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Clinical Issues in AIP:

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Presentation on theme: "Clinical Issues in AIP:"— Presentation transcript:

1 Clinical Issues in AIP:

2 Hepatic Porphyrias

3 The Heme Biosynthesis Pathway

4 AIP Autosomal Dominant Inheritance

5 AIP Most Common Porphyria, But a Rare Disorder

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

7 AIP Classic Clinical Features

8 AIP Classic Clinical Features (cont)

9 Take the Patient's History

10 Physical Examination in a Patient With AIP

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

12 You Must Have a High Index of Suspicion

13 Laboratory Evaluation (cont)

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

15 A Common Precipitating Factor Hormones

16 Other Precipitating Factors or Triggers

17 Initial Management of an Acute Porphyric Attack

18 Specific Treatment of AIP Hemin

19 Specific Treatment of AIP Carbohydrate Loading

20 Variability of Severity of AIP

21 Consequences of Metabolic Alterations

22 Long-Term Management of a Complex Disorder With Multisystem Involvement

23 Preventing Acute Porphyric Attacks

24 Liver Transplantation in AIP

25 Emerging Therapies for Correction of Hepatic Heme Biosynthesis

26 Abbreviations


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