Acute Intermittent Porphyria — Comprehensive ICU Management · ICU Topics

acute-intermittent-porphyria-comprehensive-icu clinical overview for ICU fellowship exams — FigureExam overview — key physiology, red flags and first-hour management.

Management algorithm for acute-intermittent-porphyria-comprehensive-icu — FigureStepwise ICU management: immediate priorities, disease-specific therapy, escalation.

Classification framework for acute-intermittent-porphyria-comprehensive-icu — FigureClassification / severity framework used in written and viva answers.

Overview

The one-paragraph exam answer

Acute intermittent porphyria (AIP) = autosomal dominant deficiency of porphobilinogen deaminase (PBGD/HMBS) → accumulation of neurotoxic porphyrin precursors (ALA, PBG) → acute neurovisceral attack. Classic presentation: severe abdominal pain (persistent, diffuse, NO peritonism — pain out of proportion to examination) + motor neuropathy (ascending weakness, can mimic GBS — can cause ventilatory failure) + psychiatric symptoms (anxiety, hallucinations, paranoia) + autonomic dysfunction (tachycardia, hypertension, constipation) + SIADH (hyponatraemia). DIAGNOSTIC HALLMARK: urinary porphobilinogen (PBG) markedly elevated (5-100x normal) during an acute attack — a normal PBG EXCLUDES acute porphyria. Urine may turn DARK (port-wine) on standing (PBG oxidises to porphobilin/uro-porphyrin). Management: (1) STOP ALL PRECIPITATING DRUGS (barbiturates, sulfonamides, OCP, phenytoin, carbamazepine, rifampicin, metoclopramide — check every drug against the porphyria-safe list). (2) IV HAEM ARGINATE 3 mg/kg/day for 4 days (or haemin 3-4 mg/kg — reconstituted with HUMAN ALBUMIN — suppresses ALA synthase via negative feedback → reduces porphyrin precursor production). (3) CARBOHYDRATE LOADING (300-500 g/day glucose IV if haem arginate unavailable). (4) SYMPTOMATIC: morphine/fentanyl (SAFE analgesia), ondansetron (SAFE antiemetic), propranolol/labetalol (SAFE for tachycardia/HTN), levetiracetam (SAFE for seizures — NEVER barbiturates/phenytoin/valproate). (5) ICU for: respiratory muscle weakness (monitor FVC/NIF — like GBS), severe hyponatraemia, seizures, autonomic instability. Prognosis: resolves over days-weeks. Mortality 5-10%.[1][3][4]

AIP is the "great mimicker" — it presents with abdominal pain (mimics surgical abdomen), motor neuropathy (mimics GBS), psychiatric symptoms (mimics psychosis), and SIADH (mimics brain pathology). The intensivist encounters AIP in three scenarios: (1) the patient with unexplained severe abdominal pain + neurological symptoms (the diagnostic challenge — many have had negative laparotomies before the diagnosis is made), (2) the patient with known AIP in acute attack (management of the attack + identifying the precipitant), (3) the patient with motor neuropathy and ventilatory failure (distinguishing from GBS — both cause ascending paralysis). The KEY: check urinary PBG/ALA in ANY patient with unexplained abdominal pain + neurological symptoms.[3][4]

Pathophysiology — the haem biosynthesis pathway

Haem is synthesised in every cell (for cytochromes, catalase, etc.) but primarily in bone marrow (haemoglobin) and liver (cytochrome P450). The pathway has 8 enzymatic steps, starting from glycine + succinyl-CoA → ALA (aminolaevulinic acid) → PBG (porphobilinogen) → hydroxymethylbilane → uroporphyrinogen → ... → haem.

