Porphyria - Revision history

Chris.Rishel at 06:12, 24 February 2024

2024-02-24T06:12:07Z

Seanmliu: Updated blank article

2024-02-13T20:51:07Z

Updated blank article

← Older revision		Revision as of 13:51, 13 February 2024
Line 1:		Line 1:
	{{Infobox comorbidity		{{Infobox comorbidity
	\| other_names =		\| other_names =
	\| anesthetic_relevance =		\| anesthetic_relevance = High
	\| specialty =		\| anesthetic_management = Avoid prolonged fasting, prioritize post-op pain control, avoid drugs that can trigger an acute crisis
	\| signs_symptoms =		\| specialty = Hematology
	\| diagnosis =		\| signs_symptoms = Abdominal pain, neuropathies, hypertension
	\| treatment =		\| diagnosis = Urine and serum testing for porphyrins
			\| treatment = Heme and/or glucose injection
	\| image =		\| image =
	\| caption =		\| caption =
	}}		}}

	~~Provide~~ a ~~brief summary~~ of ~~this comorbidity here~~.		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)
			* Encourage clear carbohydrate fluids (i.e. gatorade) up to 2 hours before surgery
			* Can also opt for dextrose infusions until surgery

	=== 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
			* Drugs to avoid that can precipitate acute crisis:
			** Barbiturates
			** Etomidate
			** Phenobarbital
			** Ketamine
			** Diazepam
			** Phenytoin

	=== Postoperative management<!-- Describe how this comorbidity may influence postoperative management. --> ===		=== Postoperative management<!-- Describe how this comorbidity may influence postoperative management. --> ===

	~~== Related surgical procedures<!-- List and briefly describe any procedures which may be performed specifically to treat this comorbidity or its sequelae. If none~~, ~~this section may~~ be ~~removed. --> ==~~		* 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
			* 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>

	== Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> ==		== Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> ==
			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<!-- Describe how this comorbidity is diagnosed. --> ==		== Diagnosis<!-- Describe how this comorbidity is diagnosed. --> ==

			* Urine δ-amino levulinic acid (ALA) and porphobilinogen (PBG) levels

	== Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> ==		== Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> ==
Line 32:		Line 62:
	=== Medication<!-- Describe medications used to manage this comorbidity. --> ===		=== Medication<!-- Describe medications used to manage this comorbidity. --> ===

	~~=== Surgery<!-- Describe surgical procedures used to treat 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
			* Few symptoms, if any, between crises

	== Epidemiology<!-- Describe the epidemiology of this comorbidity --> ==		== Epidemiology<!-- Describe the epidemiology of this comorbidity --> ==

	== References ==		* More common in females
			* Typically present around ages 15-40

			== References<ref>{{Cite web\|title=Login Selection - Lane Medical Library, Stanford University Medical Center\|url=https://lane.stanford.edu/discoveryLoginPage.html?entityID=https%3A%2F%2Flane.stanford.edu%2Fshibboleth&return=https%3A%2F%2Flane.stanford.edu%2FShibboleth.sso%2FLogin%3FSAMLDS%3D1%26target%3Dss%253Amem%253A6c191971fb4f07c3781eb00caacf19e9c2e2215f7c6c1b74454b333725ac3620\|access-date=2024-02-13\|website=lane.stanford.edu}}</ref><ref>{{Cite journal\|last=Wilson-Baig\|first=N.\|last2=Badminton\|first2=M.\|last3=Schulenburg-Brand\|first3=D.\|date=2021-02\|title=Acute hepatic porphyria and anaesthesia: a practical approach to the prevention and management of acute neurovisceral attacks\|url=https://pubmed.ncbi.nlm.nih.gov/33889432/\|journal=BJA education\|volume=21\|issue=2\|pages=66–74\|doi=10.1016/j.bjae.2020.09.005\|issn=2058-5357\|pmc=7810766\|pmid=33889432}}</ref><ref>{{Cite journal\|last=Wilson-Baig\|first=N.\|last2=Badminton\|first2=M.\|last3=Schulenburg-Brand\|first3=D.\|date=2021-02\|title=Acute hepatic porphyria and anaesthesia: a practical approach to the prevention and management of acute neurovisceral attacks\|url=https://pubmed.ncbi.nlm.nih.gov/33889432/\|journal=BJA education\|volume=21\|issue=2\|pages=66–74\|doi=10.1016/j.bjae.2020.09.005\|issn=2058-5357\|pmc=7810766\|pmid=33889432}}</ref><ref>{{Cite web\|last=openanesthesia\|title=Porphyrias: Anesthetic Considerations\|url=https://www.openanesthesia.org/keywords/porphyrias-anesthetic-considerations/\|access-date=2024-02-13\|website=OpenAnesthesia\|language=en-US}}</ref> ==

	[[Category:Comorbidities]]		[[Category:Comorbidities]]

Oliviamsutton: Created page with "{{Infobox comorbidity | other_names = | anesthetic_relevance = | specialty = | signs_symptoms = | diagnosis = | treatment = | image = | caption = }} Provide a brief s..."

2022-01-24T20:03:56Z

Created page with "{{Infobox comorbidity | other_names = | anesthetic_relevance = | specialty = | signs_symptoms = | diagnosis = | treatment = | image = | caption = }} Provide a brief s..."

New page

{{Infobox comorbidity
| other_names =
| anesthetic_relevance =
| specialty =
| signs_symptoms =
| diagnosis =
| treatment =
| image =
| caption =
}}

Provide a brief summary of this comorbidity here.

== Anesthetic implications ==

=== Preoperative optimization ===

=== Intraoperative management ===

=== Postoperative management ===

== Related surgical procedures ==

== Pathophysiology ==

== Signs and symptoms ==

== Diagnosis ==

== Treatment ==

=== Medication ===

=== Surgery ===

=== Prognosis ===

== Epidemiology ==

== References ==

[[Category:Comorbidities]]

← Older revision		Revision as of 23:12, 23 February 2024
Line 11:		Line 11:
	}}		}}

	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
Line 22:		Line 21:

	=== 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:
Line 33:		Line 31:

	=== 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. --> ==
Line 55:		Line 49:

	== 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

Line 61:		Line 54:

	=== Medication<!-- Describe medications used to manage this comorbidity. --> ===		=== Medication<!-- Describe medications used to manage this comorbidity. --> ===
			* Heme arginate
	* ~~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