What are the guideline sources, guidelines and references for Acute hepatic porphyrias ?
Guideline sources The following summarized guidelines for the evaluation and management of acute hepatic porphyrias are prepared by our editorial team based on guidelines from the American Gastroenterological Association (AGA 2023). 1 Guidelines 1. Screening and diagnosis Indications for screening: consider screening for acute hepatic porphyrias in 15-50 years old female patients with unexplained, recurrent severe abdominal pain without a clear etiology after an initial workup. C 2. Diagnostic investigations Laboratory testing: obtain biochemical testing of D-ALA, porphobilinogen, and creatinine on a random urine sample for the initial diagnosis of acute hepatic porphyrias. B https://web.pathway.md/diseases/receyA41NfbuurNBL 1/3 6/23/23, 1:45 AM Acute hepatic porphyrias Pathway Genetic testing: obtain genetic testing to confirm the diagnosis of acute hepatic porphyrias in patients with positive biochemical testing. B 3. Medical management Intravenous hemin: administer IV hemin, given daily, preferably into a high-flow central vein, in patients with acute attacks of acute hepatic porphyrias severe enough to require hospital admission. B Supportive care: control pain, administer antiemetics, manage systemic arterial hypertension, tachycardia, hyponatremia, and hypomagnesemia, if present, in addition to IV hemin in patients with acute attacks of acute hepatic porphyrias. B 4. Surgical interventions Liver transplantation: reserve liver transplantation for patients with acute hepatic porphyrias with intractable symptoms and significantly decreased quality of life refractory to pharmacotherapy. B 5. Patient education General counseling: Advise avoiding identifiable triggers precipitating acute attacks, such as alcohol and porphyrinogenic medications. B Counsel patients about chronic and long-term complications of acute hepatic porphyrias, including neuropathy, CKD, hypertension, and HCC, and the need for long-term monitoring. B 6. Preventative measures Prophylactic hemin therapy: consider administering prophylactic heme therapy or givosiran, given in an outpatient setting, in patients with recurrent attacks ( 4 per year). C 7. Follow-up and surveillance Monitoring for liver disease: obtain annual monitoring for liver disease in patients with acute hepatic porphyrias. B Monitoring for chronic kidney disease: obtain annual surveillance for CKD with serum creatinine and estimated GFR in patients with acute hepatic porphyrias receiving treatment. B Surveillance for hepatocellular carcinoma: obtain surveillance for HCC with liver ultrasound every 6 months, starting at the age of 50 years, in patients with acute hepatic porphyrias, regardless of the severity of symptoms. B https://web.pathway.md/diseases/receyA41NfbuurNBL 2/3 6/23/23, 1:45 AM Acute hepatic porphyrias Pathway References 1. Bruce Wang, Herbert L Bonkovsky, Joseph K Lim et al. AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review. Gastroenterology. 2023 Jan 13;S0016 5085 22 01356 7. Open https://web.pathway.md/diseases/receyA41NfbuurNBL 3/3