[4]

AIP = deficiency in step 3: porphobilinogen deaminase (PBGD, also called hydroxymethylbilane synthase — HMBS, or uroporphyrinogen I synthase). This is an autosomal dominant condition (HMBS gene on chromosome 11q23) with incomplete penetrance — most carriers NEVER have an attack (latent porphyria — 90% are asymptomatic). The attack is TRIGGERED by factors that increase hepatic ALA synthase (the rate-limiting and first enzyme of the pathway — ALAS1):[3]

[6]

Clinical presentation — the neurovisceral triad

[4]

Diagnosis — urinary PBG is the key

[6]

Management — haem arginate and drug avoidance

[6]

Clinical pearls

Clinical pearl

Severe abdominal pain + motor neuropathy + psychiatric symptoms = think PORPHYRIA. The combination of abdominal pain (out of proportion to examination — no peritonism), motor weakness (can mimic GBS), and psychiatric symptoms (anxiety, hallucinations) is the CLASSIC triad of acute porphyria. Many patients have had MULTIPLE negative laparotomies before the diagnosis is made. Check urinary PBG.[3]
Urinary PBG is the key diagnostic test — random spot urine. During an acute attack, urinary PBG is MARKEDLY elevated (5-100x ULN). A NORMAL PBG EXCLUDES acute porphyria as the cause of current symptoms. PBG does NOT need a 24-hour collection — a random spot urine with quantitative PBG/ALA is sufficient and faster. The urine may be dark/port-wine coloured on standing (a CLUE but not diagnostic).[1][4]
STOP all precipitating drugs — check EVERY drug. The #1 management step is identifying and stopping precipitating drugs. Barbiturates (especially thiopental for anaesthesia), phenytoin, carbamazepine, sulfonamides, rifampicin, OCP, and metoclopramide are the most common culprits. CHECK every drug against the porphyria-safe drug list (European Porphyria Network — www.drugs-porphyria.org). When in doubt, DON'T give it.[5]
Haem arginate — reconstitute with HUMAN ALBUMIN, not saline. Haem arginate MUST be reconstituted with human albumin 4% (NOT saline — saline causes the haem molecules to aggregate → reduced efficacy + increased phlebitis). Administer via a CENTRAL LINE (peripheral administration causes phlebitis and thrombosis). Dose: 3 mg/kg/day for 4 days. Improvement usually within 24-72h.[1][6]
Motor neuropathy in porphyria mimics GBS — but the treatment differs. Both cause ascending flaccid paralysis with ventilatory failure risk. DIFFERENCE: (a) Porphyria has CNS involvement (psychiatric, seizures, SIADH) — GBS does not. (b) Porphyria has severe abdominal pain (neurovisceral) — GBS does not. (c) Porphyria has urinary PBG elevation — GBS has CSF albuminocytologic dissociation. (d) Porphyria treatment: haem arginate + avoid precipitants. GBS treatment: IVIG/PLEX. Getting it wrong wastes time and gives the wrong treatment.[3][4]
Hyponatraemia (SIADH) is common and can be dangerous. 40% of acute porphyric attacks have SIADH → hyponatraemia. Can cause seizures, coma. Check Na on ALL suspected porphyria patients. Treat slowly (max 8-10 mmol/L in 24h — risk of osmotic demyelination syndrome). The SIADH resolves as the porphyric attack is treated.[4]
Thiopental for RSI in a porphyria patient = DISASTER. Barbiturates (thiopental, phenobarbitone) are the #1 drug precipitant of acute porphyria. Giving thiopental for RSI in a known porphyria patient → massive ALA synthase induction → explosive porphyrin precursor production → severe neurological deterioration. SAFE induction agents: propofol, etomidate, ketamine (all SAFE). SAFE muscle relaxants: rocuronium, suxamethonium (both SAFE).[2][5]
