Clinical Issues in AIP:

Hepatic Porphyrias

The Heme Biosynthesis Pathway

AIP Autosomal Dominant Inheritance

AIP Most Common Porphyria, But a Rare Disorder

Case: 24-Year-Old Woman Presents to the ED Classic Clinical Presentation

AIP Classic Clinical Features

AIP Classic Clinical Features (cont)

Take the Patient's History

Physical Examination in a Patient With AIP

Case: 24-Year-Old Woman Presents to the ED (cont) Laboratory Evaluation

You Must Have a High Index of Suspicion

Laboratory Evaluation (cont)

Case: 24-Year-Old Woman Presents to the ED (cont)

A Common Precipitating Factor Hormones

Other Precipitating Factors or Triggers

Initial Management of an Acute Porphyric Attack

Specific Treatment of AIP Hemin

Specific Treatment of AIP Carbohydrate Loading

Variability of Severity of AIP

Consequences of Metabolic Alterations

Long-Term Management of a Complex Disorder With Multisystem Involvement

Preventing Acute Porphyric Attacks

Liver Transplantation in AIP

Emerging Therapies for Correction of Hepatic Heme Biosynthesis

Abbreviations