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Acute Intermittent Porphyria

 Syn. AIP , Swedish porphyria , Pyrroporphyria, intermittent acute porphyria

Signs & Symptoms

 Abdominal pain
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]
Severe Abdominal pain
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]
Severe pain
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]
Steady pain
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]

Physical Examination

 Tachycardia
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]
Delirium
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
Disorientation
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]

Lab / Serology

 Decreased erythrocyte porphobilinogen deaminase
Note: Seen during asymptomatic period
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
anemia (negative symptom)
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
thrombocytopenia (negative symptom)
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]

Imagin Study

 Large bowel distension
Note: due to ileus
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
Small bowel distension
Note: due to ileus
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
Posterior reversible encephalopathy syndrome
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]

Complication

 Ileus
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis] Harrison [ 2990]
Hepatocellular carcinoma
Note: especially after age of 50
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
Impaired renal function
Ref: Harrison [ 2990]

Risk Factors

 Age between 20 and 40
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
20 < age < 40
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]
Female
Ref: Uptodate [ Acute intermittent porphyria: Pathogenesis, clinical features, and diagnosis]

Cause

Other