Management of Acute Hepatic Porphyrias

Acute Attack Management

The American Association for the Study of Liver Diseases recommends immediate discontinuation of porphyrinogenic triggers and administration of intravenous hemin and dextrose during acute attacks, and prophylactic therapy with hemin or givosiran for patients experiencing 4 or more attacks per year 1, 2
Stop all porphyrinogenic drugs and chemicals immediately upon suspicion of an acute attack, even before biochemical confirmation 1, 2, 3
Administer oral or intravenous dextrose (glucose) to suppress hepatic heme synthesis 1, 2
Initiate intravenous hemin therapy early in the acute phase, which is the treatment of choice for acute attacks 1, 2
Provide aggressive symptomatic management with analgesics for severe abdominal pain and antiemetics for nausea/vomiting 1, 2
Monitor and correct hyponatremia, which occurs in 25-60% of acute attacks 4, 5

Patients with 4 or more acute attacks per year should receive prophylactic therapy with either intravenous hemin or subcutaneous givosiran 1, 2, 3
Prophylactic hemin can be administered weekly or once-twice during the luteal phase for menstrual cycle-related attacks 7
The American Association for the Study of Liver Diseases classifies patients into four subgroups that determine management intensity 7, 5

Complete blood count and ferritin levels at baseline and yearly 4
Liver function tests (aminotransferases elevated in ~13% during attacks) 4
Metabolic panel including estimated glomerular filtration rate (eGFR) for renal function 4, 5
Screen for iron deficiency, which is common in young women and should be treated 4

Screen for hepatocellular carcinoma in all patients with confirmed acute hepatic porphyria, as they carry increased risk 1, 3, 5
Monitor for chronic kidney disease, typically chronic tubulointerstitial nephropathy or focal cortical atrophy 4, 5
Aggressively monitor and treat hypertension, which may help prevent renal damage 4, 5

Provide pre-conception evaluation for all women with acute hepatic porphyrias planning pregnancy 7, 5
Arrange high-risk obstetrical care during pregnancy, as hormonal changes increase attack risk 7, 5, 6
Carefully select contraceptives - progestin-containing contraceptives may trigger attacks and should be avoided 7, 5

Orthotopic liver transplantation is curative and should be considered for patients with intractable symptoms who have failed other treatment options 1, 2, 3, 7
Combined liver-kidney transplantation may benefit patients with both recurrent attacks and end-stage renal disease 7, 6