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Clinical Issues in AIP:
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Hepatic Porphyrias
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The Heme Biosynthesis Pathway
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AIP Autosomal Dominant Inheritance
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AIP Most Common Porphyria, But a Rare Disorder
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Case: 24-Year-Old Woman Presents to the ED Classic Clinical Presentation
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AIP Classic Clinical Features
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AIP Classic Clinical Features (cont)
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Take the Patient's History
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Physical Examination in a Patient With AIP
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Case: 24-Year-Old Woman Presents to the ED (cont) Laboratory Evaluation
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You Must Have a High Index of Suspicion
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Laboratory Evaluation (cont)
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Case: 24-Year-Old Woman Presents to the ED (cont)
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A Common Precipitating Factor Hormones
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Other Precipitating Factors or Triggers
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Initial Management of an Acute Porphyric Attack
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Specific Treatment of AIP Hemin
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Specific Treatment of AIP Carbohydrate Loading
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Variability of Severity of AIP
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Consequences of Metabolic Alterations
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Long-Term Management of a Complex Disorder With Multisystem Involvement
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Preventing Acute Porphyric Attacks
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Liver Transplantation in AIP
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Emerging Therapies for Correction of Hepatic Heme Biosynthesis
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Abbreviations
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