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ALA Dehydratase Deficiency Porphyria Follow-up

  • Author: Smeeta Sinha, MD; Chief Editor: Emmanuel C Besa, MD  more...
Updated: Mar 26, 2015

Further Inpatient Care

See the list below:

  • Some common medications that are known to be safe for use in ALAD deficiency porphyria (ADP) include the following: aspirin, acetaminophen, aminoglycosides, benzodiazepines, penicillin and its derivatives, ibuprofen, insulin, phenothiazines, glucocorticoids, atropine, beta-blockers, ranitidine, tetracyclines, thyroxine, gabapentin, and vigabatrin.
  • Avoid drugs that induce the cytochrome P-450 system.
  • Avoid exogenous estrogen and progesteronal agents.


See the list below:

  • The mainstay of ALAD deficiency porphyria (ADP) outpatient therapy is to prevent future acute attacks.
    • Patients should be counseled to avoid exposure to inducing medications (unsafe medications, as discussed in Further Inpatient Care) and other exogenous substances, such as styrene, lead, trichloroethylene, and bromobenzene.
    • Fasting should be avoided.
    • Smoking, an inducer of the cytochrome P-450 system, may increase ALAD deficiency porphyria (ADP) attacks and should also be avoided.


See the list below:

  • The 7 reported patients with ALAD deficiency porphyria (ADP) had markedly differing clinical courses.
  • Neurovisceral attacks can recur throughout adulthood in otherwise healthy individuals.
  • A Swedish boy who had experienced severe ALAD deficiency porphyria (ADP) attacks refractory to treatment since birth underwent liver transplantation at age 6. A modest improvement in symptoms was noted, but the child died from pneumonia at age 9.
  • A patient who presented with late-onset porphyria died from a comorbid hematologic malignancy.

Patient Education

See the list below:

  • Patients with ALAD deficiency porphyria (ADP) should be informed about the triggers of acute ALAD deficiency porphyria (ADP), as well as safe medications (as discussed in Further Inpatient Care).
  • The American Porphyria Foundation Website contains lists of safe and unsafe medications and other pertinent information for patients with porphyria diseases (
Contributor Information and Disclosures

Smeeta Sinha, MD Resident Physician, Department of Dermatology, Rutgers New Jersey Medical School

Smeeta Sinha, MD is a member of the following medical societies: Alpha Omega Alpha, Phi Beta Kappa, Sigma Xi

Disclosure: Nothing to disclose.


Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Pere Gascon, MD, PhD Professor and Director, Division of Medical Oncology, Institute of Hematology and Medical Oncology, IDIBAPS, University of Barcelona Faculty of Medicine, Spain

Pere Gascon, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, New York Academy of Medicine, New York Academy of Sciences, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Thomas H Davis, MD, FACP Associate Professor, Fellowship Program Director, Department of Internal Medicine, Section of Hematology/Oncology, Geisel School of Medicine at Dartmouth

Thomas H Davis, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Education, American College of Physicians, New Hampshire Medical Society, Phi Beta Kappa, Society of University Urologists

Disclosure: Nothing to disclose.


Mark J Shumate, MD, MPH Assistant Professor, Department of Internal Medicine, Division of Hematology/Oncology, Emory University School of Medicine

Mark J Shumate, MD, MPH is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

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