ALLMedicine™ Acute Intermittent Porphyria Center
Research & Reviews 365 results
https://doi.org/10.1007/s40265-021-01511-3 10.1002/hep4.1297 10.1056/NEJMra1608634 10.1007/s11910-020-01078-8 10.1002/humu.23067 10.1016/j.amjmed.2014.06.036 10.1016/j.amjmed.2014.10.026 10.1002/hep.29313 10.1177/0004563212474555 10.1002/hep.30936 10.1007/s40271-018-0319-3 10.1016/S0140-6736(09)61925-5 10.1111/joim.12750 10.1007/s40291-019-00438-6 10.1016/j.ymthe.2020.06.015 10.1056/NEJMe2010986 10.1073/pnas.1406228111 10.1038/mtna.2015.36 10.1056/NEJMoa1807838 10.1002/cpt.1802 10.1056/NEJMoa1913147 10.1080/23808993.2021.1838275 10.1016/j.ejim.2020.04.002 10.1358/dot.2021.57.1.3230207
Drugs Syed YY
Apr 19th, 2021 - Givosiran (Givlaari®) is an δ-aminolevulinic acid synthase 1 (ALAS1)-directed small interfering RNA (siRNA) approved for the treatment of acute hepatic porphyria (AHP). In the phase 3 ENVISION trial, givosiran significantly reduced the annualized ...
https://doi.org/10.1002/jimd.12391
Journal of Inherited Metabolic Disease; To-Figueras J, Wijngaard R et. al.
Apr 16th, 2021 - Acute intermittent porphyria (AIP) is a rare metabolic disease caused by mutations within the hydroxymethylbilane synthase gene. Previous studies have reported increased levels of plasma total homocysteine (tHcy) in symptomatic AIP patients. In th...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7913433
Orphanet Journal of Rare Diseases; Buendía-Martínez J, Barreda-Sánchez M et. al.
Feb 28th, 2021 - Acute intermittent porphyria (AIP) is a genetic disease characterized by acute neurovisceral attacks. Long-term clinical conditions, chronic symptoms and impaired health related quality of life (HRQoL) have been reported during non-attack periods ...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7809922
The Journal of International Medical Research; Huang S, Li R et. al.
Jan 12th, 2021 - Acute intermittent porphyria (AIP) is a rare autosomal dominant metabolic disease with a broad spectrum of clinical manifestations, and can be easily confused with other diseases. Many patients with porphyria may have symptoms of peripheral nerve ...
https://doi.org/10.1002/lt.25979
Liver Transplantation : Official Publication of the Ameri... Moghe A, Anderson KE
Dec 28th, 2020 - Expanding Experience With Liver Transplantation in Acute Intermittent Porphyria.|2020|Moghe A,Anderson KE,|
Guidelines 1 results
https://doi.org/10.1177/0004563212474555
Annals of Clinical Biochemistry; Stein P, Badminton M et. al.
Apr 22nd, 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 67 results see all →
Clinicaltrials.gov 441 results
https://doi.org/10.1007/s40265-021-01511-3 10.1002/hep4.1297 10.1056/NEJMra1608634 10.1007/s11910-020-01078-8 10.1002/humu.23067 10.1016/j.amjmed.2014.06.036 10.1016/j.amjmed.2014.10.026 10.1002/hep.29313 10.1177/0004563212474555 10.1002/hep.30936 10.1007/s40271-018-0319-3 10.1016/S0140-6736(09)61925-5 10.1111/joim.12750 10.1007/s40291-019-00438-6 10.1016/j.ymthe.2020.06.015 10.1056/NEJMe2010986 10.1073/pnas.1406228111 10.1038/mtna.2015.36 10.1056/NEJMoa1807838 10.1002/cpt.1802 10.1056/NEJMoa1913147 10.1080/23808993.2021.1838275 10.1016/j.ejim.2020.04.002 10.1358/dot.2021.57.1.3230207
Drugs Syed YY
Apr 19th, 2021 - Givosiran (Givlaari®) is an δ-aminolevulinic acid synthase 1 (ALAS1)-directed small interfering RNA (siRNA) approved for the treatment of acute hepatic porphyria (AHP). In the phase 3 ENVISION trial, givosiran significantly reduced the annualized ...
https://doi.org/10.1002/jimd.12391
Journal of Inherited Metabolic Disease; To-Figueras J, Wijngaard R et. al.
Apr 16th, 2021 - Acute intermittent porphyria (AIP) is a rare metabolic disease caused by mutations within the hydroxymethylbilane synthase gene. Previous studies have reported increased levels of plasma total homocysteine (tHcy) in symptomatic AIP patients. In th...
https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=9984267a-4d57-4444-9bb5-16bca7dea691
Apr 1st, 2021 - PANHEMATIN is a hemin for injection indicated for the amelioration of recurrent attacks of acute intermittent porphyria temporally related to the menstrual cycle in susceptible women, after initial carbohydrate therapy is known or suspected to be ...
https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=d58570a0-58b0-4662-b2ad-aa8d1463b4c1
Mar 28th, 2021 - For the management of manifestations of psychotic disorders. For the treatment of schizophrenia. To control nausea and vomiting. For relief of restlessness and apprehension before surgery. For acute intermittent porphyria. As an adjunct in the tre...
https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=a6065ac5-a111-449b-b51b-8bc2907d1ddb
Mar 23rd, 2021 - For the management of manifestations of psychotic disorders. For the treatment of schizophrenia. To control nausea and vomiting. For relief of restlessness and apprehension before surgery. For acute intermittent porphyria. As an adjunct in the tre...
News 9 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.medscape.com/viewarticle/889629
Dec 5th, 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/page/0/1
Mar 1st, 2016 - Treatment Treatment targets runaway heme precursor synthesis at its start and finish (Figure). Glucose-loading suppresses the initial enzyme, ALA synthase.