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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
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The three most common porphyrias and their contrasting presentations, exacerbating factors, and approaches to diagnosis and treatment

The three most common porphyrias and their contrasting presentations, exacerbating factors, and approaches to diagnosis and treatment
Disease Presenting symptoms Exacerbating factors Most important screening tests Treatment
Porphyria cutanea tarda (PCT) Chronic, blistering skin lesions* Iron; alcohol; smoking; estrogens; hepatitis C; HIV; halogenated hydrocarbons Plasma or urine porphyrins Phlebotomy; low-dose hydroxychloroquine or chloroquine
Acute intermittent porphyria (AIP) NeurovisceralΔ Drugs (mostly cytochrome P450-inducers); progesterone; dietary restrictionsΔ Urinary porphobilinogen (PBG)Δ Hemin; glucoseΔ
Erythropoietic protoporphyria (EPP) Acute, nonblistering skin photosensitivity   Erythrocyte protoporphyrin (total and metal-free) β-carotene; afamelanotide
Porphyria cutanea tarda (PCT) and acute intermittent porphyria (AIP) are distinct, but each shares features with some less common porphyrias (listed in the footnotes and discussed in detail in the overview of porphyrias topic in UpToDate).
β: beta.
* Also may occur in variegate porphyria (VP), less commonly in hereditary coproporphyria (HCP), and rarely in AIP with concomitant renal failure; much more severe in congenital erythropoietic protoporphyria (CEP) and hepatoerythropoietic porphyria (HEP).
¶ Also useful for diagnosis of variegate porphyria (VP), congenital erythropoietic porphyria (CEP), and hepatoerythropoietic porphyria (HEP).
Δ Features shared with hereditary coproporphyria (HCP), variegate porphyria (VP), and delta-aminolevulinic acid (ALA) dehydratase porphyria (ADP).
Provided by Karl Anderson, MD, FACP.
Graphic 51232 Version 8.0

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