ALLMedicine™ Acute Intermittent Porphyria Center
Research & Reviews 177 results
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...
https://doi.org/10.1016/j.jhep.2022.05.018
Journal of Hepatology; Molina L, Zhu J et. al.
Jun 1st, 2022 - Acute intermittent porphyria (AIP), caused by heterozygous germline mutations of the heme synthesis pathway enzyme HMBS (hydroxymethylbilane synthase), confers high risk of hepatocellular carcinoma (HCC) development. Yet, the role of HMBS in liver...
https://doi.org/10.1136/jclinpath-2021-207647
Journal of Clinical Pathology; Schulenburg-Brand D, Stewart F et. al.
May 19th, 2022 - The autosomal dominant acute hepatic porphyrias (AHPs), acute intermittent porphyria, hereditary coproporphyria (HCP) and variegate porphyria (VP), are low penetrance adult onset disorders caused by partial deficiency of enzymes of haem biosynthes...
https://doi.org/10.7861/clinmed.2022-0100
Clinical Medicine (London, England); Amador AP, Cordero AS et. al.
May 19th, 2022 - A 40-year-old man attended the emergency room with abdominal pain and inappropriate behaviour associated with stress, and the consumption of alcohol and cannabis. Examination revealed hypertension (155/100 mmHg), tachycardia (95 beats per minute),...
https://doi.org/10.1111/bph.15821
British Journal of Pharmacology; Vera-Yunca D, Córdoba KM et. al.
Feb 17th, 2022 - Acute intermittent porphyria (AIP) is a rare disease caused by a genetic mutation in the hepatic activity of the porphobilinogen-deaminase. We aimed to develop a mechanistic model of the enzymatic restoration effects of a novel therapy based on th...
Guidelines 1 results
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...
Drugs 53 results see all →
Clinicaltrials.gov 15 results
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...
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...
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...
https://clinicaltrials.gov/ct2/show/NCT02949830
Nov 17th, 2021 - The purpose of this study is to determine the long-term safety, tolerability and pharmacokinetics of givosiran (ALN-AS1) in AIP patients
https://clinicaltrials.gov/ct2/show/NCT03284658
May 18th, 2021 - Hereditary Tyrosinemia type 1 (HT-1) is a rare genetic disorder in which the newborn child lacks the ability to break down the amino acid tyrosine. As a result of this deficiency, toxic sub-stances build up in the blood and can cause liver failure...
News 6 results
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.
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.
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.
https://www.medscape.com/viewarticle/889629
Dec 6th, 2017 - NEW YORK (Reuters Health) - Abdominal pain and hyponatremia in a 16-year-old girl with constipation and disordered eating confounded the diagnosis of a much less common condition, acute intermittent porphyria (AIP), according to a case report publ...
https://www.mdedge.com/emergencymedicine/article/107206/hematology/case-report-acute-intermittent-porphyria
Antonio Dajer, MD, Louis Cooper, MD
Mar 1st, 2016 - Case A 34-year-old woman presented to the ED with severe, persistent abdominal pain that had begun 18 days earlier. She was 7 weeks pregnant and had been seen in the same ED the day before.