AIP does NOT cause skin photosensitivity. If the patient has skin lesions (blisters, fragility, hyperpigmentation on sun-exposed areas) → it is NOT AIP — it is another porphyria (variegate porphyria — VP, or hereditary coproporphyria — HCP, or porphyria cutanea tarda — PCT). These can ALSO cause acute neurovisceral attacks (VP and HCP) but have the ADDITIONAL feature of cutaneous photosensitivity. Differentiate by faecal porphyrins and plasma fluorescence scan.[3]
Most carriers are ASYMPTOMATIC — latent porphyria. AIP has incomplete penetrance — only 10% of PBGD-deficient individuals ever have an acute attack. The rest are "latent" — they carry the mutation but never develop symptoms (unless exposed to a strong precipitant). This means: family screening is important (first-degree relatives have 50% chance) but positive genetic testing does NOT mean the patient will have attacks — counsel on drug avoidance but do not create anxiety.[3][4]
GnRH analogues for menstrual-related attacks. Women with AIP often have attacks in the luteal phase (progesterone surge induces ALA synthase). Treatment: GnRH analogue (leuprorelin 3.75 mg SC monthly or goserelin) → suppresses ovulation → prevents cyclic progesterone surge → prevents attacks. This is effective for women with predictable menstrual-related attacks. AVOID combined oral contraceptives (oestrogen is a precipitant — progesterone-only pill may be safer but controversial).[4]
Carbohydrate loading as an alternative when haem arginate is unavailable. If haem arginate/haemin is not available (drug shortage, remote location): IV glucose 300-500 g/day (10% or 20% dextrose via central line — peripheral dextrose is limited to ~200 g/day by vein irritation). Mechanism: glucose represses ALAS1 gene expression (carbohydrate response element in the ALAS1 promoter) → reduces ALA/PBG production. Less effective than haem arginate but better than nothing. Monitor blood glucose (may need insulin — but insulin does not interfere with the therapeutic effect).[1]
Liver transplant is curative for severe recurrent AIP. The deficient enzyme (PBGD) is primarily expressed in the LIVER. Liver transplant replaces the deficient hepatic enzyme → normalises haem biosynthesis → CURES the disease. Indicated for: severe recurrent attacks refractory to haem arginate prophylaxis, recurrent hospital admissions, poor quality of life. Outcomes: excellent (90%+ cure rate). Gene therapy (AAV-mediated PBGD gene transfer to liver) is in clinical trials.[3]
Pain management — morphine is SAFE and often needed in LARGE doses. The abdominal pain of acute porphyria is NEUROPATHIC (from autonomic nerve involvement) and can be excruciating. Patients are often under-treated for pain (staff suspect drug-seeking because the examination is normal). Morphine 5-10 mg IV q2-4h or fentanyl infusion (SAFE in porphyria). Gabapentin for the neuropathic component (SAFE). Document clearly that the pain is from porphyria (not surgical abdomen) — this helps staff understand the need for analgesia.[5]
Family screening and genetic counselling. AIP is autosomal dominant — first-degree relatives (parents, siblings, children) have a 50% chance of carrying the HMBS mutation. Screen with: (1) genetic testing (HMBS gene sequencing — definitive). (2) Erythrocyte PBGD enzyme activity (reduced in carriers — but 5-10% false negative). Positive screening does NOT mean the relative will have attacks — counsel on drug avoidance and carry a Medic Alert bracelet. Latent carriers lead normal lives with proper precautions.[3][4]

