Difference between revisions of "Porphyria"
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Porphyrias are a group of hereditary diseases that cause interruptions in heme synthesis, resulting in accumulation of porphyrins. These accumulations have adverse affects across organ systems that often make anesthetic management of these patients challenging, requiring special considerations starting in the pre-operative period. Patients with porphyrias can be categorized as having acute or non-acute porphyria, with Acute Intermittent Porphyria (AIP) being the most common type. Acute patients will require alterations in anesthetic management, while non-acute patients can be managed like patients without porphyria. | '''Porphyrias''' are a group of hereditary diseases that cause interruptions in heme synthesis, resulting in accumulation of porphyrins. These accumulations have adverse affects across organ systems that often make anesthetic management of these patients challenging, requiring special considerations starting in the pre-operative period. Patients with porphyrias can be categorized as having acute or non-acute porphyria, with Acute Intermittent Porphyria (AIP) being the most common type. Acute patients will require alterations in anesthetic management, while non-acute patients can be managed like patients without porphyria. | ||
== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> == | == Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> == | ||
=== Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. --> === | === Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. --> === | ||
* Avoid prolonged fasting (>2 hours) | * Avoid prolonged fasting (>2 hours) | ||
* Encourage clear carbohydrate fluids (i.e. gatorade) up to 2 hours before surgery | * Encourage clear carbohydrate fluids (i.e. gatorade) up to 2 hours before surgery | ||
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=== Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. --> === | === Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. --> === | ||
* General and regional/neuraxial can be safely used | * General and regional/neuraxial can be safely used | ||
* Drugs to avoid that can precipitate acute crisis: | * Drugs to avoid that can precipitate acute crisis: | ||
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=== Postoperative management<!-- Describe how this comorbidity may influence postoperative management. --> === | === Postoperative management<!-- Describe how this comorbidity may influence postoperative management. --> === | ||
* Ensure adequate pain control, as excessive pain can be a trigger for acute crisis | * Ensure adequate pain control, as excessive pain can be a trigger for acute crisis | ||
== Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> == | == Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> == | ||
* Porphyrins are compounds important in the synthesis of heme | * Porphyrins are compounds important in the synthesis of heme | ||
* Porphyria occurs when the synthesis of heme is interrupted, resulting in the accumulation of porphyrins | * Porphyria occurs when the synthesis of heme is interrupted, resulting in the accumulation of porphyrins<ref>{{Cite journal|last=Kauppinen|first=Raili|date=January 15, 2005|title=Porphyrias|url=https://doi.org/10.1016/S0140-6736(05)17744-7|journal=Lancet}}</ref> | ||
<ref>{{Cite journal|last=Kauppinen|first=Raili|date=January 15, 2005|title=Porphyrias|url=https://doi.org/10.1016/S0140-6736(05)17744-7|journal=Lancet}}</ref> | |||
== Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> == | == Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> == | ||
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== Diagnosis<!-- Describe how this comorbidity is diagnosed. --> == | == Diagnosis<!-- Describe how this comorbidity is diagnosed. --> == | ||
* Urine δ-amino levulinic acid (ALA) and porphobilinogen (PBG) levels | * Urine δ-amino levulinic acid (ALA) and porphobilinogen (PBG) levels | ||
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=== Medication<!-- Describe medications used to manage this comorbidity. --> === | === Medication<!-- Describe medications used to manage this comorbidity. --> === | ||
* Heme arginate | |||
* | |||
=== Prognosis<!-- Describe the prognosis of this comorbidity --> === | === Prognosis<!-- Describe the prognosis of this comorbidity --> === | ||
* Good when acute crisis is recognized and treated appropriately | * Good when acute crisis is recognized and treated appropriately | ||
* Few symptoms, if any, between crises | * Few symptoms, if any, between crises | ||
== Epidemiology<!-- Describe the epidemiology of this comorbidity --> == | == Epidemiology<!-- Describe the epidemiology of this comorbidity --> == | ||
* More common in females | * More common in females | ||
* Typically present around ages 15-40 | * Typically present around ages 15-40 | ||
Latest revision as of 23:12, 23 February 2024
Porphyria
| Anesthetic relevance |
High |
|---|---|
| Anesthetic management |
Avoid prolonged fasting, prioritize post-op pain control, avoid drugs that can trigger an acute crisis |
| Specialty |
Hematology |
| Signs and symptoms |
Abdominal pain, neuropathies, hypertension |
| Diagnosis |
Urine and serum testing for porphyrins |
| Treatment |
Heme and/or glucose injection |
| Article quality | |
| Editor rating | |
| User likes | 0 |
Porphyrias are a group of hereditary diseases that cause interruptions in heme synthesis, resulting in accumulation of porphyrins. These accumulations have adverse affects across organ systems that often make anesthetic management of these patients challenging, requiring special considerations starting in the pre-operative period. Patients with porphyrias can be categorized as having acute or non-acute porphyria, with Acute Intermittent Porphyria (AIP) being the most common type. Acute patients will require alterations in anesthetic management, while non-acute patients can be managed like patients without porphyria.
Anesthetic implications
Preoperative optimization
- Avoid prolonged fasting (>2 hours)
- Encourage clear carbohydrate fluids (i.e. gatorade) up to 2 hours before surgery
- Can also opt for dextrose infusions until surgery
Intraoperative management
- General and regional/neuraxial can be safely used
- Drugs to avoid that can precipitate acute crisis:
- Barbiturates
- Etomidate
- Phenobarbital
- Ketamine
- Diazepam
- Phenytoin
Postoperative management
- Ensure adequate pain control, as excessive pain can be a trigger for acute crisis
Pathophysiology
- Porphyrins are compounds important in the synthesis of heme
- Porphyria occurs when the synthesis of heme is interrupted, resulting in the accumulation of porphyrins[1]
Signs and symptoms
Can be separated into acute vs nonacute, with non-acute primarily only having dermatologic symptoms
Acute:
- Abdominal pain
- Weakness/neuropathies
- Hypertension
- Tachyarrhythmias
- Respiratory arrest secondary to bulbar muscle weakness
Diagnosis
- Urine δ-amino levulinic acid (ALA) and porphobilinogen (PBG) levels
Treatment
Medication
- Heme arginate
Prognosis
- Good when acute crisis is recognized and treated appropriately
- Few symptoms, if any, between crises
Epidemiology
- More common in females
- Typically present around ages 15-40
References[2][3][4][5]
- ↑ Kauppinen, Raili (January 15, 2005). "Porphyrias". Lancet.
- ↑ "Login Selection - Lane Medical Library, Stanford University Medical Center". lane.stanford.edu. Retrieved 2024-02-13.
- ↑ Wilson-Baig, N.; Badminton, M.; Schulenburg-Brand, D. (2021-02). "Acute hepatic porphyria and anaesthesia: a practical approach to the prevention and management of acute neurovisceral attacks". BJA education. 21 (2): 66–74. doi:10.1016/j.bjae.2020.09.005. ISSN 2058-5357. PMC 7810766. PMID 33889432. Check date values in:
|date=(help) - ↑ Wilson-Baig, N.; Badminton, M.; Schulenburg-Brand, D. (2021-02). "Acute hepatic porphyria and anaesthesia: a practical approach to the prevention and management of acute neurovisceral attacks". BJA education. 21 (2): 66–74. doi:10.1016/j.bjae.2020.09.005. ISSN 2058-5357. PMC 7810766. PMID 33889432. Check date values in:
|date=(help) - ↑ openanesthesia. "Porphyrias: Anesthetic Considerations". OpenAnesthesia. Retrieved 2024-02-13.
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