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About 710 results

ALLMedicine™ Acute Intermittent Porphyria Center

Research & Reviews  186 results

Acute hepatic porphyrias no longer as rare as previously thought
https://www.mdedge.com/internalmedicine/article/260911/gastroenterology/acute-hepatic-porphyrias-no-longer-rare-previously
Carolyn Crist

Jan 30th, 2023 - Although rare, acute hepatic porphyrias (AHPs) may be more common than previously thought, particularly among women between ages 15 and 50, according to a new clinical practice update from the American Gastroenterological Association. For acute at.

Dental Health, Diet, Inflammation and Biomarkers in Patients With Acute Intermittent Porphyria(AIP)
https://clinicaltrials.gov/ct2/show/NCT01617642

Jan 23rd, 2023 - In a group of people with proven acute intermittent porphyria (AIP) mutation, some will remain asymptomatic, while others have repeated periods of porphyria symptoms. Glucose inhibits ALA synthetase (ALAS), the first rate-limiting enzyme in the ha...

Identification of Acute Intermittent Porphyria Modifying Genes
https://clinicaltrials.gov/ct2/show/NCT05502133

Jan 20th, 2023 - This study proposes to identify the predisposing/protective modifying genes that underlie the acute attacks in symptomatic patients with Acute Intermittent Porphyria (AIP), an autosomal dominant inborn error of heme biosynthesis.

AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: E...
https://doi.org/10.1053/j.gastro.2022.11.034
Gastroenterology Wang B, Bonkovsky HL et. al.

Jan 16th, 2023 - The acute hepatic porphyrias (AHP) are rare, inborn errors of heme-metabolism and include acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and porphyria due to severe deficiency of 5-aminolevulinic acid dehydratase. Ac...

Acute Intermittent Porphyria
https://emedicine.medscape.com/article/205220-overview

Jan 13th, 2023 - Practice Essentials Acute intermittent porphyria (AIP) is one of the porphyrias, a group of hereditary diseases that involve defects in heme metabolism and result in excessive secretion of porphyrins and porphyrin precursors. [1] AIP manifests as ...

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Guidelines  2 results

AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: E...
https://doi.org/10.1053/j.gastro.2022.11.034
Gastroenterology Wang B, Bonkovsky HL et. al.

Jan 16th, 2023 - The acute hepatic porphyrias (AHP) are rare, inborn errors of heme-metabolism and include acute intermittent porphyria, hereditary coproporphyria, variegate porphyria, and porphyria due to severe deficiency of 5-aminolevulinic acid dehydratase. Ac...

Best practice guidelines on clinical management of acute attacks of porphyria and their...
https://doi.org/10.1177/0004563212474555
Annals of Clinical Biochemistry; Stein P, Badminton M et. al.

Apr 23rd, 2013 - The British and Irish Porphyria Network guidelines describe best practice in the clinical assessment, investigation and management of acute porphyria attacks and their complications, including severe attacks with neuropathy. Acute attacks of porph...

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Drugs  68 results see all →

Clinicaltrials.gov  16 results

Dental Health, Diet, Inflammation and Biomarkers in Patients With Acute Intermittent Porphyria(AIP)
https://clinicaltrials.gov/ct2/show/NCT01617642

Jan 23rd, 2023 - In a group of people with proven acute intermittent porphyria (AIP) mutation, some will remain asymptomatic, while others have repeated periods of porphyria symptoms. Glucose inhibits ALA synthetase (ALAS), the first rate-limiting enzyme in the ha...

Identification of Acute Intermittent Porphyria Modifying Genes
https://clinicaltrials.gov/ct2/show/NCT05502133

Jan 20th, 2023 - This study proposes to identify the predisposing/protective modifying genes that underlie the acute attacks in symptomatic patients with Acute Intermittent Porphyria (AIP), an autosomal dominant inborn error of heme biosynthesis.

Comparision of Motor Seizure Duration of Ketofol and Propofol for Electroconvulsive Therapy
https://clinicaltrials.gov/ct2/show/NCT05408000

Jun 7th, 2022 - Electroconvulsive therapy (ECT) is a common treatment method used in severe depression and other psychiatric diseases. Currently, most ECT procedures are carried out with muscle paralysis under general anesthesia. It is important to establish an a...

Panhematin for Prevention of Acute Attacks of Porphyria
https://clinicaltrials.gov/ct2/show/NCT02922413

Nov 26th, 2021 - This is a double-blind, randomized, placebo-controlled, parallel group trial investigating the efficacy and safety of Panhematin™ for preventing acute attacks in at least 20 subjects with well-documented acute porphyria (acute intermittent porphyr...

Carbohydrate Counting and DASH Intervention Among Children With Diabetes and Celiac Disease.
https://clinicaltrials.gov/ct2/show/NCT05132725

Nov 24th, 2021 - the main objective of this study to compare the effects of three diets: carbohydrate count with gluten-free diet, DASH and carbohydrate count with gluten-free diet, and gluten-free diet alone on glycemic control, growth rate, and the quality of li...

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News  9 results

Acute hepatic porphyrias no longer as rare as previously thought
https://www.mdedge.com/internalmedicine/article/260911/gastroenterology/acute-hepatic-porphyrias-no-longer-rare-previously
Carolyn Crist

Jan 30th, 2023 - Although rare, acute hepatic porphyrias (AHPs) may be more common than previously thought, particularly among women between ages 15 and 50, according to a new clinical practice update from the American Gastroenterological Association. For acute at.

Royal Family Affliction or Not, Porphyria Is Treatable
https://www.medscape.com/viewarticle/981041

Sep 19th, 2022 - European royal families may be enormously rich, but being a blueblood doesn't always mean your blood is pristine. Queen Victoria's DNA is famously believed to have silently bequeathed hemophilia to many of her descendants, including a great-grands...

Chronic blistering rash on hands
https://www.mdedge.com/familymedicine/article/211805/dermatology/chronic-blistering-rash-hands/page/0/2?channel=203
MDedge Family Medicine;

Nov 7th, 2019 - Pseudoporphyria has a similar presentation to PCT but with no abnormalities in porphyrin metabolism. Risk factors include UV radiation exposure; use of medications such as nonsteroidal anti-inflammatory drugs, diuretics, and retinoids; chronic ren.

VIDEO: Givosiran cuts acute intermittent porphyria attacks in pivotal trial
https://www.mdedge.com/internalmedicine/article/199737/hepatology/video-givosiran-cuts-acute-intermittent-porphyria-attacks/page/0/1?channel=39313

Apr 28th, 2019 - The study’s primary endpoint was the average annualized rate of porphyria attacks during 6 months of treatment, which was 3. 2 attacks in 46 patients evaluable for efficacy on givosiran treatment and 12.

VIDEO: Givosiran cuts acute intermittent porphyria attacks in pivotal trial
https://www.mdedge.com/internalmedicine/article/199737/hepatology/video-givosiran-cuts-acute-intermittent-porphyria-attacks
Mitchel L. Zoler, PhD

Apr 28th, 2019 - VIENNA – A novel RNA-inhibitor drug, givosiran, produced a large cut in acute porphyria attacks in a pivotal trial with 94 patients with acute hepatic porphyria. Although the study also identified some safety issues with givosiran, an RNA-inhibito.

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Patient Education  3 results see all →