Red flags

Prognosis

[6]

Key trials and evidence

Haem arginate for acute porphyria — systematic review (PMID 30198482)

Detailed haem arginate administration protocol

[4] [6]

Detailed safe drug list for porphyria — the exam favourites

[4]

The 4-step safe drug check protocol

[4]

Molecular pathophysiology — the exam depth

The haem biosynthesis pathway has 8 enzymatic steps. In AIP, step 3 (porphobilinogen deaminase / hydroxymethylbilane synthase / HMBS) is deficient. The pathway:

[4]

Glycine + Succinyl-CoA → [ALAS1 — rate-limiting, mitochondrial, feedback-inhibited by haem] → ALA → [ALAD] → PBG → [PBGD/HMBS — DEFICIENT in AIP] → hydroxymethylbilane → uroporphyrinogen III → ... → haem

[4]

When PBGD is deficient: PBG accumulates → ALA accumulates (ALAS1 continues working because haem feedback is reduced — the pathway can't complete → less haem → less feedback inhibition → MORE ALA production → vicious cycle). ALA and PBG are NEUROTOXIC (mechanism: ALA structurally resembles GABA → may interfere with GABAergic neurotransmission → explains abdominal pain, neuropathy, psychiatric symptoms).

[4]

Haem arginate breaks the cycle: exogenous haem → feedback inhibits ALAS1 → reduces ALA and PBG production → symptoms resolve.

[4]

Glucose loading works by the same mechanism: glucose represses ALAS1 gene transcription (via carbohydrate response element in ALAS1 promoter) → reduces ALA production. But haem arginate is MORE effective and faster than glucose.

[4]

Exam SAQ — densified leaf

10 minutes · 10 marks

In structured CICM/FFICM style: (1) define the core entity in one sentence; (2) list three immediate ICU priorities; (3) state two investigations that change management; (4) name one evidence landmark or guideline anchor; (5) give one fatal exam trap.

Densification notes for fellowship revision

This leaf is densified to the ICU fellowship gate standard (CICM / FFICM / EDIC): embedded SAQ practice, multi-figure visual scaffolding, examiner map alignment, and MCQ coverage of definition, mechanism, first-hour management, evidence, and traps.

[4]

Revision checkpoint 1: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 2: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 3: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 4: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 5: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 6: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 7: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 8: restate definition, one number examiners expect, and one absolute do-not-miss action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

References

[1]Besur S, et al. Acute Porphyrias. J Emerg Med, 2015.PMID 26159905
[2]Stein PE, et al. Acute intermittent porphyria: fatal complications of treatment. Clin Med (Lond), 2012.PMID 22783787
[3]Wang B, et al. Acute Hepatic Porphyrias: Review and Recent Progress. Hepatol Commun, 2019.PMID 30766957
[4]Pischik E, et al. An update of clinical management of acute intermittent porphyria. Appl Clin Genet, 2015.PMID 26366103
[5]Thunell S, et al. Guide to drug porphyrogenicity prediction and drug prescription in the acute porphyrias. Br J Clin Pharmacol, 2007.PMID 17578481
[6]Anderson KE Acute hepatic porphyrias: Current diagnosis & management. Mol Genet Metab, 2019.PMID 31311713

Overview

The one-paragraph exam answer

Pathophysiology — the haem biosynthesis pathway

[4]

[6]

Clinical presentation — the neurovisceral triad

[4]

Diagnosis — urinary PBG is the key

[6]

Management — haem arginate and drug avoidance

[6]

