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Showing results for Acute Hepatic Porphyria: recommendation for evaluation and long term management
Your search for Acute Hepatic Porphyria: Recomendation for Evaluation and Long Term Management retrieved no results
Acute hepatic porphyrias: Recommendations for evaluation and long-term management.
Balwani M, Wang B, Anderson KE, Bloomer JR, Bissell DM, Bonkovsky HL, Phillips JD, Desnick RJ; Porphyrias Consortium of the Rare Diseases Clinical Research Network. Balwani M, et al. Hepatology. 2017 Oct;66(4):1314-1322. doi: 10.1002/hep.29313. Epub 2017 Sep 4. Hepatology. 2017. PMID: 28605040 Free PMC article. Review.
The acute hepatic porphyrias are a group of four inherited disorders, each resulting from a deficiency in the activity of a specific enzyme in the heme biosynthetic pathway. ...Annual assessments include biochemical testing and monitoring for long-t …
The acute hepatic porphyrias are a group of four inherited disorders, each resulting from a deficiency in the activity …
Hereditary Coproporphyria.
Wang B, Bissell DM. Wang B, et al. 2012 Dec 13 [updated 2022 May 19]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2025. 2012 Dec 13 [updated 2022 May 19]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2025. PMID: 23236641 Free Books & Documents. Review.
CLINICAL CHARACTERISTICS: Hereditary coproporphyria (HCP) is an acute (hepatic) porphyria in which the acute symptoms are neurovisceral and occur in discrete episodes. ...MANAGEMENT: Treatment of manifestations: Acute attacks are treated …
CLINICAL CHARACTERISTICS: Hereditary coproporphyria (HCP) is an acute (hepatic) porphyria in which the acute sym …
Acute Intermittent Porphyria.
Sardh E, Barbaro M. Sardh E, et al. 2005 Sep 27 [updated 2024 Feb 8]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2025. 2005 Sep 27 [updated 2024 Feb 8]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2025. PMID: 20301372 Free Books & Documents. Review.
Active (symptomatic) AIP: An individual who has experienced at least one acute attack within the last two years. Symptomatic high excreter: Urine PBG-to-creatinine ratio 4 times ULN and no acute attacks in the last two years but chronic long-standing manifest …
Active (symptomatic) AIP: An individual who has experienced at least one acute attack within the last two years. Symptomatic high exc …
Kidney Involvement in Acute Hepatic Porphyrias: Pathophysiology and Diagnostic Implications.
Ricci A, Guida CC, Manzini P, Cuoghi C, Ventura P. Ricci A, et al. Diagnostics (Basel). 2021 Dec 10;11(12):2324. doi: 10.3390/diagnostics11122324. Diagnostics (Basel). 2021. PMID: 34943561 Free PMC article. Review.
Depending on the specific enzyme involved, porphyrias manifest under drastically different clinical pictures. The most dramatic presentation of the four congenital acute hepatic porphyrias (AHPs: acute intermittent porphyria-AIP, ALAD def …
Depending on the specific enzyme involved, porphyrias manifest under drastically different clinical pictures. The most dramatic prese …