Consideration of Acute Porphyria in an Emergency Department Patient: A Case Report and Discussion of Common Pitfalls
DISCUSSION: Not considering acute porphyria in patients with recurring neurovisceral symptoms in the ED can lead to missed opportunities for diagnosing such pathologies.PMID:36427037 | DOI:10.5811/cpcem.2022.9.57507 (Source: Pain Physician)
Source: Pain Physician - November 25, 2022 Category: Anesthesiology Authors: Anthony Rios Lisa Kehrberg Hillary E Davis Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Recovery From Severe Systemic Peripheral Neuropathy Secondary to Erythropoietic Protoporphyria by Liver Transplant: A Case Report
Exp Clin Transplant. 2022 Oct;20(10):954-958. doi: 10.6002/ect.2022.0157.ABSTRACTErythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Ba...
Source: Experimental and Clinical Transplantation : official journal of the Middle East Society for Organ Transplantation - November 21, 2022 Category: Transplant Surgery Authors: Masato Shizuku Nobuhiko Kurata Kanta Jobara Taku Tanaka Akimasa Fukuta Mai Hatanaka Iwata Kazuhiro Hara Masahisa Katsuno Hajime Nakano Yasuhiro Ogura Source Type: research
Pain in acute hepatic porphyrias: Updates on pathophysiology and management
Acute hepatic porphyrias (AHPs) typically present with recurrent acute attacks of severe abdominal pain and acute autonomic dysfunction. While chronic symptoms were historically overlooked in the literature, recent studies have reported increased prevalence of chronic, mainly neuropathic, pain between the attacks. Here we characterize acute and chronic pain as prominent manifestations of the AHPs and discuss their pathophysiology and updated management. In addition to the severe abdominal pain, patients could experience low back pain, limb pain, and headache during acute attacks. Chronic pain between the attacks is typical...
Source: Frontiers in Neurology - November 21, 2022 Category: Neurology Source Type: research
Acute Intermittent Porphyria as a Rare Challenging Neuro-Metabolic Disease; a Case Report
Arch Acad Emerg Med. 2022 Sep 13;10(1):e74. doi: 10.22037/aaem.v10i1.1780. eCollection 2022.ABSTRACTPorphyria is a challenging metabolic disease due to its heterogeneous presentation symptoms and its difficult diagnosis. Many affected individuals can complain of recurrent neuro-visceral attacks per year, some of which may be persistent and life-threatening, which is confusing if there is no established diagnosis. Although the motor manifestations, autonomic changes and seizure are highly suggestive, the diagnosis is often overlooked and needs confirmatory genetic testing. To the best of our knowledge, the acute intermitten...
Source: Accident and Emergency Nursing - November 16, 2022 Category: Emergency Medicine Authors: Elsayed Abed Omer M Saeed Basem Abd El-Hamid Mohamed A Elsayed Heba Dawoud Nahla Mohamed Heikal Ahmed Hanei Elshafie Aya Hagar Hossam Emam Ahmed Farag El-Adawey Abdel-Ghaffar Fayed Ahmed Hassan Elsheshiny Mahmoud Galal Ahmed Source Type: research
Acute Intermittent Porphyria as a Rare Challenging Neuro-Metabolic Disease; a Case Report
Arch Acad Emerg Med. 2022 Sep 13;10(1):e74. doi: 10.22037/aaem.v10i1.1780. eCollection 2022.ABSTRACTPorphyria is a challenging metabolic disease due to its heterogeneous presentation symptoms and its difficult diagnosis. Many affected individuals can complain of recurrent neuro-visceral attacks per year, some of which may be persistent and life-threatening, which is confusing if there is no established diagnosis. Although the motor manifestations, autonomic changes and seizure are highly suggestive, the diagnosis is often overlooked and needs confirmatory genetic testing. To the best of our knowledge, the acute intermitten...
Source: Accident and Emergency Nursing - November 16, 2022 Category: Emergency Medicine Authors: Elsayed Abed Omer M Saeed Basem Abd El-Hamid Mohamed A Elsayed Heba Dawoud Nahla Mohamed Heikal Ahmed Hanei Elshafie Aya Hagar Hossam Emam Ahmed Farag El-Adawey Abdel-Ghaffar Fayed Ahmed Hassan Elsheshiny Mahmoud Galal Ahmed Source Type: research
Acute Intermittent Porphyria as a Rare Challenging Neuro-Metabolic Disease; a Case Report
Arch Acad Emerg Med. 2022 Sep 13;10(1):e74. doi: 10.22037/aaem.v10i1.1780. eCollection 2022.ABSTRACTPorphyria is a challenging metabolic disease due to its heterogeneous presentation symptoms and its difficult diagnosis. Many affected individuals can complain of recurrent neuro-visceral attacks per year, some of which may be persistent and life-threatening, which is confusing if there is no established diagnosis. Although the motor manifestations, autonomic changes and seizure are highly suggestive, the diagnosis is often overlooked and needs confirmatory genetic testing. To the best of our knowledge, the acute intermitten...
Source: Accident and Emergency Nursing - November 16, 2022 Category: Emergency Medicine Authors: Elsayed Abed Omer M Saeed Basem Abd El-Hamid Mohamed A Elsayed Heba Dawoud Nahla Mohamed Heikal Ahmed Hanei Elshafie Aya Hagar Hossam Emam Ahmed Farag El-Adawey Abdel-Ghaffar Fayed Ahmed Hassan Elsheshiny Mahmoud Galal Ahmed Source Type: research
Acute intermittent porphyria presenting with first episode seizure and rhabdomyolysis
(Source: Acta Neurologica Belgica)
Source: Acta Neurologica Belgica - November 5, 2022 Category: Neurology Source Type: research
Referral for sun allergy – a case of erythropoietic protoporphyria
Erythropoietic protoporphyria (EPP) is an inherited cutaneous porphyria characterized by painful, non-blistering photosensitivity usually noted in early childhood and occurring acutely after sunlight exposure. Cutaneous lesions develop within minutes of sun exposure with diffuse edema of the skin in sun-exposed areas, resembling angioedema. EPP is now recognized as the most common porphyria in children and the third most common in adults. In the review of over 100 cases in the United States, the average delay diagnosis of EPP was 13 years. (Source: Annals of Allergy, Asthma and Immunology)
Source: Annals of Allergy, Asthma and Immunology - November 1, 2022 Category: Allergy & Immunology Authors: K. Bourgoyne Tags: M337 Source Type: research