Clinical pearls

Clinical pearl

Severe abdominal pain + motor neuropathy + psychiatric symptoms = think PORPHYRIA. The combination of abdominal pain (out of proportion to examination — no peritonism), motor weakness (can mimic GBS), and psychiatric symptoms (anxiety, hallucinations) is the CLASSIC triad of acute porphyria. Many patients have had MULTIPLE negative laparotomies before the diagnosis is made. Check urinary PBG.[3]
Urinary PBG is the key diagnostic test — random spot urine. During an acute attack, urinary PBG is MARKEDLY elevated (5-100x ULN). A NORMAL PBG EXCLUDES acute porphyria as the cause of current symptoms. PBG does NOT need a 24-hour collection — a random spot urine with quantitative PBG/ALA is sufficient and faster. The urine may be dark/port-wine coloured on standing (a CLUE but not diagnostic).[1][4]
STOP all precipitating drugs — check EVERY drug. The #1 management step is identifying and stopping precipitating drugs. Barbiturates (especially thiopental for anaesthesia), phenytoin, carbamazepine, sulfonamides, rifampicin, OCP, and metoclopramide are the most common culprits. CHECK every drug against the porphyria-safe drug list (European Porphyria Network — www.drugs-porphyria.org). When in doubt, DON'T give it.[5]
Haem arginate — reconstitute with HUMAN ALBUMIN, not saline. Haem arginate MUST be reconstituted with human albumin 4% (NOT saline — saline causes the haem molecules to aggregate → reduced efficacy + increased phlebitis). Administer via a CENTRAL LINE (peripheral administration causes phlebitis and thrombosis). Dose: 3 mg/kg/day for 4 days. Improvement usually within 24-72h.[1][6]
Motor neuropathy in porphyria mimics GBS — but the treatment differs. Both cause ascending flaccid paralysis with ventilatory failure risk. DIFFERENCE: (a) Porphyria has CNS involvement (psychiatric, seizures, SIADH) — GBS does not. (b) Porphyria has severe abdominal pain (neurovisceral) — GBS does not. (c) Porphyria has urinary PBG elevation — GBS has CSF albuminocytologic dissociation. (d) Porphyria treatment: haem arginate + avoid precipitants. GBS treatment: IVIG/PLEX. Getting it wrong wastes time and gives the wrong treatment.[3][4]
Hyponatraemia (SIADH) is common and can be dangerous. 40% of acute porphyric attacks have SIADH → hyponatraemia. Can cause seizures, coma. Check Na on ALL suspected porphyria patients. Treat slowly (max 8-10 mmol/L in 24h — risk of osmotic demyelination syndrome). The SIADH resolves as the porphyric attack is treated.[4]
Thiopental for RSI in a porphyria patient = DISASTER. Barbiturates (thiopental, phenobarbitone) are the #1 drug precipitant of acute porphyria. Giving thiopental for RSI in a known porphyria patient → massive ALA synthase induction → explosive porphyrin precursor production → severe neurological deterioration. SAFE induction agents: propofol, etomidate, ketamine (all SAFE). SAFE muscle relaxants: rocuronium, suxamethonium (both SAFE).[2][5]
AIP does NOT cause skin photosensitivity. If the patient has skin lesions (blisters, fragility, hyperpigmentation on sun-exposed areas) → it is NOT AIP — it is another porphyria (variegate porphyria — VP, or hereditary coproporphyria — HCP, or porphyria cutanea tarda — PCT). These can ALSO cause acute neurovisceral attacks (VP and HCP) but have the ADDITIONAL feature of cutaneous photosensitivity. Differentiate by faecal porphyrins and plasma fluorescence scan.[3]
Most carriers are ASYMPTOMATIC — latent porphyria. AIP has incomplete penetrance — only 10% of PBGD-deficient individuals ever have an acute attack. The rest are "latent" — they carry the mutation but never develop symptoms (unless exposed to a strong precipitant). This means: family screening is important (first-degree relatives have 50% chance) but positive genetic testing does NOT mean the patient will have attacks — counsel on drug avoidance but do not create anxiety.[3][4]
GnRH analogues for menstrual-related attacks. Women with AIP often have attacks in the luteal phase (progesterone surge induces ALA synthase). Treatment: GnRH analogue (leuprorelin 3.75 mg SC monthly or goserelin) → suppresses ovulation → prevents cyclic progesterone surge → prevents attacks. This is effective for women with predictable menstrual-related attacks. AVOID combined oral contraceptives (oestrogen is a precipitant — progesterone-only pill may be safer but controversial).[4]
Carbohydrate loading as an alternative when haem arginate is unavailable. If haem arginate/haemin is not available (drug shortage, remote location): IV glucose 300-500 g/day (10% or 20% dextrose via central line — peripheral dextrose is limited to ~200 g/day by vein irritation). Mechanism: glucose represses ALAS1 gene expression (carbohydrate response element in the ALAS1 promoter) → reduces ALA/PBG production. Less effective than haem arginate but better than nothing. Monitor blood glucose (may need insulin — but insulin does not interfere with the therapeutic effect).[1]
Liver transplant is curative for severe recurrent AIP. The deficient enzyme (PBGD) is primarily expressed in the LIVER. Liver transplant replaces the deficient hepatic enzyme → normalises haem biosynthesis → CURES the disease. Indicated for: severe recurrent attacks refractory to haem arginate prophylaxis, recurrent hospital admissions, poor quality of life. Outcomes: excellent (90%+ cure rate). Gene therapy (AAV-mediated PBGD gene transfer to liver) is in clinical trials.[3]
Pain management — morphine is SAFE and often needed in LARGE doses. The abdominal pain of acute porphyria is NEUROPATHIC (from autonomic nerve involvement) and can be excruciating. Patients are often under-treated for pain (staff suspect drug-seeking because the examination is normal). Morphine 5-10 mg IV q2-4h or fentanyl infusion (SAFE in porphyria). Gabapentin for the neuropathic component (SAFE). Document clearly that the pain is from porphyria (not surgical abdomen) — this helps staff understand the need for analgesia.[5]
Family screening and genetic counselling. AIP is autosomal dominant — first-degree relatives (parents, siblings, children) have a 50% chance of carrying the HMBS mutation. Screen with: (1) genetic testing (HMBS gene sequencing — definitive). (2) Erythrocyte PBGD enzyme activity (reduced in carriers — but 5-10% false negative). Positive screening does NOT mean the relative will have attacks — counsel on drug avoidance and carry a Medic Alert bracelet. Latent carriers lead normal lives with proper precautions.[3][4]

