream, leading to neurotoxicity.

Target Audience:

The following HCPs: hematologists and gastroenterologists; physician assistants, nurse practitioners, and pharmacists who practice in any of the aforementioned areas of specialties; and any other healthcare professionals with an interest in or who clinically encounter patients with AHP.

Commercial Support Disclosure: This program is supported by an educational grant from Alnylam.

This activity is free of charge.

Release Date: July 06, 2023 -- Expiration Date: July 06, 2025

Faculty: Ashwani Singal, MD, MS


Faculty introduction, disclosures

Overview of AHP

  • AHP incidence and prevalence of recurrent attacks
  • AHP types
  • Burden of disease 
      • Pain—both acute and chronic
      • Long-term complications
      • Impact on quality of life
      • Economic costs
  • Diagnosing a challenging disease 
      • Signs and symptoms
      • “Purple flags”—when to suspect AHP
      • Abdominal pain differential diagnosis
      • Urine PGB and porphyrin testing
      • Other biochemical tests
      • Genetic tests
  • Patient cases

Treatment of AHP

·Primary attack and recurrent attacks

o   Role of heme pathway and ALAS1 in pathophysiology

o   Hemin therapy

o   When to discuss prophylactic treatments

o   Supportive care

o   Role of carbohydrate loading

· Prophylaxis

o   Patient counseling to avoid precipitating factors

o   Givosiran—first-in-class siRNA

- Indications

- Clinical trial data

- Administration and dosing

o   Hemin, iron overload, and long-term use

o   Liver transplant—when to refer

· Interspecialty collaboration

· Patient case(s)

Summary, conclusions, and best practice recap

Learning Objectives

By the end of the session the participant will be able to:

  • Integrate clinical knowledge of AHP presentation and common diagnostic mimics to identify patient cases with suspected AHP.
  • Evaluate the role of early diagnosis in the prevention of complications associated with AHP.
  • Compare pharmacologic strategies for acute and prophylactic management of AHP, including efficacy, safety, and mechanism of action.
  • Develop a comprehensive pharmacologic treatment plan for a patient presenting with recurrent AHP attacks.


ACCME Activity #202534018

This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education through ScientiaCME. ScientiaCME is accredited by the ACCME to provide continuing medical education for physicians.

Credit Designation: ScientiaCME designates this educational activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)™ toward the AMA Physician's Recognition Award. Physicians should only claim credit commensurate with the extent of their participation in the activity.


ABIM MOC Recognition Statement: Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.0 MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.

ABIM MOC Credit Type: Medical Knowledge

Physicians: For maintenance of certification (MOC) points, you must enter your board certification ID # and birth date correctly.  It is the learner's responsibility to provide this information completely and accurately at the completion of the activity. Without providing it, the learner will NOT receive MOC points for this activity. By providing this data, you acknowledge that it will be shared with ACCME and the applicable certifying board. Please note: Not all activities on this site provide MOC points. If this activity does not specify that it provides MOC points in this section, then it does NOT provide MOC points. This activity provides MOC points only for ABIM.


ScientiaCME is accredited by the Accreditation Council for Pharmacy Education (ACPE) as a provider of continuing pharmacy education. This activity is approved for 1.0 contact hours (0.1 CEUs) of continuing pharmacy education credit. Proof of participation will be posted to your NABP CPE profile within 4 to 6 weeks to participants who have successfully completed the post-test. Participants must participate in the entire presentation and complete the course evaluation to receive continuing pharmacy education credit. ACPE #0574-0000-23-025-H01-P. This is an Application (A) type activity. 

PharmacistsYou must enter your NABP # and birth date correctly so that proof of participation can be posted to your NABP CPE profile. It is the learner's responsibility to provide this information completely and accurately at the completion of the activity. Without providing it, the learner will NOT receive CPE credit for this activity.

Nurses: The American Nurses Credentialing Center (ANCC) accepts AMA PRA Category 1 Credit(s)™ from organizations accredited by the ACCME. This activity is designated for up to 1.0 AMA PRA Category 1 Credit(s)™.

Physician Assistants: The American Academy of Physician Assistants (AAPA) accepts AMA PRA Category 1 Credit™ assigned by organizations accredited by the ACCME as satisfying Category 1 CME for National Commission on Certification of Physician Assistants (NCCPA) national certification maintenance. This activity is designated for up to 1.0 AMA PRA Category 1 Credit(s)™.

Nurse Practitioners: The American Academy of Nurse Practitioners Certification Board (AANPCB) states that continuing education providers accredited by the ACCME may provide acceptable, accredited Advanced Practice Provider content. This activity is designated for up to 1.0 AMA PRA Category 1 Credit(s)™.

Faculty Disclosure and Resolution of COI

As a provider of continuing medical education, it is the policy of ScientiaCME to ensure balance, independence, objectivity, and scientific rigor in all of its educational activities. In accordance with this policy, faculty and educational planners must disclose any significant relationships with commercial interests whose products or devices may be mentioned in faculty presentations, and any relationships with the commercial supporter of the activity. The intent of this disclosure is to provide the intended audience with information on which they can make their own judgments. Additionally, in the event a conflict of interest (COI) does exist, it is the policy of ScientiaCME to ensure that the COI is resolved in order to ensure the integrity of the CME activity. For this CME activity, any COI has been resolved thru content review by ScientiaCME.

Disclosure of Faculty: Ashwani K. Singal, MD, MS, FACG, FAASLD, Professor of Medicine, University of SD Sanford School of Medicine, has received financial compensation from consulting and/or speakers bureau and/or research grants from NIAAA, NIDDK, ACG, Avera, APF, Expert Perspectives, CLD Foundation, GSK, Pleiogenix, Durect, GEN, Medscape, and GE; and has received royalties from Up-To-Date.

Disclosures of Educational Planners: Charles Turck, PharmD, BCPS, BCCCP, President of ScientiaCME, has no relevant financial disclosures.

Faculty will NOT discuss off-label uses.

All relevant financial relationships have been mitigated.

ScientiaCME adheres to the ACCME’s Standards for Integrity and Independence in Accredited Continuing Education. Any individuals in a position to control the content of a CE activity, including faculty, planners, reviewers or others are required to disclose all relevant financial relationships with ineligible entities (commercial interests). All relevant conflicts of interest have been mitigated prior to the commencement of the activity.

Commercial Support Disclosure: This program is supported by an educational grant from Alnylam.


  • Read the learning objectives above
  • Take the Pre-Test (optional). Completion of the pre-test will help us evaluate the knowledge gained by participating in this CME activity.
  • View the online activity. You may view this is in more than one session, and may pause or repeat any portion of the presentation if you need to.
  • Minimum participation threshold: Take the post-test. A score of 70% or higher is required to pass and proceed to the activity evaluation.
  • Complete the activity evaluation and CME registration. A CE certificate will be emailed to you immediately.

Cultural/Linguistic Competence & Health Disparities

System Requirements

Windows 7 or above
Internet Explorer 8
*Adobe Acrobat Reader
Mac OS 10.2.8
Safari or Chrome or Firefox
*Adobe Acrobat Reader
Internet Explorer is not supported on the Macintosh

*Required to view Printable PDF Version

Perform Pre-Test (optional)

Please take a few minutes to participate in the optional pre-test. It will help us measure the knowledge gained by participating in this activity.

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