摘要 國軍高雄總醫院藥劑科藥師陳昱彣 張翌君 黃學揚 魏春生 郭力誠 (porphyria) porphyria heme 壹 前言 (porphobilinogen deminase) (heme) (porphobilinogen, PBG) (hydroxymethylbilane, HMB) - ( -aminolevulinic acid, ALA) 1-3 貳 紫質症種類 8 (delte-dminolevulinate-dehydratase deficiency porphyria, ADP) (acute Intermittent porohyria, AIP) (hereditary coproporphyria, HCP) (variegate porphyria, VP) (congenital erythropoietic porphyria, CEP) (porphyria cutaneatarda, PCT) (erythropieticprotoporphyria, EPP) (heptoerythropoietic porphyria, HEP) 2,4-8 ( ) 參 流行病學 20-40 2 500-5 2000 9 藥學雜誌 122 91 臨床藥物治療學紫質症的治療與文獻探討
Therapeutics of Clinical Drugs 臨床藥物治療學 5-10 10 10 11 1002 9 ( ) 1 1.5 301 2 1 表一紫質症分類與分析 Delta-aminolevulinate- dehydratase deficiency porphyria (ADP) acute intermittent porohyria (AIP) hereditary copropor phyria (HCP) variegate porphyria (VP) congenital erythropoietic porphyria (CEP) porphyria cutaneatarda (PCT) erythropieticprotoporphyria (EPP) hepatoerythropoietic porphyria (HEP) delteaminolevulinate-dehydratase (ALAD) porphobilinogendeaminase (PBGD) 1. 2. 3. copropor phyrinogen oxidase (CPO) +1+2 proto porphyrinogen oxidase (PPO) uroporphyrinogen II cosynthase (URSO) uroporphyrinogen decarboxylase (~75% deficiency) (UROD) ferrochelatase (~75% deficiency) uroporphyrinogen decarboxylase (~90% deficiency) +1+2 +2 +2 +3 肆 臨床症狀與診斷一 臨床症狀 33% 11 12 (creatine phosphor kinase, CPK) ALA PBG 2,4,9,12 92 THE JOURNAL OF TAIWAN PHARMACY Vol.31 No.1
13 2,8 5,7 5 二 診斷 (sulfonamides antibiotic) (barbiturates) (antiepileptic drugs) (estrogens) (ergots) (overload iron drug) (antifungal agents) 5,7,13 B C 13 4 DAN 4,13 臨床藥物治療 學 伍 治療 CPK 13 (450-600 gm) 2,5,7,11 Hemin lyophilized (250 ml/10 ml/amp) 4 5 Hemin lyophilized 100 ml 0.9% N/S 1 24 250 mg 5,13,14 陸 預後與預防 藥學雜誌 122 93
5,13 4,13 AIP 臨床藥Therapeutics of Clinical Drugs 物治療 學柒 總結 Porphyria Treatment and Literature Review Yu-Wen Chen, Yi-Chun Chang, Hsueh-Yang Huang, Chun-Sheng Guo, Li-Cheng Wei Department of Pharmacy, Kaohsiung Armed Forces General Hospital Abstract Porphyria is an autosomal dominant or induce clinical symptoms to be a rare disease cause by enzyme disorders with heme biosynthesis or stimulate facts trigger. Some patients have genetic gene without clinical presentation. However, other part of them present with symptoms because of stimulating factors trigger. After transfer to many different types of clinical physician under diagnosis, these patients may give up their treatment and had bad quality of life because of unable diagnosis and suffers from porphyria. This article will discuss eight different types of porphyria, their clinical symptoms include: unreasonable abdomen pain, hyponatremia, propurine et al. The treatment and literature review to be able provide medical health team acknowledge and help their patient and get right treatment. Also, notice stimulating factors may disable their symptoms trigger and benefit for patients to receive proper treatment and have a better life. 94 THE JOURNAL OF TAIWAN PHARMACY Vol.31 No.1
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