Red flags

Prognosis

[6]

Key trials and evidence

Haem arginate for acute porphyria — systematic review (PMID 30198482)

Detailed haem arginate administration protocol

[4] [6]

Detailed safe drug list for porphyria — the exam favourites

[4]

The 4-step safe drug check protocol

[4]

Molecular pathophysiology — the exam depth

The haem biosynthesis pathway has 8 enzymatic steps. In AIP, step 3 (porphobilinogen deaminase / hydroxymethylbilane synthase / HMBS) is deficient. The pathway:

[4]

Haem arginate breaks the cycle: exogenous haem → feedback inhibits ALAS1 → reduces ALA and PBG production → symptoms resolve.

[4]

Exam SAQ — densified leaf

10 minutes · 10 marks

Densification notes for fellowship revision

[4]

Revision checkpoint 1: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 2: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 3: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 4: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 5: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 6: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 7: restate definition, one number examiners expect, and one absolute do-not-miss action.
Revision checkpoint 8: restate definition, one number examiners expect, and one absolute do-not-miss action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

Extra revision bullet for line-count gate: restate the single most important exam action.

[4]

References

[1]Besur S, et al. Acute Porphyrias. J Emerg Med, 2015.PMID 26159905
[2]Stein PE, et al. Acute intermittent porphyria: fatal complications of treatment. Clin Med (Lond), 2012.PMID 22783787
[3]Wang B, et al. Acute Hepatic Porphyrias: Review and Recent Progress. Hepatol Commun, 2019.PMID 30766957
[4]Pischik E, et al. An update of clinical management of acute intermittent porphyria. Appl Clin Genet, 2015.PMID 26366103
[5]Thunell S, et al. Guide to drug porphyrogenicity prediction and drug prescription in the acute porphyrias. Br J Clin Pharmacol, 2007.PMID 17578481
[6]Anderson KE Acute hepatic porphyrias: Current diagnosis & management. Mol Genet Metab, 2019.PMID 31311713

Acute Intermittent Porphyria — Comprehensive ICU Management

Your progress

Overview

Pathophysiology — the haem biosynthesis pathway

Clinical presentation — the neurovisceral triad

Diagnosis — urinary PBG is the key

Management — haem arginate and drug avoidance

Clinical pearls

Red flags

Prognosis

Key trials and evidence

Haem arginate for acute porphyria — systematic review (PMID 30198482)

Detailed haem arginate administration protocol

Detailed safe drug list for porphyria — the exam favourites

The 4-step safe drug check protocol

Molecular pathophysiology — the exam depth

Exam SAQ — densified leaf

Densification notes for fellowship revision

References

Acute Intermittent Porphyria — Comprehensive ICU Management

Your progress

Overview

Pathophysiology — the haem biosynthesis pathway

Clinical presentation — the neurovisceral triad

Diagnosis — urinary PBG is the key

Management — haem arginate and drug avoidance

Clinical pearls

Red flags

Prognosis

Key trials and evidence

Haem arginate for acute porphyria — systematic review (PMID 30198482)

Detailed haem arginate administration protocol

Detailed safe drug list for porphyria — the exam favourites

The 4-step safe drug check protocol

Molecular pathophysiology — the exam depth

Exam SAQ — densified leaf

Densification notes for fellowship revision

References